Laron Syndrome

Primary growth hormone (GH) resistance or growth hormone insensitivity syndrome, also called Laron syndrome, is a hereditary disease caused by mutations in the GH receptor or in the post-receptor signaling pathway. This disorder is characterized by postnatal growth failure resembling GH deficiency....

Full description

Saved in:
Bibliographic Details
Published in:Journal of the ASEAN Federation of Endocrine Societies 2023-09, Vol.38 (2)
Main Authors: Niladri Das, Silima Subhasnigdha Tarenia, Souvik Saha, Prashant Manohar Gaikwad, Deep Kamlesh Hathi, Soumik Goswami, Arjun Baidya, Nilanjan Sengupta
Format: Article
Language:English
Subjects:
growth hormone deficiency
Growth hormone insensitivity
Laron syndrome
short stature
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
cited_by
cites
container_end_page
container_issue 2
container_start_page
container_title Journal of the ASEAN Federation of Endocrine Societies
container_volume 38
creator Niladri Das
Silima Subhasnigdha Tarenia
Souvik Saha
Prashant Manohar Gaikwad
Deep Kamlesh Hathi
Soumik Goswami
Arjun Baidya
Nilanjan Sengupta
description Primary growth hormone (GH) resistance or growth hormone insensitivity syndrome, also called Laron syndrome, is a hereditary disease caused by mutations in the GH receptor or in the post-receptor signaling pathway. This disorder is characterized by postnatal growth failure resembling GH deficiency. Differentiating the two conditions is necessary. We present the cases of two siblings, a 16-year-old female and a 9-year-old male, born from a consanguineous union. Both had normal birth weights with subsequent severe short stature and delayed teeth eruption, with no features suggestive of any systemic illness. Serum insulin-like growth factor 1 (IGF1) and insulin-like growth factor binding protein 3 (IGFBP3) were both low. Suspecting GH deficiency, provocative testing with clonidine was done revealing peak growth hormone >40 ng/mL in both patients. In view of low IGF1 and IGFBP3 and high GH on stimulation, IGF1 generation test was done for both siblings, with values supporting the diagnosis of GH insensitivity or Laron syndrome.
doi_str_mv 10.15605/jafes.038.02.22
format article
fullrecord <record><control><sourceid>doaj</sourceid><recordid>TN_cdi_doaj_primary_oai_doaj_org_article_aa48d7ff5ed4437eaf168b646e9f35dd</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><doaj_id>oai_doaj_org_article_aa48d7ff5ed4437eaf168b646e9f35dd</doaj_id><sourcerecordid>oai_doaj_org_article_aa48d7ff5ed4437eaf168b646e9f35dd</sourcerecordid><originalsourceid>FETCH-doaj_primary_oai_doaj_org_article_aa48d7ff5ed4437eaf168b646e9f35dd3</originalsourceid><addsrcrecordid>eNqtzL0KwjAUQOEgChats6Mv0HjT_DqLouCmg1u4mkRa2kYSF99eEB_B6cA3HEKWDCiTCuS6xeAzBW4o1LSuR6SoOZiKMXMdkwKM1BUDLaakzLkFAAaSK8UKsjhhisPq_B5cir2fk0nALvvy1xk57neX7aFyEVv7TE2P6W0jNvYLMT0spldz77xFFMbpEKR3QnDtMTBlbkoovwlcOsf_-foA4OxJew</addsrcrecordid><sourcetype>Open Website</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype></control><display><type>article</type><title>Laron Syndrome</title><source>PubMed</source><creator>Niladri Das ; Silima Subhasnigdha Tarenia ; Souvik Saha ; Prashant Manohar Gaikwad ; Deep Kamlesh Hathi ; Soumik Goswami ; Arjun Baidya ; Nilanjan Sengupta</creator><creatorcontrib>Niladri Das ; Silima Subhasnigdha Tarenia ; Souvik Saha ; Prashant Manohar Gaikwad ; Deep Kamlesh Hathi ; Soumik Goswami ; Arjun Baidya ; Nilanjan Sengupta</creatorcontrib><description>Primary growth hormone (GH) resistance or growth hormone insensitivity syndrome, also called Laron syndrome, is a hereditary disease caused by mutations in the GH receptor or in the post-receptor signaling pathway. This disorder is characterized by postnatal growth failure resembling GH deficiency. Differentiating the two conditions is necessary. We present the cases