Hum Gene Ther. 2021 Aug 18. doi: 10.1089/hum.2021.008. Online ahead of print.

ABSTRACT

Silicosis is an occupational disease caused by inhalation of silica dust, which is hallmarked by progressive pulmonary fibrosis associated with poor prognosis. Wnt/β-catenin signaling is implicated in the development of fibrosis and is a therapeutic target for fibrotic diseases. Previous clinical studies of patients with pneumoconioses including silicosis revealed an increased concentration of circulating WNT3A and DKK1 proteins and inflammatory cells in bronchoalveolar lavage compared to healthy subjects. The present study evaluated the effects of adenovirus-mediated transduction of Dickkopf-1 (Dkk1), a Wnt/β-catenin signaling inhibitor, on the development of pulmonary silicosis in mice. Consistent with previous human clinical studies, our experimental studies in mice demonstrated an aberrant Wnt/β-catenin signaling activity coinciding with increased Wnt3a and Dkk1 proteins and inflammation in lungs of silica-induced silicosis mice compared to controls. Intratracheal delivery of adenovirus expressing murine Dkk1 (AdDkk1) inhibited Wnt/β-catenin activity in mouse lungs. The Adenovirus-mediated Dkk1 gene transduction demonstrated potentials to prevent silicosis development and ameliorate silica-induced lung fibrogenesis in mice, companied with the reduced expression of epithelial-mesenchymal transition (EMT) markers and deposition of extracellular matrix (ECM) proteins compared to mice treated with "null" adenoviral vector. Mechanistically, AdDkk1 is able to attenuate the lung silicosis by inhibiting a silica-induced spike in TGF-β/Smad signaling. Additionally, the forced expression of Dkk1 suppressed silica-induced epithelial cell proliferation in polarized human bronchial epithelial cells. This study provides insight into the underlying role of Wnt/β-catenin signaling in promoting the pathogenesis of silicosis and is proof-of-concept that targeting Wnt/β-catenin signaling by Dkk1 gene transduction may be an alternative approach in prevention and treatment of silicosis lung disease.

PMID:34405699 | DOI:10.1089/hum.2021.008

EClinicalMedicine. 2021 Aug 13:101079. doi: 10.1016/j.eclinm.2021.101079. Online ahead of print.

ABSTRACT

BACKGROUND: The coronavirus disease 2019 (COVID-19) has been identified in over 110 million people with no studies comparing pre-infection pulmonary function to post-infection. This study's aim was to compare pre-infection and post-infection pulmonary function tests (PFT) in COVID-19 infected patients to better delineate between preexisting abnormalities and effects of the virus.

METHODS: This was a retrospective multi-center cohort study. Patients were identified based on having COVID-19 and a pre- and post-infection PFT within one year of infection during the time period of March 1, 2020 to November 10, 2020.

FINDINGS: There was a total of 80 patients, with an even split in gender; the majority were white (n = 70, 87·5%) and never smokers (n = 42, 52·5%). The majority had mild to moderate COVID-19 disease (n = 60, 75·1%) with 25 (31·2%) requiring hospitalization. There was no difference between the pre- and post-PFT data, specifically with the forced vital capacity (FVC) (p = 0·52), forced expiratory volume in 1 s (FEV1)(p = 0·96), FEV1/FVC(p = 0·66), total lung capacity (TLC) (p = 0·21), and diffusion capacity (DLCO)(p = 0·88). There was no difference in the PFT when analyzed by hospitalization and disease severity. After adjusting for potential confounders, interstitial lung disease (ILD) was independently associated with a decreased FEV1 (-2·6 [95% CI, -6·7 to - 1·6] vs. -10·3 [95% CI, -17·7 to -2·9]; p = 0·03) and an increasing age (p = 0·01) and cystic fibrosis (-1·1 [95% CI, -4·5 to- 2·4] vs. -36·5 [95% CI, -52·1 to -21·0]; p < 0·01) were associated with decreasing FVC when comparing pre and post infection PFT. Only increasing age was independently associated with a reduction in TLC (p = 0·01) and DLCO (p = 0·02) before and after infection.

INTERPRETATION: This study showed that there is no difference in pulmonary function as measured by PFT before and after COVID-19 infection in non-critically ill classified patients. There could be a relationship with certain underlying lung diseases (interstitial lung disease and cystic fibrosis) and decreased lung function following infection. This information should aid clinicians in their interpretation of pulmonary function tests obtained following COVID-19 infection.

