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Item Coexistence of Familial Mediterranean Fever and Hyperimmunoglobulinemia D Syndrome in a Child(2015) Yilmaz, Resul; Sezer, Taner; Esmeray, Haluk; 0000-0001-7672-8100; 0000-0002-2278-1827; A-2825-2012; AAJ-5931-2021Hereditary periodic fever syndromes are Mendelian inherited single gene diseases which are also known as hereditary autoinflammatory syndromes, are characterized by recurrent attacks of fever and inflammation. Familial Mediterranean Fever and Hyperimmunoglobulinemia D syndrome are prototypes and are inherited autosomal recessively. The diagnosis is based on clinical course, family history and is confirmed with genetic mutation analysis. We describe a 5-year-old boy who had recurrent attacks of fever, skin rash, and cervical lymphadenopathy since he was 2 years old. His genetic analysis revealed homozygous M694V and V377I for MEFV and MVK gene respectively. Due to our knowledge, this is the first report of a patient who has both HIDS and FMF clinical and genetic features.Item Evaluation of video game playing status in school-age children with various variables(2021) Aydin, Beril; Oflu, Ayse; Yalcin, Sidika Songul; 34286323Objective: Excessive video game playing has several health implications on children. In this study, we evaluate the factors related to video game use in school-aged children. Material and Methods: This cross-sectional descriptive study included 160 children aged 6-8 years who applied to outpatient clinics at the Department of Pediatrics at Baskent University Hospital. Each parent completed a structured questionnaire including demographic information, video game use, average daily screen time, and parental habits and concerns about their children's screen use. Results: The mean age of first video game use was (mean +/- SD) 2.8 +/- 1.1 years. The minimum age for playing video games was 1 year. Male children spent more time playing video games. Both parental age and maternal education level were higher in the group of video gamers compared with non-gamers (p<0.05). Average time spent playing video games was 2.7 +/- 1.6 hours/day. The group of video gamers had a considerably younger age for starting watching television and higher rates for other video gamers at home than non-gamers (p=0.036 and p<0001, respectively). The group of video gamers had significantly higher rates for having a computer, tablet, and game console at home compared with non-gamers (p<0.05). Conclusions: Our study indicates a statistically significant relationship between the age of starting watching television, gender of child, parent's age, maternal education, and the categories of video gaming habits. Developing strategies toward avoiding early screen exposure in children should be taken into consideration, because it is directly related to video gaming habits in children.Item Children's Power of Food Scale: Turkish validity and reliability study(2021) Sahin-Bodur, Gulsum; Keser, Alev; Akcil-Ok, Mehtap; Unsal, Emine Nuket; Akin, Onur; 34472427Objective: The aim of the present study was to validate the Turkish version of the Children's Power of Food Scale (C-PFS-T) after translation of the original version. Design: The data were collected via face-to-face interviews using the C-PFS-T and a socio-demographic information form. BMI was calculated by dividing body weight by the square of the height. After the adaptation of the scale to Turkish language, validity and reliability analysis were conducted for the C-PFS-T. Setting: Gulhane Training and Research Hospital Department of Child Health and Diseases Nutrition and Diet Unit in Ankara. Participants: This research was conducted with volunteer children and adolescents between the ages of 9 and 16 years (n 268). Results: It was concluded that the 15-item C-PFS-T was collected under three factors as in the original version of the child version. Cronbach's alpha coefficient was found to be 0 center dot 878 for the scale. The confirmatory factor analysis results showed the acceptability and applicability of adapting the version of the C-PFS-T in terms of chi(2)/df (= 3 center dot 816), adjusted goodness-of-fit index (AGFI = 0 center dot 931), root mean square error of approximation (RMSEA = 0 center dot 082) and goodness-of-fit index (GFI = 0 center dot 852) fit indices. C-PFS-T total score's median value of obese group wasn't substantially different from normal weight group.Conclusions: It was concluded that the Turkish version of the C-PFS, which provides an assessment of the hedonic hunger status of children and adolescents with fifteen items and threesubdimensions, has sufficient reliability and validity to be applied to these subjects.Item How important urolithiasis is under 2 years of age?