bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical, electrophysiological, and molecular findings in early onset hereditary neuropathy with liability to pressure palsy
Authors: Potulska‐Chromik Anna , Sinkiewicz‐Darol Elena , Ryniewicz Barbara , Lipowska Marta , Kabzińska Dagmara , Kochański Andrzej , Kostera‐Pruszczyk Anna 
Published: March 2014

ABSTRACTIntroduction: The first episode of hereditary neuropathy with liability to pressure palsy (HNPP) in childhood is rare. Methods: We analyzed retrospectively the data of 7 patients with a deletion in PMP22 and onset of symptoms before age 18 years. Direct sequencing of the LITAF (lipopolysaccharide‐induced tumor necrosis factor) gene was performed in patients and family members. Results: Clinical presentations varied from mononeuropathies to brachial plexopathy, with recurrent episodes in 4 patients. Electrophysiological abnormalities characteristic for HNNP were found in most subjects. Analysis of the LITAF gene revealed an Ile92Val polymorphism in 6 of 7 (86%) probands and 5 of 7 (83%) family members, over 4 times greater frequency than in the general population. Conclusions: Clinical suspicion of HNPP even when nerve conduction study results do not fulfill HNPP criteria should indicate genetic testing. In our patients, early‐onset HNPP was associated frequently with isoleucine92valine LITAF polymorphism. Muscle Nerve 50: 914–918, 2014
Auf(zug) zu den Sternen
Authors: Waletzko Michael , Oppermann Raika 
Published: March 2014

AbstractLösen bald Kohlenstoffnanoröhren die Probleme der Raumfahrt? Ein Fahrstuhl ins Weltall an einem Kabel aus dem High‐Tech‐Material könnte die kostenintensive Raketentechnik ersetzen. Aber bislang ist es so, dass alle Stricke reißen …
Auf Resistenzen besser reagieren
Authors: Groß Michael 
Published: March 2014

AbstractDer Nachschub an neuartigen antibakteriellen Wirkstoffen versiegt, und es gibt immer mehr resistente Erreger. Falsche und unnötige Verwendung von Antibiotika verschärft das Problem. Eine neue Strategie im Kampf mit Infektionskrankheiten beruht vielleicht auf Viren, welche die Bakterien infizieren.
Conventional and molecular cytogenetic characterization of Burkitt lymphoma with bone marrow involvement in Brazilian children and adolescents
Authors: De Souza Mariana T. , Hassan Rocio , Liehr Thomas , Marques‐Salles Terezinha J. , Boulhosa Alice M. , Abdelhay Eliana , Ribeiro Raul C. , Silva Maria Luiza Macedo 
Published: March 2014

AbstractBackgroundBurkitt lymphoma/leukemia (BL/L) is cytogenetically characterized by the t(8;14)(q24;q32) or its variants, t(2;8)(p11;q21), and t(8;22)(q24;q11.2), which juxtapose the MYC oncogene to one of the three immunoglobulin loci. The overall cure rate of BL/L in children is 70–90%, but patients diagnosed with advanced‐stage disease have a less favorable prognosis. It is possible that secondary chromosomal abnormalities contribute to this unfavorable prognosis via chemotherapy resistance, but the results of genetic studies have been inconsistent. This study aimed to identify and characterize secondary chromosomal abnormalities associated with the t(8;14) and its variants in children with French‐American‐British‐L3 leukemia or Burkitt lymphoma with bone marrow involvement at the time of diagnosis.ProcedureChromosome analysis was based on G‐banding. Fluorescence in situ hybridization technique was applied using IGH/MYC/CEP8 dual‐fusion and MYC break‐apart probes. Multicolor chromosome banding was performed according to standard protocol.ResultsWe describe a group of BL/L with extreme adverse clinical outcome, in which secondary chromosomal abnormalities, particularly those involving the long arms of chromosomes 1 and 13, were found in 71% of cases. The IGH/MYC fusion showed molecular heterogeneity in 14% of cases and two cases exhibited three IGH/MYC fusion signals.ConclusionsSecondary chromosomal abnormalities were found in a high proportion of patients. We observed an extent of IGH/MYC heterogeneity not previously reported in Burkitt lymphoma, including the novel finding of three fusion signals in two cases. Pediatr Blood Cancer 2014; 61:1422–1426. © 2014 Wiley Periodicals, Inc.
Inflammatory activity at school age in very low birth weight bronchopulmonary dysplasia survivors
Authors: Korhonen Päivi H. , Suursalmi Piia H. , Kopeli Tarja , Nieminen Riina , Lehtimäki Lauri , Luukkaala Tiina , Korppi Matti , Saari Antti , Moilanen Eeva , Tammela Outi K.T. 
Published: March 2014

