Medical Specialties Commons^™

Stress And Negative Affect As Mediators In The Association Between Parental Social Support And Lung Function In Adolescents With Asthma, Amber Osorno, Eric Sternlicht, Pornchai Tirakitsoontorn, Azucena Talamantes, Anchalee Yuengsrigul, Zeev N. Kain, Brooke Jenkins

SURF Posters and Papers

Asthma is the leading chronic condition amongst children in the United States as 7.5% of children are diagnosed with asthma. Studies have shown that positive social support is associated with positive asthma management. A strong social support system predicts good management of asthma symptoms; however, current literature has not yet examined how social support impacts lung function as opposed to merely symptom management. Stress and negative affect have been revealed to be associated with worse asthma control, as well as exacerbation of symptoms. Stressful situations, such as the death of a family member, unemployment, and familial tensions lead to worsening …

Does The Use Of Extracorporeal Cardiopulmonary Resuscitation Improve Neurologically Favorable Survival In Patients With Refractory Out-Of-Hospital Cardiac Arrest?, Timothy Lund

Theses and Graduate Projects

In this retrospective review, the use of extracorporeal cardiopulmonary resuscitation (ECPR) using extracorporeal membrane oxygenation (ECMO) was assessed in the management of refractory ventricular fibrillation (VF) and ventricular tachycardia (VT) out-of-hospital cardiac arrest (OHCA). Multiple factors were evaluated such as the duration of manual or mechanical cardiopulmonary resuscitation (CPR), ECPR protocols and the rate of favorable neurologic outcomes for these OHCA patients. Survival with a favorable neurologic outcome is defined as cerebral performance categories (CPC) 1 or 2. Although CPR remains and will continue to be a critical component in the management of cardiac arrest, the persistently high mortality rate …

A Study Of The Relationship Between Uric Acid And Substantia Nigra Brain Connectivity In Patients With Rem Sleep Behavior Disorder And Parkinson’S Disease, Timothy M. Ellmore, Jessika Suescun, Richard J. Castriotta, Mya C. Schiess

Publications and Research

Low levels of the natural antioxidant uric acid (UA) and the presence of REM sleep behavior disorder (RBD) are both associated with an increased likelihood of developing Parkinson’s disease (PD). RBD and PD are also accompanied by basal ganglia dysfunction including decreased nigrostriatal and nigrocortical resting state functional connectivity. Despite these independent findings, the relationship between UA and substantia nigra (SN) functional connectivity remains unknown. In the present study, voxelwise analysis of covariance was used in a cross-sectional design to explore the relationship between UA and whole-brain SN functional connectivity using the eyes-open resting state fMRI method in controls without …

Collaborative Strategies To Reduce Incomplete Operating Room Instruments Trays, Richard Neusch, Nicole Wagner, Colby Fike, Suneela Nayak

Operations Transformation

In an academic tertiary medical care center, the OR was receiving incomplete instrument trays. This issue became increasingly problematic in wasted staff time and cost to the institution.

Using a tracking system to generate a missing instrument report, baseline metrics were established. Reviewing each report, an interdepartmental team comprised of members of the OR and sterile processing departments, initiated a number of countermeasures.

Since the start of these countermeasures, there has been a reduction in the receipt of incomplete OR trays. Next steps include creating a tag process by which the OR instrument trays from one case would be kept …

Hepatic Stellate Cells And Hepatocarcinogenesis, Anna E Barry, Rajkumar Baldeosingh, Ryan Lamm, Keyur Patel, Kai Zhang, Dana A Dominguez, Kayla J Kirton, Ashesh P. Shah, Hien Dang

Department of Surgery Faculty Papers

Hepatic stellate cells (HSCs) are a significant component of the hepatocellular carcinoma (HCC) tumor microenvironment (TME). Activated HSCs transform into myofibroblast-like cells to promote fibrosis in response to liver injury or chronic inflammation, leading to cirrhosis and HCC. The hepatic TME is comprised of cellular components, including activated HSCs, tumor-associated macrophages, endothelial cells, immune cells, and non-cellular components, such as growth factors, proteolytic enzymes and their inhibitors, and other extracellular matrix (ECM) proteins. Interactions between HCC cells and their microenvironment have become topics under active investigation. These interactions within the hepatic TME have the potential to drive carcinogenesis and create …

