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1.
Rheumatology (Oxford) ; 63(2): 366-375, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-37647645

RESUMO

OBJECTIVE: The main aim of this study was to define the best treatment option for multisystem inflammatory syndrome in children (MIS-C) and to analyse the role of anakinra. METHODS: This is a multicentre retrospective cohort study. Patients were treated according to the attending physician's decision. The patients were divided into four groups on the basis of the first treatment at time of admittance: (i) IVIG, (ii) IVIG and methylprednisolone (≤2 mg/kg/day), (iii) IVIG with high-dose methylprednisolone (>2 mg/kg/day) and (iv) anakinra with or without IVIG and/or methylprednisolone. Primary outcomes were defined as the presence of at least one of the following features: death, the failure of initial treatment, meaning the need for additional treatment for clinical worsening and cardiac involvement at the end of follow-up. RESULTS: Two hundred thirty-nine patients were recruited. At univariate analysis, persistent heart involvement at discharge was more frequent in those not receiving anakinra as initial treatment (3/21 vs 66/189; P = 0.047). After comparisons between the four treatment regimens, adjusting for the propensity score, we observed that early treatment with anakinra was associated with a lower probability of developing persistent heart disease at the end of follow-up (odds ratio: 0.6; 95% CI: 0.4-1.0). CONCLUSION: We report that early treatment with anakinra is safe and very effective in patients with severe MIS-C. In addition, our study suggests that early treatment with anakinra is the most favourable option for patients with a higher risk of developing a severe disease outcome.


Assuntos
COVID-19/complicações , Imunoglobulinas Intravenosas , Proteína Antagonista do Receptor de Interleucina 1 , Síndrome de Resposta Inflamatória Sistêmica , Criança , Humanos , Proteína Antagonista do Receptor de Interleucina 1/uso terapêutico , Estudos Retrospectivos , Gravidade do Paciente , Metilprednisolona
2.
Eur J Pediatr ; 182(11): 4889-4895, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37597046

RESUMO

Children with Kawasaki disease (KD), Multisystem Inflammatory Syndrome in Children (MIS-C), and Adenovirus infections (AI) of the upper respiratory tract show overlapping features. This study aims to develop a scoring system based on clinical or laboratory parameters to differentiate KD or MIS-C from AI patients. Ninety pediatric patients diagnosed with KD (n = 30), MIS-C (n = 26), and AI (n = 34) admitted to the Pediatric Emergency Unit of S.Orsola University Hospital in Bologna, Italy, from April 2018 to December 2021 were enrolled. Demographic, clinical, and laboratory data were recorded. A multivariable logistic regression analysis was performed, and a scoring system was subsequently developed. A simple model (clinical score), including five clinical parameters, and a complex model (clinic-lab score), resulting from the addition of one laboratory parameter, were developed and yielded 100% sensitivity and 80% specificity with a score ≥2 and 98.3% sensitivity and 83.3% specificity with a score ≥3, respectively, for MIS-C and KD diagnosis, as compared to AI. CONCLUSION: This scoring system, intended for both outpatients and inpatients, might limit overtesting, contribute to a more effective use of resources, and help the clinician not underestimate the true risk of KD or MIS-C among patients with an incidental Adenovirus detection. WHAT IS KNOWN: • Kawasaki Disease (KD), Multisystem Inflammatory Syndrome in Children (MIS-C) and adenoviral infections share overlapping clinical presentation in persistently febrile children, making differential diagnosis challenging. • Scoring systems have been developed to identify high-risk KD patients and discriminate KD from MIS-C patients. WHAT IS NEW: • This is the first scoring model based on clinical criteria to distinguish adenoviral infection from KD and MIS-C. • The score might be used by general pediatricians before referring febrile children to the emergency department.


