Publications by authors named "Onder Yavascan"

65 Publications

A child with a severe headache: Answers.

Pediatr Nephrol 2021 Mar 1. Epub 2021 Mar 1.

Istanbul Medipol University, Departments of Pediatric Nephrology, Faculty of Medicine, Istanbul, Turkey.

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http://dx.doi.org/10.1007/s00467-021-04932-0DOI Listing
March 2021

A child with a severe headache: Questions.

Pediatr Nephrol 2021 Mar 1. Epub 2021 Mar 1.

Departments of Pediatric Nephrology, Faculty of Medicine, Istanbul Medipol University, Istanbul, Turkey.

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http://dx.doi.org/10.1007/s00467-021-04925-zDOI Listing
March 2021

Can we use copeptin as a biomarker for masked hypertension or metabolic syndrome in obese children and adolescents?

J Pediatr Endocrinol Metab 2020 Dec 19;33(12):1551-1561. Epub 2020 Nov 19.

Department of Pediatrics, Division of Nephrology, University of Health Sciences Izmir Tepecik Training and Research Hospital, Izmir, Turkey.

Objectives: Copeptin, the C-terminal part of arginine-vasopressin, is increased in hypertensive adolescents and closely associated with metabolic syndrome (MS). We aimed to investigate whether serum copeptin can be used to differentiate masked hypertension (MHT) and MS, and the role of sodium intake, natriuretic peptide response and renin-angiotensin-aldosterone system in MHT and MS in obese youth.

Methods: Obese children aged 10-18 years with normal office blood pressure measurements were included. Patients with MHT and normotension and those with MS and non-MS were evaluated separately. Biochemical parameters, copeptin, brain natriuretic peptide (BNP), aldosterone, renin, urine sodium, and protein were evaluated. Echocardiography, fundoscopic examination, and ambulatory blood pressure monitoring were performed.

Results: There were 80 (M/F=39/41) obese patients with a mean age of 13.78 ± 1.93 years. The cases with MHT, MS, and concomitant MHT and MS were 53,24, and 13%, respectively. Copeptin levels were similar among patients with and without MHT or MS (p>0.05). However, multivariate analysis revealed that copeptin significantly increased the probability of MHT (OR 1.01, 95% CI=1.001-1.018, p=0.033). Copeptin was positively correlated with daytime systolic and diastolic load, aldosterone, BNP, and urine microalbumin/creatinine levels (p<0.05). Linear regression analyses revealed that copeptin was significantly correlated with BNP regardless of having MHT or MS in obese youth. In the MHT group, 24-h sodium excretion was not significantly correlated with BNP.

Conclusion: Copeptin may be a beneficial biomarker to discriminate MHT, but not MS in obese children and adolescents. An insufficient BNP response to sodium intake might be one of the underlying causes of MHT in obese cases.
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http://dx.doi.org/10.1515/jpem-2020-0240DOI Listing
December 2020

Risk Assessment for Arrhythmia in Pediatric Renal Transplant Recipients.

Exp Clin Transplant 2021 Feb 1. Epub 2021 Feb 1.

From the Izmir Katip Celebi University, Faculty of Medicine, Department of Pediatrics, Division of Nephrology and Rheumatology, Izmir, Turkey.

Objectives: Renal transplant recipients are at risk for ventricular arrhythmia and sudden death. To assess that risk, we compared the ventricular repolarization markers of pediatric renal transplant recipients with those of healthy children.

Materials And Methods: We included 30 children and adolescents who were followed for at least 6 months after renal transplant; 30 age- and sex-matched children were included for the control group. Demographic features, medications, and laboratory findings were recorded. Blood pressure measurements, ventricular repolarization indexes including QT dispersion, corrected QT dispersion, T-wave peak-to-end interval dispersion, the T-wave peak-to-end interval ∕ QT ratio, the T-wave peak-to-end interval ∕ corrected QT ratio, left ventricular mass index, and relative wall thickness were compared between groups. In addition, the correlations of ventricular repolarization indexes with other variables were evaluated.

Results: Blood pressure standard deviation scores, the mean heart rate, QT dispersion, corrected QT dispersion, the T-wave peak-to-end interval ∕ QT ratio, the T-wave peak-to-end interval/corrected QT ratio, left ventricular mass index, and relative wall thickness values were significantly higher in renal transplant patients, whereas T-wave peak-to-end interval dispersion, ejection fraction, and fractional shortening were similar between groups. Although ventricular repolarization indexes were similar in patients with and without left ventricular hypertrophy, only corrected QT dispersion was significantly higher in patients with hypertension (P = .006). The only variable that significantly predicted prolonged corrected QT dispersion was the systolic blood pressure standard deviation score (P = .005, β = .403).

Conclusions: Ventricular repolarization anomalies, hypertension, left ventricular hypertrophy, and cardiac geometry irregularity may be observed after renal transplant in pediatric recipients despite acceptable allograft functions and normal serum electrolyte levels. Control of systolic blood pressure would decrease the risk of ventricular repolarization abnormalities, namely, the corrected QT dispersion. Follow-up of cardiovascular risks with noninvasive methods is recommended in all pediatric renal transplant recipients.
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http://dx.doi.org/10.6002/ect.2020.0162DOI Listing
February 2021

Cardiovascular risk assessment in children and adolescents with congenital solitary kidneys.

