Burton E M, Babcock D S, Heubi J E, Gelfand M J
Department of Radiology, Children's Hospital Medical Center, Cincinnati, Ohio.
South Med J. 1990 Mar;83(3):294-302. doi: 10.1097/00007611-199003000-00010.
We reviewed the laboratory parameters, clinical information including presence or absence of stool pigmentation, and ultrasonographic findings in 67 patients with neonatal conjugated hyperbilirubinemia and liver biopsies. Hepatobiliary nuclear scintigraphy was done in 14 of the patients. Final diagnoses included extrahepatic biliary atresia, neonatal hepatitis, cystic fibrosis, metabolic liver disease, alpha 1-antitrypsin deficiency, bile duct stenosis, Alagille syndrome (arteriohepatic dysplasia), choledochal cyst, panhypopituitarism, and miscellaneous causes of intrahepatic cholestasis. A single diagnostic criterion is insufficient to distinguish the various causes of neonatal jaundice. Clinical laboratory values varied widely among patients with medical and surgical causes of jaundice. Absence of stool pigmentation was not specific for biliary atresia and was found in patients with medical causes of jaundice. Conversely, two patients with biliary atresia had pigmented stools at presentation. Ultrasonography was diagnostic only for choledochal cyst and bile duct stenosis. Nonvisualization of the gallbladder by either ultrasonography or nuclear hepatobiliary scintigraphy was nonspecific in the discrimination of medical from surgical causes of jaundice. A multidisciplinary approach to the evaluation of neonatal jaundice is necessary, since no single test or imaging modality can reliably define the cause in all cases.
我们回顾了67例新生儿结合胆红素血症患者的实验室参数、临床信息(包括有无粪便色素沉着)、超声检查结果以及肝活检情况。其中14例患者进行了肝胆核素扫描。最终诊断包括肝外胆道闭锁、新生儿肝炎、囊性纤维化、代谢性肝病、α1-抗胰蛋白酶缺乏症、胆管狭窄、阿拉吉列综合征(动脉肝发育不良)、胆总管囊肿、全垂体功能减退以及肝内胆汁淤积的其他原因。单一的诊断标准不足以区分新生儿黄疸的各种病因。黄疸的内科和外科病因患者的临床实验室值差异很大。粪便无色素沉着并非胆道闭锁所特有,在黄疸内科病因患者中也可发现。相反,两名胆道闭锁患者就诊时粪便有色素沉着。超声检查仅对胆总管囊肿和胆管狭窄具有诊断价值。超声或肝胆核素扫描未显示胆囊在区分黄疸的内科和外科病因方面并无特异性。由于没有单一的检查或影像学方法能够在所有病例中可靠地确定病因,因此采用多学科方法评估新生儿黄疸是必要的。