key: cord-0689827-6clb3928 authors: Baba, Tomoaki; Maruyama, Tomoko; Katsuragi, Shinichi; Maeda, Kyohei; Kogaki, Shigetoyo title: Multisystem inflammatory syndrome associated with SARS‐CoV‐2 in a Japanese girl date: 2021-05-19 journal: Pediatr Int DOI: 10.1111/ped.14704 sha: 936a4a153dfa2bae6bbf110b7d40f29354beebea doc_id: 689827 cord_uid: 6clb3928 nan Since the start of the coronavirus disease (COVID-19) pandemic, COVID-19-related multisystem inflammatory syndrome in children (MIS-C) has been reported mainly in Europe and North America, 1,2 but there are a few reports from Asia. 3 Here we report a case of confirmed MIS-C with cardiac dysfunction in a Japanese girl. A case of COVID-19-related Kawasaki disease has been reported in a Japanese infant 4 but, as far as we are aware, this is the first case of MIS-C to be reported in Japan. A previously healthy, 10-year-old, Japanese girl was admitted to our hospital with a 3-day history of fever, abdominal pain, and frequent watery diarrhea in December, 2020 (Fig. 1a ). She had not had any contact with anyone symptomatic for COVID-19 within the few months preceding her admission. Her body temperature was 38.9°C; heart rate, 127/ min; respiratory rate, 24/min; blood pressure, 107/61 mmHg; and her oxygen saturation was 97% in room air. She had mild tenderness in the lower right quadrant of her abdomen. Abdominal ultrasonography revealed diffuse bowel wall thickening, predominantly in the terminal ileum and ascending colon (Fig. 1b) . Abdominal contrast-enhanced computed tomography showed enlarged mesenteric lymph nodes, thickening and increased brightness of the peritoneum, ascites, and bowel wall thickening (Fig. 1c) . Laboratory examination showed a mild increase in white blood cell count (10 900/µL) and an elevated C-reactive protein (CRP) level (21.9 mg/dL). We initially diagnosed her with enterocolitis and peritonitis, and treated her with intravenous antibiotics. All patients admitted to our hospital undergo screening for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) using a polymerase chain reaction (PCR) test. Her PCR test was positive on the day of admission. On receiving the result, we considered the possibility of concurrent infectious enterocolitis and asymptomatic SARS-CoV-2 infection. However, her high fever persisted, and she developed bilateral conjunctival injection on the third day of hospitalization. She also developed inflamed cracked lips, a strawberry tongue, tender bilateral cervical adenopathy, and small annular erythema on her left shoulder on hospital day 4. Laboratory examination showed a decrease in the platelet count (74 000/µL), elevated D-dimer levels (24.3 µg/mL), and an inflammatory reaction (CRP 19.20 mg/dL, ferritin 3,685 ng/mL). Chest radiography showed cardiomegaly (cardiothoracic ratio, 61%) and blunt costophrenic angles (Fig. 1d) . Echocardiography revealed reduced left ventricular contraction (ejection fraction, 47%), pericardial effusion, and dilatation of the left main coronary artery (4.6 mm, Z-score 3.88]) with perivascular brightness (Fig. 1e) . Her brain natriuretic peptide levels were elevated (487 pg/mL). Considering her negative blood and stool cultures and positive IgG and IgM antibody titers against SARS-CoV-2, we diagnosed her with COVID-19-associated MIS-C based on WHO and CDC diagnostic criteria of MIS-C on hospital day 4. We immediately administered 2 g/kg of intravenous immunoglobulin (IVIG) and started administering 30 mg/kg/day of oral aspirin and 2 mg/kg/day of intravenous prednisolone. She developed moderate anasarca with a weight gain of 4 kg and hypoalbuminemia (1.7 g/dL). She was treated with continuous infusion of furosemide (1 mg/kg/day) and albumin supplementation. In addition, heparin was administered to prevent thrombosis associated with MIS-C and steroid administration. Her fever and Kawasaki-like symptoms (annular erythema, enlarged, tender cervical lymph nodes, and red lips) disappeared promptly after administration of IVIG and prednisolone. Around this time, her abdominal pain resolved and the ascites and pleural effusion gradually decreased. Echocardiography also showed gradual improvement of the reduced left ventricular contraction and pericardial effusion, although mild dilatation of the left main coronary artery (3.9 mm, Zscore 2.54) persisted. The prednisolone dose was tapered from 2 to 1 to 0.5 mg/kg/day as her symptoms and CRP levels improved. She was discharged on hospital day 16 once her chest X-ray and ultrasonography findings became normal. After discharge, she developed non-membranous desquamation of the fingertips. This case serves as a reminder that MIS-C can present with a broad range of clinical symptoms, including fever and gastrointestinal symptoms. Early recognition of MIS-C and antiinflammatory treatment, including IVIG and corticosteroids, are important, as children with MIS-C can rapidly deteriorate and require more intensive care, such as the use of extra corporeal membrane oxygenation. 5 During the rapid expansion of the COVID-19 pandemic in Japan, all pediatricians need to keep MIS-C in the differential diagnosis, even in pediatric patients who do not initially fulfil the diagnostic criteria for MIS-C and whose presentations mimic a more common diagnosis. Hyperinflammatory shock in children during COVID-19 pandemic 2020 Health Alert #13: Pediatric Multi-System Inflammatory Syndrome Potentially Associated with COVID-19 Multisystem inflammatory syndrome in children related to COVID-19: the first case in Korea Kawasaki disease following coronavirus disease 2019 with prolonged fecal viral shedding Acute heart failure in multisystem inflammatory syndrome in children in the context of global SARS-CoV-2 pandemic We would like to thank Editage (www.editage.com) for English language editing. The authors declare no conflict of interest. Informed consent for publishing this case report was obtained from the patient's parents.