Medicine and Health Sciences Commons^™

A Case Report Of A Primary Cerebral Abscess Due To Nocardia Asteroides In An Immunocompetent Patient, Ileana Elita Mendez, Nicole Marie Barreto, Alan Lanurias, Luis Alberto Rosas

Research Symposium

Background: Brain abscesses caused by Nocardia are rarely reported and have high mortality. It usually affects patients who are immunocompromised and initiates as a lung infection that disseminates hematogenously, though it can also affect those immunocompetent. Cerebral nocardiosis imitates brain tumors, which can delay the initiation of the appropriate antibiotic therapy.

Case Description: We present a case of a 64-year-old immunocompetent male with multiple cerebral abscesses who presented with headaches, left-sided weakness, and ataxia. Upon imaging, multiple brain lesions were revealed. The patient underwent craniotomy and drainage of the mass. He was initially empirically treated, though cultures later grew Nocardia …

A New Hybrid Graft For Open Thoracoabdominal Aortic Aneurysm Repair, Sabine Helena Wipper, Tilo Kölbel, Julia Dumfarth, Hubert Schelzig, Anthony L Estrera, Harleen K Sandhu, Florian Enzmann, E Sebastian Debus

Faculty, Staff and Student Publications

No abstract provided.

Spontaneous Celiac Artery Dissection Treated By Balloon Angioplasty, Emanuel R Tenorio, Gustavo S Oderich

Faculty, Staff and Student Publications

No abstract provided.

Bad To The Bone. Not All Bone Tumors Are Cancer: Case Of Long Bone Osteomyelitis, Tat W. Yau, Bennet D. Franz, Hanadi A. Osman, Jessica C. Rivera, Ashaur Azhar

School of Medicine Faculty Publications

Long bone osteomyelitis could mimic bony tumor in clinical presentation and imaging studies. We present a case of a 47-year man who presented with leg pain, weight loss and night sweats that initially was thought to be related to osteosarcoma, later suffered a pathologic fracture from Staphylococcus aureus osteomyelitis. This case highlights the importance of source control of infection and careful clinical evaluation including radiographic and pathologic findings that can help physicians to differentiate between competing diagnoses.

Insulin Resistance And Insulin Handling In Chronic Kidney Disease, Vishnu P Parvathareddy, Jiao Wu, Sandhya S Thomas

Faculty, Staff and Students Publications

Insulin regulates energy metabolism involving multiple organ systems. Insulin resistance (IR) occurs when organs exhibit reduced insulin sensitivity, leading to difficulties in maintaining glucose homeostasis. IR ensures decades prior to development of overt diabetes and can cause silent metabolic derangements. IR is typically seen very early in the course of chronic kidney disease (CKD) and is evident even when the estimated glomerular filtration rate (eGFR) is within the normal range and IR persists at various stages of kidney disease. In this article, we will discuss insulin handling by the kidneys, mechanisms responsible for IR in CKD, measurements and management of …

Neuromonitoring For Descending Thoracic And Thoracoabdominal Aortic Aneurysm Repair, Akiko Tanaka, Hung Nguyen, Holly N Smith, Anthony L Estrera

Faculty, Staff and Student Publications

No abstract provided.

Current Perioperative Management Of Cerebrospinal Fluid Drains, Shao-Feng Zhou, Akiko Tanaka, Anthony Estrera

Faculty, Staff and Student Publications

No abstract provided.

Current Approaches To Spinal Cord Protection During Open Thoracoabdominal Aortic Aneurysm Repair, Joseph S Coselli, Scott A Lemaire, Vicente Orozco-Sevilla, Ourania Preventza, Marc R Moon, Lauren M Barron, Subhasis Chatterjee

Faculty, Staff and Students Publications

Spinal cord deficit (SCD) is a feared complication after thoracoabdominal aortic aneurysm repair. Vigilant management throughout the perioperative period is necessary to reduce the risk of SCD. Measures for preventing SCD during the intraoperative period include preoperative optimization and recognizing patients at a higher risk of SCD. In this manuscript, we discuss intraoperative adjuncts including utilization of cerebrospinal fluid drainage, left heart bypass, mild hypothermia, selective reimplantation of intercostal and lumbar arteries, and renal and visceral vessel perfusion. From the operative to the postoperative period, careful attention to avoiding hypotension and anemia is important. If SCD is recognized early, therapeutic …

