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National Guideline Alliance (UK). Cystic Fibrosis: Diagnosis and management. London: National Institute for Health and Care Excellence (NICE); 2017 Oct 25. (NICE Guideline, No. 78.)

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Cystic Fibrosis: Diagnosis and management.

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Table 166Summary of included studies

StudyPopulationPrognostic indicatorFollow-upOutcomesComments
Baker 2016
USA
Retrospective observational study		N=63 adults with CF
Mean age (SD): 31.7 (8.0) years (18 to 57)
50.9% male
  • Low BMD, defined as z-score ≤ −1
  • Very low BMD, defined as z-score ≤ −2
  • Standard dual-energy X-ray absorptiometry (DXA) on a QDR4500A model (Hologic Inc, Bedford, MA)
2 years (data available for n=39)
  • Change in posterioranterior spine BMD
  • Multivariate model adjusting for: age, gender, fat-free max index and height
Subjects that did not have a repeat PA spine DXA were significantly younger and tended to have lower baseline PA spine z-scores.
Brenckmann 2003
Canada
Retrospective observational study		N=40 adults with CF
Mean age (SD): 28.7 (8.4) years (19 to 52)
  • Hip BMD
  • Lumbar spine
  • Total BMD
  • DXA scan. No details given.
1 year (data available for n=27)
  • Change in hip BMD
  • Change in lumbar spine
  • Change in total BMD
Subgroup analysis was not possible as sample size was too small.
N=21 participants were receiving oral or IV corticosteroids
Bhudhikanok 1998
USA
Prospective observational study		N=47 children, young people and adults with CF
Mean age, range: 20.6 years (8.4 to 48.5 years) 30 female, 19 male
  • Boy and young males n=9
  • Adult males n=6
  • Girls and young females n=11
  • Adult females n=15
  • Lumbar spine BMD (QCT)
  • Femoral neck BMD (DXA)
  • Whole body BMD (DXA)
Dual-energy X-ray (DXA) absorptiometry (QDR 100W, Hologic Corporation, Waltham, Mass)		Mean 17 months (11 to 25 months) (data available for n=41)
  • Change in lumbar spine BMD
  • Change in femoral neck BMD
  • Change in whole body BMD
Haworth 2002
UK		N=114 young people and adults with CF
Mean (SD) age: 25.1 (6.9)
  • Lumbar spine BMD
  • Femoral neck BMD
  • Total hip BMD
12 to 13 months
  • Change in lumbar spine BMD
Prospective observational studyyears (15 to 49)
  • Young cohort ≤ 24 years n=55
  • Adult cohort ≥ 25 years n=59
  • QCT and DXA
  • (reported in a previous study)
  • Change in femoral neck BMD
  • Change in total hip BMD
Papaioannou 2008
Canada Retrospective observational study		N=49 adults with CF
Mean age (SD): 25.2 (9.4) years
42.9% male
  • Lumbar spine BMD
  • Proximal femur BMD
  • Whole body BMD
  • Standard dual-energy X-ray absorptiometry (DXA) on a QDR4500A model (Hologic Inc, Bedford, MA, USA)
Mean (SD): 4.03 (1.45) years
(data available for n=10)
  • Lumbar spine BMD
  • Proximal femur BMD
  • Whole body BMD
Schulze 2006
USA
Prospective observational study		N=18 prepubertal and pubertal girls and young females
Age range: 7.6 to 17.9 years
  • Lumbar spine BMD
  • Whole body BMD
  • Standard dual-energy X-ray absorptiometry (DXA) on a QDR4500A model (Hologic Inc, Bedford, MA, USA)
Mean time: 2.13±1.14 years (range 1.6 to 4.10)
  • Change in lumbar spine BMD.
  • Change in whole body BMD.
Although the study reported data stratified by pubertal status, this was not reported in the review as sample size was very low (range 2 to 7) Significant differences were found by pubertal stage at baseline.

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