- Email: [email protected]
205 Hand grip strength and DXA in adults with cystic fibrosis
S110
9. Bone/Vitamin D/Liver Disease
Posters
201 Tibial cortical bone is impaired in adults with CF
203 Children’s bone disease and its risk factors in our centre
D. Gensburger1 , S. Boutroy2 , R. Chapurlat1 , R. Nove-Josserand3 , M. Rabilloud4 , S. Roche4 , I. Durieu5 . 1 INSERM U1033. Universit´e de Lyon, Department of Rheumatology, Hospices Civils de Lyon, Lyon, France; 2 INSERM U1033. Universit´e de Lyon, Lyon, France; 3 Centre de R´ef´erence Mucoviscidose Adulte, C.H. Lyon Sud, Hospices Civils de Lyon, Pierre-B´enite, France; 4 UMR 5558, Universit´e Lyon 1, Service de Biostatistique, Hospices Civils de Lyon, Lyon, France; 5 Centre de R´ef´erence Mucoviscidose Adulte, C.H. Lyon Sud, Hospices Civils de Lyon, Universit´e de Lyon, Pierre-B´enite, France
I.M. Ciuca1 , L.L. Pop1,2 , D.I. Onet3 , B. Almajan Guta4 , A. Ranetti5 , Z. Popa1 . 1 University of Medicine and Pharmacy Victor Babes, Pediatric II Department, Timisoara, Romania; 2 National Cystic Fibrosis Centre, Timisoara, Romania; 3 University of Medicine and Pharmacy Victor Babes, Department of Radiology, Clinical County Hospital Timisoara, Timisoara, Romania; 4 Vest University Timisoara, Phisycal Education and Sport, Timisoara, Romania; 5 Central Military Hospital, Bucuresti, Romania
Objectives: Bone disease has been well described in cystic fibrosis (CF) adult patients. While DXA remains the gold standard method to assess bone, it has important limitations. High resolution peripheral QCT (HRpQCT) could provide useful information on geometry, volumetric density (vBMD) and microarchitecture of bone that may be impaired in CF. Our aims were to assess the prevalence of CF related low BMD in a current adult CF population and to characterize the bone geometry and microarchitecture with HRpQCT at the distal radius and tibia. Methods: Fifty six CF patients had a clinical and biological evaluation, a DXA at the lumbar spine and hip and a spine radiograph. A subgroup of 43 CF and 86 age- and gender-matched healthy controls underwent HRpQCT measurements. Results: Among the 56 CF (52% men, 26±7 yrs), the prevalence of low areal BMD defined by a T-score <−2 at any site, was 20%. 7 patients (13%) had a history of fracture. In the subset of 43 CF (63% men, 29±7 yrs), the difference between CF and controls were mostly observed at the distal tibia, with lower vBMD (total: −11%, trabecular: −12% and cortical: −3%, p < 0.02), cortical thickness (−14%, p < 0.001) and trabecular number (−6%, p < 0.05) in CF compared to controls. After adjustment for age, gender, weight, height and multiple comparisons, total vBMD and cortical thickness at the tibia remained significantly lower in CF than in controls. Conclusion: In this study, bone disease seemed to be less severe than previously described with only 20% of CF patient with low areal BMD. Yet, the impaired cortical thickness leading to a lower total vBMD of the tibia, may explain the increased fracture risk in CF population.
202 Bone mineral density and fractures at the All Wales Adult CF Centre (AWACFC) E. Kealaher1 , L. Speight1 , M. Stone2 , D. Lau1 , R.I. Ketchell1 , J. Duckers1 . & Vale University Health Board, All Wales Adult CF Centre, Cardiff, United Kingdom; 2 Cardiff & Vale University Health Board, Cardiff, United Kingdom 1 Cardiff
CF related low bone mineral density (CFRLBMD) occurs in 34% CF patients (Haworth 2001). 14% of CF patients have evidence of vertebral fractures and 20% nonvertebral fractures (Paccou 2010). Aim: To determine the prevalence of CFLBMD and fractures in patients attending the AWACFC. Method: The following information taken from the annual reviews of patients attending the AWACFC: age, sex, history and nature of fractures, FEV1 %, BMI, Z score Lumbar spine (LS) and hip and whether the patient was maintained on calcium/vitamin D and bisphosphonate, duration of bisphosphonate use and change in Z score over this period. Patients completed a fracture history questionnaire. Results: 254 (144 male) patients were included with a mean (SD) age, FEV1 % and BMI of 28.8 (9.4) years, 65.7% (25.6) and 22.8 kg/m2 (4.65) respectively. Their median (range) Z score at lumbar spine (LS) and hip were −0.87 (−4.2 to 2.4) and −0.64 (−3.7 to 2.6) respectively. 57 (32 male) of 254 (22%) patients had CFRLBMD. Of these 57, 55 (96%) were on calcium/vit D and 44 (77%) on bisphosphonate therapy. Patients with CFRLBMD had lower FEV1 % [47.5 vs 70.7 (p < 0.001)] and BMI [20.7 vs 23.4 kg/m2 (p < 0.001)] and more IV antibiotics/year [3.28 vs 1.79 (p < 0.001)] than patients without CFRLBMD. 61 (38 male) of 254 (24%) had documented fractures. 12 of these 61 were currently on bisphosphonate therapy and 39 of 61 were receiving calcium/vit D supplementation. Conclusion: The prevalence of CFRLBMD at the AWACFC is slightly lower than Haworth study which may reflect a trend to a more aggressive approach to CF therapies over the past decade. The prevalence of fractures in our cohort is similar to other studies.
