bluuebunny/crossref_metadata_2025 huggingface dataset:
Solitary Peutz-Jeghers type hamartomatous polyps in the duodenum are not always associated with a low risk of cancer: two case reports
Authors: Sekino Yusuke , Inamori Masahiko , Hirai Mitsuru , Suzuki Kaori , Tsuzawa Kaoru , Akimoto Keiko , Takahata Ayako , Fujisawa Nobutaka , Saito Kumiko , Tsunemi Akisa , Tanaka Michio , Iida Hiroshi , Sakamoto Yasunari , Takahashi Hirokazu , Koide Tomoko , Tokoro Chikako , Abe Yasunobu , Nakajima Atsushi , Maeda Shin , Koyama Shigeru
Published: June 2011

Introduction

A hamartomatous polyp without associated mucocutaneous pigmentation or a family history of Peutz-Jeghers Syndrome is diagnosed as a solitary Peutz-Jeghers type hamartomatous polyp. As compared with Peutz-Jeghers Syndrome, Peutz-Jeghers type hamartomatous polyps are diagnosed with a lower risk of cancer and are regarded as a different disorder.

Case presentation

In case one, we describe an 84-year-old Japanese man with a 14 mm duodenal polyp. Endoscopic mucosal resection was performed and histological examination showed findings suggestive of a hamartomatous polyp with a focus of well-differentiated adenocarcinoma. In case two, we describe a 76-year-old Japanese man who had been treated for prostate, rectal and lung cancer. Upper gastrointestinal endoscopy revealed a duodenal polyp measuring 15 mm in diameter. Endoscopic mucosal resection was performed, and histological examination showed findings suggestive of a hamartomatous polyp. Liver and thyroid cancers were found after the endoscopic treatment.

Conclusion

Although duodenal solitary hamartomatous polyps are associated with a lower risk of cancer, four patients, including our cases, have been diagnosed with cancerous polyps. Patients with duodenal solitary hamartomatous polyps should be treated by endoscopic or surgical resection and need whole-body screening.
Arthritis, osteomyelitis, septicemia and meningitis caused by Klebsiella in a low-birth-weight newborn: a case report
Authors: Ghorashi Ziaaedin , Nezami Nariman , Hoseinpour-feizi Hamideh , Ghorashi Sona , Tabrizi Jafar Sadegh
Published: June 2011

Introduction

Klebsiella pneumoniaeis in most cases a hospital-acquired infection and presents as pneumonia, septicemia and meningitis in patients with some predisposing factors, including prematurity, intravenous catheter, history of antibiotic therapy and intravenous nutrients.

Case presentation

A low-birth-weight, 33-day-old Caucasian girl with respiratory distress syndrome was admitted to our hospital. She developed septicemia, meningitis, polyarticular arthritis and osteomyelitis by nosocomialK. pneumoniaewhich was resistant to most antibiotics except ciprofloxacin. She was therefore treated with ciprofloxacin and co-trimoxazole for eight weeks. After completion of the treatment course, she completely improved with excellent weight gain and without any adverse effects during three years of follow-up.

Conclusion

In the resistant strain ofK. pneumoniae, ciprofloxacin could be considered as a therapeutic option with the prospect of a good outcome, even in neonates and infants.
Antibodies toward infliximab are associated with low infliximab concentration at treatment initiation and poor infliximab maintenance in rheumatic diseases
Authors: Ducourau Emilie , Mulleman Denis , Paintaud Gilles , Miow Lin Delphine Chu , Lauféron Francine , Ternant David , Watier Hervé , Goupille Philippe
Published: June 2011

Introduction

A proportion of patients receiving infliximab have antibodies toward infliximab (ATI), which are associated with increased risk of infusion reaction and reduced response to treatment. We studied the association of infliximab concentration at treatment initiation and development of ATI as well as the association of the presence of ATI and maintenance of infliximab.

Methods

All patients with rheumatoid arthritis (RA) or spondyloarthritis (SpA) receiving infliximab beginning in December 2005 were retrospectively followed until January 2009 or until infliximab discontinuation. Trough serum infliximab and ATI concentrations were measured at each visit. The patients were separated into two groups: ATIpos if ATI were detected at least once during the follow-up period and ATIneg otherwise. Repeated measures analysis of variance was used to study the association of infliximab concentration at treatment initiation and the development of ATI. Maintenance of infliximab in the two groups was studied by using Kaplan-Meier curves.

Results

We included 108 patients: 17 with RA and 91 with SpA. ATI were detected in 21 patients (19%). The median time to ATI detection after initiation of infliximab was 3.7 months (1.7 to 26.0 months). For both RA and SpA patients, trough infliximab concentration during the initiation period was significantly lower for ATIpos than ATIneg patients. RA patients showed maintenance of infliximab at a median of 19.5 months (5.0 to 31.0 months) and 12.0 months (2.0 to 24.0 months) for ATIneg and ATIpos groups, respectively (P = 0.08). SpA patients showed infliximab maintenance at a median of 16.0 months (3.0 to 34.0 months) and 9.5 months (3.0 to 39.0 months) for ATIneg and ATIpos groups, respectively (P = 0.20). Among SpA patients, those who were being treated concomitantly with methotrexate had a lower risk of developing ATI than patients not taking methotrexate (0 of 14 patients (0%) vs. 25 of 77 patients (32%); P = 0.03).