Assuntos
Infecções por Adenoviridae , Síndrome de Linfonodos Mucocutâneos , Humanos , Criança , Diagnóstico Diferencial , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Infecções por Adenoviridae/complicações , Infecções por Adenoviridae/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Febre
3.
Pediatr Cardiol ; 2023 Dec 29.
Artigo em Inglês | MEDLINE | ID: mdl-38157048

RESUMO

Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19 show clinical overlap and both lack definitive diagnostic testing, making differentiation challenging. We sought to determine how cardiac biomarkers might differentiate KD from MIS-C. The International Kawasaki Disease Registry enrolled contemporaneous KD and MIS-C pediatric patients from 42 sites from January 2020 through June 2022. The study population included 118 KD patients who met American Heart Association KD criteria and compared them to 946 MIS-C patients who met 2020 Centers for Disease Control and Prevention case definition. All included patients had at least one measurement of amino-terminal prohormone brain natriuretic peptide (NTproBNP) or cardiac troponin I (TnI), and echocardiography. Regression analyses were used to determine associations between cardiac biomarker levels, diagnosis, and cardiac involvement. Higher NTproBNP (≥ 1500 ng/L) and TnI (≥ 20 ng/L) at presentation were associated with MIS-C versus KD with specificity of 77 and 89%, respectively. Higher biomarker levels were associated with shock and intensive care unit admission; higher NTproBNP was associated with longer hospital length of stay. Lower left ventricular ejection fraction, more pronounced for MIS-C, was also associated with higher biomarker levels. Coronary artery involvement was not associated with either biomarker. Higher NTproBNP and TnI levels are suggestive of MIS-C versus KD and may be clinically useful in their differentiation. Consideration might be given to their inclusion in the routine evaluation of both conditions.

4.
Int J Mol Sci ; 24(20)2023 Oct 11.
Artigo em Inglês | MEDLINE | ID: mdl-37894766

RESUMO

Multisystem inflammatory syndrome in children (MIS-C) is a postinfectious sequela of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), with some clinical features overlapping with Kawasaki disease (KD). Our research group and others have highlighted that the spike protein of SARS-CoV-2 can trigger the activation of human endogenous retroviruses (HERVs), which in turn induces inflammatory and immune reactions, suggesting HERVs as contributing factors in COVID-19 immunopathology. With the aim to identify new factors involved in the processes underlying KD and MIS-C, we analysed the transcriptional levels of HERVs, HERV-related genes, and immune mediators in children during the acute and subacute phases compared with COVID-19 paediatric patients and healthy controls. The results showed higher levels of HERV-W, HERV-K, Syn-1, and ASCT-1/2 in KD, MIS-C, and COV patients, while higher levels of Syn-2 and MFSD2A were found only in MIS-C patients. Moreover, KD and MIS-C shared the dysregulation of several inflammatory and regulatory cytokines. Interestingly, in MIS-C patients, negative correlations have been found between HERV-W and IL-10 and between Syn-2 and IL-10, while positive correlations have been found between HERV-K and IL-10. In addition, HERV-W expression positively correlated with the C-reactive protein. This pilot study supports the role of HERVs in inflammatory diseases, suggesting their interplay with the immune system in this setting. The elevated expression of Syn-2 and MFSD2A seems to be a distinctive trait of MIS-C patients, allowing to distinguish them from KD ones. The understanding of pathological mechanisms can lead to the best available treatment for these two diseases, limiting complications and serious outcomes.


Assuntos
COVID-19 , Retrovirus Endógenos , Síndrome de Linfonodos Mucocutâneos , Humanos , Criança , SARS-CoV-2/genética , COVID-19/genética , Retrovirus Endógenos/genética , Interleucina-10/genética , Síndrome de Linfonodos Mucocutâneos/genética , Projetos Piloto
5.
Acta Paediatr ; 111(10): 2010-2016, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-35751481

