Browsing by Author "Yaman A."
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Item Bilateral intravitreal dexamethasone implant for retinitis pigmentosa-related macular edema(S. Karger AG, 2013) Saatci A.O.; Selver O.B.; Seymenoglu G.; Yaman A.Purpose: To report the efficacy of intravitreal dexamethasone implant in a patient with retinitis pigmentosa and bilateral cystoid macular edema unresponsive to topical carbonic anhydrase inhibitors. Case Report: A 36-year-old man with bilateral cystoid macular edema associated with retinitis pigmentosa that was unresponsive to topical carbonic anhydrase inhibitors underwent bilateral 0.7-mg intravitreal dexamethasone implants two weeks apart. Spectral domain optical coherence tomography revealed resolution of macular edema one week following each injection in both eyes and his visual acuity improved. However, macular edema recurred two months later in OS and three months later in OD. Second implant was considered for both eyes. No implant-related complication was experienced during the follow-up of seven months. Conclusion: Inflammatory process seems to play a role in retinitis pigmentosa. Intravitreal dexamethasone implant may offer retina specialists a therapeutic option especially in cases unresponsive to other treatment regimens in eyes with retinitis pigmentosa-related macular edema. © 2013 S. Karger AG, Basel.Item A database for screening and registering late onset Pompe disease in Turkey(Elsevier Ltd, 2018) Gokyigit M.C.; Ekmekci H.; Durmus H.; Karlı N.; Koseoglu E.; Aysal F.; Kotan D.; Ali A.; Koytak P.K.; Karasoy H.; Yaman A.; Sengun İ.S.; Sayin R.; Tiftikcioglu B.I.; Soysal A.; Tutkavul K.; Bayrak A.O.; Kısabay A.; Elci M.A.; Yayla V.; Yılmaz İ.A.; Ozdamar S.E.; Erdogan C.; Tasdemir N.; Serdaroglu Oflazer P.; Deymeer F.; Parman Y.; Kendirci M.; Sayan S.; Celebi L.G.; Uluç K.; Tanrıdağ T.; Yuceyar N.; Ekmekci O.; Colakoglu B.D.; Ozturk S.; Tireli H.; Selcuki D.; Neyal A.M.; Kayran Y.; Aluclu M.U.; Koyuncuoglu H.R.; Tokucoglu F.; Secil Y.; Guney F.; Gozke E.; Balaban H.; Akalın M.A.; Koc A.F.; Mulayim S.; Turgut N.The aim of this study was to search for the frequency of late onset Pompe disease (LOPD) among patients who had a myopathy with unknown diagnosis registered in the pre-diagnostic part of a novel registry for LOPD within a collaborative study of neurologists working throughout Turkey. Included in the study were 350 patients older than 18 years who have a myopathic syndrome without a proven diagnosis by serum creatine kinase (CK) levels, electrodiagnostic studies, and/or muscle pathology, and/or genetic tests for myopathies other than LOPD. Acid alpha glucosidase (GAA) in dried blood spot was measured in each patient at two different university laboratories. LOPD was confirmed by mutation analysis in patients with decreased GAA levels from either both or one of the laboratories. Pre-diagnostic data, recorded by 45 investigators from 32 centers on 350 patients revealed low GAA levels in a total of 21 patients; from both laboratories in 6 and from either one of the laboratories in 15. Among them, genetic testing proved LOPD in 3 of 6 patients and 1 of 15 patients with decreased GAA levels from both or one of the laboratories respectively. Registry was transferred to Turkish Neurological Association after completion of the study for possible future use and development. Our collaborative study enabled collection of a considerable amount of data on the registry in a short time. GAA levels by dried blood spot even from two different laboratories in the same patient may not prove LOPD. LOPD seemed to be rarer in Turkey than in Europe. © 2017 Elsevier B.V.Item LIMPRINT study: The Turkish experience(Mary Ann Liebert Inc., 2019) Borman P.; Moffatt C.; Murray S.; Yaman A.; Denizli M.; Dalyan M.; Unsal-Delialioǧlu S.; Eyigör S.; Ayhan F.; Çaklt B.D.; Vural S.; Özdemir O.; Kurt E.; Çelik E.C.; Cerrahoǧlu L.; Kepekçi M.; Terzioǧlu F.; Donmez A.A.Background: Lymphedema and chronic edema is a major health care