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Cornelia de Lange syndrome

Congenital Malformations and Chromosomal Abnormalities

Cornelia de Lange syndrome, abbreviated CdLS, is a genetic disorder in which people experience a range of physical, cognitive and medical challenges, from mild to severe. Its presentation varies widely from person to person. Typical features include thick or long eyebrows, a small nose, small stature, developmental delay, a long or smooth philtrum, a thin upper lip and a downturned mouth. The syndrome is named after the Dutch pediatrician Cornelia Catharina de Lange, who described it in 1933, and it is also called Brachmann de Lange syndrome, Bushy syndrome and Amsterdam dwarfism. Its exact incidence is unknown, but it is estimated at between 1 in 10,000 and 1 in 30,000 people.

Facts
Classification
Body System
Nervous System 1
ICD-10 Chapter
XVII: Congenital Malformations, Deformations and Chromosomal Abnormalities 2
Communicable or Non-communicable
Non-Communicable 3
Causative Agent
Causative Agent (category)
Genetic / Hereditary 3
First Described
1933 3
Sources
1. National Library of Medicine: MeSH Descriptor Data: De Lange Syndrome
National Library of MedicineMeSH tree number C10.597.606.360.210
Quote, MeSH tree number C10.597.606.360.210
A syndrome characterized by growth retardation, severe MENTAL RETARDATION , short stature, a low-pitched growling cry, brachycephaly, low-set ears, webbed neck, carp mouth, depressed nasal bridge, bushy eyebrows meeting
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2. World Health Organization: ICD-10 Version:2019 - Q87.1
World Health OrganizationICD-10 2019 category Q87.1, Chapter XVII
Quote, ICD-10 2019 category Q87.1, Chapter XVII
Q87.1 Congenital malformation syndromes predominantly associated with short stature Incl.: Syndrome: Aarskog Cockayne De Lange
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3. Cornelia de Lange syndrome (Wikipedia)
  • Wikipedia lead
    Cornelia de Lange syndrome (CdLS) is a genetic disorder
  • Wikipedia lead [first-described-year]
    who described it in 1933
  • Lead: disease category
    Cornelia de Lange syndrome (CdLS) is a genetic disorder.
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