Considerations For Studying Sex As A Biological Variable In Spinal Cord Injury, Andrew N. Stewart, Steven M. Maclean, Arnold J. Stromberg, Jessica P. Whelan, William M. Bailey, John C. Gensel, Melinda E. Wilson

Physiology Faculty Publications

In response to NIH initiatives to investigate sex as a biological variable in preclinical animal studies, researchers have increased their focus on male and female differences in neurotrauma. Inclusion of both sexes when modeling neurotrauma is leading to the identification of novel areas for therapeutic and scientific exploitation. Here, we review the organizational and activational effects of sex hormones on recovery from injury and how these changes impact the long-term health of spinal cord injury (SCI) patients. When determining how sex affects SCI it remains imperative to expand outcomes beyond locomotor recovery and consider other complications plaguing the quality of …

Menopause In Brazilian Women With Sickle Cell Anemia With And Without Hydroxyurea Therapy., Ana Mach Queiroz, Clarisse Lopes De Castro Lobo, Samir K. Ballas

Cardeza Foundation for Hematologic Research

Women with sickle cell anemia (SCA) require special attention for myriad obstetric and gynecologic issues associated with this complex hematologic disorder. These affect all aspects of female life from menarche through menopause. Information regarding obstetric and gynecologic complications of sickle cell disease (SCD), with a few exceptions, is based primarily on observational, anecdotal, retrospective, or cohort studies that may not reflect current aspects of obstetric care.¹ Similarly, there are no recent reports about menopause (also known as climacteric) in women with SCA. The fact that life expectancy of women with SCA was determined to be 46–48 years² delayed …

Cutaneous Carcinosarcoma: A Small Case Series And Review Of The Literature Of A Rare Skin Tumor., Ethan Y Song, Sean J. Wallace Md, Hina A. Sheikh, Randolph Wojcik Md, Chetan Nayak

Department of Surgery

Carcinosarcomas are composed of epithelial and mesenchymal elements and primarily present within visceral organs. Despite being potentially aggressive, they are a rare diagnosis in the skin, and few manifestations have been reported to date. In this report, we describe two separate cases of carcinosarcoma presenting as nonhealing scalp wounds. Patient A: a 57-year-old male with a nonhealing skin lesion of ten years successfully treated with wide-local excision and local ortichochea flap reconstruction. Patient B: a 75-year-old female that presented with a painless, slow-growing hemorrhagic mass of 7 years invading the skull and dura ultimately requiring craniectomy and free-tissue transfer with …

The Spectrum Of Primary Immunodeficiencies At A Tertiary Care Hospital In Pakistan, Sonia Qureshi, Fatima Mir, Samina Junejo, Khalid Saleem, Samreen Zaidi, Abdullah B. Naveed, Khalil Ahmad, Farah Naz Qamar

Department of Paediatrics and Child Health

Background: Primary Immunodeficiency Disorders (PIDs) are well-known disorders in the West. but the recognition and diagnosis of these disorders is challenging in developing countries. We present the spectrum of PIDs seen at a tertiary care center in Pakistan, identified using clinical case definitions and molecular methods.
Methods: A retrospective chart review of children suspected to have PID was conducted at the Aga Khan University Hospital (AKUH) Karachi, Pakistan from 2010 to 2016. Data on demographics, clinical features, family history of consanguinity, sibling death, details of laboratory workup done for PID and molecular tests targeted panel next generation sequencing (NGS) or …

Dha Modulates Manf And Trem2 Abundance, Enhances Neurogenesis, Reduces Infarct Size, And Improves Neurological Function After Experimental Ischemic Stroke, Ludmila Belayev, Sung Ha Hong, Raul S. Freitas, Hemant Menghani, Shawn J. Marcell, Larissa Khoutorova, Pranab K. Mukherjee, Madigan M. Reid, Reinaldo B. Oria, Nicolas G. Bazan