FUNDING: No funding was obtained for this study.

PMID:34405138 | PMC:PMC8360709 | DOI:10.1016/j.eclinm.2021.101079

Sci Rep. 2021 Aug 17;11(1):16645. doi: 10.1038/s41598-021-96141-w.

ABSTRACT

Cystic fibrosis (CF) occurs as a result of mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which lead to misfolding, trafficking defects, and impaired function of the CFTR protein. Splicing factor proline/glutamine-rich (SFPQ) is a multifunctional nuclear RNA-binding protein (RBP) implicated in the regulation of gene expression pathways and intracellular trafficking. Here, we investigated the role of SFPQ in the regulation of the expression and function of F508del-CFTR in CF lung epithelial cells. We find that the expression of SFPQ is reduced in F508del-CFTR CF epithelial cells compared to WT-CFTR control cells. Interestingly, the overexpression of SFPQ in CF cells increases the expression as well as rescues the function of F508del-CFTR. Further, comprehensive transcriptome analyses indicate that SFPQ plays a key role in activating the mutant F508del-CFTR by modulating several cellular signaling pathways. This is the first report on the role of SFPQ in the regulation of expression and function of F508del-CFTR in CF lung disease. Our findings provide new insights into SFPQ-mediated molecular mechanisms and point to possible novel epigenetic therapeutic targets for CF and related pulmonary diseases.

PMID:34404863 | DOI:10.1038/s41598-021-96141-w

BMC Pulm Med. 2021 Aug 18;21(1):270. doi: 10.1186/s12890-021-01613-4.

ABSTRACT

BACKGROUND: Adult patients with cystic fibrosis (CF) experience daily physical symptoms and disabilities that can be challenging to address for health care teams.

METHODS: We sought to identify the most frequent topics that CF adults need to discuss with health care teams using a custom questionnaire including 62 items.

RESULTS: Fifty patients were included, 70% men, mean age 27.6 years, with a mean body mass index of 21.8 kg/m2. Mean FEV1% was 64% of predicted value. Forty-two percent of patients selected at least one topic. The most frequently selected topics were fatigue (20%), professional or scholar worries (18%), procreation (16%), physical activities (16%) and evolution of CF disease (16%). Women were more frequently concerned about fatigue, procreation and profession/school.

CONCLUSIONS: Using a custom questionnaire, we identified that CF adults express various unmet needs that extend beyond usual respiratory and nutritional concerns or treatment adherence. The interest of this questionnaire by health care team for improving therapeutic management of CF patients remains to be validated.

TRIAL REGISTRATION: The study was registered on ClinicalTrials.gov (NCT02924818) on 5th October 2016.

PMID:34404378 | DOI:10.1186/s12890-021-01613-4

Per Med. 2021 Aug 17. doi: 10.2217/pme-2021-0005. Online ahead of print.

ABSTRACT

There is little question that precision medicine will eventually be the standard of care in treatment with algorithms designed for therapy selection and is already being used in some specialties such as cystic fibrosis and multiple cancer treatments. Genetic counselors are the heart of the treatment team in relation to counseling regarding genetic risk factors and disease states. A framework for treatment within the interdisciplinary team with more defined roles and areas of specialty will need to be in place as this practice approach expands with new data and treatments. Pharmacists are poised to be of great assistance in this matrix as many of these roles are merely an extension of current tasks and responsibilities of pharmacy practice.

PMID:34402307 | DOI:10.2217/pme-2021-0005

Pediatr Pulmonol. 2021 May;56(5):901-909. doi: 10.1002/ppul.25207. Epub 2020 Dec 14.

ABSTRACT

INTRODUCTION: Annual oral glucose tolerance testing (OGTT) is the recommended screening modality for cystic fibrosis-related diabetes (CFRD) in patients with cystic fibrosis (CF). This study aimed to determine if there were patterns of progression of worsening glucose homeostasis in pediatric CF patients and to explore any relationship to lung function.

METHODS: We conducted a retrospective cohort study of CF patients, ages 10-18 years, without CFRD and with ≥3 OGTT from 2013 to 2016. Latent class mixture models were used to determine unique trajectories of 2-h OGTT glucose values (2hrGlu) over time. Multivariable linear models were used to adjust for clinical covariates.

