bluuebunny/crossref_metadata_2025_split huggingface dataset:
The clinical presentation of mitochondrial diseases in children with progressive intellectual and neurological deterioration: a national, prospective, population‐based study
Authors: VERITY CHRISTOPHER M , WINSTONE ANNE MARIE , STELLITANO LESLEY , KRISHNAKUMAR DEEPA , WILL ROBERT , McFARLAND ROBERT 
Published: September 2009

Aim  Our aim was to study the clinical presentation, mode of diagnosis, and epidemiology of mitochondrial disorders in children from the UK who have progressive intellectual and neurological deterioration (PIND).Method  Since April 1997, we have identified patients aged 16 years or younger with suspected PIND through the monthly notification card sent to all UK consultant paediatricians by the British Paediatric Surveillance Unit. Clinical details obtained from reporting paediatricians are classified by an Expert Group.Results  By July 2008, 2493 cases of PIND had been reported, among which there were 112 children (69 males, 43 females) with mitochondrial diseases presenting between birth and 14 years 7 months (median 12mo), divided into 13 subgroups. In some instances, clinical features were characteristic of mitochondrial disease, but many children presented non‐specifically with combinations of developmental delay, hypotonia, failure to thrive, and seizures; 16 children had multisystem disease at presentation. Mortality was high: 40 children had died. Blood and/or cerebrospinal fluid lactate measurements were abnormal in 87 children, and 47 of 78 brain magnetic resonance images showed increased basal ganglia signal. Definite diagnoses were usually made by muscle enzyme or genetic studies.Interpretation  This is a unique population‐based study of the mitochondrial disorders that cause childhood neurodegenerative disease. It provides detailed information about the clinical presentation and investigation of these complex cases.
Mortality shifts in Caenorhabditis elegans: remembrance of conditions past
Authors: Wu Deqing , Rea Shane L. , Cypser James R. , Johnson Thomas E. 
Published: September 2009

SummaryThe analysis of age‐specific mortality can yield insights into how anti‐aging interventions operate that cannot be matched by simple assessment of longevity. Mortality, as opposed to longevity, can be used to assess the effects of an anti‐aging intervention on a daily basis, rather than only after most animals have died. Various gerontogene mutations in Caenorhabditis elegans have been shown to increase longevity as much as tenfold and to decrease mortality at some ages even more. Environmental alterations, such as reduced food intake (dietary restriction) and lower temperature also result in reduced mortality soon after the intervention. Here, we ask how soon anti‐aging interventions, applied during adult life, affect age‐specific mortality in nematodes. Using maximum likelihood analysis, we estimated the Gompertz parameters after shifts of temperature, and of food concentration and maintenance conditions. In separate experiments, we altered expression of age‐1 and daf‐16, using RNAi. Using about 44 000 nematodes in total, to examine daily mortality, we find that for both types of environmental shift, mortality responded immediately in the first assessment, while RNAi‐induced changes resulted in a slower response, perhaps due to delayed mechanics of RNAi action. However, under all conditions there is a permanent ‘memory’ of past states, such that the initial mortality component [a] of the Gompertz equation [μ(x) = aebx] bears a permanent ‘imprint’ of that earlier state. However, ‘b’ (the rate of mortality increase with age) is always specified by the current conditions.
Canine platelet transfusions
Authors: Callan Mary Beth , Appleman Elizabeth H. , Sachais Bruce S. 
Published: September 2009

