Allergy and Immune Deficiency

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Immunology Fichas sobre Allergy and Immune Deficiency , creado por Nneka Okorocha el 06/11/2016.
Nneka Okorocha
Fichas por Nneka Okorocha, actualizado hace más de 1 año
Nneka Okorocha
Creado por Nneka Okorocha hace alrededor de 8 años
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Allergy Hx 1. How long 2. How much before you react 3. How long for symptoms to come 4. Associations: swelling, SOB? 5. Symptom severity 6. Happened before + what was the reaction then 7. Food? Cooked or raw?
Order of procedure when Ptn presents w/ allergy 1. Hx 2. Skin prick test 3. Blood total IgE, specific IgE or tryptase
Interpretation of Total IgE & Specific IgE Total - indicates allergy/atopy (non-immediate allergy), may be high if 'threat' is constant i.e. old/dust Specific - rises in presence of a specific allergy may increase total IgE No IgE doesn't mean no allergy
Why might ptns. with chronic urticaria not respond to allergy test? ~30% due to autoimmune disease
Interpretation of Serum Tryptase >11.4 shows mast cell activity High after anaphylaxis (15mins to 3hrs) Test again 24-48hrs PERSISTENT = MASTOCYTOSIS
What are C1 [esterase] inhibitors Ensure inhibition of complement proteins Stop overreaction Deficiency may lead to hypersensitivity Causes intermittent angioedema Ddx anaphylaxis/allergy BUT this is intermittent, usually adolescents
Screening tests in C1 esterase inhibitor deficiency Normal C1-INH levels are 16-33mg/dL Serum C1 and C4 levels done Abnormal can be due to: 1. Inherited angioedmea 2. SLE 3. Malnutrition 4. Septicaemia (>in infection) 5. Lupus nephritis (kidney disease)
What organisms are clues for: T cell Deficiency and Infection Viruses: CMV, VAZ, HSV, EBV, measles, resp viruses Bacteria (cocci, staph. aureus, pseudomonas): Mycobacteria, salmonella, Listeria Fungi: Candida, aspergillus, histoplasma, PCP, crypto Protozoa (giardia lamlia): toxoplasma Clinical (resp and GI sepsis): Systemic viral fungal infections, early malignancy
What organisms are clues for: B & T Cell Deficiency Same as for T-cell! But here bacteria can also cause S. typhi
What organisms act as clue for: Phagocyte Deficiency Bacteria: S.aureus and mycobacteria infections Fungi: Candida and aspergillus Clinical (sepsis and resp): lymphadenitis, skin/mouth infections, liver and lung abscesses, granulomas
Which organisms are clues for : Complement Deficiency Bacteria: meningococcus Fungi: Candida Clinical: Systemic bacterial infections + autoimmune disease
Screening for Common Variable Immune Deficiency (CVID) Presents with recurrent infection (rest, conjunctivitis, GI infection) General finding is: HYPOGAMMAGLOBULINAEMIA IgG of <4.5 IgA or IgM below the normal limit Ptn. will also lack immune response to protein antigens or immunisation Usually above 4yrs old Usually associated with: Autoimmune diseases Malignancies Granulomatous disease Dermatological manifestations
Diagnosis of Immune Deficiency 2 major infections or 1 major + 1 recurrent minor Unusual sites/organisms Unresponsive to Abx! Skin disease Chronic diarrhoea Mouth ulcers Family Hx
Pathophysiology of C1-INH deficiency SERPIN inhibits 1. C1 2. F12 of coal. cascade 3. Kallikrein (incr. bradykinin by cleaving HMWK) SERPIN deficiency means C1 & Kallikrein are increased (Incr. bradykinin = vasodilation, muscle contraction, natriuresis, all lowering BP)
How does C1-INH present? Can be acquired by splenic lymphoma ANGIO OEDEMA (skin, oropharynx, GIT), NO RASH Asphyxia, N+V and diarrhoea Low C2, C4 (normal C1 and C3)
Investigation of: Complement Dysfunction CH50 and AP50 C3 and C4 If CH50 and AP50 abnormal and C3 and C4 normal then final/terminal common pathway deficiency
Complement Dysfunction: Classical Pathway C1, C2 (commonest), C4 Outcome: SLE!!
Complement Dysfunction: Mannose Binding Lectin MBL deficiency Outcome: increased infection in immunocompromised i.e. HIV, Ab def., chemo
Complement Dysfunction: Alternative Pathway B, I , P deficiency Outcome: Infection!! Especially with meningococcus, pneumococcus, H.influenzae, Hib FX of infection
Complement Dysfunction: Terminal/final pathway C5-9 deficiency Inability to make MAC Outcome: Infection!! Especially with meningococcus, pneumococcus, H.influenzae, Hib FX of infection
Autoantibodies ANA: SLE Anti-CCP: Rheumatoid arthritis Anti-mitochondrial Ab: Primary biliary cirrhosis Anti-phospholipid Ab: Antiphospholipid syndrome Anti-transglutaminase: Coeliac, dermititis herpetiformis
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