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KAVAZ, ESRA/0000-0002-6297-6012 WOS: 000450408800008 PubMed: 30479479 Background: Choanal polyps constitute 3-6 % of all nasal polyps and are lesions which usually originate from the mucosa of the maxillary sinus and have a solitary growth pattern. Polyps originating from the sphenoid sinus are rarely seen and are known as sphenochoanal polyps. Surgical treatment of the sphenochoanal polyps is its complete excision together with the pedicle and the portion inside the sphenoid sinus. Cases report: We report the cases of three patients who were referred to the ENT outpatient department for a persistent unilateral nasal obstruction that was resistant to medical treatment. After their clinical and endoscopic examination and imaging evaluation, these patients underwent endoscopic excision of the sphenochoanal polyps which in histopathology were proven to be inflammatory polyps. Conclusion: Although rarely seen sphenochoanal polyps must be kept in mind in the differential diagnosis of unilateral sphenoid sinus and posterior nasal cavity masses.

The use of TRISO-particle-based dispersion fuel within SiC matrix and cladding materials has the potential to allow the design of extremely safe LWRs with failure-proof fuel. This paper examines the feasibility of LWR-like cycle length for such fuel with the imposed constraint of strictly retaining the original geometry of the fuel pins and assemblies. The motivation for retaining the original geometry is to provide the ability to incorporate the fuel “as-is” into existing LWRs while retaining their thermal–hydraulic characteristics. Another mandatory constraint is use of low enriched uranium (at or below 20 w/o). The feasibility of using this fuel is assessed by looking at two factors: cycle lengths and fuel material failure rates. Other considerations (e.g., safety parameters such as reactivity coefficients, feedback, etc.) were not considered at this stage of the study. The study includes the examination of increases in the TRISO kernel sizes without changing the thickness of any of the coating layers. In addition, cases where the buffer layer thickness is allowed to vary are also considered. The study shows that a naive use of UO 2 (even up to 20 w/o enrichment) results in cycle lengths too short to be practical for existing LWR designs and operational demands. Increasing fissile inventory within the fuel compacts shows that acceptable cycle lengths can be achieved. The increase of fissile inventory can be accomplished through multiple means, including higher particle packing fraction, higher enrichment, larger fuel kernel sizes, and the use of higher density fuels (that contain a higher number of U atoms per unit volume). In this study, starting with the recognized highest packing fraction practically achievable (44%), combinations of the other means have been evaluated. The models demonstrate cycle lengths comparable to those of ordinary LWRs. As expected, TRISO particles with extremely large kernels are shown to fail under all considered scenarios. In contrast, the designs that do not depart too drastically from those of the nominal NGNP HTR fuel TRISO particles are shown to perform satisfactorily and display a high rates of survival under all considered scenarios.

Purpose: AS1402 is a humanized immunoglobulin G1 antibody that targets the aberrantly glycosylated antigen MUC1, which is overexpressed in 90% of breast tumors and contributes to estrogen-mediated growth and survival of breast cancer cells in vitro by modulating estrogen receptor (ER) activity. Aromatase inhibitors have been reported to enhance antibody-dependent cell-mediated cytotoxicity elicited by antibodies in vitro. We compared the outcomes of patients with breast cancer treated with letrozole with or without AS1402. Experimental Design: The study population included 110 patients with locally advanced or metastatic hormone receptor–positive breast cancer randomized to receive 2.5 mg letrozole only once daily or with a weekly 9 mg/kg AS1402 infusion. The primary endpoint was overall response rate. Secondary endpoints included progression-free survival, time to progression, and safety. AS1402 exposure and influence of allotypes of FcγRIIIa, FcγRIIa, and MUC1 were evaluated. Results: The study was stopped early because of a trend toward worse response rates and a higher rate of early disease progression in the AS1402 + letrozole arm. Final analysis revealed no significant difference in efficacy between the study arms. Evaluated gene polymorphisms did not define patient subgroups with improved outcomes. Addition of AS1402 to letrozole was associated with manageable toxicity. Conclusions: Because adding AS1402 to letrozole did not improve outcomes compared with letrozole only, blocking ER may be a better strategy for harnessing MUC1 modulation of the ER to a clinical advantage. FcγRIIIa, FcγRIIa, and MUC1 allotype did not predict outcome for patients treated with letrozole with or without AS1402. Clin Cancer Res; 17(21); 6822–30. ©2011 AACR.