SummaryObjectiveAirway inflammation is involved in the pathogenesis of bronchopulmonary dysplasia (BPD). The aim of the study was to evaluate the inflammatory activity in plasma and exhaled air in very low birth weight (VLBW) BPD survivors at school age.MethodsTwenty‐one 6–14‐year‐old former VLBW (birth weight ≤1,500 g) children with severe radiographic BPD (radBPD), 19 without radBPD (nonBPD group) and 19 non‐asthmatic term controls underwent measurement of eosinophil cationic protein, IL‐6, IL‐8, adiponectin, adipsin, leptin, and resistin in plasma, leukotriene B4 and 8‐isoprostane in exhaled breath condensate, and NO in exhaled breath. Background data were obtained from patient records, clinical examination and parental questionnaire. Both univariate and multivariate models were applied in the statistical analysis.ResultsThere were no significant differences between the groups in any of the inflammatory markers measured. Five (25%) radBPD and 2 (11%) nonBPD children reported asthma (P = 0.058). In logistic regression analysis, exposure to chorioamnionitis was associated with low IL‐8 (OR 29.0, 95% CI 3.27–258) and postnatal corticosteroid therapy with high adiponectin (OR 32.0, 95% CI 1.29–793). High body mass index standard deviation score (BMI‐SDS) was associated with high plasma adipsin (OR 2.47, 95% CI 1.07–5.75) and leptin (OR 5.76, 95%CI 1.83–18.2) levels.ConclusionsThe inflammatory activity seems to decrease by school age in VLBW BPD survivors. Chorioamnionitis and postnatal corticosteroid treatment may modulate the inflammatory responsiveness in VLBW subjects even up to school age. The respiratory outcome in VLBW infants might be improved by preventing excessive weight gain. Pediatr Pulmonol. 2015; 50:683–690. © 2014 Wiley Periodicals, Inc.
Elevated IL‐8, TNF‐α, and MCP‐1 in men with metastatic prostate cancer starting androgen‐deprivation therapy (ADT) are associated with shorter time to castration‐resistance and overall survival
Authors: Sharma Jaya , Gray Kathryn P. , Harshman Lauren C. , Evan Carolyn , Nakabayashi Mari , Fichorova Raina , Rider Jennifer , Mucci Lorelei , Kantoff Philip W. , Sweeney Christopher J. 
Published: March 2014

BACKGROUNDChemokines and cytokines have been implicated in progression to castration‐resistant prostate cancer (CRPC).METHODSRetrospective data were accessed from 122 men with serum samples drawn at a median of 0.5 months after starting ADT for metastatic prostate cancer. MCP‐1, IL‐1‐β, IL‐2, IL‐8, IL‐6, and TNF‐α levels were measured by multiplex electrochemiluminescence assays. A multivariable Cox model assessed the association of time to CRPC and overall survival by the protein levels and adjusted for clinical variables (age and prostate specific antigen (PSA) levels at start of ADT, race, ECOG status, and extent of metastases). Associations were reported as hazard ratio (HR) with 95% confidence interval (CI).RESULTSMedian follow‐up and overall survival were 44 and 42.2 months, respectively. ECOG performance status (≥1 vs. 0) was negatively associated with overall survival [HR = 2.8 (1.1–7.0),P = 0.03], and PSA nadir < 0.2 was predictive of longer time to development of CRPC [HR = 0.3 (0.2–0.5),P < 0.0001]. The HR for time to CRPC by protein above the median was 1.4 (95% CI: 0.9, 2.2,P = 0.13) for IL‐8; 1.3 (95% CI: 0.8, 2,P = 0.18) for TNF‐α; 1.0 (95% CI: 0.7, 1.6,P = 0.95) for MCP‐1. The HR for median overall survival for protein levels above the median was: 1.9 (95% CI: 1.0, 3.5,P = 0.04) for IL‐8; 2.0 (95% CI: 1.1, 3.5,P = 0.02) for TNF‐α; 1.7 (95% CI: 1.7, 3.0,P = 0.08) for MCP‐1. There was no association with IL‐1‐β, IL‐2, or IL‐6.CONCLUSIONHigher levels of inflammation‐associated cytokines correlate with poorer prostate cancer outcomes and may guide strategies to improve prostate cancer therapy.Prostate 74:820–828, 2014. © 2014 Wiley Periodicals, Inc.
Performance analysis of an opportunistic multi‐user cognitive network with multiple primary users
Authors: Khan Fahd Ahmed , Tourki Kamel , Alouini Mohamed‐Slim , Qaraqe Khalid A. 
Published: March 2014

