IN THIS ARTICLE

• Diagnosis
• Treatment/management
• Physcial signs suggestive of CVID in patients with appropriate history
• Case outcome

A 60-year-old woman with a recent history of air and cruise ship travel presented with symptoms consistent with acute sinusitis. She had a 34–pack-year history of cigarette smoking but had quit at age 50. Her medical history was significant for hypothyroidism, hypertension, coronary artery disease, mild asthma, and COPD. Past surgical history included coronary artery bypass, abdominal hysterectomy, and cholecystectomy. Her medications included inhaled bronchodilators, thyroxin, hydrochlorothiazide, nitrates, ß-blockers, and calcium channel blockers.

Over the next five years, she presented with frequent episodes of respiratory illness for which she received multiple courses of antibiotics, inhaled bronchodilators, and oral as well as inhaled corticosteroids. She consequently became increasingly sensitized to multiple antibiotic classes and was frequently hospitalized for the treatment of her respiratory illnesses.

Common variable immunodeficiency disorders (collectively known as CVID) are the most common clinically significant immunodeficiency diseases among adults.¹ Manifesting clinically as frequent, unusually severe or recalcitrant bacterial infections of the ear, sinus, respiratory tree, and/or gastrointestinal tract, CVID is genetically induced.² Additionally, these disorders can predispose individuals to autoimmune conditions and to cancers involving B lymphocytes.³ Often thought to be a disease of younger people, CVID can occur across the age span.⁴

The immune dysfunction that characterizes CVID is believed to result from underlying genetic defects that affect the differentiation of B cells, leading to faulty immunoglobulin (Ig) synthesis. Recent advances that allow the detection of multiple novel susceptibility loci for CVID have dramatically increased our understanding of the pathophysiology and pathogenesis of this disorder.⁵ These advances are being used to refine the diagnostic parameters of CVID and in the future may help clinicians tailor treatment protocols to specific genetic defects.^5,6

Although considered rare, CVID is often unrecognized; the incidence is likely much higher than the current estimates of 1:10,000 to 1:50,000.⁷ About 13% to 23% of individuals with chronic sinusitis are thought to be affected by CVID.⁸ While it is most commonly diagnosed during the second and third decades of life, it can be diagnosed at any time during the lifespan.⁴ A high burden of disease is associated with this disorder, as hospitalizations and costly, aggressive treatment regimens are needed to manage the resultant bacterial infections and sequelae.²

Increased awareness of CVID among primary care providers is needed to assure prompt diagnosis and to avoid unnecessary complications associated with delayed treatment. The diagnostic workup is complex, and referral to immunology for specific diagnosis and treatment is strongly advised. Recognition is the first step, and primary care providers must include primary immunodeficiency disorders, including CVID, in their differential to avert a missed diagnosis and to ensure optimal treatment.⁹

CLINICAL MANIFESTATIONS/PATIENT HISTORY

Frequent and severe infections are a hallmark of CVID. The most common types of infections seen in CVID are sinusitis, conjunctivitis, otitis media, bronchitis, pneumonia, and gastroenteritis.¹⁰ These primary bacterial infections can disseminate, causing septicemia and/or central nervous system infection.¹¹ The usual infectious pathogens are encapsulated bacteria such as Streptococcus pneumoniae and Haemophilus influenzae, but atypical infections due to organisms such as Pneumocystis carinii and Mycoplasma pneumoniae also occur in some patients.^12,13

Although the majority of CVID cases occur sporadically, family history is helpful in securing the correct diagnosis.¹⁵ Known immunodeficiency, unusual susceptibility to infections, autoimmune diseases, hematologic malignancy, or death caused by infection in other family members should increase the provider’s index of suspicion for CVID.¹⁶

Many genetic defects have been implicated in CVID, yet the wide phenotypic expression found even in persons with similar genetic profiles implies that CVID has a complex genetic transmission pattern.¹⁵ Known or suspected consanguinity in parents or grandparents increases the risk for CVID.⁶

Although these family history elements occur infrequently, they increase the likelihood of severe opportunistic infection, which can cause organ damage or even death.^1,17 Being alert for these elements of family history can help to avoid delays in diagnosis and treatment and eventual organ damage.²

DIFFERENTIAL DIAGNOSIS

When considering the differential diagnosis for the primary features of CVID, other etiologies that should be considered include allergies, environmental exposures, uncontrolled gastroesophageal reflux disease, structural abnormalities of the upper respiratory tract, and celiac disease.^5,10,18,19 Far less common but still worthy of consideration are other genetic conditions, such as primary ciliary dyskinesia, cystic fibrosis, thymic dysfunction or carcinoma, and protein-losing enteropathies.^20,21

A number of conditions can cause immunosuppression. Transient reductions in serum Ig levels can occur in the presence of serious infections.²² Long-term, high-dose use of some medications, such as corticosteroids, or use of anticonvulsants may reduce antibody availability. Chronic illnesses, malignancy, and malnutrition can also play a role in immunosuppression.¹⁹ CVID shares features with a large number of primary immune diseases, and these as well as other causes of hypogammaglobulinemia must be excluded before the diagnosis of CVID can be made.¹

DIAGNOSIS

While infectious disease is a common reason patients seek medical care, few patients presenting with one will have CVID. Nevertheless, immunologic evaluations should be performed and appropriate referral to an immunology specialist is strongly recommended when more than one severe infection arises in a year’s time; when a pattern of severe or unusual infections presents over a period of time; when bronchiectasis is present; or when infections do not resolve with conventional treatment.¹⁶ In addition, the physical findings noted in the Table, when combined with a history of recurrent infections, autoimmune disorders, or lymphocytic malignancy, should prompt evaluation for CVID.^10,16,18,23

The diagnosis of CVID requires testing for low serum levels of total IgG, IgG subclasses, IgA, and IgM. In CVID, IgG and IgA levels will be reduced, and occasionally IgM levels will also be diminished.²⁴ Unless an active infection is present, there will be no change in the patient’s routine blood tests, such as the complete blood count and total complement levels.

The diagnosis is also based on demonstration of a deficient antibody response to protein (tetanus) and polysaccharide (pneumonia) vaccine antigens.²¹ A minimal reaction to these vaccines should prompt referral to an immunology specialist for additional testing and a plan of care.²⁵ However, whenever the index of suspicion for CVID is high, prompt referral to immunology should not be delayed to perform further testing.¹⁶

TREATMENT/MANAGEMENT

IgG replacement therapy, which treats the underlying pathophysiology of CVID by supplementing one of the deficient antibodies, is the standard treatment for CVID. IgG is considered a blood product since it is made from human plasma. Patients may experience untoward reactions to IgG replacement therapy, similar to transfusion reactions; such reactions commonly include back pain, low-grade fever, muscle and joint discomfort, and fatigue. These unpleasant effects can be minimized with the prophylactic use of antihistamines, antipyretics, or even glucocorticoids.²⁶

Although IgG replacement therapy has high upfront costs, it increases patients’ well-being considerably by preventing multiple or recurrent infections and the resultant hospitalizations for antibiotic therapy.²⁷ Home infusion of IgG can minimize costs as well as increase patient autonomy.²⁸ With home infusions, IgG is administered via a multisite subcutaneous route using a slow-infusion mechanical pump. Subcutaneous infusions generally take four to six hours, depending on the number of sites used. Some patients can infuse while they sleep, which increases patient satisfaction with the treatment.²⁷

Infections in persons with CVID can be severe and may lead to organ-system compromise, requiring aggressive therapy aimed at supporting the function of the affected organ systems. For example, patients with CVID can develop unrelenting vomiting and diarrhea, which may require inpatient admission for rehydration and stabilization until the infection can be treated adequately.³²

Treatment options remain limited for the subset of CVID patients who develop severe complications, such as interstitial lung disease or neoplasms. These complications are associated with a significant increase in patient mortality, and allogeneic hematopoietic stem cell transplantation may be indicated for patients who develop them. This potentially curative treatment is being explored in ongoing research trials.³³

PATIENT EDUCATION

Scrupulous hand hygiene, careful avoidance of infectious exposures, watchful food handling and preparation, and lifestyle choices that support good general health are key elements of self-care for patients who have CVID. Preventive measures serve this population well by helping to reduce some of the complications of this serious disease.

Patients with CVID should understand keys aspects regarding its diagnosis, treatment, and prognosis. Specifically, they should know that people who have CVID are born missing some of the body’s immune defenses, which increases their risk for infection, especially of the sinuses, lungs, and gut. Sometimes it takes years to make this diagnosis, because it is a rare cause of common symptoms.

