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[Familial Mediterranean fever].

F Schindera, R Löw, K H Langer

    Monatsschrift Kinderheilkunde : Organ Der Deutschen Gesellschaft Fur Kinderheilkunde
    |November 1, 1984
    PubMed
    Summary

    Familial Mediterranean Fever (FMF) is a genetic disorder. Early diagnosis in individuals from Mediterranean regions is crucial for managing symptoms like fever and abdominal pain, and preventing complications such as amyloidosis.

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    Area of Science:

    • Genetics
    • Internal Medicine
    • Pediatrics

    Background:

    • Familial Mediterranean Fever (FMF) is an inherited autoinflammatory disorder.
    • It primarily affects individuals of Mediterranean descent.
    • FMF is characterized by recurrent episodes of fever and inflammation.

    Observation:

    • A Turkish family presented with a 10-year history of undiagnosed illness.
    • Affected siblings exhibited symptoms including recurrent fever, abdominal pain, and arthralgias.
    • One sibling also developed nephrotic syndrome secondary to amyloidosis.

    Findings:

    • The clinical presentation in the siblings was consistent with Familial Mediterranean Fever.
    • The development of amyloidosis highlights a severe complication of untreated FMF.
    • The diagnosis of FMF should be considered in individuals from the Mediterranean area presenting with these symptoms.

    Implications:

    • Timely diagnosis of FMF is essential to initiate appropriate management.
    • Early intervention can prevent the progression of systemic complications like amyloidosis.
    • Genetic counseling and awareness are important for families with a history of FMF.

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