Liver damage observed in cystic fibrosis is a complicated process comprised of fibrogenesis, inflammation, remodeling, apoptosis, and cholestasis. Complexity of processes which take place in the liver and bile ducts in the course of this disease are not clearly defined. Changes in the liver are observed in 5–20% of patients with diagnosed cystic fibrosis; however they can increase mortality, shorten time of life, and deteriorate quality of life. The aim of the chapter was to review the risk factors, clinical symptoms, diagnostic methods, and treatment of liver changes in the course of cystic fibrosis.
Part of the book: Cystic Fibrosis
Primary sclerosing cholangitis (PSC) is a chronic liver disease of unknown aetiology affecting extrahepatic and/or intrahepatic bile ducts causing its inflammation and fibrosis with most frequent consequences including biliary cirrhosis and liver failure. The incidence of PSC in children and adolescents is 0.2 per 100,000 children per year, when in adults the reported incidence is higher and equals 0.5 to 1 in 100,000 individuals per year. PSC is more common among men and boys. The diagnosis is usually established in the second decade of life in the paediatric population with the mean age of diagnosis of 13.8 years. Many studies point out a strong correlation between IBD and PSC, especially ulcerative colitis. The prevalence of IBD among children with PSC diagnosis varies from 60 to 99%; however, the incidence of PSC is about 12% in patients with ulcerative colitis and fluctuates about 2–5% in Crohn’s disease diagnosed patients. Clinical symptoms are present in approximately half of cases and they are unspecific in many of them. Elevated liver enzymes and biochemical markers of cholestasis are sometimes the only signs of PSC. Gold standard for PSC diagnosis is magnetic resonance cholangiopancreatography (MRCP) as a non-invasive procedure comparing to endoscopic retrograde cholangiopancreatography (ERCP) which is also used in some cases. The aim of the study was to review the risk factors, clinical symptoms, diagnostic methods and treatment of paediatric patients with primary sclerosing cholangitis.
Part of the book: Hepatitis A and Other Associated Hepatobiliary Diseases