RESULTS: For 63 subjects, three unique 2hrGlu trajectories were identified: high (impaired glucose tolerance) to higher (n = 8), low (normal glucose tolerance [NGT]) and increasing (n = 47), and low (NGT) and flat (n = 8). There was high variability of 2hrGlu, but most patients belonged to a trajectory that increased over time. After controlling for age, pancreatic insufficiency, modulator use, and mutation type, there was a significant difference in the study baseline forced expiratory volume in 1 s percent predicted (ppFEV1) in the high to higher group compared to the low and increasing and low and flat groups (p < .005).

DISCUSSION: Among pediatric CF patients without diabetes, three 2hrGlu trajectories were identified with 87% of patients exhibiting a trajectory where glucose homeostasis worsened over time. Starting ppFEV1 was lower in those with a high to higher trajectory, supporting that lower lung function is present early in the development of CFRD.

PMID:34402224 | DOI:10.1002/ppul.25207

Respir Med Case Rep. 2021 Jun 6;33:101434. doi: 10.1016/j.rmcr.2021.101434. eCollection 2021.

ABSTRACT

We present a rare coexistence of constrictive pericarditis in a patient with cystic fibrosis. Careful attention to cardiac friction rub auscultated on initial examination prompted echocardiography revealing constrictive pericarditis further confirmed by cardiac magnetic resonance imaging that allowed for dedicated treatment in addition to management of his concurrent respiratory infection.

PMID:34401277 | PMC:PMC8349017 | DOI:10.1016/j.rmcr.2021.101434

Respir Med Case Rep. 2021 Apr 15;33:101413. doi: 10.1016/j.rmcr.2021.101413. eCollection 2021.

ABSTRACT

We present three cases in this report. Three adult brothers, homozygous for the delF508 cystic fibrosis mutation, have maintained an unusually preserved clinical condition even though they did not attend a CF Clinic during their childhood, do not attend a CF Clinic now, and do not follow standard CF care guidelines. The brothers use an alternative CF treatment regimen on which they have maintained normal lung function, height/weight, and bloodwork, and they utilize less than half the recommended dosage of pancreatic enzymes. The brothers culture only methicillin-sensitive Staphylococcus aureus, and have never cultured any other bacteria. Highly effective modulator therapies, such as elexacaftor/tezacaftor/ivacaftor, do not substantially reduce infection and inflammation in vivo in CF patients, and thus these three case reports are of special note in terms of suggesting adjunct therapeutic approaches. Finally, these three cases also raise important questions about standard CF care guidelines.

PMID:34401261 | PMC:PMC8348530 | DOI:10.1016/j.rmcr.2021.101413

Respir Med Case Rep. 2021 Mar 19;33:101386. doi: 10.1016/j.rmcr.2021.101386. eCollection 2021.

ABSTRACT

A significant dysfunction of another organ is usually considered an absolute contraindication for lung transplantation, unless multiorgan transplantation is indicated and practical, as is the case of combined lung-kidney transplantation. Few cases of combined lung-kidney transplantation have been described in the literature; however, it is known that, in certain cases, it is the only way to offer an opportunity to selected patients with renal and lung dysfunction. The authors are not aware of any previously published case of a patient receiving both extracorporeal membrane oxygenation and continuous venovenous hemodiafiltration as a bridge for combined kidney-lung transplantation. The authors present the first case of combined lung-kidney transplantation performed in Portugal.

PMID:34401253 | PMC:PMC8348689 | DOI:10.1016/j.rmcr.2021.101386

Thromb Res. 2021 Jul 10;206:36-41. doi: 10.1016/j.thromres.2021.07.007. Online ahead of print.

ABSTRACT

BACKGROUND: People with cystic fibrosis (CF) have an increased risk of thrombosis due to acquired thrombophilia secondary to chronic systemic inflammation and central venous catheter use for treatment of pulmonary infections. The objective of this study is to determine the safety and effectiveness of a risk-stratified, venous thromboembolism (VTE) prophylaxis intervention.

METHODS: This single-center, IRB-approved, retrospective study assessed patients with CF admitted to our institution for treatment of a pulmonary exacerbation from 2017 to 2019. Data and outcomes were manually extracted from the electronic medical record and internal CF clinical database. Subject characteristics, calculated VTE risk, prophylaxis interventions prescribed, VTE incidence, and adverse events were captured.

RESULTS: A total of 135 CF patients had 354 admissions for pulmonary exacerbations in the time frame of the study. The majority of admissions (88.7%) were classified as moderate or high risk for VTE using the algorithm. Overall, VTE prophylaxis intervention determined by the algorithm was initiated in 36.2% of admissions. During the study period, no VTE events occurred. Four minor bleeding adverse effects were reported in patients receiving VTE chemical prophylaxis with enoxaparin (4.2%).

