Eur J Intern Med. 2023 Nov 1:S0953-6205(23)00383-7. doi: 10.1016/j.ejim.2023.10.031. Online ahead of print.

NO ABSTRACT

PMID:37923586 | DOI:10.1016/j.ejim.2023.10.031

Handb Exp Pharmacol. 2023 Nov 4. doi: 10.1007/164_2023_700. Online ahead of print.

ABSTRACT

Sphingolipids are crucial molecules in the respiratory airways. As in most other tissues and organs, in the lung sphingolipids play an essential role as structural constituents as they regulate barrier function and fluidity of cell membranes. A lung-specific feature is the occurrence of sphingolipids as minor structural components in the surfactant. However, sphingolipids are also key signaling molecules involved in airway cell signaling and their dynamical formation and metabolism are important for normal lung physiology. Dysregulation of sphingolipid metabolism and signaling is involved in altering lung tissue and initiates inflammatory processes promoting the pathogenesis of pulmonal diseases including cystic fibrosis (CF), chronic obstructive pulmonary disease (COPD), and asthma.In the present review, the important role of specific sphingolipid species in pulmonal diseases will be discussed. Only such an understanding opens up the possibility of developing new therapeutic strategies with the aim of correcting the imbalance in sphingolipid metabolism and signaling. Such delivery strategies have already been studied in animal models of these lung diseases, demonstrating that targeting the sphingolipid profile represents new therapeutic opportunities for lung disorders.

PMID:37922034 | DOI:10.1007/164_2023_700

mSystems. 2023 Nov 3:e0047323. doi: 10.1128/msystems.00473-23. Online ahead of print.

ABSTRACT

Hard-wired within the genome of a pathogen is the information regarding factors responsible for its pathogenicity. Advances in functional genomics, particularly gene expression analysis, have made possible genome-wide interrogation of a pathogen to decipher pathogenicity-associated genes. Standard protocols assess differential expression of genes during pathogenesis; however, often a conservative approach is taken, which requires expression fold change above an arbitrarily defined threshold and a statistical significance test to infer a gene as differentially expressing. This renders a high-confidence set of differentially expressed genes in pathogenesis, however, at the cost of numerous false negatives. To circumvent this problem and comprehensively catalog pathogenicity-associated genes, we have developed a novel pipeline that uses standard protocol in combination with gene co-expression network of a pathogen constructed using publicly available RNA-Seq data sets. We assessed the efficacy of this pipeline on Pseudomonas aeruginosa PAO1, a model bacterial pathogen, highlighting the power of our network-based approach in discovering novel genes or pathways associated with the pathogenesis, or antibiotic resistance of this strain. Complementing standard protocol with a gene network-based method thus elevated the ability to identify pathogenicity-associated genes in P. aeruginosa PAO1.IMPORTANCEWe present here a new systems-level approach to decipher genetic factors and biological pathways associated with virulence and/or antibiotic treatment of bacterial pathogens. The power of this approach was demonstrated by application to a well-studied pathogen Pseudomonas aeruginosa PAO1. Our gene co-expression network-based approach unraveled known and unknown genes and their networks associated with pathogenicity in P. aeruginosa PAO1. The systems-level investigation of P. aeruginosa PAO1 helped identify putative pathogenicity and resistance-associated genetic factors that could not otherwise be detected by conventional approaches of differential gene expression analysis. The network-based analysis uncovered modules that harbor genes not previously reported by several original studies on P. aeruginosa virulence and resistance. These could potentially act as molecular determinants of P. aeruginosa PAO1 pathogenicity and responses to antibiotics.

PMID:37921470 | DOI:10.1128/msystems.00473-23

Swiss Med Wkly. 2023 Nov 1;153:40097. doi: 10.57187/smw.2023.40097.

ABSTRACT

AIMS OF THE STUDY: Vitamin A deficiency retinopathy is a potentially blinding disease. In developed countries, vitamin A deficiency due to malnutrition is rare. However, vitamin A deficiency can be caused by malabsorption resulting from bowel resection or medication. In this retrospective study, we present five cases of vitamin A deficiency retinopathy related to malabsorption secondary to medical interventions.

