J Cyst Fibros. 2021 Oct 28:S1569-1993(21)01296-0. doi: 10.1016/j.jcf.2021.06.012. Online ahead of print.

ABSTRACT

There has been a growing number of infants identified as CRMS/CFSPID in countries applying genetic testing as part of cystic fibrosis (CF) newborn screening. Currently there are neither standardized protocols for follow up beyond infancy, nor established predictors to stratify this population as high or low risk of reclassification to CF or CFTR-related disorder. We report a series of 10 children who reclassified, including eight carrying CFTR variants of varying clinical consequence and seven with initial sweat chloride measurements <30 mmol/L. The overall increase in sweat chloride concentration was 5.8 mmol/L/year. Pseudomonas aeruginosa was isolated from respiratory cultures in five subjects, and reclassification was aided by human nasal epithelial cultures in two cases. In this center's experience, 6% of all CRMS/CFSPID referrals reclassified to CF over a 12-year period. The rate of sweat chloride increase, genotype, and CFTR functional assay can potentially be used as prognostic tools in the CRMS/CFSPID population.

PMID:34756682 | DOI:10.1016/j.jcf.2021.06.012

J Cyst Fibros. 2021 Oct 29:S1569-1993(21)02103-2. doi: 10.1016/j.jcf.2021.10.001. Online ahead of print.

ABSTRACT

BACKGROUND: Various bacterial and viral assemblages composing Cystic Fibrosis (CF) lung microbiota contribute to long-term lung function decline over time. Yet, the impact of individual microorganisms on pulmonary functions remains uncertain in children with CF.

METHODS: As part of the 'Mucoviscidosis, respiratory VIruses, intracellular Bacteria and fastidious organisms'' project, children with CF were longitudinally followed in a Swiss multicentric study. Respiratory samples included mainly throat swabs and sputa samples for bacterial culture and 16S rRNA metagenomics and nasopharyngeal swabs for respiratory virus detection by molecular assays. Percentage of predicted Forced Expiratory Volume in one second (FEV1%) and Lung Clearance Index (LCI) were recorded.

RESULTS: Sixty-one children, of whom 20 (32.8%) presented with at least one pulmonary exacerbation, were included. Almost half of the 363 nasopharyngeal swabs tested by RT-PCR were positive for a respiratory virus, mainly rhinovirus (26.5%). From linear mixed-effects regression models, P. aeruginosa (-11.35, 95%CI [-17.90; -4.80], p = 0.001) was significantly associated with a decreased FEV1%, whereas rhinovirus was associated with a significantly higher FEV1% (+4.24 95%CI [1.67; 6.81], p = 0.001). Compared to conventional culture, 16S rRNA metagenomics showed a sensitivity and specificity of 80.0% and 85.4%, respectively for detection of typical CF pathogens. However, metagenomics detected a bacteria almost twice more often than culture.

CONCLUSIONS: As expected, P. aeruginosa impacted negatively on FEV1% while rhinovirus was surprisingly associated with better FEV1%. Culture-free assays identifies significantly more pathogens than standard culture, with disputable clinical correlation.

PMID:34756681 | DOI:10.1016/j.jcf.2021.10.001

Life Sci Space Res (Amst). 2020 Nov;27:49-55. doi: 10.1016/j.lssr.2020.07.005. Epub 2020 Jul 22.

ABSTRACT

The journey to Mars will be an ambitious, yet arduous task as it will entail culmination of all the information we have gathered over many decades. While the mission is of utmost importance, preservation of astronaut's well-being is paramount also. To that end, mitigation of radiation risk especially afflicting cardiovascular disease (CVD) is of great interest and challenge. Current data from astronauts on low earth orbit and Apollo missions provides insight on the risk of CVD from radiation exposure. However, data is limited given the small cohort size of astronauts who embarked on just nine prolonged missions. Therefore, a cerebral approach to understanding and mitigating risks are essential. This paper discusses the need for a predictive preclinical model to help understand and mitigate the effects of radiation on astronauts. We will discuss strengths and limitations of preclinical models and the methods of validating and constructing a model to predict human clinical outcomes. Our bedside-bench-bedside approach focuses on adapting the preclinical model through common investigative tools used between humans and animals. The result will be an optimization of preclinical model to a point of being a surrogate clinical model capable of predicting CVD outcomes in astronauts exposed to radiation.

