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Obstetric perineal trauma prevented with SAFE PASSAGES
LAS VEGAS – Severe obstetric perineal trauma can often be avoided, even in operative deliveries, with the use of a suite of evidence-based interventions, according to findings from two prospective studies.
Collectively, these interventions resulted in significant reductions in third- and fourth-degree perineal lacerations in both military and civilian settings, according to research presented at the annual Pregnancy Meeting sponsored by the Society for Maternal-Fetal Medicine.
Developed by the Military Health System, the SAFE PASSAGES protocol brings together interventions that help achieve a controlled delivery over a relaxed perineum, minimizing risk for maternal obstetric trauma.
The entire SAFE PASSAGES curriculum is available free online.
Military results
In a prospective cohort design, 272,161 deliveries conducted before the 2011 implementation of the SAFE PASSAGES training program were compared with 451,446 postimplementation deliveries. Primary outcome measures were the incidence of third- and fourth-degree perineal lacerations during vaginal deliveries with and without instrumentation.
For vaginal deliveries with instrumentation within one service branch of the military medical system (Service X), implementation of SAFE PASSAGES training was associated with a 63.6% reduction in third- and fourth-degree perineal lacerations, compared with preintervention rates (P less than .001).
The other two services – Service Y and Service Z – received just administrative encouragement and saw a 15.5% reduction and a 12.6% increase in significant obstetric trauma when instrumentation-assisted vaginal deliveries were performed (P = .04 and .30, respectively), according to Merlin Fausett, MD, an ob.gyn. currently in private practice in Missoula, Mont., who led the SAFE PASSAGES efforts before retiring from the Air Force.
For vaginal deliveries performed without instrumentation, the rates also fell for Service X, which saw a 41.8% reduction in third- and fourth-degree perineal lacerations (P less than .002). The other services saw a 16% increase and a 12% decrease with administrative encouragement alone (P = .48 and .08, respectively).
Though the military training program had initially been conducted in person, Dr. Fausett said that the program was switched to web-based simulations because of budget constraints. Efficacy remained high, he said.
Civilian results
When the team-based simulation that formed the core of the military SAFE PASSAGES training was rolled out in a large civilian health care system, similar improvements were seen.
Over an 18-month period, 675 nurses, midwives, and physicians received simulation-based training in the SAFE PASSAGES techniques. Overall, severe perineal laceration rates in the civilian facilities were down by 38.53% after adoption of SAFE PASSAGES.
“We have really achieved a culture shift,” said Emily Marko, MD, an ob.gyn. and clerkship director for the Virginia Commonwealth University School of Medicine Inova Campus in Falls Church, Va. “This really requires the whole delivery team to get involved: the patient, the patient’s support person, the nurses, any midwives or doulas that are there,” she said. “To tell you the truth, this whole program is about paying attention to the perineum, and not rushing delivery.”
Posttraining surveys showed that 95% of providers had changed their practice patterns after training in the delivery strategies.
Interventions
The emphasis in SAFE PASSAGES is to achieve a slow, controlled delivery and to minimize strain on the perineum by means of conditioning, relaxation, and positioning.
The first intervention is to have pregnant women “start” perineal massage at 36 weeks. Next, providers are urged to “alleviate” maternal fears. Providers are also encouraged to recognize posterior presentations, and to “facilitate” an anterior presentation through rotation. The “E” in SAFE stands for “eliminating” midline episiotomies – one of the more difficult practices to shift, according to both Dr. Fausett and Dr. Marko.
Despite a wealth of evidence showing fewer anal sphincter disruptions and better overall outcomes, it’s been difficult to convince U.S. physicians to adopt the mediolateral episiotomy technique that’s widely adopted in Europe, they said.
The protocol calls for “placing” a warm compress over the perineum during labor to encourage relaxation and stretching. Though prenatal perineal massage is encouraged, Dr. Marko said that intrapartum massage is not, as it’s thought to contribute to edema when performed during labor.
During delivery, leg positioning to reduce stretching of the perineum is also important: The thighs should be “adducted” to 90 degrees or less, and “straightened” to 90 degrees or less as well. Though this can make “a bit of a tight space” for the delivering physician, Dr. Marko said, it really “helps engage the pelvic muscles to support the perineum,” and a few technique adjustments make the position workable.
The perineum should be “supported” during delivery by one hand of the delivering practitioner forming a U shape with the thumb and forefinger, with the first webspace overlying the posterior fourchette. Reinforcing the importance of avoiding a midline episiotomy, the “A” of passages stands for “aiming” lateral when an episiotomy is needed.
During the delivery, the physician should “go” slow, controlling the head and delivering after, rather than during, a contraction.
It’s important to be comfortable with forceps deliveries and vacuum extractions in order to minimize both maternal and fetal trauma; thus, physicians should “excel” at operative delivery, according to the protocol.
The SAFE PASSAGES website includes comprehensive explanations, with graphics and demonstrations using a model, of both forceps and vacuum delivery techniques.
Finally, should a laceration occur, the SAFE PASSAGES website provides detailed explanations of repair techniques, with an emphasis on understanding perineal, vaginal, and anal anatomy so “superb” approximation and repair can be achieved.
Though obstetric trauma may not be life threatening, it’s still associated with significant and persistent morbidity. When perineal and pelvic floor trauma disrupts the anal sphincter, anal incontinence can occur, even after a meticulous attempt at repair. Perineal and pelvic floor trauma can result in a host of urinary and sexual problems as well.
After the intensive training period, Dr. Fausett said, “laceration rates can creep back up if people forget about it and stop paying attention to it. But where it becomes a culture, the rates can stay low. Standardized training can reduce perineal trauma rates without increasing cesarean or neonatal trauma rates,” Dr. Fausett said.
Dr. Marko and Dr. Fausett reported having no conflicts of interest.
koakes@frontlinemedcom.com
On Twitter @karioakes
LAS VEGAS – Severe obstetric perineal trauma can often be avoided, even in operative deliveries, with the use of a suite of evidence-based interventions, according to findings from two prospective studies.
Collectively, these interventions resulted in significant reductions in third- and fourth-degree perineal lacerations in both military and civilian settings, according to research presented at the annual Pregnancy Meeting sponsored by the Society for Maternal-Fetal Medicine.
Developed by the Military Health System, the SAFE PASSAGES protocol brings together interventions that help achieve a controlled delivery over a relaxed perineum, minimizing risk for maternal obstetric trauma.
The entire SAFE PASSAGES curriculum is available free online.
Military results
In a prospective cohort design, 272,161 deliveries conducted before the 2011 implementation of the SAFE PASSAGES training program were compared with 451,446 postimplementation deliveries. Primary outcome measures were the incidence of third- and fourth-degree perineal lacerations during vaginal deliveries with and without instrumentation.
For vaginal deliveries with instrumentation within one service branch of the military medical system (Service X), implementation of SAFE PASSAGES training was associated with a 63.6% reduction in third- and fourth-degree perineal lacerations, compared with preintervention rates (P less than .001).
The other two services – Service Y and Service Z – received just administrative encouragement and saw a 15.5% reduction and a 12.6% increase in significant obstetric trauma when instrumentation-assisted vaginal deliveries were performed (P = .04 and .30, respectively), according to Merlin Fausett, MD, an ob.gyn. currently in private practice in Missoula, Mont., who led the SAFE PASSAGES efforts before retiring from the Air Force.
For vaginal deliveries performed without instrumentation, the rates also fell for Service X, which saw a 41.8% reduction in third- and fourth-degree perineal lacerations (P less than .002). The other services saw a 16% increase and a 12% decrease with administrative encouragement alone (P = .48 and .08, respectively).
Though the military training program had initially been conducted in person, Dr. Fausett said that the program was switched to web-based simulations because of budget constraints. Efficacy remained high, he said.
Civilian results
When the team-based simulation that formed the core of the military SAFE PASSAGES training was rolled out in a large civilian health care system, similar improvements were seen.
Over an 18-month period, 675 nurses, midwives, and physicians received simulation-based training in the SAFE PASSAGES techniques. Overall, severe perineal laceration rates in the civilian facilities were down by 38.53% after adoption of SAFE PASSAGES.
“We have really achieved a culture shift,” said Emily Marko, MD, an ob.gyn. and clerkship director for the Virginia Commonwealth University School of Medicine Inova Campus in Falls Church, Va. “This really requires the whole delivery team to get involved: the patient, the patient’s support person, the nurses, any midwives or doulas that are there,” she said. “To tell you the truth, this whole program is about paying attention to the perineum, and not rushing delivery.”
Posttraining surveys showed that 95% of providers had changed their practice patterns after training in the delivery strategies.
Interventions
The emphasis in SAFE PASSAGES is to achieve a slow, controlled delivery and to minimize strain on the perineum by means of conditioning, relaxation, and positioning.
The first intervention is to have pregnant women “start” perineal massage at 36 weeks. Next, providers are urged to “alleviate” maternal fears. Providers are also encouraged to recognize posterior presentations, and to “facilitate” an anterior presentation through rotation. The “E” in SAFE stands for “eliminating” midline episiotomies – one of the more difficult practices to shift, according to both Dr. Fausett and Dr. Marko.
Despite a wealth of evidence showing fewer anal sphincter disruptions and better overall outcomes, it’s been difficult to convince U.S. physicians to adopt the mediolateral episiotomy technique that’s widely adopted in Europe, they said.
The protocol calls for “placing” a warm compress over the perineum during labor to encourage relaxation and stretching. Though prenatal perineal massage is encouraged, Dr. Marko said that intrapartum massage is not, as it’s thought to contribute to edema when performed during labor.
During delivery, leg positioning to reduce stretching of the perineum is also important: The thighs should be “adducted” to 90 degrees or less, and “straightened” to 90 degrees or less as well. Though this can make “a bit of a tight space” for the delivering physician, Dr. Marko said, it really “helps engage the pelvic muscles to support the perineum,” and a few technique adjustments make the position workable.
The perineum should be “supported” during delivery by one hand of the delivering practitioner forming a U shape with the thumb and forefinger, with the first webspace overlying the posterior fourchette. Reinforcing the importance of avoiding a midline episiotomy, the “A” of passages stands for “aiming” lateral when an episiotomy is needed.
During the delivery, the physician should “go” slow, controlling the head and delivering after, rather than during, a contraction.
It’s important to be comfortable with forceps deliveries and vacuum extractions in order to minimize both maternal and fetal trauma; thus, physicians should “excel” at operative delivery, according to the protocol.
The SAFE PASSAGES website includes comprehensive explanations, with graphics and demonstrations using a model, of both forceps and vacuum delivery techniques.
Finally, should a laceration occur, the SAFE PASSAGES website provides detailed explanations of repair techniques, with an emphasis on understanding perineal, vaginal, and anal anatomy so “superb” approximation and repair can be achieved.
Though obstetric trauma may not be life threatening, it’s still associated with significant and persistent morbidity. When perineal and pelvic floor trauma disrupts the anal sphincter, anal incontinence can occur, even after a meticulous attempt at repair. Perineal and pelvic floor trauma can result in a host of urinary and sexual problems as well.
After the intensive training period, Dr. Fausett said, “laceration rates can creep back up if people forget about it and stop paying attention to it. But where it becomes a culture, the rates can stay low. Standardized training can reduce perineal trauma rates without increasing cesarean or neonatal trauma rates,” Dr. Fausett said.
