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Sample records for severe hypospermatogenesis rare

  1. Hypospermatogenesis and spermatozoa maturation arrest in rats induced by mobile phone radiation.

    Science.gov (United States)

    Meo, Sultan Ayoub; Arif, Muhammad; Rashied, Shahzad; Khan, Muhammad Mujahid; Vohra, Muhammad Saeed; Usmani, Adnan Mahmood; Imran, Muhammad Babar; Al-Drees, Abdul Majeed

    2011-05-01

    To determine the morphological changes induced by mobile phone radiation in the testis of Wistar albino rats. Cohort study. Department of Physiology, College of Medicine, King Saud University, Riyadh, Saudi Arabia, from April 2007 to June 2008. Forty male Wistar albino rats were divided in three groups. First group of eight served as the control. The second group [group B, n=16] was exposed to mobile phone radiation for 30 minutes/day and the third group [group C, n=16] was exposed to mobile phone radiation for 60 minutes/day for a total period of 3 months. Morphological changes in the testes induced by mobile phone radiations were observed under a light microscope. Exposure to mobile phone radiation for 60 minutes/day caused 18.75% hypospermatogenesis and 18.75% maturation arrest in the testis of albino rats compared to matched controls. However, no abnormal findings were observed in albino rats that were exposed to mobile phone radiation for 30 minutes/day for a total period of 3 months. Long-term exposure to mobile phone radiation can cause hypospermatogenesis and maturation arrest in the spermatozoa in the testis of Wistar albino rats.

  2. [Rare cause for severe hypertriglyceridemia - case 9/2013].

    Science.gov (United States)

    Kahl, Sabine; Roden, Michael; Mörike, Klaus; Müssig, Karsten

    2013-11-01

    We report on a 48-year-old female patient with recently developed severe hypertriglyceridemia. Medical history was remarkable for breast cancer with breast-preserving surgery and chemoradiotherapy. The patient has been treated with 20 mg tamoxifen per day for three months. Laboratory results showed hypertriglyeridemia, hypercholesterolemia and lowered HDL-cholesterol. Findings were consistent with a drug-induced hypertriglyceridemia caused by anti-estrogenic therapy with tamoxifen. After consulting the patient's gynaecologist, we discontinued tamoxifen treatment. Thereupon, triglyceride levels fell consistently. There were no signs of pancreatitis, serum amylase and lipase were in the normal range. Patients with pre-diagnosed metabolic disorders, especially dyslipidemia and type 2 diabetes, should undergo regular controls of serum triglycerides during tamoxifen treatment. Also, one should keep in mind that a subacute, severe rise in serum triglyceride levels may be caused, in rare cases, by tamoxifen treatment. © Georg Thieme Verlag KG Stuttgart · New York.

  3. A rare cause of severe hepatomegaly with an improving outcome

    DEFF Research Database (Denmark)

    Godskesen, Line Elberg; Abildgaard, Niels; Kjeldsen, Jens

    2014-01-01

    A previously healthy 43-year-old man presented with dyspnoea, 15 kg weight loss, severe hepatomegaly and alkaline phosphatase at 5400 U/L. Examinations seemed to suggest cirrhosis, but blood samples did not show any signs of underlying liver disease. Liver biopsy revealed amyloid light chain (AL...

  4. Study of severe and rare complications of transarterial chemoembolization (TACE) for liver cancer

    International Nuclear Information System (INIS)

    Xia Jinglin; Ren Zhenggang; Ye Shenglong; Sharma Dilip; Lin Zhiying; Gan Yuhong; Chen Yi; Ge Ninglin; Ma Zengchen; Wu Zhiquan; Fan Jia; Qin Lunxiu; Zhou Xinda; Tang Zhaoyou; Yang Binghui

    2006-01-01

    Objective: To study severe and rare complications of transarterial chemoembolization (TACE) for liver cancer. Methods: Clinical records of severe and rare complications following TACE in 1348 cases of liver cancer from January 1997 to February 2004 were studied retrospectively. Results: A total of 2012 TACE procedures were performed for 1348 patients. There were 3 cases of spontaneous rupture of liver cancer, 1 case of perforation of duodenum, 3 cases of liver abscess (1 of them was associated with sepsis), 1 case of pulmonary embolism, 1 case of spasm of the hepatic artery, 40 cases of hepatic artery occlusion, 3 cases of femoral nerve injury, 1 case of bilioma and 1 case of acute renal failure. Conclusion: Although the severe complications of TACE are rare, the procedure should be done cautiously including super selection of hepatic artery, slow infusion of lipiodol, careful postoperative observations and early detection and management of complications

  5. Nicardipine-induced acute pulmonary edema: a rare but severe complication of tocolysis.

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    Serena, Claire; Begot, Emmanuelle; Cros, Jérôme; Hodler, Charles; Fedou, Anne Laure; Nathan-Denizot, Nathalie; Clavel, Marc

    2014-01-01

    We report four cases of acute pulmonary edema that occurred during treatment by intravenous tocolysis using nicardipine in pregnancy patients with no previous heart problems. Clinical severity justified hospitalization in intensive care unit (ICU) each time. Acute dyspnea has begun at an average of 63 hours after initiation of treatment. For all patients, the first diagnosis suspected was pulmonary embolism. The patients' condition improved rapidly with appropriate diuretic treatment and by modifying the tocolysis. The use of intravenous nicardipine is widely used for tocolysis in France even if its prescription does not have a marketing authorization. The pathophysiological mechanisms of this complication remain unclear. The main reported risk factors are spontaneous preterm labor, multiple pregnancy, concomitant obstetrical disease, association with beta-agonists, and fetal lung maturation corticotherapy. A better knowledge of this rare but serious adverse event should improve the management of patients. Nifedipine or atosiban, the efficiency of which tocolysis was also studied, could be an alternative.

  6. Legionella pneumonia associated with severe acute respiratory distress syndrome and diffuse alveolar hemorrhage - A rare association

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    Muhammad Kashif

    2017-01-01

    Full Text Available Legionella pneumophila is a common, usually underreported and undiagnosed cause of community acquired pneumonia which can lead to significant morbidity and mortality. Diffuse alveolar hemorrhage rarely have been associated with legionella infection. We present a 61-year-old man with hypertension, diabetes mellitus and obesity admitted with severe acute respiratory distress syndrome. He was found to have Legionella pneumonia with associated diffuse alveolar hemorrhage diagnosed with bronchoscopic sequential bronchoalveolar lavage. He was successfully managed with antibiotics, lung protective strategies and intravenous pulse dose steroids. This patient highlights the unusual association of Legionella infection and diffuse alveolar hemorrhage. Additionally, the case re-enforces the need for early and aggressive evaluation and management of patients presenting with pneumonia and progressive hypoxia despite adequate treatment.

  7. Study on charge transfer reaction of several organic molecules with accelerated rare gas ions

    International Nuclear Information System (INIS)

    Takahasi, Makoto; Okuda, Sachiko; Arai, Eiichi; Ichinose, Akira; Takakubo, Masaaki.

    1984-01-01

    Observing the charge transfer mass spectra of ethylbenzene, cyclobutane and methanol in Ar and Xe ion impacts, we investigated the dependence of the secondary ion peak intensities (normalized to primary ion current and target pressure) on the translational energy of primary ions (0-3500 eV).In the case of ethylbenzene, several maxima of the secondary i on peak intensities were observed in Ar and Xe ion impacts. The correlation between the maxima and the primary ion energy was examined in terms of near adiabatic theory of Massey. Supplementary studies on the energy distribution of primary ion, charge transfer cross section between methanol and Xe ion, and final product analysis in rare gas ion irradiation on cyclobutane were described. (author)

  8. Nicardipine-Induced Acute Pulmonary Edema: A Rare but Severe Complication of Tocolysis

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    Claire Serena

    2014-01-01

    Full Text Available We report four cases of acute pulmonary edema that occurred during treatment by intravenous tocolysis using nicardipine in pregnancy patients with no previous heart problems. Clinical severity justified hospitalization in intensive care unit (ICU each time. Acute dyspnea has begun at an average of 63 hours after initiation of treatment. For all patients, the first diagnosis suspected was pulmonary embolism. The patients' condition improved rapidly with appropriate diuretic treatment and by modifying the tocolysis. The use of intravenous nicardipine is widely used for tocolysis in France even if its prescription does not have a marketing authorization. The pathophysiological mechanisms of this complication remain unclear. The main reported risk factors are spontaneous preterm labor, multiple pregnancy, concomitant obstetrical disease, association with beta-agonists, and fetal lung maturation corticotherapy. A better knowledge of this rare but serious adverse event should improve the management of patients. Nifedipine or atosiban, the efficiency of which tocolysis was also studied, could be an alternative.

  9. Cyclophosphamide-induced symptomatic hyponatremia, a rare but severe side effect: a case report

    Directory of Open Access Journals (Sweden)

    Elazzazy S

    2014-09-01

    Full Text Available Shereen Elazzazy,1 Asmaa Elhassan Mohamed,2 Amaal Gulied1 1Pharmacy Department, 2Oncology Hematology Department, National Center for Cancer Care and Research (NCCCR, Hamad Medical Corporation, Doha, QatarAbstract: Cyclophosphamide is commonly used in the treatment of malignant diseases. Symptomatic severe hyponatremia induced by low-dose cyclophosphamide is very uncommon worldwide. We report a case of severe symptomatic hyponatremia that developed in a female breast cancer patient following the first cycle of chemotherapy containing low-dose cyclophosphamide. Her laboratory test showed serum Na of 112 mmol/L. Her hyponatremia was initially treated with sodium bicarbonate. She completely recovered without neurological deficits after slow correction of the serum Na concentration. Although hyponatremia is a rare toxicity it should always be considered during the usage of cyclophosphamide, even if the dosage is low, especially with concurrent use of other medications that impair water excretion, like chlorthalidone. This report describes the first reported case of cyclophosphamide-induced hyponatremia in Qatar.Keywords: AC protocol, adjuvant chemotherapy, breast cancer, cyclophosphamide, hyponatremia, thiazides

  10. Severe Rhabdomyolysis without Systemic Involvement: A Rare Case of Idiopathic Eosinophilic Polymyositis

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    Ayesha Farooq

    2015-01-01

    Full Text Available Introduction. Eosinophilic polymyositis (EPM is a rare cause of rhabdomyolysis characterized by eosinophilic infiltrates in the muscle. We describe the case of a young patient with eosinophilic polymyositis causing isolated severe rhabdomyolysis without systemic involvement. Case Presentation. A 22-year-old Haitian female with no past medical history presented with progressive generalized muscle aches without precipitating factors. Examination of the extremities revealed diffuse muscle tenderness. Laboratory findings demonstrated peripheral eosinophilia and high creatinine phosphokinase (CPK and transaminase levels. Workup for the common causes of rhabdomyolysis were negative. Her CPK continued to rise to greater than 100,000 units/L so a muscle biopsy was performed which showed widespread eosinophilic infiltrate consistent with eosinophilic polymyositis. She was started on high dose systemic corticosteroids with improvement of her symptoms, eosinophilia, and CPK level. Discussion. This case illustrates a systematic workup of rhabdomyolysis in the presence of peripheral eosinophilia. Many differential diagnoses must be considered before establishing a diagnosis of idiopathic eosinophilic polymyositis. To our knowledge, our case of eosinophilic polymyositis is unique as it presented with severe rhabdomyolysis without another organ involvement. Clinicians should maintain a high index of suspicion for this physically debilitating disease to aid in prompt diagnosis.

  11. Severe angioedema in myxedema coma: a difficult airway in a rare endocrine emergency.

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    Lee, Christopher H; Wira, Charles R

    2009-10-01

    Myxedema coma is the most lethal manifestation of hypothyroidism. It is a true medical emergency and can result in profound hemodynamic instability and airway compromise. Myxedema coma currently remains a diagnostic challenge due to the rarity of cases seen today, and failure to promptly initiate therapy with replacement thyroid hormone can be fatal. As thyroid hormone therapy can take days or weeks to reverse the manifestations of myxedema coma, interim supportive therapy is critical while awaiting clinical improvement. Some patients will require endotracheal intubation in the emergency department (ED), and physicians should be aware that unanticipated posterior pharyngeal edema in myxedema coma could severely complicate airway management. Although mechanical ventilation is a well-described adjunctive therapy for myxedema coma, reports of the potential difficulty in securing a definitive airway in these patients are rare. We describe a case of an unidentified woman who presented to the ED with myxedema coma requiring urgent endotracheal intubation and was found to have extensive posterior pharyngeal angioedema inconsistent with her relatively benign external examination. This case highlights the typical features of myxedema coma and discusses our necessity for a rescue device in definitive endotracheal tube placement. Emergency physicians should anticipate a potentially difficult airway in all myxedema coma patients regardless of the degree of external facial edema present.

  12. Neutropenia in Patients with Common Variable Immunodeficiency: a Rare Event Associated with Severe Outcome.

    Science.gov (United States)

    Guffroy, Aurélien; Mourot-Cottet, Rachel; Gérard, Laurence; Gies, Vincent; Lagresle, Chantal; Pouliet, Aurore; Nitschké, Patrick; Hanein, Sylvain; Bienvenu, Boris; Chanet, Valérie; Donadieu, Jean; Gardembas, Martine; Karmochkine, Marina; Nove-Josserand, Raphaele; Martin, Thierry; Poindron, Vincent; Soulas-Sprauel, Pauline; Rieux-Laucat, Fréderic; Fieschi, Claire; Oksenhendler, Eric; André-Schmutz, Isabelle; Korganow, Anne-Sophie

    2017-10-01

    Common variable immunodeficiency (CVID) is characterized by infections and hypogammaglobulinemia. Neutropenia is rare during CVID. The French DEFI study enrolled patients with primary hypogammaglobulinemia. Patients with CVID and neutropenia were retrospectively analyzed. Among 473 patients with CVID, 16 patients displayed neutropenia (lowest count [0-1400]*10 6 /L). Sex ratio (M/F) was 10/6. Five patients died during the follow-up (11 years) with an increased percentage of deaths compared to the whole DEFI group (31.3 vs 3.4%, P < 0.05). Neutropenia was diagnosed for 10 patients before 22 years old. The most frequent symptoms, except infections, were autoimmune cytopenia, i.e., thrombopenia or anemia (11/16). Ten patients were affected with lymphoproliferative diseases. Two patients were in the infection only group and the others belonged to one or several other CVID groups. The median level of IgG was 2.6 g/L [0.35-4.4]. Most patients presented increased numbers of CD21 low CD38 low B cell, as already described in CVID autoimmune cytopenia group. Neutropenia was considered autoimmune in 11 cases. NGS for 52 genes of interest was performed on 8 patients. No deleterious mutations were found in LRBA, CTLA4, and PIK3. More than one potentially damaging variant in other genes associated with CVID were present in most patients arguing for a multigene process. Neutropenia is generally associated with another cytopenia and presumably of autoimmune origin during CVID. In the DEFI study, neutropenia is coupled with more severe clinical outcomes. It appears as an "alarm bell" considering patients' presentation and the high rate of deaths. Whole exome sequencing diagnosis should improve management.

  13. Microstructure, Properties and Atomic Level Strain in Severely Deformed Rare Metal Niobium

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    Lembit KOMMEL

    2012-12-01

    Full Text Available The mechanical and physical properties relationship from atomic level strain/stress causes dislocation density and electrical conductivity relationship, as well as crystallites deformation and hkl-parameter change in the severely deformed pure refractory rare metal Nb at ambient temperature and during short processing times. The above mentioned issues are discussed in this study. For ultrafine-grained and nanocrystalline microstructure forming in metal the equal-channel angular pressing and hard cyclic viscoplastic deformation were used. The flat deformation and heat treatment at different parameters were conducted as follows. The focused ion beam method was used for micrometric measures samples manufacturied under nanocrystalline microstructure study by transmission electron microscope. The microstructure features of metal were studied under different orientations by X-ray diffraction scattering method, and according to the atomic level strains, dislocation density, hkl-parameters and crystallite sizes were calculated by different computation methods. According to results the evolutions of atomic level strains/stresses, induced by processing features have great influence on the microstructure and advanced properties forming in pure Nb. Due to cumulative strain increase the tensile stress and hardness were increased significantly. In this case the dislocation density of Nb varies from 5.0E+10 cm–2 to 2.0E+11 cm–2. The samples from Nb at maximal atomic level strain in the (110 and (211 directions have the maximal values of hkl-parameters, highest tensile strength and hardness but minimal electrical conductivity. The crystallite size was minimal and relative atomic level strain maximal in (211 orientation of crystal. Next, flat deformation and heat treatment increase the atomic level parameters of severely deformed metal.DOI: http://dx.doi.org/10.5755/j01.ms.18.4.3091

  14. Spontaneous fungal peritonitis: a rare but severe complication of liver cirrhosis.

    Science.gov (United States)

    Gravito-Soares, Marta; Gravito-Soares, Elisa; Lopes, Sandra; Ribeiro, Graça; Figueiredo, Pedro

    2017-09-01

    Spontaneous bacterial peritonitis is the most common infectious complication in cirrhosis. Spontaneous fungal peritonitis is rare and remains unknown. In this work, spontaneous fungal peritonitis as well as risk factors and prognosis are characterized. A retrospective case-control study of 253 consecutive admissions by peritonitis in cirrhotic patients was carried out between 2006 and 2015. Comparison of patients with spontaneous fungal peritonitis (cases) and spontaneous bacterial peritonitis with positive microbiologic ascitic fluid culture (controls) was performed. Variables such as sociodemographic and clinical features, cirrhosis etiology, liver dysfunction scores, ascitic and laboratory parameters, invasive procedures, and prognosis were evaluated. Of the 231 patients, eight (3.5%) developed spontaneous fungal peritonitis, 62.5% of cases being coinfected with bacteria. Candida spp. was isolated in 87.5% of cases, mainly Candida albicans (37.5%) and C. krusei (25.0%). Patients with spontaneous fungal peritonitis had higher ascitic fluid lactate dehydrogenase (288.4±266.6 vs. 161.0±179.5; P=0.011), blood leukocyte count (15187.5±5432.3 vs. 10969.8±6949.5; P=0.028), blood urea nitrogen (69.8±3.1 vs. 36.3±25.5; P=0.001), higher number of invasive procedures (colonoscopy: 25.0 vs. 0.8%, P=0.001; urinary catheterization: 87.5 vs. 49.6%, P=0.038; nasogastric intubation: 87.5 vs. 26.9%, P=0.001), and longer duration of hospital stay (30.0±32.9 vs. 18.9±17.0 days; P=0.031). No statistical difference was found between the two groups for Model for End-Stage Liver Disease, Model for End-Stage Liver Disease-sodium, and Child-Pugh scores. Spontaneous fungal peritonitis was associated with a worse prognosis, particularly severe sepsis/septic shock (87.5 vs. 42.8%, P=0.023), admission in the gastroenterology intensive care unit (87.5 vs. 24.4%; P=0.001), and overall (62.5 vs. 31.9%; P=0.039) or 30-day mortality (50.0 vs. 24.4%; P=0.034), with a mean diagnosis

  15. A Rare Case Report of a Collodion Baby with Severe Hypernatremia

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    Ahmad Farhat

    2017-06-01

    Full Text Available Background: Collodion baby is a rare condition (i.e., 1:300000 birth, which is referred to a neonate covered with a tight and shiny membrane desquamating within two weeks. This condition takes place as a result of the epidermal cornification disorder. Given the impairment of the skin barrier function, these neonates are at the risk of several complications, including hypernatremic dehydration, hypothermia, skin infections, fissures, conjunctivitis, sepsis, dehydration, and constrictive bands of the extremities resulting in vascular compromise and edema. This condition has a high mortality rate; accordingly, the majority of the collodion babies die within the first few weeks of birth due to the secondary complications depending on the type of mutations. Although the collodion membrane is a transient condition, it can lead to a number of complications in 45% of the newborns and result in 11% mortality rate in the first few weeks of the neonatal life. However, in the recent years, the mortality rates have been declined owing to the introduction of systemic retinoids to the clinical practice and the advanced care methods, which are used in the intensive care units. Harlequin ichthyosis has been associated with the mutation in the ABCA12 gene; therefore, genetic counseling and mutation screening of this gene should be considered.Case report: Herein, we reported a case of a collodion baby with favorable evolution, who developed hypernatremia and suffered from some complications having aroused from the condition.Conclusion: Infection prevention and supportive care of collodion babies mainly with incubator, serum therapy, and feeding support can be effective in preventing complications.

  16. Frequency of rare mutations and common genetic variations in severe hypertriglyceridemia in the general population of Spain.

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    Lamiquiz-Moneo, Itziar; Blanco-Torrecilla, Cristian; Bea, Ana M; Mateo-Gallego, Rocío; Pérez-Calahorra, Sofía; Baila-Rueda, Lucía; Cenarro, Ana; Civeira, Fernando; de Castro-Orós, Isabel

    2016-04-23

    Hypertriglyceridemia (HTG) is a common complex metabolic trait that results of the accumulation of relatively common genetic variants in combination with other modifier genes and environmental factors resulting in increased plasma triglyceride (TG) levels. The majority of severe primary hypertriglyceridemias is diagnosed in adulthood and their molecular bases have not been fully defined yet. The prevalence of HTG is highly variable among populations, possibly caused by differences in environmental factors and genetic background. However, the prevalence of very high TG and the frequency of rare mutations causing HTG in a whole non-selected population have not been previously studied. The total of 23,310 subjects over 18 years from a primary care-district in a middle-class area of Zaragoza (Spain) with TG >500 mg/dL were selected to establish HTG prevalence. Those affected of primary HTG were considered for further genetic analysis. The promoters, coding regions and exon-intron boundaries of LPL, LMF1, APOC2, APOA5, APOE and GPIHBP1 genes were sequenced. The frequency of rare variants identified was studied in 90 controls. One hundred ninety-four subjects (1.04%) had HTG and 90 subjects (46.4%) met the inclusion criteria for primary HTG. In this subgroup, nine patients (12.3%) were carriers of 7 rare variants in LPL, LMF1, APOA5, GPIHBP1 or APOE genes. Three of these mutations are described for the first time in this work. The presence of a rare pathogenic mutation did not confer a differential phenotype or a higher family history of HTG. The prevalence of rare mutations in candidate genes in subjects with primary HTG is low. The low frequency of rare mutations, the absence of a more severe phenotype or the dominant transmission of the HTG would not suggest the use of genetic analysis in the clinical practice in this population.

  17. Mitral Perivalvular Leak after Blunt Chest Trauma: A Rare Cause of Severe Subacute Mitral Regurgitation.

    Science.gov (United States)

    Marchese, Nicola; Facciorusso, Antonio; Vigna, Carlo

    2015-12-01

    Blunt chest trauma is a very rare cause of valve disorder. Moreover, mitral valve involvement is less frequent than is aortic or tricuspid valve involvement, and the clinical course is usually acute. In the present report, we describe the case of a 49-year-old man with a perivalvular mitral injury that became clinically manifest one year after a violent, nonpenetrating chest injury. This case is atypical in regard to the valve involved (isolated mitral damage), the injury type (perivalvular leak in the absence of subvalvular abnormalities), and the clinical course (interval of one year between trauma and symptoms).

  18. Two novel rare variants of APOA5 gene found in subjects with severe hypertriglyceridemia.

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    Pisciotta, Livia; Fresa, Raffaele; Bellocchio, Antonella; Guido, Virgilia; Priore Oliva, Claudio; Calandra, Sebastiano; Bertolini, Stefano

    2011-11-20

    Common variants of APOA5 gene affect plasma triglyceride (TG) in the population and a number of rare variants APOA5 have been reported in individuals with hypertriglyceridemia (HTG). APOA5 was analysed in 98 HTG individuals (plasma TG >9 mmol/L) in whom no mutations in LPL and APOC2 had been found. Two patients were found to be heterozygous for two novel APOA5 variants. The first variant (p.L253P) was identified in an obese male who consumed a diet rich in fat and simple sugars. He was also a carrier in trans of the common TG-raising p.S19W SNP (5*3 haplotype). The second variant (c.295-297 del GAG, p.E99 del) was found in a lean male with no life style or metabolic factors known to affect plasma TG. He was a carrier in trans of the TG-raising 5*2 haplotype and was homozygous for the rare c.1337T allele of a SNP of GCKR gene. No mutations in other genes affecting plasma TG (LMF1 and GPIHBP1) were found in these patients. These APOA5 variants, resulted to be deleterious in silico, were not found in 350 control subjects. These novel APOA5 variants predispose to HTG in combination with other genetic or nutritional factors. Copyright © 2011 Elsevier B.V. All rights reserved.

  19. [Myxedema coma as a rare differential diagnosis of severe consciousness disturbance].

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    Kollmar, R; Schellinger, P D; Bardutzky, J; Meisel, F; Schwaninger, M

    2002-12-01

    Myxedema coma is a rare and life-threatening complication of untreated hypothyroidism. Therefore, it must be part of the differential diagnosis in comatose patients. We report one patient who presented with CO(2) narcosis,hypothermia, bradycardia,hyporeflexia, tetraparesis, ascitis, pleural effusions, and heart insufficiency. Examination of the CSF, cranial CT, MRI, and MR angiography were normal. In suspicion of myxedema coma,the patient was treated with high dose L-thyroxine and hydrocortisone for preventing secondary adrenal insufficiency. A fast clinical recovery, decreased T4 (7.2 ng/l) and T3 (0.93 ng/l), and increased TSH (20.19 mU/l) together with the following anamnesis of radio iodine therapy and insufficient thyroxine intake confirmed the diagnosis. In conclusion, treatment of the myxedema coma must be started as soon as the laboratory results are confirmatory, since its course depends on the time of initiation of treatment.

  20. Severe neutropenia revealing a rare presentation of dengue fever: a case report.

    Science.gov (United States)

    Shourick, J; Dinh, A; Matt, M; Salomon, J; Davido, B

    2017-08-17

    Arboviruses are a common cause of fever in the returned traveler often associated with leucopenia, especially lymphopenia and thrombocytopenia. Transient neutropenia has been described in a few cases of arboviruses. However, prolonged and severe neutropenia (dengue fever, especially in the returned traveler in Europe. A 26-year-old healthy female without any medical past history, flying back from Thailand, presented a transient fever with severe neutropenia (dengue fever. Outcome was favorable without any antimicrobial therapy. Physicians should be wary of possible unusual presentation of dengue fever with prolonged neutropenia. Although such biological sign is more often associated with malaria or severe bacterial infection, it may be a sign of arbovirus.

  1. [Bartter syndrome, severe rare orphan kidney disease: a step towards therapy through pharmacogenetic and epidemiological studies].

    Science.gov (United States)

    Conte, Elena; Imbrici, Paola; Sahbani, Dalila; Liantonio, Antonella; Conte, Diana

    2018-05-01

    Bartter syndromes (BS) types 1-5 are rare salt-losing tubulopathies presenting with overlapping clinical phenotypes including marked salt wasting and hypokalemia leading to polyuria, polydipsia, volume contraction, muscle weakness and growth retardation. These diseases are due to an impairment of sodium, potassium, chloride reabsorption caused by mutations in genes encoding for ion channel or transporters expressed in specific nephron tubule segments. Particularly, BS type 3 is a clinically heterogeneous form caused by mutations in CLCNKB gene which encodes the ClC-Kb chloride channel involved in NaCl reabsorption in the renal tubule. Specific therapy for BS is lacking and the only pharmacotherapy up today available is purely symptomatic and characterized by limiting side effects. The improvement of our understanding of the phenotype/genotype correlation and of the precise pathogenic mechanisms associated with BS type 3 as well as the pharmacological characterization of ClC-K chloride channels are fundamental to design therapies tailored upon patients' mutation. This mini review focused on recent studies representing relevant forward steps in the field as well as noteworthy examples of how basic and clinical research can cooperate to gain insight into the pathophysiology of this renal channelopathy, paving the way for a personalized therapy. Copyright by Società Italiana di Nefrologia SIN, Rome, Italy.

  2. Anti-E Alloimmunization: A Rare Cause of Severe Fetal Hemolytic Disease Resulting in Pregnancy Loss

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    An-Shine Chao

    2009-01-01

    Full Text Available We report a case of severe intrauterine hemolysis caused by sole anti-E alloimmunization. A 36-year-old multipara woman presented with hydrops fetalis at 27 weeks of gestation. She had a history of previous neonatal death. In this pregnancy, she was found to have very high titer of anti-E antibody. Ultrasonography detected marked skin edema, cardiomegaly, hepatosplenomegaly, pleural effusion, ascites, placentomegaly, and polyhydramnios. The Doppler peak systolic velocity in the middle cerebral artery was 0.8 m/s, indicating severe fetal anemia. Multiple intrauterine transfusions for the anemic fetus were administered. However, persistent severe fetal anemia and placentomegaly caused poor neonatal death and mirror syndrome in the mother. Uncommon red blood cell alloimmunization has to be watched for early in any population, especially in a woman with a history of unexplained perinatal loss.

  3. Rare Variant Analysis of Human and Rodent Obesity Genes in Individuals with Severe Childhood Obesity

    NARCIS (Netherlands)

    Hendricks, Audrey E.; Bochukova, Elena G.; Marenne, Gaëlle; Keogh, Julia M.; Atanassova, Neli; Bounds, Rebecca; Wheeler, Eleanor; Mistry, Vanisha; Henning, Elana; Körner, Antje; Muddyman, Dawn; McCarthy, Shane; Hinney, Anke; Hebebrand, Johannes; Scott, Robert A.; Langenberg, Claudia; Wareham, Nick J.; Surendran, Praveen; Howson, Joanna M M; Butterworth, Adam S.; Danesh, John; Nordestgaard, Børge G.; Nielsen, Sune F.; Afzal, Shoaib; Papadia, Sofia; Ashford, Sofie; Garg, Sumedha; Millhauser, Glenn L.; Palomino, Rafael I.; Kwasniewska, Alexandra; Tachmazidou, Ioanna; O'Rahilly, Stephen; Zeggini, Eleftheria; Barroso, Inês; Farooqi, I. Sadaf; Benzeval, Michaela; Burton, Jonathan; Buck, Nicholas; Jäckle, Annette; Kumari, Meena; Laurie, Heather; Lynn, Peter; Pudney, Stephen; Rabe, Birgitta; Wolke, Dieter; Overvad, Kim; Tjønneland, Anne; Clavel-Chapelon, Francoise; Kaaks, Rudolf; Boeing, Heiner; Trichopoulou, Antonia; Ferrari, Pietro; Palli, Domenico; Krogha, Vittorio; Panico, Salvatore; Tuminoa, Rosario; Matullo, Giuseppe; Boer, Jolanda Ma; Van Der Schouw, Yvonne; Weiderpass, Elisabete; Quiros, J. Ramon; Sánchez, María José; Navarro, Carmen; Moreno-Iribas, Conchi; Arriola, Larraitz; Melander, Olle; Wennberg, Patrik; Key, Timothy J.; Riboli, Elio; Al-Turki, Saeed; Anderson, Carl A; Anney, Richard; Antony, Dinu; Soler Artigas, María; Ayub, Muhammad; Bala, Senduran; Barrett, Jeffrey C; Beales, Phil; Bentham, Jamie; Bhattacharyaa, Shoumo; Birney, Ewan; Blackwooda, Douglas; Bobrow, Martin; Bolton, Patrick F.; Boustred, Chris; Breen, Gerome; Calissanoa, Mattia; Carss, Keren; Charlton, Ruth; Chatterjee, Krishna; Chen, Lu; Ciampia, Antonio; Cirak, Sebahattin; Clapham, Peter; Clement, Gail; Coates, Guy; Coccaa, Massimiliano; Collier, David A; Cosgrove, Catherine; Coxa, Tony; Craddock, Nick; Crooks, Lucy; Curran, Sarah; Curtis, David; Daly, Allan; Danecek, Petr; Day, Ian N M; Day-Williams, Aaron G; Dominiczak, Anna; Down, Thomas; Du, Yuanping; Dunham, Ian; Durbin, Richard; Edkins, Sarah; Ekong, Rosemary; Ellis, Peter; Evansa, David M.; FitzPatrick, David R.; Flicek, Paul; Floyd, James S.; Foley, A. Reghan; Franklin, Christopher S.; Futema, Marta; Gallagher, Louise; Gaunt, Tom R.; Geihs, Matthias; Geschwind, Daniel H.; Greenwood, Celia M.T.; Griffin, Heather; Grozeva, Detelina; Guo, Xiaosen; Guo, Xueqin; Gurling, Hugh; Hart, Deborah J.; Holmans, Peter A; Howie, Bryan; Huang, Jie; Huang, Liren; Hubbard, Tim; Humphries, Steve E.; Hurles, Matthew E.; Hysi, Pirro G.; Iotchkova, Valentina; Jackson, David K.; Jamshidi, Yalda; Joyce, Chris; Karczewski, Konrad J.; Kaye, Jane; Keane, Thomas; Kemp, John P.; Kennedy, Karen; Kent, Alastair; Khawaja, Farrah; Van Kogelenberg, Margriet; Kolb-Kokocinski, Anja; Lachance, Genevieve; Langford, Cordelia; Lawson, Daniel; Lee, Irene; Lek, Monkol; Li, Rui; Li, Yingrui; Liang, Jieqin; Lin, Hong; Liu, Ryan; Lönnqvist, Jouko; Lopes, Luis R.; Lopes, Margarida; MacArthur, Daniel G.; Mangino, Massimo; Marchini, Jonathan; Maslen, John; Mathieson, Iain; McGuffin, Peter; McIntosh, Andrew M.; McKechanie, Andrew G.; McQuillin, Andrew; Memari, Yasin; Metrustry, Sarah; Migone, Nicola; Min, Josine L.; Mitchison, Hannah M; Moayyeri, Alireza; Morris, Andrew D.; Morris, James; Muntoni, Francesco; Northstone, Kate; O'Donovan, Michael C.; Onoufriadis, Alexandros; Oualkacha, Karim; Owen, Michael J; Palotie, Aarno; Panoutsopoulou, Kalliope; Parker, Victoria; Parr, Jeremy R.; Paternoster, Lavinia; Paunio, Tiina; Payne, Felicity; Payne, Stewart J.; Perry, John R. B.; Pietilainen, Olli; Plagnol, Vincent; Pollitt, Rebecca C.; Porteous, David J.; Povey, Sue; Quail, Michael A.; Quaye, Lydia; Raymond, F. Lucy; Rehnström, Karola; Richards, J Brent; Ridout, Cheryl K.; Ring, Susan M.; Ritchie, Graham R.S.; Roberts, Nicola; Robinson, Rachel L.; Savage, David B.; Scambler, Peter; Schiffels, Stephan; Schmidts, Miriam; Schoenmakers, Nadia; Scott, Richard H.; Semple, Robert K.; Serra, Eva; Sharp, Sally I.; Shaw, Adam; Shihab, Hashem A.; Shin, So Youn; Skuse, David; Small, Kerrin S; Smee, Carol; Smith, Blair H.; Davey Smith, George; Soranzo, Nicole; Southam, Lorraine; Spasic-Boskovic, Olivera; Spector, Timothy D; St Clair, David; St Pourcain, Beate; Stalker, Jim; Stevens, Elizabeth; Sun, Jianping; Surdulescu, Gabriela L; Suvisaari, Jaana; Syrris, Petros; Taylor, Rohan; Tian, Jing; Timpson, Nicholas J.; Tobin, Martin D; Valdes, Ana M.; Vandersteen, Anthony M.; Vijayarangakannan, Parthiban; Visscher, Peter M.; Wain, Louise V.; Walter, Klaudia; Walters, James T.R.; Wang, Guangbiao; Wang, Jun; Wang, Nai-Yu; Ward, Kirsten; Whyte, Tamieka; Williams, Hywel J.; Williamson, Kathleen A.; Wilson, Crispian; Wilson, Scott G.; Wong, Kim; Xu, Changjiang; Yang, Jian; Zhang, Feng; Zhang, Pingbo; Zheng, Hou Feng

    2017-01-01

    Obesity is a genetically heterogeneous disorder. Using targeted and whole-exome sequencing, we studied 32 human and 87 rodent obesity genes in 2,548 severely obese children and 1,117 controls. We identified 52 variants contributing to obesity in 2% of cases including multiple novel variants in GNAS,

  4. Severe Bone Loss induced by Orthodontic Elastic Separator: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    A E Vishwanath

    2013-01-01

    Full Text Available A displaced orthodontic elastic separator was proposed as being the source of a gingival abscess that progressed to severe bone loss and exfoliation in a healthy adolescent patient with sound periodontal status prior to commencement of orthodontic treatment. After 1 year of undergoing orthodontic treatment, the patient presented with dull pain and mobility in the left upper permanent molar for which there was no apparent etiology. On clinical examination, the patient had gingival inflammation, associated with a deep pocket and severe mobility (grade III in relation to the same teeth. Radiographic examination of an orthopantomogram and intraoral periapical radiography (IOPAR revealed a chronic periodontal abscess with severe necrosis of the periodontal ligament and severe alveolar bone loss. A radiopaque mass on the distal surface below the cementoenamel junction (CEJ was also observed. The patient was referred to the department of periodontics for assessment and appropriate treatment. On curettage, it was found that there was orthodontic elastic separator which was displaced subgingivally.

  5. Rare variant analysis of human and rodent obesity genes in individuals with severe childhood obesity

    DEFF Research Database (Denmark)

    Hendricks, Audrey E.; Bochukova, Elena G.; Marenne, Gaëlle

    2017-01-01

    Obesity is a genetically heterogeneous disorder. Using targeted and whole-exome sequencing, we studied 32 human and 87 rodent obesity genes in 2,548 severely obese children and 1,117 controls. We identified 52 variants contributing to obesity in 2% of cases including multiple novel variants in GN...

  6. Rare and common variants in LPL and APOA5 in Thai subjects with severe hypertriglyceridemia: A resequencing approach.

    Science.gov (United States)

    Khovidhunkit, Weerapan; Charoen, Supannika; Kiateprungvej, Arunrat; Chartyingcharoen, Palm; Muanpetch, Suwanna; Plengpanich, Wanee

    2016-01-01

    Severe hypertriglyceridemia usually results from a combination of genetic and environmental factors. Few data exist on the genetics of severe hypertriglyceridemia in Asian populations. To examine the genetic variants of 3 candidate genes known to influence triglyceride metabolism, LPL, APOC2, and APOA5, which encode lipoprotein lipase, apolipoprotein C-II, and apolipoprotein A-V, respectively, in a large group of Thai subjects with severe hypertriglyceridemia. We identified sequence variants of LPL, APOC2, and APOA5 by sequencing exons and exon-intron junctions in 101 subjects with triglyceride levels ≥ 10 mmol/L (886 mg/dL) and compared with those of 111 normotriglyceridemic subjects. Six different rare variants in LPL were found in 13 patients, 2 of which were novel (1 heterozygous missense variant: p.Arg270Gly and 1 frameshift variant: p.Asp308Glyfs*3). Four previously identified heterozygous missense variants in LPL were p.Ala98Thr, p.Leu279Val, p.Leu279Arg, and p.Arg432Thr. Collectively, these rare variants were found only in the hypertriglyceridemic group but not in the control group (13% vs 0%, P severe hypertriglyceridemia. A common p.Gly185Cys APOA5 variant, in particular, was quite prevalent and potentially contributed to hypertriglyceridemia in this group of patients. Copyright © 2015 National Lipid Association. Published by Elsevier Inc. All rights reserved.

  7. Rare severe mycotic infections in children receiving empirical caspofungin treatment for febrile neutropenia

    Directory of Open Access Journals (Sweden)

    Deniz Yilmaz Karapinar

    2015-09-01

    Full Text Available Empirical antifungal therapy is most often given to patients with leukemia. However breakthrough fungal infections under antifungal therapy are not uncommon. Four children, with hematologic malignant disease developed mycotic breakthrough infections while on empirical caspofungin treatment for a median of 14 (range 11–19 days. Trichosporon asahii was detected in the blood culture of two patients and Geotrichum capitatum in the other two (one patient also had positive cerebrospinal fluid culture. Because the patients’ clinical situation worsened, voriconazole was empirically added for two patients three and five days before the agent was detected. The first sterile blood culture was obtained 3–7 days of voriconazole treatment. All patients reached clear cultures but one patient died. One patient with central nervous system infection with G. capitatum had severe neurological sequelae. Very severe fungal infections can occur during empirical caspofungin therapy. Therefore, patients should be followed closely.

  8. A Rare Case of Parkinson's Disease with Severe Neck Pain Owing to Crowned Dens Syndrome

    OpenAIRE

    Teruyuki Takahashi; Masato Tamura; Keiichi Osabe; Takashi Tamiya; Kenji Miki; Mai Yamaguchi; Kanno Akira; Satoshi Kamei; Toshiaki Takasu

    2014-01-01

    Background: Pain is regarded as one of the most common nonmotor symptoms in Parkinson's disease (PD). In particular, musculoskeletal pain has been reported as the most common type of PD-associated pain. Crowned dens syndrome (CDS), related to microcrystalline deposition in the periodontoid process, is the main cause of acute or chronic cervical pain. Case Presentation: This report describes the case of an 87-year-old woman who had severe bradykinesia, muscle rigidity, gait disturbance and nec...

  9. A rare form of atrioventricular septal defect with severe subaortic stenosis.

    Science.gov (United States)

    Venugopalan, P; Agarwal, A K; Reyes, Z

    2001-06-01

    An unusual form of atrioventricular septal defect associated with severe subaortic obstruction is reported in a neonate who presented with intractable cardiac failure. The baby had a large defect in the atrioventricular septum allowing communication from the left ventricle to the right atrium, without interatrial or interventricular communication, and a cleft anterior mitral leaflet. The baby expired despite palliative surgery performed to bypass the subaortic stenosis.

  10. Alkaptonuria: A rare cause of recurrent severe back pain in the emergency department

    Directory of Open Access Journals (Sweden)

    MSeidahmed

    2012-09-01

    Full Text Available We report a 45 year-old male patient who presented to the emergency department of Hamad General Hospital with recurrent severe low back pain. Clinical examination revealed characteristic deposition of blue-brownish pigment in the sclera and ear. X-ray revealed diffuse intervertebral disc calcification. Alkaptonuria was suspected and the diagnosis was confirmed by detection of high levels of homogentisic acid in the urine.

  11. Severe Bone Loss induced by Orthodontic Elastic Separator: A Rare Case Report

    OpenAIRE

    A E Vishwanath; B K Sharmada; Sandesh S Pai; Nandini Nelvigi

    2013-01-01

    A displaced orthodontic elastic separator was proposed as being the source of a gingival abscess that progressed to severe bone loss and exfoliation in a healthy adolescent patient with sound periodontal status prior to commencement of orthodontic treatment. After 1 year of undergoing orthodontic treatment, the patient presented with dull pain and mobility in the left upper permanent molar for which there was no apparent etiology. On clinical examination, the patient had gingival inflammation...

  12. Direct detection of common and rare inversion mutations in the genetic diagnosis of severe hemophilia A

    Energy Technology Data Exchange (ETDEWEB)

    Windsor, A.S.; Lillicrap, D.P.; Taylor, S.A.M. [Queen`s Univ., Ontario (Canada)

    1994-09-01

    Approximately 50% of the cases of severe hemophilia A (factor VIII:C < 0.01 units/ml) may be due to gross rearrangements of the factor VIII gene. The mutation involves homologous sequences upstream of the factor VIII locus and within intron 22 in an intrachromosomal recombination, inversion, event. The rearrangements can readily be detected on a Southern blot using a probe that is complementary to sequences from within intron 22. We describe here the analysis of this mutation in 71 severe hemophilia A patients. Thirty two of the patients (45%) showed evidence of a rearrangement. Five different patterns of rearrangements were seen, two of which have previously been described and account for the majority of cases (pattern 1, 70% and pattern 2, 16%). Three other abnormal patterns were observed. The inversion mechanism does not usually result in the loss or gain of any genetic material, but in one patient, in whom a unique rearrangement pattern was observed (pattern 3), we have previously documented a gross deletion which removes exons 1-22 of the factor VII gene as well as sequences 5{prime} to the gene. In another individual a fourth pattern in which an extra 19.0 kb band is present was detected. In this case it is unclear as to whether the rearrangement is responsible for the disease or is simply coincident normal variation. A fifth pattern, in which an extra 16.0 kb band was detected, was observed in a family with a new mutation causing hemophilia A. The affected individual and his mother inherited a de novo rearrangement of the factor VIII gene from his unaffected grandfather, implicating it as the cause of the disease. In conclusion, testing for the factor VIII inversion mutation was positive in approximately 45% of severe hemophiliacs, 72% of whom were isolated cases, and as such should constitute the initial stage in the genetic testing protocol for these patients` families.

  13. Severe steatohepatitis in a patient with a rare neutral lipid storage disorder due to ABHD5 mutation.

    Science.gov (United States)

    Ronchetti, Anna; Prati, Daniele; Pezzotta, Maria Grazia; Tavian, Daniela; Colombo, Roberto; Callea, Francesco; Colli, Agostino

    2008-09-01

    Fatty liver disease is mainly caused by alcohol consumption, excessive body weight, dyslipidemia and impaired glucose tolerance, but inherited disorders can sometimes be involved. We report the case of a 40-year-old woman with steatohepatitis and severe portal hypertension, associated with ichthyosis, cataract and hypoacusia. The clinical, pathological and genetic findings were consistent with a diagnosis of Chanarin-Dorfman syndrome (CDS), a rare autosomal recessive inherited neutral lipid storage disorder, and genetic analysis showed that a novel ABHD5 mutation is responsible.

  14. Cryptosporidiosis: a rare and severe infection in a pediatric renal transplant recipient.

    Science.gov (United States)

    Acikgoz, Yonca; Ozkaya, Ozan; Bek, Kenan; Genc, Gurkan; Sensoy, Sema Gulnar; Hokelek, Murat

    2012-06-01

    Cryptosporidium is an intracellular protozoan parasite that causes gastroenteritis in human. In immunocompromised individuals, cryptosporidium causes far more serious disease. There is no effective specific therapy for cryptosporidiosis, and spontaneous recovery is the rule in healthy individuals. However, immunocompromised patients need effective and prolonged therapy. Here, we present our clinical experience in a six-yr-old boy who underwent living-related donor renal transplantation and who was infected with Cryptosporidium spp. Our patient was successfully treated with antimicrobial agents consisting of spiramycin, nitazoxanide, and paromomycin. At the end of second week of therapy, his stool became negative for Cryptosporidium spp. antigen and spiramycin was discontinued. Nitazoxanide and paromomycin treatment was extended to four wk. With this case, we want to emphasize that cryptosporidiosis should be considered in the differential diagnosis of severe or persistent diarrhea in solid organ transplant recipients where rigorous antimicrobial therapy is needed. © 2011 John Wiley & Sons A/S.

  15. Severe Anemia with Hemoperitoneum as a First Presentation for Multinodular Hepatocellular Carcinoma: A Rare Event in Western Countries

    Directory of Open Access Journals (Sweden)

    Thein Swe

    2016-01-01

    Full Text Available Hemoperitoneum due to spontaneous rupture of hepatocellular carcinoma is a life-threatening and rare condition in western countries with an incidence of less than 3% because of early detection of cirrhosis and neoplasm. Here, we describe a case of a 66-year-old male patient with altered mental status with hemorrhagic shock. Computed tomography scan of abdomen revealed hemoperitoneum and mass in liver. Patient underwent resection of liver tumor and biopsy revealed multinodular hepatocellular carcinoma. A high degree of suspicion is required where severe anemia and hemoperitoneum can be a first presentation for hepatocellular carcinoma especially in patients with chronic hepatitis C infection. Early diagnosis is crucial since mortality rates remain high for untreated cases.

  16. Neonatal severe hyperparathyroidism caused by homozygous mutation in CASR: A rare cause of life-threatening hypercalcemia.

    Science.gov (United States)

    Murphy, Heidi; Patrick, Jessica; Báez-Irizarry, Eileen; Lacassie, Yves; Gómez, Ricardo; Vargas, Alfonso; Barkemeyer, Brian; Kanotra, Sohit; Zambrano, Regina M

    2016-04-01

    Neonatal severe hyperparathyroidism (NSHPT) is a rare, life-threatening condition that presents with severe hypercalcemia, hyperparathyroidism, and osteopenia in the newborn period. Treatment of NSHPT traditionally includes hydration and bisphosphonates; however newer calcimimetic agents, such as cinacalcet, are now being utilized to prevent or delay parathyroidectomy which is technically difficult in the newborn. Medical treatment success is related to calcium sensing receptor (CaSR) genotype. We report a 4-day-old infant who presented with hyperbilirubinemia, poor feeding, weight loss, severe hypotonia and was ultimately diagnosed with NSHPT. The patient's total serum calcium level of 36.8 mg/dL (reference range: 8.5-10.4 mg/dL) is, to our knowledge, the highest ever documented in this setting. Exome data previously obtained on the infant's parents was re-analyzed demonstrating bi-parental heterozygosity for a mutation of the CASR gene: c.206G > A, and Sanger sequencing data confirmed the patient was a homozygote for the same mutation. Though a patient with the same CaSR gene mutation described here has responded to cinacalcet, our patient did not respond and required parathyroidectomy. Though this case has previously been published as a surgical case report, a full report of the medical management and underlying genetic etiology is warranted; this case underscores the importance of disclosing bi-parental heterozygosity for a gene causing severe neonatal disease particularly when treatment is available and illustrates the need for further in vitro studies of this CaSR mutation. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  17. Mineral-leaching chemical transport with runoff and sediment from severely eroded rare-earth tailings in southern China

    Science.gov (United States)

    Lu, Huizhong; Cao, Longxi; Liang, Yin; Yuan, Jiuqin; Zhu, Yayun; Wang, Yi; Gu, Yalan; Zhao, Qiguo

    2017-08-01

    Rare-earth mining has led to severe soil erosion in southern China. Furthermore, the presence of the mineral-leaching chemical ammonium sulfate in runoff and sediment poses a serious environmental threat to downstream water bodies. In this paper, the characteristics of mineral-leaching chemicals in surface soil samples collected in the field were studied. In addition, NH4+ and SO42- transport via soil erosion was monitored using runoff and sediment samples collected during natural rainfall processes. The results demonstrated that the NH4+ contents in the surface sediment deposits increased from the top of the heap (6.56 mg kg-1) to the gully (8.23 mg kg-1) and outside the tailing heap (13.03 mg kg-1). The contents of SO42- in the different locations of the tailing heaps ranged from 27.71 to 40.33 mg kg-1. During typical rainfall events, the absorbed NH4+ concentrations (2.05, 1.26 mg L-1) in runoff were significantly higher than the dissolved concentrations (0.93, 1.04 mg L-1), while the absorbed SO42- concentrations (2.87, 1.92 mg L-1) were significantly lower than the dissolved concentrations (6.55, 7.51 mg L-1). The dissolved NH4+ and SO42- concentrations in runoff displayed an exponentially decreasing tendency with increasing transport distance (Y = 1. 02 ṡ exp( - 0. 00312X); Y = 3. 34 ṡ exp( - 0. 0185X)). No clear trend with increasing distance was observed for the absorbed NH4+ and SO42- contents in transported sediment. The NH4+ and SO42- contents had positive correlations with the silt and clay ratio in transported sediment but negative correlations with the sand ratio. These results provide a better understanding of the transport processes and can be used to develop equations to predict the transport of mineral-leaching chemicals in rare-earth tailings, which can provide a scientific foundation for erosion control and soil management in rare-earth tailing regions in southern China.

  18. Mineral-leaching chemical transport with runoff and sediment from severely eroded rare-earth tailings in southern China

    Directory of Open Access Journals (Sweden)

    H. Lu

    2017-08-01

    Full Text Available Rare-earth mining has led to severe soil erosion in southern China. Furthermore, the presence of the mineral-leaching chemical ammonium sulfate in runoff and sediment poses a serious environmental threat to downstream water bodies. In this paper, the characteristics of mineral-leaching chemicals in surface soil samples collected in the field were studied. In addition, NH4+ and SO42− transport via soil erosion was monitored using runoff and sediment samples collected during natural rainfall processes. The results demonstrated that the NH4+ contents in the surface sediment deposits increased from the top of the heap (6.56 mg kg−1 to the gully (8.23 mg kg−1 and outside the tailing heap (13.03 mg kg−1. The contents of SO42− in the different locations of the tailing heaps ranged from 27.71 to 40.33 mg kg−1. During typical rainfall events, the absorbed NH4+ concentrations (2.05, 1.26 mg L−1 in runoff were significantly higher than the dissolved concentrations (0.93, 1.04 mg L−1, while the absorbed SO42− concentrations (2.87, 1.92 mg L−1 were significantly lower than the dissolved concentrations (6.55, 7.51 mg L−1. The dissolved NH4+ and SO42− concentrations in runoff displayed an exponentially decreasing tendency with increasing transport distance (Y = 1. 02 ⋅ exp( − 0. 00312X; Y = 3. 34 ⋅ exp( − 0. 0185X. No clear trend with increasing distance was observed for the absorbed NH4+ and SO42− contents in transported sediment. The NH4+ and SO42− contents had positive correlations with the silt and clay ratio in transported sediment but negative correlations with the sand ratio. These results provide a better understanding of the transport processes and can be used to develop equations to predict the transport of mineral-leaching chemicals in rare-earth tailings, which can provide a scientific foundation for erosion control and soil management in rare

  19. Geochemical behaviour of rare earths in Vitis vinifera grafted onto different rootstocks and growing on several soils

    International Nuclear Information System (INIS)

    Censi, P.; Saiano, F.; Pisciotta, A.; Tuzzolino, N.

    2014-01-01

    The geochemical behaviour of lanthanides and yttrium (Rare Earth Elements, REEs) has been investigated mainly in geological systems where these elements represent the best proxies of processes involving the occurrence of an interface between different media. This behaviour is assessed according to features recorded in sequences of REE concentrations along the REE series normalised with respect to a reference material. In this study, the geochemical behaviour of REE was investigated in different parts of Vitis vinifera specimens grown off-soil, on soils of different nature and grafted onto several rootstocks in order to evaluate effects induced by these changes. The results indicated that roots are the plant organs where REEs are preferentially concentrated, in particular elements from Sm to Ho (middle REE, MREE) whereas Eu enrichments occur in aerial parts. The geochemical behaviour of REE suggests that MREE enrichments in roots are due to preferential MREE interactions with biological membranes or to surface complexation with newly formed phosphates. Eu-positive anomalies suggest that Eu 3+ can form stable organic complexes in place of Ca 2+ in several biological processes in xylem fluids. The possibility that Eu mobility in these fluids can be enhanced by its reductive speciation as Eu 2+ cannot be ruled out. The assessment of the geochemical behaviour of REE according to the theory of the Tetrad Effect carried out confirms that REEs coming from soil are scavenged onto root tissues or mineral surfaces whereas their behaviour in aerial parts of V. vinifera is driven by dissolved complexation. - Highlights: • REE behaviour is driven by scavenging onto authigenic solids or membranes in roots. • REE behaviour is driven by dissolved complexation in aerial plant parts. • Positive Eu anomalies are a consequence of the REE translocation by xylem fluids. • Significant REE tetrad effects are observed in Vitis vinifera plants

  20. Severe Ophthalmological Complications of Thyroid Disease are Rare in Ibadan, Southwestern Nigeria: Results of a Pilot Study

    Directory of Open Access Journals (Sweden)

    Olufunmilola A. Ogun

    2016-01-01

    Full Text Available Background Ocular manifestations of thyroid dysfunction constitute a wide clinical spectrum ranging from minor ocular discomfort, lid retraction, lid lag and ocular injection, to sight threatening eyeball protusion and optic nerve compression. Thyroid-related eye disorders are most commonly associated with Graves' disease, and this most frequently occurs in the setting of hyperthyroidism. However, in 10% of cases, typical eye signs have also been reported in euthyroid and hypothyroid states. The severity of thyroid eye disease has been linked to cigarette smoking. There is very little data specifically reporting the ocular manifestations of thyroid disease among black African patients and there is no known report from Nigeria. This pilot study therefore focused on documenting the ocular signs accompanying thyroid dysfunction in a black African population. Aim To evaluate the pattern of ocular complications, among patients treated for thyroid disorders, in a major Nigerian teaching hospital. Results A total of 75 patients with thyroid dysfunction, were evaluated, comprising 63 females and 12 males. There was a very low prevalence of smoking among patients (<5%. Graves' disease was the commonest thyroid disorder, representing 70% of cases. Seventy-eight percent of patients were hyperthyroid, 11.8% were euthyroid and only 9.8% of patients were hypothyroid. Commonest systemic symptoms were neck swelling (68.6%, weight loss (63.8%, tremors (60.9% and palpitations (59.4%. Two-thirds of patients reported ocular symptoms consisting mainly of painless eye swelling (66.7% and ocular irritation (58%. Conjunctival injection, lid lag and lid retraction were the commonest ocular signs. Chemosis, severe proptosis and ocular motility disorder were very rare. Optic neuropathy was found in 4 patients but was related to pre-existing glaucoma. Majority of patients required only ocular emollients and tear supplements. Conclusion Severe ocular complications of

  1. Quantifying population preferences around vaccination against severe but rare diseases: A conjoint analysis among French university students, 2016.

