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https://www.readbyqxmd.com/read/27911282/role-of-rehabilitation-in-hurler-s-syndrome
#1
Sudhir Ramkishore Mishra, Mona Shastri, Jaishree Ramesh
Hurler syndrome is an inherited autosomal recessive disorder of lysosomal accumulation of un-degraded glucosaminoglycan secondary to deficiency of a-L-Iduronidase enzyme. It is most severe form of Mucopolysaccharidosis with incidence of 1:100 000. It has multisystemic involvement leading to multiple deformity, disability and death within 10th years of life. A 2 year old boy presented with umbilical hernia, gross developmental delay and a progressive spinal deformity. On detailed clinical, radiological and laboratory investigation he was diagnosed as Hurler's syndrome...
November 25, 2016: Journal of Back and Musculoskeletal Rehabilitation
https://www.readbyqxmd.com/read/27911246/role-of-follow-up-imaging-after-resection-of-brain-arteriovenous-malformations-in-pediatric-patients-a-systematic-review-of-the-literature
#2
Joaquin E Jimenez, Zachary C Gersey, Jason Wagner, Brian Snelling, Sudheer Ambekar, Eric C Peterson
OBJECTIVE Pediatric patients are at risk for the recurrence of brain arteriovenous malformation (AVM) after resection. While there is general consensus on the importance of follow-up after surgical removal of an AVM, there is a lack of consistency in the duration of that follow-up. The object of this systematic review was to examine the role of follow-up imaging in detecting AVM recurrence early and preventing AVM rupture. METHODS This systematic review was performed using articles obtained through a search of the literature contained in the MeSH database, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines...
December 2, 2016: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/27910252/memory-performance-on-the-story-recall-test-and-prediction-of-cognitive-dysfunction-progression-in-mild-cognitive-impairment-and-alzheimer-s-dementia
#3
Jong-Hwan Park, Hyuntae Park, Sang Wuk Sohn, Sungjae Kim, Kyung Won Park
AIM: To determine the factors that influence diagnosis and differentiation of patients with mild cognitive impairment (MCI) and Alzheimer's dementia (AD) by comparing memory test results at baseline with those at 1-2-year follow up. METHODS: We consecutively recruited 23 healthy participants, 44 MCI patients and 27 patients with very mild AD according to the National Institute of Neurological and Communicative Diseases and Stroke/Alzheimer's Disease and Related Disorder Association criteria for probable Alzheimer's disease and Petersen's clinical diagnostic criteria...
December 1, 2016: Geriatrics & Gerontology International
https://www.readbyqxmd.com/read/27910211/comparison-of-serum-concentrations-between-different-dosing-strategies-of-posaconazole-delayed-release-tablet-at-a-large-academic-medical-centre
#4
Sarah Welch, Andrea Pallotta, Catherine Weber, Caitlin Siebenaller, Eric Cober, Elizabeth Neuner
It is unclear if the prophylaxis dose of 300 mg/day is sufficient for achieving serum concentrations targeting the treatment of invasive fungal infections. To evaluate differences between PCZ serum concentrations in patients receiving the DRT vs the OS and in patients receiving higher doses than 300 mg/day of the DRT, a retrospective review was conducted on inpatients who received PCZ for either treatment or prophylaxis. Baseline demographics including comorbid conditions, indication and dose of therapy were collected...
December 1, 2016: Mycoses
https://www.readbyqxmd.com/read/27910089/bilateral-endolymphatic-hydrops-in-a-patient-with-migraine-variant-without-vertigo-a-case-report
#5
Isabelle Y Liu, Akira Ishiyama, Ali R Sepahdari, Kevin Johnson, Gail Ishiyama
OBJECTIVE: To use modern high-resolution inner ear imaging modalities to evaluate for endolymphatic hydrops (EH) in a patient with migraine-associated fluctuating hearing loss without vertigo spells or dizziness. BACKGROUND: EH has been well described in patients with Meniere's disease on both human temporal bone studies and modern high-resolution imaging; however, there is no study to date, to our knowledge, that examines the presence of EH in a patient with migraine and bilateral hearing loss...
