Summerskill-Walshe-Tygstrup Syndrome

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Classification according to ICD-10
K83.1 Closure of the bile duct
ICD-10 online (WHO version 2019)

When idiopathic recurrent cholestasis (Summer Skill Tygstrup) is a rare autosomal recessive genetic disorder with intermittently occurring intrahepatic obstructive jaundice in children and young adults. The prognosis is relatively good. This type of cholestasis falls under the familial hyperbilirubinemia syndrome with an increase in direct bilirubin in the blood. Clinical signs include a. Jaundice (jaundice) in the sclera and mucous membranes, and if more severe, also clearly in the skin. In addition, typical symptoms of jaundice such as tiredness, loss of appetite, nausea, itching and tenderness in the right upper abdomen (enlarged liver) appear.

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