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1.
Respirar (Ciudad Autón. B. Aires) ; 16(2): 161-168, Junio 2024.
Artigo em Espanhol | LILACS, UNISALUD, BINACIS | ID: biblio-1556158

RESUMO

La terapia de alto flujo se ha popularizado durante los últimos años, basada en sus efectos fisiológicos, la entrega de una fracción inspirada de oxígeno segura y estable, sumada al flujo calefaccionado y humidificado, lo que hizo posible su utilización en distintos escenarios. Sin embargo, los estudios que muestran estos beneficios y efectos se han realizado, principalmente, con el empleo de una cánula nasal; mientras que las características de esta terapia en los pacientes traqueostomizados no se ha desarrollado suficientemente. Proponemos aquí una revisión narrativa con las características más salientes de la terapia de alto flujo en este subgrupo de pacientes.


High-flow therapy has become popular in recent years, based on its physiological effects, the delivery of a safe and stable inspired fraction of oxygen, combined with heated and humidified flow, which made its use possible in different scenarios. However, studies demonstrating these benefits and effects have been mainly conducted using a nasal cannula, while the characteristics of this therapy in tracheostomized patients have not been sufficiently developed. We propose a narrative review highlighting the most relevant characteristics of high-flow therapy in this subgroup of patients.


Assuntos
Humanos , Masculino , Feminino , Terapia Respiratória/métodos , Traqueostomia/estatística & dados numéricos , Respiração Artificial , Revisão , Cuidados Críticos , Cânula
2.
Medicina (Kaunas) ; 60(6)2024 May 23.
Artigo em Inglês | MEDLINE | ID: mdl-38929470

RESUMO

Background and Objectives: Inadequate treatment of asthma and chronic obstructive pulmonary disease (COPD) might have a negative impact on their progression. Inhalation therapy is the cornerstone of pharmacotherapy for these conditions. However, challenges such as low adherence, negative attitudes, and misconceptions about inhaled medications still persist, impeding effective disease management. This study aimed to evaluate adherence, ascertain the level of disease control in asthma and COPD, explore potential misconceptions surrounding inhalation therapy among patients with obstructive lung diseases and the general population in Vojvodina, and evaluate the reliability of newly developed questionnaires employed in the study. Materials and Methods: This cross-sectional study utilized a battery of questionnaires encompassing sociodemographic data, the Asthma Control Test (ACT), the COPD Assessment Test (CAT), along with two novel questionnaires-one for assessing adherence and another for analyzing attitudes toward inhalation therapy. Statistical analyses were conducted using SPSS software, version 25.0. Results: The average ACT score among patients with asthma was 17.31, while it was 19.09 for the CAT questionnaire among COPD patients. The composite score on the newly developed adherence assessment questionnaire was 2.27, exhibiting a reliability coefficient lower than recommended (α = 0.468). Significant statistical differences emerged among sample subgroups regarding attitudes and misconceptions toward inhalation therapy. The reliability coefficient for this questionnaire was deemed satisfactory (α = 0.767). Conclusions: Adherence rates were notably suboptimal in both subgroups of the studied population. The disease control levels were higher among asthma patients, while they exhibited less prevalent misconceptions regarding inhalation therapy compared to COPD patients and the healthy population.


Assuntos
Asma , Adesão à Medicação , Doença Pulmonar Obstrutiva Crônica , Humanos , Estudos Transversais , Masculino , Feminino , Pessoa de Meia-Idade , Inquéritos e Questionários , Doença Pulmonar Obstrutiva Crônica/tratamento farmacológico , Adulto , Administração por Inalação , Idoso , Adesão à Medicação/estatística & dados numéricos , Adesão à Medicação/psicologia , Asma/tratamento farmacológico , Terapia Respiratória/métodos , Terapia Respiratória/estatística & dados numéricos , Reprodutibilidade dos Testes
3.
Respir Care ; 69(6): 697-712, 2024 May 28.
Artigo em Inglês | MEDLINE | ID: mdl-38806225

