Person: KIYAN, GÜRSU
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KIYAN
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GÜRSU
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Publication Metadata only Early pulmonary involvement in Niemann-Pick type B disease: Lung lavage is not useful(WILEY, 2005) KARADAĞ, BÜLENT TANER; Uyan, ZS; Karadag, B; Ersu, R; Kiyan, G; Kotiloglu, E; Sirvanci, S; Ercan, F; Dagli, T; Karakoc, F; Dagli, ENiemann-Pick disease (NPD) is a rare, autosomal-recessively inherited lipid storage disease which is characterized by intracellular deposition of sphingomyelin in various body tissues. The disease is heterogeneous and classified into six groups. Pulmonary parenchymal involvement may be a feature of several subtypes of NPD, including type B. Progressive pulmonary involvement in NPD type B is a major cause of morbidity and mortality It is usually diagnosed at older ages. Only a few cases with early pulmonary involvement have been reported. In this report, a patient with NPD type B, hospitalized with the diagnosis of pneumonia at age 3 months, is presented. Following treatment for pneumonia, she continued to have persistent respiratory symptoms and became oxygen-dependent. High-resolution computed tomography of the chest revealed diffuse interstitial changes. During follow-up, the patient developed hepatosplenomegaly. Lung, liver, and bone marrow biopsies showed characteristic findings for NPD. Biochemical studies also confirmed the diagnosis, and the sphingomyelinase enzyme level of the patient was low. Unilateral lung lavage was performed in order to decrease lipid storage as a treatment modality However, there was no clinical or radiological improvement. The patient died at age 15 months due to progressive respiratory failure. Pulmonary involvement is a rare entity in early childhood in patients with NPD type B, but should be considered in the differential diagnosis of persistent interstitial lung disease. It may cause progressive respiratory failure, but the treatment options remain limited.Publication Metadata only Epididymitis in infants with anorectal malformation(2003) KIYAN, GÜRSU; Kiyan, Gürsu; Dagli, Tolga E.; Iskit, Serdar H.; Tugtepe, HalilOBJECTIVE: To document the characteristics of epididymitis in boys with anorectal malformations. METHODS: Sixty-six boys with anorectal malformation were treated between January 1990 and January 2000, in our center. Four of these boys experienced epididymitis attacks. The first three patients had rectourinary fistula and the fourth had a low type anorectal malforation without a rectourinary fistula. RESULTS: Patients were followed up for seven to nine years and epididymitis did not recur after the definitive operation in the first three patients. The fourth patient had four other episodes of epididymitis postoperatively and no reason for these episodes could be found during the further urological evaluation. Two patients had urinary tract infection and one patient had urinary tract contamination during their episodes of epididymitis. CONCLUSION: The cause of the epididymitis can be anorectal malformation with rectourethral fistula itself or another urogenital anomaly that is associated with anorectal malformation. There is a relationship between urinary tract infection and epididymitis in these patients.Publication Metadata only Outcome of Very Low and Low Birth Weight Infants with Esophageal Atresia: Results of the Turkish Esophageal Atresia Registry(GEORG THIEME VERLAG KG, 2021) KIYAN, GÜRSU; Oztan, Mustafa O.; Soyer, Tutku; Oztorun, Can, I; Firinci, Binali; Durakbasa, Cigdem U.; Dokumcu, Zafer; Gollu, Gulnur; Akkoyun, Ibrahim; Demirel, Dilek; Karaman, Ayse; Ciftci, Ilhan; Ilhan, Huseyin; Parlak, Ayse; Ozden, Onder; Comert, Hatice S. Y.; Oral, Akgun; Tekant, Gonca; Kiyan, Gursu; Erginel, Basak; Guvenc, Unal; Erdem, Ali Onur; Erturk, Nazile; Yildiz, AbdullahIntroduction The data of the Turkish Esophageal Atresia Registry (TEAR) was evaluated to define the outcome of very low birth weight (VLBW) and low BW (LWB) infants with esophageal atresia (EA). Materials and Methods The data registered by 24 centers between 2014 and 2018 were evaluated for demographic features, prenatal findings, associated anomalies, surgical treatment, and outcome. Patients were enrolled in three groups according to their BWs (VLBW <1,500g), LWB=1,500-2,500g), and normal BW (NBW; >2,500g). Results Among the 389 cases, there were 37 patients (9.5%) in the VLBW group, 165 patients (42.4%) in the LBW group, and 187 patients (48.1%) in the NBW group. Prenatal diagnosis rates were similar among the three groups (29.7, 34.5, and 24.6%, respectively). The standard primary anastomosis was achieved at a significantly higher rate in NWB cases than in the other groups ( p <0.05). In patients with tracheoesophageal fistula (TEF), patients of the NBW group had significantly higher rates of full oral feedings, when compared with VLBW and LBW cases ( p <0.05). At the end of the first year, when we evaluate all patients, the number of cases with fistula