Medical Specialties Commons^™

Acute Cranial Neuropathies Heralding Neurosyphilis In Human Immunodeficiency Virus-Infected Patient., Saeed Alqahtani

Neurology Faculty Publications

No abstract provided.

Ruptured Splenic Artery Aneurysms And The Use Of An Adapted Fast Protocol In Reproductive Age Women With Hemodynamic Collapse: Case Series, Hope T. Jackson, Silviu C. Diaconu, Patrick J. Maluso, Bruce Abell, Juliet Lee

Surgery Faculty Publications

Nontraumatic symptomatic hypotension in all patients requires prompt diagnosis and appropriate treatment for optimum outcome. The female population specifically has an expanded differential diagnosis that should be considered when these patients present with hemodynamic collapse. While the most common causes of hypotension in pregnant patients are dehydration, ruptured ectopic pregnancy, and placental and uterine abnormalities, less common nonobstetrical etiologies such as hepatic rupture and ruptured abdominal and visceral artery aneurysms should also be considered. Splenic artery aneurysms are associated with high rates of mortality and in cases of pregnancy, maternal and fetal mortality. These high rates can be attributed to …

Loss Of Nuclear Pten In Hcv-Infected Human Hepatocytes, Wenjie Bao, Liliana Florea, Ningbin Wu, Zhao Wang, Krishna K. Banaudha, Jason Qian, Laurent Houzet, Rakesh Kumar, Ajit Kumar

Surgery Faculty Publications

Background

Hepatitis C virus (HCV) infection is a major risk factor for chronic hepatitis and hepatocellular carcinoma (HCC); however, the mechanism of HCV-mediated hepatocarcinogenesis is not well understood. Insufficiency of PTEN tumor suppressor is associated with more aggressive cancers, including HCC. We asked whether viral non-coding RNA could initiate oncogenesis in HCV infected human hepatocytes. The results presented herein suggest that loss of nuclear PTEN in HCV-infected human hepatocytes results from depletion of Transportin-2, which is a direct target of viral non-coding RNA, vmr11.

Methods

The intracellular distribution of PTEN in HCV-infected cells was monitored by immunostaining and Western blots …

Hereditary Angioedema: What The Gastroenterologist Needs To Know, M. Aamir Ali, Marie L. Borum

Surgery Faculty Publications

Up to 93% of patients with hereditary angioedema (HAE) experience recurrent abdominal pain. Many of these patients, who often present to emergency departments, primary care physicians, general surgeons, or gastroenterologists, are misdiagnosed for years and undergo unnecessary testing and surgical procedures. Making the diagnosis of HAE can be challenging because symptoms and attack locations are often inconsistent from one episode to the next. Abdominal attacks are common and can occur without other attack locations. An early, accurate diagnosis is central to managing HAE. Unexplained abdominal pain, particularly when accompanied by swelling of the face and extremities, suggests the diagnosis of …

Experience With Miniaturized Cardiopulmonary Bypass In Cardiac Surgery: A Prospective Comparison Of The Novosci Ready System To Off-Pump And Conventional Coronary Artery Bypass Grafting, Dominic Emerson, Debbie Baldwin, Miguel Pinales, Michael Greenberg, Melissa Mattes, Gregory D. Trachiotis

Surgery Faculty Publications

The major source of morbidity following cardiopulmonary bypass (CPB) is the systemic inflammatory response (SIRS response) which leads to multiple derangements in different organ systems. To combat this, miniaturized cardiopulmonary bypass circuits (MCPBC) have been created to lessen the inflammatory response to CPB. Here we examine early outcomes following coronary artery bypass grafting (CABG) using a MCPBC system compared to conventional bypass techniques at a single institution. Methods: 60 consecutive patients undergoing elective CABG were prospectively enrolled. Nine patients underwent coronary artery bypass grafting (CABG) with conventional CPB (cCABG), 33 underwent off-pump CABG (OPCAB), and the remaining 18 patients underwent …

A Pilot Academic-Community Partnership To Advance The D.C. Healthy Schools Act, Nisha Narayanan, Jonathan Tatum, Margarita Ramos, Jessica Kim, Nisha Punatar, Michele L. Mietus-Snyder

GW Research Days 2014

No abstract provided.

