Front Med (Lausanne). 2021 Aug 16;8:722124. doi: 10.3389/fmed.2021.722124. eCollection 2021.

ABSTRACT

Most studies have evaluated the impact of non-cystic fibrosis bronchiectasis (hereafter referred to as bronchiectasis) on quality of life (QoL) in patients with chronic obstructive pulmonary disease (COPD) using COPD cohorts. Accordingly, the impact of COPD on QoL in patients with bronchiectasis is not well-elucidated. We used the Korean Multicenter Bronchiectasis Audit and Research Collaboration (KMBARC) registry between August 2018 and December 2019, a prospective observational cohort that enrolled patients with bronchiectasis in Korea. We evaluated co-occurrence exposure to COPD in bronchiectasis patients, and the primary outcome was QoL according to the Bronchiectasis Health Questionnaire (BHQ). We also investigated factors associated with decreased QoL, defined as the lowest quartile of the total BHQ score. Of 598 patients with bronchiectasis, 372 (62.2%) had COPD. Bronchiectasis patients with COPD had a significantly lower total BHQ score compared with those without COPD [median = 63.1 (interquartile range: 54.8-68.6) vs. 64.8 (57.4-70.8), p = 0.020]. Multivariable analysis revealed that dyspnea [adjusted odds ratio (aOR) = 3.21, 95% confidence interval (CI) = 1.21-8.60], depression (aOR = 1.28, 95% CI = 1.16-1.44), and fatigue (aOR = 1.05, 95% CI = 1.01-1.09) were significantly associated with decreased QoL in bronchiectasis patients with COPD. In conclusion, bronchiectasis patients with COPD had significantly decreased QoL than patients without COPD. In bronchiectasis patients with COPD, dyspnea, depression, and fatigue were associated with decreased QoL.

PMID:34490307 | PMC:PMC8418120 | DOI:10.3389/fmed.2021.722124

Front Immunol. 2021 Aug 13;12:714132. doi: 10.3389/fimmu.2021.714132. eCollection 2021.

ABSTRACT

Chronic lung allograft dysfunction (CLAD) is the main cause of poor survival and low quality of life of lung transplanted patients. Several studies have addressed the role of dendritic cells, macrophages, T cells, donor specific as well as anti-HLA antibodies, and interleukins in CLAD, but the expression and function of immune checkpoint molecules has not yet been analyzed, especially in the two CLAD subtypes: BOS (bronchiolitis obliterans syndrome) and RAS (restrictive allograft syndrome). To shed light on this topic, we conducted an observational study on eight consecutive grafts explanted from patients who received lung re-transplantation for CLAD. The expression of a panel of immune molecules (PD1/CD279, PDL1/CD274, CTLA4/CD152, CD4, CD8, hFoxp3, TIGIT, TOX, B-Cell-Specific Activator Protein) was analyzed by immunohistochemistry in these grafts and in six control lungs. Results showed that RAS compared to BOS grafts were characterized by 1) the inversion of the CD4/CD8 ratio; 2) a higher percentage of T lymphocytes expressing the PD-1, PD-L1, and CTLA4 checkpoint molecules; and 3) a significant reduction of exhausted PD-1-expressing T lymphocytes (PD-1pos/TOXpos) and of exhausted Treg (PD-1pos/FOXP3pos) T lymphocytes. Results herein, although being based on a limited number of cases, suggest a role for checkpoint molecules in the development of graft rejection and offer a possible immunological explanation for the worst prognosis of RAS. Our data, which will need to be validated in ampler cohorts of patients, raise the possibility that the evaluation of immune checkpoints during follow-up offers a prognostic advantage in monitoring the onset of rejection, and suggest that the use of compounds that modulate the function of checkpoint molecules could be evaluated in the management of chronic rejection in LTx patients.

PMID:34489963 | PMC:PMC8418069 | DOI:10.3389/fimmu.2021.714132

J Cyst Fibros. 2021 Sep 3:S1569-1993(21)01359-X. doi: 10.1016/j.jcf.2021.08.018. Online ahead of print.

