Rheumatology Commons^™

Associations Of Galectin-3 Levels With Measures Of Vascular Disease In Patients With Rheumatoid Arthritis, Amanda Nussdorf, Elizabeth Park, Isabelle Amigues, Laura Geraldino-Pardilla, Sabahat Bokhari, Jon T. Giles, Joan M. Bathon

Division of Rheumatology Faculty Papers

OBJECTIVES: Galectin-3 is a beta-galactoside-binding lectin and is a marker of cardiovascular disease (CVD) in the general population. It may also play a role in joint inflammation. We asked whether serum galectin-3 is a useful marker of subclinical vascular disease in patients with rheumatoid arthritis (RA).

METHODS: RA patients without clinical CVD underwent assessment of coronary artery calcium (CAC) score, aortic inflammation (using 18Fluorodeoxyglucose positron emission-computed tomography [FDG PET/CT]), and myocardial flow reserve (MFR). Aorta FDG uptake was measured as standardized uptake values (SUV). Generalized linear models were constructed to explore the associations of galectin-3 levels with CAC score, aortic …

Case Report And Literature Review Of An Atypical Polymyalgia Rheumatica And Its Management, Saad Bilal Ahmed, Saara Ahmad, Hanmei Pan

Department of Biological & Biomedical Sciences

Polymyalgia rheumatica (PMR) is a systemic inflammatory disease of the elderly population that increases in incidence as age advances. It is characterised by the sudden or sub-acute onset of symptoms affecting the shoulder and pelvic girdles, often accompanied by constitutional symptoms. Due to the lack of consensual diagnostic criteria and specific laboratory or radiological investigations for PMR, its diagnosis can be very challenging, particularly because it can be mimicked or masked by other geriatric syndromes. PMR responds well to glucocorticoid treatment, but if left untreated, can lead to morbidity and poor quality of life. We present the case of an …

Barriers To The Diagnosis, Care, And Management Of Pediatric Patients With Ehlers-Danlos Syndrome In The United States: A Qualitative Analysis., William R. Black, Lora L. Black, Jordan T. Jones

Manuscripts, Articles, Book Chapters and Other Papers

Objectives: Ehlers-Danlos Syndromes (EDS) are a family of heritable connective tissue diseases. Primary practitioners are capable of diagnosing and managing EDS; however, few are knowledgeable and comfortable enough to see patients with EDS, resulting in delays in diagnosis and care. This study explores the barriers physicians experience with diagnosing, managing, and caring for patients with EDS, and potential resolutions to those barriers. Methods: As part of a larger online study, providers (n = 107) in the United States were asked to specify "What information would improve (their) comfort" in diagnosing, caring for, and managing EDS via open-ended questions. Results …

Pericardial Effusions And Cardiac Tamponade In Hospitalized Systemic Sclerosis Patients: Analysis Of The National Inpatient Sample, Bikash Basyal, Waqas Ullah, Chris T. Derk

Abington Jefferson Health Papers

Introduction

Clinically significant pericardial effusions and cardiac tamponade in systemic sclerosis (SSc) patients is uncommon and the factors that contribute to progression of pericardial involvement in SSc patients have not been well established.

Methods

A review of the national inpatient sample database was performed looking SSc related hospitalizations between 2002 and 2019. Data was collected on patients with pericardial effusions and cardiac tamponade and analyzed to identify and describe patient characteristics and comorbidities.

Results

Out of a total of 523,410 SSc hospitalizations, with an overall inpatient mortality rate of 4.7% (24,764 patients), pericardial effusion was identified in 3.1% of all …

Antibodies For Β2-Microglobulin And The Heavy Chains Of Hla-E, Hla-F, And Hla-G Reflect The Hla-Variants On Activated Immune Cells And Phases Of Disease Progression In Rheumatoid Arthritis Patients Under Treatment, Mepur H. Ravindranath, Narendranath M. Ravindranath, Carly J. Amato-Menker, Fatiha El Hilali, Senthamil R. Selvan, Edward J Filippone, Luis Eduardo Morales-Buenrostro

Department of Medicine Faculty Papers

Rheumatoid arthritis (RA) is a progressive, inflammatory, autoimmune, symmetrical polyarticular arthritis. It is characterized by synovial infiltration and activation of several types of immune cells, culminating in their apoptosis and antibody generation against "altered" autoantigens. β2-microglobulin (β2m)-associated heavy chains (HCs) of HLA antigens, also known as closed conformers (Face-1), undergo "alteration" during activation of immune cells, resulting in β2m-free structural variants, including monomeric open conformers (Face-2) that are capable of dimerizing as either homodimers (Face-3) or as heterodimers (Face-4). β2m-free HCs uncover the cryptic epitopes that can elicit antibodies (Abs). We report here the levels of IgM and IgG Abs …