Conclusions

High concentrations of infliximab during treatment initiation reduce the development of ATI, and the absence of ATI may be associated with prolonged maintenance of infliximab. Thus, trough serum infliximab concentration should be monitored early in patients with rheumatic diseases.
CLUE: a randomized comparative effectiveness trial of IV nicardipine versus labetalol use in the emergency department
Authors: Peacock W Frank , Varon Joseph , Baumann Brigitte M , Borczuk Pierre , Cannon Chad M , Chandra Abhinav , Cline David M , Diercks Deborah , Hiestand Brian , Hsu A , Jois-Bilowich Preeti , Kaminski Brian , Levy Philip , Nowak Richard M , Schrock Jon W
Published: June 2011

Introduction

Our purpose was to compare the safety and efficacy of food and drug administration (FDA) recommended dosing of IV nicardipine versus IV labetalol for the management of acute hypertension.

Methods

Multicenter randomized clinical trial. Eligible patients had 2 systolic blood pressure (SBP) measures ≥180 mmHg and no contraindications to nicardipine or labetalol. Before randomization, the physician specified a target SBP ± 20 mmHg (the target range: TR). The primary endpoint was the percent of subjects meeting TR during the initial 30 minutes of treatment.

Results

Of 226 randomized patients, 110 received nicardipine and 116 labetalol. End organ damage preceded treatment in 143 (63.3%); 71 nicardipine and 72 labetalol patients. Median initial SBP was 212.5 (IQR 197, 230) and 212 mmHg (IQR 200,225) for nicardipine and labetalol patients (P = 0.68), respectively. Within 30 minutes, nicardipine patients more often reached TR than labetalol (91.7 vs. 82.5%, P = 0.039). Of 6 BP measures (taken every 5 minutes) during the study period, nicardipine patients had higher rates of five and six instances within TR than labetalol (47.3% vs. 32.8%, P = 0.026). Rescue medication need did not differ between nicardipine and labetalol (15.5 vs. 22.4%, P = 0.183). Labetalol patients had slower heart rates at all time points (P < 0.01). Multivariable modeling showed nicardipine patients were more likely in TR than labetalol patients at 30 minutes (OR 2.73, P = 0.028; C stat for model = 0.72)

Conclusions

Patients treated with nicardipine are more likely to reach the physician-specified SBP target range within 30 minutes than those treated with labetalol.

Trial registration

ClinicalTrials.gov: NCT00765648
Affective disorders, anxiety disorders and the risk of alcohol
dependence and misuse
Authors: Liang Wenbin , Chikritzhs Tanya
Published: June 2011

Background

It is unclear whether common affective disorders and anxiety disorders
increase the risk of alcohol dependence and alcohol misuse.

Aims

To investigate whether affective disorders and anxiety disorders increase
the risk of alcohol dependence and alcohol misuse.

Method

This study is a retrospective cohort study based on data collected from
the 2007 Australia Mental Health and Well-Being survey. Both Poisson and
logistic regression models were used for multivariate analysis.

Results

Participants with affective disorders (relative risk (RR) = 5.46, 95% CI
4.08–7.31 for alcohol dependence within 5 years of onset; RR = 2.77, 95%
CI 1.93–3.99 after first 5 years) and anxiety disorders (RR = 3.33, 95%
CI 2.37–4.68 for alcohol dependence within first 5 years of onset; RR =
3.56, 95% CI 2.72–4.64 after first 5 years) were at higher risk of
alcohol misuse and alcohol dependence.

Conclusions

Common affective disorders and anxiety disorders may increase the risk of
alcohol dependence and alcohol misuse among the Australian
population.
Comprehensive Geriatric Assessment in the Decision-Making Process in Elderly Patients With Cancer: ELCAPA Study
Authors: Caillet Philippe , Canoui-Poitrine Florence , Vouriot Johanna , Berle Muriel , Reinald Nicoleta , Krypciak Sebastien , Bastuji-Garin Sylvie , Culine Stephane , Paillaud Elena
Published: June 2011

Purpose

To identify Comprehensive Geriatric Assessment (CGA) components independently associated with changes in planned cancer treatment.

Patients and Methods

We prospectively included 375 consecutive elderly patients with cancer (ELCAPA01 study) assessed by geriatricians using the CGA. Multivariate analysis was used to identify factors associated with changes in the cancer treatment (intensification, decrease, or delayed > 2 weeks). Change was defined as a difference between the initial treatment proposal and the final treatment selected in a multidisciplinary meeting.

Results

Mean age was 79.6 years (standard deviation [SD], 5.6 years), and 197 (52.5%) were women. The most common tumor location was the digestive system (58.7%). The mean number of comorbidities was 4.2 (SD, 2.7) per patient, and the mean Cumulative Illness Rating Scale for Geriatrics score was 11.8 (SD, 5.3). After the CGA, the initial cancer treatment plan was modified for 78 (20.8%) of 375 patients (95% CI, 16.8 to 25.3), usually to decrease treatment intensity (63 [80.8%] of 78 patients). By univariate analysis, cancer treatment changes were associated with Eastern Cooperative Oncology Group performance status ≥ 2 (73.3% in the group with changes v 41.1% in the in the group without changes; P < .001), dependency for one or more activities of daily living (ADL; 59.0% v 24.2%; P < .001), malnutrition (81.8% v 51.2%; P < .001), cognitive impairment (38.5% v 24.9%; P = .023), depression (52.6% v 21.7%; P < .001), and greater number of comorbidities (mean, 4.8 [SD, 2.9] v 4.0 [SD, 2.6]; P = .02). By multivariate analysis, factors independently associated with cancer treatment changes were a lower ADL score (odds ratio [OR], 1.25 per 0.5-point decrease; CI, 1.04 to 1.49; P = .016) and malnutrition (OR, 2.99; CI, 1.36 to 6.58; P = .007).

