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6 OMIM references -
5 associated genes
9 signs/symptoms
PROTEIN INTERACTIONS: 1
1 OMIM reference -
1 associated gene
22 signs/symptoms
Split hand-split foot malformation
Meacham syndrome

BTRC WT1
FBXW4
SHFM1
TP63
WNT10B


INTERACTOME
ASSOCIATIONS

(click on a score value to see the evidence)
TP63
(0.55)
WT1



Citations in the biomedical literature:


Split hand-split foot malformation
BTRC FBXW4 SHFM1 TP63 WNT10B
Meacham syndrome
WT1



Split hand-split foot malformation
Meacham syndrome

Synonym(s):
- Ectrodactyly
- Lobster-claw deformity
- SHFM
- Split hand foot malformation

Synonym(s):
- Meacham-Winn-Culler syndrome
- Rhabdomyomatous dysplasia - cardiopathy - genital anomalies

Classification (Orphanet):
- Rare bone disease
- Rare developmental defect during embryogenesis
- Rare genetic disease
Classification (Orphanet):
- Rare developmental defect during embryogenesis
- Rare endocrine disease
- Rare genetic disease
- Rare gynecologic or obstetric disease
- Rare urogenital disease

Classification (ICD10):
- Congenital malformations, deformations and chromosomal abnormalities -
Classification (ICD10):
- Congenital malformations, deformations and chromosomal abnormalities -

Epidemiological data:
Class of prevalence: 1-9 / 100 000
Average age onset: neonatal/infancy
Average age of death: -
Type of inheritance: autosomal dominant
Epidemiological data:
(no data available)

External references:
6 OMIM references -
No MeSH references
External references:
1 OMIM reference -
No MeSH references

Split hand-split foot malformation
Meacham syndrome

Very frequent
- Autosomal dominant inheritance
- Oligodactyly / ectrodactyly of fingers

Frequent
- Syndactyly of fingers / interdigital palm

Occasional
- Aniridia / iris hypoplasia
- Autosomal recessive inheritance
- Hand agenesis / absence
- Sensorineural deafness / hearing loss
- Trident hand / split hand / abnormal median ray
- X-linked recessive inheritance


Very frequent
- Ambiguous genitalia
- Diaphragmatic hernia / defect / agenesis
- Hypoplastic lungs / pulmonary hypoplasia / agenesis
- Uterine / uterus / Fallopian tubes anomalies
- Vagina anomalies / atresia / hydrometrocolpos / hymen imperforation

Frequent
- Absent lobe / lung / segmentation defect / abnormal lobulation / sequestration
- Death in infancy
- Hypoplastic left heart / ventricle
- Micropenis / small penis / agenesis
- Stillbirth / neonatal death
- Structural and functional anomalies of the spleen
- Undescended / ectopic testes / cryptorchidia / unfixed testes

Occasional
- Anomalous pulmonary venous return
- Asplenia / polysplenia / spleen lobulation / accessory spleen
- Atrial septal defect / interauricular communication
- Dextrocardia / abnormal heart position / cardiac heterotaxia / situs inversus
- Ectopic / horseshoe / fused kidneys
- Hypoplastic aorta / coarctation / stenosis / anomaly / aortic arch interruption
- Patent ductus arteriosus
- Tetralogy of Fallot / trilogy of Fallot
- Transposition of great vessels
- Ventricular septal defect / interventricular communication