    Science.gov (United States)

    Seanehia, Joy; Treibich, Carole; Holmberg, Christine; Müller-Nordhorn, Jacqueline; Casin, Valerie; Raude, Jocelyn; Mueller, Judith E

    2017-05-09

    Several concepts are available to explain vaccine decision making by individual and inter-individual factors, including risk perception, social conformism and altruism. However, only a few studies have quantified the weight of these determinants in vaccine acceptance. Using a conjoint analysis tool, we aimed at eliciting preferences in a student population regarding vaccination against a rare, severe and rapidly evolving hypothetical disease, similar to meningococcal serogroup C meningitis or measles. During March-May 2016, we conducted an emailing survey among university students aged 18-24years (N=775) in Rennes, France. Participants were asked to decide for or against immediate vaccination in 24 hypothetical scenarios, containing various levels of four attributes: epidemic situation, adverse events, information on vaccination coverage, and potential for indirect protection. Data were analysed using random effect estimator logit models. Participants accepted on average 52% of scenarios and all attributes significantly impacted vaccination acceptance. The highest positive effects were seen with an epidemic situation (OR 3.81, 95%-CI 3.46-4.19), 90% coverage in the community (3.64, 3.15-4.20) and potential for disease elimination from the community (2.87, 2.53-3.26). Information on "insufficient coverage" was dissuasive (vs. none of friends vaccinated: 0.65, 0.56-0.75). Controversy had a significantly greater negative effect than a confirmed risk of severe adverse events (OR 0.05 vs. 0.22). In models including participant characteristics, preference weights were unchanged, while trust in health authorities and vaccination perceptions strongly influenced acceptance themselves. The greatest significant variation of preference weights between subgroups was observed with controversy among students using alternative medicine daily (OR 0.28) and among students relying on scientific vaccine information (OR 0.02). Among young adults, potential for indirect protection and

  2. Fusion of lower limbs with severe urogenital malformation in a newborn, a rare congenital clinical syndrome: case report

    Directory of Open Access Journals (Sweden)

    Al Hadhoud F

    2017-09-01

    Full Text Available Fatemah Al Hadhoud,1 Abeer H Kamal,1 Abdulmohsen Al Anjari,1 Michael FE Diejomaoh1,2 1Department of Obstetrics and Gynecology, Maternity Hospital, 2Department of Obstetrics and Gynecology, Faculty of Medicine, Kuwait University, Kuwait City, Kuwait Background: Fused lower limbs combined with severe urogenital malformation, also known as sirenomelia, is a rare congenital clinical syndrome. The etiology is unknown, and the outcome for the affected fetus is rather uncertain. Case report: Mrs RB, a Kuwaiti woman primigravida, married to a non-consanguineous husband, had uneventful antenatal care in a private health service, until she was admitted to the Maternity Hospital, Kuwait, at 31 weeks of gestation with a 3-hour history of ruptured membranes. She had a negative family history for diabetes mellitus, and her past surgical/medical/gynecological history was noncontributory. General physical examination revealed a healthy parturient with normal vital signs, clear lungs and normal heart sounds. Obstetric examination revealed a fundal height compatible with the gestational age; there was a single living fetus in breech presentation; she was not in labor. The mother was managed conservatively with antibiotics and dexamethasone injections. Labor ensued later, progressing rapidly to the second stage. Assisted breech delivery was performed, and a live baby, birth weight 1570 g and Apgar score 3/9, was delivered. The neonate had multiple congenital abnormalities, including fusion of both lower limbs, ambiguous genitalia, cloacal anomaly and tracheoesophageal fistula. The neonate was admitted to the neonatal intensive care unit, fully investigated, managed medically and surgically and eventually discharged home after a hospital stay of 123 days for further management. Conclusion: A case of peculiar fetal anomaly called sirenomelia, the first case in Kuwait, has been presented. Survival of such babies requires costly management with average results

  3. A rare case of severe third degree friction burns and large Morel-Lavallee lesion of the abdominal wall.

    Science.gov (United States)

    Brown, Darnell J; Lu, Kuo Jung G; Chang, Kristina; Levin, Jennifer; Schulz, John T; Goverman, Jeremy

    2018-01-01

    Morel-Lavallee lesions (MLLs) are rare internal degloving injuries typically caused by blunt traumatic injuries and most commonly occur around the hips and in association with pelvic or acetabular fractures. MLL is often overlooked in the setting of poly-trauma; therefore, clinicians must maintain a high degree of suspicion and be familiar with the management of such injuries, especially in obese poly-trauma patients. We present a 30-year-old female pedestrian struck by a motor vehicle who sustained multiple long bone fractures, a mesenteric hematoma, and full-thickness abdominal skin friction burn which masked a significant underlying abdominal MLL. The internal degloving caused significant devascularization of the overlying soft tissue and skin which required surgical drainage of hematoma, abdominal wall reconstruction with tangential excision, allografting, negative pressure wound therapy, and ultimately autografting. MLL is a rare, often overlooked, internal degloving injury. Surgeons must maintain a high index of suspicion when dealing with third degree friction burns as they may mask underlying injuries such as MLL, and a delay in diagnosis can lead to increased morbidity.

  4. Severe fluoropyrimidine toxicity due to novel and rare DPYD missense mutations, deletion and genomic amplification affecting DPD activity and mRNA splicing

    DEFF Research Database (Denmark)

    van Kuilenburg, André B P; Meijer, Judith; Maurer, Dirk

    2017-01-01

    Dihydropyrimidine dehydrogenase (DPD) is the initial and rate-limiting enzyme in the catabolism of 5-fluorouracil (5FU). Genetic variations in DPD have emerged as predictive risk factors for severe fluoropyrimidine toxicity. Here, we report novel and rare genetic variants underlying DPD deficiency...... in 9 cancer patients presenting with severe fluoropyrimidine-associated toxicity. All patients possessed a strongly reduced DPD activity, ranging from 9 to 53% of controls. Analysis of the DPD gene (DPYD) showed the presence of 21 variable sites including 4 novel and 4 very rare aberrations: 3 missense...... of exon 4 immediately upstream of the mutated splice-donor site in the process of DPD pre-mRNA splicing. A lethal toxicity in two DPD patients suggests that fluoropyrimidines combined with other therapies such as radiotherapy might be particularly toxic for DPD deficient patients. Our study advocates...

  5. Arsenic trioxide induced rhabdomyolysis, a rare but severe side effect, in an APL patient: a case report.

    Science.gov (United States)

    He, Haiyan; An, Ran; Hou, Jian; Fu, Weijun

    2017-06-01

    Arsenic trioxide (ATO), a component of the traditional Chinese medicine arsenic sublimate, promotes apoptosis and induces leukemic cell differentiation. Combined with all-trans-retinotic acid (ATRA), ATO has become the first-line induction therapy in treating acute promyelocytic leukemia (APL). The most common side effects of ATO include hepatotoxicity, gastrointestinal symptoms, water-sodium retention, and nervous system damage. In this report, we present a rare side effect, rhabdomyolysis, in a 68-year-old female APL patient who was treated with ATO. After taking 10 mg ATO daily for 6 days, she presented shortness of breath, myodynia, elevated creatine kinase, and acute renal insufficiency. This report describes the first case of ATO-induced rhabdomyolysis.

  6. Presence of accessory penis, colonic duplication and several other congenital anomalies in a child: a very rare association.

    Science.gov (United States)

    Chatterjee, Sayan; Mondal, Prabodh Chandra; Pandey, Shashi Bhushan; Achar, Arun

    2014-10-01

    An accessory penis is a very rare anomaly. Only five cases have been reported thus far to our knowledge. We present the case of a child aged 2 years and 10 months who had a penis-like structure (containing phallus and glans) attached to the right buttock. Associated anomalies were a non-communicating type of colonic duplication, a paramedian stenosed anal opening, a horse-shoe kidney, posterior urethral valves, scoliosis of the lumbo-sacral spine, polydactyly and equino-varus deformity of the right foot. As far as we can tell, this is the first report of an accessory penis associated with colonic duplication and other congenital anomalies. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  7. Esophagitis in a high H. pylori prevalence area: severe disease is rare but concomitant peptic ulcer is frequent.

    Directory of Open Access Journals (Sweden)

    Julio Ponce

    Full Text Available BACKGROUND: Few data are available on the prevalence of erosive and severe esophagitis in Western countries. OBJECTIVE: To retrospectively determine the prevalence and the factors predicting erosive esophagitis and severe esophagitis in a large series of endoscopies in Spain. DESIGN: Retrospective observational study. A multivariate analysis was performed to determine variables predicting severe esophagitis. SETTING: Databases of 29 Spanish endoscopy units. PATIENTS: Patients submitted to a diagnostic endoscopy during the year 2005. INTERVENTIONS: Retrospective review of the databases. MAIN OUTCOME MEASUREMENTS: Esophagitis severity (graded according to the Los Angeles classification and associated endoscopic findings. RESULTS: Esophagitis was observed in 8.7% of the 93,699 endoscopies reviewed. Severe esophagitis (LA grade C or D accounted for 22.5% of cases of the disease and was found in 1.9% of all endoscopies. Incidences of esophagitis and those of severe esophagitis were 86.2 and 18.7 cases per 100,000 inhabitants per year respectively. Male sex (OR 1.89 and advanced age (OR 4.2 for patients in the fourth age quartile were the only variables associated with severe esophagitis. Associated peptic ulcer was present in 8.8% of cases. LIMITATIONS: Retrospective study, no data on individual proton pump inhibitors use. CONCLUSIONS: Severe esophagitis is an infrequent finding in Spain. It occurs predominantly in males and in older individuals. Peptic ulcer disease is frequently associated with erosive esophagitis.

  8. Several new phases in RE-Mg-Ge systems (RE = rare earth metal) - syntheses, structures, and chemical bonding

    International Nuclear Information System (INIS)

    Suen, Nian-Tzu; Bobev, Svilen

    2012-01-01

    Reported are the synthesis and structural characterization of Ce_5Mg_8Ge_8 (its own structure type), CeMg_2_-_xGe_2_+_x (BaAl_4-type structure), RE_4Mg_7Ge_6 (RE = Ce-Nd, Sm; La_4Mg_7Ge_6-type structure), and RE_4Mg_5Ge_6 (RE = Ce, Pr; Tm_4Zn_5Ge_6-type structure). The structures of these compounds have been established by single-crystal and powder X-ray diffraction. These compounds are closely related to each other not only in their chemical compositions but also in their structures. A common structural feature of all are MgGe_4 tetrahedra, which are connected by corner- and/or edge-sharing into complex polyanionic frameworks with the rare-earth metal atoms filling the ''empty'' space. The structures are compared to known types of structures, and we have investigated the chemical bonding in Ce_5Mg_8Ge_8 with electronic structure calculations, which were carried out by the tight-bonding linear muffin-tin orbital (TB-LMTO) method. (Copyright copyright 2012 WILEY-VCH Verlag GmbH and Co. KGaA, Weinheim)

  9. [Severe course of a rare non-tuberculous Mycobacteriosis (M. haemophilum) of the hand - case report and strategic comments].

    Science.gov (United States)

    Schumacher, O; Dabernig, J; Nenadic, I; Ingianni, G; Cedidi, C

    2008-10-01

    Mycobacterium haemophilum belongs to the group of atypical mycobacteria and is rarely reported as a cause of upper extremity and hand infections. It is of low virulence. The bacterium seems to be ubiquitous. Sources and mechanism of infection are poorly defined. A 48-year-old female patient was admitted with chronic flexor tendon synovitis of the left palm and distal forearm site. Three debridements were carried out and wound swabs were taken. No proof of bacterial colonisation was found. Histologically a granulomatous infection with Langerhans cells was revealed. Effectively calculated monotherapy with ciprofloxacin was begun. Six weeks postoperatively Mycobacterium haemophilum was cultivated in a colaboration with the National Reference Centre for Mycobacteria in Borstel. Medication was changed to triple therapy with clarithromycin, ethambutol and rifabutin. The patient could carry out her daytime job three months postoperatively. One year after first admission functional impairment needed to be treated by scar excision and radical flexor tendon tendolysis. The palmar defect was resurfaced by using a transmetacarpal DMCA 2 flap at the same time. An almost full range of motion was achieved with intensive hand and physiotherapy after a total treatment period of 15 months. Patients with upper extremity infections caused by atypical Mycobacteria need qualified hand-surgical care. The decision about need and kind of medicamentous treatment is based on germ differentiation and should be made in cooperation with the National Reference Centre for Mycobacteria in Borstel. To shorten the diagnostic gap between first admission and detection of Mycobacteria in hand infections with a non-typical course of disease we suggest a standardised approach.

  10. Severe iron overload and hyporegenerative anemia in a case with rhesus hemolytic disease: therapeutic approach to rare complications

    Directory of Open Access Journals (Sweden)

    Fatih Demircioğlu

    2010-09-01

    Full Text Available A 33 weeks’ gestation, a baby with rhesus hemolytic disease (RHD, who had received intrauterine transfusions twice, developed cholestatic hepatic disease and late hyporegenerative anemia. Her serum ferritin and bilirubin levels increased to 8842 ng/ml and 17.9 mg/dl, respectively. Liver biopsy showed cholestasis and severe iron overload. Treatment with recombinant erythropoietin (rHuEPO decreased the transfusion need, and intravenous deferoxamine resulted in a marked decreased in serum ferritin levels and normalization of liver function. In patients who have undergone intrauterine transfusions due to RHD, hyperferritinemia and late hyporegenerative anemia should be kept in mind. Chelation therapy in cases with symptomatic hyperferritinemia and rHuEPO treatment in cases with severe hyporegenerative anemia should be considered.

  11. Temporary and Long Term Design Provisions Taken on the French NPP Fleet to Cope with Extended Station Black out in case of Rare and Severe External Events

    International Nuclear Information System (INIS)

    Dupuy, Patricia; Delafond, Carine; Dubois, Alexandre

    2015-01-01

    Following the events at Fukushima, the Institute for Radiological Protection and Nuclear Safety (IRSN) has been strongly involved in a series of reviews related to the robustness of French nuclear power plants in case of 'rare and severe' external hazards. These reviews included in particular the 'stress tests' performed in 2011 as required by the European Commission. Those reviews, and the proposal made by EDF to reinforce NPPs robustness in such situation, led to the introduction of the concept of a hardened safety core (HSC) to avoid massive releases and prolonged effects in the environment in case of rare and severe natural hazards. This concept will be explained in the paper and the new specific electrical equipment as well as the interfaces with the existing electrical distribution required to implement this HSC will be explained. As the detailed design, manufacturing and installation of the HSC in all NPP sites will take several years, temporary measures have been adopted. This paper will also present the electrical sources and the distribution related to those temporary measures. The specific situation of the new built EPR reactor in Flamanville is also addressed. Lastly, in complement to the above on-site design provisions, a Nuclear Rapid Response Force has been set up by EDF to bring off-site support to French NPPs in case of emergency. The paper will describe the type of electrical equipment to be delivered and the principle for distributing the electrical power to the required loads. (authors)

  12. Mumps virus encephalomyelitis in a 19-year old male patient with an undefined severe combined immunodeficiency post-haematopoietic bone marrow transplantation: a rare fatal complication.

    Science.gov (United States)

    Eyre, Toby A; Pelosi, Emanuela; McQuaid, Stephen; Richardson, Deborah; Newman, Joan; Hill, Kate; Veys, Paul; Davies, Graham; Orchard, Kim H

    2013-06-01

    We describe a rare case of fatal mumps encephalomyelitis occurring in 19-year old male following matched unrelated donor peripheral blood haematopoietic stem cell transplantation (HSCT). The indication for HSCT was for an undefined form of severe combined immunodeficiency (SCID). Molecular typing of the mumps viral RNA isolated from neural tissue indicated that the infection was acquired at the time of a mumps outbreak in England and Wales that occurred between 2004 and 2006. This case highlights the importance of considering mumps in the differential diagnosis of central nervous system infection in highly immunosuppressed patients. Copyright © 2013 Elsevier B.V. All rights reserved.

  13. Congenital varicella-zoster virus infection. A rare case of severe brain and ocular malformations without limb or cutaneous involvement in a newborn after maternal subclinical infection

    International Nuclear Information System (INIS)

    Al-Katawee, Yousef A.; Al-Hasoun, Yousef A.; Taha, Mohamed N.; Al-Moslem, Khaled

    2005-01-01

    Although congenital varicella-zoster virus VZV infection is rare, it carries serious morbidity and mortality to the fetus and newborn infant. We report a full term female newborn infant, born to a multipara unbooked mother who had VZV subclinical infection during the first trimester of pregnancy. Routine newborn examination showed cystic malformation of the left eye, and absence of the right eye globe. Radiological work up revealed severe brain and eye malformations, serological studies of both mother and baby were positive for VZV. The baby underwent palliative surgery to the eyes, upon discharge, a plan of multidisciplinary team was made for follow up including neurologist, ophthalmologist, pediatrician and social worker. Congenital VZV infection can be severe enough to cause catastrophic fetal anomalies and damage to the vital organs as many of those infants die in infancy. (author)

  14. Severe loin pain following renal biopsy in a high-risk patient: A case report of a rare combination of pseudoaneurysm and arterio-venous fistula

    Directory of Open Access Journals (Sweden)

    Desai Madhav

    2011-01-01

    Full Text Available We report a 50-year-old male patient with diabetes mellitus and hypertension who presented with low-grade fever, anuria and renal failure. He had no prior history of nephropathy and retinopathy. Since anuria persisted, a renal biopsy was performed using automated gun, under ultrasound guidance. Two hours after the renal biopsy was performed, the patient developed severe left loin pain that required analgesics and sedatives. Ultrasound of the abdomen performed immediately, two hours and four hours after the biopsy, did not reveal any hematoma. The hemoglobin was stable when the patient developed loin pain, but after eight hours decreased to 9.1 g/dL, and computed tomography scan of the abdomen revealed a big peri-nephric hematoma around the left kidney. He was managed with blood transfusions and a selective angiogram was done. It revealed a pseudoaneurysm and arterio-venous fistula from the segmental artery of lower pole of the left kidney; both were closed by using microcoils and liquid embolic agent N-butyl-cyanoacrylate (NBCA. The only risk factor the patient had at the time of renal biopsy was severe renal failure. Our case suggests that severe loin pain immediately after renal biopsy in a patient with renal failure warrants careful follow-up of hemoglobin and imaging, even if initial imaging is normal. Further fall of hemoglobin necessitates early evaluation with angiogram, which helps in diagnosing the treatable, although rare, complications like pseudoaneurysm and arterio-venous fistula.

  15. A Rare de novo Interstitial Duplication at 4p15.2 in a Boy with Severe Congenital Heart Defects, Limb Anomalies, Hypogonadism, and Global Developmental Delay.

    Science.gov (United States)

    Liang, Liyang; Xie, Yingjun; Shen, Yiping; Yin, Qibin; Yuan, Haiming

    2016-01-01

    Proximal 4p deletion syndrome is a relatively rare genetic condition characterized by dysmorphic facial features, limb anomalies, minor congenital heart defects, hypogonadism, cafe-au-lait spots, developmental delay, tall and thin habitus, and intellectual disability. At present, over 20 cases of this syndrome have been published. However, duplication of the same region in proximal 4p has never been reported. Here, we describe a 2-year-5-month-old boy with severe congenital heart defects, limb anomalies, hypogonadism, distinctive facial features, pre- and postnatal developmental delay, and mild cognitive impairments. A de novo 4.5-Mb interstitial duplication at 4p15.2p15.1 was detected by chromosomal microarray analysis. Next-generation sequencing was employed and confirmed the duplication, but revealed no additional pathogenic variants. Several candidate genes in this interval responsible for the complex clinical phenotype were identified, such as RBPJ, STIM2, CCKAR, and LGI2. The results suggest a novel contiguous gene duplication syndrome. © 2016 S. Karger AG, Basel.

  16. Rare lung cancers

    International Nuclear Information System (INIS)

    Berzinec, P.

    2013-01-01

    The RARECARE Project (Rare Cancers in the Europe) supported by the European Union defined the rare cancers by the incidence rate of less than 6/100 000. There are several variants of lung cancer which are rare according to this definition. From the clinical point of view the most interesting are the rare adenocarcinomas and large cell neuroendocrine carcinoma. There are important differences in the diagnostic probability of EGFR and ALK mutations in the mutinous and non-mucin ous adenocarcinomas, in the signet ring cell adenocarcinomas, and large cell carcinomas. The optimal chemotherapy for neuroendocrine large cell carcinomas remains undefined. There is only very limited number of clinical trials aimed on the rare lung cancers and actually none phase III trial. Rare lung cancers continue to be a challenge both for the laboratory and the clinical research. (author)

  17. Rare earths

    Energy Technology Data Exchange (ETDEWEB)

    Cranstone, D A

    1979-01-01

    Rare earth elements are commonly extracted from the minerals monazite, bastnaesite, and xenotine. New uses for these elements are constantly developing; they have found applications in glass polishing, television tube phosphors, high-strength low-alloy steels, magnets, catalysts, refractory ceramics, and hydrogen sponge alloys. In Canada, rare earths have been produced as byproducts of the uranium mining industry, but there was no production of rare earths in 1978 or 1979. The world sources of and markets for the rare earth elements are discussed.

  18. Rare earths

    International Nuclear Information System (INIS)

    1984-01-01

    The conference was held from September 12 to 13, 1984 in Jetrichovice, Czechoslovakia. The participants heard 16 papers of which 4 were inputted in INIS. These papers dealt with industrial separation processes of rare earths, the use of chemical methods of separation from the concentrate of apatite and bastnesite, the effect of the relative permittivity of solvents in the elution of rare earth elements from a cation exchanger, and the determination of the content of different rare earth elements using X-ray fluorescence analysis and atomic absorption spectroscopy. (E.S.)

  19. [«A most strange instance of illness in several siblings»--first description of a rare neurological disease in 1830?].

    Science.gov (United States)

    Nylenna, Magne; Breivik, Noralv; Heiberg, Arvid; Larsen, Øivind

    2016-03-15

    Was district medical officer Jensen the first doctor to describe patients with Pantothenate Kinase-Associated Neurodegeneration (PKAN) in Volda in 1830? A case series of four siblings with the same disease written by district medical officer Peter Jensen (1799-1832) in Aalesund in 1830, was published in the Norwegian medical journal Eyr in 1832. The children, who were healthy almost up to school age, developed dystonic involuntary movements and deformities in all extremities, lost their ability to speak and were emaciated before they died at around the age of nine years. Further information about the family and a fifth affected child has been found in the parish records. The clinical picture is consistent with Pantothenate Kinase-Associated Neurodegeneration (PKAN), a rare condition with basal ganglia iron deposition, described in 1922 by the German neuropathologists Julius Hallervorden (1882-1965) and Hugo Spatz (1888-1969). The disease was formerly called Hallervorden-Spatz syndrome, but because of the medical activities undertaken by these two researchers before and during the Second World War, this eponym is no longer recommended.

  20. Rare particles

    International Nuclear Information System (INIS)

    Kutschera, W.

    1984-01-01

    The use of Accelerator Mass Spectrometry (AMS) to search for hypothetical particles and known particles of rare processes is discussed. The hypothetical particles considered include fractionally charged particles, anomalously heavy isotopes, and superheavy elements. The known particles produced in rare processes discussed include doubly-charged negative ions, counting neutrino-produced atoms in detectors for solar neutrino detection, and the spontaneous emission of 14 C from 223 Ra. 35 references

  1. Severe osteoarthritis of the hand associates with common variants within the ALDH1A2 gene and with rare variants at 1p31

    DEFF Research Database (Denmark)

    Styrkarsdottir, Unnur; Thorleifsson, Gudmar; Helgadottir, Hafdis T

    2014-01-01

    Osteoarthritis is the most common form of arthritis and is a major cause of pain and disability in the elderly. To search for sequence variants that confer risk of osteoarthritis of the hand, we carried out a genome-wide association study (GWAS) in subjects with severe hand osteoarthritis, using...

  2. A rare severe enamel defect on an upper pig molar from an early medieval stronghold in Prague (Czech Republic) - short communication

    Czech Academy of Sciences Publication Activity Database

    Teegen, W.-R.; Kyselý, René

    2016-01-01

    Roč. 86, č. 2 (2016), s. 273-285 ISSN 0372-5480 Institutional support: RVO:67985912 Keywords : enamel hypoplasia * severe dental malformation * infection * pig (Sus domesticus) * Middle Ages * weaning Subject RIV: AC - Archeology, Anthropology, Ethnology Impact factor: 0.302, year: 2016 http://www-staro.vef.unizg.hr/vetarhiv/papers/2016-86-2-11.pdf

  3. Hemolysis, Elevated Liver Enzymes, and Low Platelets, Severe Fetal Growth Restriction, Postpartum Subarachnoid Hemorrhage, and Craniotomy: A Rare Case Report and Systematic Review

    Directory of Open Access Journals (Sweden)

    Shadi Rezai

    2017-01-01

    Full Text Available Introduction. Hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome is a relatively uncommon but traumatic condition occurring in the later stage of pregnancy as a complication of severe preeclampsia or eclampsia. Prompt brain computed tomography (CT or magnetic resonance imaging (MRI and a multidisciplinary management approach are required to improve perinatal outcome. Case. A 37-year-old, Gravida 6, Para 1-0-4-1, Hispanic female with a history of chronic hypertension presented at 26 weeks and 6 days of gestational age. She was noted to have hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome accompanied by fetal growth restriction (FGR, during ultrasound evaluation, warranting premature delivery. The infant was delivered in stable condition suffering no permanent neurological deficit. Conclusion. HELLP syndrome is an uncommon and traumatic obstetric event which can lead to neurological deficits if not managed in a responsive and rapid manner. The central aggravating factor seems to be hypertension induced preeclamptic or eclamptic episode and complications thereof. The syndrome itself is manifested by hemolytic anemia, increased liver enzymes, and decreasing platelet counts with a majority of neurological defects resulting from hemorrhagic stroke or subarachnoid hemorrhage (SAH. To minimize adverse perinatal outcomes, obstetric management of this medical complication must include rapid clinical assessment, diagnostic examination, and neurosurgery consultation.

  4. Rare Events

    Science.gov (United States)

    2009-10-01

    Limited Operational Exercise 1. 1A Limited Operational Exercise is a multiplayer experiment designed to exploit and study information sharing and...1.4 Summary of the Study The “rare event” of interest is an extreme, deliberate act of violence , destruction or socioeconomic disruption, such as an...connection with terrorism inves- tigations. The programs then use some combination of doctrinal revision and rewards to induce the people to abandon violence

  5. Rare earths 1998 market update

    International Nuclear Information System (INIS)

    Tourre, J.M.

    1998-01-01

    The rare earth industry has always been a world of rapid change with the emergence of new markets, new ores and new players, as well as the disappearance of old applications. Rare earth based products are used in a great diversity of applications such as hard disk drives, CD drives, batteries, capacitors, pigments, ceramics, polishing powders, fuel cells, flints, catalyst converter, fluid cracking catalysts, etc. South East Asia holds the largest share of the known reserve of rare earth ores and is one of the major markets for rare earth compounds; in the last ten years, China has become the largest producer of rare earth intermediates as well as an important exporter of separated rare earth elements. Today, China has approximately 150 factories producing rare earth compounds, most of which are experiencing financial difficulties due to the lack of knowledge of true market needs, lack of control of their distribution channels and production over-capacity. Recently the Chinese rare earth producers have recognized the situation and efforts are underway to rationalize rare earth production. Japan has dominated many of the major application markets, and is by far the largest market for metal and alloy products. This will remain the case for the next five years; however, new countries are emerging as significant users of rare earth products such as Korea, Taiwan and Malaysia. During the last ten years rare earth producers adjusted to several radical changes that affected the raw materials, the application mix and the price structure. New producers have emerged, especially from China; some have subsequently stopped their activities while others have focused their efforts in a specific market segment

  6. Rare earth metals, rare earth hydrides, and rare earth oxides as thin films

    International Nuclear Information System (INIS)

    Gasgnier, M.

    1980-01-01

    The review deals with pure rare earth materials such as rare earth metals, rare earth hydrides, and rare earth oxides as thin films. Several preparation techniques, control methods, and nature of possible contaminations of thin films are described. These films can now be produced in an extremely well-known state concerning chemical composition, structure and texture. Structural, electric, magnetic, and optical properties of thin films are studied and discussed in comparison with the bulk state. The greatest contamination of metallic rare earth thin films is caused by reaction with hydrogen or with water vapour. The compound with an f.c.c. structure is the dihydride LnH 2 (Ln = lanthanides). The oxygen contamination takes place after annealing at higher temperatures. Then there appears a compound with a b.c.c. structure which is the C-type sesquioxide C-Ln 2 O 3 . At room atmosphere dihydride light rare earth thin films are converted to hydroxide Ln(OH) 3 . For heavy rare earth thin films the oxinitride LnNsub(x)Osub(y) is observed. The LnO-type compound was never seen. The present review tries to set the stage anew for the investigations to be undertaken in the future especially through the new generations of electron microscopes

  7. Rare earth germanates

    International Nuclear Information System (INIS)

    Bondar', I.A.; Vinogradova, N.V.; Dem'yanets, L.N.

    1983-01-01

    Rare earth germanates attract close attention both as an independent class of compounds and analogues of a widely spread class of natural and synthetic minerals. The methods of rare earth germanate synthesis (solid-phase, hydrothermal) are considered. Systems on the basis of germanium and rare earth oxides, phase diagrams, phase transformations are studied. Using different chemical analysese the processes of rare earth germanate formation are investigated. IR spectra of alkali and rare earth metal germanates are presented, their comparative analysis being carried out. Crystal structures of the compounds, lattice parameters are studied. Fields of possible application of rare earth germanates are shown

  8. Neonatal hemophilia: a rare presentation

    Directory of Open Access Journals (Sweden)

    Nuno Ferreira

    2015-12-01

    Full Text Available Hemophilia A is a X-linked hereditary condition that lead to decreased factor VIII activity, occurs mainly in males. Decreased factor VIII activity leads to increased risk of bleeding events. During neonatal period, diagnosis is made after post-partum bleeding complication or unexpected bleeding after medical procedures. Subgaleal hemorrhage during neonatal period is a rare, severe extracranial bleeding with high mortality and usually related to traumatic labor or coagulation disorders. Subgaleal hemorrhage complications result from massive bleeding. We present a neonate with unremarkable family history and uneventful pregnancy with a vaginal delivery with no instrumentation, presenting with severe subgaleal bleeding at 52 hours of life. Aggressive support measures were implemented and bleeding managed. The unexpected bleeding lead to a coagulation study and the diagnosis of severe hemophilia A. There were no known sequelae. This case shows a rare hemophilia presentation reflecting the importance of coagulation studies when faced with unexplained severe bleeding.

  9. Mining with Rare Cases

    Science.gov (United States)

    Weiss, Gary M.

    Rare cases are often the most interesting cases. For example, in medical diagnosis one is typically interested in identifying relatively rare diseases, such as cancer, rather than more frequently occurring ones, such as the common cold. In this chapter we discuss the role of rare cases in Data Mining. Specific problems associated with mining rare cases are discussed, followed by a description of methods for addressing these problems.

  10. Rare earth - no case for government intervention

    OpenAIRE

    Georg Zachmann

    2010-01-01

    China has officially restricted exports of rare earth for several years and announced this year it will further tighten exports. Rare earth is a group of 17 different metals, usually found clustered together. These metals have hundreds of different industry applications. For example, they are used in certain high capacity magnets, batteries and lasers. As the rare earth elements are used in sectors that are assumed to have an over-proportionate growth potential (eg. green-technology), policy ...

  11. Rare earth sulfates

    International Nuclear Information System (INIS)

    Komissarova, L.N.; Shatskij, V.M.; Pokrovskij, A.N.; Chizhov, S.M.; Bal'kina, T.I.; Suponitskij, Yu.L.

    1986-01-01

    Results of experimental works on the study of synthesis conditions, structure and physico-chemical properties of rare earth, scandium and yttrium sulfates, have been generalized. Phase diagrams of solubility and fusibility, thermodynamic and crystallochemical characteristics, thermal stability of hydrates and anhydrous sulfates of rare earths, including normal, double (with cations of alkali and alkaline-earth metals), ternary and anion-mixed sulfates of rare earths, as well as their adducts, are considered. The state of ions of rare earths, scandium and yttrium in aqueous sulfuric acid solutions is discussed. Data on the use of rare earth sulfates are given

  12. Rare diseases and orphan drugs

    Directory of Open Access Journals (Sweden)

    Domenica Taruscio

    2011-01-01

    Full Text Available According to the Regulation (EC N. 141/2000 of the European Parliament and of the Council, rare diseases are life-threatening or chronically debilitating conditions, affecting no more than 5 in 10 000 persons in the European Community. It is estimated that between 6000 to 8000 distinct rare diseases affect up to 6% of the total EU population. Therefore, these conditions can be considered rare if taken individually but they affect a significant proportion of the European population when considered as a single group. Several initiatives have been undertaken at international, European and national level to tackle public health as well as research issues related to the prevention, diagnosis, treatment and surveillance of these diseases. The development of innovative and effective medical products for their diagnosis and treatment is frequently hampered by several factors, including the limited knowledge of their natural history, the difficulties in setting up clinical studies due to the limited numbers of patients affected by a specific disease, the weak interest of sponsors due to the restricted market opportunities. Therefore, incentives and other facilitations have been adopted in many parts of the world, including in the EU, in order to facilitate the development and commercialization of diagnostic tools and treatments devoted to rare diseases. This paper illustrates mainly the European initiatives and will discuss the problematic and controversial aspects surrounding orphan drugs. Finally, activities and measures adopted in Italy are presented.

  13. Towards Rare Itemset Mining

    OpenAIRE

    Szathmary , Laszlo; Napoli , Amedeo; Valtchev , Petko

    2007-01-01

    site de la conférence : http://ictai07.ceid.upatras.gr/; International audience; We describe here a general approach for rare itemset mining. While mining literature has been almost exclusively focused on frequent itemsets, in many practical situations rare ones are of higher interest (e.g., in medical databases, rare combinations of symptoms might provide useful insights for the physicians). Based on an examination of the relevant substructures of the mining space, our approach splits the ra...

  14. Rare earth octacyanomolybdates(4)

    International Nuclear Information System (INIS)

    Zubritskaya, D.I.; Sergeeva, A.N.; Pisak, Yu.V.

    1980-01-01

    Optimal conditions for synthesis of rare-earth octacyanomolybdates(4) of the Ln 4 [Mo(CN) 8 ] 3 xnH 2 O composition (where Ln is a rare-earth element, other than Pr, Pm, Lu, Tb) have been worked out. The synthesis has been accomplished by neutralization with octacianomolybdic acid with rare-earth carbonates. The composition and structure of the compounds synthesized have been studied by infrared-spectroscopy. It has been established that rare-earth octacyanomolybdates(4) form three isostructural groups

  15. Sirenomelia Apus: A Rare Deformity

    Science.gov (United States)

    Kshirsagar, Vinayak Y.; Ahmed, Minhajuddin; Colaco, Sylvia M.

    2012-01-01

    Sirenomelia also known as the mermaid syndrome, is a rare congenital malformation of uncertain etiology. It is characterized by fusion of the lower limbs and commonly associated with severe urogenital and gastrointestinal malformations. There are approximately 300 cases reported in the literature, 15% of which are associated with twinning, most often monozygotic. The syndrome of caudal regression is thought to be the result of injury to the caudal mesoderm early in gestation. PMID:24027712

  16. Sirenomelia apus: a rare deformity.

    Science.gov (United States)

    Kshirsagar, Vinayak Y; Ahmed, Minhajuddin; Colaco, Sylvia M

    2012-07-01

    Sirenomelia also known as the mermaid syndrome, is a rare congenital malformation of uncertain etiology. It is characterized by fusion of the lower limbs and commonly associated with severe urogenital and gastrointestinal malformations. There are approximately 300 cases reported in the literature, 15% of which are associated with twinning, most often monozygotic. The syndrome of caudal regression is thought to be the result of injury to the caudal mesoderm early in gestation.

  17. Rare Disease Video Portal

    OpenAIRE

    Sánchez Bocanegra, Carlos Luis

    2011-01-01

    Rare Disease Video Portal (RD Video) is a portal web where contains videos from Youtube including all details from 12 channels of Youtube. Rare Disease Video Portal (RD Video) es un portal web que contiene los vídeos de Youtube incluyendo todos los detalles de 12 canales de Youtube. Rare Disease Video Portal (RD Video) és un portal web que conté els vídeos de Youtube i que inclou tots els detalls de 12 Canals de Youtube.

  18. Rare earth germanates

    International Nuclear Information System (INIS)

    Bondar', I.A.; Vinogradova, N.V.; Dem'yanets, L.N.

    1983-01-01

    From the viewpoint of structural chemistry and general regularities controlling formation reactions of compounds and phases in melts, solid and gaseous states, recent achievements in the chemistry of rare earth germanates are generalized. Methods of synthesizing germanates, systems on the base of germanium oxides and rare earths are considered. The data on crystallochemical characteristics are tabulated. Individual compounds of scandium germanate are also characterized. Processes of germanate formation using the data of IR-spectroscopy, X-ray phase analysis are studied. The structure and morphotropic series of rare earth germanates and silicates are determined. Fields of their present and possible future application are considered

  19. Analysis of rare categories

    CERN Document Server

    He, Jingrui

    2012-01-01

    This book focuses on rare category analysis where the majority classes have smooth distributions and the minority classes exhibit the compactness property. It focuses on challenging cases where the support regions of the majority and minority classes overlap.

  20. SYMPOSIUM: Rare decays

    Energy Technology Data Exchange (ETDEWEB)

    Anon.

    1989-04-15

    Late last year, a symposium entitled 'Rare Decays' attracted 115 participants to a hotel in Vancouver, Canada. These participants were particle physicists interested in checking conventional selection rules to look for clues of possible new behaviour outside today's accepted 'Standard Model'. For physicists, 'rare decays' include processes that have so far not been seen, explicitly forbidden by the rules of the Standard Model, or processes highly suppressed because the decay is dominated by an easier route, or includes processes resulting from multiple transitions.

  1. Rare earths and actinides

    International Nuclear Information System (INIS)

    Coqblin, B.

    1982-01-01

    This paper reviews the different properties of rare-earths and actinides, either as pure metals or as in alloys or compounds. Three different cases are considered: (i) First, in the case of 'normal' rare-earths which are characterized by a valence of 3, we discuss essentially the magnetic ordering, the coexistence between superconductivity and magnetism and the properties of amorphous rare-earth systems. (ii) Second, in the case of 'anomalous' rare-earths, we distinguish between either 'intermediate-valence' systems or 'Kondo' systems. Special emphasis is given to the problems of the 'Kondo lattice' (for compounds such as CeAl 2 ,CeAl 3 or CeB 6 ) or the 'Anderson lattice' (for compounds such as TmSe). The problem of neutron diffraction in these systems is also discussed. (iii) Third, in the case of actinides, we can separate between the d-f hybridized and almost magnetic metals at the beginning of the series and the rare-earth like the metals after americium. (orig.)

  2. SYMPOSIUM: Rare decays

    International Nuclear Information System (INIS)

    Anon.

    1989-01-01

    Late last year, a symposium entitled 'Rare Decays' attracted 115 participants to a hotel in Vancouver, Canada. These participants were particle physicists interested in checking conventional selection rules to look for clues of possible new behaviour outside today's accepted 'Standard Model'. For physicists, 'rare decays' include processes that have so far not been seen, explicitly forbidden by the rules of the Standard Model, or processes highly suppressed because the decay is dominated by an easier route, or includes processes resulting from multiple transitions

  3. Rare (Earth Elements [score

    Directory of Open Access Journals (Sweden)

    Camilo Méndez

    2014-12-01

    Full Text Available Rare (Earth Elements is a cycle of works for solo piano. The cycle was inspired by James Dillon’s Book of Elements (Vol. I-V. The complete cycle will consist of 14 pieces; one for each selected rare (earth element. The chosen elements are Neodymium, Erbium, Tellurium, Hafnium, Tantalum, Technetium, Indium, Dysprosium, Lanthanium, Cerium, Europium, Terbium, Yttrium and Darmstadtium. These elements were selected due to their special atomic properties that in many cases make them extremely valuable for the development of new technologies, and also because of their scarcity. To date, only 4 works have been completed Yttrium, Technetium, Indium and Tellurium.

  4. Rare muon processes: Experiment

    International Nuclear Information System (INIS)

    Walter, H.K.

    1998-01-01

    The decay properties of muons, especially their rare decays, can be used to study very accurately deviations from the Standard Model. Muons with extremely low energies and good spatial definition are preferred for the majority of such studies. With the upgrade of the 590-MeV ring accelerator, PSI possesses the most powerful cyclotron in the world. This makes it possible to operate high-intensity beams of secondary pions and muons. A short review on rare muon processes is presented, concerning μ-e conversion and muonium-antimuonium oscillations. A possible new search for μ→eγ is also mentioned

  5. Surveillance of rare cancers

    NARCIS (Netherlands)

    van der Zwan, Johannes Martinus

    2016-01-01

    The widespread incidence and effects of cancer have led to a growing development in cancer prevention in the form of screening and research programs and cancer registries. Because of the low number of patients with rare cancers this improvement is not applied to the same extent to all cancer

  6. Afganistan and rare earths

    Directory of Open Access Journals (Sweden)

    Emilian M. Dobrescu

    2013-05-01

    Full Text Available On our planet, over a quarter of new technologies for the economic production of industrial goods, are using rare earths, which are also called critical minerals and industries that rely on these precious items being worth of an estimated nearly five trillion dollars, or 5 percent of world gross domestic product. In the near future, competition will increase for the control of rare earth minerals embedded in high-tech products. Rare minerals are in the twenty-first century what oil accounted for in the twentieth century and coal in the nineteenth century: the engine of a new industrial revolution. Future energy will be produced increasingly by more sophisticated technological equipment based not just on steel and concrete, but incorporating significant quantities of metals and rare earths. Widespread application of these technologies will result in an exponential increase in demand for such minerals, and what is worrying is that minerals of this type are almost nowhere to be found in Europe and in other industrialized countries in the world, such as U.S. and Japan, but only in some Asian countries, like China and Afghanistan.

  7. Magnetic rare earth superlattices

    DEFF Research Database (Denmark)

    Majkrzak, C.F.; Kwo, J.; Hong, M.

    1991-01-01

    Advances in molecular beam epitaxy deposition techniques have recently made it possible to grow, an atomic plane at a time, single crystalline superlattices composed of alternating layers of a magnetic rare earth, such as Gd, Dy, Ho, or Er, and metallic Y, which has an identical chemical structure...

  8. [Severe rhabdomyolysis secondary to severe hypernatraemic dehydration].

    Science.gov (United States)

    Mastro-Martínez, Ignacio; Montes-Arjona, Ana María; Escudero-Lirio, Margarita; Hernández-García, Bárbara; Fernández-Cantalejo Padial, José

    2015-01-01

    Rhabdomyolysis is a rare paediatric condition. The case is presented of a patient in whom this developed secondary to severe hypernatraemic dehydration following acute diarrhoea. Infant 11 months of age who presented with vomiting, fever, diarrhoea and anuria for 15 hours. Parents reported adequate preparation of artificial formula and oral rehydration solution. He was admitted with malaise, severe dehydration signs and symptoms, cyanosis, and low reactivity. The laboratory tests highlighted severe metabolic acidosis, hypernatraemia and pre-renal kidney failure (Sodium [Na] plasma 181 mEq/L, urine density> 1030). He was managed in Intensive Care Unit with gradual clinical and renal function improvement. On the third day, slight axial hypotonia and elevated cell lysis enzymes (creatine phosphokinase 75,076 IU/L) were observed, interpreted as rhabdomyolysis. He was treated with intravenous rehydration up to 1.5 times the basal requirements, and he showed a good clinical and biochemical response, being discharged 12 days after admission without motor sequelae. Severe hypernatraemia is described as a rare cause of rhabdomyolysis and renal failure. In critically ill patients, it is important to have a high index of suspicion for rhabdomyolysis and performing serial determinations of creatine phosphokinase for early detection and treatment. Copyright © 2015 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  9. Sharing Rare Attitudes Attracts.

    Science.gov (United States)

    Alves, Hans

    2018-04-01

    People like others who share their attitudes. Online dating platforms as well as other social media platforms regularly rely on the social bonding power of their users' shared attitudes. However, little is known about moderating variables. In the present work, I argue that sharing rare compared with sharing common attitudes should evoke stronger interpersonal attraction among people. In five studies, I tested this prediction for the case of shared interests from different domains. I found converging evidence that people's rare compared with their common interests are especially potent to elicit interpersonal attraction. I discuss the current framework's theoretical implications for impression formation and impression management as well as its practical implications for improving online dating services.

  10. Drugs for rare disorders.

    Science.gov (United States)

    Cremers, Serge; Aronson, Jeffrey K

    2017-08-01

    Estimates of the frequencies of rare disorders vary from country to country; the global average defined prevalence is 40 per 100 000 (0.04%). Some occur in only one or a few patients. However, collectively rare disorders are fairly common, affecting 6-8% of the US population, or about 30 million people, and a similar number in the European Union. Most of them affect children and most are genetically determined. Diagnosis can be difficult, partly because of variable presentations and partly because few clinicians have experience of individual rare disorders, although they may be assisted by searching databases. Relatively few rare disorders have specific pharmacological treatments (so-called orphan drugs), partly because of difficulties in designing trials large enough to determine benefits and harms alike. Incentives have been introduced to encourage the development of orphan drugs, including tax credits and research aids, simplification of marketing authorization procedures and exemption from fees, and extended market exclusivity. Consequently, the number of applications for orphan drugs has grown, as have the costs of using them, so much so that treatments may not be cost-effective. It has therefore been suggested that not-for-profit organizations that are socially motivated to reduce those costs should be tasked with producing them. A growing role for patient organizations, improved clinical and translational infrastructures, and developments in genetics have also contributed to successful drug development. The translational discipline of clinical pharmacology is an essential component in drug development, including orphan drugs. Clinical pharmacologists, skilled in basic pharmacology and its links to clinical medicine, can be involved at all stages. They can contribute to the delineation of genetic factors that determine clinical outcomes of pharmacological interventions, develop biomarkers, design and perform clinical trials, assist regulatory decision

  11. RARE BRANCHIAL ARCH ANOMALIES

    Directory of Open Access Journals (Sweden)

    Jayanta Kumar

    2016-03-01

    Full Text Available AIM Amongst the branchial arch anomalies third arch anomaly occurs rarely and more so the fourth arch anomalies. We present our experience with cases of rare branchial arch anomalies. PATIENTS AND METHODS From June 2006 to January 2016, cases having their external opening in the lower third of sternocleidomastoid muscle with the tract going through thyroid gland and directing to pyriform sinus (PFS or cysts with internal opening in the PFS were studied. RESULTS No fourth arch anomaly was encountered. One cyst with internal opening which later on formed a fistula, three fistulae from beginning and two sinuses were encountered. The main stay of diagnosis was the fistula in the PFS and the tract lying posterior to the internal carotid artery. Simple excision technique with a small incision around the external opening was done. There was no recurrence. CONCLUSION Third arch fistula is not very rare as it was thought. Internal fistula is found in most of the cases. Though radiological investigations are helpful, fistulae can be diagnosed clinically and during operation. Extensive operation of the neck, mediastinum and pharynx is not required.

  12. Ethical aspects on rare diseases.

    Science.gov (United States)

    Barrera, Luis A; Galindo, Gilberto Cely

    2010-01-01

    In this chapter we discuss several of the most relevant subjects related to ethics on Rare Diseases. Some general aspects are discussed such as the socio-psychological problems that confront the patients and their families that finally lead to marginalization and exclusion of patients affected by these diseases from the health programs, even in wealthy countries. Then we address problems related to diagnosis and some ethical aspects of newborn screening, prenatal, pre-implantation diagnosis and reference centers, as well as some conditions that should be met by the persons and institutions performing such tasks. Alternatives of solutions for the most critical situations are proposed. Subsequently the orphan drugs subject is discussed not only from the availability point of view, prizes, industrial practices, and purchasing power in developed and developing societies. The research related to rare disease in children and other especially vulnerable conditions, the need for informed consent, review boards or ethics comities, confidentiality of the information, biobanks and pharmacogenetics are discussed.

  13. Probing supersymmetry in rare B decays

    International Nuclear Information System (INIS)

    Hewett, J.L.

    1996-10-01

    We determine the ability of future experiments to observe supersymmetric contributions to the rare decays B → X s γ and B → X s l + l - . A global fit to the Wilson coefficients which contribute to these decays is performed from Monte Carlo generated data. This fit is then compared to supersymmetric predictions for several different patterns of the superpartner spectrum

  14. Seckel syndrome: A rare case report

    OpenAIRE

    Rinky Sisodia; Ravi Kadur Sundar Raj; Vipin Goel

    2014-01-01

    Seckel syndrome (SS) is a rare, autosomal recessive syndrome; characterized by severe intrauterine and postnatal growth retardation, microcephaly, mental retardation, and typical facial appearance with beaklike protrusion of the midface (bird headed). In addition to the characteristic craniofacial dysmorphism and skeletal defects, abnormalities have been described in the cardiovascular, hematopoietic, endocrine, gastrointestinal, and central nervous systems. Usually such patients have poor ps...

  15. Aging in Rare Intellectual Disability Syndromes

    Science.gov (United States)

    Dykens, Elisabeth M.

    2013-01-01

    This review highlights several methodological challenges involved in research on aging, health, and mortality in adults with rare intellectual disability syndromes. Few studies have been performed in this area, with research obstacles that include: the ascertainment of older adults with genetic versus clinical diagnoses; likelihood that adults…

  16. Rare endocrine cancers have novel genetic alterations

    Science.gov (United States)

    A molecular characterization of adrenocortical carcinoma, a rare cancer of the adrenal cortex, analyzed 91 cases for alterations in the tumor genomes and identified several novel genetic mutations as likely mechanisms driving the disease as well as whole genome doubling as a probable driver of the disease.

  17. Rare B Decays at BaBar

    Energy Technology Data Exchange (ETDEWEB)

    Datta, M

    2005-03-14

    The authors present recent results on rare B meson decays based on data taken by the BABAR detector at the PEP-II asymmetric e{sup +}e{sup -} collider. Included in this report are measurements of branching fractions and other quantities of interest for several hadronic, radiative, electroweak, and purely leptonic decays of B mesons.

  18. Rare Earth Polyoxometalates.

    Science.gov (United States)

    Boskovic, Colette

    2017-09-19

    Longstanding and important applications make use of the chemical and physical properties of both rare earth metals and polyoxometalates of early transition metals. The catalytic, optical, and magnetic features of rare earth metal ions are well-known, as are the reversible multielectron redox and photoredox capabilities of polyoxomolybdates and polyoxotungstates. The combination of rare earth ions and polyoxometalates in discrete molecules and coordination polymers is of interest for the unique combination of chemical and physical properties that can arise. This Account surveys our efforts to synthesize and investigate compounds with rare earth ions and polyoxometalates (RE-POMs), sometimes with carboxylate-based organic coligands. Our general synthetic approach is "bottom-up", which affords well-defined nanoscale molecules, typically in crystalline form and amenable to single-crystal X-ray diffraction for structure determination. Our particular focus is on elucidation of the physical properties conferred by the different structural components with a view to ultimately being able to tune these properties chemically. For this purpose, we employ a variety of spectroscopic, magnetochemical, electrochemical, and scattering techniques in concert with theoretical modeling and computation. Studies of RE-POM single-molecule magnets (SMMs) have utilized magnetic susceptibility, inelastic neutron scattering, and ab initio calculations. These investigations have allowed characterization of the crystal field splitting of the rare earth(III) ions that is responsible for the SMM properties of slow magnetic relaxation and magnetization quantum tunneling. Such SMMs are promising for applications in quantum computing and molecular spintronics. Photophysical measurements of a family of hybrid RE-POMs with organic ligands have afforded insights into sensitization of Tb(III) and Eu(III) emission through both organic and polyoxometalate chromophores in the same molecule. Detailed

  19. Photochemistry in rare gas clusters

    International Nuclear Information System (INIS)

    Moeller, T.; Haeften, K. von; Pietrowski, R. von

    1999-01-01

    In this contribution photochemical processes in pure rare gas clusters will be discussed. The relaxation dynamics of electronically excited He clusters is investigated with luminescence spectroscopy. After electronic excitation of He clusters many sharp lines are observed in the visible and infrared spectral range which can be attributed to He atoms and molecules desorbing from the cluster. It turns out that the desorption of electronically excited He atoms and molecules is an important decay channel. The findings for He clusters are compared with results for Ar clusters. While desorption of electronically excited He atoms is observed for all clusters containing up to several thousand atoms a corresponding process in Ar clusters is only observed for very small clusters (N<10). (orig.)

  20. Photochemistry in rare gas clusters

    Energy Technology Data Exchange (ETDEWEB)

    Moeller, T.; Haeften, K. von; Pietrowski, R. von [Deutsches Elektronen-Synchrotron (DESY), Hamburg (Germany). Hamburger Synchrotronstrahlungslabor; Laarman, T. [Universitaet Hamburg, II. Institut fuer Experimentalphysik, Luruper Chaussee 149, D-22761 Hamburg (Germany)

    1999-12-01

    In this contribution photochemical processes in pure rare gas clusters will be discussed. The relaxation dynamics of electronically excited He clusters is investigated with luminescence spectroscopy. After electronic excitation of He clusters many sharp lines are observed in the visible and infrared spectral range which can be attributed to He atoms and molecules desorbing from the cluster. It turns out that the desorption of electronically excited He atoms and molecules is an important decay channel. The findings for He clusters are compared with results for Ar clusters. While desorption of electronically excited He atoms is observed for all clusters containing up to several thousand atoms a corresponding process in Ar clusters is only observed for very small clusters (N<10). (orig.)

  1. Rare B decays at LEP

    CERN Document Server

    Kluit, P M

    2001-01-01

    The results of the LEP experiments for rare B decays will be reviewed, covering hadronic final states, radiative and other rare decays and results for the inclusive charmless branching ratio. (8 refs).

