Kawasaki disease (KD)
mucocutaneous lymph node syndrome and infantile polyarteritis nodosa, is an acute febrile illness of childhood .
KD is a vasculitis with a predilection for the
Approximately 20-25% of untreated children develop coronary artery abnormalities (CAA) including aneurysms.
<5% of children treated with intravenous gammaglobulin (IVIG) develop CAA.
KD is the leading cause of acquired heart
disease in children in most developed countries.
The cause of KD remains ((unknown))
but certain epidemiologic and clinical features support an infectious origin.
A genetic role in the pathogenesis of KD seems likely, as evidenced
by the higher risk of KD in Asian children.
linkage studies and genome-wide association studies have identified significant associations between polymorphisms in the ITPKC gene, a T-cell regulator, with increased susceptibility to KD and to more-severe disease.
immunoglobulin G (FCGR2A) have also been identified as significant variants in KD patients.
KD is a vasculitis affects medium-size arteries.
The coronary arteries are the most commonly involved,also popliteal and brachial arteries.
((A 3-phase process ))to the arteriopathy of KD.
The 1st phase is in the 1st 2 wk of illness begins in the endothelium and moves
through the coronary wall.
Saccular aneurysms may form from this arteritis.
The 2nd phase is a subacute/chronic vasculitis may last weeks to years .
results in fusiform aneurysms.
The vessels affected by the subacute/chronic vasculitis cause progressive stenosis.
Thrombi may form in the lumen and obstruct blood flow.
Fever is characteristically
high (≥38.3°C [101°F]),
unresponsive to antibiotics.
The duration of fever without treatment is generally 1-2 wk, but may persist for 3-4 wk.
the 5 principal clinical criteria of KD are:
bilateral nonexudative conjunctival injection with limbal sparing
erythema of the oral and pharyngeal mucosa with strawberry tongue and red, cracked lips;
edema and erythema of the hands and feet; rash of various forms (maculo-papular, erythema multiforme, or scarlatiniform
nonsuppurative cervical lymphadenopathy, usually unilateral, with node size >1.5 cm
(Table 166-1; Figs. 166-1 to 166-4).
Perineal desquamation is common in the acute phase.
Periungual desquamation of the fingers and toes begins(( 2-3 wk ))after the onset of illness .
Gastrointestinal symptoms (vomiting, diarrhea, or abdominal pain) occur in more than 60% of patients, and at least 1 respiratory symptom (rhinorrhea or cough) occurs in 35%.
* significant irritability
* urethritis and meatitis with sterile pyuria.
Small or large joints may be affected, and the arthralgias
KD can be divided into 3 clinical phases:
The acute febrile phase is
fever + acute signs of illness and usually
lasts 1-2 wk.
The subacute phase 🤷
the development of CAA, the highest risk of sudden death from aneurysms
lasts about 3 wk.
The convalescent phase
begins when all clinical signs have disappeared and continues until (ESR) returns to normal.
typically about ((6-8 )) Wk after the onset of illness.