Chuang Gung Medical Foundation, Division of Pediatric Allergy, Asthma and Rheumatology - UNREGISTERED VERSION

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Functional Assessment

PICAR

Functional assessment:
Adaptive immunity:
	Clinical manifestations	Tests
B-cell defects	Usually onset age around 5-6 months; Recurrent bacterial sino-pulmonary infections (recurrent otitis media, sinusitis and pneumonia), encapsuled sepsis, or sepsis, particularly with encapsulated organisms; Chronic or recurrent gastroenteritis (Giardia and Enterovirus common); Chronic enteroviral menigo-encephalitis; Arthritis; Unexplained bronchiectasis	CBC/Differential , Quantitative IgM, IgG, IgA, & IgE; IgG subclass (L72-206, 208, 210,212) Anti-tetanus titers Anti-pneumococcal titers pre- & 4 wks post immunization Anti-A 7 anti-B titer (L72-855); immunfixation electrophoresis (L72-204)
T-cell defects	Usually onset age around below 2 months; Pneumocystis jroveci (carinii) pneumonia; Fungal infections; GVHD (rash, abnormal liver function tests and chronic diarrhea); Recurrent, severe, or unusual viral infections; Failure to thrive.	CBC/Differential, T & B cell subset analysis (L72-266; 267; 272), DTH reaction to Candida & BCG (M32-266), Cytotoxicity (M32-262)

Innate immunity:
	Clinical manifestations	Tests
PMN defects	Usually onset age in neonatal stage; Soft tissue abscesses or lymphadenitis; Infection with catalase +organisms (Staph aureus, Serratia, E. coli, Aspergillus); Poor wound healing; Delayed separation of the umbilical cord; Chronic gingivitis and periodontal disease; Mucosal ulcerations.	CBC/Differential, Neutrophil oxidative burst assay H2O2 production (M32-253), CD11b/CD18 (M32-264), Phagocytosis (M32-263), Chemotaxis (M32-265)
NK defects	Usually combine with other PIDs; Recurrent herpesvirus infection (including EBV, CMV, varicella, herpes simplex virus); Hemophagocytic syndrome.	Cytotoxicity (M32-262)
Complement defects	Recurrent disseminated Neisserial infections; Pyogenic bacterial infections; Angioedema of face, hands, feet, or GI tract; Autoimmune symptoms (Lupus); History suggestive of autosomal dominant inheritance.	CH50, AH50, MBL –tests, complement cascade function first CH50- (L72-220)