AbstractObjective –To review potential platelet storage options, guidelines for administration of platelets, and adverse events associated with platelet transfusions.Data Sources –Data sources included original research publications and scientific reviews.Human Data Synthesis –Transfusion of platelet concentrates (PCs) plays a key role in the management of patients with severe thrombocytopenia. Currently PCs are stored at 22°C under continuous gentle agitation for up to 5 days. Chilling of platelets is associated with rapid clearance of transfused platelets, and galactosylation of platelets has proven unsuccessful in prolonging platelet survival. Although approved by the American Association of Blood Banks, cryopreservation of human platelets in 6% DMSO largely remains a research technique. Pre‐storage leukoreduction of PCs has reduced but not eliminated acute inflammatory transfusion reactions, with platelet inflammatory mediators contributing to such reactions.Veterinary Data Synthesis –Canine plateletpheresis allows collection of a concentrate with a high platelet yield, typically 3–4.5 × 1011versus <1 × 1011for whole blood‐derived platelets, improving the ability to provide sufficient platelets to meet the recipient's transfusion needs. Cryopreservation of canine platelets in 6% DMSO offers immediate availability of platelets, with an acceptable posttransfusion in vivo platelet recovery and half‐life of 50% and 2 days, respectively. While data on administration of rehydrated lyophilized platelets in bleeding animal models are encouraging, due to a short lifespan (min) posttransfusion, their use will be limited to control of active bleeding, without a sustained increase in platelet count.Conclusions –Fresh PC remains the product of choice for control of bleeding due to severe thrombocytopenia or thrombopathia. While cryopreservation and lyophilization of canine platelets offer the benefits of immediate availability and long‐term storage, the compromise is decreased in vivo recovery and survival of platelets and some degree of impaired function, though such products could still be life saving.
Use of thromboelastography in dogs with immune‐mediated hemolytic anemia: 39 cases (2000–2008)
Authors: Sinnott Virginia B. , Otto Cynthia M. 
Published: September 2009

AbstractObjective
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To analyze thromboelastograms (TEGs) of naturally occurring cases of immune‐mediated hemolytic anemia (IMHA) in order to identify whether a hypercoagulable state was present and whether its presence was associated with differences in survival.Design
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Retrospective study spanning January 2000 to June 2008. Medical records of dogs were evaluated. Endpoints were considered death or discharge from the hospital.Setting
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Academic teaching hospital.Animals
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Thirty‐nine dogs with a diagnosis of IMHA and at least one TEG performed during hospitalization were included.Interventions
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None.Measurements and Main Results
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Four values were evaluated from the TEG: the R time (R), K time (K), alpha angle (α), and maximum amplitude. From these values, a coagulation index (CI) was calculated to classify patients as normocoagulable, hypercoagulable, or hypocoagulable. Thirty‐three of 39 patients were hypercoagulable based on the CI. The 6 remaining dogs were normocoagulable. The patients with a normocoagulable CI had an increased mortality rate (100%) when compared with the hypercoagulable patients using Fisher's exact test (P=0.02). Additionally, prolongation of partial thromboplastin time did not preclude hypercoagulable TEG values.Conclusions
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The majority of dogs with IMHA were hypercoagulable as measured by TEG. A normal CI was associated with a worse outcome in this patient population. TEG may provide additional and complementary information to prothrombin time and partial thromboplastin time relating to coagulation status in dogs with IMHA and may help predict prognosis and potentially guide clinical decisions to utilize anticoagulant drugs.
Long‐Term Complication Rates and Survival of Peritoneal Dialysis Catheters: The Role of Percutaneous Versus Surgical Placement
Authors: Perakis Kostas E. , Stylianou Kostas G. , Kyriazis John P. , Mavroeidi Vasiliki N. , Katsipi Irene G. , Vardaki Eleftheria A. , Petrakis Ioannis G. , Stratigis Spyros , Kroustalakis Nikos G. , Alegakis Athanasios K. , Daphnis Eugene K. 
Published: September 2009

