Elhoury Motea E, Faqeih Eissa, Almoukirish Abdulrahman S, Galal Mohammed O
1Department of Pediatric Cardiology,Prince Salman Heart Center,Riyadh,Saudi Arabia.
2Department of Medical Genetics,Children Hospital,King Fahad Medical City,Riyadh,Saudi Arabia.
Cardiol Young. 2015 Jan;25(1):81-6. doi: 10.1017/S1047951113001753. Epub 2013 Nov 6.
Geleophysic dysplasia is an extremely rare acromelic skeletal dysplasia resembling lysosomal storage disease. It is characterised by characteristic facial phenotype, short stature, micromelia, joint contracture, and early cardiac valvular involvement. It has been described worldwide in <40 patients. Herein, we describe the cardiac features in three Saudi sisters with proved autosomal recessive geleophysic dysplasia who showed different levels of severity of their cardiac involvement.
弹力纤维发育异常是一种极为罕见的肢端短小性骨骼发育异常,类似于溶酶体贮积病。其特征为独特的面部表型、身材矮小、四肢短小、关节挛缩以及早期心脏瓣膜受累。全球范围内报道的患者不足40例。在此,我们描述了三名沙特姐妹的心脏特征,她们被证实患有常染色体隐性弹力纤维发育异常,且心脏受累程度各不相同。