Testotoxicosis: gonadotrophin-independent male sexual precocity

Aziz, Abdul; Jafri, Syed M.; Haque, Naeemul

doi:10.1136/pgmj.68.797.225

Cited by 2 publications

(2 citation statements)

References 24 publications

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“…Testotoxicosis is another rare autosomal dominant disorder that is characterized by increased androgen production, as a consequence of the autoactivation of the LH receptor. [ 23 24 ] The hypothalamo-pituitary-adrenal axis is essentially normal in patients with testotoxicosis. Central precocious puberty is a true puberty, wherein there is a premature activation of the hypothalamo-pituitary-gonadal axis.…”

Section: Discussionmentioning

confidence: 99%

Behavioral aggressiveness in boys with sexual precocity

Kulshreshtha

Mehta

Gupta

et al. 2012

Indian J Endocr Metab

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Background:Some boys with sexual precocity are known to have behavioral problems like increased physical and verbal aggression and school and social maladjustments. It is believed to be due to premature androgen exposure. However, it is not clear why only some develop this problem, difference in etiology could be one explanation.Aim:The aim of the study is to assess behavioral aggression in boys with sexual precocity due to different disorders.Materials and Methods:Seven children, ages three to seven years, were enrolled for this study. Two were diagnosed to have congenital adrenal hyperplasia (CAH), three had testotoxicosis, while two had central precocious puberty. Parents of children with precocious puberty underwent the (CASP) questionnaire (children's aggression scale-parent version).Results:Testosterone levels were high in all patients. Parents denied any history of physical or verbal aggression in the two boys with CAH. Their CASP rating was 0. In contrast, the CASP ratings in the two boys with testotoxicosis and the two with precocious puberty for five domains ranged from 3.1 – 24.2, 2.6 – 8.3,1-5.6,0 – 7.1, and 0 – 1, respectively. In the present study, increased aggression was seen among all the patients with testotoxicosis and both with precocious puberty. In contrast, there were no symptoms of either increased verbal or physical aggression in either of the two patients with CAH.Conclusions:The hormonal milieu in the boys with CAH versus those with sexual precocity due to other causes differed in terms of cortisol and androgen precursors. The androgen excess in CAH children was a consequence of cortisol deficiency. It is possible that cortisol sufficiency is required for androgen-mediated behavioral effects.

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Section: Discussionmentioning

confidence: 99%

Behavioral aggressiveness in boys with sexual precocity

Kulshreshtha

Mehta

Gupta

et al. 2012

Indian J Endocr Metab

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“…It generally presents between 2 and 4 years of age, with advanced sexual development. Increased testicular volume and accelerated growth rate are commonly observed [ 2 ]. Testosterone levels are within adult male ranges with low levels of LH and FSH.…”

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confidence: 99%

A Case of Familial Male-Limited Precocious Puberty in a Child With Klinefelter Syndrome

Cornacchia

Bhushan

Arguello

2018

Journal of the Endocrine Society

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Familial male-limited precocious puberty (FMPP) is an autosomal dominant, male-limited disorder that causes peripheral precocious puberty in boys. Klinefelter syndrome (47, XXY) is the most common chromosomal aberration in males with associated infertility, hypogonadism, and learning disability. We report here a case of Klinefelter syndrome in a patient with FMPP. A 6-year-old boy was referred to our pediatric endocrinology department for accelerated linear growth and premature pubic hair development. He was diagnosed with FMPP based on clinical, laboratory, and genetic sequencing. Increased levels of gonadotropins prompted further investigation, leading to a subsequent diagnosis of Klinefelter syndrome through karyotype analysis. This case illustrates that patients with FMPP and elevated gonadotropins should encourage further investigation by physicians. We recommend the use of karyotype analysis in such patients who are not receiving aromatase inhibitor therapy. We hypothesize that his mutation or pretreatment with aromatase inhibitors may have a protective effect on testosterone production and sperm viability.

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Testotoxicosis: gonadotrophin-independent male sexual precocity

Cited by 2 publications

References 24 publications

Behavioral aggressiveness in boys with sexual precocity

Behavioral aggressiveness in boys with sexual precocity

A Case of Familial Male-Limited Precocious Puberty in a Child With Klinefelter Syndrome

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