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  • No Thumbnail Available
    Publication
    Does liver transplant improve neurological symptoms in wilson disease? report of 24 cases
    (Başkent Üniversitresi, 2022-11-01) Aksoy, Fuat; Arslan, İbrahim Ethem; Özgür, Taner; Dündar, Halit Ziya; Çelik, Fatih; Demir, Aylin Bican; Özbek, Sevda Erer; Kıyıcı, Murat; Özkan, Tanju Başarır; Kaya, Ekrem; AKSOY, FUAT; Arslan, İbrahim Ethem; ÖZGÜR, TANER; DÜNDAR, HALİT ZİYA; ÇELİK, FATİH; BİCAN DEMİR, AYLİN; ERER ÖZBEK, ÇİĞDEM SEVDA; KIYICI, MURAT; Özkan, Tanju Başarır; KAYA, EKREM; Tıp Fakültesi; Organ Nakli Merkezi; 0000-0001-5808-9384; 0000-0002-9245-1241; 0000-0001-6739-8605; HII-8895-2022; ABD-3885-2020; AAG-7319-2021; FPE-9941-2022; EWI-3634-2022; JKN-9078-2023; KHB-9765-2024; DLN-1836-2022; FHW-0015-2022; JKL-3648-2023
    Objectives: Wilson disease is an inherited disorder that results in copper accumulation in the tissues with liver injury and failure. Orthotopic liver transplant is one of the treatments of choice for this disease. The aim of this study was to compare the neurological symptoms, before and after orthotopic liver transplant, of patients with liver cirrhosis due to Wilson disease, who represent a special group of patients with liver failure. Materials and Methods: Between 2007 and 2020, there were 24 patients with Wilson disease resistant to medical treatment who underwent deceased donor orthotopic liver transplant and were followed up for 1 year, 5 years, and 10 years for evaluation with neurological scoring systems. Patients were also evaluated for postoperative complications and survival. Results: Of the 24 patients evaluated, there were 13 (54.2%) female patients and 11 (45.8%) male patients, and the mean age was 34 years (range, 14-57 years). One of the patients died from early postoperative sepsis. After orthotopic liver transplant, disease scores returned to normal in 16 patients and improved in the remaining patients. Before transplant, all patients required help in their daily activities. After transplant, there were significant improvements in some symptoms, and the patients became more independent in their daily lives. Conclusions: Our study shows that orthotopic liver transplant provides significant improvement in neurological symptoms and quality of life in patients with Wilson disease.
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    Celiac disease and autoimmune hepatitis presenting with fulminant hepatic failure: A case report
    (Erciyes Üniversitesi, 2021-07-01) Kaptan, Kadriye Nil; Özgür, Taner; Turan, Enes; Özkan, Tanju Başarır; Özkan, Tanju Başarır; KAPTAN, KADRİYE NİL; ÖZGÜR, TANER; TURAN, ENES; Tıp Fakültesi; Pediatri Gastroenteroloji Bölümü; CXV-7127-2022; JJX-2704-2023; JSJ-2135-2023; IGW-0111-2023
    Background: Fulminant hepatic failure (FHF) may be a rare presentation of autoimmune hepatitis (AIH) in previously asymptomatic adolescents. Celiac disease (CD) is a chronic immune disease that may be associated with severe forms of liver disease and coexists with AIH. We report a patient presenting with an FHF at the diagnosis of AIH and CD.Case Report: An 8-year-old female patient who had no known background or family history of liver disease was referred to our center with an FHF diagnosis. Her clinical and laboratory findings fulfilled the criteria of seronegative AIH and CD. After 7 days of starting the medical treatment, encephalopathy and liver function tests gradually improved.Conclusion: AIH and CD usually follow a chronic course and rarely coexist. However, they should be considered the etiologies of FHF and should be treated promptly.