PMID:34756229 | DOI:10.1016/j.lssr.2020.07.005

Asian J Androl. 2021 Nov 5. doi: 10.4103/aja202177. Online ahead of print.

ABSTRACT

Cystic fibrosis (CF) is one of the most common recessive genetic diseases, with a wide spectrum of phenotypes, ranging from infertility to severe pulmonary disease. Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene are considered the main genetic cause for CF. In this study, we recruited a consanguineous Iranian pedigree with four male patients diagnosed with congenital unilateral absence of the vas deferens (CUAVD), and one female patient diagnosed with congenital absence of the uterus (CAU). Testicular biopsy of one patient was performed, and hematoxylin and eosin (H and E) staining of testis sections displayed the presence of germ cell types ranging from spermatogonia to mature spermatids, indicating obstructive azoospermia. To explore the underlying genetic factor in this familial disorder, we therefore performed whole-exome sequencing (WES) on all available family members. WES data filtration and CFTR haplotype analysis identified compound heterozygous mutations in CFTR among four patients (two CUAVD patients carried p.H949Y and p.L997F, and one CUAVD and the female CAU patient carried p.H949Y and p.I148T). All these mutations were predicted to be deleterious by at least half of the prediction software programs and were confirmed by Sanger sequencing. Our study reported that CFTR compound heterozygous mutations in a consanguineous Iranian family cause infertility in both sexes.

PMID:34755701 | DOI:10.4103/aja202177

Appl Microbiol Biotechnol. 2021 Nov 10. doi: 10.1007/s00253-021-11667-3. Online ahead of print.

ABSTRACT

Bacteria belonging to the Burkholderia genus are extremely versatile and diverse. They can be environmental isolates, opportunistic pathogens in cystic fibrosis, immunocompromised or chronic granulomatous disease patients, or cause disease in healthy people (e.g., Burkholderia pseudomallei) or animals (as in the case of Burkholderia mallei). Since the genus was separated from the Pseudomonas one in the 1990s, the methodological tools to study and characterize these bacteria are evolving fast. Here we reviewed the techniques used in the last few years to update the taxonomy of the genus, to study gene functions and regulations, to deepen the knowledge on the drug resistance which characterizes these bacteria, and to elucidate their mechanisms to establish infections. The availability of these tools significantly impacts the quality of research on Burkholderia and the choice of the most appropriated is fundamental for a precise characterization of the species of interest.Key points• Updated techniques to study the genus Burkholderia were reviewed.• Taxonomy, genomics, assays, and animal models were described.• A comprehensive overview on recent advances in Burkholderia studies was made.

PMID:34755214 | DOI:10.1007/s00253-021-11667-3

J Cyst Fibros. 2021 Nov 6:S1569-1993(21)02110-X. doi: 10.1016/j.jcf.2021.10.008. Online ahead of print.

ABSTRACT

Physical activity (PA) and exercise have numerous benefits in Cystic Fibrosis (CF) including improved lung function, exercise capacity and quality of life. Despite these benefits, the effectiveness of interventions to promote PA in this population are still largely unknown. The objective of this review was to synthesise existing research and determine whether exercise interventions are effective in promoting PA in people with CF. Using the PRISMA guidelines, a comprehensive search was conducted. Fifteen studies (463 participants) met the inclusion criteria. Eleven studies demonstrated improvements in PA in both short- and long-term interventions. However, the interventions were variable across the included studies, with a large inconsistency in PA assessment tools used. Aerobic training and activity counselling were the two elements identified in this review which most consistently improved PA. Future research should consider larger sample sizes and the use of accurate instruments to assess and track PA levels longitudinally.

