Blackshear, J L; Stark, M E; Agnew, R C; Moussa, I D; Safford, R E; Shapiro, B P; Waldo, O A; Chen, D
2015-02-01
Gastrointestinal hemorrhage is considered to be a severe complication of von Willebrand disease. The optimal therapy for acquired von Willebrand syndrome and severe gastrointestinal bleeding with hypertrophic cardiomyopathy is undefined. Seventy-seven patients (median age, 67 years; interquartile range [IQR], 56-75 years; 49% women) with hypertrophic cardiomyopathy underwent von Willebrand factor multimer testing and acquisition of bleeding history. Bleeding was detected in 27 (36%) (median age, 74 years; IQR 66-76 years; 74% women), 20 with gastrointestinal bleeding, including 11 women with transfusion dependence. In these 11 women, the median duration of transfusion dependency was 36 months (IQR 18-44 months), and the median number of transfusions required was 25 (IQR 20-38). Two patients had undergone bowel resection for bleeding, one of them twice. Seven patients showed angiodysplasia, and the remainder had no endoscopic lesion. Bleeding recurred after bowel surgery or endoscopic intervention and medical therapy for hypertrophic cardiomyopathy in 10 of 11 patients. Two patients had septal myectomy, and six patients underwent alcohol septal ablation. With the exception of one patient in whom a significant gradient persisted after septal ablation, after the periprocedural period, patients after septal reduction therapy remained free of recurrent bleeding and need for transfusions. Acquired von Willebrand syndrome is common in hypertrophic cardiomyopathy. Gastrointestinal bleeding often recurs after endoscopic therapy, but may be relieved by structural cardiac repair. © 2014 International Society on Thrombosis and Haemostasis.
Borràs, Nina; Batlle, Javier; Pérez-Rodríguez, Almudena; López-Fernández, María Fernanda; Rodríguez-Trillo, Ángela; Lourés, Esther; Cid, Ana Rosa; Bonanad, Santiago; Cabrera, Noelia; Moret, Andrés; Parra, Rafael; Mingot-Castellano, María Eva; Balda, Ignacia; Altisent, Carme; Pérez-Montes, Rocío; Fisac, Rosa María; Iruín, Gemma; Herrero, Sonia; Soto, Inmaculada; de Rueda, Beatriz; Jiménez-Yuste, Víctor; Alonso, Nieves; Vilariño, Dolores; Arija, Olga; Campos, Rosa; Paloma, María José; Bermejo, Nuria; Berrueco, Rubén; Mateo, José; Arribalzaga, Karmele; Marco, Pascual; Palomo, Ángeles; Sarmiento, Lizheidy; Iñigo, Belén; Nieto, María del Mar; Vidal, Rosa; Martínez, María Paz; Aguinaco, Reyes; César, Jesús María; Ferreiro, María; García-Frade, Javier; Rodríguez-Huerta, Ana María; Cuesta, Jorge; Rodríguez-González, Ramón; García-Candel, Faustino; Cornudella, Rosa; Aguilar, Carlos; Vidal, Francisco; Corrales, Irene
2017-01-01
Molecular diagnosis of patients with von Willebrand disease is pending in most populations due to the complexity and high cost of conventional molecular analyses. The need for molecular and clinical characterization of von Willebrand disease in Spain prompted the creation of a multicenter project (PCM-EVW-ES) that resulted in the largest prospective cohort study of patients with all types of von Willebrand disease. Molecular analysis of relevant regions of the VWF, including intronic and promoter regions, was achieved in the 556 individuals recruited via the development of a simple, innovative, relatively low-cost protocol based on microfluidic technology and next-generation sequencing. A total of 704 variants (237 different) were identified along VWF, 155 of which had not been previously recorded in the international mutation database. The potential pathogenic effect of these variants was assessed by in silico analysis. Furthermore, four short tandem repeats were analyzed in order to evaluate the ancestral origin of recurrent mutations. The outcome of genetic analysis allowed for the reclassification of 110 patients, identification of 37 asymptomatic carriers (important for genetic counseling) and re-inclusion of 43 patients previously excluded by phenotyping results. In total, 480 patients were definitively diagnosed. Candidate mutations were identified in all patients except 13 type 1 von Willebrand disease, yielding a high genotype-phenotype correlation. Our data reinforce the capital importance and usefulness of genetics in von Willebrand disease diagnostics. The progressive implementation of molecular study as the first-line test for routine diagnosis of this condition will lead to increasingly more personalized and effective care for this patient population. PMID:28971901
Schmidt-Recla, A; Steinberg, H
2008-03-01
Emil Kraepelin started his scientific career with a pamphlet demanding complete restructure of German penal law. It is well known that Kraepelin was a recipient of Cesare Lombroso's theses on degeneration and atavism. Therefore his demand for a correctional law completely replacing penal law is easily understood. Still undiscussed however is the question of whether Kraepelin's brochure had a decisive effect on German criminal law, especially on the so-called Marburg Program of Franz von Liszt, still viewed as the first emergence of modern criminal law and policies in Germany. Examination of this shows that despite major theoretical faults, Kraepelin came to conclusions that correspond remarkably with von Liszt's. Special focus should be directed on the psychologist Wilhelm Wundt, who criticised Kraepelin's juridical attempt in a very kind yet fundamental way, and on the relationship that existed between Kraepelin and von Liszt.