The patient was referred to immunology, and a diagnosis of CVID was made. She was successfully treated with subcutaneous IgG replacement therapy. She died due to overwhelming sepsis after an episode of pneumonia at age 84.

CONCLUSION

The secret to prompt detection of CVID is adding it to the differential diagnosis of recurrent infections. Timely recognition and appropriate referral prevent serious complications, since successful treatment options are available.

Special thanks to Doug Bartelt, DNP, APNP, NP-C.

IN THIS ARTICLE

• Diagnosis
• Treatment/management
• Physcial signs suggestive of CVID in patients with appropriate history
• Case outcome

A 60-year-old woman with a recent history of air and cruise ship travel presented with symptoms consistent with acute sinusitis. She had a 34–pack-year history of cigarette smoking but had quit at age 50. Her medical history was significant for hypothyroidism, hypertension, coronary artery disease, mild asthma, and COPD. Past surgical history included coronary artery bypass, abdominal hysterectomy, and cholecystectomy. Her medications included inhaled bronchodilators, thyroxin, hydrochlorothiazide, nitrates, ß-blockers, and calcium channel blockers.

Over the next five years, she presented with frequent episodes of respiratory illness for which she received multiple courses of antibiotics, inhaled bronchodilators, and oral as well as inhaled corticosteroids. She consequently became increasingly sensitized to multiple antibiotic classes and was frequently hospitalized for the treatment of her respiratory illnesses.

Common variable immunodeficiency disorders (collectively known as CVID) are the most common clinically significant immunodeficiency diseases among adults.¹ Manifesting clinically as frequent, unusually severe or recalcitrant bacterial infections of the ear, sinus, respiratory tree, and/or gastrointestinal tract, CVID is genetically induced.² Additionally, these disorders can predispose individuals to autoimmune conditions and to cancers involving B lymphocytes.³ Often thought to be a disease of younger people, CVID can occur across the age span.⁴

The immune dysfunction that characterizes CVID is believed to result from underlying genetic defects that affect the differentiation of B cells, leading to faulty immunoglobulin (Ig) synthesis. Recent advances that allow the detection of multiple novel susceptibility loci for CVID have dramatically increased our understanding of the pathophysiology and pathogenesis of this disorder.⁵ These advances are being used to refine the diagnostic parameters of CVID and in the future may help clinicians tailor treatment protocols to specific genetic defects.^5,6

Although considered rare, CVID is often unrecognized; the incidence is likely much higher than the current estimates of 1:10,000 to 1:50,000.⁷ About 13% to 23% of individuals with chronic sinusitis are thought to be affected by CVID.⁸ While it is most commonly diagnosed during the second and third decades of life, it can be diagnosed at any time during the lifespan.⁴ A high burden of disease is associated with this disorder, as hospitalizations and costly, aggressive treatment regimens are needed to manage the resultant bacterial infections and sequelae.²

Increased awareness of CVID among primary care providers is needed to assure prompt diagnosis and to avoid unnecessary complications associated with delayed treatment. The diagnostic workup is complex, and referral to immunology for specific diagnosis and treatment is strongly advised. Recognition is the first step, and primary care providers must include primary immunodeficiency disorders, including CVID, in their differential to avert a missed diagnosis and to ensure optimal treatment.⁹

CLINICAL MANIFESTATIONS/PATIENT HISTORY

Frequent and severe infections are a hallmark of CVID. The most common types of infections seen in CVID are sinusitis, conjunctivitis, otitis media, bronchitis, pneumonia, and gastroenteritis.¹⁰ These primary bacterial infections can disseminate, causing septicemia and/or central nervous system infection.¹¹ The usual infectious pathogens are encapsulated bacteria such as Streptococcus pneumoniae and Haemophilus influenzae, but atypical infections due to organisms such as Pneumocystis carinii and Mycoplasma pneumoniae also occur in some patients.^12,13

Although the majority of CVID cases occur sporadically, family history is helpful in securing the correct diagnosis.¹⁵ Known immunodeficiency, unusual susceptibility to infections, autoimmune diseases, hematologic malignancy, or death caused by infection in other family members should increase the provider’s index of suspicion for CVID.¹⁶

Many genetic defects have been implicated in CVID, yet the wide phenotypic expression found even in persons with similar genetic profiles implies that CVID has a complex genetic transmission pattern.¹⁵ Known or suspected consanguinity in parents or grandparents increases the risk for CVID.⁶

Although these family history elements occur infrequently, they increase the likelihood of severe opportunistic infection, which can cause organ damage or even death.^1,17 Being alert for these elements of family history can help to avoid delays in diagnosis and treatment and eventual organ damage.²

DIFFERENTIAL DIAGNOSIS

When considering the differential diagnosis for the primary features of CVID, other etiologies that should be considered include allergies, environmental exposures, uncontrolled gastroesophageal reflux disease, structural abnormalities of the upper respiratory tract, and celiac disease.^5,10,18,19 Far less common but still worthy of consideration are other genetic conditions, such as primary ciliary dyskinesia, cystic fibrosis, thymic dysfunction or carcinoma, and protein-losing enteropathies.^20,21

A number of conditions can cause immunosuppression. Transient reductions in serum Ig levels can occur in the presence of serious infections.²² Long-term, high-dose use of some medications, such as corticosteroids, or use of anticonvulsants may reduce antibody availability. Chronic illnesses, malignancy, and malnutrition can also play a role in immunosuppression.¹⁹ CVID shares features with a large number of primary immune diseases, and these as well as other causes of hypogammaglobulinemia must be excluded before the diagnosis of CVID can be made.¹

DIAGNOSIS

While infectious disease is a common reason patients seek medical care, few patients presenting with one will have CVID. Nevertheless, immunologic evaluations should be performed and appropriate referral to an immunology specialist is strongly recommended when more than one severe infection arises in a year’s time; when a pattern of severe or unusual infections presents over a period of time; when bronchiectasis is present; or when infections do not resolve with conventional treatment.¹⁶ In addition, the physical findings noted in the Table, when combined with a history of recurrent infections, autoimmune disorders, or lymphocytic malignancy, should prompt evaluation for CVID.^10,16,18,23

The diagnosis of CVID requires testing for low serum levels of total IgG, IgG subclasses, IgA, and IgM. In CVID, IgG and IgA levels will be reduced, and occasionally IgM levels will also be diminished.²⁴ Unless an active infection is present, there will be no change in the patient’s routine blood tests, such as the complete blood count and total complement levels.

The diagnosis is also based on demonstration of a deficient antibody response to protein (tetanus) and polysaccharide (pneumonia) vaccine antigens.²¹ A minimal reaction to these vaccines should prompt referral to an immunology specialist for additional testing and a plan of care.²⁵ However, whenever the index of suspicion for CVID is high, prompt referral to immunology should not be delayed to perform further testing.¹⁶

TREATMENT/MANAGEMENT

IgG replacement therapy, which treats the underlying pathophysiology of CVID by supplementing one of the deficient antibodies, is the standard treatment for CVID. IgG is considered a blood product since it is made from human plasma. Patients may experience untoward reactions to IgG replacement therapy, similar to transfusion reactions; such reactions commonly include back pain, low-grade fever, muscle and joint discomfort, and fatigue. These unpleasant effects can be minimized with the prophylactic use of antihistamines, antipyretics, or even glucocorticoids.²⁶

Although IgG replacement therapy has high upfront costs, it increases patients’ well-being considerably by preventing multiple or recurrent infections and the resultant hospitalizations for antibiotic therapy.²⁷ Home infusion of IgG can minimize costs as well as increase patient autonomy.²⁸ With home infusions, IgG is administered via a multisite subcutaneous route using a slow-infusion mechanical pump. Subcutaneous infusions generally take four to six hours, depending on the number of sites used. Some patients can infuse while they sleep, which increases patient satisfaction with the treatment.²⁷

Infections in persons with CVID can be severe and may lead to organ-system compromise, requiring aggressive therapy aimed at supporting the function of the affected organ systems. For example, patients with CVID can develop unrelenting vomiting and diarrhea, which may require inpatient admission for rehydration and stabilization until the infection can be treated adequately.³²

Treatment options remain limited for the subset of CVID patients who develop severe complications, such as interstitial lung disease or neoplasms. These complications are associated with a significant increase in patient mortality, and allogeneic hematopoietic stem cell transplantation may be indicated for patients who develop them. This potentially curative treatment is being explored in ongoing research trials.³³

PATIENT EDUCATION

Scrupulous hand hygiene, careful avoidance of infectious exposures, watchful food handling and preparation, and lifestyle choices that support good general health are key elements of self-care for patients who have CVID. Preventive measures serve this population well by helping to reduce some of the complications of this serious disease.

