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1.
J. bras. nefrol ; 45(2): 244-251, June 2023. graf
Article in English | LILACS-Express | LILACS | ID: biblio-1506581

ABSTRACT

ABSTRACT The covid-19 vaccine confers direct protection and reduces transmission rates of the virus and new variants. Vaccines from Pfizer/BioNTech and CoronaVac have been cleared for children in Brazil. They are safe, effective, and immunogenic. There are no known complications associated with the use of steroids or vaccines in pediatric patients with covid-19 and nephrotic syndrome. With or without immunosuppression, these patients are not at increased risk of severe covid-19, and steroids are safe for them. A milder form of covid-19 occurs in patients with chronic kidney disease without the need for hospitalization. The vaccine response may be reduced and/or the duration of antibodies after vaccination may be shorter than in the general population. However, considering risk of exposure, vaccination against covid-19 is recommended. It is believed that patients with hemolytic-uremic syndrome are at higher risk of severe covid-19. Vaccination is recommended, although specific data on the safety and efficacy of the covid-19 vaccine are limited. There is agreement that the benefits of induced immunity outweigh the risks of immunization. Vaccination against covid-19 is recommended for children and adolescents needing kidney transplantation or who have undergone transplantation. These patients present decreased immune response after vaccination, but immunization is recommended because the benefits outweigh the risks of vaccination. Current recommendations in Brazil stipulate the use of the messenger RNA vaccine. This paper aims to provide pediatric nephrologists with the latest knowledge about vaccination against covid-19 for children with kidney disease.


Resumo A vacina covid-19 confere proteção direta, reduz as taxas de transmissão do vírus e de novas variantes. No Brasil, estão liberadas para a população pediátrica as vacinas Pfizer/BioNTech e a CoronaVac, ambas seguras, eficazes e imunogênicas. Pacientes pediátricos com síndrome nefrótica e covid-19 têm curso clínico regular sem complicações relacionadas ao uso de esteroides ou vacinas. Esses pacientes, com ou sem imunossupressão, não apresentam maior risco de covid-19 grave e o tratamento com esteroides é seguro. Os pacientes com doença renal crônica têm covid-19 mais leve, sem necessidade de hospitalização. A resposta vacinal pode ser reduzida e/ou a duração dos anticorpos pós-vacinação pode ser menor do que na população geral. Entretanto, a vacina covid-19 está recomendada, considerando o risco de exposição. Acredita-se que pacientes com síndrome hemolítico-urêmica teriam maior risco de covid-19 grave. A vacina é recomendada, embora dados específicos sobre segurança e eficácia da vacina covid-19 sejam limitados. Há concordância que os benefícios da imunidade induzida superam quaisquer riscos da imunização. A vacina covid-19 é recomendada para crianças e adolescentes candidatos ao transplante renal ou já transplantados. Esses pacientes têm resposta imunológica reduzida após a vacina, entretanto ela é recomendada porque os benefícios superam qualquer risco dessa vacinação. A recomendação atual no Brasil é a vacina de tecnologia RNA mensageiro. O objetivo deste documento é levar aos nefrologistas pediátricos os conhecimentos mais recentes sobre a vacinação contra contra-19 em crianças com doenças renais.

2.
Blood Purif ; 51 Suppl 1: 61-67, 2023 May 10.
Article in English | MEDLINE | ID: mdl-37231795

ABSTRACT

INTRODUCTION: Growth retardation is a common problem in pediatric patients with chronic kidney disease. It is unknown if the growth of children on peritoneal dialysis (PD) can be augmented by more dialysis. METHODS: We studied the effect of various peritoneal adequacy parameters on delta height standard deviation scores (SDSs) and growth velocity z-scores in 53 children (27 males) on PD, who underwent 2 longitudinal adequacy tests at 9-month intervals. None of the patients were on growth hormone. Intraperitoneal pressure and standard KDOQI guidelines were compared to the outcome measures delta height SDS and height velocity z-scores, using univariate and multivariate tests. RESULTS: At the time of the second PD adequacy test, their mean age was 9.2 ± 5.3 years; mean fill volume was 961 ± 254 mL/m2; and median total infused dialysate volume was 5.26 L/m2/day (range 2.03-15.32 L). The median total weekly Kt/V was 3.79 (range 0.9-9.5), and the median total creatinine clearance was 56.6 (range 7.6-133.48) L/week, higher than previous pediatric studies. The delta height SDS was a median of -0.12 (range -2 to +3.95)/year. The mean height velocity z-score was -1.6 ± 4.0. The only relationships discovered were between the delta height SDS and age, bicarbonate, and intraperitoneal pressure, but not for Kt/V or creatinine clearance. CONCLUSION: Our findings highlight the importance of normalization of bicarbonate concentrations to improve height z-score.

3.
J Bras Nefrol ; 45(2): 244-251, 2023.
Article in English, Portuguese | MEDLINE | ID: mdl-36282106

ABSTRACT

The covid-19 vaccine confers direct protection and reduces transmission rates of the virus and new variants. Vaccines from Pfizer/BioNTech and CoronaVac have been cleared for children in Brazil. They are safe, effective, and immunogenic. There are no known complications associated with the use of steroids or vaccines in pediatric patients with covid-19 and nephrotic syndrome. With or without immunosuppression, these patients are not at increased risk of severe covid-19, and steroids are safe for them. A milder form of covid-19 occurs in patients with chronic kidney disease without the need for hospitalization. The vaccine response may be reduced and/or the duration of antibodies after vaccination may be shorter than in the general population. However, considering risk of exposure, vaccination against covid-19 is recommended. It is believed that patients with hemolytic-uremic syndrome are at higher risk of severe covid-19. Vaccination is recommended, although specific data on the safety and efficacy of the covid-19 vaccine are limited. There is agreement that the benefits of induced immunity outweigh the risks of immunization. Vaccination against covid-19 is recommended for children and adolescents needing kidney transplantation or who have undergone transplantation. These patients present decreased immune response after vaccination, but immunization is recommended because the benefits outweigh the risks of vaccination. Current recommendations in Brazil stipulate the use of the messenger RNA vaccine. This paper aims to provide pediatric nephrologists with the latest knowledge about vaccination against covid-19 for children with kidney disease.

