IMMUNE-DEFICIENCY

Immune deficiencies
Occur when one or more components of the immune system are defective. Immune defects can be inherited or acquired. The immune defects can involve the T cell, the B cell or both compartments of the immune system, the Complement or the APC.

Lymphocyte

Adaptive immunity

Cells Of The Immune System

Common lymphoid progenitor

Macrophage Phagocytosis Monocyte Ag presentation Neutrophil PMN

Phagocytic Anti-bacterial

Eosinophil Anti-parasite
immunity

Pluripotent haemopoietic stem cell

Basophil

?Protection of mucosal surfaces?

Myeloid progenitor

Mast cell

Protection of mucosal surfaces

Inherited (Genetic) Immune defects

Genetic defects can occur in almost any molecule involved in the immune response. T cell defects DiGeorge syndrome: Failure of the thymus to be formed (thymic aplasia). Patients suffer from general susceptibility to infections since the absence of T lymphocytes impacts also on the ability of B cells to synthesize antibodies

Inherited B cell defects

X-linked agammaglobulinemia: X-chromosome-linked inability to produce B lymphocytes, leads to absence of Ig. Patients suffer mainly from infectious diseases with bacteria and viruses that require antibodies for their disposal

Mixed Immunodeficiencies
Inherited mixed T and B cell defects: Severe combined immune deficiencies (SCID). Patients suffer from total susceptibility to infections since no T and no B cells are generated. Inherited defects of phagocytic function. Inherited defects of complement: Loss of specific complement components.

HIV/AIDS

Description
HIV depends on the human body to replicate and survive. HIV enters lymphocytes, replicates within them and this leads to progressive destruction of both the cellular and humoral immune systems until the infected person becomes unable to fight infection and develops the syndrome of Acquired Immune Deficiency Syndrome (AIDS).

Acquired immune deficiency syndrome (AIDS).

The immune response becomes defective as a result of exposure to the Human Immunodeficiency Virus (HIV). The HIV infects selectively CD4+ T cells and macrophages. The disease is usually lethal due to loss of CD4+ T cells.

Infection with the human immunodeficiency virus (HIV)

Features of seroconversion: a glandular fever type illness with maculopapular rash and small orogenital ulcers. Features of early disease: generalised swollen glands skin rashes shingles

Infection with the human immunodeficiency virus (HIV)

later clinical features: weight loss intermittent fever malaise fatigue chronic diarrhoea anaemia recurrent infections TB oral candidiasis

Diagnosis:
Adequate pre-test and post-test counselling must occur. there is a "window period" of up to 3 months,? which is the time period between becoming infected and the appearance of antibodies which are detectable by blood tests. ensure that the diagnosis is recorded in such a manner that the patient's confidentiality is not breached

WHO clinical stage 3 or 4

CD4 count <200 106/l Total lymphocyte count <1.25 109/l (use if CD4 count unavailable)

Human Immunedeficiency Virus Infection in Children

Human immune deficiency virus (HIV) infection and the resulting acquired immune deficiency disease syndrome (AIDS) in children is the leading cause of suffering and death in South African children after the 1st month of life.

Diagnosis of HIV infection :

Antibody tests e.g. HIV antibody rapid test, HIV ELISA test, Western Blot test,
These tests determine the response of the body to the virus and are usually used to diagnose infection.

Antigen tests e.g. HIV DNA or RNA PCR and p24 antigen;
these tests look for parts of the HI virus but are technically difficult to perform and not routinely available in public health facilities

A positive antibody test:

In a child less than 15 months of age, may reflect maternal antibody rather than infection in the child. If a second antibody test is positive and the child has features of symptomatic HIV infection (AIDS), it is very likely that the child is HIV infected. If the child has no features of symptomatic HIV infection, the child may still be infected with HIV, but may be asymptomatic. The test should be repeated when the child is 15-18 months old In a child is over the age of 15 months, requires a second HIV antibody test to be performed. If positive the child is infected with HIV

A negative antibody test

Means that the child is not HIV infected, provided that the child has not received breast milk in the previous 6 months. If the child has received any breast milk in the previous 6 months, the test has to be repeated 6 months after stopping breastfeeding to confirm that the child is truly HIV negative

Risk of Infections