RESUMO

Over the last 3 decades, pulmonary rehabilitation (PR) has become an integral part of the management of COPD. Many other chronic respiratory diseases have similar systemic manifestations including skeletal muscle impairment, commonly through deconditioning, and may benefit from PR. However, whereas many programs may accept patients with other respiratory diseases, the program may need several adaptations to optimally manage patients. This article uses the examples of interstitial lung disease including idiopathic pulmonary fibrosis, bronchiectasis, pulmonary hypertension, post lung transplantation, and post-COVID condition to highlight exemplar clinical problems. In addition, the rationale and latest evidence for PR are described alongside the adaptations to the program, including education needs of the delivery team and close integrated care with the wider clinical team. Finally, future directions for clinical care and research are discussed.


Assuntos
COVID-19 , Transplante de Pulmão , Humanos , Doença Crônica , COVID-19/complicações , COVID-19/reabilitação , Transplante de Pulmão/reabilitação , Doença Pulmonar Obstrutiva Crônica/reabilitação , Doenças Pulmonares Intersticiais/reabilitação , Hipertensão Pulmonar/reabilitação , SARS-CoV-2 , Bronquiectasia/reabilitação , Terapia Respiratória/métodos
4.
Eur Respir J ; 63(6)2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38609097

RESUMO

BACKGROUND: International guidelines recommend airway clearance management as one of the important pillars of bronchiectasis treatment. However, the extent to which airway clearance is used for people with bronchiectasis in Europe is unclear. The aim of the study was to identify the use of airway clearance management in patients with bronchiectasis across different countries and factors influencing airway clearance use. METHODS: This was a prospective observational study using data from the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) Registry between January 2015 and April 2022. Prespecified options for airway clearance management were recorded, including airway clearance techniques, devices and use of mucoactive drugs. RESULTS: 16 723 people with bronchiectasis from 28 countries were included in the study. The mean age was 67 years (interquartile range 57-74 years, range 18-100 years) and 61% were female. 72% of the participants reported daily sputum expectoration and 52% (95% CI 51-53%) of all participants reported using regular airway clearance management. Active cycle of breathing technique was used by 28% of the participants and airway clearance devices by 16% of participants. The frequency of airway clearance management and techniques used varied significantly between different countries. Participants who used airway clearance management had greater disease severity and worse symptoms, including a higher daily sputum volume, compared to those who did not use it regularly. Mucoactive drugs were also more likely to be used in participants with more severe disease. Access to specialist respiratory physiotherapy was low throughout Europe, but particularly low in Eastern Europe. CONCLUSIONS: Only a half of people with bronchiectasis in Europe use airway clearance management. Use of and access to devices, mucoactive drugs and specialist chest physiotherapy appears to be limited in many European countries.


Assuntos
Bronquiectasia , Sistema de Registros , Humanos , Bronquiectasia/terapia , Bronquiectasia/fisiopatologia , Feminino , Pessoa de Meia-Idade , Masculino , Idoso , Europa (Continente) , Adulto , Estudos Prospectivos , Adolescente , Adulto Jovem , Idoso de 80 Anos ou mais , Manuseio das Vias Aéreas/métodos , Terapia Respiratória/métodos , Expectorantes/uso terapêutico
5.
Eur J Cardiothorac Surg ; 65(3)2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38489838

RESUMO

This clinical case shows the repercussions of acute exposure to cement dust in the respiratory tract and other mucous membranes. Following a cement dust pipe explosion, the patient endured a severe inhalation of dust. A combination of rigid bronchoscopy and a mechanical insufflation-exsufflation system was employed to remove cement debris from the airways. Respiratory physiotherapy sessions were implemented for effective secretion clearance, contributing to a successful short-term recovery. While this remains an isolated case, the unconventional techniques employed provide valuable insights for potential similar scenarios in the future.