Inherited desmosomal cardiocutaneous syndromes are characterized by the quartet of woolly hair, palmoplantar keratoderma (PPK), skin fragility and cardiac abnormalities, which are caused by mutations in genes coding for desmosomal proteins. We describe a previously unrecognized autosomal recessive syndrome in a family with arrhythmogenic right ventricular cardiomyopathy associated with alopecia and PPK (named CAPK). Genetic investigation of the family led us to find a homozygous disease-causing mutation, p.R265H, in JUP which encodes plakoglobin, a well-described member of the desmosome complex. This study expands the clinical spectrum of disorders associated with germline mutations affecting desmosomal proteins by describing a novel phenotype.

Yariz KO, Walsh T, Akay H, Duman D, Akkaynak AC, King M-C, Tekin M. A truncating mutation in GPSM2 is associated with recessive non-syndromic hearing loss. Hereditary deafness is a genetically heterogeneous phenotype for which more than 100 genomic loci have been identified thus far. By analysis of a consanguineous Palestinian family, GPSM2 was recently discovered to be the cause of autosomal recessive non-syndromic hearing loss DFNB82. Here, we report a second truncating mutation, GPSM2 p.Q562X, identified via autozygosity mapping in a consanguineous Turkish family. This report provides evidence for allelic heterogeneity of GPSM2 and confirms its causative role for non-syndromic deafness.

X-linked infantile spinal muscular atrophy (XL-SMA) is an X-linked disorder presenting with the clinical features hypotonia, areflexia, and multiple congenital contractures (arthrogryposis) associated with loss of anterior horn cells and infantile death. To identify the XL-SMA disease gene, we performed large-scale mutation analysis in genes located between markers DXS8080 and DXS7132 (Xp11.3-Xq11.1). This resulted in detection of three rare novel variants in exon 15 of UBE1 that segregate with disease: two missense mutations (c.1617 G--T, p.Met539Ile; c.1639 A--G, p.Ser547Gly) present each in one XL-SMA family, and one synonymous C--T substitution (c.1731 C--T, p.Asn577Asn) identified in another three unrelated families. Absence of the missense mutations was demonstrated for 3550 and absence of the synonymous mutation was shown in 7914 control X chromosomes; therefore, these results yielded statistical significant evidence for the association of the synonymous substitution and the two missense mutations with XL-SMA (p = 2.416 x 10(-10), p = 0.001815). We also demonstrated that the synonymous C--T substitution leads to significant reduction of UBE1 expression and alters the methylation pattern of exon 15, implying a plausible role of this DNA element in developmental UBE1 expression in humans. Our observations indicate first that XL-SMA is part of a growing list of neurodegenerative disorders associated with defects in the ubiquitin-proteasome pathway and second that synonymous C--T transitions might have the potential to affect gene expression.

Some of the rationale for nuclear energy technology development in the US has been lost or forgotten over the past two decades with the lack of a focused reactor development program. But the emergence of new R&D programs points to a need to understand how best to plan for a long-term fuel development program. The rationale for such a program is not easily found in the literature, so the authors have suggested a structure and rationale. The approach is described as four phases, with emphasis on selecting a reference fuel concept, evaluating and improving the fuel to develop a fuel specification for a reference design, obtaining data to support a licensing safety case for the fuel, and final qualification of the fuel for a specific application. Because a fuel program requires long-lead-time irradiation testing, bringing a fuel design from the initial concept through licensing might take over 20 years.

Context & objective: The Ecuadorian GH receptor deficiency (GHRD)/Laron syndrome population is the only large cohort with a single GHR mutation (E180 splice), permitting identification of numerous carrier and noncarrier first-degree relatives, to ascertain effects of heterozygosity on GH-dependent IGF-I and IGFBP-3 concentrations and on growth. Design: First-degree relatives (n = 212) of GHRD patients had specimens taken for IGF-I, IGFBP-3, and GHR genotyping. Normal statured (n = 40) and short statured (n = 40) unrelated controls had measurement of IGF-I, IGFBP-3, and stature. Results: There were no significant differences between heterozygous and homozygous normal relatives in IGF-I or IGFBP-3 standard deviation scores (SDS). Heterozygous relatives had lower mean height SDS than did homozygous normals, but with extensive overlap between genotype groups in both child and adult relatives. Height SDS in general did not relate to IGF-I or IGFBP-3 concentrations. Conclusions: GH-dependent IGF-I and IGFBP-3 secretion is not affected by heterozygosity for the E180 splice mutation that causes GHRD/Laron syndrome in the Ecuadorian population. Heterozygosity is associated with reduction in mean statural SDS, but this is not sufficient to be clinically important and not mediated through measurable differences in circulating IGF-I or IGFBP-3 related to genotype. 2007 Elsevier Ltd. All rights reserved.

