IQ& 1 .Al QUESTIONS AND ANSWERS FOR CLINICAL INSIGHTS

(See page 114 for data.1

1. On the basis of the h&tory and physical examination what diqnosis do yoyou suspect? The Centers for Disease Control case definition for Ka- wasaki disease includes fever lasting 5 or more days without other more reasonable explanation and at least four of the following criteria:

a. Bilateral conjunctival injection (nonexudative) h. At least one of the following mucous membrane

changes: injected or fissured lips, injected phar- ynx, or “strawberry tongue”

c At least one of these extremity changes: erythema of palms or soles, edema of the hands or feet, or

generalized or periungual desquamation rash d. Cervical lymphadenopathy (at least one lymph

node 1.5 cm or greater in diameter) Associated signs include extreme irritability, an-

orexia, lethargy, pyuria and urethritis, arthralgia and ar- thritis, mcningeal signs, abdominal pain, and diarrhea i Larke, 1992).

Laboratory findings are generally too variable or ap- pear too late to have diagnostic value. The crythrocytc sedimentation rate may be very high. Thrombocytosis is a consistent feature that starts about day 10, peaks be- tween davs 15 and 25, and returns to normal bv 1 month i Larke, i992).

Clinical findings and pathologic changes are catego- rized into three phases: acute (lasts about 10 days), sub- acute (between davs 10 and 2S), and convalescent (from day 25 to 2 mon&s or longer).

With regard to J.P., it is of interest to note that it was necessary to see her over 2 days tv make the diagnosis as the physical signs developed. 2. What is the differential diaposti? It includes measles, Group A streptococcal disease, staph scalded skin syndrome, various viral illnesses, and less commonly drug reaction and juvenile rheumatoid ar- thritis. Exudative pharyngitis, exudative conjunctivitis, discrete intraoral lesions, splenomegaly, and generalized adenopathy are uncommon in Kawasaki disease, and their presence should raise the possibility of other dis- orders (Rowe, 1992). 3. What is the treatment plan? Intravenous gamma globulin (IVGG) administered within the first 10 davs of illness reduces the incidence of coronary artery dilation to less than 3% (Rowe, 1992), thereby emphasizing the importance of early di- agnosis in ensuring a good prognosis. Maximal cost- eff‘ective IVGG regimen is a single dose of 2 gm/kg

infused over approximately 10 hours. Single-dose thcr- apy decreases the need for hospitalization beyond 24 hours in most cases. Alternative IVGG therapy is 400 mg/ kg daily on 4 consecutive days; this regimen is rec- ommended if there are concerns about fluid volume in patients with congestive heart failure (Larke, 1992).

Aspirin therapy is also recommended for fever man- agement and for its antithrombotic effect. During the acute phase the recommended dosage is 80 to 100 mg J kg per day divided in four doses. After the fever has resolved and the platelet count begins to rise, dosage should be reduced to 3 to 5 mg/kg daily in a single dose and continue for 1 to 2 months or indefinitely if coronary artery aneurysms are noted (Larke, 1992).

Echocardiography is recommended at about day 7 to detect pcricarditis and after 3 to 4 weeks to detect an- eurysms and should be repeated every 8 weeks until nor- mal or indefinitely if aneurysms are detected. Obviously, children with coronary artery dilation should be man- aged in close consultation with a pediatric cardiologist.

J.P. was hospitalized for 4 days of IVGG therapy and stayed on aspirin therapy for 3 months. Two weeks after the onset of her symptoms, she was afebrile although her hands remained sore and she had an abnormal gait. Platelet count was greater than l,OOO,OOO. Echocardi- ography revealed mild left ventricular enlargement but no significant coronary artery dilation. Two months af- ter onset of symptoms she was completely asymptom- atic; her platelet count was 505,000, and echocardiog- raphy results were unchanged. 4. What is the prognosis? Kawasaki disease has a 1% mortality, largely as a result of massive myocardial infarction and cardiogenic shock. With the advent of IVGG therapy that is initiated promptly, morbidity has been significantly reduced. The incidence of coronary artery dilation is less than 3% if treatment is started within the first 10 days of illness (Rowe, 1992).

Kawasaki disease is typically a self-limited illness without complications. If coronary artery aneurysms do develop, these spontaneously resolve in over 50% of cases (Pichichero, 1987). Prompt diagnosis, appropriate and prompt treatment, and cardiac evaluation and fol- low-up can assure an excellent prognosis for this illness.

One year after the diagnosis of Kawasaki disease, J.P. attends preschool regularly, with no apparent sequelae.

REFERENCES Larks, B. (1992). Notes from “Update on Kawasaki disease.” Pre-

sented at The American Academy of Pediatrics Meeting, Pediatrics for the Practitioner, Seattle, WA.

Pichichero, M. (1987). Kawasaki disease. In R. Hoekelman (ed.), Primay pediatric care. St. Louis: The C.V. Mosby Company.

Rowe, I’. (1992). Notes from “Kawasaki disease”-pediatrics for the practitioner. Presented at The Johns Hopkins Hospital, Baltimore. MI).