RESUMEN
BACKGROUND: The Kidney Donor Risk Index (KDRI) by Rao et al. was developed to measure the quality of kidney allografts. While Rao's KDRI has been found to be a robust measure of kidney allograft survival for adult kidney transplant recipients, many studies have indicated the need to create a distinct pediatric KDRI. METHODS: Our retrospective study utilized data from the United Network for Organ Sharing database. We examined 9295 deceased donor recipients' data for age < 18 years from 1990 to 2020. We performed a multivariate Cox regression to determine the significant recipient and transplant factors impacting pediatric kidney allograft survival. RESULTS: Multivariate analysis found 5 donor factors to be independently associated with graft failure or recipient death: age, female sex, anoxia as the cause of death, history of cigarette use, and cold ischemia time. Using receiver operator characteristic (ROC) curve analysis and analyzing the predictive value of each KDRI at 1, 5, and 10 years, the proposed pediatric KDRI had a statistically significant and higher predictive value for pediatric recipients at 5 (0.60 versus 0.57) and 10 years (0.61 versus 0.57) than the Rao KDRI. CONCLUSIONS: The proposed pediatric KDRI may provide a more accurate and simpler index to assess the quality of kidney allografts for pediatric recipients. However, due to the mild increase in predictive capabilities over the Rao index, the study serves as a proof of concept to develop a pediatric KDRI. Further studies should focus on increasing the index's predictive capabilities. A higher resolution version of the Graphical abstract is available as Supplementary information.
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Trasplante de Riñón , Adulto , Humanos , Niño , Femenino , Adolescente , Trasplante de Riñón/efectos adversos , Estudios Retrospectivos , Supervivencia de Injerto , Riñón , Trasplante Homólogo , Donantes de Tejidos , Receptores de TrasplantesRESUMEN
BACKGROUND: Enteroviruses can cause severe infections, including viral myocarditis, meningitis, acute flaccid myelitis, and viral myositis. METHODS/RESULTS: We report a 3-year-old female renal transplant recipient who presented to a tertiary care hospital with elevated serum liver aminotransferases and subsequently developed proximal muscle pain, weakness, and respiratory distress during the first week of hospitalization. Imaging of the lower extremities revealed diffuse myositis of the proximal thigh and pelvic muscles. A muscle biopsy was obtained and revealed necrotizing myositis with immunostaining positive for enterovirus, consistent with a diagnosis of enterovirus necrotizing myositis. She had complete resolution of symptoms with steroids, intravenous immune globulin, reduced tacrolimus dose, and physical therapy. CONCLUSIONS: Enterovirus myositis should be included in the differential diagnosis for necrotizing myositis following renal transplantation in children.
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Infecciones por Enterovirus , Enterovirus , Fascitis Necrotizante , Trasplante de Riñón , Mielitis , Miositis , Niño , Preescolar , Infecciones por Enterovirus/diagnóstico , Infecciones por Enterovirus/patología , Femenino , Humanos , Trasplante de Riñón/efectos adversos , Mielitis/complicaciones , Miositis/diagnóstico , Miositis/tratamiento farmacológico , Miositis/etiologíaRESUMEN
BACKGROUND: Kidney transplantation in small children is technically challenging. Consideration of whether to use intraperitoneal versus extraperitoneal placement of the graft depends on patient size, clinical history, anatomy, and surgical preference. We report a large single-center experience of intraperitoneal kidney transplantation and their outcomes. METHODS: We conducted a retrospective review of pediatric patients who underwent kidney transplantation from April 2011 to March 2018 at a single large volume center. We identified those with intraperitoneal placement and assessed their outcomes, including graft and patient survival, rejection episodes, and surgical or non-surgical complications. RESULTS: Forty-six of 168 pediatric kidney transplants (27%) were placed intraperitoneally in children mean age 5.5 ± 2.3 years (range 1.6-10 years) with median body weight 18.2 ± 5 kg (range 11.4-28.6 kg) during the study period. Two patients (4%) had vascular complications; 10 (22%) had urologic complications requiring intervention; all retained graft function. Thirteen patients (28%) had prolonged post-operative ileus. Eight (17%) patients had rejection episodes ≤6 months post-transplant. Only one case resulted in graft loss and was associated with recurrent focal segmental glomerular sclerosis (FSGS). Two patients (4%) had chronic rejection and subsequent graft loss by 5-year follow-up. At 7-year follow-up, graft survival was 93% and patient survival was 98%. CONCLUSIONS: The intraperitoneal approach offers access to the great vessels, which allows greater inflow and outflow and more abdominal capacity for an adult donor kidney, which is beneficial in very small patients. Risk of graft failure and surgical complications were not increased when compared to other published data on pediatric kidney transplants.
