Nebulizer cleaning and disinfection practices in families with cystic fibrosis: The relationship between attitudes, practice and microbe colonization

Abstract Context Early glucose abnormalities in people with CF (PwCF) are commonly detected by continuous glucose monitoring (CGM). Relationships between these CGM abnormalities and oral glucose tolerance testing (OGTT) in PwCF have not been fully characterized. Objective(s) 1) To determine the relationship between CGM and common OGTT-derived estimates of β-cell function, including C-peptide index and oral disposition index (oDI) and 2) to explore whether CGM can be used to screen for OGTT-defined prediabetes and cystic fibrosis related diabetes (CFRD). Study Design/Methods PwCF not on insulin and healthy controls ages 6-25 yrs were enrolled in a prospective study collecting OGTT and CGM. A subset underwent frequently-sampled OGTTs (fsOGTT) with 7-point glucose, insulin, and C-peptide measurements. Pearson’s correlation coefficient was used to test the association between select CGM and fsOGTT measures. ROC analysis was applied to CGM variables to determine the cutoff optimizing sensitivity and specificity for detecting prediabetes and CFRD. Results A total of 120 participants (controls=35, CF=85), including 69 with fsOGTTs, were included. CGM coefficient of variation correlated inversely with C-peptide index (Cpeptide30-Cpeptide0/Glucose30-Glucose0) (r=-0.45, p<0.001) and oDIcpeptide (C-peptide index)(1/cpep0) (r=-0.48, p<0.0001). In PwCF, CGM variables had ROC-AUCs ranging from 0.43-0.57 for prediabetes and 0.47-0.6 for CFRD. Conclusions Greater glycemic variability on CGM correlated with reduced β-cell function. However, CGM performed poorly at discriminating individuals with and without OGTT-defined CFRD and prediabetes. Prospective studies are now needed to determine how well the different tests predict clinically-relevant non-glycemic outcomes in PwCF.

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The Relationship between Genotype and Exercise Tolerance in Children with Cystic Fibrosis

American Journal of Respiratory and Critical Care Medicine ◽

10.1164/ajrccm.165.6.2104036 ◽

2002 ◽

Vol 165 (6) ◽

pp. 762-765 ◽

Cited By ~ 43

Author(s):

HIRAN C. SELVADURAI ◽

KAREN O. McKAY ◽

CAMERON J. BLIMKIE ◽

PETER J. COOPER ◽

CRAIG M. MELLIS ◽

...

Keyword(s):

Cystic Fibrosis ◽

Exercise Tolerance ◽

The Relationship

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Inflammation in the lungs of patients diagnosed with Cystic Fibrosis: Association with iron deficiency

SURG Journal ◽

10.21083/surg.v2i1.809 ◽

1969 ◽

Vol 2 (1) ◽

pp. 3-10

Author(s):

Dawn Armstrong ◽

Lindsay Robinson

Keyword(s):

Cystic Fibrosis ◽

Iron Deficiency ◽

Inflammatory Cytokines ◽

Wide Spectrum ◽

Iron Status ◽

Tissue Destruction ◽

Hypoxic Conditions ◽

Mediators Of Inflammation ◽

The Relationship

Cystic Fibrosis (CF) is characterized by a wide spectrum of phenotypic characteristics such as; deep coughing, increased mucous production, and weight loss. However, only recently was the role of inflammation on the etiology of the disease recognized. CF is characterized as a cyclic progression of infective exacerbations and stable periods initiated by the presence of Pseudomonas Aeruginosa (PA). An increase in inflammatory cytokines/mediators and a decrease in anti-inflammatory cytokines contribute to the net inflammation and overall tissue destruction of the lungs. PA is associated with the low iron status that is seen in 60-75% of the CF population, through the presence of iron sequestering siderophores which distract iron from the tissues. Iron deficiency (ID) initiates further symptoms such as; fatigue, tachycardia, weakness, brittle nails etc, in addition to those caused by CF. The colonization of PA may be the cause or a result of increased iron (ferritin) concentrations in the lungs, but independent of the original relationship, results in a decreased iron status. Iron is used by PA under hypoxic conditions such as in the fibrosis lung, as a source of energy. Studies on the relationship between CF and ID contribute a variety of possible causes although currently no direct connection has been discovered. At this stage, further studies in this area are needed. This review will primarily focus on the affects of CF on iron status in humans, and secondarily examine the effect of mediators of inflammation in respects to ID.

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The relationship between infection and inflammation in the early stages of lung disease from cystic fibrosis

Pediatric Pulmonology ◽

10.1002/ppul.1950200203 ◽

1995 ◽

Vol 20 (2) ◽

pp. 63-70 ◽

Cited By ~ 226

Author(s):

K. Balough ◽

M. McCubbin ◽

M. Weinberger ◽

W. Smits ◽

R. Ahrens ◽

...

