Understanding Human Lung Development through In Vitro Model Systems.

Bioessays. 2020 Apr 20;:e2000006

Authors: Conway RF, Frum T, Conchola AS, Spence JR

Abstract
An abundance of information about lung development in animal models exists; however, comparatively little is known about lung development in humans. Recent advances using primary human lung tissue combined with the use of human in vitro model systems, such as human pluripotent stem cell-derived tissue, have led to a growing understanding of the mechanisms governing human lung development. They have illuminated key differences between animal models and humans, underscoring the need for continued advancements in modeling human lung development and utilizing human tissue. This review discusses the use of human tissue and the use of human in vitro model systems that have been leveraged to better understand key regulators of human lung development and that have identified uniquely human features of development. This review also examines the implementation and challenges of human model systems and discusses how they can be applied to address knowledge gaps.

PMID: 32310312 [PubMed - as supplied by publisher]

Can Point Shear Wave Elastography Be Used as an Early Indicator of Involvement?: Evaluation of the Pancreas and Liver in Children With Cystic Fibrosis.

J Ultrasound Med. 2020 Apr 20;:

Authors: Sağlam D, Demirbaş F, Bilgici MC, Yücel S, Çaltepe G, Eren E

Abstract
OBJECTIVES: The aim of this study was to determine the effect of cystic fibrosis (CF) on pancreas and liver elasticity in young children using point shear wave elastography and to determine the relationship with clinical findings.
METHODS: Twenty-two patients with genetically proven CF, who were admitted to our pediatric gastroenterology clinic, and 22 healthy control participants were enrolled in the study. The shear wave velocity (SWV) of the liver and pancreas were measured with point shear wave elastography.
RESULTS: The 22 patients with CF included 45.5% girls with a mean age ± SD of 35 ± 35.8 months (range, 5-123 months). The 22 healthy control participants included 41.2% girls with a mean age of 58.9 ± 44.4 months (range, 2-159 months). The mean SWV of the pancreas in the patients with CF (1.06 ± 0.26 m/s) was significantly higher than that of the healthy control participants (0.85 ± 0.23 m/s; P = .01). The mean SWV of the liver in the patients with CF (1.46 ± 0.24 m/s) was significantly higher than that of the healthy control participants (1.12 ± 0.21 m/s; P = .001). The SWV of the pancreas and liver did not show any significant differences depending on ursodeoxycholic acid use, malnutrition status, and the presence of the F508 deletion mutation.
CONCLUSIONS: This study showed an increased SWV of the pancreas in children with CF, contrary to the literature. We also found an increased liver SWV even in the absence of CF-related liver disease. Ultrasound elastography may be a useful method of evaluating early changes in the pancreas and liver before the obvious clinical, laboratory, and B-mode ultrasound signs of CF-related involvement.

PMID: 32309883 [PubMed - as supplied by publisher]

Encapsulated Methionine γ-Lyase: Application in Enzyme Prodrug Therapy of Pseudomonas aeruginosa Infection.

ACS Omega. 2020 Apr 14;5(14):7782-7786

Authors: Morozova E, Kulikova V, Koval V, Anufrieva N, Chernukha M, Avetisyan L, Lebedeva L, Medvedeva O, Burmistrov E, Shaginyan I, Revtovich S, Demidkina T

Abstract
Lung disease caused by Pseudomonas aeruginosa is the leading reason for death in cystic fibrosis patients. Therapeutic efficacy of the pharmacological pairs, the naked/encapsulated mutant form of Citrobacter freundii methionine γ-lyase and the substrates, sulfoxides of S-substituted l-cysteine, generating thiosulfinates, was evaluated on the murine model of experimental sepsis caused by the multidrug-resistant P. aeruginosa 203-2 strain. The pairs containing the naked enzyme and substrates did not have antibacterial activity. The treatment of mice with the pair encapsulated enzyme and S-methyl-l-cysteine sulfoxide, generating dimethyl thiosulfinate, led to a complete recovery of the animals of the model, with the infecting dose equal to LD50. The pair generating diallyl thiosulfinate (allicin) proved to be less effective. So, the substituents, attached to the thiosulfinate moiety, affect the antibacterial activity of thiosulfinates against P. aeruginosa.

PMID: 32309686 [PubMed]

High-intensity interval training accelerates oxygen uptake kinetics and improves exercise tolerance for individuals with cystic fibrosis.

