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AIMS: The extracardiac conduit-Fontan (ECC) has become the preferred technique for univentricular heart palliation, but there are currently no data on the incidence of long-term arrhythmias. This study investigated the incidence of arrhythmias and relation to single ventricle morphology in the long-term follow-up (FU) in ECC. METHODS AND RESULTS: All patients with ECC performed in our Centre between 1987 and 2017 were included (minimum FU 5 years). Of 353 consecutive patients, 303 [57.8% males, aging 8-50 (median 20) years at last FU] were considered and divided into two groups depending on left (194 in Group 1) or right (109 in Group 2) ventricular morphology. Eighty-five (28%) experienced ≥1 arrhythmic complications, with early and late arrhythmias in 17 (5.6%) and 73 (24.1%) patients, respectively. Notably, late bradyarrhythmias occurred after 6 years in 21 (11%) patients in Group 1, and in 15 (13.8%) in Group 2 [P = 0.48]. Late tachyarrhythmias occurred in 55 (18.2%) patients after 12 years: 33 (17%) in Group 1 and 22 (20.2%) patients in Group 2 [P = 0.5]. Ventricular tachycardias (VT) were documented after 12.5 years in 14 (7.2%) patients of Group 1 and 15 (13.8%) of Group 2 [P = 0.06] with a higher incidence in Group 2 during the FU [P = 0.005]. CONCLUSION: Extracardiac conduit is related to a significant arrhythmic risk in the long-term FU, higher than previously reported. Bradyarrhythmias occur earlier but are less frequent than tachyarrhythmias. Interestingly, patients with systemic right ventricle have a significantly higher incidence of VT, especially in a very long FU.
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Arritmias Cardíacas , Técnica de Fontan , Ventrículos do Coração , Humanos , Masculino , Feminino , Técnica de Fontan/efeitos adversos , Incidência , Criança , Adolescente , Adulto , Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/fisiopatologia , Arritmias Cardíacas/diagnóstico , Pessoa de Meia-Idade , Adulto Jovem , Ventrículos do Coração/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/epidemiologia , Estudos Retrospectivos , Fatores de Tempo , Coração Univentricular/cirurgia , Coração Univentricular/epidemiologia , Coração Univentricular/fisiopatologia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Fatores de RiscoRESUMO
BACKGROUND: Childhood cancer survivors are at increased risk of developing cardiovascular diseases, presenting as the main causes of morbidity and mortality within this group. Besides the usual primary and secondary prevention in combination with screening during follow-up, the modifiable lifestyle factors of physical activity, nutrition, and body weight have not yet gained enough attention regarding potential cardiovascular risk reduction. OBJECTIVE: These practical recommendations aim to provide summarised information and practical implications to paediatricians and health professionals treating childhood cancer survivors to reduce the risk of cardiovascular late effects. METHODS: The content derives from either published guidelines or expert opinions from Association of European Paediatric and Congenital Cardiology working groups and is in accordance with current state-of-the-art. RESULTS: All usual methods of prevention and screening regarding the risk, monitoring, and treatment of occurring cardiovascular diseases are summarised. Additionally, modifiable lifestyle factors are explained, and clear practical implications are named. CONCLUSION: Modifiable lifestyle factors should definitely be considered as a cost-effective and complementary approach to already implemented follow-up care programs in cardio-oncology, which can be actively addressed by the survivors themselves. However, treating physicians are strongly encouraged to support survivors to develop and maintain a healthy lifestyle, including physical activity as one of the major influencing factors. This article summarises relevant background information and provides specific practical recommendations on how to advise survivors to increase their level of physical activity.
