Abstract
A 25 month old girl was first seen because of growth deceleration beginning at 3 months of age. Birth weight was 6½ lbs. and length 19″. P. examination was normal except for a height-age of 13 months. After estrogen priming a growth hormone (GH) stimulation test following sequential L-Dopa, arginine and glucagon peaked at 124 ng/ml from a base-line of 63 ng/ml. Serum somatomedin-C (Sm-C) concentration, both basal and post-stimulation, measured 0.24 U/ml. (N1. 1.5±0.5 U/ml. Measured by Dr. L. Underwood, Univ. of North Carolina, Chapel Hill). At the end of a 24 hour fast her blood glucose was 68 mg%. GH was given for 6 days and her Sm-C level increased to 0.43 U/ml. Four months later her metabolic response to the administration of GH for 6 days showed: release of FFA from 757 to 1341 μM/1 and no increase in urinary Ca++. Again her Sm-C increased from 0.19 to a peak of .73 U/ml. Prior to GH, her basal GH levels ranged from 8 to 14 ng/ml. By RIA her GH produced a parallel dose-response to pituitary GH standard. After the above study, the patient was discharged on GH, 0.1 U/kg three times a week. In the last 2 months her growth rate has increased from 0.5 to 1/cm/month. The data on this patient appear to rule out a defect in Sm-C synthesis or function, as well as factors, either inhibiting GH action or receptor function. The growth deceleration could be explained on the basis of an abnormal circulating GH molecule.
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Hayek, A., Peake, G. & Greenberg, R. 299 A NEW SYNDROME OF SHORT STATURE DUE TO BIOLOGICALLY INACTIVE BUT IMMUNOREACTIVE GROWTH HORMONE. Pediatr Res 12 (Suppl 4), 413 (1978). https://doi.org/10.1203/00006450-197804001-00304
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DOI: https://doi.org/10.1203/00006450-197804001-00304
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