Sample records for wasting syndrome characterized

  1. Waste Characterization Methods

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Vigil-Holterman, Luciana R.; Naranjo, Felicia Danielle

    2016-02-02

    This report discusses ways to classify waste as outlined by LANL. Waste Generators must make a waste determination and characterize regulated waste by appropriate analytical testing or use of acceptable knowledge (AK). Use of AK for characterization requires several source documents. Waste characterization documentation must be accurate, sufficient, and current (i.e., updated); relevant and traceable to the waste stream’s generation, characterization, and management; and not merely a list of information sources.

  2. ASSESSING TCDD WASTING SYNDROME IN AN IN VIVO OBESITY MODEL

    EPA Science Inventory

    TCDD is a by-product of incineration commonly found as a microcontaminant in the food supply. The TCDD wasting syndrome, characterized by prolonged weight loss, has been examined for decades. Much of this work has focused on high dose in vivo and in vitro studies....

  3. SAM syndrome is characterized by extensive phenotypic heterogeneity.

    PubMed

    Taiber, Shahar; Samuelov, Liat; Mohamad, Janan; Cohen Barak, Eran; Sarig, Ofer; Shalev, Stavit Allon; Lestringant, Gilles; Sprecher, Eli

    2018-03-31

    Severe skin dermatitis, multiple allergies and metabolic wasting (SAM) syndrome is a rare life-threatening inherited condition caused by bi-allelic mutations in DSG1 encoding desmoglein 1. The disease was initially reported to manifest with severe erythroderma, failure to thrive, atopic manifestations, recurrent infections, hypotrichosis and palmoplantar keratoderma. We present 3 new cases of SAM syndrome in 2 families and review the cases published so far. Whole exome and direct sequencing were used to identify SAM syndrome-causing mutations. Consistent with previous data, SAM syndrome was found in all 3 patients to result from homozygous mutations in DSG1 predicted to result in premature termination of translation. In contrast, as compared with patients previously reported, the present cases were found to display a wide range of clinical presentations of variable degrees of severity. The present data emphasizes the fact that SAM syndrome is characterized by extensive phenotypic heterogeneity, suggesting the existence of potent modifier traits. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  4. Cerebral salt-wasting syndrome due to hemorrhagic brain infarction: a case report.

    PubMed

    Tanaka, Tomotaka; Uno, Hisakazu; Miyashita, Kotaro; Nagatsuka, Kazuyuki

    2014-07-23

    Cerebral salt-wasting syndrome is a condition featuring hyponatremia and dehydration caused by head injury, operation on the brain, subarachnoid hemorrhage, brain tumor and so on. However, there are a few reports of cerebral salt-wasting syndrome caused by cerebral infarction. We describe a patient with cerebral infarction who developed cerebral salt-wasting syndrome in the course of hemorrhagic transformation. A 79-year-old Japanese woman with hypertension and arrhythmia was admitted to our hospital for mild consciousness disturbance, conjugate deviation to right, left unilateral spatial neglect and left hemiparesis. Magnetic resonance imaging revealed a broad ischemic change in right middle cerebral arterial territory. She was diagnosed as cardiogenic cerebral embolism because atrial fibrillation was detected on electrocardiogram on admission. She showed hyponatremia accompanied by polyuria complicated at the same time with the development of hemorrhagic transformation on day 14 after admission. Based on her hypovolemic hyponatremia, she was evaluated as not having syndrome of inappropriate secretion of antidiuretic hormone but cerebral salt-wasting syndrome. She fortunately recovered with proper fluid replacement and electrolyte management. This is a rare case of cerebral infarction and cerebral salt-wasting syndrome in the course of hemorrhagic transformation. It may be difficult to distinguish cerebral salt-wasting syndrome from syndrome of inappropriate antidiuretic hormone, however, an accurate assessment is needed to reveal the diagnosis of cerebral salt-wasting syndrome because the recommended fluid management is opposite in the two conditions.

  5. HPV16 induces a wasting syndrome in transgenic mice: Amelioration by dietary polyphenols via NF-κB inhibition.

    PubMed

    Gil da Costa, Rui M; Aragão, Sofia; Moutinho, Magda; Alvarado, Antonieta; Carmo, Diogo; Casaca, Fátima; Silva, Sandra; Ribeiro, Joana; Sousa, Hugo; Ferreira, Rita; Nogueira-Ferreira, Rita; Pires, Maria João; Colaço, Bruno; Medeiros, Rui; Venâncio, Carlos; Oliveira, Maria Manuel; Bastos, Margarida M S M; Lopes, Carlos; Oliveira, Paula A

    2017-01-15

    Cancer patients often show a wasting syndrome for which there are little therapeutic options. Dietary polyphenols have been proposed for treating this syndrome, but their usefulness in cases associated with human papillomavirus (HPV)-induced cancers is unknown. We characterized HPV16-transgenic mice as a model of cancer cachexia and tested the efficacy of long-term oral supplementation with polyphenols curcumin and rutin. Both compounds were orally administered to six weeks-old HPV16-transgenic mice showing characteristic multi-step skin carcinogenesis, for 24weeks. Skin lesions and blood, liver and spleen inflammatory changes were characterized histologically and hematologically. Hepatic oxidative stress, skeletal muscle mass and the levels of muscle pro-inflammatory transcription factor NF-κB were also assessed. Skin carcinogenesis was associated with progressive, severe, systemic inflammation (leukocytosis, hepatitis, splenitis), significant mortality and cachexia. Curcumin and rutin totally suppressed mortality while reducing white blood cells and the incidence of splenitis and hepatitis. Rutin prevented muscle wasting more effectively than curcumin. Preservation of muscle mass and reduced hepatic inflammation were associated with down-regulation of the NF-κB canonical pathway and with reduced oxidative stress, respectively. These results point out HPV16-transgenic mice as a useful model for studying the wasting syndrome associated with HPV-induced cancers. Dietary NF-κB inhibitors may be useful resources for treating this syndrome. Copyright © 2016 Elsevier Inc. All rights reserved.

  6. Pretest characterization of WIPP experimental waste

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Johnson, J.; Davis, H.; Drez, P.E.

    The Waste Isolation Pilot Plant (WIPP) near Carlsbad, New Mexico, is an underground repository designed for the storage and disposal of transuranic (TRU) wastes from US Department of Energy (DOE) facilities across the country. The Performance Assessment (PA) studies for WIPP address compliance of the repository with applicable regulations, and include full-scale experiments to be performed at the WIPP site. These experiments are the bin-scale and alcove tests to be conducted by Sandia National Laboratories (SNL). Prior to conducting these experiments, the waste to be used in these tests needs to be characterized to provide data on the initial conditionsmore » for these experiments. This characterization is referred to as the Pretest Characterization of WIPP Experimental Waste, and is also expected to provide input to other programmatic efforts related to waste characterization. The purpose of this paper is to describe the pretest waste characterization activities currently in progress for the WIPP bin-scale waste, and to discuss the program plan and specific analytical protocols being developed for this characterization. The relationship between different programs and documents related to waste characterization efforts is also highlighted in this paper.« less

  7. RH-TRU Waste Inventory Characterization by AK and Proposed WIPP RH-TRU Waste Characterization Objectives

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Most, W. A.; Kehrman, R.; Gist, C.

    2002-02-26

    The U.S. Department of Energy (DOE)-Carlsbad Field Office (CBFO) has developed draft documentation to present the proposed Waste Isolation Pilot Plant (WIPP) remote-handled (RH-) transuranic (TRU) waste characterization program to its regulators, the U.S. Environmental Protection Agency and the New Mexico Environment Department. Compliance with Title 40, Code of Federal Regulations, Parts 191 and 194; the WIPP Land Withdrawal Act (PL 102-579); and the WIPP Hazardous Waste Facility Permit, as well as the Certificates of Compliance for the 72-B and 10-160B Casks, requires that specific waste parameter limits be imposed on DOE sites disposing of TRU waste at WIPP. Themore » DOE-CBFO must control the sites' compliance with the limits by specifying allowable characterization methods. As with the established WIPP contact handled TRU waste characterization program, the DOE-CBFO has proposed a Remote-Handled TRU Waste Acceptance Criteria (RH-WAC) document consolidating the requirements from various regulatory drivers and proposed allowable characterization methods. These criteria are consistent with the recommendation of a recent National Academy Sciences/National Research Council to develop an RH-TRU waste characterization approach that removes current self imposed requirements that lack a legal or safety basis. As proposed in the draft RH-WAC and other preliminary documents, the DOE-CBFO RH-TRU waste characterization program proposes the use of acceptable knowledge (AK) as the primary method for obtaining required characterization information. The use of AK involves applying knowledge of the waste in light of the materials or processes used to generate the waste. Documentation, records, or processes providing information about various attributes of a waste stream, such as chemical, physical, and radiological properties, may be used as AK and may be applied to individual waste containers either independently or in conjunction with radiography, visual examination, assay

  8. Waste Characterization Process

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Lambert, Patrick E.

    2014-11-01

    The purpose is to provide guidance to the Radiological Characterization Reviewer to complete the radiological characterization of waste items. This information is used for Department of Transportation (DOT) shipping and disposal, typically at the Nevada National Security Site (NNSS). Complete characterization ensures compliance with DOT shipping laws and NNSS Waste Acceptance Criteria (WAC). The fines for noncompliance can be extreme. This does not include possible bad press, and endangerment to the public, employees and the environment. A Radiological Characterization Reviewer has an important role in the organization. The scope is to outline the characterization process, but does not to includemore » every possible situation. The Radiological Characterization Reviewer position requires a strong background in Health Physics; therefore, these concepts are minimally addressed. The characterization process includes many Excel spreadsheets that were developed by Michael Enghauser known as the WCT software suite. New Excel spreadsheets developed as part of this project include the Ra- 226 Decider and the Density Calculator by Jesse Bland, MicroShield Density Calculator and Molecular Weight Calculator by Pat Lambert.« less

  9. 75 FR 54631 - Proposed Approval of the Central Characterization Project's Transuranic Waste Characterization...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-09-08

    ... Central Characterization Project's Transuranic Waste Characterization Program at the Hanford Site AGENCY...) waste characterization program implemented by the Central Characterization Project (CCP) at the Hanford... characterization of TRU debris waste from Hanford-CCP during an inspection conducted on April 27-29, 2010. Using...

  10. Characterization of urban solid waste in Chihuahua, Mexico.

    PubMed

    Gomez, Guadalupe; Meneses, Montserrat; Ballinas, Lourdes; Castells, Francesc

    2008-12-01

    The characterization of urban solid waste generation is fundamental for adequate decision making in the management strategy of urban solid waste in a city. The objective of this study is to characterize the waste generated in the households of Chihuahua city, and to compare the results obtained in areas of the city with three different socioeconomic levels. In order to identify the different socioeconomic trends in waste generation and characterization, 560 samples of solid waste were collected during 1 week from 80 households in Chihuahua and were hand sorted and classified into 15 weighted fractions. The average waste generation in Chihuahua calculated in this study was 0.676 kg per capita per day in April 2006. The main fractions were: organic (48%), paper (16%) and plastic (12%). Results show an increased waste generation associated with the socioeconomic level. The characterization in amount and composition of urban waste is the first step needed for the successful implementation of an integral waste management system.

  11. Ecotoxicological characterization of hazardous wastes.

    PubMed

    Wilke, B-M; Riepert, F; Koch, Christine; Kühne, T

    2008-06-01

    In Europe hazardous wastes are classified by 14 criteria including ecotoxicity (H 14). Standardized methods originally developed for chemical and soil testing were adapted for the ecotoxicological characterization of wastes including leachate and solid phase tests. A consensus on which tests should be recommended as mandatory is still missing. Up to now, only a guidance on how to proceed with the preparation of waste materials has been standardized by CEN as EN 14735. In this study, tests including higher plants, earthworms, collembolans, microorganisms, duckweed and luminescent bacteria were selected to characterize the ecotoxicological potential of a boiler slag, a dried sewage sludge, a thin sludge and a waste petrol. In general, the instructions given in EN 14735 were suitable for all wastes used. The evaluation of the different test systems by determining the LC/EC(50) or NOEC-values revealed that the collembolan reproduction and the duckweed frond numbers were the most sensitive endpoints. For a final classification and ranking of wastes the Toxicity Classification System (TCS) using EC/LC(50) values seems to be appropriate.

  12. 40 CFR 194.24 - Waste characterization.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... system. (e) Waste may be emplaced in the disposal system only if the emplaced components of such waste... 40 Protection of Environment 26 2012-07-01 2011-07-01 true Waste characterization. 194.24 Section... PROGRAMS CRITERIA FOR THE CERTIFICATION AND RE-CERTIFICATION OF THE WASTE ISOLATION PILOT PLANT'S...

  13. 40 CFR 194.24 - Waste characterization.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... system. (e) Waste may be emplaced in the disposal system only if the emplaced components of such waste... 40 Protection of Environment 26 2013-07-01 2013-07-01 false Waste characterization. 194.24 Section... PROGRAMS CRITERIA FOR THE CERTIFICATION AND RE-CERTIFICATION OF THE WASTE ISOLATION PILOT PLANT'S...

  14. 40 CFR 194.24 - Waste characterization.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... system. (e) Waste may be emplaced in the disposal system only if the emplaced components of such waste... 40 Protection of Environment 25 2011-07-01 2011-07-01 false Waste characterization. 194.24 Section... PROGRAMS CRITERIA FOR THE CERTIFICATION AND RE-CERTIFICATION OF THE WASTE ISOLATION PILOT PLANT'S...

  15. 40 CFR 194.24 - Waste characterization.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... system. (e) Waste may be emplaced in the disposal system only if the emplaced components of such waste... 40 Protection of Environment 25 2014-07-01 2014-07-01 false Waste characterization. 194.24 Section... PROGRAMS CRITERIA FOR THE CERTIFICATION AND RE-CERTIFICATION OF THE WASTE ISOLATION PILOT PLANT'S...

  16. Combined central diabetes insipidus and cerebral salt wasting syndrome in children.

    PubMed

    Lin, Jainn-Jim; Lin, Kuang-Lin; Hsia, Shao-Hsuan; Wu, Chang-Teng; Wang, Huei-Shyong

    2009-02-01

    Central diabetes insipidus, a common consequence of acute central nervous system injury, causes hypernatremia; cerebral salt wasting syndrome can cause hyponatremia. The two conditions occurring simultaneous are rarely described in pediatric patients. Pediatric cases of combined diabetes insipidus and cerebral salt wasting after acute central nervous system injury between January 2000 and December 2007 were retrospectively reviewed, and clinical characteristics were systemically assessed. Sixteen patients, aged 3 months to 18 years, met study criteria: 11 girls and 5 boys. The most common etiologies were severe central nervous system infection (n = 7, 44%) and hypoxic-ischemic event (n = 4, 25%). In 15 patients, diabetes insipidus was diagnosed during the first 3 days after acute central nervous system injury. Onset of cerebral salt wasting syndrome occurred 2-8 days after the onset of diabetes insipidus. In terms of outcome, 13 patients died (81%) and 3 survived under vegetative status (19%). Central diabetes insipidus and cerebral salt wasting syndrome may occur after acute central nervous system injury. A combination of both may impede accurate diagnosis. Proper differential diagnoses are critical, because the treatment strategy for each entity is different.

  17. Development of characterization protocol for mixed liquid radioactive waste classification

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Zakaria, Norasalwa, E-mail: norasalwa@nuclearmalaysia.gov.my; Wafa, Syed Asraf; Wo, Yii Mei

    2015-04-29

    Mixed liquid organic waste generated from health-care and research activities containing tritium, carbon-14, and other radionuclides posed specific challenges in its management. Often, these wastes become legacy waste in many nuclear facilities and being considered as ‘problematic’ waste. One of the most important recommendations made by IAEA is to perform multistage processes aiming at declassification of the waste. At this moment, approximately 3000 bottles of mixed liquid waste, with estimated volume of 6000 litres are currently stored at the National Radioactive Waste Management Centre, Malaysia and some have been stored for more than 25 years. The aim of this studymore » is to develop a characterization protocol towards reclassification of these wastes. The characterization protocol entails waste identification, waste screening and segregation, and analytical radionuclides profiling using various analytical procedures including gross alpha/ gross beta, gamma spectrometry, and LSC method. The results obtained from the characterization protocol are used to establish criteria for speedy classification of the waste.« less

  18. Understanding Bartter syndrome and Gitelman syndrome.

    PubMed

    Fremont, Oliver T; Chan, James C M

    2012-02-01

    We aim to review the clinical features of two renal tubular disorders characterized by sodium and potassium wasting: Bartter syndrome and Gitelman syndrome. Selected key references concerning these syndromes were analyzed, together with a PubMed search of the literature from 2000 to 2011. The clinical features common to both conditions and those which are distinct to each syndrome were presented. The new findings on the genetics of the five types of Bartter syndrome and the discrete mutations in Gitelman syndrome were reviewed, together with the diagnostic workup and treatment for each condition. Patients with Bartter syndrome types 1, 2 and 4 present at a younger age than classic Bartter syndrome type 3. They present with symptoms, often quite severe in the neonatal period. Patients with classic Bartter syndrome type 3 present later in life and may be sporadically asymptomatic or mildly symptomatic. The severe, steady-state hypokalemia in Bartter syndrome and Gitelman syndrome may abruptly become life-threatening under certain aggravating conditions. Clinicians need to be cognizant of such renal tubular disorders, and promptly treat at-risk patients.

  19. Uncertainty quantification applied to the radiological characterization of radioactive waste.

    PubMed

    Zaffora, B; Magistris, M; Saporta, G; Chevalier, J-P

    2017-09-01

    This paper describes the process adopted at the European Organization for Nuclear Research (CERN) to quantify uncertainties affecting the characterization of very-low-level radioactive waste. Radioactive waste is a by-product of the operation of high-energy particle accelerators. Radioactive waste must be characterized to ensure its safe disposal in final repositories. Characterizing radioactive waste means establishing the list of radionuclides together with their activities. The estimated activity levels are compared to the limits given by the national authority of the waste disposal. The quantification of the uncertainty affecting the concentration of the radionuclides is therefore essential to estimate the acceptability of the waste in the final repository but also to control the sorting, volume reduction and packaging phases of the characterization process. The characterization method consists of estimating the activity of produced radionuclides either by experimental methods or statistical approaches. The uncertainties are estimated using classical statistical methods and uncertainty propagation. A mixed multivariate random vector is built to generate random input parameters for the activity calculations. The random vector is a robust tool to account for the unknown radiological history of legacy waste. This analytical technique is also particularly useful to generate random chemical compositions of materials when the trace element concentrations are not available or cannot be measured. The methodology was validated using a waste population of legacy copper activated at CERN. The methodology introduced here represents a first approach for the uncertainty quantification (UQ) of the characterization process of waste produced at particle accelerators. Copyright © 2017 Elsevier Ltd. All rights reserved.

  20. Solid waste generation and characterization in the University of Lagos for a sustainable waste management.

    PubMed

    Adeniran, A E; Nubi, A T; Adelopo, A O

    2017-09-01

    Waste characterization is the first step to any successful waste management policy. In this paper, the characterization and the trend of solid waste generated in University of Lagos, Nigeria was carried out using ASTM D5231-92 and Resource Conservation Reservation Authority RCRA Waste Sampling Draft Technical Guidance methods. The recyclable potential of the waste is very high constituting about 75% of the total waste generated. The estimated average daily solid waste generation in Unilag Akoka campus was estimated to be 32.2tons. The solid waste characterization was found to be: polythene bags 24% (7.73tons/day), paper 15% (4.83tons/day), organic matters 15%, (4.83tons/day), plastic 9% (2.90tons/day), inert materials 8% (2.58tons/day), sanitary 7% (2.25tons/day), textile 7% (2.25tons/day), others 6% (1.93tons/day), leather 4% (1.29tons/day) metals 3% (0.97tons/day), glass 2% (0.64tons/day) and e-waste 0% (0.0tons/day). The volume and distribution of polythene bags generated on campus had a positive significant statistical correlation with the distribution of commercial and academic structures on campus. Waste management options to optimize reuse, recycling and reduce waste generation were discussed. Copyright © 2017 Elsevier Ltd. All rights reserved.

  1. Diagnosis and Management of Combined Central Diabetes Insipidus and Cerebral Salt Wasting Syndrome After Traumatic Brain Injury.

    PubMed

    Wu, Xuehai; Zhou, Xiaolan; Gao, Liang; Wu, Xing; Fei, Li; Mao, Ying; Hu, Jin; Zhou, Liangfu

    2016-04-01

    Combined central diabetes insipidus and cerebral salt wasting syndrome after traumatic brain injury (TBI) is rare, is characterized by massive polyuria leading to severe water and electrolyte disturbances, and usually is associated with very high mortality mainly as a result of delayed diagnosis and improper management. We retrospectively reviewed the clinical presentation, management, and outcomes of 11 patients who developed combined central diabetes insipidus and cerebral salt wasting syndrome after traumatic brain injury to define distinctive features for timely diagnosis and proper management. The most typical clinical presentation was massive polyuria (10,000 mL/24 hours or >1000 mL/hour) refractory to vasopressin alone but responsive to vasopressin plus cortisone acetate. Other characteristic presentations included low central venous pressure, high brain natriuretic peptide precursor level without cardiac dysfunction, high 24-hour urine sodium excretion and hypovolemia, and much higher urine than serum osmolarity; normal serum sodium level and urine specific gravity can also be present. Timely and adequate infusion of sodium chloride was key in treatment. Of 11 patients, 5 had a good prognosis 3 months later (Extended Glasgow Outcome Scale score ≥6), 1 had an Extended Glasgow Outcome Scale score of 4, 2 died in the hospital of brain hernia, and 3 developed a vegetative state. For combined diabetes insipidus and cerebral salt wasting syndrome after traumatic brain injury, massive polyuria is a major typical presentation, and intensive monitoring of fluid and sodium status is key for timely diagnosis. To achieve a favorable outcome, proper sodium chloride supplementation and cortisone acetate and vasopressin coadministration are key. Copyright © 2016 Elsevier Inc. All rights reserved.

  2. WIPP waste characterization program sampling and analysis guidance manual

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Not Available

    1991-01-01

    The Waste Isolation Pilot Plant (WIPP) Waste Characterization Program Sampling and Analysis Guidance Manual (Guidance Manual) provides a unified source of information on the sampling and analytical techniques that enable Department of Energy (DOE) facilities to comply with the requirements established in the current revision of the Quality Assurance Program Plan (QAPP) for the WIPP Experimental-Waste Characterization Program (the Program). This Guidance Manual includes all of the sampling and testing methodologies accepted by the WIPP Project Office (DOE/WPO) for use in implementing the Program requirements specified in the QAPP. This includes methods for characterizing representative samples of transuranic (TRU) wastesmore » at DOE generator sites with respect to the gas generation controlling variables defined in the WIPP bin-scale and alcove test plans, as well as waste container headspace gas sampling and analytical procedures to support waste characterization requirements under the WIPP test program and the Resource Conservation and Recovery Act (RCRA). The procedures in this Guidance Manual are comprehensive and detailed and are designed to provide the necessary guidance for the preparation of site specific procedures. The use of these procedures is intended to provide the necessary sensitivity, specificity, precision, and comparability of analyses and test results. The solutions to achieving specific program objectives will depend upon facility constraints, compliance with DOE Orders and DOE facilities' operating contractor requirements, and the knowledge and experience of the TRU waste handlers and analysts. With some analytical methods, such as gas chromatography/mass spectrometry, the Guidance Manual procedures may be used directly. With other methods, such as nondestructive/destructive characterization, the Guidance Manual provides guidance rather than a step-by-step procedure.« less

  3. 77 FR 11112 - Proposed Approval of the Central Characterization Project's Remote-Handled Transuranic Waste...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2012-02-24

    ... Central Characterization Project's Remote-Handled Transuranic Waste Characterization Program at the...-handled (RH), transuranic (TRU) waste characterization program implemented by the Central Characterization... Criteria, EPA evaluated the characterization of RH TRU debris waste from SRS-CCP during an inspection on...

  4. 76 FR 62062 - Proposed Approval of the Central Characterization Project's Remote-Handled Transuranic Waste...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-10-06

    ... Central Characterization Project's Remote-Handled Transuranic Waste Characterization Program at Sandia..., remote-handled (RH), transuranic (TRU) waste characterization program implemented by the Central Characterization Project (CCP) at Sandia National Laboratory (SNL) in Albuquerque, New Mexico. This waste is...

  5. 76 FR 33277 - Proposed Approval of the Central Characterization Project's Remote-Handled Transuranic Waste...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-06-08

    ... Central Characterization Project's Remote-Handled Transuranic Waste Characterization Program at Bettis... radioactive remote-handled (RH) transuranic (TRU) waste characterization program implemented by the Central Characterization Project (CCP) at Bettis Atomic Power Laboratory (BAPL) in West Mifflin, Pennsylvania. This waste...

  6. [Role of myostatin in wasting syndrome associated with chronic diseases].

    PubMed

    Zamora, Elisabet; Galán, Amparo; Simó, Rafael

    2008-11-01

    Muscle wasting is a common process of numerous chronic diseases. Sarcopenia is associated with poor prognosis independently of the outcome of the disease. To date, the mechanisms by which sarcopenia induces these alterations are unknown, but the complexity of muscular metabolism anticipates that many factors can be involved. Myostatin, a new family member of transforming growth factor beta, was initially described from the observation of significant muscular growing in knock out mice for myostatin. Numerous experimental and clinical studies have provided insights in the physiologic knowledge of this protein and its implication in muscle wasting conditions. In recent years different substances have been described that counteract myostatin through numerous physiopathological mecanisms and, therefore, they might be novel therapeutic strategies against the wasting syndrome associated with chronic diseases. In spite of that, more studies are needed to improve the knowledge of all processes involved in muscle wasting in order to prevent its devastating consequences.

  7. DOE's Remote-Handled TRU Waste Characterization Program: Implementation Plan

    EPA Pesticide Factsheets

    Remote-handled (RH) transuranic (TRU) waste characterization, which involves obtaining chemical, radiological, and physical data, is a primary component of ensuring compliance of the Waste Isolation Pilot Plant (WIPP) with regulatory requirements.

  8. Waste Sampling & Characterization Facility (WSCF) Complex Safety Analysis

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    MELOY, R.T.

    2002-04-01

    This document was prepared to analyze the Waste Sampling and Characterization Facility for safety consequences by: Determining radionuclide and highly hazardous chemical inventories; Comparing these inventories to the appropriate regulatory limits; Documenting the compliance status with respect to these limits; and Identifying the administrative controls necessary to maintain this status. The primary purpose of the Waste Sampling and Characterization Facility (WSCF) is to perform low-level radiological and chemical analyses on various types of samples taken from the Hanford Site. These analyses will support the fulfillment of federal, Washington State, and Department of Energy requirements.

  9. Radiological Characterization Methodology of INEEL Stored RH-TRU Waste from ANL-E

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Rajiv N. Bhatt

    2003-02-01

    An Acceptable Knowledge (AK)-based radiological characterization methodology is being developed for RH TRU waste generated from ANL-E hot cell operations performed on fuel elements irradiated in the EBR-II reactor. The methodology relies on AK for composition of the fresh fuel elements, their irradiation history, and the waste generation and collection processes. Radiological characterization of the waste involves the estimates of the quantities of significant fission products and transuranic isotopes in the waste. Methods based on reactor and physics principles are used to achieve these estimates. Because of the availability of AK and the robustness of the calculation methods, the AK-basedmore » characterization methodology offers a superior alternative to traditional waste assay techniques. Using this methodology, it is shown that the radiological parameters of a test batch of ANL-E waste is well within the proposed WIPP Waste Acceptance Criteria limits.« less

  10. Characterization of the MVST waste tanks located at ORNL

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Keller, J.M.; Giaquinto, J.M.; Meeks, A.M.

    During the fall of 1996 there was a major effort to sample and analyze the Active Liquid Low-Level Waste (LLLW) tanks at ORNL which include the Melton Valley Storage Tanks (MVST) and the Bethel Valley Evaporator Service Tanks (BVEST). The characterization data summarized in this report was needed to address waste processing options, address concerns of the performance assessment (PA) data for the Waste Isolation Pilot Plant (WIPP), evaluate the characteristics with respect to the waste acceptance criteria (WAC) for WIPP and Nevada Test Site (NTS), address criticality concerns, and meet DOT requirements for transporting the waste. This report onlymore » discusses the analytical characterization data for the MVST waste tanks. The isotopic data presented in this report support the position that fissile isotopes of uranium and plutonium were ``denatured`` as required by administrative controls. In general, MVST sludge was found to be both hazardous by RCRA characteristics and the transuranic alpha activity was well about the limit for TRU waste. The characteristics of the MVST sludge relative to the WIPP WAC limits for fissile gram equivalent, plutonium equivalent activity, and thermal power from decay heat, were estimated from the data in this report and found to be far below the upper boundary for any of the remote-handled transuranic waste requirements for disposal of the waste in WIPP.« less

  11. Speciation and Characterization of E-Waste, Using Analytical Techniques

    NASA Astrophysics Data System (ADS)

    López, C. Cortés; Cruz, V. E. Reyes; Rodríguez, M. A. Veloz; Ávila, J. Hernández; Badillo, J. Flores; Murcia, J. A. Cobos

    Electronic waste (e-waste), have a high potential as a source of precious metals, since they can contain metals like silver, gold, platinum, copper, zinc, nickel, tin and others. In this paper some e-waste were characterized using several analytical techniques as Scanning Electron Microscopy (SEM), X-ray diffraction (XRD) and inductively coupled plasma (ICP) in addition to the thermodynamic study by Pourbaix diagrams of silver (Ag), gold (Au), platinum (Pt), copper (Cu), nickel (Ni), tin (Sn) and zinc (Zn); considering an average low concentration of HNO3 (10% v/v). With results of the characterization was determined that the e-waste is an ideal source for the recovery of valuable metals. Similarly, the thermodynamic studies showed that it is possible to obtain all metallic species except Pt, in a potential window of 1.45V to 2.0V vs SCE.

  12. Characterization of the solid low level mixed waste inventory for the solid waste thermal treatment activity - III

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Place, B.G., Westinghouse Hanford

    1996-09-24

    The existing thermally treatable, radioactive mixed waste inventory is characterized to support implementation of the commercial, 1214 thermal treatment contract. The existing thermally treatable waste inventory has been identified using a decision matrix developed by Josephson et al. (1996). Similar to earlier waste characterization reports (Place 1993 and 1994), hazardous materials, radionuclides, physical properties, and waste container data are statistically analyzed. In addition, the waste inventory data is analyzed to correlate waste constituent data that are important to the implementation of the commercial thermal treatment contract for obtaining permits and for process design. The specific waste parameters, which were analyzed,more » include the following: ``dose equivalent`` curie content, polychlorinated biphenyl (PCB) content, identification of containers with PA-related mobile radionuclides (14C, 12 79Se, 99Tc, and U isotopes), tritium content, debris and non-debris content, container free liquid content, fissile isotope content, identification of dangerous waste codes, asbestos containers, high mercury containers, beryllium dust containers, lead containers, overall waste quantities, analysis of container types, and an estimate of the waste compositional split based on the thermal treatment contractor`s proposed process. A qualitative description of the thermally treatable mixed waste inventory is also provided.« less

  13. Fetal urine biochemistry in antenatal Bartter syndrome: a case report.

    PubMed

    Rachid, Myriam L; Dreux, Sophie; Czerkiewicz, Isabelle; Deschênes, Georges; Vargas-Poussou, Rosa; Mahieu-Caputo, Dominique; Oury, Jean-François; Muller, Françoise

    2016-09-01

    Bartter syndrome is a severe inherited tubulopathy responsible for renal salt wasting, and hence electrolyte disorders and dehydration. Prenatally, it is characterized by severe polyhydramnios caused by fetal polyuria. We studied for the first time fetal urine in a Bartter syndrome case and demonstrated that the tubulopathy is already present at 24 weeks of gestation.

  14. Assessment of remote sensing technologies to discover and characterize waste sites

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    NONE

    1992-03-11

    This report presents details about waste management practices that are being developed using remote sensing techniques to characterize DOE waste sites. Once the sites and problems have been located and characterized and an achievable restoration and remediation program have been established, efforts to reclaim the environment will begin. Special problems to be considered are: concentrated waste forms in tanks and pits; soil and ground water contamination; ground safety hazards for workers; and requirement for long-term monitoring.

  15. I-NERI-2007-004-K, DEVELOPMENT AND CHARACTERIZATION OF NEW HIGH-LEVEL WASTE FORMS FOR ACHIEVING WASTE MINIMIZATION FROM PYROPROCESSING

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    S.M. Frank

    Work describe in this report represents the final year activities for the 3-year International Nuclear Energy Research Initiative (I-NERI) project: Development and Characterization of New High-Level Waste Forms for Achieving Waste Minimization from Pyroprocessing. Used electrorefiner salt that contained actinide chlorides and was highly loaded with surrogate fission products was processed into three candidate waste forms. The first waste form, a high-loaded ceramic waste form is a variant to the CWF produced during the treatment of Experimental Breeder Reactor-II used fuel at the Idaho National Laboratory (INL). The two other waste forms were developed by researchers at the Korean Atomicmore » Energy Research Institute (KAERI). These materials are based on a silica-alumina-phosphate matrix and a zinc/titanium oxide matrix. The proposed waste forms, and the processes to fabricate them, were designed to immobilize spent electrorefiner chloride salts containing alkali, alkaline earth, lanthanide, and halide fission products that accumulate in the salt during the processing of used nuclear fuel. This aspect of the I-NERI project was to demonstrate 'hot cell' fabrication and characterization of the proposed waste forms. The outline of the report includes the processing of the spent electrorefiner salt and the fabrication of each of the three waste forms. Also described is the characterization of the waste forms, and chemical durability testing of the material. While waste form fabrication and sample preparation for characterization must be accomplished in a radiological hot cell facility due to hazardous radioactivity levels, smaller quantities of each waste form were removed from the hot cell to perform various analyses. Characterization included density measurement, elemental analysis, x-ray diffraction, scanning electron microscopy and the Product Consistency Test, which is a leaching method to measure chemical durability. Favorable results from this demonstration

  16. Adequacy of a Small Quantity Site RH-TRU Waste Program in Meeting Proposed WIPP Characterization Objectives

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Biedscheid, J.; Stahl, S.; Devarakonda, M.

    2002-02-26

    The first remote-handled transuranic (RH-TRU) waste is expected to be permanently disposed of at the Waste Isolation Pilot Plant (WIPP) during Fiscal Year (FY) 2003. The first RH-TRU waste shipments are scheduled from the Battelle Columbus Laboratories (BCL) to WIPP in order to facilitate compliance with BCL Decommissioning Project (BCLDP) milestones. Milestones requiring RH-TRU waste containerization and removal from the site by 2004 in order to meet a 2006 site closure goal, established by Congress in the Defense Facilities Closure Projects account, necessitated the establishment and implementation of a site-specific program to direct the packaging of BCLDP RH-TRU waste priormore » to the finalization of WIPP RH-TRU waste characterization requirements. The program was designed to collect waste data, including audio and videotape records of waste packaging, such that upon completion of waste packaging, comprehensive data records exist from which compliance with final WIPP RH-TRU waste characterization requirements can be demonstrated. With the BCLDP data records generated to date and the development by the U.S. Department of Energy (DOE)-Carlsbad Field Office (CBFO) of preliminary documents proposing the WIPP RH-TRU waste characterization program, it is possible to evaluate the adequacy of the BCLDP program with respect to meeting proposed characterization objectives. The BCLDP characterization program uses primarily acceptable knowledge (AK) and visual examination (VE) during waste packaging to characterize RH-TRU waste. These methods are used to estimate physical waste parameters, including weight percentages of metals, cellulosics, plastics, and rubber in the waste, and to determine the absence of prohibited items, including free liquids. AK combined with computer modeling is used to estimate radiological waste parameters, including total activity on a waste container basis, for the majority of BCLDP RH-TRU waste. AK combined with direct analysis is used to

  17. Inducible nitric oxide synthase (iNOS) in muscle wasting syndrome, sarcopenia, and cachexia

    PubMed Central

    Hall, Derek T.; Ma, Jennifer F.; Di Marco, Sergio; Gallouzi, Imed-Eddine

    2011-01-01

    Muscle atrophy—also known as muscle wasting—is a debilitating syndrome that slowly develops with age (sarcopenia) or rapidly appears at the late stages of deadly diseases such as cancer, AIDS, and sepsis (cachexia). Despite the prevalence and the drastic detrimental effects of these two syndromes, there are currently no widely used, effective treatment options for those suffering from muscle wasting. In an attempt to identify potential therapeutic targets, the molecular mechanisms of sarcopenia and cachexia have begun to be elucidated. Growing evidence suggests that inflammatory cytokines may play an important role in the pathology of both syndromes. As one of the key cytokines involved in both sarcopenic and cachectic muscle wasting, tumor necrosis factor α (TNFα) and its downstream effectors provide an enticing target for pharmacological intervention. However, to date, no drugs targeting the TNFα signaling pathway have been successful as a remedial option for the treatment of muscle wasting. Thus, there is a need to identify new effectors in this important pathway that might prove to be more efficacious targets. Inducible nitric oxide synthase (iNOS) has recently been shown to be an important mediator of TNFα-induced cachectic muscle loss, and studies suggest that it may also play a role in sarcopenia. In addition, investigations into the mechanism of iNOS-mediated muscle loss have begun to reveal potential therapeutic strategies. In this review, we will highlight the potential for targeting the iNOS/NO pathway in the treatment of muscle loss and discuss its functional relevance in sarcopenia and cachexia. PMID:21832306

  18. HOUSEHOLD HAZARDOUS WASTE CHARACTERIZATION STUDY FOR PALM BEACH COUNTY, FLORIDA - A MITE PROGRAM EVALUATION

    EPA Science Inventory

    The objectives of the Household Hazardous Waste Characterization Study (the HHW Study) were to: 1) Quantity the annual household hazardous waste (HHW) tonnages disposed in Palm Beach County Florida’s (the County) residential solid waste (characterized in this study as municipal s...

  19. Activation and characterization of waste coffee grounds as bio-sorbent

    NASA Astrophysics Data System (ADS)

    Mariana; Marwan; Mulana, F.; Yunardi; Ismail, T. A.; Hafdiansyah, M. F.

    2018-03-01

    As the city well known for its culture of coffee drinkers, modern and traditional coffee shops are found everywhere in Banda Aceh, Indonesia. High number of coffee shops in the city generates large quantities of spent coffee grounds as waste without any effort to convert them as other valuable products. In an attempt to reduce environmental problems caused by used coffee grounds, this research was conducted to utilize waste coffee grounds as an activated carbon bio-sorbent. The specific purpose of this research is to improve the performance of coffee grounds bio-sorbent through chemical and physical activation, and to characterize the produced bio-sorbent. Following physical activation by carbonization, a chemical activation was achieved by soaking the carbonized waste coffee grounds in HCl solvent and carbonization process. The activated bio-sorbent was characterized for its morphological properties using Scanning Electron Microscopy (SEM), its functional groups by Fourier Transform Infra-Red Spectrophotometer (FTIR), and its material characteristics using X-Ray Diffraction (XRD). Characterization of the activated carbon prepared from waste coffee grounds shows that it meets standard quality requirement in accordance with Indonesian National Standard, SNI 06-3730-1995. Activation process has modified the functional groups of the waste coffee grounds. Comparing to natural waste coffee grounds, the resulted bio-sorbent demonstrated a more porous surface morphology following activation process. Consequently, such bio-sorbent is a potential source to be used as an adsorbent for various applications.

  20. Characterization of Fernald Silo 3 Waste

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Langton, C.A.

    This report summarizes characterization results for uranium residues from the Fernald Environmental Management Project (FEMP) Operable Unit (OU-4). These residues are currently stored in a one-million-gallon concrete silo, Silo 3, at the DOE Fernald Site, Ohio. Characterization of the Silo 3 waste is the first part of a three part study requested by Rocky Mountain Remedial Services (RMRS) through a Work for others Agreement, WFO-00-007, between the Westinghouse Savannah River Company (WSRC) and RMRS. Parts 2 and 3 of this effort include bench- and pilot-scale testing.

  1. 1QCY17 Saltstone waste characterization analysis

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Johnson, F. C.

    2017-07-25

    In the first quarter of calendar year 2017, a salt solution sample was collected from Tank 50 on January 16, 2017 in order to meet South Carolina (SC) Regulation 61-107.19 Part I C, “Solid Waste Management: Solid Waste Landfills and Structural Fill – General Requirements” and the Saltstone Disposal Facility Class 3 Landfill Permit. The Savannah River National Laboratory (SRNL) was requested to prepare and ship saltstone samples to a United States Environmental Protection Agency (EPA) certified laboratory to perform the Toxicity Characteristic Leaching Procedure (TCLP) and subsequent characterization.

  2. Impact of endocrine hyperfunction and phosphate wasting on bone in McCune-Albright syndrome.

    PubMed

    Lala, R; Matarazzo, P; Andreo, M; Defilippi, C; de Sanctis, C

    2002-01-01

    Skin dysplasia, as café-au-lait spots, bone fibrous dysplasia and peripheral endocrinopathies are the main clinical features of McCune-Albright syndrome (MAS). This illness is due to activating mutations of the Gsalpha protein and is spread with a mosaic pattern in affected tissues that consist of intermixed areas of normal and mutated cells. Peripheral endocrine secretion, free of hypothalamic pituitary control, is the hallmark of the endocrine syndromes: precocious puberty, Cushing's syndrome, hyperthyroidism and gigantism/acromegaly. In addition, phosphate wasting as hyperphosphaturia is often present. The impact of hormonal hypersecretion and phosphate loss on the bones of patients with MAS is poorly understood both in normal and fibrous bone tissue. As hypercortisolism and hyperthyroidism increase bone resorption, hyperestrogenism and growth hormone hypersecretion stimulate bone growth and mineralization, and phosphate wasting reduces bone mineral content. All these actions can be exerted at varying times and degrees in a single patient on lesional and non-lesional bones. Sonographic evidence of multiple diffused hyperechogenic spots in the testes of patients with MAS do not seem to be related to alterations in calcium-phosphate metabolism but rather to zonal dysplasia/hyperplasia of testicular tissue.

  3. Solid waste characterization in Ketao, a rural town in Togo, West Africa.

    PubMed

    Edjabou, Maklawe Essonanawe; Møller, Jacob; Christensen, Thomas H

    2012-07-01

    In Africa the majority of solid waste data is for big cities. Small and rural towns are generally neglected and waste data from these areas are often unavailable, which makes planning a proper solid waste management difficult. This paper presents the results from two waste characterization projects conducted in Kétao, a rural town in Togo during the rainy season and the dry season in 2010. The seasonal variation has a significant impact on the waste stream. The household waste generation rate was estimated at 0.22 kg person(-1) day(-1) in the dry season and 0.42 in the rainy season. Likewise, the waste moisture content was 4% in the dry season while it was 33-63% in the rainy season. The waste consisted mainly of soil and dirt characterized as 'other' (41%), vegetables and putrescibles (38%) and plastic (11%). In addition to these fractions, considerable amounts of material are either recycled or reused locally and do not enter the waste stream. The study suggests that additional recycling is not feasible, but further examination of the degradability of the organic fraction is needed in order to assess whether the residual waste should be composed or landfilled.

  4. Reduced bone density in androgen-deficient women with acquired immune deficiency syndrome wasting.

    PubMed

    Huang, J S; Wilkie, S J; Sullivan, M P; Grinspoon, S

    2001-08-01

    Women with acquired immune deficiency syndrome wasting are at an increased risk of osteopenia because of low weight, changes in body composition, and hormonal alterations. Although women comprise an increasing proportion of human immunodeficiency virus-infected patients, prior studies have not investigated bone loss in this expanding population of patients. In this study we investigated bone density, bone turnover, and hormonal parameters in 28 women with acquired immune deficiency syndrome wasting and relative androgen deficiency (defined as free testosterone < or =3.0 pg/ml, weight < or =90% ideal body weight, weight loss > or =10% from preillness maximum weight, or weight <100% ideal body weight with weight loss > or =5% from preillness maximum weight). Total body (1.04 +/- 0.08 vs. 1.10 +/- 0.07 g/cm2, human immunodeficiency virus-infected vs. control respectively; P < 0.01), anteroposterior lumbar spine (0.94 +/- 0.12 vs. 1.03 +/- 0.09 g/cm2; P = 0.005), lateral lumbar spine (0.71 +/- 0.14 vs. 0.79 +/- 0.09 g/cm2; P = 0.02), and hip (Ward's triangle; 0.68 +/- 0.14 vs. 0.76 +/- 0.12 g/cm2; P = 0.05) bone density were reduced in the human immunodeficiency virus-infected compared with control subjects. Serum N-telopeptide, a measure of bone resorption, was increased in human immunodeficiency virus-infected patients, compared with control subjects (14.6 +/- 5.8 vs. 11.3 +/- 3.8 nmol/liter bone collagen equivalents, human immunodeficiency virus-infected vs. control respectively; P = 0.03). Although body mass index was similar between the groups, muscle mass was significantly reduced in the human immunodeficiency virus-infected vs. control subjects (16 +/- 4 vs. 21 +/- 4 kg, human immunodeficiency virus-infected vs. control, respectively; P < 0.0001). In univariate regression analysis, muscle mass (r = 0.53; P = 0.004) and estrogen (r = 0.51; P = 0.008), but not free testosterone (r = -0.05, P = 0.81), were strongly associated with lumbar spine bone density in the

  5. Alternative Radiological Characterization of Sealed Source TRU Waste for WIPP Disposal (LAUR-05-8776)

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Whitworth, J.; Pearson, M.; Feldman, A.

    2006-07-01

    The Offsite Source Recovery (OSR) Project at Los Alamos National Laboratory is now shipping transuranic (TRU) waste containers to the Waste Isolation Pilot Plant (WIPP) in New Mexico for disposal. Sealed source waste disposal has become possible in part because OSR personnel were able to obtain Environmental Protection Agency (EPA) and DOE-CBFO approval for an alternative radiological characterization procedure relying on acceptable knowledge (AK) and modeling, rather than on non-destructive assay (NDA) of each container. This is the first successful qualification of an 'alternate methodology' under the radiological characterization requirements of the WIPP Waste Acceptance Criteria (WAC) by any TRUmore » waste generator site. This paper describes the approach OSR uses to radiologically characterize its sealed source waste and the process by which it obtained certification of this approach. (authors)« less

  6. Phenotypic and genotypic characterization of clinically relevant bacteria isolated from dental waste and waste workers' hands, mucosas and coats.

    PubMed

    Tagliaferri, T L; Vieira, C D; de Carvalho, M A R; Ladeira, L C D; Magalhães, P P; de Macêdo Farias, L; Dos Santos, S G

    2017-10-01

    Infectious wastes are potential sources of pathogenic micro-organisms, which may represent a risk to the professionals who manage them. In this study, we aimed to characterize the infectious bacteria present in dental waste and waste workers. The dental waste produced over 24 h was collected and waste workers were sampled by swabbing. Isolate resistance profiles were characterized by Vitek ® and PCR and biofilm formation by Congo Red agar, string test and microtitre assay. To assess similarity between the waste and the workers' samples, a random amplified polymorphic DNA test was used. Twenty-eight bacteria were identified as clinically relevant. The most frequent gene was bla TEM present in five Gram-negative micro-organisms, and one bla SHV in Klebsiella pneumoniae. All Pseudomonas aeruginosa were positive to extracellular polymeric substances formation, except one isolated from a worker. Klebsiella pneumoniae had negative results for the string test. Pseudomonas aeruginosa showed better adherence at 25°C after 48 h of incubation and K. pneumonia had the best biofilm formation at the same temperature, after 24 h. The similarity between P. aeruginosa recovered from dental waste and from workers was low, however, it is important to note that a pathogen was found on a worker's hands and that improvements in biosafety are required. Infectious dental waste can contain clinically relevant bacteria with important resistance and biofilm profiles. These micro-organisms could be transmitted to waste workers, other professionals and patients if the principles of biosafety measures are neglected. To our knowledge, no study has ever evaluated the microbial characterization and the potential contamination risk of dental infectious waste and waste handlers. The presence of clinically relevant bacteria in the hands and nasal mucosa of waste workers highlights the need for studies in this field to clarify the risk of these pathogens in dental healthcare services, and to

  7. The Advancement of Public Awareness, Concerning TRU Waste Characterization, Using a Virtual Document

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    West, T. B.; Burns, T. P.; Estill, W. G.

    2002-02-28

    Building public trust and confidence through openness is a goal of the DOE Carlsbad Field Office for the Waste Isolation Pilot Plant (WIPP). The objective of the virtual document described in this paper is to give the public an overview of the waste characterization steps, an understanding of how waste characterization instrumentation works, and the type and amount of data generated from a batch of drums. The document is intended to be published on a web page and/or distributed at public meetings on CDs. Users may gain as much information as they desire regarding the transuranic (TRU) waste characterization program,more » starting at the highest level requirements (drivers) and progressing to more and more detail regarding how the requirements are met. Included are links to: drivers (which include laws, permits and DOE Orders); various characterization steps required for transportation and disposal under WIPP's Hazardous Waste Facility Permit; physical/chemical basis for each characterization method; types of data produced; and quality assurance process that accompanies each measurement. Examples of each type of characterization method in use across the DOE complex are included. The original skeleton of the document was constructed in a PowerPoint presentation and included descriptions of each section of the waste characterization program. This original document had a brief overview of Acceptable Knowledge, Non-Destructive Examination, Non-Destructive Assay, Small Quantity sites, and the National Certification Team. A student intern was assigned the project of converting the document to a virtual format and to discuss each subject in depth. The resulting product is a fully functional virtual document that works in a web browser and functions like a web page. All documents that were referenced, linked to, or associated, are included on the virtual document's CD. WIPP has been engaged in a variety of Hazardous Waste Facility Permit modification activities. During

  8. COTARD SYNDROME IN SEMANTIC DEMENTIA

    PubMed Central

    Mendez, Mario F.; Ramírez-Bermúdez, Jesús

    2011-01-01

    Background Semantic dementia is a neurodegenerative disorder characterized by the loss of meaning of words or concepts. semantic dementia can offer potential insights into the mechanisms of content-specific delusions. Objective The authors present a rare case of semantic dementia with Cotard syndrome, a delusion characterized by nihilism or self-negation. Method The semantic deficits and other features of semantic dementia were evaluated in relation to the patient's Cotard syndrome. Results Mrs. A developed the delusional belief that she was wasting and dying. This occurred after she lost knowledge for her somatic discomforts and sensations and for the organs that were the source of these sensations. Her nihilistic beliefs appeared to emerge from her misunderstanding of her somatic sensations. Conclusion This unique patient suggests that a mechanism for Cotard syndrome is difficulty interpreting the nature and source of internal pains and sensations. We propose that loss of semantic knowledge about one's own body may lead to the delusion of nihilism or death. PMID:22054629

  9. EVALUATION OF RISKS AND WASTE CHARACTERIZATION REQUIREMENTS FOR THE TRANSURANIC WASTE EMPLACED IN WIPP DURING 1999

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Channell, J.K.; Walker, B.A.

    2000-05-01

    Specifically this report: 1. Compares requirements of the WAP that are pertinent from a technical viewpoint with the WIPP pre-Permit waste characterization program, 2. Presents the results of a risk analysis of the currently emplaced wastes. Expected and bounding risks from routine operations and possible accidents are evaluated; and 3. Provides conclusions and recommendations.

  10. Site characterization report for the basalt waste isolation project. Volume II

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    None

    1982-11-01

    The reference location for a repository in basalt for the terminal storage of nuclear wastes on the Hanford Site and the candidate horizons within this reference repository location have been identified and the preliminary characterization work in support of the site screening process has been completed. Fifteen technical questions regarding the qualification of the site were identified to be addressed during the detailed site characterization phase of the US Department of Energy-National Waste Terminal Storage Program site selection process. Resolution of these questions will be provided in the final site characterization progress report, currently planned to be issued in 1987,more » and in the safety analysis report to be submitted with the License Application. The additional information needed to resolve these questions and the plans for obtaining the information have been identified. This Site Characterization Report documents the results of the site screening process, the preliminary site characterization data, the technical issues that need to be addressed, and the plans for resolving these issues. Volume 2 contains chapters 6 through 12: geochemistry; surface hydrology; climatology, meteorology, and air quality; environmental, land-use, and socioeconomic characteristics; repository design; waste package; and performance assessment.« less

  11. Greater-than-Class C low-level waste characterization

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Piscitella, R.R.

    1991-12-31

    In 1985, Public Law 99-240 (Low-Level Radioactive Waste Policy Amendments Act of 1985) made the Department of Energy (DOE) responsible for the disposal of greater-than-Class C low-level radioactive waste (GTCC LLW). DOE strategies for storage and disposal of GTCC LLW required characterization of volumes, radionuclide activities, and waste forms. Data from existing literature, disposal records, and original research were used to estimate characteristics, project volumes, and determine radionuclide activities to the years 2035 and 2055. Twenty-year life extensions for 70% of the operating nuclear reactors were assumed to calculate the GTCC LLW available in 2055. The following categories of GTCCmore » LLW were addressed: Nuclear Utilities Waste; Potential Sealed Sources GTCC LLW; DOE-Held Potential GTCC LLW; and Other Generator Waste. It was determined that the largest volume of these wastes, approximately 57%, is generated by nuclear utilities. The Other Generator Waste category contributes approximately 10% of the total GTCC LLW volume projected to the year 2035. DOE-Held Potential GTCC LLW accounts for nearly 33% of all waste projected to the year 2035. Potential Sealed Sources GTCC LLW is less than 0.2% of the total projected volume. The base case total projected volume of GTCC LLW for all categories was 3,250 cubic meters. This was substantially less than previous estimates.« less

  12. Characterization of pain, disability, and psychological burden in Marfan syndrome.

    PubMed

    Speed, Traci J; Mathur, Vani A; Hand, Matthew; Christensen, Bryt; Sponseller, Paul D; Williams, Kayode A; Campbell, Claudia M

    2017-02-01

    The clinical manifestations of Marfan syndrome frequently cause pain. This study aimed to characterize pain in a cohort of adults with Marfan syndrome and investigate demographic, physical, and psychological factors associated with pain and pain-related disability. Two hundred and forty-five participants (73% female, 89% non-Hispanic white, 90% North American) completed an online questionnaire assessing clinical features of Marfan syndrome, pain severity, pain-related disability, physical and mental health, depressive symptoms, pain catastrophizing, and insomnia. Eighty-nine percent of respondents reported having pain with 28% of individuals reporting pain as a presenting symptom of Marfan syndrome. Almost half of individuals reported that pain has spread from its initial site. Participants in our study reported poor physical and mental health functioning, moderate pain-related disability, and mild levels of depressive symptoms, sleep disturbances, and pain catastrophizing. Those who identified pain as an initial symptom of Marfan syndrome and those who reported that pain had spread from its initial site reported greater psychological burden compared with those without pain as an initial symptom or pain spreading. Physical health is the largest predictor of pain severity and pain-related disability. While pain catastrophizing and worse mental health functioning are significant correlates of pain severity and pain-related disability, respectively. Pain is a significant and persistent problem in Marfan syndrome and is associated with profound disability and psychological burden. Further studies are indicated to better characterize the directionality of pain, pain-related disability, and psychological burden in Marfan syndrome. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  13. Associations of Dietary Protein and Energy Intakes With Protein-Energy Wasting Syndrome in Hemodialysis Patients.

    PubMed

    Beddhu, Srinivasan; Wei, Guo; Chen, Xiaorui; Boucher, Robert; Kiani, Rabia; Raj, Dominic; Chonchol, Michel; Greene, Tom; Murtaugh, Maureen A

    2017-09-01

    The associations of dietary protein and/or energy intakes with protein or energy wasting in patients on maintenance hemodialysis are controversial. We examined these in the Hemodialysis (HEMO) Study. In 1487 participants in the HEMO Study, baseline dietary protein intake (grams per kilogram per day) and dietary energy intake (kilocalories per kilograms per day) were related to the presence of the protein-energy wasting (PEW) syndrome at month 12 (defined as the presence of at least 1 criteria in 2 of the 3 categories of low serum chemistry, low body mass, and low muscle mass) in logistic regression models. In additional separate models, protein intake estimated from equilibrated normalized protein catabolic rate (enPCR) was also related to the PEW syndrome. Compared with the lowest quartile, the highest quartile of baseline dietary protein intake was paradoxically associated with increased risk of the PEW syndrome at month 12 (odds ratio [OR]: 4.11; 95% confidence interval [CI]: 2.79-6.05). This relationship was completely attenuated (OR: 1.35; 95% CI: 0.88-2.06) with adjustment for baseline body weight, which suggested mathematical coupling. Results were similar for dietary energy intake. Compared with the lowest quartile of baseline enPCR, the highest quartile was not associated with the PEW syndrome at 12 months (OR: 0.78; 95% CI: 0.54-1.12). These data do not support the use of dietary protein intake or dietary energy intake criteria in the definition of the PEW syndrome in patients on maintenance hemodialysis.

  14. Assessment of multiple geophysical techniques for the characterization of municipal waste deposit sites

    NASA Astrophysics Data System (ADS)

    Gaël, Dumont; Tanguy, Robert; Nicolas, Marck; Frédéric, Nguyen

    2017-10-01

    In this study, we tested the ability of geophysical methods to characterize a large technical landfill installed in a former sand quarry. The geophysical surveys specifically aimed at delimitating the deposit site horizontal extension, at estimating its thickness and at characterizing the waste material composition (the moisture content in the present case). The site delimitation was conducted with electromagnetic (in-phase and out-of-phase) and magnetic (vertical gradient and total field) methods that clearly showed the transition between the waste deposit and the host formation. Regarding waste deposit thickness evaluation, electrical resistivity tomography appeared inefficient on this particularly thick deposit site. Thus, we propose a combination of horizontal to vertical noise spectral ratio (HVNSR) and multichannel analysis of the surface waves (MASW), which successfully determined the approximate waste deposit thickness in our test landfill. However, ERT appeared to be an appropriate tool to characterize the moisture content of the waste, which is of prior information for the organic waste biodegradation process. The global multi-scale and multi-method geophysical survey offers precious information for site rehabilitation studies, water content mitigation processes for enhanced biodegradation or landfill mining operation planning.

  15. Waste-water characterization and hazardous-waste technical assistance survey, Bergstrom AFB tTxas. Final report, 6-15 March 1989

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hedgecock, N.S.

    1990-01-01

    At the request of 67 Combat Support Group/DEEV the Air Force Occupational and Environmental Health Laboratory conducted a waste-water characterization and hazardous-waste technical assistance survey at Bergstrom AFB (BAFB) from 6-15 Mar 89. The scope of the waste-water survey was to characterize the effluent exiting the base and the effluent from 23 industrial facilities and 10 food-serving facilities. The scope of the hazardous-waste survey was to address hazardous-waste-management practices and explore opportunities for hazardous waste minimization. Specific recommendations from the survey include: (1) Accompany City of Austin personnel during waste-water sampling procedures; (2) Sample at the manhole exiting the mainmore » lift station rather than at the lift station wet well; (3) Split waste-water samples with the City of Austin for comparison of results; (4) Ensure that oil/water separators and grease traps are functioning properly and are cleaned out regularly; (5) Limit the quantity of soaps and solvents discharged down the drain to the sanitary sewer; (6) Establish a waste disposal contract for the removal of wastes in the Petroleum Oils and Lubricants underground storage tanks. (7) Remove, analyze, and properly dispose of oil contaminated soil from accumulation sites. (8) Move indoors or secure, cover, and berm the aluminum sign reconditioning tank at 67 Civil Engineering Squadron Protective Coating. (9) Connect 67 Combat Repair Squadron Test Cell floor drains to the sanitary sewer.« less

  16. Characterization of the Old Hydrofracture Facility (OHF) waste tanks located at ORNL

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Keller, J.M.; Giaquinto, J.M.; Meeks, A.M.

    1997-04-01

    The Old Hydrofracture Facility (OHF) is located in Melton Valley within Waste Area Grouping (WAG) 5 and includes five underground storage tanks (T1, T2, T3, T4, and T9) ranging from 13,000 to 25,000 gal. capacity. During the period of 1996--97 there was a major effort to re-sample and characterize the contents of these inactive waste tanks. The characterization data summarized in this report was needed to address waste processing options, examine concerns dealing with the performance assessment (PA) data for the Waste Isolation Pilot Plant (WIPP), evaluate the waste characteristics with respect to the waste acceptance criteria (WAC) for WIPPmore » and Nevada Test Site (NTS), address criticality concerns, and to provide the data needed to meet DOT requirements for transporting the waste. This report discusses the analytical characterization data collected on both the supernatant and sludge samples taken from three different locations in each of the OHF tanks. The isotopic data presented in this report supports the position that fissile isotopes of uranium ({sup 233}U and {sup 235}U) do not satisfy the denature ratios required by the administrative controls stated in the ORNL LLLW waste acceptance criteria (WAC). The fissile isotope of plutonium ({sup 239}Pu and {sup 241}Pu) are diluted with thorium far above the WAC requirements. In general, the OHF sludge was found to be hazardous (RCRA) based on total metal content and the transuranic alpha activity was well above the 100 nCi/g limit for TRU waste. The characteristics of the OHF sludge relative to the WIPP WAC limits for fissile gram equivalent, plutonium equivalent activity, and thermal power from decay heat were estimated from the data in this report and found to be far below the upper boundary for any of the remote-handled transuranic waste (RH-TRU) requirements for disposal of the waste in WIPP.« less

  17. A new approach to characterize very-low-level radioactive waste produced at hadron accelerators.

    PubMed

    Zaffora, Biagio; Magistris, Matteo; Chevalier, Jean-Pierre; Luccioni, Catherine; Saporta, Gilbert; Ulrici, Luisa

    2017-04-01

    Radioactive waste is produced as a consequence of preventive and corrective maintenance during the operation of high-energy particle accelerators or associated dismantling campaigns. Their radiological characterization must be performed to ensure an appropriate disposal in the disposal facilities. The radiological characterization of waste includes the establishment of the list of produced radionuclides, called "radionuclide inventory", and the estimation of their activity. The present paper describes the process adopted at CERN to characterize very-low-level radioactive waste with a focus on activated metals. The characterization method consists of measuring and estimating the activity of produced radionuclides either by experimental methods or statistical and numerical approaches. We adapted the so-called Scaling Factor (SF) and Correlation Factor (CF) techniques to the needs of hadron accelerators, and applied them to very-low-level metallic waste produced at CERN. For each type of metal we calculated the radionuclide inventory and identified the radionuclides that most contribute to hazard factors. The methodology proposed is of general validity, can be extended to other activated materials and can be used for the characterization of waste produced in particle accelerators and research centres, where the activation mechanisms are comparable to the ones occurring at CERN. Copyright © 2017 Elsevier Ltd. All rights reserved.

  18. Characterization of a polyhydroxyalkanoate obtained from pineapple peel waste using Ralsthonia eutropha.

    PubMed

    Vega-Castro, Oscar; Contreras-Calderon, Jose; León, Emilson; Segura, Almir; Arias, Mario; Pérez, León; Sobral, Paulo J A

    2016-08-10

    Agro-industrial waste can be the production source of biopolymers such as polyhydroxyalkanoates. The aim of this study was to produce and characterize Polyhydroxyalkanoates produced from pineapple peel waste fermentation processes. The methodology includes different pineapple peel waste fermentation conditions. The produced biopolymer was characterized using FTIR, GC-MS and NMR. The best fermentation condition for biopolymer production was obtained using pH 9, Carbon/Nitrogen 11, carbon/phosphorus 6 and fermentation time of 60h. FTIR analyzes showed PHB group characteristics, such as OH, CH and CO. In addition, GC-MS showed two monomers with 4 and 8 carbons, referred to PHB and PHBHV. H(1) NMR analysis showed 0.88-0.97 and 5.27ppm signals, corresponding to CH3 and CH, respectively. In conclusion, polyhydroxyalkanoate production from pineapple peels waste is an alternative for the treatment of waste generated in Colombia's fruit industry. Copyright © 2016 Elsevier B.V. All rights reserved.

  19. (Low-level waste disposal facility siting and site characterization)

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Mezga, L.J.; Ketelle, R.H.; Pin, F.G.

    A US team consisting of representatives of Oak Ridge National Laboratory (ORNL), Savannah River Plant (SRP), Savannah river Laboratory (SRL), and the Department of Energy Office of Defense Waste and Byproducts Management participated in the fourth meeting held under the US/French Radioactive Waste Management Agreement between the US Department of Energy and the Commissariat a l'Energie Atomique. This meeting, held at Agence Nationale pour les Gestion des Dechets Radioactifs' (ANDRA's) Headquarters in Paris, was a detailed, technical topical workshop focusing on Low-Level Waste Disposal Facility Siting and Site Characterization.'' The meeting also included a visit to the Centre de lamore » Manche waste management facility operated by ANDRA to discuss and observe the French approach to low-level waste management. The final day of the meeting was spent at the offices of Societe Generale pour les Techniques Nouvelles (SGN) discussing potential areas of future cooperation and exchange. 20 figs.« less

  20. Wastes as Aggregates, Binders or Additions in Mortars: Selecting Their Role Based on Characterization.

    PubMed

    Farinha, Catarina Brazão; de Brito, Jorge; Veiga, Rosário; Fernández, J M; Jiménez, J R; Esquinas, A R

    2018-03-20

    The production of waste has increased over the years and, lacking a recycle or recovery solution, it is forwarded to landfill. The incorporation of wastes in cement-based materials is a solution to reduce waste deposition. In this regard, some researchers have been studying the incorporation of wastes with different functions: aggregate, binder and addition. The incorporation of wastes should take advantage of their characteristics. It requires a judicious analysis of their particles. This research involves the analysis of seven industrial wastes: biomass ashes, glass fibre, reinforced polymer dust, sanitary ware, fluid catalytic cracking, acrylic fibre, textile fibre and glass fibre. The main characteristics and advantages of each waste are enunciated and the best type of introduction in mortars is discussed. The characterization of the wastes as particles is necessary to identify the most suitable incorporation in mortars. In this research, some wastes are studied with a view to their re-use or recycling in mortars. Thus, this research focuses on the chemical, physical and mechanical characterization of industrial wastes and identification of the potentially most advantageous type of incorporation.

  1. Wastes as Aggregates, Binders or Additions in Mortars: Selecting Their Role Based on Characterization

    PubMed Central

    de Brito, Jorge; Veiga, Rosário

    2018-01-01

    The production of waste has increased over the years and, lacking a recycle or recovery solution, it is forwarded to landfill. The incorporation of wastes in cement-based materials is a solution to reduce waste deposition. In this regard, some researchers have been studying the incorporation of wastes with different functions: aggregate, binder and addition. The incorporation of wastes should take advantage of their characteristics. It requires a judicious analysis of their particles. This research involves the analysis of seven industrial wastes: biomass ashes, glass fibre, reinforced polymer dust, sanitary ware, fluid catalytic cracking, acrylic fibre, textile fibre and glass fibre. The main characteristics and advantages of each waste are enunciated and the best type of introduction in mortars is discussed. The characterization of the wastes as particles is necessary to identify the most suitable incorporation in mortars. In this research, some wastes are studied with a view to their re-use or recycling in mortars. Thus, this research focuses on the chemical, physical and mechanical characterization of industrial wastes and identification of the potentially most advantageous type of incorporation. PMID:29558418

  2. Microbial Characterization Space Solid Wastes Treated with a Heat Melt Compactor

    NASA Technical Reports Server (NTRS)

    Strayer, Richard F.; Hummerick, Mary E.; Richards, Jeffrey T.; McCoy LaShelle E.; Roberts, Michael S.; Wheeler, Raymond M.

    2012-01-01

    The on going purpose of the project efforts was to characterize and determine the fate of microorganisms in space-generated solid wastes before and after processing by candidate solid waste processing. For FY 11, the candidate technology that was assessed was the Heat Melt Compactor (HMC). The scope included five HMC. product disks produced at ARC from either simulated space-generated trash or from actual space trash, Volume F compartment wet waste, returned on STS 130. This project used conventional microbiological methods to detect and enumerate microorganisms in heat melt compaction (HMC) product disks as well as surface swab samples of the HMC hardware before and after operation. In addition, biological indicators were added to the STS trash prior to compaction in order to determine if these spore-forming bacteria could survive the HMC processing conditions, i.e., high temperature (160 C) over a long duration (3 hrs). To ensure that surface dwelling microbes did not contaminate HMC product disk interiors, the disk surfaces were sanitized with 70% alcohol. Microbiological assays were run before and after sanitization and found that sanitization greatly reduced the number of identified isolates but did not totally eliminate them. To characterize the interior of the disks, ten 1.25 cm diameter core samples were aseptically obtained for each disk. These were run through the microbial characterization analyses. Low counts of bacteria, on the order of 5 to 50 per core, were found, indicating that the HMC operating conditions might not be sufficient for waste sterilization. However, the direct counts were 6 to 8 orders of magnitude greater, indicating that the vast majority of microbes present in the wastes were dead or non-cultivable. An additional indication that the HMC was sterilizing the wastes was the results from the added commercial spore test strips to the wastes prior to HMC operation. Nearly all could be recovered from the HMC disks post-operation and all

  3. Radioactive Waste Characterization Strategies; Comparisons Between AK/PK, Dose to Curie Modeling, Gamma Spectroscopy, and Laboratory Analysis Methods- 12194

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Singledecker, Steven J.; Jones, Scotty W.; Dorries, Alison M.