4.
J. bras. nefrol ; 44(4): 579-584, Dec. 2022. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1421913

ABSTRACT

Abstract Introdução: A diálise peritoneal (DP) é importante para a pediatria. Este estudo mostrou dados de centros brasileiros que utilizam DP pediátrica. Método: Estudo transversal, observacional, descritivo com questionário eletrônico. Incluiu-se pacientes de 0-18 anos em DP cadastrados nos bancos de dados dos diversos centros. Questionário preenchido anonimamente, sem dados de identificação. Foi adotada metodologia quantitativa. Resultados: 212 pacientes estão em DP no Brasil (agosto, 2021). 80% têm menos de 12 anos de idade. A maioria realiza DP automatizada e 74% são dependentes do Sistema Único de Saúde. Em 25% dos centros faltou material de DP e em 51% os pacientes pediátricos foram convertidos de DP para HD. Conclusão: A maioria dos pacientes tinha menos de 12 anos e era dependente do SUS. A escassez de insumos aconteceu em 25% dos centros. Esses dados apontam para o problema da sustentabilidade de DP, única alternativa de TRS em crianças muito pequenas.


Resumo Introdução: A diálise peritoneal (DP) é importante para a pediatria. Este estudo mostrou dados de centros brasileiros que utilizam DP pediátrica. Método: Estudo transversal, observacional, descritivo com questionário eletrônico. Incluiu-se pacientes de 0-18 anos em DP cadastrados nos bancos de dados dos diversos centros. Questionário preenchido anonimamente, sem dados de identificação. Foi adotada metodologia quantitativa. Resultados: 212 pacientes estão em DP no Brasil (agosto, 2021). 80% têm menos de 12 anos de idade. A maioria realiza DP automatizada e 74% são dependentes do Sistema Único de Saúde. Em 25% dos centros faltou material de DP e em 51% os pacientes pediátricos foram convertidos de DP para HD. Conclusão: A maioria dos pacientes tinha menos de 12 anos e era dependente do SUS. A escassez de insumos aconteceu em 25% dos centros. Esses dados apontam para o problema da sustentabilidade de DP, única alternativa de TRS em crianças muito pequenas.

5.
Clin Kidney J ; 15(8): 1601-1611, 2022 Aug.
Article in English | MEDLINE | ID: mdl-35892013

ABSTRACT

Background: Atypical hemolytic uremic syndrome (aHUS) is an ultra-rare disease. Therefore, studies involving large samples are scarce, making registries powerful tools to evaluate cases. We present herein the first analysis of the Brazilian aHUS Registry (BRaHUS). Methods: Analysis of clinical, laboratory, genetic and treatment data from patients inserted in the BRaHUS, from 2017 to 2020, as an initiative of the Rare Diseases Committee of the Brazilian Society of Nephrology. Results: The cohort consisted of 75 patients (40 adults and 35 pediatric). There was a predominance of women (56%), median age at diagnosis of 20.7 years and a positive family history in 8% of cases. Renal involvement was observed in all cases and 37% had low C3 levels. In the <2 years of age group, males were predominant. Children presented lower levels of hemoglobin (P = .01) and platelets (P = .003), and higher levels of lactate dehydrogenase (LDH) (P = .004) than adults. Genetic analysis performed in 44% of patients revealed pathogenic variants in 66.6% of them, mainly in CFH and the CFHR1-3 deletion. Plasmapheresis was performed more often in adults (P = .005) and 97.3% of patients were treated with eculizumab and its earlier administration was associated with dialysis-free after 3 months (P = .08). Conclusions: The cohort of BRaHUS was predominantly composed of female young adults, with renal involvement in all cases. Pediatric patients had lower hemoglobin and platelet levels and higher LDH levels than adults, and the most common genetic variants were identified in CFH and the CFHR1-3 deletion with no preference of age, a peculiar pattern of Brazilian patients.

6.
J Bras Nefrol ; 44(4): 579-584, 2022.
Article in English, Portuguese | MEDLINE | ID: mdl-35348573

ABSTRACT

INTRODUçÃO: A diálise peritoneal (DP) é importante para a pediatria. Este estudo mostrou dados de centros brasileiros que utilizam DP pediátrica. MÉTODO: Estudo transversal, observacional, descritivo com questionário eletrônico. Incluiu-se pacientes de 0-18 anos em DP cadastrados nos bancos de dados dos diversos centros. Questionário preenchido anonimamente, sem dados de identificação. Foi adotada metodologia quantitativa. RESULTADOS: 212 pacientes estão em DP no Brasil (agosto, 2021). 80% têm menos de 12 anos de idade. A maioria realiza DP automatizada e 74% são dependentes do Sistema Único de Saúde. Em 25% dos centros faltou material de DP e em 51% os pacientes pediátricos foram convertidos de DP para HD. CONCLUSÃO: A maioria dos pacientes tinha menos de 12 anos e era dependente do SUS. A escassez de insumos aconteceu em 25% dos centros. Esses dados apontam para o problema da sustentabilidade de DP, única alternativa de TRS em crianças muito pequenas.


