Medical Specialties Commons^™

Therapeutic Phlebotomy Is Safe In Children With Sickle Cell Anaemia And Can Be Effective Treatment For Transfusional Iron Overload, B. Aygun, N. A. Mortier, K. Kesler, A. Lockhart, W. H. Schultz, A. R. Cohen, O. Alvarez, Z. R. Rogers, J. L. Kwiatkowski, R. E. Ware, +4 Additional Authors

Journal Articles

Serial phlebotomy was performed on sixty children with sickle cell anaemia, stroke and transfusional iron overload randomized to hydroxycarbamide in the Stroke With Transfusions Changing to Hydroxyurea trial. There were 927 phlebotomy procedures with only 33 adverse events, all of which were grade 2. Among 23 children completing 30 months of study treatment, the net iron balance was favourable (-8.7 mg Fe/kg) with significant decrease in ferritin, although liver iron concentration remained unchanged. Therapeutic phlebotomy was safe and well-tolerated, with net iron removal in most children who completed 30 months of protocol-directed treatment.

Recurrent Gain Of Function Mutation In Calcium Channel Cacna1h Causes Early-Onset Hypertension With Primary Aldosteronism, U. I. Scholl, G. Stolting, C. Nelson-Williams, A. A. Vichot, M. Choi, E. Loring, M. L. Prasad, G. Goh, C. B. Sethna, R. P. Lifton, +11 Additional Authors

Journal Articles

Many Mendelian traits are likely unrecognized owing to absence of traditional segregation patterns in families due to causation by de novo mutations, incomplete penetrance, and/or variable expressivity. Genome-level sequencing can overcome these complications. Extreme childhood phenotypes are promising candidates for new Mendelian traits. One example is early onset hypertension, a rare form of a global cause of morbidity and mortality. We performed exome sequencing of 40 unrelated subjects with hypertension due to primary aldosteronism by age 10. Five subjects (12.5%) shared the identical, previously unidentified, heterozygous CACNA1H(M1549V) mutation. Two mutations were demonstrated to be de novo events, and all mutations …

Safety And Clinical Activity Of Elosulfase Alfa In Pediatric Patients With Morquio A Syndrome (Mucopolysaccharidosis Iva) Less Than 5 Years, S. A. Jones, M. Bialer, R. Parini, K. Martin, H. Wang, K. Yang, A. J. Shaywitz, P. Harmatz

Journal Articles

BACKGROUND: Previous studies have shown that elosulfase alfa has a favorable efficacy/safety profile in Morquio A patients aged >/=5 years. This study evaluated safety and impact on urine keratan sulfate (uKS) levels and growth velocity in younger patients. METHODS: Fifteen Morquio A patients aged/kg/week for 52 weeks during the primary treatment phase of a phase II, open-label, multinational study. Primary endpoint was safety and tolerability, secondary endpoints were change in uKS and growth velocity over 52 weeks. RESULTS: All 15 patients completed the primary treatment phase. Six of 743 infusions (0.8%) administered led to adverse events (AEs) requiring infusion interruption …

Tat-Hsp70 Attenuates Experimental Lung Injury, M. M. Lyons, N. N. Raj, J. L. Chittams, L. Kilpatrick, C. S. Deutschman

Journal Articles

Sepsis, a poorly understood syndrome of disordered inflammation, is the leading cause of death in critically ill patients. Lung injury, in the form of acute respiratory distress syndrome (ARDS), is the most common form of organ injury in sepsis. The heat shock response, during which heat shock proteins (HSPs) are expressed, is an endogenous mechanism to protect cells from injury. We have found that the abundance of pulmonary HSP70 is not increased after cecal ligation and double puncture (CLP) in a rat model of sepsis-induced ARDS. Using the HIV-1 trans-activator of transcription (TAT) cell-penetrating protein, we enhanced HSP70 protein abundance …

A Self-Paced Oral Feeding System That Enhances Preterm Infants' Oral Feeding Skills, C. Lau, S. Fucile, R. J. Schanler

Journal Articles

AIM: Very low birth weight (VLBW) infants have difficulty transitioning to independent oral feeding, be they breast- or bottle-feeding. We developed a 'self-paced' feeding system that eliminates the natural presence of the positive hydrostatic pressure and internal vacuum build-up within a bottle during feeding. Such system enhanced these infants' oral feeding performance as monitored by overall transfer (OT; % ml taken/ml prescribed), rate of transfer (RT; ml/min over an entire feeding). This study hypothesizes that the improvements observed in these infants resulted from their ability to use more mature oral feeding skills (OFS). METHODS: 'Feeders and growers' born between 26-29 …