    2012-07-01

    In the coming fiscal years of potentially declining budgets, Department of Energy facilities such as the Los Alamos National Laboratory (LANL) will be looking to reduce the cost of radioactive waste characterization, management, and disposal processes. At the core of this cost reduction process will be choosing the most cost effective, efficient, and accurate methods of radioactive waste characterization. Central to every radioactive waste management program is an effective and accurate waste characterization program. Choosing between methods can determine what is classified as low level radioactive waste (LLRW), transuranic waste (TRU), waste that can be disposed of under an Authorizedmore » Release Limit (ARL), industrial waste, and waste that can be disposed of in municipal landfills. The cost benefits of an accurate radioactive waste characterization program cannot be overstated. In addition, inaccurate radioactive waste characterization of radioactive waste can result in the incorrect classification of radioactive waste leading to higher disposal costs, Department of Transportation (DOT) violations, Notice of Violations (NOVs) from Federal and State regulatory agencies, waste rejection from disposal facilities, loss of operational capabilities, and loss of disposal options. Any one of these events could result in the program that mischaracterized the waste losing its ability to perform it primary operational mission. Generators that produce radioactive waste have four characterization strategies at their disposal: - Acceptable Knowledge/Process Knowledge (AK/PK); - Indirect characterization using a software application or other dose to curie methodologies; - Non-Destructive Analysis (NDA) tools such as gamma spectroscopy; - Direct sampling (e.g. grab samples or Surface Contaminated Object smears) and laboratory analytical; Each method has specific advantages and disadvantages. This paper will evaluate each method detailing those advantages and disadvantages

  4. Characterization of Class A low-level radioactive waste 1986--1990. Volume 6: Appendices G--J

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Dehmel, J.C.; Loomis, D.; Mauro, J.

    1994-01-01

    Under contract to the US Nuclear Regulatory Commission, Office of Nuclear Regulatory Research, the firms of S. Cohen & Associates, Inc. (SC&A) and Eastern Research Group (ERG) have compiled a report that describes the physical, chemical, and radiological properties of Class-A low-level radioactive waste. The report also presents information characterizing various methods and facilities used to treat and dispose non-radioactive waste. A database management program was developed for use in accessing, sorting, analyzing, and displaying the electronic data provided by EG&G. The program was used to present and aggregate data characterizing the radiological, physical, and chemical properties of the wastemore » from descriptions contained in shipping manifests. The data thus retrieved are summarized in tables, histograms, and cumulative distribution curves presenting radionuclide concentration distributions in Class-A waste as a function of waste streams, by category of waste generators, and regions of the United States. The report also provides information characterizing methods and facilities used to treat and dispose non-radioactive waste, including industrial, municipal, and hazardous waste regulated under Subparts C and D of the Resource Conservation and Recovery Act (RCRA). The information includes a list of disposal options, the geographical locations of the processing and disposal facilities, and a description of the characteristics of such processing and disposal facilities. Volume 1 contains the Executive Summary, Volume 2 presents the Class-A waste database, Volume 3 presents the information characterizing non-radioactive waste management practices and facilities, and Volumes 4 through 7 contain Appendices A through P with supporting information.« less

  5. Waste-water characterization and hazardous-waste technical assistance survey, Mather AFB California. Final report, 28 November-9 December 1988

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Scott, S.P.; Hedgecock, N.S.

    1989-10-01

    Personnel from the AFOEHL conducted a waste-water characterization and hazardous-waste technical assistance survey at MAFB from 28 Nov to 9 Dec 1988. The scope of this survey was to characterize the waste-water, address hazardous-waste-management practices, and explore opportunities for hazardous waste minimization. The waste water survey team analyzed the base's industrial effluent, effluent from oil/water separators, and storm water. The team performed a shop-by-shop evaluation of chemical-waste-management practices. Survey results showed that MAFB needs to improve its hazardous-waste-management program. Recommendations for improvement include: (1) Collecting two additional grab samples on separate days from the hospital discharge. Analyze for EPA Methodmore » 601 to determine if the grab sample from the survey gives a true indication of what is being discharged. (2) Locate the source and prevent mercury from the hospital from discharging into the sanitary sewer. (3) Dilute the soaps used for cleaning at the Fuels Lab, Building 7060. (4) Investigate the source of chromium from the Photo Lab. (5) Clean out the sewer system manhole directly downgradient from the Photo Lab. (6) Locate the source of contamination in the West Ditch Outfall. (7) Reconnect the two oil/water separators that discharge into the storm sewerage system. (8) Investigate the source of methylene chloride coming on the base. (9) Investigate the source of mercury at Fuel Cell Repair, building 7005.« less

  6. A cognitive characterization of dyscalculia in Turner syndrome.

    PubMed

    Bruandet, Marie; Molko, Nicolas; Cohen, Laurent; Dehaene, Stanislas

    2004-01-01

    Current theories of number processing postulate that the human abilities for arithmetic are based on cerebral circuits that are partially laid down under genetic control and later modified by schooling and education. This view predicts the existence of genetic diseases that interfere specifically with components of the number system. Here, we investigate whether Turner syndrome (TS) corresponds to this definition. TS is a genetic disorder which affects one woman in 2500 and is characterized by partial or complete absence of one X chromosome. In addition to well-characterized physical and hormonal dysfunction, TS patients exhibit cognitive deficits including dyscalculia. We tested 12 women with Turner syndrome and 13 control subjects on a cognitive battery including arithmetical tests (addition, subtraction, multiplication, division) as well as tests of the understanding of numerosity and quantity (cognitive estimation, estimation, comparison, bisection, subitizing/counting). Impairments were observed in cognitive estimation, subitizing, and calculation. We examine whether these deficits can be attributed to a single source, and discuss the possible implications of hormonal and genetic factors in the neuropsychological profile of TS patients.

  7. Composition, production rate and characterization of Greek dental solid waste.

    PubMed

    Mandalidis, Alexandros; Topalidis, Antonios; Voudrias, Evangelos A; Iosifidis, Nikolaos

    2018-05-01

    The overall objective of this work is to determine the composition, characterization and production rate of Greek dental solid waste (DSW). This information is important to design and cost management systems for DSW, for safety and health considerations and for assessing environmental impact. A total of 141 kg of DSW produced by a total of 2542 patients in 20 dental practices from Xanthi, Greece was collected, manually separated and weighed over a period of four working weeks. The waste was separated in 19 sub fractions, which were classified in 2 major categories, according to Greek regulations: Domestic-type waste comprising 8% and hazardous waste comprising 92% by weight of total DSW. The latter was further classified in infectious waste, toxic waste and mixed type waste (infectious and toxic together), accounting for 88.5%, 3.5% and 0.03% of total DSW by weight, respectively. The overall unit production rates (mean ± standard error of the mean) were 381 ± 15 g/practice/d and 53.3 ± 1.4 g/patient/d for total DSW, 337 ± 14 g/practice/d and 46.6 ± 1.2 g/patient/d for total infectious DSW, 13.4 ± 0.7 g/practice/d and 2.1 ± 0.1 g/patient/d for total toxic DSW and 30.4 ± 2.5 g/practice/d and 4.6 ± 0.4 g/patient/d for domestic-type waste. Daily DSW production was correlated with daily number of patients and regression correlations were produced. DSW was subject to laboratory characterization in terms of bulk density, calorific value, moisture, ash and volatile solids content. Measured calorific values were compared to predictions from empirical models. Copyright © 2018 Elsevier Ltd. All rights reserved.

  8. Radiological Characterization Methodology for INEEL-Stored Remote-Handled Transuranic (RH TRU) Waste from Argonne National Laboratory-East

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Kuan, P.; Bhatt, R.N.

    2003-01-14

    An Acceptable Knowledge (AK)-based radiological characterization methodology is being developed for RH TRU waste generated from ANL-E hot cell operations performed on fuel elements irradiated in the EBR-II reactor. The methodology relies on AK for composition of the fresh fuel elements, their irradiation history, and the waste generation and collection processes. Radiological characterization of the waste involves the estimates of the quantities of significant fission products and transuranic isotopes in the waste. Methods based on reactor and physics principles are used to achieve these estimates. Because of the availability of AK and the robustness of the calculation methods, the AK-basedmore » characterization methodology offers a superior alternative to traditional waste assay techniques. Using the methodology, it is shown that the radiological parameters of a test batch of ANL-E waste is well within the proposed WIPP Waste Acceptance Criteria limits.« less

  9. Characterization and recycling of cadmium from waste nickel-cadmium batteries.

    PubMed

    Huang, Kui; Li, Jia; Xu, Zhenming

    2010-11-01

    A severe threat was posed due to improper and inefficient recycling of waste batteries in China. The present work considered the fundamental aspects of the recycling of cadmium from waste nickel-cadmium batteries by means of vacuum metallurgy separation in scale-up. In the first stage of this work, the characterization of waste nickel-cadmium batteries was carried out. Five types of batteries from different brands and models were selected and their components were characterized in relation to their elemental chemical composition and main phase. In the second stage of this work, the parameters affecting the recycling of cadmium by means of vacuum metallurgy separation were investigated and a L(16) (4(4)) orthogonal design was applied to optimize the parameters. With the thermodynamics theory and numerical analysis, it can be seen that the orthogonal design is an effective tool for investigating the parameters affecting the recycling of cadmium. The optimum operating parameters for the recycling of cadmium obtained by orthogonal design and verification test were 1073 K (temperature), 2.5h (heating time), 2 wt.% (the addition of carbon powder), and 30 mm (the loaded height), respectively, with recycling efficiency approaching 99.98%. The XRD and ICP-AES analyzed results show that the condensed product was characterized as metallic cadmium, and cadmium purity was 99.99% under the optimum condition. Copyright © 2010. Published by Elsevier Ltd. All rights reserved.

  10. Gitelman's Syndrome Presenting with Hypocalcaemia - A Case Report.

    PubMed

    Cader, F A; Kabir, A; Mayedah, R; Masud, M S; Quadir, F; Hossen, M N

    2015-04-01

    Gitelman's syndrome is an autosomal recessive renal tubular disorder characterized by severe hypomagnesaemia, hypokalaemia, metabolic alkalosis and hypocalcaemia. It is caused by defective NaCl transport in the Distal Convoluted Tubule and presents in adolescence or adulthood, with a distinctly more benign course than Bartter's Syndrome. The dominant clinical features are muscle weakness, fatigue, carpopedal spasm, cramps and tetany. We report the case of a 26 year old male who presented with flaccid quadriparesis and carpopedal spasms, hypokalaemia, hypomagnesaemia, hypocalcaemia and severe urinary magnesium wasting. He was treated with potassium and magnesium supplementation and regained full function of all limbs.

  11. [The use of growth hormone to treat endocrine-metabolic disturbances in acquired immunodeficiency syndrome (AIDS) patients].

    PubMed

    Spinola-Castro, Angela Maria; Siviero-Miachon, Adriana A; da Silva, Marcos Tadeu Nolasco; Guerra-Junior, Gil

    2008-07-01

    Acquired Immunodeficiency Syndrome (Aids) was initially related to HIV-associated wasting syndrome, and its metabolic disturbances to altered body composition. After Highly Active Antiretroviral Therapy (HAART) was started, malnutrition has declined and HIV-associated lipodystrophy syndrome has emerged as an important metabolic disorder. Aids is also characterized by hormonal disturbances, principally in growth hormone/insulin-like growth factor 1 (GH/IGF-1) axis. The use of recombinant human GH (hrGH) was formerly indicated to treat wasting syndrome, in order to increase lean body mass. Even though the use of hrGH in lipodystrophy syndrome has been considered, the decrease in insulin sensitivity is a limitation for its use, which has not been officially approved yet. Diversity in therapeutic regimen is another limitation to its use in Aids patients. The present study has reviewed the main HIV-related endocrine-metabolic disorders as well as the use of hrGH in such conditions.

  12. Parry-Romberg syndrome: intracranial MRI appearances.

    PubMed

    Moko, Sheree Bernadette; Mistry, Yezdi; Blandin de Chalain, Tristan Maurice

    2003-10-01

    To gain further insight into the pathogenesis of Parry-Romberg syndrome, a sporadic disease of unknown aetiology characterized by progressive wasting of one side of the face. Cranial MRI was performed in 10 patients with Parry-Romberg syndrome. The central nervous system findings are correlated to clinical findings and a review of the literature. Three patients with a history of migraine had abnormal brain findings confined to the cerebral hemisphere ipsilateral to the facial hemiatrophy. Two patients without CNS symptoms had intracranial changes, one ipsilateral, the other both ipsilateral and contralateral to the facial hemiatrophy, on MRI. These changes consisted of either intracerebral atrophy or white matter hyperintensity. Five patients without CNS symptoms had no pathological intracranial MRI appearances. A significant number of patients with Parry-Romberg syndrome may have underlying brain involvement. These findings are consistent with previous reports.

  13. Characterization of 618-11 solid waste burial ground, disposed waste, and description of the waste generating facilities

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hladek, K.L.

    1997-10-07

    The 618-11 (Wye or 318-11) burial ground received transuranic (TRTJ) and mixed fission solid waste from March 9, 1962, through October 2, 1962. It was then closed for 11 months so additional burial facilities could be added. The burial ground was reopened on September 16, 1963, and continued operating until it was closed permanently on December 31, 1967. The burial ground received wastes from all of the 300 Area radioactive material handling facilities. The purpose of this document is to characterize the 618-11 solid waste burial ground by describing the site, burial practices, the disposed wastes, and the waste generatingmore » facilities. This document provides information showing that kilogram quantities of plutonium were disposed to the drum storage units and caissons, making them transuranic (TRU). Also, kilogram quantities of plutonium and other TRU wastes were disposed to the three trenches, which were previously thought to contain non-TRU wastes. The site burial facilities (trenches, caissons, and drum storage units) should be classified as TRU and the site plutonium inventory maintained at five kilograms. Other fissile wastes were also disposed to the site. Additionally, thousands of curies of mixed fission products were also disposed to the trenches, caissons, and drum storage units. Most of the fission products have decayed over several half-lives, and are at more tolerable levels. Of greater concern, because of their release potential, are TRU radionuclides, Pu-238, Pu-240, and Np-237. TRU radionuclides also included slightly enriched 0.95 and 1.25% U-231 from N-Reactor fuel, which add to the fissile content. The 618-11 burial ground is located approximately 100 meters due west of Washington Nuclear Plant No. 2. The burial ground consists of three trenches, approximately 900 feet long, 25 feet deep, and 50 feet wide, running east-west. The trenches constitute 75% of the site area. There are 50 drum storage units (five 55-gallon steel drums welded

  14. Report: new guidelines for characterization of municipal solid waste: the Portuguese case.

    PubMed

    da Graça Madeira Martinho, Maria; Silveira, Ana Isabel; Fernandes Duarte Branco, Elsa Maria

    2008-10-01

    This report proposes a new set of guidelines for the characterization of municipal solid waste. It is based on an analysis of reference methodologies, used internationally, and a case study of Valorsul (a company that handles recovery and treatment of solid waste in the North Lisbon Metropolitan Area). In particular, the suggested guidelines present a new definition of the waste to be analysed, change the sampling unit and establish statistical standards for the results obtained. In these new guidelines, the sampling level is the waste collection vehicle and contamination and moisture are taken into consideration. Finally, focus is on the quality of the resulting data, which is essential for comparability of data between countries. These new guidelines may also be applicable outside Portugal because the methodology includes, besides municipal mixed waste, separately collected fractions of municipal waste. They are a response to the need for information concerning Portugal (e.g. Eurostat or OECD inquiries) and follow European Union municipal solid waste management policies (e.g. packaging waste recovery and recycling targets and the reduction of biodegradable waste going to landfill).

  15. Prenatal diagnosis of Bartter syndrome: amniotic fluid aldosterone.

    PubMed

    Rachid, Myriam; Dreux, Sophie; Pean de Ponfilly, Gauthier; Vargas-Poussou, Rosa; Czerkiewicz, Isabelle; Chevenne, Didier; Oury, Jean-François; Deschênes, Georges; Muller, Françoise

    2017-04-01

    Bartter syndrome is a severe inherited tubulopathy characterized at birth by salt wasting, severe polyuria, dehydration, growth retardation and secondary hyperaldosteronism. Prenatally, the disease is usually discovered following onset of severe polyhydramnios. We studied amniotic fluid aldosterone concentration in cases of Bartter syndrome and in control groups. Amniotic fluid aldosterone was assayed by radioimmunoassay. We undertook a retrospective case-control study based on 36 cases of postnatally diagnosed Bartter syndrome and 144 controls matched for gestational age. Two controls groups were defined: controls with polyhydramnios (n=72) and control without polyhydramnios (n=72). Amniotic fluid aldosterone was compared between the three groups. The median amniotic fluid aldosterone concentration in the Bartter syndrome group (90 pg/mL) did not differ significantly from that in the controls with polyhydramnios (90 pg/mL, p=0.33) or the controls without polyhydramnios (87 pg/mL, p=0.41). In conclusion, amniotic fluid aldosterone assay cannot be used for prenatal diagnosis of Bartter syndrome.

  16. Characterizing the environmental impact of metals in construction and demolition waste.

    PubMed

    Yu, Danfeng; Duan, Huabo; Song, Qingbin; Li, Xiaoyue; Zhang, Hao; Zhang, Hui; Liu, Yicheng; Shen, Weijun; Wang, Jinben

    2018-05-01

    Large quantities of construction and demolition (C&D) waste are generated in China every year, but their potential environmental impacts on the surrounding areas are rarely assessed. This study focuses on metals contained in C&D waste, characterizing the metal concentrations and their related environmental risks. C&D waste samples were collected in Shenzhen City, China, from building demolition sites, renovation areas undergoing refurbishment, landfill sites, and recycling companies (all located in Shenzhen city) that produce recycled aggregate, in order to identify pollution levels of the metals As, Cd, Cr, Cu, Pb, Ni, and Zn. The results showed that (1) the metal concentrations in most demolition and renovation waste samples were below the soil environmental quality standard for agricultural purposes (SQ-Agr.) in China; (2) Cd, Cu, and Zn led to relatively higher environmental risks than other metals, especially for Zn (DM5 tile sample, 360 mg/kg; R4 tile sample, 281 mg/kg); (3) non-inert C&D waste such as wall insulation and foamed plastic had high concentrations of As and Cd, so that these materials required special attention for sound waste management; and (4) C&D waste collected from landfill sites had higher concentrations of Cd and Cu than did waste collected from demolition and refurbishment sites.

  17. Characterization of municipal solid waste from the main landfills of Havana city

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Espinosa Llorens, Ma. del C; Lopez Torres, Matilde; Alvarez, Haydee

    The city of Havana, the political, administrative and cultural centre of Cuba, is also the centre of many of the economic activities of the nation: industries, services, scientific research and tourism. All of these activities contribute to the generation of municipal solid waste (MSW), which also impact other Cuban cities. Inadequate handling of waste and the lack of appropriate and efficient solutions for its final disposal and treatment increase the risk and possibility of contamination. The main difficulty in the development of a system of management of MSW lies in the lack of knowledge of the chemical composition of themore » waste that is generated in the country as a whole, and especially in Havana, where solid waste management decisions are made. The present study characterizes MSW in Havana city during 2004. The Calle 100, Guanabacoa and Ocho Vias landfills were selected for physical-chemical characterization of MSW, as they are the three biggest landfills in the city. A total of 16 indicators were measured, and weather conditions were recorded. As a result, the necessary information regarding the physical-chemical composition of the MSW became available for the first time in Cuba. The information is essential for making decisions regarding the management of waste and constitutes a valuable contribution to the Study on Integrated Management Plan of MSW in Havana.« less

  18. Characterization of municipal solid waste from the main landfills of Havana city.

    PubMed

    Espinosa Lloréns, Ma Del C; Torres, Matilde López; Alvarez, Haydee; Arrechea, Alexis Pellón; García, Jorge Alejandro; Aguirre, Susana Díaz; Fernández, Alejandro

    2008-01-01

    The city of Havana, the political, administrative and cultural centre of Cuba, is also the centre of many of the economic activities of the nation: industries, services, scientific research and tourism. All of these activities contribute to the generation of municipal solid waste (MSW), which also impact other Cuban cities. Inadequate handling of waste and the lack of appropriate and efficient solutions for its final disposal and treatment increase the risk and possibility of contamination. The main difficulty in the development of a system of management of MSW lies in the lack of knowledge of the chemical composition of the waste that is generated in the country as a whole, and especially in Havana, where solid waste management decisions are made. The present study characterizes MSW in Havana city during 2004. The Calle 100, Guanabacoa and Ocho Vías landfills were selected for physical-chemical characterization of MSW, as they are the three biggest landfills in the city. A total of 16 indicators were measured, and weather conditions were recorded. As a result, the necessary information regarding the physical-chemical composition of the MSW became available for the first time in Cuba. The information is essential for making decisions regarding the management of waste and constitutes a valuable contribution to the Study on Integrated Management Plan of MSW in Havana.

  19. RH-TRU Waste Characterization by Acceptable Knowledge at the Idaho National Engineering and Environmental Laboratory

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Schulz, C.; Givens, C.; Bhatt, R.

    2003-02-24

    Idaho National Engineering and Environmental Laboratory (INEEL) is conducting an effort to characterize approximately 620 drums of remote-handled (RH-) transuranic (TRU) waste currently in its inventory that were generated at the Argonne National Laboratory-East (ANL-E) Alpha Gamma Hot Cell Facility (AGHCF) between 1971 and 1995. The waste was generated at the AGHCF during the destructive examination of irradiated and unirradiated fuel pins, targets, and other materials from reactor programs at ANL-West (ANL-W) and other Department of Energy (DOE) reactors. In support of this effort, Shaw Environmental and Infrastructure (formerly IT Corporation) developed an acceptable knowledge (AK) collection and management programmore » based on existing contact-handled (CH)-TRU waste program requirements and proposed RH-TRU waste program requirements in effect in July 2001. Consistent with Attachments B-B6 of the Waste Isolation Pilot Plant (WIPP) Hazardous Waste Facility Permit (HWFP) and th e proposed Class 3 permit modification (Attachment R [RH-WAP] of this permit), the draft AK Summary Report prepared under the AK procedure describes the waste generating process and includes determinations in the following areas based on AK: physical form (currently identified at the Waste Matrix Code level); waste stream delineation; applicability of hazardous waste numbers for hazardous waste constituents; and prohibited items. In addition, the procedure requires and the draft summary report contains information supporting determinations in the areas of defense relationship and radiological characterization.« less

  20. Marshall Space Flight Center solid waste characterization and recycling improvement study

    NASA Technical Reports Server (NTRS)

    Eley, Michael H.; Crews, Lavonne; Johnston, Ben; Lee, David; Colebaugh, James

    1995-01-01

    The MSFC Facilities Office, which is responsible for disposing of all waste generated by MSFC, issued a delivery order to the University of Alabama in Huntsville (UAH) to characterize current MSFC waste streams and to evaluate their existing recycling program. The purpose of the study was to define the nature, quantity, and types of waste produced and to generate ideas for improving the present recycling program. Specifically, the following tasks were to be performed: Identify various surplus and waste materials--as identified by the Contracting Officer's Technical Representative (COTR)--by source, location, and type; Analyze MSFC's current methods for handling, storage, transport, and disposition of waste and surplussed materials; Determine the composition of various surplus and waste materials as to type and quantities from various sources and locations; Analyze different methods for the disposition of various surplus and waste materials, including quality, quantity, preparation, transport cost, and value; Study possible alternatives to current methods of handling, storage, transport, and disposition of surplus and waste materials to improve the quality and quantities recycled or sold and to reduce and minimize the quantities of surplus and waste material currently being disposed of or stored; Provide recommendations for source and centralized segregation and aggregation of materials for recycling and/or disposition; and The analysis could include identification and laboratory level evaluation of methods and/or equipment, including capital costs, operating costs, maintenance requirements, life cycle and return on investment for systems to support the waste reduction program mission.

  1. Associations of Protein-Energy Wasting Syndrome Criteria With Body Composition and Mortality in the General and Moderate Chronic Kidney Disease Populations in the United States.

    PubMed

    Beddhu, Srinivasan; Chen, Xiaorui; Wei, Guo; Raj, Dominic; Raphael, Kalani L; Boucher, Robert; Chonchol, Michel B; Murtaugh, Maureen A; Greene, Tom

    2017-05-01

    It is unknown whether the criteria used to define Protein-energy wasting (PEW) syndrome in dialysis patients reflect protein or energy wasting in the general and moderate CKD populations. In 11,834 participants in the 1999-2004 National Health and Nutrition Examination Survey, individual PEW syndrome criteria and the number of PEW syndrome categories were related to lean body and fat masses (measured by dual-energy absorptiometry) using linear regression in the entire cohort and CKD sub-population. Serum chemistry, body mass and muscle mass PEW criteria tended to be associated with lower lean body and fat masses, but the low dietary protein and energy intake criteria were associated with significantly higher protein and energy stores. When the number of PEW syndrome categories was defined by non-dietary categories alone, there was a monotonic inverse relationship with lean body and fat masses and strong positive relationship with mortality. In contrast, when dietary category alone was present, mean BMI was in the obesity range; additional presence of two non-dietary categories was associated with lower BMI and lower lean body and fat masses. Thus, the association of dietary category plus two additional non-dietary categories with lower protein or energy stores was driven by the presence of the two non-dietary categories. Results were similar in CKD subgroup. Hence, a definition of PEW syndrome without dietary variables has face validity and reflects protein or energy wasting.

  2. Microbial Characterization of Solid-Wastes Treated with Heat Melt Compaction Technology

    NASA Technical Reports Server (NTRS)

    Strayer, Richard F.; Hummerick, Mary E.; Richards, Jeffrey T.; McCoy, LaShelle E.; Roberts, Michael S.; Wheeler, Raymond M.

    2011-01-01

    The research purpose of the project was to determine the fate of microorganisms in space-generated solid wastes after processing by a Heat Melt Compactor (HMC), which is a candidate solid waste treatment technology. Five HMC product disks were generated at Ames Research Center (ARC), Waste Management Systems element. The feed for two was simulated space-generated trash and feed for three was Volume F compartment wet waste returned on STS 130. Conventional microbiological methods were used to detect and enumerate microorganisms in HMC disks and in surface swab samples of HMC hardware before and after operation. Also, biological indicator test strips were added to the STS trash prior to compaction to test if HMC processing conditions, 150 C for approx 3 hr and dehydration, were sufficient to eliminate the test bacteria on the strips. During sample acquisition at KSC, the HMC disk surfaces were sanitized with 70% alcohol to prevent contamination of disk interiors. Results from microbiological assays indicated that numbers of microbes were greatly reduced but not eliminated by the 70% alcohol. Ten 1.25 cm diameter cores were aseptically cut from each disk to sample the disk interior. The core material was run through the microbial characterization analyses after dispersal in sterile diluent. Low counts of viable bacteria (5 to 50 per core) were found but total direct counts were 6 to 8 orders of magnitude greater. These results indicate that the HMC operating conditions might not be sufficient for complete waste sterilization, but the vast majority of microbes present in the wastes were dead or non-cultivable after HMC treatment. The results obtained from analyses of the commercial spore test strips that had been added fo the wastes prior to HMC operation further indicated that the HMC was sterilizing the wastes. Nearly all strips were recovered from the HMC disks and all of these were negative for spore growth when run through the manufacturer's protocol. The 10(exp 6

  3. Waste-water characterization/hazardous-waste survey, Beale Air Force Base, California. Final report, 12-26 September 1988

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Attebery, C.W.; Zimmer, A.T.; Hedgecock, N.S.

    1989-01-01

    A waste-water characterization hazardous-waste survey was conducted at Beale AFB by USAFOEHL/ECQ personnel to provide the base with sufficient information to address a State of California Notice of Violation concerning excessive discharges of boron and cyanide from the base sewage-treatment plant (STP). The results of the survey showed that the 9th RTS Precision Photo Lab along with other film-processing organizations were major contributors to the boron and cyanide discharge problems being experienced by the base STP. Maintenance organizations that utilize soaps and detergents that contain boron and cyanide also contributed to the problem.

  4. Importance of geologic characterization of potential low-level radioactive waste disposal sites

    USGS Publications Warehouse

    Weibel, C.P.; Berg, R.C.

    1991-01-01

    Using the example of the Geff Alternative Site in Wayne County, Illinois, for the disposal of low-level radioactive waste, this paper demonstrates, from a policy and public opinion perspective, the importance of accurately determining site stratigraphy. Complete and accurate characterization of geologic materials and determination of site stratigraphy at potential low-level waste disposal sites provides the frame-work for subsequent hydrologic and geochemical investigations. Proper geologic characterization is critical to determine the long-term site stability and the extent of interactions of groundwater between the site and its surroundings. Failure to adequately characterize site stratigraphy can lead to the incorrect evaluation of the geology of a site, which in turn may result in a lack of public confidence. A potential problem of lack of public confidence was alleviated as a result of the resolution and proper definition of the Geff Alternative Site stratigraphy. The integrity of the investigation was not questioned and public perception was not compromised. ?? 1991 Springer-Verlag New York Inc.

  5. Roles of Historical Photography in Waste Site Characterization, Closure, and Remediation

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Mackey, H.

    1998-07-01

    Over 40,000 frames of vertical historical photography from 1938 to 1996 and over 10,000 frames of oblique photography from 1981 to 1991 of the 777-square kilometer Savannah River Site in south central South Carolina were reviewed, cataloged, and referenced utilizing ARCView and associated ArcInfo tools. This allows environmental reviews of over 400 potential waste units on the SRS to be conducted in a rapid fashion to support preparation of work plans, characterization, risk assessments, and closure of the waste units in a more cost effective manner.

  6. Prenatal diagnosis of Bartter syndrome: amniotic fluid aldosterone.

    PubMed

    Rachid, Myriam L; Dreux, Sophie; Pean de Ponfilly, Gauthier; Vargas-Poussou, Rosa; Czerkiewicz, Isabelle; Chevenne, Didier; Oury, Jean-François; Deschênes, Georges; Muller, Françoise

    2016-01-01

    Bartter syndrome is a severe inherited tubulopathy characterized by postnatal salt wasting, severe polyuria, dehydration, failure to thrive and secondary hyperaldosteronism. Prenatally, the disease is usually discovered following the onset of severe polyhydramnios in the second trimester. We studied amniotic fluid aldosterone concentration in Bartter syndrome and in controls. Amniotic fluid aldosterone was assayed by radioimmunoassay. We undertook a retrospective case-control study based on 36 cases of prenatally suspected and postnatally confirmed Bartter syndrome (22 with identified mutations): and 72 gestational age matched controls presenting with polyhydramnios and 72 without polyhydramnios. Amniotic fluid aldosterone was compared between the three groups. The median amniotic fluid aldosterone concentration in the Bartter syndrome group (90 pg/mL) was not different from that in the controls with polyhydramnios (90 pg/mL, P = 0.33) or without polyhydramnios (87 pg/mL, P = 0.41). Amniotic fluid aldosterone assay cannot be used for prenatal diagnosis of Bartter syndrome. © 2015 John Wiley & Sons, Ltd. © 2015 John Wiley & Sons, Ltd.

  7. Force Provider Solid Waste Characterization Study

    DTIC Science & Technology

    2004-08-01

    energy converter (WEC) and/or composter . For a five-day period in June 2000, the solid waste generated by soldiers at the Force Provider Training Module...MATERIALS REDUCTION WASTE DISPOSAL MILITARY FACILITIES SANITARY ENGINEERING DISPOSAL FORCE PROVIDER FIELD FEEDING COMPOSTS WASTES GARBAGE WASTE RECYCLING...waste reduction through onsite waste-to-energy conversion and/or composting . The work was performed by Hughes Associates, Inc., 3610 Commerce

  8. Production and characterization of activated carbon from wood wastes

    NASA Astrophysics Data System (ADS)

    Ramirez, A. P.; Giraldo, S.; Ulloa, M.; Flórez, E.; Y Acelas, N.

    2017-12-01

    Cedarwood (Cedrela Angustifolia) and teak (Tectona Grandis) woods are typically used for furniture manufacture because they have high durability, are light and easy to work. During these manufacturing process, large amount of these wastes is generated causing disposal environmental problems. In this paper, the residual wastes (sawdust) of Cedar (C) and Teak (T) are transformed into an activated material. The chemical composition of both biomass (C and T) was determinate by TGA (Thermogravimetric Analysis). Activated materials were characterized in surface area following the BET (Brunauer, Emmett and Teller) method, morphology using SEM (Scanning Electron Microscopy) and to know their functional groups a FTIR (Fourier Transform Infrared Spectroscopy) analysis was done. Their adsorption capacity was evaluated by removal of Methylene Blue (MB) and Congo Red (CR) from aqueous solutions.

  9. Viral wasting syndrome of swine: experimental reproduction of postweaning multisystemic wasting syndrome in gnotobiotic swine by coinfection with porcine circovirus 2 and porcine parvovirus.

    PubMed

    Krakowka, S; Ellis, J A; Meehan, B; Kennedy, S; McNeilly, F; Allan, G

    2000-05-01

    One-day-old gnotobiotic piglets were inoculated intranasally with in vitro passaged porcine circovirus 1 (PCV-1), PCV-2, and porcine parvovirus (PPV) alone or in combination (PCV-1/PCV-2, PCV-1/PPV, and PCV-2/PPV). Piglets were evaluated for 1) the development of porcine postweaning multisystemic wasting syndrome (PMWS), 2) distribution of viral antigens by immunochemistry, and 3) viremia and the presence of viral DNA in nasal and ocular secretions and feces. All single agent-infected piglets and piglets infected with PCV-1/PCV-2 or PCV-1/PPV were clinically asymptomatic. They were transiently viremic and seroconverted to homologous virus(es). At termination of the study on postinfection day (PID) 35, microscopic lesions were restricted to focal inflammatory cell infiltrates in livers and myocardia. One piglet given PCV-1/PPV was PPV viremic for 2 weeks after infection and had lymphangiectasia of the spiral and descending colon associated with granulomatous inflammation. All four PCV-2/PPV-inoculated piglets developed PMWS, characterized by sudden onset of depression and anorexia, icterus, and submucosal edema. One piglet became moribund on PID 27, and the remaining three piglets were euthanatized between PID 27 and PID 30 because of severe disease. Lymph nodes were small and the livers were mottled. Disseminated angiocentric granulomatous inflammation was present in all tissues examined except the brain. Multiple lightly basophilic intracytoplasmic inclusion bodies were identified in macrophages and histiocytes. PCV-2 antigen was widely distributed within macrophages; PPV antigen was sparse. Hepatocellular necrosis and bile retention were prominent. PCV-2 DNA was identified in ocular, fecal, and nasal secretions. Terminal sera contained antibodies to PPV (4/4) and PCV-2 (3/ 4). Production of PMWS in gnotobiotic swine appears to require PCV-2 and additional infectious agents such as PPV for full disease expression in gnotobiotic piglets.

  10. Characterization of household waste in Greenland

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Eisted, Rasmus, E-mail: raei@env.dtu.dk; Christensen, Thomas H.

    2011-07-15

    The composition of household waste in Greenland was investigated for the first time. About 2 tonnes of household waste was sampled as every 7th bag collected during 1 week along the scheduled collection routes in Sisimiut, the second largest town in Greenland with about 5400 inhabitants. The collection bags were sorted manually into 10 material fractions. The household waste composition consisted primarily of biowaste (43%) and the combustible fraction (30%), including anything combustible that did not belong to other clean fractions as paper, cardboard and plastic. Paper (8%) (dominated by magazine type paper) and glass (7%) were other important materialmore » fractions of the household waste. The remaining approximately 10% constituted of steel (1.5%), aluminum (0.5%), plastic (2.4%), wood (1.0%), non-combustible waste (1.8%) and household hazardous waste (1.2%). The high content of biowaste and the low content of paper make Greenlandic waste much different from Danish household waste. The moisture content, calorific value and chemical composition (55 elements, of which 22 were below detection limits) were determined for each material fraction. These characteristics were similar to what has been found for material fractions in Danish household waste. The chemical composition and the calorific value of the plastic fraction revealed that this fraction was not clean but contained a lot of biowaste. The established waste composition is useful in assessing alternative waste management schemes for household waste in Greenland.« less

  11. Characterization of ecofriendly polyethylene fiber from plastic bag waste

    NASA Astrophysics Data System (ADS)

    Soekoco, Asril S.; Noerati, Komalasari, Maya; Kurniawan, Hananto, Agus

    2017-08-01

    This paper presents the characterization of fiber morphology, fiber count and tenacity of polyethylene fiber which is made from plastic bag waste. Recycling plastic bag waste into textile fiber has not developed yet. Plastic bag waste was recycled into fiber by melt spinning using laboratory scale melt spinning equipment with single orifice nozzle and plunger system. The basic principle of melt spinning is by melting materials and then extruding it through small orifice of a spinning nozzle to form fibers. Diameter and cross section shape of Recycled polyethylene fiber were obtained by using scanning electron microscope (SEM) instrumentation. Linear density of the recycled fiber were analyzed by calculation using denier and dTex formulation and The mechanical strength of the fibers was measured in accordance with the ASTM D 3379-75 standard. The cross section of recycled fiber is circular taking the shape of orifice. Fiber count of 303.75 denier has 1.84 g/denier tenacity and fiber count of 32.52 has 3.44 g/denier tenacity. This conditions is affected by the growth of polymer chain alignment when take-up axial velocity become faster. Recycled polyethylene fiber has a great potential application in non-apparel textile.

  12. Characterization of essential oil recovered from fennel horticultural wastes.

    PubMed

    Cautela, Domenico; Vella, Filomena Monica; Castaldo, Domenico; Laratta, Bruna

    2018-05-30

    Fennel crop has been traditionally used as spice in cooking and fragrances, and in folk medicine for its spectrum of useful properties. Mediterranean is the elective natural cultivation area for this plant with Italy being a leader producer. A limit of this production is due to the high amount of wastes derived still rich of phytochemicals, which are usually underused. Hence, the extraction and characterization of essential oil from residues of fennel horticultural market was investigated to understand the potential profit of their recycling. Forty-eight compounds resulted for fennel oil waste, analysed by GC-FID-MS, with the most abundant among components was anethole. Other constituents contributing to fennel flavour were the monoterpenes limonene and nerol. The exploitation of this oil as a good source of bioactive compounds was assessed by means of its antioxidant power measured with DPPH test.

  13. Bartter Syndrome with Normal Aldosterone Level: An Unusual Presentation.

    PubMed

    Huque, S S; Rahman, M H; Khatun, S

    2016-04-01

    Bartter syndrome (BS) is a hereditary disease, with an autosomal recessive or autosomal dominant mode of transmission. It is characterized by salt wasting hypochloraemic, hypokalaemic metabolic alkalosis and hyperreninaemia with normal blood pressure. The primary defect is in the thick ascending limb of loop of Henle (TAL). Herein, we report a case that had typical features of BS like severe dehydration, severe hypokalaemia, metabolic alkalosis and failure to thrive but had normal aldosterone level which is very uncommon.

  14. Characterization and energy potential of food waste from catering service in Hangzhou, China.

    PubMed

    Guo, Xiao-Hui; Sun, Fa-Qian; Sun, Ying-Jun; Lu, Hao-Hao; Wu, Wei-Xiang

    2014-08-01

    Safe disposal of food waste is becoming an impending issue in China with the rapid increase of its production and the promotion of environmental awareness. Food waste from catering services in Hangzhou, China, was surveyed and characterized in this study. A questionnaire survey involving 632 units across the urban districts showed that 83.5% of the food waste was not properly treated. Daily food waste production from catering units was estimated to be 1184.5 tonnes. The ratio of volatile solid to total solid, easily biodegradable matter (including crude fat, crude protein and total starch) content in total solid and the ratio of total organic carbon to nitrogen varied in ranges of 90.1%-93.9%, 60.9%-72.1%, and 11.9-19.9, respectively. Based on the methane yield of 350 mL g VS(-1) in anaerobic batch tests, annual biogas energy of 1.0 × 10(9) MJ was estimated to be recovered from the food waste. Food waste from catering services was suggested to be an attractive clean energy source by anaerobic digestion. © The Author(s) 2014.

  15. Generation and collection of restaurant waste: Characterization and evaluation at a case study in Italy.

    PubMed

    Tatàno, Fabio; Caramiello, Cristina; Paolini, Tonino; Tripolone, Luca

    2017-03-01

    Because restaurants (as a division of the hospitality sector) contribute to the generation of commercial and institutional waste, thus representing both a challenge and an opportunity, the objective of the present study was to deepen the knowledge of restaurant waste in terms of the qualitative and quantitative characteristics of waste generation and the performance achievable by the implementation of a separate collection scheme. In this study, the generated waste was characterized and the implemented separate collection was evaluated at a relevant case study restaurant in a coastal tourist area of Central Italy (Marche Region, Adriatic Sea side). The qualitative (compositional) characterization of the generated total restaurant waste showed considerable incidences of, in decreasing order, food (28.2%), glass (22.6%), paper/cardboard (19.1%), and plastic (17.1%). The quantitative (parametric) characterization of the generated restaurant waste determined the unit generation values of total waste and individual fractions based on the traditional employee and area parameters and the peculiar meal parameter. In particular, the obtained representative values per meal were: 0.72kgmeal -1 for total waste, and ranging, for individual fractions, from 0.20 (for food) to 0.008kgmeal -1 (for textile). Based on the critical evaluation of some of the resulting unit waste generation values, possible influences of restaurant practices, conditions, or characteristics were pointed out. In particular, food waste generation per meal can likely be limited by: promoting and using local, fresh, and quality food; standardizing and limiting daily menu items; basing food recipes on consolidated cooking knowledge and experience; and limiting plate sizes. The evaluation of the monthly variation of the monitored separate collection, ranging from an higher level of 52.7% to a lower level of 41.4%, indicated the following: a reduction in the separate collection level can be expected at times of

  16. Central Diabetes Insipidus and Cisplatin-Induced Renal Salt Wasting Syndrome: A Challenging Combination.

    PubMed

    Cortina, Gerard; Hansford, Jordan R; Duke, Trevor

    2016-05-01

    We describe a 2-year-old female with a suprasellar primitive neuroectodermal tumor and central diabetes insipidus (DI) who developed polyuria with natriuresis and subsequent hyponatremia 36 hr after cisplatin administration. The marked urinary losses of sodium in combination with a negative sodium balance led to the diagnosis of cisplatin-induced renal salt wasting syndrome (RSWS). The subsequent clinical management is very challenging. Four weeks later she was discharged from ICU without neurological sequela. The combination of cisplatin-induced RSWS with DI can be confusing and needs careful clinical assessment as inaccurate diagnosis and management can result in increased neurological injury. © 2016 Wiley Periodicals, Inc.

  17. Greater-than-Class C low-level radioactive waste characterization: Estimated volumes, radionuclide activities, and other characteristics

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hulse, R.A.

    1991-08-01

    Planning for storage or disposal of greater-than-Class C low-level radioactive waste (GTCC LLW) requires characterization of that waste to estimate volumes, radionuclide activities, and waste forms. Data from existing literature, disposal records, and original research were used to estimate the characteristics and project volumes and radionuclide activities to the year 2035. GTCC LLW is categorized as: nuclear utilities waste, sealed sources waste, DOE-held potential GTCC LLW; and, other generator waste. It has been determined that the largest volume of those wastes, approximately 57%, is generated by nuclear power plants. The Other Generator waste category contributes approximately 10% of the totalmore » GTCC LLW volume projected to the year 2035. Waste held by the Department of Energy, which is potential GTCC LLW, accounts for nearly 33% of all waste projected to the year 2035; however, no disposal determination has been made for that waste. Sealed sources are less than 0.2% of the total projected volume of GTCC LLW.« less

  18. Kindler syndrome.

    PubMed

    Lai-Cheong, Joey E; McGrath, John A

    2010-01-01

    Kindler syndrome (MIM173650) is an autosomal recessive genodermatosis characterized by poikiloderma, trauma-induced skin blistering, mucosal inflammation, and photosensitivity. Loss-of-function mutations in the FERMT1 gene are the cause of Kindler syndrome. Kindler syndrome is categorized as a subtype of epidermolysis bullosa (EB). During infancy and childhood, there is clinical overlap between Kindler syndrome and dystrophic EB. Unlike other forms of EB, Kindler syndrome is characterized by impaired actin cytoskeleton-extracellular matrix interactions and a variable plane of blister formation at or close to the dermal-epidermal junction. This article reviews clinicopathologic and molecular features of Kindler syndrome and discusses patient management.

  19. Scoliosis in Steinert syndrome: a case report.

    PubMed

    Themistocleous, George S; Sapkas, George S; Papagelopoulos, Panayiotis J; Stilianessi, Eugenia V; Papadopoulos, Elias Ch; Apostolou, Constantinos D

    2005-01-01

    Steinert syndrome is described as an autosomal dominant condition characterized by progressive muscular wasting, myotonia, musculoskeletal manifestations and rare spinal defects. Little is reported about spinal deformity associated with this syndrome. We present a patient with Steinert syndrome complicated by scoliosis. In the literature on muscular dystrophy, other than Duchenne, little mention is given to the problem of scoliosis in general and its treatment in particular. A case report of a patient with Steinert syndrome associated with thoracic scoliosis and hypokyphosis is presented. A 17-year-old boy presented with King type II right thoracic scoliosis (T5-T11, Cobb angle of 40 degrees) and hypokyphosis--10 degrees. He was treated with posterior stabilization and instrumentation at level T3-L2 with a postoperative correction of the scoliotic curve to 20 degrees. Histopathologic examination of the muscles confirmed the diagnosis of Steinert myotonic dystrophy. At 30-month follow-up, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of deformity correction. Scoliosis in Steinert syndrome shares the characteristic of an arthrogrypotic neuromuscular curve and demands the extensive soft tissue release for optimal surgical correction. Intraoperative observations included profound tissue bleeding, abnormally tough soft tissues and a difficult recovery from anaesthesia.

  20. Waste Generation Overview, Course 23263

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Simpson, Lewis Edward

    This course, Waste Generation Overview Live (COURSE 23263), provides an overview of federal and state waste management regulations, as well as Los Alamos National Laboratory (LANL) policies and procedures for waste management operations. The course covers the activities involved in the cradle-to-grave waste management process and focuses on waste characterization, waste compatibility determinations and classification, and the storage requirements for temporary waste accumulation areas at LANL. When you have completed this course, you will be able to recognize federal, state, and LANL environmental requirements and their impact on waste operations; recognize the importance of the cradle-to-grave waste management process; identifymore » the roles and responsibilities of key LANL waste management personnel (e.g., Waste Generator, Waste Management Coordinator, Waste Stream Profile approver, and Waste Certification Official); characterize a waste stream to determine whether it meets the definition of a hazardous waste, as well as characterize the use and minimum requirements for use of acceptable knowledge (AK) for waste characterization and waste compatibility documentation requirements; and identify the requirements for setting up and managing temporary waste accumulation areas.« less

  1. Underwater characterization of control rods for waste disposal using SMOPY

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Gallozzi-Ulmann, A.; Couturier, P.; Amgarou, K.

    Storage of spent fuel assemblies in cooling ponds requires careful control of the geometry and proximity of adjacent assemblies. Measurement of the fuel burnup makes it possible to optimise the storage arrangement of assemblies taking into account the effect of the burnup on the criticality safety margins ('burnup credit'). Canberra has developed a measurement system for underwater measurement of spent fuel assemblies. This system, known as 'SMOPY', performs burnup measurements based on gamma spectroscopy (collimated CZT detector) and neutron counting (fission chamber). The SMOPY system offers a robust and waterproof detection system as well as the needed capability of performingmore » radiometric measurements in the harsh high dose - rate environments of the cooling ponds. The gamma spectroscopy functionality allows powerful characterization measurements to be performed, in addition to burnup measurement. Canberra has recently performed waste characterisation measurements at a Nuclear Power Plant. Waste activity assessment is important to control costs and risks of shipment and storage, to ensure that the activity level remains in the range allowed by the facility, and to declare activity data to authorities. This paper describes the methodology used for the SMOPY measurements and some preliminary results of a radiological characterisation of AIC control rods. After describing the features and normal operation of the SMOPY system, we describe the approach used for establishing an optimum control rod geometric scanning approach (optimum count time and speed) and the method of the gamma spectrometry measurements as well as neutron check measurements used to verify the absence of neutron sources in the waste. We discuss the results obtained including {sup 60}Co, {sup 110m}Ag and {sup 108m}Ag activity profiles (along the length of the control rods) and neutron results including Total Measurement Uncertainty evaluations. Full self-consistency checks were performed

  2. Pyrolytic Waste Plastic Oil and Its Diesel Blend: Fuel Characterization.

    PubMed

    Khan, M Z H; Sultana, M; Al-Mamun, M R; Hasan, M R

    2016-01-01

    The authors introduced waste plastic pyrolysis oil (WPPO) as an alternative fuel characterized in detail and compared with conventional diesel. High density polyethylene, HDPE, was pyrolyzed in a self-designed stainless steel laboratory reactor to produce useful fuel products. HDPE waste was completely pyrolyzed at 330-490°C for 2-3 hours to obtain solid residue, liquid fuel oil, and flammable gaseous hydrocarbon products. Comparison of the fuel properties to the petrodiesel fuel standards ASTM D 975 and EN 590 revealed that the synthetic product was within all specifications. Notably, the fuel properties included a kinematic viscosity (40°C) of 1.98 cSt, density of 0.75 gm/cc, sulphur content of 0.25 (wt%), and carbon residue of 0.5 (wt%), and high calorific value represented significant enhancements over those of conventional petroleum diesel fuel.

  3. Report of Chinese family with severe dermatitis, multiple allergies and metabolic wasting syndrome caused by novel homozygous desmoglein-1 gene mutation.

    PubMed

    Cheng, Ruhong; Yan, Ming; Ni, Cheng; Zhang, Jia; Li, Ming; Yao, Zhirong

    2016-10-01

    Recently, homozygous mutations in the desmoglein-1 (DSG1) gene and heterozygous mutation in the desmoplakin (DSP) gene have been demonstrated to be associated with severe dermatitis, multiple allergies and metabolic wasting (SAM) syndrome (Mendelian Inheritance in Man no. 615508). We aim to identify the molecular basis for a Chinese pedigree of SAM syndrome. A Chinese pedigree of SAM syndrome was subjected to mutation detection in the DSG1 gene. Sequence analysis of the DSG1 gene and quantitative reverse transcriptase polymerase chain reaction analysis for gene expression of DSG1 using cDNA derived from the epidermis of patients and controls were both performed. Skin biopsies were also taken from patients for pathological study and transmission electron microscopy observation. Novel homozygous splicing mutation c.1892-1delG in the exon-intron border of the DSG1 gene has been demonstrated to be associated with SAM syndrome. We report a new family of SAM syndrome of Asian decent and expand the spectrum of mutations in the DSG1 gene. © 2016 Japanese Dermatological Association.

  4. Primary Molecular Disorders and Secondary Biological Adaptations in Bartter Syndrome

    PubMed Central

    Deschênes, Georges; Fila, Marc

    2011-01-01

    Bartter syndrome is a hereditary disorder that has been characterized by the association of hypokalemia, alkalosis, and the hypertrophy of the juxtaglomerular complex with secondary hyperaldosteronism and normal blood pressure. By contrast, the genetic causes of Bartter syndrome primarily affect molecular structures directly involved in the sodium reabsorption at the level of the Henle loop. The ensuing urinary sodium wasting and chronic sodium depletion are responsible for the contraction of the extracellular volume, the activation of the renin-aldosterone axis, the secretion of prostaglandins, and the biological adaptations of downstream tubular segments, meaning the distal convoluted tubule and the collecting duct. These secondary biological adaptations lead to hypokalemia and alkalosis, illustrating a close integration of the solutes regulation in the tubular structures. PMID:21941653

  5. Listed waste determination report. Environmental characterization

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Not Available

    1993-06-01

    On September 23, 1988, the US Environmental Protection Agency (EPA) published a notice clarifying interim status requirements for the management of radioactive mixed waste thereby subjecting the Idaho National Engineering Laboratory (INEL) and other applicable Department of Energy (DOE) sites to regulation under the Resource Conservation and Recovery Act (RCRA). Therefore, the DOE was required to submit a Part A Permit application for each treatment, storage, and disposal (TSD) unit within the INEL, defining the waste codes and processes to be regulated under RCRA. The September 1990 revised Part A Permit application, that was approved by the State of Idahomore » identified 101 potential acute and toxic hazardous waste codes (F-, P-, and U- listed wastes according to 40 CFR 261.31 and 40 CFR 261.33) for some TSD units at the Idaho Chemical Processing Plant. Most of these waste were assumed to have been introduced into the High-level Liquid Waste TSD units via laboratory drains connected to the Process Equipment Waste (PEW) evaporator (PEW system). At that time, a detailed and systematic evaluation of hazardous chemical use and disposal practices had not been conducted to determine if F-, P-, or Unlisted waste had been disposed to the PEW system. The purpose of this investigation was to perform a systematic and detailed evaluation of the use and disposal of the 101 F-, P-, and Unlisted chemicals found in the approved September 1990 Part A Permit application. This investigation was aimed at determining which listed wastes, as defined in 40 CFR 261.31 (F-listed) and 261.33 (P & Unlisted) were discharged to the PEW system. Results of this investigation will be used to support revisions to the RCRA Part A Permit application.« less

  6. Isolation and characterization of potential lactic acid bacteria (LAB) from freshwater fish processing wastes for application in fermentative utilisation of fish processing waste

    PubMed Central

    R, Jini; HC, Swapna; Rai, Amit Kumar; R, Vrinda; PM, Halami; NM, Sachindra; N, Bhaskar

    2011-01-01

    Proteolytic and/or lipolytic lactic acid bacteria (LAB) were isolated from visceral wastes of different fresh water fishes. LAB count was found to be highest in case of visceral wastes of Mrigal (5.88 log cfu/g) and lowest in that of tilapia (4.22 log cfu/g). Morphological, biochemical and molecular characterization of the selected LAB isolates were carried out. Two isolates FJ1 (E. faecalis NCIM5367) and LP3 (P. acidilactici NCIM5368) showed both proteolytic and lipolytic properties. All the six native isolates selected for characterization showed antagonistic properties against several human pathogens. All the native isolates were sensitive to antibiotics cephalothin and clindamycin; and, resistant to cotrimoxazole and vancomycin. Considering individually, P. acidilactici FM37, P. acidilactici MW2 and E. faecalis FD3 were sensitive to erythromycin. The two strains FJ1 (E. faecalis NCIM 5367) and LP3 (P. acidilactici NCIM 5368) that had both proteolytic and lipolytic properties have the potential for application in fermentative recovery of lipids and proteins from fish processing wastes. PMID:24031786

  7. Mechanical characterization of municipal solid waste from two waste dumps at Delhi, India.

    PubMed

    Ramaiah, B J; Ramana, G V; Datta, Manoj

    2017-10-01

    The article presents the physical and mechanical properties of the emplaced municipal solid waste (MSW) recovered from different locations of the Ghazipur and Okhla dumps both located at Delhi, India. Mechanical compressibility and shear strength of the collected MSW were evaluated using a 300×300mm direct shear (DS) shear box. Compression ratio (C c ') of MSW at these two dumps varied between 0.11 and 0.17 and is falling on the lower bound of the range (0.1-0.5) of the data reported in the literature for MSW. Low C c ' of MSW is attributed to the relatively low percentages of compressible elements such as textiles, plastics and paper, coupled with relatively high percentages of inert materials such as soil-like and gravel sized fractions. Shear strength of MSW tested is observed to be displacement dependent. The mobilized shear strength parameters i.e., the apparent cohesion intercept (c') and friction angle (ϕ') of MSW at these two dumps are best characterized by c'=13kPa and ϕ'=23° at 25mm displacement and c'=17kPa and ϕ'=34° at 55mm displacement and are in the range reported for MSW in the literature. A large database on the shear strength of MSW from 18 countries that includes: the experimental data from 277 large-scale DS tests (in-situ and laboratory) and the data from back analysis of 11 failed landfill slopes is statistically analyzed. Based on the analysis, a simple linear shear strength envelope, characterized by c'=17kPa and ϕ'=32°, is proposed for MSW for preliminary use in the absence of site-specific data for stability evaluation of the solid waste landfill under drained conditions. Copyright © 2017 Elsevier Ltd. All rights reserved.

  8. Synthesis and characterization of carboxymethyl cellulose from office waste paper: A greener approach towards waste management

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Joshi, Gyanesh, E-mail: joshig@icfre.org; Naithani, Sanjay; Varshney, V.K.

    2015-04-15

    Highlights: • Carboxymethyl cellulose (CMC) was successfully prepared from waste paper. • CMC had maximum degree of substitution (DS) 1.07. • Rheological studies of CMC (DS, 1.07) showed non-Newtonian pseudoplastic behavior. • Characterization of CMC was done by FT-IR and NMR techniques. • Morphology of prepared CMC was studied by SEM. - Abstract: In the present study, functionalization of mixed office waste (MOW) paper has been carried out to synthesize carboxymethyl cellulose, a most widely used product for various applications. MOW was pulped and deinked prior to carboxymethylation. The deinked pulp yield was 80.62 ± 2.0% with 72.30 ± 1.50%more » deinkability factor. The deinked pulp was converted to CMC by alkalization followed by etherification using NaOH and ClCH{sub 2}COONa respectively, in an alcoholic medium. Maximum degree of substitution (DS) (1.07) of prepared CMC was achieved at 50 °C with 0.094 M and 0.108 M concentrations of NaOH and ClCH{sub 2}COONa respectively for 3 h reaction time. The rheological characteristics of 1–3% aqueous solution of optimized CMC product showed the non-Newtonian pseudoplastic behavior. Fourier transform infra red (FTIR), nuclear magnetic resonance (NMR) and scanning electron microscope (SEM) study were used to characterize the CMC product.« less

  9. Contribution of anorexia to tissue wasting in cachexia.

    PubMed

    Molfino, Alessio; Laviano, Alessandro; Rossi Fanelli, Filippo

    2010-12-01

    Anorexia is a severe debilitating symptom characterizing the clinical course of several chronic diseases. It negatively impacts on patient outcome by contributing to weight loss, lean body mass catabolism and adipose tissue wasting. Although disease-associated anorexia may stand alone as a clinically relevant symptom, it is now considered as a component of the cachexia syndrome. The present review discusses experimental and clinical data indicating that the pathogenic mechanisms of anorexia may also suggest a neural control of tissue wasting in cachexia. Consistent data show that selective melanocortin receptor antagonism modulates food intake and reduces wasting in experimental models of chronic disease. Consequently, ghrelin administration, whose prophagic effects are related to melanocortin antagonism, has been tested both in animal studies and human trials, with promising effects, although restoration of lean body mass has been not achieved. More interest is driven by the use of small molecules selectively antagonising hypothalamic melanocortin receptors. The 'brain-muscle axis' coordinated by the hypothalamus seems to mediate the onset of not only anorexia but also tissue wasting in cachexia, by centrally influencing energy homeostasis and the balance between anabolism and catabolism.

  10. Quantitative Characterization of Aqueous Byproducts from Hydrothermal Liquefaction of Municipal Wastes, Food Industry Wastes, and Biomass Grown on Waste

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Maddi, Balakrishna; Panisko, Ellen; Wietsma, Thomas

    Hydrothermal liquefaction (HTL) is a viable thermochemical process for converting wet solid wastes into biocrude which can be hydroprocessed to liquid transportation fuel blendstocks and specialty chemicals. The aqueous byproduct from HTL contains significant amounts (20 to 50%) of the feed carbon, which must be used to enhance economic sustainability of the process on an industrial scale. In this study, aqueous fractions produced from HTL of industrial and municipal waste were characterized using a wide variety of analytical approaches. Organic chemical compounds present in these aqueous fractions were identified using two-dimensional gas chromatography equipped with time-of-flight mass spectrometry. Identified compoundsmore » include organic acids, nitrogen compounds, alcohols, aldehydes, and ketones. Conventional gas chromatography and liquid chromatography methods were employed to quantify the identified compounds. Inorganic species, in the aqueous stream of hydrothermal liquefaction of these aqueous byproducts, also were quantified using ion chromatography and inductively coupled plasma optical emission spectroscopy. The concentrations of organic chemical compounds and inorganic species are reported, and the significance of these results is discussed in detail.« less

  11. Characterization of NLRP3 variants in Japanese cryopyrin-associated periodic syndrome patients.

    PubMed

    Ohnishi, Hidenori; Teramoto, Takahide; Iwata, Hiroaki; Kato, Zenichiro; Kimura, Takeshi; Kubota, Kazuo; Nishikomori, Ryuta; Kaneko, Hideo; Seishima, Mariko; Kondo, Naomi

    2012-04-01

    The etiology of cryopyrin-associated periodic syndrome (CAPS) is caused by germline gene mutations in NOD-like receptor family, pryin domain containing 3 (NLRP3)/cold-induced autoinflammatory syndrome 1 (CIAS1). CAPS includes diseases with various severities. The aim of this study was to characterize patients according to the disease severity of CAPS. Five Japanese patients with four kinds of gene variations in NLRP3 were found and diagnosed as CAPS or juvenile idiopathic arthritis. Two mutations in NLRP3, Y563N and E688K, found in CAPS patients exhibit significant positive activities in the nuclear factor-κB reporter gene assay. Increased serum interleukin (IL)-18 levels were only observed in severe cases of CAPS. In mild cases of CAPS, the serum IL-18 levels were not increased, although lipopolysaccharide- or hypothermia-enhanced IL-1β and IL-18 production levels by their peripheral blood mononuclear cells were detectable. This series of case reports suggests that a combination of in vitro assays could be a useful tool for the diagnosis and characterization of the disease severity of CAPS.

  12. Gamma-ray spectrometry method used for radioactive waste drums characterization for final disposal at National Repository for Low and Intermediate Radioactive Waste--Baita, Romania.

    PubMed

    Done, L; Tugulan, L C; Dragolici, F; Alexandru, C

    2014-05-01

    The Radioactive Waste Management Department from IFIN-HH, Bucharest, performs the conditioning of the institutional radioactive waste in concrete matrix, in 200 l drums with concrete shield, for final disposal at DNDR - Baita, Bihor county, in an old exhausted uranium mine. This paper presents a gamma-ray spectrometry method for the characterization of the radioactive waste drums' radionuclides content, for final disposal. In order to study the accuracy of the method, a similar concrete matrix with Portland cement in a 200 l drum was used. © 2013 The Authors. Published by Elsevier Ltd All rights reserved.

  13. Enclosure (from letter from EPA to DOE sent 3/26/04) - Technical and Regulatory Support Document: EPA's Remote Handled Waste Characterization Determination

    EPA Pesticide Factsheets

    Based on the requirements presented in 40 CFR 194.24(c )(2) to (4) and 194.22(a)(1) and using experience gained as part of the CH waste characterization program, EPA examined the DOE's RH Waste Characterization Proposal as presented in the WCPIP.

  14. Melt processed crystalline ceramic waste forms for advanced nuclear fuel cycles: CRP T21027 1813: Processing technologies for high level waste, formulation of matrices and characterization of waste forms, task 17208: Final report

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Amoroso, J. W.; Marra, J. C.

    2015-08-26

    A multi-phase ceramic waste form is being developed at the Savannah River National Laboratory (SRNL) for treatment of secondary waste streams generated by reprocessing commercial spent nuclear. The envisioned waste stream contains a mixture of transition, alkali, alkaline earth, and lanthanide metals. Ceramic waste forms are tailored (engineered) to incorporate waste components as part of their crystal structure based on knowledge from naturally found minerals containing radioactive and non-radioactive species similar to the radionuclides of concern in wastes from fuel reprocessing. The ability to tailor ceramics to mimic naturally occurring crystals substantiates the long term stability of such crystals (ceramics)more » over geologic timescales of interest for nuclear waste immobilization [1]. A durable multi-phase ceramic waste form tailored to incorporate all the waste components has the potential to broaden the available disposal options and thus minimize the storage and disposal costs associated with aqueous reprocessing. This report summarizes results from three years of work on the IAEA Coordinated Research Project on “Processing technologies for high level waste, formulation of matrices and characterization of waste forms” (T21027), and specific task “Melt Processed Crystalline Ceramic Waste Forms for Advanced Nuclear Fuel Cycles” (17208).« less

  15. Melt processed crystalline ceramic waste forms for advanced nuclear fuel cycles: CRP T21027 1813: Processing technologies for high level waste, formulation of matrices and characterization of waste forms, Task 17208: Final report

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Amoroso, J. W.; Marra, J. C.

    2015-08-26

    A multi-phase ceramic waste form is being developed at the Savannah River National Laboratory (SRNL) for treatment of secondary waste streams generated by reprocessing commercial spent nuclear. The envisioned waste stream contains a mixture of transition, alkali, alkaline earth, and lanthanide metals. Ceramic waste forms are tailored (engineered) to incorporate waste components as part of their crystal structure based on knowledge from naturally found minerals containing radioactive and non-radioactive species similar to the radionuclides of concern in wastes from fuel reprocessing. The ability to tailor ceramics to mimic naturally occurring crystals substantiates the long term stability of such crystals (ceramics)more » over geologic timescales of interest for nuclear waste immobilization [1]. A durable multi-phase ceramic waste form tailored to incorporate all the waste components has the potential to broaden the available disposal options and thus minimize the storage and disposal costs associated with aqueous reprocessing. This report summarizes results from three years of work on the IAEA Coordinated Research Project on “Processing technologies for high level waste, formulation of matrices and characterization of waste forms” (T21027), and specific task “Melt Processed Crystalline Ceramic Waste Forms for Advanced Nuclear Fuel Cycles” (17208).« less

  16. Characterization of industrial wastes as raw materials for Emulsified Modified Bitumen (EMB) formulation

    NASA Astrophysics Data System (ADS)

    Najib Razali, Mohd; Isa, Syarifah Nur Ezatie Mohd; Salehan, Noor Adilah Md; Musa, Musfafikri; Aziz, Mohd Aizudin Abd; Nour, Abdurahman Hamid; Yunus, Rosli Mohd

    2018-04-01

    This study was conducted to characterize industrial wastes for formulation of emulsified modified bitumen (EMB) in relation to their physical characteristic and elemental composition. This analysis will give information either raw materials from industrial wastes can be used for EMB formulation. Bitumen is produced from crude oil that is extracted from the ground which categorizes the crude oil as one of the non-renewable form of product. A vast environmental problem issues arises in Malaysia cause by the excessive manufacturing activity that lead to a miss-management of industrial waste has leads to the used of industrial waste in the EMB formulation. Industrial waste such as polystyrene, polyethylene and used automotive oil can be used as alternative to formulate bitumen. Then a suitable emulsifier needs to be added to produce the final product which is EMB. The emulsifier will yield a charge depends on its properties to bind the oily bitumen with water. Physical characteristic studies were performed by thermogravimetric Analysis (TGA), differential scanning calorimetry (DSC), flash point test, density rest and moisture content test. Fourier Transform Infrared Spectroscopy (FTIR) analysis was measured to determine the material’s molecular composition and structure.

  17. Environmental waste site characterization utilizing aerial photographs and satellite imagery: Three sites in New Mexico, USA

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Van Eeckhout, E.; Pope, P.; Becker, N.

    1996-04-01

    The proper handling and characterization of past hazardous waste sites is becoming more and more important as world population extends into areas previously deemed undesirable. Historical photographs, past records, current aerial satellite imagery can play an important role in characterizing these sites. These data provide clear insight into defining problem areas which can be surface samples for further detail. Three such areas are discussed in this paper: (1) nuclear wastes buried in trenches at Los Alamos National Laboratory, (2) surface dumping at one site at Los Alamos National Laboratory, and (3) the historical development of a municipal landfill near Lasmore » Cruces, New Mexico.« less

  18. Identification of Non-Pertechnetate Species In Hanford Tank Waste, Their Synthesis, Characterization, And Fundamental Chemistry

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Kenneth R. Ashely; Norman Schroeder; Jose A. Olivares

    2004-12-10

    This proposal had three major goals: (1) develop capillary electrophoresis mass spectrometry as a characterization technique, (2) separate a non-pertechnetate fraction from a waste sample and identify the non-pertechnetate species in it by CEMS, and (3) synthesize and characterize bulk quantities of the identified non-pertechnetate species and study their ligand substitution and redox chemistry.

  19. Preparation and Characterization of Graphite Waste/CeO2 Composites

    NASA Astrophysics Data System (ADS)

    Kusrini, E.; Utami, C. S.; Nasruddin; Prasetyanto, E. A.; Bawono, Aji A.

    2018-03-01

    In this research, the chemical modification of graphite waste with CeO2 was developed and characterized. Graphite waste was pretreated with mechanical to obtain the size 200 mesh (75 μm), and thermal methods at 110°C oven for 6 hours. Here, we demonstrate final properties of graphite before modification (GBM), activated graphite (GA) and graphite/CeO2 composite with variation of 0.5, 1 and 2 g of CeO2 (G0.5; G1; G2). The effect of CeO2 concentration was observed. The presence of cerium in modified graphite samples (G0.5; G1; G2) were analyzed using SEM-EDX. The results show that the best surface area was found in G2 is 26.82 m2/g. The presence of CeO2 onto graphite surface does not significantly increase the surface area of composites.

  20. Pyrolytic Waste Plastic Oil and Its Diesel Blend: Fuel Characterization

    PubMed Central

    Sultana, M.; Al-Mamun, M. R.; Hasan, M. R.

