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Background This study aimed to determine the dietary acid load of children with chronic kidney disease (CKD) and to evaluate the relationship between dietary acid load, nutritional status, and health-related quality of life (HRQOL). Method A total of 67 children aged 3-18 years with a diagnosis of CKD stages II-V were included in the study. Anthropometric measurements (body weight, height, mid-upper arm circumference, waist, and neck circumference) and 3-day food consumption records were taken to evaluate the nutritional status. The net endogenous acid production (NEAP) score was calculated to determine the dietary acid load. 'Pediatric Inventory of Quality of Life (PedsQL)' was used to assess the participants' HRQOL. Results The mean NEAP was 59.2 ± 18.96 mEq/day. Stunted and malnourished children had significantly higher NEAP than those who were not (p < 0.05). There were no significant differences in terms of HRQOL scores according to NEAP groups. The multivariate logistic regression analysis showed that waist circumference (OR: 0.890, 95% CI: 0.794-0.997), serum albumin (OR: 0.252, 95% CI: 0.068-0.929), and glomerular filtration rate (GFR) (OR: 0.985, 95% CI: 0.970-1.000) were negatively associated with high NEAP. Conclusion This study shows that a diet shifted in an acidic direction in children with CKD and a higher dietary acid load are associated with lower serum albumin, GFR, and waist circumference, but not HRQOL. These results suggest that dietary acid load might affect nutritional status and CKD progression in children with CKD. Future studies with larger samples are needed to confirm these results and to understand underlying mechanisms. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information, Author(s): Gamze Yurtdas Depboylu [sup.1] , Gülsah Kaner [sup.1] , Özgür Özdemir Simsek [sup.2] , Kübra Nur Turan [sup.1] , Belde Kasap Demir [sup.2] [sup.3] Author Affiliations: (1) grid.411795.f, 0000 [...]

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] [...]

Author(s): Gökçen Erfidan [sup.1] , Demet Alaygut [sup.2] , Özgür Özdemir Simsek [sup.1] , Seçil Arslansoyu Çamlar [sup.2] , Fatma Mutlubas [sup.2] , Belde Kasap Demir [sup.1] [sup.3] Author Affiliations: [...]

Author(s): Özgür Özdemir-Simsek [sup.1] , Gökçen Erfidan [sup.1] , Seçil Arslansoyu-Çamlar [sup.2] , Demet Alaygut [sup.2] , Fatma Mutlubas [sup.2] , Belde Kasap-Demir [sup.1] [sup.3] Author Affiliations: (1) grid.414882.3, 0000 [...]

Author(s): Demet Alaygut [sup.1] , Özgür Özdemir-Simsek [sup.1] , Fatma Ceren Sarioglu [sup.2] , Seçil Arslansoyu-Çamlar [sup.1] , Fatma Mutlubas [sup.1] , Belde Kasap-Demir [sup.3] Author Affiliations: (1) grid.414882.3, 0000 [...]

Author(s): Özgür Özdemir-Simsek [sup.1] , Belde Kasap-Demir [sup.1] [sup.2] , Gökçen Erfidan [sup.1] , Seçil Arslansoyu-Çamlar [sup.1] , Eren Soyaltin [sup.1] , Demet Alaygut [sup.1] , Fatma Mutlubas [sup.1] Author [...]

