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Chronic Cholestasis - Causes, Symptoms, and Treatment

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Chronic cholestasis is characterized by defective bile acid transport from the liver to the intestine.

Medically reviewed by

Dr. Ghulam Fareed

Published At February 28, 2023
Reviewed AtMarch 13, 2023

Introduction

Cholestasis s a liver disease where the bile(which helps in the digestion of fats) flow from the liver is blocked or reduced. This can cause an accumulation of bilirubin in the blood. The liver is responsible for producing the pigment bilirubin and excreted by the bile.

What Is Chronic Cholestasis?

Cholestasis is the compound expression of a Greek word meaning “standing still of bile.” Chronic cholestasis is defined as reduced bile flow due to defective secretions by the hepatocytes or a block in the bile flow to the extrahepatic bile ducts. Bile is an enzyme secreted by the liver that helps digest fats. When the bile flow is interrupted, it can cause increased amounts of bilirubin (produced by the liver and excreted as bile).

What Are the Causes of Cholestasis?

Cholestasis is of two types:

  • Intrahepatic cholestasis

  • Extrahepatic cholestasis.

Intrahepatic cholestasis arises within the liver and is caused by:

  • Infections like mycobacterial infections.

  • Drug-induced.

  • Genetic abnormalities.

  • Hormonal effects on bile flow during pregnancy (called cholestasis of pregnancy).

  • Total parenteral nutrition.

  • Infiltrative diseases like sarcoidosis, amyloidosis, and neoplasia

  • Liver allograft rejection.

  • Primary biliary cirrhosis.

  • Primary sclerosing cholangitis (small duct).

  • Liver flow abnormalities lead to nodular regenerative hyperplasia.

  • Alcohol-induced liver injury.

Extrahepatic cholestasis occurs due to a physical barrier to the bile passage and can be brought on due to the following:

  • Biliary obstructions, as in malignancies or pancreatitis.

  • Primary sclerosing cholangitis.

  • Secondary sclerosing cholangitis.

  • Biliary ischemia.

  • Opportunistic bile duct infections.

What Are the Symptoms of Cholestasis?

  • Jaundice (the skin and the whites of the eyes appear yellow).

  • Darkening of urine.

  • Light stools.

  • Generalized itchiness.

Jaundice is due to excess bilirubin deposited on the skin, while darkened urine is due to the excess bilirubin excreted by the kidneys. The deposition of excess bilirubin on the skin causes itching of the skin. Stools are lightened as bilirubin cannot enter the intestine, preventing excretion. Stools can have increased amounts of fat and can be foul smelling as bilirubin is unable to digest fats.

Lack of bile in the intestine can also cause calcium and vitamin D malabsorption, affecting bone tissues. In addition, vitamin K is also not absorbed properly, which can cause bleeding issues.

How Is Chronic Cholestasis Diagnosed?

  • History: A good case history is essential to diagnose chronic cholestasis.

  • Blood tests: Blood tests to check for liver enzymes

  • Physical examination: Primary biliary cirrhosis may be associated with xanthomas (a skin condition where fat gets deposited under the skin surface), hyperpigmentation, and features compatible with Raynaud’s phenomenon (a problem that causes reduced blood flow to the fingers). A palpable gallbladder can be suggestive of biliary obstruction. Hepatomegaly (an enlargement of the liver) can be present in infiltrative diseases. Common stigmata of chronic liver diseases like palmar erythema (a skin condition where the palms turn red), vascular spider (also called spider angioma, which has a red spot in the center and extensions that spread like a spider’s web), and muscle hypotrophy can be seen.

  • Laboratory examinations: Laboratory tests include abdominal ultrasonography to check for bile duct dilatation. A liver biopsy is indicated in chronic intrahepatic cholestasis, which will also help in staging the condition. If an obstruction in the bile duct is observed, more precise imaging must be done to identify the exact location and treat it accordingly.

How Is Chronic Cholestasis Treated?

Treatment for chronic cholestasis is planned after understanding the underlying cause. People with chronic cholestasis are advised to stop anything toxic to the liver, like alcohol or drugs. If a medication causes the condition, an alternative medicine can be prescribed. Surgery would be essential if the reason for bile accumulation is a gallbladder stone or tumor. For blockage of the bile duct, surgery or endoscopy would be required.

Cholestyramine, taken orally, can be used to relieve itching. This drug binds with bile in the intestine so that it does not accumulate on the skin and cause itching. In addition, supplementation of vitamin K can help control bleeding.

What Are the Risks Associated With Chronic Cholestasis?

Pregnancy cholestasis or obstetric cholestasis can be severe for the mother and the baby. While most cases are not dangerous, they can cause complications like preterm delivery, fetal distress, and stillbirth.

Obstetric cholestasis resolves after the birth of the baby. However, research shows that mothers with pregnancy cholestasis are three times more likely to have liver cancer at later stages. In addition, an increased risk of biliary cancer, thyroid diseases, diabetes, and Crohn’s disease (inflammatory bowel disease) is also present.

How Can We Prevent Chronic Cholestasis?

At the time of diagnosis, the patient might not show any symptoms, which can minimize the significance of the disease. Though chronic cholestasis can occur in anyone, irrespective of age or gender, specific preventive measures can help to stave off the condition or slow the disease’s progression.

  • Alcohol consumption: More than four drinks per day (containing 48 grams of ethanol) have been linked to an increased risk of cirrhosis, hepatocellular carcinoma, and premature death. In addition, Ascites (a condition in which fluid fills abdominal spaces), hepatic encephalopathy (a nervous disorder caused by liver disease), and variceal bleeding risk were reported to be 50% in those who consume alcohol over five years.

  • Immunizations: A one-time pneumococcal immunization is advised for patients with cirrhosis to prevent further complications. A yearly influenza vaccine should also be administered to reduce influenza-related mortality.

  • Avoid Over-The-Counter Drugs: Numerous common medications, vitamins, and mineral supplements delivered over the counter have been found to cause liver toxicity. Hence, a physician should always be consulted before starting any medication.

Conclusion

With an increase in understanding of biliary pathophysiology, new advances are possible. Chronic cholestasis can occur at any age, irrespective of gender. Recovery from the condition depends on the severity of the stage at which it was diagnosed. The underlying cause of chronic cholestasis is yet another factor determining the prognosis. For example, if the underlying cause is a gallbladder stone, its removal can cure the condition completely. On the other hand, recovery would be difficult due to liver damage.

Dr. Ghulam Fareed
Dr. Ghulam Fareed

Medical Gastroenterology

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cholestasisbile duct disease
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