kawasaki%20disease
KAWASAKI DISEASE
Kawasaki disease is an acute, febrile illness that is self-limited. It is a systemic vasculitic syndrome that primarily involves the medium- and small-sized muscular arteries of the body.
It is also known as mucocutaneous lymph node syndrome.
It affects primarily children <5 years old with peak incidence in 1-2 year of age.
The cause remains unknown but current research supports an infectious origin.
Epidemiological findings suggest that genetic predisposition and environmental factors play a role in the pathogenesis of the disease.

Monitoring

Long-Term Follow-Up

W/o Detectable Coronary Lesions

  • Children w/ no known cardiac sequelae during the 1st month of Kawasaki disease appear to return to their previous state of health, w/o signs or symptoms of cardiac impairment
  • Altered lipid metabolism persists beyond clinical resolution of the disease
  • Lower myocardial flow reserve & higher total coronary resistance have been found in those w/ history of Kawasaki disease w/ normal epicardial coronary arteries

Regression of Coronary Lesions

  • Coronary artery lesions resulting from Kawasaki disease change dynamically w/ time
  • The likelihood that an aneurysm will resolve appears to be determined by:
    • Initial size: smaller aneurysms have a greater likelihood of regression
    • <1 year of age at onset of the disease
    • Fusiform morphology
    • Location in distal coronary segment

Persistent Coronary Artery Abnormalities

  • Stenotic lesions that are secondary to marked myointimal proliferation are frequently progressive
  • Highest rate of progression to stenosis occurs in patients w/ large aneurysms
  • Worst prognosis occurs in children w/ giant aneurysms since thrombosis is promoted by sluggish blood flow w/in massively dilated vascular space & the frequent occurrence of stenotic lesions at the proximal or distal end of the aneurysms

Cardiovascular Risk Stratification

  • Stratification of patients according to their relative risk of myocardial ischemia allows for patient management to be individualized
  • The risk level for a given patient w/ coronary artery involvement may change over time because of the changes in coronary artery morphology
Risk Level I - Patients w/o coronary artery changes on echocardiography at any stage of the illness
  • No antiplatelet therapy nor restriction of physical activity is necessary beyond the initial 6-8 weeks after the onset of illness; dilatation subsides w/in 30 days after onset
  • Suggest periodic assessment & counseling about known cardiovascular risk factors every 5 years

Risk Level II - Patients w/ transient coronary artery ectasia or dilatation, which disappears w/in the initial 6-8 weeks after the onset of illness

  • No antiplatelet therapy nor restriction of physical activity is necessary beyond the initial 6-8 weeks after the onset of illness
  • Recommend risk assessment & counseling at 3-5 year intervals

Risk Level III - Patients w/ solitary small-to-medium (>3 mm but <6 mm) coronary artery aneurysm in >1 coronary arteries on echocardiography or angiography, & aneurysm persists or worsens beyond 30 days after onset

  • Long term antiplatelet therapy w/ low-dose Aspirin should be administered until the aneurysms regress
  • No restriction on physical activity in patients <11 years of age
  • For patients 11-20 years of age, stress tests w/ myocardial perfusion evaluation may be useful to guide recommendations for physical activity
    • Due to risk of bleeding, participation in competitive collision or high-impact sports is discouraged in children receiving antiplatelet therapy
  • Annual follow-up by a pediatric cardiologist w/ echocardiogram (ECG) is recommended
    • Stress test w/ myocardial perfusion imaging is recommended every 2 years in patients >10 years of age
  • Coronary angiography is indicated if myocardial ischemia is demonstrated by non-invasive tests

Risk Level IV - Patients w/ ≥1 large coronary artery aneurysm (≥6 mm) including giant aneurysms & patients in whom a coronary artery contains multiple (segmented) or complex aneurysms w/o obstruction