METHODS: Electronic charts over a ten-year period (2012-2022) were screened for vitamin A deficiency retinopathy. Only patients with vitamin A deficiency confirmed by laboratory tests were included. Symptoms, medical history, visual acuity, optical coherence tomography, fundus autofluorescence, electrophysiological examination, and vitamin A levels were reviewed.

RESULTS: Five eligible cases were identified. Median age was 44.7 years (range 22.2-88.9), median duration of ocular symptoms prior to diagnosis was 14 months, and median visual acuity was 1.0 (range 0.5-1.0, Snellen, decimal). Three patients had a history of bariatric surgery, one patient had a small bowel resection and was on octreotide treatment, and one patient suffered from cystic fibrosis and had a history of small bowel resection and severe hepatopathy. Optical coherence tomography showed various abnormalities, including a reduced interdigitation zone, subretinal drusenoid deposits, and a thinned outer nuclear layer. Electroretinogram findings ranged from abnormal oscillatory potentials to non-recordable rod responses.

CONCLUSIONS: Vitamin A deficiency retinopathy can occur following medical interventions associated with malabsorption. In cases of night blindness, vitamin A levels should be measured.

PMID:37921090 | DOI:10.57187/smw.2023.40097

Can J Kidney Health Dis. 2023 Oct 30;10:20543581231205340. doi: 10.1177/20543581231205340. eCollection 2023.

ABSTRACT

BACKGROUND: Living donor kidney transplantation (LDKT) is the optimal treatment for eligible patients with kidney failure, although it is underutilized. Contextually tailored patient- and family-centered interventions may be effective to increase LDKT.

OBJECTIVE: We outline a protocol to test the feasibility of the Multidisciplinary Support To Access living donor Kidney Transplant (MuST AKT) intervention designed to increase LDKT.

DESIGN: Non-blinded single-center pilot randomized controlled trial with a qualitative interview component.

SETTING: Academic transplant referral center in Northern Alberta Region with a population of more than 2 million in its catchment area.

PATIENTS: English-speaking patients of the age range 18 to 75 years who are referred for kidney transplantation are eligible to participate.

MEASUREMENTS: Feasibility will be assessed by indicators of recruitment, retention, and completion rates, treatment fidelity, adherence to intervention, engagement in intervention, and acceptability.

METHODS: Participants will be randomly assigned 1:1 to either standard care (control) or the experimental group who receive standard care plus the MuST AKT intervention, a person-centered program designed to assist and enable the kidney transplant candidate to achieve what is required to receive an LDKT. The intervention consists of an introductory session and 4 intervention sessions delivered in-person or virtually.

LIMITATIONS: Inferences cannot be drawn regarding the efficacy/effectiveness of the MuST AKT intervention. This study is non-blinded.

CONCLUSIONS: This pilot study is the first step in our broader initiative to increase LDKT in our health care jurisdiction. The results of this study will be used to inform the development of a future definitive randomized controlled trial.

TRIAL REGISTRATION NUMBER: NCT04666545.

PMID:37920779 | PMC:PMC10619336 | DOI:10.1177/20543581231205340

RSC Chem Biol. 2023 Aug 29;4(11):865-870. doi: 10.1039/d3cb00126a. eCollection 2023 Nov 1.

ABSTRACT

Upon undergoing mucoid conversion within the lungs of cystic fibrosis patients, the pathogenic bacterium Pseudomonas aeruginosa synthesises copious quantities of the virulence factor and exopolysaccharide alginate. The enzyme guanosine diphosphate mannose dehydrogenase (GMD) catalyses the rate-limiting step and irreversible formation of the alginate sugar nucleotide building block, guanosine diphosphate mannuronic acid. Since there is no corresponding enzyme in humans, strategies that could prevent its mechanism of action could open a pathway for new and selective inhibitors to disrupt bacterial alginate production. Using virtual screening, a library of 1447 compounds within the Known Drug Space parameters were evaluated against the GMD active site using the Glide, FRED and GOLD algorithms. Compound hit evaluation with recombinant GMD refined the panel of 40 potential hits to 6 compounds which reduced NADH production in a time-dependent manner; of which, an usnic acid derivative demonstrated inhibition six-fold stronger than a previously established sugar nucleotide inhibitor, with an IC50 value of 17 μM. Further analysis by covalent docking and mass spectrometry confirm a single site of GMD alkylation.

