Medicine and Health Sciences Commons^™

Gender Dysphoria In Adolescents With Ehlers-Danlos Syndrome., Jordan T. Jones, William R. Black, Christine Moser, Eric T. Rush, Lindsey Malloy-Walton

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: Ehlers-Danlos Syndrome represents a family of heritable connective tissue disorders that include joint hypermobility, tissue fragility, and skin hyperextensibility. Ehlers-Danlos Syndrome presents with clinical sequela across multiple body systems that require multidisciplinary care. Little is known about adolescents with Ehlers-Danlos Syndrome who are transgender and gender diverse. To date, there have been no reports of transgender and gender diverse youth in pediatric patients with Ehlers-Danlos Syndrome. The objective of this study was to characterize transgender and gender diverse adolescents with Ehlers-Danlos Syndrome seen in a pediatric multidisciplinary specialty clinic.

METHODS: A retrospective chart review was performed and it was …

Microrna Expression Levels Change In Neonatal Patients During And After Exposure To Cardiopulmonary Bypass., Lance Hsieh, Lan N. Tu, Alison Paquette, Quanhu Sheng, Shilin Zhao, Douglas C. Bittel, James O'Brien, Kasey Vickers, Peter Pastuszko, Vishal Nigam

Manuscripts, Articles, Book Chapters and Other Papers

Background The systemic inflammation that occurs after exposure to cardiopulmonary bypass (CPB), which is especially severe in neonatal patients, is associated with poorer outcomes and is not well understood. In order to gain deeper insight into how exposure to bypass activates inflammatory responses in circulating leukocytes, we studied changes in microRNA (miRNA) expression during and after exposure to bypass. miRNAs are small noncoding RNAs that have important roles in modulating protein levels and function of cells. Methods and Results We performed miRNA-sequencing on leukocytes isolated from neonatal patients with CPB (n=5) at 7 time points during the process of CPB, …

Association Of Acute Anti-Inflammatory Treatment With Medium-Term Outcomes For Coronary Artery Aneurysms In Kawasaki Disease., Kevin G. Friedman, Brian W. Mccrindle, Kyle Runeckles, Nagib Dahdah, Ashraf S. Harahsheh, Michael Khoury, Sean Lang, Cedric Manlhiot, Adriana H. Tremoulet, Geetha Raghuveer, Elif Seda Selamet Tierney, Pei-Ni Jone, Jennifer S. Li, Jacqueline R. Szmuszkovicz, Kambiz Norozi, Supriya S. Jain, Angela T. Yetman, Jane W. Newburger, International Kawasaki Disease Registry

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The impact of adjunctive anti-inflammatory treatment on outcomes for patients with Kawasaki disease (KD) and coronary artery aneurysms (CAAs) is unknown.

METHODS: Using data from the International KD Registry in patients with ≥ medium CAA we evaluate associations of treatment with outcomes and major adverse cardiac events (MACE).

RESULTS: Medium or large CAA was present in 527 (32%) patients. All were treated with intravenous immunoglobulin (IVIG), 70% were male, and the median age was 1.3 years (interquartile range: 0.4-4.0 years). The most common acute therapies included single IVIG alone in 243 (46%), multiple IVIG in 100 (19%), multiple IVIG …

Iv Sotalol Use In Pediatric And Congenital Heart Patients: A Multicenter Registry Study., Lindsey Malloy-Walton, Nicholas H. Von Bergen, Seshadri Balaji, Peter S. Fischbach, Jason M. Garnreiter, S Yukiko Asaki, Jeffrey P. Moak, Luis A. Ochoa, Philip M. Chang, Hoang H. Nguyen, Akash R. Patel, Christa Kirk, Ashley K. Sherman, Jennifer N. Avari Silva, J Philip Saul

Manuscripts, Articles, Book Chapters and Other Papers

Background There is limited information regarding the clinical use and effectiveness of IV sotalol in pediatric patients and patients with congenital heart disease, including those with severe myocardial dysfunction. A multicenter registry study was designed to evaluate the safety, efficacy, and dosing of IV sotalol. Methods and Results A total of 85 patients (age 1 day-36 years) received IV sotalol, of whom 45 (53%) had additional congenital cardiac diagnoses and 4 (5%) were greater than 18 years of age. In 79 patients (93%), IV sotalol was used to treat supraventricular tachycardia and 4 (5%) received it to treat ventricular arrhythmias. …

Comprehensive Evaluation Of Left Ventricular Deformation Using Speckle Tracking Echocardiography In Normal Children: Comparison Of Three-Dimensional And Two-Dimensional Approaches., Doaa Aly, Nitin Madan, Laura Kuzava, Alison Samrany, Anitha Parthiban

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Three-dimensional (3D) speckle tracking echocardiography (STE) can overcome some of the inherent limitations of two-dimensional (2D) STE; however, clinical experience is lacking. We aimed to assess and compare the feasibility, agreement, and reproducibility of left ventricular (LV) global longitudinal (GLS), and regional strain by 3D vs 2D STE in normal children.

METHODS: Healthy pediatric subjects (n = 105, age mean = 11.2 ± 5.5 years) were prospectively enrolled. Three-dimensional and 2D LV GLS, as well as regional strain in 16 myocardial segments were quantified. Bland Altman analysis, intra- class correlation coefficients (ICC), percent error and linear regression were used …

Surgical Management Of Atrial-Esophageal Fistula As A Complication Of Atrial Fibrillation Ablation., Amber Duda, Kevin Beers, Mohi Mitiek

Manuscripts, Articles, Book Chapters and Other Papers

Atrial-esophageal fistula (AEF) is a rare, but life-threatening complication of ablative treatments for atrial fibrillation. Although the incidence of this complication is low, the mortality is very high. There are many surgical approaches to this disease but we offer a novel technique to reduce the number of incisions used and provides central cannulation. It also allows for repair of both the esophagus and atrium and buttresses these repairs, which have both been shown to decrease morbidity and mortality. The technique has been successful in our three patients and can be considered as an approach to surgical management of AEF.