Of von Willebrand factor and platelets.
Bryckaert, Marijke; Rosa, Jean-Philippe; Denis, Cécile V; Lenting, Peter J
2015-01-01
Hemostasis and pathological thrombus formation are dynamic processes that require multiple adhesive receptor-ligand interactions, with blood platelets at the heart of such events. Many studies have contributed to shed light on the importance of von Willebrand factor (VWF) interaction with its platelet receptors, glycoprotein (GP) Ib-IX-V and αIIbβ3 integrin, in promoting primary platelet adhesion and aggregation following vessel injury. This review will recapitulate our current knowledge on the subject from the rheological aspect to the spatio-temporal development of thrombus formation. We will also discuss the signaling events generated by VWF/GPIb-IX-V interaction, leading to platelet activation. Additionally, we will review the growing body of evidence gathered from the recent development of pathological mouse models suggesting that VWF binding to GPIb-IX-V is a promising target in arterial and venous pathological thrombosis. Finally, the pathological aspects of VWF and its impact on platelets will be addressed.
Symmetries and solutions of the non-autonomous von Bertalanffy equation
NASA Astrophysics Data System (ADS)
Edwards, Maureen P.; Anderssen, Robert S.
2015-05-01
For growth in a closed environment, which is indicative of the situation in laboratory experiments, autonomous ODE models do not necessarily capture the dynamics under investigation. The importance and impact of a closed environment arise when the question under examination relates, for example, to the number of the surviving microbes, such as in a study of the spoilage and contamination of food, the gene silencing activity of fungi or the production of a chemical compound by bacteria or fungi. Autonomous ODE models are inappropriate as they assume that only the current size of the population controls the growth-decay dynamics. This is reflected in the fact that, asymptotically, their solutions can only grow or decay monotonically or asymptote. Non-autonomous ODE models are not so constrained. A natural strategy for the choice of non-autonomous ODEs is to take appropriate autonomous ones and change them to be non-autonomous through the introduction of relevant non-autonomous terms. This is the approach in this paper with the focus being the von Bertalanffy equation. Since this equation has independent importance in relation to practical applications in growth modelling, it is natural to explore the deeper relationships between the introduced non-autonomous terms through a symmetry analysis, which is the purpose and goal of the current paper. Infinitesimals are derived which allow particular forms of the non-autonomous von Bertalanffy equation to be transformed into autonomous forms for which some new analytic solutions have been found.
Guerrero, R.; Bain, O.
