Etanercept improves linear growth and bone mass acquisition in MTX-resistant polyarticular-course juvenile idiopathic arthritis.

OBJECTIVES: Chronic inflammation in juvenile idiopathic arthritis interferes with linear growth and bone mass acquisition. We prospectively evaluated and compared linear growth and evolution of bone mass acquisition and body composition in MTX-resistant polyarticular-course JIA (polyJIA) patients started on etanercept and in recently diagnosed polyJIA patients started on MTX monotherapy. METHODS: Sixteen MTX-resistant polyJIA patients were given add-on etanercept, eight recently diagnosed polyJIA patients were started on MTX. Patients were evaluated at baseline and at 1, 6, 12 and 18 months with respect to disease activity, linear growth, BMD and body composition. RESULTS: Baseline patient and disease characteristics were similar in both groups. Clinical disease activity (Pediatric ACR30) was equally well controlled in both groups. Growth velocity increased significantly allowing catch-up growth in the etanercept + MTX group only. BMD (lumbar spine Z-score) improved significantly in both groups. A significant increase of bone mineral content and lean:fat mass ratio was seen in the etanercept + MTX group, but not in the MTX group. CONCLUSION: Clinical control of disease activity by etanercept in MTX-refractory polyJIA is associated with rapidly instituted catch-up growth and improvement of bone mineralization and body composition. In recently diagnosed polyJIA patients treated with MTX the relation between clinical response and these parameters was less evident. Preliminary data on serum IL-6 and osteoprotegerin levels indicate that the beneficial effects seen with etanercept therapy may be related to its control of systemic IL-6 production and enhancement of osteoblast activity.

Lemierre syndrome in a 5-month-old male infant: Case report and review of the pediatric literature.

OBJECTIVES: To report, to the best of our knowledge, the youngest patient with Lemierre syndrome. DESIGN: Descriptive case report with review of the pediatric literature. SETTING: Pediatric intensive care unit in a tertiary referral hospital. DATA SOURCE: Systemic review of the literature, including PubMed (English-only journals) and major textbooks. PATIENT: We report a 5-month-old boy who presented with fever and a perforated left-sided otitis media. He developed left-sided complicated otitis media with retroauricular fluid collection, mastoiditis, and temporomandibular joint effusion. The clinical picture was complicated by a left internal jugular vein and left lateral sinus thrombosis. Fusobacterium necrophorum grew in the pus culture. INTERVENTIONS: Low molecular weight heparin. MEASUREMENTS AND MAIN RESULTS: No immunodeficiency and no thrombophilia were identified as predisposing conditions for Lemierre syndrome. Surgical drainage, early and adequate antibiotic treatment, and anticoagulation were followed by complete recovery. CONCLUSIONS: This case report illustrates that Lemierre syndrome can occur in infants without underlying risk factors for severe infections or thrombotic complications.

Establishment and characterization of an atypical cell line from a patient with Wiskott-Aldrich-Syndrome and bone marrow allografting

Se aislaron blastos atípicos de la sangre periférica de un niño con síndrome de Wiskott-Aldrich y autotransplante de médula ósea. Las células fueron inmunotipificadas como monoblastos y crecieron en cultivo tisular en doble tiempo de 3 a 4 días. Las células aisladas originalmente y los cultivos iniciales, contenían antígenos tanto del virus herpes humano-6 (HHV-6) como del virus herpes humano-7 (HHV-7) y ácido desoxirribonucleico (ADN). Lo que disminuyó con la desdiferenciación celular en los cultivos subsecuentes. Los sobrenadantes de los cultivos celulares contenían cantidades moderadas de interleucina-6 (IL-6) y marcados niveles de factor estimulante de colonias-granulocito-monocítico (GM-CSF). Se discutió el caso desde el punto de vista de una posible co-patogénesis viral