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Sample records for von recklinghausen disease

  1. [Von Recklinghausen disease and hepatic neurofibromatosis].

    Science.gov (United States)

    Guzman Toro, F; Hinestroza, D; Colmenares, D

    1995-01-01

    Von Recklinghausen's neurofibromatosis is one of the most common autosomal dominant disease with an estimated frecuency of 1:3000 live births. Characteristic lesions include cafe-au-lait spots and neurofibromas following the path of peripheral nerves. Liver involvement by neurofibromatosis is rare and very few cases have been reported. We present a case of a young man with Von Recklinhausen's disease and hepatic neurofibromatosis with multiple caf-au-lait spots, cutaneous neurofibromas, short stature and osseous lesions and compare the clinical, radiological, surgical and anatomopathological findings with others describe previously in the literature.

  2. A rare cause of obstructive jaundice and weight loss in Von Recklinghausen's disease

    NARCIS (Netherlands)

    Bukkems, S.F.; Stoot, J.H.M.B.; Driessen, A.L.C.; Beets Tan, R.G.; van Nieuwenhoven, M.A.; Masclee, A.

    2010-01-01

    We present the case of a patient with the rare triad of Von Recklinghausen's disease associated with a somatostatinoma and a gastrointestinal stromal tumour (GIST). The patient had recurrent jaundice, the typical somatostatinoma syndrome, positive MR imaging but negative Ga-DOTATOC PET scanning in a

  3. Congenital hepatic fibrosis associated with von Recklinghausen's disease Fibrosis hepática congénita asociada a enfermedad de von Recklinghausen

    Directory of Open Access Journals (Sweden)

    O. A. Jorge

    2006-09-01

    Full Text Available Congenital hepatic fibrosis is characterized by a ductal plate malformation with duct-like structures and fibrosis. It manifests clinically with portal hypertension and may be associated with multiple congenital defects. We present the case of a 16-year-old male with splenomegaly, leukopenia and thrombocytopenia, esophageal varices, and a histopathological diagnosis of congenital hepatic fibrosis. He exhibits "café au lait' spots and "Lisch' nodules, with a diagnosis of von Recklinghausen's disease. Congenital hepatic fibrosis belongs to the so-called fibropolycystic diseases, in which there is a disordered interaction between cells and the extracellular matrix. Von Recklinghausen's disease affects tissues derived from the neural crest and its diagnosis is based on clinical criteria. It is associated with multiple diseases. We describe its association with congenital hepatic fibrosis for the first time.La fibrosis hepática congénita se origina como consecuencia de una malformación de la placa ductal con estructuras tipo ductales acompañadas de fibrosis. Se manifiesta con hipertensión portal y puede asociarse a múltiples defectos congénitos. Presentamos un varón de 16 años con esplenomegalia, leuco- y plaquetopenia, varices esofágicas y diagnóstico histopatológico de fibrosis hepática congénita. La exploración física mostraba la existencia de manchas de "café con leche' y nódulos de "Lisch' con diagnóstico de enfermedad de von Recklinghausen. La fibrosis hepática congénita forma parte de las enfermedades fibropoliquísticas donde existiría una alteración en la interacción entre las células y la matriz extracelular. La enfermedad de von Recklinghausen afecta a los tejidos derivados de la cresta neural y su diagnóstico se basa en criterios clínicos. Se asocia a múltiples patologías. Presentamos por primera vez su asociación con fibrosis hepática congénita.

  4. Classical diagnostic radiological features of Von Recklinghausen's ...

    African Journals Online (AJOL)

    Neurofibromatosis type 1(NF-1, Von Recklinghausen disease or peripheral NF) is a rare autosomal dominant disorder with varied clinical manifestations involving the skin, nerves and bones. It is the most common of the neurocutaneous syndromes, with variable pathological and clinical expression. Approximately half of all ...

  5. Raquianestesia para cirurgia ortopédica de urgência em paciente portadora de doença de Von Recklinghausen: relato de caso Raquianestesia para cirugía ortopédica de urgencia en una paciente portadora de enfermedad de Von Recklinghausen: relato de caso Spinal block for urgency orthopedic surgery in Von Recklinghausen's disease patient: case report

    Directory of Open Access Journals (Sweden)

    Fabiano Timbó Barbosa

    2005-12-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: A doença de Von Recklinghausen é uma doença neurogenética causada por uma anomalia no cromossomo 17. O objetivo deste relato foi descrever a conduta anestésica em paciente com doença de Von Recklinghausen submetida à cirurgia ortopédica de urgência. RELATO DO CASO: Paciente do sexo feminino, 28 anos, portadora da doença de Von Recklinghausen, submetida à cirurgia ortopédica em hospital de emergência sob raquianestesia. Não apresentou complicações no período intra-operatório, nem na sala de recuperação pós-anestésica. Teve alta hospitalar no 4º dia de pós-operatório. CONCLUSÕES: No caso relatado foi possível a realização da raquianestesia sem haver necessidade de manuseio da via aérea.JUSTIFICATIVA Y OBJETIVOS: La enfermedad de Von Recklinghausen es una enfermedad neurogenética causada por una anomalía en el cromosoma 17. Este relato tiene por objetivo describir la conducta anestésica en una paciente con enfermedad de Von Recklinghausen sometida a cirugía ortopédica de urgencia. RELATO DEL CASO: Paciente del sexo femenino, 28 años, portadora de la enfermedad de Von Recklinghausen, sometida a cirugía ortopédica en un hospital de emergencia con raquianestesia. No presentó complicaciones en el período intra-operatorio, tampoco en la sala de recuperación pos-anestésica. Tuvo alta hospitalar en el 4º día de pos-operatorio. CONCLUSIONES: En el caso relatado fue posible la realización de la raquianestesia sin haber necesidad de manejar de la vía aérea.BACKGROUND AND OBJECTIVES: Von Recklinghausen's disease is a neurogenetic disease due to chromosome 17 abnormality. This report aimed at describing the anesthetic approach for Von Recklinghausen's disease patient submitted to urgency orthopedic surgery. CASE REPORT: Female patient, 28 years old, with Von Recklinghausen's disease, submitted to emergency orthopedic surgery under spinal block. There have been no complications both in

  6. Virchow's triad: Kussmaul, Quincke and von Recklinghausen.

    Science.gov (United States)

    Stanifer, John W

    2016-02-01

    For most of the 19th century, Germany was the centre of the medical world. From there the most innovating research came and many of the physicians of that era are known to nearly every medical student and physician of today. Virchow, Kussmaul, Quincke, von Recklinghausen, Müller and Schönlein are familiar names in today's medicine but insofar as they are merely eponyms associated with signs, symptoms, disease and anatomy. The story of their lives, their research and their influence on each other has been little examined. This is an essay about Virchow's relationship with his mentors Müller and Schönlein and how these relationships shaped the development of Kussmaul, Quincke and von Recklinghausen as students of Virchow and their work in medicine and clinical observation after leaving Virchow's laboratory. © The Author(s) 2014.

  7. [Orthopaedic manifestations of Von Recklinghausen's neurofibromatosis].

    Science.gov (United States)

    Trigui, Moez; Ayadi, Kamel; Sakka, Mourad; Zribi, Wassim; Frikha, Faten; Gdoura, Fakher; Sallemi, Sami; Zribi, Mohamed; Keskes, Hassib

    2011-03-01

    Von Recklinghausen's neurofibromatosis is a dominant autosomic genetic disease characterized by different clinical manifestations. The goal of this work was to study its orthopaedic manifestations and to show the characteristics of their management. A retrospective study was carried out on 15 patients having a Von Recklinghausen's neurofibromatosis. For each patient, different orthopaedic manifestations and their evolution after treatment were analyzed. These manifestations were classified in spinal deformities, pseudarthrosis of long bones and tumours of the peripheral nerves. The spinal deformities were observed in 9 cases. A dystrophic scoliosis was observed in 6 patients with an average angle of 50° and was associated to a kyphosis in 5 patients. The treatment was surgical by posterior arthrodesis in 2 cases and circumferential arthrodesis in 2 cases. The congenital curves and pseudarthroses of leg were observed in 5 cases, localized at the lower third of the leg in all cases. An Ilizarov external fixator with segmental osseous transport was carried out in 2 patients. The duration of the external fixator was 23 months ½ with 5 interventions in each case. Four plexiform neurofibromas and 3 nodular neurofibromas were observed. A transformation into neurofibrosarcoma was found in 2 patients. In one case, a resection without functional sacrifice was carried out and in the other case the patient was dead before the resection. The orthopaedic manifestations of Von Recklinghausen's neurofibromatosis are frequent, varied and have a difficult management. The functional and sometimes vital prognoses are challenging. Copyright © 2010 Elsevier Masson SAS. All rights reserved.

  8. Posterior approach for giant S1 neurofibroma in Von Recklinghausen's disease: Is total resection realistic?

    Science.gov (United States)

    Kumar, Ashish; Vinjamuri, Srinivas; Barada, Sahu P

    2013-10-01

    Bilateral sacral neurofibromas are uncommonly seen in neurofibromatosis type 1 (NF1) also known as Von Recklinghausen's disease. They often grow to enormous dimensions before detection owing to bone scalloping. Resections of "giant" S1 neurofibromas are difficult due to the limitations of operative window in sacrum and critical functions associated with S1 nerve root. We report a case of bilateral S1 neurofibromas in a patient of NF1 where she had a giant left-sided neurofibroma with extensive bone erosion and a small fusiform neurofibroma on the right side. The tumor was excised completely on the left and near totally on the right side via posterior approach. There were no postoperative neurological deficits and the patient recovered well. Usually, complete excision harbors the chances of postoperative neurological deficits due to the eloquence of the nerve root involved and complete resection without significant morbidity seems unrealistic. However, excision of giant ones may not result in grave deficits always if the patient is neurologically intact before surgery. Also, in selected patients, only posterior approach may suffice for giant neurofibromas with extensive bone scalloping and complete removal can be attempted successfully despite narrow corridors.

  9. Malignant transformation in von recklinghausen disease in a rural farmer presenting as huge metastatic soft tissue chest wall tumour: a case report.

    Science.gov (United States)

    Ekpe, E E

    2014-01-01

    Although von Recklinghausen disease is relatively common at an incidence of 1 in 4000 live births, malignant transformation in a neurofibromatosis is quite rare estimated at about 5% therefore the importance of life-long follow up of all patients von Recklinghausen disease. This is a case report of a 45-year old Nigerian-Ibibio male farmer who presented with six months history of rapid growth in one of the numerous skin nodules which had been present since his teenage age. The clinical diagnosis of malignant change in von Recklinghausen's disease was confirmed by histopathologic examination of an incisional biopsy specimen. Radiological investigations also showed the metastatic status of the disease. The patient could not afford the cytotoxic chemotherapeutic agents prescribed. He therefore left the hospital and failed to return when a free-treatment voucher was ready for treatment in the following two days. He is assumed dead at home. Lifelong follow up for von Recklinghausen disease is emphasized to enable early detection of malignant change and also expansion of the national health insurance scheme to benefit the unemployed citizens.

  10. [Emergent Caesarean Section in a Patient with a Mediastinal Tumor and Von Recklinghausen Disease: A Case Report].

    Science.gov (United States)

    Owada, Mayuko; Inomata, Shinichi; Danmura, Masato; Yamada, Kumiko; Tanaka, Makoto

    2016-06-01

    It is rare to encounter a pregnant patient with a mediastinal tumor, and if the tumor size increases as the pregnancy progresses, this increase can cause complications such as airway constriction and vascular occlusion. We report a case of a pregnant patient diagnosed with von Recklinghausen disease at the age of seven and diagnosed with a mediastinal tumors just after her present admission. The impending suffocation progressed and fetal heart rate decreased during her hospitalization. Her trachea was intubated and she was moved to an operating room for an emergent cesarean section under general anesthesia. With this rapid response, we could rescue both patient and infant. If the size of mediastinal tumor increases as pregnancy progresses, the tumor will cause suffocation by airway compression from the outside, in addition to specific airway edema on the inward side. The present case demonstrates that appropriate desisoins must be made for airway manegement and initiation of surgery.

  11. Fibrolipoma en un paciente con la enfermedad de Von Recklinghausen

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    Orlando L Rodríguez Calzadilla

    2000-08-01

    Full Text Available Se presenta el caso de un paciente, con antecedente de padecer de la enfermedad de Von Recklinghausen, el cual fue valorado y su conducta terapéutica fue diferida, por sospechar la presencia de un tumor neurofibromatoso. El paciente se remitió a la consulta externa de nuestro servicio y una vez concluido sus estudios, se realizó su tratamiento quirúrgco. El paciente no presentó complicaciones transoperatorias y posoperatorias; su estadía fue de 3 días. Anatomía patológica informó la presencia de un fibrolipoma (neoplasia mesodérmica. El control a los 3 años de operado mostró un paciente asintomático y sin recidiva local. En la literatura médica revisada no se informaron otros casos similares de pacientes con la presencia de un fibrolipoma en la enfermedad de Von Recklinghausen.The case of a patient who suffers from Von Recklinghausen´s disease is reported. The patient was evaluated and his therapeutic conduct was deferred because the presence of a neurofibromatous tumor was suspected. The patient was referred to the outpatient department of our service and once the studies were concluded, he underwent surgical treatment. The patient had no transoperative or postoperative complications. He stayed in hospital only 3 days. The Department of Pathological Anatomy reported the presence of a fibrolipoma (mesodermic neoplasia. 3 years later the patient proved to be asymptomatic and without local relapse. Similar cases of patients with a fibrolipoma in Von Recklinghausen´s disease are not reported in the reviewed medical literature.

  12. Primary carcinoma of the lung in von Recklinghausen neurofibromatosis

    Directory of Open Access Journals (Sweden)

    Gupta K

    2009-01-01

    Full Text Available von Recklinghausen neurofibromatosis (NF-1 is the most common inherited syndrome predisposing to neoplasia, particularly neural crest-derived tumors. However, lung malignancies reported in association with neurofibromatosis are sparse. We present a case of a 48-year-old man with NF-1 that manifested as carcinoma of lung, in order to discuss the linkage between these two entities.

  13. Neuropsychological function in adults with von Recklinghausen's neurofibromatosis.

    Science.gov (United States)

    Pavol, Marykay; Hiscock, Merrill; Massman, Paul; Moore Iii, Bartlett; Foorman, Barbara; Meyers, Christina

    2006-01-01

    The cognitive function of adults with von Recklinghausen's neurofibromatosis (NF-1) was examined. This study sought to replicate in an adult sample the findings of decreased visuospatial and attention abilities reported for many children with NF-1. Specifically, it was anticipated that adults with NF-1 would be classified separately from unaffected controls according to visual and attention-executive function skills. Second, this study examined whether language skills discriminated between adults with NF-1 and unaffected controls. The sample included 20 participants with NF-1 and 25 control participants. All participants were administered a battery of neuropsychological tests (Developmental Test of Visual-Motor Integration (VMI), Judgment of Line Orientation (JLO), Visual Form Discrimination, Booklet Category Test, Figure Cancellation, Peabody Picture Vocabulary Test-Revised (PPVT-R), Sentence Repetition, Controlled Oral Word Association). The results of a discriminant function analysis partially supported the hypothesis: Two of the tests of visual-spatial skill (VMI, JLO) and one of the language tests (PPVT-R) were found to be the best predictors for group membership. The discriminant function accounted for 45% of the variance between the groups and correctly classified 15 of the NF-1 participants and 21 of the control participants. Post hoc exploratory analyses revealed that the VMI was the most important test in discriminating between the groups. It is suggested that patients with NF-1 will tend to have sparing of basic cognitive functions but will have greater impairment on tests that use multiple cognitive skills.

  14. [Consanguineous marriage and morbi-mortality, short literature review based on an exceptional association: Usher syndrome and Von Recklinghausen neurofibromatosis].

    Science.gov (United States)

    Atipo-Tsiba, Pépin-Williams

    2016-01-01

    Usher syndrome is defined by the association of a progressive or non-progressive congenital sensorineural hearing loss with variable severity and a gradually blinding pigmentary retinopathy. Von Recklinghausen neurofibromatosis or Neurofibromatosis type 1 is the major clinically form of neurofibromatosis which occurs in approximately 90% of cases. Both types of disease are genetic in origin with very low prevalence. The probability of co-occurrence of these diseases in a single individual is exceptional. Inbreeding, as well as all genetic diseases, increases quite significantly the probability of their occurrence. Consanguineous marriages are still widespread in Maghreb and in some regions of the western African. This observation reports an exceptional case of this association in a 40-year-old man of Mauritanian origin born from a consanguineous union.

  15. Totally Laparoscopic Gastrectomy for Gastric Cancer Associated with Recklinghausen's Disease

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    Yoshihisa Sakaguchi

    2010-01-01

    Full Text Available This paper documents the first case of gastric cancer associated with Recklinghausen's disease, which was successfully treated by a totally laparoscopic operation. A 67-year-old woman with Recklinghausen's disease was referred to this department to undergo surgical treatment for early gastric cancer. The physical examination showed multiple cutaneous neurofibromas throughout the body surface, which made an upper abdominal incision impossible. Laparoscopic surgery requiring only small incisions was well indicated, and a totally laparoscopic distal gastrectomy with lymph node dissection was performed. Billroth I reconstruction was done intra-abdominally using a delta-shaped anastomosis. The patient followed a satisfactory postoperative course with no complications. Since the totally laparoscopic gastrectomy has many advantages over open surgery, it should therefore be preferentially used as a less invasive treatment in the field of gastric cancer.

  16. von Willebrand Disease

    Science.gov (United States)

    ... for type 1 von Willebrand disease is called desmopressin. It causes a temporary increase in the von ... injection or by being sniffed into the nose. Desmopressin may also help some people with type 2 ...

  17. Hypophosphatemic osteomalacia in von Recklinghausen neurofibromatosis: Case report and literature review

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    Aman Gupta

    2015-01-01

    Full Text Available Osteomalacia in neurofibromatosis is a rare entity and distinct from more common dysplastic skeletal affections of this disease. As a rule, it is characterized by later onset in adulthood. There is renal phosphate loss with hypophosphatemia and multiple pseudofractures in the typical cases. The hypophosphatemic conditions that interfere in bone mineralization comprise many hereditary or acquired diseases, all of them sharing the same pathophysiological mechanism-reduction in phosphate reabsorption by the renal tubuli. This process leads to chronic hyperphosphaturia and hypophosphatemia, associated with inappropriately normal or low levels of calcitriol, causing rickets in children and osteomalacia in adults.

  18. Parapharyngeal Neurofibroma Originating From The Vagus Nerve In A Patient With Von Recklinghausen′s Disease

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    Das Nillay Kanti

    2004-01-01

    Full Text Available A case of unilateral parapharyngeal neurofibroma occurring in a 25 year old female with von Recklinghausen’s disease is discussed in this report because of the rarity of such a condition. Parapharyngeal neurofibroma usually arises from the vagus nerve and very rarely they are associated with von Recklinghausen’s disease or Neurofibroamatosis type 1. It typically presents with a longâ€"standing history of throat/neck mass with/without neurological deficit. CT scan reveals a moderately enhancing mass, occasionally with central necrosis or cavitations. This, together with spindle shaped cells seen on aspiration cytology, if done, strongly suggests a neurofibroma and excision of the tumor is the treatment of choice. In this patient no sign of malignancy was detected and the two year followâ€"up period was uneventful.

  19. von Willebrand Disease

    Science.gov (United States)

    ... the condition. For example, the school nurse, teacher, daycare provider, coach, or any leader of afterschool activities ... MedlinePlus) Von Willebrand Disease (MedlinePlus) Building 31 31 Center Drive Bethesda, MD 20892 Learn more about getting ...

  20. Estudio clínico e histopatológico de la enfermedad de Von Recklinghausen en Cavidad Oral

    OpenAIRE

    Romero R., Roberto; Prof. Principal, Pas Director del Centro de Extensión Universitaria y Proyección Social.; Gutierrez M., Juan; Prof. Principal, Pas Coordinador del Departamento Académico de Ciencias Básicas.

    2014-01-01

    Twelve Von Reck1inghausen cases were studied finding more incidence within the 31-40 years group, with an incidence of 50% (6 cases). In the intraoral area there was more incidence in the palate, (5 cases 41.7%) but in the type of lesion the peripherical modules were more frequent (8 cases, 66.6%). The peripherical nodules were like nipples, but in case 4 it was like papule of 8 to 10 mm of diameter. Tumors o diffuse masses were detected in 3 cases (25%) being subcutaneous along the nerve. I...

  1. Living with von Willebrand Disease

    Science.gov (United States)

    ... the condition. For example, the school nurse, teacher, daycare provider, coach, or any leader of afterschool activities ... MedlinePlus) Von Willebrand Disease (MedlinePlus) Building 31 31 Center Drive Bethesda, MD 20892 Learn more about getting ...

  2. von Hippel-Lindau disease

    DEFF Research Database (Denmark)

    Binderup, Marie Louise Mølgaard; Gimsing, Steen; Kosteljanetz, Michael

    2013-01-01

    Endolymphatic sac tumours (ELSTs) of the inner ear occur in 16% of patients with the hereditary tumor syndrome von Hippel-Lindau disease (vHL). ELSTs of all sizes can cause irreversible hearing loss which can, however, be prevented through early diagnosis and treatment. We aim to emphasize the ch...... the challenges of prophylactic ELST screening and to explore the role of audiometry in pre-symptomatic ELST screening....

  3. von Willebrand Disease

    Science.gov (United States)

    ... or increase the risk of bleeding. Examples include aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs), clopidogrel, warfarin, or heparin. Any history of liver or kidney disease, blood or bone ...

  4. [Dislocation of the hip in a patient with von Recklinghausen disease].

    Science.gov (United States)

    Del Bosque-Herrero, A; Ezquerra-Herrando, L; Albareda-Albareda, J

    2014-01-01

    Neurofibromatosis type 1 (NF-1) is one of the most common autosomal dominant disorders affecting humans. Patients with NF-1 may present with characteristic orthopaedic manifestations such as scoliosis, congenital pseudoarthrosis and limb hyperttrophy. Dislocation of the hip associated with NF-1 is a rare occurrence. There is a relative paucity of reported cases of pathological hip dislocation in patients with NF-1, with 13 documented cases found in the published literature. Seven dislocations occurred following trivial trauma and 6 cases were deemed atraumatic. We report a case of hip dislocation in a 26 years old male with NF-1 and scoliosis, that was treated successfully by closed reduction and skin traction. Copyright © 2013 SECOT. Published by Elsevier Espana. All rights reserved.

  5. The correlation between clinical, nuclear and histologic findings in a patient with Von Recklinghausen's disease

    Directory of Open Access Journals (Sweden)

    van Diest Paul J

    2007-11-01

    Full Text Available Abstract Background Malignant peripheral nerve sheath tumours (MPNST are known to develop in patients with Neurofibromatosis type I (NF1 resulting in a decreased overall survival. The association between NF1 and the development of such MPNST has been investigated in detail. The biological behaviour however of multiple disseminated neurofibromas in patients with NF1 and the risk factors for malignant transformation remain unknown. Clinical signs are unreliable and additional imaging techniques are therefore required. Of such, positron emission tomography using [18F]-2-fluoro-2-deoxy-D-glucose (18FDG PET is used to detect malignant changes in neurofibromas. Case presentation A case is presented of a patient suffering from NF1 with clinical signs of malignant change and accumulation of 18FDG in multiple neurofibromas. Histopathological examination of 20 lesions however, did not reveal any malignant features. There was no statistically significant relation between18FDG accumulation and malignant change, but rather with pain, size and growth. Conclusion This case adds to the knowledge of the diverse biological behaviour of neurofibromas in patients with NF1

  6. Accumulation of sup 99m Tc-DTPA in neurofibroma of Recklinghausen disease

    Energy Technology Data Exchange (ETDEWEB)

    Funasaki, Hiroki; Tomita, Yasuji; Kondo, Hidemaru; Shiba, Ritsu; Honma, Genki; Sai, Shitake; Urushibara, Nobuo; Murota, Kagehisa; Kawakami, Kenji (Jikei Univ., Tokyo (Japan). School of Medicine)

    1989-07-01

    The ability of scanning with Tc-99m DTPA to detect neurofibroma in Rechlinghausen's disease was evaluated in 8 patients with confirmed neurofibroma, whose ages ranged from 10 to 58 years. They were scanned 2 hours after i.v. injection of Tc-99m DTPA (20 mCi for adults and 10 mCi for children). Tc-99m DTPA was taken up by plexiform neurofibroma in 5 patients and by pachydermatocele in 4 patients. The uptake in plexiform neurofibroma was noticeable. In one patient who had systemic multiple epidermal neurofibromas, uptake of Tc-99m DTPA was not observed. Tc-99m DTPA scanning is considered to be an extremely useful technique in localizing masses in the chest and abdomen of patients with Rechlinghausen's disease. (N.K.).

  7. How Is von Willebrand Disease Treated?

    Science.gov (United States)

    ... the condition. For example, the school nurse, teacher, daycare provider, coach, or any leader of afterschool activities ... MedlinePlus) Von Willebrand Disease (MedlinePlus) Building 31 31 Center Drive Bethesda, MD 20892 Learn more about getting ...

  8. How Is von Willebrand Disease Diagnosed?

    Science.gov (United States)

    ... the condition. For example, the school nurse, teacher, daycare provider, coach, or any leader of afterschool activities ... MedlinePlus) Von Willebrand Disease (MedlinePlus) Building 31 31 Center Drive Bethesda, MD 20892 Learn more about getting ...

  9. Genetics Home Reference: von Willebrand disease

    Science.gov (United States)

    ... Jun;5(6):1165-9. Citation on PubMed Kessler CM. Diagnosis and treatment of von Willebrand disease: ... consult with a qualified healthcare professional . About Selection Criteria for Links Data Files & API Site Map Customer ...

  10. Bilateral papillopathy as a presenting sign of pheochromocytoma associated with von Hippel–Lindau disease

    Directory of Open Access Journals (Sweden)

    Shah V

    2014-03-01

    Full Text Available Veeral Shah, Leonid Zlotcavitch, Angela M Herro, Sander R Dubovy, Zohar Yehoshua, Byron L LamBascom Palmer Eye Institute, University of Miami, Miller School of Medicine, Miami, FL, USAAbstract: A 7-year-old girl presented with decreased vision in both eyes for 1 month. Examination showed visual acuity of 20/50 and 20/60, no afferent pupillary defect, cecocentral scotomas, and bilateral optic disc edema with extensive peripapillary and macular exudates. Magnetic resonance imaging showed multiple cortical and subcortical white matter lesions. Both the laboratory workup and the systemic examination were unrevealing. However, on follow-up, the patient showed episodic elevations of blood pressure as high as 240/160. Further workup revealed elevated urine catecholamines and a right supra-adrenal mass proven to be a pheochromocytoma by histopathologic analysis. The paroxysmal hypertension resolved, and the visual acuity, visual fields, fundus exam, and neuroimaging improved. The patient was lost to follow-up until age 18 when she developed shortness of breath and was found to have multiple pulmonary metastases identified as pheochromocytoma by biopsy. Genetic testing identified a 3p25-26 (c.482 G>A VHL gene chromosomal mutation consistent with von Hippel–Lindau disease genotype. Multiple peripheral retinal vascular dilations and small retinal capillary hemangioblastomas were also found. This case highlights the importance of recognizing the lability of blood pressure often seen with pheochromocytomas, which may mask the underlying cause of hypertensive papillopathy and retinopathy, a diagnosis of low clinical suspicion in the pediatric population. The case also underscores the importance of thorough systemic workup, including genotyping to detect conditions where pheochromocytoma may be the presenting sign of the disease, such as multiple endocrine neoplasia 2A and 2B, von Hippel–Lindau disease, von Recklinghausen disease, tuberous sclerosis, and

  11. Joint bleeding in von Willebrand disease

    NARCIS (Netherlands)

    Galen, K.P.M. van

    2017-01-01

    Von Willebrand disease (VWD) is the most common inherited bleeding disorder occurring in approximately 1/100 people. Until now, joint bleeds did not get much attention in clinical research on VWD, since mucocutaneous bleeding is predominant. However, recurrent joint bleeds lead to arthropathy, the

  12. Revascularization Operation for Moyamoya Disease with Concurrent von Willebrand Disease.

    Science.gov (United States)

    Miki, Kenji; Arimura, Koichi; Nishimura, Ataru; Yoshimoto, Koji; Sayama, Tetsuro; Iihara, Koji

    2017-12-01

    Although extracranial-intracranial (EC-IC) bypass is an effective treatment strategy for symptomatic moyamoya disease, surgeons need to be cautious regarding the possibility of postoperative hemorrhagic complications in patients with a concurrent coagulation disorder. Here, we describe a case of EC-IC bypass for moyamoya disease concurrent with von Willebrand disease type 1. Following perioperative replacement of the von Willebrand factor, the patient showed an uneventful and uncomplicated clinical course. This is the first reported case of EC-IC bypass being performed for moyamoya disease in a patient with concurrent von Willebrand disease. We emphasize the importance of appropriate management with replacement of the von Willebrand factor during the perioperative period to avoid hemorrhagic complications. Copyright © 2017 Elsevier Inc. All rights reserved.

  13. von Willebrand disease phenotype and von Willebrand factor marker genotype in Doberman Pinschers.

    Science.gov (United States)

    Brooks, M B; Erb, H N; Foureman, P A; Ray, K

    2001-03-01

    To define the relationship between clinical expression of a type-1 von Willebrand disease phenotype and genotype at 2 von Willebrand factor marker loci in Doberman Pinschers. 102 client-owned Doberman Pinschers. Dogs were recruited on the basis of plasma von Willebrand factor concentration, clinical history, and pedigree. Blood samples and response to a history questionnaire were obtained for each dog. Plasma von Willebrand factor concentration was measured by use of an ELISA, and genotyping was performed via polymerase chain reaction for 1 intragenic and 1 extragenic von Willebrand factor marker. Amplification product size was determined by use of polyacrylamide gel electrophoresis (intragenic marker) or automated sequence analysis (extragenic marker). Western blots were prepared from a subset of dogs with low plasma von Willebrand factor concentration to evaluate multimer distribution. Strong associations were detected between plasma von Willebrand factor concentration and von Willebrand factor marker genotype. Twenty-five dogs had substantial reduction in plasma von Willebrand factor concentration and multiple hemorrhagic events. All were homozygous for a 157-base-pair intragenic marker allele and homozygous or compound heterozygous for 1 of 4 extragenic marker alleles. These marker genotypes were exclusively detected in dogs with low plasma von Willebrand factor concentration, although some dogs with these genotypes did not have abnormal bleeding. Type-1 von Willebrand disease in Doberman Pinschers is associated with the von Willebrand factor gene locus; however, the expression pattern in this breed appears more complex than that of a simple recessive trait.

  14. Roentgenographic features of neurofibromatosis von Recklinghausen

    Energy Technology Data Exchange (ETDEWEB)

    Galanski, M.; Vogelsang, H.; Cramer, B.M.; Thun, F.; Peters, P.E.

    1983-10-01

    The basic abnormality of neurofibromatosis consists not only of a maldevelopment of the neuroectoderm but also of the mesoderm. Therefore any organ or system of the body may be involved. Following a short clinical review of the central (acoustic) and peripheral type the well known and the more unusual radiographic findings are summarized. They include cranial and intracranial manifestations (orbitosphenoid dysplasia, bone defects of the skull, acoustic neuroma, glioma of the optic nerve and chiasm, meningioma), spinal lesions (scoliosis, vetebral scalloping, meningocele, neuroma, ependymoma), skeletal abnormalities (pseudarthrosis), cardiovascular manifestations, pulmonary fibrosis, tumors of the gastrointestinal and urinary tract and different endocrinopathies. Some of the roentgeologic symptoms are very characteristic and allow definitive diagnosis.

