Undercarboxylated osteocalcin and bone mass in 8-12 year old children with cystic fibrosis

M S Fewtrell1, C Benden2, J E Williams1

  • 1MRC Childhood Nutrition Research Centre, Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK.

Insights

Young adults with cystic fibrosis (CF) may have bone disease due to poor vitamin K status. This study found higher levels of undercarboxylated osteocalcin (uc-OC), indicating low vitamin K, linked to lower bone mass in children with CF.

Area of Science:

  • Pediatric endocrinology
  • Bone metabolism
  • Nutritional deficiencies

Background:

  • Cystic fibrosis (CF) is associated with an increased risk of bone disease in young adults.
  • Suboptimal vitamin K status and impaired osteocalcin carboxylation are hypothesized contributors to CF-related bone abnormalities.

Purpose of the Study:

  • To investigate bone mineralization and turnover in children with CF.
  • To assess the relationship between vitamin K status and bone health markers in this population.

Main Methods:

  • Dual-energy X-ray absorptiometry (DXA) for bone mineral density (BMD) and bone mineral content (BMC) of the whole body and lumbar spine (LS).
  • Measurement of 25-OH Vitamin D, parathyroid hormone (PTH), plasma osteocalcin, and pro-peptide of type 1 collagen (P1NP).
  • Assessment of vitamin K status using undercarboxylated osteocalcin (% uc-OC).

Main Results:

  • 20% of CF patients aged 8-12 years had LS BMD Z-scores below -1.0.
  • Size-adjusted bone mass was generally normal, but % uc-OC was elevated and P1NP was decreased compared to reference data.
  • Higher % uc-OC was significantly associated with lower size-adjusted LS bone mineral content and LS bone mineral apparent density.

Conclusions:

  • Children with CF exhibit markers of suboptimal vitamin K status and reduced bone formation, even with normal size-adjusted bone mass.
  • The correlation between LS bone mineral content and % uc-OC supports the hypothesis that vitamin K deficiency is a risk factor for bone disease in CF.
  • Further intervention trials are recommended to confirm these findings and explore therapeutic strategies.
Abstract

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