Publications by authors named "Caner Alparslan"

42 Publications

Brain death in a child as a result of COVID-19-associated acute stroke: The first case.

J Paediatr Child Health 2021 Mar 12. Epub 2021 Mar 12.

Pediatric Intensive Care Department, Saglik Bilimleri Üniversitesi Gazi Yasargil Training and Research Hospital, Diyarbakir, Turkey.

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http://dx.doi.org/10.1111/jpc.15421DOI 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

Retrospective evaluation of the pediatric multicystic dysplastic kidney patients: Experience of two centers from southeastern of Turkey.

Turk J Med Sci 2021 Jan 29. Epub 2021 Jan 29.

Background/aim: The objective of this study is to determine the clinical features of unilateral multicystic dysplastic kidney (MCDK) patients. Materiald and methods: The demographic, clinical, laboratory and radiologic features of MCDK patients between January 2008-June 2019 were retrospectively evaluated.

Results: A total of 111 [59 (53.2%) male and 52 (46.8%) female] patients with MCDK were followed for a mean period of 41.89±32.03 months. MCDK were located on left and right sides in 46 (41.4%) and 65 (58.6%) of children, respectively (p>0.05). A total of 87 (78.4%) patients had antenatal diagnosis. The mean age at diagnosis was 13.7±34.2 months. Of the 49 voiding cystourethrogram (VCUG) performed patients, vesicoureteral reflux (VUR) was detected in 11 patients (22.4%). Other associated urological anomalies were detected in 12 (10.8%) patients. On Tc-99m dimercaptosuccinic acid (DMSA) scintigraphy which was performed in all patients showed scarring in four children. Eight patients had history of urinary tract infection (UTI) (7.2%). Renal failure, hypertension and proteinuria was diagnosed in three children (2.7%). Sixthy-nine (62%) of patients developed compensatory hypertrophy.

Conclusion: All cases should be followed-up closely and VCUG should be reserved for patients with recurrent UTI and other urological problems indicated by ultrasonography and abnormal DMSA scan results.
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http://dx.doi.org/10.3906/sag-2011-175DOI Listing
January 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

Sex and age as determinants for high blood pressure in pediatric renal transplant recipients: a longitudinal analysis of the CERTAIN Registry.

Pediatr Nephrol 2020 03 7;35(3):415-426. Epub 2019 Dec 7.

Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Carl-Neuberg-Str. 1, 30625, Hannover, Germany.

Background: High prevalence of arterial hypertension is known in pediatric renal transplant patients, but how blood pressure (BP) distribution and control differ between age groups and whether sex and age interact and potentially impact BP after transplantation have not been investigated.

Methods: This retrospective analysis included 336 pediatric renal transplant recipients (62% males) from the Cooperative European Pediatric Renal Transplant Initiative Registry (CERTAIN) with complete BP measurement at discharge and 1, 2 and 3 years post-transplant.

Results: At discharge and 3 years post-transplant, arterial hypertension was highly prevalent (84% and 77%); antihypertensive drugs were used in 73% and 68% of the patients. 27% suffered from uncontrolled and 9% from untreated hypertension at 3 years post-transplant. Children transplanted at age < 5 years showed sustained high systolic BP z-score and received consistently less antihypertensive treatment over time. Younger age, shorter time since transplantation, male sex, higher body mass index (BMI), high cyclosporine A (CSA) trough levels, and a primary renal disease other than congenital anomalies of the kidney and urinary tract (CAKUT) were significantly associated with higher systolic BP z-score. Sex-stratified analysis revealed a significant association between high CSA and higher systolic BP in older girls that likely had started puberty already. An association between BP and estimated glomerular filtration rate was not detected.

Conclusions: BP control during the first 3 years was poor in this large European cohort. The description of age- and sex-specific risk profiles identified certain recipient groups that may benefit from more frequent BP monitoring (i.e. young children) or different choices of immunosuppression (i.e. older girls).
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http://dx.doi.org/10.1007/s00467-019-04395-4DOI Listing
March 2020

Colostomy in children on chronic peritoneal dialysis.

Pediatr Nephrol 2020 01 30;35(1):119-126. Epub 2019 Oct 30.

Children's Mercy Kansas City, Kansas City, MO, USA.

Background: This study aimed to evaluate outcome of children on chronic peritoneal dialysis (PD) with a concurrent colostomy.

Methods: Patients were identified through the International Pediatric Peritoneal Dialysis Network (IPPN) registry. Matched controls were randomly selected from the registry. Data were collected through the IPPN database and a survey disseminated to all participating sites.