2011-01-01
Parasitic nematodes from the Berlin (ZMB) and Vienna (NMW) Museum collections referred to the genus Filaria Mueller, 1787 by von Linstow or Molin were studied. Three samples were in good condition and the specimens redescribed. Litomosa hepatica (von Linstow, 1897) n. comb., sample ZMB Vermes Entozoa 3368, from the megachiropteran Pteropus neohibernicus, Bismarck Archipelago, resembles L. maki Tibayrenc, Bain & Ramanchandran, 1979, from Pteropus vampyrus, in Malaysia, but the buccal capsule differs. Both species display particular morphological characters which differ from species of Litomosa parasitic in microchiropterans. The remaining material originates from Brazil. The spicule morphology of Litomosoides circularis (von Linstow, 1899) Chandler, 1931, sample ZMB Vermes Entozoa 1059 from Hesperomys spec. (= Holochilus brasiliensis), Porto Alegre, confirms that it belongs to the sigmodontis group; the microfilaria presents characters of the genus Litomosoides, e.g. body attenuated at both extremities and salient cephalic hook. Taxonomic discussions by others confirm that species of Litomosoides belonging to the sigmodontis group and described subsequently are distinct from L. circularis. Litomosoides serpicula (Molin, 1858) Guerrero, Martin, Gardner & Bain, 2002, is redescribed, sample NMW 6323 from the bat Phyllostoma spiculatum (= Sturnira lilium), Ypanema. It is very close to L. brasiliensis Almeida, 1936, type host Moytis sp., but distinguished by a single ring in the buccal capsule, rather than two, supporting previous conclusions that the taxon L. brasiliensis, as generally regarded, may represent a complex of species. Samples NMW 6322 and NMW 6324, from other bats and also identified by Molin (1858) as Filaria serpicula, contain unidentifiable fragments of Litomosoides incertae sedis. Filaria hyalina von Linstow, 1890, sample ZMB Vermes Entozoa Q 3905 from Sorex vulgaris (= Sorex araneus), is incertae sedis because it contains two unidentifiable posterior
Kompressionstherapie - Versorgungspraxis: Informationsstand von Patienten mit Ulcus cruris venosum.
Protz, Kerstin; Heyer, Kristina; Dissemond, Joachim; Temme, Barbara; Münter, Karl-Christian; Verheyen-Cronau, Ida; Klose, Katharina; Hampel-Kalthoff, Carsten; Augustin, Matthias
2016-12-01
Eine Säule der kausalen Therapie bei Patienten mit Ulcus cruris venosum ist die Kompressionstherapie. Sie unterstützt die Abheilung, reduziert Schmerzen und Rezidive und steigert die Lebensqualität. Bislang existieren kaum wissenschaftliche Daten zu dem Versorgungsstand und fachspezifischem Wissen von Patienten mit Ulcus cruris venosum. Standardisierte Fragebögen wurden bundesweit in 55 Pflegediensten, 32 Arztpraxen, vier Wundzentren und -sprechstunden sowie einem Pflegetherapiestützpunkt von Patienten mit Ulcus cruris venosum bei Erstvorstellung anonym ausgefüllt. Insgesamt nahmen 177 Patienten (Durchschnittsalter 69,4 Jahre; 75,1% Frauen) teil. Ein florides Ulcus cruris venosum bestand im Mittel 17Monate. 31,1% hatten keine Kompressionstherapie, 40,1% Binden und 28,8% Strümpfe. Bei der Bestrumpfung hatten 13,7% KompressionsklasseIII, 64,7% KompressionsklasseII und 19,6% KompressionsklasseI. 70,6% legten die Strümpfe nach dem Aufstehen an, 21,1% trugen sie Tag und Nacht. 39,2% bereiteten die Strümpfe Beschwerden. Lediglich 11,7% hatten eine An- und Ausziehhilfe. Die Binden wurden im Mittel 40,7Wochen getragen und bei 69% nicht unterpolstert. Bei 2,8% wurde der Knöchel- und Waden-Umfang zur Erfolgskontrolle gemessen. Venensport machten 45,9%. Ein Drittel hatte keine Kompressionsversorgung, obwohl diese eine Basismaßnahme der Therapie des Ulcus cruris venosum ist. Zudem ist deren korrekte Auswahl und Anwendung angesichts der langen Bestandsdauer der Ulzerationen zu hinterfragen. Weiterführende Fachkenntnisse bei Anwendern und Verordnern sowie Patientenschulungen sind erforderlich. © 2016 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.