Conclusion

Functional status assessed by the ADL score and malnutrition were independently associated with changes in cancer treatment.
Calcium Plus Vitamin D Supplementation and the Risk of Nonmelanoma and Melanoma Skin Cancer: Post Hoc Analyses of the Women's Health Initiative Randomized Controlled Trial
Authors: Tang Jean Y. , Fu Teresa , LeBlanc Erin , Manson JoAnn E. , Feldman David , Linos Eleni , Vitolins Mara Z. , Zeitouni Nathalie C. , Larson Joseph , Stefanick Marcia L.
Published: June 2011

Purpose

In light of inverse relationships reported in observational studies of vitamin D intake and serum 25-hydroxyvitamin D levels with risk of nonmelanoma skin cancer (NMSC) and melanoma, we evaluated the effects of vitamin D combined with calcium supplementation on skin cancer in a randomized placebo-controlled trial.

Methods

Postmenopausal women age 50 to 79 years (N = 36,282) enrolled onto the Women's Health Initiative (WHI) calcium/vitamin D clinical trial were randomly assigned to receive 1,000 mg of elemental calcium plus 400 IU of vitamin D3 (CaD) daily or placebo for a mean follow-up period of 7.0 years. NMSC and melanoma skin cancers were ascertained by annual self-report; melanoma skin cancers underwent physician adjudication.

Results

Neither incident NMSC nor melanoma rates differed between treatment (hazard ratio [HR], 1.02; 95% CI, 0.95 to 1.07) and placebo groups (HR, 0.86; 95% CI, 0.64 to 1.16). In subgroup analyses, women with history of NMSC assigned to CaD had a reduced risk of melanoma versus those receiving placebo (HR, 0.43; 95% CI, 0.21 to 0.90; Pinteraction= .038), which was not observed in women without history of NMSC.

Conclusion

Vitamin D supplementation at a relatively low dose plus calcium did not reduce the overall incidence of NMSC or melanoma. However, in women with history of NMSC, CaD supplementation reduced melanoma risk, suggesting a potential role for calcium and vitamin D supplements in this high-risk group. Results from this post hoc subgroup analysis should be interpreted with caution but warrant additional investigation.
Role of American Society of Clinical Oncology in Low- and Middle-Income Countries
Authors: Patel Jyoti D. , Galsky Matthew D. , Chagpar Anees B. , Pyle Doug , Loehrer Patrick J.
Published: June 2011

The American Society of Clinical Oncology (ASCO) is a global community of health care professionals whose stated purpose is to “make a world of difference” by improving cancer care around the world. Unfortunately, cancer survival rates vary significantly among countries with differing financial and infrastructural resources. Because ASCO is a professional oncology society committed to conquering cancer through research, education, prevention, and delivery of high-quality patient care, it is ideally suited to address this issue. ASCO could bring together oncology professionals and other necessary stakeholders from around the world to improve cancer care and lessen suffering for patients worldwide.

As part of the ongoing commitment of ASCO to the future of cancer care, the Leadership Development Program was created to foster the leadership skills of early and midcareer oncologists and provide these participants with a working knowledge of the depth and breadth of the organization. As participants in the inaugural class of the ASCO Leadership Development Program, we were charged with investigating how ASCO might favorably affect cancer prevention and treatment in resource-poor countries in a cost-effective, scalable, and sustainable fashion. ASCO can significantly influence cancer care in low- and middle-income countries through a comprehensive approach that promotes cancer awareness and education, improves clinical practice by identifying and removing barriers to delivery of quality cancer care, and fosters innovation to initiate novel solutions to complex problems.
Zebrafish as a model for normal and malignant hematopoiesis
Authors: Jing Lili , Zon Leonard I.
Published: June 2011

Zebrafish studies in the past two decades have made major contributions to our understanding of hematopoiesis and its associated disorders. The zebrafish has proven to be a powerful organism for studies in this area owing to its amenability to large-scale genetic and chemical screening. In addition, the externally fertilized and transparent embryos allow convenient genetic manipulation and in vivo imaging of normal and aberrant hematopoiesis. This review discusses available methods for studying hematopoiesis in zebrafish, summarizes key recent advances in this area, and highlights the current and potential contributions of zebrafish to the discovery and development of drugs to treat human blood disorders.
Parkin’ control: regulation of PGC-1α through PARIS in Parkinson’s disease
Authors: Castillo-Quan Jorge Iván
Published: June 2011

Summary and comment on a recent Cell paper entitled ‘PARIS (ZNF746) repression of PGC-1α contributes to neurodegeneration in Parkinson’s disease’ (Shin et al., 2011).
Protein phosphatases and the regulation of mitosis
Authors: Barr Francis A. , Elliott Paul R. , Gruneberg Ulrike
Published: June 2011

Dynamic control of protein phosphorylation is necessary for the regulation of many cellular processes, including mitosis and cytokinesis. Indeed, although the central role of protein kinases is widely appreciated and intensely studied, the importance of protein phosphatases is often overlooked. Recent studies, however, have highlighted the considerable role of protein phosphatases in both the spatial and temporal control of protein kinase activity, and the modulation of substrate phosphorylation. Here, we will focus on recent advances in our understanding of phosphatase structure, and the importance of phosphatase function in the control of mitotic spindle formation, chromosome architecture and cohesion, and cell division.
Choice Inconsistencies among the Elderly: Evidence from Plan Choice in the Medicare Part D Program
Authors: Abaluck Jason , Gruber Jonathan
Published: June 2011