RESUMO

AIM: Adherence to croup management recommendations has been poorly investigated. This study aimed to describe the treatment patterns in two paediatric emergency departments and analyse the adherence to recommendations. METHODS: We conducted a retrospective chart review of children diagnosed with croup in two Italian paediatric emergency departments in 2017. Data on clinical presentation, corticosteroid administration and home therapy were collected. Length of stay, hospitalisation and re-access rates were compared among different corticosteroid treatment groups. RESULTS: We enrolled 632 patients (61.1% males) with a mean age of 42.8 ± 55.1 months. Corticosteroids were administered to 403 (63.8%) children in the emergency departments. Dexamethasone was administered to 1 (0.4%) patient. Inhaled and oral corticosteroids were given to 342 (54.1%) and 226 (35.8%) patients, respectively. Home therapy was prescribed for 603 (95.4%) patients, either with inhaled (86.2%) and/or oral (43.8%) corticosteroids. The re-access rate was 2.8%. The actual pharmaceutical costs were an estimated 10 times higher than they would have been if the recommendations had been followed. CONCLUSION: A significant gap between the evidence and clinical practice for croup treatment was observed. Improving adherence to the recommendations could lead to clinical and economic benefits.


Assuntos
Crupe , Corticosteroides/uso terapêutico , Criança , Pré-Escolar , Crupe/induzido quimicamente , Crupe/tratamento farmacológico , Dexametasona/uso terapêutico , Feminino , Glucocorticoides/uso terapêutico , Humanos , Lactente , Masculino , Estudos Retrospectivos
6.
Pediatr Cardiol ; 43(7): 1462-1470, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35316356

RESUMO

Psoriasis is a skin disorder which mostly affects adults, beginning in childhood in almost one-third of patients. In adults it is associated with increased risk for cardiovascular diseases (CVD), while this association is still debated at younger age. Our aim was to evaluate the association between psoriasis and metabolic markers and cardiovascular findings in this age group. Twenty consecutive patients previously diagnosed with psoriasis (group A) were enrolled and compared with healthy non- psoriatic age- and sex-matched subjects (group B). The severity of the disease, CV risk factors, including anthropometric data with adiposity and its distribution, blood pressure (BP), laboratory metabolic tests, echocardiography and vascular ultrasound (transcranial echo-Doppler and carotid artery echo-Doppler with carotid intima-media thickness, cIMT) were performed for each subject. Personal history for CV risk, BP, anthropometric data were similar between the two groups, while familiar history for psoriasis was more frequent in group A (p < 0.02). C-IMT was significantly higher in group A compared to B (right, p = 0.001; left, p = 0.002). In addition, c-IMT was positively correlated with disease duration, triglycerides and triglycerides/glucose. Cerebral flow velocities, cardiac measurements, systo-diastolic function, ventricle geometry and mass were normal and comparable between the two groups, and did not correlate with CV risk factors. In childhood psoriasis c-IMT could represent a marker of pre-clinical cardiovascular involvement and contribute to start a personalized management, while cardiac findings seem to be normal in the early stage of disease. Longitudinal studies can clarify the progression of CV involvement in paediatric-onset psoriasis.


Assuntos
Doenças Cardiovasculares , Psoríase , Adulto , Doenças Cardiovasculares/epidemiologia , Doenças Cardiovasculares/etiologia , Artérias Carótidas/diagnóstico por imagem , Espessura Intima-Media Carotídea , Criança , Glucose , Fatores de Risco de Doenças Cardíacas , Humanos , Psoríase/complicações , Fatores de Risco , Triglicerídeos
7.
Int J Mol Sci ; 23(17)2022 Sep 03.
Artigo em Inglês | MEDLINE | ID: mdl-36077506