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Glomeruloesclerosis Focal y Segmentaria , Fallo Renal Crónico , Trasplante de Riñón , Adulto , Niño , Preescolar , Glomeruloesclerosis Focal y Segmentaria/etiología , Rechazo de Injerto , Supervivencia de Injerto , Humanos , Lactante , Fallo Renal Crónico/etiología , Fallo Renal Crónico/cirugía , Trasplante de Riñón/métodos , Donadores Vivos , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Pediatric chronic kidney disease (CKD) is characterized by many co-morbidities, including impaired growth and development, CKD-mineral and bone disorder, anemia, dysregulated iron metabolism, and cardiovascular disease. In pediatric CKD cohorts, higher circulating concentrations of fibroblast growth factor 23 (FGF23) are associated with some of these adverse clinical outcomes, including CKD progression and left ventricular hypertrophy. It is hypothesized that lowering FGF23 levels will reduce the risk of these events and improve clinical outcomes. Reducing FGF23 levels in CKD may be accomplished by targeting two key stimuli of FGF23 production-dietary phosphate absorption and iron deficiency. Ferric citrate is approved for use as an enteral phosphate binder and iron replacement product in adults with CKD. Clinical trials in adult CKD cohorts have also demonstrated that ferric citrate decreases circulating FGF23 concentrations. This review outlines the possible deleterious effects of excess FGF23 in CKD, summarizes data from the adult CKD clinical trials of ferric citrate, and presents the Ferric Citrate and Chronic Kidney Disease in Children (FIT4KiD) study, a randomized, placebo-controlled trial to evaluate the effects of ferric citrate on FGF23 in pediatric patients with CKD stages 3-4 (ClinicalTrials.gov Identifier NCT04741646).
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Insuficiencia Renal Crónica , Niño , Compuestos Férricos , Factores de Crecimiento de Fibroblastos/metabolismo , Humanos , Hierro/uso terapéutico , Minerales , Fosfatos , Ensayos Clínicos Controlados Aleatorios como Asunto , Insuficiencia Renal Crónica/complicacionesRESUMEN
Pediatric kidney transplant recipients generally have good outcomes post-transplantation. However, the younger age and longer life span after transplantation in the pediatric population make understanding the multifactorial nature of long-term graft survival critical. This investigation analyzes factors associated with 10-year survival to identify areas for improvement in patient care. Kaplan-Meier with log-rank test and univariable and multivariable logistic regression methods were used to retrospectively analyze 7785 kidney transplant recipients under the age of 18 years from January 1, 1998, until March 9, 2008, using United Network for Organ Sharing (UNOS) data. Our end-point was death-censored 10-year graft survival after excluding recipients whose grafts failed within one year of transplant. Recipients aged 5-18 years had lower 10-year graft survival, which worsened as age increased: 5-9 years (OR: 0.66; CI: 0.52-0.83), 10-14 years (OR: 0.43; CI: 0.33-0.55), and 15-18 years (OR: 0.34; CI: 0.26-0.44). Recipient African American ethnicity (OR: 0.67; CI: 0.58-0.78) and Hispanic donor ethnicity (OR: 0.82; CI: 0.72-0.94) had worse outcomes than other donor and recipient ethnicities, as did patients on dialysis at the time of transplant (OR: 0.82; CI: 0.73-0.91). Recipient private insurance status (OR: 1.35; CI: 1.22-1.50) was protective for 10-year graft survival. By establishing the role of age, race, and insurance status on long-term graft survival, we hope to guide clinicians in identifying patients at high risk for graft failure. This study highlights the need for increased allocation of resources and medical care to reduce the disparity in outcomes for certain patient populations.
