Prospective evaluation of vascular changes in acute respiratory infections in children with cystic fibrosis

Background/aim: Acute exacerbations and chronic inflammation are risk factors for cardiovascular disease (CVD) in cystic fibrosis (CF) patients. The aim of this study was to investigate the effects of acute exacerbation therapy on arterial stiffness in children with CF. Materials and methods: Augmentation index (Aix) and pulse wave velocity (PWV) were measured before and after treatment and 1 month after the end of treatment in patients with acute exacerbation. The relationship between hemodynamic measurements and c-reactive protein (CRP) and pulmonary function tests (PFTs) was investigated. Results: Measurements before and after treatment were evaluated in 27 patients and were repeated in 21 patients who were clinically stable 1 month following acute exacerbation. There was a significant decrease in CRP and an increase in spirometry parameters after treatment. While no significant difference was found between PWV (P = 0.33), a significant difference for Aix before (41.95 ± 12.96%) and after (30.95 ± 11.47%) treatment and before treatment and stable clinical condition (34.19 ± 14.36%) was obtained (P =0.00, and P =0.01, respectively). No significant difference in heart rate and other hemodynamic measurements was found. Pretreatment Aix is associated with poor clinical condition (PFTs, BMI, and clinical score) and systemic inflammation (CRP) (P <0.05). Conclusion: The decrease of arterial stiffness (Aix) with acute exacerbation treatment in children with CF has been demonstrated. This result shows that systemic inflammation in CF may cause an increase in arterial stiffness and recurrent exacerbations may increase the risk of CVD.

Growth evaluation in children with vesicoureteral reflux.

BACKGROUND: Vesicoureteral reflux and urinary tract infection predispose children to retardation of growth, hypertension, renal scarring and renal failure. The aim of this study was to evaluate growth pattern in children with vesicoureteral reflux before and after medical/surgical treatment. METHODS: This study was a retrospective cross-sectional study. The study population included 97 children aged 0.5 to 17 years (8.8±5.5). Body weight, height and bone age of the children were measured. Weight Z score and height Z score were calculated during first visits and after medical and/or surgical treatment. Distribution, mean and standard deviation score were evaluated for the descriptive data. T-test and Mann-Whitney U test and Wilcoxon test were used for statistical analysis. RESULTS: Sixty-five percent of 97 children enrolled in this study were girls. About 48.5% of the children had unilateral and mild reflux, while 16.5% had bilateral and severe reflux. The bone age was 8.6 years. Differentiation with chronological age and bone age were not significant (P=0.294). At admission, 54.6% and 50.5% of children had negative Weight Z score and height Z score, respectively. After medical and surgical treatment, Weight Z score and height Z score were increased, however, only the increase in Weight Z score was significant (P=0.039, P=0.031, respectively). A significant reduction in bone age was found in children with renal scars compared to those without renal scars (P=0.048). CONCLUSIONS: High-grade vesicoureteral reflux had a negative impact on indices of growth in children. Medical and/or surgical treatment had positive effect on weight gain.

The effects of mesenchymal stem cells on lymphoblastic leukemia cell proliferation.

PURPOSE: Mesenchymal stem cells (MSCs) represent a new approach to the treatment of several neoplastic or non-neoplastic disorders. Their potential to repair damaged tissues through trans differentiation in conjunction with their immunomodulatory ability made them promising candidates for cell-based immunotherapy and regenerative medicine. In the present study, we aimed to determine the effects of MSCs on proliferation, apoptosis and gene expression profile of the acute lymphoblastic leukemia (ALL) cell line CCRF-CEM. METHODS: The experiments were performed after MSCs and CCRF-CEM cells were co-cultured for 72 hrs. We analyzed the gene expression patterns to predict oncogenic pathway dysregulation in the cell groups by quantitative RT-PCR and immunohistochemical staining. RESULTS: Cell proliferation was significantly inhibited in co-cultured CCRF-CEM cells compared to the control. Furthermore, growth factors, p53, Bax and Caspase-9 expressions were increased and cell-signaling gene expressions decreased significantly. Despite increased levels of growth factors (CTGF, VEGF, FGF, EGFR), the increased apoptosis level was triggered by p53/ Bax. CONCLUSION: In this study we have shown that human MSCs have inhibitory effect on their neighboring malignant leukemia cells.

