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BACKGROUND: The use of small pediatric kidneys as single grafts for transplantation is controversial, due to the potential risk for graft thrombosis and insufficient nephron mass. METHODS: Aiming to test the benefits of transplanting these kidneys, 375 children who underwent kidney transplantation in a single center were evaluated: 49 (13.1%) received a single graft from a small pediatric donor (≤ 15 kg, SPD group), 244 (65.1%) from a bigger pediatric donor (> 15 kg, BPD group), and 82 (21.9%) from adult living donors (group ALD). RESULTS: Groups had similar baseline main characteristics. After 5 years of follow-up, children from the SPD group were comparable to children from BPD and ALD in patient survival (94%, 96%, and 98%, respectively, p = 0.423); graft survival (89%, 88%, and 93%, respectively, p = 0.426); the frequency of acute rejection (p = 0.998); the incidence of post-transplant lymphoproliferative disease (p = 0.671); the odds ratio for severely increased proteinuria (p = 0.357); the rates of vascular thrombosis (p = 0.846); and the necessity for post-transplant surgical intervention prior to discharge (p = 0.905). The longitudinal evolution of eGFR was not uniform among groups. The three groups presented a decrease in eGFR, but the slope of the curve was steeper in ALD children. At 5 years, the eGFR of the ALD group was 10 ml/min/1.73m2 inferior to the others. At that time, the eGFR from the SPD group was statistically similar to the BPD group (p = 0.952). CONCLUSION: In a specialized transplant center, the use of a single small pediatric donor kidney for transplantation is as successful as bigger pediatric or adult living donors, after 5 years of follow-up. A higher resolution version of the Graphical abstract is available as Supplementary information.
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Trasplante de Riñón , Trombosis , Adulto , Niño , Rechazo de Injerto/epidemiología , Rechazo de Injerto/etiología , Supervivencia de Injerto , Humanos , Riñón , Trasplante de Riñón/efectos adversos , Donadores Vivos , Estudios Retrospectivos , Trombosis/epidemiología , Trombosis/etiología , Donantes de TejidosRESUMEN
BACKGROUND: APOL1 high-risk genotypes (HRG) are associated with increased risk of kidney disease in individuals of African ancestry. We analyzed the effects of APOL1 risk variants on an ethnically diverse Brazilian pediatric nephrotic syndrome (NS) cohort. METHODS: Multicenter study including 318 NS patients, categorized as progressors to advanced CKD [estimated glomerular filtration rate (eGFR)] < 30 mL/min/1.73 m2] and slow/non-progressors (eGFR > 30 mL/min/1.73 m2 through the study). We employed Cox regression with progression time as the outcome and APOL1 genotype as the independent variable. We tested this association in the entire cohort and three subgroups; (1) focal segmental glomerulosclerosis (FSGS), (2) steroid-resistant NS (SRNS), and (3) those who underwent kidney biopsy. RESULTS: Nineteen patients (6%) had an HRG. Of these, 47% were self-reported White. Patients with HRG manifested NS at older ages and presented higher frequencies of FSGS and SRNS. HRG patients progressed to advanced CKD more often than low-risk-genotype (LRG) children in the whole NS cohort (p = 0.001) and the three subgroups. In SRNS and biopsied patients, a single risk variant was associated with trends of higher CKD progression risk. CONCLUSIONS: Novel discoveries include a substantial prevalence of HRG among patients self-reported White, worse kidney outcomes in HRG versus LRG children in the FSGS subgroup, and a trend of higher CKD progression risk associated with a single risk variant in the SRNS cohort. These findings suggest APOL1-associated NS extends beyond patients self-reported non-White, the HRG effect is independent of FSGS, and a single risk variant may have a detrimental impact in children with NS.
