title: Clinical features, serum interleukin-6, and interferon-gamma levels of 34 turkish patients with hepatoportal sclerosis.
abstract: Hepatoportal sclerosis (HPS) is a clinical disorder of obscure pathogenesis with a variable clinical profile. The aim of the study was to summarize the clinical features of Turkish patients with HPS and to measure the serum levels of interleukin (IL)-6 and interferon (IFN)-gamma to determine the T helper cell profile in the pathogenesis. The study was conducted on 34 HPS patients (17 men, 17 women; mean age at diagnosis, 27+/-10 years) and 15 healthy controls. The clinical features of HPS patients including demographics, clinical history, laboratory, and ultrasonography findings were summarized. Serum IL-6 and IFN-gamma levels were measured by using commercially available enzyme-linked immunosorbent assay kits. Gastrointestinal bleeding was the most common dominant presenting symptom. Majority of the patients had preserved liver function tests. Serum triglyceride levels were decreased in 30%. Abdominal ultrasonography revealed well-demarcated bands of increased echogenicity surrounding the portal vein wall and sudden narrowing of the intrahepatic second-degree portal vein branches in all cases. Spontaneous shunts and/or collaterals were seen in 13 cases (37%). Extrahepatic portal vein thrombosis were seen in 7 (20%) patients after at least 5 years of disease duration. Serum levels of both IL-6 (median, 3.2 pg/mL) and IFN-gamma (median, 7.8 pg/mL) were significantly higher in HPS patients compared with the control group (median, 1 pg/mL). HPS has variable clinical profile in different geographic areas of the world. Both Th1 and 2 cells may have a role in the regulation of immune response and pathogenesis of the disease.
authors.affiliation: Department of Gastroenterology, Ankara Oncology Hospital, Ankara, Turkey.
authors.forename: Aydin Seref
authors.initials: AS
authors.lastname: Köksal
authors.forename: Seyfettin
authors.initials: S
authors.lastname: Köklü
authors.forename: Mehmet
authors.initials: M
authors.lastname: Ibiş
authors.forename: Mustafa
authors.initials: M
authors.lastname: Balci
authors.forename: Bahattin
authors.initials: B
authors.lastname: Ciçek
authors.forename: Nurgül
authors.initials: N
authors.lastname: Saşmaz
authors.forename: Burhan
authors.initials: B
authors.lastname: Sahin
mesh_terms: D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D002648:Child; D018450:Disease Progression; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006975:Hypertension, Portal / Q000097:blood* / Q000175:diagnosis / Q000453:epidemiology; D007371:Interferon-gamma / Q000097:blood*; D015850:Interleukin-6 / Q000097:blood*; D008099:Liver / Q000098:blood supply / Q000000981:diagnostic imaging / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D012598:Sclerosis / Q000097:blood / Q000453:epidemiology; D012720:Severity of Illness Index; D014421:Turkey / Q000453:epidemiology; D018608:Ultrasonography, Doppler
chemical_list: D015415:Biomarkers; D015850:Interleukin-6; D007371:Interferon-gamma