It Is Not Pneumocystis Jiroveci (Pcp), It Is Cyclophosphamide-Induced Pneumonitis, Mery Bartl, Yilen Karen Ng-Wong, Blesset Alexander, Jose Gomez Casanovas, Josenny L. Rodriguez-Paez, Andres Suarez, Christine Loftis

School of Medicine Publications and Presentations

Cyclophosphamide (CYC) is an immunosuppressive medication used to treat life-threatening complications of various rheumatic diseases like vasculitis and systemic lupus erythematosus. A rare side effect of this medication is pneumonitis, which occurs in less than 1% of patients. We describe a case of an 83-year-old woman with a past medical history of microscopic polyangiitis, who presented with progressive dyspnea at rest, exacerbated on exertion, and associated with orthopnea that was attributed to CYC-induced pneumonitis. Three months before this presentation, the patient was diagnosed with antineutrophil cytoplasmic antibodies (ANCA)-positive pauci-immune crescentic and necrotizing glomerulonephritis and started on CYC. On admission, a …

Rapid Onset Hydralazine-Induced Antineutrophil Cytoplasmic Antibody (Anca) Associated Vasculitis Presenting With Hemoptysis And Kidney Failure: Case Report, Rachel E. Easter, Andinet Gizaw, Natalie Klein

Internal Medicine Publications

Hydralazine-induced vasculitis can be challenging to recognize and diagnose as presenting symptoms vary and can mimic other conditions or diseases, however, swift intervention and treatment is key in halting progression of the disease and providing patients with the best possible outcomes. A 71-year-old African American female presented to the emergency department with weakness, fatigue, anemia, blood-streaked sputum, fever, chills, and severe myalgias. Hydralazine was prescribed to treat resistant hypertension eight months prior to presentation. Hydralazine was discontinued on hospital day (HD) 4. Pertinent laboratory values showed elevated inflammatory markers, positive antinuclear antibody (ANA), negative rheumatoid factor (RF), normal complement levels, …

Dermatologic Manifestations Of Rheumatic Disease: Cutaneous Manifestations Of Vasculitides, Julianna S. Kang, Julia Nunley, Tiffany Ho, Mavra Masood, Fnu Nutan, Beth Rubinstein

Open and Affordable Course Content at VCU

Cutaneous Manifestations of Vasculitides is a Powerpoint based learning module that begins with the definition of terms and findings often found in vasculitis, followed by a review of several systemic vasculitides, highlighting how characteristic cutaneous lesions may be predictive of the vessel of involvement as well as the associated histologic changes. Moreover, significant clinical and laboratory findings utilized to make the correct diagnosis will be emphasized. This module is not meant to be an exhaustive review of vasculitis, but to serve as an introduction to understanding disease processes and how systemic vasculitis and skin findings interrelate.

It is part of …

Dermatologic Manifestations Of Rheumatic Disease: Malignant Cutaneous Tumors, Divya Shan, Julianna S. Kang, Allison Cinats, Julia R. Nunley, Beth Rubinstein

Open and Affordable Course Content at VCU

Malignant Cutaneous Tumors is a Powerpoint-based learning module that focuses on skin cancers. It begins with the definition of terms and findings, followed by a review of several clinical cases. The goal of the module is to provide the students with the ability to differentiate the most common types of skin cancer and precancerous lesions and gain an awareness of cutaneous lymphomas. This module will include images and clinical findings from diverse skin tones to prepare students to identify cutaneous findings in a heterogeneous patient population. This module is not a comprehensive review of skin cancers but an introduction to …

An Iceberg I Can’T Handle: A Qualitative Inquiry On Perceptions Towards Paediatric Rheumatology Among Healthcare Workers In Kenya, Angela Migowa, Sasha Bernatsky, Anthony Ngugi, Helen E. Foster, Peter Muriuki, Adelaide Lusambili, Stanley Luchters

Paediatrics and Child Health, East Africa

Background: Delay in diagnosis and access to specialist care is a major problem for many children and young people with rheumatic disease in sub-Saharan Africa. Most children with symptoms of rheumatic disease present to nonspecialists for care. There is an urgent need to understand and scale-up paediatric rheumatology knowledge and skills amongst non-specialist healthcare workers to promote early diagnosis, prompt referral, and management.

