This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

No abstract

Background: In the absence of evidence-based therapies for Multisystem Inflammatory Syndrome in Children (MIS-C), we aimed to describe the similarities and differences in the evaluation and treatment of MIS-C at hospitals in the United States. Methods: We conducted a cross-sectional survey from June 16 to July 16, 2020 of U.S. pediatric hospitals regarding protocols for patients with MIS-C. Elements included hospital characteristics, clinical definition of MIS-C, evaluation, treatment, and follow-up. We summarized key findings and compared results from centers that had treated >5 patients vs. those that had treated <5 patients. Results: Forty centers of varying size and experience with MIS-C participated. About half (21/40) of centers required only 1 day of fever for MIS-C to be considered. In the evaluation of patients, there was often a tiered approach. Intravenous immunoglobulin was the most widely used medication to treat MIS-C (98% of centers). Corticosteroids were listed in 93% of protocols for primarily the moderate or severe cases. Aspirin was commonly used including for mild cases, whereas heparin or low molecular weight heparin were used primarily in severe cases. In severe cases, anakinra and vasopressors were frequently recommended. Nearly all centers (39/40) recommended follow up with cardiology. There were similar findings between centers that had treated >5 patients vs. those that had treated <5 patients. A supplement containing hospital protocols is provided. Conclusion: There are many similarities yet some key differences between hospital protocols for MIS-C. These findings can help healthcare providers learn from others regarding options for managing MIS-C patients.

No abstract

An 8-year-old girl with neurofibromatosis type 1 and mild intermittent asthma presented with an 8-day history of a daily fever between 101°F and 105°F, a nonproductive cough, congestion, a sore throat, and myalgias, despite treatment with amoxicillin for bronchitis and cefazolin for a Staphylococcus epidermidis urinary tract infection. She presented on day 8 of her illness with a macular hand rash, flank pain, and strawberry tongue and was admitted for evaluation of a fever of unknown origin. She did not have any weight loss or joint pain. Admission laboratory values were notable for a white blood cell count of 26 800 cells/mm 3 with 15% bands and 74% segmented neutrophils, an erythrocyte sedimentation rate of 52 mm per hour, C-reactive protein levels of 21.5 mg/dL, ferritin levels of 1129 ng/mL, normal results from liver function tests, a respiratory virus film array with results positive for sapovirus, and sterile blood and urine cultures. She was started on empirical ceftriaxone coverage with no improvement in symptoms. Computed tomography of the head, chest, abdomen, and pelvis revealed cervical, axillary, mediastinal, mesenteric, and inguinal lymphadenopathy. A transthoracic echocardiogram showed no evidence of intracardiac vegetations but revealed a small pericardial effusion. She remained hospitalized with almost daily fevers despite antibiotics but was otherwise hemodynamically stable. On hospital day 9, the patient developed tachycardia (130-140 beats per minute), respiratory distress (tachypnea to 62 breaths per minute with accessory respiratory muscle use), hypoxemia (pulse oxygen saturation of 89%), and hypotension (77/42 mm Hg). On examination, she had nasal flaring and subcostal retractions; her lungs were clear to auscultation. She had no visible jugular venous distention (JVD), hepatomegaly, or lower extremity edema, and her heart sounds were not muffled. A chest radiograph (CXR) revealed a newly enlarged cardiac silhouette and clear pulmonary fields (Fig 1). An electrocardiogram (ECG) showed sinus tachycardia with normal axis, low voltages, and T-wave inversions in the lateral precordial leads. A repeat echocardiogram revealed a 2.2 cm circumferential pericardial effusion with right atrial collapse in diastole, increased atrioventricular valve inflow respiratory variation, and inferior vena cava plethora consistent with tamponade physiology (Fig 2).