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Glomeruloesclerosis Focal y Segmentaria , Síndrome Nefrótico , Insuficiencia Renal Crónica , Apolipoproteína L1/genética , Niño , Receptores ErbB , Glomeruloesclerosis Focal y Segmentaria/diagnóstico , Glomeruloesclerosis Focal y Segmentaria/genética , Humanos , Síndrome Nefrótico/genéticaRESUMEN
BACKGROUND: Proper care of young children in need of kidney transplant (KT) requires many skilled professionals and an expensive hospital structure. Small children have lesser access to KT. METHODS: We describe a strategy performed in Brazil to enable and accelerate KT in children ≤15 kg based on the establishment of one specialized transplant center, focused on small children, and cooperating with distant centers throughout the country. Actions on 3 fronts were implemented: (a) providing excellent medical assistance, (b) coordinating educational activities to disseminate expertise and establish a professional network, and (c) fostering research to promote scientific knowledge. We presented the number and outcomes of small children KT as a result of this strategy. RESULTS: Three hundred forty-six pediatric KTs were performed in the specialized center from 2009 to 2017, being 130 in children ≤15 kg (38%, being 41 children ≤10 kg) and 216 in >15 kg (62%). Patient survival after 1 and 5 years of the transplant was 97% and 95% in the "small children" group, whereas, in the "heavier children" group, it was 99% and 96% (P = 0.923). Regarding graft survival, we observed in the "small children" group, 91% and 87%, whereas in the "heavier children" group, 94% and 87% (P = 0.873). These results are comparable to the literature data. Groups were similar in the incidence of reoperation, vascular thrombosis, posttransplant lymphoproliferative disease, and estimated glomerular filtration rate. CONCLUSIONS: The strategy allowed an improvement in the number of KT in small children with excellent results. We believe this experience may be useful in other locations.
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Rechazo de Injerto/epidemiología , Hospitales Pediátricos/organización & administración , Fallo Renal Crónico/cirugía , Trasplante de Riñón/estadística & datos numéricos , Tiempo de Tratamiento/organización & administración , Adolescente , Peso Corporal/fisiología , Brasil/epidemiología , Niño , Preescolar , Femenino , Estudios de Seguimiento , Tasa de Filtración Glomerular/fisiología , Rechazo de Injerto/etiología , Rechazo de Injerto/fisiopatología , Supervivencia de Injerto/fisiología , Implementación de Plan de Salud , Accesibilidad a los Servicios de Salud/organización & administración , Accesibilidad a los Servicios de Salud/estadística & datos numéricos , Hospitales Pediátricos/estadística & datos numéricos , Humanos , Incidencia , Fallo Renal Crónico/mortalidad , Fallo Renal Crónico/fisiopatología , Trasplante de Riñón/efectos adversos , Masculino , Evaluación de Programas y Proyectos de Salud , Análisis de Supervivencia , Factores de Tiempo , Resultado del TratamientoRESUMEN
BACKGROUND: Small children are less frequently transplanted when compared with older. The objective of the present study was to compare the preparation time for transplantation in children of different weights and to identify factors associated with a delay in the workup of small children. METHODS: We report on a retrospective cohort comprising all children referred for renal transplantation (RTx) workup between 2009 and 2017. The main outcome was transplantation workup time, defined as the time elapsed between the first consultation and when the child became ready for the surgery. RESULTS: A total of 389 children (63.5% males) were selected, with a median weight of 18 kg (interquartile range, 11-32). Patients were categorized into 2 groups: group A (study group): ≤15 kg (n = 165) and group B (control group): >15 kg (n = 224). The probability of being ready for RTx was comparable between groups A and B. The cumulative incidence rate difference between groups is -0.05 (95% confidence interval, -0.03 to 0.02). The median time for RTx workup was 5.4 (2.4-9.4) in group A and 4.3 (2.2-9.0) months in group B (P = 0.451). Moreover, the presence of urinary tract malformation was associated with the need for longer transplantation workup time (P < 0.001). CONCLUSIONS: In children >7 kg, the workup time for transplantation is not related to body weight. In a specialized center, children weighing 7-15 kg became ready within the same timeframe as children weighing >15 kg, despite the smaller children had greater difficulty being nourished, dialyzed, and a greater need for surgical correction of the urinary tract pretransplant.
