Nature and nurture: Williams syndrome across cultures.

This study is concerned with ways in which children with Williams syndrome (WS), a rare neurodevelopmental disorder arising from a hemizygous deletion in chromosome band 7q11.23 including the gene for elastin (ELN) and approximately 20 surrounding genes, are affected by social mores of vastly differing cultures: the United States and Japan. WS presents a compelling model for the investigation because its genetic phenotype is well defined and results in an uneven cognitive profile as well as a social phenotype typical of the syndrome including overt over-friendliness toward strangers.

Comparative analysis of in vivo and in vitro AAV vector transduction in the neonatal mouse retina: effects of serotype and site of administration.

The specificity of retinal cells transduced by AAV serotype 1, 2 or 5 vectors was determined in vivo versus in vitro in the normal P7 mouse in order to develop a rapid and accurate way to anticipate the behavior of AAV vectors in the retina. In vivo results confirm that AAV1 transduces retinal pigment epithelial cells, while AAV2 and AAV5 transduce both RPE and photoreceptor cells by subretinal injection. AAV2 was the only serotype to efficiently transduce inner retinal cells by intravitreal injection.

Affective state control for neuroprostheses.

The control and communication in man and the machine has been an active area of research since the early 1940's and since then the usage of the computing machine for the enhancement, augmentation, and rehabilitation of mankind has been broadly investigated. One active area of such research is the interface of the human brain to the computer; brain-computer-interfacing (BCI) or neuroprostheses. Current examples of functional BCI typically control the computer screen cursor movement, but require extensive subject training and significant, if not full, cognitive focus.

Complications of open abdominal aortic surgery: the endovascular solution.

Objectives: Aorto-enteric fistulas (AEFs) and para-anastomotic aneurysms (PAAs) are uncommon complications of open aortic surgery (0.5-2.5%) and (0.

Potential functions of retinoic acid receptor A in Sertoli cells and germ cells during spermatogenesis.

Elucidation of the retinoid signaling circuitry in the testis is critical to understanding how male germ cells develop to spermatozoa. Retinoic acid receptor A protein (RARA) is an essential mediator of retinoid signaling in the testis, as shown by a sterility phenotype observed for retinoic acid receptor A gene (Rara) knockout male mice. The seminiferous tubules of Rara knockout mice showed varying degrees of germ-cell degeneration.

Comparison of the relative analgesic efficacies of epidural or intramuscular diamorphine following total knee arthroplasty.

Background And Objectives: Debate has proliferated as to the true site of action of opioids when placed in the epidural space. The aim of this study was to compare the analgesic effects of a bolus of diamorphine given by the epidural or intramuscular route.

Methods: Sixty patients having elective primary total knee replacements were recruited and randomized to receive epidural or intramuscular diamorphine.

A Novel indole compound that inhibits Pseudomonas aeruginosa growth by targeting MreB is a substrate for MexAB-OprM.

Drug efflux systems contribute to the intrinsic resistance of Pseudomonas aeruginosa to many antibiotics and biocides and hamper research focused on the discovery and development of new antimicrobial agents targeted against this important opportunistic pathogen. Using a P. aeruginosa PAO1 derivative bearing deletions of opmH, encoding an outer membrane channel for efflux substrates, and four efflux pumps belonging to the resistance nodulation/cell division class including mexAB-oprM, we identified a small-molecule indole-class compound (CBR-4830) that is inhibitory to growth of this efflux-compromised strain.

Interstitial pneumonitis in Blau syndrome with documented mutation in CARD15.

This is the first report of a CARD15 mutation-positive patient with Blau syndrome who exhibited interstitial lung disease, a feature historically considered absent from Blau syndrome, while typical of the adult form of sarcoidosis. This case illustrates the continued evolution of the phenotype of a disease initially conceived as a familial inflammatory granulomatous disease limited to the triad of synovitis, dermatitis, and uveitis.

Sap flow characteristics of neotropical mangroves in flooded and drained soils.

Effects of flooding on water transport in mangroves have previously been investigated in a few studies, most of which were conducted on seedlings in controlled settings. In this study, we used heat-dissipation sap probes to determine if sap flow (J(s)) attenuates with radial depth into the xylem of mature trees of three south Florida mangrove species growing in Rookery Bay. This was accomplished by inserting sap probes at multiple depths and monitoring diurnal flow.

Eccentric utilization ratio: effect of sport and phase of training.

The eccentric utilization ratio (EUR), which is the ratio of countermovement jump (CMJ) to static jump (SJ) performance, has been suggested as a useful indicator of power performance in athletes. The purpose of the study was to compare the EUR of athletes from a variety of different sports and during different phases of training. A total of 142 athletes from rugby union, Australian Rules Football, soccer, softball, and field hockey were tested.

Retinoic acid regulation of eye and testis-specific transcripts within a complex locus.

We previously used a yeast-based enhancer trap to identify a strong, retinoic acid response element (RARE). We have now characterized testis and eye transcripts that are adjacent to this regulatory element. Bioinformatics analysis of expressed sequence tag (EST) clones and RNase protection, reverse transcription-PCR, and Northern blot assays indicate that these two RNAs are transcribed from the same locus on opposite template strands.