PMID:34753671 | DOI:10.1016/j.jcf.2021.10.008

Am J Epidemiol. 2021 Nov 9:kwab263. doi: 10.1093/aje/kwab263. Online ahead of print.

ABSTRACT

When an entire cohort of patients receives a treatment it is difficult to estimate the treatment effect in the treated because there are no directly comparable untreated patients. Attempts can be made to find a suitable control group, (e.g. historical controls), but underlying differences between the treated and untreated can result in bias. We show how negative control outcomes (NCO) combined with difference-in-differences analysis can be used to assess bias in treatment effect estimates and obtain unbiased estimates under certain assumptions. Causal diagrams and potential outcomes are used to explain the methods and assumptions. We apply the methods to UK Cystic Fibrosis (CF) Registry data to investigate the effect of ivacaftor, introduced in 2012 for a subset of the CF population with a particular genotype, on lung function and days receiving intravenous antibiotics (IV days). We consider two NCOs: outcomes measured in the pre-ivacaftor period and outcomes in individuals ineligible for ivacaftor due to their genotype. Ivacaftor was found to improve lung function in year one (~6.5 increase in FEV1%), was associated with reduced lung function decline (~0.5 decrease in annual FEV1% decline, though confidence intervals include 0), and reduced the rate of IV days (~60% over 3 years).

PMID:34753177 | DOI:10.1093/aje/kwab263

J Vasc Interv Radiol. 2021 Nov 6:S1051-0443(21)01469-X. doi: 10.1016/j.jvir.2021.10.029. Online ahead of print.

ABSTRACT

PURPOSE: To investigate the prevalence, clinical characteristics, and long-term prognosis of bronchial artery aneurysm (BAA) following bronchial artery embolization (BAE).

MATERIALS AND METHODS: The medical records of consecutive patients who underwent bronchial artery angiography between August 2013 to December 2019 were reviewed retrospectively. Patients who were diagnosed with BAA during this period were enrolled in this study. The prevalence, patients' characteristics, symptoms, comorbidities, angiographic findings, and long-term prognosis following BAE were investigated.

RESULTS: BAA was observed in 20 out of 508 patients who underwent bronchial artery angiography (3.9%). The patients' median age was 69 years (range 44-91). The main causes of BAA were cryptogenic, bronchiectasis/cystic fibrosis, and pulmonary aspergillosis. The median diameter of the ruptured BAAs was significantly smaller than that of the unruptured BAAs [5.4 mm (4.8-7.3 mm) versus 9.0 mm (7.2-13.9 mm), p = 0.009]. All patients were successfully treated with BAE without major adverse events. The median follow-up period after BAE was 970 (quartiles: 561-1796) days. The BAA-related survival rate was 100% at 2 and 3 years after BAE, and the overall survival rate after BAE was 89.2% (95% CI: 89.0-89.3) at 2 years and 74.3% (95% CI, 74.0-74.5) at 3 years. BAA related adverse events and mortality did not occur during the follow up period.

CONCLUSION: BAA was observed in 3.9 % (20/508) of patients who underwent bronchial artery angiography. All patients with BAA were successfully treated by BAE. BAA rupture and consequent mortality did not occur during follow up period.

PMID:34752932 | DOI:10.1016/j.jvir.2021.10.029

J Pharm Technol. 2020 Oct;36(5):196-201. doi: 10.1177/8755122520940710. Epub 2020 Aug 10.

