1,690 research outputs found
Thermodynamic assessment of the KF-ThF<sub>4</sub>, LiF-KF-ThF<sub>4</sub> and NaF-KF-ThF<sub>4</sub> systems
No full textA thermodynamic assessment of the KF-ThF4 binary system using the CALPHAD method is presented, where the liquid solution is described by the modified quasichemical formalism in the quadruplet approximation. The optimization of the phase diagram is based on experimental data reported in the literature and newly measured X-ray diffraction and differential scanning calorimetry data, which have allowed to solve discrepancies between past assessments. The low temperature heat capacity of α-K2ThF6 has also been measured using thermal relaxation calorimetry; from these data the heat capacity and standard entropy values have been derived at 298.15 K: Cp,mo(K2ThF6,cr,298.15K)=(193.2±3.9) J·K-1·mol-1 and Smo(K2ThF6,cr,298.15K)=(256.9±4.8) J·K-1·mol-1. Taking existing assessments of the relevant binaries, the new optimization is extrapolated to the ternary systems LiF-KF-ThF4 and NaF-KF-ThF4 using an asymmetric Kohler/Toop formalism. The standard enthalpy of formation and standard entropy of KNaThF6 are re-calculated from published e.m.f data, and included in the assessment of the ternary system. A calculated projection of the NaF-KF-ThF4 system at 300 K and the optimized liquidus projections of both systems are compared to published phase equilibrium data at room temperature and along the LiF-LiThF5 and NaF-KThF5 pseudobinaries, with good agreement.RST/Reactor Physics and Nuclear Material
Evaluation of Kayser–Fleischer ring in Wilson disease by anterior segment optical coherence tomography
No full textPurpose: The purpose of the study is to present anterior segment optical coherence tomography (AS-OCT) as an alternative method of evaluating Kayser–Fleischer (KF) ring in Wilson disease (WD) not only by ophthalmologists but also by other clinicians dealing with WD. Materials and Methods: This was a retrospective case series of six WD patients with KF ring. Evaluation of KF ring was done by naked eye examination using torch light, slit lamp biomicroscopy (SL), and AS-OCT. SL examination was done using a narrow slit of the superior cornea. AS-OCT was done using the Optovue RTvue PremierTM device (Fremont, CA, USA). Results: AS-OCT revealed KF ring as an intense hyperreflective band at the level of Descemet membrane (DM). Color scale of AS-OCT showed KF ring as greenish/greenish yellow/orange yellow/yellowish/red band. Validation of AS-OCT findings was done by second ophthalmologist, medical gastroenterologist, surgical gastroenterologist, and neurophysician. After seeing the first observation, they could identify the AS-OCT features in all pictures with ease. Conclusions: This is the first observation of KF ring in WD on AS-OCT. On AS-OCT, KF ring is visualized as intense hyperreflectivity at the level of DM in the peripheral cornea. Further, studies are needed to evaluate the usefulness of AS-OCT in WD management
Kayser-Fleischer Ring Detection and Quantification in Wilson\u27s disease using Optical Coherence Tomography
No full textDelays in the diagnosis of Wilson\u27s Disease (WD) arising from the challenging heterogeneity of presentation, lead to avoidable morbidity and mortality. Clinically a Kayser-Fleischer (KF) ring is useful in diagnosis, reported as being present in nearly all neurological WD, half of hepatic WD and less than half of presymptomatic WD. Most KF rings regress with medical therapy or liver transplantion, however, may return when treatment ceases. Lifelong monitoring of serum and urine is cumbersome for the patient and a reliable non-invasive disease activity score is sought. The aim of the study was to establish the feasibility of quantitative analysis of KF rings in established WD using non-invasive anterior segment optical coherence tomography (AS-OCT)
Wilson\u27s disease: experience at a tertiary care hospital.
