A rare cause of refractory ascites in a child: familial Mediterranean fever |
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Authors: | Murat Cakir Funda Ozgenc Masallah Baran Cigdem Arikan Murat Sezak Muge Tuncyurek Orkan Ergun Rasit Vural Yagci |
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Institution: | (1) Department of Pediatric Gastroenterology, Hepatology and Nutrition, Ege University, Bornova, Izmir, Turkey;(2) Department of Pathology, Ege University, Izmir, Turkey;(3) Department of Pediatric Surgery, Ege University, Izmir, Turkey |
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Abstract: | Familial Mediterranean fever (FMF) is hereditary episodic febrile syndrome characterized by acute attacks of fever and serosal
inflammation, generally lasting 1–3 days and resolves spontaneously. Apart from abdominal pain, patients may present with
variety of abdominal manifestations such as acute peritonitis, mechanical intestinal obstruction, diarrhea, bowel infarction,
amyloidosis and small amounts of peritoneal fluid during the acute attacks. A 6-year-old boy was admitted with massive ascites.
After extensive laboratory investigations, no causative agent could be identified. On subsequent days, he developed fever
and skin eruptions. Acute-phase reactants were increased. A second tomography revealed cystic fluid collection near the anterior
side of spleen that invades the stomach. An exploratory was performed and histopathological examination of the all resected
specimens revealed mix inflammatory cell infiltrate associated with severe myofibroblast proliferation suggesting chronic
inflammatory process on the mesenteric region. A diagnosis of FMF was suspected based on the clinical, laboratory and histopathological
findings, and a trial of colchicine therapy initiated. Ascites and other serosal inflammations improved within 1 week without
any recurrence during the next 12-month period on colchicine treatment. Atypical presentations of FMF have been increasingly
reported. Pediatricians should keep FMF in mind in the differential diagnosis of massive ascites especially in regions where
hereditary inflammatory disease are common. |
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