AbstractConsiderable controversy currently exists in the literature concerning the mode of catheter placement and its impact on the technical success of peritoneal dialysis (PD). We decided to compare the impact of the surgical versus the percutaneous insertion technique on peritoneal dialysis catheter (PDCs) complications and survival. Our study population comprised 152 patients in whom 170 PDCs were inserted between January 1990 and December 2007 at the main PD unit on the island of Crete. Eighty four catheters were surgically placed (S group) and 86 were placed percutaneously by nephrologists (N group). The total experience accumulated was 4997 patient‐months. The overall complications did not differ between the two groups. Only early leakage was more frequent in N group than S group (10.3 versus 1.9 episodes per 1000 patient‐months; p < 0.001). However, it was easily treated and did not constitute a cause of early catheter removal. Catheter survival was 91.1%, 80.7%, and 73.2%, in the S group versus 89.5%, 83.7%, and 83.7% for the N group at 1, 2, and 3 years, respectively (p = 0.2). Catheter survival has significantly increased over the last decade. Factors positively affecting PDC survival appeared to be the use of mupirocin for exit site care and the utilization of the coiled type of catheter, practices implemented mainly after 1999. Peritonitis‐free survival and patient survival were not associated with the mode of placement, while in Cox regression analysis, were longer in patients treated with automated PD. The placement mode did not affect PD outcomes. Percutaneous implantation proved a safe, simple, low cost, immediately available method for PDC placement and helped to expand our PD program.
Arterial Stent Placement in Arteriovenous Dialysis Access by Interventional Nephrologists
Authors: Asif Arif , Yevzlin Alexander S. 
Published: September 2009

AbstractArterial stenoses in patients with arteriovenous dialysis access can create a variety of problems including access dysfunction, thrombosis, and hand ischemia. While percutaneous balloon angioplasty is largely successful in the management of arterial stenoses, elastic recoil can present a real predicament to this treatment. In this report, we present two cases of arterial stenosis that demonstrated elastic recoil after angioplasty and required endovascular stent insertion. Both cases were treated successfully without any complications on an outpatient basis. This report describes arterial stent placement in patients with arteriovenous access and discusses anticoagulation considerations in such cases.
Scheduled Short‐Term Prevention With Frovatriptan for Migraine Occurring Exclusively in Association With Menstruation
Authors: Silberstein Stephen D. , Berner Todd , Tobin John , Xiang Qinfang , Campbell John C. 
Published: September 2009

Objective.— This post hoc subgroup analysis evaluated scheduled short‐term preventive frovatriptan therapy for women with migraine occurring exclusively in association with menstruation (occurring day −2 to +3; day 1 = menses start, no migraines outside this window).Background.— A previously published randomized, double‐blind, placebo‐controlled 3‐way crossover trial assessed the efficacy and safety of a scheduled 6‐day preventive regimen with frovatriptan for the treatment of menstrual migraine; the study population included women experiencing both menstrual and non‐menstrual migraine and women experiencing only menstrual migraine.Methods.— Women received each treatment (placebo, frovatriptan 2.5 mg once daily, and frovatriptan 2.5 mg twice daily) once over 3 perimenstrual periods in randomized sequence. For this subset analysis, screening questions were used to identify women with migraine occurring exclusively in association with menstruation. Efficacy was evaluated by occurrence and severity of migraine, functional impairment, and rescue medication use. Adverse events and tolerability were also assessed.Results.— Among 179 patients, the mean age (SD) was 37.3 (7.7) years and mean menstrual migraine history was 10.6 (8.7) years. Significantly fewer women experienced menstrual migraine during treatment with frovatriptan twice daily (37.7%, P < .001) or once daily (51.3%, P = .002) than during treatment with placebo (67.1%); a significant dose response was noted (P = .01, twice daily vs once daily). Significant treatment differences were also found for several secondary endpoints, but the data from this post hoc analysis must be interpreted with caution. Frovatriptan was well tolerated and most adverse events were mild or moderate and similar to those reported with the acute treatment of migraine with frovatriptan; the most common adverse events were nausea, dizziness, and headache.Conclusions.— Scheduled short‐term preventive frovatriptan therapy effectively reduced the occurrence of menstrual migraine in women with attacks occurring exclusively in association with menstruation.
Evaluation of Mechanical and Thermal Properties of Commonly Used Denture Base Resins
Authors: Phoenix Rodney D. , Mansueto Michael A. , Ackerman Neal A. , Jones Robert E. 
Published: September 2009

