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1.
Pediatr Nephrol ; 39(7): 2253-2262, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38446208

ABSTRACT

BACKGROUND: Acute kidney injury (AKI) is a life-threatening condition, especially in extreme age groups and when kidney replacement therapy (KRT) is necessary. Studies worldwide report mortality rates of 10-63% in pediatric patients undergoing KRT. METHODS: Over 13 years, this multicenter study analyzed data from 693 patients with AKI, all receiving KRT, across 74 hospitals and medical facilities in Rio de Janeiro, Brazil. RESULTS: The majority were male (59.5%), under one year old (55.6%), and treated in private hospitals (76.5%). Sixty-six percent had comorbidities. Pneumonia and congenital heart disease were the most common admission diagnoses (21.5% and 20.2%, respectively). The mortality rate was 65.2%, with lower rates in patients over 12 years (50%). Older age was protective (HR: 2.35, IQR [1.52-3.62] for neonates), and primary kidney disease had a three-fold lower mortality rate. ICU team experience (HR: 0.74, IQR [0.60-0.91]) correlated with lower mortality, particularly in hospitals treating 20 or more patients. Among the deceased, 40% died within 48 h of KRT initiation, suggesting possible late referral or treatment futility. CONCLUSIONS: This study confirms the high mortality in pediatric dialytic AKI in middle-income countries, underlining early mortality and offering critical insights for improving outcomes.


Subject(s)
Acute Kidney Injury , Renal Dialysis , Humans , Male , Acute Kidney Injury/therapy , Acute Kidney Injury/mortality , Acute Kidney Injury/epidemiology , Acute Kidney Injury/etiology , Child , Female , Child, Preschool , Infant , Brazil/epidemiology , Renal Dialysis/statistics & numerical data , Adolescent , Infant, Newborn , Retrospective Studies , Comorbidity , Risk Factors
3.
Eur J Pediatr ; 182(8): 3631-3637, 2023 Aug.
Article in English | MEDLINE | ID: mdl-37233777

ABSTRACT

The objective of this study was to reveal the signs and symptoms for the classification of pediatric patients at risk of CKD using decision trees and extreme gradient boost models for predicting outcomes. A case-control study was carried out involving children with 376 chronic kidney disease (cases) and a control group of healthy children (n = 376). A family member responsible for the children answered a questionnaire with variables potentially associated with the disease. Decision tree and extreme gradient boost models were developed to test signs and symptoms for the classification of children. As a result, the decision tree model revealed 6 variables associated with CKD, whereas twelve variables that distinguish CKD from healthy children were found in the "XGBoost". The accuracy of the "XGBoost" model (ROC AUC = 0.939, 95%CI: 0.911 to 0.977) was the highest, while the decision tree model was a little lower (ROC AUC = 0.896, 95%CI: 0.850 to 0.942). The cross-validation of results showed that the accuracy of the evaluation database model was like that of the training. CONCLUSION: In conclusion, a dozen symptoms that are easy to be clinically verified emerged as risk indicators for chronic kidney disease. This information can contribute to increasing awareness of the diagnosis, mainly in primary care settings. Therefore, healthcare professionals can select patients for more detailed investigation, which will reduce the chance of wasting time and improve early disease detection. WHAT IS KNOWN: • Late diagnosis of chronic kidney disease in children is common, increasing morbidity. • Mass screening of the whole population is not cost-effective. WHAT IS NEW: • With two machine-learning methods, this study revealed 12 symptoms to aid early CKD diagnosis. • These symptoms are easily obtainable and can be useful mainly in primary care settings.


Subject(s)
Renal Insufficiency, Chronic , Humans , Child , Case-Control Studies , Renal Insufficiency, Chronic/diagnosis , Risk Factors , Early Diagnosis , Machine Learning
5.
Pediatr Transplant ; 27(5): e14507, 2023 08.
Article in English | MEDLINE | ID: mdl-36919407

