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Oral prophylaxis and vaginal ring effective in adolescent HIV prevention
Dapivirine vaginal ring and oral pre-exposure prophylaxis (PrEP) are effective HIV prevention measures in adolescent girls, according to two studies presented at the International AIDS Society Conference on HIV Pathogenesis and Treatment in Paris.
These solutions could be critical in lowering the rate of HIV and AIDS in girls between the ages of 15 and 25 years, a population that has proven to be particularly vulnerable to HIV infection, researchers say.
Females aged 15-24 years made up 20% of new HIV infections globally in 2015, even though they represented only 11% of the adult population, according to the National Institutes of Health (NIH).
“Adolescents and young people represent a growing share of people living with HIV worldwide,” Anthony S. Fauci, MD, director of the National Institute of Allergy and Infectious Diseases (NIAID) said in a statement released by the NIH. “Science has demonstrated that the HIV prevention needs of adolescents may be different than those of adults, which is why these new study findings are so important.”
In a phase II, double-blind, placebo-controlled trial, researchers administered a 25-mg dapivirine vaginal ring to 73 patients, with a placebo group of 23 patients, gathered from six sites across the United States once every 4 weeks over a period of 24 weeks.
Patients’ ages ranged from 15 to 17 years of age and a majority were African American, with a median of three sexual partners over the course of their lifetimes.
After the 24-week period, dapivirine residual drug levels indicated a 95% adherence rate, with a reported 42% of patients in the test group reporting never having removed the vaginal ring.
Among the patients given the ring, 63% reported never feeling the ring during intercourse, while 73% of those who said they did feel the ring reported not being bothered by it.
Overall, 93% of the study population reported not being bothered by the solution, which investigators interpreted as a positive sign for the dapivirine ring as an effective HIV prevention tool.
“We are encouraged by these results of the dapivirine ring in 15- to 17-year-old girls,” Sharon Hillier, PhD, of the NIH-funded Microbicide Trials Network (MTN), said in the NIH statement. “The study has demonstrated that the ring is safe in U.S. teens, and now we need data on the safety and acceptability of the ring in African adolescent girls. The REACH study, scheduled to launch later this year, will generate [these] data.”
In a second study presented at the conference, investigators tested the safety and acceptability of daily oral Truvada (emtricitabine/tenofovir) as a PrEP solution in 148 HIV-free adolescents aged 15-19 years from two study sites in South Africa over a span of 12 months. Truvada has not yet been approved by any national regulatory body for use as oral PrEP in adolescents.
Patients were majority female (99 girls/49 boys), with 74% of the population reporting having used a condom during their last sexual encounter.
At the start of the trial, sexually transmitted infections were present in 40% of participants, a level that remained constant throughout the study.
A total of 16 (11%) participants reported grade 2 adverse effects, including headaches, nausea, abdominal pain, and skin rashes, with another 2 patients reporting weight loss during the trial, according to Katherine Gill, MBBS, of the Desmond Tutu HIV Foundation, Cape Town, South Africa.
One instance of HIV infection was reported, although the patient in question dropped out of the program 24 weeks before diagnosis.
Overall, investigators found the Truvada PrEP program to be reasonably well tolerated, with plasma tenofovir levels detectable in 57% of participants after 12 weeks, 38% after 24 weeks and 38% at the end of the study, according to Dr. Gill and her colleagues.
Both studies were funded by NIH grants. Investigators of both studies reported no relevant financial conflicts.
ezimmerman@frontlinemedcom.com
On Twitter @eaztweets
Dapivirine vaginal ring and oral pre-exposure prophylaxis (PrEP) are effective HIV prevention measures in adolescent girls, according to two studies presented at the International AIDS Society Conference on HIV Pathogenesis and Treatment in Paris.
These solutions could be critical in lowering the rate of HIV and AIDS in girls between the ages of 15 and 25 years, a population that has proven to be particularly vulnerable to HIV infection, researchers say.
Females aged 15-24 years made up 20% of new HIV infections globally in 2015, even though they represented only 11% of the adult population, according to the National Institutes of Health (NIH).
“Adolescents and young people represent a growing share of people living with HIV worldwide,” Anthony S. Fauci, MD, director of the National Institute of Allergy and Infectious Diseases (NIAID) said in a statement released by the NIH. “Science has demonstrated that the HIV prevention needs of adolescents may be different than those of adults, which is why these new study findings are so important.”
In a phase II, double-blind, placebo-controlled trial, researchers administered a 25-mg dapivirine vaginal ring to 73 patients, with a placebo group of 23 patients, gathered from six sites across the United States once every 4 weeks over a period of 24 weeks.
Patients’ ages ranged from 15 to 17 years of age and a majority were African American, with a median of three sexual partners over the course of their lifetimes.
After the 24-week period, dapivirine residual drug levels indicated a 95% adherence rate, with a reported 42% of patients in the test group reporting never having removed the vaginal ring.
Among the patients given the ring, 63% reported never feeling the ring during intercourse, while 73% of those who said they did feel the ring reported not being bothered by it.
Overall, 93% of the study population reported not being bothered by the solution, which investigators interpreted as a positive sign for the dapivirine ring as an effective HIV prevention tool.
“We are encouraged by these results of the dapivirine ring in 15- to 17-year-old girls,” Sharon Hillier, PhD, of the NIH-funded Microbicide Trials Network (MTN), said in the NIH statement. “The study has demonstrated that the ring is safe in U.S. teens, and now we need data on the safety and acceptability of the ring in African adolescent girls. The REACH study, scheduled to launch later this year, will generate [these] data.”
In a second study presented at the conference, investigators tested the safety and acceptability of daily oral Truvada (emtricitabine/tenofovir) as a PrEP solution in 148 HIV-free adolescents aged 15-19 years from two study sites in South Africa over a span of 12 months. Truvada has not yet been approved by any national regulatory body for use as oral PrEP in adolescents.
Patients were majority female (99 girls/49 boys), with 74% of the population reporting having used a condom during their last sexual encounter.
At the start of the trial, sexually transmitted infections were present in 40% of participants, a level that remained constant throughout the study.
A total of 16 (11%) participants reported grade 2 adverse effects, including headaches, nausea, abdominal pain, and skin rashes, with another 2 patients reporting weight loss during the trial, according to Katherine Gill, MBBS, of the Desmond Tutu HIV Foundation, Cape Town, South Africa.
One instance of HIV infection was reported, although the patient in question dropped out of the program 24 weeks before diagnosis.
Overall, investigators found the Truvada PrEP program to be reasonably well tolerated, with plasma tenofovir levels detectable in 57% of participants after 12 weeks, 38% after 24 weeks and 38% at the end of the study, according to Dr. Gill and her colleagues.
Both studies were funded by NIH grants. Investigators of both studies reported no relevant financial conflicts.
ezimmerman@frontlinemedcom.com
On Twitter @eaztweets
Dapivirine vaginal ring and oral pre-exposure prophylaxis (PrEP) are effective HIV prevention measures in adolescent girls, according to two studies presented at the International AIDS Society Conference on HIV Pathogenesis and Treatment in Paris.
These solutions could be critical in lowering the rate of HIV and AIDS in girls between the ages of 15 and 25 years, a population that has proven to be particularly vulnerable to HIV infection, researchers say.
Females aged 15-24 years made up 20% of new HIV infections globally in 2015, even though they represented only 11% of the adult population, according to the National Institutes of Health (NIH).
“Adolescents and young people represent a growing share of people living with HIV worldwide,” Anthony S. Fauci, MD, director of the National Institute of Allergy and Infectious Diseases (NIAID) said in a statement released by the NIH. “Science has demonstrated that the HIV prevention needs of adolescents may be different than those of adults, which is why these new study findings are so important.”
In a phase II, double-blind, placebo-controlled trial, researchers administered a 25-mg dapivirine vaginal ring to 73 patients, with a placebo group of 23 patients, gathered from six sites across the United States once every 4 weeks over a period of 24 weeks.
Patients’ ages ranged from 15 to 17 years of age and a majority were African American, with a median of three sexual partners over the course of their lifetimes.
After the 24-week period, dapivirine residual drug levels indicated a 95% adherence rate, with a reported 42% of patients in the test group reporting never having removed the vaginal ring.
Among the patients given the ring, 63% reported never feeling the ring during intercourse, while 73% of those who said they did feel the ring reported not being bothered by it.
Overall, 93% of the study population reported not being bothered by the solution, which investigators interpreted as a positive sign for the dapivirine ring as an effective HIV prevention tool.
“We are encouraged by these results of the dapivirine ring in 15- to 17-year-old girls,” Sharon Hillier, PhD, of the NIH-funded Microbicide Trials Network (MTN), said in the NIH statement. “The study has demonstrated that the ring is safe in U.S. teens, and now we need data on the safety and acceptability of the ring in African adolescent girls. The REACH study, scheduled to launch later this year, will generate [these] data.”
In a second study presented at the conference, investigators tested the safety and acceptability of daily oral Truvada (emtricitabine/tenofovir) as a PrEP solution in 148 HIV-free adolescents aged 15-19 years from two study sites in South Africa over a span of 12 months. Truvada has not yet been approved by any national regulatory body for use as oral PrEP in adolescents.
Patients were majority female (99 girls/49 boys), with 74% of the population reporting having used a condom during their last sexual encounter.
At the start of the trial, sexually transmitted infections were present in 40% of participants, a level that remained constant throughout the study.
A total of 16 (11%) participants reported grade 2 adverse effects, including headaches, nausea, abdominal pain, and skin rashes, with another 2 patients reporting weight loss during the trial, according to Katherine Gill, MBBS, of the Desmond Tutu HIV Foundation, Cape Town, South Africa.
One instance of HIV infection was reported, although the patient in question dropped out of the program 24 weeks before diagnosis.
Overall, investigators found the Truvada PrEP program to be reasonably well tolerated, with plasma tenofovir levels detectable in 57% of participants after 12 weeks, 38% after 24 weeks and 38% at the end of the study, according to Dr. Gill and her colleagues.
Both studies were funded by NIH grants. Investigators of both studies reported no relevant financial conflicts.
ezimmerman@frontlinemedcom.com
On Twitter @eaztweets
FROM IAS 2017
South African child has suppressed HIV without ART since infancy
A 9-year-old South African child treated with antiretroviral therapy (ART) for HIV infection for 40 weeks as an infant has been living with an undetectable level of the virus ever since without ART, researchers reported on July 24.
“To our knowledge, this is the first reported case of sustained control of HIV in a child enrolled in a randomized trial of ART interruption following treatment early in infancy,” said Avy Violari, MD, at the International AIDS Society Conference on HIV Pathogenesis and Treatment in Paris.
Starting with a high viral load at 9 weeks of age, the child’s treatment brought the viral load to undetectable levels and was halted after 40 weeks. Follow-up examinations and blood sampling over the next 8.5 years showed the child in good health, with only a small reservoir of virus in a tiny portion of immune cells, but a completely undetectable viral load by standard assays. The child’s immune system is healthy, and there are no symptoms of HIV infection.
“Further study is needed to learn how to induce long-term HIV remission in infected babies,” Anthony S. Fauci, MD, director of NIAID, said in an NIH news release about the case. “However, this new case strengthens our hope that by treating HIV-infected children for a brief period beginning in infancy, we may be able to spare them the burden of lifelong therapy and the health consequences of long-term immune activation typically associated with HIV disease.”
A 9-year-old South African child treated with antiretroviral therapy (ART) for HIV infection for 40 weeks as an infant has been living with an undetectable level of the virus ever since without ART, researchers reported on July 24.
“To our knowledge, this is the first reported case of sustained control of HIV in a child enrolled in a randomized trial of ART interruption following treatment early in infancy,” said Avy Violari, MD, at the International AIDS Society Conference on HIV Pathogenesis and Treatment in Paris.
Starting with a high viral load at 9 weeks of age, the child’s treatment brought the viral load to undetectable levels and was halted after 40 weeks. Follow-up examinations and blood sampling over the next 8.5 years showed the child in good health, with only a small reservoir of virus in a tiny portion of immune cells, but a completely undetectable viral load by standard assays. The child’s immune system is healthy, and there are no symptoms of HIV infection.
“Further study is needed to learn how to induce long-term HIV remission in infected babies,” Anthony S. Fauci, MD, director of NIAID, said in an NIH news release about the case. “However, this new case strengthens our hope that by treating HIV-infected children for a brief period beginning in infancy, we may be able to spare them the burden of lifelong therapy and the health consequences of long-term immune activation typically associated with HIV disease.”
A 9-year-old South African child treated with antiretroviral therapy (ART) for HIV infection for 40 weeks as an infant has been living with an undetectable level of the virus ever since without ART, researchers reported on July 24.
“To our knowledge, this is the first reported case of sustained control of HIV in a child enrolled in a randomized trial of ART interruption following treatment early in infancy,” said Avy Violari, MD, at the International AIDS Society Conference on HIV Pathogenesis and Treatment in Paris.
