Approach to primary immunodeficiency diseases in children

1. APPROACH TO PRIMARY
IMMUNODEFICIENCY
DISEASES
PRESENTER – DR. IQRA KHAN
MODERATOR - DR. (PROF) A. P. DUBEY SIR

4. DEFINITION
 PRIMARY IMMUNODEFICIENCY DISORDER – Heterogenous group of
disorders characterised by poor or absent function in one or more
components of the immune system which predisposes affected
individuals to increased frequency and severity of infection,
autoimmunity and aberrant inflammation and malignancy *
 Overall prevalence of PID of 1:1200 live births *
*Nelson textbook of paediatrics 21 edition

5. CLASSIFICATION OF PRIMARY ID
ANTIBODY DEFICIENCY (B CELL
DEFECT)
CELL MEDIATED (T CELL DEFECTS)
COMBINED B AND T CELL DEFECTS
PHAGOCYTIC DEFECTS
DISORDER OF COMPLEMENT SYSTEM

10. WHEN TO SUSPECT
IMMUNODEFICIECY DISORDERS?

12. THE JEFFREY MODEL FOUNDATION 10
WARNING SIGNS

13. CLINICAL MANIFESTATIONS -
 MOST COMMON MANIFESTATION ARE RECURRENT
SINOPULMONARY INFECTIONS
- Usually present
1. Recurrent upper respiratory infections
2. Severe bacterial infections
3. Persistent infections with incomplete or no
response to therapy
1. Paucity of lymph nodes and tonsils
- Often present
1. Persistent sinusitis or mastoiditis
(Streptococcus pneumoniae, Haemophilus,
Pneumocystis jiroveci, Staphylococcus
aureus, Pseudomonasspp.)
2. Recurrent bronchitis or pneumonia
3. Failure to thrive or growth retardation for
infants or children
4. Intermittent fever
5. Infection with unusual organisms
6. Skin lesions: rash, seborrhea, pyoderma,
necrotic abscesses, alopecia, eczema,
telangiectasia

16. EXAMINATION FINDINGS -:

23. HOW TO SCREEN?

30. PREVENTIVE MEASURES
 LIVE ATTENUATED VACCINES SHOULD NOT BE GIVEN
 CAREFUL TB SCREENING
 PROMPT IDENTIFICATION AND TREATMENT OF INFECTIONS
 ATYPICAL OPPORTUNISTIC INFECTIONS SHOULD ALSO BE
SUSPECTED
 IN CHRONIC RESPIRATORY DISEASE ,VIGOROUS ATTENTION
TO POSTURAL DRAINAGE
 PROPHYLACTIC ADMINISTRATION OF ANTIBIOTICS IN
SELECTED CASES LIKE RECURRENT OR CHRONIC
PULMONARY OR MIDDLE EAR INFECTIONS