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Supervivencia de Injerto , Fallo Renal Crónico/cirugía , Trasplante de Riñón , Adolescente , Factores de Edad , Niño , Preescolar , Femenino , Estudios de Seguimiento , Disparidades en el Estado de Salud , Humanos , Lactante , Recién Nacido , Estimación de Kaplan-Meier , Modelos Logísticos , Masculino , Factores Protectores , Estudios Retrospectivos , Factores de Riesgo , Resultado del TratamientoRESUMEN
BACKGROUND: Therapeutic plasma exchange (TPE) is used in kidney diseases as an adjunct treatment. Little has been described as to its effectiveness in kidney disorders in children. This study aimed to assess the safety, efficacy, and outcomes of patients who underwent TPE for kidney indications. METHODS: Retrospective chart review of patients receiving TPE from 2010 to 2018 for kidney indications, such as antibody-mediated rejection, bone marrow transplant-associated thrombotic microangiopathy (TA-TMA), atypical hemolytic uremic syndrome, transplant recurrence of focal segmental glomerulosclerosis, and glomerulonephritis. Outcomes assessed were trends in kidney function, mortality, and progression to stage 5 chronic kidney disease (CKD 5). Significant hypocalcemia was defined as ionized calcium < 1 mmol/L. RESULTS: A total of 641 TPE procedures were performed on 47 patients (25 male). Average age was 12.8 ± 5.9 years. Median glomerular filtration rate (GFR) improved from baseline to end of TPE treatments (pre 44.9 (19.8, 79), end 56.1 (23, 98) [p = 0.02]). Ten out of 47 children developed CKD 5. Seven out of 47 patients died; 5 had TA-TMA. Initial 7 consecutive sessions were reviewed for complications. Among 335 procedures, 41 episodes of significant hypocalcemia were noted (12.2%); only 1 was symptomatic. Of the 26 episodes (7.7%) of allergic reactions, all were associated with the use of FFP; 5 were anaphylactic. No TPE-associated mortality was noted. CONCLUSIONS: TPE is a relatively well-tolerated useful adjunct therapy in children with kidney indications. The benefit of TPE has to be balanced with risks such as hypocalcemia and allergic reactions which can occur more frequently with FFP. Graphical abstract.
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Intercambio Plasmático , Adolescente , Niño , Femenino , Humanos , Hipersensibilidad , Hipocalcemia/etiología , Hipocalcemia/terapia , Riñón , Masculino , Insuficiencia Renal Crónica/terapia , Estudios Retrospectivos , Microangiopatías Trombóticas/etiología , Microangiopatías Trombóticas/terapiaRESUMEN
INTRODUCTION: Noninvasive hematocrit monitoring (NIVHM) during pediatric hemodialysis (pedHD) provides data in real time regarding changes in hematocrit and blood volume and also provides venous oxygen saturations. The latter has been proposed to indicate changes in tissue oxygen consumption. It is not known how well NIVHM oxygen saturations (O2sat) approximate blood gas measured oximetry saturation (mO2sat) in the course of pedHD. We aimed to assess the validity and reliability of NIVHM O2sat compared to mO2sat. METHODS: This is a prospective study in 15 patients <21 years old with >90 days on hemodialysis (HD) without congenital heart disease. HD access was fistula (AVF) in 4 patients and tunneled catheters in the remainder. Pulse oximetry (spO2) was continuously monitored; mO2sat was measured via oximetry in a blood gas analyzer and NIVHM O2sat values collected at the start, middle, and end of HD treatment. RESULTS: A total of 45 dyad measurements were obtained. NIVHM O2sat correlated well with mO2sat (R = 0.89, p < 0.0001); the same was seen at pre, mid, and post HD time points (R = 0.86-0.95, p < 0.001). NIVHM O2sat was lower than mO2sat; with catheter as access, the difference was 9.3 ± 8.6 (CI: 12.3-6.22, p < 0.0001) and with AVF was 2.1 ± 0.78 (CI: 2.6-1.7, p < 0.0001). Bland-Altman analysis demonstrated the difference but did not show any systematic bias. Continuous monitor of spO2 showed no hypoxia. DISCUSSION/CONCLUSION: Intradialytic NIVHM O2sat correlates well with mO2sat but yield lower values. Future studies can include NIVHM O2sat changes as a surrogate for central venous O2 saturation changes and potentially yield useful information regarding tissue oxygen consumption in pedHD patients.
