[AA renal amyloidosis in child: a report of 5 cases]

ABSTRACT

AA amyloidosis is a rare but serious complication of chronic inflammatory diseases and chronic infections. The kidney is the most frequently affected organ in AA amyloidosis. In order to analyse the clinical and histologic characteristics of AA amyloidosis in our pediatric population, to describe their aetiology and to examine clinical out-come. Retrospective study of 5 cases of AA amyloidosis diagnosed from 1997 to 2006 in our department. 5 cases, 3 girls and 2 boys, with median age at diagnosis of 10 years and 9 meonths. The AA amyloidosis aetiologies were 3 cases with familial Mediterranean fever and 2 with juvenile chronic arthritis. In 2 patients, diagnosis was done through renal biopsy, in 2 patients by rectal biopsy and in one patient by salivary biopsy. All patients progressed to end-stage renal failure 3 years follow up diagnosis. AA renal amyloidosis has a low incidence in our children population but it remains dramatic complication. Rheumatological disorders and familial Mediterranean fever are the principal aetiology. The prognosis tends to be better with hemodiyalisis