  2. A rare sight

    CERN Multimedia

    Antonella Del Rosso and The LHCb Collaboration

    2012-01-01

    Today, at the Hadron Collider Physics Symposium in Kyoto, the LHCb collaboration has presented the evidence of a very rare B decay, the rarest ever seen. The result further shrinks the region in which scientists can still look for supersymmetry.   The graph showing evidence of the Bs0 → μ+ μ- decay. The result was presented Monday 12 November at the HCP Conference in Kyoto (photo courtesy of the LHCb Collaboration). Particle decays tell us about the inner properties and functioning of Nature’s physics processes. By studying them and their occurrence, physicists infer the rules that control them. Often, it turns out that some rare decays, which are very difficult to observe, are those in which Nature could reveal the presence of new physics. This is the case of some decays of the Bs0 particle (a particle made of a bottom anti-quark bound to a strange quark), and in particular Bs0 → μ+ μ- whose...

  3. Xanthogranulomatous Prostatitis, a Rare Prostatic Entity

    Directory of Open Access Journals (Sweden)

    Alejandro Noyola

    2017-01-01

    Full Text Available There are several benign prostatic pathologies that can clinically mimic a prostate adenocarcinoma. Xanthogranulomatous prostatitis is a benign inflammatory condition of the prostate and a rare entity. A 47-year old male, with 3 years of lower urinary tract symptoms, with a palpable hypogastric tumor, digital rectal examination: solid prostate, of approximately 60 g. Initial PSA was 0.90 ng/mL. He underwent surgical excision of the lower abdominal nodule and prostatectomy. Histopathology showed xanthogranulomatous prostatitis, without malignancy. Xanthogranulomatous prostatitis is an extremely rare entity that can simulate prostate adenocarcinoma, therefore having a correct histopathological diagnosis is essential.

  4. A rare presentation of methanol toxicity

    Directory of Open Access Journals (Sweden)

    Nikhil Gupta

    2013-01-01

    Full Text Available Methanol is a highly toxic alcohol resembling ethanol in smell and taste. Methanol poisoning is a lethal form of poisoning that can cause severe metabolic acidosis, visual disturbances, and neurological deficit. Brain lesions typically described in methanol toxicity are in the form of hemorrhagic and non-hemorrhagic necrosis of the basal ganglia and sub-cortical white matter. To our knowledge, lesions in the parietal, temporal, or frontal areas of cerebrum and cerebellar hemispheres have been rarely reported so far. We herewith report this rare presentation.

  5. Rare earth superlattices

    International Nuclear Information System (INIS)

    McMorrow, D.F.

    1997-01-01

    A review is given of recent experiments on the magnetism of rare earth superlattices. Early experiments in this field were concerned mainly with systems formed by combining a magnetic and a non-magnetic element in a superlattice structure. From results gathered on a variety of systems it has been established that the propagation of magnetic order through the non-magnetic spacer can be understood mostly on the basis of an RKKY-like model, where the strength and range of the coupling depends on the details of the conduction electron susceptibility of the spacer. Recent experiments on more complex systems indicate that this model does not provide a complete description. Examples include superlattices where the constituents can either be both magnetic, adopt different crystal structures (Fermi surfaces), or where one of the constituents has a non-magnetic singlet ground state. The results from such systems are presented and discussed in the context of the currently accepted model. (au)

  6. Rare nocturnal headaches.

    Science.gov (United States)

    Cohen, Anna S; Kaube, Holger

    2004-06-01

    This review describes rare headaches that can occur at night or during sleep, with a focus on cluster headaches, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing, hypnic headache and exploding head syndrome. It is known that cluster headaches and hypnic headache are associated with rapid eye movement sleep, as illustrated by recent polysomnographic studies. Functional imaging studies have documented hypothalamic activation that is likely to be of relevance to circadian rhythms. These headache syndromes have been shown to respond to melatonin and lithium therapy, both of which have an indirect impact on the sleep-wake cycle. There is growing evidence that cluster headache and hypnic headache are chronobiological disorders.

  7. Rare earth (3) pivalates

    International Nuclear Information System (INIS)

    Kuz'mina, N.P.; Martynenko, L.I.; Zoan An' Tu; Ch'eu Tkhi Nguet; Troyanov, S.I.; Rykov, A.N.; Korenev, Yu.M.

    1994-01-01

    Depending on synthesis conditions rare earth pivalates can be obtained in the form of either adducts NPiv·HPiv or hydrates MPiv 3 ·mH 2 O. Adducts are the most stable form of pivalates. Heating of adducts result in formation of corresponding MPiv 3 . MPiv 3 ·nHPiv compounds are characterized by IR-spectroscopy and thermal analysis data. Behaviour of MPiv 3 was studied in the regime of vacuum sublemation. Using mass spectroscopy of NdPiv 3 it was shown that gaseous phase above MPiv 3 had complex composition and contained ligomer fragments. X-ray structure analysis of [NdPiv 3 ·3HPiv] was conducted

  8. A Rare Stapes Abnormality

    Directory of Open Access Journals (Sweden)

    Hala Kanona

    2015-01-01

    Full Text Available The aim of this study is to increase awareness of rare presentations, diagnostic difficulties alongside management of conductive hearing loss and ossicular abnormalities. We report the case of a 13-year-old female reporting progressive left-sided hearing loss and high resolution computed tomography was initially reported as normal. Exploratory tympanotomy revealed an absent stapedius tendon and lack of connection between the stapes superstructure and footplate. The footplate was fixed. Stapedotomy and stapes prosthesis insertion resulted in closure of the air-bone gap by 50 dB. A review of world literature was performed using MedLine. Middle ear ossicular discontinuity can result in significant conductive hearing loss. This can be managed effectively with surgery to help restore hearing. However, some patients may not be suitable or decline surgical intervention and can be managed safely conservatively.

  9. Rare Decays at LHCb

    CERN Document Server

    Belyaev, Ivan

    2006-01-01

    Rare loop-induced decays are sensitive to New Physics in many Standard Model extensions. In this paper we discuss the reconstruction of the radiative penguin decays $B^0_d \\to K^{*0} \\gamma, B^0_s \\to \\phi \\gamma , B^0_d \\to \\omega \\gamma, \\Lambda_b \\to \\Lambda \\gamma$, the electroweak penguin decays $B^0_d \\to K^{*0} \\mu^+ \\mu^-, B^+_u \\to K^+ \\mu^+ \\mu^-$, the gluonic penguin decays $B^0_d \\to \\phi K^0_S, B^0_s \\to \\phi \\phi$, and the decay $B^0_s \\to \\mu^+\\mu^-$ at LHCb. The selection criteria, evaluated efficiencies, expected annual yields and $B/S$ estimates are presented.

  10. An Erupted Dilated Odontoma: A Rare Presentation

    Directory of Open Access Journals (Sweden)

    Gaurav Sharma

    2016-01-01

    Full Text Available A dilated odontoma is an extremely rare developmental anomaly represented as a dilatation of the crown and root as a consequence of a deep, enamel-lined invagination and is considered a severe variant of dens invaginatus. An oval shape of the tooth lacking morphological characteristics of a crown or root implies that the invagination happened in the initial stages of morphodifferentiation. Spontaneous eruption of an odontoma is a rare occurrence and the occurrence of a dilated odontoma in a supernumerary tooth is even rarer with only a few case reports documented in the English literature. We present an extremely rare case of erupted dilated odontoma occurring in the supernumerary tooth in anterior maxillary region in an 18-year-old male, which, to the best of our knowledge, is the first ever case reported in English literature.

  11. Rare wild Orchids at CERN Meyrin

    CERN Multimedia

    2005-01-01

    There are several "Floral Nature Reserve - Late Mowing" zones at CERN Meyrin. The blossoms of a rare and a not so rare type of wild orchid are currently in flower. The rare one is the bee orchid (Ophrys Apifera) which is a protected perennial. They are very unusual and in some years can appear in great numbers and then sometimes only reappear after a decade. They live in a symbiotic relationship with a soil-dwelling fungus. Its name stems from the fact that its brown, furry lip resembles and smells like a female bee, a mimicry used to attract drones to aid in pollination. The much more distributed species is the pyramidal orchid (Anacamptis Pyramidalis), which due to its size and its bright pink colour is already visible when you pass by in your car.

  12. Rare wild Orchids at CERN Meyrin

    CERN Document Server

    2005-01-01

    There are several "Floral Nature Reserve - Late Mowing" zones at CERN Meyrin. The blossoms of a rare and a not so rare type of wild orchid are currently in flower. The rare one is the bee orchid (Ophrys Apifera) which is a protected perennial. They are very unusual and in some years can appear in great numbers and then sometimes only reappear after a decade. They live in a symbiotic relationship with a soil-dwelling fungus. Its name stems from the fact that its brown, furry lip resembles and smells like a female bee, a mimicry used to attract drones to aid in pollination. The much more distributed species is the pyramidal orchid (Anacamptis Pyramidalis), which due to its size and its bright pink colour is already visible when you pass by in your car. Photos were taken on the late mowing zone adjacent to route Einstein opposite building 57 on 4 June 2005.

  13. Rare cancers are not so rare: The rare cancer burden in Europe

    NARCIS (Netherlands)

    Gatta, Gemma; van der Zwan, Jan Maarten; Casali, Paolo G.; Siesling, Sabine; Dei Tos, Angelo Paolo; Kunkler, Ian; Otter, Renee; Licitra, Lisa

    2011-01-01

    Purpose: Epidemiologic information on rare cancers is scarce. The project Surveillance of Rare Cancers in Europe (RARECARE) provides estimates of the incidence, prevalence and survival of rare cancers in Europe based on a new and comprehensive list of these diseases. Materials and methods: RARECARE

  14. Mammary analogue secretory carcinoma: A rare salivary gland tumour

    African Journals Online (AJOL)

    Salivary gland malignancy is rare, with a global annual incidence of. 3 per 100 000 people.[1,2] A rare salivary gland tumour, mammary analogue secretory carcinoma (MASC), has only recently been described.[3] The few reports and studies concerning MASC have been published in several pathology journals. We report ...

  15. Rare psi decays

    International Nuclear Information System (INIS)

    Partridge, R.

    1986-01-01

    Slightly more than ten years have passed since the psi was discovered, yet the study of psi decays continues to be an active and fruitful area of research. One reason for such longevity is that each successive experiment has increased their sensitivity over previous experiments either by improving detection efficiency or by increasing statistics. This has allowed the observation and, in some cases, detailed studies of rare psi decays. Branching ratios of ≅10-/sup 4/ are now routinely studied, while certain decay channels are beginning to show interesting effects at the 10-/sup 5/ level. Future experiments at the Beijing Electron Positron Collider (BEPC) have the potential for increasing sensitivities by one or two orders of magnitude, thus enabling many interesting studies impossible with current data samples. The author first examines the extent to which psi decays can be used to study electroweak phenomena. The remainder of this work is devoted to the more traditional task of using the psi to study quarks, gluons, and the properties of the strong interaction. Of particular interest is the study of radioactive psi decays, where a number of new particles have been discovered. Recent results regarding two of these particles, the θ(1700) and iota(1450), are discussed, as well as a study of the quark content of the eta and eta' using decays of the psi to vector-pseudoscalar final states

  16. Rare B decays at LHCb

    CERN Document Server

    Puig Navarro, Albert

    2017-01-01

    Rare decays are flavour changing neutral current processes that allow sensitive searches for phenomena beyond the Standard Model (SM). In the SM, rare decays are loop-suppressed and new particles in SM extensions can give significant contributions. The very rare decay $B^0_s\\to\\mu^+\\mu^-$ in addition helicity suppressed and constitutes a powerful probe for new (pseudo) scalar particles. Of particular interest are furthermore tests of lepton universality in rare $b\\to s\\ell^+\\ell^-$ decays. The LHCb experiment is designed for the study of b-hadron decays and ideally suited for the analysis of rare decays due to its high trigger efficiency, as well as excellent tracking and particle identification performance. Recent results from the LHCb experiment in the area of rare decays are presented, including tests of lepton universality and searches for lepton flavour violation.

  17. Celiac crisis: a rare or rarely recognized disease

    International Nuclear Information System (INIS)

    Waheed, N.; Cheema, H.A.; Suleman, H.; Fayyaz, Z.; Mushtaq, I.

    2017-01-01

    Celiac crisis is a serious life threatening complication of celiac disease characterized by profuse diarrhoea, severe dehydration and metabolic disturbances leading to neuromuscular weakness, cardiac arrhythmias and sudden death. It has been described as rare condition and not well documented in the literature. To improve awareness and facilitate diagnosis of this condition, we studied risk factors, pattern of presentation and management plans of celiac crisis. Methods: It was a descriptive cross sectional study. Patients presenting in emergency room(ER) with profuse diarrhoea leading to severe dehydration, neuromuscular weakness, and metabolic acidosis and electrolyte abnormalities enrolled in the studies after positive serology and small bowel biopsy suggestive of celiac disease. Results: Total 126 patients out of 350 fulfilled the criteria including 54 (42.8 percent) male and 71 (56.3 percent) female. The mean age at presentation was 5.25+-1.18 years. Risk factors were poor social status (97.60 percent), consanguinity (96.77 percent), early weaning with gluten contained diet (93.54 percent), and Presenting complaints were loose motion (100 percent), loss of neck holding (96.77 percent), dehydration (96.77 percent), polyuria (95.96 percent), inability to walk (67.74 percent), abdominal distension (85.86 percent). Electrolytes imbalances were hypokalaemia (2.4+-0.55), hypocalcaemia (7.29+-0.66), hypomagnesaemia (1.89+-0.50), hypophosphatemia (2.8+-0.68), hypoalbuminemia (3.05+-0.48) and metabolic acidosis (96 percent). One hundred and twenty patients were stabilized with GFD and correction of dehydration, acidosis and electrolyte imbalance. Six patients needed parenteral steroids ant total parenteral nutrition (TPN). Recovery time from crisis was mean 5.4+-2.73 days (range 3-20 days). Conclusion: Celiac crisis is a common but under recognized problem in developing countries. Commonest presenting feature is neuromuscular paralysis and biochemical abnormality is

  18. Rare-earth elements

    Science.gov (United States)

    Van Gosen, Bradley S.; Verplanck, Philip L.; Seal, Robert R.; Long, Keith R.; Gambogi, Joseph; Schulz, Klaus J.; DeYoung,, John H.; Seal, Robert R.; Bradley, Dwight C.

    2017-12-19

    The rare-earth elements (REEs) are 15 elements that range in atomic number from 57 (lanthanum) to 71 (lutetium); they are commonly referred to as the “lanthanides.” Yttrium (atomic number 39) is also commonly regarded as an REE because it shares chemical and physical similarities and has affinities with the lanthanides. Although REEs are not rare in terms of average crustal abundance, the concentrated deposits of REEs are limited in number.Because of their unusual physical and chemical properties, the REEs have diverse defense, energy, industrial, and military technology applications. The glass industry is the leading consumer of REE raw materials, which are used for glass polishing and as additives that provide color and special optical properties to the glass. Lanthanum-based catalysts are used in petroleum refining, and cerium-based catalysts are used in automotive catalytic converters. The use of REEs in magnets is a rapidly increasing application. Neodymium-iron-boron magnets, which are the strongest known type of magnets, are used when space and weight are restrictions. Nickel-metal hydride batteries use anodes made of a lanthanum-based alloys.China, which has led the world production of REEs for decades, accounted for more than 90 percent of global production and supply, on average, during the past decade. Citing a need to retain its limited REE resources to meet domestic requirements as well as concerns about the environmental effects of mining, China began placing restrictions on the supply of REEs in 2010 through the imposition of quotas, licenses, and taxes. As a result, the global rare-earth industry has increased its stockpiling of REEs; explored for deposits outside of China; and promoted new efforts to conserve, recycle, and substitute for REEs. New mine production began at Mount Weld in Western Australia, and numerous other exploration and development projects noted in this chapter are ongoing throughout the world.The REE-bearing minerals are

  19. A rare cause of wheezing in infancy.

    Science.gov (United States)

    Indinnimeo, Luciana; Pulicati, Patrizio; Della Rocca, Carlo; Barbato, Angelo

    2006-06-01

    We describe an infant with recurrent wheezing and cough caused by an oropharyngeal cyst. Mucosal oropharyngeal cysts arise from obstruction or traumatic severance of a duct in a minor salivary gland, which leads to retention of mucous secretion . The mucosal cyst of the oropharynx is a rare cause of respiratory distress in the infants. The clinical symptoms depend on the size, shape, and location of the cyst.

  20. Rare upper gastrointestinal hemorrhage of cetuximab

    OpenAIRE

    Duan, Shi-Jie; Gao, Zi-Ming; Wang, Peng-Liang; Gong, Bao-Cheng; Huang, Han-Wei; Luo, Lei; Wang, Xin; Xing, Ya-Nan; Xu, Hui-Mian; Liu, Fu-Nan

    2017-01-01

    Abstract Rationale: cetuximab, an epidermal growth factor receptor inhibitor, is a targeted therapeutic regimen of colorectal cancers. Several common adverse effects have been found, such as cutaneous or gastrointestinal toxicity. However, according to the articles had been published, upper gastrointestinal bleeding (UGIB) is considered to be rare and its mechanism remains unclear. Patient concerns: In this report, we presented a 42-year-old male patient with advanced recto-sigmoid cancer. Af...

  1. Overview of rare B-decays

    CERN Document Server

    Prisciandaro, Jessica

    2017-01-01

    Being extremely suppressed in the Standard Model, rare decays of heavy-flavoured particles are a powerful probe of New Physics, and allow to reach energies beyond those accessible through direct searches. Several new results have been obtained by the LHC experiments. In particular, $b \\to sl^+l^-$ transitions give access to a large spectrum of observables, which provide complementary information on possible New Physics contributions. In this sector, tensions with Standard Model predictions have been observed.

  2. China's rare-earth industry

    Science.gov (United States)

    Tse, Pui-Kwan

    2011-01-01

    Introduction China's dominant position as the producer of over 95 percent of the world output of rare-earth minerals and rapid increases in the consumption of rare earths owing to the emergence of new clean-energy and defense-related technologies, combined with China's decisions to restrict exports of rare earths, have resulted in heightened concerns about the future availability of rare earths. As a result, industrial countries such as Japan, the United States, and countries of the European Union face tighter supplies and higher prices for rare earths. This paper briefly reviews China's rare-earth production, consumption, and reserves and the important policies and regulations regarding the production and trade of rare earths, including recently announced export quotas. The 15 lanthanide elements-lanthanum, cerium, praseodymium, neodymium, promethium, samarium, europium, gadolinium, terbium, dysprosium, holmium, erbium, thulium, ytterbium, and lutetium (atomic numbers 57-71)-were originally known as the rare earths from their occurrence in oxides mixtures. Recently, some researchers have included two other elements-scandium and yttrium-in their discussion of rare earths. Yttrium (atomic number 39), which lies above lanthanum in transition group III of the periodic table and has a similar 3+ ion with a noble gas core, has both atomic and ionic radii similar in size to those of terbium and dysprosium and is generally found in nature with lanthanides. Scandium (atomic number 21) has a smaller ionic radius than yttrium and the lanthanides, and its chemical behavior is intermediate between that of aluminum and the lanthanides. It is found in nature with the lanthanides and yttrium. Rare earths are used widely in high-technology and clean-energy products because they impart special properties of magnetism, luminescence, and strength. Rare earths are also used in weapon systems to obtain the same properties.

  3. Severe Hypertriglyceridemia Induced Pancreatitis in Pregnancy

    OpenAIRE

    Gupta, Natasha; Ahmed, Seema; Shaffer, Lemuel; Cavens, Paula; Blankstein, Josef

    2014-01-01

    Acute pancreatitis caused by severe gestational hypertriglyceridemia is a rare complication of pregnancy. Acute pancreatitis has been well associated with gallstone disease, alcoholism, or drug abuse but rarely seen in association with severe hypertriglyceridemia. Hypertriglyceridemia may occur in pregnancy due to normal physiological changes leading to abnormalities in lipid metabolism. We report a case of severe gestational hypertriglyceridemia that caused acute pancreatitis at full term an...

  4. Pachyonychia congenita: A rare genodermatosis

    Directory of Open Access Journals (Sweden)

    Puneet Agarwal

    2013-01-01

    Full Text Available Pachyonychia congenita (PC is a rare genodermatosis with only 450 cases reported since 1906. It is of two types, type I due to mutation in genes 6a and 16, and 6b and 17 in type II with an autosomal dominant inheritance in both types. A 22 yr old female patient presented in our OPD with hypertrophy of finger and toe nails, palmoplantar keratoderma, oral punctuate leukokeratosis, hyperhidrosis in palms and soles with maceration and malodour since childhood. She had a positive family history with father and grandfather affected but less severely. Microscopy and culture of nail clippings and scrapping were done to rule out fungal infection. On biopsy acanthotic epidermis, parakeratosis, orthokeratosis were seen. No evidence of any associated malignancy was found after thorough workup. She was diagnosed as PC Type 1. She was put on topical steroids and orally on acetretin 25 mg OD. Paring of the nails was done too reduce the thickness of nails & to provide symptomatic relief. She was on a regular treatment for 3-4 months and showed some improvement in the form of reduced palmoplantar hyperkeratosis and reduced oral punctate keratosis but was later lost on followup. She showed no adverse effect to therapy during this period. This case is being reported because of its rarity.

  5. Zebra: searching for rare diseases

    DEFF Research Database (Denmark)

    Dragusin, Radu; Petcu, Paula; Lioma, Christina

    2012-01-01

    disease diagnostic hypotheses in the domain of medical IR. In this work, we build upon an existing vertical medical search engine, Zebra, that is focused on rare disease diagnosis. In previous work, Zebra has been evaluated using real-life medical cases of rare and difficult diseases, and has been found...

  6. Rare earth impact on glass structure and alteration kinetics

    International Nuclear Information System (INIS)

    Molieres, E.

    2012-01-01

    This work is related to the question of the geological deep repository of high-level waste glass. These wastes include fission products and minor actinides, elements which can be simulated by rare earths. As new glass compositions could enable increased rare earth concentrations, it is crucial to know and understand rare earth impact on glass structure on the one hand, and on glass alteration kinetics or their incorporation into an altered layer. This work studied simplified borosilicate glasses in order to limit synergetic effects between rare earths and other elements. Various complementary techniques were used to characterize pristine and altered glasses (solid-high resolution NMR, Raman spectroscopy, fluorescence, SIMS, SAXS). Firstly, the structural role of a rare earth is discussed and is compared to a calcium cation. The local environment of rare earths is also probed. Secondly, rare earth (nature and concentration) impact on several alteration regimes was studied (initial rate, rate drop). Then, after alteration, rare earth elements being retained within the altered layer, the structural impact of rare earth elements (and their local environment) in this alteration layer was also investigated. (author) [fr

  7. Poster "Maladies rares & sciences sociales"

    OpenAIRE

    Duysens, Fanny

    2018-01-01

    Le poster "Maladies rares & sciences sociales" visait à présenter la recherche doctorale de l'auteure au grand public dans le cadre de la Journée des Maladies Rares du Centre Hospitalier Universitaire de Liège dont le thème était "la recherche sur les maladies rares". Réalisé spécialement pour l'occasion, le poster explicitait de manière synthétique le sujet de recherche, la méthodologie, certains résultats, ainsi que les apports possibles des échanges entre chercheurs en sciences et sociales...

  8. Rare metal and rare earth pegmatites of Western India

    International Nuclear Information System (INIS)

    Maithani, P.B.; Nagar, R.K.

    1999-01-01

    Rajasthan Mica Belt in western India is one of the three major mica-producing Proterozoic pegmatite belts of India, the others being in Bihar and Andhra Pradesh. The pegmatites of these mica belts, in general, are associated with the rare metal (RM) and rare earth element (REE)-bearing minerals like columbite-tantalite, beryl, lepidolite and other multiple oxides. RM-REE pegmatites of Gujarat are devoid of commercially workable mica. These pegmatites are geologically characterised in this paper, based on their association with granite plutons geochemistry, and RM and REE potential. In addition to RM and RE-bearing pegmatites, granites of the Umedpur area, Gujarat also show anomalous concentration (0.97 wt%) of rare metals (6431 ppm Nb, 1266 ppm Ta, 454 ppm Sn, 173 ppm W), (1098 ppm Ce 1.36% Y 2 O 3 ) rare earths, and uranium (0.40% eU 3 O 8 ). Eluvial concentrations in the soil and panned concentrate (0.04-0.28 wt%) analysed up to 7.4%Nb 2 O 5 , 836 ppm Ta, and 1.31% Y. Discrete columbite-tantalite and betafite have been identified in these concentrates in addition to other minerals like zircon, rutile, sphene and xenotime. This area with discrete RM R EE mineral phases could be significant as a non-pegmatite source for rare metal and rare earths. (author)

  9. Florid Cementoosseous Dysplasia: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Mehmet Fatih Şentürk

    2013-01-01

    Full Text Available Florid cementoosseous dysplasia (FCOD is a rare, benign, fibroosseous, and multifocal dysplastic lesion of the jaw that consists of cellular fibrous connective tissue with bone and cementum-like tissue. FCOD is most commonly found in middle-aged black women, is generally asymptomatic, and is usually detected during radiological examination. FCOD associated with multiple impacted teeth and bone expansion is a very rare phenomenon, and there are only a few familial cases reported in the literature. In this report, a 35-year-old male Turkish patient is presented who was diagnosed with nonfamilial FCOD from clinical, radiological, and histopathological findings. To our knowledge this is the first case of the nonfamilial FCOD with this many impacted teeth and severely expanded bones.

  10. Organizational Learning in Rare Events

    DEFF Research Database (Denmark)

    Andersen, Kristina Vaarst; Tyler, Beverly; Beukel, Karin

    When organizations encounter rare events they often find it challenging to extract learning from the experience. We analyze opportunities for organizational learning in one such rare event, namely Intellectual Property (IP) litigation, i.e., when organizations take disputes regarding their intell......When organizations encounter rare events they often find it challenging to extract learning from the experience. We analyze opportunities for organizational learning in one such rare event, namely Intellectual Property (IP) litigation, i.e., when organizations take disputes regarding...... the organization little discretion to utilize any learning from past litigation success. Thus, learning appears be to most beneficial in infringement cases. Based on statistical analysis of 10,211 litigation court cases in China, we find support for our hypotheses. Our findings suggest that organizations can learn...

  11. Economic aspects of rare diseases.

    Science.gov (United States)

    Borski, Krzysztof

    2015-01-01

    Economic problems related to the prevention, diagnosis and treatment of rare diseases are presented paying particular attention to the costs of financing treatment, including the issue of its refund, which is a fundamental and difficult to solve economic problem of the health care system. Rare diseases, despite the low frequency of occurrence, together cover a large group of diseases being a serious medical, social and economic problem. The adoption of Polish National Plan for Rare Diseases resulting from the recommendations of the Council of the European Union, the extension of institutional activities related to the area of public health and social initiatives seeking innovative solutions to create a model of social support for patients and their families, with very high complexity of the issues regarding rare diseases, results in the need for a coherent, comprehensive, system operations and adoption of comprehensive solutions.

  12. Rare earth industries: Downstream business

    International Nuclear Information System (INIS)

    2011-01-01

    The value chain of the rare earths business involves mining, extraction, processing, refining and the manufacture of an extensive range of downstream products which find wide applications in such industries including aerospace, consumer electronics, medical, military, automotive, renewable wind and solar energy and telecommunications. In fact the entire gamut of the high-tech industries depends on a sustainable supply of rare earths elements. The explosive demand in mobile phones is an excellent illustration of the massive potential that the rare earths business offers. In a matter of less than 20 years, the number of cell phones worldwide has reached a staggering 5 billion. Soon, going by the report of their growth in sales, the world demand for cell phones may even exceed the global population. Admittedly, the rare earths business does pose certain risks. Top among the risks are the health and safety risks. The mining, extraction and refining of rare earths produce residues and wastes which carry health and safety risks. The residues from the extraction and refining are radioactive, while their effluent waste streams do pose pollution risks to the receiving rivers and waterways. But, as clearly elaborated in a recent report by IAEA experts, there are technologies and systems available to efficiently mitigate such risks. The risks are Rare Earth manageable. However, it is crucial that the risk and waste management procedures are strictly followed and adhered to. This is where effective monitoring and surveillance throughout the life of all such rare earths facilities is crucial. Fortunately, Malaysia's regulatory standards on rare earths follow international standards. In some areas, Malaysia's regulatory regime is even more stringent than the international guidelines. (author)

  13. Rare earth metal alloy magnets

    International Nuclear Information System (INIS)

    Harris, I.R.; Evans, J.M.; Nyholm, P.S.

    1979-01-01

    This invention relates to rare earth metal alloy magnets and to methods for their production. The technique is based on the fact that rare earth metal alloys (for e.g. cerium or yttrium) which have been crumbled to form a powder by hydride formation and decomposition can be used for the fabrication of magnets without the disadvantages inherent in alloy particle size reduction by mechanical milling. (UK)

  14. Rare beauty and charm decays

    International Nuclear Information System (INIS)

    Blake, T.

    2016-01-01

    Rare beauty and charm decays can provide powerful probes of physics beyond the Standard Model. These proceedings summarise the latest measurements of rare beauty and charm decays from the LHCb experiment at the end of Run 1 of the LHC. Whilst the majority of the measurements are consistent with SM predictions, small differences are seen in the rate and angular distribution of b → sℓ"+ℓ"− decay processes.

  15. RARE DISEASES AND GENETIC DISCRIMINATION

    Directory of Open Access Journals (Sweden)

    Mariela Yaneva – Deliverska

    2011-04-01

    Full Text Available Rare diseases are characterised by their low prevalence (less than 1/2,000 and their heterogeneity. They affect both children and adults anywhere in the world. From the medical perspective, rare diseases are characterised by the large number and broad diversity of disorders and symptoms that vary not only from disease to disease, but also within the same disease.Main characteristics of rare diseases include:· Rare diseases are often chronic, progressive, degenerative, and often life-threatening· Rare diseases are disabling: the quality of life of patients is often compromised by the lack or loss of autonomy· High level of pain and suffering for the patient and his/ her family · No existing effective cure· There are between 6000 and 8000 rare diseases· 75% of rare diseases affect children· 30% of rare disease patients die before the age of 5· 80% of rare diseases have identified genetic origins. Other rare diseases are the result of infections (bacterial or viral, allergies and environmental causes, or are degenerative and proliferative.Beyond the diversity of the diseases, rare disease patients and their families are confronted with the same wide range of difficulties arising directly from the rarity of these pathologies. The period between the emergence of the first symptoms and the appropriate diagnosis involves unacceptable and highly risky delays, as well as wrong diagnosis leading to inaccurate treatments. Living with a rare disease has implications in all areas of life, whether school, choice of future work, leisure time with friends, or affective life. It may lead to stigmatisation, isolation, exclusion from social community, discrimination for insurance subscription (health insurance, travel insurance, mortgage, and often reduced professional opportunities.Innovative treatments are often unevenly available in the EU because of delays in price determination and/or reimbursement decision, lack of experience of the treating

  16. Bayesian analysis of rare events

    Energy Technology Data Exchange (ETDEWEB)

    Straub, Daniel, E-mail: straub@tum.de; Papaioannou, Iason; Betz, Wolfgang

    2016-06-01

    In many areas of engineering and science there is an interest in predicting the probability of rare events, in particular in applications related to safety and security. Increasingly, such predictions are made through computer models of physical systems in an uncertainty quantification framework. Additionally, with advances in IT, monitoring and sensor technology, an increasing amount of data on the performance of the systems is collected. This data can be used to reduce uncertainty, improve the probability estimates and consequently enhance the management of rare events and associated risks. Bayesian analysis is the ideal method to include the data into the probabilistic model. It ensures a consistent probabilistic treatment of uncertainty, which is central in the prediction of rare events, where extrapolation from the domain of observation is common. We present a framework for performing Bayesian updating of rare event probabilities, termed BUS. It is based on a reinterpretation of the classical rejection-sampling approach to Bayesian analysis, which enables the use of established methods for estimating probabilities of rare events. By drawing upon these methods, the framework makes use of their computational efficiency. These methods include the First-Order Reliability Method (FORM), tailored importance sampling (IS) methods and Subset Simulation (SuS). In this contribution, we briefly review these methods in the context of the BUS framework and investigate their applicability to Bayesian analysis of rare events in different settings. We find that, for some applications, FORM can be highly efficient and is surprisingly accurate, enabling Bayesian analysis of rare events with just a few model evaluations. In a general setting, BUS implemented through IS and SuS is more robust and flexible.

  17. A Rare Presentation of a Rare Disease: Pulmonary Lymphomatoid Granulomatosis

    Directory of Open Access Journals (Sweden)

    Ghulam Rehman Mohyuddin

    2012-01-01

    Full Text Available A 70-year-old female presented with a 4-week history of dry cough and wheezing. Chest radiograph showed a 10.5 cm mass-like density in the anterior mediastinum which had not been previously visualized. Computed tomography scan (CT of the chest showed a right hilar mass encasing and narrowing right upper lobe bronchus and right mainstem bronchus and secondary atelectatic changes. Biopsy was consistent with a diagnosis of lymphomatoid granulomatosis Grade 3. She responded well clinically and radiologically to therapy. Lymphomatoid granulomatosis is a rare EBV-associated disorder which is considered a lymphoproliferative disease. The most common radiographic feature is multiple lung nodules. An isolated hilar mass is an exceptionally rare presentation of this rare disease.

  18. Familial gingival fibromatosis: A rare case report

    Directory of Open Access Journals (Sweden)

    Shweta Sharma

    2012-01-01

    Full Text Available Hereditary gingival fibromatosis is a rare condition that can occur as an isolated disease or as part of a syndrome or chromosomal abnormality. In severe cases, the gingival enlargement may cover the crowns of teeth and cause severe functional and aesthetic concerns. Here, we present a case of an 8-year-old girl with severe enlargement of gums in maxilla and mandible. Both deciduous and permanent teeth were not erupted in the oral cavity at all. Mutation in the Son-of-Sevenless (SOS-1 gene has been associated with the disease. The diagnosis was made based on clinical examination and family history. Surgical removal of the hyperplastic tissue was performed under general anesthesia.

  19. A rare metastasis from a rare brain tumour

    DEFF Research Database (Denmark)

    Aabenhus, Kristine; Hahn, Christoffer Holst

    2014-01-01

    This case report presents the story of a patient with an oligodendroglioma metastasizing to the bone marrow and to lymph nodes of the neck. The patient had undergone primary brain surgery 13 years prior to the discovery of metastases and radiotherapy directed at the brain tumour two months prior........ Oligodendroglioma are rare primary brain tumours of which extraneural metastasis is even more rare. The incidence of cases like this may be increasing because of better treatment and thus longer survival of patients with oligodendroglioma....

  20. Rare earth element and rare metal inventory of central Asia

    Science.gov (United States)

    Mihalasky, Mark J.; Tucker, Robert D.; Renaud, Karine; Verstraeten, Ingrid M.

    2018-03-06

    Rare earth elements (REE), with their unique physical and chemical properties, are an essential part of modern living. REE have enabled development and manufacture of high-performance materials, processes, and electronic technologies commonly used today in computing and communications, clean energy and transportation, medical treatment and health care, glass and ceramics, aerospace and defense, and metallurgy and chemical refining. Central Asia is an emerging REE and rare metals (RM) producing region. A newly compiled inventory of REE-RM-bearing mineral occurrences and delineation of areas-of-interest indicate this region may have considerable undiscovered resources.

  1. Progeria in siblings: A rare case report

    Directory of Open Access Journals (Sweden)

    R Sowmiya

    2011-01-01

    Full Text Available Progeria, also known as Hutchinson-Gilford syndrome, is an extremely rare, severe genetic condition wherein symptoms resembling aspects of aging are manifested at an early age. It is an autosomal dominant disorder. It is not seen in siblings of affected children although there are very few case reports of progeria affecting more than one child in a family. Here we are presenting two siblings, a 14-year-old male and a 13-year-old female with features of progeria, suggesting a possible autosomal recessive inheritance.

  2. HARLEQUIN ICTHYOSIS: A RARE CASE REPORT

    Directory of Open Access Journals (Sweden)

    Pradipprava Paria

    2016-01-01

    Full Text Available Harlequin ichthyosis is the most severe form of congenital ichthyosis. It is a rare autosomal recessive disorder (1:300,000. The vast majority of affected individuals are due to mutation in the ABCA12 gene, which cause a deficiency of the epidermal lipid transporter, resulting in hyperkeratosis and abnormal barrier function of skin. Infants are very susceptible to metabolic abnormalitites and infections. They usually do not survive for very long. we report here a case of a new born with harlequin ichthyosis of consaguinious parentage who had a history of similar birth previously.

  3. Seckel syndrome: A rare case report

    Directory of Open Access Journals (Sweden)

    Rinky Sisodia

    2014-01-01

    Full Text Available Seckel syndrome (SS is a rare, autosomal recessive syndrome; characterized by severe intrauterine and postnatal growth retardation, microcephaly, mental retardation, and typical facial appearance with beaklike protrusion of the midface (bird headed. In addition to the characteristic craniofacial dysmorphism and skeletal defects, abnormalities have been described in the cardiovascular, hematopoietic, endocrine, gastrointestinal, and central nervous systems. Usually such patients have poor psychomotor development. This case report presents an 8-year-old child with SS born to parents, exposed in Bhopal gas disaster.

  4. Safety aspects in rare earths recovery

    International Nuclear Information System (INIS)

    Bhattacharya, R.

    2014-01-01

    Recovery of rare earths involves mining of beach sands, mineral separation to obtain monazite and its chemical processing to obtain rare earth composites. The composites are then subjected to further chemical treatment to obtain individual rare earths. Although the separated out rare earths are not radioactive, the process for recovery of rare earths involve both radiological as well as conventional hazards. This paper highlights the safety aspects in the mining, mineral separation and chemical processing of monazite to obtain rare earths

  5. Lectures on rare B decays

    International Nuclear Information System (INIS)

    Masiero, A.

    1989-01-01

    This paper provides a pedagogical presentation of the class of the rare charmless B decays with strange particles in the final state. These processes involve the flavour changing neutral current transition b → s and, thus, they are GIM suppresses. They constitute a specially promising ground to test the Standard Model and the possible presence of new physics beyond it. In particular I show that the radiative decays b → s + π and b → s + g are priviledged places to look for large supersymmetric enhancements. The author reviews both the present experimental and theoretical situations in this field and point out the challenges that we have to meet in the 90's to have rare B physics play a role comparable to that of rare K physics

  6. Rare KL decays at Fermilab

    International Nuclear Information System (INIS)

    Schnetzer, St.

    1997-01-01

    Recent results and the future prospects for rare K L decay at Fermilab are described. A summary of all rare decay results from E799 Phase I (the 1991 run) are presented. Three new results: K L → e + e - μ + μ - , K L → π 0 μe, and π 0 → e + e - e + e - are discussed in detail. Improvements for KTeV (the 1996-1997 run) are discussed and the expected sensitivities listed. Finally, the KAMI program for rare decays with the Main Injector (2000 and beyond) is presented with emphasis on a search for the decay K L → π 0 νν-bar at O(10 -12 ) single-event-sensitivity. (author)

  7. A rare cause of hemoptysis

    Directory of Open Access Journals (Sweden)

    Meghan Aversa

    2014-01-01

    Full Text Available Angiosarcomas are rare, malignant, endothelial-cell tumors of vascular origin that can arise at any body site. They frequently metastasize to the lung, heralded by dyspnea, hemoptysis, chest pain, pneumothoraces, and diffuse pulmonary hemorrhage. However, in most cases lung metastases are discovered after the diagnosis of a primary angiosarcoma has already been established. Very rarely will an undiagnosed metastatic angiosarcoma present as diffuse pulmonary hemorrhage. We describe the case of a 59-year-old male who presented to hospital with dyspnea and hemoptysis. CT chest revealed rapidly progressing nodular changes and broncho-alveolar lavage returns were progressively bloody. Open lung wedge biopsy ultimately revealed metastatic angiosarcoma and extensive pulmonary hemorrhage. Our case highlights the key clinical, radiological, and pathological features of this rare malignancy that frequently metastasizes to the lung and reminds clinicians to consider it as a cause of hemoptysis and pulmonary hemorrhage.

  8. Ainhum - A Rare Case Report.

    Science.gov (United States)

    Prabhu, Ravi; Kannan, Narayanasamy Subbaraju; Vinoth, Sundaresan; Praveen, Chinnappan Balasubramanian

    2016-04-01

    The term 'AINHUM' is derived from the African word meaning 'to saw or cut'. True ainhum otherwise called dactylolysis spontanea is a condition involving soft tissue or digits with constricting rings commonly presenting in fifth toes, usually bilateral. It is to be differentiated from Pseudo-ainhum that occurs secondary to some hereditary and nonhereditary diseases that lead to annular constriction of digits. We report a rare case of true ainhum involving the left fourth toe only. It is a very rare case and a very few were reported worldwide. The highest incidence of ainhum has been reported in South Africa and South America. It is rarely reported in India. Ainhum when diagnosed and treated in early stages can be prevented from progressing to mutilating deformities.

  9. Rare and semi-rare decays at ATLAS

    CERN Document Server

    AUTHOR|(INSPIRE)INSPIRE-00213194; The ATLAS collaboration

    2016-01-01

    The measurements of the rare $B^0$-mesons decays processes performed by the ATLAS experiment at LHC are reviewed. Particular attention will be given to the measurement of the branching ratio of the $B^0_s$ and $B^0_d$ mesons decays into a pair of muons with the full Run 1 dataset corresponding to an integrated luminosity of 25 $\\rm{fb^{-1}}$.

  10. Dioctophymiasis: A Rare Case Report.

    Science.gov (United States)

    Chauhan, Sapna; Kaval, Sunil; Tewari, Swati

    2016-02-01

    Dioctophyma renale commonly known as "giant kidney worm' is found in the kidney of carnivorous mammals. Human infestation is rare, but results in destruction of the kidneys. Very few cases have been reported worldwide. We are here reporting a case of Dioctophymiasis in a 35-year-old male patient who presented with retention of urine and subsequent passage of worm and blood in urine. The worm was confirmed as Dioctophyma renale based on its morphology and clinical presentation. This is a very rare case report and to best of our knowledge only two cases have been reported from India.

  11. Rare B decays, rare τ decays, and grand unification

    International Nuclear Information System (INIS)

    Sher, M.; Yuan, Y.

    1991-01-01

    In multi-Higgs-boson extensions of the standard model, tree-level flavor-changing neutral currents exist naturally, unless suppressed by some symmetry. For a given rate, the exchanged scalar or pseudoscalar mass is very sensitive to the flavor-changing coupling between the first two generations. Since the Yukawa couplings of the first two generations are unknown and certainly very small, bounds which rely on some assumed value of this flavor-changing coupling are quite dubious. One might expect the size (and reliability) of the Yukawa couplings involving the third generation to be greater. In this paper, we consider processes involving τ's and B's, and determine the bounds on the flavor-changing couplings which involve third-generation fields. The strongest bound in the quark sector comes from B-bar B mixing and in the lepton sector, surprisingly, from μ→eγ. It is then noted that the flavor-changing couplings in the quark sector are related to those in the lepton sector in many grand unified theories, and one can ask whether an analysis of rare τ decays or rare B decays will provide the strongest constraints. We show that rare B decays provide the strongest bounds, and that no useful information can be obtained from rare τ decays. It is also noted that the most promising decay modes are B→Kμτ and B s →μτ, and we urge experimenters to look for rare decay modes of the B in which a τ is in the final state

  12. Rare event techniques applied in the Rasmussen study

    International Nuclear Information System (INIS)

    Vesely, W.E.

    1977-01-01

    The Rasmussen Study estimated public risks from commercial nuclear power plant accidents, and therefore the statistics of rare events had to be treated. Two types of rare events were specifically handled, those rare events which were probabilistically rare events and those which were statistically rare events. Four techniques were used to estimate probabilities of rare events. These techniques were aggregating data samples, discretizing ''continuous'' events, extrapolating from minor to catastrophic severities, and decomposing events using event trees and fault trees. In aggregating or combining data the goal was to enlarge the data sample so that the rare event was no longer rare, i.e., so that the enlarged data sample contained one or more occurrences of the event of interest. This aggregation gave rise to random variable treatments of failure rates, occurrence frequencies, and other characteristics estimated from data. This random variable treatment can be interpreted as being comparable to an empirical Bayes technique or a Bayesian technique. In the discretizing event technique, events of a detailed nature were grouped together into a grosser event for purposes of analysis as well as for data collection. The treatment of data characteristics as random variables helped to account for the uncertainties arising from this discretizing. In the severity extrapolation technique a severity variable was associated with each event occurrence for the purpose of predicting probabilities of catastrophic occurrences. Tail behaviors of distributions therefore needed to be considered. Finally, event trees and fault trees were used to express accident occurrences and system failures in terms of more basic events for which data existed. Common mode failures and general dependencies therefore needed to be treated. 2 figures

  13. Rare earths as a future resource

    International Nuclear Information System (INIS)

    Cornell, D.H.

    1988-01-01

    The fourteen rare earth or lanthanide elements have recently emerged as an important natural resource because of the rapidly growing demand in the electronic, chemical and metallurgical industries. The Symposium on rare earth elements as a future resource presented a multidisciplinary review of rare earth chemistry, geology, beneficiation, industrial applications and marketing. Papers by experts in many fields were presented on the following topics: chemical properties of the rare earth elements; the analysis of rare earth elements and minerals; beneficiation and extraction of rare earth elements; economic geochemistry and mineralogy of rare earths; present industrial uses of rare earth elements; the role of rare earth elements in high-temperature superconductors; the technical application of high-temperature superconductors; supply and demand for rare earth products - now and in the future, and the geology of rare earth deposits

  14. Assessing Rare Metal Availability Challenges for Solar Energy Technologies

    Directory of Open Access Journals (Sweden)

    Leena Grandell

    2015-08-01

    Full Text Available Solar energy is commonly seen as a future energy source with significant potential. Ruthenium, gallium, indium and several other rare elements are common and vital components of many solar energy technologies, including dye-sensitized solar cells, CIGS cells and various artificial photosynthesis approaches. This study surveys solar energy technologies and their reliance on rare metals such as indium, gallium, and ruthenium. Several of these rare materials do not occur as primary ores, and are found as byproducts associated with primary base metal ores. This will have an impact on future production trends and the availability for various applications. In addition, the geological reserves of many vital metals are scarce and severely limit the potential of certain solar energy technologies. It is the conclusion of this study that certain solar energy concepts are unrealistic in terms of achieving TW scales.

  15. Rare pion and kaon decays

    International Nuclear Information System (INIS)

    Bryman, D.

    1983-09-01

    Some rare pion and kaon decays, which provide clues to the generation puzzle, are discussed. The π→ eν/π→μ/ν branching ratio test of universality and the status of searches for K + → π + rho anti rho are reviewed

  16. Cardiofaciocutaneous syndrome: A rare entity

    Directory of Open Access Journals (Sweden)

    S Pavithra

    2012-01-01

    Full Text Available The cardiofaciocutaneous (CFC syndrome is a condition of sporadic occurrence, with patients showing multiple congenital anomalies and mental retardation and characteristic dysmorphic features. We, thus, report a rare case of this syndrome in a 1-year-old child who presented with typical features of CFC syndrome.

  17. Rare Animal Education Usingaugmented Reality

    Directory of Open Access Journals (Sweden)

    Hening Artdias

    2018-01-01

    They are extinction because destruction of forest habitats, a conflict between humans and animals, trade, hunting, the arrests beyond capacity. [1]. Is that the issue of the extinction of the animals is dominated by human behavior and nature of the wrath of them. For that, education game “Rare Animal” become formulations to raise awareness of endangered species.

  18. Rare localisations of osteochondrosis juvenilis

    Energy Technology Data Exchange (ETDEWEB)

    Schumacher, R; Schuster, W; Mueller, U

    1981-04-01

    A survey of rare localisations of osteochondrosis juvenilis is given and illustrated by cases of Morbus Friedrich, Morbus Panner, Vertebra plana Calve, Morbus Blount, Morbus Iselin, Morbus Hegemann and Morbus Thiemann. This is followed by discussion of etiology and differential diagnosis.

  19. Herpes zoster oticus: A rare clinical entity

    Directory of Open Access Journals (Sweden)

    Shailesh Gondivkar

    2010-01-01

    Full Text Available Herpes zoster oticus also known as Ramsay Hunt syndrome is a rare complication of herpes zoster in which reactivation of latent varicella zoster virus infection in the geniculate ganglion causes otalgia, auricular vesicles, and peripheral facial paralysis. Ramsay Hunt syndrome is rare in children and affects both sexes equally. Incidence and clinical severity increases when host immunity is compromised. Because these symptoms do not always present at the onset, this syndrome can be misdiagnosed. Although secondary to Bell′s palsy in terms of the cause of acute atraumatic peripheral facial paralysis, Ramsay Hunt syndrome, with incidence ranged from 0.3 to 18%, has a worse prognosis. Herpes zoster oticus accounts for about 12% cases of facial palsy, which is usually unilateral and complete and full recovery occurs in only about 20% of untreated patients. The most advisable method to treat Ramsay Hunt syndrome is the combination therapy with acyclovir and prednisone but still not promising, and several prerequisites are required for better results. We present a case of 32-year-old man suffering from Ramsay Hunt syndrome with grade V facial palsy treated effectively with rehabilitation program, after the termination of the combination therapy of acyclovir and prednisone.

  20. Adapting Knowledge Translation Strategies for Rare Rheumatic Diseases.

    Science.gov (United States)

    Cellucci, Tania; Lee, Shirley; Webster, Fiona

    2016-08-01

    Rare rheumatic diseases present unique challenges to knowledge translation (KT) researchers. There is often an urgent need to transfer knowledge from research findings into clinical practice to facilitate earlier diagnosis and better outcomes. However, existing KT frameworks have not addressed the specific considerations surrounding rare diseases for which gold standard evidence is not available. Several widely adopted models provide guidance for processes and problems associated with KT. However, they do not address issues surrounding creation or synthesis of knowledge for rare diseases. Additional problems relate to lack of awareness or experience in intended knowledge users, low motivation, and potential barriers to changing practice or policy. Strategies to address the challenges of KT for rare rheumatic diseases include considering different levels of evidence available, linking knowledge creation and transfer directly, incorporating patient and physician advocacy efforts to generate awareness of conditions, and selecting strategies to address barriers to practice or policy change.

  1. Separation of rare earths by liquid-liquid extraction

    International Nuclear Information System (INIS)

    Helgorsky, M.; Leveque, M.

    1978-01-01

    The elements of the rare earth family are characterised by very similar chemical properties connected with their special electronic structure. The purification of the rare earths sold by RHONE-POULENC is now done by the liquid-liquid extraction technique. The development of different extracting agents and also counter-current techniques have led to solvent extraction replacing the other fractionation techniques because of its efficiency and low cost. There are usually several possible solutions to the main problem of choosing the extracting agent and its mode of use. The difficulty is to find the most economical one taking account of the thermodynamic and hydrodynamic constraints of the solvent. It is shown how ideas about the separation have changed over the course of the development of the uses of the rare earths, ending finally in an integrated scheme that makes RHONE-POULENC a world leader of manufacturers of separated rare earths [fr

  2. Rare and semi-rare decays at ATLAS

    CERN Document Server

    AUTHOR|(INSPIRE)INSPIRE-00213194; The ATLAS collaboration

    2016-01-01

    The measurements of the rare $B^0$-meson-decay processes performed by the ATLAS experiment at the LHC are reviewed. Particular attention will be given to the measurement of the branching ratio of the $B^0_s$ and $B^0_d$ mesons decays into a pair of muons with the full Run 1 dataset corresponding to an integrated luminosity of 25 $\\rm{fb^{-1}}$.

  3. Twenty-First Century Diseases: Commonly Rare and Rarely Common?

    Science.gov (United States)

    Daunert, Sylvia; Sittampalam, Gurusingham Sitta; Goldschmidt-Clermont, Pascal J

    2017-09-20

    Alzheimer's drugs are failing at a rate of 99.6%, and success rate for drugs designed to help patients with this form of dementia is 47 times less than for drugs designed to help patients with cancers ( www.scientificamerican.com/article/why-alzheimer-s-drugs-keep-failing/2014 ). How can it be so difficult to produce a valuable drug for Alzheimer's disease? Each human has a unique genetic and epigenetic makeup, thus endowing individuals with a highly unique complement of genes, polymorphisms, mutations, RNAs, proteins, lipids, and complex sugars, resulting in distinct genome, proteome, metabolome, and also microbiome identity. This editorial is taking into account the uniqueness of each individual and surrounding environment, and stresses the point that a more accurate definition of a "common" disorder could be simply the amalgamation of a myriad of "rare" diseases. These rare diseases are being grouped together because they share a rather constant complement of common features and, indeed, generally respond to empirically developed treatments, leading to a positive outcome consistently. We make the case that it is highly unlikely that such treatments, despite their statistical success measured with large cohorts using standardized clinical research, will be effective on all patients until we increase the depth and fidelity of our understanding of the individual "rare" diseases that are grouped together in the "buckets" of common illnesses. Antioxid. Redox Signal. 27, 511-516.

  4. Rare events: a state of the art

    International Nuclear Information System (INIS)

    Uppuluri, V.R.R.

    1980-12-01

    The study of rare events has become increasingly important in the context of nuclear safety. Some philosophical considerations, such as the framework for the definition of a rare event, rare events and science, rare events and trans-science, and rare events and public perception, are discussed. The technical work of the Task Force on problems of Rare Events in the Reliability Analysis of Nuclear Plants (1976-1978), sponsored by OECD, is reviewed. Some recent technical considerations are discussed, and conclusions are drawn. The appendix contains an essay written by Anne E. Beachey, under the title: A Study of Rare Events - Problems and Promises

  5. Rare Earth Garnet Selective Emitter

    Science.gov (United States)

    Lowe, Roland A.; Chubb, Donald L.; Farmer, Serene C.; Good, Brian S.

    1994-01-01

    Thin film Ho-YAG and Er-YAG emitters with a platinum substrate exhibit high spectral emittance in the emission band (epsilon(sub lambda) approx. = 0.75, sup 4)|(sub 15/2) - (sup 4)|(sub 13/2),for Er-YAG and epsilon(sub lambda) approx. = 0.65, (sup 5)|(sub 7) - (sup 5)|(sub 8) for Ho-YAG) at 1500 K. In addition, low out-of-band spectral emittance, epsilon(sub lambda) less than 0.2, suggest these materials would be excellent candidates for high efficiency selective emitters in thermophotovoltaic (TPV) systems operating at moderate temperatures (1200-1500 K). Spectral emittance measurements of the thin films were made (1.2 less than lambda less than 3.0 microns) and compared to the theoretical emittances calculated using measured values of the spectral extinction coefficient. In this paper we present the results for a new class of rare earth ion selective emitters. These emitters are thin sections (less than 1 mm) of yttrium aluminum garnet (YAG) single crystal with a rare earth substitutional impurity. Selective emitters in the near IR are of special interest for thermophotovoltaic (TPV) energy conversion. The most promising solid selective emitters for use in a TPV system are rare earth oxides. Early spectral emittance work on rare earth oxides showed strong emission bands in the infrared (0.9 - 3 microns). However, the emittance outside the emission band was also significant and the efficiency of these emitters was low. Recent improvements in efficiency have been made with emitters fabricated from fine (5 - 10 microns) rare earth oxide fibers similar to the Welsbach mantle used in gas lanterns. However, the rare earth garnet emitters are more rugged than the mantle type emitters. A thin film selective emitter on a low emissivity substrate such as gold, platinum etc., is rugged and easily adapted to a wide variety of thermal sources. The garnet structure and its many subgroups have been successfully used as hosts for rare earth ions, introduced as substitutional

  6. Testicular calculus: A rare case.

    Science.gov (United States)

    Sen, Volkan; Bozkurt, Ozan; Demır, Omer; Tuna, Burcin; Yorukoglu, Kutsal; Esen, Adil

    2015-01-01

    Testicular calculus is an extremely rare case with unknown etiology and pathogenesis. To our knowledge, here we report the third case of testicular calculus. A 31-year-old man was admitted to our clinic with painful solid mass in left testis. After diagnostic work-up for a possible testicular tumour, he underwent inguinal orchiectomy and histopathologic examination showed a testicular calculus. Case hypothesis: Solid testicular lesions in young adults generally correspond to testicular cancer. Differential diagnosis should be done carefully. Future implications: In young adults with painful and solid testicular mass with hyperechogenic appearance on scrotal ultrasonography, testicular calculus must be kept in mind in differential diagnosis. Further reports on this topic may let us do more clear recommendations about the etiology and treatment of this rare disease.

