摘要胆道闭锁(BA)是以早发、持续的梗阻性黄疸为主要症状的严重疾病.因胆管堵塞导致胆酸的减少及肝功能减退导致肝脏产生蛋白能力减弱,使脂溶性维生素缺乏成为儿童BA的常见表现.脂溶性维生素A、D、E、K缺乏会分别导致夜盲症、骨钙化不良、神经系统障碍和凝血功能障碍等多系统功能障碍.其治疗主要为脂溶性维生素的补充,包括肠内外补充、不同剂型及个体化补充与检测等.虽然BA患儿脂溶性维生素缺乏已有报道,但其严重程度、与肝功能的相关性及治疗相关结果尚未得到重视.

abstractsBiliary atresia (BA)is a serious disease with early onset and persistent obstructive jaundice as the main symptom. The reducing of bile acid,decreasing of liver function and the ability of protein production,cause the fat - soluble vitamins deficiency is common in BA patients. The fat - soluble vitamins A,D,E and K deficiency will cause night blindness,adverse calcification of bone,neurological disorders,blood coagulation dysfunction and other sys-tems dysfunction. The treatment is mainly supplement of fat - soluble vitamins including internal and external supple-mentation,different dosage forms,and individualized supplement and detection. Although fat - soluble vitamins deficien-cy in children with BA has been reported,the severity,correlation with liver function,and treatment results have not been paid much attention.