CONCLUSIONS: This study provides the first reported outcomes following implementation of a risk-stratified VTE prophylaxis algorithm in hospitalized young people with CF. In this population at increased risk, use of risk-stratified prophylaxis was safe and effective in preventing VTE. Additional work to improve and maintain adherence to the algorithm and VTE prophylaxis interventions at our institution is planned and similar care should be considered at other pediatric CF care centers.

PMID:34399123 | DOI:10.1016/j.thromres.2021.07.007

Rhinology. 2021 Aug 16. doi: 10.4193/Rhin21.064. Online ahead of print.

ABSTRACT

BACKGROUND: Cystic fibrosis (CF) carriers seem to have a higher risk to develop chronic rhino-sinusitis (CRS), although the full underlying mechanisms are unknown. Ion concentrations in nasal airway surface liquid (ASL) may be influenced by the heterozygosity for CF gene mutation, with possible impacts on the development of CRS.

METHODS: A cheap and feasible standardized technique was designed to measure the ion levels in nasal ASL. With this purpose we collected, under basal conditions, samples from the nasal cavity of 165 adults: 14 homozygous for CF, 83 carriers and 68 healthy controls. Sodium (Na) and Chlorine (Cl) concentrations were then evaluated among different groups.

RESULTS: Statistical analysis revealed a significant difference of Na and Cl values between controls and carriers and between controls and homozygotes. Receiver operating characteristic (ROC) curves and derived indicators (Youden's index and Area Under the Curve, AUC) were used to further evaluate the diagnostic capability of Na and Cl concentrations to differentiate heterozygotes from controls. ROC curves demonstrated that the optimal diagnostic cut-off value of Na is at 124, and the optimal cut-off value of Cl is at 103,2.

CONCLUSION: ASL sampling can be considered a new diagnostic tool for providing quantitative information on nasal ion composition. According to our findings, Na and Cl concentrations of nasal ASL could represent a useful tool to assess heterozygotes and healthy controls.

PMID:34398939 | DOI:10.4193/Rhin21.064

J Bacteriol. 2021 Aug 16:JB0025021. doi: 10.1128/JB.00250-21. Online ahead of print.

ABSTRACT

In vitro culture media are being developed to understand how host site-specific nutrient profiles influence microbial pathogenicity and ecology. To mimic the cystic fibrosis (CF) lung environment, a variety of artificial sputum media (ASM) have been created. However, the composition of these ASM vary in the concentration of key nutrients, including amino acids, lipids, DNA, and mucin. In this work, we used feature-based molecular networking (FBMN) to perform comparative metabolomics of Pseudomonas aeruginosa, the predominant opportunistic pathogen infecting the lungs of people with CF, cultured in nine different ASM. We found that the concentration of aromatic amino acids and iron from mucin added to the media contribute to differences in the production of P. aeruginosa virulence-associated secondary metabolites. IMPORTANCE Different media formulations aiming to replicate in vivo infection environments contain different nutrients, which affects interpretation of experimental results. Inclusion of undefined components, such as commercial porcine gastric mucin (PGM), in an otherwise chemically defined medium can alter the nutrient content of the medium in unexpected ways and influence experimental outcomes.

PMID:34398662 | DOI:10.1128/JB.00250-21

Front Endocrinol (Lausanne). 2021 Jul 30;12:702823. doi: 10.3389/fendo.2021.702823. eCollection 2021.

ABSTRACT

Cystic fibrosis (CF) is a genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). Cystic fibrosis-related diabetes (CFRD) is the most common comorbidity, affecting more than 50% of adult CF patients. Despite this high prevalence, the etiology of CFRD remains incompletely understood. Studies in young CF children show pancreatic islet disorganization, abnormal glucose tolerance, and delayed first-phase insulin secretion suggesting that islet dysfunction is an early feature of CF. Since insulin-producing pancreatic β-cells express very low levels of CFTR, CFRD likely results from β-cell extrinsic factors. In the vicinity of β-cells, CFTR is expressed in both the exocrine pancreas and the immune system. In the exocrine pancreas, CFTR mutations lead to the obstruction of the pancreatic ductal canal, inflammation, and immune cell infiltration, ultimately causing the destruction of the exocrine pancreas and remodeling of islets. Both inflammation and ductal cells have a direct effect on insulin secretion and could participate in CFRD development. CFTR mutations are also associated with inflammatory responses and excessive cytokine production by various immune cells, which infiltrate the pancreas and exert a negative impact on insulin secretion, causing dysregulation of glucose homeostasis in CF adults. In addition, the function of macrophages in shaping pancreatic islet development may be impaired by CFTR mutations, further contributing to the pancreatic islet structural defects as well as impaired first-phase insulin secretion observed in very young children. This review discusses the different factors that may contribute to CFRD.