of two siblings, a 16-year-old female and a 9-year-old male, born from a consanguineous union. Both had normal birth weights with subsequent severe short stature and delayed teeth eruption, with no features suggestive of any systemic illness. Serum insulin-like growth factor 1 (IGF1) and insulin-like growth factor binding protein 3 (IGFBP3) were both low. Suspecting GH deficiency, provocative testing with clonidine was done revealing peak growth hormone &gt;40 ng/mL in both patients. In view of low IGF1 and IGFBP3 and high GH on stimulation, IGF1 generation test was done for both siblings, with values supporting the diagnosis of GH insensitivity or Laron syndrome.</description><identifier>ISSN: 0857-1074</identifier><identifier>EISSN: 2308-118X</identifier><identifier>DOI: 10.15605/jafes.038.02.22</identifier><language>eng</language><publisher>ASEAN Federation of Endocrine Societies</publisher><subject>growth hormone deficiency ; Growth hormone insensitivity ; Laron syndrome ; short stature</subject><ispartof>Journal of the ASEAN Federation of Endocrine Societies, 2023-09, Vol.38 (2)</ispartof><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids></links><search><creatorcontrib>Niladri Das</creatorcontrib><creatorcontrib>Silima Subhasnigdha Tarenia</creatorcontrib><creatorcontrib>Souvik Saha</creatorcontrib><creatorcontrib>Prashant Manohar Gaikwad</creatorcontrib><creatorcontrib>Deep Kamlesh Hathi</creatorcontrib><creatorcontrib>Soumik Goswami</creatorcontrib><creatorcontrib>Arjun Baidya</creatorcontrib><creatorcontrib>Nilanjan Sengupta</creatorcontrib><title>Laron Syndrome</title><title>Journal of the ASEAN Federation of Endocrine Societies</title><description>Primary growth hormone (GH) resistance or growth hormone insensitivity syndrome, also called Laron syndrome, is a hereditary disease caused by mutations in the GH receptor or in the post-receptor signaling pathway. This disorder is characterized by postnatal growth failure resembling GH deficiency. Differentiating the two conditions is necessary. We present the cases of two siblings, a 16-year-old female and a 9-year-old male, born from a consanguineous union. Both had normal birth weights with subsequent severe short stature and delayed teeth eruption, with no features suggestive of any systemic illness. Serum insulin-like growth factor 1 (IGF1) and insulin-like growth factor binding protein 3 (IGFBP3) were both low. Suspecting GH deficiency, provocative testing with clonidine was done revealing peak growth hormone &gt;40 ng/mL in both patients. In view of low IGF1 and IGFBP3 and high GH on stimulation, IGF1 generation test was done for both siblings, with values supporting the diagnosis of GH insensitivity or Laron syndrome.</description><subject>growth hormone deficiency</subject><subject>Growth hormone insensitivity</subject><subject>Laron syndrome</subject><subject>short stature</subject><issn>0857-1074</issn><issn>2308-118X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2023</creationdate><recordtype>article</recordtype><sourceid>DOA</sourceid><recordid>eNqtzL0KwjAUQOEgChats6Mv0HjT_DqLouCmg1u4mkRa2kYSF99eEB_B6cA3HEKWDCiTCuS6xeAzBW4o1LSuR6SoOZiKMXMdkwKM1BUDLaakzLkFAAaSK8UKsjhhisPq_B5cir2fk0nALvvy1xk57neX7aFyEVv7TE2P6W0jNvYLMT0spldz77xFFMbpEKR3QnDtMTBlbkoovwlcOsf_-foA4OxJew</recordid><startdate>20230901</startdate><enddate>20230901</enddate><creator>Niladri Das</creator><creator>Silima Subhasnigdha Tarenia</creator><creator>Souvik Saha</creator><creator>Prashant Manohar Gaikwad</creator><creator>Deep Kamlesh Hathi</creator><creator>Soumik Goswami</creator><creator>Arjun Baidya</creator><creator>Nilanjan Sengupta</creator><general>ASEAN Federation of Endocrine Societies</general><scope>DOA</scope></search><sort><creationdate>20230901</creationdate><title>Laron Syndrome</title><author>Niladri Das ; Silima Subhasnigdha