RESUMO

BACKGROUND: Kawasaki Disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) are pediatric diseases characterized by systemic inflammation and vascular injury, potentially leading to coronary artery lesions (CALs). Data on vascular injury occurring during acute COVID-19 (AC19) in children are still lacking. The aim of our study was to investigate endothelial injury in KD-, MIS-C- and AC19-dosing circulating endothelial cells (CECs). METHODS: We conducted a multicenter prospective study. CECs were enumerated by CellSearch technology through the immunomagnetic capture of CD146-positive cells from whole blood. RESULTS: We enrolled 9 KD, 20 MIS-C and 10 AC19. During the acute stage, the AC19 and KD patients had higher CECs levels than the MIS-C patients. From the acute to subacute phase, a significant CEC increase was observed in the KD patients, while a mild decrease was detected in the MIS-C patients. Cellular clusters/syncytia were more common in the KD patients. No correlation between CECs and CALs were found in the MIS-C patients. The incidence of CALs in the KD group was too low to investigate this correlation. CONCLUSIONS: Our study suggests a possible role of CECs as biomarkers of systemic inflammation and endothelial dysfunction in KD and MIS-C and different mechanisms of vascular injury in these diseases. Further larger studies are needed.


Assuntos
COVID-19 , Síndrome de Linfonodos Mucocutâneos , Lesões do Sistema Vascular , Biomarcadores , COVID-19/complicações , Criança , Células Endoteliais/patologia , Humanos , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Estudos Prospectivos , SARS-CoV-2 , Síndrome de Resposta Inflamatória Sistêmica/complicações , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico
8.
J Pediatr ; 215: 187-191, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31587860

RESUMO

OBJECTIVE: To describe clinical presentation, electrocardiographic, and echocardiographic characteristics of carditis at the time of diagnosis of acute rheumatic fever (ARF) over a 13-year period. STUDY DESIGN: A single-center retrospective chart analysis was conducted involving all consecutive patients diagnosed with ARF between 2003 and 2015. Patient age, sex, clinical characteristics, recent medical history for group A streptococcal pharyngotonsillitis and antibiotic treatment, and laboratory, echocardiographic, and electrocardiographic findings were recorded. RESULTS: Of 98 patients (62 boys, mean age 8.81 ± 3.04 years), 59 (60.2%) reported a positive history of pharyngotonsillitis; 48 (49%) had received antibiotic (mean duration of treatment of 5.9 ± 3.1 days), and, among these, 28 (58.3%) had carditis. Carditis was the second most frequent finding, subclinical in 27% of patients. Mitral regurgitation was present in 49 of 56 patients (87.5%) and aortic regurgitation in 36/56 (64.3%) no stenosis was documented. CONCLUSIONS: ARF is still present in high-income countries and can develop despite primary prophylaxis, especially when given for a short course. Our findings highlight the need for 10 days of antistreptococcal treatment to prevent ARF. Echocardiography is important because 27% of cases with carditis were subclinical.


Assuntos
Miocardite/diagnóstico , Miocardite/epidemiologia , Febre Reumática/diagnóstico , Adolescente , Antibacterianos/uso terapêutico , Insuficiência da Valva Aórtica/diagnóstico por imagem , Artrite/microbiologia , Bloqueio Atrioventricular/diagnóstico , Sedimentação Sanguínea , Criança , Pré-Escolar , Coreia/microbiologia , Países Desenvolvidos , Ecocardiografia Doppler em Cores , Eletrocardiografia , Eritema/microbiologia , Feminino , Hemoglobinas/análise , Humanos , Itália/epidemiologia , Masculino , Insuficiência da Valva Mitral/diagnóstico por imagem , Faringite/epidemiologia , Estudos Retrospectivos , Febre Reumática/tratamento farmacológico , Febre Reumática/epidemiologia , Estações do Ano , Tonsilite/epidemiologia
9.
Eur J Pediatr ; 178(3): 315-322, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30499051

