Evaluation of bone disease in patients with cystic fibrosis and end-stage lung disease

Abstract

Objective: Bone disease is a common comorbidity in patients with cystic fibrosis (CF). We sought to determine risk factors and identify potential biochemical markers for CF-related bone disease (CFBD) in a unique cohort of CF patients with end-stage lung disease undergoing lung transplantation (LTx) evaluation. Methods: All of the CF patients who were evaluated for LTx at our center between November of 1992 and December of 2010 were included in the study. Clinical data and biochemical markers of bone turnover, as well as bone mineral density (BMD) at the lumbar spine and femoral neck, were evaluated. Spearman’s rho and multivariate logistic regression analysis were used. Results: A total of 102 adult CF patients were evaluated. The mean age was 28.1 years (95% CI: 26.7-29.5), and the mean body mass index was 17.5 kg/m2 (95% CI: 17.2-18.2). Mean T-scores were −2.3 and −1.9 at the lumbar spine and femoral neck, respectively, being lower in males than in females (−2.7 vs. −2.0 at the lumbar spine and −2.2 vs. −1.7 at the femoral neck). Overall, 52% had a T-score of < −2.5 at either skeletal site. The homozygous Phe508del genotype was found in 57% of patients without osteoporosis and in 60% of those with low BMD. Mean T-scores were not particularly low in patients with severe CFTR mutations. Although the BMI correlated with T-scores at the femoral neck and lumbar spine, serum 25-hydroxyvitamin D and parathyroid hormone levels did not. Conclusions: CFBD is common in CF patients with end-stage lung disease, particularly in males and patients with a low BMI. It appears that CF mutation status does not correlate with CFBD. In addition, it appears that low BMD does not correlate with other risk factors or biochemical parameters. The prevalence of CFBD appears to have recently decreased, most likely reflecting increased efforts at earlier diagnosis and treatment.

Calcium, Phosphorus, and Vitamin D Levels in a Series of Cystic Fibrosis Patients: A Cross-Sectional Study

“…In the pediatric stage, they play an indispensable role in their growth and development [12]. Vitamin D regulates Ca and P homeostasis [17], and its deficiency leads to secondary PTH [18,108]. In our series, there were no patients with stunted growth [109], despite 12% having undernutrition based on BMI.…”

Risk factors and prognostic value of osteoporosis in hospitalized patients with bronchiectasis

“…Among asthmatic patients, osteopenia, osteoporosis, and pathologic fractures were identified as risk factors for emergency department visits [ 21 ]. In cystic fibrosis (CF) patients with end-stage lung disease, CF-related bone diseases such as decreased bone mineral density were also commonly detected [ 22 ]. There has even been a dicey combination of malignancy (lung cancer) and osteoporosis [ 23 ].…”

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

“…However, low BMD is important to treat in CF adults because it is associated with higher morbidity ( 11 , 12 ) and can lead to significant challenges if patients need future lung transplantation. ( 13 ) CFLBMD can be a relative contraindication to lung transplantation given post‐transplant treatment includes long‐term glucocorticoids, which can worsen BMD. With improved survival, people with CF are living longer, and appropriate attention should be paid to diagnosis and management of lower than expected BMD.…”

“…( 10 ) Low BMD is of particular concern in CF because increased risk of rib ( 11 ) and spine ( 12 ) fractures can lead to reduced airway clearance and exercise ability, as well as chronic pain. In addition, low BMD can lead to significant challenges in people with CF requiring lung transplantation ( 13 ) as transplantation medications, such as glucocorticoids, frequently lead to progressive worsening of BMD status. ( 14 , 15 ) Given the morbidity associated with low BMD, additional data on the true prevalence of low BMD in CF adults are needed to better manage the manifestations of low BMD.…”