BMC Sports Sci Med Rehabil. 2020;12:9

Authors: Reuveny R, DiMenna FJ, Gunaratnam C, Arad AD, McElvaney GN, Susta D, Peled M, Moyna NM

Abstract
Background: Exercise training provides benefits for individuals with cystic fibrosis; however, the optimal program is unclear. High-intensity interval training is safe and effective for improving 'functional capacity' in these individuals with peak rate of O2 uptake typically referenced. The ability to adjust submaximal rate of oxygen uptake (V̇O2 kinetics) might be more important for everyday function because maximal efforts are usually not undertaken. Moreover, the ability of high-intensity training to accelerate V̇O2 kinetics for individuals with cystic fibrosis could be enhanced with O2 supplementation during training.
Methods: Nine individuals with cystic fibrosis completed incremental cycling to limit of tolerance followed by 8 weeks of high-intensity interval cycling (2 sessions per week x ~ 45 min per session) either with (n = 5; O2+) or without (AMB) oxygen supplementation (100%). Each session involved work intervals at 70% of peak work rate followed by 60 s of recovery at 35%. For progression, duration of work intervals was increased according to participant tolerance.
Results: Both groups experienced a significant increase in work-interval duration over the course of the intervention (O2+, 1736 ± 141 v. 700 ± 154 s; AMB, 1463 ± 598 v. 953 ± 253 s; P = 0.000); however, the increase experienced by O2+ was greater (P = 0.027). During low-intensity constant-work-rate cycling, the V̇O2 mean response time was shortened post compared to pre training (O2+, 34 ± 11 v. 44 ± 9 s; AMB, 39 ± 14 v. 45 ± 17 s; P = 0.000) while during high-intensity constant-work-rate cycling, time to exhaustion was increased (O2+, 1628 ± 163 v. 705 ± 133 s; AMB, 1073 ± 633 v. 690 ± 348 s; P = 0.002) and blood [lactate] response was decreased (O2+, 4.5 ± 0.9 v. 6.3 ± 1.4 mmol. L- 1; AMB, 4.5 ± 0.6 v. 5.2 ± 1.4 mmol. L- 1; P = 0.003). These positive adaptations were similar regardless of gas inspiration during training.
Conclusion: Eight weeks of high-intensity interval training for patients with cystic fibrosis accelerated V̇O2 kinetics and increased time to exhaustion. This provides some evidence that these patients may benefit from this type of exercise.
Trial registration: This study was retrospectively registered in the ISRTCN registry on 22/06/2019 (#ISRCTN13864650).

PMID: 32308986 [PubMed]

Assessment of Selected Parameters of Liver Fibrosis and Inflammation in Patients with Diagnosed Cystic Fibrosis.

Mediators Inflamm. 2020;2020:5696185

Authors: Więcek S, Woś H, Pogorzelski A, Kordys-Darmolinska B, Mazurek H, Grzybowska-Chlebowczyk U

Abstract
Changes in the liver and bile ducts observed in patients diagnosed with cystic fibrosis result from inflammatory processes as well as fibrosis, remodeling, apoptosis, and cholestasis. As a consequence, portal hypertension, cirrhosis, and hepatic failure may develop. So far, the complexity of these processes has not been elucidated. Study Objectives. The aim of the study was to evaluate the selected parameters of hepatitis and fibrosis (Fibrotest, Actitest, and APRI) in patients diagnosed with cystic fibrosis. Material and Methods. The study included 79 patients with cystic fibrosis, aged 1 to 20 years (mean age 9.8 years), 49 girls (62%) and 30 boys (38%). The analysis involved the following: age, sex, clinical manifestations, laboratory tests evaluating pancreas function, parameters of liver damage, and cholestasis. Fibrotest, Actitest, and APRI were performed in all subjects. Results. Elevated parameters of hepatic cell damage (hypertransaminasemia) were found in 31/79 (39.2%) patients, while abnormal cholestasis parameters in 21/79 (26.6%). The abnormal results of Fibrotest were reported in 15% of patients (12/79), while of Actitest in 10% (8/79). In contrast, elevated APRI values were found in only 7.6% (6/79) of subjects. There was a statistically significant correlation between APRI and age (higher values were observed in younger children) and between Fibrotest and Actitest and pancreatic insufficiency (higher values were found in subjects without this abnormality). Moreover, Fibrotest values were significantly higher in girls. There was no correlation between Fibrotest, Actitest, and APRI values and the type of mutation. Conclusion. It appears that Fibrotest may be used as an early marker of liver fibrosis in patients with cystic fibrosis. Increased APRI values were only found in subjects with advanced hepatic lesions, most often in the form of portal hypertension.

