Browsing by Author "Erden, Esra"
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Item CD40 Ligand Deficiency With Grade III Liver Fibrosis, Transplanted by a Treosulphan-based Conditioning Regimen(Başkent Üniversitesi, 2011-10) Dogu, Figen; Ikinciogullari, Aydan; Erden, Esra; Reisli, Ismail; Cipe, Funda ErolX-linked Hyper IgM is characterized by an absence of the CD40 ligand on activated T lymphocytes resulting in defects of both cellular and humoral immunity. Patients usually present with recurrent bacterial and opportunistic infections. Chronic liver disease is seen in about 75% of patients as a complication. Here, we report a 3.5-year-old boy with X-linked Hyper IgM referred to our clinic for bone marrow transplant. He was transplanted from an HLA-identical sibling donor using a new conditioning agent, treosulphan, together with cyclophosphamide. Since 6 months of age, he has had recurrent respiratory infections, and his XHIGM was diagnosed when he was 1.5 years old. The diagnosis was confirmed by sequence analysis of the CD40L gene. On physical examination, growth failure, bilateral fine crackles in both lungs, and hepatosplenomegaly were detected. The results of his liver function tests were abnormal, and a liver biopsy showed grade III fibrosis and compensated cirrhosis. After conditioning with treosulphan (12 g/m2/d × 3 d) and cyclophosphamide (50 mg/kg/d × 4 d), bone marrow from his HLA-identical sister was infused. CD40L expression on activated lymphocytes of the patient was 84% on day +21. His posttransplant period was uneventful. He is now at posttransplant 2 years, with full donor chimerism, and mild, chronic, graft-versus-host disease on his tongue. In conclusion, treosulphan is a new agent for conditioning regimen with less toxicity in patients with severe liver disease.Item A Retrospective Evaluation of the Epithelial Changes/Lesions and Neoplasms of the Gallbladder in Turkey and a Review of the Existing Sampling Methods: A Multicentre Study(2018) Ozgun, Gonca; Esendaglı, Guldal; Akarca, F. Goknur; Balci, Serdar; Argon, Asuman; Sengiz Erhan, Selma; Turhan, Nesrin; Ince Zengin, Neslihan; Hallac Keser, Sevinc; Celik, Betul; Bulut, Tangul; Abdullazade, Samir; Erden, Esra; Savas, Berna; Bostan, Temmuz; Sagol, Ozgul; Aysal Agalar, Anil; Kepil, Nuray; Karslioglu, Yildirim; Gunal, Armagan; Markoc, Fatma; Saka, Burcu; Ozdamar, Sukru Oguz; Bahadir, Burak; Kaymaz, Esin; Isik, Emre; Ayhan, Semin; Tuncel, Deniz; Ozguven Yilmaz, Banu; Celik, Sevinc; Karabacak, Tuba; Erbarut Seven, Ipek; Ataizi Celikel, Cigdem; Gucin, Zuhal; Ekinci, Ozgur; Akyol, Gulen; 28984336Objective: As there is continuing disagreement among the observers on the differential diagnosis between the epithelial changes/lesions and neoplasms of the gallbladder, this multicentre study was planned in order to assess the rate of the epithelial gallbladder lesions in Turkey and to propose microscopy and macroscopy protocols. Material and Method: With the participation of 22 institutions around Turkey that were included in the Hepato-Pancreato-Biliary Study Group, 89,324 cholecystectomy specimens sampled from 2003 to 2016 were retrospectively evaluated. The numbers of adenocarcinomas, dysplasias, intracholecystic neoplasms/adenomas, intestinal metaplasias and reactive atypia were identified with the review of pathology reports and the regional and countrywide incidence rates were presented in percentages. Results: Epithelial changes/lesions were reported in 6% of cholecystectomy materials. Of these epithelial lesions, 7% were reported as adenocarcinoma, 0.9% as high-grade dysplasia, 4% as low-grade dysplasia, 7.8% as reactive/regenerative atypia, 1.7% as neoplastic polyp, and 15.6% as intestinal metaplasia. The remaining lesions (63%) primarily included non-neoplastic polypoids/hyperplastic lesions and antral/pyloric metaplasia. There were also differences between pathology laboratories. Conclusion: The major causes of the difference in reporting these epithelial changes/lesions and neoplasms include the differences related to the institute's oncological surgery frequency, sampling protocols, geographical dissimilarities, and differences in the diagnoses/interpretations of the pathologists. It seems that the diagnosis may change if new sections are taken from the specimen when any epithelial abnormality is seen during microscopic examination of the cholecystectomy materials.