Articles, Issue 3/2026, Брой 3/2026, Брой 3/2026, Articles

Primary sclerosing cholangitis in pediatric patients – a case series

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DOI: 10.66223/pediatria.2026.3.008

P. Hadzhiyski1,2, Hr. Naydenov1,2, Em. Nedeva-Dobreva1,2, P. Nikolov1, M. Penkov3, D. Kyoseva4, M. Baycheva1,2

1 Department of Gastroenterology, University Children’s Hospital “Prof. Ivan Mitev”, Sofia, Bulgaria
2 Department of Pediatrics, Medical University of Sofia, Bulgaria
3 Department of Radiology, University Hospital “Saint Ivan Rilski”, Sofia, Bulgaria
4 Department of General and Clinical Pathology, UMBAL “Alexandrovska”, Sofia, Bulgaria

Abstract

Sclerosing cholangitis (SC) is a heterogeneous group of diseases with similar clinical and histopathological characteristics – 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.

Keywords: primary sclerosing cholangitis, magnetic resonance cholangiopancreatography, autoimmune hepatitis

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Address for correspondence:

Assoc. Prof. Petyo Hadjiiski, MD, PhD

Department of Pediatric Gastroenterology,
Specialized Hospital for Children’s Diseases “Prof. Ivan Mitev”, Sofia

Department of Pediatrics,
Medical University – Sofia

11 Acad. Ivan Evstratiev Geshov Blvd., 1606 Sofia, Bulgaria

E-mail: dr.hadjiiski@abv.bg