(2021) Yilmaz, Aysun Cetik; Unal, Necla; Tayfur, Asli Celebi; Buyukkaragoz, Bahar; 0000-0003-0774-4419; 34842953; AAD-1877-2021Urolithiasis (UL) is a common health problem in the world and the observed incidence of this disease is increasing in the infantile period. The study included cases of UL diagnosed before the age of two who had a comprehensive analysis for possible etiologic variables and were followed for a minimum of 6 months. Of the 60 patients included in the study, 37 were male, and the male/female ratio was 1.6. The average age at diagnosis is 8.5 +/- 4.5 months. Of the cases diagnosed 41 (68.3%) were before than 1 year of age. The average time for follow-up is 28.9 +/- 22.6 months. There was a family history of stone disease in 41 (68.3%) cases. Twenty-four (40%) patients were treated for dehydration at least once before stone disease was identified. The number of patients presenting with symptoms is 43 (71.7%). Restlessness was noted as the main symptom. In 17 (28.3%) patients, stone disease was found incidentally. Metabolic causes (n: 19, 31.6%) were determined to be the most common underlying cause, followed by UTI-related causes (n: 12, 20%). During the follow-up, 57 (64%) of the stones spontaneously disappeared. The size of 16 (18%) stones reduced, while the size of eleven remained same (13%). Following their absence, nine (15%) of the stones reappeared. The essential strategy is to identify high-risk groups, to closely monitor them, and to take preventative interventions against modifiable conditions such as dehydration if possible.Item Sublingual methylcobalamin treatment is as effective as intramuscular and peroral cyanocobalamin in children age 0-3 years(2021) Kilic, Betul Orhan; Kilic, Serhat; Eroglu, Elif Sahin; Gul, Eylem; Apak, Fatma Burcu Belen; 34871525Purpose: Vitamin B12 deficiency is a cause of preventable growth and developmental retardation in children. In this respect, alternative methods such as oral and sublingual treatments are being tried. We aimed to compare the efficacy of oral, sublingual, and intramuscular vitamin B12 treatments in children aged 0-3 years. Methods: The study included 158 patients with serum vitamin B12 deficiency (serum vitamin B12 level <300 ng/L) aged 0-3 years retrospectively. According to the vitamin B12 treatment modalities, the patients were divided into three groups as oral cyanocobalamin (group 1), sublingual methylcobalamin (group 2), and intramuscular cyanocobalamin (group 3). Results: The mean values of vitamin B12 levels increased to above 300 ng/L in all three groups. This increase was statistically significant for Group 1,2 and 3 (p<0.05). Conclusion: Sublingual methylcobalamin was determined as effective as oral and intramuscular cyanocobalamin improving vitamin B12 levels aged 0-3 years. What's already known about this topic? It is already known that intramuscular and oral cyanocobalamin treatments are effective in vitamin B12 deficiency of children. What does this article add? Sublingual methylcobalamin treatment, which is a new treatment method, was found to be as effective as oral and intramuscular cyanocobalamin treatments. To our knowledge, there is no study about sublingual treatment in children and comparing oral cyanocobalamin, intramuscular cyanocobalamin, sublingual methylcobalamin.Item Risk Factors for Postoperative Prolonged Mechanical Ventilation After Pediatric Liver Transplantation(2021) Sahinturk, Helin; Ozdemirkan, Aycan; Zeyneloglu, Pinar; Torgay, Adnan; Pirat, Arash; Haberal, Mehmet; 0000-0002-3462-7632; 0000-0003-0159-4771; 31084587; AAJ-8097-2021; AAJ-1419-2021Objectives: Duration of postoperative mechanical ventilation after pediatric liver transplant may influence pulmonary functions, and postoperative prolonged mechanical ventilation is associated with higher morbidity and mortality. Here, we determined its incidence and risk factors after pediatric liver transplant at our center. Materials and Methods: We retrospectively analyzed the records of 121 children who underwent liver transplant between April 2007 and April 2017 ( 305 total liver transplant procedures were performed during this period). Prolonged mechanical ventilation was defined as postoperative tracheal extubation after 24 hours. Results: Mean age at transplant was 6.2 +/- 5.4 years and 71/121 children (58.7%) were