School of Medicine Faculty Publications

Aims: Mesencephalic astrocyte-derived neurotrophic factor (MANF) is a secretory neurotrophic factor protein that promotes repair after neuronal injury. The microglia cell surface receptor (triggering receptor expressed on myeloid cells-2; TREM2) regulates the production of pro- and antiinflammatory mediators after stroke. Here, we study MANF and TREM2 expression after middle cerebral artery occlusion (MCAo) and explore if docosahexaenoic acid (DHA) treatment exerts a potentiating effect. Methods: We used 2 hours of the MCAo model in rats and intravenously administered DHA or vehicle at 3 hours after the onset of MCAo. Neurobehavioral assessment was performed on days 1, 3, 7, and 14; …

Direct Delivery Of Antisense Oligonucleotides To The Middle And Inner Ear Improves Hearing And Balance In Usher Mice, Jennifer J. Lentz, Bifeng Pan, Abhilash Ponnath, Christopher M. Tran, Carl Nist-Lund, Alice Galvin, Hannah Goldberg, Katelyn N. Robillard, Francine M. Jodelka, Hamilton E. Farris, Jun Huang, Tianwen Chen, Hong Zhu, Wu Zhou, Frank Rigo, Michelle L. Hastings, Gwenaëlle S.G. Géléoc

School of Medicine Faculty Publications

Usher syndrome is a syndromic form of hereditary hearing impairment that includes sensorineural hearing loss and delayed-onset retinitis pigmentosa (RP). Type 1 Usher syndrome (USH1) is characterized by congenital profound sensorineural hearing impairment and vestibular areflexia, with adolescent-onset RP. Systemic treatment with antisense oligonucleotides (ASOs) targeting the human USH1C c.216G>A splicing mutation in a knockin mouse model of USH1 restores hearing and balance. Herein, we explore the effect of delivering ASOs locally to the ear to treat hearing and vestibular dysfunction associated with Usher syndrome. Three localized delivery strategies were investigated in USH1C mice: inner ear injection, trans-tympanic membrane …

The Rapid Increase In Telemedicine Visits During Covid-19, Chelsea Johnson, Kathryn Taff, Brian R. Lee, Amanda Montalbano

Patient Experience Journal

Prior to the COVID-19 pandemic, facilitated telemedicine encounters were available at outreach locations; however, our tertiary children’s hospital had not invested widely in direct to patient telemedicine. Our daily pediatric subspecialty visits dropped from an average of 2066 visits a day prior to COVID-19 in our community to 1000 patients a day during the study period. Over the four-week period from April 15 to May 12, 2020, patient and family experience ratings of percentage of positive responses (9 or 10) on the provider rating 0-10 scale between telemedicine and in-person visits were compared for our pediatric subspecialty clinics using a …

Patient Experience In A Pediatric Emergency Department During Covid-19, Beth L. Emerson Md, Erika Setzer

Patient Experience Journal

The COVID-19 pandemic has changed many dynamics in healthcare in the United States. This study explores an increase in patient experience (PE) scores in a pediatric emergency department. Visits were analyzed before and after March 8, 2020, corresponding with the first local case of COVID-19. Changes in the patient population and characteristics of survey responders were analyzed. Overall, the number of daily visits decreased (113 vs 36/day) and survey response rate decreased (3.7 vs 2.8%, p = 0.03), but PE scores increased (87.21 to 93.73, p = 0.002). Comparatively, an increase in patients with higher acuity levels by Emergency Severity …

Is This Really Happening? Family-Centered Care During Covid-19: People Before Policy, Terri Savino, Karri Crispino

Patient Experience Journal

In the middle of a global pandemic, hospitals created policies for visitor restrictions to reduce the transmission of coronavirus to protect patients and staff and developed protocols allowing only one support person to call the critical care unit for patient updates. Late on a Tuesday afternoon, the Manager of Patient Experience received a phone call asking her to call Karri, the wife of one of our patients who was on a ventilator. Karri was struggling with updating her mother-in-law because she was very upset with the news she received, making it difficult to call her husband’s mom. Karri asked the …

