Multisystem Inflammatory Syndrome in Children Related to COVID-19: the First Case in Korea

Abstract

Since mid-April 2020, cases of multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 that mimics Kawasaki disease (KD) have been reported in Europe and North America. However, no cases have been reported in Korea. We describe an 11-year old boy with fever, abdominal pain, and diarrhea who developed hypotension requiring inotropes in intensive care unit. His blood test revealed elevated inflammatory markers, thrombocytopenia, hypoalbuminemia, and coagulopathy. Afterward, he developed signs of KD such as conjunctival injection, strawberry tongue, cracked lip, and coronary artery dilatation, and parenchymal consolidation without respiratory symptoms. Microbiological tests were all negative including severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) reverse transcription polymerase chain reaction. However, serum immunoglobulin G against SARS-CoV-2 was positive in repeated tests using enzyme-linked immunosorbent assay and fluorescent immunoassay. He was recovered well after intravenous immunoglobulin administration and discharged without complication on hospital day 13. We report the first Korean child who met all the criteria of MIS-C with features of incomplete KD or KD shock syndrome.

Keywords: COVID-19; Intravenous Immunoglobulin; Kawasaki Disease; Kawasaki Disease Shock Syndrome; Multisystem Inflammatory Syndrome in Children.

Fig. 1. Abdomen and chest CT, bowel ultrasonography and simple CXR. (A) Abdominal CT finding on the emergency room visit showed enlarged lymph nodes (arrow, maximum length; 2.7 cm) with diffuse bowel wall thickening. (B) Cardiomegaly were shown on CXR on hospital day 4. (C) CT finding demonstrated cardiomegaly and pleural effusion with lung parenchymal consolidation on hospital day 4. (D) On hospital day 13 (last day of hospitalization), the enlarged lymph nodes had decreased to 0.89 cm on bowel ultrasonography.

CT = computed tomography, CXR = chest X-ray.

Fig. 2. Clinical features consistent with Kawasaki disease. (A, B) The cracked lip and strawberry tongue newly appeared on hospital day 6. (C, D) Desquamation of the perianal area and the wrist were observed on the patient's last day of hospitalization.

Fig. 3. Echocardiography findings. (A) The LMCA (4.3 mm [Z-score^a 1.64]) and the LAD (3.8 mm [Z-score 2.23]) were not tapered. (B) The RCA (4.1 mm [Z-score 2.62]) was dilated and aneurysmal change was suspected on hospital day 6. (C) On hospital day 13, the size of the LMCA (3.9 mm [Z-score 1.04]) and LAD (2.9 mm [Z-score 0.38]) had decreased. (D) The RCA size had decreased dramatically (3.1 mm [Z-score 0.70]) on the last day of patient's hospitalization.

LMCA = left main coronary artery, LAD = left anterior descending coronary artery, RCA = right coronary artery, CAL = coronary artery length. ^aThe definition of CAL has been modified, with CAL defined by a Z-score ≥ 2.5, corrected for body surface.