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1.
J Environ Manage ; 359: 121052, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38704956

RESUMO

The cement industry plays a significant role in global carbon emissions, underscoring the urgent need for measures to transition it toward a net-zero carbon footprint. This paper presents a detailed plan to this end, examining the current state of the cement sector, its carbon output, and the imperative for emission reduction. It delves into various low-CO2 technologies and emerging innovations such as alkali-activated cements, calcium looping, electrification, and bio-inspired materials. Economic and policy factors, including cost assessments and governmental regulations, are considered alongside challenges and potential solutions. Concluding with future prospects, the paper offers recommendations for policymakers, industry players, and researchers, highlighting the roadmap's critical role in achieving a carbon-neutral cement sector.


Assuntos
Carbono , Materiais de Construção , Dióxido de Carbono , Pegada de Carbono
2.
Artigo em Inglês | MEDLINE | ID: mdl-38762216

RESUMO

BACKGROUND: Few studies highlighting the critical care management of patients after heart HTx (HTx) have been published to date. This analysis provides a contemporary representation of critical care burden pre- and post-HTx, describes care differences based on pre-transplant diagnosis and outlines the impact of intensive care unit (ICU) therapies on outcomes. METHODS: Data from PC4 Collaborative Registry was analyzed for pediatric patients undergoing HTx between 08/2014-04/2022. RESULTS: 1877 HTx in 1857 patients were reported from 42 centers; 56.5% had congenital heart disease (CHD). Patients with CHD were younger, smaller, more likely male, White race, and publicly insured. Their pre-HTx ICU course was characterized by a higher need for catheterization, increased likelihood of inotropic support and mechanical ventilation with lower VAD rates. Their operative courses were significant for longer bypass and cross-clamp times. Postoperatively, CHD patients required more CPR and utilized more ICU therapies such as inotropes, ECMO and renal replacement. Those with CHD had a longer duration of ventilatory support (68.6 vs. 27.3 hours), total hospital stays (37.1 vs. 22.9 days) and higher hospital mortality (7.8% vs. 1.8%); all p<0.0001. Longer cardiopulmonary bypass and longer deep hypothermic circulatory arrest times and delayed sternal closure were independent risk factors for hospital mortality. Lastly, there was no association between total surgical volume for a center and outcomes but there was a significant relationship between center transplant volume and outcomes with higher volume centers demonstrating significantly lower mortality. CONCLUSION: A diagnosis of CHD prior to HTx is associated with a greater use of ICU specific therapies compared non-CHD cohort. Operative factors, particularly in CHD patients, are independently associated with higher hospital mortality as was low transplant volume at the center. The study provides basis for further investigating ICU and operative factors that can be modified to improve transplant outcomes.

3.
Artif Organs ; 2024 Mar 14.
Artigo em Inglês | MEDLINE | ID: mdl-38483147

RESUMO

BACKGROUND: The use of ECMO as a bridge to heart transplantation has been growing rapidly in all heart transplant recipients since the implementation of the new UNOS allocation policy; however, the impact on adult congenital heart disease (ACHD) patients is not known. METHODS: We analyzed the UNOS data (2015-2021) for ACHD patients supported with extracorporeal membrane oxygenation (ECMO) during the waitlist, before and after October 2018, to assess the impact on the waitlist and posttransplant outcomes. We compared the characteristics and outcomes of ACHD patients with or without ECMO use during the waitlist and pre- and postpolicy changes. RESULTS: A total of 23 821 patients underwent heart transplantation, and only 918 (4%) had ACHD. Out of all ACHD patients undergoing heart transplants, 6% of patients in the prepolicy era and 7.6% in the postpolicy era were on ECMO at the time of listing. Those on ECMO were younger and sicker compared to the rest of the ACHD cohort. Those on ECMO had similar profiles pre- and postpolicy change; however, there was a very significant decrease in the waitlist time [136 days (IQR 29-384) vs. 38 days (IQR 11-108), p = 0.01]. There was no difference in waitlist mortality; however, competing risk analyses showed a higher likelihood of transplantation (51% vs. 29%) and a lower likelihood of death or deterioration (31% vs. 42%) postpolicy change. Long-term outcomes posttransplant for those supported with ECMO compared to the non-ECMO cohort are similar for ACHD patients, although there was higher attrition in the first year for the ECMO cohort. CONCLUSION: The new allocation policy has resulted in shorter waitlist times and a higher likelihood of transplantation for ACHD patients supported by ECMO. However, the appropriate use of ECMO and the underuse of durable circulatory support devices in this population need further exploration.

