Jüngst B K, Spranger J
Monatsschr Kinderheilkd (1902). 1976 Jun;124(6):538-41.
The ballooning mitral valve defect has been repeatedly found in hereditary disorders of connective tissue such as the Marfan syndrome, osteogenesis imperfecta and the Ullrich-Turner syndrome. The present report concerns a nine year old girl with tricho-rhino-phalangeal dysplasia type I and this peculiar anomaly of the mitral valve. Since a literature review disclosed two more cases with tricho-rhinophalangeal dysplasia I and mitral insufficiency, the association may be more than fortuitous. Apparently the mesenchymal changes in tricho-rhino-phalangeal dysplasia I are not limited to the skeletal system.
在诸如马方综合征、成骨不全症和乌尔里希 - 特纳综合征等结缔组织遗传性疾病中,反复发现二尖瓣气球样病变。本报告涉及一名患有Ⅰ型毛发 - 鼻 - 指(趾)发育异常及二尖瓣这一特殊异常的9岁女孩。由于文献综述又发现了另外两例患有Ⅰ型毛发 - 鼻 - 指(趾)发育异常和二尖瓣关闭不全的病例,这种关联可能并非偶然。显然,Ⅰ型毛发 - 鼻 - 指(趾)发育异常中的间充质变化并不局限于骨骼系统。