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Renal and suprarenal insufficiency secondary to familial Mediterranean fever associated with amyloidosis: a case report

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:...

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Detalles Bibliográficos
Autores principales: Toros, Ahmet Burak, Erdenen, Fusun, Sari, Nagehan Didem, Gokcay, Serkan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3177917/
https://www.ncbi.nlm.nih.gov/pubmed/21851607
http://dx.doi.org/10.1186/1752-1947-5-390
Descripción
Sumario: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.