patient helvetica, pill sans-serif; font-size: 1em; background-color: #92d2dd; border: medium solid #333333; text-align: center;">Puberty is delayed among teenagers with ataxia telangiectasia.
It is even more difficult to handle psychologically as their reproductive system is also altered.
Puberty is already a difficult passage in normal times, but it is even harder to live for children with AT as their motor problems have already taken over their lives. They also face another problem: delayed puberty like in many chronic diseases and a reproductive system which is adversely affected by Ataxia Télangienctasia.
Girls
The ovary contains a stock of oocytes from intrauterine life (which will then become ovums). Thus, at birth, it is of 1 to 2 million and at puberty, of 400 000. It is less than 1000 at menopause. Girls with ataxia telangiectasia have small ovaries devoid of this initial stock: this is called premature ovarian failure which unfortunately results in sterility.
Boys
The process is similar, spermatogenesis (the process that led to the production of male spermatozoon) being blocked from the outset. Infertility is, again, inevitable.
In the already very dark picture of ataxia telangiectasia, it is clear that the announcement is not without effect, so that one may wonder whether to do it. It requires in any case psychological support (see Psychologist").
In addition, hormonal deficiencies can cause developmental delays of sexual characteristics and present long-term adverse effects, with for example the risk of osteoporosis in women. The advisability of hormone replacement therapy is to discuss with the doctor, all opinions not being consistent.