Dr. Marko and Dr. Fausett reported having no conflicts of interest.
koakes@frontlinemedcom.com
On Twitter @karioakes
LAS VEGAS – Severe obstetric perineal trauma can often be avoided, even in operative deliveries, with the use of a suite of evidence-based interventions, according to findings from two prospective studies.
Collectively, these interventions resulted in significant reductions in third- and fourth-degree perineal lacerations in both military and civilian settings, according to research presented at the annual Pregnancy Meeting sponsored by the Society for Maternal-Fetal Medicine.
Developed by the Military Health System, the SAFE PASSAGES protocol brings together interventions that help achieve a controlled delivery over a relaxed perineum, minimizing risk for maternal obstetric trauma.
The entire SAFE PASSAGES curriculum is available free online.
Military results
In a prospective cohort design, 272,161 deliveries conducted before the 2011 implementation of the SAFE PASSAGES training program were compared with 451,446 postimplementation deliveries. Primary outcome measures were the incidence of third- and fourth-degree perineal lacerations during vaginal deliveries with and without instrumentation.
For vaginal deliveries with instrumentation within one service branch of the military medical system (Service X), implementation of SAFE PASSAGES training was associated with a 63.6% reduction in third- and fourth-degree perineal lacerations, compared with preintervention rates (P less than .001).
The other two services – Service Y and Service Z – received just administrative encouragement and saw a 15.5% reduction and a 12.6% increase in significant obstetric trauma when instrumentation-assisted vaginal deliveries were performed (P = .04 and .30, respectively), according to Merlin Fausett, MD, an ob.gyn. currently in private practice in Missoula, Mont., who led the SAFE PASSAGES efforts before retiring from the Air Force.
For vaginal deliveries performed without instrumentation, the rates also fell for Service X, which saw a 41.8% reduction in third- and fourth-degree perineal lacerations (P less than .002). The other services saw a 16% increase and a 12% decrease with administrative encouragement alone (P = .48 and .08, respectively).
Though the military training program had initially been conducted in person, Dr. Fausett said that the program was switched to web-based simulations because of budget constraints. Efficacy remained high, he said.
Civilian results
When the team-based simulation that formed the core of the military SAFE PASSAGES training was rolled out in a large civilian health care system, similar improvements were seen.
Over an 18-month period, 675 nurses, midwives, and physicians received simulation-based training in the SAFE PASSAGES techniques. Overall, severe perineal laceration rates in the civilian facilities were down by 38.53% after adoption of SAFE PASSAGES.
“We have really achieved a culture shift,” said Emily Marko, MD, an ob.gyn. and clerkship director for the Virginia Commonwealth University School of Medicine Inova Campus in Falls Church, Va. “This really requires the whole delivery team to get involved: the patient, the patient’s support person, the nurses, any midwives or doulas that are there,” she said. “To tell you the truth, this whole program is about paying attention to the perineum, and not rushing delivery.”
Posttraining surveys showed that 95% of providers had changed their practice patterns after training in the delivery strategies.
Interventions
The emphasis in SAFE PASSAGES is to achieve a slow, controlled delivery and to minimize strain on the perineum by means of conditioning, relaxation, and positioning.
The first intervention is to have pregnant women “start” perineal massage at 36 weeks. Next, providers are urged to “alleviate” maternal fears. Providers are also encouraged to recognize posterior presentations, and to “facilitate” an anterior presentation through rotation. The “E” in SAFE stands for “eliminating” midline episiotomies – one of the more difficult practices to shift, according to both Dr. Fausett and Dr. Marko.
Despite a wealth of evidence showing fewer anal sphincter disruptions and better overall outcomes, it’s been difficult to convince U.S. physicians to adopt the mediolateral episiotomy technique that’s widely adopted in Europe, they said.
The protocol calls for “placing” a warm compress over the perineum during labor to encourage relaxation and stretching. Though prenatal perineal massage is encouraged, Dr. Marko said that intrapartum massage is not, as it’s thought to contribute to edema when performed during labor.
During delivery, leg positioning to reduce stretching of the perineum is also important: The thighs should be “adducted” to 90 degrees or less, and “straightened” to 90 degrees or less as well. Though this can make “a bit of a tight space” for the delivering physician, Dr. Marko said, it really “helps engage the pelvic muscles to support the perineum,” and a few technique adjustments make the position workable.
The perineum should be “supported” during delivery by one hand of the delivering practitioner forming a U shape with the thumb and forefinger, with the first webspace overlying the posterior fourchette. Reinforcing the importance of avoiding a midline episiotomy, the “A” of passages stands for “aiming” lateral when an episiotomy is needed.
During the delivery, the physician should “go” slow, controlling the head and delivering after, rather than during, a contraction.
It’s important to be comfortable with forceps deliveries and vacuum extractions in order to minimize both maternal and fetal trauma; thus, physicians should “excel” at operative delivery, according to the protocol.
The SAFE PASSAGES website includes comprehensive explanations, with graphics and demonstrations using a model, of both forceps and vacuum delivery techniques.
Finally, should a laceration occur, the SAFE PASSAGES website provides detailed explanations of repair techniques, with an emphasis on understanding perineal, vaginal, and anal anatomy so “superb” approximation and repair can be achieved.
Though obstetric trauma may not be life threatening, it’s still associated with significant and persistent morbidity. When perineal and pelvic floor trauma disrupts the anal sphincter, anal incontinence can occur, even after a meticulous attempt at repair. Perineal and pelvic floor trauma can result in a host of urinary and sexual problems as well.
After the intensive training period, Dr. Fausett said, “laceration rates can creep back up if people forget about it and stop paying attention to it. But where it becomes a culture, the rates can stay low. Standardized training can reduce perineal trauma rates without increasing cesarean or neonatal trauma rates,” Dr. Fausett said.
Dr. Marko and Dr. Fausett reported having no conflicts of interest.
koakes@frontlinemedcom.com
On Twitter @karioakes
AT THE PREGNANCY MEETING
Key clinical point:
Major finding: Third- and fourth-degree perineal lacerations dropped by as much as 64% after SAFE PASSAGES training.
Data source: Prospective cohort study of 723,607 military deliveries; prospective study of 675 providers involved in labor and delivery at four civilian hospitals in a large health care system.
Disclosures: Dr. Marko and Dr. Fausett reported having no conflicts of interest.
High levels of flu activity reported in 15 states
Fifteen states experienced high levels of flu activity for the week ending Jan. 28 as the 2016-2017 season moved past last season’s peak, according to the Centers for Disease Control and Prevention.
The proportion of outpatient visits for influenza-like illness (ILI) was 3.9% for the week ending Jan. 28, 2017, the CDC reported. That level is higher than the national baseline level of 2.2% and higher than the peak of 3.6% for the 2015-2016 season.
The week also brought reports of seven flu-related pediatric deaths, although five actually occurred during previous weeks. There have now been 15 flu-related pediatric deaths during the 2016-2017 season, the CDC reported.
Fifteen states experienced high levels of flu activity for the week ending Jan. 28 as the 2016-2017 season moved past last season’s peak, according to the Centers for Disease Control and Prevention.
The proportion of outpatient visits for influenza-like illness (ILI) was 3.9% for the week ending Jan. 28, 2017, the CDC reported. That level is higher than the national baseline level of 2.2% and higher than the peak of 3.6% for the 2015-2016 season.
The week also brought reports of seven flu-related pediatric deaths, although five actually occurred during previous weeks. There have now been 15 flu-related pediatric deaths during the 2016-2017 season, the CDC reported.
Fifteen states experienced high levels of flu activity for the week ending Jan. 28 as the 2016-2017 season moved past last season’s peak, according to the Centers for Disease Control and Prevention.
The proportion of outpatient visits for influenza-like illness (ILI) was 3.9% for the week ending Jan. 28, 2017, the CDC reported. That level is higher than the national baseline level of 2.2% and higher than the peak of 3.6% for the 2015-2016 season.
The week also brought reports of seven flu-related pediatric deaths, although five actually occurred during previous weeks. There have now been 15 flu-related pediatric deaths during the 2016-2017 season, the CDC reported.
VIDEO: Coffee break at the Hawaii Dermatology Seminar
WAILEA, HAWAII – A few attendees at the Hawaii Dermatology Seminar discussed some of the highlights of the presentations during a coffee break at the meeting.
New treatment protocols for psoriasis, details about skin disease in children, managing medication side effects, and promising data about biologics are discussed in this video, as are updates on the latest tips for treating atopic dermatitis.
The interviewees had no conflicts to disclose.
The meeting is provided by Global Academy for Medical Education/Skin Disease Education Foundation. SDEF and this news organization are owned by the same parent company.
The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel
WAILEA, HAWAII – A few attendees at the Hawaii Dermatology Seminar discussed some of the highlights of the presentations during a coffee break at the meeting.
New treatment protocols for psoriasis, details about skin disease in children, managing medication side effects, and promising data about biologics are discussed in this video, as are updates on the latest tips for treating atopic dermatitis.
The interviewees had no conflicts to disclose.
The meeting is provided by Global Academy for Medical Education/Skin Disease Education Foundation. SDEF and this news organization are owned by the same parent company.
The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel
WAILEA, HAWAII – A few attendees at the Hawaii Dermatology Seminar discussed some of the highlights of the presentations during a coffee break at the meeting.
New treatment protocols for psoriasis, details about skin disease in children, managing medication side effects, and promising data about biologics are discussed in this video, as are updates on the latest tips for treating atopic dermatitis.
The interviewees had no conflicts to disclose.
The meeting is provided by Global Academy for Medical Education/Skin Disease Education Foundation. SDEF and this news organization are owned by the same parent company.
The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel
AT SDEF HAWAII DERMATOLOGY SEMINAR
A Surgeon's Intuition: Listen Before You Operate (An interview with associate editor, Brian J. Cole, MD)
In this DocThoughts interview, The American Journal of Orthopedics' associate editor, Dr. Cole, delves into the mind of a surgeon and gives insight into the surgical decision making process for his athletes.
In this DocThoughts interview, The American Journal of Orthopedics' associate editor, Dr. Cole, delves into the mind of a surgeon and gives insight into the surgical decision making process for his athletes.
In this DocThoughts interview, The American Journal of Orthopedics' associate editor, Dr. Cole, delves into the mind of a surgeon and gives insight into the surgical decision making process for his athletes.
START again shows safety of hypofractionated doses in early breast cancer
AMSTERDAM – Analyses of patient-reported outcomes in the UK START A and B trials show that radiation doses greater than 2 Gy to the supraclavicular fossa or axilla are safe and are associated with acceptable near- and late-term toxicities, trial investigators reported.
The START trials compared standard adjuvant radiation doses and schedules with accelerated, hypofractionated doses in women with early breast cancer.
At the annual San Antonio Breast Cancer Symposium in 2012, lead investigator John Yarnold, MD, professor of clinical oncology at the Institute of Cancer Research in London, reported that a 40-Gy, 15-fraction regimen was associated with fewer adverse events over 10 years of follow-up compared with a 50-Gy, 25-fraction regimen that was at the time the standard in the United States and the United Kingdom.
Here at an annual congress sponsored by the European Cancer Organisation, Dr. Yarnold reported that among 479 patients in the two trials who had lymphatic radiation, there were no significant differences in arm or shoulder pain, swelling, motion problems, or shoulder stiffness between patients who received conventional 2-Gy fractions for a total of 50 Gy, or lower total doses of radiation divided into fractions larger than 2 Gy.
