VIDEO: Resistance to endocrine therapy a moving target

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– Hormonal ablation is a mainstay of therapy for women with hormone receptor–positive breast cancer. A significant proportion of patients, however, are either initially refractory to hormonal therapy or acquire resistance to it over time.

The difficulty for patients with breast cancer and for the physicians who treat them is that there are no simple answers to the question of which patients can continue to benefit from endocrine monotherapy. Are there adequate biomarkers for optimal follow-on therapy when a patient experiences disease progression, and what is the optimal sequence of targeted therapy with endocrine inhibitors, disrupters, or other agents?

In a video interview at the annual Miami Breast Cancer Conference, held by Physicians’ Education Resource, William J. Gradishar, MD, of Northwestern University, Chicago, discusses strategies for combating resistance to endocrine ablative therapy, and describes how new therapies and new treatment strategies are being incorporated into National Comprehensive Cancer Network breast cancer guidelines.

Dr. Gradishar reported having no clinical disclosures.

The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel
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– Hormonal ablation is a mainstay of therapy for women with hormone receptor–positive breast cancer. A significant proportion of patients, however, are either initially refractory to hormonal therapy or acquire resistance to it over time.

The difficulty for patients with breast cancer and for the physicians who treat them is that there are no simple answers to the question of which patients can continue to benefit from endocrine monotherapy. Are there adequate biomarkers for optimal follow-on therapy when a patient experiences disease progression, and what is the optimal sequence of targeted therapy with endocrine inhibitors, disrupters, or other agents?

In a video interview at the annual Miami Breast Cancer Conference, held by Physicians’ Education Resource, William J. Gradishar, MD, of Northwestern University, Chicago, discusses strategies for combating resistance to endocrine ablative therapy, and describes how new therapies and new treatment strategies are being incorporated into National Comprehensive Cancer Network breast cancer guidelines.

Dr. Gradishar reported having no clinical disclosures.

The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel

 

– Hormonal ablation is a mainstay of therapy for women with hormone receptor–positive breast cancer. A significant proportion of patients, however, are either initially refractory to hormonal therapy or acquire resistance to it over time.

The difficulty for patients with breast cancer and for the physicians who treat them is that there are no simple answers to the question of which patients can continue to benefit from endocrine monotherapy. Are there adequate biomarkers for optimal follow-on therapy when a patient experiences disease progression, and what is the optimal sequence of targeted therapy with endocrine inhibitors, disrupters, or other agents?

In a video interview at the annual Miami Breast Cancer Conference, held by Physicians’ Education Resource, William J. Gradishar, MD, of Northwestern University, Chicago, discusses strategies for combating resistance to endocrine ablative therapy, and describes how new therapies and new treatment strategies are being incorporated into National Comprehensive Cancer Network breast cancer guidelines.

Dr. Gradishar reported having no clinical disclosures.

The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel
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NetWorks: Mass shootings, MACRA, asthma in pregnancy Disaster Response Practice Operations Transplant Women’s Health

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Mass shootings

There are multiple definitions for a mass shooting. Some definitions require a certain number of people be killed. Some definitions require a certain number of people be shot. Some definitions do not include gang violence. Regardless of the definition used, the number of mass shootings in the United States is increasing.

There are also multiple definitions of what qualifies as a medical disaster. These definitions can be summarized with the statement that a medical disaster is an event that produces a number of casualties that overwhelms the local health system.

Dr. John Gaillard
In the first 31 days of 2017, there have been 30 shootings in the United States, in which four or more people were injured (www.gunviolencearchive.org/reports/mass-shooting). On average, 309 people are shot every day in the United States. Ninety-three (30%) of those victims die of their injuries (www.bradycampaign.org/key-gun-violence-statistics).

Most mass shootings fit the definition of a medical disaster. When a mass shooting occurs, medical resources are diverted from current patients to those injured in the shooting. Patients with acute medical problems unrelated to the shooting must endure a prolonged wait for medical care.

The CHEST Disaster Response NetWork feels that it is necessary to take action to reduce the number of mass shootings. Unlike natural disasters, mass shootings are man-made. As such, we should proactively work to prevent them. Prevention is a large part of medicine. Working together with community leaders, law enforcement, and government officials, we can and should work to eliminate mass shootings so that we can minimize gun-related injury and death.

MACRA: Reincarnation of Medicare physician reimbursement model

In April 2015, President Obama signed the Medicare Access and CHIP Reauthorization Act (MACRA) eradicating the detested sustainable growth rate (SGR) formula. If this is your first dive into MACRA as an eligible professional (EP), it may be a bit baffling trying to understand its impact on your practice.

Dr. Humayan Anjum
MACRA affects physician offices, not hospitals. For 2017-2018, EPs include physicians, physician-assistants, nurse practitioners, clinical nurse specialists, and nurse anesthetists. Providers in their first year of Medicare participation or with a low Medicare volume are excluded. Additionally, there are two participation pathways, Merit-Based Incentive Payment System (MIPS), which combines the current Physician Quality Reporting System, Value Modifier, and Meaningful Use programs into a single pay-for-performance payment system; or Alternative Payment Models (APMs) that provide incentives in certain alternative payment models based on proposed CMS criteria. Accountable Care Organizations, Patient-Centered Medical Homes, and Bundled Payment Models are a few examples of an APM.

Under MIPS, rules are divided into four categories. During the first year, each category will make up a certain percentage to the physician’s overall score, which will result in a penalty or payment as a lump sum in 2019. If you are an Advanced APM in 2017 and receive 25% of Medicare payments or see 20% of your Medicare patients through this model, you can earn up to a 5% incentive payment in 2019.

The performance period started on January 1, 2017. Submission of performance data is due by March 31, 2018. MACRA is complicated and here to stay. Learn and educate yourself to avoid downward payment adjustment. For full details, please visit https://qpp.cms.gov/docs/QPP_Executive_Summary_of_Final_Rule.pdf.

References

1. CMS MACRA Proposed Rule. http://1.usa.gov/1PpBpMt

2. CMS MACRA Executive Summary. https://qpp.cms.gov/docs/QPP_Executive_Summary_of_Final_Rule.pdf

3. American Medical Association. http://bit.ly/1miEtBD

4. Policy and Medicine. MACRA http://bit.ly/1PTLkKa. MIPS http://bit.ly/20RoMzZ. APMs http://bit.ly/1OlxoxH

Frailty in lung transplantation

Two of the greatest challenges in lung transplantation are to identify optimal transplant candidates and to help those transplant recipients thrive in the years following surgery. Frailty is emerging as a marker of increased posttransplant morbidity and may represent an area where both the recipient selection process and posttransplant outcomes can be optimized. Described by some as “biologic age” rather than “chronologic age,” frailty is a syndrome of functional impairment and weakness that predisposes to adverse health outcomes. The adverse effects of frailty have been described in multiple clinical scenarios, including the ICU, chronic lung diseases, heart failure, liver transplant, kidney transplant, geriatrics, and others.

Dr. Michael E. Wilson
Approximately 10% to 45% of lung transplant patients are considered to be frail, depending on the measurement used. In a cohort of lung transplant recipients, frail patients had increased 1-year mortality (21.2% increase) and 3-year mortality (24.8% increase), compared with nonfrail patients (Wilson et al. J Heart Lung Transplant. 2016;35[2]:173-178). In a cohort of patients on the lung transplant waiting list, frailty was associated with an increased risk of delisting or death before lung transplant (Singer et al. Am J Respir Crit Care Med. 2015;192[11]:1325-1334). In addition, frailty may be associated with an increased risk of hospital readmissions and acute rejection following transplant (Wilson et al. J Heart Lung Transplant. 2016;35[4]:S317).

Remaining challenges include determining which clinical assessments best define frailty in the lung transplant population, documenting the adverse effects of frailty in well-designed multicenter prospective studies, and developing interventions to mitigate the adverse effects of frailty.
 

 

Asthma treatment during pregnancy

Asthma is common in pregnancy, occurring in 3% to 8% of pregnant women. While the course of asthma during pregnancy is variable, the objectives of asthma treatment do not change and aim to prevent acute exacerbations and optimize management. Uncontrolled asthma is associated with an increased risk of perinatal morbidity. Published guidelines on pharmacologic therapies during pregnancy recommend the same step-wise approach as in nonpregnant women.

Dr. Mariam Louis
Despite this, many providers are reluctant to prescribe medications during pregnancy, and data show a reduction of refills of asthma medications during pregnancy, likely due to safety concerns. Some recent studies have suggested an increase in major congenital anomalies among pregnant asthmatics using ICS (Garne E et al. BJOG. 2016;123[10]:1609-18), albeit with large confidence intervals. These findings have not been consistently confirmed (Kallen B et al. Eur J Clin Pharmacol. 2007;63:383-8). Furthermore, studies showing a dose response association of ICS with congenital anomalies (Blais L et al. J Allergy Clin Immunol. 2009;124[6]:1229-34) suggest that disease severity may be a confounder in these associations.

The diagnosis of asthma, the use of other concurrent medications, and medication compliance may all be potential confounders. ICS use in pregnancy was associated with endocrine and metabolic disturbances in the offspring in a national cohort (Tegethoff M et al. Am J Respir Crit Care Med. 2012;185[5]:557-63). However, this study did not report on systemic steroid use, asthma severity, or details of these disturbances.

Dr. Ghada Bourjeily
In summary, ICS use remains justifiable in pregnancy (Smy L et al. Can Fam Physician. 2014;60[9]:809-12) as the risk of untreated or poorly treated asthma outweighs the possible risk of ICS use, especially when alternative drugs such as systemic steroids are not without risk. Ultimately, it should be stressed that asthma control is the goal of treatment. This should be achieved with close interaction between the pregnant woman and her health-care provider.
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Mass shootings

There are multiple definitions for a mass shooting. Some definitions require a certain number of people be killed. Some definitions require a certain number of people be shot. Some definitions do not include gang violence. Regardless of the definition used, the number of mass shootings in the United States is increasing.