Subject(s)
Nephrology , Organ Transplantation , Peritoneal Dialysis , Humans , Child , Brazil , Renal Dialysis
7.
Eur J Pediatr ; 181(2): 715-723, 2022 Feb.
Article in English | MEDLINE | ID: mdl-34553252

ABSTRACT

Fanconi anemia (FA) is a rare disease characterized by progressive bone marrow failure, cancer predisposition, and multiple systemic malformations, including congenital abnormalities of the kidney and urinary tract (CAKUT). Hematopoietic cell transplantation (HCT), the only potentially curative treatment for the hematological complications of FA, may precipitate acute kidney injury (AKI) and hypertension. We retrospectively investigated 107 FA patients who underwent HCT between 2009 and 2017. We investigated the incidence and risk factors of AKI within 100 days after HCT in a cohort of FA patients, and kidney function and hypertension over 2-year follow-up.The incidence of AKI (mainly stage I) was 18.7%. Patients aged ≥ 11 years at transplantation showed a higher risk of AKI (OR 3.53). The eGFR was 60-90 mL/min/1.73 m2 in 53 (49.5%), 55 (51.4%), 50 (50.5%), 50 (51%), and 46 (59.7%) patients before HCT, at 100 days, 6 months, 1 year, and 2 years. Within the first 100 days after HCT, hypertension was observed in 72% of the patients and was associated with cyclosporine therapy. Most (62.3%) patients had stage 2 hypertension. CAKUT was observed in 33.7% of the patients and was associated with both hypertension (86%) and diminished kidney function but not with AKI.Conlusion: Although AKI, a commonly known HCT complication, was mild in this study, the prevalence of chronic kidney disease (CKD), as well as the high incidence of hypertension, specially associated with CAKUT point out the importance of kidney care in short and long-term follow up of FA patients. What is Known: • Fanconi anemia (FA) is the most frequent inherited bone marrow failure in children, and 30% of cases have congenital anomalies of kidney (CAKUT). • Acute kidney injury and hypertension after hematopoietic cell transplantation (HCT) may impact the outcomes.. What is New: • Despite the presence of CAKUT and stage 2 CKD in 33.7% and 50% of the patients, respectively, AKI was mild and transitory after HCT in FA patients. • CAKUT in FA patients was associated with lower kidney function and hypertension after HCT.


Subject(s)
Acute Kidney Injury , Fanconi Anemia , Hematopoietic Stem Cell Transplantation , Acute Kidney Injury/epidemiology , Acute Kidney Injury/etiology , Child , Fanconi Anemia/complications , Fanconi Anemia/epidemiology , Fanconi Anemia/therapy , Hematopoietic Stem Cell Transplantation/adverse effects , Humans , Kidney , Retrospective Studies
8.
Arch Endocrinol Metab ; 64(6): 796-802, 2021 Nov 01.
Article in English | MEDLINE | ID: mdl-33049132

ABSTRACT

OBJECTIVE: The aim of this cross-sectional study was to estimate the prevalence of XLH in Paraná, a state in southern Brazil, and report the clinical features and complications of the disease. METHODS: We invited all endocrinologists (n = 205), nephrologists (n = 221), orthopedic surgeons (n = 1020), and pediatricians (n = 1000) in Paraná to fill out an electronic survey with information on patients with X-linked hypophosphatemia (XLH), and searched the records of the state's health department for all calcitriol prescriptions in 2018. RESULTS: In all, 244 (10%) specialists responded to the email, of whom 18 (7.4%) reported to be taking care of patients with XLH and answered the online survey. A total of 57 patients with XLH were identified (prevalence 5 per million inhabitants). The median age at diagnosis was 22 years, and 42.2% were children and adolescents. Fifteen patients had genetic testing showing a PHEX mutation. Overall, 91.2% had bone deformities, 30.8% had a history of fragility fractures, and 22.4% had renal complications. CONCLUSION: This study demonstrated a prevalence of XLH of 5 cases per million inhabitants in the state of Paraná, a rate lower than the one reported in other countries. Manifestations of renal calcification and bone fragility were frequent among the patients. This is the first epidemiological study evaluating the prevalence and clinical presentation of XLH in Latin America.


Subject(s)
Familial Hypophosphatemic Rickets , Genetic Diseases, X-Linked , Adolescent , Brazil/epidemiology , Child , Cross-Sectional Studies , Familial Hypophosphatemic Rickets/epidemiology , Familial Hypophosphatemic Rickets/genetics , Humans , PHEX Phosphate Regulating Neutral Endopeptidase , Prevalence
9.
Arch. endocrinol. metab. (Online) ; 64(6): 796-802, Nov.-Dec. 2020. tab, graf
Article in English | LILACS | ID: biblio-1142201

ABSTRACT

ABSTRACT Objective: The aim of this cross-sectional study was to estimate the prevalence of XLH in Paraná, a state in southern Brazil, and report the clinical features and complications of the disease. Materials and methods: We invited all endocrinologists (n = 205), nephrologists (n = 221), orthopedic surgeons (n = 1020), and pediatricians (n = 1000) in Paraná to fill out an electronic survey with information on patients with X-linked hypophosphatemia (XLH), and searched the records of the state's health department for all calcitriol prescriptions in 2018. Results: In all, 244 (10%) specialists responded to the email, of whom 18 (7.4%) reported to be taking care of patients with XLH and answered the online survey. A total of 57 patients with XLH were identified (prevalence 5 per million inhabitants). The median age at diagnosis was 22 years, and 42.2% were children and adolescents. Fifteen patients had genetic testing showing a PHEX mutation. Overall, 91.2% had bone deformities, 30.8% had a history of fragility fractures, and 22.4% had renal complications. Conclusion: This study demonstrated a prevalence of XLH of 5 cases per million inhabitants in the state of Paraná, a rate lower than the one reported in other countries. Manifestations of renal calcification and bone fragility were frequent among the patients. This is the first epidemiological study evaluating the prevalence and clinical presentation of XLH in Latin America.