Objective: Evaluation of CF bone disease presence and identification of its risk factors in our CF children population. Methods: Study included 68 children with cystic fibrosis, aged 10.2 to 18.8 years, genotyped and monitored in the National CF Centre, for 2 years. At the annual assessment, besides clinical examination, biochemical evaluation for pancreatic insufficiency, diabetes liver disease, a subgroup of 26 children were evaluated for bone mineral density using dual energy x-ray absorptiometry (DXA). Detection of body mass density − BMD (g/cm2 ) − by DXA was determined on lumbar spinal (L1−L4). The age-corrected BMD findings were expressed as Z scores and correlated with gender, nutritional status (BMI), pancreatic insufficiency, genotype and presence of related diabetes. Results: Twenty-six patients, aged over 10 years were diagnosed with CF bone disease, signifying a cumulative prevalence of 38.2%, without significant gender gap. The average Z score for BMD was −2.55. Bone disease was frequent in patients aged over 10 years with exocrine pancreatic insufficiency RR = 1.37 (95% CI 1.044– 1.804), p = 0.02, carriers of severe mutations (RR = 1.51; 95% CI 1.054–2.187) and CF liver disease. Conclusion: CF children carriers of a severe genotype who associates pancreatic insufficiency and CF liver disease were more likely predisposed to low bone mineral density. Further studies should discover other significant influences in order to prevent the development of CF bone disease and an improved life quality in cystic fibrosis children.
205 Hand grip strength and DXA in adults with cystic fibrosis L. Mead1 , H. Watson1 , C.S. Haworth1 , R.A. Floto1,2 . 1 Papworth Hospital NHS Foundation Trust, Cambridge Centre for Lung Infection. The Adult Cystic Fibrosis Centre, Cambridge, United Kingdom; 2 University of Cambridge, Cambridge Institute for Medical Research, Cambridge, United Kingdom Objectives: We have previously shown that hand grip strength (HGS) in 245 adult CF patients was significantly reduced compared to healthy reference values, declined with worsening clinical status, and may be a better indicator of nutritional status than body mass index (BMI) [1]. We extended this work to determine if reduced HGS was associated with lower bone mineral density (assessed by DXA Z scores) and lower fat-free mass (FFM). Methods: 103 whole body DXA’s and 172 DXA Z scores were performed within 6 months of HGS measurements taken using a standardised protocol. We grouped results into those with HGS <85% predicted (low HGS group) and those with 85% predicted (high HGS group). Results: This cohort had 81 males and 91 females with mean values for age 28.5 years, BMI 22.4 kg/m2 and FEV1 % predicted of 62.8%. Z scores in males within the low and high HGS groups were measured as −1.7 vs −1.1 (spine), −1.3 vs −0.5* (hip), and −1.4 vs −0.6* (neck of femur; NOF). Z scores in females within the low and high HGS groups were measured at −1.0 vs −0.4* (spine), −0.9 vs −0.2 (hip), and −1.0 vs −0.4* (NOF). *Denotes p value of <0.05 (two tailed unpaired Student’s T test). In contrast, BMI was not significantly different in any of these groups. HGS was more closely correlated to FFM than BMI in males and females. Conclusion: HGS was significantly reduced in those with lower DXA Z scores but BMI was not. HGS is more closely correlated with DXA than BMI and may be a useful additional test to assess nutritional status and disease severity in CF. Reference(s) [1] Mead et al. Handgrip strength is associated with disease severity in adults with CF. J Cyst Fibros. 2014; 13(2): S101.