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Peso Corporal , Fallo Renal Crónico/terapia , Trasplante de Riñón/normas , Cuidados Preoperatorios/normas , Tiempo de Tratamiento , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Diálisis Renal/normas , Estudios Retrospectivos , Factores de Tiempo , Adulto JovenRESUMEN
The Brazilian collaborative registry for pediatric renal transplantation began in 2004 as a multicenter initiative aimed at analyzing, reporting, and disseminating the results of pediatric renal transplantation in Brazil. Data from all pediatric renal transplants performed from January 2004 to May 2018 at the 13 participating centers were analyzed. A total of 2744 pediatric renal transplants were performed in the thirteen participating centers. The median age at transplantation was 12.2 years, with the majority being male recipients (56%). The main underlying diseases were CAKUT (40.5%) and glomerulopathy (28%). 1981 (72%) of the grafts were from deceased donors (DD). Graft survival at one year (censored by death) was 94% in the live donor group (LD) and 91% in the DD group (log-rank test P < 0.01). The patient's survival at one and 5 years was 97% and 95% for the LD group and 96% and 93% for the DD group (log-rank test P = 0.02). The graft loss rate was 19% (n = 517), more frequently caused by vascular thrombosis (n = 102) and chronic graft nephropathy (n = 90). DD recipients had 1.6 (1.0-2.2) times greater chance of death and 1.5 (1.2-1.8) times greater chance of graft loss compared to LD recipients. The mortality rate was 5.4% (n = 148), mainly due to infection (n = 69) and cardiovascular disease (n = 28). The results of this collaborative pediatric renal transplant record are comparable to other international registries, although we still have a high infection rate as a cause of death.
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Supervivencia de Injerto , Enfermedades Renales/cirugía , Trasplante de Riñón , Sistema de Registros , Adolescente , Brasil , Niño , Ciclosporina/farmacología , Femenino , Estudios de Seguimiento , Rechazo de Injerto , Humanos , Cooperación Internacional , Enfermedades Renales/complicaciones , Fallo Renal Crónico , Donadores Vivos , Masculino , Complicaciones Posoperatorias/mortalidad , Trombosis/fisiopatología , Obtención de Tejidos y ÓrganosRESUMEN
Nephrotic syndrome (NS) is one of the most challenging conditions to manage and treat, partly because we lack a specific molecular understanding of its pathogenesis and progression. This limits our ability to provide targeted therapy or precise prognostications. Fortunately, genomic discovery in NS and its translation to genomic-informed medicine is allowing us to improve our understanding of the molecular anatomy of NS and our ability to care for patients with NS. In this Core Curriculum, we review the specific genes and loci discovered in childhood NS, specifically NS of Mendelian origin, APOL1-associated NS in black patients, HLA region variants associated with steroid-sensitive NS, their biological impacts, prevalence across populations, and clinical correlates. We also review the fundamentals of genetic architecture of human disease, technologies, and analytic strategies that currently exist to discover disease-related genetic variations. A facility with the concepts and vocabulary of modern genomics and ability to interpret results of genetic studies are essential skills for nephrologists caring for children with NS. As such, we hope to empower them to understand the literature in this area, appropriately order genetic tests and accurately interpret the results, and consider how they may participate in or drive the next wave of genomic discoveries in NS.
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Pruebas Genéticas/métodos , Glomeruloesclerosis Focal y Segmentaria/diagnóstico , Glomeruloesclerosis Focal y Segmentaria/genética , Síndrome Nefrótico/diagnóstico , Síndrome Nefrótico/genética , Niño , Diagnóstico Diferencial , Pruebas Genéticas/tendencias , HumanosRESUMEN
Abstract Objective: End-stage renal disease is a health problem that consumes public and private resources. This study aimed to identify the cost of hemodialysis (either daily or conventional hemodialysis) and transplantation in children and adolescents. Methods: This was a retrospective cohort of pediatric patients with End-stage renal disease who underwent hemodialysis followed by kidney transplant. All costs incurred in the treatment were collected and the monthly total cost was calculated per patient and for each renal therapy. Subsequently, a dynamic panel data model was estimated. Results: The study included 30 children who underwent hemodialysis (16 conventional/14 daily hemodialysis) followed by renal transplantation. The mean monthly outlay for hemodialysis was USD 3500 and USD 1900 for transplant. Hemodialysis costs added up to over USD 87,000 in 40 months for conventional dialysis patients and USD 131,000 in 50 months for daily dialysis patients. In turn, transplant costs in 50 months reached USD 48,000 and USD 70,000, for conventional and daily dialysis patients, respectively. For conventional dialysis patients, transplant is less costly when therapy exceeds 16 months, whereas for daily dialysis patients, the threshold is around 13 months. Conclusion: Transplantation is less expensive than dialysis in children, and the estimated thresholds indicate that renal transplant should be the preferred treatment for pediatric patients.