  15. [Craniofacial bone abnormalities in von Recklinghausen neurofibromatosis].

    Science.gov (United States)

    Abassi-Bakir, D; Graiess-Tlili, K; Turky, A; Gharbi, H; Bouzaiene, M; Kraiem, C; Bakir, A

    1995-01-01

    Cases of cranio-facial bone anomalies were observed in 40 cases of neurofibromatosis. The cranio-facial skeletal manifestations are numerous and varied. Radiographic investigation is important to confirm the diagnosis, when neurologic and cutaneous signs are absent. The diagnosis should be easily confirmed by a conventional radiographic study.

  16. Treatment of patients with von Willebrand disease

    Directory of Open Access Journals (Sweden)

    Tuohy E

    2011-04-01

    Full Text Available Emma Tuohy1, Emma Litt1, Raza Alikhan1,21Department of Haematology, University Hospital of Wales, Cardiff, UK; 2Haemophilia and Thrombosis Centre, University Hospital of Wales, Cardiff, UKAbstract: Von Willebrand disease (vWD is the most common hereditary bleeding disorder. The aim of therapy is to correct the dual hemostatic defect, due to defective platelet adhesion-aggregation and abnormal coagulation due to Factor VIII (FVIII deficiency. The choice of treatment depends on a number of factors, including the severity of the bleed, the procedure planned, the subtype and severity of the disease and the age and morbidity of the patient. Desmopressin (DDAVP is the treatment of choice for type 1 vWD as it increases endogenous release of FVIII and von Willebrand factor (vWF and is also used in some subtypes of type 2 vWD. In those patients in whom DDAVP is ineffective or contraindicated, levels can be restored by infusing vWF:FVIII concentrates. The role of antifibrinolytic treatment is an important adjunct to replacement therapy during minor or major surgery involving mucosal surfaces. The dosing and timing of vWF:FVIII concentrates is important depending on the nature of the surgical procedure. The role of secondary prophylaxis needs to be further defined.Keywords: von Willebrand disease, treatment, DDAVP 

  17. Comparative study of CO2- and Er:YAG laser ablation of multiple cutaneous neurofibromas in von Recklinghausen's disease.

    Science.gov (United States)

    Kriechbaumer, Lukas K; Susani, Martin; Kircher, Susanne G; Distelmaier, Klaus; Happak, Wolfgang

    2014-05-01

    With a prevalence of 1 in 3,000 births, neurofibromatosis type 1 (NF1) is one of the most common genetic disorders and is characterized by an uninhibited expansion of neural tissue. Occasionally, severe deformities occur, but frequently considerable cosmetic disfigurement is caused by the development of hundreds of benign cutaneous neurofibromas. The objective of this study was to evaluate the erbium:yttrium-aluminium-garnet (Er:YAG) laser as a therapeutic option for the removal of multiple cutaneous neurofibromas. In this prospective, comparative, in vivo study, 15,580 neurofibromas (44 operations on 21 patients) were removed via electrosurgery, CO2- or Er:YAG laser ablation. In 12 adjacent test areas, we compared the zone of thermal necrosis, the postoperative pain, the time to reepithelialization, the duration of postoperative erythema and the cosmetic outcome of these surgical methods. When compared to electrosurgery and CO2 laser ablation, the Er:YAG laser ablation outperformed the other methods of tumor removal. Rapid healing by second intention as well as the minimal discomfort and scar formation following Er:YAG laser ablation were noted. After 36 months of follow-up, permanent dyspigmentation was rare and hypertrophic scarring was not observed. Er:YAG laser vaporization of multiple cutaneous neurofibromas is a simple and rapid procedure that results in significantly better cosmetic results than CO2 laser treatment or electrosurgery.

  18. Translational medicine advances in von Willebrand disease.

    Science.gov (United States)

    Lillicrap, D

    2013-06-01

    Following the recognition of von Willebrand disease (VWD) in 1926 and the cloning of the gene for von Willebrand factor (VWF) in 1985, significant advances have been made in our fundamental knowledge of both the disease and the protein. Some of this new knowledge has also begun to impact the clinical management of VWD. First, the progressive increase in our understanding of the molecular genetic basis of VWD has resulted in rational applications of molecular testing to complement the current range of phenotypic tests for VWD. These molecular genetic strategies are most effectively directed at the prenatal diagnosis of type 3 VWD and confirmatory testing for types 2B and 2N disease. In contrast, the use of molecular testing to clarify the diagnosis of type 1 VWD is of marginal benefit, at best. In terms of VWD therapies, a new recombinant VWF concentrate has recently completed successful clinical trials and is now awaiting more widespread application. There have even been some preclinical successes with VWF gene transfer although the clinical rationale for this therapeutic strategy needs careful consideration. Much more remains to be learnt about the biology of VWF and further translational advances for the enhancement of VWD care will inevitably be realized. © 2013 International Society on Thrombosis and Haemostasis.

  19. von Willebrand disease and aging : an evolving phenotype

    NARCIS (Netherlands)

    Sanders, Y. V.; Giezenaar, M. A.; Laros-van Gorkom, B. A. P.; Meijer, K.; van der Bom, J. G.; Cnossen, M. H.; Nijziel, M. R.; Ypma, P. F.; Fijnvandraat, K.; Eikenboom, J.; Mauser-Bunschoten, E. P.; Leebeek, F. W. G.

    Background: Because the number of elderly von Willebrand disease (VWD) patients is increasing, the pathophysiology of aging in VWD has become increasingly relevant. Objectives: To assess age-related changes in von Willebrand factor (VWF) and factor VIII (FVIII) levels and to compare age-related

  20. Feocromocitoma asociado a neurofibromatosis de von Recklinghausen Pheochromocytoma associated with von Recklinghausen neurofibromatosis

    Directory of Open Access Journals (Sweden)

    Ramón N. Herrera

    2007-10-01

    Full Text Available El feocromocitoma es un tumor glandular adrenal secretor de hormonas epinefrina y norepinefrina, responsables de regular la frecuencia cardíaca y la presión arterial, entre otras funciones. Este tumor puede ocurrir solo o en combinación con otros desórdenes; los factores genéticos y ambientales juegan un rol clave en su aparición. La neurofibromatosis tipo 1 (NF-1 es un desorden genético frecuente que se hereda en forma autosómica dominante, caracterizado por la formación de neurofibromas (tumores que involucran los nervios tisulares en piel, tejido subcutáneo, nervios craneales y espinales. La NF-1 se diagnostica generalmente con el examen físico. No existe un tratamiento curativo para la NF-1, pero hay modos de tratar algunas de sus complicaciones. La hipertensión arterial en la neurofibromatosis causada por un feocromocitoma es extremadamente rara con una incidencia de menos del 1% en menores de 10 años y en adultos jóvenes. Presentamos el caso clínico de una mujer joven con hipertensión de reciente diagnóstico, con la infrecuente asociación de neurofibromatosis y feocromocitoma. Discutimos los mecanismos fisiopatológicos subyacentes y sus implicancias clínicas.A pheochromo cytoma is an adrenal gland tumor that secretes epinephrine and norepinephrine hormones, and is responsible for regulating heart rate and blood pressure, among other functions. The condition can occur alone or in combination with other disorders, and genetic and environmental factors play a key role. Neurofibromatosis- 1 (NF-1 an inherited "autosomal dominant" disorder is one of the most common genetic disorders, characterized by formation of neurofibromas (tumors involving nerve tissue in the skin, subcutaneous tissue, cranial and spinal root nerves. NF1 generally is diagnosed by physical examination. There is no cure for NF1, but there are ways to treat some of its effects. Neurofibromatosis arterial hypertension caused by pheochromocytoma is extremely rare, less frecuent than 1% in childrens less than 10 years old, and young adults. We present a case of an extremely infrequent association between neurofibromatosis and a pheochromocytoma in a young woman with a newly diagnosed hypertension. We discuss the underlying pathophysiological mechanisms and clinical implications.

  1. VON WILLEBRAND DISEASE: DIAGNOSIS AND TREATMENT

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    Majda Benedik Dolničar

    2004-12-01

    Full Text Available Background. Von Willebrand disease (VWD is a most frequently inborn bleeding disorder caused by quantitative or qualitative defects of von Willebrand factor (VWF. VWF promotes platelet-vessel wall (adhesion and plateletplatelet interaction (aggregation. It is also the carrier for factor VIII (F VIII in plasma. A deficiency of VWF may results in impairment of both, primary and secondary haemostasis. Therefore, patients with VWD can have bleeding symptoms typical fot the defect in primary haemostasis (mucocutaneous haemorrhages. In severe deficiency of VWF there are also haemarthroses and haematomas typical for patients with coagulation defects. Several types and subtypes of VWD have been described. The diagnosis is based on measurements of VWF concentration and activity and F VIII activity in plasma. The tests identifying VWD subtypes are ristocetin induced platelet agglutination (RIPA, multimeric analysis of VWF and measurement of the binding of VWF to F VIII.Conclusions. Due to heterogeneity of VWF defects, the correct diagnosis of types and subtypes is sometimes difficult but is important for appropriate treatment. There are two main therapeutic options for patients with VWD: desmopressin and blood derived concentrates of F VIII/VWF. In certain cases antifibrinolytics and hormones can be suitable treatment. Desmopressin is the treatment of choice in patients with type 1 VWD. It raises endogenous F VIII and VWF and thereby corrects the intrinsic coagulation defect as well as the prolonged bleeding time (BT or closure time (CT-PFA100 in most type 1 VWD patients. In type 3 and in the majority of type 2 patients desmopressin is not effective and it is necessary to use concentrates containing F VIII and VWF. These are always effective in raising of F VIII activity, whereas the BT/CT may not be completely corrected, but the normalisation of the BT/CT is not always necessary.

  2. Doença de von Willebrand e anestesia Enfermedad de von Willebrand y anestesia Von Willebrand's disease and anesthesia

    Directory of Open Access Journals (Sweden)

    Fabiano Timbó Barbosa

    2007-06-01

    íodo perioperatorio. CONTENIDO: Se definieron las características de la enfermedad de von Willebrand en cuanto a las fisiopatologías, la clasificación, al diagnóstico laboratorial, al tratamiento actual y a los cuidados con el manejo del paciente en el período perioperatorio. CONCLUSIONES: La enfermedad de von Willebrand es el disturbio hemorrágico hereditario más común, sin embargo ella está subdiagnosticada por la complejidad de la propia enfermedad. La correcta clasificación del paciente, el uso apropiado de la desmopresina y la transfusión del factor de von Willebrand son medidas fundamentales para la realización del procedimiento anestésico exitoso.BACKGROUND AND OBJECTIVES: von Willebrand's disease is secondary to a mutation on chromosome 12, and is characterized by a qualitative and quantitative deficiency of the von Willebrand's factor. The diversity of the mutations is responsible for several different clinical manifestations, enabling the classification of several types and subtypes. The coagulopathy is manifested basically through a platelet dysfunction associated with a reduction in the serum levels of factor VIII. The objective of this review was to present the perioperative care of patients with von Willebrand's disease. CONTENTS: The physiopathology, classification, laboratorial diagnosis, and current treatment of von Willebrand's disease, as well as the perioperative management of these patients are discussed. CONCLUSIONS: von Willebrand's disease is the most common hereditary coagulopathy, but it is underdiagnosed due to the complexity of the disease itself. The right classification, proper use of desmopressin, and transfusion of von Willebrand's factor are fundamental for a successful anesthesia.

  3. Prophylaxis escalation in severe von Willebrand disease: A prospective study from the von Willebrand Disease Prophylaxis Network

    NARCIS (Netherlands)

    T.C. Abshire (Thomas Calvin); J. Cox-Gill; C.L. Kempton; F.W.G. Leebeek (Frank); M. Carcao (M.); P. Kouides (P.); S. Donfield (S.); E. Berntorp

    2015-01-01

    textabstractBackground: Treatment of mucosal bleeding (epistaxis, gastrointestinal bleeding, and menorrhagia) and joint bleeding remains problematic in clinically severe von Willebrand disease (VWD). Patients are often unresponsive to treatment (e.g. desmopressin or antifibrinolytic therapy) and may

  4. Idiopathic pulmonary hypertension causing acquired von Willebrand disease and menorrhagia.

    Science.gov (United States)

    Sokkary, Nancy A; Dietrich, Jennifer E; Venkateswaran, Lakshmi

    2011-10-01

    Von Willebrand disease (VWD) maybe inherited or acquired; both etiologies can be associated with heavy menstrual bleeding. Pulmonary arterial hypertension may result in acquired VWD due to the destruction of high molecular weight von Willebrand multimers. We report a case of menorrhagia due to acquired VWD in a patient with idiopathic pulmonary hypertension. An adolescent female with known idiopathic pulmonary hypertension developed acquired VWD. Her primary disease necessitates the use of platelet inhibitors and intermittent anticoagulation. At menarche she also developed menorrhagia due to acquired VWD. She is currently controlled with stimate and progesterone-only therapy. VWD in a patient with idiopathic pulmonary hypertension causing menorrhagia. Although VWD and menorrhagia are commonly linked, the treatment and disease process in a patient with idiopathic pulmonary arterial hypertension is incredibly complex. Published by Elsevier Inc.

  5. DEEP VEIN THROMBOSIS IN PATIENT WITH VON WILLEBRAND DISEASE

    Directory of Open Access Journals (Sweden)

    V. A. Elykomov

    2016-01-01

    Full Text Available Objective: to identify the possible factors of thrombogenic risk and ways of its prevention in patients with von Willebrand disease.Case description. Patient X., 42 years old, who suffers from von Willebrand disease type 3 with 5-years of age. Asked on reception to the traumatologist in the polyclinic of the Regional Hospital with pain in the left hip joint. Recommended planned operative treatment in the Altai Regional Clinical Hospital. Preoperative preparation included the infusion of concentrate of von Willebrand factor and coagulation factor VIII. Operation – cement total arthroplasty of the left hip joint. In the postoperative period analgesic treatment, elastic compression of the lower extremities, iron supplements, also conducted infusion of concentrate of von Willebrand factor and coagulation factor VIII for 20 days and thromboprophylactic with dabigatran. On the 3rd day after the operation the patient revealed deep vein thrombosis of the femoral segment (floating clot.Results. The patient was operated for emergency indications in the Department of endovascular surgery – installation of venous cava filter “Volan”. Dabigatran is cancelled, appointed clexane for 3 months. In our clinical example the patient lacked risk factors of pulmonary embolism as obesity, age, smoking, prolonged immobilization, estrogen therapy. Overdose of factor VIII were not observed – the level of factor did not exceed 135 % on transfusions. At the same time, the patient was found polymorphisms in the genes ITGA2, FGB, MTHFR, MTR – heterozygote, MTRR – mutant homozygote, which may indicate the genetic factors of thrombogenic risk. Also a significant risk factor was massive surgical intervention (total hip replacement. Despite preventive measures (elastic compression, thromboprophylactic dabigatran, early activation we cannot to avoid thrombotic complications.Conclusion. This article presents a case demonstrating a thrombotic complication in patients

  6. Screening of Von Willebrand Disease in Iranian Women With Menorrhagia

    Science.gov (United States)

    Rahbar, Nahid; Faranoush, Mohammad; Ghorbani, Raheb; Sadr Alsadat, Bahare

    2015-01-01

    Background: Menorrhagia is a common health problem in women, particularly those with bleeding disorders. Little is known about the course of menorrhagia or other bleeding symptoms in women with the most common congenital bleeding disorder, von Willebrand disease (vWD). Objectives: The aim of this study was to estimate the prevalence of vWD in women with diagnosed menorrhagia. Materials and Methods: In this cross-sectional study, a total of 460 consecutive patients, presenting menorrhagia, were analyzed. The initial screening and confirmation tests for the diagnosis of vWD included determination of prothrombin time (PT), partial thromboplastin time (PTT), bleeding time (BT), fibrinogen, factor VIII, vWF antigen, and vWF activity. A questionnaire was filled for every patient. The data were then analyzed using the SPSS software. Results: Mean age of our patients was 32.5 ± 10.6 years. The level of von Willebrand factor in 22.5% and von Willebrand activity in 19.6% of patients was abnormal. The prevalence of vWD among patients with menorrhagia was 24%. Conclusions: The high prevalence of vWD among our patients was the same as other previous reports, suggesting low awareness about this disease and under diagnosis of mild cases. PMID:25763275

  7. von Willebrand’s disease diagnosis and laboratory issues

    Science.gov (United States)

    CASTAMAN, G.; MONTGOMERY, R. R.; MESCHENGIESER, S. S.; HABERICHTER, S. L.; WOODS, A. I.; LAZZARI, M. A.

    2015-01-01

    Summary In this paper, the recent developments in the diagnosis and laboratory issues of von Willebrand’s disease (VWD) are presented. Dr. Castaman reviews the functional tests available for the diagnosis of VWD and their pathophysiological significance, focusing on which tests are best used in the diagnosis and classification of VWD. Dr Montgomery reviews an emerging issue that is accelerated clearance of von Willebrand factor (VWF) occurring in some variants of VWD. This phenotype can be suspected by the presence of an increased ratio between the VWF propeptide and the VWF antigen. These patients have typically a robust, but short-lived increase of FVIII and VWF after desmopressin. Dr Meschengieser reviews the determinants of bleeding after surgery in patients with VWD, emphasizing the role of bleeding history in predicting this risk. PMID:20590859

  8. Polymorphism in the promoter region of von Willebrand factor gene and von Willebrand disease type 1

    Directory of Open Access Journals (Sweden)

    Daniel Simon

    2003-12-01

    Full Text Available The -1185A/G polymorphism in the 5'-regulatory region of the von Willebrand factor (VWF gene was associated with VWF plasma levels in a normal population. This study was undertaken to evaluate whether there is a relationship between this polymorphism and type 1 von Willebrand disease (VWD, a disorder characterized by a quantitative deficiency of VWF. The association between this polymorphism and plasma VWF levels in normal Brazilian individuals was also analyzed. Control subjects (n = 460 and type 1 VWD patients (n = 41 were studied. Polymerase chain reaction (PCR amplification of the 864-bp VWF promoter region followed by AccII restriction-digestion was used to identify the -1185A/G genotypes. The -1185G allele frequency was 57% in normal individuals and 63% in type 1 VWD patients, this difference was not significant (p = 0.29. No significant association was observed between -1185A/G genotypes and VWF plasma levels in normal individuals, although VWF levels were in the same direction as those reported by another study, with subjects carrying the G allele having the lower levels. These results suggest that -1185A/G polymorphism is not associated with the partial deficiency of VWF in type 1 VWD patients.

  9. CHANGES OF VON WILLEBRAND FACTOR DURING PREGNANCY IN WOMEN WITHOUT AND WITH VON WILLEBRAND DISEASE

    Directory of Open Access Journals (Sweden)

    Giancarlo Castaman

    2013-07-01

    Full Text Available Delivery in von Willebrand disease (VWD represents a significant hemostatic challenge because of the variable pattern of changes observed during pregnancy of von Willebrand factor (VWF  and factor VIII (FVIII, the protein carried by VWF. The wide heterogeneity of phenotypes and of the underlying pathophysiological mechanisms associated with this disorder prompt a careful evaluation of pregnant women with VWD to plan the most appropriate treatment at time of parturition. VWF and FVIII increase significantly during pregnancy in normal women, already within the first trimester, reaching levels by far > 100 U/dL by the time of parturition. In women with VWD, levels at baseline of VWF and FVIII > 30 U/dL are usually associated with a high likelihood to achieve normal levels at the end of pregnancy and specific anti-hemorrhagic prophylaxis is seldom required. Women with basal level < 20 U/dL usually have a poor increase since most of these women carry mutations associated with increased VWF clearance or are compound heterozygous for different VWF mutations which prevent the achievement of satisfactory hemostatic levels. While women with mutations associated with increased clearance show a full, albeit transitory correction of their hemostatic deficiency after desmopressin administration, compound heterozygous need replacement therapy because they do not respond well to this agent. Patients with abnormal VWF:RCo/VWF:Ag ratio at baseline (e.g. < 0.6, typically associated with type 2 VWD, maintain the abnormality throughout pregnancy and VWF:RCo usually does not attain safe levels ³ 50 U/dL. These women require replacement therapy with VWF-FVIII concentrates. Delayed post-partum bleeding may occur when replacement therapy is not continued for some days. Tranexamic acid may be useful at discharge to avoid excessive lochia.

  10. Von Hippel-Lindau disease (vHL)

    DEFF Research Database (Denmark)

    Binderup, Marie Louise Mølgaard; Bisgaard, Søs Marie Luise; Harbud, Vibeke

    2013-01-01

    These clinical guidelines outline the criteria and recommendations for diagnostic and genetic work-up of families suspected of von Hippel-Lindau disease (vHL), as well as recommendations for prophylactic surveillance for vHL patients. The guideline has been composed by the Danish Coordination Group...... for vHL which is comprised of Danish doctors and specialists interested in vHL. The recommendations are based on longstanding clinical experience, Danish original research, and extensive review of the international literature. vHL is a hereditary multi-tumour disease caused by germline mutations...... of the disease, and/or in individuals with a vHL-associated manifestation; i.e. a hemangioblastoma in the retina or the central nervous system, familial or bilateral pheochromocytomas, familial, multiple, or early onset renal cell carcinomas, and in individuals with an endolymphatic sac tumour in the inner ear...

  11. Von Hippel-Lindau disease (vHL)

    DEFF Research Database (Denmark)

    Binderup, Marie Louise Mølgaard; Bisgaard, Søs Marie Luise; Harbud, Vibeke

    2013-01-01

    These clinical guidelines outline the criteria and recommendations for diagnostic and genetic work-up of families suspected of von Hippel-Lindau disease (vHL), as well as recommendations for prophylactic surveillance for vHL patients. The guideline has been composed by the Danish Coordination Group...... for vHL which is comprised of Danish doctors and specialists interested in vHL. The recommendations are based on longstanding clinical experience, Danish original research, and extensive review of the international literature. vHL is a hereditary multi-tumour disease caused by germline mutations...... cell carcinoma), the adrenal glands (pheochromocytoma), the pancreas, as well as in other organs. As many different organs can be affected, several medical specialities often take part in both diagnosis and treatment of manifestations. vHL should be suspected in individuals with a family history...

  12. Craniofacial bone anomalies in Von Recklinghausen`s neurofibromatosis; Les anomalies osseuses cranio-faciales dans la neurofibromatose de Von Recklinghausen

    Energy Technology Data Exchange (ETDEWEB)

    Abassi-Bakir, D.; Gharbi, H.; Kraiem, C.; Graiess-Tlili, K.; Turky, A.; Bouzaiene, M.; Bakir, A. [Centre Hospitalier Universitaire, Sousse (Tunisia)

    1995-12-31

    Cases of cranio-facial bone anomalies were observed in 40 cases of neurofibromatosis. The cranio-facial skeletal manifestations are numerous and varied. Radiographic investigation is important to confirm the diagnosis, when neurologic and cutaneous signs are absent. The diagnosis should be easily confirmed by a conventional radiographic study. (authors). 14 refs., 7 figs., 2 tabs.

  13. Nordic Haemophilia Council's practical guidelines on diagnosis and management of von Willebrand disease

    DEFF Research Database (Denmark)

    Lassila, Riitta; Holme, Pål André; Landorph, Andrea

    2011-01-01

    Von Willebrand disease (VWD) is the most common inherited bleeding disorder characterized by spontaneous or tissue injury-related, mostly mucocutaneous, bleeding events. VWD affects both males and females and is caused by quantitative or qualitative deficiency of Von Willebrand factor. The diagno...

  14. Current and Emerging Options for the Management of Inherited von Willebrand Disease

    NARCIS (Netherlands)

    Heijdra, J.M. (Jessica M.); M.H. Cnossen (Marjon); F.W.G. Leebeek (Frank)

    2017-01-01

    textabstractVon Willebrand disease (VWD) is the most common inherited bleeding disorder with an estimated prevalence of ~1% and clinically relevant bleeding symptoms in approximately 1:10,000 individuals. VWD is caused by a deficiency and/or defect of von Willebrand factor (VWF). The most common

  15. Myomectomy in a case of von Willebrand's disease in a low ...

    African Journals Online (AJOL)

    Von Willebrand's disease (vWD) is an inherited bleeding disorder with an estimated prevalence of 1% in the general population. It is caused by deficiency or dysfunction of von Willebrand's factor. Surgical procedure on patients with vWD is usually associated with increased haemorrhage. Keywords: Clotting Factors ...

  16. Von Willebrand Disease in the Netherlands : from genetic variation to phenotypic variability

    NARCIS (Netherlands)

    Y.V. Sanders (Yvonne)

    2015-01-01

    markdownabstractAbstract Von Willebrand Disease (VWD) is the most common inherited bleeding disorder resulting in mucocutaneous bleeding, like epistaxis, oral cavity bleeding and menorrhagia. VWD is caused by reduced or dysfunctional von Willebrand Factor (VWF). VWF levels are highly variable

  17. Pancreatic cyst development: insights from von Hippel-Lindau disease

    Directory of Open Access Journals (Sweden)

    van Asselt Sophie J

    2013-02-01

    Full Text Available Abstract Pancreatic cysts are a heterogeneous group of lesions, which can be benign or malignant. Due to improved imaging techniques, physicians are more often confronted with pancreatic cysts. Little is known about the origin of pancreatic cysts in general. Von Hippel-Lindau (VHL disease is an atypical ciliopathy and inherited tumor syndrome, caused by a mutation in the VHL tumor suppressor gene encoding the VHL protein (pVHL. VHL patients are prone to develop cysts and neuroendocrine tumors in the pancreas in addition to several other benign and malignant neoplasms. Remarkably, pancreatic cysts occur in approximately 70% of VHL patients, making it the only hereditary tumor syndrome with such a discernible expression of pancreatic cysts. Cellular loss of pVHL due to biallelic mutation can model pancreatic cystogenesis in other organisms, suggesting a causal relationship. Here, we give a comprehensive overview of various pVHL functions, focusing on those that can potentially explain pancreatic cyst development in VHL disease. Based on preclinical studies, cilia loss in ductal cells is probably an important early event in pancreatic cyst development.

  18. The effect of exercise on von Willebrand factor and ADAMTS-13 in individuals with type 1 and type 2B von Willebrand disease

    NARCIS (Netherlands)

    Stakiw, J.; Bowman, M.; Hegadorn, C.; Pruss, C.; Notley, C.; Groot, E.; Lenting, P. J.; Rapson, D.; Lillicrap, D.; James, P.

    Background: The effect of exercise on von Willebrand factor (VWF) and ADAMTS-13 levels in individuals with von Willebrand disease (VWD) has never been reported. Objectives: The aim was to quantify the effect of a standardized exercise protocol on individuals with type 1 and type 2B VWD.

  19. Acquired von Willebrand's disease and hypothyroidism: report of a case presenting with menorrhagia.

    Science.gov (United States)

    Blesing, N. E.; Hambley, H.; McDonald, G. A.

    1990-01-01

    A 17 year old woman presented with severe anaemia due to menorrhagia. On investigation, she was shown to have abnormalities of her haemostatic mechanism consistent with von Willebrand's disease Type I, although there was no family history of this disorder. In addition, she was shown to have severe primary hypothyroidism. On correction of hypothyroidism with oral thyroxine, her coagulation defects returned to normal and menorrhagia ceased. This is consistent with acquired von Willebrand's disease secondary to hypothyroidism. PMID:2217000

  20. Pregnancy-related hemangioblastoma progression and complications in von Hippel-Lindau disease.

    NARCIS (Netherlands)

    Frantzen, C.; Kruizinga, R.C.; Asselt, S.J. van; Zonnenberg, B.A.; Lenders, J.W.M.; Herder, W.W. de; Walenkamp, A.M.; Giles, R.H.; Hes, F.J.; Sluiter, W.J.; Pampus, M.G. van; Links, T.P.

    2012-01-01

    OBJECTIVE: We studied the reciprocal effect of pregnancy and von Hippel-Lindau (VHL) disease by analyzing the influence of pregnancy on VHL disease-related lesions and VHL disease on pregnancy outcome. METHODS: Medical charts and imaging reports from the VHL disease expertise centers in the

  1. Changes in bleeding patterns in von Willebrand disease after institution of long-term replacement therapy : results from the von Willebrand Disease Prophylaxis Network

    NARCIS (Netherlands)

    Holm, Elena; Abshire, Thomas C; Bowen, Joel; Álvarez, M Teresa; Bolton-Maggs, Paula; Carcao, Manuel; Federici, Augusto B; Gill, Joan Cox; Halimeh, Susan; Kempton, Christine; Key, Nigel S; Kouides, Peter; Lail, Alice; Landorph, Andrea; Leebeek, Frank; Makris, Michael; Mannucci, Pier; Mauser-Bunschoten, Eveline P; Nugent, Diane; Valentino, Leonard A; Winikoff, Rochelle; Berntorp, Erik

    Clinically, the leading symptom in von Willebrand disease (VWD) is bleeding, chiefly of mucosal type, for example, epistaxis, gingival, or gastrointestinal bleeding, and menorrhagia. In severe forms of VWD with secondary deficiency of factor VIII, spontaneous joint bleeding, resembling that observed

  2. X-ray diagnostics of thorax diseases; Roentgendiagnostik von Thoraxerkrankungen. Von der Deskription zur Diagnose

    Energy Technology Data Exchange (ETDEWEB)

    Kulke, H.M. [Wuerzburg Univ. (Germany). Universitaetsklinikum

    2013-11-01

    The book on X-ray diagnostics of thorax diseases covers the following issues: diagnostic procedures, inflammatory thorax diseases, malign thorax diseases, heart and large blood vessels, hear insufficiency, pulmonary congestion, pulmonary edema, interstitial pulmonary diseases, intensive care and traumatology.

  3. Detailed von Willebrand factor multimer analysis in patients with von Willebrand disease in the European study, molecular and clinical markers for the diagnosis and management of type 1 von Willebrand disease (MCMDM-1VWD)

    DEFF Research Database (Denmark)

    Budde, U.; Schneppenheim, R.; Eikenboom, J.

    2008-01-01

    BACKGROUND: Type 1 von Willebrand disease (VWD) is a congenital bleeding disorder characterized by a partial quantitative deficiency of plasma von Willebrand factor (VWF) in the absence of structural and/or functional VWF defects. Accurate assessment of the quantity and quality of plasma VWF...... is difficult but is a prerequisite for correct classification. OBJECTIVE: To evaluate the proportion of misclassification of patients historically diagnosed with type 1 VWD using detailed analysis of the VWF multimer structure. Patients and methods: Previously diagnosed type 1 VWD families and healthy controls...

  4. Psychosocial impact of Von Hippel-Lindau disease : levels and sources of distress

    NARCIS (Netherlands)

    Lammens, C. R. M.; Bleiker, E. M. A.; Verhoef, S.; Hes, F. J.; Ausems, M. G. E. M.; Majoor-Krakauer, D.; Sijmons, R. H.; van der Luijt, R. B.; van den Ouweland, A. M. W.; Van Os, Tam; Hoogerbrugge, N.; Garcia, E. B. Gomez; Dommering, C. J.; Gundy, C. M.; Aaronson, N. K.