AbstractConsider a multi‐user underlay cognitive network where multiple cognitive users concurrently share the spectrum with a primary network with multiple users. The channel between the secondary network is assumed to have independent but not identical Nakagami‐m fading. The interference channel between the secondary users (SUs) and the primary users is assumed to have Rayleigh fading. A power allocation based on the instantaneous channel state information is derived when a peak interference power constraint is imposed on the secondary network in addition to the limited peak transmit power of each SU. The uplink scenario is considered where a single SU is selected for transmission. This opportunistic selection depends on the transmission channel power gain and the interference channel power gain as well as the power allocation policy adopted at the users. Exact closed form expressions for the moment‐generating function, outage performance, symbol error rate performance, and the ergodic capacity are derived. Numerical results corroborate the derived analytical results. The performance is also studied in the asymptotic regimes, and the generalized diversity gain of this scheduling scheme is derived. It is shown that when the interference channel is deeply faded and the peak transmit power constraint is relaxed, the scheduling scheme achieves full diversity and that increasing the number of primary users does not impact the diversity order. Copyright © 2014 John Wiley & Sons, Ltd.
Outcome of Tumor‐Specific Mesorectal Excision for Rectal Cancer: The Impact of Laparoscopic Resection
Authors: Mohamed Zakir K. , Law Wai Lun 
Published: March 2014

AbstractObjectiveThe study aimed to compare the outcomes of laparoscopic and open resection for rectal cancer in 1,063 consecutive cases in a single center.MethodsWe performed an analysis of 11 years of experience in rectal cancer surgery and compared the outcome of laparoscopic and open surgery. Multivariate and subgroup analysis was performed to look at the effect of the level of tumor and stage of disease on short‐term outcomes like conversion rate, anastomotic leak rate, length of stay, complication rate, 30‐day mortality, and long‐term outcomes like local recurrence and survival.ResultsA total of 1,063 patients underwent rectal resection with 470 (44.2 %) patients undergoing the laparoscopic approach. Groups were comparable in terms of age, sex, or co‐morbidities, and the operating time was longer in the laparoscopic group (210 vs. 150 min; p value < 0.001). A conversion rate of 6.8 % was noted, with an anastomotic leak rate of 3.87 % in the open group and 2.97 % in the laparoscopic group. The laparoscopic group had a lower blood loss (100 vs. 350 ml; p < 0.001), lower complication rates, and shorter length of stay (6 vs. 9 days). The local recurrence rate was comparable, and the laparoscopic approach had better overall and cancer‐specific survival, even after adjusting for stages. The laparoscopic approach was an independent factor associated with better overall and cancer‐specific survival on multivariate analysis.ConclusionWe confirmed the oncological safety of laparoscopic rectal cancer surgery. Laparoscopic surgery also showed superiority in the short‐term and long‐term outcomes of rectal cancer.
Amino acid supplementation enhances urokinase production by HT-1080 cells
Authors: Khaparde Shilpa S , Roychoudhury Pradip K 
Published: March 2014

Abstract
Medium optimization is an important strategy that can lead to several fold increase in the production of proteins in cell culture. However, the usual methods of medium optimization are complex and time consuming. Urokinase is a widely employed thrombolytic drug for the treatment of stroke. We describe here medium optimization for maximizing urokinase production by HT-1080 cells using supplementation of specific amino acids. The new specifically designed method resulted in 240 % increase in urokinase productivity.
Digit Replantation in Children: A Nationwide Analysis of Outcomes and Trends of 455 Pediatric Patients
Authors: Berlin Nicholas L. , Tuggle Charles T. , Thomson James G. , Au Alexander 
Published: March 2014