ABSTRACT

A non-consanguineous two-generation family of parent and monozygous twins who all three share the same CFTR mutation genotype p.Phe508del / p.Arg117His, was examined in clinical features, sweat test, nasal potential difference and intestinal current measurements. As expected the twins were very much alike in anthropometry and appearance and shared the clinical manifestation of CFTR dysfunction albeit at different intensity but unexpectedly like in comparison to their mother they were discordant in their CFTR-mediated basic defect and the response thereof to CFTR potentiation by ivacaftor. This case report illustrates the strong impact of non-inherited factors on the electrophysiological phenotype of the most common CFTR mutation genotype of variable clinical significance.

PMID:34489188 | DOI:10.1016/j.jcf.2021.08.018

J Cyst Fibros. 2021 Sep 3:S1569-1993(21)01353-9. doi: 10.1016/j.jcf.2021.08.012. Online ahead of print.

ABSTRACT

OBJECTIVES: Two CFTR-dependent β-adrenergic sweat rate tests applying intradermal drug injections were reported to better define diagnosis and efficacy of CFTR-directed therapies. The aim of this work was to develop and test a needle-free image-based test and to provide an accurate analysis of the responses.

METHODS: The modified method was conducted by applying two successive iontophoresis sessions using the Macroduct device. Efficiency of drug delivery was tested by evaporimetry. Cholinergically stimulated sweating was evoked by pilocarpine iontophoresis. β-adrenergically stimulated sweating was obtained by iontophoresis of isoproterenol and aminophylline in the presence of atropine and ascorbic acid. A nonlinear mixed-effects (NLME) approach was applied to model volumes of sweat and subject-specific effects displaying inter- and intra-subject variability.

RESULTS: Iontophoresis provided successful transdermal delivery of all drugs, including almost neutral isoproterenol and aminophylline. Pilocarpine was used at a concentration ∼130-times lower than that used in the classical Gibson and Cooke sweat test. Addition of ascorbic acid lowered the pH of the solution, made it stable, prevented isoproterenol degradation and promoted drug iontophoresis. Maximal secretory capacity and kinetic rate of β-adrenergic responses were blunted in CF. A cutoff of 5.2 minutes for ET50, the time to reach the half maximal secretion, discriminated CF from controls with a 100% sensitivity and specificity. Heterozygous showed an apparently reduced kinetic rate and a preserved secretory capacity.

CONCLUSION: We tested a safe, well-tolerated needle-free image-based sweat test potentially applicable in children. Modelling responses by NLME allowed evaluating metrics of CFTR-dependent effects reflecting secretory capacity and kinetic rate.

PMID:34489187 | DOI:10.1016/j.jcf.2021.08.012

Mol Genet Metab. 2021 Aug 15:S1096-7192(21)00769-1. doi: 10.1016/j.ymgme.2021.08.005. Online ahead of print.

ABSTRACT

The Colorado Newborn Screening Program (CO-NBS) screens for cystic fibrosis (CF) by measuring immunoreactive trypsinogen (IRT) from two screens coupled with DNA analysis (IRT/IRT/DNA). The Colorado CF Care Center identified 8 missed CF cases among 358,187 infants screened by the CO-NSP since 2016. Retrospective analysis of CO-NSP IRT data shows that a 96th percentile floating IRT cutoff with a 50 ng/mL fixed cutoff on the first screen, and second screen 50 ng/mL fixed cutoff would have identified 7 of the 8 missed cases. These efforts demonstrate the importance of continuous quality improvement in order to increase sensitivity and reduce missed cases.

PMID:34489170 | DOI:10.1016/j.ymgme.2021.08.005

Environ Microbiol. 2021 Sep 6. doi: 10.1111/1462-2920.15745. Online ahead of print.

ABSTRACT

The saprophyte Pseudomonas aeruginosa is a versatile opportunistic pathogen causing infections in immunocompromised individuals. To facilitate its adaptation to a large variety of niches, this bacterium exploits population density-dependent gene regulation systems called quorum sensing (QS). In P. aeruginosa, three distinct but interrelated QS systems (las, rhl and pqs) regulate the production of many survival and virulence functions. In prototypical strains, the las system, through its transcriptional regulator LasR, is important for the full activation of the rhl and pqs systems. Still, LasR-deficient isolates have been reported, mostly sampled from the lungs of people with cystic fibrosis, where they are considered selected by the chronic infection environment. In this study, we show that a defect in LasR activity appears to be an actually widespread mechanism of adaptation in this bacterium. Indeed, we found abundant LasR-defective isolates sampled from hydrocarbon-contaminated soils, hospital sink drains and meat/fish market environments, using an approach based on phenotypic profiling, supported by gene sequencing. Interestingly, several LasR-defective isolates maintain an active rhl system or are deficient in pqs system signalling. The high prevalence of a LasR-defective phenotype among environmental P. aeruginosa isolates questions the role of QS in niche adaptation.