“A post hoc analysis of outcomes in 479 patients treated with radiotherapy fractions larger than 2 Gy to the supraclavicular fossa and/or the axilla raise no concerns in terms of safety or patient acceptability, and it was for this reason that this schedule has been the standard for locoregional radiotherapy in the UK for some years now,” he said.
START (Standardization of Breast Radiotherapy) A enrolled 2,236 patients from 35 UK centers with breast cancer stages T 1-3, nodal stage N0-1, and no distant metastases (M0) and randomly assigned them to 50 Gy in 25 fractions of 2 Gy each over 5 weeks, 39.0 Gy in 13 fractions of 3.0 Gy over 5 weeks, and 41.6 Gy in 13 fractions of 3.2 Gy over 5 weeks.
In START B, 2,215 women were randomly assigned to the 50 Gy or 40 Gy regimens.
As reported in 2012, over a median follow-up of 9.3 years in START A, the 41.6-Gy regimen was similar in its adverse events profile compared with the 50-Gy dose (hazard ratio [HR] 0.94, and the lower-dose regimen also was comparable in efficacy, as measured by the rate of locoregional tumor relapse.
There were numerically but not significantly more relapses in the 39-Gy dose, however.
In START B, the 40-Gy dose was associated with significantly fewer adverse events over a median of 9.9 years of follow-up (HR 0.77). Efficacy was similar, although there was a nonsignificant trend toward superiority for the hypofractionated regimen.
In the post hoc analysis, the investigators looked at adverse events in 479 total patients in START A and B, 365 of whom had completed patient-reported outcome measures on the European Organization for Research and Treatment of Cancer (EORTC) Breast Cancer–Specific Quality of Life Questionnaire (QLQ-BR23).
A total of 262 patients in START A, and 103 in START B completed the questionnaires at 0, 6, 12, 24, and 60 months. Of these patients. The majority in each arm had radiation to the supraclavicular fossa only, and a minority had axilla radiation alone or in addition to supraclavicular fossa radiation.
In START B, 5-year patient reported outcomes of severity of 3 or 4 on a 4-point scale were low in each treatment arm, with arm or hand swelling reported in 10% of patients on 50 Gy, and 6% of patients on 40 Gy, and shoulder stiffness reported in 12% on the 50-Gy dose, vs. 15% on the 40-Gy dose.
Among all 479 patients with clinical assessments at 10 years, there were also no significant differences in either arm edema or shoulder stiffness between patients who received 50 Gy and those who received other, hypofractionated in each of the two trials.
The findings lend support to the Danish Breast Cancer Cooperative Group’s SKAGEN Trial 1, which is evaluating moderately hypofractionated locoregional adjuvant radiation therapy of early breast cancer combined with a simultaneous integrated boost in patients with an indication for boost, Dr. Yarnold said.
The UK START trials were supported by Cancer Research UK, the Medical Research Council, and the National Cancer Research Institute. Dr. Yarnold reported having no conflicts of interest.
AMSTERDAM – Analyses of patient-reported outcomes in the UK START A and B trials show that radiation doses greater than 2 Gy to the supraclavicular fossa or axilla are safe and are associated with acceptable near- and late-term toxicities, trial investigators reported.
The START trials compared standard adjuvant radiation doses and schedules with accelerated, hypofractionated doses in women with early breast cancer.
At the annual San Antonio Breast Cancer Symposium in 2012, lead investigator John Yarnold, MD, professor of clinical oncology at the Institute of Cancer Research in London, reported that a 40-Gy, 15-fraction regimen was associated with fewer adverse events over 10 years of follow-up compared with a 50-Gy, 25-fraction regimen that was at the time the standard in the United States and the United Kingdom.
Here at an annual congress sponsored by the European Cancer Organisation, Dr. Yarnold reported that among 479 patients in the two trials who had lymphatic radiation, there were no significant differences in arm or shoulder pain, swelling, motion problems, or shoulder stiffness between patients who received conventional 2-Gy fractions for a total of 50 Gy, or lower total doses of radiation divided into fractions larger than 2 Gy.
“A post hoc analysis of outcomes in 479 patients treated with radiotherapy fractions larger than 2 Gy to the supraclavicular fossa and/or the axilla raise no concerns in terms of safety or patient acceptability, and it was for this reason that this schedule has been the standard for locoregional radiotherapy in the UK for some years now,” he said.
START (Standardization of Breast Radiotherapy) A enrolled 2,236 patients from 35 UK centers with breast cancer stages T 1-3, nodal stage N0-1, and no distant metastases (M0) and randomly assigned them to 50 Gy in 25 fractions of 2 Gy each over 5 weeks, 39.0 Gy in 13 fractions of 3.0 Gy over 5 weeks, and 41.6 Gy in 13 fractions of 3.2 Gy over 5 weeks.
In START B, 2,215 women were randomly assigned to the 50 Gy or 40 Gy regimens.
As reported in 2012, over a median follow-up of 9.3 years in START A, the 41.6-Gy regimen was similar in its adverse events profile compared with the 50-Gy dose (hazard ratio [HR] 0.94, and the lower-dose regimen also was comparable in efficacy, as measured by the rate of locoregional tumor relapse.
There were numerically but not significantly more relapses in the 39-Gy dose, however.
In START B, the 40-Gy dose was associated with significantly fewer adverse events over a median of 9.9 years of follow-up (HR 0.77). Efficacy was similar, although there was a nonsignificant trend toward superiority for the hypofractionated regimen.
In the post hoc analysis, the investigators looked at adverse events in 479 total patients in START A and B, 365 of whom had completed patient-reported outcome measures on the European Organization for Research and Treatment of Cancer (EORTC) Breast Cancer–Specific Quality of Life Questionnaire (QLQ-BR23).
A total of 262 patients in START A, and 103 in START B completed the questionnaires at 0, 6, 12, 24, and 60 months. Of these patients. The majority in each arm had radiation to the supraclavicular fossa only, and a minority had axilla radiation alone or in addition to supraclavicular fossa radiation.
In START B, 5-year patient reported outcomes of severity of 3 or 4 on a 4-point scale were low in each treatment arm, with arm or hand swelling reported in 10% of patients on 50 Gy, and 6% of patients on 40 Gy, and shoulder stiffness reported in 12% on the 50-Gy dose, vs. 15% on the 40-Gy dose.
Among all 479 patients with clinical assessments at 10 years, there were also no significant differences in either arm edema or shoulder stiffness between patients who received 50 Gy and those who received other, hypofractionated in each of the two trials.
The findings lend support to the Danish Breast Cancer Cooperative Group’s SKAGEN Trial 1, which is evaluating moderately hypofractionated locoregional adjuvant radiation therapy of early breast cancer combined with a simultaneous integrated boost in patients with an indication for boost, Dr. Yarnold said.
The UK START trials were supported by Cancer Research UK, the Medical Research Council, and the National Cancer Research Institute. Dr. Yarnold reported having no conflicts of interest.
AMSTERDAM – Analyses of patient-reported outcomes in the UK START A and B trials show that radiation doses greater than 2 Gy to the supraclavicular fossa or axilla are safe and are associated with acceptable near- and late-term toxicities, trial investigators reported.
The START trials compared standard adjuvant radiation doses and schedules with accelerated, hypofractionated doses in women with early breast cancer.
At the annual San Antonio Breast Cancer Symposium in 2012, lead investigator John Yarnold, MD, professor of clinical oncology at the Institute of Cancer Research in London, reported that a 40-Gy, 15-fraction regimen was associated with fewer adverse events over 10 years of follow-up compared with a 50-Gy, 25-fraction regimen that was at the time the standard in the United States and the United Kingdom.
Here at an annual congress sponsored by the European Cancer Organisation, Dr. Yarnold reported that among 479 patients in the two trials who had lymphatic radiation, there were no significant differences in arm or shoulder pain, swelling, motion problems, or shoulder stiffness between patients who received conventional 2-Gy fractions for a total of 50 Gy, or lower total doses of radiation divided into fractions larger than 2 Gy.
“A post hoc analysis of outcomes in 479 patients treated with radiotherapy fractions larger than 2 Gy to the supraclavicular fossa and/or the axilla raise no concerns in terms of safety or patient acceptability, and it was for this reason that this schedule has been the standard for locoregional radiotherapy in the UK for some years now,” he said.
START (Standardization of Breast Radiotherapy) A enrolled 2,236 patients from 35 UK centers with breast cancer stages T 1-3, nodal stage N0-1, and no distant metastases (M0) and randomly assigned them to 50 Gy in 25 fractions of 2 Gy each over 5 weeks, 39.0 Gy in 13 fractions of 3.0 Gy over 5 weeks, and 41.6 Gy in 13 fractions of 3.2 Gy over 5 weeks.
In START B, 2,215 women were randomly assigned to the 50 Gy or 40 Gy regimens.
As reported in 2012, over a median follow-up of 9.3 years in START A, the 41.6-Gy regimen was similar in its adverse events profile compared with the 50-Gy dose (hazard ratio [HR] 0.94, and the lower-dose regimen also was comparable in efficacy, as measured by the rate of locoregional tumor relapse.
There were numerically but not significantly more relapses in the 39-Gy dose, however.
In START B, the 40-Gy dose was associated with significantly fewer adverse events over a median of 9.9 years of follow-up (HR 0.77). Efficacy was similar, although there was a nonsignificant trend toward superiority for the hypofractionated regimen.
In the post hoc analysis, the investigators looked at adverse events in 479 total patients in START A and B, 365 of whom had completed patient-reported outcome measures on the European Organization for Research and Treatment of Cancer (EORTC) Breast Cancer–Specific Quality of Life Questionnaire (QLQ-BR23).
A total of 262 patients in START A, and 103 in START B completed the questionnaires at 0, 6, 12, 24, and 60 months. Of these patients. The majority in each arm had radiation to the supraclavicular fossa only, and a minority had axilla radiation alone or in addition to supraclavicular fossa radiation.
In START B, 5-year patient reported outcomes of severity of 3 or 4 on a 4-point scale were low in each treatment arm, with arm or hand swelling reported in 10% of patients on 50 Gy, and 6% of patients on 40 Gy, and shoulder stiffness reported in 12% on the 50-Gy dose, vs. 15% on the 40-Gy dose.
Among all 479 patients with clinical assessments at 10 years, there were also no significant differences in either arm edema or shoulder stiffness between patients who received 50 Gy and those who received other, hypofractionated in each of the two trials.
The findings lend support to the Danish Breast Cancer Cooperative Group’s SKAGEN Trial 1, which is evaluating moderately hypofractionated locoregional adjuvant radiation therapy of early breast cancer combined with a simultaneous integrated boost in patients with an indication for boost, Dr. Yarnold said.
The UK START trials were supported by Cancer Research UK, the Medical Research Council, and the National Cancer Research Institute. Dr. Yarnold reported having no conflicts of interest.
AT ECCO2017
Key clinical point: Additional follow-up supports continued safety of hypofractionated radiation in early breast cancer.
Major finding: There were no significant differences in either patient-reported outcomes at 5 years or clinical assessments at 10 years in patients treated at 40-Gy or 50-Gy doses.
Data source: Post hoc analysis of 479 patients treated in the UK START A and B trials.
Disclosures: The UK START trials were supported by Cancer Research UK, the Medical Research Council, and the National Cancer Research Institute. Dr. Yarnold reported having no conflicts of interest..
