Ten-month follow-up of patients with covid-19 temporally related multi-system inflammatory syndrome in children: the experience of the children hospital of Palermo

Background: In Sicily, the first wave of COVID-19 showed a low epidemic impact in paediatric population, while the second and the third waves had a higher impact on clinical presentation of COVID-19 in children and a significantly higher severe outcome in patients with multisystem inflammatory syndrome in children (MIS-C), with a frequent life-threatening progression. Methods: We describe a cohort of 22 Sicilian children (11 M; 11 F; age: 1.4-14 years), presenting with clinical features compatible with MIS-C. Patients with negative swab had a history of recent personal or parental infection. Results: The following diagnostic criteria were detected: fever (100%); cheilitis and/or pharyngeal hyperaemia (86%); latero-cervical lymphadenitis (82%); rash (73%); abdominal pain and/or vomiting and/or diarrhoea (64%); conjunctivitis (64%); hands and feet oedema (18%). 59% showed cardiac involvement (6 pericardial effusion; 8 mitral valve insufficiency; 4 insufficiency of two valves; 3 coronary artery lesions (CAL)). In all the patients, treatment was started within 72 h after the admission, with intravenous immunoglobulins (IVIG) (2 g/Kg/dose), methylprednisolone (2 mg/Kg/day in 73% of patients; 30 mg/Kg/day for 3 days, followed by 2 mg/Kg/day in 27% of patients). Two patients were treated with enoxaparin. Two patients with shock, were additionally treated with vasoactive drugs, albumin, diuretics. Cardiac involvement evolved into the complete resolution of lesions in most of the patients. All the patients were included in a follow-up, to investigate on clinical outcome and resolution of organ involvement. Cardiac valve insufficiency persisted only in 18% of children, CAL persisted only in 33% of children with coronary involvement, however without the evolution into aneurisms. Conclusions: The preferred treatment strategy was more aggressive at the diagnosis of MIS-C, to block the cytokine cascade. Most of our patients, in fact, received a first-line treatment with IVIG and steroids. This approach could explain the favourable prognosis, the rapid restoring of cardiac function also in patients with MAS or shock, and the good outcome during the 10 months follow-up in all the patients

Assessment of different manufacturing techniques for the production of bioartificial scaffolds as soft organ transplant substitutes

Introduction: The problem of organs’ shortage for transplantation is widely known: different manufacturing techniques such as Solvent casting, Electrospinning and 3D Printing were considered to produce bioartificial scaffolds for tissue engineering purposes and possible transplantation substitutes. The advantages of manufacturing techniques’ combination to develop hybrid scaffolds with increased performing properties was also evaluated.Methods: Scaffolds were produced using poly-L-lactide-co-caprolactone (PLA-PCL) copolymer and characterized for their morphological, biological, and mechanical features.Results: Hybrid scaffolds showed the best properties in terms of viability (>100%) and cell adhesion. Furthermore, their mechanical properties were found to be comparable with the reference values for soft tissues (range 1–10 MPa).Discussion: The created hybrid scaffolds pave the way for the future development of more complex systems capable of supporting, from a morphological, mechanical, and biological standpoint, the physiological needs of the tissues/organs to be transplanted

PARVOVIRUS INFECTION AND KAWASAKI DISEASE: ONE DISEASE FOR TWO SIBLINGS

Introduction: Kawasaki disease (KD) is rarely described in siblings in the same time. In these cases, an infectious trigger must be excluded. Objectives: We describe the clinical course of two brothers who showed severe KD all at once, secondary to Parvovirus infection. Methods: A 9-month-old female showed fever, pallor, vomiting, bilateral non-secreting conjunctivitis, rash. Anamnesis revealed that 12 days before, she had fever, spontaneously resolved. At admission, 9 days after fever onset, she showed fever, conjunctivitis, pharyngitis, rash, and cervical adenopathy. Haematological parameters showed: leukocytosis, neutrophilia; anaemia; CRP: 2.31; ESR: 120. ECG and echocardiography were normal, including coronary Z-scores. She showed positive Parvovirus IgM. Spontaneous defervescence occurred. Further cardiological evaluation was performed to exclude a pericarditis secondary to Parvovirus, and at day 26 after fever onset, coronary artery lesions (CAL) were documented: proximal right coronary artery Z-score of 6.02; left main coronary Z-score: 5.72; left anterior descending Z-score: 5.78. The child was promptly treated with IVIG plus ASA. A further echocardiographic evaluation showed worsening of CAL, with a sacciform aneurysm in the left anterior descending artery (Zscore: 5.08). Laboratory test did not show inflammation; however, the girl was treated with 3 bolus doses of intravenous methylprednisolone (30 mg/kg/dose). The Z-score of CAL did not change and the patient was treated with anakinra (4 mg/kg/day), with a progressive improvement of CAL, and after 2 months, Z-scores normalized. The 7-year-old brother presented fever, vomiting at the same time of the sister, with spontaneous resolution after 4 days. Four days later, he presented again fever with abdominal pain, tachypnoea and tachycardia, secondary anuria. He had: leukocytosis, neutrophilia, anemia; CRP: 0.24; CPK: 773; creatinine: 0.77; BUN: 111; elevated myocardial necrotic enzymes (c-Troponin T: 91.4; Pro-BNP: > 70.000). Echocardiogram showed generalized hypokinesia, a severe reduction of the ejection fraction (EF) (20-25%); increased left atrium (Z-score: 3.3) and mitral valve with moderate insufficiency. He received dopamine, dobutamine, furosemide plus steroids. He showed a constant improvement of echocardiographic parameters, plasmatic enzymes and clinical signs. In 16th day he was discharged with an EF of 45% and persistent septal hypokinesia. However, specific serology anti-Parvovirus was tested and showed increased IgM, with negative IgG. The cardiological outcome revealed a progressive improvement of EF, which reached the 50%. Results: CAL significantly improved after anakinra, at the contrary, the clinical evolution in the brother was different. Conclusion: We describe familial KD in two siblings which had the same infectious trigger (Parvovirus). The brother was diagnosed as a post-viral myocarditis. However, considering the two parallel and different evolution, the girl showed late CAL with aneurisms, and the brother a Kawasaki shock syndrome picture with myocardial dysfunction. Viral illnesses are recognised trigger of KD, and in these cases the rareness is the coincident KD in two siblings, with different and severe clinical course. Noteworthy, the girl had aneurisms which resolved with anakinra, a therapy which has been recently shown to be promising for this disease. Informed consent to publish had been obtained from the parents

