Orphanet: Grange syndrome

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Grange syndrome

Disease definition

Grange syndrome is characterised by stenosis or occlusion of multiple arteries (including the renal, cerebral and abdominal vessels), hypertension, brachysyndactyly, syndactyly, increased bone fragility, and learning difficulties or borderline intellectual deficit. Congenital heart defects were also reported in some cases.


Classification level: Disorder
  • Synonym(s):
    • Grange occlusive arterial syndrome
    • Progressive arterial occlusive disease-hypertension-heart defects-bone fragility-brachysyndactyly syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant or Autosomal recessive 
  • Age of onset: Childhood
  • ICD-10: Q87.8
  • OMIM: 602531
  • UMLS: C1865267
  • MeSH: -
  • GARD: -
  • MedDRA: -
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