The non-immunosuppressive management of childhood nephrotic syndrome

Abstract

Idiopathic nephrotic syndrome (INS) is one of the most common renal diseases found in the paediatric population and is associated with significant complications, including infection and thrombosis. A high proportion of children enter sustained remission before adulthood, and therapy must therefore mitigate the childhood complications, while minimising the long-term risk to health. Here we address the main complications of INS and summarise the available evidence and guidance to aid the clinician in determining the appropriate treatment for children with INS under their care. Additionally, we highlight areas where no consensus regarding appropriate management has been reached. In this review, we detail the reasons why routine prophylactic antimicrobial and antithrombotic therapy are not warranted in INS and emphasise the conservative management of oedema. When pharmacological intervention is required for the treatment of oedema, we provide guidance to aid the clinician in determining the appropriate therapy. Additionally, we discuss obesity and growth, fracture risk, dyslipidaemia and thyroid dysfunction associated with INS. Where appropriate, we describe how recent developments in research have identified potential novel therapeutic targets.

Minimal Change Disease

“…In acute management, salt and fluid intake restriction is mandatory to improve clinical manifestations (edema) (40). Steroid treatment with prednisone or prednisolone, its active metabolite, are the primary drugs used at disease onset.…”

“…Of note, live vaccines are contraindicated in patients receiving steroid-sparing agents and in patients receiving high-dose steroids; inactivated vaccines should be administered, keeping in mind that suboptimal response requiring a repeated dose may be an issue (40,57).…”

Tata Laksana Non Imunosupresan Sindrom Nefrotik pada Anak

“…8 Diperlukan pemantauan penurunan berat badan untuk menilai efektivitas tata laksana. 15 Sebagian besar sindrom nefrotik sensitif steroid tidak memerlukan terapi spesifik untuk edema. Pada edema ringan, tata laksana biasanya bersifat konservatif dengan restriksi cairan hingga dua pertiga kebutuhan rumatan dan diit rendah garam.…”

“…Diperkirakan sebagai overfill jika terdapat hipertensi, kadar albumin serum > 2,0 g/dL, dan laju filtrasi glomerulus estimasi < 50%. 15 Pemberian diuretik pada edema underfill memerlukan pemantauan hemodinamik sistemik dan fungsi ginjal untuk meyakinkan tidak terjadi penurunan volume intravaskular, dan biasanya dimulai dengan diuretik dosis rendah. 15 Pada edema overfill, perlu pemberian diuretik yang agresif, biasanya loop diuretic yang mempunyai durasi singkat, biasanya 6 jam sehingga diberikan minimal dua kali sehari.…”

“…15 Pemberian diuretik pada edema underfill memerlukan pemantauan hemodinamik sistemik dan fungsi ginjal untuk meyakinkan tidak terjadi penurunan volume intravaskular, dan biasanya dimulai dengan diuretik dosis rendah. 15 Pada edema overfill, perlu pemberian diuretik yang agresif, biasanya loop diuretic yang mempunyai durasi singkat, biasanya 6 jam sehingga diberikan minimal dua kali sehari. Penelitian membuktikan bahwa furosemid 1 mg/kgbb secara intravena dua kali lebih efektif dibandingkan furosemid 2 mg/kgbb secara oral.…”