Immunologic Deficiency Disorders

Question 1

In what ways can HIV be transmitted?

Answer 1

•Transmission: Horizontal (sexual partner), Vertical (from mom), Blood transfusions

Question 2

What is the pathophysiology of HIV?

Answer 2

• Affects CD4, T-cells, & T-lymphocytes
• HIV uses the CD4 cells to replicate itself
• Cell-mediated and humoral immunity suppressed

Question 3

What are the Common Clinical Manifestations of HIV Infection in Children?

Answer 3

• Lymphadenopathy
• Hepatosplenomegaly
• Oral candidiasis
• Chronic or recurrent diarrhea
• Failure to thrive
• Developmental delay - affects motor skills and language
• Parotitis – usually seen in kids and not in adults

Question 4

What are some common diseases seen in children with HIV?

Answer 4

• Pneumocystis carinii pneumonia – common cause of death
• Lymphoid interstitial pneumonitis (LIP) - more common in children
• Recurrent bacterial infections - more common in children
• Wasting syndrome
• Candidal esophagitis
• HIV encephalopathy
• Cytomegalovirus disease (CMV)
• Cryptosporidiosis

Question 5

What are some Distinguishing Characteristics Between Adults and Children with HIV/AIDS?

Answer 5

• Disease expression in infants influenced by:
  
  • Vertical transmission (from mother)
  • **Naïve immune system – leads to faster spread of the virus **
  • Effect of HIV on CNS – delayed growth and development, esp. language and motor skills
• AIDS defining conditions differ between adults and children

Question 6

How would you diagnose a child with HIV?

Answer 6

• < 18 months = viral culture, DNA test, HIV Ag testing (has to be positive on atleast 2/3 of tests)
• > 18months = Western blot

Question 7

How would you manage children with HIV?

Answer 7

• Slowing growth of HIV (this is the goal)
• Restoring normal growth and development
• Preventing infections and cancers – b/c of compromise, report when high fever
• Improve quality of life
• Prolong survival

Question 8

When would you start highly active anti-retroviral therapy to children?

Answer 8

• Infants (< 12 months) regardless of immunologic status (if they have HIV or AIDS)
• Child (> 12 months) with AIDS or severe immune suppression
• Consider in child with mild-moderate clinical s/s, moderate immune suppression or HIV RNA > 100,000

Goal is to reduce disease progression

Question 9

What would you educate children on in relation to HIV?

Answer 9

• Transmission and control of disease
• Universal precautions in daycare and schools
• No IV drug use
• Teach school staff that they won’t spread disease to others
• It is the law that they are allowed to go to public schools and daycares
• Take all medications as indicated
• Get immunizations when able to do so, don’t if severely immunocompromised (esp. MMR and Varicella)

Question 10

What is Severe Combined Immunodeficiency Disease (SCID) and what are some characteristics of it?

Answer 10

• Defect characterized by absence of both humoral and cell-mediated immunity – NO IMMUNE SYSTEM
• Therapeutic management
• Nursing considerations
  
  • Very susceptible to infections from birth, they will get many infections.
  • Fatal if not treated, only management is to give them a stem cell transplant.
  • Protect them from infection, give family support.
  • If stem cell transplant doesn’t work, give family a lot of support … It is always fatal.
  • If the transplant is given within 3months of diagnosis 95% of them will live (has to be diagnosed by 3 months of age)

Question 11

What is Wiskott-Aldrich Syndrome?

Answer 11

Disease Characterized by 3 abnormalities:

1. Thrombocytopenia
2. Eczema
3. Immunodeficiency of selective functions of B-lymphocytes and T-lymphocytes

X-linked recessive (like sickle-cell)

Poor prognosis