BMC Gastroenterol. 2024 Mar 14;24(1):101. doi: 10.1186/s12876-024-03188-w.

ABSTRACT

Exocrine pancreatic insufficiency (EPI) stems from a deficiency of functional pancreatic enzymes with consequent maldigestion and malnutrition. EPI shares clinical symptoms and manifestations with other disorders and is a considerable burden to individuals affected. In this narrative review, we analyzed the literature to identify relevant publications on living with EPI with the scope of individuating evidence gaps, including those related to symptoms, health-related quality of life (HRQoL), emotional functioning, disease burden, presence of comorbidities, and the use of pancreatic enzyme replacement therapy (PERT). Abdominal pain emerged as one of the most prominent symptoms. HRQoL was affected in EPI, but no articles examined emotional functioning. Comorbidities reported involved other pancreatic disorders, diabetes, gastrointestinal disorders, sarcopenia and osteopenia, cardiovascular disorders, bacterial overgrowth, and nutritional deficiencies. PERT was found to be effective in improving EPI symptoms and was well tolerated by most individuals. Our review revealed a dearth of literature evidence on patients' experience with EPI, such as emotional functioning and disease burden. We also revealed that studies on long-term effects of PERT are missing, as are studies that would help advance the understanding of the disease and its progression, risk/mitigating factors, and comorbidities. Future studies should address these identified gaps.

PMID:38481137 | PMC:PMC10938721 | DOI:10.1186/s12876-024-03188-w

Curr Pharm Biotechnol. 2024 Mar 11. doi: 10.2174/0113892010277107240227054933. Online ahead of print.

ABSTRACT

Mycobacterium tuberculosis, Mycobacterium leprae, and non-tuberculous mycobacteria (NTM) are among the most significant human pathogens within the Mycobacterium genus. These pathogens can infect people who come into contact with biomaterials or have chronic illnesses. A characteristic pathogenic trait of mycobacteria is the development of biofilms, which involves several molecules, such as the GroEL1 chaperone, glycopeptidolipids, and shorter-chain mycolic acids. Bacterial behavior is influenced by nutrients, ions, and carbon sources, which also play a regulatory role in biofilm development. Compared to their planktonic phase, mycobacterial biofilms are more resilient to environmental stresses and disinfectants. Mycobacteria that produce biofilms have been found in several environmental studies, particularly in water systems. NTM can cause respiratory problems in individuals with underlying illnesses such as cystic fibrosis, bronchiectasis, and old tuberculosis scars. Mycobacteria that grow slowly, like those in the Mycobacterium avium complex (MAC), or rapidly, like Mycobacterium abscessus, can be pathogens. Infections related to biomaterials represent a significant category of biofilm-associated infections, with rapidly growing mycobacteria being the most frequently identified organisms. A biofilm produced by M. tuberculosis can contribute to caseous necrosis and cavity formation in lung tissue. Additionally, M. tuberculosis forms biofilms on clinical biomaterials. Biofilm formation is a major contributor to antimicrobial resistance, providing defense against drugs that would typically be effective against these bacteria in their planktonic state. The antibiotic resistance of biofilm-forming microbes may render therapy ineffective, necessitating the physical removal of biofilms to cure the infection. Recently, new approaches have been developed with potential anti-biofilm compounds to increase treatment effectiveness. Understanding biofilms is crucial for the appropriate treatment of various NTM diseases, and the recent discovery of M. tuberculosis biofilms has opened up a new field of study. This review focuses on the biofilm formation of the Mycobacterial genus, the mechanisms of biofilm formation, and anti-mycobacterial biofilm agents.

PMID:38485676 | DOI:10.2174/0113892010277107240227054933

J Cyst Fibros. 2024 Mar 13:S1569-1993(24)00035-3. doi: 10.1016/j.jcf.2024.03.007. Online ahead of print.

ABSTRACT

BACKGROUND: To address sexual and reproductive health (SRH) concerns among people with cystic fibrosis(PwCF), the CF Foundation created the Sexual Health, Reproduction, and Gender Research (SHARING) Working Group. This report summarizes CF community SRH research priorities and workshop discussions/future study planning.

