RESUMO
Burosumab, a monoclonal antibody directed against the fibroblast growth factor 23 (FGF23), has been approved for the treatment of X-linked hypophosphatemia (XLH). We conducted a systematic review to compare the efficacy and safety of burosumab versus conventional therapy (phosphorus and calcitriol) on XLH treatment. After a comprehensive literature search on MEDLINE/PubMed and Embase, we found nine studies for inclusion in the analysis. Risk of bias was assessed, and a random-effects model was used to determine the effect size. Clinical, biochemical, and radiological parameters of disease severity before and after treatment were analyzed and expressed in standardized mean difference (SMD). Burosumab resulted in normalization of phosphate homeostasis with an increase in renal tubular phosphate reabsorption and significant resolution of skeletal lesions (change in Thacher's total rickets severity score SMD: -1.46, 95% confidence interval [CI]: -1.76 to -1.17, p < 0.001, improvement in deformities, and decline in serum alkaline phosphatase levels [SMD: 130.68, 95% CI: 125.26-136.1, p < 0.001)]. Conventional therapy led to similar improvements in all these parameters but to a lower degree. In adults, burosumab normalized phosphorus levels (SMD: 1.23, 95% CI: 0.98-1.47, p < 0.001) with resultant clinical improvement. Burosumab treatment was well tolerated, with only mild treatment-related adverse effects. The present review indicates a potential role for burosumab in improving rickets, deformities, and growth in children with XLH. Given its superior efficacy and safety profile, burosumab could be an effective therapeutic option in children. We suggest further studies comparing burosumab versus conventional therapy in children and adults with XLH.
Assuntos
Anticorpos Monoclonais Humanizados , Raquitismo Hipofosfatêmico Familiar , Fator de Crescimento de Fibroblastos 23 , Humanos , Raquitismo Hipofosfatêmico Familiar/tratamento farmacológico , Anticorpos Monoclonais Humanizados/uso terapêutico , Resultado do Tratamento , Calcitriol/uso terapêutico , Anticorpos Monoclonais/uso terapêutico , Fósforo/sangueRESUMO
INTRODUCTION: Metabolic bone disease of premature infants is a rare complication characterized by a lower mineral content in bone tissue. OBJECTIVE: To establish the incidence of metabolic bone disease in premature infants and to determine associated risk factors. MATERIALS AND METHOD: We conducted a descriptive prospective cohort study for one year in all newborns under 32 gestational weeks, or 1,500 g, at the Hospital Universitario de Santander to determine the incidence of metabolic bone disease. We collected demographic data and prenatal histories of the selected patients, and later, we measured serum alkaline phosphatase and serum phosphorus at the third week of birth, having as reference values for diagnosis less than 5.6 mg/dl for the first one and more than 500 UI/L for the second one. We applied statistical tools for data analysis, such as average proportions, dispersion, distribution and association measures, and binomial regression. RESULTS: From a total of 58 patients, 7 had a diagnosis of metabolic bone disease, with an incidence of 12%. The weight was reported as an independent variable for the development of the disease, being significant in children under 1,160 g, as well as prolonged parenteral nutrition for more than 24 days. When performing the multivariate analysis, low weight and short time of parenteral nutrition appeared as risk factors; in the same way, maternal age below 22 years is associated with a higher relative risk, even more than a newborn weight inferior to 1,160 g. CONCLUSION: Establishing an early intervention in patients with metabolic bone disease enhancing risk factors, such as low weight and prolonged parenteral nutrition, is critical to prevent severe complications.
Introducción. La enfermedad metabólica ósea de neonatos prematuros es una complicación poco común que se caracteriza por una disminución del contenido mineral en el hueso. Objetivo. Establecer la incidencia de la enfermedad metabólica ósea en neonatos prematuros y los factores de riesgo asociados. Materiales y métodos. Durante un año, se realizó un estudio prospectivo de cohorte, descriptivo, con todos los neonatos nacidos con menos de 32 semanas de gestación o un peso menor de 1.500 g en el Hospital Universitario de Santander. Se recolectaron datos demográficos y antecedentes prenatales de los pacientes seleccionados. A la tercera semana de nacimiento, se midieron la fosfatasa alcalina y el fósforo sérico, tomando como valores de referencia diagnóstica aquellos inferiores a 5,6 mg/dl para el primero y aquellos mayores de 500 UI/L para la segunda. Para el análisis de la información, se emplearon herramientas estadísticas, como proporciones de promedios, medidas de dispersión, distribución y asociación, y regresión binomial. Resultados. De un total de 58 pacientes, 7 tuvieron diagnóstico de enfermedad metabólica ósea, con una incidencia del 12 %. De las variables estudiadas, el peso se reportó como una variable independiente para el desarrollo de la enfermedad, significativa en aquellos neonatos con peso menor de 1.160 g, al igual que la nutrición parenteral prolongada por más de 24 días. Al hacer el análisis multivariado, La edad materna menor de 22 años representó un riesgo relativo mayor, en comparación con un peso inferior a 1.160 g. Conclusión. Se estableció la importancia de una intervención temprana en pacientes con factores de riesgo para enfermedad metabólica ósea, como bajo peso (menor de 1.160 g) y nutrición parenteral prolongada (mayor de 24 días), con el fin de prevenir complicaciones graves.