PMID:34488244 | DOI:10.1111/1462-2920.15745

Zhonghua Lao Dong Wei Sheng Zhi Ye Bing Za Zhi. 2021 Aug 20;39(8):635-640. doi: 10.3760/cma.j.cn121094-20200401-00165.

ABSTRACT

Iodine transporters of basement membrane of thyroid follicular epithelial cells can participate and exchange the iodine ions across intracellular and extracellular. Among all of the iodine rich organs, iodine ions which only exist in colloidal of thyroid follicular epithelial cells can be functioned as the raw materials, which after oxidation, iodization and coupling, to synthesize thyroid hormone (TH) and to exert its biological functions. Therefore, the iodine transported function of iodide transporters plays a pivotal role for TH biosynthesis. Furthermore, functional studies show that the abnormal expression or dysfunction of iodide transporters might serves as tumor promoters or inhibitors via regulated the mTOR signal pathway, the MAPKs signal pathway, and the NF-κB signal pathway, together contributed to the regulation of cell proliferation, invasion, metastasis and apoptosis, in which plays the role of non iodide transported function. Therefore, the non iodine transported function of iodide transporters may plays the crucial role of tumor occurrence and progression of carcinoma. Based on this information, present study was devoted to systematic summarize the iodine transported function and non iodine transported function (may affects occurrence and progression of carcinoma) of the classical iodide transporters [sodium iodide symporter (NIS) and pendrin] and novel iodine transporters[ (cystic fibrosis transmembrane conductance regulator (CFTR) , sodium multivitamin transporter (SMVT) , and anoctamin 1 (ANO1) ], respectively, in order to provide a theoretical basis and literature review reference for underlying the mechanism of iodine transporters and its regulated signal pathways for the occurrence and progression of carcinomas.

PMID:34488281 | DOI:10.3760/cma.j.cn121094-20200401-00165

J Ayub Med Coll Abbottabad. 2021 Jul-Sep;33(3):363-367.

ABSTRACT

BACKGROUND: Pulmonary exacerbations (PEx) are major contributor of significant morbidity and mortality in CF patients. Managing PEx needs standardization and without standard local practice guidelines there will be significant variation in practice in managing these children. The aim of this study is to analyse the clinical management of PEx in our setup and to document variation in practices among physicians.

METHODS: Children and adolescents ≤18 years with CF pulmonary exacerbations admitted at high dependency unit (HDU) or wards were included in the study. Frequencies of different intravenous antibiotic combinations were documented along with use of different inhaled antibiotics and inhalation therapy. Practices of different physician were further studied with regards to use of systemic steroids, oral azithromycin and inhaled antibiotics. One way ANOVA was used to assess differences between physicians' practices.

RESULTS: Fifty-seven patients were selected according to the inclusion criteria for 114 different exacerbations. Mean pulmonary exacerbation (PEx) for a patient (events/person-year) over five years was 3.16±1.41 per year and average length of stay was 5.7±4.4 days. Combination of intravenous ceftazidime and amikacin was the most frequently used regimen (28.07%). Five different physicians dealing with majority of the exacerbations (n=74) were studied further. Variability among consultants was significant in using systemic steroids (21.42-92.30%), use of maintenance oral azithromycin (0- 80%) and inhaled antibiotics (0-86.6%).

CONCLUSIONS: Significant variation exists in practices of physicians dealing with CF PEx. Variability observed in our study will definitely provide openings for local CF experts to come up with standardized inpatient exacerbation guidelines.

PMID:34487639

Front Cardiovasc Med. 2021 Aug 18;8:721354. doi: 10.3389/fcvm.2021.721354. eCollection 2021.