METHODS: Pre-workshop, we distributed a community prioritization survey on CF SRH research/care. During the workshop, we used results and reviewed existing research to establish research priorities and design studies to address identified knowledge gaps.

RESULTS: A total of 303 respondents (85 % PwCF, 15 % caregivers) completed the survey. Highly-rated SRH topics were: 1) effects of CF modulator therapy on sex hormones; 2) effects of sex hormones on CF; 3) fertility; 4) pregnancy; and 5) SRH/mental health. Twenty-four workshop participants established the need for further research on sex hormones and CF, optimizing SRH care provision, and fertility/ART.

CONCLUSION: SRH is an important and emerging area in CF and thoughtful consideration of community perspectives can ensure that future research is relevant and responsive.

PMID:38485603 | DOI:10.1016/j.jcf.2024.03.007

J Cyst Fibros. 2024 Mar 14:S1569-1993(24)00039-0. doi: 10.1016/j.jcf.2024.03.009. Online ahead of print.

ABSTRACT

BACKGROUND: Past and ongoing advancements in cystic fibrosis (CF) care warrant long-term analysis of the societal impact of the condition. This study aims to evaluate changes in key socioeconomic factors across three decades among people living with CF (pwCF), compared with both the general population and an early-onset chronic disease population.

METHODS: This nationwide, registry-based, matched cohort study included all pwCF ≥ 18 years in Denmark in the years 1990, 2000, 2010, and 2018. Each person living with CF was matched to five individuals in the general population and five individuals living with type 1 diabetes or juvenile arthritis based on age, sex, and municipality.

RESULTS: The Danish adult CF population increased nearly fourfold from 88 in 1990 to 331 in 2018, and mean age increased by ten years. The educational level of pwCF was similar to the two comparator cohorts, while pwCF were less often in employment and more often permanently outside the labor force. Personal and household income levels of the CF cohort were higher than those of the comparator cohorts.

CONCLUSIONS: The disadvantage in employment for pwCF remained, but, over time, the societal profiles of the one-year CF cohorts increasingly converged with those of the comparator cohorts, indicative of improved clinical management, extended life expectancy, and the supportive role of the Danish welfare system in reducing health inequalities. Further research should be done to evaluate the effects of the newly introduced modulator therapies on employment, considering the broader societal impact and impact on quality of life.

PMID:38485602 | DOI:10.1016/j.jcf.2024.03.009

Allergol Int. 2024 Mar 13:S1323-8930(24)00018-2. doi: 10.1016/j.alit.2024.03.001. Online ahead of print.

ABSTRACT

Airway mucus is a hydrogel with unique biophysical properties due to its primary water composition and a small proportion of large anionic glycoproteins or mucins. The predominant mucins in human mucus, MUC5AC and MUC5B, are secreted by specialized cells within the airway epithelium both in normal conditions and in response to various stimuli. Their relative proportions are correlated with specific inflammatory responses and disease mechanisms. The dysregulation of mucin expression is implicated in numerous respiratory diseases, including asthma, COPD, and cystic fibrosis, where the pathogenic role of mucus has been extensively described yet often overlooked. In airway diseases, excessive mucus production or impaired mucus clearance leads to mucus plugging, with secondary airway occlusion that contribute to airflow obstruction, asthma severity and poor control. Eosinophils and Charcot Leyden crystals in sputum contribute to the mucus burden and tenacity. Mucin may also contribute to eosinophil survival. Other mechanisms, including eosinophil-independent IL-13 release, mast-cell activation and non-type-2 (T2) cytokines, are also likely to participate in mucus pathobiology. An accurate assessment of mucus and its clinical and functional consequences require a thorough approach that includes evaluation of cellular predominance in sputum, airway cytokines and other inflammatory markers, mucus characteristics and composition and structural and functional impact measured by advanced lung imaging. This review, illustrated with clinical scenarios, provides an overview of current methods to assess mucus and its relevance to the choice of biologics to treat patients with severe asthma.

PMID:38485545 | DOI:10.1016/j.alit.2024.03.001

Eur Respir J. 2024 Mar 14:2302290. doi: 10.1183/13993003.02290-2023. Online ahead of print.

