A questionnaire-based retrospective clinical and immunological survey was conducted in 73 males with a definite diagnosis of X-linked agammaglobulinemia based on BTK sequence analysis. Forty-four… (More)
A retrospective clinical and immunological survey was conducted in 60 patients with Chronic Granulomatous Disease. A prospective controlled non-randomized study of the efficacy of long-term IFNgamma… (More)
Predictive factors of renal involvement or relapsing disease in children with Henoch-Schönlein purpura (HSP) are poorly known. We examined clinical and laboratory variables concerning HSP evolution… (More)
European review for medical and pharmacological…
2006
Familial Mediterranean fever (FMF) is the prototype of auto-inflammatory disorders and is ethnically restricted to people living in the Mediterranean basin and Middle-East. Pyrin, the protein product… (More)
We report our prospective experience with sensitivity, specificity, predictive values and efficiency of echocardiography in diagnosing AD involving the ascending aorta (type A). We studied two groups… (More)
European review for medical and pharmacological…
2006
Macrophage activation syndrome is a rare and potentially fatal complication of many childhood pathological settings, most frequently reported in systemic onset-juvenile idiopathic arthritis. The… (More)
OBJECTIVES
The aim of the study is to assess the rate of atypical manifestations at onset in pediatric systemic lupus erythematosus (SLE) and to evaluate their effect on disease outcome.
STUDY… (More)
The exact elucidation of skeletal and cartilagineous involvement in neonatal-onset multisystem inflammatory disease (NOMID) is still poorly known, and there are few data providing the long-term… (More)
Henoch-Schönlein purpura (HSP), the commonest vasculitis in children, occurs most frequently between the ages of 4 and 6 years. We report the case of a 3-year-old boy with an otomastoiditis who was… (More)
We describe for the first time a case of macrophage activation syndrome in a child with hyperimmunoglobulinemia D with periodic fever syndrome who required intensive care support. Up-regulated… (More)