4.
J Environ Manage ; 356: 120712, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38531127

RESUMO

This review paper provides a comprehensive analysis of cement-based solidification and immobilisation of nuclear waste. It covers various aspects including mechanisms, formulations, testing and regulatory considerations. The paper begins by emphasizing the importance of nuclear waste management and the associated challenges. It explores the mechanisms and principles in cement-based solidification, with a particular focus on the interaction between cement and nuclear waste components. Different formulation considerations are discussed, encompassing factors such as cement types, the role of additives and modifiers. The review paper also examines testing and characterisation methods used to assess the physical, chemical and mechanical properties of solidified waste forms. Then the paper addresses the regulatory considerations and compliance requirements for cement-based solidification. The paper concludes by critically elaborating on the current challenges, emerging trends and future research needs in the field. Overall, this review paper offers a comprehensive overview of cement-based solidification, providing valuable insights for researchers, practitioners and regulatory bodies involved in nuclear waste management.


Assuntos
Resíduos Radioativos , Gerenciamento de Resíduos , Gerenciamento de Resíduos/métodos , Resíduos Perigosos
5.
J Phys Chem B ; 127(50): 10912-10922, 2023 Dec 21.
Artigo em Inglês | MEDLINE | ID: mdl-38063349

RESUMO

Five molecular mesogens containing phenyl rings and thiophene are subjected to a detailed 13C NMR investigation. The first mesogen contains only phenyl rings, while the other four have thiophene with substitution at position 2 or 3. Two of these also have a spacer inserted between the thiophene and the rest of the core unit. The mesophase properties evaluated by complementary techniques reveal an enantiotropic nematic phase for all the cases and smectic C as well as Crystal J for a few mesogens. In addition to solution 13C NMR, the samples were studied using 1D and 2D solid-state 13C NMR experiments in the liquid crystalline phase. The chemical shifts and 13C-1H dipolar couplings obtained in the mesophase provided cutting-edge information about the molecular structure and orientation of the thiophene mesogens. Accordingly, dramatic differences in these parameters are noted for the mesogens, and consequently, the identification of 2- and 3-substituted thiophene mesogens is accomplished by a simple visual examination of the 2D spectra. Furthermore, for mesogens with a spacer between thiophene and the rest of the core, 13C chemical shifts and 13C-1H dipolar couplings showed remarkable variation, which was directly reflected in the order parameters. For instance, the order parameter (Szz) of thiophene in 2- and 3-substituted mesogens in which the spacer is absent is ∼0.63 whereas for those with spacer, it is reduced to ∼0.14-0.18. In comparison, the mesogen in which the core unit is made up of phenyl rings alone that is used to benchmark the characteristics of thiophene ordering showed an order parameter of ∼0.85. The study unambiguously demonstrates the supremacy of 13C NMR in extracting the structural and orientational information on mesogens in which thiophene is a constituent of the core unit.

6.
Cureus ; 15(9): e45595, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37868379

RESUMO

Despite improvements in the medical management of persistent pulmonary hypertension of the newborn (PPHN), a significant number of patients persist with inadequate gas exchange and are treated with extracorporeal membrane oxygenation (ECMO). Prolonged time to weaning ECMO can increase mortality risk. Therefore, multiple therapies are utilized for pulmonary hypertension treatment, including pharmacotherapy with pulmonary vasodilators, to improve the prognosis of these critical patients. We report a case of a 37 2/7-week neonate with severe PPHN refractory to triple pulmonary vasodilator therapy (inhaled nitric oxide (iNO), sildenafil, and milrinone) and required veno-venous (VV)-ECMO support to improve oxygenation. Our patient was successfully weaned from ECMO after the addition of inhaled epoprostenol (iEPO) therapy. This report indicates that inhaled prostacyclin therapy effectively helps refractory PPHN patients off extracorporeal life support (ECLS) and should be considered a valuable treatment.