No full textWilson\u27s disease (WD) is a rare autosomal recessive disorder of copper metabolism. Data regarding WD is not available from Pakistan. A cross-sectional study was conducted at The Aga Khan University Hospital, Karachi, and all patients admitted with primary and secondary diagnosis of Wilson\u27s disease were added. A total of 47 patients were seen; 68% (n = 32) were male. The mean age was 26.6 ± 9.97 years. Most of the patients presented with hepatic, (n = 22, 46.8%), neurological, (n = 17, 36.2%) and psychiatric (n = 8, 17%) symptoms. Mean ceruloplasmin level was 0.17 ± 0.13 g/dl; it was \u3c 0.25 g/dl in 39 (86.6%) patients. Serum copper (Cu) was reduced in 32 (68.1%) patients and 24-hr-urinary Cu was raised in 22 (47.6%) patients. Slit lamp examination for Kayser-Fleischer (KF) rings was done on 15 (31.9%) patients and 9 (60%) of them had KF rings. Mean serum aspartate transaminase (AST) / alanine transaminases (ALT) ratio was 1.92 and median alkaline phosphatase / total bilirubin ratio was 79.30 (IQR 35.05; 166.50)
Húsz év után. A Könyvtári Figyelő 2000–2019 között
No full textThe study provides an overview of the life of this peer-reviewed national professional journal edited at the Hungarian Library Institute in the period 2000 through 2019. Könyvtári Figyelő entered its 65th volume this year. This quarterly magazine has published 80 issues and more than 15,000 pages in PDF format in twenty years. Thanks to the support of the National Cultural Fund (NKA) printed issues continue to be published to our days. The full text of issues can be accessed and searched on an electronic interface (http://ki.oszk.hu/kf) from the beginnings (1955) to the present day (2020/1). Roughly two-thirds of each issue include research articles on the main events and developments in Hungarian librarianship, and one-third deals with the presentation of developments in the international library world. The author reviews the most important articles in the Studies, Our past, and Book reviews columns in two ten-year cycles, and refers to the professional trends summarized at the beginning of her article. The editors are increasingly oriented towards open access and the application of the Open Journal System (OJS).A tanulmány áttekintés a Könyvtári Intézetben készülő, idén 65. évfolyamába lépett, országos hatókörű, lektorált szakmai folyóirat 2000 és 2019 közötti működéséről. A negyedéves megjelenésű folyóirat húsz év alatt 80 füzetszámban, több mint 15 ezer pdf oldal terjedelemben jelent meg. Az NKA támogatásának köszönhetően ez idáig nyomtatott formában is elkészülhetett. Elektronikus felületén (http://ki.oszk.hu/kf) a kezdetektől (1955 től) napjainkig (2020/1.) elérhető és kereshető a lapszámok teljes szövege. Minden szám nagyjából kétharmadában részben a magyarországi könyvtárügy legfőbb eseményeiről és fejlesztéseiről közöl elméleti publikációkat, egyharmadában pedig a külföldi könyvtári világ fejleményeinek bemutatásával foglalkozik. Kétszer tíz éves ciklusra bontva tekinti át a szerző a Tanulmányok, a Múltunk és a Könyvszemle rovatok legfontosabb írásait, és utal a cikkek beszámolója elején felvillantott szakmai trendekkel való kapcsolatára. A szerkesztőség számára a jövő útja a nyílt hozzáférés és az Open Journal System (OJS) nyílt forráskódú szerkesztőségi rendszer kialakítása felé vezet
Enfermedad de Wilson con una presentación neurológica grave: reporte de caso
No full textIntroducción: la enfermedad de Wilson (EW) es un trastorno autosómico recesivo que afecta a personas de cualquier edad. Se describe compromiso neurológico en el 30%-40% de pacientes, sus principales manifestaciones son la disartria, ataxia y distonía, y su relación con los anillos de Kayser-Fleischer (KF) está en el 77,2%-85,2%. El tratamiento quelante tiene una respuesta paradójica al tratamiento hasta en el 50%. Se presenta un caso con manifestación neurológica grave, en el que no se evidenciaron anillos de KF con respuesta paradójica al tratamiento quelante.
Presentación del caso: hombre de 23 años sin antecedentes con un cuadro de 6 meses compatibles con episodios sincopales vasovagales y posterior ataxia, debilidad muscular, disfagia, sialorrea y disartria, con un deterioro progresivo por sepsis de origen pulmonar secundario a un episodio de broncoaspiración. Se documentó ceruloplasmina baja, cobre urinario elevado, hallazgos imagenológicos de compromiso talámico y lenticulares, biopsia con hepatitis autoinmune like y cambios similares a enfermedad del hígado graso no alcohólico (EHGNA) sin anillos de KF con respuesta paradójica al manejo quelante, progresión de la distonía grave y desenlace fatal.
Conclusiones: la EW es una enfermedad con alta carga de morbilidad, con un espectro clínico variable. El compromiso neurológico aislado sin anillos de KF es inusual, sus desenlaces dependen del inicio temprano del quelante de cobre, teniendo en cuenta que implica también un riesgo de deterioro de los síntomas ante una respuesta paradójica al tratamiento, como se observó en el caso
Effectiveness of Low Dose D-Penicillamine Therapy in Neurologic Wilson Disease - A Prospective Observational Study
No full textBackground: Wilson disease is an inherited disorder of copper metabolism that mostly manifests as hepatic and neurologic symptoms. Chelation therapy specially penicillamine is given as first line treatment in children with symptomatic Neurologic Wilson disease. Objective: Objective of the study was to assess the safety & the clinical outcome of treatment with low dose penicillamine in Neurologic Wilson disease. Methods: A longitudinal observational study was conducted at Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, a tertiary care Premier Postgraduate Medical Institution of Bangladesh. Thirty-nine (39) patients of Neurologic Wilson disease who fulfill the inclusion and exclusion criteria were evaluated at In-patient Department of Paediatric Neurology, during the period of January 2015 to December 2019. All study children were treated with low dose penicillamine (Cap Artamin 250 