MMIH syndrome
MIM.249210 15q24
megacystis-microcolon syndrome, megacystis-microcolon-intestinal hypoperistalsis syndrome
Autosomal recessive disorder
Synopsis
megacystis (dilatation of the bladder)
- +/- prune belly sequence (MIM.100100)
- +/- bilateral megaureteres
- +/- hydronephrosis
- +/- renal dysplasia
+/- oligohydramnios sequence
+/- polyhydramnios
microcolon
microileum or dilated small intestines
digestive malrotation
intestinal hypoperistalsis
short bowel
ganglion cells of the myenteric plexus are normal
Epidemiology
most affected children die during first year of life
skewed sex ratio - 31 females-to-11 males
more severe disorder in males
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