ABSTRACT

BACKGROUND AND AIM: : In cystic fibrosis (CF), gastrointestinal dysfunction and lower airway infection occur early and are independently associated with poorer outcomes in childhood. This study aimed to define the relationship between the microbiota at each niche during the first 2-years of life, its association with growth and airway inflammation, and explanatory features in the metabolome.

MATERIALS AND METHODS: Sixty-seven bronchoalveolar lavage (BAL), 62 plasma and 105 stool samples were collected from 39 infants with CF between 0-24-months who were treated with prophylactic antibiotics. 16S rRNA amplicon and shotgun metagenomic sequencing were performed on BAL and stool respectively; metabolomic analyses were performed on all sample types. Sequencing data from healthy age-matched infants were used as controls.

RESULTS: Bacterial diversity increased over the first 2-years in both BAL and stool, and microbial maturation was delayed in comparison to healthy controls from the RESONANCE cohort. Correlations between their respective abundances in both sites suggest stool may serve as a non-invasive alternative for detecting BAL Pseudomonas and Veillonella. Multi-site metabolomic analyses revealed age- and growth-related changes, associations with neutrophilic airway inflammation, and a set of core systemic metabolites. BAL Pseudomonas abundance was correlated with altered stool microbiome composition and systemic metabolite alterations, highlighting a complex gut-plasma-lung interplay and new targets with therapeutic potential.

CONCLUSION: Exploration of the gut-lung microbiome and metabolome reveals diverse multi-site interactions in CF that emerge in early life. Gut-lung metabolomic links with airway inflammation and Pseudomonas abundance warrant further investigation for clinical utility, particularly in non-expectorating patients.

PMID:38485151 | DOI:10.1183/13993003.02290-2023

Eur Respir J. 2024 Mar 14:2400156. doi: 10.1183/13993003.00156-2024. Online ahead of print.

NO ABSTRACT

PMID:38485147 | DOI:10.1183/13993003.00156-2024

ACS Appl Mater Interfaces. 2024 Mar 14. doi: 10.1021/acsami.3c18656. Online ahead of print.

ABSTRACT

Pseudomonas aeruginosa biofilms comprise three main polysaccharides: alginate, psl, and pel, which all imbue tolerance against exogenous antimicrobials. Nanoparticles (NPs) are an exciting new strategy to overcome the biofilm matrix for therapeutic delivery applications; however, zero existing FDA approvals for biofilm-specific NP formulations can be attributed to the complex interplay of physiochemical forces at the biofilm-NP interface. Here, we leverage a set of inducible, polysaccharide-specific, expressing isogenic P. aeruginosa mutants coupled with an assembled layer-by-layer NP (LbL NP) panel to characterize biofilm-NP interactions. When investigating these interactions using confocal microscopy, alginate-layered NPs associated more than dextran-sulfate-layered NPs with biofilms that had increased alginate production, including biofilms produced by mucoid P. aeruginosa isolates from people with cystic fibrosis. These differences were further confirmed in LbL NPs layered with polysaccharide- or hydrocarbon-based polymers with pendent carboxylate or sulfate functional groups. These data suggest carboxylated NP surfaces have enhanced interactions specifically with mucoid biofilms as compared to sulfated surfaces and lay the foundation for their inclusion as a design element for increasing biofilm-NP interactions and efficacious drug delivery.

PMID:38484043 | DOI:10.1021/acsami.3c18656

Arch Microbiol. 2024 Mar 14;206(4):159. doi: 10.1007/s00203-024-03921-9.

ABSTRACT

Burkholderia cepacia complex (BCC) is a Gram-negative, non-spore-forming bacterium with more than 20 opportunistic pathogenic species, most commonly found in soil and water. Due to their rapid mutation rates, these organisms are adaptable and possess high genomic plasticity. BCC can cause life-threatening infections in immunocompromised individuals, such as those with cystic fibrosis, chronic granulomatous disease, and neonates. BCC contamination is a significant concern in pharmaceutical manufacturing, frequently causing non-sterile product recalls. BCC has been found in purified water, cosmetics, household items, and even ultrasound gel used in veterinary practices. Pharmaceuticals, personal care products, and cleaning solutions have been implicated in numerous outbreaks worldwide, highlighting the risks associated with intrinsic manufacturing site contamination. Regulatory compliance, product safety, and human health protection depend on testing for BCC in pharmaceutical manufacturing. Identification challenges exist, with BCC often misidentified as other bacteria like non-lactose fermenting Escherichia coli or Pseudomonas spp., particularly in developing countries where reporting BCC in pharmaceuticals remains limited. This review comprehensively aims to address the organisms causing BCC contamination, genetic diversity, identification challenges, regulatory requirements, and mitigation strategies. Recommendations are proposed to aid pharmaceutical chemists in managing BCC-associated risks and implementing prevention strategies within manufacturing processes.

