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From dwarves to giants: South American's contribution to the history of growth hormone and related disorders
Growth Hormone and IGF Research ( IF 1.6 ) Pub Date : 2019-12-02 , DOI: 10.1016/j.ghir.2019.11.004
Cesar Luiz Boguszewski 1 , Margaret Cristina da Silva Boguszewski 2 , Wouter W de Herder 3
Affiliation  

The aim of this article is to present a historical review on giants and dwarves living in South America and the contribution of South America's researchers to scientific advances on growth hormone (GH) and human disorders related to GH excess and GH deficiency (GHD). We went back in time to investigate facts and myths stemming from countless reports of giants who lived in the Patagonia region, focusing on what is currently known about gigantism in South America. Additionally, we have reviewed the exceptional work carried out in two of the world's largest cohorts of dwarfism related to GH-IGF axis: one living in Itabaianinha, Brazil, suffering from severe GHD due to a mutation in the GHRH receptor (GHRHR) gene, and the other living in El Oro and Loja provinces of Ecuador, who are carriers of GH receptor gene mutation that causes total GH insensitivity (Laron syndrome). Importantly, we present an overview of the outstanding medical contribution of Jose Dantas de Souza Leite, a Brazilian physician that described the first cases of acromegaly, and Bernardo Alberto Houssay, an Argentine researcher graced with the Nobel Prize, who was one the first scientists to establish a link between GH and glucose metabolism.



中文翻译:

从矮人到巨人:南美对生长激素和相关疾病史的贡献

本文的目的是对生活在南美的巨人和矮人进行历史回顾,以及南美研究人员对生长激素(GH)和与GH过量和GH缺乏(GHD)相关的人类疾病的科学进步的贡献。我们回溯过去,调查了生活在巴塔哥尼亚地区的巨人的无数报道所产生的事实和神话,着眼于目前对南美巨人主义的了解。此外,我们还回顾了出色的工作,在这两个世界上最大的有关GH-IGF侏儒症的同伙进行轴:一个生活在伊塔巴亚尼尼亚,巴西,严重GHD痛苦由于GHRH受体突变(GHRHR)基因,另一个居住在厄瓜多尔的El Oro和Loja省,它们是GH受体基因突变的携带者,导致全GH不敏感(Laron综合征)。重要的是,我们概述了描述了首例肢端肥大症的巴西医生Jose Dantas de Souza Leite的杰出医学贡献,以及获得诺贝尔奖的阿根廷研究员Bernardo Alberto Houssay,他是第一位获得诺贝尔奖的科学家建立GH和葡萄糖代谢之间的联系。

更新日期:2019-12-02
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