Introduction: Pulmonary exacerbations are common events in children with cystic fibrosis (CF) and are usually treated with oral antibiotics on an outpatient basis. Even these mild clinical events are clinically meaningful and contribute to the progression of lung disease.
Areas covered: This review discusses the challenges in diagnosing pulmonary exacerbations in children in the absence of a standardized definition. It describes an approach to the management of these events and emphasizes knowledge gaps and areas of future research directions. Information to write this narrative review was collected from 1) a PubMed search [keywords: exacerbation, children, cystic fibrosis] that was not limited by date 2) a hand search of references of retrieved literature 3) personal expertise of the literature and the management of cystic fibrosis.
Expert opinion: Pulmonary exacerbations require prompt diagnosis and management to preserve lung function. More work is needed to understand the impact of CFTR modulators on the frequency and severity of these events and how they influence approaches to management. In a new era of CF care, there is a need to incorporate sensitive outcome measures into clinical care to inform treatment decisions and track treatment response.
Background: Central airway obstruction (CAO) are common abnormality that usually needing interventional bronchoscopy, and sometimes multiple rounds of treatment. However, there were few studies explore the safety of it.
Research design & methods: The records of patients who underwent interventional bronchoscopy because of CAO at Respiratory department between 1 January 2010, and 31 December 2020 were revised. The patients' clinical characteristics, information about bronchoscopy and incidence of complications were collected and analyzed.
Results: There were totally 1,482 bronchoscopy procedures conducted in the 733 CAO patients. And the incidence of major complications in the retreatment group was significantly lower than that in the first treatment group ((4.77% vs. 1.87%, χ2 = 9.78, df = 1, p < 0.01), so did the incidence of severe bleeding (2.46% vs. 0.40%, χ2 = 11.20, df = 1, p < 0.01). However, there was some variability between the two groups in age and anesthesia type. A short interval time, more treatment times, and general anesthesia were related to a lower incidence of hemorrhage. For patients who were previously bleeding, the incidence of hemorrhage was significantly higher than the incidence in the non-bleeding patients (42.93% vs. 16.33%, respectively; χ2 = 57.54, df = 1, p < 0.01).
Conclusion: For the patients with CAO, repeated interventional bronchoscopy treatment was safe, and it should be treated with discretion when retreat the patients once bleeding during previous therapeutic bronchoscopy.
Introduction: Acute pulmonary embolism (PE) is a disease with a broad spectrum of clinical presentations. While some patients can be treated at home or may even be left untreated, other patients require an aggressive approach with reperfusion treatment.
Areas covered: (1) Advanced reperfusion treatment in hemodynamically stable acute PE patients considered to be at high risk of decompensation and death, (2) the treatment of subsegmental pulmonary embolism, (3) outpatient treatment for hemodynamically stable PE patients with signs of right ventricle (RV) dysfunction, and (4) the optimal approach to identify and treatpost-PE syndrome.
Expert opinion: Outside clinical trials, hemodynamically stable acute PE patients should not be treated with primary reperfusion therapy. Thrombolysis and/or catheter-directed therapy are only to be considered as rescue treatment. Subsegmental PE can be left untreated in selected low-risk patients, after proximal deep vein thrombosis has been ruled out. Patients with an sPESI or Hestia score of 0 criteria can be treated at home, independent of the presence of RV overload. Finally, health-care providers should be aware of post-PE syndrome and diagnose chronic thromboembolic pulmonary disease (CTEPD) as early as possible. Persistently symptomatic patients without CTEPD benefit from exercise training and cardiopulmonary rehabilitation.
Introduction: Lung cancer remains the deadliest form of cancer in the world. Screening through low-dose CT scans has shown improved detection of pulmonary nodules; however, with the introduction of robotic bronchoscopy, accessing and biopsying peripheral pulmonary nodules from the airway has expanded. Improved diagnostic yield through enhanced navigation has made robotic bronchoscopy an ideal diagnostic technology for many proceduralists. Studies have demonstrated that robotic bronchoscopes can reach further with improved maneuverability into the distal airways compared to conventional bronchoscopes.
Areas covered: This review paper highlights the literature on the technological advancements associated with robotic bronchoscopy and the future directions the field of interventional pulmonary may utilize this modality for in the treatment of lung cancer. Referenced articles were included at the discretion of the authors after a database search of the particular technology discussed.
Expert opinion: As the localization of target lesions continues to improve, robotic platforms that provide reach, stability, and accuracy paves the way for future research in endoluminal treatment for lung cancer. Future studies with intratumoral injection of chemotherapy and immunotherapy and ablation modalities are likely to come in the coming years.
Introduction: Alpha1 antitrypsin deficiency (AATD), a common hereditary disorder affecting mainly lungs, liver and skin has been the focus of some of the most exciting therapeutic approaches in medicine in the past 5 years. In this review, we discuss the therapies presently available for the different manifestations of AATD and new therapies in the pipeline.
