Primary sclerosing cholangitis during childhood and adolescence

Miloh, Tamir; Bulut, Pinar

doi:10.1002/cld.251

Cited by 2 publications

(2 citation statements)

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“…However, it must not be forgotten that MRCP may be negative in small duct PSC, as Miloh’s studies confirm. He demonstrated lesions in the MRCP of 70% of patients with PSC [ 53 , 54 ]. Transabdominal US is considered to be the first line imaging modality in children, and in the presented group it was performed in all patients, although only 32% US exams showed various bile duct abnormalities.…”

Section: Discussionmentioning

confidence: 99%

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Analysis of the Clinical Course of Primary Sclerosing Cholangitis in Paediatric Population—Single Center Study

et al. 2021

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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.

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Section: Discussionmentioning

confidence: 99%

“…In all their patients with suspected PSC, Miloh et al confirmed abnormalities in the histopathological examination of the liver, mainly in the form of varying degrees of fibrosis and cirrhosis. “Onion skin” fibrosis, characteristic for PSC was confirmed in only 26.7% [ 53 , 54 ].…”

Section: Discussionmentioning

confidence: 99%