We evaluate the choices of elders across their insurance options under the Medicare Part D Prescription Drug plan, using a unique dataset of prescription drug claims matched to information on the characteristics of choice sets. We document that elders place much more weight on plan premiums than on expected out-of-pocket costs; value plan financial characteristics beyond any impacts on their own financial expenses or risk; and place almost no value on variance-reducing aspects of plans. Partial equilibrium welfare analysis implies that welfare would have been 27 percent higher if patients had all chosen rationally. (JEL D12, I11, J14)
Using Loopholes to Reveal the Marginal Cost of Regulation: The Case of Fuel-Economy Standards
Authors: Anderson Soren T , Sallee James M
Published: June 2011

Estimating the cost of regulation is difficult. Firms sometimes reveal costs indirectly, however, when they exploit loopholes to avoid regulation. We apply this insight to fuel economy standards for automobiles. These standards feature a loophole that gives automakers a bonus when they equip a vehicle with flexible-fuel capacity. Profit-maximizing automakers will equate the marginal cost of compliance using the loophole, which is observable, with the unobservable costs of strategies that genuinely improve fuel economy. Based on this insight, we estimate that tightening standards by one mile per gallon would have cost automakers just $9–$27 per vehicle in recent years. (JEL L51, L62, Q48)
The Inflation-Output Trade-Off with Downward Wage Rigidities
Authors: Benigno Pierpaolo , Antonio Ricci Luca
Published: June 2011

The macroeconomic implications of downward nominal wage rigidities are analyzed via a dynamic stochastic general equilibrium model featuring aggregate and idiosyncratic shocks. A closed-form solution for a long-run Phillips curve relates average output gap to average wage inflation: it is virtually vertical at high inflation and flattens at low inflation. Macroeconomic volatility shifts the curve outwards and reduces output. The results imply that stabilization policies play an important role, and that optimal inflation may be positive and differ across countries with different macroeconomic volatility. Results are robust to relaxing the wage constraint, for example, when large idiosyncratic shocks arise. (JEL E23, E24, E31, E63)
Ambiguity Models and the Machina Paradoxes
Authors: Baillon AurÉlien , L'Haridon Olivier , Placido Laetitia
Published: June 2011

Machina (2009) introduced two examples that falsify Choquet expected utility, presently one of the most popular models of ambiguity. This article shows that Machina's examples falsify not only the model mentioned, but also four other popular models for ambiguity of the literature, namely maxmin expected utility, variational preferences, α-maxmin, and the smooth model of ambiguity aversion. Thus, Machina's examples pose a challenge to most of the present field of ambiguity. Finally, the paper discusses how an alternative representation of ambiguity-averse preferences works to accommodate the Machina paradoxes and what drives the results. (JEL D81)
Fertility and the Personal Exemption: Comment
Authors: Crump Richard , Shah Goda Gopi , Mumford Kevin J
Published: June 2011

One of the most commonly cited studies on the effect of child subsidies on fertility, Whittington, Alm, and Peters (1990), claimed a large positive effect of child tax benefits on fertility using time series methods. We revisit this question in light of recent increases in child tax benefits by replicating this earlier study and extending the analysis. We do not find strong evidence to justify the model specification from the original paper. Moreover, even if the original specification is appropriate, we show that the results are not robust to more general measures of child tax benefits. (JEL H24, J13)
The Use of External Consultants by NHS Commissioners in England: What Lessons Can Be Drawn for GP Commissioning?
Authors: Naylor Chris , Goodwin Nick
Published: June 2011

Objectives

To describe the current use of external support by commissioners in the English NHS; assess what impact it is having; explore the factors influencing effectiveness; and draw out the implications for the future of NHS commissioning.

Methods

(1) Two national surveys of primary care trust managers. (2) Qualitative interviews and focus groups with representatives of 12 firms providing support services to commissioners. (3) Focus groups in three primary care trusts and interviews with representatives of two strategic health authorities (SHAs).

Results

The majority of survey respondents reported using external support (89% in 2010). Satisfaction levels were reasonably high, with most rating the service received as excellent or good. Perceived impact in the case study sites was mixed, but benefits were reported in a number of areas. There were cases where external support had not achieved its goals. Several factors contributed to success or failure, including difficulties around procuring external support; building effective working relationships; and implementing the ideas generated by external partners. There was evidence that commissioners do not always use support in a way that takes full advantage of the skills existing in external organizations. There was also some evidence that poorer performing organizations may be less successful in using external support effectively.

Conclusions

External support can play a role in improving the quality of commissioning. However, certain pitfalls must be avoided if it is to be used effectively. The role of external support will evolve (and may expand) as commissioning responsibilities are transferred to general practice consortia.
Indexicalities of language contact in an era of globalization: engaging with John Gumperz's legacy
Authors: Collins James
Published: June 2011

This article engages with John Gumperz's research legacy by examining his statements regarding indexicality at different stages in his career: the “Introduction” to
The dynamics of communicative practices in transmigrational contexts: ‘insulting remarks’ and ‘stylized category animations’ in everyday interactions among male youth in Germany
Authors: Günthner Susanne
Published: June 2011

