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全国儿科呼吸物理治疗单位调查:原发性纤毛运动障碍和非囊性纤维化支气管扩张症

National survey on pediatric respiratory physiotherapy units: primary ciliary dyskinesia and non-CF bronchiectasis.

作者信息

Tani Beatrice, Ullmann Nicola, Leone Paola, Boni Alessandra, Barbieri Eugenio, D'Angelo Matteo, De Dominicis Sara, Giacomodonato Beniamino, Monduzzi Stefania, Piermarini Irene, Pizziconi Chiara, Ferrari Beatrice, Cutrera Renato

机构信息

Pediatric Pulmonology & Cystic Fibrosis Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Rehabilitation Unit, Meyer Children's Hospital IRCCS, Florence, Italy.

出版信息

Ital J Pediatr. 2025 Mar 6;51(1):67. doi: 10.1186/s13052-025-01904-0.

Abstract

BACKGROUND

Currently, there is a lack of data concerning the organization and characteristics of Italian pediatric physiotherapy units for the treatment of patients with chronic lung diseases, especially those with rare conditions such as Primary Ciliary Dyskinesia (PCD) and non-Cystic Fibrosis bronchiectasis (NCFB).

METHODS

A national descriptive study based on a survey questionnaire was conducted. The questionnaire consisted of three different sections: distribution and characteristics of the centres, services provided by respiratory therapists, physiotherapists' perception of the unit. The survey was distributed to all healthcare providers via an online platform, and a descriptive data analysis was performed.

RESULTS

The survey had a response rate of 97.5% with twenty-nine responses collected. The centers are heterogeneously distributed: thirteen in the northern regions, eight in the central regions and eight in the southern regions. Of the 29 centers with a physiotherapy unit, 19 had a specialized respiratory therapy unit. Respiratory therapy was provided in different care settings: regular wards (28/29 centers, 97%), outpatient service (29/29 centers, 100%), and intensive or semi-intensive care units (17/29 centers, 59%). The interventions provided by respiratory therapists involved more than just airway clearance (29/29). More specific interventions, such as pulmonary function tests (23/29), functional tests (27/29), educational training (26/29), management of workout exercise programs (25/29) and interventions developed in collaboration with physicians such as non-invasive ventilation (NIV) (23/29) and oxygen titration (21/29) are performed. It is interesting to note that therapists are also involved in various activities, such as telemedicine, physiotherapists' research projects, and supporting alongside physicians, for the prescription at home of medical devices. Perception of the unit was also evaluated.

CONCLUSIONS

The involved centers are heterogeneous in terms of distribution and treatments offered. The role of respiratory physiotherapists still seems to be fragmented. This first descriptive analysis of the physiotherapy units and the main differences between centers opens queries on the clinical approaches used for pediatric patients with PCD in terms of respiratory physiotherapy. However,in response to evolving treatment needs, a more specialized and standardized approach to patient care is required.

摘要

背景

目前,关于意大利儿科物理治疗单位治疗慢性肺部疾病患者,尤其是原发性纤毛运动障碍(PCD)和非囊性纤维化支气管扩张(NCFB)等罕见病患者的组织情况和特点,缺乏相关数据。

方法

开展了一项基于调查问卷的全国性描述性研究。问卷由三个不同部分组成:中心的分布和特点、呼吸治疗师提供的服务、物理治疗师对该单位的看法。通过在线平台向所有医疗服务提供者发放调查问卷,并进行描述性数据分析。

结果

调查的回复率为97.5%,共收集到29份回复。这些中心分布不均:北部地区有13个,中部地区有8个,南部地区有8个。在设有物理治疗单位的29个中心中,19个有专门的呼吸治疗单位。呼吸治疗在不同的护理环境中提供:普通病房(28/29个中心,97%)、门诊服务(29/29个中心,100%)以及重症或半重症监护病房(17/29个中心,59%)。呼吸治疗师提供的干预措施不仅仅涉及气道清理(29/29)。还进行了更具体的干预措施,如肺功能测试(23/29)、功能测试(27/29)、教育培训(26/29)、制定锻炼计划(25/29)以及与医生合作开展的干预措施,如无创通气(NIV)(23/29)和氧滴定(21/29)。值得注意的是,治疗师还参与各种活动,如远程医疗、物理治疗师的研究项目,以及在医生旁边提供支持,以便在家中开具医疗设备的处方。还评估了对该单位的看法。

结论

相关中心在分布和提供的治疗方面存在差异。呼吸物理治疗师的作用似乎仍然分散。对物理治疗单位的这首次描述性分析以及各中心之间的主要差异,引发了关于PCD儿科患者呼吸物理治疗临床方法的疑问。然而,为了应对不断变化治疗需求,需要一种更专业化和标准化的患者护理方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c6d9/11887135/5f9e809ab08c/13052_2025_1904_Fig1_HTML.jpg

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