Assuntos
Insuflação , Humanos , Insuflação/métodos , Broncoscopia , Respiração Artificial , Terapia Respiratória/métodos , Modalidades de Fisioterapia , Tosse
6.
J Nanobiotechnology ; 22(1): 136, 2024 Mar 29.
Artigo em Inglês | MEDLINE | ID: mdl-38553716

RESUMO

Pulmonary fibrosis (PF) threatens millions of people worldwide with its irreversible progression. Although the underlying pathogenesis of PF is not fully understood, there is evidence to suggest that the disease can be blocked at various stages. Inhalation therapy has been applied for lung diseases such as asthma and chronic obstructive pulmonary disease, and its application for treating PF is currently under consideration. New techniques in inhalation therapy, such as the application of microparticles and nanoparticles, traditional Chinese medicine monomers, gene therapy, inhibitors, or agonists of signaling pathways, extracellular vesicle interventions, and other specific drugs, are effective in treating PF. However, the safety and effectiveness of these therapeutic techniques are influenced by the properties of inhaled particles, biological and pathological barriers, and the type of inhalation device used. This review provides a comprehensive overview of the pharmacological, pharmaceutical, technical, preclinical, and clinical experimental aspects of novel inhalation therapy for treating PF and focus on therapeutic methods that significantly improve existing technologies or expand the range of drugs that can be administered via inhalation. Although inhalation therapy for PF has some limitations, the advantages are significant, and further research and innovation about new inhalation techniques and drugs are encouraged.


Assuntos
Asma , Doença Pulmonar Obstrutiva Crônica , Fibrose Pulmonar , Humanos , Fibrose Pulmonar/tratamento farmacológico , Administração por Inalação , Doença Pulmonar Obstrutiva Crônica/tratamento farmacológico , Asma/tratamento farmacológico , Terapia Respiratória
11.
BMC Pulm Med ; 24(1): 88, 2024 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-38360672

RESUMO

BACKGROUND: Incentive spirometry (IS) as a routine respiratory therapy during the perioperative period has been widely used in clinical practice. However, the impact of IS on patients with perioperative lung cancer remains controversial. This review aimed to evaluate the efficacy of IS in perioperative pulmonary rehabilitation for patients with lung cancer. METHODS: Cochrane Library, PubMed, Web of Science, Ovid, CINAHL, Chinese National Knowledge Infrastructure, Weipu, and Wanfang Databases were searched from inception to 30 November 2023. Only randomized controlled trials were included in this systematic review. The PRISMA checklist served as the guidance for conducting this review. The quality assessment of the included studies was assessed by the Cochrane risk-of-bias tool. The meta-analysis was carried out utilizing Review Manager 5.4. Furthermore, sensitivity analysis and subgroup analysis were also performed. RESULTS: Nine studies recruited 1209 patients met our inclusion criteria. IS combined with other respiratory therapy techniques was observed to reduce the incidence of postoperative pulmonary complications, enhance pulmonary function, curtail the length of hospital stay, and lower the Borg score. Nevertheless, no improvements were found in the six-minute walk distance or quality of life score. CONCLUSIONS: Although IS demonstrates benefits as a component of comprehensive intervention measures for perioperative patients with lung cancer, it proves challenging to determine the precise impact of IS as a standalone component within the comprehensive intervention measures. Therefore, further researches are required to better understand the effectiveness of IS isolation and its interactions when integrated with additional respiratory therapies for these patients. CLINICAL TRIAL REGISTRATION: PROSPERO, https://www.crd.york.ac.uk/prospero/ , registry number: CRD42022321044.


