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• W2768210847 abstract "Le syndrome néphrotique idiopathique est le plus fréquent des glomérulopathies de l’enfant. Il se définit par une protéinurie abondante et une hypo-albuminémie. La physiopathologie de cette maladie reste largement incomprise, mais elle survient le plus souvent de façon brutale. Le traitement de première intention est la corticothérapie orale, dont il existe différents protocoles de traitement à travers le monde. Ce traitement permet d’induire une rémission dans environ 90 % des cas. Cependant, 70 % de ces patients rechutent et parmi eux, 60 % ont 5 rechutes ou plus. Les patients sont dits cortico-dépendants si une rechute survient pendant la diminution des doses de corticoïdes ou dans les 3 mois suivant leur arrêt. Des traitements d’épargne cortisonique doivent être utilisés en cas de rechutes répétées ou pour les patients cortico-dépendants, mais il n’existe pas de consensus pour ces traitements. Il peut s’agir de molécules utilisées de longue date ou d’autres beaucoup plus récentes, de nouvelles études les concernant paraissant régulièrement. Dans cette mise au point, nous présentons une revue de la littérature des différents traitements disponibles, de leurs indications et de leurs effets indésirables. 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." @default.
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• W2768210847 date "2017-12-01" @default.
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• W2768210847 title "Traitements du syndrome néphrotique cortico-dépendant de l’enfant" @default.
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• W2768210847 doi "https://doi.org/10.1016/j.arcped.2017.09.002" @default.
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