Urinary cross-linked carboxyterminal telopeptide, a bone resorption marker, decreases after vaso-occlusive crises in adults with sickle cell disease

Blood Cells Mol Dis. 2020 Feb;80:102369. doi: 10.1016/j.bcmd.2019.102369. Epub 2019 Oct 24.


People with sickle cell disease often report severe bone pain with repeated bouts of vaso-occlusive crises, but the extent of skeletal injury incurred during these painful episodes remain unclear. We sought to quantify bone degradation by comparing urinary concentrations of carboxyterminal cross-linked telopeptide of type I collagen (CTX-1), a well-described marker of bone resorption, in a prospective cohort of 52 adults with sickle cell disease enrolled in the Sickle Cell Pain Markers Study. We also questioned if changes in urinary CTX-1 concentrations correlated with changes in hemolysis and inflammatory markers measured both during and after resolution of a painful vaso-occlusive episode. Thirty-one of the 52 adults enrolled in the study had paired urine samples for CTX-1 analysis. Urinary CTX-1, corrected for urine creatinine, significantly decreased from a mean of 3.45 μg/mmol during vaso-occlusive crises to 2.62 μg/mmol at recovery (p = 0.01). Thus, increased bone loss appears to correlate with acute vaso-occlusive crises in sickle cell disease. Our finding that urinary CTX-1 can be used to probe bone degradation in sickle cell disease provides an important new tool for diagnosing and monitoring response to therapy for people with sickle cell-related bone loss.

Keywords: Bone resorption; CTX-1; Carboxyterminal telopeptide; Sickle cell disease; Vaso-occlusive crisis.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adult
  • Anemia, Sickle Cell / complications*
  • Anemia, Sickle Cell / diagnosis
  • Anemia, Sickle Cell / urine*
  • Biomarkers*
  • Bone Resorption / etiology*
  • Bone Resorption / urine*
  • Collagen Type I / urine*
  • Female
  • Humans
  • Male
  • Pain / etiology*
  • Peptides / urine*


  • Biomarkers
  • Collagen Type I
  • Peptides
  • collagen type I trimeric cross-linked peptide