Purpose: We evaluated the knowledge, attitudes and practices towards diagnosis and care of paediatric rheumatology patients among health care workers in Kenya.

Methods: We conducted 12 focus group discussions with clinical officers (third-tier community health …

Gender Dysphoria In Adolescents With Ehlers-Danlos Syndrome., Jordan T. Jones, William R. Black, Christine Moser, Eric T. Rush, Lindsey Malloy-Walton

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: Ehlers-Danlos Syndrome represents a family of heritable connective tissue disorders that include joint hypermobility, tissue fragility, and skin hyperextensibility. Ehlers-Danlos Syndrome presents with clinical sequela across multiple body systems that require multidisciplinary care. Little is known about adolescents with Ehlers-Danlos Syndrome who are transgender and gender diverse. To date, there have been no reports of transgender and gender diverse youth in pediatric patients with Ehlers-Danlos Syndrome. The objective of this study was to characterize transgender and gender diverse adolescents with Ehlers-Danlos Syndrome seen in a pediatric multidisciplinary specialty clinic.

METHODS: A retrospective chart review was performed and it was …

Treatment Of Severe Swallowing Dysfunction In Systemic Sclerosis With Ivig: Role Of Antimuscarinic Antibodies, Fabian A Mendoza, Anthony J. Dimarino, Sidney Cohen, Christopher Adkins, Shady Abdelbaki, Satish Rattan, Christopher Cao, Susie Denuna-Rivera, Sergio A. Jimenez

Division of Gastroenterology and Hepatology Faculty Papers

Oropharyngeal and esophageal dysmotility can cause serious clinical complications such as aspiration pneumonia, cachexia, and sarcopenia, with a resulting increase in mortality and disability. The current standard of care for the treatment of SSc-associated swallowing dysfunction is mainly supportive, although severe cases are usually refractory to conventional management. Recent studies have shown that the abnormal production of functional autoantibodies such as anti-cholinergic muscarinic receptor III antibodies may participate in the pathogenesis of SSc-associated gastrointestinal dysmotility and may provide a novel target for therapeutic intervention. We describe two patients with severe and rapid onset of SSc-associated severe swallowing dysfunction and esophageal …

Role Of Lipid-Lowering Therapy In Peripheral Artery Disease, Agastya Deepak Belur, Aangi J. Shah, Salim S. Virani, Mounica Vorla, Dinesh K. Kalra

Office of the Provost

Atherosclerosis is a multifactorial, lipoprotein-driven condition that leads to plaque formation within the arterial tree, leading to subsequent arterial stenosis and thrombosis that accounts for a large burden of cardiovascular morbidity and mortality globally. Atherosclerosis of the lower extremities is called peripheral artery disease and is a major cause of loss in mobility, amputation, and critical limb ischemia. Peripheral artery disease is a common condition with a gamut of clinical manifestations that affects an estimated 10 million people in the United States of America and 200 million people worldwide. The role of apolipoprotein B-containing lipoproteins, such as LDL and remnant …

Urate-Lowering Therapy For Patients With Gout On Hemodialysis, Reem Alkilany, Douglas Einstadter, Maria Antonelli

Faculty Scholarship

Objective: Gout is the most common form of inflammatory arthritis and is caused by deposition of monosodium urate crystals resulting from a high burden of uric acid (UA). High UA burden also has been associated with increased morbidity and mortality in the general population and progression to chronic kidney disease. In persons with gout and end-stage renal disease (ESRD), prior studies suggest that UA levels decrease after initiation of hemodialysis (HD). We evaluated UA level and the use of urate-lowering therapies (ULTs) in patients with gout and ESRD on HD. Methods: We performed a retrospective review of patients with gout …

A Rare Case Of Segmental Arterial Mediolysis, Ashbina Pokharel, Lauren Reinhold, Ashbita Pokharel, Judith Bateman

Conference Presentation Abstracts

Segmental arterial mediolysis (SAM) is a rare vasculopathy of unknown etiology characterized by disruption of the arterial layer, which can result in vessel dissection, hemorrhage and ischemia. Literature review has demonstrated a varied presentation ranging from benign abdominal pain to life threatening hemorrhage. We report a case of 58 year old female with past medical history of deep vein thrombosis/pulmonary embolism secondary to Protein C and S deficiency on Coumadin who presented with right flank pain for 3 days. Complete blood count and complete metabolic panel were within normal limits. CT scan of abdomen and MRI showed ischemia of segment …