Purpose: The purpose of this investigation was to evaluate and compare the mechanical and thermal properties of 6 commonly used polymethyl methacrylate denture base resins.
Materials and Methods: Sorption, solubility, color stability, adaptation, flexural stiffness, and hardness were assessed to determine compliance with ADA Specification No. 12. Thermal assessments were performed using differential scanning calorimetry and dynamic mechanical analysis. Results were assessed using statistical and observational analyses.
Results: All materials satisfied ADA requirements for sorption, solubility, and color stability. Adaptation testing indicated that microwave‐activated systems provided better adaptation to associated casts than conventional heat‐activated resins. According to flexural testing results, microwaveable resins were relatively stiff, while rubber‐modified resins were more flexible. Differential scanning calorimetry indicated that microwave‐activated systems were more completely polymerized than conventional heat‐activated materials.
Conclusion: The microwaveable resins displayed better adaptation, greater stiffness, and greater surface hardness than other denture base resins included in this investigation. Elastomeric toughening agents yielded decreased stiffness, decreased surface hardness, and decreased glass transition temperatures.
Alveolar Distraction Osteogenesis: Revive and Restore the Native Bone
Authors: Sant Sumedha , Jagtap Amit 
Published: September 2009

AbstractIn prosthodontics, knife‐edge bony alveolar ridges can cause a problem in their rehabilitation. The distraction osteogenesis process raises the medullary component of the alveolus, allowing the labial plate of the existing natural bone to be displaced. This process involves mobilization, transport, and fixation of a healthy segment of bone adjacent to the deficient site. It entails use of the gradual controlled displacement of surgically created fractures, which results in simultaneous expansion of soft tissue and bone volume. A mechanical device, the alveolar distraction device, is used for this purpose. This modality of treatment can be used in implant dentistry cases for rehabilitation of resorbed ridges. The objective of this overview is to explain this procedure wherein the alveolar housing, including the osseous and soft‐tissue components, is enlarged in a single, simultaneous process, which makes creation of an appropriate alveolar morphology possible.
Cycle Time in Packaged Software Firms
Authors: Carmel Erran 
Published: September 2009

Reduction of cycle time (i.e., time to market) is a fundamental competitive strategy in many industries. With the current proliferation of personal computer (PC) technology, software developers face intense competition. However, unlike their counterparts in other consumer goods markets, product development managers in the software industry appear to be less concerned with (or even aware of) cycle time than they are with other competitive variables.To explore the role of cycle‐time reduction in the process of developing software packages, Erran Carmel conducted a study of 15 software package companies in the Washington‐Baltimore metropolitan area. The survey results indicate that software package developers are generally unaware of cycle‐time reduction as a management concept. Instead, software developers tend to focus on rapid development, with an emphasis on “crunch” periods of intense effort aimed at meeting a deadline. During these periods of peak activity, 87% of the developers in core teams worked more than 56 hours per week and 47% worked more than 71 hours per week.In terms of the organizational variables necessary for cycle‐time reduction, all of the survey respondents point to the importance of a small, cohesive, core development team, similar to a cross‐functional team. Members of the core team are entrepreneurial and share a common vision of the product's design, use, and long‐term direction.As for the development variables associated with cycle‐time reduction, the software companies in the survey typically do not use process models or risk analysis techniques. Similarly, they devote scant resources to automated tools. For the majority of the firms in the survey, annual investment in automated software development tools is less than $1,000 per developer. On the other hand, reuse (as embodied in object‐oriented design and development) and incremental innovation are important to all of the sample firms.Although quality assurance (QA) activities are not addressed in the innovation literature, QA is a significant cycle‐time component in software development. As demonstrated by the industry practice of releasing products with long lists of known defects, the software product category clearly has quality problems. With the current boom in new users, pressure will grow for improved quality. To remain competitive, software developers need to determine how they can better integrate QA activities into the development process while reducing cycle time.
A new locus for hereditary hypotrichosis simplex maps to chromosome 13q12.12∼12.3 in a Chinese family
Authors: Xu Chao , Zhang Li , Chen Nan , Su Bin , Pan Cun‐Ming , Li Jun‐Yan , Zhang Guo‐Wei , Liu Zhi , Sheng Yan , Song Huai‐Dong 
Published: September 2009

