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Schwartz-Jampel syndrome

Congenital Malformations and Chromosomal Abnormalities

Schwartz-Jampel syndrome, abbreviated SJS and also known as chondrodystrophic myotonia, is a rare genetic disease. It is caused by a mutation in the perlecan gene, HSPG2, and produces osteochondrodysplasia, a disorder of bone and cartilage growth, associated with myotonia, which is delayed relaxation of muscle after contraction. Most people with the syndrome have a nearly normal life expectancy. The short source lead supports only these points, so the account here is intentionally brief.

Facts
Classification
Body System
Skeletal System 1
Body System
Muscular System 1
ICD-10 Chapter
VI: Diseases of the Nervous System 2
Communicable or Non-communicable
Non-Communicable 3
Transmission Route
Non-Transmissible 4
Causative Agent
Causative Agent (category)
Genetic / Hereditary 1
Prevalence Band
Rare (Under 1 in 2000) 5
Sources
1. Schwartz-Jampel syndrome (Wikipedia)
  • Lead paragraph, first sentence
    caused by a mutation in the perlecan gene (HSPG2)
  • Lead paragraph, first sentence [body-system]
    osteochondrodysplasia associated with myotonia
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2. Monarch Initiative: Schwartz-Jampel syndrome - Monarch Initiative MONDO:0009717
Monarch InitiativeCross reference: ICD10CM
Quote, Cross reference: ICD10CM
ICD10CM:G71.13
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3. National Cancer Institute (NCI Thesaurus): Schwartz-Jampel syndrome - NCI Thesaurus concept C35008
National Cancer Institute (NCI Thesaurus)Definition
Quote, Definition
A classification for rare genetic syndromes with an autosomal recessive pattern of inheritance.
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4. MedlinePlus Genetics (U.S. National Library of Medicine): Schwartz-Jampel syndrome - MedlinePlus Genetics
MedlinePlus Genetics (U.S. National Library of Medicine)Description and Inheritance
Quote, Description and Inheritance
This condition is inherited in an autosomal recessive pattern , which means both copies of the gene in each cell have mutations.
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5. Orphadata (Orphanet): Orphadata epidemiology, ORPHAcode 800
Orphadata (Orphanet)Orphanet epidemiology, ORPHAcode 800
Quote, Orphanet epidemiology, ORPHAcode 800
"PrevalenceClass": "\u003c1 / 1 000 000", "PrevalenceGeographic": "Worldwide", "PrevalenceQualification": "Class only", "PrevalenceType": "Point prevalence"
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