PMID:37920392 | PMC:PMC10619135 | DOI:10.1039/d3cb00126a

Respir Med Case Rep. 2023 Oct 17;46:101938. doi: 10.1016/j.rmcr.2023.101938. eCollection 2023.

ABSTRACT

Elexacaftor-tezacaftor-ivacaftor (ETI) therapy is shown to improve the health of individuals with cystic fibrosis (CF) who have the F508del variant. There are in vitro studies showing benefit with ETI for select rare CF variants. Limited data exists on the use of ETI in individuals with rare CF variants, particularly in those with advanced lung disease. We present 2 cases of CF individuals homozygous for the rare M1101K variant with end-stage lung disease who demonstrated sustained improvements in lung function, pulmonary exacerbation frequency, respiratory symptoms, and body mass index after 6 months of ETI treatment - similar to that expected with F508del.

PMID:37920361 | PMC:PMC10618485 | DOI:10.1016/j.rmcr.2023.101938

Tanaffos. 2023 Jan;22(1):152-159.

ABSTRACT

BACKGROUND: One of the most common reasons for mortality in patients with cystic fibrosis (CF) is lung infections, among which Pseudomonas aeruginosa (Pa) infection has the largest share. Diagnosis of Pa can be assessed by various methods such as sputum culture results and IgG antibody level via measuring the specific anti-Pa antibodies. This study aimed to select the best predictive technique in the diagnosis of Pa in CF patients through spirometry, sputum culture, and serum IgG antibody levels.

MATERIALS AND METHODS: In this cross-sectional study, blood and sputum or pharyngeal samples were taken from 68 patients with cystic fibrosis. Because spirometry was not possible in all patients, 34 patients could do the spirometry. The samples were studied concerning Pa infection. The data including variables such as age, sex, and spirometry results were obtained. Then, in the serologic method, 3 serum-specific antibody levels were determined by enzyme-linked immune sorbent assay (ELISA).

RESULTS: The average age of children was 7.4 ± 5.6 (ranging from 0.5 to 23) years. Generally, the percentage of Pa infection increased in CF patients with higher ages. A statistically direct significant relationship was observed between the concentration of serum IgG antibodies in patients with CF and Pa-positive sputum culture results (p<0.05).

CONCLUSION: Serum IgG antibodies against specific Pa antigens could be a diagnostic method against Pa infection, especially in patients who cannot expectorate. However, because of the positive and negative predictive value of both serum IgG antibody levels and the results of the sputum culture, we suggested that utilizing the combination of these methods could be beneficial in earlier diagnosis of Pa.

PMID:37920326 | PMC:PMC10618574

Tanaffos. 2023 Jan;22(1):160-166.

ABSTRACT

BACKGROUND: Cystic fibrosis is a chronic and progressive genetic disease with a worldwide prevalence. As the disease progresses, symptoms develop, and make its management more challenging. Accumulating evidence suggests that early diagnosis of CF can significantly contribute to preventing reported nutritional problems including anemia, vitamin deficiencies, and hypoalbuminemia. This cross-sectional study was conducted to assess disease severity in cystic fibrosis patients using the Shwachman-Kulczycki score, as well as to determine its relation with anemia and vitamin D deficiency.

MATERIALS AND METHODS: Clinical and CF-related laboratory data were collected from the medical records of 57 CF patients with a definitive diagnosis. At the time of diagnosis, physicians performed simultaneous, blood sampling and scoring of patients using the Shwachman scoring system.

