bluuebunny/crossref_metadata_2025_split huggingface dataset:
The outcome of surgical management of type A aortic dissection
Authors: Skripochnik Edvard , Friedman Patricia , Michler Robert E , Neragi-Miandoab Siyamek 
Published: November 2013

Background Type A aortic dissection is a serious and fatal condition. Methods We retrospectively evaluated the outcome following repair of ascending aortic dissection in a contemporary cohort of 108 patients with a mean age of 59.5 ± 13.8 years, who were treated between 2006 and 2011. Most patients were male (70, 65%). Results Circulatory arrest with a mean duration of 22 ± 16 min was performed in 42 (38.9%) patients. Perioperative mortality was 15.7% ( n = 17). Univariate analysis showed that cardiopulmonary bypass time ( p = 0.0006), age >60 years ( p = 0.028), cardiogenic shock at presentation ( p = 0.02), New York Heart Association class II–IV ( p = 0.038), hemopericardium ( p = 0.0035), and preoperative cerebrovascular accident ( p = 0.02) were predictors of mortality. Multivariate analysis indicated that age >60 years (odds ratio 7.7, 95% confidence interval: 1.52–38.96, p = 0.0136), preoperative cerebrovascular accident (odds ratio 25.2, 95% confidence interval: 2.45–258.9, p = 0.0066), hemopericardium (odds ratio 41.6, 95% confidence interval: 5.38–320.7, p = 0.0003), and cardiopulmonary bypass time (odds ratio 1.85, 95% confidence interval: 1.32–2.57, p = 0.0003) were independent predictors of perioperative mortality. The 1- and 4-year survival was 80% ± 3.8% and 69% ± 5.7%, respectively. Age >60 years (hazard ratio 3.3, 95% confidence interval: 1.4–7.9, p = 0.0064) was the only independent predictor of long-term mortality. Conclusion Our results identify the major risk factors for perioperative and long-term mortality. Age is an independent risk factor for mortality.
Acute hemodynamic effects of a selective serotonin reuptake inhibitor in postural tachycardia syndrome: A randomized, crossover trial
Authors: Mar Philip L , Raj Vidya , Black Bonnie K , Biaggioni Italo , Shibao Cyndya A , Paranjape Sachin Y , Dupont William D , Robertson David , Raj Satish R 
Published: November 2013

Background: Selective serotonin reuptake inhibitors (SSRIs) are often prescribed in patients with postural tachycardia syndrome (POTS), and act at synaptic terminals to increase monoamine neurotransmitters. We hypothesized that they act to increase blood pressure and attenuate reflex tachycardia, thereby improving symptoms. Acute hemodynamic profiles after SSRI administration in POTS patients have not previously been reported. Methods: Patients with POTS ( n=39; F=37, 39 ±9 years) underwent a randomized crossover trial with sertraline 50mg and placebo. Heart rate, systolic, diastolic, and mean blood pressure were measured with the patient seated and standing for 10 min prior to drug or placebo administration, and then hourly for 4 h. The primary endpoint was standing heart rate at 4 h. Results: At 4 h, standing heart rate and systolic blood pressure were not significantly different between sertraline and placebo. Seated systolic (106±12 mmHg vs. 101±8 mmHg; p=0.041), diastolic (72±8 mmHg vs. 69±8 mmHg; p=0.022), and mean blood pressure (86±9 mmHg vs. 81±9 mmHg; p=0.007) were significantly higher after sertraline administration than placebo. At 4 h, symptoms were worse with sertraline than placebo. Conclusions: Sertraline had a modest pressor effect in POTS patients, but this did not translate into a reduced heart rate or improved symptoms.
Variation in Tibial Tubercle–Trochlear Groove Measurement as a Function of Age, Sex, Size, and Patellar Instability
Authors: Pennock Andrew T. , Alam Milad , Bastrom Tracey 
Published: November 2013

