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Sep 30, 2010 · Familial Mediterranean fever is primarily a genetic disease due to Mendelian-recessive inheritance of Mediterranean fever gene mutations. Occurs mainly in people of Mediterranean ancestry, especially from Arab countries, Turkey, Israel, and Armenia. Characterised by recurrent attacks of fever and...
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Familial Mediterranean fever (FMF; phenotype Mendelian...
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Familial Mediterranean fever (FMF) is a genetic autoinflammatory disorder that causes recurrent fevers and painful inflammation of your abdomen, chest and joints.
FMF is an inherited disorder that usually occurs in people of Mediterranean origin — including those of Jewish, Arab, Armenian, Turkish, North African, Greek or Italian ancestry. But it can affect people in any ethnic group.
Signs and symptoms of familial Mediterranean fever usually begin during childhood. They occur in bouts called attacks that last 1-3 days. Arthritic attacks may last for weeks or months.
Signs and symptoms of FMF attacks vary, but can include:
•Fever
•Abdominal pain
•Chest pain, which can make it hard to breathe deeply
•Painful, swollen joints, usually in the knees, ankles and hips
Familial Mediterranean fever is caused by a gene change (mutation) that's passed from parents to children. The gene change affects the function of an immune system protein called pyrin, causing problems in regulating inflammation in the body.
In people with FMF, change occurs in a gene called MEFV. Many different changes in MEFV are linked to FMF. Some changes may cause very severe cases, while others may result in milder signs and symptoms.
Factors that may increase the risk of familial Mediterranean fever include:
•Family history of the disorder. If you have a family history of FMF, you have a higher risk of the disorder.
Complications can occur if familial Mediterranean fever isn't treated. Inflammation can lead to complications such as:
•Amyloidosis. During attacks of FMF, your body may produce a protein called amyloid A, not typically found in the body. Buildup of this protein causes inflammation, which can cause organ damage.
•Kidney damage. Amyloidosis can damage the kidneys, causing nephrotic syndrome. Nephrotic syndrome occurs when your kidneys' filtering systems (glomeruli) are damaged. People with nephrotic syndrome may lose large amounts of protein in their urine. Nephrotic syndrome can lead to blood clots in your kidneys (renal vein thrombosis) or kidney failure.
•Joint pain. Arthritis is common in people with FMF. The most commonly affected joints are the knees, ankles and hips.
•Infertility. Untreated inflammation caused by FMF may affect the reproductive organs, causing infertility.
•Other complications. These can include inflammation in the heart, lungs, spleen, brain and superficial veins.
Familial Mediterranean fever (FMF) is characterized be episodes of fever, peritonitis and/or pleuritis. is an autosomal-recessive, hereditary auto-inflammatory disease and has a reference in the Online Mendelian Inheritance in Man database (OMIM) ID: 249100.
Familial Mediterranean fever is an inherited autoinflammatory syndrome characterised by recurrent short episodes of high fever associated with abdominal pain, inflammation of joints and other body sites and skin rash. If untreated, amyloidosis commonly develops and may have a fatal outcome.
Familial Mediterranean fever (FMF) is an autoinflammatory disorder characterized by recurrent short episodes of fever and serositis resulting in pain in the abdomen, chest, joints and muscles. ORPHA:342. Classification level: Disorder. Synonym (s): Benign paroxysmal peritonitis. Benign recurrent polyserositis. FMF. Familial paroxysmal polyserositis
PMCID: PMC5297479 PMID: 28197466. Implication for health policy/practice/research/medical education: Mediterranean fever is an autosomal recessive disease. Its features are intermittent attacks of painful inflammation, abdominal pain, fever, and arthritis.
Familial Mediterranean fever is an autosomal recessive disorder characterized by recurrent bouts of fever and peritonitis , sometimes with pleuritis, skin lesions, arthritis, and, rarely, pericarditis. Renal amyloidosis may develop, sometimes leading to renal failure.