PMID:34394004 | PMC:PMC8361832 | DOI:10.3389/fendo.2021.702823

Front Psychol. 2021 Jul 28;12:701540. doi: 10.3389/fpsyg.2021.701540. eCollection 2021.

ABSTRACT

Different forms of dyadic coping are associated with positive outcomes in partner relationships, yet little is known about dyadic coping in parent-child relationships. The current research explored the association between parent-child dyadic coping and children's quality of life in 12-18-year old children with a chronic disease (i.e., cystic fibrosis, autoimmune diseases, and children post-cancer treatment). In a sample of 105 parent-child dyads, self-reported forms of dyadic coping (i.e., stress communication, problem-oriented, emotion-oriented, and negative dyadic coping) and children's quality of life were assessed. Children reported more stress communication and negative dyadic coping than their parents, while parents reported more problem-oriented dyadic coping and emotion-oriented dyadic coping than their children. More stress communication of the child was associated with more emotion-oriented dyadic coping and less negative dyadic coping of the parent. More negative dyadic coping of the child was associated with less stress communication, problem-oriented dyadic coping and emotion-oriented dyadic coping of the parent. Additionally, both children's and parents' negative dyadic coping were associated with lower self-reported pediatric quality of life and parents' emotion-oriented dyadic coping was associated with higher pediatric quality of life. These findings emphasize that children and their parents mutually influence each other and that dyadic coping is associated with children's quality of life. Theoretical and practical implications are discussed.

PMID:34393938 | PMC:PMC8355494 | DOI:10.3389/fpsyg.2021.701540

J Cyst Fibros. 2021 Aug 12:S1569-1993(21)01339-4. doi: 10.1016/j.jcf.2021.07.020. Online ahead of print.

ABSTRACT

Chronic Pseudomonas aeruginosa (Pa) infection is associated with increased morbidity and mortality in people with cystic fibrosis (CF). There is no gold standard definition of chronic Pa infection in CF. We compared chronic Pa definitions using encounter-based versus annualized data in the Early Pseudomonas Infection Control (EPIC) Observational study cohort, and subsequently compared annualized chronic Pa definitions across a range of U.S. cohorts spanning decades of CF care. We found that an annualized chronic Pa definition requiring at least 1 Pa+ culture in 3 of 4 consecutive years ("Green 3/4") resulted in chronic Pa metrics similar to established encounter-based modified Leeds criteria definitions, including a similar age at and proportion who fulfilled chronic Pa criteria, and a similar proportion with sustained Pa infection after meeting the chronic Pa definition. The Green 3/4 chronic Pa definition will be valuable for longitudinal analyses in cohorts with limited culture frequency.

PMID:34393091 | DOI:10.1016/j.jcf.2021.07.020

Acad Radiol. 2021 Aug 12:S1076-6332(21)00304-4. doi: 10.1016/j.acra.2021.06.017. Online ahead of print.

ABSTRACT

RATIONALE: There is no agreed upon method for quantifying ventilation defect percentage (VDP) with high sensitivity and specificity from hyperpolarized (HP) gas ventilation MR images in multiple pulmonary diseases for both pediatrics and adults, yet identifying such methods will be necessary for future multi-site trials. Most HP gas MRI ventilation research focuses on a specific pulmonary disease and utilizes one quantification scheme for determining VDP. Here we sought to determine the potential of different methods for quantifying VDP from HP 129Xe images in multiple pulmonary diseases through comparison of the most utilized quantification schemes: linear binning and thresholding.

