Benchtop Zone Refinement of Simulated Future Spent Nuclear Fuel Pyroprocessing Waste.

The UK's adoption of pyroprocessing of spent nuclear fuel as an alternative to the current aqueous processing routes requires a robust scientific underpinning of all relevant processes. One key process is the clean-up of the contaminated salt from the electroreducing and electrorefining processes. A proposed method for this clean-up is zone refining, whereby the tendency of the contaminants to remain in the liquid phase during melting and freezing is exploited to 'sweep' the contaminants to one end of the sample.

Clinical, genetic, epidemiologic, evolutionary, and functional delineation of -related autosomal recessive ectodermal dysplasia 14.

variants cause autosomal recessive ectodermal dysplasia (ARED) 14. The function of TSPEAR is unknown. The clinical features, the mutation spectrum, and the underlying mechanisms of ARED14 are poorly understood.

Macrodontia and double teeth: a review and case series.

Macrodontia is a dental anomaly pertaining to the increased size of a tooth or multiple teeth. Double teeth are dental abnormalities concerning tooth morphology and the term traditionally refers to geminated or fused teeth. These anomalies may manifest in both primary and permanent dentitions and usually become apparent in childhood.

A three-generation family with metaphyseal dysplasia, maxillary hypoplasia and brachydactyly (MDMHB) due to intragenic RUNX2 duplication.

Metaphyseal dysplasia with maxillary hypoplasia and brachydactyly (MDMHB) is an autosomal-dominant skeletal dysplasia characterised by metaphyseal flaring of the long bones, enlargement of the medial halves of the clavicles, maxillary hypoplasia, brachydactyly, dental anomalies and mild osteoporosis. To date, only one large French Canadian family and a Finnish woman have been reported with the condition. In both, intragenic duplication encompassing exons 3-5 of the RUNX2 gene was identified.

The BRICS (Bronchiectasis Radiologically Indexed CT Score): A Multicenter Study Score for Use in Idiopathic and Postinfective Bronchiectasis.

Objectives: The goal of this study was to develop a simplified radiological score that could assess clinical disease severity in bronchiectasis.

Methods: The Bronchiectasis Radiologically Indexed CT Score (BRICS) was devised based on a multivariable analysis of the Bhalla score and its ability in predicting clinical parameters of severity. The score was then externally validated in six centers in 302 patients.

Oral health-related quality of life following dental treatment under general anaesthesia for early childhood caries - a UK-based study.

Objective: To assess parental reports of changes in oral health-related quality of life (OHRQoL) of young children in the UK with early childhood caries (ECC) following dental treatment under general anaesthesia (DGA). To compare the impact of oral rehabilitation (OR) and extraction-only (Exo) treatment approaches on this.

Methods: Data were collected using the proxy reported components of the Child Oral Health-Related Quality of Life (COHRQoL ) questionnaire: the Parent-Caregivers Perceptions questionnaire (P-CPQ) and Family Impact Scale (FIS), from a convenience sample of parents of children receiving DGA at a UK Paediatric Dental Department.

Congenital Heart Disease and Primary Ciliary Dyskinesia.

Through the better understanding of the genetics and clinical associations of Primary Ciliary Dyskinesia (PCD), an autosomal recessive disorder of ciliary motility and mucociliary clearance, the association between PCD and heterotaxic congenital heart disease (CHD) has been established. In parallel, research into the cause of CHD has elucidated further the role of ciliary function on the development of normal cardiovascular structure. Increased awareness by clinicians regarding this elevated risk of PCD in patients with CHD will allow for more comprehensive screening and identification of cases in this high-risk group with earlier diagnosis leading to improved health outcomes.

Is there a relationship between increased tooth number and increased tooth size? A case report.

Unlabelled: A unifying theory has been proposed that links anomalies of tooth size and number. Application of this theory suggests that anomalies of tooth size and number may share a common aetiology but could also be predicted. This article highlights an association between macrodontia and hyperdontia as demonstrated by two clinical cases.

Prospective Changes in Health-Related Quality of Life and Emotional Outcomes in Kidney Transplantation over 6 Years.

Little is known on long-term outcomes in kidney transplantation. This study evaluated changes and predictors of generic and transplantation-specific health-related quality of life (HQoL) over six years in N = 102 kidney transplant survivors using the Short-form Health Survey-36 and the Transplant Effects questionnaire. Mixed models analysis was used to determine long-term outcomes.

Nebulized hypertonic saline via positive expiratory pressure versus via jet nebulizer in patients with severe cystic fibrosis.

