Chronic Kidney Disease In Children Research Articles

Monitoring and maintaining quality in the paediatric haemodialysis unit.

Chronic kidney disease in children is being increasingly recognised and reported worldwide, and the focus of paediatric dialysis planning has changed from acute care alone to encompass chronic care. In many parts of the world, haemodialysis for children is performed in adult units and is based on standards established for adults. This review proposes standards for paediatric haemodialysis, incorporating special requirements for children while simultaneously drawing from the adult experience. We discuss the optimum requirements, including space utilisation, equipment needed, water treatment facilities, disposables, safety standards, staffing needs, monitoring and maintenance, infection prevention, waste disposal and quality indicators. We also review recent advancements in the field that should be incorporated into future dialysis units and the steps required for achieving carbon neutrality and protecting the environment.

Is body composition important in the context of renal function in pediatric neurogenic bladder?

Neurogenic bladder due to myelomeningocele (MMC) is a significant risk factor for chronic kidney disease in children. Cystatin C (CysC) is a more accurate GFR marker than creatinine as it is unaffected by muscle mass but may be influenced by fat mass and BMI. This study evaluates: (1) GFR measurement accuracy using CysC and creatinine in MMC-related neurogenic bladder, (2) the relationship between body composition via bioelectrical impedance analysis (BIA) and renal parameters, and (3) the use of BIA for non-invasive GFR and body composition assessment. Forty children (median age 9.96years) underwent serum creatinine, CysC testing, and BIA measurements. We assessed age, sex, spinal lesion level, anthropometric measurements, BMI, and activity using Hoffer's scale. GFR was calculated using five creatinine-based formulas, three CysC-based, and three combining CysC and creatinine, including BIA GFR as an alternative approach. Creatinine-based GFR estimates were significantly higher than CysC-based GFR. Although only 30% of MMC patients met the traditional BMI criteria for overweight/obesity, 62.5% were obese based on BIA-measured body fat percentage. Significant differences were found in CysC and CysC-based GFR equations within BMI and fat mass groups. Positive correlations were observed between CysC and body weight, BMI percentiles, body fat mass and fat-to-muscle ratio. Muscle mass positively correlated with creatinine. BIA-determined fat mass percentage is a more sensitive obesity indicator than BMI in MMC patients. CysC levels and CysC-based GFR equations are influenced by body fat mass, requiring consideration of adiposity to avoid misestimating renal impairment.

FUNCTIONAL STATE OF RENAL PARENCHYMA IN CHILDREN WITH DYSMETABOLIC NEPHROPATHY

Abstract. Metabolic nephropathy in children is a relevant issue in our time, as the prevalence of this disease is increasing. Negative consequences of metabolic nephropathy include a gradual deterioration of kidney function. A significant aspect is the fact that metabolic nephropathy can lead to the development of chronic kidney disease in children. Chronicity requires continuous medical monitoring and treatment to support kidney function and prevent further complications, such as renal insufficiency and other metabolic disorders. There is no doubt that one of the leading pathogenetic factors in kidney dysfunction is hypoxia. It can be caused by both hemodynamic disturbances associated with renal pathology and tissue respiration disorders in congenital dysplasia of the kidney's connective tissue, which arises in the embryo under the influence of epigenetic factors in utero and is further exacerbated by various epigenetic factors in postnatal life. Purpose of the work to investigate the functional state of kidney parenchyma in children with dysmetabolic nephropathy. Materials and methods. Two groups of children were examined: those with a complicated course of dysmetabolic nephropathy, a history of inflammatory episodes of the urinary system organs (Group I - UN, 52 children), and dysmetabolic nephropathy with persistent crystalluria (Group II - DN, 56 children). A control group consisted of 65 healthy children. Tubular reabsorption, as a partial function of the proximal segment of the nephrons, was assessed based on urinolysis test data, specifically the levels of excretion of inorganic phosphates, proteinuria, amino acids, reduced sugars (maltose, sucrose, fructose by Benedict's test), glucose (by Gaines's test), and calcium (by Sulcovitch's test). The level of creatinine and glycosaminoglycan (GAG) excretion in daily urine in children with dysmetabolic nephropathy was determined and compared with data from healthy children in the control group. Research results. In children from both observation groups, a significantly high frequency of hyperphosphaturia (94.23% and 96.42%), calciuria (94.23% and 89.28%), glucosuria (78.85% and 73.21%), increased excretion of amino acids in the urine (40.38% and 37.5%), microproteinuria (32.69% and 28.57%), and increased excretion of reduced sugars (28.85% and 26.79%) were observed. The daily excretion of creatinine, glycosaminoglycans, and oxypyrrolidine in the urine of children with dysmetabolic nephropathy indicates a significantly reduced level of creatinine in the daily urine in the majority of children in Group I (0.56±0.19 g/L, 80.77% of those examined) and more than half of the children in Group II (0.83±0.08 g/L, 57.14% of those examined), compared to the data from healthy children in the control group (1.25±0.75 g/L, 0.0%). Conclusions. The analysis of the functional state of the kidney tissue in oxalate dysmetabolic nephropathy in children allows us to testify to the presence of tubular and glomerular partial renal tissue failure. Significant decreases in creatinine excretion and increased excretion of glycosoaminoglycans in the urine and significantly higher levels of oxyproline in the urine indicate the presence of glomerular type partial renal failure in oxalate dysmetabolic nephropathy in children and the presence of undifferentiated dysplasia of the renal connective tissue with the onset of its sclerosis with subsequent transition to interstitial nephritis. The presence of these changes in the vast majority of children with complicated course of oxalate dysmetabolic nephropathy in the period of clinical and laboratory remission of the inflammatory process indicates the priority of renal connective tissue dysplasia and paucity of renal failure in the pathogenesis of dysmetabolic nephropathy and its severity in children. Indicators of oxyproline creatinine, glycosoaminoglycans and oxyproline excretion can be used as epigenetic markers of susceptibility to dysmetabolic nephropathy in general and its more severe course in particular. Keywords: dysmetabolic nephropathy, functional state, kidney parenchyma, nephron.

