The ACTION VAD registry: A collective five-year experience.

Background: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) began in 2018 as a collaborative learning health system committed to improving outcomes in pediatric heart failure, including children and adults with congenital heart disease, supported with ventricular assist devices (VADs). This report describes patient and device characteristics, and outcomes through 1-year post-implant.

Methods: The ACTION VAD registry report was created from data submitted to the ACTION learning network from April 2018 to June 2023.

Survival does not differ by annual center transplant volume-A Pediatric Heart Transplant Society Registry study.

Background: There are conflicting data regarding the relationship between center volume and outcomes in pediatric heart transplantation. Previous studies have not fully accounted for differences in case mix, particularly in high-risk congenital heart disease (CHD) groups. We aimed to evaluate the relationship between center volume and outcomes using the Pediatric Heart Transplant Society (PHTS) Registry and explore how case mix may affect outcomes.

Clinical, immunohistochemical, and genetic characterization of splice-altering biallelic DES variants: Therapeutic implications.

Pathogenic variants in the DES gene clinically manifest as progressive skeletal muscle weakness, cardiomyopathy with associated severe arrhythmias, and respiratory insufficiency, and are collectively known as desminopathies. While most DES pathogenic variants act via a dominant mechanism, recessively acting variants have also been reported. Currently, there are no effective therapeutic interventions for desminopathies of any type.

Organ transplantation using COVID-19-positive deceased donors.

The COVID-19 pandemic has influenced organ transplantation decision making. Opinions regarding the utilization of coronavirus disease-2019 (COVID-19) donors are mixed. We hypothesize that COVID-19 infection of deceased solid organ transplant donors does not affect recipient survival.

Neonatal Myocardial Infarction: A Proposed Algorithm for Coronary Arterial Thrombus Management.

Background: Neonatal myocardial infarction is rare and is associated with a high mortality of 40% to 50%. We report our experience with neonatal myocardial infarction, including presentation, management, outcomes, and our current patient management algorithm.

Methods: We reviewed all infants admitted with a diagnosis of coronary artery thrombosis, coronary ischemia, or myocardial infarction between January 2015 and May 2021.

Case Report: Unmanipulated Matched Sibling Donor Hematopoietic Cell Transplantation In Congenital Athymia: A Lifesaving Therapeutic Approach When Facing a Systemic Viral Infection.

Congenital athymia can present with severe T cell lymphopenia (TCL) in the newborn period, which can be detected by decreased T cell receptor excision circles (TRECs) on newborn screening (NBS). The most common thymic stromal defect causing selective TCL is 22q11.2 deletion syndrome (22q11.

Heart Transplantation of a Preterm Infant With HLHS.

A 33-week gestation, 1.75-kg female infant with mitral stenosis/aortic atresia variant of hypoplastic left heart syndrome and severe ventriculo-coronary connections underwent surgical septectomy and bilateral pulmonary artery banding at five weeks of age (2.10 kg).

Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics.

Introduction: Liver fibrosis and cirrhosis are late complications in Fontan palliation. Liver biopsy is the gold standard. The goal of this study is to correlate transjugular liver biopsy (TJLB) in the setting of Fontan palliation with noninvasive testing and hemodynamics.

Outcome of antibody-mediated rejection compared to acute cellular rejection after pediatric heart transplantation.

Outcomes of ACR after pediatric HTx have been well described, but less has been reported on outcomes of AMR. We compared the clinical characteristics and cardiovascular outcomes (composite end-point of death, retransplantation, or allograft vasculopathy) of pediatric HTx recipients with AMR, ACR, and no rejection in a retrospective single-center study of 104 recipients. Twenty were treated for AMR; 15 were treated for ACR.

Perforation and right ventricular outflow tract stenting: Alternative palliation for infants with pulmonary atresia/ventricular septal defect.

Background: Right ventricular outflow tract (RVOT) stents have been used as palliation in patients with severe tetralogy of Fallot (TOF). Radiofrequency perforation of the RVOT has also been described in patients with pulmonary atresia (PA)/ventricular septal defect (VSD). However, RVOT stenting in conjunction with radiofrequency perforation as a means for establishing reliable pulmonary blood flow in patients with PA/VSD has not previously been reported.

Transcatheter stenting of the systemic-to-pulmonary artery shunt: A 7-year experience from a single tertiary center.

Background: Systemic-to-pulmonary artery shunt (SPS) dysfunction can be deleterious in shunt dependent patients and traditionally have undergone surgical revision. Data on transcatheter stenting of SPS is limited. We sought to evaluate feasibility, safety and outcomes of stenting SPS.

Comparison of ductal stenting versus surgical shunts for palliation of patients with pulmonary atresia and intact ventricular septum.

Objectives: We sought to compare ductal stenting (DS) to systemic-pulmonary artery shunt (SPS) in a contemporary cohort of patients having pulmonary atresia with intact ventricular septum (PAIVS).

Background: Alternative palliative strategies (interventional versus surgical) are currently available for initial palliation of ductal dependent patients with various forms of congenital heart disease. There is little data available to guide strategy selection.