Objective: We aimed to evaluate the clinical significance of the “unclassified” blood pressure phenotypes on left ventricular hypertrophy in children. Materials and Methods: All children evaluated with ambulatory blood pressure monitoring in the pediatric nephrology department between October 2018 and January 2021 were included in the study. Prehypertension, normotensive, white coat hypertension, masked hypertension, ambulatory hypertension groups and 2 other groups including increased blood pressure load, normal ambulatory blood pressure measurements, but normal (unclassified group 1) or high (unclassified group 2) office blood pressure measurements were defined according to the American Heart Association 2014 statement. Left ventricular mass index, left ventricular mass index/95 percentile values, and left ventricular hypertrophy ratios were compared between the groups separately to establish the influence of the unclassified cases. Results: A total of 497 children were included. There were 52 cases in normotensive, 47 cases in unclassified group 1, 50 cases in masked hypertension, 79 cases in white coat hypertension, 104 cases in unclassified group 2, and 165 cases in the ambulatory hypertension group. Left ventricular mass index/95 percentile and left ventricular hypertrophy in masked hypertension were significantly higher than normotensive but similar between normotensive and unclassified group 1 groups. Left ventricular hypertrophy was significantly higher in the ambulatory hypertension group compared to white coat hypertension, and similar between white coat hypertension and unclassified group 2 groups. Conclusion: Independent of age, we have found that interpretation of blood pressure load not only has a limited predictable effect on left ventricular hypertrophy but also causes a large group of patients to be unclassified. [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 The oculocerebrorenal syndrome of Lowe (OCRL) is a rare X-linked multi-systemic disorder, almost always characterized by the triad of congenital cataract, cognitive and behavioral impairment and a proximal tubulopathy. Methods Twenty-eight novel patients with suspected Lowe syndrome were studied. Results All patients carried OCRL gene defects with mutational hot spots at CpG dinucleotides. Mutations previously unknown in Lowe syndrome were observed in ten of the 28 patients, and carriership was identified in 30.4 % of the mothers investigated. Mapping the exact breakpoints of a complete OCRL gene deletion revealed involvement of several flanking repeat elements. We noted a similar pattern of documented clinically relevant symptoms, and even though the patient cohort comprised relatively young patients, 32 % of these patients already showed advanced chronic kidney disease. Thrombocytopenia was seen in several patients, and hyperosmia and/or hyperacusis were reported recurrently. A p.Asp523Asn mutation in a Polish patient, associated with the typical cerebrorenal spectrum but with late cataract (10 year), was also evident in two milder affected Italian brothers with ocular involvement of similar progression. Conclusions We have identified clinical features in 28 patients with suspected Lowe syndrome that had not been recognized in Lowe syndrome prior to our study. We also provide further evidence that OCRL mutations cause a phenotypic continuum with selective and/or time-dependent organ involvement. At least some of these mutants might exhibit a genotype-phenotype correlation., Author(s): Florian Recker[sup.1] , Marcin Zaniew[sup.2] , Detlef Bockenhauer[sup.3] , Nunzia Miglietti[sup.4] , Arend Bokenkamp[sup.5] , Anna Moczulska[sup.6] , Anna Rogowska-Kalisz[sup.7] , Guido Laube[sup.8] , Valerie Said-Conti[sup.9] , Belde Kasap-Demir[sup.10] [...]

Objective: To evaluate the effects of non-E. coli or extended-spectrum β-lactamase–positive (ESBL-positive) microorganism growth in the first febrile urinary tract infection (UTI) of infants on laboratory findings or renal parenchymal damage presenting the severity of inflammation, anatomic abnormalities defined by imaging studies, and recurrent UTIs in the follow-up period. Methods: The data of patients aged between 2 and 24 mo and followed up for at least 6 mo with febrile UTI guideline of the authors' pediatric-nephrology clinic, were retrospectively analyzed. Ultrasonography was performed in all the cases at the time of UTI and dimercaptosuccinic-acid (DMSA) at least 4 mo after the infection. Voiding cystourethrography (VCUG) was performed only if ultrasonography findings were abnormal, the uptake deformity was detected in DMSA scan, or the patients experienced recurrent UTIs. The patients were grouped concerning E. coli or non-E. coli and ESBL-PB or non-ESBL-BP growth in the urine cultures. Results: There were 277 infants followed up for 28.55 ± 15.24 (6–86) mo. The causative microorganisms were non-E. coli in 73 (26.4%) and ESBL-PB in 58 (20.9%) cases. CRP values, pyuria, and leukocyte-esterase positivity were significantly higher in UTIs caused by E. coli compared to non-E. coli bacteria. All clinical and laboratory findings were similar between the ESBL-PB and non-ESBL groups, but abnormal ultrasonography findings were more common in non-E. coli group. Conclusion: E. coli causes more severe inflammation, but non-E. coli infections are more frequently associated with ultrasound abnormalities. However, ESBL production did not affect either laboratory or radiological findings in the present cohort. [ABSTRACT FROM AUTHOR]