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28 Cards in this Set
- Front
- Back
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No AB fxn, no lymph nodes, no thymus
-1 st few mo life; FTT, rash, infx, diarrhea, PCP PNA, candida infx -How dx/ tx? |
SCID –X linked/ Adenosine Deaminase
-DX: Low TRECS, decr Lymphs, decrs, QIGS, low T cell; AD: no ADA Tx: BMT/ SCT, IVIG |
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which immuno def need BMT?
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CLWSK: Cheidich, LAD, Wiscott, SCID, Kostman
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Which immuno need IVIG?
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BICHA: Bruton’s, ITP, CVID, Hyper Ig M; AT
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? immuno def needs IFN gamma: ?
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CGD
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Ataxia, telangectasi;
-sinopulm infx; cancer; swallowing probs -? cause; dx, tx? |
Ataxia, telangectasi; -No ATM gene
DX: Hi AFP; DNA breakage repair deficiency ; -Ig A deficiency; T cell defects; chromosom instability Tx: IVIG |
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Purpura (low plt)
Infx;- severe; PCP Eczema, draining ears cz, dx, tx? |
Wiscott Aldrich: PIE/TIE: (thrombocytopenia, infx, ezcema)
-Low plt, small plt Low Ig M; hi A/E; T cell fx low -; no WASP gene tx: BMT |
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? genetics of Wiscott aldrich?
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AR, X-linked
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what are combo T/B cell deficiency?
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SAW: SCIDs, Ataxia telangectesia; Wiscott aldrich
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What are AB defeficeny d/o?
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XIPICTH: X- linked agammagloblemia;
Ig A defec Partial Ab deficiency; Ig G subclass deficiency; CVID; Transient Hypogammaglob of infancy; Hyper Ig M; |
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No tonsils, no adenoids; CLD, enteroviral encephalitis; > 6 mo age;
dx, cz, tx? |
Brutons; boys: X linked agammaglobulemia; No B cells, + T cells,; No IG M/A; No AB;
- tx: IVIG - no live vaccines |
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genetics of brutons?
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x-linked
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Resp infx, allergies, OM, AI; sinopulm
-anaphylaxis to blood or IG; -what meds ~ ? |
IG A deficiency – most common immunodef; No Ig A; ~ Ig G def ~anticonvulsants
-dx: No Ig A; < 10 - symptomatic -tx: Avoid Ig; ? tx antbx |
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Sinusitis, PNA; URI, diarrhea; -bronchiectasis; > 10 yo;
-cz, dx, ? |
CVID: common variable immunodef: B cell defect/variable T cell defect;
-DX: Low Ig G, A, M; decreased Ab response to protein polysacc vaccines |
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tx of CVID?
-~ with what? |
IVIG;
~ malignancy |
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Recurrent bacterial infx @ 6-24 mo; Diarrhea, FTT; esp premies; food intol, excema;
-cz, dx? tx? |
Transient Hypogammoglob of infancy
-Lag in Ig synthesis, not physiologic -Low IGs, ab variable, B cells present; occasional neutropenia tx: Watch; cont antbx |
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no switching from Ig M→A
-cz, dx? tx? |
-Hyper Ig M:
-dx: Low Ig A/G; T cell defect of CD 40 ligand tx: IVIG |
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Recurrent URI, PNA, sinsuistis NL total IgG; low G1, 2, or 3
-cz, dx? tx? |
Ig G subclass defic
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Dx > 2 yo; Ig G 4 def PNA, recurrent resp, sinusitis NL Ig G and subclass; poor AB response to pneumovax
-? dx; TX |
Partial Ab def
(Impaired polysaccarhide responseiveness -tx: Prophylactic antbx |
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-Delayed umbil cord separation > 20 days
-abscess w/o pus; -soft tissue infx, dermatitis; Hi Neutrophil count; just don’t fxn/move -cz, geneticx, tx? |
LAD (leukocyte Adhesion Def); AR;
-no adhesion; neg CD 18/11 b; -tx: BMT/SCT; antbx; GMCSF |
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list all phagocytic d/o?
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LIC: LAD; Ig E - Hyper; CGD
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Cold abscess, coarse faces; staph/skin infx;
Double teeth rows; staph infx; scoliosis; -cz, dx, tx? |
-hyper Ig E; I g E > 2000 Antbx – life
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-Skin, organ abscesses w/ S. aureus, PSA, serratia, asperg; fungal, HPSM, poor wound healing; GI obstruction- granuloma;
cz, dx?- |
CGD: chronic granulomatous:
WBC there, but can’t kiill - Neutrophil oxidative metab; - --No NADPH oxidase; X linked and AR -dx: NBT (dihydrorhodamine essay ) oxidative burst test; hi IG; nL T cell fxn |
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tx of CGD?
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IFN Gamma***BOARDS!!
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Cardiac abnl: outflow defet
Abnl facies Thymic hypoplasia Cleft palate Hypocalcemia 22 –chom -microcephaly |
diGeorge; dx: No thymic shadow; lo calcium; low T cells and TRECS; FISH
-Tx: Ca, Vit D; BMT or thymic transplant; |
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Candidia – mouth, hair, skin
~AI thyroid diz |
Mucutaneous Candidiasis Pure T cell defect;
tx: antifungals |
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-~ Rheumtic diz/SLE; what compleement def is most common; how dx? what's cause?
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Complement deficiency: ** C2 – most common;
-check CH50 |
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what complements ~Diarrhea, mengitis; is AR?
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C 6-9
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? compleemnt def ~ menigitis, sepsis? how dx?
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C 5-8, check ch50
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