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Sangre , Hematócrito , Oximetría , Oxígeno/sangre , Diálisis Renal , Venas , Adolescente , Análisis de los Gases de la Sangre , Niño , Femenino , Humanos , Masculino , Diálisis Renal/efectos adversos , Diálisis Renal/métodos , Insuficiencia Renal/sangre , Insuficiencia Renal/terapiaRESUMEN
BACKGROUND: Left ventricular hypertrophy (LVH) and arterial stiffness measured by pulse wave velocity (PWV) are independent predictors of cardiovascular (CV) mortality in adults receiving chronic dialysis. Hypertension strongly associates with elevated left ventricular mass index (LVMI) and PWV, with ambulatory blood pressure monitoring (ABPM), and central blood pressure (CBP) superior to office blood pressures (BP) in predicting CV morbidity. Few studies have described associations of office BP, ABPM, and CBP with LVMI and PWV in adolescent and young adult patients receiving hemodialysis (HD). METHODS: Cross-sectional study of 22 adolescents and young adults receiving chronic HD. Pre- and post-dialysis office BP and CBP using applanation tonometry were obtained. Twenty-four-hour ABPM was obtained midweek post-dialysis. Pre- and post-dialysis carotid-brachial PWV were obtained same day as BP measurements. Annual echocardiograms for standard care were reviewed for LVH. RESULTS: Pre-dialysis CBP index correlated with LVMI (r = 0.3, p = 0.04) and PWV (r = 0.48, p = 0.02). Hypertensive patients identified by ABPM had worse LVMI; daytime ABPM systolic BP index correlated with LVMI (r = 0.5, p = 0.02). Office BP was not associated with LVMI; only office diastolic BP was associated with PWV (r = 0.46, p = 0.02). There was no correlation of LVMI or PWV with bone health parameters, anemia, interdialytic weight gain, or residual renal function. CONCLUSIONS: Ambulatory blood pressure monitoring is superior to casual office BP obtained at time of dialysis in delineating cardiovascular morbidity in adolescent and young adult HD patients. CBP is easily performed and correlates with LVMI and PWV in adolescent and young adult HD patients; however, large-scale normative data is needed.
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Presión Arterial , Monitoreo Ambulatorio de la Presión Arterial , Hipertensión/diagnóstico , Hipertrofia Ventricular Izquierda/etiología , Fallo Renal Crónico/complicaciones , Rigidez Vascular , Adolescente , Adulto , Arterias/fisiopatología , Niño , Estudios Transversales , Femenino , Humanos , Hipertensión/etiología , Fallo Renal Crónico/terapia , Masculino , Análisis de la Onda del Pulso , Diálisis Renal , Adulto JovenRESUMEN
BACKGROUND: Management of chronic kidney disease mineral bone disorder (CKD-MBD) in pediatric patients with end-stage renal disease (ESRD) is challenging. While the use of calcimimetics is well-studied in adults on dialysis, few studies have been performed in pediatrics. Little is known about the use of cinacalcet in young children with ESRD. The aim of this study was to report the efficacy and safety of cinacalcet for treatment of secondary hyperparathyroidism in chronic dialysis patients younger than 5 years. MATERIALS AND METHODS: We included children aged < 5 years on chronic dialysis, either hemodialysis (HD) or peritoneal dialysis (PD), who were prescribed cinacalcet for more than 1 month. Retrospective chart review was performed to gather demographics, dialysis prescription, relevant mineral imbalance laboratory parameters, and medications. Data was collected for 6 consecutive months. RESULT: 18 patients (9 male), mean age at initiation of cinacalcet was 2.3 years; 13 PD and 5 HD. Average starting dose of cinacalcet: 6.2 mg daily, 0.55 mg/kg/day. Average time on dialysis was 14.4 months. Parathyroid hormone significantly decreased over the 1st month following initiation of cinacalcet from 929 (IQR 572 - 1,056) to 427 (IQR 256 - 778) pg/mL, p = 0.009. Three patients developed asymptomatic hypocalcemia (Ca < 9.4 mg/dL). Serum phosphorous decreased after initiation, and this was persistent at 6 months. Significant improvement in linear growth was observed while on cinacalcet and growth hormone (GH). CONCLUSION: Cinacalcet can be effectively used in young children on dialysis with minimal side effects. Good linear growth was seen in patients on cinacalcet and GH therapy. Long-term large scale data is necessary to confirm. Institution-based management algorithm incorporating cinacalcet would be helpful to maintain uniformity in role of cinacalcet for management of CKD-MBD.
