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2019

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Articles 1201 - 1230 of 3514

Full-Text Articles in Medical Specialties

Blunted Evoked Prouroguanylin Endocrine Secretion In Chronic Constipation., Scott A. Waldman, Renata Tenenbaum, Henry C. Foehl, Peter Winkle, Patrick Griffin Jul 2019

Blunted Evoked Prouroguanylin Endocrine Secretion In Chronic Constipation., Scott A. Waldman, Renata Tenenbaum, Henry C. Foehl, Peter Winkle, Patrick Griffin

Department of Pharmacology and Experimental Therapeutics Faculty Papers

OBJECTIVES: Prouroguanylin (ProUGN) in the intestine is cleaved to form uroguanylin (UGN), which stimulates guanylate cyclase C (GUCY2C), inducing cyclic guanosine monophosphate signaling. Paracrine release regulates fluid secretion, contributing to bowel function, whereas endocrine secretion evoked by eating forms a gut-brain axis, controlling appetite. Whereas hormone insufficiency contributes to hyperphagia in obesity, its contribution to the pathophysiology of constipation syndromes remains unexplored. Here, we compared circulating ProUGN and UGN in healthy subjects and in patients with chronic idiopathic constipation (CIC) and patients with irritable bowel syndrome with constipation (IBS-C).

METHODS: Circulating ProUGN and UGN levels were measured in 60 healthy …


Quality Assurance In Telehealth: Adherence To Evidence-Based Indicators., Daniel Halpren-Ruder, Anna Marie Chang, Judd E. Hollander, Anuh Shah Jul 2019

Quality Assurance In Telehealth: Adherence To Evidence-Based Indicators., Daniel Halpren-Ruder, Anna Marie Chang, Judd E. Hollander, Anuh Shah

Department of Emergency Medicine Faculty Papers

Background: Value enhancing telehealth (TH) lacks a robust body of formal clinically focused quality assessment studies. Innovations such as telehealth must always demonstrate that it preserves or hopefully advances quality. Introduction: We sought to determine whether adherence to the evidence-based Choosing Wisely (CW) recommendations (antibiotic stewardship) for acute sinusitis differs for encounters through direct-to-consumer (DTC) telemedicine verses "in-person" care in an emergency department (ED) or an urgent care (UC) center.

Materials and Methods: Study design was a retrospective review. Patients with a symptom complex consistent with acute sinusitis treated through DTC were matched with ED and UC patients, based upon …


A Technique For Approximating Transition Rates From Published Survival Analyses, Markian A. Pahuta, Joel Werier, Eugene K. Wai, Roy A. Patchell, Doug Coyle Jul 2019

A Technique For Approximating Transition Rates From Published Survival Analyses, Markian A. Pahuta, Joel Werier, Eugene K. Wai, Roy A. Patchell, Doug Coyle

Neurology Faculty Publications

Background: Quality-adjusted-life-years (QALYs) are used to concurrently quantify morbidity and mortality within a single parameter. For this reason, QALYs can facilitate the discussion of risks and benefits during patient counseling regarding treatment options. QALYs are often calculated using partitioned-survival modelling. Alternatively, QALYs can be calculated using more flexible and informative state-transition models populated with transition rates estimated using multistate modelling (MSM) techniques. Unfortunately the latter approach is considered not possible when only progression-free survival (PFS) and overall survival (OS) analyses are reported.

Methods: We have developed a method that can be used to estimate approximate transition rates from published PFS …


Performance Of The Timi Risk Score In Predicting Mortality After Primary Percutaneous Coronary Intervention In Elderly Women: Results From A Developing Country, Shumaila Furnaz, Musa Karim, Tariq Ashraf, Sajjad Ali, Izza Shahid, Sara Ali, Uzzam Ahmed Khawaja, Muhammad Tanzeel Ul Haque, Muhammad Shariq Usman, Tariq Jamal Siddiqi Jul 2019

Performance Of The Timi Risk Score In Predicting Mortality After Primary Percutaneous Coronary Intervention In Elderly Women: Results From A Developing Country, Shumaila Furnaz, Musa Karim, Tariq Ashraf, Sajjad Ali, Izza Shahid, Sara Ali, Uzzam Ahmed Khawaja, Muhammad Tanzeel Ul Haque, Muhammad Shariq Usman, Tariq Jamal Siddiqi

Section of Internal Medicine

Background: Despite women undergoing primary percutaneous coronary intervention (PPCI) having a higher rate of adverse outcomes than men, data evaluating prognostic risk scores, especially in elderly women, remains scarce. This study was conducted to validate the predictive value of Thrombolysis in Myocardial Infarction (TIMI) risk score in elderly female patients.