Assuntos
Neoplasias Pulmonares , Humanos , Neoplasias Pulmonares/cirurgia , Qualidade de Vida , Motivação , Terapia Respiratória/métodos , Complicações Pós-Operatórias/epidemiologia , Espirometria/métodos
12.
J Speech Lang Hear Res ; 67(3): 729-739, 2024 Mar 11.
Artigo em Inglês | MEDLINE | ID: mdl-38324264

RESUMO

PURPOSE: Expiratory muscle strength training (EMST) is increasingly being used to treat voice, cough, and swallowing deficits in a wide range of conditions. However, a multitude of aero-resistive EMST models are commercially available, and the absence of side-by-side comparative data interferes with clinicians' ability to assess which model is best suited to a particular client's needs. The primary aim of this research was to test and compare the pressure and flow parameters of six currently available EMST models to help inform clinical decision making. METHOD: We identified and tested five devices of each of six different EMST models to generate benchmark data for minimum trigger pressures across settings. The reliability was tested within each device and between five devices of the same model across settings using coefficient of variation. RESULTS: All six models required higher pressures to initiate flow at the highest setting compared to the lowest setting, as expected. Detailed descriptive statistics for each model/setting combination include average flow-triggering pressure for each model/setting and the variability across trials within a device and across devices of the same model. From these, ranked order of the least to most stable EMST model was derived. CONCLUSIONS: Systematic testing of several commercially available expiratory resistance training devices yielded clinical benchmarks and reliability data to aid clinicians in selecting an appropriate therapy device and regimen for a client based on their available airflow and air pressure as well as reliability of the device. These findings allow clinicians to directly compare key parameters across EMST devices.


Assuntos
Expiração , Treinamento Resistido , Humanos , Reprodutibilidade dos Testes , Expiração/fisiologia , Terapia Respiratória , Força Muscular/fisiologia
13.
Eur J Pharm Biopharm ; 197: 114223, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38367760

RESUMO

The lung is an attractive target organ for inhalation of RNA therapeutics, such as small interfering RNA (siRNA). However, clinical translation of siRNA drugs for application in the lung is hampered by many extra- and intracellular barriers. We previously developed hybrid nanoparticles consisting of an siRNA-loaded nanosized hydrogel (nanogel) core coated with Curosurf®, a clinically used pulmonary surfactant. The surfactant shell was shown to markedly improve particle stability and promote intracellular siRNA delivery, both in vitro and in vivo. However, the full potential of siRNA nanocarriers is typically not reached as they are rapidly trafficked towards lysosomes for degradation and only a fraction of the internalized siRNA cargo is able to escape into the cytosol. We recently reported on the repurposing of widely applied cationic amphiphilic drugs (CADs) as siRNA delivery enhancers. Due to their physicochemical properties, CADs passively accumulate in the (endo)lysosomal compartment causing a transient permeabilization of the lysosomal membrane, which facilitates cytosolic drug delivery. In this work, we assessed a selection of cationic amphiphilic ß2-agonists (i.e., salbutamol, formoterol, salmeterol and indacaterol) for their ability to enhance siRNA delivery in a lung epithelial and macrophage cell line. These drugs are widely used in the clinic for their bronchodilating effect in obstructive lung disease. As opposed to the least hydrophobic drugs salbutamol and formoterol, the more hydrophobic long-acting ß2-agonist (LABA) salmeterol promoted siRNA delivery in both cell types for both uncoated and surfactant-coated nanogels, whereas indacaterol showed this effect solely in lung epithelial cells. Our results demonstrate the potential of both salmeterol and indacaterol to be repurposed as adjuvants for nanocarrier-mediated siRNA delivery to the lung, which could provide opportunities for drug combination therapy.


Assuntos
Indanos , Polietilenoglicóis , Polietilenoimina , Surfactantes Pulmonares , Quinolonas , Surfactantes Pulmonares/química , Nanogéis , RNA Interferente Pequeno , Terapia Respiratória , Xinafoato de Salmeterol , Albuterol , Fumarato de Formoterol , Adjuvantes Farmacêuticos , Administração por Inalação , Adjuvantes Imunológicos , Tensoativos
14.
J Control Release ; 367: 223-234, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38272396

