Facial dysmorphism-shawl scrotum-joint laxity syndrome

Facial dysmorphism-shawl scrotum-joint laxity syndrome is characterised by facial dysmorphism (hypertelorism, telecanthus, downslanting palpebral fissures, ptosis, malar hypoplasia, broad nasal bridge, thin upper lip, smooth philtrum, and low-set prominent ears) and associated with joint anomalies (genu valgum or cubitus valgus, hyper-extensible joints, etc.). It has been described in two patients (a mother and her son). The boy also had hypoplastic shawl scrotum and cryptorchidism, and the mother had mild intellectual deficit.



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Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)
PMID
(PMCID)
        

Phenotype(s) retrieved from Orphanet

    Total: 24

HPO ID Term Frequency
HP:0000028 Cryptorchidism Very frequent (99-80%)
HP:0000049 Shawl scrotum Very frequent (99-80%)
HP:0000286 Epicanthus Very frequent (99-80%)
HP:0000316 Hypertelorism Very frequent (99-80%)
HP:0000319 Smooth philtrum Very frequent (99-80%)
HP:0000369 Low-set ears Very frequent (99-80%)
HP:0000411 Protruding ear Very frequent (99-80%)
HP:0000431 Wide nasal bridge Very frequent (99-80%)
HP:0000494 Downslanted palpebral fissures Very frequent (99-80%)
HP:0000506 Telecanthus Very frequent (99-80%)
HP:0000508 Ptosis Very frequent (99-80%)
HP:0002857 Genu valgum Very frequent (99-80%)
HP:0003764 Nevus Very frequent (99-80%)
HP:0010669 Hypoplasia of the zygomatic bone Very frequent (99-80%)
HP:0000239 Large fontanelles Frequent (79-30%)
HP:0000303 Mandibular prognathia Frequent (79-30%)
HP:0001256 Intellectual disability, mild Frequent (79-30%)
HP:0001537 Umbilical hernia Frequent (79-30%)
HP:0001608 Abnormality of the voice Frequent (79-30%)
HP:0001928 Abnormality of coagulation Frequent (79-30%)
HP:0002162 Low posterior hairline Frequent (79-30%)
HP:0002167 Neurological speech impairment Frequent (79-30%)
HP:0002967 Cubitus valgus Frequent (79-30%)
HP:0005692 Joint hyperflexibility Frequent (79-30%)


Phenotype(s) retrieved from case reports

    Total: 0

HPO ID Term # of case reports


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID