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https://www.readbyqxmd.com/read/28603745/lentivector-iterations-and-pre-clinical-scale-up-toxicity-testing-targeting-mobilized-cd34-cells-for-correction-of-fabry-disease
#1
Ju Huang, Aneal Khan, Bryan C Au, Dwayne L Barber, Lucía López-Vásquez, Nicole L Prokopishyn, Michel Boutin, Michael Rothe, Jack W Rip, Mona Abaoui, Murtaza S Nagree, Shaalee Dworski, Axel Schambach, Armand Keating, Michael L West, John Klassen, Patricia V Turner, Sandra Sirrs, C Anthony Rupar, Christiane Auray-Blais, Ronan Foley, Jeffrey A Medin
Fabry disease is a rare lysosomal storage disorder (LSD). We designed multiple recombinant lentivirus vectors (LVs) and tested their ability to engineer expression of human α-galactosidase A (α-gal A) in transduced Fabry patient CD34(+) hematopoietic cells. We further investigated the safety and efficacy of a clinically directed vector, LV/AGA, in both ex vivo cell culture studies and animal models. Fabry mice transplanted with LV/AGA-transduced hematopoietic cells demonstrated α-gal A activity increases and lipid reductions in multiple tissues at 6 months after transplantation...
June 16, 2017: Molecular Therapy. Methods & Clinical Development
https://www.readbyqxmd.com/read/28599693/clinical-characteristics-and-outcome-of-budd-chiari-syndrome-at-a-tertiary-care-hospital-in-pakistan
#2
Om Parkash, Aysha Khan, Saeed Hamid
OBJECTIVE: To determine the clinical characteristics of Budd-Chiari syndrome (BCS), its causes and outcome at a tertiary care hospital. STUDY DESIGN: An observational study. PLACE AND DURATION OF STUDY: The Aga Khan University Hospital,Karachi, from 2004 to 2014. METHODOLOGY: Aretrospective analysis of data was conducted. Apredesigned questionnaire was filled from medical records of patients with BCS. Clinical features, etiology, management and outcome was noted from 2004 to 2014...
May 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28586614/frequency-indications-and-complications-of-pulmonary-artery-catheter-insertion-in-adult-open-heart-surgery-patients-of-a-tertiary-care-hospital
#3
Syed Shabbir Ahmed, Mohammad Irfan Akhtar, Rehana Kamal
OBJECTIVE: Patients presenting for cardiac surgery have unstable cardiovascular disease and haemodynamics with multiple coexisting diseases. Optimal monitoring in the perioperative period is very important for best perioperative outcome. The introduction of the flow-directed pulmonary artery catheter (PAC) into clinical practice is one of the most important and popular advances in the field of cardiac anaesthesia. The objective of the study was to determine the frequency, indications and complications of pulmonary artery catheter insertion in adult open-heart surgery patients...
October 2016: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/28586578/electrophysiological-changes-in-patients-with-liver-cirrhosis-in-a-tertiary-care-hospital-in-karachi-pakistan
#4
Om Parkash, Ghulam Rehman Mohyuddin, Adil Ayub, Irfan Nazir, Arslan Arif Maan, Saeed Hamid
OBJECTIVE: Electrophysiological changes in cirrhosis are well known but least investigated especially in our country hence we wanted to see electrophysiological changes especially QT interval in cirrhotic patients. METHODS: A cross-sectional study was conducted at Aga Khan University Hospital Karachi (AKUH) in which medical records (duration 2008-2010) of cirrhotic patients were reviewed. RESULTS: Three hundred and eighty cirrhotic patients' charts were studied, 227 (59...
October 2016: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/28585580/stages-of-chronic-kidney-disease-and-soluble-transferrin-receptor-stfr-ferritin-ratio
#5
Faiza Alam, Syeda Sadia Fatima, Sabeela Noor, Ahmed Bilal, Rehana Rehman
OBJECTIVE: To assess the ratio of soluble transferrin receptor to ferritin in different stages of chronic kidney disease. METHODS: This case-control study was conducted at the Aga Khan University Hospital and the Jinnah Postgraduate Medical Centre, Karachi, from January to September 2014, and comprised chronic kidney disease patients and healthy controls. Group 1 comprised controls, whereas groups 2, 3 and 4 had patients based on their mean glomerular filtration rate...
June 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28567411/mobile-based-blended-learning-for-capacity-building-of-health-providers-in-rural-afghanistan
#6
Syeda Nateela Tirmizi, Shariq Khoja, Scott Patten, Abdul Wahab Yousafzai, Richard E Scott, Hammad Durrani, Wafa Khoja, Nida Husyin
BACKGROUND: Mobile-based blended learning initiative was launched in November 2014 in Badakshan province of Afghanistan by Tech4Life Enterprises, Aga Khan Health Service, Afghanistan (AKHS, A), and the University of Calgary, Canada. The goal of this initiative was to improve knowledge of health providers related to four major mental health problems, namely depression, psychosis, post-traumatic stress disorder (PTSD) and drug abuse. METHODS: This paper presents the results of quasi-experimental study conducted in 4 intervention districts in Badakshan for improvement in the knowledge among health providers about depression...
2017: MHealth
https://www.readbyqxmd.com/read/28567407/roshan-s-telemedicine-expanding-the-frontier-of-quality-healthcare-through-mobile-technology
#7
Shainoor Khoja
The article highlights the power of mobile technology in expanding access to quality healthcare by telling the story of the first successful operation by Afghan doctors to separate conjoined twins at The French Medical Institute for Mothers and Children (FMIC) in Kabul, Afghanistan's capital. Roshan, Afghanistan's leading telecommunications provider, set up the telemedicine link in 2007, connecting three hospitals in Kandahar, Bamiyan and Badakhshan Provinces to FMIC in Kabul, and all three to one of the best and most modern hospitals in the region: The Aga Khan University Hospital in Karachi, Pakistan...
2017: MHealth
https://www.readbyqxmd.com/read/28559519/phenotypic-low-level-isoniazid-resistance-as-a-marker-to-predict-ethionamide-resistance-in-mycobacterium-tuberculosis
#8
Salima Qamar, Joveria Q Farooqi, Kausar Jabeen, Rumina Hasan
BACKGROUND: Tuberculosis is one of the most prevalent diseases in Pakistan. Pakistan has the highest burden of MDR-TB in the Eastern Mediterranean region. Ethionamide is an anti-tuberculous drug frequently used to treat MDR-TB. Its drug susceptibility testing is not easily available in resource limited settings. Since it acts on the same target protein as isoniazid (inhA protein encoded by inhA gene), we sought to find out if phenotypic isoniazid resistance can be a marker of ethionamide resistance...
April 2017: International Journal of Mycobacteriology
https://www.readbyqxmd.com/read/28559510/rapid-detection-of-in-vitro-antituberculous-drug-resistance-among-smear-positive-respiratory-samples-using-microcolony-detection-based-direct-drug-susceptibility-testing-method
#9
Irim Iftikhar, Seema Irfan, Joveria Farooqi, Zahida Azizullah, Rumina Hasan
BACKGROUND: With the rise in multidrug-resistant tuberculosis, there is a search for newer techniques that will rapidly detect drug-resistant Mycobacterium tuberculosis. Although molecular techniques can detect resistance, culture is still considered gold standard, especially in resource-limited settings where quick, cheap, and easy techniques are needed. The aim of the study was to evaluate microcolony method thin layer agar (TLA) for quick detection of resistance against the first- and second-line antituberculous drugs in clinical isolates...