There are also multiple definitions of what qualifies as a medical disaster. These definitions can be summarized with the statement that a medical disaster is an event that produces a number of casualties that overwhelms the local health system.

Dr. John Gaillard
In the first 31 days of 2017, there have been 30 shootings in the United States, in which four or more people were injured (www.gunviolencearchive.org/reports/mass-shooting). On average, 309 people are shot every day in the United States. Ninety-three (30%) of those victims die of their injuries (www.bradycampaign.org/key-gun-violence-statistics).

Most mass shootings fit the definition of a medical disaster. When a mass shooting occurs, medical resources are diverted from current patients to those injured in the shooting. Patients with acute medical problems unrelated to the shooting must endure a prolonged wait for medical care.

The CHEST Disaster Response NetWork feels that it is necessary to take action to reduce the number of mass shootings. Unlike natural disasters, mass shootings are man-made. As such, we should proactively work to prevent them. Prevention is a large part of medicine. Working together with community leaders, law enforcement, and government officials, we can and should work to eliminate mass shootings so that we can minimize gun-related injury and death.

MACRA: Reincarnation of Medicare physician reimbursement model

In April 2015, President Obama signed the Medicare Access and CHIP Reauthorization Act (MACRA) eradicating the detested sustainable growth rate (SGR) formula. If this is your first dive into MACRA as an eligible professional (EP), it may be a bit baffling trying to understand its impact on your practice.

Dr. Humayan Anjum
MACRA affects physician offices, not hospitals. For 2017-2018, EPs include physicians, physician-assistants, nurse practitioners, clinical nurse specialists, and nurse anesthetists. Providers in their first year of Medicare participation or with a low Medicare volume are excluded. Additionally, there are two participation pathways, Merit-Based Incentive Payment System (MIPS), which combines the current Physician Quality Reporting System, Value Modifier, and Meaningful Use programs into a single pay-for-performance payment system; or Alternative Payment Models (APMs) that provide incentives in certain alternative payment models based on proposed CMS criteria. Accountable Care Organizations, Patient-Centered Medical Homes, and Bundled Payment Models are a few examples of an APM.

Under MIPS, rules are divided into four categories. During the first year, each category will make up a certain percentage to the physician’s overall score, which will result in a penalty or payment as a lump sum in 2019. If you are an Advanced APM in 2017 and receive 25% of Medicare payments or see 20% of your Medicare patients through this model, you can earn up to a 5% incentive payment in 2019.

The performance period started on January 1, 2017. Submission of performance data is due by March 31, 2018. MACRA is complicated and here to stay. Learn and educate yourself to avoid downward payment adjustment. For full details, please visit https://qpp.cms.gov/docs/QPP_Executive_Summary_of_Final_Rule.pdf.

References

1. CMS MACRA Proposed Rule. http://1.usa.gov/1PpBpMt

2. CMS MACRA Executive Summary. https://qpp.cms.gov/docs/QPP_Executive_Summary_of_Final_Rule.pdf

3. American Medical Association. http://bit.ly/1miEtBD

4. Policy and Medicine. MACRA http://bit.ly/1PTLkKa. MIPS http://bit.ly/20RoMzZ. APMs http://bit.ly/1OlxoxH

Frailty in lung transplantation

Two of the greatest challenges in lung transplantation are to identify optimal transplant candidates and to help those transplant recipients thrive in the years following surgery. Frailty is emerging as a marker of increased posttransplant morbidity and may represent an area where both the recipient selection process and posttransplant outcomes can be optimized. Described by some as “biologic age” rather than “chronologic age,” frailty is a syndrome of functional impairment and weakness that predisposes to adverse health outcomes. The adverse effects of frailty have been described in multiple clinical scenarios, including the ICU, chronic lung diseases, heart failure, liver transplant, kidney transplant, geriatrics, and others.

Dr. Michael E. Wilson
Approximately 10% to 45% of lung transplant patients are considered to be frail, depending on the measurement used. In a cohort of lung transplant recipients, frail patients had increased 1-year mortality (21.2% increase) and 3-year mortality (24.8% increase), compared with nonfrail patients (Wilson et al. J Heart Lung Transplant. 2016;35[2]:173-178). In a cohort of patients on the lung transplant waiting list, frailty was associated with an increased risk of delisting or death before lung transplant (Singer et al. Am J Respir Crit Care Med. 2015;192[11]:1325-1334). In addition, frailty may be associated with an increased risk of hospital readmissions and acute rejection following transplant (Wilson et al. J Heart Lung Transplant. 2016;35[4]:S317).

Remaining challenges include determining which clinical assessments best define frailty in the lung transplant population, documenting the adverse effects of frailty in well-designed multicenter prospective studies, and developing interventions to mitigate the adverse effects of frailty.
 

 

Asthma treatment during pregnancy

Asthma is common in pregnancy, occurring in 3% to 8% of pregnant women. While the course of asthma during pregnancy is variable, the objectives of asthma treatment do not change and aim to prevent acute exacerbations and optimize management. Uncontrolled asthma is associated with an increased risk of perinatal morbidity. Published guidelines on pharmacologic therapies during pregnancy recommend the same step-wise approach as in nonpregnant women.

Dr. Mariam Louis
Despite this, many providers are reluctant to prescribe medications during pregnancy, and data show a reduction of refills of asthma medications during pregnancy, likely due to safety concerns. Some recent studies have suggested an increase in major congenital anomalies among pregnant asthmatics using ICS (Garne E et al. BJOG. 2016;123[10]:1609-18), albeit with large confidence intervals. These findings have not been consistently confirmed (Kallen B et al. Eur J Clin Pharmacol. 2007;63:383-8). Furthermore, studies showing a dose response association of ICS with congenital anomalies (Blais L et al. J Allergy Clin Immunol. 2009;124[6]:1229-34) suggest that disease severity may be a confounder in these associations.

The diagnosis of asthma, the use of other concurrent medications, and medication compliance may all be potential confounders. ICS use in pregnancy was associated with endocrine and metabolic disturbances in the offspring in a national cohort (Tegethoff M et al. Am J Respir Crit Care Med. 2012;185[5]:557-63). However, this study did not report on systemic steroid use, asthma severity, or details of these disturbances.

Dr. Ghada Bourjeily
In summary, ICS use remains justifiable in pregnancy (Smy L et al. Can Fam Physician. 2014;60[9]:809-12) as the risk of untreated or poorly treated asthma outweighs the possible risk of ICS use, especially when alternative drugs such as systemic steroids are not without risk. Ultimately, it should be stressed that asthma control is the goal of treatment. This should be achieved with close interaction between the pregnant woman and her health-care provider.

Mass shootings

There are multiple definitions for a mass shooting. Some definitions require a certain number of people be killed. Some definitions require a certain number of people be shot. Some definitions do not include gang violence. Regardless of the definition used, the number of mass shootings in the United States is increasing.

There are also multiple definitions of what qualifies as a medical disaster. These definitions can be summarized with the statement that a medical disaster is an event that produces a number of casualties that overwhelms the local health system.

Dr. John Gaillard
In the first 31 days of 2017, there have been 30 shootings in the United States, in which four or more people were injured (www.gunviolencearchive.org/reports/mass-shooting). On average, 309 people are shot every day in the United States. Ninety-three (30%) of those victims die of their injuries (www.bradycampaign.org/key-gun-violence-statistics).

Most mass shootings fit the definition of a medical disaster. When a mass shooting occurs, medical resources are diverted from current patients to those injured in the shooting. Patients with acute medical problems unrelated to the shooting must endure a prolonged wait for medical care.

The CHEST Disaster Response NetWork feels that it is necessary to take action to reduce the number of mass shootings. Unlike natural disasters, mass shootings are man-made. As such, we should proactively work to prevent them. Prevention is a large part of medicine. Working together with community leaders, law enforcement, and government officials, we can and should work to eliminate mass shootings so that we can minimize gun-related injury and death.

MACRA: Reincarnation of Medicare physician reimbursement model

In April 2015, President Obama signed the Medicare Access and CHIP Reauthorization Act (MACRA) eradicating the detested sustainable growth rate (SGR) formula. If this is your first dive into MACRA as an eligible professional (EP), it may be a bit baffling trying to understand its impact on your practice.

Dr. Humayan Anjum
MACRA affects physician offices, not hospitals. For 2017-2018, EPs include physicians, physician-assistants, nurse practitioners, clinical nurse specialists, and nurse anesthetists. Providers in their first year of Medicare participation or with a low Medicare volume are excluded. Additionally, there are two participation pathways, Merit-Based Incentive Payment System (MIPS), which combines the current Physician Quality Reporting System, Value Modifier, and Meaningful Use programs into a single pay-for-performance payment system; or Alternative Payment Models (APMs) that provide incentives in certain alternative payment models based on proposed CMS criteria. Accountable Care Organizations, Patient-Centered Medical Homes, and Bundled Payment Models are a few examples of an APM.

Under MIPS, rules are divided into four categories. During the first year, each category will make up a certain percentage to the physician’s overall score, which will result in a penalty or payment as a lump sum in 2019. If you are an Advanced APM in 2017 and receive 25% of Medicare payments or see 20% of your Medicare patients through this model, you can earn up to a 5% incentive payment in 2019.

The performance period started on January 1, 2017. Submission of performance data is due by March 31, 2018. MACRA is complicated and here to stay. Learn and educate yourself to avoid downward payment adjustment. For full details, please visit https://qpp.cms.gov/docs/QPP_Executive_Summary_of_Final_Rule.pdf.