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Sobreviventes de Câncer , Cardiologia , Doenças Cardiovasculares , Cardiopatias Congênitas , Insuficiência Cardíaca , Neoplasias , Criança , Humanos , Adulto , Cardiopatias Congênitas/complicações , Doenças Cardiovasculares/prevenção & controle , Neoplasias/complicações , Exercício Físico , Estilo de VidaRESUMO
An observational retrospective study was conducted on 180 young competitive athletes. These children were revaluated after a mild/moderate SARS-CoV-2 infection through 3 different kind of protocol aimed at recognizing any cardiorespiratory complications due to the infection. The aim of the present study was to evaluate the results of "return to play" Italian protocols for readmission to competitive sport in the pediatric population. All of the subjects analyzed were readmitted to competitive sports after revaluation. None of the young athletes showed cardiorespiratory compromises at first and/or second level evaluation, confirming that in young population there is low risk of hospitalization and life-threatening complications after a mild/moderate infection. Italian simplified protocol for resuming sport have proved to be a valuable tool for health assessment both in adult and young athletes allowing them to resume their training in safety.
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COVID-19 , Adulto , Criança , Humanos , Atletas , COVID-19/epidemiologia , Pandemias , Estudos Retrospectivos , Volta ao Esporte , SARS-CoV-2RESUMO
OBJECTIVES: To assess the effect of a dedicated pediatric syncope unit on the diagnostic and therapeutic management of children with suspected syncope. We also evaluated the effectiveness of the pediatric syncope unit model in decreasing unnecessary tests and hospitalizations, minimizing social costs, and improving diagnostic yield. STUDY DESIGN: This single-center cohort observational, prospective study enrolled 2278 consecutive children referred to Bambino Gesù Children's Hospital from 2012 to 2017. Characteristics of the study population, number and type of admission examinations, and diagnostic findings before the pediatric syncope unit was implemented (2012-2013) and after the pediatric syncope unit was implemented (2014-2015 and 2016-2017) were compared. RESULTS: The proportion of undefined syncope, number of unnecessary diagnostic tests performed, and number of hospital stay days decreased significantly (P < .0001), with an overall decrease in costs. A multivariable logistic regression analysis, adjusted for confounding variables (age, sex, number of diagnostic tests), the period after pediatric syncope unit (2016-2017) resulted as the best independent predictor of effectiveness for a defined diagnosis of syncope (P < .0001). CONCLUSIONS: Pediatric syncope unit organization with fast-tracking access more appropriate diagnostic tests is effective in terms of accuracy of diagnostic yield and reduction of costs.
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Técnicas de Diagnóstico Cardiovascular/economia , Custos Hospitalares , Unidades Hospitalares/economia , Síncope/diagnóstico , Adolescente , Criança , Feminino , Seguimentos , Humanos , Incidência , Itália/epidemiologia , Tempo de Internação/economia , Tempo de Internação/tendências , Masculino , Estudos Prospectivos , Síncope/economia , Síncope/epidemiologiaRESUMO
OBJECTIVE: To determine whether bariatric surgery is effective for the treatment of nonalcoholic steatohepatitis (NASH) in adolescence, we compared the efficacy of laparoscopic sleeve gastrectomy (LSG) with that of lifestyle intervention (nonsurgical weight loss [NSWL]) for NASH reversal in obese adolescents. STUDY DESIGN: Obese (body mass index ≥ 35 kg/m2) adolescents (13-17 years of age) with biopsy-proven NAFLD underwent LSG, lifestyle intervention plus intragastric weight loss devices (IGWLD), or only NSWL. At baseline and 1 year after treatment, patients underwent clinical and psychosocial evaluation, blood tests, liver biopsy, polysomnography, and 24-hour ambulatory blood pressure estimation. RESULTS: Twenty patients (21%) underwent LSG, 20 (21%) underwent IGWLD, and 53 (58%) received lifestyle intervention alone (NSWL). One year after treatment, patients who underwent LSG lost 21.5% of their baseline body weight, whereas patients who underwent IGWLD lost 3.4%, and patients who underwent NSWL increase 1.7%. In patients who underwent LSG, NASH reverted completely in all patients and hepatic fibrosis stage 2 disappeared in 18 patients (90%). After IGWLD, NASH reverted in 6 patients (24%) and fibrosis in 7 (37%). Patients who received the NSWL intervention did not improve significantly. Hypertension resolved in all patients who underwent LSG with preoperative hypertension (12/12) versus 50% (4/8) of the patients who underwent IGWLD (P = .02). The cohort-specific changes in impaired glucose metabolism were similar: 100% (9/9) of affected patients who underwent LSG versus 50% (1/2) of patients who underwent IGWLD (P = .02). LSG was also more affective in resolving dyslipidemia (55% [7/12] vs 26% [10/19]; P = .05) and sleep apnea (78% [2/9] vs 30% [11/20]; P = .001). CONCLUSION: LSG was more effective than lifestyle intervention, even when combined with intragastric devices, for reducing NASH and liver fibrosis in obese adolescents after 1 year of treatment.