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    Clinical and laboratory characteristics of the children with diabetic ketoacidosis
    (Galenos Yayıncılık, 2008-12-01) Sağlam, Halil; Eren, Erdal; Çakır, Esra Deniz; Yüce, Necla; Yıldız, Nalan; Çakır, Seher; Özgür, Taner; Tarım, Ömer; SAĞLAM, HALİL; EREN, ERDAL; Çakır, Esra Deniz; Yüce, Necla; Yıldız, Nalan; Çakır, Seher; ÖZGÜR, TANER; TARIM, ÖMER FARUK; Tıp Fakültesi; Çocuk Sağlığı ve Hastalıkları Ana Bilim Dalı; 0000-0002-6598-8262; 0000-0002-1684-1053; 0000-0003-4664-7435; 0000-0003-0710-5422; C-7392-2019; JPK-3909-2023; GQO-9634-2022; JKN-1212-2023; IPY-4170-2023; EPI-7647-2022; AAG-8381-2021; EBM-1323-2022
    Aim: Diabetic ketoacidosis (DKA) is a serious condition with the highest rates of morbidity and mortality in children with type 1 diabetes mellitus (T1 DM). Hospitalization due to DKA constitutes about 2 to 8 percent of all-cause admissions. About 25% of children with newly diagnosed T1DM present with DKA. In this study, we aimed to evaluate sociodemographic and clinical characteristics of children admitted with DKA.Materials and Method: Records of 490 children admitted with T1DM between January 2003 and October 2008 were retrospectively reviewed and data on sociodemographic, clinical and laboratory characteristics of 163 children with the diagnosis of DKA was investigated in detail.Results: Of 490 children with T1DM, 163 (33.2%) had the diagnosis of DKA and total number of DKA episodes was 190. The recurrence rate was 14.2% and 85% (6 out of 7) of those with recurrent episodes were girls. The mean age was 11.02 4.57 years and DKA episodes were most frequently seen in 10-15 (39.3) age-group. DKA episodes peaked in the periods of January-February-March and children (i.e, 0-5 age group) (54 5%) The mean duration of recoveiy from acidosis was 14.29+/-12.14 hours the mean blood glucose level at baseline was 473.09 +/- 141,07 mg/dI and the mean initial HbAl c level vvas 12.04+/-2.42%, In this approximately six-year-penod, only one patient was lost and the mortality rate was 0,5% (1 tit of 190 episodes).Conclusion: Increasing the knowledge of primary care physicians who see the patients first on DKA and al3plying the DKA treatment protocol more slowly and cautiously in younger children (0-5 age group) will definitely loldver the morbidity and mortalibt of DKA.
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    Vedolizumab treatment in a patient with x-linked agammaglobulinemia, is it safe and efficient?
    (Turkish J Pediatrics, 2019-11-01) Kılıç, Sara Sebnem; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Çekiç, Sükrü; ÇEKİÇ, ŞÜKRÜ; Özgür, Taner; ÖZGÜR, TANER; Özkan, Tanju; ÖZKAN, TANJU MUNEVVER; Karali, Yasin; KARALI, YASİN; Tıp Fakültesi; Pediatri Ana Bilim Dalı; 0000-0002-9574-1842; 0000-0001-5740-9729; 0000-0001-8571-2581; AAG-8416-2021; AAH-1658-2021; L-1933-2017; AAG-8381-2021
    The loss of inflammatory regulation resulting from the absence of B-lymphocytes leads to a risk for autoimmune and autoinflammatory complications. There is no data on the use of Vedolizumab in patients with X-linked agammaglobulinemia (XLA) as well as children with another primary immunodeficiency (PID) diseases. A 4-year-old boy was admitted to our clinic with a history of recurrent respiratory tract infections. He was diagnosed with XLA based on extremely low immunoglobulins, very low level of B cells, genetic mutation of BTK gene, and family history. At the age of 8, he suffered from intermittent fever attacks, abdominal pain, weakness, oral aft, and weight loss. His clinical and laboratory features were consistent with inflammatory bowel disease. Histopathological examination of the biopsy material obtained from terminal ileum, colon and cecum showed Crohn's disease. Initially, he was treated with prednisolone and infliximab. Because of the lack of response, infliximab treatment was switched to adalimumab. Terminal ileum was resected to relieve obstruction complication. Although he had been treated with adalimumab, a significant improvement was not observed. Vedolizumab (Entyvio (TM)), a humanized monoclonal antibody alpha 4 beta 7 integrin-receptor antagonist, was commenced. After treatment with vedolizumab, his fever and abdominal pain attacks reduced, his total daily calorie intake increased and weight gain improved. He began to walk again and continued his school education properly. No side effects were observed in 18 months. This is the first immunocompromised child treated with vedolizumab. The symptoms of the patient receded and no side effect were seen during the treatment.
