In contrast to the increasing availability of information pertaining to the care of children with chronic kidney disease (CKD) from large-scale observational and interventional studies, ...epidemiological information on the incidence and prevalence of pediatric CKD is currently limited, imprecise, and flawed by methodological differences between the various data sources. There are distinct geographic differences in the reported causes of CKD in children, in part due to environmental, racial, genetic, and cultural (consanguinity) differences. However, a substantial percentage of children develop CKD early in life, with congenital renal disorders such as obstructive uropathy and aplasia/hypoplasia/dysplasia being responsible for almost one half of all cases. The most favored end-stage renal disease (ESRD) treatment modality in children is renal transplantation, but a lack of health care resources and high patient mortality in the developing world limits the global provision of renal replacement therapy (RRT) and influences patient prevalence. Additional efforts to define the epidemiology of pediatric CKD worldwide are necessary if a better understanding of the full extent of the problem, areas for study, and the potential impact of intervention is desired.
Carnitine metabolism and homeostasis is significantly altered in patients receiving maintenance dialysis. Current literature in the adult and pediatric dialysis populations suggest a high prevalence ...of carnitine deficiency, which may lead to erythropoietin-resistant anemia, cardiomyopathy, and muscle weakness. However, the results of pediatric dialysis studies are limited and have not provided the evidence necessary to support strong recommendations or guidelines pertaining to carnitine management. The characteristics and function of carnitine, the definition and consequences of deficiency, a brief overview of recent adult studies, and current studies on carnitine supplementation in pediatric hemodialysis (HD) and peritoneal dialysis (PD) populations are discussed in this review.
The multiple and complex functions of the renal tubule in regulating water, electrolyte, and mineral homeostasis make it prone to numerous genetic abnormalities resulting in malfunction. The ...phenotypic expression depends on the mode of interference with the normal physiology of the segment affected, and whether the abnormality is caused by loss of function or, less commonly, gain of function. In this review we address the current knowledge about the association between the genetics and clinical manifestations and treatment of representative disorders affecting the length of the nephron.
Our objective was to examine serum ferritin trends after conversion to permanent vascular access (PVA) among children who started hemodialysis (HD) using tunneled cuffed catheters (TCC). ...Retrospective chart reviews were completed on 98 subjects from 20 pediatric HD centers. Serum ferritin levels were collected at the creation of PVA and for two years thereafter. There were 11 (11%) arteriovenous grafts (AVG) and 87 (89%) arteriovenous fistulae (AVF). Their mean TCC use was 10.4 ± 17.3 months. Serum ferritin at PVA creation was elevated at 562.64 ± 492.34 ng/mL, increased to 753.84 ± 561.54 ng/mL (
= < 0.001) in the first year and remained at 759.60 ± 528.11 ng/mL in the second year (
= 0.004). The serum ferritin levels did not show a statistically significant linear association with respective serum hematocrit values. In a multiple linear regression model, there were three predictors of serum ferritin during the first year of follow-up: steroid-resistant nephrotic syndrome as primary etiology (
= 0.035), being from a center that enrolled >10 cases (
= 0.049) and baseline serum ferritin level (
= 0.017). Increasing serum ferritin after conversion to PVA is concerning. This increase is not associated with serum hematocrit trends. Future studies should investigate the correlation of serum transferrin saturation and ferritin levels in pediatric HD patients.
Children with lupus nephritis (LN) are at high risk of developing kidney failure requiring initiation of kidney replacement therapy. This study compared outcomes among children with LN on dialysis ...with children with non-lupus glomerular disease and investigated risk factors for adverse outcomes among children with LN on dialysis.
Retrospective cohort study.
Children and adolescents aged 6-20 years with LN (n = 231) and non-lupus glomerular disease (n = 1,726) who initiated maintenance dialysis 1991-2018 and were enrolled in the North American Pediatric Renal Trials and Collaborative Studies (NAPRTCS) registry.
Lupus nephritis.
Hospitalization, mortality, and time to transplant.
Contingency tables were used to compare hospitalizations, and multivariable cause-specific hazards models were used to compare rates of death and transplantation in children with LN compared with those with non-lupus glomerular disease. Using data from children with LN, multivariable logistic regression models were fit to evaluate the risk factors for hospitalization, and multivariable Cox regression models were fit to evaluate factors associated with kidney transplantation.
Children with LN were more likely to be hospitalized in the first year after dialysis initiation (63.3% vs 48.6%, P < 0.001) and were less likely to receive a kidney transplant in the first 3 years after dialysis initiation (year 0-1: adjusted hazard ratio AHR, 0.36 95% CI, 0.23-0.57, P < 0.001; year 1-3: AHR, 0.73 95% CI, 0.54-0.98, P = 0.04). Anemia was associated with hospitalization after dialysis initiation (adjusted OR, 4.44 95% CI, 1.44-13.66, P = 0.01). Non-White race was associated with a lower rate of kidney transplantation (AHR, 0.47 95% CI, 0.27-0.82, P = 0.01). LN was not associated with death while on dialysis (AHR, 1.21 95% CI, 0.47-3.11, P = 0.7).
The NAPRTCS registry does not collect information on lupus disease activity or medication doses and has limited data on medication use.
Children and adolescents with LN on dialysis are at higher risk for adverse outcomes including hospitalization and lower rates of kidney transplantation compared with children with non-lupus glomerular disease receiving maintenance dialysis.
The measurement of urine concentration provides information concerning the kidney's ability to appropriately respond to variations in fluid homeostasis. It also assists in the interpretation of other ...tests performed on the same urine specimen. The gold standard of estimating urinary concentration is the measurement of its osmolality; however, this procedure is not readily available to the practicing physician. Therefore, urine concentration is usually determined by measurement of its specific gravity (SG), which provides a fair estimate of urine osmolality. Over the years numerous tests have been developed to measure urine SG in a simple, quick, reliable and easily available method. These tests measure SG either directly (e.g., gravimetry) or by indirect methods (e.g., refractometry and reagent strip). All these tests have certain limitations based on their underlying physical principles. Specific gravity as measured by refractometry is influenced by proteinuria, such that for each 10 g/l protein the SG increases by 0.003. SG is also influenced by glucosuria such that it increases by approximately 0.002 per 10 g/l glucose when compared with urinary osmolality. Unlike osmolality, which is only affected by the number of particles, refractometry is affected by number, mass and chemical structure of the dissolved particles; hence large molecules like radiographic contrast or mannitol will increase SG relative to osmolality. The reagent strip is minimally affected by glucose, mannitol or radiographic contrast. However, it is affected by urinary pH such that only urine in the pH range of 7.0-7.5 can be correctly interpreted. The measurement of SG by reagent strip is based on the ionic strength of the urine and thus is significantly affected by the ionic composition of the urine and by proteins which have an electric charge in solution. In our experience, SG measured by the refractometer is consistently more accurate than the reagent strip. For the clinician who is interpreting urine SG results, it is important to be aware of these limitations and understand the reasons for possible potential errors of each particular method.
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