Starting with a high viral load at 9 weeks of age, the child’s treatment brought the viral load to undetectable levels and was halted after 40 weeks. Follow-up examinations and blood sampling over the next 8.5 years showed the child in good health, with only a small reservoir of virus in a tiny portion of immune cells, but a completely undetectable viral load by standard assays. The child’s immune system is healthy, and there are no symptoms of HIV infection.
“Further study is needed to learn how to induce long-term HIV remission in infected babies,” Anthony S. Fauci, MD, director of NIAID, said in an NIH news release about the case. “However, this new case strengthens our hope that by treating HIV-infected children for a brief period beginning in infancy, we may be able to spare them the burden of lifelong therapy and the health consequences of long-term immune activation typically associated with HIV disease.”
FROM IAS 2017
Solid organ transplantation contributes significantly to incidence of NHL among children and adolescents
Solid organ transplant recipients contribute a disproportionate fraction of pediatric non-Hodgkin lymphoma (NHL) cases, especially diffuse large B-cell lymphoma cases (DLBCL), according to an analysis of data drawn from transplant and cancer registries.
Investigators calculated that the incidence of NHL for the pediatric transplant population was 257 times higher than the general population, after analysis from the U.S. transplant registry and 16 cancer registries from around the country between 1990 and 2012. The incidence of NHL for the pediatric transplant population was 306 cases per 100,000 person-years (95% CI, 271-344), compared to 1.19 cases per 100,000 person-years (95% CI, 1.12-1.27) in the general population. Furthermore, transplant recipients made up a much larger proportion of general population DLBCL cases (7.62%; 95% CI, 6.35%-8.88%).
In the general population, the most common subtypes were DLBCL (25% of cases), Burkitt lymphoma (24%), and precursor cell lymphoblastic lymphoma (20%). Among NHLs diagnosed in transplant recipients, 65% were DLBCL, and 9% were Burkitt lymphoma, whereas there were no cases of precursor cell lymphoblastic lymphoma, reported Elizabeth L. Yanik, PhD, of Washington University, St. Louis, and her associates (Cancer. 2017 Jul 31. doi: 10.1002/cncr.30923).
The increased risk of NHL and other cancers for organ transplant recipients is primarily the result of the immunosuppressant medications administered after transplantation, leading to an increased risk of infection-related cancers, the authors said.
“NHL cases are largely attributable to EBV [Epstein-Barr virus] infections that occur while recipients are immunosuppressed, as evidenced by the high prevalence of EBV detectable in NHL tumors and particularly in cases diagnosed during the heavily immunosuppressed period early after transplantation,” wrote Dr. Yanik and her colleagues. “Patients who experience a primary EBV infection after transplantation are particularly susceptible because transplant recipients have a higher NHL risk if they are seronegative for EBV before transplantation.”
Among those at risk, Dr. Yanik and fellow investigators found transplant patients younger than 5 years were the most susceptible to NHL, making up 19.79% of those diagnosed with DLBCL.
The proportion of NHL in solid organ transplant recipients within the entire pediatric NHL population has risen over time, from 1.66% of the NHL population during 1990-1994 to 3.73% during 2010-2012.
“This trend is driven by the rising prevalence of transplant recipients in the general population and not by increases in NHL risk among transplant recipients,” noted Dr. Yanik and her coauthors. “The proportion of NHL diagnoses attributable to transplant recipients has grown over time, and it is likely that this population will be an important source of pediatric NHL cases in the future.”
This study was partially funded by the National Cancer Institute. One coauthor reported being an employee at GRAIL Inc. No other relevant financial disclosures were reported.
ezimmerman@fronlinemedcom.com
On Twitter @eaztweets
Solid organ transplant recipients contribute a disproportionate fraction of pediatric non-Hodgkin lymphoma (NHL) cases, especially diffuse large B-cell lymphoma cases (DLBCL), according to an analysis of data drawn from transplant and cancer registries.
Investigators calculated that the incidence of NHL for the pediatric transplant population was 257 times higher than the general population, after analysis from the U.S. transplant registry and 16 cancer registries from around the country between 1990 and 2012. The incidence of NHL for the pediatric transplant population was 306 cases per 100,000 person-years (95% CI, 271-344), compared to 1.19 cases per 100,000 person-years (95% CI, 1.12-1.27) in the general population. Furthermore, transplant recipients made up a much larger proportion of general population DLBCL cases (7.62%; 95% CI, 6.35%-8.88%).
In the general population, the most common subtypes were DLBCL (25% of cases), Burkitt lymphoma (24%), and precursor cell lymphoblastic lymphoma (20%). Among NHLs diagnosed in transplant recipients, 65% were DLBCL, and 9% were Burkitt lymphoma, whereas there were no cases of precursor cell lymphoblastic lymphoma, reported Elizabeth L. Yanik, PhD, of Washington University, St. Louis, and her associates (Cancer. 2017 Jul 31. doi: 10.1002/cncr.30923).
The increased risk of NHL and other cancers for organ transplant recipients is primarily the result of the immunosuppressant medications administered after transplantation, leading to an increased risk of infection-related cancers, the authors said.
“NHL cases are largely attributable to EBV [Epstein-Barr virus] infections that occur while recipients are immunosuppressed, as evidenced by the high prevalence of EBV detectable in NHL tumors and particularly in cases diagnosed during the heavily immunosuppressed period early after transplantation,” wrote Dr. Yanik and her colleagues. “Patients who experience a primary EBV infection after transplantation are particularly susceptible because transplant recipients have a higher NHL risk if they are seronegative for EBV before transplantation.”
Among those at risk, Dr. Yanik and fellow investigators found transplant patients younger than 5 years were the most susceptible to NHL, making up 19.79% of those diagnosed with DLBCL.
The proportion of NHL in solid organ transplant recipients within the entire pediatric NHL population has risen over time, from 1.66% of the NHL population during 1990-1994 to 3.73% during 2010-2012.
“This trend is driven by the rising prevalence of transplant recipients in the general population and not by increases in NHL risk among transplant recipients,” noted Dr. Yanik and her coauthors. “The proportion of NHL diagnoses attributable to transplant recipients has grown over time, and it is likely that this population will be an important source of pediatric NHL cases in the future.”
This study was partially funded by the National Cancer Institute. One coauthor reported being an employee at GRAIL Inc. No other relevant financial disclosures were reported.
ezimmerman@fronlinemedcom.com
On Twitter @eaztweets
Solid organ transplant recipients contribute a disproportionate fraction of pediatric non-Hodgkin lymphoma (NHL) cases, especially diffuse large B-cell lymphoma cases (DLBCL), according to an analysis of data drawn from transplant and cancer registries.
Investigators calculated that the incidence of NHL for the pediatric transplant population was 257 times higher than the general population, after analysis from the U.S. transplant registry and 16 cancer registries from around the country between 1990 and 2012. The incidence of NHL for the pediatric transplant population was 306 cases per 100,000 person-years (95% CI, 271-344), compared to 1.19 cases per 100,000 person-years (95% CI, 1.12-1.27) in the general population. Furthermore, transplant recipients made up a much larger proportion of general population DLBCL cases (7.62%; 95% CI, 6.35%-8.88%).
In the general population, the most common subtypes were DLBCL (25% of cases), Burkitt lymphoma (24%), and precursor cell lymphoblastic lymphoma (20%). Among NHLs diagnosed in transplant recipients, 65% were DLBCL, and 9% were Burkitt lymphoma, whereas there were no cases of precursor cell lymphoblastic lymphoma, reported Elizabeth L. Yanik, PhD, of Washington University, St. Louis, and her associates (Cancer. 2017 Jul 31. doi: 10.1002/cncr.30923).
The increased risk of NHL and other cancers for organ transplant recipients is primarily the result of the immunosuppressant medications administered after transplantation, leading to an increased risk of infection-related cancers, the authors said.
“NHL cases are largely attributable to EBV [Epstein-Barr virus] infections that occur while recipients are immunosuppressed, as evidenced by the high prevalence of EBV detectable in NHL tumors and particularly in cases diagnosed during the heavily immunosuppressed period early after transplantation,” wrote Dr. Yanik and her colleagues. “Patients who experience a primary EBV infection after transplantation are particularly susceptible because transplant recipients have a higher NHL risk if they are seronegative for EBV before transplantation.”
Among those at risk, Dr. Yanik and fellow investigators found transplant patients younger than 5 years were the most susceptible to NHL, making up 19.79% of those diagnosed with DLBCL.
The proportion of NHL in solid organ transplant recipients within the entire pediatric NHL population has risen over time, from 1.66% of the NHL population during 1990-1994 to 3.73% during 2010-2012.
“This trend is driven by the rising prevalence of transplant recipients in the general population and not by increases in NHL risk among transplant recipients,” noted Dr. Yanik and her coauthors. “The proportion of NHL diagnoses attributable to transplant recipients has grown over time, and it is likely that this population will be an important source of pediatric NHL cases in the future.”
This study was partially funded by the National Cancer Institute. One coauthor reported being an employee at GRAIL Inc. No other relevant financial disclosures were reported.
ezimmerman@fronlinemedcom.com
On Twitter @eaztweets
FROM CANCER
Key clinical point: Solid organ transplant recipients contribute a disproportionate fraction of pediatric NHL cases, especially DLBCL cases.
Major finding: Incidence of NHL among the pediatric transplant population was 257 times higher at 306 cases per 100,000 person-years (95% CI, 271-344), compared to 1.19 cases per 100,000 person-years (95% CI, 1.12-1.27) in the general pediatric population.
Data source: Retrospective cohort study of children and adolescents in the U.S. transplant registry and 16 cancer registries from around the country between 1990 and 2012.
Disclosures: This study was partially funded by the National Cancer Institute. One coauthor reported being an employee at GRAIL Inc. No other relevant financial disclosures were reported.
Thirdhand smoke shaping up as potential health hazard
DENVER – Thirdhand smoke – the persistent residue that collects on indoor surfaces where people have smoked – is “clearly” a potentially hazardous exposure, John M. Rogers, PhD, said at the annual meeting of the Teratology Society.
Everyone knows about the hazards of secondhand smoke, which have led to widespread bans on smoking in public spaces. Still, the Centers for Disease Control and Prevention estimates that 58 million nonsmokers in the United States are exposed to secondhand smoke on a regular basis. And where there is secondhand smoke, there is typically exposure to thirdhand smoke as well.
“If you walk into a hotel room you were told is a nonsmoking room and you take one breath and you know it’s not nonsmoking, that’s thirdhand smoke. Thirdhand smoke is all over the place where smokers have been,” explained Dr. Rogers, director of the toxicity assessment division at the Environmental Protection Agency in Research Triangle Park, N.C.
The main potential health risk is to young children, who ingest thirdhand smoke by the hand-to-mouth route and skin contact.
Thirdhand smoke is a much newer concept than secondhand smoke and has not yet actually been shown to pose a significant health risk. . But that is likely to change.
Thirdhand smoke has become an area of intensive research interest, with California leading the way. The Tobacco-Related Disease Research Program, a state agency funded by a tax on the sale of tobacco products, has created a research consortium on thirdhand smoke, with studies underway investigating thirdhand smoke’s precise chemical composition, cytotoxicity, genotoxicity, and true impact on public health (www.trdrp.org).
Concern regarding thirdhand smoke’s potential public health impact ramped up in response to a study in which investigators at the University of York, England, measured levels of various tobacco-specific nitrosamines, N-nitrosamines, and nicotine in house dust samples from the homes of smokers. The researchers estimated that years of early life exposure to these compounds at the levels they detected could result in one excess case of cancer per 1,000 exposed individuals (Environ Int. 2014 Oct;71:139-47).
In addition to his update on thirdhand smoke, Dr. Rogers also touched on other recent tobacco-related developments, including a determination by the Food and Drug Administration that there has been no decline in tobacco use in the last 5 years in adolescents and young adults. While cigarette smoking by young people decreased, this was offset by a large increase in the use of electronic cigarettes and a smaller rise in the use of hookah tobacco. Indeed, e-cigarette use is now about double that of cigarettes among youth.
Also of concern is evidence of a striking socioeconomic disparity in smoking prevalence: Low-education, low-income Americans have far higher tobacco use rates.
“That’s pretty alarming,” he said. “I think a lot of people in this audience probably don’t see a lot of smoking these days, but it’s still around.”
Dr. Rogers drew attention to updated evidence reviews on the reproductive and developmental effects of smoking contained in the U.S. Surgeon General’s voluminous 2014 report on the health consequences of smoking. The report concluded that there is now sufficient evidence to infer a causal relationship between maternal smoking in pregnancy, ectopic pregnancy, and orofacial clefts. The available evidence is “suggestive but not sufficient” to infer causality between maternal smoking in pregnancy and atrial septal defects, clubfoot, gastroschisis, and attention-deficit/hyperactivity disorder and other disruptive behavior disorders.
Dr. Rogers reported having no financial disclosures related to his presentation, which he noted did not necessarily reflect the views and policies of the EPA.
DENVER – Thirdhand smoke – the persistent residue that collects on indoor surfaces where people have smoked – is “clearly” a potentially hazardous exposure, John M. Rogers, PhD, said at the annual meeting of the Teratology Society.