9. Bone/Vitamin D/Liver Disease
Posters
201 Tibial cortical bone is impaired in adults with CF
203 Children’s bone disease and its risk factors in our centre
D. Gensburger1 , S. Boutroy2 , R. Chapurlat1 , R. Nove-Josserand3 , M. Rabilloud4 , S. Roche4 , I. Durieu5 . 1 INSERM U1033. Universit´e de Lyon, Department of Rheumatology, Hospices Civils de Lyon, Lyon, France; 2 INSERM U1033. Universit´e de Lyon, Lyon, France; 3 Centre de R´ef´erence Mucoviscidose Adulte, C.H. Lyon Sud, Hospices Civils de Lyon, Pierre-B´enite, France; 4 UMR 5558, Universit´e Lyon 1, Service de Biostatistique, Hospices Civils de Lyon, Lyon, France; 5 Centre de R´ef´erence Mucoviscidose Adulte, C.H. Lyon Sud, Hospices Civils de Lyon, Universit´e de Lyon, Pierre-B´enite, France
I.M. Ciuca1 , L.L. Pop1,2 , D.I. Onet3 , B. Almajan Guta4 , A. Ranetti5 , Z. Popa1 . 1 University of Medicine and Pharmacy Victor Babes, Pediatric II Department, Timisoara, Romania; 2 National Cystic Fibrosis Centre, Timisoara, Romania; 3 University of Medicine and Pharmacy Victor Babes, Department of Radiology, Clinical County Hospital Timisoara, Timisoara, Romania; 4 Vest University Timisoara, Phisycal Education and Sport, Timisoara, Romania; 5 Central Military Hospital, Bucuresti, Romania
Objectives: Bone disease has been well described in cystic fibrosis (CF) adult patients. While DXA remains the gold standard method to assess bone, it has important limitations. High resolution peripheral QCT (HRpQCT) could provide useful information on geometry, volumetric density (vBMD) and microarchitecture of bone that may be impaired in CF. Our aims were to assess the prevalence of CF related low BMD in a current adult CF population and to characterize the bone geometry and microarchitecture with HRpQCT at the distal radius and tibia. Methods: Fifty six CF patients had a clinical and biological evaluation, a DXA at the lumbar spine and hip and a spine radiograph. A subgroup of 43 CF and 86 age- and gender-matched healthy controls underwent HRpQCT measurements. Results: Among the 56 CF (52% men, 26±7 yrs), the prevalence of low areal BMD defined by a T-score <−2 at any site, was 20%. 7 patients (13%) had a history of fracture. In the subset of 43 CF (63% men, 29±7 yrs), the difference between CF and controls were mostly observed at the distal tibia, with lower vBMD (total: −11%, trabecular: −12% and cortical: −3%, p < 0.02), cortical thickness (−14%, p < 0.001) and trabecular number (−6%, p < 0.05) in CF compared to controls. After adjustment for age, gender, weight, height and multiple comparisons, total vBMD and cortical thickness at the tibia remained significantly lower in CF than in controls. Conclusion: In this study, bone disease seemed to be less severe than previously described with only 20% of CF patient with low areal BMD. Yet, the impaired cortical thickness leading to a lower total vBMD of the tibia, may explain the increased fracture risk in CF population.
202 Bone mineral density and fractures at the All Wales Adult CF Centre (AWACFC) E. Kealaher1 , L. Speight1 , M. Stone2 , D. Lau1 , R.I. Ketchell1 , J. Duckers1 . & Vale University Health Board, All Wales Adult CF Centre, Cardiff, United Kingdom; 2 Cardiff & Vale University Health Board, Cardiff, United Kingdom 1 Cardiff
CF related low bone mineral density (CFRLBMD) occurs in 34% CF patients (Haworth 2001). 14% of CF patients have evidence of vertebral fractures and 20% nonvertebral fractures (Paccou 2010). Aim: To determine the prevalence of CFLBMD and fractures in patients attending the AWACFC. Method: The following information taken from the annual reviews of patients attending the AWACFC: age, sex, history and nature of fractures, FEV1 %, BMI, Z score Lumbar spine (LS) and hip and whether the patient was maintained on calcium/vitamin D and bisphosphonate, duration of bisphosphonate use and change in Z score over this period. Patients completed a fracture history questionnaire. Results: 254 (144 male) patients were included with a mean (SD) age, FEV1 % and BMI of 28.8 (9.4) years, 65.7% (25.6) and 22.8 kg/m2 (4.65) respectively. Their median (range) Z score at lumbar spine (LS) and hip were −0.87 (−4.2 to 2.4) and −0.64 (−3.7 to 2.6) respectively. 57 (32 male) of 254 (22%) patients had CFRLBMD. Of these 57, 55 (96%) were on calcium/vit D and 44 (77%) on bisphosphonate therapy. Patients with CFRLBMD had lower FEV1 % [47.5 vs 70.7 (p < 0.001)] and BMI [20.7 vs 23.4 kg/m2 (p < 0.001)] and more IV antibiotics/year [3.28 vs 1.79 (p < 0.001)] than patients without CFRLBMD. 61 (38 male) of 254 (24%) had documented fractures. 12 of these 61 were currently on bisphosphonate therapy and 39 of 61 were receiving calcium/vit D supplementation. Conclusion: The prevalence of CFRLBMD at the AWACFC is slightly lower than Haworth study which may reflect a trend to a more aggressive approach to CF therapies over the past decade. The prevalence of fractures in our cohort is similar to other studies.
