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Familial visceral myopathy

ORPHA:2604· ICD-10 K56.0

Definition

Familial visceral myopathy is a rare hereditary myopathic degeneration of both gastrointestinal and urinary tracts that causes chronic intestinal pseudo-obstruction. It usually presents after the first decade of life with megaduodenum, megacystis and symptoms such as abdominal distension and/or pain, vomiting, constipation, diarrhea, dysphagia, and/or urinary tract infections.

Prevalence
Unknown
Inheritance
Autosomal dominant
Age of onset
All ages