Subject(s)
Humans , Child , Adolescent , Genetic Diseases, X-Linked , Familial Hypophosphatemic Rickets/genetics , Familial Hypophosphatemic Rickets/epidemiology , Brazil/epidemiology , Prevalence , Cross-Sectional Studies , PHEX Phosphate Regulating Neutral Endopeptidase
10.
Rev. bras. ter. intensiva ; 32(4): 557-563, out.-dez. 2020. tab
Article in English, Portuguese | LILACS | ID: biblio-1156243

ABSTRACT

RESUMO Objetivo: Avaliar a associação entre uso de drogas nefrotóxicas e lesão renal aguda em pacientes pediátricos graves. Métodos: Estudo de coorte retrospectivo envolvendo todas as crianças internadas na unidade de terapia intensiva de um hospital pediátrico durante o período de 1 ano. A lesão renal aguda foi definida pela classificação KDIGO. Foram incluídos pacientes com tempo de internação maior que 48 horas e idade entre 1 mês e 14 anos. Foram excluídos aqueles com nefropatia aguda ou crônica, uropatia, cardiopatia congênita ou adquirida, uso crônico de drogas nefrotóxicas, rabdomiólise e síndrome de lise tumoral. Os pacientes foram classificados quanto ao uso de drogas nefrotóxicas durante internação na unidade de terapia intensiva pediátrica. Resultados: A amostra foi composta por 226 crianças, sendo que 37,1% fizeram uso de drogas nefrotóxicas, 42,4% desenvolveram lesão renal aguda e 7,5% morreram. As medicações que, isoladamente, apresentaram associação com lesão renal aguda foram aciclovir (p < 0,001), vancomicina (p < 0,001), furosemida (p < 0,001) e ganciclovir (p = 0,008). O uso concomitante de duas ou mais drogas nefrotóxicas foi caracterizado como marcador independente de disfunção renal (p < 0,001). Após alta da unidade de terapia intensiva pediátrica, o acompanhamento da função renal na enfermaria foi inadequado em 19,8% dos casos. Conclusão: É necessário que o médico intensivista tenha conhecimento das principais drogas nefrotóxicas, de modo a prever, reduzir ou evitar danos a seus pacientes.


Abstract Objective: To evaluate the association between the use of nephrotoxic drugs and acute kidney injury in critically ill pediatric patients. Methods: This was a retrospective cohort study involving all children admitted to the intensive care unit of a pediatric hospital during a 1-year period. Acute kidney injury was defined according to the KDIGO classification. Patients with a length of hospital stay longer than 48 hours and an age between 1 month and 14 years were included. Patients with acute or chronic nephropathy, uropathy, congenital or acquired heart disease, chronic use of nephrotoxic drugs, rhabdomyolysis and tumor lysis syndrome were excluded. Patients were classified according to the use of nephrotoxic drugs during their stay at the pediatric intensive care unit. Results: The sample consisted of 226 children, of whom 37.1% used nephrotoxic drugs, 42.4% developed acute kidney injury, and 7.5% died. The following drugs, when used alone, were associated with acute kidney injury: acyclovir (p < 0.001), vancomycin (p < 0.001), furosemide (p < 0.001) and ganciclovir (p = 0.008). The concomitant use of two or more nephrotoxic drugs was characterized as an independent marker of renal dysfunction (p < 0.001). After discharge from the pediatric intensive care unit, renal function monitoring in the ward was inadequate in 19.8% of cases. Conclusion: It is necessary for intensivist physicians to have knowledge of the main nephrotoxic drugs to predict, reduce or avoid damage to their patients.


Subject(s)
Humans , Infant , Child , Pharmaceutical Preparations , Acute Kidney Injury/chemically induced , Acute Kidney Injury/epidemiology , Intensive Care Units, Pediatric , Retrospective Studies , Risk Factors , Critical Illness
11.
Rev Bras Ter Intensiva ; 32(4): 557-563, 2020.
Article in Portuguese, English | MEDLINE | ID: mdl-33470357

ABSTRACT

OBJECTIVE: To evaluate the association between the use of nephrotoxic drugs and acute kidney injury in critically ill pediatric patients. METHODS: This was a retrospective cohort study involving all children admitted to the intensive care unit of a pediatric hospital during a 1-year period. Acute kidney injury was defined according to the KDIGO classification. Patients with a length of hospital stay longer than 48 hours and an age between 1 month and 14 years were included. Patients with acute or chronic nephropathy, uropathy, congenital or acquired heart disease, chronic use of nephrotoxic drugs, rhabdomyolysis and tumor lysis syndrome were excluded. Patients were classified according to the use of nephrotoxic drugs during their stay at the pediatric intensive care unit. RESULTS: The sample consisted of 226 children, of whom 37.1% used nephrotoxic drugs, 42.4% developed acute kidney injury, and 7.5% died. The following drugs, when used alone, were associated with acute kidney injury: acyclovir (p < 0.001), vancomycin (p < 0.001), furosemide (p < 0.001) and ganciclovir (p = 0.008). The concomitant use of two or more nephrotoxic drugs was characterized as an independent marker of renal dysfunction (p < 0.001). After discharge from the pediatric intensive care unit, renal function monitoring in the ward was inadequate in 19.8% of cases. CONCLUSION: It is necessary for intensivist physicians to have knowledge of the main nephrotoxic drugs to predict, reduce or avoid damage to their patients.