Epidemiological Variation Of Acute Myocardial Infarction Relevant To In-Hospital Outcomes-Tertiary Center Experience-Saudi Arabia, Sheeren Khaled, Mohammad Almalki, Ghada Shalaby, Azmat K. Niazi, Sara Ahmed, Asma Alsilami, Mohannd Alhazmi, Zeyad Bukhary, Najeeb Jaha

Journal of the Saudi Heart Association

Background: Epidemiological related differences in patients presenting with ST- elevation myocardial infarction (STEMI) have not yet been fully characterized in the Middle East countries. The aim of this study was to assess gender, ethnic and racial variation in clinical profiles, presentation and treatment strategies with relation to the in-hospital outcomes.

Method: This is a retrospective, single center study reviewing the epidemiological details of STEMI patients who were admitted to our center during the period between October 2015 and August 2019.

Result: Out of 3079 patients presented with STEMI, 498(16%) were women, 2170 (70%) were from Middle Eastern Countries and only …

Medium-Term Complications Associated With Coronary Artery Aneurysms After Kawasaki Disease: A Study From The International Kawasaki Disease Registry., Brian W Mccrindle, Cedric Manlhiot, Jane W Newburger, Ashraf S Harahsheh, Therese M Giglia, Frederic Dallaire, Kevin Friedman, Tisiana Low, Kyle Runeckles, Mathew Mathew, Andrew S Mackie, Nadine F Choueiter, Pei-Ni Jone, Shelby Kutty, Anji T Yetman, Geetha Raghuveer, Elfriede Pahl, Kambiz Norozi, Kimberly E Mchugh, Jennifer S Li, Sarah D De Ferranti, Nagib Dahdah

Paediatrics Publications

Background Coronary artery aneurysms (CAAs) may occur after Kawasaki disease (KD) and lead to important morbidity and mortality. As CAA in patients with KD are rare and heterogeneous lesions, prognostication and risk stratification are difficult. We sought to derive the cumulative risk and associated factors for cardiovascular complications in patients with CAAs after KD. Methods and Results A 34-institution international registry of 1651 patients with KD who had CAAs (maximum CAA

Hearing Loss In Mucopolysaccharidoses: Current Knowledge And Future Directions., Jeremy Wolfberg, Keerthana Chintalapati, Shunji Tomatsu, Kyoko Nagao

Department of Pediatrics Faculty Papers

Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders caused by a deficiency of one of the enzymes involved in the degradation of glycosaminoglycans. Hearing loss is a common clinical presentation in MPS. This paper reviews the literature on hearing loss for each of the seven recognized subtypes of MPS. Hearing loss was found to be common in MPS I, II, III, IVA, VI, and VII, and absent from MPS IVB and MPS IX. MPS VI presents primarily with conductive hearing loss, while the other subtypes (MPS I, MPS II, MPS III, MPS IVA, and MPS VII) can present with …

Ewing Sarcoma Family Of Tumors-Derived Small Extracellular Vesicle Proteomics Identify Potential Clinical Biomarkers., Glenson Samuel, Jennifer Crow, Jon B. Klein, Michael L. Merchant, Emily Nissen, Devin C. Koestler, Kris Laurence, Xiaobo Liang, Kathleen Neville, Vincent S. Staggs, Atif Ahmed, Safinur Atay, Andrew K. Godwin

Manuscripts, Articles, Book Chapters and Other Papers

Purpose: Ewing Sarcoma Family of Tumors (ESFT), the second most common pediatric osseous malignancy, are characterized by the pathognomonic chromosomal EWS-ETS translocation. Outside of tumor biopsy, no clinically relevant ESFT biomarkers exist. Additionally, tumor burden assessment at diagnosis, monitoring of disease responsiveness to therapy, and detection of disease recurrence are limited to radiographic imaging. To identify new, clinically relevant biomarkers we evaluated the proteome of a subset of ESFT-derived small extracellular vesicles (sEVs).