    Von Hippel-Lindau disease (VHL) is a hereditary tumor susceptibility syndrome, characterized by an increased risk of developing multiple benign and malignant tumors at various sites and ages with limited preventive options. This study evaluates the prevalence of distress among VHL family members and

  5. A systematic review of the effects of hemophilia and von Willebrand disease on arterial trombosis

    NARCIS (Netherlands)

    Biere-Rafi, Sara; Zwiers, M.; Peters, Marjolein; Van Der Meer, Jan; Rosendaal, Frits R; Buller, Harry R; Kamphuisen, Pieter W

    Background: Patients with hemophilia and von Willebrand disease (VWD) may be protected against arterial thrombosis, through a hy-pocoagulable state or atherosclerosis. We performed a systematic review to assess the association between these clotting disorders, arterial thrombosis and the prevalence

  6. von Hippel-Lindau disease: surveillance strategy for endolymphatic sac tumors

    DEFF Research Database (Denmark)

    Poulsen, Marie Louise Mølgaard; Gimsing, Steen; Kosteljanetz, Michael

    2011-01-01

    : Up to 16% of patients with the hereditary von Hippel-Lindau disease develop endolymphatic sac tumors of the inner ear. Early diagnosis and treatment of endolymphatic sac tumors can prevent audiovestibular morbidity, but optimal endolymphatic sac tumor surveillance strategy has yet...... to be determined. We aimed to evaluate endolymphatic sac tumor surveillance to determine the best surveillance strategy....

  7. Health-related quality of life among adult patients with moderate and severe von Willebrand disease.

    NARCIS (Netherlands)

    Wee, E.M. de; Mauser-Bunschoten, E.P.; Bom, J.G. Van Der; Degenaar-Dujardin, M.E.; Eikenboom, H.C.; Fijnvandraat, K.; Goede-Bolder, A. de; Laros, B.A.P.; Meijer, K.; Raat, H.; Leebeek, F.W.

    2010-01-01

    SUMMARY BACKGROUND: von Willebrand Disease (VWD) is the most frequent inherited bleeding disorder. It is unknown how this disorder affects quality of life. OBJECTIVES: This nationwide multicenter cross-sectional study determined health-related quality of life (HR-QoL) in adult patients with moderate

  8. The effect of haemophilia and von Willebrand disease on arterial thrombosis : A systematic review

    NARCIS (Netherlands)

    Biere-Rafi, S.; Zwiers, M.; Peters, M.; van der Meer, J.; Rosendaal, F. R.; Buller, H. R.; Kamphuisen, P. W.

    Background: Patients with haemophilia and von Willebrand disease (VWD) may have a reduced cardiovascular mortality, due to a hypocoagulable state or decreased atherogenesis. We performed a systematic review to assess the association between haemophilia and VWD, and fatal and nonfatal arterial

  9. Gynaecological and obstetric bleeding in moderate and severe von Willebrand disease

    NARCIS (Netherlands)

    de Wee, Eva M.; Knol, H. Marieke; Mauser-Bunschoten, Eveline P.; van der Bom, Johanna G.; Eikenboom, Jeroen C. J.; Fijnvandraat, Karin; de Goede-Bolder, Arja; Laros-van Gorkom, Britta; Ypma, Paula F.; Zweegman, Sonja; Meijer, Karina; Leebeek, Frank W. G.

    2011-01-01

    A nation-wide cross-sectional study was initiated to assess gynaecological and obstetrical symptoms in an unselected cohort of women with moderate and severe von Willebrand disease (VWD) in the Netherlands. A total of 423 women aged >= 16 years were included. Bleeding severity was measured using the

  10. Gynaecological and obstetric bleeding in moderate and severe von Willebrand disease.

    NARCIS (Netherlands)

    Wee, E.M. de; Knol, H.M.; Mauser-Bunschoten, E.P.; Bom, J.G. Van Der; Eikenboom, J.C.; Fijnvandraat, K.; Goede-Bolder, A. de; Laros-van Gorkom, B.A.P.; Ypma, P.F.; Zweegman, S.; Meijer, K.; Leebeek, F.W.

    2011-01-01

    A nation-wide cross-sectional study was initiated to assess gynaecological and obstetrical symptoms in an unselected cohort of women with moderate and severe von Willebrand disease (VWD) in the Netherlands. A total of 423 women aged >/=16 years were included. Bleeding severity was measured using the

  11. Towards improved diagnosis of von Willebrand disease: comparative evaluations of several automated von Willebrand factor antigen and activity assays.

    Science.gov (United States)

    Favaloro, Emmanuel J; Mohammed, Soma

    2014-12-01

    von Willebrand disease (VWD) is reportedly the most common bleeding disorder and arises from deficiency and/or defects of von Willebrand factor (VWF). Laboratory diagnosis and typing has important management implications and requires a wide range of tests, including VWF activity and antigen, and involves differential identification of qualitative vs quantitative defects. We have assessed several VWF antigen and activity assays (collagen binding [VWF:CB], ristocetin cofactor [VWF:RCo] and the new Siemens INNOVANCE assay [VWF:Ac], employing latex particles and gain of function recombinant glycoprotein Ib to facilitate VWF binding and agglutination without need for ristocetin) using different instrumentation, including the new Sysmex CS-5100, with a large sample test set (n=600). We included retrospective plus prospective study designs, and also evaluated desmopressin responsiveness plus differential sensitivity to high molecular weight VWF. VWF:Ag and VWF:RCo results from different methods were respectively largely comparable, although some notable differences were evident, including one high false normal VWF:Ag value (105 U/dL) on a type 3 VWD sample, possibly due to heterophile antibody interference in the latex-based CS-5100 methodology. VWF:Ac was largely comparable to VWF:RCo, but VWF:CB showed discrepant findings to both VWF:RCo and VWF:Ac with some patients, most notably patients with type 2M VWD. (a) VWF:Ag on different platforms are largely interchangeable, as are VWF:RCo on different platforms, except for occasional (some potentially important) differences, and manufacturer recommended methods may otherwise require some assay optimization; (b) VWF:RCo and VWF:Ac are largely interchangeable, except for occasional differences that may also relate to assay design (differing optimizations); (c) VWF:CB provides an additional activity to supplement VWF:RCo or VWF:Ac activity assays, and is not interchangeable with either. Crown Copyright © 2014. Published by

  12. Diagnostic Differentiation of von Willebrand Disease Types 1 and 2 by von Willebrand Factor Multimer Analysis and DDAVP Challenge Test.

    Science.gov (United States)

    Michiels, Jan Jacques; Smejkal, Petr; Penka, Miroslav; Batorova, Angelika; Pricangova, Tatiana; Budde, Ulrich; Vangenechten, Inge; Gadisseur, Alain

    2017-09-01

    The European Clinical Laboratory and Molecular (ECLM) classification of von Willebrand disease (vWD) is based on the splitting approach which uses sensitive and specific von Willebrand factor (vWF) assays with regard to the updated molecular data on structure and function of vWF gene and protein defects. A complete set of FVIII:C and vWF ristocetine cofactor, collagen binding, and antigen, vWF multimeric analysis in low- and medium-resolution gels, and responses to desmopressin (DDAVP) of FVIII:C and vWF parameters are mandatory. The ECLM classification distinguishes recessive types 1 and 3 vWD from recessive vWD 2C due to mutations in the D1 and D2 domains and vWD 2N due to mutations in the D'-FVIII-binding domain of vWF. The ECLM classification differentiates between mild vWD type 1 with variable penetrance of bleedings from symptomatic dominant type 1 vWD secretion defect and/or clearance defect with normal vWF multimers versus vWD 1M and 2M with normal or smeary vWF multimers in low- and medium-resolution gels. High-quality multimeric analysis of vWF in medium-resolution gels based on a DDAVP challenge test clearly delineates and distinguishes each of the dominant type 2 vWDs 1/2E, 2M, 2B, 2A, and 2D caused by vWF gene mutations in the D3 multimerization domain, loss or gain-of-function mutations in the glycoprotein Ib receptor A1 domain, gene mutations in the A2 proteolytic domain, and the C-terminal dimerization domain, respectively.

  13. Pseudo (Platelet-type von Willebrand disease in pregnancy: a case report

    Directory of Open Access Journals (Sweden)

    Grover Neetu

    2013-01-01

    Full Text Available Abstract Background Pseudo (platelet-type-von Willebrand disease is a rare autosomal dominant bleeding disorder caused by an abnormal function of the glycoprotein lb protein; the receptor for von Willebrand factor. This leads to an increased removal of VWF multimers from the circulation as well as platelets and this results in a bleeding diathesis. Worldwide, less than 50 patients are reported with platelet type von Willebrand disease (PT-VWD. Case presentation We describe the management of platelet type von Willebrand disease in pregnancy of a 26 year old Caucasian primigravida. The initial diagnosis was made earlier following a significant haemorrhage post tonsillectomy several years prior to pregnancy. The patient was managed under a multidisciplinary team which included obstetricians, haematologists, anaesthetists and neonatologists. Care plans were made for the ante- natal, intra-partum and post-partum periods in partnership with the patient. The patient’s platelet count levels dropped significantly during the antenatal period. This necessitated the active exclusion of other causes of thrombocytopenia in pregnancy. A vaginal delivery was desired and plans were made for induction of labour at 38 weeks of gestation with platelet cover in view of the progressive fall of the platelet count. The patient however went into spontaneous labour on the day of induction. She was transfused two units of platelets before delivery. She had an unassisted vaginal delivery of a healthy baby. The successful antenatal counselling has encouraged the diagnosis of the same condition in her mother and sister. We found this to be a particularly interesting case as well as challenging to manage due to its rarity. Psuedo von Willebrand disease in pregnancy can be confused with a number of other differential diagnoses, such as gestational thrombocutopenia, idiopathatic thrombocytopenia, thrombotic thrombocytopenic purpura and pre-eclampsia; all need consideration

  14. Pseudo (platelet-type) von Willebrand disease in pregnancy: a case report.

    Science.gov (United States)

    Grover, Neetu; Boama, Vincent; Chou, Munazzah Rifat

    2013-01-17

    Pseudo (platelet-type)-von Willebrand disease is a rare autosomal dominant bleeding disorder caused by an abnormal function of the glycoprotein lb protein; the receptor for von Willebrand factor. This leads to an increased removal of VWF multimers from the circulation as well as platelets and this results in a bleeding diathesis. Worldwide, less than 50 patients are reported with platelet type von Willebrand disease (PT-VWD). We describe the management of platelet type von Willebrand disease in pregnancy of a 26 year old Caucasian primigravida. The initial diagnosis was made earlier following a significant haemorrhage post tonsillectomy several years prior to pregnancy. The patient was managed under a multidisciplinary team which included obstetricians, haematologists, anaesthetists and neonatologists. Care plans were made for the ante- natal, intra-partum and post-partum periods in partnership with the patient. The patient's platelet count levels dropped significantly during the antenatal period. This necessitated the active exclusion of other causes of thrombocytopenia in pregnancy. A vaginal delivery was desired and plans were made for induction of labour at 38 weeks of gestation with platelet cover in view of the progressive fall of the platelet count. The patient however went into spontaneous labour on the day of induction. She was transfused two units of platelets before delivery. She had an unassisted vaginal delivery of a healthy baby. The successful antenatal counselling has encouraged the diagnosis of the same condition in her mother and sister. We found this to be a particularly interesting case as well as challenging to manage due to its rarity. Psuedo von Willebrand disease in pregnancy can be confused with a number of other differential diagnoses, such as gestational thrombocutopenia, idiopathatic thrombocytopenia, thrombotic thrombocytopenic purpura and pre-eclampsia; all need consideration during investigations even in a case such as this where the

  15. Inheritance of von Willebrand's disease in a colony of Doberman Pinschers.

    Science.gov (United States)

    Riehl, J; Okura, M; Mignot, E; Nishino, S

    2000-02-01

    To determine the mode of inheritance of von Willebrand's disease (vWD) and perform linkage analysis between vWD and coat color or narcolepsy in a colony of Doberman Pinschers. 159 Doberman Pinschers. von Willebrand factor antigen (vWF:Ag) concentration was measured by use of ELISA, and results were used to classify dogs as having low ( 65%) vWF:Ag concentration, compared with results of analysis of standard pooled plasma. Buccal bleeding time was measured, and mode of inheritance of vWD was assessed by pedigree analysis. von Willebrand's disease was transmitted as a single autosomal gene defect. Results suggested that 27.04% of dogs were homozygous for vWD, 62.26% were heterozygous, and 10.69% did not have the defect. Most homozygous and some heterozygous dogs had prolonged bleeding times. Dogs with diluted coat colors (blue and fawn) were significantly overrepresented in the homozygous group, compared with black and red dogs, but a significant link between vWD and coat color was not detected. von Willebrand's disease is transmitted as an autosomal dominant trait with variable penetrance; most dogs in this colony (89.3%) were carriers of vWD. Homozygosity for vWD is not likely to be lethal. Some heterozygous dogs have prolonged bleeding times. An association between diluted coat colors and vWD may exist.

  16. Clinical and laboratory diagnosis of von Willebrand disease : A synopsis of the 2008 NHLBI/NIH guidelines

    NARCIS (Netherlands)

    Nichols, William L.; Rick, Margaret E.; Ortel, Thomas L.; Montgomery, Robert R.; Sadler, J. Evan; Yawn, Barbara P.; James, Andra H.; Hultin, Mae B.; Manco-Johnson, Marilyn J.; Weinstein, Mark

    Von Willebrand factor (VWF) mediates blood platelet adhesion and accumulation at sites of blood vessel injury, and also carries coagulation factor VIII (FVIII) that is important for generating procoagulant activity. Von Willebrand disease (VWD), the most common inherited bleeding disorder, affects

  17. Uptake of genetic testing and long-term tumor surveillance in von Hippel-Lindau disease

    OpenAIRE

    Chávez Mireya; Rodríguez Yaneth; Sosa Ana-Luisa; Yescas Petra; Familiar Itziar; De Biase Irene; Ochoa Adriana; Alonso Elisa; Rasmussen Astrid; López-López Marisol; Bidichandani Sanjay I

    2010-01-01

    Abstract Background von Hippel-Lindau (VHL) disease is a hereditary cancer syndrome caused by germline mutations in the VHL gene. Patients have significant morbidity and mortality secondary to vascular tumors. Disease management is centered on tumor surveillance that allows early detection and treatment. Presymptomatic genetic testing is therefore recommended, including in at-risk children. Methods We tested 17 families (n = 109 individuals) for VHL mutations including 43 children under the a...

  18. Management of type 2b von Willebrand disease in the neonatal period.

    Science.gov (United States)

    Proud, Lindsay; Ritchey, A Kim

    2017-01-01

    Von Willebrand disease (VWD) is the most common inherited bleeding disorder, affecting one in 1,000 people. Type 2b VWD is a less common subtype caused by a gain-of-function mutation in von Willebrand factor (VWF) that leads to the formation of large, ineffective VWF-platelet multimers in circulation. This unique pathophysiology creates diagnostic and treatment dilemmas. There is limited information on the management of type 2b VWD in the neonatal period. This report describes the management of a neonate with type 2b VWD with an emphasis on the added benefit of concomitant platelet transfusion and factor replacement therapy over factor replacement therapy alone. © 2016 Wiley Periodicals, Inc.

  19. A case of von Hippel-Lindau disease with exudative maculopathy

    Directory of Open Access Journals (Sweden)

    Basel T Ba′arah

    2009-01-01

    Full Text Available Von Hippel-Lindau (VHL disease is a rare multisystem familial tumor syndrome of autosomal dominant inheritance. Hallmark lesions include retinal, cerebellum and spinal cord hemangioblastomas, renal cell carcinomas, adrenal pheochromocytomas, angiomatous or cystic lesions of the kidneys, pancreas, and epididymis. We report a case of VHL disease in a 26-year-old patient who presented with exudative macular edema. Ocular and systemic studies revealed the presence of retinal and central nervous system hemangioblastomas, adrenal pheochromocytoma, multiple pancreatic, and kidney cysts. The retinal angiomas were successfully treated with argon laser photocoagulation and cryotherapy.

  20. Ga-68 Somatostatin Receptor PET/CT in von Hippel-Lindau Disease

    Energy Technology Data Exchange (ETDEWEB)

    Oh, Jong-Ryool; Min, Jung-Joon [Chonnam National Univ. Hwasun Hospital, Hwasun (Korea, Republic of); Kulkarui, Harshad; Carreras, Cecilia; Schalch, Georg; Baum, Richard P. [Nuclear Medicine and Center for PET/CT, Zentralk Bad Berka, Bad Verka (Germany)

    2012-06-15

    Von Hippel-Lindau (VHL) disease is a dominantly inherited familial cancer syndrome with a variety of benign and malignant tumors such as retinal and central nervous system hemangioblastomas, endolymphatic sac tumors, renalcysts and tumors, pancreatic cysts and tumors, pheochromo-cytomas, and epididymal cystadenomas. Cross-sectional mo-dalities (computed tomography and magnetic resonance imaging) as well as ultrasound play a major role in the initial evaluation and follow-up of the various manifestations of VHL disease. Ga-68-labeled somatostatin receptor analogs already have a significant role in the diagnosis, staging, and therapy management of neuroendocrine neoplasms and neural crest tumors. Herein, we report a case presenting a variety of malignancies in VHL and showing the usefulness of Ga-68 somatostatin receptor PET/CT as a one-stop-shop imaging modality in the management of VHL disease.

  1. Prevalence of von Willebrand disease in women with iron deficiency anaemia and menorrhagia in Taiwan.

    Science.gov (United States)

    Chen, Y-C; Chao, T-Y; Cheng, S-N; Hu, S-H; Liu, J-Y

    2008-07-01

    Iron deficiency anaemia (IDA) is a frequently encountered disease, which can be attributed to menorrhagia. Most female patients with von Willebrand disease (VWD) have menorrhagia. The aim of this study was to investigate the prevalence of VWD in women with both IDA and menorrhagia in Taiwan. From January to December 2005 and November 2006 to January 2007, 56 consecutive patients with both IDA and menorrhagia were enrolled in this study. Their median age was 41 years (range 18-53). IDA was diagnosed by anaemia plus either low ferritin or transferrin saturation. Menorrhagia was evaluated by patient's menses history. Both von Willebrand factor antigen (VWF:Ag) and ristocetin cofactor activity (VWF:RCo) were measured for each patient. Bleeding time (BT) and platelet function analyser (PFA)-100 assay were determined as ancillary tests. The VWD diagnosis was established if: (i) both VWF:Ag (menorrhagia might develop IDA at younger age (34.3 vs. 39.7, P = 0.09) and had more IDA recurrence (75% vs. 16%, P = 0.03) than those patients without VWD. Of the eight VWD patients with VWF multimer analyses, all were revealed to have type I VWD. Our study demonstrates that VWD was not uncommon in women with both IDA and menorrhagia in Taiwan.

  2. Pathology of the Nervous System in Von Hippel-Lindau Disease

    Directory of Open Access Journals (Sweden)

    Alexander O. Vortmeyer

    2015-06-01

    Full Text Available Von Hippel-Lindau (VHL disease is a tumor syndrome that frequently involves the central nervous system (CNS. It is caused by germline mutation of the VHL gene. Subsequent VHL inactivation in selected cells is followed by numerous well-characterized molecular consequences, in particular, activation and stabilization of hypoxia-inducible factors HIF1 and HIF2. The link between VHL gene inactivation and tumorigenesis remains poorly understood. Hemangioblastomas are the most common manifestation in the CNS; however, CNS invasion by VHL disease-associated endolymphatic sac tumors or metastatic renal cancer also occur, and their differentiation from primary hemangioblastoma may be challenging. Finally, in this review, we present recent morphologic insights on the developmental concept of VHL tumorigenesis which is best explained by pathologic persistence of temporary embryonic progenitor cells. 

  3. Patient-specific factors influence somatic variation patterns in von Hippel-Lindau disease renal tumours.

    Science.gov (United States)

    Fei, Suzanne S; Mitchell, Asia D; Heskett, Michael B; Vocke, Cathy D; Ricketts, Christopher J; Peto, Myron; Wang, Nicholas J; Sönmez, Kemal; Linehan, W Marston; Spellman, Paul T

    2016-05-13

    Cancer development is presumed to be an evolutionary process that is influenced by genetic background and environment. In laboratory animals, genetics and environment are variables that can largely be held constant. In humans, it is possible to compare independent tumours that have developed in the same patient, effectively constraining genetic and environmental variation and leaving only stochastic processes. Patients affected with von Hippel-Lindau disease are at risk of developing multiple independent clear cell renal carcinomas. Here we perform whole-genome sequencing on 40 tumours from six von Hippel-Lindau patients. We confirm that the tumours are clonally independent, having distinct somatic single-nucleotide variants. Although tumours from the same patient show many differences, within-patient patterns are discernible. Single-nucleotide substitution type rates are significantly different between patients and show biases in trinucleotide mutation context. We also observe biases in chromosome copy number aberrations. These results show that genetic background and/or environment can influence the types of mutations that occur.

  4. Diagnosis and Treatment of von Willebrand Disease and Rare Bleeding Disorders

    Directory of Open Access Journals (Sweden)

    Giancarlo Castaman

    2017-04-01

    Full Text Available Along with haemophilia A and B, von Willebrand disease (VWD and rare bleeding disorders (RBDs cover all inherited bleeding disorders of coagulation. Bleeding tendency, which can range from extremely severe to mild, is the common symptom. VWD, due to a deficiency and/or abnormality of von Willebrand factor (VWF, represents the most frequent bleeding disorder, mostly inherited as an autosomal dominant trait. The diagnosis may be difficult, based on a bleeding history and different diagnostic assays, which evaluate the pleiotropic functions of VWF. Different treatment options are available for optimal management of bleeding and their prevention, and long-term outcomes are generally good. RBDs are autosomal recessive disorders caused by a deficiency of any other clotting factor, apart from factor XII, and cover roughly 5% of all bleeding disorders. The prevalence of the severe forms can range from 1 case in 500,000 up to 1 in 2–3 million, according to the defect. Diagnosis is based on bleeding history, coagulation screening tests and specific factor assays. A crucial problem in RBDs diagnosis is represented by the non-linear relationship between clinical bleeding severity and residual clotting levels; genetic diagnosis may help in understanding the phenotype. Replacement therapies are differently available for patients with RBDs, allowing the successful treatment of the vast majority of bleeding symptoms.

  5. Long-term impact of joint bleeds in von Willebrand disease: a nested case-control study

    NARCIS (Netherlands)

    Galen, K.P. van; Kleijn, P. de; Foppen, W.; Eikenboom, J.; Meijer, K.; Schutgens, R.E.; Fischer, K.; Cnossen, M.H.; Meris, J. de; Fijnvandraat, K.; Bom, J.G. Van Der; Laros-van Gorkom, B.A.P.; Leebeek, F.W.; Mauser-Bunschoten, E.P.

    2017-01-01

    Patients with severe von Willebrand disease (VWD) may develop arthropathy after joint bleeds. Information on its prevalence and severity is limited. We aimed to assess the occurrence and severity of arthropathy in VWD and its impact on daily life. VWD patients with and without verified joint bleeds

  6. Long-term impact of joint bleeds in von Willebrand disease : A nested case-control study

    NARCIS (Netherlands)

    van Galen, Karin P.M.; de Kleijn, Piet; Foppen, Wouter; Eikenboom, Jeroen H C; Meijer, Karina; Schutgens, Roger E.G.; Fischer, Kathelijn; Cnossen, Marjon H; de Meris, Joke; Fijnvandraat, Karin; van der Bom, Johanna G; Laros-van Gorkom, Britta A P; Leebeek, Frank W G; Mauser-Bunschoten, Eveline P.

    2017-01-01

    Patients with severe von Willebrand disease (VWD) may develop arthropathy after joint bleeds. Information on its prevalence and severity is limited. We aimed to assess the occurrence and severity of arthropathy in VWD and its impact on daily life. VWD patients with and without verified joint bleeds

  7. Long-term impact of joint bleeds in von Willebrand disease : A nested case-control study

    NARCIS (Netherlands)

    van Galen, Karin P. M.; de Kleijn, Piet; Foppen, Wouter; Eikenboom, Jeroen; Meijer, Karina; Schutgens, Roger E. G.; Fischer, Kathelijn; Cnossen, Marjon H.; de Meris, Joke; Fijnvandraat, Karin; van der Bom, Johanna G.; Laros-van Gorkom, Britta A. P.; Leebeek, Frank W. G.; Mauser-Bunschoten, Eveline P.

    Patients with severe von Willebrand disease (VWD) may develop arthropathy after joint bleeds. Information on its prevalence and severity is limited. We aimed to assess the occurrence and severity of arthropathy in VWD and its impact on daily life. VWD patients with and without verified joint bleeds

  8. Long-term impact of joint bleeds in von Willebrand disease: A nested case-control study

    NARCIS (Netherlands)

    K.P.M. van Galen; P. de Kleijn; W. Foppen (Wouter); J.C.J. Eikenboom (Jeroen); K. Meijer; R. Schutgens (Roger); K. Fischer (Kathelijn); M.H. Cnossen (Marjon); J. de Meris (J.); K. Fijnvandraat; J.G. van der Bom (Anske); B.A.P. Laros-Van Gorkom (Britta); F.W.G. Leebeek (Frank); E.P. Mauser-Bunschoten (Eveline)

    2017-01-01

    textabstractPatients with severe von Willebrand disease (VWD) may develop arthropathy after joint bleeds. Information on its prevalence and severity is limited. We aimed to assess the occurrence and severity of arthropathy in VWD and its impact on daily life. VWD patients with and without verified

  9. Characterization of endolymphatic sac tumors and von Hippel-Lindau disease in the International Endolymphatic Sac Tumor Registry

    NARCIS (Netherlands)

    Bausch, Birke; Wellner, Ulrich; Peyre, Mathieu; Boedeker, Carsten C.; Hes, Frederik J.; Anglani, Mariagiulia; de Campos, Jose M.; Kanno, Hiroshi; Maher, Eamonn R.; Krauss, Tobias; Sanso, Gabriela; Barontini, Marta; Letizia, Claudio; Hader, Claudia; Schiavi, Francesca; Zanoletti, Elisabetta; Suarez, Carlos; Offergeld, Christian; Malinoc, Angelica; Zschiedrich, Stefan; Glasker, Sven; Bobin, Serge; Sterkers, Olivier; Huy, Patrice Tran Ba; Giraud, Sophie; Links, Thera; Eng, Charis; Opocher, Giuseppe; Richard, Stephane; Neumann, Hartmut P. H.

    Background. Endolymphatic sac tumors (ELSTs) are, with a prevalence of up to 16%, a component of von Hippel-Lindau (VHL) disease. Data from international registries regarding heritable fraction and characteristics, germline VHL mutation frequency, and prevalence are lacking. Methods. Systematic

  10. Levonorgestrel intrauterine system as a treatment option for severe menorrhagia in adolescent with type III von Willebrand disease.

    Science.gov (United States)

    Silva, Carla Donato; Geraldes, Fernanda; Silva, Isabel Santos

    2013-04-30

    The authors describe a case of an adolescent with type III von Willebrand disease and severe menorrhagia since menarche. Antifibrinolytic, hormonal (estroprogestative pill in high doses, etonogestrel implant and gonadotropin-releasing hormone agonist goserelin) and Von Willebrand Factor/Factor VIII replacement therapies were prescribed to the patient, but symptomatic control was only obtained with high doses of VWF/FVIII twice a week. In March 2012, a levonorgestrel intrauterine system was inserted in a 14-year-old. At present, the patient is asymptomatic without regular prophylaxis (VWF/FVIII replacement therapy) and has had a remarkable improvement in her quality of life.

  11. Survival and causes of death in patients with von Hippel-Lindau disease

    DEFF Research Database (Denmark)

    Binderup, Marie Louise Mølgaard; Jensen, Annette Møller; Budtz-Jørgensen, Esben

    2017-01-01

    . Surveillance is especially beneficial for truncating mutation carriers, who have the greatest RCC and central nervous system (CNS) hemangioblastoma risk.  Conclusions: vHL survival has improved over time and has become closer to that of siblings without vHL and the general population. Even though the risk of v......Background: Historically, the survival of patients with von Hippel-Lindau disease (vHL) has been poorer than that of the general population. We aimed to determine whether the survival of VHL mutation carriers and their risk of vHL-related death has changed over time and how it has been affected...... by sex, genotype and surveillance attendance.  Methods: In a retrospective cohort study, we included all known Danish vHL families with a VHL mutation. We assessed the survival and causes of death for 143 VHL mutation carriers using Cox regression models and compared vHL survival with that of 137...

  12. Pulmonary hypertension secondary to hyperviscosity in a patient with rheumatoid arthritis and acquired von Willebrand disease: a case report.

    Science.gov (United States)

    Hernández-Gilsoul, Thierry; Atisha-Fregoso, Yemil; Vargas-Ruíz, Angel G; Rivero-Sigarroa, Eduardo; Dominguez-Cherit, Guillermo; Namendys-Silva, Silvio A

    2013-10-02

    Acquired von Willebrand disease is initiated by autoantibodies and hyperviscosity syndrome caused by a massive polyclonal hypergammaglobulinemia. Acquired von Willebrand disease associated with autoimmune disease in addition to pulmonary hypertension during emergency room presentation is a rare condition. To the best of our knowledge, this is the second case reported in the literature treated with success; the first one was reported in 1987. A 28-year-old mestizo man with a 3-year history of inflammatory arthritis was admitted to our hospital. An overlap of rheumatoid arthritis with systemic lupus erythematosus was suspected; therefore methotrexate was initiated, and later changed to leflunomide because of liver toxicity. Prothrombin time, international normalized ratio and activated partial thromboplastin times were normal (11/10.4 seconds; 1.2; 31.1/26.9 seconds, respectively), von Willebrand factor activity was observed with low ristocetin cofactor at 33.6UI/dL, high von Willebrand factor antigen >200UI/dL, and a low von Willebrand factor: ristocetin cofactor to von Willebrand factor antigen ratio. He was admitted to the emergency room with a 24-hour evolution of progressive dyspnea, cough, thoracic pain, and palpitations, 104 beats/min, 60/40 mmHg, temperature of 38°C, pulse oximetric saturation 88% and 30 breaths/minute. Cold, pale and mottled skin was also observed. He was then transferred to the intensive care unit. The placement of a pulmonary artery catheter was made. The initial patterns showed a precapillary pulmonary hypertension; acute pulmonary embolism was the first choice for diagnosis. Pulmonary angiography was conducted, and when no clot was discovered, pulmonary artery hypertension associated with connective tissue disease was considered. Serum protein electrophoresis confirmed the presence of a massive polyclonal hypergammaglobulinemia, and no paraproteinemia or monoclonal cell population was found from the electrophoretic pattern of the

  13. 68Ga-DOTA-NOC PET/CT detects somatostatin receptors expression in von hippel-lindau cerebellar disease.

    Science.gov (United States)

    Ambrosini, Valentina; Campana, Davide; Allegri, Vincenzo; Opocher, Giuseppe; Fanti, Stefano

    2011-01-01

    A case of Von-Hippel Lindau (VHL) disease has been studied using 68Ga-DOTA-NOC PET/CT. PET/CT demonstrated the presence of somatostatin receptors within 2 focal areas in the cerebellum corresponding to the lesions detected by MRI. Considering the heterogeneous lesions localizations in VHL disease, PET/CT may be a useful imaging modality for diagnosing lesions of central nervous system and neuroendocrine lesions and for direct demonstration of somatostatin receptors for targeted treatment.