Purpose: X-linked infantile spinal-muscular atrophy (XL-SMA) is a rare disorder, which presents with the clinical characteristics of hypotonia, areflexia, and multiple congenital contractures (arthrogryposis) associated with loss of anterior horn cells and death in infancy. We have previously reported a single family with XL-SMA that mapped to Xp11.3-q11.2. Here we report further clinical description of XL-SMA plus an additional seven unrelated (XL-SMA) families from North America and Europe that show linkage data consistent with the same region. Methods: We first investigated linkage to the candidate disease gene region using microsatellite repeat markers. We further saturated the candidate disease gene region using polymorphic microsatellite repeat markers and single nucleotide polymorphisms in an effort to narrow the critical region. Two-point and multipoint linkage analysis was performed using the Allegro software package. Results: Linkage analysis of all XL-SMA families displayed linkage consistent with the original XL-SMA region. Conclusion: The addition of new families and new markers has narrowed the disease gene interval for a XL-SMA locus between SNP FLJ22843 near marker DXS 8080 and SNP ARHGEF9 which is near DXS7132 (Xp11.3-Xq11.1).

Currently, commercial power reactors in the United States operate on a once-through or open cycle, with the spent nuclear fuel eventually destined for long-term storage in a geologic repository. Since the fissile and transuranic (TRU) elements in the spent nuclear fuel present a proliferation risk, limit the repository capacity, and are the major contributors to the long-term toxicity and dose from the repository, methods and systems are needed to reduce the amount of TRU that will eventually require long-term storage. An option to achieve a reduction in the amount, and modify the isotopic composition of TRU requiring geological disposal is ‘burning’ the TRU in commercial light water reactors (LWRs) and/or fast reactors. Fuel forms under consideration for TRU destruction in light water reactors (LWRs) include mixed-oxide (MOX), advanced mixed-oxide, and inert matrix fuels. Fertile-free inert matrix fuel (IMF) has been proposed for use in many forms and studied by several researchers. IMF offers several advantages relative to MOX, principally it provides a means for reducing the TRU in the fuel cycle by burning the fissile isotopes and transmuting the minor actinides while producing no new TRU elements from fertile isotopes. This paper will present and discuss the results of amore » four-bundle, neutronic, thermal-hydraulic, and transient analyses of proposed inert matrix materials in comparison with the results of similar analyses for reference UOX fuel bundles. The results of this work are to be used for screening purposes to identify the general feasibility of utilizing specific inert matrix fuel compositions in existing and future light water reactors. Compositions identified as feasible using the results of these analyses still require further detailed neutronic, thermal-hydraulic, and transient analysis study coupled with rigorous experimental testing and qualification.« less

Background Mutations in the POU3F4 gene cause X-linked deafness type 3 (DFN3), which is characterized by inner ear anomalies. Methods Three Turkish, one Ecuadorian, and one Nigerian families were included based on either inner ear anomalies detected in probands or X-linked family histories. Exome sequencing and/or Sanger sequencing were performed in order to identify the causative DNA variants in these families. Results Four novel, c.707A>C (p.(Glu236Ala)), c.772delG (p.(Glu258ArgfsX30)), c.902C>T (p.(Pro301Leu)), c.987T>C (p.(Ile308Thr)), and one previously reported mutation c.346delG (p.(Ala116ProfsX26)) in POU3F4, were identified. All mutations identified are predicted to affect the POU-specific or POU homeo domains of the protein and co-segregated with deafness in all families. Conclusions Expanding the spectrum of POU3F4 mutations in different populations along with their associated phenotypes provides better understanding of their clinical importance and will be helpful in clinical evaluation and counseling of the affected individuals. Electronic supplementary material The online version of this article (doi:10.1186/s12881-015-0149-2) contains supplementary material, which is available to authorized users.

