Recurrent pulmonary exacerbations are associated with low fat free mass and low bone mineral density in young adults with cystic fibrosis

Alicandro, Gianfranco; Bisogno, Arianna; Battezzati, Alberto; Bianchi, Maria Luisa; Corti, Fabiola; Colombo, Carla

doi:10.1016/j.jcf.2013.11.001

Cited by 39 publications

(73 citation statements)

References 29 publications

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“…Forty‐one studies (33.1%) recruited adult patients aged between 18 and 57 years and a total number of 1983 patients with CF. Only 15 studies included patients older than 40 years . Ten studies (8.1%) included both children and adults (range: 4–66 years, N = 768).…”

Section: Resultsmentioning

confidence: 99%

Tools and Methods Used for the Assessment of Body Composition in Patients With Cystic Fibrosis: A Systematic Review

et al. 2019

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Background Cystic fibrosis (CF) is characterized by changes in fat mass and lean body mass that may have important prognostic value. We aim to appraise the type and frequency of application of body composition (BC) methods in child and adult patients with CF. Methods We used 4 databases (Embase, PubMed, Scopus, and Web of Science) to perform the literature search. The search was conducted from January 2017 to February 2017. Two independent reviewers selected articles based on titles and abstracts to check eligibility for inclusion. All study designs or types of articles (abstract, full text) were considered. Results Eighty‐four full‐text articles and 40 studies presented only as abstracts were selected. Sixty‐four studies included children and adolescents (age range of 0.1–18 years), and 41 studies recruited adults (range of 18–57 years); 13 studies included both age groups. Dual‐energy X‐ray absorptiometry (DXA) was used in 56 studies (33.9%), and bioelectric impedance analysis (BIA) was used in 12 studies (9.7%), whereas 38 studies (30.6%) combined different methods (up to 5 different methods) to assess BC. Conclusions The results show a large variability in the application of BC methods in patients with CF that makes the comparison between studies difficult. The only methods with a sufficient body of literature are DXA and BIA.

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Section: Resultsmentioning

confidence: 99%

Tools and Methods Used for the Assessment of Body Composition in Patients With Cystic Fibrosis: A Systematic Review

et al. 2019

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“…Lower bone strength and quality not only increases the risk of clinical fractures but also has been associated with lower lung function in cross-sectional studies 42, 43 . Furthermore, lower BMD has been associated with recurrent pulmonary exacerbation and mortality in children with CF 44, 45 .…”

Section: Cystic Fibrosis Related Bone Diseasementioning

confidence: 99%

Endocrine Disorders in Cystic Fibrosis

Blackman

Tangpricha

2016

Pediatric Clinics of North America

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Summary Cystic fibrosis can cause multiple endocrine disorders including diabetes, bone disease, short stature, and male hypogonadism. The etiologies are multifactorial but may be directly related in some cases to CFTR dysfunction. Diabetes is the most common endocrine complication and can be expected to affect in almost all people with pancreatic insufficient CF but varies widely in its age of onset. Early identification and treatment of diabetes improves morbidity and mortality in CF. Short stature during adolescence can be transient if puberty is later than average, but it can also be pathologic. Inhaled and systemic steroids can slow growth and should be used at the minimum effective doses. Bone disease in CF is a frequent cause of fragility fractures and associates with worse lung disease. Bone mineral density and vitamin D levels should be monitored. Hypogonadism has been reported in 25% of men with CF. Endocrine complications can cause morbidity and mortality in CF and need to be appropriately detected and managed as part of the medical care of individuals with CF. These complications tend to occur more frequently in older individuals and may be expected to become more common as the CF population grows older.

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“…(19). Furthermore, BMD has been positively associated with pulmonary and physical function, nutritional status and negatively associated with chronological age and recurrent pulmonary exacerbations (20). Mutation of the CFTR gene itself may have a direct role in the pathogenesis of CF-related bone disease (17).…”

Section: Cystic Fibrosismentioning

confidence: 99%

Rare causes of osteoporosis

Marcucci

Brandi

2015

ccmbm

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SummaryOsteoporosis is a metabolic bone disease characterized by loss of bone mass and strength, resulting in increased risk of fractures. It is classically divided into primary (postmenopausal or senile), secondary and idiopathic forms. There are many rare diseases, that cause directly or indirectly osteoporosis. The identification and classification of most of these rare causes of osteoporosis is crucial for the specialists in endocrinology and not, in order to prevent this bone complication and to provide for an early therapy. Several pathogenic mechanisms are involved, including various aspects of bone metabolism such as: decreased bone formation, increased bone resorption, altered calcium, phosphorus and/or vitamin D homeostasis, and abnormal collagen synthesis. In this review, less common forms of primary and secondary osteoporosis are described, specifying, if applicable: genetic causes, epidemiology, clinical features, and pathogenic mechanisms causing osteoporosis. A greater awareness of all rare causes of osteoporosis could reduce the number of cases classified as idiopathic osteoporosis and allow the introduction of appropriate and timely treatments.

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Recurrent pulmonary exacerbations are associated with low fat free mass and low bone mineral density in young adults with cystic fibrosis

Abstract: Recurrent pulmonary exacerbations are associated with reduced appendicular FFM and bone mineral density in young male adults with CF. The gender-dependent relationship between pulmonary exacerbations and body composition alteration needs to be further investigated.

Cited by 39 publications

References 29 publications

Tools and Methods Used for the Assessment of Body Composition in Patients With Cystic Fibrosis: A Systematic Review

Tools and Methods Used for the Assessment of Body Composition in Patients With Cystic Fibrosis: A Systematic Review

Endocrine Disorders in Cystic Fibrosis

Rare causes of osteoporosis

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