Hereditary Nephritis and Thin Glomerular Basement Membrane Lesion.

Mark A Lusco, Agnes B Fogo
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引用次数: 2

Abstract

Hereditary nephritis (HN) and thin glomerular basement membrane (GBM) lesion share a common clinical presentation of persistent hematuria, thin GBM by kidney biopsy electron microscopic examination, and a mutation in type IV collagen. However, the clinical course and treatment for these entities are different with varying patterns of heredity. Ultrastructural examination of a renal biopsy specimen is essential for the morphologic diagnosis of HN and thin GBM lesion, whereas light microscopy may only give limited diagnostic clues. Additional workup including immunostaining for subtypes of type IV collagen may provide further information on underlying genetic mutations. The diagnosis of HN may lead to treatment with renin-angiotensin system blockade in patients at risk of early-onset renal failure to delay progression to end-stage renal disease. Additionally, patients with isolated microscopic hematuria and thin GBM lesion are at increased risk for chronic kidney disease when associated with other comorbidities; those patients should receive regular clinical assessment to prevent renal function decline.

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遗传性肾炎与肾小球基底膜薄病变。
遗传性肾炎(HN)和薄的肾小球基底膜(GBM)病变有一个共同的临床表现,持续血尿,肾活检电镜检查薄的GBM,以及IV型胶原突变。然而,这些实体的临床过程和治疗是不同的遗传模式。肾活检标本的超微结构检查对于HN和薄性GBM病变的形态学诊断至关重要,而光镜检查可能只能提供有限的诊断线索。额外的检查包括IV型胶原蛋白亚型的免疫染色可以提供有关潜在基因突变的进一步信息。HN的诊断可能导致有早发性肾衰竭风险的患者接受肾素-血管紧张素系统阻断治疗,以延缓进展为终末期肾脏疾病。此外,孤立的显微镜下血尿和薄的GBM病变的患者,当与其他合并症相关时,慢性肾脏疾病的风险增加;这些患者应定期接受临床评估,以防止肾功能下降。
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