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https://www.readbyqxmd.com/read/28816916/developmental-screening-in-pediatric-sickle-cell-disease-disease-related-risk-and-screening-outcomes-in-4-year-olds
#1
Jeffrey Schatz, Alyssa Schlenz, Laura Reinman, Kelsey Smith, Carla W Roberts
OBJECTIVE: Studies of early child development in sickle cell disease (SCD) have found modest associations between disease-related risks and developmental status in infants and toddlers, but such associations are evident by early elementary school. We screened 4-year-old children with SCD using 2 screening strategies to assess if biomedical risk factors for neurologic disease are related to developmental screening outcomes at this intermediate age. METHODS: Seventy-seven 4-year-old children with SCD (M = 4...
August 4, 2017: Journal of Developmental and Behavioral Pediatrics: JDBP
https://www.readbyqxmd.com/read/28815969/-318c-t-polymorphism-of-the-ctla-4-gene-is-an-independent-risk-factor-for-rbc-alloimmunization-among-sickle-cell-disease-patients
#2
V B Oliveira, M R Dezan, F C A Gomes, S F Menosi Gualandro, J E Krieger, A C Pereira, J D Marsiglia, J E Levi, V Rocha, A Mendrone-Junior, E C Sabino, C L Dinardo
Cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) molecule is expressed on T-lymphocyte membrane and negatively influences the antigen-presenting process. Reduced expression of CTLA-4 due to gene polymorphisms is associated with increased risk of autoimmune disorders, whose physiopathology is similar to that of post-transfusion red blood cell (RBC) alloimmunization. Our goal was to evaluate if polymorphisms of CTLA-4 gene that affect protein expression are associated with RBC alloimmunization. This was a case-control study in which 134 sickle cell disease (SCD) patients and 253 non-SCD patients were included...
August 17, 2017: International Journal of Immunogenetics
https://www.readbyqxmd.com/read/28815799/characterization-of-opioid-use-in-sickle-cell-disease
#3
Jin Han, Jifang Zhou, Santosh L Saraf, Victor R Gordeuk, Gregory S Calip
PURPOSE: Opioid analgesics are commonly used to treat vaso-occlusive pain episodes in sickle cell disease (SCD), but comprehensive evidence characterizing opioid use in this patient population is limited. Our objective was to characterize opioid use patterns among SCD patients using a large nationwide database. METHODS: A large, US medical claims database was utilized to identify a cohort of 3882 SCD patients, and characteristics of opioid use were analyzed. Clinical variables including age, gender, medication use, health care utilization, and medical history were evaluated for correlations with opioid use...
August 16, 2017: Pharmacoepidemiology and Drug Safety
https://www.readbyqxmd.com/read/28814031/evaluation-of-walking-smoothness-using-wearable-robotic-system-curara%C3%A2-for-spinocerebellar-degeneration-patients
#4
Atsushi Tsukahara, Kunihiro Yoshida, Akira Matsushima, Kumiko Ajima, Chika Kuroda, Noriaki Mizukami, Minoru Hashimoto
This paper aimed to verify the effectiveness of the wearable robotic system "curara" for patients with spinocerebellar degeneration (SCD) by evaluating walking smoothness. The curara system supports the wearer's gait using a synchronization control method that uses a neural oscillator based on a central pattern generator network. The system reflects the motional intention by adjusting the synchronization gains. This modifies the degree of interactive coordinated motion between the curara and the wearer. As a feasibility study, we evaluated the waking smoothness of 10 patients with SCD using three gain condition settings...
July 2017: IEEE ... International Conference on Rehabilitation Robotics: [proceedings]
https://www.readbyqxmd.com/read/28811769/significance-of-electronic-health-records-a-comparative-study-of-vaccination-rates-in-patients-with-sickle-cell-disease
#5
Asli Korur, Süheyl Asma, Cigdem Gereklioglu, Soner Solmaz, Can Boga, Akatlı Kürsat Ozsahin, Altug Kut
OBJECTIVE: In this study, we investigated the influence of electronic health records (EHR) and electronic vaccination schedule applications on the vaccination status of patients who were admitted to our Center for the treatment of sickle cell disease (SCD). METHODS: The vaccination status against influenza and pneumococcus infection was determined in 93 patients who were admitted to the hematology outpatient clinic, Baskent University Adana Hospital from April 2004 to March 2009...
