A rare cause of refractory ascites in a child: familial Mediterranean fever.
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.
Cakir
Murat
M
Department of Pediatric Gastroenterology, Hepatology and Nutrition, Ege University, Bornova, Izmir, Turkey. muratcak@hotmail.com
Ozgenc
Funda
F
Baran
Masallah
M
Arikan
Cigdem
C
Sezak
Murat
M
Tuncyurek
Muge
M
Ergun
Orkan
O
Yagci
Rasit Vural
RV
D001201:Ascites / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D002648:Child; D003078:Colchicine / Q000627:therapeutic use; D010505:Familial Mediterranean Fever / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D006074:Gout Suppressants / Q000627:therapeutic use; D006801:Humans; D008297:Male; D011860:Radiography, Abdominal; D013902:Radiography, Thoracic; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D006074:Gout Suppressants; D003078:Colchicine