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

[aromatase deficiency]

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 .