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.

Keywords: Dyslipidaemia; Idiopathic nephrotic syndrome; Infection; Oedema; Thrombosis; Vaccination.

Fig. 1

Modified Indian Academy of Paediatrics guidelines for the treatment of oedema in INS. Adapted from [19], used with permission from Indian Pediatrics. Patients should only receive diuretic therapy or albumin infusions under close clinical supervision. Fluid volumes should be calculated on an estimated ‘dry’ weight rather than an oedematous weight. Electrolyte levels should be monitored in all patients receiving diuretics, and potassium supplements/spironolactone started when necessary. FeNa Fractional excretion of sodium, U _K/(U _Na+K) quotient of urine potassium and urine sodium plus potassium, I.V. intravenous, B.P. blood pressure, H.A.S. human albumin solution, P.I.C.U paediatric intensive care unit