RESUMO

Despite extensive research on corticosteroids for treating asthma, their short residence time in the lungs has limited their therapeutic effects in vivo. Nanoparticles have been widely investigated for inhaled drug delivery due to their potential benefits in prolonging drugs' residence time in the lungs. However, the retention of nanoparticles may be limited by mucus and ciliated epithelium clearance mechanisms in the airway. Herein, we anchored a neonatal-Fc-receptor-targeted peptide (FcBP) onto "mucus-penetrating" polyethylene glycol (PEG) nanoparticles (PEG-NP). Interestingly, the mucus-permeability of PEG-NP was not impaired by FcBP-functionalization. Moreover, FcBP modification enhanced cellular internalization and exocytosis via specific receptor-mediated processes, which subsequently ameliorated transepithelial transport and prolonged pulmonary retention. Importantly, after loading dexamethasone, FcBP-functionalization could effectively help nanoparticles cross the airway epithelial layer and be endocytosed by inflammatory cells, resulting in a marked decrease in inflammatory cytokines. Finally, FcBP modification significantly enhanced the therapeutic effect of dexamethasone-loaded nanoparticles in asthma mice. This study demonstrates that FcBP-functionalized PEG-NP can overcome multiple obstacles in the airway to prolong the pulmonary retention of drugs, providing a promising strategy for inhalation therapy.


Assuntos
Asma , Nanopartículas , Camundongos , Animais , Sistemas de Liberação de Medicamentos/métodos , Asma/tratamento farmacológico , Muco , Terapia Respiratória , Dexametasona/uso terapêutico , Dexametasona/farmacologia
15.
BMC Prim Care ; 25(1): 3, 2024 01 02.
Artigo em Inglês | MEDLINE | ID: mdl-38166661

RESUMO

BACKGROUND: Occupational, physical and respiratory therapists are relatively new to primary care settings, and thus their roles are still emerging. The COVID-19 pandemic was a time of abrupt changes in professional roles. Professional role adaptations are integral to the ability of health care teams to respond to day-to-day care delivery challenges, such as the current physician and nurse shortage, as well as disaster situations. This study explored the role adaptation of occupational, physical, and respiratory therapists in Canadian primary care settings throughout the first year of the COVID-19 pandemic, as well as barriers and facilitators to adaptation. METHODS: This longitudinal interpretative descriptive study purposively sampled primary care occupational, physical, and respiratory therapists from two Canadian provinces (Manitoba and Ontario). We asked participants to prepare at least 10 semi-structured audio-diary entries during a 12-week period (April - Oct 2020), followed by two semi-structured interviews (Dec 2020, Apr 2021). Questions focused on changes happening in their practice over time. Analysis was iterative, including developing a individual summaries and coding data using both inductive and pre-determined codes. We then entered an immersion/crystallization process to develop key themes related to role adaptation. RESULTS: We represent our findings with the metaphor of the game of Role Adaptation Snakes and Ladders (aka Chutes and Ladders). The pandemic was certainly not a game, but this metaphor represents the tension of being a pawn to circumstance while also being expected to take control of one's professional and personal life during a disaster. The object of the game is to move through three phases of role adaptation, from Disorienting, through Coping and Waiting, to Adapting. In the Adapting phase, the therapists creatively found ways to provide vital services for the pandemic response. The therapists were influenced both negatively and positively (snakes and ladders) by their personal circumstances, and professional meso and macro contexts. Each therapist moved across the board in a unique trajectory and timeline based on these contexts. CONCLUSIONS: Rehabilitation professionals, with adequate meso and macro system supports, can maximize their role on primary care teams by adapting their services to work to their full scope of practice.


