Kawasaki disease

Question 1

untreated patients develop cardiac problems as

Answer 1

aneurysms, with the potential for the development of coronary thrombosis, stenosis, myocardial infarction, and sudden death

Question 2

ETIOLOGY

Answer 2

features strongly support an infectious origin;
age group affected,
periodic epidemics with a wavelike geographic spread of illness during the epidemic
the self-limited nature of the illness, and the
clinical features of fever, rash, enanthem, conjunctival injection, and cervical adenopathy.
genetic causes of the disease become likely

Question 3

usually patient age is

Answer 3

less than 5 years

Question 4

CLINICAL MANIFESTATIONS

Answer 4

The acute stage begins with the onset of fever and lasts 1 to 2 weeks. This is followed by the subacute stage, which includes desquamation of the extremities and the development of coronary aneurysms. The third stage is convalescence, during which the findings resolve
Persistant fever more than 5 days
Plus at least 4 principal features
bilateral bulbar conjunctival injection, usually without exudate.
erythema of the oral and pharyngeal mucosa.
Change in lips and oral cavity :”strawberry” tongue, dry, cracked lips.
Change in extrimties: erythema and swelling of the hands and feet and peeling .
Polymorphous exanthem (maculopapular, erythema multiforme, or scarlatiniform) with accentuation in the groin area.
and nonsuppurative cervical lymphadenopathy, usually unilateral, with a node size of 1.5 cm or greater in diameter.

Question 5

fever characteristics

Answer 5

high spiking (to 104°F or higher),
remittent, and unresponsive to antibiotics.
duration of fever is generally 1-2 wk without treatment but may persist for 3-4 wk.
Prolonged fever has been shown to be a risk factor for the development of coronary artery disease

Question 6

Cardiac involvement

Answer 6

Cardiac involvement is the most important manifestation of Kawasaki disease.
Myocarditis manifested by tachycardia,,and decreased ejection fraction and decreased ventricular function occurs in at least 50% of patients.
Pericarditis with a small pericardial effusion is common during the acute illness.
Coronary artery aneurysms generally develop during the 2nd-3rd wk of illness and can be detected by two-dimensional echocardiography. Valvular regurgitation and systemic artery aneurysms may occur but are uncommon. Giant coronary artery aneurysms (8mm internal diameter) pose the greatest risk for rupture, thrombosis or stenosis, and myocardial infarction.

Question 7

SUBACUTE PHASE

Answer 7

when fever and other acute signs have abated, but irritability, anorexia, and conjunctival injection may persist. is associated with
Desquamation.
Thrombocytosis.
the development of coronary aneurysms.
and the highest risk of sudden death. This phase generally lasts until about the 4th wk.

Question 8

LABORATORY FINDINGS

Answer 8

No specific diagnostic test
An elevated ESR, C-reactive protein, and other acute phase reactants are almost universally present in the acute phase of illness and may persist for 4-6 wk.
Normocytic anemia is common.
The platelet count is generally normal in the 1st wk of illness and rapidly rises by the 2nd-3rd wk of illness and may exceed 1,000,000/mm3 .
Antinuclear antibody and rheumatoid factor are not detectable. Sterile pyuria, mild elevations of the hepatic transaminases, and cerebrospinal fluid pleocytosis may be present.
Low albumin, hyponatremia,thrombocytosi after one week,high GGT and transaminases

Question 9

the most useful test to monitor the potential development of coronary artery abnormalities and when

Answer 9

ECHOCARDIOGRAPHY
The test should be performed at diagnosis and again after 2-3 wk of illness.
If results of both of these are normal, a repeat study is performed 6-8 wk after onset of illness.

Question 10

Predictor of poor prognosis

Answer 10

Young age ,,male gender
Persistant fever and poor response to ttx
Labs abnormality

Question 11

D DX

Answer 11

MEASELES
Drug Rxn
Toxic shock syndrome
scarlet fever

Question 12

how differentiate from MEASELES

Answer 12

exudative conjunctivitis.
Koplik spots.
rash that begins on the face behind the ears.
and a low white blood cell count and ESR.

Question 13

how differentiate from drug reaction

Answer 13

periorbital edema
oral lesions
low ESR, may help to distinguish these reactions from Kawasaki disease.

Question 14

how differentiate from toxic shock

Answer 14

hypotension
renal involvement
elevated creatine phosphokinase
focus of staphylococcal infection. which are features of this illness but not of Kawasaki disease

Question 15

how differentiate from scarlet fever

Answer 15

A common clinical problem is the differentiation of from Kawasaki disease in a child who is a group A streptococcal carrier. Because patients with scarlet fever have a rapid clinical response to penicillin therapy, treatment with this therapy for 24-48 hr with clinical reassessment at that time generally clarifies the diagnosis.

Question 16

Treatment of Kawasaki Disease

Answer 16

Acute Stage

Intravenous immune globulin 2 g/kg over 10-12 hr .

aspirin 80-100 mg/ kg/24 hr divided every 6 hr orally until 14th illness day.
Convalescent Stage

Aspirin 3-5 mg/kg once daily orally until 6-8 wk after illness onset
Long-Term Therapy for Those with Coronary Abnormalities
Aspirin 3-5 mg/kg once daily, orally
± dipyridamole 4-6 mg/kg/24 hr divided in two or three doses orally (some experts add warfarin for those patients at particularly high risk of thrombosis.