Characteristics of risk factors and molecular genetic parameters in children with chronic kidney disease

Introduction. Information about the health status of children with nephrological pathology includes many manifestations of functional system disorders. However, early diagnosis of chronic kidney disease (CKD) is rarely carried out due to the lack of reliable predictors of this process.The aim: To analyze the association of anamnestic signs, laboratory and genetic research results with chronic kidney disease in children to identify potential predictors of the disease.Material and Methods. The data were obtained from a single-center retrospective catamnestic cohort study (2011–2022). The main group included 128 children with chronic kidney disease aged 1 to 17 years. The comparison group consisted of 30 children without diagnosed kidney pathology aged from 1 to 17 years. There were no statistically significant differences in sex and age between the two groups. The informative value of more than 150 features, including anamnestic ones, the state of the mother’s health during pregnancy, antenatal and perinatal factors, features of the child’s development and manifestation of the disease, the results of clinical, laboratory, and genetic research methods were evaluated. An integrated approach to the study included methods for hypotheses testing on statistical significance of indicators differences, regression analysis, and ROC analysis.Results. Hereditary burden of nephrological pathology in the family, unfavorable course of pregnancy (anemia, toxicosis of the first half), obstetric and somatic pathology in the mother, the serial number of childbirth are associated with the subsequent development of CKD. Erythrocytes in the urine and daily protein loss were detected only in the group of children with CKD. In this group, a decrease in the number of red blood cells, increased erythrocyte sedimentation rate (ESR), allergies at an early age, otitis media, persistent viral and bacterial infections, and a number of signs of connective tissue dysplasia were more common. The main group statistically significantly differed from the comparison group by a number of polymorphic genetic markers. It can be assumed that the polymorphisms AGT1 Thr174Thr, AGT2 Thr235Thr, NO3 C786T are associated with a predisposition to CKD, and the products of polymorphisms AGT1 Thr174Met, AGT2 Met235Met, R1 AGT2 C1166C, NO3 T786T may have a protective effect according to development of CKD.Conclusion. The risk factors for CKD identified in this study can be used to develop software to support medical decision making for early detection of children at high risk of disease progression.