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Cirurgia Bariátrica , Gastrectomia/métodos , Laparoscopia , Cirrose Hepática/etiologia , Hepatopatia Gordurosa não Alcoólica/etiologia , Obesidade Infantil/complicações , Obesidade Infantil/cirurgia , Adolescente , Feminino , Humanos , Estilo de Vida , Masculino , Obesidade Infantil/terapia , Estudos Prospectivos , Resultado do TratamentoRESUMO
BACKGROUND: Takotsubo cardiomyopathy (TC) is characterized by a transient decrease in ejection fraction and a reversible left ventricular dysfunction. The pathophysiology of TC is not completely understood. Heterogeneous and multifactorial mechanisms are involved: drugs, emotional and physical stress, genetic and hormonal factors. CASE PRESENTATION: A 17 year-old male with metastatic pancreatic adenocarcinoma, under chemotherapy containing 5-fluorouracil, presented severe left ventricular dysfunction requiring mechanical ventilation and inotropes administration. He completely recovered in 2 weeks. CONCLUSION: To our knowledge this is the first report of transient form of ventricular dysfunction, mimicking TC, in an adolescent. We believe that children and adolescents receiving 5-fluorouracil should be closely monitored and referred for investigation if they develop cardiac symptoms.
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Adenocarcinoma/tratamento farmacológico , Antimetabólitos Antineoplásicos/efeitos adversos , Fluoruracila/efeitos adversos , Neoplasias Pancreáticas/tratamento farmacológico , Cardiomiopatia de Takotsubo/induzido quimicamente , Adenocarcinoma/complicações , Adolescente , Antimetabólitos Antineoplásicos/uso terapêutico , Fluoruracila/uso terapêutico , Humanos , Masculino , Neoplasias Pancreáticas/complicações , Cardiomiopatia de Takotsubo/diagnósticoRESUMO
OBJECTIVE: To evaluate the impact of the 2009 Italian pediatric clinical guidelines on the management of syncope. STUDY DESIGN: A retrospective study of patients who presented to the Emergency Department (ED) of Bambino Gesù Children's Hospital with syncope during the 2 years before and then for 2 years after the establishment of the Italian pediatric clinical guidelines. Implementation of the clinical guidelines included educational seminars, additional training of health care workers, and the availability of clinical guidelines and its algorithms on ED examination rooms. RESULTS: We studied a population of 1073 patients (n = 470 vs n = 603). Most patients had neurocardiogenic syncope with a greater increase in postimplementation period (n = 241, 51.3% vs n = 454, 49.8%); we also noticed a parallel reduction of the number of patients with undefined syncope (30% vs 8.3%). We observed an increase in electrocardiogram (n = 328, 69.8% vs n = 512, 85.1%; P < .001), a reduction in electroencephalogram (n = 54, 11.5% vs n = 25, 4.1%; P < .001), and computed tomography scan/magnetic resonance imaging utilization (n = 26, 5.5% vs n = 8, 1.3%; P < .001). In addition, there was a significant reduction of hospital admission rates (n = 195; 41.5% vs n = 116, 19.2%; P < .001). The time period was significantly associated with improvements in all procedures at the multivariate analysis. CONCLUSIONS: Providing practitioners in the ED with age-oriented clinical guidelines increased the efficiency of clinical management of pediatric syncope. Our study demonstrated that the implementation of pediatric clinical guidelines on syncope improve diagnosis, reduce hospital admissions, and decrease the use of unnecessary diagnostic tests.