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    The frequency of lysosomal acid lipase deficiency in children with unexplained liver disease
    (Lippincott, 2019-03-01) Kuloğlu, Zarife; Kansu, Aydan; Selbuz, Suna; Kalaycı, Ayhan G.; Şahin, Gülseren; Kırsaçlıoğlu, Ceyda Tuna; Demirören, Kaan; Dalgıç, Büket; Kasırga, Erhun; Önal, Zerrin; İslek, Ali; Eren, Esra; Hoşnut, Ferda Özbay; Urgancı, Nafiye; Yaman, Aytaç; Özkan, Tanju; Bozbulut, Ekşi; Doğan, Güzide; Ekşi Bozbulut, Neslihan; Doğan, Güzide; Durmaz Uğurcan, Özlem; Usta, Ayşe Merve; Arslan, Duran; Akçam, Mustafa; Isik, Ishak Abdurrahman; Ecevit, Çigdem Ömür; Usta, Yusuf; Özgür, Taner; Özçay, Figen; Balamtekin, Necati; Öztürk, Yesim; Balamtekin, Necati; Öztürk, Yeşim; Cantez, Serdar; Gülerman, Fulya; Ustundag, Gonca Handan; Emiroğlu, Halil Haldun; Karacabey, Neslihan; Comba, Atakan; Erdemir, Gülin; Aydoğan, Aysen Uncuoğlu; Gökçe, Selim; Kuyum, Pınar; Gülsan, Meltem; Tosun, Mahya Sultan; Tokgöz, Yavuz; Güven, Burcu; Yüksekkaya, Hasan; Tümgör, Gökhan; Eren, Makbule; Baran, Maşallah; Gümüş, Meltem; Canan, Oğuz; Kocamaz, Halil; Gerenli, Nelgin; Çakır, Murat; Agiı, Mehmet; Hızlı, Samil; Doğan, Yasar; Çeltik, Coşkun; Deveci, Uğur; Balcı Sezer, Oya; Natl Lal-D Study Grp; ÖZKAN, TANJU MUNEVVER; ÖZGÜR, TANER; Tıp Fakültesi; AAG-8416-2021; AAG-8381-2021
    Objectives: Evidence suggests that lysosomal acid lipase deficiency (LAL-D) is often underdiagnosed because symptoms may be nonspecific. We aimed to investigate the prevalence of LAL-D in children with unexplained liver disease and to identify demographic and clinical features with a prospective, multicenter, cross-sectional study.Methods: Patients (aged 3 months-18 years) who had unexplained transaminase elevation, unexplained hepatomegaly or hepatosplenomegaly, obesity-unrelated liver steatosis, biopsy-proven cryptogenic fibrosis and cirrhosis, or liver transplantation for cryptogenic cirrhosis were enrolled. A Web-based electronic data collection system was used. LAL activity (nmol/punch/h) was measured using the dried blood spot method and classified as LAL-D(<0.02), intermediate (0.02-0.37) or normal (>0.37). Asecond dried blood spot sample was obtained from patients with intermediate LAL activity for confirmation of the result.Results: A total of 810 children (median age 5.6 years) from 795 families were enrolled. The reasons for enrollment were unexplained transaminase elevation (62%), unexplained organomegaly (45%), obesity-unrelated liver steatosis (26%), cryptogenic fibrosis and cirrhosis (6%), and liver transplantation for cryptogenic cirrhosis (<1%). LAL activity was normal in 634 (78%) and intermediate in 174 (21%) patients. LAL-D was identified in 2 siblings aged 15 and 6 years born to unrelated parents. Dyslipidemia, liver steatosis, and mild increase in aminotransferases were common features in these patients. Moreover, the 15-year-old patient showed growth failure and microvesicular steatosis, portal inflammation, and bridging fibrosis in the liver biopsy. Based on 795 families, 2 siblings in the same family were identified as LAL-D cases, making the prevalence of LAL-D in this study population, 0.1% (0.125%-0.606%). In the repeated measurement (76/174), LAL activity remained at the intermediate level in 38 patients.Conclusions: Overall, the frequency of LAL-D patients in this study (0.1%) suggests that LAL-D seems to be rare even in the selected high-risk population.
