Síndrome nefrótico idiopático: recomendaciones de la Rama de Nefrología de la Sociedad Chilena de Pediatría. Parte 1

Abstract

Idiopathic nephrotic syndrome is the most common glomerular disease in childhood, affecting 1 to 3 per 100,000 children under the age of 16. It most commonly occurs in ages between 2 and 10. Its cause is unknown and its histology corresponds to minimal change disease in 90% of cases, or focal segmental glomerulosclerosis. 80 to 90% of cases respond to steroids (steroid-sensitive nephrotic syndrome) with good prognosis and long-term preservation of renal function over time. 70% of patients with SSNS have one or more relapses in their evolution, and of these, 50% behave as frequent relapsing or steroid-dependent, a group that concentrate the risk of steroid toxicity. Patients with steroid-resistant nephrotic syndrome have a poor prognosis and 50% of them evolve to end-stage renal disease. The goal of therapy is to induce and maintain remission of the disease, reducing the risk secondary to proteinuria while minimizing the adverse effects of treatments, especially with prolonged use of corticosteroids. This paper is the result of the collaborative effort of the Nephrology Branch of the Chilean Society of Pediatrics with aims at helping pediatricians and pediatric nephrologists to treat pediatric SNI. In this first part, recommendations of steroid-sensitive nephrotic syndrome are discussed.

Histopathological findings in children with idiopathic nephrotic syndrome in Honduras, 2016-2020

“…Most cases are concentrated in patients between 2 and 10 years of age, representing the INS or primary group, while those under one year of age are attributable to a genetic cause [2]. The most frequent histopathology reported is minimal change disease (85%), followed by focal segmental glomerulosclerosis (FSGS); most cases of INS relapse for a considerable period of time from their rst episode (80-90%), but have a good long-term prognosis, with the exception of steroid-resistant patients, where 50% of patients develop terminal chronic kidney disease at 5 years [1][2][4][5]. Approximately 80-90% of children with INS respond to steroids, classifying them as corticosteroid-responsive INS [6].…”

Serum IgG Level and IgG/IgM Ratio on Admission Predict Steroid-Resistant Response in Vietnamese Children with Idiopathic Nephrotic Syndrome

“…The prevalence of idiopathic nephrotic syndrome (NS) is about 1.15 to 16.9 per of 100000 children that depends on race and geographical location. The root cause of this disease is unknown, but its pathogenesis includes various factors such as immunity, systemic features, and structure abnormalities (1)(2)(3)(4). The histopathology of idiopathic NS included minimal change nephrotic syndrome (MCNS), mesangial proliferative glomerulonephritis (MesPGN), and focal segmental glomerulosclerosis (FSGS) (5).…”