Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown etiology that was first described in the Japanese literature in 1967 and has since been recognized as both endemic in the Americas and Europe and community-wide epidemic in Asia.1 Kawasaki disease has now become the leading cause of acquired heart disease in children in North America and Japan and is increasingly recognized as a worldwide problem.1 It occurs in young children, with a yearly incidence of 80 to 90 per 100,000 children younger than 5 years in Japan and 10 per 100,000 children younger than 5 years in the United States.2
Kawasaki disease is diagnosed using clinical criteria that include fever for 5 days or longer and at least 4 of the following: (1) nonexudative conjunctival injection; (2) oral involvement, including any of strawberry tongue, mucosal hyperemia, and cracked or erythematous lips; (3) changes in the peripheral extremities, including edema or desquamation in convalescence; (4) polymorphous rash; and (5) acute cervical adenopathy greater than 1.5 cm in diameter.3
There is no specific diagnostic test or pathognomonic clinical features; those previously mentioned clinical criteria have been established to assist physicians in diagnosing KD. So clinician should be aware of the possibility of KD. In this report, we would like to present a case of KD in an infant where the disease manifestation appeared as early as 40 days after birth. Our experience highlights the need to consider KD even in a newborn with a case of prolonged fever.
A 30-day-old female infant who was previously healthy developed a high-grade fever, which was followed 2 days later by maculopapular rash on her trunk and extremities. She also had reddish discoloration of her lips and conjunctival infection. She was admitted to another hospital with those complaints and hospitalized with a diagnosis of sepsis, and she was given intravenous antibiotic for 12 days but did not recover from any of the symptoms and her fever. Levels of serum acute phase reactants including erythrocyte sedimentation rate (80 mm/h) and C-reactive protein (5 mg/dL) were remarkably high; no bacterial growth was detected in any of her culture.
She was referred to us for the evaluation of prolonged fever. Upon examination, she had desquamation of the skin at her fingers starting from the fingertips and the anal region. The child was hospitalized with the diagnosis of KD based on her medical history and physical examination findings. Her peripheral leukocyte and thrombocyte counts were 10,200/mL and 680,000/mL, respectively. Her echocardiography revealed minimal aneurysmal dilatation of coronary arteries (Fig. 1). Intravenous immunoglobulin (2 g/kg) for 12 hours and high-dosage (100 mg/kg per day divided into 4 dose/d) acetylsalicylic acid treatment were applied. Her fever disappeared after the dose of intravenous immunoglobulin therapy, and acute phase reactants including erythrocyte sedimentation rate and C-reactive protein decreased at the end of the first week. The patient was discharged from the hospital with low-dosage (3 mg/kg per day) acetylsalicylic acid. The aneurysmal dilatation was persisting on her second month follow-up echocardiography.
Kawasaki disease occurs primarily in young children, with 80% of patients are under the age of 4 years and with the peak incidence occurring at 9 to 11 months of age.2 In the United States, median age was 2 years.1 The illness is extremely rare in infants under the age of 3 months.2 In one Japanese series, only 1.7% of patients were younger than 3 months.4 The youngest reported patient in the literature is a 2-week-old neonate5 In infants, the atypical presentations (longer duration of illness before diagnosis, lower incidence of conjunctivitis, lower incidence of rash, lower incidence of extremity change, and lower C-reactive protein) are common, and this may result in a delay in diagnosis and effective treatment.1,5 The illness is rare under the age of 3 months, but coronary artery involvement-more rapid and severe coronary artery disease-is also more common in them.2,5-7 Delayed diagnosis in KD was a significant risk factor for the development of coronary artery abnormalities.8 In addition, there may be as yet unknown physiological differences among KD patients at extremes of pediatrics that make them more vulnerable to coronary complication.
Kawasaki disease is rare in Turkey, with this patient being perhaps the youngest ever reported from Turkey.9 The presence of clinical manifestation and elevated acute phase reactants in association with typical echocardiographic coronary artery dilatation established the diagnosis. The diagnosis was not suspected at an earlier date possibly because of lack of awareness of neonatal occurrence of KD. Pannaraj et al10 reported that 71 of 124 general pediatricians in San Diego County did not consider the diagnosis of KD in children younger than 6 months; similarly, 86 of 324 pediatric infectious disease subspecialists did not consider KD in their differential diagnosis of children younger than 6 months.
In this study, we would like to call pediatricians' and neonatologists' attention to consider KD even in neonates and to follow a rapid and severe course.
1. Newburger JW, Takahashi M, Gerber MA, et al. Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics
2. Rowley AH. Kawasaki syndrome. In: Gershon AA, Hotes PJ, Katz SL, et al. Krugman's Infectious Diseases of Children
. 11th ed. Philadelphia, PA: Mosby Inc; 2004;323-335.
3. America Heart Association (1990). Diagnostic guidelines for Kawasaki disease. Am J Dis Child
4. Tsuchida S, Yamanaka T, Tsuchida R, et al. Epidemiology of infant Kawasaki disease with a report on the youngest neonatal case reported in Japan. Acta Paediatr
5. Stanley TV, Grimwood K. Classical Kawasaki disease in a neonate. Arch Dis Child Fetal Neonatal Ed
6. Brenner JL, Jadavji T, Pinto A, et al. Severe Kawasaki disease in infants: two fatal cases. Can J Cardiol
7. Zsadanyi J, Lakatos L, Herczeg L, et al. Fatal outcome of Kawasaki syndrome in a 4-week-old infant. Acta Paediatr
8. Palinkas LA, Wilder MS, Kao AS, et al. Social and cultural risk factors for coronary artery aneurysms following Kawasaki syndrome. Pediatr Res
9. Kanra G, Cengiz AB, Kara A, et al. Kawasaki Hastalığı: Dokuz Vakanın Takdimi. Çocuk Sağlığı ve Hastalıkları Dergisi
. 1999;42:469-478. (In Turkish).
10. Pannaraj PS, Turner CL, Bastian JF, et al. Failure to diagnose Kawasaki disease at the extremes of the pediatric age range. Pediatr Infect Dis J