Therapeutic Hypothermia After Out-Of-Hospital Cardiac Arrest In Children, F. W. Moler, F. S. Silverstein, R. Holubkov, B. S. Slomine, J. R. Christensen, V. M. Nadkarni, K. L. Meert, C. L. Schleien, J. M. Dean, +35 Additional Authors

Journal Articles

BACKGROUND: Therapeutic hypothermia is recommended for comatose adults after witnessed out-of-hospital cardiac arrest, but data about this intervention in children are limited. METHODS: We conducted this trial of two targeted temperature interventions at 38 children's hospitals involving children who remained unconscious after out-of-hospital cardiac arrest. Within 6 hours after the return of circulation, comatose patients who were older than 2 days and younger than 18 years of age were randomly assigned to therapeutic hypothermia (target temperature, 33.0 degrees C) or therapeutic normothermia (target temperature, 36.8 degrees C). The primary efficacy outcome, survival at 12 months after cardiac arrest with a …

Use Of Serogroup B Meningococcal Vaccines In Persons Aged >= 10 Years At Increased Risk For Serogroup B Meningococcal Disease: Recommendations Of The Advisory Committee On Immunization Practices, 2015, T. Folaranmi, L. Rubin, S. W. Martin, M. Patel, J. R. Macneil

Journal Articles

No abstract provided.

Yellow Fever Vaccine Booster Doses: Recommendations Of The Advisory Committee On Immunization Practices, 2015, J. E. Staples, J. A. Bocchini, L. Rubin, M. Fischer

Journal Articles

No abstract provided.

Temperature Management Guidelines, J. W. Hammon, L. Shore-Lesserson, T. A. Dickinson

Journal Articles

No abstract provided.

The Society Of Thoracic Surgeons, The Society Of Cardiovascular Anesthesiologists, And The American Society Of Extracorporeal Technology: Clinical Practice Guidelines For Cardiopulmonary Bypass-Temperature Management During Cardiopulmonary Bypass, R. Engelman, R. A. Baker, D. S. Likosky, A. Grigore, T. A. Dickinson, L. Shore-Lesserson, J. W. Hammon

Journal Articles

To improve our understanding of the evidence-based literature supporting temperaturemanagement during adult cardiopulmonary bypass, The Society of Thoracic Surgeons, the Society of Cardiovascular Anesthesiology and the American Society of ExtraCorporeal Technology tasked the authors to conduct a review of the peer-reviewed literature, including 1) optimal site for temperature monitoring, 2) avoidance of hyperthermia, 3) peak cooling temperature gradient and cooling rate, and 4) peak warming temperature gradient and rewarming rate. Authors adopted the American College of Cardiology/American Heart Association method for development clinical practice guidelines, and arrived at the following recommendation.

Gentamicin Differentially Alters Cellular Metabolism Of Cochlear Hair Cells As Revealed By Nad(P)H Fluorescence Lifetime Imaging, Lyandysha V. Zholudeva, Kristina G. Ward, Michael G. Nichols, Heather Jensen Smith

Journal Articles: Eppley Institute

Aminoglycoside antibiotics are implicated as culprits of hearing loss in more than 120,000 individuals annually. Research has shown that the sensory cells, but not supporting cells, of the cochlea are readily damaged and/or lost after use of such antibiotics. High-frequency outer hair cells (OHCs) show a greater sensitivity to antibiotics than high- and low-frequency inner hair cells (IHCs). We hypothesize that variations in mitochondrial metabolism account for differences in susceptibility. Fluorescence lifetime microscopy was used to quantify changes in NAD(P)H in sensory and supporting cells from explanted murine cochleae exposed to mitochondrial uncouplers, inhibitors, and an ototoxic antibiotic, gentamicin (GM). …

Update On Uveitis Management., Vishali Gupta, Quan Dong Nguyen, Manfred Zierhut, Ilknur Tugal-Tutkun

Journal Articles: Ophthalmology

No abstract provided.

Geriatric Telemedicine: Background And Evidence For Telemedicine As A Way To Address The Challenges Of Geriatrics, Ronald C. Merrell

Surgery Publications

Objectives

The global population of elderly people is increasing at a remarkable rate, which may be expected to continue for some time. Older patients require more care, and with the current model of care delivery, the costs may be expected to rise, although higher cost is unsustainable. For this reason, a new pattern of practice is needed. Telemedicine will be presented as a highly effective and necessary tool in geriatrics.

