Alagille syndrome is characterized by paucity of bile ducts leading to cholestasis and may be accompanied by renal and cardiovascular abnormalities. We report 42-years old patient with hypertension, chronic renal failure and Alagille syndrome. Ischemic nephropathy was suspected, which was not confirmed on angio-CT. It seems, that chronic renal failure in the patient was due to primary parenchymal pathology.
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