OBJETIVO: Avaliar a associação entre uso de drogas nefrotóxicas e lesão renal aguda em pacientes pediátricos graves. MÉTODOS: Estudo de coorte retrospectivo envolvendo todas as crianças internadas na unidade de terapia intensiva de um hospital pediátrico durante o período de 1 ano. A lesão renal aguda foi definida pela classificação KDIGO. Foram incluídos pacientes com tempo de internação maior que 48 horas e idade entre 1 mês e 14 anos. Foram excluídos aqueles com nefropatia aguda ou crônica, uropatia, cardiopatia congênita ou adquirida, uso crônico de drogas nefrotóxicas, rabdomiólise e síndrome de lise tumoral. Os pacientes foram classificados quanto ao uso de drogas nefrotóxicas durante internação na unidade de terapia intensiva pediátrica. RESULTADOS: A amostra foi composta por 226 crianças, sendo que 37,1% fizeram uso de drogas nefrotóxicas, 42,4% desenvolveram lesão renal aguda e 7,5% morreram. As medicações que, isoladamente, apresentaram associação com lesão renal aguda foram aciclovir (p < 0,001), vancomicina (p < 0,001), furosemida (p < 0,001) e ganciclovir (p = 0,008). O uso concomitante de duas ou mais drogas nefrotóxicas foi caracterizado como marcador independente de disfunção renal (p < 0,001). Após alta da unidade de terapia intensiva pediátrica, o acompanhamento da função renal na enfermaria foi inadequado em 19,8% dos casos. CONCLUSÃO: É necessário que o médico intensivista tenha conhecimento das principais drogas nefrotóxicas, de modo a prever, reduzir ou evitar danos a seus pacientes.


Subject(s)
Acute Kidney Injury , Pharmaceutical Preparations , Acute Kidney Injury/chemically induced , Acute Kidney Injury/epidemiology , Child , Critical Illness , Humans , Infant , Intensive Care Units, Pediatric , Retrospective Studies , Risk Factors
12.
Kidney Int Rep ; 4(9): 1271-1284, 2019 Sep.
Article in English | MEDLINE | ID: mdl-31517146

ABSTRACT

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of renal failure. For several decades, ADPKD was regarded as an adult-onset disease. In the past decade, it has become more widely appreciated that the disease course begins in childhood. However, evidence-based guidelines on how to manage and approach children diagnosed with or at risk of ADPKD are lacking. Also, scoring systems to stratify patients into risk categories have been established only for adults. Overall, there are insufficient data on the clinical course during childhood. We therefore initiated the global ADPedKD project to establish a large international pediatric ADPKD cohort for deep characterization. METHODS: Global ADPedKD is an international multicenter observational study focusing on childhood-diagnosed ADPKD. This collaborative project is based on interoperable Web-based databases, comprising 7 regional and independent but uniformly organized chapters, namely Africa, Asia, Australia, Europe, North America, South America, and the United Kingdom. In the database, a detailed basic data questionnaire, including genetics, is used in combination with data entry from follow-up visits, to provide both retrospective and prospective longitudinal data on clinical, radiologic, and laboratory findings, as well as therapeutic interventions. DISCUSSION: The global ADPedKD initiative aims to characterize in detail the most extensive international pediatric ADPKD cohort reported to date, providing evidence for the development of unified diagnostic, follow-up, and treatment recommendations regarding modifiable disease factors. Moreover, this registry will serve as a platform for the development of clinical and/or biochemical markers predicting the risk of early and progressive disease.

13.
Arq. bras. cardiol ; 108(5): 452-457, May 2017. tab, graf
Article in English | LILACS | ID: biblio-838731

ABSTRACT

Abstract Background: The increased carotid intima-media thickness (CIMT) correlates with the presence of atherosclerosis in adults and describes vascular abnormalities in both hypertensive children and adolescents. Objective: To assess CIMT as an early marker of atherosclerosis and vascular damage in hypertensive children and adolescents compared with non-hypertensive controls and to evaluate the influence of gender, age, and body mass index (BMI) on CIMT on each group. Methods: Observational cohort study. A total of 133 hypertensive subjects (male, n = 69; mean age, 10.5 ± 4 years) underwent carotid ultrasound exam for assessment of CIMT. One hundred and twenty-one non-hypertensive subjects (male, n = 64; mean age, 9.8 ± 4.1 years) were selected as controls for gender, age (± 1 year), and BMI (± 10%). Results: There were no significant difference regarding gender (p = 0.954) and age (p = 0.067) between groups. Hypertensive subjects had higher BMI when compared to control group (p = 0.004), although within the established range of 10%. Subjects in the hypertensive group had higher CIMT values when compared to control group (0.46 ± 0.05 versus 0.42 ± 0.05 mm, respectively, p < 0.001; one-way ANOVA). Carotid IMT values were not significantly influenced by gender, age, and BMI when analyzed in both groups separately (Student's t-test for independent samples). According to the adjusted determination coefficient (R²) only 11.7% of CIMT variations were accounted for by group variations, including age, gender, and BMI. Conclusions: Carotid intima-media thickness was higher in hypertensive children and adolescents when compared to the control group. The presence of hypertension increased CIMT regardless of age, gender, and BMI.


Resumo Fundamento: O aumento da espessura médio-intimal carotídea (EMIC) correlaciona-se com a presença de aterosclerose em adultos e descreve anormalidades vasculares em crianças e adolescentes hipertensos. Objetivo: Avaliar a EMIC como marcador precoce de aterosclerose e dano vascular em crianças e adolescentes hipertensos em comparação com um grupo controle e avaliar a influência do sexo, idade e índice de massa corporal (IMC) sobre a EMIC em cada grupo. Métodos: Estudo observacional de coorte. Um total de 133 indivíduos hipertensos (sexo masculino, n = 69; idade média 10.5 ± 4 anos) foi submetido à ultrassonografia das artérias carótidas para avaliação da EMIC. Cento e vinte e um indivíduos saudáveis (sexo masculino, n = 64; idade média, 9.8 ± 4.1 anos) foram selecionados como controles para as seguintes características: sexo, idade (± 1ano) e IMC (±10%). Resultados: Não houve diferenças significativas entre os grupos com relação ao sexo (p = 0,954) e idade (p = 0,067). Os indivíduos hipertensos apresentaram maior IMC (p = 0,004), porém dentro da faixa estabelecida de até 10%. Os indivíduos hipertensos apresentaram maiores valores de EMIC quando comparados ao grupo-controle (0,46 ± 0,05 versus 0,42 ± 0,05 mm, respectivamente, p < 0.001; ANOVA com um parâmetro). Os valores da EMIC não foram influenciados por sexo, idade e IMC quando analisados em ambos os grupos separadamente (Teste t de Student para amostras independentes). De acordo com o coeficiente de determinação (R²) ajustado, apenas 11.7% das variações da EMIC são devidas às variações em cada grupo, incluindo idade, sexo e IMC. Conclusões: A espessura médio-intimal das carótidas apresentou-se aumentada em crianças e adolescentes hipertensos quando comparados ao grupo controle. A presença de hipertensão aumentou a EMIC independentemente de idade, sexo e IMC.


