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Weill-Marchesani syndrome

Congenital Malformations and Chromosomal Abnormalities

Weill-Marchesani syndrome is a rare genetic disorder characterised by short stature, an unusually short and broad head, other facial differences, short fingers and distinctive eye abnormalities. The eyes typically have unusually small, round lenses that may be prone to dislocating, and affected people can have varying degrees of visual impairment, from nearsightedness to blindness. It may be inherited in an autosomal recessive pattern involving the ADAMTS10 gene or in an autosomal dominant pattern involving the FBN1 gene, and in some cases neither gene is involved. It is named after the ophthalmologists Georges Weill and Oswald Marchesani, who first described it in 1932 and 1939 respectively. This entry is a reference record and not medical advice.

Facts
Classification
Body System
Eye 1
ICD-10 Chapter
XVII: Congenital Malformations, Deformations and Chromosomal Abnormalities 2
Communicable or Non-communicable
Non-Communicable 3
Transmission Route
Non-Transmissible 4
First Described
1932 1
Prevalence Band
Rare (Under 1 in 2000) 5
Causative Agent
Causative Agent (category)
Genetic / Hereditary 1
Sources
1. Weill-Marchesani syndrome (Wikipedia)
  • Lead section
    first described it in 1932 and 1939, respectively
  • Lead section [causative-agent-category]
    is a rare genetic disorder
  • Lead section [body-system]
    distinctive eye (ocular) abnormalities
View the Source
2. Orphadata (Orphanet): Orphadata cross-references for ORPHA:3449 Weill-Marchesani syndrome
Orphadata (Orphanet)ICD-10 cross-reference
Quote, ICD-10 cross-reference
"Reference": "Q87.0", "Source": "ICD-10"
View the Source
3. National Cancer Institute (NCI Thesaurus): Weill-Marchesani syndrome - NCI Thesaurus concept C85226
National Cancer Institute (NCI Thesaurus)Definition
Quote, Definition
A rare, autosomal recessive or dominant inherited connective tissue disorder.
View the Source
4. MedlinePlus Genetics (U.S. National Library of Medicine): Weill-Marchesani syndrome - MedlinePlus Genetics
MedlinePlus Genetics (U.S. National Library of Medicine)Description and Inheritance
Quote, Description and Inheritance
Mutations in the ADAMTS10 and FBN1 genes can cause Weill-Marchesani syndrome.
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5. Orphadata (Orphanet): Orphadata: Epidemiology of rare diseases (en_product9_prev)
Orphadata (Orphanet)ORPHA:3449
Quote, ORPHA:3449
Point prevalence Value and class 1-9 / 100 000
View the Source
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