Digital Commons Network^™

Rates Of Laboratory Adverse Events By Course In Paediatric Leukaemia Ascertained With Automated Electronic Health Record Extraction: A Retrospective Cohort Study From The Children’S Oncology Group, Tamara P Miller, Kelly D Getz, Yimei Li, Biniyam G Demissei, Peter C Adamson, Todd A Alonzo, Evanette Burrows, Lusha Cao, Sharon M Castellino, Marla H Daves, Brian T Fisher, Robert Gerbing, Robert W Grundmeier, Edward M Krause, Judy Lee, Philip J Lupo, Karen R Rabin, Mark Ramos, Michael E Scheurer, Jennifer J Wilkes, Lena E Winestone, Douglas S Hawkins, M Monica Gramatges, Richard Aplenc

Center for Medical Ethics and Health Policy Staff Publications

Background: Adverse events are often misreported in clinical trials, leading to an incomplete understanding of toxicities. We aimed to test automated laboratory adverse event ascertainment and grading (via the ExtractEHR automated package) to assess its scalability and define adverse event rates for children with acute myeloid leukaemia and acute lymphoblastic leukaemia.

Methods: For this retrospective cohort study from the Children's Oncology Group (COG), we included patients aged 0-22 years treated for acute myeloid leukaemia or acute lymphoblastic leukaemia at Children's Healthcare of Atlanta (Atlanta, GA, USA) from Jan 1, 2010, to Nov 1, 2018, at the Children's Hospital of Philadelphia …

Vaccine-Linked Chemotherapy With A Low Dose Of Benznidazole Plus A Bivalent Recombinant Protein Vaccine Prevents The Development Of Cardiac Fibrosis Caused By Trypanosoma Cruzi In Chronically-Infected Balb/C Mice, Victor Manuel Dzul-Huchim, Maria Jesus Ramirez-Sierra, Pedro Pablo Martinez-Vega, Miguel Enrique Rosado-Vallado, Victor Ermilo Arana-Argaez, Jaime Ortega-Lopez, Fabian Gusovsky, Eric Dumonteil, Julio Vladimir Cruz-Chan, Peter Hotez, María Elena Bottazzi, Liliana Estefania Villanueva-Lizama

Center for Medical Ethics and Health Policy Staff Publications

Background: Chagas disease (CD) is caused by Trypanosoma cruzi and affects 6-7 million people worldwide. Approximately 30% of chronic patients develop chronic chagasic cardiomyopathy (CCC) after decades. Benznidazole (BNZ), one of the first-line chemotherapy used for CD, induces toxicity and fails to halt the progression of CCC in chronic patients. The recombinant parasite-derived antigens, including Tc24, Tc24-C4, TSA-1, and TSA-1-C4 with Toll-like receptor 4 (TLR-4) agonist-adjuvants reduce cardiac parasite burdens, heart inflammation, and fibrosis, leading us to envision their use as immunotherapy together with BNZ. Given genetic immunization (DNA vaccines) encoding Tc24 and TSA-1 induce protective immunity in mice and …

Change During Cognitive And Exposure Phases Of Cognitive-Behavioral Therapy For Autistic Youth With Anxiety Disorders, Andrew G Guzick, Sophie C Schneider, Philip C Kendall, Jeffrey J Wood, Connor M Kerns, Brent J Small, Ye Eun Park, Sandra L Cepeda, Eric A Storch

Center for Medical Ethics and Health Policy Staff Publications

Objective: To evaluate improvement during the (a) cognitive and (b) exposure therapy phases of cognitive-behavioral therapy (CBT) for autistic youth with anxiety disorders.

Method: Participants were 148 autistic youth (aged 7-13; 77% male; 64% White) with clinically significant anxiety who were randomized to standard or adapted CBT. Clinician-rated anxiety severity was recorded at each appointment. Trajectories of change during (a) the cognitive phase and (b) the exposure phase of treatment were analyzed using piecewise multilevel modeling.