Background: Hereditary hypotrichosis simplex (MIM 146520, HHS) is a rare form of nonsyndromic alopecia. The locus for autosomal dominant HHS was mapped to 18p11.32‐p11.23 and 6p21.3, respectively, suggestive of genetic heterogeneity. Aim: To identify the disease‐causing gene for a four‐generation Chinese family with dominant transmission of a form of HHS. The work was carried out at State Key Laboratory of Medical Genomics. Methods: Genome‐wide screening was carried out in a Chinese family with HHS using microsatellite markers, and linkage analysis was performed using the MLINK program. Results: The highest two‐point logarithm of the odds (LOD) score was obtained with the microsatellite marker D13S217 (LOD score of 4.041 at θ = 0.00). After fine mapping and haplotype analysis, we defined a critical region of about 9.57 cM flanked by markers D13S1243 and D13S1299. The disease‐causing gene was mapped to 13q12.12∼12.3 in this family. Conclusions: A novel locus for HHS maps to chromosome 13q12.12∼12.3 in a Chinese family.Xu C, Zhang L, Chen N, Su B, Pan C‐M, Li J‐Y, Zhang G‐W, Liu Z, Sheng Y, Song H‐D. A new locus for hereditary hypotrichosis simplex maps to chromosome 13q12.12∼12.3 in a Chinese family.
Photopheresis in pediatric patients with low‐body weight using the UVAR® XTS™ system
Authors: Hillen Uwe , Meyer Sabine , Schadendorf Dirk , Kremens Bernhard 
Published: September 2009

Summary Background: Photopheresis (ECP) with the UVAR® XTS™ system has been limited to patients with a body weight > 40 kg, because extracorporeal blood volumes (ECV) may exceed 15% of the total blood volumes in low‐body‐weight patients. Because of these instrument characteristics, the use of ECP is limited in pediatric patients. Patients and methods: In 5 patients (age 4 to 15 years) with graft‐versus‐host disease and with a body weight between 13 and 34 kilograms, photopheresis was performed using the UVAR® XTS™ system. In 3 patients ECP could be performed by balancing the ECV with saline infusions. In 2 patients the system was modified in that the instrument was primed with packed red cells. During the treatment, fluid balance was guaranteed by a reservoir from a transfusion bag connected in parallel. Results: Together 223 procedures were performed, among them 28 with the modification of the system. The treatments were well tolerated, and no episodes of hypotension were observed. Conclusions: It is possible to run ECP in low‐body weight patients with the UVAR® XTS™ system.
Willingness of Nurses to Report Medication Administration Errors in Southern Taiwan: A Cross‐Sectional Survey
Authors: Lin Yu‐Hua , Ma Su‐mei 
Published: September 2009

ABSTRACT Background: Underreporting of medication administering errors (MAEs) is a threat to the quality of nursing care. The reasons for MAEs are complex and vary by health professional and institution. Aims: The purpose of this study was to explore the prevalence of MAEs and the willingness of nurses to report them. Methods: A cross‐sectional study was conducted involving a survey of 14 medical surgical hospitals in southern Taiwan. Nurses voluntarily participated in this study. A structured questionnaire was completed by 605 participants. Data were collected from February 1, 2005 to March 15, 2005 using the following instruments: MAEs Unwillingness to Report Scale, Medication Errors Etiology Questionnaire, and Personal Features Questionnaire. One additional question was used to identify the willingness of nurses to report medication errors: “When medication errors occur, should they be reported to the department?” This question helped to identify the willingness or lack thereof, to report incident errors. Results: The results indicated that 66.9% of the nurses reported experiencing MAEs and 87.7% of the nurses had a willingness to report the MAEs if there were no consequences for reporting. The nurses' willingness to report MAEs differed by job position, nursing grade, type of hospital, and hospital funding. The final logistic regression model demonstrated hospital funding to be the only statistically significant factor. The odds of a willingness to report MAEs increased 2.66‐fold in private hospitals (p = 0.032, CI = 1.09 to 6.49), and 3.28 in nonprofit hospitals (p = 0.00, CI = 1.73 to 6.21) when compared to public hospitals. Conclusions: This study demonstrates that reporting of MAEs should be anonymous and without negative consequences in order to monitor and guide improvements in hospital medication systems.