References

1. CMS MACRA Proposed Rule. http://1.usa.gov/1PpBpMt

2. CMS MACRA Executive Summary. https://qpp.cms.gov/docs/QPP_Executive_Summary_of_Final_Rule.pdf

3. American Medical Association. http://bit.ly/1miEtBD

4. Policy and Medicine. MACRA http://bit.ly/1PTLkKa. MIPS http://bit.ly/20RoMzZ. APMs http://bit.ly/1OlxoxH

Frailty in lung transplantation

Two of the greatest challenges in lung transplantation are to identify optimal transplant candidates and to help those transplant recipients thrive in the years following surgery. Frailty is emerging as a marker of increased posttransplant morbidity and may represent an area where both the recipient selection process and posttransplant outcomes can be optimized. Described by some as “biologic age” rather than “chronologic age,” frailty is a syndrome of functional impairment and weakness that predisposes to adverse health outcomes. The adverse effects of frailty have been described in multiple clinical scenarios, including the ICU, chronic lung diseases, heart failure, liver transplant, kidney transplant, geriatrics, and others.

Dr. Michael E. Wilson
Approximately 10% to 45% of lung transplant patients are considered to be frail, depending on the measurement used. In a cohort of lung transplant recipients, frail patients had increased 1-year mortality (21.2% increase) and 3-year mortality (24.8% increase), compared with nonfrail patients (Wilson et al. J Heart Lung Transplant. 2016;35[2]:173-178). In a cohort of patients on the lung transplant waiting list, frailty was associated with an increased risk of delisting or death before lung transplant (Singer et al. Am J Respir Crit Care Med. 2015;192[11]:1325-1334). In addition, frailty may be associated with an increased risk of hospital readmissions and acute rejection following transplant (Wilson et al. J Heart Lung Transplant. 2016;35[4]:S317).

Remaining challenges include determining which clinical assessments best define frailty in the lung transplant population, documenting the adverse effects of frailty in well-designed multicenter prospective studies, and developing interventions to mitigate the adverse effects of frailty.
 

 

Asthma treatment during pregnancy

Asthma is common in pregnancy, occurring in 3% to 8% of pregnant women. While the course of asthma during pregnancy is variable, the objectives of asthma treatment do not change and aim to prevent acute exacerbations and optimize management. Uncontrolled asthma is associated with an increased risk of perinatal morbidity. Published guidelines on pharmacologic therapies during pregnancy recommend the same step-wise approach as in nonpregnant women.

Dr. Mariam Louis
Despite this, many providers are reluctant to prescribe medications during pregnancy, and data show a reduction of refills of asthma medications during pregnancy, likely due to safety concerns. Some recent studies have suggested an increase in major congenital anomalies among pregnant asthmatics using ICS (Garne E et al. BJOG. 2016;123[10]:1609-18), albeit with large confidence intervals. These findings have not been consistently confirmed (Kallen B et al. Eur J Clin Pharmacol. 2007;63:383-8). Furthermore, studies showing a dose response association of ICS with congenital anomalies (Blais L et al. J Allergy Clin Immunol. 2009;124[6]:1229-34) suggest that disease severity may be a confounder in these associations.

The diagnosis of asthma, the use of other concurrent medications, and medication compliance may all be potential confounders. ICS use in pregnancy was associated with endocrine and metabolic disturbances in the offspring in a national cohort (Tegethoff M et al. Am J Respir Crit Care Med. 2012;185[5]:557-63). However, this study did not report on systemic steroid use, asthma severity, or details of these disturbances.

Dr. Ghada Bourjeily
In summary, ICS use remains justifiable in pregnancy (Smy L et al. Can Fam Physician. 2014;60[9]:809-12) as the risk of untreated or poorly treated asthma outweighs the possible risk of ICS use, especially when alternative drugs such as systemic steroids are not without risk. Ultimately, it should be stressed that asthma control is the goal of treatment. This should be achieved with close interaction between the pregnant woman and her health-care provider.
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Connect to CHEST members and leaders

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Build your network and access the CHEST Member and Leader Directory. Use the directory to search for members and leaders. Easily search by name, specialty, location, or CHEST committee name to find others in the CHEST community. All members current in their dues are included in the directory.

Access to the directory is an exclusive CHEST member benefit. To view the directory, visit the following address: https://www.chestnet.org/Get-Involved/Membership/Member-Directory.

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Build your network and access the CHEST Member and Leader Directory. Use the directory to search for members and leaders. Easily search by name, specialty, location, or CHEST committee name to find others in the CHEST community. All members current in their dues are included in the directory.

Access to the directory is an exclusive CHEST member benefit. To view the directory, visit the following address: https://www.chestnet.org/Get-Involved/Membership/Member-Directory.

 

Build your network and access the CHEST Member and Leader Directory. Use the directory to search for members and leaders. Easily search by name, specialty, location, or CHEST committee name to find others in the CHEST community. All members current in their dues are included in the directory.

Access to the directory is an exclusive CHEST member benefit. To view the directory, visit the following address: https://www.chestnet.org/Get-Involved/Membership/Member-Directory.

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In memoriam

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Sylvan Lee Weinberg, MD, FCCP, MACC, a Past President of the American College of Chest Physicians (1983-1984), died Jan 17, 2017, in Dayton, Ohio. Dr. Weinberg was born in Nashville, TN, and received both his bachelor of science and doctor of medicine degrees from Northwestern University in Evanston, IL. He spent his time as an intern, medical resident, and fellow in cardiology at the Michael Reese Hospital in Chicago and went on to serve as a physician at Good Samaritan Hospital in Dayton, Ohio, for more than 40 years, ultimately becoming chief of cardiology and founder of the first coronary care unit in Ohio. Dr. Weinberg was also a clinical professor of medicine at the Wright State University School of Medicine in Dayton, and led a group cardiology practice until his retirement in 2000.

Dr. Sylvan Lee Weinberg
A past president also of the American College of Cardiology (ACC) and the Montgomery County Medical Society, Dr. Weinberg was the founding editor of the American Heart Hospital Journal, founding co-editor of Heart & Lung, and founding editor of the Journal of The Heart Institute of Dayton. He also was associate editor of the AMA Archives of Internal Medicine, the ACC Review Journal, and served on numerous editorial boards, including CHEST, the Journal of the American College of Cardiology, and the Clinical Cardiology and Heart Journal, formerly the British Heart Journal. He was editor-in-chief of ACC’s ACCEL audio journal for 15 years, recognized and known as, “the voice of cardiology,” traveling around the world and interviewing the world’s leaders in cardiology.

CHEST extends its heartfelt condolences to Dr. Weinberg’s family and friends.
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Sylvan Lee Weinberg, MD, FCCP, MACC, a Past President of the American College of Chest Physicians (1983-1984), died Jan 17, 2017, in Dayton, Ohio. Dr. Weinberg was born in Nashville, TN, and received both his bachelor of science and doctor of medicine degrees from Northwestern University in Evanston, IL. He spent his time as an intern, medical resident, and fellow in cardiology at the Michael Reese Hospital in Chicago and went on to serve as a physician at Good Samaritan Hospital in Dayton, Ohio, for more than 40 years, ultimately becoming chief of cardiology and founder of the first coronary care unit in Ohio. Dr. Weinberg was also a clinical professor of medicine at the Wright State University School of Medicine in Dayton, and led a group cardiology practice until his retirement in 2000.

Dr. Sylvan Lee Weinberg
A past president also of the American College of Cardiology (ACC) and the Montgomery County Medical Society, Dr. Weinberg was the founding editor of the American Heart Hospital Journal, founding co-editor of Heart & Lung, and founding editor of the Journal of The Heart Institute of Dayton. He also was associate editor of the AMA Archives of Internal Medicine, the ACC Review Journal, and served on numerous editorial boards, including CHEST, the Journal of the American College of Cardiology, and the Clinical Cardiology and Heart Journal, formerly the British Heart Journal. He was editor-in-chief of ACC’s ACCEL audio journal for 15 years, recognized and known as, “the voice of cardiology,” traveling around the world and interviewing the world’s leaders in cardiology.

CHEST extends its heartfelt condolences to Dr. Weinberg’s family and friends.

 

Sylvan Lee Weinberg, MD, FCCP, MACC, a Past President of the American College of Chest Physicians (1983-1984), died Jan 17, 2017, in Dayton, Ohio. Dr. Weinberg was born in Nashville, TN, and received both his bachelor of science and doctor of medicine degrees from Northwestern University in Evanston, IL. He spent his time as an intern, medical resident, and fellow in cardiology at the Michael Reese Hospital in Chicago and went on to serve as a physician at Good Samaritan Hospital in Dayton, Ohio, for more than 40 years, ultimately becoming chief of cardiology and founder of the first coronary care unit in Ohio. Dr. Weinberg was also a clinical professor of medicine at the Wright State University School of Medicine in Dayton, and led a group cardiology practice until his retirement in 2000.

Dr. Sylvan Lee Weinberg
A past president also of the American College of Cardiology (ACC) and the Montgomery County Medical Society, Dr. Weinberg was the founding editor of the American Heart Hospital Journal, founding co-editor of Heart & Lung, and founding editor of the Journal of The Heart Institute of Dayton. He also was associate editor of the AMA Archives of Internal Medicine, the ACC Review Journal, and served on numerous editorial boards, including CHEST, the Journal of the American College of Cardiology, and the Clinical Cardiology and Heart Journal, formerly the British Heart Journal. He was editor-in-chief of ACC’s ACCEL audio journal for 15 years, recognized and known as, “the voice of cardiology,” traveling around the world and interviewing the world’s leaders in cardiology.

CHEST extends its heartfelt condolences to Dr. Weinberg’s family and friends.
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No rise in CV events seen with tocilizumab

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Patients with refractory rheumatoid arthritis who switched to tocilizumab showed no increased cardiovascular risk when compared with those who switched to a tumor necrosis factor inhibitor in a large cohort study.

Dr. Seoyoung C. Kim
To examine this issue, the investigators analyzed information regarding CV mortality in three large health care claims databases covering all 50 states. They focused on 28,028 adults with RA who switched from taking at least one biologic agent or targeted synthetic disease-modifying antirheumatic drug to either tocilizumab (9,218 patients) or a tumor necrosis factor (TNF) inhibitor (18,810 patients). These patients were followed for a mean of 1 year.

To minimize the effect of confounding by the severity of RA and the baseline CV risk, the researchers adjusted the data to account for more than 90 variables related to CV events and to RA severity.

