Abstract
Nephrotic syndrome (NS) affects 115-169 children per 100,000, with rates varying by ethnicity and location. Immune dysregulation, systemic circulating substances, or hereditary structural abnormalities of the podocyte are considered to have a role in the etiology of idiopathic NS. Following daily therapy with corticosteroids, more than 85% of children and adolescents (often aged 1 to 12 years) with idiopathic nephrotic syndrome have full proteinuria remission. Patients with steroid-resistant nephrotic syndrome (SRNS) do not demonstrate remission after four weeks of daily prednisolone therapy. The incidence of steroid-resistant nephrotic syndrome in children varies between 35 and 92 percent. A third of SRNS patients have mutations in one of the important podocyte genes. An unidentified circulating factor is most likely to blame for the remaining instances of SRNS.The aim of this article is to explore and review the genetic factors and management of steroid-resistant nephrotic syndrome. An all language literature search was conducted on MEDLINE, COCHRANE, EMBASE, and Google Scholar till September 2021. The following search strings and Medical Subject Headings (MeSH) terms were used: “Steroid resistance”, “nephrotic syndrome”, “nephrosis” and “hypoalbuminemia”. We comprehensively reviewed the literature on the epidemiology, genetics, current treatment protocols, and management of steroid-resistant nephrotic syndrome.We found that for individuals with non-genetic SRNS, calcineurin inhibitors (cyclosporine and tacrolimus) constitute the current mainstay of treatment, with around 70% of patients achieving full or partial remission and an acceptable long-term prognosis. Patients with SRNS who do not react to calcineurin inhibitors or other immunosuppressive medications may have deterioration in kidney function and may develop end-stage renal failure. Nonspecific renal protective medicines, such as angiotensin-converting enzyme inhibitors, angiotensin 2 receptor blockers, and anti-lipid medications, slow the course of the illness. Recurrent focal segmental glomerulosclerosis in the allograft affects around a third of individuals who get a kidney transplant, and it frequently responds to a combination of plasma exchange, rituximab, and increased immunosuppression. Despite the fact that these results show a considerable improvement in outcome, further multicenter controlled studies are required to determine the optimum drugs and regimens to be used.
Highlights
BackgroundNephrotic syndrome (NS) is the most common glomerular disease in children
steroid-resistant nephrotic syndrome (SRNS), which accounts for 20% of the total cases, is more likely to be associated with focal segmental glomerulosclerosis (FSGS) [4,5]
According to the International Study of Kidney Disease in Children (ISKDC), idiopathic nephrotic syndrome in children can be categorized depending on the response to steroids
Summary
Nephrotic syndrome (NS) is the most common glomerular disease in children. It is characterized as proteinuria (nephrotic range) with serum albumin less than 3 g/dl or clinical evidence of edema when albumin level cannot be done [1]. Steroid-resistant nephrotic syndrome (SRNS) accounts for almost 15% of all children with chronic kidney disease who eventually require renal transplantation [11]. Genetic testing is necessary for patients less than six years of age at diagnosis, with a positive family history of nephrotic syndrome, a history of steroid resistance, FSGS histopathology, or diffuse mesangial sclerosis [25]. In the two weeks of additional treatment for non-responders, genetic testing should be done to search for mutations in genes that encode podocyte proteins a kidney biopsy to identify the underlying cause of the disease (most common histological diagnoses include focal segmental glomerulosclerosis, minimal change nephropathy, or mesangioproliferative GN) and study the degree of fibrosis in the glomeruli and renal interstitium. Varying case reports have been done on the response of abatacept in SRNS, but no common consensus is there regarding its efficacy [89,90]
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