    2016-01-01

    The authors introduced waste plastic pyrolysis oil (WPPO) as an alternative fuel characterized in detail and compared with conventional diesel. High density polyethylene, HDPE, was pyrolyzed in a self-designed stainless steel laboratory reactor to produce useful fuel products. HDPE waste was completely pyrolyzed at 330–490°C for 2-3 hours to obtain solid residue, liquid fuel oil, and flammable gaseous hydrocarbon products. Comparison of the fuel properties to the petrodiesel fuel standards ASTM D 975 and EN 590 revealed that the synthetic product was within all specifications. Notably, the fuel properties included a kinematic viscosity (40°C) of 1.98 cSt, density of 0.75 gm/cc, sulphur content of 0.25 (wt%), and carbon residue of 0.5 (wt%), and high calorific value represented significant enhancements over those of conventional petroleum diesel fuel. PMID:27433168

  1. Characterization and Disposition of Legacy Low-Level Waste at the Y-12 National Security Complex - 12133

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Tharp, Tim; Donnelly, Jim

    2012-07-01

    The Y-12 National Security Complex (Y-12) is concluding a multi-year program to characterize and dispose of all legacy low-level waste (LLW). The inventory of legacy waste at Y-12 has been reduced from over 3500 containers in Fiscal Year (FY) 2000 to 6 containers at the end of FY2011. In addition, the site recently eliminated the inventory of other low-level waste that is greater than 365 days old (i.e., >365-Day LLW), to be in full compliance with DOE Order 435.1. A consistent technical characterization approach emerged for both of these populations of backlogged waste: (1) compile existing historical data and processmore » knowledge and conduct interviews with site personnel; (2) inspect the containers and any tags, labels, or other markings to confirm or glean additional data; (3) with appropriate monitoring, open the container, visually inspect and photograph the contents while obtaining preliminary radiological surveys; (4) obtain gross weight and field non-destructive assay (NDA) data as needed; (5) use the non-public Oak Ridge Reservation Haul Road to ship the container to a local offsite vendor for waste sorting and segregation; (6) sort, drain, sample, and remove prohibited items; and (7) compile final data and prepare for shipment to disposal. After disposing of this backlog, the focus has now turned to avoiding the recurrence of this situation by maintaining low inventories of low-level waste and shortening the duration between waste generation and disposal. An enhanced waste tracking system and monthly metric charts are used to monitor and report progress to contractor and federal site office management. During the past 2 years, the average age of LLW onsite at Y-12 has decreased from more than 180 days to less than 60 days. (authors)« less

  2. CHARACTERIZATION OF ORGANIC EMISSIONS FROM HAZARDOUS WASTE INCINERATION PROCESSES UNDER THE NEW EPA DRAFT RISK BURN GUIDANCE: MEASUREMENT ISSUES

    EPA Science Inventory

    The paper discusses measurement issues relating to the characterization of organic emissions from hazardous waste incineration processes under EPA's new risk burn guidance. The recently published draft quidance recommends that hazardous waste combustion facilities complete a mass...

  3. Waste characterization study for the Kemp's Ridley sea turtle. Technical memo

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Malone, R.F.; Guarisco, M.

    1988-02-01

    The Kemp's Ridley sea turtle, Lepidochelys kempi, is an endangered species. The National Marine Fisheries Service's Head Start program is part of an international operation to save the turtles from extinction. Under the Head Start program, eggs from the Ridley's only known wild nesting beach at Rancho Nuevo in Mexico are transported to Padre Island on the Texas coast to be hatched. The head start enables the turtles to develop a survival advantage. The principal objective was to develop baseline waste-characterization data required to design a waste-water treatment scheme for the Galveston Head Start facility. As a secondary objective, preliminarymore » testing of some filtration components was undertaken to determine which units were most appropriate for inclusion in a wastewater treatment scheme.« less

  4. Microbial characterization and hydrocarbon biodegradation potential of natural bilge waste microflora.

    PubMed

    Olivera, N L; Commendatore, M G; Delgado, O; Esteves, J L

    2003-09-01

    Shipping operations produce oily wastes that must be managed properly to avoid environmental pollution. The aim of this study was to characterize microorganisms occurring in ship bilge wastes placed in open lagoons and, particularly, to assess their potential to degrade polycyclic aromatic hydrocarbons (PAHs). A first-order kinetic was suitable for describing hydrocarbon biodegradation after 17 days of treatment. The calculated rate constants were 0.0668 and 0.0513 day(-1) with a corresponding half-life of 10.3 and 13.5 days for the aliphatic and aromatic hydrocarbon fractions, respectively. At day 17, PAH removal percentages were: acenaphtylene 100, fluorene 95.2, phenanthrene 93.6, anthracene 70.3, and pyrene 71.5. Methyl phenanthrene removals were lower than that of their parent compound (3-methyl phenanthrene 83.6, 2-methyl phenanthrene 80.8, 1-methyl phenanthrene 77.3, 9-methyl phenanthrene 75.1, and 2,7-dimethyl phenanthrene 76.6). Neither pure cultures nor the microbial community from these wastes showed extracellular biosurfactant production suggesting that the addition of an exogenously produced biosurfactant may be important in enhancing hydrocarbon bioavailability and biodegradation. DNA analysis of bilge waste samples revealed a ubiquitous distribution of the nahAc genotype in the dump pools. Although almost all of the isolates grew on naphthalene as sole carbon source, only some of them yielded nahAc amplification under the experimental conditions used. The variety of PAHs in bilge wastes could support bacteria with multiple degradation pathways and a diversity of catabolic genes divergent from the classical nah-like type.

  5. Purification and Biochemical Characterization of Glutathione S-Transferase from Down Syndrome and Normal Children Erythrocytes: A Comparative Study

    ERIC Educational Resources Information Center

    Hamed, Ragaa R.; Maharem, Tahany M.; Abdel-Meguid, Nagwa; Sabry, Gilane M.; Abdalla, Abdel-Monem; Guneidy, Rasha A.

    2011-01-01

    Down syndrome (DS) is the phenotypic manifestation of trisomy 21. Our study was concerned with the characterization and purification of glutathione S-transferase enzyme (GST) from normal and Down syndrome (DS) erythrocytes to illustrate the difference in the role of this enzyme in the cell. Glutathione S-transferase and glutathione (GSH) was…

  6. Characterization of the carbonaceous materials obtained from different agro-industrial wastes.

    PubMed

    Ensuncho-Muñoz, A E; Carriazo, J G

    2015-01-01

    This paper reports the preparation and characterization of carbonaceous materials obtained from three types of vegetable wastes provided by agricultural industries. Soft carbonization (280°C) and H3PO4-activation procedures were used to convert the agricultural wastes to carbon powders with high adsorbent capacities. This process is excellent for eliminating and exploiting the huge masses (many tons) of vegetable residues remaining after each harvest every year in several Colombian agro-industries. The powders were characterized by X-ray diffraction (XRD), IR spectroscopy, scanning electron microscopy (SEM), and N2-adsorption isotherms. XRD and IR verified the formation of carbons, and SEM showed small particles (20-500 µm) with characteristic morphology for each type of residue used and abundant cavities of different sizes. The N2-adsorption analyses showed that the carbons had high adsorption capacities with important surface area values and large pore volumes. The use of the activated carbonaceous materials as adsorbent of azo dyes (allura red and sunset yellow) from aqueous solutions was evaluated. The results showed a good adsorption capacity indicating the potentiality of these materials as pollutant adsorbents in food industry wastewaters. These results indicate that these powders can be used as potential adsorbents for different gaseous or liquid pollutants.

  7. Recurrent urinary tract infections in an infant with antenatal Bartter syndrome.

    PubMed

    Tasic, Velibor; Pota, Liljana; Gucev, Zoran

    2011-02-01

    antenatal variant of Bartter syndrome is characterized by a history of polyhydramnios, premature birth, metabolic alkalosis, hypokalemia, polyuria and renal salt wasting. In this report we present a premature female baby with antenatal Barter syndrome who had three episodes of urinary tract infection (UTI), without evidence for congenital anomaly of the kidneys or urinary tract. antenatal Bartter syndrome was diagnosed according to the standard criteria. Ultrasound scan and voiding cystourethrography were performed to exclude congenital anomaly of the kidneys and urinary tract. the baby presented with early hyperkalemia and acidosis. The typical biochemical features of the Bartter syndrome were observed in the second month. Despite appropriate treatment she had persistent hypercalciuria. The clinical course was complicated with recurrent episodes of febrile UTIs. Urinary tract system imaging did not demonstrate congenital anomalies. She finally died of severe dehydration, acidosis and renal failure. since no congenital anomaly of the kidneys or urinary tract was demonstrated in our patient, we believe that severe, persistent hypercalciuria is the most important risk factor for development of recurrent UTIs.

  8. Hazardous Waste Clean-Up Information (CLU-IN) On-line Characterization and Remediation Databases Fact Sheet

    EPA Pesticide Factsheets

    This fact sheet provides an overview of the 10 on-line characterization and remediation databases available on the Hazardous Waste Clean-Up Information (CLU-IN) website sponsored by the U.S. Environmental Protection Agency.

  9. Production and characterization of pyrolytic oils by pyrolysis of waste machinery oil.

    PubMed

    Sinağ, Ali; Gülbay, Selen; Uskan, Burçin; Uçar, Suat; Ozgürler, Sara Bilge

    2010-01-15

    The main objective of this work is to propose an alternative method for evaluation of the waste machinery oil which is an environmental problem in Turkey. For this purpose, pyrolysis of waste machinery oil was conducted in a tubular reactor. Effect of the experimental conditions (various temperatures, catalyst type) on the formation of pyrolytic oil, gas, and char was investigated. Nickel supported on silica and zeolite (HZSM-5) were used as catalysts. Properties of the pyrolytic oils were characterized by gas chromatograph equipped with a mass selective detector (GC-MS), gas chromatography with flame ionization detector (GC-FID for boiling point range distribution), nuclear magnetic resonance ((1)H NMR) spectroscopy, higher heating value measurement, and elemental analysis. The behavior of the metals in the waste machinery oil and the pyrolytic oil samples was also quantitatively detected by inductively coupled plasma (ICP) analysis. As, Cd and Cr contents of the all pyrolytic oils were found as <0.05 ppm, while Cu content of the pyrolytic oils varied between 0.3 ppm and 0.61 ppm. Only Vanadium contents of the pyrolytic oils obtained at 800 degrees C (0.342 ppm) and in the presence of HZSM5 (0.57 ppm) increased compared to that obtained by waste machinery oil (0.1 ppm). Lower metal contents of the pyrolytic oils reveal that pyrolysis of the waste machinery oils leads to the formation of environmental friendly pyrolytic oils with higher heating values.

  10. Mechanisms of androgen deficiency in human immunodeficiency virus-infected women with the wasting syndrome.

    PubMed

    Grinspoon, S; Corcoran, C; Stanley, T; Rabe, J; Wilkie, S

    2001-09-01

    Although prior studies suggest reduced androgen levels in women with acquired immune deficiency syndrome wasting, little is known regarding the regulation of adrenal and ovarian androgen secretion in such patients. We investigated ovarian and adrenal function in 13 human immunodeficiency virus-infected women with acquired immune deficiency syndrome wasting and 21 age- and body mass index-matched healthy control subjects studied in the early follicular phase. Subjects received hCG (5000 U, im) on d 1 and Cosyntropin (0.25 mg, i.v.) on d 3 after dexamethasone (1 mg, orally, at 2400 h) pretreatment on d 2. At baseline, human immunodeficiency virus-infected subjects demonstrated significantly reduced T [18 +/- 2 vs. 25 +/- 2 ng/dl (0.6 +/- 0.1 vs. 0.9 +/- 0.1 nmol/liter); P = 0.02], free T [1.5 +/- 0.1 vs. 2.4 +/- 0.2 pg/ml (5.3 +/- 0.5 vs. 8.3 +/- 0.6 pmol/liter); P = 0.001], androstenedione [119 +/- 6 vs. 162 +/- 14 ng/dl (4.16 +/- 0.20 vs. 5.66 +/- 0.48 nmol/liter); P = 0.02], and dehydroepiandrosterone sulfate [0.96 +/- 0.17 vs. 1.55 +/- 0.19 microg/ml (2.6 +/- 0.5 vs. 4.2 +/- 0.5 micromol/liter); P = 0.047] levels compared with the control subjects. T [8 +/- 2 vs. 6 +/- 2 ng/dl (0.3 +/- 0.1 vs. 0.2 +/- 0.1 nmol/liter); P = 0.48], free T [0.5 +/- 0.2 vs. 0.4 +/- 0.1 pg/ml (1.7 +/- 0.7 vs. 1.5 +/- 0.5 pmol/liter); P = 0.85], 17 hydroxyprogesterone [0.5 +/- 0.2 vs. 0.7 +/- 0.2 microg/liter (1.6 +/- 0.6 vs. 2.0 +/- 0.6 nmol/liter); P = 0.63], and androstenedione [-1 +/- 12 vs. 8 +/- 11 ng/dl (-0.03 +/- 0.42 vs. 0.28 +/- 0.39 nmol/liter), P = 0.61] responses to hCG were not different between the groups. Cortisol responses were increased and dehydroepiandrosterone sulfate responses were decreased in the human immunodeficiency virus-infected vs. control subjects after ACTH stimulation. The ratio of DHEA to cortisol was significantly decreased at 60 (71 +/- 11 vs. 107 +/- 10; P = 0.02) and 90 (63 +/- 8 vs. 102 +/- 9; P = 0.004) min post-ACTH in the human immunodeficiency

  11. Characterization of the March 2017 Tank 15 Waste Removal Slurry Sample (Combination of Slurry Samples HTF-15-17-28 and HTF-15-17-29)

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Reboul, S. H.; King, W. D.; Coleman, C. J.

    2017-05-09

    Two March 2017 Tank 15 slurry samples (HTF-15-17-28 and HTF-15-17-29) were collected during the second bulk waste removal campaign and submitted to SRNL for characterization. At SRNL, the two samples were combined and then characterized by a series of physical, elemental, radiological, and ionic analysis methods. Sludge settling as a function of time was also quantified. The characterization results reported in this document are consistent with expectations based upon waste type, process knowledge, comparisons between alternate analysis techniques, and comparisons with the characterization results obtained for the November 2016 Tank 15 slurry sample (the sample collected during the first bulkmore » waste removal campaign).« less

  12. Synthesis and Characterization of C-Cinnamal Calix [4] Resorsinarena from Cinnamon Oil Waste West Sumatra

    NASA Astrophysics Data System (ADS)

    Etika, S. B.; Nasra, E.; Rilaztika, I.

    2018-04-01

    Synthesis and characterization of compound C-Cinnamal Calix [4] Resorsinarena (CCCR) of cinnamon oil waste have been done. This study was aimed to synthesis and characterize C-Cinnamal Calix [4] Resorsinarena from cinnamaldehyde violated cinnamon oil waste. C-Cinnamal Calix [4] Resorsinarena was synthesized by electrophilic substitution reaction of cinnamaldehyde isolated by the acid and resorcinol at 77oC temperature for 2 hour. The data analysis spectrum UV-VIS and FT-IR showed that the compound isolated cinnamaldehyde same as pure cinnamaldehyde compound. The characterization of C-Cinnamal Calix [4] Resorsinarena in the form of reddish-colored solids with melting point 3580C by using UV-VIS showed the presence of double bond, FT-IR showed the absorption at the wave number 3323,94 cm-1 indicating the ‑OH group, the wave number 1610,94 cm-1 showed the vibration C=C, the strong region absorption of 1500,86 cm-1 indicating the presence of an aromatic ring, the at 1442,88 cm-1 wave number indicating the presence of CH3.

  13. Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome).

    PubMed

    Koul, Monika; Dwivedi, Rahul; Upadhyay, Vinod

    2014-01-01

    Ectrodactyly-ectodermal dysplasia- clefting syndrome (also k/a. split hand- split foot malformation /split hand-split foot ectodermal dysplasia- cleft syndrome/ectodermal dysplasia cleft lip/cleft palate syndrome) a rare form of ectodermal dysplasia, is an autosomal dominant disorder inherited as a genetic trait and characterized by a triad of (i) ectrodactyly, (ii) ectodermal dysplasia and, (iii) & facial clefts.

  14. Nodding syndrome (NS) and Onchocerca Volvulus (OV) in Northern Uganda.

    PubMed

    Lagoro, David Kitara; Arony, Denis Anywar

    2017-01-01

    Nodding Syndrome (NS) is a childhood neurological disorder characterized by atonic seizures, cognitive decline, school dropout, muscle weakness, thermal dysfunction, wasting and stunted growth. There are recent published information suggesting associations between Nodding Syndrome (NS) with cerebrospinal fluid (CSF) VGKC antibodies and serum leiomidin-1 antibody cross reacting with Onchocerca Volvulus ( OV ). These findings suggest a neuro-inflammatory cause of NS and they are important findings in the search for the cause of Nodding Syndrome. These observations perhaps provide further, the unique explanation for the association between Nodding Syndrome and Onchocerca Volvulus . Many clinical and epidemiological studies had shown a significant correlation between NS and infestation with a nematode, Onchocerca volvulus which causes a disease, Onchocerciasis , some of which when left untreated can develop visual defect ("River Blindness"). While these studies conducted in Northern Uganda and Southern Sudan indicate a statistically significant association with ( OV infection (using positive skin snips), we observe that ( OV is generally endemic in many parts of Sub Saharan Africa and Latin America and that to date, no NS cases have been recorded in those regions. This letter to the Editor is to provide additional information on the current view about the relationship between Nodding Syndrome and Onchocerca Volvulus as seen in Northern Uganda.

  15. Nodding syndrome (NS) and Onchocerca Volvulus (OV) in Northern Uganda

    PubMed Central

    Lagoro, David Kitara; Arony, Denis Anywar

    2017-01-01

    Nodding Syndrome (NS) is a childhood neurological disorder characterized by atonic seizures, cognitive decline, school dropout, muscle weakness, thermal dysfunction, wasting and stunted growth. There are recent published information suggesting associations between Nodding Syndrome (NS) with cerebrospinal fluid (CSF) VGKC antibodies and serum leiomidin-1 antibody cross reacting with Onchocerca Volvulus (OV). These findings suggest a neuro-inflammatory cause of NS and they are important findings in the search for the cause of Nodding Syndrome. These observations perhaps provide further, the unique explanation for the association between Nodding Syndrome and Onchocerca Volvulus. Many clinical and epidemiological studies had shown a significant correlation between NS and infestation with a nematode, Onchocerca volvulus which causes a disease, Onchocerciasis, some of which when left untreated can develop visual defect ("River Blindness"). While these studies conducted in Northern Uganda and Southern Sudan indicate a statistically significant association with (OV infection (using positive skin snips), we observe that (OV is generally endemic in many parts of Sub Saharan Africa and Latin America and that to date, no NS cases have been recorded in those regions. This letter to the Editor is to provide additional information on the current view about the relationship between Nodding Syndrome and Onchocerca Volvulus as seen in Northern Uganda. PMID:29138647

  16. Dual Paraneoplastic Syndromes: Small Cell Lung Carcinoma-related Oncogenic Osteomalacia, and Syndrome of Inappropriate Antidiuretic Hormone Secretion: Report of a Case and Review of the Literature

    PubMed Central

    Ng, Roland CK

    2011-01-01

    Acquired isolated renal phosphate wasting associated with a tumor, known as oncogenic osteomalacia or tumor-induced osteomalacia, is a rare paraneoplastic syndrome caused by overproduction of fibroblast growth factor 23. Oncogenic osteomalacia is usually associated with benign mesenchymal tumors. Syndrome of inappropriate antidiuretic hormone secretion (SIADH), on the other hand, is a common paraneoplastic syndrome caused by small cell carcinoma (SCC). Concomitant oncogenic osteomalacia and SIADH associated with SCC is very rare with only 4 other cases reported in the literature. The authors report a case of small cell lung cancer (SCLC)-related renal wasting hypophosphatemia and concurrent SIADH, and review the literature reporting 9 other cases of SCC associated with oncogenic osteomalacia. Almost half of reported cases of renal phosphate wasting associated with SCC concomitantly presented with SIADH. These cases had initial serum phosphorus level lower and survival periods shorter than those without SIADH. This rare combination of a dual paraneoplastic syndrome and low serum phosphorus may be a poor prognostic sign. In addition, both renal phosphate wasting and SIADH usually occur in a short period of time before identification of SCC. Therefore, renal wasting hypophosphatemia with concomitant SIADH/hyponatremia should prompt a search for SCC rather than a benign mesenchymal tumor. PMID:21886301

  17. Dual paraneoplastic syndromes: small cell lung carcinoma-related oncogenic osteomalacia, and syndrome of inappropriate antidiuretic hormone secretion: report of a case and review of the literature.

    PubMed

    Tantisattamo, Ekamol; Ng, Roland C K

    2011-07-01

    Acquired isolated renal phosphate wasting associated with a tumor, known as oncogenic osteomalacia or tumor-induced osteomalacia, is a rare paraneoplastic syndrome caused by overproduction of fibroblast growth factor 23. Oncogenic osteomalacia is usually associated with benign mesenchymal tumors. Syndrome of inappropriate antidiuretic hormone secretion (SIADH), on the other hand, is a common paraneoplastic syndrome caused by small cell carcinoma (SCC). Concomitant oncogenic osteomalacia and SIADH associated with SCC is very rare with only 4 other cases reported in the literature. The authors report a case of small cell lung cancer (SCLC)-related renal wasting hypophosphatemia and concurrent SIADH, and review the literature reporting 9 other cases of SCC associated with oncogenic osteomalacia. Almost half of reported cases of renal phosphate wasting associated with SCC concomitantly presented with SIADH. These cases had initial serum phosphorus level lower and survival periods shorter than those without SIADH. This rare combination of a dual paraneoplastic syndrome and low serum phosphorus may be a poor prognostic sign. In addition, both renal phosphate wasting and SIADH usually occur in a short period of time before identification of SCC. Therefore, renal wasting hypophosphatemia with concomitant SIADH/hyponatremia should prompt a search for SCC rather than a benign mesenchymal tumor.

  18. Regulatory controls on the hydrogeological characterization of a mixed waste disposal site, Radioactive Waste Management Complex, Idaho National Engineering Laboratory

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Ruebelmann, K.L.

    1990-01-01

    Following the detection of chlorinated volatile organic compounds in the groundwater beneath the SDA in the summer of 1987, hydrogeological characterization of the Radioactive Waste Management Complex (RWMC), Idaho National Engineering Laboratory (INEL) was required by the Resource Conservation and Recovery Act (RCRA). The waste site, the Subsurface Disposal Area (SDA), is the subject of a RCRA Corrective Action Program. Regulatory requirements for the Corrective Action Program dictate a phased approach to evaluation of the SDA. In the first phase of the program, the SDA is the subject of a RCRA Facility Investigation (RIF), which will obtain information to fullymore » characterize the physical properties of the site, determine the nature and extent of contamination, and identify pathways for migration of contaminants. If the need for corrective measures is identified during the RIF, a Corrective Measures Study (CMS) will be performed as second phase. Information generated during the RIF will be used to aid in the selection and implementation of appropriate corrective measures to correct the release. Following the CMS, the final phase is the implementation of the selected corrective measures. 4 refs., 1 fig.« less

  19. MUNICIPAL WASTE COMBUSTION ASSESSMENT: MEDICAL WASTE COMBUSTION PRACTICES AT MUNICIPAL WASTE COMBUSTION FACILITIES

    EPA Science Inventory

    The report defines and characterizes types of medical waste, discusses the impacts of burning medical waste on combustor emissions, and outlines important handling and operating considerations. Facility-specific design, handling, and operating practiced are also discussed for mun...

  20. "Syndrome in syndrome": Wernicke syndrome due to afferent loop syndrome. Case report and review of the literature.

    PubMed

    D'Abbicco, D; Praino, S; Amoruso, M; Notarnicola, A; Margari, A

    2011-01-01

    Wernicke syndrome is a rare neurological pathology due to a deficit in vitamin B1. The syndrome is common among alcohol abusers, patients with malignant tumor or gastrointestinal diseases, those who undergo hemodialysis or long-term peritoneal dialysis, pregnant women with hyperemesis, women who breast-feed, patients with hyperthyroidism or anorexia nervosa or gastric or jejunal-ileal bypass surgery for obesity, patients submitted to gastric surgery or prolonged total parenteral nutrition or prolonged intravenous therapy. We report a case of Wernicke syndrome due to afferent loop syndrome characterized by incoercible vomiting.

  1. Characterization of brominated flame retardants in construction and demolition waste components: HBCD and PBDEs.

    PubMed

    Duan, Huabo; Yu, Danfeng; Zuo, Jian; Yang, Bo; Zhang, Yukui; Niu, Yongning

    2016-12-01

    The vast majority of construction material is inert and can be managed as nonhazardous. However, structures may have either been built with some environmentally unfriendly substances such as brominated flame retardants (BFRs), or have absorbed harmful elements such as heavy metals. This study focuses on end-of-life construction materials, i.e. construction and demolition (C&D) waste components. The aim was to characterize the concentration of extremely harmful substances, primarily BFRs, including hexabromocyclododecane (HBCD) and polybrominateddiphenyl ethers (PBDEs). Results revealed extremely high contents of HBCD and PBDEs in typical C&D waste components, particularly polyurethane foam materials. Policies should therefore be developed for the proper management of C&D waste, with priority for POP-containing debris. The first priority is to develop a classification system and procedures to separate out the harmful materials for more extensive processing. Additionally, identification and quantification of the environmental implications associated with dumping-dominated disposal of these wastes are required. Finally, more sustainable materials should be selected for use in the construction industry. Copyright © 2016 Elsevier B.V. All rights reserved.

  2. Generation, characterization and reuse of solid wastes from a biodiesel production plant.

    PubMed

    Oliveira, Fernando Jorge Santos; Santana, Daniele Dos Santos; Costa, Simone Soraya Brito; Oliveira, Lenise Diniz; Liduino, Vitor Silva; Servulo, Eliana Flávia Camporese

    2017-03-01

    The aim of this study was to identify and characterize industrial solid wastes generated by a biodiesel production plant in Brazil, as well as to present strategies for the management of these materials. This plant produces every year around 100,000tons of biodiesel from vegetable oils and animal fats. The methodology of the study included technical visits, interviews with the operational and environmental management staff as well as analysis of documents, reports and computerized data systems. An approach to reduce the generation of hazardous waste was investigated. It was take into account the amount of raw material that was processed, reduction of landfill disposal, and the maximization of the their recycling and reuse. The study also identified the sources of waste generation and accordingly prepared an evaluation matrix to determine the types of waste with the higher potential for minimization. The most important residue of the process was the filter material impregnated with oil and biodiesel, requiring, therefore, measures for its minimization. The use of these residues in the production of ceramic artefacts (light bricks) was considered to be very promising, since no significant effect on the physico-chemical and mechanical properties of the artefacts produced was observed. Phytotoxicity test using seeds of Lactuva sativa (lettuce), Brassica juncea (mustard), Abelmoschus esculentus (okra), Chrysanthemum leucanthemum (daisy), Dendranthema grandiflorum (chrysanthemum) and Allium porrum (leek) were carried out. The results clearly show incorporation of the waste material into bricks did not influence relative germination and relative root elongation in comparison to control tests. Copyright © 2016 Elsevier Ltd. All rights reserved.

  3. Acceptable knowledge document for INEEL stored transuranic waste -- Rocky Flats Plant waste. Revision 2

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    NONE

    1998-01-23

    This document and supporting documentation provide a consistent, defensible, and auditable record of acceptable knowledge for waste generated at the Rocky Flats Plant which is currently in the accessible storage inventory at the Idaho National Engineering and Environmental Laboratory. The inventory consists of transuranic (TRU) waste generated from 1972 through 1989. Regulations authorize waste generators and treatment, storage, and disposal facilities to use acceptable knowledge in appropriate circumstances to make hazardous waste determinations. Acceptable knowledge includes information relating to plant history, process operations, and waste management, in addition to waste-specific data generated prior to the effective date of the RCRAmore » regulations. This document is organized to provide the reader a comprehensive presentation of the TRU waste inventory ranging from descriptions of the historical plant operations that generated and managed the waste to specific information about the composition of each waste group. Section 2 lists the requirements that dictate and direct TRU waste characterization and authorize the use of the acceptable knowledge approach. In addition to defining the TRU waste inventory, Section 3 summarizes the historical operations, waste management, characterization, and certification activities associated with the inventory. Sections 5.0 through 26.0 describe the waste groups in the inventory including waste generation, waste packaging, and waste characterization. This document includes an expanded discussion for each waste group of potential radionuclide contaminants, in addition to other physical properties and interferences that could potentially impact radioassay systems.« less

  4. TTP AL921102: An integrated geophysics program for non-intrusive characterization of mixed-waste landfill sites

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hasbrouck, J.C.

    1992-11-01

    Chem-Nuclear Geotech, Inc. (Geotech), operating contractor for the US Department of Energy Grand Junction Projects Office, is conducting the Integrated Geophysics Program for Non-Intrusive Characterization of Mixed-Waste Landfill Sites (Technical Task Plan [TTP] AL921102). The TTP is part of the Mixed-Waste Landfill Integrated Demonstration (MWLID). The objective of this task was to demonstrate that an integrated program of surface geophysics can be used to effectively and nonintrusively characterize n-mixed-waste landfill sites. To accomplish this objective, integrated field demonstrations were conducted over two previously identified areas of interest (designated Areas A and B) within the MWLID test site at the Chemicalmore » Waste Landfill (CWL), Technical Area 3, at the Sandia National Laboratories, Albuquerque, New Mexico (Figures 1 and 2). Area A was centered roughly around the Chromic Acid and Organics Pits in the southeast-central portion of the landfill and Area B was centered around the 60's Pits'' area in the northeast-central portion of the landfill. Pit locations were known in Area A and suspected in Area B. This progress report describes the geophysical surveys conducted by Geotech and presents preliminary displays and analyses. Volume 2 of this report contains the raw data for all the surveys conducted by Geotech for this TTP.« less

  5. Physical and chemical characterization of waste wood derived biochars.

    PubMed

    Yargicoglu, Erin N; Sadasivam, Bala Yamini; Reddy, Krishna R; Spokas, Kurt

    2015-02-01

    Biochar, a solid byproduct generated during waste biomass pyrolysis or gasification in the absence (or near-absence) of oxygen, has recently garnered interest for both agricultural and environmental management purposes owing to its unique physicochemical properties. Favorable properties of biochar include its high surface area and porosity, and ability to adsorb a variety of compounds, including nutrients, organic contaminants, and some gases. Physical and chemical properties of biochars are dictated by the feedstock and production processes (pyrolysis or gasification temperature, conversion technology and pre- and post-treatment processes, if any), which vary widely across commercially produced biochars. In this study, several commercially available biochars derived from waste wood are characterized for physical and chemical properties that can signify their relevant environmental applications. Parameters characterized include: physical properties (particle size distribution, specific gravity, density, porosity, surface area), hydraulic properties (hydraulic conductivity and water holding capacity), and chemical and electrochemical properties (organic matter and organic carbon contents, pH, oxidation-reduction potential and electrical conductivity, zeta potential, carbon, nitrogen and hydrogen (CHN) elemental composition, polycyclic aromatic hydrocarbons (PAHs), heavy metals, and leachable PAHs and heavy metals). A wide range of fixed carbon (0-47.8%), volatile matter (28-74.1%), and ash contents (1.5-65.7%) were observed among tested biochars. A high variability in surface area (0.1-155.1g/m(2)) and PAH and heavy metal contents of the solid phase among commercially available biochars was also observed (0.7-83 mg kg(-1)), underscoring the importance of pre-screening biochars prior to application. Production conditions appear to dictate PAH content--with the highest PAHs observed in biochar produced via fast pyrolysis and lowest among the gasification

  6. Characterization of urban waste management practices in developing Asian countries: A new analytical framework based on waste characteristics and urban dimension.

    PubMed

    Aleluia, João; Ferrão, Paulo

    2016-12-01

    This paper characterizes municipal solid waste (MSW) management practices in developing Asia, with a focus on low and middle-income countries. The analysis that is conducted supports a proposed framework that maps out the trends observed in the region in relation to two parameters, waste compositions and urban dimension, which was prepared based on a set of national and urban case studies. The management of MSW in developing Asian countries is driven, first and foremost, by a public health imperative: the collection and disposal of waste in order to avoid the spread of disease vectors from uncollected waste. This comes, however, at a high cost, with local government authorities in these countries spending up to 50% of their budgets in the provision of these services. Little or no value is derived from waste, which is typically seen as a liability and not as a resource that can be harnessed. On the other hand, in many cities in developing Asia there is an informal sector that ekes out a living from the recovery of recyclable materials found in waste. Members of this "informal waste sector" are especially active in areas that are not served by formal waste collection systems, such as slums or squatter areas. A distinctive element shared among many cities in developing Asian countries concerns the composition of the municipal solid waste. MSW in those countries tends to be richer in biodegradable organic matter, which usually accounts for more than 50% of the total waste composition, suggesting that biological methods are more appropriate for treating this organic fraction. Conversely, thermal combustion technologies, which are extensively applied in high-income countries, are technically and economically challenging to deploy in light of the lower calorific value of waste streams which are rich in organics and moisture. Specific approaches and methods are therefore required for designing adequate waste management systems in developing Asian countries. In addition

  7. EPA's Review of DOE's Inventory Tracking for TRU Wastes at Waste Control Specialists

    EPA Pesticide Factsheets

    On April 9, 2014, EPA's Waste Isolation Pilot Plant (WIPP) waste characterization team visited Waste Control Specialists (WCS) to determine whether DOE was meeting EPA's waste inventory tracking requirements at 40 CFR 194.24(c)(4).

  8. Remote-handled/special case TRU waste characterization summary

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Detamore, J.A.

    1984-03-30

    TRU wastes are those (other than high level waste) contaminated with specified quantities of certain alpha-emitting radionuclides of long half-life and high specific radiotoxicity. TRU waste is defined as /sup 226/Ra isotopic sources and those other materials that, without regard to source or form, are contaminated with transuranic elements with half-lives greater than 20 years, and have TRU alpha contamination greater than 100 nCi/g. RH TRU waste has high beta and gamma radiation levels, up to 30,000 R/hr, and thermal output may be a few hundred watts per container. The radiation levels in most of this remotely handled (RH) TRUmore » waste, however, are below 100 R/hr. Remote-handled wastes are stored at Los Alamos, Hanford, Oak Ridge, and the Idaho National Engineering Laboratory. This report presents a site by site discussion of RH waste handling, placement, and container data. This is followed by a series of data tables that were compiled in the TRU Waste Systems Office. These tables are a compendium of data that are the most up to date and accurate data available today. 10 tables.« less

  9. Municipal solid waste characterization and quantification as a measure towards effective waste management in Ghana.

    PubMed

    Miezah, Kodwo; Obiri-Danso, Kwasi; Kádár, Zsófia; Fei-Baffoe, Bernard; Mensah, Moses Y

    2015-12-01

    Reliable national data on waste generation and composition that will inform effective planning on waste management in Ghana is absent. To help obtain this data on a regional basis, selected households in each region were recruited to obtain data on rate of waste generation, physical composition of waste, sorting and separation efficiency and per capita of waste. Results show that rate of waste generation in Ghana was 0.47 kg/person/day, which translates into about 12,710 tons of waste per day per the current population of 27,043,093. Nationally, biodegradable waste (organics and papers) was 0.318 kg/person/day and non-biodegradable or recyclables (metals, glass, textiles, leather and rubbers) was 0.096 kg/person/day. Inert and miscellaneous waste was 0.055 kg/person/day. The average household waste generation rate among the metropolitan cities, except Tamale, was high, 0.72 kg/person/day. Metropolises generated higher waste (average 0.63 kg/person/day) than the municipalities (0.40 kg/person/day) and the least in the districts (0.28 kg/person/day) which are less developed. The waste generation rate also varied across geographical locations, the coastal and forest zones generated higher waste than the northern savanna zone. Waste composition was 61% organics, 14% plastics, 6% inert, 5% miscellaneous, 5% paper, 3% metals, 3% glass, 1% leather and rubber, and 1% textiles. However, organics and plastics, the two major fractions of the household waste varied considerably across the geographical areas. In the coastal zone, the organic waste fraction was highest but decreased through the forest zone towards the northern savanna. However, through the same zones towards the north, plastic waste rather increased in percentage fraction. Households did separate their waste effectively averaging 80%. However, in terms of separating into the bin marked biodegradables, 84% effectiveness was obtained whiles 76% effectiveness for sorting into the bin labeled other waste was

  10. Kindler syndrome.

    PubMed

    Kaviarasan, P K; Prasad, P V S; Shradda; Viswanathan, P

    2005-01-01

    Kindler syndrome is a rare autosomal recessive disorder associated with skin fragility. It is characterized by blistering in infancy, photosensitivity and progressive poikiloderma. The syndrome involves the skin and mucous membrane with radiological changes. The genetic defect has been identified on the short arm of chromosome 20. This report describes an 18-year-old patient with classical features like blistering and photosensitivity in childhood and the subsequent development of poikiloderma. The differential diagnosis of Kindler syndrome includes diseases like Bloom syndrome, Cockayne syndrome, dyskeratosis congenita, epidermolysis bullosa, Rothmund-Thomson syndrome and xeroderma pigmentosum. Our patient had classical cutaneous features of Kindler syndrome with phimosis as a complication.

  11. Proteomic and genomic characterization of a yeast model for Ogden syndrome

    PubMed Central

    Dörfel, Max J.; Fang, Han; Crain, Jonathan; Klingener, Michael; Weiser, Jake

    2016-01-01

    Abstract Naa10 is an Nα‐terminal acetyltransferase that, in a complex with its auxiliary subunit Naa15, co‐translationally acetylates the α‐amino group of newly synthetized proteins as they emerge from the ribosome. Roughly 40–50% of the human proteome is acetylated by Naa10, rendering this an enzyme one of the most broad substrate ranges known. Recently, we reported an X‐linked disorder of infancy, Ogden syndrome, in two families harbouring a c.109 T > C (p.Ser37Pro) variant in NAA10. In the present study we performed in‐depth characterization of a yeast model of Ogden syndrome. Stress tests and proteomic analyses suggest that the S37P mutation disrupts Naa10 function and reduces cellular fitness during heat shock, possibly owing to dysregulation of chaperone expression and accumulation. Microarray and RNA‐seq revealed a pseudo‐diploid gene expression profile in ΔNaa10 cells, probably responsible for a mating defect. In conclusion, the data presented here further support the disruptive nature of the S37P/Ogden mutation and identify affected cellular processes potentially contributing to the severe phenotype seen in Ogden syndrome. Data are available via GEO under identifier GSE86482 or with ProteomeXchange under identifier PXD004923. © 2016 The Authors. Yeast published by John Wiley & Sons, Ltd. PMID:27668839

  12. Waste Analysis Plan and Waste Characterization Survey, Barksdale AFB, Louisiana

    DTIC Science & Technology

    1991-03-01

    review to assess if analysis is needed, any analyses that are to be provided by generators, and methods to be used to meet specific waste analysis ...sampling method , sampling frequency, parameters of analysis , SW 846 test methods , Department of Transportation (DOT) shipping name and hazard class...S.e.iceA w/Atchs 2. HQ SAC/DEV Ltr, 28 Sep 90 19 119 APPENDIX B Waste Analysis Plan Rationale 21 APPENDIX B 1. SAMPLING METHOD RATIONALE: Composite Liquid

  13. Marshall Space Flight Center solid waste characterization and recycling improvement study: General office and laboratory waste, scrap metal, office and flight surplus

    NASA Technical Reports Server (NTRS)

    Eley, Michael H.; Crews, Lavonne; Johnston, Ben; Lee, David; Colebaugh, James

    1995-01-01

    The primary objectives of the study were to characterize the solid waste stream for MSFC facilities in Huntsville, Alabama, and to evaluate their present recycling program. The purpose of the study was to determine if improvements could be made in terms of increasing quantities of the present commodities collected, adding more recyclables to the program, and streamlining or improving operational efficiency. In conducting the study, various elements were implemented. These included sampling and sorting representative samples of the waste stream; visually inspecting each refuse bin, recycle bin, and roll-off; interviewing employees and recycling coordinators of other companies; touring local material recycling facilities; contacting experts in the field; and performing a literature search.

  14. Anaerobic co-digestion of commercial food waste and dairy manure: Characterizing biochemical parameters and synergistic effects.

    PubMed

    Ebner, Jacqueline H; Labatut, Rodrigo A; Lodge, Jeffrey S; Williamson, Anahita A; Trabold, Thomas A

    2016-06-01

    Anaerobic digestion of commercial food waste is a promising alternative to landfilling commercial food waste. This study characterized 11 types of commercial food wastes and 12 co-digestion blends. Bio-methane potential, biodegradable fraction, and apparent first-order hydrolysis rate coefficients were reported based upon biochemical methane potential (BMP) assays. Food waste bio-methane potentials ranged from 165 to 496 mL CH4/g VS. Substrates high in lipids or readily degradable carbohydrates showed the highest methane production. Average bio-methane potential observed for co-digested substrates was -5% to +20% that of the bio-methane potential of the individual substrates weighted by VS content. Apparent hydrolysis rate coefficients ranged from 0.19d(-1) to 0.65d(-1). Co-digested substrates showed an accelerated apparent hydrolysis rate relative to the weighted average of individual substrate rates. These results provide a database of key bio-digestion parameters to advance modeling and utilization of commercial food waste in anaerobic digestion. Copyright © 2016 Elsevier Ltd. All rights reserved.

  15. Molecular characterization of a novel X-linked syndrome involving developmental delay and deafness.

    PubMed

    Hildebrand, Michael S; de Silva, Michelle G; Tan, Tiong Yang; Rose, Elizabeth; Nishimura, Carla; Tolmachova, Tanya; Hulett, Joanne M; White, Susan M; Silver, Jeremy; Bahlo, Melanie; Smith, Richard J H; Dahl, Hans-Henrik M

    2007-11-01

    X-linked syndromes associated with developmental delay and sensorineural hearing loss (SNHL) have been characterized at the molecular level, including Mohr-Tranebjaerg syndrome and Norrie disease. In this study we report on a novel X-linked recessive, congenital syndrome in a family with developmental delay and SNHL that maps to a locus associated with mental retardation (MR) for which no causative gene has been identified. The X-linked recessive inheritance and congenital nature of the syndrome was confirmed by detailed clinical investigation and the family history. Linkage mapping of the X-chromosome was conducted to ascertain the disease locus and candidate genes were screened by direct sequencing and STRP analysis. The recessive syndrome was mapped to Xp11.3-q21.32 and a deletion was identified in a regulatory region upstream of the POU3F4 gene in affected family members. Since mutations in POU3F4 cause deafness at the DFN3 locus, the deletion is the likely cause of the SNHL in this family. The choroideremia (CHM) gene was also screened and a novel missense change was identified. The alteration changes the serine residue at position 89 in the Rab escort 1 protein (REP-1) to a cysteine (S89C). Prenylation of Rab proteins was investigated in patients and the location of REP-1 expression in the brain determined. However, subsequent analysis revealed that this change in CHM was polymorphic having no effect on REP-1 function. Although the causative gene at the MR locus in this family has not been identified, there are a number of genes involved in syndromic and nonsyndromic forms of MR that are potential candidates. Copyright 2007 Wiley-Liss, Inc.

  16. Characterizing Urban Household Waste Generation and Metabolism Considering Community Stratification in a Rapid Urbanizing Area of China.

    PubMed

    Xiao, Lishan; Lin, Tao; Chen, Shaohua; Zhang, Guoqin; Ye, Zhilong; Yu, Zhaowu

    2015-01-01

    The relationship between social stratification and municipal solid waste generation remains uncertain under current rapid urbanization. Based on a multi-object spatial sampling technique, we selected 191 households in a rapidly urbanizing area of Xiamen, China. The selected communities were classified into three types: work-unit, transitional, and commercial communities in the context of housing policy reform in China. Field survey data were used to characterize household waste generation patterns considering community stratification. Our results revealed a disparity in waste generation profiles among different households. The three community types differed with respect to family income, living area, religious affiliation, and homeowner occupation. Income, family structure, and lifestyle caused significant differences in waste generation among work-unit, transitional, and commercial communities, respectively. Urban waste generation patterns are expected to evolve due to accelerating urbanization and associated community transition. A multi-scale integrated analysis of societal and ecosystem metabolism approach was applied to waste metabolism linking it to particular socioeconomic conditions that influence material flows and their evolution. Waste metabolism, both pace and density, was highest for family structure driven patterns, followed by lifestyle and income driven. The results will guide community-specific management policies in rapidly urbanizing areas.

  17. Characterizing Urban Household Waste Generation and Metabolism Considering Community Stratification in a Rapid Urbanizing Area of China

    PubMed Central

    Xiao, Lishan; Lin, Tao; Chen, Shaohua; Zhang, Guoqin; Ye, Zhilong; Yu, Zhaowu

    2015-01-01

    The relationship between social stratification and municipal solid waste generation remains uncertain under current rapid urbanization. Based on a multi-object spatial sampling technique, we selected 191 households in a rapidly urbanizing area of Xiamen, China. The selected communities were classified into three types: work-unit, transitional, and commercial communities in the context of housing policy reform in China. Field survey data were used to characterize household waste generation patterns considering community stratification. Our results revealed a disparity in waste generation profiles among different households. The three community types differed with respect to family income, living area, religious affiliation, and homeowner occupation. Income, family structure, and lifestyle caused significant differences in waste generation among work-unit, transitional, and commercial communities, respectively. Urban waste generation patterns are expected to evolve due to accelerating urbanization and associated community transition. A multi-scale integrated analysis of societal and ecosystem metabolism approach was applied to waste metabolism linking it to particular socioeconomic conditions that influence material flows and their evolution. Waste metabolism, both pace and density, was highest for family structure driven patterns, followed by lifestyle and income driven. The results will guide community-specific management policies in rapidly urbanizing areas. PMID:26690056

  18. Descriptive summary of an outbreak of porcine post-weaning multisystemic wasting syndrome (PMWS ) in New Zealand.

    PubMed

    Neumann, E J; Dobbinson, S S A; Welch, E B M; Morris, R S

    2007-12-01

    Investigations were conducted to determine the cause of an acute, multi-farm outbreak of porcine respiratory disease that included diarrhoea and subsequent loss of body condition in affected pigs. A definition for post-weaning multisystemic wasting syndrome (PMWS) including both clinical and pathological features, previously developed for the pig industry in New Zealand, was applied to the current outbreak. In addition to self-reporting by owners of affected farms, local veterinarians, disease and epidemiology consultants, and animal health officials from the Ministry of Agriculture and Forestry (MAF) were involved in conducting farm visits and submission of diagnostic specimens. Pathogens known to be endemic in the pig industry in New Zealand as well as likely exotic diseases were excluded as causative agents of the outbreak. Clinical signs including dyspnoea, diarrhoea, and rapid loss of body condition were consistent with the New Zealand case definition for PMWS. Interstitial pneumonia, pulmonary oedema, generalised lymph-node enlargement, and presence of porcine circovirus type 2 (PCV2) inclusion bodies were consistently identified in affected pigs. Classical swine fever virus (CSFv), Porcine reproductive and respiratory syndrome virus (PRRSv), and Influenza virus were ruled out, using molecular and traditional virological techniques. Spread of the disease between farms was hypothesised to be facilitated by locally migrating flocks of black-backed seagulls. The original source of the disease incursion was not identified. Based on the consistent presence of circovirus-associated lesions in lymphoid tissues in combination with generalised enlargement of lymph nodes, histiocytic interstitial pneumonia, clinical wasting, and poor response to antibiotic therapy, a diagnosis of PMWS was made. PMWS should be considered in the differential diagnoses of sudden onset of respiratory dyspnoea, diarrhoea, and rapid loss of body condition in young pigs in New Zealand pig

  19. Hazardous Waste and Wastewater Characterization Survey, Columbus AFB, Mississippi

    DTIC Science & Technology

    1988-06-01

    behind bldg 322 (Liquid Fuels Maintenance Branch). These wastes are then picked up by a waste oil contractor. All other drummed wastes are disposed of...is responsible for custody of the waste until a contractor (currently, Chemical Waste Management) comes to pick up the waste. Prior to disposal...chemicals are used up in the process. Any leftover chemicals are drained and stored in containers for use at a later time. All empty containers are

  20. Annual Report, Fall 2016: Identifying Cost Effective Tank Waste Characterization Approaches

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Reboul, S. H.; DiPrete, D. P.

    2016-12-12

    This report documents the activities that were performed during the second year of a project undertaken to improve the cost effectiveness and timeliness of SRNL’s tank closure characterization practices. The activities performed during the first year of the project were previously reported in SRNL-STI-2015-00144. The scope of the second year activities was divided into the following three primary tasks: 1) develop a technical basis and strategy for improving the cost effectiveness and schedule of SRNL’s tank closure characterization program; 2) initiate the design and assembly of a new waste removal system for improving the throughput and reducing the personnel dosemore » associated with extraction chromatography radiochemical separations; and 3) develop and perform feasibility testing of three alternative radiochemical separation protocols holding promise for improving high resource demand/time consuming tank closure sample analysis methods.« less

  1. Recurrent Miller Fisher syndrome.

    PubMed

    Madhavan, S; Geetha; Bhargavan, P V

    2004-07-01

    Miller Fisher syndrome (MFS) is a variant of Guillan Barre syndrome characterized by the triad of ophthalmoplegia, ataxia and areflexia. Recurrences are exceptional with Miller Fisher syndrome. We are reporting a case with two episodes of MFS within two years. Initially he presented with partial ophthalmoplegia, ataxia. Second episode was characterized by full-blown presentation characterized by ataxia, areflexia and ophthalmoplegia. CSF analysis was typical during both episodes. Nerve conduction velocity study was fairly within normal limits. MRI of brain was within normal limits. He responded to symptomatic measures initially, then to steroids in the second episode. We are reporting the case due to its rarity.

  2. [Kenny-Caffey syndrome and its related syndromes].

    PubMed

    Isojima, Tsuyoshi; Kitanaka, Sachiko

    2015-11-01

    Kenny-Caffey syndrome (KCS) is a very rare dysmorphologic syndrome characterized by proportionate short stature, cortical thickening and medullary stenosis of tubular bones, delayed closure of anterior fontanelle, eye abnormalities, and hypoparathyroidism. Two types of KCS were known: the autosomal recessive form (KCS type 1), which is caused by mutations of the TBCE gene, and the autosomal dominant form (KCS type 2), which is caused by mutations of the FAM111A gene. TBCE mutation also causes hypoparathyroidism-retardation-dysmorphism syndrome, and FAM111A mutation also causes gracile bone dysplasia. These two diseases can be called as KCS-related syndromes. In this article, we review the clinical manifestations of KCS and discuss its related syndromes.

  3. The characterization of radioactive waste: a critical review of techniques implemented or under development at CEA, France

    NASA Astrophysics Data System (ADS)

    Pérot, Bertrand; Jallu, Fanny; Passard, Christian; Gueton, Olivier; Allinei, Pierre-Guy; Loubet, Laurent; Estre, Nicolas; Simon, Eric; Carasco, Cédric; Roure, Christophe; Boucher, Lionel; Lamotte, Hervé; Comte, Jérôme; Bertaux, Maïté; Lyoussi, Abdallah; Fichet, Pascal; Carrel, Frédérick

    2018-03-01

    This review paper describes the destructive and non-destructive measurements implemented or under development at CEA, in view to perform the most complete radioactive waste characterization. First, high-energy photon imaging (radiography, tomography) brings essential information on the waste packages, such as density, position and shape of the waste inside the container and in the possible binder, quality of coating and blocking matrices, presence of internal shields or structures, presence of cracks, voids, or other defects in the container or in the matrix, liquids or other forbidden materials, etc. Radiological assessment is then performed using a series of non-destructive techniques such as gamma-ray spectroscopy, which allows characterizing a wide range of radioactive and nuclear materials, passive neutron coincidence counting and active neutron interrogation with the differential die-away technique, or active photon interrogation with high-energy photons (photofission), to measure nuclear materials. Prompt gamma neutron activation analysis (PGNAA) can also be employed to detect toxic chemicals or elements which can greatly influence the above measurements, such as neutron moderators or absorbers. Digital auto-radiography can also be used to detect alpha and beta contaminated waste. These non-destructive assessments can be completed by gas measurements, to quantify the radioactive and radiolysis gas releases, and by destructive examinations such as coring homogeneous waste packages or cutting the heterogeneous ones, in view to perform visual examination and a series of physical, chemical, and radiochemical analyses on samples. These last allow for instance to check the mechanical and containment properties of the package envelop, or of the waste binder, to measure toxic chemicals, to assess the activity of long-lived radionuclides or pure beta emitters, to determine the isotopic composition of nuclear materials, etc.

  4. Characterizing the proposed geologic repository for high-level radioactive waste at Yucca Mountain, Nevada--hydrology and geochemistry

    USGS Publications Warehouse

    Stuckless, John S.; Levich, Robert A.

    2012-01-01

    This hydrology and geochemistry volume is a companion volume to the 2007 Geological Society of America Memoir 199, The Geology and Climatology of Yucca Mountain and Vicinity, Southern Nevada and California, edited by Stuckless and Levich. The work in both volumes was originally reported in the U.S. Department of Energy regulatory document Yucca Mountain Site Description, for the site characterization study of Yucca Mountain, Nevada, as the proposed U.S. geologic repository for high-level radioactive waste. The selection of Yucca Mountain resulted from a nationwide search and numerous committee studies during a period of more than 40 yr. The waste, largely from commercial nuclear power reactors and the government's nuclear weapons programs, is characterized by intense penetrating radiation and high heat production, and, therefore, it must be isolated from the biosphere for tens of thousands of years. The extensive, unique, and often innovative geoscience investigations conducted at Yucca Mountain for more than 20 yr make it one of the most thoroughly studied geologic features on Earth. The results of these investigations contribute extensive knowledge to the hydrologic and geochemical aspects of radioactive waste disposal in the unsaturated zone. The science, analyses, and interpretations are important not only to Yucca Mountain, but also to the assessment of other sites or alternative processes that may be considered for waste disposal in the future. Groundwater conditions, processes, and geochemistry, especially in combination with the heat from radionuclide decay, are integral to the ability of a repository to isolate waste. Hydrology and geochemistry are discussed here in chapters on unsaturated zone hydrology, saturated zone hydrology, paleohydrology, hydrochemistry, radionuclide transport, and thermally driven coupled processes affecting long-term waste isolation. This introductory chapter reviews some of the reasons for choosing to study Yucca Mountain as a

  5. Characterizing the proposed geologic repository for high-level radioactive waste at Yucca Mountain, Nevada: hydrology and geochemistry

    USGS Publications Warehouse

    Stuckless, John S.; Levich, Robert A.

    2012-01-01

    This hydrology and geochemistry volume is a companion volume to the 2007 Geological Society of America Memoir 199, The Geology and Climatology of Yucca Mountain and Vicinity, Southern Nevada and California, edited by Stuckless and Levich. The work in both volumes was originally reported in the U.S. Department of Energy regulatory document Yucca Mountain Site Description, for the site characterization study of Yucca Mountain, Nevada, as the proposed U.S. geologic repository for high-level radioactive waste. The selection of Yucca Mountain resulted from a nationwide search and numerous committee studies during a period of more than 40 yr. The waste, largely from commercial nuclear power reactors and the government's nuclear weapons programs, is characterized by intense penetrating radiation and high heat production, and, therefore, it must be isolated from the biosphere for tens of thousands of years. The extensive, unique, and often innovative geoscience investigations conducted at Yucca Mountain for more than 20 yr make it one of the most thoroughly studied geologic features on Earth. The results of these investigations contribute extensive knowledge to the hydrologic and geochemical aspects of radioactive waste disposal in the unsaturated zone. The science, analyses, and interpretations are important not only to Yucca Mountain, but also to the assessment of other sites or alternative processes that may be considered for waste disposal in the future. Groundwater conditions, processes, and geochemistry, especially in combination with the heat from radionuclide decay, are integral to the ability of a repository to isolate waste. Hydrology and geochemistry are discussed here in chapters on unsaturated zone hydrology, saturated zone hydrology, paleohydrology, hydrochemistry, radionuclide transport, and thermally driven coupled processes affecting long-term waste isolation. This introductory chapter reviews some of the reasons for choosing to study Yucca Mountain as a

  6. WCATS: Waste Documentation, Course No. 8504

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Simpson, Sandy

    2016-04-14

    This course was developed for individuals at Los Alamos National Laboratory (LANL) who characterize and document waste streams in the Waste Compliance and Tracking System (WCATS) according to Environmental Protection Agency (EPA) Department of Transportation (DOT) regulations, Department of Energy Orders, and other applicable criteria. When you have completed this course, you will be able to recognize how waste documentation enables LANL to characterize and classify hazardous waste for compliant treatment, storage, and disposal, identify the purpose of the waste stream profile (WSP), identify the agencies that provide guidance for waste management, and more.

  7. Characterization of the non-metal fraction of the processed waste printed circuit boards.

    PubMed

    Kumar, Amit; Holuszko, Maria E; Janke, Travis

    2018-05-01

    Electronic waste is one the fastest growing waste streams in the world and waste printed circuit boards (PCB) are the most valuable part of this stream due to the presence of gold, silver, copper, and palladium. The metal present in PCBs is mostly recovered for the market value whereas the nonmetal fractions are often ignored. This research explored the characteristics of the non-metal fraction (NMF) obtained after the processing of milled waste PCBs with a focus on responsible end-of-life solutions, in the form of non-hazardous landfilling or incineration. The NMF was characterized using sizing, assaying, loss on ignition, calorific value measurement, and thermogravimetric analysis (TGA). The result showed that the metal content in the NMF increased with decrease in the particle size for most of the metals except antimony and the result from loss on ignition (LOI) also showed that over 50% of the coarser fraction represented organic matter compared to less than 30% for the finest fraction. The study also showed that after the recovery of metals from the waste PCBs, landfill leaching for most of the metal is reduced below the environmental limits, with lead being the only exception. The lead leachate concentration of 18 mg/L was observed, which requires further treatment prior to landfilling. With an energy value of 16 GJ/t, the NMF could provide high energy recovery if incinerated but 194 mg/kg of hazardous flame retardants present in the NMF might be released if the combustion process is not closely monitored. Copyright © 2018 Elsevier Ltd. All rights reserved.

  8. Numerical Magnitude Processing Impairments in Genetic Syndromes: A Cross-Syndrome Comparison of Turner and 22Q11.2 Deletion Syndromes

    ERIC Educational Resources Information Center

    Brankaer, Carmen; Ghesquière, Pol; De Wel, Anke; Swillen, Ann; De Smedt, Bert

    2017-01-01

    Cross-syndrome comparisons offer an important window onto understanding heterogeneity in mathematical learning disabilities or dyscalculia. The present study therefore investigated symbolic numerical magnitude processing in two genetic syndromes that are both characterized by mathematical learning disabilities: Turner syndrome and 22q11.2 deletion…

  9. Establishment and characterization of Roberts syndrome and SC phocomelia model medaka (Oryzias latipes).

    PubMed

    Morita, Akihiro; Nakahira, Kumiko; Hasegawa, Taeko; Uchida, Kaoru; Taniguchi, Yoshihito; Takeda, Shunichi; Toyoda, Atsushi; Sakaki, Yoshiyuki; Shimada, Atsuko; Takeda, Hiroyuki; Yanagihara, Itaru

    2012-06-01

    Roberts syndrome and SC phocomelia (RBS/SC) are genetic autosomal recessive syndromes caused by establishment of cohesion 1 homolog 2 ( ESCO 2) mutation. RBS/SC appear to have a variety of clinical features, even with the same mutation of the ESCO2 gene. Here, we established and genetically characterized a medaka model of RBS/SC by reverse genetics. The RBS/SC model was screened from a mutant medaka library produced by the Targeting Induced Local Lesions in Genomes method. The medaka mutant carrying the homozygous mutation at R80S in the conserved region of ESCO2 exhibited clinical variety (i.e. developmental arrest with craniofacial and chromosomal abnormalities and embryonic lethality) as characterized in RBS/SC. Moreover, widespread apoptosis and downregulation of some gene expression, including notch1a, were detected in the R80S mutant. The R80S mutant is the animal model for RBS/SC and a valuable resource that provides the opportunity to extend knowledge of ESCO2. Downregulation of some gene expression in the R80S mutant is an important clue explaining non-correlation between genotype and phenotype in RBS/SC. © 2012 The Authors Development, Growth & Differentiation © 2012 Japanese Society of Developmental Biologists.

  10. Gorlin syndrome.

    PubMed

    Devi, Basanti; Behera, Binodini; Patro, Sibasish; Pattnaik, Subhransu S; Puhan, Manas R

    2013-05-01

    Gorlin Syndrome, a rare genodermatosis, otherwise known as Nevoid basal cell carcinoma syndrome (NBCCS) is a multisystem disease affecting skin, nervous system, eyes, endocrine glands, and bones. It is characterized by multiple basal cell carcinomas, palmoplantar pits, jaw cysts, and bony deformities like kyphoscoliosis and frontal bossing. We would like to report a case of Gorlin syndrome with classical features, as this is a rare genodermatosis.

  11. Green separation and characterization of fatty acids from solid wastes of leather industry in supercritical fluid CO2.

    PubMed

    Onem, Ersin; Renner, Manfred; Prokein, Michael

    2018-05-26

    Considerable tannery waste is generated by leather industry around the world. Recovery of the value-added products as natural fats from the solid wastes gained interest of many researchers. In this study, supercritical fluid separation method was applied for the fatty acid isolation from leather industry solid wastes. Pre-fleshing wastes of the double-face lambskins were used as natural fat source. Only supercritical CO 2 was used as process media without any solvent additive in high-pressure view cell equipment. The effect of different conditions was investigated for the best separation influence. The parameters of pressure (100 to 200 bar), temperature (40 to 80 °C), and time (1 to 3 h) were considered. Extraction yields and fat yields of the parameters were statistically evaluated after the processes. Maximum 78.57 wt% fat yield was obtained from leather industry fleshings in supercritical fluid CO 2 at 200 bar, 80 °C, and 2 h. Morever, conventional Soxhlet and supercritical CO 2 extracted fatty acids were characterized by using gas chromatography (GC) coupled with mass spectrometry (MS) and flame ionization detector (FID). Fourier transform infrared spectroscopy (FTIR) and differential scanning calorimetry (DSC) characterizations were also done. The results showed that supercritical fluid CO 2 extraction was highly effective for the fat separation as green solvent and leather industry tannery wastes could be used for the value-added products.

  12. Industrial wastes from the boat-building sector in the Marche Region (Italy): a parametric and chemical-physical characterization.

    PubMed

    Carchesio, M; Tatàno, F; Tosi, G; Trivellone, C H

    2013-01-01

    Using the renowned leisure boat-building sector in the Marche Region (Italy) as a case-study, this paper addresses the characterization of (1) the industrial waste generation from the building of composite material-based boats and (2) some chemical-physical properties of representative types of boat-building residues (plastic foam, hardened resin, fibre-reinforced composite residues, and sanding dust). A parametric evaluation based on the number of employees gave a representative unit generation rate per employee (UGRpE) of 1.47 tons(waste) employee(-1) year(-1) for the entire Marche regional boatbuilding district, whereas evaluations carried out separately for three case-study companies provided values of 1.56, 3.07, and 1.12 tons(waste) employee(-1) year(-1) as representative for a mass-produced motor boat builder (case-study company '1'), a customized sailing boat builder (case-study company '2'), and a mould and structural component builder (case-study company '3'), respectively. The original proposal and evaluation of two additional generation rates based on physical characteristics intrinsic to the manufactured product, i.e. the unit generation rate per boat area (UGRpA) and per boat weight (UGRpW), confirmed the higher waste generation for the sailing boat builder(representative UGRpA and UGRpW values of 0.35 tons(waste) m(-2)(boat) year(-1) and 2. 71 tons(waste) tons(-1)(boat) year(-1), respectively) compared with the motor boat builder (representative UGRpA and UGRpW values of 0.06 tons(waste) m(-2)(boat) year(-1) and 0.49 tons(waste) tons(-1)(boat) year(-1), respectively). The chemical-physical property characterization of the selected residues revealed the following aspects: a general condition of low moisture contents; significant ash contents in the glass- and carbon-fibre composite residues and the correlated sanding dust; and relatively high energy content values in the overall range 14,144-32,479 kJ kg(-1), expressed as the lower heating value.

  13. Mechanism and novel therapeutic approaches to wasting in chronic disease.

    PubMed

    Ebner, Nicole; Springer, Jochen; Kalantar-Zadeh, Kamyar; Lainscak, Mitja; Doehner, Wolfram; Anker, Stefan D; von Haehling, Stephan

    2013-07-01

    Cachexia is a multifactorial syndrome defined by continuous loss of skeletal muscle mass - with or without loss of fat mass - which cannot be fully reversed by conventional nutritional support and which may lead to progressive functional impairment and increased death risk. Its pathophysiology is characterized by negative protein and energy balance driven by a variable combination of reduced food intake and abnormal metabolism. Muscle wasting is encountered in virtually all chronic disease states in particular during advanced stages of the respective illness. Several pre-clinical and clinical studies are ongoing to ameliorate this clinical problem. The mechanisms of muscle wasting and cachexia in chronic diseases such as cancer, chronic heart failure, chronic obstructive pulmonary disease and chronic kidney disease are described. We discuss therapeutic targets and such potential modulators as appetite stimulants, selective androgen receptor modulators, amino acids and naturally occurring peptide hormones. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  14. Characterizations of the radioactive waste by the remotely-controlled collimated spectrometric system

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Stepanov, Vyacheslav E.; Potapov, Victor N.; Smirnov, Sergey V.

    Decontamination and decommissioning of the research reactors MR (Testing Reactor) and RFT (Reactor of Physics and Technology) has recently been initiated in the National Research Center (NRC) 'Kurchatov institute', Moscow. In the building, neighboring to the reactor, the storage of HLRW is located. The storage is made of monolithic concrete in which steel cells depth 4 m are located. In cells of storage the HLRW packed into cases are placed. These the radioactive waste are also subject to export on long storage in the specialized organization. For characterization of the radioactive waste in cases the remote-controlled collimated spectrometer system wasmore » used. The system consists of a spectrometric collimated gamma-ray detector, a color video camera and a control unit, mounted on a rotator, which are mounted on a tripod with the host computer. For determination of specific activity of radionuclides in cases, it is developed programs of calculation of coefficients of proportionality of specific activity to the corresponding speeds of the account in peaks of full absorption at single specific activity of radionuclides in cases. For determination of these coefficients the mathematical model of spectrometer system based on the Monte-Carlo method was used. Dependences of calibration coefficients for various radionuclides from distance between the detector and a case at various values of the radioactive waste density in cases are given. Measurements of specific activity in cases are taken and are discussed. By results of measurements decisions on the appeal of the radioactive waste being in cases are made. (authors)« less

  15. Characterization of Mast Cell Activation Syndrome

    PubMed Central

    Afrin, Lawrence B.; Self, Sally; Menk, Jeremiah; Lazarchick, John

    2016-01-01

    Background Mast cell activation syndrome (MCAS), a recently recognized non-neoplastic mast cell (MC) disease driving chronic multisystem inflammation ± allergy, appears prevalent and thus important. We report the first systematic characterization of a large MCAS population. Method Demographics, comorbidities, symptoms, family histories, and physical exam and laboratory findings were reviewed in 298 retrospective and 115 prospective MCAS patients. Blood samples from prospective subjects were examined by flow cytometry for clonal MC disease and tested for cytokines potentially driving the monocytosis frequent in MCAS. Results Demographically, white females dominated. Median ages at symptom onset/diagnosis were 9/49 years (ranges 0–88/16–92); median time from symptom onset to diagnosis was 30 years (range 1–85). Median numbers of comorbidities/symptoms/family medical issues were 11/20/4 (ranges 1–66/2–84/0–33). Gastroesophageal reflux, fatigue, and dermatographism were the most common comorbidity, symptom, and exam finding. Abnormalities in routine labs were common and diverse but typically modest. The most useful diagnostic markers were heparin, prostaglandin D2, histamine, and chromogranin A. Flow cytometric and cytokine assessments were unhelpful. Conclusions Our study highlights MCAS’s morbidity burden and challenging heterogeneity. Recognition is important given good survival and treatment prospects. PMID:28262205

  16. [Association Budd Chiari syndrome, antiphospholipid syndrome and Grave's disease].

    PubMed

    Mouelhi, Leila; Chaieb, Mouna; Debbeche, Radhouane; Salem, Mohamed; Sfar, Imene; Trabelsi, Sinda; Gorgi, Yosr; Najjar, Taoufik

    2009-02-01

    Antiphospholipid syndrome is revealed by Budd Chiari syndrome in 5% of the cases. Antiphospholipid syndrome is characterized by venous or arterial thrombosis, foetal loss and positivity of antiphospholipid antibodies, namely lupus anticoagulant, anticardiolipin antibodies and anti-beta2-glycoprotein I. Anticardiolipin antibodies was reported in auto-immune thyroid disorders, particularly in Grave's disease. Antiphospholipid syndrome associated to Grave's disease was reported in only three cases. To describe a case report of association of Grave's disease and antiphospholipid syndrome. We report the first case of Grave's disease associated with antiphospholipid syndrome, revealed by Budd Chiari syndrome. Our observation is particular by the fact that it is about a patient presenting a Grave's disease associated with antiphospholipid syndrome revealed by Budd Chiari syndrome. This triple association has never been reported in literature. Although association between antiphospholipid syndrome and Grave's disease was previously described, further studies evaluating the coexistence of these two affections in the same patient would be useful.

  17. Anaerobic digestion of municipal solid waste composed of food waste, wastepaper, and plastic in a single-stage system: performance and microbial community structure characterization.

    PubMed

    Wan, Shungang; Sun, Lei; Douieb, Yaniv; Sun, Jian; Luo, Wensui

    2013-10-01

    The performance of municipal organic solid waste anaerobic digestion was investigated using a single-stage bioreactor, and the microbial community structures were characterized during the digestion. The results showed that the biogas and methane production rates were 592.4 and 370.1L/kg with volatile solid added at the ratio of 2:1:1 for food waste, wastepaper, and plastic based on dry weight. The methane volume concentration fluctuated between 44.3% and 75.4% at steady stage. Acetic acid, propionic acid, and butyric acid were the major volatile fatty acids produced during the digestion process. The anaerobic process was not inhibited by the accumulation of ammonia and free ammonia. The bacterial community was found to consist of at least 21 bands of bacteria and 12 bands of archaea at the steady state. All of the results indicated that the mixture of food waste, wastepaper, and plastic could be efficiently co-digested using the anaerobic digestion system. Copyright © 2013 Elsevier Ltd. All rights reserved.

  18. Molecular Characterization of Three Canine Models of Human Rare Bone Diseases: Caffey, van den Ende-Gupta, and Raine Syndromes.