Patients with CVID should understand keys aspects regarding its diagnosis, treatment, and prognosis. Specifically, they should know that people who have CVID are born missing some of the body’s immune defenses, which increases their risk for infection, especially of the sinuses, lungs, and gut. Sometimes it takes years to make this diagnosis, because it is a rare cause of common symptoms.

The patient was referred to immunology, and a diagnosis of CVID was made. She was successfully treated with subcutaneous IgG replacement therapy. She died due to overwhelming sepsis after an episode of pneumonia at age 84.

CONCLUSION

The secret to prompt detection of CVID is adding it to the differential diagnosis of recurrent infections. Timely recognition and appropriate referral prevent serious complications, since successful treatment options are available.

Special thanks to Doug Bartelt, DNP, APNP, NP-C.

IN THIS ARTICLE

• Diagnosis
• Treatment/management
• Physcial signs suggestive of CVID in patients with appropriate history
• Case outcome

A 60-year-old woman with a recent history of air and cruise ship travel presented with symptoms consistent with acute sinusitis. She had a 34–pack-year history of cigarette smoking but had quit at age 50. Her medical history was significant for hypothyroidism, hypertension, coronary artery disease, mild asthma, and COPD. Past surgical history included coronary artery bypass, abdominal hysterectomy, and cholecystectomy. Her medications included inhaled bronchodilators, thyroxin, hydrochlorothiazide, nitrates, ß-blockers, and calcium channel blockers.

Over the next five years, she presented with frequent episodes of respiratory illness for which she received multiple courses of antibiotics, inhaled bronchodilators, and oral as well as inhaled corticosteroids. She consequently became increasingly sensitized to multiple antibiotic classes and was frequently hospitalized for the treatment of her respiratory illnesses.

Common variable immunodeficiency disorders (collectively known as CVID) are the most common clinically significant immunodeficiency diseases among adults.¹ Manifesting clinically as frequent, unusually severe or recalcitrant bacterial infections of the ear, sinus, respiratory tree, and/or gastrointestinal tract, CVID is genetically induced.² Additionally, these disorders can predispose individuals to autoimmune conditions and to cancers involving B lymphocytes.³ Often thought to be a disease of younger people, CVID can occur across the age span.⁴

The immune dysfunction that characterizes CVID is believed to result from underlying genetic defects that affect the differentiation of B cells, leading to faulty immunoglobulin (Ig) synthesis. Recent advances that allow the detection of multiple novel susceptibility loci for CVID have dramatically increased our understanding of the pathophysiology and pathogenesis of this disorder.⁵ These advances are being used to refine the diagnostic parameters of CVID and in the future may help clinicians tailor treatment protocols to specific genetic defects.^5,6

Although considered rare, CVID is often unrecognized; the incidence is likely much higher than the current estimates of 1:10,000 to 1:50,000.⁷ About 13% to 23% of individuals with chronic sinusitis are thought to be affected by CVID.⁸ While it is most commonly diagnosed during the second and third decades of life, it can be diagnosed at any time during the lifespan.⁴ A high burden of disease is associated with this disorder, as hospitalizations and costly, aggressive treatment regimens are needed to manage the resultant bacterial infections and sequelae.²

Increased awareness of CVID among primary care providers is needed to assure prompt diagnosis and to avoid unnecessary complications associated with delayed treatment. The diagnostic workup is complex, and referral to immunology for specific diagnosis and treatment is strongly advised. Recognition is the first step, and primary care providers must include primary immunodeficiency disorders, including CVID, in their differential to avert a missed diagnosis and to ensure optimal treatment.⁹

CLINICAL MANIFESTATIONS/PATIENT HISTORY

Frequent and severe infections are a hallmark of CVID. The most common types of infections seen in CVID are sinusitis, conjunctivitis, otitis media, bronchitis, pneumonia, and gastroenteritis.¹⁰ These primary bacterial infections can disseminate, causing septicemia and/or central nervous system infection.¹¹ The usual infectious pathogens are encapsulated bacteria such as Streptococcus pneumoniae and Haemophilus influenzae, but atypical infections due to organisms such as Pneumocystis carinii and Mycoplasma pneumoniae also occur in some patients.^12,13

Although the majority of CVID cases occur sporadically, family history is helpful in securing the correct diagnosis.¹⁵ Known immunodeficiency, unusual susceptibility to infections, autoimmune diseases, hematologic malignancy, or death caused by infection in other family members should increase the provider’s index of suspicion for CVID.¹⁶

Many genetic defects have been implicated in CVID, yet the wide phenotypic expression found even in persons with similar genetic profiles implies that CVID has a complex genetic transmission pattern.¹⁵ Known or suspected consanguinity in parents or grandparents increases the risk for CVID.⁶

Although these family history elements occur infrequently, they increase the likelihood of severe opportunistic infection, which can cause organ damage or even death.^1,17 Being alert for these elements of family history can help to avoid delays in diagnosis and treatment and eventual organ damage.²

DIFFERENTIAL DIAGNOSIS

When considering the differential diagnosis for the primary features of CVID, other etiologies that should be considered include allergies, environmental exposures, uncontrolled gastroesophageal reflux disease, structural abnormalities of the upper respiratory tract, and celiac disease.^5,10,18,19 Far less common but still worthy of consideration are other genetic conditions, such as primary ciliary dyskinesia, cystic fibrosis, thymic dysfunction or carcinoma, and protein-losing enteropathies.^20,21

A number of conditions can cause immunosuppression. Transient reductions in serum Ig levels can occur in the presence of serious infections.²² Long-term, high-dose use of some medications, such as corticosteroids, or use of anticonvulsants may reduce antibody availability. Chronic illnesses, malignancy, and malnutrition can also play a role in immunosuppression.¹⁹ CVID shares features with a large number of primary immune diseases, and these as well as other causes of hypogammaglobulinemia must be excluded before the diagnosis of CVID can be made.¹

DIAGNOSIS

While infectious disease is a common reason patients seek medical care, few patients presenting with one will have CVID. Nevertheless, immunologic evaluations should be performed and appropriate referral to an immunology specialist is strongly recommended when more than one severe infection arises in a year’s time; when a pattern of severe or unusual infections presents over a period of time; when bronchiectasis is present; or when infections do not resolve with conventional treatment.¹⁶ In addition, the physical findings noted in the Table, when combined with a history of recurrent infections, autoimmune disorders, or lymphocytic malignancy, should prompt evaluation for CVID.^10,16,18,23

The diagnosis of CVID requires testing for low serum levels of total IgG, IgG subclasses, IgA, and IgM. In CVID, IgG and IgA levels will be reduced, and occasionally IgM levels will also be diminished.²⁴ Unless an active infection is present, there will be no change in the patient’s routine blood tests, such as the complete blood count and total complement levels.

The diagnosis is also based on demonstration of a deficient antibody response to protein (tetanus) and polysaccharide (pneumonia) vaccine antigens.²¹ A minimal reaction to these vaccines should prompt referral to an immunology specialist for additional testing and a plan of care.²⁵ However, whenever the index of suspicion for CVID is high, prompt referral to immunology should not be delayed to perform further testing.¹⁶

TREATMENT/MANAGEMENT

IgG replacement therapy, which treats the underlying pathophysiology of CVID by supplementing one of the deficient antibodies, is the standard treatment for CVID. IgG is considered a blood product since it is made from human plasma. Patients may experience untoward reactions to IgG replacement therapy, similar to transfusion reactions; such reactions commonly include back pain, low-grade fever, muscle and joint discomfort, and fatigue. These unpleasant effects can be minimized with the prophylactic use of antihistamines, antipyretics, or even glucocorticoids.²⁶

Although IgG replacement therapy has high upfront costs, it increases patients’ well-being considerably by preventing multiple or recurrent infections and the resultant hospitalizations for antibiotic therapy.²⁷ Home infusion of IgG can minimize costs as well as increase patient autonomy.²⁸ With home infusions, IgG is administered via a multisite subcutaneous route using a slow-infusion mechanical pump. Subcutaneous infusions generally take four to six hours, depending on the number of sites used. Some patients can infuse while they sleep, which increases patient satisfaction with the treatment.²⁷

Infections in persons with CVID can be severe and may lead to organ-system compromise, requiring aggressive therapy aimed at supporting the function of the affected organ systems. For example, patients with CVID can develop unrelenting vomiting and diarrhea, which may require inpatient admission for rehydration and stabilization until the infection can be treated adequately.³²

Treatment options remain limited for the subset of CVID patients who develop severe complications, such as interstitial lung disease or neoplasms. These complications are associated with a significant increase in patient mortality, and allogeneic hematopoietic stem cell transplantation may be indicated for patients who develop them. This potentially curative treatment is being explored in ongoing research trials.³³

PATIENT EDUCATION

Scrupulous hand hygiene, careful avoidance of infectious exposures, watchful food handling and preparation, and lifestyle choices that support good general health are key elements of self-care for patients who have CVID. Preventive measures serve this population well by helping to reduce some of the complications of this serious disease.