High Dose Insulin Therapy, An Evidence Based Approach To Beta Blocker/Calcium Channel Blocker Toxicity, Christina Woodward, Ali Pourmand, Maryann Mazer-Amirshahi

Emergency Medicine Faculty Publications

Poison-induced cardiogenic shock (PICS) as a result of beta-blocker (?-blocker) or calcium channel blocker (CCB) overdose is a common and potentially life-threatening condition. Conventional therapies, including fluid resuscitation, atropine, cardiac pacing, calcium, glucagon, and vasopressors often fail to improve hemodynamic status. High-dose insulin (HDI) is an emerging therapeutic modality for PICS. In this article, we discuss the existing literature and highlight the therapeutic success and potential of HDI. Based on the current literature, which is limited primarily to case series and animal models, the authors conclude that HDI can be effective in restoring hemodynamic stability, and recommend considering its use …

Do Emergency Department Patients Receive A Pathological Diagnosis? A Nationally Representative Sample., Leana S. Wen, Janice A. Espinola, Joshua M. Kosowsky, Carlos A. Camargo Jr

Emergency Medicine Faculty Publications

No abstract provided.

Thiamine Pyrophosphokinase Deficiency Causes A Leigh Disease Like Phenotype In A Sibling Pair: Identification Through Whole Exome Sequencing And Management Strategies, Jamie L. Fraser, Adeline Vanderver, Sandra Yang, Taeun Chang, Laura Cramp, Gilbert Vezina, Uta Lichter-Konecki, Kristina Cusmano-Ozog, Patroula Smpokou, Kimberly A. Chapman, Dina Zand

Neurology Faculty Publications

We present a sibling pair with Leigh-like disease, progressive hypotonia, regression, and chronic encephalopathy. Whole exome sequencing in the younger sibling demonstrated a homozygous thiamine pyrophosphokinase (TPK) mutation. Initiation of high dose thiamine, niacin, biotin, α-lipoic acid and ketogenic diet in this child demonstrated improvement in neurologic function and re-attainment of previously lost milestones. The diagnosis of TPK deficiency was difficult due to inconsistent biochemical and diagnostic parameters, rapidity of clinical demise and would not have been made in a timely manner without the use of whole exome sequencing. Molecular diagnosis allowed for attempt at dietary modification with cofactor supplementation …

Minor Gynecologic Surgery: A Review Of The Training Experience And Skill Building Opportunities For Providers In Low And Middle Income Countries, Rachel Marie Clark, Leslie Siriya Bradford, Jessica Opoku-Anane, Joseph Ngonzi, Ferdous Islam, Mithila Faruque, Annekathryn Goodman

Obstetrics and Gynecology Faculty Publications

Purpose: Minor gynecologic surgery is the cornerstone of gynecologic evaluation and intervention in countries with a well-established medical infrastructure. Surgical training and exposure to minor procedures are not available in low and middle-income countries due to the complex challenges of patient delay and lack of access to healthcare, physician shortages, and the lack of ancillary services such as pathology and radiology. This paper reviews current training statistics, the international literature on minor gynecologic surgery and training strategies.

Methods: PubMed searches using MESH terms cone biopsy, dilation and curettage, and loop electrosurgical excision procedure were performed. Statistics of minor …

Mucopolysaccharidosis Iva (Morquio A Syndrome) And Vi (Maroteaux-Lamy Syndrome): Under-Recognized And Challenging To Diagnose, Ralph S. Lachman, Barbara K. Burton, Lorne A. Clarke, Scott Hoffinger, Shiro Ikegawa, Dong-Kyu Jin, Hiroki Kano, Ok-Hwa Kim, Christina Lampe, Nancy J. Mendelsohn, Renee Shediac, Pranoot Tanpaiboon, Klane K. White

Pediatrics Faculty Publications

OBJECTIVE:

Mucopolysaccharidosis IVA (MPS IVA, or Morquio A syndrome) and VI (MPS VI, or Maroteaux-Lamy syndrome) are autosomal recessive lysosomal storage disorders. Skeletal abnormalities are common initial presenting symptoms and, when recognized early, may facilitate timely diagnosis and intervention, leading to improved patient outcomes. Patients with slowly progressing disease and nonclassic phenotypes can be particularly challenging to diagnose. The objective was to describe the radiographic features of patients with a delayed diagnosis of MPS IVA or VI.