PMID:38483625 | DOI:10.1007/s00203-024-03921-9

Eur J Clin Pharmacol. 2024 Mar 14. doi: 10.1007/s00228-024-03666-w. Online ahead of print.

ABSTRACT

BACKGROUND AND OBJECTIVES: Despite being clinically utilized for the treatment of infections, the limited therapeutic range of polymyxin B (PMB), along with considerable interpatient variability in its pharmacokinetics and frequent occurrence of acute kidney injury, has significantly hindered its widespread utilization. Recent research on the population pharmacokinetics of PMB has provided valuable insights. This study aims to review relevant literature to establish a theoretical foundation for individualized clinical management.

METHODS: Follow PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines, Pop-PK studies of PMB were searched in PubMed and EMBASE database systems from the inception of the database until March 2023.

RESULT: To date, a total of 22 population-based studies have been conducted, encompassing 756 subjects across six different countries. The recruited population in these studies consisted of critically infected individuals with multidrug-resistant bacteria, patients with varying renal functions, those with cystic fibrosis, kidney or lung transplant recipients, patients undergoing extracorporeal membrane oxygenation (ECMO) or continuous renal replacement therapy (CRRT), as well as individuals with obesity or pediatric populations. Among these studies, seven employed a one-compartmental model, with the range of typical clearance (CL) and volume (Vc) being 1.18-2.5L /h and 12.09-47.2 L, respectively. Fifteen studies employed a two-compartmental model, with the ranges of the clearance (CL) and volume of the central compartment (Vc), the volume of the peripheral compartment (Vp), and the intercompartment clearance (Q) were 1.27-8.65 L/h, 5.47-38.6 L, 4.52-174.69 L, and 1.34-24.3 L/h, respectively. Primary covariates identified in these studies included creatinine clearance and body weight, while other covariates considered were CRRT, albumin, age, and SOFA scores. Internal evaluation was conducted in 19 studies, with only one study being externally validated using an independent external dataset.

CONCLUSION: We conclude that small sample sizes, lack of multicentre collaboration, and patient homogeneity are the primary reasons for the discrepancies in the results of the current studies. In addition, most of the studies limited in the internal evaluation, which confined the implementation of model-informed precision dosing strategies.

PMID:38483544 | DOI:10.1007/s00228-024-03666-w

Breathe (Sheff). 2024 Mar;20(1):230126. doi: 10.1183/20734735.0126-2023. Epub 2024 Mar 12.

ABSTRACT

Primary idiopathic hypereosinophilic syndrome is a rare condition that can cause end-organ damage in multiple systems. The advent of targeted monoclonal antibodies, such as mepolizumab, provides a safe and effective steroid-sparing treatment. https://bit.ly/4bgDP1u.

PMID:38482189 | PMC:PMC10928552 | DOI:10.1183/20734735.0126-2023

Commun Biol. 2024 Mar 13;7(1):319. doi: 10.1038/s42003-024-05966-4.

ABSTRACT

Epithelial ion and fluid transport studies in patient-derived organoids (PDOs) are increasingly being used for preclinical studies, drug development and precision medicine applications. Epithelial fluid transport properties in PDOs can be measured through visual changes in organoid (lumen) size. Such organoid phenotypes have been highly instrumental for the studying of diseases, including cystic fibrosis (CF), which is characterized by genetic mutations of the CF transmembrane conductance regulator (CFTR) ion channel. Here we present OrgaSegment, a MASK-RCNN based deep-learning segmentation model allowing for the segmentation of individual intestinal PDO structures from bright-field images. OrgaSegment recognizes spherical structures in addition to the oddly-shaped organoids that are a hallmark of CF organoids and can be used in organoid swelling assays, including the new drug-induced swelling assay that we show here. OrgaSegment enabled easy quantification of organoid swelling and could discriminate between organoids with different CFTR mutations, as well as measure responses to CFTR modulating drugs. The easy-to-apply label-free segmentation tool can help to study CFTR-based fluid secretion and possibly other epithelial ion transport mechanisms in organoids.