This paper examines “insulting remarks” and “stylized category animations” as communicative practices in interactions among young men with a migrant background living in Germany. On the basis of a detailed investigation of informal interactions among young men in various German youth centers, I will show how the participants make use of these communicative practices as interactional resources to contextualize “belonging”/“association” versus “otherness” in transmigrational contexts. As part of their communicative household, these practices are closely connected to the construction of a cultural identity among these young men. I will argue that focusing on the development and dynamics of communicative practices can provide new insight into the workings of social and cultural identities as well as into linguistic diversity in modern societies.
Image Analysis Technique for Monitoring Early Age Shrinkage Strains with Depth from the Top Surface of Monolithic Cementitious Prism Specimens
Authors: Ong K. C. G. , Chandra L. R.
Published: June 2011

This paper describes the use of the image analysis technique for monitoring the variation of early age shrinkage strains with respect to the depth from the top surface of ordinary Portland cement mortar and concrete prism specimens starting as early as 30 min after adding water to the mixture. The applicability of the image analysis technique was demonstrated on both sealed and unsealed cementitious prism specimens. As expected, in situations where the top trowelled surface is protected from moisture loss, the shrinkage strains are similar with depth from the top trowelled surface. On the other hand, magnitudes of shrinkage strains monitored vary with depth when the top trowelled surface is exposed to a dry environment from an early age. The results also showed that the effect of settlements on early age shrinkage strains monitored can be minimized by adopting the current image analysis testing arrangement. Furthermore, when the early age shrinkage strains monitored using the image analysis technique were benchmarked against the results obtained via laser sensors, comparable shrinkage strains values were observed on the sealed prism specimen. On the other hand, the laser sensors were found to provide an “average” value of shrinkage strains that develop across the whole cross section of the unsealed prism specimen.
A Meta-analysis of Interventions That Target Children's Screen Time for Reduction
Authors: Maniccia Dayna M. , Davison Kirsten K. , Marshall Simon J. , Manganello Jennifer A. , Dennison Barbara A.
Published: June 2011

BACKGROUND:

Screen time, especially television viewing, is associated with risk of overweight and obesity in children. Although several interventions have been developed to reduce children's screen time, no systematic review of these interventions exists to date.

OBJECTIVE:

This is a systematic review and meta-analysis of interventions targeting a reduction in children's screen time.

METHODS:

Effect sizes and associated 95% confidence intervals (CIs) were calculated by using a random-effects model. Heterogeneity tests, moderator analyses, assessment of bias, and sensitivity analyses were conducted. Reliability was assessed with Cohen's κ.

RESULTS:

The systematic search identified 3002 documents; 33 were eligible for inclusion, and 29 were included in analyses. Most reported preintervention and postintervention data and were published in peer-reviewed journals. Although heterogeneity was present, no moderators were identified. Overall Hedges g (−0.144 [95% CI: −0.217 to −0.072]) and standard mean difference (SMD) (−0.148 [95% CI: −0.224 to −0.071]) indicated that interventions were linked with small but statistically significant reductions in screen time in children. The results were robust; the failsafe N was large, and the funnel plot and trim-and-fill methods identified few missing studies.

CONCLUSIONS:

Results show that interventions to reduce children's screen time have a small but statistically significant effect. As the evidence base expands, and the number of screen-time interventions increases, future research can expand on these findings by examining the clinical relevance and sustainability of effects, conducting a more thorough analysis of effect modifiers, and identifying critical components of effective interventions.
Mevalonate Kinase Deficiency: A Survey of 50 Patients
Authors: Bader-Meunier Brigitte , Florkin Benoit , Sibilia Jean , Acquaviva Cécile , Hachulla Eric , Grateau Gilles , Richer Olivier , Farber Claire Michèle , Fischbach Michel , Hentgen Véronique , Jego Patrick , Laroche Cécile , Neven Bénédicte , Lequerré Thierry , Mathian Alexis , Pellier Isabelle , Touitou Isabelle , Rabier Daniel , Prieur Anne-Marie , Cuisset Laurence , Quartier Pierre ,   on behalf of the SOFREMIP (Société Francophone pour la Rhumatologie et les Maladies Inflammatoires en Pédiatrie) and CRI (Club Rhumatismes et Inflammations)
Published: June 2011

OBJECTIVE:

The goal of this study was to describe the spectrum of clinical signs of mevalonate kinase deficiency (MKD).

METHODS:

This was a retrospective French and Belgian study of patients identified on the basis of MKD gene mutations.

RESULTS:

Fifty patients from 38 different families were identified, including 1 asymptomatic patient. Symptoms began during the first 6 months of life in 30 patients (60%) and before the age of 5 years in 46 patients (92%). Symptoms consisted of febrile diarrhea and/or rash in 23 of 35 patients (66%). Febrile attacks were mostly associated with lymphadenopathy (71%), diarrhea (69%), joint pain (67%), skin lesions (67%), abdominal pain (63%), and splenomegaly (63%). In addition to febrile attacks, 27 patients presented with inflammatory bowel disease, erosive polyarthritis, Sjögren syndrome, and other chronic neurologic, renal, pulmonary, endocrine, cutaneous, hematologic, or ocular symptoms. Recurrent and/or severe infections were observed in 13 patients, hypogammaglobulinemia in 3 patients, and renal angiomyolipoma in 3 patients. Twenty-nine genomic mutations were identified; the p.Val377Ile mutation was the most frequently found (29 of 38 families). Three patients died of causes related to MKD. The disease remained highly active in 17 of the 31 surviving symptomatic patients followed up for >5 years, whereas disease activity decreased over time in the other 14 patients. Interleukin 1 antagonists were the most effective biological agents tested, leading to complete or partial remission in 9 of 11 patients.