Methods

This review will present some of the background and evidence for telemedicine as a way to address the challenges of geriatrics through geriatric telemedicine. Some of the evidence for …

Addition Of Interleukin-21 For Expansion Of T-Cells For Adoptive Immunotherapy Of Murine Melanoma, Christine Kathryn Zoon, Wen Wan, Laura Graham, Harry D. Bear

Surgery Publications

We previously demonstrated that interleukin (IL)-7/15 was superior to IL-2 for expansion of T cells in vitro for adoptive immunotherapy. We sought to ascertain whether IL-21 would further improve yield and therapeutic efficacy of T cells in culture. Naïve T cell receptor (TcR) transgenic splenocytes or antigen-sensitized lymph node cells were harvested from PMEL-1 mice and exposed to bryostatin-1 and ionomycin (B/I) for 18 h. Cells were then cultured in IL-2, IL-21, IL-7/15 or IL-7/15/21 for six days. Harvested cells were analyzed by flow cytometry and used to treat C57Bl/6 mice injected intravenously with B16 melanoma. Lungs were harvested and …

An Unusual Case Of Bony Styloid Processes That Extend To The Hyoid Bone, Shekhar K. Gadkaree, Christopher G. Hyppolite, Aisha Harun, Ryan H. Sobel, Young Kim

Surgery Publications

The embryological origin of the hyoid bone is a point of uncertainty, with controversy surrounding the relative contribution of the second pharyngeal arch to hyoid development. We encountered a 52-year-old male with bilateral bony styloid extension to the lesser cornu of the hyoid bone during the workup of a patient with laryngeal cancer. This embryological malformation clearly supports the hypothesis that the second pharyngeal arch gives rise to the lesser cornu and demonstrates an unusual clinical finding that may be encountered by otolaryngologists. We demonstrate the imaging findings and surgical management of this unusual anatomical variant and review the embryological …

Penetrating Cardiac Trauma And The Use Of Emergent Extracorporeal Membrane Oxygenation And Therapeutic Hypothermia: When Cooler Heads Prevail, Nathaniel Lee, Daniel Tang, Sudha Jayaraman

Surgery Publications

This is a case of penetrating chest trauma with traumatic arrest from cardiac tamponade, right and left ventriculotomies, mitral valve injury and ventricular septal defect. Patient underwent resuscitative thoracotomy converted to clamshell thoracotomy for haemorrhage control. Ventriculotomies were repaired on initial damage control operation. Extracorporeal membrane oxygenation cannulation and therapeutic cooling bridged to definitive cardiac repair several days later. Patient was discharged to an inpatient rehabilitation facility in 16 days.

Wses Guidelines For Management Of Clostridium Difficile Infection In Surgical Patients, Massimo Sartelli, Mark A. Malangoni, Fikri M. Abu-Zidan, Ewen A. Griffiths, Stefano Di Bella, Lynne V. Mcfarland, Ian Eltringham, Vishal G. Shelat, George C. Velmahos, Ciarán P. Kelly, Sahil Khanna, Zaid M. Abdelsattar, Layan Alrahmani, Luca Ansaloni, Goran Augustin, Miklosh Bala, Frédéric Barbut, Offir Ben-Ishay, Aneel Bhangu, Walter L. Biffl, Stephen M. Brecher, Adrián Camacho-Ortiz, Miguel A. Caínzos, Laura A. Canterbury, Fausto Catena, Shirley Chan, Jill R. Cherry-Bukowiec, Jesse Clanton, Federico Coccolini, Maria Elena Cocuz, Raul Coimbra, Charles H. Cook, Yunfeng Cui, Jacek Czepiel, Koray Das, Zaza Demetrashvili, Isidoro Di Carlo, Salomone Di Saverio, Irina Magdalena Dumitru, Catherine Eckert, Christian Eckmann, Edward H. Eiland, Mushira Abdulaziz Enani, Mario Faro, Paula Ferrada, Joseph Derek Forrester, Gustavo P. Fraga, Jean Louis Frossard, Rita Galeiras, Wagih Ghnnam, Carlos Augusto Gomes, Venkata Gorrepati, Mohamed Hassan Ahmed, Torsten Herzog, Felicia Humphrey, Jae Il Kim, Arda Isik, Rao Ivatury, Yeong Yeh Lee, Paul Juang, Luis Furuya-Kanamori, Aleksandar Karamarkovic, Peter K. Kim, Yoram Kluger, Wen Chien Ko, Francis D. Labarbera, Jae Gil Lee, Ari Leppaniemi, Varut Lohsiriwat, Sanjay Marwah, John E. Mazuski, Gokhan Metan, Ernest E. Moore, Frederick Alan Moore, Carl Erik Nord, Carlos A. Ordoñez, Gerson Alves Pereira Júnior, Nicola Petrosillo, Francisco Portella, Basant K. Puri, Arnab Ray, Mansoor Raza, Miran Rems, Boris E. Sakakushev, Gabriele Sganga, Patrizia Spigaglia, David B. Stewart, Pierre Tattevin, Jean Francois Timsit, Kathleen B. To, Cristian Tranà, Waldemar Uhl, Libor Urbánek, Harry Van Goor, Angela Vassallo, Jean Ralph Zahar, Emanuele Caproli, Pierluigi Viale