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Calcimiméticos/uso terapéutico , Cinacalcet/uso terapéutico , Hiperparatiroidismo Secundario/tratamiento farmacológico , Fallo Renal Crónico/complicaciones , Preescolar , Femenino , Humanos , Lactante , Masculino , Diálisis Peritoneal , Diálisis Renal , Estudios RetrospectivosRESUMEN
ABO-ILT have re-emerged as an alternate option for select patients awaiting transplant. However, treatment protocols for children undergoing deceased donor ABO-ILT are not standardized. We implemented a novel IS protocol for children undergoing deceased donor ABO-ILT based on pretransplant IH titers. Children with high pretransplant IH titers (≥1:32) underwent an enhanced IS protocol including plasmapheresis, rituximab, IVIG, and mycophenolate, while children with IH titers ≤1:16 received steroids and tacrolimus. We retrospectively assessed our outcomes of ABO-ILT with ABO-compatible recipients of similar age and diagnosis over a 2-year period. Ten children with median age of 8.9 months underwent ABO-ILT, 4 of 10 patients underwent enhanced IS due to high IH titers. Rates of complications (rejection, infections, biliary, and vascular) at both 1 year and up to 3 years post-transplant were comparable between the groups. Patients with ABO-ILT had good graft function with 100% survival at a median follow-up of 3.3 years. In conclusion, IS tailored to pretransplant IH titers in pediatric deceased donor ABO-ILT is feasible and can achieve outcomes similar to ABO-CLT at 1 and 3 years post-transplantation.
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Sistema del Grupo Sanguíneo ABO , Incompatibilidad de Grupos Sanguíneos , Trasplante de Hígado/normas , Niño , Preescolar , Protocolos Clínicos , Quimioterapia Combinada , Femenino , Estudios de Seguimiento , Rechazo de Injerto/inmunología , Rechazo de Injerto/prevención & control , Humanos , Inmunosupresores/uso terapéutico , Lactante , Trasplante de Hígado/métodos , Trasplante de Hígado/mortalidad , Masculino , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/prevención & control , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
PURPOSE: To investigate the utility of whole-exome sequencing (WES) to define a molecular diagnosis for patients clinically diagnosed with congenital anomalies of kidney and urinary tract (CAKUT). METHODS: WES was performed in 62 families with CAKUT. WES data were analyzed for single-nucleotide variants (SNVs) in 35 known CAKUT genes, putatively deleterious sequence changes in new candidate genes, and potentially disease-associated copy-number variants (CNVs). RESULTS: In approximately 5% of families, pathogenic SNVs were identified in PAX2, HNF1B, and EYA1. Observed phenotypes in these families expand the current understanding about the role of these genes in CAKUT. Four pathogenic CNVs were also identified using two CNV detection tools. In addition, we found one deleterious de novo SNV in FOXP1 among the 62 families with CAKUT. The clinical database of the Baylor Miraca Genetics laboratory was queried and seven additional unrelated individuals with novel de novo SNVs in FOXP1 were identified. Six of these eight individuals with FOXP1 SNVs have syndromic urinary tract defects, implicating this gene in urinary tract development. CONCLUSION: We conclude that WES can be used to identify molecular etiology (SNVs, CNVs) in a subset of individuals with CAKUT. WES can also help identify novel CAKUT genes.Genet Med 19 4, 412-420.
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Variaciones en el Número de Copia de ADN , Secuenciación del Exoma/métodos , Predisposición Genética a la Enfermedad/genética , Anomalías Urogenitales/diagnóstico , Reflujo Vesicoureteral/diagnóstico , Adolescente , Niño , Preescolar , Femenino , Factores de Transcripción Forkhead/genética , Factor Nuclear 1-beta del Hepatocito/genética , Humanos , Lactante , Péptidos y Proteínas de Señalización Intracelular/genética , Masculino , Proteínas Nucleares/genética , Factor de Transcripción PAX2/genética , Linaje , Polimorfismo de Nucleótido Simple , Proteínas Tirosina Fosfatasas/genética , Proteínas Represoras/genética , Anomalías Urogenitales/genética , Reflujo Vesicoureteral/genética , Adulto JovenRESUMEN
OBJECTIVE: Protein energy wasting is difficult to assess in pediatric hemodialysis (HD) populations because of fluid shifts. Body composition (BC) analysis aids in monitoring nutrition therapy's effects on fat and lean mass. Air displacement plethysmography (ADP) is a "practical" gold standard for fat mass (FM) measurements. We aim to compare BC measures in a pediatric HD population via anthropometry, ADP, and bioelectrical impedance (BIA). DESIGN: The study was prospective cohort study and the setting was 1 pediatric HD unit in Houston, Texas. SUBJECTS: A total of 15 pediatric and young adult patients receiving maintenance HD. INTERVENTION: ADP, BIA, and anthropometry were obtained mid-week post HD for 3 consecutive months. MAIN OUTCOME MEASURE: The primary outcome was the difference in FM as defined by various assessment methods. Secondary outcomes included correlations with anthropometry. RESULTS: ADP demonstrated a strong positive correlation to body mass index, mid-upper arm circumference, and triceps skin fold (all P < .0001). No differences in FM assessment using ADP or BIA were found except in males with advanced tanner (P = .0004). BIA underestimated FM in obese subjects (P = .005). Analysis of malnutrition status by mid-upper arm circumference revealed an overestimation of FM in BIA measures (P = .02). CONCLUSIONS: This is the first pediatric dialysis study using ADP to assess BC. Estimation of FM in pediatric HD patients via ADP and BIA has significant error rates in 2 populations: (1) males tanner ≥4: BIA underestimates FM and (2) weight extremes: BIA underestimates FM for obese and overestimates FM for malnourished. BIA should be used with caution in these populations when estimating fluid content and leads to false estimates of dry weight. ADP is a useful adjunct in assessment of nutritional status via BC in pediatric HD patients.
