Department of Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.
Eur J Neurol. 2013 Dec;20(12):1524-30. doi: 10.1111/ene.12096. Epub 2013 Feb 9.
We assessed the first evaluation at a large ventilation clinic in the Netherlands to: (i) determine what proportion of patients with motor neuron disease would benefit from earlier referral; and (ii) examine the patient preferences regarding ventilatory support.
Observational study at a single centre with a catchment area of 7.6 million inhabitants. Data on disease status, the referral process and patients' preferences regarding ventilatory support were collected during the first home ventilation services (HVS) assessment and analysed for correlation with the presence of daytime hypercapnia and suspected nocturnal hypoventilation. The latter conditions require immediate (within 48 h) or subacute (within 3 weeks) initiation of ventilatory support.
Vital capacity (in percentage of predicted value, VC%pred) was assessed by referring physicians in 84% of the 217 referred patients; the mean VC%pred was 69% (SD 16). One-hundred and ninety-one patients attended the first HVS assessment without ventilatory support, at a median of 21 days following referral: 18% had respiratory failure (daytime hypercapnia), 19% had normocapnia but were suspected of nocturnal hypoventilation, and 63% had normocapnia without symptoms. Following the HVS assessment, 25 patients (13%) declined home mechanical ventilation; this occurred more often in patients with (14/70) compared with patients without respiratory impairment (11/121; P < 0.05).
A meaningful proportion of patients who desire ventilatory support are referred to a ventilation clinic after already developing respiratory failure. Future studies could examine means, including more sensitive respiratory measures, to detect those patients who could benefit from earlier referral.
我们评估了荷兰一家大型通风诊所的首次评估，以：（i）确定有多少运动神经元疾病患者将从更早的转介中受益；（ii）检查患者对通气支持的偏好。
在一个拥有 760 万居民的集水区的单一中心进行观察性研究。在首次家庭通气服务（HVS）评估期间收集了有关疾病状况、转介过程和患者对通气支持的偏好的数据，并对其进行了分析，以与白天高碳酸血症和疑似夜间通气不足的存在相关。后一种情况需要立即（在 48 小时内）或亚急性（在 3 周内）开始通气支持。
84%的 217 名转介患者由转诊医生评估肺活量（占预计值的百分比，VC%pred）；平均 VC%pred 为 69%（SD 16）。191 名患者在没有通气支持的情况下首次参加 HVS 评估，在转诊后的中位数为 21 天：18%有呼吸衰竭（白天高碳酸血症），19%有正常碳酸血症但疑似夜间通气不足，63%有正常碳酸血症且无症状。在 HVS 评估后，25 名患者（13%）拒绝家庭机械通气；在有呼吸损害的患者中（70 例中的 14 例）比在没有呼吸损害的患者中（121 例中的 11 例）更常见（P <0.05）。
有相当一部分希望接受通气支持的患者在已经出现呼吸衰竭后被转介到通气诊所。未来的研究可以研究手段，包括更敏感的呼吸措施，以检测那些可能从更早的转介中受益的患者。