PMID:38480810 | DOI:10.1038/s42003-024-05966-4

J Cyst Fibros. 2024 Mar 12:S1569-1993(24)00032-8. doi: 10.1016/j.jcf.2024.03.004. Online ahead of print.

ABSTRACT

Antibiotics are frequently utilized for cystic fibrosis (CF)-related pulmonary exacerbation treatment. The antibiotic spectrum index (ASI) is an antimicrobial stewardship tool developed to compare the relative breadth of individual antibiotics. This study aimed to create two expanded CF-specific ASI scoring indices for use in antimicrobial stewardship research and clinical care. The first scoring index expanded the original ASI to include bacterial microorganisms common to CF airway infections (CF-ASI). The second scoring system only included scores for bacterial microorganisms classically identified in CF airway infections (CF-sASI). Sixty-two antibiotics were evaluated and included in the updated ASIs. When multiple antibiotics are prescribed, we proposed using an additive ASI approach whereby the sum of the individual prescribed antibiotic scores represents the total ASI score. The application of CF-focused ASIs into CF research and stewardship programs can help to optimize antibiotic benefits, minimize harms and allow for increased sustainability of antibiotic use in CF.

PMID:38480113 | DOI:10.1016/j.jcf.2024.03.004

J Cyst Fibros. 2024 Mar 12:S1569-1993(24)00031-6. doi: 10.1016/j.jcf.2024.03.001. Online ahead of print.

ABSTRACT

BACKGROUND: We studied the health care resource utilization (HCRU) and associated costs in the year preceding LT in pwCF or death without LT, and we estimated the overall cost of LT.

METHODS: We performed a linkage between 2006 and 2017 data from the French CF Registry (FCFR) and the French health claims database (Système National des Données de Santé; SNDS). The HCRU and associated costs were described the year before LT or before death without LT, and two years after LT.

RESULTS: Among the 7,671 patients included in the FCFR, 6,187 patients (80.7 %) were successfully matched to patients in the SNDS (males (m): 51.9 %, mean±SD age at the end of follow-up: 24.6 ± 13.6). Overall, 166 patients died without LT (m: 47.6 %, age at death: 30.4 ± 14.5) and 767 patients with primary LT (m: 48.2 %, age at transplantation: 28.0 ± 9.1) were identified. HCRU was lower among patients who died without receiving LT, with marked differences in the cost of hospital stays. The mean total cost per patient was €66,759 ± 38,249 in the year before death, €149,374 ± 62,678 in the year preceding LT, €63,919 ± 35,399 in the first year following LT, and €42,813 ± 39,967 in the second year of follow-up.

CONCLUSION: Our results indicate that HCRU was two times lower in the year before death in non-transplant pwCF than in the year before LT, which may reflect inappropriate care of CF in patients who died without receiving LT. It also shows the cost associated with LT.

PMID:38480112 | DOI:10.1016/j.jcf.2024.03.001

Clin Nutr ESPEN. 2024 Apr;60:139-145. doi: 10.1016/j.clnesp.2024.01.009. Epub 2024 Jan 17.

ABSTRACT

OBJECTIVE: Evaluate the influence of the BsmI polymorphism of the vitamin D receptor gene on vitamin D levels, and inflammatory and oxidative stress markers in patients with Cystic Fibrosis supplemented with cholecalciferol megadose.

