Maria Goretti M G Penido (original) (raw)
Papers by Maria Goretti M G Penido
Journal of Nephrology, Nov 2, 2023
The mineral and bone disease of chronic kidney disease (CKD-MBD) is a combination of three compon... more The mineral and bone disease of chronic kidney disease (CKD-MBD) is a combination of three components: abnormalities in calcium, phosphorus, PTH, fibroblast growth factor 23 (FGF23) and vitamin D metabolism; abnormalities of bone metabolism, mineralization, volume, growth and strength; and vascular and other soft tissue calcification. During the natural course of kidney disease, there is an increase in FGF23 levels, inhibition of calcitriol production, secondary hyperparathyroidism, hypocalcemia and hyperphosphatemia. These changes have consequences on the cardiovascular and bone systems. Regarding cardiovascular disease, left ventricular hypertrophy is highlighted, associated with an increase in FGF23 and vascular calcification, directly related to hyperphosphatemia. The main types of bone disease are cystic fibrous osteitis (high turnover) and adynamic bone disease (low turnover), both of which are associated with a high risk of fracture in this population. Successful kidney transplantation (KT) does not fully correct the mineral, bone and cardiovascular abnormalities generated by CKD-MBD. In the first months after transplantation, PTH and FGF23 levels remain elevated in most patients. There is an increase in the production of calcitriol by the graft. These are the main alterations responsible for a mineral phenotype that resembles primary hyperparathyroidism, with hypercalcemia, hypophosphatemia and elevated PTH levels. Cardiovascular disease does not revert after KT and the transplant patient has a higher cardiovascular risk than the general population. In relation to bone disease, in addition to the pre-existing bone alteration, specific factors of the post-KT period add damage to the bone, especially the use of corticosteroids. The main types of bone disease in renal transplant patients are bone fracture, renal osteodystrophy, osteoporosis and osteonecrosis. CKD-MBD is a complex disease that affects patients with CKD, increasing their morbidity and mortality. KT does not fully reverse the disease and still adds other specific risk factors that make its approach challenging. This review sought to show the association between the bone mineral disease of chronic kidney disease and the risk of fractures, vascular and other tissue calcifications, graft dysfunction and kidney transplant patient mortality.
Growth failure is a marked feature in children with CKD. Kidney transplantation (KTx) is the ther... more Growth failure is a marked feature in children with CKD. Kidney transplantation (KTx) is the therapeutic option that provides the greatest benefits to the pediatric population. Considering the importance of this subject, a systematic search of the literature was carried out on the principal databases from January 2015 to December 2020. The following descriptors in health science (DECs) from the VHL portal (library virtual health) were applied: pediatric kidney transplantation (PKTx), growth and development. It was found that among the etiologies, congenital abnormalities of the kidneys and urinary tract (CAKUT) were the main causes of loss of renal function. The highest mean age was 15.52 ± 1.8 years. The type of donor was reported in only 3 studies, in 1 of which the living donor was predominant. The immunosuppression (ISS) schemes after PKTx were similar in the studies, the triple scheme with corticoid, calcineurin inhibitor and anti-proliferative being used in most of them. The use of GH did not occur in 4 of the 9 studies. We could conclude that weight and height gain after PKTx is an important outcome to be evaluated. In underdeveloped or developing countries where, in addition to chronic disease, we find nutritional and economic precariousness, it is very important to know the factors that greatly contribute to the impairment of height and weight gain of these patients. Controlled and randomized studies that find answers for the control of pediatric patients after PKTx and that can be applied in our country are desired.
Urology & Nephrology Open Access Journal, Jun 12, 2020
To report a case of a patient who presented recurrence of aHUS after discontinuing use of Eculizu... more To report a case of a patient who presented recurrence of aHUS after discontinuing use of Eculizumab.
Archives of renal diseases and management, Dec 16, 2022
Introduction: The kidneys are fundamental organs for survival and the progressive loss of their f... more Introduction: The kidneys are fundamental organs for survival and the progressive loss of their functions causes the loss of regulatory, excretory and endocrine functions, essentially affecting the entire balance of the organism. Chronic Kidney Disease (CKD) is considered a complex disease and a worldwide public health problem with the number of patients continuously increasing, even in the pediatric population. CKD often progresses to end-stage requiring Renal Replacement Therapy (RRT). Kidney transplantation is the treatment of choice to maximize the survival, growth, and development of pediatric patients, however, if dialysis is necessary, Peritoneal Dialysis (PD) is a high-quality and low-cost RRT modality preferred therapy for children and adolescents with End-Stage Kidney Disease (ESKD). In this scope, this study aimed to carry out an integrative review of the survival and the epidemiological, clinical, social and economic profile of children and adolescents with PD. Methods: It is an integrative review whose data collection was carried out between January 2019 and January 2021 following the methodology suggested by the literature, using a validated data collection instrument. The following health science descriptors (DECs) from the VHL portal (virtual health library) were used: children, adolescents, chronic peritoneal dialysis, survival and epidemiology. Results: Thirty-five studies were selected and evaluated using the Critical Appraisal Skills Program (CASP). The level of evidence of the articles was assessed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) scale. Survival and epidemiological, social, economic and clinical aspects of pediatric PD in Brazil and worldwide have been described and reviewed. Conclusions: The complexity and costs involved in the care of pediatric patients in RRT impact their survival. Mortality and morbidity are higher than in healthy children and life expectancy is considerably lower. ESKD is more severe in the pediatric population and interferes with general development, weight and height gain, regulation of mineral metabolism and causes definitive cardiovascular calcifications. Kidney transplantation is the treatment of choice to maximize the survival, growth and development of pediatric patients. However, if dialysis is necessary PD is the first-choice modality in this population. Unfortunately, data are scarce in the literature on its survival and its epidemiological, social, economic and clinical aspects.
