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Binit Sureka, Aliza Mittal, Mahesh K Mittal, Kanhaiya Agarwal, Mukul Sinha, Brij Bhushan Thukral
Background: Accurate and detailed measurements of spinal canal diameter (SCD) and transverse foraminal morphometry are essential for understanding spinal column-related diseases and for surgical planning, especially for transpedicular screw fixation. This is especially because lateral cervical radiographs do not provide accurate measurements. Aim: This study was conducted to measure the dimensions of the transverse foramen sagittal and transverse diameters (SFD, TFD), SCD, and the distance of spinal canal from the transverse foramina (dSC-TF) at C1-C7 level in the Indian population...
March 2018: Neurology India
Ruchita Dixit, Sowmya Nettem, Simerjit S Madan, Htoo Htoo Kyaw Soe, Adinegara Bl Abas, Leah D Vance, Patrick J Stover
BACKGROUND: Sickle cell disease (SCD) is a group of disorders that affects haemoglobin, which causes distorted sickle- or crescent-shaped red blood cells. It is characterized by anaemia, increased susceptibility to infections and episodes of pain. The disease is acquired by inheriting abnormal genes from both parents, the combination giving rise to different forms of the disease. Due to increased erythropoiesis in people with SCD, it is hypothesized that they are at an increased risk for folate deficiency...
March 16, 2018: Cochrane Database of Systematic Reviews
Susanne Röger, Stefanie L Rosenkaimer, Anna Hohneck, Siegfried Lang, Ibrahim El-Battrawy, Boris Rudic, Erol Tülümen, Ksenija Stach, Jürgen Kuschyk, Ibrahim Akin, Martin Borggrefe
BACKGROUND: The wearable cardioverter-defibrillator (WCD) has emerged as a valuable tool to temporarily protect patients at risk for sudden cardiac death (SCD). The aim of this study was to determine the value of the WCD for therapy optimization of heart failure patients. METHODS: One hundred five consecutive patients that received WCD between 4/2012 and 9/2016 were included in the study. All patients were followed for clinical outcome and echocardiographic parameters during WCD therapy and had continued follow-up after WCD therapy, irrespective of subsequent implantable cardioverter-defibrillator (ICD) implantation...
March 15, 2018: BMC Cardiovascular Disorders
Jinyu Wang, Haitao Zhang, Mohammed Al Shibar, Belinda Willard, Alo Ray, Kurt W Runge
Telomeres, the ends of eukaryotic chromosomes, consist of repetitive DNA sequences and their bound proteins that protect the end from the DNA damage response. Short telomeres with fewer repeats are preferentially elongated by telomerase. Tel1, the yeast homolog of human ATM kinase, is preferentially recruited to short telomeres and Tel1 kinase activity is required for telomere elongation. Rif1, a telomere-binding protein, negatively regulates telomere length by forming a complex with two other telomere binding proteins, Rap1 and Rif2, to block telomerase recruitment...
March 7, 2018: DNA Repair
Gregory J Kato, Frédéric B Piel, Clarice D Reid, Marilyn H Gaston, Kwaku Ohene-Frempong, Lakshmanan Krishnamurti, Wally R Smith, Julie A Panepinto, David J Weatherall, Fernando F Costa, Elliott P Vichinsky
Sickle cell disease (SCD) is a group of inherited disorders caused by mutations in HBB, which encodes haemoglobin subunit β. The incidence is estimated to be between 300,000 and 400,000 neonates globally each year, the majority in sub-Saharan Africa. Haemoglobin molecules that include mutant sickle β-globin subunits can polymerize; erythrocytes that contain mostly haemoglobin polymers assume a sickled form and are prone to haemolysis. Other pathophysiological mechanisms that contribute to the SCD phenotype are vaso-occlusion and activation of the immune system...
March 15, 2018: Nature Reviews. Disease Primers
Jonathan W Waks, Christopher Hamilton, Saumya Das, Ashkan Ehdaie, Jessica Minnier, Sanjiv Narayan, Mark Niebauer, Merritt Raitt, Christine Tompkins, Niraj Varma, Sumeet Chugh, Larisa G Tereshchenko
PURPOSE: Implantable cardioverter-defibrillators (ICDs) improve survival of systolic heart failure (HF) patients who are at risk of sudden cardiac death (SCD). We recently showed that electrocardiographic (ECG) global electrical heterogeneity (GEH) is independently associated with SCD in the community-dwelling cohort and developed GEH SCD risk score. The Global Electrical Heterogeneity and Clinical Outcomes (GEHCO) study is a retrospective multicenter cohort designed with two goals: (1) validate an independent association of ECG GEH with sustained ventricular tachyarrhythmias and appropriate ICD therapies and (2) validate GEH ECG risk score for prediction of sustained ventricular tachyarrhythmias and appropriate ICD therapies in systolic HF patients with primary prevention ICD...