    PubMed

    Hytönen, Marjo K; Arumilli, Meharji; Lappalainen, Anu K; Owczarek-Lipska, Marta; Jagannathan, Vidhya; Hundi, Sruthi; Salmela, Elina; Venta, Patrick; Sarkiala, Eva; Jokinen, Tarja; Gorgas, Daniela; Kere, Juha; Nieminen, Pekka; Drögemüller, Cord; Lohi, Hannes

    2016-05-01

    One to two percent of all children are born with a developmental disorder requiring pediatric hospital admissions. For many such syndromes, the molecular pathogenesis remains poorly characterized. Parallel developmental disorders in other species could provide complementary models for human rare diseases by uncovering new candidate genes, improving the understanding of the molecular mechanisms and opening possibilities for therapeutic trials. We performed various experiments, e.g. combined genome-wide association and next generation sequencing, to investigate the clinico-pathological features and genetic causes of three developmental syndromes in dogs, including craniomandibular osteopathy (CMO), a previously undescribed skeletal syndrome, and dental hypomineralization, for which we identified pathogenic variants in the canine SLC37A2 (truncating splicing enhancer variant), SCARF2 (truncating 2-bp deletion) and FAM20C (missense variant) genes, respectively. CMO is a clinical equivalent to an infantile cortical hyperostosis (Caffey disease), for which SLC37A2 is a new candidate gene. SLC37A2 is a poorly characterized member of a glucose-phosphate transporter family without previous disease associations. It is expressed in many tissues, including cells of the macrophage lineage, e.g. osteoclasts, and suggests a disease mechanism, in which an impaired glucose homeostasis in osteoclasts compromises their function in the developing bone, leading to hyperostosis. Mutations in SCARF2 and FAM20C have been associated with the human van den Ende-Gupta and Raine syndromes that include numerous features similar to the affected dogs. Given the growing interest in the molecular characterization and treatment of human rare diseases, our study presents three novel physiologically relevant models for further research and therapy approaches, while providing the molecular identity for the canine conditions.

  19. Identification and characterization of a novel XK splice site mutation in a patient with McLeod syndrome.

    PubMed

    Arnaud, Lionel; Salachas, François; Lucien, Nicole; Maisonobe, Thierry; Le Pennec, Pierre-Yves; Babinet, Jérôme; Cartron, Jean-Pierre

    2009-03-01

    McLeod syndrome is a rare X-linked neuroacanthocytosis syndrome with hematologic, muscular, and neurologic manifestations. McLeod syndrome is caused by mutations in the XK gene whose product is expressed at the red blood cell (RBC) surface but whose function is currently unknown. A variety of XK mutations has been reported but no clear phenotype-genotype correlation has been found, especially for the point mutations affecting splicing sites. A man suspected of neuroacanthocytosis was evaluated by neurologic examination, electromyography, muscle biopsy, muscle computed tomography, and cerebral magnetic resonance imaging. The McLeod RBC phenotype was disclosed by blood smear and immunohematology analyses and then confirmed at the biochemical level by Western blot analysis. The responsible XK mutation was characterized at the mRNA level by reverse transcription-polymerase chain reaction (PCR), identified by genomic DNA sequencing, and verified by allele-specific PCR. A novel XK splice site mutation (IVS1-1G>A) has been identified in a McLeod patient who has developed hematologic, neuromuscular, and neurologic symptoms. This is the first reported example of a XK point mutation affecting the 3' acceptor splice site of Intron 1, and it was demonstrated that this mutation indeed induces aberrant splicing of XK RNA and lack of XK protein at the RBC membrane. The detailed characterization at the molecular biology level of this novel XK splice site mutation associated with the clinical description of the patient contributes to a better understanding of the phenotype-genotype correlation in the McLeod syndrome.

  20. Characterization and potential recycling of home building wood waste

    Treesearch

    Philip A. Araman; D.P. Hindman; M.F. Winn

    2010-01-01

    Construction waste represents a significant portion of landfill waste, estimated as 17% of the total waste stream. Wood construction waste of a 2000 square foot single family home we found to be 1500-3700 lbs of solid-sawn wood, and 1000-1800 lbs of engineered wood products (EWP). Much of the solid-sawn lumber and EWPs could be recycled into several products. Through a...

  1. Duane retraction syndrome type 1 with Usher syndrome type 2: an unreported association.

    PubMed

    Khurana, Bhawna Piplani; Khurana, Aruj Kumar; Grover, Sumit

    2015-05-07

    Duane retraction syndrome is characterized by globe retraction and palpebral fissure narrowing on adduction, with restriction of abduction, adduction, or both. Usher syndrome type 2 consists of congenital bilateral sensorineural hearing loss and retinitis pigmentosa. The authors present a case with a yet unreported association between Duane retraction syndrome type 1 and Usher syndrome type 2. Copyright 2015, SLACK Incorporated.

  2. Characterization of Discharge Areas of Radionuclides Originating From Nuclear Waste Repositories

    NASA Astrophysics Data System (ADS)

    Marklund, L.; Xu, S.; Worman, A.

    2009-05-01

    If leakages in nuclear waste repositories located in crystalline bedrock arise, radionuclides will reach the biosphere and cause a risk of radiological impact. The extent of the radiological impact depends on in which landscape elements the radionuclides emerge. In this study, we investigate if there are certain landscape elements that generally will act as discharge areas for radionuclides leaking from subsurface deposits. We also characterize the typical properties that distinguish these areas from others. In humid regions, landscape topography is the most important driving force for groundwater flow. Because groundwater is the main transporting agent for migrating radionuclides, the topography will determine the flowpaths of leaking radionuclides. How topography and heterogeneities in the subsurface affect the discharge distribution of the radionuclides is therefore an important scope of this study. To address these issues, we developed a 3-D transport model. Our analyses are based on site-specific data from two different areas in Sweden, Forsmark, Uppland, and Oskarshamn, Småland. The Swedish Nuclear Waste Management Company (SKB) has selected these two areas as candidate areas for a deep repository of nuclear waste and the areas are currently subject to site investigations. Our results suggest that there are hot-spots in the landscape i.e. areas with high probability of receiving large amounts of radionuclides from a leaking repository of nuclear waste. The hot-spots concentrate in the sea, streams, lakes and wetlands. All these elements are found at lower elevations in the landscape. This pattern is mostly determined by the landscape topography and the locations of fracture zones. There is a relationship between fracture zones and topography, and therefore the importance of the topography for the discharge area distribution is not contradicted by the heterogeneity in the bedrock. The varieties of landscape elements which have potential for receiving

  3. Characterization of a thermal power plant air heater washing waste: a case study from Iran.

    PubMed

    Saeedi, M; Amini, H R

    2007-02-01

    In Iran most of the electricity is generated by thermal power plants. As a result of fuel oil burning in winter time, the air heaters of the boilers have to be washed and cleaned frequently. The wastewater originating from air heater washing is then treated in an effluent treatment plant by chemical precipitation followed by dewatering of the sludge produced. The resulting waste is classified as specific industrial waste that should be characterized in detail under the Waste Management Act of Iran. The quantity of this waste produced in the studied power plant is about 20 tonnes year(-1). In the present investigation, the first to be carried out in Iran, seven composite samples of dewatered sludge from air heater washing wastewater treatment were subjected to investigation of the physical properties, chemical composition and leaching properties. The most likely pollutants that were of concern in this study were heavy and other hazardous metals (Cd, Co, Cr, Mn, Ni, Pb, Zn and V). The results revealed that mean pH, wet and dry density and moisture content of the waste were 6.31, 1532 kg m(-30, 1879 kg m(-3) and 15.35%, respectively. Magnetite, SiO2, P2O5, CaO, Al2O3 and MgO were the main constituents of the waste with a weight percentage order of 68.88, 5.91, 3.39, 2.64, 2.59 and 1.76%, respectively. The toxicity characteristic leaching procedure test results for some heavy and other hazardous metals showed that mean elemental concentrations of Cd, Co, Cr, Mn, Ni, Pb, V and Zn in leachate were 0.06, 1.55, 5.49, 36.32, 209.10, 0.58, 314.06 and 24.84 mg L(-1), respectively. According to the Waste Management Act of Iran this waste should be classified as hazardous and should be disposed of in accordance with hazardous waste disposal regulations.

  4. Dangerous triplet: Polycystic ovary syndrome, oral contraceptives and Kounis syndrome.

    PubMed

    Erol, Nurdan; Karaagac, Aysu Turkmen; Kounis, Nicholas G

    2014-12-26

    Polycystic ovary syndrome is characterized by ovulatory dysfunction, androgen excess and polycystic ovaries and is associated with hypertension, diabetes, metabolic syndrome and cardiovascular events. Oral contraceptives constitute first-line treatment, particularly when symptomatic hyperandrogenism is present. However, these drugs are associated with cardiovascular events and hypersensitivity reactions that pose problem in differential diagnosis and therapy. We present a 14 year-old female with polycystic ovary syndrome taking oral contraceptive and suffering from recurrent coronary ischemic attacks with increased eosinophils, and troponin levels suggesting Kounis syndrome.

  5. Use of thermal analysis techniques (TG-DSC) for the characterization of diverse organic municipal waste streams to predict biological stability prior to land application

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Fernandez, Jose M., E-mail: joseman@sas.upenn.edu; Plaza, Cesar; Polo, Alfredo

    2012-01-15

    Highlights: Black-Right-Pointing-Pointer Thermal analysis was used to assess stability and composition of organic matter in three diverse municipal waste streams. Black-Right-Pointing-Pointer Results were compared with C mineralization during 90-day incubation, FTIR and {sup 13}C NMR. Black-Right-Pointing-Pointer Thermal analysis reflected the differences between the organic wastes before and after the incubation. Black-Right-Pointing-Pointer The calculated energy density showed a strong correlation with cumulative respiration. Black-Right-Pointing-Pointer Conventional and thermal methods provide complimentary means of characterizing organic wastes. - Abstract: The use of organic municipal wastes as soil amendments is an increasing practice that can divert significant amounts of waste from landfill, and providesmore » a potential source of nutrients and organic matter to ameliorate degraded soils. Due to the high heterogeneity of organic municipal waste streams, it is difficult to rapidly and cost-effectively establish their suitability as soil amendments using a single method. Thermal analysis has been proposed as an evolving technique to assess the stability and composition of the organic matter present in these wastes. In this study, three different organic municipal waste streams (i.e., a municipal waste compost (MC), a composted sewage sludge (CS) and a thermally dried sewage sludge (TS)) were characterized using conventional and thermal methods. The conventional methods used to test organic matter stability included laboratory incubation with measurement of respired C, and spectroscopic methods to characterize chemical composition. Carbon mineralization was measured during a 90-day incubation, and samples before and after incubation were analyzed by chemical (elemental analysis) and spectroscopic (infrared and nuclear magnetic resonance) methods. Results were compared with those obtained by thermogravimetry (TG) and differential scanning calorimetry (DSC

  6. Municipal solid waste characterization and its assessment for potential methane generation: a case study.

    PubMed

    Mor, Suman; Ravindra, Khaiwal; De Visscher, Alex; Dahiya, R P; Chandra, A

    2006-12-01

    There has been a significant increase in municipal solid waste (MSW) generation in India during the last few decades and its management has become a major issue because the poor waste management practices affect the health and amenity of the cities. In the present study, various physico-chemical parameters of the MSW were analyzed to characterize the waste dumped at Gazipur landfill site in Delhi, India, which shows that it contains a high fraction of degradable organic components. The decomposition of organic components produces methane, a significant contributor to global warming. Based on the waste composition, waste age and the total amount dumped, a first-order decay model (FOD) was applied to estimate the methane generation potential of the Gazipur landfill site, which yields an estimate of 15.3 Gg/year. This value accounts to about 1-3% of existing Indian landfill methane emission estimates. Based on the investigation of Gazipur landfill, we estimate Indian landfill methane emissions at 1.25 Tg/year or 1.68 Tg/year of methane generation potential. These values are within the range of existing estimates. A comparison of FOD with a recently proposed triangular model was also performed and it shows that both models can be used for the estimation of methane generation. However, the decrease of the emission after closure is more gradual in the case of the first-order model, leading to larger gas production predictions after more than 10 years of closure. The regional and global implications of national landfill methane emission are also discussed.

  7. Ceramics in nuclear waste management

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Chikalla, T D; Mendel, J E

    1979-05-01

    Seventy-three papers are included, arranged under the following section headings: national programs for the disposal of radioactive wastes, waste from stability and characterization, glass processing, ceramic processing, ceramic and glass processing, leaching of waste materials, properties of nuclear waste forms, and immobilization of special radioactive wastes. Separate abstracts were prepared for all the papers. (DLC)

  8. Gorlin-goltz syndrome.

    PubMed

    Pandeshwar, Padma; Jayanthi, K; Mahesh, D

    2012-01-01

    The Gorlin-Goltz syndrome (GGS) (the nevoid basal cell carcinoma syndrome-NBCCS) is a rare autosomal dominant syndrome caused due to mutations in the PTCH (patched) gene found on chromosome arm 9q. The syndrome, characterized by increased predisposition to develop basal cell carcinoma and associated multiorgan anomalies, has a high level of penetrance and variable expressiveness. GGS is a multidisciplinary problem, early diagnosis of which allows introduction of secondary prophylaxis and following an appropriate treatment to delay the progress of the syndrome. The following report emphasizes the need for awareness of the diagnostic criteria of this syndrome in cases with no typical skin lesions.

  9. Remote-handled/special case TRU waste characterization summary

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Detamore, J.A.

    1984-02-27

    Remote-handled wastes are stored at Los Alamos, Hanford, Oak Ridge, and the Idaho National Engineering Laboratory. The following will be a site by site discussion of RH waste handling, placement, and container data. This will be followed by a series of data tables that were compiled in the TRU Waste Systems Office. These tables are a compendium of data that is the most up to date and accurate data available today. 2 figures, 10 tables.

  10. Characterization of carbon silica hybrid fillers obtained by pyrolysis of waste green tires by the STEM–EDX method

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Al-Hartomy, Omar A.; Department of Physics, Faculty of Science, University of Tabuk, Tabuk 71491; Al-Ghamdi, Ahmed A.

    2015-03-15

    Dual phase carbon–silica hybrid fillers obtained by pyrolysis-cum-water vapor of waste green tires have been characterized by energy dispersive X-ray spectroscopy in a scanning transmission electron microscope, silicate analysis, weight analysis, atomic absorption spectroscopy and by inductively coupled plasma–optical emission spectroscopy. The results achieved have shown that the location and distribution of the phases in the carbon silica hybrid fillers as well as their most essential characteristics are influenced by the pyrolysis conditions. The carbon phase of the filler thus obtained is located predominantly in the space among silica aggregates which have already been existing while it has been formedmore » by elastomer destruction in the course of pyrolysis. The presence of ZnS also has been found in the hybrid fillers investigated. - Highlights: • Dual phase fillers obtained by pyrolysis of waste green tires have been characterized. • The STEM–EDX method was used for characterization. • The phase distributions in the fillers are influenced by the pyrolysis conditions.« less

  11. Characterization and Delivery of Hanford High-Level Radioactive Waste Slurry

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Thien, Michael G.; Denslow, Kayte M.; Lee, K. P.

    2014-11-15

    Two primary challenges to characterizing Hanford’s high-level radioactive waste slurry prior to transfer to a treatment facility are the ability to representatively sample million-gallon tanks and to estimate the critical velocity of the complex slurry. Washington River Protection Solutions has successfully demonstrated a sampling concept that minimizes sample errors by collecting multiple sample increments from a sample loop where the mixed tank contents are recirculated. Pacific Northwest National Laboratory has developed and demonstrated an ultrasonic-based Pulse-Echo detection device that is capable of detecting a stationary settled bed of solids in a pipe with flowing slurry. These two concepts are essentialmore » elements of a feed delivery strategy that drives the Hanford clean-up mission.« less

  12. Congenital Zika Syndrome: Characterizing the Pattern of Anomalies for Pediatric Healthcare Providers

    PubMed Central

    Moore, Cynthia A.; Staples, J. Erin; Dobyns, William B.; Pessoa, André; Ventura, Camila V.; da Fonseca, Eduardo Borges; Ribeiro, Erlane Marques; Ventura, Liana O.; Neto, Norberto Nogueira; Arena, J. Fernando; Rasmussen, Sonja A.

    2017-01-01

    Importance Zika virus infection can be passed prenatally from a pregnant woman to her fetus. There is sufficient evidence to conclude that intrauterine Zika virus infection is a cause of microcephaly and serious brain anomalies, but the full spectrum of anomalies has not been delineated. To inform pediatric healthcare providers who may be called upon to evaluate and manage affected infants and children, we review the most recent evidence to better characterize congenital Zika syndrome. Observations We reviewed published reports of congenital anomalies occurring in fetuses or infants with presumed or laboratory-confirmed intrauterine Zika virus infection. Congenital anomalies were considered in the context of the presumed pathogenetic mechanism related to the neurotropic properties of the virus. We conclude that congenital Zika syndrome is a recognizable pattern of structural anomalies and functional disabilities secondary to central and perhaps peripheral nervous system damage. Although many of the components of this syndrome such as cognitive, sensory and motor disabilities are shared by other congenital infections, there are five features that are rarely seen with other congenital infections or are unique to congenital Zika virus infection: severe microcephaly with partially collapsed skull; thin cerebral cortices with subcortical calcifications; macular scarring and focal pigmentary retinal mottling; congenital contractures; and marked early hypertonia and symptoms of extrapyramidal involvement. Conclusions and Relevance Although the full spectrum of adverse reproductive outcomes caused by Zika virus infection is not yet determined, a distinctive phenotype, the congenital Zika syndrome, has emerged. Recognition of this phenotype by healthcare providers for infants and children can help ensure appropriate etiologic evaluation as well as comprehensive clinical investigation to define the range of anomalies in an affected infant and determine essential follow

  13. Nephrocalcinosis as adult presentation of Bartter syndrome type II.

    PubMed

    Huang, L; Luiken, G P M; van Riemsdijk, I C; Petrij, F; Zandbergen, A A M; Dees, A

    2014-02-01

    Bartter syndrome consists a group of rare autosomal-recessive renal tubulopathies characterised by renal salt wasting, hypokalaemic metabolic alkalosis, hypercalciuria and hyperreninaemic hyperaldosteronism. It is classified into five types. Mutations in the KCNJ1 gene (classified as type II) usually cause the neonatal form of Bartter syndrome. We describe an adult patient with a homozygous KCNJ1 mutation resulting in a remarkably mild phenotype of neonatal type Bartter syndrome.

  14. Characterization of Mast Cell Activation Syndrome.

    PubMed

    Afrin, Lawrence B; Self, Sally; Menk, Jeremiah; Lazarchick, John

    2017-03-01

    Mast cell activation syndrome (MCAS), a recently recognized nonneoplastic mast cell disease driving chronic multisystem inflammation and allergy, appears prevalent and thus important. We report the first systematic characterization of a large MCAS population. Demographics, comorbidities, symptoms, family histories, physical examination and laboratory findings were reviewed in 298 retrospective and 115 prospective patients with MCAS. Blood samples from prospective subjects were examined by flow cytometry for clonal mast cell disease and tested for cytokines potentially driving the monocytosis frequent in MCAS. Demographically, white females dominated. Median ages at symptom onset and diagnosis were 9 and 49 years, respectively (range: 0-88 and 16-92, respectively) and median time from symptom onset to diagnosis was 30 years (range: 1-85). Median numbers of comorbidities, symptoms, and family medical issues were 11, 20, and 4, respectively (range: 1-66, 2-84, and 0-33, respectively). Gastroesophageal reflux, fatigue and dermatographism were the most common comorbidity, symptom and examination finding. Abnormalities in routine laboratories were common and diverse but typically modest. The most useful diagnostic markers were heparin, prostaglandin D 2 , histamine and chromogranin A. Flow cytometric and cytokine assessments were unhelpful. Our study highlights MCAS׳s morbidity burden and challenging heterogeneity. Recognition is important given good survival and treatment prospects. Copyright © 2017 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

  15. Feasibility study of 235U and 239Pu characterization in radioactive waste drums using neutron-induced fission delayed gamma rays

    NASA Astrophysics Data System (ADS)

    Nicol, T.; Pérot, B.; Carasco, C.; Brackx, E.; Mariani, A.; Passard, C.; Mauerhofer, E.; Collot, J.

    2016-10-01

    This paper reports a feasibility study of 235U and 239Pu characterization in 225 L bituminized waste drums or 200 L concrete waste drums, by detecting delayed fission gamma rays between the pulses of a deuterium-tritium neutron generator. The delayed gamma yields were first measured with bare samples of 235U and 239Pu in REGAIN, a facility dedicated to the assay of 118 L waste drums by Prompt Gamma Neutron Activation Analysis (PGNAA) at CEA Cadarache, France. Detectability in the waste drums is then assessed using the MCNPX model of MEDINA (Multi Element Detection based on Instrumental Neutron Activation), another PGNAA cell dedicated to 200 L drums at FZJ, Germany. For the bituminized waste drum, performances are severely hampered by the high gamma background due to 137Cs, which requires the use of collimator and shield to avoid electronics saturation, these elements being very penalizing for the detection of the weak delayed gamma signal. However, for lower activity concrete drums, detection limits range from 10 to 290 g of 235U or 239Pu, depending on the delayed gamma rays of interest. These detection limits have been determined by using MCNPX to calculate the delayed gamma useful signal, and by measuring the experimental gamma background in MEDINA with a 200 L concrete drum mock-up. The performances could be significantly improved by using a higher interrogating neutron emission and an optimized experimental setup, which would allow characterizing nuclear materials in a wide range of low and medium activity waste packages.

  16. Isolation and characterization of onion degrading bacteria from onion waste produced in South Buenos Aires province, Argentina.

    PubMed

    Rinland, María Emilia; Gómez, Marisa Anahí

    2015-03-01

    Onion production in Argentina generates a significant amount of waste. Finding an effective method to recycle it is a matter of environmental concern. Among organic waste reuse techniques, anaerobic digestion could be a valuable alternative to current practices. Substrate inoculation with appropriate bacterial strains enhances the rate-limiting step (hydrolysis) of anaerobic digestion of biomass wastes. Selection of indigenous bacteria with the ability to degrade onion waste could be a good approach to find a suitable bioaugmentation or pretreatment agent. We isolated bacterial strains from onion waste in different degradation stages and from different localities. In order to characterize and select the best candidates, we analyzed the growth patterns of the isolates in a medium prepared with onion juice as the main source of nutrients and we evaluated carbon source utilization. Nine strains were selected to test their ability to grow using onion tissue and the five most remarkable ones were identified by 16S rRNA gene sequencing. Strains belonged to the genera Pseudoxanthomonas, Bacillus, Micrococcus and Pseudomonas. Two strains, Bacillus subtilis subsp. subtillis MB2-62 and Pseudomonas poae VE-74 have characteristics that make them promising candidates for bioaugmentation or pretreatment purposes.

  17. Hearing dysfunction in heterozygous Mitf(Mi-wh) /+ mice, a model for Waardenburg syndrome type 2 and Tietz syndrome.

    PubMed

    Ni, Christina; Zhang, Deming; Beyer, Lisa A; Halsey, Karin E; Fukui, Hideto; Raphael, Yehoash; Dolan, David F; Hornyak, Thomas J

    2013-01-01

    The human deafness-pigmentation syndromes, Waardenburg syndrome (WS) type 2a, and Tietz syndrome are characterized by profound deafness but only partial cutaneous pigmentary abnormalities. Both syndromes are caused by mutations in MITF. To illuminate differences between cutaneous and otic melanocytes in these syndromes, their development and survival in heterozygous Microphthalmia-White (Mitf(Mi-wh) /+) mice were studied and hearing function of these mice characterized. Mitf(Mi-wh) /+ mice have a profound hearing deficit, characterized by elevated auditory brainstem response thresholds, reduced distortion product otoacoustic emissions, absent endocochlear potential, loss of outer hair cells, and stria vascularis abnormalities. Mitf(Mi-wh) /+ embryos have fewer melanoblasts during embryonic development than their wild-type littermates. Although cochlear melanocytes are present at birth, they disappear from the Mitf(Mi-wh) /+ cochlea between P1 and P7. These findings may provide insight into the mechanism of melanocyte and hearing loss in human deafness-pigmentation syndromes such as WS and Tietz syndrome and illustrate differences between otic and follicular melanocytes. © 2012 John Wiley & Sons A/S.

  18. Wastewater characterization survey, Victor Valley Wastewater Reclamation Authority and hazardous-waste survey at George AFB, California. Final report

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Binovi, R.D.; Ng, E.K.; Tetla, R.A.

    1987-01-01

    This is a report of a survey of the Victor Wastewater Reclamation Authority Sewerage system, the sewage treatment plant, and effluent from the various operations at George AFB, California. The scope of work included the characterization of the wastewater from George AFB, as well as characterization of effluents from 29 oil/water separators servicing industrial operations on base, flow measurements at three locations on base, a microbiological evaluation of aeration basin foam, bench-scale activated-sludge studies, and a review of results from previous surveys. Recommendations: (1) AFFF (Aqueous Film Forming Foam) should never be discharged to the sewer. (2) Programming for pretreatmentmore » should proceed at selected operations. (3) More waste and wastestream analysis be performed. (4) Upgrade waste accumulation points. (5) Implement an aggressive inspection program for oil/water separators. (6) Cut down on nonessential washing.« less

  19. Poland syndrome.

    PubMed

    Sharma, Chandra Madhur; Kumar, Shrawan; Meghwani, Manoj K; Agrawal, Ravi P

    2014-01-01

    Poland's syndrome is a rare congenital condition, characterized by the absence of the sternal or breastbone portion of the pectoralis major muscle, which may be associated with the absence of nearby musculoskeletal structures. We hereby report an 8-year-old boy with typical features of Poland syndrome, the first documented case from Uttar Pradesh, India.

  20. Greater-than-Class C low-level radioactive waste characterization: Estimated volumes, radionuclide activities, and other characteristics. Revision 1

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Not Available

    1994-09-01

    The Department of Energy`s (DOE`s) planning for the disposal of greater-than-Class C low-level radioactive waste (GTCC LLW) requires characterization of the waste. This report estimates volumes, radionuclide activities, and waste forms of GTCC LLW to the year 2035. It groups the waste into four categories, representative of the type of generator or holder of the waste: Nuclear Utilities, Sealed Sources, DOE-Held, and Other Generator. GTCC LLW includes activated metals (activation hardware from reactor operation and decommissioning), process wastes (i.e., resins, filters, etc.), sealed sources, and other wastes routinely generated by users of radioactive material. Estimates reflect the possible effect thatmore » packaging and concentration averaging may have on the total volume of GTCC LLW. Possible GTCC mixed LLW is also addressed. Nuclear utilities will probably generate the largest future volume of GTCC LLW with 65--83% of the total volume. The other generators will generate 17--23% of the waste volume, while GTCC sealed sources are expected to contribute 1--12%. A legal review of DOE`s obligations indicates that the current DOE-Held wastes described in this report will not require management as GTCC LLW because of the contractual circumstances under which they were accepted for storage. This report concludes that the volume of GTCC LLW should not pose a significant management problem from a scientific or technical standpoint. The projected volume is small enough to indicate that a dedicated GTCC LLW disposal facility may not be justified. Instead, co-disposal with other waste types is being considered as an option.« less

  1. Molecular Characterization of Three Canine Models of Human Rare Bone Diseases: Caffey, van den Ende-Gupta, and Raine Syndromes

    PubMed Central

    Hytönen, Marjo K.; Arumilli, Meharji; Lappalainen, Anu K.; Owczarek-Lipska, Marta; Jagannathan, Vidhya; Hundi, Sruthi; Salmela, Elina; Venta, Patrick; Sarkiala, Eva; Jokinen, Tarja; Gorgas, Daniela; Kere, Juha; Nieminen, Pekka

    2016-01-01

    One to two percent of all children are born with a developmental disorder requiring pediatric hospital admissions. For many such syndromes, the molecular pathogenesis remains poorly characterized. Parallel developmental disorders in other species could provide complementary models for human rare diseases by uncovering new candidate genes, improving the understanding of the molecular mechanisms and opening possibilities for therapeutic trials. We performed various experiments, e.g. combined genome-wide association and next generation sequencing, to investigate the clinico-pathological features and genetic causes of three developmental syndromes in dogs, including craniomandibular osteopathy (CMO), a previously undescribed skeletal syndrome, and dental hypomineralization, for which we identified pathogenic variants in the canine SLC37A2 (truncating splicing enhancer variant), SCARF2 (truncating 2-bp deletion) and FAM20C (missense variant) genes, respectively. CMO is a clinical equivalent to an infantile cortical hyperostosis (Caffey disease), for which SLC37A2 is a new candidate gene. SLC37A2 is a poorly characterized member of a glucose-phosphate transporter family without previous disease associations. It is expressed in many tissues, including cells of the macrophage lineage, e.g. osteoclasts, and suggests a disease mechanism, in which an impaired glucose homeostasis in osteoclasts compromises their function in the developing bone, leading to hyperostosis. Mutations in SCARF2 and FAM20C have been associated with the human van den Ende-Gupta and Raine syndromes that include numerous features similar to the affected dogs. Given the growing interest in the molecular characterization and treatment of human rare diseases, our study presents three novel physiologically relevant models for further research and therapy approaches, while providing the molecular identity for the canine conditions. PMID:27187611

  2. Poland syndrome

    PubMed Central

    Sharma, Chandra Madhur; Kumar, Shrawan; Meghwani, Manoj K.; Agrawal, Ravi P.

    2014-01-01

    Poland's syndrome is a rare congenital condition, characterized by the absence of the sternal or breastbone portion of the pectoralis major muscle, which may be associated with the absence of nearby musculoskeletal structures. We hereby report an 8-year-old boy with typical features of Poland syndrome, the first documented case from Uttar Pradesh, India. PMID:24959021

  3. The short arm deletion syndrome of chromosome 4 (4p- syndrome).

    PubMed

    Zellweger, H; Bardach, J; Bordwell, J; Williams, K

    1975-01-01

    Partial deletion of the short arm of chromosome 4 (4p-) represents another (rare) cause of cleft lip and cleft palate. Further characteristic manifestations of the syndrome (also called Wolf or Wolf-Hirschhorn syndrome) are growth failure, microcephaly, prominent glabella, hypertelorism, beaked nose, poorly differentiated and low set ears, cardiac and renal malformation and hypospadias. Life expectancy is often shortened. The 4p- syndrome has many features in common with another deletion syndrome, the cri-du-chat syndrome, and also with the Smith-Lemli-Opitz syndrome. The latter is a hereditary condition with normal karyotype. The cri-du-chat syndrome is characterized by a peculiar high-pitched, mewing cry and can be differentiated from the Wolf syndrome by the different staining characteristics (banding) of chromosomes 4 and 5.

  4. Genetics Home Reference: uncombable hair syndrome

    MedlinePlus

    ... Twitter Home Health Conditions Uncombable hair syndrome Uncombable hair syndrome Printable PDF Open All Close All Enable ... to view the expand/collapse boxes. Description Uncombable hair syndrome is a condition that is characterized by ...

  5. Genetics Home Reference: Dubin-Johnson syndrome

    MedlinePlus

    ... Twitter Home Health Conditions Dubin-Johnson syndrome Dubin-Johnson syndrome Printable PDF Open All Close All Enable ... to view the expand/collapse boxes. Description Dubin-Johnson syndrome is a condition characterized by jaundice, which ...

  6. Genetics Home Reference: Kaufman oculocerebrofacial syndrome

    MedlinePlus

    ... Facebook Twitter Home Health Conditions Kaufman oculocerebrofacial syndrome Kaufman oculocerebrofacial syndrome Printable PDF Open All Close All ... Javascript to view the expand/collapse boxes. Description Kaufman oculocerebrofacial syndrome is a disorder characterized by eye ...

  7. Genetics Home Reference: Russell-Silver syndrome

    MedlinePlus

    ... Facebook Twitter Home Health Conditions Russell-Silver syndrome Russell-Silver syndrome Printable PDF Open All Close All ... Javascript to view the expand/collapse boxes. Description Russell-Silver syndrome is a growth disorder characterized by ...

  8. [Münchhausen syndrome].

    PubMed

    Robert, J C; Cremniter, D; Lejonc, J L

    1991-04-20

    Münchhausen's syndrome is characterized by fictitious illnesses associated with hospital peregrination, pseudologia fantastica with a mythomanic discourse that includes strongly structured medical elements, passivity and dependance at examinations, and aggressiveness. The whole picture is so typical that the syndrome can easily be recognized. Cases of Münchhausen's syndrome by proxy (Meadow's syndrome) have been reported during the last few years; the condition concerns children suffering from diseases which are entirely due to their parents and can be compared with the battered child syndrome. In terms of nosology, among pathomimias Münchhausen's syndrome figures as a borderline state. Since it is impossible to establish positive relations with these patients, treatment fails in almost every case.

  9. [Gorlin-Goltz syndrome--a case report].

    PubMed

    Debski, Tomasz; Jethon, Józef

    2010-06-01

    The Gorlin-Goltz syndrome (GGS) (the nevoid basal cell carcinoma syndrome-NBCCS) is an autosomal dominant syndrome caused by mutations found on chromosome 9. The syndrome is characterized by increased predisposition to develop a basal cell carcinoma and associated with multiorgan anomalies. To present a case of GGS and explain modern standards of care for patients with this syndrome. Authors report the case of a 36-year-old patient who was admitted to the Plastic Surgery Clinic due to numerous basal cell carcinomas. Previously patient underwent an orthopaedic, neurologic, dermatologic, stomatologic and surgery treatment due to particular anomalies which characterize this syndrome. Comprehensive interview and broadening of the diagnostics enabled to diagnose GGS and to introduce the appropriate treatment. GGS is a multidisciplinary problem and widespread knowledge of this syndrome could accelerate the diagnosis process. Early diagnosis of GGS allows to introduce the secondary prophylaxis and to apply the appropriate treatment to slow the progress of the syndrome.

  10. Pyrolysis of waste animal fats in a fixed-bed reactor: Production and characterization of bio-oil and bio-char

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Ben Hassen-Trabelsi, A., E-mail: aidabenhassen@yahoo.fr; Kraiem, T.; Département de Géologie, Université de Tunis, 2092, Tunis

    Highlights: • Produced bio-fuels (bio-oil and bio-char) from some animal fatty wastes. • Investigated the effects of main parameters on pyrolysis products distribution. • Determined the suitable conditions for the production of the maximum of bio-oil. • Characterized bio-oils and bio-chars obtained from several animal fatty wastes. - Abstract: Several animal (lamb, poultry and swine) fatty wastes were pyrolyzed under nitrogen, in a laboratory scale fixed-bed reactor and the main products (liquid bio-oil, solid bio-char and syngas) were obtained. The purpose of this study is to produce and characterize bio-oil and bio-char obtained from pyrolysis of animal fatty wastes. Themore » maximum production of bio-oil was achieved at a pyrolysis temperature of 500 °C and a heating rate of 5 °C/min. The chemical (GC–MS analyses) and spectroscopic analyses (FTIR analyses) of bio-oil showed that it is a complex mixture consisting of different classes of organic compounds, i.e., hydrocarbons (alkanes, alkenes, cyclic compounds…etc.), carboxylic acids, aldehydes, ketones, esters,…etc. According to fuel properties, produced bio-oils showed good properties, suitable for its use as an engine fuel or as a potential source for synthetic fuels and chemical feedstock. Obtained bio-chars had low carbon content and high ash content which make them unattractive for as renewable source energy.« less

  11. Congenital hypoventilation syndrome and Hirschsprung's disease - Haddad syndrome: A neonatal case presentation.

    PubMed

    Jaiyeola, P; El-Metwally, D; Viscardi, R; Greene, C; Woo, H

    2015-01-01

    Congenital central hypoventilation syndrome (CCHS) is an uncommon cause of apnea in the newborn characterized by the occurrence of apnea predominantly during sleep. Haddad syndrome is CCHS with Hirschsprung's disease. We report a newborn with Haddad syndrome that had a family history of spinal muscular atrophy and discuss aspects of CCHS and important considerations in the evaluation of apnea in the term newborn.

  12. Proteus syndrome*

    PubMed Central

    Rocha, Ritha de Cássia Capelato; Estrella, Mariani Paulino Soriano; do Amaral, Danielle Mechereffe; Barbosa, Angela Marques; de Abreu, Marilda Aparecida Milanez Morgado

    2017-01-01

    Proteus syndrome is a rare syndrome characterized by disproportionate overgrowth of limbs, multiple hamartomas, and vascular malformations. The cerebriform connective tissue nevi, also called cerebriform plantar hyperplasia, are present in most patients, and is the main characteristic of the syndrome. If present, even alone, they can be considered as a pathognomonic sign. This article reports a classic case of Proteus syndrome in a 2-year-old male patient who began to show a discrete asymmetry of the right hemibody in relation to the left one after birth, which increased over the months. He also showed cerebriform plantar hyperplasia and Port-wine stains, among other alterations. PMID:29166516

  13. Economic and environmental characterization of an evolving Li-ion battery waste stream.

    PubMed

    Wang, Xue; Gaustad, Gabrielle; Babbitt, Callie W; Bailey, Chelsea; Ganter, Matthew J; Landi, Brian J

    2014-03-15

    While disposal bans of lithium-ion batteries are gaining in popularity, the infrastructure required to recycle these batteries has not yet fully emerged and the economic motivation for this type of recycling system has not yet been quantified comprehensively. This study combines economic modeling and fundamental material characterization methods to quantify economic trade-offs for lithium ion batteries at their end-of-life. Results show that as chemistries transition from lithium-cobalt based cathodes to less costly chemistries, battery recovery value decreases along with the initial value of the raw materials used. For example, manganese-spinel and iron phosphate cathode batteries have potential material values 73% and 79% less than cobalt cathode batteries, respectively. A majority of the potentially recoverable value resides in the base metals contained in the cathode; this increases disassembly cost and time as this is the last portion of the battery taken apart. A great deal of compositional variability exists, even within the same cathode chemistry, due to differences between manufacturers with coefficient of variation up to 37% for some base metals. Cathode changes over time will result in a heavily co-mingled waste stream, further complicating waste management and recycling processes. These results aim to inform disposal, collection, and take-back policies being proposed currently that affect waste management infrastructure as well as guide future deployment of novel recycling techniques. Copyright © 2014 Elsevier Ltd. All rights reserved.

  14. Mazabraud syndrome associated with McCune-Albright syndrome: a case report and review of the literature.

    PubMed

    Biazzo, Alessio; Di Bernardo, Andrea; Parafioriti, Antonina; Confalonieri, Norberto

    2017-08-23

    Mazabraud syndrome is a very rare benign disorder characterized by the association of monostotic or polyostotic fibrous dysplasia and one or multiple intramuscular myxomas. McCune -Albright syndrome is a rare benign disorder characterized by the association of polyostotic fibrous dysplasia, cafè-au-lait skin pigmentations and endocrine dysfunction, such as precocious puberty, diabetes mellitus, goiter and breast fibroadenomatosis. The association of Mazabraud syndrome and McCune-Albright in the same patient is an anecdotal event. We report the case of a 28-year-old girl with Mazabraud syndrome associated with McCune-Albright syndrome. Our literature review shows that in these patients there is a higher risk of malignant transformation of fibrous dysplasia into osteosarcoma, confirming previous reports. Conversely, no malignant transformation has been reported for myxomas in isolated Mazabraud syndrome or in the association with McCune-Albright syndrome. We conclude that these patients should be scheduled to a close and long-term follow-up.

  15. Gorlin-Goltz Syndrome

    PubMed Central

    Pandeshwar, Padma; Jayanthi, K.; Mahesh, D.

    2012-01-01

    The Gorlin-Goltz syndrome (GGS) (the nevoid basal cell carcinoma syndrome—NBCCS) is a rare autosomal dominant syndrome caused due to mutations in the PTCH (patched) gene found on chromosome arm 9q. The syndrome, characterized by increased predisposition to develop basal cell carcinoma and associated multiorgan anomalies, has a high level of penetrance and variable expressiveness. GGS is a multidisciplinary problem, early diagnosis of which allows introduction of secondary prophylaxis and following an appropriate treatment to delay the progress of the syndrome. The following report emphasizes the need for awareness of the diagnostic criteria of this syndrome in cases with no typical skin lesions. PMID:23082255

  16. Functional and clinical characterization of KCNJ2 mutations associated with LQT7 (Andersen syndrome)

    PubMed Central

    Tristani-Firouzi, Martin; Jensen, Judy L.; Donaldson, Matthew R.; Sansone, Valeria; Meola, Giovanni; Hahn, Angelika; Bendahhou, Said; Kwiecinski, Hubert; Fidzianska, Anna; Plaster, Nikki; Fu, Ying-Hui; Ptacek, Louis J.; Tawil, Rabi

    2002-01-01

    Andersen syndrome (AS) is a rare, inherited disorder characterized by periodic paralysis, long QT (LQT) with ventricular arrhythmias, and skeletal developmental abnormalities. We recently established that AS is caused by mutations in KCNJ2, which encodes the inward rectifier K+ channel Kir2.1. In this report, we characterized the functional consequences of three novel and seven previously described KCNJ2 mutations using a two-microelectrode voltage-clamp technique and correlated the findings with the clinical phenotype. All mutations resulted in loss of function and dominant-negative suppression of Kir2.1 channel function. In mutation carriers, the frequency of periodic paralysis was 64% and dysmorphic features 78%. LQT was the primary cardiac manifestation, present in 71% of KCNJ2 mutation carriers, with ventricular arrhythmias present in 64%. While arrhythmias were common, none of our subjects suffered sudden cardiac death. To gain insight into the mechanism of arrhythmia susceptibility, we simulated the effect of reduced Kir2.1 using a ventricular myocyte model. A reduction in Kir2.1 prolonged the terminal phase of the cardiac action potential, and in the setting of reduced extracellular K+, induced Na+/Ca2+ exchanger–dependent delayed afterdepolarizations and spontaneous arrhythmias. These findings suggest that the substrate for arrhythmia susceptibility in AS is distinct from the other forms of inherited LQT syndrome. PMID:12163457

  17. Waste Generation Overview Refresher, Course 21464

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Simpson, Lewis Edward

    This course, Waste Generation Overview Refresher (COURSE 21464), provides an overview of federal and state waste management regulations, as well as Los Alamos National Laboratory (LANL) policies and procedures for waste management operations. The course covers the activities involved in the cradle-to- grave waste management process and focuses on waste characterization, waste compatibility determinations and classification, and the storage requirements for temporary waste accumulation areas at LANL.

  18. Waste Generated from LMR-AMTEC Reactor Concept

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hasan, Ahmed; Mohamed, Yasser, T.; Mohammaden, Tarek, F.

    2003-02-25

    The candidate Liquid Metal Reactor-Alkali Metal Thermal -to- Electric Converter (LMR-AMTEC) is considered to be the first reactor that would use pure liquid potassium as a secondary coolant, in which potassium vapor aids in the conversion of thermal energy to electric energy. As with all energy production, the thermal generation of electricity produces wastes. These wastes must be managed in ways which safeguard human health and minimize their impact on the environment. Nuclear power is the only energy industry, which takes full responsibility for all its wastes. Based on the candidate design of the LMR-AMTEC components and the coolant types,more » different wastes will be generated from LMR. These wastes must be classified and characterized according to the U.S. Code of Federal Regulation, CFR. This paper defines the waste generation and waste characterization from LMR-AMTEC and reviews the applicable U.S. regulations that govern waste transportation, treatment, storage and final disposition. The wastes generated from LMR-AMTEC are characterized as: (1) mixed waste which is generated from liquid sodium contaminated by fission products and activated corrosion products; (2) hazardous waste which is generated from liquid potassium contaminated by corrosion products; (3) spent nuclear fuel; and (4) low-level radioactive waste which is generated from the packing materials (e.g. activated carbon in cold trap and purification units). The regulations and management of these wastes are summarized in this paper.« less

  19. Tectonic characterization of a potential high-level nuclear waste repository at Yucca Mountain, Nevada

    USGS Publications Warehouse

    Whitney, John W.; O'Leary, Dennis W.

    1993-01-01

    Tectonic characterization of a potential high-level nuclear waste repository at Yucca Mountain, Nevada, is needed to assess seismic and possible volcanic hazards that could affect the site during the preclosure (next 100 years) and the behavior of the hydrologic system during the postclosure (the following 10,000 years) periods. Tectonic characterization is based on assembling mapped geological structures in their chronological order of development and activity, and interpreting their dynamic interrelationships. Addition of mechanistic models and kinematic explanations for the identified tectonic processes provides one or more tectonic models having predictive power. Proper evaluation and application of tectonic models can aid in seismic design and help anticipate probable occurrence of future geologic events of significance to the repository and its design.

  20. Characterization of global loss of imprinting in fetal overgrowth syndrome induced by assisted reproduction

    PubMed Central

    Chen, Zhiyuan; Hagen, Darren E.; Elsik, Christine G.; Ji, Tieming; Morris, Collin James; Moon, Laura Emily; Rivera, Rocío Melissa

    2015-01-01

    Embryos generated with the use of assisted reproductive technologies (ART) can develop overgrowth syndromes. In ruminants, the condition is referred to as large offspring syndrome (LOS) and exhibits variable phenotypic abnormalities including overgrowth, enlarged tongue, and abdominal wall defects. These characteristics recapitulate those observed in the human loss-of-imprinting (LOI) overgrowth syndrome Beckwith–Wiedemann (BWS). We have recently shown LOI at the KCNQ1 locus in LOS, the most common epimutation in BWS. Although the first case of ART-induced LOS was reported in 1995, studies have not yet determined the extent of LOI in this condition. Here, we determined allele-specific expression of imprinted genes previously identified in human and/or mouse in day ∼105 Bos taurus indicus × Bos taurus taurus F1 hybrid control and LOS fetuses using RNAseq. Our analysis allowed us to determine the monoallelic expression of 20 genes in tissues of control fetuses. LOS fetuses displayed variable LOI compared with controls. Biallelic expression of imprinted genes in LOS was associated with tissue-specific hypomethylation of the normally methylated parental allele. In addition, a positive correlation was observed between body weight and the number of biallelically expressed imprinted genes in LOS fetuses. Furthermore, not only was there loss of allele-specific expression of imprinted genes in LOS, but also differential transcript amounts of these genes between control and overgrown fetuses. In summary, we characterized previously unidentified imprinted genes in bovines and identified misregulation of imprinting at multiple loci in LOS. We concluded that LOS is a multilocus LOI syndrome, as is BWS. PMID:25825726

  1. Characterization of global loss of imprinting in fetal overgrowth syndrome induced by assisted reproduction.

    PubMed

    Chen, Zhiyuan; Hagen, Darren E; Elsik, Christine G; Ji, Tieming; Morris, Collin James; Moon, Laura Emily; Rivera, Rocío Melissa

    2015-04-14

    Embryos generated with the use of assisted reproductive technologies (ART) can develop overgrowth syndromes. In ruminants, the condition is referred to as large offspring syndrome (LOS) and exhibits variable phenotypic abnormalities including overgrowth, enlarged tongue, and abdominal wall defects. These characteristics recapitulate those observed in the human loss-of-imprinting (LOI) overgrowth syndrome Beckwith-Wiedemann (BWS). We have recently shown LOI at the KCNQ1 locus in LOS, the most common epimutation in BWS. Although the first case of ART-induced LOS was reported in 1995, studies have not yet determined the extent of LOI in this condition. Here, we determined allele-specific expression of imprinted genes previously identified in human and/or mouse in day ∼105 Bos taurus indicus × Bos taurus taurus F1 hybrid control and LOS fetuses using RNAseq. Our analysis allowed us to determine the monoallelic expression of 20 genes in tissues of control fetuses. LOS fetuses displayed variable LOI compared with controls. Biallelic expression of imprinted genes in LOS was associated with tissue-specific hypomethylation of the normally methylated parental allele. In addition, a positive correlation was observed between body weight and the number of biallelically expressed imprinted genes in LOS fetuses. Furthermore, not only was there loss of allele-specific expression of imprinted genes in LOS, but also differential transcript amounts of these genes between control and overgrown fetuses. In summary, we characterized previously unidentified imprinted genes in bovines and identified misregulation of imprinting at multiple loci in LOS. We concluded that LOS is a multilocus LOI syndrome, as is BWS.

  2. Syndrome in question*

    PubMed Central

    Peruzzo, Juliano; Nazar, Fernanda Luca; Tubone, Mariana Quirino; Escobar, Gabriela Fortes; Cestari, Tania Ferreira

    2015-01-01

    Waardenburg syndrome is an inherited disease characterized by sensorineural hearing loss, pigmentation changes and minor facial malformations. It has four clinical variants. We report the case of a girl who, like her mother, was affected by this syndrome. The diagnosis was made after detection and treatment of deafness. PMID:26375234

  3. [Streptococcal toxic shock syndrome].

    PubMed

    Gvozdenović, Ljiljana; Pasternak, Janko; Milovanović, Stanislav; Ivanov, Dejan; Milić, Sasa

    2010-01-01

    Streptococcal toxic shock syndrome is now recognized as a toxin-mediated, multisystem illness. It is characterized by an early onset of shock with multiorgan failure and continues to be associated with high morbidity and mortality, caused by group A Streptococcus pyogenes. The symptoms for staphylococcal and streptococcal toxic shock syndrome are similar. Streptococcal toxic shock syndrome was not well described until 1993, when children who had suffered from varicella presented roughly 2-4 weeks later with a clinical syndrome highly suggestive of toxic shock syndrome. It is characterized by a sudden onset of fever, chills, vomiting, diarrhea, muscle aches and rash. It can rapidly progress to severe and intractable hypotension and multisystem dysfunction. Almost every organ system can he involved. Complications of streptococcal toxic shock syndrome may include kidney failure, liver failure (and even death. Crystalloids and inotropic agents are used to treat the hypovolemic shock aggressively, with close monitoring of the patient's mean arterial pressure and central venous pressure. An immediate and aggressive management of hypovolemic shock is essential in streptococcal toxic shock syndrome. Targeted antibiotics are indicated: penicillin or a beta-lactam antibiotic is used for treating group A streptococci, and clindamycin has emerged as a key portion of the standard treatment.

  4. Nevoid Basal Cell Carcinoma Syndrome (Gorlin Syndrome).

    PubMed

    Bresler, Scott C; Padwa, Bonnie L; Granter, Scott R

    2016-06-01

    Nevoid basal cell carcinoma syndrome, or basal cell nevus syndrome (Gorlin syndrome), is a rare autosomal dominantly inherited disorder that is characterized by development of basal cell carcinomas from a young age. Other distinguishing clinical features are seen in a majority of patients, and include keratocystic odontogenic tumors (formerly odontogenic keratocysts) as well as dyskeratotic palmar and plantar pitting. A range of skeletal and other developmental abnormalities are also often seen. The disorder is caused by defects in hedgehog signaling which result in constitutive pathway activity and tumor cell proliferation. As sporadic basal cell carcinomas also commonly harbor hedgehog pathway aberrations, therapeutic agents targeting key signaling constituents have been developed and tested against advanced sporadically occurring tumors or syndromic disease, leading in 2013 to FDA approval of the first hedgehog pathway-targeted small molecule, vismodegib. The elucidation of the molecular pathogenesis of nevoid basal cell carcinoma syndrome has resulted in further understanding of the most common human malignancy.

  5. Quantitative, Phenotypical, and Functional Characterization of Cellular Immunity in Children and Adolescents With Down Syndrome.

    PubMed

    Schoch, Justine; Rohrer, Tilman R; Kaestner, Michael; Abdul-Khaliq, Hashim; Gortner, Ludwig; Sester, Urban; Sester, Martina; Schmidt, Tina

    2017-05-15

    Infections and autoimmune disorders are more frequent in Down syndrome, suggesting abnormality of adaptive immunity. Although the role of B cells and antibodies is well characterized, knowledge regarding T cells is limited. Lymphocyte subpopulations of 40 children and adolescents with Down syndrome and 51 controls were quantified, and phenotype and functionality of antigen-specific effector T cells were analyzed with flow cytometry after polyclonal and pathogen-specific stimulation (with varicella-zoster virus [VZV] and cytomegalovirus [CMV]). Results were correlated with immunoglobulin (Ig) G responses. Apart from general alterations in the percentage of lymphocytes, regulatory T cells, and T-helper 1 and 17 cells, all major T-cell subpopulations showed higher expression of the inhibitory receptor PD-1. Polyclonally stimulated effector CD4+ T-cell frequencies were significantly higher in subjects with Down syndrome, whereas their inhibitory receptor expression (programmed cell death 1 [PD-1] and cytotoxic T-lymphocyte antigen 4 [CTLA-4]) was similar to that of controls and cytokine expression profiles were only marginally altered. Pathogen-specific immunity showed age-appropriate levels of endemic infection, with correlation of CMV-specific cellular and humoral immunity in all subjects. Among VZV IgG-positive individuals, a higher percentage of VZV-specific T-cell-positive subjects was seen in those with Down syndrome. Despite alterations in lymphocyte subpopulations, individuals with Down syndrome can mount effector T-cell responses with similar phenotype and functionality as controls but may require higher effector T-cell frequencies to ensure pathogen control. © The Author 2017. Published by Oxford University Press for the Infectious Diseases Society of America. All rights reserved. For permissions, e-mail journals.permissions@oup.com.

  6. Characterization, quantification and management of China's municipal solid waste in spatiotemporal distributions: A review.

    PubMed

    Gu, Binxian; Jiang, Suqin; Wang, Haikun; Wang, Zibo; Jia, Renfu; Yang, Jie; He, Sheng; Cheng, Rong

    2017-03-01

    Municipal Solid Waste (MSW) is a heterogeneous waste stream, which is harmful for human health and the ecological environment if it is not well managed. Based on results from different authors by analyzing the generation, physical components and management of MSW from different cities, this paper presents an overview of the temporal trends and spatial variation characterization of MSW generation and its physical components in China. Total MSW generation has increased from 31,320 thousand tons in 1980 to 178,602 thousand tons in 2014, and MSW generation per capita has also increased from 448.3g to 653.2g. The distribution of MSW generation is mostly concentrated in the coastal southeastern region, as well as large point sources of more than 200 thousand tons per year are mostly distributed in Jiangsu, Zhejiang, Shandong, Hebei and Guangdong provinces. The review shows that the largest proportion of food waste, plastics and paper is 61.2% (54.2-65.9%, 95% CI), 9.8% (7.2-14.0%, 95% CI), 9.6% (6.7-12.3%, 95% CI), respectively, in 2014; the best estimates of other waste were as follows: 3.1% textile, 2.1% glass, 1.1% metal, 1.8% wood and grass, 1.3% rubber and leather, 1.8% ceramic, 2.5% ash, 1.2% hazardous waste, and 4.5% miscellaneous. To better manage China's MSW, several possible and appropriate solutions (e.g., concentrating on key regions, intensifying source separation, promoting green lifestyle, and establishing specialized regulations and policies) should be adopted, which might facilitate the application of China's 13th Five, and identify gaps in our knowledge of MSW management subject. Copyright © 2016 Elsevier Ltd. All rights reserved.

  7. Anthropometric Characterization of Impaired Fetal Growth: Risk Factors for and Prognosis of Newborns With Stunting or Wasting.

    PubMed

    Victora, Cesar G; Villar, José; Barros, Fernando C; Ismail, Leila Cheikh; Chumlea, Cameron; Papageorghiou, Aris T; Bertino, Enrico; Ohuma, Eric O; Lambert, Ann; Carvalho, Maria; Jaffer, Yasmin A; Altman, Douglas G; Noble, Julia A; Gravett, Michael G; Purwar, Manorama; Frederick, Ihunnaya O; Pang, Ruyan; Bhutta, Zulfiqar A; Kennedy, Stephen H

    2015-07-01

    stay, 6.7 [5.5-8.1]; respiratory distress syndrome, 4.0 [3.3-4.9]; transient tachypnea, 2.1 [1.5-2.9]; and no oral feeding for >24 hours, 5.0 [3.9-6.5]). Maternal gestational diabetes mellitus was protective against wasting (0.6; 0.5-0.8) but not against stunting (0.9; 0.7-1.1). Although newborn stunting and wasting share some common determinants, they are distinct phenotypes with their own risk factors and neonatal prognoses. To be consistent with the literature on infant and child nutrition, newborns should be classified using the 2 phenotypes of stunting and wasting. The distinction will help to prioritize preventive interventions and focus the management of fetal undernutrition.

  8. Characterization of symptoms and edema distribution in premenstrual syndrome

    PubMed Central

    Tacani, Pascale Mutti; Ribeiro, Danielle de Oliveira; Barros Guimarães, Barbara Evelyn; Machado, Aline Fernanda Perez; Tacani, Rogério Eduardo

    2015-01-01

    Background Premenstrual syndrome is a group of symptoms linked to the menstrual cycle, and edema is among these symptoms. Physiotherapy is often sought by many patients for the treatment of edema; however, for an adequate prescription of physiotherapeutic procedures, the distribution of edema throughout the body has yet to be characterized. Objective To determine the most frequent symptoms and body regions that present with edema in women during the premenstrual period. Subjects and methods Sixty women with a mean age of 24.6±4.7 years were evaluated during their premenstrual (between days 21 and 28) and menstrual period (between days 1 and 3), and the collected data included body mass, height, biotype (body-fat distribution), face, breast, limb-circumference measurements, and limb-volume estimate, and an adapted version of the Premenstrual Symptoms Screening Tool was used. Statistical analysis was performed using Student’s t-test and the test for equality of two proportions (P≤0.05). Results Premenstrual syndrome was identified in 91.7% of the women, and the most frequent symptoms were irritability (73.33%) and physical symptoms, including swelling (65%), and anxiety (58.3%). Edema was detected in the following areas: facial, epigastric, mammary, umbilical, and pubic, the mid-third of the arms, distal forearm, in both thighs and in the mid-third of the legs determined by circumference measurements, and in both upper and lower limbs, according to the estimated volume. Conclusion In this study population, the most frequent symptoms were irritability, physical symptoms, and anxiety, with distribution of edema in the face, breast, abdomen, pubic area, distal upper limb, and proximal lower limb. PMID:25792857

  9. Molecular cytogenetic characterization of the DiGeorge syndrome region using fluorescence in situ hybridization

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Lindsay, E.A.; Halford, S.; Wadey, R.

    1993-08-01

    DiGeorge syndrome (DGS) is a developmental defect characterized by cardiac defects, facial dysmorphism, and mental retardation. Several studies have described a critical region for DGS at 22q11, within which the majority of DGS patients have deletions. The authors have isolated nine cosmid and three YAC clones using previously described and newly isolated probes that have been shown to be deleted in many DGS patients. Using fluorescence in situ hybridization and digital imaging, they have mapped and ordered these clones relative to the breakpoints of two balanced translocations at 22q11 (one in a DGS patient and one in the unaffected parentmore » of a DGS child). The data indicate that the breakpoint in the unaffected individual distally limits the DGS critical region (defined as the smallest region of overlap), while proximally the region is limited by repeat-rich DNA. The critical region includes the balanced translocation breakpoint of the DGS patient that presumably disrupts the gene causing this syndrome.« less

  10. [Pseudo-Bartter syndrome--2 cases].

    PubMed

    Jóźwiak, Lucyna; Jaroszyński, Andrzej; Baranowicz-Gaszczyk, Iwona; Borowicz, Ewa; Ksiazek, Andrzej

    2010-01-01

    Bartter syndrome represents the group of renal disturbances characterized by hypokaliemia and metabolic alkalosis. Some diseases could display hypokalemic metabolic alkalosis without primary tubular dysfunction. These disorders are called pseudo-Bartter syndrome. In this paper we present 2 cases of pseudo-Bartter syndrome related among to other things to overuse of diuretic drugs.

  11. Characterization of Products from Fast Micropyrolysis of Municipal Solid Waste Biomass

    DOE PAGES

    Klemetsrud, Bethany; Ukaew, Suchada; Thompson, Vicki S.; ...

    2016-09-05

    Biomass feedstock costs remain one of the largest impediments to biofuel production economics. Municipal solid waste (MSW) represents an attractive feedstock with year-round availability, an established collection infrastructure paid for by waste generators, low cost and the potential to be blended with higher cost feedstocks to reduce overall feedstock costs. Paper waste, yard waste and construction and demolition waste (C&D) were examined for their applicability in the pyrolysis conversion pathway. Paper waste consisted of non-recyclable paper such as mixed low grade paper, food and beverage packaging, kitchen paper wastes and coated paper; yard waste consisted of grass clippings and C&Dmore » wastes consisted of engineered wood products obtained from a construction waste landfill. We tested the waste materials for thermochemical conversion potential using a bench scale fast micro-pyrolysis process. Bio-oil yields were the highest for the C&D materials and lowest for the paper waste. The C&D wastes had the highest level of lignin derived compounds (phenolic and cyclics) while the paper waste had higher levels of carbohydrate derived compounds (aldehydes, organic acids, ketones, alcohols and sugar derived). But, the paper material had higher amounts of lignin derived compounds than expected based upon lignin content that is likely due to the presence of polyphenolic resins used in paper processing. The paper and yard wastes had significantly higher levels of ash content than the C&D wastes (14-15% versus 0.5-1.3%), which further correlated to higher levels of alkali and alkaline earth metals, which are known to reduce pyrolysis bio-oil yields. There appeared to be an inverse correlation of both calcium and potassium content with the amount of chromatographic product peaks, indicative of cracking reactions occurring during product formation. Furthermore the effect of acid washing was evaluated for grass clipping and waste paper and the bio-oil yield was increased

  12. Characterization of Products from Fast Micropyrolysis of Municipal Solid Waste Biomass

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Klemetsrud, Bethany; Ukaew, Suchada; Thompson, Vicki S.

    Biomass feedstock costs remain one of the largest impediments to biofuel production economics. Municipal solid waste (MSW) represents an attractive feedstock with year-round availability, an established collection infrastructure paid for by waste generators, low cost and the potential to be blended with higher cost feedstocks to reduce overall feedstock costs. Paper waste, yard waste and construction and demolition waste (C&D) were examined for their applicability in the pyrolysis conversion pathway. Paper waste consisted of non-recyclable paper such as mixed low grade paper, food and beverage packaging, kitchen paper wastes and coated paper; yard waste consisted of grass clippings and C&Dmore » wastes consisted of engineered wood products obtained from a construction waste landfill. We tested the waste materials for thermochemical conversion potential using a bench scale fast micro-pyrolysis process. Bio-oil yields were the highest for the C&D materials and lowest for the paper waste. The C&D wastes had the highest level of lignin derived compounds (phenolic and cyclics) while the paper waste had higher levels of carbohydrate derived compounds (aldehydes, organic acids, ketones, alcohols and sugar derived). But, the paper material had higher amounts of lignin derived compounds than expected based upon lignin content that is likely due to the presence of polyphenolic resins used in paper processing. The paper and yard wastes had significantly higher levels of ash content than the C&D wastes (14-15% versus 0.5-1.3%), which further correlated to higher levels of alkali and alkaline earth metals, which are known to reduce pyrolysis bio-oil yields. There appeared to be an inverse correlation of both calcium and potassium content with the amount of chromatographic product peaks, indicative of cracking reactions occurring during product formation. Furthermore the effect of acid washing was evaluated for grass clipping and waste paper and the bio-oil yield was increased

  13. Elevated systemic galectin-1 levels characterize HELLP syndrome.

    PubMed

    Schnabel, Annegret; Blois, Sandra M; Meint, Peter; Freitag, Nancy; Ernst, Wolfgang; Barrientos, Gabriela; Conrad, Melanie L; Rose, Matthias; Seelbach-Göbel, Birgit

    2016-04-01

    Galectin-1 (gal-1), a member of a family of conserved β-galactoside-binding proteins, has been shown to exert a key role during gestation. Though gal-1 is expressed at higher levels in the placenta from HELLP patients, it is still poorly understood whether systemic gal-1 levels also differ in HELLP patients. In the present study, we evaluated the systemic expression of gal-1, together with the angiogenic factors, placental growth factor (PlGF) and soluble fms-like tyrosine kinase 1 (sFlt-1) in conjunction with HELLP syndrome severity. Systemic levels of gal-1 and sFlt-1 were elevated in patients with both early- and late-onset HELLP syndrome as compared to healthy controls. In contrast, peripheral PlGF levels were decreased in early- and late-onset HELLP. A positive correlation between systemic gal-1 levels and sFlt-1/PlGF ratios was found in early onset HELLP patients. Our results show that HELLP syndrome is associated with increased circulating levels of gal-1; integrating systemic gal-1 measurements into the diagnostic analyses of pregnant women may provide more effective prediction of HELLP syndrome development. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  14. Genetics Home Reference: cold-induced sweating syndrome

    MedlinePlus

    ... Health Conditions Cold-induced sweating syndrome Cold-induced sweating syndrome Printable PDF Open All Close All Enable ... view the expand/collapse boxes. Description Cold-induced sweating syndrome is characterized by problems with regulating body ...

  15. Food waste and the food-energy-water nexus: A review of food waste management alternatives.

    PubMed

    Kibler, Kelly M; Reinhart, Debra; Hawkins, Christopher; Motlagh, Amir Mohaghegh; Wright, James

    2018-04-01

    Throughout the world, much food produced is wasted. The resource impact of producing wasted food is substantial; however, little is known about the energy and water consumed in managing food waste after it has been disposed. Herein, we characterize food waste within the Food-Energy-Water (FEW) nexus and parse the differential FEW effects of producing uneaten food and managing food loss and waste. We find that various food waste management options, such as waste prevention, landfilling, composting, anaerobic digestion, and incineration, present variable pathways for FEW impacts and opportunities. Furthermore, comprehensive sustainable management of food waste will involve varied mechanisms and actors at multiple levels of governance and at the level of individual consumers. To address the complex food waste problem, we therefore propose a "food-waste-systems" approach to optimize resources within the FEW nexus. Such a framework may be applied to devise strategies that, for instance, minimize the amount of edible food that is wasted, foster efficient use of energy and water in the food production process, and simultaneously reduce pollution externalities and create opportunities from recycled energy and nutrients. Characterization of FEW nexus impacts of wasted food, including descriptions of dynamic feedback behaviors, presents a significant research gap and a priority for future work. Large-scale decision making requires more complete understanding of food waste and its management within the FEW nexus, particularly regarding post-disposal impacts related to water. Copyright © 2018 Elsevier Ltd. All rights reserved.

  16. Genetics Home Reference: Griscelli syndrome

    MedlinePlus

    ... Jacob CM, Cristofani L, Casella EB, Voltarelli JC, de Saint-Basile G, Espreafico EM. Griscelli syndrome: characterization ... Asal GT, Tezcan I, Metin A, Lambert N, de Saint Basile G, Sanal O. Griscelli syndrome types ...

  17. Use of thermal analysis techniques (TG-DSC) for the characterization of diverse organic municipal waste streams to predict biological stability prior to land application.

    PubMed

    Fernández, José M; Plaza, César; Polo, Alfredo; Plante, Alain F

    2012-01-01

    The use of organic municipal wastes as soil amendments is an increasing practice that can divert significant amounts of waste from landfill, and provides a potential source of nutrients and organic matter to ameliorate degraded soils. Due to the high heterogeneity of organic municipal waste streams, it is difficult to rapidly and cost-effectively establish their suitability as soil amendments using a single method. Thermal analysis has been proposed as an evolving technique to assess the stability and composition of the organic matter present in these wastes. In this study, three different organic municipal waste streams (i.e., a municipal waste compost (MC), a composted sewage sludge (CS) and a thermally dried sewage sludge (TS)) were characterized using conventional and thermal methods. The conventional methods used to test organic matter stability included laboratory incubation with measurement of respired C, and spectroscopic methods to characterize chemical composition. Carbon mineralization was measured during a 90-day incubation, and samples before and after incubation were analyzed by chemical (elemental analysis) and spectroscopic (infrared and nuclear magnetic resonance) methods. Results were compared with those obtained by thermogravimetry (TG) and differential scanning calorimetry (DSC) techniques. Total amounts of CO(2) respired indicated that the organic matter in the TS was the least stable, while that in the CS was the most stable. This was confirmed by changes detected with the spectroscopic methods in the composition of the organic wastes due to C mineralization. Differences were especially pronounced for TS, which showed a remarkable loss of aliphatic and proteinaceous compounds during the incubation process. TG, and especially DSC analysis, clearly reflected these differences between the three organic wastes before and after the incubation. Furthermore, the calculated energy density, which represents the energy available per unit of organic

  18. Impact assessment of intermediate soil cover on landfill stabilization by characterizing landfilled municipal solid waste.

    PubMed

    Qi, Guangxia; Yue, Dongbei; Liu, Jianguo; Li, Rui; Shi, Xiaochong; He, Liang; Guo, Jingting; Miao, Haomei; Nie, Yongfeng

    2013-10-15

    Waste samples at different depths of a covered municipal solid waste (MSW) landfill in Beijing, China, were excavated and characterized to investigate the impact of intermediate soil cover on waste stabilization. A comparatively high amount of unstable organic matter with 83.3 g kg(-1) dry weight (dw) total organic carbon was detected in the 6-year-old MSW, where toxic inorganic elements containing As, Cd, Cr, Cu, Mn, Ni, Pb, and Zn of 10.1, 0.98, 85.49, 259.7, 530.4, 30.5, 84.0, and 981.7 mg kg(-1) dw, respectively, largely accumulated because of the barrier effect of intermediate soil cover. This accumulation resulted in decreased microbial activities. The intermediate soil cover also caused significant reduction in moisture in MSW under the soil layer, which was as low as 25.9%, and led to inefficient biodegradation of 8- and 10-year-old MSW. Therefore, intermediate soil cover with low permeability seems to act as a barrier that divides a landfill into two landfill cells with different degradation processes by restraining water flow and hazardous matter. Copyright © 2013 Elsevier Ltd. All rights reserved.

  19. Optimization and characterization of gelatin and chitosan extracted from fish and shrimp waste

    NASA Astrophysics Data System (ADS)

    Ait Boulahsen, M.; Chairi, H.; Laglaoui, A.; Arakrak, A.; Zantar, S.; Bakkali, M.; Hassani, M.

    2018-05-01

    Fish and seafood processing industries generate large quantities of waste which are at the origin of several environmental, economic and social problems. However fish waste could contain high value-added substances such as biopolymers. This work focuses on optimizing the gelatin and chitosan extraction from tilapia fish skins and shrimp shells respectively. The gelatin extraction process was optimized using alkali acid treatment prior to thermal hydrolysis. Three different acids were tested at different concentrations. Chitosan was obtained after acid demineralization followed by simultaneous hydrothermal deproteinization and deacetylation by an alkali treatment with different concentrations of HCl and NaOH. The extracted gelatin and chitosan with the highest yield were characterized by determining their main physicochemical properties (Degree of deacetylation, viscosity, pH, moisture and ash content). Results show a significant influence of the acid type and concentration on the extraction yield of gelatin and chitosan, with an average yield of 12.24% and 3.85% respectively. Furthermore, the obtained physicochemical properties of both extracted gelatin and chitosan were within the recommended standard values of the commercial ones used in the industry.

