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Membranous nephropathy in children: clinical presentation and therapeutic approach

机译:儿童膜性肾病的临床表现和治疗方法

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摘要

The approach to the pediatric patient with membranous nephropathy (MN) can be challenging to the practitioner. The clinical presentation of the child with this histologic entity usually involves some degree of proteinuria ranging from persistent, subnephrotic-ranged proteinuria to overt nephrotic syndrome. Patients often have accompanying microscopic hematuria and may have azotemia or mild hypertension. Children presenting with nephrotic syndrome are often steroid resistant; as such, their biopsy for steroid-resistant nephrotic syndrome results in the diagnosis of MN. The practitioner treating MN in the pediatric patient must weigh the risks of immunosuppressive therapy against the benefits. In general, the child with subnephrotic proteinuria and normal renal function can likely be treated conservatively with angiotensin blockade (angiotensin-converting enzyme inhibitors or angiotensin receptor blockers) without the need for immunosuppressive therapy. Those with nephrotic syndrome are usually treated with steroids initially and often followed by alkylating agents (cyclophosphamide or chlorambucil). Calcineurin inhibitors may also be useful, but the relapse rate after their discontinuation remains high. The absence of controlled studies in children with MN makes treatment recommendations difficult, but until they are available, using the patient’s clinical presentation and risk of disease progression appears to be the most prudent approach.
机译:患有膜性肾病(MN)的小儿患者的治疗方法可能对从业者构成挑战。具有这种组织学特征的孩子的临床表现通常涉及一定程度的蛋白尿,范围从持续性,亚肾病性蛋白尿到明显的肾病综合症。患者常伴有镜下血尿,并可能患有氮质血症或轻度高血压。患有肾病综合征的儿童通常对类固醇有抵抗力。因此,他们的类固醇抵抗性肾病综合征活检可诊断为MN。在小儿患者中治疗MN的从业者必须权衡免疫抑制治疗的风险和益处。一般而言,患有肾功能不全和肾功能正常的儿童可以采用血管紧张素阻滞剂(血管紧张素转换酶抑制剂或血管紧张素受体阻滞剂)进行保守治疗,而无需进行免疫抑制治疗。患有肾病综合症的患者通常首先使用类固醇治疗,然后通常使用烷基化剂(环磷酰胺或苯丁酸氮芥)治疗。钙调磷酸酶抑制剂也可能有用,但停药后的复发率仍然很高。缺乏针对MN儿童的对照研究使治疗建议变得困难,但是在可行之前,使用患者的临床表现和疾病进展风险似乎是最谨慎的方法。

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