胆汁淤积是指胆汁无法正常量到达十二指肠。干扰源可能位于主胆管（肝外胆汁淤积）或肝脏内（肝内胆汁淤积）。

从形态学上看，胆汁淤积的特征是胆汁在肝细胞和胆道中积聚；从生物化学上看，胆汁淤积的特征是所有正常情况下从胆汁中排出的物质均被滞留。

症状通常为瘙痒和缓慢出现的黄疸。

“阻塞性黄疸”一词通常指肝外胆汁淤积。

Cholestasis is defined as failure of normal amounts of bile to reach the duodenum. The source of the interference may reside in the main bile ducts - extrahepatic cholestasis - or within the liver - intrahepatic cholestasis.

Morphologically, cholestasis is characterised by the accumulation of bile in liver cells and biliary passages; biochemically, by the retention of all substances normally excreted in the bile.

Presentation is usually with pruritus and the slow onset of jaundice.

 

The term "obstructive jaundice" usually refers to cholestasis of extra-hepatic origin.

一、分类Classification

胆汁淤积根据原发病理部位可分为：

• 肝外胆汁淤积——占所有病例的 70%
• 肝内胆汁淤积 - 剩余的 30%

Cholestasis can be divided up according to the site of the primary pathology:

• extrahepatic cholestasis - accounting for 70% of all cases
• intrahepatic cholestasis - the remaining 30%

二、临床表现Clinical features

黄疸是胆汁淤积最显著的症状之一，其发展缓慢，可能在一段时间前出现瘙痒。随后，皮肤可能变绿。

胆汁淤积的一般症状可能包括：

• 黄瘤：手掌皱褶、乳房下方、颈部。它们表明血清胆固醇升高了几个月。腱鞘上的黄瘤很少与胆汁淤积有关。
• 眼睑黄瘤
• 划痕：擦伤
• 杵状指
• 粪便稀软、苍白、量多、有异味
• 深橙色尿液

此外，肝外胆汁淤积可能表现为：

• 由于胆囊疾病、恶性肿瘤或肝包膜拉伸引起的疼痛
• 由于胆管炎上升引起的发烧
• 胆囊可触及和/或有压痛
• 肝脏肿大，通常光滑

 

Amongst the most prominent signs of cholestasis, jaundice develops slowly and may be preceded by pruritus for some time. Later, the skin may become greenish.

General signs of cholestasis may include:

• xanthomas: palmar creases, below the breast, on the neck. They indicate raised serum cholesterol of several months. Xanthomas on the tendon sheaths are uncommonly associated with cholestasis.
• xanthelasma on the eyelids
• scratch marks: excoriation
• finger clubbing
• loose, pale, bulky, offensive stools
• dark orange urine

In addition, extrahepatic cholestasis may be present with:

• pain, due to gallbladder disease, malignancy, or stretching of the liver capsule
• fever, due to ascending cholangitis
• palpable and / or tender gallbladder
• enlarged liver, usually smooth

三、初步调查Initial investigations

血液学：

• 全血细胞计数 - 可能因恶性肿瘤而贫血
• 白细胞计数升高可能表明胆管炎或潜在的恶性肿瘤
• 病毒性肝炎可能会出现白细胞减少症
• 网织红细胞增多提示肝前黄疸
• 凝血酶原时间 - 慢性肝病患者凝血酶原时间延长；胆汁淤积患者在肠外补充维生素 K 10 毫克后将恢复正常

生物化学：

• 血清转氨酶 - 肝性黄疸时血清转氨酶升高，肝外黄疸时血清转氨酶升高，但程度较轻
• 血清碱性磷酸酶 - 肝外黄疸时升高；伴随的γ-谷氨酰转移酶升高证实肝源性黄疸
• 血清胆红素 - 确认黄疸；用于监测进展
• 血清白蛋白和球蛋白 - 急性黄疸时变化不大；慢性肝性黄疸时白蛋白降低，球蛋白升高

尿液分析

凳子：

• 胆汁淤积症患者的粪便呈苍白色
• 潜血——提示癌症

腹部超声检查-评估胆管扩张、肝脏大小、肝转移、门脉血流、腹水、淋巴结肿大

胸部X光检查——显示原发性或继发性肿瘤，以及因肝脏肿大或结节而导致的右膈肌不规则或隆起

 