Resumo Objetivo: A Doença Renal em Estágio Final é um problema de saúde que consome recursos públicos e privados. Nosso objetivo é identificar o custo da hemodiálise (hemodiálise diarias ou convencional) e transplante em crianças e adolescentes. Métodos: Uma coorte retrospectiva de pacientes pediátricos com Doença Renal em Estágio Final (DREF) submetidos à hemodiálise após transplante de rim. Todos os custos incorridos no tratamento foram cobrados e o custo total mensal foi calculado por paciente e por cada terapia renal. Então, foi estimado um modelo dinâmico com dados em painel. Resultados: Estudamos 30 crianças submetidas à hemodiálise (16 hemodiálises convencionais/14 hemodiálises diárias) após transplante renal. O gasto médio mensal para hemodiálise foi US$3,5 mil e US$1,9 mil para transplante. Os custos de hemodiálise somam mais de US$87 mil em 40 meses para pacientes submetidos a hemodiálise convencional (HC) e US$131 mil em 50 meses para pacientes submetidos a hemodiálise diária (HD). Por outro lado, os custos de transplante em 50 meses atingem US$48 e US$70 mil, para pacientes submetidos a HC e HD, respectivamente. Para pacientes submetidos à hemodiálise convencional, o transplante é menos oneroso quando a terapia ultrapassa 16 meses, ao passo que para pacientes submetidos a hemodiálise diária o limiar é cerca de 13 meses. Conclusão: O transplante é menos caro que a diálise em crianças e os limiares estimados indicam que o transplante renal deve ser o tratamento preferencial para pacientes pediátricos.
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Humanos , Masculino , Femenino , Preescolar , Niño , Diálisis Renal/economía , Trasplante de Riñón/economía , Fallo Renal Crónico/economía , Brasil , Estudios Retrospectivos , Estudios de Cohortes , Costos y Análisis de Costo , Fallo Renal Crónico/cirugía , Fallo Renal Crónico/terapiaRESUMEN
OBJECTIVE: End-stage renal disease is a health problem that consumes public and private resources. This study aimed to identify the cost of hemodialysis (either daily or conventional hemodialysis) and transplantation in children and adolescents. METHODS: This was a retrospective cohort of pediatric patients with End-stage renal disease who underwent hemodialysis followed by kidney transplant. All costs incurred in the treatment were collected and the monthly total cost was calculated per patient and for each renal therapy. Subsequently, a dynamic panel data model was estimated. RESULTS: The study included 30 children who underwent hemodialysis (16 conventional/14 daily hemodialysis) followed by renal transplantation. The mean monthly outlay for hemodialysis was USD 3500 and USD 1900 for transplant. Hemodialysis costs added up to over USD 87,000 in 40 months for conventional dialysis patients and USD 131,000 in 50 months for daily dialysis patients. In turn, transplant costs in 50 months reached USD 48,000 and USD 70,000, for conventional and daily dialysis patients, respectively. For conventional dialysis patients, transplant is less costly when therapy exceeds 16 months, whereas for daily dialysis patients, the threshold is around 13 months. CONCLUSION: Transplantation is less expensive than dialysis in children, and the estimated thresholds indicate that renal transplant should be the preferred treatment for pediatric patients.