Background The short-term outcomes of pediatric digit replantation have been derived primarily from single-center/surgeon experience. The purpose of this study was to conduct a nationwide analysis of outcomes and trends of pediatric digit replantation as compared to adult patients. Methods Digit replantation patients were identified in the 1999–2011 Healthcare Cost and Utilization Project, Nationwide Inpatient Sample. Outcomes included in-hospital procedure-related and total complications, microvascular revision, amputation, and length of stay (LOS). Univariate and multivariate analyses were performed to compare pediatric and adult patients and to identify independent predictors of outcomes. The annual rate of replantation among pediatric digit amputation patients was evaluated over the study period. Results A total of 3,010 patients who underwent digit replantation were identified, including 455 pediatric patients. For all replantations, age ≤18 years was associated with a lower likelihood of suffering a total complication (odds ratio (OR) 0.66, P=0.006), requiring amputation (OR 0.62, P<0.001), and experiencing LOS >5 days (OR 0.77, P=0.019), after adjusting for comorbidity, amputation severity, digit type, number of replantations, and hospital characteristics. Similar associations were observed between patient age and replantation outcomes for single-finger replantations. The rate of pediatric replantation (range 16 to 27 %) remained consistent through the study period (incidence rate ratio 0.98, P=0.06). Conclusions The rate of pediatric replantation has been relatively low, being 27 % at most in a given year. Importantly, short-term outcomes are better in children than for adults, supporting the indication to perform replantation in this age group when the surgeon feels that replantation is feasible and safe.
A Simple Blind Tenolysis for Flexor Carpi Radialis Tendinopathy
Authors: Brink Peter R. G. , Franssen Bas B. G. M. , Disseldorp Dominique J. G. 
Published: March 2014

Background Flexor carpi radialis (FCR) tendinopathy is an entity with a chronic form (repetitiveness of work) and an acute form (acute overstretching of the wrist). Confirmation of this syndrome can be established by injection of a small amount of a local anesthetic in the sheet of the FCR at this tender point. Complete relieve of the symptoms after injection confirms the existence of a tendinopathy of the FCR. Whereas rest and/or local application of steroids do not have a persistent effect on the short term outcome, a tenolysis could be performed. Before performing a tenolysis underlying causes should be excluded or treated. Methods In this article a simple and save technique is described, using a small Beaver knife to open the osteofibrous tunnel of the flexor carpi radialis tendon, without opening the carpal tunnel. Results Relieve of complaints could be reached up to almost two third of all cases. Conclusion In cases in which non-operative treatment is not effective regarding FCR tendinopathy, a simple blind technique by opening the osteofibrous tunnel could be successful.
Targeted pancreatic cancer therapy with the small molecule drug conjugate SW IV‐134
Authors: Hashim Yassar M. , Spitzer Dirk , Vangveravong Suwanna , Hornick Mary C. , Garg Gunjal , Hornick John R. , Goedegebuure Peter , Mach Robert H. , Hawkins William G. 
Published: March 2014

Pancreatic adenocarcinoma is highly resistant to conventional therapeutics and has been shown to evade apoptosis by deregulation of the X‐linked and cellular inhibitors of apoptosis proteins (XIAP and cIAP). Second mitochondria‐derived activator of caspases (Smac) induces and amplifies cell death by reversing the anti‐apoptotic activity of IAPs. Thus, Smac‐derived peptide analogues (peptidomimetics) have been developed and shown to represent promising cancer therapeutics. Sigma‐2 receptors are overexpressed in many proliferating tumor cells including pancreatic cancer. Selected ligands to this receptor are rapidly internalized by cancer cells. These characteristics have made the sigma‐2 receptor an attractive target for drug delivery because selective delivery to cancer cells has the potential to increase therapeutic efficacy while minimizing toxicity to normal tissues.Here, we describe the initial characterization of SW IV‐134, a chemically linked drug conjugate between the sigma‐2 ligand SW43 and the Smac mimetic SW IV‐52 as a novel treatment option for pancreatic adenocarcinoma. The tumor killing characteristics of our dual‐domain therapeutic SW IV‐134 was far greater than either component in isolation or in an equimolar mix and suggests enhanced cellular delivery when chemically linked to the sigma‐2 ligand. One of the key findings was that SW IV‐134 retained target selectivity of the Smac cargo with the involvement of the NF‐κB/TNFα signaling pathway. Importantly, SW IV‐134 slowed tumor growth and improved survival in murine models of pancreatic cancer. Our data support further study of this novel therapeutic and this drug delivery strategy because it may eventually benefit patients with pancreatic cancer.
Context influences the processing of verb transitivity in French sentences: more evidence for semantic−syntax interactions
Authors: MAGNE CYRILLE , BESSON MIREILLE , ROBERT STÉPHANE 
Published: March 2014

abstractThe influence of semantic context on verb argument structure processing was investigated in two experiments using both ERP and behavioral measures. Participants were presented with sentences ending with syntactically and/or semantically congruous or incongruous noun phrases and they were asked to judge the overall acceptability of the sentences. Syntactically incongruous sentences contained an intransitive verb followed by a direct object (e.g., *L’ennemi a conspiré (INTR) un complot *‘The enemy conspired a scheme’). In line with our hypothesis, results showed that the processing of syntactic incongruities was influenced by the degree of semantic congruency between the different sentence constituents (strong in Experiment 1 and weak in Experiment 2). Thus, the same syntactic incongruity was processed differently depending upon the semantic context of the sentence, thereby demonstrating the influence of semantic context on syntactic processing. We propose a linguistic account of the differential effects of verb transitivity as a function of the semantic context based upon Cognitive Construction Grammar and Frame Semantics.
Increased severity of suicidal behavior in impulsive aggressive patients exposed to familial adversities
Authors: Lopez-Castroman J. , Jaussent I. , Beziat S. , Guillaume S. , Baca-Garcia E. , Genty C. , Olié E. , Courtet P. 
Published: March 2014