“Chronic Kidney Disease In Children Of A Paediatric Critical Care Nephrology & Dialysis Department: A Study In Bangladesh Shishu (Children) Hospital & Institute, Bangladesh”

Introduction: Chronic Kidney Disease (CKD) for children is a major problem of public health both in poor and developed countries. This study aimed to investigate the diagnosis and the management options of CRF for children. Patients and methods: This study retrospectively evaluated patients who had chronic kidney disease (CKD) in the Paediatric Critical Care & Nephrology Department in Dhaka Shishu Children Hospital, Dhaka, Bangladesh between July 2017 and December 2017. Results: Over 6 month’s period, we diagnosed and managed 41 children with CRF. The estimated incidence of CRF is 4.7 new cases per million-child population per year. Parental consanguinity was found in 16 patients (39%). Family history with kidney disease was noted in 6 cases (14.6%). Malformations of the urinary tract were observed in 24 patients (58.5%). Other causes are divided into hereditary kidney disease in 8 patients (19.5%) predominated by primary hyperoxaluria, in vascular nephropathy who were objectified in 5 patients (12%) whereas glomerulopathy were represented in 3 cases (7.5%). No etiology was found in 1 patient. Over the 6 month’s 22 patients (54%) had renal replacement therapy (RRT). Peritoneal dialysis (PD) was practiced in over then 90% of patients. A passage from peritoneal dialysis to hemodialysis was done in 8 patients. Only four patients had a kidney transplant. The rate of overall mortality in our series was 40% with median of follow-up in 54 months. Conclusion: In Tunisia and in all Low source country children with CRF must be treated by pediatric nephrologists and the pediatric renal transplantation must be developed.

Hypertension and Left Ventricular Strain in Pediatric Chronic Kidney Disease.

Left ventricular global longitudinal strain (LV GLS) on echocardiography is a sensitive yet clinically significant marker of myocardial dysfunction. Reduced LV GLS is prevalent in adults with chronic kidney disease and hypertension and is associated with adverse cardiovascular outcomes. It may be a biomarker of chronic kidney disease-associated myocardial dysfunction in children, but data are limited. Our objective was to describe LV GLS in the CKiD study (Chronic Kidney Disease in Children) and to examine the association between blood pressure (BP) and reduced LV GLS. A single apical 4-chamber view was used to estimate LV GLS. Our main analyses examined the association of clinic BP with the absolute value of LV GLS and LV GLS dichotomized at 16. Sensitivity analyses using 24-hour ambulatory BP monitoring data were also performed. Generalized estimating equations were used to account for within-person correlation and to estimate robust SEs for 95% CIs. Covariates in adjusted models included: age, sex, race, estimated glomerular filtration rate, urine protein, hemoglobin, left ventricular hypertrophy, and the use of renin-angiotensin system inhibitors. LV GLS was measured in 962 person-visits. A total of 77 assessments had an LV GLS <16. In adjusted models, both clinic systolic BP (odds ratio, 1.02 [95% CI, 1.01-1.03]) and diastolic BP (odds ratio, 1.02 [95% CI, 1.00-1.03]) percentiles were associated with LV GLS <16. Having awake or nighttime diastolic BP hypertension on ambulatory BP monitoring was significantly associated with a lower absolute value of LV GLS. Office systolic and diastolic hypertension was associated with diminished LV GLS. Only diastolic hypertension detected on ambulatory BP monitoring was associated with lower LV GLS.

Acylcarnitines and Cardiovascular Outcomes in the CKD in Children (CKiD) and Chronic Renal Insufficiency Cohort (CRIC) Studies

Acylcarnitines and Cardiovascular Outcomes in the CKD in Children (CKiD) and Chronic Renal Insufficiency Cohort (CRIC) Studies

Study of Hematological Parameters of Children with Chronic Kidney Disease on Hemodialysis and Peritoneal Dialysis in Wasit Governorate

ABSTRACT: The aim of this study was to evaluate some hematological parameters in pediatric patients with chronic kidney disease (CKD) in children who are on hemodialysis (HD) and peritoneal dialysis (PD). It is characterized by its irreversibility. Deterioration of kidney function that gradually progresses to end-stage renal function illness. Over the past two decades, the incidence of CKD in children has increased Steady increase. The major health consequences of CKD include not only progression to kidney failure, but also increased risk of cardiovascular disease. The study was conducted in the dialysis unit of Al-Zahraa Teaching Hospital in Wasit governorate and the Child Protection Teaching Hospital -Medical City in Baghdad. During the period from November 2023 to April 2024. This study aimed to examination of risk factors (height, weight, age, sex, inheritance of the disease in the family, medical history, symptoms of the disease, other diseases, treatment methods) for CKD in general. The biochemical effect was also studied and blood indicators for patients on HD and PD purpose. For the purpose of studying the hematological of patients undergoing dialysis and comparing those results with their values ​​in both HD and PD. This study was done on (80) Iraqi children (30) of them suffering from kidney failure and (50) of them was healthy with age range (1 day -17) years old. HD was performed for 22 children with CKD, and PD was performed for 8 patients with CKD. This study involved dividing patients into three Groups according to the age at which the disease begins, from (1 day-5 years), (6-11) years and (12-17) years, included 15 females and 15 males. Samples were collected based on the incidence of CKD and type of treatment, as well as the diagnosis of all patients according to international standard by the physician. The results of our study with regard to the hematological of patients undergoing PD showed a significant increase in the levels of (WBC) p ≤ 0.05 and also showed a significant decrease in the levels of (RBC, Hb, PCV, ESR) p ≤ 0.05. a non-significant increase in TSH levels p ≥ 0.05. The results of our study with regard to the hematological parameters of patients on HD showed a significant increase in the levels of (ESR) p ≤ 0.05 and a significant decrease in the levels of (WBC, RBC, PCV, Hb) p ≤ 0.05.

