Medical Specialties Commons^™

Age Of Acquisition Impacts The Brain Differently Depending On Neuroanatomical Metric, Hannah Claussenius-Kalman, Kelly A Vaughn, Pilar Archila-Suerte, Arturo E Hernandez

Faculty, Staff and Student Publications

Although researchers generally agree that a certain set of brain areas underlie bilingual language processing, there is discrepancy regarding what effect timing of language acquisition has on these regions. We aimed to investigate the neuroanatomical correlates of age of acquisition (AoA), which has been examined previously, but with inconsistent results, likely influenced by methodological differences across studies. We analyzed gray matter density, volume, and thickness using whole-brain linear models in 334 bilinguals and monolinguals. Neuroanatomical correlates of AoA differed depending on gray matter metric. Relative to early bilinguals, late bilinguals had thicker cortex in language processing and cognitive control regions, …

Benefit Of Contact Force-Guided Catheter Ablation For Treating Premature Ventricular Contractions, Ziming Zhao, Xiaowei Liu, Lianjun Gao, Yutao Xi, Qi Chen, Dong Chang, Xianjie Xiao, Jie Cheng, Yanzong Yang, Yunlong Xia, Xiaomeng Yin

The Texas Heart Institute Journal

We evaluated whether an irrigated contact force–sensing catheter would improve the safety and effectiveness of radiofrequency ablation of premature ventricular contractions originating from the right ventricular outflow tract.

We retrospectively reviewed the charts of patients with symptomatic premature ventricular contractions who underwent ablation with a contact force–sensing catheter (56 patients, SmartTouch) or conventional catheter (59 patients, ThermoCool) at our hospital from August 2013 through December 2015. During a mean follow-up of 16 ± 5 months, 3 patients in the conventional group had recurrences, compared with none in the contact force group. Complications occurred only in the conventional group (one steam …

Toward Actionable Practice Parameters For "Dual Diagnosis": Principles Of Assessment And Management For Co-Occurring Psychiatric And Intellectual/Developmental Disability., John N. Constantino, Shae Strom, Michael Bunis, Cy Nadler, Teresa Rodgers, Julia Lepage, Connie Cahalan, Amber Stockreef, Lucas Evans, Rachel Jones, Alyssa Wilson

Manuscripts, Articles, Book Chapters and Other Papers

PURPOSE OF REVIEW: Although treatment algorithms and parameters for best practice are readily available for all major syndromes of psychiatric impairment, the occurrence of psychiatric syndromes in individuals with intellectual and developmental disability (IDD) invokes serious contextual challenges for interpretation of symptoms, diagnosis, and optimization of treatment, both for clinicians and for the service sectors in which care and support of individuals with IDD are delivered. Recognizing that there exist very few definitive resources for best practice under the circumstance of this form of "dual diagnosis," the Missouri Department of Mental Health convened an expert panel to conduct a focused …

Contemporary Nstemi Management: The Role Of The Hospitalist., Charles V Pollack, Alpesh Amin, Tracy Wang, Steven Deitelzweig, Marc Cohen, David Slattery, John Fanikos, Christopher Dilascia, Regan Tuder, Scott Kaatz

Department of Emergency Medicine Faculty Papers

Non-ST-segment elevation myocardial infarction (NSTEMI) is defined as elevated cardiac biomarkers of necrosis in the absence of persistent ST-segment elevation in the setting of anginal symptoms or other acute event. It carries a poorer prognosis than most ST-segment elevation events, owing to the typical comorbidity burden of the older NSTEMI patients as well as diverse etiologies that add complexity to therapeutic decision-making. It may result from an acute atherothrombotic event ('Type 1') or as the result of other causes of mismatch of myocardial oxygen supply and demand ('Type 2'). Regardless of type and other clinical factors, the hospital medicine specialist …

Prognostic Value Of 6-Minute Walk Distance In Patients Undergoing Percutaneous Coronary Intervention: A Veterans Affairs Prospective Study, Tarun W Dasari, Bhavin Patel, Siddharth A Wayangankar, David Alexander, Yan D Zhao, Joann Schlegel, Cheuk Leung, Pedro Lozano, Faisal Latif, Udho Thadani

The Texas Heart Institute Journal

The 6-minute walk distance (6MWD) test is a useful prognostic tool in chronic heart failure. Its usefulness after percutaneous coronary intervention is unknown.

