1. An update on methods for assessing bone quality and health in Cystic fibrosis
- Author
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Kristen M. Williams, Amy Darukhanavala, Rebecca Hicks, and Andrea Kelly
- Subjects
musculoskeletal diseases ,FRAX, Fracture Risk Assessment Tool ,Endocrinology, Diabetes and Metabolism ,BMD, bone mineral density ,UD radius, Ultradistal radius ,Special Issue: CF Endocrinology Advance ,CF, Cystic Fibrosis ,Diseases of the endocrine glands. Clinical endocrinology ,Cystic fibrosis ,UBPI, Ultrasound bone profile index ,Endocrinology ,CFBD, Cystic fibrosis related bone disease ,Bone disease ,HSA, Hip Structural Analysis ,Bone mineral density ,VFA, Vertebral Fracture Assessment ,ISCD, International Society Clinical Densitometry ,DXA, dual energy x-ray absorptiometry ,pQCT, peripheral quantitative computed tomography ,LS, lumbar spine ,HAZ, height for age Z-score ,RC648-665 ,QUS, quantitative ultrasound ,TBLH, total body less head ,Fracture ,Dual energy x-ray absorptiometry ,LSC, least significant change ,SOS, speed of sound ,TBS, Trabecular Bone Score ,MRI, magnetic resonance imaging ,aBMD, areal bone mineral density ,BMC, bone mineral content - Abstract
Highlights • CF bone disease is prevalent in children and adults despite recent advances in care. • DXA is the current gold standard in the evaluation of bone strength. • Limited evidence exists for the use of MRI or HRpQCT in the assessment of CFBD. • Fracture prediction models may help guide therapy but are not yet validated in CF., With increasing life expectancy in people with Cystic fibrosis (CF), the focus of clinical care has shifted to management and prevention of non-pulmonary comorbidities. CF related bone disease, defined by low bone mineral density (BMD), is prevalent across all age groups and acknowledges the increased fractures rates that negatively impact lung function and quality of life. Dual energy X-ray absorptiometry (DXA) measurement of bone mineral content (BMC) and “areal” BMD (aBMD) is recommended for identifying and monitoring bone health in children and adults due to its low cost, low radiation exposure, and widespread availability. Recent studies in children and adolescents with chronic illness focus on adjustment of BMC and aBMD measurements for height due to the effects of short stature and delayed maturation on bone size. Expanded reference databases for alternate imaging sites such as the ultradistal radius and hip present opportunities for research and long-term monitoring. As the two-dimensional nature of DXA imposes limitations, we highlight other imaging modalities including peripheral quantitative computed tomography QCT (pQCT), magnetic resonance imaging, and quantitative ultrasound (QUS). These tools, while primarily used in a research setting, can impart information on true volumetric bone density and bone microarchitecture as well as contribute to fracture assessment and prediction. Due to the high morbidity and mortality associated with vertebral and hip fracture, we will present on vertebral fracture assessment (VFA) in both children and adults as well as applied analyses including hip structural analysis (HSA), trabecular bone score (TBS), and fracture risk assessment (FRAX) for high risk groups. Questions remain on the future clinical applicability and accessibility of these assessment and prediction tools, longitudinal monitoring through adolescence and adulthood, and how outcome measures may guide bone modifying therapies.
- Published
- 2021