scholarly journals Bronchial hygiene technique in patients with cystic fibrosis

2021 ◽  
Author(s):  
Thainá Gomes Vieira Carvalho ◽  
Joyce Mothé de Souza ◽  
Elisa Haddad Pessanha Rangel ◽  
Caio Gomes Muniz ◽  
Julia Maria Maia de Azevedo ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Respiratory Infections ◽  
Walk Test ◽  
Review Of The Literature ◽  
Peripheral Airways ◽  
Randomized Controlled ◽  
Recurrent Respiratory Infections ◽  
Consequent Improvement ◽  
Tracheal Aspiration

Cystic Fibrosis is characterized by excess pulmonary secretions that cause recurrent respiratory infections, with consequent deterioration of gas exchange. Bronchial hygiene techniques aim to mobilize secretions from the peripheral airways so that they can be eliminated by coughing or tracheal aspiration. To identify the effects of different bronchial hygiene techniques on improving lung function in patients with Cystic Fibrosis. Through a systematic review of the literature, randomized controlled trials (RCTs) published between 2007 and 2021 were selected, according to the highest score in the PEDro score. The search involved the PEDro and PubMed databases, using the following keywords: bronchial hygiene. Six ECR`s were included. One study performedthe techniques during anesthesia and observed increased resistance and reduced compliance. Regarding FEV1, 3 RCTs with hospitalized patients showed improvement in lung function, regardless of the technique used. In outpatients, there was no improvement. Regarding secretion weight, the cough machine produced more secretion than autogenous drainage, as well as a drop in saturation after the 2-min walk test, and increased FEV1. Bronchial hygiene techniques in patients with cystic fibrosis have been shown to be effective in removing mucus, with consequent improvement in lung function and aerobic fitness.

scholarly journals Pregnancy in Cystic Fibrosis: Review of the Literature and Expert Recommendations

2020 ◽  
Author(s):  
Raksha Jain ◽  
Traci Kazmerski ◽  
Lisa Zuckerwise ◽  
Natalie West ◽  
Kristina Montemayor ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Family Planning ◽  
High Risk ◽  
Lung Function ◽  
Current Literature ◽  
Review Of The Literature ◽  
The Past ◽  
Care Family ◽  
Review Current ◽  
Expert Recommendations

Cystic fibrosis (CF) was historically a disease largely afflicting children. Due to therapeutic advancement, there are now more adults with CF than children. In the past decade, medications became available that treat the underlying cause of CF and are dramatically improving lung function as well as quality and quantity of life for people with CF. As a result, more women with CF are having babies. We gathered a panel of experts in CF care, family planning, high risk obstetrics, nutrition, genetics and women with CF to review current literature on pregnancies and to provide care recommendations for this unique population.

scholarly journals Treatment of Pulmonary Disease of Cystic Fibrosis: A Comprehensive Review

Antibiotics ◽  
2021 ◽  
Vol 10 (5) ◽  
pp. 486
Author(s):  
Rosa María Girón Moreno ◽  
Marta García-Clemente ◽  
Layla Diab-Cáceres ◽  
Adrián Martínez-Vergara ◽  
Miguel Ángel Martínez-García ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Genetic Disease ◽  
Drug Response ◽  
Respiratory Infections ◽  
Regulatory Protein ◽  
Anion Channel ◽  
Transmembrane Conductance ◽  
Recurrent Respiratory Infections ◽  
Altered Protein ◽  
Genetic Complexity

Cystic fibrosis (CF) is a genetic disease that causes absence or dysfunction of a protein named transmembrane conductance regulatory protein (CFTR) that works as an anion channel. As a result, the secretions of the organs where CFTR is expressed are very viscous, so their functionality is altered. The main cause of morbidity is due to the involvement of the respiratory system as a result of recurrent respiratory infections by different pathogens. In recent decades, survival has been increasing, rising by around age 50. This is due to the monitoring of patients in multidisciplinary units, early diagnosis with neonatal screening, and advances in treatments. In this chapter, we will approach the different therapies used in CF for the treatment of symptoms, obstruction, inflammation, and infection. Moreover, we will discuss specific and personalized treatments to correct the defective gene and repair the altered protein CFTR. The obstacle for personalized CF treatment is to predict the drug response of patients due to genetic complexity and heterogeneity of uncommon mutations.

