How Can We Prevent Multisystem Complications of Cystic Fibrosis?

J. Stuart Elborn

doi:10.1055/s-2007-981651

Seminars in Respiratory and Critical Care Medicine, Inhaltsverzeichnis

Semin Respir Crit Care Med 2007; 28(3): 303-311
DOI: 10.1055/s-2007-981651

How Can We Prevent Multisystem Complications of Cystic Fibrosis?

J. Stuart Elborn¹ ^, ²

¹Respiratory Medicine Group, QUB, Northern Ireland
²Northern Ireland Adult Cystic Fibrosis Centre, BCH, Northern Ireland

Abstract

ABSTRACT

Cystic fibrosis (CF) is a multisystem disease. Some of this is accounted for by CF transmembrane regulator (CFTR) dysfunction in individual organs but in some cases this is compounded by the effects of systemic inflammation. The inflammation is in response to the chronic infection in the airways and is particularly important as a contributor to CF-related bone disease, CF-related diabetes mellitus, CF-related arthropathy, and vasculitis. Preventing these multisystem complications is difficult. Aggressive specific therapies to treat complications is critically important to maintain nutrition, stop the damaging effects of diabetes, and improve bone mineral density. Aggressive treatment of lung disease to reduce systemic inflammation is likely to be of benefit in preventing the development of CF- related bone disease and may be beneficial in at least delaying the onset of CF-related diabetes. Aggressive treatment of pulmonary infection and inflammation in conjunction with holistic management plans to treat specific organ diseases is an important strategy in improving morbidity and reducing mortality in people with CF.

KEYWORDS

Cystic fibrosis - inflammation - osteoporosis - diabetes - liver disease

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Referenzen

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J Stuart ElbornM.D.

Respiratory Medicine Group, QUB and Northern Ireland Adult Cystic Fibrosis Centre

City Hospital, Lisburn Rd., Belfast, BT9 7AB, Ireland

eMail: stuart.elborn@bch.n-i.nhs.uk