CMS finalizes CAR T-cell therapy inpatient payments

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Medical associations are expressing disappointment at the new payment scheme put forward by the Centers for Medicare & Medicaid Services for inpatient administration of two chimeric antigen receptor (CAR) T-cell therapies, calling the reimbursement insufficient for use of the expensive medications.

Courtesy Novartis

Under its Aug. 17 final rule, CMS will now categorize CAR T-cell therapies under the umbrella of the renamed Medicare Severity–Diagnosis Related Groups (MS-DRG) 016 – Autologous Bone Marrow Transplant with CC/MCC or T-cell Immunotherapy – and assign ICD-10-PCS procedure codes XW033C3 and XW043C3 to the use of axicabtagene ciloleucel (Yescarta) and tisagenlecleucel (Kymriah) in the inpatient setting for fiscal year 2019, which begins in October 2018.

CMS also approved a temporary New Technology Add-On Payment (NTAP) for use of the therapies with a maximum threshold of $186,500, according to the rule.

According to the American Society of Hematology (ASH), this payment structure is an improvement, but it hardly covers the cost of the products, nor does it account for full hospitalization costs. ASH noted that the revised MS-DRG 016 has a base payment rate of $36,000 and that the maximum NTAP payment ($186,500) is only about half of the cost for a CAR T-cell product.

“ASH is concerned that this final policy may impede access to care to this cutting-edge therapy because hospitals and academic medical centers that provide this personalized treatment will simply not be able to withstand the negative financial impact,” the society said in a statement. “While this final policy represents an improvement over current CAR T therapy reimbursement rates, ASH believes patient access to care will be jeopardized as providers and hospitals will not be able to afford to deliver the therapy at this reimbursement rate, particularly as other CAR T products receive FDA [Food and Drug Administration] approval.”

ASH and the American Society for Blood and Marrow Transplantation (ASBMT) had strongly urged CMS to develop a site-neutral, equitable payment structure that would have allowed providers to recover more product acquisition costs from CAR T-cell therapies. In its final rule, CMS stated that it was too early to develop a novel payment structure for CAR T-cell treatments and that more research is needed before such changes are made. The agency noted that in May CMS opened a national coverage determination analysis on CAR T-cell therapy for Medicare patients with advanced cancer, which is expected to be completed by May 2019.

“[CMS] is soliciting public comment … on key design considerations for developing a potential model that would test private market strategies and introduce competition to improve quality of care for beneficiaries,” the agency said in the rule. “Given the relative newness of CAR T-cell therapy, the potential model, and our request for feedback on this model approach, we believe it would be premature to adopt changes to our existing payment mechanisms.”

The payment outline by CMS is essentially the bare minimum it could have extended to CAR T-cell therapies for 2019, said Stephanie Farnia, director of health policy and strategic relations for the ASBMT.

“[ASBMT] and a number of stakeholders have been very clear in our comment letters that that would not be enough and the reasons why,” Ms. Farnia said in an interview. “It’s not going to be sufficient to cover the cost of care or the product.”

The rule also fails to address the cancer centers that are exempt from the DRG payment system, Ms. Farnia said. Eleven centers are excluded from the payment system because of past legislation that excludes exclusive cancer hospitals that do not provide noncancer services. The exempt cancer centers cannot receive additional money for new or expensive drugs and therefore will not gain any financial relief from the CAR T-cell therapy payment changes in the CMS final rule.

ASH officials plan to follow up with congressional leaders to identify ways to improve future CAR T-cell therapy payments, including a potential legislative solution. An ASH spokesperson declined to elaborate on its ideal legislative remedy.

Hospital administrators and physicians will need to have difficult conversations in the upcoming year about whether treating patients with CAR T-cell therapies is worth the cost deficits, Ms. Farnia said.


“Everyone was really counting on it being a different reimbursement scenario for the upcoming fiscal year, and it is, but again, it’s that bare minimum difference,” Ms. Farnia said. “I think a number of programs are going to be taking a look at their financial experience thus far and comparing that to the reimbursement and deciding on if they [should] continue to offer it and how to do that.”

In April 2018, CMS announced payment rates for outpatient administration of the two drugs, settling on $395,380 for axicabtagene ciloleucel and $500,839 for tisagenlecleucel. The two medications have list prices of $373,000 and $475,000, respectively.

However, physicians have raised concerns that even if the drugs are first administered in the outpatient setting, inpatient care is likely to occur with CAR T-cell therapies because some patients will need to be admitted in order to be monitored for serious side effects. In such cases, all payments will become part of the inpatient stay under CMS’s 3-day payment window rule.

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Medical associations are expressing disappointment at the new payment scheme put forward by the Centers for Medicare & Medicaid Services for inpatient administration of two chimeric antigen receptor (CAR) T-cell therapies, calling the reimbursement insufficient for use of the expensive medications.

Courtesy Novartis

Under its Aug. 17 final rule, CMS will now categorize CAR T-cell therapies under the umbrella of the renamed Medicare Severity–Diagnosis Related Groups (MS-DRG) 016 – Autologous Bone Marrow Transplant with CC/MCC or T-cell Immunotherapy – and assign ICD-10-PCS procedure codes XW033C3 and XW043C3 to the use of axicabtagene ciloleucel (Yescarta) and tisagenlecleucel (Kymriah) in the inpatient setting for fiscal year 2019, which begins in October 2018.

CMS also approved a temporary New Technology Add-On Payment (NTAP) for use of the therapies with a maximum threshold of $186,500, according to the rule.

According to the American Society of Hematology (ASH), this payment structure is an improvement, but it hardly covers the cost of the products, nor does it account for full hospitalization costs. ASH noted that the revised MS-DRG 016 has a base payment rate of $36,000 and that the maximum NTAP payment ($186,500) is only about half of the cost for a CAR T-cell product.

“ASH is concerned that this final policy may impede access to care to this cutting-edge therapy because hospitals and academic medical centers that provide this personalized treatment will simply not be able to withstand the negative financial impact,” the society said in a statement. “While this final policy represents an improvement over current CAR T therapy reimbursement rates, ASH believes patient access to care will be jeopardized as providers and hospitals will not be able to afford to deliver the therapy at this reimbursement rate, particularly as other CAR T products receive FDA [Food and Drug Administration] approval.”

ASH and the American Society for Blood and Marrow Transplantation (ASBMT) had strongly urged CMS to develop a site-neutral, equitable payment structure that would have allowed providers to recover more product acquisition costs from CAR T-cell therapies. In its final rule, CMS stated that it was too early to develop a novel payment structure for CAR T-cell treatments and that more research is needed before such changes are made. The agency noted that in May CMS opened a national coverage determination analysis on CAR T-cell therapy for Medicare patients with advanced cancer, which is expected to be completed by May 2019.

“[CMS] is soliciting public comment … on key design considerations for developing a potential model that would test private market strategies and introduce competition to improve quality of care for beneficiaries,” the agency said in the rule. “Given the relative newness of CAR T-cell therapy, the potential model, and our request for feedback on this model approach, we believe it would be premature to adopt changes to our existing payment mechanisms.”

The payment outline by CMS is essentially the bare minimum it could have extended to CAR T-cell therapies for 2019, said Stephanie Farnia, director of health policy and strategic relations for the ASBMT.

“[ASBMT] and a number of stakeholders have been very clear in our comment letters that that would not be enough and the reasons why,” Ms. Farnia said in an interview. “It’s not going to be sufficient to cover the cost of care or the product.”

The rule also fails to address the cancer centers that are exempt from the DRG payment system, Ms. Farnia said. Eleven centers are excluded from the payment system because of past legislation that excludes exclusive cancer hospitals that do not provide noncancer services. The exempt cancer centers cannot receive additional money for new or expensive drugs and therefore will not gain any financial relief from the CAR T-cell therapy payment changes in the CMS final rule.

ASH officials plan to follow up with congressional leaders to identify ways to improve future CAR T-cell therapy payments, including a potential legislative solution. An ASH spokesperson declined to elaborate on its ideal legislative remedy.

Hospital administrators and physicians will need to have difficult conversations in the upcoming year about whether treating patients with CAR T-cell therapies is worth the cost deficits, Ms. Farnia said.


“Everyone was really counting on it being a different reimbursement scenario for the upcoming fiscal year, and it is, but again, it’s that bare minimum difference,” Ms. Farnia said. “I think a number of programs are going to be taking a look at their financial experience thus far and comparing that to the reimbursement and deciding on if they [should] continue to offer it and how to do that.”

In April 2018, CMS announced payment rates for outpatient administration of the two drugs, settling on $395,380 for axicabtagene ciloleucel and $500,839 for tisagenlecleucel. The two medications have list prices of $373,000 and $475,000, respectively.

However, physicians have raised concerns that even if the drugs are first administered in the outpatient setting, inpatient care is likely to occur with CAR T-cell therapies because some patients will need to be admitted in order to be monitored for serious side effects. In such cases, all payments will become part of the inpatient stay under CMS’s 3-day payment window rule.

 

Medical associations are expressing disappointment at the new payment scheme put forward by the Centers for Medicare & Medicaid Services for inpatient administration of two chimeric antigen receptor (CAR) T-cell therapies, calling the reimbursement insufficient for use of the expensive medications.

Courtesy Novartis

Under its Aug. 17 final rule, CMS will now categorize CAR T-cell therapies under the umbrella of the renamed Medicare Severity–Diagnosis Related Groups (MS-DRG) 016 – Autologous Bone Marrow Transplant with CC/MCC or T-cell Immunotherapy – and assign ICD-10-PCS procedure codes XW033C3 and XW043C3 to the use of axicabtagene ciloleucel (Yescarta) and tisagenlecleucel (Kymriah) in the inpatient setting for fiscal year 2019, which begins in October 2018.

CMS also approved a temporary New Technology Add-On Payment (NTAP) for use of the therapies with a maximum threshold of $186,500, according to the rule.

According to the American Society of Hematology (ASH), this payment structure is an improvement, but it hardly covers the cost of the products, nor does it account for full hospitalization costs. ASH noted that the revised MS-DRG 016 has a base payment rate of $36,000 and that the maximum NTAP payment ($186,500) is only about half of the cost for a CAR T-cell product.

“ASH is concerned that this final policy may impede access to care to this cutting-edge therapy because hospitals and academic medical centers that provide this personalized treatment will simply not be able to withstand the negative financial impact,” the society said in a statement. “While this final policy represents an improvement over current CAR T therapy reimbursement rates, ASH believes patient access to care will be jeopardized as providers and hospitals will not be able to afford to deliver the therapy at this reimbursement rate, particularly as other CAR T products receive FDA [Food and Drug Administration] approval.”

ASH and the American Society for Blood and Marrow Transplantation (ASBMT) had strongly urged CMS to develop a site-neutral, equitable payment structure that would have allowed providers to recover more product acquisition costs from CAR T-cell therapies. In its final rule, CMS stated that it was too early to develop a novel payment structure for CAR T-cell treatments and that more research is needed before such changes are made. The agency noted that in May CMS opened a national coverage determination analysis on CAR T-cell therapy for Medicare patients with advanced cancer, which is expected to be completed by May 2019.

“[CMS] is soliciting public comment … on key design considerations for developing a potential model that would test private market strategies and introduce competition to improve quality of care for beneficiaries,” the agency said in the rule. “Given the relative newness of CAR T-cell therapy, the potential model, and our request for feedback on this model approach, we believe it would be premature to adopt changes to our existing payment mechanisms.”

The payment outline by CMS is essentially the bare minimum it could have extended to CAR T-cell therapies for 2019, said Stephanie Farnia, director of health policy and strategic relations for the ASBMT.

“[ASBMT] and a number of stakeholders have been very clear in our comment letters that that would not be enough and the reasons why,” Ms. Farnia said in an interview. “It’s not going to be sufficient to cover the cost of care or the product.”

The rule also fails to address the cancer centers that are exempt from the DRG payment system, Ms. Farnia said. Eleven centers are excluded from the payment system because of past legislation that excludes exclusive cancer hospitals that do not provide noncancer services. The exempt cancer centers cannot receive additional money for new or expensive drugs and therefore will not gain any financial relief from the CAR T-cell therapy payment changes in the CMS final rule.

ASH officials plan to follow up with congressional leaders to identify ways to improve future CAR T-cell therapy payments, including a potential legislative solution. An ASH spokesperson declined to elaborate on its ideal legislative remedy.

Hospital administrators and physicians will need to have difficult conversations in the upcoming year about whether treating patients with CAR T-cell therapies is worth the cost deficits, Ms. Farnia said.


“Everyone was really counting on it being a different reimbursement scenario for the upcoming fiscal year, and it is, but again, it’s that bare minimum difference,” Ms. Farnia said. “I think a number of programs are going to be taking a look at their financial experience thus far and comparing that to the reimbursement and deciding on if they [should] continue to offer it and how to do that.”

In April 2018, CMS announced payment rates for outpatient administration of the two drugs, settling on $395,380 for axicabtagene ciloleucel and $500,839 for tisagenlecleucel. The two medications have list prices of $373,000 and $475,000, respectively.

However, physicians have raised concerns that even if the drugs are first administered in the outpatient setting, inpatient care is likely to occur with CAR T-cell therapies because some patients will need to be admitted in order to be monitored for serious side effects. In such cases, all payments will become part of the inpatient stay under CMS’s 3-day payment window rule.

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Cervical cancer screening recommendations vary by age and risk

USPSTF needs to consider cost effectiveness
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Screen women for cervical cancer with basic cytology starting at age 21 years, and consider adding high-risk human papillomavirus (hrHPV) testing alone or with cytology for women aged 30 years and older, the U.S. Preventive Services Task Force recommended in an updated statement on cervical cancer screening .

The statement, accompanying evidence report, and a modeling study were published online in JAMA.

Cervical cancer deaths in the United States have declined from 2.8 deaths per 100,000 women in 2000 to 2.3 deaths per 100,000 women in 2015 because of the adoption of widespread screening, according to Susan J. Curry, PhD., of the University of Iowa, Iowa City, and her colleagues in the USPSTF (JAMA. 2018 Aug 21. doi: 10.1001/jama.2018.10897.

Based on the latest evidence and the modeling study, the USPSTF gives an A recommendation to screening women aged 21-29 years for cervical cancer every 3 years with cervical cytology alone. The task force also gives an A to screening women aged 30-65 years every 5 years with either hrHPV testing alone or in combination with cytology.

The task force recommends against screening (D recommendation) for women younger than 21 years, older than 65 years with a history of screening and low cervical cancer risk, and women who have had hysterectomies with removal of the cervix and no history of cervical cancer risk.

To update the previous recommendations issued in 2012, the task force reviewed the latest evidence and commissioned a modeling study to help determine the best screening strategies in terms of age, screening intervals, and risks vs. benefits.

In the model, researchers assessed 19 strategies for cervical cancer screening based on a hypothetical cohort of women who began screening at 21 years of age.

Overall, the different strategies were similar in effectiveness, but primary hrHPV testing and alternative cotesting were slightly more effective: Cervical cancer deaths ranged from 0.23 to 0.29 deaths per 1,000 women in strategies involving hrHPV testing or cotesting, vs. 0.30 to 0.76 deaths per 1,000 women for strategies based on the current guidelines.

In addition, switching the age of hrHPV testing from 25 years to 30 years and using a 5-year screening interval showed the most effectiveness in terms of risks vs. harms, wrote Jane K. Kim, PhD, of Harvard University, Boston, and her colleagues (JAMA. 2018 Aug 21. doi: 10.1001/jama.2017.19872). “Switching from cytology to 5-year primary hrHPV testing at age 30 years (strategy 14) was associated with a ratio of 640 colposcopies per cancer case averted; earlier switch ages required a greater number of colposcopies per cancer case averted.”

The recommendations also were supported by an evidence report including eight randomized, controlled trials of 410,556 women, five cohort studies of 402,615 women, and a meta-analysis of individual participant data including 176,464 women.

The evidence report sought to address the benefits and harms of cervical cancer screening using hrHPV screening alone as the primary screening method or paired with cytology (cotesting), compared with primary screening using cytology alone.

Overall, both hrHPV and hrHPV plus cytology were associated with higher rates of false-positives and colposcopy compared with cytology alone, “which could lead to more treatments with potential harms,” wrote Joy Melnikow, MD, of the University of California, Davis, and her colleagues (JAMA. 2018 Aug 21. doi: 10.1001/jama.2018.10400.

In addition, hrHPV testing yielded higher rates of positive cervical intraepithelial neoplasia, compared with cytology alone as initial screening.

However, further research is needed to address the impact of any cervical cancer screening strategies in populations with limited access to health care and screening, the researchers noted.

The updated USPSTF recommendations are largely in line with those issued by leading women’s health organizations including the American College of Obstetricians and Gynecologists, ASCCP, and the Society for Gynecologic Oncology, according to a joint statement.

“With a number of screening options now available, the new guidelines emphasize the importance of the patient-provider shared decision-making process to assist women in making an informed choice about which screening method is most suitable for them,” according to the statement, “However, more importantly, there needs to be a continued effort to ensure all women are adequately screened because a significant number of women in the country are not. It’s also essential for women to have access to all of the tests and that they are appropriately covered by insurance companies.

“We hope the USPSTF recommendations foster more discussions between patients and providers about cervical cancer screening, promote opportunities for patient education on the benefits and safety of HPV vaccination for cervical cancer prevention and encourage providers to offer HPV vaccines in their offices,” the statement noted.