problem in both developed and nondeveloped countries The Lymphoedema Impact and Prevelance-International (LIMPRINT) study is an international health service-based study to determine the prevalence and functional impact in adult populations of member countries of the International Lymphoedema Framework (ILF). Methods and Results: A total of 1051 patients from eight centers in Turkey were recruited using the LIMPRINT study protocol. Data were collected using the core and module tools that assess the demographic and clinical properties as well as disability and quality of life (QoL). Most of the Turkish patients were recruited from specialist lymphedema services and were found to be women, housewives, and having secondary lymphedema because of cancer treatment. The duration of lymphedema was commonly <5 years and most of them had International Society of Lymphology (ISL) grade 2 lymphedema. Cellulitis, infection, and wounds were uncommon. The majority of patients did not get any treatment or advice before. Most of the patients had impaired QoL and decreased functionality, but psychological support was neglected. Although most had social health security access to lymphedema centers, nevertheless access seemed difficult because of distance and cost. Conclusion: The study has shown the current status and characteristics of lymphedema patients, treatment conditions, the unmet need for the diagnosis and treatment, as well as burden of the disease in both patients and families in Turkey. National health policies are needed for the prevention, diagnosis, and treatment in Turkey that utilize this informative data. © Pinar Borman et al. 2019; Published by Mary Ann Liebert, Inc. 2019.Item Familial mediterranean fever mutation analysis in pediatric patients with İnflammatory Bowel Disease: A multicenter study(AVES, 2021) Urganci N.; Ozgenc F.; Kuloǧlu Z.; Yüksekkaya H.; Sari S.; Erkan T.; Önal Z.; Çaltepe G.; Akçam M.; Arslan D.; Arslan N.; Artan R.; Aydoǧan A.; Balamtekin N.; Baran M.; Baysoy G.; Çakir M.; Dalgiç B.; Doǧan Y.; Durmaz O.; Ecevit C.; Eren M.; Gökçe S.; Gülerman F.; Gürakan F.; Hizli S.; Işik I.; Kalayci A.G.; Kansu A.; Kutlu T.; Karabiber H.; Kasirga E.; Kutluk G.; Hoşnut F.O.; Özen H.; Özkan T.; Öztürk Y.; Soylu O.B.; Tutar E.; Tümgör G.; Ünal F.; Ugraş M.; Üstündaǧ G.; Yaman A.Background: The aim of the study was to evaluate familial Mediterranean fever (FMF) mutation analysis in pediatric patients with inflammatory bowel disease (IBD). The relation between MEFV mutations and chronic inflammatory diseases has been reported previously. Methods: Children with IBD (334 ulcerative colitis (UC), 224 Crohn's disease (CD), 39 indeterminate colitis (IC)) were tested for FMF mutations in this multicenter study. The distribution of mutations according to disease type, histopathological findings, and disease activity indexes was determined. Results: A total of 597 children (mean age: 10.8 ± 4.6 years, M/F: 1.05) with IBD were included in the study. In this study, 41.9% of the patients had FMF mutations. E148Q was the most common mutation in UC and CD, and M694V in IC (30.5%, 34.5%, 47.1%, respectively). There was a significant difference in terms of endoscopic and histopathological findings according to mutation types (homozygous/ heterozygous) in patients with UC (P < .05). There was a statistically significant difference between colonoscopy findings in patients with or without mutations (P = .031, P = .045, respectively). The patients with UC who had mutations had lower Pediatric Ulcerative Colitis Activity Index (PUCAI) scores than the patients without mutations (P = .007). Conclusion: Although FMF mutations are unrelated to CD patients, but observed in UC patients with low PUCAI scores, it was established that mutations do not have a high impact on inflammatory response and clinical outcome of the disease. Copyright © 2021 by The Turkish Society of Gastroenterology.Item The clinical and demographical characteristics of Turkish pediatric lymphedema patients: a multicenter study(Turkiye Klinikleri, 2022) Borman P.; Balcan A.; Eyigör S.; Coşkun E.; Ayhan F.; Çakit B.D.; Vural S.; Vural M.; Çakir E.D.P.; Çağdaş D.; Yaman A.; Cerrahoğlu L.; Doğan S.C.Background/aim: Reducing lymphedema-associated burden and disability in the pediatric setting requires improved awareness and understanding clinical properties of the lymphedema. The aim of this study was to evaluate the clinical and demographic characteristics of patients with pediatric lymphedema presented to different lymphedema centers in Turkey. Materials and methods: The socio-demographic and clinical characteristics of the children including age, gender, presence of genetic syndromes, duration of edema, site and stage of lymphedema and the received therapies were determined. Parental and children education on self-management techniques were recorded. Results: A total of 122 children (female: 66, male: 56) with a mean age of 120.7 ± 71.2 months were included from 7 centers. Of them; 92% had primary, 8% had secondary lymphedema mostly due to infection and trauma. Lymphedema was part of a syndrome in 18% of the children. The most common site of involvement was the lower extremity, followed by upper extremity and genital involvement. Lymphedema was complicated in 17 % of children, mainly with a clinical picture of cellulitis, infection, and pain. The median duration of lymphedema was 41 (5–216) months. Although most of the children had stage 2 lymphedema, only 40% of them received treatment. The most commonly received treatment was compression therapy. No family or child was educated for self-care management before. Conclusion: In conclusion, pediatric lymphedema has a comparable gender distribution and usually involves the lower extremities. Although most of the children had advanced disease, more than half of the patients did not receive any treatment indicating the unmet need for management of lymphedema. The education of patients and/or children about self-management methods were lacking. We suggest educational activities for both families of children with lymphedema and health care providers, in order to facilitate early reference to lymphedema units and to receive prompt preventive and therapeutic approaches for this suffering condition. © TÜBİTAK.Item Mortality risk factors among critically ill children with MIS-C in PICUs: a multicenter study(Springer Nature, 2023) Sık G.; Inamlık A.; Akçay N.; Kesici S.; Aygun F.; Kendırlı T.; Atay G.; Sandal O.; Varol F.; Ozkaya P.Y.; Duyu M.; Bırbılen A.Z.; Ozcan S.; Arslan G.; Kangın M.; Bayraktar S.; Altug U.; Anıl A.B.; Havan M.; Yetımakman A.F.; Dalkıran T.; Zengın N.; Oto A.; Kıhtır H.S.; Gırgın F.İ.; Telhan L.; Yıldızdas D.; Yener N.; Yukselmıs U.; Alakaya M.; Kılınc M.A.; Celegen M.; Dursun A.; Battal F.; Sarı F.; Ozkale M.; Topal S.; Kocaoglu C.; Yazar A.; Alacakır N.; Odek C.; Yaman A.; Cıtak A.; Bıngol I.; Annayev A.; Sevketoglu E.; Katlan B.; Durak C.; Gun E.; Erdogan S.; Seven P.; Sahın E.; Arı H.F.; Boyraz M.; Durak F.; Emeksız S.; Ozdemır G.; Duman M.; Talay M.N.; Yener G.O.; Luleyap D.; Harmanogulları S.; Başar E.Z.; Mercan M.; Bal A.; Kılıc N.; Ongun E.A.; Ozturk M.N.; Ekıncı F.; Udurgucu M.; Arslankoylu A.E.; Kutlu N.O.; Bukulmez A.; Özsoylu S.; Celık T.; Ozkale Y.; Kılıc A.O.Background: This study evaluated of clinical characteristics, outcomes, and mortality risk factors of a severe multisystem inflammatory syndrome in children admitted to a the pediatric intensive care unit. Methods: A retrospective multicenter cohort study was conducted between March 2020 and April 2021 at 41 PICUs in Turkey. The study population comprised 322 children diagnosed with multisystem inflammatory syndrome. Results: The organ systems most commonly involved were the cardiovascular and hematological systems. Intravenous immunoglobulin was used in 294 (91.3%) patients and corticosteroids in 266 (82.6%). Seventy-five (23.3%) children received therapeutic plasma exchange treatment. Patients with a longer duration of the PICU