Bone mineral density is related to lung function outcomes in young people with cystic fibrosis—A retrospective study

Smith, Nathan; Lim, Alan T.; Yap, Matthew; King, Louise; Liu, James N. K.; Jones, Alicia; Ranganathan, Sarath; Simm, Peter

doi:10.1002/ppul.23894

Cited by 11 publications

(12 citation statements)

References 29 publications

(71 reference statements)

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“…In a retrospective study of 202 children, 30.7% had osteopenia and 15.8% osteoporosis. 26 A meta-analysis of six small studies found that children and adolescents with CF had BMD similar to healthy controls. 4 Interestingly, we found similar BMD in PS and PI patients.…”

Section: Discussionmentioning

confidence: 99%

Understanding the interplay between factors that influence bone mineral density in CF

Gur

Bar‐Yoseph

Diab

et al. 2020

Pediatric Pulmonology

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Background and Objectives Multiple factors affect bone mineral density (BMD) in cystic fibrosis (CF). Our aim was to perform comprehensive analyses of parameters potentially contributing to BMD. Methods A prospective single‐center study assessing BMD, and correlations with multiple parameters including pancreatic status, lung functions, 6‐minute walk test (6MWT), clinical score (modified Shwachman‐Kulczycki [SK] score), vitamin D, nutritional intake, hand grip strength (HGS), habitual physical activity (smart watches), and quality of life (SF‐36 questionnaire). Results Forty CF patients, mean age 18.3 ± 8.1 years, forced expiratory volume in 1 second 74.7% ± 17.9% predicted. Fifteen (37.5%) and 11 (27.5%) had osteopenia and osteoporosis, respectively. BMD was similar in pancreatic sufficient (pancreatic sufficient [PS], n = 15) and insufficient (pancreatic insufficient [PI], n = 25); median hip z score −1.5 ((−2.7)−(+0.2)) vs −1.5 ((−3.5)−(+0.7)), P = .79; spine −0.8 ((−2.2)−(+2)) vs −1.2 ((−4.4)−(+1.5)), P = .39 in PS vs PI, respectively. BMD correlated with HGS (r = .72, P < .001 hip; r = .52, P = .001 spine) and fat‐free mass index (r = .81, P < .001 hip; r = .63, P < .001 spine). BMD z score correlated weakly with SK score and moderately with SF‐36 general health. Data from smart watches, nutrition questionnaires, and 6MWT did not correlate with BMD. In a multivariate model, age and SK score predicted spine z score BMD. Conclusions A substantial number of CF patients have low BMD. Similar rates in PS and PI suggest that other factors, such as disease severity, may contribute to low BMD. SK and age, which can easily be obtained even with limited resources, were the best predictors of low BMD. Further larger multicenter studies are warranted to evaluate the contribution of multifactorial etiologies to low BMD in CF.

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Section: Discussionmentioning

confidence: 99%

Understanding the interplay between factors that influence bone mineral density in CF

Gur

Bar‐Yoseph

Diab

et al. 2020

Pediatric Pulmonology

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“…42,47 A recent study has confirmed that the severity of pulmonary disease is related to low BMD even in young patients. 48 Furthermore, among all patients awaiting LT, the lowest BMD values are reported for patients with CF, 49,50 yet osteoporosis is common to all patients with end-stage lung diseases. [50][51][52] With prolonged immobilization after surgery and high doses of corticosteroids, LT is known to induce severe bone loss in all patients 53 and further reduces the already relatively low BMD values in patients with CF.…”

Section: Me Taboli Cboned Is E a S Ementioning

confidence: 99%

“…Many risk factors have been identified to explain bone loss: chronic lung infection with elevated levels of pro‐inflammatory cytokines, malabsorption leading to vitamin D and K deficiency, hypogonadism and delayed puberty, glucocorticoid therapy, physical inactivity, and low body mass index . A recent study has confirmed that the severity of pulmonary disease is related to low BMD even in young patients . Furthermore, among all patients awaiting LT, the lowest BMD values are reported for patients with CF, yet osteoporosis is common to all patients with end‐stage lung diseases …”

Section: Metabolic Bone Diseasementioning

confidence: 99%

Long‐term extrapulmonary comorbidities after lung transplantation in cystic fibrosis: Update of specificities

Jardel

Reynaud

Durieu

2018

Clinical Transplantation

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Lung transplantation (LT) is the standard therapeutic option for cystic fibrosis (CF) patients with end-stage lung disease. Both conditions lead to extrarespiratory complications, such as diabetes, renal insufficiency, bone disease, and cancer. The purpose of this study was to provide an update of the nonrespiratory comorbidities following LT in adult patients with CF and their specificities regarding their multisystemic underlying condition despite their younger age compared to other patients undergoing LT. Diabetes, renal insufficiency, metabolic bone disease, hypertension, liver disease, and cancer are the comorbidities considered in this review. The increase in CF adults living with a lung transplant justifies an update of knowledge for this specific situation (the prevalence of these complications, underlying risk factors), to provide better medical care and establish early diagnosis strategies.

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“…Our group has previously determined the prevalence of bone disease within a large unbiased population of Australian children and adolescents with CF and described the impact of lung function and body composition on bone health 12 . However, this only looked at cross‐sectional data, and association with lung function, body mass index (BMI), and genotype.…”

Section: Introductionmentioning

confidence: 99%

The clinical features that contribute to poor bone health in young Australians living with cystic fibrosis: A recommendation for BMD screening

Atlas

Yap

Lim

et al. 2021

Pediatric Pulmonology

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Background For Australians living with cystic fibrosis (CF), increased longevity means greater consideration needs to be given to long‐term endocrine sequelae such as CF‐related bone disease. Deficits in bone mass accrual are most likely to occur during childhood and adolescence. Current guidelines in Australia suggest repeat dual‐energy X‐ray absorptiometry (DXA) scans every 2 years. This study aims to stratify clinical factors that determine future bone health in the Australian CF population and use this to guide a more streamlined approach to bone health screening. Methods This study was a retrospective audit of all patients diagnosed with CF who were treated at the Royal Children's Hospital Melbourne, Australia from 2000 to 2016 (n = 453). Two hundred and two patients had a DXA scan in the study period (191 with height‐adjusted data) and 111 patients had more than one scan (108 with height‐adjusted data). An investigation into the associations between bone mineral density (BMD) Z score and potential risk factors was conducted using DXA and historical data. Results The main predictor of future BMD was the previous BMD Z score (p < .001). Other factors found to be determinants of BMD included nutritional status, lung function (FEV1), age, history of previous fracture, oral corticosteroid use, and the number of hospital admissions. However, after adjusting for previous BMD, evidence of an association remained only with nutritional status, FEV1, and number of hospital admissions. Conclusion Second yearly scans may be unnecessary in children with an adequate DXA score on the initial scan who remain clinically stable. However, clinical deterioration in those whose BMD was previously normal, may require closer monitoring of bone health. We propose a guideline for the frequency of DXA monitoring in relation to clinical risk factors.

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Bone mineral density is related to lung function outcomes in young people with cystic fibrosis—A retrospective study

Abstract: This is the biggest study to date to provide further evidence that the severity of pulmonary disease is related to BMD in CF patients and therefore screening guidelines for bone health in children with CF should target individuals with the poorest clinical status.

Cited by 11 publications

References 29 publications

Understanding the interplay between factors that influence bone mineral density in CF

Understanding the interplay between factors that influence bone mineral density in CF

Long‐term extrapulmonary comorbidities after lung transplantation in cystic fibrosis: Update of specificities

The clinical features that contribute to poor bone health in young Australians living with cystic fibrosis: A recommendation for BMD screening

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