A national project to develop a future capability to separate actinides and long-lived fission products from spent fuel, to transmute them, and to dispose off the remaining waste in optimal waste forms has begun in the United States. This project is based on the Accelerator-driven Transmutation of Waste (ATW) program developed during the 1990s at Los Alamos National Laboratory, and has its technological roots in several technologies that have been developed by the multi-mission laboratories of the U.S. Department of Energy (DOE). In the Fiscal Year 1999 Energy and Water Appropriation Act, the U.S. Congress directed the DOE to study ATW and by the end of FY99 to prepare a “roadmap” for developing this technology. DOE convened a steering committee, assembled four technical working groups consisting of members from many national laboratories, and consulted with several individual international and national experts. The finished product, “A Roadmap for Developing ATW Technology – A Report to Congress,” recommends a five-year, $281 M, science-based, technical-risk-reduction program. This paper provides an overview of the U.S. Roadmap for developing ATW technology, the organization of the national ATW Project, the critical issues in subsystems and technological options, deployment scenarios, institutional challenges, and academic and international collaboration.

The thermal-hydraulic design of the accelerator production of tritium (APT) tungsten neutron source is presented. A carefully engineered thermal-hydraulic design is required to remove the deposited power effectively during normal operations and remove the decay power during plant shutdown and postulated accidents. For steady-state operations and operational and anticipated transients, the design criterion is to maintain single-phase flow conditions with a margin to onset of nucleate boiling. The margin is determined based on phenomenological and geometric uncertainties associated with the design. A large margin to thermal excursion limits, such as critical heat flux and onset of flow instability, also is maintained during normal operations. In general, a very robust thermal-hydraulic design can be accomplished using the traditional models and correlations available in the engineering literature. However, two issues require further attention: maintaining adequate flows in a parallel network of flow channels and minimizing the volume fraction of heavy water to maximize tritium production.The design uses ladderlike structures that contain clad tungsten cylinders in the rungs that have coolant supplied and removed by the vertical ladder rails. Because the power density drops in the beam direction, the thickness of the tungsten cylinders is increased with increasing beam penetration length. The coolingmore » requirement is determined using a conservative criterion where the minimum wall subcooling inside the rungs is at least 40 deg. C and the minimum Reynolds number is 6000. Initial flow distribution tests were conducted with a full-scale model of an APT ladder assembly based on a preliminary design. Flow distributions can be made more even by using a larger riser than downcomer and also by increasing the flow resistance across each rung. The calculations discussed assume nominal dimensions, even though the power deposition and removal use a conservative approach. The effect of manufacturing tolerances will be investigated in future research. Also, the applicability of the critical heat flux and onset of flow instability models to small coolant channels is being verified experimentally. Further design optimization will be possible when these studies are completed.« less

The modeling of thermal-chemical behavior of targets used in accelerator applications is an important part of safety analysis. Tungsten is considered as a target material to produce tritium in a linear proton accelerator. The prediction of the chemical reactivity of tungsten in a steam flow at high temperatures is the most important part of a safety analysis of target design. The oxidation and volatilization of tungsten in steam at high temperatures is a complex phenomenon that involves various mechanisms (depending on the temperature), steam pressure, and steam velocity. A simple diffusion model that considers chemical equilibrium at the reaction interface and effective diffusion thickness, including the boundary and oxide layers, is proposed for predicting the volatilization rate. The proposed simple model predicts the available data reasonably well. The proposed model is implemented into a computer program that is developed to predict the radiological releases during postulated loss-of-coolant accidents (LOCAs). The computer program models heat production, heat transfer, and oxidation reactions in the multiple radiation enclosures representing the accelerator target elements. It treats each element of the radiation enclosures as a lumped control volume, or heat structure. Each heat structure may generate or lose heat by conduction, convection, or radiation and is subject to mass loss as a result of oxidation, melting, and volatilization. Postulated beyond-design-basis LOCAs are simulated with this computer program for the accelerator-production-of-tritium target. Sample calculations demonstrate oxidation/volatilization model capabilities and sensitivity to the assumptions selected.

Complex ladder-like structures recently have been considered as the target design for accelerator applications. The decay heat, during a postulated beyond design-basis loss-of-coolant accident in the target where all normal and emergency cooling fails, is removed mainly by radiation heat transfer. Modeling of the radiation transport in complex ladder-like structures has several challenges and limitations when the standard net-radiation model is used. This paper proposes a simplified lumped, or ‘hot-rung’ model, that considers the worst elements and utilizes the standard net-radiation method. The net-radiation model would under-predict structure temperatures if surfaces were subject to non-uniform radiosity. The proposed model was assessed to suggest corrections to account for the non-uniform radiosity. The non-uniform radiosity effect causes the proposed hot-rung model to under-predict the center-rung temperatures by ≈4–74°C when all parametrics, including temperatures up to 1500°C, were considered. These temperatures are small. The proposed model predicted that an important effect of decreasing the emissivity was smoothing of non-isothermal effects. The radiosity effects are more pronounced when there are strong temperature gradients. Uniform rung temperatures tend to decrease the radiosity effects. We concluded that a relatively simple model that is conservative with respect to radiosity effects could be developed.