ABSTRACT

Background: To provide energy for cardiopulmonary function and maintenance of blood glucose, acute aerobic exercise induces lipolysis, fatty acid oxidation (FAO), glycolysis, and glycogenolysis/gluconeogenesis. These adaptations are mediated by increases in cortisol, growth hormone (GH), and catecholamines and facilitated by a decline in insulin. Branched-chain amino acids (BCAA) also undergo catabolism during intense exercise. Here, we investigated the relationship between BCAA catabolism and metrics of cardiopulmonary function in healthy, well-developed, mature adolescent athletes undergoing an acute bout of maximal aerobic exercise. Hypothesis: We hypothesized: (a) acute maximal exercise in adolescents induces lipolysis, FAO, and BCAA catabolism associated with increases in GH and cortisol and a reduction in insulin; (b) increases in GH are associated with increases in ghrelin; and (c) metrics of cardiopulmonary function (aVO2, rVO2, aVO2/HRmax) following maximal exercise correlate with increases in GH secretion, FAO, and BCAA catabolism. Methods: Blood samples before and after maximal cardiopulmonary exercise in 11 adolescent athletes were analyzed by tandem-mass spectrometry. Paired, two-tailed student's t-tests identified significant changes following exercise. Linear regression determined if pre-exercise metabolite levels, or changes in metabolite levels, were associated with aVO2, rVO2, and aVO2/HRmax. Sex and school of origin were included as covariates in all regression analyses. Results: Following exercise there were increases in GH and cortisol, and decreases in ghrelin, but no changes in glucose or insulin concentrations. Suggesting increased lipolysis and FAO, the levels of glycerol, ketones, β-hydroxybutyrate, and acetylcarnitine concentrations increased. Pyruvate, lactate, alanine, and glutamate concentrations also increased. Plasma concentrations of valine (a BCAA) declined (p = 0.002) while valine degradation byproducts increased in association with decreases in urea cycle amino acids arginine and ornithine. Metrics of cardiopulmonary function were associated with increases in propionylcarnitine (C3, p = 0.013) and Ci4-DC/C4-DC (p < 0.01), byproducts of BCAA catabolism. Conclusions: Induction of lipolysis, FAO, gluconeogenesis, and glycogenolysis provides critical substrates for cardiopulmonary function during exercise. However, none of those pathways were significantly associated with metrics of cardiopulmonary function. The associations between rVO2, and aVO2/HRmax and C3 and Ci4-DC/C4-DC suggest that the cardiopulmonary response to maximal exercise in adolescents is linked to BCAA utilization and catabolism.

PMID:34485418 | PMC:PMC8416443 | DOI:10.3389/fcvm.2021.721354

Front Immunol. 2021 Aug 16;12:691957. doi: 10.3389/fimmu.2021.691957. eCollection 2021.

ABSTRACT

Neutrophil migration into the airways is an important process to fight infection and is mediated by cell adhesion molecules. The intercellular adhesion molecules, ICAM-1 (CD54) and ICAM-2 (CD102) are known ligands for the neutrophil integrins, lymphocyte function associated antigen (LFA)-1 (αLβ2; CD11a/CD18), and macrophage-1 antigen (Mac-1;αMβ2;CD11b/CD18) and are implicated in leukocyte migration into the lung. However, it is ill-defined how neutrophils exit the lung and the role for ICAMs in trans-epithelial migration (TEpM) across the bronchial or alveolar epithelium. We found that human and murine alveolar epithelium expressed ICAM-1, whilst the bronchial epithelium expressed ICAM-2, and both were up-regulated during inflammatory stimulation in vitro and in inflammatory lung diseases such as cystic fibrosis. Although β2 integrins interacting with ICAM-1 and -2 mediated neutrophil migration across human bronchial epithelium in vitro, neither ICAM-2 nor LFA-1 binding of ICAM-1 mediated murine neutrophil migration into the lung or broncho-alveolar space during LPS-induced inflammation in vivo. Furthermore, TEpM of neutrophils themselves resulted in increased epithelial junctional permeability and reduced barrier function in vitro. This suggests that although β2 integrins interacting with ICAMs may regulate low levels of neutrophil traffic in healthy lung or early in inflammation when the epithelial barrier is intact; these interactions may be redundant later in inflammation when epithelial junctions are disrupted and no longer limit TEpM.

PMID:34484188 | PMC:PMC8415445 | DOI:10.3389/fimmu.2021.691957

J Lab Physicians. 2021 Jun;13(2):192-194. doi: 10.1055/s-0041-1730847. Epub 2021 Jun 28.

