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1.
Frontiers in Pediatrics ; 10:1012582, 2022.
Article in English | MEDLINE | ID: covidwho-2199087

ABSTRACT

Multisystem inflammatory syndrome in children (MIS-C) is a serious condition triggered by SARS-COV-2 infection, characterized by persistent fever, multiorgan dysfunction, and increased inflammatory markers. It requires hospitalization and prompt treatment, with nearly 60% of the cases needing intensive care and 2% fatality rate. A wide spectrum of clinical characteristics and therapeutic approaches has been reported in MIS-C. We describe a series of four patients with MIS-C, defined according to the current case definitions, with a self-limiting course and no need for immunomodulatory treatment ("self-limiting MIS-C"). Few data about self-limiting MIS-C are available to date and no information on medium- and long-term outcome of this subset of patients has been reported. Although limited in size, our experience provides new insights into the MIS-C syndrome, highlighting an underestimated aspect of the disease that may have significant therapeutic implications.

2.
Cardiology in the Young ; 32(Supplement 2):S87, 2022.
Article in English | EMBASE | ID: covidwho-2062123

ABSTRACT

Background and Aim: Growing evidence has documented a severe systemic hyperinflammation syndrome affecting children previ-ously exposed to SARS-CoV-2, known as Multisystem Inflammatory Syndrome in Children (MIS-C). Cardiovascular manifestations in MIS-C are frequent (34%-82%). The aim of our study was to describe the early and late cardiac abnormalities in patients with MIS-C, assessed by standard echocardiography, speckle tracking echocardiography (STE), and cardiac MRI (CMR). Method(s): 32 consecutive patients (21M, 11F), mean age 8.25 +/- 4years (range 1.3-17.7), with confirmed MIS-C diagnosis were enrolled in this study. Clinical, laboratory and microbiological data were collected. At disease onset, all children underwent standard transthoracic echocardiography, STE with analysis of left ventricle global longitudinal strain (GLS) and 23 (75%) of them performed CMR. Patients underwent complete cardiological evaluation, including echocardiography and STE at two months (T1) and six months (T2) after diagnosis. CMR was repeated at six months after diagnosis. Result(s): Cardiovascular symptoms were present in 45.8% of cases. Thirteen children (40.6%) shared Kawasaki Disease-like symp-toms, and 5 (15.6%) needed ICU admission. Early survival was 100%. All patients showed an hyperinflammatory state. Tn-I was elevated in 20 (62.5%) and BNP in 28 (87.5%) patients. Mean LVEF at baseline was 58.8 +/- 10% with 10 patients (31%) below 55%. STE showed reduced mean LV GLS (-17.4 +/- 4%). On CMR, LGE with nonischemic pattern was evident in 8/23 patients (35%). Follow-up data showed rapid improvement of LVEF at T1 (62.5 +/- 7.5 vs. 58.8 +/- 10.6%, p value 0.044) with only three patients (10%) below <= 55% at T1 and one (4%) at T2. LV GLS remained impaired at T1 (-17.2 +/- 2.7 vs.-17.4 +/- 4, p value 0.71), and significantly improved at T2 (-19 +/- 2.6% vs.-17.4 +/- 4%, p value 0.009). LV GLS was impaired (gt;-18%) in 53% of patients at baseline and T1, while only 13% showed persistent LV GLS reduc-tion at T2. Follow-up CMR showed LGE persistence in 33.4% of cases. Conclusion(s): Even though, early cardiac involvement significantly improves during follow-up, subclinical myocardial damage seems to be still detectable 6 months follow up in one third of MIS-C patients.