The primary outcome — a composite of MI and stroke — occurred in 125 patients, 36 taking tocilizumab and 89 taking TNF inhibitors. The rate of this composite outcome was 0.52 per 100 person-years with tocilizumab and 0.59 per 100 person-years for TNF inhibitors, a nonsignificant difference, Dr. Kim and her associates reported (Arthritis Rheumatol. 2017 Feb 28. doi: 10.1002/art.40084).

There also were no significant differences between the two study groups in secondary endpoints, including rates of coronary revascularization, acute coronary syndrome, heart failure, and all-cause mortality. In addition, all subgroup analyses confirmed that tocilizumab did not raise CV risk, regardless of patient age (younger than or older than 60 years), the presence of cardiovascular disease at baseline, the presence of diabetes, the use of methotrexate, the use of oral steroids, or the use of statins.

These “reassuring” findings show that even though tocilizumab appears to raise LDL levels, “such increases do not appear to be associated with an increased risk of clinical CV events,” the investigators said.

The results confirm those reported at the 2016 American College of Rheumatology annual meeting for the 5-year, randomized, postmarketing ENTRACTE trial in which the lipid changes induced by tocilizumab did not translate into an increased risk of heart attack or stroke in RA patients.

This cohort study was sponsored by Genentech, which markets tocilizumab (Actemra). Dr. Kim reported ties to Genentech, Lilly, Pfizer, Bristol-Myers Squibb, and AstraZeneca. Her associates reported ties to Genentech, Lilly, Pfizer, AstraZeneca, Amgen, Corrona, Whiscon, Aetion, and Boehringer Ingelheim. Three of the seven authors were employees of Genentech.
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Patients with refractory rheumatoid arthritis who switched to tocilizumab showed no increased cardiovascular risk when compared with those who switched to a tumor necrosis factor inhibitor in a large cohort study.

Dr. Seoyoung C. Kim
To examine this issue, the investigators analyzed information regarding CV mortality in three large health care claims databases covering all 50 states. They focused on 28,028 adults with RA who switched from taking at least one biologic agent or targeted synthetic disease-modifying antirheumatic drug to either tocilizumab (9,218 patients) or a tumor necrosis factor (TNF) inhibitor (18,810 patients). These patients were followed for a mean of 1 year.

To minimize the effect of confounding by the severity of RA and the baseline CV risk, the researchers adjusted the data to account for more than 90 variables related to CV events and to RA severity.

The primary outcome — a composite of MI and stroke — occurred in 125 patients, 36 taking tocilizumab and 89 taking TNF inhibitors. The rate of this composite outcome was 0.52 per 100 person-years with tocilizumab and 0.59 per 100 person-years for TNF inhibitors, a nonsignificant difference, Dr. Kim and her associates reported (Arthritis Rheumatol. 2017 Feb 28. doi: 10.1002/art.40084).

There also were no significant differences between the two study groups in secondary endpoints, including rates of coronary revascularization, acute coronary syndrome, heart failure, and all-cause mortality. In addition, all subgroup analyses confirmed that tocilizumab did not raise CV risk, regardless of patient age (younger than or older than 60 years), the presence of cardiovascular disease at baseline, the presence of diabetes, the use of methotrexate, the use of oral steroids, or the use of statins.

These “reassuring” findings show that even though tocilizumab appears to raise LDL levels, “such increases do not appear to be associated with an increased risk of clinical CV events,” the investigators said.

The results confirm those reported at the 2016 American College of Rheumatology annual meeting for the 5-year, randomized, postmarketing ENTRACTE trial in which the lipid changes induced by tocilizumab did not translate into an increased risk of heart attack or stroke in RA patients.

This cohort study was sponsored by Genentech, which markets tocilizumab (Actemra). Dr. Kim reported ties to Genentech, Lilly, Pfizer, Bristol-Myers Squibb, and AstraZeneca. Her associates reported ties to Genentech, Lilly, Pfizer, AstraZeneca, Amgen, Corrona, Whiscon, Aetion, and Boehringer Ingelheim. Three of the seven authors were employees of Genentech.

 

Patients with refractory rheumatoid arthritis who switched to tocilizumab showed no increased cardiovascular risk when compared with those who switched to a tumor necrosis factor inhibitor in a large cohort study.

Dr. Seoyoung C. Kim
To examine this issue, the investigators analyzed information regarding CV mortality in three large health care claims databases covering all 50 states. They focused on 28,028 adults with RA who switched from taking at least one biologic agent or targeted synthetic disease-modifying antirheumatic drug to either tocilizumab (9,218 patients) or a tumor necrosis factor (TNF) inhibitor (18,810 patients). These patients were followed for a mean of 1 year.

To minimize the effect of confounding by the severity of RA and the baseline CV risk, the researchers adjusted the data to account for more than 90 variables related to CV events and to RA severity.

The primary outcome — a composite of MI and stroke — occurred in 125 patients, 36 taking tocilizumab and 89 taking TNF inhibitors. The rate of this composite outcome was 0.52 per 100 person-years with tocilizumab and 0.59 per 100 person-years for TNF inhibitors, a nonsignificant difference, Dr. Kim and her associates reported (Arthritis Rheumatol. 2017 Feb 28. doi: 10.1002/art.40084).

There also were no significant differences between the two study groups in secondary endpoints, including rates of coronary revascularization, acute coronary syndrome, heart failure, and all-cause mortality. In addition, all subgroup analyses confirmed that tocilizumab did not raise CV risk, regardless of patient age (younger than or older than 60 years), the presence of cardiovascular disease at baseline, the presence of diabetes, the use of methotrexate, the use of oral steroids, or the use of statins.

These “reassuring” findings show that even though tocilizumab appears to raise LDL levels, “such increases do not appear to be associated with an increased risk of clinical CV events,” the investigators said.

The results confirm those reported at the 2016 American College of Rheumatology annual meeting for the 5-year, randomized, postmarketing ENTRACTE trial in which the lipid changes induced by tocilizumab did not translate into an increased risk of heart attack or stroke in RA patients.

This cohort study was sponsored by Genentech, which markets tocilizumab (Actemra). Dr. Kim reported ties to Genentech, Lilly, Pfizer, Bristol-Myers Squibb, and AstraZeneca. Her associates reported ties to Genentech, Lilly, Pfizer, AstraZeneca, Amgen, Corrona, Whiscon, Aetion, and Boehringer Ingelheim. Three of the seven authors were employees of Genentech.
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Key clinical point: Patients with refractory RA who switch to tocilizumab show no increased cardiovascular risk, compared with those who switch to a TNF inhibitor.

Major finding: The primary outcome – the rate of a composite of MI and stroke – was 0.52 per 100 person-years with tocilizumab and 0.59 per 100 person-years for TNF inhibitors, a nonsignificant difference.

Data source: A cohort study involving 28,028 adults with RA enrolled in three large health care claims databases from all 50 states who were followed for a median of 1 year.

Disclosures: This study was sponsored by Genentech, which markets tocilizumab (Actemra). Dr. Kim reported ties to Genentech, Lilly, Pfizer, Bristol-Myers Squibb, and AstraZeneca. Her associates reported ties to Genentech, Lilly, Pfizer, AstraZeneca, Amgen, Corrona, Whiscon, Aetion, and Boehringer Ingelheim. Three of the seven authors were employees of Genentech.

Calls for faculty participation in the CHEST PREP program

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About PREP

The CHEST PREP Clinical Immersion program is an unbranded, disease-state program that educates industry members and partners to advance their knowledge into understanding that builds their confidence for engagement in clinical

conversations with health-care teams. We are seeking faculty for the following initiatives:

1. The CHEST PREP program is embarking on a curriculum and content development initiative and is seeking interested faculty members to consider participating in the development of content in the areas of CTEPH, Alpha-1 Antitrypsin, and Bronchiectasis.

2. The CHEST PREP program is seeking interested CHEST members in Chicago-based institutions to consider participating as faculty presenters in the following disease areas: COPD, Asthma, PAH, CTEPH, IPF, SCLC, and NSCLC.

Requirements for participation

1. PREP welcomes faculty who would be interested in creating two or more presentations and/or cases on an assigned topic using a flipped classroom, interactive design. A minimum of four faculty experts will be needed per disease state indicated previously. Honorarium provided.

2. PREP welcomes faculty from Chicago-based institutions who would be interested in participating as faculty presenters in the disease states indicated previously. Honorarium provided.

Selection criteria

To be considered, please indicate the disease area in which you are interested in participating as content developer or faculty presenter, as well as providing the best way to contact you. For the asthma curriculum, we have a specific need for expertise/interest in moderate to severe asthma and the use of biologics in treatment.

If you are interested in participating in this initiative, please contact Jasmine Turner (jturner@chestnet.org). Thank you.

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About PREP

The CHEST PREP Clinical Immersion program is an unbranded, disease-state program that educates industry members and partners to advance their knowledge into understanding that builds their confidence for engagement in clinical

conversations with health-care teams. We are seeking faculty for the following initiatives:

1. The CHEST PREP program is embarking on a curriculum and content development initiative and is seeking interested faculty members to consider participating in the development of content in the areas of CTEPH, Alpha-1 Antitrypsin, and Bronchiectasis.

2. The CHEST PREP program is seeking interested CHEST members in Chicago-based institutions to consider participating as faculty presenters in the following disease areas: COPD, Asthma, PAH, CTEPH, IPF, SCLC, and NSCLC.

Requirements for participation

1. PREP welcomes faculty who would be interested in creating two or more presentations and/or cases on an assigned topic using a flipped classroom, interactive design. A minimum of four faculty experts will be needed per disease state indicated previously. Honorarium provided.

2. PREP welcomes faculty from Chicago-based institutions who would be interested in participating as faculty presenters in the disease states indicated previously. Honorarium provided.

Selection criteria

To be considered, please indicate the disease area in which you are interested in participating as content developer or faculty presenter, as well as providing the best way to contact you. For the asthma curriculum, we have a specific need for expertise/interest in moderate to severe asthma and the use of biologics in treatment.