ABSTRACT

BACKGROUND: In children with kidney failure, the longer the duration of dialysis the greater the impact on growth deficit, quality of life, and life expectancy. The aim of this research is to test whether there was a shortening of treatment time from kidney failure to transplantation in pediatric patients and whether this time interval impacted height. METHODS: Observational retrospective cohort study from 2005 to 2018. The first outcome variable was time to transplantation in years, while the second was height/age standard deviation score (SDS) at transplantation. Cox regression models were used to analyze time from disease to transplantation and linear regression was employed to test the association of the year of kidney failure onset with height. RESULTS: A total of 780 children were evaluated and 517 underwent kidney transplantation after a median time of 1.9 years (IQR = 1.0-4.0). The variables significantly associated with time to transplant were: year of kidney failure onset (HR = 1.07; 95% CI: 1.05-1.10; p < .001), age at kidney failure onset <12 years (HR = 0.59; 95% CI: 0.49-0.71; p < .001), living in different state as transplant center (HR = 0.63; 95% CI: 0.53-0.77; p < .001), and undergoing blood transfusion before transplantation (HR = 0.63; 95% CI: 0.53-0.75; p < .001). Regarding growth, for each 1-year increase in the epoch of kidney failure onset, a 0.05 SDS raise in height/age is expected (p < .001). CONCLUSION: Children with recent kidney failure onset had significantly lower time to the outcome and this reduction was associated with a less severe growth deficit.


Subject(s)
Kidney Failure, Chronic , Renal Insufficiency , Child , Humans , Kidney Failure, Chronic/complications , Kidney Failure, Chronic/surgery , Retrospective Studies , Quality of Life , Renal Dialysis , Renal Insufficiency/complications , Renal Insufficiency/surgery , Treatment Outcome
6.
Kidney Int Rep ; 7(9): 2029-2038, 2022 Sep.
Article in English | MEDLINE | ID: mdl-36090503

ABSTRACT

Introduction: Post-streptococcal glomerulonephritis (PSGN) has a good prognosis in children, but few studies have evaluated the long-term renal outcomes in adults with PSGN. Methods: In a follow-up study, 47 predominantly adult patients with PSGN due to group C Streptococcus zooepidemicus were reassessed 20 years after an outbreak in Nova Serrana, Brazil. We evaluated clinical characteristics, renal outcomes, and the trajectory of the estimated glomerular filtration rate (eGFR) by the creatinine-based chronic kidney disease-epidemiology collaboration equation from 5 follow-up assessments. Logistic regression and mixed-effects regression were used in the analysis. Results: After 20 years, the participants' mean age was 56.6±15.1 years. Thirty-four (72%) patients had hypertension, 21 (44.7%) had eGFR <60 ml/min per 1.73 m2, 8 of 43 (18.6%) had urine protein-to-creatinine ratio >150 mg/g, and 25 (53%) had CKD (low eGFR and/or increased proteinuria). Increasing age was associated with CKD (odds ratio: 1.07; 95% confidence interval [CI]: 1.02-1.13; P = 0.011) in multivariate analysis. The mean eGFR decline in the last 11 years of follow-up was -3.2 ml/min per 1.73 m2 per year (95% CI: -3.7 to -2.7). Older age at baseline (coefficient -1.05 ml/min per 1.73 m2 per year; 95% CI -1.28 to -0.81; P < 0.001), and hypertension 5 years after the outbreak (coefficient -7.78 ml/min/1.73 m2; 95% CI -14.67 to -0.78; P = 0.027) were associated with lower eGFR during the whole study period. Conclusion: There was a marked worsening of renal function and a high prevalence of CKD and hypertension after 20 years of PSGN outbreak. Long-term follow-up is warranted after PSGN, especially among older patients.

7.
Pediatr Nephrol ; 37(5): 1137-1147, 2022 05.
Article in English | MEDLINE | ID: mdl-34651246

ABSTRACT

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.


Subject(s)
Kidney Transplantation , Thrombosis , Adult , Child , Graft Rejection/epidemiology , Graft Rejection/etiology , Graft Survival , Humans , Kidney , Kidney Transplantation/adverse effects , Living Donors , Retrospective Studies , Thrombosis/epidemiology , Thrombosis/etiology , Tissue Donors
8.
JPEN J Parenter Enteral Nutr ; 45(1): 57-64, 2021 01.
Article in English | MEDLINE | ID: mdl-31637763