7.
Paediatr Drugs ; 25(6): 695-707, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37639193

RESUMO

The number of childhood cancer survivors is increasing rapidly. According to American Association for Cancer Research, there are more than 750,000 childhood cancer survivors in the United States and Europe. As the number of childhood cancer survivors increases, so does cancer treatment-related cardiac dysfunction (CTRCD), leading to heart failure (HF). It has been reported that childhood cancer survivors who received anthracyclines are 15 times more likely to have late cancer treatment-related HF and have a 5-fold higher risk of death from cardiovascular (CV) disease than the general population. CV disease is the leading cause of death in childhood cancer survivors. The increasing need to manage cancer survivor patients has led to the rapid creation and adaptation of cardio-oncology. Cardio-oncology is a multidisciplinary science that monitors, treats, and prevents CTRCD. Many guidelines and position statements have been published to help diagnose and manage CTRCD, including those from the American Society of Clinical Oncology, the European Society of Cardiology, the Canadian Cardiovascular Society, the European Society of Medical Oncology, the International Late Effects of Childhood Cancer Guideline Harmonization Group, and many others. However, there remains a gap in identifying high-risk patients likely to develop cardiomyopathy and HF in later life, thus reducing primary and secondary measures being instituted, and when to start treatment when there is echocardiographic evidence of left ventricular (LV) dysfunctions without symptoms of HF. There are no randomized controlled clinical trials for treatment for CTRCD leading to HF in childhood cancer survivors. The treatment of HF due to cancer treatment is similar to the guidelines for general HF. This review describes the latest pharmacologic therapy for preventing and treating LV dysfunction and HF in childhood cancer survivors based on expert consensus guidelines and extrapolating data from adult HF trials.


Assuntos
Sobreviventes de Câncer , Cardiopatias , Insuficiência Cardíaca , Neoplasias , Disfunção Ventricular Esquerda , Adulto , Humanos , Criança , Neoplasias/complicações , Neoplasias/tratamento farmacológico , Canadá , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/tratamento farmacológico , Cardiopatias/complicações , Disfunção Ventricular Esquerda/complicações , Disfunção Ventricular Esquerda/diagnóstico , Disfunção Ventricular Esquerda/tratamento farmacológico
8.
J Cardiovasc Dev Dis ; 10(4)2023 Apr 19.
Artigo em Inglês | MEDLINE | ID: mdl-37103057

RESUMO

Cardiopulmonary exercise testing (CPET) is the clinical standard for children with congenital heart disease (CHD), heart failure (HF) being assessed for transplantation candidacy, and subjects with unexplained dyspnea on exertion. Heart, lung, skeletal muscle, peripheral vasculature, and cellular metabolism impairment frequently lead to circulatory, ventilatory, and gas exchange abnormalities during exercise. An integrated analysis of the multi-system response to exercise can be beneficial for differential diagnosis of exercise intolerance. The CPET combines standard graded cardiovascular stress testing with simultaneous ventilatory respired gas analysis. This review addresses the interpretation and clinical significance of CPET results with specific reference to cardiovascular diseases. The diagnostic values of commonly obtained CPET variables are discussed using an easy-to-use algorithm for physicians and trained nonphysician personnel in clinical practice.

9.
J Biomed Inform ; 139: 104303, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36736449

RESUMO

Expert microscopic analysis of cells obtained from frequent heart biopsies is vital for early detection of pediatric heart transplant rejection to prevent heart failure. Detection of this rare condition is prone to low levels of expert agreement due to the difficulty of identifying subtle rejection signs within biopsy samples. The rarity of pediatric heart transplant rejection also means that very few gold-standard images are available for developing machine learning models. To solve this urgent clinical challenge, we developed a deep learning model to automatically quantify rejection risk within digital images of biopsied tissue using an explainable synthetic data augmentation approach. We developed this explainable AI framework to illustrate how our progressive and inspirational generative adversarial network models distinguish between normal tissue images and those containing cellular rejection signs. To quantify biopsy-level rejection risk, we first detect local rejection features using a binary image classifier trained with expert-annotated and synthetic examples. We converted these local predictions into a biopsy-wide rejection score via an interpretable histogram-based approach. Our model significantly improves upon prior works with the same dataset with an area under the receiver operating curve (AUROC) of 98.84% for the local rejection detection task and 95.56% for the biopsy-rejection prediction task. A biopsy-level sensitivity of 83.33% makes our approach suitable for early screening of biopsies to prioritize expert analysis. Our framework provides a solution to rare medical imaging challenges currently limited by small datasets.