Materials and Methods: This was a retrospective analysis of elderly (>65 years) female patients who underwent PPCI for ST-elevated myocardial infarction (STEMI) from October 2016 to September 2018. Patients' demographic details and elements of TIMI risk score including age, co-morbidities, Killip classification; weight, anterior MI and total ischemic time …


Ralstonia Pickettii Bacteremia: An Emerging Infection In A Tertiary Care Hospital Setting, Nosheen Nasir, Muneeba Ahsan Sayeed, Bushra Jamil Jul 2019

Ralstonia Pickettii Bacteremia: An Emerging Infection In A Tertiary Care Hospital Setting, Nosheen Nasir, Muneeba Ahsan Sayeed, Bushra Jamil

Section of Internal Medicine

Ralstonia species are Gram-negative bacilli that have increasingly been recognized as emerging nosocomial pathogens, particularly in immunocompromised hosts. Ralstonia pickettii is the most clinically important pathogen from the Ralstonia genus. Nosocomial outbreaks of Ralstonia pickettii infections brought about by the use of contaminated medical solutions, including saline, sterile water, as well as disinfectants, have been reported. There have been case reports of invasive infections with variable presentations. Here, we describe three cases of Ralstonia pickettii bacteremia during a period of one year in a tertiary care hospital in Karachi, Pakistan. The first case was a 76-year-old male, known case of …


Performance Of The Timi Risk Score In Predicting Mortality After Primary Percutaneous Coronary Intervention In Elderly Women: Results From A Developing Country, Shumaila Furnaz, Musa Karim, Tariq Ashraf, Sajjad Ali, Izza Shahid, Sara Ali, Uzzam Ahmed Khawaja, Muhammad Tanzeel Ul Haque, Muhammad Shariq Usman, Tariq Jamal Siddiqi Jul 2019

Performance Of The Timi Risk Score In Predicting Mortality After Primary Percutaneous Coronary Intervention In Elderly Women: Results From A Developing Country, Shumaila Furnaz, Musa Karim, Tariq Ashraf, Sajjad Ali, Izza Shahid, Sara Ali, Uzzam Ahmed Khawaja, Muhammad Tanzeel Ul Haque, Muhammad Shariq Usman, Tariq Jamal Siddiqi

Section of Internal Medicine

Background: Despite women undergoing primary percutaneous coronary intervention (PPCI) having a higher rate of adverse outcomes than men, data evaluating prognostic risk scores, especially in elderly women, remains scarce. This study was conducted to validate the predictive value of Thrombolysis in Myocardial Infarction (TIMI) risk score in elderly female patients.

Materials and methods: This was a retrospective analysis of elderly (>65 years) female patients who underwent PPCI for ST-elevated myocardial infarction (STEMI) from October 2016 to September 2018. Patients' demographic details and elements of TIMI risk score including age, co-morbidities, Killip classification; weight, anterior MI and total ischemic …


Unique Classification Of Parathyroid Dysfunction In Patients With Transfusion Dependent Thalassemia Major Using Nomogram: A Cross Sectional Study, Hafsa Majid, Lena Jafri, Jamsheer Jehangir Talati, Sibtain Ahmed, Bushra Moiz, Aysha Habib Khan Jul 2019

Unique Classification Of Parathyroid Dysfunction In Patients With Transfusion Dependent Thalassemia Major Using Nomogram: A Cross Sectional Study, Hafsa Majid, Lena Jafri, Jamsheer Jehangir Talati, Sibtain Ahmed, Bushra Moiz, Aysha Habib Khan

Department of Pathology and Laboratory Medicine

Introduction: Hypoparathyroidism is a rare complication of iron overload in patients with transfusion dependent β thalassemia major (β-TM). We aim to determine the prevalence of parathyroid dysfunction in patients with β-TM.
Methods:Diagnosed cases of transfusion dependent β-TM between 5 and 17 years of age were recruited from outpatient clinics of a non-profit organization in Karachi, Pakistan. Blood and urine samples were collected in fasting to determine Ca, P, Alb, Mg, Cr 25OHD and iPTH. Patients were grouped on the basis of upper and lower levels of Ca, 25OHD and iPTH for assessing parathyroid dysfunction into primary hypoparathyroidism [low calcium …


Trial Of Canakinumab, An Il-1Β Receptor Antagonist, In Patients With Inclusion Body Myositis., Michalis L Kosmidis, Dimitris Pikazis, Panayotis Vlachoyiannopoulos, Athanasios G Tzioufas, Marinos C. Dalakas Jul 2019

Trial Of Canakinumab, An Il-1Β Receptor Antagonist, In Patients With Inclusion Body Myositis., Michalis L Kosmidis, Dimitris Pikazis, Panayotis Vlachoyiannopoulos, Athanasios G Tzioufas, Marinos C. Dalakas

Department of Neurology Faculty Papers

Objective: To assess whether canakinumab, a monoclonal antibody against IL-1β approved for autoinflammatory diseases, is effective as target-specific therapy in patients with sporadic inclusion body myositis (sIBM).

