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This syndrome can affect the liver, heart, skeleton, eyes, kidneys, and central nervous system. Liver involvement is one of the main clinical features, consisting of chronic cholestasis due to paucity of interlobular bile ducts [1]. Progressive liver disease, leading to cirrhosis occurs in approximately 15% of cases and may require liver transplantation (LT) [2]. These patients are at risk for hepatocellular carcinoma, therefore early detection of liver tumors are critical in the management of this disease. We describe the case Forskolin purchase of a patient with known Alagille syndrome who developed severe liver cirrhosis. Pre-transplant evaluation disclosed a nodular lesion in the segment 4 which corresponded to a focal hyperplasia learn more in advanced cirrhosis. 2.?Presentation of case A 15 year-old girl was referred to our department for evaluation for LT. The diagnosis was suspected at the age of 2 months in the presence of jaundice due to cholestasis. Liver biopsy disclosed paucity of the intrahepatic bile ducts. A pulmonary stenosis was further highlighted and treated percutaneously. Moreover, the patient exhibited the typical dysmorphic facies with a prominent forehead, deep set eyes and pointed chin giving the face a triangular appearance associated with thoracic vertebral segmentation anomalies called ��butterfly�� vertebrae. In presence of these clinical features, the diagnosis of Alagille syndrome was ascertained. The patient��s mother did not have the same physical features. We did not disclose ophthalmic and renal anomalies. Pruritus was successfully treated with ursodeoxycolic acid and rifampicin, however, liver disease worsened gradually until decompensated cirrhosis at the age of 14 year-old and then refractory ascites. At the time of evaluation, laboratory tests were as follow: elevated Sitaxentan conjugated bilirubin?=?290?��mol/l (normal value