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Pathophysiology (Theoretical Based)
Impaired Immune System function
Modifiable Factors
Environment: Exposure to virus or bacteria
Non-Modifiable Factors
Age: < 5 years old
Race: Asian (mostly in Japan/Korean/Chinese)
Gender: Boys > Girls (2:1)
Genetics: ITPKC gene(inositol-trisphosphate 3-kinase C)
GENETIC
ENVIRONMENTALGENDERAGERACE
Asian< 5 years old
MaleITPKC
inositol 1,4,5-
trisphosphate 3-
kinase C
Infection:bacterial/viral
Idiopathic
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Exposure to bacteria and/or virus
Detection of foreign Body
Activation of Cell
Immune Response
T-cells in thymus gland go in the lymph nodes B-cells
activationWidespread proliferation &
activation of platelets
Regulator T-cells in
lymph nodes are
stimulated
Effector T-cells in
lymph nodes are
stimulated
Cytotoxic T-cell
production
Suppresor
T-cells
Helper T-cells
activation
Attracts and
activates more
B-cells,
cytotoxic T-
cells, Natural
killer cells,Macrophages
and other cells
in immune
system
No control
of B-cell
activity
Lymphokine
Production
Attracts other
lymphocytes andWBC
Release of
multitude of
proinflammatory
cytokines
Spontaneous production
of cytotoxic antibodies
and immune complexes
General
Circulation
of body
Attacks and
localizes on
the system of
the body
Lymph nodesLymphadenitis Palpable lymph nodes on the cervical
area
EyesBilateral conjunctivitis, with or
without pus or drainageRedness of the eyes
Mucosa Strawberry tongue
Red mucous
membrane in mouth
Skin Lysis of normal
cells in the skin
Desquamation or peeling of the
skin, beginning on the nails
Red palms and soles of the feet
Non-vesicle-bullous, non-itchy, non-specific, polymorphic rash
in the trunk
Musculoskeletal Polyarthritis (+) Pain
Finger gangrene
CVS AneurysmInflammation of arteries
Stagnant blood flow,
activated platelets,
inflammed endothelium
Th
o
Localized stenosisMI,
