MBchB study guide

Apr 28th, 2015
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HIRSCHSPRUNG'S DISEASE (aganglionic megacolon)DefinitionPresence of large, non-functional distal intestine usually the rectum with variable involvement of the colon and small gutSites-Only the terminal rectum occurs in about 10% of cases-Extension to the sigmoid colon in 65%-More proximal colon in 10%-Entire colon with small bowel involvement in 10-15%RaceMore common in Caucasians, then blacks. Least common among the Asians Whites >BlacksSex-Males are affected X5 more frequently than females in cases in which the diseased segment is of the usual length. -Up to 70-80 % of the involved patients are male.-Females tend to have longer aganglionic segments. A familial association occurs in 5-10% of cases--more frequently when females are affected.IncidenceKNH-12 cases per year 0r 1: 4 500EtiologyCan be broadly classified as Sporadic-up to 80-90% of cases or Familial 15-17%.F

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