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    Liver involvement in congenital hypopituitarism
    (Springer India, 2019-05-01) Altay, Derya; Eren, Erdal; EREN, ERDAL; Özkan, Tanju Başarır; Özgür, Taner; ÖZGÜR, TANER; Tarım, Ömer; TARIM, ÖMER FARUK; Tıp Fakültesi; 0000-0002-1684-1053; 0000-0001-5740-9729; 0000-0002-5322-5508; AAG-8381-2021; AAG-8416-2021; JPK-3909-2023
    ObjectiveCholestatic jaundice in early infancy is a complex diagnostic challenge. Cholestasis caused by endocrine disease is rare and poorly recognized. The aim of this paper is to report patients with liver dysfunctions resulting from hypopituitarism.MethodsSix patients with liver dysfunction diagnosed as hypopituitarism were studied and followed up atUludag University Faculty of Medicine.ResultsThe median age of the patients at first presentation was 2.5 mo. Three patients were diagnosed with congenital hypopituitarism at the first visit, and the other three were diagnosed during follow-up. Serum aminotransferase levels were very high in two patients and only moderately elevated in the others. Combined adrenal, thyroid, and growth hormone deficiencies were diagnosed in two patients, while remaining 4 patients had various combinations of adrenal, thyroid, and growth hormone deficiencies. Liver function abnormalities resolved between 10 d and 2 mo follow-up after hormone replacement therapy.ConclusionsAbnormal liver biochemical test results due to hormonal deficiencies in infants should be considered in the differential diagnosis by pediatricians. Hormone replacement therapy is the basis of treatment.
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    The evaluation of gastroesophageal reflux disease with esophageal pH monitorization in children
    (Galenos Yayıncılık, 2009-12-01) Erdemir, Gülin; Özgür, Taner; Canitez, Yakup; Kayık, Bülent; Özboyacı, Ali; Özkan, Tanju B.; Erdemir, Gülin; ÖZGÜR, TANER; CANITEZ, YAKUP; Kayık, Bülent; Özboyacı, Ali; Özkan, Tanju B.; Tıp Fakültesi; Çocuk Sağlığı Hastalıkları Ana Bilim Dalı; Çocuk Alerji Bilim Dalı; AAG-8381-2021; ETR-2252-2022; CJQ-2060-2022; CYG-7798-2022; JLB-3549-2023
    Introduction: Gastroesophageal reflux disease is named when gastroesophageal reflux becomes symptomatic, influences the life comfort, and results in morbidity. In this study, we aimed to evaluate the patients admitted with GER-associated symptoms with demographic characteristics, 24-hour esophageal pH monitorization results, and responses to different treatment protocols.Materials and Method: The data of patients who admitted to Uludag University Pediatrics Outpatient Clinic between January 2008 and September 2009 with GERD-associated symptoms and underwent esophageal pH monitorization was collected retrospectively. The associations between initial symptoms, 24-hour esophageal pH monitorization results, presence of accompanying disease, sociocultural conditions, and therapy responses were evaluated.Results: Ninety-two patients (37 female, 55 male) were included and mean age was 4.42 +/- 4.33 (1 mo-18 years). The frequency of cough, vomiting and abdominal pain at admission were 49.5%, 47.3% and 22% respectively. Esophageal pH monitorization diagnosed GERD in 21 patients (22.8%). The frequency of symptoms in GERD patients who diagnosed with esophageal pH monitorization was; cough (n: 15, 33.4%), vomiting (n: 11, 25.6%), and abdominal pain (n: 5, 22.3%). Among all complaints, cough was found to be significantly associated with GERD (p=0.041). Accompanying neurological disease was present in 13.1% (n: 12), immunodeficiency 13.1% (n: 12) and bronchial reactivity 13.1% (n: 12). Therapy success was achieved in 57.7% of patients. Therapy response were similar with different therapy protocols (proton pump inhibitor+prokinetic+sucralfate, proton pump inhibitor+prokinetic, H2 receptor blocker+sucralfate, proton pump inhibitor+sucralfate, H2 receptor blocker+prokinetic, monotherapy with proton pump inhibitor or H2 receptor blocker) (p=0.068).Conclusion: GERD is a common childhood disease. Currently, diagnosis and treatment of GERD in children is still controversial. The results of our study revealed no association with therapy success and different treatment regimens, sociocultural status or accompanying disease. The complaint of cough was found to be significantly in association with GERD.