Methods: Because in sIBM IL-1β colocalizes with amyloid precursor protein and upregulates amyloid aggregates enhancing degeneration, targeting IL-1β with canakinumab may arrest disease progression. On this basis, 5 ambulatory patients with sIBM participated in an institutional review board--approved open-labeled study with 150 mg canakinumab [4 bimonthly, then monthly subcutaneous injections] for a mean period of 15.8 months. Patients were assessed bimonthly with a manual dynamometer in 12 proximal and distal muscles and with …


Gangliosides: Treatment Avenues In Neurodegenerative Disease., Pierre J. Magistretti, Fred H. Geisler, Jay S. Schneider, P. Andy Li, Hubert Fiumelli, Simonetta Sipione Jul 2019

Gangliosides: Treatment Avenues In Neurodegenerative Disease., Pierre J. Magistretti, Fred H. Geisler, Jay S. Schneider, P. Andy Li, Hubert Fiumelli, Simonetta Sipione

Department of Pathology, Anatomy, and Cell Biology Faculty Papers

Gangliosides are cell membrane components, most abundantly in the central nervous system (CNS) where they exert among others neuro-protective and -restorative functions. Clinical development of ganglioside replacement therapy for several neurodegenerative diseases was impeded by the BSE crisis in Europe during the 1990s. Nowadays, gangliosides are produced bovine-free and new pre-clinical and clinical data justify a reevaluation of their therapeutic potential in neurodegenerative diseases. Clinical experience is greatest with monosialo-tetrahexosyl-ganglioside (GM1) in the treatment of stroke. Fourteen randomized controlled trials (RCTs) in overall >2,000 patients revealed no difference in survival, but consistently superior neurological outcomes vs. placebo. GM1 was shown …


Comparison Of The Sequential Organ Failure Assessment (Sofa) And Quick Sofa Scores In Predicting In-Hospital Mortality Among Adult Critical Care Patients With Suspected Infection, Mohammed A. Said, Wangari Siika, Vitalis Mung’Ayi, Reena Shah Jul 2019

Comparison Of The Sequential Organ Failure Assessment (Sofa) And Quick Sofa Scores In Predicting In-Hospital Mortality Among Adult Critical Care Patients With Suspected Infection, Mohammed A. Said, Wangari Siika, Vitalis Mung’Ayi, Reena Shah

Internal Medicine, East Africa

Introduction: Sepsis is global health priority and the leading cause of death in critical care. The SEPSIS 3 criteria introduced in 2016 is the latest tool in diagnosing sepsis. It uses SOFA and qSOFA scores in place of the SIRS criteria for better ability to predict mortality in patients with suspected infections. The performance of these scores in critical care units outside high-income countries remains largely unknown.

Methods: We compared the performance of SOFA and qSOFA in predicting the in-hospital mortality of an adult critical care unit in Kenya. We conducted a retrospective review of all patients admitted …


Metastatic Breast Cancer In Kenya: Presentation, Pathologic Characteristics, And Patterns-Findings From A Tertiary Cancer Center, Etoroabasi Ekpe, Asim Jamal, Jasmit Shah, Judith S. Jacobson, Shahin Sayed Jul 2019

Metastatic Breast Cancer In Kenya: Presentation, Pathologic Characteristics, And Patterns-Findings From A Tertiary Cancer Center, Etoroabasi Ekpe, Asim Jamal, Jasmit Shah, Judith S. Jacobson, Shahin Sayed

Internal Medicine, East Africa

Purpose: The purpose of this research was to describe the sociodemographic and clinical characteristics of Kenyan women with metastatic breast cancer diagnosed and treated at Aga Khan University Hospital in Nairobi, Kenya from 2012 to 2018.

Patients and Methods: We reviewed charts of Kenyan women with metastatic breast cancer and analyzed sociodemographic data, breast cancer risk factors, and tumor characteristics associated with stage at diagnosis, receptor status (ie, estrogen receptor, progesterone receptor, and human epidermal growth factor receptor 2 [HER2]), and site of metastasis using χ2, analysis of variance, two-sample t tests, and logistic regressions.

Results: A total of 125 …


Acinetobacter Infections: A Retrospective Study To Determine In Hospital Mortality Rate And Clinical Factors Associated With Mortality, Rajiv Patel, Jasmit Shah, Gunturu Revathi, Wangari Siika, Reena Shah Jul 2019

Acinetobacter Infections: A Retrospective Study To Determine In Hospital Mortality Rate And Clinical Factors Associated With Mortality, Rajiv Patel, Jasmit Shah, Gunturu Revathi, Wangari Siika, Reena Shah

Internal Medicine, East Africa

A retrospective case series of acinetobacter infections at a tertiary hospital in Nairobi was conducted to determine the mortality rate and factors associated with mortality. Over an eight-year period, 80 clinically significant infections were identified. The majority of infections were ventilator-associated pneumonia (40%) and bloodstream infections (30%). Eighty-six percent of the isolates were multi-drug resistant. The mortality rate in the study cohort was 45%. Twelve patients grew Acinetobacter spp. within 48 h of hospitalization, and three of these patients had no prior healthcare contact. The mean Sequential Organ Failure Assessment score was associated with mortality from acinetobacter infections.


Commentary: The History Of Neurosurgery At Albany Medical College And Albany Medical Center Hospital, Albany, New York., Pouya Entezami, Michael R. Gooch, Adedamola Adepoju, Julie Pilitsis, Tyler J. Kenning, Alan Boulos, David L. Semenoff Jul 2019

Commentary: The History Of Neurosurgery At Albany Medical College And Albany Medical Center Hospital, Albany, New York., Pouya Entezami, Michael R. Gooch, Adedamola Adepoju, Julie Pilitsis, Tyler J. Kenning, Alan Boulos, David L. Semenoff

Department of Neurosurgery Faculty Papers

The origins of the Department of Neurosurgery at Albany Medical College closely parallel the development of early America and the establishment of modern health care.The tales of Washington Irving, the works of the Hudson River School of painters, and summers in the Catskill Mountains or Adirondacks are the stories that color the history of Upstate New York (Figure1). As a social, industrial, and political hub of the American colonies, New England’s need for centers providing structured medicine led to the creation of Albany Medical College in1839, one of the earliest such institutions in the young nation.1 Rapid progress in nearly …


Htx-011 Reduced Pain Intensity And Opioid Consumption Versus Bupivacaine Hcl In Bunionectomy: Phase Iii Results From The Randomized Epoch 1 Study., Eugene R. Viscusi, Joseph S. Gimbel, Richard A. Pollack, Jia Hu, Gwo-Chin Lee Jul 2019

Htx-011 Reduced Pain Intensity And Opioid Consumption Versus Bupivacaine Hcl In Bunionectomy: Phase Iii Results From The Randomized Epoch 1 Study., Eugene R. Viscusi, Joseph S. Gimbel, Richard A. Pollack, Jia Hu, Gwo-Chin Lee

Department of Anesthesiology Faculty Papers

BACKGROUND AND OBJECTIVES: There is a need for local anesthetics that provide consistent analgesia through 72 hours after surgery. This study evaluates the use of HTX-011 (bupivacaine and meloxicam in Biochronomerpolymer technology), an extended-release, dual-acting local anesthetic, in reducing both postoperative pain over 72 hours and postoperative opioid use when compared with bupivacaine hydrochloride (HCl) and saline placebo. Inclusion of low-dose meloxicam in HTX-011 is designed to reduce local inflammation caused by surgery, potentiating the analgesic effect of bupivacaine. Previously, significant synergy has been observed with bupivacaine and meloxicam with both given locally together.

METHODS: EPOCH 1 was a randomized, …


Extension For Community Healthcare Outcomes-Palliative Care In Africa Program: Improving Access To Quality Palliative Care, Sriram Yennurajalingam, Charles E. Amos, John Weru, Edwina Beryl, Addo Opare-Lokko, Joseph Anthony Arthur, Kristy Nguyen, Olaitan Soyannwo, Runcie C.W. Chidebe, Janet L. Williams, Zhanni Lu, Ellen Baker, Sanjeev Arora, Eduardo Bruera, Suresh Reddy Jul 2019

Extension For Community Healthcare Outcomes-Palliative Care In Africa Program: Improving Access To Quality Palliative Care, Sriram Yennurajalingam, Charles E. Amos, John Weru, Edwina Beryl, Addo Opare-Lokko, Joseph Anthony Arthur, Kristy Nguyen, Olaitan Soyannwo, Runcie C.W. Chidebe, Janet L. Williams, Zhanni Lu, Ellen Baker, Sanjeev Arora, Eduardo Bruera, Suresh Reddy

Internal Medicine, East Africa

Purpose: There is limited access to quality palliative care (PC) for patients with advanced cancer in sub-Saharan Africa. Our aim was to describe the development of the Project Extension for Community Healthcare Outcomes- Palliative Care in Africa (ECHO-PACA) program and describe a preliminary evaluation of attitudes and knowledge of participants regarding the ability of the program to deliver quality PC.