RESUMO

Since resistance to intravenous immunoglobulin (IVIG) is associated with coronary lesions (CALs) in Kawasaki disease (KD), it is crucial to identify patients at risk to protect them from coronary involvement. The available risk scores to predict IVIG resistance were developed in Asian populations in whom their effectiveness has been proven, but data on non-Asian children are limited. The aim of this study is to evaluate the ability of the Kobayashi, Egami, and Formosa risk scores to predict IVIG resistance and CALs in Italian patients with KD. A multicenter retrospective analysis involving children with KD diagnosed between 2000 and 2015 was carried out: 257 patients were enrolled (57.9% boys, 89.9% Caucasian); 43 patients were IVIG resistant (16.7%). The scores have low sensitivity and specificity in predicting IVIG resistance: respectively, KS 64% and 62.5%, ES 41.4% and 77.4%, and FS 70.8% and 44.9%. The predictive value of the 3 scores for predicting CALs was also poor.Conclusion: Kobayashi, Egami, and Formosa Scores are ineffective in predicting IVIG resistance and coronary involvement in a predominantly Caucasian cohort. A specific score system for mostly Caucasian children with KD is needed enable the early identification of those at risk for CALs who could benefit from intensified treatment. What is Known: • There are several risk scores developed in the Asian population to early identify patients with KD at risk for immunoglobulin-resistance and thus for coronary lesions. • Data are scarce on their effectiveness in non-Asian children. What is New: • We present a comprehensive analysis of the ability of 3 Asian risk scores in a cohort of mostly Caucasian children to predict immunoglobulin resistance and coronary involvement. • Low sensitivity and specificity of the Asian scores for immunoglobulin-resistance and coronary lesions suggest the need for criteria specific for different ethnicities.


Assuntos
Técnicas de Apoio para a Decisão , Resistência a Medicamentos , Cardiopatias/etiologia , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Povo Asiático , Criança , Pré-Escolar , Feminino , Cardiopatias/diagnóstico , Humanos , Lactente , Itália , Japão , Masculino , Síndrome de Linfonodos Mucocutâneos/complicações , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Sensibilidade e Especificidade , Taiwan , População Branca
10.
Cardiol Young ; 27(3): 605-608, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-27834169

RESUMO

A 12-year-old boy with a right atrium endocardial mass was initially diagnosed as having Lemierre's syndrome on the basis of previous mastoiditis and jugular vein and cerebral venous thrombosis. Lack of response to antibiotics, persistent high fever with chills, acute-phase reactants, and peripheral arterial pseudoaneurysms made us reconsider the diagnosis. Only after the late appearance of radiological pulmonary lesions and recognition of pulmonary artery aneurysms, Hughes-Stovin syndrome was diagnosed. Hughes-Stovin syndrome is an exceedingly rare vasculitis, especially in childhood, consisting of multiple pulmonary artery aneurysms and deep venous thromboses. The lack of formal diagnostic criteria and the rarity of the disease make the diagnosis very challenging, especially when respiratory complaints are not present at onset, as in the presented case. The treatment aims to reduce inflammation, although there is debate about anticoagulation therapy because of the risk of pulmonary haemorrhage.


Assuntos
Aneurisma/complicações , Endocárdio/diagnóstico por imagem , Febre/etiologia , Artéria Pulmonar , Infarto Pulmonar/complicações , Trombose dos Seios Intracranianos/complicações , Aneurisma/diagnóstico , Criança , Diagnóstico Diferencial , Febre/diagnóstico , Humanos , Masculino , Infarto Pulmonar/diagnóstico , Trombose dos Seios Intracranianos/diagnóstico , Síndrome
11.
Cardiol Young ; 26(4): 805-7, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26508431

RESUMO

Univentricular heart is a rare congenital heart malformation. Nowadays prognosis is considered to be strictly linked to surgical intervention, and survival into late adulthood is unusual. In some patients native haemodynamic circulation balances pulmonary and systemic blood flow, allowing long-term survival without the need for surgery. We report the case of a 74-year-old man with a univentricular heart in natural history, and we discuss the factors that might contribute to his extraordinary long-term survival.