Surgery Publications

In the last two decades there have been dramatic changes in the epidemiology of Clostridium difficileinfection (CDI), with increases in incidence and severity of disease in many countries worldwide. The incidence of CDI has also increased in surgical patients. Optimization of management of C difficile, has therefore become increasingly urgent. An international multidisciplinary panel of experts prepared evidenced-based World Society of Emergency Surgery (WSES) guidelines for management of CDI in surgical patients.

Quality Control Material For The Detection Of Somatic Mutations In Fixed Clinical Specimens By Next-Generation Sequencing, Catherine I. Dumur, Jorge A. Almenara, Celeste N. Powers, Andrea Ferreira-Gonzalez

Pathology Publications

Background: Targeted next generation sequencing (NGS) technology to assess the mutational status of multiple genes on formalin-fixed, paraffin embedded (FFPE) tumors is rapidly being adopted in clinical settings, where quality control (QC) practices are required. Establishing reliable FFPE QC materials for NGS can be challenging and/or expensive. Here, we established a reliable and cost-effective FFPE QC material for routine utilization in the Ion AmpliSeq™ Cancer Hotspot Panel v2 (CHP2) assay.

Methods: The performance characteristics of the CHP2 assay were determined by sequencing various cell line mixtures and 55 different FFPE tumors on the Ion Torrent PGM platform. A FFPE QC …

Nosocomial Keratitis Caused By Methicillin-Resistant Staphylococcus Aureus: Case Report And Preventative Measures, Puneet S. Braich, Shruti Aggarwal, Sabrina Mukhtar, David Rp Almeida

Ophthalmology Publications

A 47-year-old African-American woman was admitted to the intensive care unit of our community hospital for respiratory failure secondary to severe decompensated heart failure, requiring intubation. In the ensuing days, she developed a methicillin-resistant Staphylococcus aureus (MRSA) infection of the cornea, despite no growth of MRSA in multiple blood, sputum, and urine cultures. This unexpected corneal infection complicated her hospital stay, and increased morbidity and disease-related cost. Risk factors, warning signs, and preventative measures for MRSA keratitis secondary to lagophthalmos (inability to completely close one's eyelids) are outlined in this case report. Implementing simple precautions such as taping eyelids shut …

Relocation Consequences On An Ophthalmology Consultation Service From An Inpatient To Outpatient Facility, Jorawer S. Singh, Vincent M. Imbrogno, Mary K. Howard, Amandip S. Cheema, Ausra D. Selvadurai, Surbhi Bansal

Ophthalmology Publications

Importance

This study shows that relocation of an academic ophthalmology residency program from an inpatient to an outpatient setting in western New York does not affect the consult volume but affects management patterns and follow-up rates.

Objective

To investigate the effects on the ophthalmology consultation service of an academic program with relocation from a Regional Level-1 Trauma center to an outpatient facility.

Design

Consultation notes from 3 years before and 3 years after the University at Buffalo’s (UB) Department of Ophthalmology relocation from a Regional Level-1 Trauma center (Erie County Medical Center) to an outpatient facility (Ross Eye Institute) were …

Prevalence And Factors Predictive Of Intraocular Fungal Infection In Patients With Fungemia At An Academic Urban Tertiary Care Center, Elena Geraymovych, Joseph H. Conduff, Puneet S. Braich, Christopher T. Leffler, Vikram S. Brar

Ophthalmology Publications

Objective

To report the prevalence and to identify factors predictive of intraocular infection in patients with fungemia receiving prophylactic antifungal therapy.

