Télangiectasie ataxia
It is about a immunizing Déficit congenital, combined, called also syndrome of Denise Louis-Bar.
The code CIM-10 is G11.3
It is transmitted according to the recessive autosomic mode and appears as of the first childhood.
The disease associates a cerebelleuse Ataxie, télangiectasies, a gonadic disgenesis, and a immunizing Déficit. This last is responsible for frequent infections, in particular bronchopulmonary.
There exists sometimes of the complications with type of neoplasic affections (Leucémie, Lymphome).
Many treatments were successively proposed, of inconstant effectiveness.
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