If you are interested in participating in this initiative, please contact Jasmine Turner (jturner@chestnet.org). Thank you.

 

About PREP

The CHEST PREP Clinical Immersion program is an unbranded, disease-state program that educates industry members and partners to advance their knowledge into understanding that builds their confidence for engagement in clinical

conversations with health-care teams. We are seeking faculty for the following initiatives:

1. The CHEST PREP program is embarking on a curriculum and content development initiative and is seeking interested faculty members to consider participating in the development of content in the areas of CTEPH, Alpha-1 Antitrypsin, and Bronchiectasis.

2. The CHEST PREP program is seeking interested CHEST members in Chicago-based institutions to consider participating as faculty presenters in the following disease areas: COPD, Asthma, PAH, CTEPH, IPF, SCLC, and NSCLC.

Requirements for participation

1. PREP welcomes faculty who would be interested in creating two or more presentations and/or cases on an assigned topic using a flipped classroom, interactive design. A minimum of four faculty experts will be needed per disease state indicated previously. Honorarium provided.

2. PREP welcomes faculty from Chicago-based institutions who would be interested in participating as faculty presenters in the disease states indicated previously. Honorarium provided.

Selection criteria

To be considered, please indicate the disease area in which you are interested in participating as content developer or faculty presenter, as well as providing the best way to contact you. For the asthma curriculum, we have a specific need for expertise/interest in moderate to severe asthma and the use of biologics in treatment.

If you are interested in participating in this initiative, please contact Jasmine Turner (jturner@chestnet.org). Thank you.

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Pulmonary Hypertension Care Center initiative moves forward

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The Pulmonary Hypertension Association (PHA) launched its Pulmonary Hypertension Care Center (PHCC) initiative 2 years ago. This initiative was designed to raise the quality of care, as well as long-term outcomes for this disease that is often misdiagnosed and progressive. The PHCC program has designated 41 adult and 6 pediatric sites as Comprehensive Care Centers with ongoing accreditation of new sites. As part of this program, the PHA Registry was established to provide input to improve the care of PH patients. The PHA Registry (PHAR) is a multicenter, prospective observational registry of newly evaluated patients with pulmonary arterial hypertension (PAH) and has enrolled 200 patients to date. PHAR participation is open to any PHCC-accredited center.

PHCC accreditation has two pathways: Comprehensive Care Centers and Regional Care Centers. Accreditation is based on adherence “to consensus guidelines for the diagnosis and treatment of PH, the scope of PH-related services provided at the center, and the expertise of the center’s PH Care Team members.” PHCC accreditation is potentially available to all PH centers that meet the established criteria that can be found at the PHCC website.

Additional information may be found at the PHCC website (https://phassociation.org/PHCareCenters).

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The Pulmonary Hypertension Association (PHA) launched its Pulmonary Hypertension Care Center (PHCC) initiative 2 years ago. This initiative was designed to raise the quality of care, as well as long-term outcomes for this disease that is often misdiagnosed and progressive. The PHCC program has designated 41 adult and 6 pediatric sites as Comprehensive Care Centers with ongoing accreditation of new sites. As part of this program, the PHA Registry was established to provide input to improve the care of PH patients. The PHA Registry (PHAR) is a multicenter, prospective observational registry of newly evaluated patients with pulmonary arterial hypertension (PAH) and has enrolled 200 patients to date. PHAR participation is open to any PHCC-accredited center.

PHCC accreditation has two pathways: Comprehensive Care Centers and Regional Care Centers. Accreditation is based on adherence “to consensus guidelines for the diagnosis and treatment of PH, the scope of PH-related services provided at the center, and the expertise of the center’s PH Care Team members.” PHCC accreditation is potentially available to all PH centers that meet the established criteria that can be found at the PHCC website.

Additional information may be found at the PHCC website (https://phassociation.org/PHCareCenters).

 

The Pulmonary Hypertension Association (PHA) launched its Pulmonary Hypertension Care Center (PHCC) initiative 2 years ago. This initiative was designed to raise the quality of care, as well as long-term outcomes for this disease that is often misdiagnosed and progressive. The PHCC program has designated 41 adult and 6 pediatric sites as Comprehensive Care Centers with ongoing accreditation of new sites. As part of this program, the PHA Registry was established to provide input to improve the care of PH patients. The PHA Registry (PHAR) is a multicenter, prospective observational registry of newly evaluated patients with pulmonary arterial hypertension (PAH) and has enrolled 200 patients to date. PHAR participation is open to any PHCC-accredited center.

PHCC accreditation has two pathways: Comprehensive Care Centers and Regional Care Centers. Accreditation is based on adherence “to consensus guidelines for the diagnosis and treatment of PH, the scope of PH-related services provided at the center, and the expertise of the center’s PH Care Team members.” PHCC accreditation is potentially available to all PH centers that meet the established criteria that can be found at the PHCC website.

Additional information may be found at the PHCC website (https://phassociation.org/PHCareCenters).

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Alternative to 10-year ABIM exam starts 2018

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On December 14, the American Board of Internal Medicine (ABIM) announced an alternative to the 10-year Internal Medicine recertification exam, effective 2018. Currently, ABIM board–certified physicians can participate in Maintenance of Certification (MOC) by earning 100 MOC points every 5 years and passing a maintenance of certification exam every 10 years.

Beginning in 2018, physicians who are certified by the ABIM in Internal Medicine will have the option to take a lower-stakes exam every 2 years, rather than taking the current high-stakes exam every 10 years. The low-stakes exam option provides greater flexibility to the diplomate by allowing one to complete the examination at a convenient time set by the physician at home or in the office. While this new option will initially be available only to Internal Medicine diplomates, the ABIM intends to extend this alternative recertification model to subspecialties in the future.

CHEST is exploring how our education will evolve to address these key changes. For additional information, please visit ABIM’s website.

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On December 14, the American Board of Internal Medicine (ABIM) announced an alternative to the 10-year Internal Medicine recertification exam, effective 2018. Currently, ABIM board–certified physicians can participate in Maintenance of Certification (MOC) by earning 100 MOC points every 5 years and passing a maintenance of certification exam every 10 years.

Beginning in 2018, physicians who are certified by the ABIM in Internal Medicine will have the option to take a lower-stakes exam every 2 years, rather than taking the current high-stakes exam every 10 years. The low-stakes exam option provides greater flexibility to the diplomate by allowing one to complete the examination at a convenient time set by the physician at home or in the office. While this new option will initially be available only to Internal Medicine diplomates, the ABIM intends to extend this alternative recertification model to subspecialties in the future.

CHEST is exploring how our education will evolve to address these key changes. For additional information, please visit ABIM’s website.

 

On December 14, the American Board of Internal Medicine (ABIM) announced an alternative to the 10-year Internal Medicine recertification exam, effective 2018. Currently, ABIM board–certified physicians can participate in Maintenance of Certification (MOC) by earning 100 MOC points every 5 years and passing a maintenance of certification exam every 10 years.

Beginning in 2018, physicians who are certified by the ABIM in Internal Medicine will have the option to take a lower-stakes exam every 2 years, rather than taking the current high-stakes exam every 10 years. The low-stakes exam option provides greater flexibility to the diplomate by allowing one to complete the examination at a convenient time set by the physician at home or in the office. While this new option will initially be available only to Internal Medicine diplomates, the ABIM intends to extend this alternative recertification model to subspecialties in the future.

CHEST is exploring how our education will evolve to address these key changes. For additional information, please visit ABIM’s website.

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Optimal gestational age for cell-free DNA sampling in obese women

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Since cell-free (cf)DNA screening failure rates increase with gestational age in obese women, should cfDNA testing be delayed in this population?

cfDNA screening failures occur in 1% to 12% of samples, a rate that has an inverse relationship to gestational age. Recent studies have shown an increased risk for screening failures among obese women. To determine the optimal gestational age for cfDNA testing among obese women, Mary C. Livergood, MD, and colleagues at the Mercy Hospital in St. Louis, Missouri, performed a retrospective cohort study of those undergoing cfDNA testing at one center from 2011 through 2016. Study results recently were published online in the American Journal of Obstetrics and Gynecology.1

Details of the study

Adjusted odds ratios (aORs) with 95% confidence interval (CI) for a cfDNA screening failure (referred to as a “no call” in the study) were determined for each body mass index (BMI) weight class (TABLE). Each BMI weight class also was compared with the aOR of normal-weight women (BMI <25.0 kg/m2). The predicted probability of a no call was determined for each week of gestational age for normal weight and obese women and the results were compared.1

Among the 2,385 patients meeting inclusion criteria, 4.4% (n = 105) received a no call. Compared with normal weight women, the aOR of no call increased as weight increased from overweight (aOR, 2.31 [95% CI, 1.21–4.42]) to obesity class III (aOR, 8.55 [95% CI, 4.16–17.56]).1

At 21 weeks’ gestation, a cut-point was identified for obesity class II/III women (ie, there was no longer a significant difference in the probability of no call when compared with normal-weight women). From 8 to 16 weeks’ gestation, there was a 4.5% reduction in the probability of a no call for obesity class II/III women (aOR, 14.9; 95% CI, 8.95–20.78 and aOR, 10.4; 95% CI, 7.20–13.61; Ptrend<.01).1

Although the authors conclude that a cut-point of 21 weeks’ gestation allowed for optimal sampling of cfDNA in obese women, they also acknowledge that this cut-point limits a woman’s reproductive choices. However, they say that delaying cfDNA testing in obese women is a reasonable strategy to reduce the probability of screening failure.1

References
  1. Livergood MC, Lechien KA, Trudell AS. Obesity and cell-free DNA “no calls”: is there an optimal gestational age at time of sampling? [published online ahead of print January 28, 2017]. Am J Obstet Gynecol. doi:10.1016/j.ajog.2017.01.011.
  2. Health risks of obesity. MedlinePlus website. https://medlineplus.gov/ency/patientinstructions/000348.htm. Updated February 7, 2017. Accessed March 10, 2017.
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Since cell-free (cf)DNA screening failure rates increase with gestational age in obese women, should cfDNA testing be delayed in this population?
Since cell-free (cf)DNA screening failure rates increase with gestational age in obese women, should cfDNA testing be delayed in this population?