Assuntos
Insuficiência Cardíaca , Transplante de Coração , Humanos , Criança , Diagnóstico por Imagem , Aprendizado de Máquina , Medição de Risco , Complicações Pós-Operatórias
10.
Children (Basel) ; 10(1)2023 Jan 12.
Artigo em Inglês | MEDLINE | ID: mdl-36670699

RESUMO

It is disheartening for parents to discover that their children have long-term cardiac dysfunction after being cured of life-threatening childhood cancers. As the number of childhood cancer survivors increases, early and late oncology-therapy-related cardiovascular complications continues to rise. It is essential to understand that cardiotoxicity in childhood cancer survivors is persistent and progressive. A child's cancer experience extends throughout his lifetime, and ongoing care for long-term survivors is recognized as an essential part of the cancer care continuum. Initially, there was a lack of recognition of late cardiotoxicities related to cancer therapy. About 38 years ago, in 1984, pioneers like Dr. Lipshultz and others published anecdotal case reports of late cardiotoxicities in children and adolescents exposed to chemotherapy, including some who ended up with heart transplantation. At that time, cardiac tests for cancer survivors were denied by insurance companies because they did not meet appropriate use criteria. Since then, cardio-oncology has been an emerging field of cardiology that focuses on the early detection of cancer therapy-related cardiac dysfunction occurring during and after oncological treatment. The passionate pursuit of many healthcare professionals to make life better for childhood cancer survivors led to more than 10,000 peer-reviewed publications in the last 40 years. We synthesized the existing evidence-based practice and described our experiences in this review to share our current method of surveillance and management of cardiac dysfunction related to cancer therapy. This review aims to discuss the pathological basis of cancer therapy-related cardiac dysfunction and heart failure, how to stratify patients prone to cardiotoxicity by identifying modifiable risk factors, early detection of cardiac dysfunction, and prevention and management of heart failure during and after cancer therapy in children. We emphasize serial longitudinal follow-ups of childhood cancer survivors and targeted intervention for high-risk patients. We describe our experience with the new paradigm of cardio-oncology care, and collaboration between cardiologist and oncologist is needed to maximize cancer survival while minimizing late cardiotoxicity.

11.
Clin Transplant ; 37(1): e14836, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36259556

RESUMO

OBJECTIVES: Induction therapy has been increasingly used in pediatric heart transplantation. This study evaluated the impact of anti-thymocyte globulin (ATG) versus basiliximab as induction therapy on post-transplant cytomegalovirus (CMV) infection, rejection at 1 year, coronary allograft vasculopathy (CAV), and mortality in pediatric heart transplant recipients receiving antiviral prophylaxis. RESULTS: Of the 96 patients (age < 18 years) analyzed, 46 (47.9%) patients received basiliximab, and 50 (52.1%) received ATG. Median follow-up was 3.0 (IQR, 1.7-4.9) years with 32.3% reporting CMV infection. The ATG group, as compared with the basiliximab group, had similar incidences of CMV infection (36% vs. 28.3%, p = .418), CMV viremia (22% vs. 19.6%, p = .769), and CMV-positive tissue biopsy (30% vs. 22%, p = .486). The ATG group had lower incidences of rejection at 1 year (16% vs. 36.9%, p = .022) and CAV (4% vs. 23.9%, p = .006) with no difference in mortality (8% vs. 15.2%, p = .343), compared with the basiliximab group. Multivariate analysis showed that induction with ATG was associated with a lower risk of rejection at 1 year (OR, .31; 95% CI, .09-.94; p = .039) with no impact on the incidences of CMV infection (HR, 2.06; 95% CI, .54-7.89; p = .292), CAV (HR, .30; 95% CI, .04-2.58; p = .275), and mortality (HR, .39; 95% CI, .09-1.82; p = .233) compared to basiliximab induction. DISCUSSION AND CONCLUSIONS: In conclusion, induction with ATG was associated with reduction in risk of rejection at 1 year with no effects on CMV infection, CAV, and mortality in pediatric heart transplant recipients with universal antiviral prophylaxis compared with basiliximab induction therapy.