Background: Nebulized hypertonic saline is a highly effective therapy for patients with cystic fibrosis (CF), yet 10% of patients are intolerant of hypertonic saline administered via jet nebulizer. Positive expiratory pressure (PEP) nebulizers splint open the airways and offers a more controlled rate of nebulization.

Methods: In 4 consecutive adult CF patients who were intolerant of hypertonic saline via jet nebulizer, we nebulized 6% hypertonic saline via a PEP nebulizer.

In vitro hepatic metabolism of cediranib, a potent vascular endothelial growth factor tyrosine kinase inhibitor: interspecies comparison and human enzymology.

The in vitro metabolism of cediranib (4-[(4-fluoro-2-methyl-1H-indol-5-yl)oxy]-6-methoxy-7-[3-(1-pyrrolidinyl)propoxy]quinazoline), a vascular endothelial growth factor (VEGF) tyrosine kinase inhibitor (TKI) of all three VEGF receptors in late-stage development for the treatment of colorectal cancer and recurrent glioblastoma was investigated in hepatic proteins from preclinical species and humans using radiolabeled material. In human hepatocyte cultures, oxidative and conjugative metabolic pathways were identified, with pyrrolidine N(+)-glucuronidation being the major route. The primary oxidative pathways were di-and trioxidations and pyrrolidine N-oxidation.

Characterisation and identification of the human N+-glucuronide metabolite of cediranib.

Cediranib (4-[(4-fluoro-2-methyl-1H-indol-5-yl)oxy]-6-methoxy-7-[3-(1-pyrrolidinyl)propoxy]quinazoline; RECENTIN), a vascular endothelial growth factor (VEGF) tyrosine kinase inhibitor (TKI) of all three VEGF receptors, is currently in Phase III clinical trials for the first-line treatment of colorectal cancer and the treatment of recurrent glioblastoma. During its clinical development a unique human metabolite, an N(+)-glucuronide, was identified as a major circulating metabolite and one of the major metabolites excreted into faeces. Given the possibility of four sites for the conjugation of the glucuronic acid moiety, determination of the location of the conjugation site on cediranib was warranted.

A distinct autosomal recessive disorder of limb development with preaxial brachydactyly, phalangeal duplication, symphalangism and hyperphalangism.

We describe four children from two consanguineous families with distinctive hand and foot anomalies including preaxial brachydactyly, together with phalangeal duplication, symphalangism and hyperphalangism of fingers I-III. These anomalies are remarkably similar to those described in a previous case report. Additional features were noted both in this case and, to variable degrees, in the four children reported here.

Pharmacokinetic profile of intramuscular fulvestrant in advanced breast cancer.

Objective: To characterise the pharmacokinetics of a long-acting formulation of fulvestrant following intramuscular administration of single and multiple doses.

Study Design: Pharmacokinetic investigations of single and multiple doses of fulvestrant were conducted within two global phase III efficacy studies that compared intramuscular fulvestrant with oral anastrozole in postmenopausal women with hormone-sensitive advanced breast cancer (study 0020, conducted in Europe, Australia and South Africa, and study 0021, conducted in North America).

Methods: Patients received once-monthly intramuscular injections of fulvestrant 250 mg (1 x 5 mL for < or =21 months in study 0020; 2 x 2.

Pharmacokinetics of a single dose of fulvestrant prolonged-release intramuscular injection in postmenopausal women awaiting surgery for primary breast cancer.

Objective: The aim of this study was to describe the pharmacokinetics of 3 different single doses of fulvestrant-a new estrogen receptor (ER) antagonist that downregulates the ER with no known agonist effects-administered as a prolonged-release IM formulation.

Methods: Pharmacokinetic data were obtained in a randomized, partially blinded, placebo-controlled, parallel-group, Phase I/II multicenter trial involving postmenopausal women with primary breast cancer (clinical stages T1-T3, with tumors that were ER positive or of unknown ER status) awaiting curative-intent surgery. Patients received either IM fulvestrant (50, 125, or 250 mg), oral tamoxifen (20 mg, once daily), or oral placebo (once daily).

Putting nanocrystals to work: from solutions to devices.

The colloidal route to semiconductor nanocrystals is extremely flexible, with a high degree of control over size, size distribution, surface passivation and internal structure of the nanoparticles. Simple chemically controlled techniques can be used to assemble these particles into dense films or other microscopic structures, suitable for photonic devices. Working with semiconductors or semi-metals which in the bulk form have low or inverted bandgaps, and taking advantage of the blue shift in the quantum confinement regime, nanocrystals can readily be tuned to the infrared wavelengths of interest for telecommunications.