%0 Journal Article
%T Renal and suprarenal insufficiency secondary to familial Mediterranean fever associated with amyloidosis: a case report
%A Ahmet Toros
%A Fusun Erdenen
%A Nagehan Sari
%A Serkan Gokcay
%J Journal of Medical Case Reports
%D 2011
%I BioMed Central
%R 10.1186/1752-1947-5-390
%X In this report, we describe the case of a 33-year-old Turkish man with familial Mediterranean fever and chronic renal insufficiency. He was admitted to our clinic with symptoms of suprarenal insufficiency. The patient died three months later as a result of cardiac arrest.Our aim is to make a contribution to the literature by reporting a case of combined insufficiency due to the accumulation of renal and adrenal amyloid in a patient with familial Mediterranean fever, which has very rarely been described in the literature. We hope that adrenal insufficiency, which becomes fatal if not diagnosed and treated rapidly, will come to mind as easily as chronic renal failure in clinical practice.Familial Mediterranean fever (FMF) is an autoinflammatory inherited autosomal recessive disease observed especially in Jewish, Arabian, Armenian, and Turkish communities [1]. FMF occurs as a result of mutations in the Mediterranean fever (MEFV) gene. The MEFV gene resides on the 16th chromosome. More than 75 mutations associated with FMF have been found. Pyrin (or marenostrin), the protein expressed by the MEFV gene, is basically observed in myelomonocytic cells [2].The development of renal amyloid is observed during early childhood in the vast majority of patients with FMF and is associated with symptoms such as fever, abdominal pain, and inflammation attacks. Those patients are grouped as phenotype I. However, in some cases, renal amyloid is seen in older children (in the 13- to 15-year-old age group) before the emergence of FMF symptoms. These patients stay asymptomatic until the formation of renal amyloidosis and are classified as phenotype II [3].Addison's disease is the primary disease of the adrenal glands and is characterized by defects in the secretion of mineralocorticoid and glucocorticoid hormones. The most common cause of Addison's disease is autoimmune or granulomatous destruction of the adrenal glands [4].FMF is an inherited inflammatory disease characterized by recurre
%U http://www.jmedicalcasereports.com/content/5/1/390