Pathare, A; Al Hajri, F; Al Omrani, S; Al Obaidani, N; Al Balushi, B; Al Falahi, K
2018-05-13
Assessment of the severity of bleeding symptom has led to the evolution of bleeding assessment tools which are now validated. To administer the condensed molecular and clinical markers for the diagnosis and management of type 1 von Willebrand disease VWD (MCMDM-1 vWD) questionnaire to the Omani type 1 vWD patients and correlate it with the laboratory parameters. Patients and controls were personally interviewed and the condensed MCMDM-1 vWD questionnaire administered by a single investigator. Bleeding score (BS) was calculated, based on the presence or absence of the bleeding symptoms according to a standard validated questionnaire in both the patients and the controls. The median age of the patient cohort was 27 (range, 7-49) years with 60.87% of females. The median time to administer condensed MCMDM-1 BS questionnaire was 11minutes (interquartile range-IQR;7,16). Overall, bleeding from the oral cavity was the most predominant symptom (63%). The median BS was 5 (IQR;1,8) although individual scores ranged between 0 and 29. However, there was no statistically significant difference in BS between genders (males: median 4; IQR 1,6 and females: median 5, IQR 1,10) (P>.05, Kruskal-Wallis test) The Spearman's correlation value of BS was weak with FVIII:C levels and von Willebrand Ristocetin co-factor activity; very weak with von Willebrand Antigen level, and moderate with vonWillebrand Collagen Binding activity being -0.29, -0.28, -0.14 and -0.43, respectively. The BS reflects the severity of bleeding among the vWD patients. Although the BS was abnormal, it did not correlate significantly with the surrogate laboratory parameters [P>.05]. © 2018 John Wiley & Sons Ltd.
Conrad, Franziska; Winkens, Thomas; Kaatz, Martin; Goetze, Steven; Freesmeyer, Martin
2016-08-01
Bei der (18) F-Fluordeoxyglucose-Positronenemissionstomographie/Computertomographie (FDG-PET/CT) ergeben sich häufig Zufallsbefunde. In der vorliegenden Studie konzentrierten wir uns auf mittels FDG-PET/CT erhaltene Zufallsbefunde bei Patienten mit kutanem Melanom und überprüften deren Relevanz hinsichtlich weiterer diagnostischer Maßnahmen und Interventionen. Die Krankenakten von 181 konsekutiven Melanom-Patienten wurden retrospektiv ausgewertet, um das Management von Zufallsbefunden zu dokumentieren. Der Schwerpunkt lag dabei auf den histologischen Befunden. Bei 33 von 181 (18 %) Patienten lagen 39 relevante Zufallsbefunde vor, und zwar im Kolorektalbereich (n = 15 Patienten), in der Schilddrüse (n = 8), der Prostata (n = 2), dem Bewegungsapparat (n = 2), in Lymphknoten (n = 2), der Parotis (n = 1), den Mandeln (n = 1), den Nieren (n = 1) und der Gallenblase (n = 1). Bei 25 Patienten schlossen sich weitere diagnostische Verfahren an, wobei in 21 Fällen ein klinisches Korrelat nachgewiesen wurde. Bei 16 von 21 Patienten ergab sich eine Neoplasie, darunter fünf maligne Läsionen (vier Kolonkarzinome und ein Prostatakarzinom). Die Malignome wurden frühzeitig diagnostiziert und in der Mehrzahl der Fälle erfolgreich entfernt. Der Einsatz der FDG-PET/CT als Routine-Diagnostik wird in den Leitlinien empfohlen und ist indiziert bei malignem Melanom ab Stadium IIC. In dieser Studie wurden auf effektive Weise ansonsten nicht erkannte Krebserkrankungen, insbesondere Kolonkarzinome, detektiert. In den meisten Fällen war eine frühe Intervention möglich. Zufallsbefunde durch FDG-PET/CT sollten, unter Berücksichtigung des Zustands und der Wünsche des Patienten, mit den geeigneten diagnostischen Maßnahmen abgeklärt werden. © 2016 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.
