Jewish American Heritage Month

Posted By admin on February 12, 2021

Dr. Jeff Hersh| Daily News Correspondent

Q: My neighbors son was diagnosed with Mediterranean fever even though he has never left the country. Is this contagious?

A: The "full" name for this condition is Familial Mediterranean Fever (FMF), and it is an inherited condition which is not contagious.FMF has an autosomal recessive inheritance, so both parents must be carriers for a patient to have the disease. There is variable penetrance of this disease, so even having two copies of the gene does not always mean the patient will be affected. The Mediterranean part of the name is because of the increased incidence of this familial condition in people with Turkish, Armenian, Sephardic Jewish, Arab, North African and/or other Mediterranean descent, with up to 1 in 500 people or more with this direct heritage affected.The number of people with a more mixed heritage that is affected is much lower. For example, for Ashkenazi Jews with a European heritage (the heritage of most American and European Jews) only about 1 in every 75,000 is affected.

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FMF is an episodic condition, with recurrent bouts of fever and serositis (inflammation of the tissue membrane that lines one or more of the bodys organs), for example the lining of the heart (pericarditis), lungs (pleuritis), abdomen (peritonitis), a single joint (mono-arthritis), or others.

The first flareup of symptoms in patients with FMF is before the age of 10 in two-thirds of patients and before the age of 20 in over 90 percent. The remaining 10 percent of cases manifest in adulthood, and rarely even as late as age 50 or older.

The symptoms of FMF include fever in over 90 percent of bouts (hence the name). Pain/other complications from the serositis in the abdomen, chest, joint(s), muscles, genitals, head or other locations, and sometimes certain skin lesions, occur in most episodes, although episodes of just fever with no other symptoms can occur.

There is no specific test for FMF during an acute bout so the diagnosis is made based on the history and physical exam, although general blood tests to look for inflammation can be helpful.However, because the symptoms may mimic many other conditions (for example fever and abdominal pain may mimic appendicitis), other tests are often done. Genetic testing for FMF is now possible to confirm the diagnosis.

During an acute FMF attack treatment is aimed at relieving symptoms. Colchicine (a medication best known as a treatment for gout since it helps reduce inflammation and the buildup of uric acid crystals) is the first line treatment to prevent attacks and reduce inflammation (and hence the development of complications). The roughly 5 percent of patients who do not respond to colchicine treatment, and the additional 2 to 5 percent who have severe side effects (usually gastrointestinal symptoms) may be treated with other anti-inflammatory medications such as interleukin-1inhibitors or others.

Complications from FMF may include scarring from the repeated bouts of inflammation (for example leading to adhesions in the abdomen which can cause small bowel obstructions), amyloid build up (possibly leading to amyloidosis), infertility in women (from scarring and adhesions), and/or certain organ dysfunction (such as kidney compromise).

A "typical" attack of FMF includes a serositis and fever for a duration of 12 hours to 3 days in a patient who has had similar bouts 3 or more times in the past.Some patients may have several types of typical attacks, for example sometimes having peritonitis and sometimes having pleuritis or other serositis flare-up. A typical attack is a major diagnostic criterium for FMF (the diagnosis may be made based on this alone), whereas non-typical attacks (for example lasting less than 6 hours or more than four days) are considered a minor criteria; two minor criteria are required for the clinical diagnosis. Other criteria that support the diagnosis include a family history of FMF, severe symptoms (especially if symptom-free between attacks), overall responsiveness to colchicine treatment (such as decreased frequency and/or severity of attacks) and/or other symptoms (for example episodes of blood or protein in the urine).

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FMF patients typically have multiple attacks at an early age, and usually can identify a family history of this condition. Although there is presently no cure for FMF, patients who have anti-inflammatory colchicine started before they develop certain complications (for example protein in their urine or amyloidosis complications) and respond to this therapy (as most patients do) have an overall normal life expectancy.It is therefore very important for the diagnosis to be made as early as possible.

Jeff Hersh, Ph.D., M.D., can be reached at DrHersh@juno.com

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What's Up Doc? Mediterranean fever mimics many other conditions - MetroWest Daily News

Category: Sephardic
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