Growth Hormone Insensitivity

Arlan L. Rosenbloom; Jaime Guevara-Aguirre

doi:10.1007/978-3-319-73782-9_2

Growth Hormone Insensitivity

Arlan L. Rosenbloom^*
, Jaime Guevara-Aguirre

^*Corresponding author for this work

Medicina

University of Florida
Instituto de Endocrinología IEMYR

Research output: Chapter in Book/Report/Conference proceeding › Chapter › peer-review

Abstract

Growth hormone insensitivity (GHI) or resistance is the absence of growth or metabolic response to endogenous GH produced by the liver, or to recombinant human (rh) GH administered in physiologic doses. Genetic disorders that result in GHI are rare and include GH receptor deficiency (GHRD, Laron syndrome), as well as those due to mutations affecting genes of serum transducers and activators of transcription 5B (STAT5b), protein tyrosine phosphatase non-receptor type 11 (PTPN11), acid labile subunit (ALS), insulin-like growth factor-I (IGF-I), the IGF-I receptor, and pappalysin 2 (PAPP-A2). Mutation of cell surface GH binding protein, IGF-I, IGF-I binding protein, and other intracellular growth factors seldom occurs, whereas secondary GH insensitivity due to undernutrition, renal failure, and disease states is common. Limited experience with rhIGF-I therapy in GHRD demonstrates restoration of growth that is less than seen with rhGH replacement in GH deficiency, probably due to the absence of direct effects of GH in bone and muscle.

Original language	English
Title of host publication	Pediatric Endocrinology
Subtitle of host publication	A Practical Clinical Guide
Publisher	Springer Science+Business Media
Pages	31-59
Number of pages	29
ISBN (Electronic)	9783319737829
ISBN (Print)	9783319737812
DOIs	https://doi.org/10.1007/978-3-319-73782-9_2
State	Published - 1 Jan 2018

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

SDG 3 Good Health and Well-being

Keywords

Acid labile subunit (ALS)
GH binding protein (BP)
GHR gene mutation
Growth hormone (GH)
Insulin-like growth factor-I (IGF-I)
Laron syndrome
Noonan syndrome
Pappalysin 2 (PAPP-A2)
Protein tyrosine phosphatase non-receptor type 11 (PTPN11)
Receptor (R)
Recombinant human IGF-I (rhIGF-I)
Serum transducers and activators of transcription 5b (STAT5b)

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10.1007/978-3-319-73782-9_2

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abstract = "Growth hormone insensitivity (GHI) or resistance is the absence of growth or metabolic response to endogenous GH produced by the liver, or to recombinant human (rh) GH administered in physiologic doses. Genetic disorders that result in GHI are rare and include GH receptor deficiency (GHRD, Laron syndrome), as well as those due to mutations affecting genes of serum transducers and activators of transcription 5B (STAT5b), protein tyrosine phosphatase non-receptor type 11 (PTPN11), acid labile subunit (ALS), insulin-like growth factor-I (IGF-I), the IGF-I receptor, and pappalysin 2 (PAPP-A2). Mutation of cell surface GH binding protein, IGF-I, IGF-I binding protein, and other intracellular growth factors seldom occurs, whereas secondary GH insensitivity due to undernutrition, renal failure, and disease states is common. Limited experience with rhIGF-I therapy in GHRD demonstrates restoration of growth that is less than seen with rhGH replacement in GH deficiency, probably due to the absence of direct effects of GH in bone and muscle.",

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KW - Protein tyrosine phosphatase non-receptor type 11 (PTPN11)

KW - Receptor (R)

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KW - Serum transducers and activators of transcription 5b (STAT5b)

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Growth Hormone Insensitivity

Abstract

UN SDGs

Keywords

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