May 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28811736/mentored-training-to-increase-diversity-among-faculty-in-the-biomedical-sciences-the-nhlbi-summer-institute-programs-to-increase-diversity-sipid-and-the-programs-to-increase-diversity-among-individuals-engaged-in-health-related-research-pride
#6
Treva K Rice, Donna B Jeffe, Josephine E A Boyington, Jared B Jobe, Victor G Dávila-Román, Juan E Gonzalez, Lisa de Las Fuentes, Levi H C Makala, Rita Sarkar, Gbenga G Ogedegbe, Anne L Taylor, Susan Czajkowski, Dabeeru C Rao, Betty S Pace, Girardin Jean-Louis, Mohamed Boutjdir
OBJECTIVE: To report baseline characteristics of junior-level faculty participants in the Summer Institute Programs to Increase Diversity (SIPID) and the Programs to Increase Diversity among individuals engaged in Health-Related Research (PRIDE), which aim to facilitate participants' career development as independent investigators in heart, lung, blood, and sleep research. DESIGN AND SETTING: Junior faculty from groups underrepresented in the biomedical-research workforce attended two, 2-3 week, annual summer research-education programs at one of six sites...
2017: Ethnicity & Disease
https://www.readbyqxmd.com/read/28810874/sudden-cardiac-death-focus-on-the-genetics-of-channelopathies-and-cardiomyopathies
#7
REVIEW
Simona Magi, Vincenzo Lariccia, Marta Maiolino, Salvatore Amoroso, Santo Gratteri
Sudden cardiac death (SCD) describes a natural and unexpected death from cardiac causes occurring within a short period of time (generally within 1 h of symptom onset) in the absence of any other potentially lethal condition. Most SCD-related diseases have a genetic basis; in particular congenital cardiac channelopathies and cardiomyopathies have been described as leading causes of SCD. Congenital cardiac channelopathies are primary electric disorders caused by mutations affecting genes encoding cardiac ion channels or associated proteins, whereas cardiomyopathies are related to mutations in genes encoding several categories of proteins, including those of sarcomeres, desmosomes, the cytoskeleton, and the nuclear envelope...
August 15, 2017: Journal of Biomedical Science
https://www.readbyqxmd.com/read/28808516/double-whammy-acute-splenic-sequestration-crisis-in-patient-with-aplastic-crisis-due-to-acute-parvovirus-infection
#8
Parminder S Minhas, Jaspreet K Virdi, Rajeshkumar Patel
Splenic dysfunction is a major feature of sickle cell disease (SCD) and can manifest as acute splenic sequestration crisis (ASSC), which is the earliest life-threatening complication seen in patients with SCD. Aplastic crisis is another potentially deadly complication of sickle cell disease that develops when erythrocyte production temporarily drops. Infection with parvovirus B-19 frequently causes aplastic crises. These two complications are known to be mutually exclusive due to their classic presentation signs and symptoms but there have been few cases where a patient can have concomitant presentation of both phenomena, which can result in a fatal outcome...
July 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/28805916/the-reduction-of-starch-in-finishing-diets-supplemented-with-oil-does-not-prevent-the-accumulation-of-trans-10-18-1-in-lamb-meat
#9
M Costa, S P Alves, A Francisco, J Almeida, C M Alfaia, S V Martins, J A M Prates, J Santos-Silva, O Doran, R J B Bessa
The experiment was conducted to test the hypothesis that the replacement of cereal with low-starch feed ingredients in lambs' finishing diets supplemented with oils could prevent the accumulation of -10-18:1 in meat. Forty lambs were fed 1 of 4 diets supplemented with soybean oil (5.9%) and fish oil (1%) for 6 wk before slaughter. The control (CON) diet contained 43% barley, and in the other diets, barley was completely replaced by dehydrated citrus pulp (DCP), dehydrated sugar beet pulp (DBP), or soybean hulls (SH)...
August 2017: Journal of Animal Science
https://www.readbyqxmd.com/read/28805463/oxygen-uptake-at-aerobic-threshold-is-inversely-associated-with-fatal-cardiovascular-and-all-cause-mortality-events
#10
Setor K Kunutsor, Sudhir Kurl, Hassan Khan, Francesco Zaccardi, Rainer Rauramaa, Jari A Laukkanen
PURPOSE: We aimed to assess the associations of oxygen uptake at aerobic threshold (VO2 at AT) with cardiovascular and all-cause mortality. DESIGN: VO2 at AT was assessed in 1,663 middle-aged men in a cohort study. Hazard ratios (HRs) were calculated for sudden cardiac death (SCD), fatal coronary heart disease (CHD) and cardiovascular disease (CVD), and all-cause mortality. RESULTS: During a median follow-up of 25.6 years, 138 SCDs, 209 fatal CHDs, 333 fatal CVDs, and 719 all-cause mortality events occurred...