Item does not contain fulltext Hereditary hearing loss is characterized by a high degree of genetic heterogeneity. Here we present OTOGL mutations, a homozygous one base pair deletion (c.1430 delT) causing a frameshift (p.Val477Glufs( *)25) in a large consanguineous family and two compound heterozygous mutations, c.547C>T (p.Arg183( *)) and c.5238+5G>A, in a nonconsanguineous family with moderate nonsyndromic sensorineural hearing loss. OTOGL maps to the DFNB84 locus at 12q21.31 and encodes otogelin-like, which has structural similarities to the epithelial-secreted mucin protein family. We demonstrate that Otogl is expressed in the inner ear of vertebrates with a transcription level that is high in embryonic, lower in neonatal, and much lower in adult stages. Otogelin-like is localized to the acellular membranes of the cochlea and the vestibular system and to a variety of inner ear cells located underneath these membranes. Knocking down of otogl with morpholinos in zebrafish leads to sensorineural hearing loss and anatomical changes in the inner ear, supporting that otogelin-like is essential for normal inner ear function. We propose that OTOGL mutations affect the production and/or function of acellular structures of the inner ear, which ultimately leads to sensorineural hearing loss.

In this paper, a simple natural convection heat-transfer model for a safety rod placed in a perforated guide tube is proposed. The geometry is typical of the Savannah River K reactor. The proposed model for the rod and the perforated guide-tube assembly is benchmarked against prototypical test data obtained by Idaho National Engineering Laboratory. The results showed that the proposed model was in good agreement with the experimental data except at very high temperatures where the model slightly underpredicted the rod and tube temperatures. By evaluating the thermal conductivity of the fluid (air) at the wall temperature, the prediction of the high temperature natural convection data was further improved.

A computer program developed to analyze nucleate boiling over a heated surface is described. The model solves the three-dimensional transient conduction equation within the heater. The conduction solution is coupled with closure relationships to mimic the bubble dynamics and the associated heat transfer coefficients. Sample problems are run using a copper surface subject to partial nucleate boiling in saturated water at atmospheric pressure. The results are shown to be in good qualitative agreement with the pertinent experimental observations.

Using a numerical model, the effect of heating methods on saturated nucleate pool boiling is investigated parametrically for smooth and rough nickel and copper heater plates. The boiling curve moved right with decreasing thickness for the smooth and rough nickel and copper heaters in the constant-heat-flux heating method. This trend was reversed in the constant-temperature heating method; the boiling curved shifted left with decreasing heater thickness. However, the later trend was not affected by the heater material and thickness and the surface roughness (mean cavity radius). The boiling curves were identical for the constant internal generation rate and the constant-heat-flux heating method. The use of ac instead of dc resistive heating caused the boiling curve generally to move left. This behavior was not linear with the heat flux, heater material, or surface conditions. No hysterisis was found when the heat flux was increased and then decreased gradually to original values.

Objective To test by genomic analysis whether empty follicle syndrome (EFS) in a family with two affected sisters has a genetic basis. Design Whole-exome sequencing in the context of clinical genetics. Setting University hospital. Patient(s) Two women (36 and 32 years old at the time of the study) with EFS. Intervention(s) Genetic counseling based on autosomal recessive inheritance. Main Outcome Measure(s) Discovery of a mutation in the LH/choriogonadotropin receptor ( LHCGR ) as the cause of EFS. Result(s) A novel missense mutation in LHCGR , p.N400S, was homozygous in sisters with EFS and/or infertility, but not in their unaffected siblings or parents. The mutation was not present in 500 ancestry-matched control subjects. Asparagine at residue 400 is highly conserved and its substitution by serine predicted to alter critical interactions that stabilize LHCGR. Conclusion(s) We describe a genetic basis for EFS and provide strong evidence for the existence of genuine EFS in some patients. A mutation impairing the function of LHCGR explains the lack of response of these patients to repeated administration of β-hCG.

A new model has been developed where the coupled transient two-dimensional conduction equation is solved for the heater and the liquid macrolayer, while allowing for the time-wise thinning of the macrolayer. The major conclusions are: (1) dominant evaporation occurs at the liquid-vapor-solid contact point (triple point) and is required to match boiling curve behavior quantitatively; (2) evaporation at the stem interface and bubble-macrolayer interface is negligible (except near critical heat flux); and (3) the results are sensitive to closure relationships, especially to the active site-density correlation.

