We report a 21-year-old female patient who suddenly died of circulatory failure due to pressure from megacolon allied with pseudo-Hirschsprung's disease. Since 3 years before her death, she had exhibited the feeling of abdominal distention, which was diagnosed as constipation. However, her constipation did not respond well to the prescribed oral administration of laxatives and enemas. She was found dead at home in the morning, lying on her back. An autopsy revealed a decreased number of ganglion cells in the rectum, suggesting hypoganglionosis. In cases of intractable chronic constipation, careful investigation of the cause of such symptoms is important.
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http://dx.doi.org/10.1016/j.legalmed.2014.11.005 | DOI Listing |
BMC Res Notes
March 2016
Division of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan.
Background: Advances in long-term parenteral nutrition via indwelling central venous catheter have improved the quality of life and mortality in patients with life-threatening gastrointestinal diseases complicated with severely impaired absorption. However, infection to central venous catheter is still a common and critical complication for such patients. We encountered two patients under long-term parenteral nutrition who developed glomerulonephritis associated with central venous catheter infection.
View Article and Find Full Text PDFLeg Med (Tokyo)
May 2015
Department of Neurology, Aomori Prefectural Central Hospital, Aomori City, Aomori, Japan; Department of Neurophysiology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, Hirosaki City, Aomori, Japan. Electronic address:
We report a 21-year-old female patient who suddenly died of circulatory failure due to pressure from megacolon allied with pseudo-Hirschsprung's disease. Since 3 years before her death, she had exhibited the feeling of abdominal distention, which was diagnosed as constipation. However, her constipation did not respond well to the prescribed oral administration of laxatives and enemas.
View Article and Find Full Text PDFIndian J Pediatr
August 2014
Department of Pediatrics, Fernandez Hospital, Opposite Old MLA quarters, Hyderabad, 500029, Andhra Pradesh, India.
Pediatr Surg Int
December 2012
Department of Paediatric Surgery, Frere Hospital, East London, South Africa.
African degenerative leiomyopathy (ADL, DL, Bantu pseudo-Hirschsprung's disease) is a distinctive visceral myopathy, of unknown etiology, occurring in Africa. It has a classical clinical and histologic picture in young indigenous African children. It presents as intestinal pseudo-obstruction with a massive megacolon due to degeneration of smooth muscle without aganglionosis.
View Article and Find Full Text PDFIntern Med
March 2008
Department of Internal Medicine, Saiseikai Utsunomiya Hospital.
We report an autopsied 20-year-old man case of intestinal necrosis associated with megacolon from hypoganglionosis, a pseudo-Hirschsprung's disease. The patient had suffered from severe constipation since two years of age, and presented abdominal distention from age ten. Autopsy revealed marked dilatation and necrosis of the entire large intestine.
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