VIDEO: Distinctive features define pediatric psoriasis
WAILEA, HAWAII – Plaque type psoriasis continues to be the most common type of psoriasis in children, but there are other presentations that should be considered, said Wynnis Tom, MD, a pediatric dermatologist at the University of California, San Diego, and Rady Children’s Hospital, San Diego.
“We certainly see a form of what some people would call napkin dermatitis,” or “diaper psoriasis,” affecting the diaper area in young infants, Dr. Tom said in a video interview at the Hawaii Dermatology Seminar provided by Global Academy for Medical Education/Skin Disease Education Foundation.
So when a child has a more refractory diaper rash, “look around to see if there are other lesions in the surrounding area that might be more typical for psoriasis,” she noted.
“We also see a lot more guttate disease” in children with psoriasis, which is more likely to be related to infections and triggers, Dr. Tom said. The face and scalp are often affected in children, and it is important to attend to these areas early to help avoid social stigma, she added.
Dr. Tom disclosed financial relationships with companies including Celgene, Janssen, and Promius. SDEF and this news organization are owned by the same parent company.
The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel
WAILEA, HAWAII – Plaque type psoriasis continues to be the most common type of psoriasis in children, but there are other presentations that should be considered, said Wynnis Tom, MD, a pediatric dermatologist at the University of California, San Diego, and Rady Children’s Hospital, San Diego.
“We certainly see a form of what some people would call napkin dermatitis,” or “diaper psoriasis,” affecting the diaper area in young infants, Dr. Tom said in a video interview at the Hawaii Dermatology Seminar provided by Global Academy for Medical Education/Skin Disease Education Foundation.
So when a child has a more refractory diaper rash, “look around to see if there are other lesions in the surrounding area that might be more typical for psoriasis,” she noted.
“We also see a lot more guttate disease” in children with psoriasis, which is more likely to be related to infections and triggers, Dr. Tom said. The face and scalp are often affected in children, and it is important to attend to these areas early to help avoid social stigma, she added.
Dr. Tom disclosed financial relationships with companies including Celgene, Janssen, and Promius. SDEF and this news organization are owned by the same parent company.
The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel
WAILEA, HAWAII – Plaque type psoriasis continues to be the most common type of psoriasis in children, but there are other presentations that should be considered, said Wynnis Tom, MD, a pediatric dermatologist at the University of California, San Diego, and Rady Children’s Hospital, San Diego.
“We certainly see a form of what some people would call napkin dermatitis,” or “diaper psoriasis,” affecting the diaper area in young infants, Dr. Tom said in a video interview at the Hawaii Dermatology Seminar provided by Global Academy for Medical Education/Skin Disease Education Foundation.
So when a child has a more refractory diaper rash, “look around to see if there are other lesions in the surrounding area that might be more typical for psoriasis,” she noted.
“We also see a lot more guttate disease” in children with psoriasis, which is more likely to be related to infections and triggers, Dr. Tom said. The face and scalp are often affected in children, and it is important to attend to these areas early to help avoid social stigma, she added.
Dr. Tom disclosed financial relationships with companies including Celgene, Janssen, and Promius. SDEF and this news organization are owned by the same parent company.
The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel
AT SDEF HAWAII DERMATOLOGY SEMINAR
Progressive Papular Eruption on the Face and Groin
The Diagnosis: Xanthoma Disseminatum
Genital examination revealed approximately 1.5×3-cm soft, yellow-pink plaques extending from the bilateral inguinal folds to the proximal medial thighs (Figure 1). There was no mucosal, axillary, extensor extremity, or palmoplantar involvement. Histopathologic examination of a biopsy from a plaque on the left side of the lower abdomen revealed sheets of foamy histiocytes distributed throughout a fibrotic dermis. Both mononucleated and multinucleated histiocytes were present, including many Touton giant cells (Figure 2). A patchy infiltrate of lymphocytes and rare eosinophils also was noted. The histiocytes labeled with factor XIIIa but not with S-100. Laboratory tests were performed with the following pertinent findings: low-density lipoprotein, 150 mg/dL (reference range, <130 mg/dL); high-density lipoprotein, 30 mg/dL (reference range, >40 mg/dL). Total cholesterol and triglyceride levels were within reference range, and complete blood cell count and basic metabolic panel were normal.
Xanthoma disseminatum (XD)(also known as Montgomery syndrome) is a rare, nonfamilial, normolipemic non-Langerhans cell histiocytosis characterized by extensive lipid deposition in the skin, mucous membranes, and internal organs. The pathogenesis of XD is poorly understood, but it may represent a macrophage-mediated reactive process triggered by superantigens.1
Xanthoma disseminatum most commonly affects males aged 5 to 25 years.2 Clinically, it is characterized by red-brown to yellowish papules and plaques symmetrically distributed over the eyelids, trunk, face, and proximal extremities. There is a predilection for involvement of flexural and intertriginous surfaces and tendency for extension along Langer lines. Extracutaneous involvement can be a notable cause of morbidity and mortality, underscoring the importance of distinguishing XD from other clinically similar xanthomatoses. Mucous membrane involvement occurs in 40% to 60% of patients.3 The oropharynx, larynx, and corneal and conjunctival membranes are most commonly affected, resulting in dysphagia, dysphonia or dyspnea, and visual impairment, respectively. Symptoms of internal organ involvement can be manifold, including pain or limited mobility secondary to osteolytic bone lesions or muscle or synovial membrane involvement, as well as seizures, strabismus, and cerebellar ataxia due to central nervous system lesions.2-4 Approximately 40% of patients develop diabetes insipidus secondary to involvement of the pituitary meninges.3
The differential diagnosis of XD includes juvenile xanthogranuloma, papular xanthomas, eruptive xanthomas, generalized eruptive histiocytosis, progressive nodular histiocytosis, multicentric reticulohistiocytosis, eruptive syringomas, sarcoidosis, and Langerhans cell histiocytosis; the latter should be considered, especially when there is concomitant diabetes insipidus.5 Laboratory studies typically are unremarkable. Although the majority of patients are normolipemic, rates of hyperlipemia within this group are comparable to the general population, occasionally rendering it difficult for the clinician to distinguish XD from hyperlipemic xanthomatoses. As such, diagnosis and differentiation from other xanthomatous processes rests on clinicopathological correlation. Histopathology reveals dermal collections of histiocytes, some with foamy cytoplasm, that range in appearance from spindled to scalloped to Touton-like. Early histopathology demonstrates scalloped macrophages with few foamy cells; a mixture of foamy cells, scalloped cells, inflammatory cells, and Touton and foreign body giant cells is characteristic of late lesions. Immunohistochemistry stains positive for non-Langerhans cell surface markers CD68 and factor XIIIa. Electron microscopy demonstrates dense and myeloid bodies, cholesterol crystals, and lipid vacuoles.5
Three subtypes of XD have been described based on the distinct clinical courses that have been observed in patients: a common, persistent, cutaneous form; a self-limited form with spontaneous resolution; and a progressive subtype with internal organ involvement. No consistently efficacious therapies have been identified, but isolated case reports attest to the efficacy of various agents, including azathioprine, clofibrate, cyclophosphamide, glucocorticoids, chlorambucil, and combination or monotherapy with lipid-lowering agents.3,5,6 Surgical resection, cryotherapy, radiotherapy, and CO2 laser therapy may offer some temporary benefit but do not alter the typically relapsing course of the disease.7,8 Remission and long-term control of lesions was reported with use of 2-chlorodeoxyadenosine, a purine nucleoside analogue, for 5 of 8 patients in a case series.3
- Zelger B, Cerio R, Orchard G, et al. Histologic and immunohistochemical study comparing xanthoma disseminatum and histiocytosis X. Arch Dermatol. 1992;128:1207-1212.
- Mahajan V, Sharma A, Chauhan P, et al. Xanthoma disseminatum: a red herring xanthomatosis. Indian J Dermatol Venereol Leprol. 2013;79:253-254.
- Khezri F, Gibson LE, Tefferi A. Xanthoma disseminatum: effective therapy with 2-chlorodeoxyadenosine in a case series. Arch Dermatol. 2011;147:459-464.
- Weiss N, Keller C. Xanthoma disseminatum: a rare normolipemic xanthomatosis. Clin Investig. 1993;71:233-238.
- Park HY, Cho DH, Joe DH, et al. A case of xanthoma disseminatum with spontaneous resolution over 10 years: review of the literature on long-term follow-up [published online May 26, 2011]. Dermatology. 2011;222:236-243.
- Kim SM, Waters P, Vincent A, et al. Sjogren's syndrome myelopathy: spinal cord involvement in Sjogren's syndrome might be a manifestation of neuromyelitis optica. Mult Scler. 2009;15:1062-1068.
- Eisendle K, Linder D, Ratzinger G, et al. Inflammation and lipid accumulation in xanthoma disseminatum: therapeutic considerations. J Am Acad Dermatol. 2008;58(2 suppl):S47-S49.
- Kim JY, Jung HD, Choe YS, et al. A case of xanthoma disseminatum accentuating over the eyelids. Ann Dermatol. 2010;22:353-357.
The Diagnosis: Xanthoma Disseminatum
Genital examination revealed approximately 1.5×3-cm soft, yellow-pink plaques extending from the bilateral inguinal folds to the proximal medial thighs (Figure 1). There was no mucosal, axillary, extensor extremity, or palmoplantar involvement. Histopathologic examination of a biopsy from a plaque on the left side of the lower abdomen revealed sheets of foamy histiocytes distributed throughout a fibrotic dermis. Both mononucleated and multinucleated histiocytes were present, including many Touton giant cells (Figure 2). A patchy infiltrate of lymphocytes and rare eosinophils also was noted. The histiocytes labeled with factor XIIIa but not with S-100. Laboratory tests were performed with the following pertinent findings: low-density lipoprotein, 150 mg/dL (reference range, <130 mg/dL); high-density lipoprotein, 30 mg/dL (reference range, >40 mg/dL). Total cholesterol and triglyceride levels were within reference range, and complete blood cell count and basic metabolic panel were normal.
