Guidelines to Detect Congenital Immunodeficiency

ISSN 0973 - 0958

Hematological Disorders

Congenitial Immunodeficiency Detection

Hematological Disorders in Children

Anemia

Approach To A Bleeding Child

Hemophilia

Congenitial Immunodeficiency Detecting Guidelines

Red Cell Substitutes

Idiopathic Thrombocytopenic purpura in children

Hemoglobin Variants Detection by High Performance Liquid Chromatography Method

Platelet Transfusion Facts

Hematological manifestations of systemic illness

Thalassemia

Administration of Blood

Aplastic Anemia

Clinical Tools

Clinical Vignettes

Diseases & Conditions

Drugs Interaction Calculator

Podcasts

Patient Management Online

Diagnostic Dilemma

Question of the Day

Diagnostic Aid

VACCINE REMINDER

"Get the Vaccine Schedule for your child."

Ask Queries

SPECIALIST ANSWERS

Today's Poll

Should all patients with viral respiratory infection be treated with oseltamivir in current epidemic of H1N1 influenza_?

Yes, it may be H1N1

No, only if test is positive for influenza

Only in sick patients admitted in ICU

Vote

Results

GUIDELINES TO DETECT CONGENITAL IMMUNODEFIENCY

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)
Properidin deficiency; Dyskeratosis Congenita.

Etiology of Infections

E.g. 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.)

Mail To Your Friend

Print This Article

Diseases & Conditions

Pediatric Oncall Journal

Pediatric Conferences

Ped Tools

Adverse Drug Reactions

Pediatric oncall book store

Partner Sites

Infection in Children

Pedcall

Medical ADRIS

Vaccine Reminder

Pediatric Oncall Journal

Educational Section

Nutrition

Skin Problems

Swine Flu Center

Health Solutions from our sponsors

DHA

Baby Massage

Disclaimer:

The information given by www.pediatriconcall.com is provided by medical and paramedical & Health providers voluntarily for display & is meant only for informational purpose. The site does not guarantee the accuracy or authenticity of the information. Use of any information is solely at the user's own risk. The appearance of advertisement or product information in the various section in the website does not constitute an endorsement or approval by Pediatric Oncall of the quality or value of the said product or of claims made by its manufacturer.

Sitemap For Doctor

Sitemap For Parent

Sitemap For Kids