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


METHODS
A case-control study was conducted.


METHODS
Four adult men with aromatase deficiency were compared with 12 normal subjects.


METHODS
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.
Rochira
Vincenzo
V
Integrated Department of Medicine, Endocrinology, Metabolism, and Geriatrics, Chair of Endocrinology, University of Modena and Reggio Emilia, Via Giardini 1355, 41100 Modena, Italy. cesare.carani@unimore.it.
Zirilli
Lucia
L
Maffei
Laura
L
Premrou
Valeria
V
Aranda
Claudio
C
Baldi
Matteo
M
Ghigo
Ezio
E
Aimaretti
Gianluca
G
Carani
Cesare
C
Lanfranco
Fabio
F
D000328:Adult; D001120:Arginine; D001141:Aromatase / Q000172:deficiency* / Q000235:genetics; D001786:Blood Glucose / Q000378:metabolism; D001827:Body Height / Q000235:genetics / Q000502:physiology*; D015992:Body Mass Index; D001846:Bone Development / Q000235:genetics / Q000502:physiology; D016022:Case-Control Studies; D004958:Estradiol / Q000097:blood; D015914:Estrogen Replacement Therapy; D004967:Estrogens / Q000494:pharmacology; D006132:Growth Plate / Q000187:drug effects / Q000254:growth & development; D019382:Human Growth Hormone / Q000378:metabolism*; D006801:Humans; D007328:Insulin / Q000097:blood; D007334:Insulin-Like Growth Factor I / Q000378:metabolism; D008297:Male; D011388:Prolactin / Q000097:blood; D011994:Recombinant Proteins; D013739:Testosterone / Q000172:deficiency
D001786:Blood Glucose; D004967:Estrogens; D007328:Insulin; D011994:Recombinant Proteins; D019382:Human Growth Hormone; D013739:Testosterone; D004958:Estradiol; D007334:Insulin-Like Growth Factor I; D011388:Prolactin; D001120:Arginine; D001141:Aromatase