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Öğe Atopy and allergic diseases in immunoglobulin A deficiency(Bilimsel Tip Yayinevi, 2012) Artac, Hasibe; Keles, Sevgi; Karagol, Cuneyt; Gokturk, Bahar; Reisli, IsmailObjective: Immunoglobulin (Ig) A deficiency is a common immune system disorder which causes morbidity especially in preschool children. The aim of this study is to evaluate atopy and allergic diseases of cases with IgA deficiency, determinate clinical results before and after four years of age. Materials and Methods: The data records of 295 children with IgA deficiency (272 partial, 23 selective IgA deficiency) between November 2001 and 2007 were examined retrospectively. Results: The mean age at the diagnosis was 64.3 +/- 43 (4-204) months, the mean age at the beginning of infection was 29.5 +/- 36 (1-168) months. There were 121 patients under the age of four years at the diagnosis time. The clinical presentations were recurrent bronchitis (40%), tonsillitis/pharyngitis (33%), sinusitis (6%), pneumonia (4%), acute otitis media (2%), acute gastroenteritis (0.3%) and neck abscess (0.3%), respectively. Eosinophilia was present in 25% of patients, increased IgE levels was present in 38% of patients, and they were correlated with each other. Atopy was detected in 31% of patients by spesific IgE and/or skinprick test positivity. Allergic disease prevalance was found as % 78 (60% asthma, 15% allergic rhinitis, 8% atopic dermatitis, 6% urticaria, 0.7% allergic conjunctivitis). During the clinical follow-up IgA levels reached to normal levels at 17 +/- 14 months ranged between 3 and 60 months. Three patients with selective IgA deficiency had bronchiectasia. Conclusion: We found that allergic diseases were increased in these patients. Serum IgA level should be tested not only for recurrent infections but also for patients with allergic symptoms.Öğe Efficacy of intravenous immunoglobulin treatment in immunocompromised children with H1N1 influenza: a clinical observation(Wiley-Blackwell, 2016) Gokturk, Bahar; Pekcan, Sevgi; Guner, Sukru Nail; Artac, Hasibe; Keles, Sevgi; Kirac, Mine; Reisli, IsmailBackground and AimsThe appropriate treatment of pandemic H1N1 influenza which was first identified in April 2009 in Mexico is insufficient especially for immunocompromised patients. We aimed to evaluate the features and prognostic factors of the children with H1N1, especially immunocompromised ones, and whether intravenous immunoglobulin G (IVIG) replacement could aid for a better outcome. MethodsTwenty-one hospitalized children with laboratory-confirmed H1N1 were evaluated retrospectively. Data were extracted from files and electronic medical records. ResultsThe median age was 37 (1-216) months; 62% of them were under 5years of age and 71.4% had one or more underlying disorders. Main symptoms were high fever, cough, fatigue and vomiting. Lower respiratory tract manifestations were seen in 66.6% of children. Mortality rate was 4.7%. The patient who died had the lowest lymphocyte (100/mm(3)), thrombocyte (21000/mm(3)) and highest blood urea nitrogen (87mg/dL) levels. Fifty-eight percent of evaluated patients had one of the primary immunodeficiency disorders. Surprisingly, none of the six patients with primary immunodeficiency who are on regular IVIG replacement needed intensive care unit and died. Although median durations of cough, fever and hospitalization were lower, they did not change statistically according to get IVIG replacement regularly (P=0.47, 0.97, 0.09, respectively). ConclusionOur study is important while it is the first one that shows the course of primary immunodeficient children with H1N1 infection who were on regular IVIG replacement. A trial of high-dose IVIG may be a useful adjunctive therapy in severe H1N1 influenza, particularly in the immunocompromised patients.