The USPSTF research was funded by the Agency for Healthcare Research and Quality. The researchers for the modeling report were supported in part by a National Cancer Institute grant. The researchers had no relevant financial conflicts to disclose.

SOURCES: Kim J et al. JAMA. 2018 Aug 21. doi: 10.1001/jama.2017.19872; Melnikow J et al. JAMA. 2018 Aug 21. doi: 10.1001/jama.2018.10400; Curry S et al. JAMA. 2018 Aug 21. doi: 10.1001/jama.2018.10897.

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In 2016, the Society for Gynecologic Oncology (SGO) recommended screening with the newly approved hrHPV test for women aged 25 years and older, with rescreening 3 years later if the test was negative, George F. Sawaya, MD, wrote in an accompanying editorial published in JAMA Internal Medicine. The new recommendations from the U.S. Preventive Services Task Force do not endorse a single triage strategy, and do not consider costs, he said.

“Although the USPSTF sets the standard for evidence-based recommendations and acknowledges the critical value of high-quality evidence in making recommendations, it might reasonably be asked, where is the evidence of value in cervical cancer screening?” Dr. Sawaya wrote.

The updated USPSTF recommendations differ from the SGO recommendation by changing the starting age for hrHPV testing to 30 years from 25, and rescreening at 5-year intervals.

“The USPSTF recommendation that HPV testing not begin until age 30 years seems prudent,” Dr. Sawaya said, in light of the evidence report and modeling analysis of harms and benefits. He noted that the evidence reviewed by the task force showed that HPV testing and cotesting resulting in a small amount of life-years gained compared with no testing, but with the trade-off of more follow-up tests and colposcopies.

“From the perspective of society, it has been proposed that cost-effectiveness analyses be an essential part of the guideline process,” Dr. Sawaya noted. “To assist in policy decisions that many professional societies will soon face, a study that I am leading is seeking to use cost-effectiveness analyses to determine the range of reasonable options for cervical cancer screening. Such analyses may inform future screening recommendations.”

Dr. Sawaya is affiliated with the University of California, San Francisco. These comments are taken from an editorial accompanying USPSTF recommendations on cervical cancer screening (JAMA Intern Med. 2018 Aug 21. doi: 10.1001/jamainternmed.2018.4282). He disclosed serving as the principal investigator of a National Cancer Institute study on cost-effectiveness analyses to determine reasonable options for cervical cancer screening. He also served as a member of the U.S. Preventive Services Task Force from 2004 to 2008.

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In 2016, the Society for Gynecologic Oncology (SGO) recommended screening with the newly approved hrHPV test for women aged 25 years and older, with rescreening 3 years later if the test was negative, George F. Sawaya, MD, wrote in an accompanying editorial published in JAMA Internal Medicine. The new recommendations from the U.S. Preventive Services Task Force do not endorse a single triage strategy, and do not consider costs, he said.

“Although the USPSTF sets the standard for evidence-based recommendations and acknowledges the critical value of high-quality evidence in making recommendations, it might reasonably be asked, where is the evidence of value in cervical cancer screening?” Dr. Sawaya wrote.

The updated USPSTF recommendations differ from the SGO recommendation by changing the starting age for hrHPV testing to 30 years from 25, and rescreening at 5-year intervals.

“The USPSTF recommendation that HPV testing not begin until age 30 years seems prudent,” Dr. Sawaya said, in light of the evidence report and modeling analysis of harms and benefits. He noted that the evidence reviewed by the task force showed that HPV testing and cotesting resulting in a small amount of life-years gained compared with no testing, but with the trade-off of more follow-up tests and colposcopies.

“From the perspective of society, it has been proposed that cost-effectiveness analyses be an essential part of the guideline process,” Dr. Sawaya noted. “To assist in policy decisions that many professional societies will soon face, a study that I am leading is seeking to use cost-effectiveness analyses to determine the range of reasonable options for cervical cancer screening. Such analyses may inform future screening recommendations.”

Dr. Sawaya is affiliated with the University of California, San Francisco. These comments are taken from an editorial accompanying USPSTF recommendations on cervical cancer screening (JAMA Intern Med. 2018 Aug 21. doi: 10.1001/jamainternmed.2018.4282). He disclosed serving as the principal investigator of a National Cancer Institute study on cost-effectiveness analyses to determine reasonable options for cervical cancer screening. He also served as a member of the U.S. Preventive Services Task Force from 2004 to 2008.

Body

 

In 2016, the Society for Gynecologic Oncology (SGO) recommended screening with the newly approved hrHPV test for women aged 25 years and older, with rescreening 3 years later if the test was negative, George F. Sawaya, MD, wrote in an accompanying editorial published in JAMA Internal Medicine. The new recommendations from the U.S. Preventive Services Task Force do not endorse a single triage strategy, and do not consider costs, he said.

“Although the USPSTF sets the standard for evidence-based recommendations and acknowledges the critical value of high-quality evidence in making recommendations, it might reasonably be asked, where is the evidence of value in cervical cancer screening?” Dr. Sawaya wrote.

The updated USPSTF recommendations differ from the SGO recommendation by changing the starting age for hrHPV testing to 30 years from 25, and rescreening at 5-year intervals.

“The USPSTF recommendation that HPV testing not begin until age 30 years seems prudent,” Dr. Sawaya said, in light of the evidence report and modeling analysis of harms and benefits. He noted that the evidence reviewed by the task force showed that HPV testing and cotesting resulting in a small amount of life-years gained compared with no testing, but with the trade-off of more follow-up tests and colposcopies.

“From the perspective of society, it has been proposed that cost-effectiveness analyses be an essential part of the guideline process,” Dr. Sawaya noted. “To assist in policy decisions that many professional societies will soon face, a study that I am leading is seeking to use cost-effectiveness analyses to determine the range of reasonable options for cervical cancer screening. Such analyses may inform future screening recommendations.”

Dr. Sawaya is affiliated with the University of California, San Francisco. These comments are taken from an editorial accompanying USPSTF recommendations on cervical cancer screening (JAMA Intern Med. 2018 Aug 21. doi: 10.1001/jamainternmed.2018.4282). He disclosed serving as the principal investigator of a National Cancer Institute study on cost-effectiveness analyses to determine reasonable options for cervical cancer screening. He also served as a member of the U.S. Preventive Services Task Force from 2004 to 2008.

Title
USPSTF needs to consider cost effectiveness
USPSTF needs to consider cost effectiveness

 

Screen women for cervical cancer with basic cytology starting at age 21 years, and consider adding high-risk human papillomavirus (hrHPV) testing alone or with cytology for women aged 30 years and older, the U.S. Preventive Services Task Force recommended in an updated statement on cervical cancer screening .

The statement, accompanying evidence report, and a modeling study were published online in JAMA.

Cervical cancer deaths in the United States have declined from 2.8 deaths per 100,000 women in 2000 to 2.3 deaths per 100,000 women in 2015 because of the adoption of widespread screening, according to Susan J. Curry, PhD., of the University of Iowa, Iowa City, and her colleagues in the USPSTF (JAMA. 2018 Aug 21. doi: 10.1001/jama.2018.10897.

Based on the latest evidence and the modeling study, the USPSTF gives an A recommendation to screening women aged 21-29 years for cervical cancer every 3 years with cervical cytology alone. The task force also gives an A to screening women aged 30-65 years every 5 years with either hrHPV testing alone or in combination with cytology.

The task force recommends against screening (D recommendation) for women younger than 21 years, older than 65 years with a history of screening and low cervical cancer risk, and women who have had hysterectomies with removal of the cervix and no history of cervical cancer risk.

To update the previous recommendations issued in 2012, the task force reviewed the latest evidence and commissioned a modeling study to help determine the best screening strategies in terms of age, screening intervals, and risks vs. benefits.

In the model, researchers assessed 19 strategies for cervical cancer screening based on a hypothetical cohort of women who began screening at 21 years of age.

Overall, the different strategies were similar in effectiveness, but primary hrHPV testing and alternative cotesting were slightly more effective: Cervical cancer deaths ranged from 0.23 to 0.29 deaths per 1,000 women in strategies involving hrHPV testing or cotesting, vs. 0.30 to 0.76 deaths per 1,000 women for strategies based on the current guidelines.

In addition, switching the age of hrHPV testing from 25 years to 30 years and using a 5-year screening interval showed the most effectiveness in terms of risks vs. harms, wrote Jane K. Kim, PhD, of Harvard University, Boston, and her colleagues (JAMA. 2018 Aug 21. doi: 10.1001/jama.2017.19872). “Switching from cytology to 5-year primary hrHPV testing at age 30 years (strategy 14) was associated with a ratio of 640 colposcopies per cancer case averted; earlier switch ages required a greater number of colposcopies per cancer case averted.”

The recommendations also were supported by an evidence report including eight randomized, controlled trials of 410,556 women, five cohort studies of 402,615 women, and a meta-analysis of individual participant data including 176,464 women.

The evidence report sought to address the benefits and harms of cervical cancer screening using hrHPV screening alone as the primary screening method or paired with cytology (cotesting), compared with primary screening using cytology alone.

Overall, both hrHPV and hrHPV plus cytology were associated with higher rates of false-positives and colposcopy compared with cytology alone, “which could lead to more treatments with potential harms,” wrote Joy Melnikow, MD, of the University of California, Davis, and her colleagues (JAMA. 2018 Aug 21. doi: 10.1001/jama.2018.10400.

In addition, hrHPV testing yielded higher rates of positive cervical intraepithelial neoplasia, compared with cytology alone as initial screening.

However, further research is needed to address the impact of any cervical cancer screening strategies in populations with limited access to health care and screening, the researchers noted.

The updated USPSTF recommendations are largely in line with those issued by leading women’s health organizations including the American College of Obstetricians and Gynecologists, ASCCP, and the Society for Gynecologic Oncology, according to a joint statement.

“With a number of screening options now available, the new guidelines emphasize the importance of the patient-provider shared decision-making process to assist women in making an informed choice about which screening method is most suitable for them,” according to the statement, “However, more importantly, there needs to be a continued effort to ensure all women are adequately screened because a significant number of women in the country are not. It’s also essential for women to have access to all of the tests and that they are appropriately covered by insurance companies.

“We hope the USPSTF recommendations foster more discussions between patients and providers about cervical cancer screening, promote opportunities for patient education on the benefits and safety of HPV vaccination for cervical cancer prevention and encourage providers to offer HPV vaccines in their offices,” the statement noted.

The USPSTF research was funded by the Agency for Healthcare Research and Quality. The researchers for the modeling report were supported in part by a National Cancer Institute grant. The researchers had no relevant financial conflicts to disclose.

SOURCES: Kim J et al. JAMA. 2018 Aug 21. doi: 10.1001/jama.2017.19872; Melnikow J et al. JAMA. 2018 Aug 21. doi: 10.1001/jama.2018.10400; Curry S et al. JAMA. 2018 Aug 21. doi: 10.1001/jama.2018.10897.

 

Screen women for cervical cancer with basic cytology starting at age 21 years, and consider adding high-risk human papillomavirus (hrHPV) testing alone or with cytology for women aged 30 years and older, the U.S. Preventive Services Task Force recommended in an updated statement on cervical cancer screening .

The statement, accompanying evidence report, and a modeling study were published online in JAMA.

Cervical cancer deaths in the United States have declined from 2.8 deaths per 100,000 women in 2000 to 2.3 deaths per 100,000 women in 2015 because of the adoption of widespread screening, according to Susan J. Curry, PhD., of the University of Iowa, Iowa City, and her colleagues in the USPSTF (JAMA. 2018 Aug 21. doi: 10.1001/jama.2018.10897.

Based on the latest evidence and the modeling study, the USPSTF gives an A recommendation to screening women aged 21-29 years for cervical cancer every 3 years with cervical cytology alone. The task force also gives an A to screening women aged 30-65 years every 5 years with either hrHPV testing alone or in combination with cytology.

The task force recommends against screening (D recommendation) for women younger than 21 years, older than 65 years with a history of screening and low cervical cancer risk, and women who have had hysterectomies with removal of the cervix and no history of cervical cancer risk.

To update the previous recommendations issued in 2012, the task force reviewed the latest evidence and commissioned a modeling study to help determine the best screening strategies in terms of age, screening intervals, and risks vs. benefits.

In the model, researchers assessed 19 strategies for cervical cancer screening based on a hypothetical cohort of women who began screening at 21 years of age.

Overall, the different strategies were similar in effectiveness, but primary hrHPV testing and alternative cotesting were slightly more effective: Cervical cancer deaths ranged from 0.23 to 0.29 deaths per 1,000 women in strategies involving hrHPV testing or cotesting, vs. 0.30 to 0.76 deaths per 1,000 women for strategies based on the current guidelines.

In addition, switching the age of hrHPV testing from 25 years to 30 years and using a 5-year screening interval showed the most effectiveness in terms of risks vs. harms, wrote Jane K. Kim, PhD, of Harvard University, Boston, and her colleagues (JAMA. 2018 Aug 21. doi: 10.1001/jama.2017.19872). “Switching from cytology to 5-year primary hrHPV testing at age 30 years (strategy 14) was associated with a ratio of 640 colposcopies per cancer case averted; earlier switch ages required a greater number of colposcopies per cancer case averted.”

The recommendations also were supported by an evidence report including eight randomized, controlled trials of 410,556 women, five cohort studies of 402,615 women, and a meta-analysis of individual participant data including 176,464 women.

The evidence report sought to address the benefits and harms of cervical cancer screening using hrHPV screening alone as the primary screening method or paired with cytology (cotesting), compared with primary screening using cytology alone.

Overall, both hrHPV and hrHPV plus cytology were associated with higher rates of false-positives and colposcopy compared with cytology alone, “which could lead to more treatments with potential harms,” wrote Joy Melnikow, MD, of the University of California, Davis, and her colleagues (JAMA. 2018 Aug 21. doi: 10.1001/jama.2018.10400.

In addition, hrHPV testing yielded higher rates of positive cervical intraepithelial neoplasia, compared with cytology alone as initial screening.

However, further research is needed to address the impact of any cervical cancer screening strategies in populations with limited access to health care and screening, the researchers noted.

The updated USPSTF recommendations are largely in line with those issued by leading women’s health organizations including the American College of Obstetricians and Gynecologists, ASCCP, and the Society for Gynecologic Oncology, according to a joint statement.

“With a number of screening options now available, the new guidelines emphasize the importance of the patient-provider shared decision-making process to assist women in making an informed choice about which screening method is most suitable for them,” according to the statement, “However, more importantly, there needs to be a continued effort to ensure all women are adequately screened because a significant number of women in the country are not. It’s also essential for women to have access to all of the tests and that they are appropriately covered by insurance companies.

“We hope the USPSTF recommendations foster more discussions between patients and providers about cervical cancer screening, promote opportunities for patient education on the benefits and safety of HPV vaccination for cervical cancer prevention and encourage providers to offer HPV vaccines in their offices,” the statement noted.

The USPSTF research was funded by the Agency for Healthcare Research and Quality. The researchers for the modeling report were supported in part by a National Cancer Institute grant. The researchers had no relevant financial conflicts to disclose.

SOURCES: Kim J et al. JAMA. 2018 Aug 21. doi: 10.1001/jama.2017.19872; Melnikow J et al. JAMA. 2018 Aug 21. doi: 10.1001/jama.2018.10400; Curry S et al. JAMA. 2018 Aug 21. doi: 10.1001/jama.2018.10897.

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FDA grants orphan designation to DHODH inhibitor for AML

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The Food and Drug Administration has granted orphan drug designation to ASLAN003 as a treatment for acute myeloid leukemia (AML).

ASLAN003 is a small molecule inhibitor of the human dihydroorotate dehydrogenase (DHODH) enzyme. This second-generation DHODH inhibitor is being developed by Aslan Pharmaceuticals. The company is currently conducting a phase 2 trial (NCT03451084) of ASLAN003 in patients with newly diagnosed or relapsed/refractory AML. Aslan expects to report interim data from this trial in the second half of 2018.

Aslan has already completed a phase 1 trial (NCT01992367) of ASLAN003 in healthy volunteers. The results suggested that ASLAN003 has an “excellent” pharmacokinetic profile, according to Aslan, and the drug was considered well tolerated in the volunteers.

ASLAN003 has also demonstrated “potent” inhibition of DHODH, according to the drug sponsor. In fact, the company said the binding affinity of ASLAN003 to DHODH has proven to be up to two orders of magnitude stronger than first-generation DHODH inhibitors, such as leflunomide and teriflunomide, but it has less toxicity.

In addition, ASLAN003 has been shown to differentiate blast cells into granulocytes in AML cell lines that do not respond to all-trans retinoic acid. These results were published in Cell in 2016.

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The Food and Drug Administration has granted orphan drug designation to ASLAN003 as a treatment for acute myeloid leukemia (AML).

ASLAN003 is a small molecule inhibitor of the human dihydroorotate dehydrogenase (DHODH) enzyme. This second-generation DHODH inhibitor is being developed by Aslan Pharmaceuticals. The company is currently conducting a phase 2 trial (NCT03451084) of ASLAN003 in patients with newly diagnosed or relapsed/refractory AML. Aslan expects to report interim data from this trial in the second half of 2018.

Aslan has already completed a phase 1 trial (NCT01992367) of ASLAN003 in healthy volunteers. The results suggested that ASLAN003 has an “excellent” pharmacokinetic profile, according to Aslan, and the drug was considered well tolerated in the volunteers.

