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PubMed | Hannover Medical School, University of Duisburg - Essen, Hacettepe University, University of Hamburg and 15 more.
Type: Clinical Trial | Journal: PloS one | Year: 2015

The extent and relevance of altered bone metabolism for statural growth in children with chronic kidney disease is controversial. We analyzed the impact of renal dysfunction and recombinant growth hormone therapy on a panel of serum markers of bone metabolism in a large pediatric chronic kidney disease cohort.Bone alkaline phosphatase (BAP), tartrate-resistant acid phosphatase 5b (TRAP5b), sclerostin and C-terminal FGF-23 (cFGF23) normalized for age and sex were analyzed in 556 children aged 6-18 years with an estimated glomerular filtration rate (eGFR) of 10-60 ml/min/1.73 m2. 41 children receiving recombinant growth hormone therapy were compared to an untreated matched control group.Standardized levels of BAP, TRAP5b and cFGF-23 were increased whereas sclerostin was reduced. BAP was correlated positively and cFGF-23 inversely with eGFR. Intact serum parathormone was an independent positive predictor of BAP and TRAP5b and negatively associated with sclerostin. BAP and TRAP5B were negatively affected by increased C-reactive protein levels. In children receiving recombinant growth hormone, BAP was higher and TRAP5b lower than in untreated controls. Sclerostin levels were in the normal range and higher than in untreated controls. Serum sclerostin and cFGF-23 independently predicted height standard deviation score, and BAP and TRAP5b the prospective change in height standard deviation score.Markers of bone metabolism indicate a high-bone turnover state in children with chronic kidney disease. Growth hormone induces an osteoanabolic pattern and normalizes osteocyte activity. The osteocyte markers cFGF23 and sclerostin are associated with standardized height, and the markers of bone turnover predict height velocity.


Oner O.,Dr Sami Ulus Childrens Hospital | Oner O.,Childrens Hospital | Oner P.,Dr Sami Ulus Childrens Hospital | Bozkurt O.H.,Diskapi Childrens Hospital | And 4 more authors.
Child Psychiatry and Human Development | Year: 2010

Objective: It has been suggested that both low iron and zinc levels might be associated with Attention Deficit Hyperactivity Disorder (ADHD) symptoms. However, the association of zinc and iron levels with ADHD symptoms has not been investigated at the same time in a single sample. Method: 118 subjects with ADHD (age = 7-14 years, mean = 9.8, median = 10) were included in the study. The relationship between age, gender, ferritin, zinc, hemoglobin, mean corpuscular volume and reticulosite distribution width and behavioral symptoms of children and adolescents with ADHD were investigated with multiple linear regression analysis. Results: Results showed that subjects with lower zinc level had higher Conners Parent Rating Scale (CPRS) Total, Conduct Problems and Anxiety scores, indicating more severe problems. CPRS Hyperactivity score was associated both with zinc and ferritin levels. Conners Teacher Rating Scale (CTRS) scores were not significantly associated with zinc or ferritin levels. Conclusions: Results indicated that both low zinc and ferritin levels were associated with higher hyperactivity symptoms. Zinc level was also associated with anxiety and conduct problems. Since both zinc and iron are associated with dopamine metabolism, it can be speculated that low zinc and iron levels might be associated with more significant impairment in dopaminergic transmission in subjects with ADHD. © Springer Science+Business Media, LLC 2010.


Baskin E.,Baskent University | Ozen S.,Hacettepe University | Cakar N.,Diskapi Childrens Hospital | Bayrakci U.S.,Baskent University | And 2 more authors.
Pediatric Nephrology | Year: 2010

Cyclophosphamide (CYC) has been the landmark in the treatment of lupus nephritis. However, long-term treatment with CYC is associated with significant side effects. We aimed to evaluate the efficacy of short-term intravenous (IV) CYC treatment as a remission induction treatment followed by azathioprine (AZA) or mycophenolate mofetil (MMF) as a maintenance treatment. Twenty patients (18 girls) with biopsy-proven class III (5) and IV (15) lupus nephritis were included in to the study. Detailed clinical and laboratory data and patient outcomes were evaluated. All patients received three methylprednisolone (MP) IV pulses, followed by oral prednisone 0.5-1 mg/kg per day and one IV pulse of CYC per month for 6 months. Azathioprine was started as a remission-maintaining treatment. In ten of 20 patients, treatment was switched to MMF. The mean age at the time of diagnosis was 16.11±3.49 years, and the mean duration of follow-up was 49.6±27 months. Fourteen patients (70%) had complete remission, three (15%) had partial remission, one (5%) continued to have active disease, and two (10%) progressed to end-stage renal disease. Nine of the patients (45%) with complete remission had received AZA, and switching to MMF increased complete remission rate (additional five patients; 25%). In conclusion, short-term (6-month) IV bolus CYC treatment followed by AZA is a safe and effective treatment in children with severe lupus nephritis, and using MMF increases remission rate in resistant cases. © 2009 IPNA.