ABSTRACT

Background: Piperacillin/tazobactam (PTZ) extended infusion (EI) is often used empirically in the intensive care unit (ICU). Gram-negative (GN) organisms with PTZ minimum inhibitory concentrations (MICs) >16/4 µg/mL are considered intermediate or resistant. Objective: The objective of this study was to evaluate MICs of GN isolates from the ICU to determine whether the hospital protocol for PTZ 3.375 g EI over 4 hours administered every 8 hours is an appropriate empiric regimen for ICU patients and to evaluate patient-specific risk factors associated with elevated MICs. Methods: All ICU patients admitted during 2017 with a confirmed GN organism from a non-urinary source were included for retrospective chart review. Patients with cystic fibrosis or cultures obtained >48 hours prior to ICU admission were excluded. Demographics, GN organism, culture source, risk factors for resistance, susceptibility profile, comorbidities, and creatinine clearance were collected. Appropriateness was defined as PTZ MIC ≤16/4 µg/mL in >80% of isolates. Results: Two hundred and thirty-one patients were included. The average patient was 56 years old. The majority of patients were white (64.1%) and male (69.7%). Pseudomonas aeruginosa (41%) was the most common organism isolated. Overall, 28% of GN isolates had MICs >16/4 µg/mL. Dialysis (P = .01), intravenous antibiotics within 90 days (P < .001), and presence of wounds/trauma (P = .01) were associated with elevated MICs. Conclusion: Current PTZ EI 3.375 g dosing regimens may not provide adequate empiric coverage for some GN organisms in ICU patients, especially for those who have previously received intravenous antibiotics, are on dialysis, or have wounds/trauma.

PMID:34752564 | PMC:PMC7453472 | DOI:10.1177/8755122520940710

Pediatr Pulmonol. 2021 Nov 9. doi: 10.1002/ppul.25753. Online ahead of print.

ABSTRACT

Noninvasive ventilation (NIV) use was initially reported in cystic fibrosis (CF) in 1991 as a bridge to lung transplantation, and over the decades, the use of NIV has increased in the CF population. Individuals with CF are prone to various physiologic changes as lung function worsens, and they benefit from NIV for advanced lung disease. As life expectancy in CF has been increasing due to advances such as highly effective modulator therapy, people with CF may also benefit from NIV for other diagnosis beyond advanced lung disease. NIV can improve gas exchange, quality of sleep, exercise tolerance and augment airway clearance in CF. CF providers can readily become comfortable with this therapeutic modality. In this review, we will summarize the physiologic basis for NIV use in CF, describe indications for initiation, and discuss how to order and monitor patients on NIV. We will discuss aspects unique to people with CF and the use of NIV, as well as suggestions on how to reduce risks such as infection. We hope that this serves as a resource for CF providers, in particular those who do not have dedicated training in sleep medicine as we all continue to care for the CF patient population This article is protected by copyright. All rights reserved.

PMID:34751000 | DOI:10.1002/ppul.25753

J Clin Psychol Med Settings. 2021 Nov 8. doi: 10.1007/s10880-021-09831-y. Online ahead of print.

ABSTRACT

Self-compassion is increasingly recognised as an important and beneficial factor in quality of life and mental health-related research, but research within the adult cystic fibrosis (CF) population is scarce. In a cross-sectional study, 114 (56 female, 58 male) adults with CF completed and returned a series of validated questionnaires that assessed CF-related quality of life, negative emotional states (depression, anxiety and stress), self-compassion, and self-criticism. Quality of life and self-compassion were positively correlated, and each in turn were inversely correlated with negative emotional states and self-criticism. Negative emotional states correlated positively to self-criticism. Self-compassion and/or self-criticism moderated ten relationships between various sub-domains of quality of life and negative emotions. Psychological interventions that increase self-compassion may be beneficial for enhancing mental health and quality of life for adults with CF.

PMID:34750694 | DOI:10.1007/s10880-021-09831-y

J Physiol Anthropol. 2021 Nov 8;40(1):19. doi: 10.1186/s40101-021-00269-7.

ABSTRACT

BACKGROUND: Cystic fibrosis (CF) is one of the most common autosomal recessive diseases. Factors contributing to disease exacerbations and survival rate of CF patients are type of mutation in the CFTR gene, poor nutritional status, lung failure, and infection development by Pseudomonas aeruginosa. The study aimed to evaluate the relationship between the severity of mutation, nutritional status, lung function, and Pseudomonas aeruginosa prevalence and survival rate in adult patients with cystic fibrosis.

