Condition

Camurati-Engelmann disease

Diseases of the Musculoskeletal System and Connective Tissue

Camurati-Engelmann disease, abbreviated CED and also called progressive diaphyseal dysplasia, is a very rare autosomal dominant genetic disorder that produces characteristic anomalies of the skeleton. It is a form of dysplasia in which bones become heavily thickened, especially along the shafts of the long bones. The skull bones may also thicken, narrowing the passages that carry nerves and blood vessels and possibly leading to sensory deficits, blindness or deafness. It often appears in childhood and is considered inherited, although many patients have no family history. It progresses slowly and has no cure, though there is treatment. It is named for M. Camurati and G. Engelmann. This record is not medical advice.

Facts
Classification
Body System
Skeletal System 1
ICD-10 Chapter
XVII: Congenital Malformations, Deformations and Chromosomal Abnormalities 2
Communicable or Non-communicable
Non-Communicable 3
Transmission Route
Non-Transmissible 3
Causative Agent
Causative Agent (category)
Genetic / Hereditary 1
Prevalence Band
Rare (Under 1 in 2000) 4
Sources
1. Camurati-Engelmann disease (Wikipedia)
  • Opening sentence
    very rare autosomal dominant genetic disorder
  • Opening sentence [body-system]
    anomalies in the skeleton
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2. Monarch Initiative: Camurati-Engelmann disease - Monarch Initiative MONDO:0007542
Monarch InitiativeCross reference: ICD10CM
Quote, Cross reference: ICD10CM
ICD10CM:Q78.3
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3. MedlinePlus Genetics (National Library of Medicine): Camurati-Engelmann disease - MedlinePlus Genetics
MedlinePlus Genetics (National Library of Medicine)
  • Causes
    Mutations in the TGFB1 gene cause Camurati-Engelmann disease.
  • Description and Inheritance
    Mutations in the TGFB1 gene cause Camurati-Engelmann disease.
View the Source
4. Orphadata (Orphanet): Orphadata: Epidemiology of rare diseases (en_product9_prev)
Orphadata (Orphanet)ORPHA:1328
Quote, ORPHA:1328
Point prevalence Class only <1 / 1 000 000
View the Source
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