Haematology:

• full blood count - may be anaemic due to malignancy
• raised white count may indicate cholangitis or underlying malignancy
• leucopaenia may occur in viral hepatitis
• reticulocytosis indicates pre-hepatic jaundice
• prothrombin time - prolonged in chronic liver disease; will return to normal in cholestasis following parenteral vitamin K, 10 mg

Biochemistry:

• serum transaminases - increased in hepatic jaundice and to a lesser degree, in extrahepatic jaundice
• serum alkaline phosphatase - raised in extra-hepatic jaundice; hepatic origin confirmed by concomitant rise in gamma glutamyl transferase
• serum bilirubin - confirms jaundice; used to monitor progress
• serum albumin and globulin - little change in acute jaundice; albumin decreased and globulin increased in chronic hepatic jaundice

Urinalysis

Stools:

• pale stools in cholestasis
• occult blood - suggests carcinoma

Abdominal ultrasound - assesses bile duct dilatation, liver size, liver metastases, portal blood flow, ascites, lymphadenopathy

Chest x-ray - to show primary or secondary tumours, and any irregularity or elevation of the right diaphragm due to enlarged or nodular liver

四、诊断Diagnosis

Cholestasis is confirmed by:

• elevated serum bilirubin - in proportion to duration of cholestasis; returns to normal once cholestasis is relieved
• raised serum alkaline phosphatase - to more than 3X upper limit of normal;
• LFTs - aminotransferases mildly raised; raised gamma GT
• increased urinary bilirubin
• urinary urinobilinogen is excreted in proportion to amount of bile reaching the duodenum i.e. absence of urinobilinogen indicates complete biliary obstruction

Identification of cause:

• dilated ducts on ultrasound - percutaneous transhepatic cholangiograpy
• undilated ducts on ultrasound - endoscopic retrograde cholangio-pancreatography
• needle biopsy of the liver

 

胆汁淤积可通过以下方式确诊：

• 血清胆红素升高 - 与胆汁淤积持续时间成正比；胆汁淤积缓解后恢复正常
• 血清碱性磷酸酶升高 - 超过正常上限的 3 倍；
• LFT - 氨基转移酶轻度升高；γ-GT 升高
• 尿胆红素升高
• 尿中尿胆原的排出量与到达十二指肠的胆汁量成比例，即尿胆原的缺失表明胆道完全阻塞

病因鉴定：

• 超声检查发现胆管扩张 - 经皮肝穿刺胆管造影术
• 超声检查未扩张的导管 - 内镜逆行胰胆管造影
• 肝脏穿刺活检

五、管理Management

 

综合管理：

• 营养——问题是肠道胆汁盐缺乏：
  • 必须保持热量和蛋白质摄入量，但减少脂肪摄入。额外的脂肪由中链甘油三酯（如 Portagen 或椰子油）提供
  • 脂溶性维生素 - A、D、E、K - 如果有缺乏的迹象，可能需要补充
• 瘙痒症 - 选择：
  • 胆道外引流
  • 考来烯胺
  • 光疗
  • 熊去氧胆酸
  • 利福平
• 肝性骨营养不良症可能需要维生素 D、磷酸盐或钙；绝经后妇女需要雌激素

肝外胆汁淤积通常需要手术治疗。

请注意，在胆汁淤积相关高脂血症中使用氯贝特治疗会导致血清胆固醇升高。

 

General management:

• nutrition - problem is that of intestinal bile salt deficiency:
  • calorie and protein intake must be maintained but fat reduced. Additional fat supplied by medium chain triglycerides e.g. Portagen or coconut oil
  • fat soluble vitamins - A, D, E, K - may need to be supplemented if signs of deficiency
• pruritus - options:
  • external biliary drainage
  • cholestyramine
  • phototherapy
  • ursodeoxycholic acid
  • rifampicin
• hepatic osteodystrophy may require vitamin D, phosphate or calcium; oestrogens in postmenopausal women

Extrahepatic cholestasis often requires surgical management.

 

Note that the use of clofibrate therapy in the hyperlipidaemia associated of cholestasis is associated with a raised serum cholesterol.