Patients with CVID should understand keys aspects regarding its diagnosis, treatment, and prognosis. Specifically, they should know that people who have CVID are born missing some of the body’s immune defenses, which increases their risk for infection, especially of the sinuses, lungs, and gut. Sometimes it takes years to make this diagnosis, because it is a rare cause of common symptoms.

The patient was referred to immunology, and a diagnosis of CVID was made. She was successfully treated with subcutaneous IgG replacement therapy. She died due to overwhelming sepsis after an episode of pneumonia at age 84.

CONCLUSION

The secret to prompt detection of CVID is adding it to the differential diagnosis of recurrent infections. Timely recognition and appropriate referral prevent serious complications, since successful treatment options are available.

Special thanks to Doug Bartelt, DNP, APNP, NP-C.

The Trump administration has drafted a plan that would allow more employers to opt out of offering no-cost contraception coverage to women based on religious or moral grounds, according to a leaked copy of the interim rule.

The proposed rule, first obtained by the media outlet Vox, would greatly expand the religious exemption under the Affordable Care Act for employers otherwise subject to the ACA’s contraception mandate. If approved, the rule would allow additional entities and employers that object to the mandate for religious or moral reasons to be exempt from providing coverage. The exception would apply to plans sponsored by objecting employers, whether or not they operate as a nonprofit, according to the leaked rule.

[email protected]

On Twitter @legal_med

The Trump administration has drafted a plan that would allow more employers to opt out of offering no-cost contraception coverage to women based on religious or moral grounds, according to a leaked copy of the interim rule.

The proposed rule, first obtained by the media outlet Vox, would greatly expand the religious exemption under the Affordable Care Act for employers otherwise subject to the ACA’s contraception mandate. If approved, the rule would allow additional entities and employers that object to the mandate for religious or moral reasons to be exempt from providing coverage. The exception would apply to plans sponsored by objecting employers, whether or not they operate as a nonprofit, according to the leaked rule.

[email protected]

On Twitter @legal_med

The Trump administration has drafted a plan that would allow more employers to opt out of offering no-cost contraception coverage to women based on religious or moral grounds, according to a leaked copy of the interim rule.

The proposed rule, first obtained by the media outlet Vox, would greatly expand the religious exemption under the Affordable Care Act for employers otherwise subject to the ACA’s contraception mandate. If approved, the rule would allow additional entities and employers that object to the mandate for religious or moral reasons to be exempt from providing coverage. The exception would apply to plans sponsored by objecting employers, whether or not they operate as a nonprofit, according to the leaked rule.

[email protected]

On Twitter @legal_med

VANCOUVER – Investigators at McMaster University in Hamilton, Ontario, are zeroing in on a biomarker blood test panel to diagnose endometriosis without surgery.

At the World Congress on Endometriosis, they described a decision tree incorporating blood levels of brain-derived neurotrophic factor (BDNF), glycodelin, and zinc-alpha₂-glycoprotein (ZAG), quantified by enzyme-linked immunosorbent assay. ZAG levels above 91.58 ng/mL – the first cut in the decision tree, glycodelin above 39.19 ng/mL – the second cut, and BDNF above 953.9 pg/mL identified endometriosis with a sensitivity of 89.2% and a specificity of 70.0%. The combination outperformed any biomarker on its own.

BDNF is a protein involved in neurogenesis, angiogenesis, and apoptosis resistance, all hallmark pathogenic features of endometriosis. Glycodelin and ZAG are associated with secretory endometrium.

M. Alexander Otto/Frontline Medical News

[email protected]

VANCOUVER – Investigators at McMaster University in Hamilton, Ontario, are zeroing in on a biomarker blood test panel to diagnose endometriosis without surgery.

At the World Congress on Endometriosis, they described a decision tree incorporating blood levels of brain-derived neurotrophic factor (BDNF), glycodelin, and zinc-alpha₂-glycoprotein (ZAG), quantified by enzyme-linked immunosorbent assay. ZAG levels above 91.58 ng/mL – the first cut in the decision tree, glycodelin above 39.19 ng/mL – the second cut, and BDNF above 953.9 pg/mL identified endometriosis with a sensitivity of 89.2% and a specificity of 70.0%. The combination outperformed any biomarker on its own.

BDNF is a protein involved in neurogenesis, angiogenesis, and apoptosis resistance, all hallmark pathogenic features of endometriosis. Glycodelin and ZAG are associated with secretory endometrium.

M. Alexander Otto/Frontline Medical News

[email protected]

VANCOUVER – Investigators at McMaster University in Hamilton, Ontario, are zeroing in on a biomarker blood test panel to diagnose endometriosis without surgery.

At the World Congress on Endometriosis, they described a decision tree incorporating blood levels of brain-derived neurotrophic factor (BDNF), glycodelin, and zinc-alpha₂-glycoprotein (ZAG), quantified by enzyme-linked immunosorbent assay. ZAG levels above 91.58 ng/mL – the first cut in the decision tree, glycodelin above 39.19 ng/mL – the second cut, and BDNF above 953.9 pg/mL identified endometriosis with a sensitivity of 89.2% and a specificity of 70.0%. The combination outperformed any biomarker on its own.

BDNF is a protein involved in neurogenesis, angiogenesis, and apoptosis resistance, all hallmark pathogenic features of endometriosis. Glycodelin and ZAG are associated with secretory endometrium.

M. Alexander Otto/Frontline Medical News

[email protected]

Up to 80% of women who are of reproductive age have uterine fibroids that cause heavy and prolonged bleeding.¹ Additional concerns include infertility and pain, says James Simon, MD, Clinical Professor at George Washington University in Washington, DC. Black women are more likely than white women to undergo hysterectomy for uterine fibroids. They also are more likely to experience severe or very severe symptoms from fibroids and report that these symptoms interfere with physical activities, relationships, and work.²

Current medical treatments include on- and off-label use of oral contraceptives, gonadotropin-releasing hormone (GnRH) receptor agonists, and progestins. Data on investigational oral GnRH antagonists and oral selective progesterone-receptor modulators (SPRMs) were presented at the 2017 Annual Clinical and Scientific Meeting of the American College of Obstetricians and Gynecologists.

Details of VENUS I

In the phase 3, randomized, controlled VENUS I trial, investigators assessed the efficacy and safety of ulipristal acetate (UPA), an SPRM, by race and body mass index (BMI) in premenopausal women (aged 18 to 50) with symptomatic uterine fibroids.¹

Simon and colleagues randomly assigned participants to UPA 5 mg, UPA 10 mg, or placebo once daily for 12 weeks, followed by a 12-week treatment-free follow-up period. UPA, at 5 and 10 mg, was significantly more efficacious than placebo in rate of and time to amenorrhea (P<.0001). The superiority was observed regardless of race and BMI. In addition, women taking UPA versus placebo reported significantly less impact on activities due to uterine fibroids. The study authors concluded that “UPA treatment provides effective control of bleeding and improvement in physical and social activities for women with symptomatic uterine fibroids, regardless of race and BMI.”¹

Establishing the ideal treatment period for UPA to avoid progesterone-associated endometrial changes (in this study it was 12 weeks of once-daily therapy) is a current goal, said Simon.