Results: Fifteen centers reported 20 children who received chronic PD with a co-existing colostomy. The most common cause of end stage kidney disease was congenital anomalies of the kidney and urinary tract (n = 16, 80%). The main reason for colostomy placement was anorectal malformation (n = 13, 65%). The median age at colostomy creation and PD catheter (PDC) insertion were 0.1 (IQR, 0-2.2) and 2.8 (IQR 0.2-18.8) months, respectively. The colostomies and PDCs were present together for a median 18 (IQR, 4.9-35.8) months. The median age at PDC placement in 46 controls was 3.4 (IQR, 0.2-7.4) months of age. Fourteen patients (70%) developed 39 episodes of peritonitis. The annualized peritonitis rate was significantly higher in the colostomy group (1.13 vs. 0.70 episodes per patient year; p = 0.02). Predominant causative microorganisms were Staphylococcus aureus (15%) and Pseudomonas aeruginosa (13%). There were 12 exit site infection (ESI) episodes reported exclusively in colostomy patients. Seven colostomy children (35%) died during their course of PD, in two cases due to peritonitis.

Conclusion: Although feasible in children with a colostomy, chronic PD is associated with an increased risk of peritonitis and mortality. Continued efforts to reduce infection risk for this complex patient population are essential.
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http://dx.doi.org/10.1007/s00467-019-04372-xDOI Listing
January 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

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

Time-averaged hemoglobin values, not hemoglobin cycling, have an impact on outcomes in pediatric dialysis patients.

Pediatr Nephrol 2018 11 13;33(11):2143-2150. Epub 2018 Aug 13.

Department of Pediatric Nephrology, Cerrahpasa School of Medicine, Istanbul University, Istanbul, Turkey.

Background: During erythropoietin-stimulating agent (ESA) treatment, hemoglobin (Hb) levels usually fluctuate; this phenomenon is known as "Hb cycling (HC)." In this study, we aimed to evaluate the predictors of HC and its impact on left ventricular hypertrophy (LVH) as a patient-important outcome parameter in pediatric dialysis patients.

Methods: Records of patients followed up in nine pediatric nephrology centers between 2008 and 2013 were reviewed. More than 1 g/dL decrease or increase in Hb level was considered as HC. Patients were divided into two groups according to 12-month Hb trajectory as rare cycling (RC) (≤ 3) and frequent cycling (FC) (> 3 fluctuation) as well as three groups based on T-A-Hb levels: < 10, 10-11, and > 11 g/dL.

Results: Two hundred forty-five dialysis (160 peritoneal dialysis (PD) and 85 hemodialysis (HD)) patients aged 12.3 ± 5.1 (range 0.5-21) years were enrolled in this study. Fifty-two percent of the patients had RC, 45% had FC, and only 3% had no cycling. There were no differences between HC groups with respect to age, dialysis modality, having anemia, hospitalization rate, residual urine volume, and mortality. Although left ventricular mass index (LVMI) tended to be higher in RC than FC group (65 ± 37 vs 52 ± 23 g/m, p = 0.056), prevalence of LVH was not different between the groups (p = 0.920). In regression analysis, FC was not a risk factor for LVH, but low T-A Hb level (< 10 g/dL) was a significant risk for LVH (OR = 0.414, 95% CI 0.177-0.966, p = 0.04). The target Hb levels were more often achieved in PD patients, and the number of deaths was significantly lower in non-anemic patients (Hb level > 11 g/dL).

Conclusion: Hb cycling is common among dialysis patients. Severity of anemia rather than its cycling has more significant impact on the prevalence of LVH and on inflammatory state.
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http://dx.doi.org/10.1007/s00467-018-4013-4DOI Listing
November 2018

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

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

The association between monosymptomatic enuresis and allergic diseases in children.

Turk J Pediatr 2018 ;60(4):415-420

Departments of Allergy and Immunology, Dokuz Eylul University Faculty of Medicine, İzmir, Turkey.

Yılmaz-Durmuş S, Alaygut D, Soylu A, Alparslan C, Köse SŞ, Anal Ö. The association between monosymptomatic enuresis and allergic diseases in children. Turk J Pediatr 2018; 60: 415-420. This clinical study was designed to evaluate correlation between monosymptomatic enuresis (MSE) and allergic diseases (asthma, allergic rhinitis, eczema, and food allergy) in pediatric patients. The study was conducted on 50 pediatric patients with a MSE clinic who were ≥7 years old and applied to two tertiary health institutions between November 2015 and June 2016. Fifty healthy children of similar age, who applied to pediatric outpatient clinics for various reasons, were included as the control group. A questionnaire questioning the presence of food allergy and enuresis in the family and also including the questions of International Study of Asthma and Allergies in Childhood (ISAAC) was distributed to the parents of the children included in the study. It was found that 52% of 100 children participating in the study were boys and 48% were girls and their mean age was 10.8±2.8 years. While allergic diseases accompanied 34% of the cases with enuresis, this rate was found as 12% in the control group (p < 0.01). It was determined that the family history in terms of enuresis and atopy was at a higher rate in the study group (40% and 26%, respectively) and at a lower rate in the control group (2% and 6%, respectively) (p < 0.01). It was observed that allergic diseases were more frequent in the cases with MSE at a statistically significant level compared to the group without enuresis.
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http://dx.doi.org/10.24953/turkjped.2018.04.009DOI Listing
May 2019

Predictive factors for the outcome of high flow nasal cannula therapy in a pediatric intensive care unit: Is the SpO/FiO ratio useful?