CONCLUSION:

MKD is not only an autoinflammatory syndrome but also a multisystemic inflammatory disorder, a possible immunodeficiency disorder, and a condition that predisposes patients to the development of renal angiomyolipoma.
Standard Terminology for Fetal, Infant, and Perinatal Deaths
Authors: Barfield Wanda Denise ,   the Committee on Fetus and Newborn
Published: June 2011

Accurately defining and reporting perinatal deaths (ie, fetal and infant deaths) is a critical first step in understanding the magnitude and causes of these important events. In addition to obstetric health care providers, neonatologists and pediatricians should know the current US definitions and reporting requirements for live births, fetal deaths, and infant deaths. Correct identification of these vital events will improve our local, state, and national data so that these deaths can be better addressed and reduced.
Validation of the STRIVE model for coupling ecological processes and surface water flow
Authors: De Doncker L. , Troch P. , Verhoeven R. , Buis K. , Meire P.
Published: June 2011

The 1D model package STRIVE is verified for simulating the interaction between ecological processes and surface water flow. The model is general and can be adapted and further developed according to the research question. The hydraulic module, based on the Saint-Venant equations, is the core part. The presence of macrophytes influences the water quality and the discharge due to the flow resistance of the river, expressed by Manning's coefficient, and allows an ecological description of the river processes. Based on the advection–dispersion equation, water quality parameters are incorporated and modelled. Calculation of the water quantity parameters, coupled with water quality and inherent validation and sensitivity analysis, is the main goal of this research. An important study area is the River Aa near Poederlee (Belgium), a lowland river with a wealth of vegetation growth, where discharge and vegetation measurements are carried out on a regular basis. The developed STRIVE model shows good and accurate calculation results. The work highlights the possibility of STRIVE to model flow processes, water quality aspects and ecological interaction combined and separately. Coupling of discharges, water levels, amount of biomass and tracer values provides a powerful prediction modelling tool for the ecological behaviour of lowland rivers.
Subsurface characterization using a D-optimality based pilot point method
Authors: Jung Yong , Ranjithan Ranji S. , Mahinthakumar G.
Published: June 2011

Detailed hydraulic conductivity estimation is a difficult problem as the number of direct measurements available at a typical field site is relatively few and sparse. A common approach to estimate hydraulic conductivity is to combine direct hydraulic conductivity measurements with secondary measurements such as hydraulic head and tracer concentrations in an inverse modeling approach. Even with secondary measurements this may constitute an underdetermined (or over-parameterized) inverse problem giving rise to ‘non-unique’ and incorrect estimates. One approach to reduce over-parameterization is to estimate hydraulic conductivity at a few carefully chosen points called ‘pilot points’ (i.e. reduction in parameter space). This paper develops a D-optimality based criterion method (DBM) for pilot point selection and tests its effectiveness for estimating hydraulic conductivity fields using several synthetic cases. Results show that the selected pilot points using this approach lead to a more accurate hydraulic conductivity characterization than either random or sequential pilot point location selection methods.
Impact of an incomplete solute mixing model on sensor network design
Authors: Romero-Gomez Pedro , Lansey Kevin E. , Choi Christopher Y.
Published: June 2011

Incomplete mixing models have recently been shown to better represent solute transport at junctions of pressurized water systems, compared to a complete mixing assumption. The present work incorporated an incomplete solute mixing model into a methodology for sensor network design. Water quality simulations conducted using both mixing models were carried out to generate pollution matrices that provided the input data for the set covering optimization formulation. Multiple contamination and detection scenarios were simulated by considering both a minimum hazard level of the contaminant and a maximum volume of contaminated water consumed. Examination and comparison of outcomes demonstrated that the water quality solver used may impact sensor network designs in three ways by altering: (i) the minimum number of monitoring stations required for full detection coverage, (ii) the optimal layout of stations over the water network and (iii) the detection domain of some stations.
Probabilistic streamflow forecasts based on hydrologic persistence and large-scale climate signals in central Texas
Authors: Wei Wenge , Watkins David W.
Published: June 2011

Skillful streamflow forecasts at seasonal lead times may be useful to water managers seeking to provide reliable water supplies and maximize system benefits. In this study, streamflow autocorrelation and large-scale climate information are used to generate probabilistic streamflow forecasts for the Lower Colorado River system in central Texas. A number of potential predictors are evaluated for forecasting flows in various seasons, including large-scale climate indices related to the El Niño/Southern Oscillation (ENSO), Pacific Decadal Oscillation (PDO), North Atlantic Oscillation (NAO) and others. Results indicate that, of the predictors evaluated, only hydrologic persistence and Pacific Ocean sea surface temperature patterns associated with ENSO and PDO provide forecasts which are statistically better than climatology. An ordinal polytomous logistic regression approach is proposed as a means of incorporating multiple predictor variables into a probabilistic forecast model. Forecast performance is assessed through a cross-validation procedure, using distribution-oriented metrics, and implications for decision making are discussed.
Synthesis and characterization of new complexes of some divalent transitional metals with isonicotinamido-4-chlorobenzalaldimine
Authors: Ababei Viorica , Kriza Angela , Andronescu Cristian , Musuc Magdalena
Published: June 2011