Assessment of brain-derived neurotrophic factor and irisin concentration in children with chronic kidney disease: a pilot study

Patients suffering from chronic kidney disease (CKD) are particularly placed at risk of multiorgan complications. One of them is malnutrition, which adds up to a higher mortality factor among them. This study was designed to determine the usefulness of brain-derived neurotrophic factor (BDNF) and irisin assays in the assessment of CKD development. The study group included 28 children with CKD at stages 2-5 treated conservatively. The outcome of our study revealed decreased serum BDNF and irisin levels in CKD patients, whereas urine concentrations were increased for BDNF and decreased for irisin, comparing to healthy controls. There was a positive correlation between anthropometric measures and urine BDNF concentration, as well as anthropometric measures and both serum and urine irisin levels in the study group, however no dependence of the tested markers on the stage of CKD was observed. In recent years, a role of myokines was described as vital for maintaining metabolic homeostasis therefore we suspect a potential role of these multifaceted markers in detecting malnutrition in CKD children.

Разработка интеллектуальной системы поддержки принятия врачебных решений для прогнозирования хронической болезни почек у детей.

Introduction. Chronic kidney disease is often diagnosed too late. Currently, the diagnostic accuracy is 44.1%, which highlights the urgent need to improve diagnostic methods. The purpose of the study: to develop a model – a system of support of medical decisions to predict chronic kidney disease in children. Materials and methods. А one-center retrospective cohort study (2011–2022) of children with chronic kidney disease of 1–4 stages in the age from 1 to 17 years. To construct a predictive model for diagnosing chronic kidney disease in children, an ensemble learning method was used, using which the models obtained by machine learning algorithms were combined: multi-factor logistic regression and decision tree. The models use five variables: asthenic physique in a child, loss of protein and red blood cells with urine, ESR and blood serum sodium. Results. The study involved 158 patients. The main group includes 128 children with chronic kidney disease of stage 1–4 aged 1–17. The comparison group was 30 children with no diagnosed kidney pathology aged 1 to 17. The children of the two groups did not differ statistically by sex and age. A model has been obtained to predict chronic kidney disease in children on a test sample with an accuracy of 93.5% [87.1; 100.0]%; a sensitivity of 92.0% [82.1; 100.0]; a specificity of 100.0% [100.0; 100.0.0]; ROC-AUC = 98.7%;100.0].0. Obtained model of excellent quality (&gt;90%). The model describes 90.3% [83.8; 96.1]% variance. Conclusion. The proposed model demonstrates excellent predictive ability and may be of important clinical importance for predicting the chronic process in primary health care, where symptoms associated with the risk of chronic kidney disease, may be overlooked. Predicting and developing early nephroprotective strategies can lead to better treatment outcomes and prolong life.

