Managing Antiphospholipid Syndrome in Children and Adolescents: Current and Future Prospects.

Pediatric antiphospholipid syndrome (APS) is a rare acquired multisystem autoimmune thromboinflammatory condition characterized by thrombotic and non-thrombotic clinical manifestations. APS in children and adolescents typically presents with large-vessel thrombosis, thrombotic microangiopathy, and, rarely, obstetric morbidity. Non-thrombotic clinical manifestations are frequently seen in pediatric APS and may be present even before the vascular thrombotic events occur. We review insights into the pathogenesis of APS and discuss potential targets for therapy. The identification of multiple immunologic abnormalities in patients with APS reveals molecular targets for current or future treatment. Management strategies, especially for APS in adolescents, require screening for additional prothrombotic risk factors and consideration of counseling regarding contraceptive strategies, lifestyle recommendations, treatment adherence, and mental health issues associated with this autoimmune thrombophilia. The main goal of therapy in pediatric APS is the prevention of thrombosis. The management of acute thrombosis events in children and adolescents is the same as for primary APS, which involves isolated occurrences, and secondary APS, which is seen in association with another autoimmune disease, e.g., systemic lupus erythematosus. A pediatric hematologist should be consulted so other differential thrombophilic conditions can be eliminated. Therapy includes unfractionated heparin or low-molecular-weight heparin followed by vitamin K antagonists. Treatment of catastrophic APS involves triple therapy (anticoagulation, intravenous corticosteroid pulse therapy, and plasma exchange) and may include intravenous immunoglobulin for children and adolescents with this condition. New drugs such as eculizumab and sirolimus seem to be promising drugs for APS.

Intermittent abdominal pain in IgA vasculitis.

OBJECTIVE: To assess intermittent abdominal pain in IgA vasculitis patients and its relation to demographic data, clinical manifestations and treatments. METHODS: A retrospective cohort study included 322 patients with IgA vasculitis (EULAR/PRINTO/PRES criteria) seen at the Pediatric Rheumatology Unit in the last 32 years. Sixteen patients were excluded due to incomplete data in medical charts. Intermittent abdominal pain was characterized by new abdominal pain after complete resolution in the first month of disease. RESULTS: Intermittent abdominal pain was observed in 35/306 (11%) IgA vasculitis patients. The median time between first and second abdominal pain was 10 days (3-30 days). The main treatment of intermittent abdominal pain included glucocorticoid [n=26/35 (74%)] and/or ranitidine [n=22/35 (63%)]. Additional analysis showed that the frequency of intermittent purpura/petechiae (37 vs. 21%; p=0.027) and the median of purpura/petechiae duration [20 (3-90) vs. 14 (1-270) days; p=0.014] were significantly higher in IgA vasculitis patients with intermittent abdominal pain compared to those without. Gastrointestinal bleeding (49 vs. 13%; p<0.001), nephritis (71 vs. 45%; p=0.006), glucocorticoid (74 vs. 44%; p=0.001) and intravenous immunoglobulin use (6 vs. 0%; p=0.036) were also significantly higher in the former group. The frequency of ranitidine use was significantly higher in IgA vasculitis patients with intermittent abdominal pain versus without (63 vs. 28%; p<0.001), whereas the median of ranitidine duration was reduced in the former group [35 (2-90) vs. 60 (5-425) days; p=0.004]. CONCLUSIONS: Intermittent abdominal pain occurred in nearly a tenth of IgA vasculitis patients, in the first 30 days of disease, and was associated with other severe clinical features. Therefore, this study suggests that these patients should be followed strictly with clinical and laboratorial assessment, particularly during the first month of disease course.

An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus.

Childhood-onset systemic lupus erythematosus (cSLE) is a prototype of a multisystemic, inflammatory, heterogeneous autoimmune condition. This disease is characterized by simultaneous or sequential organ and system involvement, with unpredictable flare and high levels of morbidity and mortality. Racial/ethnic background, socioeconomic status, cost of medications, difficulty accessing health care, and poor adherence seem to impact lupus outcomes and treatment response. In this article, the management of cSLE patients is updated. Regarding pathogenesis, a number of potential targets for drugs have been studied. However, most treatments in pediatric patients are off-label drugs with recommendations based on inadequately powered studies, therapeutic consensus guidelines, or case series. Management practices for cSLE patients include evaluations of disease activity and cumulative damage scores, routine non-live vaccinations, physical activity, and addressing mental health issues. Antimalarials and glucocorticoids are still the most common drugs used to treat cSLE, and hydroxychloroquine is recommended for nearly all cSLE patients. Disease-modifying antirheumatic drugs (DMARDs) should be standardized for each patient, based on disease flare and cSLE severity. Mycophenolate mofetil or intravenous cyclophosphamide is suggested as induction therapy for lupus nephritis classes III and IV. Calcineurin inhibitors (cyclosporine, tacrolimus, voclosporin) appear to be another good option for cSLE patients with lupus nephritis. Regarding B-cell-targeting biologic agents, rituximab may be used for refractory lupus nephritis patients in combination with another DMARD, and belimumab was recently approved by the US Food and Drug Administration for cSLE treatment in children aged > 5 years. New therapies targeting CD20, such as atacicept and telitacicept, seem to be promising drugs for SLE patients. Anti-interferon therapies (sifalimumab and anifrolumab) have shown beneficial results in phase II randomized control trials in adult SLE patients, as have some Janus kinase inhibitors, and these could be alternative treatments for pediatric patients with severe interferon-mediated inflammatory disease in the future. In addition, strict control of proteinuria and blood pressure is required in cSLE, especially with angiotensin-converting enzyme inhibitor and angiotensin receptor blocker use.

