title: [Acromegaly associated with McCune-Albright syndrome].
abstract: McCune-Albright syndrome is characterized by polyostotic fibrous dysplasia, cafe au lait pigmentation of the skin, and multiple endocrinopathies. The authors report a history of a 30-years-old man who had a pathologic humerus fracture at the age of 14 years. The diagnosis of polyostotic fibrous dysplasia was established by radiologic examinations and bone biopsy. Fourteen years thereafter, active acromegaly due to a pituitary microadenoma was diagnosed using hormone measurements and pituitary magnetic resonance imaging. Pituitary surgery was refused because of an extensive skull involvement caused by the fibrous dysplasia. After an unsuccessful therapy with bromocriptine lasting three months, long-acting octreotide (Sandostatin LAR, Novartis) treatment was started. After a 12-months course of treatment, serum growth hormone levels markedly decreased, clinical symptoms improved, but serum insulin-like growth factor I levels remained unchanged. These observations that serum insulin-like growth factor I levels failed to reflect the decrease of serum growth hormone concentrations after long-acting octreotide treatment suggest that the increased production of insulin-like growth factor I in patients with acromegaly due to McCune-Albright syndrome may involve mechanism(s) other than increased growth hormone levels.
authors.affiliation: AOK II. Belgyógyászati Klinika, Semmelweis Egyetem, Budapest.
authors.forename: Miklós
authors.initials: M
authors.lastname: Tóth
authors.forename: Judit
authors.initials: J
authors.lastname: Töke
authors.forename: Edit
authors.initials: E
authors.lastname: Kiss
authors.forename: Irén
authors.initials: I
authors.lastname: Bernád
authors.forename: Pál
authors.initials: P
authors.lastname: Miheller
authors.forename: Nikolette
authors.initials: N
authors.lastname: Szücs
authors.forename: Károly
authors.initials: K
authors.lastname: Rácz
mesh_terms: D000172:Acromegaly / Q000188:drug therapy* / Q000209:etiology*; D000236:Adenoma / Q000097:blood / Q000150:complications; D000328:Adult; D000970:Antineoplastic Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D003692:Delayed-Action Preparations; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D006728:Hormones / Q000627:therapeutic use; D019382:Human Growth Hormone / Q000097:blood; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000378:metabolism; D008297:Male; D015282:Octreotide / Q000008:administration & dosage / Q000627:therapeutic use*; D010911:Pituitary Neoplasms / Q000097:blood / Q000150:complications; D016896:Treatment Outcome; D016312:Treatment Refusal
chemical_list: D000970:Antineoplastic Agents; D003692:Delayed-Action Preparations; D006728:Hormones; D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I; D015282:Octreotide
vernacular_title: Acromegalia McCune-Albright szindrómában.