MATERIALS AND METHODS:

This was a retrospective study. The records of 5 MPS IVA and 3 MPS VI patients with delayed diagnosis were …

Gauging Regional Differences In The Hiv Prevalence Rate Among Injection Drug Users In The U.S., Jichuan Wang, Brian Kelly

Pediatrics Faculty Publications

This article aims to introduce and demonstrate the application of the standardization and decomposition analysis (SDA) method to gauge differences in HIV prevalence rates among injection drug users (IDUs) across regions (Northeast, South, Midwest, and West) in the U.S. Using the SDA, the regional HIV prevalence rates were standardized and a rate difference between regions was decomposed into component effects, such as the “real” rate difference, and component effects attributed to differences in specific compositions of confounding factors. A total of 9,824 injection drug users (IDUs) retrieved from the national database of the National Institute on Drug Abuse’s Cooperative Agreement …

Superresolution Imaging Of Human Cytomegalovirus Vmia Localization In Sub-Mitochondrial Compartments, Shivaprasad Bhuvanendran, Kyle Salka, Kristen Rainey, Sen Chandra Sreetama, Elizabeth Williams, Margretha Leeker, Vidhya Prasad, Jonathan Boyd, George H. Patterson, Jyoti K. Jaiswal, Anamaris M. Colberg-Poley

Pediatrics Faculty Publications

The human cytomegalovirus (HCMV) viral mitochondria-localized inhibitor of apoptosis (vMIA) protein, traffics to mitochondria-associated membranes (MAM), where the endoplasmic reticulum (ER) contacts the outer mitochondrial membrane (OMM). vMIA association with the MAM has not been visualized by imaging. Here, we have visualized this by using a combination of confocal and superresolution imaging. Deconvolution of confocal microscopy images shows vMIA localizes away from mitochondrial matrix at the Mitochondria-ER interface. By gated stimulated emission depletion (GSTED) imaging, we show that along this interface vMIA is distributed in clusters. Through multicolor, multifocal structured illumination microscopy (MSIM), we find vMIA clusters localize away from …

Perinatal Pharmacology, Karel M. Allegaert, V. Fanos, John N. Van Den Anker, Stephanie L. Läer

Pediatrics Faculty Publications

No abstract provided.

Rheumatic Heart Disease In Uganda: The Association Between Mhc Class Ii Hla Dr Alleles And Disease: A Case Control Study, Emmy Okello, Andrea Z. Beaton, Charles Mondo, Paul Kruszka, Noah Kiwanuka, Richard Odoi-Adome, Juergen Freers

Pediatrics Faculty Publications

Background

Rheumatic heart disease (RHD), the only long term consequence of acute rheumatic fever, remains a leading cause of morbidity and mortality among young adults in Uganda. An inherited susceptibility to acute rheumatic fever centers around the major histocompatibility class II human leucocyte antigens. However, there is paucity of data from sub-Saharan Africa. This study compares the frequency of HLA class II DR alleles between RHD cases and normal controls in Uganda.

Methods

One hundred ninety-nine participants including 96 established RHD cases aged 5–60 years and 103 age and sex matched normal controls were recruited for participation. DNA was manually …

Preface Of The Special Issue: “Recent Cmv Research”, Kayla Dufrene, Roberta L. Debiasi, Anamaris M. Colberg-Poley

Pediatrics Faculty Publications

No abstract provided.

Expression Of A Secreted Fibroblast Growth Factor Binding Protein-1 (Fgfbp1) In Angioproliferative Kaposi Sarcoma, Patricio E. Ray, Ali Al-Attar, Xue-Hui Liu, Jharna R. Das, Elena Tassi, Anton Wellstein

Pediatrics Faculty Publications

Objective: Kaposi’s sarcoma (KS) is an angioproliferative disease frequently seen in patients with the acquired immunodeficiency syndrome (AIDS). Previous studies suggest that the HIV-1 protein Tat and Fibroblast Growth Factor 2 (FGF-2) have synergistic angiogenic effects in AIDS-KS tumors. However, the mechanisms by which FGF-2 is released and activated in KS tumors are not clearly defined. We carried out this study to determine whether an FGFbinding protein (FGFBP1 or BP1) that enhances the angiogenic activity of FGF-2 is expressed in AIDS-KS tumors, and to define whether BP1, FGF-2, and HIV-Tat protein-protein interactions could play a potential clinically role in the …