Results: Compared to the psychoeducation and cognitive therapy phases, the exposure phase corresponded with significantly more rapid symptom reduction, b = -.11, 95% CI …

Kctd7 Deficiency Induces Myoclonic Seizures Associated With Purkinje Cell Death And Microvascular Defects, Justine H Liang, Jonathan Alevy, Viktor Akhanov, Ryan Seo, Cory A Massey, Danye Jiang, Joy Zhou, Roy V Sillitoe, Jeffrey L Noebels, Melanie A Samuel

Duncan NRI Faculty and Staff Publications

Mutations in the potassium channel tetramerization domain-containing 7 (KCTD7) gene are associated with a severe neurodegenerative phenotype characterized by childhood onset of progressive and intractable myoclonic seizures accompanied by developmental regression. KCTD7-driven disease is part of a large family of progressive myoclonic epilepsy syndromes displaying a broad spectrum of clinical severity. Animal models of KCTD7-related disease are lacking, and little is known regarding how KCTD7 protein defects lead to epilepsy and cognitive dysfunction. We characterized Kctd7 expression patterns in the mouse brain during development and show that it is selectively enriched in specific regions as the brain matures. We further …

Evidence For An Association Between Coffin-Siris Syndrome And Congenital Diaphragmatic Hernia, Yoel Gofin, Xiaonan Zhao, Amanda Gerard, Fernando Scaglia, Michael F Wangler, Samantha A Schrier Vergano, Daryl A Scott

Duncan NRI Faculty and Staff Publications

Coffin-Siris syndrome (CSS) is an autosomal dominant neurodevelopmental syndrome that can present with a variety of structural birth defects. Pathogenic variants in 12 genes have been shown to cause CSS. Most of these genes encode proteins that are a part of the mammalian switch/sucrose non-fermentable (mSWI/SNF; BAF) complex. An association between genes that cause CSS and congenital diaphragmatic hernia (CDH) has been suggested based on case reports and the analysis of CSS and CDH cohorts. Here, we describe an unpublished individual with CSS and CDH, and we report additional clinical information on four published cases. Data from these individuals, and …

‘Fly-Ing’ From Rare To Common Neurodegenerative Disease Mechanisms, Mengqi Ma, Matthew J Moulton, Shenzhao Lu, Hugo J Bellen

Duncan NRI Faculty and Staff Publications

Genome sequencing advances have enabled researchers and clinicians to probe vast numbers of human variants to distinguish pathogenic from benign variation. Model organisms have been critical in variant assessment and delineating molecular mechanisms of some of the diseases caused by these variants. The fruit fly, Drosophila melanogaster, has played a valuable role in this endeavor, taking advantage of its genetic technologies and established biological knowledge. In this review, we highlight the utility of the fly in studying the function of genes associated with rare neurological diseases that have led to a better understanding of common disease mechanisms. We emphasize …

Removal Of Kcnq2 From Parvalbumin-Expressing Interneurons Improves Anti-Seizure Efficacy Of Retigabine, Junzhan Jing, Corrinne Dunbar, Alina Sonesra, Ana Chavez, Suhyeorn Park, Ryan Yang, Heun Soh, Maxwell Lee, Anastasios V Tzingounis, Edward C Cooper, Xiaolong Jiang, Atul Maheshwari

Duncan NRI Faculty and Staff Publications

Anti-seizure drug (ASD) targets are widely expressed in both excitatory and inhibitory neurons. It remains unknown if the action of an ASD upon inhibitory neurons could counteract its beneficial effects on excitatory neurons (or vice versa), thereby reducing the efficacy of the ASD. Here, we examine whether the efficacy of the ASD retigabine (RTG) is altered after removal of the Kv7 potassium channel subunit KCNQ2, one of its drug targets, from parvalbumin-expressing interneurons (PV-INs). Parvalbumin-Cre (PV-Cre) mice were crossed with Kcnq2-floxed (Kcnq2fl/fl) mice to conditionally delete Kcnq2 from PV-INs. In these conditional knockout mice (cKO, PV-Kcnq2 …

Arrhythmia Burden And Heart Rate Response During Exercise In Anderson-Fabry Disease, Adam W Powell, Samuel G Wittekind, Wayne A Mays, Sean M Lang, Timothy K Knilans, Carlos E Prada, Robert J Hopkin, Clifford Chin

The Texas Heart Institute Journal

Patients with Anderson-Fabry disease (AFD) have an elevated incidence of resting arrhythmias and ischemic heart disease, but their exercise arrhythmia burden and ischemic changes are not well understood. In addition, little research has been done on heart rate recovery in these patients.