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Composición Corporal , Impedancia Eléctrica , Pletismografía , Diálisis Renal/efectos adversos , Adolescente , Adulto , Índice de Masa Corporal , Niño , Femenino , Humanos , Masculino , Evaluación Nutricional , Estado Nutricional , Obesidad/etiología , Obesidad/terapia , Estudios Prospectivos , Desnutrición Proteico-Calórica/diagnóstico , Desnutrición Proteico-Calórica/etiología , Desnutrición Proteico-Calórica/terapia , Insuficiencia Renal Crónica/complicaciones , Insuficiencia Renal Crónica/terapia , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: Children with chronic kidney disease (CKD) may have impaired caloric intake through a variety of mechanisms, with decreased appetite as a putative contributor. In adult CKD, decreased appetite has been associated with poor clinical outcomes. There is limited information about this relationship in pediatric CKD. METHODS: A total of 879 participants of the Chronic Kidney Disease in Children (CKiD) study were studied. Self-reported appetite was assessed annually and categorized as very good, good, fair, or poor/very poor. The relationship between appetite and iohexol or estimated glomerular filtration rate (ieGFR), annual changes in anthropometrics z-scores, hospitalizations, emergency room visits, and quality of life were assessed. RESULTS: An ieGFR < 30 ml/min per 1.73 m(2) was associated with a 4.46 greater odds (95 % confidence interval: 2.80, 7.09) of having a worse appetite than those with ieGFR >90. Appetite did not predict changes in height, weight, or BMI z-scores. Patients not reporting a very good appetite had more hospitalizations over the next year than those with a very good appetite. Worse appetite was significantly associated with lower parental and patient reported quality of life. CONCLUSIONS: Self-reported appetite in children with CKD worsens with lower ieGFR and is correlated with clinical outcomes, including hospitalizations and quality of life.
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Apetito , Insuficiencia Renal Crónica/complicaciones , Adolescente , Niño , Estudios de Cohortes , Femenino , Tasa de Filtración Glomerular , Hospitalización/estadística & datos numéricos , Humanos , Masculino , Estudios Prospectivos , Calidad de VidaRESUMEN
BACKGROUND: Maintaining hemoglobin (Hgb) levels within a target range is difficult. Non-invasive hematocrit monitoring (NIVH) continuously monitors both the hematocrit and percent change in intravascular blood volume in real time. Based on the data reported here, NIVH can be utilized as a tool for anemia management in pediatric hemodialysis patients. METHODS: Monthly, mid-week pre-dialysis, Hgb levels were obtained for 12 consecutive months. Concurrent with monthly Hgb, hematocrit was recorded at the start of the dialysis treatment using NIVH. Hgb (oHgb) was calculated using the adult equation Hgb = 0.3112*HCT + 0.71, and a linear regression model was used to derive a pediatric specific equation (pHgb = 0.28*CRIT Hct + 2.5). RESULTS: A total of 310 observations were obtained from 47 patients. The mean actual hemoglobin (mHgb) was 11.14 ± 1.4, and the mean derived hemoglobin from the adult equation, oHgb, was 10.3 ± 1.3 (p = 0.0001). For the target hemoglobin of 10-12 gm/dl, the adult equation was 72 % sensitive and 63 % specific, whereas the pediatric equation was 93 % sensitive and 70 % specific. CONCLUSIONS: The newly derived pediatric equation (pHgb = 0.28*CRIT Hct + 2.5) improved the prediction capability compared to the standard equation with lower false-negative and false-positive rates.