stay had more frequent respiratory, hematological, or renal involvement, and also had higher D-dimer, CK-MB, and procalcitonin levels. A total of 16 patients died, with mortality higher in patients with renal, respiratory, or neurological involvement, with severe cardiac impairment or shock. The non-surviving group also had higher leukocyte counts, lactate and ferritin levels, and a need for mechanical ventilation. Conclusions: In cases of MIS-C, high levels of D-dimer and CK-MB are associated with a longer duration of PICU stay. Non-survival correlates with elevated leukocyte counts and lactate and ferritin levels. We were unable to show any positive effect of therapeutic plasma exchange therapy on mortality. Impact: MIS-C is a life-threatening condition.Patients need to be followed up in the intensive care unit.Early detection of factors associated with mortality can improve outcomes.Determining the factors associated with mortality and length of stay will help clinicians in patient management.High D-dimer and CK-MB levels were associated with longer PICU stay, and higher leukocyte counts, ferritin and lactate levels, and mechanical ventilation were associated with mortality in MIS-C patients.We were unable to show any positive effect of therapeutic plasma exchange therapy on mortality. © 2023, The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.Item Neonatal Resuscitation Practices in Turkey: A Survey of the Turkish Neonatal Society and the Union of European Neonatal and Perinatal Societies(AVES, 2023) Okulu E.; Koç E.; Erdeve Ö.; Akdağ A.; Aktaş S.; Aydemir Ö.; Aygün C.; Bayraktar B.T.; Cebeci B.; Çelik H.T.; Çelik K.; Engür D.; Ertuğrul S.; Fettah N.D.; Gültekin N.D.; Güçyetmez Z.Ş.T.; Gülen P.; Hirfanoğlu İ.M.; İnce Z.; Kader Ş.; Kahvecioğlu D.; Kanburoğlu M.K.; Karagöl B.S.; Kılıç İ.; Koroğlu Ö.A.; Melekoğlu N.A.; Narter F.; Olukman Ö.; Ongun H.; Ovalı F.; Özer E.A.; Özkan E.Ö.; Öztürk D.Y.; Özüdoğru E.; Sarıcı D.; Satar M.; Takçı Ş.; Tanrıverdi S.; Taşkın E.; Tayman C.; Tekgündüz K.Ş.; Tunç G.; Türkmen M.K.; Tüzün F.; Uslu S.; Ünal S.; Ünkar Z.A.; Yaman A.; Yaşa B.; Yıldırım Ş.; Yılmaz A.; Yılmaz F.H.; Yücesoy E.Objective: Optimal care in the delivery room is important to decrease neonatal morbidity and mortality. We aimed to evaluate neonatal resuscitation practices in Turkish centers. Materials and Methods: A cross-sectional survey consisted of a 91-item questionnaire focused on delivery room practices in neonatal resuscitation and was sent to 50 Turkish centers. Hospitals with <2500 and those with ≥2500 births/year were compared. Results: In 2018, approximately 240 000 births occurred at participating hospitals with a median of 2630 births/year. Participating hospitals were able to provide nasal continuous-positive- airway-pressure/high-flow nasal cannula, mechanical ventilation, high-frequency oscillatory ventilation, inhaled nitric oxide, and therapeutic hypothermia similarly. Antenatal counseling was routinely performed on parents at 56% of all centers. A resuscitation team was present at 72% of deliveries. Umbilical cord management for both term and preterm infants was similar between centers. The rate of delayed cord clamping was approximately 60% in term and late preterm infants. Thermal management for preterm infants (<32 weeks) was similar. Hospitals had appropriate equipment with similar rates of interventions and management, except conti nuous-positive-airway-pressure and positive-end-expiratory-pressure levels (cmH2 O) used in preterm infants (P = .021, and P = .032). Ethical and educational aspects were also similar. Conclusions: This survey provided information on neonatal resuscitation practices in a sam-ple of hospitals from all regions of Turkey and allowed us to see weaknesses in some fields. Although adherence to the guidelines was high among centers, further implementations are required in the areas of antenatal counseling, cord management, and circulation assessment in the delivery room. © 2023, AVES. All rights reserved.