ASLAN003 has also demonstrated “potent” inhibition of DHODH, according to the drug sponsor. In fact, the company said the binding affinity of ASLAN003 to DHODH has proven to be up to two orders of magnitude stronger than first-generation DHODH inhibitors, such as leflunomide and teriflunomide, but it has less toxicity.

In addition, ASLAN003 has been shown to differentiate blast cells into granulocytes in AML cell lines that do not respond to all-trans retinoic acid. These results were published in Cell in 2016.

 

The Food and Drug Administration has granted orphan drug designation to ASLAN003 as a treatment for acute myeloid leukemia (AML).

ASLAN003 is a small molecule inhibitor of the human dihydroorotate dehydrogenase (DHODH) enzyme. This second-generation DHODH inhibitor is being developed by Aslan Pharmaceuticals. The company is currently conducting a phase 2 trial (NCT03451084) of ASLAN003 in patients with newly diagnosed or relapsed/refractory AML. Aslan expects to report interim data from this trial in the second half of 2018.

Aslan has already completed a phase 1 trial (NCT01992367) of ASLAN003 in healthy volunteers. The results suggested that ASLAN003 has an “excellent” pharmacokinetic profile, according to Aslan, and the drug was considered well tolerated in the volunteers.

ASLAN003 has also demonstrated “potent” inhibition of DHODH, according to the drug sponsor. In fact, the company said the binding affinity of ASLAN003 to DHODH has proven to be up to two orders of magnitude stronger than first-generation DHODH inhibitors, such as leflunomide and teriflunomide, but it has less toxicity.

In addition, ASLAN003 has been shown to differentiate blast cells into granulocytes in AML cell lines that do not respond to all-trans retinoic acid. These results were published in Cell in 2016.

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A systems-based charter on physician well-being

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Don’t blame the burned-out clinician

 

“You can teach a canary in a coal mine to meditate, but it is still going to die.”

I have seen the canary sentiment above – used as a metaphor for health care and burnout – pop up a few times on Twitter, attributed to a few different thoughtful doctors, including Jenny Ramsey, MD, of the Cleveland Clinic (at Hospital Medicine 2018); Lucy Kalanithi, MD, a clinical assistant professor of medicine at Stanford (Calif.) University and widow of Paul Kalanithi, MD, of “When Breath Becomes Air” fame; and Stuart Slavin, MD, associate dean for curriculum and a professor of pediatrics at Saint Louis University.

Dr. Christopher Moriates

To be honest, I am rather burned out on reading about physician burnout at this point. Nevertheless, I love the canary idea; it is such a perfect visual of the current problem facing physicians.

I was thinking about the meditating canary when I read the new “Charter on Physician Well-Being,” published in JAMA and already endorsed by most major medical organizations/acronyms, including SHM, ACP, SGIM, AMA, AAMC, AAIM, ABIM, ACCME, APA, and the IHI. This physician well-being charter was created by the Collaborative for Healing and Renewal in Medicine, a group that includes leading medical centers and organizations.

What makes this different from previous attempts at addressing burnout? The charter takes a systems-based approach to physician well-being. Aha, of course! As the patient safety movement realized more than 2 decades ago, real progress would only be made when we stopped focusing our attention, blame, and interventions on individuals and instead looked at systems; now, the physician well-being movement has officially made the same bold proclamation.

It is not the fault of the burned-out physician who apparently just needs to be hammered over the head with better coping skills – just as the majority of medical errors would not be fixed by continuing to tell physicians that they screwed up and should figure out how not to do that again!

We need to make real changes to the system. For example, one of the charter’s authors, Colin P. West, MD, PhD, highlighted why it is important that organizations commit to optimizing highly functioning interprofessional teams: “Can you imagine @KingJames [LeBron James] or @Oprah applying their unique skills AND personally seating the crowd, collecting stats, assessing satisfaction, etc.? So why do we?”

The authors also call for organizations to commit to reducing time spent on documentation and administration. Hallelujah!

Now the question is whether this charter will actually have any teeth or whether it will have the same fate as our canary, slowly fading away, never to be heard from again?

Read the full post at hospitalleader.org.

Dr. Moriates is the assistant dean for health care value and an associate professor of internal medicine at the University of Texas, Austin.
 

Also in The Hospital Leader

What’s a Cost, Charge, and Price?” by Brad Flansbaum, DO, MPH, MHM

There Is a ‘You’ in Team,” by Tracy Cardin, ACNP-BC, SFHM

‘Harper’s Index’ of Hospital Medicine 2018,” by Jordan Messler, MD, SFHM

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Don’t blame the burned-out clinician

Don’t blame the burned-out clinician

 

“You can teach a canary in a coal mine to meditate, but it is still going to die.”

I have seen the canary sentiment above – used as a metaphor for health care and burnout – pop up a few times on Twitter, attributed to a few different thoughtful doctors, including Jenny Ramsey, MD, of the Cleveland Clinic (at Hospital Medicine 2018); Lucy Kalanithi, MD, a clinical assistant professor of medicine at Stanford (Calif.) University and widow of Paul Kalanithi, MD, of “When Breath Becomes Air” fame; and Stuart Slavin, MD, associate dean for curriculum and a professor of pediatrics at Saint Louis University.

Dr. Christopher Moriates

To be honest, I am rather burned out on reading about physician burnout at this point. Nevertheless, I love the canary idea; it is such a perfect visual of the current problem facing physicians.

I was thinking about the meditating canary when I read the new “Charter on Physician Well-Being,” published in JAMA and already endorsed by most major medical organizations/acronyms, including SHM, ACP, SGIM, AMA, AAMC, AAIM, ABIM, ACCME, APA, and the IHI. This physician well-being charter was created by the Collaborative for Healing and Renewal in Medicine, a group that includes leading medical centers and organizations.

What makes this different from previous attempts at addressing burnout? The charter takes a systems-based approach to physician well-being. Aha, of course! As the patient safety movement realized more than 2 decades ago, real progress would only be made when we stopped focusing our attention, blame, and interventions on individuals and instead looked at systems; now, the physician well-being movement has officially made the same bold proclamation.

It is not the fault of the burned-out physician who apparently just needs to be hammered over the head with better coping skills – just as the majority of medical errors would not be fixed by continuing to tell physicians that they screwed up and should figure out how not to do that again!

We need to make real changes to the system. For example, one of the charter’s authors, Colin P. West, MD, PhD, highlighted why it is important that organizations commit to optimizing highly functioning interprofessional teams: “Can you imagine @KingJames [LeBron James] or @Oprah applying their unique skills AND personally seating the crowd, collecting stats, assessing satisfaction, etc.? So why do we?”

The authors also call for organizations to commit to reducing time spent on documentation and administration. Hallelujah!

Now the question is whether this charter will actually have any teeth or whether it will have the same fate as our canary, slowly fading away, never to be heard from again?

Read the full post at hospitalleader.org.

Dr. Moriates is the assistant dean for health care value and an associate professor of internal medicine at the University of Texas, Austin.
 

Also in The Hospital Leader

What’s a Cost, Charge, and Price?” by Brad Flansbaum, DO, MPH, MHM

There Is a ‘You’ in Team,” by Tracy Cardin, ACNP-BC, SFHM

‘Harper’s Index’ of Hospital Medicine 2018,” by Jordan Messler, MD, SFHM

 

“You can teach a canary in a coal mine to meditate, but it is still going to die.”

I have seen the canary sentiment above – used as a metaphor for health care and burnout – pop up a few times on Twitter, attributed to a few different thoughtful doctors, including Jenny Ramsey, MD, of the Cleveland Clinic (at Hospital Medicine 2018); Lucy Kalanithi, MD, a clinical assistant professor of medicine at Stanford (Calif.) University and widow of Paul Kalanithi, MD, of “When Breath Becomes Air” fame; and Stuart Slavin, MD, associate dean for curriculum and a professor of pediatrics at Saint Louis University.

Dr. Christopher Moriates

To be honest, I am rather burned out on reading about physician burnout at this point. Nevertheless, I love the canary idea; it is such a perfect visual of the current problem facing physicians.

I was thinking about the meditating canary when I read the new “Charter on Physician Well-Being,” published in JAMA and already endorsed by most major medical organizations/acronyms, including SHM, ACP, SGIM, AMA, AAMC, AAIM, ABIM, ACCME, APA, and the IHI. This physician well-being charter was created by the Collaborative for Healing and Renewal in Medicine, a group that includes leading medical centers and organizations.

What makes this different from previous attempts at addressing burnout? The charter takes a systems-based approach to physician well-being. Aha, of course! As the patient safety movement realized more than 2 decades ago, real progress would only be made when we stopped focusing our attention, blame, and interventions on individuals and instead looked at systems; now, the physician well-being movement has officially made the same bold proclamation.

It is not the fault of the burned-out physician who apparently just needs to be hammered over the head with better coping skills – just as the majority of medical errors would not be fixed by continuing to tell physicians that they screwed up and should figure out how not to do that again!

We need to make real changes to the system. For example, one of the charter’s authors, Colin P. West, MD, PhD, highlighted why it is important that organizations commit to optimizing highly functioning interprofessional teams: “Can you imagine @KingJames [LeBron James] or @Oprah applying their unique skills AND personally seating the crowd, collecting stats, assessing satisfaction, etc.? So why do we?”

The authors also call for organizations to commit to reducing time spent on documentation and administration. Hallelujah!

Now the question is whether this charter will actually have any teeth or whether it will have the same fate as our canary, slowly fading away, never to be heard from again?

Read the full post at hospitalleader.org.

Dr. Moriates is the assistant dean for health care value and an associate professor of internal medicine at the University of Texas, Austin.
 

Also in The Hospital Leader

What’s a Cost, Charge, and Price?” by Brad Flansbaum, DO, MPH, MHM

There Is a ‘You’ in Team,” by Tracy Cardin, ACNP-BC, SFHM

‘Harper’s Index’ of Hospital Medicine 2018,” by Jordan Messler, MD, SFHM

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Telangiectatic Patch on the Neck

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The Diagnosis: Unilateral Nevoid Telangiectasia

Unilateral nevoid telangiectasia (UNT) is an uncommon, or perhaps underreported, cutaneous condition involving telangiectatic patches in a unilateral dermatomal or blaschkoid pattern.1 The condition has been described as either congenital or acquired. Congenital UNT is thought to be a result of somatic mosaicism, whereby a mutation during embryogenesis leads to a distinct population of cells expressing the vascular malformation.1 Congenital UNT has been associated with Becker nevus, which also is thought to be a result of somatic mosaicism, further providing evidence for this theory, though it is unclear whether this finding is incidental.2 The acquired form often is associated with fluctuation of hormones, such as in pregnancy or with oral contraceptive initiation, as well as with hepatic disease as seen in our patient. However, there are many cases of acquired UNT with no implicated underlying disease, alcohol abuse, or hormonal changes, which calls into question if UNT is definitively an estrogen-related condition.3 One study demonstrated an increased level of estrogen and progesterone receptors in affected skin, which may have led to expression of the cutaneous changes at that site.4 More research is needed to elucidate this point, as other studies have not reproduced similar findings.

Congenital UNT occurs more commonly in males, whereas the acquired variant is seen more frequently in females. The third and fourth cervical dermatomes most often are involved.5 Most lesions persist without spontaneous resolution. Treatment options are limited and include pulsed dye laser treatment and makeup application to cover the telangiectatic patches. The main side effect seen with pulsed dye laser treatment is reversible pigmentary changes, with 1 report of textural skin change.6

A biopsy was deemed unnecessary for the clinical diagnosis in our patient because there was a clear explanation for the physical examination findings due to long-standing underlying liver disease. When biopsied, UNT characteristically demonstrates dilated dermal capillaries.5 Our patient elected not to pursue laser therapy but expressed interest in using makeup to camouflage the lesion.

The differential diagnosis includes acquired nevus flammeus, which typically is present on the face and often appears following mechanical or thermal trauma. Angioma serpiginosum most often occurs on the buttocks and legs as small red papules or puncta coalescing into a serpiginous linear arrangement. It often appears in childhood. Angiosarcoma is an aggressive malignancy that often occurs on the head and neck in elderly patients. It is associated with areas of long-standing lymphedema and often appears as a bruiselike lesion. Rosacea typically is not fixed in its clinical appearance and presents as transitory flushing of the head and neck with or without a history of acneform eruptions on the face. It typically is not unilateral.

References
  1. Wilkin JK. Unilateral dermatomal superficial telangiectasia. Arch Dermatol. 1984;120:579-580.  
  2. Karakaş M, Durdu M, Sönmezoğlu S, et al. Unilateral nevoid telangiectasia. J Dermatol. 2004;31:109-112.
  3. Taskapan O, Harmanyeri Y, Sener O, et al. Acquired unilateral nevoid telangiectasia syndrome. Acta Derm Venereol. 1997;77:62-63.
  4. Uhlin SR, McCarty KS Jr. Unilateral nevoid telangiectatic syndrome: the role of estrogen and progesterone receptors. Arch Dermatol. 1983;119:226-228.
  5. Derrow AE, Adams BB, Timani S, et al. Acquired unilateral nevoid telangiectasia in a 51-year-old female. Int J Dermatol. 2008;47:1331-1333.
  6. Sharma VK, Khandpur S. Unilateral nevoid telangiectasia--response to pulsed dye laser. Int J Dermatol. 2006;45:960-964.
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From the Department of Dermatology, University of Kansas Medical Center, Kansas City. 

The authors report no conflict of interest.

Correspondence: Aly Barland, MD, 1850 W Mountain View Ave, Longmont, CO 80501 ([email protected]).

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From the Department of Dermatology, University of Kansas Medical Center, Kansas City. 

The authors report no conflict of interest.

Correspondence: Aly Barland, MD, 1850 W Mountain View Ave, Longmont, CO 80501 ([email protected]).

Author and Disclosure Information

From the Department of Dermatology, University of Kansas Medical Center, Kansas City. 

The authors report no conflict of interest.

Correspondence: Aly Barland, MD, 1850 W Mountain View Ave, Longmont, CO 80501 ([email protected]).

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The Diagnosis: Unilateral Nevoid Telangiectasia

Unilateral nevoid telangiectasia (UNT) is an uncommon, or perhaps underreported, cutaneous condition involving telangiectatic patches in a unilateral dermatomal or blaschkoid pattern.1 The condition has been described as either congenital or acquired. Congenital UNT is thought to be a result of somatic mosaicism, whereby a mutation during embryogenesis leads to a distinct population of cells expressing the vascular malformation.1 Congenital UNT has been associated with Becker nevus, which also is thought to be a result of somatic mosaicism, further providing evidence for this theory, though it is unclear whether this finding is incidental.2 The acquired form often is associated with fluctuation of hormones, such as in pregnancy or with oral contraceptive initiation, as well as with hepatic disease as seen in our patient. However, there are many cases of acquired UNT with no implicated underlying disease, alcohol abuse, or hormonal changes, which calls into question if UNT is definitively an estrogen-related condition.3 One study demonstrated an increased level of estrogen and progesterone receptors in affected skin, which may have led to expression of the cutaneous changes at that site.4 More research is needed to elucidate this point, as other studies have not reproduced similar findings.

Congenital UNT occurs more commonly in males, whereas the acquired variant is seen more frequently in females. The third and fourth cervical dermatomes most often are involved.5 Most lesions persist without spontaneous resolution. Treatment options are limited and include pulsed dye laser treatment and makeup application to cover the telangiectatic patches. The main side effect seen with pulsed dye laser treatment is reversible pigmentary changes, with 1 report of textural skin change.6

A biopsy was deemed unnecessary for the clinical diagnosis in our patient because there was a clear explanation for the physical examination findings due to long-standing underlying liver disease. When biopsied, UNT characteristically demonstrates dilated dermal capillaries.5 Our patient elected not to pursue laser therapy but expressed interest in using makeup to camouflage the lesion.

The differential diagnosis includes acquired nevus flammeus, which typically is present on the face and often appears following mechanical or thermal trauma. Angioma serpiginosum most often occurs on the buttocks and legs as small red papules or puncta coalescing into a serpiginous linear arrangement. It often appears in childhood. Angiosarcoma is an aggressive malignancy that often occurs on the head and neck in elderly patients. It is associated with areas of long-standing lymphedema and often appears as a bruiselike lesion. Rosacea typically is not fixed in its clinical appearance and presents as transitory flushing of the head and neck with or without a history of acneform eruptions on the face. It typically is not unilateral.

The Diagnosis: Unilateral Nevoid Telangiectasia

Unilateral nevoid telangiectasia (UNT) is an uncommon, or perhaps underreported, cutaneous condition involving telangiectatic patches in a unilateral dermatomal or blaschkoid pattern.1 The condition has been described as either congenital or acquired. Congenital UNT is thought to be a result of somatic mosaicism, whereby a mutation during embryogenesis leads to a distinct population of cells expressing the vascular malformation.1 Congenital UNT has been associated with Becker nevus, which also is thought to be a result of somatic mosaicism, further providing evidence for this theory, though it is unclear whether this finding is incidental.2 The acquired form often is associated with fluctuation of hormones, such as in pregnancy or with oral contraceptive initiation, as well as with hepatic disease as seen in our patient. However, there are many cases of acquired UNT with no implicated underlying disease, alcohol abuse, or hormonal changes, which calls into question if UNT is definitively an estrogen-related condition.3 One study demonstrated an increased level of estrogen and progesterone receptors in affected skin, which may have led to expression of the cutaneous changes at that site.4 More research is needed to elucidate this point, as other studies have not reproduced similar findings.