METHODS: A study of 124 (68 ♀ and 56 ♂) adults with CF aged 18-51 years were evaluated for (a) type of mutation in the CFTR gene, (b) nutritional status (BMI), (c) lung function (FEV1%), and (d) Pseudomonas aeruginosa prevalence. For statistical calculations, Kaplan-Meier analysis of survival, chi-squared test for multiple samples, and logistic regression were used.

RESULTS: The type of mutation (χ2 = 12.73, df = 3, p = 0.005), FEV1% (χ2 = 15.20, df = 2, p = 0.0005), Pseudomonas aeruginosa prevalence (χ2 = 11.48, df = 3, p = 0.009), and BMI (χ2 = 31.08, df = 4, p < 0.000) significantly differentiated the probability of survival of patients with CF. The shortest life expectancy was observed in patients with a severe type of mutation on both alleles, FEV1% < 40, subjects in whom Pseudomonas culture was extensively drug-resistant or pandrug-resistant, and patients whose BMI was lower than 18.5 kg/m2. The period from 30 to 40 years of age was the most critical in CF adults' lifespan. The risk of adults with CF death doubled with Pseudomonas aeruginosa prevalence (OR = 2.06, 95% CI 1.29; 2.28) and eightfold when the bacteria acquired antibiotic resistance (OR = 8.11, 95% CI 1.67; 38.15).

CONCLUSIONS: All factors included in the study were significantly related to the survival rate of patients with cystic fibrosis.

PMID:34749804 | DOI:10.1186/s40101-021-00269-7

Am J Respir Cell Mol Biol. 2021 Nov 8. doi: 10.1165/rcmb.2021-0423ED. Online ahead of print.

NO ABSTRACT

PMID:34748716 | DOI:10.1165/rcmb.2021-0423ED

Microb Drug Resist. 2021 Nov 5. doi: 10.1089/mdr.2020.0238. Online ahead of print.

ABSTRACT

We characterized Staphylococcus aureus strains isolated from cystic fibrosis (CF) patients during screening for multidrug-resistant strains to determine mechanisms of antibiotic resistance and conduct spa typing. We investigated 53 S. aureus isolates collected from different CF patients, excluding multiple isolates from the same patient. Genotypic characterization was based on spa type (protein A); staphylococcal cassette chromosome mec (SCCmec) type for S. aureus resistant to methicillin (methicillin-resistant S. aureus [MRSA]); and resistance to the most common macrolides, lincosamides, and streptogramins b and fluoroquinolones. Most strains (78.41%) were resistant to one or more antibiotics; 16.96% were MRSA, whereas 69.81% showed resistance to erythromycin. MRSA strains revealed the acquisition and insertion of SCCmec of class I (n = 1) (hospital-acquired), IV (n = 5), and V (n = 1) (community-acquired), along with two cases that were not typeable. We detected 34 different spa types, with t571 being the most frequent. The spa minimum spanning tree of the tested strains showed evidence of strain relatedness.

PMID:34748406 | DOI:10.1089/mdr.2020.0238

Med Decis Making. 2021 Nov 7:272989X211049213. doi: 10.1177/0272989X211049213. Online ahead of print.

ABSTRACT

BACKGROUND: Patient surveillance using repeated biomarker measurements presents an opportunity to detect and treat disease progression early. Frequent surveillance testing using biomarkers is recommended and routinely conducted in several diseases, including cancer and diabetes. However, frequent testing involves tradeoffs. Although surveillance tests provide information about current disease status, the complications and costs of frequent tests may not be justified for patients who are at low risk of progression. Predictions based on patients' earlier biomarker values may be used to inform decision making; however, predictions are uncertain, leading to decision uncertainty.

METHODS: We propose the Personalized Risk-Adaptive Surveillance (PRAISE) framework, a novel method for embedding predictions into a value-of-information (VOI) framework to account for the cost of uncertainty over time and determine the time point at which collection of biomarker data would be most valuable. The proposed sequential decision-making framework is innovative in that it leverages the patient's longitudinal history, considers individual benefits and harms, and allows for dynamic tailoring of surveillance intervals by considering the uncertainty in current information and estimating the probability that new information may change treatment decisions, as well as the impact of this change on patient outcomes.

