Immunologic Deficiency Disorder

HAART (highly active antiretroviral therapy)

retrovirus that is transmitted by lymphocytes and monocytes. Found in blood, semen, vaginal secretions and breast milk. Incubation period is months to years.

occurs through intimate sexual contact or parenteral exposure to blood or body fluids containing visible blood

occurs when and HIV-infected pregnant woman passes the infection to her infant

primarily infects a specific subset of T lymphocytes, the CD₄⁺ T cells. Virus takes over the machinery of the CD₄⁺ lymphocytes, using it to replace itself, rendering the cell dysfunctional. the count gradually decreases over time leading to progressive immunodeficiency.

• *lymphadenopathy
• *hepatosplenomegaly
• *oral candidiasis
• *diarrhea
• *failure to thrive
• *developmental delay
• *parotitis

pneumonia, recurrent bacterial infections, wasting syndrome, HIV encephalopathy, herpes, short stature, malnutrition...etc.

• After 18 months of age, the ELISA (HIV enzyme-linked immunosorbent assay) and Western blot immunoassay are used for determination.
• In babies <18 months, the maternal antibodies cover this up so the PCR (HIV polymerase chain reaction) is used for detection

Goals: slowing growth of virus, preventing and treating opportunistic infections, and providing nutritional support and symptomatic treatment.

work at various stages of HIV life cycles to prevent reproduction of functional new virus particles. NOT CURABLE but these drugs can suppress viral replication and prevent further deterioration of immune system. THERAPY IS LIFE LONG!

Pneumocystis carinii pneumonia (PCP). Most frequently b/t 3-6 months of age.

Immunization against all common childhood illnesses is recommended to children exposed and infected with HIV. If severely immunocompromised....don't give chicken pox and MMR vaccine. Immediate prophylaxis after exposure to vaccines is warrented bc of poor antibody production to vaccines

• Education on transmission and control of infections is essential, safety issues, ongoing education about HIV to dispel myths, PREVENTION IS KEY to education....routes of transmission, hazards of IV drug use, sex, etc..
• Ongoing assessment of pain is crucial (emla cream, tylenol, nsaids, opiods)

absence of both humoral and cell-mediated immunity. (terms swiss-type lymphopenic agammaglobulinemia and X linked lymphopenic agammaglobulinemia) also describe this disorder

Susceptability to infection occurs early in life (first month). Failure to thrive is a consequence of this illness.

Usually based on history of recurrent, severe infections from early infancy, familial history of the disorder and specific laboratory findings, which include lymphopenia, lack of lymphocyte response to antigens, and absence of plasma cells in bone marrow.

Definitive treatment is HSCT from histocompatible donor (usually parent) or matched un-related donor. IVIG infusions and PCP prophylaxis are used to augment the humoral immunity until the transplant is performed

Preventing infection and supporting child and family. Since prognosis is VERY poor, if there is no compatible donor, nursing care is directed at supporting family in caring for a child with a life-threatening illness.

WAS is an X-linked recessive disorder characterized by a triad: thrombocytopenia, eczema, and immunodeficiency of selective functions of B lymphocytes and T-lymphocytes.

bloody diarrhea from thrombocytopenia, as child grows older....recurrent infection and eczeme become more severe and bleeding becomes less frequent. Chronic infection with herpes is frequent and may lead to vision loss.

Counteracting the bleeding tendencies with platelet transfusions, using IV gamma globulin to provide passive immunity and administering prophylactic antibiotics to prevent and control infection

Only curative therapy is HSCT from matched donor

Poor prognosis, main consideration is supporting family in the care of a fatally ill child. Controlling physical problems, prevention or control of infections.