Professional Documents
Culture Documents
Rashmita Devi
Tutor
AIIMS Kalyani
Introduction
• Immunodeficiency disorders may be caused by
a defect or deficiency in phagocytic cells, B
lymphocytes, T lymphocytes, or the
complement system
Classification
Primary immunodeficiency
• Primary immunodeficiencies, rare disorders
with genetic origins, are seen primarily in
infants and young children.
• Symptoms usually develop early in life after
protection from maternal antibodies
decreases
• These disorders may involve one or more
components of the immune system
PHAGOCYTIC DYSFUNCTION
• Primary defects of phagocytes are genetic in
origin and affect the innate immune system
• In some types of phagocytic disorders, the
neutrophils are impaired so that they cannot exit
the circulation and travel to sites of infection.
• In some disorders, the neutrophil count may be
very low; in others, it may be very high because
the neutrophils remain in the vascular system.
Clinical Manifestations
• Increased incidence of bacterial and fungal
infections
• ( fungal :Candida viral: herpes simplex or
herpes zoster virus.)
• One type of phagocytic disorder, hyper
immuno globulinemia E (HIE) syndrome,
formerly known as Job syndrome
• Severe neutropenia may be accompanied by
deep and painful mouth ulcers, gingivitis,
stomatitis, and cellulitis
Job syndrome
• Job Syndrome (Hyper-IgE
syndrome) is a rare, primary
immunodeficiency
distinguished by the clinical
triad of atopic dermatitis,
recurrent skin staphylococcal
infections, and recurrent
pulmonary infections.
• The disease is characterized by
elevated IgE levels with an early
onset in primary childhood
Assessment and Diagnostic Findings
• History
• Lab test :
1. Nitroblue tetrazolium reductase test
a blood test that measures the ability of the
immune system to convert the colorless
nitroblue tetrazolium (NBT) to a deep blue.
Medical Management
• Treating bacterial infections with prophylactic
antibiotic therapy
• treatment for fungal and viral infections
• Granulocyte transfusion
• Granulocyte-macrophage colony-stimulating
factor (GM-CSF) or granulocyte colony-
stimulating factor (G-CSF)
• (these proteins draw non lymphoid stem cells
from the bone marrow and hasten their
maturation.)
B-CELL DEFICIENCIES
• Two types of inherited B-cell deficiencies.
• The first type results from lack of differentiation
of B-cell precursors into mature B cells.
• As a result, plasma cells are lacking, leading to a
complete lack of antibody production against
invading bacteria, viruses, and other pathogens.
• The second type of B-cell deficiency results from
a lack of differentiation of B cells into plasma
cells.
• Only diminished antibody production occurs
with this disorder.
Clinical Manifestations
• Recurrent pyogenic infections usually occur by 5
to 6 months of age
• More than half of patients develop pernicious
anemia.
• Lymphoid hyperplasia of the small intestine and
spleen and gastric atrophy detected by biopsy of
the stomach are common findings.
• Other autoimmune diseases, such as arthritis and
hypothyroidism, frequently develop in patients.
Diagnostic evaluation
• Sex-linked agammaglobulinemia may be
diagnosed by the marked deficiency or
complete absence of all serum
immunoglobulins.
• Antibody titers to confirm successful
childhood vaccination are determined by
specific serologic tests.
Medical Management
• Intravenous immunoglobulin (IVIG)
• Antimicrobial therapy is prescribed for respiratory
infections to prevent complications such as pneumonia,
sinusitis, and otitis media.
• Intestinal infestation with G. lamblia is treated with a
10-day course of metronidazole (Flagyl) or a 7-day
course of quinacrine hydrochloride (Atabrine)
Vital signs
Development of pain, neurologic signs,
cough, and skin lesions are monitored and
reported.
Pulse rate and respiratory rate should be
counted for a full minute, as even subtle
changes can signal deterioration in the
patient’s clinical status.
Assessment also focuses on nutritional
status; stress level and coping skills; use
of alcohol, drugs, or tobacco; and
general hygiene, all of which may affect
immune function.
Strategies the patient has used to
reduce risk for infection are identified.
Other aspects of nursing care
• Directed toward reducing the patient’s risk for
infection
• Measures aimed at improving immune status
and treating infection, improving the nutritional
status, and maintaining bowel and bladder
function.
• These include careful hand hygiene,
encouraging the patient to cough and perform
deep-breathing exercises at regular intervals,
• protecting the integrity of the skin and mucous
membranes.
• Health care personnel must use strict aseptic
technique when performing invasive
procedures, such as dressing changes,
venipunctures, and bladder catheterizations.
• Other aspects of nursing care include assisting
the patient in managing stress and in adopting
a lifestyle that enhances immune system
function