RESULTS: The mean age of patients was 16.12±6.48 years. Total scores of 86-100, 71-85, 56-70, 41-55, and <40, were reported in 5.4%, 7.1%, 14.3%, 14.3%, and 58.9% of CF patients, respectively. A significant correlation was found between disease severity and patients' age (P=0.02). The analysis also showed that the disease severity was significantly higher in anemic patients when compared to non-anemics (p =0.006). Based on the results, 33 patients with normochromic, 11 patients with microcytic, and 6 patients with macrocytic anemia were diagnosed in this study. We did not find a significant difference between disease severity and vitamin D levels (P=0.150).

CONCLUSION: The scoring system used in the current study could reflect properly the clinical status of CF patients. However, simultaneous use of various methods using a larger sample size for comparison of results is suggested to improve the accuracy of findings.

PMID:37920324 | PMC:PMC10618591

Life Sci Alliance. 2023 Nov 2;7(1):e202302045. doi: 10.26508/lsa.202302045. Print 2024 Jan.

ABSTRACT

CFTR is a membrane protein that functions as an ion channel. Mutations that disrupt its biosynthesis, trafficking or function cause cystic fibrosis (CF). Here, we present a novel in vitro model system prepared using CRISPR/Cas9 genome editing with endogenously expressed WT-CFTR tagged with a HiBiT peptide. To enable the detection of CFTR in the plasma membrane of live cells, we inserted the HiBiT tag in the fourth extracellular loop of WT-CFTR. The 11-amino acid HiBiT tag binds with high affinity to a large inactive subunit (LgBiT), generating a reporter luciferase with bright luminescence. Nine homozygous clones with the HiBiT knock-in were identified from the 182 screened clones; two were genetically and functionally validated. In summary, this work describes the preparation and validation of a novel reporter cell line with the potential to be used as an ultimate building block for developing unique cellular CF models by CRISPR-mediated insertion of CF-causing mutations.

PMID:37918963 | DOI:10.26508/lsa.202302045

J Pediatr. 2023 Oct 31:113812. doi: 10.1016/j.jpeds.2023.113812. Online ahead of print.

ABSTRACT

OBJECTIVE: To identify predictors of change in lung function and body weight during healthcare transition in cystic fibrosis (CF).

METHODS: We conducted a retrospective cohort study using data from the CF Foundation Patient Registry (CFFPR) and the web-based transition program CF RISE (Responsibility. Independence. Self-care. Education) for patients ages 16 to 25 who transitioned to adult care from 2013 through 2019. We modeled change in FEV1 % predicted and weight using linear regression fit with generalized estimating equations. Predictors included gap in care (time between last pediatric and first adult outpatient visit), transition program engagement, and sociodemographic and medical factors.

RESULTS: Among 12,420 adolescents and young adults (AYAs), 3,876 transitioned to adult care with a median gap in care of 7.6 months. Patients from CF centers with higher rates of CF RISE engagement had improved lung function and weight at their first adult outpatient visit. Coverage on a parent's insurance plan and absence of CF complications predicted increased lung function. History of a non-lung transplant and sinus disease predicted increased weight. Comorbid diabetes mellitus and gaps in care >3 months predicted decreased lung function with longer gaps in care associated with greater decrease. A gap in care of 6-9 months predicted decreased weight. Control variables including baseline FEV1 and weight, and exacerbation status were also statistically significant.

CONCLUSIONS: Findings suggest two promising targets to improve transition of AYAs with CF: increasing AYA engagement in CF RISE and reducing gaps in care during the transition period.

PMID:37918520 | DOI:10.1016/j.jpeds.2023.113812

Antioxid Redox Signal. 2023 Nov 2. doi: 10.1089/ars.2023.0410. Online ahead of print.

ABSTRACT

SIGNIFICANCE: Gasotransmitters are small gas molecules that are endogenously generated and have well-defined physiological functions. The most well-defined gasotransmitters currently are nitric oxide (NO), carbon monoxide (CO) and hydrogen sulfide (H2S), while other potent gasotransmitters include ammonia, methane, cyanide, hydrogen gas, and sulfur dioxide. Gasotransmitters play a role in various respiratory diseases such as asthma, chronic obstructive pulmonary disease, obstructive sleep apnoea, lung infection, bronchiectasis, cystic fibrosis, primary ciliary dyskinesia, and COVID-19.