Jornal De Pediatria, Apr 1, 2001
Objective: to analyze the clinical history and evolution of children and adolescents with IH, emp... more Objective: to analyze the clinical history and evolution of children and adolescents with IH, emphasizing some of their peculiar features. Methods: we followed 471 patients with IH at an outpatient clinic. Patients were submitted to the following protocol: abdominal X-ray, kidney and urinary tract ultrasonography; urinary ionogram, blood gas and biochemical analyses; 24-hour urine for measurement of calcium and other electrolytes and creatinine; urinalysis, urine culture and phase-contrast microscopy; second morning urine collected after fasting for measurement of calcium and creatinine. Results: at the time of diagnosis, 6% of the patients were infants, 15% preschool children, 55% school children, and 24% adolescents; 56% of them were boys. Clinical and laboratory findings were: 47% had hematuria and abdominal pain, 31% had isolated hematuria, 14% isolated abdominal pain, and 8% had urinary tract infection, nocturnal enuresis, suprapubic pain or urethralgia, or the frequency/urgency syndrome with urinary incontinence. Hypercalciuria was associated with urolithiasis in 56% of patients. There was association with hyperuricosuria in 18.5% of the cases, and hypocitraturia in 8.5% of the cases. Evolution was poor for 33% of the patients, with recurrence of nephrolithiasis, persistence of hematuria, and abdominal pain. Conclusions: IH must be diagnosed and treated with criteria in order to reduce consequences such as hematuria, abdominal pain, urinary stone formation and possible bone involvement. Signs and symptoms such as urgency and urinary incontinence, suprapubic pain and nocturnal enuresis may result from renal hyperexcretion of calcium.
Brazilian Journal of Nephrology
The covid-19 vaccine confers direct protection and reduces transmission rates of the virus and ne... more 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 saf...
Residência Pediátrica, 2023
Rare disease or an orphan disease is a disease that affects a small percentage of the population.... more Rare disease or an orphan disease is a disease that affects a small percentage of the population. Most of these diseases are present throughout the patients life, even if the symptoms do not appear immediately. They are often fatal or chronically debilitating and the impact on the quality of life of patients and their families is significant. Patients with rare diseases often experience delays in diagnosis, which can negatively impact management and delay treatment. Familial genetic testing or cascade genotyping of all newly diagnosed individuals can speed up the diagnosis of these diseases and allow more patients to be identified at a younger age. Care for patients with chronic and rare diseases is complex, mainly due to lack of knowledge about the disease, which makes accurate and early diagnosis difficult, in addition to the need to perform specific tests, which are sometimes highly complex and costly. Added to these factors are difficulties in obtaining adequate treatment when available, raising awareness of the patient and family about the disease and adherence to treatment. Residência Pediátrica; 2023: Ahead of Print. DOI: 10.25060/residpediatr-2023-1062 Nota aos leitores: pode haver informações pendentes, por se tratar de um artigo em “visualização pré-publicação”. A multidisciplinary approach is very important: care provided by a doctor, nurse, psychologist, nutritionist and social worker. These professionals, in addition to medical care, address the particularities of the disease and treatment, the impact on the life of the patient and his family, the approach to psychological and social issues, and guidance regarding medications and diets.
Urology & Nephrology Open Access Journal, Oct 23, 2020
Prevention of kidney disease must begin before a woman becomes pregnant. For this prevention, it ... more Prevention of kidney disease must begin before a woman becomes pregnant. For this prevention, it is necessary to act with doctors, health professionals, parents, schools and the community. This early prevention is important because the pathophysiology of chronic noncommunicable diseases in adults would be related to events that occur during the prenatal period and in the first year of life. In an unfavorable intrauterine environment, the embryo/ fetus undergoes adaptations resulting from epigenetics and intrinsic factors that could cause a renal maturation compromised. This is what we call fetal and perinatal programming of kidney diseases in adults. There are three types of prevention: the primary that aims to eliminate or reduce exposure to risk factors for chronic kidney disease, the secondary that eliminates or reduces exposure to risk factors and the tertiary that reduces or postpones long-term disease complications. However, the best and most effective prevention is the primary one with actions to control the future mother, the pregnant woman, the infant and older children until adolescence. The main primary prevention agents for kidney disease include the community, doctors and other health professionals, parents and caregivers, schools and day care centers. In this way, it will be possible to control the explosion of chronic kidney disease worldwide.
Jornal De Pediatria, Apr 1, 2002
Artigo submetido em 16.08.01, aceito em 12.12.01. Resumo Objetivo: determinar valores de referênc... more Artigo submetido em 16.08.01, aceito em 12.12.01. Resumo Objetivo: determinar valores de referência regionais para a excreção urinária de cálcio, ácido úrico e citrato e estabelecer correlação entre essas excreções em urina em 24h e amostras únicas, para uso na prática clínica. Material e métodos: 125 crianças e adolescentes saudáveis, selecionados aleatoriamente, foram submetidos ao protocolo: exame clínico, bioquímica de sangue, hemograma, paratormônio, urina em 24h, urina em amostra única colhida com jejum, e exame parasitológico de fezes. Resultados: o valor máximo para a excreção de cálcio em urina em 24h foi 3,75mg/kg, em mg/dl do ritmo de filtração glomerular-RFG foi 0,10, e, para a relação cálcio/creatinina(mg/dl) na urina, em amostra única em jejum, foi de 0,25. Observou-se correlação positiva entre a excreção de cálcio em urina em 24h, e urina em amostra única em jejum (mg/dl e mg/dl do RFG). Os valores máximos para a excreção de ácido úrico em urina em 24h foram 600, 450 e 320mg e 13, 15 e 18 mg/kg para adolescentes, escolares e pré-escolares, respectivamente; em mg/dl do RFG em amostra única de urina foi 0,47. Observou-se correlação positiva para a excreção de ácido úrico em urina em 24h e urina em amostra única em jejum. Os valores médios para a excreção de citrato em urina em 24h foram 1,6, 1,1 e 0,5mmol para adolescentes, escolares e pré-escolares, respectivamente; para a relação citrato/creatinina em urina, em amostra única, com jejum, foi 0,3. Conclusões: as excreções de cálcio e ácido úrico em urina em 24h se correlacionaram com aquelas das amostras urinárias simplificadas, permitindo o uso destas para diagnósticos metabólicos, estudos populacionais e controle de pacientes hipercalciúricos e hiperuricosúricos sem controle de esfíncter vesical; o quociente citrato/creatinina em urina, em amostra única, pode ser utilizado para controle de pacientes com hipocitratúria.