Xanthoma disseminatum (XD)(also known as Montgomery syndrome) is a rare, nonfamilial, normolipemic non-Langerhans cell histiocytosis characterized by extensive lipid deposition in the skin, mucous membranes, and internal organs. The pathogenesis of XD is poorly understood, but it may represent a macrophage-mediated reactive process triggered by superantigens.1
Xanthoma disseminatum most commonly affects males aged 5 to 25 years.2 Clinically, it is characterized by red-brown to yellowish papules and plaques symmetrically distributed over the eyelids, trunk, face, and proximal extremities. There is a predilection for involvement of flexural and intertriginous surfaces and tendency for extension along Langer lines. Extracutaneous involvement can be a notable cause of morbidity and mortality, underscoring the importance of distinguishing XD from other clinically similar xanthomatoses. Mucous membrane involvement occurs in 40% to 60% of patients.3 The oropharynx, larynx, and corneal and conjunctival membranes are most commonly affected, resulting in dysphagia, dysphonia or dyspnea, and visual impairment, respectively. Symptoms of internal organ involvement can be manifold, including pain or limited mobility secondary to osteolytic bone lesions or muscle or synovial membrane involvement, as well as seizures, strabismus, and cerebellar ataxia due to central nervous system lesions.2-4 Approximately 40% of patients develop diabetes insipidus secondary to involvement of the pituitary meninges.3
The differential diagnosis of XD includes juvenile xanthogranuloma, papular xanthomas, eruptive xanthomas, generalized eruptive histiocytosis, progressive nodular histiocytosis, multicentric reticulohistiocytosis, eruptive syringomas, sarcoidosis, and Langerhans cell histiocytosis; the latter should be considered, especially when there is concomitant diabetes insipidus.5 Laboratory studies typically are unremarkable. Although the majority of patients are normolipemic, rates of hyperlipemia within this group are comparable to the general population, occasionally rendering it difficult for the clinician to distinguish XD from hyperlipemic xanthomatoses. As such, diagnosis and differentiation from other xanthomatous processes rests on clinicopathological correlation. Histopathology reveals dermal collections of histiocytes, some with foamy cytoplasm, that range in appearance from spindled to scalloped to Touton-like. Early histopathology demonstrates scalloped macrophages with few foamy cells; a mixture of foamy cells, scalloped cells, inflammatory cells, and Touton and foreign body giant cells is characteristic of late lesions. Immunohistochemistry stains positive for non-Langerhans cell surface markers CD68 and factor XIIIa. Electron microscopy demonstrates dense and myeloid bodies, cholesterol crystals, and lipid vacuoles.5
Three subtypes of XD have been described based on the distinct clinical courses that have been observed in patients: a common, persistent, cutaneous form; a self-limited form with spontaneous resolution; and a progressive subtype with internal organ involvement. No consistently efficacious therapies have been identified, but isolated case reports attest to the efficacy of various agents, including azathioprine, clofibrate, cyclophosphamide, glucocorticoids, chlorambucil, and combination or monotherapy with lipid-lowering agents.3,5,6 Surgical resection, cryotherapy, radiotherapy, and CO2 laser therapy may offer some temporary benefit but do not alter the typically relapsing course of the disease.7,8 Remission and long-term control of lesions was reported with use of 2-chlorodeoxyadenosine, a purine nucleoside analogue, for 5 of 8 patients in a case series.3
The Diagnosis: Xanthoma Disseminatum
Genital examination revealed approximately 1.5×3-cm soft, yellow-pink plaques extending from the bilateral inguinal folds to the proximal medial thighs (Figure 1). There was no mucosal, axillary, extensor extremity, or palmoplantar involvement. Histopathologic examination of a biopsy from a plaque on the left side of the lower abdomen revealed sheets of foamy histiocytes distributed throughout a fibrotic dermis. Both mononucleated and multinucleated histiocytes were present, including many Touton giant cells (Figure 2). A patchy infiltrate of lymphocytes and rare eosinophils also was noted. The histiocytes labeled with factor XIIIa but not with S-100. Laboratory tests were performed with the following pertinent findings: low-density lipoprotein, 150 mg/dL (reference range, <130 mg/dL); high-density lipoprotein, 30 mg/dL (reference range, >40 mg/dL). Total cholesterol and triglyceride levels were within reference range, and complete blood cell count and basic metabolic panel were normal.
Xanthoma disseminatum (XD)(also known as Montgomery syndrome) is a rare, nonfamilial, normolipemic non-Langerhans cell histiocytosis characterized by extensive lipid deposition in the skin, mucous membranes, and internal organs. The pathogenesis of XD is poorly understood, but it may represent a macrophage-mediated reactive process triggered by superantigens.1
Xanthoma disseminatum most commonly affects males aged 5 to 25 years.2 Clinically, it is characterized by red-brown to yellowish papules and plaques symmetrically distributed over the eyelids, trunk, face, and proximal extremities. There is a predilection for involvement of flexural and intertriginous surfaces and tendency for extension along Langer lines. Extracutaneous involvement can be a notable cause of morbidity and mortality, underscoring the importance of distinguishing XD from other clinically similar xanthomatoses. Mucous membrane involvement occurs in 40% to 60% of patients.3 The oropharynx, larynx, and corneal and conjunctival membranes are most commonly affected, resulting in dysphagia, dysphonia or dyspnea, and visual impairment, respectively. Symptoms of internal organ involvement can be manifold, including pain or limited mobility secondary to osteolytic bone lesions or muscle or synovial membrane involvement, as well as seizures, strabismus, and cerebellar ataxia due to central nervous system lesions.2-4 Approximately 40% of patients develop diabetes insipidus secondary to involvement of the pituitary meninges.3
The differential diagnosis of XD includes juvenile xanthogranuloma, papular xanthomas, eruptive xanthomas, generalized eruptive histiocytosis, progressive nodular histiocytosis, multicentric reticulohistiocytosis, eruptive syringomas, sarcoidosis, and Langerhans cell histiocytosis; the latter should be considered, especially when there is concomitant diabetes insipidus.5 Laboratory studies typically are unremarkable. Although the majority of patients are normolipemic, rates of hyperlipemia within this group are comparable to the general population, occasionally rendering it difficult for the clinician to distinguish XD from hyperlipemic xanthomatoses. As such, diagnosis and differentiation from other xanthomatous processes rests on clinicopathological correlation. Histopathology reveals dermal collections of histiocytes, some with foamy cytoplasm, that range in appearance from spindled to scalloped to Touton-like. Early histopathology demonstrates scalloped macrophages with few foamy cells; a mixture of foamy cells, scalloped cells, inflammatory cells, and Touton and foreign body giant cells is characteristic of late lesions. Immunohistochemistry stains positive for non-Langerhans cell surface markers CD68 and factor XIIIa. Electron microscopy demonstrates dense and myeloid bodies, cholesterol crystals, and lipid vacuoles.5
Three subtypes of XD have been described based on the distinct clinical courses that have been observed in patients: a common, persistent, cutaneous form; a self-limited form with spontaneous resolution; and a progressive subtype with internal organ involvement. No consistently efficacious therapies have been identified, but isolated case reports attest to the efficacy of various agents, including azathioprine, clofibrate, cyclophosphamide, glucocorticoids, chlorambucil, and combination or monotherapy with lipid-lowering agents.3,5,6 Surgical resection, cryotherapy, radiotherapy, and CO2 laser therapy may offer some temporary benefit but do not alter the typically relapsing course of the disease.7,8 Remission and long-term control of lesions was reported with use of 2-chlorodeoxyadenosine, a purine nucleoside analogue, for 5 of 8 patients in a case series.3
- Zelger B, Cerio R, Orchard G, et al. Histologic and immunohistochemical study comparing xanthoma disseminatum and histiocytosis X. Arch Dermatol. 1992;128:1207-1212.
- Mahajan V, Sharma A, Chauhan P, et al. Xanthoma disseminatum: a red herring xanthomatosis. Indian J Dermatol Venereol Leprol. 2013;79:253-254.
- Khezri F, Gibson LE, Tefferi A. Xanthoma disseminatum: effective therapy with 2-chlorodeoxyadenosine in a case series. Arch Dermatol. 2011;147:459-464.
- Weiss N, Keller C. Xanthoma disseminatum: a rare normolipemic xanthomatosis. Clin Investig. 1993;71:233-238.
- Park HY, Cho DH, Joe DH, et al. A case of xanthoma disseminatum with spontaneous resolution over 10 years: review of the literature on long-term follow-up [published online May 26, 2011]. Dermatology. 2011;222:236-243.
- Kim SM, Waters P, Vincent A, et al. Sjogren's syndrome myelopathy: spinal cord involvement in Sjogren's syndrome might be a manifestation of neuromyelitis optica. Mult Scler. 2009;15:1062-1068.
- Eisendle K, Linder D, Ratzinger G, et al. Inflammation and lipid accumulation in xanthoma disseminatum: therapeutic considerations. J Am Acad Dermatol. 2008;58(2 suppl):S47-S49.
- Kim JY, Jung HD, Choe YS, et al. A case of xanthoma disseminatum accentuating over the eyelids. Ann Dermatol. 2010;22:353-357.
- Zelger B, Cerio R, Orchard G, et al. Histologic and immunohistochemical study comparing xanthoma disseminatum and histiocytosis X. Arch Dermatol. 1992;128:1207-1212.
- Mahajan V, Sharma A, Chauhan P, et al. Xanthoma disseminatum: a red herring xanthomatosis. Indian J Dermatol Venereol Leprol. 2013;79:253-254.
- Khezri F, Gibson LE, Tefferi A. Xanthoma disseminatum: effective therapy with 2-chlorodeoxyadenosine in a case series. Arch Dermatol. 2011;147:459-464.
- Weiss N, Keller C. Xanthoma disseminatum: a rare normolipemic xanthomatosis. Clin Investig. 1993;71:233-238.
- Park HY, Cho DH, Joe DH, et al. A case of xanthoma disseminatum with spontaneous resolution over 10 years: review of the literature on long-term follow-up [published online May 26, 2011]. Dermatology. 2011;222:236-243.
- Kim SM, Waters P, Vincent A, et al. Sjogren's syndrome myelopathy: spinal cord involvement in Sjogren's syndrome might be a manifestation of neuromyelitis optica. Mult Scler. 2009;15:1062-1068.
- Eisendle K, Linder D, Ratzinger G, et al. Inflammation and lipid accumulation in xanthoma disseminatum: therapeutic considerations. J Am Acad Dermatol. 2008;58(2 suppl):S47-S49.
- Kim JY, Jung HD, Choe YS, et al. A case of xanthoma disseminatum accentuating over the eyelids. Ann Dermatol. 2010;22:353-357.
A 28-year-old man presented for evaluation of numerous papules on the face and groin that first appeared in adolescence and had been increasing in size and number over the last several years. The lesions occasionally were pruritic. Review of systems was noncontributory. His medical history was notable for asthma, and there were no affected family members. Physical examination revealed numerous symmetrically distributed, soft, yellow-pink, 1- to 5-mm papules coalescing into plaques on the bilateral malar cheeks extending to the medial canthi and the maxillary, mandibular, zygomatic, and submental regions, as well as the bilateral external auditory meatus.
Hyperpigmented Papules and Plaques
The Diagnosis: Persistent Still Disease
At the time of presentation, the patient had not taken systemic medications for a year. Laboratory studies revealed leukocytosis with neutrophilia and a serum ferritin level of 5493 ng/mL (reference range, 15-200 ng/mL). Rheumatoid factor and antinuclear antibody serologies were within reference range. Microbiologic workup was negative. Lymph node and bone marrow biopsies were negative for a lymphoproliferative disorder. Skin biopsies were performed on the back and forearm. Histologic evaluation revealed orthokeratosis, slight acanthosis, and dyskeratosis confined to the upper layers of the epidermis without evidence of interface dermatitis. There was a mixed perivascular infiltrate composed of lymphocytes and neutrophils with no attendant vasculitic change (Figure).
The patient was discharged on prednisone and seen for outpatient follow-up weeks later. Six weeks later, the cutaneous eruption remained unchanged. The patient was unable to start other systemic medications due to lack of insurance and ineligibility for the local patient-assistance program; he was subsequently lost to follow-up.