Methods: An interdisciplinary team at the MD Anderson Cancer Center, guided by experts in PC in sub-Saharan Africa, adapted a standardized curriculum based on PC needs in the region. Participants were then recruited, and monthly telementoring sessions were held for …


Hypopituitarism Other Than Sellar And Parasellar Tumors Or Traumatic Brain Injury Assessed In A Tertiary Hospital, Sarwar Malik, Zareen Kiran,, Owais Rashid, Minaz Mawani, Asma Gulab, Qamar Masood, Najmul Islam Jul 2019

Hypopituitarism Other Than Sellar And Parasellar Tumors Or Traumatic Brain Injury Assessed In A Tertiary Hospital, Sarwar Malik, Zareen Kiran,, Owais Rashid, Minaz Mawani, Asma Gulab, Qamar Masood, Najmul Islam

Section of Diabetes, Endocrinology and Metabolism

Objective: Data regarding the etiology, clinical and biochemical patterns in hypopituitarism is scant for Pakistan. We describe the characteristics of patients with hypopituitarism other than sellar and parasellar tumors or traumatic brain injury from a tertiary care center in Pakistan.

Methods: We conducted a retrospective descriptive study in the Aga Khan University Hospital, Karachi, Pakistan. We studied all patients presenting with hypopituitarism, between January 2004 and December 2013. Clinical, hormonal and imaging data pertinent to the study was collected according to inclusion criteria.

Results: Forty-two patients presented to the endocrinology clinics at the Aga Khan University Hospital during the study …


Acute-On-Chronic Liver Failure: Consensus Recommendations Of The Asian Pacific Association For The Study Of The Liver (Apasl): An Update, Shiv Kumar Sarin, Ashok Choudhury, Manoj K. Sharma, Rakhi Maiwall, Mamun Al Mahtab, Salimur Rahman, Saeed Hamid, Amna S. Butt, Wasim Jafri, Zaigham Abbas Jul 2019

Acute-On-Chronic Liver Failure: Consensus Recommendations Of The Asian Pacific Association For The Study Of The Liver (Apasl): An Update, Shiv Kumar Sarin, Ashok Choudhury, Manoj K. Sharma, Rakhi Maiwall, Mamun Al Mahtab, Salimur Rahman, Saeed Hamid, Amna S. Butt, Wasim Jafri, Zaigham Abbas

Section of Gastroenterology

The first consensus report of the working party of the Asian Pacific Association for the Study of the Liver (APASL) set up in 2004 on acute-on-chronic liver failure (ACLF) was published in 2009. With international groups volunteering to join, the "APASL ACLF Research Consortium (AARC)" was formed in 2012, which continued to collect prospective ACLF patient data. Based on the prospective data analysis of nearly 1400 patients, the AARC consensus was published in 2014. In the past nearly four-and-a-half years, the AARC database has been enriched to about 5200 cases by major hepatology centers across Asia. The data published during …


The Concentration Of Total Nucleated Cells In Harvested Bone Marrow For Transplantation Has Decreased Over Time., Nicole L. Prokopishyn, Brent R. Logan, Deidre M. Kiefer, Jennifer A. Sees, Pintip Chitphakdithai, Ibrahim A. Ahmed, Paolo N. Anderlini, Amer M. Beitinjaneh, Christopher Bredeson, Jan Cerny, Saurabh Chhabra, Andrew Daly, Miguel Angel Diaz, Nosha Farhadfar, Haydar A. Frangoul, Siddhartha Ganguly, Dennis A. Gastineau, Usama Gergis, Gregory A. Hale, Peiman Hematti, Rammurti T. Kamble, Kimberly A. Kasow, Hillard M. Lazarus, Jane L. Liesveld, Hemant S. Murthy, Maxim Norkin, Richard F. Olsson, Mona Papari, Bipin N. Savani, Jeffrey Szer, Edmund K. Waller, Baldeep Wirk, Jean A. Yared, Michael A. Pulsipher, Nirali N. Shah, Galen E. Switzer, Paul V. O'Donnell, Dennis L. Confer, Bronwen E. Shaw Jul 2019

The Concentration Of Total Nucleated Cells In Harvested Bone Marrow For Transplantation Has Decreased Over Time., Nicole L. Prokopishyn, Brent R. Logan, Deidre M. Kiefer, Jennifer A. Sees, Pintip Chitphakdithai, Ibrahim A. Ahmed, Paolo N. Anderlini, Amer M. Beitinjaneh, Christopher Bredeson, Jan Cerny, Saurabh Chhabra, Andrew Daly, Miguel Angel Diaz, Nosha Farhadfar, Haydar A. Frangoul, Siddhartha Ganguly, Dennis A. Gastineau, Usama Gergis, Gregory A. Hale, Peiman Hematti, Rammurti T. Kamble, Kimberly A. Kasow, Hillard M. Lazarus, Jane L. Liesveld, Hemant S. Murthy, Maxim Norkin, Richard F. Olsson, Mona Papari, Bipin N. Savani, Jeffrey Szer, Edmund K. Waller, Baldeep Wirk, Jean A. Yared, Michael A. Pulsipher, Nirali N. Shah, Galen E. Switzer, Paul V. O'Donnell, Dennis L. Confer, Bronwen E. Shaw

