Digital Commons Network^™

Diagnostic Journey And Impact Of Enzyme Replacement Therapy For Mucopolysaccharidosis Iva: A Sibling Control Study., Can Ficicioglu, Dena R Matalon, Nicole Luongo, Caitlin Menello, Tracy Kornafel, Andrew J Degnan

Abington Jefferson Health Papers

BACKGROUND: Mucopolysaccharidosis (MPS) IVA, also known as Morquio A syndrome, is a rare autosomal recessive lysosomal storage disorder caused by a deficiency in the enzyme N-acetylgalactosamine-6-sulfatase. Early recognition, diagnosis, and treatment of this progressive, multisystem disease by enzyme replacement therapy (ERT) can lead to improved outcomes and reduced mortality.

METHODS: This report documents the diagnostic journey and treatment with ERT of three siblings with MPS IVA. Clinical outcome measures included growth, endurance, imaging, cardiac, respiratory, ophthalmology, and laboratory evaluations.

RESULTS: Three siblings, diagnosed at 14.7, 10.1, and 3.2 years of age, demonstrated clinical improvement with weekly infusions of 2.0 mg/kg …

Effects Of Left Ventricular Assist Device On Pulmonary Functions And Pulmonary Hemodynamics: A Meta-Analysis, Waqas Ullah, Casey Meizinger, Zain Ali, Ankur Panchal, Rehan Saeed, Donald C. Haas, Eduardo Rame

Abington Jefferson Health Papers

BACKGROUND: Given current evidence, the effect of left ventricular assist device (LVAD) implantation on pulmonary function tests remains controversial.

AIM: To better understand the factors contributing to the changes seen on pulmonary function testing and the correlation with pulmonary hemodynamics after LVAD implantation.

METHODS: Electronic databases were queried to identify relevant articles. The summary effect size was estimated as a difference of overall means and standard deviation on a random-effects model.

RESULTS: A total of four studies comprising 219 patients were included. The overall mean forced expiratory volume in one second (FEV1), forced vital capacity (FVC) and diffusion lung capacity …

Medical Therapy Vs Early Revascularization In Diabetics With Chronic Total Occlusions: A Meta-Analysis And Systematic Review, Muhammad Shayan Khan, Farhad Sami, Hemindermeet Singh, Waqas Ullah, Ma'en Al-Dabbas, Khalid Hamid Changal, Tanveer Mir, Zain Ali, Ameer Kabour

Abington Jefferson Health Papers

BACKGROUND: Management of chronic total occlusions (CTO) in diabetics is challenging, with a recent trend towards early revascularization [ER: Percutaneous coronary intervention (PCI) and bypass grafting] instead of optimal medical therapy (OMT). We hypothesize that ER improves morbidity and mortality outcomes in diabetic patients with CTOs as compared to OMT.

AIM: To determine the long term clinical outcomes and to compare morbidity and mortality between OMT and ER in diabetic patients with CTOs.

METHODS: Potentially relevant published clinical trials were identified in Medline, Embase, chemical abstracts and Biosis (from start of the databases till date) and pooled hazard ratios (HR) …

Safety And Efficacy Of Soluble Guanylate Cyclase Stimulators In Patients With Heart Failure: A Systematic Review And Meta-Analysis., Waqas Ullah, Maryam Mukhtar, Aws Al-Mukhtar, Rehan Saeed, Margot Boigon, Donald Haas, Eduardo. Rame

Abington Jefferson Health Papers

BACKGROUND: The utility of novel oral soluble guanylate cyclase (sGC) stimulators (vericiguat and riociguat), in patients with reduced or preserved ejection fraction heart failure (HFrEF/HFpEF) is currently unclear.

AIM: To determine the efficacy and safety of sGC stimulators in HF patients.

METHODS: Multiple databases were searched to identify relevant randomized controlled trials (RCTs). Data on the safety and efficacy of sGC stimulators were compared using relative risk ratio (RR) on a random effect model.

RESULTS: Six RCTs, comprising 5604 patients (2801 in sGC stimulator group and 2803 placebo group) were included. The primary endpoint (a composite of cardiovascular mortality and …

The Difficult Clinical Decision Of Thrombolytic Therapy For Submassive Pulmonary Embolism In A Community Hospital., Qian Zhang, Jonathan Vayalumkal, John Ricely, Daniel L. Gray, Ahmad Raza

Abington Jefferson Health Papers

Submassive or intermediate-risk pulmonary embolism (PE) occurs when an acute PE episode is associated with radiographic evidence of right heart strain without hemodynamic instability. Further risk stratification is important in determining whether systemic thrombolytic therapy should be administered when weighing the risks and benefits. It includes the risk of death from acute PE versus the risk of bleeding. This decision could be further complicated in institutions where there is a lack of complete therapeutic options, which increases the importance of the expertise of a pulmonologist or an intensivist to decide whether rescue reperfusion is needed. We describe the case of …

"Brain Dead" Donor: A Case Of Brain Death Mimic Due To Pentobarbital Overdose, Artem Minalyan, Vincent Chan, Qian Zhang

Abington Jefferson Health Papers

No abstract provided.