New complexes of isonicotinamido-4-chlorobenzalaldimine (INHCBA) with Cu(II),
Co(II) and Cd(II), having formula of the type [M(INHCBA)(ac)2]?xH2O (M =
Cu2+, x = 1, M = Co2+, x = 2, M = Cd2+, x = 0, ac = CH3COO-), and with
Cu(II), Mn(II) and Zn(II), having formula of the type
[M(INHCBA)2(H2O)2]SO4?xH2O (M = Cu2+, Mn2+, x = 2 and Zn2+ x = 2.5) were
synthesized and characterized by analytical and physico-chemical techniques,
i.e., elemental analyses, IR, UV-VIS- NIR and EPR spectroscopy as well as
thermal analysis and the determination of the molar conductivity and
magnetic moments. The thermal behaviour of complexes was studied by
thermogravimetry (TG), differential thermal analysis (DTA) and differential
scanning calorimetry (DSC). The ligand behaves as bidentate NO, being
coordinated through the azomethine nitrogen and carbonylic oxygen. The heats
of decomposition, ?H, associated with the exothermal effects, were also
determined.
Institutional Adaptation, Trust and Change in the German Political Economy
Authors: Allen Christopher S.
Published: June 2011

Henry Farrell, The Political Economy of Trust: Institutions, Interests and Interfirm Cooperation in Italy and Germany (New York: Cambridge University Press, 2009).

Jeremy Leaman, The Political Economy of Germany under Chancellors Kohl and Schroeder: Decline of the German Model? (New York: Berghahn, 2009)

Wolfgang Streeck, Re-Forming Capitalism: Institutional Change in the German Political Economy (New York: Oxford University Press, 2009)
Single Crystals of the Isotypic Series BaLu2Ch4 (Ch = S, Se and Te) with CaFe2O4-Type Structure
Authors: Schurz Christian M. , Schleid Thomas
Published: June 2011

Single crystals of ternary chalcogenides with the composition BaLu2Ch4 (Ch = S, Se and Te; orthorhombic, Pnma; a = 1211.4–1353.6, b = 395.6–438.5, c = 1427.8–1593.6 pm) could be obtained after attempts to synthesize ternary lutetium(III) nitride chalcogenides using the elements (Lu and Ch) along with BaN3Cl as a nitrogen source. Their crystal structures are isotypic with CaFe2O4 containing two sorts of {[LuChCh]8–} chains built up of edge-linked [(Lu1)(Ch2)(Ch3)3(Ch4)2]9– and [(Lu2)(Ch1)3(Ch2)2(Ch4)]9– octahedra, respectively. A further interconnection via the chalcogenide anions (Ch3)2– and (Ch1)2– leads to double chains, where either (Lu1)3+ or (Lu2)3+ coordinates these chalcogenide anions as well. The three-dimensional framework {[Lu2Ch4]2–} emerges from the corner-linkage of the two kinds of double chains forming large channels apt to take up the Ba2+ cations. These divalent cations exhibit eight contacts to chalcogenide anions resulting in the formation of bicapped trigonal prisms [BaCh8]14–.
Application of the EGM Method to a LED-Based Spotlight: A Constrained Pseudo-Optimization Design Process Based on the Analysis of the Local Entropy Generation Maps
Authors: Giangaspero Giorgio , Sciubba Enrico
Published: June 2011

In this paper, the entropy generation minimization (EGM) method is applied to an industrial heat transfer problem: the forced convective cooling of a LED-based spotlight. The design specification calls for eighteen diodes arranged on a circular copper plate of 35 mm diameter. Every diode dissipates 3 W and the maximum allowedtemperature of the plate is 80 °C. The cooling relies on the forced convection driven by a jet of air impinging on the plate. An initial complex geometry of plate fins is presented and analyzed with a commercial CFD code that computes the entropy generation rate. A pseudo-optimization process is carried out via a successive series of design modifications based on a careful analysis of the entropy generation maps. One of the advantages of the EGM method is that the rationale behind each step of the design process can be justified on a physical basis. It is found that the best performance is attained when the fins are periodically spaced in the radial direction.
Energy Use in Day Care Centers and Schools
Authors: Airaksinen Miimu
Published: June 2011

Typically the aim in the construction process is to calculate the energy, space and cost efficiency in the design phase. These factors’ influence on decision making extends to the whole building process. How these decisions affect the use of the building and user satisfaction as well as maintenance is still not that well understood. This study analyses different schools and day care centers and their energy as well as primary energy use. The buildings are located in southern Finland. Each building has had different objectives with respect to energy efficiency in the design phase. Our objective was to find out how those decisions made in the design and construction phase have influenced the overall energy performance of the building compared to existing building stock of similar building type. The results show that the studied buildings had lower thermal energy consumption compared to existing building stock. Thus the special attention in the design phase allowed achieving the desired goal. However, for the electricity consumption such a correlation could not be found. One of the reasons could be also different service level of buildings (more equipment). Also other quality values could not be compared since such data were not available from the existing building stock. As many earlier studies have indicated users have a high influence on the energy consumption. In the future, when feed-back from the users are obtained it will be interesting to analyze the results and compare what kind of influence that user behavior will have on the overall energy consumption of the studied buildings.
Delineating Chromosomal Breakpoints in Radiation-Induced Papillary Thyroid Cancer
Authors: Weier Heinz-Ulrich G. , Ito Yuko , Kwan Johnson , Smida Jan , Weier Jingly F. , Hieber Ludwig , Lu Chun-Mei , Lehmann Lars , Wang Mei , Kassabian Haig J. , Zeng Hui , O’Brien Benjamin
Published: June 2011