recanalization and esophageal anastomotic strictures (AS) requiring esophageal dilatation was similar among the groups. The weight and height measurements at 6 months and 1 year of age of the survivors were similar in all the groups. The overall mortality rate was significantly higher in the VLBW and LBW groups, when compared with the NBW patients, even in patients with tension-free anastomosis ( p <0.05). The incidence of the associated anomalies was 90.6% in cases with mortality, which was significantly higher than in survivors (59.6%; p <0.05). According to Spitz's classification, the survival rate was 87.1% in class I, 55.3% in class II, and 16.7% in class III. The most common causes of mortality were associated with cardiovascular diseases, pneumonia, and sepsis. Conclusion The national data of TEAR demonstrates that the developmental and feeding parameters are better in NBW patients. Although VLBW patients have higher risk of developing fistula canalization than the LBW and NBW groups, long-term complications, such as anastomotic strictures, weight, and height values, after 1 year are similar in both groups. According to our results, associated anomalies and LBWs are still significant risk factors for mortality in cases with EA.Publication Metadata only Double intussusception in an infant(W B SAUNDERS CO, 2002) KIYAN, GÜRSU; Kiyan, G; Tugtepe, H; Iskit, SH; Dagli, TEIntussusception is a common surgical disorder in infancy. Double intussusception (DI), however, is an extremely rare form of this pathology. The authors report a case of idiopathic DI in an 8-month-old girl, which is the third case of idiopathic DI in childhood in the English-language literature. Copyright 2002, Elsevier Science (USA). All rights reserved.Publication Metadata only Interstitial hernia: a diagnostic dilemma in infants and children(1998) KIYAN, GÜRSU; Iskit, S. H.; Dağli, T. E.; Kiyan, G.; Durakbaşa, C. U.The features of the rare interstitial hernia, as seen in four children, are described. All were boys ranging in age from 7 days to 7 years. The presenting sign was swelling in the right side of the abdomen associated with undescended testis. The hernias arose at the deep inguinal ring and expanded superiorly between the internal and external oblique muscles. Repair involved a standard herniotomy and orchidopexy through an oblique inguinal incision-except in one patient with a vanishing testis. The importance of being aware of this rare type of hernia is stressed.Publication Metadata only Esophageal balloon dilation in children: prospective analysis of hemodynamic changes and complications during general anesthesia(ELSEVIER SCIENCE INC, 2007) KIYAN, GÜRSU; Gercek, Arzu; Dogan, Varlik; Dagli, Tolga; Ay, Binnaz; Kiyan, Gursu; Gogus, YilmazStudy Objective: To investigate hemodynamic changes and complications in children during balloon dilation of esophageal strictures. Design: Prospective, controlled study. Setting: University teaching hospital. Patients: 5 ASA physical status I and II pediatric patients with benign esophageal stricture related to ingestion of caustic substances. Interventions: Anesthesia was induced with intravenous propofol two mg/kg and cisatracurium 0.2 mg/kg and maintained with 66% nitrous oxide and one minimum alveolar concentration of sevoflurane in oxygen. In each session, balloon size was increased until the stricture was opened. Measurements and Results: A total of 18 sessions and 99 dilations in 5 children performed over a one-year period were included in the study. In 8 of 18 sessions, esophageal stricture was located in the middle one third of the esophagus; and in the others, in the upper one third. Four cases experienced bleeding; two cases, inability to ventilate due to obstruction of the endotracheal tube tip by the inflated balloon; and two cases, postextubation bronchospasm. In 95 of the 99 dilations, while the balloon was inflated, heart rate was faster and blood pressure increased significantly. Conclusion: Anesthesiologists should keep in mind the possibility of hemodynamic instability and possible endotracheal tube tip obstruction by the inflated balloon and safeguard the airway against bleeding, secretions, and radio-opaque fluid during esophageal balloon dilation. (c) 2007 Elsevier Inc. All tights reserved.Publication Metadata only Testicular biopsy during orchidopexy procedure: Does it have an adverse effect on fertility?