Areas covered: We review therapeutic options for the individual lung, liver and skin manifestations of AATD along with approaches which aim to treat all three. Along with this renewed interest in treating AATD come challenges. How is AAT best delivered to the lung? What is the desired level of AAT in the circulation and lungs which therapeutics should aim to provide? Will treating the liver disease increase the potential for lung disease? Are there treatments to target the underlying genetic defect with the potential to prevent all aspects of AATDrelated disease?
Expert opinion: With a relatively small population able to participate in clinical studies, increased awareness and diagnosis of AATD is urgently needed. Better, more sensitive clinical parameters will assist in the generation of acceptable and robust evidence of therapeutic effect for current and emerging treatments.
Background: Chronic obstructive pulmonary disease (COPD) patients experience a progressive limitation of their functionality accompanying their clinical evolution. Concretely, severe COPD patients usually require the figure of a caregiver. Caregiver burden has yet to be explored in other similar chronic diseases. The objective is to propose a cutoff point in different functional impairment aspects, to predict the presence of caregiver burden.
Methods: Severe COPD patients were divided into two groups according to the caregiver burden, measured with the Zarit Burden Interview (ZBI). The patients were assessed with the London Chest Activity of Daily Living (LCADL) scale, the Functional Independence Measure (FIM), and the World Health Organization Disability Assessment Schedule 2.0 (WHODAS 2.0).
Results: 70 COPD patients and their caregivers were included in this cross-sectional study. The ROC curve indicated a cutoff point of 19 in the LCADL scale (AUC = 0.722). Dependence in daily life activities had a cutoff point of 123 in the FIM (AUC = 0.776). Social participation in activities of daily living had a cutoff point of 37 in the WHODAS 2.0 (AUC = 0.739).
Conclusion: Dyspnea related to functional status, dependence in daily life activities, and social participation in activities of daily living of severe COPD patients can predict caretaker burden.
Introduction: Patients with neurocognitive impairment (NI) have multiple medical needs, with respiratory problems leading to an important reduction in quality of life and life expectancy. We aimed to explain that the origin of chronic respiratory symptoms in patients with NI is multifactorial.
Areas covered: In people with NI there is a high prevalence of swallowing dysfunction and hypersalivation inducing aspiration; cough efficacy is decreased resulting in chronic lung infection; sleep-disordered breathing is frequent and muscle mass is abnormal due to malnutrition. Technical investigations are not always specific and sensitive enough to better diagnose the causes of the respiratory symptoms; moreover, they can sometimes be difficult to perform in this vulnerable patient population. We provide a clinical pathway to adopt to identify, prevent, and treat respiratory complications in children and young adults with NI. A holistic approach in discussion with all care providers and the parents is highly recommended.
Expert opinion: The care for people with NI and chronic respiratory problems is challenging. The interplay between several causative factors may be difficult to entangle. Well-performed clinical research in this field is largely missing and should be encouraged. Only then, evidence-based clinical care will become possible for this vulnerable patient group.
Introduction: Cell therapy has emerged as an alternative option for chronic lung diseases with the highest rates of morbidity and mortality rates worldwide.
Areas covered: This review addresses the definition of mesenchymal stromal cells (MSCs), their properties, mechanisms of action, as well as preclinical and clinical studies that have used cell therapy in chronic lung diseases such as asthma, chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, pulmonary arterial hypertension, and silicosis. Ongoing clinical trials are also presented.
Expert opinion: Experimental evidence has shown that MSCs have immunomodulatory and regenerative properties that could rescue impaired lung function and histoarchitecture. Their beneficial effects have been mainly associated with their ability to communicate with target cells through the secretion of soluble mediators and extracellular vesicles or even through transfer of organelles (e.g. mitochondria). MSC-derived conditioned medium, extracellular vesicles and mitochondria induce beneficial effects in selected scenarios. The initial results in clinical trials were modest compared with the experimental results, therefore researchers were encouraged to move from bedside back to bench to develop new strategies able to potentiate the effects of MSCs.
Objectives: Treatment guidelines have recommended tiotropium bromide inhalation (TBI), a long-acting muscarinic antagonist, for chronic obstructive pulmonary disease (COPD); however, its efficacy in symptomatic Chinese patients with COPD remains uninvestigated.
Methods: This multicenter, prospective, observational study enrolled patients with COPD assessment test (CAT) scores exceeding 10 points from 19 hospitals spread across China. All patients received TBI and underwent follow-up for 3 months. The demographic and clinical information were assessed.
Results: The final analysis included 378 patients. The forced expiratory volume in 1 s (FEV1) and FEV1/forced vital capacity (FVC) of all participants improved markedly after 3 months of treatment (FEV1: mean 1.33 L versus 1.61 L, P < 0.001; FEV1/FVC: mean 0.53 versus 0.62, P < 0.001). The mean CAT scores decreased from 26.56 to 16.28 (P < 0.001). Patients classified into group D based on the Global Initiative for COPD guidelines showed greater improvement in FEV1 and FEV1/FVC than that in patients in group B. The proportion of patients with acute exacerbations also declined from 28.6% in the first month to 4.2% in the third month.
Conclusion: TBI for 3 months could effectively and safely attenuate symptoms and airflow obstruction in symptomatic Chinese patients with COPD.
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