Integrating adolescent substance abuse treatment with HIV services: evidence-based models and baseline descriptions.

Adolescents with substance use disorders are at high risk for contracting Human Immunodeficiency Virus (HIV)/Acquired Immunodeficiency Syndrome (AIDS) and other sexually transmitted infections (STIs). Adolescence is the period of sexual maturation that compounds the issues associated with infection transmission for this risk-taking group. Integrated treatment models for implementing HIV education, counseling, and testing is a promising approach.

MRI versus ultrasonography to assess meniscal abnormalities in acute knees.

While magnetic resonance imaging (MRI) is often considered the "gold standard" diagnostic imaging modality for detection of meniscal abnormalities, it is associated with misdiagnosis in as high as 47% of cases, is costly, and is not readily available to a large number of patients. Ultrasonographic examination of the knee has been reported to be an effective diagnostic tool for this purpose with the potential to overcome many of the shortcomings of MRI. The purpose of this study is to determine the clinical usefulness of ultrasonography for diagnosis of meniscal pathology in patients with acute knee pain and compare its diagnostic accuracy to MRI in a clinical setting.

Reduced-intensity conditioning significantly improves survival of patients with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell transplantation.

Recent experience suggests that reduced-intensity conditioning (RIC) regimens can improve the outcomes of patients with hemophagocytic lymphohistiocytosis (HLH) undergoing allogeneic hematopoietic cell transplantation (HCT). However, studies directly comparing RIC to myeloablative conditioning (MAC) regimens are lacking. Forty patients with HLH underwent allogeneic HCT between 2003-2009 at Cincinnati Children's Hospital.

Application of evidence-based practice through a float project.

Evidence-based practice must become a common, acceptable process for the bedside nurse. This paper demonstrates a process for engaging nurses in the use of evidence for direct practice. An example using the revision of a float policy is provided as a model for staff nurses to incorporate evidence in a mechanism to elicit change.

Medical and legal aspects of disability medicine.

Physicians are seeing more patients with legal issues during their visits. Physicians often refer patients to other physicians willing to address these problems. Physicians treating such patients need a thorough understanding ofthe legal system governing a claim and the implications of opinions rendered.

Protease inhibitors and cardiovascular disease: analysis of the Los Angeles County adult spectrum of disease cohort.

Since protease inhibitors (PIs) were first introduced in 1995, research has shown that use of PIs greatly improves rates of survival, while slowing HIV disease progression. However, there are concerns that use of PIs may be associated with an increased risk of cardiovascular disease (CVD). To examine the relationship between PI use and CVD among HIV-infected patients, a large retrospective/prospective observational study was conducted.

Use of TaqMan real-time reverse transcription-PCR for rapid detection, quantification, and typing of norovirus.

Noroviruses (NoVs) are the most commonly identified cause of outbreaks and sporadic cases of acute gastroenteritis. We evaluated and optimized NoV-specific TaqMan real-time reverse transcription (RT)-PCR assays for the rapid detection and typing of NoV strains belonging to genogroups GI and GII and adapted them to the LightCycler platform. We expanded the detection ability of the assays by developing an assay that detects the GIV NoV strain.

High-dose weekly AmBisome antifungal prophylaxis in pediatric patients undergoing hematopoietic stem cell transplantation: a pharmacokinetic study.

Disseminated fungal infection causes significant morbidity and mortality in children undergoing hematopoietic stem cell transplantation (HSCT). The widespread use of prophylactic oral triazoles has limitations of poor absorption, interindividual variability in metabolism, and hepatic toxicity. AmBisome (amphotericin B liposomal complex) has a better safety profile than the parent drug amphotericin B and produces higher plasma and tissue concentrations.

The effects of maxillary protraction therapy with or without rapid palatal expansion: a prospective, randomized clinical trial.

Introduction: The purpose of this controlled randomized clinical trial was to quantify the effects of maxillary protraction with or without palatal expansion.

Methods: Forty-six children aged 5 to 10 years were randomly assigned to 1 of 3 groups: (1) facemask with palatal expansion, (2) facemask without palatal expansion, and (3) observation for 12 months. Cephalometric analysis with traditional cephalometric measurements, an x-y coordinate system, and an occlusal-plane analysis were used.

Creating an international nursing practice and education workplace.

In today's global workplace, both nursing practice and education need to promote international health. Nurses from a comprehensive 373-patient-bed hospital at Cincinnati Children's Hospital Medical Center and faculty from the College of Nursing, University of Cincinnati, in southwest Ohio have successfully collaborated to develop several unique international nursing exchange programs. The goals of these programs are to increase cultural sensitivity and nursing knowledge relevant to a global community.

An automated tissue preclassification approach for telepathology: implementation and performance analysis.

Telepathology is generally defined as the use of telecommunications technologies in the practice of anatomic or surgical pathology. In the usual telepathology scenario, a remotely located pathologist views images of tissues samples in order to render a diagnosis of the biopsy. Some telepathology systems involve interactive remote control of a microscope-based imaging system which delivers diagnostic quality imagery to the remote pathologist.