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• W2022986548 abstract "Question: A 57-year-old woman was admitted to our hospital with upper abdominal pain, accompanied by a fever of 38.6°C and panperitonitis. She had experienced the same symptoms of fever and panperitonitis for the previous 2 years. The symptoms would disappear after 2–3 days, but this cycle repeated every few months, and she had frequently been admitted to other hospitals for short durations. The patient had previously undergone surgery for appendicitis, but had no history of oral medication. Laboratory tests on admission revealed a white blood cell count of 17,660/mm3, neutrophil count of 14,834/mm3, and C-reactive protein level of 6.83 mg/dL, indicating a strong inflammatory response. Swelling of the mesenteric lymph nodes and an elevated density of mesenteric fatty tissue were noted on abdominal computed tomography (Figure A). Upper gastrointestinal endoscopy and colonoscopy did not reveal any abnormalities. Capsule endoscopy (CE) revealed hyperemia and erosions with white exudate in the jejunal mucosa (Figure B). Double balloon endoscopy (DBE) revealed hyperemia, edema, fragility, and erosion in the jejunum mucosa (Figure C). What is the diagnosis? Look on page 25 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. The patient had no history of nonsteroidal anti-inflammatory drug use and, although various types of infection (bacterial, viral, and parasitic infections) and autoimmune disease were included in the differential diagnosis, all were rejected. A biopsy of the reddish erosions of the jejunal mucosa was performed by DBE, and neutrophil-dominated inflammatory cell infiltration was found, focused in the lamina propria of the mucous membrane (Figure D). The patient was treated with fasting, transfusions, and intravenous antibiotics. After 3 days, the fever and abdominal pain had dissipated. On the day 14 after admission, CE revealed that the hyperemia and edema in the mucosa had markedly improved. The patient was temporarily discharged before a definitive diagnosis was reached. However, she continued to experience the same symptoms (fever ≥38°C and abdominal pain) every few months, and these would naturally dissipate with only fasting and transfusions after 2–3 days. These findings met the Tel-Hashomer criteria for familial Mediterranean fever (FMF), and because the symptoms did not recur after the initiation of oral colchicine, a definitive diagnosis was obtained. CE, performed after colchicine was started, revealed normal mucosa in small intestine (Figure E). MEFV gene analysis revealed no mutation. FMF is a monogenic, autoinflammatory disease characterized by recurrent fever associated with serositis that lasts <3 days.1Sohar E. Gafni J. Pras M. et al.Familial Mediterranean fever. A survey of 470 cases and review of the literature.Am J Med. 1967; 43: 227-253Abstract Full Text PDF PubMed Scopus (1033) Google Scholar FMF is caused by mutations in the MEVF gene, which is located on the short arm of chromosome 16, although nearly 10% of FMF patients with the MEFV gene are normal.2Padeh S. Livneh A. Pras E. et al.Familial Mediterranean fever in children presenting with attacks of fever alone.J Rheumatol. 2010; 37: 865-869Crossref PubMed Scopus (40) Google Scholar Although only a few endoscopic findings have been reported, colonoscopy revealed hyperemia, fragility, and patchy ulcerations with white exudate in the large intestinal mucosa.3Gurkan O.E. Dalgic B. Gastrointestinal mucosal involvement without amyloidosis in children with familial Mediterranean fever.J Pediatr Gastr Nutr. 2013; 57: 319-323Crossref PubMed Scopus (17) Google Scholar To our knowledge, this case report is the first study to describe observations for the small intestine, which, because they are similar to the findings for the large intestine described previously, indicate that CE and DBE are useful in the diagnosis of FMF." @default.
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• W2022986548 date "2015-01-01" @default.
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• W2022986548 title "Recurrent Abdominal Pain Accompanied by Small Intestinal Lesions" @default.
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• W2022986548 doi "https://doi.org/10.1053/j.gastro.2014.08.006" @default.
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