Abstract

Cystic Fibrosis (CF) is a genetic disease inherited by an autosomal recessive mechanism and characterized by a progressive and severe multi-organ failure. Mutations in Cystic Fibrosis Conductance Regulator (CFTR) protein cause duct obstructions from dense mucus secretions and chronic inflammation related to organ damage. The progression of the disease is characterized by a decline of lung function associated with metabolic disorders and malnutrition, musculoskeletal disorders and thoracic deformities, leading to a progressive decrement of the individual’s quality of life. The World Health Organization (WHO) qualifies Physical Activity (PA) as a structured activity produced by skeletal muscles’ movements that requires energy consumption. In the last decade, the number of studies on PA increased considerably, including those investigating the effects of exercise on cognitive and brain health and mental performance. PA is recommended in CF management guidelines, since it improves clinic outcomes, such as peripheral neuropathy, oxygen uptake peak, bone health, glycemic control and respiratory functions. Several studies regarding the positive effects of exercise in patients with Cystic Fibrosis were carried out, but the link between the effects of exercise and cognitive and brain health in CF remains unclear. Animal models showed that exercise might improve learning and memory through structural changes of brain architecture, and such a causal relationship can also be described in humans. Indeed, both morphological and environmental factors seem to be involved in exercise-induced neural plasticity. An increase of gray matter volume in specific areas is detectable as a consequence of regular training in humans. Neurobiological processes associated with brain function improvements include biochemical modifications, such as neuromodulator or neurohormone release, brain-derived neurotrophic factor (BDNF) production and synaptic activity changes. From a functional point of view, PA also seems to be an environmental factor enhancing cognitive abilities, such as executive functions, memory and processing speed. This review describes the current state of research regarding the impacts of physical activity and exercise on cognitive functions, introducing a possible novel field of research for optimizing the management of Cystic Fibrosis.

Highlights

  • Cystic Fibrosis (CF) is a congenital systemic disease inherited as an autosomal recessive disorder, with manifestation since birth, characterized by chronic and progressive symptoms [1]

  • The disease is caused by mutations that occur in Cystic Fibrosis Conductance Regulator (CFTR) gene, encoding a protein largely expressed in epithelial cells, which has the role of regulating the chloride efflux across the cellular membrane

  • Frequent disease exacerbations lead to a diffuse sedentariness among CF patients, who show a marked decrease in exercise tolerance, partly related to impaired muscle function and malnutrition, in addition to lung diseases [7]

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Summary

Introduction

Cystic Fibrosis (CF) is a congenital systemic disease inherited as an autosomal recessive disorder, with manifestation since birth, characterized by chronic and progressive symptoms [1]. Rodents housed in an enriched environment with a running wheel and sustained cognitive stimulation showed enhanced learning and memory consolidation These findings in animal studies have raised interesting questions regarding the effect of PA on cognitive and brain health in humans. The aim of the paper is to highlight the existing knowledge about CF and neurocognitive functions impairments, proposing a starting point to speculate about the possibility of developing further studies on the link existing between the effects of PA and the neurocognitive functions of CF patients. These studies may lead to a general improvement of management of CF patients, especially in CF children

Psychological Symptoms and Neuronal Impairment in CF
Role of Physical Activity in Neurocognitive Functions in CF
Findings
Conclusions

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