Pediatric Nephrology, May 3, 2012
Phosphate is one of the most abundant minerals in the body, and its serum levels are regulated by... more Phosphate is one of the most abundant minerals in the body, and its serum levels are regulated by a complex set of processes occurring in the intestine, skeleton, and kidneys. The currently known main regulators of phosphate homeostasis include parathyroid hormone (PTH), calcitriol, and a number of peptides collectively known as the "phosphatonins" of which fibroblast growth factor-23 (FGF-23) has been best defined. Maintenance of extracellular and intracellular phosphate levels within a narrow range is important for many biological processes, including energy metabolism, cell signaling, regulation of protein synthesis, skeletal development, and bone integrity. The presence of adequate amounts of phosphate is critical for the process of apoptosis of mature chondrocytes in the growth plate. Without the presence of this mineral in high enough quantities, chondrocytes will not go into apoptosis, and the normal physiological chain of events that includes invasion of blood vessels and the generation of new bone will be blocked, resulting in rickets and delayed growth. In the rest of the skeleton, hypophosphatemia will result in osteomalacia due to an insufficient formation of hydroxyapatite. This review will address phosphate metabolism and its role in bone health.
Brazilian Journal of Nephrology, Sep 24, 2021
Dengue infection in kidney transplant recipients: clinical course and its impact on renal functio... more Dengue infection in kidney transplant recipients: clinical course and its impact on renal function Infecção por dengue em receptores de transplante renal: curso clínico e seu impacto na função renal Introdução: Receptores de transplante renal (RTR) apresentam maior risco de infecção pelo vírus da dengue (DENV). O objetivo deste estudo foi delinear apresentação clínica e perfil laboratorial da infecção por DENV em RTR e seu impacto na função renal. Métodos: Este foi um estudo retrospectivo de RTR diagnosticados com infecção por DENV. Incluiu-se pacientes adultos que estiveram no Centro Nefrológico da Santa Casa de Belo Horizonte entre Abril/Setembro, 2019. Foram excluídos pacientes que não assinaram o Termo de Consentimento Livre e Esclarecido. Dados foram coletados do banco de dados e registros médicos. O estudo foi aprovado pelo Comitê de Ética Institucional local e obteve-se o Consentimento Livre e Esclarecido. Resultados: Avaliou-se dezenove RTR. Principais sinais e sintomas foram mialgia, cefaleia/dor retro-orbital, febre, sintomas gastrointestinais. Dois pacientes apresentaram colecistite aguda sem cálculo, três sofreram derrame pleural e/ou pericárdico, um desenvolveu miocardite aguda. Todos os pacientes estavam sob imunossupressão com prednisona, tacrolimus, micofenolato. A maioria não estava recebendo terapia de indução. Suspensão/redução temporária da imunossupressão foi necessária em 58% dos pacientes e leucopenia foi a razão mais comum. Trombocitopenia foi comum e 58% dos pacientes desenvolveram lesão renal aguda. Todos recuperaram a função renal. Conclusões: A infecção por DENV em pacientes RTR parece seguir um curso semelhante ao da população em geral. Embora não houvesse grupo controle, suspeitamos que imunossupressão, doença renal preexistente ou tipo de doador não foram fator determinante na maioria dos pacientes. Disfunção renal transitória foi comum, mas reversível. Nenhum paciente veio ao óbito ou sofreu perda do enxerto. Resumo Descritores: Vírus da dengue; Transplante de Rim; Imunossupressão; Disfunção Primária do Enxerto. Introduction: Kidney transplant recipients (KTR) are at increased risk for dengue virus (DENV) infection. The aim of this study was to outline the clinical presentation and laboratory profile of DENV infection in KTR and its impact on renal function. Methods: This was a retrospective study of KTR diagnosed with DENV infection. Adult patients who visited Santa Casa de Belo Horizonte Nephrology Center between April and September 2019 were included. Patients who did not sign the Informed Consent were excluded. Data were collected from the database and medical records. The study was approved by the local Institutional Ethics Committee and the Informed Consent was obtained. Results: Nineteen KTR were evaluated. The main signs and symptoms were myalgia, headache/retro-orbital pain, fever, and gastrointestinal symptoms. Two patients had acute cholecystitis without calculus, three experienced pleural and/ or pericardial effusion, and one developed acute myocarditis. All patients were under immunosuppression with prednisone, tacrolimus, and mycophenolate, and most were not receiving induction therapy. Temporary suspension/reduction of immunosuppression was required in 58% of patients and leukopenia was the most common reason. Thrombocytopenia was common and 58% of patients developed acute kidney injury. All patients recovered renal function. Conclusions: DENV infection in KTR patients seems to follow a similar course as in the general population. Although there was no control group, we suspect that immunosuppression, preexisting kidney disease or type of donor was not a determining factor in most patients. Transient renal dysfunction was common but reversible. No patient experienced death or graft loss.
InTech eBooks, Nov 14, 2011
As for readers, this license allows users to download, copy and build upon published chapters eve... more As for readers, this license allows users to download, copy and build upon published chapters even for commercial purposes, as long as the author and publisher are properly credited, which ensures maximum dissemination and a wider impact of our publications. Notice Statements and opinions expressed in the chapters are these of the individual contributors and not necessarily those of the editors or publisher. No responsibility is accepted for the accuracy of information contained in the published chapters. The publisher assumes no responsibility for any damage or injury to persons or property arising out of the use of any materials, instructions, methods or ideas contained in the book.