  20. Characterization of radioactive wastes with respect to harmful materials

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Kugel, Karin; Steyer, Stefan; Brennecke, Peter

    In addendum 4 to the license of the German KONRAD repository, which considers mainly radiological aspects, a water law permit was issued in order to prevent the pollution of the near-surface groundwater. The water law permit stipulates limitations for 10 radionuclides and 2 groups of radionuclides as well as mass limitations for 94 substances and materials relevant for water protection issues. Two collateral clauses, i.e. additional requirements imposed by the licensing authority, include demands on the monitoring, registering and balancing of non-radioactive harmful substances and materials /1/. In order to fulfill the requirements of the water law permit the Germanmore » Federal Office for Radiation Protection (BfS) being the operator of the KONRAD repository has developed a concept, which ensures the compliance with all requirements of the water law permit and which provides standardized easy manageable guidance for the waste producers to describe their wastes. On 15 March 2011 the competent water authority, the 'Niedersaechsischer Landesbetrieb fuer Wasserwirtschaft, Kuesten- und Naturschutz' (NLWKN) issued the approval for this concept. Being the most essential part of this concept the procedural method and the developed description of nonradioactive waste package constituents by use of standardized lists of materials and containers is addressed and presented in this paper. The waste producer has to describe his waste package in a standardized way on the base of the lists of materials and containers. For each material in the list a comprehensive description is given comprising the composition, scope of application, quality control measures, thresholds and other data. Each entry in the list has to be approved by NLWKN. The scope of the lists is defined by the waste producers' needs. Using some particular materials as examples, the approval procedure for including materials in the list is described. The procedure of describing the material composition has to

  1. Characterization of industrial waste from a natural gas distribution company and management strategies: a case study of the East Azerbaijan Gas Company (Iran).

    PubMed

    Taghipour, Hassan; Aslhashemi, Ahmad; Assadi, Mohammad; Khodaei, Firoz; Mardangahi, Baharak; Mosaferi, Mohammad; Roshani, Babak

    2012-10-01

    Although a fundamental prerequisite for the successful implementation of any waste management plan is the availability of sufficient and accurate data, there are few available studies regarding the characterization and management of gas distribution company waste (GDCW). This study aimed to characterize the industrial waste generated by the East Azerbaijan Gas Distribution Company (EAGDC) and to present environmental management strategies. The EAGDC serves 57 cities and 821 villages with a total population of more than 2.5 million as well as numerous industrial units. The methodology of this study was based on a checklist of data collected from each zone of the company, site visits (observation), and quantity and quality analysis according to the formal data available from different zones. The results indicate that more than 35 different kinds of industrial solid waste are generated in different industrial installations. The most important types of generated waste include empty barrels (including mercaptans, diesel fuel, deionized waters and oil), faulty gas meters and regulators, a variety of industrial oils, sleeves, filter elements and faulty pipes, valves and fittings. The results indicated that, currently, GDCW is generally handled and disposed of with domestic waste, deposited in companies' installation yards and stores or, sometimes, recycled through non-scientific approaches that can create health risks to the public and the environment, even though most of the GDCW was determined to be recyclable or reusable materials. This study concludes that gas distribution companies must pay more attention to source reduction, recycling and reusing of waste to preserve natural resources, landfill space and the environment.

  2. Thermal degradation of paper industry wastes from a recovered paper mill using TGA. Characterization and gasification test.

    PubMed

    Arenales Rivera, Jorge; Pérez López, Virginia; Ramos Casado, Raquel; Sánchez Hervás, José-María

    2016-01-01

    In this survey, a refuse derived fuel (RDF) was produced from paper industry wastes through a mechanical treatment (MT). The two main wastes generated from a recovered paper mill were rejects and de-inking sludge, which were produced principally in the pulping and de-inking processes, respectively. This work presents raw wastes characterization, fuel preparation and gasification tests performed in a circulating fluidized bed (CFB) gasifier pilot plant. The characterization was carried out by proximate and ultimate analysis. Several blends of pre-conditioned rejects and de-inking sludge were densified by means of pelletizing, studying the energy consumption and its quality properties. Besides, thermal degradation of blends was studied under thermogravimetric analysis (TGA). The experimental runs were made from 30 to 900°C in nitrogen atmosphere at three heating ranges, β=5, 10 and 20°C/min. Two thermal stages were identified during the thermal degradation, which are linked to cellulose and plastic degradation. In addition, kinetics parameters were estimated by the application of non-isothermal methods: Kissinger-Akahira-Sunose (KAS), Flynn-Ozawa-Wall (FOW) and Coats and Redfern. The activation energy values were about 140-160 kJ/mol and 60-80 kJ/mol for plastic and cellulosic materials, respectively. Regarding waste valorisation, a blend composed of 95% of rejects and 5% of de-inking sludge was selected for gasification tests. The energy consumption during the preparation was recorded and a gasification tests were done to prove the usability of these pellets in a CFB gasifier. The main results were a net calorific value (NCV) of 5 MJ/Nm(3) and a total tar content of 11.44 g/Nm(3) at an equivalence ratio (ER) of 0.3. Copyright © 2015 Elsevier Ltd. All rights reserved.

  3. Bartter syndrome associated with nephropathic cystinosis

    PubMed Central

    Sanosi, Ali Al

    2016-01-01

    Bartter syndrome is a rare inherited defect in the thick ascending limb of the loop of Henle. It is characterized by low potassium levels (hypokalaemia), increased blood pH (alkalosis) and normal to low blood pressure. There are three types of Bartter syndrome: neonatal, the classic type and Gitelman syndrome. Nephropathic cystinosis is an autosomal recessive disorder characterized by accumulation of free cystine in lysosomes due to disorder of lysosomal transport that can lead to end stage renal failure within 10 years and multiorgan impairment. We report a 5 year 9 month old child with Bartter syndrome associated with nephropathic cystinosis, hypothyroidism and rickets. Hitherto, only a handful of similar cases have been reported in the literature. PMID:28096565

  4. Bartter syndrome associated with nephropathic cystinosis.

    PubMed

    Osman, Nader M; Sanosi, Ali Al

    2016-01-01

    Bartter syndrome is a rare inherited defect in the thick ascending limb of the loop of Henle. It is characterized by low potassium levels (hypokalaemia), increased blood pH (alkalosis) and normal to low blood pressure. There are three types of Bartter syndrome: neonatal, the classic type and Gitelman syndrome. Nephropathic cystinosis is an autosomal recessive disorder characterized by accumulation of free cystine in lysosomes due to disorder of lysosomal transport that can lead to end stage renal failure within 10 years and multiorgan impairment. We report a 5 year 9 month old child with Bartter syndrome associated with nephropathic cystinosis, hypothyroidism and rickets. Hitherto, only a handful of similar cases have been reported in the literature.

  5. Cardiac involvement in antiphospholipid syndrome associated with Sneddon syndrome: a challenging diagnosis.

    PubMed

    Faustino, Ana; Paiva, Luís; Morgadinho, Ana; Trigo, Emília; Botelho, Ana; Costa, Marco; Leitão-Marques, António

    2014-02-01

    Sneddon syndrome is a rare clinical entity characterized by the association of ischemic cerebrovascular disease and livedo reticularis. The authors report a case of stroke and myocardial infarction in a 39-year-old man with Sneddon syndrome and antiphospholipid syndrome who subsequently met some criteria for systemic lupus erythematosus, highlighting the complexity of cardiovascular involvement in systemic diseases. Copyright © 2013 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  6. The Integrated Waste Tracking System - A Flexible Waste Management Tool

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Anderson, Robert Stephen

    2001-02-01

    The US Department of Energy (DOE) Idaho National Engineering and Environmental Laboratory (INEEL) has fully embraced a flexible, computer-based tool to help increase waste management efficiency and integrate multiple operational functions from waste generation through waste disposition while reducing cost. The Integrated Waste Tracking System (IWTS)provides comprehensive information management for containerized waste during generation,storage, treatment, transport, and disposal. The IWTS provides all information necessary for facilities to properly manage and demonstrate regulatory compliance. As a platformindependent, client-server and Web-based inventory and compliance system, the IWTS has proven to be a successful tracking, characterization, compliance, and reporting tool that meets themore » needs of both operations and management while providing a high level of management flexibility.« less

  7. Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome)

    PubMed Central

    Kiran, N. K.; Tilak Raj, T. N.; Mukunda, K. S.; Rajashekar Reddy, V.

    2012-01-01

    The Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by odontogenic keratocysts in the jaw, multiple basal cell nevi carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the odontogenic keratocysts are usually one of the first manifestations of the syndrome. This case report presents a patient diagnosed as NBCCS by clinical, radiographic and histological findings in a 13-year-old boy. This paper highlights the importance of early diagnosis of NBCCS which can help in preventive multidisciplinary approach to provide a better prognosis for the patient. PMID:23633824

  8. Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome).

    PubMed

    Kiran, N K; Tilak Raj, T N; Mukunda, K S; Rajashekar Reddy, V

    2012-10-01

    The Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by odontogenic keratocysts in the jaw, multiple basal cell nevi carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the odontogenic keratocysts are usually one of the first manifestations of the syndrome. This case report presents a patient diagnosed as NBCCS by clinical, radiographic and histological findings in a 13-year-old boy. This paper highlights the importance of early diagnosis of NBCCS which can help in preventive multidisciplinary approach to provide a better prognosis for the patient.

  9. Syndrome by Any Other Name. . .

    ERIC Educational Resources Information Center

    Bowers, Drew

    2008-01-01

    The word "syndrome" is one of those words that has slipped into one's vocabulary with few realizing what exactly it means or all the implications it carries. The word "syndrome" can be defined as "a group of signs and symptoms that occur together and characterize a particular abnormality or condition." Typically, a syndrome will be defined by…

  10. Genetics Home Reference: Senior-Løken syndrome

    MedlinePlus

    ... Facebook Twitter Home Health Conditions Senior-Løken syndrome Senior-Løken syndrome Printable PDF Open All Close All ... Javascript to view the expand/collapse boxes. Description Senior-Løken syndrome is a rare disorder characterized by ...

  11. Characterization and quantification of corticosteroid-binding globulin in a southern toad, Bufo terrestris, exposed to coal-combustion-waste

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Ward, C.K.; Fontes, C.; Breuner, C.W.

    2007-05-15

    Corticosteroid-binding globulin (CBG) is a plasma protein that binds corticosterone and may regulate access of hormone to tissues. The role of CBG during a stress response is not clear. In this study, southern toads, Bufo terrestris, were exposed to a chronic pollutant (coal-combustion-waste), to determine changes in CBG and free corticosterone levels. Since toads exposed to chronic pollutants in previous studies did not exhibit the predicted changes in metabolic rate and mass, but did experience a significant elevation in total corticosterone, we hypothesized that CBG would likewise increase and thus, mitigate the effects of a chronic (i.e. 2 months) pollutantmore » stressor. To conduct this study, we first characterized the properties of CBG in southern toads. After characterization, we monitored the changes in CBG, total corticosterone, and free corticosterone in male toads that were exposed to either coal-combustion-waste or control conditions. CBG increased in all groups throughout the experiment. Total corticosterone, on the other hand, was only significantly elevated at four weeks of exposure to coal-combustion-waste. The increase in CBG did not parallel the increase in total corticosterone; as a result, free corticosterone levels were not buffered by CBG, but showed a peak at four weeks similar to total corticosterone. This finding indicates that, in this species, CBG may not provide a protective mechanism during long-term pollution exposure.« less

  12. Gorlin-goltz syndrome.

    PubMed

    Mehta, Dn; Raval, N; Patadiya, H; Tarsariya, V

    2014-03-01

    The Gorlin-Goltz syndrome (GGS) (the nevoid basal cell carcinoma syndrome) is a rare autosomal dominant syndrome caused due to mutations in the patched gene found on chromosome arm 9 q. It shows high penetrance and variable expressivity; is characterized by basal cell carcinomas, odontogenic keratocysts, palmar and/or plantar pits and ectopic calcifications of the falx cerebri. Until date, very few cases of GGS have been reported in India. Early diagnosis and treatment as well as genetic counseling are essential for this syndrome. A rare case report of a patient with characteristic features of GGS diagnosed at a rural dental college of Gujarat, India is presented here. This case report draws attention of the valuable role of dentist in diagnosis and early management of this syndrome.

  13. The Capgras syndrome in paranoid schizophrenia.

    PubMed

    Silva, J A; Leong, G B

    1992-01-01

    Capgras syndrome is characterized by a delusion of impostors who are thought to be physically similar but psychologically distinct from the misidentified person. This syndrome is generally thought to be relatively rare. Most of our knowledge about Capgras syndrome derives from single case studies and small series of cases usually from diagnostically heterogeneous groups. In this article, a series of 31 patients suffering from both paranoid schizophrenia and Capgras syndrome is described. Issues pertaining to the phenomenology of Capgras syndrome, the possible relation between Capgras syndrome and other delusional misidentification syndromes, and a neurobiological hypothesis aimed at explaining Capgras syndrome are discussed.

  14. WIPP Remote-Handled TRU Waste Program Update

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Most, W.; Kehrman, B.

    2006-07-01

    There are two major regulatory approval milestones necessary in order to commence disposal operations for remote-handled transuranic (RH TRU) waste at the Waste Isolation Pilot Plant (WIPP)-the RH TRU hazardous waste permit modification request [1] and the radiological characterization plan [2]. One of those milestones has been achieved. The US Environmental Protection Agency (EPA) issued its final decision to approve the Department of Energy's (DOE) RH TRU radiological characterization plan along with the RH TRU Waste Characterization Program Implementation Plan [3], on March 26, 2004. The RH TRU hazardous waste permit modification request still awaits agency approval. In EPA's decisionmore » to approve the DOE's RH TRU radiological characterization plan, the EPA also set forth the process for approving site-specific RH TRU waste characterization programs. Included in the March 29, 2005, RH TRU second Notice of Deficiency [4] (NOD) on the Class 3 Permit Modification Request for RH TRU Waste, the New Mexico Environment Department (NMED) requested that the Permittees combine their responses for the RH TRU Waste NOD with the Section 311 permit modification request NOD. The Combined Response Document was submitted April 28, 2005 [5]. Another NOD [6] was issued by the NMED on September 1, 2005, to clarify the Permittees' proposal and submit these clarifications to the administrative record. Combining both the chap. 311 [7] and RH TRU waste permit modification requests allows for both the regulator and Permittees to expedite action on the modification requests. The Combined Response Document preserves human resources and costs by having only one administrative process for both modification requests. Facility readiness requirements of the RH TRU waste final permit [8] must be implemented to declare that the WIPP is ready to receive RH TRU waste for storage and disposal. To demonstrate readiness, the WIPP is preparing for an Operational Readiness Review (ORR) of the RH

  15. De novo mutations in NALCN cause a syndrome characterized by congenital contractures of the limbs and face, hypotonia, and developmental delay.

    PubMed

    Chong, Jessica X; McMillin, Margaret J; Shively, Kathryn M; Beck, Anita E; Marvin, Colby T; Armenteros, Jose R; Buckingham, Kati J; Nkinsi, Naomi T; Boyle, Evan A; Berry, Margaret N; Bocian, Maureen; Foulds, Nicola; Uzielli, Maria Luisa Giovannucci; Haldeman-Englert, Chad; Hennekam, Raoul C M; Kaplan, Paige; Kline, Antonie D; Mercer, Catherine L; Nowaczyk, Malgorzata J M; Klein Wassink-Ruiter, Jolien S; McPherson, Elizabeth W; Moreno, Regina A; Scheuerle, Angela E; Shashi, Vandana; Stevens, Cathy A; Carey, John C; Monteil, Arnaud; Lory, Philippe; Tabor, Holly K; Smith, Joshua D; Shendure, Jay; Nickerson, Deborah A; Bamshad, Michael J

    2015-03-05

    Freeman-Sheldon syndrome, or distal arthrogryposis type 2A (DA2A), is an autosomal-dominant condition caused by mutations in MYH3 and characterized by multiple congenital contractures of the face and limbs and normal cognitive development. We identified a subset of five individuals who had been putatively diagnosed with "DA2A with severe neurological abnormalities" and for whom congenital contractures of the limbs and face, hypotonia, and global developmental delay had resulted in early death in three cases; this is a unique condition that we now refer to as CLIFAHDD syndrome. Exome sequencing identified missense mutations in the sodium leak channel, non-selective (NALCN) in four families affected by CLIFAHDD syndrome. We used molecular-inversion probes to screen for NALCN in a cohort of 202 distal arthrogryposis (DA)-affected individuals as well as concurrent exome sequencing of six other DA-affected individuals, thus revealing NALCN mutations in ten additional families with "atypical" forms of DA. All 14 mutations were missense variants predicted to alter amino acid residues in or near the S5 and S6 pore-forming segments of NALCN, highlighting the functional importance of these segments. In vitro functional studies demonstrated that NALCN alterations nearly abolished the expression of wild-type NALCN, suggesting that alterations that cause CLIFAHDD syndrome have a dominant-negative effect. In contrast, homozygosity for mutations in other regions of NALCN has been reported in three families affected by an autosomal-recessive condition characterized mainly by hypotonia and severe intellectual disability. Accordingly, mutations in NALCN can cause either a recessive or dominant condition characterized by varied though overlapping phenotypic features, perhaps based on the type of mutation and affected protein domain(s). Copyright © 2015 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  16. Characterization of Incorporation the Glass Waste in Adhesive Mortar

    NASA Astrophysics Data System (ADS)

    Santos, D. P.; Azevedo, A. R. G.; Hespanhol, R. L.; Alexandre, J.

    Ehe search for reuse generated waste in urban centers, intending to preserve natural resources, has remained fairly constant, both in context of preventing exploitation of resources as the emplacement of waste on the environment. Glass waste glass created a serious environmental problem, mainly because of inconsistency of its flows. Ehe use of this product as a mineral additive, finely ground, cement replacement and aggregate is a promising direction for recycling. This work aims to study the influence of glass waste from cutting process in adhesive mortar, replacing part of cement. Ehe glass powder is used replacing Portland cement at 10, 15 and 20% by mass. Ehe produced mortars will be evaluated its performance in fresh and hardened states through tests performed in laboratory. Ehe selected feature is indicated by producers of additive and researchers to present good results when used as adhesive mortar.

  17. Synthesis and characterization of carboxymethyl cellulose from office waste paper: a greener approach towards waste management.

    PubMed

    Joshi, Gyanesh; Naithani, Sanjay; Varshney, V K; Bisht, Surendra S; Rana, Vikas; Gupta, P K

    2015-04-01

    In the present study, functionalization of mixed office waste (MOW) paper has been carried out to synthesize carboxymethyl cellulose, a most widely used product for various applications. MOW was pulped and deinked prior to carboxymethylation. The deinked pulp yield was 80.62 ± 2.0% with 72.30 ± 1.50% deinkability factor. The deinked pulp was converted to CMC by alkalization followed by etherification using NaOH and ClCH2COONa respectively, in an alcoholic medium. Maximum degree of substitution (DS) (1.07) of prepared CMC was achieved at 50 °C with 0.094 M and 0.108 M concentrations of NaOH and ClCH2COONa respectively for 3h reaction time. The rheological characteristics of 1-3% aqueous solution of optimized CMC product showed the non-Newtonian pseudoplastic behavior. Fourier transform infra red (FTIR), nuclear magnetic resonance (NMR) and scanning electron microscope (SEM) study were used to characterize the CMC product. Copyright © 2014 Elsevier Ltd. All rights reserved.

  18. Molecular and clinical characterization of a patient with a chromosome 4p deletion, Wolf-Hirschhorn syndrome, and congenital glaucoma.

    PubMed

    Finzi, S; Pinto, C F; Wiggs, J L

    2001-03-01

    Wolf-Hirschhorn syndrome is a developmental disorder associated with hemizygous deletion of the distal short arm of chromosome 4. We have identified a patient affected with Wolf-Hirschhorn syndrome and early onset glaucoma. Five other patients with Wolf-Hirschhorn syndrome and early onset glaucoma or ocular anomalies associated with early onset glaucoma have been previously described, suggesting that the association with Wolf-Hirschhorn syndrome is not coincidental. The infrequent association of early onset glaucoma suggests that the chromosomal region commonly deleted in Wolf-Hirschhorn patients does not contain genes responsible for early onset glaucoma. In this study, we performed a molecular characterization of the deleted chromosome 4 to determine the extent of the deletion in an attempt to begin to identify the chromosomal region responsible for the associated glaucoma. Using microsatellite repeat markers located on 4p, we determined that the deletion spanned a 60-cM region including the minimal Wolf-Hirschhorn region. The proximal breakpoint occurred between markers D4S3045 and D4S2974. These results support the hypothesis that patients with Wolf-Hirschhorn syndrome and early onset glaucoma may have large deletions of 4p that include a gene(s) that may be responsible for a dominant form of congenital glaucoma.

  19. Fast characterization of solid organic waste content with near infrared spectroscopy in anaerobic digestion.

    PubMed

    Charnier, Cyrille; Latrille, Eric; Jimenez, Julie; Lemoine, Margaux; Boulet, Jean-Claude; Miroux, Jérémie; Steyer, Jean-Philippe

    2017-01-01

    The development of anaerobic digestion involves both co-digestion of solid wastes and optimization of the feeding recipe. Within this context, substrate characterisation is an essential issue. Although it is widely used, the biochemical methane potential is not sufficient to optimize the operation of anaerobic digestion plants. Indeed the biochemical composition in carbohydrates, lipids, proteins and the chemical oxygen demand of the inputs are key parameters for the optimisation of process performances. Here we used near infrared spectroscopy as a robust and less-time consuming tool to predict the solid waste content in carbohydrates, lipids and nitrogen, and the chemical oxygen demand. We built a Partial Least Square regression model with 295 samples and validated it with an independent set of 46 samples across a wide range of solid wastes found in anaerobic digestion units. The standard errors of cross-validation were 90mgO 2 ⋅gTS -1 carbohydrates, 2.5∗10 -2 g⋅gTS -1 lipids, 7.2∗10 -3 g⋅gTS -1 nitrogen and 99mgO 2 ⋅gTS -1 chemical oxygen demand. The standard errors of prediction were 53mgO 2 ⋅gTS -1 carbohydrates, 3.2∗10 -2 g⋅gTS -1 lipids, 8.6∗10 -3 g⋅gTS -1 nitrogen and 83mgO 2 ⋅gTS -1 chemical oxygen demand. These results show that near infrared spectroscopy is a new fast and cost-efficient way to characterize solid wastes content and improve their anaerobic digestion monitoring. Copyright © 2016 Elsevier Ltd. All rights reserved.

  20. Qualitative and quantitative metals liberation assessment for characterization of various waste printed circuit boards for recycling.

    PubMed

    Priya, Anshu; Hait, Subrata

    2017-12-01

    Metals liberation and composition are decisive attributes in characterization of e-waste for metal recycling. Though end-of-life printed circuit board (PCB) is an integral part of e-waste as secondary resource reservoir, yet no standardized procedure exists for metals liberation and dissolution for its characterization. Thus, the paper aims at assessment of metals liberation upon comminution employing scanning electron microscopy with energy dispersive X-ray spectroscopy (SEM-EDS) followed by comparative assessment of the existing United States Environmental Protection Agency (USEPA) digestion procedures, viz., USEPA 3050B, USEPA 3051A, and USEPA 3052, in effective dissolution of metals from comminuted particles of waste PCBs of computer, laptop, mobile phone, and television. Effect of comminution and digestion conditions was assessed to have significant role in metal liberation and dissolution from PCBs. The SEM-EDS analysis demonstrated partial release of metals from the silica matrix of PCBs. The USEPA digestion methods showed statistically significant (P < 0.05) difference with greater dissolution of metals complexed to PCB matrix by the USEPA 3052 method owing to use of strong acid like hydrofluoric acid. Base metals like Cu and Zn and toxic metals such as Pb and Cd were present in abundance in PCBs and in general exceeded the total threshold limit concentration (TTLC). The maximum contents of Cu (20.13 ± 0.04 wt.%) and Zn (1.89 ± 0.05 wt.%) in laptop PCBs, Pb (2.26 ± 0.08 wt.%) in TV PCBs, and Cd (0.0812 ± 0.0008 wt.%) in computer PCBs were observed.

  1. Reproduction of post-weaning multi-systemic wasting syndrome in an animal disease model as a tool for vaccine testing under controlled conditions.

    PubMed

    McKillen, John; McNair, Irene; Lagan, Paula; McKay, Karen; McClintock, Julie; Casement, Veronica; Charreyre, Catherine; Allan, Gordon

    2016-04-01

    Snatch farrowed, colostrum deprived piglets were inoculated with different combinations of porcine circovirus 2, porcine parvovirus and Erysipelothrix rhusiopathiae candidate vaccines. 10 piglets were mock-vaccinated. Following virus challenge with a combined porcine circovirus 2/porcine parvovirus inoculum, all animals were monitored and samples taken for serology, immunohistochemistry and qPCR. At 24 dpc all non-vaccinated animals remaining were exhibiting signs of post-weaning multi-systemic wasting syndrome which was confirmed by laboratory analysis. Details of the study, analysis of samples and performance of the candidate vaccines are described. Copyright © 2016 Elsevier Ltd. All rights reserved.

  2. A U-bearing composite waste form for electrochemical processing wastes

    NASA Astrophysics Data System (ADS)

    Chen, X.; Ebert, W. L.; Indacochea, J. E.

    2018-04-01

    Metallic/ceramic composite waste forms are being developed to immobilize combined metallic and oxide waste streams generated during electrochemical recycling of used nuclear fuel. Composites were made for corrosion testing by reacting HT9 steel to represent fuel cladding, Zr and Mo to simulate metallic fuel waste, and a mixture of ZrO2, Nd2O3, and UO2 to represent oxide wastes. More than half of the added UO2 was reduced to metal and formed Fe-Zr-U intermetallics and most of the remaining UO2 and all of the Nd2O3 reacted to form zirconates. Fe-Cr-Mo intermetallics were also formed. Microstructure characterization of the intermetallic and ceramic phases that were generated and tests conducted to evaluate their corrosion behaviors indicate composite waste forms can accommodate both metallic and oxidized waste streams in durable host phases.

  3. Radiological Characterization Technical Report on Californium-252 Sealed Source Transuranic Debris Waste for the Off-Site Source Recovery Project at Los Alamos National Laboratory

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Feldman, Alexander

    2014-04-24

    This document describes the development and approach for the radiological characterization of Cf-252 sealed sources for shipment to the Waste Isolation Pilot Plant. The report combines information on the nuclear material content of each individual source (mass or activity and date of manufacture) with information and data on the radionuclide distributions within the originating nuclear material. This approach allows for complete and accurate characterization of the waste container without the need to take additional measurements. The radionuclide uncertainties, developed from acceptable knowledge (AK) information regarding the source material, are applied to the summed activities in the drum. The AK informationmore » used in the characterization of Cf-252 sealed sources has been qualified by the peer review process, which has been reviewed and accepted by the Environmental Protection Agency.« less

  4. Characterization of food waste-recycling wastewater as biogas feedstock.

    PubMed

    Shin, Seung Gu; Han, Gyuseong; Lee, Joonyeob; Cho, Kyungjin; Jeon, Eun-Jeong; Lee, Changsoo; Hwang, Seokhwan

    2015-11-01

    A set of experiments was carried out to characterize food waste-recycling wastewater (FRW) and to investigate annual and seasonal variations in composition, which is related to the process operation in different seasons. Year-round samplings (n=31) showed that FRW contained high chemical oxygen demand (COD; 148.7±30.5g/L) with carbohydrate (15.6%), protein (19.9%), lipid (41.6%), ethanol (14.0%), and volatile fatty acids (VFAs; 4.2%) as major constituents. FRW was partly (62%) solubilized, possibly due to partial fermentation of organics including carbohydrate. Biodegradable portions of carbohydrate and protein were estimated from acidogenesis test by first-order kinetics: 72.9±4.6% and 37.7±0.3%, respectively. A maximum of 50% of the initial organics were converted to three major VFAs, which were acetate, propionate, and butyrate. The methane potential was estimated as 0.562L CH4/g VSfeed, accounting for 90.0% of the theoretical maximum estimated by elemental analysis. Copyright © 2015 Elsevier Ltd. All rights reserved.

  5. Gorlin-Goltz Syndrome

    PubMed Central

    Mehta, DN; Raval, N; Patadiya, H; Tarsariya, V

    2014-01-01

    The Gorlin-Goltz syndrome (GGS) (the nevoid basal cell carcinoma syndrome) is a rare autosomal dominant syndrome caused due to mutations in the patched gene found on chromosome arm 9 q. It shows high penetrance and variable expressivity; is characterized by basal cell carcinomas, odontogenic keratocysts, palmar and/or plantar pits and ectopic calcifications of the falx cerebri. Until date, very few cases of GGS have been reported in India. Early diagnosis and treatment as well as genetic counseling are essential for this syndrome. A rare case report of a patient with characteristic features of GGS diagnosed at a rural dental college of Gujarat, India is presented here. This case report draws attention of the valuable role of dentist in diagnosis and early management of this syndrome. PMID:24761254

  6. Genetics Home Reference: thiamine-responsive megaloblastic anemia syndrome

    MedlinePlus

    ... Thiamine-responsive megaloblastic anemia syndrome Thiamine-responsive megaloblastic anemia syndrome Printable PDF Open All Close All Enable ... the expand/collapse boxes. Description Thiamine-responsive megaloblastic anemia syndrome is a rare condition characterized by hearing ...

  7. 40 CFR 194.24 - Waste characterization.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... other information and methods. (b) The Department shall submit in the compliance certification... proposed for disposal in the disposal system, WIPP complies with the numeric requirements of § 194.34 and... release. (2) Identify and describe the method(s) used to quantify the limits of waste components...

  8. Challenges to diagnosis of HIV-associated wasting.

    PubMed

    Kotler, Donald

    2004-12-01

    There is a wide variability in the clinical presentation of the protein energy malnutrition often characterized as wasting in patients infected with HIV. Moreover, the clinical presentation has evolved over time. Initially, protein energy malnutrition was characterized by profound weight loss and depletion of body cell mass (BCM). Recently, unrelated concurrent metabolic abnormalities, such as lipodystrophy, may complicate the diagnosis of HIV wasting. Although measures of BCM are relatively accurate for the diagnosis of HIV wasting, the optimal tools for assessing BCM are not necessarily available to the clinician. From the practical standpoint, HIV wasting may be a self-evident diagnosis in advanced stages, but effective interpretation of the early signs of HIV wasting requires familiarity with other complications included in the differential diagnosis.

  9. Synopsis of hydrologic data collected by waste management for characterization of unsaturated transport at Area G

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Vold, E.

    1998-03-01

    Data which have been collected by Los Alamos National Laboratory waste management for the hydrologic characterization of the subsurface at the low level radioactive waste disposal facility, Area G, are reported and discussed briefly. The data includes Unsaturated Flow Apparatus measurements of the unsaturated conductivity in samples from borehole G-5. Analysis compares these values to the predictions from van Genuchten estimates, and the implications for transport and data matching are discussed, especially at the location of the Vapor Phase Notch (VPN). There, evaporation drives a significant vapor flux and the liquid flux cannot be measured accurately by the UFA device.more » Data also include hydrologic characterization of samples from borehole G-5, Area G surface soils, Los Alamos (Cerros de Rio) basalt, Tsankawi and Cerro-Toledo layers, the Vapor Phase Notch (VPN), and additional new samples from the uppermost tuff layer at Area G. Hydraulic properties from these sample groups can be used to supplement the existing data base. The data in this report can be used to improve the accuracy and reduce the uncertainty in future computational modeling of the unsaturated transport at Area G. This report supports the maintenance plan for the Area G Performance Assessment.« less

  10. Development of a simple and sensitive method for the characterization of odorous waste gas emissions by means of solid-phase microextraction (SPME) and GC-MS/olfactometry.

    PubMed

    Kleeberg, K K; Liu, Y; Jans, M; Schlegelmilch, M; Streese, J; Stegmann, R

    2005-01-01

    A solid-phase microextraction (SPME) method has been developed for the extraction of odorous compounds from waste gas. The enriched compounds were characterized by gas chromatography-mass spectrometry (GC-MS) and gas chromatography followed by simultaneous flame ionization detection and olfactometry (GC-FID/O). Five different SPME fiber coatings were tested, and the carboxen/polydimethylsiloxane (CAR/PDMS) fiber showed the highest ability to extract odorous compounds from the waste gas. Furthermore, parameters such as exposure time, desorption temperature, and desorption time have been optimized. The SPME method was successfully used to characterize an odorous waste gas from a fat refinery prior to and after waste gas treatment in order to describe the treatment efficiency of the used laboratory scale plant which consisted of a bioscrubber/biofilter combination and an activated carbon adsorber. The developed method is a valuable approach to provide detailed information of waste gas composition and complements existing methods for the determination of odors. However, caution should be exercised if CAR/PDMS fibers are used for the quantification of odorous compounds in multi-component matrices like waste gas emissions since the relative affinity of each analyte was shown to differ according to the total amount of analytes present in the sample.

  11. Peeling skin syndrome.

    PubMed

    Ilknur, Turna; Demirtaşoğlu, Melda; Akarsu, Sevgi; Lebe, Banu; Güneş, Ali Tahsin; Ozkan, Sebnem

    2006-01-01

    Peeling skin syndrome is a rare disease characterized by widespread painless peeling of the skin. To date, several cases have been described with different clinical features called peeling skin syndrome. Previous reports describe two types (type A and type B) of peeling skin syndrome, both of which show generalized desquamation, sparing palms and soles. We report a 23-year old man who has been classified as neither type A nor type B, and whose history, clinical features and histopathological findings led to a diagnosis of peeling skin syndrome. In addition, the desquamation pattern in our patient was different from that of both types because our case's palms and soles were involved too.

  12. Association of Amelogenesis Imperfecta and Bartter's Syndrome.

    PubMed

    Kumar, A C V; Alekya, V; Krishna, M S V V; Alekya, K; Aruna, M; Reddy, M H K; Sangeetha, B; Ram, R; Kumar, V S

    2017-01-01

    Bartter's syndrome is an autosomal recessive renal tubular disorder characterized by hypokalemia, hypochloremia, metabolic alkalosis, and hyperreninemia with normal blood pressure. Bartter's syndrome is associated with hypercalciuria and nephrocalcinosis. Amelogenesis imperfecta (AI) is a group of hereditary disorders that affect dental enamel. AI could be part of several syndromes. The enamel renal syndrome is the association of AI and nephrocalcinosis. We report two patients of AI with Bartter's syndrome.

  13. [Clinical characteristics of Rett Syndrome].

    PubMed

    Abbes, Zeineb; Bouden, Asma; Halayem, Soumaya; Othman, Sami; Bechir Halayem, Mohamed

    2011-10-01

    Rett Syndrome is a neurodevelopmental disorder, one of the least commonly occurring autism spectrum disorders (ASD),affecting mainly females. To describe features and molecular specificities of Rett syndrome. To identify articles for this review, a Pubmed search was conducted using the following keywords: Rett syndrome, regression,mutation, stereotypes. This syndrome is characterized by cognitive impairment,communication dysfunction, stereotypic movement disorder, and growth failure. It is generally caused by mutations in the MECP2 gene. Rett Syndrome has a prevalence ranging from 10-20 000 females. Specific treatment is not available, but patients need a careful planning for long-term care, with multidisciplinary approaches.

  14. Milestones for Selection, Characterization, and Analysis of the Performance of a Repository for Spent Nuclear Fuel and High-Level Radioactive Waste at Yucca Mountain.

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Rechard, Robert P.

    This report presents a concise history in tabular form of events leading up to site identification in 1978, site selection in 1987, subsequent characterization, and ongoing analysis through 2008 of the performance of a repository for spent nuclear fuel and high-level radioactive waste at Yucca Mountain in southern Nevada. The tabulated events generally occurred in five periods: (1) commitment to mined geologic disposal and identification of sites; (2) site selection and analysis, based on regional geologic characterization through literature and analogous data; (3) feasibility analysis demonstrating calculation procedures and importance of system components, based on rough measures of performance usingmore » surface exploration, waste process knowledge, and general laboratory experiments; (4) suitability analysis demonstrating viability of disposal system, based on environment-specific laboratory experiments, in-situ experiments, and underground disposal system characterization; and (5) compliance analysis, based on completed site-specific characterization. Because the relationship is important to understanding the evolution of the Yucca Mountain Project, the tabulation also shows the interaction between four broad categories of political bodies and government agencies/institutions: (a) technical milestones of the implementing institutions, (b) development of the regulatory requirements and related federal policy in laws and court decisions, (c) Presidential and agency directives and decisions, and (d) critiques of the Yucca Mountain Project and pertinent national and world events related to nuclear energy and radioactive waste.« less

  15. Gorlin-Goltz syndrome.

    PubMed

    Jawa, Deepti Singh; Sircar, Keya; Somani, Rani; Grover, Neeraj; Jaidka, Shipra; Singh, Sanjeet

    2009-07-01

    Gorlin-Goltz syndrome is an autosomal dominant inherited disorder characterized by the presence of multiple odontogenic keratocysts along with various cutaneous, dental, osseous, ophthalmic, neurological, and sex organ abnormalities. Early diagnosis is essential as it may progress to aggressive basal cell carcinomas and neoplasias. Gorlin-Goltz syndrome has rarely been reported from India. We report here one such patient, diagnosed at a rural hospital.

  16. Unmasking Diogenes Syndrome

    PubMed Central

    Nayak, Kashinath; Gopinath, Hima; Kini, Hema; Kumar, Pramod

    2015-01-01

    Diogenes syndrome is characterized by extreme self-neglect, social withdrawal, and poor personal and domestic hygiene. We report a case of Diogenes syndrome presenting with dermatitis passivata. An unusual “mask” of dirt resembling a carapace, onset of neglect after awareness of a breast lump and resumption of personal grooming and social activities after removal of the lump and counseling were seen. PMID:26120158

  17. A U-bearing composite waste form for electrochemical processing wastes

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Chen, X.; Ebert, W. L.; Indacochea, J. E.

    Metallic/ceramic composite waste forms are being developed to immobilize combined metallic and oxide waste streams generated during electrochemical recycling of used nuclear fuel. Composites were made for corrosion testing by reacting HT9 steel to represent fuel cladding, Zr and Mo to simulate metallic fuel waste, and a mixture of ZrO2, Nd2O3, and UO2 to represent oxide wastes. More than half of the added UO2 was reduced to metal and formed Fe-Zr-U intermetallics and most of the remaining UO2 and all of the Nd2O3 reacted to form zirconates. Fe-Cr-Mo intermetallics were also formed. Microstructure characterization of the intermetallic and ceramic phasesmore » that were generated and tests conducted to evaluate their corrosion behaviors indicate composite waste forms can accommodate both metallic and oxidized waste streams in durable host phases. (c) 2018 Elsevier B.V. All rights reserved.« less

  18. Noonan's Syndrome and Autoimmune Thyroiditis

    ERIC Educational Resources Information Center

    Vesterhus, Per; Aarskog, Dagfinn

    1973-01-01

    Thyroid abnormalities were studies in seven boys and three girls, 4- to 17-years-old, with Noonan's syndrome, characterized by mental retardation, ocular anomalies (wide spaced eyes, drooped eye lids, or strabismus), heart lesions, characteristics of Turner's syndrome, and normal karyotypes (chromosome arrangement). (MC)

  19. NEVADA TEST SITE WASTE ACCEPTANCE CRITERIA

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    U.S. DEPARTMENT OF ENERGY, NATIONAL NUCLEAR SECURITY ADMINISTRATION, NEVADA SITE OFFICE

    This document establishes the U. S. Department of Energy, National Nuclear Security Administration Nevada Site Office (NNSA/NSO) waste acceptance criteria (WAC). The WAC provides the requirements, terms, and conditions under which the Nevada Test Site will accept low-level radioactive and mixed waste for disposal. Mixed waste generated within the State of Nevada by NNSA/NSO activities is accepted for disposal. It includes requirements for the generator waste certification program, characterization, traceability, waste form, packaging, and transfer. The criteria apply to radioactive waste received at the Nevada Test Site Area 3 and Area 5 Radioactive Waste Management Site for storage or disposal.

  20. Confirmation that RIPK4 mutations cause not only Bartsocas-Papas syndrome but also CHAND syndrome.

    PubMed

    Busa, Tiffany; Jeraiby, Mohammed; Clémenson, Alix; Manouvrier, Sylvie; Granados, Viviana; Philip, Nicole; Touraine, Renaud

    2017-11-01

    CHAND syndrome is an autosomal recessive disorder characterized by curly hair, ankyloblepharon, and nail dysplasia. Only few patients were reported to date. A homozygous RIPK4 mutation was recently identified by homozygosity mapping and whole exome sequencing in three patients from an expanded consanguineous kindred with a clinical diagnosis of CHAND syndrome. RIPK4 was previously known to be implicated in Bartsocas-Papas syndrome, the autosomal recessive form of popliteal pterygium syndrome. We report here two cases of RIPK4 homozygous mutations in a fetus with severe Bartsocas-Papas syndrome and a patient with CHAND syndrome. The patient with CHAND syndrome harbored the same mutation as the one identified in the family previously reported. We thus confirm the implication of RIPK4 gene in CHAND syndrome in addition to Bartsocas-Papas syndrome and discuss genotype/phenotype correlations. © 2017 Wiley Periodicals, Inc.

  1. Nevada Test Site Waste Acceptance Criteria

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    U. S. Department of Energy, National Nuclear Security Administration Nevada Site Office

    This document establishes the U.S. Department of Energy (DOE), National Nuclear Security Administration Nevada Site Office (NNSA/NSO) waste acceptance criteria (WAC). The WAC provides the requirements, terms, and conditions under which the Nevada Test Site (NTS) will accept low-level radioactive (LLW) and mixed waste (MW) for disposal. It includes requirements for the generator waste certification program, characterization, traceability, waste form, packaging, and transfer. The criteria apply to radioactive waste received at the NTS Area 3 and Area 5 Radioactive Waste Management Complex (RWMC) for storage or disposal.

  2. Volatile organic compound emissions from green waste composting: Characterization and ozone formation

    NASA Astrophysics Data System (ADS)

    Kumar, Anuj; Alaimo, Christopher P.; Horowitz, Robert; Mitloehner, Frank M.; Kleeman, Michael J.; Green, Peter G.

    2011-04-01

    Composting of green waste separated from the disposed solid waste stream reduces biodegradable inputs into landfills, and contributes valuable soil amendments to agriculture. Agencies in regions with severe air quality challenges, such as California's San Joaquin Valley (SJV), have raised concerns about gases emitted during the composting process, which are suspected to contribute to persistent high levels of ground-level ozone formation. The goal of the current study is to thoroughly characterize volatile organic compound (VOC) emissions from green waste compost piles of different ages (fresh tipped piles, 3-6 day old windrows, and 2-3 week old windrows). Multiple sampling and analytical approaches were applied to ensure the detection of most gaseous organic components emitted. More than 100 VOCs were detected and quantified in this study, including aliphatic alkanes, alkenes, aromatic hydrocarbons, biogenic organics, aldehydes, ketones, alcohols, furans, acids, esters, ether, halogenated hydrocarbons and dimethyl disulfide (DMDS). Alcohols were found to be the dominating VOC in the emissions from a compost pile regardless of age, with fluxes ranging from 2.6 to 13.0 mg m -2 min -1 with the highest emissions coming from the younger composting windrows (3-6 days). Average VOC emissions other than alcohols were determined to be 2.3 mg m -2 min -1 from younger windows, which was roughly two times higher than either the fresh tipping pile (1.2 mg m -2 min -1) or the older windrows (1.4 mg m -2 min -1). It was also observed that the older windrows emit a slightly larger proportion of more reactive compounds. Approximately 90% of the total VOCs were found to have maximum incremental reactivity of less than 2. Net ozone formation potential of the emissions was also assessed.

  3. Genetics Home Reference: Rabson-Mendenhall syndrome

    MedlinePlus

    ... syndrome is a rare disorder characterized by severe insulin resistance, a condition in which the body's tissues and ... as energy. In people with Rabson-Mendenhall syndrome , insulin resistance impairs blood sugar regulation and ultimately leads to ...

  4. Gingival fibromatosis with hypertrichosis syndrome: Case series of rare syndrome.

    PubMed

    Balaji, Preetha; Balaji, S M

    2017-01-01

    Gingival fibromatosis with hypertrichosis syndrome is an extremely rare genetic condition characterized by profound overgrowth of hair and gums, as well as other variable features. Gingival fibromatosis is characterized by a large increase in the gingival dimension which extends above the dental crowns, covering them partially or completely. They were found to have a genetic origin, may also occur in isolation or be part of a syndrome, or acquired origin, due to specific drugs administered systemically. Congenital generalized hypertrichosis is a heterogeneous group of diseases with continuing excessive growth of terminal hair without androgenic stimulation. It has informally been called werewolf syndrome because the appearance is similar to that of a werewolf. Various syndromes have been associated with these features such as epilepsy, mental retardation, cardiomegaly, or osteochondrodysplasia. As so far very few cases have been reported in literature, we are reporting a series of three cases with management of the same. The excess gingival tissues, in these cases, were removed by conventional gingivectomy under general anesthesia. The postoperative result was uneventful and the patient's appearance improved significantly. Good esthetic result was achieved to allow patient to practice oral hygiene measures. Though this is not a serious condition clinically, psychosocial trauma cannot be neglected owing to the cosmetic disfigurement it produces.

  5. 40 CFR 194.8 - Approval process for waste shipment from waste generator sites for disposal at the WIPP.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... waste generator site will be conveyed in a letter from the Administrator's authorized representative to... transmittal to the WIPP Waste Information System database of waste characterization data, in accordance with... will be conveyed in a letter from the Administrator's authorized representative to DOE. EPA will not...

  6. 40 CFR 194.8 - Approval process for waste shipment from waste generator sites for disposal at the WIPP.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... waste generator site will be conveyed in a letter from the Administrator's authorized representative to... transmittal to the WIPP Waste Information System database of waste characterization data, in accordance with... will be conveyed in a letter from the Administrator's authorized representative to DOE. EPA will not...

  7. 40 CFR 194.8 - Approval process for waste shipment from waste generator sites for disposal at the WIPP.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... waste generator site will be conveyed in a letter from the Administrator's authorized representative to... transmittal to the WIPP Waste Information System database of waste characterization data, in accordance with... will be conveyed in a letter from the Administrator's authorized representative to DOE. EPA will not...

  8. 40 CFR 194.8 - Approval process for waste shipment from waste generator sites for disposal at the WIPP.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... waste generator site will be conveyed in a letter from the Administrator's authorized representative to... transmittal to the WIPP Waste Information System database of waste characterization data, in accordance with... will be conveyed in a letter from the Administrator's authorized representative to DOE. EPA will not...

  9. Recognizing and preventing refeeding syndrome.

    PubMed

    Adkins, Susan M

    2009-01-01

    Refeeding syndrome is an uncommon but potentially fatal phenomenon that can occur in patients receiving parenteral, enteral, or oral feedings after a period of sustained malnutrition or starvation. This syndrome is characterized by hypophosphatemia, hypokalemia, and hypomagnesemia. The purpose of this article was to bring an acute awareness of refeeding syndrome to the critical care nurse. The recognition, pathogenesis, clinical manifestations, potential life threatening complications, and treatment are presented.

  10. Quantitative comparison between PGNAA measurements and MCNP calculations in view of the characterization of radioactive wastes in Germany and France

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Mauerhofer, E.; Havenith, A.; Kettler, J.

    The Forschungszentrum Juelich GmbH (FZJ), together with the Aachen University Rheinisch-Westfaelische Technische Hochschule (RWTH) and the French Alternative Energies and Atomic Energy Commission (CEA Cadarache) are involved in a cooperation aiming at characterizing toxic and reactive elements in radioactive waste packages by means of Prompt Gamma Neutron Activation Analysis (PGNAA). The French and German waste management agencies have indeed defined acceptability limits concerning these elements in view of their projected geological repositories. A first measurement campaign was performed in the new Prompt Gamma Neutron Activation Analysis (PGNAA) facility called MEDINA, at FZJ, to assess the capture gamma-ray signatures of somemore » elements of interest in large samples up to waste drums with a volume of 200 liter. MEDINA is the acronym for Multi Element Detection based on Instrumental Neutron Activation. This paper presents MCNP calculations of the MEDINA facility and quantitative comparison between measurement and simulation. Passive gamma-ray spectra acquired with a high purity germanium detector and calibration sources are used to qualify the numerical model of the crystal. Active PGNAA spectra of a sodium chloride sample measured with MEDINA then allow for qualifying the global numerical model of the measurement cell. Chlorine indeed constitutes a usual reference with reliable capture gamma-ray production data. The goal is to characterize the entire simulation protocol (geometrical model, nuclear data, and postprocessing tools) which will be used for current measurement interpretation, extrapolation of the performances to other types of waste packages or other applications, as well as for the study of future PGNAA facilities.« less

  11. Quantitative comparison between PGNAA measurements and MCNP calculations in view of the characterization of radioactive wastes in Germany and France

    NASA Astrophysics Data System (ADS)

    Mauerhofer, E.; Havenith, A.; Carasco, C.; Payan, E.; Kettler, J.; Ma, J. L.; Perot, B.

    2013-04-01

    The Forschungszentrum Jülich GmbH (FZJ), together with the Aachen University Rheinisch-Westfaelische Technische Hochschule (RWTH) and the French Alternative Energies and Atomic Energy Commission (CEA Cadarache) are involved in a cooperation aiming at characterizing toxic and reactive elements in radioactive waste packages by means of Prompt Gamma Neutron Activation Analysis (PGNAA) [1]. The French and German waste management agencies have indeed defined acceptability limits concerning these elements in view of their projected geological repositories. A first measurement campaign was performed in the new Prompt Gamma Neutron Activation Analysis (PGNAA) facility called MEDINA, at FZJ, to assess the capture gamma-ray signatures of some elements of interest in large samples up to waste drums with a volume of 200 liter. MEDINA is the acronym for Multi Element Detection based on Instrumental Neutron Activation. This paper presents MCNP calculations of the MEDINA facility and quantitative comparison between measurement and simulation. Passive gamma-ray spectra acquired with a high purity germanium detector and calibration sources are used to qualify the numerical model of the crystal. Active PGNAA spectra of a sodium chloride sample measured with MEDINA then allow for qualifying the global numerical model of the measurement cell. Chlorine indeed constitutes a usual reference with reliable capture gamma-ray production data. The goal is to characterize the entire simulation protocol (geometrical model, nuclear data, and postprocessing tools) which will be used for current measurement interpretation, extrapolation of the performances to other types of waste packages or other applications, as well as for the study of future PGNAA facilities.

  12. Behavioral and Emotional Disturbance in Individuals with Williams Syndrome.

    ERIC Educational Resources Information Center

    Einfeld, Stewart L.; Tonge, Bruce J.; Florio, Tony

    1997-01-01

    Comparison of behavioral and emotional disturbance in 70 children and adolescents with Williams Syndrome (characterized by mental retardation and short stature) and a control group, found Williams Syndrome subjects were more likely to be diagnosed with a psychiatric disorder characterized by anxiety, hyperactivity, preoccupations, and…

  13. Characterization of Asthma-Chronic Obstructive Pulmonary Disease Overlap Syndrome: A Qualitative Analysis.

    PubMed

    Rodrigue, Claudie; Beauchesne, Marie-France; Mallette, Valérie; Lemière, Catherine; Larivée, Pierre; Blais, Lucie

    2017-06-01

    Approximately 15-20% of patients with chronic obstructive pulmonary disease (COPD) also display characteristics of asthma. In May 2014, the asthma-COPD overlap syndrome (ACOS) was briefly addressed in the Global Initiative for Asthma (GINA) and Global Initiative for Chronic Obstructive Lung Disease (GOLD) strategy documents. We evaluated how pulmonologists diagnose and treat ACOS and how they assess its control. Pulmonologists from two university healthcare centers, having ≥ 1 year experience, treating patients with asthma, COPD, or ACOS, were invited to participate in focus groups. Two focus groups (1 hour duration) were convened with seven and five participants, respectively. According to pulmonologists from both institutions, ACOS is a new name for an existing syndrome rather than a new disease. It is characterized by incomplete reversible airflow limitations and changes in forced expiratory volume in one second over time. The pulmonologists noted that its diagnosis must be based on clinical characteristics, pulmonary function test results, and clinical intuition. To diagnose ACOS, pulmonologists must rely on their clinical judgment. They also agreed that the treatment of patients with ACOS should target the features of both asthma and COPD. Pulmonologists from both institutions used asthma control criteria to assess ACOS control. A deeper understanding would enable clinicians to establish specific criteria for the diagnosis, treatment, and follow-up of subjects with ACOS.

  14. The mixed low-level waste problem in BE/NWN capsule

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hensley, D.C.

    1999-07-01

    The Boh Environmental, LLC (BE) and Northwest Nuclear, LLC (NWN) program addresses the problem of diminishing capacity in the United States to store mixed waste. A lack of an alternative program has caused the US Department of Energy (DOE) to indefinitely store all of its mixed waste in Resource Conservation and Recovery Act (RCRA) compliant storage facilities. Unfortunately, this capacity is fast approaching the administrative control limit. The combination of unique BE encapsulation and NWN waste characterization technologies provides an effective solution to DOE's mixed-waste dilemma. The BE ARROW-PAK technique encapsulates mixed low-level waste (MLLW) in extra-high molecular weight, high-densitymore » polyethylene, pipe-grade resin cylinders. ARROW-PAK applications include waste treatment, disposal, transportation (per 49 CFR 173), vault encasement, and interim/long-term storage for 100 to 300 yr. One of the first demonstrations of this treatment/storage technique successfully treated 880 mixed-waste debris drums at the DOE Hanford Site in 1997. NWN, deploying the APNea neutron assay technology, provides the screening and characterization capability necessary to ensure that radioactive waste is correctly categorized as either transuranic (TRU) or LLW. MLLW resulting from D and D activities conducted at the Oak Ridge East Tennessee Technology Park will be placed into ARROW-PAK containers following comprehensive characterization of the waste by NWN. The characterized and encapsulated waste will then be shipped to a commercial disposal facility, where the shipments meet all waste acceptance criteria of the disposal facility including treatment criteria.« less

  15. Characterization of food waste generators: a Hawaii case study.

    PubMed

    Okazaki, W K; Turn, S Q; Flachsbart, P G

    2008-12-01

    Information on food waste disposal and on recycling methods and recycled amounts is reported. Data were obtained from a mail and phone survey of all licensed food establishments in Hawaii conducted in 2004 and 2005. Of 8253 licensed food establishments, 5033 completed surveys. It was found that relationships exist between food establishment size (measured by the number of meals served per day or the number of employees) and the amount of food an establishment recycled; establishment type and recycling behavior; and establishment type and amount recycled. The amount of food waste recycled in the state of Hawaii was estimated to be 264,000 L/day and annual food waste generation was estimated to be 336,000 tonnes.

  16. Shah-Waardenburg Syndrome

    PubMed Central

    Mahmoudi, Abdelhalim; Rami, Mohamed; Khattala, Khalid; Elmadi, Aziz; Afifi, My Abderrahmane; Youssef, Bouabdallah

    2013-01-01

    Shah-Waardenburg syndrome (SWS) is a neurocristopathy and is characterized by Hirschsprung's disease (HD), deafness, and depigmentation of hairs, skin, and iris. Is a very rare congenital disorder with variable clinical expression. This report describes a 4-day-old male newborn with Waardenburg's syndrome associated with aganglionosis of the colon and terminal ileum, and review the relevant literature for draws attention to the causal relationship between these two entities. PMID:23565307

  17. Shah-Waardenburg syndrome.

    PubMed

    Mahmoudi, Abdelhalim; Rami, Mohamed; Khattala, Khalid; Elmadi, Aziz; Afifi, My Abderrahmane; Youssef, Bouabdallah

    2013-01-01

    Shah-Waardenburg syndrome (SWS) is a neurocristopathy and is characterized by Hirschsprung's disease (HD), deafness, and depigmentation of hairs, skin, and iris. Is a very rare congenital disorder with variable clinical expression. This report describes a 4-day-old male newborn with Waardenburg's syndrome associated with aganglionosis of the colon and terminal ileum, and review the relevant literature for draws attention to the causal relationship between these two entities.

  18. Etiology of the protein-energy wasting syndrome in chronic kidney disease: a consensus statement from the International Society of Renal Nutrition and Metabolism (ISRNM).

    PubMed

    Carrero, Juan Jesús; Stenvinkel, Peter; Cuppari, Lilian; Ikizler, T Alp; Kalantar-Zadeh, Kamyar; Kaysen, George; Mitch, William E; Price, S Russ; Wanner, Christoph; Wang, Angela Y M; ter Wee, Pieter; Franch, Harold A

    2013-03-01

    Protein-energy wasting (PEW), a term proposed by the International Society of Renal Nutrition and Metabolism (ISRNM), refers to the multiple nutritional and catabolic alterations that occur in chronic kidney disease (CKD) and associate with morbidity and mortality. To increase awareness, identify research needs, and provide the basis for future work to understand therapies and consequences of PEW, ISRNM provides this consensus statement of current knowledge on the etiology of PEW syndrome in CKD. Although insufficient food intake (true undernutrition) due to poor appetite and dietary restrictions contribute, other highly prevalent factors are required for the full syndrome to develop. These include uremia-induced alterations such as increased energy expenditure, persistent inflammation, acidosis, and multiple endocrine disorders that render a state of hypermetabolism leading to excess catabolism of muscle and fat. In addition, comorbid conditions associated with CKD, poor physical activity, frailty, and the dialysis procedure per se further contribute to PEW. Published by Elsevier Inc.

  19. [Tall stature: some classical syndromes].

    PubMed

    Gusbin, N; Verloes, A; Daly, A; Beckers, A

    2006-01-01

    We describe the findings of XYY syndrome in the setting of encountering an individual with this particular condition in the endocrinology clinic. XYY syndrome is a relatively frequent if unfamiliar condition, which is characterized by taller than average height. The extra Y chromosome may play a role in determining the height of these individuals. From this case, a differential diagnosis of tall stature is outlined, in addition to a description of the principal syndromes associated with gigantism. These primarily include Klinefelter syndrome, Marfan syndrome, androgen resistance and growth hormone excess. These various entities are described from the point of view of their symptomatology, biology, pathophysiology and therapeutic characteristics.

  20. Syndrome in question: Gorlin-Goltz syndrome.

    PubMed

    Ribeiro, Pauline Lyrio; Souza, João Basílio de; Abreu, Karina Demoner de; Brezinscki, Marisa Simon; Pignaton, Christine Chambo

    2016-01-01

    The Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an uncommon disorder caused by a mutation in Patched, tumor suppressor gene. It is mainly characterized by numerous early onset basal cell carcinomas, odontogenic cysts of jaw and skeletal abnormalities. Due to the wide clinical spectrum, treatment and management of its modalities are not standardized and should be individualized and monitored by a multidisciplinary team. We report a typical case in a 30-year-old man with multiple basal cell carcinomas, keratotic pits of palmar creases and bifid ribs, with a history of several corrective surgeries for keratocystic odontogenic tumors, among other lesions characteristic of the syndrome.

  1. Pollution characterization of liquid waste of the factory complex Fertial (Arzew, Algeria).

    PubMed

    Redouane, Fares; Mourad, Lounis

    2016-03-01

    The industrial development in Algeria has made a worrying situation for all socioeconomic stakeholders. Indeed, this economic growth is marked in recent years by the establishment of factories and industrial plants that discharge liquid waste in marine shorelines. These releases could destabilize the environmental balance in the coming years, hence the need to support the processing of all sources of pollution. Remediation of such discharges requires several steps of identifying the various pollutants to their treatments. Therefore, the authors conducted this first work of characterization of industrial effluents generated by the mineral fertilizer factory complex Fertial (Arzew), and discussed the pollution load generated by this type of industry. This monitoring would establish a tool for reflection and decision support developed by a management system capable of ensuring effective and sustainable management of effluents from industrial activities of Fertial. The authors conducted this first work of characterization of industrial effluents generated by the mineral fertilizer factory complex Fertial (Arzew), and discussed the pollution load generated by this type of industry. This monitoring would establish a tool for reflection and decision support developed by a management system capable of ensuring effective and sustainable management of effluents from industrial activities of Fertial.

  2. Plant-derived therapeutics for the treatment of metabolic syndrome.

    PubMed

    Graf, Brittany L; Raskin, Ilya; Cefalu, William T; Ribnicky, David M

    2010-10-01

    Metabolic syndrome is defined as a set of coexisting metabolic disorders that increase an individual's likelihood of developing type 2 diabetes, cardiovascular disease and stroke. Medicinal plants, some of which have been used for thousands of years, serve as an excellent source of bioactive compounds for the treatment of metabolic syndrome because they contain a wide range of phytochemicals with diverse metabolic effects. In order for botanicals to be effectively used against metabolic syndrome, however, botanical preparations must be characterized and standardized through the identification of their active compounds and respective modes of action, followed by validation in controlled clinical trials with clearly defined endpoints. This review assesses examples of commonly known and partially characterized botanicals to describe specific considerations for the phytochemical, preclinical and clinical characterization of botanicals associated with metabolic syndrome.

  3. CHARACTERIZATION AND PH/EH-BASED LEACHING TESTS OF MINING WASTES CONTAINING MERCURY

    EPA Science Inventory

    This study was undertaken as a part of developing treatment alternatives for waste materials, primarily waste rock and roaster tailings, from sites contaminated with mercury (Hg) mining wastes. Leaching profiles of waste rock over a range of different pH and oxidation-reduction (...

  4. CHARACTERIZATION AND PH/EH-BASED LEACHING TESTS OF MINING WASTES CONTAINING MERCURY

    EPA Science Inventory

    This study was undertaken as a part of developing treatment alternatives for waste materials, primarily waste rock and roaster tailings, from sites contaminated with Mercury (Hg) mining wastes. Leaching profiles of waste rock over a range of different pH and oxidation-reduction ...

  5. Increased overall cortical connectivity with syndrome specific local decreases suggested by atypical sleep-EEG synchronization in Williams syndrome.

    PubMed

    Gombos, Ferenc; Bódizs, Róbert; Kovács, Ilona

    2017-07-21

    Williams syndrome (7q11.23 microdeletion) is characterized by specific alterations in neurocognitive architecture and functioning, as well as disordered sleep. Here we analyze the region, sleep state and frequency-specific EEG synchronization of whole night sleep recordings of 21 Williams syndrome and 21 typically developing age- and gender-matched subjects by calculating weighted phase lag indexes. We found broadband increases in inter- and intrahemispheric neural connectivity for both NREM and REM sleep EEG of Williams syndrome subjects. These effects consisted of increased theta, high sigma, and beta/low gamma synchronization, whereas alpha synchronization was characterized by a peculiar Williams syndrome-specific decrease during NREM states (intra- and interhemispheric centro-temporal) and REM phases of sleep (occipital intra-area synchronization). We also found a decrease in short range, occipital connectivity of NREM sleep EEG theta activity. The striking increased overall synchronization of sleep EEG in Williams syndrome subjects is consistent with the recently reported increase in synaptic and dendritic density in stem-cell based Williams syndrome models, whereas decreased alpha and occipital connectivity might reflect and underpin the altered microarchitecture of primary visual cortex and disordered visuospatial functioning of Williams syndrome subjects.

  6. [Antisynthetase syndrome without muscle involvement].

    PubMed

    Júdez Navarro, Enrique; Martínez Carretero, Myriam; Martínez Jiménez, Gonzalo Fidel

    2007-11-01

    Antisynthetase syndrome is a well defined syndrome characterized by the presence of interstitial lung disease in association with arthritis, miositis, mechanic's hands and Ruynaud's phenomenon in the presence of antisynthetase antibodies, especially Ac anti-Jo1. We described the case of a 68-year-old man with this syndrome in the absence of inflammatory muscle disease. Copyright © 2007 Elsevier España S.L Barcelona. Published by Elsevier Espana. All rights reserved.

  7. [Evans syndrome, pregnancy, and preeclampsia].

    PubMed

    Hernández-Salazar, E; Martínez-Abundis, C E; González-Ortiz, C M

    2001-02-01

    Evans' syndrome is an unusual illness of autoimmune etiology, characterized by thrombocytopenia and hemolytic anemia. This is more frequent in females throughout first half of the life and during pregnancy. The present paper describes two pregnant women with Evans syndrome associated to preeclampsia. This report emphasizes how the hematology and coagulation abnormalities of preeclampsia could be added to those abnormalities observed in Evans' syndrome. This association constitutes a severe disease of difficult treatment.

  8. Lethal pallister-killian syndrome: Phenotypic similarity with fryns syndrome

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Ignacio Rodriquez, J.; Garcia, I.; Alvarez, J.

    1994-11-01

    The Pallister-Killian syndrome is a sporadic multiple congenital anomaly syndrome characterized by {open_quotes}coarse{close_quotes} face, profound mental retardation, and epilepsy. Chromosomes of peripheral lymphocytes are usually normal, but tissue cultures show varying degrees of mosaicism for isochromosome 12p. In babies who die neonatally of severe malformations, including diaphragmatic hernia, and who also have a {open_quotes}coarse{close_quotes} face, acral hypoplasia, and other internal anomalies, Fryns syndrome is more likely to be suspected than Pallister-Killian syndrome, especially if karyotyping is unavailable or if peripheral lumphocytes have a normal chromosome constitution. An initial diagnosis of Fryns syndrome had to be modified in 3 successive newbornmore » infants since chromosome analysis or in situ hybridization with a chromosome 12 probe on kidney tissue demonstrated the mosaic aneuploidy characteristic of Pallister-Killian syndrome. These 3 patients confirm that a similar pattern of malformations can be present in both conditions at birth. It consists of {open_quotes}coarse{close_quotes} face, acral hypoplasia, diaphragmatic hernia, and other defects. Newborn infants who present this phenotype, but lack a conclusively normal chromosome test, may not have Fryns syndrome. A diagnosis of Fryns syndrome should be made carefully to avoid the risk of inappropriate genetic counseling. 31 refs., 10 figs., 1 tab.« less

  9. Microwave Synthesis and Characterization of Waste Soybean Oil-Based Gemini Imidazolinium Surfactants with Carbonate Linkage

    NASA Astrophysics Data System (ADS)

    Tripathy, Divya Bajpai; Mishra, Anuradha

    Gemini surfactants are presently gaining attention due to their unusual self-assembling characteristics and incomparable interfacial activity. Current research work involves the cost-effective microwave (MW) synthesis of waste soybean oil-based gemini imidazolinium surfactants (GIS) having a carbonate linkage in its spacer moiety. Structural characterizations of the materials have been done using FT-IR, 1H-NMR and 13C-NMR. Using indigenous and natural material as base and MW as energy source for synthesizing the GIS with easily degradable chemical moiety make them to be labeled as green surfactants.

  10. [The refeeding syndrome].

    PubMed

    Lambers, Wietske M; Kraaijenbrink, Bastiaan; Siegert, Carl E H

    2015-01-01

    The refeeding syndrome may occur during reintroduction of carbohydrates in malnourished patients. This syndrome is characterized by reduced plasma electrolyte levels, hypophosphataemia being most prevalent. The symptoms can vary from minor symptoms to severe neurological or cardiac symptoms. The pathophysiological mechanism comprises an increase in insulin levels, resulting in shifts of phosphate, potassium and magnesium into the intracellular environment, as well as fluid retention and relative deficiency of vitamin B1. There is growing interest in the screening and treatment of patients with malnutrition, due to which the incidence of refeeding syndrome is probably increasing. Currently, there is no single definition of this syndrome and therefore there is no solid scientific basis for screening and treatment. In this article we describe the rationale for screening and additional laboratory investigations. A prospective, controlled trial is important to define the clinical relevance of the refeeding syndrome and optimize its treatment.

  11. Basal cell nevus syndrome or Gorlin syndrome.

    PubMed

    Thalakoti, Srikanth; Geller, Thomas

    2015-01-01

    Basal cell nevus syndrome (BCNS) or Gorlin syndrome is a rare neurocutaneous syndrome sometimes known as the fifth phacomatosis, inherited in autosomal dominant fashion with complete penetrance and variable expressivity. Gorlin syndrome is characterized by development of multiple basal cell carcinomas (BCCs), jaw cysts, palmar or plantar pits, calcification of falx cerebri, various developmental skeletal abnormalities such as bifid rib, hemi- or bifid vertebra and predisposition to the development of various tumors. BCNS is caused by a mutation in the PTCH1 gene localized to 9q22.3. Its estimated prevalence varies between 1/55600 and 1/256000 with an equal male to female ratio. The medulloblastoma variant seen in Gorlin syndrome patients is of the desmoplastic type, characteristically presenting during the first 3 years of life. Therefore, children with desmoplastic medulloblastoma should be carefully screened for other features of BCNS. Radiation therapy for desmoplastic medulloblastoma should be avoided in BCNS patients as it may induce development of invasive BCCs and other tumors in the skin area exposed to radiation. This syndrome is a multisystem disorder so involvement of multiple specialists with a multimodal approach to detect and treat various manifestations at early stages will reduce the long-term sequelae and severity of the condition. Life expectancy is not significantly altered but morbidity from complications and cosmetic scarring can be substantial. © 2015 Elsevier B.V. All rights reserved.

  12. Synthesis and characterization of black ceramic pigments by recycling of two hazardous wastes

    NASA Astrophysics Data System (ADS)

    Du, Minxing; Du, Yi; Chen, Zhongtao; Li, Zhongfu; Yang, Kai; Lv, Xingjie; Feng, Yibing

    2017-09-01

    In this study, two different industrial wastes, namely vanadium tailing and leather sludge, were used as less expensive alternative raw materials for the synthesis of black ceramic pigments to be used in commercial glazes. The pigments were based on hematite structure (FexCr1-x)2O3 and prepared by the common solid-state reaction method, under optimal formulation and processing conditions. The synthesized pigments were characterized in typical ceramic glazes and ceramic tile bodies. Optimal color development was achieved when the Fe/Cr mole ratios were 2.0 with 40 wt% content of vanadium tailing at 1200 °C. The coloring properties were similar to those imparted by a commercial black pigment.