August 14, 2017: Annals of Medicine
https://www.readbyqxmd.com/read/28803985/mir-199a-3p-affects-adipocytes-differentiation-and-fatty-acid-composition-through-targeting-scd
#11
Zhendong Tan, Jingjing Du, Linyuan Shen, Chendong Liu, Jideng Ma, Lin Bai, Yanzhi Jiang, Guoqing Tang, Mingzhou Li, Xuewei Li, Shunhua Zhang, Li Zhu
Body fat mass is closely associated to diseases related to obesity. MicroRNAs (miRNAs, miR) are important regulatory molecules that function as post-transcriptional gene regulators of adipocyte development. In the current study, we revealed that reduced expression of miR-199a-3p in adipose tissue resulting from high fat diet (HFD)-induced obesity in mice. Overexpression of miR-199a-3p promoted adipocyte proliferation by regulating the expression of regulating factors of the cell cycle. Furthermore, miR-199a-3p blunted lipid accumulation in 3T3-L1 adipocytes...
August 10, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28802541/opioid-doses-and-acute-care-utilization-outcomes-for-adults-with-sickle-cell-disease-emergency-department-versus-acute-care-unit
#12
Robert E Molokie, Chariz Montminy, Corissa Dionisio, Muhammad Ahmen Farooqui, Michel Gowhari, Yingwei Yao, Marie L Suarez, Miriam O Ezenwa, Judith M Schlaeger, Zaijie J Wang, Diana J Wilkie
BACKGROUND: Acute care units (ACUs) with focused sickle cell disease (SCD) care have been shown to effectively address pain and limit hospitalizations compared to emergency departments (ED), the reason for differences in admission rates is understudied. Our aim was compare effects of usual care for adult SCD pain in ACU and ED on opioid doses and discharge pain ratings, hospital admission rates and lengths of stay. METHODS: In a retrospective, comparative cohort, single academic tertiary center study, 148 adults with sickle cell pain received care in the ED, ACU or both...
July 13, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28801954/an-analysis-of-inpatient-pediatric-sickle-cell-disease-incidence-costs-and-outcomes
#13
Laura M Bou-Maroun, Fabien Meta, Curtis J Hanba, Andrew D Campbell, Gregory A Yanik
OBJECTIVE: To identify characteristics of pediatric sickle cell disease (SCD) hospitalizations and to examine admission demographics and medical expenditures. METHODS: Admissions with SCD were identified from the 2009 and 2012 releases of the Healthcare and Cost Utilization Project's Kids Inpatient Database. Disease-specific secondary diagnoses including acute chest syndrome (ACS), vaso-occlusive pain crisis (VOC), splenic sequestration, and stroke/transient ischemic attack were analyzed for patient and hospital demographics...
August 12, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28801805/pulmonary-cta-in-sickle-cell-patients-quantitative-assessment-of-enhancement-quality
#14
Jeff Jensen, Tony Lin, Elliot K Fishman, Pamela T Johnson
PURPOSE: The purpose of this study was to validate the observation that pulmonary artery (PA) enhancement is often decreased in sickle cell disease (SCD) patients imaged with MDCT for suspected pulmonary embolism and determine whether contrast infusion parameters are accountable for lower enhancement levels. MATERIALS AND METHODS: Retrospective comparison of 35 adult SCD patients imaged for suspected pulmonary embolism (PE) in our emergency department using 128-slice dual source MDCT scanner to 34 age and weight matched adult controls...
August 11, 2017: Emergency Radiology
https://www.readbyqxmd.com/read/28801781/acute-pain-crisis-in-a-patient-with-sickle-cell-disease-undergoing-ovarian-simulation-for-fertility-preservation-prior-to-curative-stem-cell-transplantation-case-report-and-literature-review
#15
Michelle Matthews, Rebecca Pollack
PURPOSE: Hematopoietic stem cell transplantation (HSCT) is a cure for sickle cell disease (SCD) but frequently results in permanent sterility. The complications associated with oocyte cryopreservation and risks of future pregnancy are increased in SCD patients. This case report discusses risk reduction strategies and includes a literature review of pregnancy after HSCT. CASE: A 23-year-old woman underwent ovarian stimulation for fertility preservation resulting in cancelation due to acute pain crisis...
