Kawasaki Disease

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Kawasaki Disease by Mind Map: Kawasaki Disease

1. Pathophysiology

1.1. acute systemic vasculitis of unknown cause

1.1.1. extensive inflammation

1.1.1.1. arterioles

1.1.1.2. venules

1.1.1.3. capillaries

1.2. segmental damage to medium size muscular arteries

1.2.1. mainly coronary arteries

1.2.1.1. coronary artery aneurysm

1.2.1.1.1. severe scar formation and stenosis

1.2.2. coronary thrombosis

1.2.2.1. myocardial ischemia

1.3. etiology

1.3.1. most common in children of Korean and Japanese descent, but is seen in every racial group

1.3.2. 75% of cases occur in children under age 5

1.3.3. 20% develop coronary artery dilation or aneurysm formation

1.3.4. infants younger than 1 year are most seriously affected and are at the greatest risk for cardiac involvement

1.3.5. not spread by person-to-person contact, however, several factors support infectious etiologic factors

1.3.6. most cases reported in late winter and early spring

2. Diagnostics

2.1. based on clinical findings and lab results

2.1.1. must have fever for 5+ days along with 4 of 5 clinical criteria

2.1.1.1. 1. changes in extermities

2.1.1.1.1. acute phase: edema, erythema of palms and soles

2.1.1.1.2. subacute phase: periungual desquamation of hands and feet

2.1.1.2. 2. bilateral conjunctival injection without exudation

2.1.1.3. 3. changes in oral mucous membranes

2.1.1.3.1. erythema of lips

2.1.1.3.2. oropharyngeal reddening ("strawberry tongue")

2.1.1.4. 4. polymorphous rash

2.1.1.5. 5. cervical lymphadenopathy

2.1.1.5.1. one lymph node >1.5 cm

3. Clinical Signs and Symptoms

3.1. acute phase

3.1.1. high fever

3.1.1.1. abrupt onset

3.1.1.2. unresponsive to antibiotics or antipyretics

3.1.2. child typically extremely irritable

3.1.3. inflammation of the myocardium

3.1.3.1. myocarditis

3.1.3.2. valvulitis

3.1.3.3. dysrhythmias

3.1.4. polymorphous rash

3.1.5. cervical lymphadenopathy

3.1.6. edema

3.1.7. erythema of palms, soles, and lips

3.1.8. erythema of oropharyngeal membranes ("strawberry tongue")

3.1.9. bilateral conjunctivitis without exudate

3.2. subacute phase

3.2.1. resolution of fever

3.2.2. lasts until all clinical signs have disappeared

3.2.3. greatest risk for development of coronary artery aneurysm

3.2.3.1. echocardiogram to monitor myocardial and coronary artery status

3.2.3.2. can cause acute myocardial infarction

3.2.3.2.1. signs and symptoms

3.2.4. irritability continues from acute phase

3.2.5. periungual desquamation of hands and feet

3.3. convalescent phase

3.3.1. all clinical signs have resolved

3.3.2. lab values have not returned to normal

3.3.3. phase complete when all blood values return to normal

3.3.3.1. 6-8 weeks following initial onset

4. Managament

4.1. medications

4.1.1. high dose IVGG

4.1.1.1. retreatment for those who continue to have a fever following

4.1.2. IVIG in first 10 days to reduce coronary artery abnormalities

4.1.2.1. monitor vital signs frequently for reactions

4.1.2.2. monitor cardiac status because large volume of fluid delivered to heart with diminished left ventricular function

4.1.3. aspirin (80-100mg/kg/day in divided doses every 6 hours)

4.1.4. after fever subsides, aspirin continued at antiplatelet dose (3-5mg/kg/day)

4.1.4.1. continues indefinitely if coronary artery abnormalities occur

4.1.5. additional anticoagulants in those with medium or giant coronary artery aneurysms

4.1.5.1. clopidogrel (Plavix)

4.1.5.2. enoxaparin (Lovenox)