Assuntos
Infecções por Citomegalovirus , Transplante de Coração , Humanos , Criança , Adolescente , Basiliximab/uso terapêutico , Imunossupressores/uso terapêutico , Quimioterapia de Indução , Rejeição de Enxerto/tratamento farmacológico , Rejeição de Enxerto/etiologia , Rejeição de Enxerto/epidemiologia , Infecções por Citomegalovirus/tratamento farmacológico , Infecções por Citomegalovirus/etiologia , Infecções por Citomegalovirus/prevenção & controle , Soro Antilinfocitário/uso terapêutico , Antivirais/uso terapêutico , Transplante de Coração/efeitos adversos , Transplantados , Estudos Retrospectivos
12.
Clin Pediatr (Phila) ; 62(1): 8-16, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-35801262

RESUMO

We aimed to study the disparity in the clinical profile and outcomes of hospitalized Multisystem Inflammatory Syndrome in Children (MIS-C) patients at our center. The second goal was to examine the temporal association with preceding SARS-CoV-2 infection by race/ethnicity in our community in Mississippi. We found the racial disparity in the prevalence of MIS-C exceeded its temporal association with SARS-CoV-2 infections. We included 51 consecutive MIS-C patients hospitalized, whose median age was 9 (interquartile range [IQR] 5-12) years, 58% were male, 71% were black, 25% were white, and 4% belonged to other groups. We found a delay between onset of symptoms and hospitalization in black patients compared with white patients with a median of 2 (IQR 0-7) vs median of 0 (0-5) urgent care visits (P = .022), respectively. Black patients were hospitalized longer (median 8, IQR 2-39 days) than whites (median 5, IQR 3-14 days), P = .047. A total of 38.9% of blacks and 23.1% of whites were admitted to intensive care unit (P = .498); 36.1% of blacks had severe cardiac involvement vs 23.1% of white patients, P = .531. Future studies of MIS-C are required to improve health equity for children.


Assuntos
COVID-19 , SARS-CoV-2 , Criança , Pré-Escolar , Feminino , Humanos , Masculino , COVID-19/complicações , COVID-19/epidemiologia , Etnicidade , Mississippi/epidemiologia
13.
Clin Transplant ; 37(3): e14843, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36494889

RESUMO

AIMS: We analyzed the impact of the revised pediatric heart allocation policy on types of ventricular assist device (VAD) utilization, and waitlist (WL) and post-heart transplant (HT) survival outcomes in congenital heart disease (CHD) versus non-CHD patients before (Era-1) and after (Era-2) pediatric heart allocation policy implementation. METHODS: We retrospectively reviewed the UNOS database from December 16, 2011, through March 31, 2021, for patients < 18 years old and listed for primary HT. We compared the differences observed between Era-1 and Era-2. RESULTS: 5551 patients were listed for HT, of whom 2447(44%) were in Era-1 and 3104(56%) were in Era-2. CHD patients were listed as status 1A unchanged, but the number of patients listed as status 1B decreased in Era-2, whereas the number of non-CHD patients listed as status 1A decreased, but status 1B increased. In Era-2 compared to Era-1, both temporary (1% to 4%, p < .001) and durable VAD (13.6% to 17.8%, p < .001) utilization increased, and the transplantation rate per 100-patient years increased in both groups. The median WL period for CHD patients increased marginally from 70 to 71 days (p = .06), whereas for non-CHD patients it decreased from 61 to 54 days (p < .001). Adjusted 90-day WL survival increased from 84% to 88%, p = .016 in CHD, but there was no significant change in non-CHD patients (p = .57). There was no significant difference in 1-year post-HT survival in CHD and non-CHD patients between Era-1 and Era-2. CONCLUSIONS: In summary, after the revised heart allocation policy implementation, temporary and durable VAD support increased, HT rate increased, waitlist duration marginally increased in the CHD cohort and decreased in the non-CHD cohort, and 90-day WL survival probability improved in children with CHD without significant change in 1-year post-HT outcomes. Future studies are needed to identify changes to the policy that may further improve the listing criteria to improve WL duration and post-HT survival.


