FASEB J. 2021 Nov;35(11):e21987. doi: 10.1096/fj.202101315R.

ABSTRACT

Autosomal dominant polycystic kidney disease (ADPKD) is associated with the formation of renal cysts. We have devised a therapeutic approach, based on reversing the cyst phenotype from secretion to absorption by using VX-809, a modulator of the cystic fibrosis transmembrane regulator trafficking and processing. Our goal is to test VX-809 in RC/RC mice bearing the R3277C human mutation to demonstrate its therapeutic potential. We found that by 5 months of age, RC/RC mice had large cysts and impaired renal function, but when treated with VX-809 between the ages of 3 and 5 months, or 6 and 8 months, the cyst area was reduced in both groups, suggesting that VX-809 had shrunk previously existing cysts. After 2 months of treatment, the cyst size was lower than that of untreated animals of the same age. Our co-localization studies confirmed that cystic fibrosis transmembrane conductance regulator (CFTR) is found predominately at the apical membrane in the untreated animals of each age group, consistent with its role in Cl- secretion; after VX-809 treatment, the basolateral membrane co-localization of CFTR increased ~4-fold, accompanied by a decrease of ~2-3-fold in its apical co-localization, indicating that VX-809 alters the phenotype to favor fluid absorption. Sodium/hydrogen exchanger and epithelial sodium channel, found in normal kidneys at the apical membrane, were almost absent from the cysts. VX-809 restored both levels toward normal. HSP27 is highly expressed in RC/RC mice and lowered toward normal by VX-809. Our demonstration of cyst reduction, improved renal function, and generation of an absorptive phenotype all strongly support the therapeutic potential of VX-809 as a treatment for ADPKD. We show here in an animal model of slowly progressing cyst formation typical of human ADPKD that VX-809 reduces the growth of already established cysts. The magnitude of the effect in the RC/RC mouse model when compared to previous experiments using the same mouse model to evaluate tolvaptan indicates that CFTR modulators warrant further development as a treatment for ADPKD.

PMID:34662459 | DOI:10.1096/fj.202101315R

Biochem Genet. 2021 Oct 18. doi: 10.1007/s10528-021-10141-z. Online ahead of print.

ABSTRACT

The Iranian gene pool is seen as an important human genetic resource for investigating the region connecting Mesopotamia and the Iranian plateau. The main objective of this study was to explore gene flow in nine Iranian ethnic/subpopulation groups (402 samples) by examining mtDNA HVS2 sequence variations. This then allowed us to detect mtDNA HVS2 sequence mutations in two independent thalassemia and cystic fibrosis patient sample groups. The patient groups did not explicitly belong to any of the aforementioned nine subpopulations. Across all subpopulations, the haplogroups B4a1c3a, H2a2a1, N10b, H2a2a2, and J1 were seen to be predominant. High haplogroup diversities along with admixture of the exotic groups were observed in this study. The Arab subpopulation was shown to be independent from the others. It was revealed that there is a far distant relationship between Arab and Azeri groups. The thalassemia patient group, represented an almost random sample of most Iranian ethnic groups, and revealed few significant differences (P < 0.05) in their HVS2 sequence. It turned out that the IVS II-I (G → A) mutation in the thalassemia β-globin gene was highly significant. Since the thalassemia patients in the present study represent many unique haplotypes, we can begin to comprehend the importance of mtDNA with this disease and the necessity for more studies in this context.

PMID:34661819 | DOI:10.1007/s10528-021-10141-z

Front Cell Infect Microbiol. 2021 Sep 30;11:745851. doi: 10.3389/fcimb.2021.745851. eCollection 2021.

ABSTRACT

The opportunistic human pathogen Pseudomonas aeruginosa is responsible for a variety of acute infections and is a major cause of mortality in chronically infected patients with cystic fibrosis (CF). Considering the intrinsic and acquired resistance of P. aeruginosa to currently used antibiotics, new therapeutic strategies against this pathogen are urgently needed. Whereas virulence factors of P. aeruginosa are well characterized, the interplay between P. aeruginosa and the innate immune response during infection remains unclear. Zebrafish embryo is now firmly established as a potent vertebrate model for the study of infectious human diseases, due to strong similarities of its innate immune system with that of humans and the unprecedented possibilities of non-invasive real-time imaging. This model has been successfully developed to investigate the contribution of bacterial and host factors involved in P. aeruginosa pathogenesis, as well as rapidly assess the efficacy of anti-Pseudomonas molecules. Importantly, zebrafish embryo appears as the state-of-the-art model to address in vivo the contribution of innate immunity in the outcome of P. aeruginosa infection. Of interest, is the finding that the zebrafish encodes a CFTR channel closely related to human CFTR, which allowed to develop a model to address P. aeruginosa pathogenesis, innate immune response, and treatment evaluation in a CF context.