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Técnicas e Procedimentos Diagnósticos/estatística & dados numéricos , Serviço Hospitalar de Emergência/estatística & dados numéricos , Fidelidade a Diretrizes/estatística & dados numéricos , Hospitalização/estatística & dados numéricos , Guias de Prática Clínica como Assunto , Síncope/diagnóstico , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Itália , Masculino , Estudos RetrospectivosRESUMO
UNLABELLED: The aim of this study was to analyse the relationship between insulin-glucose metabolism, nocturnal blood pressure dipping and nonalcoholic fatty liver disease (NAFLD) in obese adolescents without diabetes. One hundred one consecutive children, with biopsy-proven NAFLD, were included in this study. Blood samples were drawn for the analyses of liver function tests, insulin-glucose metabolism and lipid profile appraisal. An ambulatory blood pressure measurement (ABPM) was performed. Seventy-six children (75.3 %) were systolic nondippers, and 23 of them were diastolic nondippers (30.3 %). No differences were found in the anthropometric parameters between the two groups. When compared to the systolic dippers, the systolic nondippers had higher medians of mean nocturnal blood pressure, glucose at 0, 60 and 120 min in the oral glucose tolerance test (OGTT), OGTT insulin at all time points and insulin-resistance values. No correlation of histopathological features with dipping/nondipping statuses was found. CONCLUSIONS: We found an association between a nocturnal blood pressure fall and measures of insulin levels, independent of obesity, or daytime blood pressure levels, among the obese patients with NAFLD. Although no association between nondipping profiles and NAFLD was observed in our study, further studies with a longer term follow-up are needed, to better elucidate the complex link between these particular entities.
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Pressão Sanguínea/fisiologia , Resistência à Insulina/fisiologia , Hepatopatia Gordurosa não Alcoólica/fisiopatologia , Obesidade Infantil/fisiopatologia , Adolescente , Antropometria , Glicemia , Determinação da Pressão Arterial , Monitorização Ambulatorial da Pressão Arterial , Criança , Feminino , Teste de Tolerância a Glucose , Humanos , Masculino , Estudos ProspectivosRESUMO
Despite notable advancements in immunotherapy in the past decades, allogeneic hematopoietic stem cell transplantation (allo-HCT) remains a promising, potentially curative treatment modality. Only a limited number of studies have performed a direct comparison of two prevalent rabbit anti-thymocyte globulin (r-ATG) formulations-specifically, Thymoglobuline (ATG-T, formerly Genzyme) and Grafalon (ATG-G, formerly Fresenius). The primary objective of our retrospective analysis was to compare the outcomes of adult patients undergoing matched or mismatched unrelated donor (MUD/MMUD) allo-HCT, with a graft-versus-host disease (GvHD) prophylaxis based on either ATG-T or ATG-G. A total of 87 patients who had undergone allo-HCT between 2012 and 2022 were included. We observed no significant differences between ATG-T and ATG-G concerning the occurrence of acute graft-versus-host disease (aGvHD), regardless of its severity. Conversely, chronic graft-versus-host disease (cGvHD) occurred less frequently in the ATG-T group compared to the ATG-G group (7.5% vs. 38.3%, p = 0.001). The negative impact of ATG-G on cGvHD was confirmed by multivariate analysis (HR 8.12, 95% CI 2.06-32.0, p = 0.003). Patients treated with ATG-T manifested a higher incidence of cytomegalovirus (CMV) reactivations (70% vs. 31.9%, p < 0.001), with a shorter time between transplant and CMV (<61 days, 77.8% vs. 33.3%, p = 0.008) and a higher median CMV copy number (1000 vs. 0, p = 0.004). Notably, despite a higher occurrence of CMV reactivations in the ATG-T cohort, most patients were asymptomatic compared to ATG-G (85.7% vs. 43.8%, p = 0.005). By multivariate analysis, only aGvHD had an influence on CMV reactivations (HR 0.18, 95% CI 0.04-0.75, p = 0.019). Finally, we observed no significant differences in terms of 5-year overall survival (OS) and 3-year relapse-free survival (RFS) while comparing ATG-T and ATG-G (32.0% vs. 40.3%, p = 0.423; 66.7% vs. 60.4%, p = 0.544, respectively).
