Management of steroid-resistant nephrotic syndrome in children and adolescents

Tullus, Kjell; Webb, Hazel; Bagga, Arvind

doi:10.1016/s2352-4642(18)30283-9

Cited by 117 publications

(107 citation statements)

References 83 publications

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“…Early recurrence is normally characterized by massive proteinuria within hours to days after transplantation, whereas late recurrence develops several months or year after transplantation [7]. Clinically, FSGS presents with a triad of findings: nephrotic range proteinuria (proteinuria > 50 mg/kg/day), low serum albumin (< 2.5 g/dL), and edema [4]. The pathophysiology behind FSGS is still being fully elucidated, but there have been great strides over the past several decades.…”

Section: Classification and Pathophysiologymentioning

confidence: 99%

“…The pathophysiology behind FSGS is still being fully elucidated, but there have been great strides over the past several decades. Genetic causes have been found in some patients with FSGS [4]. These genetic causes include various mutations affecting structural, mitochondrial, nuclear, and other intracellular proteins [4].…”

Section: Classification and Pathophysiologymentioning

confidence: 99%

“…Genetic causes have been found in some patients with FSGS [4]. These genetic causes include various mutations affecting structural, mitochondrial, nuclear, and other intracellular proteins [4]. While there are genetic etiologies for FSGS, the majority of patients likely have a nongenetic cause.…”

Section: Classification and Pathophysiologymentioning

confidence: 99%

“…Throughout the last 20 years, there has been a steady increase in the incidence of FSGS, most likely due to increase in patients progressing to end-stage renal disease (ESRD). Although, the progression of FSGS to ESRD, most commonly in FSGS patients, is resistant to steroid and immunosuppressive drugs [3,4]. Of those patients that undergo renal transplantation, it has been reported that up to 56% of patients will have a recurrence of FSGS (rFSGS) in the transplanted kidney [1,5].…”

Section: Introductionmentioning

confidence: 99%

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Extracorporeal Therapies in the Treatment of Focal Segmental Glomerulosclerosis

et al. 2020

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Focal segmental glomerulosclerosis (FSGS) is one of the most frequent and severe glomerular kidney disease with frequent progression to end-stage renal disease and a high rate of recurrence in renal transplantations. Due to intolerance or resistance to the current immunomodulatory treatments, the management of FSGS is a therapeutic challenge. Over the last few years, development in extracorporeal therapies has shown potential beneficial outcomes in drug-resistant and recurrent FSGS patients. Thus, this study reviews the current literature on the use of extracorporeal therapies, such as plasma exchange therapy, immunoadsorption, and low-density lipoprotein apheresis, for the treatment of FSGS in the pediatric population.

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Section: Classification and Pathophysiologymentioning

confidence: 99%

Section: Classification and Pathophysiologymentioning

confidence: 99%

Section: Classification and Pathophysiologymentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Extracorporeal Therapies in the Treatment of Focal Segmental Glomerulosclerosis

et al. 2020

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“…Relapse is defined as a urinary protein‐to‐creatinine ratio of ≥2000 mg/g (≥200 mg/mmol) or ≥3+ protein on urine dipstick for three consecutive days after remission has already been achieved . Although up to approximately 85% of paediatric NS patients are reportedly sensitive to steroids, most cases relapse, with approximately half becoming frequent relapsers or steroid‐dependent. Frequent relapsers are defined as at least two relapses over a 6‐month or more than three over a 12‐month period, and steroid‐dependent NS is defined as relapses during treatment or within 2 weeks of stopping corticosteroids …”

Section: Introductionmentioning

confidence: 99%

Review on long‐term non‐renal complications of childhood nephrotic syndrome

et al. 2019

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Background Nephrotic syndrome (NS) is the most common glomerular childhood disease. A body of literature has described the long‐term renal prognosis of childhood‐onset idiopathic NS. However, the nonrenal outcomes have not been studied as much. Aim We aimed to discuss the long‐term non‐renal outcomes of childhood NS, highlighting studies with a follow‐up period of more than 10 years. Results We reviewed the literature and found that a number of immunosuppressive agents have stopped inflammation, stabilised the podocyte cytoskeleton and reduce proteinuria. However, prolonged treatment has frequently been associated with a high risk of renal and non‐renal complications in patients with a complicated disease course, defined as frequent relapses or steroid dependency. Non‐renal complications may include impaired longitudinal growth and pubertal development, undesirable fertility outcomes, ocular complications, bone mineral diseases and potential malignancies. Conclusion This review discusses and summarises the non‐renal outcomes of idiopathic childhood NS.

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Glucocorticoid receptors and their upstream epigenetic regulators in adults with steroid‐resistant nephrotic syndrome

et al. 2020

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Steroid-resistant nephrotic syndrome (SRNS) is a clinical challenge with variable clinical outcomes. In patients with SRNS, unsuccessful anti-inflammatory and anti-proteinuric effects of steroids lead to end-stage renal disease (ESRD). Our objective was to define the expression pattern of the glucocorticoid receptors (GR) α and β and their epigenetic regulators (miR-24, miR-30a, and miR-370) in a group of adults with SRNS. In this regard, sixty primary NS patients with focal segmental glomerulosclerosis (FSGS, N = 30) and membranous glomerulonephritis (MGN, N = 30) and also healthy volunteers (N = 24) were enrolled. Real-time PCR was performed to evaluate the expression levels of the aforementioned genes in peripheral blood mononuclear cell (PBMC) samples.

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Management of steroid-resistant nephrotic syndrome in children and adolescents

Cited by 117 publications

References 83 publications

Extracorporeal Therapies in the Treatment of Focal Segmental Glomerulosclerosis

Extracorporeal Therapies in the Treatment of Focal Segmental Glomerulosclerosis

Review on long‐term non‐renal complications of childhood nephrotic syndrome

Glucocorticoid receptors and their upstream epigenetic regulators in adults with steroid‐resistant nephrotic syndrome

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