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免疫球蛋白轻链淀粉样变性的营养状况前瞻性研究。

A prospective study of nutritional status in immunoglobulin light chain amyloidosis.

机构信息

National Amyloidosis Centre, Centre for Amyloidosis & Acute Phase Proteins, University College London, UK.

出版信息

Haematologica. 2013 Jan;98(1):136-40. doi: 10.3324/haematol.2012.070359. Epub 2012 Sep 14.

Abstract

Weight loss is common in systemic immunoglobulin light chain amyloidosis but there are limited data on the impact of nutritional status on outcome. Using the Patient-Generated Subjective Global Assessment (PG-SGA) score, we prospectively examined nutritional status in 110 consecutive newly-diagnosed, treatment-naïve patients with immunoglobulin light chain amyloidosis attending the UK National Amyloidosis Centre. At study entry, 72 of 110 (66%) patients had a PG-SGA score of 4 or over, indicating malnutrition requiring specialist nutritional intervention. Number of amyloidotic organs, elevated alkaline phosphatase, presence of autonomic neuropathy and advanced Mayo disease stage were independently associated with poor nutritional status (P<0.05). Quality of life was substantially poorer among those with higher PG-SGA scores (P<0.001). Furthermore, PG-SGA score was a powerful independent predictor of patient survival (P=0.02). Malnutrition is prevalent and is associated with poor quality of life and reduced survival among patients with systemic immunoglobulin light chain amyloidosis. The PG-SGA score would be an appropriate tool to evaluate whether nutritional intervention could improve patient outcomes.

摘要

体重减轻在系统性免疫球蛋白轻链淀粉样变性中很常见,但关于营养状况对预后的影响的数据有限。我们使用患者生成的主观整体评估(PG-SGA)评分,前瞻性地检查了 110 例新诊断、未经治疗的免疫球蛋白轻链淀粉样变性患者的营养状况,这些患者均在英国国家淀粉样变性中心就诊。在研究开始时,110 例患者中有 72 例(66%)PG-SGA 评分为 4 分或以上,表明存在营养不良,需要专业的营养干预。淀粉样变性器官的数量、碱性磷酸酶升高、自主神经病的存在和 Mayo 疾病晚期是与不良营养状况相关的独立因素(P<0.05)。PG-SGA 评分较高的患者生活质量明显较差(P<0.001)。此外,PG-SGA 评分是患者生存的有力独立预测因子(P=0.02)。营养不良在系统性免疫球蛋白轻链淀粉样变性患者中很常见,与生活质量差和生存率降低有关。PG-SGA 评分将是评估营养干预是否可以改善患者预后的合适工具。

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