August 11, 2017: Journal of Assisted Reproduction and Genetics
https://www.readbyqxmd.com/read/28801090/leptin-concentrations-and-scd-1-indices-in-classical-homocystinuria-evidence-for-the-role-of-sulfur-amino-acids-in-the-regulation-of-lipid-metabolism
#16
Soraia Poloni, Poli Mara Spritzer, Roberta H Mendes, Vânia D'almeida, Kamila Castro, Fernanda Sperb-Ludwig, Johanna Kugele, Sara Tucci, Henk J Blom, Ida V D Schwartz
BACKGROUND: We describe body composition, lipid metabolism and Stearoyl-CoA desaturase-1 (SCD-1) indices in patients with classical homocystinuria (HCU). METHODS: Eleven treated HCU patients and 16 healthy controls were included. Body composition and bone mineral density were assessed by dual X-ray absorptiometry. Sulfur-containing amino acids (SAA) and their derivatives (total homocysteine, cysteine, methionine, S-adenosylmethionine, S-adenosylhomocysteine, and glutathione), lipids (free fatty acids, acylcarnitines, triglycerides and lipoproteins), glucose, insulin, leptin, adiponectin, and isoprostanes were measured in plasma...
August 8, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28799862/disrupted-topologic-efficiency-of-white-matter-structural-connectome-in-individuals-with-subjective-cognitive-decline
#17
Ni Shu, Xiaoni Wang, Qiuhui Bi, Tengda Zhao, Ying Han
Purpose To determine whether individuals with subjective cognitive decline (SCD), which is defined by memory complaints with normal performance at objective neuropsychologic examinations, exhibit disruptions of white matter (WM) connectivity and topologic alterations of the brain structural connectome. Materials and Methods Diffusion-tensor magnetic resonance imaging and graph theory approaches were used to investigate the topologic organization of the brain structural connectome in 36 participants with SCD (21 women: mean age, 62...
August 11, 2017: Radiology
https://www.readbyqxmd.com/read/28797896/predictors-of-mortality-in-adults-with-sickle-cell-disease-admitted-to-intensive-care-unit-in-bahrain
#18
Sana Abdulaaziz Al Khawaja, Zainab Mahdi Ateya, Ridha Abdulla Al Hammam
OBJECTIVE: Sickle cell disease (SCD) is one of the most common genetic blood disorders in Bahrain. However, there is a paucity of data regarding the clinical presentation of SCD patients who require ICU admission. This study aimed to describe the epidemiological data of SCD patients admitted to the ICU and to identify predictors of mortality in order to help intensivists identify patients at most risk. METHOD: This study used a retrospective, descriptive, and correlational design...
July 15, 2017: Journal of Critical Care
https://www.readbyqxmd.com/read/28797161/masked-inherited-primary-arrhythmia-syndromes-in-sudden-cardiac-death-patients-accompanied-by-coronary-vasospasm
#19
Ki Hong Lee, Hyung Wook Park, Jeong Nam Eun, Jeong Gwan Cho, Nam Sik Yoon, Mi Ran Kim, Yo Han Ku, Hyukjin Park, Seung Hun Lee, Jeong Han Kim, Min Chul Kim, Woo Jin Kim, Hyun Kuk Kim, Jae Yeong Cho, Keun-Ho Park, Doo Sun Sim, Hyun Ju Yoon, Kye Hun Kim, Young Joon Hong, Ju Han Kim, Youngkeun Ahn, Myung Ho Jeong, Jong Chun Park
Background/Aims: Coronary vasospasms are one of the important causes of sudden cardiac death (SCD). Provocation of coronary vasospasms can be useful, though some results may lead to false positives, with patients potentially experiencing recurrent SCD despite appropriate medical treatments. We hypothesized that it is not coronary vasospasms but inherited primary arrhythmia syndromes (IPAS) that underlie the development of SCD. Methods: We analyzed 74 consecutive patients (3...
August 11, 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28795011/cardiac-screening-to-prevent-sudden-death-in-young-athletes
#20
REVIEW
Christopher Schmehil, Devika Malhotra, Dilip R Patel
Sudden cardiac death (SCD) is a sudden and unexpected death caused by loss of heart of function. SCD may occur in any population, but when it occurs on the playing field in a young individual, communities worldwide are affected. Although these events are rare, media coverage of sudden cardiac arrests in young athletes have created the impression that these events are far more common than they appear. With a heightened awareness of SCD in young athletes, screening methods have been developed to try and prevent these events from occurring...
July 2017: Translational pediatrics
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