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69. Kawasaki Disease in Children
Authors:
MICHEAL RAJ MIKITHA,
POOTHALINGAM JEYASANTHI DHARANYA,
KINGSLY FELIX SUTHA ADLIN KIRASHITHA,
SONKAR MANYA,
TEKAM GAURI DIWAKAR,
ALIMOVA NURGUL ABDYASHYMOVNA
(1,2,3,4,5, Student , International Medical Faculty, Osh State University , Osh, Kyrgyzstan
6, Teacher, International Medical Faculty, Osh State University , Osh, Kyrgyzstan)
Abstract
Kawasaki disease (KD), also called mucocutaneous lymph node syndrome, is an acute, selflimiting systemic vasculitis that primarily affects mediumsized arteries, particularly the coronary arteries. It predominantly affects infants and young children and is a leading cause of acquired heart disease in children in developed countries.
Epidemiology
Age: Most common in children below 5 years, especially between 6 months and 4 years. Sex: More common in boys (maletofemale ratio 1.5:1).
Geographical distribution: Highest incidence in Japan, followed by South Korea, Taiwan, and China. Seasonality: Commonly occurs in winter and early spring.
Incidence:
Japan: ~300 per 100,000 children <5 years
North America/Europe: 8–25 per 100,000 children <5 years
India: Increasing number of diagnosed cases over the last two decades.
Mortality: <0.5% with early diagnosis and treatment.
Etiology and Pathogenesis
Although the exact cause remains unknown, Kawasaki disease is believed to be a result of genetic predisposition, environmental or infectious triggers, and immunemediated vascular inflammation.
1. Infectious Triggers
Suspected viral (adenovirus, coronavirus, parvovirus B19) and bacterial agents (Staphylococcus aureus, Streptococcus pyogenes).
However, no single pathogen has been consistently identified.
2. Genetic Susceptibility
Familial clustering and higher rates among siblings and identical twins.
Gene polymorphisms in ITPKC, CASP3, FCGR2A, and other immuneregulatory genes increase susceptibility.
3. Immunopathogenesis
Hyperactivation of T cells, monocytes, and cytokines (IL1, IL6, TNF, IL17).
This leads to endothelial cell injury, causing vasculitis of mediumsized arteries — particularly coronary arteries. Formation of coronary artery aneurysms results from ongoing inflammation and vessel wall weakening.
Clinical Features
The disease progresses through three overlapping phases:
1. Acute Febrile Phase (1–2 weeks)
Highgrade fever lasting 5 days, unresponsive to antibiotics. Bilateral nonexudative conjunctivitis.
Oral mucosal changes: red cracked lips, strawberry tongue, erythema of oropharynx. Polymorphous rash (maculopapular, urticarial, or scarlatiniform).
Peripheral extremity changes: erythema, edema of hands and feet. Cervical lymphadenopathy (>1.5 cm, usually unilateral).
2. Subacute Phase (2–4 weeks) Resolution of fever but persistent irritability.
Desquamation (peeling) of fingers and toes. Thrombocytosis develops.
Coronary artery aneurysms may appear.
3. Convalescent Phase (4–8 weeks)
Gradual disappearance of all clinical symptoms. Inflammatory markers return to normal.
Diagnostic Criteria (AHA) Complete Kawasaki Disease
Fever for 5 days plus four or more of the following:
1. Bilateral nonexudative conjunctivitis
2. Oral mucosal changes (cracked lips, strawberry tongue)
3. Peripheral extremity changes (erythema, edema, or desquamation)
4. Polymorphous rash
5. Cervical lymphadenopathy (>1.5 cm)
Incomplete (Atypical) Kawasaki Disease
Fever for 5 days plus 2–3 criteria, supported by laboratory findings ( ESR/CRP) or echocardiographic evidence of coronary involvement.
Laboratory Findings Leukocytosis with left shift Elevated ESR and CRP
Thrombocytosis (subacute phase) Normocytic normochromic anemia Mildly elevated liver enzymes Sterile pyuria
Echocardiography: Coronary dilatation, aneurysm, pericardial effusion, or valvular regurgitation.
Biomarkers for Diagnosis of Kawasaki Disease
Although the disease has been known for decades, diagnosis remains primarily clinical as no specific biomarkers have been established.
Recent studies have identified potential markers:
Elevated NTproBNP (Nterminal probrain natriuretic peptide) Increased IL17 levels
These may help differentiate incomplete Kawasaki disease from other infectious illnesses in children and serve as adjunct tools for diagnosis in uncertain cases.
Differential Diagnosis
Condition Key Differentiating Features
Scarlet Fever Exudative pharyngitis, positive throat culture, response to antibiotics Measles Presence of Koplik spots, characteristic rash pattern
Stevens–Johnson Syndrome Bullous mucosal lesions, drug history
Toxic Shock Syndrome Hypotension, multiorgan dysfunction, positive staphylococcal culture Viral Exanthems Milder symptoms, lack of mucosal and limb involvement
Complications Cardiac
Coronary artery aneurysm Myocarditis
Pericarditis
Valvular insufficiency
Myocardial infarction (rare but severe)
Other Complications Arthritis or arthralgia Hepatobiliary dysfunction
Aseptic meningitis
Sensorineural hearing loss (rare)
Treatment
1. Acute Phase
Goal: Reduce inflammation and prevent coronary aneurysm formation. Intravenous Immunoglobulin (IVIG):
2 g/kg single dose over 10–12 hours (administered within 10 days of illness).
➤ Significant decrease in new coronary artery abnormalities and shorter duration of fever. Aspirin:
High dose: 30–50 mg/kg/day (divided every 6 hours) during the acute phase. Low dose: 3–5 mg/kg/day for 6–8 weeks (antiplatelet effect).
Corticosteroids:
For IVIGresistant cases or severe systemic inflammation (e.g., methylprednisolone pulse).
Refractory Kawasaki Disease:
May require a second IVIG dose or infliximab (antiTNF agent).
Medical FollowUp
Echocardiography: at diagnosis, 2 weeks, and 6–8 weeks after onset. Without coronary involvement: Discharge after 6–8 weeks.
With aneurysms: Longterm cardiology followup with anticoagulation (warfarin, lowmolecularweight heparin). Exercise: Avoid strenuous activity if coronary abnormalities persist.
Vaccinations: Defer live vaccines (MMR, varicella) for 11 months after IVIG administration.
Kawasaki Disease in India
Incidence: Data from multiple Asian countries, including India, indicate a steady increase in cases over the last 20 years.
Clinical awareness: Physicians should consider KD proactively in all children (especially infants) with fever >5 days.
Public health note: A large proportion of cardiac morbidity due to KD is preventable through early recognition and prompt treatment.
Prognosis
With timely IVIG therapy, complete recovery occurs in the majority of patients. Coronary artery aneurysm formation is the main determinant of longterm outcome. Overall mortality is less than 0.5%.
References
1. McCrindle BW et al., AHA Guidelines for Diagnosis, Treatment, and LongTerm Management of Kawasaki Disease, Circulation, 2017;135:e927–e999.
2. Rowley AH, Shulman ST. Kawasaki Disease: Clinical Features and Pathogenesis. Lancet, 2018.
3. Singh S et al., Kawasaki Disease in India: Current Trends and Challenges. Indian J Pediatr, 2020.