  7. Chondroectodermal dysplasia: a rare syndrome.

    Directory of Open Access Journals (Sweden)

    Dana Tahririan

    2014-06-01

    Full Text Available Chondroectodermal dysplasia (Ellis-Van Creveld syndrome is a rare autosomal recessive congenital abnormality. This syndrome is characterized by a spectrum of clinical findings, among which chondrodystrophy, polydactyly, ectodermal dysplasia, and congenital cardiac anomalies are the most common. It is imperative to not overlook the cardiac complications in patients with this syndrome during dental procedures. The case presented here, although quite rare, was detected under normal conditions and can be alarming for dental care providers. Clinical reports outline the classical and unusual oral and dental manifestations, which help health care providers diagnose chondroectodermal dysplasia, and refer patients with this syndrome to appropriate health care professionals to receive treatment to prevent further cardiac complications and bone deformities.

  8. Rare earth industries: Upstream business

    International Nuclear Information System (INIS)

    2011-01-01

    Evidently, many factors contribute to the rush to invest in the unprecedented revival of rare earths. One major reason has to do with the rapidly growing world demand. The other reason relates to the attractive price of rare earths which is projected to stay strong in the coming years. This is because supply is predicted to have difficulty keeping pace with demand. Experts believe a major driver of global rare earths demand is the forecasted expansion in the green economy. Climate change is a major driver of the green economy. With climate change, there is concern that the uncontrolled emission of the greenhouse gases, especially carbon dioxide, can lead to catastrophic consequences for the world. This has been documented in countless studies and reports. Another important driver of the green economy is the growing shortfall in many resources. The world is now experiencing declines in key resources to meet a growing global demand. With more than 6 billion people now in the world and growing, the pressure exerted on global resources including energy, water and food is a major concern. Recent demand surge in China and India has dented the supply position of major world resources. The much quoted Stern Report from the UK has warned that, unless immediate steps are taken to reduce greenhouse gas emissions, it may be a costly exercise to undertake the corrections later. Since energy use, especially fossil fuels, is a major contributor to climate change, greener options are being sought. Add to that the fact that the fossil energy resources of the world are declining, the need to seek alternatives becomes even more urgent. One option is to change to renewable energy sources. These include such potentials as solar, wind and biomass. Rare earths have somehow become a critical feature of the technologies in such renewable. Another option is to improve the efficient use of energy in transport, buildings and all the other energy intensive industries. Again the technologies in

  9. Rare gases in Samoan xenoliths

    Science.gov (United States)

    Poreda, R. J.; Farley, K. A.

    1992-09-01

    The rare gas isotopic compositions of residual harzburgite xenoliths from Savai'i (SAV locality) and an unnamed seamount south of the Samoan chain (PPT locality) provide important constraints on the rare gas evolution of the mantle and atmosphere. Despite heterogeneous trace element compositions, the rare gas characteristics of the xenoliths from each of the two localities are strikingly similar. SAV and PPT xenoliths have 3He/ 4He ratios of11.1 ± 0.5 R A and21.6 ± 1 R A, respectively; this range is comparable to the 3He/ 4He ratios in Samoan lavas and clearly demonstrates that they have trapped gases from a relatively undegassed reservoir. The neon results are not consistent with mixing between MORB and a plume source with an atmospheric signature. Rather, the neon isotopes reflect either a variably degassed mantle (with a relative order of degassing of Loihi Honda et al. that the 20Ne/ 22Ne ratio in the mantle more closely resembles the solar ratio than the atmospheric one. 40Ar/ 36Ar ratios in the least contaminated samples range from 4,000 to 12,000 with the highest values in the 22 RA PPT xenoliths. There is no evidence for atmospheric 40Ar/ 36Ar ratios in the mantle source of these samples, which indicates that the lower mantle may have 40Ar/ 36Ar ratios in excess of 5,000. Xenon isotopic anomalies in 129Xe and 136Xe are as high as 6%, or about half of the maximum MORB excess and are consistent with the less degassed nature of the Samoan mantle source. These results contradict previous suggestions that the high 3He/ 4He mantle has a near-atmospheric heavy rare gas isotopic composition.

  10. Os Odontoideum: Rare Cervical Lesion

    Directory of Open Access Journals (Sweden)

    Kristie A Robson

    2011-05-01

    Full Text Available We report the case of a 22-year-old Marine who presented to the emergency department, after a martial arts exercise, with transient weakness and numbness in all extremities. Computed tomography cervical spine radiographs revealed os odontoideum. Lateral flexion–extension radiographs identified atlanto-axillary instability. This abnormality is rare and can be career ending for military members who do not undergo surgical fusion. [West J Emerg Med. 2011;12(4:520–522.

  11. Carcinoma caecum - a rare presentation

    International Nuclear Information System (INIS)

    Ali, T.; Mudasser, S.; Mudsser, S.; Sarwar, M.

    2010-01-01

    Caecal and right sided colonic cancers mostly present with fatigue, weakness and iron deficiency anemia. Such tumours rarely perforate posteriorly and involve the retroperitoneum. We report a case of an old Omani lady who came with insidious sign and symptoms of perforated caecum leading to retroperitoneal collection and necrotizing fasciitis of abdominal wall due to carcinoma of caecum. She underwent surgery but despite active intervention, she died because of septicemic shock. (author)

  12. Intracranial chondroma: a rare entity.

    Science.gov (United States)

    Maheshwari, Veena; Mehdi, Ghazala; Varshney, Manoranjan; Jain, Anshu; Vashishtha, Sonal; Gaur, Kavita; Srivastava, Vinod Kumar

    2011-05-12

    Intracranial chondroma is a rare benign cartilaginous tumour with an incidence of less than 1% of all primary intracranial tumours. The authors are reporting here a case of intracranial chondroma in a 40-year-old man who presented with 5-month history of headache and gradual diminution of vision. A tentative diagnosis of chondroma was made on imprint cytology which was confirmed on histopathological examination.

  13. Macrodystrophia lipomatosa: a rare presentation

    Directory of Open Access Journals (Sweden)

    Sushil G. Kachewar

    2011-07-01

    Full Text Available Macrodystrophia lipomatosa is a rare congenital nonhereditary mesenchymal hamartomatous malformation resulting in localized gigantism of parts of extremities that manifests clinically as macrodactyly or megalodactyly. Radiological and Pathological hallmark is the disproportionate fibroadipose tissue proliferation in subcutaneous tissue, nerve sheaths, and periosteum that leads to soft tissue and bony enlargement. As imaging is the key to early diagnosis, findings on various modalities like plain radiographs, CT scan and MRI are highlighted here.

  14. Genetic analysis of rare disorders

    DEFF Research Database (Denmark)

    van den Berg, Stéphanie M; von Bornemann Hjelmborg, Jacob

    2012-01-01

    Twin concordance rates provide insight into the possibility of a genetic background for a disease. These concordance rates are usually estimated within a frequentistic framework. Here we take a Bayesian approach. For rare diseases, estimation methods based on asymptotic theory cannot be applied due....... The Bayesian method is able to include prior information on both concordance rates and prevalence rates at the same time and is illustrated using twin data on cleft lip and rheumatoid arthritis....

  15. Rare Earth Oxide Thin Films

    CERN Document Server

    Fanciulli, Marco

    2007-01-01

    Thin rare earth (RE) oxide films are emerging materials for microelectronic, nanoelectronic, and spintronic applications. The state-of-the-art of thin film deposition techniques as well as the structural, physical, chemical, and electrical properties of thin RE oxide films and of their interface with semiconducting substrates are discussed. The aim is to identify proper methodologies for the development of RE oxides thin films and to evaluate their effectiveness as innovative materials in different applications.

  16. Proceedings of the international conference on science, technology and applications of rare earths

    International Nuclear Information System (INIS)

    2015-01-01

    Rare Earth Elements (REEs) are extensively used in clean energy applications like wind turbines, hybrid car batteries/electric motors, solar energy collectors, permanent magnets, phosphors, multifunctional pigments, thin film technologies, defence - related systems, etc. The use of rare earth elements in modern technology has increased several folds over the past few years in both domestic and international sectors due to the growing economy. The current global demand for rare earths is expected to provide a myriad of business opportunities for rare earth industries across the world including India for the utilization of rare earths in green energy, technology and industry. Papers relevant to INIS are indexed separately

  17. Rare earth niobate coordination polymers

    Science.gov (United States)

    Muniz, Collin N.; Patel, Hiral; Fast, Dylan B.; Rohwer, Lauren E. S.; Reinheimer, Eric W.; Dolgos, Michelle; Graham, Matt W.; Nyman, May

    2018-03-01

    Rare-earth (RE) coordination polymers are infinitely tailorable to yield luminescent materials for various applications. Here we described the synthesis of a heterometallic rare-earth coordination compound ((CH3)2SO)3(RE)NbO(C2O4)3((CH3)2SO) = dimethylsulfoxide, DMSO, (C2O2= oxalate), (RE=La, Ce, Pr, Nd, Sm, Eu, Gd, Tb). The structure was obtained from single crystal X-ray diffraction of the La analogue. The Nb˭O and DMSO terminal-bonding character guides assembly of an open framework structure with noncentrosymmetric RE-coordination geometry, and large spacing between the RE centers. A second structure was observed by PXRD for the smaller rare earths (Dy, Ho, Er, Yb); this structure has not yet been determined. The materials were further characterized using FTIR, and photoluminescence measurements. Characteristic excitation and emission transitions were observed for RE = Nd, Sm, Eu, and Tb. Quantum yield (QY) measurements were performed by exciting Eu and Tb analoges at 394 nm (QY 66%) and 464 nm (QY 71%) for Eu; and 370 nm (QY=40%) for Tb. We attribute the high QY and bright luminescence to two main structure-function properties of the system; namely the absence of water in the structure, and absence of concentration quenching.

  18. Rare earth industry in India

    International Nuclear Information System (INIS)

    Singh, D.S.

    2016-01-01

    Rare Earths (RE) comprises of 17 elements i.e. elements from atomic No. 57-71 (lanthanide series) along with yttrium (atomic No. 39) and scandium (atomic No. 21). They exhibit special electronic, magnetic, optical and catalytic properties. The first 7 elements in the lanthanide series from atomic Nos. 57 to 63 (La to Eu) are called Light Rare Earths (LRE), while the remaining elements from atomic Nos. 64 to 71 (Gd to Lu) are grouped as Heavy Rare Earths (HRE). Scandium and Yttrium have properties similar to HRE. The concentration of the REs in the earth's crust is as high as some other elements including that of copper. The only difference is that REs do not occur as separate minerals amenable for easy exploration and mining and are widely distributed across the earth's surface, hence they are called as REs. Resources In India, monazite has been the principal source of RE. It occurs in association with other heavy minerals, such as ilmenite, rutile, zircon etc. in the beach sands and inland placer deposits. The monazite content in this assemblage varies from negligible quantity to as high as 5%. As per AMD resource estimation, the reported resource of monazite in India is about 11.93 million tons which corresponds with about 6.9 million tons of RE oxides. Although India possesses large deposits of monazite, the heavier RE are not present in sufficient quantities in this mineral. (author)

  19. Rare earths: occurrence, production and applications

    International Nuclear Information System (INIS)

    Murthy, T.K.S.; Mukherjee, T.K.

    2002-01-01

    The mining and processing of rare earth minerals, particularly of monazite, began in a modest way in 1880s for commercialized production of mantle for gas lighting. For all major applications up to mid-twentieth century- production of lighter flints, misch metal as a metallurgical alloying agent, colouring, decolourizing and polishing agents for glass, petroleum cracking catalysts and arc-carbons, unseparated or partially separated rare earths were adequate. These applications continue till today. With the development and industrial application of powerful techniques like ion exchange and solvent extraction for the separation of rare earths, the decades after 1960 saw increasing utilization of the specific properties of the individual rare earths. Some of these advanced technological applications include: special glass for optical systems including camera lenses, phosphors for colour television, cathode ray tubes and fluorescent lighting, X-ray intensification screens, high intensity permanent magnets, electro optical devices, lasers, hydrogen storage materials, hydride rechargeable batteries, photomagnetic data storage systems, autoexhaust catalysts, special ceramics of unusual toughness, artificial diamonds and nonpoisonous plastic colorants. The topics covered in the book include rare earths: their story identity, rare earth resources, processing of ores and recovery of mixed rare earths products, separation and purification of rare earths, nonmetallic applications of rare earths, rare earth metals: production and applications, rare earth alloys and their applications, analysis of rare earth, processing of rare earth resources in India by Indian Rare Earth Ltd. and availability and market conditions

  20. A Rare Presentation of Cyclitis Induced Myopia.

    Science.gov (United States)

    Ijaz, Umar; Habib, Asad; Rathore, Hassan Sajjad

    2018-03-01

    Unilateral cyclitis leading to myopia is a rare and clinical relevant entity. In clinical settings, pseudomyopia is generally encountered in the form of accommodative spasm, which is always bilateral. Cyclitis due to inflammation, on the other hand, can cause pseudomyopia unilaterally and it is a very rare presentation. A young male with acute anterior uveitis, presented with acute episode of unilateral myopia. When patient was examined on first visit, there were no cells in anterior chamber; so he was started on cycloplegic eye drops, but his condition didn't improve. Examination on subsequent visit revealed cellular reaction in anterior chamber and narrowing of anterior chamber angles on anterior segment optical coherence tomography (OCT). Treatment for uveitis was started and patient's visual acuity and refractive error improved. Pseudomyopia is a known complication of several drugs and certain medical conditions. The possible mechanism is supraciliary exudation causing relaxation of zonular fibers and increased convexity of the crystalline lens. Myopia in the setting of a mild cellular reaction can easily be missed and has not been reported yet to the best of authors' literature search.

  1. Alkaptonuria: a very rare metabolic disorder.

    Science.gov (United States)

    Aquaron, Robert

    2013-10-01

    Alkaptonuria (AKU) is a very rare autosomal recessive disorder of tyrosine metabolism in the liver due to deficiency of homogentisate 1,2 dioxygenase (HGD) activity, resulting in the accumulation of homogentisic acid (HGA). Circulating HGA pass into various tissues through-out the body, mainly in cartilage and connective tissues, where its oxidation products polymerize and deposit as a melanin-like pigment. Gram quantities of HGA are excreted in the urine. AKU is a progressive disease and the three main features, according the chronology of appearance, are: darkening of the urine at birth, then ochronosis (blue-dark pigmentation of the connective tissue) clinically visible at around 30 yrs in the ear and eye, and finally a severe ochronotic arthropathy at around 50 yrs with spine and large joints involvements. Cardiovascular and renal complications have been described in numerous case report studies. A treatment now is available in the form of a drug nitisinone, which decreases the production of HGA. The enzymatic defect in AKU is caused by the homozygous or compound heterozygous mutations within the HGD gene. This disease has a very low prevalence (1:100,000-250,000) in most of the ethnic groups, except Slovakia and Dominican Republic, where the incidence has shown increase up to 1:19,000. This review highlights classical and recent findings on this very rare disease.

  2. A Rare Complication of Hyperplastic Gastric Polyp

    Directory of Open Access Journals (Sweden)

    Suresh Kumar Nayudu

    2013-01-01

    Full Text Available Hyperplastic gastric polyps are incidentally diagnosed during upper gastrointestinal endoscopy. They are known to cause gastric outlet obstruction and chronic blood loss leading to iron deficiency anemia. However, hyperplastic gastric polyp presenting as acute severe upper gastrointestinal bleeding is very rare. To the best of our knowledge, there have been two cases of hyperplastic gastric polyps presenting as acute gastrointestinal bleeding in the medical literature. We present a case of a 56-year-old African American woman who was admitted to our hospital with symptomatic anemia and sepsis. The patient developed acute upper gastrointestinal bleeding during her hospital stay. She underwent emergent endoscopy, but bleeding could not be controlled. She underwent emergent laparotomy and wedge resection to control the bleeding. Biopsy of surgical specimen was reported as hyperplastic gastric polyp. We recommend that physicians should be aware of this rare serious complication of hyperplastic gastric polyps as endoscopic polypectomy has diagnostic and therapeutic benefits in preventing future complications including bleeding.

  3. Hyponatremia in a Teenager: A Rare Diagnosis.

    Science.gov (United States)

    Correia, Filipa; Fernandes, Alexandre; Mota, Teresa C; Garcia, Milagros; Castro-Correia, Cíntia; Fontoura, Manuel; Ribeiro, Augusto

    2015-12-01

    Hyponatremia is a common electrolyte alteration which has the potential for significant morbidity and mortality. Endocrine disorders, such as primary hypothyroidism and adrenal insufficiency are uncommon causes of hyponatremia. We present the case of a teenager with symptomatic hyponatremia caused by a rare disorder. A 17-year-old boy was admitted to the emergency department with abdominal pain, nausea and vomiting, asthenia, and weight loss. He was in poor general condition, hypotensive, and he had dry mucous membranes and skin as well as mucosa hyperpigmentation. The laboratory findings showed severe hyponatremia, hyperkalemia, and renal dysfunction. The patient started inotropic support and antibiotics. Plasma cortisol and corticotropin levels allowed the diagnosis of primary adrenal insufficiency. He began replacement therapy with hydrocortisone and fludrocortisone, with gradual symptom resolution. An abdominal computed tomography scan showed adrenal hypoplasia. Findings for antiadrenal and antithyroid antibodies were positive, allowing the diagnosis of autoimmune polyglandular syndrome type II. Adrenal insufficiency is a rare disease, especially in children, and its clinical manifestations are due to glucocorticoid and mineralocorticoid deficiency. In most of the cases, symptoms are nonspecific, requiring a high index of clinical suspicion. If the diagnosis and treatment are delayed, acute adrenal insufficiency carries a high morbidity and mortality.

  4. [Nephroblastoma and xeroderma pigmentosum: A rare association].

    Science.gov (United States)

    Lahlimi, F; Harif, M; Elhoudzi, J

    2016-01-01

    Xeroderma pigmentosum (XP) is a rare, genetically heterogeneous, autosomal recessive disorder, more common in cases of consanguinity. The basic defect underlying the clinical manifestations is a nucleotide excision repair defect leading to the defective repair of DNA damaged by ultraviolet (UV) radiation. XP is characterized by a high incidence of skin cancer on exposed regions. We report the case of a 5-year-old boy, followed for xeroderma pigmentosum since the age of 4 years. His sister also has the same anomaly. He presented an abdominal mass revealed by abdominal pain and vomiting. Radiological examinations revealed a nephroblastoma with lung metastases. He received primary chemotherapy for six cycles (vincristine, and actinomycin-d adriamycin), then surgery with ureteronephrectomy. Pathological examination of the nephrectomy specimen confirmed the diagnosis of Wilms tumor with a diffuse anaplastic component reaching 50%. The patient was treated according to the GFAOP stage III protocol, with high histological risk. The outcome was favorable but complicated by renal failure due to the toxicity of the treatment. He is currently in complete remission at 1 year from the end of treatment. The association of xeroderma pigmentosum and nephroblastoma is a rare combination. This case illustrates the problem of management of both severe and difficult conditions. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  5. The European Union Policy in the Field of Rare Diseases.

    Science.gov (United States)

    Moliner, Antoni Montserrat; Waligora, Jaroslaw

    2017-01-01

    Rare diseases, are defined by the European Union as life-threatening or chronically debilitating diseases with low prevalence (less than 5 per 10,000). The specificities of rare diseases - limited number of patients and scarcity of relevant knowledge and expertise - single them out as a unique domain of very high European added-value.The legal instruments at the disposal of the European Union, in terms of the Article 168 of the Treaties, are very limited. However a combination of instruments using the research and the pharmaceutical legal basis and an intensive and creative use of funding from the Health Programmes has permitted to create a solid basis that Member States have considered enough to put rare diseases in a privileged position in the health agenda.The adoption of the Commission Communication, in November 2008, and of the Council Recommendation, in June 2009, and in 2011 the adoption of the Directive on Cross-border healthcare., have created an operational framework to act in the field of rare disease with European coordination in several areas (classification and codification, European Reference Networks, orphan medicinal products, the Commission expert group on rare diseases, etc.).Rare diseases is an area with high and practical potential for the European cooperation.

  6. Extraction studies on rare earths using dinonyl phenyl phosphoric acid

    International Nuclear Information System (INIS)

    Anitha, M.; Singh, D.K.; Kotekar, M.K.; Vijayalakshmi, R.; Singh, H.

    2011-01-01

    Rare earths are widely used in phosphor materials, magnetic substances, alloys, catalyst, lasers, superconductors, solid oxide fuel cells and in nuclear applications. The high value of these elements depends on their effective separation into high purity compounds. The separation into individual rare earths is very difficult to achieve, due to the very low separation factors between two adjacent rare earths arising due to similar chemical properties. Taking the advantage of variation in basicity, the separation is generally accomplished by solvent extraction or ion exchange. There are several references on the separation of rare earth in different media employing various types of extractants such as 2-ethylhexyl 2-ethyhexylphosphonic acid (EHEHPA) and di-2-ethyl hexyl phosphoric acid (D2EHPA) which have been widely used for the separation and purification of rare earths. Dinonyl phenyl phosphoric acid (DNPPA) is an organo phosphorus extractant (pKa = 2.54) and is an aromatic analogue of D2EHPA, which extracts metal ion by cation exchange mechanism. DNPPA was explored to recover rare earths from phosphate media such as wet process phosphoric acid and merchant grade acid. However, there is no information available in literature on DNPPA for RE extraction from chloride medium. Therefore, an attempt has been made in the present study to investigate the feasibility of using DNPPA for extraction of La(III), Dy(III) and Y(III) from chloride medium

  7. POLYCYSTIC OVARIES WITH STROMAL HYPERTHECOSIS: A RARE PRESENTATION

    Directory of Open Access Journals (Sweden)

    Shaila

    2016-05-01

    Full Text Available Presenting a 47-year-old peri-menopausal lady with hypomenorrhoea, temporal baldness, alopecia, hirsutism. The histopathology was polycystic ovaries with stromal hyperthecosis. Hughesdon described hyperthecosis as a severe form of PCOS. Hyperthecosis is rare in young women, with clinical features similar to PCOS. However, these women are usually more virilised.

  8. Assessing long-term and rare adverse effects of medicines

    NARCIS (Netherlands)

    Duijnhoven, R.G.|info:eu-repo/dai/nl/357218574

    2016-01-01

    Clinical studies in the development of new medicines are primarily designed to investigate efficacy. Knowledge of adverse effects is therefore limited at the time of approval of new medicines. In this thesis several studies were conducted to investigate long-term and rare adverse effects of

  9. Congenital pulmonary arteriovenous malformation: a rare cause of ...

    African Journals Online (AJOL)

    Pulmonary arteriovenous malformation (PAVM) is a rare condition in which there is abnormal connection between pulmonary arteries and veins. The disorder usually appears in late childhood or early adult life, with dyspnea on exertion, clubbing or cyanosis. We present two patients with severe cyanosis and their work-up ...

  10. Kallmann syndrome and paranoid schizophrenia: A rare combination

    NARCIS (Netherlands)

    W.M.A. Verhoeven (Wim); J.I.M. Egger (Jos); J.E.J.M. Hovens (Hans); E.H. Hoefsloot (Lies)

    2013-01-01

    textabstractKallmann syndrome (KS) is a genetically heterogeneous and rare disorder characterised by the combination of hypothalamic hypogonadism and anosmia/hyposmia, a variable degree of intellectual disability and several somatic anomalies. In about one-third of the patients, mutations have been

  11. Thanatophoric Dysplasia: A Rare Entity

    Directory of Open Access Journals (Sweden)

    N.S. Naveen

    2011-05-01

    Full Text Available Thanatophoric dysplasia is the lethal skeletal dysplasia characterized by marked underdevelopment of the skeleton and short-limb dwarfism. The child will be having a short neck, narrow thoracic cage and protuberant abdomen. Other anatomical features include a relatively enlarged head with frontal bossing, prominent eyes, hypertelorism and the depressed nasal bridge. The diagnosis is usually made with the ultrasonography in the second trimester. In this study we report a case of this rare entity with emphasis on its anatomical features, abnormalities and clinical profile with relevant review of literature.

  12. Microfibrillar cardiomyopathy: A rare case

    Directory of Open Access Journals (Sweden)

    Narender Kumar

    2011-01-01

    Full Text Available Microfibrillar cardiomyopathy is a very rare cause of restrictive cardiomyopathy (RCM. The index case was a male patient who presented with shortness of breath and pedal edema. Further clinical investigations favored a clinical diagnosis of RCM. An endomyocardial biopsy revealed subendocardial and interstitial hyaline eosinophillic material resembling amyloid that did not stain with Congo red. An electron microscopic examination showed that this material was composed of twisted linear and bundles of tangled microfibrils. The etiology of the microfibrillar deposition is currently unknown. The pathologists should entertain the diagnosis of microfibrillar cardiomyopathy in suspected cases of amyloidosis that are negative for Congo red.

  13. Maxillary Osteomyelitis: A Rare Entity

    Directory of Open Access Journals (Sweden)

    Ayaaz Habib

    2016-01-01

    Full Text Available Osteomyelitis of the maxilla is now a rare event with the advent of antibiotics. The two predominant causes are odontogenic infections and sinusitis. Immunocompromised states such as diabetes, HIV, and malnutrition increase the risk of osteomyelitis. It is important to recognize this early as it is a difficult entity to treat with potentially serious consequences. We report an unusual case of right sided maxillary osteomyelitis in a lady with poorly controlled diabetes in rural Lincolnshire. Biopsy of the right maxillary bone showed features of acute osteomyelitis. This responded well to a prolonged course of oral antibiotics.

  14. Retroperitoneal Schwannoma: A Rare Case

    Directory of Open Access Journals (Sweden)

    Murat Kalaycı

    2011-01-01

    Case Presentation. A 38-year-old woman was admitted to the emergency service with the complaints of progressive abdominal pain and nausea for the last 24 hours. Abdominal examination was compatible with acute abdomen. Acute appendicitis was diagnosed by CT. During CT evaluation, a round shaped soft-tissue mass at the retroperitoneal area inferior to the right kidney was detected, The mass was resected and histology revealed schwannoma. Conclusion. Rare tumoral lesions with benign course such as schwannoma can be detected incidentally.

  15. Cosmology tests in rare kaon decays

    Directory of Open Access Journals (Sweden)

    Duk Viacheslav

    2016-01-01

    Full Text Available The Standard Model (SM of particle physics is an extremely successful theory that effectively describes strong and electroweak interactions up to the energies presently accessible. Still, the SM does not explain the observed parameters of neutrino oscillations, baryon asymmetry of the Universe and Dark Matter (DM, and contains a fine-tuning of 16 orders of magnitude (the gauge hierarchy problem. Various New Physics (NP models beyond the SM have been developed in order to address the above limitations. This paper concentrates on several models related to cosmology and their tests in rare kaon decays. In particular, recent NA48/2 results on the search for heavy neutrinos, light in-flatons and dark photons are presented. Prospects for the ongoing NA62 experiment are discussed.

  16. Exclusive rare radiative decays of B mesons

    International Nuclear Information System (INIS)

    Barik, N.; Kar, S.; Dash, P.C.

    1998-01-01

    The exclusive rare radiative B decays are studied in the relativistic independent quark model based on the confining potential in the scalar-vector harmonic form. The relevant form factors as well as the branching ratios for the processes B 0 →K *0 γ and B ± →K *± γ have been estimated in reasonable agreement with the available experimental data. The result compares well with several other model predictions. The calculation has been extended to the CKM-favored process B s →φγ and CKM-suppressed processes B u,d →ργ and B s →K * γ. copyright 1997 The American Physical Society

  17. A RARE CASE OF HUGE CERVICAL FIBROID

    Directory of Open Access Journals (Sweden)

    Tushar Tatyaba Palve

    2017-07-01

    Full Text Available PRESENTATION OF CASE Leiomyomas are the most common tumor of uterus and the female pelvis. It is impossible to determine true incidence, all though frequently quoted incidence of 50% seems reasonable at post mortem examination1 . However only 1- 2% of it are confined to cervix. Cervical leiomyomata are mostly single and are subserous or interstitial in origin. Generally these tumours presents with retention of urine, constipation, sensation of something coming out of vagina, & rarely at times present with abdominal mass which may mimic ovarian cancer. 47 year old unmarried nulligravida with complaints of severe dysmenorrhagia since one year and lump in abdomen which has grown over period of one year. There was no bowel and bladder complaints, On physical examination pallor present, on abdominal examination 26-28 week firm solid mass arising from pelvis with restricted mobility was present, Clinically no ascites, patient was investigated on OPD basis.

  18. A RARE CASE OF ALKAPTONURIA: CASE REPORT

    Directory of Open Access Journals (Sweden)

    Dharmambal

    2016-01-01

    Full Text Available INTRODUCTION Alkaptonuria is a rare metabolic autosomal recessive disorder due to Homogentisic acid oxidase deficiency. There is generalized deposition of oxidized HGA in tissues mainly in fibrous and cartilaginous tissues. The disease is generally characterized by dark urine, ochronosis, and arthritis. A 50-year-old female attended the hospital with severe Joint Pain, Itching, Parasitophobia, Difficulty in hearing, Lack of clear vision and Discoloration of urine for different durations ranging from 6 months to 10 years. Alkaptonuria was suspected and accordingly detailed clinical examination and investigations were carried out. Biochemical analysis was positive for Alkaptonuria, X-ray was supportive for the diagnosis, and Histopathological examination revealed alkaptonuric pathology. The treatment was initiated with Ascorbic acid and protein restricted diet with symptomatic management and being followed up.

  19. A Rare Cause of Upper Airway Obstruction in a Child

    OpenAIRE

    Ahmed, H.; Ndiaye, C.; Barry, M. W.; Thiongane, Aliou; Mbaye, A.; Zemene, Y.; Ndiaye, I. C.

    2017-01-01

    Ventricular band cyst is a rare condition in children but can result in severe upper airway obstruction with laryngeal dyspnea or death. The diagnosis should be considered in any stridor in children with previous history of intubation or respiratory infections. We report a case of a 4-year-old girl, received in an array of severe respiratory distress, emergency endoscopy was done, and a large ventricular tape band cyst obstructing the air way was found. Complete excision was made, and postope...

  20. Putting a Face on Rare Diseases

    Science.gov (United States)

    ... who have a rare and potentially dangerous disease, Ehlers-Danlos syndrome. Photo Courtesy of: Patricia Weltin That has been ... daughters with a rare and potentially dangerous disease, Ehlers-Danlos syndrome (EDS), a connective tissue disorder causing joint dislocations, ...

  1. Catalogue of Korean manuscripts and rare books

    DEFF Research Database (Denmark)

    Lerbæk Pedersen, Bent

    2014-01-01

    Catalogue of Korean manuscripts and rare books in The Royal Library, Copenhagen and the National Museum of Denmark......Catalogue of Korean manuscripts and rare books in The Royal Library, Copenhagen and the National Museum of Denmark...

  2. Screening for severe combined immunodeficiency in neonates

    OpenAIRE

    Kelly, Brian T; Tam, Jonathan S; Verbsky, James W; Routes, John M

    2013-01-01

    Brian T Kelly,1 Jonathan S Tam,1 James W Verbsky,1,2 John M Routes1,2 1Department of Pediatrics, 2Department of Microbiology and Molecular Genetics, Medical College of Wisconsin, Milwaukee, WI, USA Abstract: Severe combined immunodeficiency (SCID) is a rare disease that severely affects the cellular and humoral immune systems. Patients with SCID present with recurrent or severe infections and often with chronic diarrhea and failure to thrive. The disease is uniformly fatal, making early diag...

  3. Radioactive rare gas recoverying device

    International Nuclear Information System (INIS)

    Kasai, Shigeo

    1989-01-01

    The apparatus of the present invention comprises a vessel for containing coolants, an introduction valve and an introduction pipe for introducing radioactive rare gases and an adsorption floor disposed in the coolants. A josephson device is disposed being immersed in the coolants between a radiation detector for detecting the radioactive level adsorbed to the adsorption floor and a driving section for driving the introduction valve by the signal from the detector. With this constitution, radioactive rare gases introduced into the coolants and then cooled and liquefied are recovered by the adsorption floor. As the adsorption proceeds and when the radioactivity level exceeds a maximum level in the effective shielding range of the recovery apparatus, the signal current from the radiation detector also exceeds a predetermined level. If radioactivity exceeds the maximum level, the electrical resistance of the josephson device is increased infinitely by the josephson effect to close the introduction valve. Accordingly, the radioactivity is not absorbed beyond the effective shielding range. (I.S.)

  4. Purdue Rare Isotope Measurement Laboratory

    Science.gov (United States)

    Caffee, M.; Elmore, D.; Granger, D.; Muzikar, P.

    2002-12-01

    The Purdue Rare Isotope Measurement Laboratory (PRIME Lab) is a dedicated research and service facility for accelerator mass spectrometry. AMS is an ultra-sensitive analytical technique used to measure low levels of long-lived cosmic-ray-produced and anthropogenic radionuclides, and rare trace elements. We measure 10Be (T1/2 = 1.5 My), 26Al (.702 My), 36Cl (.301 My), and 129I (16 My), in geologic samples. Applications include dating the cosmic-ray-exposure time of rocks on Earth's surface, determining rock and sediment burial ages, measuring the erosion rates of rocks and soils, and tracing and dating ground water. We perform sample preparation and separation chemistries for these radio-nuclides for our internal research activities and for those external researchers not possessing this capability. Our chemical preparation laboratories also serve as training sites for members of the geoscience community developing these techniques at their institutions. Research at Purdue involves collaborators among members of the Purdue Departments of Physics, Earth and Atmospheric Sciences, Chemistry, Agronomy, and Anthropology. We also collaborate and serve numerous scientists from other institutions. We are currently in the process of modernizing the facility with the goals of higher precision for routinely measured radio-nuclides, increased sample throughput, and the development of new measurement capabilities for the geoscience community.

  5. Metallothermic reduction of rare earth oxides

    International Nuclear Information System (INIS)

    Sharma, R.A.

    1986-01-01

    Rare earth oxides can be reduced to rare earth metals by a novel, high yield, metallothermic process. The oxides are dispersed in a suitable, molten, calcium chloride bath along with sodium metal. The sodium reacts with the calcium chloride to produce calcium metal which reduces the rare earth oxides to rare earth metals. The metals are collected in a discrete layer in the reaction vessel

  6. Rare earth-iron-boron premanent magnets

    International Nuclear Information System (INIS)

    Ghendehari, M.H.

    1988-01-01

    This patent describes a method for producing rare earth-iron-boron permanent magnets containing added rare earth oxide, comprising the steps of: (a) mixing a particulate alloy containing at least one rare earth metal, iron, and boron with at least one particulate rare earth oxide; (b) aligning magnetic domains of the mixture in a magnetic field; (c) compacting the aligned mixture to form a shape; and (d) sintering the compacted shape

  7. [Anorectal pain in children: rare or rarely recognised?].

    Science.gov (United States)

    Sonneveld, Laura J H; Engelberts, Adèle C; van den Elzen, Annette P M

    2016-01-01

    Anorectal pain is a common symptom, often as part of functional gastrointestinal disorders. Children seldom present with this complaint. Proctalgia fugax and chronic proctalgia are both anorectal pain syndromes but differ in duration and frequency of episodes and in pain characteristics. No research has been conducted on anorectal pain syndromes in children. We present two patients. Firstly, an 8-year-old girl who suffered from anorectal cramps. We found no underlying cause apart from constipation. The symptoms disappeared spontaneously. The second concerned an 8-year-old boy who presented with recurrent anorectal cramps. He was diagnosed with celiac disease. Anorectal dysfunction and visceral hypersensitivity have been described in adult celiac patients. Symptoms of anorectal pain in children are rare probably because it often remains unrecognised. Noninvasive diagnostic methods and interventions are preferred in paediatric medicine. Screening for celiac disease in children with anorectal pain episodes should be considered.

  8. A Rare Cutaneous Adnexal Tumour with a Rare Presentation

    Directory of Open Access Journals (Sweden)

    Vijayalaxmi S. Patil

    2016-04-01

    Full Text Available Proliferating Trichilemmal Tumour (PTT is a rapidly growing large cutaneous adnexal neoplasm. Although biologically considered as benign, it may be locally aggressive. Malignant transformation of these lesions, known as Malignant Proliferating Trichilemmal Tumour (MPTT has rarely been reported. So far in the literature, only 39 well-documented cases of MPTT have been reported. MPTT has been stated to be a neoplasm of the older age group according to review of the literature. We present a case of MPTT in a young male. A 25 year old male presented with a scalp swelling of 2 years duration with a recent rapid enlargement. The swelling was excised and histopathological examination of the excised specimen revealed features of MPTT. The differential diagnosis of MPTTis squamous cell carcinoma as both share common features. Accurate diagnosis of MPTT is essential since it has a tendency to metastasize and recur more frequently than squamous cell carcinoma.

  9. Mohs micrographic surgery of rare cutaneous tumours

    NARCIS (Netherlands)

    Flohil, S.C.; Lee, C.B. van; Beisenherz, J.; Mureau, M.A.M.; Overbeek, L.I.H.; Nijsten, T.; Bos, R.R.

    2017-01-01

    BACKGROUND: Recurrence rates after Mohs micrographic surgery (MMS) for rare cutaneous tumours are poorly defined. OBJECTIVE: To investigate the recurrence rate after MMS for rare cutaneous tumours at a university centre. METHODS & MATERIALS: Retrospective review of all rare cutaneous tumours treated

  10. 75 FR 47458 - TRICARE; Rare Diseases Definition

    Science.gov (United States)

    2010-08-06

    ... tribal governments, in aggregate or by the private sector, of $100 million or more in any one year... TRICARE; Rare Diseases Definition AGENCY: Office of the Secretary, DoD. ACTION: Final rule. SUMMARY: This final rule revises the definition of rare diseases to adopt the definition of a rare disease as...

  11. Direct Reuse of Rare Earth Permanent Magnets—Coating Integrity

    DEFF Research Database (Denmark)

    Høgberg, Stig; Holbøll, Joachim; Mijatovic, Nenad

    2017-01-01

    Rare earth permanent magnets can be reused directly as an alternative to traditional recycling methods, in which scrapped magnets are reprocessed into new magnets by undergoing many of the original energy-intensive and expensive production processes. Direct reuse entails using segmented magnet...... assemblies built by several small standard-sized magnets that can be reused directly in a number of different applications. A central part of the direct reuse strategy is to separate and demagnetize magnets by heating them to the Curie temperature. We investigated the validity of direct reuse as a rare earth...

  12. Postoperative pyoderma gangrenosum: A rare complication after appendectomy

    Directory of Open Access Journals (Sweden)

    G Faghihi

    2015-01-01

    Full Text Available Pyoderma gangrenosum (PG is an uncommon inflammatory ulcerative skin disease. It is characterized by painful progressive necrosis of the wound margins. Rarely, postoperative pyoderma gangrenosum (PPG manifests as a severe disturbance of wound healing following surgical interventions. Only rare cases of this complication have been reported after appendectomy. We report a case of PPG in a 29-year-old female after appendectomy. She was successfully treated with oral prednisolone. Postoperative pyoderma gangrenosum should be kept in mind in the differential diagnosis of any postoperative delayed wound healing, because this disease is simply distinguished from a postoperative wound.

  13. Postoperative pyoderma gangrenosum: A rare complication after appendectomy

    Science.gov (United States)

    Faghihi, G; Abtahi-Naeini, B; Nikyar, Z; Jamshidi, K; Bahrami, A

    2015-01-01

    Pyoderma gangrenosum (PG) is an uncommon inflammatory ulcerative skin disease. It is characterized by painful progressive necrosis of the wound margins. Rarely, postoperative pyoderma gangrenosum (PPG) manifests as a severe disturbance of wound healing following surgical interventions. Only rare cases of this complication have been reported after appendectomy. We report a case of PPG in a 29-year-old female after appendectomy. She was successfully treated with oral prednisolone. Postoperative pyoderma gangrenosum should be kept in mind in the differential diagnosis of any postoperative delayed wound healing, because this disease is simply distinguished from a postoperative wound. PMID:25511218

  14. Gingival enlargement unveiling sarcoidosis: Report of a rare case

    Directory of Open Access Journals (Sweden)

    Sabeeha Abbas Kadiwala

    2013-01-01

    Full Text Available Sarcoidosis is classified as an acquired systemic granulomatous disease. Because of the fact that sarcoidosis affects multiple tissues and organs, it is characterized by many potential signs and symptoms, as well as by the presence of non-caseating granulomas in the organs involved. Although oral sarcoidosis is relatively rare, it may however, present in the oral cavity. This report presents a rare case of sarcoidosis with the initial presenting symptom as severe generalized gingival enlargement. The gingival enlargement was treated by gingivectomy. After histopathological examination of gingival biopsy and certain special investigations, a diagnosis of sarcoidosis was made.

  15. Merkel Cell Carcinoma: Interdisciplinary Management of a Rare Disease

    International Nuclear Information System (INIS)

    Schneider, S.; Thurnher, D.; Erovic, B. M.

    2013-01-01

    The goal of this paper is to review contemporary multidisciplinary treatment with reference to Milkier cell carcinoma. Management of this rare but highly aggressive skin cancer is a complex undertaking that necessitates an understanding of its etiology, epidemiology, clinical presentation, and the coordinated work of several clinical specializations. Recent Findings. The contemporary literature employs a multidisciplinary approach to achieve the best patient's treatment. Conclusion. This paper presents an algorithm for contemporary management for the rare and aggressive Merkel cell carcinoma. Multidisciplinary approach in a tumor center provides high-quality care for patients with Merkel cell carcinoma.

  16. Merkel Cell Carcinoma: Interdisciplinary Management of a Rare Disease

    Directory of Open Access Journals (Sweden)

    Sven Schneider

    2013-01-01

    Full Text Available Background. The goal of this paper is to review contemporary multidisciplinary treatment with reference to Merkel cell carcinoma. Management of this rare but highly aggressive skin cancer is a complex undertaking that necessitates an understanding of its etiology, epidemiology, clinical presentation, and the coordinated work of several clinical specializations. Recent Findings. The contemporary literature employs a multidisciplinary approach to achieve the best patient's treatment. Conclusion. This paper presents an algorithm for contemporary management for the rare and aggressive Merkel cell carcinoma. Multidisciplinary approach in a tumor center provides high-quality care for patients with Merkel cell carcinoma.

  17. Hiccup: An Extremely Rare Presentation of Thyrotoxicosis of Graves’ Disease

    Directory of Open Access Journals (Sweden)

    Irshad Parray

    2011-03-01

    Full Text Available Persistent hiccup is a rare but potentially severe condition that can be symptomatic of a variety of diseases or idiopathic. Most episodes last only a few minutes and are self-limited, but hiccup can get persistent and become a real problem for physician and patient alike. The center of hiccup may be activated by a great variety of stimuli travelling along different nerve pathways and bring different effecter responses. We report a case of persistent hiccup as a presentation of impending thyroid storm of Graves’ disease. Though the condition is rare, clinicians should remain alert to the possibility of this diagnosis.

  18. Thin-film method-XRF determination of the composition of rare earth oxides

    International Nuclear Information System (INIS)

    Xiao Deming

    1992-01-01

    The author describes the thin-film sample preparation by precipitation-pumping filtering method and the composition of rare earth oxide materials by XRF determination. The determination limits are 0.01% to 0.17%. The coefficients of variation are in the range of 0.85% to 14.9%. The analytical results of several kinds of rare earth oxide materials show that this method can be applied to the determination of the composition of rare earth oxide mixtures

  19. Severe Hypertriglyceridemia Induced Pancreatitis in Pregnancy

    Directory of Open Access Journals (Sweden)

    Natasha Gupta

    2014-01-01

    Full Text Available Acute pancreatitis caused by severe gestational hypertriglyceridemia is a rare complication of pregnancy. Acute pancreatitis has been well associated with gallstone disease, alcoholism, or drug abuse but rarely seen in association with severe hypertriglyceridemia. Hypertriglyceridemia may occur in pregnancy due to normal physiological changes leading to abnormalities in lipid metabolism. We report a case of severe gestational hypertriglyceridemia that caused acute pancreatitis at full term and was successfully treated with postpartum therapeutic plasma exchange. Patient also developed several other complications related to her substantial hypertriglyceridemia including preeclampsia, chylous ascites, retinal detachment, pleural effusion, and chronic pericarditis. This patient had no previous family or personal history of lipid abnormality and had four successful prior pregnancies without developing gestational hypertriglyceridemia. Such a severe hypertriglyceridemia is usually seen in patients with familial chylomicronemia syndromes where hypertriglyceridemia is exacerbated by the pregnancy, leading to fatal complications such as acute pancreatitis.

  20. Waardenburg syndrome: A rare case

    Directory of Open Access Journals (Sweden)

    Shivlal M Rawlani

    2018-01-01

    Full Text Available Waardenburg Syndrome is a rare disorder of neural crest cell development. It is genetically inherited. Varying in prevalence from 1:42000 to 1:50,000, it compromises approximately 2-5% of congenital deaf children. The syndrome is not expressed in its complete form, in about 20% cases, which adds for its heterogenisity . Even among people affected in the same family,the features do vary. Unilateral heterochromia that manifests as lighter pigmentation of one iris is associated with Waardenburg syndrome and Parry-Romberg syndrome and less commonly with Hirschsprung disease. A case of ten yrs. old boy with a typical facial profile and hearing loss is reported.

  1. Ultra-Rare B Decays

    International Nuclear Information System (INIS)

    Grinstein, Benjamin

    2004-01-01

    A good place to look for deviations from the Standard Model is in decay modes of B mesons, like purely leptonic decays B → lv, for which a very long Standard Model lifetime is due to an accidental suppression of the decay amplitude. For other rare decay modes involving no hadrons in the final state (e.g., B → γl+l-, B → γlvl and B → vv-barγ) new results on QCD factorization in exclusive processes show that all the decay rates are given in terms of a single universal form factor. Hence, trustworthy relations between different processes can be used to test the Standard Model of electroweak interactions. Sometimes, surprisingly, a large energy expansion may allow computation when a hadron is in the final state. An example is B → πl+l- which can be used to settle the ambiguity in α from a measurement of sin2α from CP asymmetries

  2. Rare complications of cesarean scar

    International Nuclear Information System (INIS)

    Mahajan, Divyesh; Kang, Mandeep; Sandhu, Manavjit Singh; Jain, Vanita; Kalra, Naveen; Khandelwal, Niranjan

    2013-01-01

    Cesarean scar pregnancy (CSP) and cesarean scar dehiscence (CSD) are the most dreaded complications of cesarean scar (CS). As the incidence of CS is increasing worldwide, so is the incidence of CSP, especially in cases with assisted reproduction techniques. It is of utmost importance to diagnose CSP in the early first trimester, as it can lead to myometrial rupture with fatal outcome. On the other hand, CSD may be encountered during pregnancy or in the postpartum period. CSD in the postpartum period is very rare and can cause secondary postpartum hemorrhage (PPH) leading to increased maternal morbidity or even death if not diagnosed and managed promptly. Both complications can be diagnosed on ultrasonography (USG) and confirmed on magnetic resonance imaging (MRI). These two conditions carry high morbidity and mortality. In this article, we highlight the role of imaging in the early diagnosis and management of these conditions

  3. Rare complications of cesarean scar

    Directory of Open Access Journals (Sweden)

    Divyesh Mahajan

    2013-01-01

    Full Text Available Cesarean scar pregnancy (CSP and cesarean scar dehiscence (CSD are the most dreaded complications of cesarean scar (CS. As the incidence of CS is increasing worldwide, so is the incidence of CSP, especially in cases with assisted reproduction techniques. It is of utmost importance to diagnose CSP in the early first trimester, as it can lead to myometrial rupture with fatal outcome. On the other hand, CSD may be encountered during pregnancy or in the postpartum period. CSD in the postpartum period is very rare and can cause secondary postpartum hemorrhage (PPH leading to increased maternal morbidity or even death if not diagnosed and managed promptly. Both complications can be diagnosed on ultrasonography (USG and confirmed on magnetic resonance imaging (MRI. These two conditions carry high morbidity and mortality. In this article, we highlight the role of imaging in the early diagnosis and management of these conditions.

  4. Splenic abscess: a rare presentation

    Directory of Open Access Journals (Sweden)

    Mohit Bhatia

    2015-01-01

    Full Text Available Splenic abscess is a rare clinical entity with an incidence of 0.2-0.7% in autopsy-based studies. When untreated, splenic abscess is associated with nearly 100% mortality; in treated patients, the mortality rate is 16.6% during the first 90 days. It mostly occurs in patients with neoplasia, immunodeficiency, trauma, diabetes or splenic infarct. The incidence of splenic abscess is thought to be growing because of the increase in the number of immunocompromised patients who are particularly at risk for this disease and also because of the widespread use of diagnostic modalities. However, the optimal treatment for this remains unclear. We present a case of a 42-year-old man diagnosed with multiloculated splenic abscess and was subjected to splenectomy.

  5. WHY ARE PULSAR PLANETS RARE?

    Energy Technology Data Exchange (ETDEWEB)

    Martin, Rebecca G.; Livio, Mario; Palaniswamy, Divya [Department of Physics and Astronomy, University of Nevada, Las Vegas, 4505 South Maryland Parkway, Las Vegas, NV 89154 (United States)

    2016-12-01

    Pulsar timing observations have revealed planets around only a few pulsars. We suggest that the rarity of these planets is due mainly to two effects. First, we show that the most likely formation mechanism requires the destruction of a companion star. Only pulsars with a suitable companion (with an extreme mass ratio) are able to form planets. Second, while a dead zone (a region of low turbulence) in the disk is generally thought to be essential for planet formation, it is most probably rare in disks around pulsars, because of the irradiation from the pulsar. The irradiation strongly heats the inner parts of the disk, thus pushing the inner boundary of the dead zone out. We suggest that the rarity of pulsar planets can be explained by the low probability for these two requirements to be satisfied: a very low-mass companion and a dead zone.

  6. Liposarcome dorsal: aspect clinique rare

    Science.gov (United States)

    Agbessi, Odry; Arrob, Adil; Fiqhi, Kamal; Khalfi, Lahcen; Nassih, Mohammed; El Khatib, Karim

    2015-01-01

    Décrit la première fois par Virchow en 1860, le liposarcome est une tumeur mésenchymateuse rare. Cette rareté est relative car les liposarcomes représentent quand même 14 à 18% de l'ensemble des tumeurs malignes des parties molles et ils constituent le plus fréquent des sarcomes des parties molles. Pour la majorité des auteurs, il ne se développerait jamais sur un lipome ou une lipomatose préexistant. Nous rapportons un cas de volumineux liposarcome de la face dorsale du tronc. L'histoire de la maladie, l'aspect clinique inhabituel « de tumeur dans tumeur », l'aspect de la pièce opératoire nous fait évoquer la possibilité de la transformation maligne d'un lipome bénin préexistant. PMID:26113914

  7. Severe hyponatremia associated with escitalopram

    Directory of Open Access Journals (Sweden)

    Gautam Rawal

    2017-01-01

    Full Text Available Hyponatremia is a rare but potentially fatal complication of selective serotonin reuptake inhibitor (SSRI therapy with only limited cases of escitalopram as the causative drug. We report the case of a 54-year-old hypertensive female who was admitted to the hospital with seizure episode and subsequently diagnosed to have severe hyponatremia due to SSRI-induced syndrome of inappropriate antidiuretic hormone (SIADH with the cause attributed to the short history of intake of escitalopram for depression. All SSRIs, including escitalopram, can cause SIADH and should be used with caution in the depressive patients with regular monitoring of electrolytes, especially in the elderly.

  8. Heavy rare earths, permanent magnets, and renewable energies: An imminent crisis

    International Nuclear Information System (INIS)

    Smith Stegen, Karen

    2015-01-01

    This article sounds the alarm that a significant build-out of efficient lighting and renewable energy technologies may be endangered by shortages of rare earths and rare earth permanent magnets. At the moment, China is the predominant supplier of both and its recent rare earth industrial policies combined with its own growing demand for rare earths have caused widespread concern. To diversify supplies, new mining—outside of China—is needed. But what many observers of the “rare earth problem” overlook is that China also dominates in (1) the processing of rare earths, particularly the less abundant heavy rare earths, and (2) the supply chains for permanent magnets. Heavy rare earths and permanent magnets are critical for many renewable energy technologies, and it will require decades to develop new non-Chinese deposits, processing capacity, and supply chains. This article clarifies several misconceptions, evaluates frequently proposed solutions, and urges policy makers outside of China to undertake measures to avert a crisis, such as greater support for research and development and for the cultivation of intellectual capital. - Highlights: • Rare earths are needed for many efficient lighting and renewable energy technologies. • The industries for rare earths and permanent magnets are dominated by China. • China's reliability is compromised, necessitating non-Chinese mining and processing. • Recycling, substitution and reducing rare earth content are long-term solutions only. • Policy makers should support development of supply chains and intellectual capital

  9. Rare earth elements: end use and recyclability

    Science.gov (United States)

    Goonan, Thomas G.

    2011-01-01

    Rare earth elements are used in mature markets (such as catalysts, glassmaking, lighting, and metallurgy), which account for 59 percent of the total worldwide consumption of rare earth elements, and in newer, high-growth markets (such as battery alloys, ceramics, and permanent magnets), which account for 41 percent of the total worldwide consumption of rare earth elements. In mature market segments, lanthanum and cerium constitute about 80 percent of rare earth elements used, and in new market segments, dysprosium, neodymium, and praseodymium account for about 85 percent of rare earth elements used. Regardless of the end use, rare earth elements are not recycled in large quantities, but could be if recycling became mandated or very high prices of rare earth elements made recycling feasible.

  10. The Chinese Society of Rare Earth is Studying The Feasibility of Marketing Rare Earth Futures

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    Lin Donglu,secretary-general of the Chinese Society of Rare Earth recently said,the Chinese Society of Rare Earth undertook the research on subject of the National Social Science Fund Foundation on the reform of Chinese rare earth trading pricing mechanism on promoting RMB globalization,and is focusing on studying the feasibility of marketing rare earth futures variety.

  11. Rare metal granites and related rocks of the Ukrainian shield

    Directory of Open Access Journals (Sweden)

    Esipchuk, K.Ye.

