BACKGROUND: @entity23454 ( @entity23454 ) is a rare condition with a poor outcome. A nationwide survey was conducted to identify the clinical features and outcomes of @entity23454 in Japan. METHODS: Data were collected via a questionnaire, which was sent to 353 pediatric/pediatric surgical departments in Japan. RESULTS: Of the 28 @entity1 registered as having a certain diagnosis of @entity23454 , 19 (male/female, 3/16) @entity1 were analyzed. All of the @entity1 developed functional @entity1744 in the neonatal period and showed a distended bladder and microcolon in the radiological examination. A histopathology assessment of the full-thickness of intestinal specimens showed no pathological abnormalities in all @entity1 . Although various medications were given, the @entity1 did not show significant improvement. Drainage stomas were created in the jejunum (n=11) and colon (n=5). Sixteen @entity1 were maintained by @entity3147 ( @entity3147 ). Nine @entity1 died of sepsis or @entity1578 . The five- and ten-year survival rates were 63% and 57%, respectively. CONCLUSIONS: @entity23454 @entity1 developed severe functional @entity1321 in the neonatal period and had no specific therapeutic intervention. The majority of @entity23454 @entity1 required long-term @entity3147 . Small bowel or multivisceral transplantation may be necessary to improve the outcome of this condition.

XXXX : A report of a nationwide survey in Japan.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity23454 :: ('MESH:C536138', 'Disease') :: ['Megacystis microcolon intestinal hypoperistalsis syndrome', 'MMIHS'] @entity1578 :: ('MESH:D017093', 'Disease') :: ['liver failure'] @entity1321 :: ('MESH:D007415', 'Disease') :: ['intestinal obstruction'] @entity3147 :: ('MESH:D044342', 'Disease') :: ['parenteral nutrition', 'PN'] @entity1744 :: ('MESH:D043183', 'Disease') :: ['bowel obstruction']
@entity23454 :: (MESH:C536138,Disease) :: ['Megacystis microcolon intestinal hypoperistalsis syndrome']