J Clin Hypertens (Greenwich) 2021 Feb 2;23(2):245-252. Epub 2021 Jan 2.

Department of Pediatric Cardiology, University of Health Sciences İzmir Tepecik Training and Research Hospital, İzmir, Türkiye.

Patients with solitary kidneys (SKs) are at risk of hypertension (HT) and associated end-organ damage. The authors aimed to evaluate whether children with congenital SKs (CSKs) have higher office, ambulatory, or central blood pressure (BP), increased arterial stiffness or left ventricular mass index, or any risk for arrhythmia. With this purpose, patients with CSK and healthy controls being followed up between January 2018 and June 2019 were enrolled in the study. Demographic, biochemical, and office blood pressure (BP) data were recorded. Then, ambulatory blood pressure monitoring (ABPM) and measurements of central BP (cBP), pulse wave velocity (PWV), and augmentation index (AIx@75) were obtained. Ventricular repolarization parameters were acquired by 12-lead electrocardiography. Left ventricular mass index (LVMI) and abdominal aortic stiffness parameters including strain, pressure strain elastic modulus (Ep), and normalized Ep (Ep*) were calculated with echocardiographic measurements. Finally, 36 children with CSK and 36 healthy controls were included. Serum creatinine, uric acid, total cholesterol levels, ABPM parameters, cBP levels, and PWV values were significantly higher, and eGFR levels were significantly lower in the CSK group. VR parameters, abdominal aortic stiffness indices, and LVMI were similar between the groups. CSK increased the risk of HT in ABPM (HT ) by 6 times. PWV was significantly correlated with Ep and Ep* in cases with CSK. Determination of cBP and PWV along with 24-hour ABPM would be a useful tool in children with CSK.
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http://dx.doi.org/10.1111/jch.14159DOI Listing
February 2021

DEMOGRAPHIC AND CLINICAL CHARACTERISTICS OF CHILDREN WITH AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE: A SINGLE CENTER EXPERIENCE.

Turk J Med Sci 2020 Dec 14. Epub 2020 Dec 14.

Background: In children with autosomal dominant polycystic kidney disease (ADPKD), clinical manifestations range from severe neonatal presentation to renal cysts found by chance. We aimed to evaluate demographic, clinical, laboratory findings, and genetic analysis of children with ADPKD.

Methods: We evaluated children diagnosed with ADPKD between January 2006 and January 2019. The diagnosis was established by family history, ultrasound findings, and/or genetic analysis. The demographic, clinical, and laboratory findings were evaluated retrospectively. Patients <10 years and ?10 years at the time of diagnosis were divided into 2 groups and parameters were compared between the groups.

Results: There were 41 children (M/F:18/23) diagnosed with ADPKD. The mean age at diagnosis was 7.2±5.1 (0.6?16.9) years and the follow-up duration was 59.34±40.56(8-198) months. Five patients (12%) were diagnosed as very early onset ADPKD. All patients had a positive family history. Genetic analysis was performed in 29 patients (PKD1 mutations in 21, PKD2 mutations in 1, no mutation in 3). Cysts were bilateral in 35 (85%) of the patients. Only one patient had hepatic cysts. No valvular defect was defined in 12 patients detected. Only 1 patient had hypertension. None of them had chronic kidney disease. No difference could be demonstrated in gender, laterality of the cysts, maximum cyst diameter, cyst or kidney enlargement, follow-up duration, or GFR at last visit between Groups 1 and 2.

Conclusions: The majority of children with ADPKD had preserved renal functions and slight cyst enlargement during their follow up. However, may have different renal problems deserving closed follow-up.
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http://dx.doi.org/10.3906/sag-2009-79DOI Listing
December 2020

A partial response to abatacept in a patient with steroid resistant focal segmental glomerulosclerosis.

Turk J Pediatr 2020 ;62(4):663-667

Department of Pediatric Nephrology, University of Health Sciences, Tepecik Training and Research Hospital, İzmir, Turkey.

Background: Herein we present our experience with abatacept in a patient diagnosed with primary focal segmental glomerulosclerosis (FSGS) and resistant to steroid and other immunosuppressives.

Case: A 17-year-old girl was diagnosed with idiopathic nephrotic syndrome (NS) at the age of 8 years. Kidney biopsy was performed when she did not respond to 6-weeks of steroid (2mg/kg) therapy followed by three doses of pulse methylprednisolone (PMP) and considered as steroid resistant NS. The biopsy revealed focal segmental glomerulosclerosis (FSGS) and cyclophosphamide was added to the steroid treatment but the patient had no response. The genetic analysis revealed G34G/A318A compound homozygous synonym aminoacid variation in NPHS2 gene, thus all immunosuppressive regimes were stopped and she was put on supportive treatment. Throughout this period, she had nephrotic range of proteinuria, however serum albumin levels were > 3g/dl. At the end of two years, the patient had NS with severe edema and hypoalbuminemia. When the genetic analysis was interpreted again, it was found to be consistent with a polymorphism rather than a mutation. Following 3 doses of PMP, oral steroid treatment was resumed and cyclosporine (CsA) was added to the treatment at the fifth year of follow up. However, she was unresponsive to CsA at the end of the first year as well as mycophenolate mofetil used for 12 months and rituximab used for 6 months, respectively. Then abatacept was instituted and proteinuria decreased below 1 gr/day and serum albumin levels increased to 3 g/dl at the end of 6 doses. Serum albumin levels remained stable in the following 7 months.