Asunto(s)
Fallo Renal Crónico/economía , Trasplante de Riñón/economía , Diálisis Renal/economía , Brasil , Niño , Preescolar , Estudios de Cohortes , Costos y Análisis de Costo , Femenino , Humanos , Fallo Renal Crónico/cirugía , Fallo Renal Crónico/terapia , Masculino , Estudios RetrospectivosRESUMEN
BACKGROUND: The aims of this study were to identify the genetic mutations profile in Brazilian children with nephrotic syndrome (NS) and to determine a genotype-phenotype correlation in this disease. METHODS: Next-generation sequencing and mutation analysis were performed on 24 genes related to NS in a cross-sectional study involving 95 children who underwent kidney transplantation due to NS, excluding congenital cases. RESULTS: A total of 149 variants were identified in 22 of 24 sequenced genes. The mutations were classified as pathogenic, likely pathogenic, likely benign and benign per the chance of causing the disease. NPHS2 was the most common mutated gene. We identified 8 (8.4%) patients with hereditary NS and 5 (5%) patients with probably genetically caused NS. COL4A3-5 variants were found as well, but it is not clear whether they should be considered isolated FSGS or simply a misdiagnosed type of the Alport spectrum. Considering the clinical results, hereditary NS patients presented a tendency to early disease onset when compared with the other groups (P = 0.06) and time to end stage renal disease (ESRD) was longer in this group (P = 0.03). No patients from hereditary NS group had NS recurrence after transplantation. CONCLUSIONS: This is the first study in children with steroid-resistant NS who underwent kidney transplantation using next-generation sequencing. Considering our results, we believe this study has shed some light to the uncertainties of genotype-phenotype correlation in NS, where several genes cooperate to produce or even to modify the course of the disease.
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Trasplante de Riñón , Síndrome Nefrótico/genética , Síndrome Nefrótico/cirugía , Adolescente , Brasil , Niño , Preescolar , Biología Computacional , Estudios Transversales , Análisis Mutacional de ADN , Femenino , Estudios de Asociación Genética , Genotipo , Secuenciación de Nucleótidos de Alto Rendimiento , Humanos , Lactante , Masculino , MutaciónRESUMEN
Endocan is an important biomarker of inflammation and endothelial dysfunction that increases in association with several chronic diseases. Few published data have described the role of endocan in pediatric renal transplant (RT) patients. We evaluated the endocan concentrations in 62 children who underwent renal transplantation and assessed their relationships with the patients' blood pressure and loss of renal function. The endocan levels were significantly elevated in the pediatric RT patients who had hypertension and a loss of renal function. We determined positive correlations between the endocan concentrations and the hemodynamic variables (systolic blood pressure: r = 0.416; P = 0.001; pulse pressure: r = 0.412; P = 0.003). The endocan levels were inversely correlated with the estimated glomerular filtration rate (r = -0.388; P = 0.003). An endocan cutoff concentration of 7.0 ng/mL identified pediatric RT patients who had hypertension and a loss of renal function with 100% sensitivity and 75% specificity. In conclusion, the endocan concentrations were significantly elevated in pediatric RT patients who had both hypertension and a loss of renal function. The correlations between the endocan levels and the hemodynamic variables and the markers of renal function strengthen the hypothesis that it is an important marker of cardiorenal risk.
RESUMEN
BACKGROUND: The aim of this study was to describe the access and factors associated with kidney transplantation for children in different regions of Brazil. METHODS: We analyzed a cohort of 1211 children enrolled on the transplant list from January 2011 to December of 2013. We fitted regression models to investigate factors associated with: (a) undergoing kidney transplantation from a deceased donor, and (b) being removed from the waiting list. RESULTS: The incidence of transplantation was uneven across regions, with the lowest rate at 0.4 per million age-related population (pmarp) in the Midwest and the highest incidence rate of 8.3 cases pmarp in the South. Children from the North and the Midwest regions had a 3-4 times lower probability of undergoing a deceased donor transplant (p < 0.05). Apart from the geographic region, age of recipients and GDP influenced the outcome. The likelihood of undergoing transplantation was very low in the youngest children in the North and Midwest. The number of transplant centers was not associated with either outcome. CONCLUSIONS: Factors of inequality in transplantation in Brazil are of macroeconomic origin, but there is room to reduce inequalities. Training existing transplant center professionals in the care of children could diminish the discrepancies.