Subject(s)
Humans , Male , Female , Child , Adolescent , Atherosclerosis/diagnostic imaging , Carotid Intima-Media Thickness , Hypertension/diagnostic imaging , Body Mass Index , Sex Factors , Cohort Studies , Age Factors
14.
Arq Bras Cardiol ; 108(5): 452-457, 2017 05.
Article in English, Portuguese | MEDLINE | ID: mdl-28444064

ABSTRACT

Background: The increased carotid intima-media thickness (CIMT) correlates with the presence of atherosclerosis in adults and describes vascular abnormalities in both hypertensive children and adolescents. Objective: To assess CIMT as an early marker of atherosclerosis and vascular damage in hypertensive children and adolescents compared with non-hypertensive controls and to evaluate the influence of gender, age, and body mass index (BMI) on CIMT on each group. Methods: Observational cohort study. A total of 133 hypertensive subjects (male, n = 69; mean age, 10.5 ± 4 years) underwent carotid ultrasound exam for assessment of CIMT. One hundred and twenty-one non-hypertensive subjects (male, n = 64; mean age, 9.8 ± 4.1 years) were selected as controls for gender, age (± 1 year), and BMI (± 10%). Results: There were no significant difference regarding gender (p = 0.954) and age (p = 0.067) between groups. Hypertensive subjects had higher BMI when compared to control group (p = 0.004), although within the established range of 10%. Subjects in the hypertensive group had higher CIMT values when compared to control group (0.46 ± 0.05 versus 0.42 ± 0.05 mm, respectively, p < 0.001; one-way ANOVA). Carotid IMT values were not significantly influenced by gender, age, and BMI when analyzed in both groups separately (Student's t-test for independent samples). According to the adjusted determination coefficient (R²) only 11.7% of CIMT variations were accounted for by group variations, including age, gender, and BMI. Conclusions: Carotid intima-media thickness was higher in hypertensive children and adolescents when compared to the control group. The presence of hypertension increased CIMT regardless of age, gender, and BMI.


Fundamento: O aumento da espessura médio-intimal carotídea (EMIC) correlaciona-se com a presença de aterosclerose em adultos e descreve anormalidades vasculares em crianças e adolescentes hipertensos. Objetivo: Avaliar a EMIC como marcador precoce de aterosclerose e dano vascular em crianças e adolescentes hipertensos em comparação com um grupo controle e avaliar a influência do sexo, idade e índice de massa corporal (IMC) sobre a EMIC em cada grupo. Métodos: Estudo observacional de coorte. Um total de 133 indivíduos hipertensos (sexo masculino, n = 69; idade média 10.5 ± 4 anos) foi submetido à ultrassonografia das artérias carótidas para avaliação da EMIC. Cento e vinte e um indivíduos saudáveis (sexo masculino, n = 64; idade média, 9.8 ± 4.1 anos) foram selecionados como controles para as seguintes características: sexo, idade (± 1ano) e IMC (±10%). Resultados: Não houve diferenças significativas entre os grupos com relação ao sexo (p = 0,954) e idade (p = 0,067). Os indivíduos hipertensos apresentaram maior IMC (p = 0,004), porém dentro da faixa estabelecida de até 10%. Os indivíduos hipertensos apresentaram maiores valores de EMIC quando comparados ao grupo-controle (0,46 ± 0,05 versus 0,42 ± 0,05 mm, respectivamente, p < 0.001; ANOVA com um parâmetro). Os valores da EMIC não foram influenciados por sexo, idade e IMC quando analisados em ambos os grupos separadamente (Teste t de Student para amostras independentes). De acordo com o coeficiente de determinação (R²) ajustado, apenas 11.7% das variações da EMIC são devidas às variações em cada grupo, incluindo idade, sexo e IMC. Conclusões: A espessura médio-intimal das carótidas apresentou-se aumentada em crianças e adolescentes hipertensos quando comparados ao grupo controle. A presença de hipertensão aumentou a EMIC independentemente de idade, sexo e IMC.


Subject(s)
Atherosclerosis/diagnostic imaging , Carotid Intima-Media Thickness , Hypertension/diagnostic imaging , Adolescent , Age Factors , Body Mass Index , Child , Cohort Studies , Female , Humans , Male , Sex Factors
15.
Arq. bras. cardiol ; 106(4): 327-332, Apr. 2016. tab, graf
Article in English | LILACS | ID: lil-780788

ABSTRACT

Abstract Background: Carotid intima-media thickness (CIMT) has been shown to be increased in children and adolescents with traditional cardiovascular risk factors such as obesity, hypertension, and chronic kidney disease, compared with those of healthy children. Objective: To assess the influence of sex, age and body mass index (BMI) on the CIMT in healthy children and adolescents aged 1 to 15 years. Methods: A total of 280 healthy children and adolescents (males, n=175; mean age, 7.49±3.57 years; mean BMI, 17.94±4.1 kg/m2) were screened for CIMT assessment. They were divided into 3 groups according to age: GI, 1 to 5 years [n=93 (33.2%); males, 57; mean BMI, 16±3 kg/m2]; GII, 6 to 10 years [n=127 (45.4%); males, 78; mean BMI, 17.9±3.7 kg/m2], and GIII, 11 to 15 years [n=60 (21.4%); males, 40; mean BMI, 20.9±4.5 kg/m2]. Results: There was no significant difference in CIMT values between male and female children and adolescents (0.43±0.06 mm vs. 0.42±0.05 mm, respectively; p=0.243). CIMT correlated with BMI neither in the total population nor in the 3 age groups according to Pearson correlation coefficient. Subjects aged 11 to 15 years had the highest CIMT values (GI vs. GII, p=0.615; GI vs. GIII, p=0.02; GII vs. GIII, p=0.004). Conclusions: CIMT is constant in healthy children younger than 10 years, regardless of sex or BMI. CIMT increases after the age of 10 years.


