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The objective of this research is to determine whether there is a difference in the Ca, P, Fe, F, Mg, and Sr concentrations in teeth from children with and without cystic fibrosis (CF) as a function of age, sex of the child, maternal smoking habit, maternal drinking habit, and fluoridation of water supply. The nuclear analytical methods used were Proton-Induced X-Ray Emission (PIXE) and Proton-Induced Gamma Emission (PIGE). The findings are: (1) the concentration of Mg is higher in the teeth of children with cystic fibrosis who took antibiotics other than tetracycline (CF+NT) than in non-cystic fibrosis (NCF) teeth for the “female” category; (2) P concentration is less in (CF+NT) teeth for the “age greater than ten” group; (3) Fe concentration is greater for females in the NCF group; (4) Ca and Fe concentrations are lower for CF than for NCF in the “mother smokes” group; (5) F and Sr concentrations for CF+NT are higher for the “mother drinks” group than for the “mother does not drink” group; (6) “mother smokes” shows a depletion of Ca and Fe compared to “mother does not smoke” for CF+NT. (7) “fluoridation of water supply” shows an increase in F, except for NCF teeth.

Cystic fibrosis is the most commonly occurring genetic disorder among Caucasians. The purpose of this study was to evaluate the effects of acupuncture for pain management in patients with cystic fibrosis; clinical data was obtained regarding the integration of acupuncture into the management of pain in patients with cystic fibrosis. Visual analog scales were used for pain measurement. Acupuncture was found to be effective in decreasing pain complaints in patients with cystic fibrosis. No side effects or complications were reported in relation to the acupuncture treatment. Acupuncture is effective for managing symptoms and illnesses associated with cystic fibrosis. Further randomized controlled trials will be necessary to evaluate additional efficacy in pain management and the improvement of the quality of life of patients with cystic fibrosis.

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Background: Autonomic nervous system balance is altered in cystic fibrosis (CF), although its influence on physical fitness has been poorly explored.

Objective: This study aimed to evaluate the association of heart rate variability (HRV) with exercise capacity and levels of daily physical activity in children and adolescents with mild-to-moderate CF.

Methods: A cross-sectional study including individuals with CF aged 6–18 years, not under CFTR modulator therapy, was performed. Sociodemographic (age, sex) and clinical information (airway colonization, pancreatic insufficiency, and genotyping) were collected. In addition, exercise capacity (modified shuttle test — MST), lung function (spirometry), body composition (bioimpedance), levels of daily physical activity (5-day accelerometer), and HRV (both at rest and during the MST) were evaluated.

Results: 30 individuals (20 females) aged $11.2\pm 3.7$ years, mean FEV${}_{1}62.8\pm 27.6$%, were included. A sympathovagal balance (LF/HF) increase ($p<0.001$) during the MST was shown, indicating a predominance of sympathetic modulation. The standard deviation of all RR intervals (SDNN) and the high frequency (HF) index during exercise correlated significantly with FEV₁ ($r=0.45$, $p=0.01$ and $r=0.46$, $p=0.01$; respectively). MST distance also correlated positively and significantly with SDNN ($r=0.43$, $p=0.01$), square root of the mean of the sums of squares of frequencies between RR intervals greater than 50ms — RMSSD ($r=0.53$, $p<0.01$), low frequency — LF ($r=0.48$, $p<0.01$), HF ($r=0.64$, $p<0.01$), dispersion of points perpendicular to the short-term identity line — SD1 ($r=0.40$, $p=0.02$) and negatively with LF/HF ($r=-0.57$, $p<0.01$). Regarding daily physical activity, SDNN at rest ($r=0.37$, $p=0.04$) and exercise ($r=0.41$, $p=0.02$) showed positive correlations with time in moderate-to-vigorous activities. When normalizing the SDNN and classifying individuals as normal or altered, those presenting altered SDNN showed poorest FEV₁ ($p=0.001$) and lower exercise capacity ($p=0.027$).

Conclusion: HRV correlates with lung function, exercise capacity and levels of daily physical activity in children and adolescents with CF. The study highlights the influence of CF on autonomic function and suggests HRV measurement as an easy tool to be used in clinical settings as an alternative marker to monitor CF individuals.

There has been considerable research on harnessing the unique properties of stem and progenitor cells to develop more effective therapeutic approaches for respiratory diseases with limited treatment options. Cell-based gene therapy, combined with genetic engineering to enhance regenerative cell function, can be a powerful strategy able to provide additional benefits, and may overcome many of the limitations of cell or gene therapy alone. This dual strategy not only allows the direct targeting of cells to the lung for regenerative interventions, but also provides a site-specific source to release therapeutic proteins to the lung microvasculature or alveoli. This review discusses relevant literature on the use of stem/progenitor cells-based gene therapy for the treatment of acute and chronic pulmonary disorders, including acute lung injury, pulmonary arterial hypertension, emphysema, cystic fibrosis, and cancer therapy for lung metastasis. Different types of stem and progenitor cells such as mesenchymal stem cells or endothelial progenitor cells, which have been used in these therapeutic approaches, will also be covered.

Cystic fibrosis (CF) remains a devastating and incurable disease. One of the first targets for lung gene therapy and studies of lung gene transfer, early promising results were achieved in vitro and in small animal models. However, studies in primate models and in patients were discouraging, despite a large number of clinical trials, and gene therapy approaches for CF have generally fallen out of favour. Newer approaches with cell-based therapy, utilizing either embryonic stem cells or extra embryonic-derived adult stem cells (bone marrow or cord blood), have been investigated recently and may provide viable future therapeutic options. In parallel, further understanding of the role of endogenous progenitor cells in CF lungs may also provide both mechanistic understanding and potential therapeutic approaches for CF lung disease. In this chapter, endogenous lung progenitors in CF and the potential use of cell therapy-based approaches for CF will be considered.

The availability of multiple publicly-available datasets studying the same phenomenon has the promise of accelerating scientific discovery. Meta-analysis can address issues of reproducibility and often increase power. The promise of meta-analysis is especially germane to rarer diseases like cystic fibrosis (CF), which affects roughly 100,000 people worldwide. A recent search of the National Institute of Health’s Gene Expression Omnibus revealed 1.3 million data sets related to cancer compared to about 2,000 related to CF. These studies are highly diverse, involving different tissues, animal models, treatments, and clinical covariates. In our search for gene expression studies of primary human airway epithelial cells, we identified three studies with compatible methodologies and sufficient metadata: GSE139078, Sala Study, and PRJEB9292. Even so, experimental designs were not identical, and we identified significant batch effects that would have complicated functional analysis. Here we present quantile discretization and Bayesian network construction using the Hill climb method as a powerful tool to overcome experimental differences and reveal biologically relevant responses to the CF genotype itself, exposure to virus, bacteria, and drugs used to treat CF. Functional patterns revealed by cluster Profiler included interferon signaling, interferon gamma signaling, interleukins 4 and 13 signaling, interleukin 6 signaling, interleukin 21 signaling, and inactivation of CSF3/G-CSF signaling pathways showing significant alterations. These pathways were consistently associated with higher gene expression in CF epithelial cells compared to non-CF cells, suggesting that targeting these pathways could improve clinical outcomes. The success of quantile discretization and Bayesian network analysis in the context of CF suggests that these approaches might be applicable to other contexts where exactly comparable data sets are hard to find.