Cell-Based Experimental Evidence To The Anti-Covid-19 Potential Of Ashwagandha And Honeybee Propolis Ingredients, Renu Wadhwa, Durai Sundar, Yoshiyuki Ishida, Keiji Terao, Sunil C. Kaul

Research Symposium

Background: The COVID-19 pandemic emerged in December 2019 by a novel strain of SARS-CoV-2 (Severe Acute Respiratory Syndrome Coronavirus 2) has led to new endeavours in repurposing of existing drugs, anti-COVID-19 vaccine and drug development. Natural products, due to their general safety and wider availability, have attracted research and public attention. In this study, we report anti-COVID potential of compounds from honeybee propolis and Ayurvedic herb, Ashwagandha. Effect of active ingredients was studied on human cell surface receptors (ACE-2:Angiotensin Converting Enzyme-2/Spike protein and TMPRSS2:Transmembrane Protease Serine 2), critical for virus infection and virus main protease (Mpro, essential for virus replication), …

Using Cartesian Coordinate Systems To Create, Classify, And Retrieve Biomedical Time-Series: Applications To 24-Hour Ambulatory Blood Pressure Monitoring, Antonio Garcia, Gladys E. Maestre, John Blangero, Jesus D. Melgarejo, Julio Facelli

Research Symposium

Background: Ambulatory Blood Pressure Measurement (ABPM) allows physicians to monitor blood pressure variability under everyday living conditions and predicts clinical outcomes better than conventional blood-pressure measurement. ABPM can demonstrate mean arterial pressure (MAP) behavior over 24 hours relevant to clinical practice, such as nocturnal hypertension or increased blood pressure variability. We hypothesized that individuals with the same cardiovascular health status would have the same MAP signal (MAPs) waveform.

Methods: This study reutilizes a data subset from the IDACO Consortium to create 24-hour MAPs. We assigned all the MAPs to data matrix X, performed principal components analysis (PCA) to X, and …

Obesity Management In Primary Care Medicine: A Review Of Obesity Perception And Barriers To Weight Loss, Asma Syed, Saima Siddiqui

Research Symposium

Background: This project addresses the obesity epidemic in America. According to the CDC, in 2018, the US obesity prevalence was 42.4%. Obesity is linked to many conditions including heart disease, stroke, type 2 diabetes, and certain types of cancer. Successfully treating obesity can decrease these leading causes of preventable and premature deaths. A proper understanding of the successes and failures of current obesity management in primary care medicine with the help of qualitative research through patient and provider interviews can elucidate the best practices to reduce the burden of disease in America.

Methods: A review of literature was conducted …

An Unusual Case Of Chronic Superior Mesenteric Vein Thrombosis, Ans Albustamy, Eric Sanchez, Oscar Lopez, Schamma Salomon

Research Symposium

Introduction:Intestinal ischemia can be caused by any process that reduces intestinal blood flow. Mesenteric venous thrombosis (MVT) describes thrombosis of the superior or inferior mesenteric vein, and represents approximately 10 percent of all cases of acute mesenteric ischemia. The incidence of MVT has increased over the past 40 years, likely as a result of greater use of abdominal CT, and may present with acute abdominal pain or as an asymptomatic incidental finding on abdominal imaging. We present a rare and unusual case of MVT.

Case presentation: A 42 year old female with decompensated alcoholic liver cirrhosis presented to the …

Euglycemic Diabetic Ketoacidosis With A Decompensated Hypothyroidism In The Setting Of A Recent Cerebrovascular Accident: A Case Report, Jose Gerardo Gomez Casanovas, Alfarooq Alshaikhli, Sergio Saenz, Daniela Hernandez

Research Symposium

Euglycemic diabetic ketoacidosis (EDKA) is an underdiagnosed endocrine emergency. It consists of an increased anion gap metabolic acidosis (pH < 7.3 or serum bicarbonate < 18 mmol/L), moderate ketonuria with a blood glucose levels

The Patient is a 73-year-old female with a past medical history of Diabetes Mellitus, Hypertension, Hyperlipidemia, Hypothyroidism and deafness and muteness since birth. Patient had a recent ischemic stroke 5 days prior to the admission and was admitted at a different hospital for management of her stroke. Patient left against medical advice and presented to our ED later that day complaining of rectal prolapse. Of note, the patient is non-compliant with her medications. Upon admission, she is alert, oriented, and in …

A Stomach Bug? Not What You May Think: A Case Of Post-Viral Gastroparesis Caused By Sars-Cov-2, Adrienne Anderson, Nina Shyama Appareddy, Jose Ramos, Grecia Dominguez Rivera, Robinson Pena, Jose E. Campo Maldonado

Research Symposium

Gastroparesis has a wide range of etiologies including diabetes, medications, post-surgical, post-viral and idiopathic (1). SARS-CoV-2 can cause gastrointestinal symptoms which typically resolve within the first few weeks of infection (2). However, some sequelae persist beyond the initial infectious period.

