Background: Inflammatory bowel diseases with an early-onset form (EO-IBDs) make up a special disease group with certain clinical and phenotypic characteristics. This article discusses the features of such early onset in a group of children, based on five years of monitoring a registry of children with IBD from a specialized center. Methods: This retrospective single-center cohort study included pediatric patients diagnosed with EO-IBD between 2019 and 2024. Clinical, laboratory, and endoscopic data were collected from medical records, including fecal calprotectin, inflammatory markers, disease activity indices, and endoscopic severity scores. Localization was classified according to the Paris system, and histological activity was assessed using the IBD-DCA score. Results: There were 20 patients with ulcerative colitis (UC) and 17 with Crohn’s disease (CD). Clinical activity was moderate or high (p = 0.179). UC was more characterized by diarrhea and rectal bleeding. CD was more often accompanied by abdominal pain, weight loss, and fever. In total, 82.4% of patients with CD had an inflammatory form. UC-like intestinal lesion was typical of both nosologies—L3 64.7% and E4 60% forms in CD and UC, respectively. Morphological activity was moderate for both nosologies (p = 0.54). IBD-U was present in 43.2% of patients. The median time after which it was possible to diagnose UC was 24 weeks (IQR 20–48) and 40 weeks (IQR 30–45.5) for CD (p = 0.56). Conclusions: Our study confirms the presence of characteristic signs of EO-IBD development, such as a frequent family history of IBD, high or moderate clinical activity during diagnosis verification, colon damage, and a high frequency of extraintestinal manifestations.

Aim: to present a clinical observation of paraneoplastic manifestations of Hodgkin’s lymphoma — liver damage in the development of vanishing bile duct syndrome.Clinical case. Patient Sh., 18-years-old female, was admitted with complaints of yellowing of the skin and sclera, severe general weakness, and fever. Mechanical jaundice, viral hepatitis, Wilson — Konovalov disease, autoimmune hepatitis, and infections were excluded. A hypothesis was put forward about drug-induced hepatitis; some improvement was noted against the background of therapy with ursodeoxycholic acid and prednisolone, but fever and neutrophilic leukocytosis persisted. Physical examination and imaging revealed enlarged lymph nodes in various groups on both sides of the diaphragm, enlarged liver and spleen. PET-CT revealed active accumulation of 18F-fluorodeoxyglucose in the enlarged lymph nodes and bone marrow; lymphoproliferative disease was suspected. During follow- up, laboratory signs of cytolysis and cholestasis persisted, severe jaundice, decreased liver protein-synthetic function, and signs of portal hypertension were noted. A liver biopsy was performed, which revealed ductopenia in most portal tracts, without signs of inflammation (vanishing bile duct syndrome). The presence of splenomegaly, supra- and subdiaphragmatic lymphadenopathy contradicted the diagnosis of “drug-induced cholestasis”. A septic process was excluded. According to the examination, including histological examination of the enlarged lymph node, Hodgkin’s lymphoma, nodular sclerosis, with damage to the supra- and subdiaphragmatic lymph nodes, liver (“vanishing bile duct syndrome” — paraneoplastic reaction) and spleen, stage IIIB according to the Ann Arbor classification, were diagnosed. After polychemotherapy, the fever resolved, and laboratory parameters showed significant positive dynamics. Control PET-CT did not reveal foci of pathological accumulation of 18F-fluorodeoxyglucose. The patient continues to take ursodeoxycholic acid.Conclusion. Liver involvement in Hodgkin’s lymphoma may manifest as vanishing bile duct syndrome, which is essentially a manifestation of paraneoplastic syndrome. Achieving complete remission and ursodeoxycholic acid therapy are considered to be the key to resolving ductopenia.