cfDNA screening failures occur in 1% to 12% of samples, a rate that has an inverse relationship to gestational age. Recent studies have shown an increased risk for screening failures among obese women. To determine the optimal gestational age for cfDNA testing among obese women, Mary C. Livergood, MD, and colleagues at the Mercy Hospital in St. Louis, Missouri, performed a retrospective cohort study of those undergoing cfDNA testing at one center from 2011 through 2016. Study results recently were published online in the American Journal of Obstetrics and Gynecology.1

Details of the study

Adjusted odds ratios (aORs) with 95% confidence interval (CI) for a cfDNA screening failure (referred to as a “no call” in the study) were determined for each body mass index (BMI) weight class (TABLE). Each BMI weight class also was compared with the aOR of normal-weight women (BMI <25.0 kg/m2). The predicted probability of a no call was determined for each week of gestational age for normal weight and obese women and the results were compared.1

Among the 2,385 patients meeting inclusion criteria, 4.4% (n = 105) received a no call. Compared with normal weight women, the aOR of no call increased as weight increased from overweight (aOR, 2.31 [95% CI, 1.21–4.42]) to obesity class III (aOR, 8.55 [95% CI, 4.16–17.56]).1

At 21 weeks’ gestation, a cut-point was identified for obesity class II/III women (ie, there was no longer a significant difference in the probability of no call when compared with normal-weight women). From 8 to 16 weeks’ gestation, there was a 4.5% reduction in the probability of a no call for obesity class II/III women (aOR, 14.9; 95% CI, 8.95–20.78 and aOR, 10.4; 95% CI, 7.20–13.61; Ptrend<.01).1

Although the authors conclude that a cut-point of 21 weeks’ gestation allowed for optimal sampling of cfDNA in obese women, they also acknowledge that this cut-point limits a woman’s reproductive choices. However, they say that delaying cfDNA testing in obese women is a reasonable strategy to reduce the probability of screening failure.1

cfDNA screening failures occur in 1% to 12% of samples, a rate that has an inverse relationship to gestational age. Recent studies have shown an increased risk for screening failures among obese women. To determine the optimal gestational age for cfDNA testing among obese women, Mary C. Livergood, MD, and colleagues at the Mercy Hospital in St. Louis, Missouri, performed a retrospective cohort study of those undergoing cfDNA testing at one center from 2011 through 2016. Study results recently were published online in the American Journal of Obstetrics and Gynecology.1

Details of the study

Adjusted odds ratios (aORs) with 95% confidence interval (CI) for a cfDNA screening failure (referred to as a “no call” in the study) were determined for each body mass index (BMI) weight class (TABLE). Each BMI weight class also was compared with the aOR of normal-weight women (BMI <25.0 kg/m2). The predicted probability of a no call was determined for each week of gestational age for normal weight and obese women and the results were compared.1

Among the 2,385 patients meeting inclusion criteria, 4.4% (n = 105) received a no call. Compared with normal weight women, the aOR of no call increased as weight increased from overweight (aOR, 2.31 [95% CI, 1.21–4.42]) to obesity class III (aOR, 8.55 [95% CI, 4.16–17.56]).1

At 21 weeks’ gestation, a cut-point was identified for obesity class II/III women (ie, there was no longer a significant difference in the probability of no call when compared with normal-weight women). From 8 to 16 weeks’ gestation, there was a 4.5% reduction in the probability of a no call for obesity class II/III women (aOR, 14.9; 95% CI, 8.95–20.78 and aOR, 10.4; 95% CI, 7.20–13.61; Ptrend<.01).1

Although the authors conclude that a cut-point of 21 weeks’ gestation allowed for optimal sampling of cfDNA in obese women, they also acknowledge that this cut-point limits a woman’s reproductive choices. However, they say that delaying cfDNA testing in obese women is a reasonable strategy to reduce the probability of screening failure.1

References
  1. Livergood MC, Lechien KA, Trudell AS. Obesity and cell-free DNA “no calls”: is there an optimal gestational age at time of sampling? [published online ahead of print January 28, 2017]. Am J Obstet Gynecol. doi:10.1016/j.ajog.2017.01.011.
  2. Health risks of obesity. MedlinePlus website. https://medlineplus.gov/ency/patientinstructions/000348.htm. Updated February 7, 2017. Accessed March 10, 2017.
References
  1. Livergood MC, Lechien KA, Trudell AS. Obesity and cell-free DNA “no calls”: is there an optimal gestational age at time of sampling? [published online ahead of print January 28, 2017]. Am J Obstet Gynecol. doi:10.1016/j.ajog.2017.01.011.
  2. Health risks of obesity. MedlinePlus website. https://medlineplus.gov/ency/patientinstructions/000348.htm. Updated February 7, 2017. Accessed March 10, 2017.
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Phacomatosis Cesioflammea in Association With von Recklinghausen Disease (Neurofibromatosis Type I)

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To the Editor:

Vascular lesions associated with melanocytic nevi were first described by Ota et al1 in 1947 and given the name phacomatosis pigmentovascularis. In 2005, Happle2 reclassified phacomatosis pigmentovascularis into 3 well-defined types: (1) phacomatosis cesioflammea: blue spots (caesius means bluish gray in Latin) and nevus flammeus; (2) phacomatosis spilorosea: nevus spilus coexisting with a pale pink telangiectatic nevus; and (3) phacomatosis cesiomarmorata: blue spots and cutis marmorata telangiectatica congenita. In 2011 Joshi et al3 described a case of a 31-year-old woman who had a port-wine stain in association with neurofibromatosis type I (NF-1). We present a case of phacomatosis cesioflammea in association with NF-1.

A 20-year-old woman presented to our outpatient section with a bluish black birthmark on the left side of the face since birth with the onset of multiple painless flesh-colored nodules on the trunk and arms of 1 year’s duration. She reported having occasional pruritus over the nodular lesions. Cutaneous examination showed multiple well-defined café au lait macules (0.5–3.0 cm) with regular margins. Multiple flesh-colored nodules were evident on the upper arms (Figure 1) and trunk. The nodules were firm in consistency and showed buttonholing phenomenon with some of the lesions demonstrating bag-of-worms consistency on palpation. Both palms showed multiple brownish frecklelike macules (Figure 2). A single bluish patch extended from the left ala of the nose to the sideburns. Adjoining the bluish patch was a subtle, ill-defined, nonblanchable red patch extending from the lower margin of the bluish patch to the mandibular ridge (Figure 3). Ocular examination showed melanosis bulbi of the left sclera and a few iris hamartomas (Lisch nodules) in both eyes. A biopsy of the skin nodule was obtained under local anesthesia after obtaining the patient’s informed consent; the specimen was fixed in 10% buffered formalin. A hematoxylin and eosin–stained section showed a well-circumscribed nonencapsulated tumor in the dermis composed of loosely spaced spindle cells and wavy collagenous strands (Figure 4). Routine hemogram and blood biochemistry including urinalysis were within reference range. Radiologic examination of the long bones was unremarkable. Our patient had 3 of 6 criteria defined by the National Institutes of Health for diagnosis of NF-1.4 On clinicopathological correlation we made a diagnosis of phacomatosis cesioflammea in association with NF-1. We have reassured the patient about the benign nature of vascular nevus. She was informed that the skin nodules could increase in size during pregnancy and to regularly follow-up with an eye specialist if any visual abnormalities occur.

Figure 1. Cutaneous neurofibromas (molluscum fibrosa)(black arrows) on the left upper arm.

Figure 2. Multiple brownish frecklelike macules on both palmar surfaces.

Figure 3. Evidence of twin spotting with pigmentary nevus (nevus of Ota) along with adjoining vascular nevus (nevus flammeus, port-wine stain).

Figure 4. Histopathology of a neurofibroma showing proliferation of spindle cells with elongated wavy nuclei (H&E, original magnification ×10).

The term phacomatosis is applied to genetically determined disorders of tissue derived from ectodermal origin (eg, skin, central nervous system, eyes) and commonly includes NF-1, tuberous sclerosis, and von Hippel-Lindau syndrome. Neurofibromatosis type I was first described by German pathologist Friedrich Daniel von Recklinghausen.5 Phacomatosis pigmentovascularis has been defined as the association of vascular nevus with a pigmentary nevus. Its pathogenesis can be explained by the twin spotting phenomenon.6 Twin spots are paired patches of mutant tissue that differ from each other and from the surrounding normal background skin. They can occur as 2 clinical types: allelic and nonallelic twin spotting. Our patient had nonallelic twin spots for 2 nevoid conditions: vascular (nevus flammeus) and pigmentary (nevus of Ota). Nevus of Ota was distributed in the V2 segment (maxillary nerve) of the fifth cranial nerve along with classical melanosis bulbi, which is considered a characteristic clinical feature of nevus of Ota (nevus cesius).7 Nevus flammeus (port-wine stain) is a vascular malformation presenting with flat lesions that persists throughout a patient’s life. The phenomenon of twin spotting, or didymosis (didymos means twin in Greek), has been proposed for co-occurrence of vascular and pigmented nevi.8 The association of NF-1 along with phacomatosis cesioflammea (a twin spot) could be explained from mosaicism of tissues derived from neuroectodermal and mesenchymal elements. Neurofibromatosis type I can occur as a mosaic disorder due to either postzygotic germ line or somatic mutations in the NF1 gene located on the proximal long arm of chromosome 17.9 Irrespective of the mutational event, a mosaic patient has a mixture of cells, some have normal copies of a particular gene and others have an abnormal copy of the same gene. Somatic mutation can lead to segmental (localized), generalized, or gonadal mosaicism. Somatic mutations occurring early during embryonic development produce generalized mosaicism, and generalized mosaics clinically appear similar to nonmosaic NF-1 cases.10,11 However, due to a lack of adequate facilities for mutation analysis and financial constraints, we were unable to confirm our case as generalized somatic mosaic for NF1 gene.