An Unlikely Combination Of Neutrophilic Predominant Pleural Effusion With Acid-Fast Bacilli Positivity, Vincent Chan, Qian Zhang, Ahmad Raza, Artem Minalyan, William Ward

Abington Jefferson Health Papers

No abstract provided.

A Roller Coaster Ride Of Idiopathic Fibrosing Mediastinitis, Qian Zhang, Khine Shan

Abington Jefferson Health Papers

No abstract provided.

A Community Assessment Of Rapid Response Team Events Preceding Cardiopulmonary Arrest: Does It Improve Outcomes?, Vincent Chan, Ahmad Raza, Qian Zhang, Artem Minalyan, Rajesh Patel

Abington Jefferson Health Papers

No abstract provided.

Rare Cause Of Fatal Liver Disease: A Case Of Acute Liver Failure In A Patient With Recently Diagnosed Metastatic Ovarian Carcinoma, Artem Minalyan, Qian Zhang, Vincent Chan

Abington Jefferson Health Papers

No abstract provided.

Survival Analysis Based On The Duration Of Cpr After In-Hospital Cardiac Arrest, Ahmad Raza, Nayab Nadeem, Ahmad Arslan, Zain Ali, Qian Zhang, Usama Sadiq, Rajesh Patel

Abington Jefferson Health Papers

No abstract provided.

Acute Covid-19 Induced Fulminant Systemic Vascular Thrombosis: A Novel Entity., Zain Ali, Waqas Ullah, Rehan Saeed, Ammar Ashfaq, Bilal Lashari, Md

Abington Jefferson Health Papers

No abstract provided.

The De Garengeot Hernia: A Case Report Of An Unusual Presentation Of Appendicitis., Davek Sharma, Jacob Katsnelson, Emmanuel Nwachuku, Jeffrey Kolff

Abington Jefferson Health Papers

INTRODUCTION: A De Garengeot hernia is defined by a femoral hernia containing the appendix. Acute appendicitis within a femoral hernia is an extremely rare surgical presentation and occurs in only 0.08-0.013% of cases as cited by the literature.

PRESENTATION OF CASE: A 64-year-old female presented to the Emergency Department of our hospital with acute onset of a right-sided groin bulge that occurred earlier that day after doing heavy lifting. Her workup revealed acute appendicitis contained within an incarcerated right femoral hernia. The patient underwent laparoscopic appendectomy with open femoral hernia repair. Intraoperatively, the appendiceal tip was incarcerated within the hernia …

The Awareness Of Negative Pressure Pulmonary Edema In The Medical Intensive Care Unit., Qian Zhang, Jonathan Vayalumkal, John Ricely, Sarah Elrod, Ahmad Raza

Abington Jefferson Health Papers

Negative pressure pulmonary edema (NPPE) is a noncardiogenic-related rapid onset of bilateral pulmonary edema secondary to various etiologies that lead to upper airway obstruction. Despite the fact that it is more commonly seen in the emergency department (ED), pediatric intensive care unit (PICU), and the postanesthesia care unit (PACU), there is a lower disease prevalence in the medical intensive care unit (MICU) as it sometimes could be overlooked upon. Prompt treatment often leads to favorable clinical outcomes. We detail a 55-year-old lady with a past medical history of nephrolithiasis, obesity, and obstructive sleep apnea presented with right flank pain due …

Efficacy Of Hydroxychloroquine And Tocilizumab In Patients With Covid-19: Single-Center Retrospective Chart Review., Sohaib Roomi, Waqas Ullah, Faizan Ahmed, Soban Farooq, Usama Sadiq, Asad Chohan, Munnam Jafar, Maryum Saddique, Shristi Khanal, Robert Watson, Margot Boigon

Abington Jefferson Health Papers

BACKGROUND: During the initial phases of the COVID-19 pandemic, there was an unfounded fervor surrounding the use of hydroxychloroquine (HCQ) and tocilizumab (TCZ); however, evidence on their efficacy and safety have been controversial.

