title: Difficulties in diagnosis and treatment of acromegaly in a patient with a McCune-Albright syndrome. A case report and a review of literature.
abstract: We describe a female patient aged 43, who at the age of five was diagnosed with polyostotic fibrous dysplasia (FD). The patient was intermittently treated in our department since the age 33, for approximately 10 years, with intravenous bisphosphonates. At the age of 42 acromegaly was diagnosed incidentally, since clinical manifestations were poor, and, if present earlier, they had been related to FD. Only retrospectively, having biochemical confirmation of GH excess, we could relate them to acromegaly. Because of the involvement of the base of the skull there was no possibility of transphenoidal surgery. Long-acting somatostatin analogues were started, but no response was observed, with IGF-1 and GH being even higher during than before treatment. After the 37-year-history of FD, the occurrence of additional endocrine disorder enabled to make diagnosis of McCune-Albright syndrome (MAS) even in the absence of two out of three classical manifestations such as café-au-lait skin pigmentation and peripheral precocious puberty in the past medical history.
authors.affiliation: Department of Endocrinology, Metabolism and Internal Medicine, Karol Marcinkowski University of Medical Sciences, Poznań, Poland. dbblaszyk@ump.edu.pl
authors.forename: Daria
authors.initials: D
authors.lastname: Baszko-Błaszyk
authors.forename: Julia
authors.initials: J
authors.lastname: Slynko
authors.forename: Włodzimierz
authors.initials: W
authors.lastname: Liebert
authors.forename: Piotr
authors.initials: P
authors.lastname: Sosnowski
authors.forename: Jerzy
authors.initials: J
authors.lastname: Sowiński
authors.forename: Ryszard
authors.initials: R
authors.lastname: Waśko
mesh_terms: D000172:Acromegaly / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000188:drug therapy*; D000328:Adult; D050071:Bone Density Conservation Agents / Q000008:administration & dosage*; D003937:Diagnosis, Differential; D004164:Diphosphonates / Q000008:administration & dosage*; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000188:drug therapy*; D006801:Humans; D012886:Skull / Q000000981:diagnostic imaging; D013004:Somatostatin / Q000008:administration & dosage*; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D017211:Treatment Failure
chemical_list: D050071:Bone Density Conservation Agents; D004164:Diphosphonates; D013004:Somatostatin