ABSTRACT

Pandoraea apista is a novel gram-negative bacillus usually isolated from respiratory specimens of cystic fibrosis patients. Few cases of bacteremia have also been reported due to this rare pathogen. Emergence of multidrug-resistant isolates of this bacillus is of grave concern. Here, we report a very interesting and unusual case of Pandoraea apista bacteremia in a coronavirus disease (COVID)-positive elderly diabetic man suffering from pneumonia. Prompt isolation and antibiotic sensitivity testing guided the patient's treatment and yielded favorable outcome. The need of automated methods for identification and sensitivity testing limits the reporting of this rare but important pathogen in hospital settings. Detailed research work and studies are needed in this direction to better understand this pathogen and its clinical manifestations for better patient outcome.

PMID:34483568 | PMC:PMC8409125 | DOI:10.1055/s-0041-1730847

Mol Genet Metab. 2021 Aug 21:S1096-7192(21)00772-1. doi: 10.1016/j.ymgme.2021.08.008. Online ahead of print.

ABSTRACT

Heterozygous (carrier) status for an autosomal recessive condition is traditionally considered to lack significance for an individual's health, but this assumption has been challenged by a growing body of evidence. Carriers of several autosomal recessive disorders and some X-linked diseases are potentially at risk for the pathology manifest in homozygotes. This minireview provides an overview of the literature regarding health risks to carriers of two common autosomal recessive conditions on the Recommended Uniform Screening Panel: sickle cell disease [sickle cell trait (SCT)] and cystic fibrosis (CF). We also consider and comment on bioethical and policy implications for newborn blood screening (NBS). Health risks for heterozygotes, while relatively low for individuals, are often influenced by intrinsic (e.g., other genomic variants or co-morbidities) and extrinsic (environmental) factors, which present opportunities for personalized genomic medicine and risk counseling. They create a special challenge, however, for developing screening/follow-up policies and for genetic counseling, particularly after identification and reporting of heterozygote status through NBS. Although more research is needed, this minireview of the SCT and CF literature to date leads us to propose that blanket terms such as "healthy heterozygotes" or "unaffected carriers" should be superseded in communications about NBS results, in favor of a more nuanced paradigm of setting expectations for health outcomes with "genotype-to-risk." In the molecular era of NBS, it remains clear that public health needs to become better prepared for the full range of applied genetics.

PMID:34483044 | DOI:10.1016/j.ymgme.2021.08.008

Curr Opin Pulm Med. 2021 Sep 1. doi: 10.1097/MCP.0000000000000824. Online ahead of print.

ABSTRACT

PURPOSE OF REVIEW: Liver disease (CFLD) as a complication of cystic fibrosis is recognized as a more severe disease phenotype in both children and adults. We review recent advances in understanding the disease mechanism and consider the implications of new strategies for the diagnosis and management of cystic fibrosis in those with evidence of clinically significant liver disease.

RECENT FINDINGS: Evidence suggests that the prevalence of CFLD has not declined with the introduction of newborn screening. Furthermore, children with CFLD, who have been diagnosed with cystic fibrosis following newborn screening continue to have a much higher mortality rate compared with those with no liver disease. There is further data suggesting noncirrhotic obliterative portal venopathy as the predominant pathological mechanism in the majority of children and young adults receiving a liver transplantation. Little progress has been made in developing an accurate noninvasive test for early diagnosis or monitoring disease progression in CFLD. The benefit of new modulator therapies is not well understood in those with established CFLD, whereas the risk of hepatotoxicity as a complication of treatment must be carefully monitored.

SUMMARY: Better understanding of the pathophysiology of CFLD would allow a standardized approach to diagnosis, with the potential to improve outcomes for those with CFLD.

PMID:34482340 | DOI:10.1097/MCP.0000000000000824

Curr Opin Pulm Med. 2021 Sep 2. doi: 10.1097/MCP.0000000000000828. Online ahead of print.

ABSTRACT

PURPOSE OF REVIEW: Radiological imaging has a crucial role in pulmonary evaluation in cystic fibrosis (CF), having been shown to be more sensitive than pulmonary function testing at detecting structural lung changes. The present review summarizes the latest published information on established and evolving pulmonary imaging techniques for assessing people with this potentially life-limiting disorder.

