Bone health and disease in cystic fibrosis

Abstract

Low bone mineral density is common in children and adults with CF. It has a multifactorial aetiology that includes direct effects of CFTR dysfunction on bone cell activity, as well as the secondary effects of CFTR dysfunction including pancreatic insufficiency (leading to malnutrition/malabsorption of fat soluble vitamins) and pulmonary infection (leading to systemic inflammation and increased bone resorption). Strategies to improve bone health in CF include optimising general CF nutritional and pulmonary care and the judicious use of bisphosphonates in selected patients. CFTR correctors/potentiators may have positive impact on bone metabolism in people with CF.

Keywords: Bone; Cystic fibrosis; Osteomalacia; Osteoporosis.