Brazilian Journal of Nephrology, 2020
Pediatric Nephrology, Jan 2, 2021
Jornal De Pediatria, May 1, 2020
OBJECTIVE To describe the dietary patterns and occurrence of metabolic disorders in children and ... more OBJECTIVE To describe the dietary patterns and occurrence of metabolic disorders in children and adolescents with urolithiasis treatment at a referral hospital in southern Brazil in order to learn the features of urolithiasis in this population to better develop preventive actions. METHODS Descriptive study conducted between 2016 and 2017 in a tertiary care referral hospital. Fourty patients aged 2-19 years old with urolithiasis proven by imaging were included. Clinical and dietary data were obtained through interviews and medical records. For statistical analyses, the chi-squared test was performed. RESULTS 40 individuals were analyzed. Mean age at diagnosis was 7.2±4 years. 25% were overweight or obese. 95% had metabolic disorders, hypocitraturia being the predominant type. Protein intake was adequate in all participants and carbohydrate intake, in 70% of them; 37.5% had lipid intake above recommended and 65% had low fiber intake. The mean daily sodium intake was 2.64g (±1.74), with 55% of participants ingesting more than the recommended amount. A total of 52.5% had low potassium intake, with a mean of 4.79g/day (±2.49). Calcium intake was adequate in 27.5%. No significant differences were identified in relation to mean daily consumption among participants with or without the various metabolic disorders. CONCLUSION Pediatric urolithiasis is often accompanied by metabolic disorders; therefore, metabolic evaluation should be part of the diagnostic process and subsequent analysis of these patients' dietary patterns, helping to optimize treatment and prevent recurrences and complications.
Jornal De Pediatria, Mar 15, 2002
Artigo submetido em 16.08.01, aceito em 12.12.01. Resumo Objetivo: determinar valores de referênc... more Artigo submetido em 16.08.01, aceito em 12.12.01. Resumo Objetivo: determinar valores de referência regionais para a excreção urinária de cálcio, ácido úrico e citrato e estabelecer correlação entre essas excreções em urina em 24h e amostras únicas, para uso na prática clínica. Material e métodos: 125 crianças e adolescentes saudáveis, selecionados aleatoriamente, foram submetidos ao protocolo: exame clínico, bioquímica de sangue, hemograma, paratormônio, urina em 24h, urina em amostra única colhida com jejum, e exame parasitológico de fezes. Resultados: o valor máximo para a excreção de cálcio em urina em 24h foi 3,75mg/kg, em mg/dl do ritmo de filtração glomerular-RFG foi 0,10, e, para a relação cálcio/creatinina(mg/dl) na urina, em amostra única em jejum, foi de 0,25. Observou-se correlação positiva entre a excreção de cálcio em urina em 24h, e urina em amostra única em jejum (mg/dl e mg/dl do RFG). Os valores máximos para a excreção de ácido úrico em urina em 24h foram 600, 450 e 320mg e 13, 15 e 18 mg/kg para adolescentes, escolares e pré-escolares, respectivamente; em mg/dl do RFG em amostra única de urina foi 0,47. Observou-se correlação positiva para a excreção de ácido úrico em urina em 24h e urina em amostra única em jejum. Os valores médios para a excreção de citrato em urina em 24h foram 1,6, 1,1 e 0,5mmol para adolescentes, escolares e pré-escolares, respectivamente; para a relação citrato/creatinina em urina, em amostra única, com jejum, foi 0,3. Conclusões: as excreções de cálcio e ácido úrico em urina em 24h se correlacionaram com aquelas das amostras urinárias simplificadas, permitindo o uso destas para diagnósticos metabólicos, estudos populacionais e controle de pacientes hipercalciúricos e hiperuricosúricos sem controle de esfíncter vesical; o quociente citrato/creatinina em urina, em amostra única, pode ser utilizado para controle de pacientes com hipocitratúria.
Brazilian Journal of Nephrology, 2016
Introduction: Pediatric urolithiasis has become more prevalent in recent decades, with high recur... more Introduction: Pediatric urolithiasis has become more prevalent in recent decades, with high recurrence rates and considerable morbidity. Most children with idiopathic urolithiasis have an underlying metabolic abnormality and proper research provides therapeutic interventions to reduce the formation of new stones and its complications. Objective: To identify demographic and clinical characteristics of pediatric urolithiasis, etiology, treatment management, disease recurrence and patient outcomes in a tertiary care pediatric hospital. Methods: A retrospective descriptive study of pediatric patients admitted to the Hospital Infantil Joana de Gusmão in Florianópolis, SC, Brazil, who were diagnosed with urolithiasis, from January 2002 to December 2012. Data were obtained from medical records. Those patients with diagnosis confirmed by imaging and 24hr urine or single sample urine were included. Results: We evaluated 106 pediatric patients (65% M). Average age at diagnosis was 8.0 ± 4.2 and 85% of them had positive family history of urolithiasis. Abdominal pain, renal colic and urinary tract infection were the main manifestations. 93.2% had metabolic abnormality and hypercalciuria was the most common. Pharmacological treatment was established in 78% of cases. Potassium citrate and hydrochlorothiazide were used. Surgical treatment was performed in 38% of patients. There was response to treatment in 39% of patients with recurrence of urolithiasis in 34.2% of them. Only 4.7% of patients continued follow-up, 6.6% were referred to other services, 8.5% were discharged and 73.8% lost follow-up. Conclusion: Pediatric urolithiasis deserves a detailed metabolic evaluation after their initial presentation for treatment, monitoring and prevention of its formation and its complications.
Pediatric Nephrology, Apr 13, 2012
World journal of nephrology, 2016
contributed equally to the conception and design of the study, the acquisition, analysis and inte... more contributed equally to the conception and design of the study, the acquisition, analysis and interpretation of data, and the drafting and critical revision of the article; Souza BN, Leite AF and de Deus LMC performed the research and analyzed the data.