    1993-12-01

    Full Text Available Two rare metal leucocratic granites, Perga and Kamennaya complexes, can be distinquished on the Ukrainian shield. The Perga complex consists of medium- and coarse grained, mainly porphyric, biotite, riebeckite and aegirine granites, granite porphyries, microclinites and albitites with rare metal mineralization (genthelvite, phenacite, tantalite, cassiterite and wolframite etc.. Granites from several stocks (up to 30 km2 in the northwestern part of the shield, situated along the fracture zone, restricted the large Korosten pluton of rapakivi granites to the northwest. The age of these granites (Pb-Pb and U-Pb methods on zircon and monazite practically coincide with the age of rapakivi granites being 1750 Ma. Within the Korosten complex of rapakivi granites we consider that zinnwaldite granites, which are characterized by fluorite and topazine mineralization, represent the final phase of pluton. These granites differ from the Perga ones by their low content of rare metals. The Kamennaya Mogila complex lies in the southeastern part of the Ukrainian shield. It consists of biotite and muscovite-biotite, medium- and coarse-grained (also porphyric, and occasionally greisining granites with rare metal mineralization (cassiterite, columbite, molybdenite, wolframite and beryl. Granites form several stocks (5-30 km2 situated 10-30 km to the west-northwest of the South-Kalchik gabbro-syenite-granite pluton. Granitoids in both of these complexes have similar isotopic ages (1800 Ma. Leucocratic subalkaline granites (the Novoyanisol type are known within the pluton itself, occupying an intermediate position between the above mentioned in terms of mineral and geochemical composition. The gabbro-syenite-granite formation of the Nearazov region has a substantial similarity to the anorthosite-rapakivi-granite formation. In this respect the relation of each of them to rare metal granites is rather remarkable. This relation is, most probably, not only spatial, but

  12. Computing return times or return periods with rare event algorithms

    Science.gov (United States)

    Lestang, Thibault; Ragone, Francesco; Bréhier, Charles-Edouard; Herbert, Corentin; Bouchet, Freddy

    2018-04-01

    The average time between two occurrences of the same event, referred to as its return time (or return period), is a useful statistical concept for practical applications. For instance insurances or public agencies may be interested by the return time of a 10 m flood of the Seine river in Paris. However, due to their scarcity, reliably estimating return times for rare events is very difficult using either observational data or direct numerical simulations. For rare events, an estimator for return times can be built from the extrema of the observable on trajectory blocks. Here, we show that this estimator can be improved to remain accurate for return times of the order of the block size. More importantly, we show that this approach can be generalised to estimate return times from numerical algorithms specifically designed to sample rare events. So far those algorithms often compute probabilities, rather than return times. The approach we propose provides a computationally extremely efficient way to estimate numerically the return times of rare events for a dynamical system, gaining several orders of magnitude of computational costs. We illustrate the method on two kinds of observables, instantaneous and time-averaged, using two different rare event algorithms, for a simple stochastic process, the Ornstein–Uhlenbeck process. As an example of realistic applications to complex systems, we finally discuss extreme values of the drag on an object in a turbulent flow.

  13. Spectrographic determination of some rare earths in thorium compounds

    International Nuclear Information System (INIS)

    Brito, J. de.

    1977-01-01

    A method for spectrographic determination of Gd, Sm, Dy, Eu, Y, Yb, Tm and Lu in thorium compounds has been developed. Sensibilities of 0.01 μg rare earths/g Th02 were achieved. The rare earth elements were chromatographycally separated in a nitric acid-ether-cellulose system. The solvent mixture was prepared by dissolving 11% of concentrated nitric acid in ether. The method is based upon the sorption of the rare earths on activated cellulose, the elements being eluted together with 0.01 M HNO 3 . The retention of the 152 , 154 Eu used as tracer was 99,4%. The other elements showed recoveries varying from 95 to 99%. A direct carrier destillation procedure for the spectrochemical determination of the mentioned elements was used. Several concentrations of silver chloride were used to study the volatility behavior of the rare earths. 2%AgCl was added to the matrix as definite carrier, being lantanum selected as internal standard. The average coefficient of variation for this method was +- -+ 7%. The method has been appleid to the analysis of rare earths in thorium coumpounds prepared by Thorium Purification Pilot Plant at Atomic Energy Institute, Sao Paulo [pt

  14. Pachydermoperiostosis: a rare mimicker of acromegaly.

    Science.gov (United States)

    Abdullah, Noor Rafhati Adyani; Jason, Wong Lok Chin; Nasruddin, Azraai Bahari

    2017-01-01

    Pachydermoperiostosis is a very rare osteoarthrodermopathic disorder whose clinical and radiographic presentations may mimic those of acromegaly. In the evaluation of patients with acromegaloid appearances, pachydermoperiostosis should be considered as a differential diagnosis. In this article, we report a 17-year-old boy who presented with 2-year history of acral enlargement and facial appearance changes associated with joint pain and excessive sweating. He had been investigated extensively for acromegaly, and the final diagnosis was pachydermoperiostosis. There is a broad range of differential diagnosis for acromegaloid features such as acromegaly, pseudoacromegaly with severe insulin resistance, Marfan's syndrome, McCune-Albright and a rare condition called pachydermoperiostosis.Once a patient is suspected to have acromegaly, the first step is biochemical testing to confirm the clinical diagnosis, followed by radiologic testing to determine the cause of the excess growth hormone (GH) secretion. The cause is a somatotroph adenoma of the pituitary in over 95 percent of cases.The first step is measurement of a serum insulin-like growth factor 1 (IGF1). A normal serum IGF1 concentration is strong evidence that the patient does not have acromegaly.If the serum IGF1 concentration is high (or equivocal), serum GH should be measured after oral glucose administration. Inadequate suppression of GH after a glucose load confirms the diagnosis of acromegaly.Once the presence of excess GH secretion is confirmed, the next step is pituitary magnetic resonance imaging (MRI).Atypical presentation warrants revision of the diagnosis. This patient presented with clubbing with no gigantism, which is expected in adolescent acromegalics as the growth spurt and epiphyseal plate closure have not taken place yet.

  15. A rare cause of fatal intracranial haemorrhage.

    LENUS (Irish Health Repository)

    Neligan, A

    2012-01-31

    INTRODUCTION: We report the case of a 53-year-old farmer with a 5-day history of severe headache, photophobia and neck stiffness. Full blood count (platelets 173), coagulation screen were normal throughout. Liver function tests remained normal apart from an elevated gamma-GT (156). CT Brain was normal. CSF analysis showed a WCC of 454\\/mm(3) (60% lymphocytes), elevated CSF protein (1.42 g\\/l) and a normal CSF glucose. He was commenced on IV antibiotics and IV acyclivor and improved. On day 3 of admission, he complained of a sudden severe headache, became unresponsive (GCS 3\\/15). INVESTIGATIONS: CT Brain showed a massive left intraventricular haemorrhage. He died 4 days later. Subsequent serum serology for leptospirosis was positive. A repeat sample taken 4 days post-admission, showed a rising IgM indicating active leptospirosis. Detailed pathological examination confirmed intracerebral haemorrhage with normal cerebral vasculature. CONCLUSION: Leptospirosis is a rare cause of intracerebral haemorrhage even in the absence of coagulopathy.

  16. Ellis–Van Creveld syndrome in siblings: A rare case report

    Directory of Open Access Journals (Sweden)

    Sabitha Gokulraj

    2016-01-01

    Full Text Available Ellis–Van Creveld syndrome or chondroectodermal dysplasia is a rare autosomal recessive disorder presenting several skeletal manifestations and congenital heart malformations. Ellis–Van Creveld syndrome comprises of a tetrad of clinical manifestations of chondrodysplasia, polydactyly, ectodermal dysplasia, and cardiac defects. Here, we are presenting a very rare case of Ellis–Van Creveld syndrome in siblings.

  17. Gastric lactobezoar - a rare disorder?

    Directory of Open Access Journals (Sweden)

    Heinz-Erian Peter

    2012-01-01

    Full Text Available Abstract Gastric lactobezoar, a pathological conglomeration of milk and mucus in the stomach of milk-fed infants often causing gastric outlet obstruction, is a rarely reported disorder (96 cases since its first description in 1959. While most patients were described 1975-1985 only 26 children have been published since 1986. Clinically, gastric lactobezoars frequently manifest as acute abdomen with abdominal distension (61.0% of 96 patients, vomiting (54.2%, diarrhea (21.9%, and/or a palpable abdominal mass (19.8%. Respiratory (23.0% and cardiocirculatory (16.7% symptoms are not uncommon. The pathogenesis of lactobezoar formation is multifactorial: exogenous influences such as high casein content (54.2%, medium chain triglycerides (54.2% or enhanced caloric density (65.6% of infant milk as well as endogenous factors including immature gastrointestinal functions (66.0%, dehydration (27.5% and many other mechanisms have been suggested. Diagnosis is easy if the potential presence of a gastric lactobezoar is thought of, and is based on a history of inappropriate milk feeding, signs of acute abdomen and characteristic features of diagnostic imaging. Previously, plain and/or air-, clear fluid- or opaque contrast medium radiography techniques were used to demonstrate a mass free-floating in the lumen of the stomach. This feature differentiates a gastric lactobezoar from intussusception or an abdominal neoplasm. Currently, abdominal ultrasound, showing highly echogenic intrabezoaric air trapping, is the diagnostic method of choice. However, identifying a gastric lactobezoar requires an investigator experienced in gastrointestinal problems of infancy as can be appreciated from the results of our review which show that in not even a single patient gastric lactobezoar was initially considered as a possible differential diagnosis. Furthermore, in over 30% of plain radiographs reported, diagnosis was initially missed although a lactobezoar was clearly

  18. Sarcoidosis, Celiac Disease and Deep Venous Thrombosis: a Rare Association

    Directory of Open Access Journals (Sweden)

    Gökhan Çelik

    2011-11-01

    Full Text Available Sarcoidosis is a multisystem granulomatous disorder of unknown etiology and it may rarely be associated with a second disorder. Celiac disease is an immune-mediated enteropathy characterized with malabsorption caused by gluten intolerance, and several reports indicate an association between celiac disease and sarcoidosis. In addition, although celiac disease is associated with several extraintestinal pathologies, venous thrombosis has been rarely reported. Herein we present a rare case report of a patient with a diagnosis of sarcoidosis, celiac disease and deep venous thrombosis because of the rare association of these disorders. The patient was admitted with abdominal pain, weight loss, chronic diarrhea and a 5-day history of swelling in her right leg. A diagnosis of deep venous thrombosis was achieved by doppler ultrasonographic examination. The diagnosis of celiac disease was made by biopsy of duodenal mucosa and supported with elevated serum level of anti-gliadin IgA and IgG, and a diagnosis of sarcoidosis was achieved by transbronchial needle aspiration from the subcarinal lymph node during flexible bronchoscopy.

  19. Rare and very rare adverse effects of clozapine

    Directory of Open Access Journals (Sweden)

    De Fazio P

    2015-08-01

    knowledge of the drug, clinical vigilance, and rapid intervention can drastically reduce the morbidity and mortality related to CLZ treatment. Keywords: clozapine, rare adverse effects, schizophrenia, atypical antipsychotic

  20. New physics from rare beauty

    CERN Document Server

    AUTHOR|(CDS)2077817; Hulsbergen, W

    2010-01-01

    The Standard Model (SM) of particle physics offers a framework to describe sub-nuclear physics processes. Despite its success in describing a large variety of sub-nuclear phenomena, the SM leaves open a number of questions and is regarded by particle physicists as an effective quantum field theory, rather than a credible candidate to be the ultimate theory of fundamental interactions. Particle physicists postulates the existence of New Physics (NP) beyond the SM. This would result into the creation of new particles that could then be observed at higher energy regimes than hitherto explored. These particles could be observed directly or indirectly, through their contributions to quantum loops. A promising ground to look for NP are the Flavor Changing Neutral Currents (FCNCs). Due to its precise theoretical prediction, one of the most promising rare decay channel is the $B_s$ meson decaying into two muons. In this dissertation the strategy for an early measurement of the $B_{s} \\rightarrow \\mu \\mu$ branching ra...

  1. Recycling of Rare Earth Elements

    Science.gov (United States)

    Lorenz, Tom; Bertau, Martin

    2017-01-01

    Any development of an effective process for rare earth (RE) recycling has become more and more challenging, especially in recent years. Since 2011, when commodity prices of REs had met their all-time maximum, prices have dropped rapidly by more than 90 %. An economic process able to offset these fluctuations has to take unconventional methods into account beside well-known strategies like acid/basic leaching or solvent extraction. The solid-state chlorination provides such an unconventional method for mobilizing RE elements from waste streams. Instead of hydrochloric acid this kind of chlorination decomposes NH4Cl thermally to release up to 400 °C hot HCl gas. After cooling the resulting solid metal chlorides may be easily dissolved in pH-adjusted water. Without producing strongly acidic wastes and with NH4Cl as cheap source for hydrogen chloride, solid-state chlorination provides various advantages in terms of costs and disposal. In the course of the SepSELSA project this method was examined, adjusted and optimized for RE recycling from fluorescent lamp scraps as well as Fe14Nd2B magnets. Thereby many surprising influences and trends required various analytic methods to examine the reasons and special mechanisms behind them.

  2. Rare stable isotopes in meteorites

    International Nuclear Information System (INIS)

    Wilson, G.C.

    1981-01-01

    Secondary Ion Mass Spectrometry (SIMS) using accelerators has been applied with success to cosmic ray exposure ages and terrestrial residence times of meteorites by measuring cosmogenic nuclides of Be, Cl, and I. It is proposed to complement this work with experiments on rare stable isotopes, in the hope of setting constraints on the processes of solar nebula/meteoritic formation. The relevant species can be classified as: a) daughter products of extinct nuclides (halflife less than or equal to 2 x 10 8 y) -chronology of the early solar system; b) products of high temperature astrophysical processes - different components incorporated into the solar nebula; and c) products of relatively low temperature processes, stellar winds and cosmic ray reactions - early solar system radiation history. The use of micron-scale primary ion beams will allow detailed sampling of phases within meteorites. Strategies of charge-state selection, molecular disintegration and detection should bring a new set of targets within analytical range. The developing accelerator field is compared to existing (keV energy) ion microprobes

  3. Rare primary headaches: clinical insights.

    Science.gov (United States)

    Casucci, G; d'Onofrio, F; Torelli, P

    2004-10-01

    So-called "rare" headaches, whose prevalence rate is lower than 1% or is not known at all and have been reported in only a few dozen cases to date, constitute a very heterogeneous group. Those that are best characterised from the clinical point of view can be classified into forms with prominent autonomic features and forms with sparse or no autonomic features. Among the former are trigeminal autonomic cephalalgias (TACs) and hemicrania continua, while the latter comprise classical trigeminal neuralgia, hypnic headache, primary thunderclap headache, and exploding head syndrome. The major clinical discriminating factor for the differential diagnosis of TACs is the relationship between duration and frequency of attacks: the forms in which pain is shorter lived are those with the higher frequency of daily attacks. Other aspects to be considered are the time pattern of symptoms, intensity and timing of attacks, the patient's behaviour during the attacks, the presence of any triggering factors and of the refractory period after an induced attack, and response to therapy, especially with indomethacin. Often these are little known clinical entities, which are not easily detected in clinical practice. For some of them, e. g., thunderclap headache, it is always necessary to perform instrumental tests to exclude the presence of underlying organic diseases.

  4. RARE METASTASES OF MALIGNANT MELANOMA

    Directory of Open Access Journals (Sweden)

    Marija Trenkić-Božinović

    2014-09-01

    Full Text Available Melanomas are malignant neoplasms that originate from melanocytes. The most common are on the skin and mucous membranes. Choroidal melanomas are quite different from cutaneous melanomas with regard to presentation, metastases, and treatment. We report two cases of metastatic gastric malignant melanoma of the eye and skin, with reference to the literature. The first patient was a woman aged 23 years, who underwent gastrectomy 22 months after enucleation of the eye due to malignant choroid melanoma. The second patient was a man, 72 years old, who underwent surgery 28 months before because of malignant melanoma of the skin of the forehead. Paraffin sections, 4 μm thick were stained using a classic method, as well as immunohistochemical DAKO APAAP method, using a specific S - 100 antibody and Melan A antibodies. The stomach is considered a rare place for the development of metastases. Metastases in the stomach are often limited to the submucosal as well as the serousmuscular layer, as noted in one of our patients. Metastatic melanoma of the gastrointestinal tract should be suspected in any patient with a history of malignant melanoma and new gastrointestinal symptoms. Because of the similarity between certain common histopathological types of malignant melanoma, primarily achromatic, and types of primary cancers of the stomach, the following immunohistochemical studies are needed: Melan A and S - 100 protein ( markers of malignant melanoma , as well as mucins: MUC5AC, MUC2 and CDX2 ( markers of different types of primary gastric carcinoma.

  5. Tale of two rare diseases

    Directory of Open Access Journals (Sweden)

    Ravindra Shukla

    2013-01-01

    Full Text Available Idiopathic Hypogonadotropic hypogonadism (IHH phenotype is variable & various genes have been decribed in association with IHH.We describe association of IHH with mosaic trisomy 13. A 20 year old male presented with lack of development of secondary sexual characters, normal height, micropenis, small testes, gynaecomastia, absence of axillary and pubic hairs, hyposmia,synkinesis, bilateral horizontal nystagmus and high arched palate. Investigations showed low gonadotropin,low total testosterone, LH after stimulation with 100 mcg tryptorelin sc was 11.42 mU/mL at 40 min. MRI of hypothalamo-pituitary region showed normal olfactory bulb and tract but shallow olfactory sulcus . Karyotype showed homologous Robertsonian translocation of chromosome 13. This case fits classical IHH except for LH rise on stimulation.Features of Patau syndrome which is associated with trisomy 13 are absent in our case. Mosaic trisomy 13, which can otherwise be rare incidental finding , has not been described in association with IHH.Causal association of novel mutation on chromosome 13 leading to aforementioned phenotype cannot be rule out.

  6. Expert services for rare anaemias across Europe

    Directory of Open Access Journals (Sweden)

    Beatrice Gulbis

    2013-03-01

    Full Text Available New challenges and priorities are given in the EU Health programme 2007-2013. The objectives of the programme are to improve citizens’ health security, to promote health to improve prosperity and solidarity, and to generate and disseminate health knowledge. If challenges and priorities have been defined globally for rare diseases by the European Commission, persons involved in rare anaemias have taken the opportunity to contribute to the empowerment of patients with rare anaemias. One of the ENERCA partners objectives was the mapping of existing centres that take care of patients with rare anaemias in Europe. Another goal was to obtain a directory of facilities available per centre for patients with rare anaemias. We thought that with those results it could realistically help to define a consensus regarding the criteria to be recognised as a centre of expertise for haemoglobinopathies and very rare anaemias.

  7. Raman scattering of rare earth hexaborides

    International Nuclear Information System (INIS)

    Ogita, Norio; Hasegawa, Takumi; Udagawa, Masayuki; Iga, Fumitoshi; Kunii, Satoru

    2009-01-01

    Raman scattering spectra were measured for the rare-earth hexaborides RB 6 (R = Ce, Gd, or Dy). All Raman-active phonons due to B 6 vibrations were observed in the range 600 - 1400 cm -1 . Anomalous peaks were detected below 200 cm -1 , which correspond to vibrations of rare-earth ion excited by second-order Raman scattering process. The intensity and energy of the rare-earth mode decrease with decreasing temperature. This suggests that the rare-earth ion vibrates in a shallow and anharmonic potential due to the boron cage. Using the reported values of mean square displacement of rare-earth ion, we estimated the anharmonic contribution for the rare-earth vibrations.

  8. Rare earth metals for automotive exhaust catalysts

    International Nuclear Information System (INIS)

    Shinjoh, Hirohumi

    2006-01-01

    The usage of rare earth metals for automotive exhaust catalysts is demonstrated in this paper. Rare earth metals have been widely used in automotive catalysts. In particular, three-way catalysts require the use of ceria compounds as oxygen storage materials, and lanthana as both a stabilizer of alumina and a promoter. The application for diesel catalysts is also illustrated. Effects of inclusion of rare earth metals in automotive catalysts are discussed

  9. Rare earth industries: Strategies for Malaysia

    International Nuclear Information System (INIS)

    2011-01-01

    Evidently, many reports cite Malaysia as having reasonably substantial amounts of rare earths elements. In fact, based on the rare earths found in the residual tin deposits alone, Malaysia has about 30,000 tonnes. This does not take into account unmapped deposits which experts believe may offer more tonnages of rare earths. Brazil which is reported to have about 48,000 tonnes has announced plans to invest aggressively in the rare earths business. China has on record the largest reserves with about 36 million tonnes. This explains why China has invested heavily in the entire value chain of the rare earths business. Chinas committed investment in rare earths started many years ago when the country's foremost leaders proclaimed the strategic position of rare earths in the world economy. That forecast is now a reality where the rise in the green high-tech economy is seen driving global demand for rare earths in a big way. Malaysia needs to discover and venture into new economic growth areas. This will help fuel the country's drive to achieve a high income status by 2020 as articulated in the New Economic Model (NEM) and the many supporting Economic Transformation Plans that the Government has recently launched. Rare earths may be the new growth area for Malaysia. However, the business opportunities should not just be confined to the mining, extraction and production of rare earths elements alone if Malaysia is to maximise benefits from this industry. The industry's gold mine is in the downstream products. This is also the sector that China wants to expand. Japan which now controls about 50 % of the global market for downstream rare earths-based high-tech components is desperately looking for partners to grow their stake in the business. Malaysia needs to embark on the right strategies in order to build the rare earths industry in the country. What are the strategies? (author)

  10. Expert services for rare anaemias across Europe

    OpenAIRE

    Beatrice Gulbis

    2013-01-01

    New challenges and priorities are given in the EU Health programme 2007-2013. The objectives of the programme are to improve citizens’ health security, to promote health to improve prosperity and solidarity, and to generate and disseminate health knowledge. If challenges and priorities have been defined globally for rare diseases by the European Commission, persons involved in rare anaemias have taken the opportunity to contribute to the empowerment of patients with rare anaemias. One o...

  11. Why some plant species are rare.

    Science.gov (United States)

    Wieger Wamelink, G W; Wamelink, G W Weiger; Goedhart, Paul W; Frissel, Joep; Frissel, Josep Y

    2014-01-01

    Biodiversity, including plant species diversity, is threatened worldwide as a result of anthropogenic pressures such as an increase of pollutants and climate change. Rare species in particular are on the verge of becoming extinct. It is still unclear as to why some plant species are rare and others are not. Are they rare due to: intrinsic reasons, dispersal capacity, the effects of management or abiotic circumstances? Habitat preference of rare plant species may play an important role in determining why some species are rare. Based on an extensive data set of soil parameters we investigated if rarity is due to a narrow habitat preference for abiotic soil parameters. For 23 different abiotic soil parameters, of which the most influential were groundwater-table, soil-pH and nutrient-contents, we estimated species responses for common and rare species. Based on the responses per species we calculated the range of occurrence, the range between the 5 and 95 percentile of the response curve giving the habitat preference. Subsequently, we calculated the average response range for common and rare species. In addition, we designed a new graphic in order to provide a better means for presentation of the results. The habitat preferences of rare species for abiotic soil conditions are significantly narrower than for common species. Twenty of the twenty-three abiotic parameters showed on average significantly narrower habitat preferences for rare species than for common species; none of the abiotic parameters showed on average a narrower habitat preference for common species. The results have major implications for the conservation of rare plant species; accordingly management and nature development should be focussed on the maintenance and creation of a broad range of environmental conditions, so that the requirements of rare species are met. The conservation of (abiotic) gradients within ecosystems is particularly important for preserving rare species.

  12. Seckel syndrome with severe sinus bradycardia.

    Science.gov (United States)

    Ramasamy, Chandramohan; Satheesh, Santhosh; Selvaraj, Raja

    2015-03-01

    Seckel syndrome is an uncommon form of microcephalic dwarfism. The authors report a young boy with Seckel syndrome who presented with severe sinus bradycardia with symptoms of syncope and presyncope. Implantation of a permanent pacemaker was necessary in view of the severe symptoms. Although uncommon, cardiac abnormalities have been rarely reported in Seckel syndrome. This is the one of the few reports of rhythm abnormalities in this condition.

  13. Alaska's rare earth deposits and resource potential

    Science.gov (United States)

    Barker, James C.; Van Gosen, Bradley S.

    2012-01-01

    Alaska’s known mineral endowment includes some of the largest and highest grade deposits of various metals, including gold, copper and zinc. Recently, Alaska has also been active in the worldwide search for sources of rare earth elements (REE) to replace exports now being limitedby China. Driven by limited supply of the rare earths, combined with their increasing use in new ‘green’ energy, lighting, transportation, and many other technological applications, the rare earth metals neodymium, europium and, in particular, the heavy rare earth elements terbium, dysprosium and yttrium are forecast to soon be in critical short supply (U.S. Department of Energy, 2010).

  14. Genetic and Rare Diseases Information Center (GARD)

    Data.gov (United States)

    Federal Laboratory Consortium — NCATS collaborates with the National Human Genome Research Institute (NHGRI) to support GARD, a center designed to provide comprehensive information about rare and...

  15. Haemangiopericytoma of greater omentum. A rare cause of acute abdominal pain.

    Science.gov (United States)

    Bovino, A; Basso, L; Di Giacomo, G; Codacci Pisanelli, M; Basile, U; De Toma, G

    2003-12-01

    Haemangiopericytoma (HPT) is a rare neoplasm that can occur in any part of the human body. In this report, we describe the case of a patient with sudden severe upper abdominal pain caused by primary HPT in the greater omentum.

  16. Rare Books As Teaching Tools

    Science.gov (United States)

    Gino, M. C.; Wise, G.

    2003-05-01

    The use of historic science illustrations in the classroom offers unique opportunities to meet the National Science Standard that "students should develop understanding of science as a human endeavor, of the nature of scientific knowledge, and of historical perspectives" (Content Standard G, Science Education Standards, 1996, National Academy Press, Washington, DC). The Dudley Observatory has launched an effort to use its outstanding collection of rare astronomy books to meet this challenge. The example featured here is the illustration "Systema Solare et Planetarium" from the book Atlas novus coelestis (1742) by Johann Gabriel Doppelmayr (1671-1750). This illustration is significant in the evolution of astronomy because it is one of the first popular depictions of the solar system picturing the planets in their accurate relative sizes and providing numerical estimates of planetary and solar dimensions and distances. Perhaps at least as important, from the educational viewpoint, it is visually appealing, culturally intriguing and filled with puzzling items that might serve as the basis for inquiry-based learning. For example, why is the page sprinkled with what appear to be appeals to theology ("Ex His Creatorem") and expressions of wonder or even horror ("perceptum horridem")? Why does its map of the world depict California as an island? A structure for using this and other historic illustrations in the classroom might be based on the following general questions: What is the purpose of the illustration? What is included that a modern scientist might leave out, or left out that a modern scientist might include? How accurate are the quantitative results presented? How does the conceptual treatment resemble and differ from modern treatments? Viewing the heavens as an 18th century astronomer wanted his public to see them is an excellent approach to achieving the humanistic and historical perspective that the educational standard seeks.

  17. Study of charmonium rare decays

    International Nuclear Information System (INIS)

    Brient, J.C.

    1986-09-01

    This thesis presents the study of rare decays of charmonium states formed in the interaction of an antiproton beam with an hydrogen gas jet target. Electromagnetic final states are used to sign the charmonium state formation (e + e - , e + e - + Χ, γγ). The selection of events used a two arms non magnetic spectrometer, with a charged track system, a threshold Cerenkov counter to tag the electron (positron), and an e.m. calorimeter. Energy scan technic have been used to observe the resonant formation through the excitation curves. Parameters of the states (mass, total and partial widths) are extracted from these curves using a statistical analysis. Two types of decays have been studied in this thesis: 1 P 1 charmonium state decay to the ψ (signed by its e + e - decay). In the energy scan around the center of gravity of the P charmonium states, we observe a cluster of 5 events, in a narrow mass range. This cluster correspond to a 2.7 σ signal. The most probable interpretation of this signal is given by a narrow resonance, with a mass of 3526. MeV. Due to the properties (mass, width and decay) of this signal, this could be interpreted as the 1 P 1 charmonium state. 2 photons decay of the η c and Χ 2 . 22 γγ events are observed, 15 in the η c region, and 7 in the Χ 2 region. This sample is interpreted as a direct observation of η c and Χ 2 decay into γγ. Parameters of these decays, (γγ partial width), are extracted using a maximum likekihood analysis. Theoretical models of charmonium explain correctly the properties of the charmonium, including the results presented in this thesis. 57 refs [fr

  18. A rare cause of osteonecrosis

    Directory of Open Access Journals (Sweden)

    Paolo Agostinis

    2012-01-01

    Full Text Available IntroductionHereditary hemochromatosis (HH is an autosomal recessive disorder caused by mutations in the HFE gene, which increase intestinal iron absorption. The prevalence of C282Y homozygosity, which causes the disorder, is 0.5% in Caucasian populations. The clinical manifestations are related to excess iron in the tissues, especially the liver, heart, pancreas, pituitary, and skin. They include fatigue, loss of libido or impotence in males, liver disease, skin pigmentation, diabetes mellitus, cardiac enlargement—with or without heart failure, and conduction defects. The classic triad of cirrhosis, diabetes mellitus, and skin pigmentation (“bronze diabetes” results from a combination of iron deposits and melanin. It occurs late in the disease, when the total body iron content is more than five times the normal value, about 20 grams. Left untreated, approximately half of all patients with HH eventually develop arthralgia or arthropathy. Chondrocalcinosis, chronic pseudo-osteoarthritis, and osteoporosis are the major rheumatic manifestations of HH. The cause of the arthropathy is still unknown. Iron deposits within joints may trigger a number of pathologic events, such as free radical generation and crystal deposition, which stimulate immune complex formation and inflammation.Materials and methodsWe describe the case of a 48-year-old male suffering from chronic bilateral ankle pain.ResultsThe work-up revealed osteonecrosis of ankle. The patient also presented high plasma ferritin levels and homozygosity for the C282Y mutation. Other than HH, which was confirmed by liver biopsy, the patient had no other risk factors for osteonecrosis.DiscussionHH represents a rare cause of osteonecrosis, and there are no prior reports of aseptic osteonecrosis of the ankle in a patient with this disease. The pathogenetic mechanism remains unknown.

  19. Pheochromocytoma: a rare cause of childhood hypertensive encephalopathy

    International Nuclear Information System (INIS)

    Aftab, S.; Yasmeen, T.; Hamid, M.H.; Sarwar, M.; Sipra, H.; Sheikh, A.; Haider, N.; Hanif, G.

    2012-01-01

    Pheochromocytomas are rare neuroendocrine tumours of chromaffin tissues. They are catecholamine secreting tumours which cause severe hypertension and other systemic disturbances. Of all the causes of childhood hypertension, pheochromocytoma constitutes less than 1%. We report the case of a 12 years old child who presented with hypertensive encephalopathy, confirmed histologically to be secondary to pheochromocytoma, and cured with meticulous critical care and surgical resection. (author)

  20. Random magnetism in amorphous rare-earth alloys (invited)

    Science.gov (United States)

    Sellmyer, D. J.; Nafis, S.

    1985-04-01

    Several aspects of the magnetic transitions seen in rare-earth metallic glasses are discussed, particularly with reference to recent theoretical work. These include: (a) apparent double transitions observed in Gd glasses where exchange fluctuations are important, (b) evidence for a correlated speromagnetic state recently predicted by Chudnovsky and Serota, and (c) the analysis of a Tb glass with strong random anisotropy in terms of an Ising-type spin-glass transition.

  1. Eosinophilic Gastroenteritis as a Rare Cause of Recurrent Epigastric Pain

    Directory of Open Access Journals (Sweden)

    Mohammad Taghi Safari

    2016-04-01

    Full Text Available Eosinophilic gastroenteritis (EGE is a rare inflammatory disorder of gastrointestinal tract characterized by eosinophilic infiltration of the bowel wall. It can mimic many gastrointestinal disorders due to its wide spectrum of presentations. Diagnose is mostly based on excluding other disorders and a high suspicion. Here we report a case of 26 year old man with a history of sever epigastric pain followed by nausea, vomiting since a few days before admission with final diagnosis of EGE.

  2. Sirenomelia: A Rare Case of Foetal Congenital Anomaly

    OpenAIRE

    Dharmraj, Meena; Gaur, Sumitra

    2012-01-01

    Sirenomelia, alternatively known as ?mermaid syndrome? is a very rare congenital deformity in which the legs are fused together, giving them the appearance of the tail of a mermaid?. Other birth defects are always associated with sirenomelia, most commonly abnormalities of the kidneys, large intestines, and genitalia. The present case is a one of sirenomelia associated with an absent right kidney, mild left hydronephrosis, single umbilical artery, and severe oligohydramnios. We discuss the fi...

  3. Sirenomelia: a rare case of foetal congenital anomaly.

    Science.gov (United States)

    Dharmraj, Meena; Gaur, Sumitra

    2012-10-01

    Sirenomelia, alternatively known as 'mermaid syndrome' is a very rare congenital deformity in which the legs are fused together, giving them the appearance of the tail of a mermaid'. Other birth defects are always associated with sirenomelia, most commonly abnormalities of the kidneys, large intestines, and genitalia. The present case is a one of sirenomelia associated with an absent right kidney, mild left hydronephrosis, single umbilical artery, and severe oligohydramnios. We discuss the findings, relative to the present literature and related etiopathogenesis.

  4. Humeral Metastasis from Cervical Cancer: A Rare Case Report

    OpenAIRE

    Sonia Chhabra; KanikaTaneja; Megha Ralli; Sunita Singh; Aditi Arora; Sohrab Arora; Pansi Gupta

    2015-01-01

    Long bone metastasis in cervical cancer is a rare presentation generally seen in the lumbar column or ribs. The reported rates of bone metastases are between 15%-29%. It is associated with poor prognosis. Bone scan and magnetic resonance imaging are useful techniques for diagnosis. In this case report, a 32-year old female with a previous history of cervical carcinoma FIGO stage IIIA presented with severe pain and swelling in her right humerus. X-ray and magnetic resonance imag...

  5. Oxygen stabilized rare-earth iron intermetallic compounds

    International Nuclear Information System (INIS)

    Dariel, M.P.; Malekzadeh, M.; Pickus, M.R.

    1975-10-01

    A new, oxygen-stabilized intermetallic compound was identified in sintered, pre-alloyed rare-earth iron powder samples. Its composition corresponds to formula R 12 Fe 32 O 2 and its crystal structure belongs to space group Im3m. The presence of these compounds was observed, so far, in several R--Fe--O systems, with R = Gd, Tb, Dy, Ho, Er, and Y

  6. General geochemical properties and abundances of the rare earth elements

    International Nuclear Information System (INIS)

    Henderson, P.

    1984-01-01

    This chapter reviews some of the fundamental aspects of rare earth elements (REE) geochemistry and gives data on abundances in the solar system, the bulk Earth and the Earth's crust. It describes the state of knowledge on the partitioning of the REE, especially in igneous rock systems, and cites reference works concerned with the REE. Several chemical properties of REE are discussed (oxidation states; redox conditions; element coordination and ionic radii; element substitution). (Auth.)

  7. Chemical analysis of rare earth elements

    International Nuclear Information System (INIS)

    Tsukahara, Ryoichi; Sakoh, Takefumi; Nagai, Iwao

    1994-01-01

    Recently attention has been paid to ICP-AES or ICP-MS, and the reports on the analysis of rare earth elements by utilizing these methods continue to increase. These reports have become to take about 30% of the reports on rare earth analysis, and this is because these methods are highly sensitive to rare earth elements, and also these methods have spread widely. In ICP-AES and ICP-MS, mostly solution samples are measured, therefore, solids must be made into solution. At the time of quantitatively determining the rare earth elements of low concentration, separation and concentration are necessary. Referring to the literatures reported partially in 1990 and from 1991 to 1993, the progress of ICP-AES and ICP-MS is reported. Rare earth oxides and the alloys containing rare earth elements are easily decomposed with acids, but the decomposition of rocks is difficult, and its method is discussed. The separation of the rare earth elements from others in geochemical samples, cation exchange process is frequently utilized. Also solvent extraction process has been studied. For the separation of rare earth elements mutually, chromatography is used. The spectral interference in spectral analysis was studied. The comparison of these methods with other methods is reported. (K.I)

  8. Rare earth oxyhydrides and preparation process

    International Nuclear Information System (INIS)

    Diaz, H.

    1986-01-01

    Rare earth oxyhydrides of formula RE 1-q Th q Ni 5-p M p O x H y are claimed. RE is a rare earth, Th can be replaced by Yt, M is Cu, Mn, Al, Fe, Cr or Co, o O C and the hydrides are oxidized. They are catalysts for various chemical reactions [fr

  9. Process for lead removal from rare earth

    International Nuclear Information System (INIS)

    Bollat, A.; Sabot, J.L.

    1987-01-01

    An aqueous solution of rare earth chlorides and lead chlorides, with a chloride concentration of at least 2 moles/liter and a pH between 2 and 4, is extracted by an alkylphosphonic acid ester and rare earth(s) is (are) recovered from the organic phase [fr

  10. [A rare form of granuloma annulare].

    Science.gov (United States)

    Bogdanowski, T; Wygledowska-Kania, M

    1995-01-01

    We present a four-year-old girl with a doubly rare form of granuloma annulare with non-typical localisation of superficial nodules on the palms and predisposition to ulceration which is very rare in this type of superficial nodules. The diagnosis was proved by histological examination. After the local cryotherapy (ethyl chloride) the lesions almost completely disappeared.

  11. Pseudomyxoma peritonei (PMP) – a rare entity

    African Journals Online (AJOL)

    Department of Radiology, University of Pretoria. Corresponding author: Z Ebrahim (zaeem@iafrica.com). Pseudomyxoma peritonei (PMP) is a rare complication of mucinous tumours of appendiceal or ovarian origin that results in peritoneal and omental implants. In addition to the appendix and the ovary, other rare.

  12. Theory of Rare-Earth Alloys

    DEFF Research Database (Denmark)

    Lindgård, Per-Anker

    1977-01-01

    A mean-field random alloy theory combined with a simple calculation of the exchange interaction J(c,Q) is shown to quantitatively account for the phase diagrams for alloys of rare-earth metals with Y, Lu, Sc, and other rare-earth metals. A concentration-dependent J(c,Q) explains the empirical 2...

  13. Rare Earth Elements Distribution in Beryl

    International Nuclear Information System (INIS)

    El Gawish, H.K.; Nada, N.; Ghaly, W.A.; Helal, A.I.

    2012-01-01

    Laser ablation method is applied to a double focusing inductively coupled plasma mass spectrometer to determine the rare earth element distribution in some selected beryl samples. White, green and blue beryl samples are selected from the Egyptian eastern desert. Distributions of chondrite- normalized plot for the rare earth element in the selected beryl samples are investigated

  14. Omphalocele with Dextrocardia - A Rare Association

    OpenAIRE

    Vikal Chandra Shakya; C S Agrawal; N R Shrestha; K Dhungel; S Adhikary

    2009-01-01

    Omphalocele is frequently associated with many other congenital malformations. In cardiac anomalies, association of omphalocele with dextrocardia has been rarely noticed before. We present here a child with dextrocardia and omphalocele alongwith a brief review of the literature on this rare association. Key Words: congenital malformations, dextrocardia, omphalocele

  15. Rare thoracic cancers, including peritoneum mesothelioma

    NARCIS (Netherlands)

    Siesling, Sabine; van der Zwan, Jan Maarten; Izarzugaza, Isabel; Jaal, Jana; Treasure, Tom; Foschi, Roberto; Ricardi, Umberto; Groen, Harry; Tavilla, Andrea; Ardanaz, Eva

    Rare thoracic cancers include those of the trachea, thymus and mesothelioma (including peritoneum mesothelioma). The aim of this study was to describe the incidence, prevalence and survival of rare thoracic tumours using a large database, which includes cancer patients diagnosed from 1978 to 2002,

  16. Rare thoracic cancers, including peritoneum mesothelioma

    NARCIS (Netherlands)

    Siesling, Sabine; Zwan, J.M.V.D.; Izarzugaza, I.; Jaal, J.; Treasure, T.; Foschi, R.; Ricardi, U.; Groen, H.; Tavilla, A.; Ardanaz, E.

    2012-01-01

    Rare thoracic cancers include those of the trachea, thymus and mesothelioma (including peritoneum mesothelioma). The aim of this study was to describe the incidence, prevalence and survival of rare thoracic tumours using a large database, which includes cancer patients diagnosed from 1978 to 2002,

  17. International strategic minerals inventory summary report; rare-earth oxides

    Science.gov (United States)

    Jackson, W.D.; Christiansen, Grey

    1993-01-01

    Bastnaesite, monazite, and xenotime are currently the most important rare-earth minerals. Bastnaesite occurs as a primary mineral in carbonatites. Monazite and xenotime also can be found in primary deposits but are recovered principally from heavy-mineral placers that are mined for titanium or tin. Each of these minerals has a different composition of the 15 rare-earth elements. World resources of economically exploitable rare-earth oxides (REO) are estimated at 93.4 million metric tons in place, composed of 93 percent in primary deposits and 7 percent in placers. The average mineral composition is 83 percent bastnaesite, 13 percent monazite, and 4 percent of 10 other minerals. Annual global production is about 67,000 metric tons of which 41 percent is from placers and 59 percent is from primary deposits; mining methods consist of open pits (94 percent) and dredging (6 percent). This output could be doubled if the operations that do not currently recover rare earths would do so. Resources are more than sufficient to meet the demand for the predictable future. About 52 percent of the world's REO resources are located in China. Ranking of other countries is as follows: Namibia (22 percent), the United States (15 percent), Australia (6 percent), and India (3 percent); the remainder is in several other countries. Conversely, 38 percent of the production is in China, 33 percent in the United States, 12 percent in Australia, and 5 percent each in Malaysia and India. Several other countries, including Brazil, Canada, South Africa, Sri Lanka, and Thailand, make up the remainder. Markets for rare earths are mainly in the metallurgical, magnet, ceramic, electronic, chemical, and optical industries. Rare earths improve the physical and rolling properties of iron and steel and add corrosion resistance and strength to structural members at high temperatures. Samarium and neodymium are used in lightweight, powerful magnets for electric motors. Cerium and yttrium increase the

  18. Hepatitis E-induced severe myositis.

    Science.gov (United States)

    Mengel, Annerose M; Stenzel, Werner; Meisel, Andreas; Büning, Carsten

    2016-02-01

    Hepatitis E virus (HEV) is endemic in Asian and African countries but is rarely reported in Western countries. Although there are some prominent neurological manifestations, HEV is rarely recognized by neurologists. This is a case report of myositis induced by HEV. We report the life-threatening case of a 57-year-old man with flaccid tetraparesis due to myositis, acute hepatitis, and renal failure caused by HEV infection. Muscle biopsy revealed scattered myofiber necrosis with a diffuse, mild lymphomonocytic infiltrate in the endomysium and perimysium. Because the patient suffered from an acute HEV infection with a rapidly progressive course of severe myopathy, we started ribavirin treatment. He recovered partially within 3 weeks and recovered fully within 6 months. This case highlights a neurological manifestation of endemic HEV infection with severe myositis in a patient with alcoholic chronic liver disease. Ribavirin treatment is effective in severe HEV infection and may also lead to rapid neurological recovery. © 2015 Wiley Periodicals, Inc.

  19. Rare Earth Metals: Resourcefulness and Recovery

    Science.gov (United States)

    Wang, Shijie

    2013-10-01

    When we appreciate the digital revolution carried over from the twentieth century with mobile communication and the Internet, and when we enjoy our high-tech lifestyle filled with iDevices, hybrid cars, wind turbines, and solar cells in this new century, we should also appreciate that all of these advanced products depend on rare earth metals to function. Although there are only 136,000 tons of annual worldwide demand, (Cho, Rare Earth Metals, Will We Have Enough?)1 rare earth metals are becoming such hot commodities on international markets, due to not only to their increasing uses, including in most critical military hardware, but also to Chinese growth, which accounts for 95% of global rare earth metal production. Hence, the 2013 technical calendar topic, planned by the TMS/Hydrometallurgy and Electrometallurgy Committee, is particularly relevant, with four articles (including this commentary) contributed to the JOM October Issue discussing rare earth metals' resourcefulness and recovery.

  20. Delayed access to treatments for rare diseases: who's to blame?

    Science.gov (United States)

    Feltmate, Karen; Janiszewski, Peter M; Gingerich, Sheena; Cloutier, Michael

    2015-04-01

    The development and commercialization of drugs for rare diseases, termed 'orphan drugs', has historically been economically unattractive. However, because of the introduction of legislation that provides financial and regulatory incentives for the development of orphan drugs, new developments are making their way through the regulatory approval processes. Unfortunately, delays in availability of new drugs for treating rare disease continue to persist. This paper reviews the approach of several regulatory jurisdictions to orphan drugs in an effort to determine their relative effectiveness in providing patient access. Generally speaking, regulatory authorities across jurisdictions have recognized the need to enhance timely access to safe, effective treatment for patients with rare diseases and have been able to shift the approval timelines for access to new care. The greater impediment to orphan drug access appears to be funding, particularly in publicly sponsored health-care systems. Redundancies in federal and provincial reviews of orphan drugs can result in significant delays in access to new drugs. Clearly, more must be done to accelerate access to the treatments so desperately needed by patients. Public payers must be held accountable for their process and decisions--especially for rare disease therapies. © 2015 Asian Pacific Society of Respirology.

  1. Advanced system for separation of rare-earth fission products

    International Nuclear Information System (INIS)

    Baker, J.D.; Gehrke, R.J.; Greenwood, R.C.; Meikrantz, D.H.

    1982-01-01

    A microprocessor-controlled radiochemical separation system has been further advanced to separate individual rare-earth elements from mixed fission products in times of a few minutes. The system was composed of an automated chemistry system fed by two approximately 300 μg 252 Cf sources coupled directly by a He-jet to transport the fission products. Chemical separations were performed using two high performance liquid chromatography columns coupled in series. The first column separated the rare-earth group by extraction chromatography using dihexyldiethylcarbamoylmethylphosphonate (DHDECMP) adsorbed on Vydac C 8 resin. The second column isolated the individual rare-earth elements by cation exchange chromatography using Aminex A-9 resin with α-hydroxyisobutyric acid (α-HIBA) as the eluent. Significant results, which have been obtained to date with this advanced system, are the identification of several new neutron-rich rare-earth isotopes including 155 Pm (T=48+-4 s) and 163 Gd (T=68+-3 s). In addition, a half-life of 41+-4 s is reported for 160 Eu. (author)

  2. A Rare Cause of Upper Airway Obstruction in a Child

    Directory of Open Access Journals (Sweden)

    H. Ahmed

    2017-01-01

    Full Text Available Ventricular band cyst is a rare condition in children but can result in severe upper airway obstruction with laryngeal dyspnea or death. The diagnosis should be considered in any stridor in children with previous history of intubation or respiratory infections. We report a case of a 4-year-old girl, received in an array of severe respiratory distress, emergency endoscopy was done, and a large ventricular tape band cyst obstructing the air way was found. Complete excision was made, and postoperative prophylaxis tracheotomy was done. The postoperative course was uneventful with improvement of clinical and endoscopic signs.

  3. Rare earths and rare earth alloys electrolytic preparation process and device for this process

    International Nuclear Information System (INIS)

    Seon, F.; Barthole, G.

    1986-01-01

    Electrolysis of a molten salt of rare earth or rare earth alloy for preparation of the metal or alloy is described. The molten salt bath comprises at least a rare earth chloride, at least an alkaline or alkaline earth chloride and at least an alkaline or alkaline earth fluoride [fr

  4. Magnetic Rare-Earth Superlattices

    DEFF Research Database (Denmark)

    Majkrzak, C.F.; Gibbs, D.; Böni, P.

    1988-01-01

    The magnetic structures of several single‐crystal, magnetic rare‐earth superlattice systems grown by molecular‐beam epitaxy are reviewed. In particular, the results of recent neutron diffraction investigations of long‐range magnetic order in Gd‐Y, Dy‐Y, Gd‐Dy, and Ho‐Y periodic superlattices...... are presented. In the Gd‐Y system, an antiphase domain structure develops for certain Y layer spacings, whereas modified helical moment configurations are found to occur in the other systems, some of which are commensurate with the chemical superlattice wavelength. References are made to theoretical interaction...

  5. The importance of international collaboration for rare diseases research: a European perspective.

    Science.gov (United States)

    Julkowska, D; Austin, C P; Cutillo, C M; Gancberg, D; Hager, C; Halftermeyer, J; Jonker, A H; Lau, L P L; Norstedt, I; Rath, A; Schuster, R; Simelyte, E; van Weely, S

    2017-09-01

    Over the last two decades, important contributions were made at national, European and international levels to foster collaboration into rare diseases research. The European Union (EU) has put much effort into funding rare diseases research, encouraging national funding organizations to collaborate together in the E-Rare program, setting up European Reference Networks for rare diseases and complex conditions, and initiating the International Rare Diseases Research Consortium (IRDiRC) together with the National Institutes of Health in the USA. Co-ordination of the activities of funding agencies, academic researchers, companies, regulatory bodies, and patient advocacy organizations and partnerships with, for example, the European Research Infrastructures maximizes the collective impact of global investments in rare diseases research. This contributes to accelerating progress, for example, in faster diagnosis through enhanced discovery of causative genes, better understanding of natural history of rare diseases through creation of common registries and databases and boosting of innovative therapeutic approaches. Several examples of funded pre-clinical and clinical gene therapy projects show that integration of multinational and multidisciplinary expertize generates new knowledge and can result in multicentre gene therapy trials. International collaboration in rare diseases research is key to improve the life of people living with a rare disease.

  6. Analyzing rare diseases terms in biomedical terminologies

    Directory of Open Access Journals (Sweden)

    Erika Pasceri

    2012-03-01

    Full Text Available Rare disease patients too often face common problems, including the lack of access to correct diagnosis, lack of quality information on the disease, lack of scientific knowledge of the disease, inequities and difficulties in access to treatment and care. These things could be changed by implementing a comprehensive approach to rare diseases, increasing international cooperation in scientific research, by gaining and sharing scientific knowledge about and by developing tools for extracting and sharing knowledge. A significant aspect to analyze is the organization of knowledge in the biomedical field for the proper management and recovery of health information. For these purposes, the sources needed have been acquired from the Office of Rare Diseases Research, the National Organization of Rare Disorders and Orphanet, organizations that provide information to patients and physicians and facilitate the exchange of information among different actors involved in this field. The present paper shows the representation of rare diseases terms in biomedical terminologies such as MeSH, ICD-10, SNOMED CT and OMIM, leveraging the fact that these terminologies are integrated in the UMLS. At the first level, it was analyzed the overlap among sources and at a second level, the presence of rare diseases terms in target sources included in UMLS, working at the term and concept level. We found that MeSH has the best representation of rare diseases terms.

  7. Research on manufacturing aluminum - rare earth alloy with a high content of rare earth (> 20% RE) from total rare earth oxides by thermit reduction

    International Nuclear Information System (INIS)

    Ngo Trong Hiep; Dam Van Tien; Tran Duy Hai; Ngo Xuan Hung and Ly Thanh Vu

    2004-01-01

    In this report, several theoretical principles of thermit reduction method used for metal oxides to obtain metals, ferroalloys and ligatua with technical purity are presented. Manufacture of aluminum-rare earth alloys by thermit reduction is also described in the report. Data that are generalized based on thermo-kinetic calculation of the thermit reduction and selection of technological flow-sheet based on thermal effect will partly clarify research results in investigating typical features of the process and identify measures to reduce metal loss in discharged slags. (author)

  8. Ethical and social aspects on rare diseases

    Directory of Open Access Journals (Sweden)

    Krajnović Dušanka

    2012-01-01

    Full Text Available Rare diseases are a heterogenic group of disorders with a little in common except of their rarity affecting by less than 5 : 10.000 people. In the world is registered about 6000-8000 rare diseases with 6-8% suffering population only in the European Union. In spite of rarity, they represent an important medical and social problem due to their incidence. For many rare diseases have no treatment, but if it exists and if started on time as being available to patients, there is a good prognosis for them to be able for normal life. The problems of patients affected by rare diseases are related to the lack of diagnosis and timely undergoing as well as their treatment or prevention. Orphan drugs are products intended for treatment, diagnosis or prevention of rare diseases, but for their development and marketing the industry has not been interested in yet because of their marketing reasons. Patients suffering from a rare disease although belonging to the vulnerable group for their specific health needs, is becoming invisible in the health care system due to their additional needs un properly recognized. Ethical problems faced by patients, but also health care professionals are related to the allocation of medical diagnostics, unequal approach to health care, inappropriately specialized social services as well as therapy and rare orphan drugs unavailability. Ethical questions related to clinical trails on orphan drugs, population screening and epidemiology testing on rare diseases will also be discussed in this paper. [Projekat Ministarstva nauke Republike Srbije, br. 41004: Rare diseases: Molecular pathophysiology, the diagnostic and therapeutical modalities, social, ethical and legal aspects

  9. Severe Urticaria Following Erythromycin Therapy

    Directory of Open Access Journals (Sweden)

    Anada S. Prasad

    1960-01-01

    Full Text Available Enythromycin is believed to cause no serlous reactions after large doses, nausea, rarely vomiting and occasionally abdominal pain and diarrhea may occur, (Herrell, 1954. According to' Kagan and Faller (1955 no allergic reaction to' erythromycin has been reported. Phlebitis and thrombosis have been observed, (Shoe maker & Yow, 1954. In a report by Solomon and Johnson, (1955 toxic reactions were recorded as being uncommon. In a large series of cases, only one patient had a rash, another had fever possibly due to the drug, nne had nausea and vomiting and 4 had loose bowel movements while under therapy. Among the 122 patients treated with erythromcin, therapy was stopped owing to side effects in only one. My patient had severe urticaria within 24 hours of beginning therapy with erythromycin and a positive skin test was observed. It was believed to be an allergic reaction caused by the drug.

  10. Rare upper gastrointestinal hemorrhage of cetuximab: A case report.

    Science.gov (United States)

    Duan, Shi-Jie; Gao, Zi-Ming; Wang, Peng-Liang; Gong, Bao-Cheng; Huang, Han-Wei; Luo, Lei; Wang, Xin; Xing, Ya-Nan; Xu, Hui-Mian; Liu, Fu-Nan

    2017-12-01

    cetuximab, an epidermal growth factor receptor inhibitor, is a targeted therapeutic regimen of colorectal cancers. Several common adverse effects have been found, such as cutaneous or gastrointestinal toxicity. However, according to the articles had been published, upper gastrointestinal bleeding (UGIB) is considered to be rare and its mechanism remains unclear. In this report, we presented a 42-year-old male patient with advanced recto-sigmoid cancer. After palliative operation, the patient suffered from complete upper gastrointestinal (GI) obstruction, which was induced by extensive abdominal metastasis of the tumor. Considering his poor condition, we chose the targeted drug, cetuximab, as his further treatment. But after the application of cetuximab, the UGIB immediately happened twice in this patient. UGIB, as a rare complication of cetuximab, occured to the patient. We stopped the bleeding with thrombin, hemocoagulase and somatostatin and suspended the subsequent treatment plan of cetuximab. At the same time, anti-shock treatment was given immediately. He was died of respiratory and circulatory failure caused by UGIB and advanced tumor eventually. UGIB should be considered as a rare but severe complication of cetuximab. When cetuximab is applied for patients with advanced colon tumors, more cautions should be required if the patients are accompanied by upper gastrointestinal obstruction. In addition, for those patients who suffered from UGIB recently, cetuximab should be prohibited if the Rockall score ranged > 5 points. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

  11. Thermochemistry of rare-earth trifluorides

    International Nuclear Information System (INIS)

    Kim, K.Y.; Johnson, C.E.

