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  #1  
Old 09-29-2012
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Arrow Gyrus Daily Questions; Internal Medicine #43

A 23-year-old Turkish female presents to the emergency department for evaluation of acute abdominal pain. She reports that she has had multiple episodes of se-vere abdominal pain since age 15. These episodes have been very severe, once prompting exploratory laparot-omy at age 18 with removal of the appendix, which was histologically benign. She reports that the pain lasts ap-proximately 2 or 3 days and then resolves entirely without intervention. There are no clear triggers for the pain. Past evaluation has included normal upper and lower endos-copy, normal small bowel series, and multiple CT scans that have shown only small amounts of free fluid in the abdominal cavity. In addition, the patient recently devel-oped a migratory arthritis affecting her knees and ankles.
The patient is currently on no medications. Multiple other family members have similar complaints. On physi-cal examination the patient appears in moderate distress, lying very still. Temperature is 39.8°C (103.6°F). Heart rate is 130, and blood pressure is 112/66. She has evidence of a pleural effusion on the right with decreased breath sounds and dullness to percussion of half the lung field.
She has a regular tachycardia without murmurs. Bowel sounds are hypoactive, and there is moderate diffuse ab-dominal tenderness. There is mild rebound tenderness diffusely throughout the abdomen without guarding. Her left knee is swollen and erythematous with an effusion.
Laboratory studies show a white blood cell count of 15,300/mm 3 (90% neutrophils). Erythrocyte sedimenta-tion rate is 110 s. Arthrocentesis reveals a white blood cell count of 68,000 with 98% neutrophils. Culture is negative at 1 week. The patient’s symptoms resolve over the course of 72 h. What is the best therapy for prevention of the pa-tient’s symptoms?

A. Azathioprine
B. Colchicine
C. Hemin
D. Indomethacin
E. Prednisone
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  #2  
Old 09-29-2012
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i think he has Familial Mediterranean fever . About treatment i am with B. Colchicine
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B. Colchicine
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Old 09-29-2012
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The answer is B.Colchicine

This is a classic presentation of familial Mediterraneanfever, an inherited disease most common in Armenians, Arabs, Turks, and non-Ashkenazi Jews.

Febrile episodes begin in early childhood, with more than 90% of patients experiencing the first attack by age 20. Fever is invariably a feature of an acute attack. Other common features include severe serositis presenting most frequently as peritonitis or pleuritis. The pain is often so severe that exploratory laparotomy may be performed to search for a source of peritonitis. CT imaging shows only small amounts of free fluid in the abdomen or pleural space.

On laboratory testing this fluid represents sterile neutrophilia in response to the intense serosal inflammation. Other manifestations of the disease include acute monoarthritis with large sterile, neutrophilic effusions and a rash that resembles erysipelas on the lower extremity. The attacks are self-limited and resolve within 72 h, although the joint symptoms may persist.

Amyloidosis as a result of chronic inflammation is a common manifestation late in the disease. Laboratory studies are nonspecific, showing changes expected with acute inflammation.

Diagnosis usually can be made with clinical criteria alone, although there is gene testing available for the most common mutations that cause the disease.

Treatment is targeted at preventing attacks with colchicine, a drug that inhibits microtubule formation and has been demonstrated to decrease the frequency and intensity of the attacks.

In addition, it can prevent the development of amyloidosis. There are no alternative therapies available, although investigations into the use of interferon and tumor necrosis factor inhibitors are ongoing
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Old 04-10-2013
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Question What about these manifestations with FMF ??

I have found today in the hospital a case diagnosed FMF depending on PCR MEFV gene......but I have also found in this case splenomegaly about 4 cm below costal margin ....also CBC (Microcytic hypochromic anemia) and the patient(female child 9 years old) is pale and looks ill .....and LDH is elevated
....I think splenomegaly from amyloidosis..... but I want to know the cause of microcytic hypochromic anemia ?????
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