ESPE Abstracts (2015) 84 P-3-664

Short Stature in Osteogenesis Imperfecta is not Caused by Deficiencies in IGF1 or IGF-BP3

Oliver Semler, Heike Hoyer-Kuhn, Gabriel Allo & Eckhard Schoenau

Children’s Hospital University Cologne, Cologne, Germany

Background: Osteogenesis imperfecta is a rare collagen related hereditary disease leading to recurrent fractures, reduced mobility, muscular weakness and short stature.

Objective and hypotheses: It was always discussed if the reduced height is a consequence of the impaired collagen production, a reaction of the body to the brittleness of bones or if the patient might suffer from an additional deficiency of growth hormone (GH).

Method: In a retrospective analysis 60 children (28 male; OI type 3 n=22, OI type 4 n=38) were investigated during their regular yearly examinations and stratified according to the clinical severity of the disease.

Results: Results are presented in Table 1 showing severely reduced height z-scores with normal IGF1 and IGF-BP3 levels.

Number of patients 22 38
Height z-score −6.43 −3.62 0.0011
Median [IQR] [−7.939/−5.172] [−4.576/−2.849]
IGF1 z-score −1.45 −0.95 0.2881
Median [IQR] [−2.376/−0.913] [−1.574/−0.883]
IGFBP3 z-score −0.13 0.05 0.6717
Median [IQR] [−0.742/0.226] [−0.321/0.152]

Conclusions: Patients with OI III and IV have a severely reduced height with a significant difference of z-scores depending on the severity of the disease but without alterations of IGF1 and IGF-BP3. Therefore there is no evidence for a general GH deficiency in children with OI and a treatment with GH seems to be unreasonable.

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