Everyone knows about the hazards of secondhand smoke, which have led to widespread bans on smoking in public spaces. Still, the Centers for Disease Control and Prevention estimates that 58 million nonsmokers in the United States are exposed to secondhand smoke on a regular basis. And where there is secondhand smoke, there is typically exposure to thirdhand smoke as well.
“If you walk into a hotel room you were told is a nonsmoking room and you take one breath and you know it’s not nonsmoking, that’s thirdhand smoke. Thirdhand smoke is all over the place where smokers have been,” explained Dr. Rogers, director of the toxicity assessment division at the Environmental Protection Agency in Research Triangle Park, N.C.
The main potential health risk is to young children, who ingest thirdhand smoke by the hand-to-mouth route and skin contact.
Thirdhand smoke is a much newer concept than secondhand smoke and has not yet actually been shown to pose a significant health risk. . But that is likely to change.
Thirdhand smoke has become an area of intensive research interest, with California leading the way. The Tobacco-Related Disease Research Program, a state agency funded by a tax on the sale of tobacco products, has created a research consortium on thirdhand smoke, with studies underway investigating thirdhand smoke’s precise chemical composition, cytotoxicity, genotoxicity, and true impact on public health (www.trdrp.org).
Concern regarding thirdhand smoke’s potential public health impact ramped up in response to a study in which investigators at the University of York, England, measured levels of various tobacco-specific nitrosamines, N-nitrosamines, and nicotine in house dust samples from the homes of smokers. The researchers estimated that years of early life exposure to these compounds at the levels they detected could result in one excess case of cancer per 1,000 exposed individuals (Environ Int. 2014 Oct;71:139-47).
In addition to his update on thirdhand smoke, Dr. Rogers also touched on other recent tobacco-related developments, including a determination by the Food and Drug Administration that there has been no decline in tobacco use in the last 5 years in adolescents and young adults. While cigarette smoking by young people decreased, this was offset by a large increase in the use of electronic cigarettes and a smaller rise in the use of hookah tobacco. Indeed, e-cigarette use is now about double that of cigarettes among youth.
Also of concern is evidence of a striking socioeconomic disparity in smoking prevalence: Low-education, low-income Americans have far higher tobacco use rates.
“That’s pretty alarming,” he said. “I think a lot of people in this audience probably don’t see a lot of smoking these days, but it’s still around.”
Dr. Rogers drew attention to updated evidence reviews on the reproductive and developmental effects of smoking contained in the U.S. Surgeon General’s voluminous 2014 report on the health consequences of smoking. The report concluded that there is now sufficient evidence to infer a causal relationship between maternal smoking in pregnancy, ectopic pregnancy, and orofacial clefts. The available evidence is “suggestive but not sufficient” to infer causality between maternal smoking in pregnancy and atrial septal defects, clubfoot, gastroschisis, and attention-deficit/hyperactivity disorder and other disruptive behavior disorders.
Dr. Rogers reported having no financial disclosures related to his presentation, which he noted did not necessarily reflect the views and policies of the EPA.
DENVER – Thirdhand smoke – the persistent residue that collects on indoor surfaces where people have smoked – is “clearly” a potentially hazardous exposure, John M. Rogers, PhD, said at the annual meeting of the Teratology Society.
Everyone knows about the hazards of secondhand smoke, which have led to widespread bans on smoking in public spaces. Still, the Centers for Disease Control and Prevention estimates that 58 million nonsmokers in the United States are exposed to secondhand smoke on a regular basis. And where there is secondhand smoke, there is typically exposure to thirdhand smoke as well.
“If you walk into a hotel room you were told is a nonsmoking room and you take one breath and you know it’s not nonsmoking, that’s thirdhand smoke. Thirdhand smoke is all over the place where smokers have been,” explained Dr. Rogers, director of the toxicity assessment division at the Environmental Protection Agency in Research Triangle Park, N.C.
The main potential health risk is to young children, who ingest thirdhand smoke by the hand-to-mouth route and skin contact.
Thirdhand smoke is a much newer concept than secondhand smoke and has not yet actually been shown to pose a significant health risk. . But that is likely to change.
Thirdhand smoke has become an area of intensive research interest, with California leading the way. The Tobacco-Related Disease Research Program, a state agency funded by a tax on the sale of tobacco products, has created a research consortium on thirdhand smoke, with studies underway investigating thirdhand smoke’s precise chemical composition, cytotoxicity, genotoxicity, and true impact on public health (www.trdrp.org).
Concern regarding thirdhand smoke’s potential public health impact ramped up in response to a study in which investigators at the University of York, England, measured levels of various tobacco-specific nitrosamines, N-nitrosamines, and nicotine in house dust samples from the homes of smokers. The researchers estimated that years of early life exposure to these compounds at the levels they detected could result in one excess case of cancer per 1,000 exposed individuals (Environ Int. 2014 Oct;71:139-47).
In addition to his update on thirdhand smoke, Dr. Rogers also touched on other recent tobacco-related developments, including a determination by the Food and Drug Administration that there has been no decline in tobacco use in the last 5 years in adolescents and young adults. While cigarette smoking by young people decreased, this was offset by a large increase in the use of electronic cigarettes and a smaller rise in the use of hookah tobacco. Indeed, e-cigarette use is now about double that of cigarettes among youth.
Also of concern is evidence of a striking socioeconomic disparity in smoking prevalence: Low-education, low-income Americans have far higher tobacco use rates.
“That’s pretty alarming,” he said. “I think a lot of people in this audience probably don’t see a lot of smoking these days, but it’s still around.”
Dr. Rogers drew attention to updated evidence reviews on the reproductive and developmental effects of smoking contained in the U.S. Surgeon General’s voluminous 2014 report on the health consequences of smoking. The report concluded that there is now sufficient evidence to infer a causal relationship between maternal smoking in pregnancy, ectopic pregnancy, and orofacial clefts. The available evidence is “suggestive but not sufficient” to infer causality between maternal smoking in pregnancy and atrial septal defects, clubfoot, gastroschisis, and attention-deficit/hyperactivity disorder and other disruptive behavior disorders.
Dr. Rogers reported having no financial disclosures related to his presentation, which he noted did not necessarily reflect the views and policies of the EPA.
EXPERT ANALYSIS FROM TERATOLOGY SOCIETY 2017
Inflammatory bowel disease rate higher among urban residents
Children born in urban areas are more likely to develop inflammatory bowel disease (IBD) when they grow up than are children born in rural areas, a Canadian study showed.
With rising rates of IBD in developing nations and urbanized areas, the investigators interpreted these findings as a positive step toward further understanding, and eventually eliminating, the risk of developing IBD.
The retrospective, population-based study gathered a total of 45,567 IBD patients: 6,662 living in rural residences and 38,905 living in urban residences in Nova Scotia, Ontario, Alberta, and Manitoba, Canada.
Patients in rural areas were on average older than urban patients (average age, 43 years vs. 40 years). Rural patients were also, on average, diagnosed later than were urban patients, with an average age at diagnosis of 42 years, compared with 38 years for urban residents.
The IBD incidence rate among urban patients was 33.16/100,000 (95% CI, 27.24-39.08), compared with 30.72/100,000 (95% CI, 23.81-37.64) among rural residents (Am J Gastroenterol. 2017 Jul 25. doi: 10.1038/ajg.2017.208).
Exposure to these environments while growing up was especially significant, with the lowest rate among children younger than 10 years in rural areas (incidence rate ratio, 0.58; 95% CI, 0.43-0.73), followed by adolescents between 10 and 17.9 years (IRR, 0.72; 95% CI, 0.64-0.81), according to investigators.
The incidence rate of IBD among rural children stayed consistent from birth through age 5 years, which may be evidence that development of IBD later in life is correlated with patients’ time in rural areas, the investigators reported.
Although Dr. Benchimol and his coauthors could not point to the exact reason for these results, they said factors such as diet and early exposure to animals, which may help develop useful bacteria that could help fight IBD development, are possible explanations.
“The mechanism by which rurality protects against IBD is uncertain, and may include dietary and lifestyle factors, environmental exposures, or segregation of individuals with different genetic risk profiles,” the investigators wrote. “These effects may be stronger in children because their gut microbiome is in evolution and may be vulnerable to changes in the first 2 years of life.”
This study was limited by certain classification factors, such as what constitutes an urban or rural area, which may have affected the outcomes. A lack of information on the effects of confounding factors, particularly ethnicity, genotype, phenotype, disease severity, or family history also limited this study, the investigators said.
The Janssen Future Leaders in IBD Program funded the study. Investigators reported receiving financial support from or holding leadership positions in the Canadian Institutes of Health Research, the Canadian Child Health Clinician Scientist program, and the Nova Scotia Health Research Foundation.
ezimmerman@frontlinemedcom.com
On Twitter @eaztweets
Children born in urban areas are more likely to develop inflammatory bowel disease (IBD) when they grow up than are children born in rural areas, a Canadian study showed.
With rising rates of IBD in developing nations and urbanized areas, the investigators interpreted these findings as a positive step toward further understanding, and eventually eliminating, the risk of developing IBD.
The retrospective, population-based study gathered a total of 45,567 IBD patients: 6,662 living in rural residences and 38,905 living in urban residences in Nova Scotia, Ontario, Alberta, and Manitoba, Canada.
Patients in rural areas were on average older than urban patients (average age, 43 years vs. 40 years). Rural patients were also, on average, diagnosed later than were urban patients, with an average age at diagnosis of 42 years, compared with 38 years for urban residents.
The IBD incidence rate among urban patients was 33.16/100,000 (95% CI, 27.24-39.08), compared with 30.72/100,000 (95% CI, 23.81-37.64) among rural residents (Am J Gastroenterol. 2017 Jul 25. doi: 10.1038/ajg.2017.208).
Exposure to these environments while growing up was especially significant, with the lowest rate among children younger than 10 years in rural areas (incidence rate ratio, 0.58; 95% CI, 0.43-0.73), followed by adolescents between 10 and 17.9 years (IRR, 0.72; 95% CI, 0.64-0.81), according to investigators.
The incidence rate of IBD among rural children stayed consistent from birth through age 5 years, which may be evidence that development of IBD later in life is correlated with patients’ time in rural areas, the investigators reported.
Although Dr. Benchimol and his coauthors could not point to the exact reason for these results, they said factors such as diet and early exposure to animals, which may help develop useful bacteria that could help fight IBD development, are possible explanations.
“The mechanism by which rurality protects against IBD is uncertain, and may include dietary and lifestyle factors, environmental exposures, or segregation of individuals with different genetic risk profiles,” the investigators wrote. “These effects may be stronger in children because their gut microbiome is in evolution and may be vulnerable to changes in the first 2 years of life.”
This study was limited by certain classification factors, such as what constitutes an urban or rural area, which may have affected the outcomes. A lack of information on the effects of confounding factors, particularly ethnicity, genotype, phenotype, disease severity, or family history also limited this study, the investigators said.
The Janssen Future Leaders in IBD Program funded the study. Investigators reported receiving financial support from or holding leadership positions in the Canadian Institutes of Health Research, the Canadian Child Health Clinician Scientist program, and the Nova Scotia Health Research Foundation.
ezimmerman@frontlinemedcom.com
On Twitter @eaztweets
Children born in urban areas are more likely to develop inflammatory bowel disease (IBD) when they grow up than are children born in rural areas, a Canadian study showed.
With rising rates of IBD in developing nations and urbanized areas, the investigators interpreted these findings as a positive step toward further understanding, and eventually eliminating, the risk of developing IBD.
The retrospective, population-based study gathered a total of 45,567 IBD patients: 6,662 living in rural residences and 38,905 living in urban residences in Nova Scotia, Ontario, Alberta, and Manitoba, Canada.
Patients in rural areas were on average older than urban patients (average age, 43 years vs. 40 years). Rural patients were also, on average, diagnosed later than were urban patients, with an average age at diagnosis of 42 years, compared with 38 years for urban residents.
The IBD incidence rate among urban patients was 33.16/100,000 (95% CI, 27.24-39.08), compared with 30.72/100,000 (95% CI, 23.81-37.64) among rural residents (Am J Gastroenterol. 2017 Jul 25. doi: 10.1038/ajg.2017.208).
Exposure to these environments while growing up was especially significant, with the lowest rate among children younger than 10 years in rural areas (incidence rate ratio, 0.58; 95% CI, 0.43-0.73), followed by adolescents between 10 and 17.9 years (IRR, 0.72; 95% CI, 0.64-0.81), according to investigators.
The incidence rate of IBD among rural children stayed consistent from birth through age 5 years, which may be evidence that development of IBD later in life is correlated with patients’ time in rural areas, the investigators reported.
Although Dr. Benchimol and his coauthors could not point to the exact reason for these results, they said factors such as diet and early exposure to animals, which may help develop useful bacteria that could help fight IBD development, are possible explanations.