RECENT ADVANCES: Gasotransmitters can act as biomarkers that facilitate disease diagnosis, indicate disease severity, predict disease exacerbation and evaluate disease outcomes. They also have cell-protective properties that many studies have been conducted to explore their pharmacological applications. Innovative drug donors and drug delivery methods have been invented to amplify their therapeutic effects.

CRITICAL ISSUES: In this article, we briefly reviewed the physiological and pathophysiological function of some gasotransmitters in the respiratory system, the progress in detecting exhaled gasotransmitters, as well as innovative drugs derived from these molecules.

FUTURE DIRECTIONS: the current challenge for gasotransmitter research includes further exploring their physiological and pathological function, clarifying their complicated interactions, exploring suitable drug donors and delivery devices, and characterizing new members of gasotransmitters.

PMID:37917094 | DOI:10.1089/ars.2023.0410

mSystems. 2023 Nov 2:e0072423. doi: 10.1128/msystems.00724-23. Online ahead of print.

ABSTRACT

Shotgun metagenomic sequencing has the potential to provide bacterial strain-level resolution which is of key importance to tackle a host of clinical questions. While bioinformatic tools that achieve strain-level resolution are available, thorough benchmarking is needed to validate their use for less investigated and low biomass microbiomes like those from the upper respiratory tract. We analyzed a previously published data set of longitudinally collected nasopharyngeal samples from Bangladeshi infants (Microbiota and Health study) and a novel data set of oropharyngeal samples from Swiss children with cystic fibrosis. Data from bacterial cultures were used for benchmarking the parameters of StrainPhlAn 3, a bioinformatic tool designed for strain-level resolution. In addition, StrainPhlAn 3 results were compared with metagenomic assemblies derived from StrainGE and newly derived whole-genome sequencing data. After optimizing the analytical parameters, we compared StrainPhlAn 3 results to culture gold standard methods and achieved sensitivity values of 87% (Streptococcus pneumoniae), 80% (Moraxella catarrhalis), 75% (Haemophilus influenzae), and 57% (Staphylococcus aureus) for 420 nasopharyngeal and 75% (H. influenzae) and 46% (S. aureus) for 260 oropharyngeal samples. Comparing the phylogenetic tree of the core genome of 50 S. aureus isolates with a corresponding marker gene tree generated by StrainPhlAn 3 revealed a striking similarity in tree topology for all but three samples indicating adequate strain resolution. In conclusion, a comparison of StrainPhlAn 3 results to data from bacterial cultures revealed that strain-level tracking of the respiratory microbiome is feasible despite the high content of host DNA when parameters are carefully optimized to fit low biomass microbiomes.IMPORTANCEThe usage of 16S rRNA gene sequencing has become the state-of-the-art method for the characterization of the microbiota in health and respiratory disease. The method is reliable for low biomass samples due to prior amplification of the 16S rRNA gene but has limitations as species and certainly strain identification is not possible. However, the usage of metagenomic tools for the analyses of microbiome data from low biomass samples is not straight forward, and careful optimization is needed. In this work, we show that by validating StrainPhlAn 3 results with the data from bacterial cultures, the strain-level tracking of the respiratory microbiome is feasible despite the high content of host DNA being present when parameters are carefully optimized to fit low biomass microbiomes. This work further proposes that strain retention analyses are feasible, at least for more abundant species. This will help to better understand the longitudinal dynamics of the upper respiratory microbiome during health and disease.

PMID:37916972 | DOI:10.1128/msystems.00724-23

Biol Direct. 2023 Nov 1;18(1):71. doi: 10.1186/s13062-023-00430-5.

ABSTRACT

Cancer immunotherapy, alone or in combination with conventional therapies, has revolutionized the landscape of antineoplastic treatments, with dendritic cells (DC) emerging as key orchestrators of anti-tumor immune responses. Among the distinct DC subsets, conventional type 1 dendritic cells (cDC1) have gained prominence due to their unique ability to cross-present antigens and activate cytotoxic T lymphocytes. This review summarizes the distinctive characteristics of cDC1, their pivotal role in anticancer immunity, and the potential applications of cDC1-based strategies in immunotherapy.