  14. Postpartum Hemorrhage in Women with Von Willebrand Disease - A Retrospective Observational Study.

    Directory of Open Access Journals (Sweden)

    Igor Govorov

    Full Text Available von Willebrand disease (VWD is a hereditary bleeding disorder, caused by a deficiency in the levels and/or function of von Willebrand factor (VWF. Women with VWD appear to be at increased risk of experiencing postpartum hemorrhage (PPH, though the levels of VWF increase during pregnancy. There is limited knowledge of how PPH is associated with the subtype of VWD, plasma levels of other coagulations factors than VWF and given hemostatic treatment.The aims were to investigate the incidence of PPH in women with VWD and to analyse the correlation between PPH and: (1 type of VWD, (2 laboratory monitoring of VWF and FVIII and (3 hemostatic drug treatment.This was a retrospective observational study. The study participants (n = 34 were recruited from the Coagulation Unit, Karolinska University hospital. Fifty-nine deliveries, which occurred in 14 different obstetrics units (years 1995-2012 were included in the study.The incidence of primary PPH was 44%, severe primary PPH 20% and secondary PPH 12%. VWD type 3 was associated with a higher risk of experiencing severe primary PPH compared to other subtypes. FVIII:C in pregnancy was inversely correlated to blood loss during delivery. There was a significantly higher incidence of secondary PPH when the VWD diagnosis was unknown at time of delivery.The women with VWD are at higher risk of PPH, especially those with type 3 VWD or when diagnosis is unknown prior to delivery. Identification of pregnant women with undiagnosed VWD may be of importance in order to prevent PPH.

  15. Genetic heterogeneity in a large cohort of Indian type 3 von Willebrand disease patients.

    Directory of Open Access Journals (Sweden)

    Priyanka Kasatkar

    Full Text Available Though von Willebrand disease (VWD is a common coagulation disorder, due to the complexity of the molecular analysis of von Willebrand factor gene (VWF, not many reports are available from this country. Large size of the gene, heterogeneous nature of mutations and presence of a highly homologous pseudogene region are the major impediments in the genetic diagnosis of VWD. The study is aimed at unravelling the molecular pathology in a large series of VWD patients from India using an effective strategy.We evaluated 85 unrelated Indian type 3 VWD families to identify the molecular defects using a combination of techniques i.e. PCR-RFLP, direct DNA sequencing and multiple ligation probe amplification (MLPA.Mutations could be characterized in 77 unrelated index cases (ICs. 59 different mutations i.e. nonsense 20 (33.9%, missense 13 (22%, splice site 4 (6.8%, gene conversions 6 (10.2%, insertions 2 (3.4%, duplication 1 (1.7%, small deletions 10 (17% and large deletions 3 (5.1% were identified, of which 34 were novel. Two common mutations i.e. p.R1779* and p.L970del were identified in our population with founder effect. Development of alloantibodies to VWF was seen in two patients, one with nonsense mutation (p.R2434* and the other had a large deletion spanning exons 16-52.The molecular pathology of a large cohort of Indian VWD patients could be identified using a combination of techniques. A wide heterogeneity was observed in the nature of mutations in Indian VWD patients.

  16. Technological advances in diagnostic testing for von Willebrand disease: new approaches and challenges.

    Science.gov (United States)

    Hayward, C P M; Moffat, K A; Graf, L

    2014-06-01

    Diagnostic tests for von Willebrand disease (VWD) are important for the assessment of VWD, which is a commonly encountered bleeding disorder worldwide. Technical innovations have been applied to improve the precision and lower limit of detection of von Willebrand factor (VWF) assays, including the ristocetin cofactor activity assay (VWF:RCo) that uses the antibiotic ristocetin to induce plasma VWF binding to glycoprotein (GP) IbIXV on target platelets. VWF-collagen-binding assays, depending on the type of collagen used, can improve the detection of forms of VWD with high molecular weight VWF multimer loss, although the best method is debatable. A number of innovations have been applied to VWF:RCo (which is commonly performed on an aggregometer), including replacing the target platelets with immobilized GPIbα, and quantification by an enzyme-linked immunosorbent assay (ELISA), immunoturbidimetric, or chemiluminescent end-point. Some common polymorphisms in the VWF gene that do not cause bleeding are associated with falsely low VWF activity by ristocetin-dependent methods. To overcome the need for ristocetin, some new VWF activity assays use gain-of-function GPIbα mutants that bind VWF without the need for ristocetin, with an improved precision and lower limit of detection than measuring VWF:RCo by aggregometry. ELISA of VWF binding to mutated GPIbα shows promise as a method to identify gain-of-function defects from type 2B VWD. The performance characteristics of many new VWF activity assays suggest that the detection of VWD, and monitoring of VWD therapy, by clinical laboratories could be improved through adopting newer generation VWF assays. © 2014 John Wiley & Sons Ltd.

  17. From Wilhelm von Humboldt to Hitler-are prominent people more prone to have Parkinson's disease?

    Science.gov (United States)

    Horowski; Horowski; Calne; Calne

    2000-10-01

    We describe Parkinsonism in prominent people, where Wilhelm von Humboldt and Adolf Hitler provide just two spectacular, opposing examples. In both of them, there is little if any evidence that the disease did influence their life ambitions, methods of achieving them or cognitive function in general. Thus, Hitler's Parkinsonism should remain a 'footnote' to history, and historians should acknowledge that in his last years, his trembling, his curbed posture, his slow walking, mask-like face and low voice did not indicate remorse, fear or depression as a consequence of his crimes, but were mere expressions of his disease which, until the end, had no impact on his intellectual skills and methods. The apparently higher incidence of Parkinsonism in prominent people may be just due to their higher visibility, or a consequence of disease-related personality traits (e.g. ambition, perfectionism, rigidity) which may contribute to becoming, e.g., a prominent authoritarian person. Perhaps even some early behaviour pattern (such as repressed emotions or acting in public-which could even increase the risk of some infection) contributes to a greater vulnerability for developing Parkinsonism. Further studying other prominent cases might lead us to better understanding of risk factors and the expression of early Parkinsonism.

  18. Cytogenetic abnormalities in tumors of patients with von Hippel-Lindau disease.

    Science.gov (United States)

    Jordan, D K; Patil, S R; Divelbiss, J E; Vemuganti, S; Headley, C; Waziri, M H; Gurll, N J

    1989-10-15

    Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder that causes the development of benign and malignant tumors in several organ systems. Tumors causing significant morbidity include retinal angioma, cerebellar hemangioblastoma (CH), renal cell carcinoma (RCC), and pheochromocytoma (Pheo). Cytogenetic studies of tumors in VHL patients are rare. Cytogenetic findings in tumors from 12 patients with VHL disease, including four RCCs, three CHs, and five Pheos are presented. Three of the four RCC cases were abnormal. Monosomy 3 or a deletion of 3p was present in all three abnormal cases. Complete or partial trisomy of chromosome 5 was present in two cases. A deletion of 14q, trisomy 7, and a missing Y were each observed in one case. These findings indicate that a deletion of 3p may be a primary cytogenetic change in RCCs associated with VHL disease in addition to playing a role in sporadic RCC. Duplications of 5q and deletions of 14q may be important secondary changes in the progression of the malignant phenotype. No visible cytogenetic abnormalities were observed in the three CHs, or in four of the Pheos. One of the five Pheos was found to exhibit mosaic trisomy 7; its significance is unclear at the present time.

  19. Endolymphatic sac tumour in von Hippel-Lindau disease: management strategies.

    Science.gov (United States)

    Zanoletti, E; Girasoli, L; Borsetto, D; Opocher, G; Mazzoni, A; Martini, A

    2017-10-01

    Endolymphatic sac tumour (ELST) is infrequent, as emerges from small series reported in the literature. It is a slow-growing malignancy with local aggressiveness and a low risk of distant metastases. It is often misdiagnosed because of the late onset of symptoms and difficulty in obtaining a biopsy. Its frequency is higher in von Hippel-Lindau (VHL) disease (a genetic systemic syndrome involving multiple tumours), with a prevalence of around 25%. The diagnosis is based on radiology, with specific patterns on contrast-enhanced MRI and typical petrous bone erosion on bone CT scan. Our experience of ELST in the years between 2012-2015 concerns 7 cases, one of which was bilateral, in patients with VHL disease. Four of the 7 patients underwent 5 surgical procedures at our institution. Each case is described in detail, including clinical symptoms, and the intervals between symptom onset, diagnosis and therapy. Postoperative morbidity was low after early surgery on small tumours, whereas extensive surgery for large tumours was associated with loss of cranial nerve function (especially VII, IX, X). The critical sites coinciding with loss of neurological function were the fallopian canal, jugular foramen, petrous apex and intradural extension into the posterior cranial fossa. Early surgery on small ELST is advocated for patients with VHL disease, in whom screening enables a prompt diagnosis and consequently good prognosis. © Copyright by Società Italiana di Otorinolaringologia e Chirurgia Cervico-Facciale, Rome, Italy.

  20. Von Hippel-Lindau disease: an evaluation of natural history and functional disability

    Science.gov (United States)

    Feletti, Alberto; Anglani, Mariagiulia; Scarpa, Bruno; Schiavi, Francesca; Boaretto, Francesca; Zovato, Stefania; Taschin, Elisa; Gardi, Mario; Zanoletti, Elisabetta; Piermarocchi, Stefano; Murgia, Alessandra; Pavesi, Giacomo; Opocher, Giuseppe

    2016-01-01

    Background Although many studies have been published about specific lesions characterizing von Hippel-Lindau(VHL) disease, none have dealt with the natural history of the whole disease and the consequent disabilities. We aim to define the comprehensive natural history of VHL disease and to describe the functional disabilities and their impact upon patients' quality of life, thereby tailoring the follow-up schedule accordingly. Methods We performed a prospective analysis on 128 VHL-affected patients beginning in 1996. For each affected organ, we defined intervals between the first and subsequent VHL-related manifestations and compared them with current VHL surveillance protocols. We looked for any association of the number of involved organs with age, sex, type of VHL gene mutation, and functional domain mutation. Ultimately, we assessed the organ-specific disabilities caused by VHL disease. Results Hemangioblastomas show different patterns of progression depending on their location, whereas both renal cysts and carcinomas have similar progression rates. Surgery for pheochromocytoma and CNS hemangioblastoma is performed earlier than for pancreatic or renal cancer. The number of involved organs is associated with age but not with sex, type of VHL gene mutation, or functional domain mutation. A thorough analysis of functional disabilities showed that age is related to the first-appearing functional impairment, but it is not predictive of the final number of disabilities. Conclusions Our study defines the disease progression and provides a comprehensive view of the syndrome over time. We analyzed for the first time the functional disability of VHL patients, assessing the progression for each function. PMID:26763786

  1. Management of endolymphatic sac tumors: sporadic cases and von Hippel-Lindau disease.

    Science.gov (United States)

    Nevoux, Jérôme; Nowak, Catherine; Vellin, Jean-François; Lepajolec, Christine; Sterkers, Olivier; Richard, Stéphane; Bobin, Serge

    2014-06-01

    To analyze the difference between the endolymphatic sac tumors (ELSTs) in sporadic cases and in von Hippel-Lindau (VHL) disease. Retrospective case review in a tertiary referral center. Fourteen cases of ELST, occurring since 1998, were reviewed. We analyzed the initial symptoms, characteristics of the tumor, treatment, sequelae, and follow-up for each group. The ELSTs were sporadic in 6 cases and associated with VHL disease in 8 cases. The mean age at the time of the first surgery was 26 years (range, 12-41). All except two of the patients presented with a unilateral tumor. The initial symptoms were hearing loss (n = 9), tinnitus (n = 7), and/or vertigo (n = 5). Hearing loss was more prevalent in the sporadic cases. Preoperative arteriography was performed for 4 patients, with embolization performed for 1 patient. The size of the tumor was significantly larger in the sporadic cases (31.7 mm) than in the cases of VHL disease (19.3 mm). The surgical approach was more extensive in the sporadic cases. The surgeons found 2 types of tumors. Cystic tumors with massive bleeding invading the surrounding structures (the dura mater or jugular bulb) were more common in the sporadic cases. Fibrous tumors that infiltrate the bone and have moderate bleeding were more common in the cases associated with VHL disease.Two patients with small lesions were not operated on but were followed for 6 years without tumor growth. They died of metastasis from gastric and kidney cancer. Four recurrences occurred during the 14 years of follow-up. Four facial palsies and 8 cases of profound deafness were encountered postoperatively. Sporadic tumors are more aggressive than those associated with VHL disease. Complete surgical resection should be the goal of treatment. Preoperative angiography with embolization is recommended. In some cases, embolization may be impossible, and preoperative or postoperative radiotherapy should be discussed.

  2. Intestinal Neurofibromatosis in von Recklinghausen’s Disease: Presenting as Chronic Anemia due to Recurrent Intestinal Hemorrhage

    Science.gov (United States)

    Hahn, Jee Sook; Chung, Jae Bock; Han, Seung Hee; Lee, Seung Woo; Noh, Sung Hoon; Lee, Jong Tae; Chun, Soo Il; Kim, Gyung Ho

    1992-01-01

    Neurofibromatosis (von Recklinghausen’s disease) is a neuroectodermal disorder characterized by pigmentary changes of the skin (café-au-lait spots), cutaneous and visceral tumors (neurofibromas) and systemic abnormalities. The involvement of gastrointestinal tract in neurofibromatosis is not common. The most common symptoms, refer able to lesions in the gut, are hematemesis, melena and abdominal pain. We experienced a case of intestinal neurofibroma in von Recklinghausen’s disease. The patient was a 39 year-old female who had suffered from chronic iron deficiency anemia and recurrent gastrointestinal hemorrhage due to two neurofibromas of jejunum for 3 years, which was diagnosed by superior mesenteric and ileal arteriogram and 99mTc pertechnate-labelled RBC scan, and treated by segmental resection of jejunum with end to end anastomosis. PMID:1339078

  3. Management of renal cell carcinoma in von Hippel-Lindau disease.

    Science.gov (United States)

    Hes, F J; Slootweg, P J; van Vroonhoven, T J; Hené, R J; Feldberg, M A; Zewald, R A; Ploos van Amstel, J K; Höppener, J W; Pearson, P L; Lips, C J

    1999-01-01

    An evaluation of nephron-sparing surgery (NSS) or radical nephrectomy (RN) for treating renal cell carcinoma (RCC) in patients with von Hippel-Lindau disease (VHL) was carried out. Between 1976 and 1997, 10 patients with RCC from four VHL families, of whom seven were from one family, were studied by clinical and histopathological examination. Before 1991, three patients were treated using RN, and thereafter five patients were treated using NSS. Two patients were not operated on. RCCs in our patients showed a slow growth rate (on average 0.3 cm year-1), and asymptomatic patients presented with tumours of low-grade malignancy. In all patients, tumours were surrounded by a fibrous pseudocapsule. In 5 out of 17 tumours, pseudocapsular invasion was observed, and three of these five tumours broke through the pseudocapsule. To date, these patients have not shown a less favourable outcome than those without pseudocapsular involvement by tumour growth. Multicentricity of RCC was relatively low (4.6 lesions per kidney). In two of the three RN patients, only a single satellite lesion, in the direct vicinity of a RCC, was found in one kidney. Six tumours (1.8-5.5 cm) were enucleated by NSS. During a mean follow-up of 30 months, renal function in these patients was well preserved. In our patients, RCCs grew slowly, were of low grade, had a dense fibrous pseudocapsule and were thus good candidates for NSS.

  4. Heavy menstrual bleeding and health-associated quality of life in women with von Willebrand's disease.

    Science.gov (United States)

    Govorov, Igor; Ekelund, Lena; Chaireti, Roza; Elfvinge, Petra; Holmström, Margareta; Bremme, Katarina; Mints, Miriam

    2016-05-01

    Women with the inherited bleeding disorder von Willebrand's disease (VWD) face gender-specific hemostatic challenges during menstruation. Heavy menstrual bleeding (HMB) can negatively affect their overall life activities and the health-associated quality of life. The purpose of the present study was to investigate whether women with VWD experienced HMB and an impaired health-associated quality of life. The study subjects were recruited from the Coagulation Unit of Karolinska University Hospital. Information was retrieved from various self-administered forms and medical records. Of the 30 women (18-52 years) that were included in the present study, 50% suffered from HMB, although the majority received treatment for HMB. In addition, almost all the included women perceived limitations in the overall life activities due to menstruation. The health-associated quality of life for women with HMB was significantly lower (Pwomen of the general population. In conclusion, women with VWD experienced reduced health-associated quality of life as a result of HMB. Therefore, preventing limitations in overall life activities and improving their health-associated quality of life thorough counseling on menstrual bleeding is important for women with VWD.

  5. Cost-utility analysis of von Willebrand disease screening in adolescents with menorrhagia.

    Science.gov (United States)

    Sidonio, Robert Francis; Smith, Kenneth J; Ragni, Margaret V

    2010-09-01

    To construct a decision analysis model to evaluate the cost utility of von Willebrand disease (VWD) testing in adolescents with menorrhagia. A 20-year Markov decision analytic model was constructed to evaluate the cost utility of two strategies: testing or not testing for VWD. The model includes probabilities of remaining well, suffering an acute menorrhagia bleeding event, surgical complications, oral contraceptive pill complications, or dying. Probabilities, costs, and utilities were estimated from published literature. The prevalence of type 1 VWD in adolescent females with menorrhagia was estimated at 13%. The cost of testing adolescents with menorrhagia for VWD was $1790, versus $1251 for not testing for VWD. The effectiveness of not testing in quality-adjusted life-years (QALYs) gained (14.237 QALYs) was similar to the VWD testing strategy (14.246 QALYs). Compared with not testing for VWD, screening for VWD had an incremental cost-effectiveness ratio of $62,791 per QALY, a value typically considered economically reasonable. In adolescents with menorrhagia, testing for VWD before the initiation of oral contraceptives is cost-effective. Copyright (c) 2010 Mosby, Inc. All rights reserved.

  6. Variable bleeding phenotype in an Amish pedigree with von Willebrand disease.

    Science.gov (United States)

    Gupta, Sweta; Heiman, Meadow; Duncan, Natalie; Hinckley, Jesse; Di Paola, Jorge; Shapiro, Amy D

    2016-10-01

    Through a cross-sectional study design, the bleeding phenotype in the Amish in Indiana (IN) and Wisconsin (WI) was described using two different bleeding scores. von Willebrand factor (VWF) testing was performed and bleeding questionnaires from Centers for Disease Control and Prevention (CDC) and European MCMDM-1 (Tosetto bleeding score (BS)) were administered to the IN and WI cohort respectively. Seven hundred and seventy nine subjects were recruited, 17% were diagnosed with VWD based on Ristocetin cofactor, VWF:RCo  T. The WI AF were much younger at a mean age 15 years vs 26 years in IN AF cohort. The AF subjects had a median VWF:RCo of 13IU/dl with a statistically significant higher median BS 1 versus 0 in the WI AF vs WI Unaffected (UA), 2 vs 1 in the IN AF vs IN UA, P Amish with VWD, despite a unifying mutation. Am. J. Hematol. 91:E431-E435, 2016. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  7. Uptake of genetic testing and long-term tumor surveillance in von Hippel-Lindau disease

    Directory of Open Access Journals (Sweden)

    Chávez Mireya

    2010-01-01

    Full Text Available Abstract Background von Hippel-Lindau (VHL disease is a hereditary cancer syndrome caused by germline mutations in the VHL gene. Patients have significant morbidity and mortality secondary to vascular tumors. Disease management is centered on tumor surveillance that allows early detection and treatment. Presymptomatic genetic testing is therefore recommended, including in at-risk children. Methods We tested 17 families (n = 109 individuals for VHL mutations including 43 children under the age of 18. Personalized genetic counseling was provided pre and post-test and the individuals undergoing presymptomatic testing filled out questionnaires gathering socio-demographic, psychological and psychiatric data. Mutation analysis was performed by direct sequencing of the VHL gene. Mutation-carriers were screened for VHL disease-related tumors and were offered follow-up annual examinations. Results Mutations were identified in 36 patients, 17 of whom were asymptomatic. In the initial screening, we identified at least one tumor in five of 17 previously asymptomatic individuals. At the end of five years, only 38.9% of the mutation-carriers continued participating in our tumor surveillance program. During this time, 14 mutation carriers developed a total of 32 new tumors, three of whom died of complications. Gender, education, income, marital status and religiosity were not found to be associated with adherence to the surveillance protocol. Follow-up adherence was also independent of pre-test depression, severity of disease, or number of affected family members. The only statistically significant predictor of adherence was being symptomatic at the time of testing (OR = 5; 95% CI 1.2 - 20.3; p = 0.02. Pre-test anxiety was more commonly observed in patients that discontinued follow-up (64.7% vs. 35.3%; p = 0.01. Conclusions The high initial uptake rate of genetic testing for VHL disease, including in minors, allowed the discontinuation of unnecessary screening

  8. Characterization of recessive severe type 1 and 3 von willebrand disease (vwd), asymptomatic heterozygous carriers versus bloodgroup o-related von willebrand factor deficiency, and dominant type 1 VWD

    NARCIS (Netherlands)

    J.J. Michiels (Jan); Z. Berneman (Zwi); A. Gadisseur (Alain); M. van der Planken (Marc); W. Schroyens (Wilfried); A. van de Velde (Ann); H.H.D.M. van Vliet (Huib)

    2006-01-01

    textabstractRecessive type 3 von Willebrand disease (VWD) is caused by homozygosity or double heterozygosity for two non-sense mutations (null alleles). Type 3 VWD is easy to diagnose by the combination of a strongly prolonged bleeding time (BT), absence of ristocetine-induced platelet aggregation

  9. Plasmatic ADAMTS-13 metalloprotease and von Willebrand factor in children with cyanotic congenital heart disease.

    Science.gov (United States)

    Soares, R P S; Bydlowski, S P; Nascimento, N M; Thomaz, A M; Bastos, E N M; Lopes, A A

    2013-04-01

    Changes in plasma von Willebrand factor concentration (VWF:Ag) and ADAMTS-13 activity (the metalloprotease that cleaves VWF physiologically) have been reported in several cardiovascular disorders with prognostic implications. We therefore determined the level of these proteins in the plasma of children with cyanotic congenital heart disease (CCHD) undergoing surgical treatment. Forty-eight children were enrolled (age 0.83 to 7.58 years). Measurements were performed at baseline and 48 h after surgery. ELISA, collagen-binding assays and Western blotting were used to estimate antigenic and biological activities, and proteolysis of VWF multimers. Preoperatively, VWF:Ag and ADAMTS-13 activity were decreased (65 and 71% of normal levels considered as 113 (105-129) U/dL and 91 ± 24% respectively, P membranes, followed by proteolytic cleavage. A low preoperative ADAMTS-13 activity, a longer activated partial thromboplastin time and the need for cardiopulmonary bypass correlated with postoperative bleeding (P < 0.05). Postoperatively, ADAMTS-13 activity increased but less extensively than VWF:Ag (respectively, 2.23 and 2.83 times baseline, P < 0.0001), resulting in an increased VWF:Ag/ADAMTS-13 activity ratio (1.20 to 1.54, respectively, pre- and postoperative median values, P = 0.0029). ADAMTS-13 consumption was further confirmed by decreased ADAMTS-13 antigenic concentration (0.91 ± 0.30 to 0.70 ± 0.25 µg/mL, P < 0.0001) and persistent proteolysis of VWF multimers. We conclude that, in pediatric CCHD, changes in circulating ADAMTS-13 suggest enzyme consumption, associated with abnormal structure and function of VWF.

  10. Von Hippel-Lindau disease associated with myasthenia gravis not related to thymoma

    Directory of Open Access Journals (Sweden)

    Paolo Pozzato

    2013-04-01

    Full Text Available BACKGROUND Von Hippel-Lindau disease (VHL is a rare autosomal dominant inherited disorder characterized by an increased risk of tumours in a number of locations (eyes, brain, adrenal gland, pancreas, liver, kidneys, or other areas of the body. It is caused by germline mutation in the VHL gene. The VHL gene is a tumour suppressor gene that has been identified on the short arm of chromosome 3. CASE REPORT We report a case of a 60 year-old female with the clinical diagnosis of VHL type 1 (cerebellar haemangioblastoma, pancreatic cysts with subsequent steatorrhoea, and bilateral renal carcinoma who developed weakness and fatigability of skeletal muscles, left lid ptosis, snarling expression and nasal timbre speech. Acetylcholine receptor antibodies were negative in serum, while the electrodiagnostic test demonstrated an alteration of neuromuscolar junction which was consistent with the diagnosis of myasthenia gravis. Contrast-enhanced TC scan of the anterior mediastinum was performed, which excluded thymus enlargement. VHL gene evaluation in this patient identified a new mutation (c279delC9 and polymorphism c291C>G. At present the patient still suffers from ataxia and dysmetria due to cerebellar involvement in VHL, while fatigue and lid ptosis improved after the treatment with oral pyridostigmine 60 mg tid. DISCUSSION AND CONCLUSIONS To our knowledge this is the first report of a case of VHL associated with myasthenia gravis without thymoma. A case of VHL associated with a form of myasthenia gravis related to thymoma has been recently reported. In our case the absence of acetylcholine receptor antibodies may suggest a genetic origin also for the myasthenia gravis.

  11. Plasmatic ADAMTS-13 metalloprotease and von Willebrand factor in children with cyanotic congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Soares, R.P.S. [Fundação Pró-Sangue Hemocentro de São Paulo, São Paulo, SP (Brazil); Bydlowski, S.P.; Nascimento, N.M. [Laboratório de Investigação Médica-31, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP (Brazil); Thomaz, A.M.; Bastos, E.N.M.; Lopes, A.A. [Faculdade de Medicina, Instituto do Coração, Universidade de São Paulo, São Paulo, SP (Brazil)

    2013-04-05

    Changes in plasma von Willebrand factor concentration (VWF:Ag) and ADAMTS-13 activity (the metalloprotease that cleaves VWF physiologically) have been reported in several cardiovascular disorders with prognostic implications. We therefore determined the level of these proteins in the plasma of children with cyanotic congenital heart disease (CCHD) undergoing surgical treatment. Forty-eight children were enrolled (age 0.83 to 7.58 years). Measurements were performed at baseline and 48 h after surgery. ELISA, collagen-binding assays and Western blotting were used to estimate antigenic and biological activities, and proteolysis of VWF multimers. Preoperatively, VWF:Ag and ADAMTS-13 activity were decreased (65 and 71% of normal levels considered as 113 (105-129) U/dL and 91 ± 24% respectively, P < 0.003) and correlated (r = 0.39, P = 0.0064). High molecular weight VWF multimers were not related, suggesting an interaction of VWF with cell membranes, followed by proteolytic cleavage. A low preoperative ADAMTS-13 activity, a longer activated partial thromboplastin time and the need for cardiopulmonary bypass correlated with postoperative bleeding (P < 0.05). Postoperatively, ADAMTS-13 activity increased but less extensively than VWF:Ag (respectively, 2.23 and 2.83 times baseline, P < 0.0001), resulting in an increased VWF:Ag/ADAMTS-13 activity ratio (1.20 to 1.54, respectively, pre- and postoperative median values, P = 0.0029). ADAMTS-13 consumption was further confirmed by decreased ADAMTS-13 antigenic concentration (0.91 ± 0.30 to 0.70 ± 0.25 µg/mL, P < 0.0001) and persistent proteolysis of VWF multimers. We conclude that, in pediatric CCHD, changes in circulating ADAMTS-13 suggest enzyme consumption, associated with abnormal structure and function of VWF.

  12. Diagnosing von Willebrand disease: a short history of laboratory milestones and innovations, plus current status, challenges, and solutions.

    Science.gov (United States)

    Favaloro, Emmanuel J

    2014-07-01

    von Willebrand disease (VWD) is a disorder characterized by deficiency of, or defects in, von Willebrand factor (VWF). VWD was originally identified by Erik Adolf von Willebrand, who in early 1924 investigated a large family suffering from a bleeding disorder that seemed to differ from hemophilia. Erik von Willebrand undertook some initial laboratory investigations to conclude the involvement of a plasma factor, the lack of which prolonged the bleeding time, but failed to impair coagulation times and clot retraction. By the end of the 1960s, VWD was accepted as a combined deficiency of factor VIII (FVIII) and another plasma factor responsible for normal platelet adhesion. Just how these two functions were related to each other was less clear and the diagnostic tests available at the time were poorly reproducible, cumbersome, and unreliable; thus, VWD was poorly delineated from other coagulation and platelet disorders. The early 1970s saw a revolution in diagnostics when ristocetin was identified to induce platelet aggregation, and this formed the basis of the first consistent and reliable VWF "activity" test, permitting quantification of the platelet adhesive function missing in VWD. Concurrently, immunoprecipitating techniques specific for VWF were defined, and the application of such technologies permitted a clearer understanding of both VWF and VWD heterogeneity. Continued exploration of the structure and function of VWF contributed greatly to the understanding of platelet physiology, ligand receptor interaction and pathways of cellular interaction and activation. Recently, additional assays evaluating other functions of VWF, including collagen binding, platelet glycoprotein Ib binding, and FVIII binding, have improved the diagnosis of VWD. The purpose of this narrative review is to explore the history of phenotypic VWD diagnostics, with a focus on laboratory milestones from the past as well highlighting recent and ongoing innovations, and ongoing challenges and

  13. Von Willebrand factor and fibrinolytic parameters during the desmopressin test in patients with Cushing's disease

    Science.gov (United States)

    Giraldi, Francesca Pecori; Ambrogio, Alberto G; Fatti, Letizia M; Rubini, Valentina; Cozzi, Giovanna; Scacchi, Massimo; Federici, Augusto B; Cavagnini, Francesco

    2011-01-01

    AIMS Desmopressin, a vasopressin analogue, is used for various clinical purposes, including haemostasis and, in recent times, the diagnostic work-up of patients with Cushing's syndrome, a condition associated with a known prothrombotic profile. We decided to evaluate whether and to what extent a diagnostic dose of desmopressin induces significant changes in endothelial parameters in patients with Cushing's disease (CD) and obese and normal weight controls. METHODS Twelve patients with CD, 10 obese and five normal weight controls were studied. Von Willebrand antigen (VWF : Ag), tissue plasminogen activator (t-PA) and plasminogen activator inhibitor type 1 (PAI-1) were measured at baseline and up to 4 h after 10 µg desmopressin i.v. RESULTS Desmopressin 10 µg transiently increased VWF : Ag and t-PA and decreased PAI-1 in all subjects. The magnitude of the VWF : Ag and t-PA increases after desmopressin was comparable in the three groups (VWF : Ag peak-to-basal ratio 1.9 ± 0.17, 1.5 ± 0.11 and 1.8 ± 0.13 and t-PA peak-to-basal ratio 1.6 ± 0.18, 1.6 ± 0.20 and 1.8 ± 0.24 for CD, obese and controls, respectively, all NS). The PAI-1 decrease observed in patients with CD was comparable with obese (0.7 ± 0.07 and 0.6 ± 0.09, NS) and controls (0.7 ± 0.07 vs. 0.4 ± 0.09, P = 0.08). CONCLUSIONS Administration of desmopressin to patients with CD for diagnostic purposes induces a transitory increase in VWF : Ag counterbalanced by a decrease in PAI-1 and increase in t-PA. The magnitude of these changes is largely comparable with that observed in obese and normal weight controls. Our data show that testing with desmopressin does not induce disease-specific changes in endothelial markers in patients with CD. PMID:21143510

  14. ["Euthanasia" operation by Nazis on patients with psychiatric or hereditary diseases, and Bishop von Galen of Münster].