Details of elagolix phase 2b study

Simon also presented data on another investigational drug, elagolix, an orally administered GnRH antagonist, which has an inhibiting effect on luteinizing hormone and follicle-stimulating hormone secretion. This in turn reduces production of estradiol and progesterone. Elagolix also is being studied for the treatment of endometriosis.³

In the uterine fibroids study, a 24-week, multicenter, double-blind, randomized controlled, parallel group trial, 567 premenopausal women aged 18 to 51 with heavy menstrual bleeding of >80 mL of blood loss were assigned to placebo or elagolix 300 mg twice per day or 600 mg once per day alone or in combination with add-back therapy (with estradiol/norethindrone acetate) to prevent bone loss and menopausal symptoms.⁴

Compared with placebo, women treated with elagolix with or without add-back therapy had significant reductions from baseline in mean menstrual blood loss. Women treated with elagolix also had significant increases in hemoglobin concentration from baseline to month 6 compared with placebo.⁴

Adverse effects were similar to menopause symptoms: bone loss, hot flashes, night sweats, headaches, and disturbed sleep, said Simon. With the add-back treatments, adverse effects were mitigated in a dose-dependent fashion, he pointed out, and were most likely tolerable compared with the heavy bleeding experienced by women at the start of the study.

Up to 80% of women who are of reproductive age have uterine fibroids that cause heavy and prolonged bleeding.¹ Additional concerns include infertility and pain, says James Simon, MD, Clinical Professor at George Washington University in Washington, DC. Black women are more likely than white women to undergo hysterectomy for uterine fibroids. They also are more likely to experience severe or very severe symptoms from fibroids and report that these symptoms interfere with physical activities, relationships, and work.²

Current medical treatments include on- and off-label use of oral contraceptives, gonadotropin-releasing hormone (GnRH) receptor agonists, and progestins. Data on investigational oral GnRH antagonists and oral selective progesterone-receptor modulators (SPRMs) were presented at the 2017 Annual Clinical and Scientific Meeting of the American College of Obstetricians and Gynecologists.

Details of VENUS I

In the phase 3, randomized, controlled VENUS I trial, investigators assessed the efficacy and safety of ulipristal acetate (UPA), an SPRM, by race and body mass index (BMI) in premenopausal women (aged 18 to 50) with symptomatic uterine fibroids.¹

Simon and colleagues randomly assigned participants to UPA 5 mg, UPA 10 mg, or placebo once daily for 12 weeks, followed by a 12-week treatment-free follow-up period. UPA, at 5 and 10 mg, was significantly more efficacious than placebo in rate of and time to amenorrhea (P<.0001). The superiority was observed regardless of race and BMI. In addition, women taking UPA versus placebo reported significantly less impact on activities due to uterine fibroids. The study authors concluded that “UPA treatment provides effective control of bleeding and improvement in physical and social activities for women with symptomatic uterine fibroids, regardless of race and BMI.”¹

Establishing the ideal treatment period for UPA to avoid progesterone-associated endometrial changes (in this study it was 12 weeks of once-daily therapy) is a current goal, said Simon.

Details of elagolix phase 2b study

Simon also presented data on another investigational drug, elagolix, an orally administered GnRH antagonist, which has an inhibiting effect on luteinizing hormone and follicle-stimulating hormone secretion. This in turn reduces production of estradiol and progesterone. Elagolix also is being studied for the treatment of endometriosis.³

In the uterine fibroids study, a 24-week, multicenter, double-blind, randomized controlled, parallel group trial, 567 premenopausal women aged 18 to 51 with heavy menstrual bleeding of >80 mL of blood loss were assigned to placebo or elagolix 300 mg twice per day or 600 mg once per day alone or in combination with add-back therapy (with estradiol/norethindrone acetate) to prevent bone loss and menopausal symptoms.⁴

Compared with placebo, women treated with elagolix with or without add-back therapy had significant reductions from baseline in mean menstrual blood loss. Women treated with elagolix also had significant increases in hemoglobin concentration from baseline to month 6 compared with placebo.⁴

Adverse effects were similar to menopause symptoms: bone loss, hot flashes, night sweats, headaches, and disturbed sleep, said Simon. With the add-back treatments, adverse effects were mitigated in a dose-dependent fashion, he pointed out, and were most likely tolerable compared with the heavy bleeding experienced by women at the start of the study.

Up to 80% of women who are of reproductive age have uterine fibroids that cause heavy and prolonged bleeding.¹ Additional concerns include infertility and pain, says James Simon, MD, Clinical Professor at George Washington University in Washington, DC. Black women are more likely than white women to undergo hysterectomy for uterine fibroids. They also are more likely to experience severe or very severe symptoms from fibroids and report that these symptoms interfere with physical activities, relationships, and work.²

Current medical treatments include on- and off-label use of oral contraceptives, gonadotropin-releasing hormone (GnRH) receptor agonists, and progestins. Data on investigational oral GnRH antagonists and oral selective progesterone-receptor modulators (SPRMs) were presented at the 2017 Annual Clinical and Scientific Meeting of the American College of Obstetricians and Gynecologists.

Details of VENUS I

In the phase 3, randomized, controlled VENUS I trial, investigators assessed the efficacy and safety of ulipristal acetate (UPA), an SPRM, by race and body mass index (BMI) in premenopausal women (aged 18 to 50) with symptomatic uterine fibroids.¹

Simon and colleagues randomly assigned participants to UPA 5 mg, UPA 10 mg, or placebo once daily for 12 weeks, followed by a 12-week treatment-free follow-up period. UPA, at 5 and 10 mg, was significantly more efficacious than placebo in rate of and time to amenorrhea (P<.0001). The superiority was observed regardless of race and BMI. In addition, women taking UPA versus placebo reported significantly less impact on activities due to uterine fibroids. The study authors concluded that “UPA treatment provides effective control of bleeding and improvement in physical and social activities for women with symptomatic uterine fibroids, regardless of race and BMI.”¹

Establishing the ideal treatment period for UPA to avoid progesterone-associated endometrial changes (in this study it was 12 weeks of once-daily therapy) is a current goal, said Simon.

Details of elagolix phase 2b study

Simon also presented data on another investigational drug, elagolix, an orally administered GnRH antagonist, which has an inhibiting effect on luteinizing hormone and follicle-stimulating hormone secretion. This in turn reduces production of estradiol and progesterone. Elagolix also is being studied for the treatment of endometriosis.³

In the uterine fibroids study, a 24-week, multicenter, double-blind, randomized controlled, parallel group trial, 567 premenopausal women aged 18 to 51 with heavy menstrual bleeding of >80 mL of blood loss were assigned to placebo or elagolix 300 mg twice per day or 600 mg once per day alone or in combination with add-back therapy (with estradiol/norethindrone acetate) to prevent bone loss and menopausal symptoms.⁴

Compared with placebo, women treated with elagolix with or without add-back therapy had significant reductions from baseline in mean menstrual blood loss. Women treated with elagolix also had significant increases in hemoglobin concentration from baseline to month 6 compared with placebo.⁴

Adverse effects were similar to menopause symptoms: bone loss, hot flashes, night sweats, headaches, and disturbed sleep, said Simon. With the add-back treatments, adverse effects were mitigated in a dose-dependent fashion, he pointed out, and were most likely tolerable compared with the heavy bleeding experienced by women at the start of the study.

The total number of minimally invasive cosmetic procedures topped 15 million for the first time in 2016, with onabotulinumtoxinA injections leading the way, according to the American Society of Plastic Surgeons.

An estimated 15.4 million minimally invasive cosmetic procedures were performed last year, with onabotulinumtoxinA making up over 45% – approximately 7.1 million anatomic sites injected – of that total, ASPS data show.

Next in popularity was injection of soft tissue fillers, with just over 2.6 million procedures – almost 17% of the procedures performed in 2016 – followed by chemical peels with almost 1.4 million procedures, laser hair removal with 1.1 million procedures, and microdermabrasion at 775,000, the ASPS reported.

The total number of minimally invasive procedures was up 3% from the 14.96 million performed in 2015, while onabotulinumtoxinA was up 4%, soft tissue fillers were up 2%, chemical peels were up 4%, laser hair removal was down 1%, and microdermabrasion was down 3%. Larger changes were seen among some less common procedures: injection lipolysis was up 18% (55,660 procedures in 2016), fat injection was up 13% (79,208 procedures in 2016), and acellular dermal matrix procedures were down 18% (7,809 in 2016), according to the ASPS Tracking Operations and Outcomes for Plastic Surgeons database and an annual survey of board-certified dermatologists, otolaryngologists, and plastic surgeons (final sample = 703).

The total number of minimally invasive cosmetic procedures topped 15 million for the first time in 2016, with onabotulinumtoxinA injections leading the way, according to the American Society of Plastic Surgeons.

