approach to a case of immunodeficiency in children
DESCRIPTION
Immunodeficiency disorders, Cell mediated immunity, Humoral immunityTRANSCRIPT
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Dr Veena Singh
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Immunoglobulin levels vs. age
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Innate and adaptive immunity
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Immunologic defects(1) T cell
(2) B cell
(3) Macrophage
(4) Complement
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Primary immunodeficiencyInherited genetic defects in the immune cell
development or function or inherited deficiency in a particular immune molecule
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Secondary immunodeficiencyLoss of previously functional immunity due to
infection, toxicity, radiation, splenectomy and malnutrition
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When to suspect immunodeficiency ?
Very frequent infectionsVery severe infectionsFamily historyUnusual clinical presentation Unusual microorganism at unusual site
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Additional clues..>8 ear infections per year>2 serious sinus infections per year>2 months treatment with poor result Requirement of iv antibiotics Failure to thrive with or without diarrhea
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Common clinical featuresRecurrent respiratory tract infectionsSevere bacterial infectionPersistent infection with incomplete responsePersistent sinusitis/mastoiditisFTT/Growth retardationDiarrhea/malabsorption
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Occasionally presentLymphadenopathyHepatosplenomegalyRecurrent meningitisPyodermaDeep infections: osteomyelitis, cellulitis
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Detailed historyUpto 6 monthsHistory of newborn seizures, cardiac anomalyDelayed cord detachment, recurrent infectionDiarrhea, pneumonia, oral thrush, FTTBloody stools, draining ears, eczema
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6 months to 5 yearsPersistent thrush, nail dystrophy,
endocrinopathiesShort stature, fine hair, severe varicellaOculocutaneous albinism with recurrent
infectionLymphadenopathy, dermatitis, pneumonia
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Examination Clinically-BCG scar absentwasted, stunted, malnourishedSkin eczema present, hypopigmented patches Oral thrush, nail fungal infectionAbsence of tonsils and lymph nodesRespiratory system-b/l ronchi , wheeze,
crepts
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Initial immunologic testing of the child with recurrent infections Complete blood count,differential,ESRLymphocyte,neutrophils,platelets,Howell jolly bodies,ESR Screening tests for B-cell cefectsIgA, if abnormal IgG, IgM measurementIsohemagglutininsAntibody titres to tetanus, diphtheria,
H.influenzae
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Cont…. Screening test for Tcell defectAbsolute lymphocyte countCandida albicans intradermal test Screening test for phagocytic cell defectAbsolute neutrophil countRespiratory burst assay Screening test for complement
deficiencyCH50
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Laboratory tests to assess immune function(1) T cell: Enumeration (flow cytometry),
functional assays (mitogen response, DTH skin tests)
(2) B cell: Enumeration, circulating antibody levels
(3) Macrophage: Enumeration, functional assays (nitroblue tetrazolium)
(4) Complement: Direct measurement of complement components, complement hemolysis assay
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THANK YOU...