PMID:34660345 | PMC:PMC8515127 | DOI:10.3389/fcimb.2021.745851

Front Cell Infect Microbiol. 2021 Sep 29;11:729476. doi: 10.3389/fcimb.2021.729476. eCollection 2021.

ABSTRACT

Resistance of the human pathogenic fungus Aspergillus fumigatus to antifungal agents is on the rise. However, links between patient infections, their potential acquisition from local environmental sources, and links to global diversity remain cryptic. Here, we used genotyping analyses using nine microsatellites in A. fumigatus, in order to study patterns of diversity in France. In this study, we genotyped 225 local A. fumigatus isolates, 112 azole susceptible and 113 azole resistant, collected from the Bourgogne-Franche-Comté region (Eastern France) and sampled from both clinical (n = 34) and environmental (n = 191) sources. Azole-resistant clinical isolates (n = 29) were recovered mainly from cystic fibrosis patients and environmental isolates (n = 84) from market gardens and sawmills. In common with previous studies, the TR34/L98H allele predominated and comprised 80% of resistant isolates. The genotypes obtained for these local TR34/L98H isolates were integrated into a broader analysis including all genotypes for which data are available worldwide. We found that dominant local TR34/L98H genotypes were isolated in different sample types at different dates (different patients and types of environments) with hospital air and patient's isolates linked. Therefore, we are not able to rule out the possibility of some nosocomial transmission. We also found genotypes in these same environments to be highly diverse, emphasizing the highly mixed nature of A. fumigatus populations. Identical clonal genotypes were found to occur both in the French Eastern region and in the rest of the world (notably Australia), while others have not yet been observed and could be specific to our region. Our study demonstrates the need to integrate patient, healthcare, and environmental sampling with global databases in order to contextualize the local-scale epidemiology of antifungal resistant aspergillosis.

PMID:34660341 | PMC:PMC8512841 | DOI:10.3389/fcimb.2021.729476

Front Cell Infect Microbiol. 2021 Sep 30;11:701362. doi: 10.3389/fcimb.2021.701362. eCollection 2021.

ABSTRACT

The genus Burkholderia contains over 80 different Gram-negative species including both plant and human pathogens, the latter of which can be classified into one of two groups: the Burkholderia pseudomallei complex (Bpc) or the Burkholderia cepacia complex (Bcc). Bpc pathogens Burkholderia pseudomallei and Burkholderia mallei are highly virulent, and both have considerable potential for use as Tier 1 bioterrorism agents; thus there is great interest in the development of novel vaccines and therapeutics for the prevention and treatment of these infections. While Bcc pathogens Burkholderia cenocepacia, Burkholderia multivorans, and Burkholderia cepacia are not considered bioterror threats, the incredible impact these infections have on the cystic fibrosis community inspires a similar demand for vaccines and therapeutics for the prevention and treatment of these infections as well. Understanding how these pathogens interact with and evade the host immune system will help uncover novel therapeutic targets within these organisms. Given the important role of the complement system in the clearance of bacterial pathogens, this arm of the immune response must be efficiently evaded for successful infection to occur. In this review, we will introduce the Burkholderia species to be discussed, followed by a summary of the complement system and known mechanisms by which pathogens interact with this critical system to evade clearance within the host. We will conclude with a review of literature relating to the interactions between the herein discussed Burkholderia species and the host complement system, with the goal of highlighting areas in this field that warrant further investigation.

PMID:34660335 | PMC:PMC8515183 | DOI:10.3389/fcimb.2021.701362

World Allergy Organ J. 2021 Sep 28;14(9):100578. doi: 10.1016/j.waojou.2021.100578. eCollection 2021 Sep.

