Browsing by Author "Ozen, S."
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Article A Case of Langerhans Cell Histiocytosis With Anal Fistula(Kurume University School of Medicine, 2010) Akbayram, S.; Akgun, C.; Ozen, S.; Kaya, A.; Tuncer, O.; Yuca, S.A.; Oner, A.F.Langerhans cell histiocytosis (LCH) is an uncommon clinically heterogeneous disorder characterized by the proliferation and accumulation of Langerhans cells with local infiltration of tissues and organ destruction. LCH takes many clinical forms, affecting different systems and different sites in the same system with variable outcomes. Bone, skin, lymph node, pituitary, liver, lung, bone marrow and spleen involvement can be seen in patients with LCH. Involvement of the perianal site is rare. In this article, a 16-month-old boy with multiple organ involvement including skin, liver, lung, and bone is presented. Aside from these systemic involvements, he also had a simple anal fistula. According to our best knowledge, this case of LCH with anal fistula is only the second to be reported in childhood. We would like to emphasize that LCH may be associated with anal fistula; therefore, we suggest that patients with LCH should be examined for this condition.Article Effects of Hyaluronic Acid on Postoperative Adhesion of Tendo Calcaneus Surgery: an Experimental Study in Rats(2002) Tuncay, I.; Ozbek, H.; Atik, B.; Ozen, S.; Akpinar, F.Adhesions are a significant problem after tendon surgery. The effects of hyaluronic acid on adhesion formation of the tendo calcaneus were investigated in this study. Twenty Wistar rats were utilized. Both tendo calcanei were incised transversely, and then repaired. Hyaluronic acid (0.2 cc) was injected into peritendinous tissue on the right side, while the same amount of normal saline was injected to the left side as a control. The animals were sacrificed 40days after the experiment. Both the right and left tendon adhesions were evaluated both macroscopically and microscopically for the presence of adhesions (grading scale 0-4). Throughout the experimental period, there was no difference in range of motion of the ankle between the two groups. Macroscopically, there were fewer adhesions in the experimental group (mean 0.6 ± 0.8) compared to the controls (mean 1.1 ± 0.2). This difference was not statistically significant (p =. 096). Histopathologically, these parameters were similar in both the experimental (mean 1.15 ± 0.98) and the control groups (mean 1.9 ± 1.25). This difference was significant (p =. 043). Hyaluronic acid may be effective for prevention of adhesions in the tendo calcaneus though this effect could not be demonstrated experimentally.Article Natural History of Congenital Generalized Lipodystrophy: a Nationwide Study From Turkey(Endocrine Society, 2016) Akinci, B.; Onay, H.; Demir, T.; Ozen, S.; Kayserili, H.; Akinci, G.; Garg, A.Context: Congenital generalized lipodystrophy (CGL) is a rare autosomal recessive disorder characterized by near-total lack of body fat. Objective: We aimed to study natural history and disease burden of various subtypes of CGL. Design: We attempted to ascertain nearly all patients with CGL in Turkey. Setting: This was a nationwide study. Patients or Other Participants: Participants included 33 patients (22 families) with CGL and 30 healthy controls. Main Outcome Measure(s): We wanted to ascertain genotypes by sequencing of the known genes. Whole-body magnetic resonance imaging was used to investigate the extent of fat loss. Metabolic abnormalities and end-organ complications were measured on prospective follow-up. Results: Analysis of the AGPAT2 gene revealed four previously reported and four novel mutations (CGL1; c.144C-A, c.667-705delinsCTGCG, c.268delC, and c.316<1G-T). Analysis of the BSCL2 gene revealed four different homozygous and one compound heterozygous possible disease-causing mutations (CGL2), including four novel mutations (c.280C-T, c.631delG, c.62A-T, and c.465-468delGACT).Twohomozygous PTRFmutations(c.481-482insGTGAandc.259C-T)wereidentified (CGL4). Patients with CGL1 had preservation of adipose tissue in the palms, soles, scalp, and orbital region, and had relatively lower serum adiponectin levels as compared to CGL2 patients. CGL4 patients had myopathy and other distinct clinical features. All patients developed various metabolic abnormalities associated with insulin resistance. Hepatic involvement was more severe in CGL2. End-organ complications were observed at young ages. Two patients died at age 62 years from cardiovascular events. Conclusions: CGL patients from Turkey had both previously reported and novel mutations of the AGPAT2, BSCL2, and PTRF genes. Our study highlights the early onset of severe metabolic abnormalities and increased risk of end-organ complications in patients with CGL. © 2016 by the Endocrine Society.Article Ovarian Gonodoblastoma With Yolk Sac Tumor in a Young 46, Xx Female: Case Report(I R O G Canada, inc, 2007) Erdemoglu, E.; Ozen, S.Background: Gonodoblastomas with ovarian germ cell tumors (OGCTs) other than dysgerminoma are very unusual. This is the first case report of a pure endodermal sinus tumor of the ovary with gonodoblastoma in a 46 XX, female. Case: A 19-year-old female was admitted to our hospital with an abdominal mass and pain. She had elevated levels of CA-125 and AFP Fertility sparing surgery was undertaken and revealed a Stage IA endodermal sinus tumor and gonodoblastoma in the same ovary. The patient refused chemotherapy and was followed-up for six months without disease. Conclusion: Gonodoblastomas may occur in conjunction with OGCTs. Tumor markers and immunohistochemical examination may help in the diagnosis of these OGCTs with gonodoblastoma.