Congenital UNT occurs more commonly in males, whereas the acquired variant is seen more frequently in females. The third and fourth cervical dermatomes most often are involved.5 Most lesions persist without spontaneous resolution. Treatment options are limited and include pulsed dye laser treatment and makeup application to cover the telangiectatic patches. The main side effect seen with pulsed dye laser treatment is reversible pigmentary changes, with 1 report of textural skin change.6

A biopsy was deemed unnecessary for the clinical diagnosis in our patient because there was a clear explanation for the physical examination findings due to long-standing underlying liver disease. When biopsied, UNT characteristically demonstrates dilated dermal capillaries.5 Our patient elected not to pursue laser therapy but expressed interest in using makeup to camouflage the lesion.

The differential diagnosis includes acquired nevus flammeus, which typically is present on the face and often appears following mechanical or thermal trauma. Angioma serpiginosum most often occurs on the buttocks and legs as small red papules or puncta coalescing into a serpiginous linear arrangement. It often appears in childhood. Angiosarcoma is an aggressive malignancy that often occurs on the head and neck in elderly patients. It is associated with areas of long-standing lymphedema and often appears as a bruiselike lesion. Rosacea typically is not fixed in its clinical appearance and presents as transitory flushing of the head and neck with or without a history of acneform eruptions on the face. It typically is not unilateral.

References
  1. Wilkin JK. Unilateral dermatomal superficial telangiectasia. Arch Dermatol. 1984;120:579-580.  
  2. Karakaş M, Durdu M, Sönmezoğlu S, et al. Unilateral nevoid telangiectasia. J Dermatol. 2004;31:109-112.
  3. Taskapan O, Harmanyeri Y, Sener O, et al. Acquired unilateral nevoid telangiectasia syndrome. Acta Derm Venereol. 1997;77:62-63.
  4. Uhlin SR, McCarty KS Jr. Unilateral nevoid telangiectatic syndrome: the role of estrogen and progesterone receptors. Arch Dermatol. 1983;119:226-228.
  5. Derrow AE, Adams BB, Timani S, et al. Acquired unilateral nevoid telangiectasia in a 51-year-old female. Int J Dermatol. 2008;47:1331-1333.
  6. Sharma VK, Khandpur S. Unilateral nevoid telangiectasia--response to pulsed dye laser. Int J Dermatol. 2006;45:960-964.
References
  1. Wilkin JK. Unilateral dermatomal superficial telangiectasia. Arch Dermatol. 1984;120:579-580.  
  2. Karakaş M, Durdu M, Sönmezoğlu S, et al. Unilateral nevoid telangiectasia. J Dermatol. 2004;31:109-112.
  3. Taskapan O, Harmanyeri Y, Sener O, et al. Acquired unilateral nevoid telangiectasia syndrome. Acta Derm Venereol. 1997;77:62-63.
  4. Uhlin SR, McCarty KS Jr. Unilateral nevoid telangiectatic syndrome: the role of estrogen and progesterone receptors. Arch Dermatol. 1983;119:226-228.
  5. Derrow AE, Adams BB, Timani S, et al. Acquired unilateral nevoid telangiectasia in a 51-year-old female. Int J Dermatol. 2008;47:1331-1333.
  6. Sharma VK, Khandpur S. Unilateral nevoid telangiectasia--response to pulsed dye laser. Int J Dermatol. 2006;45:960-964.
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A 55-year-old woman presented to our clinic for a total-body skin examination and was noted to have a completely blanchable telangiectatic patch on the right side of the neck extending down onto the chest and breast. The patient reported that it had been present for 15 years and had slowly expanded in size. The lesion was asymptomatic. Pertinent medical history included cryptogenic cirrhosis of the liver, and she was undergoing a workup for a liver transplant.

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Some PE patients don’t require hospitalization

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A new study suggests that certain patients with acute pulmonary embolism (PE) may be better off receiving outpatient treatment.

Researchers tested outpatient anticoagulant therapy in 200 patients with PE with a low mortality risk. At 90 days of follow-up, there were no deaths or recurrences of venous thromboembolism (VTE), but one patient experienced major bleeding after a traumatic injury.

A majority of patients said they were satisfied with outpatient care.

Joseph R. Bledsoe, MD, of Intermountain Medical Center in Salt Lake City, and his colleagues reported these results in Chest.

The researchers tracked patients who were treated for acute PE in five Intermountain Healthcare emergency departments (EDs) from 2013 to 2016. The patients had to have a low mortality risk according to the Pulmonary Embolism Severity Index (score less than 86), echocardiography (no signs of right heart strain), and whole-leg compression ultrasound. Patients could not have deep vein thrombosis proximal to the popliteal vein, hypoxia, hypotension, hepatic failure, or renal failure. They had to be eligible for therapeutic anticoagulation and could not have any condition requiring hospitalization.

With these criteria, the researchers selected 200 patients. They were observed in the ED or hospital for 12-24 hours and then discharged with anticoagulant therapy. Patients received rivaroxaban (n = 149), enoxaparin transitioned to warfarin (n = 26), apixaban (n = 24), or enoxaparin alone (n = 1).

Results

The study’s primary outcome was the 90-day composite rate of all-cause mortality, recurrent symptomatic VTE, and major bleeding. There were no deaths and no cases of recurrent VTE, but one patient did experience major bleeding at day 61 because of a traumatic thigh injury.

Within 7 days of study enrollment, there were 19 patients (9.5%) who returned to the ED and 2 patients (1%) who were admitted to the hospital. One patient with pulmonary infarct was admitted for pain control (day 2); the other was admitted for an elective coronary intervention (day 7) because of a positive cardiac stress test.

Within 30 days, 32 patients (16%) returned to the ED, and 5 (3%) were admitted to the hospital for events unrelated to their PE.

The study also showed that patients were largely satisfied with outpatient care. Of the 146 patients who completed a satisfaction survey at 90 days, 89% said they would choose outpatient management if they had another PE in the future.

“We found a large subset of patients with blood clots who’d do well at home; in fact, who probably did better at home,” Dr. Bledsoe said. “When patients are sent home versus staying in the hospital, they’re at lower risk of getting another infection. It’s a lot less expensive, too.”

Currently, the standard of care in the United States for acute PE is hospitalization for all patients. That’s recommended, in part, because their overall mortality rate is 17%. However, the lower mortality rate among some appropriately risk-stratified patients suggests that at-home care, which has become the norm in some European countries, leads to better outcomes for those patients overall and less chance of a hospital-introduced infection, according to Dr. Bledsoe. “Our findings show that if you appropriately risk-stratify patients, there are a lot of people with blood clots who are safe to go home.”

He added that similar research should be conducted outside of the Intermountain Healthcare system to confirm the results of this study and that a larger group of patients should be studied.

The investigators reported no conflicts related to this study.

SOURCE: Bledsoe JR et al. Chest. 2018 Aug;154(2):249-56.

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A new study suggests that certain patients with acute pulmonary embolism (PE) may be better off receiving outpatient treatment.

Researchers tested outpatient anticoagulant therapy in 200 patients with PE with a low mortality risk. At 90 days of follow-up, there were no deaths or recurrences of venous thromboembolism (VTE), but one patient experienced major bleeding after a traumatic injury.

A majority of patients said they were satisfied with outpatient care.

Joseph R. Bledsoe, MD, of Intermountain Medical Center in Salt Lake City, and his colleagues reported these results in Chest.

The researchers tracked patients who were treated for acute PE in five Intermountain Healthcare emergency departments (EDs) from 2013 to 2016. The patients had to have a low mortality risk according to the Pulmonary Embolism Severity Index (score less than 86), echocardiography (no signs of right heart strain), and whole-leg compression ultrasound. Patients could not have deep vein thrombosis proximal to the popliteal vein, hypoxia, hypotension, hepatic failure, or renal failure. They had to be eligible for therapeutic anticoagulation and could not have any condition requiring hospitalization.

With these criteria, the researchers selected 200 patients. They were observed in the ED or hospital for 12-24 hours and then discharged with anticoagulant therapy. Patients received rivaroxaban (n = 149), enoxaparin transitioned to warfarin (n = 26), apixaban (n = 24), or enoxaparin alone (n = 1).

Results

The study’s primary outcome was the 90-day composite rate of all-cause mortality, recurrent symptomatic VTE, and major bleeding. There were no deaths and no cases of recurrent VTE, but one patient did experience major bleeding at day 61 because of a traumatic thigh injury.

Within 7 days of study enrollment, there were 19 patients (9.5%) who returned to the ED and 2 patients (1%) who were admitted to the hospital. One patient with pulmonary infarct was admitted for pain control (day 2); the other was admitted for an elective coronary intervention (day 7) because of a positive cardiac stress test.

Within 30 days, 32 patients (16%) returned to the ED, and 5 (3%) were admitted to the hospital for events unrelated to their PE.

The study also showed that patients were largely satisfied with outpatient care. Of the 146 patients who completed a satisfaction survey at 90 days, 89% said they would choose outpatient management if they had another PE in the future.

“We found a large subset of patients with blood clots who’d do well at home; in fact, who probably did better at home,” Dr. Bledsoe said. “When patients are sent home versus staying in the hospital, they’re at lower risk of getting another infection. It’s a lot less expensive, too.”

Currently, the standard of care in the United States for acute PE is hospitalization for all patients. That’s recommended, in part, because their overall mortality rate is 17%. However, the lower mortality rate among some appropriately risk-stratified patients suggests that at-home care, which has become the norm in some European countries, leads to better outcomes for those patients overall and less chance of a hospital-introduced infection, according to Dr. Bledsoe. “Our findings show that if you appropriately risk-stratify patients, there are a lot of people with blood clots who are safe to go home.”

He added that similar research should be conducted outside of the Intermountain Healthcare system to confirm the results of this study and that a larger group of patients should be studied.

The investigators reported no conflicts related to this study.

SOURCE: Bledsoe JR et al. Chest. 2018 Aug;154(2):249-56.

 

A new study suggests that certain patients with acute pulmonary embolism (PE) may be better off receiving outpatient treatment.

Researchers tested outpatient anticoagulant therapy in 200 patients with PE with a low mortality risk. At 90 days of follow-up, there were no deaths or recurrences of venous thromboembolism (VTE), but one patient experienced major bleeding after a traumatic injury.

A majority of patients said they were satisfied with outpatient care.

Joseph R. Bledsoe, MD, of Intermountain Medical Center in Salt Lake City, and his colleagues reported these results in Chest.

The researchers tracked patients who were treated for acute PE in five Intermountain Healthcare emergency departments (EDs) from 2013 to 2016. The patients had to have a low mortality risk according to the Pulmonary Embolism Severity Index (score less than 86), echocardiography (no signs of right heart strain), and whole-leg compression ultrasound. Patients could not have deep vein thrombosis proximal to the popliteal vein, hypoxia, hypotension, hepatic failure, or renal failure. They had to be eligible for therapeutic anticoagulation and could not have any condition requiring hospitalization.

With these criteria, the researchers selected 200 patients. They were observed in the ED or hospital for 12-24 hours and then discharged with anticoagulant therapy. Patients received rivaroxaban (n = 149), enoxaparin transitioned to warfarin (n = 26), apixaban (n = 24), or enoxaparin alone (n = 1).

Results

The study’s primary outcome was the 90-day composite rate of all-cause mortality, recurrent symptomatic VTE, and major bleeding. There were no deaths and no cases of recurrent VTE, but one patient did experience major bleeding at day 61 because of a traumatic thigh injury.

Within 7 days of study enrollment, there were 19 patients (9.5%) who returned to the ED and 2 patients (1%) who were admitted to the hospital. One patient with pulmonary infarct was admitted for pain control (day 2); the other was admitted for an elective coronary intervention (day 7) because of a positive cardiac stress test.

Within 30 days, 32 patients (16%) returned to the ED, and 5 (3%) were admitted to the hospital for events unrelated to their PE.

The study also showed that patients were largely satisfied with outpatient care. Of the 146 patients who completed a satisfaction survey at 90 days, 89% said they would choose outpatient management if they had another PE in the future.

“We found a large subset of patients with blood clots who’d do well at home; in fact, who probably did better at home,” Dr. Bledsoe said. “When patients are sent home versus staying in the hospital, they’re at lower risk of getting another infection. It’s a lot less expensive, too.”

Currently, the standard of care in the United States for acute PE is hospitalization for all patients. That’s recommended, in part, because their overall mortality rate is 17%. However, the lower mortality rate among some appropriately risk-stratified patients suggests that at-home care, which has become the norm in some European countries, leads to better outcomes for those patients overall and less chance of a hospital-introduced infection, according to Dr. Bledsoe. “Our findings show that if you appropriately risk-stratify patients, there are a lot of people with blood clots who are safe to go home.”

He added that similar research should be conducted outside of the Intermountain Healthcare system to confirm the results of this study and that a larger group of patients should be studied.

The investigators reported no conflicts related to this study.

SOURCE: Bledsoe JR et al. Chest. 2018 Aug;154(2):249-56.

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Key clinical point: There were no deaths or recurrences of pulmonary embolism at 90 days in a group of patients stratified by criteria for low risk.

Major finding: At 90 days of follow-up, there were no deaths or recurrences of venous thromboembolism.

Study details: Researchers tested outpatient anticoagulant therapy in 200 patients with pulmonary embolism with a low mortality risk.

Disclosures: The investigators reported no conflicts related to this study.

Source: Bledsoe JR et al. Chest. 2018 Aug;154(2):249-56.

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The power of connection

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Over the past several months, I’ve found myself increasingly reflective on the intersection of culture, relationships, and my professional work with those who have an autism spectrum disorder. Last winter, an adolescent boy treated by myself and other providers died by suicide. Adam (name changed) had been diagnosed with autism as a toddler and had struggled with anxiety and depression for several years; in the office, as he grew into an athletic teenager, Adam spoke more frequently about “not fitting in” with his peers and therapeutic focus was placed on building Adam’s sense of himself and fostering his self-confidence and perceived self-competence. His unexpected death was a tremendous shock, and his loved ones – including the clinical team – desperately searched for answers that could help add some understanding to the heartbreaking event.

Rawpixel/Thinkstock


Around the time of Adam’s death, I was teaching an undergraduate course about the neuroscience of relationships. The class was learning about the brain in love and the importance of social connectedness in overall health. We discussed the reward pathways, libido, notions of synchrony, the meaning of intimacy, prairie voles, dating trends amongst millennials, attachment principles, Harry Harlow’s work with primates, and the dangers associated with loneliness and isolation. Needless to say, my clinical work and my teaching were marked by similarities in theme, themes involving the importance of connection that were worth attending to – particularly as they have heavily influenced my interest in child and adolescent psychiatry since medical school.

In the spring of 2018, these themes were again revisited in the setting of several events. I attended the annual meeting of the Association of Directors of Medical Student Education in Psychiatry (ADMSEP), where Dr. Robert Englander provided an inspiring keynote address on the subject of love as a domain of competence in medical education. He referenced the practice of Metta meditation and how compassion and loving kindness meditation is being studied as a tool for healing and treatment. Certainly, preliminary results from examining the effectiveness of these interventions are promising.1 In June, many of us also were shaken by the deaths of both Kate Spade and Anthony Bourdain, occurring at a time when Centers for Disease Control and Prevention published data indicating rising suicide rates across most of the United States.2

Notably, relationship problems were reported as a major contributor to deaths by suicide. Concurrently, here in Vermont, the 2017 Youth Risk Behavior Survey results were released.Particularly striking were the numbers surrounding the rates of high school LGBT-identified students who thought about suicide (33%) and how they compared to cisgender heterosexual students’ reporting (8%).3 Making sense of these numbers is complicated and many factors appear to be informing the statistics. One can’t help but wonder about the impact of feeling marginalized and isolated on rates of suicide in certain populations. It’s also known that rural Americans have higher suicide rates compared with those living in metropolitan areas, and the lack of social integration and access to mental health care has been examined as a risk factor for these statistics.4,5

 

 



As I attempted to search for answers and reconcile national news with my clinical and teaching experiences, I was struck by something that Andrew Solomon eloquently captured in his June 2018 New Yorker article that again touches upon the theme of connection.6 Mr. Solomon writes “modernity is alienating” and about how he receives correspondence from those who struggle with depression. “What is most striking to me is how alone many of them are ... these people are so alone that they are effectively invisible to the rest of us ... many of them describe suicidal feelings,” he noted.

The power of connection in our day-to-day work is undeniable. The influence of human interaction and appreciating one’s unique narrative is a bedrock of clinical care and can unquestionably allow us to better understand individual suffering, deliver optimal care, and combat shame as Hannah Gadsby boldly shared in her recent Netflix comedy special “Nanette.” This shame can drive one to experience earth-shattering depressive episodes and influence thoughts of suicide. “We simply cannot make it on our own,” Ms. Gadsby explains, “we’re humans. We’re to be connected.” Humans are indeed hardwired for connection; isolation and disconnection can lead to significant health problems and are linked with mental health concerns. The former U.S. Surgeon General Vivek H. Murthy, MD, has referred to loneliness as an epidemic, and those with autism may be at increased risk for feeling lonely and isolated.7,8

Synthesizing thoughts about relationships, suicide, loneliness, love, well-being, and autism produces a complicated web of, well, connections. Suicide in the autism population hasn’t been well researched, but one 2016 study revealed sobering numbers about suicide being a leading cause of premature death in people with autism.9 How do these numbers associate with feelings of isolation, wanting to fit in, and troubles talking about emotions – all of which can characterize those with ASD? Data, not surprisingly, support the role of loneliness as a risk factor for the development of depression and suicidal ideation in those with ASD.10 In addition, social-communication challenges, even in the absence of an autism diagnosis, are related to depression and suicidality.11 Another recent study showed a relationship between autistic traits and depression symptoms, an association seemingly linked to being bullied.12 We cannot continue to hold onto the myth that individuals with autism don’t desire relationships and love because it’s these desires and not being able to fulfill them, limited opportunities to engage in meaningful experiences, and feeling different that can lead to negative outcomes.

