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Acrofrontofacionasal dysostosis

MedGen UID:
349729
Concept ID:
C1860118
Disease or Syndrome
Synonyms: Acrofrontofacionasal dysostosis syndrome; Richieri Costa Colletto syndrome
SNOMED CT: Acrofrontofacionasal dysostosis (720408003); Acro-fronto-facio-nasal dysostosis (720408003); Richieri Costa Colletto syndrome (720408003)
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:0008715
Orphanet: ORPHA1784

Definition

A very rare congenital malformation syndrome with the association of facial and skeletal anomalies, severe intellectual deficit and occasional genitourinary anomalies. The cranio-facial malformations are numerous and variable and include brachycephaly or microbrachycephaly. Other skeletal malformations are also present, with syndactyly of fingers, hypoplastic toes, anomalies of feet structure and fibular hypoplasia. Short stature may be observed. Eye anomalies include bilateral ptosis, cataract and congenital glaucoma. In some male patients, hypospadias and bifid scrotum are reported. Patients suffer from potentially severe intellectual deficit and present with anomalies of the cortical gyration. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
Follow this link to review classifications for Acrofrontofacionasal dysostosis in Orphanet.

Professional guidelines

PubMed

Abdolrahimzadeh S, Fameli V, Mollo R, Contestabile MT, Perdicchi A, Recupero SM
Biomed Res Int 2015;2015:781294. Epub 2015 Sep 16 doi: 10.1155/2015/781294. PMID: 26451378Free PMC Article

Recent clinical studies

Etiology

Abdolrahimzadeh S, Fameli V, Mollo R, Contestabile MT, Perdicchi A, Recupero SM
Biomed Res Int 2015;2015:781294. Epub 2015 Sep 16 doi: 10.1155/2015/781294. PMID: 26451378Free PMC Article

Diagnosis

Abdolrahimzadeh S, Fameli V, Mollo R, Contestabile MT, Perdicchi A, Recupero SM
Biomed Res Int 2015;2015:781294. Epub 2015 Sep 16 doi: 10.1155/2015/781294. PMID: 26451378Free PMC Article
Prontera P, Urciuoli R, Siliquini S, Macone S, Stangoni G, Donti E, Cantisani TA, Elia M, Belcastro V
Am J Med Genet A 2011 Dec;155A(12):3125-7. Epub 2011 Nov 3 doi: 10.1002/ajmg.a.34295. PMID: 22052670
Guion-Almeida ML, Richieri-Costa A
Clin Dysmorphol 1999 Jan;8(1):1-4. PMID: 10327243

Prognosis

Abdolrahimzadeh S, Fameli V, Mollo R, Contestabile MT, Perdicchi A, Recupero SM
Biomed Res Int 2015;2015:781294. Epub 2015 Sep 16 doi: 10.1155/2015/781294. PMID: 26451378Free PMC Article

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