Conclusion: Partial remission including the decrease in proteinuria and increase in albumin levels achieved in our patient encourages the usage of abatacept in patients who do not respond to multiple immunosuppressive therapies.
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http://dx.doi.org/10.24953/turkjped.2020.04.018DOI Listing
January 2020

Calyceal diverticulum of the kidney in pediatric patients - Is it as rare as you might think?

J Pediatr Urol 2020 Aug 29;16(4):487.e1-487.e6. Epub 2020 May 29.

Katip Celebi University, Faculty of Medicine, Department of Pediatric Nephrology and Rheumatology, Izmir, Turkey.

Objective: Calyceal diverticulum (CD) is rarely diagnosed in children. It mimics other cystic lesions of the kidney and may be very difficult to identify. Displaying the communication of these fluid-filled cavities with the collecting system of the kidney via imaging methods is the key diagnostic method. The purpose of this study is to determine the presence of calyceal diverticulum in pediatric patients followed up due to simple renal cyst and/or parapelvic cyst.

Material, And Method: Patients who were newly diagnosed or being followed up with the ultrasonographic diagnosis of simple cyst(s) or parapelvic cyst(s) in the Department of Pediatric Nephrology of Izmir University of Health Sciences Tepecik Training and Research Hospital between December 2015 and July 2018 were included in the study. Age, gender, admission symptoms, recurrent urinary tract infections, stone disease, trauma history, accompanying urinary system and systemic anomalies, localization, and size of the cyst(s) at admission and during follow-up, treatment type and developing complications were noted. Contrast MR urography was applied to all patients.

Results: A total of 43 patients [mean age:132 ± 54.9 (12-228) months] including 25 girls were enrolled in the study. The mean follow-up period was 36. 74 ± 19 (1-90) months. The cysts were located mostly in the right kidney and also in the upper pole of the kidney. The mean cyst size was 20.4 ± 11 (8-58) mm at admission, and comparably 20.3 ± 8.6 (10-45) mm at the end of the follow-up. In contrast MR urography, a total of 16 cases were observed at first glance to be associated with pelvicalyceal system. However, in the postcontrast images taken at the 5th minute it was determined that 9 of them were compatible with calyceal diverticulum.

Conclusion: Although it is thought that well-circumscribed solitary renal fluid collections may be thought to be a simple renal cyst at first glance, the differential diagnosis of calyceal diverticulum (CD) should be absolutely kept in mind. Contrast studies and late-phase postcontrast images are needed for the diagnosis of CD. Although the standard management of CDs in children is conservative, surgical intervention may be rarely required.
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http://dx.doi.org/10.1016/j.jpurol.2020.05.151DOI Listing
August 2020

Recurrent debilitating calf pain associated with fasciitis in Familial Mediterranean fever and response to canacinumab.

Turk J Pediatr 2020 ;62(3):482-486

Division of Nephrology, Department of Pediatrics, Health Sciences University, İzmir Tepecik Training and Research Hospital, İzmir, Turkey.

Background: Myalgia is one of the presenting symptoms of Familial Mediterranean Fever (FMF), which is reported at a rate of 27-39.6%. Recurrent fasciitis in those cases are extremely rare. We aimed to present a case with FMF having radiologically proven fasciitis.

Case: An 11-year-old male patient with a diagnosis of FMF and M694V homozygote mutation, using colchicine regularly at a dose of 2 mg/day (0.08 mg/kg/day) for 4 years; was admitted to the hospital with severe pain and tenderness on the right calf. There were no accompanying symptoms like fever or abdominal pain. He described three similar episodes with pain and tenderness in left and right calves, which were not accompanied by fever in the last 6 months. The erythrocyte sedimentation rate and C-reactive protein levels were high and serum creatinine kinase was normal. The day after initiation of non-steroidal anti-inflammatory drug (NSAID), his complaints regressed. However, after a week, he again had a severe calf pain. Lower extremity arterial and venous doppler ultrasonography was normal. Increased peripheral signal intensity and fasciitis around the soleus muscle was defined in MRI. With NSAIDs, myalgia disappeared in a few days and acute phase reactants decreased within a week. In the follow up, canakinumab was prescribed due to febrile attacks as frequent as once a month and calf pain observed almost weekly. Thereafter, both febrile attacks and recurrent debilitating calf pain were completely ceased.

Conclusion: There are three cases in the literature with fasciitis related myalgia. With this case, we wanted to emphasize fasciitis as a cause of FMF associated myalgia on MRI. In such cases MRI may be helpful to demonstrate fascial involvement.
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http://dx.doi.org/10.24953/turkjped.2020.03.017DOI Listing
January 2020

A diagnostic dilemma in a child with macroscopic hematuria, nephrotic syndrome and hypocomplementemia: Answers.

