Definition:
Familial paroxysmal peritonitis or also known as Familial Mediterranean fever is an inherited inflammatory disorder usually occurring among people with Mediterranean origin. A rare disorder, it is typically diagnosed during childhood.
Diagnosis:
There is no specific test available to detect the condition. Once other diseases are ruled out, doctors make a diagnosis based on the signs and symptoms, family history, ethnicity, blood tests, and genetic testing.
Treatment:
There is no cure for Familial paroxysmal peritonitis. A drugs called colchicine however, has been proven to reduce inflammation of the body. Before said drug was developed, patients were required to undergo a dialysis or kidney transplantation. The former is done to remove wastes from the blood. Nowadays the two procedures are only recommended to those who do not adhere to therapy or cannot tolerate colchicines.
Symptoms and Signs:
Signs and symptoms of the disease usually occur among people younger than 20 years old. Collectively called attacks, the signs and symptoms last for 2-3 days with the first 12 hours as peaks. Among the typical attacks are sudden fever, abdominal pain, chest pain, achy, swollen joints, constipation followed by diarrhea, and a rash on the legs particularly the area below the knees.
There are also less common signs symptoms including muscle aches, inflammation of the female reproductive organs, swollen, tender scrotum, and inflammation of the blood vessels.
Causes:
A gene defect on chromosome 16 causes the condition. However, researchers are not certain as to how said defect causes the disease. It may be because people with a mutation in this gene don't make protein that inhibits the inflammatory response in certain parts of the body.
Familial paroxysmal peritonitis is also inherited in an autosomal recessive trait.
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familialparoxysmalperitonitis
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