“The mechanism by which rurality protects against IBD is uncertain, and may include dietary and lifestyle factors, environmental exposures, or segregation of individuals with different genetic risk profiles,” the investigators wrote. “These effects may be stronger in children because their gut microbiome is in evolution and may be vulnerable to changes in the first 2 years of life.”
This study was limited by certain classification factors, such as what constitutes an urban or rural area, which may have affected the outcomes. A lack of information on the effects of confounding factors, particularly ethnicity, genotype, phenotype, disease severity, or family history also limited this study, the investigators said.
The Janssen Future Leaders in IBD Program funded the study. Investigators reported receiving financial support from or holding leadership positions in the Canadian Institutes of Health Research, the Canadian Child Health Clinician Scientist program, and the Nova Scotia Health Research Foundation.
ezimmerman@frontlinemedcom.com
On Twitter @eaztweets
FROM THE AMERICAN JOURNAL OF GASTROENTEROLOGY
Key clinical point:
Major finding: The incidence of IBD among urban residents was 33.16/100,000 (95% CI, 27.24-39.08), compared with 30.72/100,000 (95% CI, 23.81-37.64) among rural residents.
Data source: A population-based, retrospective analysis of residents among four Canadian provinces between 1999 and 2010.
Disclosures: The Janssen Future Leaders in IBD Program sponsored the study. Investigators reported receiving financial support from or holding leadership positions in the Canadian Institutes of Health Research, the Canadian Child Health Clinician Scientist program, and the Nova Scotia Health Research Foundation.
Tips for navigating pityriasis lichenoides in children
CHICAGO – Pityriasis lichenoides chronica (PLC) is a benign, chronic disorder that can last from 1 to 5 years, yet no formal treatment standards exist.
“Topical corticosteroids will not alter the course of the disease, but they provide symptomatic relief when there is pruritus,” Sibel Ersoy-Evans, MD, said at the World Congress of Pediatric Dermatology. “Oral antibiotics, especially erythromycin, and phototherapy are the most common modalities used in children. Systemic immunosuppressants are rarely needed.”
Dr. Ersoy-Evans, a dermatologist at Hacettepe University in Ankara, Turkey, described pityriasis lichenoides (PL) as a spectrum with two polar ends: PLC and pityriasis lichenoides et varioliformis acuta (PLEVA). Its incidence and prevalence are unknown, but age distribution peaks at 2, 5, and 10 years of age. The average age of onset is 6.5 years, it’s slightly more common in boys, and there is no racial or ethnic predilection.
PLEVA presents with symmetrical, reddish-brown papules that later evolve into vesicular, purpuric, necrotic papules. The scalp, face, mucosa, palms, and soles are spared. Resolution occurs with varioliform scarring and dyspigmentation in 2-18 months. On the other hand, chronic PL is characterized by asymptomatic scaly papules and plaques. The lesions start de novo or evolve from PLEVA lesions and they resolve with hypo-hyperpigmentation in about 8-20 months. In what is believed to be the largest study of its kind, Dr. Ersoy-Evans and her associates respectively reviewed 124 patients with PL. They observed that 57% of patients had PLEVA, primarily those who were white, and 37% had PLC (J Am Acad Dermatol. 2007;56[2]:205-10). The median age of onset was 6 years in PLC patients, versus 5 years in those with PLEVA. Age peaks were observed at 2-3 years and 5-7 years.
They also observed that 30% of cases had a history of recent infection prior to the eruption. “Most patients had generalized distribution, and there wasn’t a significant difference in duration according to the distribution of the lesions,” she said. “Postinflammatory pigmentary changes were observed in 90% of the cases, most commonly hypopigmentation. Pruritus was the most common symptom observed, and the disease was relapsing/recurring in 77% of patients. The duration was longer in PL patients.”
A separate study of 25 children and 32 adults with PL found that hypopigmentation was more common in children (72% vs. 19%, respectively), fewer children experienced remission (20% vs. 78%), and phototherapy was more effective than oral antibiotics in children (Br J Dermatol. 2007;157[5]:941-5).
PL runs a chronic, remitting course in 25%-77% of patients, but there are infrequent reports about progression into cutaneous T-cell lymphoma. “We have not observed any lymphoma development in our cohort with a 10-year follow-up, but long-term follow-up is necessary, especially if clonality is present,” Dr. Ersoy-Evans said. The diagnosis of PL is mostly clinical, but histologic examination may be necessary in some cases. “CD8-positive cells predominate in PLEVA, whereas CD4-positive cells predominate in PLC,” she said.
In the review of 124 patients that she and her associates published in 2007, erythromycin estolate or ethylsuccinate was administered to 80% of children, and 67% of these children showed at least a partial response, with a median response time of 2 months.
In a separate study of 24 children with PL, the response rate was highest (83%) after 3.8 months of erythromycin therapy (Ped Dermatol. 2012; 29[6]:719-24). Dr. Ersoy-Evans stated that oral erythromycin should be continued at least 2 months for a response.
A separate study evaluated the use of phototherapy in 18 children with PLC for a mean of 3.7 months (Ped Dermatol 2008;25[6]:599-605). Among 12 patients who received broadband ultraviolet B radiation for a mean of 3.7 months, the response rate was 83%, which was achieved after a mean of 18 sessions. All five patients who received narrow-band UVB cleared, and the mean number of sessions for a response was 22. One patient who received psoralen and ultraviolet A therapy had no response. A larger review of the published literature found that the delivery of narrow-band UVB has the lowest recurrence rate in PL patients treated with phototherapy (Am J Clin Dermatol. 2016;17:583-91). “Given the efficacy and favorable side effects, I think that phototherapy can be a promising option for PL in children,” Dr. Ersoy-Evans said.
She reported having no financial disclosures
CHICAGO – Pityriasis lichenoides chronica (PLC) is a benign, chronic disorder that can last from 1 to 5 years, yet no formal treatment standards exist.
“Topical corticosteroids will not alter the course of the disease, but they provide symptomatic relief when there is pruritus,” Sibel Ersoy-Evans, MD, said at the World Congress of Pediatric Dermatology. “Oral antibiotics, especially erythromycin, and phototherapy are the most common modalities used in children. Systemic immunosuppressants are rarely needed.”
Dr. Ersoy-Evans, a dermatologist at Hacettepe University in Ankara, Turkey, described pityriasis lichenoides (PL) as a spectrum with two polar ends: PLC and pityriasis lichenoides et varioliformis acuta (PLEVA). Its incidence and prevalence are unknown, but age distribution peaks at 2, 5, and 10 years of age. The average age of onset is 6.5 years, it’s slightly more common in boys, and there is no racial or ethnic predilection.
PLEVA presents with symmetrical, reddish-brown papules that later evolve into vesicular, purpuric, necrotic papules. The scalp, face, mucosa, palms, and soles are spared. Resolution occurs with varioliform scarring and dyspigmentation in 2-18 months. On the other hand, chronic PL is characterized by asymptomatic scaly papules and plaques. The lesions start de novo or evolve from PLEVA lesions and they resolve with hypo-hyperpigmentation in about 8-20 months. In what is believed to be the largest study of its kind, Dr. Ersoy-Evans and her associates respectively reviewed 124 patients with PL. They observed that 57% of patients had PLEVA, primarily those who were white, and 37% had PLC (J Am Acad Dermatol. 2007;56[2]:205-10). The median age of onset was 6 years in PLC patients, versus 5 years in those with PLEVA. Age peaks were observed at 2-3 years and 5-7 years.
They also observed that 30% of cases had a history of recent infection prior to the eruption. “Most patients had generalized distribution, and there wasn’t a significant difference in duration according to the distribution of the lesions,” she said. “Postinflammatory pigmentary changes were observed in 90% of the cases, most commonly hypopigmentation. Pruritus was the most common symptom observed, and the disease was relapsing/recurring in 77% of patients. The duration was longer in PL patients.”
A separate study of 25 children and 32 adults with PL found that hypopigmentation was more common in children (72% vs. 19%, respectively), fewer children experienced remission (20% vs. 78%), and phototherapy was more effective than oral antibiotics in children (Br J Dermatol. 2007;157[5]:941-5).
PL runs a chronic, remitting course in 25%-77% of patients, but there are infrequent reports about progression into cutaneous T-cell lymphoma. “We have not observed any lymphoma development in our cohort with a 10-year follow-up, but long-term follow-up is necessary, especially if clonality is present,” Dr. Ersoy-Evans said. The diagnosis of PL is mostly clinical, but histologic examination may be necessary in some cases. “CD8-positive cells predominate in PLEVA, whereas CD4-positive cells predominate in PLC,” she said.
In the review of 124 patients that she and her associates published in 2007, erythromycin estolate or ethylsuccinate was administered to 80% of children, and 67% of these children showed at least a partial response, with a median response time of 2 months.
In a separate study of 24 children with PL, the response rate was highest (83%) after 3.8 months of erythromycin therapy (Ped Dermatol. 2012; 29[6]:719-24). Dr. Ersoy-Evans stated that oral erythromycin should be continued at least 2 months for a response.
A separate study evaluated the use of phototherapy in 18 children with PLC for a mean of 3.7 months (Ped Dermatol 2008;25[6]:599-605). Among 12 patients who received broadband ultraviolet B radiation for a mean of 3.7 months, the response rate was 83%, which was achieved after a mean of 18 sessions. All five patients who received narrow-band UVB cleared, and the mean number of sessions for a response was 22. One patient who received psoralen and ultraviolet A therapy had no response. A larger review of the published literature found that the delivery of narrow-band UVB has the lowest recurrence rate in PL patients treated with phototherapy (Am J Clin Dermatol. 2016;17:583-91). “Given the efficacy and favorable side effects, I think that phototherapy can be a promising option for PL in children,” Dr. Ersoy-Evans said.
She reported having no financial disclosures
CHICAGO – Pityriasis lichenoides chronica (PLC) is a benign, chronic disorder that can last from 1 to 5 years, yet no formal treatment standards exist.
“Topical corticosteroids will not alter the course of the disease, but they provide symptomatic relief when there is pruritus,” Sibel Ersoy-Evans, MD, said at the World Congress of Pediatric Dermatology. “Oral antibiotics, especially erythromycin, and phototherapy are the most common modalities used in children. Systemic immunosuppressants are rarely needed.”
Dr. Ersoy-Evans, a dermatologist at Hacettepe University in Ankara, Turkey, described pityriasis lichenoides (PL) as a spectrum with two polar ends: PLC and pityriasis lichenoides et varioliformis acuta (PLEVA). Its incidence and prevalence are unknown, but age distribution peaks at 2, 5, and 10 years of age. The average age of onset is 6.5 years, it’s slightly more common in boys, and there is no racial or ethnic predilection.
PLEVA presents with symmetrical, reddish-brown papules that later evolve into vesicular, purpuric, necrotic papules. The scalp, face, mucosa, palms, and soles are spared. Resolution occurs with varioliform scarring and dyspigmentation in 2-18 months. On the other hand, chronic PL is characterized by asymptomatic scaly papules and plaques. The lesions start de novo or evolve from PLEVA lesions and they resolve with hypo-hyperpigmentation in about 8-20 months. In what is believed to be the largest study of its kind, Dr. Ersoy-Evans and her associates respectively reviewed 124 patients with PL. They observed that 57% of patients had PLEVA, primarily those who were white, and 37% had PLC (J Am Acad Dermatol. 2007;56[2]:205-10). The median age of onset was 6 years in PLC patients, versus 5 years in those with PLEVA. Age peaks were observed at 2-3 years and 5-7 years.
They also observed that 30% of cases had a history of recent infection prior to the eruption. “Most patients had generalized distribution, and there wasn’t a significant difference in duration according to the distribution of the lesions,” she said. “Postinflammatory pigmentary changes were observed in 90% of the cases, most commonly hypopigmentation. Pruritus was the most common symptom observed, and the disease was relapsing/recurring in 77% of patients. The duration was longer in PL patients.”
A separate study of 25 children and 32 adults with PL found that hypopigmentation was more common in children (72% vs. 19%, respectively), fewer children experienced remission (20% vs. 78%), and phototherapy was more effective than oral antibiotics in children (Br J Dermatol. 2007;157[5]:941-5).
PL runs a chronic, remitting course in 25%-77% of patients, but there are infrequent reports about progression into cutaneous T-cell lymphoma. “We have not observed any lymphoma development in our cohort with a 10-year follow-up, but long-term follow-up is necessary, especially if clonality is present,” Dr. Ersoy-Evans said. The diagnosis of PL is mostly clinical, but histologic examination may be necessary in some cases. “CD8-positive cells predominate in PLEVA, whereas CD4-positive cells predominate in PLC,” she said.
In the review of 124 patients that she and her associates published in 2007, erythromycin estolate or ethylsuccinate was administered to 80% of children, and 67% of these children showed at least a partial response, with a median response time of 2 months.
In a separate study of 24 children with PL, the response rate was highest (83%) after 3.8 months of erythromycin therapy (Ped Dermatol. 2012; 29[6]:719-24). Dr. Ersoy-Evans stated that oral erythromycin should be continued at least 2 months for a response.