PEDIATRICIANS AFTER RESIDENCY: A SURVEY OF PERSONAL/PROFESSIONAL DATA AND ISSUES.

OBJECTIVE: To assess personal, professional, medical, and scientific educational characteristics and issues reported by pediatricians. METHODS: Cross-sectional study based on an online survey including 614 pediatricians who graduated in the last 15 years at a University Pediatric Department in Brazil. RESULTS: The response rate was 331/614(54%). The majority were females (82%), the median age was 33 years (27-40) and median years of pediatric practice was 5 (1-13). High workload (>60 hours/week) occurred in 25% and 47% earned ≥15 minimum wages/month. The most work-related issues reported were long working hours, poor social life and a sedentary lifestyle (>50%). Pediatricians were further divided into two groups, according to years of pediatric clinical practice: group 1 (≤5 years) and group 2 (>5 years). The median of overall satisfaction with pediatric residency [8(0-10) vs. 9 (4-10); p=0.002] was significantly reduced in group 1. The frequencies of workload >60 hours, work on pediatric ward and pediatric intensive care were significantly higher in the first group (p<0.05). Regarding main issues related to clinical practice in the last year, long working hours (73 vs. 53%; p<0.001), poor social life (75 vs. 62%; p=0.018) and harassment (23 vs. 4%; p=0.003) were significantly higher in the first group. CONCLUSIONS: Very early career pediatricians (≤5 years) reported higher workload, lower income, work-related issues and different location of pediatric practice compared to early career pediatricians (>5 years). The overall satisfaction with pediatric residency was good, however, reduced in very early career pediatricians.

Pediatricians after residency: a survey of personal/professional data and issues / Pediatras após a residência médica: um questionário sobre dados e problemas pessoais/profissionais

ABSTRACT Objective: To assess personal, professional, medical, and scientific educational characteristics and issues reported by pediatricians. Methods: Cross-sectional study based on an online survey including 614 pediatricians who graduated in the last 15 years at a University Pediatric Department in Brazil. Results: The response rate was 331/614(54%). The majority were females (82%), the median age was 33 years (27-40) and median years of pediatric practice was 5 (1-13). High workload (>60 hours/week) occurred in 25% and 47% earned ≥15 minimum wages/month. The most work-related issues reported were long working hours, poor social life and a sedentary lifestyle (>50%). Pediatricians were further divided into two groups, according to years of pediatric clinical practice: group 1 (≤5 years) and group 2 (>5 years). The median of overall satisfaction with pediatric residency [8(0-10) vs. 9 (4-10); p=0.002] was significantly reduced in group 1. The frequencies of workload >60 hours, work on pediatric ward and pediatric intensive care were significantly higher in the first group (p<0.05). Regarding main issues related to clinical practice in the last year, long working hours (73 vs. 53%; p<0.001), poor social life (75 vs. 62%; p=0.018) and harassment (23 vs. 4%; p=0.003) were significantly higher in the first group. Conclusions: Very early career pediatricians (≤5 years) reported higher workload, lower income, work-related issues and different location of pediatric practice compared to early career pediatricians (>5 years). The overall satisfaction with pediatric residency was good, however, reduced in very early career pediatricians.

RESUMO Objetivo: Avaliar características e problemas pessoais, profissionais, médicos e de educação científica reportados por pediatras. Métodos: Estudo transversal com base em uma pesquisa online, incluindo 614 pediatras formados nos últimos 15 anos no Departamento de Pediatria de uma universidade brasileira. Resultados: A taxa de resposta foi de 331/614 (54%). A maioria dos participantes era do sexo feminino (82%), a mediana de idade foi de 33 anos (27-40 anos) e a mediana de tempo de prática pediátrica foi de 5 anos (1-13). Jornada de trabalho elevada (>60 horas/semana) foi relatada por 25% dos entrevistados e 47% recebiam ≥15 salários mínimos/mês. Os problemas relacionados ao trabalho mais frequentes foram jornadas longas de trabalho, vida social insatisfatória e sedentarismo (>50%). Os pediatras foram divididos em dois grupos de acordo com os anos de prática clínica pediátrica: grupo 1 (≤5 anos) e grupo 2 (>5 anos). A mediana de satisfação geral com a residência pediátrica [8 (0-10) versus 9 (4-10); p=0,002] foi significativamente menor no grupo 1. As frequências de jornada de trabalho >60 horas, trabalho na enfermaria pediátrica e na terapia intensiva pediátrica foram significativamente maiores no primeiro grupo (p<0,05). Quanto aos principais problemas relacionados à prática clínica no ano anterior, jornadas longas de trabalho (73 versus 53%; p<0,001), vida social insatisfatória (75 versus 62%; p=0,018) e assédio (23 versus 4%; p=0,003) foram significativamente mais elevados no grupo 1. Conclusões: Pediatras em início de carreira (≤5 anos) relataram maior jornada de trabalho, menor renda, problemas relacionados ao trabalho e diferentes locais de trabalho em comparação com pediatras mais experientes (>5 anos). A satisfação geral com a residência pediátrica foi boa, porém menor em pediatras do primeiro grupo.

