MCOS was the original name of Kawasaki disease --- it stands for mucocutaneous ocular syndrome. This child had died suddenly & unexpectedly.
Kawasaki disease is virtually unheard of in children older than 15 years of age.
In this convalescent phase remaining symptoms resolve & laboratory values normalize.
The complete list of associated symptom is too long to go into in detail here,but virtually every organ system can be involved. Patients can have cough, rhinorrhea, or a pulmonary infiltrate. They can have diarrhea, vomiting, abdominal pain, hydrops of gallbladder, mild jaundice, and mild increase of serum transaminase levels. They can have striking irritability and an aseptic meningitis with a mononuclear pleocytosis in cerebrospinal fluid as well as a facial palsy and hearing loss. Finally, they can develop myositis, arthralgias, and arthritis. Heme: hemophagocytosis Renal: ARF, renal artery aneurysms Skin: transverse furrows of fingernails (Beau’s lines) during convalescence Others: Peripheral gangrene, orbital myositis, Avascular necrosis of the femoral head
In fact, these 2 etiologies have been found to account for over 80% of patients initially thought to have Kawasaki disease but ultimately not diagnosed as such. These would include toxic shock syndrome & staph scalded skin syndrome.
In the first week, patients can develop bilateral, painless bulbar conjunctival injection without exudate. There tends to be no redness adjacent to the pupil and these patients may also have anterior uveitis which is shown on acute phase slit lamp exam in 80% In the first week, they may also have erythema and cracking of lips, a strawberry tongue, and/or diffuse injection of oral and pharyngeal mucosae.
The lymphadenopathy occurs early in the disease, and can be very transient. It may or may not be bilateral but is not generalized throughout the body. Lymph nodes, by definition, must be 1.5 cm in diameter or larger. About 90% of patients will have fever, conjunctival erythema, and oral changes and 70% will have lymphadenopathy.
In acute phase, the most common blood laboratory findings are: a leukocytosis with left shift, mild anemia, an increased erythrocyte sedimentation rate or C-reactive protein, hypoalbuminemia, elevation of liver transaminases Later in the illness, the platelet count tends to rise during the second week and can remain elevated for longer than 6-8 weeks. Finally, the urine can show sterile pyuria of urethral origin and occasional proteinuria.
From the cardiovascular standpoint, Patients can have no signs or symptoms. Sometimes, they have tachycardia out of proportion to the extent of fever. They can have a gallop rhythm or distant heart sounds suggestive of myocarditis or pericarditis with an effusion. They can have a flow murmur or a murmur due to valvar insufficiency. Or they can have frank congestive heart failure and/or an infarction. Their EKG can show a number of changes, including arrhythmias, abnormal Q waves, prolonged PR and/or QT intervals, low voltage, and ST-T–wave changes. Their chest x-ray can show cardiomegaly or pulmonary edema.
Ectasia is defined as coronary artery size larger than normal for age but without discrete aneurysm.
These signs/symptoms would include mitral regurgitation or pericardial effusion.
The management of patients is divided into four categories. . .
What is Kawasaki Disease?Idiopathic multisystem disease characterized by vasculitis of small & medium blood vessels, including coronary arteriesA self-limited vasculitis of unknown etiology that predominantly affects children younger than 5 years. It is now the most common cause of acquired heart disease in children*Burns, J. Adv. Pediatr. 48:157. 2001.
Epidemiology• 80% of cases in children < 4 yrs• Males:females = 2:1• Positive family history in 1% but 13% risk of occurrence in twins.• in-hospital mortality ≈ 0.17%• Annual incidence of 4-15/100,000 children under 5 years of age• Seasonal variation – More cases in winter and spring but occurs throughout the year
Phases of Disease• Acute (1-2 weeks from onset) – Febrile, irritable, toxic appearing – Oral changes, rash, edema/erythema of feet• Subacute (2-8 weeks from onset) – Desquamation, may have persistent arthritis or arthralgias – Gradual improvement even without treatment• Convalescent (Months to years later)
Differential Diagnosis• Infectious – Measles & Group A beta-hemolytic strep can closely resemble KD – Bacterial: severe staph infections w/toxin release – Viral: adenovirus, enterovirus, EBV, roseola• Immunological/Allergic – JRA (systemic onset) – Hypersensitivity reactions
Kawasaki disease - AHA diagnostic criteriaFever of ≥ 5 days duration + four of five criteria 1. 3. Oropharyngeal changes Bilateral non-purulent conjunctival injection (90%+ of cases) (90%+ of cases)2. 4. Changes in peripheral Polymorphous rash extremities (95%+ of cases) (90%+ of cases) 5. Cervical lymphadenopathy (~75% of cases)
Atypical or Incomplete Kawasaki Disease• Present with < 4 of 5 diagnostic criteria• Compatible laboratory findings• Still develop coronary artery aneurysms• No other explanation for the illness• More common in children < 1 year of age
Trager, J. D. N Engl J Med 333(21): 1391. 1995.
Coronary Arterial Changes• Vary in severity from echogenicity due to thickening and edema or asymptomatic coronary artery ectasia to giant aneurysms• May lead to myocardial infarction, sudden death, or ischemic heart disease
Coronary Aneurysms• Patients most likely to develop aneurysms – Younger than 6 months, older than 8 years – Males – Fevers persist for greater than 14 days – Persistently elevated ESR – Thrombocytosis – Pts who manifest s/s of cardiac involvement
Management Categories1.Pharmacologic therapy:• IVIG: 2g/kg as one-time dose• Aspirin – High dose (80-100 mg/kg/day) until afebrile x 48 hrs &/or decrease in acute phase reactants – Decrease to low dose (3-5 mg/kg/day) for 6-8 weeks or until platelet levels normalize2.Physical activity: decrease for 4-6wk3.Follow-up and diagnostic testing4.Invasive testing