Dr. Jeremiah Dickerson


Talking about suicide is critical in helping to prevent suicide, and there is a need for prevention and screening strategies tailored to specific communities and groups of individuals. How do we go about identifying specific risk factors and address them accordingly, especially in the setting of autism, where risk factors such as behavior change and changes in social interactions can be common features innate to the developmental disorder?

Changes in policy and practice are needed, and all people in crisis deserve supports that appreciate their unique challenges and individual strengths.13 Clinically, being mindful of the consequences of victimization and the power of connectedness and its role in health and well-being is critically important. The Centers for Disease Control and Prevention states that “connectedness is a common thread that weaves together many of the influences of suicidal behavior and has direct relevance for prevention,” and providers should strive to enhance positive connectedness between persons, between individuals, families and community organizations, and among organizations and social institutions.14

Spend time with patients asking about their relationships, desires, and satisfaction with social connections. Ask about loneliness and feelings of rejection while communicating to marginalized individuals that they matter. Finally, appreciate the benefits of safety planning.15 Developing a tailored safety plan with someone who is endorsing suicidal thinking, making follow-up connections (i.e., a telephone call), communicating concern, and instilling hope can save lives.
 

 

Dr. Dickerson, a child and adolescent psychiatrist, is assistant professor of psychiatry at the University of Vermont, Burlington, where he is director of the autism diagnostic clinic. Email him at [email protected].

References

1. Harv Rev Psychiatry. 2018 Jul/Aug;26(4):201-15.

2. MMWR Morb Mortal Wkly Rep. 2018 Jun 8;67(22):617-24.

3. www.healthvermont.gov/YRBS

4. MMWR Surveill Summ. 2017 Oct;66(18):1-16.

5. JAMA Pediatr. 2015 May;169(5):466-73.

6. “Anthony Bourdain, Kate Spade, and the Preventable Tragedies of Suicide,” By Anthony Solomon, The New Yorker. Jun 8, 2018.

7. “Work and the Loneliness Epidemic,” By Vivek H. Murthy, Harvard Business Review. Sep 28, 2017.

8. Child Dev. 2000 Mar-Apr;71(2):447-56.

9. Br J Psychiatry. 2016 Mar;208(3):232-8.

10. Depress Anxiety. 2018 Jul;35(7):648-57.

11. J Am Acad Child Adolesc Psychiatry. 2018 May;57(5):313-20.

12. JAMA Psychiatry. 2018 Aug 1;75(8):835-43.

13. The Lancet Psychiatry. 2017 Jun;4(6):e11.

14. “Promoting Individual, Family, and Community Connectedness to Prevent Suicidal Behavior,” Centers for Disease Control and Prevention, National Center for Injury Prevention and Control, www.cdc.gov/injury.

15. JAMA Psychiatry. 2018 Jul 11. doi: 10.1001/jamapsychiatry.2018.1776.

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Over the past several months, I’ve found myself increasingly reflective on the intersection of culture, relationships, and my professional work with those who have an autism spectrum disorder. Last winter, an adolescent boy treated by myself and other providers died by suicide. Adam (name changed) had been diagnosed with autism as a toddler and had struggled with anxiety and depression for several years; in the office, as he grew into an athletic teenager, Adam spoke more frequently about “not fitting in” with his peers and therapeutic focus was placed on building Adam’s sense of himself and fostering his self-confidence and perceived self-competence. His unexpected death was a tremendous shock, and his loved ones – including the clinical team – desperately searched for answers that could help add some understanding to the heartbreaking event.

Rawpixel/Thinkstock


Around the time of Adam’s death, I was teaching an undergraduate course about the neuroscience of relationships. The class was learning about the brain in love and the importance of social connectedness in overall health. We discussed the reward pathways, libido, notions of synchrony, the meaning of intimacy, prairie voles, dating trends amongst millennials, attachment principles, Harry Harlow’s work with primates, and the dangers associated with loneliness and isolation. Needless to say, my clinical work and my teaching were marked by similarities in theme, themes involving the importance of connection that were worth attending to – particularly as they have heavily influenced my interest in child and adolescent psychiatry since medical school.

In the spring of 2018, these themes were again revisited in the setting of several events. I attended the annual meeting of the Association of Directors of Medical Student Education in Psychiatry (ADMSEP), where Dr. Robert Englander provided an inspiring keynote address on the subject of love as a domain of competence in medical education. He referenced the practice of Metta meditation and how compassion and loving kindness meditation is being studied as a tool for healing and treatment. Certainly, preliminary results from examining the effectiveness of these interventions are promising.1 In June, many of us also were shaken by the deaths of both Kate Spade and Anthony Bourdain, occurring at a time when Centers for Disease Control and Prevention published data indicating rising suicide rates across most of the United States.2

Notably, relationship problems were reported as a major contributor to deaths by suicide. Concurrently, here in Vermont, the 2017 Youth Risk Behavior Survey results were released.Particularly striking were the numbers surrounding the rates of high school LGBT-identified students who thought about suicide (33%) and how they compared to cisgender heterosexual students’ reporting (8%).3 Making sense of these numbers is complicated and many factors appear to be informing the statistics. One can’t help but wonder about the impact of feeling marginalized and isolated on rates of suicide in certain populations. It’s also known that rural Americans have higher suicide rates compared with those living in metropolitan areas, and the lack of social integration and access to mental health care has been examined as a risk factor for these statistics.4,5

 

 



As I attempted to search for answers and reconcile national news with my clinical and teaching experiences, I was struck by something that Andrew Solomon eloquently captured in his June 2018 New Yorker article that again touches upon the theme of connection.6 Mr. Solomon writes “modernity is alienating” and about how he receives correspondence from those who struggle with depression. “What is most striking to me is how alone many of them are ... these people are so alone that they are effectively invisible to the rest of us ... many of them describe suicidal feelings,” he noted.

The power of connection in our day-to-day work is undeniable. The influence of human interaction and appreciating one’s unique narrative is a bedrock of clinical care and can unquestionably allow us to better understand individual suffering, deliver optimal care, and combat shame as Hannah Gadsby boldly shared in her recent Netflix comedy special “Nanette.” This shame can drive one to experience earth-shattering depressive episodes and influence thoughts of suicide. “We simply cannot make it on our own,” Ms. Gadsby explains, “we’re humans. We’re to be connected.” Humans are indeed hardwired for connection; isolation and disconnection can lead to significant health problems and are linked with mental health concerns. The former U.S. Surgeon General Vivek H. Murthy, MD, has referred to loneliness as an epidemic, and those with autism may be at increased risk for feeling lonely and isolated.7,8

Synthesizing thoughts about relationships, suicide, loneliness, love, well-being, and autism produces a complicated web of, well, connections. Suicide in the autism population hasn’t been well researched, but one 2016 study revealed sobering numbers about suicide being a leading cause of premature death in people with autism.9 How do these numbers associate with feelings of isolation, wanting to fit in, and troubles talking about emotions – all of which can characterize those with ASD? Data, not surprisingly, support the role of loneliness as a risk factor for the development of depression and suicidal ideation in those with ASD.10 In addition, social-communication challenges, even in the absence of an autism diagnosis, are related to depression and suicidality.11 Another recent study showed a relationship between autistic traits and depression symptoms, an association seemingly linked to being bullied.12 We cannot continue to hold onto the myth that individuals with autism don’t desire relationships and love because it’s these desires and not being able to fulfill them, limited opportunities to engage in meaningful experiences, and feeling different that can lead to negative outcomes.

Dr. Jeremiah Dickerson


Talking about suicide is critical in helping to prevent suicide, and there is a need for prevention and screening strategies tailored to specific communities and groups of individuals. How do we go about identifying specific risk factors and address them accordingly, especially in the setting of autism, where risk factors such as behavior change and changes in social interactions can be common features innate to the developmental disorder?

Changes in policy and practice are needed, and all people in crisis deserve supports that appreciate their unique challenges and individual strengths.13 Clinically, being mindful of the consequences of victimization and the power of connectedness and its role in health and well-being is critically important. The Centers for Disease Control and Prevention states that “connectedness is a common thread that weaves together many of the influences of suicidal behavior and has direct relevance for prevention,” and providers should strive to enhance positive connectedness between persons, between individuals, families and community organizations, and among organizations and social institutions.14

Spend time with patients asking about their relationships, desires, and satisfaction with social connections. Ask about loneliness and feelings of rejection while communicating to marginalized individuals that they matter. Finally, appreciate the benefits of safety planning.15 Developing a tailored safety plan with someone who is endorsing suicidal thinking, making follow-up connections (i.e., a telephone call), communicating concern, and instilling hope can save lives.
 

 

Dr. Dickerson, a child and adolescent psychiatrist, is assistant professor of psychiatry at the University of Vermont, Burlington, where he is director of the autism diagnostic clinic. Email him at [email protected].

References

1. Harv Rev Psychiatry. 2018 Jul/Aug;26(4):201-15.

2. MMWR Morb Mortal Wkly Rep. 2018 Jun 8;67(22):617-24.

3. www.healthvermont.gov/YRBS

4. MMWR Surveill Summ. 2017 Oct;66(18):1-16.

5. JAMA Pediatr. 2015 May;169(5):466-73.

6. “Anthony Bourdain, Kate Spade, and the Preventable Tragedies of Suicide,” By Anthony Solomon, The New Yorker. Jun 8, 2018.

7. “Work and the Loneliness Epidemic,” By Vivek H. Murthy, Harvard Business Review. Sep 28, 2017.

8. Child Dev. 2000 Mar-Apr;71(2):447-56.

9. Br J Psychiatry. 2016 Mar;208(3):232-8.

10. Depress Anxiety. 2018 Jul;35(7):648-57.

11. J Am Acad Child Adolesc Psychiatry. 2018 May;57(5):313-20.

12. JAMA Psychiatry. 2018 Aug 1;75(8):835-43.

13. The Lancet Psychiatry. 2017 Jun;4(6):e11.

14. “Promoting Individual, Family, and Community Connectedness to Prevent Suicidal Behavior,” Centers for Disease Control and Prevention, National Center for Injury Prevention and Control, www.cdc.gov/injury.

15. JAMA Psychiatry. 2018 Jul 11. doi: 10.1001/jamapsychiatry.2018.1776.

 

Over the past several months, I’ve found myself increasingly reflective on the intersection of culture, relationships, and my professional work with those who have an autism spectrum disorder. Last winter, an adolescent boy treated by myself and other providers died by suicide. Adam (name changed) had been diagnosed with autism as a toddler and had struggled with anxiety and depression for several years; in the office, as he grew into an athletic teenager, Adam spoke more frequently about “not fitting in” with his peers and therapeutic focus was placed on building Adam’s sense of himself and fostering his self-confidence and perceived self-competence. His unexpected death was a tremendous shock, and his loved ones – including the clinical team – desperately searched for answers that could help add some understanding to the heartbreaking event.

Rawpixel/Thinkstock


Around the time of Adam’s death, I was teaching an undergraduate course about the neuroscience of relationships. The class was learning about the brain in love and the importance of social connectedness in overall health. We discussed the reward pathways, libido, notions of synchrony, the meaning of intimacy, prairie voles, dating trends amongst millennials, attachment principles, Harry Harlow’s work with primates, and the dangers associated with loneliness and isolation. Needless to say, my clinical work and my teaching were marked by similarities in theme, themes involving the importance of connection that were worth attending to – particularly as they have heavily influenced my interest in child and adolescent psychiatry since medical school.

In the spring of 2018, these themes were again revisited in the setting of several events. I attended the annual meeting of the Association of Directors of Medical Student Education in Psychiatry (ADMSEP), where Dr. Robert Englander provided an inspiring keynote address on the subject of love as a domain of competence in medical education. He referenced the practice of Metta meditation and how compassion and loving kindness meditation is being studied as a tool for healing and treatment. Certainly, preliminary results from examining the effectiveness of these interventions are promising.1 In June, many of us also were shaken by the deaths of both Kate Spade and Anthony Bourdain, occurring at a time when Centers for Disease Control and Prevention published data indicating rising suicide rates across most of the United States.2

Notably, relationship problems were reported as a major contributor to deaths by suicide. Concurrently, here in Vermont, the 2017 Youth Risk Behavior Survey results were released.Particularly striking were the numbers surrounding the rates of high school LGBT-identified students who thought about suicide (33%) and how they compared to cisgender heterosexual students’ reporting (8%).3 Making sense of these numbers is complicated and many factors appear to be informing the statistics. One can’t help but wonder about the impact of feeling marginalized and isolated on rates of suicide in certain populations. It’s also known that rural Americans have higher suicide rates compared with those living in metropolitan areas, and the lack of social integration and access to mental health care has been examined as a risk factor for these statistics.4,5

 

 



As I attempted to search for answers and reconcile national news with my clinical and teaching experiences, I was struck by something that Andrew Solomon eloquently captured in his June 2018 New Yorker article that again touches upon the theme of connection.6 Mr. Solomon writes “modernity is alienating” and about how he receives correspondence from those who struggle with depression. “What is most striking to me is how alone many of them are ... these people are so alone that they are effectively invisible to the rest of us ... many of them describe suicidal feelings,” he noted.

The power of connection in our day-to-day work is undeniable. The influence of human interaction and appreciating one’s unique narrative is a bedrock of clinical care and can unquestionably allow us to better understand individual suffering, deliver optimal care, and combat shame as Hannah Gadsby boldly shared in her recent Netflix comedy special “Nanette.” This shame can drive one to experience earth-shattering depressive episodes and influence thoughts of suicide. “We simply cannot make it on our own,” Ms. Gadsby explains, “we’re humans. We’re to be connected.” Humans are indeed hardwired for connection; isolation and disconnection can lead to significant health problems and are linked with mental health concerns. The former U.S. Surgeon General Vivek H. Murthy, MD, has referred to loneliness as an epidemic, and those with autism may be at increased risk for feeling lonely and isolated.7,8

Synthesizing thoughts about relationships, suicide, loneliness, love, well-being, and autism produces a complicated web of, well, connections. Suicide in the autism population hasn’t been well researched, but one 2016 study revealed sobering numbers about suicide being a leading cause of premature death in people with autism.9 How do these numbers associate with feelings of isolation, wanting to fit in, and troubles talking about emotions – all of which can characterize those with ASD? Data, not surprisingly, support the role of loneliness as a risk factor for the development of depression and suicidal ideation in those with ASD.10 In addition, social-communication challenges, even in the absence of an autism diagnosis, are related to depression and suicidality.11 Another recent study showed a relationship between autistic traits and depression symptoms, an association seemingly linked to being bullied.12 We cannot continue to hold onto the myth that individuals with autism don’t desire relationships and love because it’s these desires and not being able to fulfill them, limited opportunities to engage in meaningful experiences, and feeling different that can lead to negative outcomes.

Dr. Jeremiah Dickerson


Talking about suicide is critical in helping to prevent suicide, and there is a need for prevention and screening strategies tailored to specific communities and groups of individuals. How do we go about identifying specific risk factors and address them accordingly, especially in the setting of autism, where risk factors such as behavior change and changes in social interactions can be common features innate to the developmental disorder?

Changes in policy and practice are needed, and all people in crisis deserve supports that appreciate their unique challenges and individual strengths.13 Clinically, being mindful of the consequences of victimization and the power of connectedness and its role in health and well-being is critically important. The Centers for Disease Control and Prevention states that “connectedness is a common thread that weaves together many of the influences of suicidal behavior and has direct relevance for prevention,” and providers should strive to enhance positive connectedness between persons, between individuals, families and community organizations, and among organizations and social institutions.14

Spend time with patients asking about their relationships, desires, and satisfaction with social connections. Ask about loneliness and feelings of rejection while communicating to marginalized individuals that they matter. Finally, appreciate the benefits of safety planning.15 Developing a tailored safety plan with someone who is endorsing suicidal thinking, making follow-up connections (i.e., a telephone call), communicating concern, and instilling hope can save lives.
 

 

Dr. Dickerson, a child and adolescent psychiatrist, is assistant professor of psychiatry at the University of Vermont, Burlington, where he is director of the autism diagnostic clinic. Email him at [email protected].

References

1. Harv Rev Psychiatry. 2018 Jul/Aug;26(4):201-15.

2. MMWR Morb Mortal Wkly Rep. 2018 Jun 8;67(22):617-24.

3. www.healthvermont.gov/YRBS

4. MMWR Surveill Summ. 2017 Oct;66(18):1-16.

5. JAMA Pediatr. 2015 May;169(5):466-73.

6. “Anthony Bourdain, Kate Spade, and the Preventable Tragedies of Suicide,” By Anthony Solomon, The New Yorker. Jun 8, 2018.

7. “Work and the Loneliness Epidemic,” By Vivek H. Murthy, Harvard Business Review. Sep 28, 2017.