Several morphologic abnormalities have been reported with phacomatosis cesioflammea. Wu et al12 reported a single case of phacomatosis cesioflammea associated with pectus excavatum in a 9-month-old infant. Shields et al13 suggested that a thorough ocular examination on a periodic basis is essential to rule out melanoma of ocular tissues in patients with nevus flammeus and ocular melanosis.

Phacomatosis cesioflammea can occur in association with NF-1. The exact incidence of association is not known. The nevoid condition can be treated with appropriate lasers.

References
  1. Ota M, Kawamura T, Ito N. Phacomatosis pigmentovascularis (Ota). Jpn J Dermatol. 1947;52:1-3.
  2. Happle R. Phacomatosis pigmentovascularis revisited and reclassified. Arch Dermatol. 2005;141:385-388.
  3. Joshi A, Manchanda Y, Rijhwani M. Port-wine-stain with rare associations in two cases from Kuwait: phakomatosis pigmentovascularis redefined. Gulf J Dermatol Venereol. 2011;18:59-64.
  4. Neurofibromatosis. Conference Statement. National Institutes of Health Consensus. Arch Neurol. 1988;45:575-578.
  5. Gerber PA, Antal AS, Neumann NJ, et al. Neurofibromatosis. Eur J Med Res. 2009;14:102-105.
  6. Goyal T, Varshney A. Phacomatosis cesioflammea: first case report from India. Indian J Dermatol Venereol Leprol. 2010;76:307.
  7. Happle R. Didymosis cesioanemica: an unusual counterpart of phakomatosis cesioflammea. Eur J Dermatol. 2011;21:471.
  8. Happle R, Steijlen PM. Phacomatosis pigmentovascularis interpreted as a phenomenon of twin spots [in German]. Hautarzt. 1989;40:721-724.
  9. Adigun CG, Stein J. Segmental neurofibromatosis. Dermatol Online J. 2011;17:25.
  10. Ruggieri M, Huson SM. The clinical and diagnostic implications of mosaicism in the neurofibromatoses. Neurology. 2001;56:1433-1443.
  11. Boyd KP, Korf BR, Theos A. Neurofibromatosis type 1. J Am Acad Dermatol. 2009;61:1-14.
  12. Wu CY, Chen PH, Chen GS. Phacomatosis cesioflammea associated with pectus excavatum. Acta Derm Venereol. 2009;89:309-310.
  13. Shields CL, Kligman BE, Suriano M, et al. Phacomatosis pigmentovascularis of cesioflammea type in 7 patients: combination of ocular pigmentation (melanocytosis or melanosis) and nevus flammeus with risk for melanoma. Arch Ophthalmol. 2011;129:746-750.
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From Mahatma Gandhi Institute of Medical Sciences, Sewagram, Wardha, Maharashtra, India. Drs. Madke, Kar, and Singh are from the Department of Dermatology, Venereology, and Leprosy. Dr. Gangane is from the Department of Pathology.

The authors report no conflict of interest.

Correspondence: Sumit Kar, MD, Department of Dermatology, Venereology, and Leprosy, Mahatma Gandhi Institute of Medical Sciences, Sewagram, Wardha, Maharashtra, India (karmgims@gmail.com).

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The authors report no conflict of interest.

Correspondence: Sumit Kar, MD, Department of Dermatology, Venereology, and Leprosy, Mahatma Gandhi Institute of Medical Sciences, Sewagram, Wardha, Maharashtra, India (karmgims@gmail.com).

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From Mahatma Gandhi Institute of Medical Sciences, Sewagram, Wardha, Maharashtra, India. Drs. Madke, Kar, and Singh are from the Department of Dermatology, Venereology, and Leprosy. Dr. Gangane is from the Department of Pathology.

The authors report no conflict of interest.

Correspondence: Sumit Kar, MD, Department of Dermatology, Venereology, and Leprosy, Mahatma Gandhi Institute of Medical Sciences, Sewagram, Wardha, Maharashtra, India (karmgims@gmail.com).

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To the Editor:

Vascular lesions associated with melanocytic nevi were first described by Ota et al1 in 1947 and given the name phacomatosis pigmentovascularis. In 2005, Happle2 reclassified phacomatosis pigmentovascularis into 3 well-defined types: (1) phacomatosis cesioflammea: blue spots (caesius means bluish gray in Latin) and nevus flammeus; (2) phacomatosis spilorosea: nevus spilus coexisting with a pale pink telangiectatic nevus; and (3) phacomatosis cesiomarmorata: blue spots and cutis marmorata telangiectatica congenita. In 2011 Joshi et al3 described a case of a 31-year-old woman who had a port-wine stain in association with neurofibromatosis type I (NF-1). We present a case of phacomatosis cesioflammea in association with NF-1.

A 20-year-old woman presented to our outpatient section with a bluish black birthmark on the left side of the face since birth with the onset of multiple painless flesh-colored nodules on the trunk and arms of 1 year’s duration. She reported having occasional pruritus over the nodular lesions. Cutaneous examination showed multiple well-defined café au lait macules (0.5–3.0 cm) with regular margins. Multiple flesh-colored nodules were evident on the upper arms (Figure 1) and trunk. The nodules were firm in consistency and showed buttonholing phenomenon with some of the lesions demonstrating bag-of-worms consistency on palpation. Both palms showed multiple brownish frecklelike macules (Figure 2). A single bluish patch extended from the left ala of the nose to the sideburns. Adjoining the bluish patch was a subtle, ill-defined, nonblanchable red patch extending from the lower margin of the bluish patch to the mandibular ridge (Figure 3). Ocular examination showed melanosis bulbi of the left sclera and a few iris hamartomas (Lisch nodules) in both eyes. A biopsy of the skin nodule was obtained under local anesthesia after obtaining the patient’s informed consent; the specimen was fixed in 10% buffered formalin. A hematoxylin and eosin–stained section showed a well-circumscribed nonencapsulated tumor in the dermis composed of loosely spaced spindle cells and wavy collagenous strands (Figure 4). Routine hemogram and blood biochemistry including urinalysis were within reference range. Radiologic examination of the long bones was unremarkable. Our patient had 3 of 6 criteria defined by the National Institutes of Health for diagnosis of NF-1.4 On clinicopathological correlation we made a diagnosis of phacomatosis cesioflammea in association with NF-1. We have reassured the patient about the benign nature of vascular nevus. She was informed that the skin nodules could increase in size during pregnancy and to regularly follow-up with an eye specialist if any visual abnormalities occur.

Figure 1. Cutaneous neurofibromas (molluscum fibrosa)(black arrows) on the left upper arm.

Figure 2. Multiple brownish frecklelike macules on both palmar surfaces.

Figure 3. Evidence of twin spotting with pigmentary nevus (nevus of Ota) along with adjoining vascular nevus (nevus flammeus, port-wine stain).

Figure 4. Histopathology of a neurofibroma showing proliferation of spindle cells with elongated wavy nuclei (H&E, original magnification ×10).

The term phacomatosis is applied to genetically determined disorders of tissue derived from ectodermal origin (eg, skin, central nervous system, eyes) and commonly includes NF-1, tuberous sclerosis, and von Hippel-Lindau syndrome. Neurofibromatosis type I was first described by German pathologist Friedrich Daniel von Recklinghausen.5 Phacomatosis pigmentovascularis has been defined as the association of vascular nevus with a pigmentary nevus. Its pathogenesis can be explained by the twin spotting phenomenon.6 Twin spots are paired patches of mutant tissue that differ from each other and from the surrounding normal background skin. They can occur as 2 clinical types: allelic and nonallelic twin spotting. Our patient had nonallelic twin spots for 2 nevoid conditions: vascular (nevus flammeus) and pigmentary (nevus of Ota). Nevus of Ota was distributed in the V2 segment (maxillary nerve) of the fifth cranial nerve along with classical melanosis bulbi, which is considered a characteristic clinical feature of nevus of Ota (nevus cesius).7 Nevus flammeus (port-wine stain) is a vascular malformation presenting with flat lesions that persists throughout a patient’s life. The phenomenon of twin spotting, or didymosis (didymos means twin in Greek), has been proposed for co-occurrence of vascular and pigmented nevi.8 The association of NF-1 along with phacomatosis cesioflammea (a twin spot) could be explained from mosaicism of tissues derived from neuroectodermal and mesenchymal elements. Neurofibromatosis type I can occur as a mosaic disorder due to either postzygotic germ line or somatic mutations in the NF1 gene located on the proximal long arm of chromosome 17.9 Irrespective of the mutational event, a mosaic patient has a mixture of cells, some have normal copies of a particular gene and others have an abnormal copy of the same gene. Somatic mutation can lead to segmental (localized), generalized, or gonadal mosaicism. Somatic mutations occurring early during embryonic development produce generalized mosaicism, and generalized mosaics clinically appear similar to nonmosaic NF-1 cases.10,11 However, due to a lack of adequate facilities for mutation analysis and financial constraints, we were unable to confirm our case as generalized somatic mosaic for NF1 gene.

Several morphologic abnormalities have been reported with phacomatosis cesioflammea. Wu et al12 reported a single case of phacomatosis cesioflammea associated with pectus excavatum in a 9-month-old infant. Shields et al13 suggested that a thorough ocular examination on a periodic basis is essential to rule out melanoma of ocular tissues in patients with nevus flammeus and ocular melanosis.

Phacomatosis cesioflammea can occur in association with NF-1. The exact incidence of association is not known. The nevoid condition can be treated with appropriate lasers.

