Fregonezi G A de Freitas, Regiane-Resqueti V, Pradas J, Vigil L, Casan P
Departament de Pneumologia, Hospital de la Santa Creu i de Sant Pau, Facultat de Medicina. Universitat Autònoma de Barcelona, Barcelona, España.
Arch Bronconeumol. 2006 May;42(5):218-24. doi: 10.1016/s1579-2129(06)60449-7.
To study lung function abnormalities and health-related quality of life (HRQL) in a group of patients diagnosed with generalized myasthenia gravis, and to analyze the relationship between these 2 sets of variables.
In a prospective study of 20 patients diagnosed with generalized myasthenia gravis, the following parameters were measured: spirometry, static lung volumes, breathing pattern, maximal respiratory pressures, and maximum voluntary ventilation. HRQL was assessed using the 36-item short form general health questionnaire (SF-36).
The mean (SD) age of the patients was 64 (11) years. Patients were classified into 2 groups depending on whether they had IIa (12 patients) or IIb (8 patients) type disease. A small decrease in total lung capacity (86%) and slight reductions in maximal inspiratory pressure (88%) and maximum voluntary ventilation (63% in group IIb) were observed. The HRQL domains most affected were those related to physical activity and self-perceived health status in all groups, although women were more affected. The scores relating to vitality and physical activity were found to be significantly associated with forced vital capacity and lung volumes. Tidal volume was associated with maximal inspiratory and expiratory pressures, inspiratory capacity, and maximum voluntary ventilation. The respiratory rate to tidal volume ratio was inversely associated with the first three of these variables.
A very slight restrictive pattern and a reduction in inspiratory muscle strength were observed. The HRQL domains most affected were those related to physical activity and the patients' self-perceived health status. The weakness of the respiratory muscles contributes to the abnormalities observed in lung function and to the deterioration of health-related quality of life.
研究一组被诊断为全身型重症肌无力患者的肺功能异常情况及健康相关生活质量（HRQL），并分析这两组变量之间的关系。
对20例被诊断为全身型重症肌无力的患者进行前瞻性研究，测量以下参数：肺量计检查、静态肺容量、呼吸模式、最大呼吸压力和最大自主通气量。使用36项简短形式一般健康问卷（SF-36）评估HRQL。
患者的平均（标准差）年龄为64（11）岁。根据患者患有IIa型（12例）或IIb型（8例）疾病，将患者分为两组。观察到总肺容量略有下降（86%），最大吸气压力略有降低（88%），最大自主通气量在IIb组中降低（63%）。在所有组中，受影响最大的HRQL领域是与身体活动和自我感知健康状况相关的领域，尽管女性受影响更大。发现与活力和身体活动相关的得分与用力肺活量和肺容量显著相关。潮气量与最大吸气和呼气压力、吸气容量和最大自主通气量相关。呼吸频率与潮气量之比与前三个变量呈负相关。
观察到非常轻微的限制性模式和吸气肌力量降低。受影响最大的HRQL领域是与身体活动和患者自我感知健康状况相关的领域。呼吸肌无力导致肺功能异常及健康相关生活质量恶化。