Assuntos
Cardiopatias Congênitas , Ventrículos do Coração/anormalidades , Idoso , Humanos , Masculino , Sobreviventes
12.
J Pediatr ; 221: 263, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32303356
13.
Pediatr Nephrol ; 29(5): 893-900, 2014 May.
Artigo em Inglês | MEDLINE | ID: mdl-24337408

RESUMO

BACKGROUND: Cardiovascular disease (CVD) is a common cause of morbidity and mortality in children with chronic kidney disease (CKD). Left ventricular hypertrophy (LVH) and diastolic dysfunction (LVDD) are early markers. The aims of this study were to evaluate (1) LVH and LVDD, using both conventional echocardiographic evaluation and Tissue Doppler Imaging (TDI), and (2) the correlation between cardiac disease and possible risk factors, in children with CKD. METHODS: The study cohort comprised 34 paediatric patients with CKD and 34 healthy children (mean ± standard deviation: age 9 ± 4.6 and 8.2 ± 4.3 years, respectively). Thirteen (38 %) patients were in CKD stage 2, 15 (44 %) in stage 3 and six (18 %) in stage 4-5. LVH was defined as a left ventricular mass index (LVMI) of >95th percentile (38 g/h(2.7)). RESULTS: Left ventricular hypertrophy was present in 13 patients (38 %). Diastolic function evaluated with TDI (E'/A' = early/late diastolic myocardial velocity) worsened with the reduction of glomerular filtration rate (p = 0.020). There was a positive correlation between LVMI and body mass index-standard deviation score (p = 0.020) and a negative correlation between E'/A' and serum phosphorus and calcium levels and their respective product (p = 0.004, p = 0.017, p < 0.001). The relaxation index E' was reduced in 68 % of patients. CONCLUSION: Based on our results, TDI is a simple procedure and would appear to be a more accurate diagnostic tool than conventional echocardiography in the early diagnosis of LVDD.


Assuntos
Coração/fisiopatologia , Hipertrofia Ventricular Esquerda/patologia , Insuficiência Renal Crônica/patologia , Adolescente , Cálcio/sangue , Criança , Pré-Escolar , Feminino , Taxa de Filtração Glomerular , Testes de Função Cardíaca , Humanos , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Hipertrofia Ventricular Esquerda/etiologia , Masculino , Fósforo/sangue , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/fisiopatologia , Fatores de Risco , Ultrassonografia Doppler
14.
Biomedicines ; 12(9)2024 Sep 03.
Artigo em Inglês | MEDLINE | ID: mdl-39335523

RESUMO

Background: Kawasaki disease (KD) is a systemic vasculitis of medium arteries, particularly involving coronary arteries. Coronary artery lesions (CALs) is the most serious complication in the acute stage, potentially leading to ischemic cardiomyopathy, myocardial infarction and sudden death. Environmental factors and genetic background contribute to individual susceptibility to develop CALs. The aim of this study was to define the risk factors for CALs in an Italian cohort. Methods: Data of KD patients from 10 Italian sites were registered into a REDCap database where demographic and clinical data, laboratory findings and coronary status were recorded. KD was diagnosed according to AHA definition. We used multiple logistic regression analysis to identify independent risk factors for CALs. Results: A total of 517 patients were enrolled, mainly Caucasians (83.6%). Presentation was complete in 321 patients (62.8%) and IVIG responsiveness in 360 (70%). CALs developed in 136/517 (26.31%). Gender, age, ethnicity, clinical presentation, fever duration, non-coronary cardiac events, Hb, albumin and CRP were significantly different between patients with and without CALs, while seasonality was not. Male gender, age < 18 months, Asian ethnicity, incomplete presentation and fever > 10 days were independent risk factors for CALs. Conclusions: Age younger than 18 months, incomplete KD and longer fever duration are risk factors for CALs. Asian ethnicity also represents a risk factor in our Italian Cohort.