Assuntos
Doenças Ósseas Metabólicas , Humanos , Colômbia/epidemiologia , Recém-Nascido , Incidência , Doenças Ósseas Metabólicas/epidemiologia , Estudos Prospectivos , Feminino , Masculino , Fatores de Risco , Idade Gestacional , Nutrição Parenteral , Recém-Nascido Prematuro , Fosfatase Alcalina/sangue , Doenças do Prematuro/epidemiologia , Doenças do Prematuro/sangue , Hospitais Universitários , Fósforo/sangueRESUMO
We aimed to determine the knowledge of potassium and phosphorus in patients undergoing hemodialysis (HD) and to associate it with serum levels and other clinical variables. This cross-sectional study included 73 patients of both sexes, who were over 18 years old and had undergone HD for at least 3 months at Hospital Geral de Caxias do Sul, Caxias do Sul, Brazil, between January and April 2019. Knowledge of phosphorus and potassium was measured by a questionnaire composed of 16 multiple choice questions (two general, seven about phosphorus, and seven about potassium) applied by the interviewer. For each mineral, a maximum of nine points could be scored. The mean ± standard deviation of correct answers was 9.78 ± 2.99 points for all questions, being higher for potassium (6.75 ± 1.65 points) than phosphorus (4.64 ± 2.10 points; P <0.001). A positive correlation was found between specific knowledge of phosphorus and its serum levels (r = 0.305; P = 0.009), but not for potassium (r = 0.101; P = 0.395). The number of correct answers positively correlated with the level of education (r = 0.390; P = 0.001) and negatively with age (r = -0.372; P = 0.001). The HD patients had intermediate levels of knowledge of phosphorus and potassium, with greater knowledge of potassium. Patients with higher serum phosphorus levels demonstrated greater knowledge about it, whereas this pattern was not observed for potassium levels. Knowledge of phosphorus and potassium was associated with younger patients and a higher level of education.
Assuntos
Conhecimentos, Atitudes e Prática em Saúde , Fósforo , Potássio , Diálise Renal , Humanos , Feminino , Masculino , Estudos Transversais , Fósforo/sangue , Pessoa de Meia-Idade , Potássio/sangue , Adulto , Idoso , Brasil , Inquéritos e QuestionáriosRESUMO
Resumen Introducción: Aun con adecuado protocolo de desconexión de la ventilación mecánica (DVM), el procedimiento falla en 15 a 30 % de los casos. Objetivo: Evaluar la asociación entre factores de riesgo independientes y fracaso posextubación en pacientes con DVM en una unidad de cuidados intensivos. Método: Estudio de cohorte, longitudinal, prospectivo, analítico, que incluyó pacientes sometidos a ventilación mecánica por más de 24 horas y que fueron extubados. Se obtuvieron reportes preextubación de hemoglobina, albúmina, fósforo, índice cintura-cadera y puntuación SOFA. Se definió como fracaso de extubación al reinicio de la ventilación mecánica en 48 horas o menos. Resultados: Se extubaron 123 pacientes, 74 hombres (60 %); la edad promedio fue de 50 ± 18 años. Ocurrió fracaso de extubación en 37 (30 %). Como factores de riesgo independentes se asoció hipoalbuminemia en 29 (23.8 %, RR = 1.43, IC 95 % = 1.11-1.85) e hipofosfatemia en 18 (14.6 %, RR = 2.98, IC 95 % = 1.66-5.35); se observaron dos o más factores de riesgo independientes en 22.7 % (RR = 1.51, IC 95 % = 1.14-2.00). Conclusiones: Identificar los factores de riesgo independentes antes de la DVM puede ayudar a reducir el fracaso de la extubación y la morbimortalidad asociada.