Background: The tibial tubercle–trochlear groove (TT-TG) measurement was developed to quantify morphologic abnormalities about the knee associated with patellar instability and to help guide surgical decision making. Purpose: To assess variations in TT-TG as a function of patient age and size in a population of patients with patellar instability compared with those with no instability. Study Design: Cross-sectional study; Level of evidence, 3. Methods: All patients younger than 20 years who underwent surgery for patellar instability from 2010 to 2012 were included in this retrospective study. A total of 180 patients with no history of patellar instability or patellofemoral complaints were used as a control group. The TT-TG was measured on magnetic resonance axial images using the centers of the tibial tubercle and the trochlear groove. Data were normalized based on patient height, weight, body mass index (BMI), and femur width. Alpha was set at P < .05 to declare significance. Results: The average age of the 45 patellar instability patients was 15.4 years (range, 10-18 years), their mean TT-TG was 16.3 mm (range, 6.5-26 mm), and 51% were male. By comparison, the mean age of the control group was 16 years (range, 10-19 years), the mean TT-TG was 11.7 mm (range, 3-22 mm), and 58% were male. The TT-TG and the normalized TT-TG for height, weight, BMI, and femur width were all greater in the patellar instability group compared with the control group ( P ≤ .001). Thirty-one percent of patients had a TT-TG greater than 20 mm in the instability group compared with 3% in the control group ( P < .05, specificity 97%, sensitivity 31%). The TT-TG was found to increase as a function of height in both groups ( r = 0.14, P = .04) and decreased with age only in the instability group ( r = −0.3, P = .04). Conclusion: An elevated TT-TG is associated with patellar instability both in pediatric and adolescent patients. However, this measurement varies as a function of patient age and height, with each centimeter in height increasing the TT-TG by 0.12. Normalization of TT-TG to patient height may control for size variations and should be undertaken in the work-up and management of patients with patellar instability.
A Randomized, Double-Blind Pilot Study of Dexmedetomidine Versus Midazolam for Intensive Care Unit Sedation
Authors: MacLaren Robert , Preslaski Candice R. , Mueller Scott W. , Kiser Tyree H. , Fish Douglas N. , Lavelle James C. , Malkoski Stephen P. 
Published: November 2013

Introduction: Sedation with dexmedetomidine may facilitate ventilator liberation and limit the occurrence of delirium. No trial has assessed patient recall or the development of psychological outcomes after dexmedetomidine sedation. This pilot study evaluated whether transitioning benzodiazepine sedation to dexmedetomidine alters patient recall and the incidence of anxiety, depression, or acute stress disorder (ASD). Methods: This investigation was a randomized, double-blind, single-center study. Existing continuous benzodiazepine sedation was converted to dexmedetomidine or midazolam when patients qualified for daily awakenings. Sedation was titrated to achieve Riker sedation agitation scores of 3 to 4. The intensive care unit (ICU) Stressful Experiences Questionnaire, hospital anxiety and depression scale, and the impact of event scale-revised were administered before hospital discharge to assess recall, anxiety, depression, and manifestations of ASD. Results: A total of 11 patients received dexmedetomidine, and 12 patients received midazolam. Median dosing was 0.61 µg/kg/h for 3.5 days for dexmedetomidine and 3.7 mg/h for 3 days for midazolam. Attainment of goal sedation and analgesia was similar; however, more dexmedetomidine patients experienced agitation and pain. The median duration of mechanical ventilation from study drug initiation to extubation was 3.4 days in dexmedetomidine patients and 2.9 days in midazolam patients. Dexmedetomidine patients remembered 18.5 experiences compared with 8.5 in midazolam patients ( P = .015). Rates of anxiety and depression were similar. In all, 5 (62.5%) dexmedetomidine patients and 1 (12.5%) midazolam patient manifested ASD ( P = .063), and 1 dexmedetomidine patient and 5 midazolam patients developed new-onset delirium ( P = .07). Hypotension occurred in 10 (90.9%) dexmedotomidine patients and 6 (50%) midazolam patients ( P = .069). Conclusions: Transitioning benzodiazepine sedation to dexmedetomidine when patients qualify for daily awakenings may reduce the development of delirium and facilitate remembrance of ICU experiences but may lead to manifestations of ASD. Monitoring hypotension is required for both the sedatives. Additional comparative studies focusing on the long-term impact of ICU recall and psychological outcomes are needed.
“This won’t hurt a bit”
Authors: Brakoulias Vlasios 
Published: November 2013