Children's Hospital Association Consensus Statements For Comorbidities Of Childhood Obesity, Elizabeth Estrada, Ihuoma Eneli, Sarah Hampl, Michele L. Mietus-Snyder, Nazrat Mirza, Erinn Rhodes, Brooke Sweeney, Lydia Tinjaero-Deck, Susan J. Woolford, Stephen J. Pont

Pediatrics Faculty Publications

Background: Childhood obesity and overweight affect approximately 30% of US children. Many of these children have obesity-related comorbidities, such as hypertension, dyslipidemia, fatty liver disease, diabetes, polycystic ovary syndrome (PCOS), sleep apnea, psychosocial problems, and others. These children need routine screening and, in many cases, treatment for these conditions. However, because primary care pediatric providers (PCPs) often are underequipped to deal with these comorbidities, they frequently refer these patients to subspecialists. However, as a result of the US pediatric subspecialist shortage and considering that 12.5 million children are obese, access to care by subspecialists is limited. The aim of this …

Working Together For Global Health Goals: The United States Agency For International Development And Faith-Based Organizations, Clydette L. Powell

Pediatrics Faculty Publications

For many years, and before the term “FBO” was used for faith-based organizations, the United States Agency for International Development (USAID) has supported the work of FBOs in global health and development. The Agency has long recognized the impact of FBOs within that development space, because these organizations are often well positioned to reach the hard-to-reach and to go the last mile because of their strong ties to the community. Moreover, FBOs deliver a substantial percentage of the health services in some developing countries. Faith partners, whether Catholic, Protestant, Buddhist, Hindu, Muslim, or other, have an important role to play …

Proposed Guidelines For The Diagnosis And Management Of Methylmalonic And Propionic Acidemia., Matthias R Baumgartner, Friederike Hörster, Carlo Dionisi-Vici, Goknur Haliloglu, Daniela Karall, Kimberly A. Chapman, +19 Additional Authors

Pediatrics Faculty Publications

Methylmalonic and propionic acidemia (MMA/PA) are inborn errors of metabolism characterized by accumulation of propionic acid and/or methylmalonic acid due to deficiency of methylmalonyl-CoA mutase (MUT) or propionyl-CoA carboxylase (PCC). MMA has an estimated incidence of ~ 1: 50,000 and PA of ~ 1:100'000 -150,000. Patients present either shortly after birth with acute deterioration, metabolic acidosis and hyperammonemia or later at any age with a more heterogeneous clinical picture, leading to early death or to severe neurological handicap in many survivors. Mental outcome tends to be worse in PA and late complications include chronic kidney disease almost exclusively in MMA …

Aspirin Use And Knowledge In The Community: A Population- And Health Facility Based Survey For Measuring Local Health System Performance., Gregory A Roth, Catherine W. Gillespie, Ali A Mokdad, Danny D Shen, David W Fleming, Andy Stergachis, Christopher J L Murray, Ali H Mokdad

Pediatrics Faculty Publications

BACKGROUND: Little is known about the relationship between cardiovascular risk, disease and actual use of aspirin in the community.

METHODS: The Measuring Disparities in Chronic Conditions (MDCC) study is a community and health facility-based survey designed to track disparities in the delivery of health interventions for common chronic diseases. MDCC includes a survey instrument designed to collect detailed information about aspirin use. In King County, WA between 2011 and 2012, we surveyed 4633 white, African American, or Hispanic adults (45% home address-based sample, 55% health facility sample). We examined self-reported counseling on, frequency of use and risks of aspirin for …

Immunologic Special Forces: Anti-Pathogen Cytotoxic T-Lymphocyte Immunotherapy Following Hematopoietic Stem Cell Transplantation, Michael Keller, Catherine M. Bollard

Pediatrics Faculty Publications

Anti-pathogen adoptive T-cell immunotherapy has been proven to be highly effective in preventing or controlling viral infections following hematopoietic stem cell transplantation. Recent advances in manufacturing protocols allow an increased number of targeted pathogens, eliminate the need for viral transduction, broaden the potential donor pool to include pathogen-naïve sources, and reduce the time requirement for production. Early studies suggest that anti-fungal immunotherapy may also have clinical benefit. Future advances include further broadening of the pathogens that can be targeted and development of T-cells with resistance to pharmacologic immunosuppression.

