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Megacystis-microcolon-intestinal hypoperistalsis syndrome.

作者信息

Kupferman J C, Stewart C L, Schapfel D M, Kaskel F J, Fine R N

机构信息

Department of Pediatrics, School of Medicine, State University of New York at Stony Brook 11794-8111, USA.

出版信息

Pediatr Nephrol. 1995 Oct;9(5):626-7. doi: 10.1007/BF00860960.

Abstract

The megacystis-microcolon-intestinal hypoperistalsis syndrome is a congenital disorder characterized by urinary bladder distension and hypoperistalsis throughout the entire gastrointestinal tract. We present a new case with the typical clinical, radiological, and pathological findings of the syndrome. The diagnosis should be suspected in a patient who present clinically with intestinal obstruction and urinary retention, and confirmed with imaging studies, including abdominal plain films, urinary tract ultrasonography, and contrast studies of the colon and the bladder. The prognosis is generally very poor. Our patient died secondary to sepsis on day 5 of life.

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