In a prospective observational study, patients underwent a 6MWD test within 2 weeks after percutaneous coronary intervention. The primary endpoint was major adverse cardiovascular events (MACE) (death, acute coronary syndrome, and heart failure admission) at one year. Receiver operating characteristic curves and area under the curve were used to determine the 6MWD test's predictive power, and the Youden index was used to measure its effectiveness.

A total of 212 patients were enrolled (98% men; mean age, 65 …

Unusual Cause Of Heart Failure In A Patient With Marfan Syndrome: A Late Complication Of Bioprosthetic Valved Graft Replacement, Prerna B Bansal, Hari P Chaliki, Kantha R Kolla, Roger L Click, Alberto Pochettino

The Texas Heart Institute Journal

A high-velocity gradient across the left ventricular outflow tract is most often caused by aortic valve stenosis. We describe the unusual case of a high-velocity gradient caused by a kinked ascending aortic graft in a 69-year-old man who had Marfan syndrome. The patient had a history of ascending aortic aneurysm and had previously undergone replacement of the aortic root and ascending aorta with use of a bioprosthetic valved graft. The kinking was caused by dilation of the native aortic arch. The patient underwent successful hemi-arch replacement and repair of the kinked graft. Late complications and reoperation after proximal aortic surgery …

Modified Pull-Through Technique For Cardiac Resynchronization Therapy Upgrades In Patients With Occluded Access Veins, Guram Imnadze, Khaled Awad, Wolfgang Kranig, Irakli Giorgberidze

The Texas Heart Institute Journal

The number of procedures for upgrading implantable devices for cardiac resynchronization therapy has increased considerably during the last decade. A major challenge that operators face in these circumstances is occlusion of the access vein. We have modified a pull-through method to overcome this obstacle. Six consecutive patients with occluded access veins and well-developed collateral networks underwent a procedure in which the occluded vein was recanalized by snaring the existing atrial lead via transfemoral access. Upgrading the device was successful in all patients; none had intraprocedural complications. Our experience shows that our modified pull-through technique may be a feasible alternative for …

Obstruction Of 2 Coronary Arteries From Different Causes Immediately After Transapical Transcatheter Aortic Valve Replacement, Umihiko Kaneko, Ryuji Koshima, Hirosato Doi, Tsutomu Fujita

The Texas Heart Institute Journal

Coronary obstruction, a rare complication of transcatheter aortic valve replacement, can be fatal. Few data exist on this phenomenon, and, to date, authors have reported only single coronary lesions. We present a case in which 2 coronary arteries obstructed immediately after transapical transcatheter aortic valve replacement. The patient was an 81-year-old woman with symptomatic severe aortic stenosis who underwent transapical transcatheter aortic valve replacement. Immediately after an Edwards Sapien XT valve was deployed, she experienced sudden cardiogenic shock resulting from obstruction of the left main coronary artery ostium and the distal left anterior descending coronary artery. The left main obstruction …

Assessing Lymphatic Route Of Csf Outflow And Peripheral Lymphatic Contractile Activity During Head-Down Tilt Using Near-Infrared Fluorescence Imaging, John C Rasmussen, Sunkuk Kwon, Amanda Pinal, Alexander Bareis, Fred C Velasquez, Christopher F Janssen, John R Morrow, Caroline E Fife, Ron J Karni, Eva M Sevick-Muraca

Faculty, Staff and Student Publications

Evidence overwhelmingly suggests that the lymphatics play a critical role in the clearance of cerebrospinal fluid (CSF) from the cranial space. Impairment of CSF outflow into the lymphatics is associated with a number of pathological conditions including spaceflight-associated neuro-ocular syndrome (SANS), a problem that limits long-duration spaceflight. We used near-infrared fluorescence lymphatic imaging (NIRFLI) to dynamically visualize the deep lymphatic drainage pathways shared by CSF outflow and disrupted during head-down tilt (HDT), a method used to mimic the cephalad fluid shift that occurs in microgravity. After validating CSF clearance into the lymph nodes of the neck in swine, a pilot …

Outcomes Following Percutaneous Coronary Intervention In Patients With Cancer, Raymundo A Quintana, Dominique J Monlezun, Giovanni Davogustto, Humberto R Saenz, Francisco Lozano-Ruiz, Daisuke Sueta, Kenichi Tsujita, Uri Landes, Ali E Denktas, Mahboob Alam, David Paniagua, Daniel Addison, Hani Jneid

Faculty, Staff and Students Publications

BACKGROUND: Randomized clinical trials demonstrated the benefits of percutaneous coronary interventions (PCI) in diverse clinical settings. Patients with cancer were not routinely included in these studies.

