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Long-Term Treatment With Letrozole in a Boy With Familial Male-Limited Precocious Puberty

BACKGROUND: The long-term follow-up in children with familial male-limited precocious puberty (FMPP) who were treated with letrozole, triptorelin, and spironolactone is limited, especially considering the efficiency and safety. OBJECTIVE: We describe the clinical characteristics and long-term treatm...

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Autores principales: Yuan, Xin, Chen, Ruimin, Zhang, Ying, Yang, Xiaohong, Lin, Xiangquan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9329579/
https://www.ncbi.nlm.nih.gov/pubmed/35909557
http://dx.doi.org/10.3389/fendo.2022.906852
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author Yuan, Xin
Chen, Ruimin
Zhang, Ying
Yang, Xiaohong
Lin, Xiangquan
author_facet Yuan, Xin
Chen, Ruimin
Zhang, Ying
Yang, Xiaohong
Lin, Xiangquan
author_sort Yuan, Xin
collection PubMed
description BACKGROUND: The long-term follow-up in children with familial male-limited precocious puberty (FMPP) who were treated with letrozole, triptorelin, and spironolactone is limited, especially considering the efficiency and safety. OBJECTIVE: We describe the clinical characteristics and long-term treatment with letrozole on adult height of a boy diagnosed with FMPP, confirmed by analysis of the LHCGR gene. METHODS: Physical examinations, bone age (BA), testosterone, and gonadotropin levels were measured as well as gene sequencing of the proband and parents. RESULTS: The boy was referred to the hospital at 3.1 years of age due to peripheral precocious puberty. His height was 116.8cm (+5.1SD) and BA was 9 years. Genetic analysis revealed a patrilineal c.1703C>T.(p.Ala568Val) mutation of the LHCGR gene. After treating with letrozole for 1.6 years, the height according to BA went from -3.52SD to -2.82SD. Triptorelin was added at age 4.7 years based on both the evidence of central puberty and his growth velocity according to BA. During the 6.9 years of treatment, he had a height gain of 51.9cm, and BA increased 5.2 years. At age 10, his present height is 168.7cm (0.05SD) and BA is 14.7 years. No adverse effects of treatment were encountered. CONCLUSION: A patrilineal mutation of the LHCGR gene has been identified in a boy with FMPP. His height is 168.7cm (-0.05SD) which is approaching his adult height after long-term treatment with letrozole, triptorelin, and spironolactone.
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spelling pubmed-93295792022-07-29 Long-Term Treatment With Letrozole in a Boy With Familial Male-Limited Precocious Puberty Yuan, Xin Chen, Ruimin Zhang, Ying Yang, Xiaohong Lin, Xiangquan Front Endocrinol (Lausanne) Endocrinology BACKGROUND: The long-term follow-up in children with familial male-limited precocious puberty (FMPP) who were treated with letrozole, triptorelin, and spironolactone is limited, especially considering the efficiency and safety. OBJECTIVE: We describe the clinical characteristics and long-term treatment with letrozole on adult height of a boy diagnosed with FMPP, confirmed by analysis of the LHCGR gene. METHODS: Physical examinations, bone age (BA), testosterone, and gonadotropin levels were measured as well as gene sequencing of the proband and parents. RESULTS: The boy was referred to the hospital at 3.1 years of age due to peripheral precocious puberty. His height was 116.8cm (+5.1SD) and BA was 9 years. Genetic analysis revealed a patrilineal c.1703C>T.(p.Ala568Val) mutation of the LHCGR gene. After treating with letrozole for 1.6 years, the height according to BA went from -3.52SD to -2.82SD. Triptorelin was added at age 4.7 years based on both the evidence of central puberty and his growth velocity according to BA. During the 6.9 years of treatment, he had a height gain of 51.9cm, and BA increased 5.2 years. At age 10, his present height is 168.7cm (0.05SD) and BA is 14.7 years. No adverse effects of treatment were encountered. CONCLUSION: A patrilineal mutation of the LHCGR gene has been identified in a boy with FMPP. His height is 168.7cm (-0.05SD) which is approaching his adult height after long-term treatment with letrozole, triptorelin, and spironolactone. Frontiers Media S.A. 2022-07-14 /pmc/articles/PMC9329579/ /pubmed/35909557 http://dx.doi.org/10.3389/fendo.2022.906852 Text en Copyright © 2022 Yuan, Chen, Zhang, Yang and Lin https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Endocrinology
Yuan, Xin
Chen, Ruimin
Zhang, Ying
Yang, Xiaohong
Lin, Xiangquan
Long-Term Treatment With Letrozole in a Boy With Familial Male-Limited Precocious Puberty
title Long-Term Treatment With Letrozole in a Boy With Familial Male-Limited Precocious Puberty
title_full Long-Term Treatment With Letrozole in a Boy With Familial Male-Limited Precocious Puberty
title_fullStr Long-Term Treatment With Letrozole in a Boy With Familial Male-Limited Precocious Puberty
title_full_unstemmed Long-Term Treatment With Letrozole in a Boy With Familial Male-Limited Precocious Puberty
title_short Long-Term Treatment With Letrozole in a Boy With Familial Male-Limited Precocious Puberty
title_sort long-term treatment with letrozole in a boy with familial male-limited precocious puberty
topic Endocrinology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9329579/
https://www.ncbi.nlm.nih.gov/pubmed/35909557
http://dx.doi.org/10.3389/fendo.2022.906852
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