ABSTRACT

BACKGROUND: We investigated to what extent serum copper influences hemoglobin (Hb), neutrophil, and platelet counts and the factors associated with serum copper in children with intestinal failure (IF) who have their micronutrient status monitored according to a standard protocol. METHODS: Children with IF admitted to a pediatric intestinal rehabilitation program and receiving home parenteral nutrition (PN) were followed up prospectively. Patients received vitamins and multi-trace elements (TEs) as part of the PN. Copper, iron, zinc, vitamin A, and cobalamin serum levels were routinely monitored at 3-month intervals or monthly when a deficiency was detected. Complete blood counts were performed biweekly. Repeated-measures analyses were used to estimate the effect of explanatory variables on the outcomes. RESULTS: Thirteen children with a median time receiving PN of 16.6 months were included. An average of 7 copper measurements per patient were performed; 53.8% of patients had a low serum level at least twice during the follow-up. Eight patients with cholestasis had TEs of PN discontinued. In the multivariable analysis, copper was not associated with Hb levels; an increase of 10 µg/dL in serum copper resulted in an increase of 240/mm3 (95% confidence interval [CI], 18.0-30.1) neutrophils and of 8429/mm3 (95% CI, 466-1219) platelets (P < .001). Time of PN without copper, direct bilirubin levels, and ostomy were associated with lower serum copper. CONCLUSION: The increase in serum copper was associated with significant increases in neutrophil and platelet counts. Time without copper in PN, cholestasis, and having an ostomy negatively influenced copper status.


Subject(s)
Parenteral Nutrition, Home , Short Bowel Syndrome , Trace Elements , Child , Copper , Humans , Retrospective Studies , Risk Factors , Short Bowel Syndrome/complications , Short Bowel Syndrome/therapy
9.
Pediatr Transplant ; 25(2): e13856, 2021 03.
Article in English | MEDLINE | ID: mdl-32997892

ABSTRACT

AR is a major relevant and challenging topic in pediatric kidney transplantation. Our objective was to evaluate cumulative incidence of AR in pediatric kidney transplant patient, risk factors for this outcome, and impact on allograft function and survival. A retrospective cohort including pediatric patients that underwent kidney transplantation between 2011 and 2015 was designed. Risk factors for AR were tested by competing risk analysis. To estimate its impact, graft survival and difference in GFR were evaluated. Two hundred thirty patients were included. As a whole, the incidence of AR episodes was 0.16 (95% CI = 0.12-0.20) per person-year of follow-up. And cumulative incidence of AR was 23% in 1 year and 39% in 5 years. Risk factors for AR were number of MM (SHR 1.36 CI 1.14-1.63 P = .001); ISS with CSA, PRED, and AZA (SHR 2.22 CI 1.14-4.33 P = .018); DGF (SHR 2.49 CI 1.57-3.93 P < .001); CMV infection (SHR 5.52 CI 2.27-11.0 P < .001); and poor adherence (SHR 2.28 CI 1.70-4.66 P < .001). Death-censored graft survival in 1 and 5 years was 92.5% and 72.1%. Risk factors for graft loss were number of MM (HR 1.51 CI 1.07-2.13 P = .01), >12 years (HR 2.66 CI 1.07-6.59 P = .03), and PRA 1%-50% (HR 2.67 CI 1.24-5.73 P = .01). Although occurrence of AR did not influence 5-year graft survival, it negatively impacted GFR. AR was frequent in patients assessed and associated with number of MM, ISS regimen, DGF, CMV infection, and poor adherence, and had deleterious effect on GFR.


Subject(s)
Graft Rejection/epidemiology , Kidney Transplantation , Kidney/physiopathology , Adolescent , Brazil/epidemiology , Child , Child, Preschool , Female , Follow-Up Studies , Graft Rejection/etiology , Graft Rejection/physiopathology , Graft Survival , Humans , Incidence , Kaplan-Meier Estimate , Kidney Function Tests , Male , Proportional Hazards Models , Retrospective Studies , Risk Factors
10.
Transplantation ; 104(8): e236-e242, 2020 08.
Article in English | MEDLINE | ID: mdl-32732842

ABSTRACT

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.


Subject(s)
Graft Rejection/epidemiology , Hospitals, Pediatric/organization & administration , Kidney Failure, Chronic/surgery , Kidney Transplantation/statistics & numerical data , Time-to-Treatment/organization & administration , Adolescent , Body Weight/physiology , Brazil/epidemiology , Child , Child, Preschool , Female , Follow-Up Studies , Glomerular Filtration Rate/physiology , Graft Rejection/etiology , Graft Rejection/physiopathology , Graft Survival/physiology , Health Plan Implementation , Health Services Accessibility/organization & administration , Health Services Accessibility/statistics & numerical data , Hospitals, Pediatric/statistics & numerical data , Humans , Incidence , Kidney Failure, Chronic/mortality , Kidney Failure, Chronic/physiopathology , Kidney Transplantation/adverse effects , Male , Program Evaluation , Survival Analysis , Time Factors , Treatment Outcome
11.
Transplantation ; 104(3): 591-596, 2020 03.
Article in English | MEDLINE | ID: mdl-31335768

ABSTRACT

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.