Materials and methods: We performed the first high quality proteomic study of ESFT-derived sEVs from 5 ESFT cell lines representing the most common EWS-ETS fusion types and …

Fosl1 Is A Novel Mediator Of Endotoxin/Lipopolysaccharide-Induced Pulmonary Angiogenic Signaling., Christopher R. Nitkin, Sheng Xia, Heather Menden, Wei Yu, Min Xiong, Daniel P. Heruth, Shui Qing Ye, Venkatesh Sampath

Manuscripts, Articles, Book Chapters and Other Papers

Systemic sepsis is a known risk factor for bronchopulmonary dysplasia (BPD) in premature infants, a disease characterized by dysregulated angiogenesis and impaired vascular and alveolar development. We have previoulsy reported that systemic endotoxin dysregulates pulmonary angiogenesis resulting in alveolar simplification mimicking BPD in neonatal mice, but the underlying mechanisms remain unclear. We undertook an unbiased discovery approach to identify novel signaling pathways programming sepsis-induced deviant lung angiogenesis. Pulmonary endothelial cells (EC) were isolated for RNA-Seq from newborn C57BL/6 mice treated with intraperitoneal lipopolysaccharide (LPS) to mimic systemic sepsis. LPS significantly differentially-regulated 269 genes after 6 h, and 1,934 genes after …

Medium-Term Complications Associated With Coronary Artery Aneurysms After Kawasaki Disease: A Study From The International Kawasaki Disease Registry., Brian W. Mccrindle, Cedric Manlhiot, Jane W Newburger, Ashraf S. Harahsheh, Therese M. Giglia, Frederic Dallaire, Kevin Friedman, Tisiana Low, Kyle Runeckles, Mathew Mathew, Andrew S. Mackie, Nadine F. Choueiter, Pei-Ni Jone, Shelby Kutty, Anji T. Yetman, Geetha Raghuveer, Elfriede Pahl, Kambiz Norozi, Kimberly E Mchugh, Jennifer S. Li, Sarah D De Ferranti, Nagib Dahdah, International Kawasaki Disease Registry

Manuscripts, Articles, Book Chapters and Other Papers

Background Coronary artery aneurysms (CAAs) may occur after Kawasaki disease (KD) and lead to important morbidity and mortality. As CAA in patients with KD are rare and heterogeneous lesions, prognostication and risk stratification are difficult. We sought to derive the cumulative risk and associated factors for cardiovascular complications in patients with CAAs after KD. Methods and Results A 34-institution international registry of 1651 patients with KD who had CAAs (maximum CAA Z score ≥2.5) was used. Time-to-event analyses were performed using the Kaplan-Meier method and Cox proportional hazard models for risk factor analysis. In patients with CAA Z scores ≥10, …

Low Anterior Resection Syndrome, Theresa H Nguyen, Reena V Chokshi

Faculty, Staff and Students Publications

PURPOSE OF REVIEW: Low anterior resection syndrome is a highly prevalent condition that can develop after anal sphincter-sparing surgery for rectal cancer and impair quality of life. In this review, we summarize the major features and pathophysiology of this syndrome and discuss treatment approaches.

RECENT FINDINGS: Quality of life correlates significantly with severity of low anterior resection syndrome. Prompt assessment and initiation of therapy are essential to rehabilitating damaged mechanical and neural structures. Anorectal manometry demonstrates a global decrease in sphincteric function postoperatively, though in many patients, function does recover. Transanal irrigation, pelvic floor rehabilitation, and biofeedback are the mainstays …

Metabolism Of Jq1, An Inhibitor Of Bromodomain And Extra Terminal Bromodomain Proteins, In Human And Mouse Liver Microsomes†, Feng Li, Kevin R Mackenzie, Prashi Jain, Conrad Santini, Damian W Young, Martin M Matzuk