RECENT FINDINGS: Chest computed tomography (CT) has taken over the predominant role of chest radiography in many centres for the initial assessment and surveillance of CF lung disease. However, several emerging techniques offer a promising means of pulmonary imaging using less ionizing radiation. This is of particular importance given these patients tend to require repeated imaging throughout their lives from a young age. Such techniques include ultra-low-dose CT, tomosynthesis, dynamic radiography and magnetic resonance imaging. In addition, deep-learning algorithms are anticipated to improve diagnostic accuracy.

SUMMARY: The recent introduction of triple-combination CF transmembrane regulator therapy has put further emphasis on the need for sensitive methods of monitoring treatment response to allow for early adaptation of treatment regimens in order to limit irreversible lung damage. Further research is needed to establish how emerging imaging techniques can contribute to this safely and effectively.

PMID:34482339 | DOI:10.1097/MCP.0000000000000828

Indian Pediatr. 2021 Sep 4:S097475591600362. Online ahead of print.

ABSTRACT

OBJECTIVE: To document morbidities in adolescents with cystic fibrosis from India.

METHODS: Details of children with cystic fibrosis surviving beyond 15 years of age were extracted from hospital records, and analyzed.

RESULTS: 43 children [Median (IQR) age 18.7 (17, 20.6) years, were enrolled. Median (IQR) body mass index was 15.82 (13.5, 19.05) kg/m2. Pseudomonas species were isolated from respiratory specimens of 34 (79%) adolescents. Allergic bronchopulmonary aspergillosis (ABPA) and Cystic fibrosis related diabetes (CFRD) were seen in 12 (28%) and 11 (26%) patients, respectively. Conjugated hyperbilirubinemia and distal intestinal obstruction syndrome (DIOS) were diagnosed in 15 (35%) and 6 (14%) children, respectively. Pseudomonas species colonization (P=0.04) and multiple pulmonary exacerbation in last one year (P=0.0002) were significant predictors of FEV1%.

CONCLUSION: Malnutrition, Chronic airway colonization, ABPA, CFRD, conjugated hyperbilirubinemia, DIOS are morbidities observed in adolescents with CF in India. The data support the need for early screening of CF associated morbidities.

PMID:34480466

J Cyst Fibros. 2021 Aug 31:S1569-1993(21)01351-5. doi: 10.1016/j.jcf.2021.08.010. Online ahead of print.

ABSTRACT

Few studies have evaluated clofazimine (CLOF) drug monitoring and safety in children. We treated 10 children, 8 with CF, for NTM infection with multiple antimicrobials, including CLOF. All had serial blood CLOF concentrations measured and were followed for adverse events. Despite CLOF dose escalation, most children with CF did not reach a target CLOF concentration. Our data suggest that children with CF may require earlier initiation of CLOF at higher doses than is currently recommended.

PMID:34479810 | DOI:10.1016/j.jcf.2021.08.010

J Cyst Fibros. 2021 Aug 31:S1569-1993(21)01357-6. doi: 10.1016/j.jcf.2021.08.016. Online ahead of print.

ABSTRACT

BACKGROUND: Despite therapeutic advances, people with cystic fibrosis (CF) develop progressive worsening and exacerbations of their lung disease, which can lead to acute respiratory failure. Historically, survival after mechanical ventilation (MV) has been poor. Outcomes related to use of extracorporeal membrane oxygenation (ECMO) have not been well described in CF.

METHODS: We conducted a retrospective analysis of adult patients with CF admitted to the ICU for acute respiratory failure and requiring invasive MV with or without ECMO between July 1, 2006 and June 30, 2016. Separate analysis for the subgroup of MV patients who were eligible for transplant was conducted.

RESULTS: Mortality for all patients with respiratory failure requiring advanced support was 37%. Ten of 28 (36%) MV patients, 10 of 26 (38%) ECMO+MV patients and 7 of the 21 (33%) transplant eligible MV patients died. Intensive care unit (ICU) length of stay (LOS) was 24.5±16.6 days for ECMO+MV; 12.9±9.0 days for MV (p=0.001), and 12.3 ±10 days for transplant eligible MV patients (p=0.005 for ECMO+MV comparison). Seven transplant eligible MV patients (33%) and 16 ECMO+MV patients (62%) underwent lung transplantation (p<0.001) during the hospital admission. One and 2-year survival for individuals who survived ICU admission was similar regardless of mode of support. Cox-proportional hazards model did not yield any variables that significantly influenced ICU mortality, 1-year or 2-year mortality.

