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Duane syndrome

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https://www.readbyqxmd.com/read/28927162/myelodysplastic-syndrome-unclassifiable-mds-u-with-1-blasts-is-a-distinct-subgroup-of-mds-u-with-a-poor-prognosis
#1
Elizabeth Margolskee, Robert P Hasserjian, Duane Hassane, Wayne Tam, Susan Mathew, Chi Young Ok, Sa A Wang, Jean Oak, Daniel A Arber, Attilio Orazi
Objectives: Three situations qualify as myelodysplastic syndrome, unclassifiable (MDS-U): (1) refractory cytopenia with dysplasia and 1% blasts in peripheral blood (BL), (2) pancytopenia with unilineage dysplasia (Pan), and (3) persistent cytopenia, less than 5% bone marrow blasts, and less than 10% dysplastic cells and presence of MDS-defining cytogenetic abnormalities (CG). We compared the clinicopathologic features and mutational profiles for these three groups. Methods: MDS-U cases were reviewed at four major academic institutions...
July 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28918207/the-association-between-cardiac-physiology-acquired-brain-injury-and-postnatal-brain-growth-in-critical-congenital-heart-disease
#2
Shabnam Peyvandi, Hosung Kim, Joanne Lau, A James Barkovich, Andrew Campbell, Steven Miller, Duan Xu, Patrick McQuillen
OBJECTIVE: To assess the trajectory of perioperative brain growth in relationship to cardiac diagnosis and acquired brain injuries. METHODS: This was a cohort study of term neonates with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA). Subjects underwent magnetic resonance imaging of the brain pre- and postoperatively to determine the severity of brain injury and total and regional brain volumes by the use of automated morphometry...
August 24, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28910146/management-of-ards-and-refractory-hypoxemia-a-multicenter-observational-study
#3
Erick H Duan, Neill Kj Adhikari, Frederick D'Aragon, Deborah J Cook, Sangeeta Mehta, Waleed Alhazzani, Ewan Goligher, Emmanuel Charbonney, Yaseen M Arabi, Tim Karachi, Alexis F Turgeon, Lori Hand, Qi Zhou, Peggy Austin, Jan Friedrich, Francois Lamontagne, François Lauzier, Rakesh Patel, John Muscedere, Richard Hall, Pierre Aslanian, Thomas Piraino, Martin Albert, Sean M Bagshaw, Mike Jacka, Gordon Wood, William Henderson, Delbert Dorscheid, Niall D Ferguson, Maureen O Meade
RATIONALE: Clinicians' current practice patterns in the management of acute respiratory distress syndrome (ARDS) and refractory hypoxemia are not well described. OBJECTIVES: To describe mechanical ventilation strategies and treatment adjuncts for adults with ARDS including refractory hypoxemia. METHODS: Prospective cohort study (March 2014-February 2015) of mechanically ventilated adults with moderate-to-severe ARDS requiring FiO2 ≥0.50 in 24 ICUs...
September 14, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28904560/antidiabetic-effect-of-tibetan-medicine-tang-kang-fu-san-on-high-fat-diet-and-streptozotocin-induced-type-2-diabetic-rats
#4
Bailu Duan, Zhongqiu Zhao, Ling Lin, Jing Jin, Lijun Zhang, Hui Xiong, Na Ta, Tiexiang Gao, Zhinan Mei
The aim of this study was to investigate the antidiabetic effects of a Tibetan medicine, Tang-Kang-Fu-San (TKFS), on experimental type 2 diabetes mellitus (T2DM) rats and to explore its underlying mechanisms. Firstly two major chemical compositions of TKFS, gallic acid and curcumin, were characterized by HPLC fingerprint analysis. Next T2DM in rats was induced by high-fat diet and a low-dose streptozotocin (STZ 35 mg/kg). Then oral gavage administration of three different doses of TKFS (0.3 g/kg, 0.6 g/kg, and 1...