PMID:37907944 | PMC:PMC10619282 | DOI:10.1186/s13062-023-00430-5

Paediatr Respir Rev. 2023 Sep 15:S1526-0542(23)00064-7. doi: 10.1016/j.prrv.2023.09.001. Online ahead of print.

ABSTRACT

Highly effective modulator therapies (HEMTs) have revolutionised the management approach of most patients living with cystic fibrosis (CF) who have access to these therapies. Clinical trials have reported significant improvements across multiorgan systems, with patients surviving longer. However, there are accumulating case reports and observational data describing various adverse events following initiation of HEMTs including drug-to-drug interactions, drug induced liver injury, Stevens-Johnson syndrome, and neurocognitive symptoms including psychosis and depression, which have required discontinuation of therapy. Current clinical trials are assessing efficacy in younger patients with CF, yet long-term studies are also required to better understand the safety profile in the real-world setting across all ages and the impact of HEMT dose alteration or discontinuation.

PMID:37914566 | DOI:10.1016/j.prrv.2023.09.001

N Engl J Med. 2023 Nov 2;389(18):1693-1707. doi: 10.1056/NEJMra2216474.

NO ABSTRACT

PMID:37913507 | DOI:10.1056/NEJMra2216474

Andes Pediatr. 2023 Jun;94(3):392-400. doi: 10.32641/andespediatr.v94i3.4494.

ABSTRACT

Several studies have supported the positive effect of respiratory rehabilitation (RR) in children and adolescents (CRA) with chronic respiratory diseases (CRD); however, qualitative aspects related to the experiences and perceptions about RR have been scarcely studied.

OBJECTIVE: to analyze the qualitative evidence regarding the perceptions and experiences of patients, families and professionals related to the RR of children and adolescents with CKD.

METHODS: Review of qualitative studies in 5 databases. We used MeSH terms and free English-language terms grouped into three dimensions: patients, intervention, and research design. The study subjects had to be patients, their families, teachers or treating health teams. No restrictions were placed on language or year of publication. The search strategy was configured as follows: ((Cystic fibrosis) OR (Asthma) OR (Neuromuscular diseases)) AND ((Respiratory rehabilitation) OR (Exercise)) AND ((Qualitative research) OR (Phenomenology) OR (Grounded theory) OR (Ethnography)). Two independent authors analyzed atingent titles, abstracts and long texts. Finally, a qualitative description of the results was made.

RESULTS: Twenty-one qualitative studies were selected, all on patients, family members, teachers or professionals treating patients with cystic fibrosis (CF), asthma or neuromuscular diseases (NMD). Perception of benefits, parental influence, enjoyment of the protocols, and time required to engage in physical activity were categories identified in all three groups. Aspects such as perceived safety on school grounds and parental stress were specific categories, highlighted in the context of asthma and CF respectively.

CONCLUSION: Several general and specific factors of a qualitative nature influence the experience of children with CRD during RR. Future studies conducted in our cultural context should be conducted to confirm these results.

PMID:37909943 | DOI:10.32641/andespediatr.v94i3.4494

Infect Immun. 2023 Nov 1:e0041623. doi: 10.1128/iai.00416-23. Online ahead of print.

