Pollock-BarZiv Stacey M, Cohen Marsha M, Maclean Heather, Downey Gregory P
The Institute of Medical Sciences, University of Toronto, Canada.
Respir Med. 2005 Jul;99(7):901-9. doi: 10.1016/j.rmed.2004.12.005. Epub 2005 Jan 25.
Lymphangioleiomyomatosis (LAM), a rare pulmonary disease that occurs primarily in women is characterized by progressive cystic lung lesions causing respiratory failure, which may require lung transplantation. It has been observed that in diseases of the lungs, objective medical tests frequently do not translate to subjective functional status. However, patient's perceptions of functional status directly impact quality of life. In order to evaluate this relationship in LAM, a cross sectional study of 31 lung transplant candidates and 43 lung transplant recipients with LAM was conducted to evaluate functional status. Objective tests (Pulmonary function tests, PFTs, 6 min walk) were obtained in 19 candidates and 23 recipients, and subjective function was assessed in all women using the Functional Performance Inventory. For both groups statistically significant relationships emerged between forced vital capacity with subjective measures of function including total, social and recreational function (P < 0.05). As well diffusing capacity related significantly to total function, ability to maintain household chores, body care, and social function (P < 0.05), where higher scores were associated with better subjective function. Forced expiratory volume in one second was not found to significantly relate to subjective function in these groups, except the physical exercise domain in transplant recipients. However, despite better PFT results in double lung transplant recipients, no differences were found in subjective measures of function between single and double lung recipients. Results suggest that clinicians need to better recognize that certain aspects of spirometry/6 min walk tests do not necessarily correlate well with patient's perceptions of their function. This has implications for the assessment and follow-up of women living with LAM; considering subjective aspects of function may help focus interventions and improve quality of life.
淋巴管平滑肌瘤病（LAM）是一种主要发生于女性的罕见肺部疾病，其特征为进行性囊性肺病变，可导致呼吸衰竭，可能需要进行肺移植。据观察，在肺部疾病中，客观的医学检查结果往往不能转化为主观的功能状态。然而，患者对功能状态的认知直接影响生活质量。为了评估LAM中的这种关系，对31例LAM肺移植候选者和43例LAM肺移植受者进行了一项横断面研究，以评估功能状态。对19例候选者和23例受者进行了客观测试（肺功能测试、6分钟步行试验），并使用功能表现量表对所有女性的主观功能进行了评估。对于两组而言，用力肺活量与包括总体、社交和娱乐功能在内的主观功能测量之间均出现了具有统计学意义的关系（P<0.05）。此外，弥散能力与总体功能、维持家务、身体护理和社交功能显著相关（P<0.05），分数越高表明主观功能越好。在这些组中，除了移植受者的体育锻炼领域外，一秒用力呼气量与主观功能之间未发现显著相关性。然而，尽管双肺移植受者的肺功能测试结果更好，但单肺和双肺受者在主观功能测量方面未发现差异。结果表明，临床医生需要更好地认识到，肺量计/6分钟步行试验的某些方面不一定与患者对自身功能的认知密切相关。这对LAM女性患者的评估和随访具有启示意义；考虑功能的主观方面可能有助于聚焦干预措施并改善生活质量。