We retrospectively reviewed charts of patients with AFD who underwent maximal effort cardiopulmonary exercise testing (CPET) (n=44; 38.2 ± 13.8 yr; 23 men) from 2012 through 2018. Electrocardiographic, Holter monitoring, echocardiographic, cardiac magnetic resonance imaging, and patient demographic data were collected.

No patient had adverse events that necessitated CPET termination, whereas 25 (57%) had ectopy during CPET, including …

Transcatheter Aortic Valve Implantation For Severe Aortic Stenosis And Large Membranous Interventricular Septal Aneurysm, Maheedhar Gedela, Tomasz Stys, Terezia Petraskova, Adam Stys

The Texas Heart Institute Journal

No abstract provided.

Impact Of Active And Historical Cancers On The Management And Outcomes Of Acute Myocardial Infarction Complicating Cardiogenic Shock, Sri Harsha Patlolla, Anusha G Bhat, Pranathi R Sundaragiri, Wisit Cheungpasitporn, Rajkumar P Doshi, Sudeep K Siddappa Malleshappa, Deepak K Pasupula, Wissam A Jaber, William J Nicholson, Saraschandra Vallabhajosyula

The Texas Heart Institute Journal

BACKGROUND: There are limited data on the outcomes of acute myocardial infarction-cardiogenic shock (AMI-CS) in patients with concomitant cancer.

METHODS: A retrospective cohort of adult AMI-CS admissions was identified from the National Inpatient Sample (2000-2017) and stratified by active cancer, historical cancer, and no cancer. Outcomes of interest included in-hospital mortality, use of coronary angiography, use of percutaneous coronary intervention, do-not-resuscitate status, palliative care use, hospitalization costs, and hospital length of stay.

RESULTS: Of the 557,974 AMI-CS admissions during this 18-year period, active and historical cancers were noted in 14,826 (2.6%) and 27,073 (4.8%), respectively. From 2000 to 2017, there …

Improved Right Ventricular Systolic Function After Cardiac Resynchronization Therapy In Patients With Heart Failure, Hakimeh Sadeghian, Ali Kazemisaied, Mehrnaz Rezvanfard, Arash Jalali, Afsaneh Sadeghian, Haleh Ashraf, Farbod Semnani, Amirhossein Ghaseminejad Raeini

The Texas Heart Institute Journal

Background

Since the introduction of cardiac resynchronization therapy (CRT) to improve left ventricular function, the effect of CRT on the right ventricle in patients with heart failure has not been well described.

Methods

We evaluated the effect of CRT on right ventricular systolic function in 20 patients (80% men; mean [SD] age, 58.5 [9.8] y) with cardiomyopathy and right ventricular systolic dysfunction (New York Heart Association class III or IV, left ventricular ejection fraction ≤35%, and QRS interval ≥120 ms). The median follow-up time was 15 months. Right ventricular systolic function, defined as a tricuspid annular plane systolic excursion (TAPSE) …

Combined Disease Of The Quadricuspid Aortic Valve, Robert Blazekovic, Jasmina Catic, Stjepan Barisin, Marko Kusurin, Savica Gjorgjievska, Stanislav Glumbic

The Texas Heart Institute Journal

No abstract provided.

Dean’S Report: University Of Michigan School Of Dentistry Advancing Dental Education And Oral Health Science Is Our Passion And Mission, Jan Hu Bds, Phd

The Journal of the Michigan Dental Association

The Dean of the University of Michigan School of Dentistry, Jan Hu’s annual report to our readers, highlights the school's dedication to dental education and oral health science. The report underscores the school's commitment to maintaining high standards in the face of changing times. Key Highlights:

1. Educational Normalcy: Despite the challenges posed by the pandemic, the school has returned to a sense of normalcy in educating DDS, dental hygiene, and graduate students. Stringent infection control measures are still in place.
2. Vibrant Research: The school's research endeavors remain strong, with over 200 papers, articles, and reviews published in prestigious academic journals …

J Mich Dent Assoc September 2022

The Journal of the Michigan Dental Association

Monthly, The Journal of the Michigan Dental Association brings news, information, and feature articles to our state's oral health community and the MDA's 6,200+ members. No publication reaches more Michigan dentists! In this issue, the reader will find the following original content:

• Annual reports to our readers from each of our state dental schools.
• A 10-Minute EBD column on the use of Articaine local anesthetic.
• News you need, an Editorial and regular department articles, Dentistry and the Law, Staff Matters, Advocacy, Headquarters Report and component news.