OBJECTIVE: The purpose of this study is to evaluate the overall clinical effectiveness of HCQ and TCZ in patients with COVID-19. We hypothesize that HCQ and TCZ use in these patients will be associated with a reduction in in-hospital mortality, upgrade to intensive medical care, invasive mechanical ventilation, or acute renal failure needing dialysis.

METHODS: A retrospective cohort study was performed to determine the impact of HCQ …

Hamman-Rich Syndrome: A Diagnosis Of Exclusion In The Covid-19 Pandemic., Qian Zhang, Ahmad Raza, Vincent Chan, Artem Minalyan, John Madara, Md

Abington Jefferson Health Papers

Hamman-Rich syndrome is a rapidly progressive interstitial lung disease with acute respiratory distress syndrome physiology. It carries a grave prognosis and a high early mortality rate. It is often distinguished from other similar pulmonary pathologies based on the clinical course, laboratory findings, bronchoalveolar lavage testing, and pathology report. We detail a 77-year-old lady with no prior pulmonary disease, smoking history, or occupational and environmental exposures present to the emergency department found to be in acute hypoxic respiratory failure with impressive progressive radiographic findings. The presumptive diagnosis of Hamman-Rich syndrome was made based on a combination of factors after ruling out …

Safety And Efficacy Of Hydroxychloroquine In Covid-19: A Systematic Review And Meta-Analysis, Waqas Ullah, Hafez M. Abdullah, Sohaib Roomi, Yasar Sattar, Talal Almas, Smitha Narayana Gowda, Rehan Saeed, Maryam Mukhtar, Ammar Ahmad, Tony Oliver, M. Chadi Alraies, Donald C. Haas, David L. Fischman

Abington Jefferson Health Papers

Background: During the initial phases of the coronavirus disease 2019 (COVID-19) epidemic, there was an unfounded fervor surrounding the use of hydroxychloroquine (HCQ); however, recently, the Centers for Disease Control and Prevention (CDC) has recommended against routine use of HCQ outside of study protocols citing possible adverse outcomes.

Methods: Multiple databases were searched to identify articles on COVID-19. An unadjusted odds ratio (OR) was used to calculate the safety and efficacy of HCQ on a random effect model.

Results: Twelve studies comprising 3,912 patients (HCQ 2,512 and control 1400) were included. The odds of all-cause mortality (OR: 2.23, 95% confidence …

Comparative Accuracy Of Non-Invasive Imaging Versus Right Heart Catheterization For The Diagnosis Of Pulmonary Hypertension: A Systematic Review And Meta-Analysis., Waqas Ullah, Artem Minalyan, Sameer Saleem, Nayab Nadeem, Hafez M Abdullah, Abdelmohaymin Abdalla, Vincent Chan, Rehan Saeed, Maria Khan, Sara Collins, Maryam Mukhtar, Harshwant Grover, Yasar Sattar, Ankur Panchal, Smitha Narayana Gowda, Uneza Khwaja, Bilal Lashari, David L Fischman

Abington Jefferson Health Papers

Background: Right heart catheterization (RHC) is the gold-standard in the diagnosis of pulmonary hypertension (PH) but at the cost of procedure-related complications. We sought to determine the comparative accuracy of RHC versus non-invasive imaging techniques such as computed tomography (CT), magnetic resonance imaging (MRI), and transthoracic echocardiography (TTE).

Methods: Pulmonary hypertension was defined as a mean pulmonary artery pressure (mPAP) of>20 mmHg. Multiple databases were queried for relevant articles. Raw data were pooled using a bivariate model to calculate the measures of diagnostic accuracy and to estimate Hierarchical Summary Receiver Operating Characteristic (HSROC) on Stata 13.

Results: A total …

Percutaneous Intervention Or Bypass Graft For Left Main Coronary Artery Disease? A Systematic Review And Meta-Analysis, Waqas Ullah, Yasar Sattar, Irfan Ullah, Ammu Susheela, Maryam Mukhtar, M Chadi Alraies, Mamas A Mamas, D L Fischman

Abington Jefferson Health Papers

Background: The safety and efficacy of percutaneous coronary intervention (PCI) versus coronary artery bypass grafting (CABG) for stable left main coronary artery disease (LMCAD) remains controversial.

Methods: Digital databases were searched to compare the major adverse cardiovascular and cerebrovascular events (MACCE) and its components. A random effect model was used to compute an unadjusted odds ratio (OR).