3.
Eur Heart J Cardiovasc Imaging ; 23(8): 1066-1074, 2022 07 21.
Article in English | MEDLINE | ID: covidwho-1873887

ABSTRACT

AIMS: Multisystem inflammatory syndrome in children (MIS-C) with cardiovascular manifestations are frequent. However, there is lacking evidence regarding cardiological follow-up of this cohort of patients. The aim of our study was to describe the early and mid-term cardiac abnormalities assessed by standard and speckle-tracking echocardiography (STE), and cardiac MRI (CMR). METHODS AND RESULTS: We enrolled 32 patients (21 male, 11 female), mean age 8.25 ± 4years, with diagnosis of MIS-C. During admission, all children underwent TTE, STE with analysis of left ventricle global longitudinal strain (GLS) and CMR. Patients underwent cardiological evaluation at 2 (T1) and 6 months (T2) after discharge. Cardiac MRI was repeated at 6 months after discharge. Mean left ventricular ejection fraction (LVEF) at baseline was 58.8 ± 10% with 10 patients (31%) below 55%. Speckle-tracking echocardiography showed reduced mean LV GLS (-17.4 ± 4%). On CMR, late gadolinium enhancement (LGE) with non-ischaemic pattern was evident in 8 of 23 patients (35%). Follow-up data showed rapid improvement of LVEF at T1 (62.5 ± 7.5 vs. 58.8 ± 10.6%, P-value 0.044) with only three patients (10%) below ≤ 55% at T1. Left ventricular (LV) GLS remained impaired at T1 (-17.2 ± 2.7 vs.-17.4 ± 4, P-value 0.71) and significantly improved at T2 (-19 ± 2.6% vs. -17.4 ± 4%, P-value 0.009). LV GLS was impaired (>-18%) in 53% of patients at baseline and T1, whereas only 13% showed persistent LV GLS reduction at T2. Follow-up CMR showed LGE persistence in 33.4% of cases. CONCLUSION: Early cardiac involvement significantly improves during follow-up of MIS-C patients. However, subclinical myocardial dysfunction seems to be still detectable after 6 months of follow-up in a not negligible proportion of them.


Subject(s)
Heart Defects, Congenital , Ventricular Dysfunction, Left , COVID-19/complications , Child , Child, Preschool , Contrast Media , Echocardiography/methods , Female , Follow-Up Studies , Gadolinium , Humans , Magnetic Resonance Imaging , Magnetic Resonance Imaging, Cine/methods , Male , Stroke Volume , Systemic Inflammatory Response Syndrome , Ventricular Function, Left
4.
Pediatric Rheumatology ; 18(SUPPL 2), 2020.
Article in English | EMBASE | ID: covidwho-1029730

ABSTRACT

Introduction: During SARS-COV-2 pandemic, different reports have been published regarding children who developed hyperinflammatory syndrome with certain or probable relationship with SARS-COV-2. These patients presented incomplete or atypical manifestations of Kawasaki disease (KD), particularly abdominal pain, myocarditis and macrophage activation syndrome features. Objectives: To report a case of SARS-COV-2-related Kawasaki-like disease with severe cardiac involvement. Methods: case report description. Results: A 10-year-old previously healthy girl presented progressively worsening abdominal pain, high grade fever for 3 days and vomiting. Lab tests showed WBC 11680/mmc, N 9370/mmc, Creactive protein (CRP) 329 mg/L, procalcitonin (PCT) 0,74 ug/L, PT-INR 1,35 and elevated D-dimer and fibrinogen levels (817 ug/L and 9,45 g/L respectively). Abdomen ultrasound revealed lymphadenopathies and hyperechogenic mesentery in the right lower quadrant, although the appendix was not visualized. She underwent laparoscopy showing moderate quantity of free fluid and appendectomy was performed. Thereafter she continued to complain of high-grade fever, abdominal pain and diarrhoea, despite broad-spectrum antibiotics. Blood, urine and stool cultures were negative. Bilateral non-exudative conjunctivitis was present. Moreover, the lab tests showed persistent marked elevation of CRP (370 mg/L), WBC 15590/mmc, N 14070/mmc, hypoalbuminemia (23 g/L), elevated ferritin and triglycerides (458 ug/L and 221 mg/dl). By taking into consideration the concomitant SARS-COV-2 pandemic, nasopharyngeal and rectal swabs were taken with negative results. Conversely, serological test showed anti-SARS-COV-2 IgG antibodies and absence of IgM. The family medical history showed that the mother had presented fever, cough, ageusia and anosmia one month before, preceded by a contact with a SARS-COV-2 positive case, while the patient was asymptomatic at that time. Suspecting a KD-like disease she was referred to our Paediatric Rheumatology Unit: cardiological assessment revealed negative Twaves in V4-V5-V6 on EKG while standard and advanced echocardiography showed mild mitral and tricuspid insufficiencies, mild dilatation of the left main coronary artery (LMCA, z score +2), normal global function (FEVS 2D 58%) but reduced longitudinal strain (GLS-16%). Lab tests confirmed myocardial injury with troponin (TnI) 100,1 ng/l and brain natriuretic peptide (P-BNP) 593 ng/L. A single infusion of intravenous immunoglobulin 2 g/kg associated with methylprednisolone (1 mg/kg/day) led to a rapid clinical improvement with apyrexia and resolution of abdominal pain and conjunctivitis. Blood test confirmed gradual normalization of inflammatory markers, ferritin, troponin and BNP and EKG showed positive T-waves. Shortly after the discharge, while she was on prednisone 0.5 mg/kg/day and acetylsalicylic acid 100 mg/day, she referred some episodes of heart pounding, lasting about ten minutes with spontaneous resolution. Three weeks after onset, cardiac MRI was normal, however, speckle tracking echocardiography showed persistent dilatation of LMCA and reduction of global longitudinal strain (GLS-14%). 24-hour EKG-Holter detected episodes of supraventricular tachycardia and several ventricular and supraventricular extrasystoles. Thus, oral atenolol therapy was started. Conclusion: In our patient SARS-COV-2 induced a possible postinfectious antibody or immune-complex mediated reaction that led to KD-like disease with acute surgical abdomen presentation and persistent myocardial damage and arrythmias. Speckle tracking echocardiography appears more reliable than MRI in early detection of myocardial damage in patients with preserved left ventricular ejection fraction.