April 2017: International Journal of Mycobacteriology
https://www.readbyqxmd.com/read/28551388/anaplastic-lymphoma-kinase-protein-positive-diffuse-large-b-cell-lymphoma-a-developing-world-experience
#10
Huzaifah Salat, Nasir Ud Din, Tariq Moatter, Naila Kayani, Arsalan Ahmed
Anaplastic lymphoma kinase (ALK) positive diffuse large B-cell lymphoma (ALK+DLBCL) is a rare, distinct and aggressive subtype of non-Hodgkin's lymphoma (NHL). These tumors are considered to be derived from post-germinal center B cells but peculiarly their distinction is based on the fact that they are ALK-positive neoplastic B cells but lack expression of B cell markers (CD19,CD20, CD79a), T cell markers (CD3, CD5) and CD30. Its broad differential diagnosis and similarities to plasmablastic lymphoma, immunoblastic DLBCL, Anaplastic large-cell lymphoma (ALCL) of T-null cell lineage, and poorly differentiated/anaplastic carcinoma pose a grave challenge to physicians with conventional costly treatment for DLBCL failing to yield any clinical or prognostic significance in ALK+DLBCL...
June 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28550145/paediatric-innovation-in-pakistan-our-experience-and-a-call-to-action
#11
Walid Farooqi, Faysal Subhani, Asad Mian
Standardisation in paediatric medicine may have the unintended effect of stifling innovation. Thinking outside the box becomes even more important in low-income to middle-income countries like Pakistan, where a large paediatric population requires healthcare. In addition, there is always a lack of funds, making an innovative, low-cost and high impact solution all the more necessary. While regulation and formal research is an integral part of the process, the local synthesis of a solution must start with a creative idea...
May 26, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28545192/atypical-teratoid-rhabdoid-tumor-of-brain-a-clinicopathologic-study-of-eleven-patients-and-review-of-literature
#12
Nasir Ud Din, Abrar Barakzai, Aisha Memon, Sheema Hasan, Zubair Ahmad
Background: Atypical teratoid/ rhabdoid tumor (AT/RT) is a rare aggressive embryonal central nervous system (CNS) tumor of infancy and early childhood. Majority of the cases arise in the posterior fossa, and remaining in the cerebrum. Aims: To analyze the clinicopathologic features of AT/RT on a cohort of cases. Materials and methods: All reported cases of AT/RT at the Department of Pathology and Laboratory Medicine, Aga Khan University Hospital (AKUH) from 2007 to 2016 were reviewed for clinical and pathological features...
April 1, 2017: Asian Pacific Journal of Cancer Prevention: APJCP
https://www.readbyqxmd.com/read/28523050/frequency-of-color-blindness-in-pre-employment-screening-in-a-tertiary-health-care-center-in-pakistan
#13
Shaukat Ali Chhipa, Farzeen K Hashmi, Shehreen Ali, Mustafa Kamal, Khabir Ahmad
OBJECTIVE: To describe the frequency of color vision deficiency among Pakistani adults presenting for pre-employment health screening in a tertiary care hospital. METHODS: The cross-sectional study was carried out at the Aga Khan University Hospital, Karachi, and the data was collected for color vision deficiency, age, gender, and job applied for from pre-employment examination during 2013-2014. IBM SPSS 20 was used for statistical analysis. RESULTS: Three thousand four hundred and thirty seven persons underwent pre-employment screening during 2013 and 2014; 1837 (53...
March 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28508572/clinical-characteristics-and-factors-associated-with-mortality-in-idiopathic-pulmonary-fibrosis-an-experience-from-a-tertiary-care-center-in-pakistan
#14
Ali Bin Sarwar Zubairi, Huzaifa Ahmad, Maryam Hassan, Sajjad Sarwar, Aamir Abbas, Talha Shahzad, Muhammad Irfan
INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease (ILD) that predominantly affects older adults. IPF has the highest mortality burden of all ILDs. Data on mortality in patients with IPF is limited in developing countries. OBJECTIVES: To identify factors associated with mortality in patients with IPF at a tertiary care center in Pakistan. METHODS: A retrospective chart review was conducted at the Aga Khan University Hospital (AKUH) in Karachi, Pakistan from January 2005 to December 2015...
May 15, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28507349/antibiotics-in-acute-calculous-cholecystitis-do-tokyo-guidelines-influence-the-surgeons-practices
#15
Hassaan Bari, Muhammad Rizwan Khan, Amir Hafeez Shariff
OBJECTIVE: To observe changes in surgeons' practice of antibiotic usage in patients with acute cholecystitis before and after the implementation of Tokyo Guidelines. METHODS: This retrospective, descriptive study was conducted at the Aga Khan University Hospital, Karachi, and comprised the medical records of all patients with the diagnosis of acute calculus cholecystitis who presented in 2009 and those who presented in 2014 after the implementation of Tokyo Guidelines...