    Science.gov (United States)

    Izumi, Hyonosuke

    2003-07-01

    In so-called "euthanasia" operations, Nazis murdered patients with psychiatric or hereditary diseases in large numbers. Psychiatric patients in Germany were sent to six institutions, where they were deprived of their lives in gas chambers. In his sermon delivered on 3rd August, 1941, at St Lambert's Church in Münster, Bishop von Galen of Münster intensely condemned this cruel operation in public. Quoting the fifth commandment, "Thou shall not kill", he said it was sinful to kill innocent people on account of their unproductiveness. By the influence of this brave sermon, Hitler had to order the closure of the institutions, though the "euthanasia" operation itself was secretly continued.

  15. Pitfalls in Interventional Pain Medicine: Hyponatremia after DDAVP for a Patient with Von Willebrand Disease Undergoing an Epidural Steroid Injection

    Directory of Open Access Journals (Sweden)

    Talal W. Khan

    2017-01-01

    Full Text Available Desmopressin (DDAVP, a synthetic analog of vasopressin, has been used in patients with von Willebrand disease (VWD, mild hemophilia A, and platelet dysfunction to reduce the risk of bleeding associated with surgical and interventional procedures. We report the case of a patient with VWD presenting with a bulging disc and radicular pain that underwent transforaminal epidural steroid injections. Her course was complicated with the interval development of headaches and dizziness symptomatic of moderate hyponatremia, likely due to excessive fluid intake. This report highlights a relatively rare side effect of DDAVP when used for prophylaxis in patients with VWD and reinforces the need for vigilance in these patients.

  16. Computed tomography of Crohn`s disease and ulcerative colitis; Computertomographische Morphologie von Morbus Crohn und Colitis ulcerosa

    Energy Technology Data Exchange (ETDEWEB)

    Klein, H.M. [Klinik fuer Radiologische Diagnostik der RWTH, Aachen (Germany); Wein, B. [Klinik fuer Radiologische Diagnostik der RWTH, Aachen (Germany); Adam, G. [Klinik fuer Radiologische Diagnostik der RWTH, Aachen (Germany); Ruppert, D. [Klinik fuer Radiologische Diagnostik der RWTH, Aachen (Germany); Guenther, R.W. [Klinik fuer Radiologische Diagnostik der RWTH, Aachen (Germany)

    1995-07-01

    We analysed the CT examinations of 109 patients with 197 involved bowel locations. 81 patients suffered from Crohn`s disease, 28 from ulcerative colitis. Diagnosis was based on the combination of clinical, endoscopic and histopathologic findings. Three radiologists evaluated the CT series concerning the presence of morphologic changes analogous to conventional radiographic findings. In Crohn`s disease, we found irregular outer contours in 26% of cases. The bowel wall was thickened in 82%. In acute phases, the bowel wall was thickened in 100%. Abscess and fistula as complications of inflammatory disease were present in 26 and 14% respectively. In ulcerative colitis, a target sign of the bowel wall was present in 40%, whereas in Crohn`s disease a homogeneous wall density was present in all but two cases. Reduced attenuation due to submucosal fat deposits was found in 16% and mucosal tunneling in 27% of cases with ulcerative colitis. Even if severe mucosal destructions were found, the outer contour of the gut was smooth and regular in 95% of the ulcerative colitis cases. CT can provide additional information on acuity, extent and complications in inflammatory bowel disease. In combination with conventional radiographic findings a three-step classification for Crohn`s disease and ulcerative colitis (early changes, acute and chronic phase) can be proposed. (orig./MG) [Deutsch] Wir untersuchten computertomographisch 109 Patienten mit 197 erkrankten Darmsegmenten. 81 Patienten litten an M. Crohn und 28 an Colitis ulcerosa. Die Diagnose wurde durch Kombination klinischer, endoskopischer, radiologischer und bioptischer Befunde gesichert. Drei Radiologen bewerteten die Computertomographien und verglichen die Ergebnisse mit den konventionell-radiologischen Befunden. Bei den Patienten mit M. Crohn fanden wir in 26% der Faelle eine irregulaere aeussere Darmwand. Eine Darmwandverdickung lag in 82% vor und fand sich regelmaessig in Darmabschnitten mit floriden Veraenderungen

  17. Long-term impact of joint bleeds in von Willebrand disease: a nested case-control study.

    Science.gov (United States)

    van Galen, Karin P M; de Kleijn, Piet; Foppen, Wouter; Eikenboom, Jeroen; Meijer, Karina; Schutgens, Roger E G; Fischer, Kathelijn; Cnossen, Marjon H; de Meris, Joke; Fijnvandraat, Karin; van der Bom, Johanna G; Laros-van Gorkom, Britta A P; Leebeek, Frank W G; Mauser-Bunschoten, Eveline P

    2017-09-01

    Patients with severe von Willebrand disease (VWD) may develop arthropathy after joint bleeds. Information on its prevalence and severity is limited. We aimed to assess the occurrence and severity of arthropathy in VWD and its impact on daily life. VWD patients with and without verified joint bleeds were matched for age, sex and Factor VIII level or von Willebrand Factor activity in a nested case-control study within the Willebrand in the Netherlands study. Assessments included the Hemophilia Joint Health Score (0-124), Pettersson score (0-13 per joint X-ray), Hemophilia Activity List score (0-100), joint pain (Visual Analog Scale 0-10), and the Impact on Participation and Autonomy questionnaire (0-20). Arthropathy was defined as a Hemophilia Joint Health Score of 10 or higher, or a Pettersson score over 3 of at least one joint. We included 48 patients with verified joint bleeds (cases) and 48 controls: 60% males, mean age 46 years (range 18-80), median von Willebrand Factor activity 5 versus 8 IU/dL and Factor VIII 24 versus 36 IU/dL. Arthropathy occurred in 40% of the cases versus 10% of the controls (PList score: 88 vs. 100, P3: 13 of 19 vs. 3 of 28, P<0.01, and median score on the participation questionnaire 6.1 vs. 0.9, P<0.01). In conclusion, arthropathy occurs in 40% of VWD patients after joint bleeds and is associated with pain, radiological abnormalities, functional limitations, and less social participation (Dutch trial register: NTR4548). Copyright© 2017 Ferrata Storti Foundation.

  18. Utility of platelet function analyzer as a screening tool for the diagnosis of von Willebrand disease in adolescents with menorrhagia.

    Science.gov (United States)

    Naik, Swati; Teruya, Jun; Dietrich, Jennifer E; Jariwala, Purvi; Soundar, Esther; Venkateswaran, Lakshmi

    2013-07-01

    Von Willebrand disease (VWD), and in particular, VWD type 1 and low VW factor (defined as Von Willebrand Ristocetin cofactor activity (RCoF) menorrhagia and both groups benefit from similar management. Platelet function analyzer (PFA-100®) is often used as a screening test to detect VWD. We analyzed the utility of PFA-100® as a screening tool in the detection of VWD type 1 and low VW factor (VWF) in an exclusive adolescent population with menorrhagia. The study population consisted of adolescents with menorrhagia who had simultaneously drawn blood samples for VWD and PFA-100®. Abnormal PFA-100® was defined as values >183 seconds for collagen/epinephrine and/or >126 seconds for collagen/ADP. Of a total of 235 patients tested, 23 patients had RCoF menorrhagia. We conclude that in the setting of adolescent menorrhagia, PFA-100® does not have utility as an initial screening test for the diagnosis of VWD and in particular, low VWF and that clinicians need to be aware of this limitation of PFA-100® while evaluating adolescents with menorrhagia. Copyright © 2013 Wiley Periodicals, Inc.

  19. Hypoxia-inducible factor-2α stabilizes the von Hippel-Lindau (VHL) disease suppressor, Myb-related protein 2.

    Science.gov (United States)

    Okumura, Fumihiko; Joo-Okumura, Akiko; Nakatsukasa, Kunio; Kamura, Takumi

    2017-01-01

    Ubiquitin ligase von Hippel-Lindau tumor suppressor (pVHL) negatively regulates protein levels of hypoxia-inducible factor-α (HIF-α). Loss of pVHL causes HIF-α accumulation, which contributes to the pathogenesis of von Hippel-Lindau (VHL) disease. In contrast, v-Myb avian myeloblastosis viral oncogene homolog-like 2 (MYBL2; B-Myb), a transcription factor, prevents VHL pathogenesis by regulating gene expression of HIF-independent pathways. Both HIF-α and B-Myb are targets of pVHL-mediated polyubiquitination and proteasomal degradation. Here, we show that knockdown of HIF-2α induces downregulation of B-Myb in 786-O cells, which are deficient in pVHL, and this downregulation is prevented by proteasome inhibition. In the presence of pVHL and under hypoxia-like conditions, B-Myb and HIF-2α are both upregulated, and the upregulation of B-Myb requires expression of HIF-2α. We also show that HIF-2α and B-Myb interact in the nucleus, and this interaction is mediated by the central region of HIF-2α and the C-terminal region of B-Myb. These data indicate that oncogenic HIF-2α stabilizes B-Myb to suppress VHL pathogenesis.

  20. Rinoplastia em paciente com doença de Von Willebrand: relato de caso Rinoplastia en paciente con enfermedad de Von Willebrand: relato de caso Rhinoplasty in a patient with Von Willebrand disease: case report

    Directory of Open Access Journals (Sweden)

    Roberto Martins Matos Junior

    2007-12-01

    minutos. En el postoperatorio fue prescrito ácido amino caproico (500 mg por vía oral a cada 8 horas, por 48 horas, con alta hospitalaria después de 24 horas, sin presentar ninguna complicación postoperatoria. CONCLUSIONES: La infusión de crioprecipitado o plasma, utilizado en la profilaxis y tratamiento de las complicaciones hemorrágicas, produce un pico de concentración máxima de factor VIII después 48 horas, y se sustenta por 72 horas; sin embargo, incluso aprobado por el FDA, ha sido una práctica utilizada solamente en circunstancias de emergencia, debido al riesgo relativo de contaminación viral. A 1-desamino, 8-D-arginina vasopresina (DDAVP-desmopresina estimula el aumento de la concentración del factor VIII, con la ventaja de eliminar la exposición a patógenos transmitidos por la sangre, además de la posibilidad de administración por vía nasal, subcutánea y venosa.BACKGROUND AND OBJECTIVES: Patients with von Willebrand disease present abnormal bleeding after being wounded or during surgeries since it affects primary and secondary hemostasia due to changes in factor VIII. The objective of this report was to elucidate the pre-, peri-, and postoperative management of patients with this disorder. CASE REPORT: A 42-year old white female, with 165 cm, 61 kg, ASA II, with a diagnosis of type 1 von Willebrand disease, underwent pre-anesthetic evaluation for rhinoplasty. She was cleared for surgery after hematological evaluation with a positive DDAVP IN26 test. On the day of the surgery, the patient received pre-anesthetic medication, was adequately monitored, oxygen was administered through a nasal cannula and intravenous desmopressin (0.4 µg.kg-1 in 100 mL of normal saline was administered 30 minutes before the surgery. Induction was accomplished with intravenous sufentanil (1 µg.kg-1, propofol (4 mg.kg-1, and rocuronium (0.6 mg.kg-1. The patient was intubated and installed on mechanical ventilation with a CO2 absorber system and maintenance consisted

  1. A 12.3-kb Duplication Within the VWF Gene in Pigs Affected by Von Willebrand Disease Type 3

    Directory of Open Access Journals (Sweden)

    Stefanie Lehner

    2018-02-01

    Full Text Available Von Willebrand Disease (VWD type 3 is a serious and sometimes fatal hereditary bleeding disorder. In pigs, the disease has been known for decades, and affected animals are used as models for the human disease. Due to the recessive mode of inheritance of VWD type 3, severe bleeding is typically seen in homozygous individuals. We sequenced the complete porcine VWF (Von Willebrand Factor complementary DNA (cDNA and detected a tandem duplication of exons 17 and 18, causing a frameshift and a premature termination codon (p.Val814LeufsTer3 in the affected pig. Subsequent next generation sequencing on genomic DNA proved the existence of a 12.3-kb tandem duplication associated with VWD. This duplication putatively originates from porcine Short Interspersed Nuclear Elements (SINEs located within VWF introns 16 and 18 with high identity. The premature termination truncates the VWF open reading frame by a large part, resulting in an almost entire loss of the mature peptide. It is therefore supposed to account for the severe VWD type 3. Our results further indicate the presence of strong, nonsense-mediated decay in VWF messenger RNA (mRNA containing the duplication, which was supported by the almost complete absence of the complete VWF protein in immunohistochemistry analysis of the VWD-affected pig. In the past, differentiation of wild-type and heterozygous pigs in this VWD colony had to rely on clinical examinations and additional laboratory methods. The present study provides the basis to distinguish both genotypes by performing a rapid and simple genetic analysis.

  2. Preimplantation genetic diagnosis of Von Hippel-Lindau disease cancer syndrome by combined mutation and segregation analysis

    Directory of Open Access Journals (Sweden)

    Denilce R. Sumita

    2007-03-01

    Full Text Available Von Hippel-Lindau (VHL disease is an autosomal dominant cancer syndrome, associated with the development of tumors and cysts in multiple organ systems, whose expression and age of onset are highly variable. The VHL disease tumor suppressor gene (VHL maps to 3p25-p26 and mutations ranging from a single base change to large deletions have been detected in patients with VHL disease. We developed a single cell PCR protocol for preimplantation genetic diagnosis (PGD of VHL disease to select unaffected embryos on the basis of the detection of the specific mutation and segregation analysis of polymorphic linked markers. Multiplex-nested PCR using single buccal cells of an affected individual were performed in order to test the accuracy and reliability of this single-cell protocol. For each locus tested, amplification efficiency was 83% to 87% and allelic drop-out rates ranged from 12% to 8%. Three VHL disease PGD cycles were performed on cells from a couple with paternal transmission of a 436delC mutation in exon 2 of the VHL gene, leading to the identification of three unaffected embryos. Independent of the mutation present, this general PGD protocol for the diagnosis of VHL disease can be used in families informative for either the D3S1038 or D3S1317 microsatellite markers.

  3. SOMATIC MUTATIONS OF THE VON HIPPEL-LINDAU DISEASE TUMOR-SUPPRESSOR GENE IN NONFAMILIAL CLEAR-CELL RENAL-CARCINOMA

    NARCIS (Netherlands)

    FOSTER, K; PROWSE, A; van den Berg, Anke; FLEMING, S; HULSBEEK, MMF; CROSSEY, PA; RICHARDS, FM; CAIRNS, P; FERGUSONSMITH, MA; BUYS, CHCM; MAHER, ER

    1994-01-01

    Loss of heterozygosity (LOH) studies have suggested that somatic mutations of a tumour suppressor gene or genes on chromosome 3p are a critical event in the pathogenesis of non-familial renal cell carcinoma (RCC). Germline mutations of the von Hippel - Lindau (VHL) disease gene predispose to early

  4. Antifibrinolytic therapy for preventing oral bleeding in patients with haemophilia or Von Willebrand disease undergoing minor oral surgery or dental extractions

    NARCIS (Netherlands)

    van Galen, Karin Pm|info:eu-repo/dai/nl/325853886; Engelen, Eveline T; Mauser-Bunschoten, Evelien P|info:eu-repo/dai/nl/074719718; van Es, Robert Jj|info:eu-repo/dai/nl/216460646; Schutgens, Roger Eg|info:eu-repo/dai/nl/258752084

    2015-01-01

    BACKGROUND: Minor oral surgery or dental extractions (oral or dental procedures) are widely performed and can be complicated by hazardous oral bleeding, especially in people with an inherited bleeding disorder such as haemophilia or Von Willebrand disease. The amount and severity of singular

  5. Dogs with hearth diseases causing turbulent high-velocity blood flow have changes in patelet function and von Willebrand factor multimer distribution

    DEFF Research Database (Denmark)

    Tarnow, Inge; Kristensen, Annemarie Thuri; Olsen, Lisbeth Høier

    2005-01-01

    The purpose of this prospective study was to investigate platelet function using in vitro tests based on both high and low shear rates and von Willebrand factor (vWf) multimeric composition in dogs with cardiac disease and turbulent high-velocity blood flow. Client-owned asymptomatic, untreated d...

  6. Importance of SPECT/CT for resolving diseases of the jaw; Stellenwert der SPECT/CT zur Abklaerung von Kiefererkrankungen

    Energy Technology Data Exchange (ETDEWEB)

    Strobel, K.; Huellner, M.W.; Bolouri, C.; Veit-Haibach, P.; Sol Perez-Lago, M. del [Luzerner Kantonsspital, Institut fuer Nuklearmedizin und Roentgendiagnostik, Luzern (Switzerland); Merwald, M.; Kuttenberger, J. [Luzerner Kantonsspital, Klinik fuer Mund-, Kiefer- und Gesichtschirurgie, Luzern (Switzerland)

    2012-07-15

    Diseases of the jaw, such as osteomyelitis, condylar hyperactivity and tumors need adequate imaging to evaluate the extension and activity for therapy planning. Conventional planar scintigraphy, orthopantomography, computed tomography (CT) and magnetic resonance imaging (MRI) can be used for the evaluation of jaw diseases. Single photon emission computed tomography/computed tomography (SPECT/CT) provides metabolic and morphologic information in one imaging step and is becoming increasingly more available in larger hospitals. The SPECT/CT is superior to planar scintigraphy alone, CT and orthopantomography in the evaluation of the extension and activity of osteomyelitis and jaw tumors. In our hospital SPECT/CT has replaced the other imaging modalities in the evaluation of osteomyelitis and condylar hyperactivity. If available SPECT/CT should be performed for the evaluation of osteomyelitis of the jaw. (orig.) [German] Kiefererkrankungen wie Osteomyelitis, kondylaere Hyperaktivitaet und Kiefertumoren benoetigen eine exakte bildgebende Abklaerung, um die Therapie adaequat planen zu koennen. Die klassische planare Skelettszintigraphie, Orthopantomographie, CT und MRT koennen zur Abklaerung von Kiefererkrankungen eingesetzt werden. Die ''single photon emission computed tomography''/CT (SPECT/CT) bietet metabolische und morphologische Informationen in einem Untersuchungsgang und ist zunehmend in groesseren Kliniken verfuegbar. Die SPECT/CT ist zur Beurteilung der Ausbreitung und Aktivitaet einer Osteomyelitis und von Kiefertumoren der planaren Szintigraphie alleine, der CT und der Orthopantomographie ueberlegen. In unserer Klinik hat die SPECT/CT die bisherigen Verfahren zur Beurteilung der Kieferosteomyelitis und kondylaeren Hyperaktivitaet abgeloest. Sofern verfuegbar, sollte zur Abklaerung einer Kieferosteomyelitis die SPECT/CT eingesetzt werden. (orig.)

  7. Molecular and clinical profile of von Willebrand disease in Spain (PCM-EVW-ES): comprehensive genetic analysis by next-generation sequencing of 480 patients.

    Science.gov (United States)

    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.

  8. Clinical differential diagnosis of diseases of the oral cavity and oropharynx; Klinische Differentialdiagnostik von Erkrankungen der Mundhoehle und des Oropharynx

    Energy Technology Data Exchange (ETDEWEB)

    Grevers, G. [Klinik und Poliklinik fuer Hals-Nasen- und Ohrenheilkunde, Kopf- und Halschirurgie der Ludwig-Maximilians-Universitaet, Muenchen (Germany)

    1996-03-01

    In the present paper, common disorders of the oral cavity and oropharynx are described with special emphasis on differential diagnostic aspects. The first part of this presentation covers different inflammatory diseases, mainly focusing on complications like peritonsillar, para- and retropharyngeal abscesses, and Ludwig`s angina. These clinical entities can lead to further life-threatening complications, including deep neck infections and mediastinitis. The diagnostic vlaue and necessity of modern imaging in these cases are emphasized. In the second part, the author reports on the incidence, etiology and clinical course of tumors of the oral cavity and oropharynx with special regard to malignancies. Tumors in these areas have been increasing in number over the past decades mainly due to changes in alcohol and nicotine consumption in the developed countries. Diagnostic management includes a thorough clinical evaluation as well as modern imaging for tumor delineation and possible bone infiltration, depending on the site of the original tumor. In addition, therapeutic considerations are discussed, focusing on surgical tumor removal and soft tissue replacement using different pedicled flaps and free flaps. It is also emphasized that postoperative radiotherapy is mandatory in most malignant tumors in this area. (orig.) [Deutsch] In der vorliegenden Arbeit werden verschiedene klinisch relevante Krankheitsbilder in Mundhoehle und Oropharynx unter differentialdiagnostischen Aspekten vorgestellt. Um die Thematik uebersichtlich zu gestalten, wird auf eine Besprechung von Erkrankungen dieser Region verzichtet, die selten sind oder den Hals-Nasen-Ohrenarzt nicht betreffen. Im einzelnen wird zunaechst die Klinik, Diagnostik und Therapie von Entzuendungen angesprochen. In diesem Zusammenhang werden v.a. Komplikationen wie z.B. Para-, Retropharyngealabszess, Mundbodenabszess und Zungengrundabszess vorgestellt, weil diese Krankheitsbilder u.U. zu weiterreichenden Komplikationen

  9. Assessing the clinical severity of type 1 von Willebrand disease patients with a microchip flow-chamber system.

    Science.gov (United States)

    Nogami, K; Ogiwara, K; Yada, K; Shida, Y; Takeyama, M; Yaoi, H; Minami, H; Furukawa, S; Hosokawa, K; Shima, M

    2016-04-01

    The clinical phenotype of von Willebrand disease (VWD) is heterogeneous, and von Willebrand factor ristocetin cofactor activity (VWF:RCo) does not always reflect clinical severity, especially in VWD type 1. We have reported the potential of a microchip flow-chamber system (Total-Thrombus Formation Analysis System [T-TAS®]) for assessing physiologic hemostasis in VWD. Aim To evaluate the relationship between T-TAS, bleeding score (BS) and laboratory test results in type 1 VWD patients. Microchips coated with collagen (platelet chip [PL-chip]) or collagen/thromboplastin (AR-chip) were used to assess platelet thrombus formation (PTF) at high shear rates or fibrin-rich PTF at low shear rates, respectively, in whole blood from 50 patients. The times needed for the flow pressure to increase by 10 kPa and 30 kPa (T10 and T30 ) from baseline were calculated from flow pressure curves. BS was determined by the use of a standardized questionnaire. PL-T10 values correlated with BS (R(2) ~ 0.45) better than VWF:RCo (R(2) ~ 0.36), irrespective of the flow rate, whereas AR-T10 showed only a weak correlation with BS (R(2) ~ 0.18). Patients with PL-T10 > 10 min or AR-T10 > 30 min had lower VWF levels and higher BS than those with PL-T10 ≤ 10 min or AR-T10 ≤ 30 min, and the greatest differences were observed with PL-T10. Clinical severity appeared to correlate best with PL-T10 > 8 min. BS was significantly higher in patients with VWF:RCo of 8 min than in those with PL-T10 ≤ 8 min. T-TAS could be a useful technique for discriminating and predicting BS in VWD type 1 patients. © 2016 International Society on Thrombosis and Haemostasis.

  10. A rapid, automated VWF ristocetin cofactor activity assay improves reliability in the diagnosis of Von Willebrand disease.

    Science.gov (United States)

    Bowyer, Annette E; Shepherd, Fiona; Kitchen, Stephen; Makris, Michael

    2011-04-01

    The effective diagnosis and monitoring of Von Willebrand Disease (VWD) requires an accurate assessment of ristocetin co-factor activity (VWF:RCo). Current methodologies include automated platelet aggregometry and manual visual agglutination both of which are laborious to perform and notoriously subject to a high degree of inter and intra assay variation. We have evaluated an automated VWF:RCo assay (BC Von Willebrand Reagent, Siemens, Marberg, Germany) for use on the Sysmex CS2100i analyser (Milton Keynes, UK) and retrospectively compared the results with an in-house manual visual agglutination assay and VWF antigen (Siemens) in normal subjects and in 53 patients with various types of VWD and 23 patients following VWF therapeutic treatment. The intra and interassay CV was improved with the automated assay (2.3% and 3.8% respectively) compared to 7% with the manual VWF:RCo assay. Good correlation was found between the two assays (r=0.91) in 53 patients with VWD. The mean manual VWF:RCo was 0.25IU/ml and mean automated VWF:RCo was 0.27IU/ml. A comparable increase in VWF:RCo following treatment, mostly with Desmopressin, was found in 13 patients with type 1 VWD (mean 3.9 fold increase with manual VWF:RCo and 3.1 fold with the automated VWF:RCo). In 13 patients with type 2 or 3 VWD following treatment mostly with concentrate , a higher increase was found with the automated VWF:RCo assay than the manual assay (mean 11.9 fold manually and mean 20.3 automated). The automated VWF:RCo assay shows enhanced precision and analysis time in this difficult and time consuming laboratory test and its introduction should greatly improve the reliability of VWF testing. Copyright © 2010. Published by Elsevier Ltd.

  11. Adrenal Cortex-Sparing Surgery for Bilateral Multiple Pheochromocytomas in a Patient with Von Hippel-Lindau Disease

    Directory of Open Access Journals (Sweden)

    Tarık Esen

    2012-01-01

    Full Text Available Pheochromocytomas can be a part of familial neoplastic syndromes, in which case they tend to be multiple and involve both adrenal glands. Therefore, sparing adrenocortical function represents a major concern while dealing with these hereditary lesions. Herein, we describe the clinical characteristics and the management strategy of a patient with von Hippel-Lindau (VHL disease who had multiple, bilateral pheochromocytomas as well as bilateral renal masses, pancreatic masses, and a paracaval mass. Only a portion of the left adrenal gland has remained in situ after two consecutive open surgeries and a percutaneous radiofrequency ablation which have been performed to treat the various components of this syndrome. No adrenal or extra-adrenal pheochromocytoma recurrences have been detected during a follow-up period of more than 2 years. Pancreatic and adrenal functions were normal throughout the postoperative period and never necessitated any replacement therapy. Adrenal cortex-sparing surgery is a valid option for VHL disease patients who present with synchronous bilateral adrenal pheochromocytomas.

  12. Characterization of endolymphatic sac tumors and von Hippel-Lindau disease in the International Endolymphatic Sac Tumor Registry.

    Science.gov (United States)

    Bausch, Birke; Wellner, Ulrich; Peyre, Mathieu; Boedeker, Carsten C; Hes, Frederik J; Anglani, Mariagiulia; de Campos, Jose M; Kanno, Hiroshi; Maher, Eamonn R; Krauss, Tobias; Sansó, Gabriela; Barontini, Marta; Letizia, Claudio; Hader, Claudia; Schiavi, Francesca; Zanoletti, Elisabetta; Suárez, Carlos; Offergeld, Christian; Malinoc, Angelica; Zschiedrich, Stefan; Glasker, Sven; Bobin, Serge; Sterkers, Olivier; Ba Huy, Patrice Tran; Giraud, Sophie; Links, Thera; Eng, Charis; Opocher, Giuseppe; Richard, Stephane; Neumann, Hartmut P H

    2016-04-01

    Endolymphatic sac tumors (ELSTs) are, with a prevalence of up to 16%, a component of von Hippel-Lindau (VHL) disease. Data from international registries regarding heritable fraction and characteristics, germline VHL mutation frequency, and prevalence are lacking. Systematic registration of ELSTs from international centers of otorhinolaryngology and from multidisciplinary VHL centers' registries was performed. Molecular genetic analyses of the VHL gene were offered to all patients. Our population-based registry comprised 93 patients with ELST and 1789 patients with VHL. The prevalence of VHL germline mutations in apparently sporadic ELSTs was 39%. The prevalence of ELSTs in patients with VHL was 3.6%. ELST was the initial manifestation in 32% of patients with VHL-ELST. Prevalence of ELST in VHL disease is much lower compared to the literature. VHL-associated ELSTs can be the first presentation of the syndrome and mimic sporadic tumors, thus emphasizing the need of molecular testing in all presentations of ELST. © 2015 Wiley Periodicals, Inc. Head Neck 38: 673-679, 2016. © 2015 Wiley Periodicals, Inc.

  13. Elevated preoperative von Willebrand factor is associated with perioperative thrombosis in infants and neonates with congenital heart disease.

    Science.gov (United States)

    Hunt, R; Hoffman, C M; Emani, S; Trenor, C C; Emani, S M; Faraoni, D; Kimchi-Sarfaty, C; Ibla, J C

    2017-12-01

    Essentials Perioperative thrombosis is a major cause of morbidity and mortality in congenital heart disease. Neonates and infants undergoing repair of congenital heart lesions were prospectively followed. Elevated von Willebrand factor (VWF) to ADAMTS-13 activity ratios typified the postoperative period. Thrombosis was associated with preoperative VWF activity and cryoprecipitate transfusion SUMMARY: Background The surgical repair of congenital heart malformations is frequently complicated by perioperative thrombosis of unclear etiology. An imbalance between von Willebrand factor (VWF) and ADAMTS-13 is an emerging variable in thrombosis. Objectives To describe perioperative changes to VWF, ADAMTS-13 and NETosis, and evaluate clinical and biochemical associations with postoperative thrombosis. Methods Neonates and infants undergoing palliation or definitive surgical repair of congenital heart malformations were recruited (n = 133). Preoperative and postoperative plasma levels of VWF, ADAMTS-13 and markers of NETosis were determined. Patients were followed for up to 30 days for the occurrence of thrombosis. Univariate and multivariate logistic regression analyses were conducted to identify variables associated with thrombosis. Results We identified significant postoperative increases in VWF activity, VWF level, DNA-histone complexes and cell-free DNA with an overall decrease in ADAMTS-13 activity. Patients experiencing postoperative thrombotic events (9%) were characterized by surgery performed at a lower intraoperative temperature, higher preoperative lactic acid levels, and higher preoperative VWF activity and level. A multivariate logistic regression model identified preoperative VWF activity (odds ratio (OR) 8.39 per IU mL-1 , 95% confidence interval [CI] 1.73-40.55) and transfusion of cryoprecipitate (OR 1.10 per mL kg-1 , 95% CI 1.03-1.17) as being associated with thrombosis. Conclusions Pediatric patients undergoing surgical repair of congenital

  14. Response to desmopressin is influenced by the genotype and phenotype in type 1 von Willebrand disease (VWD): results from the European Study MCMDM-1VWD

    DEFF Research Database (Denmark)

    Castaman, G.; Lethagen, S.; Federici, A.B.

    2008-01-01

    We have prospectively evaluated the biologic response to desmopressin in 77 patients with type 1 von Willebrand disease (VWD) enrolled within the Molecular and Clinical Markers for the Diagnosis and Management of type 1 VWD project. Complete response to desmopressin was defined as an increase...... of subtle multimeric abnormalities did not hamper potential clinically useful responses, as in typical type 1 VWD Udgivelsesdato: 2008/4/1...

  15. [Surgical treatment of a pheocromocytoma bilateral in a 5 year old patient with the von Hippel-Lindau disease].