A commentary from the European Renal Best Practice (ERBP) on the Kidney Disease Improving Global Outcomes (KDIGO) 2024 Clinical Practice Guideline for the Evaluation and Management of Chronic Kidney Disease in children and adults

Abstract The Kidney Disease: Improving Global Outcomes (KDIGO) 2024 Guidelines for identification and management of chronic kidney disease (CKD) are a welcome development coming 12 years after the paradigm changing 2012 guidelines. We are living in an unprecedented era in nephrology with novel therapies, including sodium-glucose cotransporter-2 inhibitors, glucagon-like peptide-1 receptor agonists and non-steroidal mineralocorticoid receptor antagonists now being proven in multiple randomised controlled clinical trials to reduce both the progression of CKD and cardiovascular morbidity and mortality. The KDIGO 2024 CKD guideline is aimed at a broad audience looking after children and adults with CKD and provide practical and actionable steps to improve care. This commentary reviews the guideline sections pertaining to the evaluation and risk assessment of individuals with CKD from a European perspective. We feel that despite the last guideline being published 12 years ago, and that the assessment of CKD has been emphasized by many other national/international nephrology, cardiology and diabetology guidelines and societies, the diagnosis and treatment of CKD remains poor across Europe. As such the KDIGO 2024 CKD Guidelines should be seen as an urgent call to action to improve diagnosis and care of children and adults with CKD across Europe. We know what we need to do. We now need to get on and do it.

Cystatin C as a Marker of Kidney Function in Children.

This review examines the reliability of cystatin C as a biomarker for kidney function in paediatric populations. Chronic kidney disease (CKD) affects a significant number of children globally, leading to severe health complications such as anaemia, hypertension, and growth disorders. Traditionally, kidney function has been assessed using the estimated glomerular filtration rate derived from serum creatinine, though this method is flawed due to variability in muscle mass, age, gender, and diet. Cystatin C offers an alternative as it is less influenced by these factors. Evidence from various studies indicates that cystatin C provides a more accurate assessment of kidney function, especially in neonates and children with urinary tract malformations. Additionally, it is more reliable in early detection of acute kidney injury in paediatric intensive care units. Despite its potential, cystatin C is not yet widely adopted in clinical guidelines, primarily due to a lack of large-scale paediatric studies. Nonetheless, existing research supports its utility in providing a consistent and precise measure of kidney function across different paediatric age groups, suggesting that it could enhance early diagnosis and management of CKD in children if more extensive validation studies are conducted.

Efficacy and Safety of Allopurinol on Chronic Kidney Disease Progression: A Systematic Review and Meta-Analysis.

Hyperuricemia is associated with the progression of chronic kidney disease (CKD). Whether urate-lowering treatment with allopurinol can delay disease progression remains controversial. Relevant databases were searched. Randomized clinical trials comparing the efficacy and -safety of allopurinol in patients with CKD were selected. The primary outcomes were changes in serum uric acid concentration and estimated glomerular filtration rate (eGFR). Random-effects modeling was used to -calculate the standard mean difference (SMD) with 95% CIs. Four trials enrolling 698 participants were included. All were 2-arm parallel trials with a mean duration follow-up of 22.5 months. Congenital anomalies of the kidney and urinary tract were the most common cause of CKD in children, whereas diabetes was the leading cause of CKD in adults. Allopurinol significantly increased the eGFR compared with control groups (SMD, 2.04; 95% CI, 0.60-3.49; p = 0.005; I2 = 98.23%). Allopurinol led to a significant decrease in serum uric acid concentration compared with the control group (SMD, -5.16; 95% CI, -8.31 to -2.01; p = 0.001; I2 = 98.80%). No significant difference in adverse effects was identified between treatment and control groups. Allopurinol treatment in patients with CKD and hyperuricemia slows the decline in eGFR as compared with placebo, without risk of increased adverse effects.

Phenotypic quantification of Nphs1-deficient mice.

Steroid-resistant nephrotic syndrome (SRNS) is the second most frequent cause of chronic kidney disease in children and young adults. The most severe form of steroid-resistant nephrotic syndrome is congenital nephrotic syndrome Finnish type (CNSF), caused by biallelic loss-of-function variants inNPHS1,encoding nephrin. Since each of the 68 monogenic causes of steroid-resistant nephrotic syndrome represents a rare cause of the disease, tailoring therapeutic interventions to multiple molecular targets remains challenging, suggesting gene replacement therapy (GRT) as a viable alternative. To set the ground for a gene replacement study in vivo, we established rigorous, quantifiable, and reproducible phenotypic assessment of a conditional Nphs1 knockout mouse model. By breeding a floxed Nphs1fl/- mouse (Nphs1tm1Afrn/J) previously studied for pancreatic β-cell survival with a podocin promoter-driven Cre recombinase mouse model (Tg(NPHS2-Cre)295Lbh/J), we generated mice with podocyte-specific nephrin deficiency (Nphs1fl/fl NPHS2-Cre +). We observed a median survival to postnatal day P5 in nephrin-deficient mice, whereas heterozygous control mice and wild type (WT) control group showed 90% and 100% survival, respectively (at P50 days). Light microscopy analysis showed a significantly higher number of renal-tubular microcysts per kidney section in nephrin-deficient mice compared to the control groups (P < 0.0022). Transmission electron microscopy demonstrated reduced foot process (FP) density in nephrin-deficient mice compared to controls (P < 0.0001). Additionally, proteinuria quantitation using urine albumin-to-creatinine ratio (UACR) was significantly higher in nephrin-deficient mice compared to controls. This study represents the first comprehensive description of the kidney phenotype in anephrin-deficient mouse model, laying the foundation for future gene replacement therapy endeavors.

