Hypertriglyceridemia and Megacystis‐Microcolon‐Intestinal Hypoperistalsis Syndrome

Japanese clinical practice guidelines for allied disorders of Hirschsprung's disease, 2017

“…Small bowel transplantation for MMIHS has never been performed in Japan (as of January 2016). Papers on organ transplantation have been reported only from overseas, and most of the cases involved multiple organ transplantations, given that hepatic failure and renal failure were present at the time of transplantation in the majority of cases . Multivisceral transplantation can be performed only overseas, and combined liver/small bowel transplantation is still considered to be difficult to perform in Japan.…”

Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan

“…The authors documented that the hypoperistalsis was largely refractory to pharmacologic treatment and the etiology was unknown, and they subsequently named this condition megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS). Since then, cases of MMIHS have been reported sporadically [3][4][5][6][7][8]. This is the first report of a nationwide survey for pediatric MMIHS in Japan.…”

“…Most of the patients have to be maintained by parenteral nutrition (PN), which often leads to life threatening complications, such as parenteral nutrition-associated liver disorder and catheter-related blood stream infections. Although there have been some case reports of MMIHS [3][4][5][6][7][8], clinical features and outcomes remain unclarified in this intractable condition. We attempted to clarify the clinical features and treatment of MMIHS based on the data collected with the nationwide survey in Japan.…”

Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS): Report of a case with prolonged survival and literature review

“…Hypertriglyceridemia as a result of hepatotoxicity associated with long-term TPN and the lack of enteral stimulation for the production of chylomicrons has been reported [17].…”

“…Neurological deficit [5] No Dysarthria [5] No Miscellaneous Decortication [5] No Hypertriglyceridemia [17] Yes Low serum selenium [5] No…”