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Allogeneic hematopoietic cell transplantation (allo-HSCT) stands as an effective treatment method for various hematologic malignancies. However, graft-versus-host disease (GvHD), an intricate immunological phenomenon where donor immune cells target recipient tissues, remains a significant challenge, particularly in mismatched unrelated donors (MMUD). Post-transplant cyclophosphamide (PTCy) has emerged as a promising immunosuppressive strategy, revolutionizing haploidentical transplantation and demonstrating promise in MMUD settings. Background/Objectives: This study aimed to evaluate the impact of PTCy on MMUD allo-HSCT outcomes, specifically its effects on GvHD incidence and overall survival, compared to anthitymocyte globulin (ATG). Methods: One hundred seventy-four patients were classified into three groups based on the type of transplantation: PTCy-haplo (114/174; 65.5%), PTCy-MMUD (23/174; 13.2%), and ATG-MMUD (37/174; 21.2%). Results: Our findings showed that PTCy-MMUD significantly reduced acute GvHD occurrence compared to PTCy-haplo and ATG-MMUD approaches (p = 0.006). The delayed onset of acute GvHD in the PTCy-MMUD group suggests a more controlled immune reconstitution, contributing to the lower incidence. Importantly, PTCy-MMUD exhibited enhanced five-year overall survival rates, aligning with the notion that reduced GvHD correlates with improved patient outcomes (p = 0.032). Conclusions: We believe that this study contributes valuable insights into PTCy-MMUD's management, underscoring its potential to significantly reduce GvHD incidence and enhance survival outcomes. Although further investigations and clinical trials are warranted, this research underscores the promising role of PTCy-based GvHD prophylaxis in improving MMUD allo-HCT success.
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BACKGROUND: Despite a successful repair of tetralogy of Fallot (rToF) in childhood, residual lesions are common and can contribute to impaired exercise capacity. Although both cycle ergometer and treadmill protocols are often used interchangeably these approaches have not been directly compared. In this study we examined cardiopulmonary exercise test (CPET) measurements in rToF. METHODS: Inclusion criteria were clinically stable rToF patients able to perform a cardiac magnetic resonance imaging (CMR) and two CPET studies, one on the treadmill (incremental Bruce protocol) and one on the cycle ergometer (ramped protocol), within 12 months. Demographic, surgical and clinical data; functional class; QRS duration; CMR measures; CPET data and international physical activity questionnaire (IPAQ) scores of patients were collected. RESULTS: Fifty-seven patients were enrolled (53% male, 20.5 ± 7.8 years at CPET). CMR measurements included a right ventricle (RV) end-diastolic volume index of 119 ± 22 mL/m2, a RV ejection fraction (EF) of 55 ± 6% and a left ventricular (LV) EF of 56 ± 5%. Peak oxygen consumption (VO2)/Kg (25.5 ± 5.5 vs. 31.7 ± 6.9; p < 0.0001), VO2 at anaerobic threshold (AT) (15.3 ± 3.9 vs. 22.0 ± 4.5; p < 0.0001), peak O2 pulse (10.6 ± 3.0 vs. 12.1± 3.4; p = 0.0061) and oxygen uptake efficiency slope (OUES) (1932.2 ± 623.6 vs. 2292.0 ± 639.4; p < 0.001) were significantly lower on the cycle ergometer compared with the treadmill, differently from ventilatory efficiency (VE/VCO2) max which was significantly higher on the cycle ergometer (32.2 ± 4.5 vs. 30.4 ± 5.4; p < 0.001). Only the VE/VCO2 slope at the respiratory compensation point (RCP) was similar between the two methodologies (p = 0.150). CONCLUSIONS: The majority of CPET measurements differed according to the modality of testing, with the exception being the VE/VCO2 slope at RCP. Our data suggest that CPET parameters should be interpreted according to test type; however, these findings should be validated in larger populations and in a variety of institutions.