Resumo Fundamentos: A espessura médio-intimal (EMI) na artéria carótida comum tem se mostrado aumentada em crianças e adolescentes com fatores de risco tradicionais, como obesidade, hipertensão e doença renal crônica, quando comparada à de crianças saudáveis. Objetivos: Avaliar a influência do sexo, idade e índice de massa corpórea (IMC) sobre a EMI em crianças e adolescentes saudáveis entre 1 e 15 anos de idade. Métodos: Este estudo incluiu 280 indivíduos saudáveis (sexo masculino, n=175; idade, 7,49±3,57 anos; IMC, 17,94±4,1 kg/m2), que foram divididos em 3 grupos de acordo com a faixa etária: GI, crianças de 1 a 5 anos [n=93 (33,2%); sexo masculino, 57; IMC, 16±3 kg/m2]; GII, crianças de 6 a 10 anos [n=127 (45,4%); sexo masculino, 78; IMC, 17,9±3,7 kg/m2]; e GIII, crianças de 11 a 15 anos [n=60 (21,4%); sexo masculino, 40; IMC, 20,9±4,5 kg/m2]. Resultados: Não houve diferença significativa nos valores da EMI entre os sexos (masculino, 0,43±0,06 mm; feminino, 0,42±0.05 mm; p=0,243). A EMI não se correlacionou com o IMC na população total ou nos 3 grupos de acordo com o coeficiente de correlação de Pearson. Crianças entre 11 e 15 anos apresentaram maiores valores de EMI (GI vs. GII, p=0,615; GI vs. GIII, p=0,02; GII vs. GIII, p=0,004). Conclusões: Independentemente de sexo e IMC, a EMI é constante em crianças saudáveis abaixo dos 10 anos de idade, aumentando a partir daí.


Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Carotid Arteries/anatomy & histology , Carotid Intima-Media Thickness , Reference Values , Cardiovascular Diseases/etiology , Carotid Arteries/diagnostic imaging , Body Mass Index , Linear Models , Sex Factors , Risk Factors , Analysis of Variance , Age Factors , Hypertension/complications , Obesity/complications
16.
Clin Kidney J ; 9(2): 295-302, 2016 Apr.
Article in English | MEDLINE | ID: mdl-26985383

ABSTRACT

BACKGROUND: The aim of this study was to describe the experience of pediatric and young adult hemodialysis (HD) patients from a global cohort. METHODS: The Pediatric Investigation and Close Collaborative Consortium for Ongoing Life Outcomes for MONitoring Dialysis Outcomes (PICCOLO MONDO) study provided de-identified electronic information of 3244 patients, ages 0-30 years from 2000 to 2012 in four regions: Asia, Europe, North America and South America. The study sample was categorized into pediatric (≤18 years old) and young adult (19-30 years old) groups based on the age at dialysis initiation. RESULTS: For those with known end-stage renal disease etiology, glomerular disease was the most common diagnosis in children and young adults. Using Europe as a reference group, North America [odds ratio (OR) 2.69; CI 1.29, 5.63] and South America (OR 4.21; CI 2.32, 7.63) had the greatest mortality among young adults. North America also had higher rates of overweight, obesity, hypertension, cardiovascular disease, hospitalizations and secondary diabetes compared with all other regions. Initial catheter use was greater for North American (86.4% in pediatric patients and 75.2% in young adults) and South America (80.6% in pediatric patients and 75.9% in young adults). Catheter use at 1-year follow-up was most common in North American children (77.3%) and young adults (62.9%). Asia had the lowest rate of catheter use. For both age groups, dialysis adequacy (equilibrated Kt/V) ranged between 1.4 and 1.5. In Asia, patients in both age groups had significantly longer treatment times than in any other region. CONCLUSIONS: The PICCOLO MONDO study has provided unique baseline and 1-year follow-up information on children and young adults receiving HD around the globe. This cohort has brought to light aspects of care in these age groups that warrant further investigation.

17.
Arq Bras Cardiol ; 106(4): 327-32, 2016 Apr.
Article in English, Portuguese | MEDLINE | ID: mdl-26959401

ABSTRACT

BACKGROUND: Carotid intima-media thickness (CIMT) has been shown to be increased in children and adolescents with traditional cardiovascular risk factors such as obesity, hypertension, and chronic kidney disease, compared with those of healthy children. OBJECTIVE: To assess the influence of sex, age and body mass index (BMI) on the CIMT in healthy children and adolescents aged 1 to 15 years. METHODS: A total of 280 healthy children and adolescents (males, n=175; mean age, 7.49±3.57 years; mean BMI, 17.94±4.1 kg/m2) were screened for CIMT assessment. They were divided into 3 groups according to age: GI, 1 to 5 years [n=93 (33.2%); males, 57; mean BMI, 16±3 kg/m2]; GII, 6 to 10 years [n=127 (45.4%); males, 78; mean BMI, 17.9±3.7 kg/m2], and GIII, 11 to 15 years [n=60 (21.4%); males, 40; mean BMI, 20.9±4.5 kg/m2]. RESULTS: There was no significant difference in CIMT values between male and female children and adolescents (0.43±0.06 mm vs. 0.42±0.05 mm, respectively; p=0.243). CIMT correlated with BMI neither in the total population nor in the 3 age groups according to Pearson correlation coefficient. Subjects aged 11 to 15 years had the highest CIMT values (GI vs. GII, p=0.615; GI vs. GIII, p=0.02; GII vs. GIII, p=0.004). CONCLUSIONS: CIMT is constant in healthy children younger than 10 years, regardless of sex or BMI. CIMT increases after the age of 10 years.