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Accesibilidad a los Servicios de Salud , Disparidades en Atención de Salud , Enfermedades Renales/cirugía , Trasplante de Riñón , Evaluación de Procesos, Atención de Salud , Adolescente , Factores de Edad , Brasil/epidemiología , Niño , Preescolar , Femenino , Necesidades y Demandas de Servicios de Salud , Humanos , Enfermedades Renales/diagnóstico , Enfermedades Renales/epidemiología , Masculino , Evaluación de Necesidades , Características de la Residencia , Factores Socioeconómicos , Donantes de Tejidos/provisión & distribución , Resultado del Tratamiento , Listas de EsperaRESUMEN
INTRODUCTION: There are few reports in the literature estimating the epidemiologic characteristics of pediatric chronic dialysis. These patients have impaired physical growth, high number of comorbidities and great need for continuous attention of specialized services with high demand for complex and costly procedures. OBJECTIVE: The aim of this study was to estimate the incidence and prevalence rates and describe the characteristics of children and adolescents undergoing chronic dialysis treatment in a Brazilian demographic health survey. MATERIALS AND METHODS: A cross-sectional study was performed in a representative sample of dialysis centers (nc = 239) that was established from the 2011 Brazilian Nephrology Society Census (Nc = 708). We collected data encompassing the five Brazilian macro-regions. We analyzed the data from all patients under 19 years of age. The sample population consisted of 643 children and adolescents who were on chronic dialysis program anytime in 2012. Data collection was carried out in the dialysis services by means of patients' records reviews and personal interviews with the centers' leaders. RESULTS: We estimated that there were a total of 1,283 pediatric patients on chronic dialysis treatment in Brazil, resulting in a prevalence of 20.0 cases per million age-related population (pmarp) (95% CI: 14.8-25.3) and an incidence of 6.6 cases pmarp in 2012 (95% CI: 4.8-8.4). The South region had the highest prevalence and incidence rates of patients under dialysis therapy, 27.7 (95% CI: 7.3-48.1) and 11.0 (95% CI: 2.8-19.3) cases pmarp, respectively; the lowest prevalence and incidence rates were found in the North-Midwest region, 13.8 (95% CI: 6.2-21.4), and in the Northeast region, 3.8 (95% CI: 1.4-6.3) cases pmarp, respectively. CONCLUSION: Brazil has an overall low prevalence of children on chronic dialysis treatment, figuring near the rates from others countries with same socioeconomic profile. There are substantial differences among regions related to pediatric chronic dialysis treatment. Joint strategies aiming to reduce inequities and improving access to treatment and adequacy of services across the Brazilian regions are necessary to provide an appropriate care setting for this population group.
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Fallo Renal Crónico/epidemiología , Fallo Renal Crónico/terapia , Diálisis Renal/estadística & datos numéricos , Adolescente , Brasil/epidemiología , Niño , Estudios Transversales , Femenino , Encuestas Epidemiológicas , Humanos , Incidencia , Fallo Renal Crónico/diagnóstico , Masculino , Prevalencia , Factores SocioeconómicosRESUMEN
The technical aspects of RT in low-weight children should be specific, particularly with regard to VA. This retrospective study assesses the main VA options in paediatric RTs and proposes a new strategy for renal artery trajectory when using the Ao and the right iVC. The sample included 81 patients and was categorized into a group of children weighing <16 kg and the other group of children weighing 16 kg or more. The smaller children received the graft predominantly on the Ao and iVC (63%); however, the VA options varied in children weighing more than 16 kg, with anastomoses predominantly to the common iliac vessels (46%). In the first group, when the Ao was the selected vessel for anastomosis on the right side, the trajectory adopted for the transplanted kidney artery was posterior to the iVC. This strategy may reduce the risk of compression of the iVC by the renal artery of the donor kidney and may reconstitute the normal anatomy of the renal artery. Moreover, it did not represent a risk factor for graft loss in this sample.
