Abstract
Cystic fibrosis (CF) is an autosomal recessive multiorgan disease that is caused by mutations in a gene, cystic fibrosis transmembrane conductance regulator (CFTR). A host of epidemiology has demonstrated a “gender gap” or sex-based disparity in outcomes of people with CF, where females have more pulmonary exacerbations and a shortened life expectancy relative to males. The etiology of this disparity is not fully elucidated but appears to be multifactorial. In this chapter, we will review the sex-based differences in diagnosis of disease, pulmonary and extrapulmonary manifestations of CF, and host immune response as it relates to puberty, reproductive year, and menopause. We will also review the role of sex hormones on the sex disparity and the proposed mechanisms of how hormones such as estrogen, progesterone, and testosterone contribute to the differing outcomes.
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Bozkanat, K.M., Jain, R. (2021). Sex Differences in Cystic Fibrosis Across the Lifespan. In: Silveyra, P., Tigno, X.T. (eds) Sex-Based Differences in Lung Physiology. Physiology in Health and Disease. Springer, Cham. https://doi.org/10.1007/978-3-030-63549-7_6
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