    Science.gov (United States)

    Blanco, J A; Blanco, D; Alastrue, A; Castellví, A; Isnard, R M; Pintos, G; Mangas, A; Roig, N; Casasa, J M

    2004-01-01

    The disease of Von Hippel Lindau (VHL) is hereditary and causes a predisposition to the development of tumours. Organs such as the cerebellum, the pancreas, the kidney, the suprarenal glands and the retina are more usually affected by this disease. We present the case of a 5-year-old patient who suffers from asiymptomatic high blood pressure. In the family antecedents, it is relevant the case of the father, with pheocromocytoma bilateral, which led us to carry out a genetic study of his two sons. Our patient, the younger; presented a mutation of the VHL gene in the short arm of the chromosome 3. In one of the periodic controls, it could be detected high blood pressure of 160/100 mm. Hg, clinically asymptomatic. The other child did not present a genetic mutation and has no disease. The presence of high catecholamines, the detection of a 3 cm left suprarenal mass through the ecography, the TAC that did not show a right suprarenal pathology and the MBIG scintigraphy confirmed the diagnostic of pheocromocytoma. The RNM showed another 0.8-cm mass which confirmed a pheocromocytoma bilateral. We started the treatment against high blood pressure with fenoxibenzamine and diltiazem, and we controlled this problem. We also prepared the pre-and-post operation anesthetic strategy, which is so important for the surgical success. The operation started by a laparoscopic, we made left adrenalectomy and we had to reconvert to laparotomy to make partial right adrenalectomy. Six months after the operation, the patient is free from symptomatology and follows a treatment with glucocorticoides with smaller and smaller doses. The case is exceptional because it embodies the following characteristics: early diagnostic age, family affectation and discovery of asymptomatic high blood pressure. It needed an appropriate preanesthetic and anesthetic preparation, which gave way to an operation without complications. The postoperation was also stable and presented no complications.

  16. Syringomyelia following surgery for a spontaneous spinal subdural hematoma in a 13-year-old girl with congenital von Willebrand disease: case report and literature review.

    Science.gov (United States)

    Ben Nsir, A; Boubaker, A; Jemel, H

    2016-04-01

    Spontaneous spinal subdural hematomas are rare. Their occurrence in a child with congenital von Willebrand disease and the complication of their surgery by a large secondary syringomyelia have never been previously reported. A 13-year-old girl with congenital von Willebrand disease presented to our emergency department in January 2011 for sudden onset of severe back pain centered in her thoracic spine rapidly aggravated by signs of acute myelopathy without any precipitating factor. MRI scan revealed a thoracic subdural collection anterior to the spinal cord at the T7-T9 level, hyperintense on T1- and T2-weighted sequences consistent with an acute spinal subdural hemorrhage. Evacuation of the subdural hematoma was realized immediately after hemostasis parameter correction, and post-operative course was uneventful with full functional recovery. One year later, the patient presented once again but with progressive and more severe myelopathy caused by a large syringomyelia extending from the T5 level to the conus medullaris. A syringopleural shunting was performed and the patient was unrolled under an intensive care and rehabilitation program. Her condition remarkably improved and she became able to walk independently within 2 weeks post-operatively. von Willebrand disease should be included as a possible factor of spontaneous spinal subdural hemorrhage. Surgery is advised in emergency and can be associated with remarkable recovery especially in children. Delayed syringomyelia can complicate the post-operative course and can be successfully addressed by syringopleural shunting. Long-term clinical and radiological follow-up is advocated.

  17. Pathological and Clinical Features and Management of Central Nervous System Hemangioblastomas in von Hippel-Lindau Disease

    Directory of Open Access Journals (Sweden)

    Hiroshi Kanno

    2014-08-01

    Full Text Available Central nervous system (CNS hemangioblastoma is the most common manifestation of von Hippel-Lindau (VHL disease. It is found in 70-80% of VHL patients. Hemangioblastoma is a rare form of benign vascular tumor of the CNS, accounting for 2.0% of CNS tumors. It can occur sporadically or as a familial syndrome. CNS hemangioblastomas are typically located in the posterior fossa and the spinal cord. VHL patients usually develop a CNS hemangioblastoma at an early age. Therefore, they require a special routine for diagnosis, treatment and follow-up. The surgical management of symptomatic tumors depends on many factors such as symptom, location, multiplicity, and progression of the tumor. The management of asymptomatic tumors in VHL patients is controversial since CNS hemangioblastomas grow with intermittent quiescent and rapid-growth phases. Preoperative embolization of large solid hemangioblastomas prevents perioperative hemorrhage but is not necessary in every case. Radiotherapy should be reserved for inoperable tumors. Because of complexities of VHL, a better understanding of the pathological and clinical features of hemangioblastoma in VHL is essential for its proper management.

  18. Endolymphatic sac tumor with von Hippel-Lindau disease: report of a case with atypical pathology of endolymphatic sac tumor.

    Science.gov (United States)

    Yang, Xiang; Liu, Xue-Song; Fang, Yuan; Zhang, Xiu-Hui; Zhang, Yue-Kang

    2014-01-01

    The authors described a case of a patient with co-existing endolymphatic sac tumor (ELST) and hemangioblastoma in the posterior cranial fossa, which belonged to a subtype of Von Hippel-Lindau (VHL) disease confirmed by the test of VHL-gene. The signs in this 42-year-old female included intermittent headache and dizziness. Imaging revealed a giant mass in the right cerebellopontine angle (CPA) region and another lesion in the left cerebellar hemisphere. The results of biopsy after two operations confirmed the diagnosis respectively. Both of the tumors were resected totally. Nevertheless, we had to confess the misdiagnosis as vascular tumor instead of ELST at the initial diagnosis because of the rarity of ELST associated with atypical histological characteristics. The purposes we reported this case were to describe the atypical pathological feature of ELST and the mutation of germline VHL not mentioned in previously literature, furthermore, to foster understanding of ELSTs with the avoidance of the similar misdiagnosis as far as possible in future.

  19. Posterior approach for giant S1 neurofibroma in von Recklinghausen′s disease: Is total resection realistic?

    Directory of Open Access Journals (Sweden)

    Ashish Kumar

    2013-01-01

    Full Text Available Bilateral sacral neurofibromas are uncommonly seen in neurofibromatosis type 1 (NF1 also known as Von Recklinghausen′s disease. They often grow to enormous dimensions before detection owing to bone scalloping. Resections of "giant" S1 neurofibromas are difficult due to the limitations of operative window in sacrum and critical functions associated with S1 nerve root. We report a case of bilateral S1 neurofibromas in a patient of NF1 where she had a giant left-sided neurofibroma with extensive bone erosion and a small fusiform neurofibroma on the right side. The tumor was excised completely on the left and near totally on the right side via posterior approach. There were no postoperative neurological deficits and the patient recovered well. Usually, complete excision harbors the chances of postoperative neurological deficits due to the eloquence of the nerve root involved and complete resection without significant morbidity seems unrealistic. However, excision of giant ones may not result in grave deficits always if the patient is neurologically intact before surgery. Also, in selected patients, only posterior approach may suffice for giant neurofibromas with extensive bone scalloping and complete removal can be attempted successfully despite narrow corridors.

  20. East African Medical Journal - Vol 82, No 1 (2005)

    African Journals Online (AJOL)

    Editorial: Safe Motherhood in Africa: Achievable goal or a dream? ZP Qureshi ... Socio-Demographic Correlates Of Bipolar Disorder In Butajira, Rural Ethiopia. D Kebede, A Alem, T Shibre, ... Malignant Peripheral Nerve Sheath Tumour Associated With Von Recklinghausen\\'s Disease: Case Report. JN Legbo, BB Shehu, SA ...

  1. Clinical and molecular features of familial and sporadic cases of von Hippel-Lindau disease from Mexico.

    Science.gov (United States)

    Chacon-Camacho, Oscar F; Rodriguez-Dennen, Fernando; Camacho-Molina, Alejandra; Rasmussen, Astrid; Alonso-Vilatela, Elisa; Zenteno, Juan C

    2010-04-01

    von Hippel-Lindau disease (VHL) is an uncommon autosomal dominant condition predisposing to the development of tumours in a variety of body organs and caused by germline mutations in VHL, a tumour suppressor gene located on 3p. Up to 60% of VHL patients show ocular involvement with retinal hemangioblastoma being the most common observed lesion. In this study, we describe the clinical and genetic characteristics of two familial and one apparently non-familial case of VHL ascertained at our institution. Clinical evaluation included ophthalmologic examination and imaging exams for tumours identification; molecular analysis consisted of PCR amplification of the complete VHL gene coding sequence (three exons) and automated nucleotide sequencing. A total of eight affected subjects were demonstrated to carry a causative mutation in VHL. Affected subjects from family #1 had a c.245G > C change, predicting a p.R82P substitution, affected individuals from family #2 were shown to have a c.266T > C change, leading to a p.L89P missense substitution, whereas the apparently non-familial case had a c.298-299insA mutation. One subject from family #2 was a non-penetrant carrier. No ocular anomalies were found in two adult affected subjects carrying the p.L89P mutation. Considerable interfamilial and intrafamilial clinical variability as well as one instance of non penetrance were recorded in these VHL disease cases. Three different mutations were demonstrated, including the c.298-299insA one base insertion, which has been previously described in two unrelated families from our country. Although additional studies are needed, our data suggest that this insertion could be a 'founder' mutation.

  2. Perioperative outcomes of syndromic paraganglioma and pheochromocytoma resection in patients with von Hippel-Lindau disease, multiple endocrine neoplasia type 2, or neurofibromatosis type 1.

    Science.gov (United States)

    Butz, James J; Yan, Qi; McKenzie, Travis J; Weingarten, Toby N; Cavalcante, Alexandre N; Bancos, Irina; Young, William F; Schroeder, Darrell R; Martin, David P; Sprung, Juraj

    2017-12-01

    Pheochromocytoma and/or paraganglioma associated with neurofibromatosis type 1, multiple endocrine neoplasia type 2A, and von Hippel-Lindau disease have different catecholamine biochemical phenotypes. We examined perioperative outcomes of pheochromocytoma/paraganglioma resection in 3 syndromic forms. Retrospective review of patients undergoing resection of syndromic pheochromocytoma/paraganglioma from 2000 through 2016. Eighty-one patients underwent pheochromocytoma/paraganglioma resection (multiple endocrine neoplasia type 2A, n = 36; neurofibromatosis type 1, n = 26; von Hippel-Lindau disease, n = 19). Tumor size differed across groups; patients with neurofibromatosis type 1 and von Hippel-Lindau disease had the largest tumors (P = .017). Larger tumor volumes correlated with higher urine 24-hour total metanephrine (r = 0.94, P neoplasia type 2A, von Hippel-Lindau disease, and neurofibromatosis type 1, respectively). High adrenergic secretion (24-hour urine metanepinephrine) was found in neurofibromatosis type 1 (median, 861 μg/24 h), similar to that found in multiple endocrine neoplasia type 2A (median, 809 μg/24 h). The highest noradrenergic secretion (24-hour urine normetanephrine) occurred with von Hippel-Lindau disease (median, 4,598 μg/24 h), followed by neurofibromatosis type 1 and multiple endocrine neoplasia type 2A (median, 1,607 and 923 μg/24 h, respectively). The highest graded complications occurred among patients with neurofibromatosis type 1 (P = .036). However, when comparing postoperative outcomes across 3 groups in those who had laparoscopic resection, there was no significant difference (P = .955). Patients with neurofibromatosis type 1 had the most volatile intraoperative hemodynamic course and more severe postoperative complications. These complications are related to large tumors associated with abundant catecholamine secretion and the fact that a high proportion underwent open resection. Among only patients who underwent

  3. Identification and Characterization of Novel Variations in Platelet G-Protein Coupled Receptor (GPCR Genes in Patients Historically Diagnosed with Type 1 von Willebrand Disease.

    Directory of Open Access Journals (Sweden)

    Jacqueline Stockley

    Full Text Available The clinical expression of type 1 von Willebrand disease may be modified by co-inheritance of other mild bleeding diatheses. We previously showed that mutations in the platelet P2Y12 ADP receptor gene (P2RY12 could contribute to the bleeding phenotype in patients with type 1 von Willebrand disease. Here we investigated whether variations in platelet G protein-coupled receptor genes other than P2RY12 also contributed to the bleeding phenotype. Platelet G protein-coupled receptor genes P2RY1, F2R, F2RL3, TBXA2R and PTGIR were sequenced in 146 index cases with type 1 von Willebrand disease and the potential effects of identified single nucleotide variations were assessed using in silico methods and heterologous expression analysis. Seven heterozygous single nucleotide variations were identified in 8 index cases. Two single nucleotide variations were detected in F2R; a novel c.-67G>C transversion which reduced F2R transcriptional activity and a rare c.1063C>T transition predicting a p.L355F substitution which did not interfere with PAR1 expression or signalling. Two synonymous single nucleotide variations were identified in F2RL3 (c.402C>G, p.A134 =; c.1029 G>C p.V343 =, both of which introduced less commonly used codons and were predicted to be deleterious, though neither of them affected PAR4 receptor expression. A third single nucleotide variation in F2RL3 (c.65 C>A; p.T22N was co-inherited with a synonymous single nucleotide variation in TBXA2R (c.6680 C>T, p.S218 =. Expression and signalling of the p.T22N PAR4 variant was similar to wild-type, while the TBXA2R variation introduced a cryptic splice site that was predicted to cause premature termination of protein translation. The enrichment of single nucleotide variations in G protein-coupled receptor genes among type 1 von Willebrand disease patients supports the view of type 1 von Willebrand disease as a polygenic disorder.

  4. 3 tesla magnetic resonance imaging in children and adults with congenital heart disease; 3-Tesla-Magnetresonanztomographie zur Untersuchung von Kindern und Erwachsenen mit angeborenen Herzfehlern

    Energy Technology Data Exchange (ETDEWEB)

    Voges, I.; Hart, C.; Kramer, H.H.; Rickers, C. [Universitaetsklinikum Schleswig-Holstein, Klinik fuer angeborene Herzfehler und Kinderkardiologie, Kiel (Germany); Jerosch-Herold, M. [Harvard Medical School, Department of Radiology, Brigham and Women' s Hospital, Boston (United States); Helle, M. [Universitaetsklinikum Schleswig-Holstein, Institut fuer Neuroradiologie, Kiel (Germany)

    2010-09-15

    Cardiovascular magnetic resonance imaging (CMR) has become a routinely used imaging modality for congenital heart disease. A CMR examination allows the assessment of thoracic anatomy, global and regional cardiac function, blood flow in the great vessels and myocardial viability and perfusion. In the clinical routine cardiovascular MRI is mostly performed at field strengths of 1.5 Tesla (T). Recently, magnetic resonance systems operating at a field strengths of 3 T became clinically available and can also be used for cardiovascular MRI. The main advantage of CMR at 3 T is the gain in the signal-to-noise ratio resulting in improved image quality and/or allowing higher acquisition speed. Several further differences compared to MRI systems with lower field strengths have to be considered for practical applications. This article describes the impact of CMR at 3 T in patients with congenital heart disease by meanings of methodical considerations and case studies. (orig.) [German] Die kardiovaskulaere Magnetresonanztomographie (MRT) hat sich zu einer etablierten bildgebenden Methode zur Untersuchung von Patienten mit angeborenen Herzfehlern entwickelt. Sie erlaubt in einer einzigen Untersuchung die exakte Beurteilung von Anatomie, globaler und regionaler Funktion, Blutfluessen sowie der myokardialen Perfusion und Vitalitaet. In der klinischen Routine erfolgen die Untersuchungen zumeist bei einer Feldstaerke von 1,5 Tesla (T), mittlerweile gibt es jedoch Geraete und Bildgebungstechniken, die die kardiovaskulaere MRT auch bei 3 T ermoeglichen. Der wesentliche Vorteil der MRT bei 3 T ist das hoehere Signal-zu-Rausch-Verhaeltnis, das sowohl zu einer Verbesserung der Bildqualitaet als auch zu einer Verkuerzung der Untersuchungszeit genutzt werden kann. Darueber hinaus bestehen verschiedene andere Unterschiede gegenueber Systemen mit niedriger Feldstaerke, die im praktischen Einsatz beachtet werden muessen. Dieser Artikel beschreibt die Erfahrungen der 3-T-MRT fuer die

  5. Diseases of the peritoneum and mesenterium; Erkrankungen von Peritoneum und Mesenterium

    Energy Technology Data Exchange (ETDEWEB)

    Ba-Ssalamah, A.; Uffmann, M.; Bastati, N.; Schima, W. [Medizinische Universitaet Wien, Universitaetsklinik fuer Radiodiagnostik, Wien (Austria)

    2009-07-15

    Peritoneal diseases can be seen in the different imaging modalities either as fluid collections or solid tumors along the ligaments, mesenteries, and spaces of the peritoneal cavity. The broad spectrum of different abnormalities includes inflammatory, infectious, traumatic, and neoplastic diseases. In this article, a large variety of peritoneal abnormalities such as ascites, peritonitis, intraperitoneal hemorrhage, and both primary and secondary peritoneal tumors are discussed. The different imaging modalities, characteristic radiological features, and typical pathways of anatomic spread are explained. (orig.) [German] Peritoneale Erkrankungen koennen sich in den verschiedenen Bildgebungsmodalitaeten entweder als Gas-/Fluessigkeitsansammlung oder als weichteildichte Gewebevermehrung entlang der verschiedenen Ligamente und Mesenterien der Peritonealhoehle manifestieren. Dieses breite Spektrum der pathologischen Veraenderungen beinhaltet entzuendliche, infektioese, neoplastische und verschiedenste Erkrankungen anderer Genese. In vorliegendem Artikel wird ein grosses Spektrum dieser Pathologien wie Aszites, Peritonitis, intraabdominelle Blutung und verschiedene primaere und sekundaere peritoneale Tumoren vorgestellt. Des Weiteren wird der Einsatz der verschiedenen radiologischen Untersuchungsmodalitaeten, v. a. der Computertomographie (CT) als wichtigster Untersuchungsmethode, erlaeutert. Die charakteristischen Bildgebungsmerkmale und die typischen anatomischen Ausbreitungswege werden erklaert. (orig.)

  6. Anterolateral meningocele

    Energy Technology Data Exchange (ETDEWEB)

    Rapf, C.

    1986-08-01

    The author gives a detailed description of diagnosis and differential diagnosis of a thoracic lateral meningocele associated with known neurofibromatosis (von Recklinghausen's disease), using plain roentgenography, myelography and computed tomography.

  7. Transcriptional repression of the Neurofibromatosis-1 tumor suppressor by the t(8;21) fusion protein.

    NARCIS (Netherlands)

    Yang, G.; Khalaf, W.; Locht, L.; Jansen, J.H.; Gao, M.; Thompson, M.A.; Reijden, B.A. van der; Gutmann, D.H.; Delwel, R.; Clapp, D.W.; Hiebert, S.W.

    2005-01-01

    Von Recklinghausen's disease is a relatively common familial genetic disorder characterized by inactivating mutations of the Neurofibromatosis-1 (NF1) gene that predisposes these patients to malignancies, including an increased risk for juvenile myelomonocytic leukemia. However, NF1 mutations are

  8. Elevated levels of plasma von Willebrand factor and the risk of macro- and microvascular disease in type 2 diabetic patients with microalbuminuria

    DEFF Research Database (Denmark)

    Gaede, P; Vedel, P; Parving, H H

    2001-01-01

    according to baseline plasma von Willebrand factor levels below or above the median. The main outcome was cardiovascular disease (cardiovascular mortality, non-fatal stroke, non-fatal myocardial infarction, coronary artery bypass graft and revascularization or amputation of legs), progression to diabetic...... nephropathy or progression in diabetic retinopathy. RESULTS: At baseline the two groups were comparable for HbA(1c), fasting levels of s-total-cholesterol, s-HDL-cholesterol and s-triglycerides, systolic and diastolic blood pressure, gender, known diabetes duration, smoking habits, previous cardiovascular...

  9. Cesariana em paciente com doença de von Willebrand associada à infecção pelo HIV: relato de caso Cesárea en paciente con enfermedad de von Willebrand asociada a la infección por el HIV: relato de caso Anesthesia for cesarean section in patient with von Willebrand's disease and HIV infection: case report

    Directory of Open Access Journals (Sweden)

    Vanessa Rezende Balle

    2004-12-01

    Willebrand y HIV positiva sometida a cesárea. RELATO DEL CASO: Paciente de 24 años, portadora de anemia microcítica, enfermedad de von Willebrand y HIV, llegó a la emergencia obstétrica en inicio de trabajo de alumbramiento. No realizó prenatal. Fue indicada cesárea a fin de disminuir los riesgos de transmisión vertical en paciente con carga vírica de HIV desconocida. Presentaba hematomas por el cuerpo e historia de hematoma de pared abdominal en cesárea anterior. Los tests de coagulación estaban un poco alterados. Después de infusión de concentrado de factor VIII fue realizada anestesia general. Madre y recién nacido presentaron evolución satisfactoria. CONCLUSIONES: La evaluación de manifestaciones clínicas en pacientes con coagulopatia es fundamental en la decisión del tipo de anestesia que será indicada para cada paciente. La evaluación debe ser individualizada, considerando los riesgos y beneficios de la técnica escogida. En estas pacientes, se debe siempre restringir al máximo la indicación de interrupción de la gestación por vía alta, optándose siempre por los métodos menos invasivos. La terapia con concentrado de factor VIII es actualmente la mejor opción de tratamiento, corrigiendo la deficiencia específica y dismunuyendo los riesgos de transmisión vírica.BACKGROUND AND OBJECTIVES: Von Willebrand's disease is the most common hereditary coagulation disorder in young women. The incidence of HIV infection among women has been progressively increasing, and vertical transmission may account for 25% of cases. This report aimed at describing the case of an HIV-positive patient with von Willebrand's disease scheduled for cesarean section. CASE REPORT: Female HIV-positive patient, 24 years old, with microcytic anemia and von Willebrand's disease, admitted to the emergency room in early labor. She had no pre-natal care. Cesarean section was indicated to lower vertical transmission risks since HIV viral count was unknown. Patient had hematomas

  10. Von Gierke disease

    Science.gov (United States)

    ... C. Edens Hurst, MD, MS, Assistant Professor in Medical Genetics, The University of Alabama at Birmingham, Birmingham, AL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A. ...

  11. Pancreatic neuroendocrine tumor with complete replacement of the pancreas by serous cystic neoplasms in a patient with von Hippel-Lindau disease: a case report.

    Science.gov (United States)

    Maeda, Shimpei; Motoi, Fuyuhiko; Oana, Shuhei; Ariake, Kyohei; Mizuma, Masamichi; Morikawa, Takanori; Hayashi, Hiroki; Nakagawa, Kei; Kamei, Takashi; Naitoh, Takeshi; Unno, Michiaki

    2017-09-25

    von Hippel-Lindau disease is a dominantly inherited multi-system syndrome with neoplastic hallmarks. Pancreatic lesions associated with von Hippel-Lindau include serous cystic neoplasms, simple cysts, and neuroendocrine tumors. The combination of pancreatic neuroendocrine tumors and serous cystic neoplasms is relatively rare, and the surgical treatment of these lesions must consider both preservation of pancreatic function and oncological clearance. We report a patient with von Hippel-Lindau disease successfully treated with pancreas-sparing resection of a pancreatic neuroendocrine tumor where the pancreas had been completely replaced by serous cystic neoplasms, in which pancreatic function was preserved. A 39-year-old female with von Hippel-Lindau disease was referred to our institution for treatment of a pancreatic neuroendocrine tumor. Abdominal computed tomography demonstrated a well-enhanced mass, 4 cm in diameter in the tail of the pancreas, and two multilocular tumors with several calcifications, 5 cm in diameter, in the head of the pancreas. There was complete replacement of the pancreas by multiple cystic lesions with diameters ranging from 1 to 3 cm. Magnetic resonance cholangiopancreatography showed innumerable cystic lesions on the whole pancreas and no detectable main pancreatic duct. Endoscopic ultrasound-guided fine-needle aspiration of the mass in the pancreatic tail showed characteristic features of a neuroendocrine tumor. A diagnosis of pancreatic neuroendocrine tumor in the tail of the pancreas and mixed-type serous cystic neoplasms replacing the whole pancreas was made and she underwent distal pancreatectomy while avoiding total pancreatectomy. The stump of the pancreas was sutured as firm as possible using a fish-mouth closure. The patient made a good recovery and was discharged on postoperative day 9. She is currently alive and well with no symptoms of endocrine or exocrine pancreatic insufficiency 8 months after surgery. A pancreas

  12. Haemostasis prophylaxis using single dose desmopressin acetate and extended use epsilon aminocaproic acid for adenotonsillectomy in patients with type 1 von Willebrand disease.

    Science.gov (United States)

    Santoro, C; Hsu, F; Dimichele, D M

    2012-03-01

    In patients with confirmed or suspected type 1 von Willebrand disease (VWD), adenotonsillectomy has been reported to be associated with a rate of peri-operative hemorrhage between 8 and 23%. Desmopressin acetate (DDAVP, 1-deamino 8-D arginine- vasopressin) is the treatment of choice for type 1 patients with baseline von Willebrand factor levels of 10 IU/dL or greater. DDAVP is generally well tolerated; however, severe hyponatremia and seizures have been reported in young children less than 2 years of age, limiting its use in this age group. Antifibrinolytic therapy plays an important adjunctive role in the effective treatment of mucocutaneous bleeding, particularly in the oropharynx where the salivary concentration of fibrinolytic enzymes is high. During the past 10 years, we treated 6 pediatric patients with mild/moderate type 1 VWD undergoing an adenotonsillar procedure at our institution with the same hemostatic regimen consisting of one single dose of DDAVP and an extended use of EACA. In this small case series, the above mentioned prophylactic treatment regimen was both well tolerated and efficacious in controlling hemorrhage. Furthermore, DDAVP-related complications were avoided in a pediatric population with a higher risk of developing them. © 2011 Blackwell Publishing Ltd.

  13. Anestesia para septoplastia e turbinectomia em paciente portador de doença de von Willebrand: relato de caso Anestesia para septoplastia y turbinectomia en paciente portador de enfermedad de von Willebrand: relato de caso Anesthesia for septoplasty and turbinectomy in von Willebrand disease patient: case report

    Directory of Open Access Journals (Sweden)

    Múcio Paranhos de Abreu

    2003-06-01

    OBJECTIVES: Although von Willebrand’s disease is the most common hereditary hemorrhagic disorder, there are few reports in Brazilian literature relating this disease to anesthesia. This report aimed at describing a case of general anesthesia for septoplasty and turbinectomy in a von Willebrand’s disease type I patient, prophylactically treated with desmopressin (1-deamine-8-D- arginine vasopressin, DDAVP in the pre and postoperative period. CASE REPORT: A female patient, 19 years old, 58 kg, with hypothyroidism controlled with L-tiroxine (75 mg had her von Willebrand’s disease manifested three years before after a wisdom tooth extraction with persistent bleeding in the postoperative period. To prevent new per and postoperative hemorrhagic episodes, patient was prophylactically treated with desmopressin (0.3 µg.kg-1. Anesthesia was induced with midazolam (2.5 mg, fentanyl (150 µg, droperidol (2.5 mg, lidocaine (60 mg, atracurium (30 mg and metoprolol (4 mg, followed by tracheal intubation and ventilation under intermittent positive pressure. Anesthesia was maintained with 2% sevoflurane in a mixture of 50% oxygen and nitrous oxide. This technique provided a good heart rate and blood pressure control during surgery. Patient remained with a nasal tampon for 24 hours and no bleeding was observed at its removal. Patient was discharged the day after surgery uneventfully. There were no immediate or late postoperative bleeding. CONCLUSIONS: The prophylactic treatment with DDAVP associated to the anesthetic technique used in this case was effective in controlling peri and postoperative bleeding.

  14. Choroba von Willebranda – najczęstsza osoczowa skaza krwotoczna

    Directory of Open Access Journals (Sweden)

    Bożena Sokołowska

    2010-08-01

    Full Text Available Von Willebrand disease is the most common hereditary haemorrhagic disorder. A majority of patients (70%have classic mild or moderate (type I von Willebrand disease, and women are mostly affected. Clinical manifestationsinclude menorrhagia and post partum bleeding.

  15. Interaktive Visualisierung von Strukturmechaniksimulationen

    OpenAIRE

    Sommer, Ove

    2003-01-01

    A) Motivation und Problemstellung Der Computer ist heute eines der wichtigsten Hilfsmittel beim Entwurf und der Entwicklung von Fahrzeugen. Zunächst wurde er von Konstrukteuren für das Computer Aided Design (CAD) von virtuellen Fahrzeugmodellen eingesetzt. Inzwischen ist er in vielen anderen Bereichen der Fahrzeugentwicklung unentbehrlich geworden: der Entwicklungszyklus von Automobilen ist durch die Computer-gestützte numerische Simulation substanziell verkürzt worden. An virtuellen Prot...

  16. Phase II prospective open-label trial of recombinant interleukin-11 in women with mild von Willebrand disease and refractory menorrhagia.

    Science.gov (United States)

    Ragni, Margaret V; Jankowitz, Rachel C; Jaworski, Kristen; Merricks, Elizabeth P; Kloos, Mark T; Nichols, Timothy C

    2011-10-01

    Lack of effective treatment for menorrhagia is the greatest unmet healthcare need in women with von Willebrand disease (VWD). We conducted a single-centre phase II clinical trial to determine efficacy and safety of recombinant IL-11 (rhIL-11, Neumega®) given subcutaneously for up to seven days during six consecutive menstrual cycles each in seven women with mild VWD and menorrhagia refractory to haemostatic or hormonal agents. rhIL-11 reduced menstrual bleeding severity as measured by pictorial blood assessment chart (PBAC) ≥ 50% (to 0.05. Platelet VWF mRNA expression by quantitative PCR increased mean four-fold (1.0-13.5). rhIL-11 was well tolerated with grade 1 or less fluid retention, flushing, conjunctival erythema, and local bruising. In summary, rhIL-11 reduces menorrhagia safely and warrants further study.

  17. Phase II prospective open-label trial of recombinant interleukin-11 in women with mild von Willebrand disease and refractory menorrhagia*

    Science.gov (United States)

    Ragni, Margaret V.; Jankowitz, Rachel C.; Jaworski, Kristen; Merricks, Elizabeth P.; Kloos, Mark T.; Nichols, Timothy C.

    2014-01-01

    Summary Lack of effective treatment for menorrhagia is the greatest unmet healthcare need in women with von Willebrand disease (VWD). We conducted a single-centre phase II clinical trial to determine efficacy and safety of recombinant IL-11 (rhIL-11, Neumega®) given subcutaneously for up to seven days during six consecutive menstrual cycles each in seven women with mild VWD and menorrhagia refractory to haemostatic or hormonal agents. rhIL-11 reduced menstrual bleeding severity as measured by pictorial blood assessment chart (PBAC) ≥50% (to 0.05. Platelet VWF mRNA expression by quantitative PCR increased mean four-fold (1.0–13.5). rhIL-11 was well tolerated with grade 1 or less fluid retention, flushing, conjunctival erythema, and local bruising. In summary, rhIL-11 reduces menorrhagia safely and warrants further study. PMID:21833452

  18. von Willebrand disease type 1 in Doberman Pinscher dogs: genotyping and prevalence of the mutation in the Buenos Aires region, Argentina.

