Symptomatic management of nephrotic syndrome in children
- Patrick Niaudet, MD
Patrick Niaudet, MD
- Section Editor — Pediatric Nephrology
- Professor of Pediatrics
- Hôpital Necker-Enfants Malades, Paris, France
The nephrotic syndrome (NS) is caused by renal diseases that increase the permeability across the glomerular filtration barrier. It is classically characterized by four clinical features, but the first two are used diagnostically because the last two may not be seen in all patients:
●Nephrotic range proteinuria − Urine protein excretion >50 mg/kg per day
●Hypoalbuminemia − Serum albumin <3 g/dL (30 g/L)
- McCaffrey J, Lennon R, Webb NJ. The non-immunosuppressive management of childhood nephrotic syndrome. Pediatr Nephrol 2016; 31:1383.
- Vande Walle JG, Donckerwolcke RA. Pathogenesis of edema formation in the nephrotic syndrome. Pediatr Nephrol 2001; 16:283.
- Hogg RJ, Portman RJ, Milliner D, et al. Evaluation and management of proteinuria and nephrotic syndrome in children: recommendations from a pediatric nephrology panel established at the National Kidney Foundation conference on proteinuria, albuminuria, risk, assessment, detection, and elimination (PARADE). Pediatrics 2000; 105:1242.
- Sullivan MJ 3rd, Hough DR, Agodoa LC. Peripheral arterial thrombosis due to the nephrotic syndrome: the clinical spectrum. South Med J 1983; 76:1011.
- Yamauchi H, Hopper J Jr. Hypovolemic shock and hypotension as a complication in the nephrotic syndrome. Report of ten cases. Ann Intern Med 1964; 60:242.
- Kapur G, Valentini RP, Imam AA, Mattoo TK. Treatment of severe edema in children with nephrotic syndrome with diuretics alone--a prospective study. Clin J Am Soc Nephrol 2009; 4:907.
- Garin EH. A comparison of combinations of diuretics in nephrotic edema. Am J Dis Child 1987; 141:769.
- Deschênes G, Wittner M, Stefano A, et al. Collecting duct is a site of sodium retention in PAN nephrosis: a rationale for amiloride therapy. J Am Soc Nephrol 2001; 12:598.
- Doucet A, Favre G, Deschênes G. Molecular mechanism of edema formation in nephrotic syndrome: therapeutic implications. Pediatr Nephrol 2007; 22:1983.
- Haws RM, Baum M. Efficacy of albumin and diuretic therapy in children with nephrotic syndrome. Pediatrics 1993; 91:1142.
- Reid CJ, Marsh MJ, Murdoch IM, Clark G. Nephrotic syndrome in childhood complicated by life threatening pulmonary oedema. BMJ 1996; 312:36.
- Welch TR, Gianis J, Sheldon CA. Perforation of the scrotum complicating nephrotic syndrome. J Pediatr 1988; 113:336.
- Kerlin BA, Haworth K, Smoyer WE. Venous thromboembolism in pediatric nephrotic syndrome. Pediatr Nephrol 2014; 29:989.
- Kerlin BA, Ayoob R, Smoyer WE. Epidemiology and pathophysiology of nephrotic syndrome-associated thromboembolic disease. Clin J Am Soc Nephrol 2012; 7:513.
- Raffini L, Trimarchi T, Beliveau J, Davis D. Thromboprophylaxis in a pediatric hospital: a patient-safety and quality-improvement initiative. Pediatrics 2011; 127:e1326.
- Kerlin BA. Current and future management of pediatric venous thromboembolism. Am J Hematol 2012; 87 Suppl 1:S68.
- McIntyre P, Craig JC. Prevention of serious bacterial infection in children with nephrotic syndrome. J Paediatr Child Health 1998; 34:314.
- Liponski I, Cochat P, Gagnadoux MF, et al. [Bacterial complications of nephrotic syndrome in children]. Presse Med 1995; 24:19.
- Pasini A, Aceto G, Ammenti A, et al. Best practice guidelines for idiopathic nephrotic syndrome: recommendations versus reality. Pediatr Nephrol 2015; 30:91.
- Ulinski T, Leroy S, Dubrel M, et al. High serological response to pneumococcal vaccine in nephrotic children at disease onset on high-dose prednisone. Pediatr Nephrol 2008; 23:1107.
- Furth SL, Arbus GS, Hogg R, et al. Varicella vaccination in children with nephrotic syndrome: a report of the Southwest Pediatric Nephrology Study Group. J Pediatr 2003; 142:145.
- Goldstein SL, Somers MJ, Lande MB, et al. Acyclovir prophylaxis of varicella in children with renal disease receiving steroids. Pediatr Nephrol 2000; 14:305.
- Weng FL, Shults J, Herskovitz RM, et al. Vitamin D insufficiency in steroid-sensitive nephrotic syndrome in remission. Pediatr Nephrol 2005; 20:56.
- Freundlich M, Jofe M, Goodman WG, Salusky IB. Bone histology in steroid-treated children with non-azotemic nephrotic syndrome. Pediatr Nephrol 2004; 19:400.
- Gulati S, Godbole M, Singh U, et al. Are children with idiopathic nephrotic syndrome at risk for metabolic bone disease? Am J Kidney Dis 2003; 41:1163.
- Leonard MB, Feldman HI, Shults J, et al. Long-term, high-dose glucocorticoids and bone mineral content in childhood glucocorticoid-sensitive nephrotic syndrome. N Engl J Med 2004; 351:868.
- Prescott WA Jr, Streetman DA, Streetman DS. The potential role of HMG-CoA reductase inhibitors in pediatric nephrotic syndrome. Ann Pharmacother 2004; 38:2105.
- Coleman JE, Watson AR. Hyperlipidaemia, diet and simvastatin therapy in steroid-resistant nephrotic syndrome of childhood. Pediatr Nephrol 1996; 10:171.
- Sanjad SA, al-Abbad A, al-Shorafa S. Management of hyperlipidemia in children with refractory nephrotic syndrome: the effect of statin therapy. J Pediatr 1997; 130:470.
- Hattori M, Chikamoto H, Akioka Y, et al. A combined low-density lipoprotein apheresis and prednisone therapy for steroid-resistant primary focal segmental glomerulosclerosis in children. Am J Kidney Dis 2003; 42:1121.
- Dagan A, Cleper R, Krause I, et al. Hypothyroidism in children with steroid-resistant nephrotic syndrome. Nephrol Dial Transplant 2012; 27:2171.
- Ito S, Kano K, Ando T, Ichimura T. Thyroid function in children with nephrotic syndrome. Pediatr Nephrol 1994; 8:412.
- Gansevoort RT, de Zeeuw D, de Jong PE. Dissociation between the course of the hemodynamic and antiproteinuric effects of angiotensin I converting enzyme inhibition. Kidney Int 1993; 44:579.
- Heeg JE, de Jong PE, van der Hem GK, de Zeeuw D. Efficacy and variability of the antiproteinuric effect of ACE inhibition by lisinopril. Kidney Int 1989; 36:272.
- Salt restriction
- - Furosemide and albumin
- Fluid restriction
- Treatment of venous thromboembolism
- OTHER DIETARY MEASURES
- Caloric intake
- Calcium and vitamin D
- HYPERTENSION AND ACE INHIBITORS
- SOCIETY GUIDELINE LINKS
- SUMMARY AND RECOMMENDATIONS