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Kenny-Caffey syndrome

Congenital Malformations and Chromosomal Abnormalities

Kenny-Caffey syndrome type 2 is an extremely rare autosomal dominant genetic condition characterised by dwarfism, farsightedness, small eyes and skeletal abnormalities. It is caused by a heterozygous mutation in the FAM111A gene on chromosome 11. Fewer than sixty confirmed cases have been reported, males and females are affected equally, and it is most often sporadic, although an affected mother can pass it to her children. Frederic Kenny and Louis Linarelli described it in 1966 in a mother and her son, and the pediatric radiologist John Caffey described the radiographic findings of the same family in 1967. A recessive type 1, which usually includes intellectual disability, is also known as Sanjad-Sakati syndrome. This entry is a reference record and 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 1
First Described
1966 1
Prevalence Band
Rare (Under 1 in 2000) 4
Causative Agent
Causative Agent (category)
Genetic / Hereditary 1
Sources
1. Kenny-Caffey syndrome (Wikipedia)
  • Lead section
    originally described by Frederic Kenny and Louis Linarelli in 1966
  • Lead section [causative-agent-category]
    an extremely rare autosomal dominant genetic condition
  • Lead section [body-system]
    skeletal abnormalities
  • Lead
    Kenny-Caffey syndrome type 2 (KCS2) is an extremely rare autosomal dominant genetic condition characterized by dwarfism, farsightedness, microphthalmia, and skeletal abnormalities.
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2. Orphadata (Orphanet): Orphadata cross-references for ORPHA:2333 Kenny-Caffey syndrome
Orphadata (Orphanet)ICD-10 cross-reference
Quote, ICD-10 cross-reference
"Reference": "Q87.1", "Source": "ICD-10"
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3. National Cancer Institute (NCI Thesaurus): Kenny-Caffey syndrome - NCI Thesaurus concept C130991
National Cancer Institute (NCI Thesaurus)Definition
Quote, Definition
A genetic condition characterized by long bone sclerosis and thickening, short stature, and head and eye anomalies.
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4. Orphadata (Orphanet): Orphadata: Epidemiology of rare diseases (en_product9_prev)
Orphadata (Orphanet)ORPHA:2333
Quote, ORPHA:2333
Point prevalence Class only <1 / 1 000 000
View the Source
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