Abstract
Kawasaki disease (KD) is a febrile vasculitis, which is commonly defined by fever and at least four specific clinical symptoms. Incomplete KD is defined by suggestive echocardiographic findings with an incomplete clinical picture. Refractory KD is diagnosed in patients resistant to intravenous immunoglobulin (IVIG). We report the case of a 6-month-old male infant admitted to our clinic for persistent fever and onset of a generalized polymorphous rash, accompanied by high fever, rhinorrhea, and cough for the past 7 days. The laboratory tests, on the day of admission, revealed leukocytosis with neutrophilia, anemia, thrombocytosis, hypernatremia, hypoalbuminemia, elevated C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR). Echocardiography showed dilation of the left anterior descending coronary artery (LAD). Based on all these findings, we established the diagnosis of KD, and we initiated IVIG and intravenous pulsed methylprednisolone, with an initial favorable outcome. However, the symptoms reappeared, and we administered a second higher single dose of IVIG, but without any clinical improvement. Moreover, the laboratory parameters and echocardiographic findings worsened. We reinitiated a longer course of intravenous methylprednisolone in a smaller dose, which had a favorable impact on the clinical, laboratory, and echocardiographic parameters. Multiple uncertainties exist related to the management of refractory KD despite the wide spectrum of therapeutic options that have been proposed. Our case demonstrates that in patients refractory to aggressive initial therapy, low or moderate doses of steroid given daily may be helpful.
Highlights
Kawasaki disease (KD) or mucocutaneous lymph node syndrome was named after Tomisaku Kawasaki, a Japanese pediatrician who described this febrile vasculitis for the first time in 1967 [1]
The complications can be widely diverse during the acute phase, including valvulitis, myocarditis, pericarditis, or KD shock syndrome, the occurrence of coronary artery aneurysms (CAAs) remains the most important, usually appearing later, in the subacute to convalescent phase [13]
The laboratory tests on the day of admission revealed leukocytosis (34,590/μl) with neutrophilia (28,000/μl), anemia (Hb: 7.5 g/dl, Htc: 23.5%, medium erythrocyte volume (MEV): 73 fl, medium erythrocyte hemoglobin (MEH): 23.3 pg), thrombocytosis (648,000/μl), hypernatremia (154.1 mmol/l), hypoalbuminemia (2.48 g/dl), elevated C-reactive protein (CRP) (311.33 mg/l), and erythrocyte sedimentation rate (ESR) (65 mm/h)
Summary
Kawasaki disease (KD) or mucocutaneous lymph node syndrome was named after Tomisaku Kawasaki, a Japanese pediatrician who described this febrile vasculitis for the first time in 1967 [1]. As for European countries, the data from the United Kingdom reported an annual incidence of 8.4/100,000 in children below 5 years of age whereas, in Denmark and the Netherlands, the incidence is smaller, ∼4–5 cases in 100,000 children under the same critical age [8] It is well-known that KD is triggered by an infectious agent, its pathogenesis remains unclear. Factors that predict refractory KD should be identified to administer an early and appropriate treatment The aim of this case report is to underline the challenges associated with the management of refractory KD diagnosed in a young infant.
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