An estimated 15.4 million minimally invasive cosmetic procedures were performed last year, with onabotulinumtoxinA making up over 45% – approximately 7.1 million anatomic sites injected – of that total, ASPS data show.

Next in popularity was injection of soft tissue fillers, with just over 2.6 million procedures – almost 17% of the procedures performed in 2016 – followed by chemical peels with almost 1.4 million procedures, laser hair removal with 1.1 million procedures, and microdermabrasion at 775,000, the ASPS reported.

The total number of minimally invasive procedures was up 3% from the 14.96 million performed in 2015, while onabotulinumtoxinA was up 4%, soft tissue fillers were up 2%, chemical peels were up 4%, laser hair removal was down 1%, and microdermabrasion was down 3%. Larger changes were seen among some less common procedures: injection lipolysis was up 18% (55,660 procedures in 2016), fat injection was up 13% (79,208 procedures in 2016), and acellular dermal matrix procedures were down 18% (7,809 in 2016), according to the ASPS Tracking Operations and Outcomes for Plastic Surgeons database and an annual survey of board-certified dermatologists, otolaryngologists, and plastic surgeons (final sample = 703).

The total number of minimally invasive cosmetic procedures topped 15 million for the first time in 2016, with onabotulinumtoxinA injections leading the way, according to the American Society of Plastic Surgeons.

An estimated 15.4 million minimally invasive cosmetic procedures were performed last year, with onabotulinumtoxinA making up over 45% – approximately 7.1 million anatomic sites injected – of that total, ASPS data show.

Next in popularity was injection of soft tissue fillers, with just over 2.6 million procedures – almost 17% of the procedures performed in 2016 – followed by chemical peels with almost 1.4 million procedures, laser hair removal with 1.1 million procedures, and microdermabrasion at 775,000, the ASPS reported.

The total number of minimally invasive procedures was up 3% from the 14.96 million performed in 2015, while onabotulinumtoxinA was up 4%, soft tissue fillers were up 2%, chemical peels were up 4%, laser hair removal was down 1%, and microdermabrasion was down 3%. Larger changes were seen among some less common procedures: injection lipolysis was up 18% (55,660 procedures in 2016), fat injection was up 13% (79,208 procedures in 2016), and acellular dermal matrix procedures were down 18% (7,809 in 2016), according to the ASPS Tracking Operations and Outcomes for Plastic Surgeons database and an annual survey of board-certified dermatologists, otolaryngologists, and plastic surgeons (final sample = 703).

Several state regulations governing the sales or use of e-cigarettes and related products were associated with lower proportions of youth trying or regularly using vaping products, a new study found.

Restricting sales of electronic vapor products to minors, however, was not linked to a lower risk of vaping among teens.

Thinkstockphotos

• Statewide prohibition of vaping products on school property or in workplaces, which includes Arizona, New Hampshire, Vermont, and Virginia for schools and North Dakota for workplaces.

• Prohibition of sales to minors under age 18 years, present in 24 states.

• Prohibition or restriction of sales of e-cigarette products from vending machines, present in 17 states.

• Prohibition or restriction of self-service displays of vaping products, present in 11 states.

• Prohibition or restriction of sampling of electronic vapor products, present in Arizona, Delaware, Kentucky, Maryland, New Hampshire, North Carolina, Oklahoma, and South Carolina.

For most of the regulations, teens had a reduced likelihood of trying or currently using vaping products after adjusting for age, ethnicity, grade level, race, region, and sex. Risk of ever trying a vaping product was 12% lower in states that prohibited their use on school grounds or in workplaces, 6% lower in states that barred sales to those under age 18, and 7% lower in states that restricted or prohibited self-service vaping displays.

The risk of youth currently using electronic vapor products was 5% lower in states with the school grounds and workplace restrictions, and 13% lower in states that restricted self-service displays. Laws restricting minor sales were unrelated to the risk of current vaping among youth. Restricting vending machine sales of vaping products had no association with the risk of a teen ever trying vaping, but it was linked to a 7% lower risk of current use of the products among teens. All these associations were statistically significant based on confidence interval values.

Interestingly, a statistically significant risk increase in vaping use occurred for teens in states that restricted or outlawed sampling of vaping products. The risk was 8% higher for ever trying a product and 20% higher for current use. But those findings also could indicate the possibility of reverse causation.

“It’s possible that states that were particularly concerned about sampling had the worst problems – were the ones more likely to institute a ban on that practice, and that would generate the counterintuitive finding,” Dr. Keim said in an interview. “With the data currently available, we can’t look at teen use both before and after the restrictions, just afterwards, but with more data for 2017, it would provide a clearer picture of all of the associations we examined.”

Aside from these laws, other interventions have the potential to reduce vaping among teens as well.

“Restrictions on use in various types of public places and on school grounds may be additional helpful approaches, similar to what has been done with cigarettes,” Dr. Keim said. Although their analysis included laws that prohibited use on school grounds, only four states have one of these laws.

Several state regulations governing the sales or use of e-cigarettes and related products were associated with lower proportions of youth trying or regularly using vaping products, a new study found.

Restricting sales of electronic vapor products to minors, however, was not linked to a lower risk of vaping among teens.

Thinkstockphotos

• Statewide prohibition of vaping products on school property or in workplaces, which includes Arizona, New Hampshire, Vermont, and Virginia for schools and North Dakota for workplaces.

• Prohibition of sales to minors under age 18 years, present in 24 states.

• Prohibition or restriction of sales of e-cigarette products from vending machines, present in 17 states.

• Prohibition or restriction of self-service displays of vaping products, present in 11 states.

• Prohibition or restriction of sampling of electronic vapor products, present in Arizona, Delaware, Kentucky, Maryland, New Hampshire, North Carolina, Oklahoma, and South Carolina.

For most of the regulations, teens had a reduced likelihood of trying or currently using vaping products after adjusting for age, ethnicity, grade level, race, region, and sex. Risk of ever trying a vaping product was 12% lower in states that prohibited their use on school grounds or in workplaces, 6% lower in states that barred sales to those under age 18, and 7% lower in states that restricted or prohibited self-service vaping displays.

The risk of youth currently using electronic vapor products was 5% lower in states with the school grounds and workplace restrictions, and 13% lower in states that restricted self-service displays. Laws restricting minor sales were unrelated to the risk of current vaping among youth. Restricting vending machine sales of vaping products had no association with the risk of a teen ever trying vaping, but it was linked to a 7% lower risk of current use of the products among teens. All these associations were statistically significant based on confidence interval values.

Interestingly, a statistically significant risk increase in vaping use occurred for teens in states that restricted or outlawed sampling of vaping products. The risk was 8% higher for ever trying a product and 20% higher for current use. But those findings also could indicate the possibility of reverse causation.

“It’s possible that states that were particularly concerned about sampling had the worst problems – were the ones more likely to institute a ban on that practice, and that would generate the counterintuitive finding,” Dr. Keim said in an interview. “With the data currently available, we can’t look at teen use both before and after the restrictions, just afterwards, but with more data for 2017, it would provide a clearer picture of all of the associations we examined.”

Aside from these laws, other interventions have the potential to reduce vaping among teens as well.

“Restrictions on use in various types of public places and on school grounds may be additional helpful approaches, similar to what has been done with cigarettes,” Dr. Keim said. Although their analysis included laws that prohibited use on school grounds, only four states have one of these laws.

Several state regulations governing the sales or use of e-cigarettes and related products were associated with lower proportions of youth trying or regularly using vaping products, a new study found.

Restricting sales of electronic vapor products to minors, however, was not linked to a lower risk of vaping among teens.

Thinkstockphotos

• Statewide prohibition of vaping products on school property or in workplaces, which includes Arizona, New Hampshire, Vermont, and Virginia for schools and North Dakota for workplaces.

• Prohibition of sales to minors under age 18 years, present in 24 states.

• Prohibition or restriction of sales of e-cigarette products from vending machines, present in 17 states.

• Prohibition or restriction of self-service displays of vaping products, present in 11 states.

• Prohibition or restriction of sampling of electronic vapor products, present in Arizona, Delaware, Kentucky, Maryland, New Hampshire, North Carolina, Oklahoma, and South Carolina.

For most of the regulations, teens had a reduced likelihood of trying or currently using vaping products after adjusting for age, ethnicity, grade level, race, region, and sex. Risk of ever trying a vaping product was 12% lower in states that prohibited their use on school grounds or in workplaces, 6% lower in states that barred sales to those under age 18, and 7% lower in states that restricted or prohibited self-service vaping displays.