Measuring Blood Pressure In Primary Care For Accurate Diagnosis And Decision Making: Implementing Evidence-Based Protocols Across A Health System, Gertrude W. Manchester, Deirdre C. Donahue, Catarina I. Kiefe, Lori Pelletier, Marie A. Sosa, Sheri A. Keitz

The Lahey Journal

Abstract

Background: Evidence-based blood pressure (BP) measurement is required for optimal clinical decision-making while improper measurement risks daily diagnostic and therapeutic errors.

Objective: To implement and sustain an evidence-based BP measurement protocol without extra resources and disseminate the protocol across a primary care network.

Design, Setting, and Participants: A Plan-Do-Study-Act (PDSA) framework for quality improvement was used to design a standardized BP measurement protocol including a 5-minute rest period only for patients with persistently elevated blood pressure. The protocol was developed using Goldratt’s Theory of Constraints and Layers of Resistance to ensure multidisciplinary buy-in and was implemented in 3 groups …

Repurposing Normal Chromosomal Microarray Data To Harbor Genetic Insights Into Congenital Heart Disease, Nephi Walton, Hoang Nguyen, Sara Procknow, Darren Johnson, Alexander Anzelmi, Patrick Jay

Department of Medicine Faculty Papers

About 15% of congenital heart disease (CHD) patients have a known pathogenic copy number variant. The majority of their chromosomal microarray (CMA) tests are deemed normal. Diagnostic interpretation typically ignores microdeletions smaller than 100 kb. We hypothesized that unreported microdeletions are enriched for CHD genes. We analyzed "normal" CMAs of 1762 patients who were evaluated at a pediatric referral center, of which 319 (18%) had CHD. Using CMAs from monozygotic twins or replicates from the same individual, we established a size threshold based on probe count for the reproducible detection of small microdeletions. Genes in the microdeletions were sequentially filtered …

Pca-Clf: A Classifier Of Prostate Cancer Patients Into Patients With Indolent And Aggressive Tumors Using Machine Learning, Yashwanth Karthik Kumar Mamidi, Tarun Karthik Kumar Mamidi, Md Wasi Ul Kabir, Jiande Wu, Md Tamjidul Hoque, Chindo Hicks

School of Medicine Faculty Publications

A critical unmet medical need in prostate cancer (PCa) clinical management centers around distinguishing indolent from aggressive tumors. Traditionally, Gleason grading has been utilized for this purpose. However, tumor classification using Gleason Grade 7 is often ambiguous, as the clinical behavior of these tumors follows a variable clinical course. This study aimed to investigate the application of machine learning techniques (ML) to classify patients into indolent and aggressive PCas. We used gene expression data from The Cancer Genome Atlas and compared gene expression levels between indolent and aggressive tumors to identify features for developing and validating a range of ML …

Exploring Conformational Landscapes And Cryptic Binding Pockets In Distinct Functional States Of The Sars-Cov-2 Omicron Ba.1 And Ba.2 Trimers: Mutation-Induced Modulation Of Protein Dynamics And Network-Guided Prediction Of Variant-Specific Allosteric Binding Sites, Gennady M. Verkhivker, Mohammed Alshahrani, Grace Gupta

Mathematics, Physics, and Computer Science Faculty Articles and Research

A significant body of experimental structures of SARS-CoV-2 spike trimers for the BA.1 and BA.2 variants revealed a considerable plasticity of the spike protein and the emergence of druggable binding pockets. Understanding the interplay of conformational dynamics changes induced by the Omicron variants and the identification of cryptic dynamic binding pockets in the S protein is of paramount importance as exploring broad-spectrum antiviral agents to combat the emerging variants is imperative. In the current study, we explore conformational landscapes and characterize the universe of binding pockets in multiple open and closed functional spike states of the BA.1 and BA.2 Omicron …