Abstract Introduction: Even with an adequate mechanical ventilation weaning (MVW) protocol, the procedure fails in 15 to 30 % of cases. Objective: To assess the association between independent risk factors (IRFs) and post-extubation failure in patients undergoing MVW in an intensive care unit. Method: Longitudinal, prospective, analytical cohort study in patients on mechanical ventilation for more than 24 hours and who were extubated. Pre-extubation reports of hemoglobin, albumin, phosphorus, waist-hip ratio and SOFA score were obtained. Extubation failure was defined as resumption of mechanical ventilation within 48 hours or less. Results: 123 patients were extubated, out of whom 74 were males (60 %); average age was 50 ± 18 years. Extubation failure occurred in 37 (30 %). Hypoalbuminemia was associated as an independent risk factor in 29 (23.8 %, RR = 1.43, 95 % CI = 1.11-1.85) and hypophosphatemia was in 18 (14.6 %, RR = 2.98, 95 % CI = 1.66-5.35); two or more IRFs were observed in 22.7 % (RR = 1.51, 95 % CI = 1.14-2.00). Conclusions: Identifying independent risk factors prior to MVW can help reduce the risk of extubation failure and associated morbidity and mortality.
Assuntos
Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Desmame do Respirador , Extubação/efeitos adversos , Fósforo/sangue , Fatores de Tempo , Albumina Sérica/análise , Estudos Transversais , Estudos Prospectivos , Fatores de Risco , Estudos de Coortes , Retratamento , Pesquisas sobre Atenção à Saúde/estatística & dados numéricos , Relação Cintura-Quadril , Extubação/estatística & dados numéricos , Unidades de Terapia IntensivaRESUMO
BACKGROUND: optimal management of end-stage renal disease (ESRD) in hemodialysis (HD) patients should be more studied because it is a serious risk factor for mortality, being considered an unquestionable global priority. METHODS: we performed a retrospective cohort study from the Nephrology Service in Brazil evaluating the survival of patients with ESRD in HD during 20 years. Kaplan-Meier method with the Log-Rank and Cox's proportional hazards model explored the association between survival time and demographic factors, quality of treatment and laboratory values. RESULTS: Data from 422 patients were included. The mean survival time was 6.79 ± 0.37. The overall survival rates at first year was 82,3%. The survival time correlated significantly with clinical prognostic factors. Prognostic analyses with the Cox proportional hazards regression model and Kaplan-Meier survival curves further identified that leukocyte count (HR = 2.665, 95% CI: 1.39-5.12), serum iron (HR = 8.396, 95% CI: 2.02-34.96), serum calcium (HR = 4.102, 95% CI: 1.35-12.46) and serum protein (HR = 4.630, 95% CI: 2.07-10.34) as an independent risk factor for the prognosis of survival time, while patients with chronic obstructive pyelonephritis (HR = 0.085, 95% CI: 0.01-0.74), high ferritin values (HR = 0.392, 95% CI: 0.19-0.80), serum phosphorus (HR = 0.290, 95% CI: 0.19-0.61) and serum albumin (HR = 0.230, 95% CI: 0.10-0.54) were less risk to die. CONCLUSION: survival remains low in the early years of ESRD treatment. The present study identified that elevated values of ferritin, serum calcium, phosphorus, albumin, leukocyte, serum protein and serum iron values as a useful prognostic factor for the survival time.
Assuntos
Falência Renal Crônica/mortalidade , Terapia de Substituição Renal , Idoso , Idoso de 80 Anos ou mais , Biomarcadores/sangue , Proteínas Sanguíneas/análise , Cálcio/sangue , Feminino , Humanos , Ferro/sangue , Estimativa de Kaplan-Meier , Falência Renal Crônica/sangue , Falência Renal Crônica/terapia , Contagem de Leucócitos , Masculino , Pessoa de Meia-Idade , Fósforo/sangue , Prognóstico , Modelos de Riscos Proporcionais , Insuficiência Renal Crônica/sangue , Insuficiência Renal Crônica/mortalidade , Insuficiência Renal Crônica/terapia , Estudos Retrospectivos , Fatores de Risco , Albumina Sérica , Taxa de SobrevidaRESUMO
Chronic kidney disease is a common disease in dogs, and factors such as serum concentrations of creatinine, albumin, and phosphorus at the moment of diagnosis may influence the survival of these patients. The present retrospective study aimed to evaluate the relationship between survival in dogs with chronic kidney disease and laboratory parameters (creatinine, phosphorus, albumin, and hematocrit) and nutritional parameters (body condition score, muscle mass score, type of food, appetite and feeding method). A total of 116 dogs with chronic kidney disease stages 2 to 4 were included, and survival was calculated considering the time between diagnosis and death. Survival curves were configurated by Kaplan-Meier analysis and a comparison between survival curves was performed by the log-rank test. Factors related to survival were disease stage (p<0.0001), serum phosphorus concentration (p = 0.0005), hematocrit (0.0001), body condition score (p = 0.0391), muscle mass score (p = 0.0002), type of food (p = 0.0009), feeding method (p<0.0001) and appetite (p = 0.0007). Based on data obtained in this study, it is possible to conclude that early diagnosis, as well as nutritional evaluation and renal diet intake, are determinant strategies to increase survival in dogs with chronic kidney disease.