Öğe The known genetic defects in common variable immunodeficiency(Bilimsel Tip Yayinevi, 2014) Kara, Reyhan; Gokturk, Bahar; Acar, AynurCommon variable immunodeficiency (CVID) is a relatively common form of immunodeficiency disorders which constitutes a mixed group of heterogeneous conditions linked by lack of immune globulin production and primary antibody failure. Recently, it is understood that various monogenic defects determine the variability in phenotype and have roles in the immunopathogenesis of CVID. In this review, the molecular defects related to CVID which are ICOS (inducible co-stimulator), TACI (transmembrane activator and calcium-modulator and cyclophilin ligand interactor), CD19, MSH5 (MutS Homologue 5 Mutations), BAFF-R (B cell activating factor receptor), CD20, CD81, CD21, LRBA (lipopolysaccharide-responsive beige-like anchor), and their genetic basis were aimed to be reviewed.Öğe OCULAR FINDINGS IN 22Q11.2 DELETION SYNDROME(Springer/Plenum Publishers, 2014) Gokturk, Bahar; Bozkurt, Banu; Yildirim, Mahmut Selman; Reisli, Ismail[Abstract Not Availabe]Öğe Ocular Findings in Children With 22q11.2 Deletion Syndrome(Slack Inc, 2016) Gokturk, Bahar; Topcu-Yilmaz, Pinar; Bozkurt, Banu; Yildirim, Mahmut Selman; Guner, Sukru Nail; Sayar, Esra Hazar; Reisli, IsmailPurpose: To identify the ocular features of children diagnosed as having 22q11.2 deletion syndrome in a Turkish population, which is the most common microdeletion syndrome with a wide range of facial and ocular abnormalities. Methods: Sixteen children aged between 4 months and 18 years with a microdeletion in chromosome 22q11.2 underwent a detailed ophthalmological examination including uncorrected and best corrected visual acuity testing, stereoscopic vision examination, biomicroscopic and indirect fundus examination, and ocular motility testing. Results: All patients had at least one ocular abnormality. The major abnormalities were eyelid abnormalities (eye hooding, narrow palpebral fissure, telecanthus, hypertelorism, sparse and thin eyebrows and eyelashes, blepharitis, and distichiasis), posterior embryotoxon, and tortuous retinal vessels in at least half of the patients. Other ophthalmological disorders were refractive errors, iris remnants, and strabismus. Conclusions: The chromosome 22q11.2 deletion syndrome is associated with a wide range of ocular disorders, which necessitates a comprehensive eye examination for appropriate treatment and follow-up. Ocular findings sometimes can provide a clue to the diagnosis of 22q11.2 deletion.Öğe Reduced CD19 expression and decreased memory B cell numbers in transient hypogammaglobulinemia of infancy(Springer-Verlag Italia Srl, 2013) Artac, Hasibe; Kara, Reyhan; Gokturk, Bahar; Reisli, IsmailWe aimed to evaluate the role of the CD19 complex in the pathogenesis of transient hypogammaglobulinemia of infancy (THI) and to better characterize the subsets of memory B cells. The study population consisted of 22 male and 14 female patients with a mean age at presentation of 20 +/- A 9.9 months. The CD19 complex and B cell subsets were evaluated by flow cytometry. While the CD19 median fluorescence index (MFI) in patients with THI was significantly lower than controls (122.9 +/- A 66.7 in patients; 184.2 +/- A 39 in controls, p < 0.01), expression of CD21 and CD81 was increased (94.4 +/- A 3, 96.8 +/- A 2.5 % in patients; 91 +/- A 3.9; 94.7 +/- A 3.5 % in controls, p < 0.01 vs. p < 0.05, respectively). The expressions of switched memory B cells and IgM memory B cells were found to be reduced in THI. Considering that the CD19 complex regulates the events following antigen stimulation, the change in CD19 complex detected in THI may be related to insufficiency of antibody production.Öğe Risk Factors and Clinical Determinants in Bronchiolitis of Infancy(Aves, 2020) Atay, Ozge; Pekcan, Sevgi; Gokturk, Bahar; Ozdemir, MehmetOBJECTIVES: The aims of this study was to demonstrate the viral pathogens, to evaluate the clinical prognosis, risk factors for recurrence, severity of acute viral bronchiolitis episodes among pediatric patients. MATERIALS AND METHODS: Our study included 101 children aged between 2 months and 2 years diagnosed with clinical bronchiolitis between September 2011 and April 2012. The demographics and clinical, laboratory, and radiological results of the patients were recorded. Nasopharyngeal swab samples were