ABSTRACT

The concept of treatment of an allergy with the offending allergen was introduced more than a century ago. Allergen immunotherapy (AIT) is the only disease modifying treatment of allergic diseases caused by inhalational allergens and insect venoms. Despite this, only few AIT products have reached licensure in the US or an official marketing authorization status in European countries. Moreover, most of these AIT products are provided on an individual patient basis as named patient products (NPP) in Europe, while individualized preparations of (mixed) allergenic extract vials for subcutaneous administration (compounding) is common practice in the US. AIT products are generally considered safe and well tolerated, but the major practical clinical development challenge is to define the optimal dose and prove the efficacy and safety of these products using state-of-the art Phase II and pivotal Phase III studies. In planning Phase II-III AIT studies, a thorough understanding of the study challenges is essential (e.g. variability and non-validated status of subjective primary endpoints, limitations of pollen season definitions) and dogmas of these products (e.g., for sublingual immunotherapy (SLIT) trials double-blinding conditions cannot be maintained, resulting in stronger placebo responses in the active treatment group and inflated treatment effects in Phase III). There is future promise for more objective biomarker endpoints (e.g. basophil activation (CD63 and CD203c), subsets of regulatory dendritic, T and B cells, IL-10-producing group 2 innate lymphoid cells; alone or in combination) to overcome several of these dogmas and challenges; innovation in AIT clinical trials can only progress with integral biomarker research to complement the traditional endpoints in Phase II-III clinical development. The aim of this paper is to provide an overview of these dogmas, challenges and recommendations based on published data, to facilitate the design of Phase III studies and improve the evidence basis of safe and effective AIT products.

PMID:34659627 | PMC:PMC8487954 | DOI:10.1016/j.waojou.2021.100578

J Mol Med (Berl). 2021 Oct 18. doi: 10.1007/s00109-021-02114-x. Online ahead of print.

ABSTRACT

Multiple myeloma patients are often treated with immunomodulatory drugs, proteasome inhibitors, or monoclonal antibodies until disease progression. Continuous therapy in combination with the underlying disease frequently results in severe humoral and cellular immunodeficiency, which often manifests in recurrent infections. Here, we report on the clinical management and immunological data of three multiple-myeloma patients diagnosed with COVID-19. Despite severe hypogammaglobulinemia, deteriorated T cell counts, and neutropenia, the patients were able to combat COVID-19 by balanced response of innate immunity, strong CD8+ and CD4+ T cell activation and differentiation, development of specific T-cell memory subsets, and development of anti-SARS-CoV-2 type IgM and IgG antibodies with virus-neutralizing capacities. Even 12 months after re-introduction of lenalidomide maintenance therapy, antibody levels and virus-neutralizing antibody titers remained detectable, indicating persisting immunity against SARS-CoV-2. We conclude that in MM patients who tested positive for SARS-CoV-2 and were receiving active MM treatment, immune response assessment could be a useful tool to help guide decision-making regarding the continuation of anti-tumor therapy and supportive therapy. KEY MESSAGES: Immunosuppression due to multiple myeloma might not be the crucial factor that is affecting the course of COVID-19. In this case, despite pre-existing severe deficits in CD4+ T-cell counts and IgA und IgM deficiency, we noticed a robust humoral and cellular immune response against SARS-CoV-2. Evaluation of immune response and antibody titers in MM patients that were tested positive for SARS-CoV-2 and are on active MM treatment should be performed on a larger scale; the findings might affect further treatment recommendations for COVID-19, MM treatment re-introduction, and isolation measures.

PMID:34657968 | DOI:10.1007/s00109-021-02114-x

J Cyst Fibros. 2021 Oct 14:S1569-1993(21)01424-7. doi: 10.1016/j.jcf.2021.09.019. Online ahead of print.

ABSTRACT

BACKGROUND: In cystic fibrosis, the respiratory epithelium is the target tissue of both the genetic abnormality of the disease and of external aggressions, notably by pathogens (Pseudomonas aeruginosa). A detailed characterisation of the cystic fibrosis bronchial epithelium is however lacking, as most previous studies focused on the nasal epithelium or on cell lines. This study aimed to characterise the abnormal phenotype and epithelial-to-mesenchymal transition in cystic fibrosis bronchial epithelium and to evaluate in cell cultures whether abnormalities persist ex vivo.

METHODS: Explant lung tissues (n = 44) were assessed for bronchial epithelial cell phenotyping by immunostaining. Human bronchial epithelial cells were derived from basal cells isolated from cystic fibrosis patients or control donors and cultured in air-liquid interface for 2, 4 or 6 weeks.