Borràs, Nina; Batlle, Javier; Pérez-Rodríguez, Almudena; López-Fernández, María Fernanda; Rodríguez-Trillo, Ángela; Lourés, Esther; Cid, Ana Rosa; Bonanad, Santiago; Cabrera, Noelia; Moret, Andrés; Parra, Rafael; Mingot-Castellano, María Eva; Balda, Ignacia; Altisent, Carme; Pérez-Montes, Rocío; Fisac, Rosa María; Iruín, Gemma; Herrero, Sonia; Soto, Inmaculada; de Rueda, Beatriz; Jiménez-Yuste, Víctor; Alonso, Nieves; Vilariño, Dolores; Arija, Olga; Campos, Rosa; Paloma, María José; Bermejo, Nuria; Berrueco, Rubén; Mateo, José; Arribalzaga, Karmele; Marco, Pascual; Palomo, Ángeles; Sarmiento, Lizheidy; Iñigo, Belén; Nieto, María Del Mar; Vidal, Rosa; Martínez, María Paz; Aguinaco, Reyes; César, Jesús María; Ferreiro, María; García-Frade, Javier; Rodríguez-Huerta, Ana María; Cuesta, Jorge; Rodríguez-González, Ramón; García-Candel, Faustino; Cornudella, Rosa; Aguilar, Carlos; Vidal, Francisco; Corrales, Irene
2017-12-01
Molecular diagnosis of patients with von Willebrand disease is pending in most populations due to the complexity and high cost of conventional molecular analyses. The need for molecular and clinical characterization of von Willebrand disease in Spain prompted the creation of a multicenter project (PCM-EVW-ES) that resulted in the largest prospective cohort study of patients with all types of von Willebrand disease. Molecular analysis of relevant regions of the VWF , including intronic and promoter regions, was achieved in the 556 individuals recruited via the development of a simple, innovative, relatively low-cost protocol based on microfluidic technology and next-generation sequencing. A total of 704 variants (237 different) were identified along VWF , 155 of which had not been previously recorded in the international mutation database. The potential pathogenic effect of these variants was assessed by in silico analysis. Furthermore, four short tandem repeats were analyzed in order to evaluate the ancestral origin of recurrent mutations. The outcome of genetic analysis allowed for the reclassification of 110 patients, identification of 37 asymptomatic carriers (important for genetic counseling) and re-inclusion of 43 patients previously excluded by phenotyping results. In total, 480 patients were definitively diagnosed. Candidate mutations were identified in all patients except 13 type 1 von Willebrand disease, yielding a high genotype-phenotype correlation. Our data reinforce the capital importance and usefulness of genetics in von Willebrand disease diagnostics. The progressive implementation of molecular study as the first-line test for routine diagnosis of this condition will lead to increasingly more personalized and effective care for this patient population. Copyright© 2017 Ferrata Storti Foundation.
Antonini, Tanya N; Van Horn Kerne, Valerie; Axelrad, Marni E; Karaviti, Lefkothea P; Schwartz, David D
2015-07-01
DK phocomelia/von Voss Cherstvoy syndrome is a rare condition characterized by upper limb and urogenital abnormalities and various brain anomalies. Previously reported cases have noted significant developmental delays, although no formal testing of cognitive abilities has been reported. In this paper we describe results from a comprehensive neuropsychological evaluation of a 12-year-old male with DK phocomelia syndrome. Test findings indicated mild impairment in intellectual functioning, with more significant impairment in adaptive skills and academic achievement. The neuropsychological profile converged with neurological findings, showing a distinct pattern of strengths and weaknesses that suggests functional compromise of posterior brain regions with relatively well-preserved functioning of more anterior regions. Specifically, impairments were evident in perceptual reasoning, visual perception, and visuomotor integration, whereas normal or near normal functioning was evident in memory, receptive language, social cognition, attention, and most aspects of executive functioning. To our knowledge this is the first report to describe the neurocognitive profile of an individual with DK phocomelia syndrome. © 2015 Wiley Periodicals, Inc.
[Homocysteine and von Willebrand factor in chronic alcoholism].
Koriakin, A M; Epifantseva, N N; Dadyka, I V; Gorbatovskiĭ, Ia A
2010-04-01
The levels of homocysteine (HC) and von Willebrand factor (VWF) as cardiovascular risk factors were studied in patients with Stage II chronic alcoholism. Forty-one men with Stage II chronic alcoholism without clinical signs of somatic and infectious diseases were examined. Their median age was 37 (range 32-40) years; the alcoholization period was 12 (range 8-17) years. Plasma HC and VWF (amount and activity) levels were determined. In 63.4% of chronic alcoholic patients, HC levels was twice as high as in the controls; in 80.6%, both the content and activity of VWF were increased. There was no correlation between the levels of HC and VWF. Vascular endothelial damage concurrent with hyperhomocysteinemia increases a cardiovascular risk in patients with Stage II chronic alcoholism.
Hypoxia and cell cycle regulation of the von Hippel-Lindau tumor suppressor
Liu, Weijun; Xin, Hong; Eckert, David T.; Brown, Julie A.; Gnarra, James R.
