title: [A Preliminary Exploration on the Pathogenesis of Osteopenia in Patients with Hemophilia].
abstract: OBJECTIVE
  To investigate the influencing factors and pathogenesis of osteopenia in the patients with hemophilia.
  
  
  METHODS
  Twenty-three patients with hemophilia were admitted in the hospital affiliated to North China University of Science and technology from March to August 2015, including 13 severe cases, 10 mild and moderate cases. All the patients accepted the detection of serum I collagen cross-linking N terminal peptide (NTX I), osteoprotegerin (OPG), bone alkaline phosphatase (BALP), basic fibroblast growth factor (bFGF), insulin-like growth factor (IGF) and transforming growth factor-β1 (TGF-β1), the score scale of activity ability was recorded according to the criteria published by the U.S. Center for disease prevention and control in 2002, and 21 patients received the measurement of bone mineral density. According to the World Health Organization (WHO) definition, the clinical significance of bone mineral density (BMD) was assessed by measuring the Z level.
  
  
  RESULTS
  Z level>-2 was recorded in 10 cases, Z≤-2 was recorded in 11 cases; the levels of body mass index (BMI) and human bone alkaline phosphatase (BALP) reflecting bone formation in 11 cases (Z≤-2) were lower than there in 10 cases (Z>-2) (P<0.05); the levels of BALP (r=0.489, P<0.05), IGF (r=0.538, P<0.05) and BMI (r=0.572, P<0.01) positively correlated significantly with BMD (P<0.05); the levels of bFGF (r=0.570, P<0.01) and OPG (r=0.505, P<0.05) positively correlated with NTX I, indicating bone destruction (P<0.05); the score of activity ability of severe patients was significantly lower than that of mild and moderate cases (P<0.05), BMD levels of these 2 groups were not statistically different (P>0.05).
  
  
  CONCLUSIONS
  The BMD level does not correlate with the clinial grouping of hemophilia, the low body mass index may be a risk factor for bone lose; the mechanism of hemophilia patient's bone lose may be related with the decrease of osteogenic activity, the IGF can prevent bone lose in hemophilia, the bFGF and OPG can promote bone metabolism of the patients with hemophilia.
authors.affiliation: Department of Hematology, The Affiliated Hospital of North China University of Science and Technology, Tangshan 063000, Hebei Province, China.
authors.forename: Xiao-Yang
authors.initials: XY
authors.lastname: Hao
authors.affiliation: Department of Hematology, The Affiliated Hospital of North China University of Science and Technology, Tangshan 063000, Hebei Province, China.
authors.forename: Lin-Hong
authors.initials: LH
authors.lastname: Wang
authors.affiliation: Department of Hematology, The Affiliated Hospital of North China University of Science and Technology, Tangshan 063000, Hebei Province, China.
authors.forename: Yan-Yan
authors.initials: YY
authors.lastname: Xie
authors.affiliation: Department of Hematology, The Affiliated Hospital of North China University of Science and Technology, Tangshan 063000, Hebei Province, China.
authors.forename: Wen-Yue
authors.initials: WY
authors.lastname: Qi
authors.affiliation: Department of Hematology, The Affiliated Hospital of North China University of Science and Technology, Tangshan 063000, Hebei Province, China.
authors.forename: Song
authors.initials: S
authors.lastname: Zhang
authors.affiliation: Department of Hematology, The Affiliated Hospital of North China University of Science and Technology, Tangshan 063000, Hebei Province, China.
authors.forename: Mei-Rong
authors.initials: MR
authors.lastname: Yang
authors.affiliation: Department of Hematology, The Affiliated Hospital of North China University of Science and Technology, Tangshan 063000, Hebei Province, China. E-mail: hbyzy2011@163.com.
authors.forename: Zhen-Yu
authors.initials: ZY
authors.lastname: Yan
authors.affiliation: Department of Hematology, The Affiliated Hospital of North China University of Science and Technology, Tangshan 063000, Hebei Province, China.
authors.forename: Nai-Yao
authors.initials: NY
authors.lastname: Chen
mesh_terms: D000469:Alkaline Phosphatase / Q000378:metabolism; D015415:Biomarkers; D015519:Bone Density; D001851:Bone Diseases, Metabolic / Q000473:pathology*; D001842:Bone and Bones / Q000473:pathology; D024042:Collagen Type I / Q000378:metabolism; D016222:Fibroblast Growth Factor 2 / Q000378:metabolism; D006467:Hemophilia A / Q000473:pathology*; D006801:Humans; D010012:Osteogenesis; D053244:Osteoprotegerin / Q000378:metabolism; D010455:Peptides / Q000378:metabolism; D013002:Somatomedins / Q000378:metabolism; D053773:Transforming Growth Factor beta1 / Q000378:metabolism
chemical_list: D015415:Biomarkers; D024042:Collagen Type I; D053244:Osteoprotegerin; D010455:Peptides; D013002:Somatomedins; C508960:TGFB1 protein, human; C506030:TNFRSF11B protein, human; D053773:Transforming Growth Factor beta1; C052929:collagen type I trimeric cross-linked peptide; D016222:Fibroblast Growth Factor 2; D000469:Alkaline Phosphatase