  13. Antithyroid Arthritis Syndrome: A Case Report and Review of the Literature

    PubMed Central

    Takaya, Kazuhiko; Kimura, Natsumi; Hiyoshi, Toru

    2016-01-01

    We herein report the case of a 38-year-old Japanese woman with antithyroid arthritis syndrome who experienced severe migratory polyarthritis after the initiation of thiamazole therapy. The patient's symptoms promptly disappeared without any sequelae after the withdrawal of the drug. Antithyroid arthritis syndrome is poorly characterized, and the findings from our literature review indicate that this syndrome exhibits serological features that are distinct from those of antithyroid agent-induced vasculitis syndrome. The absence of autoantibodies, especially anti-neutrophil cytoplasmic antibodies, may help characterize and diagnose antithyroid arthritis syndrome. Furthermore, physicians' awareness of this syndrome is essential for its diagnosis in clinical practice. PMID:27980264

  14. 40 CFR 761.61 - PCB remediation waste.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    .... Subpart N of this part provides a method for collecting new site characterization data or for assessing... left after cleanup is completed. (i) Bulk PCB remediation waste. Bulk PCB remediation waste includes... similar material of minimum thickness spread over the area where remediation waste was removed or left in...

  15. Systematic characterization of generation and management of e-waste in China.

    PubMed

    Duan, Huabo; Hu, Jiukun; Tan, Quanyin; Liu, Lili; Wang, Yanjie; Li, Jinhui

    2016-01-01

    Over the last decade, there has been much effort to promote the management of e-waste in China. Policies have been affected to prohibit imports and to control pollution. Research has been conducted in laboratories and on large-scale industrial operations. A subsidy system to support sound e-waste recycling has been put in place. However, the handling of e-waste is still a concern in China and the issue remains unresolved. There has been relatively little work to follow up this issue or to interpret continuing problems from the perspective of sustainable development. This paper first provides a brief overview of conventional and emerging environmental pollution in Chinese "famous" e-waste dismantling areas, including Guiyu in Guangdong and Wenling in Zhejiang. Environmentalists have repeatedly proven that these areas are significantly polluted. Importing and backyard recycling are decreasing but are ongoing. Most importantly, no work is being done to treat or remediate the contaminated environmental media. The situation is exacerbated by the rising tide of e-waste generated by domestic update of various electronics. This study, therefore, employs a Sales Obsolescence Model approach to predict the generation of e-waste. When accounting for weight, approximately 8 million tons of e-waste will be generated domestically in 2015, of which around 50% is ferrous metals, followed by miscellaneous plastic (30%), copper metal and cables (8%), aluminum (5%), and others (7%). Of this, 3.6% will come from scrap PCBs and 0.2% from lead CRT glass. While more and more end-of-life electronics have been collected and treated by formal or licensed recyclers in China in terms of our analysis, many of them only have dismantling and separation activities. Hazardous e-wastes, including those from PCBs, CRT glass, and brominated flame retardant (BFR) plastics, have become problematic and probably flow to small or backyard recyclers without environmentally sound management. Traditional

  16. Waste certification program plan for Oak Ridge National Laboratory. Revision 1

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Orrin, R.C.

    1997-05-01

    This document defines the waste certification program developed for implementation at Oak Ridge National Laboratory (ORNL). The document describes the program structure, logic, and methodology for certification of ORNL wastes. The purpose of the waste certification program is to provide assurance that wastes are properly characterized and that the Waste Acceptance Criteria (WAC) for receiving facilities are met. The program meets the waste certification requirements outlined in US Department of Energy (DOE) Order 5820.2A, Radioactive Waste Management, and ensures that 40 CFR documentation requirements for waste characterization are met for mixed (both radioactive and hazardous) and hazardous (including polychlorinated biphenyls)more » waste. Program activities will be conducted according to ORNL Level 1 document requirements.« less

  17. Pyrolysis of Date palm waste in a fixed-bed reactor: Characterization of pyrolytic products.

    PubMed

    Bensidhom, Gmar; Ben Hassen-Trabelsi, Aïda; Alper, Koray; Sghairoun, Maher; Zaafouri, Kaouther; Trabelsi, Ismail

    2018-01-01

    The pyrolysis of several Tunisian Date Palm Wastes (DPW): Date Palm Rachis (DPR), Date Palm Leaflets (DPL), Empty Fruit Bunches (EFB) and Date Palm Glaich (DPG) was run using a fixed-bed reactor, from room temperature to 500°C, with 15°C/min as heating rate and -5°C as condensation temperature, in order to produce bio-oil, biochar and syngas. In these conditions, the bio-oil yield ranges from 17.03wt% for DPL to 25.99wt% for EFB. For the biochar, the highest yield (36.66wt%) was obtained for DPL and the lowest one (31.66wt%) was obtained from DPG while the syngas production varies from 39.10wt% for DPR to 46.31wt% DPL. The raw material and pyrolysis products have been characterized using elemental analysis thermogravimetric analysis (TGA), Fourier transform infrared spectroscopy (FTIR), scanning electron microscopy (SEM). The syngas composition has been characterized using gas analyzer. Copyright © 2017 Elsevier Ltd. All rights reserved.

  18. OSSA Space Station waste inventory

    NASA Technical Reports Server (NTRS)

    Rasmussen, Daryl N.; Johnson, Catherine C.; Bosley, John J.; Curran, George L.; Mains, Richard

    1987-01-01

    NASA's Office of Space Science and Applications has compiled an inventory of the types and quantities of the wastes that will be generated by the Space Station's initial operational phase in 35 possible mission scenarios. The objective of this study was the definition of waste management requirements for both the Space Station and the Space Shuttles servicing it. All missions, when combined, will produce about 5350 kg of gaseous, liquid and solid wastes every 90 days. A characterization has been made of the wastes in terms of toxicity, corrosiveness, and biological activity.

  19. The role of acceptable knowledge in transuranic waste disposal operations - 11117

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Chancellor, Christopher John; Nelson, Roger

    2010-11-08

    The Acceptable Knowledge (AK) process plays a key role in the delineation of waste streams destined for the Waste Isolation Pilot Plant (WIPP). General Electric's Vallecitos Nuclear Center (GEVNC) provides for an ideal case study of the application of AK in a multiple steward environment. In this review we will elucidate the pivotal role Acceptable Knowledge played in segregating Department of Energy (DOE) responsibilities from a commercial facility. The Acceptable Knowledge process is a necessary component of waste characterization that determines whether or not a waste stream may be considered for disposal at the WIPP site. This process may bemore » thought of as an effort to gain a thorough understanding of the waste origin, chemical content, and physical form gleaned by the collection of documentation that concerns generator/storage site history, mission, and operations; in addition to waste stream specific information which includes the waste generation process, the waste matrix, the quantity of waste concerned, and the radiological and chemical make up of the waste. The collection and dissemination of relevant documentation is the fundamental requirement for the AK process to work. Acceptable Knowledge is the predominant process of characterization and, therefore, a crucial part of WIPP's transuranic waste characterization program. This characterization process, when conducted to the standards set forth in WIPP's operating permit, requires confirmation/verification by physical techniques such as Non-Destructive Examination (NDE), Visual Examination (VE), and Non-Destructive Assay (NDA). These physical characterization techniques may vary in their appropriateness for a given waste stream; however, nothing will allow the substitution or exclusion of AK. Beyond the normal scope of operations, AK may be considered, when appropriate, a surrogate for the physical characterization techniques in a procedure that appeals to concepts such As Low As Reasonably

  20. Phenotypic variability in gap junction syndromic skin disorders: experience from KID and Clouston syndromes' clinical diagnostics.

    PubMed

    Kutkowska-Kaźmierczak, Anna; Niepokój, Katarzyna; Wertheim-Tysarowska, Katarzyna; Giza, Aleksandra; Mordasewicz-Goliszewska, Maria; Bal, Jerzy; Obersztyn, Ewa

    2015-08-01

    Connexins belong to the family of gap junction proteins which enable direct cell-to-cell communication by forming channels in adjacent cells. Mutations in connexin genes cause a variety of human diseases and, in a few cases, result in skin disorders. There are significant differences in the clinical picture of two rare autosomal dominant syndromes: keratitis-ichthyosis-deafness (KID) syndrome and hidrotic ectodermal dysplasia (Clouston syndrome), which are caused by GJB2 and GJB6 mutations, respectively. This is despite the fact that, in both cases, malfunctioning of the same family proteins and some overlapping clinical features (nail dystrophy, hair loss, and palmoplantar keratoderma) is observed. KID syndrome is characterized by progressive vascularizing keratitis, ichthyosiform erythrokeratoderma, and neurosensory hearing loss, whereas Clouston syndrome is characterized by nail dystrophy, hypotrichosis, and palmoplantar keratoderma. The present paper presents a Polish patient with sporadic KID syndrome caused by the mutation of p.Asp50Asn in GJB2. The patient encountered difficulties in obtaining a correct diagnosis. The other case presented is that of a family with Clouston syndrome (caused by p.Gly11Arg mutation in GJB6), who are the first reported patients of Polish origin suffering from this disorder. Phenotype diversity among patients with the same genotypes reported to date is also summarized. The conclusion is that proper diagnosis of these syndromes is still challenging and should always be followed by molecular verification.

  1. Characterization of antibiotic resistance genes in representative organic solid wastes: Food waste-recycling wastewater, manure, and sewage sludge.

    PubMed

    Lee, Jangwoo; Shin, Seung Gu; Jang, Hyun Min; Kim, Young Beom; Lee, Joonyeob; Kim, Young Mo

    2017-02-01

    In this research, the distribution of antibiotic resistance genes (ARGs) was characterized in representative organic solid waste (OSW) in Korea: food waste-recycling wastewater (FRW), manure, and sewage sludge. The amounts of total ARG (gene copies/16S rRNA gene copies) was greatest in manure followed by sewage sludge and FRW. Interestingly, there were significantly different patterns in the diversity and mechanisms of ARGs. For example, a significant proportion of ARGs were tetracycline resistant genes in all the OSW (40.4-78.2%). β-lactam antibiotics resistant genes were higher in the FRW samples than in other types of OSW but sulfonamides resistant genes represented the greatest proportion in sludge. Regarding the characteristics of antibiotic resistance mechanisms, there was a relatively higher proportion of the ribosomal protection mechanism to tetracycline observed in the FRW and manure samples. However, tetracycline resistant genes with direct interaction were relatively higher in the sewage sludge samples. sul1 was the dominant subtype in all the OSW types and detection of ermB was observed although there was no ermC detected in sewage sludge. There were significant correlations between the occurrences of ARG subtypes: tetB and tetG in all OSW (P<0.01); tetE and tetQ only in sludge (P<0.01). The Class 1 integron-integrase gene (intI1) was significantly correlated with total ARGs only in manure and sludge (P<0.05), revealing potential horizontal gene transfer in these OSW. Copyright © 2016 Elsevier B.V. All rights reserved.

  2. Parry-Romberg syndrome (progressive hemifacial atrophy) with spasmodic dysphonia--a rare association.

    PubMed

    Mugundhan, K; Selvakumar, C J; Gunasekaran, K; Thiruvarutchelvan, K; Sivakumar, S; Anguraj, M; Arun, S

    2014-04-01

    Parry-Romberg syndrome is a rare clinical entity characterised by progressive hemifacial atrophy with appearance of 'saber'. Various neurological and otorhinolaryngological disorders are associated with this syndrome. The association of Parry -Romberg syndrome with Spasmodic dysphonia has rarely been reported. A 37 year old female presented with progressive atrophy of tissues of left side of face for 10 years and change in voice for 1 year. On examination, wasting and atrophy of tissues including tongue was noted on left side of the face. ENT examination revealed adductor spasmodic dysphonia. We report the rare association of Parry -Romberg syndrome with spasmodic dysphonia.

  3. Aging and Intellectual Disability: Insights from Mouse Models of Down Syndrome

    ERIC Educational Resources Information Center

    Ruparelia, Aarti; Pearn, Matthew L.; Mobley, William C.

    2013-01-01

    Down syndrome (DS) is one of many causes of intellectual disability (ID), others including but not limited to, fetal alcohol syndrome, Fragile X syndrome, Rett syndrome, Williams syndrome, hypoxia, and infection. Down syndrome is characterized by a number of neurobiological problems resulting in learning and memory deficits and early onset…

  4. Genetic Modifiers of the Physical Malformations in Velo-Cardio-Facial Syndrome/DiGeorge Syndrome

    ERIC Educational Resources Information Center

    Aggarwal, Vimla S.; Morrow, Bernice E.

    2008-01-01

    Velo-cardio-facial syndrome/DiGeorge syndrome (VCFS/DGS), the most common micro-deletion disorder in humans, is characterized by craniofacial, parathyroid, and thymic defects as well as cardiac outflow tract malformations. Most patients have a similar hemizygous 3 million base pair deletion on 22q11.2. Studies in mouse have shown that "Tbx1", a…

  5. Geologic and geophysical characterization studies of Yucca Mountain, Nevada, a potential high-level radioactive-waste repository

    USGS Publications Warehouse

    Whitney, J.W.; Keefer, W.R.

    2000-01-01

    In recognition of a critical national need for permanent radioactive-waste storage, Yucca Mountain in southwestern Nevada has been investigated by Federal agencies since the 1970's, as a potential geologic disposal site. In 1987, Congress selected Yucca Mountain for an expanded and more detailed site characterization effort. As an integral part of this program, the U.S. Geological Survey began a series of detailed geologic, geophysical, and related investigations designed to characterize the tectonic setting, fault behavior, and seismicity of the Yucca Mountain area. This document presents the results of 13 studies of the tectonic environment of Yucca Mountain, in support of a broad goal to assess the effects of future seismic and fault activity in the area on design, long-term performance, and safe operation of the potential surface and subsurface repository facilities.

  6. Chemical composition and methane potential of commercial food wastes.

    PubMed

    Lopez, Victoria M; De la Cruz, Florentino B; Barlaz, Morton A

    2016-10-01

    There is increasing interest in anaerobic digestion in the U.S. However, there is little information on the characterization of commercial food waste sources as well as the effect of waste particle size on methane yield. The objective of this research was to characterize four commercial food waste sources: (1) university dining hall waste, (2) waste resulting from prepared foods and leftover produce at a grocery store, (3) food waste from a hotel and convention center, and (4) food preparation waste from a restaurant. Each sample was tested in triplicate 8L batch anaerobic digesters after shredding and after shredding plus grinding. Average methane yields for the university dining, grocery store, hotel, and restaurant wastes were 363, 427, 492, and 403mL/dry g, respectively. Starch exhibited the most complete consumption and particle size did not significantly affect methane yields for any of the tested substrates. Lipids represented 59-70% of the methane potential of the fresh substrates. Copyright © 2016 Elsevier Ltd. All rights reserved.

  7. CHARACTERIZATION AND RECYCLING OF WASTE WATER FROM GUAYULE LATEX EXTRACTION

    USDA-ARS?s Scientific Manuscript database

    Guayule commercialization for latex production to be used in medical products and other applications is now a reality. Currently, waste water following latex extraction is discharged into evaporation ponds. As commercialization reaches full scale, the liquid waste stream from latex extraction will b...

  8. Characterization of dermatoglyphics in PHOX2B-confirmed congenital central hypoventilation syndrome.

    PubMed

    Todd, Emily S; Scott, Nicole M; Weese-Mayer, Debra E; Weinberg, Seth M; Berry-Kravis, Elizabeth M; Silvestri, Jean M; Kenny, Anna S; Hauptman, Susan A; Zhou, Lili; Marazita, Mary L

    2006-08-01

    Individuals with congenital central hypoventilation syndrome have characteristic variants in the PHOX2B gene (primarily polyalanine expansion mutations). The PHOX2B gene acts as a transcriptional activator in the promotion of pan-neuronal differentiation in the autonomic nervous system during early embryologic development, with a primary role in the sympathetic noradrenergic phenotype in vertebrates. Because sympathetic innervation has been hypothesized to affect the development of dermatoglyphic pattern types, we hypothesized that individuals with PHOX2B-confirmed congenital central hypoventilation syndrome would have characteristic dermatoglyphic patterning and that the dermatoglyphic phenotype would be related to the disease-defining PHOX2B genotype. Dermatoglyphic pattern type frequency, left/right symmetry, and genotype/phenotype correlation were assessed for 33 individuals with PHOX2B-confirmed congenital central hypoventilation syndrome and compared with published control data. Dermatoglyphic pattern type frequencies were altered in congenital central hypoventilation syndrome cases versus controls. In particular, there was an increase of arches in females and ulnar loops in males, with the largest differences for the left hand and for individuals with both congenital central hypoventilation syndrome and Hirschsprung disease. Dissimilarity scores between the congenital central hypoventilation syndrome and congenital central hypoventilation syndrome + Hirschsprung disease cases were not significantly different, nor were dissimilarity scores between all of the female and all of the male cases. No significant association was found between the number of polyalanine repeats in the PHOX2B genotypic category and dermatoglyphic pattern frequencies in the congenital central hypoventilation syndrome study groups. These results represent the first report describing specific dermatoglyphic patterning in congenital central hypoventilation syndrome and suggest a

  9. Gorlin-goltz syndrome: a rare case.

    PubMed

    Ganguly, Satyaki; Jaykar, Kranti C; Kumar, Rajesh; Jha, Abhijeet Kumar; Banerjee, P K

    2015-01-01

    Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome is characterized by multiple basocellular epitheliomas, keratocysts in the jaws, bifid ribs, palmar and/or plantar pits and ectopic calcifications of the falx cerebri. We describe a case of Gorlin-Goltz syndrome illustrating the importance of a thorough examination including the examination of palms and soles and detailed investigations in a patient having lesions suggestive of basal cell carcinoma and multiple naevi.

  10. Educational Considerations for Children with Tourette's Syndrome.

    ERIC Educational Resources Information Center

    Jones, Kevin; Johnson, Genevieve Marie

    1992-01-01

    This article provides an introduction to Tourette's Syndrome, an inherited neurological disorder characterized by motor and vocal tics. Considered are prevalence of the syndrome, common characteristics, instructional strategies, and the critical role of the teacher. (Author/DB)

  11. The salivary proteome profile in patients affected by SAPHO syndrome characterized by a top-down RP-HPLC-ESI-MS platform.

    PubMed

    Sanna, Monica; Firinu, Davide; Manconi, Paolo Emilio; Pisanu, Maria; Murgia, Giuseppe; Piras, Valentina; Castagnola, Massimo; Messana, Irene; del Giacco, Stefano Renato; Cabras, Tiziana

    2015-06-01

    SAPHO syndrome is a rare and often unrecognized disease with prominent inflammatory cutaneous and articular symptoms characterized by musculoskeletal manifestations (synovitis, hyperostosis, osteomyelitis) associated with dermatological conditions (severe acne and pustulosis). The acidic soluble fraction of whole saliva from 10 adult women affected by SAPHO syndrome and from a group of 28 healthy women was analysed by RP-HPLC-ESI-MS with the aim of discovering salivary biomarkers of the disorder. The levels of the oral proteins and peptides were correlated with clinical data. The following proteins showed a significant decreased concentration in saliva of SAPHO subjects with respect to controls: cystatin S1 and SN, histatins, the major acidic PRPs, P-C and P-B peptides. The cystatin SN abundance lowered according to the disease duration and histatins showed positive correlations with the C reactive protein. Statistical analysis performed excluding one patient with a different pattern of salivary proteins/peptides highlighted a positive relationship between cystatin S1, histatins 3, histatin 5, and the neutrophil count. Moreover, histatin 3 correlated positively with the total white cell count and negatively with the erythrocyte sedimentation rate. Levels and frequency of S100A12 protein showed a trend to increase in SAPHO patients. The high expression of this pro-inflammatory protein is probably related to the inflammatory response and to the altered neutrophil responses to functional stimuli that characterize SAPHO syndrome suggesting a possible application as a salivary biomarker.

  12. Twelfth cranial nerve involvement in Guillian Barre syndrome

    PubMed Central

    Nanda, Subrat Kumar; Jayalakshmi, Sita; Ruikar, Devashish; Surath, Mohandas

    2013-01-01

    Guillian Barre Syndrome (GBS) is associated with cranial nerve involvement. Commonest cranial nerves involved were the facial and bulbar (IXth and Xth). Involvement of twelfth cranial nerve is rare in GBS. We present a case of GBS in a thirteen years old boy who developed severe tongue weakness and wasting at two weeks after the onset of GBS. The wasting and weakness of tongue improved at three months of follow up. Brief review of the literature about XIIth cranial nerve involvement in GBS is discussed. PMID:24250180

  13. Twelfth cranial nerve involvement in Guillian Barre syndrome.

    PubMed

    Nanda, Subrat Kumar; Jayalakshmi, Sita; Ruikar, Devashish; Surath, Mohandas

    2013-07-01

    Guillian Barre Syndrome (GBS) is associated with cranial nerve involvement. Commonest cranial nerves involved were the facial and bulbar (IXth and Xth). Involvement of twelfth cranial nerve is rare in GBS. We present a case of GBS in a thirteen years old boy who developed severe tongue weakness and wasting at two weeks after the onset of GBS. The wasting and weakness of tongue improved at three months of follow up. Brief review of the literature about XIIth cranial nerve involvement in GBS is discussed.

  14. Histological characterization of regression in acquired immunodeficiency syndrome-related Kaposi's sarcoma.

    PubMed

    Pantanowitz, Liron; Dezube, Bruce J; Pinkus, Geraldine S; Tahan, Steven R

    2004-01-01

    Kaposi's sarcoma (KS) is an angioproliferative lesion that may regress or progress. Progression is related to spindle cell proliferation and the expression of human herpes virus-8 latency genes, including latent nuclear antigen-1 (LNA-1), cyclin-D1, and bcl-2. KS regression has not been well characterized histologically. Therefore, this study was undertaken to characterize the histopathology of pharmacologically induced regressed cutaneous KS. Skin punch biopsies from eight patients with acquired immunodeficiency syndrome (AIDS)-related KS, that regressed following chemotherapy with paclitaxel or the angiogenesis inhibitor Col-3, were investigated by light microscopy. Comparative immunophenotyping on pre- and post-treatment specimens for CD31, LNA-1, cyclin-D1, bcl-2, and CD117 (c-kit) was performed. Clinical and histologic features of regression were similar for paclitaxel and Col-3 treatment. On clinical examination, lesions flattened, became smaller, and lost their purple-red appearance, resulting in an orange-brown macule. Histological regression was divided into partial (n = 3) and complete (n = 5) regression. Partially regressed lesions had a significant reduction of spindle cells in the dermal interstitium, with residual spindle cells arranged around superficial and mid-dermal capillaries. Complete regression was characterized by an absence of detectable spindle cells, with a slight increase in capillaries of the superficial plexus. All regressed samples exhibited a prominent, superficial, perivascular, lymphocytic infiltrate and abundant dermal hemosiderin-laden macrophages. This clinicopathologic picture resembled the findings of pigmented purpura. CD31 staining correlated with the reduction of spindle cells. Regression was accompanied by a quantitative and qualitative decrease in LNA-1 and cyclin-D1 immunoreactivity, but no change in bcl-2 or c-kit expression. Pharmacologically induced regression of AIDS-related cutaneous KS is characterized by a complete

  15. Cerebral gigantism (Sotos' syndrome) and cataracts.

    PubMed

    Yeh, H; Price, R L; Lonsdale, D

    1978-01-01

    A five-year-old girl with cerebral gigantism (Sotos' syndrome) and cataracts is described. Sotos' syndrome, characterized by generalized gigantism with normal endocrine studies has rarely been reported with ocular abnormalities and never with cataracts. It is important to study any child with cataracts for systemic disease.

  16. An Educator's Guide to Tourette Syndrome.

    ERIC Educational Resources Information Center

    Bronheim, Suzanne

    Intended for educators, the booklet presents information on Tourette's Syndrome (TS), an inherited neurological disorder characterized by involuntary multiple motor and vocal tics. The first section describes Tourette Syndrome--its causes, symptons, and treatments. In the second section, suggestions are provided to help teachers and school…

  17. Characterization of Heat-treated Clay Minerals in the Context of Nuclear Waste Disposal

    NASA Astrophysics Data System (ADS)

    Matteo, E. N.; Wang, Y.; Kruichak, J. N.; Mills, M. M.

    2015-12-01

    Clay minerals are likely candidates to aid in nuclear waste isolation due to their low permeability, favorable swelling properties, and high cation sorption capacities. Establishing the thermal limit for clay minerals in a nuclear waste repository is a potentially important component of repository design, as flexibility of the heat load within the repository can have a major impact on the selection of repository design. For example, the thermal limit plays a critical role in the time that waste packages would need to cool before being transferred to the repository. Understanding the chemical and physical changes, if any, that occur in clay minerals at various temperatures above the current thermal limit (of 100 °C) can enable decision-makers with information critical to evaluating the potential trade-offs of increasing the thermal limit within the repository. Most critical is gaining understanding of how varying thermal conditions in the repository will impact radionuclide sorption and transport in clay materials either as engineered barriers or as disposal media. A variety of repository-relevant clay minerals (illite, mixed layer illite/smectite, and montmorillonite), were heated for a range of temperatures between 100-1000 °C. These samples were characterized to determine surface area, mineralogical alteration, and cation exchange capacity (CEC). Our results show that for conditions up to 500 °C, no significant change occurs, so long as the clay mineral remains mineralogically intact. At temperatures above 500 °C, transformation of the layered silicates into silica phases leads to alteration that impacts important clay characteristics. Sandia National Laboratories is a multi-program laboratory managed and operated by Sandia Corporation, a wholly owned subsidiary of Lockheed Martin Corporation, for the U.S. Department of Energy's Nation Nuclear Security Administration under contract DE-AC04-94AL85000. SAND Number: SAND2015-6524 A

  18. Toxic metals in WEEE: characterization and substance flow analysis in waste treatment processes.

    PubMed

    Oguchi, Masahiro; Sakanakura, Hirofumi; Terazono, Atsushi

    2013-10-01

    Waste electrical and electronic equipment (WEEE) has received extensive attention as a secondary source of metals. Because WEEE also contains toxic substances such as heavy metals, appropriate management of these substances is important in the recycling and treatment of WEEE. As a basis for discussion toward better management of WEEE, this study characterizes various types of WEEE in terms of toxic metal contents. The fate of various metals contained in WEEE, including toxic metals, was also investigated in actual waste treatment processes. Cathode-ray tube televisions showed the highest concentration and the largest total amount of toxic metals such as Ba, Pb, and Sb, so appropriate recycling and disposal of these televisions would greatly contribute to better management of toxic metals in WEEE. A future challenge is the management of toxic metals in mid-sized items such as audio/visual and ICT equipment because even though the concentrations were not high in these items, the total amount of toxic metals contained in them is not negligible. In the case of Japan, such mid-sized WEEE items as well as small electronic items are subject to municipal solid waste treatment. A case study showed that a landfill was the main destination of toxic metals contained in those items in the current treatment systems. The case study also showed that changes in the flows of toxic metals will occur when treatment processes are modified to emphasize resource recovery. Because the flow changes might lead to an increase in the amount of toxic metals released to the environment, the flows of toxic metals and the materials targeted for resource recovery should be considered simultaneously. Copyright © 2012 Elsevier B.V. All rights reserved.

  19. Preliminary ECLSS waste water model

    NASA Technical Reports Server (NTRS)

    Carter, Donald L.; Holder, Donald W., Jr.; Alexander, Kevin; Shaw, R. G.; Hayase, John K.

    1991-01-01

    A preliminary waste water model for input to the Space Station Freedom (SSF) Environmental Control and Life Support System (ECLSS) Water Processor (WP) has been generated for design purposes. Data have been compiled from various ECLSS tests and flight sample analyses. A discussion of the characterization of the waste streams comprising the model is presented, along with a discussion of the waste water model and the rationale for the inclusion of contaminants in their respective concentrations. The major objective is to establish a methodology for the development of a waste water model and to present the current state of that model.

  20. Kindler syndrome.

    PubMed

    Sharma, Ramesh Chander; Mahajan, Vikram; Sharma, Nand Lal; Sharma, Ashok K

    2003-09-01

    Kindler syndrome is a rare genodermatosis characterized by acral bullae and photosensitivity. The photosensitivity improves with advancing age and results in progressive poikiloderma and cutaneous atrophy, and many additional features have also been described. This report describes two male Kindler syndrome patients with classical features of acral blistering and photosensitivity in childhood, and subsequent development of poikiloderma, leukokeratosis of oro-ano-genital mucosae, phimosis and meatal stenosis. The first patient had additional ophthalmic features of chronic simple conjunctivitis caused by persistent irritation, multiple stromal nebular corneal opacities and thickened corneal nerves. The second patient showed skeletal changes, namely a dome-shaped skull (turri-cephaly), bifid fourth rib, missing fifth rib, short fourth and fifth metacarpals and mandibular abnormalities. This is the first report of such ophthalmic and skeletal features of Kindler syndrome.

  1. Production and characterization of rhamnolipid using palm oil agricultural refinery waste.

    PubMed

    Radzuan, Mohd Nazren; Banat, Ibrahim M; Winterburn, James

    2017-02-01

    In this research we assess the feasibility of using palm oil agricultural refinery waste as a carbon source for the production of rhamnolipid biosurfactant through fermentation. The production and characterization of rhamnolipid produced by Pseudomonas aeruginosa PAO1 grown on palm fatty acid distillate (PFAD) under batch fermentation were investigated. Results show that P. aeruginosa PAO1 can grow and produce 0.43gL -1 of rhamnolipid using PFAD as the sole carbon source. Identification of the biosurfactant product using mass spectrometry confirmed the presence of monorhamnolipid and dirhamnolipid. The rhamnolipid produced from PFAD were able to reduce surface tension to 29mNm -1 with a critical micelle concentration (CMC) 420mgL -1 and emulsify kerosene and sunflower oil, with an emulsion index up to 30%. Results demonstrate that PFAD could be used as a low-cost substrate for rhamnolipid production, utilizing and transforming it into a value added biosurfactant product. Copyright © 2016 Elsevier Ltd. All rights reserved.

  2. Differences in manifestations of Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome.

    PubMed

    Meester, Josephina A N; Verstraeten, Aline; Schepers, Dorien; Alaerts, Maaike; Van Laer, Lut; Loeys, Bart L

    2017-11-01

    Many different heritable connective tissue disorders (HCTD) have been described over the past decades. These syndromes often affect the connective tissue of various organ systems, including heart, blood vessels, skin, joints, bone, eyes, and lungs. The discovery of these HCTD was followed by the identification of mutations in a wide range of genes encoding structural proteins, modifying enzymes, or components of the TGFβ-signaling pathway. Three typical examples of HCTD are Marfan syndrome (MFS), Ehlers-Danlos syndrome (EDS), and Loeys-Dietz syndrome (LDS). These syndromes show some degree of phenotypical overlap of cardiovascular, skeletal, and cutaneous features. MFS is typically characterized by cardiovascular, ocular, and skeletal manifestations and is caused by heterozygous mutations in FBN1 , coding for the extracellular matrix (ECM) protein fibrillin-1. The most common cardiovascular phenotype involves aortic aneurysm and dissection at the sinuses of Valsalva. LDS is caused by mutations in TGBR1/2 , SMAD2/3 , or TGFB2/3 , all coding for components of the TGFβ-signaling pathway. LDS can be distinguished from MFS by the unique presence of hypertelorism, bifid uvula or cleft palate, and widespread aortic and arterial aneurysm and tortuosity. Compared to MFS, LDS cardiovascular manifestations tend to be more severe. In contrast, no association is reported between LDS and the presence of ectopia lentis, a key distinguishing feature of MFS. Overlapping features between MFS and LDS include scoliosis, pes planus, anterior chest deformity, spontaneous pneumothorax, and dural ectasia. EDS refers to a group of clinically and genetically heterogeneous connective tissue disorders and all subtypes are characterized by variable abnormalities of skin, ligaments and joints, blood vessels, and internal organs. Typical presenting features include joint hypermobility, skin hyperextensibility, and tissue fragility. Up to one quarter of the EDS patients show aortic aneurysmal

  3. Differences in manifestations of Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome

    PubMed Central

    Meester, Josephina A. N.; Verstraeten, Aline; Schepers, Dorien; Alaerts, Maaike; Van Laer, Lut

    2017-01-01

    Many different heritable connective tissue disorders (HCTD) have been described over the past decades. These syndromes often affect the connective tissue of various organ systems, including heart, blood vessels, skin, joints, bone, eyes, and lungs. The discovery of these HCTD was followed by the identification of mutations in a wide range of genes encoding structural proteins, modifying enzymes, or components of the TGFβ-signaling pathway. Three typical examples of HCTD are Marfan syndrome (MFS), Ehlers-Danlos syndrome (EDS), and Loeys-Dietz syndrome (LDS). These syndromes show some degree of phenotypical overlap of cardiovascular, skeletal, and cutaneous features. MFS is typically characterized by cardiovascular, ocular, and skeletal manifestations and is caused by heterozygous mutations in FBN1, coding for the extracellular matrix (ECM) protein fibrillin-1. The most common cardiovascular phenotype involves aortic aneurysm and dissection at the sinuses of Valsalva. LDS is caused by mutations in TGBR1/2, SMAD2/3, or TGFB2/3, all coding for components of the TGFβ-signaling pathway. LDS can be distinguished from MFS by the unique presence of hypertelorism, bifid uvula or cleft palate, and widespread aortic and arterial aneurysm and tortuosity. Compared to MFS, LDS cardiovascular manifestations tend to be more severe. In contrast, no association is reported between LDS and the presence of ectopia lentis, a key distinguishing feature of MFS. Overlapping features between MFS and LDS include scoliosis, pes planus, anterior chest deformity, spontaneous pneumothorax, and dural ectasia. EDS refers to a group of clinically and genetically heterogeneous connective tissue disorders and all subtypes are characterized by variable abnormalities of skin, ligaments and joints, blood vessels, and internal organs. Typical presenting features include joint hypermobility, skin hyperextensibility, and tissue fragility. Up to one quarter of the EDS patients show aortic aneurysmal

  4. Particle size fractionation as a method for characterizing the nutrient content of municipal green waste used for composting.

    PubMed

    Haynes, R J; Belyaeva, O N; Zhou, Y-F

    2015-01-01

    In order to better characterize mechanically shredded municipal green waste used for composting, five samples from different origins were separated into seven particle size fractions (>20mm, 10-20mm, 5-10mm, 2-5mm, 1-2mm, 0.5-1.0mm and <0.5mm diameter) and analyzed for organic C and nutrient content. With decreasing particle size there was a decrease in organic C content and an increase in macronutrient, micronutrient and ash content. This reflected a concentration of lignified woody material in the larger particle fractions and of green stems and leaves and soil in the smaller particle sizes. The accumulation of nutrients in the smaller sized fractions means the practice of using large particle sizes for green fuel and/or mulch does not greatly affect nutrient cycling via green waste composting. During a 100-day incubation experiment, using different particle size fractions of green waste, there was a marked increase in both cumulative CO2 evolution and mineral N accumulation with decreasing particle size. Results suggested that during composting of bulk green waste (with a high initial C/N ratio such as 50:1), mineral N accumulates because decomposition and net N immobilization in larger particles is slow while net N mineralization proceeds rapidly in the smaller (<1mm dia.) fractions. Initially, mineral N accumulated in green waste as NH4(+)-N, but over time, nitrification proceeded resulting in accumulation of NO3(-)-N. It was concluded that the nutrient content, N mineralization potential and decomposition rate of green waste differs greatly among particle size fractions and that chemical analysis of particle size fractions provides important additional information over that of a bulk sample. Copyright © 2014 Elsevier Ltd. All rights reserved.

  5. Idaho National Engineering Laboratory code assessment of the Rocky Flats transuranic waste

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    NONE

    1995-07-01

    This report is an assessment of the content codes associated with transuranic waste shipped from the Rocky Flats Plant in Golden, Colorado, to INEL. The primary objective of this document is to characterize and describe the transuranic wastes shipped to INEL from Rocky Flats by item description code (IDC). This information will aid INEL in determining if the waste meets the waste acceptance criteria (WAC) of the Waste Isolation Pilot Plant (WIPP). The waste covered by this content code assessment was shipped from Rocky Flats between 1985 and 1989. These years coincide with the dates for information available in themore » Rocky Flats Solid Waste Information Management System (SWIMS). The majority of waste shipped during this time was certified to the existing WIPP WAC. This waste is referred to as precertified waste. Reassessment of these precertified waste containers is necessary because of changes in the WIPP WAC. To accomplish this assessment, the analytical and process knowledge available on the various IDCs used at Rocky Flats were evaluated. Rocky Flats sources for this information include employee interviews, SWIMS, Transuranic Waste Certification Program, Transuranic Waste Inspection Procedure, Backlog Waste Baseline Books, WIPP Experimental Waste Characterization Program (headspace analysis), and other related documents, procedures, and programs. Summaries are provided of: (a) certification information, (b) waste description, (c) generation source, (d) recovery method, (e) waste packaging and handling information, (f) container preparation information, (g) assay information, (h) inspection information, (i) analytical data, and (j) RCRA characterization.« less

  6. CHARACTERIZATION OF SPONTANEOUS AND INDUCED PUBERTY IN GIRLS WITH TURNER SYNDROME.

    PubMed

    Folsom, Lisal J; Slaven, James E; Nabhan, Zeina M; Eugster, Erica A

    2017-07-01

    To characterize puberty in girls with Turner syndrome (TS) and determine whether specific patient characteristics are associated with the timing of menarche. We also sought to compare spontaneous versus induced puberty in these patients. Medical records of girls followed in our Pediatric Endocrine clinic for TS from 2007 to 2015 were reviewed. Fifty-three girls were included, of whom 10 (19%) achieved menarche spontaneously and 43 (81%) received hormone replacement therapy (HRT). Of girls receiving HRT, a younger age at estrogen initiation correlated with a longer time to menarche (P = .02), and a mosaic karyotype was associated with a shorter time to menarche (P = .02), whereas no relationship was seen for body mass index, estrogen regimen, or maternal age at menarche. Nineteen girls (44%) receiving HRT had bleeding on estrogen alone at a wide dose range and were more likely to be on transdermal than oral preparations (P = .01). Girls with spontaneous puberty achieved menarche at a younger age (P<.01) and were more likely to have mosaic TS (P = .02). Significant variability in the timing of menarche exists among girls with TS. However, age at pubertal induction and karyotype were significantly correlated with age at menarche in our patients. A wide range of estrogen doses is seen in girls who bleed prior to progesterone, suggesting extreme variability in estrogen sensitivity among patients with TS. Girls achieving spontaneous menarche are younger and more likely to have a mosaic karyotype than those with induced menarche. Large-scale prospective studies are needed to confirm these results. BMI = body mass index; HRT = hormone replacement therapy; TS = Turner syndrome.

  7. The Architecture of the Pollen Hoarding Syndrome in Honey Bees: Implications for Understanding Social Evolution, Behavioral Syndromes, and Selective Breeding.

    PubMed

    Rueppell, Olav

    2014-05-01

    Social evolution has influenced every aspect of contemporary honey bee biology, but the details are difficult to reconstruct. The reproductive ground plan hypothesis of social evolution proposes that central regulators of the gonotropic cycle of solitary insects have been coopted to coordinate social complexity in honey bees, such as the division of labor among workers. The predicted trait associations between reproductive physiology and social behavior have been identified in the context of the pollen hoarding syndrome, a larger suite of interrelated traits. The genetic architecture of this syndrome is characterized by a partially overlapping genetic architecture with several consistent, pleiotropic QTL. Despite these central QTL and an integrated hormonal regulation, separate aspects of the pollen hoarding syndrome may evolve independently due to peripheral QTL and additionally segregating genetic variance. The characterization of the pollen hoarding syndrome has also demonstrated that this syndrome involves many non-behavioral traits, which may be the case for numerous "behavioral" syndromes. Furthermore, the genetic architecture of the pollen hoarding syndrome has implications for breeding programs for improving honey health and other desirable traits: If these traits are comparable to the pollen hoarding syndrome, consistent pleiotropic QTL will enable marker assisted selection, while sufficient additional genetic variation may permit the dissociation of trade-offs for efficient multiple trait selection.

  8. Characterization of vascular complications in experimental model of fructose-induced metabolic syndrome.

    PubMed

    El-Bassossy, Hany M; Dsokey, Nora; Fahmy, Ahmed

    2014-12-01

    Vascular dysfunction is an important complication associated with metabolic syndrome (MS). Here we fully characterized vascular complications in a rat model of fructose-induced MS. MS was induced by adding fructose (10%) to drinking water to male Wistar rats of 6 weeks age. Blood pressure (BP) and isolated aorta responses phenylephrine (PE), KCl, acetylcholine (ACh), and sodium nitroprusside (SNP) were recorded after 6, 9, and 12 weeks of fructose administration. In addition, serum levels of glucose, insulin, uric acid, tumor necrosis factor α (TNFα), lipids, advanced glycation end products (AGEs), and arginase activity were determined. Furthermore, aortic reactive oxygen species (ROS) generation, hemeoxygenase-1 expression, and collagen deposition were examined. Fructose administration resulted in a significant hyperinslinemia after 6 weeks which continued for 12 weeks. It was also associated with a significant increase in BP after 6 weeks which was stable for 12 weeks. Aorta isolated from MS animals showed exaggerated contractility to PE and KCl and impaired relaxation to ACh compared with control after 6 weeks which were clearer at 12 weeks of fructose administration. In addition, MS animals showed significant increases in serum levels of lipids, uric acid, AGEs, TNFα, and arginase enzyme activity after 12 weeks of fructose administration. Furthermore, aortae isolated from MS animals were characterized by increased ROS generation and collagen deposition. In conclusion, adding fructose (10%) to drinking water produces a model of MS with vascular complications after 12 weeks that are characterized by insulin resistance, hypertension, disturbed vascular reactivity and structure, hyperuricemia, dyslipidemia, and low-grade inflammation.

  9. The tumor secretory factor ZAG promotes white adipose tissue browning and energy wasting.

    PubMed

    Elattar, Sawsan; Dimri, Manali; Satyanarayana, Ande

    2018-03-23

    Cachexia is a complex tissue-wasting syndrome characterized by inflammation, hypermetabolism, increased energy expenditure, and anorexia. Browning of white adipose tissue (WAT) is one of the significant factors that contribute to energy wasting in cachexia. By utilizing a cell implantation model, we demonstrate here that the lipid mobilizing factor zinc-α 2 -glycoprotein (ZAG) induces WAT browning in mice. Increased circulating levels of ZAG not only induced lipolysis in adipose tissues but also caused robust browning in WAT. Stimulating WAT progenitors with ZAG recombinant protein or expression of ZAG in mouse embryonic fibroblasts (MEFs) strongly enhanced brown-like differentiation. At the molecular level, ZAG stimulated peroxisome proliferator-activated receptor γ (PPARγ) and early B cell factor 2 expression and promoted their recruitment to the PR/SET domain 16 (Prdm16) promoter, leading to enhanced expression of Prdm16, which determines brown cell fate. In brown adipose tissue, ZAG stimulated the expression of PPARγ and PPARγ coactivator 1α and promoted recruitment of PPARγ to the uncoupling protein 1 (Ucp1) promoter, leading to increased expression of Ucp1. Overall, our results reveal a novel function of ZAG in WAT browning and highlight the targeting of ZAG as a potential therapeutic application in humans with cachexia.-Elattar, S., Dimri, M., Satyanarayana, A. The tumor secretory factor ZAG promotes white adipose tissue browning and energy wasting.

  10. Because You Asked about Waardenburg Syndrome.

    ERIC Educational Resources Information Center

    National Inst. on Deafness and Other Communications Disorders, Bethesda, MD.

    This pamphlet uses a question-and-answer format to provide information about Waardenburg syndrome, an inherited disorder often characterized by varying degrees of hearing loss and changes in skin and hair pigmentation. The pamphlet covers: causes of Waardenburg syndrome. characteristics, types, research being done, ways to help in research…

  11. Characterization of microbial and chemical composition of shuttle wet waste with permanent gas and volatile organic compound analyses

    NASA Astrophysics Data System (ADS)

    Peterson, B. V.; Hummerick, M.; Roberts, M. S.; Krumins, V.; Kish, A. L.; Garland, J. L.; Maxwell, S.; Mills, A.

    2004-01-01

    Solid-waste treatment in space for Advanced Life Support, ALS, applications requires that the material can be safely processed and stored in a confined environment. Many solid-wastes are not stable because they are wet (40-90% moisture) and contain levels of soluble organic compounds that can contribute to the growth of undesirable microorganisms with concomitant production of noxious odors. In the absence of integrated Advanced Life Support systems on orbit, permanent gas, trace volatile organic and microbiological analyses were performed on crew refuse returned from the volume F "wet" trash of three consecutive Shuttle missions (STS-105, 109, and 110). These analyses were designed to characterize the short-term biological stability of the material and assess potential crew risks resulting from microbial decay processes during storage. Waste samples were collected post-orbiter landing and sorted into packaging material, food waste, toilet waste, and bulk liquid fractions deposited during flight in the volume F container. Aerobic and anaerobic microbial loads were determined in each fraction by cultivation on R2A and by acridine orange direct count (AODC). Dry and ash weights were performed to determine both water and organic content of the materials. Experiments to determine the aerobic and anaerobic biostability of refuse stored for varying periods of time were performed by on-line monitoring of CO 2 and laboratory analysis for production of hydrogen sulfide and methane. Volatile organic compounds and permanent gases were analyzed using EPA Method TO15 by USEPA et al. [EPA Method TO15, The Determination of Volatile Organic Compounds (VOCs) in Ambient Air using SUMMA, Passivated Canister Sampling and Gas Chromatographic Analysis, 1999] with gas chromatography/mass spectrometry and by gas chromatography with selective detectors. These baseline measures of waste stream content, labile organics, and microbial load in the volume F Shuttle trash provide data for waste

  12. Characterization of microbial and chemical composition of shuttle wet waste with permanent gas and volatile organic compound analyses

    NASA Technical Reports Server (NTRS)

    Peterson, B. V.; Hummerick, M.; Roberts, M. S.; Krumins, V.; Kish, A. L.; Garland, J. L.; Maxwell, S.; Mills, A.

    2004-01-01

    Solid-waste treatment in space for Advanced Life Support, ALS, applications requires that the material can be safely processed and stored in a confined environment. Many solid-wastes are not stable because they are wet (40-90% moisture) and contain levels of soluble organic compounds that can contribute to the growth of undesirable microorganisms with concomitant production of noxious odors. In the absence of integrated Advanced Life Support systems on orbit, permanent gas, trace volatile organic and microbiological analyses were performed on crew refuse returned from the volume F "wet" trash of three consecutive Shuttle missions (STS-105, 109, and 110). These analyses were designed to characterize the short-term biological stability of the material and assess potential crew risks resulting from microbial decay processes during storage. Waste samples were collected post-orbiter landing and sorted into packaging material, food waste, toilet waste, and bulk liquid fractions deposited during flight in the volume F container. Aerobic and anaerobic microbial loads were determined in each fraction by cultivation on R2A and by acridine orange direct count (AODC). Dry and ash weights were performed to determine both water and organic content of the materials. Experiments to determine the aerobic and anaerobic biostability of refuse stored for varying periods of time were performed by on-line monitoring of CO2 and laboratory analysis for production of hydrogen sulfide and methane. Volatile organic compounds and permanent gases were analyzed using EPA Method TO15 by USEPA et al. [EPA Method TO15, The Determination of Volatile Organic Compounds (VOCs) in Ambient Air using SUMMA, Passivated Canister Sampling and Gas Chromatographic Analysis,1999] with gas chromatography/mass spectrometry and by gas chromatography with selective detectors. These baseline measures of waste stream content, labile organics, and microbial load in the volume F Shuttle trash provide data for waste

  13. Characterization of microbial and chemical composition of shuttle wet waste with permanent gas and volatile organic compound analyses.

    PubMed

    Peterson, B V; Hummerick, M; Roberts, M S; Krumins, V; Kish, A L; Garland, J L; Maxwell, S; Mills, A

    2004-01-01

    Solid-waste treatment in space for Advanced Life Support, ALS, applications requires that the material can be safely processed and stored in a confined environment. Many solid-wastes are not stable because they are wet (40-90% moisture) and contain levels of soluble organic compounds that can contribute to the growth of undesirable microorganisms with concomitant production of noxious odors. In the absence of integrated Advanced Life Support systems on orbit, permanent gas, trace volatile organic and microbiological analyses were performed on crew refuse returned from the volume F "wet" trash of three consecutive Shuttle missions (STS-105, 109, and 110). These analyses were designed to characterize the short-term biological stability of the material and assess potential crew risks resulting from microbial decay processes during storage. Waste samples were collected post-orbiter landing and sorted into packaging material, food waste, toilet waste, and bulk liquid fractions deposited during flight in the volume F container. Aerobic and anaerobic microbial loads were determined in each fraction by cultivation on R2A and by acridine orange direct count (AODC). Dry and ash weights were performed to determine both water and organic content of the materials. Experiments to determine the aerobic and anaerobic biostability of refuse stored for varying periods of time were performed by on-line monitoring of CO2 and laboratory analysis for production of hydrogen sulfide and methane. Volatile organic compounds and permanent gases were analyzed using EPA Method TO15 by USEPA et al. [EPA Method TO15, The Determination of Volatile Organic Compounds (VOCs) in Ambient Air using SUMMA, Passivated Canister Sampling and Gas Chromatographic Analysis,1999] with gas chromatography/mass spectrometry and by gas chromatography with selective detectors. These baseline measures of waste stream content, labile organics, and microbial load in the volume F Shuttle trash provide data for waste

  14. Gorlin-Goltz Syndrome: A Rare Case

    PubMed Central

    Ganguly, Satyaki; Jaykar, Kranti C; Kumar, Rajesh; Jha, Abhijeet Kumar; Banerjee, PK

    2015-01-01

    Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome is characterized by multiple basocellular epitheliomas, keratocysts in the jaws, bifid ribs, palmar and/or plantar pits and ectopic calcifications of the falx cerebri. We describe a case of Gorlin-Goltz syndrome illustrating the importance of a thorough examination including the examination of palms and soles and detailed investigations in a patient having lesions suggestive of basal cell carcinoma and multiple naevi. PMID:25814758

  15. Liquid Secondary Waste Grout Formulation and Waste Form Qualification

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Um, Wooyong; Williams, B. D.; Snyder, Michelle M. V.

    This report describes the results from liquid secondary waste (LSW) grout formulation and waste form qualification tests performed at Pacific Northwest National Laboratory (PNNL) for Washington River Protection Solutions (WRPS) to evaluate new formulations for preparing a grout waste form with high-sulfate secondary waste simulants and the release of key constituents from these grout monoliths. Specific objectives of the LSW grout formulation and waste form qualification tests described in this report focused on five activities: 1.preparing new formulations for the LSW grout waste form with high-sulfate LSW simulants and solid characterization of the cured LSW grout waste form; 2.conducting themore » U.S. Environmental Protection Agency (EPA) Method 1313 leach test (EPA 2012) on the grout prepared with the new formulations, which solidify sulfate-rich Hanford Tank Waste Treatment and Immobilization Plant (WTP) off-gas condensate secondary waste simulant, using deionized water (DIW); 3.conducting the EPA Method 1315 leach tests (EPA 2013) on the grout monoliths made with the new dry blend formulations and three LSW simulants (242-A evaporator condensate, Environmental Restoration Disposal Facility (ERDF) leachate, and WTP off-gas condensate) using two leachants, DIW and simulated Hanford Integrated Disposal Facility (IDF) Site vadose zone pore water (VZPW); 4.estimating the 99Tc desorption K d (distribution coefficient) values for 99Tc transport in oxidizing conditions to support the IDF performance assessment (PA); 5.estimating the solubility of 99Tc(IV)-bearing solid phases for 99Tc transport in reducing conditions to support the IDF PA.« less

  16. A human neurodevelopmental model for Williams syndrome.

    PubMed

    Chailangkarn, Thanathom; Trujillo, Cleber A; Freitas, Beatriz C; Hrvoj-Mihic, Branka; Herai, Roberto H; Yu, Diana X; Brown, Timothy T; Marchetto, Maria C; Bardy, Cedric; McHenry, Lauren; Stefanacci, Lisa; Järvinen, Anna; Searcy, Yvonne M; DeWitt, Michelle; Wong, Wenny; Lai, Philip; Ard, M Colin; Hanson, Kari L; Romero, Sarah; Jacobs, Bob; Dale, Anders M; Dai, Li; Korenberg, Julie R; Gage, Fred H; Bellugi, Ursula; Halgren, Eric; Semendeferi, Katerina; Muotri, Alysson R

    2016-08-18

    Williams syndrome is a genetic neurodevelopmental disorder characterized by an uncommon hypersociability and a mosaic of retained and compromised linguistic and cognitive abilities. Nearly all clinically diagnosed individuals with Williams syndrome lack precisely the same set of genes, with breakpoints in chromosome band 7q11.23 (refs 1-5). The contribution of specific genes to the neuroanatomical and functional alterations, leading to behavioural pathologies in humans, remains largely unexplored. Here we investigate neural progenitor cells and cortical neurons derived from Williams syndrome and typically developing induced pluripotent stem cells. Neural progenitor cells in Williams syndrome have an increased doubling time and apoptosis compared with typically developing neural progenitor cells. Using an individual with atypical Williams syndrome, we narrowed this cellular phenotype to a single gene candidate, frizzled 9 (FZD9). At the neuronal stage, layer V/VI cortical neurons derived from Williams syndrome were characterized by longer total dendrites, increased numbers of spines and synapses, aberrant calcium oscillation and altered network connectivity. Morphometric alterations observed in neurons from Williams syndrome were validated after Golgi staining of post-mortem layer V/VI cortical neurons. This model of human induced pluripotent stem cells fills the current knowledge gap in the cellular biology of Williams syndrome and could lead to further insights into the molecular mechanism underlying the disorder and the human social brain.

  17. An overview on characterization, utilization and leachate analysis of biomedical waste incinerator ash.

    PubMed

    Rajor, Anita; Xaxa, Monika; Mehta, Ratika; Kunal

    2012-10-15

    Solid waste management is one of the major global environmental issues, as there is continuous increase in industrial globalization and generation of waste. Solid wastes encompass the heterogeneous mass of throwaways from the urban community as well as the homogeneous accumulations of agricultural, industrial and mineral wastes. Biomedical waste pose a significant impact on health and environment. A proper waste management system should be required to dispose hazardous biomedical waste and incineration should be the best available technology to reduce the volume of this hazardous waste. The incineration process destroys pathogens and reduces the waste volume and weight but leaves a solid material called biomedical waste ash as residue which increases the levels of heavy metals, inorganic salts and organic compounds in the environment. Disposal of biomedical waste ash in landfill may cause contamination of groundwater as metals are not destroyed during incineration. The limited space and the high cost for land disposal led to the development of recycling technologies and the reuse of ash in different systems. In order to minimize leaching of its hazardous components into the environment several studies confirmed the successful utilization of biomedical waste ash in agriculture and construction sector. This paper presents the overview on the beneficial use of ash in agriculture and construction materials and its leachate characteristics. This review also stressed on the need to further evaluate the leachate studies of the ashes and slag for their proper disposal and utilization. Copyright © 2012 Elsevier Ltd. All rights reserved.

  18. The 22q13.3 Deletion Syndrome (Phelan-McDermid Syndrome)

    PubMed Central

    Phelan, K.; McDermid, H.E.

    2012-01-01

    The 22q13.3 deletion syndrome, also known as Phelan-McDermid syndrome, is a contiguous gene disorder resulting from deletion of the distal long arm of chromosome 22. In addition to normal growth and a constellation of minor dysmorphic features, this syndrome is characterized by neurological deficits which include global developmental delay, moderate to severe intellectual impairment, absent or severely delayed speech, and neonatal hypotonia. In addition, more than 50% of patients show autism or autistic-like behavior, and therefore it can be classified as a syndromic form of autism spectrum disorders (ASD). The differential diagnosis includes Angelman syndrome, velocardiofacial syndrome, fragile X syndrome, and FG syndrome. Over 600 cases of 22q13.3 deletion syndrome have been documented. Most are terminal deletions of ∼100 kb to >9 Mb, resulting from simple deletions, ring chromosomes, and unbalanced translocations. Almost all of these deletions include the gene SHANK3 which encodes a scaffold protein in the postsynaptic densities of excitatory synapses, connecting membrane-bound receptors to the actin cytoskeleton. Two mouse knockout models and cell culture experiments show that SHANK3 is involved in the structure and function of synapses and support the hypothesis that the majority of 22q13.3 deletion syndrome neurological defects are due to haploinsufficiency of SHANK3, although other genes in the region may also play a role in the syndrome. The molecular connection to ASD suggests that potential future treatments may involve modulation of metabotropic glutamate receptors. PMID:22670140

  19. Fibromyalgia and chronic fatigue syndrome in children.

    PubMed

    Itoh, Yasuhiko; Shigemori, Tomoko; Igarashi, Tohru; Fukunaga, Yoshitaka

    2012-04-01

    Fibromyalgia (FM) is characterized by widespread persistent pain and the presence of multiple discrete tender points. Chronic fatigue syndrome (CFS) is a syndrome characterized by debilitating fatigue associated with a variable number of non-specific complaints. Because neither condition had necessarily been recognized in children until recently, those patients have been treated as having school refusal without being diagnosed as having either syndrome. There is a considerable overlap of clinical symptoms between these two syndromes. It is therefore controversial as to whether these syndromes have the same pathogenesis or not. The aim of the present study was to clarify the relationship between these syndromes in children. Fifteen patients with FM and 21 patients with CFS were investigated both clinically and immunologically. Immunological assessments included thorough analysis of autoantibodies using several techniques. Anti-nuclear antibody titers were higher and the prevalence of anti-Sa antibody was far more frequent in CFS patients than in FM patients. CFS and FM are different from each other at least in childhood, from an immunological aspect, although some patients could have both conditions. © 2011 The Authors. Pediatrics International © 2011 Japan Pediatric Society.

  20. Wastewater Characterization and Hazardous Waste Survey, Reese Air Force Base, Texas

    DTIC Science & Technology

    1988-04-01

    drain. The pH of caustic soda C’?- generally classifies this waste as hazardous. h. Personnel from the CE Power Production shop are neutralizing spent ...been changed out. A disposal practice for this waste will be formulated upon determination of whether or not the spent solvent is hazardous. Shop...sampling has been S performed to determine the characteristics of this waste. Spent rags are also thrown in the trash. Personnel also perform cadmium

  1. 1998 report on Hanford Site land disposal restrictions for mixed waste

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Black, D.G.

    1998-04-10

    This report was submitted to meet the requirements of Hanford Federal Facility Agreement and Consent Order (Tri-Party Agreement) Milestone M-26-01H. This milestone requires the preparation of an annual report that covers characterization, treatment, storage, minimization, and other aspects of managing land-disposal-restricted mixed waste at the Hanford Facility. The US Department of Energy, its predecessors, and contractors on the Hanford Facility were involved in the production and purification of nuclear defense materials from the early 1940s to the late 1980s. These production activities have generated large quantities of liquid and solid mixed waste. This waste is regulated under authority of bothmore » the Resource Conservation and Recovery Act of l976 and the Atomic Energy Act of 1954. This report covers only mixed waste. The Washington State Department of Ecology, US Environmental Protection Agency, and US Department of Energy have entered into the Tri-Party Agreement to bring the Hanford Facility operations into compliance with dangerous waste regulations. The Tri-Party Agreement required development of the original land disposal restrictions (LDR) plan and its annual updates to comply with LDR requirements for mixed waste. This report is the eighth update of the plan first issued in 1990. The Tri-Party Agreement requires and the baseline plan and annual update reports provide the following information: (1) Waste Characterization Information -- Provides information about characterizing each LDR mixed waste stream. The sampling and analysis methods and protocols, past characterization results, and, where available, a schedule for providing the characterization information are discussed. (2) Storage Data -- Identifies and describes the mixed waste on the Hanford Facility. Storage data include the Resource Conservation and Recovery Act of 1976 dangerous waste codes, generator process knowledge needed to identify the waste and to make LDR determinations

  2. TTP AL921102: An integrated geophysics program for non-intrusive characterization of mixed-waste landfill sites. FY 1992 year-end progress report: Volume 1

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hasbrouck, J.C.

    1992-11-01

    Chem-Nuclear Geotech, Inc. (Geotech), operating contractor for the US Department of Energy Grand Junction Projects Office, is conducting the Integrated Geophysics Program for Non-Intrusive Characterization of Mixed-Waste Landfill Sites (Technical Task Plan [TTP] AL921102). The TTP is part of the Mixed-Waste Landfill Integrated Demonstration (MWLID). The objective of this task was to demonstrate that an integrated program of surface geophysics can be used to effectively and nonintrusively characterize n-mixed-waste landfill sites. To accomplish this objective, integrated field demonstrations were conducted over two previously identified areas of interest (designated Areas A and B) within the MWLID test site at the Chemicalmore » Waste Landfill (CWL), Technical Area 3, at the Sandia National Laboratories, Albuquerque, New Mexico (Figures 1 and 2). Area A was centered roughly around the Chromic Acid and Organics Pits in the southeast-central portion of the landfill and Area B was centered around the ``60`s Pits`` area in the northeast-central portion of the landfill. Pit locations were known in Area A and suspected in Area B. This progress report describes the geophysical surveys conducted by Geotech and presents preliminary displays and analyses. Volume 2 of this report contains the raw data for all the surveys conducted by Geotech for this TTP.« less

  3. Bardet-Biedl syndrome and Usher syndrome.

    PubMed

    Koenig, Rainer

    2003-01-01

    Bardet-Biedl syndrome (BBS) and Usher syndrome (USH) are the most prevalent syndromic forms of retinitis pigmentosa (RP), together they make up almost a quarter of the patients with RP. BBS is defined by the association of retinopathy, obesity, hypogonadism, renal dysfunction, postaxial polydactyly and mental retardation. This clinically complex syndrome is genetically heterogeneous with linkage to more than 6 loci, and 4 genes have been cloned so far. Recent molecular data present evidence that, in some instances, the clinical manifestation of BBS requires recessive mutations in 1 of the 6 BBS loci plus one or two additional mutations in a second BBS locus (tri- or tetra-allelic inheritance). USH is characterized by the combination of congenital or early-onset sensorineural deafness, RP, and variable degrees of vestibular dysfunction. Each of the three clinical types is genetically heterogeneous: 7 loci have been mapped for type 1, three loci for type 2, and two loci for type 3. Currently, 6 USH genes (MYO7A, USH1C, CDH23, PCDH15, USH2A, USH3) have been identified. Pathogenetically, mutations of the USH1 genes seem to result in defects of auditory and retinal sensory cells, the USH 2 phenotype is caused by defects of extracellular matrix or cell surface receptor proteins, and USH3 may be due to synaptic disturbances. The considerable contribution of syndromic forms of RP requires interdisciplinary approaches to the clinical and diagnostic management of RP patients.

  4. Characterizing food waste substrates for co-digestion through biochemical methane potential (BMP) experiments.

    PubMed

    Lisboa, Maria Sol; Lansing, Stephanie

    2013-12-01

    Co-digestion of food waste with dairy manure is increasingly utilized to increase energy production and make anaerobic digestion more affordable; however, there is a lack of information on appropriate co-digestion substrates. In this study, biochemical methane potential (BMP) tests were conducted to determine the suitability of four food waste substrates (meatball, chicken, cranberry and ice cream processing wastes) for co-digestion with flushed dairy manure at a ratio of 3.2% food waste and 96.8% manure (by volume), which equated to 14.7% (ice-cream) to 80.7% (chicken) of the VS being attributed to the food waste. All treatments led to increases in methane production, ranging from a 67.0% increase (ice cream waste) to a 2940% increase (chicken processing waste) compared to digesting manure alone, demonstrating the large potential methane production of food waste additions compared to relatively low methane production potential of the flushed dairy manure, even if the overall quantity of food waste added was minimal. Copyright © 2013 Elsevier Ltd. All rights reserved.

  5. [Cyclic Cushing's Syndrome - rare or rarely recognized].

    PubMed

    Kiałka, Marta; Doroszewska, Katarzyna; Mrozińska, Sandra; Milewicz, Tomasz; Stochmal, Ewa

    2015-01-01

    Cyclic Cushing's syndrome is a type of Cushing's disease which is characterized by alternating periods of increasing and decreasing levels of cortisol in the blood. The diagnostic criteria for cyclic Cushing's syndrome are at least three periods of hypercortisolism alternating with at least two episodes of normal levels of serum cortisol concentration. The epidemiology, signs, symptoms, pathogenesis and treatment of cyclic Cushing's syndrome have been discussed.

  6. Down Syndrome - Genetics and Cardiogenetics.

    PubMed

    Plaiasu, Vasilica

    2017-09-01

    During the last years, Down syndrome has been the focus of special attention. Down syndrome is a genetic disorder characterized by distinct physical features and some degree of cognitive disability. Patients with Down syndrome also present many other congenital anomalies. The mapping for phenotypes to specific regions of chromosome 21 permits to identify which genes (or small regions) contribute to the phenotypic features of Down syndrome and thus, to understand its pathogenesis. Mainly there are three cytogenetic forms of Down syndrome: free trisomy 21, mosaic trisomy 21 and robertsonian translocation trisomy 21. Prenatal and postnatal testing has become commonly used to diagnose different cases presenting the same pathology. Early clinical diagnosis is extremely important for patient prognosis. Lately, advances in Down syndrome research have been registered, but little is known about cardiovascular phenotype in Down syndrome. About half of patients with Down syndrome have congenital heart disease, and atrioventricular septal defects are the most common defects found. Basic research on Down syndrome is now rapidly accelerating, using new genomic technologies. There were many studies performed to identify a correlation between genotype and phenotype in Down syndrome.

  7. Comprehensive waste characterization and organic pollution co-occurrence in a Hg and As mining and metallurgy brownfield.

    PubMed

    Gallego, J R; Esquinas, N; Rodríguez-Valdés, E; Menéndez-Aguado, J M; Sierra, C

    2015-12-30

    The abandonment of Hg-As mining and metallurgy sites, together with long-term weathering, can dramatically degrade the environment. In this work it is exemplified the complex legacy of contamination that afflicts Hg-As brownfields through the detailed study of a paradigmatic site. Firstly, an in-depth study of the former industrial process was performed to identify sources of different types of waste. Subsequently, the composition and reactivity of As- and Hg-rich wastes (calcines, As-rich soot, stupp, and flue dust) was analyzed by means of multielemental analysis, mineralogical characterization (X-ray diffraction, electronic, and optical microscopy, microbrobe), chemical speciation, and sequential extractions. As-rich soot in the form of arsenolite, a relatively mobile by-product of the pyrometallurgical process, and stupp, a residue originated in the former condensing system, were determined to be the main risk at the site. In addition, the screening of organic pollution was also aimed, as shown by the outcome of benzo(a) pyrene and other PAHs, and by the identification of unexpected Hg organo-compounds (phenylmercury propionate). The approach followed unravels evidence from waste from the mining and metallurgy industry that may be present in other similar sites, and identifies unexpected contaminants overlooked by conventional analyses. Copyright © 2015 Elsevier B.V. All rights reserved.

  8. Further Characterization of CELSS Wastes: A Review of Solid Wastes Present to Support Potential Secondary Biomass Production

    NASA Technical Reports Server (NTRS)

    Muller, Matthew S.

    1996-01-01

    Controlled ecological life support systems (CELSS) may one day play an essential role in extraterrestrial colonies. Key to the success of any CELSS will be the system's ability to approach a self-supporting status through recovery and reuse of basic resources. Primary CELSS solid wastes with potential to support secondary biomass production will be inedible plant biomass and metabolic human wastes. Solid waste production is summarized and reported as 765 g N per day per person, including 300 g C and 37 g N per day per person. One Resource Recovery configuration using the bioprocessing of solid wastes into a Tilapia feed stream is examined. Based on estimated conversion efficiencies, 12 g of protein per day per person is produced as a nutrition supplement. The unique tissue composition of crops produced at the Kennedy Space Center CELSS Program highlights the need to evaluate Resource Recovery components with data generated in the CELSS environment.

  9. Development of new materials from waste electrical and electronic equipment: Characterization and catalytic application.

    PubMed

    Souza, J P; Freitas, P E; Almeida, L D; Rosmaninho, M G

    2017-07-01

    Wastes of electrical and electronic equipment (WEEE) represent an important environmental problem, since its composition includes heavy metals and organic compounds used as flame-retardants. Thermal treatments have been considered efficient processes on removal of these compounds, producing carbonaceous structures, which, together with the ceramic components of the WEEE (i.e. silica and alumina), works as support material for the metals. This mixture, associated with the metals present in WEEE, represents promising systems with potential for catalytic application. In this work, WEEE was thermally modified to generate materials that were extensively characterized. Raman spectrum for WEEE after thermal treatment showed two carbon associated bands. SEM images showed a metal nanoparticles distribution over a polymeric and ceramic support. After characterization, WEEE materials were applied in ethanol steam reforming reaction. The system obtained at higher temperature (800°C) exhibited the best activity, since it leads to high conversions (85%), hydrogen yield (30%) and H 2 /CO ratio (3,6) at 750°C. Copyright © 2017 Elsevier Ltd. All rights reserved.

  10. Nuclear waste management. Semiannual progress report, October 1982-March 1983

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Chikalla, T.D.; Powell, J.A.

    1983-06-01

    This document is one of a series of technical progress reports designed to report radioactive waste management programs at the Pacific Northwest Laboratory. Accomplishments in the following programs are reported: waste stabilization; Materials Characterization Center; waste isolation; low-level waste management; remedial action; and supporting studies.