8. Child Dev. 2000 Mar-Apr;71(2):447-56.

9. Br J Psychiatry. 2016 Mar;208(3):232-8.

10. Depress Anxiety. 2018 Jul;35(7):648-57.

11. J Am Acad Child Adolesc Psychiatry. 2018 May;57(5):313-20.

12. JAMA Psychiatry. 2018 Aug 1;75(8):835-43.

13. The Lancet Psychiatry. 2017 Jun;4(6):e11.

14. “Promoting Individual, Family, and Community Connectedness to Prevent Suicidal Behavior,” Centers for Disease Control and Prevention, National Center for Injury Prevention and Control, www.cdc.gov/injury.

15. JAMA Psychiatry. 2018 Jul 11. doi: 10.1001/jamapsychiatry.2018.1776.

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Integrated pain program reduced LOS for sickle cell patients

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– Pediatric patients who received interdisciplinary outpatient care for sickle cell disease–related chronic pain experienced a reduction in average length of stay for pain-related hospitalizations, according to an exploratory analysis of patient outcomes at a single center.

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Experiences at Children’s Mercy Hospital in Kansas City, Mo., have added to the body of evidence supporting integrative care for sickle cell disease (SCD) pain, Derrick L. Goubeaux, DO, said during an interview at the annual symposium of the Foundation for Sickle Cell Disease Research.

With time, chronic pain can become an overlay on pain from vasoocclusive crises as patients with SCD age, shifting the way that patients and providers think about pain, said Dr. Goubeaux, a pediatric hematology/oncology fellow at Children’s Mercy Hospital in Kansas City.

Using a collaborative approach that pulls in psychologists, social workers, and pain management specialists, the hospital’s multidisciplinary Sickle Cell Integrated Pain Program (SCIPP) seeks to optimize pain control by adding nonpharmacologic measures to medications, he said.

Dr. Goubeaux and his colleagues conducted a retrospective chart review that looked at individuals who received care from, or were referred to, the institution’s SCD program. Included in the study were patients who received care for SCD for at least 2 years before their referral to the SCIPP clinic, so that investigators could compare care for those patients before and after SCIPP clinic integration. The study also included patients who had not yet been integrated into SCIPP clinic care, for comparison.

Though the seven patients who were integrated into the SCIPP clinic did not have fewer hospitalizations than the five who were not referred, average length of stay (LOS) for the SCIPP patients dropped from 11 days to 8 days. Mean LOS also decreased for the non-SCIPP patients, from 7.4 to 5.8 days. The number of admissions per month for both groups increased over the study period, from a mean of 0.41 to 0.84 admissions per month for SCIPP patients, and from 0.27 to 0.43 for non-SCIPP patients.

The patients, who ranged in age from 138 to 253 months, mostly had HbSS SCD, but HbSbeta0, HbSD, and HbSC patients were also included. Four patients in the SCIPP group and two of the non-SCIPP patients were taking hydroxyurea.

Noting that data collection is still in the early stages, Dr. Goubeaux and his collaborators observed that “the LOS has shortened by 3 days in the integrated group, compared to 1.6 days in the [non-SCIPP] group.” They are currently also investigating whether costs per admission and admission-associated opioid use differs for patients integrated into the SCIPP clinic.

Aside from the small number of patients studied, Dr. Goubeaux and his colleagues acknowledged that even non-SCIPP patients are likely to have had pain management and psychology consultations during their inpatient stays – and these consults are conducted by SCIPP-associated providers.

Dr. Goubeaux reported no relevant disclosures or outside sources of funding.

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– Pediatric patients who received interdisciplinary outpatient care for sickle cell disease–related chronic pain experienced a reduction in average length of stay for pain-related hospitalizations, according to an exploratory analysis of patient outcomes at a single center.

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Experiences at Children’s Mercy Hospital in Kansas City, Mo., have added to the body of evidence supporting integrative care for sickle cell disease (SCD) pain, Derrick L. Goubeaux, DO, said during an interview at the annual symposium of the Foundation for Sickle Cell Disease Research.

With time, chronic pain can become an overlay on pain from vasoocclusive crises as patients with SCD age, shifting the way that patients and providers think about pain, said Dr. Goubeaux, a pediatric hematology/oncology fellow at Children’s Mercy Hospital in Kansas City.

Using a collaborative approach that pulls in psychologists, social workers, and pain management specialists, the hospital’s multidisciplinary Sickle Cell Integrated Pain Program (SCIPP) seeks to optimize pain control by adding nonpharmacologic measures to medications, he said.

Dr. Goubeaux and his colleagues conducted a retrospective chart review that looked at individuals who received care from, or were referred to, the institution’s SCD program. Included in the study were patients who received care for SCD for at least 2 years before their referral to the SCIPP clinic, so that investigators could compare care for those patients before and after SCIPP clinic integration. The study also included patients who had not yet been integrated into SCIPP clinic care, for comparison.

Though the seven patients who were integrated into the SCIPP clinic did not have fewer hospitalizations than the five who were not referred, average length of stay (LOS) for the SCIPP patients dropped from 11 days to 8 days. Mean LOS also decreased for the non-SCIPP patients, from 7.4 to 5.8 days. The number of admissions per month for both groups increased over the study period, from a mean of 0.41 to 0.84 admissions per month for SCIPP patients, and from 0.27 to 0.43 for non-SCIPP patients.

The patients, who ranged in age from 138 to 253 months, mostly had HbSS SCD, but HbSbeta0, HbSD, and HbSC patients were also included. Four patients in the SCIPP group and two of the non-SCIPP patients were taking hydroxyurea.

Noting that data collection is still in the early stages, Dr. Goubeaux and his collaborators observed that “the LOS has shortened by 3 days in the integrated group, compared to 1.6 days in the [non-SCIPP] group.” They are currently also investigating whether costs per admission and admission-associated opioid use differs for patients integrated into the SCIPP clinic.

Aside from the small number of patients studied, Dr. Goubeaux and his colleagues acknowledged that even non-SCIPP patients are likely to have had pain management and psychology consultations during their inpatient stays – and these consults are conducted by SCIPP-associated providers.

Dr. Goubeaux reported no relevant disclosures or outside sources of funding.

 

– Pediatric patients who received interdisciplinary outpatient care for sickle cell disease–related chronic pain experienced a reduction in average length of stay for pain-related hospitalizations, according to an exploratory analysis of patient outcomes at a single center.

Dr_Microbe/Thinkstock

Experiences at Children’s Mercy Hospital in Kansas City, Mo., have added to the body of evidence supporting integrative care for sickle cell disease (SCD) pain, Derrick L. Goubeaux, DO, said during an interview at the annual symposium of the Foundation for Sickle Cell Disease Research.

With time, chronic pain can become an overlay on pain from vasoocclusive crises as patients with SCD age, shifting the way that patients and providers think about pain, said Dr. Goubeaux, a pediatric hematology/oncology fellow at Children’s Mercy Hospital in Kansas City.

Using a collaborative approach that pulls in psychologists, social workers, and pain management specialists, the hospital’s multidisciplinary Sickle Cell Integrated Pain Program (SCIPP) seeks to optimize pain control by adding nonpharmacologic measures to medications, he said.

Dr. Goubeaux and his colleagues conducted a retrospective chart review that looked at individuals who received care from, or were referred to, the institution’s SCD program. Included in the study were patients who received care for SCD for at least 2 years before their referral to the SCIPP clinic, so that investigators could compare care for those patients before and after SCIPP clinic integration. The study also included patients who had not yet been integrated into SCIPP clinic care, for comparison.

Though the seven patients who were integrated into the SCIPP clinic did not have fewer hospitalizations than the five who were not referred, average length of stay (LOS) for the SCIPP patients dropped from 11 days to 8 days. Mean LOS also decreased for the non-SCIPP patients, from 7.4 to 5.8 days. The number of admissions per month for both groups increased over the study period, from a mean of 0.41 to 0.84 admissions per month for SCIPP patients, and from 0.27 to 0.43 for non-SCIPP patients.

The patients, who ranged in age from 138 to 253 months, mostly had HbSS SCD, but HbSbeta0, HbSD, and HbSC patients were also included. Four patients in the SCIPP group and two of the non-SCIPP patients were taking hydroxyurea.

Noting that data collection is still in the early stages, Dr. Goubeaux and his collaborators observed that “the LOS has shortened by 3 days in the integrated group, compared to 1.6 days in the [non-SCIPP] group.” They are currently also investigating whether costs per admission and admission-associated opioid use differs for patients integrated into the SCIPP clinic.

Aside from the small number of patients studied, Dr. Goubeaux and his colleagues acknowledged that even non-SCIPP patients are likely to have had pain management and psychology consultations during their inpatient stays – and these consults are conducted by SCIPP-associated providers.

Dr. Goubeaux reported no relevant disclosures or outside sources of funding.

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Key clinical point: Pain-related hospital stays were shorter for sickle cell disease patients receiving multidisciplinary pain care.

Major finding: Mean length of stay dropped from 11 days to 8 days after patients were referred to a multidisciplinary care clinic.

Study details: A retrospective chart review of 12 pediatric patients with chronic sickle cell disease-related pain.

Disclosures: The authors reported no conflicts of interest or outside sources of funding.

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Alopecia areata linked to mental health disorders

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Alopecia areata is associated with greater frequency of mental health disorders, according to a new analysis of U.S. hospitalizations.

Specifically, the analysis found, alopecia areata patients are at risk for any mental health disorder, anxiety disorders, attention-deficit/hyperactivity disorder, dementia, mood disorders, personality disorders, and suicide or intentionally self-inflicted injury. The report was published in the Journal of the American Academy of Dermatology.

The researchers worked with 87,053,155 adult and child records from the 2002-2012 National Inpatient Sample, which represents 20% of U.S. hospitalizations. They identified inpatients with alopecia areata based on the ICD-9-CM code and compared them to all patients without the condition.

Overall, 5,605 patients had alopecia areata, which was the secondary diagnosis more than 99% of the time. Compared with inpatients without alopecia areata, those with the disorder were more likely to be younger (42.2 vs. 47.9 years; P less than .0001), female (61.7% vs. 58.6%; P = .0297), and uninsured (8.1% vs. 5.5%; P less than .0001). In addition, inpatients with alopecia areata had a greater frequency of mental health disorders (32.8% vs. 20.0%; P less than .0001) and were more likely to have a primary mental health diagnosis (5.5% vs. 2.2%; P less than .0001), reported Vivek Singam of Northwestern University, Chicago, and his associates.

Among 15 mental health or classes of disorders examined, alopecia areata patients were at greater risk in 13 of them. The only exceptions were delirium/dementia/amnestic/cognitive disorders and disorders diagnosed in infancy, childhood, or adolescence.

Alopecia areata patients with a mental health disorder had a mean hospital stay of 6.0 days (95% confidence interval, 5.4.-6.6) and hospitalization cost of $11,907 (95% CI, $10,312-$13,503).

Previous studies had shown similar relationships. However, previous studies showed lower risk of alopecia areata and schizophrenia and no increased risk of ADHD, compared with the current study’s findings. The authors could offer no explanation for those differences.

The strengths of the current analysis include its use of a large-scale, nationally representative cohort and its large sample size, as well its inclusion of a broad range of mental health disorders. Because of its cross-sectional design, the study could not establish the temporal relationship between alopecia areata and mental health disorders.

It is unclear whether psychosocial stress might cause or exacerbate alopecia areata, or whether alopecia areata can lead to or worsen mental health disorders.

The researchers called for additional studies to understand this relationship and potential mechanisms.

The Agency for Healthcare Research and Quality and the Dermatology Foundation funded the study. The researchers declared having no conflicts of interest.

SOURCE: Singam V et al. J Am Acad Dermatol. 2018 Aug 6. doi: 10.1016/j.jaad.2018.07.044.

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Alopecia areata is associated with greater frequency of mental health disorders, according to a new analysis of U.S. hospitalizations.

Specifically, the analysis found, alopecia areata patients are at risk for any mental health disorder, anxiety disorders, attention-deficit/hyperactivity disorder, dementia, mood disorders, personality disorders, and suicide or intentionally self-inflicted injury. The report was published in the Journal of the American Academy of Dermatology.

The researchers worked with 87,053,155 adult and child records from the 2002-2012 National Inpatient Sample, which represents 20% of U.S. hospitalizations. They identified inpatients with alopecia areata based on the ICD-9-CM code and compared them to all patients without the condition.

Overall, 5,605 patients had alopecia areata, which was the secondary diagnosis more than 99% of the time. Compared with inpatients without alopecia areata, those with the disorder were more likely to be younger (42.2 vs. 47.9 years; P less than .0001), female (61.7% vs. 58.6%; P = .0297), and uninsured (8.1% vs. 5.5%; P less than .0001). In addition, inpatients with alopecia areata had a greater frequency of mental health disorders (32.8% vs. 20.0%; P less than .0001) and were more likely to have a primary mental health diagnosis (5.5% vs. 2.2%; P less than .0001), reported Vivek Singam of Northwestern University, Chicago, and his associates.

Among 15 mental health or classes of disorders examined, alopecia areata patients were at greater risk in 13 of them. The only exceptions were delirium/dementia/amnestic/cognitive disorders and disorders diagnosed in infancy, childhood, or adolescence.

Alopecia areata patients with a mental health disorder had a mean hospital stay of 6.0 days (95% confidence interval, 5.4.-6.6) and hospitalization cost of $11,907 (95% CI, $10,312-$13,503).

Previous studies had shown similar relationships. However, previous studies showed lower risk of alopecia areata and schizophrenia and no increased risk of ADHD, compared with the current study’s findings. The authors could offer no explanation for those differences.

The strengths of the current analysis include its use of a large-scale, nationally representative cohort and its large sample size, as well its inclusion of a broad range of mental health disorders. Because of its cross-sectional design, the study could not establish the temporal relationship between alopecia areata and mental health disorders.

It is unclear whether psychosocial stress might cause or exacerbate alopecia areata, or whether alopecia areata can lead to or worsen mental health disorders.

The researchers called for additional studies to understand this relationship and potential mechanisms.

The Agency for Healthcare Research and Quality and the Dermatology Foundation funded the study. The researchers declared having no conflicts of interest.

SOURCE: Singam V et al. J Am Acad Dermatol. 2018 Aug 6. doi: 10.1016/j.jaad.2018.07.044.

 

Alopecia areata is associated with greater frequency of mental health disorders, according to a new analysis of U.S. hospitalizations.

Specifically, the analysis found, alopecia areata patients are at risk for any mental health disorder, anxiety disorders, attention-deficit/hyperactivity disorder, dementia, mood disorders, personality disorders, and suicide or intentionally self-inflicted injury. The report was published in the Journal of the American Academy of Dermatology.

The researchers worked with 87,053,155 adult and child records from the 2002-2012 National Inpatient Sample, which represents 20% of U.S. hospitalizations. They identified inpatients with alopecia areata based on the ICD-9-CM code and compared them to all patients without the condition.

Overall, 5,605 patients had alopecia areata, which was the secondary diagnosis more than 99% of the time. Compared with inpatients without alopecia areata, those with the disorder were more likely to be younger (42.2 vs. 47.9 years; P less than .0001), female (61.7% vs. 58.6%; P = .0297), and uninsured (8.1% vs. 5.5%; P less than .0001). In addition, inpatients with alopecia areata had a greater frequency of mental health disorders (32.8% vs. 20.0%; P less than .0001) and were more likely to have a primary mental health diagnosis (5.5% vs. 2.2%; P less than .0001), reported Vivek Singam of Northwestern University, Chicago, and his associates.

Among 15 mental health or classes of disorders examined, alopecia areata patients were at greater risk in 13 of them. The only exceptions were delirium/dementia/amnestic/cognitive disorders and disorders diagnosed in infancy, childhood, or adolescence.

Alopecia areata patients with a mental health disorder had a mean hospital stay of 6.0 days (95% confidence interval, 5.4.-6.6) and hospitalization cost of $11,907 (95% CI, $10,312-$13,503).

Previous studies had shown similar relationships. However, previous studies showed lower risk of alopecia areata and schizophrenia and no increased risk of ADHD, compared with the current study’s findings. The authors could offer no explanation for those differences.

The strengths of the current analysis include its use of a large-scale, nationally representative cohort and its large sample size, as well its inclusion of a broad range of mental health disorders. Because of its cross-sectional design, the study could not establish the temporal relationship between alopecia areata and mental health disorders.

It is unclear whether psychosocial stress might cause or exacerbate alopecia areata, or whether alopecia areata can lead to or worsen mental health disorders.

The researchers called for additional studies to understand this relationship and potential mechanisms.

The Agency for Healthcare Research and Quality and the Dermatology Foundation funded the study. The researchers declared having no conflicts of interest.

SOURCE: Singam V et al. J Am Acad Dermatol. 2018 Aug 6. doi: 10.1016/j.jaad.2018.07.044.

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Key clinical point: Alopecia areata patients should be monitored closely for mental health disorders.

Major finding: Overall, 32.8% of hospitalized alopecia areata patients had a mental health disorder, compared with 20.0% of controls.

Study details: Retrospective analysis of 87,053,155 U.S. adults and children.

Disclosures: The Agency for Healthcare Research & Quality and the Dermatology Foundation funded the study. The researchers declared having no conflicts of interest.

Source: Singam V et al. J Am Acad Dermatol. 2018 Aug 6. doi: 10.1016/j.jaad.2018.07.044.