RESULTS: Enhanced mucin 5AC and decreased β-tubulin expression were observed in cystic fibrosis airways reflecting a decreased ciliated/goblet cell ratio, associated with increased number of vimentin-positive cells, indicating epithelial-to-mesenchymal transition process. These features were recapitulated in vitro, in cystic fibrosis-derived reconstituted epithelium. However, they were not induced by CFTR inhibition or Pseudomonas infection, and most abnormalities tended to disappear in long-term culture (6 weeks) except for increased fibronectin release, an epithelial-to-mesenchymal transition marker.

CONCLUSIONS: This study provides new insights into airway epithelial changes in cystic fibrosis, which are imprinted through an acquired mechanism that we could not relate to CFTR function.

PMID:34657818 | DOI:10.1016/j.jcf.2021.09.019

J Heart Lung Transplant. 2021 Sep 20:S1053-2498(21)02502-X. doi: 10.1016/j.healun.2021.09.007. Online ahead of print.

NO ABSTRACT

PMID:34657795 | DOI:10.1016/j.healun.2021.09.007

Int Immunopharmacol. 2021 Sep 28;101(Pt B):108201. doi: 10.1016/j.intimp.2021.108201. Online ahead of print.

ABSTRACT

One of the major clinical features of COVID-19 is a hyperinflammatory state, which is characterized by high expression of cytokines (such as IL-6 and TNF-α), chemokines (such as IL-8) and growth factors and is associated with severe forms of COVID-19. For this reason, the control of the "cytokine storm" represents a key issue in the management of COVID-19 patients. In this study we report evidence that the release of key proteins of the COVID-19 "cytokine storm" can be inhibited by mimicking the biological activity of microRNAs. The major focus of this report is on IL-8, whose expression can be modified by the employment of a molecule mimicking miR-93-5p, which is able to target the IL-8 RNA transcript and modulate its activity. The results obtained demonstrate that the production of IL-8 protein is enhanced in bronchial epithelial IB3-1 cells by treatment with the SARS-CoV-2 Spike protein and that IL-8 synthesis and extracellular release can be strongly reduced using an agomiR molecule mimicking miR-93-5p.

PMID:34653729 | DOI:10.1016/j.intimp.2021.108201

Altern Ther Health Med. 2021 Oct 15:AT7040. Online ahead of print.

ABSTRACT

As babies and young children are more vulnerable to respiratory illnesses, pediatric respiratory disorders are a leading cause of death and morbidity. Chronic respiratory illnesses in children include tuberculosis, asthma, bronchopulmonary dysplasia and bronchiectasis. Furthermore, hereditary pulmonary diseases such as primary ciliary dyskinesia and cystic fibrosis are seen in children at a lower rate. Most children are examined by non-professional physicians (eg, senior nursing staff) who are specialized in pediatrics, as nursing practice in outpatient settings grows. Some people have a poor understanding of how to care for ill children. As a result, these professionals must be informed of any risk factors and can identify "red flags" in a sick child quickly so that further care may be escalated properly. Some children will require hospitalization for breathing assistance and additional therapy including antibiotics and hydration. With the growth of "non-medical practitioners" in nursing practice, it is essential to be aware of whether or not to administer antibiotic medication, especially given the risks of antibiotic misuse. Nurses are responsible for administering medications and assisting with various therapies, as well as for caring for children and their families during an illness. In specific circumstances, children and parents must be informed about protective and preventative steps that can help reduce the risk for eventual pulmonary disorders, such as vaccinations and smoking cessation.

PMID:34653020

J Clin Endocrinol Metab. 2021 Oct 15:dgab730. doi: 10.1210/clinem/dgab730. Online ahead of print.

NO ABSTRACT

PMID:34652439 | DOI:10.1210/clinem/dgab730

Andes Pediatr. 2021 Aug;92(4):526-533. doi: 10.32641/andespediatr.v92i4.2693.

ABSTRACT

INTRODUCTION: Cystic fibrosis (CF) is a multisystemic disease, with high morbidity and mortality, and its early diag nosis improves results. Lung conditions are the main cause of morbidity and mortality and are clo sely related to nutritional status and survival. There is little national information about the liver and gastrointestinal characteristics in pediatric patients with CF.

OBJECTIVE: to describe at a gastrointes tinal level, the general, nutritional, and genetic characteristics and the evolution of CF carriers with/ without neonatal screening.

