Overview
Cholestasis is not a disease; rather, it is a symptom of many diseases. It is defined as a pathologic state of reduced bile formation or flow. This definition applies more to the experimental situation, where the rates of bile formation and flow can be measured, than to human cholestasis, where neither can be assessed. Therefore, the clinical definition of cholestasis is any condition in which substances normally excreted into bile are retained. The serum concentrations of conjugated bilirubin and bile salts are the most commonly measured.
Not all substances normally excreted into bile are retained to the same extent in various cholestatic disorders. In some conditions, serum bile salts may be markedly elevated while bilirubin is only modestly elevated and vice versa. However, demonstrable retention of several substances is needed to establish a diagnosis of cholestasis.
Only in rare disorders of bilirubin metabolism does an isolated increase in the serum concentration of conjugated bilirubin appear, so increased serum conjugated bilirubin indicates cholestasis. The histopathologic definition of cholestasis is the appearance of bile within the elements of the liver, usually associated with secondary cell injury.
Symptoms and Signs
Cholestasis typically is noted in the first 2 wk of life. Infants are jaundiced and often have dark urine (conjugated bilirubin), acholic stools, and hepatomegaly. If cholestasis persists, chronic pruritus is common, as are symptoms and signs of fat-soluble vitamin deficiency; progression on growth charts may show a decline. If the underlying disorder causes hepatic fibrosis and cirrhosis, portal hypertension with subsequent abdominal distention from ascites, dilated abdominal veins, and upper GI bleeding from esophageal varices may develop.
Diagnosis
- Total and direct bilirubin
- Liver function tests
- Tests for metabolic, infectious, and genetic causes
- Hepatobiliary scan
- Occasionally, liver biopsy
Cholestasis is reduction or stoppage of bile flow :
- Disorders of the liver, bile duct, or pancreas can cause cholestasis.
- The skin and whites of the eyes look yellow, the skin itches, urine is dark, and stools may become light-colored and smell foul.
- Laboratory and often imaging tests are needed to identify the cause.
- Treatment depends on the cause, but drugs can help relieve itching.
With cholestasis the flow of bile (the digestive fluid produced by the liver) is impaired at some point between the liver cells and the duodenum (the first segment of the small intestine). When bile flow is stopped, the pigment bilirubin (a waste product formed when old or damaged red blood cells are broken down) escapes into the bloodstream and accumulates.
What is obstetric cholestasis (intrahepatic cholestasis of pregnancy – ICP)?
Obstetric cholestasis (sometimes called "cholestasis of pregnancy", "OC", "intrahepatic cholestasis of pregnancy", "ICP") affects the liver, which in some women seems to be oversensitive to pregnancy hormones. Bile is produced in the liver and normally it flows down the bile ducts into the intestines where it helps with the digestion of food. If you have OC, the flow of bile into the intestines is reduced and so bile salts build up in your blood.
The main symptom is itching, which is usually worse at night so can result in fatigue and insomnia. It often begins on the palms of the hands and the soles of feet and can become generalised. Some women are made so desperate by the itching that they scratch themselves until their skin is bleeding. Less commonly, women can develop jaundice. The itching completely disappears within a couple of weeks of giving birth.
How is OC managed?
Two drugs are currently used to manage OC. Ursodeoxycholic acid is favoured in the specialist centres for OC. It appears to eliminate or reduce the itching and can result in the liver function and bile acid results returning to normal. Steroids (in particular dexamethasone) can also be considered but need careful management.
Mothers with OC may be at risk of bleeding after the birth. This is because bile is needed to absorb vitamin K from food, and vitamin K helps the blood to clot. So in some hospitals the mother is given vitamin K daily by mouth until delivery to protect her from this small risk of bleeding. The baby is also protected by the vitamin K.
As far as the baby is concerned, the principal aim of treatment is to eliminate the risk of stillbirth by delivering him as soon as his lungs are mature enough for him to survive outside the womb. Scans will be used to monitor his growth and wellbeing. At present, doctors think it best to deliver the baby at about 35 to 38 weeks. If women with OC have their labours induced at this time, their babies are very likely to survive, while if pregnancy is allowed to continue to 40 weeks, the risk of stillbirth increases.
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