2010-01-01
Inactivation of von Hippel-Lindau tumor suppressor protein (pVHL) is associated with von Hippel-Lindau disease, an inherited cancer syndrome, as well as the majority of patients with sporadic clear cell renal carcinoma (RCC). While the involvement of pVHL in oxygen sensing through targeting HIFα subunits to ubiquitin-dependent proteolysis has been well documented, less is known about pVHL regulation under both normoxic and hypoxic conditions. We found that pVHL levels decreased in hypoxia and that hypoxia-induced cell cycle arrest is associated with pVHL expression in RCC cells. pVHL levels fluctuate during the cell cycle, paralleling cyclin B1 levels, with decreased levels in mitosis and G1. pVHL contains consensus Destruction box sequences, and pVHL associates with Cdh1, an activator of the anaphase promoting complex/cyclosome (APC/C) E3 ubiquitin ligase. We show that pVHL has a decreased half-life in G1, Cdh1 downregulation results in increased pVHL expression, while Cdh1 overexpression results in decreased pVHL expression. Taken together these results suggest that pVHL is a novel substrate of APC/CCdh1. Destruction box-independent pVHL degradation was also detected, indicating that other ubiquitin ligases are also activated for pVHL degradation. PMID:20802534
PREFACE: EmerQuM 11: Emergent Quantum Mechanics 2011 (Heinz von Foerster Congress)
NASA Astrophysics Data System (ADS)
Grössing, Gerhard
2012-05-01
These proceedings comprise the plenary lectures and poster contributions of the 'Heinz von Foerster Conference 2011' on Emergent Quantum Mechanics (EmerQuM11), which was held at the University of Vienna, 11-13 November 2011. With the 5th International Heinz von Foerster Conference convened at the occasion of von Foerster's 100th birthday, the organizers opted for a twin conference to take place at the Large and Small Ceremonial Halls of the University's main building, respectively. The overall topic was chosen as 'Self-Organization and Emergence', a topic to which von Foerster was an early contributor. While the first conference ('Self-Organization and Emergence in Nature and Society') addressed a more general audience, the second one ('Emergent Quantum Mechanics') was intended as a specialist meeting with a contemporary topic that could both serve as an illustration of von Foerster's intellectual heritage and, more generally, point towards future directions in physics. We thus intended to bring together many of those physicists who are interested in or are working on attempts to understand quantum mechanics as emerging from a suitable classical (or, more generally, deeper level) physics. EmerQuM11 was organized by the Austrian Institute for Nonlinear Studies (AINS), with essential support from the Wiener Institute for Social Science Documentation and Methodology (WISDOM), the Department of Contemporary History at the University of Vienna, and the Heinz von Foerster-Gesellschaft. There were a number of individuals who contributed to the smooth course of our meeting and whom I would like to sincerely thank: Christian Bischof, Thomas Elze, Marianne Ertl, Gertrud Hafner, Werner Korn, Angelika Krawanja, Florian Krug and his team, Sonja Lang, Albert Müller, Ilse Müller, Irene Müller, Karl Müller, Armin Reautschnig, Marion Schirrmacher, Anton Staudinger, Roman Zlabinger, and, last but not least, my AINS colleagues Siegfried Fussy, Herbert Schwabl and Johannes Mesa
[Countryside obstetrics--Dr. Franz von Ottenthal and the South Tyrolean Medical Market (1860-1869)].
Hilber, Marina
2012-01-01
The paper focuses on the structure of the obstetric market in a rural region of alpine South Tyrol (today: Italy) throughout the 1860s. Besides midwives and parturient women, also male obstetricians are traceable in the actual research area of the Tauferer Ahrntal. Among them was the general practitioner Dr. Franz von Ottenthal, whose medical records (Historiae Morborum, 1847-1899) are used to reconstruct the participation of physicians in the obstetric market. Special emphasis lies on the evaluation of the predominant hierarchies (midwives/surgeons/physicians) and the position of Franz von Ottenthal in this specialized medical sub-segment. Therefore, the quantitative extent of obstetric intervention as well as the qualitative dimension of treatments (medication, surgeries) is investigated. So far, the relevance of Ottenthals obstetric practice as to the gender ratio has been measured as rather high, considering that he generally treated more female than male patients. An attempt will be made to estimate the significance of the obstetric segment within his practice in order to find an explanatory approach for the higher female medical demand.
