title: Children with severe Osteogenesis imperfecta and short stature present on average with normal IGF-I and IGFBP-3 levels.
abstract: BACKGROUND
  Osteogenesis imperfecta (OI) is characterized by bone fragility and short stature. Data about IGF-I/IGFBP-3 levels are rare in OI. Therefore IGF-I/IGFBP-3 levels in children with different types of OI were investigated.
  
  
  METHODS
  IGF-I and IGFBP-3 levels of 60 children (male n=38) were assessed in a retrospective cross-sectional setting.
  
  
  RESULTS
  Height/weight was significant different [height z-score type 3 versus type 4: p=0.0011 and weight (p≤0.0001)] between OI type 3 and 4. Mean IGF-I levels were in the lower normal range (mean±SD level 137.4±109.1 μg/L). Mean IGFBP-3 measurements were in the normal range (mean±SD 3.105±1.175 mg/L). No significant differences between OI type 3 and 4 children have been observed (IGF-I: p=0.0906; IGFBP-3: p=0.2042).
  
  
  CONCLUSIONS
  Patients with different severities of OI have IGF-I and IGFBP-3 levels in the lower normal range. The type of OI does not significantly influence these growth factors.
authors.forename: Heike
authors.initials: H
authors.lastname: Hoyer-Kuhn
authors.forename: Laura
authors.initials: L
authors.lastname: Höbing
authors.forename: Julia
authors.initials: J
authors.lastname: Cassens
authors.forename: Eckhard
authors.initials: E
authors.lastname: Schoenau
authors.forename: Oliver
authors.initials: O
authors.lastname: Semler
mesh_terms: D000293:Adolescent; D050071:Bone Density Conservation Agents / Q000008:administration & dosage / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D004164:Diphosphonates / Q000008:administration & dosage / Q000627:therapeutic use; D005260:Female; D006130:Growth Disorders / Q000209:etiology*; D006776:Hospitals, Pediatric; D006785:Hospitals, University; D006801:Humans; D007223:Infant; D007262:Infusions, Intravenous; D018972:Insulin-Like Growth Factor Binding Protein 3 / Q000097:blood*; D007334:Insulin-Like Growth Factor I / Q000032:analysis*; D008297:Male; D010013:Osteogenesis Imperfecta / Q000097:blood / Q000188:drug therapy / Q000503:physiopathology*; D010044:Outpatient Clinics, Hospital; D012189:Retrospective Studies; D012720:Severity of Illness Index
chemical_list: D050071:Bone Density Conservation Agents; D004164:Diphosphonates; D018972:Insulin-Like Growth Factor Binding Protein 3; D007334:Insulin-Like Growth Factor I; C583557:IGF1 protein, human; C515497:IGFBP3 protein, human; C053389:6-amino-1-hydroxyhexane-1,1-diphosphonate