Results: A total of 43 studies (37 observational and 6 RCTs) consisting of 29,187 patients (PCI 13,709 and CABG 15,478) were identified. The 30-day rate of MACCE (OR, 0.56; 95% CI, 0.42-0.76;

Conclusion: PCI might offer early safety advantages, while CABG provides greater …

A Mysterious Paratracheal Mass: Parathyroid Carcinoma., Qian Zhang, Khine S. Shan, Neethu Gopisetti, Md, Thomas Yoon, Iqra Iqbal

Abington Jefferson Health Papers

Parathyroid carcinoma is a rare cause of primary hyperparathyroidism. We detail a 60-years-old gentleman who was otherwise healthy presented to the hospital due to acute encephalopathy. He was subsequently found to have parathyroid carcinoma as the cause of the acute encephalopathy with impressive serum calcium and parathyroid hormone levels. The parathyroid carcinoma was later surgically resected with the diagnosis confirmed via pathology specimen. The patient was safely discharged from the hospital with recommendations of close routine outpatient followup.

A Silent Fatal Presentation Of Pulmonary Embolism: Reflection And Discussion., Qian Zhang, Zain U. Abideen, Khine S. Shan, Thomas Yoon, Muhammad Farooq

Abington Jefferson Health Papers

Acute pulmonary embolism is a common medical condition that clinicians face in practice. It is important to have a prompt diagnosis with proper management as it is associated with high morbidity and mortality. However, a timely diagnosis is often difficult to obtain especially when the presenting symptoms are atypical, but the consequence could be fatal. We present an 80-year-old gentleman who presented with a near-syncope episode who subsequently was found to have acute extensive bilateral pulmonary embolisms after a code blue event.

A Mysterious Paratracheal Mass: Pulmonary Agenesis., Qian Zhang, Khine S. Shan

Abington Jefferson Health Papers

A 35-year-old lady with a history of possible tuberculosis infection 15 years ago presented to the clinic with the chief complaint of cough. Incidental chest CT showed a right paratracheal and medial right apical heterogeneous soft tissue mass with central areas of calcification that warranted further investigation. A routine endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) discovered isolated lobar pulmonary agenesis as the underlying cause of the mass without findings of malignancy on pathology reports.

Cost-Effectiveness And Diagnostic Accuracy Of Telemedicine In Macular Disease And Diabetic Retinopathy: A Systematic Review And Meta-Analysis., Waqas Ullah, Sana Khan Pathan, Ankur Panchal, Swapna Anandan, Kaiser Saleem, Yasar Sattar, Ejaz Ahmad, Maryam Mukhtar, Haq Nawaz

Abington Jefferson Health Papers

OBJECTIVE: To determine cost-effectiveness and the diagnostic accuracy of teleophthalmology (TO) in the detection of macular edema (ME) and various grades of diabetic retinopathy (DR).

METHODS: MEDLINE, EMBASE, and Cochrane databases were searched for TO, ME, and DR on May 25, 2016. The search was updated on April 2, 2019. Pooled sensitivity and specificity for ME and various grades of DR were determined using Meta-Disc software. A systematic review of the articles discussing the cost-effectiveness of TO screening was also performed.

RESULTS: Thirty-three articles on the diagnostic accuracy and 28 articles on the cost-effectiveness were selected.

CONCLUSIONS: Telescreening is moderately …

An Exceedingly Rare Presentation Of Severe Folate Deficiency-Induced Non-Immune Hemolytic Anemia., Qian Zhang, Khine S. Shan, Babatunde A. Ogunnaike, Atsoufi Amewuame-Kpehor, Travis Nace

Abington Jefferson Health Papers

Macrocytic anemia is usually associated with vitamin B12 or folate deficiency. However, folate deficiency was rarely reported as a cause of hemolytic anemia. We present a case of a young man with a history of alcohol abuse who initially presented with an acute on chronic abdominal pain and was found to have jaundice and scleral icterus. His liver enzymes were unremarkable, and his abdominal imaging did not reveal any acute pathology. However, he was found to have a severe non-immune hemolytic anemia secondary to folate deficiency.

Efficacy Of Allopurinol In Cardiovascular Diseases: A Systematic Review And Meta-Analysis, Waqas Ullah, Shristi Khanal, Rozi Khan, Bikash Basyal, Samavia Munir, Artem Minalyan, M. Chadi Alraies, David L. Fischman

Abington Jefferson Health Papers

Background: Given current evidence, the use of allopurinol for the prevention of major cardiovascular events (acute cardiovascular syn-drome (ACS) or cardiovascular mortality) in patients undergoing cor-onary artery bypass graft (CABG), after index ACS or heart failure remains unknown.