5.
Pediatric Rheumatology ; 18(SUPPL 2), 2020.
Article in English | EMBASE | ID: covidwho-1029414

ABSTRACT

Introduction: Macrophage activation syndrome (MAS) is a rare, potentially life-threatening complication of some rheumatologic diseases1. Objectives: We report the case of a child with systemic onset Juvenile Idiopathic Arthritis (sJIA) complicated by severe MAS and acute myocarditis, needing veno-arterial Extracorporeal Membrane Oxygenation (VA-ECMO), successfully rescued by high dose intravenous Anakinra (HDIV-ANA). Methods: Case report's description Results: A two-year-old boy presented with one month history of fever associated with limping gait, cervical lymphadenopathy and skin rash. Laboratory tests showed elevation of inflammatory markers and ferritin. By exclusion criteria, sJIA was diagnosed and steroid therapy started. After a soft tissue bacterial infection, fever relapsed and laboratory tests were consistent with MAS (day 1): Hb 8.5 g/dL, PLT 44000/mm3;FDP 1522 ug/L, CRP 100 mg/L, ferritin 2200 ug/L. High doses intravenous metilprednisolone and oral Cyclosporin A (CSA) were started. On day 2 he presented a Systemic Capillary Leak Syndrome and acute myocarditis. He was admitted into the pediatric intensive care unit (PICU) where intravenous immunoglobulin and subcutaneous Anakinra (ANA) were added. On day 4, due to an episode of cardiac arrest, VA-ECMO was started and we tried high dose intravenous ANA (HDIV-ANA, 8 mg/Kg/day q6h). This treatment brought immediate benefit: echocardiography showed progressive resolution of myocarditis so that VA-ECMO was definitely weaned off in six days. Laboratory test showed isolated neutropenia (PMNs 0-100/mm3). Suspecting a iatrogenic cause, HDIV-ANA was gradually reduced to the maintenance dose without benefit. On day 22, ANA was stopped and neutropenia resolved. Analysis of PRF1 gene revealed a mutation (c.[272C>T] p.[Ala91Val]) in heterozygosis. 49 days after admission he was discharged on oral prednisone and CSA. Neither neurological nor other organ consequences related to MAS were reported. A few months later, on tapering down of therapy, he relapsed. ANA was restarted with rapid improvement and no side effects, including neutropenia. Currently, after 12 months, the disease is in clinical remission on medication. Conclusion: MAS is a rare life-threatening complication of sJIA, triggered by infections in up to one-third of the patients 2. It is the result of a cytokine storm that lead to a dysregulated inflammatory activation of the immune system, with rapid progression to multiorgan failure. Treatment usually includes high dose corticosteroids and immunosuppressive agents. Recently, the use of selective cytokine inhibitors has been suggested. No standardized guidelines are available to date, but the use of ANA has been already reported, pointing out the need for a higher doses regimen in refractory cases. MAS in our patient appeared after a soft tissue infection which could have act as triggering factor in a patient with sJIA and genetic predisposing pattern. The choice of intravenous administration of ANA was partly due to the generalized edema and partly to the severe discoaugulopathy. Considering the higher doses needed for rapidly suppressing the cytokine storm and ANA pharmacokinetics, we split the daily dose into four administrations. No major adverse events were reported, except for a transient neutropenia, already reported 6. Based on our experience, HDIV-ANA is a safe and effective treatment for refractory life-threatening sJIA-related MAS. This therapeutic approach may be also considered in the current pandemic COVID-19 emergency where recent evidence showed IL1-driven MAS-like complication triggered by SARS-COV-2 virus as predictor of bad outcome7.

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