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OBJECTIVE: Hepatic steatosis is strongly associated with insulin resistance, but causative mechanisms that link these conditions are still largely unknown. Nowadays, it is difficult to establish whether fatty liver is the cause of insulin resistance or instead the complex metabolic derangements of insulin resistance determine hepatic steatosis and its progression to fibrosis. In patients with familial hypobetalipoproteinemia (FHBL), hepatic steatosis is because of the genetically determined defective form of apolipoprotein B, independently of metabolic derangements. Therefore patients with FHBL represent a good in vivo model to evaluate the relationships between fatty liver and insulin sensitivity. METHODS: We evaluated insulin resistance through HOMA-IR in 60 children with echografic and histological features of steatosis; 30 of whom had nonalcoholic fatty liver disease (NAFLD) and 30 had FHBL. RESULTS: All patients had histological features of hepatic steatosis. Patients with FHBL were hypolipidemic, as expected. No significant differences between two groups were observed in liver function tests. IRI and HOMA-IR were statistically higher in NAFLD subjects compared to the FHBL group. CONCLUSION: In our study, we demonstrated that in children with FHBL, hepatic steatosis is dissociated from insulin resistance. This finding suggests that fat accumulation per se may be not a sufficient causal factor leading to insulin resistance, and that other mediators may be involved in the development of alteration in glucose metabolism and metabolic syndrome in patients with NAFLD.
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Fígado Gorduroso/metabolismo , Hipobetalipoproteinemia Familiar por Apolipoproteína B/metabolismo , Resistência à Insulina , Adolescente , Glicemia/metabolismo , Criança , Jejum/sangue , Fígado Gorduroso/sangue , Fígado Gorduroso/complicações , Feminino , Homeostase , Humanos , Hipobetalipoproteinemia Familiar por Apolipoproteína B/sangue , Hipobetalipoproteinemia Familiar por Apolipoproteína B/complicações , Insulina/sangue , Lipídeos/sangue , Testes de Função Hepática , Masculino , Síndrome Metabólica/sangue , Síndrome Metabólica/complicações , Síndrome Metabólica/metabolismo , Análise Multivariada , Hepatopatia Gordurosa não Alcoólica , Fatores de RiscoRESUMO
Hypertension and reduced HRV are frequent in heart-transplanted recipients. We studied 26 young recipients to investigate the relationship between BP and HRV during simultaneous 24-h monitoring. Presence of CAV was considered. All HRV measures were significantly lower than normal values. Significant correlations were found between mean daytime systolic BP and the rMSSD (p = 0.04), and mean daytime DBP and SDANN for all 5-min segments (p = 0.03) and between rMSSD and mean nighttime DBP (p = 0.03). Four patients were hypertensive during daytime, seven had a reduced nocturnal fall and two had a nocturnal rise in BP. Eight patients showed severe CAV grade (grade IV) on the Stanford scale, 13 moderate (grade III) and five mild (grade I-II). After a follow-up time of 30 months, four of the 13 patients (30%) with CAV grade III showed an increase to grade IV and all showed abnormalities of both HRV and ABPM patterns. The relationship between HRV abnormalities and arterial hypertension and CAV should be further explored.
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Frequência Cardíaca , Transplante de Coração/métodos , Hipertensão/diagnóstico , Hipertensão/fisiopatologia , Adolescente , Adulto , Aloenxertos , Pressão Sanguínea , Monitorização Ambulatorial da Pressão Arterial , Cateterismo Cardíaco , Criança , Ritmo Circadiano/fisiologia , Progressão da Doença , Eletrocardiografia , Feminino , Seguimentos , Coração/fisiopatologia , Humanos , Masculino , Monitorização Fisiológica/métodos , Adulto JovemRESUMO
Kidney graft failure is not a homogenous disease and the Banff classification distinguishes several types of graft rejection. The maintenance of a transplant and the treatment of its failure require specific medications and differ due to the underlying molecular mechanism. As a consequence, patients suffering from different rejection types will experience distinct side-effects upon therapy. The review is focused on comparing treatment regimens as well as presenting the latest insights into innovative therapeutic approaches in patients with an ongoing active ABMR, chronic active ABMR, chronic ABMR, acute TCMR, chronic active TCMR, borderline and mixed rejection. Furthermore, the profile of cardiovascular adverse effects in relation to the applied therapy was subjected to scrutiny. Lastly, a detailed assessment and comparison of different approaches were conducted in order to identify those that are the most and least detrimental for patients suffering from kidney graft failure.