CONCLUSION: Survival for CF patients with acute respiratory failure requiring MV with or without ECMO has improved over time. ECMO may be an appropriate modality for respiratory support in patients with CF and acute respiratory failure who have greater risk of death from MV alone.

PMID:34479809 | DOI:10.1016/j.jcf.2021.08.016

J Cardiothorac Vasc Anesth. 2021 Jul 30:S1053-0770(21)00622-4. doi: 10.1053/j.jvca.2021.07.041. Online ahead of print.

ABSTRACT

OBJECTIVE: Data on chronic pain after lung transplantation are heterogeneous. This study prospectively explored the prevalence, characteristics, consequences, and preoperative predictors of pain in lung transplant recipients.

DESIGN: A prospective cohort study.

SETTING: The Foch University Hospital, Suresnes, France.

PARTICIPANTS: Patients registered on the waiting list for double-lung transplantation in the authors' institution from August 2008 to October 2013 and transplanted.

INTERVENTIONS: Database prospectively completed in real time during consultations with a pain-certified anesthesiologist before lung transplantation and six months after surgery.

MEASUREMENTS AND MAIN RESULTS: The assessments explored pain in three components: physical (intensity, location, neuropathic and sensory qualifications, treatments), mental (anxiety and depression), and quality of life. Seventy-two patients underwent all assessments. The prevalence of six-month postoperative pain was 68.0%. Among patients with pain, 83.3% reported mild average pain and 26.5% had neuropathic pain. All patients who responded to the questionnaire took analgesics frequently, but only 9.1% took opioids. Patients with pain reported higher levels of anxiety (p = 0.02) and depression (p = 0.01). Additionally, they presented with increased difficulty in ambulation (p = 0.03), work (p = 0.02), and sleep (p = 0.02). The maximum level of preoperative pain was an independent risk factor of six-month postoperative pain (p = 0.03).

CONCLUSIONS: The authors report a high prevalence of chronic pain with concomitant psychosocial repercussions despite a reported mild intensity. Perioperative measures, such as personalized and detailed management plans, could improve patient satisfaction.

PMID:34479783 | DOI:10.1053/j.jvca.2021.07.041

J Natl Med Assoc. 2021 Aug 31:S0027-9684(21)00190-5. doi: 10.1016/j.jnma.2021.08.038. Online ahead of print.

ABSTRACT

INTRODUCTION: Cystic fibrosis (CF), an inherited autosomal recessive disease that results in the accumulation of mucus and damage primarily to the respiratory and digestive tracts is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. In the United States, it has been estimated that CF occurs in 1 out of 3500 infants. The objective of this study was to explore the patient and hospital characteristics associated with CF hospitalizations and inpatient mortality in pediatric CF patients.

METHODS: Utilizing the National Inpatient Sample database from 2008 to 2017, a retrospective cohort study was conducted to analyze the hospitalization rates, associated factors, and the inpatient mortality of CF patients 0-17 years of age. Hospitalizations with a diagnosis of CF were identified with ICD-9-CM and ICD-10-CM codes. Adjusted survey logistic regression models were utilized to determine factors associated with CF hospitalizations and in-hospital deaths in CF patients.

RESULTS: There were a total of 98,660 (about 0.2%) CF hospitalizations in patients 17 years of age or younger during the study period. Non-Hispanic (NH) White CF patients had the highest prevalence of CF (26.30 per 10,000 hospitalizations). The prevalence of inpatient deaths were highest among those identified as NH-Others and NH-Blacks (71.35 and 68.83 per 10,000 CF hospitalizations, respectively. When compared with NH-White category, those belonging to NH-Black, Hispanic and Other racial/ethnic sub-group had reduced odds of being hospitalized with CF.

DISCUSSION: Despite our finding of an increased likelihood of being hospitalized for CF among NH-White and male pediatric patients, no association between race or sex and CF inpatient death was observed when adjusted for covariates. More research is needed to determine the impact of sex and race on CF mortality rates.

PMID:34479733 | DOI:10.1016/j.jnma.2021.08.038

Mol Med. 2021 Sep 3;27(1):98. doi: 10.1186/s10020-021-00354-8.

NO ABSTRACT

PMID:34479472 | DOI:10.1186/s10020-021-00354-8