collected and analyzed through polymerase chain reaction (PCR) method. The patients were followed up for at least one year for new episodes, existence of wheezing, frequency of pulmonary infections, and progression of asthma. RESULTS: In half of the patients, determinants were indicated through the PCR method, with the most frequent being respiratory syncytial virus (44%). The frequency of bronchiolitis was higher in prematures (p<0.005). There was a relationship between crowded family structure and the existence of wheezing (p=0.003), increased recurrence (p=0.014), and need for inhaler treatment (p=0.014). The frequency was higher in patients living in urban cities (p<0.001), in houses with heating stoves (p=0.001), and in houses with smokers (p=0.001). Patients living in houses with heating stoves had more severe episodes (p=0.018). Recurrent wheezing and the need for regular inhaler usage were positively correlated with high API scores (p=0.008 and p=0.002, respectively). CONCLUSION: Prematurity, exposure to smoking, living in a crowded house with heating stoves, and an urban life are the risk factors for frequent bronchiolitis. The API can be used to predict the recurrence of bronchiolitis.Öğe Spontaneous pneumomediastinum as a complication in human bocavirus infection(Wiley-Blackwell, 2014) Pekcan, Sevgi; Gokturk, Bahar; Kucukapan, Hasibe Uygun; Arslan, Ugur; Findik, DuyguThe most common causes of spontaneous pneumomediastinum (SPM) in children are asthma attack and respiratory tract infection. Here, we describe a case of SPM in a human bocavirus-infected 2-year-old boy with bronchiolitis.Öğe Systemic Atopy and Immunoglobulin Deficiency in Turkish Patients with Vernal Keratoconjunctivitis(Taylor & Francis Inc, 2013) Bozkurt, Banu; Artac, Hasibe; Arslan, Nasha; Gokturk, Bahar; Bozkurt, Mete Kaan; Reisli, Ismail; Irkec, MuratPurpose: To determine the prevalence of systemic atopy and immunoglobulin (Ig) deficiencies in vernal keratoconjunctivitis (VKC). Methods: Sixty-seven VKC subjects (79.1% boys) with a mean age of 11.3 +/- 4.3 years were included. Serum Ig levels and specific IgE levels were measured using the nephelometric method and reversed enzyme immunoassay with sandwich ELISA technique, respectively. The patients underwent epidermal skin tests with commercial extracts. Results: Family history of atopy and associated systemic allergies were detected in 32.8 and 40.3% of the subjects, respectively. Blood eosinophilia, elevated total, and specific IgE and positive skin tests were detected in 33.8, 42.2, 50, and 35% of the subjects, respectively. Out of 62 subjects, low levels of IgA, IgG, IgM, and IgG3 were detected in 12.9, 8, 6.5, and 1.6% of the patients, respectively. Conclusion: IgE-mediated mechanisms are involved in approximately 40% of VKC patients. A new finding was the higher incidence of Ig deficiency.Öğe THE VALUE OF MEAN PLATELET VOLUME/PLATELET COUNT RATIO TO PREDICT 22q11.2 DELETION SYNDROME(Springer/Plenum Publishers, 2014) Gokturk, Bahar; Guner, Sukru Nail; Kara, Reyhan; Kirac, Mine; Keles, Sevgi; Artac, Hasibe; Reisli, Ismail[Abstract Not Availabe]Öğe Visual Hallucinations Induced by Clarithromycin in a Child: A Case Report and Literature Review(Lippincott Williams & Wilkins, 2019) Gokturk, Bahar; Erden, SemihObjective Our aim was to present a child with visual hallucinations possibly associated with oral clarithromycin administration. Case report A 4-year-old child was admitted to our hospital with an onset of visual hallucinations after taking the second dose of clarithromycin by mouth. The symptoms gradually disappeared in a week once the clarithromycin therapy had been discontinued. She was observed for a month without any symptoms or further treatment. She was suspected of having Hoigne syndrome (also called as antibiomania) induced by clarithromycin syndrome. Conclusion This report highlights neuropsychological adverse effects due to therapeutic doses of clarithromycin therapy as a possible adverse effect in children.Öğe Would mean platelet volume/platelet count ratio be used as a novel formula to predict 22q11.2 deletion syndrome?