Pediatr Nephrol 2020 07 16;35(7):1213-1216. Epub 2020 Jan 16.

Department of Pediatrics, Division of Nephrology, University of Health Sciences, Tepecik Training and Research Hospital, Izmir, Turkey.

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http://dx.doi.org/10.1007/s00467-019-04455-9DOI Listing
July 2020

A diagnostic dilemma in a child with macroscopic hematuria, nephrotic syndrome, and hypocomplementemia: Questions.

Pediatr Nephrol 2020 07 16;35(7):1211-1212. Epub 2020 Jan 16.

Department of Pediatrics, Division of Nephrology, University of Health Sciences, Tepecik Training and Research Hospital, Izmir, Turkey.

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http://dx.doi.org/10.1007/s00467-019-04451-zDOI Listing
July 2020

A Child Diagnosed With Treatment-Resistant Polyarteritis Nodosa: Can the Clinical Diagnosis Be Different?

Arch Rheumatol 2019 09 22;34(3):338-342. Epub 2019 Apr 22.

Department of Pediatric Nephrology, Katip Çelebi University Faculty of Medicine, Izmir, Turkey.

Polyarteritis nodosa (PAN) is a necrotizing systemic vasculitis involving the wall of small and medium sized arteries. The histological aspect is defined by the presence of fibrinoid necrosis and an infiltrate rich in neutrophil polynuclears in the artery wall and rare granulomas. CECR1 (Cat Eye Syndrome Chromosome Region 1) gene mutation causing adenosine deaminase 2 (ADA2) deficiency is also associated with systemic inflammation, vasculopathy, and frequently PAN. Strokes, neurological involvement, and gastrointestinal involvement have poor prognosis in the cases with ADA2 deficiency particularly in early stage. In this article, we report a 17-year-old male patient diagnosed with PAN who had severe gastrointestinal system involvement that was resistant to intensive and conventional immunosuppressive treatment and showed a fatal course despite the emergency surgical intervention. After the patient was exitus, he was detected to have a heterozygous mutation (V276A) of familial Mediterranean fever (FMF) and also a homozygous ADA2 mutation. The aim of this article is to highlight that ADA2 deficiency may be present in treatment-resistant PAN cases who apply due to severe systemic involvement. In this case, accompanying FMF mutation was also observed.
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http://dx.doi.org/10.5606/ArchRheumatol.2019.7075DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6768779PMC
September 2019

Ambulatory blood pressure monitoring parameters in obese children and adolescents with masked hypertension.

Blood Press Monit 2019 Dec;24(6):277-283

Department of Pediatrics Division of Nephrology, İzmir Tepecik Training and Research Hospital.

Objective: We aimed to compare the demographic, laboratory, and ambulatory blood pressure monitoring (ABPM) parameters of patients with masked hypertension (MHT), define factors predicting MHT, and determine the ABPM parameters affecting left ventricular mass index (LVMI) in obese youth.

Methods: Data of obese patients were evaluated retrospectively. Patients with ambulatory hypertension (AHT), white-coat hypertension (WCHT), MHT, or normotension (NT) were determined. Demographic and laboratory findings, office and ABPM measurements, blood pressure variability (BPV), and heart rate variability (HRV) were compared between the groups. The factors predicting MHT and the association between LVMI and ABPM, BPV/HRV parameters were analyzed.

Results: None of the 118 patients (M/F: 52/66) had WCHT. Three groups were formed: AHT (n: 60, 51%), MHT (n: 46, 39%), and NT (n: 12, 10%). Striae were significantly more frequent in AHT and MHT groups (P: 0.003). Cut-off levels for office BP measurements predicting MHT were 0.85 and 0.76 for systolic and diastolic BP (SBP and DBP) indexes, respectively. Most of the ABPM parameters of MHT group were as high as those of AHT group. On regression analysis, only daytime MAP had a positive (β: 0.340; P < 0.01) and diastolic dip (β: -0.204; P < 0.01) had a significantly negative association with LVMI.

Conclusion: Stria and cut-off levels for office SBP/DBP indexes, which were defined for the first time in this study, may determine the patients at risk of MHT. Although BPV or HRV had no relation to LVMI, daytime MAP and diastolic dip represented independent associations with LVMI.
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http://dx.doi.org/10.1097/MBP.0000000000000402DOI Listing
December 2019

Periumbilical swelling, erythema, and discharge in a girl: Answers.

Pediatr Nephrol 2020 03 16;35(3):411-413. Epub 2019 Sep 16.

Katip Celebi University, Faculty of Medicine, Department of Pediatric Nephrology, Izmir, Turkey.

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http://dx.doi.org/10.1007/s00467-019-04354-zDOI Listing
March 2020

Periumbilical swelling, erythema, and discharge in a girl: Questions.

Pediatr Nephrol 2020 03 16;35(3):409-410. Epub 2019 Sep 16.

Faculty of Medicine, Department of Pediatric Nephrology, Izmir Katip Celebi University, Izmir, Turkey.