scholarly journals A multi-centre, randomized, controlled trial on coaching and telemonitoring in patients with cystic fibrosis: conneCT CF

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Stephanie Thee ◽  
Mirjam Stahl ◽  
Rainald Fischer ◽  
Sivagurunathan Sutharsan ◽  
Manfred Ballmann ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Randomized Controlled Trial ◽  
Lung Function ◽  
Hospital Admissions ◽  
Controlled Trial ◽  
Forced Expiratory Volume ◽  
Inhalation Therapy ◽  
Secondary Outcome ◽  
Randomized Controlled ◽  
Behavior Change Interventions

Abstract Background The extend of lung disease remains the most important prognostic factor for survival in patients with cystic fibrosis (CF), and lack of adherence is the main reason for treatment failure. Early detection of deterioration in lung function and optimising adherence are therefore crucial in CF care. We implement a randomized controlled trial to evaluate efficacy of telemonitoring of adherence, lung function, and health condition in combination with behavior change interventions using innovative digital technologies. Methods This is a multi-centre, randomized, controlled, non-blinded trial aiming to include 402 patients ≥ 12 years-of-age with CF. A standard-of-care arm is compared to an arm receiving objective, continuous monitoring of adherence to inhalation therapies, weekly home spirometry using electronic devices with data transmission to patients and caring physicians combined with video-conferencing, a self-management app and professional telephone coaching. The duration of the intervention phase is 18 months. The primary endpoint is time to the first protocol-defined pulmonary exacerbation. Secondary outcome measures include number of and time between pulmonary exacerbations, adherence to inhalation therapy, changes in forced expiratory volume in 1 s from baseline, number of hospital admissions, and changes in health-related quality of life. CF-associated medical treatment and care, and health care related costs will be assessed by explorative analysis in both arms. Discussion This study offers the opportunity to evaluate the effect of adherence interventions using telemedicine capable devices on adherence and lung health, possibly paving the way for implementation of telemedicine in routine care for patients with CF. Trial registration: This study has been registered with the German Clinical Trials Register (Identifier: DRKS00024642, date of registration 01 Mar 2021, URL: https://www.drks.de/drks_web/navigate.do?navigationId=trial.HTML&TRIAL_ID=DRKS00024642).

scholarly journals Brazilian consensus on non-cystic fibrosis bronchiectasis

2019 ◽  
Vol 45 (4) ◽  
Author(s):  
Mônica Corso Pereira ◽  
Rodrigo Abensur Athanazio ◽  
Paulo de Tarso Roth Dalcin ◽  
Mara Rúbia Fernandes de Figueiredo ◽  
Mauro Gomes ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Respiratory Infections ◽  
Scientific Evidence ◽  
Critical Assessment ◽  
Review Of The Literature ◽  
Insufficient Evidence ◽  
Consensus Document ◽  
Airway Symptoms ◽  
The Subject ◽  
Expert Consensus Document

ABSTRACT Bronchiectasis is a condition that has been increasingly diagnosed by chest HRCT. In the literature, bronchiectasis is divided into bronchiectasis secondary to cystic fibrosis and bronchiectasis not associated with cystic fibrosis, which is termed non-cystic fibrosis bronchiectasis. Many causes can lead to the development of bronchiectasis, and patients usually have chronic airway symptoms, recurrent infections, and CT abnormalities consistent with the condition. The first international guideline on the diagnosis and treatment of non-cystic fibrosis bronchiectasis was published in 2010. In Brazil, this is the first review document aimed at systematizing the knowledge that has been accumulated on the subject to date. Because there is insufficient evidence on which to base recommendations for various treatment topics, here the decision was made to prepare an expert consensus document. The Brazilian Thoracic Association Committee on Respiratory Infections summoned 10 pulmonologists with expertise in bronchiectasis in Brazil to conduct a critical assessment of the available scientific evidence and international guidelines, as well as to identify aspects that are relevant to the understanding of the heterogeneity of bronchiectasis and to its diagnostic and therapeutic management. Five broad topics were established (pathophysiology, diagnosis, monitoring of stable patients, treatment of stable patients, and management of exacerbations). After this subdivision, the topics were distributed among the authors, who conducted a nonsystematic review of the literature, giving priority to major publications in the specific areas, including original articles, review articles, and systematic reviews. The authors reviewed and commented on all topics, producing a single final document that was approved by consensus.