    1981-01-01

    Using the most recent crystallographic data, the Born-Lande equation was employed to calculate lattice energies of the rare-earth trifluorides. The excellent agreement ( 0 sub(f)(MX 3 ,c,298.15K) can be estimated. The magnitude of the monotonic change of ΔH 0 sub(f)(MX 3 ) for the rare-earth trihalides series (14 4f electrons) is comparable to the energy change between Sc and Ti in which only one 3d electron is added. This energy change is consistent with the chemical evidence that the electrons in the f-orbitals of rare earths contribute negligibly to the bonding. (author)

  12. Rare kaon, muon, and pion decay

    International Nuclear Information System (INIS)

    Littenberg, L.

    1998-01-01

    The author discusses the status of and prospects for the study of rare decays of kaons, muons, and pions. Studies of rare kaon decays are entering an interesting new phase wherein they can deliver important short-distance information. It should be possible to construct an alternative unitarity triangle to that determined in the B sector, and thus perform a critical check of the Standard Model by comparing the two. Rare muon decays are beginning to constrain supersymmetric models in a significant way, and future experiments should reach sensitivities which this kind of model must show effects, or become far less appealing

  13. Ecology and exploration of the rare biosphere.

    Science.gov (United States)

    Lynch, Michael D J; Neufeld, Josh D

    2015-04-01

    The profound influence of microorganisms on human life and global biogeochemical cycles underlines the value of studying the biogeography of microorganisms, exploring microbial genomes and expanding our understanding of most microbial species on Earth: that is, those present at low relative abundance. The detection and subsequent analysis of low-abundance microbial populations—the 'rare biosphere'—have demonstrated the persistence, population dynamics, dispersion and predation of these microbial species. We discuss the ecology of rare microbial populations, and highlight molecular and computational methods for targeting taxonomic 'blind spots' within the rare biosphere of complex microbial communities.

  14. Recovery of rare earths from red mud

    International Nuclear Information System (INIS)

    Bautista, R.G.

    1992-01-01

    The prospect for the recovery of rare earths from red mud, the bauxite tailings from the production of alumina is examined. The Jamaican red mud by far has the higher trace concentrations of lanthanum, cerium, neodymium, and yttrium. Scandium is also present. The dissolution of the rare earth is a major extraction problem because of the large volume of other materials. The recovery processes that have been proposed include the production of co-products such as iron, alumina, and titanium concentrates, with the rare earths going with the titanium. In this paper a critical examination of the possible processes are presented with the recommended research projects to be carried out

  15. Severe Aplastic Anemia (SAA)

    Science.gov (United States)

    ... page Print this page My Cart Severe aplastic anemia (SAA) Severe aplastic anemia (SAA) is a disease ... leukemia (ALL) Other diseases What is severe aplastic anemia (SAA)? SAA is a bone marrow disease. The ...

  16. Cardiac arrest following ventilator fire: A rare cause

    Directory of Open Access Journals (Sweden)

    K Nazeer Ahmed

    2012-01-01

    Full Text Available Operating room fires are rare events, but when occur they result in serious and sometimes fatal consequences. Anaesthesia ventilator fire leading to cardiac arrest is a rare incident and has not been reported. We report a near catastrophic ventilator fire leading to cardiac arrest in a patient undergoing subtotal thyroidectomy. In the present case sparks due to friction or electrical short circuit within the ventilator might have acted as source of ignition leading to fire and explosion in the oxygen rich environment. The patient was successfully resuscitated and revived with uneventful recovery and no adverse sequelae. The cardiac arrest was possibly due to severe hypoxia resulting from inhalation of smoke containing high concentrations of carbon monoxide and other noxious gases.

  17. Thyrotoxic Neuropathy: A rare cause of acute flaccid paraplegia

    Directory of Open Access Journals (Sweden)

    Aiman K. Al-Wahaibi

    2018-01-01

    Full Text Available Acute polyneuropathy is a rare manifestation of severe hyperthyroidism. We report a 22-year-old Omani male who presented to the Sohar Hospital, Sohar, Oman, in 2016 with acute-onset rapidly progressive flaccid areflexic paraplegia as the presenting manifestation of thyrotoxicosis. Nerve conduction studies revealed mixed axonal and demyelinating polyneuropathy in both the motor and sensory nerves. Treatment of the hyperthyroidism with β-blockers and carbimazole along with physiotherapy resulted in the patient’s full recovery and the alleviation of his symptoms. Besides highlighting this rare association, this report underscores the importance of including thyroid function tests in the evaluation of patients with acute polyneuropathy.

  18. Gastric volvulus following diagnostic upper gastrointestinal endoscopy: a rare complication.

    Science.gov (United States)

    Karthikeyan, Vilvapathy Senguttuvan; Sistla, Sarath Chandra; Ram, Duvuru; Rajkumar, Nagarajan

    2014-02-10

    Esophagogastroduodenoscopy (EGD) is a commonly used, safe diagnostic modality for evaluation of epigastric pain and rarely its major complications include perforation, haemorrhage, dysrhythmias and death. Gastric volvulus has been reported to complicate percutaneous endoscopic gastrostomy but its occurrence after diagnostic EGD has not yet been reported in literature. The successful management relies on prompt diagnosis and gastric untwisting, decompression and gastropexy or gastrectomy in full thickness necrosis of the stomach wall. A 38-year-old woman presented with epigastric pain and EGD showed pangastritis. Immediately after EGD she developed increased severity of pain, vomiting and abdominal distension. Emergency laparotomy carried out for peritoneal signs revealed eventration of left hemidiaphragm with the stomach twisted anticlockwise in the longitudinal axis. After gastric decompression and untwisting of volvulus, anterior gastropexy and gastrostomy was carried out. Hence, we report this rare complication of diagnostic endoscopy and review the existing literature on the management.

  19. Rare presentation of pancreatic schwannoma: a case report

    Directory of Open Access Journals (Sweden)

    Tofigh Arash

    2008-08-01

    Full Text Available Abstract Introduction Schwannoma is a rare tumor among pancreatic neoplasms. Schwannomas vary in size, and most of them are cystic, mimicking pancreatic cystic lesions. Generally, a definitive diagnosis is made at the time of histological analysis. The mainstay treatment is surgical resection. Case presentation We report an unusual presentation of pancreatic schwannoma with abdominal pain and several episodes of cholangitis in a 54-year-old Caucasian (Iranian man. The condition was not diagnosed pre-operatively and Whipple's procedure was performed. Conclusion Pancreatic schwannoma is an important clinical entity to include in the differential diagnosis of pancreatic lesions. Pre-operative diagnosis is difficult but computed tomographic findings may be helpful. The tumor may also have atypical and rare presentations, such as cholangitis and weight loss. For benign tumors, simple enucleation is usually adequate, whereas malignant tumors require standard oncological resection.

  20. Recommendations for the tuning of rare event probability estimators

    International Nuclear Information System (INIS)

    Balesdent, Mathieu; Morio, Jérôme; Marzat, Julien

    2015-01-01

    Being able to accurately estimate rare event probabilities is a challenging issue in order to improve the reliability of complex systems. Several powerful methods such as importance sampling, importance splitting or extreme value theory have been proposed in order to reduce the computational cost and to improve the accuracy of extreme probability estimation. However, the performance of these methods is highly correlated with the choice of tuning parameters, which are very difficult to determine. In order to highlight recommended tunings for such methods, an empirical campaign of automatic tuning on a set of representative test cases is conducted for splitting methods. It allows to provide a reduced set of tuning parameters that may lead to the reliable estimation of rare event probability for various problems. The relevance of the obtained result is assessed on a series of real-world aerospace problems

  1. Family studies to find rare high risk variants in migraine.

    Science.gov (United States)

    Hansen, Rikke Dyhr; Christensen, Anne Francke; Olesen, Jes

    2017-12-01

    variants (less than five), while other studies found several possible variants. Not all of them were genome wide significant. Four studies performed follow-up analyses in unrelated cases and controls and calculated odds ratios that supported an association between detected variants and risk of disease. Studies of 11 diseases identified rare variants that segregated fully or to a large degree with the disease in the pedigrees. It is possible to find rare high risk variants for common complex diseases through a family-based approach. One study using a family approach and NGS to find rare variants in migraine has already been published but with strong limitations. More studies are under way.

  2. Rare Neurological Complications After Sleeve Gastrectomy.

    Science.gov (United States)

    Tabbara, Malek; Carandina, Sergio; Bossi, Manuela; Polliand, Claude; Genser, Laurent; Barrat, Christophe

    2016-12-01

    Bariatric surgery is considered to be the most effective treatment of morbid obesity and improvement of obesity-related comorbidities, such as type II diabetes. However, both peripheral and central neurological complications can occur after bariatric surgery. Such complications tend to occur more frequently after bypass surgery than after sleeve gastrectomy (SG). The objective of this study was to identify the patients that presented post-operative neurological complications after undergoing SG and describe the incidence, presentation, and management of these complications. This was a retrospective study of 592 cases of SG performed between 2009 and 2014 with a special focus on patients who presented neurological complications. Of the 592 SG cases, only seven (1.18 %) patients presented neurological complications. All patients had uneventful post-operative course, but all reported feeding difficulties, accompanied by severe dysphagia, and rapid weight loss, with a mean weight loss of 35 kg (30-40 kg) 3 months after SG. All patients were readmitted owing to neurological symptoms that included paresthesia, abolition of deep tendon reflexes of the lower limbs, muscle pain, and motor and sensitive deficits in some cases. There were two cases of Wernicke's encephalopathy. All patients were treated for neuropathy secondary to vitamin B1 deficiency and had a significant improvement and/or resolution of their symptoms. Neurological complications after SG are rare and are often preceded by gastrointestinal symptoms, rapid weight loss, and lack of post-operative vitamin supplementation. Re-hospitalization and multidisciplinary team management are crucial to establish the diagnosis and initiate treatment.

  3. A rare case of bilateral aspergillus endophthalmitis

    Directory of Open Access Journals (Sweden)

    Saurabh Gupta

    2015-12-01

    Full Text Available Aspergillus endophthalmitis is a devastating inflammatory condition of the intraocular cavities that may result in irreparable loss of vision and rapid destruction of the eye. Almost all cases in the literature have shown an identified source causing aspergillus endophthalmitis as a result of direct extension of disease. We present a rare case of bilateral aspergillus endophthalmitis. A 72-year-old woman with a history of diabetes mellitus, congenital Hirschsprung disease, and recent culture-positive candida pyelonephritis with hydronephrosis status post-surgical stent placement presented with difficulty opening her eyes. She complained of decreased vision (20/200 with pain and redness in both eyes – right worse then left. Examination demonstrated multiple white fungal balls in both retinas consistent with bilateral fungal endophthalmitis. Bilateral vitreous taps for cultures and staining were performed. Patient was given intravitreal injections of amphotericin B, vancomycin, ceftazidime, and started on oral fluconazole. Patient was scheduled for vitrectomy to decrease organism burden and to remove loculated areas of infection that would not respond to systemic antifungal agents. Four weeks after initial presentation, the fungal cultures revealed mold growth consistent with aspergillus. Patient was subsequently started on voriconazole and fluconazole was discontinued due to poor efficacy against aspergillus. Further workup was conducted to evaluate for the source of infection and seeding. Transthoracic cardiogram was unremarkable for any vegetation or valvular abnormalities. MRI of the orbits and sinuses did not reveal any mass lesions or bony destruction. CT of the chest was unremarkable for infection. Aspergillus endophthalmitis may occur because of one of these several mechanisms: hematogenous dissemination, direct inoculation by trauma, and contamination during surgery. Our patient's cause of bilateral endophthalmitis was through an

  4. Enzymatic approaches to rare sugar production.

    Science.gov (United States)

    Zhang, Wenli; Zhang, Tao; Jiang, Bo; Mu, Wanmeng

    Rare sugars have recently attracted much attention because of their potential applications in the food, nutraceutical, and pharmaceutical industries. A systematic strategy for enzymatic production of rare sugars, named Izumoring, was developed >10years ago. The strategy consists of aldose-ketose isomerization, ketose C-3 epimerization, and monosaccharide oxidation-reduction. Recent development of the Izumoring strategy is reviewed herein, especially the genetic approaches to the improvement of rare sugar-producing enzymes and the applications of target-oriented bioconversion. In addition, novel non-Izumoring enzymatic approaches are also summarized, including enzymatic condensation, phosphorylation-dephosphorylation cascade reaction, aldose epimerization, ulosonic acid decarboxylation, and biosynthesis of rare disaccharides. Copyright © 2017 Elsevier Inc. All rights reserved.

  5. The BNL rare kaon decay program

    Energy Technology Data Exchange (ETDEWEB)

    Littenberg, L.

    1996-12-31

    The rare kaon decay program at Brookhaven National Laboratory is reviewed. Results from the last round of experiments are briefly discussed. The three experiments currently collecting data are described. Prospects for future experiments are discussed.

  6. Macrodystrophia Lipomatosa: A rare presentation | Kachewar ...

    African Journals Online (AJOL)

    Macrodystrophia lipomatosa is a rare congenital nonhereditary mesenchymal hamartomatous malformation resulting in localized gigantism of parts of extremities that manifests clinically as macrodactyly or megalodactyly. Radiological and Pathological hallmark is the disproportionate fibroadipose tissue proliferation in ...

  7. Therapeutics for Rare and Neglected Diseases

    Data.gov (United States)

    Federal Laboratory Consortium — There are more than 6,500 identified rare and neglected diseases, yet only about 250 treatments are available for these conditions. The limited numbers of patients...

  8. Sintered cobalt-rare earth intermetallic product

    International Nuclear Information System (INIS)

    Benz, M.G.

    1975-01-01

    This patent describes a sintered product having substantially stable permanent magnet properties in air at room temperature. It comprises compacted particulate cobalt--rare earth alloy consisting essentially of a Co 5 R intermetallic phase and a CoR intermetallic phase which is richer in rare earth metal content than the Co 5 R phase, where R is a rare earth metal. The Co 5 R intermetallic phase is present in an amount of at least 65 percent by weight of the sintered product and the CoR intermetallic phase which is richer in rare earth metal content than the Co 5 R phase is present in a positive amount having a value ranging up to about 35 percent by weight of the product. The sintered product has a density of at least 87 percent and has pores which are substantially noninterconnecting and wherein the component grains have an average size less than 30 microns

  9. Thermodynamics of rare earths in steelmaking

    International Nuclear Information System (INIS)

    Vahed, A.; Kay, D.A.R.

    1976-01-01

    The standard free energies of formation of the oxides, sulfides and oxysulfides of cerium and lanthanum under steelmaking conditions have been calculated and used to predict the behavior of rare earths in steelmaking. Deoxidation and desulfurization constants, expressed in terms of Henrian activities, have been used to construct a precipitation diagram which indicates the sequence of rare earth inclusion formation. An enrichment of lanthanum in (RE)-oxysulfide and cerium in (RE)-sulfide is predicted. It is also predicted that rare earths should be able to reduce the soluble oxygen and sulfur contents of liquid steel well below the contents presently found in most industrial and laboratory practices. A simple method of calculating steelmaking additions for complete rare earth control of inclusion composition is presented

  10. International Rare Histiocytic Disorders Registry (IRHDR)

    Science.gov (United States)

    2018-04-18

    Rare Histiocytic Disorders (RHDs); Juvenile Xanthogranuloma (JXG); Reticulohistiocytoma (Epithelioid Histiocytoma); Xanthoma Disseminatum (XD); Multicentric Reticulohistiocytosis (MRH); Systemic Juvenile Xanthogranuloma; Erdheim-Chester Disease (ECD); Multi-system Rosai-Dorfman Disease (RDD)

  11. Eventration of diaphragm with a rare association

    Directory of Open Access Journals (Sweden)

    Syed Shafiq

    2014-01-01

    Full Text Available Eventration of the diaphragm is a rare anomaly of unknown origin characterized by a permanent high position of one or rarely both the leaflets of the diaphragm, providing a potential space for the displacement of abdominal viscera on the affected side(s. The etiology, diagnosis, and management of this condition remains a controversial subject. We report a rare case of infiltrating squamous cell carcinoma of the esophagus in a patient with eventration of the diaphragm and gastric volvulus who presented to us with retrosternal discomfort. To the best of our knowledge, this rare association has not been reported in the literature, although one case of esophageal adenocarcinoma arising from Barrett′s esophagus in association with eventration of the diaphragm has been reported previously. This case again emphasis the varied associations of an eventration of the diaphragm.

  12. Medium Effects in Reactions with Rare Isotopes

    International Nuclear Information System (INIS)

    Bertulani, C A; Karakoç, M

    2012-01-01

    We discuss medium effects in knockout reactions with rare isotopes of weakly-bound nuclei at intermediate energies. We show that the poorly known corrections may lead to sizable modifications of knockout cross sections and momentum dsitributions.

  13. Semi-leptonic and rare decays

    CERN Document Server

    Borzumati, Francesca M; Aoki, M; Bevan, A; Cottingham, W N; Dighe, Amol S; Gambino, Paolo; De Groot, N; Harrison, P F; Khalil, S; Kim, C S; Liniger, P; Misiak, M; Reina, L; Ricciardi, G; Shibata, E I; Uraltsev, N; Wyler, D

    2001-01-01

    We review the theoretical and experimental results on semi-leptonic and rare B decays presented in working group 2 of the UK phenomenology workshop on heavy flavour physics and CP violation in Durham, 2000. (0 refs).

  14. Aspergillus Salpingitis: A Rare Case Report

    African Journals Online (AJOL)

    rare entity, the correct diagnosis is of great importance for the indication of proper therapy. KEY WORDS: America ... Salpingitis is the most common serious infection in women .... immune systems, especially those recipients of stem-cell and.

  15. Fascinating world of rare earth research

    International Nuclear Information System (INIS)

    Gschneidner, K.A. Jr.

    1977-01-01

    The first part of this paper concerns some of the notable events which occurred early in the author's career as a rare earther and some of the major events which took place in the two decades 1950 to 1970. The notable changes and advances in the rare earth research world since the 1971 Durham Conference are described in the second and largest part of the paper. The final portion is concerned with actinide developments since 1971

  16. Urethral Leiomyoma: A Rare Clinical Entity

    Directory of Open Access Journals (Sweden)

    Ng Beng Kwang

    2016-01-01

    Full Text Available Extrauterine leiomyomas are encountered occasionally, which can pose a diagnostic dilemma and challenge to the gynaecologist. We report a rare case of urethral leiomyoma. A 31-year-old woman with history of primary subfertility presented with mass at her urethral meatus and lower urinary tract symptoms. She underwent examination under anaesthesia and excision of the urethral mass. Histopathological examination confirmed leiomyoma. Diagnosis and management of this common growth situated at a rare location were reviewed and discussed.

  17. Rare B decays and new physics studies

    International Nuclear Information System (INIS)

    Long, Owen

    2006-01-01

    I present a review of using rare B decays to search for physics beyond the Standard Model. B decays that proceed either through annihilation or loop topologies at leading order in the Standard Model provide unique probes in the search for new physics. The latest experimental results from the B factories (Babar and Belle) and the Tevatron experiments (CDF and D0) on rare decays and their impact on various scenarios for new physics will be presented. (author)

  18. 12 Ministries Control Rare Earth Exports

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    <正>"It is very natural to reserve rare earth as a strategic resource.Many countries do this,including China."On April 8,Sun Lihui,Vice Director of Metal Section of Chemicals Import & Export Commerce Chamber of China Minmetals Corporation told a reporter that as early as 2006,China has launched a strategic plan for rare earth,"but it was interrupted by the subsequent financial crisis."

  19. Sirenomelia with oesophageal atresia: a rare association.

    Science.gov (United States)

    Sathe, Pragati Aditya; Ghodke, Ratnaprabha Kundlikrao; Kandalkar, Bhuvaneshwari Mahendra

    2014-02-01

    We are reporting a rare case of sirenomelia with oesophageal atresia. Sirenomelia is a lethal sporadic defect of which lower gastrointestinal tract anomalies are characteristic findings. Respiratory and upper gastrointestinal tract malformations like oesophageal atresia occur in about 20-35% of cases. Though its occurrence has been described, it has been reported only rarely. This report aims at describing this uncommon association along with its histological features.

  20. 2004 Top 10 Chinese Rare Earth Events

    Institute of Scientific and Technical Information of China (English)

    2005-01-01

    1. Management to the Investment in Rare Earth IndustryConfirmedIn July 2004, "Decision on the Reform in Investment System" was formally publicized by the State Council of the People's Republic of China. The fifth item in the Decision stipulates that ore exploitation, smelting & separation and rare earth deep-processed projects with total investment over RMB¥100 million should be approved by the investment governing department of the State Council, and that other

  1. Ambras syndrome: A rare case report

    Directory of Open Access Journals (Sweden)

    A Ishita

    2016-01-01

    Full Text Available Congenital generalized hypertrichosis associated with gingival hyperplasia are rare cases published in literature. The frequency incidence of generalized congenital hypertrichosis is about one to billions of people. Hypertrichosis and gingival hyperplasia are termed as Ambras syndrome (AS, which can be noticed at birth or soon after. Here, is a rare case report of 4-year-old male child who presented with generalized hypertrichosis with gingival fibromatosis and dysmorphic facial features.

  2. Mammography with rare earth intensifying screens

    International Nuclear Information System (INIS)

    Maurer, H.J.; Goos, F.

    1987-01-01

    Screens basing on rare earth phosphors with suitable films green or blue sensitive may be used in mammography with grids without diagnostic losses. Highest definition will be obtained with medium densities on film. High-speed screens may reduce dose, but definition is poor. Best compromise between speed and high definition may be reached with relative low thickness of phosphor layers. A system of high definition films (Medichrome) and special rare earth screens give best results. (orig.) [de

  3. Rare earths refining by vacuum sublimation method

    International Nuclear Information System (INIS)

    Rytus, N.N.

    1983-01-01

    The process of rare earths refining by the sUblimation; method in high and superhigh oil-free vacuum, is investigated. The method is effective for rare earths obtaining and permits to prepare metal samples with a high value of electric resistance ratio γ=RsUb(298 K)/Rsub(4.2 K). The estimation of general purity is performed for Sm, Eu, Yb, Tm, Dy, Ho, Er and Se

  4. Rare Z decays and neutrino flavor universality

    Energy Technology Data Exchange (ETDEWEB)

    Durieux, Gauthier [Deutsches Elektronen-Synchrotron (DESY), Hamburg (Germany); Cornell Univ. Ithaca, NY (United States). Lab. for Elementary Particle Physics; Univ. Catholique de Louvain, Louvain-la-Neuve (Belgium). Centre for Cosmology, Particle Physics and Phenomenology; Grossman, Yuval; Kuflik, Erik [Cornell Univ. Ithaca, NY (United States). Lab. for Elementary Particle Physics; Koenig, Matthias [Mainz Univ. (Germany). PRISMA Cluster of Excellence; Mainz Univ. (Germany). Mainz Inst. for Theoretical Physics; Ray, Shamayita [Cornell Univ. Ithaca, NY (United States). Lab. for Elementary Particle Physics; Calcutta Univ. (India). Dept. of Physics

    2015-12-15

    We study rare four-body decays of the Z-boson involving at least one neutrino and one charged lepton. Large destructive interferences make these decays very sensitive to the Z couplings to neutrinos. As the identified charged leptons can determine the neutrino flavors, these decays probe the universality of the Z couplings to neutrinos. The rare four-body processes could be accurately measured at future lepton colliders, leading to percent level precision.

  5. Rare species are valued big time.

    Directory of Open Access Journals (Sweden)

    Elena Angulo

    Full Text Available BACKGROUND: It has recently been postulated that the value humans place on rarity could cause the extinction of rare species. This is because people are willing to pay the high costs of exploiting the last individuals. Many hobbies, such as ecotourism or the keeping of exotic pets may cause this effect--known as the anthropogenic Allee effect. However, the entire theory relies on the insofar undemonstrated assumption that people do value rarity. METHODOLOGY/PRINCIPAL FINDINGS: In order to quantify how much people valued rare species relative to common ones, we created online slideshows of photographs of either rare or common species on an Internet web site. The slideshow with photographs of rare species attracted more visitors, and visitors spent, in general, more time waiting to view it. CONCLUSIONS/SIGNIFICANCE: We provide evidence that people value rare more than common species. As we did not target consumers of a specific market, this finding suggests that the anthropogenic Allee effect is likely be driven by a large part of the population. Given the substantial participation in our online experiment, we highlight the potential of the world wide web resource as a tool for conservation action. However, the evidence presented here that the general public value rare species, combined with the assumption that anthropogenic Allee effect is operating, implies that conservationists should be prudent when using rarity to promote conservation.

  6. Sintered cobalt-rare earth intermetallic product

    International Nuclear Information System (INIS)

    Benz, M.C.

    1975-01-01

    A process is described for preparing novel sintered cobalt--rare earth intermetallic products which can be magnetized to form permanent magnets having stable improved magnetic properties. A cobalt--rare earth metal alloy is formed having a composition which at sintering temperature falls outside the composition covered by the single Co 5 R intermetallic phase on the rare earth richer side. The alloy contains a major amount of the Co 5 R intermetallic phase and a second solid CoR phase which is richer in rare earth metal content than the Co 5 R phase. The specific cobalt and rare earth metal content of the alloy is substantially the same as that desired in the sintered product. The alloy, in particulate form, is pressed into compacts and sintered to the desired density. The sintered product is comprised of a major amount of the Co 5 R solid intermetallic phase and up to about 35 percent of the product of the second solid CoR intermetallic phase which is richer in rare earth metal content than the Co 5 R phase

  7. Fatal attraction: rare species in the spotlight.

    Science.gov (United States)

    Angulo, Elena; Deves, Anne-Laure; Saint Jalmes, Michel; Courchamp, Franck

    2009-04-07

    The exploitation of rare and endangered species can end in the species's extinction because the increased value people associate with rarity increases the economic incentive to exploit the last individuals, creating a positive feedback loop. This recently proposed concept, called the anthropogenic Allee effect (AAE), relies on the assumption that people do value rarity, but this remains to be established. Moreover, it also remains to be determined whether attraction to rarity is a trait confined to a minority of hobbyists (e.g. wildlife collectors, exotic pet owners) or characteristic of the general public. We estimated how much the general public valued rare species compared with common ones, using five different metrics related to personal investment: time spent, physical effort, unpleasantness, economic investment and risk. We surveyed the visitors of a zoo. To see the rare species, the visitors to the zoo invested more time in searching and contemplation, they were ready to expend more physical effort, they tolerated more unpleasant conditions, they were willing to pay more and, finally, they risked more to obtain (steal) a rare species. Our results provide substantial evidence of how the general public places more value on rare species, compared with common species. This confirms the AAE as an actual process, which in addition concerns a large part of the population. This has important consequences for the conservation of species that are rare now, or that could become so in the future.

  8. Fine structure transition cross sections for several alkali+rare gas systems

    International Nuclear Information System (INIS)

    Olson, R.E.

    1975-01-01

    The energy dependence E(cm) 2 P1/2→ 2 P3/2 fine structure transition of the lowest excited states of the alkali are calculated for the following systems: Na, K, Rb+He, Ne, Ar and Cs+He. Encouraging agreement between theory and experiment is obtained [fr

  9. A successful method for severe gestational thrombocytopenia: A rare case study

    Directory of Open Access Journals (Sweden)

    Adnan İncebıyık

    2013-01-01

    Full Text Available In a normally progressing pregnancy, especially in thethird trimester thrombocyte numbers reduce by about10%. The reason for this reduction is thought to be thehemodilution and increased consumption in pregnancy.Generally, it is recognized by chance during routine bloodcount and it is the second most common hematologicanomaly during pregnancy. Once thrombocytopenia isdiagnosed in pregnancy, research must be done to determinewhether it is a life-threatening risk factor in terms ofthe mother-fetus-infant. The majority of thrombocytopeniaseen in pregnancy is gestational thrombocytopenia. Thispaper examines an active labor, normal birthing case witha thrombocyte count of 7916/mm3.Key words: Gestational thrombocytopenia, idiopathicthrombocytopenic purpura, normal birth

  10. Several rare freshwater planktic cyanobacteria (Cyanoprokaryotes) from reservoirs in South America

    Czech Academy of Sciences Publication Activity Database

    Komárek, Jiří; Komárková, Jaroslava

    2007-01-01

    Roč. 34, č. 1 (2007), s. 49-58 ISSN 0073-2877 R&D Projects: GA AV ČR KSK6005114; GA AV ČR IAA6005704 Institutional research plan: CEZ:AV0Z60050516; CEZ:AV0Z60170517 Keywords : cyanobacteria * plancton * tropical reservoars Subject RIV: EF - Botanics

  11. Severe febrile systemic reaction to pneumococcal vaccine.

    Science.gov (United States)

    Hasan, Syed; Yousef, Mahmoud; Shridharani, Sachin

    2005-02-01

    Polyvalent pneumococcal polysaccharide vaccine (Pneumovax, PPV) has been shown to substantially reduce the risk of Streptococcus pneumoniae infections in susceptible individuals. Side effects, such as mild local erythema, induration, pain and fever, have been reported with various frequencies. Rarely, systemic symptoms, including high fever, headache, nausea and photophobia, have been reported in the literature. This case report describes a 38-year-old male who developed severe and prolonged local and systemic symptoms necessitating hospitalization following a dose of pneumovax.

  12. Bazex Syndrome with Hypoalbuminemia and Severe Ascites

    OpenAIRE

    Matsui, Hidetoshi; Iwae, Shigemichi; Hirayama, Yuji; Yonezawa, Koichiro; Shigeji, Jun

    2016-01-01

    Bazex syndrome is a rare paraneoplastic dermatosis. The underlying malignancy frequently is squamous cell carcinoma of the upper aerodigestive tract or cervical lymph nodes from an unknown primary site. We report a 63-year-old man with squamous cell carcinoma of cervical lymph nodes from an unknown primary site. He developed a mass on the right side of his neck, cutaneous lesions diagnosed as Bazex syndrome, hypoalbuminemia, and severe ascites. Right neck dissection was performed. After neck ...

  13. Ochronosis: a case of severe ochronotic arthropathy

    Directory of Open Access Journals (Sweden)

    Berat Meryem Alkan

    2011-10-01

    Full Text Available Ochronosis involves primarily the large cartilaginous joint surfaces, ribs, intervertebral discs, ear cartilage etc. We report on a 52-year-old woman with typical alkaptonuric ochronosis with dark urine, blue-black pigmentation of the auriculae and hands, spondylarthropathy and severe hip and shoulder joints involvement. The differential diagnosis of this rare condition is discussed with a review of the literature.

  14. State of rare earth impurities in gallium and indium antimonides

    International Nuclear Information System (INIS)

    Evgen'ev, S.B.; Kuz'micheva, G.M.

    1990-01-01

    State of rare earth impurities in indium and gallium antimonides was studied. Results of measuring density and lattice parameter of samples in GaSb-rare earth and InSb-rare earth systems are presented. It is shown that during rare earth dissolution in indium and gallium antimonides rare earth atoms occupy interstitial positions or, at least, are displaced from lattice points

  15. Echocardiographic diagnosis of rare pathological patterns of sinus of Valsalva aneurysm.

    Directory of Open Access Journals (Sweden)

    Yali Yang

    Full Text Available To evaluate the value and improve the diagnostic accuracy of echocardiography in the diagnosis of a sinus of Valsalva aneurysm (SVA with rare pathological patterns.Echocardiographic features and surgical findings from 270 Chinese patients with SVA treated in the last 18 years (1995-2013 at the Union Hospital were compared retrospectively; 22 of 270 cases had rare patterns.The patients with SVA, a rare origin, a rare extending position, or a rare course accounted for 3.4%, 7.4%, and 0.4% of the 270 cases, respectively. The three most common aneurysmal complications of the patients with rare patterns were severe aortic regurgitation (16, obstruction of the ventricular outflow tract or valvular orifice (3, and conduction disturbance (3. The origin, course, extending position and rupture status of the SVAs determined by echocardiography were entirely consistent with surgical findings in 81.8% of the 22 cases. With the exception of one failed diagnosis of an aneurysmal wall dissection and one misdiagnosis of a descending aortic dissection, the echocardiographic results of SVA complications and associated cardiovascular lesions were also confirmed.We could accurately diagnose SVAs with rare pathological patterns by echocardiographic identification of distinguishing features. However, for several conditions, we could not accurately identify the origin or course of the aneurysm or define its relationship to adjacent structures using conventional echocardiography alone. Therefore, we recommend combining conventional echocardiography with different imaging techniques, such as transesophageal echocardiography, three-dimensional echocardiography, computed tomography angiography, and aortic angiography.

  16. Mechanical Properties of Magnesium-Rare Earth Alloy Systems: A Review

    Directory of Open Access Journals (Sweden)

    Sravya Tekumalla

    2014-12-01

    Full Text Available Magnesium-rare earth based alloys are increasingly being investigated due to the formation of highly stable strengthening phases, activation of additional deformation modes and improvement in mechanical properties. Several investigations have been done to study the effect of rare earths when they are alloyed to pure magnesium and other Mg alloys. In this review, the mechanical properties of the previously investigated different magnesium-rare earth based binary alloys, ternary alloys and other higher alloys with more than three alloying elements are presented.

  17. Rare earths production and marketing opportunities

    International Nuclear Information System (INIS)

    Falconnet, P.G.

    1988-01-01

    The rare earths (RE) market is relatively small. The total production during 1968 was only 10000 tons (REO) which rose to 27000 tons (REO) during 1985. The three major areas of application, which are volume market for ceric rare earths are catalysts, glass ceramics and metallurgy. Among the other uses of rare earths, the permanent magnets, lamp phosphors and fine ceramics have registered significant growth in RE consumption. Monazite and bastnasite are the main natural source for rare earths and processing of these for one of the rare earths in high demand leads to over production of some others not in demand, thus creating a balance problem. The growth in RE market has always been influenced by the technology shifts and product substitution. For example, the RE consumption during 1974/76 for desulfurization of steel had substantially decreased due to the usage of calcium. Similarly, 1985 had witnessed a drastic cut in the use of REs in fluid cracking due to the introduction of stabilized zeolites which contain less REO. Thus, the overall compound growth rate of demand was only 3.9 % per year during the period 1970-1985. At present, 37 % of the rare earths production goes to the glass/ceramics industry, 33 % for catalyst and 25 % to metallurgy. The price of REs constantly shows a downward trend. This trend coupled with the rapid changes taking place in the various technological fields, demands greater flexibility and high marketing skills from the RE producers. The key factor for future expansion of RE market will be the development of 'high volume' application of ceric rare earths. (author) 2 figs., 8 tabs

  18. Extraction of rare earths from iron-rich rare earth deposits

    OpenAIRE

    Bisaka, K.; Thobadi, I.C.; Pawlik, C.

    2017-01-01

    Rare earth metals are classified as critical metals by the United Nations, as they have found wide application in the fabrication of magnets, particularly those used in green energy technologies which mitigate global warming. Processing of ores containing rare earth elements is complex, and differs according to the nature of each ore. In the conventional process, run of mine (ROM) ores are processed in a physical separation plant to produce a concentrate from which rare earth elements are ext...

  19. The burden of rare cancers in Italy: the surveillance of rare cancers in Italy (RITA) project.

    Science.gov (United States)

    Trama, Annalisa; Mallone, Sandra; Ferretti, Stefano; Meduri, Francesca; Capocaccia, Riccardo; Gatta, Gemma

    2012-01-01

    The project Surveillance of rare cancers in Italy (RITA) provides, for the first time, estimates of the burden of rare cancers in Italy based on the list of rare cancers proposed in collaboration with the European project Surveillance of Rare Cancers in Europe (RARECARE). RITA analyzed data from Italian population-based cancer registries (CR). The period of diagnosis was 1988 to 2002, and vital status information was available up to December 31, 2003. Incidence rates were estimated for the period 1995-2002, survival for the years 2000-2002 (with the period method of Brenner), and complete prevalence at January 1, 2003. Rare cancers are those with an incidence <6/100,000/year. In Italy, every year there are 60,000 new diagnoses of rare cancers corresponding to 15% of all new cancer diagnoses. Five-year relative survival was on the average worse for rare cancers (53%) than for common cancers (73%). A total of 770,000 patients were living in Italy in 2008 with a diagnosis of a rare cancer, 22% of the total cancer prevalence. Our estimates constitute a useful base for further research and support the idea that rare cancers are a public health problem that deserves attention. Centers of expertise for rare cancers that pool cases, expertise and resources could ensure an adequate clinical management for these diseases. Our data also showed that cancer registries are suitable sources of data to estimate incidence, prevalence and survival for rare cancers and should continue to monitoring rare cancers in Italy.

  20. Absorption spectroscopy of complex rare earth ion doped hybrid materials over a broad wavelength range

    OpenAIRE

    Dekker, R.; Worhoff, Kerstin; Stouwdam, J.W.; van Veggel, F.C.J.M.; Driessen, A.

    2005-01-01

    In the present work we applied a measurement setup to determine several relevant properties of rare-earth doped nanoparticles dispersed in polymer slab waveguides in a single absorption measurement: background absorption of the polymer host material, water absorption, polymer composition (overtones), rare earth concentration, and ligand contribution (increase of exponential loss trend in the UV). Furthermore, nanoparticle size and concentration in case of a refractive index mismatch (1//spl l...

  1. Refined potentials for rare gas atom adsorption on rare gas and alkali-halide surfaces

    Science.gov (United States)

    Wilson, J. W.; Heinbockel, J. H.; Outlaw, R. A.

    1985-01-01

    The utilization of models of interatomic potential for physical interaction to estimate the long range attractive potential for rare gases and ions is discussed. The long range attractive force is calculated in terms of the atomic dispersion properties. A data base of atomic dispersion parameters for rare gas atoms, alkali ion, and halogen ions is applied to the study of the repulsive core; the procedure for evaluating the repulsive core of ion interactions is described. The interaction of rare gas atoms on ideal rare gas solid and alkali-halide surfaces is analyzed; zero coverage absorption potentials are derived.

  2. ESCMID and ECMM joint clinical guidelines for the diagnosis and management of rare invasive yeast infections

    NARCIS (Netherlands)

    Arendrup, M.C.; Boekhout, T.; Akova, M.; Meis, J.F.; Cornely, O.A.; Lorthlaro, O.

    The mortality associated with invasive fungal infections remains high with that involving rare yeast pathogens other than Candida being no exception. This is in part due to the severe underlying conditions typically predisposing patients to these healthcare-related infections (most often severe

  3. Response of the rare biosphere to environmental stressors in a highly diverse ecosystem (Zodletone spring, OK, USA).

    Science.gov (United States)

    Coveley, Suzanne; Elshahed, Mostafa S; Youssef, Noha H

    2015-01-01

    Within highly diverse ecosystems, the majority of bacterial taxa are present in low abundance as members of the rare biosphere. The rationale for the occurrence and maintenance of the rare biosphere, and the putative ecological role(s) and dynamics of its members within a specific ecosystem is currently debated. We hypothesized that in highly diverse ecosystems, a fraction of the rare biosphere acts as a backup system that readily responds to environmental disturbances. We tested this hypothesis by subjecting sediments from Zodletone spring, a sulfide- and sulfur-rich spring in Southwestern OK, to incremental levels of salinity (1, 2, 3, 4, and 10% NaCl), or temperature (28°, 30°, 32°, and 70 °C), and traced the trajectories of rare members of the community in response to these manipulations using 16S rRNA gene analysis. Our results indicate that multiple rare bacterial taxa are promoted from rare to abundant members of the community following such manipulations and that, in general, the magnitude of such recruitment is directly proportional to the severity of the applied manipulation. Rare members that are phylogenetically distinct from abundant taxa in the original sample (unique rare biosphere) played a more important role in the microbial community response to environmental disturbances, compared to rare members that are phylogenetically similar to abundant taxa in the original sample (non-unique rare biosphere). The results emphasize the dynamic nature of the rare biosphere, and highlight its complexity and non-monolithic nature.

  4. Mechanical ventilation for severe asthma.

    Science.gov (United States)

    Leatherman, James

    2015-06-01

    Acute exacerbations of asthma can lead to respiratory failure requiring ventilatory assistance. Noninvasive ventilation may prevent the need for endotracheal intubation in selected patients. For patients who are intubated and undergo mechanical ventilation, a strategy that prioritizes avoidance of ventilator-related complications over correction of hypercapnia was first proposed 30 years ago and has become the preferred approach. Excessive pulmonary hyperinflation is a major cause of hypotension and barotrauma. An appreciation of the key determinants of hyperinflation is essential to rational ventilator management. Standard therapy for patients with asthma undergoing mechanical ventilation consists of inhaled bronchodilators, corticosteroids, and drugs used to facilitate controlled hypoventilation. Nonconventional interventions such as heliox, general anesthesia, bronchoscopy, and extracorporeal life support have also been advocated for patients with fulminant asthma but are rarely necessary. Immediate mortality for patients who are mechanically ventilated for acute severe asthma is very low and is often associated with out-of-hospital cardiorespiratory arrest before intubation. However, patients who have been intubated for severe asthma are at increased risk for death from subsequent exacerbations and must be managed accordingly in the outpatient setting.

  5. INTRAMUSCULAR HYDATID CYST OF PARASPINAL MUSCLE: A RARE LOCATION

    Directory of Open Access Journals (Sweden)

    Bhargava Vardhana Reddy

    2015-05-01

    Full Text Available INTRODUCTION: Hydatid disease has a worldwide distribution and causes health problems in endemic countries. The parasite has a "dog - sheep" cycle with man as an intermediate accidental host. When humans ingest the eggs of the tapeworm, the embryos that emer ge penetrate the intestinal mucosa and are transported via the circulation to various organs. Most commonly they reach the liver, lungs and the other organs are rarely affected. Primary hydatid cyst of skeletal muscle is rare, occurring in 1 - 3% of all case s. (1,2 The prevalence of intramuscular hydatid disease is reported to be less than 0.5% , because muscle is an unfavourable site for infestation because of high levels of lactic acid in muscle. (3 The diagnosis is difficult because of the unusual location, low prevalence and complicated cysts may mimic solid or complex lesions. (4 The differential diagnosis in these cases must include malignant soft - tissue tumors such as myxoid liposarcoma, soft tissue abscesses and chronic hematoma. (5 Hydatid disease of h umans caused by Echinococcus granulosus has been recognized as a major public health problem. It is found in all sheep - raising countries of the world. In India, the highest prevalence is reported from Andhra Pradesh and Tamil Nadu. (6 Injudicious approach in the management of these rare presentations may be the root cause of severe anaphylactic shock and systemic dissemination. We report an unusual case of primary hydatidosis of the paraspinal muscles

  6. Rare B-meson decays at the crossroads

    Energy Technology Data Exchange (ETDEWEB)

    Ali, Ahmed

    2016-07-15

    Experimental era of rare B-decays started with the measurement of B→K{sup *}γ by CLEO in 1993, followed two years later by the measurement of the inclusive decay B→X{sub s}γ, which serves as the standard candle in this field. The frontier has moved in the meanwhile to the experiments at the LHC, in particular, LHCb, with the decay B{sup 0}→μ{sup +}μ{sup -} at about 1 part in 10{sup 10} being the smallest branching fraction measured so far. Experimental precision achieved in this area has put the standard model to unprecedented stringent tests and more are in the offing in the near future. I review some key measurements in radiative, semileptonic and leptonic rare B-decays, contrast them with their estimates in the SM, and focus on several mismatches reported recently. They are too numerous to be ignored, yet, standing alone, none of them is significant enough to warrant the breakdown of the SM. Rare B-decays find themselves at the crossroads, possibly pointing to new horizons, but quite likely requiring an improved theoretical description in the context of the SM. An independent precision experiment such as Belle II may help greatly in clearing some of the current experimental issues.

  7. Rare B-meson decays at the crossroads

    International Nuclear Information System (INIS)

    Ali, Ahmed

    2016-07-01

    Experimental era of rare B-decays started with the measurement of B→K * γ by CLEO in 1993, followed two years later by the measurement of the inclusive decay B→X s γ, which serves as the standard candle in this field. The frontier has moved in the meanwhile to the experiments at the LHC, in particular, LHCb, with the decay B 0 →μ + μ - at about 1 part in 10 10 being the smallest branching fraction measured so far. Experimental precision achieved in this area has put the standard model to unprecedented stringent tests and more are in the offing in the near future. I review some key measurements in radiative, semileptonic and leptonic rare B-decays, contrast them with their estimates in the SM, and focus on several mismatches reported recently. They are too numerous to be ignored, yet, standing alone, none of them is significant enough to warrant the breakdown of the SM. Rare B-decays find themselves at the crossroads, possibly pointing to new horizons, but quite likely requiring an improved theoretical description in the context of the SM. An independent precision experiment such as Belle II may help greatly in clearing some of the current experimental issues.

  8. [Research funding for rare diseases in Germany].

    Science.gov (United States)

    Wissing, Frank; Bruckner-Tuderman, Leena

    2017-05-01

    There is high need for more research in the field of rare diseases. Not only must the causes and mechanisms of the numerous and often heterogeneous diseases be delineated, but criteria must also be defined for optimal stratification of patients for individualized therapies. In this context, research and innovative diagnostics are linked together more closely than in other fields of medicine. The early stages of disease-oriented research can be performed in individual institutions but, due to low numbers of patients, late translation and transfer into clinics requires multicentric and international collaboration. In Germany research on rare diseases takes place mostly in faculties of medicine at universities. Since the institutional financial support is very low, research grants have substantial significance. The German Research Foundation (DFG) and the Federal Ministry of Education and Research (BMBF) are the main grant agencies for national projects, but foundations and patient advocacy groups also finance research to a certain extent. The ERA-Net "E-Rare" and the programs of the EU target primarily international cross-border projects and patient trials. All of these programs need to be adapted more efficiently to the particular needs of rare disease research. For national and international research projects on rare diseases, sufficient funds are needed but also sustainable interdisciplinary platforms and centers must be established in order to share expert knowledge and to implement complex programs such as proof-of-concept studies in humans.

  9. Rare earth elements and permanent magnets (invited)

    Science.gov (United States)

    Dent, Peter C.

    2012-04-01

    Rare earth (RE) magnets have become virtually indispensible in a wide variety of industries such as aerospace, automotive, electronics, medical, and military. RE elements are essential ingredients in these high performance magnets based on intermetallic compounds RECo5, RE2TM17 (TM: transition metal), and RE2TM14B. Rare earth magnets are known for their superior magnetic properties—high induction, and coercive force. These properties arise due to the extremely high magnetocrystalline anisotropy made possible by unique 3d-4f interactions between transition metals and rare earths. For more than 40 years, these magnets remain the number one choice in applications that require high magnetic fields in extreme operating conditions—high demagnetization forces and high temperature. EEC produces and specializes in RECo5 and RE2TM17 type sintered magnets. Samarium and gadolinium are key RE ingredients in the powder metallurgical magnet production processes which include melting, crushing, jet milling, pressing, sintering, and heat treating. The magnetic properties and applications of these magnets will be discussed. We will also briefly discuss the past, current, and future of the permanent magnet business. Currently, over 95% of all pure rare earth oxides are sourced from China, which currently controls the market. We will provide insights regarding current and potential new magnet technologies and designer choices, which may mitigate rare earth supply chain issues now and into the future.

  10. Rare event simulation using Monte Carlo methods

    CERN Document Server

    Rubino, Gerardo

    2009-01-01

    In a probabilistic model, a rare event is an event with a very small probability of occurrence. The forecasting of rare events is a formidable task but is important in many areas. For instance a catastrophic failure in a transport system or in a nuclear power plant, the failure of an information processing system in a bank, or in the communication network of a group of banks, leading to financial losses. Being able to evaluate the probability of rare events is therefore a critical issue. Monte Carlo Methods, the simulation of corresponding models, are used to analyze rare events. This book sets out to present the mathematical tools available for the efficient simulation of rare events. Importance sampling and splitting are presented along with an exposition of how to apply these tools to a variety of fields ranging from performance and dependability evaluation of complex systems, typically in computer science or in telecommunications, to chemical reaction analysis in biology or particle transport in physics. ...

  11. [SZCZECIN CITIZENS' KNOWLEDGE ABOUT RARE DISEASES].

    Science.gov (United States)

    Walat, Anna; Skoczylas, Michal Marian; Welnicka, Agnieszka; Kulig, Malgorzata; Rodak, Przemyslaw; Walczak, Zuzanna; Jablońska, Agata

    2014-01-01

    The aim of the study was to assess knowledge about rare diseases among citizens of Szczecin (Poland). The study was performed by questioning 242 adult customers of Turzyn Shopping Centre in Szczecin (149 females and 93 males). The survey was conducted in the shopping mall on 23 February 2013 (control group) and during the celebration of Rare Disease Day and the 12th Polish Nationwide Cystic Fibrosis Week ("Dolina Mukolinków") on 2 March 2013 (research group). The research tool was a questionnaire devised by the authors and filled out by the writing authors interviewer's answers. In the study group more people knew about the existence of Rare Disease Day than in the control group (86.02% vs 57.72%, chi-square test χ2 > χ2(1); 0.001, p χ2(1); 0.001, p < 0.001). The respondents from the research group knew more about Rare Disease Day and defined the idea of it as closed in a significantly higher degree than the control group. There was no significant difference in the detailed knowledge about rare diseases in either group. This might indicate the need to educate society and patients, along with their families.

  12. Acute severe asthma presenting in late pregnancy.

    Science.gov (United States)

    Holland, S M; Thomson, K D

    2006-01-01

    Asthma is the commonest pre-existing medical condition to complicate pregnancy. Acute severe asthma in pregnancy is rare, but poses difficult problems. In particular, the decision about when and where to deliver the fetus is complex, since maternal response to asthma treatment is unpredictable. We report the successful management of a parturient presenting with acute severe asthma at 37 weeks' gestation. The controversies involved and the importance of adopting a multi-disciplinary team approach to optimise maternal and neonatal outcomes are discussed.

  13. Palmer Drought Severity Index

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — PDSI from the Dai dataset. The Palmer Drought Severity Index (PDSI) is devised by Palmer (1965) to represent the severity of dry and wet spells over the U.S. based...

  14. Gorlin-Goltz syndrome: A rare case report

    Directory of Open Access Journals (Sweden)

    Chetan A Pol

    2013-01-01

    Full Text Available 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.

  15. Gorlin-Goltz syndrome: A rare case report.

    Science.gov (United States)

    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.

  16. Pulmonary capillary haemangiomatosis: a rare cause of pulmonary hypertension.

    Science.gov (United States)

    Babu, K Anand; Supraja, K; Singh, Raj B

    2014-01-01

    Pulmonary capillary haemangiomatosis (PCH) is a rare disorder of unknown aetiology, characterised by proliferating capillaries that invade the pulmonary interstitium, alveolar septae and the pulmonary vasculature. It is often mis-diagnosed as primary pulmonary hypertension and pulmonary veno-occlusive disease. Pulmonary capillary haemangiomatosis is a locally aggressive benign vascular neoplasm of the lung. We report the case of a 19-year-old female who was referred to us in the early post-partum period with severe pulmonary artery hypertension, which was diagnosed as PCH by open lung biopsy.

  17. Two Rare Cases of Hypernatremic Haemorrhagic Encephalopathy in Children.

    Directory of Open Access Journals (Sweden)

    Dr Shital Turakhia

    2016-12-01

    Full Text Available Hypernatremia is defined as increased serum sodium concentration more than 145mEq/L. It occurs most commonly in pediatric patients, geriatric patients and patients with debilitated conditions in ICU who suffers from severe water losses. Newborns and toddlers are at high risk as they have poor thirst control and regulatory mechanism. It occurs when patient is suffering from diarrhea and then mismanagement by over treating the patient with sodium containing fluids. CNS complications and radiological findings are rarely recognized and reported. We hereby report cases of two such pediatric patients who were referred to our hospital from primary health care centers with complain of convulsions and diarrhea.

  18. Rogue-Elephant-Inflicted Panfacial Injuries: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Santosh Kumar Yadav

    2012-01-01

    Full Text Available Attacks by elephants, the largest of the “large animals,” produce many fatalities a year. Most attacks are provoked, although rogue elephants are occasionally responsible. Trampling, goring, tossing the individual with the trunk, or crushing with the knees produces the injuries. Injuries from encounters with large animals represent a significant health risk for rural communities. Wild-animal-inflicted maxillofacial injuries are rare, and limited literature is available describing their management. We present a case of severe maxillofacial injuries caused by the attack of a rogue elephant.

  19. Pyomyoma as a Rare Source of Postpartum Sepsis

    Directory of Open Access Journals (Sweden)

    A. DeMaio

    2015-01-01

    Full Text Available Pyomyoma, also known as suppurative leiomyoma, is a rare clinical complication that occurs when a leiomyoma undergoes infarction and subsequent infection. A high index of suspicion is required to make the diagnosis and can be guided by a classic triad of symptoms that includes abdominal pain, sepsis without an obvious source, and a history of leiomyoma. In the vast majority of these cases, total abdominal hysterectomy is required to avoid severe morbidity and potential mortality. We present an unusual case of a postpartum pyomyoma that was successfully treated without the need for hysterectomy. With strong clinical suspicion, early diagnosis, and appropriate management, some affected patients may preserve fertility.

  20. Level density and thermal properties in rare earth nuclei

    International Nuclear Information System (INIS)

    Siem, S.; Schiller, A.; Guttormsen, M.; Hjorth-Jensen, M.; Melby, E.; Rekstad, J.

    2000-01-01

    The level density at low spin has been extracted for several nuclei in the rare earth region using the ( 3 He,α) reaction. Within the framework of the microcanonical ensemble, the entropy and the temperature of the nuclei are derived. The temperature curve shows bumps which are associated with the break up of Cooper pairs. The entropies of the even-even and even-odd nuclei have been compared. The nuclear heat capacity is deduced within the framework of the canonical ensemble and exhibits an S-formed shape as function of temperature. (author)

  1. Acute amebic appendicitis: Report of a rare case

    Directory of Open Access Journals (Sweden)

    Singh Naorem

    2010-10-01

    Full Text Available Acute appendicitis of amebic origin is considered a rare cause of acute appendicitis. We report a case of amebic appendicitis presenting with fever, severe pain in the right lower quadrant of the abdomen and rebound tenderness. Lab investigations revealed neutrophilic leukocytosis. The patient underwent appendectomy. Histopathological examination revealed numerous Entameba histolytica trophozoites in the mucosa of the appendix. Acute appendicitis of amebic origin does not appear frequently. Appendicular amebiasis can give the clinical features of acute appendicitis and should be treated accordingly.

  2. A Rare Occurance with Epidermolysis Bullosa Disease: Dilated Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Derya Cimen

    2014-02-01

    Full Text Available Epidermolysis bullosa is a congenital and herediter vesiculobullous disease. Dystrophic form of this disease is characterized by severe malnutrition, failure to thrive, adhesions at fingers, joint contractures related with the formation of scar tissues, carcinoma of the skin, anemia, hipoalbuminemia, wound enfections and sepsis. Rarely, mortal dilated cardiomyopathy may occur in patients. In this report we present a 13 year-old pediatric patient with dilated cardiomyopathy, clinically diagnosed with Epidermolysis bullosa as well as a review of recent related literature.

  3. Recovering heavy rare earth metals from magnet scrap

    Science.gov (United States)

    Ott, Ryan T.; McCallum, Ralph W.; Jones, Lawrence L.

