Abstract
Hyposomatotropinism can be due to a pituitary defect of growth hormone (GH) secretion, a hypothalamic defect of GH-releasing hormone (GHRH) secretion or to a Neurosecretory Defect (NSGHD). Twenty-nine patients, aged 5-17 years, with height below the 5th percentile, delayed bone age and no organic disease were studied. GH status was evaluated by [1] a combined hormonal stimulation test (CHST) of sequentially administered insulin, GnRH, TRH, and L-Dopa, [2] 8-hour overnight GH test (CGH) and [3] IV (1-44)hp GHRH-NH2 infusion. Fourteen patients with growth rate (GR) of 3.7±1.1 cm/yr had a normal GH response on CHST (12.9 ± 4.5 ng/ml.) and GHRH (24.6±12.6) but subnormal OGH (2.6±0.7) compatible with NSGHD. Five patients with GR of 2.8±0.6 cm/yr had a subnormal GH response on CHST (2.6±1.4) and OGH(1.6±0.3) but a normal GH response to GHRH (11.3±5.7) compatible with GHRH deficiency. Two patients with a GR of 3.0 cm/yr had subnormal responses on CHST (2.3), OGH (1.3) and GHRH (1.6) compatible with pituitary GH deficiency. Eight patients with GR of 4.9±1.3 cm/yr had normal responses on all 3 tests, deficiency is mainly hypothalamic in origin. The most cammon form is the NSGHD with a clinical spectrum from a virtually normal growth rate down to severe growth stunting. GHRH deficiency is a less common, but is more common than a pituitary GH deficiency. This study demonstrates that combined physiologic and pharmacologic tests of GH reserve can determine the origin of the disorder.
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Pugliese, M., Lifshitz, F., Fort, P. et al. HYPOTHALAMIC HYPOSOMATOTROPINISM IN CHILDREN WITH SHORT STATURE. Pediatr Res 21 (Suppl 4), 252 (1987). https://doi.org/10.1203/00006450-198704010-00509
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DOI: https://doi.org/10.1203/00006450-198704010-00509