Faculty, Staff and Students Publications

JQ1 is a small-molecule inhibitor of the bromodomain and extra terminal (BET) protein family that potently inhibits the bromodomain testis-specific protein (BRDT), which is essential for spermatogenesis. JQ1 treatment produces a reversible contraceptive effect by targeting the activity of BRDT in mouse male germ cells, validating BRDT as a male contraceptive target. Although JQ1 possesses favourable physical properties, it exhibits a short half-life. Because the details of xenobiotic metabolism play important roles in the optimization of drug candidates and in determining the role of metabolism in drug efficacy, we investigated the metabolism of JQ1 in human and mouse liver microsomes. …

Prevalence Of Cardiovascular Diseases Risk Factors Among Jordanians, Wesam Saleh Alsaud, Mohammad Tabbaa, Violet Kasabri, Maysa Suyagh, Muneer Abu Alsamen, Hussain Haddad, Anas Sheweiki

Journal of the Saudi Heart Association

Background and aims: One of the most common causes of death worldwide is cardiovascular diseases (CVDs). This study evaluated the prevalence of CVDs risk factors (RFs) and their constellation electively among the Jordanian population and assessing the most prevalent RF interplay with the rest of CVDs RFs as well as the impact of age and gender dimorphism on the frequencies of coexistence of multiple CVDs risk factors (RFs) among the Jordanian population. Methods and results: In this observational multicenter study, a total of 1449 subjects were enrolled. The mean age (± SD) was 44.35 ± 14.46 years; 796 (54.9%) of …

The Landscape Of Rna Polymerase Ii-Associated Chromatin Interactions In Prostate Cancer, Susmita G Ramanand, Yong Chen, Jiapei Yuan, Kelly Daescu, Maryou Bk Lambros, Kathleen E Houlahan, Suzanne Carreira, Wei Yuan, Guemhee Baek, Adam Sharp, Alec Paschalis, Mohammed Kanchwala, Yunpeng Gao, Adam Aslam, Nida Safdar, Xiaowei Zhan, Ganesh V Raj, Chao Xing, Paul C Boutros, Johann De Bono, Michael Q Zhang, Ram S Mani

College of Science & Mathematics Departmental Research

Transcriptional dysregulation is a hallmark of prostate cancer (PCa). We mapped the RNA polymerase II-associated (RNA Pol II-associated) chromatin interactions in normal prostate cells and PCa cells. We discovered thousands of enhancer-promoter, enhancer-enhancer, as well as promoter-promoter chromatin interactions. These transcriptional hubs operate within the framework set by structural proteins - CTCF and cohesins - and are regulated by the cooperative action of master transcription factors, such as the androgen receptor (AR) and FOXA1. By combining analyses from metastatic castration-resistant PCa (mCRPC) specimens, we show that AR locus amplification contributes to the transcriptional upregulation of the AR gene by increasing …

Go On With The Good Work – The Still-Hildreth Osteopathic Sanatorium, Robyn Oro

Notes from the Archives

No abstract provided.

Evaluation Of Aspirin And Statin Therapy Use And Adherence In Patients With Premature Atherosclerotic Cardiovascular Disease, Dhruv Mahtta, David J. Ramsey, Mahmoud Al Rifai, Khurram Nasir, Zainab Samad, David Aguilar, Hani Jneid, Christie M. Ballantyne, Laura A. Petersen, Salim S. Virani

Section of Cardiology

Importance: Studies on the use of and adherence to secondary prevention therapies in patients with premature and extremely premature atherosclerotic cardiovascular disease (ASCVD) are lacking.
Objective: To evaluate and compare aspirin use, any statin use, high-intensity statin use, and statin adherence among patients with premature or extremely premature ASCVD compared with patients with nonpremature ASCVD.
Design, setting, and participants: This multicenter cross-sectional study used the clinical and administrative data sets of the US Department of Veterans Affairs (VA) to identify adult patients with at least 1 primary care visit in the VA health care system between October 1, 2014, and …

Expanding Pharmacotherapy Data Collection, Analysis, And Implementation In Eras® Programs—The Methodology Of An Exploratory Feasibility Study, Eric Johnson, Richard Parrish Ii, Gregg Nelson, Kevin Elias, Brian Kramer, Marian Gaviola

