Abstract

Prurit (itchy skin) is a common symptom accompanying chronic cholestasis. The pathogenesis of pruritus in cholestatic diseases is not well understood. It may include a specific neural pathway regulated by substances that can cause skin itching, such as bile acids, opioids, serotonin and lysophosphatidic acid. In exceptional cases, itch refractory to therapy may lead to the need for invasive therapy, including liver transplantation. While the therapeutic management of cholestatic pruritus is well known in adults, children do not agree on the difficulty of conducting controlled clinical trials. The strategy to combat cholestatic pruritus in children is currently based on several areas of specific therapy, which should be associated with skin care and non-specific treatment of cholestasis, including ursodeoxycholic acid. Prurit should be evaluated during treatment as objectively as possible. The first line of treatment for cholestatic pruritus is rifampicin. Second-line therapy is discussed in each case. In the most severe cases, invasive treatments may be suggested. The aim of this work was to review the pathophysiological mechanisms associated with cholestatic pruritus in children and suggest potential therapeutic methods.

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