小结
定义
病史和体格检查
关键诊断因素
- 新生儿黄疸
- 无胆汁便
其他诊断因素
- 小便黄赤
- 青肿
- 肝肿大
- 腹水
危险因素
- 遗传易感性
- 病毒感染
- 环境因素或行为暴露
诊断性检查
首要检查
- 血清总胆红素和直接或结合胆红素
- 新生儿筛查（包括对诊断半乳糖血症、甲状腺功能障碍、囊性纤维化及一系列代谢性疾病的检测）
- 凝血酶原时间 (PT)，INR
- FBC 和分类计数
- 血清 AST、ALT、ALP、γ-GT
- 腹部超声
需考虑的检查
- 肝胆闪烁显像（锝 Tc99m- 二异丙基 - 乙酰苯胺 - 亚氨基亚乙酸扫描）
- 肝脏活检
- 胆管造影
- 胸部 X 线检查 (CXR)
- 感染筛查：血培养和尿培养
- 尿液 PCR 检测巨细胞病毒
- 血浆或血清氨基酸
- α-1 水平和蛋白酶抑制剂 (Pi) 型
- 随机血清皮质醇
- 尿有机酸
- 尿琥珀酰丙酮
- 尿胆汁酸
- 乳酸盐 / 丙酮酸盐比例
治疗流程
无终末期肝病的胆道梗阻婴儿
患终末期肝病的胆道梗阻婴儿
肝门肠吻合术后
撰稿人
作者
Jessi Erlichman, MPH
Senior Director
Research Administration
The Children's Hospital of Philadelphia
Philadelphia
PA
利益声明
JE receives payment for contributions to UpToDate.
Kathleen Loomes, MD
Professor of Pediatrics
Department of Pediatrics
The Children's Hospital of Philadelphia
Philadelphia
PA
利益声明
KL receives payment for contributions to UpToDate. KL is on an advisory panel for Albireo Pharmaceuticals and Mirum Pharmaceuticals and is a consultant for Travere Therapeutics.
鸣谢
Jessi Erlichman and Dr Kathleen Loomes would like to gratefully acknowledge Dr Jonathan A. Flick and Dr Barbara A. Haber, previous contributors to this topic.
利益声明
JAF and BAH declare that they have no competing interests.
同行评议者
Benjamin L. Shneider, MD
Professor of Pediatrics
Service Chief in Pediatric Gastroenterology, Hepatology and Nutrition
Baylor College of Medicine
Texas Children’s Hospital
Houston
TX
利益声明
BLS has received research funding from, and been a consultant to, several pharmaceutical companies that make antibiotic agents that might be used for treating bacterial prostatitis.
Mark D. Stringer, MD
Former Professor of Paediatric Surgery
University of Otago
Dunedin
New Zealand
利益声明
MDS declares that he has no competing interests.
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