Prevalence and Risk Factors for Low Bone Mineral Density in Adults With Cystic Fibrosis

Boyle, Rebecca L; Psoter, Kevin J.; Merlo, Christian A.; Sidhaye, Aniket; Lechtzin, Noah; Patel, Shivani; Montemayor, Kristina; Horne, A.; West, Natalie E.

doi:10.1002/jbm4.10666

JBMR Plus

2022

DOI: 10.1002/jbm4.10666

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Prevalence and Risk Factors for Low Bone Mineral Density in Adults With Cystic Fibrosis

Rebecca L Boyle

Kevin J. Psoter

Christian A. Merlo

et al.

Abstract: Single-center studies have suggested that up to 70% of adults with cystic fibrosis (CF) have lower than expected bone mineral density (BMD), substantially higher than the 25% prevalence reported from national registries. We determined the prevalence of low BMD in CF adults at our center and assessed risk factors for low BMD. This retrospective cohort study was conducted in all CF patients ≥18 years of age who had a dual-energy X-ray absorptiometry (DXA) scan performed at the Johns Hopkins Adult Cystic Fibrosis… Show more

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Cited by 4 publications

References 30 publications

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Longitudinal changes in BMD in adults with cystic fibrosis

Jad,

Ma,

Stanojevic

et al. 2024

Journal of Bone and Mineral Research

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Improved survival in people with cystic fibrosis (pwCF) presents new complexities of care, including CF-related bone disease, a common complication in older pwCF. The trajectory of bone loss with age in this population remains unclear. The objective of this study was to estimate the average rate of change in BMD in adults with CF. This retrospective study included adults with CF, aged 25-48 yr, followed between January 2000 and December 2021. Subjects with at least one DXA scan were included. Scans obtained posttransplantation, after the initiation of bisphosphonates or cystic fibrosis transmembrane conductance regulator modulator therapy was excluded. The primary outcome was BMD (g/cm2) at the LS and FN. A linear mixed-effects model with both random intercept and random slope terms was used to estimate the average annual change in BMD. A total of 1502 DXA scans in 500 adults (average age 28.4 y) were included. There was a statistically significant annual decline in BMD of −0.008 gm/cm2/yr (95% CI, −0.009 to −0.007) at the FN and −0.006 gm/cm2/yr (95% CI, −0.007 to −0.004) at the LS. Relative to BMD at age 25, there was a 18.8% decline at the FN by age 48 yr and a 11% decline at the LS. Pancreatic insufficient subjects had a faster rate of decline in BMD compared with pancreatic sufficient subjects. After adjusting for markers of disease severity, the annual rate of decline remained significant. Individuals with CF experience bone loss at an age when it is not anticipated, thereby entering early adulthood, where further bone loss is inevitable especially with the decrease in estrogen during menopause, with suboptimal BMD. As the CF population ages, it will become very important to consider interventions to maximize bone health.

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Longitudinal changes in BMD in adults with cystic fibrosis

Jad,

Ma,

Stanojevic

et al. 2024

Journal of Bone and Mineral Research

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Clinical analysis of 1,038 cases of odontogenic jawbone cysts

Du,

Wang,

Lan

et al. 2024

BMC Oral Health

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Associations of the <i>VDR</i> gene with clinical manifestations and complications of cystic fibrosis

Loshkova

Kondratyeva

Zhekaite

et al. 2023

Pulʹmonologiâ (Mosk.)

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Cystic fibrosis (CF) is the most common severe autosomal recessive disease in the Caucasoid population caused by mutations in the CF transmembrane regulator (CFTR) gene. However, the course of the disease may be modulated by genetic factors other than the CFTR gene and may be pleiotropically influenced by VDR (Vitamin D Receptor) gene. The aim of the study was to search for associations between genetic variants (c.1206T>C(A>G), c.152T>C, c.1174+283G>A) of VDR gene and clinically significant manifestations of CF, complications, and responses to therapy. Methods. Patients with CF (n = 283) and healthy children (n = 333), who formed the control group, were examined. Calcidiol levels were tested in all subjects. Polymorphic variants of VDR gene (c.1206T>C(A>G), c.152T>C, c.1174+283G>A) were tested by polymerase chain reaction and restriction fragment length polymorphism analysis. Results. It was found that carriers of the TT genotype of the c.152T>C FokI variant of VDR gene are 6.3 times more likely to develop meconium ileus (odds ratio – OR – 6.375; p = 0.011), 3.2 times more likely – respiratory failure (OR – 3.253; p = 0.079), 3.4 times more likely – chronic lung infection (CIL) caused by Pseudomonas aeruginosa (OR – 3.432; p = 0.026), and 4 times more likely – CIL caused by non-fermenting gram-negative bacteria (OR – 4.056; p = 0.009). Carriers of the CC genotype of the c.1206T>C(A>G) TaqI genetic variant use systemic corticosteroids more frequently (66% vs 7%) (OR – 0.034; p = 0.001). It was shown that the AA genotype of the BsmlI polymorphism (c.1174 + 283G>A) is 4 times more likely to be detected in children with CF-associated liver diseases (OR – 4.300; p = 0.051). Conclusion. The contribution of all studied genetic variants c.1206T>C(A>G) TaqI, c.152T>C FokI, BsmlI (c.1174+283G>A) of the VDR gene to the clinical manifestations, complications and response to therapy in CF is described.

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Prevalence and Risk Factors for Low Bone Mineral Density in Adults With Cystic Fibrosis

Cited by 4 publications

References 30 publications

Longitudinal changes in BMD in adults with cystic fibrosis

Longitudinal changes in BMD in adults with cystic fibrosis

Clinical analysis of 1,038 cases of odontogenic jawbone cysts

Associations of the <i>VDR</i> gene with clinical manifestations and complications of cystic fibrosis

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