家族性地中海热伴淀粉样变性继发肾和肾上腺功能不全：一例报告
Renal and suprarenal insufficiency secondary to familial Mediterranean fever associated with amyloidosis: a case report.
作者信息
Toros Ahmet Burak, Erdenen Fusun, Sari Nagehan Didem, Gokcay Serkan
机构信息
Department of Gastroenterology, Istanbul Education and Research Hospital, Samatya, Fatih, Istanbul, Turkey.
出版信息
J Med Case Rep. 2011 Aug 18;5:390. doi: 10.1186/1752-1947-5-390.
INTRODUCTION
Familial Mediterranean fever is an autosomal recessive disease that predominantly affects people of the Mediterranean coast. One of the most frequent complications of the disease is amyloidosis. This clinical entity is known as secondary (also called AA) amyloidosis.
CASE PRESENTATION
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.
CONCLUSION
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.
引言
家族性地中海热是一种常染色体隐性疾病，主要影响地中海沿岸人群。该疾病最常见的并发症之一是淀粉样变性。这种临床病症被称为继发性（也称为AA型）淀粉样变性。
病例介绍
在本报告中，我们描述了一名33岁患有家族性地中海热和慢性肾功能不全的土耳其男子的病例。他因肾上腺功能不全症状入住我们的诊所。患者三个月后因心脏骤停死亡。
结论
我们的目的是通过报告一例家族性地中海热患者因肾脏和肾上腺淀粉样蛋白积累导致联合功能不全的病例，为文献做出贡献，该病例在文献中很少被描述。我们希望在临床实践中，肾上腺功能不全（如果不迅速诊断和治疗将致命）能像慢性肾衰竭一样容易被想到。
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