Anakinra Treatment Efficacy In Reduction Of Inflammatory Biomarkers In Covid-19 Patients: A Meta-Analysis, Zunaira Naveed, Musharraf Sarwar, Zahid Ali, Danish Saeed, Khadija Choudhry, Azza Sarfraz, Zouina Sarfraz, Miguel Felix, Ivan Cherrez-Ojeda

Department of Paediatrics and Child Health

Introduction: Anakinra is being empirically considered for the treatment of COVID-19 patients. The aim is to assess the efficacy of anakinra treatment on inflammatory marker reduction, including c-reactive protein (CRP) concentrations, serum ferritin, and serum d-dimer levels.
Methods: Adhering to PRISMA 2020 statement guidelines, a systematic search was conducted across the following databases from December 2019 until January 10, 2022: PubMed/MEDLINE, Cochrane Central, Web of Science, Scopus, and EMBASE. The following keywords were employed: Anakinra, COVID*, SARS-CoV-2, inflammatory, CRP, D-dimer, Ferritin, hematological, laboratory, clinical, trials. The findings were collated and presented in a tabulated manner, and statistically analyzed using Review …

Telemedicine For Rheumatology Patients In Covid-19 Pandemic: Perspectives Of Patients, Muhammad Mehmood Riaz, Saad Bin Zafar Mahmood, Noreen Nasir

Section of Internal Medicine

Telemedicine use for patients with autoimmune rheumatic conditions during the pandemic mandates better characterization. We conducted a telephonic survey on patients who consulted rheumatologists remotely to determine their attitudes toward telemedicine. The study was conducted at Aga Khan University Hospital, a tertiary care hospital in Pakistan which is a lower-middle-income country. Descriptive analyses were performed. Fifty patients visited tele-rheumatology clinic, 35 (70.0%) were female with a mean (SD) age of 47.6 (18.1) years. Majority (27, 54.0%) learned about telemedicine from hospital website and outpatient clinic desk or helpline. Lack of examination was the biggest concern with teleconsultation (18, 22.2%). Most …

Down Syndrome-Associated Arthritis (Da): Diagnostic And Management Challenges., Jordan T. Jones, Jacqueline Kitchen, Nasreen Talib

Manuscripts, Articles, Book Chapters and Other Papers

Down syndrome (DS) is one of the most common birth defects in the United States, the most common genomic disorder of intellectual disability, and results from trisomy 21. This chromosome disorder causes an extensive, heterogenous phenotype that results in a broad presentation of symptoms that includes atlantoaxial instability, congenital heart defects, muscle hypotonia, hypothyroidism, hematologic disorders, recurrent infections, and autoimmune diseases. The autoimmune diseases are caused by immune system dysregulation that results in increased pro-inflammatory cytokines, along with other innate and adaptive immune system dysregulation. This is the likely cause of the increased risk of inflammatory arthritis or Down syndrome-associated …

Dermatologic Manifestations Of Rheumatic Disease: Cutaneous Manifestations Of Sarcoidosis, Sarah Shapiro, Sindhuja Koppu, Mavra Masood, Beth Rubinstein, Huzaefah Syed

Open and Affordable Course Content at VCU

Cutaneous Manifestations of Sarcoidosis is a Powerpoint based learning module that depicts fictional patient scenarios detailing sarcoidosis and erythema nodosum. It explores other cutaneous and non-cutaneous manifestations, diagnostic criteria, and medical evaluation of sarcoidosis and the possible etiologies, workup, treatment, and prognosis of erythema nodosum. Health and racial disparities experienced by patients with sarcoidosis are also highlighted and discussed. It takes approximately 25 minutes to complete. This module was created for use in the VCU School of Medicine M1 Movement block, Rheumatology and Dermatology sections.

It is part of the Dermatologic Manifestations of Rheumatic Disease project, which provides overviews of …

The Efficacy Of Sequential Biologic Agents In Refractory Rheumatoid Arthritis After Failure Of Initial Dmard And Anti-Tumor Necrosis Factor Therapy, Antonio Giovanni Versace, Caterina Oriana Aragona, Daniela La Rosa, Marianna Chiappalone, Maria Concetta Tringali, Alberta De Gaetano, Charles Frederick Moore Jr., Donatella Sangari, William Neal Roberts, Gianluca Bagnato

