CONTEXT: 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.OBJECTIVE: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.DESIGN AND SETTING:A case-control study was conducted.PATIENTS:Four adult men with aromatase deficiency were compared with 12 normal subjects.MAIN OUTCOME MEASURES: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.RESULTS: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.CONCLUSIONS: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.

Tall stature without growth hormone: four male patients with aromatase deficiency / Rochira, Vincenzo; Zirilli, Lucia; L., Maffei; V., Premrou; C., Aranda; M., Baldi; E., Ghigo; G., Aimaretti; Carani, Cesare; F., Lanfranco. - In: THE JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM. - ISSN 0021-972X. - STAMPA. - 95:(2010), pp. 1626-1633. [10.1210/jc.2009-1743]

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

ROCHIRA, Vincenzo;ZIRILLI, Lucia;CARANI, Cesare;
2010

Abstract

CONTEXT: 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.OBJECTIVE: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.DESIGN AND SETTING:A case-control study was conducted.PATIENTS:Four adult men with aromatase deficiency were compared with 12 normal subjects.MAIN OUTCOME MEASURES: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.RESULTS: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.CONCLUSIONS: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.
2010
95
1626
1633
Tall stature without growth hormone: four male patients with aromatase deficiency / Rochira, Vincenzo; Zirilli, Lucia; L., Maffei; V., Premrou; C., Aranda; M., Baldi; E., Ghigo; G., Aimaretti; Carani, Cesare; F., Lanfranco. - In: THE JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM. - ISSN 0021-972X. - STAMPA. - 95:(2010), pp. 1626-1633. [10.1210/jc.2009-1743]
Rochira, Vincenzo; Zirilli, Lucia; L., Maffei; V., Premrou; C., Aranda; M., Baldi; E., Ghigo; G., Aimaretti; Carani, Cesare; F., Lanfranco
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11380/708957
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