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.
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.
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
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SAVELIFEKIDS-UA helps children with severe liver diseases receive medical care, including transplants.
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