Adjunctive Diagnostic Studies Completed Following Detection Of Candidemia In Children: Secondary Analysis Of Observed Practice From A Multicenter Cohort Study Conducted By The Pediatric Fungal Network, Rachel L Wattier, Robert F T Bucayu, Craig L K Boge, Rachael K Ross, Inci Yildirim, Theoklis E Zaoutis, Debra L Palazzi, Surabhi B Vora, Elio Castagnola, Martha Avilés-Robles, Lara Danziger-Isakov, Alison C Tribble, Tanvi S Sharma, Antonio C Arrieta, Gabriela Maron, David M Berman, Dwight E Yin, Lillian Sung, Michael Green, Emmanuel Roilides, Kiran Belani, José Romero, Pere Soler-Palacin, Eduardo López-Medina, Dawn Nolt, Ibrahim Zaid Bin Hussain, William J Muller, Sarmistha B Hauger, Natasha Halasa, Daniel Dulek, Alice Pong, Blanca E Gonzalez, Mark J Abzug, Fabianne Carlesse, Anna R Huppler, Sujatha Rajan, Catherine Aftandilian, Monica I Ardura, Arunaloke Chakrabarti, Benjamin Hanisch, Christine M Salvatore, Lena Klingspor, Elizabeth D Knackstedt, Irja Lutsar, Maria E Santolaya, Sydney Shuster, Sarah K Johnson, William J Steinbach, Brian T Fisher

Faculty, Staff and Students Publications

BACKGROUND: Adjunctive diagnostic studies (aDS) are recommended to identify occult dissemination in patients with candidemia. Patterns of evaluation with aDS across pediatric settings are unknown.

METHODS: Candidemia episodes were included in a secondary analysis of a multicenter comparative effectiveness study that prospectively enrolled participants age 120 days to 17 years with invasive candidiasis (predominantly candidemia) from 2014 to 2017. Ophthalmologic examination (OE), abdominal imaging (AbdImg), echocardiogram, neuroimaging, and lumbar puncture (LP) were performed per clinician discretion. Adjunctive diagnostic studies performance and positive results were determined per episode, within 30 days from candidemia onset. Associations of aDS performance with episode characteristics …

Differential Effects Of Pancreatic Cancer-Derived Extracellular Vesicles Driving A Suppressive Environment, Anurag Purushothaman, Jacqueline Oliva-Ramírez, Warapen Treekitkarnmongkol, Deivendran Sankaran, Mark W Hurd, Nagireddy Putluri, Anirban Maitra, Cara Haymaker, Subrata Sen

Faculty, Staff and Student Publications

Pancreatic ductal adenocarcinoma (PDAC) cells display extensive crosstalk with their surrounding environment to regulate tumor growth, immune evasion, and metastasis. Recent advances have attributed many of these interactions to intercellular communication mediated by small extracellular vesicles (sEVs), involving cancer-associated fibroblasts (CAF). To explore the impact of sEVs on monocyte lineage transition as well as the expression of checkpoint receptors and activation markers, peripheral blood monocytes from healthy subjects were exposed to PDAC-derived sEVs. Additionally, to analyze the role of sEV-associated HA in immune regulation and tissue-resident fibroblasts, monocytes and pancreatic stellate cells were cultured in the presence of PDAC sEVs …

Pet Imaging Of Microglia Using Pbr28suv Determines Therapeutic Efficacy Of Autologous Bone Marrow Mononuclear Cells Therapy In Traumatic Brain Injury, Supinder S Bedi, Michael C Scott, Max A Skibber, Akshita Kumar, Henry W Caplan, Hasen Xue, David Sequeira, Alison L Speer, Fanni Cardenas, Franciska Gudenkauf, Karen Uray, Amit K Srivastava, Alan R Prossin, Charles S Cox

Faculty, Staff and Student Publications

Traumatic brain injury (TBI) results in activated microglia. Activated microglia can be measured in vivo by using positron emission topography (PET) ligand peripheral benzodiazepine receptor standardized uptake values (PBR28suv). Cell based therapies have utilized autologous bone marrow mononuclear cells (BMMNCs) to attenuate activated microglia after TBI. This study aims to utilize in vivo PBR28suv to assess the efficacy of BMMNCs therapy after TBI. Seventy-two hours after CCI injury, BMMNCs were harvested from the tibia and injected via tail-vein at 74 h after injury at a concentration of 2 million cells per kilogram of body weight. There were three groups of …