  • Long term antiplatelet therapy is recommended
    • Adjunctive therapy w/ Warfarin w/ a target International Normalized Ratio (INR) of 2-2.5 is recommended for patients w/ giant aneurysms
    • Low molecular weight Heparin may be considered as an alternative to Warfarin for infants & toddlers
  • Recommendation about physical activity should be guided by annual stress tests w/ myocardial perfusion evaluation
    • Collision or high-impact sports should be discouraged
    • Participation in non contact dynamic or recreational sports is encouraged if there is no evidence of stress-induced myocardial ischemia
  • Cardiology evaluation w/ echocardiogram (ECG) should be done at 6 months intervals
  • Stress tests w/ myocardial perfusion evaluation should be performed annually
  • Monitor for known risk factors of atherosclerosis; counsel family accordingly
  • Cardiac catheterization w/ selective coronary angiography should be performed 6-12 months after recovery from the acute illness, or sooner if clinically indicated
  • Follow-up angiography may be indicated if noninvasive studies suggest myocardial ischemia
  • For females of childbearing age, reproductive counseling is strongly recommended

Risk Level V - Patients w/ coronary artery obstruction confirmed by angiography

  • Long-term antiplatelet therapy, w/ or w/o adjunctive therapy w/ Warfarin anticoagulation, is recommended
  • Beta-blockers should be considered to reduce myocardial oxygen consumption
  • Recommendation about dynamic physical activities should be based on the patient’s response to stress testing
    • Collision or high-impact sports should be discouraged
  • Patients should avoid a sedentary lifestyle
  • Cardiology evaluation w/ an echocardiogram (ECG) should be obtained at 6 months intervals
  • Stress tests w/ myocardial perfusion evaluation should be performed annually
  • Monitor patient for known risk factors of atherosclerosis; counsel family accordingly
  • Cardiac catheterization w/ selective coronary angiography is recommended to address the therapeutic options of bypass grafting or catheter intervention & to identify the extent of collateral perfusion
  • Repeat cardiac catheterization may be indicated when new onset or worsening myocardial ischemia is suggested by noninvasive diagnostic testing or clinical presentation
  • For females of childbearing age, reproductive counseling is strongly recommended

Prognosis

  • Depends on cardiac involvement & its long-term sequelae
  • Coronary artery aneurysms develop in 20-40% of untreated children or in patients treated w/ only Aspirin, which may eventually lead to rupture in adulthood, ischemic heart disease & myocardial infarction (MI)
  • Aneurysms might be demonstrated in other extraparenchymal muscular arteries except the central nervous system (CNS) arteries

Prevention

  • Schedule for administration of inactivated childhood vaccines should not be interrupted
  • It may be necessary to reschedule administration of live viral vaccines (eg measles, varicella-containing vaccines) to at least 9-11 months after intravenous immunoglobulin (IVIG) administration
    • If the risk of exposure to measles is high, patients may be vaccinated earlier & then reimmunized ≥11 months after intravenous immunoglobulin (IVIG) administration if the patient has an inadequate serological response
  • Annual influenza vaccination is recommended in patients who are on long-term therapy w/ Aspirin to reduce the risk of Reye’s syndrome
Digital Edition
Asia's trusted medical magazine for healthcare professionals. Get your MIMS JPOG - Malaysia digital copy today!
DOWNLOAD
Editor's Recommendations
Most Read Articles
Audrey Abella, 31 Jan 2017
Nonsteroidal anti-inflammatory drug (NSAID) treatment for patent ductus arteriosus (PDA) in preterm infants did not reduce the odds of mortality or bronchopulmonary dysplasia (BPD), a recent US study found.
Jairia Dela Cruz, 13 Oct 2016
Children born to obese mothers are at increased risk of developing autism spectrum disorder (ASD) compared with children born to normal-weight mothers, according to data from a review and meta-analysis.
Yap Te-Lu, Anette Sundfor Jacobsen, 01 Oct 2013

Antenatal hydronephrosis (ANH) is a general term used to describe the dilatation of the fetal renal pelvis and/or its calyces. In pelviectasis, there is only dilatation of the renal pelvis; while in caliectasis, there is dilatation of the calyces. ANH is the most commonly diagnosed congenital urinary tract anomaly, which is detected by prenatal screening in 1–5% of all pregnancies