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Growth hormone excess in children with neurofibromatosis type 1-associated and sporadic optic pathway tumors.
- Source :
-
The Journal of pediatrics [J Pediatr] 2011 Mar; Vol. 158 (3), pp. 433-6. Date of Electronic Publication: 2010 Oct 28. - Publication Year :
- 2011
-
Abstract
- Objective: To describe the clinical manifestations of growth hormone (GH) excess in children with optic pathway tumors (OPT).<br />Study Design: Descriptive case series of 5 children with OPT, 3 with associated neurofibromatosis type 1, referred for evaluation of accelerated linear growth. GH excess was evaluated by oral glucose tolerance tests with frequent sampling of GH levels. Precocious puberty was evaluated by basal luteinizing hormone and sex steroid hormone levels. Stimulation testing with leuprolide acetate (20 μg/kg subcutaneously) was conducted in patients with normal baseline testing.<br />Results: All patients had OPT involving both the hypothalamus and optic chiasm. All patients had elevated levels of the growth factor insulin-like growth factor 1 and on stimulation testing demonstrated an inability to suppress GH levels to < 1.0 ng/mL, indicating the presence of unregulated GH secretion. Additionally, all patients displayed biochemical evidence of precocious puberty.<br />Conclusions: GH excess may be an under-recognized occurrence in the setting of neurofibromatosis type 1 and OPT. GH excess in such patients may contribute to continued brain tumor growth. Given the potential adverse consequences of unrestrained GH excess, all children with chiasmal or hypothalamic tumors who have rapid growth should be evaluated for both precocious puberty and GH excess.<br /> (Copyright © 2011 Mosby, Inc. All rights reserved.)
Details
- Language :
- English
- ISSN :
- 1097-6833
- Volume :
- 158
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- The Journal of pediatrics
- Publication Type :
- Academic Journal
- Accession number :
- 21030036
- Full Text :
- https://doi.org/10.1016/j.jpeds.2010.09.013