Effects of Body Mass Index Changes In Pediatric Kidney Transplant Patients.

BACKGROUND: The negative effects of pretransplant obesity and post-transplant body mass index (BMI) increase on graft survival have been reported in recent years. The aim of this study is to evaluate the effects of BMI changes on post-transplant graft function, lipid profile, and blood pressure. METHODS: The study included 133 pediatric patients transplanted between 1994 and 2019 in Ege University. BMI Z-scores (BMIZs) were calculated according to age and sex before and after transplantation using the World Health Organization criteria. Patients with BMIZs >+1 standard deviation (SD) were defined as overweight, and those with BMIZs >+2 SD were defined as obese: Group 1: Obese or overweight before transplantation; Group 2: Thin or normal weight before and 2 years after transplantation; and Group 3: Thin or normal weight before transplantation and obese or overweight 2 years after transplantation. RESULTS: At the time of transplantation 8% of the patients were overweight, and 1% were obese. Overweight and obesity statistically significantly increased (31.6%) 2 years after renal transplantation (P = .001). Obese and overweight patients have lower high-density lipoprotein levels and were younger at the time of transplantation. Graft functions, lipid levels, and blood glucose levels of the groups were similar (P > .05). The only significant difference between the groups was that Group 1 patients were younger than Group 2. CONCLUSIONS: Obesity develops at a significant rate in pediatric patients after renal transplantation. In this study, we could not demonstrate negative effects of obesity and being overweight in terms of post-transplant graft function, lipid profile, blood glucose, and blood pressure.

Quality of Life and Psychological Well-Being in Children and Adolescents After Renal Transplantation.

BACKGROUND: Kidney transplantation is the predominant treatment option in patients with end-stage renal disease. The aim of this study was to evaluate the effect of the presence of a psychiatric disorder on the quality of life in children and adolescents after kidney transplantation. METHODS: A total of 43 patients aged 6 to 18 years were included in the study. All participants and their parents were asked to complete the Pediatric Quality of Life Inventory (PedsQL), and families only, the Strengths and Challenges Questionnaire. Psychiatric symptoms and disorders of the patients were evaluated according to the Schedule for Mood Disorders and Schizophrenia for School-Age Children/Now and Lifetime Turkish Version. Patients were divided into 2 groups according to psychiatric symptoms and disorders. RESULTS: The most common psychiatric disorder was attention deficit and hyperactivity disorder (26%). The questionnaires filled out by the patients revealed a lower Total PedsQL Score (P = .003), PedsQL Physical Functionality Score (P = .019), and PedsQL Social Functioning Score (P = .016) in patients with psychiatric disorders. When the parents filled out the questionnaires, the Total PedsQL Score was similar in both groups. The PedsQL Emotional Functionality Score (P = .001) and PedsQL School Functionality Score (P = .004) were significantly lower in patients with psychiatric disorders. The Strengths and Difficulties Questionnaire revealed significantly higher total (P = .014) and hyperactivity/inattention subscale scores (P = .001) in those with a psychiatric disorder. CONCLUSIONS: Psychiatric disorders in kidney transplant patients adversely affect the quality of life.

Role of Systemic Inflammatory Markers in Pediatric Kidney Transplantation.

