Growth Hormone Insensitivity

Arlan L. Rosenbloom; Timothy P. Foster; Michael Haller; Jaime Guevara-Aguirre

doi:10.1007/978-3-031-66296-6_2

Growth Hormone Insensitivity

Arlan L. Rosenbloom
, Timothy P. Foster
, Michael Haller
, Jaime Guevara-Aguirre

Medicina

University of Florida

Producción científica: Capítulo del libro/informe/acta de congreso › Capítulo › revisión exhaustiva

Resumen

Growth hormone insensitivity (GHI) or resistance is the absence of a 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 mutations affecting genes of signal transducer and activator of transcription 5B (STAT5b), protein tyrosine phosphatase nonreceptor type 11 (PTPN11), acid labile subunit (ALS), insulin-like growth factor-I (IGF-I), the IGF-I receptor, and pappalysin 2 (PAPP-A2). 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.

Idioma original	Inglés
Título de la publicación alojada	Pediatric Endocrinology
Subtítulo de la publicación alojada	A Practical Clinical Guide, Fourth Edition
Editorial	Springer Nature
Páginas	35-72
Número de páginas	38
ISBN (versión digital)	9783031662966
ISBN (versión impresa)	9783031662959
DOI	https://doi.org/10.1007/978-3-031-66296-6_2
Estado	Publicada - 1 ene. 2024

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abstract = "Growth hormone insensitivity (GHI) or resistance is the absence of a 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 mutations affecting genes of signal transducer and activator of transcription 5B (STAT5b), protein tyrosine phosphatase nonreceptor type 11 (PTPN11), acid labile subunit (ALS), insulin-like growth factor-I (IGF-I), the IGF-I receptor, and pappalysin 2 (PAPP-A2). 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 - Noonan syndrome

KW - Pappalysin 2 (PAPP-A2)

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

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KW - Recombinant human IGF-I (rhIGF-I)

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

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PB - Springer Nature

ER -

Growth Hormone Insensitivity

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