Children with severe Osteogenesis imperfecta and short stature present on average with normal IGF-I and IGFBP-3 levels

J Pediatr Endocrinol Metab. 2016 Jul 1;29(7):813-8. doi: 10.1515/jpem-2015-0385.

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.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Bone Density Conservation Agents / administration & dosage
  • Bone Density Conservation Agents / therapeutic use
  • Child
  • Child, Preschool
  • Cohort Studies
  • Cross-Sectional Studies
  • Diphosphonates / administration & dosage
  • Diphosphonates / therapeutic use
  • Female
  • Growth Disorders / etiology*
  • Hospitals, Pediatric
  • Hospitals, University
  • Humans
  • Infant
  • Infusions, Intravenous
  • Insulin-Like Growth Factor Binding Protein 3 / blood*
  • Insulin-Like Growth Factor I / analysis*
  • Male
  • Osteogenesis Imperfecta / blood
  • Osteogenesis Imperfecta / drug therapy
  • Osteogenesis Imperfecta / physiopathology*
  • Outpatient Clinics, Hospital
  • Retrospective Studies
  • Severity of Illness Index

Substances

  • Bone Density Conservation Agents
  • Diphosphonates
  • IGF1 protein, human
  • IGFBP3 protein, human
  • Insulin-Like Growth Factor Binding Protein 3
  • Osteogenesis Imperfecta, Type IV
  • Insulin-Like Growth Factor I
  • 6-amino-1-hydroxyhexane-1,1-diphosphonate

Supplementary concepts

  • Osteogenesis imperfecta, type 3