Intermittent Acute Angle Closure Glaucoma And Chronic Angle Closure Following Topiramate Use With Plateau Iris Configuration, Lamise Z. Rajjoub, Nisha Chadha, David A. Belyea

Ophthalmology Faculty Publications

This is a case report describing recurrent intermittent acute angle closure episodes in the setting of topiramate use in a female suffering from migraines. Despite laser peripheral iridotomy placement for the pupillary block component, and the discontinuation of topiramate, the acute angle closure did not resolve in the left eye with chronic angle closure and the patient required urgent trabeculectomy. The right eye responded to laser peripheral iridotomy immediately and further improved after the cessation of topiramate. While secondary angle closure glaucoma due to topiramate use has been widely reported, its effects in patients with underlying primary angle closure glaucoma …

Emerging Biomarkers And Metabolomics For Assessing Toxic Nephropathy And Acute Kidney Injury (Aki) In Neonatology, M. Mussap, A. Noto, V. Fanos, John N. Van Den Anker

Pediatrics Faculty Publications

Identification of novel drug-induced toxic nephropathy and acute kidney injury (AKI) biomarkers has been designated as a top priority by the American Society of Nephrology. Increasing knowledge in the science of biology and medicine is leading to the discovery of still more new biomarkers and of their roles in molecular pathways triggered by physiological and pathological conditions. Concomitantly, the development of the so-called “omics” allows the progressive clinical utilization of a multitude of information, from those related to the human genome (genomics) and proteome (proteomics), including the emerging epigenomics, to those related to metabolites (metabolomics). In preterm newborns, one of …

Autosomal Recessive Polycystic Kidney Disease: The Prototype Of The Hepato-Renal Fibrocystic Diseases, Lisa Guay-Woodford

Pediatrics Faculty Publications

Autosomal recessive polycystic kidney disease (ARPKD) is a severe, typically early onset form of renal cystic disease. The care of ARPKD patients has traditionally been the purview of pediatric nephrologists for management of systemic hypertension and progressive renal insufficiency. However, the disease has multisystem manifestations and a comprehensive care strategy frequently requires a multidisciplinary team. In severely affected infants, the diagnosis often is first suspected by obstetricians when enlarged, echogenic kidneys and oligohydramnios are detected on prenatal ultrasounds. Neonatologists are central to the care of these infants, who may have respiratory compromise due to pulmonary hypoplasia and massively enlarged kidneys. …

Low-Dose Ketamine For Children And Adolescents With Acute Sickle Cell Disease Related Pain: A Single Center Experience, Caitlin M. Neri, Sophie R. Pestieau, Heather Young, Angelo Elmi, Julia C. Finkel, Deepika S. Darbari

Pediatrics Faculty Publications

Background: Opioids are the mainstay of therapy for painful vasoocclusive episodes (VOEs) in sickle cell disease (SCD). Based on limited studies, low-dose ketamine could be a useful adjuvant analgesic for refractory SCD pain, but its safety and efficacy has not been evaluated in pediatric SCD.

Procedure: Using retrospective chart review we recorded and compared characteristics of hospitalizations of 33 children with SCD hospitalized with VOE who were treated with low-dose ketamine and opioid PCA vs. a paired hospitalization where the same patients received opioid PCA without ketamine. We seek to 1) describe a single center experience using adjuvant low-dose ketamine …

Broad And Direct Interaction Between Tlr And Siglec Families Of Pattern Recognition Receptors And Its Regulation By Neu1., Guo-Yun Chen, Nicholas K. Brown, Wei Wu, Zahra Khedri, Hai Yu, Xi Chen, Diantha Van De Vlekkert, Alessandra D'Azzo, Pan Zheng, Yang Liu

