最近审阅: 3 十一月 2020
最近更新: 23 一月 2018

小结

定义

病史和体格检查

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
  • 免疫失调
  • 病毒感染
  • 环境因素或行为暴露
  • 母源性糖尿病 (DM)

诊断性检查

首要检查

  • 血清总胆红素和结合胆红素
  • 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
更多 首要检查

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
  • ERCP
  • 胸部 X 线检查 (CXR)
  • 感染筛查:血培养和尿培养 +/- 脊髓液检查
  • 巨细胞病毒尿液 PCR
  • 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
更多 需考虑的检查

治疗流程

撰稿人

Administrative Director

GI Clinical Research

The Children's Hospital of Philadelphia

Philadelphia

PA

利益声明

JE receives payment for contributions to UpToDate.

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 and Wolters Kluwer. KL is a consultant for Albireo Pharmaceuticals and Mirum Pharmaceuticals.

Dr 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.

同行评议者展开全部内容

Director

Pediatric Hepatology Center

Visiting Professor of Pediatrics

University of Pittsburgh

Pediatric Hepatology Center

Pittsburgh

PA

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.

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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