To the Editor:

Vascular lesions associated with melanocytic nevi were first described by Ota et al1 in 1947 and given the name phacomatosis pigmentovascularis. In 2005, Happle2 reclassified phacomatosis pigmentovascularis into 3 well-defined types: (1) phacomatosis cesioflammea: blue spots (caesius means bluish gray in Latin) and nevus flammeus; (2) phacomatosis spilorosea: nevus spilus coexisting with a pale pink telangiectatic nevus; and (3) phacomatosis cesiomarmorata: blue spots and cutis marmorata telangiectatica congenita. In 2011 Joshi et al3 described a case of a 31-year-old woman who had a port-wine stain in association with neurofibromatosis type I (NF-1). We present a case of phacomatosis cesioflammea in association with NF-1.

A 20-year-old woman presented to our outpatient section with a bluish black birthmark on the left side of the face since birth with the onset of multiple painless flesh-colored nodules on the trunk and arms of 1 year’s duration. She reported having occasional pruritus over the nodular lesions. Cutaneous examination showed multiple well-defined café au lait macules (0.5–3.0 cm) with regular margins. Multiple flesh-colored nodules were evident on the upper arms (Figure 1) and trunk. The nodules were firm in consistency and showed buttonholing phenomenon with some of the lesions demonstrating bag-of-worms consistency on palpation. Both palms showed multiple brownish frecklelike macules (Figure 2). A single bluish patch extended from the left ala of the nose to the sideburns. Adjoining the bluish patch was a subtle, ill-defined, nonblanchable red patch extending from the lower margin of the bluish patch to the mandibular ridge (Figure 3). Ocular examination showed melanosis bulbi of the left sclera and a few iris hamartomas (Lisch nodules) in both eyes. A biopsy of the skin nodule was obtained under local anesthesia after obtaining the patient’s informed consent; the specimen was fixed in 10% buffered formalin. A hematoxylin and eosin–stained section showed a well-circumscribed nonencapsulated tumor in the dermis composed of loosely spaced spindle cells and wavy collagenous strands (Figure 4). Routine hemogram and blood biochemistry including urinalysis were within reference range. Radiologic examination of the long bones was unremarkable. Our patient had 3 of 6 criteria defined by the National Institutes of Health for diagnosis of NF-1.4 On clinicopathological correlation we made a diagnosis of phacomatosis cesioflammea in association with NF-1. We have reassured the patient about the benign nature of vascular nevus. She was informed that the skin nodules could increase in size during pregnancy and to regularly follow-up with an eye specialist if any visual abnormalities occur.

Figure 1. Cutaneous neurofibromas (molluscum fibrosa)(black arrows) on the left upper arm.

Figure 2. Multiple brownish frecklelike macules on both palmar surfaces.

Figure 3. Evidence of twin spotting with pigmentary nevus (nevus of Ota) along with adjoining vascular nevus (nevus flammeus, port-wine stain).

Figure 4. Histopathology of a neurofibroma showing proliferation of spindle cells with elongated wavy nuclei (H&E, original magnification ×10).

The term phacomatosis is applied to genetically determined disorders of tissue derived from ectodermal origin (eg, skin, central nervous system, eyes) and commonly includes NF-1, tuberous sclerosis, and von Hippel-Lindau syndrome. Neurofibromatosis type I was first described by German pathologist Friedrich Daniel von Recklinghausen.5 Phacomatosis pigmentovascularis has been defined as the association of vascular nevus with a pigmentary nevus. Its pathogenesis can be explained by the twin spotting phenomenon.6 Twin spots are paired patches of mutant tissue that differ from each other and from the surrounding normal background skin. They can occur as 2 clinical types: allelic and nonallelic twin spotting. Our patient had nonallelic twin spots for 2 nevoid conditions: vascular (nevus flammeus) and pigmentary (nevus of Ota). Nevus of Ota was distributed in the V2 segment (maxillary nerve) of the fifth cranial nerve along with classical melanosis bulbi, which is considered a characteristic clinical feature of nevus of Ota (nevus cesius).7 Nevus flammeus (port-wine stain) is a vascular malformation presenting with flat lesions that persists throughout a patient’s life. The phenomenon of twin spotting, or didymosis (didymos means twin in Greek), has been proposed for co-occurrence of vascular and pigmented nevi.8 The association of NF-1 along with phacomatosis cesioflammea (a twin spot) could be explained from mosaicism of tissues derived from neuroectodermal and mesenchymal elements. Neurofibromatosis type I can occur as a mosaic disorder due to either postzygotic germ line or somatic mutations in the NF1 gene located on the proximal long arm of chromosome 17.9 Irrespective of the mutational event, a mosaic patient has a mixture of cells, some have normal copies of a particular gene and others have an abnormal copy of the same gene. Somatic mutation can lead to segmental (localized), generalized, or gonadal mosaicism. Somatic mutations occurring early during embryonic development produce generalized mosaicism, and generalized mosaics clinically appear similar to nonmosaic NF-1 cases.10,11 However, due to a lack of adequate facilities for mutation analysis and financial constraints, we were unable to confirm our case as generalized somatic mosaic for NF1 gene.

Several morphologic abnormalities have been reported with phacomatosis cesioflammea. Wu et al12 reported a single case of phacomatosis cesioflammea associated with pectus excavatum in a 9-month-old infant. Shields et al13 suggested that a thorough ocular examination on a periodic basis is essential to rule out melanoma of ocular tissues in patients with nevus flammeus and ocular melanosis.

Phacomatosis cesioflammea can occur in association with NF-1. The exact incidence of association is not known. The nevoid condition can be treated with appropriate lasers.

References
  1. Ota M, Kawamura T, Ito N. Phacomatosis pigmentovascularis (Ota). Jpn J Dermatol. 1947;52:1-3.
  2. Happle R. Phacomatosis pigmentovascularis revisited and reclassified. Arch Dermatol. 2005;141:385-388.
  3. Joshi A, Manchanda Y, Rijhwani M. Port-wine-stain with rare associations in two cases from Kuwait: phakomatosis pigmentovascularis redefined. Gulf J Dermatol Venereol. 2011;18:59-64.
  4. Neurofibromatosis. Conference Statement. National Institutes of Health Consensus. Arch Neurol. 1988;45:575-578.
  5. Gerber PA, Antal AS, Neumann NJ, et al. Neurofibromatosis. Eur J Med Res. 2009;14:102-105.
  6. Goyal T, Varshney A. Phacomatosis cesioflammea: first case report from India. Indian J Dermatol Venereol Leprol. 2010;76:307.
  7. Happle R. Didymosis cesioanemica: an unusual counterpart of phakomatosis cesioflammea. Eur J Dermatol. 2011;21:471.
  8. Happle R, Steijlen PM. Phacomatosis pigmentovascularis interpreted as a phenomenon of twin spots [in German]. Hautarzt. 1989;40:721-724.
  9. Adigun CG, Stein J. Segmental neurofibromatosis. Dermatol Online J. 2011;17:25.
  10. Ruggieri M, Huson SM. The clinical and diagnostic implications of mosaicism in the neurofibromatoses. Neurology. 2001;56:1433-1443.
  11. Boyd KP, Korf BR, Theos A. Neurofibromatosis type 1. J Am Acad Dermatol. 2009;61:1-14.
  12. Wu CY, Chen PH, Chen GS. Phacomatosis cesioflammea associated with pectus excavatum. Acta Derm Venereol. 2009;89:309-310.
  13. Shields CL, Kligman BE, Suriano M, et al. Phacomatosis pigmentovascularis of cesioflammea type in 7 patients: combination of ocular pigmentation (melanocytosis or melanosis) and nevus flammeus with risk for melanoma. Arch Ophthalmol. 2011;129:746-750.
References
  1. Ota M, Kawamura T, Ito N. Phacomatosis pigmentovascularis (Ota). Jpn J Dermatol. 1947;52:1-3.
  2. Happle R. Phacomatosis pigmentovascularis revisited and reclassified. Arch Dermatol. 2005;141:385-388.
  3. Joshi A, Manchanda Y, Rijhwani M. Port-wine-stain with rare associations in two cases from Kuwait: phakomatosis pigmentovascularis redefined. Gulf J Dermatol Venereol. 2011;18:59-64.
  4. Neurofibromatosis. Conference Statement. National Institutes of Health Consensus. Arch Neurol. 1988;45:575-578.
  5. Gerber PA, Antal AS, Neumann NJ, et al. Neurofibromatosis. Eur J Med Res. 2009;14:102-105.
  6. Goyal T, Varshney A. Phacomatosis cesioflammea: first case report from India. Indian J Dermatol Venereol Leprol. 2010;76:307.
  7. Happle R. Didymosis cesioanemica: an unusual counterpart of phakomatosis cesioflammea. Eur J Dermatol. 2011;21:471.
  8. Happle R, Steijlen PM. Phacomatosis pigmentovascularis interpreted as a phenomenon of twin spots [in German]. Hautarzt. 1989;40:721-724.
  9. Adigun CG, Stein J. Segmental neurofibromatosis. Dermatol Online J. 2011;17:25.
  10. Ruggieri M, Huson SM. The clinical and diagnostic implications of mosaicism in the neurofibromatoses. Neurology. 2001;56:1433-1443.
  11. Boyd KP, Korf BR, Theos A. Neurofibromatosis type 1. J Am Acad Dermatol. 2009;61:1-14.
  12. Wu CY, Chen PH, Chen GS. Phacomatosis cesioflammea associated with pectus excavatum. Acta Derm Venereol. 2009;89:309-310.
  13. Shields CL, Kligman BE, Suriano M, et al. Phacomatosis pigmentovascularis of cesioflammea type in 7 patients: combination of ocular pigmentation (melanocytosis or melanosis) and nevus flammeus with risk for melanoma. Arch Ophthalmol. 2011;129:746-750.
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Phacomatosis Cesioflammea in Association With von Recklinghausen Disease (Neurofibromatosis Type I)
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Practice Points

  • Phacomatosis cesioflammea can be associated with neurofibromatosis type I.
  • The port-wine stain component of phacomatosis cesioflammea may develop nodularity in long-standing cases.
  • The Nd:YAG laser is beneficial for treating blue spots of phacomatosis cesioflammea.
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