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Peso Corporal , Arteria Ilíaca/cirugía , Fallo Renal Crónico/cirugía , Trasplante de Riñón/métodos , Arteria Renal/cirugía , Vena Cava Inferior/cirugía , Adolescente , Anastomosis Quirúrgica , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVE: To estimate the prevalence of pediatric end-stage renal disease and evaluate demographics and renal disease characteristics in state of São Paulo over the year 2008. METHODS: Observational, descriptive, and cross-sectional study based on a population sample with subjects < 18 years. The data collecting assumed three forms: 1. A questionnaire for dialysis units; 2. Search in the Transplant Center to determine the number and characteristics of patients who had been in a transplant waiting list over the study period; 3. Search in the database of patients registered at the Latin American Collaborative Registry of Pediatric Kidney Transplantation. RESULTS: Data from 301 patients aged 9.0 ± 5.8, including 140 girls (46.5%), resulting in an estimate prevalence of 23.4 cases per million age-related population (pmarp). The age group most frequently found was 10 to 15 years (32.2%), and urinary tract malformation was the most usual known etiology (24.9%). Most children underwent kidney transplantation (62.1%) and among subjects on dialysis, hemodialysis was predominant (71.2%). The Sistema Único de Saúde - Unified National Health System - (SUS) provided the financial support for treatments. CONCLUSION: The prevalence of 23.4 cases pmarp found by the authors is lower than that reported in Western world. We believe data were underestimated in the present study, as few dialysis units returned the completed questionnaire. This potential bias does not invalidate the exploratory character of results. Further mechanisms for retrospective and earlier data collecting on pediatric chronic renal disease (CRD) are needed so that the burden of this serious health condition can be appropriately sized up.
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Fallo Renal Crónico/epidemiología , Adolescente , Distribución por Edad , Brasil/epidemiología , Niño , Preescolar , Estudios Transversales , Femenino , Humanos , Lactante , Recién Nacido , Trasplante de Riñón/estadística & datos numéricos , Masculino , Prevalencia , Diálisis Renal/estadística & datos numéricos , Distribución por SexoRESUMEN
OBJETIVO: Estimar a prevalência da doença renal crônica terminal (DRCT) pediátrica e avaliar os dados demográficos e as características nefrológicas dessa doença no Estado de São Paulo no ano de 2008. MÉTODOS: Estudo observacional, descritivo e transversal com base em amostra da população de indivíduos < 18 anos. A coleta de casos ocorreu de três formas: 1. Questionário para as unidades de diálise; 2. Consulta junto à Central de Transplantes para determinar o número e as características de pacientes que estiveram em fila de transplante renal; 3. Consulta ao banco de dados dos pacientes registrados no Estudo Colaborativo do Registro Latino-Americano de Transplantes Renais Pediátricos. RESULTADOS: Dados de 301 pacientes, com idade de 9,0±5,8 anos, sendo 140 meninas (46,5 por cento), resultando em estimativa de prevalência de 23,4 casos por milhão da população com idade compatível (pmpic). A faixa etária mais representada foi a dos 10 a 15 anos (32,2 por cento), e as malformações do trato urinário representaram a etiologia conhecida mais comum (24,9 por cento). A maioria das crianças recebeu transplante renal (62,1 por cento), e entre os indivíduos em diálise predominou a hemodiálise (71,2 por cento). O SUS foi o financiador mais importante dos tratamentos. CONCLUSÃO: A prevalência de 23,4 casos pmpic que encontramos é inferior à reportada no mundo ocidental. Acreditamos que haja subestimativa de dados em nosso estudo, pois poucas unidades de diálise responderam o questionário. Tal viés não invalida o caráter exploratório dos resultados. É imperativo mecanismos de captação de dados sobre a DRC pediátrica de maneira prospectiva e nos estágios mais precoces da doença para dimensionarmos esse grave problema de saúde em nosso meio.
OBJECTIVE: To estimate the prevalence of pediatric end-stage renal disease and evaluate demographics and renal disease characteristics in state of São Paulo over the year 2008. METHODS: Observational, descriptive, and cross-sectional study based on a population sample with subjects < 18 years. The data collecting assumed three forms: 1. A questionnaire for dialysis units; 2. Search in the Transplant Center to determine the number and characteristics of patients who had been in a transplant waiting list over the study period; 3. Search in the database of patients registered at the Latin American Collaborative Registry of Pediatric Kidney Transplantation. RESULTS: Data from 301 patients aged 9.0 ± 5.8, including 140 girls (46.5 percent), resulting in an estimate prevalence of 23.4 cases per million age-related population (pmarp). The age group most frequently found was 10 to 15 years (32.2 percent), and urinary tract malformation was the most usual known etiology (24.9 percent). Most children underwent kidney transplantation (62.1 percent) and among subjects on dialysis, hemodialysis was predominant (71.2 percent). The Sistema Único de Saúde - Unified National Health System - (SUS) provided the financial support for treatments. CONCLUSION: The prevalence of 23.4 cases pmarp found by the authors is lower than that reported in Western world. We believe data were underestimated in the present study, as few dialysis units returned the completed questionnaire. This potential bias does not invalidate the exploratory character of results. Further mechanisms for retrospective and earlier data collecting on pediatric chronic renal disease (CRD) are needed so that the burden of this serious health condition can be appropriately sized up.