A new approach to address the effect of surface radiation on a laminar film boiling flow over a horizontal flat plate is suggested. Previously developed approximate expressions of the thermal boundary-layer thickness and the wall shear stress of a moving surface in a flowing liquid are used to circumvent the complicated simultaneous solution of the vapor and liquid flow. Approximate closed-form expressions to predict the wall heat transfer and skin friction are obtained. For a water-steam system at atmospheric pressure within the wall temperature range considered (Tw

Subcooled laminar forced convection film boiling flow on a wedge is analyzed considering the streamwise pressure gradient imposed on the flow and the streamwise buoyancy force acting on the vapor film. A twophase boundary-layer model is proposed, and the local similarity concept is applied to obtain an approximate solution of the governing equations. For a water-steam system at atmospheric pressure considered within this study, wall skin friction results display a strong dependency on the streamwise buoyancy force driving the vapor film and the external pressure gradient. Adverse streamwise buoyancy force acting on the vapor film, which is the case on the lower surface of a horizontally aligned wedge, may cause vapor flow separation. In contrast to wall skin friction dependency, the wall heat transfer parameter shows a secondary dependence on the streamwise pressure gradient and the buoyancy force.

The authors consider the high-heat-flux, pool nucleate boiling region of a horizontal surface and propose a theoretical basis for estimating the constant of proportionality between the mean macrolayer thickness and the mean vapor-stem diameter at critical heat flux. This constant is found to be of the same order of magnitude (−10% difference) as that suggested by Gaertner [5]. While Gaertner's estimate is based on photographic evidence, the current approach uses a theoretical basis together with experimental data.

The Global Nuclear Energy Program (GNEP) includes a program element for the development and construction of an advanced sodium cooled fast reactor to demonstrate the burning (transmutation) of significant quantities of minor actinides obtained from a separations process and fabricated into a transuranic bearing fuel assembly. To demonstrate and qualify transuranic (TRU) fuel in a fast reactor, an Advanced Burner Reactor (ABR) prototype is needed. The ABR would necessarily be started up using conventional metal alloy or oxide (U or U, Pu) fuel. Startup fuel is needed for the ABR for the first 2 to 4 core loads of fuel in the ABR. Following start up, a series of advanced TRU bearing fuel assemblies will be irradiated in qualification lead test assemblies in the ABR. There are multiple options for this startup fuel. This report provides a description of the possible startup fuel options as well as possible fabrication alternatives available to the program in the current domestic and international facilities and infrastructure.

This report contains a technical summary package in response to a Level 2 milestone in the transmutation fuel campaign (TFC) management work-package calling for input to the Secretarial decision. At present, the form of the Secretarial decision package is not fully defined, and it is not clear exactly what will be required from the TFC as a final input. However, it is anticipated that a series oftechnical and programmatic documents will need to be provided in support of a wider encompassing document on GNEP technology development activities. The TFC technical leadership team provides this report as initial input to the secretarial decision package which is being developed by the Technical Integration Office (TIO) in support of Secretarial decision. This report contains a summary of the TFC execution plan with a work breakdown structure, highlevel schedule, major milestones, and summary description of critical activities in support of campaign objectives. Supporting documents referenced in this report but provided under separate cover include: • An updated review of the state-of-the art for transmutation fuel development activities considering national as well as international fuel research and development testing activities. • A definition of the Technology Readiness Level (TRL) used to systematically define and execute the transmutation fuel development activities.

The technology demonstration element of the Global Nuclear Energy Partnership (GNEP) program is aimed at demonstrating the closure of the fuel cycle by destroying the transuranic (TRU) elements separated from spent nuclear fuel (SNF). Multiple recycle through fast reactors is used for burning the TRU initially separated from light-water reactor (LWR) spent nuclear fuel. For the initial technology demonstration, the preferred option to demonstrate the closed fuel cycle destruction of TRU materials is a sodium-cooled fast reactor (FR) used as burner reactor. The sodium-cooled fast reactor represents the most mature sodium reactor technology available today. This report provides a review of the current state of development of fuel systems relevant to the sodium-cooled fast reactor. This report also provides a review of research and development of TRU-metal alloy and TRU-oxide composition fuels. Experiments providing data supporting the understanding of minor actinide (MA)-bearing fuel systems are summarized and referenced.