Pediatrics Faculty Publications

Both pathogen- and tissue damage-associated molecular patterns induce inflammation through toll-like receptors (TLRs), while sialic acid-binding immunoglobulin superfamily lectin receptors (Siglecs) provide negative regulation. Here we report extensive and direct interactions between these pattern recognition receptors. The promiscuous TLR binders were human SIGLEC-5/9 and mouse Siglec-3/E/F. Mouse Siglec-G did not show appreciable binding to any TLRs tested. Correspondingly, Siglece deletion enhanced dendritic cell responses to all microbial TLR ligands tested, while Siglecg deletion did not affect the responses to these ligands. TLR4 activation triggers Neu1 translocation to cell surface to disrupt TLR4:Siglec-E interaction. Conversely, sialidase inhibitor Neu5Gc2en prevented TLR4 ligand-induced …

Characterization Of Cytomegalovirus Lung Infection In Non-Hiv Infected Children, Sonia M. Restrepo-Gualteros, Lina E. Jaramillo-Barberi, Monica Gonzalez-Santos, Carlos E. Rodriguez-Martinez, Geovanny F. Perez, Maria J. Gutierrez, Gustavo Nino

Pediatrics Faculty Publications

Cytomegalovirus (CMV) is a prevalent pathogen in the immunocompromised host and invasive pneumonia is a feared complication of the virus in this population. In this pediatric case series we characterized CMV lung infection in 15 non-HIV infected children (median age 3 years; IQR 0.2–4.9 years), using current molecular and imaging diagnostic modalities, in combination with respiratory signs and symptoms. The most prominent clinical and laboratory findings included cough (100%), hypoxemia (100%), diffuse adventitious breath sounds (100%) and increased respiratory effort (93%). All patients had abnormal lung images characterized by ground glass opacity/consolidation in 80% of cases. CMV was detected in …

Population Pharmacokinetics Of Ciprofloxacin In Neonates And Young Infants Less Than 3 Months Age, Wei Zhao, Helen Hill, Chantal Le Guellec, Tim Neal, Sarah Mahoney, Stephanie Paulus, Charlotte Castellan, Behrouz Kassai, John N. Van Den Anker, Gregory L. Kearns, Mark A. Turner, Evelyne Jacqz-Aigrain

Pediatrics Faculty Publications

Ciprofloxacin is used in neonates with suspected or documented Gram-negative serious infections. Currently, its use is off-label partly because of lack of pharmacokinetic studies. Within the FP7 EU project TINN (Treat Infection in NeoNates), our aim was to evaluate the population pharmacokinetics of ciprofloxacin in neonates and young infants <3 months of age and define the appropriate dose in order to optimize ciprofloxacin treatment in this vulnerable population. Blood samples were collected from neonates treated with ciprofloxacin and concentrations were quantified by high-pressure liquid chromatography-mass spectrometry. Population pharmacokinetic analysis was performed using NONMEM software. The data from 60 newborn infants (postmenstrual age [PMA] range, 24.9 to 47.9 weeks) were available for population pharmacokinetic analysis. A two-compartment model with first-order elimination showed the best fit with the data. A covariate analysis identified that gestational age, postnatal age, current weight, serum creatinine concentration, and use of inotropes had a significant impact on ciprofloxacin pharmacokinetics. Monte Carlo simulation demonstrated that 90% of hypothetical newborns with a PMA of <34 weeks treated with 7.5 mg/kg twice daily and 84% of newborns with a PMA ≥34 weeks and young infants receiving 12.5 mg/kg twice daily would reach the AUC/MIC target of 125, using the standard EUCAST MIC susceptibility breakpoint of 0.5 mg/liter. The associated risks of overdose for the proposed dosing regimen were <8%. The population pharmacokinetics of ciprofloxacin was evaluated in neonates and young infants <3 months old, and a dosing regimen was established based on simulation.

Cerebral Pressure Passivity In Newborns With Encephalopathy Undergoing Therapeutic Hypothermia, Rathinaswamy B. Govindan, An Nguyen Massaro, Nickie N. Andescavage, Taeun Chang, Adre J. Du Plessis

Neurology Faculty Publications

We extended our recent modification of the power spectral estimation approach to quantify spectral coherence. We tested both the standard and the modified approaches on simulated data, which showed that the modified approach was highly specific and sensitive to the coupling introduced in the simulation while the standard approach lacked these features. We also applied the modified and standard approaches to quantify the pressure passivity in 4 infants receiving therapeutic hypothermia. This was done by measuring the coupling between continuous cerebral hemoglobin differences and mean arterial blood pressure. Our results showed that the modified approach identified a lower pressure passivity …