Methods: Multiple databases were queried to identify studies com-paring the efficacy of allopurinol in patients undergoing CABG, after ACS or heart failure. The unadjusted odds ratio (OR) was calculated using a random effect model.

Results: A total of nine studies comprising 850 patients (allopurinol 480, control 370) were identified. The pooled OR of periprocedural ACS (OR: 0.25, 95% confidence interval (CI): 0.06 …

Diagnostic Accuracy Of Cardiac Magnetic Resonance Versus Fractional Flow Reserve: A Systematic Review And Meta-Analysis., Waqas Ullah, Sohaib Roomi, Hafez M. Abdullah, Maryam Mukhtar, Zain Ali, Ping Ye, Donald C. Haas, Vincent M. Figueredo

Abington Jefferson Health Papers

Background: Fractional flow reserve (FFR) is considered the gold standard for diagnosis of coronary artery disease (CAD). Stress Cardiac magnetic resonance (SCMR) has been recently gaining traction as a non-invasive alternative to FFR.

Methods: Studies comparing the diagnostic accuracy of SCMR versus FFR were identified and analyzed using Review Manager (RevMan) 5.3 and Stata software.

Results: A total of 28 studies, comprising 2,387 patients, were included. The pooled sensitivity and specificity for SCMR were 86% and 86% at the patient level, and 82% and 88% at the vessel level, respectively. When the patient-level data were stratified based on the FFR …

As The Covid-19 Pandemic Drags On, Where Have All The Stemis Gone?, Waqas Ullah, Yasar Sattar, Rehan Saeed, Asrar Ahmad, Margot I. Boigon, Donald C. Haas, M. Chadi Alraies

Abington Jefferson Health Papers

In the midst of current pandemic individuals with all sorts of medical ailments are afraid to venture into health-care settings and risk contracting Coronavirus disease 2019 (COVID-19). Irrespective of the inherent thromboembolic risks associated with COVID-19, this puts patients at high risk of cardiac complications

A Rare Case Report And Literature Review Of Anabolic-Androgenic Steroids (Aas)-Induced Acute Myocardial Infarction, Qian Zhang, Khine S. Shan, Ahmad Raza, Neelima Manda, Travis Nace

Abington Jefferson Health Papers

Anabolic-androgenic steroids (AAS) are synthetic drugs manufactured to mimic the male sex hormone testosterone [1]. They are usually prescribed for medical conditions including but not limited to impotence in males, endometriosis, hypogonadism, and aplastic anemia [2]. It has also been used historically by athletes and bodybuilders to enhance performance as it can stimulate protein synthesis leading to an increase in muscle size and strength [3]. However, the use of AAS has become a serious global public health dilemma as it has been used widely by the general population to improve physical strength and appearance [1]. In …

Outcomes Of Open Mitral Valve Replacement Versus Transcatheter Mitral Valve Repair; Insight From The National Inpatient Sample Database., Waqas Ullah, Yasar Sattar, Maryam Mukhtar, Hafez M. Abdullah, Vincent M. Figueredo, Donald C. Haas, David L. Fischman, M. Chadi Alraies

Abington Jefferson Health Papers

Background: Transcatheter mitral valve repair and replacement (TMVR) is a minimally invasive alternative to conventional open-heart mitral valve replacement (OMVR). The present study aims to compare the burden, demographics, cost, and complications of TMVR and OMVR.

Methods: The United States National Inpatient Sample (US-NIS) for the year 2017 was queried to identify all cases of TMVR and OMVR. Categorical and continuous data were analyzed using Pearson chi-square and independent t-test analysis, respectively. An adjusted odds ratio (aOR) based on the ordinal logistic regression (OLR) model was calculated to determine the association between outcome variables.

Results: Of 19,580 patients, 18,460 …

A Rare Association Of Takotsubo Cardiomyopathy With Neuroleptic Malignant Syndrome, Waqas Ullah, Muhammad Arslan Cheema, Ammar Ashfaq, Maryam Mukhtar, Zain Ali, Mamoon Ur Rashid, Vincent Figueredo

Abington Jefferson Health Papers

Neuroleptic malignant syndrome is a potentially fatal neurological condition secondary toantipsychotic medication. It is characterized by distinctive clinical findings and autonomicdisturbances. NMS has not been associated with Takotsubo cardiomyopathy (TCM).

TCM is an abnormal response to physiological stressors resulting from the autonomicabnormalities which at times can mimic myocardial infarction (MI). We present a unique caseof a 54-year-old female with bipolar disease presenting with lithium and haloperidol-inducedNMS complicated by TCM. The purpose of this case is to make clinicians aware of this rareassociation.