PMID: 32308556 [PubMed - in process]

Negative chronotropic and inotropic effects of lubiprostone on iPS cell-derived cardiomyocytes via activation of CFTR.

BMC Complement Med Ther. 2020 Apr 19;20(1):118

Authors: Akita H, Yoshie S, Ishida T, Takeishi Y, Hazama A

Abstract
BACKGROUND: Lubiprostone (LBP) is a novel chloride channel opener that has been reported to activate chloride channel protein 2 (ClC-2) and cystic fibrosis transmembrane conductance regulator (CFTR). LBP facilitates fluid secretion by activating CFTR in the intestine and is used as a drug for treating chronic constipation. While ClC-2 and CFTR expression has been confirmed in cardiomyocytes (CMs), the effect of LBP on CMs has not yet been investigated. Thus, the present study aimed to investigate the effect of LBP on CMs using mouse-induced pluripotent stem (iPS) cell-derived CMs (iPS-CMs).
METHODS: We induced mouse iPS cells into CMs through embryoid body (EB) formation. We compared the differentiated cells to CMs isolated from adult and fetal mice using gene expression, spontaneous beating rate, and contraction ratio analyses.
RESULTS: Gene expression analysis revealed that, in the iPS-CMs, the mRNA expression of the undifferentiated cell markers Rex1 and Nanog decreased, whereas the expression of the unique cardiomyocyte markers cardiac troponin I (cTnI) and cardiac troponin T (cTNT), increased. Immunostaining showed that the localization of cTnI and connexin-43 in the iPS-CMs was similar to that in the primary fetal CMs (FCMs) and adult CMs (ACMs). LBP decreased the spontaneous beating rate of the iPS-CMs and FCMs, and decreased the contraction ratio of the iPS-CMs and ACMs. The reduction in the beating rate and contraction ratio caused by LBP was inhibited by glycine hydrazide (GlyH), which is a CFTR inhibitor.
CONCLUSION: These results suggest that LBP stimulates CFTR in CMs and that LBP has negative chronotropic and inotropic effects on CMs. LBP may be useful for treating cardiac diseases such as heart failure, ischemia, and arrhythmia.

PMID: 32306956 [PubMed - in process]

The double wrinkled colon sign.

Abdom Radiol (NY). 2019 03;44(3):1187-1188

Authors: Pathak P, Dyer RB, Tappouni R

Abstract

PMID: 30367213 [PubMed - indexed for MEDLINE]

StatPearls

Book. 2020 01

Authors:

Abstract
Cystic fibrosis (CF) is an autosomal recessive (AR) disorder that commonly affects the White population with an annual incidence of approximately 1 in 3,500 live births.[1][2][3] This multisystem disorder is characterized by genetic mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene on chromosome 7, which encrypts a protein essential for the regulation of transmembrane chloride reabsorption.[1][3] CFTR, a chloride channel, is expressed in secretory epithelial cells lining the airways, digestive system, reproductive system, and the skin.[2] Homozygous mutations in the CFTR gene impairs the transport of chloride ions and the movement of water into and out of cells resulting in the inspissation of secretions leading to organ dysfunction. Although CF commonly affects the airways, approximately 10% to 15% of patients with CF demonstrate Cystic fibrosis-associated liver disease (CFLD).[4] The improvement in the diagnostic modalities and management of CF that has had a positive impact on the life expectancy of patients with CF. Consequently, there has been a significant emergence of liver dysfunction in CF complicating the clinical course of the disease.[5][1] CFTR dysfunction has a significant effect on cholangiocyte function causing an alteration in the final bile composition that causes chronic damage to the biliary epithelium resulting in the development of a broad spectrum of hepatobiliary complications ranging from cholestasis, progressive periportal fibrosis, biliary obstruction causing focal biliary cirrhosis which may progress to multinodular cirrhosis, portal hypertension, and hepatic decompensation.[6][7][8]

PMID: 32310546

StatPearls

Book. 2020 01

Authors:

Abstract
Dornase alfa is utilized in the treatment and management of cystic fibrosis (CF) in conjunction with standard therapies. Dornase alfa was discovered in 1992 as a mucolytic agent for the treatment of hyper viscous mucous produced in patients with CF, after noticing in the 1950s that the use of bovine deoxyribonuclease decreased the thickness of the sputum in CF patients.[1]

PMID: 32310478

Decreased airway epithelial ion transport was associated with the severity of the respiratory syncytial virus infection and complications in infants.