male. Immediate tracheal extubation was achieved in 68 children (56.2%). Postoperative prolonged mechanical ventilation was needed in 12 children (9.9%), with mean extubation time of 78.0 +/- 83.4 hours. Reintubation was required in 13.4%. Logistic regression analysis revealed that presence of preoperative hepatic encephalopathy (odds ratio of 0.130; 95% confidence interval, 0.027-0.615; P =.01), high aspartate amino transferase levels (odds ratio of 1.001; 95% confidence interval, 1.000-1.002; P =.02), intraoperative usage of more packed red blood cells (odds ratio of 1.001; 95% confidence interval, 1.000-1.002; P =.04), and longer surgery duration (odds ratio of 0.723; 95% confidence interval, 0.555-0.940, P =.01) were independent risk factors for postoperative prolonged mechanical ventilation. Although mean length of intensive care unit stay was significantly longer (12.6 +/- 13.6 vs 6.0 +/- 0.6 days; P =.001), mortality was similar in children with and without postoperative prolonged mechanical ventilation. Conclusions: Our results indicate that postoperative prolonged mechanical ventilation was needed in 9.9% of our children. Predictors of postoperative prolonged mechanical ventilation after pediatric liver transplant at our center were preoperative presence of hepatic encephalopathy, high aspartate amino transferase levels, intraoperative usage of more packed red blood cells, and longer surgery duration.Item Evaluation of cortical thickness and brain volume on 3 Tesla magnetic resonance imaging in children with frontal lobe epilepsy(2020) Rahatli, Feride Kural; Sezer, Taner; Has, Arzu Ceylan; Agildere, Ahmet Muhtesem; 0000-0002-2278-1827; 0000-0003-4223-7017; 0000-0002-4226-4034; 31802343; AAJ-5931-2021; AAB-5802-2020; AAL-9808-2021Background Frontal lobe epilepsy (FLE) is the most common epilepsy syndrome in the pediatric population; however, brain magnetic resonance imaging (MRI) of the children with FLE is frequently normal. We use both cortical thickness and brain volume measurements to report on cortical changes in children with FLE. Our aim was to determine cortical thickness and brain volume changes on 3 Tesla MRI of children with FLE and normal brain magnetic resonance imaging. Methods Twenty-seven children with FLE and 27 healthy controls received brain magnetic resonance imaging. Cortical thickness and regional brain volumes were assessed using three-dimensional volumetric T1-weighted imaging and patients were compared with controls. Results In children with FLE, statistically significant (p < 0.05) cortical thinning were found in the bilateral middle frontal gyrus, bilateral occipitotemporal and medial lingual gyrus, left subcallosal gyrus, left short insular gyrus, and right long insular gyrus. Statistically significant volume reductions in right and left hemisphere cortical white matter, total cortical white matter, bilateral thalamus, bilateral putamen, bilateral globus pallidus, right caudate nucleus, brain stem, and right cerebellar cortex were found. Conclusion Cortical thinning in frontal and extra-frontal lobes and volume loss in a variety of brain regions were found in children with FLE.Item The relationship between body mass index and renal length in obese children(2020) Parmaksiz, Gonul; Kekec, Senay Demir; Cengiz, Nurcan Dinler; Noyan, Aytul; 0000-0003-2373-1837; 31997076; AAD-5713-2021; AAM-2935-2021Background Obesity in the pediatric population is a severe public health problem and is associated with various comorbidities. Renal length is an important clinical parameter for the diagnosis and follow-up of renal diseases. The aim of this study was to determine the relationship between renal length (measured ultrasonographically) and body mass index (BMI) in obese children, and to develop nomograms for renal length according to BMI. Methods Renal ultrasound was performed in 368 children without renal disease. Each child's age, gender, weight, height, and BMI (kg/m(2)) were recorded. The children were divided into three groups according to BMI percentiles: obese group: BMI >= 95th percentile; overweight group: BMI 85th-94th percentile; normal weight group: BMI 5th-84th percentile. Results Weight, height, BMI, and right and left renal length differed significantly between the three groups (p = 0.001). There were significant correlations between renal length with age, weight, height, and BMI. Measurement of renal length was independently associated with BMI, age, and height. BMI was used to create renal length nomograms for obese children, based on multiple