Subject(s)
Carotid Arteries/anatomy & histology , Carotid Intima-Media Thickness , Adolescent , Age Factors , Analysis of Variance , Body Mass Index , Cardiovascular Diseases/etiology , Carotid Arteries/diagnostic imaging , Child , Child, Preschool , Female , Humans , Hypertension/complications , Infant , Linear Models , Male , Obesity/complications , Reference Values , Risk Factors , Sex Factors
18.
Int J Cardiol Heart Vasc ; 9: 48-51, 2015 Dec 07.
Article in English | MEDLINE | ID: mdl-28785705

ABSTRACT

AIM: To assess the effects of each traditional cardiovascular risk factor (hypertension, diabetes mellitus, dyslipidemia, and smoking), including the presence of coronary artery disease (CAD), on carotid intima-media thickness (CIMT) and to assess the degree of carotid plaque occurrence. METHODS: A total of 553 outpatients (216 men and 337 women; mean age 67.06 ± 12.44 years) who underwent a carotid artery ultrasound were screened for carotid plaque and CIMT measurements. RESULTS: The CIMT medians were higher in males (P < .001) and in patients with hypertension (P < .001). A linear increase occurred in mean CIMT of 0.0059 mm for each year of increase in age. The presence of plaque indicated a tendency to correlate with CIMT (P = .067). The presence of hypertension associated with diabetes (P = .0061; estimated difference 0.0494 mm) or dyslipidemia (P = .0016; estimated difference 0.0472 mm) or CAD (P = .0043; estimated difference 0.0527 mm) increased the mean CIMT measurements. The probability of plaque occurrence in carotid arteries is influenced by the age (P < .001) and is higher in patients with dyslipidemia (P = .008) and CAD (P < .001). CONCLUSIONS: Hypertension is the strongest cardiovascular risk factor that increases CIMT, followed by age and male sex. Age and dyslipidemia increase the probability of carotid plaque. Increased CIMT and plaque could be present in the same patient caused by different risk factors and with independent effects on the artery wall and different clinical prognoses.

19.
Int Tinnitus J ; 18(2): 149-55, 2013.
Article in English | MEDLINE | ID: mdl-25773108

ABSTRACT

INTRODUCTION: Chronic kidney disease is a slowly progressive disease that causes irreversible loss of renal function and is considered a public health problem worldwide. OBJECTIVE: To evaluate the vestibular behavior in patients with chronic kidney disease undergoing renal transplantation. METHODS: A retrospective cross-sectional study was performed. Thirty patients were evaluated, 33.3% female and 66.7% male (mean age 16.9 (± 3.6) years old). Patients underwent the following procedures: anamnesis, ENT (ear, nose, and throat) evaluation and vestibular evaluation. RESULTS: The patients reported dizziness when they were on dialysis. 50% the patients showed an abnormality in the vestibular test, which occurred in the caloric test. The abnormality was more prevalent in the peripheral vestibular system and there was a predominance of deficit peripheral vestibular disorders. CONCLUSION: The dizziness was the most significant symptom for the vestibular test in correlation with neurotological symptoms. Alteration in the vestibular exam occurred in the caloric test, there was a prevalence of alterations for the peripheral vestibular system with a predominance of deficit vestibular dysfunction. We emphasize the need to show professionals involved in patients with chronic kidney disease, those undergoing renal transplant and dialitic treatment the importance of prevention and early identification of otoneurological involvement.

20.
Codas ; 25(3): 202-8, 2013.
Article in English, Portuguese | MEDLINE | ID: mdl-24408329

ABSTRACT

PURPOSE: To investigate the auditory behavior of patients with chronic renal failure (CRF) undergoing kidney transplantation. METHODS: Thirty patients were evaluated, 10 (33.33%) females and 20 (66.67%) males, aging from 13 to 26 years (average, 16.97 years; standard deviation, 3.60 years). Patients underwent the following procedures: anamnesis, otolaryngological examination, audiological evaluation (pure tone and high frequency), acoustic impedance measurements and central auditory processing evaluation. A control group was used to compare the high-frequency audiometry results. RESULTS: The following observations were made: absence of auditory complaints at the time of anamnesis; pure-tone audiometry was predominantly normal; patients presented lower hearing levels at the high-frequency audiometry, when compared to the control group, and as for the acoustic impedance measurements, curves of the type A were predominant; there was a change of the central auditory processing for 14 patients (46.67%) in the Staggered Spondaic Word Test (SSW); there was a significant difference between the age variable and the result of the pure-tone audiometry, that is, hearing sensitivity in thresholds from 250Hz to 8,000Hz decreased with advancing age; and the relation between the type of donor and the SSW test result was significant. Rates were higher when the patients had been transplanted from deceased donors compared to living donors. CONCLUSION: There were no changes in conventional audiological and high-frequency evaluation, or in the central auditory processing. Professionals involved in the care of kidney transplantation recipients must be better informed about the care, prevention, and early identification of auditory disorders.


Subject(s)
Hearing Disorders/etiology , Kidney Failure, Chronic/complications , Kidney Transplantation , Adolescent , Adult , Audiometry , Auditory Threshold , Case-Control Studies , Female , Hearing Tests , Humans , Kidney Failure, Chronic/physiopathology , Male , Young Adult
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