Omenn syndrome: a lack of tolerance on the background of deficient lymphocyte development and maturation

M Hönig, K Schwarz - Current opinion in rheumatology, 2006 - journals.lww.com
M Hönig, K Schwarz
Current opinion in rheumatology, 2006journals.lww.com
Research has begun to clarify the genetic defects and the conditions underlying the lack of
tolerance enforcement that predispose to Omenn syndrome. Clinical applications of this
research include the identification of the causative genetic defect in the majority of Omenn
syndrome cases and the use of this genetic knowledge in family and prenatal analyses and
in difficult differential autoimmune diagnoses.
Summary
Research has begun to clarify the genetic defects and the conditions underlying the lack of tolerance enforcement that predispose to Omenn syndrome. Clinical applications of this research include the identification of the causative genetic defect in the majority of Omenn syndrome cases and the use of this genetic knowledge in family and prenatal analyses and in difficult differential autoimmune diagnoses.
Lippincott Williams & Wilkins