Bloom syndrome is a rare autosomal recessive genetic disorder marked by short stature, a predisposition to cancer and genomic instability. It is caused by mutations in the BLM gene, a member of the RecQ DNA helicase family; mutations in two other family members, WRN and RECQL4, are linked to Werner syndrome and Rothmund-Thomson syndrome. Cells from a person with Bloom syndrome show striking genomic instability, including excessive crossovers between homologous chromosomes and sister chromatid exchanges. The New York dermatologist Dr. David Bloom discovered and first described the condition in 1954, and older literature sometimes calls it Bloom-Torre-Machacek syndrome. It belongs to a wider class of disorders characterized by chromosomal or genomic instability together with cancer predisposition.
Facts
Classification
ICD-10 ChapterXVII: Congenital Malformations, Deformations and Chromosomal Abnormalities 1 Communicable or Non-communicable First Described Prevalence Band Causative Agent
Causative Agent (category) Sources
1. World Health Organization ICD-10: ICD-10 Version:2019 - Q80-Q89
World Health Organization ICD-10Category Q82.8Quote, Category Q82.8
Q82.8 Other specified congenital malformations of skin
View the Source 2. Bloom syndrome (Wikipedia)
Wikipedia lead
rare autosomal recessive genetic disorder
Wikipedia lead [first-described-year]
discovered and first described the condition in 1954
Lead: disease category
Bloom syndrome (often abbreviated as BS in literature) is a rare autosomal recessive genetic disorder characterized by short stature, predisposition to the development of cancer, and genomic instability.
View the Source3. Orphadata (Orphanet): Orphadata epidemiology, ORPHAcode 125
Orphadata (Orphanet)Orphanet epidemiology, ORPHAcode 125Quote, Orphanet epidemiology, ORPHAcode 125
"PrevalenceClass": "\u003c1 / 1 000 000", "PrevalenceGeographic": "Worldwide", "PrevalenceQualification": "Class only", "PrevalenceType": "Point prevalence"
View the Source National Library of Medicine: MeSH Descriptor Data: Bloom Syndrome
National Library of MedicineMeSH tree number C20.673.795.313Quote, MeSH tree number C20.673.795.313
An autosomal recessive disorder characterized by telangiectatic ERYTHEMA of the face, photosensitivity, DWARFISM and other abnormalities, and a predisposition toward developing cancer.
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