Growth hormone excess in children with neurofibromatosis type 1-associated and sporadic optic pathway tumors

Jami L Josefson*, Robert H Listernick, Jason R Fangusaro, Joel Charrow, Reema L Habiby

*Corresponding author for this work

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

Objective: To describe the clinical manifestations of growth hormone (GH) excess in children with optic pathway tumors (OPT). 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. 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. 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.

Original languageEnglish (US)
Pages (from-to)433-436
Number of pages4
JournalJournal of Pediatrics
Volume158
Issue number3
DOIs
StatePublished - Mar 1 2011

Fingerprint

Neurofibromatosis 1
Precocious Puberty
Growth Hormone
Neoplasms
Hypothalamic Neoplasms
Growth
Leuprolide
Optic Chiasm
Gonadal Steroid Hormones
Somatomedins
Glucose Tolerance Test
Luteinizing Hormone
Brain Neoplasms
Hypothalamus
Growth hormone excess
Intercellular Signaling Peptides and Proteins

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

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title = "Growth hormone excess in children with neurofibromatosis type 1-associated and sporadic optic pathway tumors",
abstract = "Objective: To describe the clinical manifestations of growth hormone (GH) excess in children with optic pathway tumors (OPT). 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. 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. 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.",
author = "Josefson, {Jami L} and Listernick, {Robert H} and Fangusaro, {Jason R} and Joel Charrow and Habiby, {Reema L}",
year = "2011",
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doi = "10.1016/j.jpeds.2010.09.013",
language = "English (US)",
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pages = "433--436",
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T1 - Growth hormone excess in children with neurofibromatosis type 1-associated and sporadic optic pathway tumors

AU - Josefson, Jami L

AU - Listernick, Robert H

AU - Fangusaro, Jason R

AU - Charrow, Joel

AU - Habiby, Reema L

PY - 2011/3/1

Y1 - 2011/3/1

N2 - Objective: To describe the clinical manifestations of growth hormone (GH) excess in children with optic pathway tumors (OPT). 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. 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. 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.

AB - Objective: To describe the clinical manifestations of growth hormone (GH) excess in children with optic pathway tumors (OPT). 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. 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. 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.

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