Familial Mediterranean Fever
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چکیده
Description Familial Mediterranean fever is an autosomal recessive disorder mostly prevalent in Mediterranean populations. It is characterized by recurrent selflimited episodes of fever, arthritis, serositis, and skin rash, with marked accumulations of polymorphonuclear leukocytes in affected areas during attacks. The most severe complication of the disorder is the development of amyloidosis, leading to nephrotic syndrome and end-stage renal disease. Two disease phenotypes have been identified, whether amyloidosis appears years after the appearance of the other clinical signs (phenotype I), or whether it is the first or the only familial Mediterranean fever presenting sign (phenotype II), which is less frequent. Estimates of the incidence of FMF in specific eastern Mediterranean populations range from 1 in 2000 to 1 in 100, depending on the population studied
منابع مشابه
Familial Mediterranean Fever: Review of Literature and Report of Two Cases
Familial Mediterranean fever, an autosomal recessive disorder, is a member of the periodic fever syndromes, and considered to be the most common cause of recurrent febrile episodes in children. It is important to understand the disorder as familial Mediterranean fever falls on a spectrum of various presentations; the recurrent episodes of familial Mediterranean fever may be so severe that the q...
متن کاملFAMILIAL MEDITERRANEAN FEVER: A STUDY OF 32 CASES
From April 1983 to September 1990, 32 patients with familial mediterranean fever (FMF) were studied. FMF is characterized by short, self-limited, febrile episodes that occur with inflammation of serosal surfaces. Major symptoms include fever and abdominal pain, presenting as acute surgical abdomen. These attacks are associated with considerable morbidity and in some patients lead to unnece...
متن کاملPostoperative Respiratory Distress in a Patient with History of Familial Mediterranean Fever
Postoperative respiratory distress and pulmonary edema can be seen after a wide variety of serious clinical situations, or rare diseases such as familial Mediterranean fever (FMF). FMF is a multisystemic disorder characterized by recurrent bouts of fever and pain due to inflammation of the peritoneum, synovia, or pleura. We report a case with history of FMF who developed postoperative respirato...
متن کاملGenetic Analysis of Southwestern Iranian Patients with Familial Mediterranean Fever
Background: Familial Mediterranean fever (FMF) is an autosomal recessive genetic disorder characterized by recurrent episodes of self-limited fever and serosal tissues inflammation. Methods: To evaluate clinical symptoms and common genetic mutations in southwestern Iranian patients with FMF, 20 unrelated patients were enrolled in this study based on clinical criteria. A panel of 12 common ME...
متن کاملGenotype Pattern of Pediatric Familial Mediterranean Fever in Jordan: A Single Center Experience
Background Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder caused by mutations in the MEFV gene. The disease is especially common among Mediterranean ancestry, mostly Armenian, Turkish, Jewish and Arab populations. We aimed to describe genotype pattern of FMF in the Jordanian children and to compare it with other populations. Materials and Methods A retros...
متن کاملFamilial Mediterranean Fever (FMF): Mysterious Presentations and Challenging Points From Diagnosis to Management in Acute Care Settings; A Literature Review
Introduction: Familial Mediterranean fever (FMF) is an autosomal recessive disease considered to be the most common entity of a rare group of disorders known as auto-inflammatory syndromes which have acute presentations in emergency settings. Methods: A search of Web of Science, Google Scholar, Cochrane, and PubMed databases for articles published before ...
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