TY  - JOUR
AU  - Thalhammer, Julian
AU  - Kindle, Gerhard
AU  - Nieters, Alexandra
AU  - Rusch, Stephan
AU  - Seppänen, Mikko R J
AU  - Fischer, Alain
AU  - Grimbacher, Bodo
AU  - Edgar, David
AU  - Buckland, Matthew
AU  - Mahlaoui, Nizar
AU  - Ehl, Stephan
AU  - European Society for Immunodeficiencies Registry Working Party
PY  - 2021
DO  - 10.1016/j.jaci.2021.04.015
UR  - https://hdl.handle.net/10668/24545
T2  - The Journal of allergy and clinical immunology
AB  - Inborn errors of immunity (IEI) are rare diseases, which makes diagnosis a challenge. A better description of the initial presenting manifestations should improve awareness and avoid diagnostic delay. Although increased infection susceptibility is a...
LA  - en
KW  - Primary immunodeficiency
KW  - autoimmune
KW  - immune dysregulation
KW  - inborn error of immunity
KW  - inflammatory
KW  - presenting symptom
KW  - registry
KW  - syndromic
KW  - warning signs
KW  - Adult
KW  - Age Factors
KW  - Autoimmune Diseases
KW  - Child
KW  - Cohort Studies
KW  - Female
KW  - Granuloma
KW  - Humans
KW  - Infections
KW  - Lymphoproliferative Disorders
KW  - Male
KW  - Middle Aged
KW  - Primary Immunodeficiency Diseases
KW  - Registries
KW  - Sex Factors
TI  - Initial presenting manifestations in 16,486 patients with inborn errors of immunity include infections and noninfectious manifestations.
TY  - research article
VL  - 148
ER  -