METHODS: We performed a single-arm, non-randomized pre- and post-study of 17 patients aged 5 to 20 years with cystic fibrosis diagnosed with vitamin D insufficiency/deficiency 25-hydroxy vitamin< 30 ng/mL. Individuals were genotyped for the BsmI polymorphism of the vitamin D receptor gene and all received cholecalciferol supplementation of 4,000 IU daily for children aged 5 to 10 years and 10,000 IU for children over 10 years of age for 8 weeks. Interviews were conducted with personal data, sun exposure, anthropometric and blood samples of 25-hydroxy vitamin parathormone, serum calcium, ultrasensitive C-reactive protein, alpha 1 acid glycoprotein, total antioxidant capacity, malondialdehyde and kidney and liver function. Inter- and intra-group assessment was assessed by paired t-test Anova test or its non-parametric counterparts.

RESULTS: The individuals were mostly male and reported no adverse effects from the use of supplementation, 64 % had 25-hydroxy vitamin levels >30 ng/mL. Patients with BB and Bb genotypes showed increased serum levels of 25-hydroxy vitamin. The group with BB genotype showed a reduction in alpha 1 acid glycoprotein. And individuals with the bb genotype had high levels of malondialdehyde compared to the pre-intervention time.

CONCLUSION: It is concluded that variations of the BsmI polymorphism of the vitamin D receptor gene have different responses in vitamin D levels and markers of inflammation and oxidative stress.

PMID:38479902 | DOI:10.1016/j.clnesp.2024.01.009

BMJ Open Respir Res. 2024 Mar 13;11(1):e002161. doi: 10.1136/bmjresp-2023-002161.

ABSTRACT

OBJECTIVES: Vasoactive drugs have exhibited clinical efficacy in addressing pulmonary arterial hypertension, manifesting a significant reduction in morbidity and mortality. Pulmonary hypertension may complicate advanced interstitial lung disease (PH-ILD) and is associated with high rates of disability, hospitalisation due to cardiac and respiratory illnesses, and mortality. Prior management hinged on treating the underlying lung disease and comorbidities. However, the INCREASE trial of inhaled treprostinil in PH-ILD has demonstrated that PH-ILD can be effectively treated with vasoactive drugs.

METHODS: This comprehensive systematic review examines the evidence for vasoactive drugs in the management of PH-ILD.

RESULTS: A total of 1442 pubblications were screened, 11 RCTs were considered for quantitative synthesis. Unfortunately, the salient studies are limited by population heterogeneity, short-term follow-up and the selection of outcomes with uncertain clinical significance.

CONCLUSIONS: This systematic review underscores the necessity of establishing a precision medicine-oriented strategy, directed at uncovering and addressing the intricate cellular and molecular mechanisms that underlie the pathophysiology of PH-ILD.

PROSPERO REGISTRATION NUMBER: CRD42023457482.

PMID:38479818 | DOI:10.1136/bmjresp-2023-002161

Res Q Exerc Sport. 2024 Mar 13:1-9. doi: 10.1080/02701367.2024.2320234. Online ahead of print.

ABSTRACT

Purpose: Maintaining physical fitness plays an important role in the management of people with cystic fibrosis (pwCF). Longitudinal data on the course of physical fitness and the potential impact of the introduction of highly effective CFTR modulator therapy with elexacaftor/tezacaftor/ivacaftor (ETI) in adult pwCF are scarce. Methods: Health-related and skill-related components of physical fitness were assessed using an incremental cycle test (Wpeak), plus forward bend (FB), prone bent knee hip extension (HE), plank leg raise (PLR), standing long jump (SLJ), and standing on one leg (OLS). Relevant disease-specific clinical data (body mass index [BMI] and forced expiratory volume in 1 second [FEV1]) were recorded. Results: Twenty-eight adult pwCF (age 26.0 ± 7.8 years) were followed over 5.6 ± 0.9 years; 21 started ETI therapy during this period. Significant improvements from baseline were noted in BMI (p < 0.001) and health-related fitness components (HE, p = 0.002; PLR, p = < 0.001), whereas Wpeak and FB remained stable over time (all p > 0.05). Skill-related components (SLJ, OLS) showed no change (all p > 0.05). Subgroup analysis revealed significant improvements in BMI, FEV1, and health-related fitness measures of muscular strength and endurance (HE, p = 0.009; PLR, p < 0.001) only in pwCF using ETI. Conclusion: Despite the improvements, the impact of ETI on the individual parameters was small. Other factors than implementation of ETI alone need to be considered on the way to a high level of physical fitness in adult pwCF.