RESULTS: When applied to data from cystic fibrosis patients, PRAISE lowers costs by allowing some patients to skip a visit, compared to an "always test" strategy. It does so without compromising expected survival, by recommending less frequent testing among those who are unlikely to be treated at the skipped time point.

CONCLUSIONS: A VOI-based approach to patient monitoring is feasible and could be applied to several diseases to develop more cost-effective and personalized strategies for ongoing patient care.

HIGHLIGHTS: In many patient-monitoring settings, the complications and costs of frequent tests are not justified for patients who are at low risk of disease progression. Predictions based on patient history may be used to individualize the timing of patient visits based on evolving risk.We propose Personalized Risk-Adaptive Surveillance (PRAISE), a novel method for personalizing the timing of surveillance testing, where prediction modeling projects the disease trajectory and a value-of-information (VOI)-based pragmatic decision-theoretic framework quantifies patient- and time-specific benefit-harm tradeoffs.A VOI-based approach to patient monitoring could be applied to several diseases to develop more personalized and cost-effective strategies for ongoing patient care.

PMID:34747265 | DOI:10.1177/0272989X211049213

Front Med (Lausanne). 2021 Oct 21;8:737724. doi: 10.3389/fmed.2021.737724. eCollection 2021.

NO ABSTRACT

PMID:34746176 | PMC:PMC8566914 | DOI:10.3389/fmed.2021.737724

Front Cell Infect Microbiol. 2021 Oct 22;11:734296. doi: 10.3389/fcimb.2021.734296. eCollection 2021.

ABSTRACT

Pseudomonas aeruginosa and Aspergillus fumigatus infections frequently co-localize in lungs of immunocompromised patients and individuals with cystic fibrosis (CF). The antifungal activity of P. aeruginosa has been described for its filtrates. Pyoverdine and pyocyanin are the principal antifungal P. aeruginosa molecules active against A. fumigatus biofilm metabolism present in iron-limited or iron-replete planktonic P. aeruginosa culture filtrates, respectively. Using various P. aeruginosa laboratory wild-type strains (PA14, PAO1, PAK), we found antifungal activity against Aspergillus colonies on agar. Comparing 36 PA14 and 7 PAO1 mutants, we found that mutants lacking both major siderophores, pyoverdine and pyochelin, display higher antifungal activity on agar than their wild types, while quorum sensing mutants lost antifungal activity. Addition of ferric iron, but not calcium or magnesium, reduced the antifungal effects of P. aeruginosa on agar, whereas iron-poor agar enhanced antifungal effects. Antifungal activity on agar was mediated by PQS and HHQ, via MvfR. Among the MvfR downstream factors, rhamnolipids and elastase were produced in larger quantities by pyoverdine-pyochelin double mutants and showed antifungal activity on agar. In summary, antifungal factors produced by P. aeruginosa on agar differ from those produced by bacteria grown in liquid cultures, are dependent on quorum sensing, and are downregulated by the availability of ferric iron. Rhamnolipids and elastase seem to be major mediators of Pseudomonas' antifungal activity on a solid surface.

PMID:34746024 | PMC:PMC8570168 | DOI:10.3389/fcimb.2021.734296

J Clin Transl Endocrinol. 2021 Oct 13;26:100267. doi: 10.1016/j.jcte.2021.100267. eCollection 2021 Dec.

ABSTRACT

Spontaneous episodes of hypoglycemia can occur in people with cystic fibrosis (CF) without diabetes, who are not on glucose lowering medications. Spontaneous hypoglycemia in CF could occur both in the fasting or postprandial state (reactive hypoglycemia). The pathophysiology of fasting hypoglycemia is thought to be related to malnutrition and increased energy expenditure in the setting of inflammation and acute infections. Reactive hypoglycemia is thought to be due to impaired first phase insulin release in response to a glucose load, followed by a delayed and extended second phase insulin secretion; ineffective counterregulatory response to dropping glucose levels may also play a role. The overall prevalence of spontaneous hypoglycemia varies from 7 to 69% as examined with oral glucose tolerance test (OGTT) or with continuous glucose monitoring (CGM) under free living conditions. Spontaneous hypoglycemia in CF is associated with worse lung function, higher hospitalization rates, and worse clinical status. In addition, patients with CF related diabetes on glucose-lowering therapies are at risk for iatrogenic hypoglycemia. In this article, we will review the pathophysiology, prevalence, risk factors, clinical implications, and management of spontaneous and iatrogenic hypoglycemia in patients with CF.

