Pediatric Immunodeficiency

Add by hongdau | Jul 08, 2017 05:49  2155 |  477
Pediatric Immunodeficiency

Map Outline

1 Adaptive
1.1 Humoral
1.1.1 B-cells (Ig, CD40, 50) X-linked Agammaglobunemia (Bruton's) Pt: Boys, B-cells @6mo Sinopulmonary infxns Common Variable Immunodeficiency (CVID) mild XLA presents during teenage years IgA Deficiency poor mucosal defenses Sinopulmonary infxns Bouts of GI bugs ASx - until analphylaxis after blood transfusion Hyper IgM cannot convert IgM to IgG non-specific immunodef
1.2 Combination
1.2.1 Wiskott-Aldrich X-linked: boys atopic dermatitis low platelets normal infxns
1.2.2 Ataxia Telangiectasia immunodef DNA repair mech leukemia lymphoma
1.2.3 SCID ADA def no B's, no T's immediate immunodef
1.3 Cellular
1.3.1 T-cells (CD4, CD8) DiGeorge 22q11.2 del 3rd pharyngeal pouch CATCH-22 wide-spaced eyes low set ears small face absent thymic shadow infxns w/ Fungi + PCP Acquired HIV
2 Innate
2.1 Phagocytosis
2.1.1 Macrophages Chronic Granulomatous Disease no respiratory burst but can phagocytize susceptible to catalase-positive orgs Staph abscesses Leukocyte Adhesion Deficiency WBCs can't leave the bloodstream delayed separation of the cord incr fever incr WBC no pus Chediak-Higashi autosomal recessive giant granules in neutrophils assoc w/ albinism neuropathy neutropenia
2.1.2 NK
2.2 Complement
2.2.1 C1-Esterase spontaneous angioedema
2.2.2 C5-C9 Mac Attack if infected with Neisseria

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