Immune Deficiency disorders

Immunodeficiencies

Deficiencies in the proper expression of the immune system response.

Primary immunodeficiencies

Secondary immunodeficiencies

Immunodeficiencies classification

• Deficiencies resulting from the improper development of the immunosuppressive cells and tissues  

• Mainly genetic disorders seen in children

• Example: T-Cell Deficiency: Di-Goerge’s Syndrome ( Thymic Hypoplasia) - Absence of thymus in the neonate

Primary immunodeficiencies

• Any condition that can interfere with the normal growth or expression of the immune response system

• Example: HIV/ AIDS

Secondary Immunodeficiencies

HIV

• CD4 cells

_______Weakening of the body’s immune system from opportunistic infections due to an infection that attacks the body’s immune system, specifically the _______

semen, vaginal fluids, blood, breastmilk

4 body fluids that transmit HIV

• 80% sexual contact

• 10 % use of needles

• <10% mother to child

three modes of Transmission of HIV

• Attachment

• uncoating

• DNA synthesis

• Integration

• Transcription

• translation

• Cleavage

• Budding

HIV LIFE CYCLE

• Glycoproteins (GP120 and GP41)

• CD4+ receptor and chemokine coreceptors (CCR5)

Attachment:

_______________of HIV bind with the host’s uninfected ______________resulting in fusion of HIV with CD4+ T Cell membrane

• two single strands of viral RNA

• three viral enzymes: reverse transcriptase, integrase, and protease

UNCOATING

• HIV’s viral core = _______ + ________: __________ are emptied into the CD4+ T Cell membrane

Uncoating

• HIV’s viral core = two single strands of viral RNA + three viral enzymes: reverse transcriptase, integrase, and protease are emptied into the CD4+ T Cell membrane

• reverse transcriptase

DNA Synthesis

• Through ___________, HIV changes its genetic material from RNA to DNA resulting in double stranded DNA that carries instruction for viral replication

integrase

Integration:

• Through action of _______, the new viral DNA enters the nucleus of the CD4+ T cell is blended with the DNA of the CD4+ T cell resulting in permanent, lifelong infection

transcription

• single-stranded messenger RNA (mRNA)

_________
CD4+ T cell is activated and the double-stranded DNA forms ___________ and builds new viruses

Translation

• polyproteins

________

• New chains of new proteins and enzymes (________) created by mRNA contain the components needed in the construction of new viruses

Cleavage

__________

• The polyprotein chain is cut by the HIV enzyme resulting into individual proteins that make up the new virus

Budding

• The viral RNA and new proteins migrate to the membrane of the infected CD4+ T cell, exit from the cell and restart the process.

Stage 1: Asymptomatic

• fever, bulding of lymph nodes

Stage 2: Mild Disease

• antibodies detectable in blood

Stage 3: Advance Disease

• opportunistic infection

AIDS/ AHD

• severe infection and development of cancer

Stages of HIV disease

• CD4+ T cells = ≥ 200-499/µL

• Candidiasis, Idiopathic thrombocytopenic purpura, peripheral neuropathy, fever (38.5°C), or diarrhea exceeding 1 month in duration

  
  

Symptomatic HIV infection

• CD4+ count

• Symptoms

Acquired immunodeficiency syndrome

advanced HIV disease

AIDS stands for?

also known as?

• <200 / uL

• wasting syndrome, Kaposi’s sarcoma, Pneumocystis jiroveci pneumonia (PCP), toxoplasmosis of the brain, Mycobacterium tuberculosis

  
  

AIDS

• Cd4+ count

• Symptoms

• Shortness of breath, dyspnea, cough, chest pain, and fever

• Associated Diseases: Pneumocystis Pneumonia (most common infection), Mycobacterium avium Complex Tuberculosis

HIV RESPIRATORY SYSTEM

Loss of appetite, nausea, vomiting, oral and esophageal candidiasis, and chronic diarrhea causing profound weight loss

Associated Diseases: Oral candidiasis and Wasting syndrome

HIV GASTROINTESTINAL

Flat or raised, brownish pink to deep purple skin discolorations, lymphedema, pain

Associated Diseases: Kaposi’s sarcoma , B-Cell Lymphomas, and Cervical carcinoma

HIV ONCOLOGIC

Kaposi sarcoma

(most common HIV-related malignancy)

• Motor dysfunction and behavioral change

• Associated Diseases:

  • Peripheral neuropathy

  • HIV encephalopathy

  • Cryptococcus neoformans

  • Progressive multifocal leukoencephalopathy

HIV NEUROLOGIC

• Rashes, skin discolorations

• Associated Diseases:

  • Herpes zoster and herpes simplex

  • Molluscum contagiosum

  • Seborrheic dermatitis

  • Eczema or Psoriasis

HIV INTEGUMENTARY

• Persistent, recurrent vaginal candidiasis

• Associated Diseases:

  • Ulcerative sexually transmitted diseases

  • (STDs are more severe in women with HIV)

HIV GYNECOLOGIC

CIA/ ELISA

western blot

Viral load

CD4 / CD8 test

Ora quick

Diagnostic Test for HIV

ELISA / CIA

Antibodies are detected, resulting in positive results, and marking the end of the window period

Western Blot

detects antibodies to HIV; used to confirm EIA

Viral Load

Measures HIV RNA in the plasma

CD4 / CD8 Test

These are markers found on lymphocytes. HIV kills CD4+

cells, which results in  significantly impaired immune system

Ora quick

In-home HIV Self Test. Works by looking body’ responses (antibodies) to fighting HIV. + result is preliminary and ff

up confirmatory test is needed

• PreP

• HAART (highly active antiretroviral therapy)

Treatment for HIV

Abstinence

Be faithful
Consistent and correct condom use

Do not use Drugs and Alcohol

Education

PRe & Post exposure prophylaxis

HIV PREVENTION

AZT Retrovir / Zidovudine

Pharmacotherapy for HIV

Malaise, Nausea, headache, Fever, Insomnia, Myalgia

Side effects of AZT (retrovir/ zidovudine)

Bone Marrow Suppression (serum level determination should be done at regular basis); weekly CBC should be monitored

Toxic effect of AZT (retrovir/ zidovudine)

•  Avoid pregnancy, Do not donate blood, sperm, organs

Precaution for AZT (retrovir/ zidovudine)

Primary immunodeficiencies

• Deficiencies resulting from the improper development of the immunosuppressive cells and tissues

T cell deficiency : Di-George’s Syndrome (thymic hypoplasia)

Example of Primary immunodeficiencies

• Sharing of infected injection drug use equipment

• Sexual relations with infected individuals

• Infant born to mothers with HIV infection and/or who are breast fed by HIV infected mothers

• People who received organ transplants, HIV-infected blood, or blood products

HIGH RISK FOR HIV

Reverse transcription

_______ produces double strand HIV

HIV

transmission

Enters body

Attack CD4+ T cells

Immunosupression

5 pathophysiology of HIV

CD4+ T Cells

_______ is important in immunity against cancer, other viruses, and parasites

 Pneumocystis Pneumonia

most common infection due to HIV

50 copies/ mL

1-3 months

Treatment shoudl reduce viral load to less than ______ within _______. the risk from HIV then becomes zero

CD4/ CD8

 markers found on lymphocytes.

• reduction in replication of HIV,

• reduction in morbidity and mortality

• Rise in CD4+ T cell count

• Reduce viral load

HAART is for

• 
  

• biohazard bag/red bag

• 1:10

• 20

• hot water, air drying)

• use bleach

• razors, toothbrush

• Use heavy plastic bags to be used for articles contaminated with body fluids (____________)

• Use of ____ Bleach/Water Solution

• Boiling equipment (___minutes)

• Dishwashing (________,______-)

• Laundry (_____)

• Do not share ____-,______. These are contaminated with body secretions

• abstinence

• mutual, monogamous sexual relationship

• avoid multiple partners

• soluble lubricants and condoms

• Advice client to practice safe sex as follows:

advanced HIV disease,

All children younger than 5 years of age living with HIV are considered to have _________ regardless of clinical or immunological status. 

Hep B

Hep C

MPox

HIV can make other infections, such as ______,________and____, get worse.

True

no single test can provide a full HIV positive diagnosis; confirmatory testing is required,

28 days

window period

In most cases, people develop antibodies to HIV within ____s of infection. During this time, people are in the so-called “_______” when they have low levels of antibodies which cannot be detected by many rapid tests, but they may still transmit HIV to others. People who have had a recent high-risk exposure and test negative can have a further test after 28 days.

rapid antibody testing

virological testing

For children less than 18 months of age,_______is not sufficient to identify HIV infection – ______ must be provided as early as birth or at 6 weeks of age.

• oral tenofovir (TDF)-based PrEP  

• dapivirine vaginal ring

• long-acting injectable cabotegravir

• long-acting injectable lenacapavir.

WHO recommends the following PrEP methods:  

ARVs

_____ can also be used to prevent mothers from passing HIV to their children.

• People taking ART and who have no evidence of virus in the blood will not pass HIV to their sexual partners.

antiretroviral drugs

There is no cure for HIV infection. It is treated with ______, which stop the virus from replicating in the body.