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http://dx.doi.org/10.1007/s00467-019-04353-0DOI Listing
March 2020

Low levels of urinary epidermal growth factor predict chronic kidney disease progression in children.

Kidney Int 2019 07 20;96(1):214-221. Epub 2019 Mar 20.

Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, Heidelberg University Hospital, Heidelberg, Germany. Electronic address:

Urinary epidermal growth factor (uEGF) has recently been identified as a promising biomarker of chronic kidney disease (CKD) progression in adults with glomerular disease. Low levels of uEGF predict CKD progression and appear to reflect the extent of tubulointerstitial damage. We investigated the relevance of uEGF in pediatric CKD. We performed a post hoc analysis of the Cardiovascular Comorbidity in Children with CKD (4C) study, which prospectively follows children aged 6-17 years with baseline estimated glomerular filtration rate (eGFR) of 10-60 ml/min/1.73 m. uEGF levels were measured in archived urine collected within 6 months of enrollment. Congenital abnormalities of the kidney and urinary tract were the most common cause of CKD, with glomerular diseases accounting for <10% of cases. Median eGFR at baseline was 28 ml/min/1.73 m, and 288 of 623 participants (46.3%) reached the composite endpoint of CKD progression (50% eGFR loss, eGFR < 10 ml/min/1.73 m, or initiation of renal replacement therapy). In a Cox proportional hazards model, higher uEGF/Cr was associated with a decreased risk of CKD progression (HR 0.76; 95% CI 0.69-0.84) independent of age, sex, baseline eGFR, primary kidney disease, proteinuria, and systolic blood pressure. The addition of uEGF/Cr to a model containing these variables resulted in a significant improvement in C-statistics, indicating better prediction of the 1-, 2- and 3-year risk of CKD progression. External validation in a prospective cohort of 222 children with CKD demonstrated comparable results. Thus, uEGF may be a useful biomarker to predict CKD progression in children with CKD.
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http://dx.doi.org/10.1016/j.kint.2019.01.035DOI Listing
July 2019

Can we predict vesicoureteral reflux resolution in patients with non-neurogenic lower urinary tract dysfunction?

Int J Urol 2019 06 14;26(6):638-642. Epub 2019 Mar 14.

Department of Urology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Objective: To analyze factors influencing reflux resolution in patients with the coexistence of non-neurogenic lower urinary tract dysfunction and vesicoureteral reflux.

Methods: The data of 153 children who were diagnosed with vesicoureteral reflux and accompanying non-neurogenic lower urinary tract dysfunction between 2010 and 2015 were retrospectively evaluated. Patients with neurogenic and anatomical malformations, monosymptomatic nocturnal enuresis, previous history of vesicoureteral reflux surgery, irregular and/or incomplete follow-up data were excluded. After exclusion of 55 patients, 98 patients were enrolled in this study. Patients were divided into two groups according to the presence of spontaneous vesicoureteral reflux resolution during the follow-up period. Group 1 consisted of 54 children with spontaneous vesicoureteral reflux resolution, whereas group 2 included 44 children without resolution. Medical history, physical examination, urinalysis, uroflowmetry combined with electromyography, ultrasonography, as well as the Dysfunctional Voiding and Incontinence Symptom Score questionnaire were also evaluated.

Results: The mean age at presentation was 7.57 ± 0.23 years (range 5-13 years), and the mean follow-up period was 28.3 months. Significant differences were noted between the two groups in terms of dysfunctional voiding and incontinence symptom score, bladder wall thickness, and the post-void residual urine volumes. In addition, lower urinary tract symptoms, namely frequency, urgency and daytime incontinence, were found to be higher in group 2. In multivariate analysis, post-void residual urine volume and Dysfunctional Voiding and Incontinence Symptom Score were found to affect reflux resolution rates (P = 0.002, P = 0.002, respectively).

Conclusions: The absence of significant post-void residual urine volume, and a low Dysfunctional Voiding and Incontinence Symptom Score increase the likelihood of spontaneous resolution rates of vesicoureteral reflux in children with non-neurogenic lower urinary tract dysfunction.
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http://dx.doi.org/10.1111/iju.13946DOI Listing
June 2019

A rare cause of neonatal hypertension: Congenital mesoblastic nephroma.

Turk J Pediatr 2018 ;60(2):198-200

Departments of Pediatric Nephrology, Tepecik Training and Research Hospital, İzmir, Turkey.

Soyaltın E, Alaygut D, Alparslan C, Özdemir T, Arslansoyu-Çamlar S, Mutlubaş F, Kasap-Demir B, Yavaşcan Ö. A rare cause of neonatal hypertension: Congenital mesoblastic nephroma. Turk J Pediatr 2018; 60: 198-200. A rare cause of neonatal hypertension: Congenital Mesoblastic Nephroma (CMN) is a rare renal tumor in childhood and has been reported with palpable abdominal mass, hypertension, hematuria, polyuria and hypercalcemia. Histopathologically it has been classified into two histological types: classic and cellular. We present a 32-week gestation infant and his histopathology reports of cellular CMN presented with refractory hypertension.
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http://dx.doi.org/10.24953/turkjped.2018.02.014DOI Listing
May 2019

A novel homozygous W99G mutation in CLDN-16 gene causing familial hypomagnesemic hypercalciuric nephrocalcinosis in Turkish siblings.

