THE BLUNTED VENTILATORY RESPONSE IN PATIENTS WITH AUTONOMIC NEUROPATHY

虽然自主神经功能障碍(AD)在许多内科疾病中已被描述，但很少有人认识到AD涉及呼吸系统的临床后遗症。为探讨全身性外周AD对呼吸机能反应的影响，我们测定了12例健康人和8例家族性淀粉样变性多发性神经病和AD(I型FAP)患者对高氧性进行性高碳酸血症的反应性。所有受试者都进行了肺活量测定，并对每个患者在休息时呼吸室内空气进行了动脉血气分析。通过使用自动调节呼气末二氧化碳分压(PETCO2)和氧分压(PETO2)的系统测量高碳酸血症反应性，根据作为PETCO2函数的通气斜率来评估。患者的静息pH和PaO_2基本在正常范围，但有5例患者的PaCO_2升高超过44毫米汞柱，且晚期AD患者的PaCO_2升高有限，且患者的平均基础分钟通气量(VE)为SI…显著低于正常人(p&lt；0.01)，但平均呼吸频率差异无统计学意义(p&gt；0.7)。FAP患者静息PaCO2与AD严重程度呈显著正相关(p&lt；0.01)，基线VE与AD呈显著负相关(p&lt；0.03)，对CO2的反应性显著低于正常人(p&lt；0.05)。这些发现提示，全身性外周自主神经功能可能在调节高碳酸血症时的通气性方面发挥作用。我们的结论是，FAP合并外周性AD应列为呼吸不足的原因。尽管自主神经功能障碍(AD)在许多内科疾病中已被描述，但很少有临床认识(AD的后遗症累及呼吸系统)。为探讨全身性外周AD对呼吸机能反应的影响，我们测定了12例健康人和8例家族性淀粉样变性多发性神经病合并AD(L FAP)患者对高氧性进行性高碳酸血症的反应性。所有受试者都进行了肺活量测定，并对每个患者在休息时呼吸室内空气进行了动脉血气分析。使用自动调节呼气末二氧化碳分压(PETCO2)和氧分压(PETO2)的系统测量高碳酸血症反应性，根据通气斜率作为PETCO2的函数进行评估。患者的静息pH和PaO2基本在正常范围，但有5例PaCO_2升高超过44 mm Hg，且晚期AD患者PaCO_2升高幅度较小。此外，患者的平均基础分钟通气量(VE)显著低于正常人(p&lt；0.01)，而平均呼吸频率的差异无统计学意义(p&gt；0.7)。FAP患者静息PaCO2与AD严重程度呈显著正相关(p&lt；0.01)，基线VE与AD呈显著负相关(p&lt；0.03)，对CO2的反应性显著低于正常人(p&lt；0.05)。这些发现提示，在高碳酸血症期间，全身自主神经功能可能在调节通气量中发挥作用。我们的结论是，FAP合并周围性AD应列为换气不足的原因。较少

Although autonomic dysfunction (AD) has been described in many medical disorders, there are little recognized clinica sequelae of AD involving respiratory system. To investigate the effect of systemic peripheral AD on ventilatory response in human, we measured the responsiveness to hyperoxic progressive hypercapnia in 12 healthy subjects and 8 patients with familial amyloidotic polyneuropathy and AD (type I FAP). Spirometry were performed in all subjects and arterial blood gas analysis was done in each patient breathing room air at rest. The hypercapnic responsiveness, measured by using a system in which end-tidal PCO2 (PETCO2) and PO2 (PETO2) were automatically regulated, were assessed in terms of the slope of ventilation as a function of the PETCO2. The resting pH and PaO2 of the patients were almost in normal range, but PaCO2 in five were elevated over 44 mmHg, and this was limited in patients with advanced AD.Furthermore the mean base line minute ventilation (VE) of patients was si … More gnificantly lower than that of normal subjects (p<0.01), however the difference of mean respiratory frequency was not significant (p>0.7). There was significant correlation between resting PaCO2 and the severity of of AD (p <0.01) and significant negative correlation between base line VE and AD (p<0.03) in patients with FAP.The magnitudes of responsiveness to CO2 of these patients were significantly lower than those of normal subjects (p<0.05). These findings suggest systemic peripheral autonomic functions may play a role in regulating ventilation during hypercapnia. We concluded FAP with peripheral AD should be listed as a cause of hypoventilation.Although autonomic dysfunction (AD) has been described in many medical disorders, there are little recognized clinica (sequelae of AD involving respiratory system. To investigate the effect of systemic peripheral AD on ventilatory response in human, we measured the responsiveness to hyperoxic progressive hypercapnia in 12 healthy subjects and 8 patients with familial amyloidotic polyneuropathy and AD (type l FAP). Spirometry were performed in all subjects and arterial blood gas analysis was done in each patient breathing roomair at rest. The hypercapnic responsiveness, measured by using a system in which end-tidal PCO2 (PETCO2) and PO2 (PETO2) were automatically regulated, were assessed n terms of the slope of ventilation as a function of the PETCO2. The resting pH and PaO2 of the patients were almost in normal range, but PaCO2 in five were elevated over 44 mmHg, and this was limited in patients with advanced AD.Furthermore the mean base line minute ventilation (VE) of patients was significantly lower than that of normal subjects (p<0.01), however the difference of mean respiratory frequency was not significant (p>0.7). There was significant correlation between resting PaC02 and the severity of of AD (p <0.01) and sinificant negative correlation between base line VE and AD (p<0.03) in patients with FAP.The magnitudes of responsiveness to CO2 of these patients were significantly lower than those of normal subjects (p<0.05). These findings suggest systemic perjpheraj autonomic functions may play a role in regualting ventilation during hypercapnia. We concluded FAP with peripheral AD should be listed as a cause of hypoventilation. Less