儿童原发性肾病综合症治理方案(英文)

Management strategy for idiopathic nehprotic syndrome in children

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。

Corticosteroid, alkylating agents, like cyclophosphamide and chlorambucil, nave been used to treat idiopathic nephrotic syndrome for more than fifty years, changing the outcome of these children. However, with long-term use of steroid, especially high dosages, they have unbearable side effects. Newer agents like cyclosporine A, levamisole, tacrolimus, mycophenolate mofetil, have been used to spare those unwanted side effects. In the choice of drugs, the benefits obtained will have to be evaluated against possible side effects, with drug cost also taken into consideration. Though most steroid sensitive nephrotic children may run a relapsing course, have a good prognosis with many becoming non-relapsers or infrequent relapsers in adulthood, the treatment approach should aim at using the minimal amount of drug required to keep patient in remission to tie them over childhood. As for steroid resistant nephrotic syndrome children, especially for focal segmental glomerulosclerosis （ FSGS ） , because of possible grave prognosis of going into end-stage renal failure, more aggressive approach should be adopted, including the use of strong immunosuppressants, such as, cyclosporine, tacrolimus, or mycophenolate mofetil if necessary. The long-term goals of treatment, other than those of physical and medical conditions, should also consider the growth, education, and psychological impact of the disease and side effects of drugs on the child, especially during an adolescent period, so as to allow them having normal development into adulthood.