Turk J Pediatr 2018 ;60(1):76-80

Departments of Pediatric Nephrology, İzmir Tepecik Training and Research Hospital, , Izmir.

Alparslan C, Öncel EP, Akbay S, Alaygut D, Mutlubaş F, Tatlı M, Konrad M, Yavaşcan Ö, Kasap-Demir B. A novel homozygous W99G mutation in CLDN-16 gene causing familial hypomagnesemic hypercalciuric nephrocalcinosis in Turkish siblings. Turk J Pediatr 2018; 60: 76-80. Familial hypomagnesemic hypercalciuric nephrocalcinosis (FHHNC) (OMIM: 248250) is characterized by hypomagnesemia, hypercalciuria and nephrocalcinosis. FHHNC inevitably progresses to end-stage renal disease in decades. Mutations in CLDN-16 and CLDN-19 genes are associated with disrupted magnesium handling in the thick ascending limp of Henle`s loop. Patients with mutations in these genes share similar clinical features, and those with CLDN-19 gene mutations have ocular findings in addition. A 2-month-old boy, was admitted to our clinic with the complaints of upper respiratory tract infection. He was the first-born child of consanguineous parents. Laboratory findings revealed hypocalcemia and hypomagnesemia. Bilateral medullary nephrocalcinosis was detected on abdominal ultrasound. His ophthalmologic examination was unremarkable. With hypomagnesemia, hypercalciuria and nephrocalcinosis, the patient was considered to have FHHNC. Oral magnessium supplementation was initiated. Four years of follow-up has been completed uneventfully. When 6-days-old the brother of the case above was admitted with seizure. The patient was resistant to calcium and anticonvulsant drugs and the seizure activity could only be controlled after magnesium infusion. Biochemistry profile revealed hypocalcemia and hypomagnesemia. Urinary calcium extraction was 11 mg/kg/day. Medullary nephrocalcinosis was reported on renal ultrasound. His eye examination, echocardiography, transfontanel ultrasound and electroencephalography were normal. Due to the triad of hypomagnesemia, hypercalciuria and nephrocalcinosis, and the medical history of his elder brother, he was diagnosed with FHHNC. After correction of the electrolyte abnormalities, he was discharged from hospital and is currently being followed-up without any problem. In this manuscript, we shared our experience about a novel homozygous mutation (W99C) in CLDN-16 gene causing FHHNC in a couple of Turkish siblings.
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http://dx.doi.org/10.24953/turkjped.2018.01.011DOI Listing
January 2019

JC polyomavirus replication and associated disease in pediatric renal transplantation: an international CERTAIN Registry study.

Pediatr Nephrol 2018 Dec 30;33(12):2343-2352. Epub 2018 Jul 30.

Department of Pediatrics I, University Children's Hospital, Im Neuenheimer Feld 430, D-69120, Heidelberg, Germany.

Background: JC polyomavirus (JCPyV)-associated nephropathy (JCPyVAN) is a severe, but rare complication in adult renal transplant (RTx) recipients. Related data in pediatric patients are scarce.

Methods: Based on the CERTAIN Registry, we therefore performed a multi-center, retrospective study on the JCPyV antibody status, prevalence of JCPyV replication, and its associated disease in 139 pediatric RTx recipients (mean age, 8.5 ± 5.3 years). JCPyV DNA in plasma and/or urine was measured by quantitative PCR at a median time of 3.2 (IQR, 0.3-8.1) years post-transplant.

Results: 53.2% of patients were JCPyV-seronegative prior to transplantation; younger age was associated with JCPyV seronegativity. 34/139 (24.5%) patients post-transplant showed active JCPyV replication in either urine (22.0%), plasma (13.4%), or both (7.6%). JCPyV viremia occurred significantly (p < 0.001) more often in patients with viruria (34.6%) than in those without (7.6%), but 7/118 (5.9%) had isolated viremia. High-level viruria (> 10 copies/mL) was found in 29.6% of viruric patients. A higher net state of immunosuppression constituted an independent risk factor for JCPyV replication both in urine and plasma (OR 1.2, p < 0.02). Male patients tended to have a higher risk of JCPyV viremia than females (OR 4.3, p = 0.057). There was one male patient (0.7%) with JCPyVAN 7 years post-transplant, which resolved after reduction of immunosuppressive therapy. No patient exhibited progressive multifocal leukoencephalopathy.

Conclusions: This first multi-center study on JCPyV in pediatric renal transplant recipients shows that JCPyV replication is common (24.5%), with strong immunosuppression being a significant risk factor, but associated nephropathy is rare.
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http://dx.doi.org/10.1007/s00467-018-4029-9DOI Listing
December 2018

Successful Management of a Rare Cause of Hemolytic Uremic Syndrome With Eculizumab in a Child.

J Pediatr Hematol Oncol 2018 07;40(5):401-404

Departments of Pediatric Nephrology.

Background: Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. It very rarely coexists with acute lymphoblastic leukemia (ALL) emerging before, simultaneously, or after the diagnosis has been made, and management of the patient may be difficult.

