PDF(Pubmed)
Abstract：
\"Cast nephropathy\" (CN) is a pathological feature of myeloma kidney, also seen to a lesser extent in the context of severe nephrotic syndrome from non-haematological diseases. The name relates to obstruction of distal tubules by \"casts\" of luminal proteins concentrated by intensive water reabsorption resulting from dehydration or high-dose diuretics. Filtered proteins form complexes with endogenous tubular Tamm-Horsfall glycoprotein. The resulting gel further slows or stops luminal flow upon complete obstruction of distal convoluted tubules and collecting ducts. Thus, a tubular obstructive form of acute kidney injury (AKI) is a common consequence of CN. The pathogenesis of CN will be reviewed in light of recent advances in the understanding of monoclonal disorders of B lymphocytes, leading to the release of immunoglobulin components (free light chains, FLC) into the bloodstream and their filtration across the glomerular basement membrane. Treatment aiming at reduction of the circulating burden of FLC may help recovery of renal function in a fraction of these patients, besides filling the void between the onset of AKI, histopathological diagnosis, and full response to pharmacologic treatment.
摘要：
“铸型肾病”（CN）是骨髓瘤肾脏的病理特征，在非血液疾病的严重肾病综合征的情况下，也有较小的程度。该名称涉及由脱水或高剂量利尿剂引起的强烈的水重吸收而浓缩的腔内蛋白质的“铸件”阻塞远端小管。过滤的蛋白质与内源性管状Tamm-Horsfall糖蛋白形成复合物。在完全阻塞远端回旋小管和收集管时,所得的凝胶进一步减慢或停止管腔流动。因此，急性肾损伤（AKI）的肾小管阻塞形式是CN的常见后果。将根据对B淋巴细胞单克隆疾病的认识的最新进展，对CN的发病机制进行综述。导致免疫球蛋白成分的释放（游离轻链，FLC）进入血流并通过肾小球基底膜过滤。旨在减少FLC循环负担的治疗可能有助于部分患者的肾功能恢复。除了填补AKI发作之间的空白，组织病理学诊断,以及对药物治疗的全面反应。