METHODS/RESULTS: Literature search of PubMed, Cochrane, Medline, SCOPUS, EMBASE, and ClinicalTrials was conducted to identify studies that assessed one-year all-cause, cardiovascular and non-cardiovascular mortality in patients with historical or active cancer. Using the random effects model, we computed risk ratios (RRs) and standardized mean differences and their 95% confidence intervals for the dichotomous and continuous measures and outcomes, respectively. Of 171 articles evaluated in total, 5 eligible studies were included in this meta-analysis. In …

Everolimus-Induced Pneumonitis: A Diagnostic Challenge, Andrew Gulde, Elshad Hasanov, Kayla Krug, Gus W Krucke

Faculty, Staff and Student Publications

No abstract provided.

Diagnostic Criteria For Detection Of Retinal Nerve Fibre Layer Thickness And Neuroretinal Rim Width Abnormalities In Glaucoma, Feihui Zheng, Marco Yu, Christopher Kai-Shun Leung

Faculty, Staff and Student Publications

BACKGROUND/AIMS: Although measurements of the Bruch's membrane opening minimum rim width (BMO-MRW) and retinal nerve fibre layer thickness (RNFLT) with optical coherence tomography (OCT) have been widely adopted in the diagnostic evaluation of glaucoma, there is no consensus on the diagnostic criteria to define BMO-MRW and RNFLT abnormalities. This study investigated the sensitivities and specificities of different diagnostic criteria based on the OCT classification reports for detection of glaucoma.

METHODS: 340 eyes of 137 patients with glaucoma and 87 healthy individuals, all with axial length ≤26mm, had global and sectoral BMO-MRW and RNFLT measured with Spectralis OCT (Heidelberg Engineering). Six …

Final Two-Year Outcomes For The Sentry Bioconvertible Inferior Vena Cava Filter In Patients Requiring Temporary Protection From Pulmonary Embolism., Michael D Dake, Timothy P Murphy, Albrecht H Krämer, Michael D Darcy, Luke E Sewall, Michael A Curi, Matthew S Johnson, Frank Arena, James L Swischuk, Gary M Ansel, Mitchell J Silver, Souheil Saddekni, Jayson S Brower, Robert Mendes, Sentry Trial Investigators

Heart and Vascular Articles

PURPOSE: To report final 2-year outcomes with the Sentry bioconvertible inferior vena cava (IVC) filter in patients requiring temporary protection against pulmonary embolism (PE).

MATERIALS AND METHODS: In a prospective multicenter trial, the Sentry filter was implanted in 129 patients with documented deep vein thrombosis (DVT) and/or PE (67.5%) or who were at temporary risk of developing DVT/PE (32.6%). Patients were monitored and bioconversion status ascertained by radiography, computed tomography (CT), and CT venography through 2 years.

RESULTS: The composite primary 6-month endpoint of clinical success was achieved in 97.4% (111/114) of patients. The rate of new symptomatic PE was …

Rationale And Design Of The Steroids To Reduce Systemic Inflammation After Infant Heart Surgery (Stress) Trial, Kevin D Hill, H Scott Baldwin, David P Bichel, Ryan J Butts, Reid C Chamberlain, Alicia M Ellis, Eric M Graham, Jesse Hickerson, Christoph P Hornik, Jeffrey P Jacobs, Marshall L Jacobs, Robert Db Jaquiss, Prince J Kannankeril, Sean M O'Brien, Rachel Torok, Joseph W Turek, Jennifer S Li, Stress Network Investigators