A separate study evaluated the use of phototherapy in 18 children with PLC for a mean of 3.7 months (Ped Dermatol 2008;25[6]:599-605). Among 12 patients who received broadband ultraviolet B radiation for a mean of 3.7 months, the response rate was 83%, which was achieved after a mean of 18 sessions. All five patients who received narrow-band UVB cleared, and the mean number of sessions for a response was 22. One patient who received psoralen and ultraviolet A therapy had no response. A larger review of the published literature found that the delivery of narrow-band UVB has the lowest recurrence rate in PL patients treated with phototherapy (Am J Clin Dermatol. 2016;17:583-91). “Given the efficacy and favorable side effects, I think that phototherapy can be a promising option for PL in children,” Dr. Ersoy-Evans said.
She reported having no financial disclosures
AT WCPD 2017
Alopecia may be permanent in one in four pediatric HSCT patients
CHICAGO – Late dermatologic manifestations in children who have received hematopoietic stem cell transplants may be more common than previously thought, according to results of a new study.
Johanna Song, MD, and her collaborators reported that, in their prospective pediatric study, 25% of patients had permanent alopecia and 16% had psoriasis, noting that late nonmalignant skin effects of hematopoietic stem cell transplantation (HSCT) have been studied primarily retrospectively, and in adults. Vitiligo and nail changes were also seen.
In a poster presentation at the World Congress of Dermatology, Dr. Song and her colleagues noted that these figures are higher than the previously reported pediatric rates of 1.7% for vitiligo and 15.6% for permanent alopecia. “Early recognition of these late effects can facilitate prompt and appropriate treatment, if desired,” they said.
The single-center, cross-sectional cohort study tracked pediatric patients over an 18-month period and included patients who were at least 1 year post allogeneic HSCT and had not relapsed. Patients who were not English speaking were excluded.
The median age of the 85 patients enrolled in the study was 13.8 years, and participants were a median of 3.6 years post transplant at the time of enrollment. The study’s analysis attempted to determine which patient, transplant, and disease factors might be associated with the late nonmalignant skin changes, according to Dr. Song, a resident dermatologist at Harvard University, Boston, and her colleagues.
Most – 52– of the patients (61.2%) had hematologic malignancies; 12 patients (14.1%) received their transplant for bone marrow failure, and 11 patients (12.5%) had immunodeficiency. Three patients (3.5%) received HSCT for other malignancies, and seven (8.2%) for nonmalignant diseases.
Diffuse hair thinning was seen in 13 (62%) of the 21 patients who had alopecia, while 11 (52%) of the patients with alopecia had an androgenetic hair loss pattern. Chronic graft versus host disease (GVHD), skin chronic GVHD, a HSCT regimen that included busulfan conditioning, and a family history of early male-pattern alopecia were all significantly associated with post-HSCT permanent alopecia (P less than .05 for all).
The patients with androgenetic alopecia may be experiencing an accelerated time course of a condition to which they are already genetically disposed, noted Dr. Song and her colleagues.
Psoriasis was commonly seen on the scalp, affecting 11 of the 14 patients with psoriasis (79%), and involved the face in five of the patients (36%). Just one patient had psoriasis elsewhere on the body. There was a nonsignificant trend towards human leukocyte antigen mismatch among patients who had psoriasis. Although “psoriasis may be a marker of persistent immune dysregulation,” the investigators said, they did not identify any associated risk factors that would point toward this mechanism in their analysis.
Twelve patients (14%) had vitiligo, with halo nevi seen in four of these patients. Children who were younger than age 10 years and those who received their transplant for primary immunodeficiency were significantly more likely to have vitiligo (P less than .05 for both). Specific possible mechanisms triggering vitiligo could include thymic dysfunction resulting in loss of self-tolerance, and donor alloreactivity against the patient’s host antigens, according to the authors.
Nail changes such as pterygium and nail pitting, ridging, or thickening were seen in just five patients, all of whom had chronic GVHD of the skin. “Nail changes are likely a result of persistent inflammation and immune dysregulation from chronic GVHD,” said Dr. Song.
These late effects “can significantly impact patients’ quality of life,” according to the authors, who called for larger studies that follow pediatric HSCT patients longitudinally, beginning before the transplant – and for more investigation into the pathogenesis of specific late effects.
Dr. Song reported no relevant conflicts of interest.
koakes@frontlinemedcom.com
On Twitter @karioakes
CHICAGO – Late dermatologic manifestations in children who have received hematopoietic stem cell transplants may be more common than previously thought, according to results of a new study.
Johanna Song, MD, and her collaborators reported that, in their prospective pediatric study, 25% of patients had permanent alopecia and 16% had psoriasis, noting that late nonmalignant skin effects of hematopoietic stem cell transplantation (HSCT) have been studied primarily retrospectively, and in adults. Vitiligo and nail changes were also seen.
In a poster presentation at the World Congress of Dermatology, Dr. Song and her colleagues noted that these figures are higher than the previously reported pediatric rates of 1.7% for vitiligo and 15.6% for permanent alopecia. “Early recognition of these late effects can facilitate prompt and appropriate treatment, if desired,” they said.
The single-center, cross-sectional cohort study tracked pediatric patients over an 18-month period and included patients who were at least 1 year post allogeneic HSCT and had not relapsed. Patients who were not English speaking were excluded.
The median age of the 85 patients enrolled in the study was 13.8 years, and participants were a median of 3.6 years post transplant at the time of enrollment. The study’s analysis attempted to determine which patient, transplant, and disease factors might be associated with the late nonmalignant skin changes, according to Dr. Song, a resident dermatologist at Harvard University, Boston, and her colleagues.
Most – 52– of the patients (61.2%) had hematologic malignancies; 12 patients (14.1%) received their transplant for bone marrow failure, and 11 patients (12.5%) had immunodeficiency. Three patients (3.5%) received HSCT for other malignancies, and seven (8.2%) for nonmalignant diseases.
Diffuse hair thinning was seen in 13 (62%) of the 21 patients who had alopecia, while 11 (52%) of the patients with alopecia had an androgenetic hair loss pattern. Chronic graft versus host disease (GVHD), skin chronic GVHD, a HSCT regimen that included busulfan conditioning, and a family history of early male-pattern alopecia were all significantly associated with post-HSCT permanent alopecia (P less than .05 for all).
The patients with androgenetic alopecia may be experiencing an accelerated time course of a condition to which they are already genetically disposed, noted Dr. Song and her colleagues.
Psoriasis was commonly seen on the scalp, affecting 11 of the 14 patients with psoriasis (79%), and involved the face in five of the patients (36%). Just one patient had psoriasis elsewhere on the body. There was a nonsignificant trend towards human leukocyte antigen mismatch among patients who had psoriasis. Although “psoriasis may be a marker of persistent immune dysregulation,” the investigators said, they did not identify any associated risk factors that would point toward this mechanism in their analysis.
Twelve patients (14%) had vitiligo, with halo nevi seen in four of these patients. Children who were younger than age 10 years and those who received their transplant for primary immunodeficiency were significantly more likely to have vitiligo (P less than .05 for both). Specific possible mechanisms triggering vitiligo could include thymic dysfunction resulting in loss of self-tolerance, and donor alloreactivity against the patient’s host antigens, according to the authors.
Nail changes such as pterygium and nail pitting, ridging, or thickening were seen in just five patients, all of whom had chronic GVHD of the skin. “Nail changes are likely a result of persistent inflammation and immune dysregulation from chronic GVHD,” said Dr. Song.
These late effects “can significantly impact patients’ quality of life,” according to the authors, who called for larger studies that follow pediatric HSCT patients longitudinally, beginning before the transplant – and for more investigation into the pathogenesis of specific late effects.
Dr. Song reported no relevant conflicts of interest.
koakes@frontlinemedcom.com
On Twitter @karioakes
CHICAGO – Late dermatologic manifestations in children who have received hematopoietic stem cell transplants may be more common than previously thought, according to results of a new study.
Johanna Song, MD, and her collaborators reported that, in their prospective pediatric study, 25% of patients had permanent alopecia and 16% had psoriasis, noting that late nonmalignant skin effects of hematopoietic stem cell transplantation (HSCT) have been studied primarily retrospectively, and in adults. Vitiligo and nail changes were also seen.
In a poster presentation at the World Congress of Dermatology, Dr. Song and her colleagues noted that these figures are higher than the previously reported pediatric rates of 1.7% for vitiligo and 15.6% for permanent alopecia. “Early recognition of these late effects can facilitate prompt and appropriate treatment, if desired,” they said.
The single-center, cross-sectional cohort study tracked pediatric patients over an 18-month period and included patients who were at least 1 year post allogeneic HSCT and had not relapsed. Patients who were not English speaking were excluded.
The median age of the 85 patients enrolled in the study was 13.8 years, and participants were a median of 3.6 years post transplant at the time of enrollment. The study’s analysis attempted to determine which patient, transplant, and disease factors might be associated with the late nonmalignant skin changes, according to Dr. Song, a resident dermatologist at Harvard University, Boston, and her colleagues.
Most – 52– of the patients (61.2%) had hematologic malignancies; 12 patients (14.1%) received their transplant for bone marrow failure, and 11 patients (12.5%) had immunodeficiency. Three patients (3.5%) received HSCT for other malignancies, and seven (8.2%) for nonmalignant diseases.
Diffuse hair thinning was seen in 13 (62%) of the 21 patients who had alopecia, while 11 (52%) of the patients with alopecia had an androgenetic hair loss pattern. Chronic graft versus host disease (GVHD), skin chronic GVHD, a HSCT regimen that included busulfan conditioning, and a family history of early male-pattern alopecia were all significantly associated with post-HSCT permanent alopecia (P less than .05 for all).
The patients with androgenetic alopecia may be experiencing an accelerated time course of a condition to which they are already genetically disposed, noted Dr. Song and her colleagues.
Psoriasis was commonly seen on the scalp, affecting 11 of the 14 patients with psoriasis (79%), and involved the face in five of the patients (36%). Just one patient had psoriasis elsewhere on the body. There was a nonsignificant trend towards human leukocyte antigen mismatch among patients who had psoriasis. Although “psoriasis may be a marker of persistent immune dysregulation,” the investigators said, they did not identify any associated risk factors that would point toward this mechanism in their analysis.
Twelve patients (14%) had vitiligo, with halo nevi seen in four of these patients. Children who were younger than age 10 years and those who received their transplant for primary immunodeficiency were significantly more likely to have vitiligo (P less than .05 for both). Specific possible mechanisms triggering vitiligo could include thymic dysfunction resulting in loss of self-tolerance, and donor alloreactivity against the patient’s host antigens, according to the authors.
Nail changes such as pterygium and nail pitting, ridging, or thickening were seen in just five patients, all of whom had chronic GVHD of the skin. “Nail changes are likely a result of persistent inflammation and immune dysregulation from chronic GVHD,” said Dr. Song.
These late effects “can significantly impact patients’ quality of life,” according to the authors, who called for larger studies that follow pediatric HSCT patients longitudinally, beginning before the transplant – and for more investigation into the pathogenesis of specific late effects.
Dr. Song reported no relevant conflicts of interest.
koakes@frontlinemedcom.com
On Twitter @karioakes
AT WCPD 2017
Key clinical point:
Major finding: Permanent alopecia was seen in 25% of patients, and 16% had psoriasis a median of 3.6 years after transplant.
Data source: A single-center prospective study of 85 children in routine post-HSCT follow-up.
Disclosures: Dr. Song reported no conflicts of interest.
Care of infants with ichthyosis requires ‘all hands on deck’
CHICAGO – The neonatal period and early infancy are especially critical for patients with ichthyosis, because compromised barrier function increases risk for morbidity and mortality.
“There are minimal data presently to guide management of patients with ichthyosis, making it a time of uncertainty,” Brittany Craiglow, MD, said at the World Congress of Pediatric Dermatology. “You’re going to want to get all hands on deck for the care of these patients. And don’t forget about the family – involve them in the care as much as possible. Reassure them; normalize their feelings, acknowledge them.”
There are six general phenotypes of ichthyosis that differ from the eventual “mature” phenotype and are associated with numerous genes: collodion baby, armor-like scale, exuberant vernix, erythroderma and scale, bullae and erosions, and generalized scale.
The collodion baby phenotype is characterized by a shiny parchment-like membrane that covers the baby’s body, ectropion, and fissures, and is commonly associated with autosomal recessive congenital ichthyosis (ARCI). “About 10% of babies with ARCI are self healing, so they’ll go on to have largely normal skin,” Dr. Craiglow said. Guidelines for managing this phenotype can be found in the Journal of the American Academy of Dermatology (2012 Dec;67[6]:1362-74).
Armor-like scale is pathognomonic for harlequin ichthyosis. “This condition is associated with the highest mortality in the neonatal period,” she said. “In addition to the potential complications associated with other phenotypes, babies with harlequin ichthyosis can also have issues related to constriction of movement and flexibility and digital ischemia.” Tips for practical management of this phenotype were published online in the journal Pediatrics (2017 Jan;139[1]).