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Allogeneic hematopoietic stem cell transplantation (allo-HCT) is a potentially curative treatment modality, frequently used for patients suffering from haematological malignancies. In the last two decades, there have been multiple randomised controlled trials (RCTs), review articles, and meta-analyses addressing the efficacy of rabbit anti-thymocyte globulin (r-ATG) as a graft-versus-host disease (GvHD) prophylaxis. Nevertheless, only a few aimed to compare the effectiveness of different r-ATG formulations. Since the last article we retrieved comparing different r-ATGs in GvHD prophylaxis dates back to 2017, we performed a systematic literature review of articles published since 2017 to this day, utilising PubMed, Scopus, Cochrane, and MEDLINE, with the main endpoints being prophylaxis of acute GvHD (aGvHD) and chronic GvHD (cGvHD). We subjected to scrutiny a total of five studies, of which four compared the differences between Thymoglobulin (ATG-T) and Grafalon (ATG-G), and one discussed the impact of ATG-T dose. Overall, cGvHD, aGvHD grades II-IV, TRM, OS, NRM, LFS, relapse, overall infections, and EBV reactivation do not seem to be affected by the type of utilised rATG. However, data on aGvHD grades III-IV, GRFS, moderate-severe cGvHD, and CMV reactivation is conflicting. Through our research, we sought to summarise the most recent findings concerning r-ATGs in allo-HCT, and provide insight into the differences between the targets and origin of various ATG formulations.
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BACKGROUND: Myeloid sarcoma (MS), also known as granulocytic sarcoma or chloroma, is a rare disease entity characterized by the emergence of an extramedullary tumor, which may be antecedent, coexisting, or manifest secondarily to an ongoing malignancy of lymphoid origin. Owing to its low prevalence, scientific reports addressing this matter comprise mainly retrospective studies with a limited number of participants, rather low-quality research, and only few case reports. Despite MS's rarity, the need for enhancing their diagnostic tools and refinement of their therapeutic regimens is broadly recognized among physicians. CASE SUMMARY: In this case series, we present the clinical histories of two patients diagnosed with MS. The former (Case 1) exhibited MS of the sternum alongside chronic myeloid leukemia (CML), while in case of the latter (Case 2) MS presented as the initial manifestation of a current acute myeloid leukemia (AML). Treatment for both patients included chemotherapy (CHTH) and radiation (RT); however, patient 1 with CML died due to cardiorespiratory insufficiency secondary to an infection, while patient 2 is in clinical remission (CR) for 16 months since their MS diagnosis. Furthermore, a comprehensive analysis of previously reported cases was conducted which incorporated MS in patients with AML and CML. CONCLUSION: The objective of this report was to emphasize the heterogeneity among the clinical manifestations of MS, to underline the relevance of the histopathological and molecular diagnostic tools in opting for the appropriate therapy, and that, in spite of it occurring rather uncommonly, physicians should think of MS in the presence of pathological masses in patients under risk of hematological malignancies.
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Anti-thymocyte globulin (ATG) and post-transplantation cyclophosphamide (PTCy) are two frequently utilised strategies in graft-versus-host disease (GvHD) prophylaxis following allogeneic hematopoietic cell transplantation (allo-HCT), currently approved for different recipient-donor settings. In addition, being efficacious in preventing GvHD owing to their T-cell depleting capacity, the employment of these two agents increases the risk of infections, including CMV reactivation, which stands as one of the most common and serious infections following allo-HCT. We performed a systematic literature review of articles published until 1 September 2023, through PubMed, MEDLINE, and Scopus, with the main endpoint being CMV reactivation after PTCy or ATG allo-HCT. The majority of the studies included in the analysis provide supporting evidence for a reduced risk of CMV reactivations following the use of PTCy compared to ATG, although not all findings reached statistical significance. Additionally, it appears that utilising a haploidentical donor leads to a higher incidence of CMV infections and clinically significant CMV infections (CS-CMVis) compared to other donor settings in PTCy allo-HCT. This study aims to compare the risk of CMV infections following allo-HCT in patients who have received either ATG or PTCy as GvHD prophylaxis and discuss other factors that could influence the infectious outcomes of patients who have undergone allo-HCT.
