Pediatric Immunodeficiency

Add by hongdau | Jul 08, 2017 05:49  1627 |  351
Pediatric Immunodeficiency
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Immunodeficiency
1 Adaptive
1.1 Humoral
1.1.1 B-cells (Ig, CD40, 50)
1.1.1.1 X-linked Agammaglobunemia (Bruton's)
1.1.1.1.1 Pt: Boys, B-cells @6mo Sinopulmonary infxns
1.1.1.2 Common Variable Immunodeficiency (CVID)
1.1.1.2.1 mild XLA
1.1.1.2.1.1 presents during teenage years
1.1.1.3 IgA Deficiency
1.1.1.3.1 poor mucosal defenses
1.1.1.3.1.1 Sinopulmonary infxns Bouts of GI bugs
1.1.1.3.1.1.1 ASx - until analphylaxis after blood transfusion
1.1.1.4 Hyper IgM
1.1.1.4.1 cannot convert IgM to IgG
1.1.1.4.1.1 non-specific immunodef
1.2 Combination
1.2.1 Wiskott-Aldrich
1.2.1.1 X-linked: boys
1.2.1.1.1 atopic dermatitis low platelets normal infxns
1.2.2 Ataxia Telangiectasia
1.2.2.1 immunodef
1.2.2.1.1 DNA repair mech leukemia lymphoma
1.2.3 SCID
1.2.3.1 ADA def
1.2.3.1.1 no B's, no T's
1.2.3.1.1.1 immediate immunodef
1.3 Cellular
1.3.1 T-cells (CD4, CD8)
1.3.1.1 DiGeorge
1.3.1.1.1 22q11.2 del 3rd pharyngeal pouch
1.3.1.1.1.1 CATCH-22 wide-spaced eyes low set ears small face
1.3.1.1.1.1.1 absent thymic shadow
1.3.1.1.1.1.1.1 infxns w/ Fungi + PCP
1.3.1.2 Acquired HIV
2 Innate
2.1 Phagocytosis
2.1.1 Macrophages
2.1.1.1 Chronic Granulomatous Disease
2.1.1.1.1 no respiratory burst but can phagocytize
2.1.1.1.1.1 susceptible to catalase-positive orgs
2.1.1.1.1.1.1 Staph abscesses
2.1.1.2 Leukocyte Adhesion Deficiency
2.1.1.2.1 WBCs can't leave the bloodstream
2.1.1.2.1.1 delayed separation of the cord
2.1.1.2.1.1.1 incr fever incr WBC no pus
2.1.1.3 Chediak-Higashi
2.1.1.3.1 autosomal recessive
2.1.1.3.1.1 giant granules in neutrophils
2.1.1.3.1.1.1 assoc w/ albinism neuropathy neutropenia
2.1.2 NK
2.2 Complement
2.2.1 C1-Esterase
2.2.1.1 spontaneous angioedema
2.2.2 C5-C9 Mac Attack
2.2.2.1 if infected with Neisseria

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