March 14, 2018: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
Jim T Vehmeijer, Barbara Jm Mulder, Joris R de Groot
Sudden cardiac death (SCD), mainly caused by ventricular arrhythmias, is one of the leading causes of mortality in adult congenital heart disease (ACHD) patients. An implantable cardioverter defibrillator (ICD) may prevent SCD, but risk stratification remains challenging. In this review, we will address the current guideline recommendations for ICD implantation in ACHD patients, as well as review a recent study in which the discriminative ability for SCD of these guidelines is evaluated. In this study, the guideline recommendations were applied to patients who died of SCD and living controls...
March 13, 2018: Anatolian Journal of Cardiology
Hitomi Hosoya, Jeffrey Levine, Peter Abt, David Henry, David L Porter, Saar Gill
Sickle-cell disease (SCD) leads to recurrent vaso-occlusive crises, chronic end-organ damage, and resultant physical, psychological, and social disabilities. Although hematopoietic stem-cell transplantation (HSCT) is potentially curative for SCD, this procedure is associated with well-recognized morbidity and mortality and thus is ideally offered only to patients at high risk of significant complications. However, it is difficult to identify patients at high risk before significant complications have occurred, and once patients experience significant organ damage, they are considered poor candidates for HSCT...
March 13, 2018: Blood Advances
Liselotte C R Hensen, Kathleen Goossens, Tomaz Podlesnikar, Joris I Rotmans, J Wouter Jukema, Victoria Delgado, Jeroen J Bax
BACKGROUND: Patients with advanced chronic kidney disease (CKD) have high risk for sudden cardiac death (SCD) and may benefit from implantable cardioverter-defibrillators (ICDs). However, the risk for ICD-related complications is also high in this population. Therefore, there is an unmet need for accurate risk stratification tools to identify patients with CKD at risk for ventricular arrhythmias (VAs), who may benefit from ICD implantation. The aim of this hypothesis-generating study was to investigate the association between left ventricular (LV) mechanical dispersion and LV global longitudinal strain (GLS) measured using two-dimensional speckle-tracking echocardiography and VA and SCD in patients with CKD...
March 10, 2018: Journal of the American Society of Echocardiography
Christine Berg, Allison King, Dorothy Farrar Edwards
Using descriptive case studies, this paper describes a short-term mentoring program using goal attainment scaling for young adults with sickle cell disease (SCD). Two participants received three and seven visits, respectively, before dropping out of the program with no significant change in goal attainment scores. Although the program supported meaningful individualized goals, repeated and cumulative effects of hospitalizations, sickle cell pain episodes, family health issues, unsafe location of residence, and transportation appeared to remain significant barriers for to achieve stated goals...
March 13, 2018: Occupational Therapy in Health Care
Heather Reagin, Etan Marks, Stephen Weis, Joseph Susa
We report a case in which a 43-year-old African American male with medical history of sickle cell disease (SCD) presented with a nonhealing ulcer. Biopsy revealed features of livedoid vasculopathy. Previously, livedoid vasculopathy had only been described in a patient with sickle cell trait, but never in a patient with SCD. Livedoid vasculopathy most commonly affects the distal lower extremities and is characterized by irregular, punched-out, painful ulcers that heal with stellate white scars of atrophie blanche...
February 28, 2018: American Journal of Dermatopathology
Karin Fisher, Andrea M Laikin, Katianne M Howard Sharp, Catherine A Criddle, Tonya M Palermo, Cynthia W Karlson
Limited research is available on the relationship between objective sleep patterns and pain in children with SCD. Research in other chronic pain populations suggests that the effect of sleep disruption on pain may be stronger than the effect of pain on sleep that night. To examine the bi-directional relationship between objective sleep patterns and daily pain in a pediatric SCD sample. Participants were 30 African American children with SCD 8-18 years (13 ± 2.8 years; 66.7% female) with frequent pain...
March 12, 2018: Journal of Behavioral Medicine
Nafisatu Bello, Abubakar Tukur Dawakin Kudu, Azeezat Bolanle Adetokun, Dalha Wada Taura, Yusuf Dan'asabe Jobbi, Mustapha Umar, Ibrahim Yusuf
Background and Objectives: Bacterial infection in sickle cell anaemic patients is a major cause of mortality and requires proper treatment with appropriate antibiotics. However, continue defiant of these infections causing pathogens to many antibiotics and inadequate screening methods in overburden health care facilities such as our in Kano, Nigeria necessitates the conduct of this study. A research was therefore conducted to isolate, characterize and test for antimicrobial susceptibility of bacteraemia-causing pathogens from febrile children with and without sickle cell disease in Kano, Nigeria...