Subject(s)
Body Weight , Kidney Failure, Chronic/therapy , Kidney Transplantation/standards , Preoperative Care/standards , Time-to-Treatment , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Renal Dialysis/standards , Retrospective Studies , Time Factors , Young Adult
12.
Appetite ; 135: 137-145, 2019 04 01.
Article in English | MEDLINE | ID: mdl-30439381

ABSTRACT

The present study explored the consumption of ultra-processed foods and its association with food addiction in overweight children. The prevalence of food addiction was investigated using the Yale Food Addiction Scale for Children in overweight 9-11 year-old children (BMI/age ≥1 Z score) of both sexes from two schools (n = 139). Food intake was estimated by a food frequency questionnaire and the food items were classified into 4 categories: minimally processed, culinary ingredients, processed foods and ultra-processed foods (UPF), based on their degree of processing. Among the children, 95% showed at least one of the seven symptoms of food addiction and 24% presented with a diagnosis of food addiction. In analysis of covariance adjusted for age and sex, a tendency of higher consumption of added sugar (refined sugar, honey, corn syrup) and UPF was found among those diagnosed with food addiction. Multiple logistic regression adjusted for sugar, sodium and fat ingestion showed that consumption of cookies/biscuits (OR = 4.19, p = 0.015) and sausages (OR = 11.77, p = 0.029) were independently associated with food addiction. The identification of foods that may be associated with addictive behavior is very important for correctly treating and preventing childhood obesity, which continues to be one of the greatest health problems in the world.


Subject(s)
Diet , Feeding Behavior , Food Addiction/etiology , Food Handling , Food Technology , Pediatric Obesity/etiology , Child , Diet Surveys , Eating , Energy Intake , Fast Foods/adverse effects , Female , Food Supply , Humans , Logistic Models , Male , Meat Products/adverse effects , Odds Ratio , Overweight
13.
J. pediatr. (Rio J.) ; 94(1): 93-99, Jan.-Feb. 2018. tab, graf
Article in English | LILACS | ID: biblio-894097

ABSTRACT

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.


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Renal Dialysis/economics , Kidney Transplantation/economics , Kidney Failure, Chronic/economics , Brazil , Retrospective Studies , Cohort Studies , Costs and Cost Analysis , Kidney Failure, Chronic/surgery , Kidney Failure, Chronic/therapy
14.
J Pediatr (Rio J) ; 94(1): 93-99, 2018.
Article in English | MEDLINE | ID: mdl-28750890

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.


Subject(s)
Kidney Failure, Chronic/economics , Kidney Transplantation/economics , Renal Dialysis/economics , Brazil , Child , Child, Preschool , Cohort Studies , Costs and Cost Analysis , Female , Humans , Kidney Failure, Chronic/surgery , Kidney Failure, Chronic/therapy , Male , Retrospective Studies
15.
Transplantation ; 101(12): 2905-2912, 2017 12.
Article in English | MEDLINE | ID: mdl-28658201

ABSTRACT

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.


Subject(s)
Kidney Transplantation , Nephrotic Syndrome/genetics , Nephrotic Syndrome/surgery , Adolescent , Brazil , Child , Child, Preschool , Computational Biology , Cross-Sectional Studies , DNA Mutational Analysis , Female , Genetic Association Studies , Genotype , High-Throughput Nucleotide Sequencing , Humans , Infant , Male , Mutation
16.
Pediatr Crit Care Med ; 17(6): 585-6, 2016 06.
Article in English | MEDLINE | ID: mdl-27261662
17.
Pediatr Crit Care Med ; 17(2): e50-7, 2016 Feb.
Article in English | MEDLINE | ID: mdl-26695729

