Kotthoff S, Böse T, Vogt J
Universität Göttingen, Abteilung für Pädiatrische Kardiologie.
Klin Padiatr. 1990 Jan-Feb;202(1):50-2. doi: 10.1055/s-2007-1025485.
We present a case of a 6 week old infant with Alagille syndrome. Cardiological evaluation revealed peripheral pulmonary arterial stenosis and left ventricular endocardial fibroelastosis. While peripheral pulmonary arterial stenosis are typical for the syndrome this is the first case reported with endocardial fibroelastosis. Complex cardiac malformations may worsen the prognosis in Alagille syndrome. They require early diagnosis and therapy.
我们报告一例6周大患有阿拉吉列综合征的婴儿。心脏评估显示外周肺动脉狭窄和左心室心内膜弹力纤维增生症。虽然外周肺动脉狭窄是该综合征的典型表现,但这是首例报告合并心内膜弹力纤维增生症的病例。复杂的心脏畸形可能会使阿拉吉列综合征的预后恶化。它们需要早期诊断和治疗。