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    Vedolizumab treatment in a patient with x-linked agammaglobulinemia, is it safe and efficient?
    (Türk Pediatri Dergisi, 2019-11-01) Çekiç, Şükrü; Özgür, Taner; Karalı, Yasin; Özkan, Tanju; Kılıç, Sara Şebnem; ÇEKİÇ, ŞÜKRÜ; ÖZGÜR, TANER; KARALI, YASİN; ÖZKAN, TANJU MUNEVVER; KILIÇ GÜLTEKİN, SARA ŞEBNEM; Tıp Fakültesi; Çocuk Sağlığı ve Hastalıkları Ana Bilim Dalı; 0000-0002-9574-1842; 0000-0001-5740-9729; 0000-0001-8571-2581; AAG-8416-2021; AAG-8381-2021; AAH-1658-2021; L-1933-2017; FFS-1974-2022
    The loss of inflammatory regulation resulting from the absence of B-lymphocytes leads to a risk for autoimmune and autoinflammatory complications. There is no data on the use of Vedolizumab in patients with X-linked agammaglobulinemia (XLA) as well as children with another primary immunodeficiency (PID) diseases. A 4-year-old boy was admitted to our clinic with a history of recurrent respiratory tract infections. He was diagnosed with XLA based on extremely low immunoglobulins, very low level of B cells, genetic mutation of BTK gene, and family history. At the age of 8, he suffered from intermittent fever attacks, abdominal pain, weakness, oral aft, and weight loss. His clinical and laboratory features were consistent with inflammatory bowel disease. Histopathological examination of the biopsy material obtained from terminal ileum, colon and cecum showed Crohn's disease. Initially, he was treated with prednisolone and infliximab. Because of the lack of response, infliximab treatment was switched to adalimumab. Terminal ileum was resected to relieve obstruction complication. Although he had been treated with adalimumab, a significant improvement was not observed. Vedolizumab (Entyvio (TM)), a humanized monoclonal antibody alpha 4 beta 7 integrin-receptor antagonist, was commenced. After treatment with vedolizumab, his fever and abdominal pain attacks reduced, his total daily calorie intake increased and weight gain improved. He began to walk again and continued his school education properly. No side effects were observed in 18 months. This is the first immunocompromised child treated with vedolizumab. The symptoms of the patient receded and no side effect were seen during the treatment.
