Gbadegesin Rasheed A, Hernandez Loren P Herrera, Brophy Patrick D
Division of Nephrology, Department of Pediatrics, Duke University School of Medicine, Durham, NC, United States.
Division of Anatomic Pathology, Mayo Clinic, Department of Pathology, Rochester, MN, United States.
Front Pediatr. 2021 May 4;9:614948. doi: 10.3389/fped.2021.614948. eCollection 2021.
Minimal change disease (MCD) is the most common cause of nephrotic syndrome worldwide. For decades, the foundation of the treatment has been corticosteroids. However, relapse rate is high and up to 40% of patients develop frequent relapsing/steroid dependent course and one third become steroid resistant. This requires treatment with repeated courses of corticosteroids, and second and third line immunomodulators increasing the incidence of drug related adverse effects. More recently, there have been reports of a very small subset of Nephrotic Syndrome (NS) patients who are initially steroid sensitive and later become secondarily steroid resistant. The disease course in this small subset is often protracted leading ultimately to end stage kidney disease requiring dialysis or kidney transplantation. Unfortunately, patients with this disease course do not do well post transplantation because 80% of them will develop disease recurrence that will ultimately lead to graft failure. Few approaches have been tried over many years to reduce the frequency of relapses, and steroid dependence and there is absolutely no therapeutic intervention for patients who develop secondary steroid resistance. Nonetheless, their therapeutic index is low, evidencing the need of a safer complementary treatment. Several hypotheses, including an oxidative stress-mediated mechanism, and immune dysregulation have been proposed to date to explain the underlying mechanism of Minimal Change Disease (MCD) but its specific etiology remains elusive. Here, we report a case of a 54-year-old man with steroid and cyclosporine resistant MCD. The patient rapidly progressed to end stage kidney disease requiring initiation of chronic dialysis. Intradialytic parenteral nutrition (IDPN), albumin infusion along with a proprietary dietary supplement, as part of the supportive therapy, led to kidney function recovery and complete remission of MCD without relapses.
微小病变病（MCD）是全球范围内肾病综合征最常见的病因。几十年来，治疗的基础一直是使用皮质类固醇。然而，复发率很高，高达40%的患者会发展为频繁复发/类固醇依赖病程，三分之一的患者会出现类固醇抵抗。这就需要反复使用皮质类固醇疗程进行治疗，以及使用二线和三线免疫调节剂，从而增加了药物相关不良反应的发生率。最近，有报道称一小部分肾病综合征（NS）患者最初对类固醇敏感，后来继发类固醇抵抗。这一小部分患者的病程往往迁延不愈，最终导致终末期肾病，需要进行透析或肾移植。不幸的是，患有这种病程的患者移植后情况不佳，因为其中80%会出现疾病复发，最终导致移植失败。多年来尝试了几种方法来降低复发频率和类固醇依赖性，对于继发类固醇抵抗的患者绝对没有治疗干预措施。尽管如此，这些治疗方法的治疗指数较低，表明需要一种更安全的辅助治疗方法。迄今为止，已经提出了几种假说，包括氧化应激介导的机制和免疫失调，以解释微小病变病（MCD）的潜在机制，但其具体病因仍然难以捉摸。在此，我们报告一例54岁男性患有类固醇和环孢素抵抗的MCD。该患者迅速进展为终末期肾病，需要开始进行慢性透析。作为支持治疗的一部分，透析期间胃肠外营养（IDPN）、输注白蛋白以及一种专利膳食补充剂，导致肾功能恢复，MCD完全缓解且无复发。
