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皮质激素、烷基化物如环磷酰胺、苯丁酸氮芥用于原发性肾病综合症近50年,改变了肾病综合症儿童的预后。然而,长期使用激素,尤其较高剂量却引起严重副作用。较新药物如环孢A、左旋咪唑、他克莫司、霉酚酸酯可免去不少激素副作用。在选择药物时,应衡量及平衡效益与副作用,并考虑药物价格。虽然大部份肾病综合症儿童都会复发,然而预后却良好,不少肾病综合症儿童成长后不再复发,或只间有复发,故治疗方案应以最少药物以维持缓解,以过渡儿童期,避免不必要的副作用。至于激素抵抗(SRNS)者,尤其局部节段性肾小球硬化(FSGS),预后可致终末期肾衰;治疗方面,用药可较为进取,多需较强免疫抑制剂如环孢A、他克莫司、霉酚酸酯等加以控制。治疗肾病综合症儿童的长期目标,还需考虑生长、教育与心理影响,尤其在过渡青春期时,能让他们正常地成长。
Corticosteroids, alkylates such as cyclophosphamide, chlorambucil for primary nephrotic syndrome for nearly 50 years have changed the prognosis of children with nephrotic syndrome. However, long-term use of hormones, especially at higher doses, causes serious side effects. Newer drugs such as cyclosporine A, levamisole, tacrolimus, mycophenolate can eliminate a lot of hormonal side effects. When choosing a drug, measure and balance the benefits and side effects and consider the price of the drug. Although most children with nephrotic syndrome will relapse, however, the prognosis is good. Many children with nephrotic syndrome will not relapse after their growth or only relapse. Therefore, the treatment plan should be at least the least in order to ease the transition to childhood, Avoid unnecessary side effects. As for hormone resistance (SRNS), especially in localized segmental glomerulosclerosis (FSGS), the prognosis can cause end-stage renal failure; treatment, medication can be more aggressive, require more strong immunosuppressive agents such as cyclosporine A, he Cremophor, mycophenolate, etc. to be controlled. The long-term goal of treating children with nephrotic syndrome needs to be considered for growth, education and psychosocial impact, especially during adolescence, allowing them to grow normally.