December 2, 2016: Headache
https://www.readbyqxmd.com/read/27909627/recurrence-of-childhood-nephrogenic-adenoma-in-urinary-bladder-developed-four-years-after-previous-surgery-despite-intravesical-sodium-hyaluronate-therapy
#6
Burak Özçift, Ayper Kaçar, Hüseyin Tuğrul Tiryaki
Nephrogenic adenoma (NA) is a rarely seen benign metaplastic lesion of the urinary tract. Its etiology is uncertain, but induced by chronic inflammation, irritation, and trauma. NA is located in the urinary tract, most commonly in the bladder. NA usually presents with hematuria and lower urinary tract symptoms. In the literature it is mostly seen in adults but about 30 cases of NA's have been reported in children. Treatment of intravesical lesions consists of transurethral resection (TUR) and fulguration and rarely partial or total cystectomy may be required in ineffective TUR...
December 2016: Turkish Journal of Urology
https://www.readbyqxmd.com/read/27909552/vonoprazan-20-mg-vs-lansoprazole-30-mg-for-endoscopic-submucosal-dissection-induced-gastric-ulcers
#7
Kazuya Takahashi, Yuichi Sato, Junji Kohisa, Jun Watanabe, Hiroki Sato, Kenichi Mizuno, Satoru Hashimoto, Shuji Terai
AIM: To compare the healing effects of vonoprazan and lansoprazole on gastric ulcers induced by endoscopic submucosal dissection (ESD). METHODS: Data were obtained from a total of 26 patients. Fourteen patients were randomized to the vonoprazan group and 12 were randomized to the lansoprazole group. Patients were administered either 20 mg vonoprazan or 30 mg lansoprazole per day after ESD. Endoscopic images just after ESD, on day 8, and on day 28 were used for the evaluation of the shrinking rate of ESD ulcers...
November 16, 2016: World Journal of Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/27909526/achieving-bidirectional-long-delays-in-pulmonary-vein-antral-lines-prior-to-bidirectional-block-in-patients-with-paroxysmal-atrial-fibrillation-the-bi-bi-technique-for-atrial-fibrillation-ablation
#8
Adel F Mina Md Facc Fhrs, Nicholas L Warnecke Pa-C
Background: Pulmonary Vein Antral isolation (PVAI) is currently the standard of care for both paroxysmal and persistent atrial fibrillation ablation. Reconnection to the pulmonary vein is the most common cause of recurrence of atrial fibrillation. Achieving the endpoint of bidirectional block (BDB) for cavotricuspid isthmus dependant flutter has improved our outcomes for atrial flutter ablation. With this we tried to achieve long delays in the pulmonary veins antral lines prior to complete isolation comparable to those delays found in patient with bidirectional block of atrial flutter lines...
August 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27909481/ice-guided-crt-is-there-evidence-of-reverse-remodeling
#9
REVIEW
Antonio Rossillo Md, Angelo B Ramondo Md
Cardiac resynchronization therapy (CRT) is an accepted treatment for patients with heart failure (HF), impaired left ventricular (LV) function, and a wide QRS complex. However, more than 30% of eligible patients fail to benefit from CRT. It is clearly necessary to define the characteristics of the best candidates for this therapy. To this end, surface ECG and echocardiography have been tested. Unfortunately, however, neither of these examinations has proved sufficiently able to identify the best patients. A tailored approach based on the evaluation of both electrical and mechanical delay to guide LV lead placement seems to be the most reasonable strategy in order to increase the efficacy of CRT therapy...
February 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27909469/value-of-the-wearable-cardioverter-defibrillator-wcd-as-a-bridging-therapy-before-implantation-of-a-cardioverter-defibrillator-icd
#10
REVIEW
Priv- Doz, Dr Johannes Sperzel
Wearable cardioverter defibrillators (WCD), initially available in 2002, have recently experienced more routine use in many institutions as a means of preventing sudden cardiac death (SCD) prior to implantable cardioverter defibrillator (ICD) evaluation or implantation. WCD differ from ICD by their noninvasive nature, making them well suited for patient populations who have a chance for significant cardiac recovery (such as after an acute myocardial infarction). Despite their noninvasive nature, WCD treatment of sustained ventricular tachyarrhythmias is highly successful...
