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T-B- тяжкий комбінований імунодефіцит

ORPHA:317419· ICD-10 D81.1· T-B- severe combined immunodeficiency

Визначення(English summary)

T-B- severe combined immunodeficiency (SCID; see this term) is a group of rare monogenic primary immunodeficiency disorders characterized by a lack of functional peripheral T and B lymphocytes, resulting in recurrent early-onset severe respiratory viral, bacterial or fungal infections, diarrhea and failure to thrive. Hypersensitivity to ionizing radiation is a characteristic feature of some of its sub-types.