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Limb disorders v0.202 SPINT2 Rebecca Foulger Marked gene: SPINT2 as ready
Limb disorders v0.202 SPINT2 Rebecca Foulger Gene: spint2 has been classified as Amber List (Moderate Evidence).
Limb disorders v0.202 SPINT2 Rebecca Foulger commented on gene: SPINT2: Added 'watchlist' tag: require further evidence for a diagnostic rating.
Limb disorders v0.202 SPINT2 Rebecca Foulger Tag watchlist tag was added to gene: SPINT2.
Limb disorders v0.202 SPINT2 Rebecca Foulger Classified gene: SPINT2 as Amber List (moderate evidence)
Limb disorders v0.202 SPINT2 Rebecca Foulger Added comment: Comment on list classification: Updated rating from Red to Amber. 2 cases from literature where congenital diarrhea (CSD) presents with hexadactyly. Need more evidence before rating as diagnostic.
Limb disorders v0.202 SPINT2 Rebecca Foulger Gene: spint2 has been classified as Amber List (Moderate Evidence).
Limb disorders v0.200 SPINT2 Rebecca Foulger Publications for gene: SPINT2 were set to 24142340; 19185281
Limb disorders v0.199 SPINT2 Rebecca Foulger Phenotypes for gene: SPINT2 were changed from Polydactyly; Diarrhea 3, secretory sodium, congenital, syndromic, 270420; hexadactyly to Polydactyly; Diarrhea 3, secretory sodium, congenital, syndromic, 270420; hexadactyly; congenital sodium diarrhea with additional syndromic features
Limb disorders v0.180 SPINT2 Rebecca Foulger commented on gene: SPINT2: PMID:19185281 (Heinz-Erian et al., 2009) reviewed data from a large cohort of CSD patients (n = 24).1 Swedish female patient with syndromic CSD had a homozygous SPINT2 allele (c.488A→G, p.Y163C) and presented with an extra digit on her right hand.
Limb disorders v0.180 SPINT2 Rebecca Foulger commented on gene: SPINT2: In 9 children from 7 families with MIM:270420, Salomon et al. (2014, PMID:24142340) identified either homozygosity for the Y163C mutation in SPINT2 or compound heterozygosity for Y163C and another variant in SPINT2. One of the patients (an Italian boy) exhibited additional hexadactyly.
Limb disorders v0.180 SPINT2 Rebecca Foulger Added comment: Comment on phenotypes: Biallelic, loss of function mutations in SPINT2 cause a syndromic form of congenital sodium diarrhea (CSD) which can (rarely) include hexadactyly.
Limb disorders v0.180 SPINT2 Rebecca Foulger Phenotypes for gene: SPINT2 were changed from Polydactyly; Diarrhea 3, secretory sodium, congenital, syndromic, 270420; hexadactyly to Polydactyly; Diarrhea 3, secretory sodium, congenital, syndromic, 270420; hexadactyly
Limb disorders v0.179 SPINT2 Rebecca Foulger Phenotypes for gene: SPINT2 were changed from Polydactyly to Polydactyly; Diarrhea 3, secretory sodium, congenital, syndromic, 270420; hexadactyly
Limb disorders v0.178 SPINT2 Rebecca Foulger Added comment: Comment on mode of inheritance: Biallelic MOI supported by OMIM and literature (PMIDs:19185281,24142340).
Limb disorders v0.178 SPINT2 Rebecca Foulger Mode of inheritance for gene: SPINT2 was changed from to BIALLELIC, autosomal or pseudoautosomal
Limb disorders v0.177 SPINT2 Rebecca Foulger Publications for gene: SPINT2 were set to
Limb disorders SPINT2 Ellen McDonagh Added gene to panel