Assuntos
Doenças do Cão/metabolismo , Laboratórios , Estado Nutricional , Insuficiência Renal Crônica/veterinária , Animais , Doenças do Cão/sangue , Cães , Feminino , Estimativa de Kaplan-Meier , Masculino , Fósforo/sangue , Insuficiência Renal Crônica/sangue , Insuficiência Renal Crônica/metabolismo , Análise de SobrevidaRESUMO
Parathyroid hormone (PTH) has an important role in the maintenance of serum calcium levels. It activates renal 1α-hydroxylase and increases the synthesis of the active form of vitamin D (1,25[OH]2D3). PTH promotes calcium release from the bone and enhances tubular calcium resorption through direct action on these sites. Hallmarks of secondary hyperparathyroidism associated with chronic kidney disease (CKD) include increase in serum fibroblast growth factor 23 (FGF-23), reduction in renal 1,25[OH]2D3 production with a decline in its serum levels, decrease in intestinal calcium absorption, and, at later stages, hyperphosphatemia and high levels of PTH. In this paper, we aim to critically discuss severe CKD-related hyperparathyroidism, in which PTH, through calcium-dependent and -independent mechanisms, leads to harmful effects and manifestations of the uremic syndrome, such as bone loss, skin and soft tissue calcification, cardiomyopathy, immunodeficiency, impairment of erythropoiesis, increase of energy expenditure, and muscle weakness.
Assuntos
Hiperparatireoidismo Secundário/etiologia , Hormônio Paratireóideo/metabolismo , Insuficiência Renal Crônica/complicações , Uremia/etiologia , Remodelação Óssea , Osso e Ossos/metabolismo , Cálcio/metabolismo , Metabolismo Energético , Fator de Crescimento de Fibroblastos 23 , Fatores de Crescimento de Fibroblastos/metabolismo , Humanos , Hiperparatireoidismo Secundário/metabolismo , Fósforo/sangue , Insuficiência Renal Crônica/metabolismo , Uremia/metabolismoRESUMO
BACKGROUND: Bone mineral disease after transplantation persists and is an issue that must be addressed owing to the cardiovascular impact it presents. The objective of this study is to present the behavior of calcium, phosphorus, and parathormone (PTH) before renal transplantation (RT) and throughout the 12 months after transplant surgery. METHODS: A longitudinal observational study of RT patients was performed from 2013 to 2017 in 2 renal transplant units in Mexico. In total, 1009 records of patients with RT were analyzed. Calcium, phosphorus, and PTH levels were studied before transplantation and for 12 months after. Central tendency and dispersion were measured, the difference of means was established with chi square or student t tests, and the significant value of P was set at <.05. We also used the SPSS statistical package, version 25. RESULTS: Phosphorus had a median pre-RT of 5.73, which decreased to 2.8 in the first month post-transplant and then increased to 3.41 at 12 months post-RT. The median PTH, on the other hand, started at 420.60 and decreased to 67.45. Calcium began at 9.04 and hit a plateau of 9.58 during month 12 after the surgical event. CONCLUSIONS: Of the 3 biochemical parameters evaluated, phosphorus was the one that most corrected itself after transplantation. Despite a tendency toward hypophosphatemia in the first month after transplantation, it began to normalize from month 6 on. Meanwhile, calcium was the biochemical value that changed the least after transplantation.