Background: In our quest to improve the lives of our patients, we can often be swept up by the euphoria associated with new treatments. At times, our enthusiasm for new treatments can lead us to forget the fundamental principles of patient care and lose focus of an individual’s suffering and personal journey. Objective: To illustrate the importance of understanding a patient’s predicament in the context of our own need to cure. Methods: Oil on canvas (92 × 122cm). Results: By focusing on a centrally positioned patient who has hypothetically just had new electronic devices installed into her scalp and chest wall, I was able to see a weakened lady with a turbulent past attempting to protect herself with her jet black hair. Many around her remain oblivious to the storm. An observant medical student glances away from his textbook to see that all is not well outside the hospital walls. Conclusions: Several perspectives are required to adequately assess the way that we treat patients. Contrary to our expectations, our treatments might hurt a bit.
Site Selection and Pain Outcome After Autologous Bone Graft Harvest
Authors: Baumhauer Judith , Pinzur Michael S. , Donahue Rafe , Beasley William , DiGiovanni Christopher 
Published: November 2013

Background: In foot and ankle surgery, there are multiple sites used for autologous bone graft, including the proximal (PT) or distal tibia (DT), calcaneus (C), and iliac crest (ICBG). There has been no comparison between these anatomic areas and the potential for acute or persistent pain at 1 year. The purpose of this study was to prospectively compare patient-reported outcomes of acute and persistent pain at 1 year after surgery to determine if harvest site selection made a difference. Methods: As part of a clinical trial examining ankle and hindfoot fusion rates with autograft compared with synthetic graft, the autologous bone graft harvest sites were assessed with visual analog pain outcome scores at 3, 24, 36, and 52 weeks after surgery. Patients with a score of 20+ defined clinically significant pain. Four harvest sites were compared: ICBG, PT, DT, and C. Fisher exact test was used to compare the graft site pain between locations. Results: Twelve percent of subjects reported clinically significant pain at 24 weeks and 8.5% at 52 weeks postoperatively. Each lower extremity harvest site (C, DT, PT) showed higher rates of clinically significant graft harvest site pain than the ICBG at 52 weeks. Conclusions: Autologous bone graft harvest carried a risk of persistent pain at up to 1 year (weeks 24-52) in 18% of patients. Lower-extremity bone graft sites had the greatest risk for persistent pain at 1 year. Level of Evidence: Level II, prospective comparative study.
The effect of real-time teleconsultations between hospital-based nurses and patients with severe COPD discharged after an exacerbation
Authors: Sorknaes Anne Dichmann , Bech Mickael , Madsen Hanne , Titlestad Ingrid L , Hounsgaard Lise , Hansen-Nord Michael , Jest Peder , Olesen Finn , Lauridsen Joergen , Østergaard Birte 
Published: November 2013

Summary We investigated the effect of daily real-time teleconsultations for one week between hospital-based nurses specialised in respiratory diseases and patients with severe COPD discharged after acute exacerbation. Patients admitted with acute exacerbation of chronic obstructive pulmonary disease (AECOPD) at two hospitals were recruited at hospital discharge. They were randomly assigned to intervention or control. The telemedicine equipment consisted of a briefcase with built-in computer including a web camera, microphone and measurement equipment. The primary outcome was the mean number of total hospital readmissions within 26 weeks of discharge. A total of 266 patients (mean age 72 years) were allocated to either intervention (n = 132) or control (n = 134). There was no significant difference in the unconditional total mean number of hospital readmissions after 26 weeks: mean 1.4 (SD 2.1) in the intervention group and 1.6 (SD 2.4) in the control group. In a secondary analysis, there was no significant difference between the two groups in mortality, time to readmission, mean number of total hospital readmissions, mean number of readmissions with AECOPD, mean number of total hospital readmission days or mean number of readmission days with AECOPD calculated at 4, 8, 12 and 26 weeks. Thus the addition of one week of teleconsultations between hospital-based nurses and patients with severe COPD discharged after hospitalisation did not significantly reduce readmissions or affect mortality.
‘Consumed by breathing’ – a critical interpretive meta-synthesis of the qualitative literature
Authors: Harrison Samantha Louise , Apps Lindsay , Singh Sally J , Steiner Michael C , Morgan Mike DL , Robertson Noelle 
Published: November 2013

