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Detailed Pathophysiology of Minimal Change Disease: Insights into Podocyte Dysfunction, Immune Dysregulation, and Genetic Susceptibility.
Int J Mol Sci
November 2024
Department of Nephrology, Hypertension, Transplantation and Internal Medicine, Central University Hospital, Medical University of Lodz, Pomorska 251, 92-213 Lodz, Poland.
Minimal Change Disease (MCD) is a predominant cause of idiopathic nephrotic syndrome in the pediatric population, yet presents significant clinical challenges due to its frequent relapses and steroid resistance. Despite its relatively benign histological appearance, MCD is characterized by severe proteinuria, hypoalbuminemia, and edema, which may affect patient outcomes. Current treatment strategies primarily rely on corticosteroids, which are effective in inducing remission but are associated with high relapse rates, steroid resistance, and numerous long-term side effects, underscoring the need for more targeted and effective therapeutic approaches.
View Article and Find Full Text PDFCombination of rituximab and low-dose glucocorticoids for idiopathic refractory nephrotic syndrome with MCD/FSGS: a single-center prospective cohort study.
Ren Fail
December 2024
Department of Nephrology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
Background: The treatment of idiopathic refractory nephrotic syndrome (IRNS) remains a difficult problem in clinical practice. This study aims to determine the efficacy and safety of combining low-dose glucocorticoids with rituximab in IRNS treatment.
Methods: This prospective, single-center cohort study enrolled 60 patients who were diagnosed with refractory IRNS with minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS) and treated at First Affiliated Hospital of Sun Yat-sen University.
To biopsy or not to biopsy a teenager with typical idiopathic nephrotic syndrome? Start steroids first.
Pediatr Nephrol
February 2025
Néphrologie Pédiatrique, Centre de Référence du Syndrome Néphrotique Idiopathique de L'enfant Et L'adulte, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique - Hôpitaux de Paris (APHP), Institut Imagine, INSERM U1163, Université Paris Cité, 149 Rue de Sèvres, 75015, Paris, France.
Article Synopsis
- - Minimal change disease (MCD) and focal segmental glomerulosclerosis are common in children with idiopathic nephrotic syndrome, with MCD being highly responsive to steroids and generally not requiring a kidney biopsy at diagnosis.
- - Kidney biopsies should be reserved for atypical symptoms, steroid resistance, or to investigate possible systemic diseases, as they are invasive and can cause complications.
- - New non-invasive biomarkers are being developed to reduce the need for biopsies, and starting steroid treatment in teenagers with typical nephrotic syndrome is a sensible approach.
Renal and Urinary Conditions: Nephrotic Syndrome.
FP Essent
August 2024
Womack Army Medical Center Family Medicine Residency Program, Fort Liberty, North Carolina.
Article Synopsis
- Patients with nephrotic syndrome (NS) show symptoms like swelling, high protein in urine, low albumin levels, and high lipids, with different causes in children (usually minimal change disease) versus adults (mainly FSGS and membranous nephropathy).
- Diagnosis involves checking proteinuria and serum levels, with kids often improving on steroid treatment, while adults typically need a kidney biopsy, unless they test positive for specific antibodies indicative of MN.
- Treatment for NS includes glucocorticoids to reduce proteinuria, but alternative therapies are needed for frequent recurrences or steroid resistance, along with lifestyle changes and precautions against blood clots for high-risk patients.
Differentiated metabolomic profiling reveals plasma amino acid signatures for primary glomerular disease.
Amino Acids
July 2024
Blood Purification Center, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450052, P. R. China.
Primary glomerular disease (PGD) is an idiopathic cause of renal glomerular lesions that is characterized by proteinuria or hematuria and is the leading cause of chronic kidney disease (CKD). The identification of circulating biomarkers for the diagnosis of PGD requires a thorough understanding of the metabolic defects involved. In this study, ultra-high performance liquid chromatography-tandem mass spectrometry was performed to characterize the amino acid (AA) profiles of patients with pathologically diagnosed PGD, including minimal change disease (MCD), focal segmental glomerular sclerosis (FSGS), membranous nephropathy, and immunoglobulin A nephropathy.
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