Methods

A retrospective review of patients who received prophylactic antifungal therapy and a dilated fundus examination at an academic urban tertiary care center from 2000 to 2007. Basic demographic information, fungal species grown, antifungal agent(s) used, number of positive blood culture specimens, visual acuity, visual symptoms, and known risks of disseminated candidiasis were noted. Logistic regression analysis was used to determine the factors significantly associated with intraocular fungal infection.

Results

A total of 132 patients with positive fungemia culture …

Infrared Imaging Enhances Retinal Crystals In Bietti’S Crystalline Dystrophy, Vikram S. Brar, William H. Benson

Ophthalmology Publications

Infrared imaging dramatically increased the number of crystalline deposits visualized compared with clinical examination, standard color fundus photography, and red free imaging in patients with Bietti’s crystalline dystrophy. We believe that this imaging modality significantly improves the sensitivity with which these lesions are detected, facilitating earlier diagnosis and may potentially serve as a prognostic indicator when examined over time.

Disappearing Before My Eyes, Edward F. Ansello

Director's Editorials from Age in Action

Dr. Edward Ansello details the last memories he made and recalled with his mother, who struggled with memory loss in later life.

Preventing Financial Exploitation, Edward F. Ansello

Director's Editorials from Age in Action

Financial exploitation of older adults isn’t going away. We each need to be aware and proactive.

Remembering Our Friends, Edward F. Ansello

Director's Editorials from Age in Action

We remember three men whose caring for others was, indeed, exceptional: Bill Egelhoff, Benny Lambert, and Frank Hall.

Available Telephone Services For Older Virginians Who Are Deaf Or Hard Of Hearing, Clayton E. Bowen

Case Studies from Age in Action

Educational Objectives:

1. To identify signs of hearing loss in older adults.

2. To introduce the Virginia Department for the Deaf and Hard of Hearing (VDDHH) and some of its programs and services.

3. To examine three VDDHH services and qualification guidelines.

4. To review how service recipients benefit from these programs.

Sleep Disorders And Cardiac Consequences, Robert D. Vorona

Case Studies from Age in Action

Educational Objectives:

1. Review the clear relationship between obstructive sleep apnea and hypertension.

2. Discuss the cardiac consequences of sleep apnea, including coronary heart disease, heart failure, and different dysrhythmias.

3. Understand that sleep disorders beyond obstructive sleep apnea, such as insufficient sleep itself, may have an impact on the cardiovascular system.

4. Appreciate the potential benefits of identifying and treating sleep disorders for our health.

Bringing Comfort To People With Advanced Dementia, Ann Wyatt

Case Studies from Age in Action

Educational Objectives

1. Explain the purpose of looking at palliative care through a dementia lens.

2. Define dementia-capable palliative care.

3. Identify and provide examples of specific care practices that can bring comfort to people with advanced dementia.

4. Describe the process that staff use in identifying what brings comfort to individual residents.

Building Interprofessional Teams Through Evidence Based Practice Training In Falls Prevention, Constance L. Coogle, Edward F. Ansello, Patricia Slattum, Pamela L. Parsons

Case Studies from Age in Action

Objectives

1. Analyze an Evidence Based Practice (EBP) program on falls prevention as a vehicle for successful interprofessional team-building.

2. Examine ways that organizational characteristics can affect team functioning.

3. Compare interprofessional teambuilding in two sites.

4. Discuss elements of interprofessional teams that contributed to practice changes.

Mri Evaluation Of Lumbar Disc Degenerative Disease, Pokhraj Suthar, Rupal Patel, Chetan Mehta, Narrotam Patel

Orthopaedic Surgery Publications

Introduction:

Lower back pain secondary to degenerative disc disease is a condition that affects young to middle-aged persons with peak incidence at approximately 40 y. MRI is the standard imaging modality for detecting disc pathology due to its advantage of lack of radiation, multiplanar imaging capability, excellent spinal soft-tissue contrast and precise localization of intervertebral discs changes.

Aims and Objective:

To evaluate the characterization, extent, and changes associated with the degenerative lumbar disc disease by Magnetic Resonance Imaging.

Study Design:

Cross-sectional and observational study.

Materials and Methods:

A total 109 patients of the lumbar disc degeneration with age group between …