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Deep brain stimulation (DBS) is a relatively dated treatment procedure that emerged in the late 1980s. Nonetheless, numerous studies are being carried out to examine its influence on the human brain and develop new treatment indications. This systematic review aims to summarize the current state of knowledge referring to DBS, investigate novel insights into its indications, and discuss the technical aspects and rationale behind DBS application. In particular, we sought to subject to scrutiny the application of DBS specifically in anorexia nervosa (AN), various addiction types, depression, and obsessive-compulsive disorders (OCDs). The method is supposed to offer promising results, especially in pharmacologically resistant forms of the upper-mentioned psychiatric disorders. Moreover, further insight has been provided into the historical notions of the method and differences in the surgical approach in specific disease entities. Furthermore, we mark the possible influence of comorbidities on treatment results. Our review consists of articles and studies found on PubMed, Google Scholar, Cochrane, and Scopus, which were then analyzed with scrutiny in the identification process, including the most resourceful ones. After methodological quality and risk of bias assessment, a total of 53 studies were included. To this date, DBS's usefulness in the treatment of AN, OCDs, depression, and addictions has been proven, despite an ongoing debate concerning the technical aspects and parameters when applying DBS. To the best of our knowledge, we have not found any paper that would recapitulate the current state of DBS in the context of psychiatric disorders with an addition of technical insights.
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BACKGROUND: Advances in the medical-surgical field have significantly increased the life expectancy of patients undergoing solid organ transplantation but this exposes patients to long-term complications due to chronic therapies and changes in lifestyle. It is known that children affected by pathology tend to be more sedentary and inactivity represents a further risk factor for the onset of non-communicable diseases. The aim of the present study was to compare the lifestyle of two groups of young patients: one group of healthy subjects (HG) and one group of kidney or liver transplant recipients (TG). METHODS: Patients were asked to complete Physical Activity Questionnaire for Older Children (PAQ-C). RESULTS: A total of 104 subjects were recruited (50.9% male, mean age 12.8 ± 3.16 years old). No significant differences were observed in the final score between groups when comparing subjects based on health condition (Healthy 2.69 ± 0.65 vs. Transplant Group 2.42 ± 0.88), the intensity of sports activities (Competitive 2.82 ± 0.59 vs. Not Competitive 2.53 ± 0.7) or type of transplant (Liver 2.51 ± 0.91 vs. Kidney 2.16 ± 0.75). CONCLUSION: The results of this study showed a worrying reality: children are engaged in low levels of physical activity regardless of their health status and in general the level of activity does not reach the recommended values even in the absence of contraindications. So, it is necessary to encourage healthy children to practice more PA and to introduce PA prescriptions for transplanted children to prevent their health from deteriorating due to sedentariness.
RESUMO
Multisystem inflammatory syndrome in children (MIS-C) is a serious health condition that imposes a long-term follow-up. The purpose of our pilot study is to evaluate the usefulness of the cardiopulmonary stress test (CPET) in the follow-up after MIS-C. All patients admitted for MIS-C in our hospital in the 12 months preceding the date of observation were considered for inclusion in the study. Pre-existing cardio-respiratory diseases and/or the lack of collaboration were the exclusion criteria. At enrolment, each subject passed a cardiological examination, rest ECG, echocardiogram, 24 h Holter-ECG, blood tests, and a CPET complete of spirometry. A total of 20 patients met the inclusion criteria (11.76 ± 3.29 years, 13 male). In contrast to the normality of all second-level investigations, CPET showed lower-than-expected peakVO2 and peak-oxygen-pulse values (50% of cases) and higher-than-expected VE/VCO2-slope values (95% of cases). A statistically significant inverse correlation was observed between P-reactive-protein values at admission and peakVO2/kg values (p = 0.034), uric acid values at admission, and peakVO2 (p = 0.011) or peak-oxygen-pulse expressed as a percentage of predicted (p = 0.021), NT-proBNP values at admission and peakVO2 expressed as a percentage of predicted (p = 0.046). After MIS-C (4-12 months) relevant anomalies can be observed at CPET, which can be a valuable tool in the follow-up after this condition.