title: Association of severe haemophilia A with osteoporosis: a densitometric and biochemical study.
abstract: Following a femoral neck fracture and vertebral compression fractures in two patients with severe haemophilia A, bone density and turnover were measured in 19 males with severe haemophilia A (all HIV negative, 18/19 hepatitis C antibody positive) and in 19 age/sex matched controls. Bone density at the lumbar spine (L2-4), measured by dual energy X-ray absorptiometry, was significantly lower in the haemophiliac patients (HPs) at (mean +/- SEM) 1.109 +/- 0.042 g/cm2 vs. 1.234 +/- 0.027 in controls; p = 0.018. Femoral neck density was also lower at 0.877 +/- 0.034 g/cm2 (HPs) vs. 1.067 +/- 0.032; p < 0.0005. No significant differences were evident between the groups for serum calcium, parathyroid hormone, luteinizing hormone, follicle-stimulating hormone or 1,25 dihydroxyvitamin D3, nor for fasting urinary hydroxyproline, pyridinoline or deoxypyridinoline excretion. Serum total alkaline phosphatases was elevated in HPs at 200 +/- 10 U/l vs. 158 +/- 8; p = 0.004. Similarly, gamma-glutamyl transferase was elevated at 42 +/- 7 U/l (HPs) vs. 20 +/- 2; p = 0.007. Serum total testosterone and sex-hormone-binding globulin (SHBG) were higher in HPs at 26 +/- 2.5 nmol/l vs. 17.4 +/- 1.6 (p = 0.009) and 56 +/- 6 nmol/l vs. 27 +/- 3 (p = 0.0005), respectively. Free androgen index, however, was lower in HPs at 44 +/- 5 vs 69 +/- 7; p = 0.008. These results suggest significant osteopenia associated with haemophilia A. This may be partly due to liver dysfunction in HPs, but other factors, e.g. relative immobilization, may also be relevant.
authors.affiliation: University Department of Medicine, Glasgow Royal Infirmary, UK.
authors.forename: S J
authors.initials: SJ
authors.lastname: Gallacher
authors.forename: C
authors.initials: C
authors.lastname: Deighan
authors.forename: A M
authors.initials: AM
authors.lastname: Wallace
authors.forename: R A
authors.initials: RA
authors.lastname: Cowan
authors.forename: W D
authors.initials: WD
authors.lastname: Fraser
authors.forename: J A
authors.initials: JA
authors.lastname: Fenner
authors.forename: G D
authors.initials: GD
authors.lastname: Lowe
authors.forename: I T
authors.initials: IT
authors.lastname: Boyle
mesh_terms: D015502:Absorptiometry, Photon; D000293:Adolescent; D000328:Adult; D000368:Aged; D000469:Alkaline Phosphatase / Q000097:blood; D015519:Bone Density; D005272:Femur Neck / Q000378:metabolism; D006467:Hemophilia A / Q000150:complications* / Q000378:metabolism; D006801:Humans; D008159:Lumbar Vertebrae / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D010024:Osteoporosis / Q000209:etiology* / Q000378:metabolism; D012738:Sex Hormone-Binding Globulin / Q000032:analysis; D013739:Testosterone / Q000097:blood
chemical_list: D012738:Sex Hormone-Binding Globulin; D013739:Testosterone; D000469:Alkaline Phosphatase