4.1.5.3. warfarin

4.1.6. Digoxin to increase contractility of the heart

4.1.7. angiotensin enzyme inhibitors to reduce afterload on the heart

4.1.8. lasix to remove excess fluid

4.1.8.1. potassium supplements

4.1.8.2. bananas

4.1.8.3. green leafy vegetables

4.2. prognosis

4.2.1. most recover fully after treatment

4.2.2. serious morbidity occurs with cardiovascular complications

4.2.3. those with giant aneurysms have the highest risk for complications

4.3. monitor cardiac status

4.3.1. strict intake and output

4.3.2. daily weight recording

4.3.3. fluid administration with extreme care because myocarditis is common

4.3.4. frequent assessment for signs of heart failure

4.3.4.1. decreased urinary output

4.3.4.2. gallop rhythm

4.3.4.3. tachycardia

4.3.4.4. respiratory distress

4.4. symptomatic relief

4.4.1. minimize skin discomfort

4.4.1.1. cool cloths

4.4.1.2. unscented lotions

4.4.1.3. soft, loose clothing

4.4.2. mouth care during acute phase

4.4.2.1. lubricating ointment to decrease mucosal inflammation

4.4.3. offer clear liquids and soft foods

4.4.4. relieve patient's irritability

4.4.4.1. quiet, dark environment to decrease stimuli

4.4.4.2. support parent efforts to comfort inconsolable child

4.4.4.2.1. provide respite care for the family

4.4.4.2.2. alleviate feelings of guilt and embarrassment regarding child's behavior

5. Family Concerns

5.1. discharge teaching

5.1.1. progression of Kawasaki Disease

5.1.1.1. importance of follow up monitoring

5.1.1.1.1. frequency and type determined by the presence or absence of coronary damage

5.1.1.1.2. recommended that child be screened and treated for coronary risk factors as they grow older

5.1.1.1.3. prevention and detection of coronary ischemia

5.1.1.2. when to contact their health care provider

5.1.2. irritability likely to continue for up to 2 months after the onset of symptoms

5.1.3. periungual desquamation begins in the second and third weeks

5.1.3.1. fingers first then feet

5.1.3.2. peeling is painless

5.1.3.3. new skin may be tender

5.1.4. arthritis may occur and persist for several weeks

5.1.4.1. temporary

5.1.4.2. occurs most often in the larger weight-bearing joints

5.1.4.3. stiffness triggers

5.1.4.3.1. in the morning after waking

5.1.4.3.2. cold weather

5.1.4.3.3. after naps

5.1.4.4. passive range of motion exercises help increase flexibility

5.1.5. any live immunizations (measles, mumps, and rubella, varicella) should be deferred for 11 months after the administration of IVGG

5.1.5.1. body may not produce enough antibodies for lifelong immunity

5.1.5.2. receiving the varicella vaccine during aspirin therapy may cause Reye's Syndrome

5.1.6. record temperature following discharge, report any fever to the health care provider

5.1.7. children with large aneurysms

5.1.7.1. possibility of myocardial infarction

5.1.7.2. signs and symptoms of cardiac ischemia in a child

5.1.7.3. CPR education

5.2. longterm concerns regarding child's health

5.2.1. lower overall perception of child's health status

5.2.2. stress, anxiety, fear, and uncertainty

6. Child Concerns

6.1. development of cardiac sequelae

6.1.1. heart failure

6.1.2. coronary artery aneurysm

6.1.3. myocardial ischemia

6.1.4. myocardial infarction

6.2. impact on quality of life

6.2.1. continuous antiplatelet therapy increases bleeding risk

6.2.1.1. may change the way the child plays

6.2.1.2. may not be able to participate in certain activities/sports

6.2.2. with development of cardiac sequelae, child may experience long-term cardiac complications

6.2.2.1. premature development of heart disease

6.2.2.2. higher risk for heart disease overall

6.2.2.3. frequent visits to healthcare facilities

6.2.2.4. may feel isolated by others due to illness

6.3. behavioral changes during course of illness

6.3.1. child experiences up to two months of extreme irritability

6.3.1.1. may have impact on social relationships

6.3.1.2. may experience problems in school or miss significant amount of school entirely

6.3.2. may experience fear and anxiety while receiving treatment

6.3.3. may not be able to understand what's happening to them