Multiple prevalent fractures in relation to macroscopic bone architecture in patients with cystic fibrosis

Stahl, Mirjam; Holfelder, Christian; Kneppo, Carolin; Kieser, Meinhard; Kasperk, Christian; Schöenau, Eckhard; Sommerburg, Olaf; Tönshoff, Burkhard

doi:10.1016/j.jcf.2016.06.004

Cited by 16 publications

(21 citation statements)

References 26 publications

(36 reference statements)

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“…[ 3 ] A more recent study by Stahl et al demonstrated that several pQCT parameters, including SSI and trabecular BMD at the distal radius, are able to differentiate between CF patients with 1 fracture or less and those with more than one fracture. [ 24 ] Finally, a study by Bai et al compared pQCT results at the radius in children ages 7–18 years with CF with healthy controls. [ 25 ] They found that although the children with CF had thicker cortical bone, they still had reduced bone strength compared to controls.…”

Section: Discussionmentioning

confidence: 99%

Peripheral quantitative computed tomography detects differences at the radius in prepubertal children with cystic fibrosis compared to healthy controls

et al. 2018

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IntroductionIn 2015, 11.9% of people with cystic fibrosis (CF) in the United States had osteopenia, 5.1% osteoporosis, and 0.3% experienced a fracture. Screening for CF-related bone disease starts in childhood, and dual energy x-ray absorptiometry (DXA) is the recommended method. It is unknown whether peripheral quantitative computed tomography (pQCT) can detect deficits earlier than DXA. This study compared pQCT and DXA scans in a group of pre-pubertal children with CF and healthy controls.MethodsThis was a cross-sectional study of children at Tanner stage 1. A pQCT scan of the radius at proximal and distal sites was performed plus a total body DXA scan. Serum C-reactive protein, interleukin-6 and tumor necrosis factor-alpha were also measured.ResultsA total of 34 subjects completed the study; 14 with CF and 20 controls. At the distal radius, pQCT showed a lower total bone mineral density (BMD) Z-score for the CF group (P = 0.01 and P = 0.03 for 2 different reference databases) compared to controls. At the proximal site, the polar strength-strain index was lower in the CF group (P = 0.017). Finally, the total body BMD Z-score by DXA was lower in the CF group, although it did not meet the definition of reduced bone density (P = 0.004). Biomarkers of inflammation were not different.ConclusionsIn this group of pre-pubertal children with CF, measures of bone strength and density by both pQCT and DXA were reduced compared to healthy controls.

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Section: Discussionmentioning

confidence: 99%

Peripheral quantitative computed tomography detects differences at the radius in prepubertal children with cystic fibrosis compared to healthy controls

et al. 2018

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“…Cortical bone composition and density are key determinants of bone strength and fracture risk in humans [18] . Recent studies reported an elevated fracture rate in CF patients [ 5 , 12 , 31 ] in relation with macroscopic bone architecture [32] , and others described deficits in trabecular and cortical bone as well as strength in prepubertal children, adolescents and adults with CF [ 8 , 33-35 ]. People with CF have several risk factors to develop low bone mass and density including chronic lung inflammation, pancreatic insufficiency, malnutrition, delayed puberty, glucocorticoid use, and possibly CFTR dysfunction itself [ 3 , 11 ].…”

Section: Discussionmentioning

confidence: 99%

“…Well done cross-sectional and longitudinal studies have indicated diminished bone accrual during the skeletal formative period of late childhood and adolescence [11,13,14] . A recent report showed a 9-fold higher fracture rate in young German CF adults compared to the age-matched reference population [15] . It is still unknown if the smaller stature in CF patients is a direct cause of loss of CFTR or a manifestation of nutritional deficiencies and inflammation [16] .…”

Section: Introductionmentioning

confidence: 95%

“…Several studies made on animal models (rats, mice and pigs) with CF shortly after birth revealed abnormalities in bone development [24][25][26][27] and tracheal cartilage with early airflow obstruction [27][28][29][30][31] , suggesting the critical role of CFTR dysfunction. Several other species (ferret [32] , zebrafish [33] ) were used for this investigation on CF pathology.…”

Section: Introductionmentioning

confidence: 99%

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CFTR-deficient pigs display alterations of bone microarchitecture and composition at birth

Braux

Jourdain

Guillaume

et al. 2020

Journal of Cystic Fibrosis

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Background: The lack of cystic fibrosis transmembrane conductance regulator (CFTR) function causes cystic fibrosis (CF), predisposing to severe lung disease, reduced growth and osteopenia. Both reduced bone content and strength are increasingly recognized in infants with CF before the onset of significant lung disease, suggesting a developmental origin and a possible role in bone disease pathogenesis. The role of CFTR in bone metabolism is unclear and studies on humans are not feasible. Deletion of CFTR in pigs (CFTR −/ − pigs) displays at birth severe malformations similar to humans in the intestine, respiratory tract, pancreas, liver, and male reproductive tract. Methods: We compared bone parameters of CFTR −/ − male and female pigs with those of their wild-type (WT) littermates at birth. Morphological and microstructural properties of femoral cortical and trabecular bone were evaluated using micro-computed tomography (μCT), and their chemical compositions were examined using Raman microspectroscopy. Results: The integrity of the CFTR −/ − bone was altered due to changes in its microstructure and chemical composition in both sexes. Low cortical thickness and high cortical porosity were found in CFTR −/pigs compared to sex-matched WT littermates. Moreover, an increased chemical composition heterogeneity associated with higher carbonate/phosphate ratio and higher mineral crystallinity was found in CFTR −/ − trabecular bone, but not in CFTR −/ − cortical bone. Conclusions: The loss of CFTR directly alters the bone composition and metabolism of newborn pigs. Based on these findings, we speculate that bone defects in patients with CF could be a primary, rather than a secondary consequence of inflammation and infection.

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“…Low bone mineral density (BMD) is a common complication of CF, and microarchitecture assessment by high‐resolution peripheral scanner showed impaired volumetric density . The severity of the lung disease is the main determinant of bone loss, leading to increased fracture risk, selectively in the spine and ribs . Nevertheless, despite the improvement of care over recent years, a significant number of patients with CF had low BMD Z scores and reported fractures .…”

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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Multiple prevalent fractures in relation to macroscopic bone architecture in patients with cystic fibrosis

Abstract: Bone densitometry is a useful tool for noninvasive assessment of fracture risk in CF patients.

Cited by 16 publications

References 26 publications

Peripheral quantitative computed tomography detects differences at the radius in prepubertal children with cystic fibrosis compared to healthy controls

Peripheral quantitative computed tomography detects differences at the radius in prepubertal children with cystic fibrosis compared to healthy controls

CFTR-deficient pigs display alterations of bone microarchitecture and composition at birth

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

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