This issue also includes one article reprinted with permission.

• Student A by ADA Trustee …

Headquarters Report: Inspire! Support! Lead! With The 2022 Leadership Forum, Karen Burgess Cae

The Journal of the Michigan Dental Association

The Michigan Dental Association highlights the importance of volunteer leaders in the organization's success and the satisfaction of its membership. The in-person Leadership Forum for 2022 offers an exciting lineup, including topics on dental practice trends, legislative action, inclusion, and hot topics in organized dentistry. Breakout sessions cover leadership development, time management, emotional intelligence, and component engagement. Whether experienced or new to volunteer leadership, attending the Leadership Forum provides opportunities for personal and professional growth. Additionally, the article mentions various leadership opportunities available for members and encourages recognition through the MDA Committed Colleague program.

Staff Matters: Should You Create A Combined Pto Bank?, Jodi Schafer Sphr, Shrm-Scp

The Journal of the Michigan Dental Association

Consider consolidating separate leave banks into a combined Paid Time Off (PTO) system to simplify administration and boost employee flexibility. PTO allows staff to use their leave days for any purpose without providing a reason. While it fosters trust and personal accountability, it may lead to employees saving days for vacations and working while sick. A PTO policy should address availability, employee type, coordination, and communication to encourage PTO utilization, prevent burnout, and enhance work-life balance.

Periprosthetic Knee Infection After Benign Tumor Excision Complicated By Carbapenem Resistant Enterobacteriaceae: A Case Report, Jennifer Adams, Daniel Habenicht, Duncan Ramsey

School of Medicine Publications

Introduction

Periprosthetic joint infection (PJI) is a major complication after total knee arthroscopy. Enterobacter is a rare cause of PJI.

Case presentation

We present a 65 year old Caucasian man who presented with acute right knee PJI with Carbapenem-resistant Enterobacteriaceae (CRE) two months after undergoing right knee intra-articular mass removal with endoprosthetic reconstruction. The periprosthetic joint infection (PJI) was treated with revision with 1-stage static spacer and IV meropenem.

Discussion

CRE is an uncommon cause of PJI, but when it does occur, it commonly infects patients who are immunosuppressed or have specific risk factors. For an immunocompetent patient …

Diagnosing A Rare Thoracic Intramedullary Spinal Dermoid Cyst Using Dwi With Adc Mapping: Case Report, Jennifer Adams, Daniel Habenicht, Sohum Desai

School of Medicine Publications

Introduction

Dermoid cysts are rare lesions generally associated with embryological errors that occur during neural tube closure. Intramedullary lesions are extremely rare, especially within the upper thoracic spinal cord.

Case presentation

We report a case of a 19-year-old male who had an intramedullary thoracic dermoid cyst presenting with progressive ataxia, lower limb weakness, and hyperreflexia. MRI demonstrated a 1.2 × 1.8-cm intramedullary thoracic dermoid cyst causing significant spinal cord compression, which was successfully removed via full resection. The patient had an uncomplicated postoperative course, with improvement in preoperative deficits.

Discussion

This is a unique case documenting a thoracic spinal cord …

Perceived Burdensomeness And Thwarted Belongingness Influence The Childhood Polyvictimization And Suicide Ideation Association Among Hispanic Undergraduates, Claire Hsieh, Ruby Charak, Ines Cano-Gonzalez, Leo Gonzalez, Michelle E. Roley, Joseph D. Hovey

Psychological Science Faculty Publications

The present study examined the mediating effect of perceived burdensomeness (PB) and thwarted belongingness (TB) in the association between childhood polyvictimization and suicide ideation (past week) among 528 Hispanic college students. Nearly 10% reported polyvictimization, 19.8% had suicide ideation, and polyvictimization was a risk factor of suicide ideation through PB and TB. The indirect effect through PB was stronger than the indirect effect through TB. Interventions should focus on PB and TB to alleviate suicide ideation among Hispanic undergraduate students.