    Science.gov (United States)

    Crespi, Julian A; Barrientos, Laura S; Giovambattista, Guillermo

    2018-03-01

    von Willebrand disease (vWD) is the most common inherited coagulopathy in dogs, particularly in Doberman Pinschers. We developed a pyrosequencing-based assay to estimate the frequency of the c.7437G>A mutation associated with vWD type 1 in the Doberman Pinscher population of Buenos Aires, Argentina. We found a 0.41 frequency for the mutated allele, which varied significantly within families (family 1 = 0.43, family 2 = 0.58, unrelated animals = 0.35). The use of a popular founder male carrier of mutant allele A increased vWD incidence within a family and in the general population. The mode of inheritance was confirmed as autosomal dominant with incomplete penetrance. No differences were found between sexes and coat colors. Pyrosequencing was a good complement to clinical and coagulation tests for vWD type 1 diagnosis and a useful alternative for detecting the c.7437G>A mutation.

  19. Antifibrinolytic therapy for preventing oral bleeding in patients with haemophilia or Von Willebrand disease undergoing minor oral surgery or dental extractions.

    Science.gov (United States)

    van Galen, Karin P M; Engelen, Eveline T; Mauser-Bunschoten, Evelien P; van Es, Robert J J; Schutgens, Roger E G

    2015-12-24

    Minor oral surgery or dental extractions (oral or dental procedures) are widely performed and can be complicated by hazardous oral bleeding, especially in people with an inherited bleeding disorder such as haemophilia or Von Willebrand disease. The amount and severity of singular bleedings depend on disease-related factors, such as the severity of the haemophilia, both local and systemic patient factors (such as periodontal inflammation, vasculopathy or platelet dysfunction) and intervention-related factors (such as the type and number of teeth extracted or the dimension of the wound surface). Similar to local haemostatic measures and suturing, antifibrinolytic therapy is a cheap, safe and potentially effective treatment to prevent bleeding complications in individuals with bleeding disorders undergoing oral or dental procedures. However, a systematic review of trials reporting outcomes after oral surgery or a dental procedure in people with an inherited bleeding disorder, with or without, the use of antifibrinolytic agents has not been performed to date. The primary objective was to assess the efficacy of local or systemic use of antifibrinolytic agents to prevent bleeding complications in people with haemophilia or Von Willebrand disease undergoing oral or dental procedures. Secondary objectives were to assess if antifibrinolytic agents can replace or reduce the need for clotting factor concentrate therapy in people with haemophilia or Von Willebrand disease and to further establish the effects of these agents on bleeding in oral or dental procedures for each of these populations. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register, compiled from electronic database searches of the Cochrane Central Register of Controlled Trials (CENTRAL), of MEDLINE and from handsearching of journals and conference abstract books. We additionally searched the reference lists of relevant articles and reviews. We searched Pub

  20. Von Willebrand factor and aging.

    Science.gov (United States)

    Konkle, Barbara A

    2014-09-01

    von Willebrand factor (VWF) plays critical roles in initiating primary hemostasis and extending the half-life of coagulation factor VIII in circulation. VWF levels increase with age and elevated levels are associated with an increased risk of venous thromboembolism and cardiovascular disease (CVD). Patients with von Willebrand disease (VWD) due to a deficiency or dysfunction of VWF may have symptoms that ameliorate with aging or may have exacerbation of their disease. Bleeding sites of particular challenge in the aging patient include gastrointestinal bleeding and hematuria. Some medications used to treat VWD should be used with special precaution in older patients, including desmopressin and VWF-containing factor concentrates. Patients with VWD may have some protection from CVD, but in those patients who develop CVD, management is very challenging, given the role of antiplatelet therapy as the mainstay of treatment. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  1. Functional Imaging of Paragangliomas with an Emphasis on Von Hippel–Lindau-Associated Disease: A Mini Review

    Directory of Open Access Journals (Sweden)

    Ioannis Ilias

    2017-09-01

    Full Text Available Few reports have presented data and results on functional (i.e., nuclear medicine imaging of paragangliomas and pheochromocytomas (PGLs/PHEOs for von Hippel–Lindau (VHL patients. Nuclear medicine localization modalities for chromaffin tumors can be specific or nonspecific. Specific methods make use of the expression of the human norepinephrine transporter (hNET and vesicular monoamine transporters (VMATs by these tumors. These permit the use of radiolabeled ligands that enter the synthesis and storage pathway of catecholamines. Nonspecific methods are not related to the synthesis, uptake, or storage of catecholamines but make use of the tumors’ high glucose metabolism or expression of somatostatin receptors. Consensuses and guidelines suggest that metastatic and sporadic PHEOs/PGLs in VHL patients (as in patients with chromaffin tumors of yet unknown genotype should be evaluated first with 18F-dihydroxyphenylalanine (18F-DOPA positron emission tomography/computed tomography (PET/CT. The functional imaging of second choice is 123I-metaiodobenzylguanidine (123I-MIBG for PHEOs in VHL patients. 123I-MIBG, 68Ga-DOTATATE/DOTATOC/DOTANOC PET/CT, or 18F-fluorodeoxyglucose (18F-FDG PET/CT can be a second choice of functional imaging for PGLs in VHL patients.

  2. Lernen von und bei Max von Laue

    Science.gov (United States)

    Thiessen, P. A.

    Erinnerungen an die Entstehungsgeschichte und die Frühzeiten der Röntgenstrukturanalyse.Translated AbstractLearning at Max von LaueRemembrances of the historical rise and the early times of X-ray structure analysis.

  3. Effect of outdoor and indoor air pollution on lung function, bronchial hyperresponsiveness and risk of pulmonary disease of adults; Langzeitevaluation von Umwelteinfluessen auf Lungenfunktion, bronchiale Hyperreagibilitaet und Krankheitsrisiko von Erwachsenen

    Energy Technology Data Exchange (ETDEWEB)

    Eltschka, R. [Freiburg Univ. (Germany). Zentrum Innere Medizin

    1992-10-01

    The effect of outdoor exposure to SO{sub 2}, NO{sub 2}, particulate pollutants, ozone and pollen on pulmonary function and respiratory discomfort was studied over a period of 1 1/2 yr in 133 no smoking adults at the city of Freiburg. Concentration of indoor and outdoor pollutants were measured. Between January and December 1989 we found in comparison with other large towns low levels of SO{sub 2}, NO{sub 2} and particulate combustion products. The evaluation of individual exposure to air pollutants with lung function measurements shows that participants with essential hyperreactivity demonstrate a significant decrease of the FEV1/IVC - ratio with a threshold value of about 30 {mu}g/m{sup 3} NO{sub 2}. The analyses of the longitudinal study shows a significant correlation between daily peakflow measurements and ozone levels in patients with symptomatic asthma bronchiale and chronic obstructive pulmonary disease. For pollen, SO{sub 2}, NO{sub 2} and anorganic particulate pollutants we did not find any significant effects on peak expiratory flow measurements. (orig./MG) [Deutsch] Der Einfluss von Aussenluftschadstoffen (SO{sub 2}, NO{sub 2}, Staub und Ozon) auf die Lunge wurde in Freiburg in Form einer 1 1/2-jaehrigen Longitudinalstudie bei 133 nicht rauchenden Erwachsenen untersucht. Waehrend des Beobachtungszeitraumes 10/88 - 4/90 erfolgte die Messung der Aussen- und Innenluftschadstoffe. Die Messungen des DW und der LFU zeigen fuer den Zeitraum Januar-Dezember 1989 im Vergleich zu anderen Grossstaedten niedrige Schwefeldioxid-, Stickoxid- und Staubkonzentrationen. Die Querschnittsuntersuchung mit Anamnesefragebogen, Bodyplethysmographie und inhalativem Provokationstest zeigt bei Teilnehmern mit essentieller Hyperreagibilitaet eine signifikante Abnahme des gemessenen und adjustierten Tiffeneau-Index durch NO{sub 2} mit einem Schwellenwert bei 30 {mu}g/m{sup 3}. Die multivariate Analyse der Longitudinalstudie zeigt bei Patienten mit symptomatischem Asthma und

  4. Parallel Regulation of von Hippel-Lindau Disease by pVHL-Mediated Degradation of B-Myb and Hypoxia-Inducible Factor α

    Science.gov (United States)

    Uematsu, Keiji; Byrne, Stuart D.; Hirano, Mie; Joo-Okumura, Akiko; Nishikimi, Akihiko; Shuin, Taro; Fukui, Yoshinori; Nakatsukasa, Kunio

    2016-01-01

    pVHL, the protein product of the von Hippel-Lindau (VHL) tumor suppressor gene, is a ubiquitin ligase that targets hypoxia-inducible factor α (HIF-α) for proteasomal degradation. Although HIF-α activation is necessary for VHL disease pathogenesis, constitutive activation of HIF-α alone did not induce renal clear cell carcinomas and pheochromocytomas in mice, suggesting the involvement of an HIF-α-independent pathway in VHL pathogenesis. Here, we show that the transcription factor B-Myb is a pVHL substrate that is degraded via the ubiquitin-proteasome pathway and that vascular endothelial growth factor (VEGF)- and/or platelet-derived growth factor (PDGF)-dependent tyrosine 15 phosphorylation of B-Myb prevents its degradation. Mice injected with B-Myb knockdown 786-O cells developed dramatically larger tumors than those bearing control cell tumors. Microarray screening of B-Myb-regulated genes showed that the expression of HIF-α-dependent genes was not affected by B-Myb knockdown, indicating that B-Myb prevents HIF-α-dependent tumorigenesis through an HIF-α-independent pathway. These data indicate that the regulation of B-Myb by pVHL plays a critical role in VHL disease. PMID:27090638

  5. Cuidados nos pacientes com hemofilia e doença de von Willebrand na cirurgia eletiva otorrinolaringológica Otolaryngology surgery: management of elective surgery in patients with haemophilia and von Willebrand disease

    Directory of Open Access Journals (Sweden)

    Marise P. C. Marques

    2003-01-01

    Full Text Available FORMA DE ESTUDO Clínico prospectivo. MATERIAL E MÉTODO: Foi realizado um estudo prospectivo de 10 anos de 20 pacientes com hemofilias ou doença de von Willebrand (DvW com indicação de cirurgia otorrinolaringológica. Os pacientes foram submetidos a um total de 25 cirurgias otorrinolaringológicas eletivas. A idade média foi de 23,75 anos (2 a 62 anos. O grupo de estudo consistiu em 14 hemofílicos, 11 com hemofilia A grave (1 do sexo feminino, uma portadora com 30% de atividade de fator VIII (FVIII, um hemofílico B leve e uma com deficiência grave de fator X; 6 com DvW, 4 tinham o tipo 1 (3 mulheres, um o tipo 2A e um o tipo 3. Treze hemofílicos tinham síndrome de imunodeficiência adquirida. A duração média do procedimento foi de 1 hora e 37 minutos (15 minutos a 12 horas. O defeito da coagulação foi corrigido com desmopressina (DDAVP, com concentrado de FVIII de pureza intermediária 8Y, com criopreciptado ou com complexo protrombínico não ativado (PPSB, de acordo com os níveis plasmáticos do fator e da severidade da cirurgia. O ácido épsilon aminocapróico também foi usado em associação. Em 1 hemofílico A grave houve sangramento pós-operatório que se resolveu com a elevação do nível mínimo de FVIII para 80% e em 1 paciente com DvW do Tipo 3 houve sangramento pós-operatório pela dificuldade de identificação do melhor concentrado a ser reposto. Após o uso do concentrado de pureza intermediária 8Y, houve controle do sangramento. RESULTADO: Todos os outros pacientes apresentaram a hemostasia considerada normal ou excelente. CONCLUSÃO: Concluiu-se que pacientes com hemofilias ou DvW não apresentam um risco cirúrgico aumentado se for realizada uma terapia adequada.STUD DESIGN: Clinical prospective. MATERIAL AND METHOD: A 10-year prospective research was conducted in 20 patients with hemophilia or von Willebrand disease (vWD. They were submitted to a total of 25 elective otolaryngological surgical events. The

  6. Trends in clinical management of women with von Willebrand disease: a survey of 75 women enrolled in haemophilia treatment centres in the United States.

    Science.gov (United States)

    Kirtava, A; Crudder, S; Dilley, A; Lally, C; Evatt, B

    2004-03-01

    To assess the management of women with von Willebrand disease( vWD) in an Heamophilia Treatment Center (HTC) setting. A total of 75 women with vWd who were registered in HTCs in the United States participated in this study. A telephone interview elicited information about symptoms pertaining to bleeding disorders, diagnostic issues, referral patterns, treatment modalities before and after the enrollment in the HTC, HTC services provided, and satisfaction with care in the HTC. Menorrhagia was the most commonly reported symptom (84%). The average time from the first symptom until clinician recognition was 16 years (range 0-39). In HTC, DDAVP was the most commonly used drug (31%). Of the 75 women, 71 reported a strong positive opinion and satisfaction about their care in the HTCs. Women with VWD were typically diagnosed with the condition well into adulthood, in spite of the fact that majority of them experienced several bleeding symptoms beginning in early childhood. In general an HTC setting is appropriate for management of women with bleeding disorders. Diagnosis, treatment and education provided in the HTCs were viewed positively by those surveyed.

  7. Two childhood pheochromocytoma cases due to von Hippel -Lindau disease, one associated with pancreatic neuroendocrine tumor; a rare manifestation.

    Science.gov (United States)

    Dağdeviren Çakır, Aydilek; Turan, Hande; Aykut, Ayça; Durmaz, Asude; Ercan, Oya; Evliyaoğlu, Olcay

    2017-10-12

    (VHL) disease is an autosomal dominantly inherited disorder characterized by hemangioblastomas of retina and central nervous system (CNS); renal cysts, clear cell carcinoma; PCC; endolymphatic sac tumors; cystadenomas of the epididymis in males, broad ligament of uterus in females; pancreatic cysts, cystadenomas and neuroendocrine tumors. We here report two cases of VHL disease presented with PCC as the first manifestation. Hemangioblastoma of CNS in the first case and PNET in the second case developed during follow- up and led to the diagnosis of VHL disease. Genetic analyses of cases revealed p.Arg161Gln (c.482G>A) and p.Leu129Pro(c.386T>G) heterozygous missense mutation in VHL gene, respectively. In children, PCC may be the only and/or initial manifestation of the disease with delayed manifestations of the syndrome in other organs. PNET is a very rare manifestation of VHL disease. To best of our knowledge, this is the second case in literature, presenting with combination of PNET and bilateral PCC as components of childhood VHL disease. Pediatric patients diagnosed with PCC should be investigated for the genetic causes especially for VHL.

  8. De ziekte van Von Hippel-Lindau

    NARCIS (Netherlands)

    Los, M.; Links, T.P.; Lenders, J.W.M.; Voest, E.E.

    2000-01-01

    Von Hippel-Lindau disease (VHL) is an autosomal dominant inherited cancer syndrome. The disease was diagnosed in three patients: a 22-year-old woman who presented with decreased vision due to retinal angiomatosis and in whom a renal carcinoma was diagnosed five years later at a routine VHL analysis,

  9. Through the looking glass: an exploratory study of the lived experiences and unmet needs of families affected by Von Hippel-Lindau disease.

    Science.gov (United States)

    Kasparian, Nadine A; Rutstein, Alison; Sansom-Daly, Ursula M; Mireskandari, Shab; Tyler, Janet; Duffy, Jessica; Tucker, Katherine M

    2015-01-01

    Despite well-established protocols for the medical management of Von Hippel-Lindau disease (VHL), families affected by this rare tumour syndrome continue to face numerous psychological, social, and practical challenges. To our knowledge, this is one of the first qualitative studies to explore the psychosocial difficulties experienced by families affected by VHL. A semi-structured interview was developed to explore patients' and carers' experiences of VHL along several life domains, including: self-identity and self-esteem, interpersonal relationships, education and career opportunities, family communication, physical health and emotional well-being, and supportive care needs. Quantitative measures were also used to examine the prevalence of anxiety, depression, and disease-specific distress in this sample. Participants were recruited via the Hereditary Cancer Clinic at the Prince of Wales Hospital in Sydney, Australia. A total of 23 individual telephone interviews were conducted (15 patients, 8 carers), yielding a response rate of 75%. A diverse range of experiences were reported, including: sustained uncertainty about future tumour development, frustration regarding the need for lifelong medical screening, strained family relationships, difficulties communicating with others about VHL, perceived social isolation and limited career opportunities, financial and care-giving burdens, complex decisions in relation to childbearing, and difficulties accessing expert medical and psychosocial care. Participants also provided examples of psychological growth and resilience, and voiced support for continued efforts to improve supportive care services. More sophisticated systems for connecting VHL patients and their families with holistic, empathic, and person-centred medical and psychosocial care are urgently needed.

  10. Comparison of automated von Willebrand factor activity assays

    DEFF Research Database (Denmark)

    Timm, Annette; Hillarp, Andreas; Philips, Malou

    2015-01-01

    INTRODUCTION: Von Willebrand Disease (VWD) is the most common inherited bleeding disorder. Measurement of von Willebrand factor (VWF) activity in plasma is often based on platelet agglutination stimulated by the ristocetin cofactor activity. Novel assays, based on latex beads with recombinant...

  11. Ranking of MR in the diagnosis of diseases of the shoulder joint. Stellenwert der Magnetresonanztomographie in der Diagnostik von Schultergelenkerkrankungen

    Energy Technology Data Exchange (ETDEWEB)

    Vestring, T.; Bongartz, G.; Erlemann, R.; Reuther, G.; Krings, W.; Peters, P.E. (Muenster Univ. (Germany, F.R.). Inst. fuer Klinische Radiologie); Konermann, W.; Saathoff, J.; Drescher, H. (Muenster Univ. (Germany, F.R.). Orthopaedische Klinik und Poliklinik (Huefferstiftung))

    1991-02-01

    In a prospective study 43 patients with shoulder pain were examined by sonography and MRI. The findings were controlled by plain radiography, arthrography, and CT arthrography. Joint effusions and humeral head defects were equally identified by MR and sonography. In the diagnosis of labrum lesions, rotator cuff lesions, subacromial spurs, and synovial inflammatory disease sonography was not as accurate as MR. A special MR scoring system improved the diagnosis of an impingement syndrome. (orig.).

  12. Protein replacement therapy and gene transfer in canine models of hemophilia A, hemophilia B, von willebrand disease, and factor VII deficiency.

    Science.gov (United States)

    Nichols, Timothy C; Dillow, Aaron M; Franck, Helen W G; Merricks, Elizabeth P; Raymer, Robin A; Bellinger, Dwight A; Arruda, Valder R; High, Katherine A

    2009-01-01

    Dogs with hemophilia A, hemophilia B, von Willebrand disease (VWD), and factor VII deficiency faithfully recapitulate the severe bleeding phenotype that occurs in humans with these disorders. The first rational approach to diagnosing these bleeding disorders became possible with the development of reliable assays in the 1940s through research that used these dogs. For the next 60 years, treatment consisted of replacement of the associated missing or dysfunctional protein, first with plasma-derived products and subsequently with recombinant products. Research has consistently shown that replacement products that are safe and efficacious in these dogs prove to be safe and efficacious in humans. But these highly effective products require repeated administration and are limited in supply and expensive; in addition, plasma-derived products have transmitted bloodborne pathogens. Recombinant proteins have all but eliminated inadvertent transmission of bloodborne pathogens, but the other limitations persist. Thus, gene therapy is an attractive alternative strategy in these monogenic disorders and has been actively pursued since the early 1990s. To date, several modalities of gene transfer in canine hemophilia have proven to be safe, produced easily detectable levels of transgene products in plasma that have persisted for years in association with reduced bleeding, and correctly predicted the vector dose required in a human hemophilia B liver-based trial. Very recently, however, researchers have identified an immune response to adeno-associated viral gene transfer vector capsid proteins in a human liver-based trial that was not present in preclinical testing in rodents, dogs, or nonhuman primates. This article provides a review of the strengths and limitations of canine hemophilia, VWD, and factor VII deficiency models and of their historical and current role in the development of improved therapy for humans with these inherited bleeding disorders.

  13. Carl von Clausewitz

    DEFF Research Database (Denmark)

    Højrup, Thomas

    2009-01-01

    Forskningsbaseret præsentation af Carl von Clausewitz' krigsteori og dens betydning for statsbegrebet og udviklingen af teori om statsdannelse og statssystem. Krigsbegrebets betydning for politikbegrebet ekspliciteres, og de indre relationer imellem de to begreber demonstreres med konkrete eksemp...

  14. Ellis Von Creveld Syndrome

    Directory of Open Access Journals (Sweden)

    Afshar H

    1999-01-01

    Full Text Available One patient with Ellis Von Creveld syndrome contains: dwarfism, congenital heart"ndisease, ectodermal dysplasia, polyductyly, an abnormally wide labial frenum and maxillary"nmolars with single root.

  15. Disease specific stress of tumor patients at the beginning of radiotherapy. Effect on psychosocial support requirement; Krankheitsspezifische Belastungen von Tumorpatienten zu Beginn einer Strahlentherapie. Auswirkungen auf den psychosozialen Betreuungsbedarf

    Energy Technology Data Exchange (ETDEWEB)

    Sehlen, S.; Hollenhorst, H.; Schymura, B.; Firsching, M.; Duehmke, E. [Ludwig-Maximilians-Univ., Muenchen (Germany). Klinik und Poliklinik fuer Strahlentherapie und Radioonkologie; Aydemir, U. [Inst. fuer Biometrie und Epidemiologie, Klinikum Grosshadern, Ludwig-Maximilians-Univ., Muenchen (Germany); Herschbach, P. [Technische Univ. Muenchen (Germany). Inst. und Poliklinik fuer Psychosomatische Medizin, Psychotherapie und medizinische Psychologie

    2001-10-01

    Purpose: Radiotherapy brings a tumor patient into a special life situation in which different variables play a role of often unknown importance. The goal of this study was to investigate disease specific stress of tumor patients at the beginning of radiotherapy with established psychodiagnostic questionnaires and to evaluate the effect on psychosocial support requirement in order to reduce stress and to improve quality of life and compliance during radiotherapeutical treatment. Patients and Methods: 732 patients were screened, of whom 446 (60.9%) fulfilled the criteria for inclusion (refusals 21.0%, low Karnofsky performance status 6.6%, management problems 3.4%, language barriers 3.0%, cognitive restrictions 2.6%, death 2.5%). Disease specific aspects of stress in the questionnaire (Fragebogen zur Belastung von Krebspatienten, FBK), life situation (LS) and self-defined care requirements (BB) were self-rated by patients with different tumor types before radiotherapy. Medical and sociodemographic data were also documented. We investigated 446 patients (262 male, 184 female; median age 60.0 years) with different diagnoses. Results: Stress was observed mainly due to reduction of efficiency, anxiety and pain on the subscales. Women had a significant higher stress on subscales of pain (p=0.016) and anxiety (p=0.009), patients younger than 45 years in the subscale information (p=0.002) and patients older than 45 and younger than 60 years in the subscale anxiety (p=0.002) and the total score (p=0.003). Patients with mamma carcinoma had the highest stress. The maximum percentages of patients under high stress were found for the subscales of efficiency (43%) and anxiety (40%). The support requirement was characterized by the need of more medical information and dialogue with the doctor. We saw a significant correlation of high stress and high care requirement. Conclusions: Psychosocial support should be founded on psychosocial stress diagnostic and self-defined care

  16. Radiation therapy of benign diseases: patterns of care study in Germany; Strahlentherapie von gutartigen Erkrankungen: eine Bestandsaufnahme fuer Deutschland

    Energy Technology Data Exchange (ETDEWEB)

    Seegenschmiedt, M.H. [Alfried Krupp Krankenhaus Essen (Germany). Klinik fuer Radioonkologie, Strahlentherapie und Nuklearmedizin; Katalinic, A. [Erlangen-Nuernberg Univ., Erlangen (Germany). Inst. fuer Medizinische Statistik und Dokumentation; Makoski, H.B. [Staedtische Kliniken Duisburg (Germany). Strahlenklinik; Haase, W. [St. Vincentius Krankenhaus Karlsruhe (Germany). Klinik fuer Radioonkologie und Strahlentherapie; Gademann, G. [Magdeburg Univ. (Germany). Klinik fuer Strahlentherapie; Hassenstein, E. [Krankenhaus Nordwest, Frankfurt am Main (Germany). Radioonkologische Klinik

    1999-11-01

    A questionnaire was mailed in 3 years (1994, 1995, 1996) to all radiation facilities in Germany, which assessed equipment, indications, number of patients and treatment concepts. A total of 134 (88%) institutions returned all requested data: 22 in East and 112 in West Germany; 30 in university and 104 in community/private hospitals. The average data of each institution and of all institutions were analyzed for frequencies and ratios between different regions and institutions. A mean of 2 (range 1 to 7) megavoltage (Linac/Cobalt 60) and 1.4 (range 0 to 4) orthovoltage units were available per institution; 32 (24%) institutions had no orthovoltage equipment. A mean of 20,082 patients were treated per year: 456 (2%) for inflammatory diseases (221 hidradenitis, 78 nail bed infection, 23 parotitis, 134 not specified), 12,600 (63%) for degenerative diseases (2,711 peritendinitis humeroscapularis, 1,555 epicondylitis humeri, 1,382 heel spur, 2,434 degenerative osteoarthritis, 4,518 not specified), 927 (5%) for hypertrophic diseases (146 Dupuytren's contracture, 382 keloids, 155 Peyronie's disease, 244 not specified), 1,210 (6%) for functional disorders (853 Graves' orbitopathy, 357 not specified), and 4,889 (24%) for other disorders (e.g. 3,680 heterotopic ossification prophylaxis). In univariate analysis, there were significant geographical (West vs East Germany) differences in the use of radiotherapy for inflammatory and degenerative disorders and institutional differences (university vs community/private hospitals) in the use of radiotherapy for hypertrophic and functional disorders (p<0.05). The prescribed dose concepts were mostly in the low dose range (<10 Gy), but varied widely and inconsistently within geographic regions and institution types. (orig./MG) [German] 1994, 1995 und 1996 wurde an alle strahlentherapeutischen Institutionen in Deutschland ein Fragebogen verschickt, mit dem technische Ausstattung, Indikationsspektrum, Patientenzahl und

  17. Probability of causation for radiation-induced diseases: Part: assessment by IREP; Verursacherwahrscheinlichkeit fuer Strahlenbedingte Erkrankungen, Teil: Pruefung von IREP

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    2006-07-01

    ) should be looked for. As a first step a German participation in a working group of the IAEA is suggested. It prepares the report ''Attributing radiation-linked disease to occupational exposure''. 10) Alternative methods of compensation of risk due to occupational radiation exposure should be studied. These include compensation according to lifetime lost, or additional pay for radiation-exposed workers. 11) It could be discussed to also estimate the probability of causation for non-cancer endpoints (e.g. circulatory diseases) and if necessary to include them in additional tables. 12) In view of the complexity and the importance of the estimation of the probability of causation after exposure to ionizing radiation, the know-how about the details of assumptions, data sets and simplifications should be preserved in Germany by training appropriate PhD students and/or post-docs. (orig.)

  18. von Willebrand Disease (For Parents)

    Science.gov (United States)

    ... disorder. The most common treatment for vWD is desmopressin, a medication that causes a temporary increase in ... surgery, although they usually can be treated with desmopressin. Medication to inhibit the breakdown of blood clots ( ...

  19. Potential role of {sup 68}Ga-DOTATOC PET/CT in screening for pancreatic neuroendocrine tumour in patients with von Hippel-Lindau disease

    Energy Technology Data Exchange (ETDEWEB)

    Prasad, Vikas; Brenner, Winfried [Charite Universitaetsmedizin Berlin, Department of Nuclear Medicine, Campus Virchow-Klinikum, Berlin (Germany); Tiling, Nikolaus; Ploeckinger, Ursula [Charite Universitaetsmedizin Berlin, Interdisziplinaeren Stoffwechsel-Centrum, Campus Virchow Klinikum, Berlin (Germany); Denecke, Timm [Charite Universitaetsmedizin Berlin, Department of Radiology, Berlin (Germany)

    2016-10-15

    Neuroendocrine tumours of the pancreas (pNET) are observed in 8 - 17 % of patients with von Hippel-Lindau disease (vHLD), and 11 - 20 % of these patients develop metastatic disease. MRI and CT have a very high resolution; however, their sensitivity and specificity for the detection of pNET amongst cystic lesions in the pancreas of vHLD patients are generally considered insufficient. In contrast, {sup 68}Ga-DOTATOC PET/CT demonstrates a high sensitivity for the diagnosis and staging of neuroendocrine tumours. In this study we investigated the potential role of {sup 68}Ga-DOTATOC PET/CT in screening of patients with vHLD. {sup 68}Ga-DOTATOC PET/three-phase contrast-enhanced CT was performed according to guidelines in all consecutive vHLD patients between January 2012 and November 2015. All patients underwent additional MRI imaging of the abdomen, spine, and head. Chromogranin A (CgA) was determined at the time of the PET/CT examination. A lesion seen on {sup 68}Ga-DOTATOC PET in the pancreas was defined as positive if the uptake was visually higher than in the surrounding tissues. Lesions were quantified using maximum SUV. Overall, 20 patients (8 men, 12 women; mean age 44.7 ± 11.1 years) were prospectively examined. Genetically, 12 patients had type 1 vHLD and 8 had type 2 vHLD. {sup 68}Ga-DOTATOC PET/CT detected more pNET than morphological imaging (CT or MRI): 11 patients (55 %; 8 type 1, 3 type 2) vs. 9 patients (45 %; 6 type 1, 3 type 2). The concentration of CgA was mildly elevated in 2 of 11 patients with pNET. The mean SUVmax of the pancreatic lesions was 18.9 ± 21.9 (range 5.0 - 65.6). Four patients (36.4 %) had multiple pNETs. The mean size of the lesions on CT and/or MRI was 10.4 ± 8.3 mm (range 4 - 38 mm), and 41.1 % were larger than 10 mm. In addition, somatostatin receptor-positive cerebellar and spinal haemangioblastomas were detected in three patients (SUVmax 2.1 - 10.1). One patient presented with a solitary somatostatin receptor-positive lymph

  20. Von Braun Fountain

    Science.gov (United States)

    1999-01-01

    A fountain representing a rocket launch was dedicated in the Von Braun courtyard outside of Building 4200 at Marshall Space Flight Center during the weekend celebrating the 30th arniversary of the Apollo 11 lunar landing. On hand for the festivities were many of the Saturn and Apollo astronauts.

  1. Rhizostomeen von Manila

    NARCIS (Netherlands)

    Stiasny, G.

    1924-01-01

    Die hier beschriebene kleine Scyphomedusen-Sammlung wurde von Herrn Director P. B. Sivickis, Dept. of Zoology, University of Philippines, Manila, dem Rijksmuseum van Natuurlijke Historie in Leiden überwiesen. Das Material wurde im December 1922 in Manila-bay gefischt und befindet sich in bestem

  2. Karman, Prof. Theodore von

    Indian Academy of Sciences (India)

    Home; Fellowship. Fellow Profile. Elected: 1961 Honorary. Karman, Prof. Theodore von. Date of birth: 11 May 1881. Date of death: 6 May 1963. YouTube; Twitter; Facebook; Blog. Academy News. IAS Logo. Theory Of Evolution. Posted on 23 January 2018. Joint Statement by the Three Science Academies of India on the ...

  3. Schmetterlinge von Madeira

    NARCIS (Netherlands)

    Martin, K.