Adult-onset Still disease is a rare, systemic, inflammatory condition with a broad spectrum of clinical presentations.1-3 Still disease affects all age groups, and children with Still disease (<16 years) usually have a concurrent diagnosis of juvenile idiopathic arthritis (formerly known as juvenile rheumatoid arthritis).1,2,4 Still disease preferentially affects adolescents and adults aged 16 to 35 years, with more than 75% of new cases occurring in this age range.1 Worldwide, the incidence and prevalence of Still disease is disputed with no conclusive rates established.1,3
Still disease is characterized by 4 cardinal signs: high spiking fevers (temperature, ≥39°C); leukocytosis with a predominance of neutrophils (≥10,000 cells/mm3 with ≥80% neutrophils); arthralgia or arthritis; and an evanescent, nonpruritic, salmon-colored morbilliform eruption of the skin, typically on the trunk or extremities.2 Histologic evaluation of the classic Still disease eruption displays perivascular inflammation of the superficial dermis with infiltration by lymphocytes and histiocytes.3
In 1992, major and minor diagnostic criteria were established for adult-onset Still disease. For diagnosis, patients must meet 5 criteria, including 2 major criteria.5 Major criteria include arthralgia or arthritis present for more than 2 weeks, fever (temperature, >39°C) for at least 1 week, the classic Still disease morbilliform eruption (ie, salmon colored, evanescent, morbilliform), and leukocytosis with more than 80% neutrophils. Minor criteria include sore throat, lymphadenopathy and/or splenomegaly, negative rheumatoid factor and antinuclear antibody serologies, and abnormal liver function (defined as elevated transaminases).5 Although not included in the diagnostic criteria, there have been reports of elevated serum ferritin levels in patients with Still disease, a finding that potentially is useful in distinguishing between active and inactive rheumatic conditions.6,7
Several case reports have described persistent Still disease, a subtype of Still disease in which patients present with brown-red, persistent, pruritic macules, papules, and plaques that are widespread and oddly shaped.8,9 Histologically, this subtype is characterized by necrotic keratinocytes in the epidermis and dermal perivascular inflammation composed of neutrophils and lymphocytes.10 This histology differs from classic Still disease in that the latter typically does not have superficial epidermal dyskeratosis. Our case is consistent with reports of persistent Still disease.
Although the etiology of Still disease remains to be elucidated, HLA-B17, -B18, -B35, and -DR2 have been associated with the disease.3 Furthermore, helper T cell TH1, IL-2, IFN-γ, and tumor necrosis factor α have been implicated in disease pathology, enabling the use of newer targeted pharmacologic therapies. Canakinumab, an IL-1β inhibitor, has been found to improve arthritis, fever, and rash in patients with Still disease.11 These findings are particularly encouraging for patients who have not experienced improvement with traditional antirheumatic drugs, such as our patient who was not steroid responsive.3
Although a salmon-colored, evanescent, morbilliform eruption in the context of other systemic signs and symptoms readily evokes consideration of Still disease, the less common fixed cutaneous eruption seen in our case may evade accurate diagnosis. Our case aims to increase awareness of this unusual and rare subtype of the cutaneous eruption of Still disease, as a timely diagnosis may prevent potentially life-threatening sequelae including cardiopulmonary disease and respiratory failure.3,5,9
- Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still's disease [published online October 11, 2005]. Ann Rheum Dis. 2006;65:564-572.
- Fautrel B. Adult-onset Still disease. Best Pract Res Clin Rheumatol. 2008;22:773-792.
- Bagnari V, Colina M, Ciancio G, et al. Adult-onset Still's disease. Rheumatol Int. 2010;30:855-862.
- Ravelli A, Martini A. Juvenile idiopathic arthritis. Lancet. 2007;369:767-778.
- Yamaguchi M, Ohta A, Tsunematsu, T, et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol. 1992;19:424-430.
- Van Reeth C, Le Moel G, Lasne Y, et al. Serum ferritin and isoferritins are tools for diagnosis of active adult Still's disease. J Rheumatol. 1994;21:890-895.
- Novak S, Anic F, Luke-Vrbanic TS. Extremely high serum ferritin levels as a main diagnostic tool of adult-onset Still's disease. Rheumatol Int. 2012;32:1091-1094.
- Fortna RR, Gudjonsson JE, Seidel G, et al. Persistent pruritic papules and plaques: a characteristic histopathologic presentation seen in a subset of patients with adult-onset and juvenile Still's disease. J Cutan Pathol. 2010;37:932-937.
- Yang CC, Lee JY, Liu MF, et al. Adult-onset Still's disease with persistent skin eruption and fatal respiratory failure in a Taiwanese woman. Eur J Dermatol. 2006;16:593-594.
- Lee JY, Yang CC, Hsu MM. Histopathology of persistent papules and plaques in adult-onset Still's disease. J Am Acad Dermatol. 2005;52:1003-1008.
- Kontzias A, Efthimiou P. The use of canakinumab, a novel IL-1β long-acting inhibitor in refractory adult-onset Still's disease. Sem Arthritis Rheum. 2012;42:201-205.
The Diagnosis: Persistent Still Disease
At the time of presentation, the patient had not taken systemic medications for a year. Laboratory studies revealed leukocytosis with neutrophilia and a serum ferritin level of 5493 ng/mL (reference range, 15-200 ng/mL). Rheumatoid factor and antinuclear antibody serologies were within reference range. Microbiologic workup was negative. Lymph node and bone marrow biopsies were negative for a lymphoproliferative disorder. Skin biopsies were performed on the back and forearm. Histologic evaluation revealed orthokeratosis, slight acanthosis, and dyskeratosis confined to the upper layers of the epidermis without evidence of interface dermatitis. There was a mixed perivascular infiltrate composed of lymphocytes and neutrophils with no attendant vasculitic change (Figure).
The patient was discharged on prednisone and seen for outpatient follow-up weeks later. Six weeks later, the cutaneous eruption remained unchanged. The patient was unable to start other systemic medications due to lack of insurance and ineligibility for the local patient-assistance program; he was subsequently lost to follow-up.
Adult-onset Still disease is a rare, systemic, inflammatory condition with a broad spectrum of clinical presentations.1-3 Still disease affects all age groups, and children with Still disease (<16 years) usually have a concurrent diagnosis of juvenile idiopathic arthritis (formerly known as juvenile rheumatoid arthritis).1,2,4 Still disease preferentially affects adolescents and adults aged 16 to 35 years, with more than 75% of new cases occurring in this age range.1 Worldwide, the incidence and prevalence of Still disease is disputed with no conclusive rates established.1,3
Still disease is characterized by 4 cardinal signs: high spiking fevers (temperature, ≥39°C); leukocytosis with a predominance of neutrophils (≥10,000 cells/mm3 with ≥80% neutrophils); arthralgia or arthritis; and an evanescent, nonpruritic, salmon-colored morbilliform eruption of the skin, typically on the trunk or extremities.2 Histologic evaluation of the classic Still disease eruption displays perivascular inflammation of the superficial dermis with infiltration by lymphocytes and histiocytes.3
In 1992, major and minor diagnostic criteria were established for adult-onset Still disease. For diagnosis, patients must meet 5 criteria, including 2 major criteria.5 Major criteria include arthralgia or arthritis present for more than 2 weeks, fever (temperature, >39°C) for at least 1 week, the classic Still disease morbilliform eruption (ie, salmon colored, evanescent, morbilliform), and leukocytosis with more than 80% neutrophils. Minor criteria include sore throat, lymphadenopathy and/or splenomegaly, negative rheumatoid factor and antinuclear antibody serologies, and abnormal liver function (defined as elevated transaminases).5 Although not included in the diagnostic criteria, there have been reports of elevated serum ferritin levels in patients with Still disease, a finding that potentially is useful in distinguishing between active and inactive rheumatic conditions.6,7
Several case reports have described persistent Still disease, a subtype of Still disease in which patients present with brown-red, persistent, pruritic macules, papules, and plaques that are widespread and oddly shaped.8,9 Histologically, this subtype is characterized by necrotic keratinocytes in the epidermis and dermal perivascular inflammation composed of neutrophils and lymphocytes.10 This histology differs from classic Still disease in that the latter typically does not have superficial epidermal dyskeratosis. Our case is consistent with reports of persistent Still disease.
Although the etiology of Still disease remains to be elucidated, HLA-B17, -B18, -B35, and -DR2 have been associated with the disease.3 Furthermore, helper T cell TH1, IL-2, IFN-γ, and tumor necrosis factor α have been implicated in disease pathology, enabling the use of newer targeted pharmacologic therapies. Canakinumab, an IL-1β inhibitor, has been found to improve arthritis, fever, and rash in patients with Still disease.11 These findings are particularly encouraging for patients who have not experienced improvement with traditional antirheumatic drugs, such as our patient who was not steroid responsive.3
Although a salmon-colored, evanescent, morbilliform eruption in the context of other systemic signs and symptoms readily evokes consideration of Still disease, the less common fixed cutaneous eruption seen in our case may evade accurate diagnosis. Our case aims to increase awareness of this unusual and rare subtype of the cutaneous eruption of Still disease, as a timely diagnosis may prevent potentially life-threatening sequelae including cardiopulmonary disease and respiratory failure.3,5,9
The Diagnosis: Persistent Still Disease
At the time of presentation, the patient had not taken systemic medications for a year. Laboratory studies revealed leukocytosis with neutrophilia and a serum ferritin level of 5493 ng/mL (reference range, 15-200 ng/mL). Rheumatoid factor and antinuclear antibody serologies were within reference range. Microbiologic workup was negative. Lymph node and bone marrow biopsies were negative for a lymphoproliferative disorder. Skin biopsies were performed on the back and forearm. Histologic evaluation revealed orthokeratosis, slight acanthosis, and dyskeratosis confined to the upper layers of the epidermis without evidence of interface dermatitis. There was a mixed perivascular infiltrate composed of lymphocytes and neutrophils with no attendant vasculitic change (Figure).
The patient was discharged on prednisone and seen for outpatient follow-up weeks later. Six weeks later, the cutaneous eruption remained unchanged. The patient was unable to start other systemic medications due to lack of insurance and ineligibility for the local patient-assistance program; he was subsequently lost to follow-up.
Adult-onset Still disease is a rare, systemic, inflammatory condition with a broad spectrum of clinical presentations.1-3 Still disease affects all age groups, and children with Still disease (<16 years) usually have a concurrent diagnosis of juvenile idiopathic arthritis (formerly known as juvenile rheumatoid arthritis).1,2,4 Still disease preferentially affects adolescents and adults aged 16 to 35 years, with more than 75% of new cases occurring in this age range.1 Worldwide, the incidence and prevalence of Still disease is disputed with no conclusive rates established.1,3
Still disease is characterized by 4 cardinal signs: high spiking fevers (temperature, ≥39°C); leukocytosis with a predominance of neutrophils (≥10,000 cells/mm3 with ≥80% neutrophils); arthralgia or arthritis; and an evanescent, nonpruritic, salmon-colored morbilliform eruption of the skin, typically on the trunk or extremities.2 Histologic evaluation of the classic Still disease eruption displays perivascular inflammation of the superficial dermis with infiltration by lymphocytes and histiocytes.3
In 1992, major and minor diagnostic criteria were established for adult-onset Still disease. For diagnosis, patients must meet 5 criteria, including 2 major criteria.5 Major criteria include arthralgia or arthritis present for more than 2 weeks, fever (temperature, >39°C) for at least 1 week, the classic Still disease morbilliform eruption (ie, salmon colored, evanescent, morbilliform), and leukocytosis with more than 80% neutrophils. Minor criteria include sore throat, lymphadenopathy and/or splenomegaly, negative rheumatoid factor and antinuclear antibody serologies, and abnormal liver function (defined as elevated transaminases).5 Although not included in the diagnostic criteria, there have been reports of elevated serum ferritin levels in patients with Still disease, a finding that potentially is useful in distinguishing between active and inactive rheumatic conditions.6,7
Several case reports have described persistent Still disease, a subtype of Still disease in which patients present with brown-red, persistent, pruritic macules, papules, and plaques that are widespread and oddly shaped.8,9 Histologically, this subtype is characterized by necrotic keratinocytes in the epidermis and dermal perivascular inflammation composed of neutrophils and lymphocytes.10 This histology differs from classic Still disease in that the latter typically does not have superficial epidermal dyskeratosis. Our case is consistent with reports of persistent Still disease.