Assuntos
COVID-19 , Humanos , COVID-19/epidemiologia , Pandemias , Ontário/epidemiologia , Terapia Respiratória , Atenção Primária à Saúde
16.
Int J Pharm ; 652: 123853, 2024 Mar 05.
Artigo em Inglês | MEDLINE | ID: mdl-38280500

RESUMO

Respiratory infection caused by multi-drug resistant (MDR) Pseudomonas aeruginosa is challenging to treat. In this study, we investigate the optimal dose of anti-pseudomonas phage PEV31 (103, 105, and 108 PFU/mL) combined with ciprofloxacin (ranging from 1/8× MIC to 8× MIC) to treat the MDR P. aeruginosa strain FADD1-PA001 using time-kill studies. We determined the impact of phage growth kinetics in the presence of ciprofloxacin through one-step growth analysis. Single treatments with either phage PEV31 or ciprofloxacin (except at 8× MIC) showed limited bactericidal efficiency, with bacterial regrowth observed at 48 h. The most effective treatments were PEV31 at multiplicity of infection (MOI) of 0.1 and 100 combined with ciprofloxacin at concentrations above 1× MIC, resulting in a >4 log10 reduction in bacterial counts. While the burst size of phage PEV31 was decreased with increasing ciprofloxacin concentration, robust antimicrobial effects were still maintained in the combination treatment. Aerosol samples collected from vibrating mesh nebulization of the combination formulation at phage MOI of 100 with 2× MIC effectively inhibited bacterial density. In summary, our combination treatments eradicated in vitro bacterial growth and sustained antimicrobial effects for 48 h. These results indicated the potential application of nebulization-based strategies for the combination treatment against MDR lung infections.


Assuntos
Bacteriófagos , Infecções por Pseudomonas , Humanos , Ciprofloxacina/farmacologia , Infecções por Pseudomonas/tratamento farmacológico , Infecções por Pseudomonas/microbiologia , Aerossóis e Gotículas Respiratórios , Antibacterianos/farmacologia , Antibacterianos/uso terapêutico , Terapia Respiratória , Pseudomonas aeruginosa , Testes de Sensibilidade Microbiana
17.
Respir Care ; 69(2): 275-279, 2024 Jan 24.
Artigo em Inglês | MEDLINE | ID: mdl-38267233
18.
Med Sci Sports Exerc ; 56(2): 266-276, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-37707508

RESUMO

PURPOSE: This study aimed to determine if time-efficient, high-resistance inspiratory muscle strength training (IMST), comprising 30 inhalation-resisted breaths per day, improves cardiorespiratory fitness, exercise tolerance, physical function, and/or regional body composition in healthy midlife and older adults. METHODS: We performed a double-blind, randomized, sham-controlled clinical trial (NCT03266510) testing 6 wk of IMST (30 breaths per day, 6 d·wk -1 , 55%-75% maximal inspiratory pressure) versus low-resistance sham training (15% maximal inspiratory pressure) in healthy men and women 50-79 yr old. Subjects performed a graded treadmill exercise test to exhaustion, physical performance battery (e.g., handgrip strength, leg press), and body composition testing (dual x-ray absorptiometry) at baseline and after 6 wk of training. RESULTS: Thirty-five participants (17 women, 18 men) completed high-resistance IMST ( n = 17) or sham training ( n = 18). Cardiorespiratory fitness (V̇O 2peak ) was unchanged, but exercise tolerance, measured as treadmill exercise time during a graded exercise treadmill test, increased with IMST (baseline, 539 ± 42 s; end intervention, 606 ± 42 s; P = 0.01) but not sham training (baseline, 562 ± 39 s; end intervention, 553 ± 38 s; P = 0.69). IMST increased peak RER (baseline, 1.09 ± 0.02; end intervention, 1.13 ± 0.02; P = 0.012), peak ventilatory efficiency (baseline, 25.2 ± 0.8; end intervention, 24.6 ± 0.8; P = 0.036), and improved submaximal exercise economy (baseline, 23.5 ± 1.1 mL·kg -1 ⋅min -1 ; end intervention, 22.1 ± 1.1 mL·kg -1 ⋅min -1 ; P < 0.001); none of these factors were altered by sham training (all P > 0.05). Changes in plasma acylcarnitines (targeted metabolomics analysis) were consistently positively correlated with changes in exercise tolerance after IMST but not sham training. IMST was associated with regional increases in thorax lean mass (+4.4%, P = 0.06) and reductions in trunk fat mass (-4.8%, P = 0.04); however, peripheral muscle strength, muscle power, dexterity, and mobility were unchanged. CONCLUSIONS: These data suggest that high-resistance IMST is an effective, time-efficient lifestyle intervention for improving exercise tolerance in healthy midlife and older adults.