Akkoyun I.,Dr Faruk Sukan Maternity And Children Hospital | Akbiyik F.,Diskapi Childrens Hospital | Altunkeser A.,Dr Faruk Sukan Maternity And Children Hospital
Journal of Pediatric Surgery | Year: 2011

Even in countries where it is considered endemic, splenic hydatid cyst is a very rare disease in childhood. Partial or total splenectomy has generally been the treatment of choice for this condition. This is the first report of laparoscopic cystectomy as treatment of isolated splenic hydatid cyst in childhood. A 10-year-old girl with isolated splenic hydatid cyst was successfully treated by laparoscopic cystectomy and splenic preservation. The authors have demonstrated that laparoscopic cystectomy for an isolated splenic hydatid cyst is technically feasible, safe, and is associated with a shorter hospital stay and good cosmetic appearance. © 2011 Elsevier Inc. All rights reserved.


Hanoglu D.,Hacettepe University | Ozgur T.,Hacettepe University | Ayvaz D.,Hacettepe University | Koker M.,Diskapi Childrens Hospital | Sanal O.,Hacettepe University
Journal of Investigational Allergology and Clinical Immunology | Year: 2011

Chronic granulomatous disease (CGD) is a primary immunodefi ciency disorder caused by inherited defects in the nicotinamide adenine dinucleotide phosphate oxidase complex. The neutrophils of patients with CGD can ingest bacteria normally, but the oxidative processes that lead to superoxide anion formation, hydrogen peroxide production, nonoxidative pathway activation, and bacterial killing are impaired. Serious infections result from microorganisms that produce catalase. Immunoglobulin levels of patients with CGD are usually normal or elevated. We describe a patient with CGD associated with hypogammaglobulinemia, an unusual co-occurrence. © 2011 Esmon Publicidad.


Akinci A.,Kudret Eye Hospital | Oner O.,Diskapi Childrens Hospital | Munir K.,Harvard University
Cornea | Year: 2010

Purpose: To evaluate the central corneal thickness (CCT) in children with intellectual disability (ID) and to determine the association between ID-related variables and CCT. Methods: A total of 77 subjects with ID and 38 healthy controls were included in the study. The subjects with ID were subdivided into mild (IQ 50-69; n = 34), moderate (IQ 35-49; n = 30), and severe (IQ <34; n = 13) groups and syndromic (n = 40) versus nonsyndromic (n = 37) distinctions. All children underwent CCT assessment by ultrasound pachymetry, in addition to complete ophthalmologic examination. Analysis of variance, χ test, and linear regression analysis were used for statistical analysis. Results: CCT was significantly higher in the ID group (549.7 ± 21.4 μm) than that the control group (521.6 ± 16.9 μm; P < 0.0001). Linear regression analysis revealed that presence of syndromic etiology significantly predicted higher CCT among the individuals with ID (P < 0.0001). Conclusions: Children with ID have an increased CCT compared with healthy controls. Syndromic etiology is the only variable that predicts higher CCT among the individuals with ID. Although the finding of an increased CCT in children with ID is statistically significant, it is not clinically significant. © 2010 by Lippincott Williams & Wilkins.


Aim: The normative data for the newborn clitoral length will enable physicians to avoid overdiagnosing or underdiagnosing disorders related to the condition. The is study is the first to investigate clitoris length in newborn females in Turkey. Materials and methods: A total of 325 newborns with normal gestational age born by uncomplicated spontaneous vaginal delivery were included in the study. The body weight, body length, head circumference, and clitoral size were measured. Results: A special equation was generated to estimate clitoris length, and clitoral length percentiles were prepared. The 3rd (2.00 mm) and 97th percentile (8.04 mm) values for clitoris length were determined. The best cut-off point for cases where the labia majora covered the clitoris was determined. Conclusion: The equations and the percentile curves presented herein could be used as guides for expected clitoris length. Further studies, including larger measurement numbers, would enable the development of more sensitive equations and percentiles. © Tübitak.


Senel E.,Diskapi Childrens Hospital | Akbiyik F.,Diskapi Childrens Hospital | Atayurt H.,Diskapi Childrens Hospital | Tiryaki H.T.,Diskapi Childrens Hospital
Pediatric Surgery International | Year: 2010