BACKGROUND: Neutrophil-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), systemic immune-inflammation index (SII = N × P/L), and neutrophil percentage-albumin ratio (NPAR) have become accepted markers of inflammation in recent years. These indices are used as indicators of disease activity, mortality, and morbidity in many diseases. This study evaluated the relationship between inflammatory indices and graft function in pediatric kidney transplant recipients. METHODS: Medical records of pediatric patients who underwent kidney transplantation at Ege University between 1995 and 2020 were reviewed retrospectively. Demographic, clinical, and laboratory data were recorded during the third month, first year, and fifth year of transplantation and at the last visit. RESULTS: The median age of the 119 patients (60 boys/59 girls) at the time of transplantation was 154 months, and the median follow-up period was 101 months. According to Spearman correlation analysis, patients' final creatinine levels were positively correlated with NLR (r = 0.319), PLR (r = 0.219), SII (r = 0.214), and NPAR (r = 0.347) of the last visit; final estimate glomerular filtration rate levels were negatively correlated with NLR (P = .010, r = -0.250) and NPAR (P = .004, r = -0.277). The median NPAR of the patients with chronic allograft dysfunction at the last visit was found to be statistically significantly higher than without (P = .032). CONCLUSION: NLR, PLR, SII, and NPAR values are correlated with creatinine levels after 5 years of kidney transplantation. The NPAR and final creatinine levels had the highest correlation coefficient among these inflammatory markers. These results suggest that inflammatory markers, especially NPAR, may be a candidate to be an indicator of ongoing inflammation in the graft.

Low-density lipoprotein and blood pressure are important risk factors for vasculopathy in children on dialysis: A single center study.

INTRODUCTION: We searched for risk factors of cardiovascular assessment among children on dialysis. METHODS: This is a cross-sectional study of cardiovascular assessment of all patients on dialysis at Ege University Children's Hospital. Pediatric patients between the ages of 6 and 21 who were on HD and peritoneal dialysis treatment were included in the study. Cardiovascular evaluation included left ventricular mass index (LVMI), pulse wave velocity (PWV), and carotid intima-media thickness (cIMT) measurements. RESULTS: Nineteen patients were included in this study. The LDL had a correlation with the cIMT z-score, but not to PWVz-score. Binary Logistic regression analysis found that only LDL was significantly associated to increased cIMT. CONCLUSION: This study reports an association between high LDL and high BP increased cIMT on dialyzed children. Strategies to reduce LDL and BP in dialysis patients may prevent vasculopathy and long-term cardiovascular complications.

Effect of Missing Data Imputation on Deep Learning Prediction Performance for Vesicoureteral Reflux and Recurrent Urinary Tract Infection Clinical Study.

Missing observations are always a challenging problem that we have to deal with in diseases that require follow-up. In hospital records for vesicoureteral reflux (VUR) and recurrent urinary tract infection (rUTI), the number of complete cases is very low on demographic and clinical characteristics, laboratory findings, and imaging data. On the other hand, deep learning (DL) approaches can be used for highly missing observation scenarios with its own missing ratio algorithm. In this study, the effects of multiple imputation techniques MICE and FAMD on the performance of DL in the differential diagnosis were compared. The data of a retrospective cross-sectional study including 611 pediatric patients were evaluated (425 with VUR, 186 with rUTI, 26.65% missing ratio) in this research. CNTK and R 3.6.3 have been used for evaluating different models for 34 features (physical, laboratory, and imaging findings). In the differential diagnosis of VUR and rUTI, the best performance was obtained by deep learning with MICE algorithm with its values, respectively, 64.05% accuracy, 64.59% sensitivity, and 62.62% specificity. FAMD algorithm performed with accuracy = 61.52, sensitivity = 60.20, and specificity was found out to be 61.00 with 3 principal components on missing imputation phase. DL-based approaches can evaluate datasets without doing preomit/impute missing values from datasets. Once DL method is used together with appropriate missing imputation techniques, it shows higher predictive performance.

Cytomegalovirus Experience in Pediatric Kidney Transplantation in 26 Years' Time.

