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Risk factors and retreatment for relapse in childhood primary nephrotic syndrome treated with rituximab.
Pediatr Nephrol
January 2025
Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450052, Henan, China.
Background: The effectiveness of rituximab (RTX) for steroid-dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS) in children is well documented. However, there are insufficient data on relapse risk factors. Additionally, the retreat regimen for relapsed children requires further investigation.
View Article and Find Full Text PDFOutcomes Using a Standardized Provincial Childhood Nephrotic Syndrome Clinical Pathway.
Can J Kidney Health Dis
December 2024
Division of Nephrology, BC Children's Hospital, Vancouver, Canada.
Background: In 2013, the British Columbia (BC) Childhood Nephrotic Syndrome Clinical Pathway (CNSCP) was developed to standardize the care of children with nephrotic syndrome (NS). In BC, children access nephrology care at BC Children's Hospital (BCCH) and multiple regional clinics.
Objective: The primary objective was to compare induction therapy and clinical outcomes between BCCH and regional clinics since implementation of the CNSCP.
Childhood idiopathic nephrotic syndrome: recent advancements shaping future guidelines.
Pediatr Nephrol
December 2024
Néphrologie Pédiatrique, Centre de Référence du Syndrome Néphrotique Idiopathique de L'enfant Et L'adulte, Hôpital Necker - Enfants Malades, APHP, Inserm U1163, Institut Imagine, Université Paris Cité, Paris, France.
Childhood idiopathic nephrotic syndrome is an important pediatric kidney disease associated with significant morbidities and even mortality. Several guidelines have been developed to standardize the terminology and patient care among the pediatric nephrology community. Since the publication of these guidelines, there have been major breakthroughs in the disease management and the understanding of underlying pathogenesis through multi-omics investigations, including the identification of anti-nephrin autoantibodies, genetic susceptibility loci, and the pathogenic role of B cell subsets.
View Article and Find Full Text PDFTrifunctional protein deficiency (TFP) is a disorder of fatty acid beta-oxidation associated with metabolic, cardiac, and liver dysfunction in severe forms. We present two siblings diagnosed by newborn screening and confirmed by biochemical testing at birth. Their clinical course was complicated by recurrent rhabdomyolysis, retinopathy, and hypoparathyroidism.
View Article and Find Full Text PDFIncidence and Risk Factors for Hypertension among Children with Nephrotic Syndrome.
J Pediatr
December 2024
Division of Nephrology, The Hospital for Sick Children, Toronto, Ontario, Canada; Child Health Evaluative Sciences, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada; Women's College Hospital, Department of Medicine, Toronto Ontario, Canada; Department of Pediatrics, Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada. Electronic address:
Objectives: To determine incidence and risk factors for hypertension in childhood nephrotic syndrome.
Study Design: Using data from the Insight into Nephrotic Syndrome (INSIGHT) study, a prospective observational childhood nephrotic syndrome cohort from Toronto, Canada, we evaluated hypertension incidence and time-to-hypertension overall and stratified by 1) steroid-resistance or steroid-sensitivity, and 2) frequently-relapsing, steroid dependent, or infrequently-relapsing. Hypertension was defined as stage 1-2 hypertensive blood pressure on two consecutive visits or anti-hypertensive medication initiation.
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