Langer Giedion syndrome

Langer-Giedion syndrome (LGS), also known as trichorhinophanageal syndrome type II, is an extremely rare autosomal dominant genetic disorder.

Pathology

The effects are seen mainly on the skeletal system and primarily involves

  • hair: tricho
  • nose: rhino
  • digits of the hands and feet: phalangeal

There can be however multiple of other features such as:

  • multiple exostoses
  • mental retardation
  • lose redundant skin
Genetics

It occurs from a deletion within chromosome 8.

History and etymology

Named after 2,3:

  • Andreas Giedion, Swiss radiologist
  • Leonard O Langer JrAmerican (US) radiologist 
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Article Information

rID: 10592
Section: Syndromes
Synonyms or Alternate Spellings:
  • Trichorhinophanageal syndrome type II

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