  11. Microstructure and wear characterization of aluminum matrix composites reinforced with industrial waste fly ash particulates synthesized by friction stir processing

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Dinaharan, I., E-mail: dinaweld2009@gmail.com

    Fly ash (FA) is a waste product of coal combustion in thermal power plants which is available in massive quantities all over the world causing land pollution. This paper reports the characterization of AA6061 aluminum matrix composites (AMCs) reinforced with FA particles synthesized using friction stir processing (FSP). The volume fraction of FA particles was varied from 0 to 18 in steps of 6. The prepared AMCs were characterized using optical microscopy (OM), scanning electron microscopy (SEM) and electron backscattered diagram (EBSD). The wear rate was estimated using a pin-on-disc wear apparatus. FA particles were observed to be distributed homogeneouslymore » in the AMC irrespective of the location within the stir zone. The EBSD micrographs revealed remarkable grain refinement in the AMC. The incorporation of FA particles enhanced the microhardness and wear resistance of the AMC. The strengthening mechanisms of the AMC were discussed and correlated to the observed microstructures. The wear mechanisms were identified by characterizing the wear debris and worn surfaces. - Highlights: •Industrial waste fly ash was used to produce aluminum matrix composites. •Friction stir processing was used to produce AA6061/Fly Ash composite. •Fly ash particles refined the grains of aluminum matrix. •Fly ash particles enhanced the hardness and wear resistance. •Successful utilization of fly ash to make aluminum composites reduces land pollution.« less

  12. Radiological features of familial Gorlin-Goltz syndrome.

    PubMed

    Hegde, Shruthi; Shetty, Shishir Ram

    2012-03-01

    Gorlin-Goltz syndrome is an autosomal dominant disorder principally characterized by cutaneous basal cell carcinomas, multiple keratocystic odontogenic tumors, and skeletal anomalies. This syndrome may be diagnosed early by dentist because keratocystic odontogenic tumors are usually one of the first manifestations of the syndrome. Early diagnosis and treatment are of utmost importance in reducing the severity of long term sequelae of this syndrome. This report presents a rare event of Gorlin-Goltz syndrome occurring in a 39-year-old male and his 8-year-old daughter. The clinical and investigative features of this familial disorder has been described in detail.

  13. Radiological features of familial Gorlin-Goltz syndrome

    PubMed Central

    Shetty, Shishir Ram

    2012-01-01

    Gorlin-Goltz syndrome is an autosomal dominant disorder principally characterized by cutaneous basal cell carcinomas, multiple keratocystic odontogenic tumors, and skeletal anomalies. This syndrome may be diagnosed early by dentist because keratocystic odontogenic tumors are usually one of the first manifestations of the syndrome. Early diagnosis and treatment are of utmost importance in reducing the severity of long term sequelae of this syndrome. This report presents a rare event of Gorlin-Goltz syndrome occurring in a 39-year-old male and his 8-year-old daughter. The clinical and investigative features of this familial disorder has been described in detail. PMID:22474649

  14. Gorlin-Goltz syndrome.

    PubMed

    Joshi, Priya Shirish; Deshmukh, Vijay; Golgire, Someshwar

    2012-01-01

    Gorlin-Goltz syndrome is an uncommon autosomal dominant inherited disorder, which is characterized by multiple odontogenic Keratocysts and basal cell carcinomas, skeletal, dental, ophthalmic, and neurological abnormalities, intracranial ectopic calcifications of the falx cerebri, and facial dysmorphism. Pathogenesis of the syndrome is attributed to abnormalities in the long arm of chromosome 9 (q22.3-q31) and loss or mutations of human patched gene (PTCH1 gene). Diagnosis is based upon established major and minor clinical and radiological criteria and ideally confirmed by deoxyribo nucleic acid analysis. We report a case of a 9-year-old girl presenting with three major and one minor feature of Gorlin-Goltz syndrome. Radiologic findings of the syndrome are easily identifiable on Orthopantomogram, chest X-ray, and Computed tomography scans. These investigations prompt an early verification of the disease, which is very important to prevent recurrence and better survival rates from the coexistent diseases.

  15. DOORS syndrome: phenotype, genotype and comparison with Coffin-Siris syndrome.

    PubMed

    Campeau, Philippe M; Hennekam, Raoul C

    2014-09-01

    DOORS syndrome (Deafness, Onychodystrophy, Osteodystrophy, mental Retardation, Seizures) is characterized mainly by sensorineural deafness, shortened terminal phalanges with small nails of hands and feet, intellectual deficiency, and seizures. Half of the patients with all clinical features have mutations in TBC1D24. We review here the manifestations of patients clinically diagnosed with DOORS syndrome. In this cohort of 32 families (36 patients) we detected 13 individuals from 10 families with TBC1D24 mutations. Subsequent whole exome sequencing in the cohort showed the same de novoSMARCB1 mutation (c.1130G>A), known to cause Coffin-Siris syndrome, in two patients. Distinguishing features include retinal anomalies, Dandy-Walker malformation, scoliosis, rocker bottom feet, respiratory difficulties and absence of seizures, and 2-oxoglutaric aciduria in the patients with the SMARCB1 mutation. We briefly discuss the heterogeneity of the DOORS syndrome phenotype and the differential diagnosis of this condition. © 2014 Wiley Periodicals, Inc.

  16. SKIV2L Mutations Cause Syndromic Diarrhea, or Trichohepatoenteric Syndrome

    PubMed Central

    Fabre, Alexandre; Charroux, Bernard; Martinez-Vinson, Christine; Roquelaure, Bertrand; Odul, Egritas; Sayar, Ersin; Smith, Hilary; Colomb, Virginie; Andre, Nicolas; Hugot, Jean-Pierre; Goulet, Olivier; Lacoste, Caroline; Sarles, Jacques; Royet, Julien; Levy, Nicolas; Badens, Catherine

    2012-01-01

    Syndromic diarrhea (or trichohepatoenteric syndrome) is a rare congenital bowel disorder characterized by intractable diarrhea and woolly hair, and it has recently been associated with mutations in TTC37. Although databases report TTC37 as being the human ortholog of Ski3p, one of the yeast Ski-complex cofactors, this lead was not investigated in initial studies. The Ski complex is a multiprotein complex required for exosome-mediated RNA surveillance, including the regulation of normal mRNA and the decay of nonfunctional mRNA. Considering the fact that TTC37 is homologous to Ski3p, we explored a gene encoding another Ski-complex cofactor, SKIV2L, in six individuals presenting with typical syndromic diarrhea without variation in TTC37. We identified mutations in all six individuals. Our results show that mutations in genes encoding cofactors of the human Ski complex cause syndromic diarrhea, establishing a link between defects of the human exosome complex and a Mendelian disease. PMID:22444670

  17. Chemical characterization, nano-particle mineralogy and particle size distribution of basalt dust wastes.

    PubMed

    Dalmora, Adilson C; Ramos, Claudete G; Oliveira, Marcos L S; Teixeira, Elba C; Kautzmann, Rubens M; Taffarel, Silvio R; de Brum, Irineu A S; Silva, Luis F O

    2016-01-01

    Understanding the geochemistry of basalt alteration is central to the study of agriculture systems. Various nano-minerals play an important role in the mobilization of contaminants and their subsequent uptake by plants. We present a new analytical experimental approach in combination with an integrated analytical protocol designed to study basalt alteration processes. Recently, throughout the world, ultra-fine and nano-particles derived from basalt dust wastes (BDW) during "stonemeal" soil fertilizer application have been of great concern for their possible adverse effects on human health and environmental pollution. Samples of BDW utilized were obtained from companies in the Nova Prata mining district in southern Brazil for chemical characterization and nano-mineralogy investigation, using an integrated application of advanced characterization techniques such as X-ray diffraction (XRD), High Resolution-Transmission Electron microscopy (HR-TEM)/Energy Dispersive Spectroscopy (EDS)/(selected-area diffraction pattern) SAED, Field Emission-Scanning Electron Microscopy (FE-SEM/EDS), and granulometric distribution analysis. The investigation has revealed that BDW materials are dominated by SiO2, Al2O3, and Fe2O3, with a complex micromineralogy including alkali feldspar, augite, barite, labradorite, hematite, heulandrite, gypsum, kaolinite, quartz, and smectite. In addition, we have identified a number of trace metals such as Cd, Cu, Cr, and Zn, that are preferentially concentrated into the finer, inhalable, dust fraction and, thus, could present a health hazard in the urban areas around the basalt mining zone. The implication of this observation is that use of these nanometric-sized particulates as soil fertilizer may present different health challenges to those of conventional fertilizers, inviting future work regarding the relative toxicities of these materials. Our investigation on the particle size distribution, nano-particle mineralogy and chemical composition in

  18. Myelodysplastic syndrome precedes the onset of remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome.

    PubMed

    Matsunaga, Takafumi; Izumi, Yasumori; Iwanaga, Nozomi; Kawahara, Chieko; Shigemitsu, Yoshika; Yoshida, Shinichiro; Kawakami, Atsushi; Ogawa, Daisuke; Migita, Kiyoshi

    2015-01-01

    Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome is characterized by symmetrical synovitis predominantly involving the wrists, and is associated with marked pitting edema of the dorsum of the hands. Although the etiology of RS3PE syndrome is still unknown, several putative associations with malignancies and hematological disorders have been reported. Myelodysplastic syndrome (MDS) is characterized by infective hematopoiesis with possible transformation to leukemia; however, an association between RS3PE syndrome and MDS has been rarely reported. Here, we describe a 67-year-old man with MDS with refractory anemia who developed RS3PE syndrome 3 months after the diagnosis of MDS. The patient presented with polyarthritis with pitting edema at the dorsum of the hands, the elevated serum levels of C-reactive protein and a proinflammatory cytokine, interleukin-6, and the elevated plasma levels of vascular endothelial growth factor (VEGF). VEGF has been shown to be involved in the pathogenesis of RS3PE syndrome. Treatment with low doses of corticosteroids resulted in the regression of polyarthritis and pitting edema of the dorsum of the hands, as well as a reduction in the elevated levels of plasma VEGF. Partial resolution of refractory anemia was also observed with steroid therapy. In summary, RS3PE syndrome developed shortly after MDS was identified in this patient. The sequence of clinical events suggests that MDS-mediated immunological abnormalities including inflammatory cytokine induction may be responsible for the association between MDS and RS3PE syndrome. Patients with RS3PE syndrome should be screened for hematological disorders that promote proinflammatory mediators.

  19. NEVADA TEST SITE WASTE ACCEPTANCE CRITERIA, JUNE 2006

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    U.S. DEPARTMENT OF ENERGY, NATIONAL NUCLEAR SECURITY ADMINISTRATION NEVADA SITE OFFICE

    This document establishes the U.S. Department of Energy (DOE), National Nuclear Security Administration Nevada Site Office (NNSA/NSO) waste acceptance criteria (WAC). The WAC provides the requirements, terms, and conditions under which the Nevada Test Site (NTS) will accept low-level radioactive (LLW) and mixed waste (MW) for disposal. It includes requirements for the generator waste certification program, characterization, traceability, waste form, packaging, and transfer. The criteria apply to radioactive waste received at the NTS Area 3 and Area 5 Radioactive Waste Management Complex (RWMC) for storage or disposal.

  20. Kindler syndrome.

    PubMed

    Ashton, G H S

    2004-03-01

    Kindler syndrome is a rare, autosomal recessive skin fragility disorder characterized by blistering in infancy, followed by photosensitivity and progressive poikiloderma. Ultrastructural examination reveals marked basement membrane reduplication and variable levels of cleavage at the dermal-epidermal junction. The molecular pathology underlying Kindler syndrome has recently been shown to involve loss-of-function mutations in a novel gene, KIND1, encoding kindlin-1. Immunofluorescence, gene expression and cell biology studies have shown that kindlin-1 is expressed mainly in basal keratinocytes and plays a role in the attachment of the actin cytoskeleton via focal contacts to the extracellular matrix. Thus, Kindler syndrome is the first genodermatosis caused by a defect in actin-extracellular matrix linkage rather than the classic keratin-extracellular matrix linkage underlying the pathology of other inherited skin fragility disorders such as epidermolysis bullosa. This article reviews the clinical features as well as the molecular and cellular pathology of Kindler syndrome and highlights the importance of the new protein, kindlin-1, in cell-matrix adhesion and its intriguing link to photosensitivity.

  1. Muscle wasting in cancer: the role of mitochondria.

    PubMed

    Argilés, Josep M; López-Soriano, Francisco J; Busquets, Silvia

    2015-05-01

    The aim of the present review is to examine the impact of mitochondrial dysfunction in cancer cachexia. Oxidative pathways are altered in this tissue during muscle wasting and this seems to be a consequence of mitochondrial abnormalities that include altered morphology and function, decreased ATP synthesis and uncoupling. An alteration of energy balance is the immediate cause of cachexia. Both alterations in energy intake and expenditure are responsible for the wasting syndrome associated with different types of pathological conditions, such as cancer. Different types of molecular mechanisms contribute to energy expenditure and, therefore, involuntary body weight loss, one of which is mitochondrial dysfunction.

  2. Hallermann-Streiff Syndrome

    PubMed Central

    Thomas, Jayakar; Ragavi, B Sindhu; Raneesha, PK; Ahmed, N Ashwak; Cynthia, S; Manoharan, D; Manoharan, R

    2013-01-01

    Hallermann-Streiff syndrome (HSS) is a rare disorder characterized by dyscephalia, with facial and dental abnormalities. We report a 12-year-old female child who presented with abnormal facial features, dental abnormalities and sparse scalp hair. PMID:24082185

  3. U.S. Geological Survey research in radioactive waste disposal - Fiscal years 1986-1990

    USGS Publications Warehouse

    Trask, N.J.; Stevens, P.R.

    1991-01-01

    The report summarizes progress on geologic and hydrologic research related to the disposal of radioactive wastes. The research efforts are categorized according to whether they are related most directly to: (1) high-level wastes, (2) transuranic wastes, (3) low-level and mixed low-level and hazardous wastes, or (4) uranium mill tailings. Included is research applicable to the identification and geohydrologic characterization of waste-disposal sites, to investigations of specific sites where wastes have been stored, to development of techniques and methods for characterizing disposal sites, and to studies of geologic and hydrologic processes related to the transport and/or retention of waste radionuclides.

  4. Myostatin gene inactivation prevents skeletal muscle wasting in cancer.

    PubMed

    Gallot, Yann S; Durieux, Anne-Cécile; Castells, Josiane; Desgeorges, Marine M; Vernus, Barbara; Plantureux, Léa; Rémond, Didier; Jahnke, Vanessa E; Lefai, Etienne; Dardevet, Dominique; Nemoz, Georges; Schaeffer, Laurent; Bonnieu, Anne; Freyssenet, Damien G

    2014-12-15

    Cachexia is a muscle-wasting syndrome that contributes significantly to morbidity and mortality of many patients with advanced cancers. However, little is understood about how the severe loss of skeletal muscle characterizing this condition occurs. In the current study, we tested the hypothesis that the muscle protein myostatin is involved in mediating the pathogenesis of cachexia-induced muscle wasting in tumor-bearing mice. Myostatin gene inactivation prevented the severe loss of skeletal muscle mass induced in mice engrafted with Lewis lung carcinoma (LLC) cells or in Apc(Min) (/+) mice, an established model of colorectal cancer and cachexia. Mechanistically, myostatin loss attenuated the activation of muscle fiber proteolytic pathways by inhibiting the expression of atrophy-related genes, MuRF1 and MAFbx/Atrogin-1, along with autophagy-related genes. Notably, myostatin loss also impeded the growth of LLC tumors, the number and the size of intestinal polyps in Apc(Min) (/+) mice, thus strongly increasing survival in both models. Gene expression analysis in the LLC model showed this phenotype to be associated with reduced expression of genes involved in tumor metabolism, activin signaling, and apoptosis. Taken together, our results reveal an essential role for myostatin in the pathogenesis of cancer cachexia and link this condition to tumor growth, with implications for furthering understanding of cancer as a systemic disease. ©2014 American Association for Cancer Research.

  5. Moyamoya syndrome in a patient with Noonan-like syndrome with loose anagen hair.

    PubMed

    Choi, Jin-Ho; Oh, Moon-Yeon; Yum, Mi-Sun; Lee, Beom Hee; Kim, Gu-Hwan; Yoo, Han-Wook

    2015-03-01

    Noonan-like syndrome with loose anagen hair is one of the RASopathies characterized by Noonan syndrome-like features with unique ectodermal abnormalities. This syndrome is caused by mutations in the SHOC2 gene. We encountered a patient with moyamoya syndrome associated with Noonan-like syndrome with loose anagen hair presenting with transient ischemic attacks. A 6-year-old girl was diagnosed with Noonan-like syndrome with loose anagen hair because of profound short stature and ectodermal anomalies such as sparse and easily pluckable hair. A heterozygous mutation of c.4A>G (p.S2G) in the SHOC2 gene was identified, and recombinant human growth hormone therapy was initiated at 8 years of age. At age 10, she manifested recurrent left hemiplegia. Moreover, cerebrovascular imaging revealed occlusion or narrowing of both internal carotid arteries and both middle cerebral arteries with distal moyamoya-like vessels. She is treated with aspirin and calcium channel blocker. We describe the first case of Noonan-like syndrome with loose anagen hair associated with moyamoya syndrome, although it has been reported to be associated with a few cases of other RASopathies, including Noonan, cardiofaciocutaneous, and Costello syndromes. This report emphasizes the associations between cerebrovascular anomalies and Noonan-like syndrome with loose anagen hair. Copyright © 2015 Elsevier Inc. All rights reserved.

  6. Waste minimization for commercial radioactive materials users generating low-level radioactive waste

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Fischer, D.K.; Gitt, M.; Williams, G.A.

    1991-07-01

    The objective of this document is to provide a resource for all states and compact regions interested in promoting the minimization of low-level radioactive waste (LLW). This project was initiated by the Commonwealth of Massachusetts, and Massachusetts waste streams have been used as examples; however, the methods of analysis presented here are applicable to similar waste streams generated elsewhere. This document is a guide for states/compact regions to use in developing a system to evaluate and prioritize various waste minimization techniques in order to encourage individual radioactive materials users (LLW generators) to consider these techniques in their own independent evaluations.more » This review discusses the application of specific waste minimization techniques to waste streams characteristic of three categories of radioactive materials users: (1) industrial operations using radioactive materials in the manufacture of commercial products, (2) health care institutions, including hospitals and clinics, and (3) educational and research institutions. Massachusetts waste stream characterization data from key radioactive materials users in each category are used to illustrate the applicability of various minimization techniques. The utility group is not included because extensive information specific to this category of LLW generators is available in the literature.« less

  7. The Architecture of the Pollen Hoarding Syndrome in Honey Bees: Implications for Understanding Social Evolution, Behavioral Syndromes, and Selective Breeding

    PubMed Central

    Rueppell, Olav

    2014-01-01

    Social evolution has influenced every aspect of contemporary honey bee biology, but the details are difficult to reconstruct. The reproductive ground plan hypothesis of social evolution proposes that central regulators of the gonotropic cycle of solitary insects have been coopted to coordinate social complexity in honey bees, such as the division of labor among workers. The predicted trait associations between reproductive physiology and social behavior have been identified in the context of the pollen hoarding syndrome, a larger suite of interrelated traits. The genetic architecture of this syndrome is characterized by a partially overlapping genetic architecture with several consistent, pleiotropic QTL. Despite these central QTL and an integrated hormonal regulation, separate aspects of the pollen hoarding syndrome may evolve independently due to peripheral QTL and additionally segregating genetic variance. The characterization of the pollen hoarding syndrome has also demonstrated that this syndrome involves many non-behavioral traits, which may be the case for numerous “behavioral” syndromes. Furthermore, the genetic architecture of the pollen hoarding syndrome has implications for breeding programs for improving honey health and other desirable traits: If these traits are comparable to the pollen hoarding syndrome, consistent pleiotropic QTL will enable marker assisted selection, while sufficient additional genetic variation may permit the dissociation of trade-offs for efficient multiple trait selection. PMID:25506100

  8. Language and Literacy Development of Children with Williams Syndrome

    ERIC Educational Resources Information Center

    Mervis, Carolyn B.

    2009-01-01

    Williams syndrome is a rare neurodevelopmental disorder caused by deletion of approximately 25 genes on chromosome 7q11.23. Children with the syndrome evidence large individual differences in both broad language and reading abilities. Nevertheless, as a group, children with this syndrome show a consistent pattern characterized by relative…

  9. Fat, oil and grease waste from municipal wastewater: characterization, activation and sustainable conversion into biofuel.

    PubMed

    Pastore, Carlo; Pagano, Michele; Lopez, Antonio; Mininni, Giuseppe; Mascolo, Giuseppe

    2015-01-01

    Fat, oil and grease (FOG) recovered by the oil/water separator of a wastewater treatment plant (WWTP) were sampled, characterized, activated and converted into biofuel. Free acids (50-55%) and fatty soaps (26-32%) not only composed the main components, but they were also easily separable from the starting waste. The respective free fatty acid profiles were gas-chromatographically evaluated, interestingly verifying that free acids had a different profile (mainly oleic acid) with respect to the soapy fraction (saturated fatty acids were dominant). The inorganic composition was also determined for soaps, confirming that calcium is the most commonly present metal. The chemical activation of this fatty waste was made possible by converting the starting soaps into the respective free fatty acids by using formic acid as activator, coproducing the relevant formates. The activated fatty matter was then converted into biofuel through direct esterification under very mild conditions (345 K, atmospheric pressure) and obtaining thermodynamic conversion in less than 2 h. The process was easily scaled up, isolating at the end pure biodiesel (purity > 96%) through distillation under vacuum, providing a final product conformed to commercial purposes.

  10. Characterization and Leaching Tests of the Fluidized Bed Steam Reforming (FBSR) Waste Form for LAW Immobilization

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Neeway, James J.; Qafoku, Nikolla; Brown, Christopher F.

    2013-10-01

    Several supplemental technologies for treating and immobilizing Hanford low activity waste (LAW) have been evaluated. One such immobilization technology is the Fluidized Bed Steam Reforming (FBSR) granular product. The FBSR granular product is composed of insoluble sodium aluminosilicate (NAS) feldspathoid minerals. Production of the FBSR mineral product has been demonstrated both at the industrial and laboratory scale. Pacific Northwest National Laboratory (PNNL) was involved in an extensive characterization campaign. This goal of this campaign was study the durability of the FBSR mineral product and the mineral product encapsulated in a monolith to meet compressive strength requirements. This paper gives anmore » overview of results obtained using the ASTM C 1285 Product Consistency Test (PCT), the EPA Test Method 1311 Toxicity Characteristic Leaching Procedure (TCLP), and the ASTMC 1662 Single-Pass Flow-Through (SPFT) test. Along with these durability tests an overview of the characteristics of the waste form has been collected using Scanning Electron Microscopy (SEM), X-ray Diffraction (XRD), microwave digestions for chemical composition, and surface area from Brunauer, Emmett, and Teller (BET) theory.« less

  11. Quantitative, competitive PCR analysis of porcine circovirus DNA in serum from pigs with postweaning multisystemic wasting syndrome.

    PubMed

    Liu, Q; Wang, L; Willson, P; Babiuk, L A

    2000-09-01

    A competitive PCR (cPCR) assay was developed for monitoring porcine circovirus (PCV) DNA in serum samples from piglets. The cPCR was based on competitive coamplification of a 502- or 506-bp region of the PCV type 1 (PCV1) or PCV2 ORF2, respectively, with a known concentration of competitor DNA, which produced a 761- or 765-bp fragment, respectively. The cPCR was validated by quantification of a known amount of PCV wild-type plasmids. We also used this technique to determine PCV genome copy numbers in infected cells. Furthermore, we measured PCV DNA loads in clinical samples. More than 50% of clinically healthy piglets could harbor both types of PCV. While PCV1 was detected in only 3 of 16 pigs with postweaning multisystemic wasting syndrome (PMWS), all the sick piglets contained PCV2. A comparison of the PCV2 DNA loads of healthy and sick animals revealed a significant difference, indicating that the development of PMWS may require a certain amount of PCV2.

  12. Rikkunshito, a ghrelin potentiator, ameliorates anorexia–cachexia syndrome

    PubMed Central

    Fujitsuka, Naoki; Uezono, Yasuhito

    2014-01-01

    Anorexia–cachexia syndrome develops during the advanced stages of various chronic diseases in which patients exhibit a decreased food intake, weight loss, and muscle tissue wasting. For these patients, this syndrome is a critical problem leading to an increased rate of morbidity and mortality. The present pharmacological therapies for treating anorexia–cachexia have limited effectiveness. The Japanese herbal medicine rikkunshito is often prescribed for the treatment of anorexia and upper gastrointestinal (GI) disorders. Thus, rikkunshito is expected to be beneficial for the treatment of patients with anorexia–cachexia syndrome. In this review, we summarize the effects of rikkunshito and its mechanisms of action on anorexia–cachexia. Persistent loss of appetite leads to a progressive depletion of body energy stores, which is frequently associated with cachexia. Consequently, regulating appetite and energy homeostasis is critically important for treating cachexia. Ghrelin is mainly secreted from the stomach, and it plays an important role in initiating feeding, controlling GI motility, and regulating energy expenditure. Recent clinical and basic science studies have demonstrated that the critical mechanism of rikkunshito underlies endogenous ghrelin activity. Interestingly, several components of rikkunshito target multiple gastric and central sites, and regulate the secretion, receptor sensitization, and degradation of ghrelin. Rikkunshito is effective for the treatment of anorexia, body weight loss, muscle wasting, and anxiety-related behavior. Furthermore, treatment with rikkunshito was observed to prolong survival in an animal model of cachexia. The use of a potentiator of ghrelin signaling, such as rikkunshito, may represent a novel approach for the treatment of anorexia–cachexia syndrome. PMID:25540621

  13. Genetic modifiers of Velo- cardio- facial syndrome/DiGeorge syndrome

    PubMed Central

    Aggarwal, Vimla S.; Morrow, Bernice E.

    2009-01-01

    Velo-cardio-facial syndrome/DiGeorge syndrome (VCFS/DGS), the most common micro-deletion disorder in humans, is characterized by craniofacial, parathyroid and thymic defects as well as cardiac outflow tract malformations. Most patients have a similar hemizygous 3 million base pair deletion on 22q11.2. Studies in mouse have shown that Tbx1, a T- box containing transcription factor present on the deleted region, is likely responsible for the etiology of the syndrome. Furthermore, mutations in TBX1 have been found in rare non-deleted patients. Despite having the same sized deletion, most VCFS/DGS patients exhibit significant clinical variability. Stochastic, environmental and genetic factors likely modify the phenotype of patients with the disorder. Here, we review mouse genetics studies which may help identify genetic modifiers for VCFS/DGS. PMID:18636633

  14. Loss of Dermatan-4-Sulfotransferase 1 Function Results in Adducted Thumb-Clubfoot Syndrome

    PubMed Central

    Dündar, Munis; Müller, Thomas; Zhang, Qi; Pan, Jing; Steinmann, Beat; Vodopiutz, Julia; Gruber, Robert; Sonoda, Tohru; Krabichler, Birgit; Utermann, Gerd; Baenziger, Jacques U.; Zhang, Lijuan; Janecke, Andreas R.

    2009-01-01

    Adducted thumb-clubfoot syndrome is an autosomal-recessive disorder characterized by typical facial appearance, wasted build, thin and translucent skin, congenital contractures of thumbs and feet, joint instability, facial clefting, and coagulopathy, as well as heart, kidney, or intestinal defects. We elucidated the molecular basis of the disease by using a SNP array-based genome-wide linkage approach that identified distinct homozygous nonsense and missense mutations in CHST14 in each of four consanguineous families with this disease. The CHST14 gene encodes N-acetylgalactosamine 4-O-sulfotransferase 1 (D4ST1), which catalyzes 4-O sulfation of N-acetylgalactosamine in the repeating iduronic acid-α1,3-N-acetylgalactosamine disaccharide sequence to form dermatan sulfate. Mass spectrometry of glycosaminoglycans from a patient's fibroblasts revealed absence of dermatan sulfate and excess of chondroitin sulfate, showing that 4-O sulfation by CHST14 is essential for dermatan sulfate formation in vivo. Our results indicate that adducted thumb-clubfoot syndrome is a disorder resulting from a defect specific to dermatan sulfate biosynthesis and emphasize roles for dermatan sulfate in human development and extracellular-matrix maintenance. PMID:20004762

  15. Autoimmune Limbic Encephalitis and Syndrome of Inappropriate Antidiuretic Hormone Secretion Associated with Lamotrigine-induced Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) Syndrome.

    PubMed

    Ozisik, Lale; Tanriover, Mine Durusu; Saka, Esen

    2016-01-01

    Drug rash with eosinophilia and systemic symptoms (DRESS) is a severe drug hypersensitivity reaction characterized by rash, fever and multi-organ failure. Limbic encephalitis (LE) is a rare disorder characterized by cognitive dysfunction with memory disturbance, seizures and psychiatric symptoms. We herein present an unusual case of DRESS syndrome due to lamotrigine with reactivation of Epstein-Barr virus, which developed autoimmune LE and syndrome of inappropriate antidiuretic hormone secretion. Discontinuation of lamotrigine, administration of methylprednisolone and intravenous immunoglobulin led to improvement. The LE in this case might have been caused by an autoimmune inflammatory mechanism associated with DRESS syndrome.

  16. Frasier syndrome with childhood-onset renal failure.

    PubMed

    Buzi, F; Mella, P; Pilotta, A; Felappi, B; Camerino, G; Notarangelo, L D

    2001-01-01

    The Wilms' tumour 1 (WT1) gene encodes a protein which is believed to exert transcriptional and tumour-suppressor activities. Mutations of this gene have occasionally been associated with Wilms' tumour (<15% of cases) and, more consistently, with three syndromes characterized by urogenital abnormalities (WAGR, Denys-Drash and Frasier syndrome). SUBJECT/METHOD: A 25-year-old phenotypic female with a 46,XY karyotype presented with amenorrhoea. An ultrasound scan showed streak gonads and a rudimentary uterus. The patient had a history of post-streptococcal glomerulonephrosis, when aged 4 years, which had rapidly progressed to kidney failure, requiring transplantation at age 8. Frasier syndrome was suspected and confirmed by genetic analysis. In fact, direct sequencing of the PCR product of the intron 9 donor splice site revealed a substitution of guanine for adenine in position +5. Besides being one of the few Frasier syndrome cases to be genetically characterized, this case is interesting because of the unusually early-onset renal failure. Copyright 2001 S. Karger AG, Basel

  17. Seckel syndrome: a report of a case.

    PubMed

    Ramalingam, K; Kaliyamurthy, S D; Govindarajan, M; Swathi, S

    2012-01-01

    Seckel syndrome, first defined by Seckel in 1960, is a rare (incidence 1:10,000), genetically heterogeneous autosomal recessive disorder presenting at birth. This syndrome is characterized by a proportionate dwarfism of prenatal onset, a severe microcephaly with a "bird-headed" like appearance (beaked nose, receding forehead, prominent eyes, and micrognathia), and mental retardation. The significance of dental alterations in this syndrome resides in the defect, hypoplastic enamel, being limited to the primary dentition; in most instances the second primary molar tooth is not affected. A case of the Seckel syndrome is presented.

  18. Burning mouth syndrome.

    PubMed

    Thoppay, Jaisri R; De Rossi, Scott S; Ciarrocca, Katharine N

    2013-07-01

    Burning mouth syndrome (BMS) is a chronic condition that is characterized by burning symptoms of the oral mucosa without obvious clinical examination findings. This syndrome has complex characteristics, but its cause remains largely enigmatic, making treatment and management of patients with BMS difficult. Despite not being accompanied by evident organic changes, BMS can significantly reduce the quality of life for such patients. Therefore, it is incumbent on dental professionals to diagnose and manage patients with BMS as a part of comprehensive care. Copyright © 2013. Published by Elsevier Inc.

  19. Physical and chemical characterization of fly ashes from Swiss waste incineration plants and determination of the ash fraction in the nanometer range.

    PubMed

    Buha, Jelena; Mueller, Nicole; Nowack, Bernd; Ulrich, Andrea; Losert, Sabrina; Wang, Jing

    2014-05-06

    Waste incineration had been identified as an important source of ultrafine air pollutants resulting in elaborated treatment systems for exhaust air. Nowadays, these systems are able to remove almost all ultrafine particles. However, the fate of ultrafine particles caught in the filters has received little attention so far. Based on the use of engineered nano-objects (ENO) and their transfer into the waste stream, it can be expected that not only combustion generated nanoparticles are found in fly ashes but that many ENO finally end up in this matrix. A more detailed characterization of the nanoparticulate fraction of fly ashes is therefore needed. Physical and chemical characterizations were performed for fly ashes from five selected waste incineration plants (WIPs) with different input materials such as municipal waste, wood and sewage sludge. The intrinsic densities of the fly ashes were in the range of 2.7-3.2 g/cm(3). When the fly ash particle became airborne, the effective density depended on the particle size, increasing from 0.7-0.8 g/cm(3) for 100-150 nm to 2 g/cm(3) for 350-500 nm. The fly ash samples were fractionated at 2 μm, yielding fine fractions (<2 μm) and coarse fractions (>2 μm). The size distributions of the fine fractions in the airborne form were further characterized, which allowed calculation of the percentage of the fly ash particles below 100 nm. We found the highest mass-based percentage was about 0.07%; the number percentage in the fine fraction was in the range of 4.8% to 22%. Comparison with modeling results showed that ENO may constitute a considerable part of the fly ash particles below 100 nm. Chemical analyses showed that for the municipal waste samples Ca and Al were present in higher concentrations in the coarse fraction; for the mixed wood and sludge sample the P concentration was higher in the coarse fraction; for most other samples and elements they were enriched in the fine fraction. Electron microscopic images of fly ashes

  20. Sotos syndrome (cerebral gigantism): analysis of 8 cases.

    PubMed

    Melo, Débora Gusmão; Acosta, Angelina Xavier; Salles, Maria Aparecida de Almeida; Pina-Neto, João Monteiro de; Castro, José Daniel Vieira de; Santos, Antonio Carlos

    2002-06-01

    Sotos syndrome or cerebral gigantism is characterized by macrocephaly, overgrowth, mental retardation and central nervous system abnormalities. Congenital heart defects may be present. We report 8 patients with this syndrome and relate their clinical features, neuroimaging and echocardiographic findings.

  1. Gorlin-Goltz syndrome

    PubMed Central

    Joshi, Priya Shirish; Deshmukh, Vijay; Golgire, Someshwar

    2012-01-01

    Gorlin-Goltz syndrome is an uncommon autosomal dominant inherited disorder, which is characterized by multiple odontogenic Keratocysts and basal cell carcinomas, skeletal, dental, ophthalmic, and neurological abnormalities, intracranial ectopic calcifications of the falx cerebri, and facial dysmorphism. Pathogenesis of the syndrome is attributed to abnormalities in the long arm of chromosome 9 (q22.3-q31) and loss or mutations of human patched gene (PTCH1 gene). Diagnosis is based upon established major and minor clinical and radiological criteria and ideally confirmed by deoxyribo nucleic acid analysis. We report a case of a 9-year-old girl presenting with three major and one minor feature of Gorlin-Goltz syndrome. Radiologic findings of the syndrome are easily identifiable on Orthopantomogram, chest X-ray, and Computed tomography scans. These investigations prompt an early verification of the disease, which is very important to prevent recurrence and better survival rates from the coexistent diseases. PMID:22363371

  2. Exploding head syndrome.

    PubMed

    Sharpless, Brian A

    2014-12-01

    Exploding head syndrome is characterized by the perception of abrupt, loud noises when going to sleep or waking up. They are usually painless, but associated with fear and distress. In spite of the fact that its characteristic symptomatology was first described approximately 150 y ago, exploding head syndrome has received relatively little empirical and clinical attention. Therefore, a comprehensive review of the scientific literature using Medline, PsycINFO, Google Scholar, and PubMed was undertaken. After first discussing the history, prevalence, and associated features, the available polysomnography data and five main etiological theories for exploding head syndrome are summarized. None of these theories has yet reached dominance in the field. Next, the various methods used to assess and treat exploding head syndrome are discussed, as well as the limited outcome data. Finally, recommendations for future measure construction, treatment options, and differential diagnosis are provided. Copyright © 2014 Elsevier Ltd. All rights reserved.

  3. Characterization and evaluation of sorption potential of the iron mine waste after Samarco dam disaster in Doce River basin - Brazil.

    PubMed

    Almeida, Cristiane Aparecida; Oliveira, André Fernando de; Pacheco, Anderson Almeida; Lopes, Renata Pereira; Neves, Antônio Augusto; Lopes Ribeiro de Queiroz, Maria Eliana

    2018-06-14

    The Fundão dam collapsed releasing 60 million tons of mining waste into the environment. The mining wastes should be better studied, since some of them are deposited on the alluvial plains soil and at the bottom of the rivers, like sediments, of the region affected. Thus, this work aims to perform the chemical, physical and adsorptive characterization of the samples colleted in region de Paracatu de Baixo. The mining waste has uneven surface, with sizes ranging from 2 to 200 μm, pHPCZ in 6.0. Are composed predominantly by kaolinite, goethite, hematite, gibbsite and quartz. It has been classified as non-hazardous residues. The adsorption studies showed the mining waste have a low Cationic Exchange Capacity. The sorption process was occurs by ion exchange and the kinetics follows the pseudo second order model (R 2  > 0.78). The process is endothermic (ΔH in 29.33 kJ mol -1 ) and spontaneous (ΔG in -24.7 kJ mol -1 at 25 °C). The Langmuir model presented a better fit (R 2  > 0.995) to the experimental data. Therefore, the methylene blue can be used as a cation model to predict the behavior of cationic species on the mining waste, with maximum adsorption capacity of 4.42 mg g -1 at 25 °C. Copyright © 2018. Published by Elsevier Ltd.

  4. Cotard Syndrome with Catatonia: Unique Combination

    PubMed Central

    Basu, Aniruddha; Singh, Priti; Gupta, Rajiv; Soni, Sandeep

    2013-01-01

    Cotard syndrome is a rare psychiatric condition characterized by extreme nihilistic delusions. Catatonia though common, its combination with the Cotard syndrome is exceeding rare and more so the response with the pharmacotherapy as in our case. Since, both are found in organic conditions the importance of studying such a case is to understand the underlying neurobiologic determinants. PMID:24249939

  5. Cotard syndrome with catatonia: unique combination.

    PubMed

    Basu, Aniruddha; Singh, Priti; Gupta, Rajiv; Soni, Sandeep

    2013-07-01

    Cotard syndrome is a rare psychiatric condition characterized by extreme nihilistic delusions. Catatonia though common, its combination with the Cotard syndrome is exceeding rare and more so the response with the pharmacotherapy as in our case. Since, both are found in organic conditions the importance of studying such a case is to understand the underlying neurobiologic determinants.

  6. Munchausen Syndrome by Proxy: Evaluation and Treatment.

    ERIC Educational Resources Information Center

    Parnell, Teresa F.; Day, Deborah O.

    Munchausen Syndrome by Proxy (MSBP) is characterized by a significant caretaker, usually a mother, deliberately inducing and/or falsely reporting illness in a child. The potentially fatal outcome of undetected MSBP makes the understanding of this syndrome gravely important. Early detection and effective intervention can be accomplished through the…

  7. Prevalence of Burnout Syndrome in patients admitted with acute coronary syndrome.

    PubMed

    Prosdócimo, Ana Cláudia Giaxa; Lucina, Luciane Boreki; Marcia, Olandoski; Jobs, Priscila Megda João; Schio, Nicolle Amboni; Baldanzi, Fernanda Fachin; Costantini, Costantino Ortiz; Benevides-Pereira, Ana Maria Teresa; Guarita-Souza, Luiz Cesar; Faria-Neto, José Rocha

    2015-03-01

    Burnout Syndrome is the extreme emotional response to chronic occupational stress, manifesting as physical and mental exhaustion. Although associated with higher prevalence of cardiovascular risk factors, no study so far has evaluated whether the Burnout Syndrome could be a prevalent factor in non-elderly individuals active in the labor market, admitted for acute coronary syndrome (ACS). To evaluate the prevalence of the Burnout Syndrome in non-elderly, economically active patients, hospitalized with ACS. Cross-sectional study conducted in a tertiary and private cardiology center, with economically active patients aged <65 years, hospitalized with diagnosis of ACS. The Burnout Syndrome was evaluated with the Burnout Syndrome Inventory (BSI), which assesses workplace conditions and four dimensions that characterize the syndrome: emotional exhaustion (EE), emotional distancing (EmD), dehumanization (De) and professional fulfillment (PF). The Lipp's Stress Symptoms Inventory for Adults (LSSI) was applied to evaluate global stress. Of 830 patients evaluated with suspected ACS, 170 met the study criteria, 90% of which were men, overall average age was 52 years, and 40.5% had an average income above 11 minimum wages. The prevalence of the Burnout Syndrome was 4.1%. When we evaluated each dimension individually, we found high EE in 34.7%, high De in 52.4%, high EDi in 30.6%, and low PF in 5.9%. The overall prevalence of stress was 87.5%. We found a low prevalence of Burnout Syndrome in an economically active, non-elderly population among patients admitted for ACS in a tertiary and private hospital.

  8. Prevalence of Burnout Syndrome in Patients Admitted with Acute Coronary Syndrome

    PubMed Central

    Prosdócimo, Ana Cláudia Giaxa; Lucina, Luciane Boreki; Marcia, Olandoski; Jobs, Priscila Megda João; Schio, Nicolle Amboni; Baldanzi, Fernanda Fachin; Costantini, Costantino Ortiz; Benevides-Pereira, Ana Maria Teresa; Guarita-Souza, Luiz Cesar; Faria-Neto, José Rocha

    2015-01-01

    Background Burnout Syndrome is the extreme emotional response to chronic occupational stress, manifesting as physical and mental exhaustion. Although associated with higher prevalence of cardiovascular risk factors, no study so far has evaluated whether the Burnout Syndrome could be a prevalent factor in non-elderly individuals active in the labor market, admitted for acute coronary syndrome (ACS). Objective To evaluate the prevalence of the Burnout Syndrome in non-elderly, economically active patients, hospitalized with ACS. Methods Cross-sectional study conducted in a tertiary and private cardiology center, with economically active patients aged <65 years, hospitalized with diagnosis of ACS. The Burnout Syndrome was evaluated with the Burnout Syndrome Inventory (BSI), which assesses workplace conditions and four dimensions that characterize the syndrome: emotional exhaustion (EE), emotional distancing (EmD), dehumanization (De) and professional fulfillment (PF). The Lipp’s Stress Symptoms Inventory for Adults (LSSI) was applied to evaluate global stress. Results Of 830 patients evaluated with suspected ACS, 170 met the study criteria, 90% of which were men, overall average age was 52 years, and 40.5% had an average income above 11 minimum wages. The prevalence of the Burnout Syndrome was 4.1%. When we evaluated each dimension individually, we found high EE in 34.7%, high De in 52.4%, high EDi in 30.6%, and low PF in 5.9%. The overall prevalence of stress was 87.5%. Conclusion We found a low prevalence of Burnout Syndrome in an economically active, non-elderly population among patients admitted for ACS in a tertiary and private hospital. PMID:25517388

  9. Dover AFB Characterization/Hazardous Waste Management Survey, Dover AFB, Delaware.

    DTIC Science & Technology

    1986-07-01

    chromium ion (chromate, chromic acid) needs to be reduced to the insoluble trivalent ion ( chromium oxide, chromic hydroxide) to facilitate effective...precipitation. The good removal efficiency seen in the Jar tests indicates the chromium may already be in the trivalent oxidation state, possibly reduced...fails the EP toxicity test for chromium alone, the waste may be excluded from being a hazardous waste, if the chromium is primarily in the trivalent

  10. 10 CFR 60.18 - Review of site characterization activities. 2

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... developed, and on the progress of waste form and waste package research and development. The semiannual... of site characterization will be established. Other topics related to site characterization shall...

  11. 10 CFR 60.18 - Review of site characterization activities. 2

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... developed, and on the progress of waste form and waste package research and development. The semiannual... of site characterization will be established. Other topics related to site characterization shall...

  12. 10 CFR 60.18 - Review of site characterization activities. 2

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... developed, and on the progress of waste form and waste package research and development. The semiannual... of site characterization will be established. Other topics related to site characterization shall...

  13. 10 CFR 60.18 - Review of site characterization activities. 2

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... developed, and on the progress of waste form and waste package research and development. The semiannual... of site characterization will be established. Other topics related to site characterization shall...

  14. Microstructural and mineralogical characterization of selected shales in support of nuclear waste repository studies

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Lee, S.Y.; Hyder, L.K.; Alley, P.D.

    1988-01-01

    Five shales were examined as part of the Sedimentary Rock Program evaluation of this medium as a potential host for a US civilian nuclear waste repository. The units selected for characterization were the Chattanooga Shale from Fentress County, Tennessee; the Pierre Shale from Gregory County, South Dakota; the Green River Formation from Garfield County, Colorado; and the Nolichucky Shale and Pumpkin Valley Shale from Roane County, Tennessee. The micromorphology and structure of the shales were examined by petrographic, scanning electron, and high-resolution transmission electron microscopy. Chemical and mineralogical compositions were studied through the use of energy-dispersive x-ray, neutron activation, atomicmore » absorption, thermal, and x-ray diffraction analysis techniques. 18 refs., 12 figs., 2 tabs.« less

  15. The group of epidermal nevus syndromes Part I. Well defined phenotypes.

    PubMed

    Happle, Rudolf

    2010-07-01

    The epidermal nevus syndromes represent a group of distinct disorders that can be distinguished by the type of associated epidermal nevus and by the criterion of presence or absence of heritability. Well defined syndromes characterized by organoid epidermal nevi include Schimmelpenning syndrome, phacomatosis pigmentokeratotica, nevus comedonicus syndrome, angora hair nevus syndrome, and Becker nevus syndrome. The molecular basis of these disorders has so far not been identified. By contrast, the group of syndromes characterized by keratinocytic nevi comprises three phenotypes with a known molecular etiology in the form of CHILD (congenital hemidysplasia with ichthyosiform nevus and limb defects) syndrome, type 2 segmental Cowden disease, and fibroblast growth factor receptor 3 epidermal nevus syndrome (García-Hafner-Happle syndrome), whereas Proteus syndrome is still of unknown origin. From this overview, it is clear that a specific type of these disorders cannot be classified by the name "epidermal nevus syndrome" nor by the terms "organoid nevus syndrome" or "keratinocytic nevus syndrome." After completing this learning activity, participants should be able to distinguish nine different epidermal nevus syndromes by their characteristic features, understand the practical significance of avoiding terms like "epidermal nevus syndrome" or "keratinocytic nevus syndrome" to define any specific entity within this group of disorders, and differentiate between nonhereditary traits and those bearing a genetic risk because of either Mendelian or non-Mendelian inheritance. Copyright (c) 2010 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

  16. Combined Pulmonary Fibrosis and Emphysema Syndrome

    PubMed Central

    Rounds, Sharon I. S.

    2012-01-01

    There is increasing clinical, radiologic, and pathologic recognition of the coexistence of emphysema and pulmonary fibrosis in the same patient, resulting in a clinical syndrome known as combined pulmonary fibrosis and emphysema (CPFE) that is characterized by dyspnea, upper-lobe emphysema, lower-lobe fibrosis, and abnormalities of gas exchange. This syndrome frequently is complicated by pulmonary hypertension, acute lung injury, and lung cancer. The CPFE syndrome typically occurs in male smokers, and the mortality associated with this condition, especially if pulmonary hypertension is present, is significant. In this review, we explore the current state of the literature and discuss etiologic factors and clinical characteristics of the CPFE syndrome. PMID:22215830

  17. Antimony smelting process generating solid wastes and dust: characterization and leaching behaviors.

    PubMed

    Guo, Xuejun; Wang, Kunpeng; He, Mengchang; Liu, Ziwei; Yang, Hailin; Li, Sisi

    2014-07-01

    A large amount of solid waste has been produced by the antimony smelting process in the "World Capital of Antimony", Xikuangshan area in China. This study comprehensively investigated the physical and chemical characteristics of the various solid wastes, as well as the leaching behavior of the solid wastes, which included water-quenched slag, arsenic-alkali residue, desulfurized slag and blast furnace dust. These four types of waste were enriched in a variety of heavy metals and metalloids and more specifically with As and Sb levels up to 8.6 × 10⁴ and 3.16×10⁵ mg/kg, respectively, in arsenic-alkali residue. For desulfurized slag and water-quenched slag, the leaching concentration of Sb significantly exceeded the acceptable limits during the leaching tests using the toxicity characteristic leaching procedure and the synthetic precipitation leaching procedure. In addition, As leaching in arsenic-alkali residue was extraordinarily hazardous, being three orders of magnitude higher than the regulatory level of As. According to the results of the extraction tests, all the tested wastes were classified as hazardous waste. Copyright © 2014. Published by Elsevier B.V.

  18. MUNICIPAL WASTE COMBUSTION ASSESSMENT ...

    EPA Pesticide Factsheets

    The report defines and characterizes types of medical waste, discusses the impacts of burning medical waste on combustor emissions, and outlines important handling and operating considerations. Facility-specific design, handling, and operating practiced are also discussed for municipal waste combustors (MWCs) that reportedly accept medical waste in the U.S., Europe, and Canada. nly very limited data are available on the emission impacts associated with the combustion of medical waste in MWGs. Especially lacking is information needed to fully evaluate the impacts on acid gas, dioxin, and metals emissions, as well as the design and operating requirements for complete destruction of solvents, cytotoxic chemicals, and pathogens. The EPA's Office of Air Quatity Planning and Standards is developing emission standards and guidelines for new and existing MWCs under Sections 111(b) and 111(d) of the Clean Air Act. In support of these regulatory development efforts, the Air and Energy Engineering Research Laboratory in EPA's Office of Research and Development has conducted an assessment to examine the incineration of medical waste in MWGs from an emission standpoint. Potential worker safety and health problems associated with handling of medical wastes and residues were also identified. information

  19. Gorlin-Goltz syndrome: A series of three cases.

    PubMed

    Patankar, Amod P; Kshirsagar, Rajesh A; Dugal, Arun; Mishra, Akshay; Ram, Hari

    2014-01-01

    The Gorlin-Goltz syndrome (GGS) is also known as nevoid basal cell carcinoma syndrome. It is characterized by multiple keratocystic odontogenic tumors (KCOTs) in the jaw, multiple basal cell nevi carcinomas and skeletal abnormities. The syndrome may be diagnosed early by a dentist during the routine radiographic exams in the first decade of life, since the KCOTs are usually one of the first manifestations of the syndrome. This article reports the series of 3 cases, emphasizing its clinical and radiographic manifestations of GGS.

  20. Characterization of Microsatellites in Pseudogymnoascus destructans for White-nose Syndrome Genetic Analysis.

    PubMed

    Drees, Kevin P; Parise, Katy L; Rivas, Stephanie M; Felton, Lindsey L; Puechmaille, Sébastien J; Keim, Paul; Foster, Jeffrey T

    2017-10-01

    Despite only emerging in the past decade, white-nose syndrome has become among the most devastating wildlife diseases known. The pathogenic fungus Pseudogymnoascus destructans infects hibernating bats and typically leads to high rates of mortality at hibernacula during winter in North America. We developed a set of genetic markers to better differentiate P. destructans isolates. We designed and successfully characterized these 23 microsatellite markers of P. destructans for use in disease ecology and epidemiology research. We validated these loci with DNA extracted from a collection of P. destructans isolates from the US and Canada, as well as from Europe (the likely introduction source based on currently available data). Genetic diversity calculated for each locus and for the multilocus panel as a whole indicates sufficient allelic diversity to differentiate among and between samples from both Europe and North America. Indices of genetic diversity indicate a loss of allelic diversity that is consistent with the recent introduction and rapid spread of an emerging pathogen.

  1. Characterization of chitin and chitosan synthesized from red snapper (Lutjanus sp.) scale's waste

    NASA Astrophysics Data System (ADS)

    Takarina, N. D.; Fanani, A. A.

    2017-07-01

    Chitin and chitosan are natural biopolymer which are useful for industrial, medical and environmental field. Study about using fish scale sources especially saltwater fish is still limited. Red snapper (Lutjanus sp) is common tropical saltwater fish that known as important source of marine products, particularly in Indonesia. Correspondingly, the consumption of this species has generated significant amount of discarded scale wastes recently and hence can cause adverse impact on the environment. Utilizing the fish scale as alternative sources of chitin and chitosan can be one solution dealing with environmental problem. Therefore, this research aimed to characterize the chitin and chitosan derived from the red snapper scale wastes. Chitin were extracted by deproteination and demineralization while chitosan using deacetylation. Morphology of the chitin and chitosan were analyzed using electron dispersal spectroscopy (EDS) and scanning electron microscope (SEM), while degree of deacetylation using fourier transform infrared spectroscopy (FTIR). Proximate analysis showed that content of moisture, ash, and nitrogen in chitin were 3.20 %, 2.40 %, 0.04 %, respectively while in chitosan were 6.14 %, 1.18 %, 0.03 % respectively. Furthermore, amount of C, O, Na, Al, P and Ca elements were obtained from chitin and chitosan samples by energy dispersed spectroscopy respectively. The degree of deacetylation for both chitin and chitosan showed high value more than 75 %. Hence, by considering the chemical properties of red snapper scales, it confirms that this species is a promising alternative source for both chitin and chitosan.

  2. Quantitative and nutritional characterization of fruit and vegetable waste from marketplace: a potential use as bovine feedstuff?

    PubMed

    Angulo, Joaquin; Mahecha, Liliana; Yepes, Sergio A; Yepes, Angela M; Bustamante, Gilberto; Jaramillo, Harold; Valencia, Edward; Villamil, Tomás; Gallo, Jorge

    2012-03-01

    There are different sources for the generation of solid waste, and marketplaces are considered one of them. Fruit and vegetable waste (FV) from a marketplace in Colombia was quantitatively and nutritionally characterized to contribute to its use in bovine feeding and to contribute minimizing its environmental impact. The evaluation was carried out 7 days per week during 4 periods of the year. FV was grouped by cluster analysis using SAS(®) 2006. FV was composed of 43% fruit, 30% vegetables and 27% stems, leaves, leaf wrappers, corncobs, roots, refuse and others. FV was defined in four main groups. On average, FV contained 10% crude protein (CP), 36.6% neutral detergent fiber (NDF), 29.6% acid detergent fiber (ADF), 87.8% ruminal degradability at 24 h, 3657 kcal/kg, 0.59% calcium (Ca(+2)), and 0.21% phosphorous (P). There were no statistical differences between days or between periods of evaluation (p > 0.05) for CP or for Ca(+2). As for NDF and ADF, there were statistically significant differences between periods but not between days. The microbiological parameters only increased when the humidity was up to 12%. FV represents a potential feedstuff for bovine feeding, and its recycling could avoid the discharge of a large amount of waste to landfills, which would minimize its environmental impact. Copyright © 2010. Published by Elsevier Ltd.

  3. Hazardous-waste-characterization survey of unknown drums at the 21st Tactical Fighter Wing, Elmendorf and Shemya Air Force Bases, and Galena and King Salmon Airports, Alaska. Final report 2-13 Aug 91

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Bishop, M.S.

    1991-12-01

    At the request of the USAF Regional Hospital Elmendorf/SGPB (PACAF), the Armstrong Laboratory, Occupational and Environmental Health Directorate, conducted a hazardous waste characterization survey of unknown drums at Elmendorf AFB from 2 Aug - 13 Aug 91. The scope of the survey was to sample and characterize drums of unknown material stored at Elmendorf AFB, Shemya AFB, and Galena and King Salmon Airports. Several waste streams were sampled at Elmendorf AFB to revalidate sample results from a previous survey.

  4. Dimensional and chemical characterization of particles at a downwind receptor site of a waste-to-energy plant

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Buonanno, G., E-mail: buonanno@unicas.i; Stabile, L.; Avino, P.

    2010-07-15

    In the last years numerous epidemiological studies were carried out to evaluate the effects of particulate matter on human health. In industrialized areas, anthropogenic activities highly contribute to the fine and ultrafine particle concentrations. Then, it is important to characterize the evolution of particle size distribution and chemical composition near these emission points. Waste incineration represents a favorable technique for reducing the waste volume. However, in the past, municipal waste incinerators (MWIs) had a bad reputation due to the emission of toxic combustion byproducts. Consequently, the risk perception of the people living near MWIs is very high even if inmore » Western countries waste incineration has nowadays to be considered a relatively clean process from a technical point of view. The study here presented has an exemplary meaning for developing appropriate management and control strategies for air quality in the surrounding of MWIs and to perform exposure assessment for populations involved. Environment particles were continuously measured through a SMPS/APS system over 12 months. The monitoring site represents a downwind receptor of a typical MWI. Furthermore, elements and organic fractions were measured by means of the Instrumental Neutron Activation Analysis and using dichotomous and high volume samplers. Annual mean values of 8.6 x 10{sup 3} +- 3.7 x 10{sup 2} part. cm{sup -3} and 31.1 +- 9.0 mug m{sup -3} were found for number and mass concentration, typical of a rural site. Most of the elements can be attributed to long-range transport from other natural and/or anthropogenic sources. Finally, the Polycyclic Aromatic Hydrocarbons present low concentrations with a mean value of 24.6 ng m{sup -3}.« less

  5. Adaption of the Magnetometer Towed Array geophysical system to meet Department of Energy needs for hazardous waste site characterization

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Cochran, J.R.; McDonald, J.R.; Russell, R.J.

    1995-10-01

    This report documents US Department of Energy (DOE)-funded activities that have adapted the US Navy`s Surface Towed Ordnance Locator System (STOLS) to meet DOE needs for a ``... better, faster, safer and cheaper ...`` system for characterizing inactive hazardous waste sites. These activities were undertaken by Sandia National Laboratories (Sandia), the Naval Research Laboratory, Geo-Centers Inc., New Mexico State University and others under the title of the Magnetometer Towed Array (MTA).

  6. Site and soil characterization of hazardous waste sites using an expert system guide

    NASA Astrophysics Data System (ADS)

    Cameron, Roy E.

    1993-03-01

    An expert system guide (knowledge book) has been devised to assist field personnel who must identify, describe, sample, and interpret size and soil characteristics of hazardous waste sites. The guide takes an approach that will be unfamiliar to most soil and environmental scientists and is directed to on-scene coordinators and project managers and others who may have little soil science training. It meets the need of the U.S. Environmental Protection Agency for standard procedures, guidelines, or protocols that address soil and site contamination, particularly heavy metals. The guide is organized to include: (1) general considerations and processes for collecting and using site and soils data, (2) detailed knowledge frames (descriptive profiles) of likely site and soil conditions, (3) a citation of references, (4) an appendix listing common sources of characterization data, and (5) a glossary of more than 900 general definitions.

  7. The characterization and risk assessment of the `Red Forest` radioactive waste burial site at Chernobyl Nuclear Power Plant

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Bungai, D.A.; Skalskij, A.S.; Dzhepo, S.P.

    The `Red Forest` radioactive waste burials created during emergency clean-up activities at Chernobyl Nuclear Power Plant represent a serious source of radioactive contamination of the local ground water system with 9OSr concentration in ground water exceeding the drinking water standard by 3-4 orders of magnitude. In this paper we present results of our hydrogeological and radiological `Red Forest` site characterization studies, which allow us to estimate 9OSr subsurface migration parameters. We use then these parameters to assess long terrain radionuclide transport to groundwater and surface water, and to analyze associated health risks. Our analyses indicate that 9OSr transport via groundmore » water pathway from `Red Forest` burials to the adjacent Pripyat River is relatively insignificant due to slow release of 9OSr from the waste burials (less than 1% of inventory per year) and due to long enough ground water residence time in the subsurface, which allows substantial decay of the radioactive contaminant. Tins result and our previous analyses indicate, that though conditions of radioactive waste storage in burials do not satisfy Ukrainian regulation on radiation protection, health risks caused by radionuclide migration to ground water from `Red Forest` burials do not justify application of expensive countermeasures.« less

  8. Recovery and characterization of proteins from pangas (Pangasius pangasius) processing waste obtained through pH shift processing.

    PubMed

    Surasani, Vijay Kumar Reddy; Kudre, Tanaji; Ballari, Rajashekhar V

    2018-04-01

    Study was conducted to recover proteins from pangas (Pangasius pangasius) processing waste (fillet frames) using pH shift method and to characterize the recovered isolates. pH 2.0 from acidic range and pH 13.0 from alkaline range were found to have maximum protein recovery (p < 0.05). During the recovery process, acidic pH (pH 2.0) was found to have minimal effect on proteins resulting in more stable isolates and strong protein gels. Alkaline pH (pH 13.0) caused protein denaturation resulting in less stable proteins and poor gel network. Both acidic and alkaline-aided processing caused significant (p < 0.05) reductions in total lipid, myoglobin, and pigment content thus by resulting in whiter protein isolates and gels. The content of total essential amino acids increased during pH shift processing, indicating the enrichment of essential amino acids. No microbial counts were detected in any of the isolates prepared using acid and alkaline extraction methods. pH shift processing was found to be promising in the utilization of fish processing waste for the recovery of functional proteins from pangas processing waste thus by reducing the supply demand gap as well pollution problems.

  9. Development of iron phosphate ceramic waste form to immobilize radioactive waste solution

    NASA Astrophysics Data System (ADS)

    Choi, Jongkwon; Um, Wooyong; Choung, Sungwook

    2014-09-01

    The objective of this research was to develop an iron phosphate ceramic (IPC) waste form using converter slag obtained as a by-product of the steel industry as a source of iron instead of conventional iron oxide. Both synthetic off-gas scrubber solution containing technetium-99 (or Re as a surrogate) and LiCl-KCl eutectic salt, a final waste solution from pyrochemical processing of spent nuclear fuel, were used as radioactive waste streams. The IPC waste form was characterized for compressive strength, reduction capacity, chemical durability, and contaminant leachability. Compressive strengths of the IPC waste form prepared with different types of waste solutions were 16 MPa and 19 MPa for LiCl-KCl eutectic salt and the off-gas scrubber simulant, respectively, which meet the minimum compressive strength of 3.45 MPa (500 psi) for waste forms to be accepted into the radioactive waste repository. The reduction capacity of converter slag, a main dry ingredient used to prepare the IPC waste form, was 4136 meq/kg by the Ce(IV) method, which is much higher than those of the conventional Fe oxides used for the IPC waste form and the blast furnace slag materials. Average leachability indexes of Tc, Li, and K for the IPC waste form were higher than 6.0, and the IPC waste form demonstrated stable durability even after 63-day leaching. In addition, the Toxicity Characteristic Leach Procedure measurements of converter slag and the IPC waste form with LiCl-KCl eutectic salt met the universal treatment standard of the leachability limit for metals regulated by the Resource Conservation and Recovery Act. This study confirms the possibility of development of the IPC waste form using converter slag, showing its immobilization capability for radionuclides in both LiCl-KCl eutectic salt and off-gas scrubber solutions with significant cost savings.

  10. Development of iron phosphate ceramic waste form to immobilize radioactive waste solution

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Choi, Jongkwon; Um, Wooyong; Choung, Sungwook

    The objective of this research was to develop an iron phosphate ceramic (IPC) waste form using converter slag obtained as a by-product of the steel industry as a source of iron instead of conventional iron oxide. Both synthetic off-gas scrubber solution containing technetium-99 (or Re as a surrogate) and LiCl-KCl eutectic salt, a final waste solution from pyrochemical processing of spent nuclear fuel, were used as radioactive waste streams. The IPC waste form was characterized for compressive strength, reduction capacity, chemical durability, and contaminant leachability. Compressive strengths of the IPC waste form prepared with different types of waste solutions weremore » 16 MPa and 19 MPa for LiCl-KCl eutectic salt and the off-gas scrubber simulant, respectively, which meet the minimum compressive strength of 3.45 MPa (500 psi) for waste forms to be accepted into the radioactive waste repository. The reduction capacity of converter slag, a main dry ingredient used to prepare the IPC waste form, was 4,136 meq/kg by the Ce(IV) method, which is much higher than those of the conventional Fe oxides used for the IPC waste form and the blast furnace slag materials. Average leachability indexes of Tc, Li, and K for the IPC waste form were higher than 6.0, and the IPC waste form demonstrated stable durability even after 63-day leaching. In addition, the Toxicity Characteristic Leach Procedure measurements of converter slag and the IPC waste form with LiCl-KCl eutectic salt met the universal treatment standard of the leachability limit for metals regulated by the Resource Conservation and Recovery Act. This study confirms the possibility of development of the IPC waste form using converter slag, showing its immobilization capability for radionuclides in both LiCl-KCl eutectic salt and off-gas scrubber solutions with significant cost savings.« less

  11. Munchausen syndrome and Munchausen syndrome by proxy: a narrative review.

    PubMed

    Sousa Filho, Daniel de; Kanomata, Elton Yoji; Feldman, Ricardo Jonathan; Maluf Neto, Alfredo

    2017-01-01

    The Munchausen syndrome and Munchausen syndrome by proxy are factitious disorders characterized by fabrication or induction of signs or symptoms of a disease, as well as alteration of laboratory tests. People with this syndrome pretend that they are sick and tend to seek treatment, without secondary gains, at different care facilities. Both syndromes are well-recognized conditions described in the literature since 1951. They are frequently observed by health teams in clinics, hospital wards and emergency rooms. We performed a narrative, nonsystematic review of the literature, including case reports, case series, and review articles indexed in MEDLINE/PubMed from 1951 to 2015. Each study was reviewed by two psychiatry specialists, who selected, by consensus, the studies to be included in the review. Although Munchausen syndrome was first described more than 60 years ago, most of studies in the literature about it are case reports and literature reviews. Literature lacks more consistent studies about this syndrome epidemiology, therapeutic management and prognosis. Undoubtedly, these conditions generate high costs and unnecessary procedures in health care facilities, and their underdiagnose might be for lack of health professional's knowledge about them, and to the high incidence of countertransference to these patients and to others, who are exposed to high morbidity and mortality, is due to symptoms imposed on self or on others.

  12. Munchausen syndrome and Munchausen syndrome by proxy: a narrative review

    PubMed Central

    de Sousa, Daniel; Kanomata, Elton Yoji; Feldman, Ricardo Jonathan; Maluf, Alfredo

    2017-01-01

    ABSTRACT The Munchausen syndrome and Munchausen syndrome by proxy are factitious disorders characterized by fabrication or induction of signs or symptoms of a disease, as well as alteration of laboratory tests. People with this syndrome pretend that they are sick and tend to seek treatment, without secondary gains, at different care facilities. Both syndromes are well-recognized conditions described in the literature since 1951. They are frequently observed by health teams in clinics, hospital wards and emergency rooms. We performed a narrative, nonsystematic review of the literature, including case reports, case series, and review articles indexed in MEDLINE/PubMed from 1951 to 2015. Each study was reviewed by two psychiatry specialists, who selected, by consensus, the studies to be included in the review. Although Munchausen syndrome was first described more than 60 years ago, most of studies in the literature about it are case reports and literature reviews. Literature lacks more consistent studies about this syndrome epidemiology, therapeutic management and prognosis. Undoubtedly, these conditions generate high costs and unnecessary procedures in health care facilities, and their underdiagnose might be for lack of health professional's knowledge about them, and to the high incidence of countertransference to these patients and to others, who are exposed to high morbidity and mortality, is due to symptoms imposed on self or on others. PMID:29364370

  13. Characterization of brominated flame retardants from e-waste components in China.

    PubMed

    Yu, Danfeng; Duan, Huabo; Song, Qingbin; Liu, Yicheng; Li, Ying; Li, Jinhui; Shen, Weijun; Luo, Jiahui; Wang, Jinben

    2017-10-01

    Many studies show that high levels of many toxic metals and persistent and bio-accumulative chemicals have been found in electronic waste (e-waste) dismantling sites and their surrounding environmental media. Both flame-retardant plastic housing materials and printed circuit boards (PCBs) could be the major contributors. However, relatively little work has focused on the use or content of toxic substances and their changing in scrap housing materials and PCBs from home appliances. This study evaluated the existence of brominated flame retardants (BFRs, including polybrominated diphenyl ethers (PBDEs) and Tetrabromobisphenol-A (TBBPA)) in housing plastics and PCBs from home appliances collected from various e-waste recyclers in China. These were then analyzed for the potential migration of BFRs from the e-waste components into their recycled products. The results show that both PBDEs and TBBPA were found with high level in most of e-waste samples, indicating that the widespread use of BFRs in home appliances are entering into the end-of-life stage. For the plastics samples, CRT TVs and LCD monitors should be given priority for the control of BFRs. Regarding PBDEs, the dominant congeners of BDE-209 in the plastics samples contributed 90.72-93.54% to the total concentrations of PBDEs, yet there are large variations for PCBs samples: BDE-28, -47, -99, and -153 were also important congeners compositions, except for BDE-209. Compared with previous studies, the BFRs concentrations in current Chinese e-waste are trending to decline. This study also found that BFRs in housing plastics and PCBs will be transferred into the recycled products with other purpose use, and the new products could have highly enriched capacities for BFRs. The obtained results could be helpful to manage e-waste and their components properly in order to minimize associated environmental and health risks of BFRs, particularly for their further reuse. Copyright © 2017 Elsevier Ltd. All rights reserved.

  14. Fluidized bed steam reformed mineral waste form performance testing to support Hanford Supplemental Low Activity Waste Immobilization Technology Selection

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Jantzen, C. M.; Pierce, E. M.; Bannochie, C. J.