PATIENTS AND METHOD: Retrospective study carried out in 4 public referral hospitals in the Metropolitan Region. The diagnosis of CF confirmed with two positive sweat tests (Gibson and Cooke method) was considered as an inclusion criterion. Those patients with unconfir med neonatal screening tests through Immunoreactive Trypsinogen (IRT) or with only one positive sweat test were excluded. Sex, age, nutritional status, date of diagnosis, clinical presentation at the onset, evolution, and therapies received were recorded as clinical variables, and as laboratory ones, genetic study by means of a diagnostic panel with 36 mutations. The STATA 12 software was used for statistical analysis.

RESULTS: 127 patients were included. Respiratory manifestations (recurrent obstructive bronchial syndrome and pneumonia) were present in >60% and gastrointestinal ones (mainly malabsorption and malnutrition syndrome) in >80% of patients. On average, diagnostic confirmation took 4 months. The diagnosis guided by IRT was associated with better nutritional outcomes in the evolution of the patient. In 81.1% of the patients, the genetic study was performed. The most frequent mutations were those associated with DF508 (deletion of phenylalanine 508). 5.8% of the patients presented mutations not included in the gene panel used.

CONCLUSIONS: Gas trointestinal CF appears with pancreatic, intestinal, and hepatic pathology throughout life. Malnutri tion is a frequently present factor, which worsens the prognosis. The management of gastrointestinal manifestations and malnutrition are relevant to improve the morbidity and mortality of CF patients.

PMID:34652370 | DOI:10.32641/andespediatr.v92i4.2693

NPJ Aging Mech Dis. 2021 Oct 14;7(1):26. doi: 10.1038/s41514-021-00079-2.

ABSTRACT

Mitochondrial dysfunction and bioenergetics failure are common pathological hallmarks in Huntington's disease (HD) and aging. In the present study, we used the YAC128 murine model of HD to examine the effects of mutant huntingtin on mitochondrial parameters related to aging in brain and skeletal muscle. We have conducted a cross-sectional natural history study of mitochondrial DNA changes in the YAC128 mouse. Here, we first show that the mitochondrial volume fraction appears to increase in the axons and dendrite regions adjacent to the striatal neuron cell bodies in old mice. Mitochondrial DNA copy number (mtDNAcn) was used as a proxy measure for mitochondrial biogenesis and function. We observed that the mtDNAcn changes significantly with age and genotype in a tissue-specific manner. We found a positive correlation between aging and the mtDNAcn in striatum and skeletal muscle but not in cortex. Notably, the YAC128 mice had lower mtDNAcn in cortex and skeletal muscle. We further show that mtDNA deletions are present in striatal and skeletal muscle tissue in both young and aged YAC128 and WT mice. Tracking gene expression levels cross-sectionally in mice allowed us to identify contributions of age and genotype to transcriptional variance in mitochondria-related genes. These findings provide insights into the role of mitochondrial dynamics in HD pathogenesis in both brain and skeletal muscle, and suggest that mtDNAcn in skeletal muscle tissue may be a potential biomarker that should be investigated further in human HD.

PMID:34650085 | DOI:10.1038/s41514-021-00079-2

Pediatr Pulmonol. 2021 Oct 14. doi: 10.1002/ppul.25669. Online ahead of print.

ABSTRACT

INTRODUCTION: Chronic rhinosinusitis is common among individuals with cystic fibrosis (CF) and has an impact on quality of life. Sinus surgery is a treatment option, but minimal literature exists regarding prevalence and indications.

METHODS: Using the linked CF Foundation Patient Registry (CFFPR) - Pediatric Health Information Systems (PHIS) database, we investigated variability in receipt of surgery, predictors of surgery, and time to first surgery. We included individuals less than 18 receiving care between 2006 and 2015 at a CF Foundation care program that is also a PHIS-participating-hospital. We used logistic regression to examine predictors of receipt of surgery and a Kaplan-Meier curve to examine time to first surgery among those born 2005-2007.

RESULTS: There were 11,545 children and adolescents and 2156 (18.7%) received at least one surgery. Variation in number of surgeries was observed across hospitals (median: 63 [IQR, 33-110]). There was an inconsistent pattern between receipt of surgery and markers of disease severity; those receiving surgery having increased odds of treatment use and pulmonary exacerbations and decreased odds of lower lung function and body mass index. Among the cohort of young children, 159 (14%) had at least one surgery with a median age at first surgery of 5.6 (IQR, 3.9-7.0).

