背景:需要确定原发性纤毛运动障碍(PCD)和囊性纤维化(CF)中各种生理机制之间的复杂相互作用。这项研究调查了呼吸功能之间的相互作用,锻炼能力,肌肉力量,PCD和CF患者的炎症和氧化/抗氧化反应。
方法:该研究包括30例PCD患者,30例CF患者,和29名年龄和性别匹配的健康受试者。使用改良的穿梭步行测试(MSWT)评估运动能力。使用握力(HGS)来评估一般肌肉力量。还评估了氧化应激-炎症参数。通过肺活量测定法进行肺功能检查。关于1秒用力呼气量(FEV1)z评分,PCD和CF患者被细分为正常,温和,和严重/中度组。
结果:PCD和CF患者的用力肺活量(FVC)z评分低于对照组。FEV1,FEV1/FVC,峰值呼气流量(PEF),PCD和用力呼气中流量(FEF25-75%)z评分低于其他组。轻度PCD和正常CF患者的HGS均低于对照组。重度/中度PCD患者的MSWT距离低于对照组。过氧化氢酶(CAT),谷胱甘肽S-转移酶(GST),谷胱甘肽过氧化物酶(GPx),丙二醛(MDA)水平在研究组之间没有显着差异,但重度/中度PCD中的超氧化物歧化酶(SOD)水平,正常CF中的谷胱甘肽(GSH)水平高于对照组。与对照组相比,正常PCD和CF患者的白介素6(IL-6)水平更高。PCD中IL-1β水平高于对照组。此外,在一些患者组中还确定了这些参数之间的相关性.
结论:稳态与呼吸功能有关,有氧性能,肌肉力量,炎症反应,PCD和CF中的氧化剂/抗氧化剂平衡受到影响。共同评估这些机制可能有助于阐明这些罕见疾病的病理生理学。
BACKGROUND: There is a need to identify the complex interplay between various physiological mechanisms in primary ciliary dyskinesia (PCD) and cystic fibrosis (CF). The study investigated the interaction between respiratory function, exercise capacity, muscle strength, and inflammatory and oxidant/antioxidant responses in patients with PCD and CF.
METHODS: The study included 30 PCD patients, 30 CF patients, and 29 age and sex-matched healthy subjects. Exercise capacity was assessed using the modified shuttle walk test (MSWT). Handgrip strength (HGS) was used to evaluate general muscle strength. Oxidative stress-inflammatory parameters were also assessed. Pulmonary function test was performed by spirometry. Regarding the forced expiratory volume in 1 second (FEV1) z-score, patients with PCD and CF were subdivided into normal, mild, and severe/moderate groups.
RESULTS: Forced vital capacity (FVC) z-scores were lower in PCD and CF patients than controls. FEV1, FEV1/FVC, peak expiratory flow (PEF), and forced mid expiratory flow (FEF25-75%) z-scores were lower in PCD than in the other groups. HGS was lower in both mild PCD and normal CF patients relative to the controls. MSWT distance was lower in severe/moderate PCD patients than controls. Catalase (CAT), glutathione S-transferase (GST), glutathione peroxidase (GPx), and malondialdehyde (MDA) levels did not differ significantly among the study groups, but superoxide dismutase (SOD) level in severe/moderate PCD, and glutathione (GSH) level in normal CF were higher than in controls. Interleukin-6 (IL-6) level was higher in patients with normal PCD and CF compared to the controls. IL-1β level was higher in PCD compared to controls. Additionally, correlations among these parameters were also determined in some patient groups.
CONCLUSIONS: Homeostasis related to respiratory function, aerobic performance, muscle strength, inflammatory response, and oxidant/antioxidant balance were affected in PCD and CF. Evaluating these mechanisms together may contribute to elucidating the pathophysiology of these rare diseases.