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Background Ventricular repolarization (VR) increases the risk of sudden cardiac death due to ventricular arrhythmia. We aimed to evaluate the blood pressure (BP) parameters affecting VR in obese children. Methods Obese (BMI [greater than or equal to] 95p) and healthy children [greater than or equal to] 120 cm between January 2017 and June 2019 were included. Demographic and laboratory data, peripheral and central BPs evaluated by a device capable of ambulatory blood pressure monitoring (ABPM), and pulse wave analysis were assessed. Electrocardiographic ventricular repolarization indices, left ventricular mass index (LVMI), and relative wall thickness (RWT) were calculated. Results A total of 52 obese and 41 control patients were included. Uric acid, triglyceride, total cholesterol, LDL, and ALT values, systolic and diastolic office BPs, 24-h, daytime and nighttime systolic and mean arterial BPs, daytime diastolic BP SDS levels, daytime and nighttime systolic loads, daytime diastolic load, 24-h, daytime and nighttime central systolic and diastolic BPs, and pulse wave velocity values were significantly higher, whereas 24-h, daytime and nighttime AIx@75 were similar between the groups. fT4 levels of obese cases were significantly lower. QTcd and Tp-ed were higher in obese patients. Although RWT was higher in obese cases, LVMI values and cardiac geometry classifications were similar. The independent factors affecting VR in obese cases were younger age and higher diastolic load at night (B = - 2.83, p = 0.010; B = 0.257, p = 0.007, respectively). Conclusion Obese patients have higher peripheral and central BP, arterial stiffness, and higher VR indices that develop before an increase in LVMI. It would be useful to prevent obesity from an early age and follow up nighttime diastolic load to control VR associated sudden cardiac death in obese children. Graphical abstract, Author(s): Belde Kasap Demir [sup.1] [sup.2] , Eren Soyaltin [sup.1] [sup.2] , Caner Alparslan [sup.3] , Tülay Demircan [sup.4] , Seçil Arslansoyu Çamlar [sup.2] [sup.5] , Demet Alaygut [sup.2] [sup.5] [...]

Copyright of Ege Journal of Medicine is the property of Ege University, Faculty of Medicine and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Journal of Behcet Uz Children's Hospital is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Author(s): Eren Soyaltin [sup.1] , Belde Kasap-Demir [sup.1] [sup.2] , Caner Alparslan [sup.1] , Seçil Arslansoyu-Çamlar [sup.1] , Elif Perihan Öncel [sup.3] , Özgür Kirbiyik [sup.4] , Demet Alaygut [sup.1] [...]

Objective: Diabetic nephropathy is one of the most important complications in diabetes mellitus. We aimed to examine the influence of organ-specific antibody seropositivity in diabetic nephropathy. Methods: Patients with type 1 diabetes and no evidence of celiac disease, thyroid dysfunction, and other kidney diseases and with an annual average HbA1c level <10%, body mass index <25 kg/m2, and normal blood pressure were enrolled. Results: Eighty patients (39 boys and 41 girls) were evaluated. Twenty patients with moderately increased albuminuria (diabetic nephropathy group) and 60 patients without albuminuria (control group) showed no statistical difference in age, gender, diabetes duration, age at diagnosis, kidney function tests, and mean blood pressure measurements. Compared to control group, the mean anti-thyroglobulin level and anti-thyroxin peroxidase level were statistically higher in the diabetic nephropathy group, P = .004 and P = .045, respectively. However, the thyroid function tests were normal in either group. Conclusion: Determination of the impact of autoantibody seropositivity on the risk of diabetic nephropathy in type 1 diabetic children could be a non-traditional marker in the risk assessment of diabetic nephropathy [ABSTRACT FROM AUTHOR]

Aim Urinary tract infections (UTIs) represent a common febrile illness in infancy. The study compared two UTI guidelines in terms of number of imaging studies, presence of parenchymal damage and radiation exposure in patients with the first febrile UTI between 2 and 24 months of age. Method The results of Tepecik UTI Guideline-1 used until 2012 (Group 1, n  = 105) were retrospectively compared with Tepecik UTI Guideline-2 (Group 2) used after 2013. In Group 1, urinary tract ultrasonography (US), dimercaptosuccinic acid (DMSA) and voiding cystourethrography (VCUG) were made in all patients. In Group 2, if the US result was abnormal, patients were evaluated with VCUG and DMSA. If the US was normal, only DMSA was performed. If the DMSA was abnormal, the VCUG was undergone (n : 43, 40.9%). Results The abnormal VCUG detection rate was 69.2% in Group 1 and 30.8% in Group 2 (p  = 0.09). Sensitivity and specificity of US in the diagnosis of vesicoureteral reflux (VUR) was 15.9% and 96.7% in Group 1 and 61.5% and 70.5% in Group 2, respectively. Abnormal DMSA findings were observed among 33.3% (Groups 1) and 66.7% (Groups 2) subjects, respectively (p  > 0.05). The median radiation exposure (500 mrem) of patients in Group 1 was statistically significantly higher than those in Group 2 (200 mrem) (p  < 0.001). Conclusion The VCUG should not be the first examination to be considered in such patients. We think that Tepecik UTI Guideline-2 reduces unnecessary invasive procedure and radiation exposure and not missed VUR in the management of children with UTI at 2–24 months. Needs prospective follow-up studies before considering this recommendation. [ABSTRACT FROM AUTHOR]

Author(s): Eren Soyaltin [sup.1] , Belde Kasap-Demir [sup.1] [sup.2] , Caner Alparslan [sup.1] , Seçil Arslansoyu-Çamlar [sup.1] , Elif Perihan Öncel [sup.3] , Özgür Kirbiyik [sup.4] , Demet Alaygut [sup.1] [...]

