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

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Functional Assessment


Functional assessment:

Adaptive immunity:


Clinical manifestations


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


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)

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