PMID:38478996 | DOI:10.1080/02701367.2024.2320234

Pediatr Pulmonol. 2024 Mar 13. doi: 10.1002/ppul.26950. Online ahead of print.

ABSTRACT

BACKGROUND AND OBJECTIVES: There are limited data on cystic fibrosis (CF) transmembrane conductance regulator-related metabolic syndrome (CRMS) outcomes beyond infancy. The goal of this study was to analyze outcomes of infants with CRMS up to the age of 9-10 years using the CF Foundation Patient Registry (CFFPR).

METHODS: We analyzed data from the CFFPR for individuals with CF and CRMS born between 2010 and 2020. We classified all patients based on the clinical diagnosis reported by the CF care center and the diagnosis using CFF guideline definitions for CF and CRMS, classifying children into groups based on agreement between clinical report and guideline criteria. Descriptive statistics for the cohort were calculated for demographics, nutritional outcomes, and microbiology for the first year of life and lung function and growth outcomes were summarized for ages 6-10 years.

RESULTS: From 2010 to 2020, there were 8765 children with diagnosis of CF or CRMS entered into the CFFPR with sufficient diagnostic data for classification, of which 7591 children had a clinical diagnosis of CF and 1174 had a clinical diagnosis of CRMS. CRMS patients exhibited normal nutritional indices and pulmonary function up to age 9-10 years. The presence of respiratory bacteria associated with CF, such as Pseudomonas aeruginosa from CRMS patients ranged from 2.1% to 9.1% after the first year of life.

CONCLUSIONS: Children with CRMS demonstrate normal pulmonary and nutritional outcomes into school age. However, a small percentage of children continue to culture CF-associated respiratory pathogens after infancy.

PMID:38477633 | DOI:10.1002/ppul.26950

Soc Work Health Care. 2024 Mar 13:1-21. doi: 10.1080/00981389.2024.2324858. Online ahead of print.

ABSTRACT

This cross-sectional quantitative study was conducted to evaluate the coping strategies of parents of children with cystic fibrosis. The research sample is the parents (n: 112) who presented to Thoracic Medicine Department at Hacettepe University Pediatric Hospital between 3 April 2021 - 28 May 2021 and volunteered to participate in the study. Sociodemographic Data Form and Coping Orientation to Problems Experienced Inventory (COPE Inventory) were used in the collection of data. The study examined coping strategies according to children's characteristics such as age, sex, education, and parents' independent variables such as employment status, income status, number of individuals and children in the family, communication with other families, social and financial support. Data were analyzed using Mann-Whitney and Kruskal-Wallis tests. Research findings show that religious coping was the most frequently preferred coping strategy, and behavioral disengagement was the least commonly used coping strategy. Emotion-Focused Coping Strategies were also commonly used. Social work interventions and strategies play an important role in helping parents to adopt positive coping strategies and improve their skills.

PMID:38477619 | DOI:10.1080/00981389.2024.2324858

Cell Stress. 2024 Mar 12;8:21-50. doi: 10.15698/cst2024.03.294. eCollection 2024.

ABSTRACT

The eight biological hallmarks of health that we initially postulated (Cell. 2021 Jan 7;184(1):33-63) include features of spatial compartmentalization (integrity of barriers, containment of local perturbations), maintenance of homeostasis over time (recycling & turnover, integration of circuitries, rhythmic oscillations) and an array of adequate responses to stress (homeostatic resilience, hormetic regulation, repair & regeneration). These hallmarks affect all eight somatic strata of the human body (molecules, organelles, cells, supracellular units, organs, organ systems, systemic circuitries and meta-organism). Here we postulate that mental and socioeconomic factors must be added to this 8×8 matrix as an additional hallmark of health ("psychosocial adaptation") and as an additional stratum ("psychosocial interactions"), hence building a 9×9 matrix. Potentially, perturbation of each of the somatic hallmarks and strata affects psychosocial factors and vice versa. Finally, we discuss the (patho)physiological bases of these interactions and their implications for mental health improvement.

PMID:38476764 | PMC:PMC10928495 | DOI:10.15698/cst2024.03.294