May 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28495809/comparison-of-enteral-versus-intravenous-potassium-supplementation-in-hypokalaemia-in-paediatric-patients-in-intensive-care-post-cardiac-surgery-open-label-randomised-equivalence-trial-eips
#16
Naveed Ur Rehman Siddiqu, Quratulain Merchant, Babar S Hasan, Arjumand Rizvi, Muneer Amanullah, Amina Rehmat, Anwarul Ul Haq
OBJECTIVES: The primary objective was to compare the efficacy of enteral potassium replacement (EPR) and intravenous potassium replacement (IVPR) as first-line therapy. Secondary objectives included comparison of adverse effects and number of doses required to resolve the episode of hypokalaemia. TRIAL DESIGN: The EIPS trial is designed as a randomised, equivalence trial between two treatment arms. STUDY SETTING: The study was conducted at the paediatric cardiac intensive care unit (PCICU) at Aga Khan University Hospital, Karachi...
May 10, 2017: BMJ Open
https://www.readbyqxmd.com/read/28492150/selective-screening-for-organic-acidurias-and-amino-acidopathies-in-pakistani-children
#17
Noreen Abbas Sherazi, Aysha Habib Khan, Lena Jafri, Azeema Jamil, Nasir Ali Khan, Bushra Afroze
OBJECTIVE: To determine the frequency of organic acidurais (OA) and amino acidopathies (AA) in selected high-risk patients screened in two years. STUDY DESIGN: Retrospective Observational study. PLACE AND DURATION OF STUDY: The Aga Khan University Hospital (AKUH), Karachi, from January 2013 to December 2014. METHODOLOGY: Patients with OA and AA were included in the study and patients with IMDs other than OA and AA were excluded...
April 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28458949/low-cost-quality-initiatives-for-management-of-neurosurgical-patients-in-developing-nations-perspective-from-a-tertiary-care-centre-in-pakistan
#18
Hamid H Rai, Muhammad Waqas, Muhammad S Shamim
BACKGROUND: Targeting resources on cost-effective care strategies is pertinent for developing nations, specifically for already burdened specialties such as neurosurgery, where without state support and insurance companies, expenses are borne by the patient themselves. METHODS: This was a descriptive review of literary work published by the section of neurosurgery at Aga Khan University Hospital, Pakistan. We searched PubMed and CiNAHL databases to identify articles, which were then critically analyzed and discussed from a perspective of low-cost quality care...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28435575/outpatient-percutaneous-release-of-trigger-finger-a-cost-effective-and-safe-procedure
#19
Z Marij, Q Aurangzeb, H R Rizwan, R Haroon, M H Pervaiz
INTRODUCTION: Trigger finger is a common cause of pain and disability of the hand. Percutaneous release results in earlier functional recovery and patient satisfaction. This is a rapid and cost-effective method which saves a surgical procedure and results in better functional outcome. MATERIALS AND METHODS: This is a prospective observational study conducted on fifty-two fingers and thumbs in 52 patients treated from 1st July 2014 till 31st December 2014, in the Orthopaedic Section, Department of Surgery, Aga Khan University Hospital, Karachi, Pakistan...
March 2017: Malaysian Orthopaedic Journal
https://www.readbyqxmd.com/read/28431334/one-hundred-injured-patients-a-day-multicenter-emergency-room-surveillance-of-trauma-in-pakistan
#20
A A Hyder, S He, W Zafar, M U Mir, J A Razzak
OBJECTIVES: Injuries increasingly contribute to the global burden of disease in low- and middle-income countries. This study presents results from a large-scale surveillance study on injury from several urban emergency departments (EDs) in Pakistan. The objective is to document the burden of injuries that present to the healthcare system in Pakistan and to test the feasibility of an ED-based injury and trauma surveillance system. STUDY DESIGN: Cross-sectional study conducted using active surveillance approach...
July 2017: Public Health
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