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Congenital labioscrotal agenesis-cerebellar malformation-corneal dystrophy-facial dysmorphism syndrome

MedGen UID:
1798878
Concept ID:
C5567455
Disease or Syndrome
Synonyms: Congenital agenesis of labia majora or scrotum-cerebellar malformation-corneal dystrophy-facial dysmorphism syndrome; congenital agenesis of labia majora or scrotum-cerebellar malformation-corneal dystrophy-facial dysmorphism syndrome; Congenital labioscrotal agenesis, cerebellar malformation, corneal dystrophy, facial dysmorphism syndrome; congenital labioscrotal agenesis-cerebellar malformation-corneal dystrophy-facial dysmorphism syndrome
SNOMED CT: Congenital labioscrotal agenesis, cerebellar malformation, corneal dystrophy, facial dysmorphism syndrome (1172594000)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
 
Monarch Initiative: MONDO:0044643
Orphanet: ORPHA495875

Definition

A rare genetic multiple congenital anomalies/dysmorphic syndrome with characteristics of global developmental delay, intellectual disability, absent scrotum or labia majora, absent or underdeveloped nipples and a tuft of hair extruding from the lactiferous ducts, bilateral corneal opacities, and dysmorphic craniofacial features (microcephaly, short forehead, and ear abnormalities, among others). Patients also show horizontal nystagmus and ataxic gait. Brain MRI reveals small cerebellar hemispheres and vermis and a small pons. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital labioscrotal agenesis-cerebellar malformation-corneal dystrophy-facial dysmorphism syndrome

Recent clinical studies

Etiology

Miranda ML, Oliveira-Filho AG, Lemos-Marini SH, Guerra G Jr, Bustorff-Silva JM
J Pediatr Surg 2004 Jul;39(7):1030-3. doi: 10.1016/j.jpedsurg.2004.03.060. PMID: 15213892

Diagnosis

Corona-Rivera JR, Acosta-León J, León-Hernández MÁ, Martínez-Macías FJ, Bobadilla-Morales L, Corona-Rivera A
Am J Med Genet A 2014 Jan;164A(1):199-203. Epub 2013 Nov 15 doi: 10.1002/ajmg.a.36210. PMID: 24243754
Coslovsky R, Ashkenazy M, Lancet M, Barash A, Borenstein R
J Endocrinol Invest 1985 Feb;8(1):63-5. doi: 10.1007/BF03350643. PMID: 3989237

Prognosis

Miranda ML, Oliveira-Filho AG, Lemos-Marini SH, Guerra G Jr, Bustorff-Silva JM
J Pediatr Surg 2004 Jul;39(7):1030-3. doi: 10.1016/j.jpedsurg.2004.03.060. PMID: 15213892

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