Recurrent translocations are well known hallmarks of many human solid tumors and hematological disorders, where patient- and breakpoint-specific information may facilitate prognostication and individualized therapy. In thyroid carcinomas, the proto-oncogenes RET and NTRK1 are often found to be activated through chromosomal rearrangements. However, many sporadic tumors and papillary thyroid carcinomas (PTCs) arising in patients with a history of exposure to elevated levels of ionizing irradiation do not carry these known abnormalities. We developed a rapid scheme to screen tumor cell metaphase spreads and identify candidate genes of tumorigenesis and neoplastic progression for subsequent functional studies. Using a series of overnight fluorescence in situ hybridization (FISH) experiments with pools comprised of bacterial artificial chromosome (BAC) clones, it now becomes possible to rapidly refine breakpoint maps and, within one week, progress from the low resolution Spectral Karyotyping (SKY) maps or Giemsa-banding (G-banding) karyotypes to fully integrated, high resolution physical maps including a list of candiate genes in the critical regions.
Prevalence of Alcohol Use and Associated Factors in Urban Hospital Outpatients in South Africa
Authors: Pengpid Supa , Peltzer Karl , Heever Hendry Van der
Published: June 2011

The aim of this study was to assess the prevalence of alcohol use and associated factors among outpatients in an urban hospital in South Africa. The sample included 1,532 (56.4% men and women 43.6%) consecutively selected patients from different hospital outpatient departments. Results indicate that 41.2% of men and 18.3% of women were found to be hazardous drinkers, and 3.6% of men and 1.4% of women meet criteria for probable alcohol dependence or harmful drinking as defined by the Alcohol Use Disorder Identification Test (AUDIT). Two in five patients (40.5%) were hazardous or harmful drinkers and/or had anxiety or depression. Logistic multiple regression found that for men tobacco use and not having been diagnosed with diabetes and for women tobacco use and having been diagnosed with migraine headache was associated with hazardous and harmful drinking. Although the study is cross-sectional, it does identify groups that may be at high risk of alcohol misuse and for whom intervention is urgent. Because prevalence of hazardous and harmful alcohol use is high in this population, routine screening should be introduced in hospital out-patient settings.
Molecular Cloning and Characterization of cDNA Encoding a Putative Stress-Induced Heat-Shock Protein from Camelus dromedarius
Authors: Elrobh Mohamed S. , Alanazi Mohammad S. , Khan Wajahatullah , Abduljaleel Zainularifeen , Al-Amri Abdullah , Bazzi Mohammad D.
Published: June 2011

Heat shock proteins are ubiquitous, induced under a number of environmental and metabolic stresses, with highly conserved DNA sequences among mammalian species. Camelus dromedaries (the Arabian camel) domesticated under semi-desert environments, is well adapted to tolerate and survive against severe drought and high temperatures for extended periods. This is the first report of molecular cloning and characterization of full length cDNA of encoding a putative stress-induced heat shock HSPA6 protein (also called HSP70B′) from Arabian camel. A full-length cDNA (2417 bp) was obtained by rapid amplification of cDNA ends (RACE) and cloned in pET-b expression vector. The sequence analysis of HSPA6 gene showed 1932 bp-long open reading frame encoding 643 amino acids. The complete cDNA sequence of the Arabian camel HSPA6 gene was submitted to NCBI GeneBank (accession number HQ214118.1). The BLAST analysis indicated that C. dromedaries HSPA6 gene nucleotides shared high similarity (77–91%) with heat shock gene nucleotide of other mammals. The deduced 643 amino acid sequences (accession number ADO12067.1) showed that the predicted protein has an estimated molecular weight of 70.5 kDa with a predicted isoelectric point (pI) of 6.0. The comparative analyses of camel HSPA6 protein sequences with other mammalian heat shock proteins (HSPs) showed high identity (80–94%). Predicted camel HSPA6 protein structure using Protein 3D structural analysis high similarities with human and mouse HSPs. Taken together, this study indicates that the cDNA sequences of HSPA6 gene and its amino acid and protein structure from the Arabian camel are highly conserved and have similarities with other mammalian species.
Keeping an Eye on Myocilin: A Complex Molecule Associated with Primary Open-Angle Glaucoma Susceptibility
Authors: Menaa Farid , Braghini Carolina Ayumi , Vasconcellos Jose Paulo Cabral De , Menaa Bouzid , Costa Vital Paulino , Figueiredo Eugênio Santana De , Melo Monica Barbosa De
Published: June 2011

MYOC encodes a secretary glycoprotein of 504 amino acids named myocilin. MYOC is the first gene to be linked to juvenile open-angle glaucoma (JOAG) and some forms of adult-onset primary open-angle glaucoma (POAG). The gene was identified as an up-regulated molecule in cultured trabecular meshwork (TM) cells after treatment with dexamethasone and was originally referred to as trabecular meshwork-inducible glucocorticoid response (TIGR). Elevated intraocular pressure (IOP), due to decreased aqueous outflow, is the strongest known risk factor for POAG. Increasing evidence showed that the modulation of the wild-type (wt) myocilin protein expression is not causative of glaucoma while some misfolded and self-assembly aggregates of mutated myocilin may be associated with POAG in related or unrelated populations. The etiology of the disease remains unclear. Consequently, a better understanding of the molecular mechanisms underlyingPOAG is required to obtain early diagnosis, avoid potential disease progression, and develop new therapeutic strategies. In the present study, we review and discuss the most relevant studies regarding structural characterizations, expressions, molecular interactions, putative functions of MYOC gene and/or its corresponding protein in POAG etiology.