Cardiac rhabdomyomata and megacystis-microcolon-intestinal hypoperistalsis syndrome.

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Cardiac rhabdomyomata and megacystis-microcolon-intestinal hypoperistalsis syndrome.

Multiple cardiac rhabdomyomata were discovered on necropsy tissue review of a previously well child with megacystis-microcolon-intestinal hypoperistalsis syndrome, who died unexpectedly at home at 40 months of age. Multiple cardiac rhabdomyomata occur rarely and have not previously been reported with this syndrome. They are most frequently associated with tuberous sclerosis. The finding of mult...

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Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome

We report a case of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) in a newborn female infant who presented with an abdominal mass, absent bowel sounds, and feeding intolerance with bilious emesis. MMIHS is a rare congenital bowel and bladder defect requiring surgery and chronic total parenteral nutrition in an attempt to sustain life. With few exceptions, it is predominately...

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Megacystis microcolon intestinal hypoperistalsis syndrome.

Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare and the most severe form of functional intestinal obstruction in the newborn. The major features of this congenital and usually lethal anomaly are abdominal distension, bile-stained vomiting, and absent or decreased bowel peristalsis. Abdominal distension is a consequence of the distended, unobstructed urinary bladder w...

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Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome (MMIHS).

Introduction Megacystismicrocolon -intestinal hypoperistalsis syndrome(MMIHS) also called as BERDON SYNDROME is a rare congenital disease characterized by massive abdominal distension caused by a largely dilated non-obstructed urinary bladder, microcolon and decreased or absent intestinal peristalsis. It poses a diagnostic and therapeutic challenge to the surgeon. It the most severe form of fun...

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Intrauterine death in megacystis-microcolon-intestinal hypoperistalsis syndrome.

Megacystis-microcolon-intestinal hypoperistalsis syndrome is an uncommon condition, possibly inherited as an autosomal recessive trait. This report describes an affected sib pair with intrauterine death of one of the sibs.

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ژورنال

عنوان ژورنال: Journal of Medical Genetics

سال: 1991

ISSN: 1468-6244

DOI: 10.1136/jmg.28.4.274