{"id":3236,"date":"2026-09-04T11:18:19","date_gmt":"2026-09-04T08:18:19","guid":{"rendered":"https:\/\/pediatria-spisanie.eu\/2026\/09\/04\/primary-sclerosing-cholangitis-in-pediatric-patients-a-case-series\/"},"modified":"2026-09-04T17:20:23","modified_gmt":"2026-09-04T14:20:23","slug":"primary-sclerosing-cholangitis-in-pediatric-patients-a-case-series","status":"publish","type":"post","link":"https:\/\/pediatria-spisanie.eu\/en\/2026\/09\/04\/primary-sclerosing-cholangitis-in-pediatric-patients-a-case-series\/","title":{"rendered":"Primary sclerosing cholangitis in pediatric patients \u2013 a case series"},"content":{"rendered":"\n

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\n\nP. Hadzhiyski1,2<\/sup>, \nHr. Naydenov1,2<\/sup>, \nEm. Nedeva-Dobreva1,2<\/sup>, \nP. Nikolov1<\/sup>, \nM. Penkov3<\/sup>, \nD. Kyoseva4<\/sup>, \nM. Baycheva1,2<\/sup>\n<\/strong>\n<\/p>\n\n

\n1<\/sup> Department of Gastroenterology, University Children\u2019s Hospital \u201cProf. Ivan Mitev\u201d, Sofia, Bulgaria
\n2<\/sup> Department of Pediatrics, Medical University of Sofia, Bulgaria
\n3<\/sup> Department of Radiology, University Hospital \u201cSaint Ivan Rilski\u201d, Sofia, Bulgaria
\n4<\/sup> Department of General and Clinical Pathology, UMBAL \u201cAlexandrovska\u201d, Sofia, Bulgaria\n<\/p>\n\n\t\t<\/div>\n\t<\/div>\n\t\n\t\n\n

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Abstract<\/h2>\n\n\n\n

Sclerosing cholangitis (SC) is a heterogeneous group of diseases with similar clinical and histopathological characteristics \u2013 fibrosis, strictures of the bile ducts and cholestasis. When the cause of SC is not known, the diagnosis is established as primary sclerosing cholangitis (PSC). PSC is often associated with inflammatory bowel disease (IBD) in 50-80% of the cases. In 25-40% of all cases, the condition is associated with autoimmune hepatitis (AIH). The gold standard for diagnosis is magnetic resonance cholangiopancreatography (MRCP). We present a case series of pediatric patients with PSC with different clinical presentation, disease severity and progression. Treatment of patients includes ursodeoxycholic acid, corticosteroids and immunomodulator with variable therapeutic success. The incidence of PSH in children is low. The disease may be oligosymptomatic at first and therapeutic options are limited. Future studies are needed to clarify the treatment of the disease and improve the quality of life of patients. The aim of this publication is to present the clinical features, diagnostic difficulties, and therapeutic approach in four children with primary sclerosing cholangitis.<\/p>\n\n\n\n

Keywords<\/strong><\/strong>: primary sclerosing cholangitis, magnetic resonance cholangiopancreatography, autoimmune hepatitis<\/em><\/p>\n\n\n

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20. \u041a\u043e\u0444\u0438\u043d\u043e\u0432\u0430 \u0414. \u041e\u0446\u0435\u043d\u043a\u0430 \u043d\u0430 \u0444\u0438\u0431\u0440\u043e\u0437\u0430\u0442\u0430 \u043f\u0440\u0438 \u0445\u0440\u043e\u043d\u0438\u0447\u043d\u0438 \u0447\u0435\u0440\u043d\u043e\u0434\u0440\u043e\u0431\u043d\u0438 \u0437\u0430\u0431\u043e\u043b\u044f\u0432\u0430\u043d\u0438\u044f. \u0414\u0438\u0441\u0435\u0440\u0442\u0430\u0446\u0438\u043e\u043d\u0435\u043d \u0442\u0440\u0443\u0434 \u0437\u0430 \u043f\u0440\u0438\u0441\u044a\u0436\u0434\u0430\u043d\u0435 \u043d\u0430 \u043e\u0431\u0440\u0430\u0437\u043e\u0432\u0430\u0442\u0435\u043b\u043d\u0430 \u0438 \u043d\u0430\u0443\u0447\u043d\u0430 \u0441\u0442\u0435\u043f\u0435\u043d \u201e\u0434\u043e\u043a\u0442\u043e\u0440\u201c, \u041c\u0435\u0434\u0438\u0446\u0438\u043d\u0441\u043a\u0438 \u0443\u043d\u0438\u0432\u0435\u0440\u0441\u0438\u0442\u0435\u0442 \u2013 \u0421\u043e\u0444\u0438\u044f; 2020, 1-146.<\/p>\n\n\n\n

