Síndrome nefrótico idiopático: recomendaciones de la Rama de Nefrología de la Sociedad Chilena de Pediatría. Parte 2

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. Steroid-resistant nephrotic syndrome represents 10-20% of idiopathic nephrotic syndrome in pediatrics. It has a poor prognosis, and its management is a significant therapeutic challenge. Half of patients evolve to end-stage renal disease within 5 years, and are additionally exposed to complications secondary to persistent NS and to the adverse effects of immunosuppressive therapy. The primary goal of treatment is to achieve complete remission, but even a partial remission is associated with a better renal survival than the lack of response. 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 idiopathic nephrotic syndrome. In this second part, handling of steroid-resistant nephrotic syndrome as well as nonspecific therapies are discussed.

Proteinuria and nephrotic syndrome, how to treat it

“…3 Factors associated with greater risk are: shorter time of first relapse (more consistent marker), number of relapses in the first 6 months, younger age and hematuria at onset, male sex and longer time in first remission. 2,3 The long-term prognosis of renal function for most cases is good.…”

“…It is characterized by nephrotic range proteinuria, hypoalbuminemia and generalized edema. 1,2 In Table I, the definitions. 2,3 Epidemiology and etiology 90% of Nephrotic Syndromes are idiopathic or primary.…”

“…The majority begin between 2 and 10 years, corresponding to idiopathic Nephrotic Syndrome (INS) or primary NS in 90%. 2 The incidence in paediatric population less than 16 years old is 2-7 new cases per 100,000 children per year, with a prevalence of 15 cases per 100,000 children. It occurs preferably between 2 and 8 years of age, with maximum incidence between 3-5 years.…”

“…The second in frequency, focal and segmental glomerulosclerosis, with a response rate to corticosteroids of 20-50%. 2 There are other causes, less frequent in paediatrics, of secondary NS to systemic lupus erythematosus, glomerulonephritis, Schöenlein Henoch purpura, vasculitis, malignancies and infections.…”

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).…”