Manuscripts, Articles, Book Chapters and Other Papers

Bone marrow (BM) is an essential source of hematopoietic stem cell grafts for many allogeneic hematopoietic cell transplant (HCT) recipients, including adult patients (for specific diseases and transplantation strategies) and the majority of pediatric recipient. However, since the advent of granulocyte colony-stimulating factor-mobilized peripheral blood stem cell (PBSC) grafts, there has been a significant decrease in the use of BM in HCT, thought to be due mainly to the increased logistical challenges in harvesting BM compared with PBSCs, as well as generally no significant survival advantage of BM over PBSCs. The decreased frequency of collection has the potential to impact …


Initiating The Ketogenic Diet In Infants With Treatment Refractory Epilepsy While Maintaining A Breast Milk Diet, Jean-Baptist Lepichon, Lindsey M. Thompson, Megan Gustafson, Ahmed Abdelmoity Jul 2019

Initiating The Ketogenic Diet In Infants With Treatment Refractory Epilepsy While Maintaining A Breast Milk Diet, Jean-Baptist Lepichon, Lindsey M. Thompson, Megan Gustafson, Ahmed Abdelmoity

Manuscripts, Articles, Book Chapters and Other Papers

© 2019 The Authors

Purpose: The ketogenic diet has been found to be safe and effective in the treatment of drug resistant epilepsy in childhood. The age range of children undergoing this treatment has steadily been going down. There is strong evidence that it is a safe alternative in infants with drug resistant seizures. The American Academy of Pediatrics strongly supports continuing a breast milk diet until infants are at least six months of age. The purpose of this study is to evaluate the safety and efficacy of the ketogenic diet in infants while maintaining a breast milk diet.

Method: …


Biochemical And Clinical Response After Umbilical Cord Blood Transplant In A Boy With Early Childhood-Onset Beta-Mannosidosis., Troy C. Lund, Weston P. Miller, Julie B. Eisengart, Katrina Simmons, Laura Pollard, Deborah L. Renaud, David A. Wenger, Marc C. Patterson, Paul J. Orchard Jul 2019

Biochemical And Clinical Response After Umbilical Cord Blood Transplant In A Boy With Early Childhood-Onset Beta-Mannosidosis., Troy C. Lund, Weston P. Miller, Julie B. Eisengart, Katrina Simmons, Laura Pollard, Deborah L. Renaud, David A. Wenger, Marc C. Patterson, Paul J. Orchard

Department of Neurology Faculty Papers

BACKGROUND: Deficiency in the enzyme β-mannosidase was described over three decades ago. Although rare in occurrence, the presentation of childhood-onset β-mannosidase deficiency consists of hypotonia in the newborn period followed by global development delay, behavior problems, and intellectual disability. No effective pharmacologic treatments have been available.

METHODS: We report 2-year outcomes following the first umbilical cord blood transplant in a 4-year-old boy with early childhood-onset disease.

RESULTS: We show restoration of leukocyte β-mannosidase activity which remained normal at 2 years posttransplant, and a simultaneous increase in plasma β-mannosidase activity and dramatic decrease in urine-free oligosaccharides were also observed. MRI of …


Current Laboratory Techniques Commonly Used For The Detection Of Molecular Biomarkers And Potential Technologies On The Horizon, Muhammad Shariq Shaikh, Zeeshan Ansar Ahmed Jul 2019

Current Laboratory Techniques Commonly Used For The Detection Of Molecular Biomarkers And Potential Technologies On The Horizon, Muhammad Shariq Shaikh, Zeeshan Ansar Ahmed

Department of Pathology and Laboratory Medicine

No abstract provided.


Guillain-Barre Syndrome Following A Snakebite: A Case Report And Review Of Literature, Sajid Hameed, Mubashar Memon, Sara Khan Jul 2019

Guillain-Barre Syndrome Following A Snakebite: A Case Report And Review Of Literature, Sajid Hameed, Mubashar Memon, Sara Khan

Section of Neurology

Guillain-Barre syndrome (GBS) is preceded by a respiratory or gastrointestinal illness in more than 50% of the patients. A rare association of GBS with a preceding snakebite is reported in the literature in only four previous cases. We present a case report of a patient diagnosed with GBS following the bite of a yellow-bellied sea snake (Hydrophis platurus) and a brief review of the available literature.