An Ecchordosis Physaliphora, A Rare Entity, Involving The Central Nervous System: A Systematic Review Of The Literature, Rajesh K Gupta, Thejasvi A Reddy, Ashutosh Gupta, Rohan Samant, Carlos A Perez, Anam Haque

Faculty, Staff and Student Publications

Ecchordosis physaliphora (EP) is a benign notochordal remnant, which is often an incidental finding; however, it can rarely present with neurological symptoms. We performed a systematic review of the literature for cases of symptomatic EP published in PubMed, Web of Science and Embase from January 1982 to May 2023. This is the largest review to date and revealed 60 cases including ours. Headache (55%) and CSF rhinorrhea (32%) were the most frequent clinical manifestations. The majority of symptomatic EP lesions were located in the prepontine region (77%) and required surgical resection (75%). EP should be considered in patients with neurologic …

Inhibition Of Trap1 Accelerates The Dna Damage Response, Activation Of The Heat Shock Response And Metabolic Reprogramming In Colon Cancer Cells, Nobel Bhasin, Prerna Dabral, Lakmini Senavirathna, Sheng Pan, Ru Chen

Faculty, Staff and Students Publications

BACKGROUND: Colorectal cancer (CRC) is one of the major causes of cancer-related mortality worldwide. The tumor microenvironment plays a significant role in CRC development, progression and metastasis. Oxidative stress in the colon is a major etiological factor impacting tumor progression. Tumor necrosis factor receptor-associated protein 1 (TRAP1) is a mitochondrial member of the heat shock protein 90 (HSP90) family that is involved in modulating apoptosis in colon cancer cells under oxidative stress. We undertook this study to provide mechanistic insight into the role of TRAP1 under oxidative stress in colon cells.

METHODS: We first assessed the The Cancer Genome Atlas …

Setd2 Safeguards The Genome Against Isochromosome Formation, Frank M Mason, Emily S Kounlavong, Anteneh T Tebeje, Rashmi Dahiya, Tiffany Guess, Abid Khan, Logan Vlach, Stephen R Norris, Courtney A Lovejoy, Ruhee Dere, Brian D Strahl, Ryoma Ohi, Peter Ly, Cheryl Lyn Walker, W Kimryn Rathmell

Faculty, Staff and Students Publications

Isochromosomes are mirror-imaged chromosomes with simultaneous duplication and deletion of genetic material which may contain two centromeres to create isodicentric chromosomes. Although isochromosomes commonly occur in cancer and developmental disorders and promote genome instability, mechanisms that prevent isochromosomes are not well understood. We show here that the tumor suppressor and methyltransferase SETD2 is essential to prevent these errors. Using cellular and cytogenetic approaches, we demonstrate that loss of SETD2 or its epigenetic mark, histone H3 lysine 36 trimethylation (H3K36me3), results in the formation of isochromosomes as well as isodicentric and acentric chromosomes. These defects arise during DNA replication and are …

Tailoring The Gut Microbiome Through Diet To Target Short Chain Fatty Acids And Trimethylamine N-Oxide For Heart Failure Prevention, Julia M. Gabel

PANDION: The Osprey Journal of Research and Ideas

The number of people diagnosed with heart failure is on the rise—it’s becoming an epidemic. There are treatment options available for heart failure, but unfortunately there is not a cure. However, most cases of heart failure can be prevented. One mechanism that provides promise for preventing heart failure is the implementation of a heart healthy diet. However, not in the context that most are familiar with. In this case, a heart healthy diet is in reference to the ability of diet to tailor the gut microbiome in order to target specific metabolites that are associated with heart failure. Diet plays …

Dispensing With Reductionism And Dualism: Biological Anthropological Perspectives Towards Understanding Disease, Epidemics, And Pandemics, Anne Grauer

Anthropology: Faculty Publications and Other Works

At no time in recent history has the topic of disease, epidemics and pandemics been more at the forefront of public conversation than it is now. Previously, the threat of epidemics such as Ebola, the 1918 influenza epidemic, and the plague, were commonly framed as “back then” or “over there.” Perhaps COVID ended the public's delusion. For biological anthropologists, however, the perspective has differed appreciably. Pandemics and epidemics are viewed as integral and repeated aspects of human existence, and the kindling to start an outbreak of gargantuan proportion has been delicately stacked for centuries (if not millennia). If the public …

