CONTEXT: GH excess is a serious complication of McCune-Albright syndrome (MAS) and has been associated with craniofacial morbidity. OBJECTIVE: The aim of the study was to determine whether early diagnosis and treatment of MAS-associated GH excess prevents optic neuropathy and hearing impairment, the major morbidities associated with GH excess. DESIGN AND SETTING: A retrospective cross-sectional analysis was conducted at a clinical research center. PATIENTS: Twenty-two subjects with MAS-associated GH excess and 21 control MAS subjects without GH excess were included in the study. INTERVENTION: Biochemical testing included random GH, nadir GH after glucose load, nadir GH on frequent sampling, and IGF-I Z-score. Subjects underwent imaging, ophthalmological, audiological, and otolaryngological assessment. Treatment included octreotide, pegvisomant, transphenoidal surgery, and/or radiotherapy as indicated. MAIN OUTCOME MEASURE: Association of optic neuropathy and hearing impairment to age at GH excess diagnosis/treatment was measured. RESULTS: Of 129 MAS subjects, 26 (20%) were diagnosed with GH excess based on elevation of two measures of GH function. Of these, 22 subjects were candidates for pharmacological intervention. Optic neuropathy was significantly correlated with intervention status, with no cases in the early intervention group (diagnosed/treated before age 18) or the control group, and four of seven (57%) in the late intervention group (diagnosed/treated after age 18) (Fisher's exact test; odds ratio, 0.027; P = 0.0058). Early diagnosis/intervention was not associated with reduction in hearing deficits (odds ratio, 1.25; P = 1.00). Mean head circumference SD score was significantly higher in the late (6.08; range, 2.70 to 22.56) than the early intervention (2.67; range, -0.65 to 6.72) or control groups (2.13; range, -2.06 to 7.79) (P = 0.003). CONCLUSIONS: Early diagnosis/treatment of GH excess in MAS is important to prevent optic neuropathy and craniofacial expansion. The relationship between hearing deficits and GH excess remains less clear and requires further study.
CONTEXT: GH excess is a serious complication of McCune-Albright syndrome (MAS) and has been associated with craniofacial morbidity. OBJECTIVE: The aim of the study was to determine whether early diagnosis and treatment of MAS-associated GH excess prevents optic neuropathy and hearing impairment, the major morbidities associated with GH excess. DESIGN AND SETTING: A retrospective cross-sectional analysis was conducted at a clinical research center. PATIENTS: Twenty-two subjects with MAS-associated GH excess and 21 control MAS subjects without GH excess were included in the study. INTERVENTION: Biochemical testing included random GH, nadir GH after glucose load, nadir GH on frequent sampling, and IGF-I Z-score. Subjects underwent imaging, ophthalmological, audiological, and otolaryngological assessment. Treatment included octreotide, pegvisomant, transphenoidal surgery, and/or radiotherapy as indicated. MAIN OUTCOME MEASURE: Association of optic neuropathy and hearing impairment to age at GH excess diagnosis/treatment was measured. RESULTS: Of 129 MAS subjects, 26 (20%) were diagnosed with GH excess based on elevation of two measures of GH function. Of these, 22 subjects were candidates for pharmacological intervention. Optic neuropathy was significantly correlated with intervention status, with no cases in the early intervention group (diagnosed/treated before age 18) or the control group, and four of seven (57%) in the late intervention group (diagnosed/treated after age 18) (Fisher's exact test; odds ratio, 0.027; P = 0.0058). Early diagnosis/intervention was not associated with reduction in hearing deficits (odds ratio, 1.25; P = 1.00). Mean head circumference SD score was significantly higher in the late (6.08; range, 2.70 to 22.56) than the early intervention (2.67; range, -0.65 to 6.72) or control groups (2.13; range, -2.06 to 7.79) (P = 0.003). CONCLUSIONS: Early diagnosis/treatment of GH excess in MAS is important to prevent optic neuropathy and craniofacial expansion. The relationship between hearing deficits and GH excess remains less clear and requires further study.
Authors: Sunday O Akintoye; Marilyn H Kelly; Beth Brillante; Natasha Cherman; Sarah Turner; John A Butman; Pamela G Robey; Michael T Collins Journal: J Clin Endocrinol Metab Date: 2006-05-23 Impact factor: 5.958
Authors: Helen Madsen; Manuel Thomas Borges; Janice M Kerr; Kevin O Lillehei; B K Kleinschmidt-Demasters Journal: J Neurooncol Date: 2010-11-21 Impact factor: 4.130
Authors: Michael T Collins; Harvey Kushner; James C Reynolds; Caroline Chebli; Marilyn H Kelly; Anurag Gupta; Beth Brillante; Arabella I Leet; Mara Riminucci; Pamela Gehron Robey; Paolo Bianco; Shlomo Wientroub; Clara C Chen Journal: J Bone Miner Res Date: 2004-11-16 Impact factor: 6.741
Authors: M L Hartman; J D Veldhuis; M L Vance; A C Faria; R W Furlanetto; M O Thorner Journal: J Clin Endocrinol Metab Date: 1990-05 Impact factor: 5.958
Authors: Janice S Lee; Edmond FitzGibbon; John A Butman; Craig R Dufresne; Harvey Kushner; Shlomo Wientroub; Pamela G Robey; Michael T Collins Journal: N Engl J Med Date: 2002-11-21 Impact factor: 91.245
Authors: W Patricia Bandettini; Alexander S Karageorgiadis; Ninet Sinaii; Douglas R Rosing; Vandana Sachdev; Marie Helene Schernthaner-Reiter; Evgenia Gourgari; Georgios Z Papadakis; Meg F Keil; Charalampos Lyssikatos; J Aidan Carney; Andrew E Arai; Maya Lodish; Constantine A Stratakis Journal: Endocr Relat Cancer Date: 2016-07-18 Impact factor: 5.678
Authors: Alison M Boyce; Carmen Brewer; Timothy R DeKlotz; Christopher K Zalewski; Kelly A King; Michael T Collins; H Jeffrey Kim Journal: JAMA Otolaryngol Head Neck Surg Date: 2018-02-01 Impact factor: 6.223