Sabina Wiecek

By Sabina Wiecek

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

By Sabina Wiecek

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

By Sabina Wiecek

Inflammatory bowel disease is a group of chronic disorders of the gastrointestinal tract, including Lesniowski-Crohn disease, ulcerative colitis, and indeterminate colitis. The most frequently occurring symptoms in patients with IBD, including ulcerative colitis, involve abdominal discomfort, recurring and often bloody diarrhoea, weight loss, and the resulting anaemia and/or cachexia. Extraintestinal manifestations of ulcerative colitis may precede the diagnosis of inflammatory bowel disease, they may also occur during remission (pyoderma gangrenosum, uveitis, spondylitis, and PSC) or accompany an exacerbation of the disease (erythema nodosum, episcleritis, aphthae, and some forms of peripheral spondyloarthritis). This study focuses on the most common extraintestinal manifestations and complications in ulcerative colitis in paediatric patients.

Part of the book: Ulcerative Colitis

By Sabina Wiecek and Anna Buczynska

In recent years, an increase in the incidence of Inflammatory Bowel Disease has been observed, with particular emphasis on Crohn’s disease and ulcerative colitis in the paediatric population, also in the youngest age group. Underlying factors are genetic, environmental (including the microbiome) and immunological. Clinical manifestations are very often uncharacteristic, and the clinical picture is dominated by abdominal pain, weight/growth deficiency and/or diarrhoea. Similar symptoms occur in the course of lactose intolerance and small intestinal bacterial overgrowth (SIBO). On the other hand, Inflammatory Bowel Diseases (IBD) seem to favour the onset of SIBO and lactose intolerance. Only the diagnosis of these disease entities ensures appropriate therapeutic management. The manuscript analyses the latest literature on the co-occurrence of these disease entities in patients with IBD—especially Crohn’s disease and ulcerative colitis, clinical symptoms, and diagnostic and therapeutic procedures.

Part of the book: Miscellaneous Considerations in Inflammatory Bowel Disease [Working title]