15.
Can J Cardiol ; 40(1): 58-72, 2024 01.
Artigo em Inglês | MEDLINE | ID: mdl-37290536

RESUMO

BACKGROUND: Patients with multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) have overlapping clinical features. We compared demographics, clinical presentation, management, and outcomes of patients according to evidence of previous SARS-CoV-2 infection. METHODS: The International Kawasaki Disease Registry (IKDR) enrolled KD and MIS-C patients from sites in North, Central, and South America, Europe, Asia, and the Middle East. Evidence of previous infection was defined as: Positive (household contact or positive polymerase chain reaction [PCR]/serology), Possible (suggestive clinical features of MIS-C and/or KD with negative PCR or serology but not both), Negative (negative PCR and serology and no known exposure), and Unknown (incomplete testing and no known exposure). RESULTS: Of 2345 enrolled patients SARS-CoV-2 status was Positive for 1541 (66%) patients, Possible for 89 (4%), Negative for 404 (17%) and Unknown for 311 (13%). Clinical outcomes varied significantly among the groups, with more patients in the Positive/Possible groups presenting with shock, having admission to intensive care, receiving inotropic support, and having longer hospital stays. Regarding cardiac abnormalities, patients in the Positive/Possible groups had a higher prevalence of left ventricular dysfunction, and patients in the Negative and Unknown groups had more severe coronary artery abnormalities. CONCLUSIONS: There appears to be a spectrum of clinical features from MIS-C to KD with a great deal of heterogeneity, and one primary differentiating factor is evidence for previous acute SARS-CoV-2 infection/exposure. SARS-CoV-2 Positive/Possible patients had more severe presentations and required more intensive management, with a greater likelihood of ventricular dysfunction but less severe coronary artery adverse outcomes, in keeping with MIS-C.


Assuntos
COVID-19 , Síndrome de Linfonodos Mucocutâneos , Síndrome de Resposta Inflamatória Sistêmica , Criança , Humanos , COVID-19/epidemiologia , SARS-CoV-2 , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Síndrome de Linfonodos Mucocutâneos/terapia , Sistema de Registros
16.
Thromb Res ; 239: 109040, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38795561

RESUMO

BACKGROUND AND AIM: Hughes-Stovin syndrome (HSS) is a rare systemic vasculitis with widespread venous/arterial thrombosis and pulmonary vasculitis. Distinguishing between pulmonary embolism (PE) and in-situ thrombosis in the early stages of HSS is challenging. The aim of the study is to compare clinical, laboratory, and computed tomography pulmonary angiography (CTPA) characteristics in patients diagnosed with PE versus those with HSS. METHODS: This retrospective study included 40 HSS patients with complete CTPA studies available, previously published by the HSS study group, and 50 patients diagnosed with PE from a single center. Demographics, clinical and laboratory findings, vascular thrombotic events, were compared between both groups. The CTPA findings were reviewed, with emphasis on the distribution, adherence to the mural wall, pulmonary infarction, ground glass opacification, and intra-alveolar hemorrhage. Pulmonary artery aneurysms (PAAs) in HSS were assessed and classified. RESULTS: The mean age of HSS patients was 35 ± 12.3 years, in PE 58.4 ± 17 (p < 0.0001). Among PE 39(78 %) had co-morbidities, among HSS none. In contrast to PE, in HSS both major venous and arterial thrombotic events are seen.. Various patterns of PAAs were observed in the HSS group, which were entirely absent in PE. Parenchymal hemorrhage was also more frequent in HSS compared to PE (P < 0.001). CONCLUSION: Major vascular thrombosis with arterial aneurysms formation are characteristic of HSS. PE typically appear loosely-adherent and mobile whereas "in-situ thrombosis" seen in HSS is tightly-adherent to the mural wall. Mural wall enhancement and PAAs are distinctive pulmonary findings in HSS. The latter findings have significant therapeutic ramifications.