Yield Of Muscle Biopsy In Patients With Findings Of Myopathy On Electrodiagnostic Testing, Sarah H. Siddiqui, Raheel Ahmed, Safia Awan, Ambreen Zain, Sara Khan Jul 2019

Yield Of Muscle Biopsy In Patients With Findings Of Myopathy On Electrodiagnostic Testing, Sarah H. Siddiqui, Raheel Ahmed, Safia Awan, Ambreen Zain, Sara Khan

Section of Neurology

Background: The evaluation of neuromuscular diseases includes detailed clinical assessment, blood testing, electrodiagnostic studies (EDS), biopsy, and genetic tests. EDS alone cannot provide a specific diagnosis. Further testing in the form of genetic tests or muscle biopsy (MB) is required. Objective The objective of the study is to evaluate the yield of MB in patients with findings of myopathy on electrodiagnostic testing and assess the factors affecting an abnormal biopsy outcome.

Methods: Electromyography (EMG)/nerve conduction studies (NCS) performed for suspected myopathy over 5 years from 2011 to 2016, at the neurophysiology department of a tertiary care center in Pakistan, were …


Comparison Of Cyanoacrylate Closure And Radiofrequency Ablation For The Treatment Of Incompetent Great Saphenous Veins: 36-Month Outcomes Of The Veclose Randomized Controlled Trial., Nick Morrison, Raghu Kolluri, Michael Vasquez, Monte Madsen, Andrew Jones, Kathleen Gibson Jul 2019

Comparison Of Cyanoacrylate Closure And Radiofrequency Ablation For The Treatment Of Incompetent Great Saphenous Veins: 36-Month Outcomes Of The Veclose Randomized Controlled Trial., Nick Morrison, Raghu Kolluri, Michael Vasquez, Monte Madsen, Andrew Jones, Kathleen Gibson

Heart and Vascular Articles

OBJECTIVE: To evaluate the 36-month efficacy and safety of cyanoacrylate closure for the treatment of incompetent great saphenous veins in comparison with radiofrequency ablation.

METHODS: In this multicenter, prospective, randomized controlled trial, 222 symptomatic subjects with incompetent great saphenous veins were assigned to either cyanoacrylate closure or radiofrequency ablation. The primary endpoint, complete closure of the target great saphenous vein, was determined using duplex ultrasound examination starting from three-month visit.

RESULTS: At month 36, the great saphenous vein closure rates were 94.4% for the cyanoacrylate closure group and 91.9% for the radiofrequency ablation group. Stable improvement in symptoms and quality …


Factors Associated With The Discontinuation Of Modern Methods Of Contraception In The Low Income Areas Of Sukh Initiative Karachi: A Community-Based Case Control Study, Rozina Thobani, Saleem Jessani, Iqbal Azam Syed, Sayyeda Reza, Neelofar Sami, Shafquat Rozi, Farina Abrejo, Sarah Saleem Jul 2019

Factors Associated With The Discontinuation Of Modern Methods Of Contraception In The Low Income Areas Of Sukh Initiative Karachi: A Community-Based Case Control Study, Rozina Thobani, Saleem Jessani, Iqbal Azam Syed, Sayyeda Reza, Neelofar Sami, Shafquat Rozi, Farina Abrejo, Sarah Saleem

Department of Paediatrics and Child Health

Introduction:
Discontinuation of a contraceptive method soon after its initiation is becoming a public health problem in Low middle income countries and may result in unintended pregnancy and related unwanted consequences. A better understanding of factors behind discontinuation of a modern method would help in designing interventions to continue its use till desired spacing goals are achieved. |
Objective:
To determine factors associated with the discontinuation of modern contraceptive methods within six months of its use compared to continued use of modern method for at least six months in low-income areas of Karachi, Pakistan.
Method:
Community-based case-control study was …


Effect Of Diabetic Counseling Based On Conversation Map As Compared To Routine Counseling On Diabetes Management Self-Efficacy And Diabetic Distress Among Patients With Diabetes In Pakistan: A Randomized Controlled Trial (Study Protocol), Rubina Qasim, Sarfaraz Masih, Mehwish Hussain, Akhter Ali, Ajmal Khan, Yousaf Shah, Hakim Shah, Mohammad Tahir Yousafzai Jul 2019

Effect Of Diabetic Counseling Based On Conversation Map As Compared To Routine Counseling On Diabetes Management Self-Efficacy And Diabetic Distress Among Patients With Diabetes In Pakistan: A Randomized Controlled Trial (Study Protocol), Rubina Qasim, Sarfaraz Masih, Mehwish Hussain, Akhter Ali, Ajmal Khan, Yousaf Shah, Hakim Shah, Mohammad Tahir Yousafzai