Keyword(s):

Cystic Fibrosis ◽

Lung Disease ◽

Early Stages ◽

Infection And Inflammation ◽

The Relationship

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Investigating the Measurement of Physical Activity and Associated Factors in Youth and Adults with Cystic Fibrosis

10.23889/suthesis.58596 ◽

2021 ◽

Author(s):

◽

Mayara S. Bianchim

Keyword(s):

Physical Activity ◽

Cystic Fibrosis ◽

Lung Function ◽

Vigorous Physical Activity ◽

The Body ◽

Machine Learning Algorithms ◽

Cut Points ◽

Measurement And Analysis ◽

The Relationship ◽

Crucial Part

Cystic Fibrosis (CF) is a multisystemic condition that affects almost every organ in the body, but especially the lungs. Regular physical activity (PA) can significantly slow disease progression and has become a crucial part of CF care. Previous research evaluating PA in CF has been hindered by the use of cut-points developed for healthy populations and the investigation of collinear movement behaviours as independent entities, both of which are likely to have confounded their findings and any subsequent inferences regarding associated health outcomes. Therefore, the overall aim of this thesis was to investigate the measurement and analysis of PA in those with CF. An initial systematic review provided recommendations for research calibrating accelerometry in paediatric clinical populations, highlighting that the pathophysiology of the condition must be accounted for and that the protocol should include a broad range of activities varying in intensity (Chapter 4). Subsequently, Chapter 5 developed and cross-validated raw acceleration CF-specific cut-points in youth which were then further assessed in Chapter 6, demonstrating that the CF-specific thresholds were associated with higher levels of moderate-to-vigorous physical activity (MVPA) and sedentary time (SED) and lower levels of light PA compared to generic cut-points. Furthermore, lung function was associated with light PA when using condition-specific thresholds. Further investigation of the relationship between PA and health in Chapter 7 found that reallocating time from sedentary to any other behaviour was beneficial for lung function, with the greatest improvements observed when SED was reallocated to sleep or MVPA. Finally, Chapter 8 developed and validated machine learning algorithms that achieved excellent accuracy to classify PA types and intensities in youth with CF. In conclusion, these findings significantly advance the assessment of PA, enhancing our understanding of the relationship between PA and health in CF and informing future condition-specific PA guidelines, care strategies and interventions.

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Experience of adults with cystic fibrosis: a perspective based on social phenomenology

Revista Brasileira de Enfermagem ◽

10.1590/0034-7167-2017-0749 ◽

2018 ◽

Vol 71 (6) ◽

pp. 2891-2898

Author(s):

Samara Macedo Cordeiro ◽

Maria Cristina Pinto de Jesus ◽

Renata Evangelista Tavares ◽

Deise Moura de Oliveira ◽

Miriam Aparecida Barbosa Merighi

Keyword(s):

Cystic Fibrosis ◽

Life History ◽

Qualitative Study ◽

Health Professionals ◽

Daily Life ◽

The Social ◽

The World ◽

Social Prejudice ◽

The Relationship ◽

Social Phenomenology

ABSTRACT Objective: To understand the experience of adults living with cystic fibrosis. Method: A qualitative study based on the social phenomenology by Alfred Schütz, carried out with 12 adults interviewed in 2016. The statements were analyzed and organized into concrete categories. Results: The following categories were evidenced: “The biopsychosocial impact of the disease on daily life”, “Social prejudice as a generator of embarrassment”, “Coping strategies” and “Fear, uncertainties and the desire to carry out life projects”. Final considerations: The understanding of the experience lived by adults with cystic fibrosis allowed unveiling intersubjective aspects experienced by this public that should be considered by health professionals in the care of this group. It is up to the professionals involved in assisting these people to develop care strategies aimed at completeness, respect for the world of meanings of each individual, their life history, and intersubjectivity that is specially built in the relationship between professionals and people with cystic fibrosis.

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Quantification of chloride in sweat with the Cystic Fibrosis Indicator System

Clinical Chemistry ◽

10.1093/clinchem/36.1.96 ◽

1990 ◽

Vol 36 (1) ◽

pp. 96-98 ◽

Cited By ~ 4

Author(s):

W J Warwick ◽

L G Hansen ◽

M E Werness

Keyword(s):

Cystic Fibrosis ◽

High Resolution ◽

Chloride Concentration ◽

Indicator System ◽

Sweat Test ◽

The Relationship ◽

Estimation Program ◽

Ibm Pc

Abstract We examined the relation between chloride concentration and the area of complexed chloride of Medtronic's Cystic Fibrosis Indicator System, using a high-resolution x-y coordinated digitizer to measure the circumference of the chloride precipitation ring. These digitized points were entered directly into an IBM PC computer, where the area of the chloride precipitation was calculated with use of a repetitive rectangular estimation program. Using these data, we determined the relationship between the area of chloride precipitation and the chloride concentration of the standard NaCl solutions. When the area of the ring of chloride precipitation in the system's patch is measured immediately after the sweat test is completed, the concentration of chloride in the sweat can be calculated with a reproducibility equal to that of the Gibson-Cooke sweat test.

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