The exuberant vernix/cephalic hyperkeratosis phenotype generally appears in children with keratitis-ichthyosis-deafness syndrome (KID) and ichthyosis prematurity syndrome (IPS). Special considerations in KID include a hearing test and ophthalmology exam, while special considerations in IPS include respiratory compromise and atopic diathesis. Electron microscopy is diagnostic, characterized by curvilinear bodies in the granular layer.
The erythroderma and scale phenotype occurs most commonly in ARCI and Netherton syndrome. “Special considerations in Netherton syndrome include failure to thrive/growth failure,” Dr. Craiglow said. “Hair shaft abnormalities are usually present later, and nutritional support is really important.”
Bullae and erosions are hallmark signs of epidermolytic/superficial ichthyosis. On biopsy, epidermolytic hyperkeratosis is diagnostic for this phenotype. At the same time, cases with normal skin or xerosis are suggestive of X-linked ichthyosis, ichthyosis vulgaris, erythrokeratodermas, and Sjögren-Larsson syndrome.
Genetic testing for ichthyosis is generally readily available, Dr. Craiglow said. She advised clinicians to obtain a sample soon after birth to confirm the clinical diagnosis, assist with assessing prognosis, and enable genetic counseling. “It’s important to help identify those at risk for systemic complications,” she said. “Obtaining insurance coverage may be easier when sent during hospital admission.”
Babies with moderate to severe congenital ichthyosis are typically cared for in the neonatal ICU of a tertiary care center by a multidisciplinary team consisting of neonatology, dermatology, nursing, nutrition, and genetics, as well as ophthalmology, otolaryngology, orthopedics, plastic surgery, and spiritual/religious services in many cases. “These babies often have impaired thermoregulation,” Dr. Craiglow said. “They need to be in an isolette, generally with humidity somewhere between 50% and 70% – you don’t want it too high, because they can overheat. It’s also important to get them out of the isolette and into an open crib when they’re ready. That can help with bonding and has been shown to decrease hospital stay.”
Infection is a common culprit for morbidity and mortality. “In general, there are not a lot of data to guide our management; but generally, we don’t recommend prophylactic antibiotics,” Dr. Craiglow said. “Some people do surveillance cultures just to know what microbes are there in case there are signs of infection. Look for level of alertness, because they’re not always going to have a fever. Look for hemodynamic instability, irritability, or poor feeding, and have a low threshold to do your cultures and treat if necessary.”
Pain control is an imperative aspect of pain management.
“Typical newborn pain parameters of facial expression and extremity tone may be hard to interpret,” she said. “Look at heart rate, blood pressure, crying, level of arousal, and have a low threshold to treat for pain, especially prior to changing dressings. Acetaminophen and NSAIDs and even opioids in some cases might be indicated. Families want to know that pain is being adequately controlled.”
Retinoids are generally used in patients with harlequin ichthyosis. “In the United States, we generally use acitretin, but there is no liquid formulation, so you have to enlist help from a compounding pharmacy to mix a formulation of 0.5-0.1 mg/kg per day,” Dr. Craiglow said. “You want to start as soon as you can. Topical retinoids such as tazarotene are also an option.”
Resources that she recommends for parents include the Foundation for Ichthyosis and Related Skin Types, the Ichthyosis Support Group, and the European Network for Ichthyosis.
Dr. Craiglow reported having no relevant financial disclosures.
CHICAGO – The neonatal period and early infancy are especially critical for patients with ichthyosis, because compromised barrier function increases risk for morbidity and mortality.
“There are minimal data presently to guide management of patients with ichthyosis, making it a time of uncertainty,” Brittany Craiglow, MD, said at the World Congress of Pediatric Dermatology. “You’re going to want to get all hands on deck for the care of these patients. And don’t forget about the family – involve them in the care as much as possible. Reassure them; normalize their feelings, acknowledge them.”
There are six general phenotypes of ichthyosis that differ from the eventual “mature” phenotype and are associated with numerous genes: collodion baby, armor-like scale, exuberant vernix, erythroderma and scale, bullae and erosions, and generalized scale.
The collodion baby phenotype is characterized by a shiny parchment-like membrane that covers the baby’s body, ectropion, and fissures, and is commonly associated with autosomal recessive congenital ichthyosis (ARCI). “About 10% of babies with ARCI are self healing, so they’ll go on to have largely normal skin,” Dr. Craiglow said. Guidelines for managing this phenotype can be found in the Journal of the American Academy of Dermatology (2012 Dec;67[6]:1362-74).
Armor-like scale is pathognomonic for harlequin ichthyosis. “This condition is associated with the highest mortality in the neonatal period,” she said. “In addition to the potential complications associated with other phenotypes, babies with harlequin ichthyosis can also have issues related to constriction of movement and flexibility and digital ischemia.” Tips for practical management of this phenotype were published online in the journal Pediatrics (2017 Jan;139[1]).
The exuberant vernix/cephalic hyperkeratosis phenotype generally appears in children with keratitis-ichthyosis-deafness syndrome (KID) and ichthyosis prematurity syndrome (IPS). Special considerations in KID include a hearing test and ophthalmology exam, while special considerations in IPS include respiratory compromise and atopic diathesis. Electron microscopy is diagnostic, characterized by curvilinear bodies in the granular layer.
The erythroderma and scale phenotype occurs most commonly in ARCI and Netherton syndrome. “Special considerations in Netherton syndrome include failure to thrive/growth failure,” Dr. Craiglow said. “Hair shaft abnormalities are usually present later, and nutritional support is really important.”
Bullae and erosions are hallmark signs of epidermolytic/superficial ichthyosis. On biopsy, epidermolytic hyperkeratosis is diagnostic for this phenotype. At the same time, cases with normal skin or xerosis are suggestive of X-linked ichthyosis, ichthyosis vulgaris, erythrokeratodermas, and Sjögren-Larsson syndrome.
Genetic testing for ichthyosis is generally readily available, Dr. Craiglow said. She advised clinicians to obtain a sample soon after birth to confirm the clinical diagnosis, assist with assessing prognosis, and enable genetic counseling. “It’s important to help identify those at risk for systemic complications,” she said. “Obtaining insurance coverage may be easier when sent during hospital admission.”
Babies with moderate to severe congenital ichthyosis are typically cared for in the neonatal ICU of a tertiary care center by a multidisciplinary team consisting of neonatology, dermatology, nursing, nutrition, and genetics, as well as ophthalmology, otolaryngology, orthopedics, plastic surgery, and spiritual/religious services in many cases. “These babies often have impaired thermoregulation,” Dr. Craiglow said. “They need to be in an isolette, generally with humidity somewhere between 50% and 70% – you don’t want it too high, because they can overheat. It’s also important to get them out of the isolette and into an open crib when they’re ready. That can help with bonding and has been shown to decrease hospital stay.”
Infection is a common culprit for morbidity and mortality. “In general, there are not a lot of data to guide our management; but generally, we don’t recommend prophylactic antibiotics,” Dr. Craiglow said. “Some people do surveillance cultures just to know what microbes are there in case there are signs of infection. Look for level of alertness, because they’re not always going to have a fever. Look for hemodynamic instability, irritability, or poor feeding, and have a low threshold to do your cultures and treat if necessary.”
Pain control is an imperative aspect of pain management.
“Typical newborn pain parameters of facial expression and extremity tone may be hard to interpret,” she said. “Look at heart rate, blood pressure, crying, level of arousal, and have a low threshold to treat for pain, especially prior to changing dressings. Acetaminophen and NSAIDs and even opioids in some cases might be indicated. Families want to know that pain is being adequately controlled.”
Retinoids are generally used in patients with harlequin ichthyosis. “In the United States, we generally use acitretin, but there is no liquid formulation, so you have to enlist help from a compounding pharmacy to mix a formulation of 0.5-0.1 mg/kg per day,” Dr. Craiglow said. “You want to start as soon as you can. Topical retinoids such as tazarotene are also an option.”
Resources that she recommends for parents include the Foundation for Ichthyosis and Related Skin Types, the Ichthyosis Support Group, and the European Network for Ichthyosis.
Dr. Craiglow reported having no relevant financial disclosures.
CHICAGO – The neonatal period and early infancy are especially critical for patients with ichthyosis, because compromised barrier function increases risk for morbidity and mortality.
“There are minimal data presently to guide management of patients with ichthyosis, making it a time of uncertainty,” Brittany Craiglow, MD, said at the World Congress of Pediatric Dermatology. “You’re going to want to get all hands on deck for the care of these patients. And don’t forget about the family – involve them in the care as much as possible. Reassure them; normalize their feelings, acknowledge them.”
There are six general phenotypes of ichthyosis that differ from the eventual “mature” phenotype and are associated with numerous genes: collodion baby, armor-like scale, exuberant vernix, erythroderma and scale, bullae and erosions, and generalized scale.
The collodion baby phenotype is characterized by a shiny parchment-like membrane that covers the baby’s body, ectropion, and fissures, and is commonly associated with autosomal recessive congenital ichthyosis (ARCI). “About 10% of babies with ARCI are self healing, so they’ll go on to have largely normal skin,” Dr. Craiglow said. Guidelines for managing this phenotype can be found in the Journal of the American Academy of Dermatology (2012 Dec;67[6]:1362-74).
Armor-like scale is pathognomonic for harlequin ichthyosis. “This condition is associated with the highest mortality in the neonatal period,” she said. “In addition to the potential complications associated with other phenotypes, babies with harlequin ichthyosis can also have issues related to constriction of movement and flexibility and digital ischemia.” Tips for practical management of this phenotype were published online in the journal Pediatrics (2017 Jan;139[1]).
The exuberant vernix/cephalic hyperkeratosis phenotype generally appears in children with keratitis-ichthyosis-deafness syndrome (KID) and ichthyosis prematurity syndrome (IPS). Special considerations in KID include a hearing test and ophthalmology exam, while special considerations in IPS include respiratory compromise and atopic diathesis. Electron microscopy is diagnostic, characterized by curvilinear bodies in the granular layer.
The erythroderma and scale phenotype occurs most commonly in ARCI and Netherton syndrome. “Special considerations in Netherton syndrome include failure to thrive/growth failure,” Dr. Craiglow said. “Hair shaft abnormalities are usually present later, and nutritional support is really important.”
Bullae and erosions are hallmark signs of epidermolytic/superficial ichthyosis. On biopsy, epidermolytic hyperkeratosis is diagnostic for this phenotype. At the same time, cases with normal skin or xerosis are suggestive of X-linked ichthyosis, ichthyosis vulgaris, erythrokeratodermas, and Sjögren-Larsson syndrome.
Genetic testing for ichthyosis is generally readily available, Dr. Craiglow said. She advised clinicians to obtain a sample soon after birth to confirm the clinical diagnosis, assist with assessing prognosis, and enable genetic counseling. “It’s important to help identify those at risk for systemic complications,” she said. “Obtaining insurance coverage may be easier when sent during hospital admission.”
Babies with moderate to severe congenital ichthyosis are typically cared for in the neonatal ICU of a tertiary care center by a multidisciplinary team consisting of neonatology, dermatology, nursing, nutrition, and genetics, as well as ophthalmology, otolaryngology, orthopedics, plastic surgery, and spiritual/religious services in many cases. “These babies often have impaired thermoregulation,” Dr. Craiglow said. “They need to be in an isolette, generally with humidity somewhere between 50% and 70% – you don’t want it too high, because they can overheat. It’s also important to get them out of the isolette and into an open crib when they’re ready. That can help with bonding and has been shown to decrease hospital stay.”
Infection is a common culprit for morbidity and mortality. “In general, there are not a lot of data to guide our management; but generally, we don’t recommend prophylactic antibiotics,” Dr. Craiglow said. “Some people do surveillance cultures just to know what microbes are there in case there are signs of infection. Look for level of alertness, because they’re not always going to have a fever. Look for hemodynamic instability, irritability, or poor feeding, and have a low threshold to do your cultures and treat if necessary.”
Pain control is an imperative aspect of pain management.
“Typical newborn pain parameters of facial expression and extremity tone may be hard to interpret,” she said. “Look at heart rate, blood pressure, crying, level of arousal, and have a low threshold to treat for pain, especially prior to changing dressings. Acetaminophen and NSAIDs and even opioids in some cases might be indicated. Families want to know that pain is being adequately controlled.”
Retinoids are generally used in patients with harlequin ichthyosis. “In the United States, we generally use acitretin, but there is no liquid formulation, so you have to enlist help from a compounding pharmacy to mix a formulation of 0.5-0.1 mg/kg per day,” Dr. Craiglow said. “You want to start as soon as you can. Topical retinoids such as tazarotene are also an option.”
Resources that she recommends for parents include the Foundation for Ichthyosis and Related Skin Types, the Ichthyosis Support Group, and the European Network for Ichthyosis.
Dr. Craiglow reported having no relevant financial disclosures.
AT WCPD 2017
Precise cause of pityriasis rosea remains elusive
CHICAGO – Pityriasis rosea was recognized as early as 1798, yet its precise cause remains elusive.