Pharmacy Practice and Science Faculty Publications

Surgical organizations dedicated to the improvement of patient outcomes have led to a worldwide paradigm shift in perioperative patient care. Since 2012, the Enhanced Recovery After Surgery (ERAS^®) Society has published guidelines pertaining to perioperative care in numerous disciplines including elective colorectal and gynecologic/oncology surgery patients. The ERAS^® and ERAS-USA^® Societies use standardized methodology for collecting and assessing various surgical parameters in real-time during the operative process. These multi-disciplinary groups have constructed a bundled framework of perioperative care that entails 22 specific components of clinical interventions, which are logged in a central database, allowing a system …

A Surgeon's Handedness In Direct Anterior Approach-Hip Replacement., Xiangpeng Kong, Minzhi Yang, Alvin Ong, Renwen Guo, Jiying Chen, Yan Wang, Wei Chai

Department of Orthopaedic Surgery Faculty Papers

BACKGROUND: The impact of handedness on clinical outcomes was easily overlooked in hip replacement. This study aimed to find whether the component positioning and hip function were affected by the handedness in total hip arthroplasty (THA) through direct anterior approach (DAA).

METHODS: Total 102 patients who underwent bilateral DAA-THAs simultaneously between May 2016 and November 2018 in our institute were reviewed. All surgeries were operated by one right-handed surgeon. Their demographic, cup positioning, stem alignment, femoral stem fit, Harris hip score (HHS), intraoperative and postoperative complications were used to evaluate the role of handedness in DAA.

RESULTS: The inclination of …

A Global Slc7a7 Knockout Mouse Model Demonstrates Characteristic Phenotypes Of Human Lysinuric Protein Intolerance, Bridget M Stroup, Ronit Marom, Xiaohui Li, Chih-Wei Hsu, Cheng-Yen Chang, Luan D Truong, Brian Dawson, Ingo Grafe, Yuqing Chen, Ming-Ming Jiang, Denise Lanza, Jennie Rose Green, Qin Sun, J P Barrish, Safa Ani, Audrey E Christiansen, John R Seavitt, Mary E Dickinson, Farrah Kheradmand, Jason D Heaney, Brendan Lee, Lindsay C Burrage

Faculty, Staff and Students Publications

Lysinuric protein intolerance (LPI) is an inborn error of cationic amino acid (arginine, lysine, ornithine) transport caused by biallelic pathogenic variants in SLC7A7, which encodes the light subunit of the y+LAT1 transporter. Treatments for the complications of LPI, including growth failure, renal disease, pulmonary alveolar proteinosis, autoimmune disorders and osteoporosis, are limited. Given the early lethality of the only published global Slc7a7 knockout mouse model, a viable animal model to investigate global SLC7A7 deficiency is needed. Hence, we generated two mouse models with global Slc7a7 deficiency (Slc7a7em1Lbu/em1Lbu; Slc7a7Lbu/Lbu and Slc7a7em1(IMPC)Bay/em1(IMPC)Bay; Slc7a7Bay/Bay) using CRISPR/Cas9 technology by introducing a deletion of exons …

A Pilot Study Of The Preliminary Efficacy Of Pain Buddy: A Novel Intervention For The Management Of Children’S Cancer-Related Pain, John F. Hunter, Amanda M. Acevedo, Sergio Gago-Masague, Alexandra Kain, Christine Yun, Lilibeth Torno, Brooke N. Jenkins, Michelle A. Fortier

Psychology Faculty Articles and Research

Objectives

Cancer‐related pain in children is prevalent and undermanaged. Mobile health (mHealth) applications provide a promising avenue to address the gap in pain management in children with cancer. Pain Buddy is a multicomponent mHealth application developed to manage cancer‐related pain in children. The goal of this paper is to present preliminary efficacy data of the impact of Pain Buddy on children's pain severity and frequency.

Methods

In a randomized controlled trial over 60 days, children (N = 48) reported daily pain on a tablet while receiving usual care. Those in the intervention group (N = 20) received remote …