Internal Medicine Faculty Publications

Introduction/Objective: The efficacy of biologic therapy in the treatment of rheumatoid arthritis (RA) has been well-established but, in practice, a quarter of patients will either not respond to the first biologic agent or will suffer an adverse event requiring a switch to a different drug. While clinical guidelines exist to help guide therapy and previous studies have examined sequential use of anti-TNF agents, there is little data to inform a multiple switch strategy. Our aim was to measure the efficacy of multiple switches of biologic in severe refractory RA. Methods: We enrolled 111 patients whose therapy with one anti-TNF agent …

Acquired Factor Viii Deficiency Presenting As Gross Hematuria In A Hispanic, Pregnant Patient With Previously Undiagnosed Connective Tissue Disease, Christine Loftis, Emilia C. Dulgheru, Rosa White

School of Medicine Publications and Presentations

Acquired factor VIII deficiency is a bleeding disorder caused by the presence of autoantibodies against clotting factor VIII. We report a case of a 24-year-old pregnant woman who presented with gross hematuria secondary to acquired factor VIII deficiency in the presence of a previously undiagnosed connective tissue disease. *is article includes a literature review of pregnancy-related cases of acquired factor VIII deficiency. We also reviewed various therapeutic approaches for the management of the acquired factor inhibitor which include achieving hemostasis and elimination of the inhibitor via immunosuppressive agents. *is case report describes the rare presentation of acquired factor VIII deficiency …

Dermatomyositis- Related Intestinal Dysmotility, Christine Loftis, Rosa White, Emilia C. Dulgheru

School of Medicine Publications and Presentations

Dermatomyositis (DM) is an inflammatory myopathy (IIM) characterized by proximal muscle weakness and pathognomonic skin lesions. A 69-year-old woman with a recent diagnosis of DM 1 month prior, treated with corticosteroids and immunomodulators, presented to our inpatient rehabilitation with worsening dysphagia and constipation. At the time of our evaluation, physical examination was notable for erythematous papules over the metacarpophalangeal joints, proximal interphalangeal joints, elbows, and knees as well as a violaceous rash on the face. Muscle strength was diminished bilaterally with proximal distribution being affected greater than distal. Laboratory studies were notable for the creatine kinase (CK) level of 31 …

Short Term Outcomes Of Covid-19 In Lupus: Propensity Score Matched Analysis From A Nationwide Multi-Centric Research Network, Rahul Raiker, Haig Pakhchanian, Charles Deyoung, Latika Gupta, Sinan Kardes, Sakir Ahmed, Chengappa Kavadichanda

Student Papers, Posters & Projects

Objectives

To determine the severity and outcome of COVID-19 among individuals with lupus as compared to controls. The secondary objective was to identify the risk association of sex, race, presence of nephritis, and use of various immunomodulators with COVID-19 outcomes.

Methods

Retrospective data of individuals with lupus with and without COVID-19 between January 2020 to May 2021 was retrieved from the TriNetX. A one-to-one matched COVID-19 positive control was selected using propensity score(PS) matching. We assessed several outcomes, including all-cause mortality, hospitalisation, intensive care unit (ICU) admission, mechanical ventilation, severe COVID, acute kidney injury (AKI), Haemodialysis, acute respiratory distress syndrome …

Knowledge About Juvenile Idiopathic Arthritis-Associated Uveitis: More Frequent Reminders Are Associated With Higher Patient And Family Uveitis Knowledge., Ashley M. Cooper, Elaine R. Flanagan, Tova Ronis, Baruch Goldberg, Ashley K. Sherman, Chelsey Smith, Gary N. Holland, Carra Uveitis Workgroup

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Chronic anterior uveitis is a sight-threatening complication of juvenile idiopathic arthritis (JIA) and a primary contributor to long-term morbidity in people with JIA. Levels of knowledge about uveitis among JIA patients and their parents are unknown. A survey of JIA patients and parents was conducted to assess knowledge about uveitis complications and recommended screening.

METHODS: A survey was developed consisting of six demographic questions, six arthritis/uveitis history questions, and nine uveitis knowledge questions. The survey was administered to JIA patients age 14 and older and parents of patients with JIA at three pediatric rheumatology practices and online through the …

The Covid-19 Assessment For Survival At Admission (Casa) Index: A 12 Months Observational Study, Gianluca Bagnato, Daniela La Rosa, Carmelo Ioppolo, Alberta De Gaetano, Marianna Chiappalone, Natalia Zirilli, Valeria Viapiana, Maria Concetta Tringali, Simona Tomeo, Caterina Oriana Aragona, Francesca Napoli, Sara Lillo, Natasha Irrera, William Neal Roberts, Egidio Imbalzano, Antonio Micari, Elvira Ventura Spagnolo, Giovanni Squadrito, Sebastiano Gangemi, Antonio Giovanni Versace

Internal Medicine Faculty Publications

Objective: Coronavirus disease 2019 (COVID-19) is a disease with a high rate of progression to critical illness. However, the stratification of patients at risk of mortality is not well defined. In this study, we aimed to define a mortality risk index to allocate patients to the appropriate intensity of care.