2017: Evidence-based Complementary and Alternative Medicine: ECAM
https://www.readbyqxmd.com/read/28877834/clinicopathological-features-of-idiopathic-membranous-nephropathy-in-33-adolescents
#5
Chao Li, Hang Li, Yu-Bing Wen, Jia-Ning Li, Wei-Feng Lin, Jian-Fang Cai, Lin Duan, Yan Li, Xue-Mei Li, Xue-Wang Li
Objective To investigate the clinicopathological features and prognosis of idiopathic membranous nephropathy(IMN)in adolescents. Methods This was a retrospective study on IMN patients hospitalized between June 2012 and December 2014,and a total of 33 IMN patients aged between 13 and 24 years old were enrolled in the study.Meanwhile,33 IMN patients aged more than 24 years old were selected randomly as control group during the same period.Diagnosis was confirmed by renal biopsy,and the secondary causes of membranous nephropathy were ruled out...
August 20, 2017: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/28872507/protection-of-the-temporomandibular-joint-during-syndromic-mandibular-distraction-with-a-novel-condylar-offloading-device
#6
Sagar T Mehta, Neal Moores, Duane Yamashiro, Faizi Siddiqi, Barbu Gociman
Mandibular distraction for severe micrognathia in syndromic patients often leads to temporomandibular joint (TMJ) ankylosis, which requires further interventions to regain joint motion. The increased incidence of postdistraction ankylosis is likely related to increased prevalence of preoperative joint pathology in syndromic micrognathic patients. Previous studies have demonstrated that offloading the pressure on the condyle during the distraction process can prevent such TMJ pathology. In this article, the authors describe a successful new method for offloading the condyle to prevent postdistraction ankylosis using an external TMJ offloading device...
September 1, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28863211/exome-sequencing-identifies-a-novel-mutation-of-the-gdi1-gene-in-a-chinese-non-syndromic-x-linked-intellectual-disability-family
#7
Yongheng Duan, Sheng Lin, Lichun Xie, Kaifeng Zheng, Shiguo Chen, Hui Song, Xuchun Zeng, Xueying Gu, Heyun Wang, Linghua Zhang, Hao Shao, Wenxu Hong, Lijie Zhang, Shan Duan
X-linked intellectual disability (XLID) has been associated with various genes. Diagnosis of XLID, especially for non-syndromic ones (NS-XLID), is often hampered by the heterogeneity of this disease. Here we report the case of a Chinese family in which three males suffer from intellectual disability (ID). The three patients shared the same phenotype: no typical clinical manifestation other than IQ score ≤ 70. For a genetic diagnosis for this family we carried out whole exome sequencing on the proband, and validated 16 variants of interest in the genomic DNA of all the family members...
July 2017: Genetics and Molecular Biology
https://www.readbyqxmd.com/read/28859437/slc44a4-mutation-causes-autosomal-dominant-hereditary-postlingual-non-syndromic-mid-frequency-hearing-loss
#8
Zhaoxin Ma, Wenjun Xia, Fei Liu, Jing Ma, Shaoyang Sun, Jin Zhang, Nan Jiang, Xu Wang, Jiongjiong Hu, Duan Ma
No abstract text is available yet for this article.
August 15, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28845325/ocular-causes-of-abnormal-head-position-strabismus-clinic-data
#9
Kadriye Erkan Turan, Hande Taylan Şekeroğlu, İrem Koç, Esra Vural, Jale Karakaya, Emin Cumhur Şener, Ali Şefik Sanaç
OBJECTIVES: To determine the most common ocular causes and types of abnormal head position (AHP) and describe their clinical features. MATERIALS AND METHODS: Patients with AHP who had been followed in the strabismus unit were retrospectively reviewed. Demographic features and orthoptic characteristics were recorded. RESULTS: A total of 163 patients including 61 women (37.4%) and 102 men (62.6%), with a mean age of 19.9±18.3 were recruited...
August 2017: Turkish Journal of Ophthalmology
https://www.readbyqxmd.com/read/28835036/-a-case-of-hemophagocytic-syndrome-secondary-to-extranodal-nasal-type-nk-t-cell-lymphoma-%C3%A2-b
#10
S X Zhang, D Luo, H S Liu, Y Bao, C Pei, Y Q Duan
No abstract text is available yet for this article.
August 9, 2017: Zhonghua Kou Qiang Yi Xue za Zhi, Zhonghua Kouqiang Yixue Zazhi, Chinese Journal of Stomatology
https://www.readbyqxmd.com/read/28830623/remifentanil-a-help-in-topical-strabismus-surgery
#11
I Sánchez-Guillén, R López, M A Calle, A B Diez-Lobo
OBJECTIVE: To analyze the analgesic effect of remifentanil, side effects and complications in topical strabismus surgery. To study the results of strabismus surgery with this type of anesthesia. MATERIAL AND METHOD: Retrospective descriptive study. We included 39 patients undergoing strabismus surgery with topical anesthesia and analgesia-based sedation with remifentanil. The data of the anesthetic and surgical technique, surgical results and stability of the deviation angle were analyzed...