Objective To derive an improved understanding of how patients respond to, appraise, and understand the experience of an acute exacerbation of chronic obstructive pulmonary disease via a critical interpretive meta-synthesis. Methods Search terms – Exacerbate* OR hospital* AND ‘Chronic obstructive’ OR emphysema OR bronchitis AND interview* OR qualitative. Inclusion criteria – Primary research published in English of patients’ experiences of an acute exacerbation of chronic obstructive pulmonary disease. Data extraction and synthesis – eight papers were identified. Data were extracted by three researchers and constructs elicited via reciprocal translational analysis. Findings Themes reflected two domains of understanding an acute exacerbation: (1) Acute effect – encompassing intense emotions, somatic awareness and patients need for rescue. (2) Sustained regulation – reflecting life as interrupted, ongoing beliefs and behaviour and help-seeking. Discussion Acute exacerbations of chronic obstructive pulmonary disease result in heightened patient arousal, vigilance and powerlessness in response to symptoms. Ongoing, psychological interventions which promote partnership working by both acknowledging patients fear and shaping their appraisals may mitigate distress, enhancing the impact of health messages and engagement in pulmonary rehabilitation.
Altered cortical microarchitecture in patients with monoclonal gammopathy of undetermined significance
Authors: Farr Joshua N. , Zhang Wei , Kumar Shaji K. , Jacques Richard M. , Ng Alvin C. , McCready Louise K. , Rajkumar S. Vincent , Drake Matthew T. 
Published: November 2013

Key PointsMGUS patients have significantly increased cortical bone porosity and reduced bone strength relative to matched controls.
GATA2 deficiency: a protean disorder of hematopoiesis, lymphatics, and immunity
Authors: Spinner Michael A. , Sanchez Lauren A. , Hsu Amy P. , Shaw Pamela A. , Zerbe Christa S. , Calvo Katherine R. , Arthur Diane C. , Gu Wenjuan , Gould Christine M. , Brewer Carmen C. , Cowen Edward W. , Freeman Alexandra F. , Olivier Kenneth N. , Uzel Gulbu , Zelazny Adrian M. , Daub Janine R. , Spalding Christine D. , Claypool Reginald J. , Giri Neelam K. , Alter Blanche P. , Mace Emily M. , Orange Jordan S. , Cuellar-Rodriguez Jennifer , Hickstein Dennis D. , Holland Steven M. 
Published: November 2013

AbstractHaploinsufficiency of the hematopoietic transcription factor GATA2 underlies monocytopenia and mycobacterial infections; dendritic cell, monocyte, B, and natural killer (NK) lymphoid deficiency; familial myelodysplastic syndromes (MDS)/acute myeloid leukemia (AML); and Emberger syndrome (primary lymphedema with MDS). A comprehensive examination of the clinical features of GATA2 deficiency is currently lacking. We reviewed the medical records of 57 patients with GATA2 deficiency evaluated at the National Institutes of Health from January 1, 1992, to March 1, 2013, and categorized mutations as missense, null, or regulatory to identify genotype-phenotype associations. We identified a broad spectrum of disease: hematologic (MDS 84%, AML 14%, chronic myelomonocytic leukemia 8%), infectious (severe viral 70%, disseminated mycobacterial 53%, and invasive fungal infections 16%), pulmonary (diffusion 79% and ventilatory defects 63%, pulmonary alveolar proteinosis 18%, pulmonary arterial hypertension 9%), dermatologic (warts 53%, panniculitis 30%), neoplastic (human papillomavirus+ tumors 35%, Epstein-Barr virus+ tumors 4%), vascular/lymphatic (venous thrombosis 25%, lymphedema 11%), sensorineural hearing loss 76%, miscarriage 33%, and hypothyroidism 14%. Viral infections and lymphedema were more common in individuals with null mutations (P = .038 and P = .006, respectively). Monocytopenia, B, NK, and CD4 lymphocytopenia correlated with the presence of disease (P &lt; .001). GATA2 deficiency unites susceptibility to MDS/AML, immunodeficiency, pulmonary disease, and vascular/lymphatic dysfunction. Early genetic diagnosis is critical to direct clinical management, preventive care, and family screening.
Bendamustine-bortezomib-dexamethasone is an active and well-tolerated regimen in patients with relapsed or refractory multiple myeloma
Authors: Ludwig Heinz , Kasparu Hedwig , Leitgeb Clemens , Rauch Elisabeth , Linkesch Werner , Zojer Niklas , Greil Richard , Seebacher Adelheid , Pour Ludek , Weißmann Adalbert , Adam Zdeněk 
Published: November 2013

Key PointsBendamustine-bortezomib-dexamethasone is active and well tolerated in relapsed/refractory myeloma.
Analysis of Sry duplications on the Rattus norvegicus Y-chromosome
Authors: Prokop Jeremy W , Underwood Adam C , Turner Monte E , Miller Nic , Pietrzak Dawn , Scott Sarah , Smith Chris , Milsted Amy 
Published: November 2013

Abstract

Background
Gene copy number variation plays a large role in the evolution of genomes. In Rattus norvegicus and other rodent species, the Y-chromosome has accumulated multiple copies of Sry loci. These copy number variations have been previously linked with changes in phenotype of animal models such as the spontaneously hypertensive rat (SHR). This study characterizes the Y-chromosome in the Sry region of Rattus norvegicus, while addressing functional variations seen in the Sry protein products.