Wilhelm von Humboldt and the "Orient": On Edward W. Said's Remarks on Humboldt's Orientalist Studies
ERIC Educational Resources Information Center
Messling, Markus
2008-01-01
From an epistemological perspective, Wilhelm von Humboldt's studies on the Oriental and East Asian languages and writing systems (Egyptian hieroglyphs, Sanskrit, Chinese, Polynesian) raise the question of his position in the Orientalist discourse of his time. Said [Said, E.W., 1978. "Orientalism. Western Conceptions of the Orient, fourth…
Obser, T; Ledford-Kraemer, M; Oyen, F; Brehm, M A; Denis, C V; Marschalek, R; Montgomery, R R; Sadler, J E; Schneppenheim, S; Budde, U; Schneppenheim, R
2016-09-01
Essentials Von Willebrand disease IIC Miami features high von Willebrand factor (VWF) with reduced function. We aimed to identify and characterize the elusive underlying mutation in the original family. An inframe duplication of VWF exons 9-10 was identified and characterized. The mutation causes a defect in VWF multimerization and decreased VWF clearance from the circulation. Background A variant of von Willebrand disease (VWD) type 2A, phenotype IIC (VWD2AIIC), is characterized by recessive inheritance, low von Willebrand factor antigen (VWF:Ag), lack of VWF high-molecular-weight multimers, absence of VWF proteolytic fragments and mutations in the VWF propeptide. A family with dominantly inherited VWD2AIIC but markedly elevated VWF:Ag of > 2 U L(-1) was described as VWD type IIC Miami (VWD2AIIC-Miami) in 1993; however, the molecular defect remained elusive. Objectives To identify the molecular mechanism underlying the phenotype of the original VWD2AIIC-Miami. Patients and Methods We studied the original family with VWD2AIIC-Miami phenotypically and by genotyping. The identified mutation was recombinantly expressed and characterized by standard techniques, confocal imaging and in a mouse model, respectively. Results By Multiplex ligation-dependent probe amplification we identified an in-frame duplication of VWF exons 9-10 (c.998_1156dup; p.Glu333_385dup) in all patients. Recombinant mutant (rm)VWF only presented as a dimer. Co-expressed with wild-type VWF, the multimer pattern was indistinguishable from patients' plasma VWF. Immunofluorescence studies indicated retention of rmVWF in unusually large intracellular granules in the endoplasmic reticulum. ADAMTS-13 proteolysis of rmVWF under denaturing conditions was normal; however, an aberrant proteolytic fragment was apparent. A decreased ratio of VWF propeptide to VWF:Ag and a 1-desamino-8-d-arginine vasopressin (DDAVP) test in one patient indicated delayed VWF clearance, which was supported by clearance data after
Modeling of a Von Platen-Munters diffusion absorption refrigeration cycle
NASA Astrophysics Data System (ADS)
Agostini, Bruno; Agostini, Francesco; Habert, Mathieu
2016-09-01
This article presents a thermodynamical model of a Von-Platen diffusion absorption refrigeration cycle for power electronics applications. It is first validated by comparison with data available in the literature for the classical water-ammonia-helium cycle for commercial absorption fridges. Then new operating conditions corresponding to specific ABB applications, namely high ambient temperature and new organic fluids combinations compatible with aluminium are simulated and discussed. The target application is to cool power electronics converters in harsh environments with high ambient temperature by providing refrigeration without compressor, for passive components losses of about 500 W, with a compact and low cost solution.
Contiguity and Entire Separability of States on von Neumann Algebras
NASA Astrophysics Data System (ADS)
Haliullin, Samigulla
2017-12-01
We introduce the notions of the contiguity and entirely separability for two sequences of states on von Neumann algebras. The ultraproducts technique allows us to reduce the study of the contiguity to investigation of the equivalence for two states. Here we apply the Ocneanu ultraproduct and the Groh-Raynaud ultraproduct (see Ocneanu (1985), Groh (J. Operator Theory, 11, 2, 395-404 1984), Raynaud (J. Operator Theory, 48, 1, 41-68, 2002), Ando and Haagerup (J. Funct. Anal., 266, 12, 6842-6913, 2014)), as well as the technique developed in Mushtari and Haliullin (Lobachevskii J. Math., 35, 2, 138-146, 2014).
Amadeo, A; Bertulezzi, G; Civelli, L; Porta, C; Moroni, M
1998-10-01
We found high endothelin-1 and von Willebrand factor plasma titers not only in two individuals (daughter and father) affected with Pseudoxanthoma elasticum but also in a young unaffected relative. These findings raise the possibility that these molecules could be the first biochemical fingerprints of this, still not clinically evident, rare inherited disorder of elastic tissue.
