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    Hepatocellular adenomas in the Turkish population: reclassification according to updated World Health Organization criteria
    (Wiley, 2021) Deniz, Kemal; Umetsu, Sarah E.; Ferrell, Linda; Yilmaz, Funda; Gulluoglu, Mine; Sagol, Ozgul; Dogusoy, Gulen B.
    Aims Hepatocellular adenoma (HCA) is an uncommon liver neoplasm, and studies of HCA subtypes have been primarily limited to France, the USA, and Japan. The aim of this study was to describe the clinicopathological features of HCA subtypes in Turkey. Methods and results The resection specimens of 59 cases diagnosed as 'hepatocellular adenoma' collected from 15 institutions were reviewed to confirm the diagnosis and to classify them according to the current World Health Organization 2019 classification. Immunostaining for glutamine synthetase, liver fatty acid-binding protein, C-reactive protein, beta-catenin and reticulin was performed. Of the 59 cases, 48 (81%) were diagnosed as HCA. We identified 24 (50%) hepatocyte nuclear factor 1 alpha (HNF1 alpha)-inactivated HCAs, five (10%) inflammatory HCAs, 15 (32%) beta-catenin-activated HCAs, three (6%) beta-catenin-activated inflammatory HCAs, and one (2%) unclassified HCA. HCA patients were predominantly female (female/male ratio of 5:1); they had a median age of 34 years and a median tumour diameter of 60 mm. In the beta-catenin-activated HCA group, nine cases (19%) showed cytoarchitectural atypia, and were also referred to as atypical hepatocellular neoplasms. In the beta-catenin-activated HCA group, three cases (6%) showed focal areas supportive of transition to HCA. The original diagnosis of HCA was changed to well-differentiated hepatocellular carcinoma in nine cases and to focal nodular hyperplasia in two cases. Conclusion In our series, the major HCA subtype was HNF1 alpha-inactivated HCA. We found a low incidence of inflammatory-type HCA. Our data also showed that beta-catenin-activated hepatocellular neoplasms, including cases with atypical histology, constituted a relatively high proportion of the cases. These findings are in contrast to those of most other studies of HCA subtypes.
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    Liver transplantation in Wilson’s disease: Long-term experience of single center
    (2019) Agalar, Cihan; Ozbilgin, Mucahit; Unek, Tarkan; Egeli, Tufan; Akarsu, Mesut; Aysal, Anil; Sagol, Ozgul; Ayhan, Ziya; Astarcioglu, Ibrahim
    Aim: Liver transplantation (LT) is the most effective treatment method for preventing progressive and lethal complications of Wilson’s disease (WD). Despite the deceased donor liver transplantation (DDLT) and living donor liver transplantation (LDLT) are performed in many centers for WD, a limited number of reports were published, about long-term results. The aim of this study is to share the long-term outcomes of single center.Material and Methods: Patients who underwent LT for the WD, between 1997 and 2017 were included. Patient’s survival data, death causes, neurological symptoms and follow-up data were analyzed retrospectively.Results: Eighteen patients (8DDLT, 10LDLT) with the median age of 17.11±9.88 (6-43) were included. Donor relationship was familial in all LDLT patients. Median follow-up time was 80.57±67.59 (0.23-240.9) months and the median survival time was 173.74±25.13 months. Two patients (11.1%) died in the perioperative period (0-90 days) and totally 3 patients (16.6%) died in the postoperative 0-12 month’s period. The survival rates of the patient’s at 1-, 5- and 10 years were 83.3%, 75% and 67.3%, respectively. After LT, neurological symptoms disappeared in 6 of 7 patients and no improvement was observed in one patient. During follow-up period, chronic rejection was seen in 5 patients, 3 patients were treated with medical procedure and 2 patients died due to chronic rejection.Conclusions: Our findings are consistent with the literature, long-term survival is achieved in patients with no mortality, in the postoperative 0-12 month period and after LT, neurological symptoms are disappeared in most of the patients; according to this data, LT is an effective treatment method for the WD and complications.Keywords: Wilson disease; liver transplantation; long term results; living donor.