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Carcinoma of the colon in a child

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Colon cancer is not common in childhood even though cases have been reported in children and adolescents.1,2 Although it is sporadic, it can arise in the setting of predisposing illnesses such as familial polyposis syndrome or inflammatory bowel disease.2-5 Only 1 or 2 cases per million children are reported globally each year, but the incidence has been noted to be on the rise.2 The nonspecific gastrointestinal symptoms and anemia as features of the disease could also be seen in other common childhood ailments, such as helminthiasis in our region in West Africa. As a result, unless there is a high index of suspicion at the outset, there is a risk that colon cancer will be diagnosed at a late stage, especially in children with no apparent predisposing factor.

In this case, an 11-year-old girl presented to our institution with abdominal pain, melena, abdominal swelling, and iron deficiency anemia. A positive family history of colon cancer in the mother and a brain tumor in an elder sibling prompted a search for and subsequent diagnosis of colon cancer. Her case highlights the importance of a high index of suspicion in making an early diagnosis to achieve the best possible outcomes. This case is being reported in line with the SCARE guidelines.6
 

Case summary and presentation

An 11-year-old girl presented to our facilty with recurrent abdominal pain of 8 months duration, a 4-month history of progressive paleness of the palms, and a month-long fever. There was an associated change in bowel habit to about 2-3 times per day, weight loss despite a preserved appetite, and black, tarry stools. A month before she presented, she developed low-grade pyrexia, dysuria, and pica. She was treated for iron deficiency anemia at a peripheral hospital where she first sought for care with oral iron, folic acid, and vitamin C, but with no improvement in symptoms.

She was the youngest of 8 children born to parents who were first cousins. Her father had died in a car accident when she was a year old, and her mother had died 6 years later after being diagnosed with and treated for colon cancer. An elder sibling died of a brain tumor at the age of 9 years.

On admission to our institution, the girl looked acutely ill. She was severely pale, but afebrile and anicteric. She had no petechial or purpuric skin rashes, but had glossitis with areas of papules on the anterior two-thirds of the dorsum of the tongue. She had no gingival hypertrophy, but had significant peripheral lymphadenopathy and weighed 67% of the weight for her age. In addition, she had generalized abdominal pain and a soft, well-circumscribed tender mass located at the right iliac fossa was palpated and estimated to be 8 cm x 6 cm.

A full blood count showed severe hypochromic microcytic anemia, with a red blood cell count of 2.53 x 1012/L, packed cell volume of 9%, white blood cell count 9.4 x109/L, platelet cell count of 453 x 109/L, mean corpuscular volume of 48.6 fl, and a red cell distribution width of 23.7%. Iron studies could not be done because we lacked the facilities, but a bone marrow aspiration biopsy showed reduced bone marrow iron stores. A fecal occult blood test was positive for blood, but negative for culture, ova, or cysts. An abdominopelvic ultrasound showed the well-circumscribed mass at the right iliac fossa, and that was confirmed by a computed-tomographic scan (Figure 1).



An upper endoscopy revealed fundal and prepyloric erosions and reflux eosophagitis. Although findings from a sigmoidoscopy were normal, a histology of biopsied tissues showed features of chronic inflammation.

There was a delay in arriving at the final diagnosis because the patient’s family faced financial difficulties and some of the imaging procedures were not available at our institution. Other diagnoses that were entertained and managed in this case were iron deficiency anemia from peptic ulcer disease. Six weeks after her initial presentation to our institution, the patient had an exploratory laparotomy. The findings intra-operatively were those of a huge tumor involving the ascending colon measuring 16 x14 cm and extending to involve the cecum and mesenteric lymph nodes (Figure 2).



Kidneys, liver and spleen were macroscopically normal. An assessment of Duke’s stage 3C colon cancer was made and she had an extended radical hemicolectomy with anastomosis.

A 44.5-cm long right hemicolectomy segment comprising a 17-cm ileal segment, a 6-cm cecum, 21.5-cm ascending colon, and an 8-cm appendix was removed. The tumor was located in the ascending colon at 7.5 cm from the distal resection margin and extending 1 cm into the cecum. It had a circumference of 27 cm with fibrinous exudates on its peritoneal surface. Dissection revealed uneven circumferential thickening of the bowel wall, luminal dilatation, marked mucosal ulcerations, and liquid content made up of fecal material and necrotic debris. The tumor cut surface was solid white. We also removed 4 lymph nodes. Other uninvolved areas showed focal mucosal hyperemia, but no polyps were observed. Histology showed moderately differentiated adenocarcinoma (pT4) with ¼ nodal involvement (Figure 3).



The patient’s postoperative course was uneventful, and she had adjuvant chemotherapy with oral capecitabine and intravenous oxaliplatin. She completed the 8-cycle protocol with excellent clinical response and minimal adverse events were recorded. A repeat abdominal CT scan showed no residual tumor (Figure 4), and her full blood count showed normal hematological profile with no evidence of iron deficiency.



She is presently on follow up 2 years after confirmation of the diagnosis. (Her histological diagnosis was made June 2016, and her last clinic follow-up was March 2018.
 

 

 

Discussion

Our patient presented with symptoms of abdominal pain, dysuria, melena, and pallor as in other case reports.7-10 A diagnosis of iron deficiency anemia was initially entertained in view of the hematologic profile, and for which management was instituted. The findings of gastric and duodenal erosions on endoscopy further supported the assumption for and treatment of peptic ulcer disease. Iron deficiency in this patient was owing to chronic blood loss from a tumour located at the upper parts of the. Vague and nonspecific symptoms are associated with delayed diagnosis and poor prognosis.1-5,11 Nonspecificity of symptoms is typical feature of colon cancer as reported in other studies.1,11-13 However, the strong family history of colon cancer heightened suspicion in this case, otherwise the diagnosis of an ascending colon tumor could have been delayed until much later and with graver consequences.

The diagnosis of colon cancer in this child was made about a year after her initial symptoms, and 3 months after her presentation to us. Ascending and transverse colon cancers are usually diagnosed late because the symptoms of intestinal obstruction – frank bleeding – will not present until the illness is substantially advanced. Ameh and Nmadu reported a case series of 8 patients from our facility with rectosigmoid tumor, of whom 6 had mucinous adenocarcinaoma and 5 of those 6 had stage 3C disease. Although the patient in the present case had an advanced disease at diagnosis, she had a moderately differentiated histology in contrast to the 6 previously reported cases, who had mucinous histology.14

Previous studies have shown that colorectal carcinoma is a rare disease worldwide, with an annual age-adjusted incidence of 0.38 people/million.1,2 When it occurs in the young, familial or hereditary predisposition should be highly suspected.1-3 To date, there is scant literature on children younger than 16 years in Nigeria.15 Various studies have found a relationship between patients with early-stage colon cancer and inherited genetic predisposition to the disease.2,5 Familial adenomatous polyposis syndrome is an autosomal dominant disorder characterized by the development of polyps during the first decade of life, extensive polyposis in the second decade, and transformation into frank carcinoma in early adulthood.1-5

Although our patient’s mother was diagnosed with and died of colon cancer, the type of which could not be ascertained because her records could not be traced. However, the operative and histological findings in this patient did not suggest the presence of polyposis. The clinical phenotype for the autosomal recessive mismatch repair deficiency includes susceptibity to glioma, leukemia, lymphoma, and colorectal carcinoma in children and young adults.1,5 Screening for genetic markers in the child in the present case might have identified the genetic abnormalities involved and would have been invaluable in the evaluation of her 6 surviving siblings and further management of this family. In conclusion. A high index of suspicion should prompt inclusion of colon cancer in the differential diagnosis of nonspecific gastrointestinal symptoms associated with colon cancer in children.

Acknowledgment

The authors obtained written informed consent from the patient and her elder sibling before writing this report. In addition, the authors thank all the staff involved in the management of this child in the pediatric medical and surgical wards.

References

1. Sultan I, Rodriguez-Galindo C, El-Taani H, Pastore G, Casanova M, Gallino G, Ferrari A. Distinct features of colorectal cancer in children and adolescents. A population-based study of 159 cases. Cancer. 2010;1;116(3):758-65. 
2. Ferrari A. Intestinal carcinomas. In: Schneider DT, Brecht IB, Olson TA, Ferrari A (eds). Rare tumors in children and adolescents. 1st ed. Copyright, Springer-Verlag Berlin Heidelberg; 2012; chap 32. 
3. Hill DA, Furman WL, Bilups CA, Riedly SE, Cain AM, Rao BN. Colorectal carcinoma in childhood and adolescence: a clinicopathological review. J Clin Oncol. 2007;25(36):5808-5814. 
4. Saab OKR, Furman WL. Epidemiology and management options for colorectal cancer in children. Paediatr Drugs. 2008;10(3):177-192. 
5.  Bertario L, Signoroni S. Gastrointestinal cancer predisposition syndromes. In: Schneider DT, Brecht IB, Olson TA, Ferrari A (eds). Rare tumors in children and adolescents. Copyright, Springer-Verlag Berlin Heidelberg; 2012; chap 30. 
6.  Agha RA, Fowler AJ, Saetta A, et al, for the SCARE Group. The SCARE Statement: consensus-based surgical case report guidelines. Int J Surg. 2016;34:180-186. 
7. Tricoli JV, Seibel NL, Blair DG, Albritton K, Hayes-Lattin B. Unique characteristics of adolescent and young adult acute lymphoblastic leukemia, breast cancer, and colon cancer. J Natl Cancer Inst. 2011;103(8):628-635.  
8. Begum M, Khan ZJ, Hassan K, Karim S. Carcinoma colon of a child presenting with abdominal pain. Bangaladesh J Child Health. 2014;38(1):44-47. 
9. Woods R, Larkin JO, Muldoon C, Kennedy MJ, Mehigan B, McCormick P. Metastatic paediatric colorectal carcinoma. Ir Med J. 2012;105(3):88-89. 
10. Bjoernsen LP, Lindsay MB. An unusual case of pediatric abdominal pain. CJEM. 2011;13(2):133-138. 
11. Takalkar UV, Asegaonkar SB, Kulkarni U, Jadhav A, Advani S, Reddy DN. Carcinoma of colon in an adolescent: a case report with review of literature. Int J Sci Rep 2015;1(2):151-3. 
12. Zamir N, Ahmad S, Akhtar J. Mucinous adenocarcinoma of colon. APSP J Case Rep. 2010;1(2):20. 
13. Al-Tonbary Y, Darwish A, El-Hussein A, Fouda A. Adenocarcinoma of the colon in children: case series and mini-review of the literature. Hematol Oncol Stem Cell Ther. 2013;6(1):29-33. 
14. Ameh EA, Nmadu PT. Colorectal adenocarcinoma in children and adolescents: a report of 8 patients from Zaria, Nigeria. West Afr J Med. 2000;19(4):273-276. 
15. Ibrahim, AE, Afolayan KA, Adeniji OM, Buhari KB. Colorectal carcinoma in children and young adults in Ilorin, Nigeria. West Afr J Med. 2011;30(3):202-205.

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Hafsat Rufai Ahmad, MBBS, FWACP,1 Jamilu Abdullahi Faruk, MBBS, FWACP,1 Tahir Tunde Sholadoye, MBBS, FWACS,2 Aisha Jubril Mohammed, MBBS,1 Halima O Aliyu, MBBS,3 Sani Malam Mado, MBBS, FNMC,4 Kumo Bello, MBBS, FWACP,5 and Olufemi Gboye Ogunrinde, MBBS, FWACP1

 
1Haematology-Oncology Unit, Department of Paediatrics; 2Paediatric Surgery Unit; 3Department of Pathology; 4Gasteroenterology Unit, Department of Paediatrics; and 5Gastroenterology Unit, Department of Internal Medicine, all at Ahmadu Bello University Teaching Hospital Shika-Zaria, Nigeria 

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Hafsat Rufai Ahmad, MBBS, FWACP,1 Jamilu Abdullahi Faruk, MBBS, FWACP,1 Tahir Tunde Sholadoye, MBBS, FWACS,2 Aisha Jubril Mohammed, MBBS,1 Halima O Aliyu, MBBS,3 Sani Malam Mado, MBBS, FNMC,4 Kumo Bello, MBBS, FWACP,5 and Olufemi Gboye Ogunrinde, MBBS, FWACP1

 
1Haematology-Oncology Unit, Department of Paediatrics; 2Paediatric Surgery Unit; 3Department of Pathology; 4Gasteroenterology Unit, Department of Paediatrics; and 5Gastroenterology Unit, Department of Internal Medicine, all at Ahmadu Bello University Teaching Hospital Shika-Zaria, Nigeria 

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Hafsat Rufai Ahmad, MBBS, FWACP,1 Jamilu Abdullahi Faruk, MBBS, FWACP,1 Tahir Tunde Sholadoye, MBBS, FWACS,2 Aisha Jubril Mohammed, MBBS,1 Halima O Aliyu, MBBS,3 Sani Malam Mado, MBBS, FNMC,4 Kumo Bello, MBBS, FWACP,5 and Olufemi Gboye Ogunrinde, MBBS, FWACP1

 
1Haematology-Oncology Unit, Department of Paediatrics; 2Paediatric Surgery Unit; 3Department of Pathology; 4Gasteroenterology Unit, Department of Paediatrics; and 5Gastroenterology Unit, Department of Internal Medicine, all at Ahmadu Bello University Teaching Hospital Shika-Zaria, Nigeria 

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Colon cancer is not common in childhood even though cases have been reported in children and adolescents.1,2 Although it is sporadic, it can arise in the setting of predisposing illnesses such as familial polyposis syndrome or inflammatory bowel disease.2-5 Only 1 or 2 cases per million children are reported globally each year, but the incidence has been noted to be on the rise.2 The nonspecific gastrointestinal symptoms and anemia as features of the disease could also be seen in other common childhood ailments, such as helminthiasis in our region in West Africa. As a result, unless there is a high index of suspicion at the outset, there is a risk that colon cancer will be diagnosed at a late stage, especially in children with no apparent predisposing factor.

In this case, an 11-year-old girl presented to our institution with abdominal pain, melena, abdominal swelling, and iron deficiency anemia. A positive family history of colon cancer in the mother and a brain tumor in an elder sibling prompted a search for and subsequent diagnosis of colon cancer. Her case highlights the importance of a high index of suspicion in making an early diagnosis to achieve the best possible outcomes. This case is being reported in line with the SCARE guidelines.6
 

Case summary and presentation

An 11-year-old girl presented to our facilty with recurrent abdominal pain of 8 months duration, a 4-month history of progressive paleness of the palms, and a month-long fever. There was an associated change in bowel habit to about 2-3 times per day, weight loss despite a preserved appetite, and black, tarry stools. A month before she presented, she developed low-grade pyrexia, dysuria, and pica. She was treated for iron deficiency anemia at a peripheral hospital where she first sought for care with oral iron, folic acid, and vitamin C, but with no improvement in symptoms.

She was the youngest of 8 children born to parents who were first cousins. Her father had died in a car accident when she was a year old, and her mother had died 6 years later after being diagnosed with and treated for colon cancer. An elder sibling died of a brain tumor at the age of 9 years.

On admission to our institution, the girl looked acutely ill. She was severely pale, but afebrile and anicteric. She had no petechial or purpuric skin rashes, but had glossitis with areas of papules on the anterior two-thirds of the dorsum of the tongue. She had no gingival hypertrophy, but had significant peripheral lymphadenopathy and weighed 67% of the weight for her age. In addition, she had generalized abdominal pain and a soft, well-circumscribed tender mass located at the right iliac fossa was palpated and estimated to be 8 cm x 6 cm.

A full blood count showed severe hypochromic microcytic anemia, with a red blood cell count of 2.53 x 1012/L, packed cell volume of 9%, white blood cell count 9.4 x109/L, platelet cell count of 453 x 109/L, mean corpuscular volume of 48.6 fl, and a red cell distribution width of 23.7%. Iron studies could not be done because we lacked the facilities, but a bone marrow aspiration biopsy showed reduced bone marrow iron stores. A fecal occult blood test was positive for blood, but negative for culture, ova, or cysts. An abdominopelvic ultrasound showed the well-circumscribed mass at the right iliac fossa, and that was confirmed by a computed-tomographic scan (Figure 1).



An upper endoscopy revealed fundal and prepyloric erosions and reflux eosophagitis. Although findings from a sigmoidoscopy were normal, a histology of biopsied tissues showed features of chronic inflammation.

There was a delay in arriving at the final diagnosis because the patient’s family faced financial difficulties and some of the imaging procedures were not available at our institution. Other diagnoses that were entertained and managed in this case were iron deficiency anemia from peptic ulcer disease. Six weeks after her initial presentation to our institution, the patient had an exploratory laparotomy. The findings intra-operatively were those of a huge tumor involving the ascending colon measuring 16 x14 cm and extending to involve the cecum and mesenteric lymph nodes (Figure 2).



Kidneys, liver and spleen were macroscopically normal. An assessment of Duke’s stage 3C colon cancer was made and she had an extended radical hemicolectomy with anastomosis.

A 44.5-cm long right hemicolectomy segment comprising a 17-cm ileal segment, a 6-cm cecum, 21.5-cm ascending colon, and an 8-cm appendix was removed. The tumor was located in the ascending colon at 7.5 cm from the distal resection margin and extending 1 cm into the cecum. It had a circumference of 27 cm with fibrinous exudates on its peritoneal surface. Dissection revealed uneven circumferential thickening of the bowel wall, luminal dilatation, marked mucosal ulcerations, and liquid content made up of fecal material and necrotic debris. The tumor cut surface was solid white. We also removed 4 lymph nodes. Other uninvolved areas showed focal mucosal hyperemia, but no polyps were observed. Histology showed moderately differentiated adenocarcinoma (pT4) with ¼ nodal involvement (Figure 3).