February 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27909435/immune-reconstitution-after-allogeneic-hematopoietic-stem-cell-transplantation
#11
REVIEW
Justyna Ogonek, Mateja Kralj Juric, Sakhila Ghimire, Pavankumar Reddy Varanasi, Ernst Holler, Hildegard Greinix, Eva Weissinger
The timely reconstitution and regain of function of a donor-derived immune system is of utmost importance for the recovery and long-term survival of patients after allogeneic hematopoietic stem cell transplantation (HSCT). Of note, new developments such as umbilical cord blood or haploidentical grafts were associated with prolonged immunodeficiency due to delayed immune reconstitution, raising the need for better understanding and enhancing the process of immune reconstitution and finding strategies to further optimize these transplant procedures...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27908962/pediatric-traumatic-gallbladder-rupture
#12
W Landon Jackson, Patrick C Bonasso, R Todd Maxson
Blunt abdominal trauma causing gallbladder rupture is exceptionally rare in children. This injury is rare due to the size and anatomical location of the gallbladder, and a rapid diagnosis is often difficult to achieve due to delayed presentation. We report a ruptured gallbladder in an 11-year-old male as a result of blunt abdominal trauma from an all-terrain vehicle accident. Possible gallbladder injury was indicated on computed tomography and patient was successfully managed with cholecystectomy.
December 1, 2016: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/27908910/hospital-variation-in-time-to-epinephrine-for-non-shockable-in-hospital-cardiac-arrest
#13
Rohan Khera, Paul S Chan, Michael W Donnino, Saket Girotra
BACKGROUND: -For patients with in-hospital cardiac arrests due to non-shockable rhythms, delays in epinephrine administration beyond 5 minutes is associated with worse survival. However, the extent of hospital variation in delayed epinephrine administration and its impact on hospital-level outcomes is unknown. METHODS: -Within Get with the Guidelines-Resuscitation, we identified 103,932 adult patients (≥18 years) at 548 hospitals with an in-hospital cardiac arrest due to a non-shockable rhythm who received at least 1 dose of epinephrine between 2000 to 2014...
December 1, 2016: Circulation
https://www.readbyqxmd.com/read/27908531/chemotherapy-delay-after-primary-debulking-surgery-for-ovarian-cancer
#14
Brandon-Luke L Seagle, Sharlay K Butler, Anna E Strohl, Wilberto Nieves-Neira, Shohreh Shahabi
OBJECTIVE: To determine the association of chemotherapy delay with overall survival (OS) and investigate predictors of delay among a population-representative American ovarian cancer cohort. METHODS: An observational retrospective cohort analysis of women with ovarian cancer who received National Comprehensive Cancer Network guideline-consistent care was performed with the 1998-2011 National Cancer Data Base. Chemotherapy delay was defined as initiation of multiagent chemotherapy >28days from primary debulking surgery...
November 28, 2016: Gynecologic Oncology
https://www.readbyqxmd.com/read/27908336/appropriate-antibiotic-therapy
#15
REVIEW
Michael G Allison, Emily L Heil, Bryan D Hayes
Prescribing antibiotics is an essential component of initial therapy in sepsis. Early antibiotics are an important component of therapy, but speed of administration should not overshadow the patient-specific characteristics that determine the optimal breadth of antimicrobial therapy. Cultures should be drawn before antibiotic therapy if it does not significantly delay administration. Combination antibiotic therapy against gram-negative infections is not routinely required, and combination therapy involving vancomycin and piperacillin/tazobactam is associated with an increase in acute kidney injury...
