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By the end of this section, you will be able to:
  • Discuss inherited and acquired immunodeficiencies
  • Explain the four types of hypersensitivity and how they differ
  • Give an example of how autoimmune disease breaks tolerance

This section is about how the immune system goes wrong. When it goes haywire, and becomes too weak or too strong, it leads to a state of disease. The factors that maintain immunological homeostasis are complex and incompletely understood.

Immunodeficiencies

As you have seen, the immune system is quite complex. It has many pathways using many cell types and signals. Because it is so complex, there are many ways for it to go wrong. Inherited immunodeficiencies arise from gene mutations that affect specific components of the immune response. There are also acquired immunodeficiencies with potentially devastating effects on the immune system, such as HIV.

Inherited immunodeficiencies

A list of all inherited immunodeficiencies is well beyond the scope of this book. The list is almost as long as the list of cells, proteins, and signaling molecules of the immune system itself. Some deficiencies, such as those for complement, cause only a higher susceptibility to some Gram-negative bacteria. Others are more severe in their consequences. Certainly, the most serious of the inherited immunodeficiencies is severe combined immunodeficiency disease (SCID)    . This disease is complex because it is caused by many different genetic defects. What groups them together is the fact that both the B cell and T cell arms of the adaptive immune response are affected.

Children with this disease usually die of opportunistic infections within their first year of life unless they receive a bone marrow transplant. Such a procedure had not yet been perfected for David Vetter, the “boy in the bubble,” who was treated for SCID by having to live almost his entire life in a sterile plastic cocoon for the 12 years before his death from infection in 1984. One of the features that make bone marrow transplants work as well as they do is the proliferative capability of hematopoietic stem cells of the bone marrow. Only a small amount of bone marrow from a healthy donor is given intravenously to the recipient. It finds its own way to the bone where it populates it, eventually reconstituting the patient’s immune system, which is usually destroyed beforehand by treatment with radiation or chemotherapeutic drugs.

New treatments for SCID using gene therapy, inserting nondefective genes into cells taken from the patient and giving them back, have the advantage of not needing the tissue match required for standard transplants. Although not a standard treatment, this approach holds promise, especially for those in whom standard bone marrow transplantation has failed.

Human immunodeficiency virus/aids

Although many viruses cause suppression of the immune system, only one wipes it out completely, and that is the previously mentioned HIV. It is worth discussing the biology of this virus, which can lead to the well-known AIDS, so that its full effects on the immune system can be understood. The virus is transmitted through semen, vaginal fluids, and blood, and can be caught by risky sexual behaviors and the sharing of needles by intravenous drug users. There are sometimes, but not always, flu-like symptoms in the first 1 to 2 weeks after infection. This is later followed by seroconversion. The anti-HIV antibodies formed during seroconversion are the basis for most initial HIV screening done in the United States. Because seroconversion takes different lengths of time in different individuals, multiple AIDS tests are given months apart to confirm or eliminate the possibility of infection.

Questions & Answers

what is oesophagus?
Bhavani Reply
oesophagus also known as food pip
ABDULLAH
What is the meaning of dissection
Beryl Reply
what's the meaning of polar?
Jackson Reply
Sign of anaemia or whitish color or Hgb luck
Kassahn
Are you talking of Polar anaemia or what, because the word polar doesnt have one meaning
Kingsley
what are the three types of adrenocorticoids hormones
Lubega Reply
glucocorticoids mineralocorticoid and catecholamines
Kartik
what is polar and non-polar
Jackson Reply
polar unequal share of electron while non polar is equal share
Quran
why are phosphate molecules negatively charged?
Jackson Reply
Bocz of unpaired elections
Javid
because of unpaired electrons
ABDULLAH
what are amphipathic molecules?
Jackson Reply
Amphipathic molecules are molecules with both polar and non polar regions
Mohammed
How does the male organ develop
MADUBULA Reply
Hw does the male organ develop
MADUBULA
Review your questions madam
Aliyu
what is anaphylaxis?
Rugut Reply
it is an imagency condition resulting from abnormal and immediate alagic response to a substance to which the body has become intensively sensitized
Lubega
different between drug and medicine
ado Reply
drugs have no medical application (cocaine, heroin, crystal meth). medicine have medical purpose (fentanyl, albuterol, aspirin, ect ect)
Jordan
medicine is a substance or preparation used in treating disease,drug is chemical compound medicine are drugs but all drugs are not medicines
Wafa
assalam o alaikum
Sidra
what happen to ECF and ICF regarding to OEDEMA
Zwanga Reply
what are the smooth muscles of the heart
Sintung Reply
stomach
Sidra
identify external features of kidney
saba Reply
kidney weight on males?
saba
and female ?
saba
Sle full name & treatment
Samim
Syplymic lupw erythematous
Samim
blood supply to spleen ?
saba
g..
saba
ya 135 g females
saba
130 on male
saba
130g on male
saba
g samim
saba
no 130grms on male
saba
mbbs
saba
good saba khan
Shahab
Tom kya krti ho st?
saba
your name?
saba
ok
saba
tum kya krti ho? muskan
saba
ok
saba
good muskan
Shahab
ok bye I m studying
saba
Define cranial nerves with oder
Javid
O-olfactory O-optic O-occumulator T-trochlear T-trigemenal A-abducent F-facial A-auditory G-glossopharyngeal V-vagus A-acessory
Aniee
opd mean?
Shahab
out patient department
mahesh
thnxx
Shahab
Ty Aniee singh
Javid
Ap sb us book s parhte h
Areeej
Read KD Tripathi book of Pharmacology.
mayank
Hello
mayank
CT Scan means
Sintung
what's health?
Sintung
hello
Philip
hi
Sintung
CT means - computerized tomography
Vivek
connective tissue
Dee
they link some body organs
Sintung
Difference between drug and medicine
Javid
hiii muskan
saba
what is tomography?
Gideon
tomography ☝
shahid
yes
Gideon
how hemolytic anemia cause due to gas gangrene?
Huma Reply
what's gangrene?
Sidra
why retro abdominal region called or named "flank "?
Huma Reply

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Source:  OpenStax, Anatomy & Physiology. OpenStax CNX. Feb 04, 2016 Download for free at http://legacy.cnx.org/content/col11496/1.8
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