ABSTRACT
Introducción: El daño renal es frecuente en niños con trasplante hepático (TH), aunque su detección es un desafío. Nuestro objetivo fue evaluar el daño renal agudo (DRA) perioperatorio y analizar la prevalencia de enfermedad renal crónica (ERC) mediante diferentes fórmulas de estimación de la tasa de filtración glomerular (TFG). Métodos: Análisis transversal unicéntrico de una cohorte de niños menores de 18años con TH. Estimamos la TFG utilizando la fórmula Schwartz bedside 2009 basada en la creatinina, Caucasian Asian Pediatric and Adult cohort (CAPA) para cistatinaC y la fórmula combinada de Pottel Full Age Spectrum (FAS). Analizamos la concordancia mediante prueba de Bland Altman y el índice kappa. Medimos la albuminuria, la presión arterial y el volumen urinario por 100ml de filtrado glomerular. Analizamos los factores de riesgo asociados a ERC mediante un análisis univariante y multivariante. Resultados: Se incluyeron 52 pacientes, con una mediana de edad de 9,21años y 5,42años de evolución. Quince (28,8%) tuvieron DRA. Cinco niños (10%) presentaban ERC. El único factor de riesgo asociado fue el fallo hepático agudo en el momento del TH (OR: 8,57, p=0,04). Hubo poca concordancia entre las diferentes fórmulas de estimación. La fórmula de Schwartz clasificó a un paciente con ERC, mientras que Pottel FAS combinada y CAPA clasificaron a cuatro. Hasta el 42% de los niños sin ERC tenían algún marcador de daño renal. Conclusiones: El uso exclusivo de la fórmula Schwartz bedside 2009 para estimar el FG puede limitar el diagnóstico de ERC en niños con TH. La presencia de otros marcadores de daño renal es frecuente y su detección puede prevenir la progresión de la ERC. (AU)
Introduction: Kidney injury associated with paediatric liver transplantation (LT) is common, but its evaluation is challenging. Our aim was to analyse the presence of perioperative acute kidney injury (AKI) and study the prevalence of chronic kidney disease (CKD) using different glomerular filtration rate (GFR) estimation formulas. Methods: We conducted a cross-sectional study in a cohort of children aged less than 18years with a history of LT followed up for 5.42years. We estimated the GFR using the creatinine-based Schwartz bedside formula (2009), the cystatin C-based Caucasian Asian Pediatric and Adult cohort (CAPA) equation and the combined full-age spectrum (FAS) formula as modified by Pottel. We analysed the agreement between them using the Bland-Altman method and the kappa statistic. We measured the albumin level in urine, the urine volume adjusted to 100mL of GFR and blood pressure. We performed univariate and multivariate analyses of the risk factors associated with CKD. Results: The sample included 52 patients with a median age of 9.21years. Fifteen (28.8%) had AKI. Five (10%) had CKD and the only associated risk factor was acute liver failure at the time of LT (odds ratio, 8.57; P=.04). There was poor agreement between the different estimation formulas. One patient was classified as having CKD with the Schwartz formula compared to four patients with the CAPA and the Pottel combined FAS formulas. Up to 42% of children without CKD had some positive marker of kidney injury. Conclusions: The exclusive use of the 2009 Schwartz bedside formula to estimate GFR may lead to underdiagnosis of CKD in children post LT. Other markers of kidney injury are common, and their detection may help prevent the progression of CKD. (AU)
Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Renal Insufficiency, Chronic/prevention & control , Liver Transplantation , Acute Kidney Injury , Cross-Sectional Studies , Glomerular Filtration RateABSTRACT
INTRODUCTION: Kidney injury associated with paediatric liver transplantation (LT) is common, but its evaluation is challenging. Our aim was to analyse the presence of perioperative acute kidney injury (AKI) and study the prevalence of chronic kidney disease (CKD) using different glomerular filtration rate (GFR) estimation formulas. METHODS: We conducted a cross-sectional study in a cohort of children aged less than 18 years with a history of LT followed up for 5.42 years. We estimated the GFR using the creatinine-based Schwartz bedside formula (2009), the cystatin C-based Caucasian Asian Pediatric and Adult cohort (CAPA) equation and the combined Full-Age Spectrum (FAS) formula as modified by Pottel. We analysed the agreement between them using the Bland-Altman method and the kappa statistic. We measured the albumin level in urine, the urine volume adjusted to 100â¯mL of GFR and blood pressure. We performed univariate and multivariate analyses of the risk factors associated with CKD. RESULTS: The sample included 52 patients with a median age of 9.21 years. Fifteen (28.8%) had AKI. Five (10%) had CKD and the only associated risk factor was acute liver failure at the time of LT (odds ratio, 8.57; Pâ¯=â¯0.04). There was poor agreement between the different estimation formulas. One patient was classified as having CKD with the Schwartz formula compared to four patients with the CAPA and the Pottel combined FAS formulas. Up to 42% of children without CKD had some positive marker of kidney injury. CONCLUSIONS: The exclusive use of the 2009 Schwartz bedside formula to estimate GFR may lead to underdiagnosis of CKD in children post LT. Other markers of kidney injury are common, and their detection may help prevent the progression of CKD.