Assuntos
Angiografia por Tomografia Computadorizada , Embolia Pulmonar , Humanos , Embolia Pulmonar/diagnóstico por imagem , Feminino , Masculino , Adulto , Pessoa de Meia-Idade , Estudos Retrospectivos , Angiografia por Tomografia Computadorizada/métodos , Vasculite/diagnóstico por imagem , Vasculite/complicações , Idoso , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/patologia
17.
Cardiol Young ; 23(5): 769-72, 2013 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-23164435

RESUMO

Nutritional rickets is still occasionally found in high-income countries, especially in populations at risk, and induced hypocalcaemia is a rare but possible cause of dilated cardiomyopathy. Although rare, physicians need to consider nutritional rickets in the differential diagnosis of hypocalcaemia cardiac failure, especially in high-risk populations such as immigrants. Despite being a reversible condition, the prognosis depends on the severity and time of diagnosis. We report two cases of exclusively breastfed infants with congestive cardiac failure due to hypokinetic dilated cardiomyopathy who had completely different outcomes. This report supports the need for prevention of this deficiency and underlies the role of vitamin D supplementation.


Assuntos
Cardiomiopatia Dilatada/etiologia , Hipocalcemia/complicações , Raquitismo/complicações , Cardiomiopatia Dilatada/diagnóstico por imagem , Cardiomiopatia Dilatada/tratamento farmacológico , Feminino , Humanos , Hipocalcemia/tratamento farmacológico , Lactente , Masculino , Raquitismo/tratamento farmacológico , Resultado do Tratamento , Ultrassonografia , Deficiência de Vitamina D/complicações , Deficiência de Vitamina D/tratamento farmacológico
18.
Nutrients ; 15(12)2023 Jun 07.
Artigo em Inglês | MEDLINE | ID: mdl-37375557

RESUMO

The relationship between nutrition and cardiovascular diseases is powerful and complex [...].


Assuntos
Cardiologia , Doenças Cardiovasculares , Sistema Cardiovascular , Criança , Humanos , Doenças Cardiovasculares/prevenção & controle
19.
Pathogens ; 12(8)2023 Jul 30.
Artigo em Inglês | MEDLINE | ID: mdl-37623957

RESUMO

Multisystem inflammatory syndrome (MIS-C) is a rare condition associated with COVID-19 affecting children, characterized by severe and aberrant systemic inflammation leading to nonspecific symptoms, such as gastrointestinal, cardiac, respiratory, hematological, and neurological disorders. In the last year, we have experienced a progressive reduction in the incidence and severity of MIS-C, reflecting the worldwide trend. Thus, starting from the overall trend in the disease in different continents, we reviewed the literature, hypothesizing the potential influencing factors contributing to the reduction in cases and the severity of MIS-C, particularly the vaccination campaign, the spread of different SARS-CoV-2 variants (VOCs), and the changes in human immunological response. The decrease in the severity of MIS-C and its incidence seem to be related to a combination of different factors rather than a single cause. Maturation of an immunological memory to SARS-CoV-2 over time, the implication of mutations of key amino acids of S protein in VOCs, and the overall immune response elicited by vaccination over the loss of neutralization of vaccines to VOCs seem to play an important role in this change.

20.
Front Nutr ; 10: 1332881, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38188871

RESUMO

Children, adolescents, and young adult cancer survivors (CAYAs) constitute a growing population requiring a customized approach to mitigate the incidence of severe complications throughout their lifetimes. During cancer treatment, CAYAs cancer survivors undergo significant disruptions in their nutritional status, elevating the risks of mortality, morbidity, and cardiovascular events. The assessment of nutritional status during cancer treatment involves anthropometric and dietary evaluations, emphasizing the necessity for regular assessments and the timely identification of risk factors. Proactive nutritional interventions, addressing both undernutrition and overnutrition, should be tailored to specific age groups and incorporate a family-centered approach. Despite encouraging interventions, a notable evidence gap persists. The goal of this review is to comprehensively examine the existing evidence on potential nutritional interventions for CAYAs cancer survivors. We explore the evidence so far collected on the nutritional intervention strategies elaborated for CAYAs cancer survivors that should target both undernutrition and overnutrition, being age-specific and involving a family-based approach. Furthermore, we suggest harnessing artificial intelligence (AI) to anticipate and prevent malnutrition in CAYAs cancer survivors, contributing to the identification of novel risk factors and promoting proactive, personalized healthcare.

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