August 19, 2017: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/28826864/surgical-outcomes-of-cavernous-sinus-syndrome-in-pituitary-adenomas
#12
Weilun Fu, Lian Duan, Sumin Geng
OBJECT: The type of pituitary adenoma with a manifestation that includes cavernous sinus syndrome is rare. Based on the clinical data of 70 patients, this study investigated the pathogenesis, imaging characteristics, and prognostic factors of pituitary adenoma with cavernous sinus syndrome. METHODS: We conducted a retrospective analysis of the characteristics of pituitary adenoma patients with cavernous sinus syndrome who received surgical treatment. The patients were classified into different prognosis groups according to the time required for the patients to recover from the cavernous sinus syndrome...
August 18, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28783050/comparison-of-metabolic-syndrome-indicators-in-two-samples-of-central-and-south-americans-living-in-the-washington-d-c-area-in-1993-1994-and-2008-2009-secular-changes-in-metabolic-syndrome-in-hispanics
#13
Regina Gill, Robert T Jackson, Marguerite Duane, Allison Miner, Saira A Khan
The Central and South American populations are growing rapidly in the US; however, there is a paucity of information about their health status. Objectives: we estimated the prevalence of metabolic syndrome (MetS) and its individual components from two cohorts of Central and South Americans. Methods: This cross-sectional, medical record extraction survey sampled 1641 adults from a Washington, D.C clinic. A questionnaire was used to collect socio-demographic, medical history, anthropometric, biochemical, and clinical data...
August 5, 2017: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/28763016/a-multiplex-rt-pcr-assay-to-detect-and-discriminate-porcine-reproductive-and-respiratory-syndrome-viruses-in-clinical-specimens
#14
Keli Yang, Yongxiang Tian, Danna Zhou, Zhengying Duan, Rui Guo, Zewen Liu, Fangyan Yuan, Wei Liu
Outbreaks of highly pathogenic porcine reproductive and respiratory syndrome virus (HP-PRRSV) have led to large economic losses in China. The attenuated vaccine (HP-PRRSV JXA1-R) was used to control HP-PRRSV. However, in recent years, co-infection with classical PRRSV (C-PRRSV), HP-PRRSV, and/or HP-PRRSV JXA1-R has been increasing in China, resulting in a significant impact on PRRSV diagnostics and management. To facilitate rapid discrimination of HP-PRRSV JXA1-R from HP-PRRSV and C-PRRSV, a multiplex RT-PCR assay for the visual detection of HP-PRRSV JXA1-R, HP-PRRSV, and C-PRRSV was established and evaluated with reference PRRSV strains and clinical samples...
August 1, 2017: Viruses
https://www.readbyqxmd.com/read/28742027/a-regularized-deep-learning-approach-for-clinical-risk-prediction-of-acute-coronary-syndrome-using-electronic-health-records
#15
Zhengxing Huang, Wei Dong, Huilong Duan, Jiquan Liu
OBJECTIVE: Acute coronary syndrome (ACS), as a common and severe cardiovascular disease, is a leading cause of death and the principal cause of serious long-term disability globally. Clinical risk prediction of ACS is important for early intervention and treatment. Existing ACS risk scoring models are based mainly on a small set of hand-picked risk factors and often dichotomize predictive variables to simplify the score calculation [1-3]. METHODS: This study develops a regularized stacked denoising auto-encoder (SDAE) model to stratify clinical risks of ACS patients from a large volume of electronic health records (EHR)...
July 24, 2017: IEEE Transactions on Bio-medical Engineering
https://www.readbyqxmd.com/read/28716375/aging-and-post-intensive-care-syndrome-a-critical-need-for-geriatric-psychiatry
#16
REVIEW
Sophia Wang, Duane Allen, You Na Kheir, Noll Campbell, Babar Khan
Because of the aging of the intensive care unit (ICU) population and an improvement in survival rates after ICU hospitalization, an increasing number of older adults are suffering from long-term impairments because of critical illness, known as post-intensive care syndrome (PICS). This article focuses on PICS-related cognitive, psychological, and physical impairments and the impact of ICU hospitalization on families and caregivers. The authors also describe innovative models of care for PICS and what roles geriatric psychiatrists could play in the future of this rapidly growing population...