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    Normal or elevated prolactin is a good indicator to show pituitary stalk interruption syndrome in patients with multiple pituitary hormone deficiency
    (Walter De Gruyter Gmbh, 2022-09-23) Eren, Erdal; Öngen, Yasemin Denkboy; Özgür, Taner; Özpar, Rıfat; Demirbaş, Özgecan; Yazıcı, Zeynep; Tarım, Ömer; EREN, ERDAL; DENKBOY ÖNGEN, YASEMİN; ÖZGÜR, TANER; ÖZPAR, RİFAT; DEMİRBAŞ, ÖZGECAN; YAZICI, ZEYNEP; TARIM, ÖMER FARUK; Tıp Fakültesi; Çocuk Endokrinolojisi Ana Bilim Dalı; 0000-0002-1684-1053; 0000-0001-6649-9287; 0000-0002-6922-5203; 0000-0002-5322-5508; KHZ-1491-2024; JPK-3909-2023; AAH-5062-2021; AAI-2303-2021; FPE-9941-2022; GQX-9760-2022; CCU-8073-2022
    Objectives To determine the importance of serum prolactin (PRL) in the detection of pituitary stalk interruption syndrome (PSIS) in children with multiple pituitary hormone deficiency (MPHD). We hypothesized that PRL elevation might be a diagnostic indicator of pituitary stalk pathologies. Methods Clinical, radiological, and laboratory features of the 50 cases of MPHD were studied. Results The median age at presentation of the 50 cases (52%, n=26 were female) was 6.61 (0.02-18.9) years. PSIS was detected in 60% (n=30), pituitary hypoplasia in 32% (n=16), partial empty sella in 6% (n=3), and only 2% (n=1) was reported as normal. Out of 50 patients, 21.3% (n=10) were hypoprolactinemic, 44.7% (n=19) were normoprolactinemic, and 34% (n=16) were hyperprolactinemic. The median PRL value was 27.85 (4.21-130) ng/mL in patients with PSIS and 5.57 (0-41.8) ng/mL in patients without PSIS. Additional hormone deficiencies, especially ACTH and LH were detected in follow-up. Conclusions Patients with normal or high prolactin levels deserve special attention regarding the possibility of PSIS. Furthermore, we emphasize the importance of regular follow-up and monitoring for multiple pituitary hormone deficiencies in all patients with a single pituitary hormone deficiency.
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    The diagnostic value of endoscopic narrow band imaging in helicobacter pylori gastritis in children
    (Aves, 2015-03-01) Özgür, Taner; Özkan, Tanju Başarır; Erdemir, Gülin; Özakın, Cüneyt; Yerci, Ömer; ÖZGÜR, TANER; Özkan, Tanju Başarır; Erdemir, Gülin; ÖZAKIN, CÜNEYT; YERCİ, ÖMER; Tıp Fakültesi; Mikrobiyoloji Ana Bilim Dalı; 0000-0002-9726-8219; 0000-0001-5740-9729; 0000-0001-5428-3630; AAG-8381-2021; AAG-8392-2021; DKU-2575-2022; GBW-0079-2022; EIS-5114-2022
    Background/Aims: In this study we aimed to investigate the sensitivity and specificity of Narrow Band Imaging (NBI) in H. pylori gastritis and compare them with those of rapid urease test and urea breath test.Materials and Methods: A hundred sixty-five children who admitted to Uludag University Pediatric Gastroenterology Unit between October 2009-March 2011 with upper gastrointestinal symptoms were consecutively enrolled. During the endoscopy procedure gastric corporeal, antral and fundal images were obtained, afterwards the same areas were visualized with narrow band imaging and images were recorded again.Results: The study included 68 (41.2%) boys and 97(58.8%) girls. The mean age of the patients were 11.88 +/- 4.55. Tissue culture positivity and/or histopathological staining for H. pylori was determined in 56 (33.9%) patients (Group 1) and the other patients (n:109, 43.6%) didn't have an evidence of H. pylori infection (Group 2). Narrow band images have supported H. pylori infection in 56.4%. The sensitivity of narrow band images for determining H. pylori infection was 92.86% (95% CI 82.7-98), specificity was 62.39% (95% CI 52.6-71.5).Conclusion: Our study is the first to show the role of NBI in diagnosing H. pylori infection in children, as well as determining the sensitivity and specificity of the technique. The specificity is low; however, we suggest that the specific mucosal view of H. pylori gastritis provided by NBI is useful for identifying the areas from which the biopsies should be taken. Moreover, by using this technique, treatment of H. pylori infection may be initiated immediately without performing rapid urease test and without waiting for histopathology report and tissue culture.