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The Hunger Project Bolen Report
Ohm Society
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de Lange Syndrome Print E-mail

Dr. Kennedy A relatively common birth defect syndrome with multiple malformations and mental retardation of unknown origin. de Lange syndrome is recognized by the presence of:

  • Prenatal and postnatal growth retardation
  • Delayed development and mental retardation
  • Abnormally small head (microcephaly)
  • Hair low on the nape of the neck
  • Characteristic facial appearance with
  • Low-set ears,
  • Hair well down onto the forehead
  • Bushy eyebrows
  • Eyebrows that meet in the middle (synophrys)
  • Unusually long eyelashes
  • Depressed bridge of the nose
  • Uptilted tip of the nose
  • Forward-directed nostrils
  • Protuberance of the upper jaw (maxillary prognathism)
  • "Carp-shaped" mouth
  • Small, widely spaced teeth
  • Upper limb anomalies with flat spadelike hands with a "simian" (single transverse) palmar crease and short tapering fingers

    The majority of children with de Lange syndrome have no known family history of the disorder. There are, however, some reports of familial cases. These reports suggest autosomal dominant transmission with a mildly affected parent having a more seriously affected child. The chance is from 2 to 5% that a child whose sib has de Lange syndrome will also have the syndrome. In familial de Lange syndrome, a gene on chromosome 5 is mutated.



The information in this article is not meant to be medical advice.�Treatment for a medical condition should come at the recommendation of your personal physician.

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