title: Acromegaly and hyperprolactinemia in a patient with polyostotic fibrous dysplasia: dynamic endocrine studies and treatment with the somatostatin analogue octreotide.
abstract: Acromegaly and hyperprolactinemia have been described in association with polyostotic fibrous dysplasia; the pathogenetic mechanisms involved in the development of the endocrinopathies is unknown. We report a 26-year-old man with polyostotic fibrous dysplasia and hypersecretion of GH and PRL. Plasma GH, PRL, and insulin-like growth factor-I (IGF-I) were elevated. Glucose-non-suppressible plasma GH concentrations, GH responsiveness to TRH and GHRH, and GH suppression after a test-dose of somatostatin, octreotide, and bromocriptine were found. Plasma GHRH levels were within the normal range (< 25 ng/l). Computed tomography of the sella turcica and visual fields were normal. [111In-DTPA-D-Phe1]-octreotide scintigraphy were used to localize a possible tumor; no radioactivity was visualized at the site of the hypothalamus, the pituitary or elsewhere in the body but a considerable accumulation of radioactivity was found in the os frontalis. Therapy with octreotide by continuous sc infusion partially suppressed GH and IGF-I (and normalized PRL). The results suggest that hypersecretion of GH in our patient is not due to a GH-secreting pituitary tumor, eutopic or ectopic hypersecretion of GHRH or autonomous somatotroph function. The origin of the disease in this patient might be an abnormal hypothalamic regulation of somatotrophs and/or an alteration in the transmembrane signalling systems.
authors.affiliation: Department of Endocrinology, University Hospital Utrecht, The Netherlands.
authors.forename: M B
authors.initials: MB
authors.lastname: Garcia
authors.forename: H P
authors.initials: HP
authors.lastname: Koppeschaar
authors.forename: C J
authors.initials: CJ
authors.lastname: Lips
authors.forename: J H
authors.initials: JH
authors.lastname: Thijssen
authors.forename: E P
authors.initials: EP
authors.lastname: Krenning
mesh_terms: D000172:Acromegaly / Q000097:blood / Q000150:complications* / Q000188:drug therapy*; D000328:Adult; D001971:Bromocriptine / Q000627:therapeutic use; D005358:Fibrous Dysplasia, Monostotic / Q000097:blood / Q000150:complications* / Q000188:drug therapy*; D013006:Growth Hormone / Q000097:blood; D013007:Growth Hormone-Releasing Hormone / Q000097:blood; D006801:Humans; D006966:Hyperprolactinemia / Q000097:blood / Q000150:complications* / Q000188:drug therapy*; D007279:Injections, Subcutaneous; D007334:Insulin-Like Growth Factor I / Q000032:analysis; D007457:Iodine Radioisotopes; D008297:Male; D015282:Octreotide / Q000008:administration & dosage / Q000627:therapeutic use*; D011388:Prolactin / Q000097:blood; D013004:Somatostatin / Q000627:therapeutic use; D013997:Time Factors
chemical_list: D007457:Iodine Radioisotopes; D001971:Bromocriptine; D013004:Somatostatin; D007334:Insulin-Like Growth Factor I; D011388:Prolactin; D013006:Growth Hormone; D013007:Growth Hormone-Releasing Hormone; D015282:Octreotide