Assuntos
Tolerância ao Exercício , Treinamento Resistido , Idoso , Feminino , Humanos , Masculino , Força da Mão , Força Muscular/fisiologia , Músculos , Terapia Respiratória , Método Duplo-Cego
19.
Rev Mal Respir ; 41(1): 43-50, 2024 Jan.
Artigo em Francês | MEDLINE | ID: mdl-38057240

RESUMO

In adults and teenagers, airway clearance physiotherapy techniques (ACPT) are various and numerous. However, they for still awaiting scientific validation. Among ACPTs, Slow Expiration with the Glottis Opened in the Lateral Posture (ELTGOL), Autogenic Drainage (DA), and Active Cycling Breathing Technique (ACBT) present a Grade B level of evidence with weak recommendations. Even though these maneuvers are widely applied, precise description of chest physiotherapy (CP) is largely absent from the scientific literature; it is difficult to standardize its implementation and reproduce the results; scientific validation and faithful execution of the techniques are consequently problematic. In this paper, the authors aim to depict each of the three CP techniques as precisely as possible; with this in mind, graphic modeling of the different respiratory exercises is presented in such a way that they can be easily learned, applied and reproduced by physiotherapists.


Assuntos
Fibrose Cística , Drenagem Postural , Adulto , Humanos , Adolescente , Drenagem Postural/métodos , Terapia Respiratória/métodos , Exercícios Respiratórios , Modalidades de Fisioterapia
20.
Am J Phys Med Rehabil ; 103(3): 233-237, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-37881957

RESUMO

OBJECTIVES: The aims of the study are to present noninvasive respiratory management outcomes using continuous noninvasive ventilatory support and mechanical in-exsufflation from infancy for spinal muscular atrophy type 1 and to consider bearing on new medical therapies. DESIGN: Noninvasive ventilatory support was begun for consecutively referred symptomatic infants with spinal muscular atrophy type 1 from 1 to 10 mos of age. Intercurrent episodes of respiratory failure were managed by intubation then extubation to continuous noninvasive ventilatory support and mechanical in-exsufflation despite failing ventilator weaning and extubation attempts. Intubations, tracheotomies, and survival were monitored. RESULTS: Of 153 patients with spinal muscular atrophy 1 consecutively referred since 1995, 37 became continuous noninvasive ventilatory support dependent, almost half before 10 yrs of age. Of the 37, 18 required continuous noninvasive ventilatory support for a mean 18.6 ± 3.3 yrs to a mean 25.3 (range, 18-30) yrs of age, dependent from as young as 4 mos of age with 0 to 40 ml of vital capacity. One of the 18 died from COVID-19 acute respiratory distress syndrome at age 24 after 23 yrs of continuous noninvasive ventilatory support. Extubation success rate of 85% per attempt (150/176) resulted in only one undergoing tracheotomy. CONCLUSIONS: Medical treatments begun during the first 6 wks of age convert spinal muscular atrophy 1 into spinal muscular atrophy 2 or 3 but cough flows remain inadequate to avoid many pneumonias that, once resolved by a treatment paradigm of extubation to continuous noninvasive ventilatory support and mechanical in-exsufflation, eliminates need to resort to tracheotomies.


Assuntos
Atrofia Muscular Espinal , Atrofias Musculares Espinais da Infância , Lactente , Humanos , Adulto Jovem , Adulto , Atrofias Musculares Espinais da Infância/terapia , Respiração Artificial/métodos , Desmame do Respirador , Terapia Respiratória/métodos , Atrofia Muscular Espinal/terapia
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