Acta Paediatr. 2020 Apr 18;:

Authors: Kaskinen A, Alexandersson A, Andersson S, Saxén H, Peltola V, Kolho KL, Helve O

Abstract
The respiratory syncytial virus (RSV) primarily infects airway epithelial cells. It also decreases airway epithelial sodium transport, especially through the epithelial sodium channel (ENaC) (1), which is crucial for osmosis-based fluid absorption across respiratory epithelium. Meanwhile, the electrochemical balance is maintained by secretion of chloride ions through apical chloride channels, including the cystic fibrosis transmembrane conductance regulator (CFTR) (1). In animals, ENaC inhibition has caused middle ear fluid collection in acute otitis media (AOM) (2).

PMID: 32306429 [PubMed - as supplied by publisher]

Age-Dependent Progression in Lung Pathophysiology can be Prevented by Restoring Fatty Acid and Ceramide Imbalance in Cystic Fibrosis.

Lung. 2020 Apr 18;:

Authors: Youssef M, De Sanctis JB, Shah J, Dumut DC, Hajduch M, Petrof BJ, Radzioch D

Abstract
PURPOSE: Cystic fibrosis (CF) is a progressive disease which causes a continuous decline in lung capacity with age. Our study aimed to investigate the age-dependent deterioration in lung function and the effects of treatment with Fenretinide formulation (LAU-7b) in Cftr knockout (KO) mice.
METHODS: Non-invasive whole-body plethysmography (WBP) was done to measure the baseline lung functions of KO and wild-type (WT) mice at the ages of 2 and 4 months. Mice were then treated for 21 days with PBS or 10 mg/kg/day LAU-7b initiated at 4 and 7 months. Standard airway resistance measurements, haematoxylin and eosin staining, and analysis of lipids, and markers of oxidation were performed.
RESULTS: The 4- and 7-month-old KO mice had significantly higher lung enhanced pause (Penh) and resistance values than age-matched WT mice and 2-month-old KO mice. Likewise, analysis of ceramides showed that PBS-treated mice had higher levels of long-chain ceramides (LCCs; C14-C18) and lower levels of very-long-chain ceramides (VLCCs; C24-C26) compared to LAU-7b-treated mice. Cftr KO mice displayed markedly greater inflammatory cell infiltration and epithelial hyperplasia at the ages of 2, 4, and 7 months compared to WT. LAU-7b treatment significantly diminished this cellular infiltration and epithelial hyperplasia compared to PBS-treated mice.
CONCLUSION: Our results demonstrate a progressive age-dependent decline in lung function in Cftr KO mice. Treatment with LAU-7b corrects the lipid imbalance observed in the aging KO and WT mice and, more importantly, inhibits the age-dependent deterioration in lung physiology and histopathology.

PMID: 32306138 [PubMed - as supplied by publisher]

Overlooking the landscape of palliative care in cystic fibrosis.

J Cyst Fibros. 2020 Apr 15;:

Authors: Chin M, McIntosh ID, Somayaji R

PMID: 32305179 [PubMed - as supplied by publisher]

Peanut oral immunotherapy protects patients from accidental allergic reactions to peanut.

J Allergy Clin Immunol Pract. 2020 Apr 15;:

Authors: Trendelenburg V, Blumchen K, Bellach J, Ahrens F, Gruebl A, Hamelmann E, Hansen G, Heinzmann A, Nemat K, Holzhauser T, Röder M, Niggemann B, Beyer K

PMID: 32304836 [PubMed - as supplied by publisher]

Impact of COVID-19 on people with cystic fibrosis.

Lancet Respir Med. 2020 Apr 15;:

Authors: Colombo C, Burgel PR, Gartner S, van Koningsbruggen-Rietschel S, Naehrlich L, Sermet-Gaudelus I, Southern KW

PMID: 32304639 [PubMed - as supplied by publisher]

Celiac Disease in Patients with Cystic Fibrosis on Ivacaftor: A Case Series.