One Size Does Not Fit All: Discharge Teaching And Child Challenging Behaviors, Norah L. Johnson, Stacee M. Lerret, Michele Polfuss, Karen Gralton, Cori A. Gibson, Sheikh Iqbal Ahamed, Riddhiman Adib, Rosemary White-Traut, Kathleen Sawin, Roger L. Brown

College of Nursing Faculty Research and Publications

This study compares quality of discharge teaching and care coordination for parents of children with challenging behaviors participating in a nursing implementation project, which used an interactive iPad application, to usual discharge care. Unlike parents in the larger quasi-experimental longitudinal project, parents of children with challenging behaviors receiving the discharge teaching application (n = 14) reported lower mean scores on the quality of discharge teaching scale–delivery subscale (M = 8.2, SD = 3.1) than parents receiving usual care (n = 11) (M = 9.6, SD = 4.7) and lower scores on the Care Transition Measure (M = …

Recommendations For Whole Genome Sequencing In Diagnostics For Rare Diseases, Erika Souche, Sergi Beltran, Erwin Brosens, John W Belmont, Magdalena Fossum, Olaf Riess, Christian Gilissen, Amin Ardeshirdavani, Gunnar Houge, Marielle Van Gijn, Jill Clayton-Smith, Matthis Synofzik, Nicole De Leeuw, Zandra C Deans, Yasemin Dincer, Sebastian H Eck, Saskia Van Der Crabben, Meena Balasubramanian, Holm Graessner, Marc Sturm, Helen Firth, Alessandra Ferlini, Rima Nabbout, Elfride De Baere, Thomas Liehr, Milan Macek, Gert Matthijs, Hans Scheffer, Peter Bauer, Helger G Yntema, Marjan M Weiss

Center for Medical Ethics and Health Policy Staff Publications

In 2016, guidelines for diagnostic Next Generation Sequencing (NGS) have been published by EuroGentest in order to assist laboratories in the implementation and accreditation of NGS in a diagnostic setting. These guidelines mainly focused on Whole Exome Sequencing (WES) and targeted (gene panels) sequencing detecting small germline variants (Single Nucleotide Variants (SNVs) and insertions/deletions (indels)). Since then, Whole Genome Sequencing (WGS) has been increasingly introduced in the diagnosis of rare diseases as WGS allows the simultaneous detection of SNVs, Structural Variants (SVs) and other types of variants such as repeat expansions. The use of WGS in diagnostics warrants the re-evaluation …

Will Anti-Vaccine Activism In The Usa Reverse Global Goals?, Peter J Hotez

Center for Medical Ethics and Health Policy Staff Publications

In the time of the COVID-19 pandemic, anti-vaccine activism in the USA accelerated, amplified and formed an alliance with political groups and even extremists. An organized, well-funded and empowered anti-science movement now threatens to spill over and threaten all childhood immunizations in the USA and globally.

An Ultra-Brief Screening Scale For Pediatric Obsessive-Compulsive Disorder: The Oci-Cv-5, Amitai Abramovitch, Jonathan S Abramowitz, Dean Mckay, Heining Cham, Kennedy S Anderson, Lara J Farrell, Daniel A Geller, Gregory L Hanna, Sharna Mathieu, Joseph F Mcguire, David R Rosenberg, S Evelyn Stewart, Eric A Storch, Sabine Wilhelm

Center for Medical Ethics and Health Policy Staff Publications

Background: Obsessive-compulsive disorder (OCD) is an often disabling and chronic condition that is normally assessed using diagnostic interviews or lengthy self-report questionnaires. This makes routine screening in general health settings impractical, and as a result OCD is often under-(or mis-)recognized. The present study reports on the development of an ultra-brief version of the Obsessive-Compulsive Inventory-Child Version (OCI-CV) which may be administered routinely as a screener for pediatric OCD.

Method: A total of 489 youth diagnosed with OCD, 259 non-clinical controls, and 299 youth with other disorders completed the OCI-CV and other indices of psychopathology. Using item analyses, we extracted five …

United We Stand: On The Benefits Of Coordinated Punishment, Vicente Calabuig, Natalia Jiménez, Gonzalo Olcina, Ismael Rodriguez-Lara

ESI Working Papers

Coordinated punishment occurs when punishment decisions are complements; i.e., this punishment device requires a specific number of punishers to be effective; otherwise, no damage will be inflicted on the target. While societies often rely on this punishment device, its benefits are unclear compared with uncoordinated punishment, where punishment decisions are substitutes. We argue that coordinated punishment can prevent the free-riding of punishers and show, both theoretically and experimentally, that this may be beneficial for cooperation in a team investment game, compared with uncoordinated punishment.

