İntrahepatik kolestazla başvuran sekonder amiloidozlu Ailevi Akdeniz Ateşi olgusu

Ailevi Akdeniz Ateşi Akdeniz havzasında yaşayan popülasyonu etkileyen otozomal resesif bir hastalıktır. Ailevi Akdeniz Ateşi'nin en yaygın komplikasyonlarından biri amiloidozdur. Amiloid AA tipidir ve sekonder amiloidoza özgüdür. İntrahepatik kolestaz, hepatik amiloidoz ve Ailevi Akdeniz Ateşli bir olgu sunumu yapılmıştır. Bu hasta tanıdan 14 ay sonra renal tutulum nedeniyle ölmüştür.

Anahtar Kelimeler:

Ailevi Akdeniz Ateşi, amiloidoz, intrahepatik kolestaz, karaciğer

A case of familial mediterranean fever with seconder amyloidosis and intrahepatic cholestasis signs

Familial Mediterranean fever (FMF) is an autosomal recessive hereditary disease which primarily affects populations surrounding the Mediterranean basin. One of the most significant complications of FMF is amyloidosis. The amyloid is of the AA type, which is typical of secondary amyloidosis. We report a case of a 24-year-old male with intrahepatic cholestasis, hepatic amyloid and FMF. The patient died because of renal involvement after 14 months.

Keywords:

Familial Mediterranean fever, amyloidosis, intrahepatic cholestasis, liver,

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