Faculty, Staff and Students Publications

For decades, physicians have administered corticosteroids in the perioperative period to infants undergoing heart surgery with cardiopulmonary bypass (CPB) to reduce the postoperative systemic inflammatory response to CPB. Some question this practice because steroid efficacy has not been conclusively demonstrated and because some studies indicate that steroids could have harmful effects. STRESS is a randomized, placebo-controlled, double-blind, multicenter trial designed to evaluate safety and efficacy of perioperative steroids in infants (age < 1 year) undergoing heart surgery with CPB. Participants (planned enrollment = 1,200) are randomized 1:1 to methylprednisolone (30 mg/kg) administered into the CPB pump prime versus placebo. The trial is nested within the existing infrastructure of the Society of Thoracic Surgeons Congenital Heart Surgery Database. The primary outcome is a global rank score of mortality, major morbidities, and hospital length of stay with components ranked commensurate with their clinical severity. Secondary outcomes include several measures of major postoperative morbidity, postoperative hospital length of stay, and steroid-related safety outcomes including prevalence of hyperglycemia and postoperative infectious complications. STRESS will be one of the largest trials ever conducted in children with heart disease and will answer a decades-old question related to safety and efficacy of perioperative steroids in infants undergoing heart surgery with CPB. The pragmatic "trial within a registry" design may provide a mechanism for conducting low-cost, high-efficiency trials in a heretofore-understudied patient population.

Losartan Rescues Inflammation-Related Mucociliary Dysfunction In Relevant Models Of Cystic Fibrosis, Michael D Kim, Nathalie Baumlin, Makoto Yoshida, Deepika Polineni, Sebastian F Salathe, Joseph K David, Charles A Peloquin, Adam Wanner, John S Dennis, Juliette Sailland, Philip Whitney, Frank T Horrigan, Juan R Sabater, William M Abraham, Matthias Salathe

Faculty, Staff and Students Publications

Rationale: Despite therapeutic progress in treating cystic fibrosis (CF) airway disease, airway inflammation with associated mucociliary dysfunction remains largely unaddressed. Inflammation reduces the activity of apically expressed large-conductance Ca²⁺-activated and voltage-dependent K⁺ (BK) channels, critical for mucociliary function in the absence of CFTR (CF transmembrane conductance regulator).

Objectives: To test losartan as an antiinflammatory therapy in CF using CF human bronchial epithelial cells and an ovine model of CF-like airway disease.

Methods: Losartan’s antiinflammatory effectiveness to rescue BK activity and thus mucociliary function was tested in vitro using primary, fully redifferentiated human airway epithelial cells homozygous for …

A Retrospective Matched-Cohort Study Of 3994 Lower Extremity Wounds Of Multiple Etiologies Across 644 Institutions Comparing A Bioactive Human Skin Allograft, Theraskin, Plus Standard Of Care, To Standard Of Care Alone, Geoff C Gurtner, Aimee D Garcia, Katie Bakewell, Jason B Alarcon

Faculty, Staff and Students Publications

Most chronic wounds are related to comorbidities, for which no clinical trials are performed. This retrospective propensity matched-cohort study examined data from 2 074 000 lower extremity wounds across 644 institutions to determine the effectiveness of TheraSkin plus standard of care (SOC; n = 1997) versus SOC alone (n = 1997). Multivariate modelling comparing outcomes such as healing rates, percent area reductions (PARs), amputations, recidivism, treatment completion, and medical transfers were evaluated. A higher proportion of wounds in the treatment group compared with the controls were more likely to close (68.3% versus 60.3%), particularly wounds with exposed structures (64% versus …

Whole Exome Sequencing Of Highly Aggregated Lung Cancer Families Reveals Linked Loci For Increased Cancer Risk On Chromosomes 12q, 7p, And 4q, Anthony M Musolf, Bilal A Moiz, Haiming Sun, Claudio W Pikielny, Yohan Bossé, Diptasri Mandal, Mariza De Andrade, Colette Gaba, Ping Yang, Yafang Li, Ming You, Ramaswamy Govindan, Richard K Wilson, Elena Y Kupert, Marshall W Anderson, Ann G Schwartz, Susan M Pinney, Christopher I Amos, Joan E Bailey-Wilson

Faculty, Staff and Students Publications

BACKGROUND: Lung cancer kills more people than any other cancer in the United States. In addition to environmental factors, lung cancer has genetic risk factors as well, though the genetic etiology is still not well understood. We have performed whole exome sequencing on 262 individuals from 28 extended families with a family history of lung cancer.

