Paediatric and Congenital Cardiology Service, Starship Child Health, Auckland, New Zealand.
Northern Hub - Genetic Health Service NZ, Auckland, New Zealand.
Clin Genet. 2022 Nov;102(5):461-462. doi: 10.1111/cge.14199. Epub 2022 Aug 3.
PPP1R13L-associated cardiocutaneous syndrome is an autosomal recessive condition that presents with life-threatening dilated cardiomyopathy in early childhood, with or without features of inflammation on cardiac histology. There is also a variably expressed ectodermal phenotype.
PPP1R13L 相关的心肌皮肤综合征是一种常染色体隐性遗传病,在儿童早期表现为危及生命的扩张型心肌病,伴有或不伴有心肌组织学炎症特征。也存在可变表达的外胚层表型。