The risk of youth currently using electronic vapor products was 5% lower in states with the school grounds and workplace restrictions, and 13% lower in states that restricted self-service displays. Laws restricting minor sales were unrelated to the risk of current vaping among youth. Restricting vending machine sales of vaping products had no association with the risk of a teen ever trying vaping, but it was linked to a 7% lower risk of current use of the products among teens. All these associations were statistically significant based on confidence interval values.

Interestingly, a statistically significant risk increase in vaping use occurred for teens in states that restricted or outlawed sampling of vaping products. The risk was 8% higher for ever trying a product and 20% higher for current use. But those findings also could indicate the possibility of reverse causation.

“It’s possible that states that were particularly concerned about sampling had the worst problems – were the ones more likely to institute a ban on that practice, and that would generate the counterintuitive finding,” Dr. Keim said in an interview. “With the data currently available, we can’t look at teen use both before and after the restrictions, just afterwards, but with more data for 2017, it would provide a clearer picture of all of the associations we examined.”

Aside from these laws, other interventions have the potential to reduce vaping among teens as well.

“Restrictions on use in various types of public places and on school grounds may be additional helpful approaches, similar to what has been done with cigarettes,” Dr. Keim said. Although their analysis included laws that prohibited use on school grounds, only four states have one of these laws.

Real-time PCR techniques for identifying the pathogens responsible for onychomycosis can offer some advantages over conventional diagnostic approaches but also have their limitations, say the authors of a study published in Mycoses.

Anissa Z. Hafirassou of Frères-Mentouri, Constantine University, Algeria, and colleagues analyzed nail samples from 70 patients with clinical signs of onychomycosis and 15 healthy controls using four different real-time PCR assays – a panfungal, a pandermatophyte, an assay for Candida and one for Aspergillus – and conventional methods.

copyright Metin Cengiz Bar/Thinkstock

Real-time PCR techniques for identifying the pathogens responsible for onychomycosis can offer some advantages over conventional diagnostic approaches but also have their limitations, say the authors of a study published in Mycoses.

Anissa Z. Hafirassou of Frères-Mentouri, Constantine University, Algeria, and colleagues analyzed nail samples from 70 patients with clinical signs of onychomycosis and 15 healthy controls using four different real-time PCR assays – a panfungal, a pandermatophyte, an assay for Candida and one for Aspergillus – and conventional methods.

copyright Metin Cengiz Bar/Thinkstock

Real-time PCR techniques for identifying the pathogens responsible for onychomycosis can offer some advantages over conventional diagnostic approaches but also have their limitations, say the authors of a study published in Mycoses.

Anissa Z. Hafirassou of Frères-Mentouri, Constantine University, Algeria, and colleagues analyzed nail samples from 70 patients with clinical signs of onychomycosis and 15 healthy controls using four different real-time PCR assays – a panfungal, a pandermatophyte, an assay for Candida and one for Aspergillus – and conventional methods.

copyright Metin Cengiz Bar/Thinkstock

The Society for Vascular Surgery has its very own member affinity program, offering members access to best-in-class products and services coupled with special member discounts.

The portfolio includes a number of insurance, financial and private-practice related products that will assist members in their day-to-day lives. The result: a selection of financial and practice solutions to protect and benefit your families, incomes, practices, offices and staff – even your own slice of cyberspace.

For more information, visit vsweb.org/AffinityProgram, call 855-533-1776 or email [email protected].
 

The Society for Vascular Surgery has its very own member affinity program, offering members access to best-in-class products and services coupled with special member discounts.

The portfolio includes a number of insurance, financial and private-practice related products that will assist members in their day-to-day lives. The result: a selection of financial and practice solutions to protect and benefit your families, incomes, practices, offices and staff – even your own slice of cyberspace.

For more information, visit vsweb.org/AffinityProgram, call 855-533-1776 or email [email protected].
 

The Society for Vascular Surgery has its very own member affinity program, offering members access to best-in-class products and services coupled with special member discounts.

The portfolio includes a number of insurance, financial and private-practice related products that will assist members in their day-to-day lives. The result: a selection of financial and practice solutions to protect and benefit your families, incomes, practices, offices and staff – even your own slice of cyberspace.

For more information, visit vsweb.org/AffinityProgram, call 855-533-1776 or email [email protected].
 

CHICAGO – A gluten-free diet is the cornerstone of treatment for celiac disease, but healing of the gut may take longer in some patients than in others.

New findings presented at the annual Digestive Disease Week suggest that, even though patients treated with a gluten-free diet generally experience clinical improvement during the first few weeks or months of making dietary changes, serologic and especially histologic normalization may take longer – and it is not always certain that it will occur.

designer491/Thinkstock

[email protected]

CHICAGO – A gluten-free diet is the cornerstone of treatment for celiac disease, but healing of the gut may take longer in some patients than in others.

New findings presented at the annual Digestive Disease Week suggest that, even though patients treated with a gluten-free diet generally experience clinical improvement during the first few weeks or months of making dietary changes, serologic and especially histologic normalization may take longer – and it is not always certain that it will occur.

designer491/Thinkstock

[email protected]

CHICAGO – A gluten-free diet is the cornerstone of treatment for celiac disease, but healing of the gut may take longer in some patients than in others.

New findings presented at the annual Digestive Disease Week suggest that, even though patients treated with a gluten-free diet generally experience clinical improvement during the first few weeks or months of making dietary changes, serologic and especially histologic normalization may take longer – and it is not always certain that it will occur.

designer491/Thinkstock

[email protected]

CHICAGO – Out-of-pocket costs may present a barrier to colorectal screening, and removing those costs could reduce colorectal cancer deaths, according to new data presented at the annual Digestive Disease Week.

These data imply that removing copayments could result in a 16% decrease in colorectal cancer–related deaths among Medicare beneficiaries, explained lead author Elisabeth Peterse, PhD, of the department of public health, Erasmus Medical Center, Rotterdam, the Netherlands.

The research also demonstrated that waiving copayments is cost effective, she added.

Despite the effectiveness of colorectal cancer screening, only 58% of eligible individuals adhere to current screening recommendations, Dr. Peterse noted. Financial barriers may play a role in the lack of adherence, as studies have found that removing out-of-pocket costs is one of the most effective interventions for increasing screening.

“But despite the fact that the Affordable Care Act has been successful in partially eliminating cost sharing for colorectal screening, Medicare beneficiaries may still face unexpected out-of-pocket liabilities,” said Dr. Peterse.

Out-of-pocket costs can be complicated, given that they can depend largely on how a procedure is coded. A screening colonoscopy or fecal immunochemical test (FIT) is completely covered if it is coded as a screening test, but follow-up colonoscopies come with 20% copayments.

A screening colonoscopy with polypectomy and a follow-up colonoscopy that is done after a positive fecal immunochemical test are coded as diagnostic rather than screening, so the patient has out-of-pocket costs, she explained.

To explore how waiving the cost of screening could impact colorectal cancer–related mortality and cost effectiveness, the researchers conducted an analysis using a microsimulation model for a cohort composed of 65-year-old individuals.

In the simulation, they estimated colorectal cancer–related mortality, quality-adjusted life-years, and total cost of screening and treatment using the current Medicare copayment schedule. These were then compared with outcomes for alternative situations.

The study was conducted in two parts, explained Dr. Peterse. In the first part, the researchers looked at five scenarios: one in which the 20% copayment was intact. In the second, the copayment was waived without having any impact on adherence. In the third, the investigators looked at a 5% increase in adherence but only at diagnostic follow-up.

In the fourth and fifth scenarios, the investigators looked at 5% and 10% increases in adherence, in both first screening and diagnostic follow-up, she added.

In the study’s second part, the researchers also estimated the threshold increase in participation at which copayment removal would be cost effective, using a $50,000 willingness-to-pay threshold.

They found that without screening, the expected mortality would be 25 colorectal deaths per 1,000 people in a population of 65-year-old individuals. With screening, the number was reduced to 12.8 deaths per 1,000 65-year-olds for colonoscopy, and 14.9 deaths per 1,000 for FIT screening. The total associated costs for screening and treatment for the two modalities were $3.02 million and $2.87 million.

If waiving the copayments had no impact in increasing screening levels, the cost of screening was estimated to increase to $3.1 million (2.8% increase) for colonoscopy and $2.9 million (1.6% increase) for FIT.