Profile of health professionals who completed a master's, doctoral, or post-doctoral degree in one Brazilian pediatric program.

OBJECTIVE: This study aimed to determine the personal and professional characteristics, and the physical, psychiatric/psychological, and professional issues that exist among master's-, doctoral-, and post-doctoral-level health professionals. METHODS: A cross-sectional, online, self-reported survey of 452 postgraduates who completed master's, doctoral, or post-doctoral degrees in one graduate program in pediatrics in São Paulo, Brazil, was conducted. RESULTS: The response rate was 47% (211/453). The majority of participants were women (78%) and physicians (74%), and the median age was 47 years (28-71). Master's, doctoral, and post-doctoral degrees were reported by 73%, 53%, and 3%, respectively. High workload (>40 hours/week) occurred in 59%, and 45% earned ≥15 minimum wages/month. At least one participation in scientific meeting in the past year was reported by 91%, and 79% had published their research. Thirty-nine percent served as a member of a faculty of an institution of higher learning. The data were analyzed by two age groups: participants aged ≤48 years (group 1) and participants aged >48 years (group 2). The median rating of overall satisfaction with the profession in the past year [8 (0-10) vs. 9 (1-10), p=0.0113]; workload >40 hours/week (53% vs. 68%, p=0.034); and ≥15 minimum wages/month (37% vs. 56%, p=0.0083) were significantly lower in group 1. Further analysis by gender revealed that the median rating of overall satisfaction with the profession in the past year [8 (0-10) vs. 9 (3-10), p=0.0015], workload >40 hours/week (53% vs. 83%, p=0.0002), and ≥15 minimum wages/month (37% vs. 74%, p=0.0001) were significantly lower in women compared with men. The median rating of overall satisfaction with the mentorship supervision provided was significantly higher among the women 10 (5-10) vs. 10 (2-10), p=0.0324]. CONCLUSIONS: The majority of master's-, doctoral-, and post-doctoral-level health professionals were women and physicians, and had published their thesis. Younger postgraduates and women reported low salaries, less likelihood of working >40 hours/week, and less overall satisfaction with their profession. Further longitudinal and qualitative studies are warranted to assess career trajectories after graduation.

Profile of health professionals who completed a master's, doctoral, or post-doctoral degree in one Brazilian pediatric program

OBJECTIVE: This study aimed to determine the personal and professional characteristics, and the physical, psychiatric/psychological, and professional issues that exist among master's-, doctoral-, and post-doctoral-level health professionals. METHODS: A cross-sectional, online, self-reported survey of 452 postgraduates who completed master's, doctoral, or post-doctoral degrees in one graduate program in pediatrics in São Paulo, Brazil, was conducted. RESULTS: The response rate was 47% (211/453). The majority of participants were women (78%) and physicians (74%), and the median age was 47 years (28-71). Master's, doctoral, and post-doctoral degrees were reported by 73%, 53%, and 3%, respectively. High workload (>40 hours/week) occurred in 59%, and 45% earned ≥15 minimum wages/month. At least one participation in scientific meeting in the past year was reported by 91%, and 79% had published their research. Thirty-nine percent served as a member of a faculty of an institution of higher learning. The data were analyzed by two age groups: participants aged ≤48 years (group 1) and participants aged >48 years (group 2). The median rating of overall satisfaction with the profession in the past year [8 (0-10) vs. 9 (1-10), p=0.0113]; workload >40 hours/week (53% vs. 68%, p=0.034); and ≥15 minimum wages/month (37% vs. 56%, p=0.0083) were significantly lower in group 1. Further analysis by gender revealed that the median rating of overall satisfaction with the profession in the past year [8 (0-10) vs. 9 (3-10), p=0.0015], workload >40 hours/week (53% vs. 83%, p=0.0002), and ≥15 minimum wages/month (37% vs. 74%, p=0.0001) were significantly lower in women compared with men. The median rating of overall satisfaction with the mentorship supervision provided was significantly higher among the women 10 (5-10) vs. 10 (2-10), p=0.0324]. CONCLUSIONS: The majority of master's-, doctoral-, and post-doctoral-level health professionals were women and physicians, and had published their thesis. Younger postgraduates and women reported low salaries, less likelihood of working >40 hours/week, and less overall satisfaction with their profession. Further longitudinal and qualitative studies are warranted to assess career trajectories after graduation.