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Novel variant syndrome associated with congenital hepatic fibrosis

World Journal of Clinical Cases 2015年第10期3卷 904-910页

作者： Yusuf Bayraktar Ozlem Yonem Kubilay Varl? Hande Taylan Ali Shorbagi cenk sokmensuer Department of Gastroenterology Faculty of MedicineHacettepe University Department of Gastroenterology Cumhuriyet University Department of Neurology Hacettepe University Department of Ophthalmology Hacettepe University Department of Pathology Hacettepe University

Congenital hepatic fibrosis is part of many different malformation syndromes, of which oculo-encephalohepato-renal syndrome is the most common. These syndromes largely overlap, and so accurate classification of individual patients may be difficult. We present herein three syndromic siblings who were products of a consanguineous marriage. We investigated in detail at least six organ systems in these patients, namely the liver, brain, eye, kidneys, skeleton, and gonads. The common features observed in these three cases were congenital hepatic fibrosis, retinitis pigmentosa, truncal obesity, rotatory nystagmus, mental retardation, advanced myopia, and high-arched palate. The clinical dysmorphology in these patients was distinct and lacked the major features of the known syndromes associated with congenital hepatic fibrosis. Although some features of these presented cases are similar to those found in Bardet-Biedl syndrome(BBS), the absence of some major criteria of BBS(polydactyly, renal abnormality, and hypogonadism) suggests that this may be a new syndrome. All three patients remain under follow-up in the departments of Gastroenterology, Ophthalmology, and Neurology at Hacettepe University.

关键词： Congenital hepatic fibrosis Nystagmus Mental retardation Retinitis pigmentosa High-arched palate

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Comprehensive clinicopathologic characteristics of intraabdominal neurogenic tumors:Single institution experience

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World Journal of Clinical Cases 2021年第10期9卷 2218-2227页

作者： Cem Simsek Meral Uner Feride Ozkara Orkun Akman Aytekin Akyol Taylan Kav cenk sokmensuer Gokhan Gedikoglu Department of Gastroenterology Hacettepe UniversityAnkara 06230Turkey Department of Pathology Hacettepe UniversityAnkara 06230Turkey Department of Internal Medicine Hacettepe UniversityAnkara 06230Turkey Department of Pathology Yozgat City HospitalYozgat 66100Turkey

BACKGROUND Neurogenic tumors are rare but represent an important consideration in the differential diagnosis of abdominal mesenchymal *** on their incidence,pathological features and clinical characteristics are *** To advance the overall knowledge on the histologic,immunohistochemical,clinical and radiologic characteristics of neurogenic tumors through this case *** An established database of a nationwide tertiary referral center,covering a 15-year period(2005 and 2020),was retrospectively *** of neurogenic tumor cases were confirmed by two experts following review of the macroscopic,histological and immunohistochemical records along with findings from analysis of archived tissue sections for each included *** microarrays were constructed for cases lacking necessary immunohistochemical *** data and follow-up information were collected from the hospital records and the patients themselves,when *** The study included 19 cases of intraabdominal neurogenic tumors,representing 12 women and 7 men, between 18 and 86 years of age (median: 51 years). Finalconfirmed diagnoses were 12 schwannomas, 2 diffuse submucosal neurofibromatoses,2 ganglioneuromas, 2 malignant peripheral sheath nerve tumors,and 1 mucosal Schwann cell hamartoma. Sizes of the tumors were variable, with amedian diameter of 4 cm;the two largest (> 10 cm) were schwannomas. Themajority of cases were asymptomatic at presentation, but the most frequentsymptom was abdominal pain. Gastrointestinal tract lesions were detected withendoscopy and extra-luminal lesions were detected with cross-sectional *** cases were S100-positive and CD117-negative;most cases were negative fordesmin, epithelial membrane antigen, smooth muscle actin and CD34. In all but 5cases, the Ki67 proliferation index was ≤ 1%.CONCLUSION Re-evaluation of 19 cases of abdominal neurogenic tumors demonstrated considerablevariability in clinicopathologic ch

关键词： Neurogenic tumors Mesenchymal tumors Schwannoma Neurofibroma Malignant peripheral sheath tumor Intraabdominal

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Outcomes of partial splenic embolization in patients with massive splenomegaly due to idiopathic portal hypertension

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World Journal of Gastroenterology 2016年第43期22卷 9623-9630页

作者： Omer Ozturk Gonca Eldem Bora Peynircioglu Taylan Kav Aysegul Gormez Barbaros Erhan Cil Ferhun Balkanci cenk sokmensuer Yusuf Bayraktar Department of Gastroenterology Hacettepe University School of Medicine06100 Ankara Turkey Department of Radiology Hacettepe University School of Medicine06100 Ankara Turkey Department of Pathology Hacettepe University School of Medicine06100 Ankara Turkey

AIM To determine the outcomes of partial splenic em-bolization(PSE) for massive splenomegaly due to idiopathic portal hypertension(IPH).METHODS In this prospective study, we evaluated the charac-teristics and prognosis of consecutive patients with IPH who underwent PSE for all indications at a single medical center between June 2009 and January 2015. The inclusion criteria were: presence of hypersplenism, massive splenomegaly, and resultant pancytopenia. The exclusion criteria were: presence of other diseases causing portal hypertension. During the post-PSE period, the patients were hospitalized. All patients underwent abdominal computed tomography imaging 4 wk post-PSE to determine total splenic and non-infarcted splenic *** A total of 11 patients, with median age of 33.27 ± 4.8 years, were included in the study. Mean spleen size was 22.9 cm(21-28 cm), and severe hypersplenismwas diagnosed in all patients before PSE. Post-PSE, leukocyte and platelet counts increased significantly, reaching peak levels in the second week with gradual decreases thereafter. Liver function tests did not exhibit significant changes during post-intervention follow-up. All patients developed post-embolization syndrome, and one patient experienced serious complications; all complications were successfully treated with conservative therapy and no death occurred. CONCLUSION Our findings showed that PSE has a lower complication rate than previously-reported surgical complication rates, which supports this intervention as a viable alternative for high-risk operable patients with severe hypersplenism.

关键词： Partial splenic embolization Idiopathic portal hypertension Hypersplenism Massive splenomegaly

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