Introduction: Anorectal malformations comprise a wide spectrum of diseases. The main concerns for the surgeon in correcting these anomalies are bowel control, urinary control, and sexual function. The aim of this study was to evaluate fecal continence together with additional urinary anomalies in patients with anorectal malformations (ARM) and determine which of them is more troublesome for life quality in long-term follow-up. Materials and methods: A total of 93 cases followed up and treated at our clinic between 1983 and 2009 were evaluated for urinary pathologies, fecal continence, and their quality of life (QOL). Patients were classified according to the international system (Krickenbeck), and were evaluated on the basis of voluntary bowel movement, constipation, and fecal soiling. They were also classified as good, fair, and poor regarding fecal continence. The urinary system pathologies, the medical and surgical treatments received, and the pediatric nephrology follow-up results were all evaluated. The patients were grouped by age and their QOL scored using a telephone interview. Results: The mean age (±SD) was 8.47 ± 4.85 (3-25) years, and the mean follow-up period was 6.96 ± 4.55 (1-23) years. Evaluation of the patients in relation to their fecal continence revealed that 35 (37.6%) had constipation, 22 (23.6%) had fecal soiling, and voluntary bowel movements were absent in 7 (7.5%). Fecal continence was evaluated using the Krickenbeck classification, and it was good in 74 (79.6%), fair in 12 (12.9%), and poor in 7 (7.5%). QOL evaluation of these cases showed markedly decreasing QOL as the cases changed from good to poor fecal continence. Urinary system pathology was detected in 35 (37.6%) of the cases with 22 (23.7%) having severe uropathology. We compared the 22 patients with severe uropathology and 71 cases without severe uropathology for QOL and found the ARM group with severe uropathology to have significantly lower physical QOL, psychosocial QOL, and total QOL values. Reviewing the patients by fecal continence and urinary pathologies together regarding QOL showed that patients with good fecal continence and no additional urinary abnormality had the best QOL. The QOL decreased considerably in both the group with a fecal incontinence problem but no additional urinary abnormality and the group with good fecal continence but serious additional urinary abnormality. The QOL was worst in patients with urinary pathology and fecal incontinence. Discussion: Anorectal malformations constitute a wide spectrum of disease. Urinary anomalies and their complications significantly increase the morbidity in these children even after the correction of the ARM. In the evaluation of the published series, we noted that the main issue was fecal continence, and there was not enough data concerning the urinary system pathologies of the patients when they reach adult ages. Anorectal dysfunction has a negative effect on QOL in ARM patients, but is not life threatening. Such patients can lead normal life although the QOL is affected. However, vesicoureteral dysfunction can cause permanent damage in other organs. This damage may cause mortality depending on the level of the malformation. Evaluating the patients considering fecal continence and urinary system pathology together showed that nearly 10% of the ARM patients had serious problems affecting their long-term QOL related to fecal control, whereas nearly a fourth of the cases had additional anomalies of the urinary system that will affect the QOL despite all treatments used, as the development of pyelonephritis, hypertension, and end-stage renal disease should be expected in these patients. Conclusion: Urinary system anomalies in patients with ARM are at least as serious and complex as gastrointestinal system anomalies and create more problems than fecal incontinence during long-term follow-up. © 2010 Springer-Verlag.


Oner P.,Dr Sami Ulus Childrens Hospital | Oner O.,Dr Sami Ulus Childrens Hospital | Azik F.M.,Diskapi Childrens Hospital | Cop E.,Dr Sami Ulus Childrens Hospital | Munir K.M.,Harvard University
Pediatrics International | Year: 2012

Background: Iron is a co-factor of tyrosine hydroxylase which is a critical enzyme in dopamine synthesis. Dopamine has been implicated in the pathophysiology of attention deficit hyperactivity disorder (ADHD). Our objective was to investigate the association of ferritin level with parent and teacher ratings and cognitive measures after controlling for age, sex, ADHD subtype, comorbid conditions, hemoglobin, mean corpuscular volume and reticulocyte distribution width in a large sample. Methods: The study included 713 children and adolescents with ADHD (613 boys; age 7-15 years). Conners' Parent Rating Scale (CPRS) and Conners' Teacher Rating Scale (CTRS) were obtained. In a subgroup of patients we conducted Digit Span, Digit Symbol, Trail-making Tests as measures of attention and executive functioning. Results: Multiple regression analysis indicated that CPRS Hyperactivity score was significantly associated with ferritin level (B =-0.12; t =-3.1; P < 0.01). Other CPRS and CTRS scores as well as cognitive measures were not associated with ferritin level. Conclusions: Although it is not possible to make an inference on causality in cross-sectional studies, the results of this largest-scale cross-sectional field study to date suggest that lower ferritin level might be associated with parent-reported hyperactivity after controlling for important confounding factors. © 2012 The Authors. Pediatrics International © 2012 Japan Pediatric Society.


Objectives: To evaluate the success of the combined tubularized island flap (Duckett technique) and Thiersch-Duplay techniques in one-stage correction of proximal hypospadias. Methods: Thirty-four patients underwent surgical treatment for penoscrotal hypospadias in the last 9 years. Combined tubularized island flap (Duckett technique) and tubularized ventral skin (Thiersch-Duplay) procedures were performed in each case. The proximal urethra was repaired by the Thiersch-Duplay technique through the base of the phallus, and distal urethral repair was conducted via the tubularized island flap procedure. Results: The follow-up lasted from 5 months to 6 years (mean 4.1 years). The overall complication rate was 26%, but no recurrent chordee was detected. Recurrent fistulae were noted in 7 patients (20.5%). Anastomatic stricture was noted in 3 patients (8.8%). Conclusion: A combination of Duckett's preputial tube and the Thiersch-Duplay procedure was employed to treat 34 cases of severe hypospadias, with a success rate of 74%. In our procedure, the island flap tube is relatively short. Oblique anastomoses of proximal and distal tubes were made, and the pedicule of the flap supported the neourethra following successful interventions. Because of the low complication and high success rates, this combination of two procedures is recommended for repair of severe proximal hypospadias. © 2010 S. Karger AG, Basel.

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