Medium-size-vessel vasculitis

Medium-size-artery vasculitides do occur in childhood and manifest, in the main, as polyarteritis nodosa (PAN), cutaneous PAN and Kawasaki disease. Of these, PAN is the most serious, with high morbidity and not inconsequential mortality rates. New classification criteria for PAN have been validated that will have value in epidemiological studies and clinical trials. Renal involvement is common and recent therapeutic advances may result in improved treatment options. Cutaneous PAN is a milder disease characterised by periodic exacerbations and often associated with streptococcal infection. There is controversy as to whether this is a separate entity or part of the systemic PAN spectrum. Kawasaki disease is an acute self-limiting systemic vasculitis, the second commonest vasculitis in childhood and the commonest cause of childhood-acquired heart disease. Renal manifestations occur and include tubulointerstitial nephritis and renal failure. An infectious trigger and a genetic predisposition seem likely. Intravenous immunoglobulin (IV-Ig) and aspirin are effective therapeutically, but in resistant cases, either steroid or infliximab have a role. Greater understanding of the pathogenetic mechanisms involved in these three types of vasculitis and better long-term follow-up data will lead to improved therapy and prediction of prognosis

Concomitant compression of median and ulnar nerves in a hemophiliac patient: A case report

A 15-year-old boy, with a diagnosis of hemophilia A, suffered bleeding into his left forearm 5 months before being admitted to our medical center. His neurological examination revealed a pronounced median neuropathy and a minor ulnar neuropathy on the left side. There was marked muscle atrophy on the thenar side and, to a lesser degree, on the hypothenar side and in the forearm. Electromyographic findings demonstrated an evident, nearly complete, sensorimotor axonal loss in the median nerve. Magnetic resonance imaging studies showed atrophy in muscles of the left forearm and median nerve. The patient was diagnosed as having median nerve axonotmesis and ulnar nerve neuropraxia due to compartment syndrome. In hemophiliac patients, frequent single nerve compressions (often involving the femoral nerve) can be seen. However, concomitant median and ulnar nerve injuries with differing severity are rare. © 2002 Éditions scientifiques et médicales Elsevier SAS

case series with long term follow-up

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent self-limited attacks of fever and polyserositis. Reactive amyloid A amyloidosis is the most devastating complication of FMF, and amyloidosis continues to occur in the colchicine era in untreated and noncompliant patients. Unfortunately, there is no proven effective treatment for established amyloidosis. In this report, we present four FMF-related amyloidosis patients that were treated with long term infliximab therapy with the longest duration of follow-up, together with the literature review. Infliximab was very effective in controlling gastrointestinal system findings and protracted arthritis, and it also had a favorable impact on the clinical findings of nephrotic syndrome in these patients. In conclusion, by controlling debilitating complaints of amyloidosis with infliximab, quality of life increases in these patients, and they get rid of recurrent hospitalizations and return to school or work

Clinical characteristics of patients with genetically confirmed alport syndrome

Abstract Not Availabl

Turkish Atypical Hemolytic Uremic Syndrome Registry: Renal biopsy findings and prognosis

Abstract Not Availabl