Gluten-Free Diet in Childhood Difficult-to-Treat Nephrotic Syndrome: A Pilot Feasibility Study.
Gluten-Free Diet in Childhood Difficult-to-Treat Nephrotic Syndrome: A Pilot Feasibility Study.
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DOI：
10.1159/000525587
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发表时间：
2022
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在高达40%的病例中，儿童时期的微小病症可能伴随着频繁复发或类固醇依赖的病程。用于治疗这些患者的二线免疫抑制药物与显著的不良反应有关。有必要为难治性肾病综合征寻找更安全的替代疗法。因此，我们进行了一项开放标签的可行性研究，以评估无麸质饮食作为治疗难治性肾病综合征儿童患者的安全性和有效性。作为第二个目标，我们试图确定血浆卓诺林浓度是否可以识别那些更有可能对这种干预做出反应的人。17名患者接受为期6个月的无麸质饮食。阳性反应被定义为与前6个月相比复发率降低50%或停用一种免疫抑制药物的能力。5名参与者(29%)对饮食干预反应积极。无麸质饮食耐受性良好，没有临床或实验室不良反应。在无法从无面筋饮食中获益的患者中，血浆中的氮蛋白浓度升高。对于难治性肾病综合征患者，无麸质饮食可能是一种有用的辅助干预措施，可以在诉诸二线免疫抑制治疗之前实施。发展血浆带状蛋白水平作为预测疗效的生物标志物将有助于在肾病综合征的治疗中合理使用无面筋饮食。
Minimal change disease in childhood can follow a frequently relapsing or steroid-dependent course in up to 40% of cases. Second-line immunosuppressive medications that are used to manage these patients are associated with significant adverse effects. There is a need for safer alternative treatments for difficult-to-treat nephrotic syndrome. Therefore, we conducted an open-label feasibility study to assess the safety and efficacy of a gluten-free diet as treatment for pediatric patients with difficult-to-treat nephrotic syndrome. As a second aim, we sought to determine if the plasma zonulin concentration can identify those who are more likely to respond to this intervention. Seventeen patients were placed on a gluten-free diet for 6 months. A positive response was defined as a 50% reduction in the relapse rate compared to the preceding 6 months or the ability to discontinue 1 immunosuppressive drug. Five (29%) participants had a positive response to the dietary intervention. The gluten-free diet was well tolerated with no clinical or laboratory adverse events. Plasma zonulin concentration was elevated in patients who failed to benefit from the gluten-free diet. A gluten-free diet may be a useful adjunctive intervention for patients with difficult-to-treat nephrotic syndrome that can be implemented prior to resorting to second-line immunosuppressive therapy. Development of the plasma zonulin level as a biomarker to predict efficacy would facilitate rational use of a gluten-free diet in the management of nephrotic syndrome.