Journal of Nephrology, Nov 2, 2023
The mineral and bone disease of chronic kidney disease (CKD-MBD) is a combination of three compon... more The mineral and bone disease of chronic kidney disease (CKD-MBD) is a combination of three components: abnormalities in calcium, phosphorus, PTH, fibroblast growth factor 23 (FGF23) and vitamin D metabolism; abnormalities of bone metabolism, mineralization, volume, growth and strength; and vascular and other soft tissue calcification. During the natural course of kidney disease, there is an increase in FGF23 levels, inhibition of calcitriol production, secondary hyperparathyroidism, hypocalcemia and hyperphosphatemia. These changes have consequences on the cardiovascular and bone systems. Regarding cardiovascular disease, left ventricular hypertrophy is highlighted, associated with an increase in FGF23 and vascular calcification, directly related to hyperphosphatemia. The main types of bone disease are cystic fibrous osteitis (high turnover) and adynamic bone disease (low turnover), both of which are associated with a high risk of fracture in this population. Successful kidney transplantation (KT) does not fully correct the mineral, bone and cardiovascular abnormalities generated by CKD-MBD. In the first months after transplantation, PTH and FGF23 levels remain elevated in most patients. There is an increase in the production of calcitriol by the graft. These are the main alterations responsible for a mineral phenotype that resembles primary hyperparathyroidism, with hypercalcemia, hypophosphatemia and elevated PTH levels. Cardiovascular disease does not revert after KT and the transplant patient has a higher cardiovascular risk than the general population. In relation to bone disease, in addition to the pre-existing bone alteration, specific factors of the post-KT period add damage to the bone, especially the use of corticosteroids. The main types of bone disease in renal transplant patients are bone fracture, renal osteodystrophy, osteoporosis and osteonecrosis. CKD-MBD is a complex disease that affects patients with CKD, increasing their morbidity and mortality. KT does not fully reverse the disease and still adds other specific risk factors that make its approach challenging. This review sought to show the association between the bone mineral disease of chronic kidney disease and the risk of fractures, vascular and other tissue calcifications, graft dysfunction and kidney transplant patient mortality.
Growth failure is a marked feature in children with CKD. Kidney transplantation (KTx) is the ther... more Growth failure is a marked feature in children with CKD. Kidney transplantation (KTx) is the therapeutic option that provides the greatest benefits to the pediatric population. Considering the importance of this subject, a systematic search of the literature was carried out on the principal databases from January 2015 to December 2020. The following descriptors in health science (DECs) from the VHL portal (library virtual health) were applied: pediatric kidney transplantation (PKTx), growth and development. It was found that among the etiologies, congenital abnormalities of the kidneys and urinary tract (CAKUT) were the main causes of loss of renal function. The highest mean age was 15.52 ± 1.8 years. The type of donor was reported in only 3 studies, in 1 of which the living donor was predominant. The immunosuppression (ISS) schemes after PKTx were similar in the studies, the triple scheme with corticoid, calcineurin inhibitor and anti-proliferative being used in most of them. The use of GH did not occur in 4 of the 9 studies. We could conclude that weight and height gain after PKTx is an important outcome to be evaluated. In underdeveloped or developing countries where, in addition to chronic disease, we find nutritional and economic precariousness, it is very important to know the factors that greatly contribute to the impairment of height and weight gain of these patients. Controlled and randomized studies that find answers for the control of pediatric patients after PKTx and that can be applied in our country are desired.
Urology & Nephrology Open Access Journal, Jun 12, 2020
To report a case of a patient who presented recurrence of aHUS after discontinuing use of Eculizu... more To report a case of a patient who presented recurrence of aHUS after discontinuing use of Eculizumab.
Archives of renal diseases and management, Dec 16, 2022
Introduction: The kidneys are fundamental organs for survival and the progressive loss of their f... more Introduction: The kidneys are fundamental organs for survival and the progressive loss of their functions causes the loss of regulatory, excretory and endocrine functions, essentially affecting the entire balance of the organism. Chronic Kidney Disease (CKD) is considered a complex disease and a worldwide public health problem with the number of patients continuously increasing, even in the pediatric population. CKD often progresses to end-stage requiring Renal Replacement Therapy (RRT). Kidney transplantation is the treatment of choice to maximize the survival, growth, and development of pediatric patients, however, if dialysis is necessary, Peritoneal Dialysis (PD) is a high-quality and low-cost RRT modality preferred therapy for children and adolescents with End-Stage Kidney Disease (ESKD). In this scope, this study aimed to carry out an integrative review of the survival and the epidemiological, clinical, social and economic profile of children and adolescents with PD. Methods: It is an integrative review whose data collection was carried out between January 2019 and January 2021 following the methodology suggested by the literature, using a validated data collection instrument. The following health science descriptors (DECs) from the VHL portal (virtual health library) were used: children, adolescents, chronic peritoneal dialysis, survival and epidemiology. Results: Thirty-five studies were selected and evaluated using the Critical Appraisal Skills Program (CASP). The level of evidence of the articles was assessed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) scale. Survival and epidemiological, social, economic and clinical aspects of pediatric PD in Brazil and worldwide have been described and reviewed. Conclusions: The complexity and costs involved in the care of pediatric patients in RRT impact their survival. Mortality and morbidity are higher than in healthy children and life expectancy is considerably lower. ESKD is more severe in the pediatric population and interferes with general development, weight and height gain, regulation of mineral metabolism and causes definitive cardiovascular calcifications. Kidney transplantation is the treatment of choice to maximize the survival, growth and development of pediatric patients. However, if dialysis is necessary PD is the first-choice modality in this population. Unfortunately, data are scarce in the literature on its survival and its epidemiological, social, economic and clinical aspects.