February 2017: Emergency Medicine Clinics of North America
https://www.readbyqxmd.com/read/27908292/conservatively-treated-congenital-hyperinsulinism-chi-due-to-k-atp-channel-gene-mutations-reducing-severity-over-time
#16
Maria Salomon-Estebanez, Sarah E Flanagan, Sian Ellard, Lindsey Rigby, Louise Bowden, Zainab Mohamed, Jacqueline Nicholson, Mars Skae, Caroline Hall, Ross Craigie, Raja Padidela, Nuala Murphy, Tabitha Randell, Karen E Cosgrove, Mark J Dunne, Indraneel Banerjee
BACKGROUND: Patients with Congenital Hyperinsulinism (CHI) due to mutations in K-ATP channel genes (K-ATP CHI) are increasingly treated by conservative medical therapy without pancreatic surgery. However, the natural history of medically treated K-ATP CHI has not been described; it is unclear if the severity of recessively and dominantly inherited K-ATP CHI reduces over time. We aimed to review variation in severity and outcomes in patients with K-ATP CHI treated by medical therapy. METHODS: Twenty-one consecutively presenting patients with K-ATP CHI with dominantly and recessively inherited mutations in ABCC8/KCNJ11 were selected in a specialised CHI treatment centre to review treatment outcomes...
December 1, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27907937/tranexamic-acid-for-trauma-resuscitation-in-the-united-states-of-america
#17
Mark Walsh, Scott Thomas, Ernest Moore, Hunter Moore, Andres Piscoya, Daniel Hake, Michael Son, Tim Pohlman, Julie Wegner, John Bryant, Alberto Grassetto, Patrick Davis, Nathan Nielsen, Anton Crepinsek, Jacob T Shreve, Francis Castellino
The utilization of tranexamic acid (TXA) for the management of bleeding trauma patients has been a subject of much debate on both sides of the Atlantic and in Australia. As a result of the large randomized controlled study called the Clinical Randomization of an Antifibrinolytic in Severe Hemorrhage (CRASH-2), there was an initial enthusiasm for the use of TXA to treat bleeding patients. However, the adoption of TXA in the United States was delayed by concerns of "knowledge and evidence gaps" of the CRASH-2 study and because of a lack of mechanistic rationale that would explain the survival benefit noted in the study...
December 1, 2016: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/27907230/reverse-geroscience-how-does-exposure-to-early-diseases-accelerate-the-age-related-decline-in-health
#18
Ronald A Kohanski, Steven G Deeks, Claudia Gravekamp, Jeffrey B Halter, Kevin High, Arti Hurria, Rebecca Fuldner, Paige Green, Robin Huebner, Francesca Macchiarini, Felipe Sierra
Aging is the major risk factor for both the development of chronic diseases and loss of functional capacity. Geroscience provides links among the biology of aging, the biology of disease, and the physiology of frailty, three fields where enormous progress has been made in the last few decades. While, previously, the focus was on the role of aging in susceptibility to disease and disability, the other side of this relationship, which is the contribution of disease to aging, has been less explored at the molecular/cellular level...
December 1, 2016: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/27907128/fasa-registry-on-acute-myocardial-infarction-farmi-feasibility-study-and-pilot-phase-results
#19
Ehsan Bahramali, Alireza Askari, Habib Zakeri, Mojtaba Farjam, Azizallah Dehghan, Kazem Zendehdel
BACKGROUND: Myocardial infarction (MI) is the leading cause of death in Iran. Every attempt to improve treatment patterns and patient outcomes needs a surveillance system to both consider the efficacy and safety measures. Fasa Registry on Myocardial Infarction (FaRMI) is the first population-based registry for acute MI in Iran targeted to provide meticulous description of patients' characteristics, to explore the management patterns of these patients, to discover the degree of adherence to the practice guidelines, and to investigate the determinants of poor in-hospital and later outcomes...
2016: PloS One
https://www.readbyqxmd.com/read/27906936/analgesic-effect-of-nalbuphine-when-added-to-intravenous-regional-anesthesia-a-randomized-control-trial
#20
Mohamed Hassan Bakri, Eman Ahmed Ismail, Sayed Kaoud Abd-Elshafy
BACKGROUND: Different adjuvant drugs are currently added to lidocaine for intravenous regional anesthesia (IVRA) to decrease tourniquet and postoperative pain. OBJECTIVE: The aim of the study was to examine the effect of nalbuphine when added toIVRA. STUDY DESIGN: Prospective, randomized, double-blind, controlled clinical trial. SETTING: Assiut University Hospitals. METHODS: One hundred-six adult patients scheduled for unilateral hand surgery under IVRA were randomized into 2 equal groups...
November 2016: Pain Physician
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