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Camptodactyly syndrome, Guadalajara type 2

MedGen UID:
419870
Concept ID:
C2931680
Disease or Syndrome
Synonym: Guadalajara camptodactyly syndrome type II
SNOMED CT: Camptodactyly syndrome Guadalajara type 2 (720603002)
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:0008899
OMIM®: 211920
Orphanet: ORPHA1326

Definition

An extremely rare multiple congenital anomaly syndrome with characteristics of distinctive intrauterine growth retardation, skeletal dysplasia with multiple malformations including camptodactyly of all fingers, bilateral hallux valgus, short second, fourth and fifth toes, hypoplastic patella, microcephaly, low-set ears, short neck, cuboid-shaped vertebral bodies, pectus excavatum, hip dislocation, and hypoplastic pubic region and genitalia. Described in two sisters there have been no further descriptions in the literature since 1985. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
Follow this link to review classifications for Camptodactyly syndrome, Guadalajara type 2 in Orphanet.

Recent clinical studies

Diagnosis

Zechi-Ceide RM, Guion-Almeida ML, Richieri-Costa A
Clin Dysmorphol 2002 Apr;11(2):129-32. doi: 10.1097/00019605-200204000-00011. PMID: 12002144

Supplemental Content

Recent activity

  • Camptodactyly syndrome, Guadalajara type 2
    Camptodactyly syndrome, Guadalajara type 2
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    Nucleotide sequences of a gene that are involved in the regulation of GENETIC TRANSCRIPTION.<br/>Year introduced: 2006
    MeSH
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    Chemicals and substances that impart color including soluble dyes and insoluble pigments. They are used in INKS; PAINTS; and as INDICATORS AND REAGENTS....<br/>Year introduced: 2006(1963)
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