J Crit Care 2018 04 6;44:436-444. Epub 2017 Sep 6.

Tepecik Teaching and Research Hospital, Pediatric Emergency Department, Izmir, Turkey.

Objectives: To determine the predictive factors for the outcome of high-flow nasal cannula (HFNC) therapy in a pediatric intensive care unit (PICU).

Materials And Methods: We prospectively included all patients with acute respiratory distress/failure aged 1month to 18years who were admitted to the PICU between January 2015 and May 2016 and treated with HFNC as a primary support and for postextubation according to our pre-established protocol. HFNC failure was defined as the need for escalation to non-invasive ventilation (NIV) or invasive mechanical ventilation (MV). HFNC responders and nonresponders were compared based on clinical data obtained just before HFNC and at 30, 60, and 120min, 12, 24, and 48h, and at the end of therapy.

Results: A total of 204 patients (median age: 16.5months) participated in the study. Twenty-six (12.7%) patients required escalation (4 to NIV and 22 to MV). Age >120months, higher PRISM-III and respiratory scores, and a lower SpO/FiO (S/F) ratio at admission were predictors of HFNC failure. Achievement of the S/F>200 goal at 60min significantly predicted successful HFNC.

Conclusion: Monitoring the S/F ratio might be useful and practical to avoid delaying escalation to another ventilation support. Failure to achieve S/F>200 at 60min should be a warning for the escalation of respiratory support.
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http://dx.doi.org/10.1016/j.jcrc.2017.09.003DOI Listing
April 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

Effect of the timing of dialysis initiation on left ventricular hypertrophy and ınflammation in pediatric patients.

Pediatr Nephrol 2017 09 10;32(9):1595-1602. Epub 2017 Apr 10.

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

Background: The optimal time for dialysis initiation in adults and children with chronic kidney disease remains unclear. The aim of this study was to evaluate the impact of dialysis timing on different outcome parameters, in particular left ventricular (LV) morphology and inflammation, in pediatric patients receiving peritoneal dialysis and hemodialysis.

Methods: The medical records of pediatric dialysis patients who were followed-up in nine pediatric nephrology centers in Turkey between 2008 and 2013 were retrospectively reviewed. In addition to demographic data, we retrieved anthropometric measurements, data on dialysis treatment modalities, routine biochemical parameters, complete blood count, serum ferritin, parathormone, C-reactive protein (CRP), and albumin levels, as well as echocardiographic data and hospitalization records. The patients were divided into two groups based on their estimated glomerular filtration rate (eGFR) levels at dialysis initiation, namely, an early-start group, characterized by an eGFR of >10 ml/min/1.73 m, and a late-start group, with an eGFR of < 7 ml/min/1.73 m. The collected data were compared between these groups.

Results: A total of 245 pediatric dialysis patients (mean age ± standard deviation 12.3 ± 5.1 years, range 0.5-21 years) were enrolled in this study. Echocardiographic data were available for 137 patients, and the mean LV mass index (LVMI) was 58 ± 31 (range 21-215) g/m. The LVMI was 75 ± 30 g/m(n = 81) and 34 ± 6 g/m(n = 56) in patients with or without LV hypertrophy (LVH) (p < 0.001). Early-start (eGFR >10 ml/min/1.73 m) versus late-start dialysis (eGFR < 7 ml/min/1.73 m) groups did not significantly differ in LVMI and LVH status (p > 0.05) nor in number of hospitalizations. Serum albumin levels were significantly higher in the early-dialysis group compared with the late-dialysis group (3.3 ± 0.7 vs. 3.1 ± 0.7 g/dl, respectively; p < 0.05). The early-start group had relatively higher time-averaged albumin levels (3.2 ± 0.5 vs. 3.1 ± 0.5 g/dl; p = > 0.05) and relatively lower CRP levels (3.64 ± 2.00 vs. 4.37 ± 3.28 mg/L, p > 0.05) than the late-start group, but these differences did not reach statistical significance.

Conclusion: Although early dialysis initiation did not have a significant effect on important clinical outcome parameters, including LVH, inflammatory state, and hospitalization, in our pediatric dialysis patients, this area of study deserves further attention.
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http://dx.doi.org/10.1007/s00467-017-3660-1DOI Listing
September 2017

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

Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literature.