INTRODUCTION: In this study,we investigated the presence of cytomegalovirus (CMV) infection in kidney transplanted children and its effect on kidney dysfunction. MATERIAL AND METHODS: One hundred thirty-five pediatric renal transplant patients were included in this study. The presence of CMV infection, CMV risk status, and other clinical features of the patients were evaluated retrospectively. RESULTS: Fifty-three percent of all patients and 68.8% of patients with CMV were male. The mean age was 12 years in all patients and CMV groups. According to the CMV risk classification, 40.9% of the patients with CMV infection/disease were in the high-risk group (CMV D+R-). In CMV risk groups, the presence of CMV infection/disease was similar. Cold ischemia time, male sex (patients and donors), deceased donor, higher HLA-mismatches, and cumulative antithymocyte globulin dose were found as risk factors for CMV infection/disease. Acute rejection/graft failure was observed in 27% of all patients. CMV infection has no effect on rejection/graft failure and survival. DISCUSSION: The frequency and risk factors of CMV in renal transplant children in our study were consistent with the literature. CONCLUSIONS: CMV infection was found in one-fifth of our patients and the majority (71.9%) of them developed infection in the first 6 months. In one-third of our patients acute rejection/graft failure was observed. There was no effect of CMV infection on rejection/graft failure and survival in pediatric patients with proper and effective treatment.

Evaluation of increased arterial stiffness in pediatric patients with cystic fibrosis by augmentation index and pulse wave velocity analysis.

With the increase in life expectancy, cardiovascular complications of cystic fibrosis (CF) have come to the forefront. Increased arterial stiffness is a marker of increased cardiovascular risk. The aim of this study was to compare both pulse wave velocity (PWV) and augmentation index (Aix) measurements in children with CF and to compare them with healthy controls. We hypothesized that children with CF had increased arterial stiffness, although traditional risk factors for CVD were not observed. Forty-four patients and age and sex-matched 30 healthy controls were included in the study. Hemodynamic measurements were compared in both groups, together with traditional risk factors. Peripheral blood pressure parameters of CF and control groups were similar (P > .05). Bodyweight and BMI were significantly lower in the CF group (P < .001). Serum cholesterol, HDL, and LDL levels were significantly lower in the CF group, whereas fasting blood glucose and triglyceride levels were significantly higher than the control group (P < .05). Mean ± SD Aix was significantly higher in the CF group (33.22 ± 13.87%) compared with the control group (24.93 ± 10.58%), respectively (P < .05), while PWV was similar. No significant correlation between PWV and Aix and fasting blood glucose and lipid profile in both groups (P > .05). Children with CF have been shown to have increased arterial stiffness compared to healthy children. Although there are not many traditional risk factors, increased arterial stiffness have been demonstrated in children with CF. The effects of this process starting from childhood on the development of CVD in adulthood are not known. Therefore, further studies are needed.

Pediatric Kidney Transplantation in Patients With Urologic Anomalies.

INTRODUCTION: and Aim. End-stage renal disease owing to structural urologic anomalies is frequent in the pediatric population. Impaired bladder function is thought to have a negative effect on graft function and survival. The aim of this study was to present our single-center experience and long-term follow-up results in pediatric patients who underwent renal transplantation for urologic reasons and to compare graft survival among patients who underwent transplantation for nonurologic reasons. METHOD: The paper records of renal transplanted children (<18 years of age) held by Ege University Medical Faculty between 1998 and 2018 were evaluated retrospectively. Patients with normal bladder function who underwent transplantation for urologic reasons were defined as group A, whereas patients who had impaired bladder function and underwent transplantation for urologic reasons were defined as group B; a control group was defined as group C. RESULTS: Eighty-three patients were included in the study. The creatinine values of the patients at their last visit were no different between groups (P = .930). One-, 5-, and 10-year graft survival rates were 97%, 89%, and 74%, respectively, in group A; 100% for all years in group B; and 97%, 94%, and 80%, respectively, in group C. There was no statistically significant difference in terms of graft survival between groups (P = .351). CONCLUSION: Children with end-stage renal disease owing to urologic abnormalities may be good candidates for kidney transplantation with a favorable prognosis for graft function and survival.

Lead exposure and urinary N-acetyl beta D glucosaminidase activity in adolescent workers in auto repair workshops.

