Cystic Fibrosis

Zemanick, Edith T.; Hoffman, Lucas R.

doi:10.1016/j.pcl.2016.04.003

Cited by 40 publications

(22 citation statements)

References 106 publications

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“…Therefore, vitamin D deficiency, common in CF patients due to malabsorption, can influence immune response, and has been associated with inflammatory markers, bacterial infection, and pulmonary exacerbation …”

Section: Introductionsupporting

confidence: 71%

Lung disease and vitamin D levels in cystic fibrosis infants and preschoolers

Oliveira¹,

Matsunaga²,

Rodrigues³

et al. 2019

Pediatric Pulmonology

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Introduction: Vitamin D acts on the immune system and lung response. Patients with cystic fibrosis (CF) may be deficient in this vitamin. The aims of the study were to evaluate vitamin D levels and severity of lung disease in infants and preschoolers diagnosed with CF, and to compare them to a group of children without pancreatic insufficiency (PI). Methods: Patients with CF up to 4 years old were included, and compared to an agematched group of children without diagnosis of CF. CF group had medical records and High Resolution Thorax Computed Tomography (HRCCT) evaluated in order to verify the severity of lung disease. Information on demographic data, sun exposure habits, supplemental vitamin D therapy, and on the season at the time of vitamin D sampling were collected for both groups.Results: This study included 45 patients in the CF group and 102 in the non-CF group, with no differences in age (P = 0.327) between them. There was no association between vitamin D levels and markers of lung disease in the CF group. The non-CF group had lower daily sun exposure (P = 0.034), and lower supplementation than the CF group (P < 0.001). Supplementation and seasonality were the determinant variables for vitamin D levels, which were lower for non-supplemented children and for assessments during fall/winter. Conclusion:There was no association between lung disease severity and vitamin D levels in CF group. Supplementation and seasonality were associated to higher vitamin levels. K E Y W O R D Scystic fibrosis, infants and preschoolers, lung disease, neonatal screening, vitamin D

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Section: Introductionsupporting

confidence: 71%

Lung disease and vitamin D levels in cystic fibrosis infants and preschoolers

Oliveira¹,

Matsunaga²,

Rodrigues³

et al. 2019

Pediatric Pulmonology

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“…Progressive airways disease due to persistent and recurrent bacterial infection is the primary cause of morbidity and mortality in patients with cystic fibrosis (CF) (Ratjen et al, 2015). Accordingly, many studies have investigated infection dynamics and identified associations between infections with several specific organisms and a poor prognosis (Zemanick and Hoffman, 2016). Not surprisingly, the focus of many such studies has been on "classical" CF pathogens -prevalent organisms such as Pseudomonas aeruginosa, Staphylococcus aureus, and the Burkholderia cepacia complex.…”

Section: Introductionmentioning

confidence: 99%

Epidemiology of E. coli in Cystic Fibrosis Airways Demonstrates the Capacity for Persistent Infection but Not Patient-Patient Transmission

et al. 2020

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Escherichia coli is frequently isolated from the respiratory secretions of cystic fibrosis (CF) patients yet is not considered a classical CF pathogen. Accordingly, little is known about the natural history of this organism in the CF airways, as well as the potential for patientto-patient transmission. Patients attending the Calgary Adult CF Clinic (CACFC) between January 1983 and December 2016 with at least one E. coli-positive sputum culture were identified by retrospective review. Annual E. coli isolates from the CACFC biobank from each patient were typed by pulsed-field gel electrophoresis (PFGE) and isolates belonging to shared pulsotypes were sequenced. Single nucleotide polymorphism (SNP) and phylogenetic analysis were used to investigate the natural history of E. coli infection and identify potential transmission events. Forty-five patients with E. coli-positive sputum cultures were identified. Most patients had a single infection episode with a single pulsotype, while replacement of an initial pulsotype with a second was observed in three patients. Twenty-four had E. coli recovered from their sputum more than once and 18 patients had persistent infections (E. coli carriage >6 months with ≥3 positive cultures). Shared pulsotypes corresponded to known extraintestinal pathogenic E. coli strains: ST-131, ST-73, and ST-1193. Phylogenetic relationships and SNP distances among isolates within shared pulsotypes were consistent with independent acquisition of E. coli by individual patients. Most recent common ancestor date estimates of isolates between patients were inconsistent with patient-to-patient transmission. E. coli infection in CF is a dynamic process that appears to be characterized by independent acquisition within our patient population and carriage of unique sets of strains over time by individual patients.

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“…Important organisms related to chronic pulmonary infections in CF patients are Staphylococcus aureus and Pseudomonas aeruginosa (5,6). They can persist for a long time, i.e., 6 to 70 months, in the CF lung and thereby adapt to their specific environ-ment (7,8).…”

mentioning

confidence: 99%

Bactericidal and Fungicidal Activity of N -Chlorotaurine Is Enhanced in Cystic Fibrosis Sputum Medium

Gruber

Moser

Nagl

et al. 2017

Antimicrob Agents Chemother

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Lung infections with multiresistant pathogens are a major problem among patients suffering from cystic fibrosis (CF). N-Chlorotaurine (NCT), a microbicidal active chlorine compound with no development of resistance, is well tolerated upon inhalation. The aim of this study was to investigate the in vitro bactericidal and fungicidal activity of NCT in artificial sputum medium (ASM), which mimics the composition of CF mucus. The medium was inoculated with bacteria (Staphylococcus aureus, including some methicillin-resistant S. aureus [MRSA] strains, Pseudomonas aeruginosa, and Escherichia coli) or spores of fungi (Aspergillus fumigatus, Aspergillus terreus, Candida albicans, Scedosporium apiospermum, Scedosporium boydii, Lomentospora prolificans, Scedosporium aurantiacum, Scedosporium minutisporum, Exophiala dermatitidis, and Geotrichum sp.), to final concentrations of 10 7 to 10 8 CFU/ml. NCT was added at 37°C, and time-kill assays were performed. At a concentration of 1% (10 mg/ml, 55 mM) NCT, bacteria and spores were killed within 10 min and 15 min, respectively, to the detection limit of 10 2 CFU/ml (reduction of 5 to 6 log 10 units). Reductions of 2 log 10 units were still achieved with 0.1% (bacteria) and 0.3% (fungi) NCT, largely within 10 to 30 min. Measurements by means of iodometric titration showed oxidizing activity for 1, 30, 60, and Ͼ60 min at concentrations of 0.1%, 0.3%, 0.5%, and 1.0% NCT, respectively, which matches the killing test results. NCT demonstrated broad-spectrum microbicidal activity in the milieu of CF mucus at concentrations ideal for clinical use. The microbicidal activity of NCT in ASM was even stronger than that in buffer solution; this was particularly pronounced for fungi. This finding can be explained largely by the formation, through transhalogenation, of monochloramine, which rapidly penetrates pathogens.

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Cystic Fibrosis

Cited by 40 publications

References 106 publications

Lung disease and vitamin D levels in cystic fibrosis infants and preschoolers

Lung disease and vitamin D levels in cystic fibrosis infants and preschoolers

Epidemiology of E. coli in Cystic Fibrosis Airways Demonstrates the Capacity for Persistent Infection but Not Patient-Patient Transmission

Bactericidal and Fungicidal Activity of N -Chlorotaurine Is Enhanced in Cystic Fibrosis Sputum Medium

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