CONCLUSIONS: The use of sinus surgery is frequent, but variable, among children and adolescents. Clinical factors are associated with receipt of surgery, but further understanding is needed on other factors that impact variability in use. Our study indicates the need for additional evaluation of the management of CF-related CRS and indications for surgery.

PMID:34648689 | DOI:10.1002/ppul.25669

J Gen Physiol. 2021 Dec 6;153(12):e202012625. doi: 10.1085/jgp.202012625. Epub 2021 Oct 14.

ABSTRACT

The ATP-binding cassette (ABC) transporter superfamily includes many proteins of clinical relevance, with genes expressed in all domains of life. Although most members use the energy of ATP binding and hydrolysis to accomplish the active import or export of various substrates across membranes, the cystic fibrosis transmembrane conductance regulator (CFTR) is the only known animal ABC transporter that functions primarily as an ion channel. Defects in CFTR, which is closely related to ABCC subfamily members that bear function as bona fide transporters, underlie the lethal genetic disease cystic fibrosis. This article seeks to integrate structural, functional, and genomic data to begin to answer the critical question of how the function of CFTR evolved to exhibit regulated channel activity. We highlight several examples wherein preexisting features in ABCC transporters were functionally leveraged as is, or altered by molecular evolution, to ultimately support channel function. This includes features that may underlie (1) construction of an anionic channel pore from an anionic substrate transport pathway, (2) establishment and tuning of phosphoregulation, and (3) optimization of channel function by specialized ligand-channel interactions. We also discuss how divergence and conservation may help elucidate the pharmacology of important CFTR modulators.

PMID:34647973 | DOI:10.1085/jgp.202012625

Med Sci (Paris). 2021 Oct;37(10):939-941. doi: 10.1051/medsci/2021157. Epub 2021 Oct 14.

NO ABSTRACT

PMID:34647884 | DOI:10.1051/medsci/2021157

Pediatr Pulmonol. 2021 Oct 14. doi: 10.1002/ppul.25727. Online ahead of print.

ABSTRACT

In this commentary, we discuss health disparities, reflecting on our experience in delayed diagnosis of cystic fibrosis based on race and bias in health care practitioners This article is protected by copyright. All rights reserved.

PMID:34647698 | DOI:10.1002/ppul.25727

Infect Prev Pract. 2021 Jun 6;3(3):100153. doi: 10.1016/j.infpip.2021.100153. eCollection 2021 Sep.

ABSTRACT

BACKGROUND: Positive expiratory pressure (PEP) devices are an important element of the management of cystic fibrosis, and of other respiratory diseases. Whereas there have been reports in the literature of contamination of airway clearance devices and their surfaces by microbial pathogens, there is little evidence available regarding such contamination and its contribution to respiratory infection.

AIM: To establish whether pathogenic bacteria can contaminate PEP devices in the context of normal cleaning and maintenance practices.

METHODS: Patients' home-use clearance devices were brought to a routine clinic appointment and collected for microbiology sampling and analysis. The patients were provided with replacement devices. Nineteen such devices were collected from 17 patients, reflecting use of multiple devices by some patients. Swabs were taken and cultured from each patient's used device, the patient's airway, as well as from new unopened and unused devices that acted as controls.

RESULTS: Seven of 19 devices (37%) tested positive for presence of pathogenic bacteria. Device-cleaning methods varied among patients and non-sterilization methods were found to be ineffective at removing pathogens. Microbial species found on the devices did not correlate with those identified from airway swabs.

CONCLUSION: This study demonstrates the presence of pathogens on positive expiratory pressure devices. The potential for transmission of these pathogens to the patient's airway and the risk of infection remains unclear and requires further study.

PMID:34647008 | PMC:PMC8498708 | DOI:10.1016/j.infpip.2021.100153

ERJ Open Res. 2021 Oct 11;7(4):00249-2021. doi: 10.1183/23120541.00249-2021. eCollection 2021 Oct.

ABSTRACT

Lessons learnt from the pandemic show that telehealth for cystic fibrosis allows multidisciplinary visits but better means for monitoring of lung function and microbiology are needed https://bit.ly/2V7g09x.

PMID:34646879 | PMC:PMC8364745 | DOI:10.1183/23120541.00249-2021