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Hematócrito/métodos , Hemoglobinas/análisis , Diálisis Renal , Adolescente , Anemia/prevención & control , Niño , Preescolar , Femenino , Humanos , Lactante , Fallo Renal Crónico/terapia , Masculino , Estudios Retrospectivos , Adulto JovenAsunto(s)
Raquitismo Hipofosfatémico Familiar/complicaciones , Genu Valgum/etiología , Hipercalciuria/complicaciones , Niño , Análisis Mutacional de ADN , Diagnóstico Diferencial , Raquitismo Hipofosfatémico Familiar/diagnóstico , Raquitismo Hipofosfatémico Familiar/genética , Raquitismo Hipofosfatémico Familiar/terapia , Predisposición Genética a la Enfermedad , Genu Valgum/diagnóstico , Genu Valgum/fisiopatología , Genu Valgum/terapia , Humanos , Hipercalciuria/diagnóstico , Hipercalciuria/genética , Hipercalciuria/terapia , Masculino , Mutación , Valor Predictivo de las Pruebas , Pronóstico , Proteínas Cotransportadoras de Sodio-Fosfato de Tipo IIc/genéticaRESUMEN
BACKGROUND: Coronary calcifications (CC) portend increased mortality in adults receiving hemodialysis (HD), however the risk factors associated with CC progression are not well known in pediatric patients. Our previous cross-sectional studies demonstrated high CC prevalence (31 %) in pediatric patients, which were significantly associated with high serum phosphorus (P), fibroblast growth factor 23 (FGF) levels, dialysis vintage, and low cholesterol. The current study was undertaken to determine and elucidate CC progression in pediatric HD patients. METHODS: A 1-year prospective longitudinal study of 16 pediatric patients (ten male; mean age, 16.9 ± 3 years; range, 10.1-20.4 years) receiving chronic HD was conducted. RESULTS: CC were observed in five of 16 (31.3 %) patients on baseline computed tomogram (CT) scan; 14/16 patients underwent 1-year CT. All patients with initial CC who completed CT at 1 year (3/5) progressed; one patient had new CC and none of the patients had resolved CC. Mean Agatston score increased from 23.4 ± 18.06 HU (baseline) to 169 ± 298.9 HU. Patients with CC progression had higher mean serum P (8.6 ± 1.8 mg/dl vs. 6.3 ± 1.1 mg/dl, p = 0.015) and FGF 23 levels (3,994 ± 860.5 pg/ml vs. 2,327 ± 1,206.4 pg/ml, p = 0.028). Serum P and FGF 23 levels were positively correlated with final Agatston scores (R = 0.65, p = 0.01 for serum P and R = 0.54, p = 0.045 for FGF 23) and change in Agatston scores (R = 0.65, p = 0.01 for serum P and R = 0.52, p = 0.048 for FGF 23). CONCLUSIONS: Our study shows that CC is progressive in pediatric patients receiving HD and that increased serum P and FGF 23 levels are associated with this progression.
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Calcinosis/diagnóstico por imagen , Cardiomiopatías/diagnóstico por imagen , Enfermedad Coronaria/diagnóstico por imagen , Factores de Crecimiento de Fibroblastos/sangre , Fósforo/sangre , Diálisis Renal/efectos adversos , Adolescente , Calcinosis/sangre , Calcinosis/etiología , Cardiomiopatías/sangre , Cardiomiopatías/etiología , Niño , Progresión de la Enfermedad , Ensayo de Inmunoadsorción Enzimática , Femenino , Factor-23 de Crecimiento de Fibroblastos , Humanos , Estudios Longitudinales , Masculino , Prevalencia , Tomografía Computarizada por Rayos X , Adulto JovenRESUMEN
BACKGROUND: Normalized protein catabolic rate (nPCR) calculation depends on estimating the urea generation between consecutive hemodialysis (HD) treatments. Two-point nPCR using blood urea nitrogen (BUN) before and after the same HD treatment has not been validated in pediatric patients, who typically receive a more intense HD dose than adults. This study aimed to compare nPCR calculated with a two-point vs. a three-point nPCR model in pediatric HD patients. METHODS: Pediatric patients receiving HD at 2 units were enrolled. Three BUN measurements were obtained around a midweek HD treatment: one prior to HD (preBUN1), one 30 s after HD (30sBUN), and one prior to the subsequent HD (preBUN2). The two-point nPCR model was calculated using preBUN1 and 30sBUN and the three-point nPCR model was calculated using preBUN2 and 30sBUN. RESULTS: Seventy-six BUN sets from 35 patients were analyzed. Mean age was 16.4 ± 3.5 years. Mean dry weight was 51.4 ± 17.1 kg. Mean spKt/V was 1.54 ± 0.23. Mean preBUN2 was significantly lower than mean preBUN1 (60.2 ± 18.6 vs. 64.0 ± 18.9 mg/dl, p = 0.0001). nPCR obtained from the three-point model was significantly lower than nPCR obtained from the two-point model (1.07 ± 0.31 vs. 1.17 ± 0.31 g/kg/day, p = 0.00001). Seven of 76 (9.2 %) paired comparisons yielded three-point nPCR <1 vs. two-point nPCR >1. CONCLUSIONS: Our data show that in pediatric patients receiving HD, the ((1) two-point and three-point models lead to significantly different nPCRs, and (2) inaccurate protein intake assessment may result from reliance on a two-point model for nPCR estimates.