ABSTRACT

OBJECTIVES: Serum albumin is a strong biomarker of disease severity and prognosis in adult patients. In contrast, its value as predictor of outcome in critically ill children has not been established. We aimed to determine whether admission hypoalbuminemia is associated with outcome in a general pediatric population of critically ill patients, taking into account the inflammatory response, disease severity, and nutritional status of the patient. DESIGN: Analysis of prospectively collected database. SETTING: PICU of a teaching hospital. PATIENTS: Two hundred seventy-one patients consecutively admitted. Neonates, patients with chronic liver or kidney disease, inborn errors of metabolism, those who received prior administration of albumin solution, and readmissions were excluded. MEASUREMENTS AND MAIN RESULTS: Outcome variables were 60-day mortality, probability of ICU discharge at 60 days, and ventilator-free days. Potential exposure variables for the outcome were sex, age, nutritional status, albumin, C-reactive protein and serum lactate at admission, and Pediatric Index of Mortality 2 score. Admission hypoalbuminemia was present in 64.2% of patients. After adjustment for confounding factors, only serum lactate, Pediatric Index of Mortality 2 score, and serum albumin were associated with higher mortality: the increase of 1.0 g/dL in serum albumin at admission resulted in a 73% reduction in the hazard of death (hazard ratio, 0.27; 95% CI, 0.14-0.51; p < 0.001). The increase of 1 g/dL in serum albumin was also independently associated with a 33% rise in the probability of ICU discharge (subhazard ratio, 1.33; 95% CI, 1.07-1.64; p = 0.008) and increased ventilator-free-days (odds ratio, 1.86; 95% CI, 0.56-3.16; p = 0.005). CONCLUSIONS: Hypoalbuminemia at admission to a PICU is associated with higher 60-day mortality, longer duration of mechanical ventilation, and lower probability of ICU discharge. These associations are independent of the magnitude of inflammatory response, clinical severity, and nutritional status.


Subject(s)
Child Mortality , Critical Illness/mortality , Hospital Mortality , Hypoalbuminemia/complications , Serum Albumin/metabolism , Child , Child, Preschool , Female , Hospitals, Teaching , Humans , Hypoalbuminemia/mortality , Infant , Intensive Care Units, Pediatric/statistics & numerical data , Length of Stay , Male , Patient Outcome Assessment , Prognosis , Prospective Studies , Respiration, Artificial , Risk Factors
18.
PLoS One ; 10(8): e0135649, 2015.
Article in English | MEDLINE | ID: mdl-26285019

ABSTRACT

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.


Subject(s)
Kidney Failure, Chronic/epidemiology , Kidney Failure, Chronic/therapy , Renal Dialysis/statistics & numerical data , Adolescent , Brazil/epidemiology , Child , Cross-Sectional Studies , Female , Health Surveys , Humans , Incidence , Kidney Failure, Chronic/diagnosis , Male , Prevalence , Socioeconomic Factors
19.
Pediatr Transplant ; 18(4): 342-9, 2014 Jun.
Article in English | MEDLINE | ID: mdl-24646422

ABSTRACT

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.


Subject(s)
Body Weight , Iliac Artery/surgery , Kidney Failure, Chronic/surgery , Kidney Transplantation/methods , Renal Artery/surgery , Vena Cava, Inferior/surgery , Adolescent , Anastomosis, Surgical , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Male , Retrospective Studies , Treatment Outcome
20.
Nutrition ; 28(3): 267-70, 2012 Mar.
Article in English | MEDLINE | ID: mdl-21872433

ABSTRACT

OBJECTIVE: To determine the nutritional status of a cohort of children admitted to a pediatric intensive care unit (ICU) and to assess the effect of malnutrition as an independent risk factor affecting outcome in this patient group. METHODS: In a prospective cohort study, 385 children admitted to the ICU of a teaching hospital over a 2-y period were assessed for nutritional status at admission and clinical outcome. The outcome variables were 30-d mortality, length of ICU stay, and length of mechanical ventilation. Potential exposure variables were gender, age, diagnosis (clinical versus surgical), septic shock, malnutrition, and scores on the Pediatric Index of Mortality and Pediatric Logistic Organ Dysfunction. Nutritional status was determined using z scores of weight for age, height for age, and body mass index, based on the World Health Organization child growth standards. Patients with z score < -2 of anthropometric indexes were considered malnourished. RESULTS: 175 patients (45.5%) were malnourished on admission. Sixteen patients of the malnourished group (9.14%) and 25 patients (11.9%) of the non-malnourished group died. Malnutrition was associated with greater length of mechanical ventilation and length of ICU stay, but not with mortality on univariate analysis. Malnutrition was associated with greater length of ventilation on the multiple logistic regression model (OR 1.76, 95%; CI 1.08-2.88; P = 0.024). CONCLUSION: Malnutrition is common among children admitted to an ICU. This factor was not a predictor of mortality but showed independent association with length of mechanical ventilation.


Subject(s)
Critical Illness/mortality , Length of Stay , Malnutrition/epidemiology , Respiration, Artificial , Child, Preschool , Chronic Disease , Female , Humans , Infant , Intensive Care Units, Pediatric , Logistic Models , Male , Nutrition Assessment , Nutritional Status , Prospective Studies , Risk Factors , Shock, Septic/epidemiology
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