T Cell Impairment Is Predictive for a Severe Clinical Course in NEMO Deficiency.

Stephanie HellerUwe KölschThomas MaggRenate KrügerAndrea ScheuernHolm SchneiderAnna EichingerVolker WahnNadine UnterwalderMyriam LorenzKlaus SchwarzChristian MeiselAnsgar SchulzFabian HauckHorst Von Bernuth

Published in: Journal of clinical immunology (2020)

The loss of the ZF-domain and the impaired T cell proliferation accompanied by almost complete persistence of naïve T cells despite severe infections are suggestive for a profound immunodeficiency. Allogenic HSCT should be considered early for these patients before chronic sequelae occur.

Keyphrases

cell proliferation
end stage renal disease
ejection fraction
chronic kidney disease
newly diagnosed
early onset
peritoneal dialysis
drug induced
intellectual disability
autism spectrum disorder