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Arch Pediatr. 2017 Dec;24(12):1312-1320. doi: 10.1016/j.arcped.2017.09.002. Epub 2017 Nov 13.

[Treatments of steroid-dependent nephrotic syndrome in children].

[Article in French]

Author information

1
Service de néphrologie pédiatrique, centre de référence du syndrome néphrotique idiopathique, université Paris Diderot, hôpital Robert-Debré, Assistance publique-Hôpitaux de Paris, 48, boulevard Serrurier, 75019 Paris, France. Electronic address: anne.couderc@aphp.fr.
2
Service de néphrologie pédiatrique, CHU de Nice, Archet 2, 151, route St-Antoine, 06200 Nice, France.
3
Département de pédiatrie, hôpital Mère-Enfant, 8, avenue Dominique-Larrey, 87042 Limoges cedex, France.
4
Département de pédiatrie, CHU de Nancy, rue du Morvan, 54511 Vandœuvre-lès-Nancy, France.
5
Service de néphrologie pédiatrique, centre de référence du syndrome néphrotique idiopathique, université Paris Diderot, hôpital Robert-Debré, Assistance publique-Hôpitaux de Paris, 48, boulevard Serrurier, 75019 Paris, France.
6
Service de néphrologie et transplantation, centre de référence du syndrome néphrotique idiopathique, institut francilien de recherche en néphrologie et transplantation, hôpital Henri-Mondor, Assistance publique-Hôpitaux de Paris, 51, avenue du Maréchal-de-Lattre-de-Tassigny, 94010 Créteil cedex, France.
7
Service de néphrologie pédiatrique, centre de référence du syndrome néphrotique idiopathique, institut Imagine, université Paris Descartes, hôpital Necker-Enfants-Malades, Assistance publique-Hôpitaux de Paris, 149, rue de Sèvres, 75015 Paris, France.

Abstract

Primary nephrotic syndrome (NS) is the most common glomerular disease in children. It is characterized by massive proteinuria and hypoalbuminemia. It typically has a sudden onset and more than 70% of patients will experience at least one relapse. An immunological origin has long been postulated, although the precise molecular mechanisms underlying the disease remain debated. Steroids are the first-line therapy with cumulative dose and duration of initial treatment varying among countries. Steroid-sparing agents may be indicated in case of steroid-dependency or frequent relapses. However, no consensus exists regarding the different treatment options. These treatments are mostly suspensive and therefore, need to be prolonged for several months. Levamisole, an antihelminthic drug, also has an immunomodulatory function, and alone or in combination with steroids, it can decrease cumulative steroid dose and relapses. It is usually well tolerated, and its principal side effects are cytopenia and elevated liver enzymes. Mycophenolate mofetil is an immunosuppressive agent whose reported side effects are cytopenia and diarrhea. Calcineurin inhibitors (cyclosporine or tacrolimus) have long been used in steroid-dependent patients. Their major side effects are hirsutism, gum hypertrophy, and nephrotoxicity, leading to interstitial kidney fibrosis and chronic kidney disease. Cyclophosphamide is an efficient treatment but its gonadal toxicity is a major drawback to its use. More recent drugs such as rituximab are very effective but require hospitalization for the infusion and induce an increased risk of opportunistic infection, prolonged neutropenia, and anaphylaxis. In this review, we present the available treatments, their indications, and the side effects.

PMID:
29146214
DOI:
10.1016/j.arcped.2017.09.002
[Indexed for MEDLINE]

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