    This report describes the benchscale testing with simulant and radioactive Hanford Tank Blends, mineral product characterization and testing, and monolith testing and characterization. These projects were funded by DOE EM-31 Technology Development & Deployment (TDD) Program Technical Task Plan WP-5.2.1-2010-001 and are entitled “Fluidized Bed Steam Reformer Low-Level Waste Form Qualification”, Inter-Entity Work Order (IEWO) M0SRV00054 with Washington River Protection Solutions (WRPS) entitled “Fluidized Bed Steam Reforming Treatability Studies Using Savannah River Site (SRS) Low Activity Waste and Hanford Low Activity Waste Tank Samples”, and IEWO M0SRV00080, “Fluidized Bed Steam Reforming Waste Form Qualification Testing Using SRS Low Activity Wastemore » and Hanford Low Activity Waste Tank Samples”. This was a multi-organizational program that included Savannah River National Laboratory (SRNL), THOR® Treatment Technologies (TTT), Pacific Northwest National Laboratory (PNNL), Oak Ridge National Laboratory (ORNL), Office of River Protection (ORP), and Washington River Protection Solutions (WRPS). The SRNL testing of the non-radioactive pilot-scale Fluidized Bed Steam Reformer (FBSR) products made by TTT, subsequent SRNL monolith formulation and testing and studies of these products, and SRNL Waste Treatment Plant Secondary Waste (WTP-SW) radioactive campaign were funded by DOE Advanced Remediation Technologies (ART) Phase 2 Project in connection with a Work-For-Others (WFO) between SRNL and TTT.« less

  15. 40 CFR 761.269 - Sampling liquid PCB remediation waste.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 40 Protection of Environment 30 2010-07-01 2010-07-01 false Sampling liquid PCB remediation waste..., AND USE PROHIBITIONS Cleanup Site Characterization Sampling for PCB Remediation Waste in Accordance with § 761.61(a)(2) § 761.269 Sampling liquid PCB remediation waste. (a) If the liquid is single phase...

  16. Production and characterization of rhamnolipid biosurfactant from waste frying coconut oil using a novel Pseudomonas aeruginosa D.

    PubMed

    George, S; Jayachandran, K

    2013-02-01

    To improve biosurfactant production economics by the utilization of potential low-cost materials. In an attempt to utilize cost-effective carbon sources in the fermentative production of biosurfactants, various pure and waste frying oils were screened by a standard biosurfactant producing strain. Considering the regional significance, easy availability and the economical advantages, waste frying coconut oil was selected as the substrate for further studies. On isolation of more competent strains that could use waste frying coconut oil efficiently as a carbon source, six bacterial strains were isolated on cetyltrimethyl ammonium bromide-methylene blue agar plate, from a soil sample collected from the premises of a coconut oil mill. Among these, Pseudomonas aeruginosa D was selected as the potential producer of rhamnolipid. Spectrophotometric method, TLC, methylene blue active substance assay, drop collapse technique, surface tension measurement by Du Nouy ring method and emulsifying test confirmed the rhamnolipid producing ability of the selected strain and various process parameters were optimized for the production of maximum amount of biosurfactant. Rhamnolipid components purified and separated by ethyl acetate extraction, preparative silica gel column chromatography, HPLC and TLC were characterized by fast atom bombardment mass spectrometry as a mixture of dirhamnolipids and monorhamnolipids. The rhamnolipid homologues detected were Rha-Rha-C(10) -C(10) , Rha-C(12) -C(10) and Rha-C(10) -C(8) /Rha-C(8) -C(10) . These results indicated the possibility of waste frying coconut oil to be used as a very effective alternate substrate for the economic production of rhamnolipid by a newly isolated Ps. aeruginosa D. Results of this study throws light on the alternate use of already used cooking oil as high-energy source for producing a high value product like rhamnolipid. This would provide options for the food industry other than the recycling and reuse of waste frying

  17. 40 CFR 761.345 - Form of the waste to be sampled.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... Characterization for PCB Disposal in Accordance With § 761.62, and Sampling PCB Remediation Waste Destined for Off... waste and PCB remediation waste destined for off-site disposal must be in the form of either flattened...

  18. Robust telerobotics - an integrated system for waste handling, characterization and sorting

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Couture, S.A.; Hurd, R.L.; Wilhelmsen, K.C.

    The Mixed Waste Management Facility (MWMF) at the Lawrence Livermore National Laboratory was designed to serve as a national testbed to demonstrate integrated technologies for the treatment of low-level organic mixed waste at a pilot-plant scale. Pilot-scale demonstration serves to bridge the gap between mature, bench-scale proven technologies and full-scale treatment facilities by providing the infrastructure needed to evaluate technologies in an integrated, front-end to back-end facility. Consistent with the intent to focus on technologies that are ready for pilot scale deployment, the front-end handling and feed preparation of incoming waste material has been designed to demonstrate the application ofmore » emerging robotic and remotely operated handling systems. The selection of telerobotics for remote handling in MWMF was made based on a number of factors - personnel protection, waste generation, maturity, cost, flexibility and extendibility. Telerobotics, or shared control of a manipulator by an operator and a computer, provides the flexibility needed to vary the amount of automation or operator intervention according to task complexity. As part of the telerobotics design effort, the technical risk of deploying the technology was reduced through focused developments and demonstrations. The work involved integrating key tools (1) to make a robust telerobotic system that operates at speeds and reliability levels acceptable to waste handling operators and, (2) to demonstrate an efficient operator interface that minimizes the amount of special training and skills needed by the operator. This paper describes the design and operation of the prototype telerobotic waste handling and sorting system that was developed for MWMF.« less

  19. Cotard and Capgras syndrome after ischemic stroke.

    PubMed

    Sottile, Fabrizio; Bonanno, Lilla; Finzi, Giuseppina; Ascenti, Giorgio; Marino, Silvia; Bramanti, Placido; Corallo, Francesco

    2015-04-01

    Capgras and Cotard are delusional misidentification syndromes characterized by delusions about oneself, others, places, and objects. To date, there are few cases of comorbidity of both syndromes. We describe a case of aphasic stroke patient affected by cerebral ischemia localized in right temporoparietal region. The patient showed a typical clinical picture of delusional disorder attributable, through psychological assessment, to comorbidity of both Capgras and Cotard syndromes. Copyright © 2015 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  20. Kindler syndrome: report of two cases.

    PubMed

    Mendes, Luciana; Nogueira, Lisiane; Vilasboas, Virginia; Talhari, Carolina; Talhari, Sinésio; Santos, Mônica

    2012-01-01

    Kindler syndrome is a rare autosomal recessive genodermatosis characterized by trauma-induced blisters, progressive poikiloderma and varying degrees of photosensitivity. In 2003, loss-of-function mutations were identified in the gene KIND1 mapped to chromosome 20p12.3. In this paper, we report Kindler syndrome in two children born to consanguineous parents presenting acral blistering, photosensitivity, poikiloderma, cutaneous atrophy and periodontitis.

  1. Characterization of pyrolysis products derived from three biological wastes and their effect on plant growth and soil water retention

    NASA Astrophysics Data System (ADS)

    Bouqbis, Laila; Werner Koyro, Hans; Kammann, Claudia; Zohra Ainlhout, Lalla Fatima; Boukhalef, Laila; Cherif Harrouni, Moulay

    2018-05-01

    Over two-thirds of Morocco can be classified as semiarid, arid and desert with low and variable rainfalls. While the country is subject to frequent drought, groundwater resources are predominantly consume by irrigated agriculture leading to the depletion of water resources and degradation of soil quality. Application of bio-resources wastes to soils after pyrolysis process is well documented to help retain water and nutrients in soils. In this study, three bio-resources wastes derived from argan shells, wood chip, a blend of paper sludge and wheat husks are characterized for physical and chemical properties. To determine the potential impact of salt stress and toxic substances the second part of this study focused on the effect these bio-resources wastes have on germination of salad and barley respectively. The three bio-resources obtained from different biomass showed some unique properties compared to the soil, such as high electrical conductivity (EC), high content of K, Na and Mg, low content of heavy metals. Moreover, the water holding capacities increased with increasing application of bio-resources wastes. Concerning the phytotoxic tests, no negative effect was observed neither for salad (Lactuca sativa L.) nor for barley (Hordeum vulgare) indicating that the three bio-resources could be safely used for agriculture. Collectively, the use of these bio-resources wastes as a soil amendment is anticipated to increase both water and nutrient and could provide the potential for a better plant growth mainly in semiarid, arid and desert climatic conditions like the case of Morocco in which the agricultural practices reserve a majority of the water resources to be used for irrigation.

  2. A case of Pitt-Hopkins syndrome with absence of hyperventilation.

    PubMed

    Inati, Adlette; Abbas, Hussein A; Korjian, Serge; Daaboul, Yazan; Harajeily, Mohamad; Saab, Raya

    2013-12-01

    Pitt-Hopkins syndrome is characterized by mental retardation, hyperventilation, and dysmorphic features due to TCF4 mutations. We report a case of Pitt-Hopkins syndrome in a 2½-year-old boy presenting with psychomotor retardation, recurrent respiratory tract infections, and dysmorphic features with absence of hyperventilation or other breathing abnormalities. Comparative genomic hybridization and quantitative real-time polymerase chain reaction were used to confirm TCF4 haploinsufficiency. Pitt-Hopkins syndrome is a rare debilitating disease that should be in the differential diagnosis of other neurodevelopmental disorders characterized by mental retardation and hypotonicity despite the absence of hyperapnea and seizures. Quantitative real-time polymerase chain reaction is another method to identify TCF4 and to confirm Pitt-Hopkins syndrome diagnosis.

  3. Gorlin-Goltz syndrome: A series of three cases

    PubMed Central

    Patankar, Amod P.; Kshirsagar, Rajesh A.; Dugal, Arun; Mishra, Akshay; Ram, Hari

    2014-01-01

    The Gorlin-Goltz syndrome (GGS) is also known as nevoid basal cell carcinoma syndrome. It is characterized by multiple keratocystic odontogenic tumors (KCOTs) in the jaw, multiple basal cell nevi carcinomas and skeletal abnormities. The syndrome may be diagnosed early by a dentist during the routine radiographic exams in the first decade of life, since the KCOTs are usually one of the first manifestations of the syndrome. This article reports the series of 3 cases, emphasizing its clinical and radiographic manifestations of GGS. PMID:25937738

  4. In vivo and in vitro functional characterization of Andersen's syndrome mutations.

    PubMed

    Bendahhou, Saïd; Fournier, Emmanuel; Sternberg, Damien; Bassez, Guillaume; Furby, Alain; Sereni, Carole; Donaldson, Matthew R; Larroque, Marie-Madeleine; Fontaine, Bertrand; Barhanin, Jacques

    2005-06-15

    The inward rectifier K(+) channel Kir2.1 carries all Andersen's syndrome mutations identified to date. Patients exhibit symptoms of periodic paralysis, cardiac dysrhythmia and multiple dysmorphic features. Here, we report the clinical manifestations found in three families with Andersen's syndrome. Molecular genetics analysis identified two novel missense mutations in the KCNJ2 gene leading to amino acid changes C154F and T309I of the Kir2.1 open reading frame. Patch clamp experiments showed that the two mutations produced a loss of channel function. When co-expressed with Kir2.1 wild-type (WT) channels, both mutations exerted a dominant-negative effect leading to a loss of the inward rectifying K(+) current. Confocal microscopy imaging in HEK293 cells is consistent with a co-assembly of the EGFP-fused mutant proteins with WT channels and proper traffick to the plasma membrane to produce silent channels alone or as hetero-tetramers with WT. Functional expression in C2C12 muscle cell line of newly as well as previously reported Andersen's syndrome mutations confirmed that these mutations act through a dominant-negative effect by altering channel gating or trafficking. Finally, in vivo electromyographic evaluation showed a decrease in muscle excitability in Andersen's syndrome patients. We hypothesize that Andersen's syndrome-associated mutations and hypokalaemic periodic paralysis-associated calcium channel mutations may lead to muscle membrane hypoexcitability via a common mechanism.

  5. In vivo and in vitro functional characterization of Andersen's syndrome mutations

    PubMed Central

    Bendahhou, Saïd; Fournier, Emmanuel; Sternberg, Damien; Bassez, Guillaume; Furby, Alain; Sereni, Carole; Donaldson, Matthew R; Larroque, Marie-Madeleine; Fontaine, Bertrand; Barhanin, Jacques

    2005-01-01

    The inward rectifier K+ channel Kir2.1 carries all Andersen's syndrome mutations identified to date. Patients exhibit symptoms of periodic paralysis, cardiac dysrhythmia and multiple dysmorphic features. Here, we report the clinical manifestations found in three families with Andersen's syndrome. Molecular genetics analysis identified two novel missense mutations in the KCNJ2 gene leading to amino acid changes C154F and T309I of the Kir2.1 open reading frame. Patch clamp experiments showed that the two mutations produced a loss of channel function. When co-expressed with Kir2.1 wild-type (WT) channels, both mutations exerted a dominant-negative effect leading to a loss of the inward rectifying K+ current. Confocal microscopy imaging in HEK293 cells is consistent with a co-assembly of the EGFP-fused mutant proteins with WT channels and proper traffick to the plasma membrane to produce silent channels alone or as hetero-tetramers with WT. Functional expression in C2C12 muscle cell line of newly as well as previously reported Andersen's syndrome mutations confirmed that these mutations act through a dominant-negative effect by altering channel gating or trafficking. Finally, in vivo electromyographic evaluation showed a decrease in muscle excitability in Andersen's syndrome patients. We hypothesize that Andersen's syndrome-associated mutations and hypokalaemic periodic paralysis-associated calcium channel mutations may lead to muscle membrane hypoexcitability via a common mechanism. PMID:15831539

  6. Characterization of air pollution control residues produced in a municipal solid waste incinerator in Portugal.

    PubMed

    Quina, Margarida J; Santos, Regina C; Bordado, João C; Quinta-Ferreira, Rosa M

    2008-04-01

    This study is mainly related with the physical and chemical characterization of a solid waste, produced in a municipal solid waste (MSW) incineration process, which is usually referred as air pollution control (APC) residue. The moisture content, loss on ignition (LOI), particle size distribution, density, porosity, specific surface area and morphology were the physical properties addressed here. At the chemical level, total elemental content (TC), total availability (TA) and the leaching behaviour with compliance tests were determined, as well as the acid neutralization capacity (ANC). The main mineralogical crystalline phases were identified, and the thermal behaviour of the APC residues is also shown. The experimental work involves several techniques such as laser diffraction spectrometry, mercury porosimetry, helium pycnometry, gas adsorption, flame atomic absorption spectrometry (FAAS), ion chromatography, scanning electron microscopy (SEM), X-ray fluorescence (XRF), X-ray diffraction (XRD) and simultaneous thermal analysis (STA). The results point out that the APC residues do not comply with regulations in force at the developed countries, and therefore the waste should be considered hazardous. Among the considered heavy metals, lead, zinc and chromium were identified as the most problematic ones, and their total elemental quantities are similar for several samples collected in an industrial plant at different times. Moreover, the high amount of soluble salts (NaCl, KCl, calcium compounds) may constitute a major problem and should be taken into account for all management strategies. The solubility in water is very high (more than 24% for a solid/liquid ratio of 10) and thus the possible utilizations of this residue are very limited, creating difficulties also in the ordinary treatments, such as in solidification/stabilization with binders.

  7. Education and Children with Down Syndrome: Neuroscience, Development, and Intervention

    ERIC Educational Resources Information Center

    Fidler, Deborah J.; Nadel, Lynn

    2007-01-01

    Of the recent advances in education-related research in Down syndrome, the characterization of the Down syndrome behavioral phenotype has become a potentially critical tool for shaping education and intervention in this population. This article briefly reviews the literature on brain-behavior connections in Down syndrome and identifies aspects of…

  8. Production and characterization of films based on blends of chitosan from blue crab (Callinectes sapidus) waste and pectin from Orange (Citrus sinensis Osbeck) peel.

    PubMed

    Baron, Ricardo Duran; Pérez, Latife Lúquez; Salcedo, Jesús Mejía; Córdoba, Luis Pérez; Sobral, Paulo José do Amaral

    2017-05-01

    The objective of this study was to develop and characterize films based on blends of chitosan and pectin, produced in laboratory scale, from industrial wastes. The chitosan was obtained by termoalcaline deacetylation of chitin, extracted from blue crab (Callinectes sapidus) waste and characterized according to degree of deacetylation (DD) and viscosimetric molecular weight (Mw); and pectin was extracted by conventional heating, from orange (Citrus sinensis Osbeck) peel and characterized according to degree of esterification (DE) and molecular weight (Mw). The Ch:P based films were prepared by the casting method in different Ch:P ratios [0: 100, 25:75, 50:50, 75:25 and 100:0], and compared to two controls [0:100 and 100:0], of commercial pectin and chitosan. Glycerol was used as a plasticizer at concentrations of 0.2g/g macromolecules. The addition of high concentrations of pectin in the formulations resulted in films with high solubility and an increase in moisture. No significant difference (P>0.05) in the degree of swelling (DS) and water vapor permeability (WVP) of the films was observed. Ch:P blend films were less stiff and therefore more elastic and flexible than films based on only one biopolymer. The control films presented better results in terms of color, being brighter and less opaque than other film formulations. These data suggest that chitosan or pectin obtained from agro-industrial waste is a potential matrix to produce biodegradable films for future food applications. Copyright © 2017 Elsevier B.V. All rights reserved.

  9. Gorlin Syndrome.

    PubMed

    Palacios-Álvarez, I; González-Sarmiento, R; Fernández-López, E

    2018-04-01

    Gorlin syndrome is a rare autosomal dominant disease caused by mutations in the sonic hedgehog signaling pathway. Of particular importance is the PTCH1 gene. The disease is characterized by the development of multiple basal cell carcinomas at young ages. These tumors may present with other skin manifestations such as palmoplantar pits and with extracutaneous manifestations such as odontogenic keratocysts and medulloblastoma. Although the dermatologist may be key for recognizing clinical suspicion of the syndrome, a multidisciplinary team is usually necessary for diagnosis, treatment, and follow-up. Skin treatment may be complicated due to the large number of basal cell carcinomas and the extent of involvement. In recent years, new drugs that inhibit targets in the sonic hedgehog pathway have been developed. Although these agents appear promising options for patients with Gorlin syndrome, their efficacy is limited by adverse effects and the development of resistance. Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.

  10. [Hyperimmunoglobulin D syndrome].

    PubMed

    Drenth, J P; Denecker, N E; Prieur, A M; Van der Meer, J W

    1995-09-16

    The hyper-IgD syndrome is a rare entity characterized by early onset of attacks of periodic fever. All patients have an elevated serum IgD (> 100 U/ml). Symptoms during attacks include joint involvements (arthralgias/arthritis), abdominal complaints (vomiting, pain, diarrhoea), skin lesions, swollen lymph nodes, and headache. In 1992 an International hyper-IgD study group was established, and to date the diagnosis has been made in 60, mainly European patients; 14 come from France. The disorder occurs in families and is transmitted by autosomal recessive inheritance. Linkage studies indicate that the gene encoding for familial Mediterranean fever is different from the gene for the hyper-IgD syndrome. In children the hyper-IgD syndrome should be distinguished from two other periodic febrile disorders. CINCA (chronic inflammatory, neurological, cutaneous and articular syndrome) and FAPA (periodic fever, adenopathies, pharyngitis, and aphtous stomatitis) share some symptoms with the hyper-IgD syndrome but in these syndromes serum IgD is normal. The pathogenesis remains to be elucidated but during attacks all patients have an acute-phase response with elevated C-reactive protein concentrations. During the febrile episodes, the inflammatory cytokines such as IL-6 TNF alpha, IFN gamma are increased together with natural occurring inhibitors such as IL-1ra and sTNFr. There is no therapy for the syndrome and patients will experience attacks during their entire life although frequency and severity tend to diminish with age.

  11. Tank characterization report for single-shell tank 241-C-109

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Simpson, B.C.

    1997-05-23

    One of the major functions of the Tank Waste Remediation System (TWRS) is to characterize wastes in support of waste management and disposal activities at the Hanford Site. Analytical data from sampling and analysis, along with other available information about a tank, are compiled and maintained in a tank characterization report (TCR). This report and its appendices serve as the TCR for single-shell tank 241-C-109. The objectives of this report are: (1) to use characterization data in response to technical issues associated with tank 241 C-109 waste; and (2) to provide a standard characterization of this waste in terms ofmore » a best-basis inventory estimate. The response to technical issues is summarized in Section 2.0, and the best-basis inventory estimate is presented in Section 3.0. Recommendations regarding safety status and additional sampling needs are provided in Section 4.0. Supporting data and information are contained in the appendices.« less

  12. Electrochemical/Pyrometallurgical Waste Stream Processing and Waste Form Fabrication

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Steven Frank; Hwan Seo Park; Yung Zun Cho

    This report summarizes treatment and waste form options being evaluated for waste streams resulting from the electrochemical/pyrometallurgical (pyro ) processing of used oxide nuclear fuel. The technologies that are described are South Korean (Republic of Korea – ROK) and United States of America (US) ‘centric’ in the approach to treating pyroprocessing wastes and are based on the decade long collaborations between US and ROK researchers. Some of the general and advanced technologies described in this report will be demonstrated during the Integrated Recycle Test (IRT) to be conducted as a part of the Joint Fuel Cycle Study (JFCS) collaboration betweenmore » US Department of Energy (DOE) and ROK national laboratories. The JFCS means to specifically address and evaluated the technological, economic, and safe guard issues associated with the treatment of used nuclear fuel by pyroprocessing. The IRT will involve the processing of commercial, used oxide fuel to recover uranium and transuranics. The recovered transuranics will then be fabricated into metallic fuel and irradiated to transmutate, or burn the transuranic elements to shorter lived radionuclides. In addition, the various process streams will be evaluated and tested for fission product removal, electrolytic salt recycle, minimization of actinide loss to waste streams and waste form fabrication and characterization. This report specifically addresses the production and testing of those waste forms to demonstrate their compatibility with treatment options and suitability for disposal.« less

  13. Clinical presentation of Churg-Strauss syndrome in children: A 12-year-old-boy with ANCA-negative Churg-Strauss syndrome.

    PubMed

    Razenberg, Femke G E M; Heynens, Jan W C M; Jan de Vries, Geeuwke; Duijts, Liesbeth; de Jongste, Johan C; de Blic, Jacques; Rosias, Philippe P R

    2012-01-01

    Churg-Strauss syndrome is an uncommon multisystem disorder characterized by asthma, eosinophilia and vasculitis. We report on a 12-year-old boy with asthma and deterioration of his general condition, who was eventually diagnosed with an ANCA-negative Churg-Strauss syndrome. The propositus included, 50 cases of childhood Churg-Strauss syndrome have been reported. The patient characteristics and clinical characteristics of these children are summarized. The respiratory tract is most frequently involved with pulmonary infiltrates, asthma and sinusitis. Early recognition of childhood Churg-Strauss syndrome is important as delayed diagnosis can lead to severe organ involvement, and possible fatal outcome.

  14. Autoimmune lymphoproliferative syndrome: more than a FAScinating disease

    PubMed Central

    Bride, Karen; Teachey, David

    2017-01-01

    Autoimmune lymphoproliferative syndrome (ALPS) is an inherited syndrome characterized by abnormal lymphocyte survival caused by failure of apoptotic mechanisms to maintain lymphocyte homeostasis. This failure leads to the clinical manifestations of non-infectious and non-malignant lymphadenopathy, splenomegaly, and autoimmune pathology, most commonly, autoimmune cytopenias. Since ALPS was first characterized in the early 1990s, insights in disease biology have improved both diagnosis and management of this syndrome. Sirolimus is the best-studied and most effective corticosteroid-sparing therapy for ALPS and should be considered first-line for patients in need of chronic treatment. This review highlights practical clinical considerations for the diagnosis and management of ALPS. Further studies could reveal new proteins and regulatory pathways that are critical for lymphocyte activation and apoptosis. PMID:29123652

  15. The Hallermann-Streiff Syndrome

    ERIC Educational Resources Information Center

    Nevin, N. C.; And Others

    1974-01-01

    Described are the physical and genetic factors in a case of Hallermann-Streiff Syndrome (characterized by dwarfism and other anomalies, with or without retardation) in an 11-year old severely physically and mentally handicapped Irish girl. (CL)

  16. 40 CFR 761.347 - First level sampling-waste from existing piles.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... Purposes of Characterization for PCB Disposal in Accordance With § 761.62, and Sampling PCB Remediation... a cone; that is, having a circular base with PCB bulk product waste or PCB remediation waste... one pile. If the PCB bulk product waste or PCB remediation waste consists of more than one pile or...

  17. Childhood moyamoya disease accompanying Leigh syndrome.

    PubMed

    Cullu, Nesat; Karakas, Ekrem; Karakas, Omer; Deveer, Mehmet; Calik, Mustafa; Boyaci, Fatima Nurefsan

    2013-12-01

    Moyamoya disease is a cerebrovasculopathy of unknown etiology during the course of which the main and terminal veins of the internal carotid artery undergo progressive vein occlusion. Leigh syndrome is a mitochondrial encephalomyopathy that occurs due to "cytochrome c oxidase deficiency" characterized by psychomotor retardation, difficulty in eating, seizures, hypotonia, respiratory disorders and high lactate levels. Many diseases and syndromes have been defined that are associated with Moyamoya disease. To the best of our knowledge, the association of moyamoya disease with Leigh syndrome has not been defined as yet. In this study, the clinical and imaging results of a 3-year-old male child displaying the association of Moyamoya disease and Leigh syndrome are presented.

  18. Hypercalcemia in tumor lysis syndrome.

    PubMed

    Shah, Binay Kumar

    2014-09-01

    Tumor lysis syndrome (TLS) is characterized by hyperkalemia, hyperuricemia, hypocalcemia and hyperphosphatemia. This report describes a case of hypercalcemia in TLS in a patient with diffuse large B cell lymphoma.

  19. Characterization and Leaching Tests of the Fluidized Bed Steam Reforming (FBSR) Waste Form for LAW Immobilization - 13400

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Neeway, James J.; Qafoku, Nikolla P.; Peterson, Reid A.

    2013-07-01

    Several supplemental technologies for treating and immobilizing Hanford low activity waste (LAW) have been evaluated. One such immobilization technology is the Fluidized Bed Steam Reforming (FBSR) granular product. The FBSR granular product is composed of insoluble sodium aluminosilicate (NAS) feldspathoid minerals. Production of the FBSR mineral product has been demonstrated both at the industrial and laboratory scale. Pacific Northwest National Laboratory (PNNL) was involved in an extensive characterization campaign. The goal of this campaign was to study the durability of the FBSR mineral product and the encapsulated FBSR product in a geo-polymer monolith. This paper gives an overview of resultsmore » obtained using the ASTM C 1285 Product Consistency Test (PCT), the EPA Test Method 1311 Toxicity Characteristic Leaching Procedure (TCLP), and the ASTMC 1662 Single-Pass Flow-Through (SPFT) test. Along with these durability tests an overview of the characteristics of the waste form has been collected using Scanning Electron Microscopy (SEM), X-ray Diffraction (XRD), microwave digestions for chemical composition, and surface area from Brunauer, Emmett, and Teller (BET) theory. (authors)« less

  20. [Blue rubber bleb naevus syndrome].

    PubMed

    Akutko, Katarzyna; Krzesiek, Elzbieta; Iwańczak, Barbara

    2012-10-01

    Blue rubber bleb nevus syndrome (Bean syndrome) is a rare disease characterized by the presence of multiple vascular malformation of rubber-like consistence. This disease is of a genetic origin and most often is caused by sporadic mutation, however, exist reports on autosomal dominant type of heritance. Nevi are most frequently met in the skin and alimentary tract but may be present in all organs and tissues. The most frequent symptom of Bean syndrome is anaemia due to ferrum deficiency, which is a result of chronic hemorrhagia from vascular malformations in the alimentary tract. Vascular anomalies on the skin are usually asymptomatic. Other symptoms are less frequent and depend on the localisation of vascular changes and therefore patients with Bean syndrome require meticulous analysis of reported multiple - specialistic medical care.

  1. WOOD PRODUCTS IN THE WASTE STREAM: CHARACTERIZATION AND COMBUSTION EMISSIONS - VOLUME 2. APPENDICES

    EPA Science Inventory

    The report gives results of a study of technical, public policy, and regulatory issues that affect the processing and combustion of waste wood for fuel. (NOTE: Waste wood is wood that is separated from a solid-waste stream, processed into a uniform-sized product, and reused for o...

  2. Active and passive computed tomography mixed waste focus area final report

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Roberson, G P

    1998-08-19

    The Mixed Waste Focus Area (MWFA) Characterization Development Strategy delineates an approach to resolve technology deficiencies associated with the characterization of mixed wastes. The intent of this strategy is to ensure the availability of technologies to support the Department of Energy's (DOE) mixed waste low-level or transuranic (TRU) contaminated waste characterization management needs. To this end the MWFA has defined and coordinated characterization development programs to ensure that data and test results necessary to evaluate the utility of non-destructive assay technologies are available to meet site contact handled waste management schedules. Requirements used as technology development project benchmarks are basedmore » in the National TRU Program Quality Assurance Program Plan. These requirements include the ability to determine total bias and total measurement uncertainty. These parameters must be completely evaluated for waste types to be processed through a given nondestructive waste assay system constituting the foundation of activities undertaken in technology development projects. Once development and testing activities have been completed, Innovative Technology Summary Reports are generated to provide results and conclusions to support EM-30, -40, or -60 end user/customer technology selection. The Active and Passive Computed Tomography non-destructive assay system is one of the technologies selected for development by the MWFA. Lawrence Livermore National Laboratory's (LLNL) is developing the Active and Passive Computed Tomography (A&PCT) nondestructive assay (NDA) technology to identify and accurately quantify all detectable radioisotopes in closed containers of waste. This technology will be applicable to all types of waste regardless of .their classification; low level, transuranic or provide results and conclusions to support EM-30, -40, or -60 end user/customer technology selection. The Active and Passive Computed Tomography non

  3. Multiple jaw cysts-unveiling the Gorlin-Goltz syndrome.

    PubMed

    Manjima, S; Naik, Zameera; Keluskar, Vaishali; Bagewadi, Anjana

    2015-03-01

    Gorlin-Goltz syndrome or basal cell nevus syndrome is a comparatively rare syndrome characterized by basal cell nevi, odontogenic keratocysts, and skeletal anomalies. Diagnosis is based on the major and minor clinical and radiographic criteria. Dentist plays a major role in the diagnosis of this disease due to the oral and maxillofacial manifestations of the syndrome. In some cases, jaw cysts are diagnosed by routine radiographs advised by the dentists. Odontogenic keratocysts in such syndromic patients will be multiple and extensive and in some cases results in cortical expansion and facial disfigurement. Thorough clinical examination and investigations prompt an early confirmation of the syndrome, which is very essential to avoid morbidity associated with the syndrome. Here, we report a case of multiple odontogenic cysts in a 16-year-old patient which later was diagnosed as a case of Gorlin Goltz syndrome.

  4. Multiple jaw cysts-unveiling the Gorlin-Goltz syndrome

    PubMed Central

    Manjima, S.; Naik, Zameera; Keluskar, Vaishali; Bagewadi, Anjana

    2015-01-01

    Gorlin-Goltz syndrome or basal cell nevus syndrome is a comparatively rare syndrome characterized by basal cell nevi, odontogenic keratocysts, and skeletal anomalies. Diagnosis is based on the major and minor clinical and radiographic criteria. Dentist plays a major role in the diagnosis of this disease due to the oral and maxillofacial manifestations of the syndrome. In some cases, jaw cysts are diagnosed by routine radiographs advised by the dentists. Odontogenic keratocysts in such syndromic patients will be multiple and extensive and in some cases results in cortical expansion and facial disfigurement. Thorough clinical examination and investigations prompt an early confirmation of the syndrome, which is very essential to avoid morbidity associated with the syndrome. Here, we report a case of multiple odontogenic cysts in a 16-year-old patient which later was diagnosed as a case of Gorlin Goltz syndrome. PMID:25821359

  5. Flowsheets and source terms for radioactive waste projections

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Forsberg, C.W.

    1985-03-01

    Flowsheets and source terms used to generate radioactive waste projections in the Integrated Data Base (IDB) Program are given. Volumes of each waste type generated per unit product throughput have been determined for the following facilities: uranium mining, UF/sub 6/ conversion, uranium enrichment, fuel fabrication, boiling-water reactors (BWRs), pressurized-water reactors (PWRs), and fuel reprocessing. Source terms for DOE/defense wastes have been developed. Expected wastes from typical decommissioning operations for each facility type have been determined. All wastes are also characterized by isotopic composition at time of generation and by general chemical composition. 70 references, 21 figures, 53 tables.

  6. [Molecular updates on Usher syndrome].

    PubMed

    Roux, A-F

    2005-01-01

    Usher syndrome (USH) is an autosomal recessive disorder characterized by the association of sensorineural hearing loss and retinitis pigmentosa (RP). Usher syndrome is both clinically and genetically heterogeneous. Three clinical subtypes are defined with respect to vestibular dysfunction and the degree of hearing loss. Type I (USH1) patients have profound hearing loss and vestibular dysfunction from birth. Type II (USH2) is the most frequent and patients tend to have less severe hearing impairment and normal vestibular response. Type III (USH3) is characterized by a progressive loss of hearing and is found more frequently among Finnish patients. Recently, major breakthroughs have been made in the molecular genetics of Usher syndrome as a number of chromosomal loci and causative genes have been identified in each clinical subtype. Twelve loci are known and the corresponding genes have been cloned for six of them. Although their functions are not always clearly established, a common role is emerging for the proteins identified within each subtype. As a result, each subtype could emanate from defects affecting distinct cellular mechanisms.

  7. Prevention of infectious diseases in patients with Good syndrome.

    PubMed

    Multani, Ashrit; Gomez, Carlos A; Montoya, José G

    2018-08-01

    Good syndrome is a profoundly immunocompromising condition with heterogeneous immune deficits characterized by the presence of thymoma, low-to-absent B-lymphocyte counts, hypogammaglobulinemia, and impaired cell-mediated immunity. Opportunistic infectious diseases associated with Good syndrome represent a diagnostic and therapeutic challenge, given their protean clinical manifestations. Although these infectious complications have been reviewed in prior publications, recommendations regarding their prevention have been lacking. Good syndrome usually occurs in adult patients between the ages of 40 and 70 years. Immunologically, it is characterized by low or absent peripheral blood B lymphocytes, hypogammaglobulinemia, and variable defects in cell-mediated immunity including low CD4 T counts, inverted CD4:CD8 T-lymphocyte ratio, and reduced T-lymphocyte mitogen proliferative responses. Patients with Good syndrome are susceptible to a variety of infectious diseases, of which the most common are recurrent bacterial sinopulmonary infections, mucocutaneous candidiasis, and CMV tissue-invasive disease. Preventive guidelines including targeted antimicrobial prophylaxis and vaccination strategies can mitigate infectious complications in patients with Good syndrome. Immunological deficits and infectious complications in Good syndrome have been described for over 60 years. Further research is needed to elucidate its exact pathogenesis and define the mechanistic relationship between thymoma and hypogammaglobulinemia. However, tailored prophylactic strategies can be recommended for patients with Good syndrome.

  8. Characterization of Non-pertechnetate Species Relevant to the Hanford Tank Waste

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Chatterjee, Sayandev; Andersen, Amity; Du, Yingge

    Among radioactive constituents present in the tank waste stored at the U.S. DOE Hanford Site, technetium-99 (Tc), which is generated from the fission of 235U and 239Pu in high yields, presents a unique challenge in that it has a long half-life ( = 292 keV; T1/2 = 2.11105 y) and exists predominately in soluble forms in the liquid supernatant and salt cake fractions of the waste. In the strongly alkaline environments prevalent in most of the tank waste, its dominant chemical form is pertechnetate (TcO 4 -, oxidation state +7). However, attempts to remove Tc from the Hanford tank wastemore » using ion-exchange processes specific to TcO 4 - only met with limited success, particularly when processing tank waste samples containing elevated concentrations of organic complexants. This suggests that a significant fraction of the soluble Tc can be present as low-valent Tc (oxidation state < +7) (non-pertechnetate). The chemical identities of these non-pertechnetate species are poorly understood. Previous analysis of the SY-101 and SY-103 tank waste samples provided strong evidence that non-pertechnetate can be comprised of [fac-Tc(CO) 3] + complexes containing Tc in oxidation state +1 (Lukens et al. 2004). During the last three years, our team has expanded this work and demonstrated that high-ionic-strength solutions typifying tank waste supernatants promote oxidative stability of the [fac-Tc(CO) 3] + species (Rapko et al. 2013a; 2013b; Levitskaia et al. 2014; Chatterjee et al. 2015). Obtained results also suggest possible stabilization of Tc(VI) and potentially Tc(IV) oxidation states in the high-ionic-strength alkaline matrices particularly in the presence of organic chelators, so that Tc(IV, VI) can serve as important redox intermediates facilitating the reduction of Tc(VII) to Tc(I). Designing strategies for effective Tc management, including separation and immobilization, necessitates understanding the molecular structure of the non-pertechnetate species and their

  9. The Episodic Syndromes That Maybe Associated with Migraines.

    PubMed

    Lebron, Diana; Vasconcellos, Elza

    2016-02-01

    The "childhood periodic syndromes" have been renamed "the episodic syndromes that maybe associated with migraines". These syndromes were initially considered precursors of migraines that only occurred in childhood; however recent literature suggests that the episodic syndromes can occur in adults with known migraine and does not necessarily present as a precursor. This review article discusses the recent literature regarding the episodic syndromes and potential treatments. These disorders are seen by multiple subspecialists, therefore it is important to recognize and use the same definitions, criteria and nomenclature. A collaborative and multidisciplinary approach is critical to characterize, manage and potentially improve outcomes. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Genetic mutations in Gorlin-Goltz syndrome.

    PubMed

    Daneswari, Muthumula; Reddy, Mutjumula Swamy Ranga

    2013-07-01

    Gorlin-Goltz syndrome is a rare multisystemic disease inherited in a dominant autosomal at a high level of penetrance and variable expressiveness. It is mainly characterized by basal cell carcinoma, odontogenic keratocyst and skeletal anomalies. Diagnosis is based upon established major and minor clinical and radiographic criteria and gene mutation analysis. This article presents a case of Gorlin-Goltz syndrome, its genetic predisposition, diagnosis and management.

  11. Waste minimization for commercial radioactive materials users generating low-level radioactive waste. Revision 1

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Fischer, D.K.; Gitt, M.; Williams, G.A.

    1991-07-01

    The objective of this document is to provide a resource for all states and compact regions interested in promoting the minimization of low-level radioactive waste (LLW). This project was initiated by the Commonwealth of Massachusetts, and Massachusetts waste streams have been used as examples; however, the methods of analysis presented here are applicable to similar waste streams generated elsewhere. This document is a guide for states/compact regions to use in developing a system to evaluate and prioritize various waste minimization techniques in order to encourage individual radioactive materials users (LLW generators) to consider these techniques in their own independent evaluations.more » This review discusses the application of specific waste minimization techniques to waste streams characteristic of three categories of radioactive materials users: (1) industrial operations using radioactive materials in the manufacture of commercial products, (2) health care institutions, including hospitals and clinics, and (3) educational and research institutions. Massachusetts waste stream characterization data from key radioactive materials users in each category are used to illustrate the applicability of various minimization techniques. The utility group is not included because extensive information specific to this category of LLW generators is available in the literature.« less

  12. [Mania associated with Usher syndrome type II].

    PubMed

    Praharaj, Samir Kumar; Acharya, Mahima; Sarvanan, Arul; Kongasseri, Sreejayan; Behere, Rishikesh V; Sharma, P S V N

    2012-01-01

    Usher syndrome (or Hallgren syndrome) is an autosomal recessive genetic disorder characterized by sensorineural deafness, retinitis pigmentosa, and variable vestibular deficit; Usher syndrome type II is the most common form. Various neuropsychiatric disorders have been reported to occur in those with Usher syndrome, including schizophrenia-like disorder, atypical psychosis, recurrent depressive illness, neurotic disorder, and mental retardation; however, bipolar disorder is not common in those with Usher syndrome. Herein we describe a 30-year-old male with Usher syndrome type II that developed features indicative of a probable manic episode. The patient had complete remission of symptoms in response to treatment with olanzapine 20 mg d-1. In persons with dual sensory impairment there are inherent problems with assessment and diagnosis is difficult due to their limited communication abilities. The diagnosis of Usher syndrome depends heavily on behavioral observation and disturbances in vegetative functions.

  13. Gorlin-Goltz syndrome

    PubMed Central

    Şereflican, Betül; Tuman, Bengü; Şereflican, Murat; Halıcıoğlu, Sıddıka; Özyalvaçlı, Gülzade; Bayrak, Seval

    2017-01-01

    Gorlin-Goltz syndrome is a rare multisystemic disease inherited in an autosomal dominant pattern. It is characterized by numerous basal cell carcinoma of the skin, jaw cysts, and skeletal anomalies such as frontal bossing, vertebral anomalies, palmoplantar pits, and falx cerebri calcification. There is a tendency to tumors including medullablastoma, fibroma, rabdomyoma, leiomyosarcoma etc.. The diagnosis is based on major and minor clinical and radiologic criteria. Early diagnosis and treatment are of utmost importance in reducing the severity of long-term sequelae of this syndrome. In this article, we present a 15-year-old boy who was admitted to our clinic with brown-black papules and plaques on his scalp and was thought to have Gorlin-Goltz syndrome. He had a history of medulloblastoma that was treated with surgical resection followed by cranial radiotherapy and unilateral retinoblastoma. We present this case, because association of Gorlin-Goltz syndrome and retinoblastoma has not been described previously in the literature and we aimed to draw attention to radiation-induced basal cell carcinomas. PMID:29062253

  14. Gorlin-Goltz syndrome.

    PubMed

    Şereflican, Betül; Tuman, Bengü; Şereflican, Murat; Halıcıoğlu, Sıddıka; Özyalvaçlı, Gülzade; Bayrak, Seval

    2017-09-01

    Gorlin-Goltz syndrome is a rare multisystemic disease inherited in an autosomal dominant pattern. It is characterized by numerous basal cell carcinoma of the skin, jaw cysts, and skeletal anomalies such as frontal bossing, vertebral anomalies, palmoplantar pits, and falx cerebri calcification. There is a tendency to tumors including medullablastoma, fibroma, rabdomyoma, leiomyosarcoma etc.. The diagnosis is based on major and minor clinical and radiologic criteria. Early diagnosis and treatment are of utmost importance in reducing the severity of long-term sequelae of this syndrome. In this article, we present a 15-year-old boy who was admitted to our clinic with brown-black papules and plaques on his scalp and was thought to have Gorlin-Goltz syndrome. He had a history of medulloblastoma that was treated with surgical resection followed by cranial radiotherapy and unilateral retinoblastoma. We present this case, because association of Gorlin-Goltz syndrome and retinoblastoma has not been described previously in the literature and we aimed to draw attention to radiation-induced basal cell carcinomas.

  15. Behçet's syndrome pathophysiology and potential therapeutic targets.

    PubMed

    Emmi, Giacomo; Silvestri, Elena; Squatrito, Danilo; D'Elios, Mario Milco; Ciucciarelli, Lucia; Prisco, Domenico; Emmi, Lorenzo

    2014-04-01

    Behçet syndrome is a systemic inflammatory disorder characterized by multiorgan involvement such as oral and genital ulcers, uveitis, skin lesions as well as by less frequent, but often more severe, central nervous system and vascular manifestations. The pathogenetic mechanisms are still incompletely known; however the interaction between a specific genetic background and environmental or infectious factors certainly contributes to the immune dysregulation that characterizes this disease. The discovery of new immunological pathways in Behçet syndrome pathogenesis may help us to set up new treatments. In this review, we will focus our attention on the possible mechanisms underlying Behçet syndrome pathogenesis and their potential role as novel therapeutic targets.

  16. Glass Waste Forms for Oak Ridge Tank Wastes: Fiscal Year 1998 Report for Task Plan SR-16WT-31, Task B

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Andrews, M.K.

    1999-05-10

    Using ORNL information on the characterization of the tank waste sludges, SRTC performed extensive bench-scale vitrification studies using simulants. Several glass systems were tested to ensure the optimum glass composition (based on the glass liquidus temperature, viscosity and durability) is determined. This optimum composition will balance waste loading, melt temperature, waste form performance and disposal requirements. By optimizing the glass composition, a cost savings can be realized during vitrification of the waste. The preferred glass formulation was selected from the bench-scale studies and recommended to ORNL for further testing with samples of actual OR waste tank sludges.

  17. Audiological findings in Noonan syndrome.

    PubMed

    Tokgoz-Yilmaz, Suna; Turkyilmaz, Meral Didem; Cengiz, Filiz Basak; Sjöstrand, Alev Pektas; Kose, Serdal Kenan; Tekin, Mustafa

    2016-10-01

    The aim of this study was to evaluate audiologic properties of patients with Noonan syndrome and compare these findings with those of unaffected peers. The study included 17 children with Noonan syndrome and 20 typically developing children without Noonan syndrome. Pure tone and speech audiometry, immitancemetric measurement, otoacoustic emissions measurement and auditory brainstem response tests were applied to all (n = 37) children. Hearing thresholds of children with Noonan syndrome were higher (poorer) than those observed unaffected peers, while the hearing sensitivity of the both groups were normal limits (p = 0.013 for right, p = 0.031 for left ear). Transient evoked otoacoustic emissions amplitudes of the children with Noonan syndrome were lower than the children without Noonan syndrome (p = 0.005 for right, p = 0.002 for left ear). Middle ear pressures and auditory brainstem response values were within normal limits and there was no difference between the two groups (p > 0.05). General benefit of the present study is to characterize the audiologic findings of children with Noonan syndrome, which is beneficial in clinics evaluating children with Noonan syndrome. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  18. Gorlin syndrome (nevoid basal cell carcinoma syndrome): update and literature review.

    PubMed

    Fujii, Katsunori; Miyashita, Toshiyuki

    2014-10-01

    Gorlin syndrome, also called nevoid basal cell carcinoma syndrome, is an autosomal dominant neurocutaneous disease characterized by developmental anomalies such as palmar pits and rib anomaly, and tumorigenesis such as medulloblastoma and basal cell carcinoma. This syndrome is mainly caused by a mutation of PTCH1, a human homologue of Drosophila patched, including frameshift, missense, or nonsense mutations. Genotype-phenotype correlation has not been established. PTCH1 is a member of hedgehog signaling, which is a highly conserved pathway in vertebrates, composed of hedgehog, SMO, and GLI proteins as well as PTCH1. Given that hedgehog signaling regulates cell growth and development, disorder of this pathway gives rise to not only developmental anomalies but also diverse tumors such as those seen in Gorlin syndrome. We recently reported, for the first time, a nationwide survey of Gorlin syndrome in Japan, noting that the frequency was 1/235,800 in the Japanese population, and that the frequency of basal cell carcinomas was significantly lower in Japan than in the USA and Europe, suggesting that ethnicity and genetic background contribute to these differences. Given that many clinical trials using newly discovered molecular inhibitors are still ongoing, these agents should become the new therapeutic options for hedgehog pathway-dependent tumors in patients with or without Gorlin syndrome. © 2014 Japan Pediatric Society.

  19. [Practical update of Tsaktubo syndrome].

    PubMed

    Núñez-Gil, Iván J; Mejía-Rentería, Hernán D; Martínez-Losas, Pedro

    2016-03-04

    Takotsubo syndrome, apical ballooning or «broken heart» syndrome, is a growing diagnostic entity which clinically mimics an acute coronary syndrome. Included into the stress cardiomyopathy group of cardiopathies, this condition is characterized by the absence of potentially responsible coronary lesions, while displaying a transient abnormal ventricular motion, usually affecting various coronary territories. It is generally observed in postmenopausal women and frequently seen in the presence of a stressful situation, both physical and emotional. With a prevalence of 1.2% among patients undergoing a cardiac catheterization with a suspected diagnosis of acute coronary syndromes, Takotsubo syndrome usually has a good prognosis. However, complications can occur in the acute phase, generally heart failure, which can even lead to death. In this review we discuss the latest available information on this disease and present it in a practical and useful way for the attending physician. Copyright © 2015 Elsevier España, S.L.U. All rights reserved.

  20. [Joubert syndrome and related disorders].

    PubMed

    Paprocka, Justyna; Jamroz, Ewa

    2012-01-01

    The cerebellum plays a role not only in motor control but also in motor learning and cognition. Joubert syndrome is a rare heterogeneous inherited genetic disorder characterized by ataxia, hypotonia, developmental delay, and at least one of the following features: neonatal respiratory disturbances or abnormal eye movement. The estimated frequency of Joubert syndrome in the United States is around 1 : 100 000. The term Joubert syndrome and related disorders (JSRD) has been recently coined to describe all disorders presenting with molar tooth sign on brain neuroimaging. Joubert syndrome is believed to be a representative of a new group of disorders named ciliopathies. The identification of seven causal genes (NPHP1, AHI1, CEP290, RPGRIP1L, TMEM67/MKS3, ARL13B, CC2D2A) has led to substantial progress in the understanding of the genetic basis of Joubert syndrome. The authors focus on clinical presentation of JSRD, differential diagnosis and molecular background.

  1. FGF-23 in fibrous dysplasia of bone and its relationship to renal phosphate wasting

    PubMed Central

    Riminucci, Mara; Collins, Michael T.; Fedarko, Neal S.; Cherman, Natasha; Corsi, Alessandro; White, Kenneth E.; Waguespack, Steven; Gupta, Anurag; Hannon, Tamara; Econs, Michael J.; Bianco, Paolo; Gehron Robey, Pamela

    2003-01-01

    FGF-23, a novel member of the FGF family, is the product of the gene mutated in autosomal dominant hypophosphatemic rickets (ADHR). FGF-23 has been proposed as a circulating factor causing renal phosphate wasting not only in ADHR (as a result of inadequate degradation), but also in tumor-induced osteomalacia (as a result of excess synthesis by tumor cells). Renal phosphate wasting occurs in approximately 50% of patients with McCune-Albright syndrome (MAS) and fibrous dysplasia of bone (FD), which result from postzygotic mutations of the GNAS1 gene. We found that FGF-23 is produced by normal and FD osteoprogenitors and bone-forming cells in vivo and in vitro. In situ hybridization analysis of FGF-23 mRNA expression identified “fibrous” cells, osteogenic cells, and cells associated with microvascular walls as specific cellular sources of FGF-23 in FD. Serum levels of FGF-23 were increased in FD/MAS patients compared with normal age-matched controls and significantly higher in FD/MAS patients with renal phosphate wasting compared with those without, and correlated with disease burden bone turnover markers commonly used to assess disease activity. Production of FGF-23 by FD tissue may play an important role in the renal phosphate–wasting syndrome associated with FD/MAS. PMID:12952917

  2. 40 CFR 761.345 - Form of the waste to be sampled.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... Characterization for PCB Disposal in Accordance With § 761.62, and Sampling PCB Remediation Waste Destined for Off-Site Disposal, in Accordance With § 761.61 § 761.345 Form of the waste to be sampled. PCB bulk product waste and PCB remediation waste destined for off-site disposal must be in the form of either flattened...

  3. Gorlin syndrome and bilateral ovarian fibroma

    PubMed Central

    Pirschner, Fernanda; Bastos, Pollyana Marçal; Contarato, George Luiz; Bimbato, Anna Carolina Bon Lima; Filho, Antônio Chambô

    2012-01-01

    INTRODUCTION Gorlin syndrome (GS), also known as nevoid basal cell carcinoma syndrome (NBCCS), is a rare hereditary, autosomal dominant disease that affects various systems. Its prevalence is estimated at 1/57,000 to 1/256,000 of the population. It is characterized by basal cell carcinomas, multiple odontogenic keratocysts, skeletal abnormalities and ovarian fibroma, among other disorders. PRESENTATION OF CASE To report the case of a young patient with Gorlin syndrome and bilateral ovarian fibroma. DISCUSSION A 20-year old patient with Gorlin syndrome presented with facial asymmetry, broad nasal root, dental abnormalities, micrognathism, convergent strabismus, multiple pigmented lesions on the trunk and face, pectus excavatum, kyphoscoliosis and a palpable mass in the abdomen occupying the entire pelvic region. CONCLUSION Gorlin–Goltz syndrome is a hereditary pathology that includes numerous clinical manifestations. Diagnosis is clinical and genetic confirmation is unnecessary. PMID:22771908

  4. Understanding and preventing computer vision syndrome.

    PubMed

    Loh, Ky; Redd, Sc

    2008-01-01

    The invention of computer and advancement in information technology has revolutionized and benefited the society but at the same time has caused symptoms related to its usage such as ocular sprain, irritation, redness, dryness, blurred vision and double vision. This cluster of symptoms is known as computer vision syndrome which is characterized by the visual symptoms which result from interaction with computer display or its environment. Three major mechanisms that lead to computer vision syndrome are extraocular mechanism, accommodative mechanism and ocular surface mechanism. The visual effects of the computer such as brightness, resolution, glare and quality all are known factors that contribute to computer vision syndrome. Prevention is the most important strategy in managing computer vision syndrome. Modification in the ergonomics of the working environment, patient education and proper eye care are crucial in managing computer vision syndrome.

  5. Synthesis and Characterization of Tc(I) Carbonyl Nitrosyl Species Relevant to the Hanford Tank Waste: FY 2016 Status Report

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hall, Gabriel B.; Chatterjee, Sayandev; Levitskaia, Tatiana G.

    Among long-lived radioactive constituents in the Hanford tank waste, Tc presents a unique challenge in that it exists predominantly in the liquid phase, generally in the anionic form of pertechnetate, TcO 4 -, which is highly volatile at low-activity waste (LAW) vitrification melter temperatures and mobile in the Hanford site’s subsurface environment. The complex behavior of Tc under storage, treatment, and immobilization conditions significantly affects its management options, which to-date remain uncertain. In strongly alkaline environments, Tc exists as pertechnetate, TcO 4 - (oxidation state +7), and in the reduced forms (oxidation state < +7) collectively known as non-pertechnetate species.more » Pertechnetate is a well-characterized, anionic Tc species that can be removed from LAW by anion exchange or solvent extraction methods. There is no definitive information on the origin of the non-pertechnetate Tc species, nor is there a comprehensive description of their composition and behavior. It has been recently proposed that the non-pertechnetate species can comprise Tc(I) metal center and carbonyl or mixed carbonyl nitrosyl ligands stabilizing low-valent Tc. Recent work by our group has significantly expanded this previous work, generating a series of Tc(I) carbonyl compounds and demonstrating that they can be generated from reduction of TcO 4 - in the simulated Hanford tank waste in presence of CO at elevated temperature (Levitskaia et al. 2014). These results are consistent with the previous proposal that [Tc(CO) 3] + species can be present in the Hanford tank waste and suggest that the low Tc(I) oxidation state is stabilized by the π-accepting ability of the CO ligands. The continuation work has been initiated to develop model Tc carbonyl nitrosyl compounds and investigate their potential presence in the Hanford tank wastes. This report summarizes our to-date results.« less

  6. Clinical characterization and mutation spectrum in Hispanic families with adenomatous polyposis syndromes.

    PubMed

    Cruz-Correa, Marcia; Diaz-Algorri, Yaritza; Mendez, Vanessa; Vazquez, Pedro Juan; Lozada, Maria Eugenia; Freyre, Katerina; Lathroum, Liselle; Gonzalez-Pons, Maria; Hernandez-Marrero, Jessica; Giardiello, Francis; Rodriguez-Quilichini, Segundo

    2013-09-01

    Several genetically defined hereditary colorectal cancer (CRC) syndromes are associated with colonic polyposis including familial adenomatous polyposis (FAP) and MUTYH adenomatous polyposis (MAP). Limited data exists on the clinical characterization and genotypic spectrum of polyposis syndromes among Hispanics. To describe the phenotype and genotype of Puerto Rican Hispanic patients with FAP and MUTYH and compare with other ethnic and racial groups. Probands were identified from the Puerto Rico Familial Colorectal Cancer Registry (PURIFICAR). Recruited individuals completed risk factors, medical, and family history questionnaires and underwent genetic testing for genotype analysis. Frequency analysis, Chi square, Fisher's exact and Wilcoxon rank-sum tests were used for statistical analysis methods. A total of 31 FAP (from 19 families) and 13 MAP (from 13 families) Hispanic patients recruited from the PURIFICAR were evaluated. Among the FAP cases, mean age at diagnosis was 27.6 (range 9-71 years); 67.7 % cases had more than 100 polyps and 41.9 % had upper gastrointestinal polyps. Among the 19 FAP families, there were 77 affected FAP individuals and 26 colorectal cancer cases. Genetic mutations were available for 42.2 % of FAP families; all mutations identified were unique. Surgeries were reported in 31 cases; 14 (45.2 %) prophylactic surgeries and 6 (19.4 %) therapeutic surgeries for management of CRC. Among MAP cases, mean age at diagnosis was 53 (range 34-76 years). Genetic analysis revealed homozygous biallelic mutations (G382D) in 53.8 %, compound heterozygous mutations (G382/Y165C) in 23 %, and non-G382/Y165C monoallelic mutations in 23 %. Familial cancer registries should be promoted as vehicles for detection, education and follow up of families at-risk of acquiring familial cancers. PURIFICAR is the first and only familial cancer registry in Puerto Rico providing these services to families affected with familial cancer syndromes promoting education, testing

  7. Sotos Syndrome. Clinical Exchange.

    ERIC Educational Resources Information Center

    Shuey, Elaine M.; Jamison, Kristen

    1996-01-01

    Sotos syndrome is characterized by high birth length, rapid bone growth, distinctive facial features, and possible verbal and motor delays. It is more common in males than females. Developmental deficits, specific learning problems, and speech/language delays may also occur. (DB)

  8. Adults with Asperger Syndrome: A Childhood Disorder Grows Up

    ERIC Educational Resources Information Center

    Wilkinson, Lee A.

    2007-01-01

    Asperger syndrome is a chronic developmental disorder characterized by problems in social relatedness, empathic communication and understanding, and circumscribed interests. The inclusion of Asperger's Disorder (Asperger syndrome) in the "Diagnostic and Statistical Manual of Mental Disorders" (DSM-IV; American Psychiatric Association, 1994), has…

  9. Estimation and characterization of decontamination and decommissioning solid waste expected from the Plutonium Finishing Plant

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Millar, J.S.; Pottmeyer, J.A.; Stratton, T.J.

    1995-01-01

    Purpose of the study was to estimate the amounts of equipment and other materials that are candidates for removal and subsequent processing in a solid waste facility when the Hanford Plutonium Finishing Plant is decontaminated and decommissioned. (Building structure and soil are not covered.) Results indicate that {approximately}5,500 m{sup 3} of solid waste is expected to result from the decontamination and decommissioning of the Pu Finishing Plant. The breakdown of the volumes and percentages of waste by category is 1% dangerous solid waste, 71% low-level waste, 21% transuranic waste, 7% transuranic mixed waste.

  10. Cf-252 Characterization Documents

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Feldman, Alexander

    2014-03-14

    Six documents were written by Vance and Associates under contract to the Off-Site Source Recovery Project of Los Alamos National Laboratory. These Six documents provided the basis for characterization of Californium-252 sealed sources and for the packaging and manifesting of this material for disposal at the Waste Isolation Pilot Project. The Six documents are: 1. VA-OSR-10, Development of radionuclide distributions for Cf-252 sealed sources. 2. VA-OSR-11, Uncertainty analysis for Cf-252 sealed sources. 3. VA-OSR-12, To determine the radionuclides in the waste drums containing Cf-252 sealed source waste that are required to be reported under the requirements of the WIPP WACmore » and the TRAMPAC. 4. VA-OSR-13, Development of the spreadsheet for the radiological calculations for the characterization of Cf-252 sources. 5. VA-OSR-14, Relative importance of neutron-induced fission in Cf-252 sources. 6. VA-OSR-15, Determine upper bound of decay product inventories from a drum of Cf-252 sources. These six documents provide the technical basis for the characterization of Cf-252 sources and will be part of the AK documentation required for submittal to the Central Characterization Project (CCP) of WIPP.« less

  11. Long-term characterization of the diet-induced obese and diet-resistant rat model: a polygenetic rat model mimicking the human obesity syndrome.

    PubMed

    Madsen, Andreas Nygaard; Hansen, Gitte; Paulsen, Sarah Juel; Lykkegaard, Kirsten; Tang-Christensen, Mads; Hansen, Harald S; Levin, Barry E; Larsen, Philip Just; Knudsen, Lotte Bjerre; Fosgerau, Keld; Vrang, Niels

    2010-09-01

    The availability of useful animal models reflecting the human obesity syndrome is crucial in the search for novel compounds for the pharmacological treatment of obesity. In the current study, we have performed an extensive characterization of the obesity syndrome in a polygenetic animal model, namely the selectively bred diet-induced obese (DIO) and diet-resistant (DR) rat strains. We show that they constitute useful models of the human obesity syndrome. DIO and DR rats were fed either a high-energy (HE) or a standard chow (Chow) diet from weaning to 9 months of age. Metabolic characterization including blood biochemistry and glucose homeostasis was examined at 2, 3, 6, and 9 months of age. Furthermore, in 6-month-old HE-fed DIO rats, the anti-obesity effects of liraglutide and sibutramine were examined in a 28-day study. Only HE-fed DIO rats developed visceral obesity, hyperleptinemia, hyperinsulinemia, and dyslipidemia, and showed a worsening of glucose tolerance over time. In line with the hyperlipidemic profile, a severe hepatic fat infiltration was observed in DIO rats at 6 months of age. The effects of liraglutide and sibutramine were tested in 6-month-old DIO rats. Both compounds effectively reduced food intake and body weight in DIO rats. Liraglutide furthermore improved glucose tolerance when compared with sibutramine. Our data highlights the usefulness of a polygenetic animal model for screening of compounds affecting food intake, body weight, and glucose homeostasis. Furthermore, the results underscore the effectiveness of GLP-1 mimetics both as anti-diabetes and anti-obesity agents.

  12. Characterization and Analysis of Liquid Waste from Marcellus Shale Gas Development.

    PubMed

    Shih, Jhih-Shyang; Saiers, James E; Anisfeld, Shimon C; Chu, Ziyan; Muehlenbachs, Lucija A; Olmstead, Sheila M

    2015-08-18

    Hydraulic fracturing of shale for gas production in Pennsylvania generates large quantities of wastewater, the composition of which has been inadequately characterized. We compiled a unique data set from state-required wastewater generator reports filed in 2009-2011. The resulting data set, comprising 160 samples of flowback, produced water, and drilling wastes, analyzed for 84 different chemicals, is the most comprehensive available to date for Marcellus Shale wastewater. We analyzed the data set using the Kaplan-Meier method to deal with the high prevalence of nondetects for some analytes, and compared wastewater characteristics with permitted effluent limits and ambient monitoring limits and capacity. Major-ion concentrations suggested that most wastewater samples originated from dilution of brines, although some of our samples were more concentrated than any Marcellus brines previously reported. One problematic aspect of this wastewater was the very high concentrations of soluble constituents such as chloride, which are poorly removed by wastewater treatment plants; the vast majority of samples exceeded relevant water quality thresholds, generally by 2-3 orders of magnitude. We also examine the capacity of regional regulatory monitoring to assess and control these risks.

  13. Gorlin-Goltz syndrome: A rare case report.

    PubMed

    Pol, Chetan A; Ghige, Suvarna K; Kalaskar, Ritesh R; Gosavi, Suchitra R

    2013-10-01

    Gorlin-Goltz syndrome is an uncommon autosomal dominant inherited disorder characterized by numerous basal cell carcinomas, odontogenic keratocysts (OKCs) and musculoskeletal malformations. A rare case of this syndrome observed in a 13-year-old male patient is presented in which multiple OKCs were causing disfigurement of the lower jaw as well as displacement and malocclusion of the teeth. Early diagnosis and treatment of this syndrome is important to reduce the severity of complications including cutaneous and cerebral malignancy and oromaxillofacial deformation and destruction due to jaw cysts.

  14. Characterization and ethanol potential from giant cassava (Manihot esculenta) stem waste biomass

    NASA Astrophysics Data System (ADS)

    Septia, E.; Supriadi; Suwinarti, W.; Amirta, R.

    2018-04-01

    Manihot esculenta stem waste biomass is promising material for ethanol production since it is unutilized substance from cassava production. Nowadays, cassava is the most common food in Indonesian society. The aims of this study were to identify availability and characteristic of giant cassava (M. esculenta) stem waste biomass for ethanol feedstock. In term of that, four plots with the size of 5m x 5m were made to calculate the total stem biomass obtained after harvesting process. In this study, various concentrations of alkaline were used to degrade lignin from the substrate. The effects of alkaline pretreatment were investigated using TAPPI method and the ethanol yield was estimated using modified NREL protocol. The results showed that the potential dry stem waste biomass from harvesting of M. esculenta was approximately 10.5 ton/ha. Further, alkaline pretreatment of stem waste biomass with 2% of NaOH coupled with the enzymatic saccharification process using meicelase was showed the highest production of sugar to reach of 38.49 % of total reduction sugar and estimated potentially converted to 2,62 L/ha of ethanol. We suggested M. esculenta stem waste biomass could be used as sustainable feedstock for ethanol production in Indonesia.

  15. [A parotitis as primary infection of Lemierre's syndrome].

    PubMed

    Valleix, B; Floccard, B; Hautin, E; Faure, F; Allaouchiche, B

    2011-09-01

    Lemierre's syndrome is a classical presentation of human necrobacillosis. It is characterized by a primary infection in the face including a septic thrombophlebitis of the internal jugular vein and disseminated metastatic abcesses. Fusobacterium necrophorum is the main pathogen found in that syndrome. The diagnosis is based on clinical features, then on the microbiology with positive anaerobic blood cultures as key role and finally on the computed tomography. Most of the time a well-chosen antibiotic treatment against anaerobic pathogens and Gram negative bacilli is efficient but surgery can be useful. We report a case of a 73 years old man, which seems to be unique because it is the first case reported of a Lemierre's syndrome characterized by a parotitis infected by F. necrophorum. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

  16. Discovery of Antinuclear Antibodies in Pigs Infected with Porcine Circovirus Type 2

    USDA-ARS?s Scientific Manuscript database

    Introduction. Porcine circovirus type 2 (PCV2) causes post-weaning-multisystemic-wasting-syndrome (PMWS), a swine disease first observed in Canada in 1991 (1). It is characterized by general wasting, respiratory disease, jaundice and pallor in young pigs resulting in production losses and variable...

  17. Lynch syndrome and Lynch syndrome mimics: The growing complex landscape of hereditary colon cancer

    PubMed Central

    Carethers, John M; Stoffel, Elena M

    2015-01-01

    Hereditary non-polyposis colorectal cancer (HNPCC) was previously synonymous with Lynch syndrome; however, identification of the role of germline mutations in the DNA mismatch repair (MMR) genes has made it possible to differentiate Lynch syndrome from other conditions associated with familial colorectal cancer (CRC). Broadly, HNPCC may be dichotomized into conditions that demonstrate defective DNA MMR and microsatellite instability (MSI) vs those conditions that demonstrate intact DNA MMR. Conditions characterized by MMR deficient CRCs include Lynch syndrome (germline MMR mutation), Lynch-like syndrome (biallelic somatic MMR mutations), constitutional MMR deficiency syndrome (biallelic germline MMR mutations), and sporadic MSI CRC (somatic biallelic methylation of MLH1). HNPCC conditions with intact DNA MMR associated with familial CRC include polymerase proofreading associated polyposis and familial colorectal cancer type X. Although next generation sequencing technologies have elucidated the genetic cause for some HNPCC conditions, others remain genetically undefined. Differentiating between Lynch syndrome and the other HNPCC disorders has profound implications for cancer risk assessment and surveillance of affected patients and their at-risk relatives. Clinical suspicion coupled with molecular tumor analysis and testing for germline mutations can help differentiate the clinical mimicry within HNPCC and facilitate diagnosis and management. PMID:26309352

  18. Lynch syndrome and Lynch syndrome mimics: The growing complex landscape of hereditary colon cancer.

    PubMed

    Carethers, John M; Stoffel, Elena M

    2015-08-21

    Hereditary non-polyposis colorectal cancer (HNPCC) was previously synonymous with Lynch syndrome; however, identification of the role of germline mutations in the DNA mismatch repair (MMR) genes has made it possible to differentiate Lynch syndrome from other conditions associated with familial colorectal cancer (CRC). Broadly, HNPCC may be dichotomized into conditions that demonstrate defective DNA MMR and microsatellite instability (MSI) vs those conditions that demonstrate intact DNA MMR. Conditions characterized by MMR deficient CRCs include Lynch syndrome (germline MMR mutation), Lynch-like syndrome (biallelic somatic MMR mutations), constitutional MMR deficiency syndrome (biallelic germline MMR mutations), and sporadic MSI CRC (somatic biallelic methylation of MLH1). HNPCC conditions with intact DNA MMR associated with familial CRC include polymerase proofreading associated polyposis and familial colorectal cancer type X. Although next generation sequencing technologies have elucidated the genetic cause for some HNPCC conditions, others remain genetically undefined. Differentiating between Lynch syndrome and the other HNPCC disorders has profound implications for cancer risk assessment and surveillance of affected patients and their at-risk relatives. Clinical suspicion coupled with molecular tumor analysis and testing for germline mutations can help differentiate the clinical mimicry within HNPCC and facilitate diagnosis and management.

  19. Prune Belly Syndrome

    PubMed Central

    Tagore, Koyye Ravindranath; Ramineni, Asok Kumar S.; Vijaya Lakshmi, A. R.; N., Bhavani

    2011-01-01

    Prune belly syndrome is a rare congenital disorder of the urinary system, characterized by a triad of abnormalities. The aetiology is not known. Many infants are either stillborn or die within the first few weeks of life from severe lung or kidney problems, or a combination of congenital anomalies. PMID:22606508

  20. U.S. Geological Survey research in radioactive waste disposal - Fiscal years 1983, 1984, and 1985

    USGS Publications Warehouse

    Dinwiddie, G.A.; Trask, N.J.

    1986-01-01

    The report summarizes progress on geologic and hydrologic research related to the disposal of radioactive wastes. The research is described according to whether it is related most directly to: (1) high-level and transuranic wastes, (2) low-level wastes, or (3) uranium mill tailings. Included is research applicable to the identification and geohydrologic characterization of waste-disposal sites, to investigations of specific sites where wastes have been stored, and to studies of regions or environments where waste-disposal sites might be located. A significant part of the activity is concerned with techniques and methods for characterizing disposal sites and studies of geologic and hydrologic processes related to the transport and (or) retention of waste radionuclides.