title: Clinical characteristics and management of growth hormone excess in patients with McCune-Albright syndrome.
abstract: OBJECTIVE
  McCune-Albright syndrome (MAS) is a sporadic, postzygotic disease presenting with fibrous dysplasia, cafe-au-lait spots and multiple endocrinopathies. Growth hormone (GH) excess is an uncommon but potentially severe complication of MAS. This study aims to describe the clinical manifestations of GH excess in the context of MAS and analyze the responses of these patients to treatments.
  
  
  METHODS
  Retrospective clinical study.
  
  
  METHODS
  Clinical data from 52 MAS patients were analyzed. Serum GH and IGF1 levels, as well as nadir GH levels after an oral glucose tolerance test and alkaline phosphatase (ALP) levels were determined before and after the treatment.
  
  
  RESULTS
  In total, 13 MAS patients (25%) had the complication of GH excess, including 10 males (76.9%). Among them, all had FD, and 6 patients had sphenoidal bone involvement. Visual deficits were present in 8 patients, and hearing deficits were present in 5. Olfactory dysfunction was observed in 3 patients. Evident pituitary adenomas were confirmed in 9 patients by MRI. These patients underwent surgery with or without pretreatment of long-acting somatostatin analogue octreotide, and 6 achieved complete remission. The serum ALP levels decreased significantly after treatment for GH excess.
  
  
  CONCLUSIONS
  MAS with GH excess is more common in male patients. GH excess can lead to more severe skeletal lesions in MAS patients involving more of the craniofacial bones. Complete trans-sphenoidal complete tumor excision with neuronavigational guidance is effective and could lower ALP levels. LAR is recommended as a preoperative treatment and when patients fail to achieve complete remission after surgery.
authors.affiliation: Department of Neurosurgery.
authors.forename: Yong
authors.initials: Y
authors.lastname: Yao
authors.affiliation: Department of Neurosurgery.
authors.forename: Yang
authors.initials: Y
authors.lastname: Liu
authors.affiliation: Key Laboratory of Endocrinology of National Health and Family Planning CommissionDepartment of Endocrinology.
authors.forename: Linjie
authors.initials: L
authors.lastname: Wang
authors.affiliation: Department of Neurosurgery.
authors.forename: Kan
authors.initials: K
authors.lastname: Deng
authors.affiliation: Key Laboratory of Endocrinology of National Health and Family Planning CommissionDepartment of Endocrinology.
authors.forename: Hongbo
authors.initials: H
authors.lastname: Yang
authors.affiliation: Key Laboratory of Endocrinology of National Health and Family Planning CommissionDepartment of Endocrinology.
authors.forename: Lin
authors.initials: L
authors.lastname: Lu
authors.affiliation: Department of RadiologyPeking Union Medical College Hospital, Chinese Academy of Medical Science, Peking Union Medical College, Beijing, China.
authors.forename: Feng
authors.initials: F
authors.lastname: Feng
authors.affiliation: Department of Neurosurgery.
authors.forename: Bing
authors.initials: B
authors.lastname: Xing
authors.affiliation: Department of RadiologyPeking Union Medical College Hospital, Chinese Academy of Medical Science, Peking Union Medical College, Beijing, China.
authors.forename: Hui
authors.initials: H
authors.lastname: You
authors.affiliation: Key Laboratory of Endocrinology of National Health and Family Planning CommissionDepartment of Endocrinology.
authors.forename: Zimeng
authors.initials: Z
authors.lastname: Jin
authors.affiliation: Department of Neurosurgery.
authors.forename: Renzhi
authors.initials: R
authors.lastname: Wang
authors.affiliation: Key Laboratory of Endocrinology of National Health and Family Planning CommissionDepartment of Endocrinology.
authors.forename: Hui
authors.initials: H
authors.lastname: Pan
authors.affiliation: Key Laboratory of Endocrinology of National Health and Family Planning CommissionDepartment of Endocrinology.
authors.forename: Shi
authors.initials: S
authors.lastname: Chen
authors.affiliation: Key Laboratory of Endocrinology of National Health and Family Planning CommissionDepartment of Endocrinology.
authors.forename: Huijuan
authors.initials: H
authors.lastname: Zhu
mesh_terms: D000172:Acromegaly / Q000097:blood / Q000175:diagnosis* / Q000601:surgery; D000236:Adenoma / Q000097:blood / Q000000981:diagnostic imaging* / Q000601:surgery; D000328:Adult; D002648:Child; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000097:blood / Q000175:diagnosis* / Q000601:surgery; D019382:Human Growth Hormone / Q000097:blood*; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000032:analysis*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010911:Pituitary Neoplasms / Q000097:blood / Q000000981:diagnostic imaging* / Q000601:surgery; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I