Assuntos
Doenças Ósseas Metabólicas/epidemiologia , Cálcio/sangue , Transplante de Rim , Hormônio Paratireóideo/sangue , Fósforo/sangue , Adulto , Doenças Ósseas Metabólicas/etiologia , Feminino , Humanos , Estudos Longitudinais , Masculino , México , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
Seabirds have been widely used for monitoring the health of the oceans in diverse marine regions. Among low-cost survey strategies, systematic surveys of seabirds beached on coasts have been developed since the 20th century. However, these studies do not always address blood aspects. The assessment of the health status of birds based on the analysis of hematological and plasma chemistry is crucial to evaluate the overall health status profile of live organisms. Here, the authors study the variability of blood parameters by sex, age class, and year of beached Magellanic Penguin during the nonreproductive period in northern Argentina. Of 44 penguins, 77% were categorized as younger juveniles and the rest as older juveniles, and were captured and studied in coastal areas of Buenos Aires Province during the summers of 2017 and 2018. The mean body weight of beached penguins was affected by the age class of the individuals; most of the younger juveniles showed poor condition in terms of body mass (1,761 ± 235 g). No significant differences were observed in body weight between years and sex. Still, there were significant differences between years for alkaline phosphatase (ALP) and creatine phosphokinase (CPK) values. Twelve of the 20 blood parameters analyzed differ significantly with the age class of the beached penguins; younger juveniles were in a state of inanition. Our results may serve as a necessary first step in improving the conservation status of the Magellanic Penguin in nonbreeding grounds of Argentina, and call for a better knowledge of the health status of the species along its annual cycle.
Assuntos
Contagem de Eritrócitos/veterinária , Hematócrito , Contagem de Leucócitos/veterinária , Estações do Ano , Spheniscidae/sangue , Envelhecimento , Fosfatase Alcalina/sangue , Animais , Animais Selvagens , Anticorpos Heterófilos , Argentina , Glicemia , Proteínas Sanguíneas , Colesterol/sangue , Creatina Quinase/sangue , Feminino , Hemoglobinas , Contagem de Linfócitos/veterinária , Masculino , Fósforo/sangue , Transaminases/sangue , Ureia/sangue , Ácido Úrico/sangueRESUMO
INTRODUCTION: Even with an adequate mechanical ventilation weaning (MVW) protocol, the procedure fails in 15 to 30 % of cases. OBJECTIVE: To assess the association between independent risk factors (IRFs) and post-extubation failure in patients undergoing MVW in an intensive care unit. METHOD: Longitudinal, prospective, analytical cohort study in patients on mechanical ventilation for more than 24 hours and who were extubated. Pre-extubation reports of hemoglobin, albumin, phosphorus, waist-hip ratio and SOFA score were obtained. Extubation failure was defined as resumption of mechanical ventilation within 48 hours or less. RESULTS: 123 patients were extubated, out of whom 74 were males (60 %); average age was 50 ± 18 years. Extubation failure occurred in 37 (30 %). Hypoalbuminemia was associated as an independent risk factor in 29 (23.8 %, RR = 1.43, 95 % CI = 1.11-1.85) and hypophosphatemia was in 18 (14.6 %, RR = 2.98, 95 % CI = 1.66-5.35); two or more IRFs were observed in 22.7 % (RR = 1.51, 95 % CI = 1.14-2.00). CONCLUSIONS: Identifying independent risk factors prior to MVW can help reduce the risk of extubation failure and associated morbidity and mortality. INTRODUCCIÓN: Aun con adecuado protocolo de desconexión de la ventilación mecánica (DVM), el procedimiento falla en 15 a 30 % de los casos. OBJETIVO: Evaluar la asociación entre factores de riesgo independientes y fracaso posextubación en pacientes con DVM en una unidad de cuidados intensivos. MÉTODO: Estudio de cohorte, longitudinal, prospectivo, analítico, que incluyó pacientes sometidos a ventilación mecánica por más de 24 horas y que fueron extubados. Se obtuvieron reportes preextubación de hemoglobina, albúmina, fósforo, índice cintura-cadera y puntuación SOFA. Se definió como fracaso de extubación al reinicio de la ventilación mecánica en 48 horas o menos. RESULTADOS: Se extubaron 123 pacientes, 74 hombres (60 %); la edad promedio fue de 50 ± 18 años. Ocurrió fracaso de extubación en 37 (30 %). Como factores de riesgo independentes se asoció hipoalbuminemia en 29 (23.8 %, RR = 1.43, IC 95 % = 1.11-1.85) e hipofosfatemia en 18 (14.6 %, RR = 2.98, IC 95 % = 1.66-5.35); se observaron dos o más factores de riesgo independientes en 22.7 % (RR = 1.51, IC 95 % = 1.14-2.00). CONCLUSIONES: Identificar los factores de riesgo independentes antes de la DVM puede ayudar a reducir el fracaso de la extubación y la morbimortalidad asociada.