The patient’s postoperative course was uneventful, and she had adjuvant chemotherapy with oral capecitabine and intravenous oxaliplatin. She completed the 8-cycle protocol with excellent clinical response and minimal adverse events were recorded. A repeat abdominal CT scan showed no residual tumor (Figure 4), and her full blood count showed normal hematological profile with no evidence of iron deficiency.



She is presently on follow up 2 years after confirmation of the diagnosis. (Her histological diagnosis was made June 2016, and her last clinic follow-up was March 2018.
 

 

 

Discussion

Our patient presented with symptoms of abdominal pain, dysuria, melena, and pallor as in other case reports.7-10 A diagnosis of iron deficiency anemia was initially entertained in view of the hematologic profile, and for which management was instituted. The findings of gastric and duodenal erosions on endoscopy further supported the assumption for and treatment of peptic ulcer disease. Iron deficiency in this patient was owing to chronic blood loss from a tumour located at the upper parts of the. Vague and nonspecific symptoms are associated with delayed diagnosis and poor prognosis.1-5,11 Nonspecificity of symptoms is typical feature of colon cancer as reported in other studies.1,11-13 However, the strong family history of colon cancer heightened suspicion in this case, otherwise the diagnosis of an ascending colon tumor could have been delayed until much later and with graver consequences.

The diagnosis of colon cancer in this child was made about a year after her initial symptoms, and 3 months after her presentation to us. Ascending and transverse colon cancers are usually diagnosed late because the symptoms of intestinal obstruction – frank bleeding – will not present until the illness is substantially advanced. Ameh and Nmadu reported a case series of 8 patients from our facility with rectosigmoid tumor, of whom 6 had mucinous adenocarcinaoma and 5 of those 6 had stage 3C disease. Although the patient in the present case had an advanced disease at diagnosis, she had a moderately differentiated histology in contrast to the 6 previously reported cases, who had mucinous histology.14

Previous studies have shown that colorectal carcinoma is a rare disease worldwide, with an annual age-adjusted incidence of 0.38 people/million.1,2 When it occurs in the young, familial or hereditary predisposition should be highly suspected.1-3 To date, there is scant literature on children younger than 16 years in Nigeria.15 Various studies have found a relationship between patients with early-stage colon cancer and inherited genetic predisposition to the disease.2,5 Familial adenomatous polyposis syndrome is an autosomal dominant disorder characterized by the development of polyps during the first decade of life, extensive polyposis in the second decade, and transformation into frank carcinoma in early adulthood.1-5

Although our patient’s mother was diagnosed with and died of colon cancer, the type of which could not be ascertained because her records could not be traced. However, the operative and histological findings in this patient did not suggest the presence of polyposis. The clinical phenotype for the autosomal recessive mismatch repair deficiency includes susceptibity to glioma, leukemia, lymphoma, and colorectal carcinoma in children and young adults.1,5 Screening for genetic markers in the child in the present case might have identified the genetic abnormalities involved and would have been invaluable in the evaluation of her 6 surviving siblings and further management of this family. In conclusion. A high index of suspicion should prompt inclusion of colon cancer in the differential diagnosis of nonspecific gastrointestinal symptoms associated with colon cancer in children.

Acknowledgment

The authors obtained written informed consent from the patient and her elder sibling before writing this report. In addition, the authors thank all the staff involved in the management of this child in the pediatric medical and surgical wards.

Colon cancer is not common in childhood even though cases have been reported in children and adolescents.1,2 Although it is sporadic, it can arise in the setting of predisposing illnesses such as familial polyposis syndrome or inflammatory bowel disease.2-5 Only 1 or 2 cases per million children are reported globally each year, but the incidence has been noted to be on the rise.2 The nonspecific gastrointestinal symptoms and anemia as features of the disease could also be seen in other common childhood ailments, such as helminthiasis in our region in West Africa. As a result, unless there is a high index of suspicion at the outset, there is a risk that colon cancer will be diagnosed at a late stage, especially in children with no apparent predisposing factor.

In this case, an 11-year-old girl presented to our institution with abdominal pain, melena, abdominal swelling, and iron deficiency anemia. A positive family history of colon cancer in the mother and a brain tumor in an elder sibling prompted a search for and subsequent diagnosis of colon cancer. Her case highlights the importance of a high index of suspicion in making an early diagnosis to achieve the best possible outcomes. This case is being reported in line with the SCARE guidelines.6
 

Case summary and presentation

An 11-year-old girl presented to our facilty with recurrent abdominal pain of 8 months duration, a 4-month history of progressive paleness of the palms, and a month-long fever. There was an associated change in bowel habit to about 2-3 times per day, weight loss despite a preserved appetite, and black, tarry stools. A month before she presented, she developed low-grade pyrexia, dysuria, and pica. She was treated for iron deficiency anemia at a peripheral hospital where she first sought for care with oral iron, folic acid, and vitamin C, but with no improvement in symptoms.

She was the youngest of 8 children born to parents who were first cousins. Her father had died in a car accident when she was a year old, and her mother had died 6 years later after being diagnosed with and treated for colon cancer. An elder sibling died of a brain tumor at the age of 9 years.

On admission to our institution, the girl looked acutely ill. She was severely pale, but afebrile and anicteric. She had no petechial or purpuric skin rashes, but had glossitis with areas of papules on the anterior two-thirds of the dorsum of the tongue. She had no gingival hypertrophy, but had significant peripheral lymphadenopathy and weighed 67% of the weight for her age. In addition, she had generalized abdominal pain and a soft, well-circumscribed tender mass located at the right iliac fossa was palpated and estimated to be 8 cm x 6 cm.

A full blood count showed severe hypochromic microcytic anemia, with a red blood cell count of 2.53 x 1012/L, packed cell volume of 9%, white blood cell count 9.4 x109/L, platelet cell count of 453 x 109/L, mean corpuscular volume of 48.6 fl, and a red cell distribution width of 23.7%. Iron studies could not be done because we lacked the facilities, but a bone marrow aspiration biopsy showed reduced bone marrow iron stores. A fecal occult blood test was positive for blood, but negative for culture, ova, or cysts. An abdominopelvic ultrasound showed the well-circumscribed mass at the right iliac fossa, and that was confirmed by a computed-tomographic scan (Figure 1).



An upper endoscopy revealed fundal and prepyloric erosions and reflux eosophagitis. Although findings from a sigmoidoscopy were normal, a histology of biopsied tissues showed features of chronic inflammation.

There was a delay in arriving at the final diagnosis because the patient’s family faced financial difficulties and some of the imaging procedures were not available at our institution. Other diagnoses that were entertained and managed in this case were iron deficiency anemia from peptic ulcer disease. Six weeks after her initial presentation to our institution, the patient had an exploratory laparotomy. The findings intra-operatively were those of a huge tumor involving the ascending colon measuring 16 x14 cm and extending to involve the cecum and mesenteric lymph nodes (Figure 2).



Kidneys, liver and spleen were macroscopically normal. An assessment of Duke’s stage 3C colon cancer was made and she had an extended radical hemicolectomy with anastomosis.

A 44.5-cm long right hemicolectomy segment comprising a 17-cm ileal segment, a 6-cm cecum, 21.5-cm ascending colon, and an 8-cm appendix was removed. The tumor was located in the ascending colon at 7.5 cm from the distal resection margin and extending 1 cm into the cecum. It had a circumference of 27 cm with fibrinous exudates on its peritoneal surface. Dissection revealed uneven circumferential thickening of the bowel wall, luminal dilatation, marked mucosal ulcerations, and liquid content made up of fecal material and necrotic debris. The tumor cut surface was solid white. We also removed 4 lymph nodes. Other uninvolved areas showed focal mucosal hyperemia, but no polyps were observed. Histology showed moderately differentiated adenocarcinoma (pT4) with ¼ nodal involvement (Figure 3).



The patient’s postoperative course was uneventful, and she had adjuvant chemotherapy with oral capecitabine and intravenous oxaliplatin. She completed the 8-cycle protocol with excellent clinical response and minimal adverse events were recorded. A repeat abdominal CT scan showed no residual tumor (Figure 4), and her full blood count showed normal hematological profile with no evidence of iron deficiency.



She is presently on follow up 2 years after confirmation of the diagnosis. (Her histological diagnosis was made June 2016, and her last clinic follow-up was March 2018.
 

 

 

Discussion

Our patient presented with symptoms of abdominal pain, dysuria, melena, and pallor as in other case reports.7-10 A diagnosis of iron deficiency anemia was initially entertained in view of the hematologic profile, and for which management was instituted. The findings of gastric and duodenal erosions on endoscopy further supported the assumption for and treatment of peptic ulcer disease. Iron deficiency in this patient was owing to chronic blood loss from a tumour located at the upper parts of the. Vague and nonspecific symptoms are associated with delayed diagnosis and poor prognosis.1-5,11 Nonspecificity of symptoms is typical feature of colon cancer as reported in other studies.1,11-13 However, the strong family history of colon cancer heightened suspicion in this case, otherwise the diagnosis of an ascending colon tumor could have been delayed until much later and with graver consequences.

The diagnosis of colon cancer in this child was made about a year after her initial symptoms, and 3 months after her presentation to us. Ascending and transverse colon cancers are usually diagnosed late because the symptoms of intestinal obstruction – frank bleeding – will not present until the illness is substantially advanced. Ameh and Nmadu reported a case series of 8 patients from our facility with rectosigmoid tumor, of whom 6 had mucinous adenocarcinaoma and 5 of those 6 had stage 3C disease. Although the patient in the present case had an advanced disease at diagnosis, she had a moderately differentiated histology in contrast to the 6 previously reported cases, who had mucinous histology.14

Previous studies have shown that colorectal carcinoma is a rare disease worldwide, with an annual age-adjusted incidence of 0.38 people/million.1,2 When it occurs in the young, familial or hereditary predisposition should be highly suspected.1-3 To date, there is scant literature on children younger than 16 years in Nigeria.15 Various studies have found a relationship between patients with early-stage colon cancer and inherited genetic predisposition to the disease.2,5 Familial adenomatous polyposis syndrome is an autosomal dominant disorder characterized by the development of polyps during the first decade of life, extensive polyposis in the second decade, and transformation into frank carcinoma in early adulthood.1-5

Although our patient’s mother was diagnosed with and died of colon cancer, the type of which could not be ascertained because her records could not be traced. However, the operative and histological findings in this patient did not suggest the presence of polyposis. The clinical phenotype for the autosomal recessive mismatch repair deficiency includes susceptibity to glioma, leukemia, lymphoma, and colorectal carcinoma in children and young adults.1,5 Screening for genetic markers in the child in the present case might have identified the genetic abnormalities involved and would have been invaluable in the evaluation of her 6 surviving siblings and further management of this family. In conclusion. A high index of suspicion should prompt inclusion of colon cancer in the differential diagnosis of nonspecific gastrointestinal symptoms associated with colon cancer in children.

Acknowledgment

The authors obtained written informed consent from the patient and her elder sibling before writing this report. In addition, the authors thank all the staff involved in the management of this child in the pediatric medical and surgical wards.

References

1. Sultan I, Rodriguez-Galindo C, El-Taani H, Pastore G, Casanova M, Gallino G, Ferrari A. Distinct features of colorectal cancer in children and adolescents. A population-based study of 159 cases. Cancer. 2010;1;116(3):758-65. 
2. Ferrari A. Intestinal carcinomas. In: Schneider DT, Brecht IB, Olson TA, Ferrari A (eds). Rare tumors in children and adolescents. 1st ed. Copyright, Springer-Verlag Berlin Heidelberg; 2012; chap 32. 
3. Hill DA, Furman WL, Bilups CA, Riedly SE, Cain AM, Rao BN. Colorectal carcinoma in childhood and adolescence: a clinicopathological review. J Clin Oncol. 2007;25(36):5808-5814. 
4. Saab OKR, Furman WL. Epidemiology and management options for colorectal cancer in children. Paediatr Drugs. 2008;10(3):177-192. 
5.  Bertario L, Signoroni S. Gastrointestinal cancer predisposition syndromes. In: Schneider DT, Brecht IB, Olson TA, Ferrari A (eds). Rare tumors in children and adolescents. Copyright, Springer-Verlag Berlin Heidelberg; 2012; chap 30. 
6.  Agha RA, Fowler AJ, Saetta A, et al, for the SCARE Group. The SCARE Statement: consensus-based surgical case report guidelines. Int J Surg. 2016;34:180-186. 
7. Tricoli JV, Seibel NL, Blair DG, Albritton K, Hayes-Lattin B. Unique characteristics of adolescent and young adult acute lymphoblastic leukemia, breast cancer, and colon cancer. J Natl Cancer Inst. 2011;103(8):628-635.  
8. Begum M, Khan ZJ, Hassan K, Karim S. Carcinoma colon of a child presenting with abdominal pain. Bangaladesh J Child Health. 2014;38(1):44-47. 
9. Woods R, Larkin JO, Muldoon C, Kennedy MJ, Mehigan B, McCormick P. Metastatic paediatric colorectal carcinoma. Ir Med J. 2012;105(3):88-89. 
10. Bjoernsen LP, Lindsay MB. An unusual case of pediatric abdominal pain. CJEM. 2011;13(2):133-138. 
11. Takalkar UV, Asegaonkar SB, Kulkarni U, Jadhav A, Advani S, Reddy DN. Carcinoma of colon in an adolescent: a case report with review of literature. Int J Sci Rep 2015;1(2):151-3. 
12. Zamir N, Ahmad S, Akhtar J. Mucinous adenocarcinoma of colon. APSP J Case Rep. 2010;1(2):20. 
13. Al-Tonbary Y, Darwish A, El-Hussein A, Fouda A. Adenocarcinoma of the colon in children: case series and mini-review of the literature. Hematol Oncol Stem Cell Ther. 2013;6(1):29-33. 
14. Ameh EA, Nmadu PT. Colorectal adenocarcinoma in children and adolescents: a report of 8 patients from Zaria, Nigeria. West Afr J Med. 2000;19(4):273-276. 
15. Ibrahim, AE, Afolayan KA, Adeniji OM, Buhari KB. Colorectal carcinoma in children and young adults in Ilorin, Nigeria. West Afr J Med. 2011;30(3):202-205.

References

1. Sultan I, Rodriguez-Galindo C, El-Taani H, Pastore G, Casanova M, Gallino G, Ferrari A. Distinct features of colorectal cancer in children and adolescents. A population-based study of 159 cases. Cancer. 2010;1;116(3):758-65. 
2. Ferrari A. Intestinal carcinomas. In: Schneider DT, Brecht IB, Olson TA, Ferrari A (eds). Rare tumors in children and adolescents. 1st ed. Copyright, Springer-Verlag Berlin Heidelberg; 2012; chap 32. 
3. Hill DA, Furman WL, Bilups CA, Riedly SE, Cain AM, Rao BN. Colorectal carcinoma in childhood and adolescence: a clinicopathological review. J Clin Oncol. 2007;25(36):5808-5814. 
4. Saab OKR, Furman WL. Epidemiology and management options for colorectal cancer in children. Paediatr Drugs. 2008;10(3):177-192. 
5.  Bertario L, Signoroni S. Gastrointestinal cancer predisposition syndromes. In: Schneider DT, Brecht IB, Olson TA, Ferrari A (eds). Rare tumors in children and adolescents. Copyright, Springer-Verlag Berlin Heidelberg; 2012; chap 30. 
6.  Agha RA, Fowler AJ, Saetta A, et al, for the SCARE Group. The SCARE Statement: consensus-based surgical case report guidelines. Int J Surg. 2016;34:180-186. 
7. Tricoli JV, Seibel NL, Blair DG, Albritton K, Hayes-Lattin B. Unique characteristics of adolescent and young adult acute lymphoblastic leukemia, breast cancer, and colon cancer. J Natl Cancer Inst. 2011;103(8):628-635.  
8. Begum M, Khan ZJ, Hassan K, Karim S. Carcinoma colon of a child presenting with abdominal pain. Bangaladesh J Child Health. 2014;38(1):44-47. 
9. Woods R, Larkin JO, Muldoon C, Kennedy MJ, Mehigan B, McCormick P. Metastatic paediatric colorectal carcinoma. Ir Med J. 2012;105(3):88-89. 
10. Bjoernsen LP, Lindsay MB. An unusual case of pediatric abdominal pain. CJEM. 2011;13(2):133-138. 
11. Takalkar UV, Asegaonkar SB, Kulkarni U, Jadhav A, Advani S, Reddy DN. Carcinoma of colon in an adolescent: a case report with review of literature. Int J Sci Rep 2015;1(2):151-3. 
12. Zamir N, Ahmad S, Akhtar J. Mucinous adenocarcinoma of colon. APSP J Case Rep. 2010;1(2):20. 
13. Al-Tonbary Y, Darwish A, El-Hussein A, Fouda A. Adenocarcinoma of the colon in children: case series and mini-review of the literature. Hematol Oncol Stem Cell Ther. 2013;6(1):29-33. 
14. Ameh EA, Nmadu PT. Colorectal adenocarcinoma in children and adolescents: a report of 8 patients from Zaria, Nigeria. West Afr J Med. 2000;19(4):273-276. 
15. Ibrahim, AE, Afolayan KA, Adeniji OM, Buhari KB. Colorectal carcinoma in children and young adults in Ilorin, Nigeria. West Afr J Med. 2011;30(3):202-205.

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The Journal of Community and Supportive Oncology - 16(3)
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The Journal of Community and Supportive Oncology - 16(3)
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