Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia · 2019
A rare association of pathological variant of Alport's syndrome caused by hemizygous 5' splice mutation in intron 10 of COL4A5 gene with metachondromatosis due to heterozygous missense variation in protein tyrosine phosphatase nonreceptor type 11 gene.
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Sethi Suman, Mehta Sudhir, Makkar Vikas, Kaur Simran, Sohal P M
Аннотация
Metachondromatosis is a rare disorder of autosomal inheritance with incomplete penetrance, which is characterized by formation of osteochondroma and enchondroma, caused by loss of function of the protein tyrosine phosphatase nonreceptor type 11 (PTPN11) gene. Diagnosis is made based on the distribution and orientation of lesions with history of regression of lesions with time and confirmed by genetic mutation of PTPN11 gene. We report a rare case of a 24-year-old male with Alport's syndrome with metachondromatosis due to missense variation in PTPN11 gene.
Предметные рубрики MeSH
Bone NeoplasmsChondromatosisCollagen Type IVDNA Mutational AnalysisExostoses, Multiple HereditaryGenetic Predisposition to DiseaseHemizygoteHeterozygoteHumansMaleMutationMutation, MissenseNephritis, HereditaryPhenotypeProtein Tyrosine Phosphatase, Non-Receptor Type 11RNA Splice SitesRisk FactorsYoung Adult