Subject(s)
Acute Kidney Injury , Liver Transplantation , Renal Insufficiency, Chronic , Adult , Humans , Child , Liver Transplantation/adverse effects , Cross-Sectional Studies , Kidney/physiology , Renal Insufficiency, Chronic/epidemiology , Renal Insufficiency, Chronic/etiology , Renal Insufficiency, Chronic/diagnosis , Glomerular Filtration Rate/physiology , Acute Kidney Injury/diagnosis , Acute Kidney Injury/epidemiology , Acute Kidney Injury/etiologyABSTRACT
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Humans , Journal Impact Factor , Scientific Publication Indicators , Journal Impact Factor/history , Pandemics , Coronavirus Infections/epidemiologyABSTRACT
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Subject(s)
History, 21st Century , Periodicals as Topic/history , Periodicals as Topic/trends , Journal Impact Factor , Pandemics , Coronavirus Infections/epidemiologyABSTRACT
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History, 21st Century , Periodicals as Topic/history , Periodicals as Topic/trends , Publishing , SpainABSTRACT
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Subject(s)
History, 21st Century , Periodicals as Topic/history , Periodicals as Topic/trends , Journal Impact Factor , Societies , PediatricsABSTRACT
BACKGROUND: X-linked hypophosphatemia (XLH) is a hereditary rare disease caused by loss-of-function mutations in PHEX gene leading tohypophosphatemia and high renal loss of phosphate. Rickets and growth retardation are the major manifestations of XLH in children, but there is a broad phenotypic variability. Few publications have reported large series of patients. Current data on the clinical spectrum of the disease, the correlation with the underlying gene mutations, and the long-term outcome of patients on conventional treatment are needed, particularly because of the recent availability of new specific medications to treat XLH. RESULTS: The RenalTube database was used to retrospectively analyze 48 Spanish patients (15 men) from 39 different families, ranging from 3 months to 8 years and 2 months of age at the time of diagnosis (median age of 2.0 years), and with XLH confirmed by genetic analysis. Bone deformities, radiological signs of active rickets and growth retardation were the most common findings at diagnosis. Mean (± SEM) height was - 1.89 ± 0.19 SDS and 55% (22/40) of patients had height SDS below-2. All cases had hypophosphatemia, serum phosphate being - 2.81 ± 0.11 SDS. Clinical manifestations and severity of the disease were similar in both genders. No genotype-phenotype correlation was found. Conventional treatment did not attenuate growth retardation after a median follow up of 7.42 years (IQR = 11.26; n = 26 patients) and failed to normalize serum concentrations of phosphate. Eleven patients had mild hyperparathyroidism and 8 patients nephrocalcinosis. CONCLUSIONS: This study shows that growth retardation and rickets were the most prevalent clinical manifestations at diagnosis in a large series of Spanish pediatric patients with XLH confirmed by mutations in the PHEX gene. Traditional treatment with phosphate and vitamin D supplements did not improve height or corrected hypophosphatemia and was associated with a risk of hyperparathyroidism and nephrocalcinosis. The severity of the disease was similar in males and females.
