Craniometaphyseal dysplasia is a rare skeletal disorder that results from a mutation in the ANKH or GJA1 genes. The condition is characterized by abnormal facial features, impairment of cranial nerves, and malformation of the long bones in the limbs. The skull, the nerves that leave it and the limb bones are therefore all involved. This entry records what the source lead supports and is not medical advice; the inheritance patterns tied to each gene, the age of onset, frequency and management are left for later depth work.
Facts
Classification
ICD-10 ChapterXVII: Congenital Malformations, Deformations and Chromosomal Abnormalities 2 Communicable or Non-communicable Causative Agent
Causative Agent (category) Prevalence Band Sources
1. Craniometaphyseal dysplasia (Wikipedia)
Lead paragraph
results from a mutation in the ANKH or GJA1 genes
Lead paragraph [body-system]
is a rare skeletal disorder
View the Source2. Orphadata (Orphanet): Orphadata cross-references for ORPHA:1522 Craniometaphyseal dysplasia
Orphadata (Orphanet)ICD-10 cross-referenceQuote, ICD-10 cross-reference
"Reference": "Q78.8", "Source": "ICD-10"
View the Source 3. MedlinePlus Genetics (National Library of Medicine): Craniometaphyseal dysplasia - MedlinePlus Genetics
MedlinePlus Genetics (National Library of Medicine)CausesQuote, Causes
Mutations in the ANKH gene cause autosomal dominant craniometaphyseal dysplasia.
View the Source 4. Orphadata (Orphanet): Orphadata: Epidemiology of rare diseases (en_product9_prev)
Orphadata (Orphanet)ORPHA:1522Quote, ORPHA:1522
Point prevalence Class only <1 / 1 000 000
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