PURPOSE: To investigate levels of lead (Pb) exposure and renal tubular damage among adolescent workers in auto repair workshops in Turkey. METHODS: The study was conducted on 39 adolescent workers (mean age: 16.18 +/- 3.19 years) in auto repair workshops (8 autoelectrician, 10 motor repairman, 8 auto painter, 5 turner, 8 bonnet straighter). Thirteen adult employees of battery production in the workshops (mean age: 32.08 +/- 10.94 years) and 29 healthy rural adolescent (mean age: 14.78 +/- 2.68 years) constituted the control groups. The level of blood Pb was investigated by an atomic absorption spectrophotometer and urinary N-acetyl beta- D glucosaminidase (NAG) activity was measured by a colorimetric method. Mann-Whitney U test was performed to examine group differences. RESULTS: All subjects and controls had normal blood urea, creatinine, uric acid, sodium, potassium levels, normal routine urine examination and tubular phosphorus reabsorption. Blood Pb levels in auto repair workers (8.13 +/- 7.41 mug/dL) were significantly higher than the rural control group (3.49 +/- 1.39 mug/dL) but lower than the battery workers (25.27 +/- 9.82 mug/dL). Urinary NAG (U/gr creatinine) (4.71 +/- 2.11) was lower than the battery workers (7.39 +/- 4.37), however significantly higher than the normal control group (3.07 +/- 1.20). In addition, auto painters had higher levels of Pb exposure and urinary NAG activity than the other workers (p <.05). CONCLUSION: Chronic low dose Pb exposure was found to cause renal tubular injury in children workers of auto repair workshops.

Free carnitine levels in children with steroid-sensitive nephrotic syndrome.

BACKGROUND: Carnitine transports long-chain fatty acids across the inner mitochondrial membrane. Carnitine metabolism is disturbed in some renal diseases, such as chronic renal failure. Previous studies have shown that children had normal serum free carnitine (FC) and total carnitine levels in idiopathic nephrotic syndrome, IgA nephropathy, non-IgA nephropathy and focal segmental glomerulosclerosis. The aim of the present study was to determine FC concentrations in plasma and urine during acute and remission periods of steroid-sensitive nephrotic syndrome (SSNS) and its association with hyperlipidemia. METHODS: Plasma and urinary FC concentrations were assayed in 15 children with SSNS in acute and in 16 children in remission period. Six of them were followed-up longitudinally in both periods. RESULTS: Plasma FC concentrations were significantly higher in the acute period of the disease than in the remission period and of the controls. The patients had lower FC levels in the remission period as compared to the controls. Urinary FC concentration was decreased in acute disease period when compared to the remission period and the controls. The FC concentrations in plasma and urine did not correlate with each other. Plasma FC exhibited significant positive correlation with low-density lipoprotein cholesterol, total cholesterol and trygliceride, but negative correlation with high-density lipoprotein cholesterol. CONCLUSIONS: The present study showed disturbed FC concentration in SSNS. Increased plasma and decreased urinary FC levels in acute disease might be associated with its altered renal handling or some extrarenal factors such as hyperlipidemia.

Effect of hemodialysis on carnitine levels in children with chronic renal failure.

BACKGROUND: Impaired structural and metabolic integrity of the kidney in chronic renal failure (CRF) effects carnitine metabolism by means of many factors. Depletion due to hemodialysis (HD) is one of the major concerns. The aim of the study was to investigate the effects of chronic renal failure and HD on plasma free carnitine (FC) concentrations in children. METHODS: Plasma FC concentrations were measured in age-matched 14 undialyzed patients, 20 dialyzed patients and 12 healthy children. In the HD group, measurements were done pre- and postdialysis and an hour after ceasing HD. None of the children have been receiving exogenous l-carnitine replacement. RESULTS: Plasma FC concentrations on either HD or conservative treatment were found to be decreased as compared to the healthy subjects (P < 0.001 and P = 0.001, respectively). The patients on HD had lower levels of plasma FC at the predialysis period than those on conservative treatment (P = 0.01). The FC levels significantly dropped at the postdialysis period as compared to those at the predialysis period (P < 0.001), but recovered at 1 h after ceasing HD. The mean duration of HD did not correlate with plasma FC levels at predialysis period. CONCLUSIONS: Children with CRF, either dialyzed or undialyzed, have decreased plasma FC levels. Hemo-dialysis treatment significantly depletes plasma FC concentrations during the procedure, but predialysis levels are reached 1 hr after ceasing HD.