Diagnosis and treatment of antiphospholipid syndrome in childhood: A review.

The antiphospholipid syndrome (APS) is a multisystem autoimmune disease characterized by recurrent fetal loss and thromboembolic events associated with the presence of elevated titres of antiphospholipid antibodies (aPL). The purpose of this review is to summarize what is currently known about the diagnosis and treatment of pediatric APS, to highlight key differences between APS presenting in adults versus children throughout, and to identify areas where future research is needed.

Pediatric antiphospholipid syndrome.

Antiphospholipid syndrome (APS) is a multisystem autoimmune condition characterized by vascular thromboses associated with persistently positive antiphospholipid antibodies. There is currently a paucity of data (incidence, prevalence, thrombosis risk, and effective treatment) in pediatric APS. The purpose of this report is to review the current literature on APS in children and neonates, identify the gaps in current knowledge, and suggest avenues for studies to fill those gaps.

Catch-up growth during tocilizumab therapy for systemic juvenile idiopathic arthritis: results from a phase III trial.

Objective: To investigate the impact of tocilizumab treatment on growth and growth-related laboratory parameters in patients with systemic juvenile idiopathic arthritis (JIA) enrolled in a phase III clinical trial.

Methods: Patients with systemic JIA ages 2-17 years (n = 112) received tocilizumab in a 12-week, randomized, placebo-controlled period and a long-term open-label extension. Height velocity and standard deviation (SD) score; levels of insulin-like growth factor 1 (IGF-1), osteocalcin (OC), and C-telopeptide of type I collagen (CTX-I); and Juvenile Arthritis Disease Activity Score in 71 joints (JADAS-71) were measured in a post hoc analysis of 83 patients who never received growth hormone and did not reach Tanner stage 5 by the end of the first year of treatment.

Variability in delivery of care and echocardiogram surveillance of Kawasaki disease.

Objective: The objective of this study is to characterize variability in the acute management of Kawasaki disease and compliance of echocardiogram surveillance with published American Heart Association recommendations.

Design: Retrospective review.

Setting: Tertiary care children's hospital.

Lupus-associated causal mutation in neutrophil cytosolic factor 2 (NCF2) brings unique insights to the structure and function of NADPH oxidase.

Systemic lupus erythematosus (SLE), the prototypic systemic autoimmune disease, is a debilitating multisystem autoimmune disorder characterized by chronic inflammation and extensive immune dysregulation in multiple organ systems, resulting in significant morbidity and mortality. Here, we present a multidisciplinary approach resulting in the identification of neutrophil cytosolic factor 2 (NCF2) as an important risk factor for SLE and the detailed characterization of its causal variant. We show that NCF2 is strongly associated with increased SLE risk in two independent populations: childhood-onset SLE and adult-onset SLE.