    2017-08-08

    A method of treating rare earth metal-bearing permanent magnet scrap, waste or other material in a manner to recover the heavy rare earth metal content separately from the light rare earth metal content. The heavy rare earth metal content can be recovered either as a heavy rare earth metal-enriched iron based alloy or as a heavy rare earth metal based alloy.

  4. Rare earth mobility in hydrothermal ore-forming systems

    International Nuclear Information System (INIS)

    Cornell, D.H.; Schade, J.; Scheepers, R.; Watkeys, M.K.

    1988-01-01

    Rocks and ores which form by magmatic processes display a range of chondrite-normalised rare earth profiles. One REE (rare earth elements) profile feature which seems unrelated to magmatic processes is the birdwing profile, in which both heavy and light rare earths are enriched relative to the middle rare earths. Birdwing rare earth profiles are an easily identified geochemical anomaly. It is proposed that rare earth geochemistry could be applied in geochemical prospecting for ore formed by hydrothermal processes. 5 figs

  5. Determinants of performance in rare strategic events

    DEFF Research Database (Denmark)

    Andersen, Kristina Vaarst; Beukel, Karin

    The paper develops and tests a theoretical framework explaining how emotions and learning affect outcomes of rare strategic events, namely Intellectual Property litigations. We investigate how organizations? negative emotions influence performance outcome in IP litigations negatively. Though...... cumulative learning in rare strategic events is scarce, and cannot be understood through the standard framework of routines and capability development, we argue that firms may learn from rare events, and propose that learning moderates the negative effect of emotions. We test this utilizing data on all...... publically available IP litigation cases in China from 2001 to 2009 (n=13,030). We find that when organizations undergo emotional negative stress they lose IP litigations more often, but development of roadmaps though past successes moderate the negative effects from emotional distress....

  6. Rapid analysis of some rare earth magnets

    International Nuclear Information System (INIS)

    Raoot, K.N.; Raoot, Sarala; Rukmani Desikan, N.

    1978-01-01

    A simple complexometric method for the quick analysis of the constituent elements in some quaternary rare earth magnets of the type RE-Co-Cu-Fe and RE-Ni-Cu-Fe is described. The technique is based on a total titration, subsequent release of EDTA from rare earth with ammonium fluoride and that from copper (II) with ascorbic acid and thiourea followed by determination of the excess and liberated EDTA by lead nitrate in a weak acid medium using xylenol orange indicator. In another, aliquot iron (III) and rare earth are first masked with sodium fluoride, and copper (II) with ascorbic acid and thiourea before cobalt (II) or nickel (II) is estimated by back titration. Iron is calculated by difference. The new method yields accurate and reproducible results with error not exceeding 1%. A set of three samples can conveniently be analysed in two hours. (author)

  7. Coprecipitation of rare earth elements with hydroxyapatite

    International Nuclear Information System (INIS)

    Fujino, Osamu

    1979-01-01

    The distribution behavior of trace rare earth elements between an aqueous phase and hydroxyapatite crystals was investigated. The apatite prepared by adding phosphate ion extremely slowly to an aqueous solution containing calcium, rare earth elements, ethylenediamine and nitrilotriacetate ion at 80 0 C. Apparently the coprecipitation reaction seems to be anomalous, because the apparent distribution coefficient did not have a constant value through the reaction. But when the true distribution coefficient was calculated by using the thermodynamic data at 80 0 C, it was revealed that rare earth ions were coprecipitated obeying logarithmic distribution law. The true distribution coefficient values of trivalent yttrium, scandium, europium and cerium ions were about 10sup(7.3), 10sup(9.8), 10sup(7.4) and 10sup(6.5) respectively. (author)

  8. Material efficiency: rare and critical metals.

    Science.gov (United States)

    Ayres, Robert U; Peiró, Laura Talens

    2013-03-13

    In the last few decades, progress in electronics, especially, has resulted in important new uses for a number of geologically rare metals, some of which were mere curiosities in the past. Most of them are not mined for their own sake (gold, the platinum group metals and the rare Earth elements are exceptions) but are found mainly in the ores of the major industrial metals, such as aluminium, copper, zinc and nickel. We call these major metals 'attractors' and the rare accompanying metals 'hitch-hikers'. The key implication is that rising prices do not necessarily call forth greater output because that would normally require greater output of the attractor metal. We trace the geological relationships and the functional uses of these metals. Some of these metals appear to be irreplaceable in the sense that there are no known substitutes for them in their current functional uses. Recycling is going to be increasingly important, notwithstanding a number of barriers.

  9. Rare earths: harvesting basic research for technology

    International Nuclear Information System (INIS)

    Jagatap, B.N.

    2014-01-01

    In recent years, rare earths are increasingly becoming a versatile platform for basic research that presents enormous technological potentials. A variety of nano-sized inorganic matrices varying from oxides, phosphates, gallates and aluminates, tungstates, stannates, vanadates to fluorides doped with different lanthanide ions have been synthesized and their optical properties have been investigated in the Chemistry Group, BARC. Another interesting application is laser cooling of solids using rare earth doped glasses with potential applications in remote cooling of electronic devices. Combining the luminescence properties of rare earths with photonic crystals is yet another potent area with wide ranging applications. In this presentation we provide an overview of these developments with examples from the R and D programs of the Chemistry Group, BARC

  10. Rapid analysis of some rare earth magnets

    Energy Technology Data Exchange (ETDEWEB)

    Raoot, K N; Raoot, S; Rukmani Desikan, N [Defence Metallurgical Research Lab., Hyderabad (India)

    1978-12-01

    A simple complexometric method for the quick analysis of the constituent elements in some quaternary rare earth magnets of the type RE-Co-Cu-Fe and RE-Ni-Cu-Fe is described. The technique is based on a total titration, subsequent release of EDTA from rare earth with ammonium fluoride and that from copper (II) with ascorbic acid and thiourea followed by determination of the excess and liberated EDTA by lead nitrate in a weak acid medium using xylenol orange indicator. In another, aliquot iron (III) and rare earth are first masked with sodium fluoride, and copper (II) with ascorbic acid and thiourea before cobalt (II) or nickel (II) is estimated by back titration. Iron is calculated by difference. The new method yields accurate and reproducible results with error not exceeding 1%. A set of three samples can conveniently be analysed in two hours.

  11. On fluorozirconates and fluorohafnates of rare earths

    International Nuclear Information System (INIS)

    Korenev, Yu.M.; Antipov, P.I.; Novoselova, A.V.

    1980-01-01

    It has been shown by the method of X-ray phase analysis that on interaction between rare-earth fluorides and zirconium and hafnium tetrafluorides, compounds with 1:1, 1:2, 1:3 molar ratios of components are formed. Compounds of the LnHfF 4 type are prepared for all rare-earths. Fluoro-metals of the LnHf 2 F 11 composition are typical only of light lanthanides from lanthanum to neodymium, while pentafluorated salts Ln(EF 5 ) 3 are formed in the reaction between EF 4 with fluorides of heavy rare-earth elements from samarium to lutecium, as well as with yttrium trifluoride. Parameters of unit cells of heptafluohafnates and pentafluometallates are determined

  12. Thermogravimetric study of rare earth concentrates

    International Nuclear Information System (INIS)

    Delyagejd, V.V.; Anisimova, V.N.; Eremenko, Z.V.; Kutsev, V.S.

    1974-01-01

    Methods of thermogravimetric, chemical and phase analysis were used in measuring the concentration of rare-earth elements of different origins. At temperatures 400-800 deg C a gradual decomposition of fluorocarbonates takes place leading to the formation of derivatives of corresponding oxides and oxyfluorides. For concentrates containing siderite the process takes place at 550-600 deg C followed by oxidation of bivalent iron into trivalent state. Reaction of rare-earth elements with sodium carbonate and the increase in the concentration of the latter results in a narrowing down of the interval of temperatures at which decomposition takes place. Under these conditions an intense reaction and a fusion take place leading to the formation of eutectic at 500-600 deg C and further synthesis of sodium fluoride and oxyfluoride derivatives of calcium and rare-earth elements

  13. Childhood sarcoidosis: A rare but fascinating disorder

    Directory of Open Access Journals (Sweden)

    Gedalia Abraham

    2008-09-01

    Full Text Available Abstract Childhood sarcoidosis is a rare multisystemic granulomatous disorder of unknown etiology. In the pediatric series reported from the southeastern United States, sarcoidosis had a higher incidence among African Americans. Most reported childhood cases have occurred in patients aged 13–15 years. Macrophages bearing an increased expression of major histocompatibility class (MHC II molecules most likely initiate the inflammatory response of sarcoidosis by presenting an unidentified antigen to CD4+ Th (helper-inducer lymphocytes. A persistent, poorly degradable antigen driven cell-mediated immune response leads to a cytokine cascade, to granuloma formation, and eventually to fibrosis. Frequently observed immunologic features include depression of cutaneous delayed-type hypersensitivity and a heightened helper T cell type 1 (Th1 immune response at sites of disease. Circulating immune complexes, along with signs of B cell hyperactivity, may also be found. The clinical presentation can vary greatly depending upon the organs involved and age of the patient. Two distinct forms of sarcoidosis exist in children. Older children usually present with a multisystem disease similar to the adult manifestations, with frequent hilar lymphadenopathy and pulmonary infiltrations. Early-onset sarcoidosis is a unique form of the disease characterized by the triad of rash, uveitis, and arthritis in children presenting before four years of age. The diagnosis of sarcoidosis is confirmed by demonstrating a typical noncaseating granuloma on a biopsy specimen. Other granulmatous diseases should be reasonably excluded. The current therapy of choice for sarcoidosis in children with multisystem involvement is oral corticosteroids. Methotrexate given orally in low doses has been effective, safe and steroid sparing in some patients. Alternative immunosuppressive agents, such as azathioprine, cyclophosphamide, chlorambucil, and cyclosporine, have been tried in adult cases

  14. Rainfall effects on rare annual plants

    Science.gov (United States)

    Levine, J.M.; McEachern, A.K.; Cowan, C.

    2008-01-01

    Variation in climate is predicted to increase over much of the planet this century. Forecasting species persistence with climate change thus requires understanding of how populations respond to climate variability, and the mechanisms underlying this response. Variable rainfall is well known to drive fluctuations in annual plant populations, yet the degree to which population response is driven by between-year variation in germination cueing, water limitation or competitive suppression is poorly understood.We used demographic monitoring and population models to examine how three seed banking, rare annual plants of the California Channel Islands respond to natural variation in precipitation and their competitive environments. Island plants are particularly threatened by climate change because their current ranges are unlikely to overlap regions that are climatically favourable in the future.Species showed 9 to 100-fold between-year variation in plant density over the 5–12 years of censusing, including a severe drought and a wet El Niño year. During the drought, population sizes were low for all species. However, even in non-drought years, population sizes and per capita growth rates showed considerable temporal variation, variation that was uncorrelated with total rainfall. These population fluctuations were instead correlated with the temperature after the first major storm event of the season, a germination cue for annual plants.Temporal variation in the density of the focal species was uncorrelated with the total vegetative cover in the surrounding community, suggesting that variation in competitive environments does not strongly determine population fluctuations. At the same time, the uncorrelated responses of the focal species and their competitors to environmental variation may favour persistence via the storage effect.Population growth rate analyses suggested differential endangerment of the focal annuals. Elasticity analyses and life table response

  15. Postoperative care of symphysiotomy performed for severe shoulder dystocia with fetal demise

    Directory of Open Access Journals (Sweden)

    Joy Anderson

    2017-04-01

    Précis: Symphysiotomy for severe shoulder dystocia is rarely utilized in the United States. We describe a case of symphysiotomy done for severe shoulder dystocia at an outside institution, and the patient's subsequent care at our institution.

  16. Two rare cases of laryngeal intralymphatic histiocytosis

    DEFF Research Database (Denmark)

    Reznitsky, Martin; Daugaard, Søren; Charabi, Birgitte Wittenborg

    2016-01-01

    We report two rare cases of intralymphatic histiocytosis causing, respectively, recurrent and persistent episodes of upper airway swelling and breathing difficulties. Case 1 was a 39-year-old man who was referred with recurrent upper airway swelling causing difficulty in breathing. A direct....... Extensive investigations were performed but discovered no abnormal findings. He received CO2 laser treatment twice and the swelling decreased. Intralymphatic histiocytosis is extremely rare in upper airway pathology. It is an important differential diagnosis in patients with recurrent and chronic laryngeal...

  17. Rare kaon decay: challenges and perspectives

    CERN Document Server

    D'Ambrosio, Giancarlo

    2015-01-01

    I review rare kaon decays. I introduce the flavor problem and p ossible solutions. Very rare kaon decays like K → π ν ̄ ν are very important to this purpose but also K → π l + l − . A new interesting channel is K → π π ee . Chiral dynamics is important to disentangle short distanc e effects. We discuss also the decays K 0 → μ + μ − , which have received recently some attention due to the measurement by LHCB.therefore we will study also K L → π 0 e + e − , K + → π + π 0 γ , K + → π + π 0 e + e − and related channels

  18. Excitons in the rare gas solids

    International Nuclear Information System (INIS)

    1988-01-01

    Excitons play a prominent role in the chemistry and physics of condensed matter. Excitons in the rare gas solids, the prototypical van der Waals insulators, will be the focus of the remainder of this report. The goal here is to investigate the controversies surrounding the description of excitons in insulators and, therefore the simplest class of these solids, namely the rare gas solids, is chosen as the exemplary system. Specific problems associated with molecular crystals are, therefore, avoided and only the salient features of excitons are thus considered. 47 refs., 9 figs., 4 tabs

  19. A rare anomaly: Double right coronary artery

    Directory of Open Access Journals (Sweden)

    Dursun Çayan Akkoyun

    2013-01-01

    Full Text Available Coronary artery anomalies are rare anomalies. Theseare usually asymptomatic and are discovered incidentally.Double right coronary artery (RCA is a rare coronaryartery anomaly. Although there is controversy aboutidentification and classification of double RCA, it is oftena benign condition, but it can be complicated by atherosclerosisand can lead to serious conditions such asmyocardial infarction (MI and may be accompanied byother anomalies. In our case, double RCA were detectedin coronary angiography for acute anterior MI, and in thenext session successful percutaneous coronary interventionwas performed.Key words: Coronary anomaly, coronary angiography,coronary stenosis

  20. Nuclear orientation on rare earth nickel alloys

    International Nuclear Information System (INIS)

    Nishimura, K.

    1998-01-01

    A hyperfine interaction study of the light rare earth elements, Ce, Pr, Nd and Pm, in the rare earth nickel and CeNi 2 Al 5 compounds by means of the low temperature nuclear orientation is summarised. The magnitudes and directions of the magnetic hyperfine fields obtained through measurements of γ-ray anisotropy and angular distributions reveal the magnetic structures of the ions. The experiments extracted peculiar results for the magnetic properties of the ions, and show certain novel features of the technique to the study of solid-state magnetism. Copyright (1998) Australian Journal of Physics

  1. Rare kaon decays at LAMPF II

    International Nuclear Information System (INIS)

    Sanford, T.W.L.

    1982-06-01

    At LAMPF II, intense beams of kaons will be available that will enable the rare kaon-decay processes to be investigated. This note explores some of the possibilities, which divide into two classes: (1) those that test the standard model of Weinberg and Salam and (2) those that are sensitive to new interactions. For both classes, experiments have been limited not by systematic errors but rather by statistical ones. LAMPF II with its intense flux of kaons thus will enable the frontier of rare kaon decay to be realistically probed

  2. Glomus tumor of penis- A rare case

    Directory of Open Access Journals (Sweden)

    Madhuri S. Kate

    2013-01-01

    Full Text Available Glomus tumors are rare, painful, and benign soft tissue tumors of the skin arising from the glomus body, an arteriovenous thermoregulatory structure. This lesion is usually found on the extremities. Glomus tumor involving the external genital organs including penis is extremely rare. Until now, only few cases have been reported in the available literature. A 22 year old male patient presented with a painful nodule over the penis. The pathologic diagnosis was glomus tumor of the glans penis. We report herewith a case of glomus tumor of penis diagnosed on histopathologic examination. Glomus tumor, being a benign neoplasm, complete extirpation of the glomus tumor is the treatment of choice.

  3. A rare presentation of hydatid cyst

    International Nuclear Information System (INIS)

    Sabir, N.; Yildirim, B.; Alatas, E.; Cetin, B.

    2005-01-01

    Hydatid disease, although known to occur in most body areas, is extremely rare in the female reproductive system. There are different modes of presentation for the disease; however, we report and discuss a case presented with cystic vesicles passing through the vagina, which is considered as a rare presentation for secondary involvement of the uterus and both ovaries. We confirmed diagnosis with radiological examinations and serological tests. We operated on the patient, and studied the excised cysts microscopically. The gynecologist should be aware of hydatid cyst when vaginally passing a grape like vesicle is presented by the patient. (author)

  4. Odontoameloblastoma: A rare case with unusual presentation

    Directory of Open Access Journals (Sweden)

    Supreet Jain

    2016-01-01

    Full Text Available The odontoameloblastoma (OA, also known as ameloblastic odontoma, is a very rare odontogenic tumor that is similar to ameloblastoma in its locally aggressive behavior. OA includes odontogenic ectomesenchyme in addition to odontogenic epithelium that resembles an ameloblastoma both in structure and in behavior. Its clinical presentation, however, often mimics the more innocuous odontoma, and hence, the recognition of its aggressive nature is commonly only ascertained after its histopathologic diagnosis following enucleation. This paper presents a rare case of OA with unusual clinical and radiological features.

  5. Ciliates and the rare biosphere: a review.

    Science.gov (United States)

    Dunthorn, Micah; Stoeck, Thorsten; Clamp, John; Warren, Alan; Mahé, Frédéric

    2014-01-01

    Here we provide a brief review of the rare biosphere from the perspective of ciliates and other microbial eukaryotes. We trace research on rarity from its lack of much in-depth focus in morphological and Sanger sequencing projects, to its central importance in analyses using high throughput sequencing strategies. The problem that the rare biosphere is potentially comprised of mostly errors is then discussed in the light of asking community-comparative, novel-diversity, and ecosystem-functioning questions. © 2014 The Author(s) Journal of Eukaryotic Microbiology © 2014 International Society of Protistologists.

  6. Features of rare earth element (3) complexing

    International Nuclear Information System (INIS)

    Martynenko, L.I.

    1991-01-01

    Reasons for nonobeyance to the regularity of tetrad ''W'' effect of rare earth chelate complex compounds are discussed in the review. The concept of metal-ligand ionic bond in rare earth complexes is put in the basis of the consideration. From this viewpoint mutual influence of ligands in lower, higher, polynuclear and different-ligand complexes, formed by the ligands of low, medium and high denticity, is discussed. Problems of intermolecular interaction of complexes with different structure are considered in relation to problems of variation of chelate volatility and selectivity in the processes of sublimation and precipitation

  7. CP violation in rare K decays

    International Nuclear Information System (INIS)

    Ecker, G.

    1990-01-01

    The investigation of rare K decays calls for a unified treatment of short- and long-distance aspects as provided by chiral perturbation theory. For the standard model with three generations, the theoretical predictions for signals of CP violation in those decays are reviewed. With direct CP violation as the main target, special emphasis is given to the charge asymmetries in charged K decays and to the especially rare decays K L → π 0 ll-bar. Time dependent rate asymmetries in K 0 decays and the longitudinal muon polarization in K L → μ + μ - are also discussed. 50 refs., 3 figs., 1 tab. (Author)

  8. Neurothekeoma of petrous apex: A rare entity

    Directory of Open Access Journals (Sweden)

    Zarina Abdul Assis

    2013-01-01

    Full Text Available Intraosseous nerve sheath tumors are very rare tumors accounting for lesser than 0.2% of primary bone tumors. We present an 18-year-old female who presented with left facial paresis for the last 1 year. Magnetic resonance imaging (MRI demonstrated expansile, multiseptated, enhancing bony lesion in the left petrous apex. There was also abnormal enhancement of the 7-8 th nerve complex within the internal auditory canal. Tumor was excised by subtemporal extradural approach. The lesion was diagnosed as intraosseous neurothekeoma on histopathology. This is an extremely rare tumor and its MRI appearance in this location is being described for the first time in literature.

  9. A novel rare sugar inhibitor of murine herpes simplex keratitis.

    Science.gov (United States)

    Muniruzzaman, Syed; McIntosh, Megan; Hossain, Ahamed; Izumori, Ken; Bhattacharjee, Partha S

    2016-06-01

    To determine the therapeutic efficacy of a novel rare sugar, l-psicose, for the treatment of HSV-1 induced herpetic stromal keratitis (HSK) in a mouse eye model. One rare sugar l-psicose was assayed for HSV-1 inhibition of in vitro virus adsorption. The IC50 and IC90 values of l-psicose were determined using plaque reduction assay (PRA) in CV-1 cell. Female Balb/c mice were corneally infected with HSV-1, strain KOS-GFP; A topical eye drop treatment of l-psicose was started 24 h after infection and continued four times daily for ten consecutive days. The severity of HSK was monitored by slit lamp examination in a masked fashion and Infectious HSV-1 shedding was determined by PRA. l-psicose was found to have anti-viral activity in vitro at an IC50 dose of 99.5 mM and an IC90 dose of 160 mM. Topical eye drop treatment with 200 mM l-psicose in PBS solution significantly reduced the severity of HSK compared to the mock treatment group. The in vivo mouse ocular model results of l-psicose therapy correlated with accelerated clearance of virus from eye swabs. The results suggest that topical treatment with rare sugar l-psicose has efficacy against HSK through inhibition of HSV-1. Copyright © 2016 The Authors. Production and hosting by Elsevier B.V. All rights reserved.

  10. Present Status of Inclusive Rare $B$ Decays

    CERN Document Server

    Hurth, Tobias

    2003-01-01

    We give a status report on inclusive rare B decays, highlighting recent developments and open problems. We focus on the decay modes $B \\to X_{s,d} \\gamma$, $ B \\to X_s \\ell^+\\ell^-$ and $B \\to X_s \

  11. Thermoluminescence dosimetry of rare earth doped calcium ...

    Indian Academy of Sciences (India)

    Unknown

    CaAl2O4) doped with different rare earth ions have been studied and their suitability for radiation dosimetry applications is discussed. It is observed that monocalcium aluminate doped with cerium is a good dosimeter having linear response up to ...

  12. Rare primary retroperitoneal teratoma masquerading as adrenal ...

    African Journals Online (AJOL)

    J.M. Ratkal

    Abstract. Objectives: To present a rare case of Primary mature cystic teratoma of right adrenal gland in adult female with an aim to review the published literature. Materials and Methods: The case details of a lady presenting with vague upper abdominal pain and on investigation was found to have a right adrenal mass were ...

  13. Spread of Rare Fungus from Vancouver Island

    Centers for Disease Control (CDC) Podcasts

    2006-12-20

    Cryptococcus gattii, a rare fungus normally found in the tropics, has infected people and animals on Vancouver Island, Canada. Dr. David Warnock, Director, Division of Foodborne, Bacterial, and Mycotic Diseases, CDC, discusses public health concerns about further spread of this organism.  Created: 12/20/2006 by Emerging Infectious Diseases.   Date Released: 12/29/2006.

  14. Ultrasonic attenuation in rare-earth monoarsenides

    Indian Academy of Sciences (India)

    2015-11-27

    Home; Journals; Pramana – Journal of Physics; Volume 86; Issue 6. Ultrasonic attenuation in rare-earth monoarsenides .... Proceedings of the International Workshop/Conference on Computational Condensed Matter Physics and Materials Science (IWCCMP-2015). Posted on November 27, 2015. Guest Editors: Anurag ...

  15. Rare earth oxyhalogenide base thermoluminescent material

    International Nuclear Information System (INIS)

    Rabatin, J.G.

    1976-01-01

    A process is described that consists to expose a thermoluminescent material to ionizing radiations, the material being a rare earth oxyhalogenide with terbium additions, to heat this material up to the emission of visible radiations and to measure the emitted radiations which are proportional to the ionizing radiation dose [fr

  16. Stamina and Clout Define This Rare Breed.

    Science.gov (United States)

    Harrington-Lueker, Donna

    1991-01-01

    Takeover artists are a rare breed. Persons hired to put bankrupt school systems back on the road to academic solvency need stamina, clout, and plenty of experience. For all their state-given powers, takeover superintendents must identify key constituencies, build bridges, and promote belief in change from within. (MLH)

  17. Genetic diversity among endangered rare Dalbergia ...

    African Journals Online (AJOL)

    Genetic diversity among endangered rare Dalbergia cochinchinensis (Fabaceae) genotypes in Vietnam revealed by random amplified polymorphic DNA (RAPD) ... The number of amplified fragments varied from 1 (OPR15, OPB05, RA142, OPR08, UBC348, OPE14 and OPO04) to 8 (OPP19) and their sizes ranged from 250 ...

  18. A rare case of Charlin's syndrome

    Directory of Open Access Journals (Sweden)

    Mohit P Singh

    2017-01-01

    Full Text Available Charlin's syndrome is an extremely rare condition characterized by pain in the nasal and paranasal areas, which is precipitated by touching the lateral aspect of the ipsilateral nostril. We are presenting one such case of a 42-year-old man who was admitted to Dr. Chaudhary Hospital and Medical Research Centre.

  19. Fetus in fetu: A very rare anomaly

    Directory of Open Access Journals (Sweden)

    Sanjay Choudhuri

    2014-11-01

    Full Text Available “Fetus in fetu” is a condition seen rarely in the literature, less than 200 cases reported till now. It is a calcified mass or an encapsulated pendunculated tumor thought to be due to unequal division of totipotent cells of a blastocyst, resulting in a small cellular mass in a more mature embryo thus forming a monozygotic, diamniotic twin pregnancy.

  20. Cerium and rare earth separation process

    International Nuclear Information System (INIS)

    Martin, M.; Rollat, M.

    1986-01-01

    An aqueous solution containing cerium III and rare earths is oxidized in the anodic compartment of an electrolytic cell, cerium IV is extracted by an organic solvent, the organic phase containing Ce IV is reduced in the catodic compartment of the same electrolytic cell and cerium III is extracted in a nitric aqueous phase [fr

  1. A Rare Seen Case Report: Sirenomelia

    Directory of Open Access Journals (Sweden)

    Nilay Piskinpasa

    2016-01-01

    Full Text Available Sirenomelia is a rare congenital anomaly characterized by abnormal development of the caudal body structures.The disease can be diagnosed antenatally by the careful ultrasound examination. In this paper we aimed to discuss a case which had one lower extremity,spinal angulations and anhydramnios image detected by ultrasound examination.We diagnosed sirenomelia after termination.

  2. Limb body wall complex: A rare anomaly

    Directory of Open Access Journals (Sweden)

    Panduranga Chikkannaiah

    2013-01-01

    Full Text Available We present autopsy findings of a case of limb body wall complex (LBWC. The fetus had encephalocele, genitourinary agenesis, skeletal anomalies and body wall defects. The rare finding in our case is the occurrence of both cranial and urogenital anomalies. The presence of complex anomalies in this fetus, supports embryonal dysplasia theory of pathogenesis for LBWC.

  3. Rare decays and search for new physics

    CERN Document Server

    Koppenburg, Patrick

    2014-01-01

    In absence of direct signs of new physics at the LHC, rare decays of heavy flavoured particles provide an ideal laboratory to look for deviations from the Standard Model and explore an energy regime beyond the LHC reach. Here, new results from the LHC and the $B$ factories are presented, with a particular focus on electroweak penguin-mediated $b\\rightarrow s$ transitions

  4. Lifetime measurements of the rare earths

    International Nuclear Information System (INIS)

    Stahnke, H.J.

    1981-01-01

    The lifetime of excited energy levels of Praseodymium, Neodymium, Gadolinium, Holmium and Erbium are measured. The measurements were done on atomic beams excited by laser radiation. The experimental results allow an interpretation of the electronic structure of the rare earths. (BEF)

  5. A rare cause of Cushing's syndrome

    DEFF Research Database (Denmark)

    Folkestad, Lars; Andersen, Marianne Skovsager; Nielsen, Anne Lerberg

    2014-01-01

    Excess glucocorticoid levels cause Cushing's syndrome (CS) and may be due to pituitary, adrenal or ectopic tumours. Adrenocorticotropic hormone (ACTH) levels are useful in identifying adrenal tumours. In rare cases, ACTH-producing phaeochromocytomas are the cause of CS. We present two cases of ACTH...

  6. Teratoid Wilms′ tumor - A rare renal tumor

    Directory of Open Access Journals (Sweden)

    Biswanath Mukhopadhyay

    2011-01-01

    Full Text Available Teratoid Wilms′ tumor is an extremely rare renal tumor. We report a case of unilateral teratoid Wilms′ tumor in a 4-year-old girl. The patient was admitted with a right-sided abdominal mass. The mass was arising from the right kidney. Radical nephrectomy was done and the patient had an uneventful recovery. Histopathology report showed teratoid Wilms′ tumor.

  7. A rare indication for liver resection.

    Science.gov (United States)

    Popescu, I; Zamfir, R; Braşoveanu, V; Boroş, M; Herlea, V

    2005-01-01

    We present the rare case of a young female with a right upper abdomen tumoral mass and suffering abdominal discomfort. A combination of ultrasonography, computed tomography, magnetic resonance imaging and laparotomy was utilized to conclude a diagnosis of Riedel's lobe. Laparotomy and a resection of Riedel's lobe were selected as the correct therapeutic solutions.

  8. Method for storing radioactive rare gases

    International Nuclear Information System (INIS)

    Watabe, Atsushi; Nagao, Hiroyuki; Takiguchi, Yukio; Kanazawa, Toshio; Soya, Masataka.

    1975-01-01

    Object: To safely and securely store radioactive rare gases for a long period of time. Structure: The waste gases produced in nuclear power plant are cooled by a cooler and then introduced into a low temperature adsorbing device so that the gases are adsorbed by adsorbents, and then discharged into atmosphere through the purifying gas discharge line. When the radioactive rare gases reach a level of saturation in the amount of adsorption, they are heated and extracted by a suction pump and heated by a heater. The gases are then introduced into an oxygen-impurity removing device and the purified rare gases containing no oxygen and impurities are cooled by a cooler and fed into a gas holder. When the amount of radioactive rare gases stored within the gas holder reaches a given level, they are compressed and sealed by a compressure into a storing cylinder and residual gases in the piping are sucked and recovered into the gas holder, after which the cylinder is removed and stored in a fixed room. (Kamimura, M.)

  9. Focal dermal hypoplasia: A rare case report

    Directory of Open Access Journals (Sweden)

    Sahana M Srinivas

    2015-01-01

    Full Text Available Focal dermal hypoplasia (Goltz syndrome is a rare genetic multisystem disorder primarily involving the skin, skeletal system, eyes, and face. We report the case of an eight-month-old female child who presented with multiple hypopigmented atrophic macules along the lines of blaschko, skeletal anomalies, umbilical hernia, developmental delay, hypoplastic nails, syndactyly, and lobster claw deformity characteristic of Goltz syndrome.

  10. A RARE CASE OF AZEOTROPIC COPOLYMERIZATION

    NARCIS (Netherlands)

    JONGSMA, T; KIMKES, P; CHALLA, G

    1991-01-01

    Copolymerizations with both reactivity ratios equal to one are rare. Therefore, we report here on the radical copolymerization of styrene and p-tert-butoxy-carbonyl-oxy-styrene in toluene with AIBN as initiator. We found it to be an azeotropic copolymerization for all compositions, i.e. f1 = F1 and

  11. Cervical myositis ossificans traumatica: a rare location

    International Nuclear Information System (INIS)

    Baysal, T.; Sarac, K.; Kutlu, R.; Baysal, O.; Ersoy, Y.; Elmali, N.

    1999-01-01

    An unusual case of myositis ossificans traumatica lesion located in the paraspinal region is reported. Despite the contiguity of the lesion with the cervical vertebrae and ominous appearance of the biopsy material, the history of antecedent trauma and computed tomography findings allowed preoperative accurate diagnosis. To our knowledge, myositis ossificans traumatica located in the cervical paraspinal region is very rare. (orig.)

  12. Plant for removing radioactive rare gases

    International Nuclear Information System (INIS)

    An, Buzai; Kanazawa, Toshio

    1977-01-01

    The outline of the pilot plant to remove and recover radioactive rare gases generated from nuclear power plants, reprocessing installations for nuclear fuel, nuclear research installations, etc. is described below. Among the studies of various processes such as liquefaction and distillation, absorption into solvents, active carbon adsorption, diaphragm method, etc., the liquefaction and distillation process by rectification at low temperature has been positively developed. It is in the stage of practical application for removing rare gases in waste gases from reprocessing and nuclear power plants. This is the process with high safety and excellent rare gas removing capability. Further research and development have been also made for selective adsorption and desorption method at low temperature which is very efficient as there is no release of long life nuclides such as Krypton-85. Rare gases recovered by the above mentioned removal systems must be stored safely for a long time as their half lives are long and specific radioactivities are high. The study has been made continuously on the storage methods including adsorption in cylinders and remotely automatically sealing storing system. (Kobatake, H.)

  13. Spread of Rare Fungus from Vancouver Island

    Centers for Disease Control (CDC) Podcasts

    Cryptococcus gattii, a rare fungus normally found in the tropics, has infected people and animals on Vancouver Island, Canada. Dr. David Warnock, Director, Division of Foodborne, Bacterial, and Mycotic Diseases, CDC, discusses public health concerns about further spread of this organism

  14. Anomalies in photofission of rare earth nuclei

    Energy Technology Data Exchange (ETDEWEB)

    Gann, A.V.; Nazarova, T.S.; Noga, V.I.; Ranyuk, Y.N.; Sorokin, P.V.; Telegin, Y.N.

    1979-09-01

    Measurements of photofission produced by 1-GeV bremsstrahlung in the heavy rare earth elements show an anomalously large cross section compared to that predicted by the liquid drop model. These measurements check the results obtained previously with 1-GeV protons by Andronenko et al. (JETP Lett. 24, 573 (1976)).

  15. Fournier gangrene: rare complication of rectal cancer.

    Science.gov (United States)

    Ossibi, Pierlesky Elion; Souiki, Tarik; Ibn Majdoub, Karim; Toughrai, Imane; Laalim, Said Ait; Mazaz, Khalid; Tenkorang, Somuah; Farih, My Hassan

    2015-01-01

    Fournier's Gangrene is a rare complication of rectal cancer. Its discovery is often delayed. It's incidence is about 0.3/100,000 populations in Western countries. We report a patient with peritoneal perforation of rectal cancer revealed by scrotal and perineal necrotizing fasciitis.

  16. Rare earth phosphors and phosphor screens

    International Nuclear Information System (INIS)

    Buchanan, R.A.; Maple, T.G.; Sklensky, A.F.

    1981-01-01

    Advances in the use of stabilized rare earth phosphors and of conversion screens using these materials are examined. In particular the new phosphors discussed in this invention consist of oxybromides of yttrium, lanthanum and gadolinium with a luminescent activator ion stabilized by an oxychloride or oxyfluoride surface layer and the conversion screens include trivalent cerium as the activator ion. (U.K.)

  17. Health Systems Sustainability and Rare Diseases.

    Science.gov (United States)

    Ferrelli, Rita Maria; De Santis, Marta; Egle Gentile, Amalia; Taruscio, Domenica

    2017-01-01

    The paper is addressing aspects of health system sustainability for rare diseases in relation to the current economic crisis and equity concerns. It takes into account the results of the narrative review carried out in the framework of the Joint Action for Rare Diseases (Joint RD-Action) "Promoting Implementation of Recommendations on Policy, Information and Data for Rare Diseases", that identified networks as key factors for health systems sustainability for rare diseases. The legal framework of European Reference Networks and their added value is also presented. Networks play a relevant role for health systems sustainability, since they are based upon, pay special attention to and can intervene on health systems knowledge development, partnership, organizational structure, resources, leadership and governance. Moreover, sustainability of health systems can not be separated from the analysis of the context and the action on it, including fiscal equity. As a result of the financial crisis of 2008, cuts of public health-care budgets jeopardized health equity, since the least wealthy suffered from the greatest health effects. Moreover, austerity policies affected economic growth much more adversely than previously believed. Therefore, reducing public health expenditure not only is going to jeopardise citizens' health, but also to hamper fair and sustainable development.

  18. Process technology - rare and refractory metals

    International Nuclear Information System (INIS)

    Gupta, C.K.; Bose, D.K.

    1989-01-01

    India has fairly rich resreves of rare and refractory metals. Abundant sources of ilmenite, rutile, zircon and rare earths are found in the placer deposits of the southern and eastern coasts of the country. Columbite-tantalite occur in mica and the mining belts of Bihar and cassiterite deposits are found in Bastar (Madhya Pradesh). Vanadium as a minor associate occurs in bauxites and in the vast deposits of titaniferrous magnetites. Over the years, research and development and pilot plant works in many research organisations in India have built up a sound technological base in the country for process metallurgy of many refractory and rare earth metals starting from their indigenous sources. The present paper provides a comprehensive view of the developments that have taken place till now on the processing of various refractory and rare earth metals with particular reference to the extensive work carried out at the Department of Atomic Energy. The coverage includes mineral benification separation of individual elements, preparation of pure intermediates, techniques of reduction to metal and final purification. The paper also reviews some of the recent developments that have been taken place in these fields and the potential application of these metals in the foreseeable future. (author). 22 refs., 18 fi g., 7 tabs

  19. Enamel renal syndrome: A rare case report

    Directory of Open Access Journals (Sweden)

    S V Kala Vani

    2012-01-01

    Full Text Available Enamel renal syndrome is a very rare disorder associating amelogenesis imperfecta with nephrocalcinosis. It is known by various synonyms such as amelogenesis imperfecta nephrocalcinosis syndrome, MacGibbon syndrome, Lubinsky syndrome, and Lubinsky-MacGibbon syndrome. It is characterized by enamel agenesis and medullary nephrocalcinosis. This paper describes enamel renal syndrome in a female patient born in a consanguineous family.

  20. Non-rare earth magnetic nanoparticles

    Science.gov (United States)

    Carpenter, Everett E.; Huba, Zachary J.; Carroll, Kyler J.; Farghaly, Ahmed; Khanna, Shiv N.; Qian, Meichun; Bertino, Massimo

    2017-09-26

    Continuous flow synthetic methods are used to make single phase magnetic metal alloy nanoparticles that do not contain rare earth metals. Soft and hard magnets made from the magnetic nanoparticles are used for a variety of purposes, e.g. in electric motors, communication devices, etc.

  1. Timbuktu Rare Manuscripts Project: Promoting African partnerships ...

    African Journals Online (AJOL)

    This article describes the Timbuktu Rare Manuscripts Project. The project is concerned with providing assistance to Mali in the conservation and preservation of the rich trove of manuscripts to be found in and around the immediate environs of Timbuktu. The project is a result of a bilateral agreement concluded between the ...

  2. Multiple familial trichoepithelioma: a rare cutaneous tumour

    International Nuclear Information System (INIS)

    Arfan-ul-Bari; Simeen-ber-Rehman

    2004-01-01

    Multiple familial trichoepithelioma (MFT) is a rare autosomal dominant skin disease that presents as many small tumours predominantly on the face. We report a case of multiple familial trichoepithelioma occurring in three members of a family. They were diagnosed simultaneously. Only one was treated with medium depth chemical peeling with partial response. (author)

  3. Bisalbuminemia: A Rare Variant of Albumin

    Directory of Open Access Journals (Sweden)

    Carla Garcez

    2017-04-01

    Discussion: We describe a rare case of hereditary bisalbuminemia in a portuguese family. In general, this condition shows no pathological significance, however it is of interest to the clinicians the knowledge of this analytic change for better orientation of their patients.

  4. Severe accident phenomena

    International Nuclear Information System (INIS)

    Jokiniemi, J.; Kilpi, K.; Lindholm, I.; Maekynen, J.; Pekkarinen, E.; Sairanen, R.; Silde, A.

    1995-02-01

    Severe accidents are nuclear reactor accidents in which the reactor core is substantially damaged. The report describes severe reactor accident phenomena and their significance for the safety of nuclear power plants. A comprehensive set of phenomena ranging from accident initiation to containment behaviour and containment integrity questions are covered. The report is based on expertise gained in the severe accident assessment projects conducted at the Technical Research Centre of Finland (VTT). (49 refs., 32 figs., 12 tabs.)

  5. Processes for the production of rare earths from monazite (Paper No. 36)

    International Nuclear Information System (INIS)

    Murthy, T.K.S.

    1979-01-01

    A few typical cases are briefly described to illustrate different methods available for rare earth concentration and separation from mixed rare earths chloride obtained for monazite. In the case of cerium, rare earths chloride mixture is treated with sodium sulphate to precipitate rare earths as double sulphates from which hydroxide cake is prepared. The cake is dried to oxidise cerium. Trivalent rare earths are selectively leached and the product is treated with HNO 3 . The resulting ceric nitrate solution is purified by liquid-liquid extraction using TBP. The scrubbed extract is reduced with H 2 O 2 and cerous nitrate is recovered by evaporation. Lanthanum is first concentrated by selective precipitation of hydroxides from rare earths chloride using air-ammonia mixture. The hydroxyde cake is dissolved in HNO 3 and NH 4 NO 3 to get the double nitrate which is subjected to counter current crystallisation for purification. Europium is present at a concentration of 0.01% in the rare earths chloride. It is concentrated in several steps by liquid-liquid extraction using di-2-ethyl hexyl phosphoric acid. The product is purified by selective reduction and europium is obtained as europium sulphate. In the same solvent extraction process samarium and gadolinium are also concentrated to about 25%. They are further upgraded to above 90% purity by repetion of liquid-liquid extraction technique. Cerium, lanthanum and europium obtained by the above processes analyse > 99% as oxides. (M.G.B.)

  6. Investigation of the role of TCF4 rare sequence variants in schizophrenia.

    Science.gov (United States)

    Basmanav, F Buket; Forstner, Andreas J; Fier, Heide; Herms, Stefan; Meier, Sandra; Degenhardt, Franziska; Hoffmann, Per; Barth, Sandra; Fricker, Nadine; Strohmaier, Jana; Witt, Stephanie H; Ludwig, Michael; Schmael, Christine; Moebus, Susanne; Maier, Wolfgang; Mössner, Rainald; Rujescu, Dan; Rietschel, Marcella; Lange, Christoph; Nöthen, Markus M; Cichon, Sven

    2015-07-01

    Transcription factor 4 (TCF4) is one of the most robust of all reported schizophrenia risk loci and is supported by several genetic and functional lines of evidence. While numerous studies have implicated common genetic variation at TCF4 in schizophrenia risk, the role of rare, small-sized variants at this locus-such as single nucleotide variants and short indels which are below the resolution of chip-based arrays requires further exploration. The aim of the present study was to investigate the association between rare TCF4 sequence variants and schizophrenia. Exon-targeted resequencing was performed in 190 German schizophrenia patients. Six rare variants at the coding exons and flanking sequences of the TCF4 gene were identified, including two missense variants and one splice site variant. These six variants were then pooled with nine additional rare variants identified in 379 European participants of the 1000 Genomes Project, and all 15 variants were genotyped in an independent German sample (n = 1,808 patients; n = 2,261 controls). These data were then analyzed using six statistical methods developed for the association analysis of rare variants. No significant association (P power analyses suggest that further research into the possible involvement of rare TCF4 sequence variants in schizophrenia risk is warranted by the assessment of larger cohorts with higher statistical power to identify rare variant associations. © 2015 Wiley Periodicals, Inc.

  7. A rare case of bleeding disorder: Glanzmann's thrombasthenia.

    Science.gov (United States)

    Swathi, Jami; Gowrishankar, A; Jayakumar, S A; Jain, Karun

    2017-01-01

    Glanzmann's thrombasthenia (GT) is a rare bleeding disorder, which is characterized by a lack of platelet aggregation. It is characterized by qualitative or quantitative abnormalities of the platelet membrane glycoprotein IIb/IIIa. Physiologically, this platelet receptor normally binds several adhesive plasma proteins, and this facilitates attachment and aggregation of platelets to ensure thrombus formation at sites of vascular injury. The lack of resultant platelet aggregation in GT leads to mucocutaneous bleeding whose manifestation may be clinically variable, ranging from easy bruising to severe and potentially life-threatening hemorrhages. To highlight this rare but potentially life-threating disorder, GT. We report a case of GT that was first detected because of the multiple episodes of gum bleeding. The patient was an 18-year-old girl who presented with a history of repeated episodes of gum bleeding since childhood. Till the first visit to our hospital, she had not been diagnosed with GT despite a history of bleeding tendency, notably purpura in areas of easy bruising, gum bleeding, and prolonged bleeding time after abrasions and insect stings. GT was diagnosed on the basis of prolonged bleeding time, lack of platelet aggregation with adenosine di phosphate, epinephrine and collagen. GT should always be considered as differential diagnosis while evaluating any case of bleeding disorder.

  8. Mucormycosis: a rare fungal infection in tornado victims.

    Science.gov (United States)

    Austin, Cindy L; Finley, Phillip J; Mikkelson, Debbie R; Tibbs, Brian

    2014-01-01

    This article reviews four immunocompetent patients who developed a rare fungal infection, mucormycosis, secondary to multiple traumatic injuries sustained during an EF-5 tornado in Joplin, MO. Commonly found in soil and decaying organic matter, mucorales are fungi associated with soft tissue and cutaneous infections. Onset of this fungal infection can occur without clinical signs, presenting several days to several weeks after injury, delaying diagnosis. A multidisciplinary treatment approach including aggressive antifungal therapy and aggressive surgical debridement is critical. This diagnosis should be considered in all patients presenting with injuries sustained from high-velocity embedment of debris such as natural disasters or explosions. We present four cases of mucormycosis, species Apophysomyces trapeziformis. Data reported includes predisposing factors, number of days between injury and diagnosis of mucormycosis, surgical treatment, antifungal therapy, outcomes, and potential risk factors that may have contributed to the development of mucormycosis.

  9. A Rare Case of Neonatal Hypophosphatasia: A Case Report

    Directory of Open Access Journals (Sweden)

    Nasim Pouralizadeh

    2018-03-01

    Full Text Available Hypophosphatasia is a rare hereditary disorder of bone metabolism.In this article, we presented the case of a male infant with a soft skull and short, deformed limbs at birth, followed by seizures and respiratory distress during admission in the neonatal intensive care unit (NICU. Prenatal ultrasound showed limb hypoplasia, skull hypomineralization, and polyhydramnios. Seizures occurred on day nine of admission. The neonate was intubated after pneumonia on day 12of birth and died due to the same cause and respiratory failure on day 14. Clinical presentation and low alkaline phosphatase (ALP confirmed the diagnosis of hypophosphatasia. The disorder covers a spectrum of severe neonatal type with severe hypomineralization to various adult types with osteomalacia and dental problems. Prenatal hypophosphatasia is diagnosed based on the clinical signs, including soft skull, short limbs, breathing difficulty, seizures, respiratory distress, laboratory results (low ALP and high pyridoxal 5-phosphate, and radiographic findings (hypomineralization and metaphyseal dysplasia.

  10. CT findings in severe thoracic sarcoidosis

    International Nuclear Information System (INIS)

    Hennebicque, Anne-Sophie; Brillet, Pierre-Yves; Moulahi, Hassen; Brauner, Michel W.; Nunes, Hilario; Valeyre, Dominique

    2005-01-01

    Severe thoracic sarcoidosis includes manifestations with significant clinical and functional impairment and a risk of mortality. Severe thoracic sarcoidosis can take on various clinical presentations and is associated with increased morbidity. The purpose of this article was to describe the CT findings in severe thoracic sarcoidosis and to explain some of their mechanisms. Subacute respiratory insufficiency is a rare and early complication due to a high profusion of pulmonary lesions. Chronic respiratory insufficiency due to pulmonary fibrosis is a frequent and late complication. Three main CT patterns are identified: bronchial distortion, honeycombing and linear opacities. CT can be helpful in diagnosing some mechanisms of central airway obstruction such as bronchial distortion due to pulmonary fibrosis or an extrinsic bronchial compression by enlarged lymph nodes. An intrinsic narrowing of the bronchial wall by endobronchial granulomatous lesions may be suggested by CT when it shows evidence of bronchial mural thickening. Pulmonary hypertension usually occurs in patients with end-stage pulmonary disease and is related to fibrotic destruction of the distal capillary bed and to the resultant chronic hypoxemia. Several other mechanisms may contribute to the development of pulmonary hypertension including extrinsic compression of major pulmonary arteries by enlarged lymph nodes and secondary pulmonary veno-occlusive disease. Aspergilloma colonization of a cavity is the main cause of hemoptysis in sarcoidosis. Other rare causes are bronchiesctasis, necrotizing bronchial aspergillosis, semi-invasive pulmonary aspergillosis, erosion of a pulmonary artery due to a necrotic sarcoidosis lesion, necrosis of parenchymal sarcoidosis lesions and specific endobronchial macroscopic lesions. (orig.)

  11. Genetic/metabolic effect of iron metabolism and rare anemias

    Directory of Open Access Journals (Sweden)

    Clara Camaschella

    2013-03-01

    Full Text Available Advances in iron metabolism have allowed a novel classification of iron disorders and to identify previously unknown diseases. These disorders include genetic iron overload (hemochromatosis and inherited iron-related anemias, in some cases accompanied by iron overload. Rare inherited anemias may affect the hepcidin pathway, iron absorption, transport, utilization and recycling. Among the genetic iron-related anemias the most common form is likely the iron-refractory iron-deficiency anemia (IRIDA, due to mutations of the hepcidin inhibitor TMPRSS6 encoding the serine protease matriptase-2. IRIDA is characterized by hepcidin up-regulation, decrease iron absorption and macrophage recycling and by microcytic- hypochromic anemia, unresponsive to oral iron. High serum hepcidin levels may suggest the diagnosis, which requires demonstrating the causal TMPRSS6 mutations by gene sequencing. Other rare microcytic hypochromic anemias associated with defects of iron transport-uptake are the rare hypotransferrinemia, and DMT1 and STEAP3 mutations. The degree of anemia is variable and accompanied by secondary iron overload even in the absence of blood transfusions. This is due to the iron-deficient or expanded erythropoiesis that inhibits hepcidin transcription, increases iron absorption, through the erythroid regulator, as in untransfused beta-thalassemia. Sideroblastic anemias are due to decreased mitochondrial iron utilization for heme or sulfur cluster synthesis. Their diagnosis requires demonstrating ringed sideroblasts by Perl’s staining of the bone marrow smears. The commonest X-linked form is due to deltaamino- levulinic-synthase-2-acid (ALAS2 mutations. The recessive, more severe form, affects SLC25A38, which encodes a potential mitochondrial importer of glycine, an amino acid essential for ALA synthesis and thus results in heme deficiency. Two disorders affect iron/sulfur cluster biogenesis: deficiency of the ATP-binding cassette B7 (ABCB7 causes X

  12. De-severing distance

    DEFF Research Database (Denmark)

    Jensen, Hanne Louise; de Neergaard, Maja

    2016-01-01

    De-severing Distance This paper draws on the growing body of mobility literature that shows how mobility can be viewed as meaningful everyday practices (Freudendal –Pedersen 2007, Cresswell 2006) this paper examines how Heidegger’s term de-severing can help us understand the everyday coping with ...

  13. Severe Bleeding: First Aid

    Science.gov (United States)

    ... 12, 2017. Jevon P, et al. Part 5 — First-aid treatment for severe bleeding. Nursing Times. 2008;104:26. Oct. 19, 2017 Original article: http://www.mayoclinic.org/first-aid/first-aid-severe-bleeding/basics/ART-20056661 . Mayo ...

  14. Allergy in severe asthma

    NARCIS (Netherlands)

    Del Giacco, Stefano R.; Bakirtas, A.; Bel, E.; Custovic, A.; Diamant, Z.; Hamelmann, E.; Heffler, E.; Kalayci, O.; Saglani, S.; Sergejeva, S.; Seys, S.; Simpson, A.; Bjermer, Leif

    It is well recognized that atopic sensitization is an important risk factor for asthma, both in adults and in children. However, the role of allergy in severe asthma is still under debate. The term 'Severe Asthma' encompasses a highly heterogeneous group of patients who require treatment on steps

  15. Pregnancy-associated severe sepsis.

    Science.gov (United States)

    Oud, Lavi

    2016-04-01

    This article examines the contemporary knowledge and uncertainties about the burden of pregnancy-associated severe sepsis (PASS), and its manifestations, management, and outcomes. There are relatively sparse data on PASS, related in part to infrequent reports and varying use of terminology and case definitions. PASS remains rare, although its incidence appears to be rapidly rising in some high-resource countries, affecting especially women with limited resources, minorities, and those with chronic illness. High level of clinician vigilance and rapid initiation of appropriate antimicrobial therapy, coupled with effective systemic support for organ dysfunction and correction of occult and overt hypoperfusion are the keys to limit adverse outcomes. However, timely diagnosis and provision of effective care remain a challenge, with reported prevalent delay in recognition and delivery of time-sensitive care interventions among maternal decedents. The mortality rate of PASS has been rising and its case fatality, although relatively low, has remained unchanged, in contrast to the outcome gains in the general population. The long-term sequelae of PASS remain unknown. The relatively limited contemporary data on PASS suggest a rising public health hazard in the obstetric population in high-resource countries, with ongoing challenges in assuring consistent provision of time-sensitive care.

  16. Currency flaw severity. [Banknotes

    Energy Technology Data Exchange (ETDEWEB)

    Johnson, C.; Burnett, M.; Goodman, C.; Sherrod, R.; Schmoyer, R.; Harrison, C.; Uppuluri, R.

    1986-01-01

    A survey of currency flaw severity was carried out using 300 banknotes and 37 judges. Each judge assigned each note to one of five flaw severity categories. These categories correspond to severity grades of 1 to 5 with 1 equivalent to ''always accepted'' and 5 ''never accepted.'' An average flaw severity grade for each note was obtained by taking the mean of the severity grades assigned to that note by the 37 judges. Thus, each note has a single numerical real-number flaw grade between 1 and 5. Mathematical modeling of the currency flaw survey results is continuing with some very promising initial results. Our present model handles common excess ink and missing ink flaw types quite well. We plan to extend the model to ink level, mash, setoff and blanket impression flaw types.

  17. Collaborative research efforts and related activities of the Office of Rare Diseases Research at the USA National Institutes of Health

    Directory of Open Access Journals (Sweden)

    Stephen C. Groft

    2009-12-01

    Full Text Available

    Introduction: Rare diseases present unique challenges to meet the numerous and varied needs of the rare diseases community and it is required to identify and address these needs. Significant financial and personnel resources are required to address these needs identified. The Office of Rare Diseases Research (ORDR at the USA National Institutes of Health (NIH has attempted to meet many of these needs in collaborative efforts with the research Institutes and Centers of NIH and other partners in the private and public sectors in the USA and around the world. Several of the activities of the NIH and the ORDR are presented as possible collaborative efforts available to research investigators and include the Rare Diseases Clinical Research Network, the Bench-to-Bedside research program at NIH, the Genetic and Rare Diseases Information center, the genetic test development program, and the information on clinical research studies made available through Clinical trials.gov. The value of an appropriate family medical history is discussed as are the provisions of the Genetic Information Non-Discrimination Act of 2008 (GINA. Definitions of rare or orphan diseases vary from country to country and may cause some confusion to the rare diseases community.

    Conclusions: Rare diseases are not limited by geographical or historical boundaries and global partnerships of the rare diseases community are experiencing rapid expansion to assist in the development of orphan products for the prevention, diagnosis and treatment of rare diseases and conditions. The unmet needs of the rare diseases community require additional innovative research and educational programs to reach the extensive global populations affected by the thousands of different rare diseases including activities with the National Organization for Rare Disorders and the Genetic Alliance.