Subclavian Steal Phenomenon Associated With Vascular Ring In An Infant Who Had A Prenatally Diagnosed Right Aortic Arch With An Atretic Aberrant Left Subclavian Artery, Daiji Takajo, Jennifer Blake, Sanjeev Aggarwal

The Texas Heart Institute Journal

Vascular ring is a rare congenital anomaly in which the abnormal origin of the aorta or its branches and pulmonary arteries leads to encircling and compression of the trachea and esophagus. A right aortic arch (RAA) with an aberrant left subclavian artery is one of the most common forms of vascular ring. Here, we report a case of a prenatally diagnosed vascular ring resulting from an RAA with an aberrant left subclavian artery. When the infant was 7 months of age, the development of noisy breathing prompted further evaluation with cardiac magnetic resonance imaging that showed an atretic left subclavian …

Hypertrophic Cardiomyopathy And Pregnancy: A Retrospective Analysis From A Tertiary Care Hospital, Pooja Sikka, Vanita Suri, Seema Chopra, Neelam Aggarwal, Subhas Chandra Saha, Ramandeep Bansal, Rajesh Vijayvergiya, Ajay Bahl

The Texas Heart Institute Journal

Pregnancy in women with hypertrophic cardiomyopathy is not well described. In this retrospective study, we analyzed data on pregnant women with hypertrophic cardiomyopathy who were under follow-up care in the cardiology department of a tertiary care hospital. We reviewed data on all women registered in the hypertrophic cardiomyopathy cohort and those who attended the cardio-obstetric clinic and delivered between January 2010 and June 2019. From these 2 groups, we identified 7 pregnant women with hypertrophic cardiomyopathy who delivered during this period. These 7 women (mean [SD] age, 25 [3.3] years) had a total of 15 pregnancies (range per woman,1–4). This …

Emergence Of A Norm From Resistance: Using Simulation To Explore The Macro Implications Of Social Identity Theory, Khadijeh Salimi, Jesse T. Richman, Regina Karp, George P. Richardson, David Anderson

Political Science & Geography Faculty Publications

We usually hope that social norms discourage injustice. However, we are all witnesses to harmful norms enforced by governments, such as xenophobia, which need to be contested and changed. Previous studies have concluded that it is possible to change a harmful norm through contestation by powerless actors if suitable structural conditions exist. However, these structural conditions have not been sufficiently studied and, as such, are the focus of this paper. Our paper begins with a review of well-established micro-level theories of social identity theory (SIT), recast as a set of 42 discrete theoretical statements. These statements are then re-expressed in …

Meta-Analysis: Outcomes Of Surgical And Medical Management Of Diabetic Foot Osteomyelitis, David H. Truong, Roger Bedimo, Matthew Malone, Dane K. Wukich, Orhan Oz, Amanda L. Killeen, Lawrence A. Lavery

School of Podiatric Medicine Publications

Background

The aim of this study was to evaluate clinical outcomes in the published literature on medical and surgical management of diabetic foot osteomyelitis (DFO).

Methods

A PubMed and Google Scholar search of articles relating to DFO was performed over the dates of January 1931 to January 2020. Articles that involved Charcot arthropathy, case reports, small case series, review articles, commentaries, nonhuman studies, and non-English articles were excluded. The Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) tool was used to rate the bias of each study. A meta-analysis was performed using random-effects and inverse variance methods. The search yielded 1192 …

B-Cell Lymphoblastic Lymphoma Of The Paranasal Sinus: A Rare Clinical Entity, Brian Anderson, Christopher M. Metz

Conference Presentation Abstracts

No abstract provided.

Perceptions Of The Virtual Interview Process: A National Survey Of Rhinology Fellowship Directors, Jared A. Johnson, Benjamin S. Johnson, Michael T. Chung, Adam Folbe

Conference Presentation Abstracts

No abstract provided.