ABSTRACT

Cystic fibrosis (CF) is a genetic disease affecting epithelial ion transport, resulting in thickened mucus and impaired mucociliary clearance. Persons with CF (pwCF) experience life-long infections of the respiratory mucosa caused by a diverse array of opportunists, which are leading causes of morbidity and mortality. In recent years, there has been increased appreciation for the range and diversity of microbes causing CF-related respiratory infections. The introduction of new therapeutics and improved detection methodology has revealed CF-related opportunists such as Achromobacter xylosoxidans (Ax). Ax is a Gram-negative bacterial species which is widely distributed in environmental sources and has been increasingly observed in sputa and other samples from pwCF, typically in patients in later stages of CF disease. In this study, we characterized CF clinical isolates of Ax and tested colonization and persistence of Ax in respiratory infection using immortalized human CF respiratory epithelial cells and BALB/c mice. Genomic analyses of clinical Ax isolates showed homologs for factors including flagellar synthesis, antibiotic resistance, and toxin secretion systems. Ax isolates adhered to polarized cultures of CFBE41o- human immortalized CF bronchial epithelial cells and caused significant cytotoxicity and depolarization of cell layers. Ax colonized and persisted in mouse lungs for up to 72 h post infection, with inflammatory consequences that include increased neutrophil influx in the lung, lung damage, cytokine production, and mortality. We also identified genes that are differentially expressed in synthetic CF sputum media. Based on these results, we conclude that Ax is an opportunistic pathogen of significance in CF.

PMID:37909751 | DOI:10.1128/iai.00416-23

J Bras Pneumol. 2023 Oct 30;49(5):e20230201. doi: 10.36416/1806-3756/e20230201. eCollection 2023.

ABSTRACT

OBJECTIVE: Mobile health (mHealth) applications are scarce for children and adolescents with chronic pulmonary diseases (CPDs). This study aimed to map and describe the contents of the mHealth apps available for use in children and adolescents with CPDs.

METHODS: We performed a systematic mapping review of published scientific literature in PubMed, Scopus, and Cochrane Library by February of 2023, using relevant keywords. Inclusion criteria were as follows: children aged < 18 years with CPDs; and studies published in English on mHealth apps.

RESULTS: A total number of 353 studies were found, 9 of which met the inclusion criteria. These studies described seven mHealth apps for Android and iOS, designed either for asthma (n = 5) or for cystic fibrosis (n = 2). Five content areas were identified: education/information; pharmacological treatment; emergency; support; and non-pharmacological treatment. The studies (4, 2, and 3, respectively) showed consistent findings using qualitative, quantitative, and mixed methodologies.

CONCLUSIONS: This mapping review provided a guided selection of the most appropriate mHealth apps for use in children and adolescents with CPDs based on the needs of each target population. However, these mHealth apps have limited capabilities to reinforce disease self-management and provide information related to treatment compliance.

PMID:37909552 | DOI:10.36416/1806-3756/e20230201

Heliyon. 2023 Oct 23;9(11):e21469. doi: 10.1016/j.heliyon.2023.e21469. eCollection 2023 Nov.

ABSTRACT

People with cystic fibrosis-related diabetes (CFRD) suffer from chronic infections with Staphylococcus aureus and/or Pseudomonas aeruginosa. In people with CFRD, the concentration of glucose in the airway surface liquid (ASL) was shown to be elevated from 0.4 to 4 mM. The effect of glucose on bacterial growth/interactions in ASL is not well understood and here we studied the relationship between these lung pathogens in artificial sputum medium (ASM), an environment similar to ASL in vivo. S. aureus exhibited more rapid adaptation to growth in ASM than P. aeruginosa. Supplementation of ASM with glucose significantly increased the growth of S. aureus (p < 0.01, n = 5) and P. aeruginosa (p < 0.001, n = 3). ASM conditioned by the presence of S. aureus promoted growth of P. aeruginosa with less lag time compared with non-conditioned ASM, or conditioned medium that had been heated to 121 °C. Stable co-culture of S. aureus and P. aeruginosa could be established in a 50:50 mix of ASM and S. aureus-conditioned supernatant. These data indicate that glucose, in a nutrient depleted environment, can promote the growth of S. aureus and P. aeruginosa. In addition, heat labile factors present in S. aureus pre-conditioned ASM promoted the growth of P. aeruginosa. We suggest that the use of ASM allows investigation of the effects of nutrients such as glucose on common lung pathogens. ASM could be further used to understand the relationship between S. aureus and P. aeruginosa in a co-culture scenario. Our model of stable co-culture could be extrapolated to include other common lung pathogens and could be used to better understand disease progression in vitro.

PMID:37908712 | PMC:PMC10613906 | DOI:10.1016/j.heliyon.2023.e21469