scholarly journals Frequency of Cystic Fibrosis in Children with Recurrent Respiratory Infections by Sweat Chloride Testing: A Hospital-Based Cross-sectional Study

2020 ◽  
Vol 24 (4) ◽  
pp. 302-305
Author(s):  
Nahdia Zaman ◽  
Anam Zafar ◽  
Maryam Amjad ◽  
Shahzadi Sumbal Ghazi ◽  
Uzma Abid ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Respiratory Infections ◽  
Cross Sectional Study ◽  
P Value ◽  
Sectional Study ◽  
Cross Sectional ◽  
Sweat Chloride ◽  
Recurrent Respiratory Infections ◽  
Ethical Approval ◽  
Tract Infections

Objective: This study was designed to find out the frequency of cystic fibrosis in children with recurrent respiratory infections by performing a sweat chloride test.Material and Methods: This cross-sectional study was carried out from 1st September 2015 to 28th February 2016 in the department of Pediatric Medicine at the Children’s Hospital and the Institute of Child Health, Lahore after ethical approval. A total of 300 cases of recurrent respiratory tract infections were included. The sweat chloride test was done by using pilocarpine-induced iontophoresis and measuring chloride levels on the forearm or thigh by sweat analyzer at the time of induction. Cystic fibrosis was labeled if sweat chloride level ranges above 60 mEq/L. Data was collected on Performa and SPSS version 23 was used to analyze the data. Results: Mean age of patients was 6.24 ± 2.7 years with 52.7% males and 47.3% females. Most patients (86.3%) have more than 5 episodes of respiratory infection in a year. Cystic fibrosis was diagnosed in 19 (6.3%) patients. There was no relationship between the frequency of respiratory infections in a year and cystic fibrosis (p-value 0.78).Conclusion: This hospital-based study showed quite a high incidence of cystic fibrosis in our population. So facilities including neonatal screening along with sweat chloride testing and genetic analysis should be increased.

scholarly journals Diagnostic Barriers in Children with Immunodeficiencies in Central Asia: A Case-Based Discussion

2021 ◽  
Vol 13 (3) ◽  
pp. 483-489
Author(s):  
Zhanna Dauyey ◽  
Dimitri Poddighe
Keyword(s):  
Cystic Fibrosis ◽  
Central Asia ◽  
Low Socioeconomic Status ◽  
English Language ◽  
Respiratory Infections ◽  
Clinical History ◽  
Medical Problem ◽  
Medical Attention ◽  
Clinical Issue ◽  
Recurrent Respiratory Infections

Common variable immunodeficiency (CVID) is a primary immune deficit (PID) mainly characterized by hypogammaglobulinemia. In addition to increased susceptibility to infections and several immune-mediated manifestations, patients with CVID frequently develop bronchiectasis because of recurrent respiratory infections. This occurrence could be more likely if the diagnosis of CVID is delayed, as it often happens in less resourced clinical settings. A 15-year-old female patient was admitted to a tertiary hospital in Kazakhstan for consultation regarding a previous and established diagnosis of bronchiectasis. The clinical history was characterized by recurrent respiratory infections for several years, in addition to the development of a mixed restrictive-obstructive respiratory syndrome. Therefore, she underwent chest computerized tomography, which confirmed the presence of multiple and bilateral bronchiectasis. The clinical discussion on this patient highlighted that serum immunoglobulins were never measured previously and, thus, their assessment was strongly recommended. Based on that, a diagnosis of CVID was finally achieved, and the patient started the appropriate immunoglobulin replacement therapy. To our knowledge, this report is the first English-language publication on CVID and bronchiectasis from Central Asia. Bronchiectasis is currently an important medical problem in developing countries and populations with low socioeconomic status, where the diagnosis of the underlying cystic fibrosis and non-cystic fibrosis comorbidities can be delayed and more difficult than in countries with more accessible health care systems and facilities. This case report emphasized this important clinical issue in Central Asia and should raise the medical attention and awareness of this health problem, in order to improve the diagnostic timing and rate.

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