Decreased T cell immunity is a feature of -
Digeorge syndrome
Hyper lgM syndrome
Severe congenital neutropenia
Chronic granulomatous diseas
Ans. is 'a' i.e., Di George syndrome Classification of Primary Immune Deficiency DiseasesDeficiencies of the Innate Immune Systemo Phagocytic cells# Impaired production severe congenital neutropenia (SCN)# Asplenia# Impaired adhesion leukocyte adhesion deficiency (LAD)# Impaired killing; chronic granulomatous disease (CGD)o Innate immunity receptors and signal transduction# Defects in Toll-like receptor signaling# Mendelian susceptibility to mycobacterial diseaseo Complement deficiencies# Classical, alternative, and lectin pathways# Lytic phase Deficiencies of the Adaptive Immune Systemo T lymphocyteso impaired developmento Impaired survival, migration, functionSevere combined immunedeficieneies (SOD) DiGeorge syndrome.Severe combined immunodeficiencies.Hyper-lgE syndrome (autosomal dominant).CD401 igand deficiency.Wiskott-Aldrich syndrome.Ataxia-telangiectasia and other DNA repair deficiencies.o B lymphocytesy Impaired developmenty Impaired function XL and AR agammaglobulinemiaHyper-IgM syndromeCommon variable immunodeficiencies (CVID)IgA deficiencyRegulatory Defectso Innate immunityAutoinflammatory syndrome (outside the scope of this chapter) Severe colitiso Adaptive immunityHemophagoevtic lymphohistiocytosis (HLH)Autoimmune lymphoproliferation syndrome (ALPS)Autoimmunity and inflammatory diseases (IPEX, APECED)Abbreviations: A PEC ED. autoimmune polyendocrinopathv candidiasis ectodermal dysplasia; AR, autosomal recessive,1PEX, immunodysregulation polyendocrinopathy enteropathy X-linked syndrome, XL, X-linked.
Autoimmune Diseases