BackgroundThe mechanisms by which childhood abuse and family history of suicidal behavior (FHS) lead to an increased risk of suicidal behavior are still unknown. Impulsive aggression may play an intermediate role. We investigated whether greater scores for aggression and impulsivity might be associated with the effects of FHS and/or childhood abuse on the severity of suicidal behavior.MethodWe examined the scores of three scales measuring impulsive aggression in a sample of 696 suicide attempters. We compared the highest and lowest scores with regard to reports of childhood abuse and FHS using adjusted multinomial regression models. Genetic polymorphisms of the serotonergic system known to be associated with impulsive aggression were also analyzed.ResultsPatients with high impulsive aggressive scores showed significant differences in sociodemographic, clinical and suicidal features compared with patients with low impulsive aggressive scores. Adjusted results showed that combinations of some types of childhood abuse and FHS, particularly emotional abuse and emotional neglect, are associated with high impulsivity and hostility scores. The SS genotype of the serotonin transporter gene (5-HTTLPR) was associated with high levels of impulsivity when the subjects reported emotional abuse [odds ratio (OR) 5.55, 95% confidence interval (CI) 1.75–17.5] or physical abuse (OR 5.03, 95% CI 1.50–16.9) in their childhood.ConclusionsOur results support the role of impulsive aggression as one of the links that may connect childhood abuse and FHS with severity of suicidal behavior.
Stepped care treatment delivery for depression: a systematic review and meta-analysis
Authors: van Straten A. , Hill J. , Richards D. A. , Cuijpers P. 
Published: March 2014

BackgroundIn stepped care models patients typically start with a low-intensity evidence-based treatment. Progress is monitored systematically and those patients who do not respond adequately step up to a subsequent treatment of higher intensity. Despite the fact that many guidelines have endorsed this stepped care principle it is not clear if stepped care really delivers similar or better patient outcomes against lower costs compared with other systems. We performed a systematic review and meta-analysis of all randomized trials on stepped care for depression.MethodWe carried out a comprehensive literature search. Selection of studies, evaluation of study quality and extraction of data were performed independently by two authors.ResultsA total of 14 studies were included and 10 were used in the meta-analyses (4580 patients). All studies used screening to identify possible patients and care as usual as a comparator. Study quality was relatively high. Stepped care had a moderate effect on depression (pooled 6-month between-group effect size Cohen'sdwas 0.34; 95% confidence interval 0.20–0.48). The stepped care interventions varied greatly in number and duration of treatment steps, treatments offered, professionals involved, and criteria to step up.ConclusionsThere is currently only limited evidence to suggest that stepped care should be the dominant model of treatment organization. Evidence on (cost-) effectiveness compared with high-intensity psychological therapy alone, as well as with matched care, is required.
Marked, transient, emotion-triggered QT accentuation in an adolescent female with type 1 long QT syndrome
Authors: Anderson Heather N. , Medford Beth A. , Ackerman Michael J. 
Published: March 2014

AbstractType 1 long QT syndrome is the most common long QT syndrome genetic subtype. Exercise and emotional stress can precipitate sudden cardiac events in patients with type 1 long QT syndrome; however, the precise mechanism remains elusive. We report the case of a teenage girl with type 1 long QT syndrome secondary to a rare frameshift mutation (p. L191fs+90X) in the KCNQ1-encoded Kv7.1 potassium channel. During emotional distress, her continuous QTc recordings precipitously increased, peaking within minutes to 669 ms and then returning to baseline (520 ms) as she calmed without concomitant increase in heart rate. This is the first described case documenting transient, marked accentuation of the QTc interval in a long QT syndrome patient during emotional distress. Such events may be triggered by transient accentuation of the intrinsic perturbation in cardiac repolarisation and increase the risk of degeneration to a ventricular arrhythmia. This case illustrates the need improved understanding of the complex interaction between emotion and cardiac stability in patients with long QT syndrome.