Growth Hormone Excess in Children with Optic Pathway Tumors Is a Transient Phenomenon

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4 Citations (Scopus)

Abstract

Background/Aims: Growth hormone (GH) excess in children with chiasmal optic pathway tumors (OPT), often associated with neurofibromatosis type 1 (NF1), is likely underrecognized. These children have elevated insulin-like growth factor 1 (IGF-1) levels, evidence of rapid growth despite treatment of precocious puberty, and failure to suppress GH levels following oral glucose challenge. The aim of this report is to describe the treatment course and natural history of this rare clinical condition in 7 patients. Methods: This is a descriptive case series of 5 children previously described and 2 additional children more recently diagnosed at our institution. All 7 children had clinical and biochemical evidence of GH excess and received treatment with the somatostatin analog octreotide. Results: Length of treatment varied among the patients. Five of the 7 patients have had resolution of GH excess and currently have normal IGF-1 levels without treatment. Conclusions: Unrestrained GH secretion occurs in a subset of children with OPT with potential adverse outcomes. Since GH excess appears to resolve over time, the benefit of treatment to alter outcomes or prevent tumor progression is unclear.

Original languageEnglish (US)
Pages (from-to)35-38
Number of pages4
JournalHormone Research in Paediatrics
Volume86
Issue number1
DOIs
StatePublished - Aug 1 2016

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Neoplasms
Somatomedins
Growth Hormone
Therapeutics
Precocious Puberty
Neurofibromatosis 1
Octreotide
Somatostatin
Natural History
Growth hormone excess
Glucose
Growth

Keywords

  • Growth hormone excess
  • Neurofibromatosis type 1
  • Optic pathway tumor
  • Somatostatin analogs

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

Cite this

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title = "Growth Hormone Excess in Children with Optic Pathway Tumors Is a Transient Phenomenon",
abstract = "Background/Aims: Growth hormone (GH) excess in children with chiasmal optic pathway tumors (OPT), often associated with neurofibromatosis type 1 (NF1), is likely underrecognized. These children have elevated insulin-like growth factor 1 (IGF-1) levels, evidence of rapid growth despite treatment of precocious puberty, and failure to suppress GH levels following oral glucose challenge. The aim of this report is to describe the treatment course and natural history of this rare clinical condition in 7 patients. Methods: This is a descriptive case series of 5 children previously described and 2 additional children more recently diagnosed at our institution. All 7 children had clinical and biochemical evidence of GH excess and received treatment with the somatostatin analog octreotide. Results: Length of treatment varied among the patients. Five of the 7 patients have had resolution of GH excess and currently have normal IGF-1 levels without treatment. Conclusions: Unrestrained GH secretion occurs in a subset of children with OPT with potential adverse outcomes. Since GH excess appears to resolve over time, the benefit of treatment to alter outcomes or prevent tumor progression is unclear.",
keywords = "Growth hormone excess, Neurofibromatosis type 1, Optic pathway tumor, Somatostatin analogs",
author = "Josefson, {Jami L.} and Robert Listernick and Joel Charrow and Habiby, {Reema L.}",
year = "2016",
month = "8",
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doi = "10.1159/000447326",
language = "English (US)",
volume = "86",
pages = "35--38",
journal = "Hormone Research in Paediatrics",
issn = "1663-2818",
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T1 - Growth Hormone Excess in Children with Optic Pathway Tumors Is a Transient Phenomenon

AU - Josefson, Jami L.

AU - Listernick, Robert

AU - Charrow, Joel

AU - Habiby, Reema L.

PY - 2016/8/1

Y1 - 2016/8/1

N2 - Background/Aims: Growth hormone (GH) excess in children with chiasmal optic pathway tumors (OPT), often associated with neurofibromatosis type 1 (NF1), is likely underrecognized. These children have elevated insulin-like growth factor 1 (IGF-1) levels, evidence of rapid growth despite treatment of precocious puberty, and failure to suppress GH levels following oral glucose challenge. The aim of this report is to describe the treatment course and natural history of this rare clinical condition in 7 patients. Methods: This is a descriptive case series of 5 children previously described and 2 additional children more recently diagnosed at our institution. All 7 children had clinical and biochemical evidence of GH excess and received treatment with the somatostatin analog octreotide. Results: Length of treatment varied among the patients. Five of the 7 patients have had resolution of GH excess and currently have normal IGF-1 levels without treatment. Conclusions: Unrestrained GH secretion occurs in a subset of children with OPT with potential adverse outcomes. Since GH excess appears to resolve over time, the benefit of treatment to alter outcomes or prevent tumor progression is unclear.

AB - Background/Aims: Growth hormone (GH) excess in children with chiasmal optic pathway tumors (OPT), often associated with neurofibromatosis type 1 (NF1), is likely underrecognized. These children have elevated insulin-like growth factor 1 (IGF-1) levels, evidence of rapid growth despite treatment of precocious puberty, and failure to suppress GH levels following oral glucose challenge. The aim of this report is to describe the treatment course and natural history of this rare clinical condition in 7 patients. Methods: This is a descriptive case series of 5 children previously described and 2 additional children more recently diagnosed at our institution. All 7 children had clinical and biochemical evidence of GH excess and received treatment with the somatostatin analog octreotide. Results: Length of treatment varied among the patients. Five of the 7 patients have had resolution of GH excess and currently have normal IGF-1 levels without treatment. Conclusions: Unrestrained GH secretion occurs in a subset of children with OPT with potential adverse outcomes. Since GH excess appears to resolve over time, the benefit of treatment to alter outcomes or prevent tumor progression is unclear.

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KW - Neurofibromatosis type 1

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KW - Somatostatin analogs

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