METHODS: Parametric genetic linkage analysis was performed on these samples using two distinct analyses-the lung cancer only (LCO) analysis, where only patients with lung cancer were coded as affected, and the all aggregated cancers (AAC) analysis, where other cancers seen in the pedigree were …

Proteome Alterations In Pancreatic Ductal Adenocarcinoma, Sheng Pan, Teresa A Brentnall, Ru Chen

Faculty, Staff and Students Publications

Proteins are the essential functional biomolecules profoundly implicated in all aspects of pancreatic tumorigenesis and its progression. While common genomic factors, such as KRAS, TP53, SMAD4, and CDKN2A have been well recognized in association of pancreatic ductal adenocarcinoma (PDAC), our understanding of functional changes at the proteome level merits further investigation. Malignance associated proteome alterations can be attributed to the convoluted outcomes from genetic, epigenetic and environmental factors in initiating and progressing PDAC, and may reflect on changes in protein expressional level, structure, localization, as well as post-translational modifications (PTMs) status. The study of localized or systemic proteome alterations in …

Metabolic Dysregulation In The Atp7b-/- Wilson's Disease Mouse Model, Clavia Ruth Wooton-Kee, Matthew Robertson, Ying Zhou, Bingning Dong, Zhen Sun, Kang Ho Kim, Hailan Liu, Yong Xu, Nagireddy Putluri, Pradip Saha, Cristian Coarfa, David D Moore, Alli M Nuotio-Antar

Faculty, Staff and Students Publications

Inactivating mutations in the copper transporter Atp7b result in Wilson’s disease. The Atp7b−/− mouse develops hallmarks of Wilson’s disease. The activity of several nuclear receptors decreased in Atp7b−/− mice, and nuclear receptors are critical for maintaining metabolic homeostasis. Therefore, we anticipated that Atp7b−/− mice would exhibit altered progression of diet-induced obesity, fatty liver, and insulin resistance. Following 10 wk on a chow or Western-type diet (40% kcal fat), parameters of glucose and lipid homeostasis were measured. Hepatic metabolites were measured by liquid chromatography–mass spectrometry and correlated with transcriptomic data. Atp7b−/− mice fed a chow diet presented …

The Mitochondrial Hsp90 Paralog Trap1 Forms An Oxphos-Regulated Tetramer And Is Involved In Mitochondrial Metabolic Homeostasis, Abhinav Joshi, Li Dai, Yanxin Liu, Jungsoon Lee, Nastaran Mohammadi Ghahhari, Gregory Segala, Kristin Beebe, Lisa M Jenkins, Gaelyn C Lyons, Lilia Bernasconi, Francis T F Tsai, David A Agard, Len Neckers, Didier Picard

Faculty, Staff and Students Publications

BACKGROUND: The molecular chaperone TRAP1, the mitochondrial isoform of cytosolic HSP90, remains poorly understood with respect to its pivotal role in the regulation of mitochondrial metabolism. Most studies have found it to be an inhibitor of mitochondrial oxidative phosphorylation (OXPHOS) and an inducer of the Warburg phenotype of cancer cells. However, others have reported the opposite, and there is no consensus on the relevant TRAP1 interactors. This calls for a more comprehensive analysis of the TRAP1 interactome and of how TRAP1 and mitochondrial metabolism mutually affect each other.

RESULTS: We show that the disruption of the gene for TRAP1 in …

Elagolix For Heavy Menstrual Bleeding In Women With Uterine Fibroids., William D. Schlaff, Ronald T. Ackerman, Ayman Al-Hendy, David F. Archer, Kurt T. Barnhart, Linda D. Bradley, Bruce R. Carr, Eve C. Feinberg, Sandra M. Hurtado, Jinhee Kim, Ran Liu, R. Garn Mabey, Charlotte D. Owens, Alfred Poindexter, Elizabeth E. Puscheck, Henry Rodriguez-Ginorio, James A. Simon, Ahmed M. Soliman, Elizabeth A. Stewart, Nelson B. Watts, Ozgul Muneyyirci-Delale

Department of Obstetrics and Gynecology Faculty Papers

BACKGROUND: Uterine fibroids are hormone-responsive neoplasms that are associated with heavy menstrual bleeding. Elagolix, an oral gonadotropin-releasing hormone antagonist resulting in rapid, reversible suppression of ovarian sex hormones, may reduce fibroid-associated bleeding.

