Primary Sclerosing Cholangitis in Children and Adolescents

Fagundes, Eleonora Druve Tavares; Ferreira, Alexandre Rodrígues; Hosken, Caroline Caldeira; Queiroz, Thaís Costa Nascentes

doi:10.1590/s0004-2803.201700000-50

Cited by 19 publications

(19 citation statements)

References 15 publications

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“…PSC is rare, with an incidence of 0.2 in 100000 children. 17 , 18 It is a chronic cholestatic disease featured by narrowing and destruction of the intra-hepatic and extra-hepatic bile ducts. Two PSC cases were confirmed with after deranged liver functions, abnormal U/S liver, MRCP and liver biopsy.…”

Section: Discussionmentioning

confidence: 99%

Clinical spectrum of chronic liver disease with final outcome in children at a tertiary centre: A single - centre study

Seerat

Khan

Atique³

et al. 2021

Pak J Med Sci

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Background and Objectives: Chronic liver disease (CLD) in children present a broad spectrum of symptoms. Limited resources in Paediatric Hepatology in developing countries like Pakistan present considerable challenges in investigating and treating children with chronic liver disease in a timely fashion. This study aimed to determine the spectrum and outcomes of CLD other than chronic hep B & C virus (HBV& HCV) liver disease in children. Methods: This retrospective descriptive study was conducted at the Paediatric Gastroenterology and Hepatology Department, Pakistan Kidney and Liver Institute and Research Centre in Lahore, Pakistan. The duration of the study was from August 2019 to January 2020. A total of 162 children of CLD were seen during this period of time. Of 162 there were 130 children with chronic HBV & HCV who were excluded from this study. 32 children aged 15 years or younger with chronic liver disease were included. The referrals were received from primary and secondary health care centres in different parts of the country. The data were collected from hospital electronic medical records database and then incorporated into a spreadsheet for analysis. The statistical analysis was performed by applying t-test with p value determined. Results: Of 32 children autoimmune hepatitis (n=11; 34.3%) was the most common cause for chronic liver disease referrals, followed by progressive familial intrahepatic cholestasis type-2, (n=7; 21.8%), post Kasai for biliary atresia, (n=4; 12.5%), glycogen storage disease type-1 (n=5; 15.6%), Wilson disease (n=3; 9.3%) and primary sclerosing cholangitis (n=2; 6.2%). The diagnosis was principally established with the assistance of liver ultrasound, liver biopsy, magnetic resonance cholangiopancreatography and genetic testing. Conclusion: Autoimmune hepatitis was the most common chronic liver disease. Our systematic approach, in addition to an extensive workup, helped us to diagnose and then initiate an appropriate treatment, which resulted in a more optimal outcome. Prompt referrals to tertiary centres are recommended where resources and expertise are available to reduce patient morbidity and mortality. doi: https://doi.org/10.12669/pjms.37.3.3862 How to cite this:Seerat I, Khan ED, Atique M, Aujla UI. Clinical spectrum of chronic liver disease with final outcome in children at a tertiary centre: A single - centre study. Pak J Med Sci. 2021;37(3):---------. doi: https://doi.org/10.12669/pjms.37.3.3862 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Section: Discussionmentioning

confidence: 99%

Clinical spectrum of chronic liver disease with final outcome in children at a tertiary centre: A single - centre study

Seerat

Khan

Atique³

et al. 2021

Pak J Med Sci

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“…Although chronic hepatobiliary diseases are reported as a risk factor for the occurrence of gallstones in childhood (21) , symptomatic cholelithiasis has not been reported as the presentation of PSC (4,6,10,22) . Thus, the aim of this study was the diagnosis of PSC following the initial unusual presentation with symptomatic cholelithiasis, that followed an atypical clinical course which could not be explained by cholelithiasis alone.…”

Section: Symptomatic Cholelithiasis As the Presentation Of Pediatric Primary Sclerosing Cholangitis -Case Series And Literature Reviewmentioning

confidence: 99%

Symptomatic Cholelithiasis as the Presentation of Pediatric Primary Sclerosing Cholangitis - Case Series and Literature Review

Alvarenga

Sandy

Gomez

et al. 2021

Arq. Gastroenterol.

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BACKGROUND: Primary sclerosing cholangitis (PSC) is a rare hepatobiliary disorder, whose etiology remains not fully elucidated. Given how rare PSC is in childhood, until the recent publication of a multicenter international collaboration, even data on its characteristics and natural history were scarce. Symptomatic cholelithiasis has not been previously reported as the presentation of PSC. OBJECTIVE: The aim of this study was the diagnosis of PSC following the initial unusual presentation with symptomatic cholelithiasis, that followed an atypical clinical course that could not be explained by cholelithiasis alone. A literature review was also conducted. METHODS: We conducted a retrospective chart review of three patients, who were diagnosed and/or followed at the Clinics Hospital, University of Campinas - Sao Paulo/ Brazil, between 2014 and 2020. Data analyzed included gender, age of presentation, past medical history, imaging findings, laboratory results, endoscopic evaluation, response to medical therapy and follow-up. RESULTS: Age at time of presentation with cholelithiasis varied from 10 to 12 years. In two of the cases reported, a more subacute onset of symptoms preceded the episode of cholelithiasis. Two patients were managed with cholecystectomy, not followed by any surgical complications, one patient was managed conservatively. Percutaneous liver biopsy was performed in all three cases, showing histological findings compatible with PSC. Associated inflammatory bowel disease (IBD) was not seen in any of the patients. The patients have been followed for a mean time of 3.4 years. CONCLUSION: PSC and cholelithiasis are both rare in the pediatric population. This study reports on symptomatic cholelithiasis as a presentation of PSC and raises the importance of suspecting an underlying hepatobiliary disorder in children with cholelithiasis without any known predisposing factors and/or that follow an atypical clinical course for cholelithiasis alone.

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“…Even though the highest morbidity rate concerns people aged between 20 and 40, PSC has been more frequently diagnosed in paediatric patients. (0.1–0.5/100,000/annum) [ 1 , 2 , 3 , 4 , 5 , 6 ]. The clinical picture is not characteristic.…”

Section: Introductionmentioning

confidence: 99%

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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Primary Sclerosing Cholangitis in Children and Adolescents

Cited by 19 publications

References 15 publications

Clinical spectrum of chronic liver disease with final outcome in children at a tertiary centre: A single - centre study

Clinical spectrum of chronic liver disease with final outcome in children at a tertiary centre: A single - centre study

Symptomatic Cholelithiasis as the Presentation of Pediatric Primary Sclerosing Cholangitis - Case Series and Literature Review

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

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