mg) rather with conventional dose penicillamine (Cap Artamin 500 mg or 20 mg/kg/day). Subsequent follow up examination was performed at 2 weeks, 1, 3 and 6 months. Follow up was done by global assessment scoring (GAS) and slit lamp examination to see the clinical improvement after treatment with low dose penicillamine therapy. Results: Total number of studied cases were 39. Mean age was 10.2 ± 3.1 year and male to female ratio was 2:1. Most of the patients (66.67%) were arrived from rural area and 20.51% children had history of consanguineous mating parents. Common presenting features were progressive deterioration of school performance (89.74%), gait disturbance (92.31%), dysarthria (92.31%) and dystonia (82.06%) of our studied children. Ophthalmological manifestations like KF ring (100%) found in all patients. Neuroimaging showed bilateral basal ganglia involvement in (63.63%) children followed by hyperintense signal changes (18.18%) and ventricular dilatation in (18.18%) of cases. Majority of the children (74.36%) were improved with low penicillamine therapy clinically and KF ring disappeared in (5.12%) cases after drug therapy on follow up. Commonest side effects were worsening of neurological symptoms in (25.64%) and rash & thrombocytopenia in (5.1%) cases after penicillamine therapy. Conclusion: About three-fourth children of studied Neurologic Wilson disease showed gradual improvement with low-dose penicillamine therapy. Moreover, one-fourth of cases experienced neurological deterioration, which was lower than previously used high dose penicillamine therapy. Therefore, low dose penicillamine may be beneficial as an initial therapy for Wilson disease with neurological manifestations
Thermodynamic assessment of the LiF-NiF<sub>2</sub>, NaF-NiF<sub>2</sub> and KF-NiF<sub>2</sub> systems
No full textUsing the modified quasi-chemical model in the quadruplet approximation, three new thermodynamic assessments of binary systems useful for the detailed operational design of the Molten Salt Reactor are presented: AF-NiF2 (A = Li, Na, K). These systems are particularly relevant for the study of the molten salt-structural materials interaction, as the salt containment is made of a Ni-based alloy. Using powder X-ray Diffraction (XRD) and Differential Scanning Calorimetry (DSC), new experimental data were gathered for two of these systems, LiF-NiF2 and KF-NiF2, and compared to previous experimental assessments. Our data have confirmed the formation of a (Li1-2xNix)F solid solution. The three thermodynamic models show a very good agreement with the experimental data. The melting point of NiF2 was measured for the first time to be T = (1629 ± 5) K, and the thermal expansion coefficient for Li2NiF4 was found to be α=27.6·10-6K-1 in the temperature range T = (298–773) K.Accepted Author ManuscriptRST/Reactor Physics and Nuclear Material
Rare Presentation of Wilson Disease in an 11-year-old Sudanese Girl
No full textBackground: Wilson disease is an inherited disorder in which excessive amount ofcopper accumulates in various tissues of the body. Clinical features related to copperdeposition in the liver may appear in the first and second decades followed byneurologic and psychiatric thereafter; however, many patients have a combinationof these symptoms.Case: We report a case of 11 year-old girl, admitted to Wad Medani PediatricTeaching Hospital with generalized body swellings for four days. Initial investigationsshowed proteinuria and hypoalbuminemia, thought to be due to nephrotic syndrome.Days later, patient developed jaundice and neuropsychiatric manifestations. A slitlamb examination confirmed the presence of Kayser–Fleischer ring (KF ring) andshe scored high in the scoring system for the diagnosis of Wilson disease. Dpenicillaminetreatment therapy was started and unfortunately the patient’s clinicalcondition deteriorated gradually, and eventually went into deep coma and died. Wilsondisease mainly affects the liver, but the initial presentation was completely compatiblewith nephrotic syndrome.Conclusion: Diagnosis of Wilson disease should be suspected in a child presentingwith generalized body swellings even in the absence of clinical evidence of hepaticand/or neuropsychiatric involvements.Keywords: Wilson disease, nephrotic syndrome, case report, pediatrics, Suda
Analysis of airway secretions in a model of sulfur dioxide induced chronic obstructive pulmonary disease (COPD)
No full textHypersecretion and chronic phlegm are major symptoms of chronic obstructive pulmonary disease (COPD) but animal models of COPD with a defined functional hypersecretion have not been established so far. To identify an animal model of combined morphological signs of airway inflammation and functional hypersecretion, rats were continuously exposed to different levels of sulfur dioxide (SO2, 5 ppm, 10 ppm, 20 ppm, 40 ppm, 80 ppm) for 3 (short-term) or 20–25 (long-term) days. Histology revealed a dose-dependent increase in edema formation and inflammatory cell infiltration in short-term-exposed animals. The submucosal edema was replaced by fibrosis after long-term-exposure. The basal secretory activity was only significantly increased in the 20 ppm group. Also, stimulated secretion was significantly increased only after exposure to 20 ppm. BrdU-assays and AgNOR-analysis demonstrated cellular metaplasia and glandular hypertrophy rather than hyperplasia as the underlying morphological correlate of the hypersecretion.
In summary, SO2-exposure can lead to characteristic airway remodeling and changes in mucus secretion in rats. As only long-term exposure to 20 ppm leads to a combination of hypersecretion and airway inflammation, only this mode of exposure should be used to mimic human COPD. Concentrations less or higher than 20 ppm or short term exposure do not induce the respiratory symptom of hypersecretion. The present model may be used to characterize the effects of new compounds on mucus secretion in the background of experimental COPD
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