Intrahepatic cholestasis

What is Intrahepatic Cholestasis?

Intrahepatic Cholestasis is a pathological condition in which the formation or flow of bile from hepatocytes (liver cells) into the small bile ducts is impaired. As a result, bile acids and pigments accumulate in the liver and bloodstream, leading to cell damage, jaundice, itching, and deficiency of fat-soluble vitamins.

  • It can have different causes, including:
  • Genetic (as in PFIC – Progressive Familial Intrahepatic Cholestasis)
  • Hormonal (for example, intrahepatic cholestasis of pregnancy)
  • Toxic or drug-induced (as a side effect of medications, alcohol, or chemicals)
  • Secondary to other diseases (such as viral hepatitis, autoimmune liver diseases, or metabolic disorders)

Frequently Asked Questions

1. What are the signs of Intrahepatic Cholestasis?
  • Intense skin itching (especially at night)

  • Jaundice (yellowing of the skin and mucous membranes)

  • Dark urine, pale stools

  • Fatigue, weakness

  • In chronic cases: growth delay, osteoporosis, bleeding (due to vitamin K deficiency)

It depends on the form:

  • In PFIC — yes, autosomal recessive inheritance

  • In pregnancy-related cholestasis — genetic predisposition combined with hormonal changes

  • In drug-induced or secondary cholestasis — heredity does not play a role

  • Elimination of the cause (stopping medications, treating infection, managing pregnancy)

  • Ursodeoxycholic acid (improves bile flow)

  • Medications to reduce itching (cholestyramine, rifampicin, modern IBAT inhibitors)

  • Supportive therapy: fat-soluble vitamins (A, D, E, K), high-calorie diet

  • In severe cases: liver transplantation

  • In mild cases (such as pregnancy-related cholestasis), the condition completely resolves after delivery and does not harm the mother

  • In severe hereditary forms (PFIC, other genetic defects), progression to cirrhosis and liver failure is possible

  • Long-term cholestasis increases the risk of liver damage and vitamin deficiencies

  • Clinical signs (itching, jaundice, changes in stool/urine color)

  • Blood tests (bilirubin, alkaline phosphatase, GGT, bile acids)

  • Ultrasound of the liver and bile ducts (to rule out mechanical obstruction)

  • If needed, liver biopsy

  • Genetic testing when hereditary cholestasis is suspected

  • Liver: cholestasis, fibrosis, cirrhosis

  • Bones: osteoporosis due to vitamin D deficiency

  • Circulatory system: bleeding due to vitamin K deficiency

  • Nervous system and development: growth and developmental delays in severe cases

  • Fetus during pregnancy: risk of preterm birth or complications

There is no radical cure. In drug-induced or pregnancy-related cholestasis, removing the cause leads to complete resolution of symptoms. In hereditary forms, the only radical option remains liver transplantation.

Recommendations for Parents

  • Carefully watch for signs of itching, jaundice, and changes in stool or urine color

  • Follow a diet with limited fatty foods, and add vitamins as prescribed by the doctor

  • Regularly perform blood tests and liver check-ups

  • Do not give your child any medications without consulting a doctor, especially those that may harm the liver

  • Remember: with proper monitoring and treatment, symptoms can be controlled, and your child’s quality of life can be maintained