Background and Objectives: Primary sclerosing cholangitis (PSC) is a rare cholestatic disease of the liver of unknown etiology, severe course and poor prognosis. PSC most often co-occurs with inflammatory bowel diseases (IBD), especially with ulcerative colitis (UC). The aim of the study was the analysis of the clinical course of primary sclerosing cholangitis in children, hospitalized in the Gastroenterology Unit in Katowice. Materials and Methods: The analysis included 30 patients, aged from 7 to 18 years, 21/30 boys (70%) and 9/30 girls (30%), diagnosed with PSC in the years 2009–2019. The analysis included the age at diagnosis, clinical symptoms, course of the disease, coexisting diseases, laboratory and imaging results, and complications. Results: The average age at diagnosis was 13 years. 22/30 (73.3%) patients suffered from UC, 4/30 (13.3%) were diagnosed with Crohn’s disease (CD), 2/30 (6.66%) with Eosinophilic Colitis (EC). 2/30 patients (6.66%) had no clinical evidence of coexistent IBD to date. In addition, 7/30 (23.3%) had an overlap syndrome of primary sclerosing cholangitis/autoimmune hepatitis. When PSC was detected before IBD (6/30–20%), patients had complications more often compared to those diagnosed with IBD first or PSC and IBD at the same time. At the moment of diagnosis 6/30 (20%) patients presented with abdominal pain, which was the most common symptom, 3/30 (10%) jaundice, while 17/30 (56.6%) were asymptomatic but had abnormal results of the laboratory tests. Conclusions: Monitoring liver markers in IBD patients is important since most PSC cases are asymptomatic and their elevation might be the first sign of the disease. Patients diagnosed with PSC before IBD diagnosis are more likely to have a more aggressive course of the disease.
Background Primary sclerosing cholangitis (PSC) is a rare liver cholestatic disease with unknown aetiology, severe clinical course and poor prognosis. PSC most often co-occurs with inflammatory bowel diseases (IBD), especially ulcerative colitis (UC). The aim of the study. We aimed to analyse the clinical course of patients diagnosed with PSC hospitalised in the Department of Gastroenterology, Medical University of Silesia in Katowice in 2009–2018. Methods The analysis included 30 patients, aged from 7 to 18 years (mean age 13.5 years), 21/30 boys (70%) and 9/30 girls (30%), diagnosed with PSC. The analysis included age of diagnosis, clinical symptoms, course of the disease, laboratory and imaging results and complications. Results The average diagnosis age was 13 years. 22/30(73.3%) patients suffered from UC and 4/30 (13.3%) were diagnosed with Crohn’s disease. 2/30 (6.66%) patients remained IBD-free. Among them 7/30(23.3%) had an overlap syndrome—primary sclerosing cholangitis and autoimmune hepatitis. At the time diagnosis 6/30 (20%) patients presented abdominal pain, which was the most common symptom, while 16/30 (57%) were asymptomatic but had abnormal laboratory test results. When PSC was detected before IBD, patients had complications more often compared with those diagnosed with IBD first or PSC and IBD at the same time. Moreover, complications were observed more often in patients with changes characteristic of PSC visible in their first USG than in patients without mentioned abnormalities in first USG. Conclusion Patients diagnosed with PSC before IBD diagnosis are more likely to have a more aggressive course of the disease. Monitoring liver markers in IBD patients is important since most PSC cases are asymptomatic and their elevation might be the first sign of the disease.
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