“We still don’t have a lot of information on it, because it’s self limited and it resolves,” John C. Browning, MD, said at the World Congress of Pediatric Dermatology. “There hasn’t been quite as much of a burning push for research into pityriasis rosea as there has been for pityriasis rubra pilaris, for instance.”
Classic pityriasis rosea (PR) is characterized by oval scaly, erythematous lesions on the trunk and extremities, sparing the face, scalp, palms, and soles, said Dr. Browning, a pediatric dermatologist who is chief of dermatology at the Children’s Hospital of San Antonio, Texas. The hallmark sign is a so-called herald patch, an oval, slightly scaly patch with a pale center, which usually appears on the trunk and remains isolated for about 2 weeks before the generalized papulosquamous eruption begins. This typically lasts for about 45 days but can range from 2 weeks to 5 months.
Symptoms of PR may include malaise, nausea, loss of appetite, headache, difficulty concentrating, irritability, gastrointestinal upset, upper respiratory symptoms, joint pain, lymph node swelling, sore throat, and low-grade fever. Pruritus is variable, both in frequency and in intensity, and can be exacerbated by topical medications. Some studies have found a higher female-to-male ratio, while other studies have shown no such association.
“Only 6% of PR cases have been reported in children under 10 years of age,” Dr. Browning said. “PR in dark-skinned children tends to have more facial involvement and a scaly appearance, compared with lighter-skinned children.”
Human herpesvirus (HHV) 6 and HHV 7, members of the Roseolovirus genus of HHVs, have been implicated in triggering PR. These viruses cause a primary infection and can establish latent infection with reactivation if altered immunity develops.
“That’s probably why we don’t see PR in younger children, because that’s when the primary infection is happening,” Dr. Browning said. “These viruses are commonly acquired during childhood, with adult seroprevalence in the range of 80%-90%. Latency occurs in monocytes, bone marrow progenitor cells, in salivary glands, the brain, and in the kidneys, so it’s pretty widespread.”
He added that controversy exists as to whether HHV 6 and 7 cause PR, because older diagnostic methods only detected the presence of HHV DNA, rather than viral load. “HHV reactivation, rather than primary infection, is more likely the cause of PR as supported by sporadic occurrence of PR, reduced contagiousness, age of PR onset, possible relapse during a limited span of time, and frequent occurrence after stress or immunosuppressive states such as pregnancy,” Dr. Browning said.
Classical presentations of PR are not clinically worrisome, but atypical cases could indicate other triggers, such as drug-induced PR. “This is typically more itchy, can have mucous membrane involvement, and you’re not going to see the herald patch,” he said. “It’s more likely to be confluent with no prodromal symptoms.” Agents that have been implicated in drug-induced PR include isotretinoin, terbinafine, and adalimumab.
Other conditions that can trigger PR include secondary syphilis (characterized by involvement of the palms and soles, lymphadenopathy, and greater lesional infiltration); seborrheic dermatitis (characterized by greater involvement of the scalp and other hairy parts of the body); nummular eczema (more pruritic); and pityriasis lichenoides chronica, which involves more chronic and relapsing lesions. Histology of PR reveals focal parakeratosis in the epidermis in mounds with exocytosis of lymphocytes, variable spongiosis, mild acanthosis, and a thinned granular layer.
Dr. Browning noted that PR is more common in pregnancy. One study of 38 pregnant women with PR found that 13% miscarried before 16 weeks, compared with a normal miscarriage rate of 10% (J Am Acad Dermatol. 2008 May; 58[5 Suppl 1]:S78-83). “Neonatal hypotonia, weal motility, and hyporeactivity have been reported,” he said. “And with immunocompromised patients, you might see a longer, protracted course of PR.”
Although no treatment is recommended for classical cases of PR, Dr. Browning said that topical steroids “are widely employed because we all want to do something, especially if there’s some pruritus.” According to a position statement on the management of patients with PR, erythromycin has been reported to shorten the duration of rash and pruritus, but it can cause gastrointestinal disturbance (J Eur Acad Dermatol Venereol. 2016 Oct;30[10]:1670-81). Acyclovir has been reported to hasten clearance of PR in one placebo-controlled study, but PR has also been reported in another patient taking low doses of acyclovir. Phototherapy has also been found to be beneficial.
Dr. Browning reported having no financial disclosures.
CHICAGO – Pityriasis rosea was recognized as early as 1798, yet its precise cause remains elusive.
“We still don’t have a lot of information on it, because it’s self limited and it resolves,” John C. Browning, MD, said at the World Congress of Pediatric Dermatology. “There hasn’t been quite as much of a burning push for research into pityriasis rosea as there has been for pityriasis rubra pilaris, for instance.”
Classic pityriasis rosea (PR) is characterized by oval scaly, erythematous lesions on the trunk and extremities, sparing the face, scalp, palms, and soles, said Dr. Browning, a pediatric dermatologist who is chief of dermatology at the Children’s Hospital of San Antonio, Texas. The hallmark sign is a so-called herald patch, an oval, slightly scaly patch with a pale center, which usually appears on the trunk and remains isolated for about 2 weeks before the generalized papulosquamous eruption begins. This typically lasts for about 45 days but can range from 2 weeks to 5 months.
Symptoms of PR may include malaise, nausea, loss of appetite, headache, difficulty concentrating, irritability, gastrointestinal upset, upper respiratory symptoms, joint pain, lymph node swelling, sore throat, and low-grade fever. Pruritus is variable, both in frequency and in intensity, and can be exacerbated by topical medications. Some studies have found a higher female-to-male ratio, while other studies have shown no such association.
“Only 6% of PR cases have been reported in children under 10 years of age,” Dr. Browning said. “PR in dark-skinned children tends to have more facial involvement and a scaly appearance, compared with lighter-skinned children.”
Human herpesvirus (HHV) 6 and HHV 7, members of the Roseolovirus genus of HHVs, have been implicated in triggering PR. These viruses cause a primary infection and can establish latent infection with reactivation if altered immunity develops.
“That’s probably why we don’t see PR in younger children, because that’s when the primary infection is happening,” Dr. Browning said. “These viruses are commonly acquired during childhood, with adult seroprevalence in the range of 80%-90%. Latency occurs in monocytes, bone marrow progenitor cells, in salivary glands, the brain, and in the kidneys, so it’s pretty widespread.”
He added that controversy exists as to whether HHV 6 and 7 cause PR, because older diagnostic methods only detected the presence of HHV DNA, rather than viral load. “HHV reactivation, rather than primary infection, is more likely the cause of PR as supported by sporadic occurrence of PR, reduced contagiousness, age of PR onset, possible relapse during a limited span of time, and frequent occurrence after stress or immunosuppressive states such as pregnancy,” Dr. Browning said.
Classical presentations of PR are not clinically worrisome, but atypical cases could indicate other triggers, such as drug-induced PR. “This is typically more itchy, can have mucous membrane involvement, and you’re not going to see the herald patch,” he said. “It’s more likely to be confluent with no prodromal symptoms.” Agents that have been implicated in drug-induced PR include isotretinoin, terbinafine, and adalimumab.
Other conditions that can trigger PR include secondary syphilis (characterized by involvement of the palms and soles, lymphadenopathy, and greater lesional infiltration); seborrheic dermatitis (characterized by greater involvement of the scalp and other hairy parts of the body); nummular eczema (more pruritic); and pityriasis lichenoides chronica, which involves more chronic and relapsing lesions. Histology of PR reveals focal parakeratosis in the epidermis in mounds with exocytosis of lymphocytes, variable spongiosis, mild acanthosis, and a thinned granular layer.
Dr. Browning noted that PR is more common in pregnancy. One study of 38 pregnant women with PR found that 13% miscarried before 16 weeks, compared with a normal miscarriage rate of 10% (J Am Acad Dermatol. 2008 May; 58[5 Suppl 1]:S78-83). “Neonatal hypotonia, weal motility, and hyporeactivity have been reported,” he said. “And with immunocompromised patients, you might see a longer, protracted course of PR.”
Although no treatment is recommended for classical cases of PR, Dr. Browning said that topical steroids “are widely employed because we all want to do something, especially if there’s some pruritus.” According to a position statement on the management of patients with PR, erythromycin has been reported to shorten the duration of rash and pruritus, but it can cause gastrointestinal disturbance (J Eur Acad Dermatol Venereol. 2016 Oct;30[10]:1670-81). Acyclovir has been reported to hasten clearance of PR in one placebo-controlled study, but PR has also been reported in another patient taking low doses of acyclovir. Phototherapy has also been found to be beneficial.
Dr. Browning reported having no financial disclosures.
CHICAGO – Pityriasis rosea was recognized as early as 1798, yet its precise cause remains elusive.
“We still don’t have a lot of information on it, because it’s self limited and it resolves,” John C. Browning, MD, said at the World Congress of Pediatric Dermatology. “There hasn’t been quite as much of a burning push for research into pityriasis rosea as there has been for pityriasis rubra pilaris, for instance.”
Classic pityriasis rosea (PR) is characterized by oval scaly, erythematous lesions on the trunk and extremities, sparing the face, scalp, palms, and soles, said Dr. Browning, a pediatric dermatologist who is chief of dermatology at the Children’s Hospital of San Antonio, Texas. The hallmark sign is a so-called herald patch, an oval, slightly scaly patch with a pale center, which usually appears on the trunk and remains isolated for about 2 weeks before the generalized papulosquamous eruption begins. This typically lasts for about 45 days but can range from 2 weeks to 5 months.
Symptoms of PR may include malaise, nausea, loss of appetite, headache, difficulty concentrating, irritability, gastrointestinal upset, upper respiratory symptoms, joint pain, lymph node swelling, sore throat, and low-grade fever. Pruritus is variable, both in frequency and in intensity, and can be exacerbated by topical medications. Some studies have found a higher female-to-male ratio, while other studies have shown no such association.
“Only 6% of PR cases have been reported in children under 10 years of age,” Dr. Browning said. “PR in dark-skinned children tends to have more facial involvement and a scaly appearance, compared with lighter-skinned children.”
Human herpesvirus (HHV) 6 and HHV 7, members of the Roseolovirus genus of HHVs, have been implicated in triggering PR. These viruses cause a primary infection and can establish latent infection with reactivation if altered immunity develops.
“That’s probably why we don’t see PR in younger children, because that’s when the primary infection is happening,” Dr. Browning said. “These viruses are commonly acquired during childhood, with adult seroprevalence in the range of 80%-90%. Latency occurs in monocytes, bone marrow progenitor cells, in salivary glands, the brain, and in the kidneys, so it’s pretty widespread.”
He added that controversy exists as to whether HHV 6 and 7 cause PR, because older diagnostic methods only detected the presence of HHV DNA, rather than viral load. “HHV reactivation, rather than primary infection, is more likely the cause of PR as supported by sporadic occurrence of PR, reduced contagiousness, age of PR onset, possible relapse during a limited span of time, and frequent occurrence after stress or immunosuppressive states such as pregnancy,” Dr. Browning said.
Classical presentations of PR are not clinically worrisome, but atypical cases could indicate other triggers, such as drug-induced PR. “This is typically more itchy, can have mucous membrane involvement, and you’re not going to see the herald patch,” he said. “It’s more likely to be confluent with no prodromal symptoms.” Agents that have been implicated in drug-induced PR include isotretinoin, terbinafine, and adalimumab.
Other conditions that can trigger PR include secondary syphilis (characterized by involvement of the palms and soles, lymphadenopathy, and greater lesional infiltration); seborrheic dermatitis (characterized by greater involvement of the scalp and other hairy parts of the body); nummular eczema (more pruritic); and pityriasis lichenoides chronica, which involves more chronic and relapsing lesions. Histology of PR reveals focal parakeratosis in the epidermis in mounds with exocytosis of lymphocytes, variable spongiosis, mild acanthosis, and a thinned granular layer.
Dr. Browning noted that PR is more common in pregnancy. One study of 38 pregnant women with PR found that 13% miscarried before 16 weeks, compared with a normal miscarriage rate of 10% (J Am Acad Dermatol. 2008 May; 58[5 Suppl 1]:S78-83). “Neonatal hypotonia, weal motility, and hyporeactivity have been reported,” he said. “And with immunocompromised patients, you might see a longer, protracted course of PR.”
Although no treatment is recommended for classical cases of PR, Dr. Browning said that topical steroids “are widely employed because we all want to do something, especially if there’s some pruritus.” According to a position statement on the management of patients with PR, erythromycin has been reported to shorten the duration of rash and pruritus, but it can cause gastrointestinal disturbance (J Eur Acad Dermatol Venereol. 2016 Oct;30[10]:1670-81). Acyclovir has been reported to hasten clearance of PR in one placebo-controlled study, but PR has also been reported in another patient taking low doses of acyclovir. Phototherapy has also been found to be beneficial.
Dr. Browning reported having no financial disclosures.
EXPERT ANALYSIS FROM WCPD 2017
PHM17 session summary: Kawasaki Disease updates
NASHVILLE, TENN. – A panel of experts discussed highlights from the 2017 AHA Kawasaki Guidelines at Pediatric Hospital Medicine, sponsored by the Society of Hospital Medicine, the American Academy of Pediatrics, and the Academic Pediatric Association.