J Pediatr Gastroenterol Nutr. 2020 Apr 16;:

Authors: Hjelm M, Shaikhkhalil AK

Abstract
Cystic fibrosis transmembrane conductance regulator (CFTR) protein modulators have revolutionized care for individuals with CF with positive effects on the gastrointestinal (GI) tract. There is emerging evidence linking CFTR dysfunction to celiac disease. We present three cases of patients with CF, genotype F508del/G551D, treated with CFTR modulator, Ivacaftor, and diagnosed with celiac disease. These patients tested for celiac disease because they had persistent GI symptoms that had partially improved with ivacaftor. This case series highlights the importance of a better understanding of how CFTR modulators impact the GI tract, their possible link to celiac disease, and the importance of considering celiac disease when evaluating GI symptoms in individuals with CF.

PMID: 32304549 [PubMed - as supplied by publisher]

Outcomes of Children With Cystic Fibrosis Admitted to PICUs.

Pediatr Crit Care Med. 2020 Apr 17;:

Authors: Smith MA, McGarry ME, Ly NP, Zinter MS

Abstract
OBJECTIVES: Data on outcomes of children with cystic fibrosis admitted to PICUs are limited and outdated. Prior studies cite PICU mortality rates ranging from 37.5% to 100%. Given the advances made in cystic fibrosis care, we expect outcomes for these patients to have changed significantly since last studied. We provide an updated report on PICU mortality and the factors associated with death among critically ill children with cystic fibrosis.
DESIGN: Retrospective multicenter cohort analysis utilizing data from the Virtual Pediatric Systems database.
SETTING: Data were collected from 135 PICUs from January 1, 2009, to June 20, 2018.
PATIENTS: One-thousand six-hundred thirty-three children with cystic fibrosis accounting for 2,893 PICU admissions were studied.
INTERVENTIONS: None.
MEASUREMENTS AND MAIN RESULTS: The primary outcome was mortality during PICU admission. Predictors included demographics, anthropometrics, diagnoses, clinical characteristics, and critical care interventions. Odds ratios of mortality were calculated in univariate and multivariable analyses to assess differences in mortality associated with predictor variables. Generalized estimating equation models were used to account for multiple admissions per patient. The overall PICU mortality rate was 6.6%. Factors associated with increased odds of mortality included hemoptysis/pulmonary hemorrhage, pneumothorax, gastrointestinal bleeding, bacterial/fungal infections, lower body mass index/malnutrition, and need for noninvasive or invasive respiratory support. Intubation/mechanical ventilation occurred in 26.4% of the 2,893 admissions and was associated with a 19.1% mortality rate. Of the nonsurvivors, 20.7% died without receiving mechanical ventilation.
CONCLUSIONS: The mortality rate during PICU admissions for patients with cystic fibrosis is lower than has been reported in prior studies, both in the overall cohort and in the subset requiring invasive mechanical ventilation. These data provide updated insight into the prognosis for cystic fibrosis patients requiring critical care.

PMID: 32304511 [PubMed - as supplied by publisher]

Real-World Outcomes Among Patients with Cystic Fibrosis Treated with Ivacaftor: 2012-2016 Experience.

Pulm Ther. 2020 Apr 18;:

Authors: Higgins M, Volkova N, Moy K, Marshall BC, Bilton D

Abstract
INTRODUCTION: In this long-term, postapproval, observational study, data from the US Cystic Fibrosis Foundation Patient Registry and the UK Cystic Fibrosis Registry were used to evaluate the impact of ivacaftor treatment on cystic fibrosis (CF) by comparing outcomes in ivacaftor-treated patients with those in matched untreated comparator patients. Registry data from up to 5 years of ivacaftor availability in the US and up to 4 years of availability in the UK were evaluated.
METHODS: Starting in the first year of ivacaftor availability, ivacaftor-treated patients in each registry were matched 1:5 to comparator patients who never received ivacaftor. Clinical endpoints were evaluated in annual cross-sectional safety analyses. The key endpoints were death, organ transplants, pulmonary exacerbation, and hospitalization. Relative risks and 95% CIs were calculated to compare the ivacaftor and comparator cohorts in each registry.
RESULTS: Here, we report the complete and final results of the annual cross-sectional safety analyses across the duration of the study, with up to 5 years of follow-up. Data show a pattern of lower risk of death, transplant, pulmonary exacerbation, and hospitalization among ivacaftor-treated patients in both registries.
CONCLUSIONS: Ivacaftor-treated patients had consistently favorable clinical outcomes relative to untreated comparators, and no new safety concerns were identified. While general limitations of observational research apply, these findings support disease modification by CF transmembrane conductance regulator (CFTR) modulator therapy with ivacaftor. Future research of novel CFTR modulators will need to explore alternative methods for comparator selection for evaluation of clinical data given the evolving landscape of CF treatment.