Jornal De Pediatria, Apr 1, 2001
Objective: to analyze the clinical history and evolution of children and adolescents with IH, emp... more Objective: to analyze the clinical history and evolution of children and adolescents with IH, emphasizing some of their peculiar features. Methods: we followed 471 patients with IH at an outpatient clinic. Patients were submitted to the following protocol: abdominal X-ray, kidney and urinary tract ultrasonography; urinary ionogram, blood gas and biochemical analyses; 24-hour urine for measurement of calcium and other electrolytes and creatinine; urinalysis, urine culture and phase-contrast microscopy; second morning urine collected after fasting for measurement of calcium and creatinine. Results: at the time of diagnosis, 6% of the patients were infants, 15% preschool children, 55% school children, and 24% adolescents; 56% of them were boys. Clinical and laboratory findings were: 47% had hematuria and abdominal pain, 31% had isolated hematuria, 14% isolated abdominal pain, and 8% had urinary tract infection, nocturnal enuresis, suprapubic pain or urethralgia, or the frequency/urgency syndrome with urinary incontinence. Hypercalciuria was associated with urolithiasis in 56% of patients. There was association with hyperuricosuria in 18.5% of the cases, and hypocitraturia in 8.5% of the cases. Evolution was poor for 33% of the patients, with recurrence of nephrolithiasis, persistence of hematuria, and abdominal pain. Conclusions: IH must be diagnosed and treated with criteria in order to reduce consequences such as hematuria, abdominal pain, urinary stone formation and possible bone involvement. Signs and symptoms such as urgency and urinary incontinence, suprapubic pain and nocturnal enuresis may result from renal hyperexcretion of calcium.
Brazilian Journal of Nephrology
The covid-19 vaccine confers direct protection and reduces transmission rates of the virus and ne... more 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 saf...
Residência Pediátrica, 2023
Rare disease or an orphan disease is a disease that affects a small percentage of the population.... more Rare disease or an orphan disease is a disease that affects a small percentage of the population. Most of these diseases are present throughout the patients life, even if the symptoms do not appear immediately. They are often fatal or chronically debilitating and the impact on the quality of life of patients and their families is significant. Patients with rare diseases often experience delays in diagnosis, which can negatively impact management and delay treatment. Familial genetic testing or cascade genotyping of all newly diagnosed individuals can speed up the diagnosis of these diseases and allow more patients to be identified at a younger age. Care for patients with chronic and rare diseases is complex, mainly due to lack of knowledge about the disease, which makes accurate and early diagnosis difficult, in addition to the need to perform specific tests, which are sometimes highly complex and costly. Added to these factors are difficulties in obtaining adequate treatment when available, raising awareness of the patient and family about the disease and adherence to treatment. Residência Pediátrica; 2023: Ahead of Print. DOI: 10.25060/residpediatr-2023-1062 Nota aos leitores: pode haver informações pendentes, por se tratar de um artigo em “visualização pré-publicação”. A multidisciplinary approach is very important: care provided by a doctor, nurse, psychologist, nutritionist and social worker. These professionals, in addition to medical care, address the particularities of the disease and treatment, the impact on the life of the patient and his family, the approach to psychological and social issues, and guidance regarding medications and diets.
Urology & Nephrology Open Access Journal, Oct 23, 2020
Prevention of kidney disease must begin before a woman becomes pregnant. For this prevention, it ... more Prevention of kidney disease must begin before a woman becomes pregnant. For this prevention, it is necessary to act with doctors, health professionals, parents, schools and the community. This early prevention is important because the pathophysiology of chronic noncommunicable diseases in adults would be related to events that occur during the prenatal period and in the first year of life. In an unfavorable intrauterine environment, the embryo/ fetus undergoes adaptations resulting from epigenetics and intrinsic factors that could cause a renal maturation compromised. This is what we call fetal and perinatal programming of kidney diseases in adults. There are three types of prevention: the primary that aims to eliminate or reduce exposure to risk factors for chronic kidney disease, the secondary that eliminates or reduces exposure to risk factors and the tertiary that reduces or postpones long-term disease complications. However, the best and most effective prevention is the primary one with actions to control the future mother, the pregnant woman, the infant and older children until adolescence. The main primary prevention agents for kidney disease include the community, doctors and other health professionals, parents and caregivers, schools and day care centers. In this way, it will be possible to control the explosion of chronic kidney disease worldwide.
Jornal De Pediatria, Apr 1, 2002
Artigo submetido em 16.08.01, aceito em 12.12.01. Resumo Objetivo: determinar valores de referênc... more Artigo submetido em 16.08.01, aceito em 12.12.01. Resumo Objetivo: determinar valores de referência regionais para a excreção urinária de cálcio, ácido úrico e citrato e estabelecer correlação entre essas excreções em urina em 24h e amostras únicas, para uso na prática clínica. Material e métodos: 125 crianças e adolescentes saudáveis, selecionados aleatoriamente, foram submetidos ao protocolo: exame clínico, bioquímica de sangue, hemograma, paratormônio, urina em 24h, urina em amostra única colhida com jejum, e exame parasitológico de fezes. Resultados: o valor máximo para a excreção de cálcio em urina em 24h foi 3,75mg/kg, em mg/dl do ritmo de filtração glomerular-RFG foi 0,10, e, para a relação cálcio/creatinina(mg/dl) na urina, em amostra única em jejum, foi de 0,25. Observou-se correlação positiva entre a excreção de cálcio em urina em 24h, e urina em amostra única em jejum (mg/dl e mg/dl do RFG). Os valores máximos para a excreção de ácido úrico em urina em 24h foram 600, 450 e 320mg e 13, 15 e 18 mg/kg para adolescentes, escolares e pré-escolares, respectivamente; em mg/dl do RFG em amostra única de urina foi 0,47. Observou-se correlação positiva para a excreção de ácido úrico em urina em 24h e urina em amostra única em jejum. Os valores médios para a excreção de citrato em urina em 24h foram 1,6, 1,1 e 0,5mmol para adolescentes, escolares e pré-escolares, respectivamente; para a relação citrato/creatinina em urina, em amostra única, com jejum, foi 0,3. Conclusões: as excreções de cálcio e ácido úrico em urina em 24h se correlacionaram com aquelas das amostras urinárias simplificadas, permitindo o uso destas para diagnósticos metabólicos, estudos populacionais e controle de pacientes hipercalciúricos e hiperuricosúricos sem controle de esfíncter vesical; o quociente citrato/creatinina em urina, em amostra única, pode ser utilizado para controle de pacientes com hipocitratúria.