Further exploration of antimicrobial ketodihydronicotinic acid derivatives by multiple parallel syntheses.

A synthetic reexamination of a series of ketodihydronicotinic acid class antibacterial agents was undertaken in an attempt to improve their therapeutic potential. A convenient new synthesis was developed involving hetero Diels-Alder chemistry producing 74 new analogs in a multiple parallel synthetic manner and these were examined in vitro for their antimicrobial potential. Several compounds demonstrated significant broad-spectrum activity against clinically derived bacterial strains but previously known 1-(2,4-difluorophenyl)-6-(4-dimethylaminophenyl)-4-pyridone-3-carboxylic acid (7) remained the most potent compound in this class.

Integrating simulation training into the nursing curriculum.

The use of simulation is gaining momentum in nurse education across the UK. The Nursing and Midwifery Council is currently investigating the use of simulation in pre-registration nursing. This article gives a brief history of simulation, discusses competence issues and why simulation is best placed to teach nurses in today's health service.

New C25 carbamate rifamycin derivatives are resistant to inactivation by ADP-ribosyl transferases.

A novel series of 3-morpholino rifamycins in which the C25 acetate group was replaced by a carbamate group were prepared and found to exhibit significantly improved antimicrobial activity than rifampin against Mycobacterium smegmatis. Further characterization of such compounds suggests that relatively large groups attached to the rifamycin core via a C25 carbamate linkage prevent inactivation via ribosylation of the C23 alcohol as catalyzed by the endogenous rifampin ADP-ribosyl transferase of M. smegmatis.

Superoxide, peroxynitrite and oxidative/nitrative stress in inflammation.

A considerable body of evidence suggests that formation of potent reactive oxygen species and resulting oxidative/nitrative stress play a major role in acute and chronic inflammation and pain. Much of the knowledge in this field has been gathered by the use of pharmacological and genetic approaches. In this mini review, we will evaluate recent advances made towards understanding the roles of reactive oxygen species in inflammation, focusing in particular on superoxide and peroxynitrite.

Pediatric granulomatous arthritis: an international registry.

Objective: Blau syndrome and its sporadic counterpart, early-onset sarcoidosis, share an identical phenotype featuring the classic triad of arthritis, dermatitis, and uveitis and are associated with mutations of CARD15 in 50-90% of cases. We chose the term "pediatric granulomatous arthritis" to refer to both. An international registry was established in the spring of 2005 to define the phenotype spectrum and establish the mutation frequency and variants.

Jellyfish aggregations and leatherback turtle foraging patterns in a temperate coastal environment.

Leatherback turtles (Dermochelys coriacea) are obligate predators of gelatinous zooplankton. However, the spatial relationship between predator and prey remains poorly understood beyond sporadic and localized reports. To examine how jellyfish (Phylum Cnidaria: Orders Semaeostomeae and Rhizostomeae) might drive the broad-scale distribution of this wide ranging species, we employed aerial surveys to map jellyfish throughout a temperate coastal shelf area bordering the northeast Atlantic.

Endovascular repair of a recurrent aortocaval fistula and anastamotic false aneurysm.

Aortocaval fistula (ACF) and false aneurysm are a recognized complication of open abdominal aortic aneurysm (AAA) repair. Untreated they are often fatal. However, open surgical repair of this complication is associated with a high operative mortality and a significant complication rate.

An evaluation of a new test of reactive agility and its relationship to sprint speed and change of direction speed.

The purpose of this study was to evaluate the reliability and validity of a new test of agility, the reactive agility test (RAT), which included anticipation and decision-making components in response to the movements of a tester. Thirty-eight Australian football players took part in the study, categorized into either a higher performance group (HPG) (n=24) or lower performance group (LPG) (n=14) based on playing level from the previous season. All participants undertook testing of a 10m straight sprint (10mSS), a 8-9m change of direction speed test (CODST), and the RAT.

Summary of notifiable diseases--United States, 2004.

The Summary of Notifiable Diseases--United States, 2004 contains the official statistics, in tabular and graphic form, for the reported occurrence of nationally notifiable infectious diseases in the United States for 2004. Unless otherwise noted, the data are final totals for 2004 reported as of December 2, 2005. These statistics are collected and compiled from reports sent by state health departments to the National Notifiable Diseases Surveillance System (NNDSS), which is operated by CDC in collaboration with the Council of State and Territorial Epidemiologists (CSTE).

Development of the MAGIC congenital heart disease catheterization database for interventional outcome studies.

As the field of catheter-based therapies for congenital heart disease continues to expand, we lack the evidence-based data to make appropriate therapeutic decisions in the catheterization laboratory. A stumbling block to the determination of evidence-based therapies is our inability to simply and reliably share outcome data across multiple centers. We investigated whether a commonly used congenital heart disease catheterization database program (PedCath) could be used as an automatic catheterization data submission tool to a central database for outcome analysis.

Leaf gas exchange characteristics of three neotropical mangrove species in response to varying hydroperiod.

We determined how different hydroperiods affected leaf gas exchange characteristics of greenhouse-grown seedlings (2002) and saplings (2003) of the mangrove species Avicennia germinans (L.) Stearn., Laguncularia racemosa (L.