Commentary on “Pediatric Idiopathic Steroid-sensitive Nephrotic Syndrome Diagnosis and Therapy - Short version of the updated German Best Practice Guideline (S2e)”

Clinical practice guidelines for nephrotic syndrome: consensus is emerging

“…Based on the results of these trials, ISPN recommends that initial therapy should comprise of 6 weeks each of daily and alternate day prednisone therapy (Table 1) [3]. While similar advice was given by the GSPN [7], KDIGO guidelines recommend that physicians could either follow 8 weeks or 12 weeks initial treatment, with empiric advice to prefer the former in patients with rapid (< 7 days) remission or comorbidities such as obesity or hypertension, and consider prolonged (16-24 weeks) therapy in young patients with delayed remission [6].…”

“…Comparison between recent clinical practice guidelines of the Kidney Disease Improving Global Outcomes (KDIGO) [6], German Society for Pediatric Nephrology (GSPN) [7], and the Indian Society of Pediatric Nephrology (ISPN) [3] (adapted with permission from [3]) Prednisone is dosed either by body surface area or by weight. While dosing using body weight is convenient, it might result in considerable underdosing, especially in younger children [15].…”

“…However, the evidence that short-and medium-term outcomes are better following dosing of initial therapy based on body surface area versus body weight is equivocal [12,15]. While the ISKDC and GSPN recommend that prednisone be dosed on body surface area, guidelines from ISPN and KDIGO recommend either approach, with preference to surface area-based dosing in young children [3,6,7]. Once-daily dosing of prednisone is more convenient than divided doses and is associated with similar time to remission [16].…”

“…Guidelines from the Indian Society of Pediatric Nephrology (ISPN) that were first published in 2001 were revised in 2008-2009 and more recently in 2021 [3,4]. We discuss these guidelines and compare them to recently published advice from the International Pediatric Nephrology Association (IPNA) [5], Kidney Disease Improving Global Outcomes (KDIGO) [6], and the German Society of Pediatric Nephrology (GSPN) (Tables 1 and 2) [7].…”

Management des kongenitalen nephrotischen Syndroms

“…Negative genetische Testergebnisse, unauffällige Infektionsparameter und eine Nierenbiopsie, die eine diffuse mesangiale Sklerose ausschließt, sollten vor Beginn einer immunsuppressiven Behandlung vorliegen. Die medikamentöse Therapie sollte dann entsprechend der Leitlinie der Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften (AWMF) zum idiopathischen nephrotischen Syndrom des Kindes- und Jugendalters erfolgen 25 , 26 .…”