Asunto(s)
Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Fallo Renal Crónico/epidemiología , Distribución por Edad , Brasil/epidemiología , Estudios Transversales , Trasplante de Riñón/estadística & datos numéricos , Prevalencia , Diálisis Renal/estadística & datos numéricos , Distribución por SexoRESUMEN
BACKGROUND: Renal grafts have demonstrated capacity to adapt to the recipient. The aim of this study was to assess the growth of renal grafts transplanted to children, during the first year posttransplant. METHODS: We conducted a 1 year prospective study comparing the growth of renal grafts from children and adults donors transplanted in children, and correlated this growth with graft function. Two groups were studied: (a) group 1-32 children transplanted with pediatric deceased kidneys from donors younger than 16 years and (b) group 2-31 children transplanted with organs from adult living donors. Anthropometric assessment, sonographic measurement of the graft, and serum creatinine at 1 week, 1, 6, and 12 months posttransplantation were performed. RESULTS: Children from group 1 presented an 18% increase in graft volume after the sixth month of transplant, whereas in group 2 grafts presented a 14% reduction in volume, mainly during the first month; the variation in renal diameters was not uniform. Children from group 1 presented a glomerular filtration rate (GFR) increase during the follow-up (46-102 mL/min/1.73 m). After 1 year, GFR and graft volume were similar. Growth of individuals from both groups was comparable. CONCLUSIONS: In the short term, pediatric kidneys raise volume and GFR, whereas adult kidneys slightly lower volume and GFR when transplanted in children. Taking our and other studies results into consideration, we can hypothesize that in addition to compensatory hypertrophy, pediatric grafts are likely capable of continued somatic growth.
Asunto(s)
Trasplante de Riñón/fisiología , Riñón/crecimiento & desarrollo , Donadores Vivos/estadística & datos numéricos , Donantes de Tejidos/estadística & datos numéricos , Adolescente , Adulto , Estatura , Índice de Masa Corporal , Peso Corporal , Cadáver , Niño , Preescolar , Familia , Femenino , Humanos , Riñón/fisiología , Masculino , Persona de Mediana EdadRESUMEN
A broad spectrum of renal changes is observed in patients with sickle cell anemia, and ideal therapeutic measures for the management of these alterations are still being studied. Affected patients have deficient urinary concentration and potassium excretion. Perhaps owing to a compensatory mechanism, the proximal tubules are in a condition of "hyperfunction", with increased sodium and phosphorus reabsorption and greater creatinine and uric acid secretion. Mild tubular acidosis may be present. No treatment has been reported for these tubular changes, except for care in the maintenance of hydration. The use of anti-inflammatory drugs is being studied in order to inhibit the prostaglandins involved in the process. Increased renal blood flow, glomerular filtration rate, and filtration fraction are frequent findings. Hematuria commonly occurs as a consequence of red blood cell sickling in the renal medulla, papillary necrosis, or even renal medullary carcinoma. Measures such as increased fluid ingestion, urine alkalinization and, if necessary, administration of epsilon-aminocaproic acid and certain invasive procedures have been proposed to treat hematuria. Nephropathy in patients with sickle cell anemia can be manifested by proteinuria and, more rarely, nephrotic syndrome. Drugs such as prednisone and cyclophosphamide are ineffective for the treatment of patients with nephrotic syndrome. Angiotensin converting enzyme inhibitors decrease proteinuria, but their long-term effect in preventing the progression of glomerular disease has not been established. Chronic renal failure, although infrequent, may be one of the manifestations of this disease. Hemodialysis and transplantation are satisfactory therapeutic options for patients with end-stage renal disease.