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Deafness-epiphyseal dysplasia-short stature syndrome

MedGen UID:
371330
Concept ID:
C1832438
Disease or Syndrome
Synonyms: Chitty Hall Baraitser syndrome; Deafness, femoral epiphyseal dysplasia, short stature and developmental delay; Growth retardation, deafness, femoral epiphyseal dysplasia, and lacrimal duct obstruction
SNOMED CT: Deafness with epiphyseal dysplasia and short stature syndrome (716238003); Chitty Hall Baraitser syndrome (716238003)
 
Monarch Initiative: MONDO:0011047
OMIM®: 601351
Orphanet: ORPHA3218

Definition

This syndrome has characteristics of sensorineural deafness, short stature, femoral epiphyseal dysplasia, umbilical and inguinal hernias and developmental delay (growth retardation and mild intellectual deficit). It has been described in two brothers born to consanguineous parents. They also have dysmorphic features (triangular face, pointed chin) and bilateral obstruction of lacrimal ducts. This syndrome is transmitted as an autosomal recessive trait. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDeafness-epiphyseal dysplasia-short stature syndrome
Follow this link to review classifications for Deafness-epiphyseal dysplasia-short stature syndrome in Orphanet.

Recent clinical studies

Diagnosis

Walne AJ, Collopy L, Cardoso S, Ellison A, Plagnol V, Albayrak C, Albayrak D, Kilic SS, Patıroglu T, Akar H, Godfrey K, Carter T, Marafie M, Vora A, Sundin M, Vulliamy T, Tummala H, Dokal I
Haematologica 2016 Oct;101(10):1180-1189. Epub 2016 Sep 9 doi: 10.3324/haematol.2016.147769. PMID: 27612988Free PMC Article

Clinical prediction guides

Walne AJ, Collopy L, Cardoso S, Ellison A, Plagnol V, Albayrak C, Albayrak D, Kilic SS, Patıroglu T, Akar H, Godfrey K, Carter T, Marafie M, Vora A, Sundin M, Vulliamy T, Tummala H, Dokal I
Haematologica 2016 Oct;101(10):1180-1189. Epub 2016 Sep 9 doi: 10.3324/haematol.2016.147769. PMID: 27612988Free PMC Article

Supplemental Content

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