Methods: This is a 12 months observational longitudinal study designed to develop and validate a pragmatic mortality risk score to stratify COVID-19 patients aged ≥18 years and admitted to hospital between March 2020 and March 2021. Main outcome was in-hospital mortality.

Results: 244 patients were included in the study (mortality …

Brief Musculoskeletal Screen And Patient Education For Down Syndrome-Associated Arthritis., Jordan T. Jones, Chelsey Smith, Nasreen Talib

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Identifying The Content And Context Of Pain Within Paediatric Rheumatology Healthcare Professional Curricula In The Uk: A Summative Content Analysis., Rebecca Rachael Lee, Janet E. Mcdonagh, Mark Connelly, Sarah Peters, Lis Cordingley

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The curriculum for professionals working in paediatric rheumatology should include pain but it is unclear to what extent this currently occurs. The aim of this study was to identify pain-related curriculum content and the context in which pain is presented in educational and training documentation for healthcare professionals in this clinical speciality.

METHODS: Core curricula documents from UK based professional organisations were identified in partnership with healthcare professionals. Documents were analysed using a summative content analysis approach. Key pain terms were quantified and weighted frequencies were used to explore narrative pain themes. Latent content was interpreted qualitatively to explore …

Outcomes Of Covid-19 In Patients With Rheumatoid Arthritis: A Multicenter Research Network Study In The United States., Rahul Raiker, Charles Deyoung, Haig Pakhchanian, Sakir Ahmed, Chengappa Kavadichanda, Latika Gupta, Sinan Kardeş

Student Papers, Posters & Projects

OBJECTIVES: To investigate outcomes of Coronavirus Disease-2019 (COVID-19) in patients with rheumatoid arthritis (RA) as compared to the general population. Additionally, outcomes were explored among RA patients stratified by sex, race, and medications use through sub-cohort analyses.

METHODS: This comparative cohort study used a US multicenter research network (TriNetX) to extract data on all adult RA patients who were diagnosed with COVID-19, and adults without RA who were diagnosed with COVID-19 (comparative cohort) anytime from January 20, 2020 to April 11, 2021. COVID-19 outcomes were assessed within 30 days after its diagnosis. Baseline characteristics that included demographics and comorbidities were …

Atypical Presentation Of Systemic Arthritis In A Toddler With Down Syndrome., Emily Worley, Weijie Li, Jordan T. Jones

Manuscripts, Articles, Book Chapters and Other Papers

Systemic juvenile idiopathic arthritis (sJIA) is a chronic, inflammatory disease of childhood, which is characterized by the combination of arthritis, serositis, daily, high-spiking fevers, and evanescent macular rash and can present with the life-threatening complication of macrophage activation syndrome (MAS). Children with Down syndrome (DS) have complex medical challenges related to abnormalities in their immune system, which can cause a broad spectrum of disease manifestations, which can occur atypically. Children with DS are at increased risk for arthritis and interstitial lung disease (ILD) associated with sJIA that has high mortality. This case report outlines an atypical presentation of sJIA in …

Detection Of Subclinical Rheumatic Heart Disease In Children Using A Deep Learning Algorithm On Digital Stethoscope: A Study Protocol, Fatima Ali, Babar Hasan, Huzaifa Ahmad, Zahra Hoodbhoy, Zainab Bhuriwala, Muhammad Hanif, Shahab U. Ansari, Devyani Chowdhury

Department of Paediatrics and Child Health

Introduction: Rheumatic heart diseases (RHDs) contribute significant morbidity and mortality globally. To reduce the burden of RHD, timely initiation of secondary prophylaxis is important. The objectives of this study are to determine the frequency of subclinical RHD and to train a deep learning (DL) algorithm using waveform data from the digital auscultatory stethoscope (DAS) in predicting subclinical RHD.
Methods and analysis: We aim to recruit 1700 children from a group of schools serving the underprivileged over a 12-month period in Karachi (Pakistan). All consenting students within the age of 5-15 years with no underlying congenital heart disease will be eligible …