2018: Mediterranean Journal of Hematology and Infectious Diseases
B Palkoci, M Vojtko, J Fialová, D Osinová, M Lajčiaková
Background: Collection of kidneys from extended criteria donors (ECD) with diagnosed brain-death forms a part of the collection program that increases the number of transplantations. Objective: To compare the results of ECD with those of standard criteria donors (SCD). Methods: In a retrospective analysis in a group of 156 kidney donors, we identified ECD donors. We detected the basic parameters of the donors before kidney collection, and then evaluated the function of the graft, the survival of the graft, and the survival of the patients after 1, 3, and 5 years of transplantation...
2018: International Journal of Organ Transplantation Medicine
David A Klingbeil, Ethan R Van Norman, Katherine E McLendon, Sarah G Ross, John C Begeny
Recently, researchers have argued that using quantitative effect sizes in single-case design (SCD) research may facilitate the identification evidence-based practices. Indices to quantify nonoverlap are among the most common methods for quantifying treatment effects in SCD research. Tau-U represents a family of effect size indices that were developed to address criticisms of previously developed measures of nonoverlap. However, more research is necessary to determine the extent to which Tau-U successfully addresses proposed limitations of other nonoverlap methods...
March 1, 2018: Behavior Modification
Benjamin Zielonka, Alan R Cohen, Kim Smith-Whitley, Bhavya S Doshi
Although musculoskeletal pain in patients with sickle cell disease (SCD) is most frequently the result of vaso-occlusive episodes, clinicians often consider other etiologies including osteomyelitis, avascular necrosis, and trauma. In this study, we report the case of a young female with SCD with hip and back pain secondary to a nontraumatic iliopsoas periosteal hematoma with evidence of adjacent bone infarction. The pathophysiology, diagnostic considerations, and management of periosteal hematomas in SCD are reviewed...
March 12, 2018: Pediatric Blood & Cancer
Shannon N Nees, Jonathan N Flyer, Anjali Chelliah, Jeffrey D Dayton, Lorraine Touchette, David Kalfa, Paul J Chai, Emile A Bacha, Brett R Anderson
OBJECTIVES: Anomalous aortic origin of a coronary artery (AAOCA) from the opposite sinus of Valsalva is a rare cardiac anomaly associated with sudden cardiac death (SCD). Single-center studies describe surgical repair as safe, although medium- and long-term effects on symptoms and risk of SCD remain unknown. We sought to describe outcomes of surgical repair of AAOCA. METHODS: We reviewed institutional records for patients who underwent AAOCA repair, from 2001 to 2016, at 2 affiliated institutions...
February 8, 2018: Journal of Thoracic and Cardiovascular Surgery
Ilknur Kozanoglu, Hakan Ozdogu
Sickle cell disease (SCD) is a life-threatening chronic condition primarily caused by genetic mutation. The disease is characterized by intermittent vaso-occlusive events and chronic hemolytic anemia. Acute complications in patients with SCD are difficult to manage due to the pathophysiological nature of the disease. Transfusion therapy is the cornerstone of management of acute complications and significantly reduces SCD morbidity and mortality. Red cell exchange (RCE), which is characterized by low iron accumulation and volume overload, has been widely used for transfusion therapy in recent years...
February 21, 2018: Transfusion and Apheresis Science
Kathleen N Zimmerman, James E Pustejovsky, Jennifer R Ledford, Erin E Barton, Katherine E Severini, Blair P Lloyd
Varying methods for evaluating the outcomes of single case research designs (SCD) are currently used in reviews and meta-analyses of interventions. Quantitative effect size measures are often presented alongside visual analysis conclusions. Six measures across two classes-overlap measures (percentage non-overlapping data, improvement rate difference, and Tau) and parametric within-case effect sizes (standardized mean difference and log response ratio [increasing and decreasing])-were compared to determine if choice of synthesis method within and across classes impacts conclusions regarding effectiveness...
March 7, 2018: Research in Developmental Disabilities
Cheryl L Maier, Phillip J Gross, Christina L Dean, Satheesh Chonat, Andrew Ip, Morgan McLemore, Fuad El Rassi, Sean R Stowell, Cassandra D Josephson, Ross M Fasano
BACKGROUND: Fever accompanying vaso-occlusive crisis is a common presentation in patients with sickle cell disease (SCD) and carries a broad differential diagnosis. Here, we report a case of transfusion-transmitted malaria in a patient with SCD presenting with acute vaso-occlusive crisis and rapidly decompensating to multisystem organ failure (MSOF). CASE REPORT: An 18-year-old African American male with SCD was admitted after multiple days of fever and severe generalized body pain...
March 9, 2018: Transfusion
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