| Abstract [eng] |
Discussion: Primary sclerosing cholangitis is a chronic bile duct disease in which inflammation, fibrosis and cholestasis result in stricture of the intrahepatic and/or extrahepatic ducts. The exact cause of the disease remains unclear, but the majority of cases are closely related to inflammatory bowel disease. About half of the patients disease start with abdominal pain, jaundice, scratches and intense itching of the skin. In primary sclerosing cholangitis, elevated cholestatic liver parameters (alkaline phosphatase, γ – glutamyltransferase) are common, but hyperbilirubinemia, elevated transaminases, hypoalbuminemia and thrombocytopenia have been reported in some patients. The gold standard for diagnosing the disease is cholangiography, which gives a specific image of the "beads" in the bile ducts. Primary sclerosing cholangitis tends to progress and various complications often develop during the course of the disease: dominant bile duct strictures, cholelithiasis, increased risk of hepatobiliary tumors, cirrhosis and portal hypertension. For this reason, early diagnosis and intensive treatment, starting with drug therapy, balloon dilatation, and duct stenting, are important, but liver transplantation is the most effective and only treatment that improves patient survival. Case presentation: This paper presents a clinical case of a 35-year-old man who was admitted to hospital for ascites, encephalopathy, jaundice and shortness of breath during exercise. Significant hyperbilirubinemia, thrombocytopenia, non-transfusion-level anemia, decreased prothrombin, hypoalbuminemia and hyponatremia were observed in laboratory studies. Ascites and splenomegaly have been observed in imaging studies. The patient was diagnosed with primary sclerosing cholangitis complicated by cirrhosis, portal hypertension (refractory ascites, hepatopulmonary syndrome, hepatic encephalopathy has developed) and liver transplantation was performed. 1.5 years after the procedure, the patient's symptoms disappeared and the general condition improved significantly. |