Objective: Evaluation of CF bone disease presence and identification of its risk factors in our CF children population. Methods: Study included 68 children with cystic fibrosis, aged 10.2 to 18.8 years, genotyped and monitored in the National CF Centre, for 2 years. At the annual assessment, besides clinical examination, biochemical evaluation for pancreatic insufficiency, diabetes liver disease, a subgroup of 26 children were evaluated for bone mineral density using dual energy x-ray absorptiometry (DXA). Detection of body mass density − BMD (g/cm2 ) − by DXA was determined on lumbar spinal (L1−L4). The age-corrected BMD findings were expressed as Z scores and correlated with gender, nutritional status (BMI), pancreatic insufficiency, genotype and presence of related diabetes. Results: Twenty-six patients, aged over 10 years were diagnosed with CF bone disease, signifying a cumulative prevalence of 38.2%, without significant gender gap. The average Z score for BMD was −2.55. Bone disease was frequent in patients aged over 10 years with exocrine pancreatic insufficiency RR = 1.37 (95% CI 1.044– 1.804), p = 0.02, carriers of severe mutations (RR = 1.51; 95% CI 1.054–2.187) and CF liver disease. Conclusion: CF children carriers of a severe genotype who associates pancreatic insufficiency and CF liver disease were more likely predisposed to low bone mineral density. Further studies should discover other significant influences in order to prevent the development of CF bone disease and an improved life quality in cystic fibrosis children.
205 Hand grip strength and DXA in adults with cystic fibrosis L. Mead1 , H. Watson1 , C.S. Haworth1 , R.A. Floto1,2 . 1 Papworth Hospital NHS Foundation Trust, Cambridge Centre for Lung Infection. The Adult Cystic Fibrosis Centre, Cambridge, United Kingdom; 2 University of Cambridge, Cambridge Institute for Medical Research, Cambridge, United Kingdom Objectives: We have previously shown that hand grip strength (HGS) in 245 adult CF patients was significantly reduced compared to healthy reference values, declined with worsening clinical status, and may be a better indicator of nutritional status than body mass index (BMI) [1]. We extended this work to determine if reduced HGS was associated with lower bone mineral density (assessed by DXA Z scores) and lower fat-free mass (FFM). Methods: 103 whole body DXA’s and 172 DXA Z scores were performed within 6 months of HGS measurements taken using a standardised protocol. We grouped results into those with HGS <85% predicted (low HGS group) and those with 85% predicted (high HGS group). Results: This cohort had 81 males and 91 females with mean values for age 28.5 years, BMI 22.4 kg/m2 and FEV1 % predicted of 62.8%. Z scores in males within the low and high HGS groups were measured as −1.7 vs −1.1 (spine), −1.3 vs −0.5* (hip), and −1.4 vs −0.6* (neck of femur; NOF). Z scores in females within the low and high HGS groups were measured at −1.0 vs −0.4* (spine), −0.9 vs −0.2 (hip), and −1.0 vs −0.4* (NOF). *Denotes p value of <0.05 (two tailed unpaired Student’s T test). In contrast, BMI was not significantly different in any of these groups. HGS was more closely correlated to FFM than BMI in males and females. Conclusion: HGS was significantly reduced in those with lower DXA Z scores but BMI was not. HGS is more closely correlated with DXA than BMI and may be a useful additional test to assess nutritional status and disease severity in CF. Reference(s) [1] Mead et al. Handgrip strength is associated with disease severity in adults with CF. J Cyst Fibros. 2014; 13(2): S101.