Although the etiology of Still disease remains to be elucidated, HLA-B17, -B18, -B35, and -DR2 have been associated with the disease.3 Furthermore, helper T cell TH1, IL-2, IFN-γ, and tumor necrosis factor α have been implicated in disease pathology, enabling the use of newer targeted pharmacologic therapies. Canakinumab, an IL-1β inhibitor, has been found to improve arthritis, fever, and rash in patients with Still disease.11 These findings are particularly encouraging for patients who have not experienced improvement with traditional antirheumatic drugs, such as our patient who was not steroid responsive.3
Although a salmon-colored, evanescent, morbilliform eruption in the context of other systemic signs and symptoms readily evokes consideration of Still disease, the less common fixed cutaneous eruption seen in our case may evade accurate diagnosis. Our case aims to increase awareness of this unusual and rare subtype of the cutaneous eruption of Still disease, as a timely diagnosis may prevent potentially life-threatening sequelae including cardiopulmonary disease and respiratory failure.3,5,9
- Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still's disease [published online October 11, 2005]. Ann Rheum Dis. 2006;65:564-572.
- Fautrel B. Adult-onset Still disease. Best Pract Res Clin Rheumatol. 2008;22:773-792.
- Bagnari V, Colina M, Ciancio G, et al. Adult-onset Still's disease. Rheumatol Int. 2010;30:855-862.
- Ravelli A, Martini A. Juvenile idiopathic arthritis. Lancet. 2007;369:767-778.
- Yamaguchi M, Ohta A, Tsunematsu, T, et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol. 1992;19:424-430.
- Van Reeth C, Le Moel G, Lasne Y, et al. Serum ferritin and isoferritins are tools for diagnosis of active adult Still's disease. J Rheumatol. 1994;21:890-895.
- Novak S, Anic F, Luke-Vrbanic TS. Extremely high serum ferritin levels as a main diagnostic tool of adult-onset Still's disease. Rheumatol Int. 2012;32:1091-1094.
- Fortna RR, Gudjonsson JE, Seidel G, et al. Persistent pruritic papules and plaques: a characteristic histopathologic presentation seen in a subset of patients with adult-onset and juvenile Still's disease. J Cutan Pathol. 2010;37:932-937.
- Yang CC, Lee JY, Liu MF, et al. Adult-onset Still's disease with persistent skin eruption and fatal respiratory failure in a Taiwanese woman. Eur J Dermatol. 2006;16:593-594.
- Lee JY, Yang CC, Hsu MM. Histopathology of persistent papules and plaques in adult-onset Still's disease. J Am Acad Dermatol. 2005;52:1003-1008.
- Kontzias A, Efthimiou P. The use of canakinumab, a novel IL-1β long-acting inhibitor in refractory adult-onset Still's disease. Sem Arthritis Rheum. 2012;42:201-205.
- Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still's disease [published online October 11, 2005]. Ann Rheum Dis. 2006;65:564-572.
- Fautrel B. Adult-onset Still disease. Best Pract Res Clin Rheumatol. 2008;22:773-792.
- Bagnari V, Colina M, Ciancio G, et al. Adult-onset Still's disease. Rheumatol Int. 2010;30:855-862.
- Ravelli A, Martini A. Juvenile idiopathic arthritis. Lancet. 2007;369:767-778.
- Yamaguchi M, Ohta A, Tsunematsu, T, et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol. 1992;19:424-430.
- Van Reeth C, Le Moel G, Lasne Y, et al. Serum ferritin and isoferritins are tools for diagnosis of active adult Still's disease. J Rheumatol. 1994;21:890-895.
- Novak S, Anic F, Luke-Vrbanic TS. Extremely high serum ferritin levels as a main diagnostic tool of adult-onset Still's disease. Rheumatol Int. 2012;32:1091-1094.
- Fortna RR, Gudjonsson JE, Seidel G, et al. Persistent pruritic papules and plaques: a characteristic histopathologic presentation seen in a subset of patients with adult-onset and juvenile Still's disease. J Cutan Pathol. 2010;37:932-937.
- Yang CC, Lee JY, Liu MF, et al. Adult-onset Still's disease with persistent skin eruption and fatal respiratory failure in a Taiwanese woman. Eur J Dermatol. 2006;16:593-594.
- Lee JY, Yang CC, Hsu MM. Histopathology of persistent papules and plaques in adult-onset Still's disease. J Am Acad Dermatol. 2005;52:1003-1008.
- Kontzias A, Efthimiou P. The use of canakinumab, a novel IL-1β long-acting inhibitor in refractory adult-onset Still's disease. Sem Arthritis Rheum. 2012;42:201-205.
A 25-year-old Hispanic man with a history of juvenile idiopathic arthritis was admitted with a high-grade fever (temperature, >38.9°C) and diffuse nonlocalized abdominal pain of 2 days' duration. Physical examination revealed tachycardia, axillary lymphadenopathy, and hepatosplenomegaly. Cutaneous findings consisted of striking hyperpigmented patches on the chest and back, and hyperpigmented scaly lichenoid papules and plaques on the upper and lower extremities. The plaques on the lower extremities exhibited koebnerization. The patient reported that the eruption initially presented at 16 years of age as pruritic papules on the legs, which gradually spread to involve the arms, chest, and back. Prior treatments of juvenile idiopathic arthritis included prednisone, methotrexate, infliximab, and etanercept, though they were intermittent and temporary. Over time, the cutaneous eruption evolved into its current morphology and distribution, with periods of clearance observed while receiving systemic medications.
Fewer infant deaths during ‘39-week rule’ era
LAS VEGAS – Closer adherence by U.S. physicians to the “39-week rule” for elective deliveries appears to have cut net neonatal mortality in an analysis of more than 14 million deliveries during 2008-2012.
This net drop in mortality occurred despite a concurrent rise in stillbirths, Rachel A. Pilliod, MD, said at the annual Pregnancy Meeting sponsored by the Society for Maternal-Fetal Medicine. The increase in stillbirths was more than counterbalanced by a larger drop in infant deaths during the same period.
“It’s not a one-to-one trade, where each stillbirth corresponds to an infant death that is subsequently avoided. It’s hard to make this trade-off when counseling parents,” she said. “We think that there has been some effect from increasing gestational age on reducing overall mortality, but we need to do even better on identifying high risk [deliveries].”
What is “unacceptable,” Dr. Pilliod said, is if a woman needs an earlier delivery but it gets pushed back because of a poorly informed application of the 39-week rule.
Her study used data collected by the National Center for Health Statistics on U.S. deliveries each year, focusing on pregnancies that were singletons and nonanomalous.
She compared the 7,388,782 deliveries during 2008 and 2009 and 6,980,962 births during 2011 and 2012, selecting the 2-year time periods on either side of the Joint Commission’s 2010 adoption of a quality measure aimed at decreasing elective deliveries prior to 39 weeks gestation.
The Joint Commission’s action had its desired effect. Deliveries at 39 weeks jumped from 36% of all elective births in 2008 and 2009 to 43% in 2011 and 2012, while deliveries at 38 weeks show the biggest drop, from 22% to 20%, Dr. Pilliod reported (Am J Obstet Gynecol. 2017 Jan. doi: 10.1016/j.ajog.2016.11.959).
Concurrent with the rise in 39-week births and a drop in neonates with shorter gestation times, the incidence of stillbirths rose from 9.32 per 10,000 births in 2008 and 2009 to 10.15, an increase of 0.83 per 10,000 births.
But during the same periods the incidence of infant deaths fell, from 20.63 per 10,000 births in 2008 and 2009 to 19.0 in 2011 and 2012, a reduction of 1.63 per 10,000. Overall the stillbirth and infant death data combined for a net mortality reduction of 0.8 per 10,000 births.
mzoler@frontlinemedcom.com
On Twitter @mitchelzoler
LAS VEGAS – Closer adherence by U.S. physicians to the “39-week rule” for elective deliveries appears to have cut net neonatal mortality in an analysis of more than 14 million deliveries during 2008-2012.
This net drop in mortality occurred despite a concurrent rise in stillbirths, Rachel A. Pilliod, MD, said at the annual Pregnancy Meeting sponsored by the Society for Maternal-Fetal Medicine. The increase in stillbirths was more than counterbalanced by a larger drop in infant deaths during the same period.
“It’s not a one-to-one trade, where each stillbirth corresponds to an infant death that is subsequently avoided. It’s hard to make this trade-off when counseling parents,” she said. “We think that there has been some effect from increasing gestational age on reducing overall mortality, but we need to do even better on identifying high risk [deliveries].”
What is “unacceptable,” Dr. Pilliod said, is if a woman needs an earlier delivery but it gets pushed back because of a poorly informed application of the 39-week rule.
Her study used data collected by the National Center for Health Statistics on U.S. deliveries each year, focusing on pregnancies that were singletons and nonanomalous.
She compared the 7,388,782 deliveries during 2008 and 2009 and 6,980,962 births during 2011 and 2012, selecting the 2-year time periods on either side of the Joint Commission’s 2010 adoption of a quality measure aimed at decreasing elective deliveries prior to 39 weeks gestation.
The Joint Commission’s action had its desired effect. Deliveries at 39 weeks jumped from 36% of all elective births in 2008 and 2009 to 43% in 2011 and 2012, while deliveries at 38 weeks show the biggest drop, from 22% to 20%, Dr. Pilliod reported (Am J Obstet Gynecol. 2017 Jan. doi: 10.1016/j.ajog.2016.11.959).
Concurrent with the rise in 39-week births and a drop in neonates with shorter gestation times, the incidence of stillbirths rose from 9.32 per 10,000 births in 2008 and 2009 to 10.15, an increase of 0.83 per 10,000 births.
But during the same periods the incidence of infant deaths fell, from 20.63 per 10,000 births in 2008 and 2009 to 19.0 in 2011 and 2012, a reduction of 1.63 per 10,000. Overall the stillbirth and infant death data combined for a net mortality reduction of 0.8 per 10,000 births.
mzoler@frontlinemedcom.com
On Twitter @mitchelzoler
LAS VEGAS – Closer adherence by U.S. physicians to the “39-week rule” for elective deliveries appears to have cut net neonatal mortality in an analysis of more than 14 million deliveries during 2008-2012.
This net drop in mortality occurred despite a concurrent rise in stillbirths, Rachel A. Pilliod, MD, said at the annual Pregnancy Meeting sponsored by the Society for Maternal-Fetal Medicine. The increase in stillbirths was more than counterbalanced by a larger drop in infant deaths during the same period.
“It’s not a one-to-one trade, where each stillbirth corresponds to an infant death that is subsequently avoided. It’s hard to make this trade-off when counseling parents,” she said. “We think that there has been some effect from increasing gestational age on reducing overall mortality, but we need to do even better on identifying high risk [deliveries].”
What is “unacceptable,” Dr. Pilliod said, is if a woman needs an earlier delivery but it gets pushed back because of a poorly informed application of the 39-week rule.