regression analysis (R-2 = 0.32 and p = 0.0001). Mean renal length was highest in the obese group (96.9 +/- 13.4 mm) and lowest in the normal weight group (88.3 +/- 12.9 mm). Conclusions Ultrasonographic measurement of the renal length according to BMI in children can be a useful method in evaluating these children. Smaller-than-normal kidneys can easily remain undiagnosed in obese and overweight children and this nomogram offers an additional method to evaluate the renal size in obese children.Item Inherited coagulation disorders in Turkish children: A retrospective, single-center cohort study(2020) Oner, Nergiz; Gursel, Turkiz; Kaya, Zuhre; Keskin, Ebru Yilmaz; Kocak, Ulker; Albayrak, Meryem; Yenicesu, Idil; Apak, Burcu Belen; Isik, Melek; 31980335Objective: This study aims to investigate the distribution, clinical characteristics and outcome of inherited coagulation disorders (ICD) in Turkish children. Subjects and methods: Data from all children (age < 18 years) with ICD examined in our center were retrospectively reviewed. Results: There were 403 children with ICD (233 males and 170 females) with a median age of four years at diagnosis. The percentages of von Willebrand disease (vWd), hemophilia and rare bleeding disorders (RBD) were 40 %, 34 % and 26 %, type-1, type-2 and type-3 vWd were 63 % 17 % and 20 %, hemophilia A and B were 84 % and 16 %, and severe, moderate and mild hemophilia were 48 %, 30 % and 22 %, respectively. Factor VII and FXI deficiencies were the most prevalent, comprising 56 % and 22 % of all children with RBD, respectively. Parental consanguinity rates were 72 % in type-3 vWd and 61 % in severe RBD. The overall prevalence of gastrointestinal bleedings was 4.5 % (18/403), intracranial bleeding (ICB) was 4.96 % (20/403), mortality from ICB was 30 % (6/20) and the overall mortality rate was 1.49 % (6/403). No life-threatening bleeding was seen during regular prophylaxis. Chronic arthropathy prevalence in severe hemophilia was 8 % with primary prophylaxis and 53 % with demand therap. Inhibitor prevalence was 14 % in hemophilia-A and 5 % in hemophiliaB. Conclusions: These data show that vWd is the most common ICD, type-3 vWd and RBD are prevalent due to frequent consanguineous marriages and diagnosis of ICD is substantially delayed in Turkish children. Prophylactic replacement therapy prevents occurrence of life-threatening bleedings and reduces the development of hemophilic arthropathy.Item Evaluation of non-infectious complications of peritoneal dialysis in children: a multicenter study(2020) Aksoy, Gulsah Kaya; Ekim, Mesiha; Bakkaloglu, Sevcan A.; Coskun, Seda; Delibas, Ali; Conkar, Secil; Yilmaz, Dilek; Kara, Aslihan; Saygili, Seha K.; Buyukkaragoz, Bahar; Yildirim, Zeynep Y.; Comak, Elif; Gurgoze, Metin K.; Sever, Lale; Noyan, Aytul; Bayazit, Aysun K.; Dusunsel, Ruhan; 32728843; AAD-5713-2021Background Peritoneal dialysis (PD) is the most common kidney replacement therapy in children. Complications associated with PD affect treatment success and sustainability. The aim of this study was to investigate the frequency of PD-related noninfectious complications and the predisposing factors. Methods Retrospective data from 11 centers in Turkey between 1998 and 2018 was collected. Non-infectious complications of peritoneal dialysis (NICPD), except metabolic ones, in pediatric patients with regular follow-up of at least 3 months were evaluated. Results A total of 275 patients were included. The median age at onset of PD and median duration of PD were 9.1 (IQR, 2.5-13.2) and 7.6 (IQR, 2.8-11.9) years, respectively. A total of 159 (57.8%) patients encountered 302 NICPD within the observation period of 862 patient-years. The most common NIPCD was catheter dysfunction (n = 71, 23.5%). At least one catheter revision was performed in 77 patients (28.0%). Longer PD duration and presence of swan neck tunnel were associated with the development of NICPD (OR 1.191; 95% CI 1.079-1.315, p = 0.001 and OR 1.580; 95% CI 0.660-0.883, p = 0.048, respectively). Peritoneal dialysis was discontinued in 145 patients; 46 of whom (16.7%) switched to hemodialysis. The frequency of patients who were transferred to hemodialysis due to NICPD was 15.2%. Conclusions Peritoneal dialysis-related non-infectious complications may lead to discontinuation of therapy. Presence of swan neck tunnel and long duration of PD increased the rate of NICPD. Careful monitoring of patients is necessary to ensure that PD treatment can be maintained safely.
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