Case: We present the case of a 7-year-old boy who was diagnosed with HUS and initially managed by hemodialysis (HD). Thereafter, HUS progressed, and neurological findings developed. The patient was treated with eculizumab, agressive blood pressure control, and antiepileptic drugs. At the fifth month of follow-up, the patient was diagnosed with acute B-cell lymphoblastic leukemia with fever, bone pain, hepatosplenomegaly, and pancytopenia. After initiation of ALL treatment, he had no episodes of HUS, despite cessation of eculizumab.

Conclusion: In conclusion, eculizumab may be a treatment of choice to prevent further systemic damage in recurrent HUS episodes of patients with borderline changes in the bone marrow until ALL is constantly diagnosed.
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http://dx.doi.org/10.1097/MPH.0000000000001121DOI Listing
July 2018

Can a hand radiograph indicate a special diagnosis in a child with chronic kidney disease? Answers.

Pediatr Nephrol 2018 05 24;33(5):801-803. Epub 2017 Jul 24.

Department of Pediatrics, Division of Nephrology, İzmir Tepecik Training and Research Hospital, İzmir, Turkey.

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http://dx.doi.org/10.1007/s00467-017-3742-0DOI Listing
May 2018

Can a hand radiograph indicate a special diagnosis in a child with chronic kidney disease? Questions.

Pediatr Nephrol 2018 05 12;33(5):799-800. Epub 2017 Jul 12.

İzmir Tepecik Training and Research Hospital Department of Pediatrics Division of Nephrology, İzmir, Turkey.

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http://dx.doi.org/10.1007/s00467-017-3740-2DOI Listing
May 2018

Pure Red Cell Aplasia Due to Parvovirus B19: Erythropoietin-Resistant Anemia in a Pediatric Kidney Recipient.

Exp Clin Transplant 2017 06 6;15(3):369-371. Epub 2017 Apr 6.

From the Department of Pediatric Hematology and Oncology, Tepecik Education and Training Hospital, Izmir, Turkey.

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http://dx.doi.org/10.6002/ect.2016.0263DOI Listing
June 2017

Peritoneal Dialysis Access Revision in Children: Causes, Interventions, and Outcomes.

Clin J Am Soc Nephrol 2017 01 29;12(1):105-112. Epub 2016 Nov 29.

Due to the number of contributing authors, the affiliations are provided in the Supplemental Material.

Background And Objectives: Little published information is available about access failure in children undergoing chronic peritoneal dialysis. Our objectives were to evaluate frequency, risk factors, interventions, and outcome of peritoneal dialysis access revision.

Design, Setting, Participants, & Measurements: Data were derived from 824 incident and 1629 prevalent patients from 105 pediatric nephrology centers enrolled in the International Pediatric Peritoneal Dialysis Network Registry between 2007 and 2015.

Results: In total, 452 access revisions were recorded in 321 (13%) of 2453 patients over 3134 patient-years of follow-up, resulting in an overall access revision rate of 0.14 per treatment year. Among 824 incident patients, 186 (22.6%) underwent 188 access revisions over 1066 patient-years, yielding an access revision rate of 0.17 per treatment year; 83% of access revisions in incident patients were reported within the first year of peritoneal dialysis treatment. Catheter survival rates in incident patients were 84%, 80%, 77%, and 73% at 12, 24, 36, and 48 months, respectively. By multivariate logistic regression analysis, risk of access revision was associated with younger age (odds ratio, 0.93; 95% confidence interval, 0.92 to 0.95; P<0.001), diagnosis of congenital anomalies of the kidney and urinary tract (odds ratio, 1.28; 95% confidence interval, 1.03 to 1.59; P=0.02), coexisting ostomies (odds ratio, 1.42; 95% confidence interval, 1.07 to 1.87; P=0.01), presence of swan neck tunnel with curled intraperitoneal portion (odds ratio, 1.30; 95% confidence interval, 1.04 to 1.63; P=0.02), and high gross national income (odds ratio, 1.10; 95% confidence interval, 1.02 to 1.19; P=0.01). Main reasons for access revisions included mechanical malfunction (60%), peritonitis (16%), exit site infection (12%), and leakage (6%). Need for access revision increased the risk of peritoneal dialysis technique failure or death (hazard ratio, 1.35; 95% confidence interval, 1.10 to 1.65; P=0.003). Access dysfunction due to mechanical causes doubled the risk of technique failure compared with infectious causes (hazard ratio, 1.95; 95% confidence interval, 1.20 to 2.30; P=0.03).

Conclusions: Peritoneal dialysis catheter revisions are common in pediatric patients on peritoneal dialysis and complicate provision of chronic peritoneal dialysis. Attention to potentially modifiable risk factors by pediatric nephrologists and pediatric surgeons should be encouraged.
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http://dx.doi.org/10.2215/CJN.05270516DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5220659PMC
January 2017

Pretransplant Stable Systolic Cardiac Functions Play an Important Role in Short-term Systolic Cardiac Functions After Kidney Transplant in Children.

Exp Clin Transplant 2017 Feb 11;15(1):34-39. Epub 2016 Feb 11.

