■呼吸功能不全是成骨不全症(OI)患者死亡的主要原因。然而,评估OI的肺功能存在挑战。常用的肺功能检查如肺活量测定和身体体积描记术有时很难对OI患者进行,和参考间隔并不总是适用的。强制振荡技术(FOT)是一种对患者友好的方法,用于检测呼吸异常,无需患者付出任何努力。
■本研究探讨了FOT在OI患者临床治疗中评估呼吸功能的可行性。
■12名OI患者,包括八个与SillenceOI,两个与OIIV,还有两个是OIIII,做了肺活量测定,身体体积描记术,FOT,沙丁胺醇给药前后。
■FOT测量显示出与肺活量测定和身体体积描记术结果一致的趋势。服用沙丁胺醇后,8Hz时的电阻降低,表明FOT能够检测支气管阻塞及其通过药物缓解(p<0.05)。几乎所有患者呼气时的共振频率都高于吸气时的共振频率,提示阻塞性疾病。该技术可深入了解肺通气的吸气和呼气损害。主要FOT参数在重复测量中显示出相对较高的可重复性。
■OI患者在安静呼吸期间可以通过FOT检测到支气管阻塞,使其成为其他肺功能测量的容易执行的替代方案。该技术可以检测拟交感神经药物的支气管扩张剂作用。它有可能提供有关呼气流量限制的信息,肺限制,并降低肺顺应性。
UNASSIGNED: Respiratory insufficiency is a leading cause of death in individuals with osteogenesis imperfecta (OI). However, evaluating pulmonary function in OI presents challenges. Commonly used pulmonary function tests such as spirometry and body plethysmography are sometimes difficult to perform for OI patients, and reference intervals are not always applicable. The forced oscillation technique (FOT) is a patient-friendly method for detecting respiratory abnormalities that requires no effort from the patient.
UNASSIGNED: This study investigates the feasibility of FOT in the evaluation of respiratory function in the clinical management of OI patients.
UNASSIGNED: Twelve OI patients, comprising eight with Sillence OI I, two with OI IV, and two with OI III, underwent spirometry, body plethysmography, and FOT, both pre-and post-administration of salbutamol.
UNASSIGNED: FOT measurements exhibited consistent trends that aligned with spirometry and body plethysmography findings. The resistance at 8 Hz decreased after the administration of salbutamol, indicating that FOT is able to detect bronchial obstruction and its alleviation by medication (p < 0.05). The resonant frequency during expiration was higher than during inspiration in nearly all patients, suggesting obstructive disease. The technique gives insight into both inspiratory and expiratory impairment of pulmonary ventilation. The main FOT parameters showed a relatively high repeatability in duplicate measurements.
UNASSIGNED: Bronchial obstruction can be detected by FOT in patients with OI during quiet breathing, making it an easily executable alternative to other lung function measurements. The technique can detect the bronchodilator effect of sympathomimetic medication. It has the potential to provide information on expiratory flow limitation, pulmonary restriction, and reduced lung compliance.