(KARGER, 2005) KIYAN, GÜRSU; Iskit, SH; Tugtepe, H; Tugay, M; Kiyan, G; Kotiloglu, E; Dagli, TEIntroduction: The aim of this study is to evaluate the effect of the gonadal biopsy procedure on fertility and histology of the experimentally created undescended testis. Materials and Methods: Thirty Wistar- Albino rat litters were divided into three groups. In the first two groups, undescended testes were obtained by dividing the right gubernaculum in the first 48 h and the third group constituted the sham group. When the rats were 30 days old, orchidopexy was performed in the first group (O), whereas orchidopexy and testicular biopsy were performed in the second group (OB). After the fertility study all rats were sacrificed at week 11 and testicular weights, mean seminiferous tubular diameters (MSTD) and mean testicular biopsy scores ( MTBS) were measured. Results: One testis in the O group and three testes in the OB group were atrophied and these rats were excluded from the study. Testicular weights, MSTD and MTBS values of the operated side in the O and OB groups were lower than those in the sham group. There was a statistically significant cant difference between the MSTD values of the ipsilateral testes in the OB group and sham group ( p < 0.05). MTBS values of the ipsilateral testes in the O and OB groups were <8. There was no difference in all parameters in the contralateral testes between all groups (p > 0.05). All the evaluated rats were fertile. While there was no difference in number of fetuses and fertility scores between the O and OB group, however, the differences were significant between each of these groups and the sham group (p < 0.05) Conclusion: Testicular biopsy in the unilateral undescended testis model results in limited histological changes without affecting the fertility.Publication Metadata only The roles of free oxygen radicals, nitric oxide, and endothelin in caustic injury of rat esophagus(W B SAUNDERS CO-ELSEVIER INC, 2004) DAĞLI, EMRULLAH TOLGA; Ozel, SK; Dagli, TE; Yuksel, M; Kiyan, G; Kotiloglu, EPurpose: The authors aimed to find out the roles of free oxygen radicals, nitric oxide (NO), and endothelin (ET) in caustic injury of rat esophagus. Methods: Forty-five Wistar albino rats were used to form 6 groups. The study groups are summarized as 1, sham (S; n = 7); 2, sham + L-arginine (SA; n 7); 3, sham + L-NAME (SN; n = 7); 4, injury (I; n = 8); 5, injury + L-arginine (IA; n = 8); 6, injury + L-NAME (IN; n = 8). Normal saline in the sham groups and 50% NaOH in the caustic injury groups were administered to the distal esophagus. Free oxygen radicals and NO were detected by chemiluminescence from tissue samples, and they were correlated with histologic examinations. Tissue ET was measured also with immunohistochemistry. Results: The injury was verified histologically. Free oxygen radical levels were found to be increased as well as NO and ET with the caustic injury (P < .05). L-arginine caused a histologic increase in the injury that was close to statistical significance (P = .08). L-NAME showed no significant effect. Conclusions: Free radicals, NO, and ET increase in the early phase of caustic esophageal injury. Understanding their early interactions during the caustic injury may help in future therapeutic strategies. (C) 2004 Elsevier Inc. All rights reserved.Publication Metadata only Double balloon esophageal catheter for diagnosis of tracheo-esophageal fistula(SPRINGER-VERLAG, 2003) KIYAN, GÜRSU; Kiyan, G; Dagli, TE; Tugtepe, H; Kodalli, NCongenital H-type and recurrent tracheo-esophageal fistulas (TEF) are always difficult to diagnose. For a more accurate diagnosis we designed a new double balloon catheter, which is a modification of esophageal dilatation balloon. The catheter has two balloons to occlude the esophagus proximal and distal to the fistula. The fistula can be identified by passing of the contrast material to the tracheal tree, which was injected into the esophageal segment between the inflated balloons. To prove the efficiency of this catheter, a TEF was created surgically in a New Zealand rabbit. On the postoperative fourteenth day the catheter was tried and the fistula could be visualized easily by injecting the contrast material. We think this technique may be of use in the diagnosis of TEF in children.Publication Open Access First documented case of pulmonary alveolar proteinosis with atopy presenting secondary to CSFR2B mutation(2022-08-01) KIYAN, GÜRSU; Sasihuseyinoglu A. S. , ÖZCAN D., Avci A., KIYAN G.Pulmonary alveolar proteinosis (PAP) is a rare lung disorder in which surfactant-derived lipoproteins accumulate excessively within pulmonary alveoli, causing severe respiratory distress. It is essential to gain a better understanding of the signs to clinically diagnose PAP and include PAP among the differential diagnoses of interstitial pulmonary diseases or other diseases with similar manifestations. We describe a 2.5-year patient with atopy who presented with pulmonary infiltration, recurrent wheezing, and cough despite steroid and salbutamol administration via inhalation. High-resolution computed tomography revealed crazy-paving patterns in both lungs, suggesting PAP. An open lung biopsy revealed intra-alveolar granular amphophilic material, which was strongly positive on periodic acid-Schiff staining. The results of pulmonary-associated surfactant protein B and C gene analyses were normal. However, granulocyte-macrophage colony-stimulating factor receptor beta-protein was not detected in leucocytes, and a novel mutation was identified in the CSF2RB gene. The patient was diagnosed with PAP and treated with whole-lung lavage. Key Words: Pulmonary alveolar proteinosis, Child, Atopy, Wheezing.