Pediatric Nephrology, May 3, 2012
Phosphate is one of the most abundant minerals in the body, and its serum levels are regulated by... more Phosphate is one of the most abundant minerals in the body, and its serum levels are regulated by a complex set of processes occurring in the intestine, skeleton, and kidneys. The currently known main regulators of phosphate homeostasis include parathyroid hormone (PTH), calcitriol, and a number of peptides collectively known as the "phosphatonins" of which fibroblast growth factor-23 (FGF-23) has been best defined. Maintenance of extracellular and intracellular phosphate levels within a narrow range is important for many biological processes, including energy metabolism, cell signaling, regulation of protein synthesis, skeletal development, and bone integrity. The presence of adequate amounts of phosphate is critical for the process of apoptosis of mature chondrocytes in the growth plate. Without the presence of this mineral in high enough quantities, chondrocytes will not go into apoptosis, and the normal physiological chain of events that includes invasion of blood vessels and the generation of new bone will be blocked, resulting in rickets and delayed growth. In the rest of the skeleton, hypophosphatemia will result in osteomalacia due to an insufficient formation of hydroxyapatite. This review will address phosphate metabolism and its role in bone health.
Brazilian Journal of Nephrology, Sep 24, 2021
Dengue infection in kidney transplant recipients: clinical course and its impact on renal functio... more Dengue infection in kidney transplant recipients: clinical course and its impact on renal function Infecção por dengue em receptores de transplante renal: curso clínico e seu impacto na função renal Introdução: Receptores de transplante renal (RTR) apresentam maior risco de infecção pelo vírus da dengue (DENV). O objetivo deste estudo foi delinear apresentação clínica e perfil laboratorial da infecção por DENV em RTR e seu impacto na função renal. Métodos: Este foi um estudo retrospectivo de RTR diagnosticados com infecção por DENV. Incluiu-se pacientes adultos que estiveram no Centro Nefrológico da Santa Casa de Belo Horizonte entre Abril/Setembro, 2019. Foram excluídos pacientes que não assinaram o Termo de Consentimento Livre e Esclarecido. Dados foram coletados do banco de dados e registros médicos. O estudo foi aprovado pelo Comitê de Ética Institucional local e obteve-se o Consentimento Livre e Esclarecido. Resultados: Avaliou-se dezenove RTR. Principais sinais e sintomas foram mialgia, cefaleia/dor retro-orbital, febre, sintomas gastrointestinais. Dois pacientes apresentaram colecistite aguda sem cálculo, três sofreram derrame pleural e/ou pericárdico, um desenvolveu miocardite aguda. Todos os pacientes estavam sob imunossupressão com prednisona, tacrolimus, micofenolato. A maioria não estava recebendo terapia de indução. Suspensão/redução temporária da imunossupressão foi necessária em 58% dos pacientes e leucopenia foi a razão mais comum. Trombocitopenia foi comum e 58% dos pacientes desenvolveram lesão renal aguda. Todos recuperaram a função renal. Conclusões: A infecção por DENV em pacientes RTR parece seguir um curso semelhante ao da população em geral. Embora não houvesse grupo controle, suspeitamos que imunossupressão, doença renal preexistente ou tipo de doador não foram fator determinante na maioria dos pacientes. Disfunção renal transitória foi comum, mas reversível. Nenhum paciente veio ao óbito ou sofreu perda do enxerto. Resumo Descritores: Vírus da dengue; Transplante de Rim; Imunossupressão; Disfunção Primária do Enxerto. Introduction: Kidney transplant recipients (KTR) are at increased risk for dengue virus (DENV) infection. The aim of this study was to outline the clinical presentation and laboratory profile of DENV infection in KTR and its impact on renal function. Methods: This was a retrospective study of KTR diagnosed with DENV infection. Adult patients who visited Santa Casa de Belo Horizonte Nephrology Center between April and September 2019 were included. Patients who did not sign the Informed Consent were excluded. Data were collected from the database and medical records. The study was approved by the local Institutional Ethics Committee and the Informed Consent was obtained. Results: Nineteen KTR were evaluated. The main signs and symptoms were myalgia, headache/retro-orbital pain, fever, and gastrointestinal symptoms. Two patients had acute cholecystitis without calculus, three experienced pleural and/ or pericardial effusion, and one developed acute myocarditis. All patients were under immunosuppression with prednisone, tacrolimus, and mycophenolate, and most were not receiving induction therapy. Temporary suspension/reduction of immunosuppression was required in 58% of patients and leukopenia was the most common reason. Thrombocytopenia was common and 58% of patients developed acute kidney injury. All patients recovered renal function. Conclusions: DENV infection in KTR patients seems to follow a similar course as in the general population. Although there was no control group, we suspect that immunosuppression, preexisting kidney disease or type of donor was not a determining factor in most patients. Transient renal dysfunction was common but reversible. No patient experienced death or graft loss.
InTech eBooks, Nov 14, 2011
As for readers, this license allows users to download, copy and build upon published chapters eve... more As for readers, this license allows users to download, copy and build upon published chapters even for commercial purposes, as long as the author and publisher are properly credited, which ensures maximum dissemination and a wider impact of our publications. Notice Statements and opinions expressed in the chapters are these of the individual contributors and not necessarily those of the editors or publisher. No responsibility is accepted for the accuracy of information contained in the published chapters. The publisher assumes no responsibility for any damage or injury to persons or property arising out of the use of any materials, instructions, methods or ideas contained in the book.
