Congenital Immunodeficiency Detection Guidelines

4th Pediatric Infectious Diseases Conference

ISSN 0973 - 0958

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GUIDELINES TO DETECT CONGENITAL IMMUNODEFICIENCY

Dr. Zeenat Currimbhoy,
Consultant Hematologist and Immunologist
B.J. Wadia Childrens Hospital
Prince Aly Khan Hospital

Consequences of absence of normal immunity:

Risk of infections, recurrent and severe, in a young child.
Autoimmunity, because the host cannot completely get rid of microbial pathogens and their antigens which persist.
Malignancies, especially lymphomas, others being leukemias and gastric carcinomas, are several fold more frequent in immunodeficient (ID) children.

Components of the defense system
- Phagocytic system (Neutrophils, Monocytes-Macrophages), accounts for 18% of ID cases.
- Complement system, for 2%, and
- The Immune System, for 80%, excluding solely IgA deficiency. (B-lymphocyte defects for 50%, T-lymphocyte defects for 10%, and Combined B- and T- cell defects for 20%.)
Common variable Immunodeficiency (CVID) and Selective IgA deficiency are the commonest IDs and autoimmune problems appear frequently among them.

Suspicion of IDs arises if:

Repeated and/or persistent severe infections
Failure to thrive (FTT), specially in T-cell defects
Chronic persistent diarrhea (T-cell, also B-cell defects) or chronic persistent respiratory tract infections (B-cell defects)
Skin changes e.g. eczema, extensive monilial rash

Age of onset:

Birth to six months: Recurrent infections + FTT; T-cell or T-cell associated defects
Over six months: due to B-cell defects/Antibody (Ab) deficiencies

Sex and family history of affected males:

In ID, X-linked gene abnormalities predominate:

X-linked agammaglobulinemia (XLA)
X-linked Hyper-IgM Syndrome
Severe combined immunodeficiency (X-SCID)
Wiskott-Aldrich syndrome(WAS)
X-linked lymphoproliferative syndrome (XLPS)
Chronic Granulomatous Disease (CGD)
Properdin deficiency; Dyskeratosis Congenita

Etiology of Infections:

Eg. Pulmonary Infections:

B-cell defects: Pneumococci, Hemophilus influenzae
T-cell defects: Mycobacteria, Pneumocystis carinii, viruses + FTT
Phagocytes: Staph. Aureus, Fungus (Aspergillosis), B. Cepacia.

Next : Congenitial Immunodeficiency Detecting Guidelines(Contd.)

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