PMID:34745906 | PMC:PMC8551648 | DOI:10.1016/j.jcte.2021.100267

BMC Pulm Med. 2021 Nov 8;21(1):353. doi: 10.1186/s12890-021-01728-8.

ABSTRACT

BACKGROUND: High-resolution computed tomography (HRCT) is the gold standard for the evaluation of cystic fibrosis (CF) lung disease; however, lung ultrasound (LUS) is being increasingly used for the assessment of lung in these patients due to its lower cost, availability, and lack of irradiation. We aimed to determine the diagnostic performance of LUS for the evaluation of CF pulmonary exacerbation.

METHODS: This cross-sectional study included patients with CF pulmonary exacerbation admitted to Masih Daneshvari Hospital, Tehran, Iran, from March 21, 2020 to March 20, 2021. Age, gender, and body mass index (BMI) of the patients were recorded. All patients underwent chest X-ray (CXR), HRCT, and LUS on admission. Pleural thickening, atelectasis, air bronchogram, B-line, and consolidation were noted in LUS and then compared with the corresponding findings in CXR and HRCT. Taking HRCT findings as reference, sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and diagnostic accuracy (DA) of LUS and CXR for the detection of each pulmonary abnormality were determined.

RESULTS: Of the 30 patients included in this study, with a mean age of 19.62 ± 5.53 years, 14 (46.7%) were male. Of the 15 patients aged 2-20 years, BMI was below the 5th percentile in 10 (66.7%), within the 5-10 percentiles in 1 (6.7%), 10-25 percentiles in 3 (20%), and 25-50 percentiles in 1 (6.7%). The mean BMI for 15 patients > 20 years was 18.03 ± 2.53 kg/m2. LUS had better diagnostic performance compared to CXR for the detection of air bronchogram, consolidation, and pleural thickening (area under the receiver operating characteristic curve [AUROC]: 0.966 vs. 0.483, 0.900 vs. 0.575, and 0.656 vs. 0.531, respectively). Also, LUS was 100% and 96.7% specific for the diagnosis of pleural effusion and atelectasis, respectively.

CONCLUSIONS: LUS appears to be superior to CXR and comparable with HRCT for the evaluation of CF pulmonary exacerbation, especially in terms of air bronchogram and consolidation detection. LUS can be used to lengthen the HRCT evaluation intervals in this regard or utilized along with HRCT for better evaluation of CF pulmonary exacerbation.

PMID:34743707 | DOI:10.1186/s12890-021-01728-8

Drugs. 2021 Nov 7. doi: 10.1007/s40265-021-01635-6. Online ahead of print.

ABSTRACT

Pseudomonas aeruginosa is a Gram-negative bacterial pathogen that is a common cause of nosocomial infections, particularly pneumonia, infection in immunocompromised hosts, and in those with structural lung disease such as cystic fibrosis. Epidemiological studies have identified increasing trends of antimicrobial resistance, including multi-drug resistant (MDR) isolates in recent years. P. aeruginosa has several virulence mechanisms that increase its ability to cause severe infections, such as secreted toxins, quorum sensing and biofilm formation. Management of P. aeruginosa infections focuses on prevention when possible, obtaining cultures, and prompt initiation of antimicrobial therapy, occasionally with combination therapy depending on the clinical scenario to ensure activity against P. aeruginosa. Newer anti-pseudomonal antibiotics are available and are increasingly being used in the management of MDR P. aeruginosa.

PMID:34743315 | DOI:10.1007/s40265-021-01635-6