Tarenia ; Souvik Saha ; Prashant Manohar Gaikwad ; Deep Kamlesh Hathi ; Soumik Goswami ; Arjun Baidya ; Nilanjan Sengupta</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-doaj_primary_oai_doaj_org_article_aa48d7ff5ed4437eaf168b646e9f35dd3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2023</creationdate><topic>growth hormone deficiency</topic><topic>Growth hormone insensitivity</topic><topic>Laron syndrome</topic><topic>short stature</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Niladri Das</creatorcontrib><creatorcontrib>Silima Subhasnigdha Tarenia</creatorcontrib><creatorcontrib>Souvik Saha</creatorcontrib><creatorcontrib>Prashant Manohar Gaikwad</creatorcontrib><creatorcontrib>Deep Kamlesh Hathi</creatorcontrib><creatorcontrib>Soumik Goswami</creatorcontrib><creatorcontrib>Arjun Baidya</creatorcontrib><creatorcontrib>Nilanjan Sengupta</creatorcontrib><collection>Directory of Open Access Journals</collection><jtitle>Journal of the ASEAN Federation of Endocrine Societies</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Niladri Das</au><au>Silima Subhasnigdha Tarenia</au><au>Souvik Saha</au><au>Prashant Manohar Gaikwad</au><au>Deep Kamlesh Hathi</au><au>Soumik Goswami</au><au>Arjun Baidya</au><au>Nilanjan Sengupta</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Laron Syndrome</atitle><jtitle>Journal of the ASEAN Federation of Endocrine Societies</jtitle><date>2023-09-01</date><risdate>2023</risdate><volume>38</volume><issue>2</issue><issn>0857-1074</issn><eissn>2308-118X</eissn><abstract>Primary growth hormone (GH) resistance or growth hormone insensitivity syndrome, also called Laron syndrome, is a hereditary disease caused by mutations in the GH receptor or in the post-receptor signaling pathway. This disorder is characterized by postnatal growth failure resembling GH deficiency. Differentiating the two conditions is necessary. We present the cases of two siblings, a 16-year-old female and a 9-year-old male, born from a consanguineous union. Both had normal birth weights with subsequent severe short stature and delayed teeth eruption, with no features suggestive of any systemic illness. Serum insulin-like growth factor 1 (IGF1) and insulin-like growth factor binding protein 3 (IGFBP3) were both low. Suspecting GH deficiency, provocative testing with clonidine was done revealing peak growth hormone &gt;40 ng/mL in both patients. In view of low IGF1 and IGFBP3 and high GH on stimulation, IGF1 generation test was done for both siblings, with values supporting the diagnosis of GH insensitivity or Laron syndrome.</abstract><pub>ASEAN Federation of Endocrine Societies</pub><doi>10.15605/jafes.038.02.22</doi><oa>free_for_read</oa></addata></record>
fulltext fulltext
identifier ISSN: 0857-1074
ispartof Journal of the ASEAN Federation of Endocrine Societies, 2023-09, Vol.38 (2)
issn 0857-1074
2308-118X
language eng
recordid cdi_doaj_primary_oai_doaj_org_article_aa48d7ff5ed4437eaf168b646e9f35dd
source PubMed
subjects growth hormone deficiency
Growth hormone insensitivity
Laron syndrome
short stature
title Laron Syndrome
url http://sfxeu10.hosted.exlibrisgroup.com/loughborough?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-06T15%3A03%3A09IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-doaj&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Laron%20Syndrome&rft.jtitle=Journal%20of%20the%20ASEAN%20Federation%20of%20Endocrine%20Societies&rft.au=Niladri%20Das&rft.date=2023-09-01&rft.volume=38&rft.issue=2&rft.issn=0857-1074&rft.eissn=2308-118X&rft_id=info:doi/10.15605/jafes.038.02.22&rft_dat=%3Cdoaj%3Eoai_doaj_org_article_aa48d7ff5ed4437eaf168b646e9f35dd%3C/doaj%3E%3Cgrp_id%3Ecdi_FETCH-doaj_primary_oai_doaj_org_article_aa48d7ff5ed4437eaf168b646e9f35dd3%3C/grp_id%3E%3Coa%3E%3C/oa%3E%3Curl%3E%3C/url%3E&rft_id=info:oai/&rft_id=info:pmid/&rfr_iscdi=true