METHODS: We conducted two identical, double-blind, randomized, placebo-controlled, 6-month phase 3 trials (Elaris Uterine Fibroids 1 and 2 [UF-1 and UF-2]) to evaluate the efficacy and safety of elagolix at a dose of 300 mg twice daily with hormonal "add-back" therapy (to replace reduced levels of endogenous hormones; in this case, estradiol, 1 mg, and norethindrone acetate, 0.5 mg, once daily) in women with fibroid-associated bleeding. An elagolix-alone …

Review Of Alterations In Perlecan-Associated Vascular Risk Factors In Dementia, Amanda L. Trout, Ibolya Rutkai, Ifechukwude J. Biose, Gregory J. Bix

Neurology Faculty Publications

Perlecan is a heparan sulfate proteoglycan protein in the extracellular matrix that structurally and biochemically supports the cerebrovasculature by dynamically responding to changes in cerebral blood flow. These changes in perlecan expression seem to be contradictory, ranging from neuroprotective and angiogenic to thrombotic and linked to lipid retention. This review investigates perlecan's influence on risk factors such as diabetes, hypertension, and amyloid that effect Vascular contributions to Cognitive Impairment and Dementia (VCID). VCID, a comorbidity with diverse etiology in sporadic Alzheimer's disease (AD), is thought to be a major factor that drives the overall clinical burden of dementia. Accordingly, changes …

Rhoa-Rock Signaling As A Therapeutic Target In Traumatic Brain Injury, Shalaka Mulherkar, Kimberley F Tolias

Faculty, Staff and Students Publications

Traumatic brain injury (TBI) is a leading cause of death and disability worldwide. TBIs, which range in severity from mild to severe, occur when a traumatic event, such as a fall, a traffic accident, or a blow, causes the brain to move rapidly within the skull, resulting in damage. Long-term consequences of TBI can include motor and cognitive deficits and emotional disturbances that result in a reduced quality of life and work productivity. Recovery from TBI can be challenging due to a lack of effective treatment options for repairing TBI-induced neural damage and alleviating functional impairments. Central nervous system (CNS) …

Evidence For The Misfolding Of The A1 Domain Within Multimeric Von Willebrand Factor In Type 2 Von Willebrand Disease, Alexander Tischer, Maria A Brehm, Venkata R Machha, Laurie Moon-Tasson, Linda M Benson, Katelynn J Nelton, Rachel R Leger, Tobias Obser, Marina Martinez-Vargas, Steven T Whitten, Dong Chen, Rajiv K Pruthi, H Robert Bergen, Miguel A Cruz, Reinhard Schneppenheim, Matthew Auton

Faculty, Staff and Students Publications

Von Willebrand factor (VWF), an exceptionally large multimeric plasma glycoprotein, functions to initiate coagulation by agglutinating platelets in the blood stream to sites of vascular injury. This primary hemostatic function is perturbed in type 2 dysfunctional subtypes of von Willebrand disease (VWD) by mutations that alter the structure and function of the platelet GPIbα adhesive VWF A1 domains. The resulting amino acid substitutions cause local disorder and misfold the native structure of the isolated platelet GPIbα-adhesive A1 domain of VWF in both gain-of-function (type 2B) and loss-of-function (type 2M) phenotypes. These structural effects have not been explicitly observed in A1 …

Development And Characterization Of A Wee1 Kinase Degrader, Zhengnian Li, Benika J Pinch, Calla M Olson, Katherine A Donovan, Radosław P Nowak, Caitlin E Mills, David A Scott, Zainab M Doctor, Nicholas A Eleuteri, Mirra Chung, Peter K Sorger, Eric S Fischer, Nathanael S Gray

Faculty, Staff and Students Publications

The G1/S cell cycle checkpoint is frequently dysregulated in cancer, leaving cancer cells reliant on a functional G2/M checkpoint to prevent excessive DNA damage. Wee1 regulates the G2/M checkpoint by phosphorylating CDK1 at Tyr15 to prevent mitotic entry. Previous drug development efforts targeting Wee1 resulted in the clinical-grade inhibitor, AZD1775. However, AZD1775 is burdened by dose-limiting adverse events, and has off-target PLK1 activity. In an attempt to overcome these limitations, we developed Wee1 degraders by conjugating AZD1775 to the cereblon (CRBN)-binding ligand, pomalidomide. The resulting lead compound, ZNL-02-096, degrades Wee1 while sparing PLK1, induces G2/M accumulation at 10-fold lower doses …

