ID 111685
Title Alternative
ECTOPIC GHRH SYNDROME IN MEN TYPE I
Author
Yamasaki, Ryuichi The University of Tokushima
Saito, Haruhiko The University of Tokushima
Kameyama, Kazuhito The University of Tokushima
Matsumura, Mitsuhiro Tokushima City Hospital
Harada, Kunihiko The University of Tokushima
Saito, Shiro The University of Tokushima
Content Type
Journal Article
Description
A 36-yr-old man with multiple endocrine neoplasia (MEN) type I had an ectopic growth hormone-releasing hormone (GHRH) syndrome due to a GHRH-secreting pancreatic tumor. The immunoreactive (IR)-GHRH concentration in his plasma ranged from 161 to 400pg/ml (299±61pg/ml, mean±SD; normal, 10.4±4.1pg/ml), and a significant correlation was found between his plasma IR-GHRH and GH (r=0.622, p<0.02). After removal of the pancreatic tumor, the high plasma GH concentration returned to nearly the normal range (42.2±31.3 to 9.6±3.8ng/ml). These changes paralleled the normalization of his plasma IR-GHRH (16.1±3.8pg/ml) and some of his symptoms related to acromegaly improved. However, plasma GH (7.7±1.3ng/ml) and IGF-I (591± 22ng/ml) concentrations were high at 12 months after surgery, suggesting adenomatous changes in the pituitary somatotrophs.
Before surgery, exogenous GHRH induced a marked increase in plasma GH, and somatostatin and its agonist (SMS201-995) completely suppressed GH secretion, but not IR-GHRH release. No pulsatile secretion of either IR-GHRH or GH was observed during sleep. An apparent increase in the plasma GH concentration was observed in response to administration of TRH, glucose, arginine or insulin, while plasma IR-GHRH did not show any fluctuation. However, these responses of plasma GH were reduced or no longer observed one month and one year after surgery.
These results indicate that 1) a moderate increase in circulating GHRH due to ectopic secretion from a pancreatic tumor stimulated GH secretion resulting in acromegaly, and evoked GH responses to various provocative tests indistinguishable from those in patients with classical acromegaly, and 2) the ectopic secretion of GHRH may play an etiological role in the pituitary lesion of this patient with MEN type I.
Journal Title
Endocrinologia Japonica
ISSN
21856370
00137219
NCID
AA00635052
Publisher
The Japan Endocrine Society
Volume
35
Issue
1
Start Page
97
End Page
109
Published Date
1988-02
EDB ID
DOI (Published Version)
URL ( Publisher's Version )
FullText File
language
eng
TextVersion
Publisher
departments
Medical Sciences
Oral Sciences