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Neurofaciodigitorenal syndrome

MedGen UID:
163212
Concept ID:
C0796088
Disease or Syndrome
Synonym: Freire-Maia Pinheiro Opitz syndrome
SNOMED CT: Neurofaciodigitorenal syndrome (725908007); Freire Maia Pinheiro Opitz syndrome (725908007); NFDR (neurofaciodigitorenal) syndrome (725908007)
 
Monarch Initiative: MONDO:0009740
OMIM®: 256690
Orphanet: ORPHA2673

Definition

A multiple developmental anomalies syndrome with characteristics of neurological abnormalities (including megalencephaly, hypotonia, intellectual disability, abnormal EEG), dysmorphic facial features (high prominent forehead, grooved nasal tip, ptosis, ear anomalies) and acrorenal defects (such as triphalangism, broad halluces, unilateral renal agenesis). Additionally, intrauterine growth restriction, short stature and congenital heart defects may be associated. There have been no further descriptions in the literature since 1997. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNeurofaciodigitorenal syndrome
Follow this link to review classifications for Neurofaciodigitorenal syndrome in Orphanet.

Recent clinical studies

Diagnosis

Mégarbané A
Clin Dysmorphol 2001 Apr;10(2):129-33. doi: 10.1097/00019605-200104000-00010. PMID: 11310993

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