Outcomes In Pediatric Studies Of Medium-Chain Acyl-Coa Dehydrogenase (Mcad) Deficiency And Phenylketonuria (Pku): A Review., Michael Pugliese, Kylie Tingley, Andrea Chow, Nicole Pallone, Maureen Smith, Alvi Rahman, Pranesh Chakraborty, Michael T Geraghty, Julie Irwin, Laure Tessier, Stuart G Nicholls, Martin Offringa, Nancy J Butcher, Ryan Iverson, Tammy J Clifford, Sylvia Stockler, Brian Hutton, Karen Paik, Jessica Tao, Becky Skidmore, Doug Coyle, Kathleen Duddy, Sarah Dyack, Cheryl R Greenberg, Shailly Jain Ghai, Natalya Karp, Lawrence Korngut, Jonathan Kronick, Alex Mackenzie, Jennifer Mackenzie, Bruno Maranda, John J Mitchell, Murray Potter, Chitra Prasad, Andreas Schulze, Rebecca Sparkes, Monica Taljaard, Yannis Trakadis, Jagdeep Walia, Beth K Potter

Paediatrics Publications

BACKGROUND: Inherited metabolic diseases (IMDs) are a group of individually rare single-gene diseases. For many IMDs, there is a paucity of high-quality evidence that evaluates the effectiveness of clinical interventions. Clinical effectiveness trials of IMD interventions could be supported through the development of core outcome sets (COSs), a recommended minimum set of standardized, high-quality outcomes and associated outcome measurement instruments to be incorporated by all trials in an area of study. We began the process of establishing pediatric COSs for two IMDs, medium-chain acyl-CoA dehydrogenase (MCAD) deficiency and phenylketonuria (PKU), by reviewing published literature to describe outcomes reported by authors, …

Increased Episodes Of Aspiration On Videofluoroscopic Swallow Study In Children With Nasogastric Tube Placement., Sarah T. Edwards, Linda Ernst, Ashley K. Sherman, Ann M. Davis

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Given the limited evidence available, the impact of nasogastric (NG) tube placement on swallowing in children is not well understood. When a child needs to be fed enterally, the current standard is to initially place an NG tube and leave it in place for the first few months of supplemental or total enteral nutrition. It is important to understand if placement of NG tubes has a negative effect on a patient's swallow.

METHODS: We retrospectively reviewed the charts of those children who had videofluoroscopic swallow studies (VFSS) to identify all children who had an NG tube in place at …

Fatigue In Young People With Duchenne Muscular Dystrophy, Basmah El-Aloul, Kathy N Speechley, Yi Wei, Piotr Wilk, Craig Campbell

Paediatrics Publications

AIM: To describe fatigue in Duchenne muscular dystrophy (DMD) from patients' and parents' perspectives and to explore risk factors for fatigue in children and adolescents with DMD.

METHOD: A multicentre, cross-sectional study design was used. Seventy-one patients (all males; median age 12y, age range 5-17y) identified via the Canadian Neuromuscular Disease Registry, and their parents completed questionnaires. Subjective fatigue was assessed using the Pediatric Quality of Life Inventory Multidimensional Fatigue Scale by child self-report and parent proxy-report.

RESULTS: Patients with DMD across ages and disease stages experienced greater fatigue compared to typically developing controls from published data. Sleep disturbance symptoms …

Covid-19 Psychosis: A Potential New Neuropsychiatric Condition Triggered By Novel Coronavirus Infection And The Inflammatory Response?, S. J. Ferrando, L. Klepacz, S. Lynch, M. Tavakkoli, R. Dornbush, R. Baharani, Y. Smolin, A. Bartell

Journal Articles

No abstract provided.

Introduction To The Special Series: Translating Behavioral Medicine Research To Prevent And Control The Spread Of Covid-19., A. M. Delamater, S. M. Miller, D. J. Bowen, M. A. Diefenbach, K. P. Tercyak

Journal Articles

No abstract provided.