Bone density and body composition in children with growth hormone insensitivity syndrome receiving recombinant IGF-I

N J Shaw, N C Fraser, S Rose, N J Crabtree, C M Boivin
Clinical Endocrinology 2003, 59 (4): 487-91

OBJECTIVE: There are few reports of the metabolic action of insulin-like growth factor 1 (IGF-I) in vivo. Growth hormone insensitivity syndrome is a good model to examine the effects of IGF-I deficiency. This study was designed to assess body composition and bone density in children with growth hormone insensitivity syndrome before and after receiving treatment with recombinant IGF-I.

DESIGN: A prospective longitudinal study.

PATIENTS: Four prepubertal boys age 6.1-9.8 years with short stature due to growth hormone insensitivity syndrome.

MEASUREMENTS: Assessment of body fat by skinfold thickness measurements and dual energy X-ray absorptiometry (DXA) was made during the first 6 months of recombinant IGF-I treatment. Assessment of lumbar spine bone density by DXA was performed prior to IGF-I treatment and during the subsequent five years.

RESULTS: Each child showed a significant reduction in fat mass (0.26-1.22 kg) after 6 weeks of IGF-I treatment. Bone density prior to treatment was reduced in comparison to age-matched controls but calculated volumetric bone density was within the normal range. Volumetric bone density progressively improved over the 5-year treatment period.

CONCLUSIONS: Children with growth hormone insensitivity syndrome exhibit a metabolic response to IGF-I within 6 weeks analogous to that seen in GH-deficient children receiving GH. Bone density when corrected for body size is within normal limits and demonstrates a response to IGF-I, confirming the anabolic action on bone.

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