Results
Eleven Sry loci have been identified in the SHR with one (nonHMG Sry) containing a frame shift mutation. The nonHMGSry is found and conserved in the related WKY and SD rat strains. Three new, previously unidentified, Sry loci were identified in this study (Sry3BII, Sry4 and Sry4A) in both SHR and WKY. Repetitive element analysis revealed numerous LINE-L1 elements at regions where conservation is lost among the Sry copies. In addition we have identified a retrotransposed copy of Med14 originating from spliced mRNA, two autosomal genes (Ccdc110 and HMGB1) and a normal mammalian Y-chromosome gene (Zfy) in the Sry region of the rat Y-chromosome. Translation of the sequences of each Sry gene reveals eight proteins with amino acid differences leading to changes in nuclear localization and promoter activation of a Sry-responsive gene. Sry-β (coded by the Sry2 locus) has an increased cytoplasmic fraction due to alterations at amino acid 21. Sry-γ has altered gene regulation of the Sry1 promoter due to changes at amino acid 76.

Conclusions
The duplication of Sry on the Rattus norvegicus Y-chromosome has led to proteins with altered functional ability that may have been selected for functions in addition to testis determination. Additionally, several other genes not normally found on the Y-chromosome have duplicated new copies into the region around the Sry genes. These suggest a role of active transposable elements in the evolution of the mammalian Y-chromosome in species such as Rattus norvegicus.
Trends in chloroquine resistance marker, Pfcrt-K76T mutation ten years after chloroquine withdrawal in Tanzania
Authors: Mohammed Asia , Ndaro Arnold , Kalinga Akili , Manjurano Alphaxard , Mosha Jackline F , Mosha Dominick F , van Zwetselaar Marco , Koenderink Jan B , Mosha Frank W , Alifrangis Michael , Reyburn Hugh , Roper Cally , Kavishe Reginald A 
Published: November 2013

Abstract

Background

Plasmodium falciparum resistance to anti-malarial drugs remains a major obstacle to the control of malaria. In 2001 Tanzania replaced chloroquine (CQ) with sulphadoxine-pyrimethamine (SP) as first-line drug, which in turn was replaced by artemisinin combination therapy in 2006. SP has however, continued to be used in intermittent preventive treatment of malaria in pregnancy (IPTp) despite reports of high levels of resistance to SP due to the lack of alternatives to SP for IPTp. Recent reports have indicated recovery of CQ-susceptibility in Malawi, Kenya, Mozambique, and Tanzania based on the prevalence of wild types at codon 76 of the Pfcrt gene in indigenous P. falciparum populations. The current prevalence of this Pfcrt- 76 CQ resistance marker from six regions of Tanzania mainland is hereby reported.

Methods
DNA extracted from filter-paper dried blood spots and rapid diagnostics kit strips collected from finger-prick blood were used to genotype the Pfcrt-76 resistance marker using PCR-RFLP. Data from previously published studies were used to generate CQ susceptibility recovery trends using logistic regression model.

Results
Seven hundred and forty one (741) samples were genotyped. The current frequency of the CQ-susceptible Pfcrt-K76 was above 92% and did not differ between regions in Tanzania (χ 2 = 2.37; p = 0.795). The K76 allelic prevalence was between 85.7 and 93% in regions (χ 2 = 7.88, p = 0.163). The CQ resistance recovery trends showed regional variability that may be caused by differences in malaria transmission intensity, but overall the trends converge as the susceptibility levels in all regions approach >90%.

Conclusions
CQ withdrawal in Tanzania has resulted into >90% recovery of susceptibility in ten years of withdrawal. These findings are in support of the search for CQ-based combination drugs as a possible future alternative to SP for IPTp in places where full recovery of CQ-susceptibility will be evident.