A case of familial Mediterranean fever with amyloidosis as the first manifestation

Citation
S. Kutlay et al., A case of familial Mediterranean fever with amyloidosis as the first manifestation, AM J KIDNEY, 38(6), 2001, pp. NIL_23-NIL_26
Citations number
17
Categorie Soggetti
Urology & Nephrology
Journal title
AMERICAN JOURNAL OF KIDNEY DISEASES
ISSN journal
02726386 → ACNP
Volume
38
Issue
6
Year of publication
2001
Pages
NIL_23 - NIL_26
Database
ISI
SICI code
0272-6386(200112)38:6<NIL_23:ACOFMF>2.0.ZU;2-6
Abstract
We describe a 22-year-old Turkish woman with nephrotic syndrome who had a h istory of acute myelocytic leukemia. After careful clinical evaluation, the patient underwent a renal biopsy. Light microscopic examination showed dep osition of Congo-positive material both In the mesangium and around the sma ll vessels. By histochemical analyses, the deposited material was proved to be amyloid A (AA). Because the patient's history did not reveal any event that might explain the development of secondary amyloidosis, she was screen ed for mutations causing familial Mediterranean fever (FMF) and was found t o be homozygous for the M694V mutation by denaturing gradient gel electroph oresis. We recommend that FMF-Phenotype If and the development of amyloid n ephropathy, before or without other symptoms of FMF, should be kept In mind in the face of unexplained proteinuria/amyloidosis, especially In high-ris k ethnic groups. (C) 2001 by the National Kidney Foundation, Inc.