Session
Kawasaki Disease Reconsidered: New AHA Guidelines
Presenters
John Darby, MD, Marietta DeGuzman, MD, Kristen Sexson, MD, PhD, MPH, Stanford Shulman, MD, Nisha Tamaskar, MD
Session summary
For the second year in a row, the session highlighting American Heart Association updates on Kawasaki disease did not disappoint and again attracted a large crowd of community and academic pediatric hospitalists. The newly-revised 2017 AHA Kawasaki Guidelines hot off the press by McCrindle et al. was reviewed in detail.
A secondary theory investigates the tropospheric wind patterns from central Asia and has indicated a possible link to outbreaks of KD in Chile. Despite previous investigation of carpet cleaning and risk for KD, no causal link has been identified.
Experts addressed pathophysiology, diagnosis, and management. Below are highlights from the new 2017 AHA Kawasaki Guideline Update in conjunction with points from the panel discussion.
Pathophysiology
• Cause is likely to be a common ubiquitous agent that in genetically inclined children will lead to a particular inflammatory response that manifests clinically as KD.
• A new theory about how the “ubiquitous agent” is spread by wind patterns.
Diagnosis
• Confirmed that infants younger than 1 year of age are more difficult to diagnose because they don’t present classically so it must be on the differential.
• The new algorithm makes it clearer that infants with fever for 7 days without symptoms should get lab screening tests for KD.
• Those who have classic symptoms and lab abnormalities consistent with KD but in whom fever is still at 3-4 days may be diagnosed with KD prior to the “5 days of fever rule” because these tend to be a sicker cohort of patients with higher rate of complications. Pretest probability and suspicion for KD must be high to treat before 5 days.
• Importance of the Z-score when evaluating an echocardiogram completed on a patient with suspected KD was stressed with a score greater than or equal to 2.5 reaching a level of significance for the patient’s body size.
Management
• It is still agreed that IVIG is first line therapy.
• For refractory KD (not responsive within 36 hours of first dose IVIG), management is more controversial. Experts on the panel agreed that they would likely provide a second dose of IVIG before thinking about steroids.
• Moderate dose aspirin is just as effective as high dose aspirin in the acute phase of KD.
• For more detailed information regarding the role of corticosteroids in KD, refer to Dr. Carl Galloway’s article in The Hospitalist July 2017 issue.
• A certain subset of patients may benefit from steroids if given early in the disease course, including those who present in shock syndrome. Steroids would still be in conjunction with IVIG treatment.
• Even though the new guidelines recommend a longer course of steroids for those refractory cases of KD in high-risk patients, panel experts are still unsure about evidence behind the claim.
The RAISE study was referenced and indicates there are significantly different outcomes for patients with severe disease placed on steroid therapy in combination with IVIG. In this group of patients, the incidence of coronary artery aneurysms was 23% in the IVIG-only group compared to 3% in the IVIG + steroid group (P less than .0001). This study and a recent Cochrane review that supported use of steroids in KD were completed in a homogeneous population of Japanese children and may not be generalizable to children in the United States.
Hyponatremia has been used as a diagnostic criterion for severe KD in Japanese children and was referenced as an indicator for addition of steroid therapy. Also, studies investigating the necessity of ASA at 80-100 mg/kg/d, a common practice for patients with KD treated in the United States, were compared to medium-dose ASA (30-50 mg/kg/d). There was no clinically significant difference in patient outcome or development of aneurysm formation between these two dosing regimens.
Key takeaways for Pediatric HM
• Diagnosis of classic KD remains unchanged and includes 5 or more days of fever and at least four clinical features (extremity changes, rash, conjunctivitis, oral changes, and cervical lymphadenopathy).
• Infants with fever of 7 days or more without other explanation should be evaluated for KD.
• Echocardiographic findings should be adjusted for body surface area and are significant if Z-score greater than or equal to 2.5.
• Moderate- to high-dose ASA is appropriate as an adjunct to IVIG until the patient is afebrile.
• Steroid therapy (for a total of 14 days) should be considered for high-risk patients.
Dr. King is associate program director, University of Minnesota Pediatric Residency Program. Dr. Hopkins is assistant professor of pediatrics, Johns Hopkins All Children’s Hospital.
NASHVILLE, TENN. – A panel of experts discussed highlights from the 2017 AHA Kawasaki Guidelines at Pediatric Hospital Medicine, sponsored by the Society of Hospital Medicine, the American Academy of Pediatrics, and the Academic Pediatric Association.
Session
Kawasaki Disease Reconsidered: New AHA Guidelines
Presenters
John Darby, MD, Marietta DeGuzman, MD, Kristen Sexson, MD, PhD, MPH, Stanford Shulman, MD, Nisha Tamaskar, MD
Session summary
For the second year in a row, the session highlighting American Heart Association updates on Kawasaki disease did not disappoint and again attracted a large crowd of community and academic pediatric hospitalists. The newly-revised 2017 AHA Kawasaki Guidelines hot off the press by McCrindle et al. was reviewed in detail.
A secondary theory investigates the tropospheric wind patterns from central Asia and has indicated a possible link to outbreaks of KD in Chile. Despite previous investigation of carpet cleaning and risk for KD, no causal link has been identified.
Experts addressed pathophysiology, diagnosis, and management. Below are highlights from the new 2017 AHA Kawasaki Guideline Update in conjunction with points from the panel discussion.
Pathophysiology
• Cause is likely to be a common ubiquitous agent that in genetically inclined children will lead to a particular inflammatory response that manifests clinically as KD.
• A new theory about how the “ubiquitous agent” is spread by wind patterns.
Diagnosis
• Confirmed that infants younger than 1 year of age are more difficult to diagnose because they don’t present classically so it must be on the differential.
• The new algorithm makes it clearer that infants with fever for 7 days without symptoms should get lab screening tests for KD.
• Those who have classic symptoms and lab abnormalities consistent with KD but in whom fever is still at 3-4 days may be diagnosed with KD prior to the “5 days of fever rule” because these tend to be a sicker cohort of patients with higher rate of complications. Pretest probability and suspicion for KD must be high to treat before 5 days.
• Importance of the Z-score when evaluating an echocardiogram completed on a patient with suspected KD was stressed with a score greater than or equal to 2.5 reaching a level of significance for the patient’s body size.
Management
• It is still agreed that IVIG is first line therapy.
• For refractory KD (not responsive within 36 hours of first dose IVIG), management is more controversial. Experts on the panel agreed that they would likely provide a second dose of IVIG before thinking about steroids.
• Moderate dose aspirin is just as effective as high dose aspirin in the acute phase of KD.
• For more detailed information regarding the role of corticosteroids in KD, refer to Dr. Carl Galloway’s article in The Hospitalist July 2017 issue.
• A certain subset of patients may benefit from steroids if given early in the disease course, including those who present in shock syndrome. Steroids would still be in conjunction with IVIG treatment.
• Even though the new guidelines recommend a longer course of steroids for those refractory cases of KD in high-risk patients, panel experts are still unsure about evidence behind the claim.
The RAISE study was referenced and indicates there are significantly different outcomes for patients with severe disease placed on steroid therapy in combination with IVIG. In this group of patients, the incidence of coronary artery aneurysms was 23% in the IVIG-only group compared to 3% in the IVIG + steroid group (P less than .0001). This study and a recent Cochrane review that supported use of steroids in KD were completed in a homogeneous population of Japanese children and may not be generalizable to children in the United States.
Hyponatremia has been used as a diagnostic criterion for severe KD in Japanese children and was referenced as an indicator for addition of steroid therapy. Also, studies investigating the necessity of ASA at 80-100 mg/kg/d, a common practice for patients with KD treated in the United States, were compared to medium-dose ASA (30-50 mg/kg/d). There was no clinically significant difference in patient outcome or development of aneurysm formation between these two dosing regimens.
Key takeaways for Pediatric HM
• Diagnosis of classic KD remains unchanged and includes 5 or more days of fever and at least four clinical features (extremity changes, rash, conjunctivitis, oral changes, and cervical lymphadenopathy).
• Infants with fever of 7 days or more without other explanation should be evaluated for KD.
• Echocardiographic findings should be adjusted for body surface area and are significant if Z-score greater than or equal to 2.5.
• Moderate- to high-dose ASA is appropriate as an adjunct to IVIG until the patient is afebrile.
• Steroid therapy (for a total of 14 days) should be considered for high-risk patients.
Dr. King is associate program director, University of Minnesota Pediatric Residency Program. Dr. Hopkins is assistant professor of pediatrics, Johns Hopkins All Children’s Hospital.
NASHVILLE, TENN. – A panel of experts discussed highlights from the 2017 AHA Kawasaki Guidelines at Pediatric Hospital Medicine, sponsored by the Society of Hospital Medicine, the American Academy of Pediatrics, and the Academic Pediatric Association.
Session
Kawasaki Disease Reconsidered: New AHA Guidelines
Presenters
John Darby, MD, Marietta DeGuzman, MD, Kristen Sexson, MD, PhD, MPH, Stanford Shulman, MD, Nisha Tamaskar, MD
Session summary
For the second year in a row, the session highlighting American Heart Association updates on Kawasaki disease did not disappoint and again attracted a large crowd of community and academic pediatric hospitalists. The newly-revised 2017 AHA Kawasaki Guidelines hot off the press by McCrindle et al. was reviewed in detail.
A secondary theory investigates the tropospheric wind patterns from central Asia and has indicated a possible link to outbreaks of KD in Chile. Despite previous investigation of carpet cleaning and risk for KD, no causal link has been identified.
Experts addressed pathophysiology, diagnosis, and management. Below are highlights from the new 2017 AHA Kawasaki Guideline Update in conjunction with points from the panel discussion.
Pathophysiology
• Cause is likely to be a common ubiquitous agent that in genetically inclined children will lead to a particular inflammatory response that manifests clinically as KD.
• A new theory about how the “ubiquitous agent” is spread by wind patterns.
Diagnosis
• Confirmed that infants younger than 1 year of age are more difficult to diagnose because they don’t present classically so it must be on the differential.
• The new algorithm makes it clearer that infants with fever for 7 days without symptoms should get lab screening tests for KD.
• Those who have classic symptoms and lab abnormalities consistent with KD but in whom fever is still at 3-4 days may be diagnosed with KD prior to the “5 days of fever rule” because these tend to be a sicker cohort of patients with higher rate of complications. Pretest probability and suspicion for KD must be high to treat before 5 days.
• Importance of the Z-score when evaluating an echocardiogram completed on a patient with suspected KD was stressed with a score greater than or equal to 2.5 reaching a level of significance for the patient’s body size.
Management
• It is still agreed that IVIG is first line therapy.
• For refractory KD (not responsive within 36 hours of first dose IVIG), management is more controversial. Experts on the panel agreed that they would likely provide a second dose of IVIG before thinking about steroids.
• Moderate dose aspirin is just as effective as high dose aspirin in the acute phase of KD.
• For more detailed information regarding the role of corticosteroids in KD, refer to Dr. Carl Galloway’s article in The Hospitalist July 2017 issue.
• A certain subset of patients may benefit from steroids if given early in the disease course, including those who present in shock syndrome. Steroids would still be in conjunction with IVIG treatment.
• Even though the new guidelines recommend a longer course of steroids for those refractory cases of KD in high-risk patients, panel experts are still unsure about evidence behind the claim.
The RAISE study was referenced and indicates there are significantly different outcomes for patients with severe disease placed on steroid therapy in combination with IVIG. In this group of patients, the incidence of coronary artery aneurysms was 23% in the IVIG-only group compared to 3% in the IVIG + steroid group (P less than .0001). This study and a recent Cochrane review that supported use of steroids in KD were completed in a homogeneous population of Japanese children and may not be generalizable to children in the United States.
Hyponatremia has been used as a diagnostic criterion for severe KD in Japanese children and was referenced as an indicator for addition of steroid therapy. Also, studies investigating the necessity of ASA at 80-100 mg/kg/d, a common practice for patients with KD treated in the United States, were compared to medium-dose ASA (30-50 mg/kg/d). There was no clinically significant difference in patient outcome or development of aneurysm formation between these two dosing regimens.
Key takeaways for Pediatric HM
• Diagnosis of classic KD remains unchanged and includes 5 or more days of fever and at least four clinical features (extremity changes, rash, conjunctivitis, oral changes, and cervical lymphadenopathy).
• Infants with fever of 7 days or more without other explanation should be evaluated for KD.
• Echocardiographic findings should be adjusted for body surface area and are significant if Z-score greater than or equal to 2.5.
• Moderate- to high-dose ASA is appropriate as an adjunct to IVIG until the patient is afebrile.
• Steroid therapy (for a total of 14 days) should be considered for high-risk patients.
Dr. King is associate program director, University of Minnesota Pediatric Residency Program. Dr. Hopkins is assistant professor of pediatrics, Johns Hopkins All Children’s Hospital.
At PHM 2017