[Heart involvement in multisystem inflammatory syndrome in children correlated with SARS-CoV-2 infection: a review by ANMCO/SICP]

Acute clinical manifestations of COVID-19 are generally less severe in childhood, however a proportion of them can develop a severe systemic hyperinflammatory syndrome after SARS-CoV-2 infection, known as the multisystem inflammatory syndrome (multisystem inflammatory syndrome in children, MIS-C). Car-diovascular manifestations in MIS-C are frequent (34-82%), including myocardial dysfunction, coronary artery dilation or aneurysms, arrhythmias, conduction abnormalities, pericarditis and valvulitis. The most affected cases can develop cardiogenic shock needing intensive care unit admission, inotropic support and sometimes even mechanical circulatory support. The elevation of myocardial necrosis markers, the fre-quently transient left ventricular systolic dysfunction and the presence of changes on magnetic resonance imaging, support the hypothesis of an immune-mediated post-viral pathogenesis similar to myocarditis. Although MIS-C shows excellent short-term survival, further studies are needed to demonstrate complete reversibility of residual subclinical heart damage

Documento di consenso ANMCO/SICP/SIGO: Gravidanza e cardiopatie congenite

The success of cardiac surgery over the past 50 years has increased numbers and median age of survivors with congenital heart disease (CHD). Adults now represent two-thirds of patients with CHD; in the United States alone the number is estimated to exceed 1 million. In this population many affected women reach reproductive age and wish to have children. While in many CHD patients pregnancy can be accomplished successfully, some special situations with complex anatomy, iatrogenic or residual pathology are associated with an increased risk of severe maternal and fetal complications. Pre-conception counseling allows women to come to truly informed choices. Risk stratification tools can also help high-risk women to eventually renounce to pregnancy and to adopt safe contraception options. Once pregnant, women identified as intermediate or high-risk should receive multidisciplinary care involving a cardiologist, an obstetrician and an anesthesiologist with specific expertise in managing this peculiar medical challenge. This document is intended to provide cardiologists working in hospitals where an Obstetrics and Gynecology Department is available with a streamlined and practical tool, useful for them to select the best management strategies to deal with a woman affected by CHD who desires to plan pregnancy or is already pregnant

Recommendations for cardiovascular magnetic resonance and computed tomography in congenital heart disease: a consensus paper from the CMR/CCT working group of the Italian Society of Pediatric Cardiology (SICP) and the Italian College of Cardiac Radiology endorsed by the Italian Society of Medical and Interventional Radiology (SIRM) Part I

Cardiovascular magnetic resonance (CMR) and computed tomography (CCT) are advanced imaging modalities that recently revolutionized the conventional diagnostic approach to congenital heart diseases (CHD), supporting echocardiography and often replacing cardiac catheterization. Nevertheless, correct execution and interpretation require in-depth knowledge of all technical and clinical aspects of CHD, a careful assessment of risks and benefits before each exam, proper imaging protocols to maximize diagnostic information, minimizing harm. This position paper, written by experts from the Working Group of the Italian Society of Pediatric Cardiology and from the Italian College of Cardiac Radiology of the Italian Society of Medical and Interventional Radiology, is intended as a practical guide for applying CCT and CMR in children and adults with CHD, wishing to support Radiologists, Pediatricians, Cardiologists and Cardiac Surgeons in the multimodality diagnostic approach to these patients. The first part provides a review of the most relevant literature in the field, describes each modality's advantage and drawback, making considerations on the main applications, image quality, and safety issues. The second part focuses on clinical indications and appropriateness criteria for CMR and CCT, considering the level of CHD complexity, the clinical and logistic setting and the operator expertise