Vitamin D and its associations with blood pressure in the Chronic Kidney Disease in Children (CKiD) cohort.

Vitamin D (25OHD) can modulate pathways and mechanisms that regulate blood pressure (BP). Observational studies in children and adults have shown an inverse association between 25OHD and BP. Studies evaluating associations between 25OHD and BP in pediatric chronic kidney disease are limited. We evaluated the associations between 25OHD and BP using data from the Chronic Kidney Disease in Children (CKiD) study. Clinic or ambulatory BP index was defined as participant's BP divided by 95th age-sex-height-specific BP percentile, an index > 1 suggests hypertension. Primary outcomes of interest were changes in systolic and diastolic clinic and ambulatory BP indices over follow-up. Linear mixed-effects models were used to evaluate associations between BP indices and 25OHD. The study cohort consisted of 370 participants who contributed 970 person-visits. A subset of 194 participants with ambulatory BP data contributed 465 person-visits. There was an association between baseline 25OHD levels and clinic systolic BP index such that for every 10ng/ml lower 25OHD, clinic systolic BP index was 1.0% higher (95%CI: 0.2-1.8, p = 0.016) between participants. The association between clinic diastolic BP index with baseline 25OHD was not significant. For within-person changes, longitudinal decreases in 25OHD were not significantly associated with concomitant increases in clinic systolic or diastolic BP index. There were no significant associations between 25OHD levels at baseline or longitudinally with 24-h ABPM indices. Low 25OHD levels were associated with higher clinic systolic BP in children with CKD. Vitamin D supplementation to maintain normal 25OHD levels might be a useful adjunctive treatment in optimizing BP control in these high-risk patients.

Prevalence and associated factors of impaired kidney functions among children and adolescents in India: insights from the Comprehensive National Nutrition Survey (CNNS) 2016-18

BackgroundChronic kidney disease (CKD) is a significant public health problem. The burden of CKD in children and adolescents in India is not well described. We used data from the recent Comprehensive National Nutrition Survey (CNNS) to estimate the prevalence of impaired kidney function (IKF) and its determinants in children and adolescents between the ages of 5 and 19.MethodsCNNS 2016–18 adopted a multi-stage sampling design using probability proportional to size sampling procedure after geographical stratification of urban and rural areas. Serum creatinine was tested once in 24,690 children and adolescents aged 5–19 years. The estimated glomerular filtration rate (eGFR) was derived using the revised Schwartz equation. The eGFR value below 60 ml/min/1.73 m2 is defined as IKF. Bivariate analysis was done to depict the weighted prevalence, and multivariable logistic regression examined the predictors of IKF.ResultsThe mean eGFR in the study population was 113.3 + 41.4 mL/min/1.73 m2. The overall prevalence of IKF was 4.9%. The prevalence in the 5–9, 10–14, and 15–19 year age groups was 5.6%, 3.4% and 5.2%, respectively. Regression analysis showed age, rural residence, non-reserved social caste, less educated mothers, Islam religion, children with severe stunting or being overweight/obese, and residence in Southern India to be predictors of IKF.ConclusionsThe prevalence of IKF among children and adolescents in India is high compared to available global estimates. In the absence of repeated eGFR-based estimates, these nationally representative estimates are intriguing and call for further assessment of socio-demographic disparities, genetics, and risk behaviours to have better clinical insights and public health preparedness.

Wilms tumor characteristics, surgical management, outcomes, and chronic kidney disease in children with WAGR syndrome: A report from the International WAGR Syndrome Association survey.