21. Razumilava N, Gores GJ, Lindor KD. Cancer surveillance in patients with primary sclerosing cholangitis. Hepatology 2011;54:1842\u20131852.<\/p>\n\n\n

<\/div>\n\n\n\n

Address for correspondence:<\/strong><\/p>\n\n\n\n

Assoc. Prof. Petyo Hadjiiski, MD, PhD<\/p>\n\n\n\n

Department of Pediatric Gastroenterology,
Specialized Hospital for Children\u2019s Diseases \u201cProf. Ivan Mitev\u201d, Sofia<\/p>\n\n\n\n

Department of Pediatrics,
Medical University \u2013 Sofia<\/p>\n\n\n\n

11 Acad. Ivan Evstratiev Geshov Blvd., 1606 Sofia, Bulgaria<\/p>\n\n\n\n

E-mail: dr.hadjiiski@abv.bg<\/a><\/p>\n\n\n\n

<\/p>\n\n\n\n

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<\/p>\n","protected":false},"excerpt":{"rendered":"

P. Hadzhiyski\u00b9\u02d2\u00b2, Hr. Naydenov\u00b9\u02d2\u00b2, Em. Nedeva-Dobreva\u00b9\u02d2\u00b2, P. Nikolov\u00b9, M. Penkov\u00b3, D. Kyoseva\u2074, M. Baycheva\u00b9\u02d2\u00b2 ||| \u00b9 Department of Gastroenterology, University Children\u2019s Hospital \u201cProf. Ivan Mitev\u201d, Sofia, Bulgaria ||| \u00b2 Department of Pediatrics, Medical University of Sofia, Bulgaria ||| \u00b3 Department of Radiology, University Hospital \u201cSaint Ivan Rilski\u201d, Sofia, Bulgaria ||| \u2074 Department of General and Clinical Pathology, UMBAL \u201cAlexandrovska\u201d, Sofia, Bulgaria<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_pediatria_doi":"10.66223\/pediatria.2026.3.008","footnotes":""},"categories":[1301,2684,3399,2679,1],"tags":[3355,3354,3353,3348,3352,3356,3443,3358,1230,3326,3351,3357,3447,3360,3350,3349,3450,3359],"class_list":["post-3236","post","type-post","status-publish","format-standard","hentry","category-articles","category-issue-3-2026","category-broj-3-2026","category-statii","tag-autoimmune-hepatitis","tag-magnetic-resonance-cholangiopancreatography","tag-primary-sclerosing-cholangitis","tag-primary-sclerosing-cholangitis-in-pediatric-patients-a-case-series","tag-avtoimunen-hepatit","tag-d-koseva4","tag-em-nedeva-dobreva1","tag-m-bajcheva1","tag-m-penkov3","tag-magnitno-rezonansna-holangiopankreatografiya","tag-p-nikolov1","tag-p-hadzhijski1","tag-parvichen-sklerozirasth-holangit","tag-parvichen-sklerozirasth-holangit-v-detska-vazrast-klinichni-sluchai","tag-hr-najdenov1"],"_links":{"self":[{"href":"https:\/\/pediatria-spisanie.eu\/en\/wp-json\/wp\/v2\/posts\/3236","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/pediatria-spisanie.eu\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/pediatria-spisanie.eu\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/pediatria-spisanie.eu\/en\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/pediatria-spisanie.eu\/en\/wp-json\/wp\/v2\/comments?post=3236"}],"version-history":[{"count":4,"href":"https:\/\/pediatria-spisanie.eu\/en\/wp-json\/wp\/v2\/posts\/3236\/revisions"}],"predecessor-version":[{"id":3245,"href":"https:\/\/pediatria-spisanie.eu\/en\/wp-json\/wp\/v2\/posts\/3236\/revisions\/3245"}],"wp:attachment":[{"href":"https:\/\/pediatria-spisanie.eu\/en\/wp-json\/wp\/v2\/media?parent=3236"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/pediatria-spisanie.eu\/en\/wp-json\/wp\/v2\/categories?post=3236"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/pediatria-spisanie.eu\/en\/wp-json\/wp\/v2\/tags?post=3236"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}