Assuntos
Cardiopatias Congênitas , Insuficiência Cardíaca , Transplante de Coração , Coração Auxiliar , Humanos , Criança , Adolescente , Estudos Retrospectivos , Políticas , Listas de Espera
14.
ASAIO J ; 69(2): 210-217, 2023 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-35438653

RESUMO

This retrospective study included children aged ≤18 years who had durable ventricular assist devices (VADs) as a bridge to transplantation from the United Network Organ Sharing (UNOS) database between 2011 and 2020. We evaluated 90 day waitlist mortality and 1 year posttransplant mortality after VAD implantation in children stratified by race/ethnicity: Black, White, and Others. The VAD was used in a higher proportion of Black children listed for heart transplantation (HT) (26%) versus Other (25%) versus White (22%); p < 0.01. Black children had Medicaid health insurance coverage (67%) predominantly at the time of listing for HT. There was no significant overall difference in waitlist survival among the three groups supported with VAD at the time of listing (log-rank p = 0.4). On the other hand, the 90 day waitlist mortality after the VAD implantation at listing and while listed was the lowest among Black (6%) compared with White (13%) and Other (14%) ( p < 0.01). The multivariate regression analysis showed that Other race (hazard ratio [HR], 2.29; p < 0.01), Black race (HR, 2.13; p < 0.01), use of mechanical ventilation (HR, 1.72; p = 0.01), and Medicaid insurance (HR, 1.54; p = 0.04) were independently associated with increased 1 year posttransplant mortality. In conclusion, Black children had more access to durable VAD support than White children. The 90 day waitlist mortality was significantly lower in Black children compared with White and Other after VAD implantation. However, Black and Other racial/ethnic children with VAD at transplant had higher 1 year posttransplant mortality than White children. Future studies to elucidate the reasons for these disparities are needed.


Assuntos
Insuficiência Cardíaca , Transplante de Coração , Coração Auxiliar , Estados Unidos , Humanos , Criança , Etnicidade , Estudos Retrospectivos , Modelos de Riscos Proporcionais , Listas de Espera , Insuficiência Cardíaca/cirurgia
15.
Pediatr Cardiol ; 44(3): 513-529, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-35978175

RESUMO

Diastolic dysfunction (DD) refers to abnormalities in the mechanical function of the left ventricle (LV) during diastole. Severe LVDD can cause symptoms and the signs of heart failure (HF) in the setting of normal or near normal LV systolic function and is referred to as diastolic HF or HF with preserved ejection fraction (HFpEF). Pediatric cardiologists have long speculated HFpEF in children with congenital heart disease and cardiomyopathy. However, understanding the risk factors, clinical course, and validated biomarkers predictive of the outcome of HFpEF in children is challenging due to heterogeneous etiologies and overlapping pathophysiological mechanisms. The natural history of HFpEF varies depending upon the patient's age, sex, race, geographic location, nutritional status, biochemical risk factors, underlying heart disease, and genetic-environmental interaction, among other factors. Pediatric onset HFpEF is often not the same disease as in adults. Advances in the noninvasive evaluation of the LV diastolic function by strain, and strain rate analysis with speckle-tracking echocardiography, tissue Doppler imaging, and cardiac magnetic resonance imaging have increased our understanding of the HFpEF in children. This review addresses HFpEF in children and identifies knowledge gaps in the underlying etiologies, pathogenesis, diagnosis, and management, especially compared to adults with HFpEF.


Assuntos
Insuficiência Cardíaca Diastólica , Insuficiência Cardíaca , Humanos , Criança , Volume Sistólico/fisiologia , Função Ventricular Esquerda/fisiologia , Ecocardiografia/métodos
16.
Children (Basel) ; 9(10)2022 Sep 22.
Artigo em Inglês | MEDLINE | ID: mdl-36291380

RESUMO

Patients with nephrotic syndrome (NS) are at an increased risk for thromboembolic events, such as deep venous and arterial thrombosis and pulmonary embolism (PE). In general, PE in children differs from adults in incidence, predisposition, pathophysiology, presenting symptoms, and management strategies. There is a lack of treatment guidelines for PE in children, and the management strategies are mostly extrapolated from adult data. This case report highlights the presentation of acute cor pulmonale due to massive PE associated with NS and a successful pulmonary endarterectomy that reversed the child's pulmonary hypertension and normalized right ventricular function.

17.
Children (Basel) ; 9(10)2022 Sep 29.
Artigo em Inglês | MEDLINE | ID: mdl-36291431

RESUMO

Whole exome sequencing has identified an infant girl with fulminant dilated cardiomyopathy (DCM), leading to severe acute heart failure associated with ribosomal protein large 3-like (RPL3L) gene pathologic variants. Other genetic tests for mitochondrial disorders by sequence analysis and deletion testing of the mitochondrial genome were negative. Secondary causes for DCM due to metabolic and infectious etiologies were ruled out. She required a Berlin-Excor left ventricular assist device due to worsening of her heart failure as a bridge to orthotopic heart transplantation. At three months follow-up after heart transplantation, she has been doing well. We reviewed the literature on published RPL3L-related DCM cases and their outcomes. Bi-allelic variants in RPL3L have been reported in only seven patients from four unrelated families in the literature. RPL3L is a newer and likely pathogenic gene associated with a severe form of early-onset dilated cardiomyopathy with poor prognosis necessitating heart transplantation.

