title: Sickle cell bone disease: response to vitamin D and calcium.
abstract: Bone disease with osteoporosis and osteomalacia are common in sickle cell disease (SCD). Some patients have vitamin D deficiency and low bone mineral density (BMD). The role of vitamin D and calcium supplementation to restore bone health in SCD has not been well studied. In 14 adults with SCD, we measured 25(OH)D (25-hydroxyvitamin D) and BMD at the femoral neck, lumbar spine, and distal third of the ulna plus radius, along with markers of bone resorption (CTx; C-terminal component of pro-collagen type I) and bone formation (osteocalcin) before and after 12 months of vitamin D(2) and calcium carbonate treatment. Pretreatment, all patients were vitamin D deficient with a mean 25(OH)D level of 11.6 [corrected] +/- 4 [corrected] ng/ml, had low BMD at the lumbar spine (L-spine), 0.87 +/- 0.11 g/cm(2) (mean Z-score of -2.6 3 +/- 0.71 SD and T score of -2.31 +/- 0.75 SD), femoral neck, 0.8 +/- 0.18 g/cm(2) (mean Z-score -1.36 +/- 0.84, T-score -1.14 +/- 0.75), and the distal radius and ulna, 0.6 +/- 0.17 g/cm(2) (mean Z-score -1.18 +/- 0.79, T-score -1.01 +/- 0.74) and had elevated CTx (0.87 +/- 0.5 ng/ml) and osteocalcin levels (12.3 +/- 3.7 ng/mul). After treatment, all patients corrected their 25(OH)D level (34.6 [corrected] +/- 11 [corrected] ng/ml) (P < 0.001) with a 3.6% +/- 3.9% increase in BMD at the L-spine (P = 0.009), 4.6% +/- 8.5% increase at the femoral neck (P = 0.05) and 6.5% +/- 12.6% increase at the distal radius plus ulna (P = 0.09). CTx, osteocalcin, and PTH(i) levels were unchanged. Treatment of adult SCD with vitamin D and calcium can restore 25(OH)D levels to normal and improve BMD, but, markers of bone resorption remained unchanged. Screening for vitamin D deficiency and BMD in SCD patients seems warranted.
authors.affiliation: The Center of Excellence in Sickle Cell Disease and the Vitamin D, Skin and Bone Research Laboratories, Boston University School of Medicine, Boston, MA 02118, USA. adeboye.adewoye@bmc.org
authors.forename: Adeboye H
authors.initials: AH
authors.lastname: Adewoye
authors.forename: Tai C
authors.initials: TC
authors.lastname: Chen
authors.forename: Qianli
authors.initials: Q
authors.lastname: Ma
authors.forename: Lillian
authors.initials: L
authors.lastname: McMahon
authors.forename: Jeff
authors.initials: J
authors.lastname: Mathieu
authors.forename: Alan
authors.initials: A
authors.lastname: Malabanan
authors.forename: Martin H
authors.initials: MH
authors.lastname: Steinberg
authors.forename: Michael F
authors.initials: MF
authors.lastname: Holick
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications*; D015519:Bone Density; D001862:Bone Resorption / Q000097:blood / Q000188:drug therapy* / Q000209:etiology; D002119:Calcium Carbonate / Q000008:administration & dosage / Q000627:therapeutic use*; D024042:Collagen Type I / Q000097:blood; D004359:Drug Therapy, Combination; D004872:Ergocalciferols / Q000008:administration & dosage / Q000627:therapeutic use*; D005272:Femur Neck / Q000737:chemistry; D006801:Humans; D008159:Lumbar Vertebrae / Q000737:chemistry; D015675:Osteocalcin / Q000097:blood; D010018:Osteomalacia / Q000097:blood / Q000188:drug therapy* / Q000209:etiology; D010024:Osteoporosis / Q000097:blood / Q000188:drug therapy* / Q000209:etiology; D010281:Parathyroid Hormone / Q000097:blood; D010455:Peptides / Q000097:blood; D010865:Pilot Projects; D014807:Vitamin D / Q000031:analogs & derivatives / Q000097:blood; D014808:Vitamin D Deficiency / Q000097:blood / Q000150:complications / Q000188:drug therapy*
chemical_list: D024042:Collagen Type I; D004872:Ergocalciferols; D010281:Parathyroid Hormone; D010455:Peptides; C052929:collagen type I trimeric cross-linked peptide; D015675:Osteocalcin; D014807:Vitamin D; C104450:25-hydroxyvitamin D; D002119:Calcium Carbonate