Complete remission of nephrotic syndrome in secondary amyloidosis of familial Mediterranean fever following colchicine treatment Ailevi Akdeniz ateşi ve sekonder amiloidozise bağlı nefrotik sendromun

Abstract

Keywords

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Ailevi Akdeniz ateşi ve sekonder amiloidozise bağlı nefrotik sendromun kolşisin tedavisi altında remisyonu

Abstract

Ateş, artrit ve karın ağrısı atakları ile 18 yıldır ailevi Akdeniz ateşi hikayesi olan 25 yaşında Türk kadın hasta, 1999 yılında nefrotik sendrom kliniği ile hastanemize başvurdu. Renal ve intestinal mukozadan (rektum) yapılan biyopsilerde amiloid depolanması saptandı. Hastada homozigot M694V mutasyonu mevcut idi. Kolşisin tedavisi altında klinik belirtiler ve nefrotik düzeydeki proteinüri kayboldu. Literatürde benzer vakalar nadir olarak tarif edilmiştir. Bu hastada ailevi Akdeniz ateşin tanı ve tedavisinde 18 yıllık bir gecikme ve kolşisin tedavisi altında sekonder amiloidozise bağlı nefrotik sendromun kaybolarak iyi bir seyir göstermesi tartışıldı.

Keywords

• AA amiloidozise, ailevi Akdeniz ateşi, nefrotik sendrom, kolşisin

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