But if copayments were removed and there were a 5% increase in adherence, colorectal cancer deaths were estimated to decline to 11.7 (–8.3%) and 13.9 (–6.3%) per 1,000 for colonoscopy and FIT, respectively. That would result in cost-effectiveness ratios of $19,288 and $7,894 for no copayment versus having a copayment. Increasing adherence to 10% would result in an even lower ratio, noted Dr. Peterse.

The threshold increase for participating in screening programs – the point where removing a copayment becomes cost effective – was a 1.8% increase in colonoscopy screening and a 0.8% increase for FIT.

The conclusion is that waiving copayments is cost effective, Dr. Peterse said.

Dr. Peterse added that a limitation to the analysis is that the study authors don’t know to what extent patients are even aware of the copayments. “So, we don’t know if it is a barrier, and we didn’t take other insurance scenarios into account,” she said.

Dr. Peterse declared no relevant disclosures.

CHICAGO – Out-of-pocket costs may present a barrier to colorectal screening, and removing those costs could reduce colorectal cancer deaths, according to new data presented at the annual Digestive Disease Week.

These data imply that removing copayments could result in a 16% decrease in colorectal cancer–related deaths among Medicare beneficiaries, explained lead author Elisabeth Peterse, PhD, of the department of public health, Erasmus Medical Center, Rotterdam, the Netherlands.

The research also demonstrated that waiving copayments is cost effective, she added.

Despite the effectiveness of colorectal cancer screening, only 58% of eligible individuals adhere to current screening recommendations, Dr. Peterse noted. Financial barriers may play a role in the lack of adherence, as studies have found that removing out-of-pocket costs is one of the most effective interventions for increasing screening.

“But despite the fact that the Affordable Care Act has been successful in partially eliminating cost sharing for colorectal screening, Medicare beneficiaries may still face unexpected out-of-pocket liabilities,” said Dr. Peterse.

Out-of-pocket costs can be complicated, given that they can depend largely on how a procedure is coded. A screening colonoscopy or fecal immunochemical test (FIT) is completely covered if it is coded as a screening test, but follow-up colonoscopies come with 20% copayments.

A screening colonoscopy with polypectomy and a follow-up colonoscopy that is done after a positive fecal immunochemical test are coded as diagnostic rather than screening, so the patient has out-of-pocket costs, she explained.

To explore how waiving the cost of screening could impact colorectal cancer–related mortality and cost effectiveness, the researchers conducted an analysis using a microsimulation model for a cohort composed of 65-year-old individuals.

In the simulation, they estimated colorectal cancer–related mortality, quality-adjusted life-years, and total cost of screening and treatment using the current Medicare copayment schedule. These were then compared with outcomes for alternative situations.

The study was conducted in two parts, explained Dr. Peterse. In the first part, the researchers looked at five scenarios: one in which the 20% copayment was intact. In the second, the copayment was waived without having any impact on adherence. In the third, the investigators looked at a 5% increase in adherence but only at diagnostic follow-up.

In the fourth and fifth scenarios, the investigators looked at 5% and 10% increases in adherence, in both first screening and diagnostic follow-up, she added.

In the study’s second part, the researchers also estimated the threshold increase in participation at which copayment removal would be cost effective, using a $50,000 willingness-to-pay threshold.

They found that without screening, the expected mortality would be 25 colorectal deaths per 1,000 people in a population of 65-year-old individuals. With screening, the number was reduced to 12.8 deaths per 1,000 65-year-olds for colonoscopy, and 14.9 deaths per 1,000 for FIT screening. The total associated costs for screening and treatment for the two modalities were $3.02 million and $2.87 million.

If waiving the copayments had no impact in increasing screening levels, the cost of screening was estimated to increase to $3.1 million (2.8% increase) for colonoscopy and $2.9 million (1.6% increase) for FIT.

But if copayments were removed and there were a 5% increase in adherence, colorectal cancer deaths were estimated to decline to 11.7 (–8.3%) and 13.9 (–6.3%) per 1,000 for colonoscopy and FIT, respectively. That would result in cost-effectiveness ratios of $19,288 and $7,894 for no copayment versus having a copayment. Increasing adherence to 10% would result in an even lower ratio, noted Dr. Peterse.

The threshold increase for participating in screening programs – the point where removing a copayment becomes cost effective – was a 1.8% increase in colonoscopy screening and a 0.8% increase for FIT.

The conclusion is that waiving copayments is cost effective, Dr. Peterse said.

Dr. Peterse added that a limitation to the analysis is that the study authors don’t know to what extent patients are even aware of the copayments. “So, we don’t know if it is a barrier, and we didn’t take other insurance scenarios into account,” she said.

Dr. Peterse declared no relevant disclosures.

CHICAGO – Out-of-pocket costs may present a barrier to colorectal screening, and removing those costs could reduce colorectal cancer deaths, according to new data presented at the annual Digestive Disease Week.

These data imply that removing copayments could result in a 16% decrease in colorectal cancer–related deaths among Medicare beneficiaries, explained lead author Elisabeth Peterse, PhD, of the department of public health, Erasmus Medical Center, Rotterdam, the Netherlands.

The research also demonstrated that waiving copayments is cost effective, she added.

Despite the effectiveness of colorectal cancer screening, only 58% of eligible individuals adhere to current screening recommendations, Dr. Peterse noted. Financial barriers may play a role in the lack of adherence, as studies have found that removing out-of-pocket costs is one of the most effective interventions for increasing screening.

“But despite the fact that the Affordable Care Act has been successful in partially eliminating cost sharing for colorectal screening, Medicare beneficiaries may still face unexpected out-of-pocket liabilities,” said Dr. Peterse.

Out-of-pocket costs can be complicated, given that they can depend largely on how a procedure is coded. A screening colonoscopy or fecal immunochemical test (FIT) is completely covered if it is coded as a screening test, but follow-up colonoscopies come with 20% copayments.

A screening colonoscopy with polypectomy and a follow-up colonoscopy that is done after a positive fecal immunochemical test are coded as diagnostic rather than screening, so the patient has out-of-pocket costs, she explained.

To explore how waiving the cost of screening could impact colorectal cancer–related mortality and cost effectiveness, the researchers conducted an analysis using a microsimulation model for a cohort composed of 65-year-old individuals.

In the simulation, they estimated colorectal cancer–related mortality, quality-adjusted life-years, and total cost of screening and treatment using the current Medicare copayment schedule. These were then compared with outcomes for alternative situations.

The study was conducted in two parts, explained Dr. Peterse. In the first part, the researchers looked at five scenarios: one in which the 20% copayment was intact. In the second, the copayment was waived without having any impact on adherence. In the third, the investigators looked at a 5% increase in adherence but only at diagnostic follow-up.

In the fourth and fifth scenarios, the investigators looked at 5% and 10% increases in adherence, in both first screening and diagnostic follow-up, she added.

In the study’s second part, the researchers also estimated the threshold increase in participation at which copayment removal would be cost effective, using a $50,000 willingness-to-pay threshold.

They found that without screening, the expected mortality would be 25 colorectal deaths per 1,000 people in a population of 65-year-old individuals. With screening, the number was reduced to 12.8 deaths per 1,000 65-year-olds for colonoscopy, and 14.9 deaths per 1,000 for FIT screening. The total associated costs for screening and treatment for the two modalities were $3.02 million and $2.87 million.

If waiving the copayments had no impact in increasing screening levels, the cost of screening was estimated to increase to $3.1 million (2.8% increase) for colonoscopy and $2.9 million (1.6% increase) for FIT.

But if copayments were removed and there were a 5% increase in adherence, colorectal cancer deaths were estimated to decline to 11.7 (–8.3%) and 13.9 (–6.3%) per 1,000 for colonoscopy and FIT, respectively. That would result in cost-effectiveness ratios of $19,288 and $7,894 for no copayment versus having a copayment. Increasing adherence to 10% would result in an even lower ratio, noted Dr. Peterse.

The threshold increase for participating in screening programs – the point where removing a copayment becomes cost effective – was a 1.8% increase in colonoscopy screening and a 0.8% increase for FIT.

The conclusion is that waiving copayments is cost effective, Dr. Peterse said.

Dr. Peterse added that a limitation to the analysis is that the study authors don’t know to what extent patients are even aware of the copayments. “So, we don’t know if it is a barrier, and we didn’t take other insurance scenarios into account,” she said.

Dr. Peterse declared no relevant disclosures.