GH Responsiveness in Children With Noonan Syndrome Compared to Turner Syndrome

BackgroundDespite different genetic background, Noonan syndrome (NS) shares similar phenotype features to Turner syndrome (TS) such as short stature, webbed neck and congenital heart defects. TS is an entity with decreased growth hormone (GH) responsiveness. Whether this is found in NS is debated.Me...

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Autores principales:	Jovanna Dahlgren, Kerstin Albertsson-Wikland
Formato:	article
Lenguaje:	EN
Publicado:	Frontiers Media S.A. 2021
Materias:	growth hormone growth response height IGFI IGFBP3 Noonan syndrome Diseases of the endocrine glands. Clinical endocrinology RC648-665
Acceso en línea:	https://doaj.org/article/1ed29ffb3a4b4255908b7eba6bff9e17
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spelling	oai:doaj.org-article:1ed29ffb3a4b4255908b7eba6bff9e172021-11-09T15:00:39ZGH Responsiveness in Children With Noonan Syndrome Compared to Turner Syndrome1664-239210.3389/fendo.2021.737893https://doaj.org/article/1ed29ffb3a4b4255908b7eba6bff9e172021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fendo.2021.737893/fullhttps://doaj.org/toc/1664-2392BackgroundDespite different genetic background, Noonan syndrome (NS) shares similar phenotype features to Turner syndrome (TS) such as short stature, webbed neck and congenital heart defects. TS is an entity with decreased growth hormone (GH) responsiveness. Whether this is found in NS is debated.MethodsData were retrieved from combined intervention studies including 25 children diagnosed with NS, 40 diagnosed with TS, and 45 control children (all prepubertal). NS-children and TS-girls were rhGH treated after investigation of the GH/IGFI-axis. GH was measured with poly- and monoclonal antibodies; 24hGH-profile pattern analysed by PULSAR. The NS-children were randomly assigned to Norditropin® 33 or 66 μg/kg/day, and TS-girls were consecutively treated with Genotropin® 33 or 66 μg/kg/day.ResultsHigher PULSAR-estimates of 24h-profiles were found in both NS-children and TS-girls compared to controls: Polyclonal GHmax24h-profile (Mean ± SD) was higher in both groups (44 ± 23mU/L, p<0.01 in NS; 51 ± 47, p<0.001 in TS; compared to 30 ± 23 mU/L in controls) as was GH-baseline (1.4 ± 0.6 mU/L in NS; 2.4 ± 2.4 mU/L in TS, p<0.01 for both, compared to 1.1 ± 1.2 mU/L in controls). Pre-treatment IGFISDS was 2.2 lower in NS-children (-1.7 ± 1.3) compared to TS-girls (0.6 ± 1.8, p<0.0001). GHmax, IGFI/IGFBP3-ratioSDS, and chronological age at start of GH accounted for 59% of the variance in first-year growth response in NS.ConclusionBoth prepubertal NS-children and TS-girls had a high GH secretion, but low IGFI/IGFBP3 levels only in NS-children. Both groups presented a broad individual response. NS-children showed higher response in IGFI and growth, pointing to higher responsiveness to GH treatment than TS-girls.Jovanna DahlgrenKerstin Albertsson-WiklandFrontiers Media S.A.articlegrowth hormonegrowth responseheightIGFIIGFBP3Noonan syndromeDiseases of the endocrine glands. Clinical endocrinologyRC648-665ENFrontiers in Endocrinology, Vol 12 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	growth hormone growth response height IGFI IGFBP3 Noonan syndrome Diseases of the endocrine glands. Clinical endocrinology RC648-665
spellingShingle	growth hormone growth response height IGFI IGFBP3 Noonan syndrome Diseases of the endocrine glands. Clinical endocrinology RC648-665 Jovanna Dahlgren Kerstin Albertsson-Wikland GH Responsiveness in Children With Noonan Syndrome Compared to Turner Syndrome
description	BackgroundDespite different genetic background, Noonan syndrome (NS) shares similar phenotype features to Turner syndrome (TS) such as short stature, webbed neck and congenital heart defects. TS is an entity with decreased growth hormone (GH) responsiveness. Whether this is found in NS is debated.MethodsData were retrieved from combined intervention studies including 25 children diagnosed with NS, 40 diagnosed with TS, and 45 control children (all prepubertal). NS-children and TS-girls were rhGH treated after investigation of the GH/IGFI-axis. GH was measured with poly- and monoclonal antibodies; 24hGH-profile pattern analysed by PULSAR. The NS-children were randomly assigned to Norditropin® 33 or 66 μg/kg/day, and TS-girls were consecutively treated with Genotropin® 33 or 66 μg/kg/day.ResultsHigher PULSAR-estimates of 24h-profiles were found in both NS-children and TS-girls compared to controls: Polyclonal GHmax24h-profile (Mean ± SD) was higher in both groups (44 ± 23mU/L, p<0.01 in NS; 51 ± 47, p<0.001 in TS; compared to 30 ± 23 mU/L in controls) as was GH-baseline (1.4 ± 0.6 mU/L in NS; 2.4 ± 2.4 mU/L in TS, p<0.01 for both, compared to 1.1 ± 1.2 mU/L in controls). Pre-treatment IGFISDS was 2.2 lower in NS-children (-1.7 ± 1.3) compared to TS-girls (0.6 ± 1.8, p<0.0001). GHmax, IGFI/IGFBP3-ratioSDS, and chronological age at start of GH accounted for 59% of the variance in first-year growth response in NS.ConclusionBoth prepubertal NS-children and TS-girls had a high GH secretion, but low IGFI/IGFBP3 levels only in NS-children. Both groups presented a broad individual response. NS-children showed higher response in IGFI and growth, pointing to higher responsiveness to GH treatment than TS-girls.
format	article
author	Jovanna Dahlgren Kerstin Albertsson-Wikland
author_facet	Jovanna Dahlgren Kerstin Albertsson-Wikland
author_sort	Jovanna Dahlgren
title	GH Responsiveness in Children With Noonan Syndrome Compared to Turner Syndrome
title_short	GH Responsiveness in Children With Noonan Syndrome Compared to Turner Syndrome
title_full	GH Responsiveness in Children With Noonan Syndrome Compared to Turner Syndrome
title_fullStr	GH Responsiveness in Children With Noonan Syndrome Compared to Turner Syndrome
title_full_unstemmed	GH Responsiveness in Children With Noonan Syndrome Compared to Turner Syndrome
title_sort	gh responsiveness in children with noonan syndrome compared to turner syndrome
publisher	Frontiers Media S.A.
publishDate	2021
url	https://doaj.org/article/1ed29ffb3a4b4255908b7eba6bff9e17
work_keys_str_mv	AT jovannadahlgren ghresponsivenessinchildrenwithnoonansyndromecomparedtoturnersyndrome AT kerstinalbertssonwikland ghresponsivenessinchildrenwithnoonansyndromecomparedtoturnersyndrome
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GH Responsiveness in Children With Noonan Syndrome Compared to Turner Syndrome

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