  18. Intra-group separation of rare earths using new organic phosphorus ligands

    International Nuclear Information System (INIS)

    Hadic, Sanela

    2017-01-01

    the decomposition products had been identified, reaction paths were proposed. The impact of this decomposition on the separation of rare earths was followed, in single extraction experiments, over a period of up to several months.

  19. Proceedings of the national conference on rare earth processing and utilization - 2014: abstracts

    International Nuclear Information System (INIS)

    Anitha, M.; Dasgupta, Kinshuk; Singh, D.K.

    2014-01-01

    The rare earth elements (REEs) are becoming increasingly important in the transition to a low-carbon, circular economy, considering their essential role in permanent magnets, lamp phosphors, rechargeable nickel metal hydride batteries and catalysts and other green applications. The increasing popularity of hybrid and electric cars, wind turbines and compact fluorescent lamps is causing an increase in the demand and price of REEs. The European Commission considers the REEs as the most critical raw materials group, with the highest supply risk. According to the medium-term criticality matrix of the U.S. Department of Energy (DOE), the five most critical REEs are neodymium (Nd), europium (Eu), terbium (Tb), dysprosium (Dy) and yttrium (Y). China is presently producing more than 90% of all rare earths, although they possess less than 40% of the proven reserves. Due to large and increasing domestic demands, China tightened its REE export quota from 2012 onwards. These export quotas caused serious problems for REE users outside of China. To tackle the REE supply challenge, several approaches have been proposed. Fortunately India is blessed with large resources of rare earths in the form of monazite found in the beach sands of Kerala, Tamil Nadu and Odisha. Indian Rare Earths Limited at Aluva near Kochi used to produce mainly mixed rare earths chloride and export to USA, UK, France, Japan, etc. During the 1990s and early 2000s this plant exported pure oxides of samarium, neodymium, etc. to developed countries. This national conference has expanded its canvas by including newer emerging areas in rare earths recycling, environmental issues, recent advances in rare earth material science, rare earth research and development initiatives around the world which provide a platform for the growth of rare earth Industry. Papers relevant to INIS are indexed separately

  20. Severe recurrent achalasia cardia responding to treatment of severe autoimmune acquired haemophilia

    OpenAIRE

    Al-Jafar, H; Laffan, M; Al-Sabah, S; Elmorsi, M; Habeeb, M; Alnajar, F

    2012-01-01

    Acquired haemophilia A and severe acquired achalasia are both very rare conditions with unknown aetiology. Haemophilia A is a haemorrhagic disease induced by deficiency or malfunction of coagulation factor VIII. Congenital haemophilia is an inherited disease transmitted by the mother through X-linked inheritance and primarily affects males. However, acquired haemophilia A is a serious, sudden-onset, autoimmune disease that affects either sex. In addition, achalasia is a disease of the oesopha...

  1. Severe childhood malnutrition

    DEFF Research Database (Denmark)

    Bhutta, Zulfiqar A; Berkley, James A; Bandsma, Robert H J

    2017-01-01

    The main forms of childhood malnutrition occur predominantly in children malnutrition. Here, we use...... the term 'severe malnutrition' to describe these conditions to better reflect the contributions of chronic poverty, poor living conditions with pervasive deficits in sanitation and hygiene, a high prevalence of infectious diseases and environmental insults, food insecurity, poor maternal and fetal...... nutritional status and suboptimal nutritional intake in infancy and early childhood. Children with severe malnutrition have an increased risk of serious illness and death, primarily from acute infectious diseases. International growth standards are used for the diagnosis of severe malnutrition and provide...

  2. Reverse (posterior) cloaca with congenital rectal stenosis and pseudohermaphrodism: a rare entity with rare association

    Science.gov (United States)

    Ahmed, Intezar; Kureel, Shiv Narain; Chandra, Naveen

    2010-01-01

    Reverse (posterior) cloaca is itself a rare entity and association of this defect with other congenital syndromes or defects is extremely rare to find. To diagnose this unique anomaly one should be aware about this very infrequently encountered clinical entity. Reverse cloaca is a very rare variant of anorectal malformation in which urethra and vagina fused to form a urogenital sinus that diverts posteriorly and opens in the anterior rectal wall at the anus or immediately anterior to it. We report a neonate with the rare clinical entity of reverse cloaca type C, pseudophallus with rudimentary urethra as a component of female pseudohermaphrodism and congenital rectal stenosis. Surprisingly, there was no associated urological abnormality on ultrasonography and laparotomy. PMID:22797480

  3. Several complex variables

    International Nuclear Information System (INIS)

    Field, M.J.

    1976-01-01

    Topics discussed include the elementary of holomorphic functions of several complex variables; the Weierstrass preparation theorem; meromorphic functions, holomorphic line bundles and divisors; elliptic operators on compact manifolds; hermitian connections; the Hodge decomposition theorem. ( author)

  4. Severe malaria in Europe

    DEFF Research Database (Denmark)

    Kurth, Florian; Develoux, Michel; Mechain, Matthieu

    2017-01-01

    BACKGROUND: Malaria remains one of the most serious infections for travellers to tropical countries. Due to the lack of harmonized guidelines a large variety of treatment regimens is used in Europe to treat severe malaria. METHODS: The European Network for Tropical Medicine and Travel Health (Trop......Net) conducted an 8-year, multicentre, observational study to analyse epidemiology, treatment practices and outcomes of severe malaria in its member sites across Europe. Physicians at participating TropNet centres were asked to report pseudonymized retrospective data from all patients treated at their centre...... for microscopically confirmed severe Plasmodium falciparum malaria according to the 2006 WHO criteria. RESULTS: From 2006 to 2014 a total of 185 patients with severe malaria treated in 12 European countries were included. Three patients died, resulting in a 28-day survival rate of 98.4%. The majority of infections...

  5. Investigation of rare elements by electrochemical methods

    International Nuclear Information System (INIS)

    Zarinskij, V.A.

    1988-01-01

    The use of electrochemical methods for the study of complexing, separation of rare element mixtures, their preparation in lower oxidation states, and also for the development of highly sensitive methods of the element determination, is considered in the review. Voltammetric methods of Pt, Au, Re determination are considered, as well as Re preparation in oxidation states +5, +3 by electrolytic methods. The possibility to use electrodialysis methods for purification of insoluble compounds of rare earths (RE) from impurities, and for separation of Re and Mo with simultaneous purification of Re from K and other elements is shown. The application of high-frequency conductometry to analytic chemistry and to the study of Th, In, RE complexing and kinetics of the reactions is considered

  6. Rare K decays: Challenges and Perspectives

    International Nuclear Information System (INIS)

    Smith, C.

    2014-09-01

    At this stage of the LHC (Large Hadron Collider) program, the prospect for a new physics signal in the very rare K → Kνν-bar decays may be dented, but remains well alive thanks to their intrinsic qualities. First, these decays are among the cleanest observables in the quark flavor sector. When combined with their terrible suppression in the SM (Standard Model), they thus offer uniquely sensitive probes. Second, the LHC capabilities are not ideal for all kinds of new physics, even below the TeV scale. For example, rather elusive scenarios like natural-SUSY-like hierarchical spectrum, baryon number violation, or new very light but very weakly interacting particles may well induce deviations in rare K decays. Even though experimentalists should brace themselves for tiny deviations, these modes thus have a clear role to play in the LHC era. (author)

  7. Zosteriform cutaneous leiomyoma: a rare cutaneous neoplasm

    International Nuclear Information System (INIS)

    Bari, A.U.

    2013-01-01

    Cutaneous leiomyomas are firm, round to oval, skin-coloured to brownish papules and nodules that may present as a solitary, few discrete or multiple clustered lesions. Different uncommon patterns of multiple leiomyoma distribution have been noted as bilateral, symmetrical, linear, zosteriform, or dermatomal-like arrangement. One such rare presentation was seen in a 23-year-old patient who presented with zosteriform skin coloured, occasionally painful cutaneous lesions over left shoulder region. Histopathology confirmed the diagnosis of cutaneous leiomyoma. He was symptomatically managed with non-steroidal anti-inflammatory agents and topical capcicum cream. Case is reported here due to rare occurrence of this benign cutaneous neoplasm in an atypical pattern and on uncommon site. (author)

  8. The detection of clusters in rare diseases

    Energy Technology Data Exchange (ETDEWEB)

    Besag, J. (Washington Univ., Seattle, WA (USA) Newcastle upon Tyne Univ. (UK)); Newell, J. (Newcastle upon Tyne Univ. (UK))

    1991-01-01

    Tests for clustering of rare diseases investigate whether an observed pattern of cases in one or more geographical regions could reasonably have arisen by chance alone, bearing in mind the variation in background population density. In contrast, tests for the detection of clusters are concerned with screening a large region for evidence of individual 'hot spots' of disease but without any preconception about their likely locations; the results of such tests may form the basis for subsequent small area investigations, statistical or non-statistical, but will rarely be an end in themselves. The main intention of the paper is to describe and illustrate a new technique for the identification of small clusters of disease. A secondary purpose is to discuss some common pitfalls in the application of tests of clustering to epidemiological data. (author).

  9. Oral lymphangioma: A rare case report

    Directory of Open Access Journals (Sweden)

    Harsha Bhayya

    2015-01-01

    Full Text Available Lymphangiomas are benign hamartomatous tumors of the lymphatic channels which present as developmental malformations arising from sequestration of lymphatic tissue that do not communicate with the rest of the lymphatic channels. Lymphatic vessels are filled with a clear protein-rich fluid containing few lymph cells. It can also occur in association with hemangioma. The onset of lymphangiomas are either at birth (60% to 70% or up to two years of age (90% and rare in adults. Lymphangiomas have marked predilection for the head and neck region (50-70%. The most common location in the mouth is the dorsum of tongue, followed by lips, buccal mucosa, soft palate, and floor of the mouth. On tongue, they may present as a localized or a diffused growth which may enlarge to cause macroglossia, impaired speech and difficulty in mastication. Herewith, we present a rare case of lymphangioma of tongue leading to macroglossia in a 8-year-old boy.

  10. Rare earth oxide doping in oxide cathodes

    International Nuclear Information System (INIS)

    Engelsen, Daniel den; Gaertner, Georg

    2006-01-01

    The effect on life performance and poisoning with O 2 by doping oxide cathodes with rare earth oxides and pseudo rare earth oxides, notably yttria, is qualitatively explained in terms of electrolysis of BaO during emission of electrons. Doped cathodes show less electrolysis and consume therefore less Ba during life: consequently, doped cathodes have a better life performance. However, the lower Ba-production makes doped cathodes more sensitive to oxygen poisoning. The experimentally found relation between conductivity and yttria concentration was the motive to propose a new model for the crystal imperfections in BaO. In this new imperfection model most Y 3+ -ions will combine with barium vacancies, therefore, the increase of the conductivity is modest and also the effect on the position of the Fermi level is modest. By assuming a combination of bulk and surface conductivity, the agreement between experiment and theory can be improved further

  11. Pancreatic pseudopapillary tumour: A rare misdiagnosed entity.

    Science.gov (United States)

    Affirul, C A; Qisti, F N; Zamri, Z; Azlanuddin, A; Hairol, A O; Razman, J

    2014-01-01

    Solid pseudo papillary pancreatic tumour is a rare entity. The atypical presentation causes a delayed or misdiagnosis of these pathology. It commonly affects the female population in the 2nd and 3rd decade of life. The presentation varies from non-specific abdominal pain to incidental findings in asymptomatic patients. It is a low-grade premalignant condition that is curable by excision of the tumour. This paper presents a 17-year-old girl with intra-abdominal mass diagnosed with solid pseudo papillary tumour that underwent Whipple's procedure. We discuss the presentations, diagnosis and pathology findings of this rare pathology. The diagnosis remains an enigma in view of the nature and location of the tumour. Resection is still the best choice remains for this condition. Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved.

  12. Bioproduction strategies for rare hexose sugars

    Science.gov (United States)

    Izumori, Ken

    2002-03-01

    A new strategy for the bioproduction of all ketohexoses was developed using hexitols as intermediates. Biocatalysts used to employ the strategy were D-tagatose 3-epimerase, which epimerizes ketohexoses at the C-3 position, and oxidoreductases, which catalyze oxidation-reduction reactions between ketohexoses and the corresponding hexitols. Arranging all the ketohexoses and hexitols in a symmetric ring and connecting them with 20 biochemical reactions, I was able to construct a design for the bioproduction of all the rare ketohexoses. Various aldose isomerases transform ketohexoses into the corresponding aldohexoses, so the strategy is useful for the bioproduction of all the rare hexose sugars. Furthermore, the design revealed that there are four routes to the L-hexose world from the D-hexose one.

  13. Adult primary hypoparathyroidism: A rare presentation

    Directory of Open Access Journals (Sweden)

    Ashima Datey Chakrabarty

    2013-01-01

    Full Text Available The common causes of stridor in adults are abscesses or swelling of upper airway, tumors, paralysis or malfunction of vocal cords. Laryngospasm due to hypocalcemia is a rare cause of stridor in adults, although occasionally reported in the neonates. We report an elderly lady having stridor and laryngospasm, secondary to acquired hypoparathyroidism and secondary hypocalcemia, without risk factors for hypoparathyroidism such as recent neck surgery or irradiation. We did an extensive review of literature to find only a few cases of acquired primary hypoparathyroidism in adults with the only complaint being stridor. This case underlines the fact that a common symptom like stridor rarely occurs due to uncommon causes. This case is being reported for its rarity and amenability to complete cure in event of correct diagnosis.

  14. A Rare Case of Petrified Ear

    Directory of Open Access Journals (Sweden)

    Kathryn E. Buikema

    2012-01-01

    Full Text Available Calcification or ossification of the auricle, also referred to as petrified ear, is a rare diagnosis in dermatology. In medical literature, it has most often been attributed to trauma, hypothermia and frostbite, or hypercalcemia secondary to a metabolic or endocrine disorder, such as Addison's disease. Here, we report the clinical and radiologic findings of a 79-year-old African American male whose unilateral petrified auricle was an incidental finding. He had a preceding history of hyperparathyroidism and subsequent hypercalcemia treated with a subtotal parathyroidectomy three years prior to presentation. In addition to laboratory analysis, a history and physical examination was performed which revealed no other signs of hypercalcemia. Radiologic studies demonstrated partial ossification of the external auricular cartilage on the left side. The patient was diagnosed with the rare occurrence of a petrified ear. In light of this case, we provide a discussion concerning the possible etiologies of this diagnosis including appropriate patient evaluation and possible treatment recommendations.

  15. Rare Isotopes Physics in the Multimessenger Era

    Science.gov (United States)

    Schatz, Hendrik

    2018-06-01

    While these isotopes only exist for fractions of seconds, their properties shape the resulting cosmic distribution of elements and the astronomical observables including spectra, neutrinos, and gravitational waves. The long standing challenge in nuclear astrophysics of the production of the relevant isotopes in the laboratory is now overcome with a new generation of rare isotope accelerator facilities now coming online. One example is the FRIB facility under construction at Michigan State University for the US Department of Energy, Office of Science, Office of Nuclear Physics. These new capabilities in nuclear physics coincide with advances in astronomy directly related to the cosmic sites where these isotopes are created, in particular in time domain and gravitational wave astronomy. I will discuss the importance of rare isotope physics in interpreting multi-messenger observations and how advances in nuclear physics and astronomy when combined promise to lead us towards a comprehensive theory of the origin of the elements.

  16. Hypokalemic periodic paralysis: Three rare secondary causes

    Directory of Open Access Journals (Sweden)

    Prasanna Eswaradass Venkatesan

    2015-01-01

    Full Text Available Periodic paralysis is a rare neuromuscular disorder, related to a defect in muscle ion channels, characterized by episodes of painless muscle weakness, which may be precipitated by heavy exercise, fasting, or high-carbohydrate meals. Hypokalemic periodic paralysis may be familial (primary or secondary. Here, we report three cases of secondary causes of hypokalemic periodic paralysis. On evaluation, case 1 had distal renal tubular acidosis (RTA due to Sjogren′s syndrome, case 2 had drug induced proximal RTA (Fanconi′s syndrome and case 3 had thyrotoxicosis. Clinician must be aware of causes of secondary PP as recognition and diagnosis can completely prevent further attacks of periodic paralysis. Each of the above case is rare, but completely treatable if diagnosed. Low dose steroids with bicarbonate replacement in case 1, stopping tenofovir in case 2 and carbimazole therapy in case 3 prevented further attacks of periodic paralysis and cardiopulmonary complications.

  17. The rare isotope accelerator (RIA) facility project

    International Nuclear Information System (INIS)

    Christoph Leemann

    2000-01-01

    The envisioned Rare-Isotope Accelerator (RIA) facility would add substantially to research opportunities for nuclear physics and astrophysics by combining increased intensities with a greatly expanded variety of high-quality rare-isotope beams. A flexible superconducting driver linac would provide 100 kW, 400 MeV/nucleon beams of any stable isotope from hydrogen to uranium onto production targets. Combinations of projectile fragmentation, target fragmentation, fission, and spallation would produce the needed broad assortment of short-lived secondary beams. This paper describes the project's background, purpose, and status, the envisioned facility, and the key subsystem, the driver linac. RIA's scientific purposes are to advance current theoretical models, reveal new manifestations of nuclear behavior, and probe the limits of nuclear existence [3]. Figures 1 and 2 show, respectively, examples of RIA research opportunities and the yields projected for pursuing them. Figure 3 outlines a conceptual approach for delivering the needed beams

  18. Imaging of a rare disorder: macrodystrophia lipomatosa

    Directory of Open Access Journals (Sweden)

    Farhana Ebrahim Suleman

    2010-06-01

    Full Text Available Macrodystrophia lipomatosa (MDL is described as a rare, non-hereditary, congenital condition presenting with localized macrodactyly and a proliferation of mesenchymal elements. There is in particular a marked increase in fibroadipose tissue(1. We describe two cases presenting to our department in a six month period, with a history of disproportionately large limbs since birth. While our first case demonstrated all the typical features of MDL, our second case failed to demonstrate osseous gigantism although the other features of MDL were present. An extensive search of the literature failed to yield any cases described without osseus gigantism but at the same time the other radiological features failed to fit in with any other related syndromes and the most appropriate diagnosis appears to be MDL. Thus we concluded that this may be a case of a rare, atypical MDL that was arrested or frustrated and therefore failed to demonstrate full expression of the syndrome.

  19. Factitious disorder: a rare cause of haematemesis.

    Science.gov (United States)

    McFarlane, Michael; Eaden, Jayne; Burch, Nicola; Disney, Ben

    2017-10-01

    Acute upper gastrointestinal (GI) bleeding is a common condition in the UK with 50-70,000 admissions per year. In 20% of cases no cause can be found on endoscopy. Here, we present the case of a young female patient who was admitted on three occasions with large volume haematemesis and bleeding from other sites. She was extensively investigated and underwent multiple endoscopic procedures. She was eventually diagnosed with factitious disorder after concerns were raised about the inconsistent nature of her presentations. She was found to be venesecting herself from her intravenous cannula, and ingesting the blood to simulate upper GI bleeding. This is a rare cause of 'haematemesis' but perhaps not as rare as is thought.

  20. Quantum Theory of Rare-Earth Magnets

    Science.gov (United States)

    Miyake, Takashi; Akai, Hisazumi

    2018-04-01

    Strong permanent magnets mainly consist of rare earths (R) and transition metals (T). The main phase of the neodymium magnet, which is the strongest magnet, is Nd2Fe14B. Sm2Fe17N3 is another magnet compound having excellent magnetic properties comparable to those of Nd2Fe14B. Their large saturation magnetization, strong magnetocrystalline anisotropy, and high Curie temperature originate from the interaction between the T-3d electrons and R-4f electrons. This article discusses the magnetism of rare-earth magnet compounds. The basic theory and first-principles calculation approaches for quantitative description of the magnetic properties are presented, together with applications to typical compounds such as Nd2Fe14B, Sm2Fe17N3, and the recently synthesized NdFe12N.

  1. Rare diseases in the media - Report April-June 2014 - Observatory for Rare Diseases FEDER (OBSER

    Directory of Open Access Journals (Sweden)

    Josep Solves Almela

    2015-12-01

    Full Text Available This report presents the analysis of how Spanish mass media dealt with the so-called rare deseases during the months of April, May and June of 2014. The report has the same general objective of the first one for the previous three months: understand how rare diseases are presented in the Spanish media and, correspondingly, how that media representation evolves. In this report, the data of the first trimester is compared to the second one.

  2. Severe accident management guidelines

    International Nuclear Information System (INIS)

    Uhle, Jennifer

    2014-01-01

    The events at Fukushima Daiichi have highlighted the importance of Severe Accident Management Guidelines (SAMGs). As the world has learned from the catastrophe and countries are considering changes to their nuclear regulatory programs, the content of SAMGs and their regulatory control are being evaluated. This presentation highlights several factors that are being addressed in the United States as rulemaking is underway pertaining to SAMGs. The question of how to be prepared for the unexpected is discussed with specific insights gleaned from Fukushima. (author)

  3. Probing new physics in rare charm processes

    International Nuclear Information System (INIS)

    Hewett, J.L.

    1994-09-01

    The possibility of using the charm system to search for new physics is addressed. Phenomena such as D 0 - bar D 0 mixing and rare decays of charmed mesons are first examined in the Standard Model to test the present understanding and to serve as benchmarks for signals from new sources. The effects of new physics from various classes of non-standard dynamical models on D 0 - bar D 0 mixing are investigated

  4. Revisiting Cementoblastoma with a Rare Case Presentation

    Directory of Open Access Journals (Sweden)

    Vijayanirmala Subramani

    2017-01-01

    Full Text Available Cementoblastoma is a rare benign odontogenic neoplasm which is characterized by the proliferation of cellular cementum. Diagnosis of cementoblastoma is challenging because of its protracted clinical, radiographic features, and bland histological appearance; most often cementoblastoma is often confused with other cementum and bone originated lesions. The aim of this article is to overview/revisit, approach the diagnosis of cementoblastoma, and also present a unique radiographic appearance of a cementoblastoma lesion associated with an impacted tooth.

  5. Hybrid Odontogenic Lesion: A Rare Entity

    Directory of Open Access Journals (Sweden)

    Reza Imani

    2017-03-01

    Full Text Available Hybrid tumors are very rare tumors composed of two different tumor entities, each of which conforms to an exactly defined tumor category. A 14-year-old boy was referred for an intraosseous painless lesion with a histopathological feature of multiple odontogenic lesions including calcifying odontogenic cyst, complex odontoma and ameloblastic fibro-odontoma. The final diagnosis considered to be a hybrid odontogenic lesion.

  6. Diagnostic study about lanthanides (rare earths)

    International Nuclear Information System (INIS)

    Ribeiro, G.F.

    1985-01-01

    The world situation of rare earths (lanthanides) is evaluated, and a comparison of the Brazilian situation in respect to other countries is established, concerning the following aspects: geology of mineral deposits; main sources, uses, reserves and production; their consumption, prices and state-of-art of geological researches and industrial processes for physical and chemical separation / concentration of these elements. (C.L.B.) [pt

  7. Eudialyte as potential industrial rare metal sources

    International Nuclear Information System (INIS)

    Chekmarev, A.M.; Chizhevskaya, S.V.; Masloboev, V.A.; Efremova, E.A.; Sharonov, A.V.

    1989-01-01

    The structural features and chemial compostion of eudialyte, zirconium silicate mineral, containing zirconium, niobium, tantalum, rare earth and other elements are considered. A brief review of studies of sulfuric acid decomposition of endialyte, including autoclave one, aimed at extraction of zirconium and other valuable components is given. The leaching methods providing a high direct zirconium yield into the solution, preparation of well-filtered pulps and permitting to realize further reprocessing of solutions by the extraction methods are discussed. 20 refs.; 4 figs

  8. A Rare Case of Oestrogen Dermatitis.

    Science.gov (United States)

    Bourgeault, Emilie; Bujold, Janie; Doucet, Marie-Eve

    Oestrogen dermatitis is a rare disorder characterised by cyclical eruptions in association with a woman's menstrual cycle. A 43-year-old woman with an 8-year history of cyclical inguinal dermatitis, with a negative patch test, was tested with intradermal progesterone and oestrogen. Intradermal testing was positive for oestrogen only. In a female patient with cyclical dermatitis, it is important to consider oestrogen or progesterone dermatitis in the differential diagnosis.

  9. Mineralogy of the rare earth elements

    International Nuclear Information System (INIS)

    Clark, A.M.

    1984-01-01

    This paper contains mineralogic properties of the rare earth elements (REE). Notes are given on total REE abundances, distribution patterns, and modes of occurrence. References are confined as far as possible to papers containing usable REE data. The minerals are grouped alphabetically within each major cationic group. The paper includes an alphabetic table of mineral names, chemical formulas, crystal system and section number. It functions as a handy entrance to the mineralogic and bibliographic paper. (G.J.P.)

  10. Structure of small rare earth clusters

    International Nuclear Information System (INIS)

    Rayane, D.; Benamar, A.; Tribollet, B.; Broyer, M.; Melinon, P.

    1991-01-01

    Rare earth clusters are produced by the inert gas condensation technique. The observed size distribution shows large peaks at n=13, 19, 23, 26, 29, 32, 34, 37, 39, 45, .... The beginning of this sequence (up to 34) has been already observed in argon clusters and recently by our group in barium clusters; this sequence may be interpreted in terms of icosahedral structures corresponding to the addition of caps on a core icosahedron of 13 atoms. (orig.)

  11. Adult primary hypoparathyroidism: A rare presentation

    OpenAIRE

    Chakrabarty, Ashima Datey

    2013-01-01

    The common causes of stridor in adults are abscesses or swelling of upper airway, tumors, paralysis or malfunction of vocal cords. Laryngospasm due to hypocalcemia is a rare cause of stridor in adults, although occasionally reported in the neonates. We report an elderly lady having stridor and laryngospasm, secondary to acquired hypoparathyroidism and secondary hypocalcemia, without risk factors for hypoparathyroidism such as recent neck surgery or irradiation. We did an extensive review of l...

  12. Economic Modeling Considerations for Rare Diseases.

    Science.gov (United States)

    Pearson, Isobel; Rothwell, Ben; Olaye, Andrew; Knight, Christopher

    2018-05-01

    To identify challenges that affect the feasibility and rigor of economic models in rare diseases and strategies that manufacturers have employed in health technology assessment submissions to demonstrate the value of new orphan products that have limited study data. Targeted reviews of PubMed, the National Institute for Health and Care Excellence's (NICE's) Highly Specialised Technologies (HST), and the Scottish Medicines Consortium's (SMC's) ultra-orphan submissions were performed. A total of 19 PubMed studies, 3 published NICE HSTs, and 11 ultra-orphan SMC submissions were eligible for inclusion. In rare diseases, a number of different factors may affect the model's ability to comply with good practice recommendations. Many products for the treatment of rare diseases have an incomplete efficacy and safety profile at product launch. In addition, there is often limited available natural history and epidemiology data. Information on the direct and indirect cost burden of an orphan disease also may be limited, making it difficult to estimate the potential economic benefit of treatment. These challenges can prevent accurate estimation of a new product's benefits in relation to costs. Approaches that can address such challenges include using patient and/or clinician feedback to inform model assumptions; data from disease analogues; epidemiological techniques, such as matching-adjusted indirect comparison; and long-term data collection. Modeling in rare diseases is often challenging; however, a number of approaches are available to support the development of model structures and the collation of input parameters and to manage uncertainty. Copyright © 2018 International Society for Pharmacoeconomics and Outcomes Research (ISPOR). Published by Elsevier Inc. All rights reserved.

  13. Primary periosteal lymphoma - rare and unusual

    Energy Technology Data Exchange (ETDEWEB)

    Abdelwahab, Ibrahim F. [Coney Island Hospital, Department of Radiology, New York, NY (United States); Hoch, Benjamin [Mount Sinai School of Medicine, CUNY, Department of Pathology, New York, NY (United States); Hermann, George [Mount Sinai School of Medicine, CUNY, Department of Radiology, New York, NY (United States); Bianchi, Stefano [Clinique et Fondation des Grangettes, Geneva (Switzerland); Klein, Michael J. [UAB School of Medicine, Department of Pathology, Birmingham, AL (United States); Springfield, Dempsey S. [Mount Sinai School of Medicine, CUNY, Department of Orthopedics, New York, NY (United States)

    2007-04-15

    We describe a primary periosteal lymphoma that involved only the periosteum without affecting the adjacent medulla or the regional lymph nodes. No other lymphomatous foci were found in either the distant lymph nodes or viscera. This unusual presentation simulates the imaging appearance of surface lesions of bone, namely benign and malignant tumors, and departs from the typical appearance of primary lymphoma of bone. Therefore, this rare type of lymphoma should be considered in the differential diagnosis of surface bone lesions. (orig.)

  14. Cervical myositis ossificans traumatica: a rare location

    Energy Technology Data Exchange (ETDEWEB)

    Baysal, T.; Sarac, K.; Kutlu, R. [Dept. of Radiology, Inonu University, Malatya (Turkey); Baysal, O.; Ersoy, Y. [Dept. of Physical Therapy and Rehabilitation, Inonu Univ., Malatya (Turkey); Elmali, N. [Dept. of Orthopedics and Traumatology, Inonu Univ., Malatya (Turkey)

    1999-05-01

    An unusual case of myositis ossificans traumatica lesion located in the paraspinal region is reported. Despite the contiguity of the lesion with the cervical vertebrae and ominous appearance of the biopsy material, the history of antecedent trauma and computed tomography findings allowed preoperative accurate diagnosis. To our knowledge, myositis ossificans traumatica located in the cervical paraspinal region is very rare. (orig.) With 4 figs., 16 refs.

  15. Galactosemia: a rare case in pediatric practice

    Directory of Open Access Journals (Sweden)

    S. Ya. Volgina

    2015-01-01

    Full Text Available Galactosemia is a rare life-threatening inherited autosomal recessive disease, the differential diagnosis of which has been very difficult up to date, especially if there are no results of neonatal screening for some reasons. The paper describes a clinical case of a 10-day patient with type I classic galactosemia and reflects the importance of a timely diagnostic search and switching him to lactose-free formulas.

  16. Bilateral cerebellopontine arachnoid cyst: A rare entity.

    Science.gov (United States)

    Sharma, Anand; Sharma, Achal; Mittal, Radhey S; Gandhi, Ashok

    2015-01-01

    Bilateral cerebellopontine angle (CPA) arachnoid cysts (ACs) are very rare: only one case is reported in literature. Pathogenesis of those cysts is unknown; they are thought to be congenital. The presenting symptoms of CPA AC are frequently nonspecific or otological. The management of ACs of the CPA is controversial. We are reporting two cases of bilateral CPA AC with their pathophysiology and review of literature.

  17. Angiographic appearances of rare renal tumours

    International Nuclear Information System (INIS)

    Schmidt, M.; Taenzer, V.

    1980-01-01

    Oncocytomas, called oxyphil proximal tubular adenomas in the Anglo Saxon literature, and benign hypernephromas are non-malignant, usually symptomless, rare tumours belonging to the renal adenomas. Oncocytomas have angiographic appearances sufficiently uniform to permit a tentative diagnosis. Histologically benign hypernephromas do not possess characteristic angiographic appearances and, in the presence of tumour in the renal vein or necrotic avascular areas, must be regarded as potentially malignant. (orig.) [de

  18. Atomic masses of rare-earth isotopes

    International Nuclear Information System (INIS)

    Schmidt-Ott, W.D.; Kantus, R.; Runte, E.

    1981-01-01

    A survey is given of decay energies of rare-earth isotopes measured in electron-capture decay by relative Psub(K) ratios, ECsub(K)/β + , and EC/β + ratios. Atomic masses of A = 147 isotopes and of 146 Gd and 148 Dy were derived. The masses of these isotopes and of α-decaying precessors are compared with predictions of current mass formulae. The subshell closure at Z = 64 is shown for N = 82, and 84 isotones. (orig.)

  19. Epitaxial rare-earth superlattices and films

    International Nuclear Information System (INIS)

    Salamon, M.B.; Beach, R.S.; Flynn, C.P.; Matheny, A.; Tsui, F.; Rhyne, J.J.

    1992-01-01

    This paper reports on epitaxial growth of rare-earth superlattices which is demonstrated to have opened important new areas of research on magnetic materials. The propagation magnetic order through non-magnetic elements, including its range and anisotropy, has been studied. The importance of magnetostriction in determining the phase diagram is demonstrated by the changes induced by epitaxial clamping. The cyrstallinity of epitaxial superlattices provides the opportunity to study interfacial magnetism by conventional x-ray and neutron scattering methods

  20. Pachydermoperiostosis: a rare mimicker of acromegaly

    OpenAIRE

    Abdullah, Noor Rafhati Adyani; Jason, Wong Lok Chin; Nasruddin, Azraai Bahari

    2017-01-01

    Pachydermoperiostosis is a very rare osteoarthrodermopathic disorder whose clinical and radiographic presentations may mimic those of acromegaly. In the evaluation of patients with acromegaloid appearances, pachydermoperiostosis should be considered as a differential diagnosis. In this article, we report a 17-year-old boy who presented with 2-year history of acral enlargement and facial appearance changes associated with joint pain and excessive sweating. He had been investigated extensively ...

  1. [A rarely known headache: Airplane travel headache].

    Science.gov (United States)

    Azman, Filiz; Erkılınç, Büşra; Çabalar, Murat; Çağırıcı, Sultan; Yayla, Vildan

    2017-01-01

    Recently, headache associated with airplane travel has gained importance with case reports and took its place in the classification of headache in 2013. This rare condition has different spesific characteristic from the primary headaches and its pathophysiology is not clear yet. In this case report, a 27-years-old female patient was diagnosed with the headache associated with airplane travel by history, examination and imaging findings. The possible pathophysiology and treatment were discussed.

  2. Pachydermoperiostosis: a rare mimicker of acromegaly

    Directory of Open Access Journals (Sweden)

    Noor Rafhati Adyani Abdullah

    2017-05-01

    Full Text Available Pachydermoperiostosis is a very rare osteoarthrodermopathic disorder whose clinical and radiographic presentations may mimic those of acromegaly. In the evaluation of patients with acromegaloid appearances, pachydermoperiostosis should be considered as a differential diagnosis. In this article, we report a 17-year-old boy who presented with 2-year history of acral enlargement and facial appearance changes associated with joint pain and excessive sweating. He had been investigated extensively for acromegaly, and the final diagnosis was pachydermoperiostosis.

  3. Cartap poisoning: A rare case report.

    Science.gov (United States)

    Kumar, A S Praveen; Amalnath, Deepak; Dutta, T K

    2011-10-01

    Cartap is a pesticide commonly used to control weevil and caterpillars. It is an analogue of nereistoxin, a neurotoxic substance isolated from the marine annelid Lumbriconereis heteropoda. It causes neuromuscular blockade. Poisoning with cartap is very rare and not yet reported from India. We report a 35-year-old lady with cartap poisoning who presented with nausea, vomiting, and dyspnea. She improved with N-acetyl cysteine and symptomatic management.

  4. Cartap poisoning: A rare case report

    OpenAIRE

    Kumar, A. S. Praveen; Amalnath, Deepak; Dutta, T. K.

    2011-01-01

    Cartap is a pesticide commonly used to control weevil and caterpillars. It is an analogue of nereistoxin, a neurotoxic substance isolated from the marine annelid Lumbriconereis heteropoda. It causes neuromuscular blockade. Poisoning with cartap is very rare and not yet reported from India. We report a 35-year-old lady with cartap poisoning who presented with nausea, vomiting, and dyspnea. She improved with N-acetyl cysteine and symptomatic management.

  5. Rare tumors of the rectum. Narrative review.

    Science.gov (United States)

    Errasti Alustiza, José; Espín Basany, Eloy; Reina Duarte, Angel

    2014-11-01

    Most rectal neoplasms are adenocarcinomas, but there is a small percentage of tumors which are of other histological cell lines such as neuroendocrine tumors, sarcomas, lymphomas and squamous cell carcinomas, which have special characteristics and different treatments. We have reviewed these rare tumors of the rectum from a clinical and surgical point of view. Copyright © 2013 AEC. Published by Elsevier Espana. All rights reserved.

  6. Allergy in severe asthma.

    Science.gov (United States)

    Del Giacco, S R; Bakirtas, A; Bel, E; Custovic, A; Diamant, Z; Hamelmann, E; Heffler, E; Kalayci, Ö; Saglani, S; Sergejeva, S; Seys, S; Simpson, A; Bjermer, L

    2017-02-01

    It is well recognized that atopic sensitization is an important risk factor for asthma, both in adults and in children. However, the role of allergy in severe asthma is still under debate. The term 'Severe Asthma' encompasses a highly heterogeneous group of patients who require treatment on steps 4-5 of GINA guidelines to prevent their asthma from becoming 'uncontrolled', or whose disease remains 'uncontrolled' despite this therapy. Epidemiological studies on emergency room visits and hospital admissions for asthma suggest the important role of allergy in asthma exacerbations. In addition, allergic asthma in childhood is often associated with severe asthma in adulthood. A strong association exists between asthma exacerbations and respiratory viral infections, and interaction between viruses and allergy further increases the risk of asthma exacerbations. Furthermore, fungal allergy has been shown to play an important role in severe asthma. Other contributing factors include smoking, pollution and work-related exposures. The 'Allergy and Asthma Severity' EAACI Task Force examined the current evidence and produced this position document on the role of allergy in severe asthma. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  7. PEG-asparaginase induced severe hypertriglyceridemia.

    Science.gov (United States)

    Galindo, Rodolfo J; Yoon, Justin; Devoe, Craig; Myers, Alyson K

    2016-04-01

    Asparaginase (ASP) is an effective chemotherapy agent extensively used in children with acute lymphocytic leukemia (ALL). There has been a recent interest in using ASP in adults with ALL, particularly the less toxic pegylated (PEG) formulation. Hypertriglyceridemia (HTG) is a rare complication of PEG-ASP therapy. We report two cases of obese patients who developed severe HTG after receiving PEG for ALL. Both patients were incidentally found to have severe HTG (TG of 4,330 and 4,420 mg/dL). In both patients, there was no personal or family history of dyslipidemia or hypothyroidism. There was no evidence of pancreatitis or skin manifestations of HTG. Both patients were treated with PEG cessation, low-fat diet and pharmacotherapy. Both patients were re-challenged with PEG, with subsequent increase in TG but no associated complications. TG returned to baseline after discontinuing PEG and while on therapy for HTG. A literature review of PEG-induced HTG in adults demonstrated similar results: asymptomatic presentation despite very severe HTG. HTG is a rare but clinically important adverse effect of PEG. Underlying obesity and/or diabetes may represent risk factors. Clinicians should monitor TG levels during PEG therapy to avoid TG-induced pancreatitis.

  8. Bisphenol A induces spermatocyte apoptosis in rare minnow Gobiocypris rarus

    International Nuclear Information System (INIS)

    Zhang, Yingying; Cheng, Mengqian; Wu, Lang; Zhang, Guo; Wang, Zaizhao

    2016-01-01

    Highlights: • Adult male G. rarus were exposed to 225 μg/L BPA for 7, 21 and 63 days. • BPA could induce spermatocyte apoptosis in rare minnow testis. • The mitochondrial apoptotic pathway participated in the germ cell apoptosis. • The spermatocyte apoptosis was likely initiated by BPA induced meiosis arrest. - Abstract: Bisphenol A (BPA) is an endocrine disruptor, and could induce germ cells apoptosis in the testis of mammals. But whether it could affect fish in the same mechanism has not’ been studied till now. In the present study, to investigate the influence of BPA on testis germ cells in fish, adult male rare minnow Gobiocypris rarus were exposed to 225 μg L"−"1 (0.99 μM) BPA for 1, 3 and 9 weeks. Through TdT-mediated dUTP nick end labeling (TUNEL) and transmission electron microscope (TEM) analysis, we found that the amount of apoptotic spermatocytes significantly increased in a time dependent manner following BPA exposure. Western Blot results showed that the ratio of Bcl2/Bax, the important apoptosis regulators in intrinsic mitochondrial apoptotic pathway, was significantly decreased. qPCR showed that mRNA expression of several genes in mitochondrial apoptotic pathway including bcl2, bax, casp9, cytc and mcl1b were significantly changed following BPA exposure. In addition, mRNA expression of meiosis regulation genes (kpna7 and wee2), and genes involved in both apoptosis and meiosis (birc5, ccna1, and gsa1a) were also affected by BPA. Taken together, the present study demonstrated that BPA could induce spermatocytes apoptosis in rare minnow testis, and the apoptosis was probably under regulation of intrinsic mitochondrial apoptotic pathway. Moreover, the spermatocyte apoptosis was likely initiated by BPA induced meiosis arrest.

  9. Bisphenol A induces spermatocyte apoptosis in rare minnow Gobiocypris rarus

    Energy Technology Data Exchange (ETDEWEB)

    Zhang, Yingying; Cheng, Mengqian; Wu, Lang; Zhang, Guo; Wang, Zaizhao, E-mail: zzwang@nwsuaf.edu.cn

    2016-10-15

    Highlights: • Adult male G. rarus were exposed to 225 μg/L BPA for 7, 21 and 63 days. • BPA could induce spermatocyte apoptosis in rare minnow testis. • The mitochondrial apoptotic pathway participated in the germ cell apoptosis. • The spermatocyte apoptosis was likely initiated by BPA induced meiosis arrest. - Abstract: Bisphenol A (BPA) is an endocrine disruptor, and could induce germ cells apoptosis in the testis of mammals. But whether it could affect fish in the same mechanism has not’ been studied till now. In the present study, to investigate the influence of BPA on testis germ cells in fish, adult male rare minnow Gobiocypris rarus were exposed to 225 μg L{sup −1} (0.99 μM) BPA for 1, 3 and 9 weeks. Through TdT-mediated dUTP nick end labeling (TUNEL) and transmission electron microscope (TEM) analysis, we found that the amount of apoptotic spermatocytes significantly increased in a time dependent manner following BPA exposure. Western Blot results showed that the ratio of Bcl2/Bax, the important apoptosis regulators in intrinsic mitochondrial apoptotic pathway, was significantly decreased. qPCR showed that mRNA expression of several genes in mitochondrial apoptotic pathway including bcl2, bax, casp9, cytc and mcl1b were significantly changed following BPA exposure. In addition, mRNA expression of meiosis regulation genes (kpna7 and wee2), and genes involved in both apoptosis and meiosis (birc5, ccna1, and gsa1a) were also affected by BPA. Taken together, the present study demonstrated that BPA could induce spermatocytes apoptosis in rare minnow testis, and the apoptosis was probably under regulation of intrinsic mitochondrial apoptotic pathway. Moreover, the spermatocyte apoptosis was likely initiated by BPA induced meiosis arrest.

  10. Severe sepsis secondary to emphysematous cystitis

    Directory of Open Access Journals (Sweden)

    M.M. Gargouri

    2015-03-01

    Full Text Available Emphysematous cystitis (EC is a rare, rapidly progressive infection characterized by gas formation within the bladder wall. Gas production is mainly due to fermentation of glucose or albumin by the pathogenic organisms. We present the case of a patient with EC who presented with severe sepsis of unknown origin. He was also found to have two vesical calculi and a horseshoe kidney. EC was diagnosed on ultrasonography which showed circumferential, acoustic shadowing around the bladder wall. Computed tomography (CT confirmed the diagnosis. Rapid healing could be achieved with appropriate antibiotics and bladder drainage. Endoscopic disintegration of the stones was performed three weeks later, and follow-up was uneventful.

  11. Intrathoracic lipoblastoma presenting with severe respiratory distress

    OpenAIRE

    Joseph Motshedi Sekgololo; Risenga Frank Chauke; Peter Shere Ramoroko; Khondker Mofazzal Hossain

    2017-01-01

    Lipoblastoma is a rare benign tumour which originates from an adipose tissue. In this study we report the case of a three year old boy who presented with a large intrathoracic tumour occupying the whole of the left hemithorax. He presented in severe respiratory distress. A chest X-ray showed total opacity of the left hemithorax, and CT-scan showed a low attenuation mass inkeeping with fat in the left hemithorax. A complete resection of a tumour was undertaken, with histopathology report confi...

  12. Anthropogenic Cycles of Rare Earth Elements

    Science.gov (United States)

    Du, X.; Graedel, T. E.

    2009-12-01

    This research will develop quantitatively resolved anthropogenic cycles and in-use stocks for the rare earth metals specifically cerium, lanthanum and dysprosium in Japan, China, and the U.S. for the year of 2007. Rare earth elements (REE) is a group of 17 scare metals widely used in a growing number of emerging technologies and have been in high demand for emerging technologies as raw materials during past the three decades. New market participants from newly industrializing countries, primarily China, have had strong impacts on the demand of share. Consequently, the importance to sustain a reliable, steady, uninterrupted supply on global market triggered comprehensive research to recognize and understand the life cycles of rare earths. Moreover, because China plays a dominant role in mining production since 1990, it requires the assessment for the countries, which are almost completely dependent on imports from China with respect to rare earth resources. The study aims to analyze the flows and stocks of rare earth elements individually as elemental form in spite of their natural geological co-occurrence and mixed composition in applications. By applying the method of Material Flow Analysis (MFA) work has been done on evaluating current and historical flows of specific technologically significant materials, for example, copper, zinc, nickel, etc., determining the stocks available in different types of reservoirs (e.g., lithosphere, in-use) and the flows among the reservoirs, developing scenarios of possible futures of metal use, and assessing the environmental and policy implications of the results. Therefore, REE as a new target deserves inclusion because of its potential demand-supply conflict and importance to secure the competitive advantage of technical innovation in future. This work will generate a quantitatively resolved anthropogenic life cycle and in-use stocks for REE for the main target countries for a chosen year, 2007, providing flows and stocks from

  13. Community Assembly Processes of the Microbial Rare Biosphere.

    Science.gov (United States)

    Jia, Xiu; Dini-Andreote, Francisco; Falcão Salles, Joana

    2018-03-14

    Our planet teems with microorganisms that often present a skewed abundance distribution in a local community, with relatively few dominant species coexisting alongside a high number of rare species. Recent studies have demonstrated that these rare taxa serve as limitless reservoirs of genetic diversity, and perform disproportionate types of functions despite their low abundances. However, relatively little is known about the mechanisms controlling rarity and the processes promoting the development of the rare biosphere. Here, we propose the use of multivariate cut-offs to estimate rare species and phylogenetic null models applied to predefined rare taxa to disentangle the relative influences of ecoevolutionary processes mediating the assembly of the rare biosphere. Importantly, the identification of the factors controlling rare species assemblages is critical for understanding the types of rarity, how the rare biosphere is established, and how rare microorganisms fluctuate over spatiotemporal scales, thus enabling prospective predictions of ecosystem responses. Copyright © 2018 Elsevier Ltd. All rights reserved.

  14. Severe childhood malnutrition.

    Science.gov (United States)

    Bhutta, Zulfiqar A; Berkley, James A; Bandsma, Robert H J; Kerac, Marko; Trehan, Indi; Briend, André

    2017-09-21

    The main forms of childhood malnutrition occur predominantly in children malnutrition. Here, we use the term 'severe malnutrition' to describe these conditions to better reflect the contributions of chronic poverty, poor living conditions with pervasive deficits in sanitation and hygiene, a high prevalence of infectious diseases and environmental insults, food insecurity, poor maternal and fetal nutritional status and suboptimal nutritional intake in infancy and early childhood. Children with severe malnutrition have an increased risk of serious illness and death, primarily from acute infectious diseases. International growth standards are used for the diagnosis of severe malnutrition and provide therapeutic end points. The early detection of severe wasting and kwashiorkor and outpatient therapy for these conditions using ready-to-use therapeutic foods form the cornerstone of modern therapy, and only a small percentage of children require inpatient care. However, the normalization of physiological and metabolic functions in children with malnutrition is challenging, and children remain at high risk of relapse and death. Further research is urgently needed to improve our understanding of the pathophysiology of severe malnutrition, especially the mechanisms causing kwashiorkor, and to develop new interventions for prevention and treatment.

  15. The industry of metallic rare earths (R.E.)

    International Nuclear Information System (INIS)

    Poirier, P.

    1979-01-01

    The following subjects are discussed: rare earths resources (rare earths abondance and world reserves, main ores). Rare earths separation and purification (ionic exchange, solvent extraction). Metallic rare earths and their mixtures, metallothermic reduction of oxides or fluorides (Ca, Mg, Al, Si or rare earth metals), Co-reduction process for intermetallic compounds (SmCo 5 ). Industrial applications of metallic rare earths (traditional applications such as flints, nodular cast iron, steel refining, magnesium industrie, applications under development such as rare earths/cobalt magnets, LaNi 5 for hydrogen storage, special alloys (automotive post combustion), magnetostrictive alloys). Economical problems: rare earth are elements relatively abundant and often at easily accessible prices. However, this group of 15 elements are liable to certain economical restraints. It is difficult to crack ore for only one rare earth. Availability of one given rare earth must be associated with the other corresponding rare earths to absorb all the other rare earths in other applications. Rare-earth industry has a strong expanding rate. 20% per year average for 6 years with Rhone-Poulenc. Thanks to their exceptional, specific characteristics rare earths have a bright future particularly for their metals

  16. [Large vessel vasculitis with myelodysplastic syndrome: A rare association].

    Science.gov (United States)

    Galland, J; Kawski, H; Guichard, J-F; Maurier, F

    2017-07-01

    The vasculitis can be the consequence of malignancy: most often hematologic rather than solid tumors. The association between large vessels vasculitis and myelodysplastic syndrome is rare. A 55-year-old man experienced asthenia, fever, polyarthritis and inflammatory syndrome. Haematological investigations found a type 2 refractory anemia with excess blasts (RAEB-2) with discovery of severe anemia (Hb: 7,8g/dl) and thrombopenia (platelets: 40,000/mm 3 ). Radiological examinations found thoracic aortitis and carotid vasculitis. Treatment in the form of steroids and azacitidine was instituted. The lack of control of both RAEB-2 and vasculitis was responsible for the death of the patient. Myelodysplastic syndrome and large vessels vasculitis is a rare but serious association disease. The lack of efficiency of corticosteroids seems to be common. Prognosis depends on the haematological treatment effectiveness. Copyright © 2016 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  17. Mounier-Kuhn syndrome: a rare cause of bronchial dilation.

    Science.gov (United States)

    Celik, Burcin; Bilgin, Salih; Yuksel, Canan

    2011-01-01

    Mounier-Kuhn syndrome, or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by marked tracheobronchial dilation and recurrent lower respiratory tract infections. Diagnosis is typically accomplished with the use of computed tomography and bronchoscopy, as well as pulmonary function testing. Patients may be asymptomatic; however, symptoms can range from minimal with preserved lung function to severe respiratory failure. Therapy, if any, is supportive but minimal. Surgery rarely has a place in the treatment of Mounier-Kuhn syndrome.Herein, we report the case of a 58-year-old man with chronic obstructive pulmonary disease who had a chronic cough, increased sputum production, and chest pain. Thoracic computed tomography showed tracheal dilation (diameter, 34 mm) and multiple diverticula in the posterior region of the trachea. Fiberoptic bronchoscopy revealed enlarged main bronchi, the dilated trachea, and prominent tracheal diverticula. Pulmonary function testing disclosed impaired respiratory function. Histopathologic examination of biopsy specimens from the bronchi and the tracheal wall supported the diagnosis of Mounier-Kuhn syndrome. The patient was released from the hospital and his condition was monitored for 2 years, during which time he developed no lower respiratory tract infections.Regardless of radiologic findings that suggest recurrent lower respiratory tract infection, we recommend that Mounier-Kuhn syndrome be considered in the differential diagnosis.

  18. Intracranial extramedullary hematopoiesis: a rare cause of headaches.

    Science.gov (United States)

    Singer, Adam; Quencer, Robert

    2014-01-01

    Ectopic bone marrow production, known as extramedullary hematopoiesis, may result in symptoms due to compression on normal structures. We present the multimodality imaging findings and subsequent management of a rare case of symptomatic extramedullary hematopoiesis within the calvarium. Case report. A 54-year-old male with a history of myelofibrosis and no previous diagnosis of a headache disorder presented to the emergency department with worsening severe bilateral headaches. A nonenhanced CT of the brain was performed and diffuse extra-axial nodular hyperdensities were visualized. MRI of the brain demonstrated diffuse extra-axial avidly enhancing nodular masses, dural thickening and marked susceptibility. No paravertebral masses, typical for extramedullary hematopoiesis, were present in the chest or abdomen. Although the clinical team considered a biopsy to confirm the diagnosis, we suggested a noninvasive confirmatory test. The subsequent Tc99m sulfur colloid scan corroborated the diagnosis. The patient was then referred to radiation oncology for treatment. In summary, extramedullary hematopoiesis is a hematologic compensatory disorder that rarely occurs within the CNS and may cause neurological compromise due to compression on underlying structures. The diagnosis can be made with noninvasive imaging and treated with low dose radiation therapy. Copyright © 2013 by the American Society of Neuroimaging.

  19. A novel method for fat suppression in RARE sequences

    International Nuclear Information System (INIS)

    Higuchi, Nobuya

    1999-01-01

    Rapid acquisition relaxation-enhanced (RARE) sequences utilize one or several Carr-Purcell-Meiboom-Gill (CPMG) echo trains to sample a number of k-space lines each repetition time TR. The technique can rapidly generate multislice T2-weighted images which, as a rule, are strikingly similar in contrast to conventional T2-weighted spin-echo (SE) images. An exception to this rule is the appearance of very bright signal from fat in T2-weighted RARE images as compared to conventional T2-weighted SE images. To reduce this fat signal, we introduce a time delay, τc, between the 90 degrees x and first 180 degrees y pulse of each echo train such that a phase angle of pi/2 develops between fat and the reference (water) line at echo maxima. The technique leads to single-acquisition fat suppression without the use of frequency-selective saturation pulses and concomitant loss of slices per TR. A Bloch equation analysis is used to identify two major mechanisms contributing to suppression of off-resonance spins such that w τc =pi/2. Namely, the CPMG sequence becomes a CP sequence with no self-correction properties for imperfect 180 degrees pulses leading to enhanced signal decay, and the raw k-space data matrix become segmented into blocks alternately multiplied by +/- i, leading to signal dispersion following Fourier transformation. (K.H.)

  20. Rare-earth metal transition metal borocarbide and nitridoborate superconductors

    Energy Technology Data Exchange (ETDEWEB)

    Niewa, Rainer; Shlyk, Larysa; Blaschkowski, Bjoern [Stuttgart Univ. (Germany). Inst. fuer Anorganische Chemie

    2011-07-01

    Few years after the discovery of superconductivity in high-T{sub c} cuprates, borocarbides and shortly after nitridoborates with reasonably high T{sub c}s up to about 23 K attracted considerable attention. Particularly for the rare-earth metal series with composition RNi{sub 2}[B{sub 2}C] it turned out, that several members exhibit superconductivity next to magnetic order with both T{sub c} above or below the magnetic ordering temperature. Therefore, these compounds have been regarded as ideal materials to study the interplay and coexistence of superconductivity and long range magnetic order, due to their comparably high ordering temperatures and similar magnetic and superconducting condensation energies. This review gathers information on the series RNi{sub 2}[B{sub 2}C] and isostructural compounds with different transition metals substituting Ni as well as related series like RM[BC], RM[BN], AM[BN] and R{sub 3}M{sub 2}[BN]{sub 2}N (all with R = rare-earth metal, A = alkaline-earth metal, M = transition metal) with special focus on synthesis, crystal structures and structural trends in correspondence to physical properties. (orig.)