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Introduction - MIS-C is the most relevant complication of SARS-CoV-2 infection and has an incidence of 1 case / 2,200 children and adolescents with previous infection. The clinical picture is characterized by a multiorgan involvement and an insufficiently known response to therapy. Objectives - Retrospective analysis carried out in the Paediatric Department of the Ravenna Hospital from April 2020 to March 2022 of the cases admitted with MIS-C, with description of the presentation characteristics, the differences with respect to Kawasaki disease and the therapeutic strategies adopted also with regard to the clinical response. Results - Thirteen cases diagnosed with MIS-C with an average age of 6 years and four months were hospitalized, 4 cases were of Italian origin, 6 of Balkan and 3 of African. All the cases presented with fever> 39 ° C with severe malaise / irritability, associated with skin rash in 9 cases, conjunctivitis in 7 and cheilitis in 5. The most prominent manifestations of organ involvement were abdominal pain / diarrhoea (N = 9), myocardial dysfunction in the absence of coronary involvement (N = 5), presence of pulmonary thickening (N = 5), painful laterocervical lymphadenitis with reduced neck motility (N = 5), subnephrotic proteinuria (N = 7) and hypertransaminasaemia (N = 8). Two cases with “encephalitic-like” psychomotor slowing, one with important ascites and one with oedema of the para/retropharyngeal tissues were clinically relevant for the severity of the onset. Twelve cases were treated with IVIG and methylprednisolone (2 mg /kg), 4 of them were found to be non-responders and required the use of steroid boluses (4 cases) and anakinra (4 cases), in three of the latter in association with steroid boluses, with rapid and decisive clinical response. Conclusions - The serious clinical manifestations that characterize MIS-C do not concern only cardiac involvement. Therapy with an IL-1 inhibitor, anakinra, must also be envisaged as a possible first-line treatment in case of a particularly severe clinical picture at the onset.

Friedreich ataxia is the most common form of hereditary ataxia. Heart involvement in Friedreich ataxia is common and can include increased left ventricular wall thickness, atrial fibrillation, and in the later stages, a reduction of left ventricular ejection fraction. We present the case of a 45-year-old man with a history of paroxysmal atrial fibrillation and a congestive heart failure, hypertension, age ⩾ 75 years, diabetes mellitus, stroke, vascular disease, age 65–74 years, and female sex (CHA2DS2-VASc) score of only 1 (because of reduced left ventricular ejection fraction) who presented with pneumonia and was also found to have atrial fibrillation with a rapid ventricular response. Despite already being on long-term therapy with a non-vitamin K-antagonist oral anticoagulant, a transesophageal echocardiogram showed a mobile floating thrombus in the left atrial appendage. In accordance with previous necropsy evidence of thrombosis and thromboembolism in Friedreich ataxia subjects who likely have had only non-sex-related CHA2DS2-VASc score ⩽1, this case suggests that the risk of thromboembolism in Friedreich ataxia subjects with atrial fibrillation may not be adequately predicted by the sole CHA2DS2-VASc score.