Collins D M, Shannon F T, Campbell C B
Aust N Z J Med. 1981 Feb;11(1):48-51. doi: 10.1111/j.1445-5994.1981.tb03735.x.
Arteriohepatic dysplasia is a rare congenital syndrome in which attenuated peripheral pulmonary arteries are associated with liver impairment. The earliest indication is usually persistent cholestatic jaundice in the first few months after birth. Although this is invariably associated with pruritus, bile acid kinetic studies have not been reported in such patients. This report describes a girl with a particularly mild form of the syndrome who was never observed to be clinically or biochemically jaundiced. Bile acid studies indicated that there was a defect in bile acid excretion but not in uptake or conjugation.
动脉肝发育不良是一种罕见的先天性综合征，其特征为外周肺动脉变细并伴有肝功能损害。最早的迹象通常是出生后最初几个月持续的胆汁淤积性黄疸。尽管这总是伴有瘙痒，但此类患者的胆汁酸动力学研究尚未见报道。本报告描述了一名患有该综合征特别轻微形式的女孩，其从未出现过临床或生化黄疸。胆汁酸研究表明，胆汁酸排泄存在缺陷，但摄取或结合方面无缺陷。