Brazilian Journal of Nephrology, 2020
Pediatric Nephrology, Jan 2, 2021
Jornal De Pediatria, May 1, 2020
OBJECTIVE To describe the dietary patterns and occurrence of metabolic disorders in children and ... more OBJECTIVE To describe the dietary patterns and occurrence of metabolic disorders in children and adolescents with urolithiasis treatment at a referral hospital in southern Brazil in order to learn the features of urolithiasis in this population to better develop preventive actions. METHODS Descriptive study conducted between 2016 and 2017 in a tertiary care referral hospital. Fourty patients aged 2-19 years old with urolithiasis proven by imaging were included. Clinical and dietary data were obtained through interviews and medical records. For statistical analyses, the chi-squared test was performed. RESULTS 40 individuals were analyzed. Mean age at diagnosis was 7.2±4 years. 25% were overweight or obese. 95% had metabolic disorders, hypocitraturia being the predominant type. Protein intake was adequate in all participants and carbohydrate intake, in 70% of them; 37.5% had lipid intake above recommended and 65% had low fiber intake. The mean daily sodium intake was 2.64g (±1.74), with 55% of participants ingesting more than the recommended amount. A total of 52.5% had low potassium intake, with a mean of 4.79g/day (±2.49). Calcium intake was adequate in 27.5%. No significant differences were identified in relation to mean daily consumption among participants with or without the various metabolic disorders. CONCLUSION Pediatric urolithiasis is often accompanied by metabolic disorders; therefore, metabolic evaluation should be part of the diagnostic process and subsequent analysis of these patients' dietary patterns, helping to optimize treatment and prevent recurrences and complications.
Jornal De Pediatria, Mar 15, 2002
Artigo submetido em 16.08.01, aceito em 12.12.01. Resumo Objetivo: determinar valores de referênc... more Artigo submetido em 16.08.01, aceito em 12.12.01. Resumo Objetivo: determinar valores de referência regionais para a excreção urinária de cálcio, ácido úrico e citrato e estabelecer correlação entre essas excreções em urina em 24h e amostras únicas, para uso na prática clínica. Material e métodos: 125 crianças e adolescentes saudáveis, selecionados aleatoriamente, foram submetidos ao protocolo: exame clínico, bioquímica de sangue, hemograma, paratormônio, urina em 24h, urina em amostra única colhida com jejum, e exame parasitológico de fezes. Resultados: o valor máximo para a excreção de cálcio em urina em 24h foi 3,75mg/kg, em mg/dl do ritmo de filtração glomerular-RFG foi 0,10, e, para a relação cálcio/creatinina(mg/dl) na urina, em amostra única em jejum, foi de 0,25. Observou-se correlação positiva entre a excreção de cálcio em urina em 24h, e urina em amostra única em jejum (mg/dl e mg/dl do RFG). Os valores máximos para a excreção de ácido úrico em urina em 24h foram 600, 450 e 320mg e 13, 15 e 18 mg/kg para adolescentes, escolares e pré-escolares, respectivamente; em mg/dl do RFG em amostra única de urina foi 0,47. Observou-se correlação positiva para a excreção de ácido úrico em urina em 24h e urina em amostra única em jejum. Os valores médios para a excreção de citrato em urina em 24h foram 1,6, 1,1 e 0,5mmol para adolescentes, escolares e pré-escolares, respectivamente; para a relação citrato/creatinina em urina, em amostra única, com jejum, foi 0,3. Conclusões: as excreções de cálcio e ácido úrico em urina em 24h se correlacionaram com aquelas das amostras urinárias simplificadas, permitindo o uso destas para diagnósticos metabólicos, estudos populacionais e controle de pacientes hipercalciúricos e hiperuricosúricos sem controle de esfíncter vesical; o quociente citrato/creatinina em urina, em amostra única, pode ser utilizado para controle de pacientes com hipocitratúria.
Brazilian Journal of Nephrology, 2016
Introduction: Pediatric urolithiasis has become more prevalent in recent decades, with high recur... more Introduction: Pediatric urolithiasis has become more prevalent in recent decades, with high recurrence rates and considerable morbidity. Most children with idiopathic urolithiasis have an underlying metabolic abnormality and proper research provides therapeutic interventions to reduce the formation of new stones and its complications. Objective: To identify demographic and clinical characteristics of pediatric urolithiasis, etiology, treatment management, disease recurrence and patient outcomes in a tertiary care pediatric hospital. Methods: A retrospective descriptive study of pediatric patients admitted to the Hospital Infantil Joana de Gusmão in Florianópolis, SC, Brazil, who were diagnosed with urolithiasis, from January 2002 to December 2012. Data were obtained from medical records. Those patients with diagnosis confirmed by imaging and 24hr urine or single sample urine were included. Results: We evaluated 106 pediatric patients (65% M). Average age at diagnosis was 8.0 ± 4.2 and 85% of them had positive family history of urolithiasis. Abdominal pain, renal colic and urinary tract infection were the main manifestations. 93.2% had metabolic abnormality and hypercalciuria was the most common. Pharmacological treatment was established in 78% of cases. Potassium citrate and hydrochlorothiazide were used. Surgical treatment was performed in 38% of patients. There was response to treatment in 39% of patients with recurrence of urolithiasis in 34.2% of them. Only 4.7% of patients continued follow-up, 6.6% were referred to other services, 8.5% were discharged and 73.8% lost follow-up. Conclusion: Pediatric urolithiasis deserves a detailed metabolic evaluation after their initial presentation for treatment, monitoring and prevention of its formation and its complications.
Pediatric Nephrology, Apr 13, 2012
World journal of nephrology, 2016
contributed equally to the conception and design of the study, the acquisition, analysis and inte... more contributed equally to the conception and design of the study, the acquisition, analysis and interpretation of data, and the drafting and critical revision of the article; Souza BN, Leite AF and de Deus LMC performed the research and analyzed the data.