Her study used data collected by the National Center for Health Statistics on U.S. deliveries each year, focusing on pregnancies that were singletons and nonanomalous.
She compared the 7,388,782 deliveries during 2008 and 2009 and 6,980,962 births during 2011 and 2012, selecting the 2-year time periods on either side of the Joint Commission’s 2010 adoption of a quality measure aimed at decreasing elective deliveries prior to 39 weeks gestation.
The Joint Commission’s action had its desired effect. Deliveries at 39 weeks jumped from 36% of all elective births in 2008 and 2009 to 43% in 2011 and 2012, while deliveries at 38 weeks show the biggest drop, from 22% to 20%, Dr. Pilliod reported (Am J Obstet Gynecol. 2017 Jan. doi: 10.1016/j.ajog.2016.11.959).
Concurrent with the rise in 39-week births and a drop in neonates with shorter gestation times, the incidence of stillbirths rose from 9.32 per 10,000 births in 2008 and 2009 to 10.15, an increase of 0.83 per 10,000 births.
But during the same periods the incidence of infant deaths fell, from 20.63 per 10,000 births in 2008 and 2009 to 19.0 in 2011 and 2012, a reduction of 1.63 per 10,000. Overall the stillbirth and infant death data combined for a net mortality reduction of 0.8 per 10,000 births.
mzoler@frontlinemedcom.com
On Twitter @mitchelzoler
AT THE PREGNANCY MEETING
Key clinical point:
Major finding: Net mortality dropped by 0.8 per 10,000 births from 2008 and 2009 to 2011 and 2012.
Data source: Review of U.S. birth records from the National Center for Health Statistics during 2008-2012.
Disclosures: Dr. Pilliod reported having no financial disclosures.
Study supports multigene germline testing in colorectal cancer
About 10% of patients with colorectal cancer had at least one germline mutation known to increase the risk of cancer, according to results from a large single-center retrospective cohort study published in Journal of Clinical Oncology.
The findings “raise the provocative question of whether all patients with colorectal cancer (CRC) should undergo multigene germline testing for inherited cancer susceptibility,” especially because the results have implications for both patients and family members, said Matthew B. Yurgelun, MD, of Dana-Farber Cancer Institute in Boston, and his associates.
Hereditary factors long have been known to play a role in CRC, but clinicians have routinely limited genetic testing to phenotypically high-risk patients and to specific syndromes. For example, all patients with CRC are now typically tested for microsatellite instability (MSI) and mismatch repair deficiency (MRD) to identify the 2%-4% with Lynch syndrome. Likewise, patients with colorectal polyposis are routinely tested for familial adenomatous polyposis (FAP) and MUTYH-associated polyposis (MAP), the researchers noted.
To explore the prevalence of germline mutations among unselected patients with CRC, they studied 1,058 consecutive cases treated at Dana-Farber Institute between December 2008 and March 2014. The researchers tested blood samples for 25 genes linked to inherited cancer risk, reviewed medical records, and sifted through questionnaires to assess personal and family cancer histories, tumor locations, and the results of tests for MSI, MMR, and KRAS/NRAS and BRAF mutations. They also categorized each gene as high or moderate penetrance based on published estimates of the lifetime risk of cancer associated with pathogenic mutations of that gene.
Pathogenic germline mutations were found in 105 patients (9.9%; 95% confidence interval, 8.2%-11.9%). Lynch syndrome was the most common single mutational genotype, affecting 33 patients (3.1%). Twenty-eight of 29 (97%) MSI/MMR tests were abnormal, confirming the reliability of MSI/MMR testing for diagnosing Lynch syndrome, the researchers noted
Among 74 (7%) patients with mutations besides Lynch syndrome, 23 (2%) patients had mutations in high-penetrance genes, including 11 (1%) with BRCA1/2 mutations, five with APC, three with biallelic MUTYH, two with PALB2, one with CDKN2A, and one with TP53.
Notably, 15 of these patients had no clinical history that would have suggested an underlying mutation, the researchers noted. Another 38 (3.6%) patients had moderate-penetrance CRC risk gene mutations, including 19 patients with monoallelic MUTYH, 17 patients with APC*I1307K, and two patients with CHEK2 mutations, they said.
Additional analysis failed to link pathogenic mutations to age at CRC diagnosis, family history of CRC, or personal history of other cancers. Thus, clinicians should not skip comprehensive germline testing in CRC just because patients lack these characteristics, the researchers said. Indeed, the findings “clearly illustrate that genetic factors that underlie CRC extend beyond [the] well-recognized familial CRC syndromes, are markedly more common than previously appreciated, frequently occur in patients with CRC who lack classic high-risk features, and are found predominantly in genes for which specialized risk-reducing interventions are recommended,” they emphasized.
The National Cancer Institute and Myriad Genetics supported the work. Dr. Yurgelun disclosed research funding from Myriad Genetics.
About 10% of patients with colorectal cancer had at least one germline mutation known to increase the risk of cancer, according to results from a large single-center retrospective cohort study published in Journal of Clinical Oncology.
The findings “raise the provocative question of whether all patients with colorectal cancer (CRC) should undergo multigene germline testing for inherited cancer susceptibility,” especially because the results have implications for both patients and family members, said Matthew B. Yurgelun, MD, of Dana-Farber Cancer Institute in Boston, and his associates.
Hereditary factors long have been known to play a role in CRC, but clinicians have routinely limited genetic testing to phenotypically high-risk patients and to specific syndromes. For example, all patients with CRC are now typically tested for microsatellite instability (MSI) and mismatch repair deficiency (MRD) to identify the 2%-4% with Lynch syndrome. Likewise, patients with colorectal polyposis are routinely tested for familial adenomatous polyposis (FAP) and MUTYH-associated polyposis (MAP), the researchers noted.
To explore the prevalence of germline mutations among unselected patients with CRC, they studied 1,058 consecutive cases treated at Dana-Farber Institute between December 2008 and March 2014. The researchers tested blood samples for 25 genes linked to inherited cancer risk, reviewed medical records, and sifted through questionnaires to assess personal and family cancer histories, tumor locations, and the results of tests for MSI, MMR, and KRAS/NRAS and BRAF mutations. They also categorized each gene as high or moderate penetrance based on published estimates of the lifetime risk of cancer associated with pathogenic mutations of that gene.
Pathogenic germline mutations were found in 105 patients (9.9%; 95% confidence interval, 8.2%-11.9%). Lynch syndrome was the most common single mutational genotype, affecting 33 patients (3.1%). Twenty-eight of 29 (97%) MSI/MMR tests were abnormal, confirming the reliability of MSI/MMR testing for diagnosing Lynch syndrome, the researchers noted
Among 74 (7%) patients with mutations besides Lynch syndrome, 23 (2%) patients had mutations in high-penetrance genes, including 11 (1%) with BRCA1/2 mutations, five with APC, three with biallelic MUTYH, two with PALB2, one with CDKN2A, and one with TP53.
Notably, 15 of these patients had no clinical history that would have suggested an underlying mutation, the researchers noted. Another 38 (3.6%) patients had moderate-penetrance CRC risk gene mutations, including 19 patients with monoallelic MUTYH, 17 patients with APC*I1307K, and two patients with CHEK2 mutations, they said.
Additional analysis failed to link pathogenic mutations to age at CRC diagnosis, family history of CRC, or personal history of other cancers. Thus, clinicians should not skip comprehensive germline testing in CRC just because patients lack these characteristics, the researchers said. Indeed, the findings “clearly illustrate that genetic factors that underlie CRC extend beyond [the] well-recognized familial CRC syndromes, are markedly more common than previously appreciated, frequently occur in patients with CRC who lack classic high-risk features, and are found predominantly in genes for which specialized risk-reducing interventions are recommended,” they emphasized.
The National Cancer Institute and Myriad Genetics supported the work. Dr. Yurgelun disclosed research funding from Myriad Genetics.
About 10% of patients with colorectal cancer had at least one germline mutation known to increase the risk of cancer, according to results from a large single-center retrospective cohort study published in Journal of Clinical Oncology.
The findings “raise the provocative question of whether all patients with colorectal cancer (CRC) should undergo multigene germline testing for inherited cancer susceptibility,” especially because the results have implications for both patients and family members, said Matthew B. Yurgelun, MD, of Dana-Farber Cancer Institute in Boston, and his associates.
Hereditary factors long have been known to play a role in CRC, but clinicians have routinely limited genetic testing to phenotypically high-risk patients and to specific syndromes. For example, all patients with CRC are now typically tested for microsatellite instability (MSI) and mismatch repair deficiency (MRD) to identify the 2%-4% with Lynch syndrome. Likewise, patients with colorectal polyposis are routinely tested for familial adenomatous polyposis (FAP) and MUTYH-associated polyposis (MAP), the researchers noted.
To explore the prevalence of germline mutations among unselected patients with CRC, they studied 1,058 consecutive cases treated at Dana-Farber Institute between December 2008 and March 2014. The researchers tested blood samples for 25 genes linked to inherited cancer risk, reviewed medical records, and sifted through questionnaires to assess personal and family cancer histories, tumor locations, and the results of tests for MSI, MMR, and KRAS/NRAS and BRAF mutations. They also categorized each gene as high or moderate penetrance based on published estimates of the lifetime risk of cancer associated with pathogenic mutations of that gene.
Pathogenic germline mutations were found in 105 patients (9.9%; 95% confidence interval, 8.2%-11.9%). Lynch syndrome was the most common single mutational genotype, affecting 33 patients (3.1%). Twenty-eight of 29 (97%) MSI/MMR tests were abnormal, confirming the reliability of MSI/MMR testing for diagnosing Lynch syndrome, the researchers noted
Among 74 (7%) patients with mutations besides Lynch syndrome, 23 (2%) patients had mutations in high-penetrance genes, including 11 (1%) with BRCA1/2 mutations, five with APC, three with biallelic MUTYH, two with PALB2, one with CDKN2A, and one with TP53.
Notably, 15 of these patients had no clinical history that would have suggested an underlying mutation, the researchers noted. Another 38 (3.6%) patients had moderate-penetrance CRC risk gene mutations, including 19 patients with monoallelic MUTYH, 17 patients with APC*I1307K, and two patients with CHEK2 mutations, they said.
Additional analysis failed to link pathogenic mutations to age at CRC diagnosis, family history of CRC, or personal history of other cancers. Thus, clinicians should not skip comprehensive germline testing in CRC just because patients lack these characteristics, the researchers said. Indeed, the findings “clearly illustrate that genetic factors that underlie CRC extend beyond [the] well-recognized familial CRC syndromes, are markedly more common than previously appreciated, frequently occur in patients with CRC who lack classic high-risk features, and are found predominantly in genes for which specialized risk-reducing interventions are recommended,” they emphasized.
The National Cancer Institute and Myriad Genetics supported the work. Dr. Yurgelun disclosed research funding from Myriad Genetics.
FROM THE JOURNAL OF CLINICAL ONCOLOGY
Key clinical point: Consider comprehensive germline testing in all patients with colorectal cancer.
Major finding: A total of 9.9% of patients had pathogenic germline mutations.
Data source: A single-center retrospective cohort study of 1,058 unselected patients with colorectal cancer.
Disclosures: The National Cancer Institute and Myriad Genetics supported the work. Dr. Yurgelun disclosed research funding from Myriad Genetics.