Novel Genetic And Phenotypic Expansion In Gosr2-Related Progressive Myoclonus Epilepsy, Lea Hentrich, Mered Parnes, Timothy Edward Lotze, Rohini Coorg, Tom J De Koning, Kha M Nguyen, Calvin K Yip, Heinz Jungbluth, Anne Koy, Hormos Salimi Dafsari

Faculty, Staff and Students Publications

Biallelic variants in the Golgi SNAP receptor complex member 2 gene (GOSR2) have been reported in progressive myoclonus epilepsy with neurodegeneration. Typical clinical features include ataxia and areflexia during early childhood, followed by seizures, scoliosis, dysarthria, and myoclonus. Here, we report two novel patients from unrelated families with a GOSR2-related disorder and novel genetic and clinical findings. The first patient, a male compound heterozygous for the GOSR2 splice site variant c.336+1G>A and the novel c.364G>A,p.Glu122Lys missense variant showed global developmental delay and seizures at the age of 2 years, followed by myoclonus at the age …

Blockade Of Interferon Signaling Decreases Gut Barrier Integrity And Promotes Severe West Nile Virus Disease, Shih-Ching Lin, Fang R Zhao, Hana Janova, Adrian Gervais, Summer Rucknagel, Kristy O Murray, Jean-Laurent Casanova, Michael S Diamond

Faculty, Staff and Students Publications

The determinants of severe disease caused by West Nile virus (WNV) and why only ~1% of individuals progress to encephalitis remain poorly understood. Here, we use human and mouse enteroids, and a mouse model of pathogenesis, to explore the capacity of WNV to directly infect gastrointestinal (GI) tract cells and contribute to disease severity. At baseline, WNV poorly infects human and mouse enteroid cultures and enterocytes in mice. However, when STAT1 or type I interferon (IFN) responses are absent, GI tract cells become infected, and this is associated with augmented GI tract and blood-brain barrier (BBB) permeability, accumulation of gut-derived …

A Rare Cause Of Diarrhea: Intestinal Spirochetosis In An Hiv-Positive Patient., Lacey Falgout, Shilpa Kailas, Supraja Sridhar, Nisha Loganantharaj

School of Medicine Faculty Publications

Intestinal spirochetosis (IS) is a rare gastrointestinal infection with vague presenting symptoms. Diagnosis is confirmed histopathologically. Risk factors include homosexuality and HIV. Antibiotic treatment with metronidazole usually leads to resolution of symptoms. We present the case of a 56-year-old HIV-positive man with chronic, watery diarrhea who was diagnosed with IS. This case highlights the importance of considering IS in the differential in HIV-positive patients with nonspecific gastrointestinal symptoms after more common etiologies have been ruled out.

Protein S Antibody As An Adjunct Therapy For Hemophilia B, Hope P. Wilson, Aliyah Pierre, Ashley L. Paysse, Narender Kumar, Brian C. Cooley, Pratyadipta Rudra, Adrianne W. Dorsey, Diana Polania-Villanueva, Sabyasachi Chatterjee, Maissaa Janbain, Maria C. Velez, Rinku Majumder

School of Medicine Faculty Publications

ABSTRACT: Hemophilia B (HB) is caused by an inherited deficiency of plasma coagulation factor IX (FIX). Approximately 60% of pediatric patients with HB possess a severe form of FIX deficiency (< 1% FIX activity). Treatment typically requires replacement therapy through the administration of FIX. However, exogenous FIX has a limited functional half-life, and the natural anticoagulant protein S (PS) inhibits activated FIX (FIXa). PS ultimately limits thrombin formation, which limits plasma coagulation. This regulation of FIXa activity by PS led us to test whether inhibiting PS would extend the functional half-life of FIX and thereby prolong FIX-based HB therapy. We assayed clotting times and thrombin generation to measure the efficacy of a PS antibody for increasing FIX activity in commercially obtained plasma and plasma from pediatric patients with HB. We included 11 pediatric patients who lacked additional comorbidities and coagulopathies. In vivo, we assessed thrombus formation in HB mice in the presence of the FIXa ± PS antibody. We found an accelerated rate of clotting in the presence of PS antibody. Similarly, the peak thrombin formed was significantly greater in the presence of the PS antibody, even in plasma from patients with severe HB. Furthermore, HB mice injected with PS antibody and FIX had a 4.5-fold higher accumulation of fibrin at the thrombus induction site compared with mice injected with FIX alone. Our findings imply that a PS antibody would be a valuable adjunct to increase the effectiveness of FIX replacement therapy in pediatric patients who have mild, moderate, and severe HB.