PMID: 32304091 [PubMed - as supplied by publisher]

Asymptomatic case of Covid-19 in an infant with cystic fibrosis.

J Cyst Fibros. 2020 Apr 13;:

Authors: Poli P, Timpano S, Goffredo M, Padoan R, Badolato R

PMID: 32303430 [PubMed - as supplied by publisher]

Blood co-expression modules identify potential modifier genes of diabetes and lung function in cystic fibrosis.

PLoS One. 2020;15(4):e0231285

Authors: Pineau F, Caimmi D, Magalhães M, Fremy E, Mohamed A, Mely L, Leroy S, Murris M, Claustres M, Chiron R, De Sario A

Abstract
Cystic fibrosis (CF) is a rare genetic disease that affects the respiratory and digestive systems. Lung disease is variable among CF patients and associated with the development of comorbidities and chronic infections. The rate of lung function deterioration depends not only on the type of mutations in CFTR, the disease-causing gene, but also on modifier genes. In the present study, we aimed to identify genes and pathways that (i) contribute to the pathogenesis of cystic fibrosis and (ii) modulate the associated comorbidities. We profiled blood samples in CF patients and healthy controls and analyzed RNA-seq data with Weighted Gene Correlation Network Analysis (WGCNA). Interestingly, lung function, body mass index, the presence of diabetes, and chronic P. aeruginosa infections correlated with four modules of co-expressed genes. Detailed inspection of networks and hub genes pointed to cell adhesion, leukocyte trafficking and production of reactive oxygen species as central mechanisms in lung function decline and cystic fibrosis-related diabetes. Of note, we showed that blood is an informative surrogate tissue to study the contribution of inflammation to lung disease and diabetes in CF patients. Finally, we provided evidence that WGCNA is useful to analyze-omic datasets in rare genetic diseases as patient cohorts are inevitably small.

PMID: 32302349 [PubMed - as supplied by publisher]

CT score and correlation with lung function and microbiology of adult patients with cystic fibrosis with predominant I1234V genotype in Qatar.

Qatar Med J. 2020;2020(1):4

Authors: Thomas M, Raja M, Albakri M, Najim M, Chandra P, Allangawi M

Abstract
Background: Computed tomography (CT) features of cystic fibrosis (CF) lung disease can be objectively quantified using current CT scoring systems to assess the extent and severity of the disease. The aims of this study were to calculate the Santamaria CT scores in adult patients with CF with the predominant CFTR I1234V genotype, determine its reliability, and correlate these parameters with lung function, microbial colonization, compliance to treatment, and exacerbations. Methodology: This retrospective observational study was conducted on adult patients with CF who were regularly followed up in the adult CF service at Qatar via CT scans that were taken not during an acute exacerbation. CT scans were scored using the Santamaria scoring system. Corresponding spirometry, microbiological data of sputum culture, and relevant clinical data were correlated with individual CT scores. Results: Only 23 of the 31 patients underwent CT when not in an acute exacerbation and were included in the study analysis. A total of 20 (87%) patients had the I1234V genotype. There was good agreement between the two radiologists on the Santamaria CT scores with an intraclass correlation coefficient (ICC) value of 0.991. Bronchiectasis was the most consistent finding, followed by interlobular and intralobular septal thickening. Patients with poor lung function and frequent exacerbations had significantly higher CT scores (p = 0.015). The CT scores of patients colonized with Pseudomonas aeruginosa were higher but nonsignificant (p = 0.20). The mean CT scores were significantly higher in patients who were noncompliant to regular treatment than in those who were compliant (p = 0.012). Conclusion: Santamaria CT scores comprise a reliable scoring system for adult patients with CF and can be used to determine the extent and severity of lung disease. P. aeruginosa colonization causes more structural lung damage than other common colonizing organisms. Noncompliance to treatment has a significant impact on the increasing severity of CF lung disease.

PMID: 32300549 [PubMed]