Turk J Pediatr 2017 ;59(4):463-466

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

Alparslan C, Kamit-Can F, Anıl AB, Olgaç-Dündar N, Çavuşoğlu D, Göç Z. Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literature. Turk J Pediatr 2017; 59: 463-466. Febrile infection-related epilepsy syndrome (FIRES) is a catastrophic epilepsy syndrome which is characterized by acute onset of refractory status epilepticus following a febrile infection occurring in previously normal children. Despite the various treatment options that have been tried, exact treatment strategy is still undetermined. This is the first pediatric case of FIRES from Turkey which was successfully treated with intravenous immunoglobulin (IVIG). A previously healthy 8-year-old boy was referred to our hospital with a pre-diagnosis of status epilepticus and encephalitis. He presented with acute onset of convulsions and unconsciousness following fever and malaise lasting 7 days. On physical examination Glasgow coma scale was 12, his pupils were miotic. He had cafe-au-lait spots on his body. His fundus examination, cerebrospinal fluid findings and cranial magnetic resonance imaging did not reveal any abnormality. Results of comprehensive search for metabolic, toxicological, infectious and autoimmune etiologies were all negative. Generalized slowing was seen on the electroencephalography (EEG) of the patient indicating possible encephalopathy. The patient developed convulsive status epilepticus and was intubated on day 5. His seizures were controlled by continuous infusion of midazolam, thiopental and used for 4 days. Phenytoin, levetiracetam, topiramate were used simultaneously. IVIG was administered as an immunomodulator for refractory seizures on day-9. The patient was extubated on day 11. The diagnosis was made after a comprehensive negative search for central nervous system infection, autoimmune and metabolic diseases. At follow up it was learnt that he had had only two seizures in two years. Status epilepticus did not recur. Clinicians should keep in mind FIRES which is a diagnosis of exclusion especially in refractory status epilepticus. IVIG treatment could have a benefit in these patients.
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http://dx.doi.org/10.24953/turkjped.2017.04.014DOI Listing
December 2018

Delayed Graft Function in Kidney Transplantation: Risk Factors and Impact on Early Graft Function.

Prog Transplant 2016 Jun 6;26(2):172-7. Epub 2016 Apr 6.

Tepecik Training and Research Hospital, Department of Transplantation and General Surgery, Izmir, Turkey.

Context: Although kidney transplantations are routinely performed at many centers in Turkey, the incidence and risk factors associated with delayed graft function (DGF) here have not yet been well defined.

Objective: The aim of this study is to evaluate the incidence and risk factors of DGF and its impact on early graft function.

Design: The medical charts of 154 adult patients who underwent deceased donor kidney transplantation between 2000 and 2014 in a single center were reviewed retrospectively.

Setting: Delayed graft function-related risk factors for donors, recipients, and the transplant surgery itself were analyzed, and their relation with graft function was evaluated.

Main Outcomes Measures: The median recipient age was 39 years. The median cold ischemia time (CIT) was 840 minutes (14 hours). The incidence of DGF and acute rejection were 57.8% and 8.4%, respectively. Higher serum creatinine levels at 3, 6, and 12 months were observed in patients with DGF compared to other patients without DGF (P < .05). Patients with DGF had poor graft function (glomerular filtration rate ≤ 50) at 3 and 6 months (P < .05), but these correlations were not seen at 12 months (P = not significant).

Results: This study showed that DGF was a common and serious problem associated with poor graft functions at 3, 6, and 12 months after transplantation. Extra effort to shorten CIT as an independent risk factor for DGF could have protective effect on graft functions.
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http://dx.doi.org/10.1177/1526924816640978DOI Listing
June 2016

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

The need for mechanical ventilation in a child exposed to a laundry detergent pod.

Turk J Pediatr 2016 ;58(3):323-326

Department of Pediatric Intensive Care Unit, Katip Çelebi Univercity, Faculty of Medicine, İzmir, Turkey.

Laundry detergent pods (LDPs) are a new, concentrated form of detergent covered by a membrane of polyvinyl alcohol or other water-soluble material. In contrast to traditional laundry detergents, the spectrum of responses to exposure to LDPs ranges from mild to life-threatening events. This is a case report of a 3-year-old male who ingested part of an LDP, leading to a depressed level of consciousness, upper airway obstruction, and severe respiratory distress. The patient required intubation and mechanical ventilation for 2 days before being discharged. This rare, severe clinical pattern demonstrates the potential toxicity of these laundry detergents. In the literature, few cases that required intubation and ventilation have been reported. To our knowledge, this is also the first case of LDP exposure reported from Turkey.
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http://dx.doi.org/10.24953/turkjped.2016.03.017DOI Listing
June 2017

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