4. Case study
This 2-year-old boy developed a high fever of 2 days’ duration.
Examination showed a miserable child with mild conjunctivitis,
a rash and cervical lymphadenopathy.
A viral infection was diagnosed and his mother was reassured.
When he presented to hospital 3 days later, he was noted to
have cracked red lips.
He was admitted and a full septic screen, including a lumbar
puncture, was performed and antibiotics started. The following
day, he was still febrile and irritable; his C-reactive protein
(CRP) had risen to 135, and ESR (erythrocyte sedimentation
rate) to 125.
5. Summary of vasculitis
immune-mediated :
Immune complex deposition
Antineutrophil cytoplasmic antibodies (ANCAs)
Anti-endothelial cell antibodies
invasion of vascular walls by infectious pathogens
Physical and chemical injury
6. Vessel Disease Notes
Large
Giant-cell arteritis >50. Arteries of head.
Takayasu arteritis F <40. “Pulseless disease”
Medium
Polyarteritis nodosa Young adults. Widespread.
Kawasaki diseaseKawasaki disease <4. Coronary disease. Lymph nodes.<4. Coronary disease. Lymph nodes.
Small
Wegener granulomatosis Lung, kidney. c-ANCA.
Churg-Strauss syndrome Lung. Eosinophils. Asthma. p-ANCA.
Microscopic polyangiitis Lung, kidney. p-ANCA.
7. Kawasaki disease
Kawasaki disease is a condition that causes inflammation in the walls of
medium-sized arteries throughout the body, including the coronary arteries,
which supply blood to the heart muscle.
Kawasaki disease is a group of specific symptoms and physical signs.
Kawasaki disease is associated with swelling and damage of the blood vessels,
or arteries. This swelling and damage is called inflammation.
Kawasaki disease often affects the arteries that supply the heart with blood.
These are called the coronary arteries.
Children who get the disease are usually less than 5 years old.
It is very rare to get the disease after a child is 10 years old.
The risk of a child getting Kawasaki disease for the second time is very rarethe second time is very rare.
8. Causes
Although the specific cause is unknown.
it is likely to be the result of immune hyperreactivity to a
variety of triggers in a genetically susceptible host (a
polymorphism in the ITPKCITPKC genegene, a negative regulator of T-
cell activation on chromosome 19chromosome 19 is strongly associated
with susceptibility to the disease).
9.
10. Phase 1 – Lasts 2 Weeks
104° temperature for 5
days
Red eyes
Sore throat
Swollen lymph nodes
18. Prognosis
With early treatment, rapid recovery from the acute symptoms can be expected,
and the risk of coronary artery aneurysms is greatly reduced.
Untreated, the acute symptoms of Kawasaki disease are self-limited (i.e. the
patient will recover eventually), but the risk of coronary artery involvement is
much greater.
Overall, about 2% of patients die from complications of coronary vasculitis.
Patients who have had Kawasaki disease should have an echocardiogram
initially every few weeks, and then every one or two years to screen for
progression of cardiac involvement.
A child with Kawasaki Disease will be irritable and cranky. At this stage they may be misdiagnosed with an earache or general cold. They probably won’t eat during this phase and dehydration can be a problem. The parents usually have a feeling that something is “just not right.” Antibiotics may be given, but will be of no help.
The child will be very uncomfortable and lethargic. They have no appetite and their body aches. The rash is red, dry and will itch. It is generally worse in the groin area.
The rash on the feet and hands make it very hard for the child to walk. The swelling can be painful. Could be misdiagnosed as Scarlet Fever at this stage.
Phase 2 starts about 2 weeks after fever has started. There should have been a positive diagnosis of Kawasaki Disease by this point. There are no standardized tests to diagnosis Kawasaki’s, other than qualifying the child with 4 out of 5 symptoms associated with the disease. That is why it is so important for doctors to familiarize themselves with these symptoms when presented together. The quicker the disease has been diagnosed, the less chance of lasting heart effects.
Along with the hands and feet peeling, my daughter also had her scalp peel. The rashes and the skin peeling, after we were discharged from the hospital, were almost as bad as being in the hospital with the disease itself.
Kawasaki’s can be easily misdiagnosed as one of these other diseases. That is why it is so important to know the symptoms and how they work together to confirm a diagnosis in the early stages. If treated incorrectly, lasting heart problems may occur.
After Kawasaki’s has been confirmed, Aspirin therapy is given to reduce blood clots. This is the only time a child should take aspirin, because of its association with Reye’s syndrome. However, when my daughter had Kawasaki’s, she tested positive for Influenza at the same time, so aspirin therapy was not even a choice for her.
The Gamma Globulin is given to decrease heart problems. If given within the first 10 days of the Kawasaki’s symptoms presenting themselves, it is a very successful treatment.
After being in the hospital for a few days, the child will have a series of EKG tests to determine if there is fluid around the heart or if any aneurysms have occurred. These tests may be given periodically for the next few months or even years if a problem has been detected.