18.
Infez Med ; 30(3): 344-352, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36148163

RESUMO

Multisystem Inflammatory Syndrome in Children (MIS-C), a rare condition, has been reported approximately 2-4 weeks after the onset of COVID-19 in children and adolescents, causing inflammation in multiple systems, including cardiovascular and respiratory, digestive, and central nervous systems. This condition is also known as hyperinflammatory shock, Kawasaki-like disease, and Pediatric Inflammatory Multisystem Syndrome (PIMS). The signs and symptoms include but are not limited to fever, rash, peripheral edema, gastrointestinal symptoms, conjunctivitis, and shock. Thirty-eight studies met our criteria, with a total of 5822 patients. The most affected population was between 5-18 years of age. We noted that MIS-C presented with a wide range of signs and symptoms that overlap with Kawasaki Disease, including high fever, sore throat, malaise, tachypnea, tachycardia, conjunctival injection, mucosal edema, cardiac involvement, and gastrointestinal symptoms. It causes an increase in IL-17A, IL-6, and arterial damage, a distinct difference from Kawasaki disease. The laboratory findings in MIS-C showed an increase in inflammatory markers like CRP, ESR, ferritin, leukocytes, and TNF-α. WHO stated that 23% of affected children with MIS-C had underlying conditions like chronic lung diseases, cardiovascular disease, and immunosuppression. In most affected children, aspirin and IVIG were successful, which resulted in a decrease in the inflammatory markers. We find that MIS-C is a rare, but potentially fatal pediatric complication, after COVID-19 infection. The aim of this article is to study the emerging relationship between COVID-19 and MIS-C in children and adolescents affected by this condition, to discuss the immunological mechanisms, and explore potential therapies.

19.
Ann Pediatr Cardiol ; 15(1): 44-52, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35847406

RESUMO

A patent foramen ovale (PFO) is a frequent incidental finding during echocardiography in otherwise healthy children. In most healthy children with a diagnosis of isolated incidental PFO, no further follow-up or intervention is necessary. In some children, PFO is associated with certain clinical syndromes such as cryptogenic stroke, decompression sickness, migraine, and platypnea-orthodeoxia syndrome. This review discusses PFO anatomy, diagnostic imaging, PFO-associated clinical situations, management options, and the role of PFO in certain congenital heart disease. This review also highlights the current deficiency of pediatric data guiding management of these uncommon but important PFO-associated conditions. Future multicenter randomized controlled studies are necessary to guide the management of these unique and challenging PFO-associated conditions.

20.
Children (Basel) ; 9(7)2022 Jul 16.
Artigo em Inglês | MEDLINE | ID: mdl-35884045

RESUMO

Myocarditis comprises many clinical presentations ranging from asymptomatic to sudden cardiac death. The history, physical examination, cardiac biomarkers, inflammatory markers, and electrocardiogram are usually helpful in the initial assessment of suspected acute myocarditis. Echocardiography is the primary tool to detect ventricular wall motion abnormalities, pericardial effusion, valvular regurgitation, and impaired function. The advancement of cardiac magnetic resonance (CMR) imaging has been helpful in clinical practice for diagnosing myocarditis. A recent Scientific Statement by the American Heart Association suggested CMR as a confirmatory test to diagnose acute myocarditis in children. However, standard CMR parametric mapping parameters for diagnosing myocarditis are unavailable in pediatric patients for consistency and reliability in the interpretation. The present review highlights the unmet clinical needs for standard CMR parametric criteria for diagnosing acute and chronic myocarditis in children and differentiating dilated chronic myocarditis phenotype from idiopathic dilated cardiomyopathy. Of particular relevance to today's practice, we also assess the potential and limitations of CMR to diagnose acute myocarditis in children exposed to severe acute respiratory syndrome coronavirus-2 infections. The latter section will discuss the multi-inflammatory syndrome in children (MIS-C) and mRNA coronavirus disease 19 vaccine-associated myocarditis.

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