A5034550272- Transplantation: Methods and Outcomes
- Cardiomyopathy and Myosin Studies
- Congenital Heart Disease Studies
- Mechanical Circulatory Support Devices
- Cardiovascular Function and Risk Factors
- Diverse academic and cultural studies
- Cardiac Structural Anomalies and Repair
- Heart Failure Treatment and Management
- Viral Infections and Immunology Research
- Renal Transplantation Outcomes and Treatments
- Italian Literature and Culture
- Cardiovascular Effects of Exercise
- Cardiac Arrhythmias and Treatments
- Italian Fascism and Post-war Society
- Cardiac pacing and defibrillation studies
- Historical and Environmental Studies
- Congenital heart defects research
- Cardiac Valve Diseases and Treatments
- Pneumocystis jirovecii pneumonia detection and treatment
- Adenosine and Purinergic Signaling
- Pulmonary Hypertension Research and Treatments
- Urban Planning and Landscape Design
- Renaissance Literature and Culture
- Medieval European Literature and History
- Architecture, Modernity, and Design
Jackson Memorial Hospital
2008-2025
University of Miami
2012-2024
University of Milan
2014-2022
Miami Transplant Institute
2020
Wayne State University
2017-2018
Children's Hospital of Michigan
2017-2018
Cincinnati Children's Hospital Medical Center
2011-2017
Le Bonheur Children's Hospital
2017
Boston Children's Hospital
2017
Creative Commons
2016
Restrictive cardiomyopathy (RCM) has been associated with poor prognosis in childhood. The goal of the present analysis was to use Pediatric Cardiomyopathy Registry analyze outcomes childhood RCM, a focus on impact phenotype comparing pure RCM cases that have additional features hypertrophic (HCM).We analyzed database (1990-2008; N=3375) for RCM. Cases were defined as when only assigned diagnosis. Additional documentation HCM at any time used criterion RCM/HCM phenotype. accounted 4.5%...
Myocarditis is a cause of new-onset dilated cardiomyopathy phenotype in children, with small studies reporting high rates recovery left ventricular (LV) function.The presenting characteristics and outcomes children myocarditis diagnosed clinically biopsy confirmation (n=119) or probable by alone (n=253) were compared idiopathic (n=1123). Characteristics at presentation assessed as possible predictors outcomes. The distributions time to death, transplantation, echocardiographic normalization...
Pediatric dilated cardiomyopathy (DCM) is the leading indication for heart transplantation after 1 year of age. Risk factors by etiology at clinical presentation have not been determined separately death and in population-based studies. Competing risks analysis may inform patient prioritization listing.The Cardiomyopathy Registry enrolled 1731 children diagnosed with DCM from 1990 to 2007. Etiologic, demographic, echocardiographic data collected diagnosis were analyzed competing methods...
Background Pediatric cardiomyopathy is a genetically heterogeneous disease with substantial morbidity and mortality. Current guidelines recommend genetic testing in children hypertrophic, dilated, or restrictive cardiomyopathy, but practice variations exist. Robust data on clinical practices diagnostic yield are lacking. This study aimed to identify the causes of investigate practices. Methods Results Children familial idiopathic were enrolled from 14 institutions North America. Probands...
Research comparing the survival of children with familial dilated cardiomyopathy (FDCM) to that idiopathic (IDCM) has produced conflicting results.We analyzed data from FDCM or IDCM using National Heart, Lung, and Blood Institute-funded Pediatric Cardiomyopathy Registry. Compared (n=647), (n=223) were older (mean 6.2 versus 4.5 years, P<0.001), less often had heart failure (64% 78%, less-depressed mean left ventricular fractional shortening z scores (-7.85±3.98 -9.06±3.89, P<0.001) lower...
RASopathies are a clinically heterogeneous group of conditions caused by mutations in 1 16 proteins the RAS-mitogen activated protein kinase (RAS-MAPK) pathway. Recently, RIT1 were identified as novel cause for Noonan syndrome. Here we provide additional functional evidence causal role and expand associated phenotypic spectrum. We two de novo missense variants p.Met90Ile p.Ala57Gly. Both resulted increased MEK-ERK signaling compared to wild-type, underscoring gain-of-function primary...
ABSTRACT Background Short‐term outcomes following heart transplantation in children have improved, but comparable improvements long‐term survival continues to barriers. We sought investigate and identify protective risk factors associated with children. Methods The Pediatric Heart Transplant Society (PHTS) database was queried for transplant recipients from 1993 2010 who were ≤ 10 years of age at time transplant. Patients conditional graft > 3 ≥ analyzed. Survival time‐to‐event compared...
p53 influences genomic stability, apoptosis, autophagy, response to stress, and DNA damage. New p53-target genes could elucidate mechanisms through which controls cell integrity damage.DRAGO (drug-activated gene overexpressed, KIAA0247) was characterized by bioinformatics methods as well real-time polymerase chain reaction, chromatin immunoprecipitation luciferase assays, time-lapse microscopy, viability assays. Transgenic mice (94 p53(-/-) 107 p53(+/-) on a C57BL/6J background) were used...
Abstract We aimed to determine whether malignancy after pediatric HT x for ACM affects overall post‐ survival. Patients <18y listed in the PHTS database between 1993 and 2014 were compared those with DCM . A 2:1 matched cohort was also compared. Wait‐list survival, along freedom from common complications, Eighty subjects due , whereas 1985 Although wait‐list survival higher group, lower cohort. Neither difference persisted analysis. Primary cause of death group infection, which than...
Abstract: Hypertrophic obstructive cardiomyopathy (HOCM) associated with the use of tacrolimus is a rare complication liver and intestinal transplantation seen almost exclusively among pediatric patients. Reduction dosage or conversion to cyclosporin A (CsA) has been used as an effective treatment in reviewed cases. We present three transplant recipients who developed hypertrophic while under immunosuppression were treated sirolimus (Rapamycin ® ). The patients (ages 6 yr, 12 yr 11 months)...
Danon disease is a rare entity associated with the clinical triad of mental retardation, skeletal myopathy, and severe hypertrophic cardiomyopathy. We report two cases describe results cardiac magnetic resonance imaging studies that were conducted to assess pattern hypertrophy.