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Nitrógeno de la Urea Sanguínea , Proteínas en la Dieta/metabolismo , Modelos Biológicos , Evaluación Nutricional , Estado Nutricional , Desnutrición Proteico-Calórica/diagnóstico , Diálisis Renal , Adolescente , Biomarcadores/sangre , California , Humanos , Valor Predictivo de las Pruebas , Desnutrición Proteico-Calórica/sangre , Desnutrición Proteico-Calórica/etiología , Desnutrición Proteico-Calórica/fisiopatología , Análisis de Regresión , Diálisis Renal/efectos adversos , Reproducibilidad de los Resultados , Texas , Factores de Tiempo , Resultado del Tratamiento , Adulto JovenRESUMEN
Approximately 30% of all children and neonates admitted to the intensive care unit (ICU) experience acute kidney injury (AKI). Children with AKI are largely poorly fed and experience high rates of malnutrition. Nutrition prescription and provision are exceptionally challenging for critically ill neonates, infants, and children with AKI given the dynamic nature of AKI and its respective treatment modalities. Managing the nutrition prescription of critically ill neonates, infants, and children with AKI requires nutrition support clinicians to have a high-level understanding of the various treatment modalities for AKI, which can affect the patient's protein, fluid, electrolyte, and mineral needs. Accurate and timely nutrition assessment in critically ill neonates and children with AKI can be flawed owing to difficulty obtaining accurate anthropometric parameters. Recently, the Pediatric Renal Nutrition Taskforce introduced clinical practice recommendations for the nutrition management of children with AKI. In this review, we will discuss the practical implications of these recent guidelines and work to bridge the knowledge and practice gaps for pediatric and neonatal nutrition support clinicians providing nutrition therapy for patients with AKI in the ICU. We also appraise special nutrition-related considerations for neonates with AKI given newer available renal replacement treatment modalities.
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Lesión Renal Aguda , Diálisis Renal , Lactante , Recién Nacido , Humanos , Niño , Enfermedad Crítica/terapia , Estado Nutricional , Riñón , Lesión Renal Aguda/terapiaRESUMEN
Increased mortality of adult chronic hemodialysis (HD) patients is associated with coronary calcifications (CC), increased serum phosphorus (P), use of calcium (Ca)-containing P-binders, and vitamin D deficiency. Serum concentration of fibroblast growth factor 23 (FGF 23) is markedly elevated in adults receiving dialysis and is independently associated with increased mortality. Although coronary calcifications have been described in pediatric and adult HD patients, no significant association between serum FGF 23 and CC has been reported. In our study, 5/16 patients had CC. Patients with CC were older, had longer dialysis vintage and higher serum P. Serum Ca, total PTH, elemental Ca intake, and calcitriol doses were not different for CC patients. Serum FGF 23 levels were markedly elevated in all patients (mean 4,024, range 874-8,253), but significantly higher in patients with CC (4,247 ± 10,35 vs 2,427 ± 11,92, p = 0.01) and positively correlated with Agatston calcification score (r = 0.69, p = 0.003) and serum P (r = 0.49, p = 0.05). Using multivariate analysis, serum FGF 23 and serum P remained the most significant factors associated with Agatston score. This study confirms the occurrence of CC in pediatric HD patients and is the first to show a significant association between CC and elevated serum FGF 23 in children.