Background: Patients with nephrotic syndrome (NS) are at a high risk of cardiovascular disease, obesity, and dyslipidemia. The aim of this study was to evaluate the formation of epicardial adipose tissue (EAT) and investigate electrocardiographic (ECG) parameters in patients. Methods: Thirty-two patients aged 0–18 years and 15 control patients were compared. In the patient group, physical examination and laboratory parameters were recorded. Atrial depolarization and ventricular repolarization parameters in ECG were compared between the groups. EAT was evaluated with M-mode measurements on echocardiography. Results: There was no difference between the groups in terms of sex, age, body mass index, systolic and diastolic BP. EAT was found to be significantly higher in the patient group. In ECG evaluations it was determined that atrial depolarization and ventricular repolarization parameters increased in the patient group. Conclusions: Cardiovascular morbidity and mortality are high in kidney diseases. Measurement and follow-up of EAT and ECG findings as a noninvasive parameter can provide information in NS. [ABSTRACT FROM AUTHOR]

Aim: Kidney transplantation is the most preferred long-term treatment of children with end stage renal disease since 1954. Graft dysfunction has been divided into three categories based on timing of presentation. In this article, we aim to present the etiologic factors of acute graft dysfunction in the first year of renal transplantation in children. Materials and Methods: The patients, diagnosed with acute allograft dysfunction in first year of kidney transplantation, in University of Health Sciences Turkey, Tepecik Training and Research Hospital between March 2005 and October 2017 were analyzed prospectively in this study. Results: Over the 15 year period, 56 pediatric renal allograft patients were followed in University of Health Sciences Turkey, Tepecik Training and Research Hospital. During this period, 25 patients had diagnosed with early allograft dysfunction. Five patients were admitted two times in a year with allograft dysfunction. The mean age of patients was 12.6 (4.0±21.0) years, with a male and female ratio of 17:8. Delayed graft function was occurred in four patients (16%). Among 25 renal allograft recipients showed early graft dysfunction on average of 4.2 months (1-10 months) after transplantation. The etiology of early graft dysfunction showed 10% immunologic diseases and 90% non-immunologic factors. Five patients (16.7%) diagnosed with urinary tract infection, one patient (3.3%) diagnosed with cytomegalovirus nephropathy, five patients (16.7%) diagnosed with BK nephropathy, eight patients (26.7%) diagnosed with acute cyclosporine toxicity, seven patients (23.3%) were diagnosed with dehydration and one patient (3.3%) diagnosed with urologic anomalies. Twelve patients had performed renal allograft biopsy. The histological findings were consistent with T-cell mediated rejection in two patients; B-cell mediated rejection in one patient and viral nephropathy in five patients. Non-specific histological findings were determined in five patients. Conclusion: Early recognition of the etiology of graft dysfunction that develops at 1 year and appropriate treatment will contribute to the preservation of long-term graft dysfunction. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Tepecik Education & Research Hospital / İzmir Tepecik Eğitim ve Araştırma Hastanesi Dergisi is the property of Logos Medical Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Journal of Tepecik Education & Research Hospital / İzmir Tepecik Eğitim ve Araştırma Hastanesi Dergisi is the property of Logos Medical Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background/aim: The objective of this study is to determine the clinical features of unilateral multicystic dysplastic kidney (MCDK) patients. Materials and methods: The demographic, clinical, laboratory, and radiologic features of MCDK patients at Diyarbakır Children's Hospital and Diyarbakır Gazi Yaşargil Training and Research Hospital 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 was located on the left and right sides in 46 (41.4%) and 65 (58.6%) of the 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 was detected in 11 patients (22.4%). Other associated urological anomalies in the patients 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 UTI (7.2%). Renal failure, hypertension, and proteinuria were diagnosed in three children (2.7%). Sixty-nine (62%) 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. [ABSTRACT FROM AUTHOR]

Background/aim: 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. Materials and 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 sex, laterality of the cysts, maximum cyst diameter, cyst or kidney enlargement, follow-up duration, or GFR at last visit between Groups 1 and 2. Conclusion: The majority of children with ADPKD had preserved renal functions and slight cyst enlargement during their follow-up. However, they may have different renal problems deserving closed follow-up. [ABSTRACT FROM AUTHOR]

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 (HTABPM) 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Tepecik Education & Research Hospital / İzmir Tepecik Eğitim ve Araştırma Hastanesi Dergisi is the property of Logos Medical Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]