Department of Paediatrics and Child Health

Background:
Novel interactive and pictorial health education tool named Diabetes Conversation Map (DCM) might be effective for the improvement of diabetes management self-efficacy (DMSE) and diabetes distress (DD) among diabetic patients in lower middle-income setting. This study protocol will assess the effectiveness of DCM as compared to routine care (RC) to improve DMSE, decrease DD and glycated hemoglobin (HbA1c) among patients with type 2 diabetes (T2DM).
Methods:
This will be two arms randomized controlled trial, conducted at national institute of diabetes and endocrinology (NIDE) in Karachi, Pakistan. A sample of 120 T2DM patients of age 30–60 years with suboptimal diabetes …


Increasing Actual And Perceived Burden Of Tick-Borne Disease In Maine, Robert P. Smith Md Mph, Carol A. Mccarthy Md, Susan P. Elias Phd Jun 2019

Increasing Actual And Perceived Burden Of Tick-Borne Disease In Maine, Robert P. Smith Md Mph, Carol A. Mccarthy Md, Susan P. Elias Phd

Journal of Maine Medical Center

Introduction: The burden of tick-borne disease (TBD) in Maine has steadily increased since the first case of Lyme disease was reported in the late 1980s. The emergence of five different agents of TBD in Maine has been challenging and confusing for clinicians and the public.

Methods: We reviewed the ecology of emerging of tick -borne disease, then reviewed risk factors for tick bites and tick-borne disease in Maine. We then compared the burden of TBD versus community-acquired comparison infections in terms of hospitalizations, deaths, and media attention.

Results and Discussion: In Maine, risk of exposure to bites from the vector …


A Case Report Of Water Hemlock Poisoning, Blue Butterfield, Alicia Bond Md, Dan Meyer, Gary Diperna, Tamas Peredy Md Jun 2019

A Case Report Of Water Hemlock Poisoning, Blue Butterfield, Alicia Bond Md, Dan Meyer, Gary Diperna, Tamas Peredy Md

Journal of Maine Medical Center

Introduction: Water hemlock poisoning is an uncommon cause of seizures, gastrointestinal upset, and renal failure. This poisoning occurs infrequency and is likely to go unrecognized without a proper history and consideration in the differential diagnosis.

Clinical Findings: A 23-year-old male with an unremarkable past medical history presented to the emergency department after being found unresponsive at a farm where he was employed. He had several tonic-clonic seizures en route. Initial evaluation was unremarkable and included toxicology screening, lumbar puncture, and brain imaging.

Main diagnoses, therapeutics, interventions, and outcomes: The patient was treated with broad-spectrum antibiotics and antivirals with a suspected …


Signs Of Psychosis Leading To A Diagnosis Of Progressive Multifocal Leukoencephalopathy: A Case Report, Bryan L. Walker, Christina Holt Md, Msc Jun 2019

Signs Of Psychosis Leading To A Diagnosis Of Progressive Multifocal Leukoencephalopathy: A Case Report, Bryan L. Walker, Christina Holt Md, Msc

Journal of Maine Medical Center

Introduction: Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating process of the central nervous system that occurs in in the setting of severe immunocompromise. Patients with PML develop varying focal neurological deficits and mental status changes that have not been well-described previously.

Clinical findings: We present a patient who was found by security wandering the lobby of this hospital. He was oriented only to self and unable to state a reason for presentation. Medical records were limited and included a visit to an outside hospital that documented a past medical history of HIV infection. He exhibited multiple signs of psychosis …


Increased 18f-Fdg-Pet Uptake In Granulomatosis With Polyangiitis: Case Report And Review Of The Literature, Rama El-Yafawi, Patricia Cantlin, Thomas E. Van Der Kloot Jun 2019

Increased 18f-Fdg-Pet Uptake In Granulomatosis With Polyangiitis: Case Report And Review Of The Literature, Rama El-Yafawi, Patricia Cantlin, Thomas E. Van Der Kloot

Journal of Maine Medical Center

We present an unusual case of granulomatosis with polyangiitis (GPA), which initially presented as a large lung mass, without renal or other systemic features. The lung mass was most concerning for malignancy, and positron emission tomography (PET) further supported this concern, with intense uptake in the mass as well as in mediastinal and hilar lymph nodes. Biopsies however were non-diagnostic, and the patient developed acute kidney injury with active urinary sediment, leading to a definitive diagnosis of GPA. With supportive care and treatment directed at GPA, she was ultimately able to discontinue hemodialysis, and the lung mass resolved. This case …