From the Department of Pediatrics and Pediatric Nephrology, Izmir Tepecik Training and Research Hospital, Izmir, Turkey.

Objectives: In this study, our aim was to evaluate the systolic cardiac parameters and related risk factors in children within 6 months after kidney transplant.

Materials And Methods: We retrospectively evaluated 24 children who received kidney transplants. Clinical and laboratory parameters before and after transplant were recorded. Results were evaluated statistically, with a P value less than .05 considered significant.

Results: Before transplant, systolic cardiac functions were within normal limits. After transplant, ejection fraction (63.35% ± 5.38% vs 66.95% ± 4.62%; P = .01) was significantly increased and left ventricular mass index (32.63 ± 17.21 g/m2.7 vs 31.29 ± 15.65 g/m2.7; P = .78) was not significantly decreased, whereas fractional shortening (52.16% ± 15.32% vs 59.8% ± 12.94%; P = .54) did not change. Systolic blood pressure, systolic blood pressure index, diastolic blood pressure, and diastolic blood pressure index values were not statistically different before and after transplant (P > .05). The number of antihypertensive agents was significantly decreased (P = .001). Before and after transplant, cardiac geometry was normal in 15 patients (62.5%) and 17 patients (70.8%).

Conclusions: Our patients, who had stable systolic cardiac function before transplant, showed further improvements in systolic cardiac function even within 6 months after transplant. Therefore, strictly monitored and controlled blood pressure, volume, anemia, and nutrition in children before transplant may play important roles in achieving better cardiac systolic function after kidney transplant.
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http://dx.doi.org/10.6002/ect.2015.0208DOI Listing
February 2017

Evaluation of Nutritional Status in Children during Predialysis, or Treated By Peritoneal Dialysis or Hemodialysis.

J Trop Pediatr 2016 Jun 13;62(3):178-84. Epub 2016 Jan 13.

Departments of Biochemistry, Adnan Menderes University Faculty of Medicine, Aydın 09100, Turkey.

Objective: Malnutrition is one of the major causes of morbidity and mortality in children with chronic kidney disease (CKD). The objective of this study was to evaluate nutritional status of children with stage 3-4 CKD and treated by peritoneal dialysis or hemodialysis using anthropometric measurements, biochemical parameters and bioelectrical impedance analysis.

Patients And Methods: The study included a total of 52 patients and 46 healthy children.

Results: In anthropometric evaluation, the children with CKD had lower values for standard deviation score for weight, height, body mass index, skinfold thickness and mid-arm circumference than those of healthy children (p < 0.05). The fat mass (%) and the body cell mass (%) measurements performed by bioelectrical impedance analysis were lower compared with the control group (p < 0.05).

Conclusion: It is considered that bioelectrical impedance analysis measurement should be used with anthropometric measurements, which are easy to perform, to achieve more accurate nutritional evaluation in children.
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http://dx.doi.org/10.1093/tropej/fmv094DOI Listing
June 2016

Toilet training age and influencing factors: a multicenter study.

Turk J Pediatr 2015 Mar-Apr;57(2):172-6

Department of Urology Tepecik Training and Research Hospital, Ege University Faculty of Medicine, Izmir, Turkey.

To determine toilet training age and the factors influencing this in our country, 1500 children who had completed toilet training were evaluated in a multicenter study. The mean age of toilet training was 22.32 ± 6.57 months. The duration it took to complete toilet training was 6.60 ± 2.20 months on the average. In univariant analysis, toilet training age increased as the parental education level, specifically that of the mother, increased. The training age of children whose mothers had over 12 years of education differed significantly from that of children of mothers with less education. There was no significant difference in toilet training age with regard to the education level of the father, or the employment status of the mother. We also found significant differences with respect to family income level, toilet type and training method. In multivariant analysis, family income >5000 TL and use of a potty chair were determined to be factors affecting toilet training age. In conclusion, toilet training age in Turkey, a developing country, was found to be lower than that in developed countries.
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August 2016

A Comparison of Bladder Catheterization and Suprapubic Aspiration Methods for Urine Sample Collection From Infants With a Suspected Urinary Tract Infection.

Clin Pediatr (Phila) 2016 Aug 29;55(9):819-24. Epub 2015 Sep 29.

Tepecik Teaching and Research Hospital, Izmir, Turkey.

This study compares 2 sampling methods for urine cultures in young infants. We analyzed data on urine samples obtained from 83 infants using 2 sources of urine: suprapubic bladder aspiration (SPA) and bladder catheterization. All specimens were subjected to both urinalysis and culture, and the results compared. Eighty-three infants with positive urine culture results obtained by bladder catheterization were subjected to SPA. Of these, only 24 (28.9%) and 20 (24%) yielded positive urine culture and abnormal urinalysis data, respectively. Samples obtained via catheterization had a high false-positive rate (71.1%). The sensitivity and specificity of urinalysis were 66.7% (95% CI, 44.68% to 84.33%) and 93.22% (95% CI, 83.53% to 98.08%), respectively. In infants younger than 12 months, SPA is the best method to avoid bacterial contamination, showing better results than transurethral catheterization.
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http://dx.doi.org/10.1177/0009922815608278DOI Listing
August 2016