Children with WAGR (Wilms tumor, aniridia, genitourinary anomalies, and range of development delays) syndrome are predisposed to Wilms tumor (WT) and intrinsic kidney disease. Using the comprehensive International WAGR Syndrome Association (IWSA) survey of children with WAGR syndrome, we analyzed tumor characteristics, treatment and congenital risk factors, and kidney function in children with WAGR and WT. Descriptive statistics were utilized including demographics, treatment strategies, and patient outcomes. Comparisons were made between patients with WAGR and WT to those with WAGR alone. A multivariable logistic regression was completed for risk of developing WT and to identify predictors of chronic kidney disease (CKD). Sixty-four of 145 children with WAGR developed WT (44.1%). Three relapsed and one died. CKD developed in five children with WAGR without WT (5/81, 6.2%), and in 34 with WAGR and WT (34/64, 28.3%). Children with WAGR and WT were younger (p=.017), and had a greater association with CKD than WAGR children without WT (p<.0001). Two children with WT required hemodialysis, and one underwent kidney transplantation. By univariate analysis, CKD at any stage was associated with complete nephrectomy for the WT surgery (p<.0001), chemotherapy duration greater than 12months, and three-drug therapy. Upon multivariate analysis, prior nephrectomy was the only significant variable (p=.0002). Epidemiological analysis of children with WAGR demonstrated favorable oncologic outcomes, but high rate of early CKD in those who developed WT. Further study of the use of nephron-sparing surgery in children with WAGR and strategies to delay or treat early CKD are needed.

Prevalence of chronic kidney disease in children of school going age visiting outpatient department of A Tertiary Care Hospital in Karachi, Pakistan.

Objective: To determine the prevalence of chronic kidney disease (CKD) among children of school-going age. Study Design: Cross-sectional study. Setting: Department of Pediatric Medicine, National Institute of Child Health, Karachi, Pakistan. Period: July 2023 to December 2023. Methods: A total of 130 children of both genders, aged between 5-15 years, visiting outpatient department of pediatric medicine were analyzed. Demographic and clinical information were noted. Laboratory investigations like urinalysis and serum creatinine were performed. CKD was diagnosed on the basis of “Kidney Disease Outcomes Quality Initiative (KDOQI)” guidelines. Results: In a total of 130 children, 67 (51.5%) children were male. Overall, the mean age was 8.43±2.45 years. Hypertension was diagnosed in 10 (7.7%) children. Anemia was present in 103 (79.2%) patients. The mean hemoglobin, serum creatinine, and blood urea nitrogen were 9.41±1.98 g/dl, 0.48±0.37 mg/dl, and 34.20±33.18 mg/dl, respectively. CKD was diagnosed in 10 (7.7%) children. Among these 10 children diagnosed with CKD, 7 were having CKD stage-1 whereas CKD stage-2 was noted in 3 children. Presence of hypertension (p=0.006), and albuminuria (p&lt;0.001) were associated with CKD. Conclusion: The prevalence of CKD in school going age children was 7.7%. Presence of hypertension, and albuminuria were linked with CKD.

Advancing Genetic Testing in Kidney Diseases: Report From a National Kidney Foundation Working Group

About 37 million people in the United States have chronic kidney disease, a disease that encompasses diseases of multiple causes. About 10% or more of kidney diseases in adults and about 70% of selected chronic kidney diseases in children are expected to be explained by genetic causes. Despite the advances in genetic testing and an increasing understanding of the genetic bases of certain kidney diseases, genetic testing in nephrology lags behind other medical fields. More understanding of the benefits and logistics of genetic testing is needed to advance the implementation of genetic testing in chronic kidney diseases. Accordingly, the National Kidney Foundation convened a Working Group of experts with diverse expertise in genetics, nephrology, and allied fields to develop recommendations for genetic testing for monogenic disorders and to identify genetic risk factors for oligogenic and polygenic causes of kidney diseases. Algorithms for clinical decision making on genetic testing and a road map for advancing genetic testing in kidney diseases were generated. An important aspect of this initiative was the use of a modified Delphi process to reach group consensus on the recommendations. The recommendations and resources described herein provide support to nephrologists and allied health professionals to advance the use of genetic testing for diagnosis and screening of kidney diseases.

The Association Between Markers of Inflammation and the Progression of Chronic Kidney Disease in Children with Congenital Anomalies of the Kidney and Urinary Tract (CAKUT).

The Association Between Markers of Inflammation and the Progression of Chronic Kidney Disease in Children with Congenital Anomalies of the Kidney and Urinary Tract (CAKUT).