(Allergy Immunol Soc Thailand, 2016) Gokturk, Bahar; Guner, Sukru Nail; Kara, Reyhan; Kirac, Mine; Keles, Sevgi; Artac, Hasibe; Zamani, Ayse GulBackground: The diagnosis of 22q11.2 deletion syndrome depends on a time-consuming and expensive method, fluorescence in situ hybridisation (FISH). Objectives: We aimed to determine new parameters which can aid for in the diagnosis of 22q11.2 deletion syndrome. Methods: Twenty two patients with 22q11.2 or 10p13 deletion were evaluated retrospectively. Results: Facial-dysmorphism and mental-motor retardation were detected in 100% of patients. Mean platelet (PLT) counts were lower (224,980 versus 354,000, p = 0.001), mean PLT volume (MPV) (9.95 versus 7.07, p = 0.002), and MPV/PLTx10(5) ratios (5.36 versus 2.08, p < 0.001) were higher in patients with 22q11.2 deletion compared with the control group. Area under the receiver-operator characteristic (ROC) curve was 0.864, sensitivity was 84.6%, specificity was 90.9%, positive predictive value (PPV) was 91.7%, and negative predictive value (NPV) was 83.3% when MPV was 8.6. Area under ROC curve was 0.864, sensitivity was 76.9%, specificity was 90.1%, PPV was 90.1%, and NPV was 76.3% when PLT was 265,500. Area under ROC curve was 0.906, sensitivity was 84.6%, specificity was 100%, PPV was 100%, and NPV was 84.6% when MPV/PLTx10(5) was 3.3. Expression of PLT surface markers which were not in the GPIb-V-IX receptor complex (CD61, CD41a) increased as the surface area increased, but markers which were in a complex (CD42a, CD42b) did not change. Conclusions: High MPV/PLT value can be a good predictor for the diagnosis of 22q11.2 deletion syndrome. We suggest that in patients with facial dysmorphism and retardation in neurodevelopmental milestones and if MPV >= 8.6fl, MPV/PLTx10(5) ratio >= 3.3 and PLT count <= 265,500/mm(3), the patients should be tested by FISH analysis to confirm the 22q11.2 deletion. If there are no macrothrombocytes, the 10p13 deletion should be tested in suspected cases.Öğe Would mean platelet volume/platelet count ratio be used as a novel formula to predict 22q11.2 deletion syndrome?(Allergy Immunol Soc Thailand, 2016) Gokturk, Bahar; Guner, Sukru Nail; Kara, Reyhan; Kirac, Mine; Keles, Sevgi; Artac, Hasibe; Zamani, Ayse GulBackground: The diagnosis of 22q11.2 deletion syndrome depends on a time-consuming and expensive method, fluorescence in situ hybridisation (FISH). Objectives: We aimed to determine new parameters which can aid for in the diagnosis of 22q11.2 deletion syndrome. Methods: Twenty two patients with 22q11.2 or 10p13 deletion were evaluated retrospectively. Results: Facial-dysmorphism and mental-motor retardation were detected in 100% of patients. Mean platelet (PLT) counts were lower (224,980 versus 354,000, p = 0.001), mean PLT volume (MPV) (9.95 versus 7.07, p = 0.002), and MPV/PLTx10(5) ratios (5.36 versus 2.08, p < 0.001) were higher in patients with 22q11.2 deletion compared with the control group. Area under the receiver-operator characteristic (ROC) curve was 0.864, sensitivity was 84.6%, specificity was 90.9%, positive predictive value (PPV) was 91.7%, and negative predictive value (NPV) was 83.3% when MPV was 8.6. Area under ROC curve was 0.864, sensitivity was 76.9%, specificity was 90.1%, PPV was 90.1%, and NPV was 76.3% when PLT was 265,500. Area under ROC curve was 0.906, sensitivity was 84.6%, specificity was 100%, PPV was 100%, and NPV was 84.6% when MPV/PLTx10(5) was 3.3. Expression of PLT surface markers which were not in the GPIb-V-IX receptor complex (CD61, CD41a) increased as the surface area increased, but markers which were in a complex (CD42a, CD42b) did not change. Conclusions: High MPV/PLT value can be a good predictor for the diagnosis of 22q11.2 deletion syndrome. We suggest that in patients with facial dysmorphism and retardation in neurodevelopmental milestones and if MPV >= 8.6fl, MPV/PLTx10(5) ratio >= 3.3 and PLT count <= 265,500/mm(3), the patients should be tested by FISH analysis to confirm the 22q11.2 deletion. If there are no macrothrombocytes, the 10p13 deletion should be tested in suspected cases.