June 1, 2017: American Journal of Geriatric Psychiatry
https://www.readbyqxmd.com/read/28677207/new-role-of-lrp5-associated-with-nonsyndromic-autosomal-recessive-hereditary-hearing-loss
#17
Wenjun Xia, Jiongjiong Hu, Fei Liu, Jing Ma, Shaoyang Sun, Jin Zhang, Kaiyue Jin, Jianbo Huang, Nan Jiang, Xu Wang, Wen Li, Zhaoxin Ma, Duan Ma
Human hearing loss is a common neurosensory disorder about which many basic research and clinically relevant questions are unresolved. At least 50% of hearing loss are due to a genetic etiology. Although hundreds of genes have been reported, there are still hundreds of related deafness genes to be found. Clinical, genetic, and functional investigations were performed to identify the causative mutation in a distinctive Chinese family with postlingual nonsyndromic sensorineural hearing loss. Whole-exome sequencing (WES) identified lipoprotein receptor-related protein 5 (LRP5), a member of the low-density lipoprotein receptor family, as the causative gene in this family...
July 4, 2017: Human Mutation
https://www.readbyqxmd.com/read/28674241/mutation-analysis-of-col4a3-and-col4a4-genes-in-a-chinese-autosomal-dominant-alport-syndrome-family
#18
Liwei Guo, Duan Li, Shuangshuang Dong, Donghao Wan, Baosheng Yang, Yanmei Huang
Autosomal dominant Alport syndrome (ADAS) accounts for 5% of all cases of Alport syndrome (AS), a primary basement membrane disorder arising from mutations in genes encoding the type IV collagen protein family.Mutations in COL4A3 and COL4A4 genes were reported to be associated with ADAS. In this study, clinical data in a large consanguineous family with seven affected members were reviewed, and genomic DNA was extracted. For mutation screening, all exons of COL4A3 and COL4A4 genes were polymerase chain reaction-amplified and direct sequenced from genomic DNA, and the mutations were analyzed by comparing with members in this family, 100 ethnicitymatched controls and the sequence of COL4A3 and COL4A4 genes from GenBank...
June 2017: Journal of Genetics
https://www.readbyqxmd.com/read/28666948/bile-acid-deficiency-in-a-subgroup-of-patients-with-irritable-bowel-syndrome-with-constipation-based-on-biomarkers-in-serum-and-fecal-samples
#19
Priya Vijayvargiya, Irene Busciglio, Duane Burton, Leslie Donato, Alan Lueke, Michael Camilleri
BACKGROUND & AIMS: Short-term administration of delayed-release chenodeoxycholic acid to patients with irritable bowel syndrome with constipation (IBS-C) accelerates colonic transit and reduces symptoms. A preliminary study has shown that patients with IBS-C have reduced levels of bile acids (BAs) in feces and reduced synthesis of BA. We compared the levels of primary and secondary BAs in fecal samples collected over a 48-hour period from patients with IBS-C on a diet that contained 100 g fat per day, and compared them with levels in samples from healthy volunteers (controls)...
June 27, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28655092/-evaluation-of-clinical-characteristics-myd88-l265p-mutation-cxcr4-whim-mutation-and-prognosis-in-waldenstr%C3%A3-m-macroglobulinemia-a-single-center-retrospective-study-of-93-patients
#20
X X Cao, Q Meng, H Cai, Y Y Mao, M H Duan, T N Zhu, W Zhang, B Han, J L Zhuang, H C Cai, M Chen, J Feng, X Han, Y Zhang, C Yang, L Zhang, D B Zhou, J Li
Objective: To evaluate the clinical characteristics, MYD88(L265P) mutation, CXCR4(W)HIM mutation and prognosis in patients with Waldenström macroglobulinemia (WM). Methods: The clinical characteristics, International Prognostic Scoring System for symptomatic WM (WPSS) , and overall survival (OS) were retrospectively assayed in 93 patients with newly diagnosed WM at Peking Union Medical College Hospital during January 2000 to August 2016. The MYD88(L265P) mutation and CXCR4(W)HIM mutation were tested among 34 patients...
June 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
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