MATERIALS AND METHODS: HP 129Xe MRI was performed in a total of 176 subjects (125 pediatrics and 51 adults, age 20.98±16.48 years) who were either healthy controls (n = 23) or clinically diagnosed with cystic fibrosis (CF) (n = 37), lymphangioleiomyomatosis (LAM) (n = 29), asthma (n = 22), systemic juvenile idiopathic arthritis (sJIA) (n = 11), interstitial lung disease (ILD) (n = 7), or were bone marrow transplant (BMT) recipients (n = 47). HP 129Xe ventilation images were acquired during a ≤16 second breath-hold using a 2D multi-slice gradient echo sequence on a 3T Philips scanner (TR/TE 8.0/4.0ms, FA 10-12°, FOV 300 × 300mm, voxel size≈3 × 3 × 15mm). Images were analyzed using 5 different methods to quantify VDPs: linear binning (histogram normalization with binning into 6 clusters) following either linear or a variant of a nonparametric nonuniform intensity normalization algorithm (N4ITK) bias-field correction, thresholding ≤60% of the mean signal intensity with linear bias-field correction, and thresholding ≤60% and ≤75% of the mean signal intensity following N4ITK bias-field correction. Spirometry was successfully obtained in 84% of subjects.

RESULTS: All quantification schemes were able to label visually identifiable ventilation defects in similar regions within all subjects. The VDPs of control subjects were significantly lower (p<0.05) compared to BMT, CF, LAM, and ILD subjects for most of the quantification methods. No one quantification scheme was better able to differentiate individual disease groups from the control group. Advanced statistical modeling of the VDP quantification schemes revealed that in comparing controls to the combined disease group, N4ITK bias-field corrected 60% thresholding had the highest predictive efficacy, sensitivity, and specificity at the VDP cut-point of 2.3%. However, compared to the thresholding quantification schemes, linear binning was able to capture and label subtle low-ventilation regions in subjects with milder obstruction, such as subjects with asthma.

CONCLUSION: The difference in VDP between healthy controls and patients varied between the different disease states for all quantification methods. Although N4ITK bias-field corrected 60% thresholding was superior in separating the combined diseased group from controls, linear binning is able to better label low-ventilation regions unlike the current, 60% thresholding scheme. For future clinical trials, a consensus will need to be reached on which VDP scheme to utilize, as there are subtle advantages for each for specific disease.

PMID:34393064 | DOI:10.1016/j.acra.2021.06.017

Ann Allergy Asthma Immunol. 2021 Aug 12:S1081-1206(21)00568-8. doi: 10.1016/j.anai.2021.08.010. Online ahead of print.

NO ABSTRACT

PMID:34391901 | DOI:10.1016/j.anai.2021.08.010

J Cyst Fibros. 2021 Aug 11:S1569-1993(21)01340-0. doi: 10.1016/j.jcf.2021.07.021. Online ahead of print.

NO ABSTRACT

PMID:34391678 | DOI:10.1016/j.jcf.2021.07.021

Pediatr Int. 2021 Aug 13. doi: 10.1111/ped.14951. Online ahead of print.

ABSTRACT

BACKGROUND: Cystic fibrosis (CF) is an autosomal recessively inherited disease. Clinical findings vary by age of the patient, the organ systems involved, and the severity of the CFTR gene mutation. Pancreatic and liver involvement is prominent and exocrine pancreatic insufficiency is observed in the majority of patients. Point shear wave elastography (pSWE) is a non-invasive method that can quantitatively determine tissue elasticity and stiffness. In this study, the morphological evaluation of the pancreas was performed using the pSWE technique in pediatric patients diagnosed with CF. The effectiveness of this method for early detection of pancreatic insufficiency was investigated.

METHODS: Fifty-five patients with CF (24 girls, 31 boys) and 60 healthy children (29 girls, 31 boys) without any chronic diseases and who were suitable for the pSWE examination were included in the study.

RESULTS: The mean value of pSWE was 1.12±0.16 in the healthy group and 0.97±0.16 in the patients with cystic fibrosis. There was a statistically significant difference between the two groups (p <0.001). Significant negative correlations were found with between pSWE and age (r:-0.319; p=0.018), height (r:-0.293; p=0.03), serum glucose (r:-0.346; p=0.01), HbA1C (r:-0.592; p=0.02), and duration of the disease (r:-0.806; p<0.001).

CONCLUSION: Investigating pancreatic elasticity and detecting pancreatic insufficiency using pSWE (a simple, inexpensive, and non-invasive method) in the early period before overt laboratory and clinical symptoms of EPI can positively contribute to long-term results in young patients with CF.

PMID:34390069 | DOI:10.1111/ped.14951

Genet Med. 2021 Aug 13. doi: 10.1038/s41436-021-01281-z. Online ahead of print.

NO ABSTRACT

PMID:34389817 | DOI:10.1038/s41436-021-01281-z