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Tall stature without growth hormone: four male patients with aromatase deficiency.

Research paper by Vincenzo V Rochira, Lucia L Zirilli, Laura L Maffei, Valeria V Premrou, Claudio C Aranda, Matteo M Baldi, Ezio E Ghigo, Gianluca G Aimaretti, Cesare C Carani, Fabio F Lanfranco

Indexed on: 19 Feb '10Published on: 19 Feb '10Published in: The Journal of clinical endocrinology and metabolism



Abstract

From preliminary observations, GH-IGF-I seems to be compromised in men with aromatase deficiency. The GH deficiency (GHD) coexists paradoxically with tall stature, raising the question whether or not a true GHD is part of this rare syndrome.To evaluate the GH secretion in aromatase-deficient men, their GH response to the GHRH plus arginine (GHRH-ARG) test was compared with that of normal subjects. The effect of estrogen replacement treatment on the GH-IGF-I axis in aromatase-deficient men was evaluated before and during therapy.A case-control study was conducted.Four adult men with aromatase deficiency were compared with 12 normal subjects.We measured the GH response to GHRH-ARG in aromatase-deficient men (at baseline and during estrogen treatment) and in normal subjects. Basal serum IGF-I was measured in both patients and controls.The response of GH to GHRH-ARG was severely impaired in men with aromatase deficiency and resulted in significantly lower (P < 0.001) levels than in normal subjects. Although normal, serum IGF-I levels were also significantly lower (P < 0.001) than in normal subjects. Both GH peak and IGF-I concentrations were not modified by estrogen therapy in men with aromatase deficiency.In aromatase-deficient men, GH response to potent provocative stimuli is impaired and is not restored by exogenous estrogens. Furthermore, a tall stature may be reached, notwithstanding the coexistence of GHD, if a prolonged time for growth is available due to a delay in bone maturation, and other growth factors different from GH (mainly insulin) promote growth.