Lack of response of nonsuppressible insulin-like activity to short term administration of human growth hormone in thalassemia major

The Journal of Clinical Endocrinology and Metabolism
G A WertherA C Herington

Abstract

We have recently demonstrated that nonsuppressible insulin-like activity (NSILA-S) is very low or undetectable in beta-thalassemia major. The mechanism for this deficiency has been proposed to result from a defect at the GH receptor level or at a site distal to the receptor in the pathway leading to NSILA-S generation. To study this proposal further, the NSILA-S response to exogenous human GH (hGH) was assessed in six affected subjects (aged 12-20 yr), four of whom were on or below the third percentile for height and three of whom were prepubertal. On the basis of immunoreactive and receptor-active hGH responses to exercise, GH deficiency as a primary cause of low NSILA-S levels was essentially ruled out in five of the six patients. hGH (5 mg) was administered im daily for 3 days. Bioassayable NSILA-S failed to rise normally in response to the exogenous hGH in four of the six subjects. In the other two, a limited response was seen only on day 4, reaching 54% and 29%, respectively, of the mean peak response in normal adults. The impaired production of NSILA-S in thalassemia major thus appears to be due to hGH insensitivity and not to a biologically inactive endogenous hGH molecule. These results confirm that the site of the defe...Continue Reading

Citations

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