title: Polyglandular endocrine failure in a patient with amyloidosis secondary to familial Mediterranean fever.
abstract: Familial Mediterranean fever (FMF) is 1 of the major causes of secondary amyloidosis. Renal involvement is the main clinical complication and it mostly presents with nephrotic syndrome and chronic renal failure. Although deposition of amyloid has been reported in several endocrine glands such as the adrenal, thyroid, and testes, clinically significant functional impairment is uncommon. Herein, we describe a patient in whom the diagnosis of FMF was based on molecular screening and who presented with recurrent hypoglycemic attacks and extensive amyloid deposition affecting various organ function including adrenal, thyroid, parathyroid, testes, intestinal system, and the heart.
authors.affiliation: Departments of Nephrology and Urology, Ankara University Medical School, Ibni Sina Hospital, Ankara, Turkey. kenankeven@hotmail.com
authors.forename: K
authors.initials: K
authors.lastname: Keven
authors.forename: E
authors.initials: E
authors.lastname: Oztas
authors.forename: H
authors.initials: H
authors.lastname: Aksoy
authors.forename: N
authors.initials: N
authors.lastname: Duman
authors.forename: B
authors.initials: B
authors.lastname: Erbay
authors.forename: S
authors.initials: S
authors.lastname: Ertürk
mesh_terms: D000686:Amyloidosis / Q000209:etiology*; D004700:Endocrine System Diseases / Q000209:etiology*; D010505:Familial Mediterranean Fever / Q000150:complications*; D006801:Humans; D007003:Hypoglycemia / Q000209:etiology; D007410:Intestinal Diseases / Q000209:etiology; D007676:Kidney Failure, Chronic / Q000150:complications / Q000628:therapy; D008297:Male; D008875:Middle Aged; D006435:Renal Dialysis