U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Camptodactyly syndrome, Guadalajara type 1(GCS1; FTSS)

MedGen UID:
395241
Concept ID:
C1859359
Disease or Syndrome
Synonyms: CAMPTODACTYLY SYNDROME, GUADALAJARA, TYPE I; Faciothoracoskeletal syndrome; GCS 1
SNOMED CT: Camptodactyly syndrome Guadalajara type 1 (720602007)
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:0008898
OMIM®: 211910
Orphanet: ORPHA1327

Definition

A rare syndrome consisting of growth retardation, facial dysmorphism, camptodactyly and skeletal anomalies. To date only eight cases have been reported in the literature. Dysmorphic features include flat face, epicanthic folds, telecanthus, small downturned mouth, small ears with attached lobule and abnormal dental eruption and occlusion. Some patients had psychomotor development delayed. The reported cases suggest the condition is hereditary and is transmitted as an autosomal recessive trait. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Recent clinical studies

Diagnosis

Figuera LE, Ramírez-Dueñas ML, García-Cruz D, Villar V, Cantú JM
Clin Genet 1993 Jan;43(1):11-5. doi: 10.1111/j.1399-0004.1993.tb04418.x. PMID: 7681735

Supplemental Content

Recent activity

  • Camptodactyly syndrome, Guadalajara type 1
    Camptodactyly syndrome, Guadalajara type 1
    MedGen
  • Hair Dyes
    Hair Dyes
    Dyes used as cosmetics to change hair color either permanently or temporarily.<br/>Year introduced: 1991(1979)
    MeSH
  • Genes, sry
    Genes, sry
    The primary testis-determining gene in mammalians, located on the Y CHROMOSOME. It codes for a high mobility group box transcription factor (TRANSCRIPTION FACTORS) which initi...<br/>Year introduced: 2002
    MeSH
  • Genes, Immediate-Early
    Genes, Immediate-Early
    Genes that show rapid and transient expression in the absence of de novo protein synthesis. The term was originally used exclusively for viral genes where immediate-early refe...<br/>Year introduced: 1994
    MeSH
  • Anaphase
    Anaphase
    The phase of cell nucleus division following METAPHASE, in which the CHROMATIDS separate and migrate to opposite poles of the spindle.<br/>Year introduced: 1981(1978)
    MeSH

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...