The Italian Society for Pediatric Nephrology (SINePe) consensus document on the management of nephrotic syndrome in children: Part I - Diagnosis and treatment of the first episode and the first relapse

Abstract

This consensus document is aimed at providing an updated, multidisciplinary overview on the diagnosis and treatment of pediatric nephrotic syndrome (NS) at first presentation. It is the first consensus document of its kind to be produced by all the pediatric nephrology centres in Italy, in line with what is already present in other countries such as France, Germany and the USA. It is based on the current knowledge surrounding the symptomatic and steroid treatment of NS, with a view to providing the basis for a separate consensus document on the treatment of relapses. NS is one of the most common pediatric glomerular diseases, with an incidence of around 2–7 cases per 100000 children per year. Corticosteroids are the mainstay of treatment, but the optimal therapeutic regimen for managing childhood idiopathic NS is still under debate. In Italy, shared treatment guidelines were lacking and, consequently, the choice of steroid regimen was based on the clinical expertise of each individual unit. On the basis of the 2015 Cochrane systematic review, KDIGO Guidelines and more recent data from the literature, this working group, with the contribution of all the pediatric nephrology centres in Italy and on the behalf of the Italian Society of Pediatric Nephrology, has produced a shared steroid protocol that will be useful for National Health System hospitals and pediatricians. Investigations at initial presentation and the principal causes of NS to be screened are suggested. In the early phase of the disease, symptomatic treatment is also important as many severe complications can occur which are either directly related to the pathophysiology of the underlying NS or to the steroid treatment itself. To date, very few studies have been published on the prophylaxis and treatment of these early complications, while recommendations are either lacking or conflicting. This consensus provides indications for the prevention, early recognition and treatment of these complications (management of edema and hypovolemia, therapy and prophylaxis of infections and thromboembolic events). Finally, recommendations about the clinical definition of steroid resistance and its initial diagnostic management, as well as indications for renal biopsy are provided.

Refractory Oedema of Nephrotic Syndrome in a Resource Poor Setting: A Case Presentation

“…Aetiologically, NS is divided into the primary (idiopathic) and the secondary types. The "minimal change disease" (MCD) constitutes about 76% of the idiopathic type [2]. Although about 95% of MCD patients respond to steroids, 75% of them can relapse requiring recommencement of treatment [2].…”

“…The incidence of NS is 2 -7 cases per 100,000 children per year, while the prevalence is 16 cases per 100,000 [2]. Aetiologically, NS is divided into the primary (idiopathic) and the secondary types.…”

“…In the underfill hypothesis, severe hypoalbuminaemia decreases intravascular oncotic pressure, causing circulatory volume depletion and subsequent sodium and water retention [2] [3] [4] [5] [6]. The reduction in plasma oncotic pressure also causes transudation of fluid from the intravascular to the interstitial space.…”

“…The "overfill" theory states that a primary renal defect in sodium excretion leads to sodium and water retention, thereby causing hypervolemia and oedema [2] [3] [4] [5] [6].…”

“…The pathogenesis of oedema in NS is explained by two different hypotheses, the "underfill" and "overfill" mechanisms [2]- [8].…”