    1941-01-01

    Wer auf einer ozeanischen Insel Schmetterlinge sammeln will, darf seine Erwartungen nicht zu hoch spannen; aber dennoch waren meine Frau und ich sehr enttäuscht, als uns auf einer Reise nach Madeira von einer dort angesessenen, gebildeten Dame gesagt wurde, sie habe auf der Insel niemals einen

  4. Inflammatory bowel diseases (IBD) - critical discussion of etiology, pathogenesis, diagnostics, and therapy; Chronisch entzuendliche Darmerkrankungen - Kritische Diskussion von Aetiologie, Pathogenese, Diagnostik und Therapie

    Energy Technology Data Exchange (ETDEWEB)

    Ochsenkuehn, T.; Sackmann, M.; Goeke, B. [Medizinische Klinik II, Klinikum der Universitaet Muenchen-Grosshadern (Germany)

    2003-01-01

    Aims Crohn's disease and ulcerative colitis are the most frequent inflammatory bowel diseases (IBD) with a prevalence of approximately one out of 500.Cytokine research opened new and potent treatment options and thus stimulated clinical and basic research.However, the IBD still remain a challenge for patients and physicians,demanding close cooperation between gastroenterologists,radiologists and surgeons.The basic understanding of IBD,which is necessary for efficient diagnostic and therapeutic concepts is reviewed. Based upon recent publications and our clinical experience we discuss aspects of etiology,pathogenesis,diagnostics,and therapy of Crohn's disease and ulcerative colitis. A genetically influenced, exaggerated and sustained immune response against the own gut flora seems to be one of the most important factors in the pathogenesis of IBD.Not less important are environmental influences.For instance, cigarette smoking had been judged to have some negative influence on the natural course of Crohn's disease.Now,however, recent studies show that smoking is even a significant independent risk factor in the pathogenesis of IBD. Since IBD and especially Crohn's disease can effect the whole body, detailed analysis of inflammatory organ involvement is necessary before therapy.For instance, the MRIenteroclysis technique adds a necessary diagnostic tool for the exploration of those parts of the small bowel that cannot been reached by routine endoscopy like the upper ileum and the lower jejunum. In terms of therapy, a change of paradigms can be observed: patients will no longer be treated only when symptoms arise, but will early be integrated into a therapeutic concept, which is determined by site and extent of the disease and adapted to the abilities and needs of the patient.Furthermore,immunosuppressive agents like azathioprine and 6-mercaptopurine will establish as central concept in the medical treatment of IBD.Discussion IBD-therapy should

  5. Evidence for the absence of cerebral glucose-6-phosphatase activity in glycogen storage disease type I (Von Gierke's disease)

    Energy Technology Data Exchange (ETDEWEB)

    Phelps, M.E.; Mazziotta, J.C.; Hawkins, R.A.; Philippart, M.

    1981-01-01

    Glycogen storage disease type I (GSD-I) is characterized by a functional deficit in glucose-6-phosphatase that normally hydrolyzes glucose-6-PO/sub 4/ to glucose. This enzyme is primarily found in liver, kidney, and muscle but it is also present in brain, where it appears to participate in the regulation of cerebral tissue glucose. Since most neurological symptoms in GSD-I patients involve systemic hypoglycemia, previous reports have not examined possible deficiencies in phosphatase activity in the brain. Positron computed tomography, F-18-labeled 2-fluorodeoxyglucose (FDG) and a tracer kinetic model for FDG were used to measure the cortical plasma/tissue forward and reverse transport, phosphorylation and dephosphorylation rate constants, tissue/plasma concentration gradient, tissue concentration turnover rate for this competitive analog of glucose, and the cortical metabolic rates for glucose. Studies were carried out in age-matched normals (N = 13) and a single GSD-I patient. The dephosphorylation rate constant in the GSD-I patient was about one tenth the normal value indicating a low level of cerebral phosphatase activity. The other measured parameters were within normal limits except for the rate of glucose phosphorylation which reflected a cortical glucose metabolic rate one half the normal value. Since glucose transport and tissue glucose concentration was normal, the reduced cortical glucose metabolism probably results from the use of alternative substrates (..beta..-hydroxybutyrate and acetoacetate) which are consistently elevated in the plasma of GSD-I patients.

  6. Kommunikative Konstitution von Organisationen

    DEFF Research Database (Denmark)

    Schoeneborn, Dennis; Wehmeier, Stefan

    2014-01-01

    Beiträge zur Unternehmenskommunikation behandeln in der Regel (strategisch) geplante interne oder externe Kommunikation. Der vorliegende Beitrag stellt einen alternativen Theorieansatz vor, der aktuell im nordamerikanischen Forschungsgebiet „Organizational Communication“ vorherrschend ist....... zunehmende Aufmerksamkeit erhält Der sogenannten „CCO-Perspektive“ („Communicative Constitution of Organizations“) zufolge bestehen Unternehmen aus einer Vielzahl von internen und externen Kommunikationspraktiken, die nur in bedingtem Maße strategisch steuerbar sind. Zugleich sind es eben diese...

  7. Kulturelle Aspekte von Textsorten

    Directory of Open Access Journals (Sweden)

    Siegfried Heusinger

    1998-12-01

    Full Text Available Ich fasse meine Überlegungen zu kulturellen Aspekten von Textsorten zusammen: 1. Mit unseren Kulturverständnis ist die terminologisierte Wortgruppe "interkulturelle Kommunikation" nicht zu begreifen als Kommunikation zwischen Menschen verschiedener kultureller Bindungen, sondern sie ist nicht zu begreifen als Kommunikation zwischen Menschen verschiedener kultureller Bindungen, sondern sie ist ethnisch wie auch ethisch angepasstes sprachlichkommunikatives Verhalten an kulturelle Traditionen der jeweils für den Kommunkations- akt vereinbarten Sprache. 2. Das Adjektiv "interkulturell" referiert auf idiomatisierte Wendungen im zwischenmenschlichen Kontakt wie auch auf kulturell gebundene Unterschiede in der Benennungsmotivation (z.B. anloben/österr./, vereidigen /dt./ 3. Das Adjektiv "interkulturell" ist ebenfalls zu beziehen auf den Austausch von Erfahrungen und Ideen, auf den Dialog von Kulturen. 4. Textsorten existieren in ihrer kulturellen in ihrer kulturellen Tradition, wobei das Kulturspezifische vornehmlich in traditionellen (aber entwicklungsoffenen Formulierungs- und Gestaltungsmustern zu suchen iost. Andere Textsortencharakteristika wie die situative Einbettung, die kommunikative Zwecksetzung, die thematische Bindung und die Komposition haben offenbar kaum eine kulturspezifische Prägung. 5. Im Trend zur Internationalisierung der Textsorten erweisen sich die kulturell geprägten Textmustervor allem zwischen angrenzenden Nationen häufig als affin.

  8. Nutzerorientiertes Management von materiellen und immateriellen Informationsobjekten

    OpenAIRE

    Hübsch, Chris

    2001-01-01

    Schaffung einer stabilen, erweiterbaren und skalierbaren Infrastruktur für die Bereitstellung von Diensten im Umfeld von Bibliotheken und ähnlichen wissensanbietenden Einrichtungen unter Verwendung von XML-RPC und Python.

  9. Assessment of the olfactory function in Italian patients with type 3 von Willebrand disease caused by a homozygous 253 Kb deletion involving VWF and TMEM16B/ANO2.

    Directory of Open Access Journals (Sweden)

    Valentina Cenedese

    Full Text Available Type 3 Von Willebrand disease is an autosomal recessive disease caused by the virtual absence of the von Willebrand factor (VWF. A rare 253 kb gene deletion on chromosome 12, identified only in Italian and German families, involves both the VWF gene and the N-terminus of the neighbouring TMEM16B/ANO2 gene, a member of the family named transmembrane 16 (TMEM16 or anoctamin (ANO. TMEM16B is a calcium-activated chloride channel expressed in the olfactory epithelium. As a patient homozygous for the 253 kb deletion has been reported to have an olfactory impairment possibly related to the partial deletion of TMEM16B, we assessed the olfactory function in other patients using the University of Pennsylvania Smell Identification Test (UPSIT. The average UPSIT score of 4 homozygous patients was significantly lower than that of 5 healthy subjects with similar sex, age and education. However, 4 other members of the same family, 3 heterozygous for the deletion and 1 wild type, had a slightly reduced olfactory function indicating that socio-cultural or other factors were likely to be responsible for the observed difference. These results show that the ability to identify odorants of the homozygous patients for the deletion was not significantly different from that of the other members of the family, showing that the 253 kb deletion does not affect the olfactory performance. As other genes may compensate for the lack of TMEM16B, we identified some predicted functional partners from in silico studies of the protein-protein network of TMEM16B. Calculation of diversity for the corresponding genes for individuals of the 1000 Genomes Project showed that TMEM16B has the highest level of diversity among all genes of the network, indicating that TMEM16B may not be under purifying selection and suggesting that other genes in the network could compensate for its function for olfactory ability.

  10. 18F-FDG and 68Ga-DOTATATE PET/CT in von Hippel-Lindau Disease-Associated Retinal Hemangioblastoma.

    Science.gov (United States)

    Papadakis, Georgios Z; Millo, Corina; Jassel, Inderbir S; Bagci, Ulas; Sadowski, Samira M; Karantanas, Apostolos H; Patronas, Nicholas J

    2017-03-01

    Retinal hemangioblastomas are highly vascular benign tumors that can be encountered either sporadically or within the von Hippel-Lindau (VHL) syndrome. We report a case of a VHL patient with retinal hemangioblastoma who underwent PET/CT scans using F-FDG and Ga-DOTATATE. The tumor showed low-level F-FDG and increased Ga-DOTATATE activity, suggesting cell-surface overexpression of somatostatin receptors. The presented case indicates the clinical applications of somatostatin receptor imaging with Ga-DOTA-conjugated peptides in detection and follow-up of VHL manifestations, screening of asymptomatic gene carriers, and in diagnosis of sporadic retinal hemangioblastomas, which may have similar features on MRI with other retinal tumors.

  11. Sygdomsmekanismer og terapeutiske perspektiver ved neurofibromatose 1

    DEFF Research Database (Denmark)

    Kjærulff, Ole; Wandall, Hans

    2012-01-01

    Neurofibromatosis 1 (NF1, von Recklinghausen's disease) is a dominantly inherited multi-organ disease defined primarily by café au lait patches and neurofibromas. NF1 predisposes to cancer and is associated with cognitive dysfunction and learning defects. In recent years, considerable progress has...

  12. Diagnostic imaging of venous disease. Pt. I. Methods in the diagnosis of veins and thrombosis; Bildgebende Diagnostik von Erkrankungen der Venen. T. 1. Methoden der Venendiagnostik und Diagnostik der Thrombose

    Energy Technology Data Exchange (ETDEWEB)

    Krueger, K. [Vivantes Humboldt-Klinikum, Institut fuer Radiologie und Interventionelle Therapie, Berlin (Germany); Wildberger, J. [Helios Klinikum Berlin-Buch, Institut fuer Roentgendiagnostik, Berlin (Germany); Haage, P. [Helios Klinikum Wuppertal, Klinik fuer Diagnostische und Interventionelle Radiologie, Wuppertal (Germany); Landwehr, P. [Diakoniekrankenhaus Henriettenstiftung, Klinik fuer Diagnostische und Interventionelle Radiologie, Hannover (Germany)

    2008-10-15

    Diseases of the venous system are common. A variety of diagnostic imaging methods are available. Of these, ultrasound with color-coded duplex sonography is the preferred method in the diagnosis of vein diseases, especially in patients with suspected deep venous thrombosis (DVT) and varicosis. Compression sonography is a very accurate test in the diagnosis of DVT of the lower and upper extremities in symptomatic patients. Still today, phlebography is an important tool in the diagnosis of venous diseases. Computed tomography (CT) plays an important role in disorders of the superior and inferior vena cava and of the iliac vein. Today, the role of MR venography in vein diseases is limited. (orig.) [German] Erkrankungen des Venensystems betreffen grosse Teile der Bevoelkerung. Verschiedene bildgebende Verfahren stehen in der Diagnostik zur Verfuegung. Unter diesen ist die Sonographie, ergaenzt durch die farbkodierte Duplexsonographie, die Methode der Wahl. Dies gilt insbesondere fuer die haeufigsten Venenerkrankungen: die Phlebothrombose und die Varicosis. Die Kompressionssonographie weist bei symptomatischen Patienten eine hohe Sensitivitaet und Spezifitaet in der Diagnostik der Thrombose der tiefen Venen der oberen und unteren Extremitaet auf. Die Phlebographie stellt trotz der Bedeutung der Sonographie auch zum gegenwaertigen Zeitpunkt noch eine wichtige Methode in der Diagnostik von Venenerkrankungen dar. Die Computertomographie wird ueberwiegend fuer die Diagnostik der Koerperstammvenen eingesetzt. Die MR-Phlebographie hat bislang keinen breiten Einsatz in der Routinediagnostik der Venen finden koennen. (orig.)

  13. Laboratory Testing for Von Willebrand Factor Multimers.

    Science.gov (United States)

    Oliver, Susan; Lau, Kun Kan Edwin; Chapman, Kent; Favaloro, Emmanuel J

    2017-01-01

    Von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders develop due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for the VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. As an additional step, an evaluation of VWF structural features by multimer analysis is useful in selective investigations. The current paper therefore describes a protocol for assessment of VWF multimers by gel electrophoresis, thus enabling identification of protein bands that represent differently sized multimers. The sample protocol described in this chapter is the methodology developed by Sebia.

  14. Acquired von Willebrand syndrome: A rare disorder of heterogeneous etiology

    Directory of Open Access Journals (Sweden)

    P Kasatkar

    2013-01-01

    Full Text Available Context: Acquired von Willebrand syndrome (AVWS is a rare bleeding disorder that mimics the inherited form of von Willebrand disease (VWD in terms of laboratory findings and clinical presentation. Aims: To study the etiology of acquired VWD. Settings and Design: The patients referred from various hospitals in and out of Mumbai were included in the study. Materials and Methods: Six patients with AVWS diagnosed at this center over the last 10 years were analyzed against 171 patients with inherited VWD. The differential diagnosis of AVWS was made based on reduced levels of von Willebrand antigen and von Willebrand ristocetin cofactor, decrease in ristocetin induced platelet aggregation, absence of correction in mixing studies with no prior history of bleeding problems and a negative family history for bleeding disorders. Results: In three patients, the disease was associated with systematic lupus erythematosus, out of which one was also associated with Kikuchi lymphadenitis and second with autoimmune hemolytic anemia. Fourth case was associated with hypothyroidism and fifth was a case of dermatitis and vitiligo. The last patient was a case of hemophilia A with Burkitts lymphoma, who developed autoantibodies to von Willebrand factor. Except two patients, all other patients responded to immune suppressive therapy with corticosteroids, while the patient with hypothyroidism responded to oral thyroxine. Conclusion: AVWS is a rare condition and may often be missed or diagnosed as inherited disease associated with heterogeneous disease conditions.

  15. Volumetric MRI for evaluation of regional pattern and progression of neocortical degeneration in Alzheimer's disease; MR-Volumetrie zur Darstellung von Verteilung und zeitlicher Abfolge neokortikaler Degeneration bei Morbus Alzheimer

    Energy Technology Data Exchange (ETDEWEB)

    Leinsinger, G. [Institut fuer Klinische Radiologie, Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Muenchen (Germany); Institut fuer Klinische Radiologie, LMU Muenchen, Ziemssenstrasse 1, 80336, Muenchen (Germany); Teipel, S.; Pruessner, J.; Hampel, H. [Klinik fuer Psychiatrie, Ludwig-Maximilians-Universitaet Muenchen, Muenchen (Germany); Wismueller, A.; Born, C.; Meindl, T.; Flatz, W.; Schoenberg, S.; Reiser, M. [Institut fuer Klinische Radiologie, Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Muenchen (Germany)

    2003-07-01

    Volumetric analysis of the corpus callosum and hippocampus using MRI in Alzheimer's disease (AD) to evaluate the regional pattern and progression of neocortical neurodegeneration. In subsequent studies we investigated patients with AD and healthy controls. Volumetry was based on MRI-data from a sagittal 3D T1w-gradient echo sequence. The corpus callosum (CC) was measured in a midsagittal slice, and subdivided into 5 subregions. Volumetry of the hippocampus/amygdala-formation (HAF) was performed by segmentation in coronary reoriented slices. In AD patients we found a significant atrophy in the rostrum und splenium of CC. The atrophy was correlated with the severity of dementia, but no correlation was found with the load of white matter lesions. In comparison with {sup 18}FDG-PET, we found a significant correlation of regional CC-atrophy with the regional decline of cortical glucose metabolism. A ROC-analysis demonstrated no significant differences in the diagostic accuracy of HAF volumetry and regional CC volumetry of the splenium (region C5) even in mild stages of dementia. Regional atrophy of CC can be used as a marker of neocortical degeneration even in early stages of dementia in AD. (orig.) [German] Volumetrische Analyse des Corpus callosum und Hippokampus mittels MRT bei der Alzheimer-Erkrankung (AD), mit dem Ziel die regionale Verteilung und Progression der neokortikalen relativ zur allokortikalen Neurodegeneration zu erfassen. In mehreren Studienabschnitten wurden Patienten mit AD und gesunde Kontrollen untersucht. Als Grundlage fuer die Volumetrie diente eine sagittale 3D-T1w-Gradientenechosequenz. Die Vermessung des Corpus callosum (CC) erfolgte in der mittsagittalen Schicht, wobei 5 Subregionen definiert wurden. Die Volumetrie des Hippokampus-Amygdala-Komplexes (HAK) wurde durch Segmentierung an koronar reorientierten Schichten durchgefuehrt. Bei Patienten mit AD fand sich eine signifikante Atrophie in Rostrum und Splenium des CC. Dabei zeigte sich

  16. Diagnostics for diseases of the gallbladder and biliary tract from the viewpoint of the internist and surgeon. Demands made on radiological diagnostics; Diagnostik von Erkrankungen der Gallenblase und - Wege aus Sicht des Internisten und Chirurgen. Anforderungen an die radiologische Diagnostik

    Energy Technology Data Exchange (ETDEWEB)

    Reimann, F.M. [Krankenhaus Salem, Heidelberg (Germany); Friess, H. [Krankenhaus Salem, Heidelberg (Germany); Universitaetsklinikum Heidelberg (Germany). Abteilung fuer Allgemein-, Viszeral- und Unfallchirurgie

    2005-11-01

    Jaundice and colic pain of the right upper quadrant are the main symptoms of biliary diseases. Gallstone-related diseases often lead to hospital admission. The evaluation of a patient with biliary symptoms requires a combination of history taking, physical examination, laboratory analysis, and imaging modalities. A high-quality magnetic resonance imaging (MRI) or computed tomography (CT) scan is usually sufficient to evaluate a patient with painless jaundice. Ultrasonography is helpful as an initial screening test to guide the diagnostic work-up. Invasive methods (e.g., ERCP) are mainly used for palliation of patients with incurable disease. (orig.) [German] Erkrankungen der Gallenwege manifestieren sich mit den beiden Kardinalsymptomen Ikterus und kolikartigen rechtsseitigen Oberbauchschmerzen. Die durch Gallensteine verursachten Beschwerden und Erkrankungen zaehlen zu den haeufigsten gastroenterologischen Krankheitsbildern, die eine Klinikaufnahme erfordern. Die Abklaerung eines Patienten mit biliaeren Symptomen erfolgt durch eine Kombination von Anamnese, Medikamentenanamnese, koerperlicher Untersuchung, Laboranalysen und bildgebenden Verfahren. Bei Patienten mit malignen Tumoren im Bereich der ableitenden Gallenwege reichen in vielen Faellen Computertomographie (CT) oder Magnetresonanztomographie (MRT) zur Abklaerung des schmerzlosen Ikterus aus, um Therapieentscheidungen fuer den Patienten zu faellen. Der Ultraschall ist fuer die Planung der weiteren Diagnostik oft entscheidend. Der Stellenwert invasiver Methoden, wie z. B. der endoskopischen retrograden Cholangio-Pankreatikographie (ERCP), liegt vornehmlich in der Intervention, insbesondere zur Palliation bei Patienten, deren Erkrankung nicht mehr heilbar ist. (orig.)

  17. An ELISA for the quantitation of von Willebrand Factor

    DEFF Research Database (Denmark)

    Vinholt, Pernille Just; Overgaard, Martin; Diederichsen, Axel Cosmus Pyndt

    2013-01-01

    BACKGROUND: Von Willebrand factor (VWF) is pivotal in arterial thrombosis, and osteoprotegerin (OPG) is besides being a bone protein also related to cardiovascular diseases. OPG can bind VWF, but the significance of this interaction is not known. OBJECTIVES: The aim was to develop an assay...... for measurement of von Willebrand factor-osteoprotegerin complex (VWF:OPG) in human plasma. Furthermore, the significance of VWF:OPG complex as a marker of cardiovascular disease (CVD) was evaluated. PATIENTS/METHODS: A sandwich ELISA for quantification of VWF:OPG was developed using a polyclonal rabbit anti...

  18. John von Neumann Birthday Centennial

    Energy Technology Data Exchange (ETDEWEB)

    Grcar, Joseph F.

    2004-11-12

    In celebration of John von Neumann's 100th birthday, a series of four lectures were presented on the evening of February 10, 2003 during the SIAM Conference on Computational Science and Engineering in San Diego. The venue was appropriate because von Neumann spent much of the later part of his life, in the 1950's, as an unofficial ambassador for computational science. He was then the only senior American scientist who had experience with the new computers (digital, electronic, and programmable) and a vision of their future importance. No doubt he would have relished the chance to attend a meeting such as this. The first speaker, William Aspray, described the ''interesting times'' during which computers were invented. His remarks were based on his history [1] of this period in von Neumann's life. We were honored to have John von Neumann's daughter, Marina von Neumann-Whitman, as our second speaker. Other accounts of von Neumann's life can be found in books by two of his colleagues [2] and [3]. Our third speaker, Peter Lax, provided both mathematical and international perspectives on John von Neumann's career. Finally, Pete Stewart spoke about von Neumann's numerical error analysis [4] in the context of later work; this talk did not lend itself to transcription, but readers may consult the historical notes in [5]. Our thanks to all the speakers for a remarkable evening. We are grateful to the DOE Applied Mathematical Sciences (AMS) program for partially supporting these lectures. Thanks are also due to SIAM and William Kolata, to our emcee, Gene Golub, to Paul Saylor for recording and editing, and to Barbara Lytle for the transcriptions. More about von Neumann's work can be learned from the recent American Mathematical Society proceedings [6].

  19. Browse Title Index

    African Journals Online (AJOL)

    Items 51 - 100 of 390 ... Vol 54, No 3 (2013), Bilateral orbital infarction and retinal detachment in a previously undiagnosed sickle cell hemoglobinopathy African child, Abstract ... Andrew E Edo. Vol 50, No 4 (2009), Clinico-Pathologic Presentation and Management of neurofibromatosis type 1(Von Recklinghausen's) Disease ...

  20. Author Details

    African Journals Online (AJOL)

    Nyandaiti, YW. Vol 50, No 4 (2009) - Articles Clinico-Pathologic Presentation and Management of neurofibromatosis type 1(Von Recklinghausen's) Disease among North-Eastern Nigerians: A six year review. Abstract. ISSN: 2229-774X. AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians ...

  1. Schwannoma extending from the umbilical region to the mid-thigh ...

    African Journals Online (AJOL)

    They commonly arise from the cranial nerves as acoustic schwannomas and are extremely rare in the pelvis and retroperitoneal area (<0.5% of reported cases) unless they are combined with Von Recklinghausen disease (type 1 neurofibromatosis). We report the case of a 23-year-old woman with a mass extending from the ...

  2. Neurofibromatosis type 1: a rare cause of parotid swelling in a child ...

    African Journals Online (AJOL)

    Von Recklinghausen disease, also known as neurofibromatosis type 1, is an autosomal dominant disorder that presents as neurocutaneous syndrome. These patients have increased chances of developing other tumors such as plexiform neurofibromas. Plexiform neurofibromas are a proliferation of Schwann cells in the ...

  3. Genetic linkage studies with neurofibromatosis: the question of heterogeneity.

    Science.gov (United States)

    Spence, M A; Sparkes, R S; Parry, D M; Bale, S J; Cortessis, V; Mulvihill, J J

    1987-01-01

    Three new families are reported for standard gene linkage markers and classical peripheral neurofibromatosis (Von Reckling-hausen disease). Additional data are summarised for the exclusion map. One family gives slight evidence of close linkage with the Gc locus on chromosome 4, raising again the question of possible genetic heterogeneity in NF. PMID:3118031

  4. Alejandro Von Humboldt

    Directory of Open Access Journals (Sweden)

    Gerardo Paz Otero

    1965-09-01

    Full Text Available Diverso fue y sigue siéndolo, el destino de los dos hermanos Humboldt, Guillermo y Alejandro. Sino que se inicia con el nacimiento: Guillermo, el mayor, nace el 22 de junio de 1767 en Potsdam, residencia de los emperadores prusianos, la ciudad de los palacios imperiales, el imperio del militarismo germano; su cuna se meció cerca al palacete de Sans-Soussi, donde Federico el Grande forja ba el poderío de Prusia, cultivaba las ciencias y las artes, anfitrionaba a los intelectuales de Europa, y era "vasallo espiritual de Volta ire", según la aguda frase de Goethe. Alejandro viene al mundo dos años después (14 de septiembre de 1769 en Berlín, en la casa burguesa de la Jagerstrasse (calle del cazador que su madre Elizabeth von Humboldt heredara de su primer esposo.

  5. Der Einfluss von Phlorizin auf den programmierten Zelltod von Erythrozyten

    OpenAIRE

    Meier, Anja Doris

    2013-01-01

    Erythrozyten sind in der Lage, suizidalen Zelltod zu betreiben. Von einigen Substanzen ist bereits bekannt, dass sie die sogenannte Eryptose beeinflussen. Beispiele dafür sind Quecksilber, Monensin, Benzethonium und Oridonin, welche zu den Stimulatoren der Eryptose gehören. Blebbistatin, Koffein und Endothelin, gehören zu den Inhibitoren. In dieser Studie sollte untersucht werden, welchen Einfluss Phlorizin auf den Ablauf des suizidalen Zelltodes von Erythrozyten hat. Es wurde belegt, dass si...

  6. Einsatz von Nickel-Katalysatoren zum Cracken von Teerprodukten aus der Niederdruckaufkohlung von Stahl mit Ethin

    OpenAIRE

    Mbadinga Mouanda, Gelase

    2009-01-01

    Die Niederdruckaufkohlung von Stahl ist ein modernes Verfahren, das zur Einsatzhärtung von Stahlbauteilen dient. Bei Temperaturen zwischen 900 und 1050 °C und Drücken unter 50 mbar werden Bauteile durch heterogene Pyrolyse von Ethin in einem Ofen einsatzgehärtet. Das während der Pyrolyse erzeugte Abgas enthält aromatische Kohlenwasserstoffe und kann katalytisch behandelt werden. Der Katalysator wird durch Rußablagerung desaktiviert und muss zyklisch durch Verbrennung mit Luft regeneriert werd...

  7. Ueber einige Coelenterata von Australien

    NARCIS (Netherlands)

    Stiasny, G.

    1931-01-01

    I. Hydromedusen. *Olindias singularis Browne. II. Scyphomedusen. *Atolla wyvillei Haeckel. *Linuche unguiculata O. Swartz. Pelagia noctiluca Péron u. Lesueur. Cyanea capillata var. annaskala von Lendenfeld. Netrostoma coerulescens Maas. *Versura anadyomene (Maas) Mayer. Catostylus mosaicus L.

  8. Kleben von Kunststoff mit Metall

    CERN Document Server

    Brockmann, W; Käufer, H

    1989-01-01

    Das Buch behandelt das Kleben von Kunststoffen mit Metallen in einer fur den Praktiker verstandlichen und umsetzbaren Form. Es leitet zu Klebeverfahren an, die optimale Ergebnisse hinsichtlich Qualitat, Dauerhaftigkeit und Wirtschaftlichkeit liefern.

  9. Fluorido-Komplexe von Technetium

    OpenAIRE

    Mariappan Balasekaran, Samundeeswari

    2013-01-01

    Zusammenfassung Diese Dissertationsschrift befasst sich mit der Synthese und Charakterisierung neuer Technetiumfluoride mit dem Metall in den Oxidationsstufen “+1”, “+2”, “+4” und “+6”. Im ersten Kapitel wird über die Isolierung von unterschiedlichen Salzen von Fluoridonitridotechnetaten(VI) entweder aus Nitridotechnetium(VI)-säure oder aus Pertechnetat durch den Einsatz geeigneter Reduktionsmittel berichtet. Das Cäsiumsalz dieser Verbindung bildet einen oxido-verbrückten, dimeren Kompl...

  10. Effiziente chemoenzymatische Synthese von dhydroartemisinaldehyd

    OpenAIRE

    Demiray, Melodi; Tang, Xiaoping; Wirth, Thomas; Faraldos, Juan A.; Allemann, Rudolf K.

    2017-01-01

    Artemisinin aus der Pflanze Artemisia annua ist das wirkungsvollste Arzneimittel zur Behandlung von Malaria. Die Sesquiterpen-Cyclase Amorphadien-Synthase, ein Cytochrom-abhängiges CYP450 und eine Aldehyd-Reduktase wandeln in der Pflanze Farnesyl-Diphosphat (FDP) in Dihydroartemisinaldehyd (DHAAl) um, welches ein Schlüsselzwischenprodukt in der Biosynthese von Artemisinin und eine halbsynthetische Vorstufe in der chemischen Synthese des Arzneimittels ist. Hier berichten wir über einen chemoen...

  11. The oncolytic activity of Newcastle disease virus in clear cell renal carcinoma cells in normoxic and hypoxic conditions: the interplay between von Hippel-Lindau and interferon-β signaling.

    Science.gov (United States)

    Ch'ng, Wei-Choong; Stanbridge, Eric J; Yusoff, Khatijah; Shafee, Norazizah

    2013-07-01

    Viral-mediated oncolysis is a promising cancer therapeutic approach offering an increased efficacy with less toxicity than the current therapies. The complexity of solid tumor microenvironments includes regions of hypoxia. In these regions, the transcription factor, hypoxia inducible factor (HIF), is active and regulates expression of many genes that contribute to aggressive malignancy, radio-, and chemo-resistance. To investigate the oncolytic efficacy of a highly virulent (velogenic) Newcastle disease virus (NDV) in the presence or absence of HIF-2α, renal cell carcinoma (RCC) cell lines with defective or reconstituted wild-type (wt) von Hippel-Lindau (VHL) activity were used. We show that these RCC cells responded to NDV by producing only interferon (IFN)-β, but not IFN-α, and are associated with increased STAT1 phosphorylation. Restoration of wt VHL expression enhanced NDV-induced IFN-β production, leading to prolonged STAT1 phosphorylation and increased cell death. Hypoxia augmented NDV oncolytic activity regardless of the cells' HIF-2α levels. These results highlight the potential of oncolytic NDV as a potent therapeutic agent in the killing of hypoxic cancer cells.

  12. Diurnal variation of von Willebrand factor in plasma

    DEFF Research Database (Denmark)

    Timm, Annette; Fahrenkrug, Jan; Jørgensen, Henrik L

    2014-01-01

    BACKGROUND: Quantitation of von Willebrand factor (VWF) in plasma is a central element in assessing von Willebrand disease (VWD). VWF activity is known to vary, which has partly been ascribed to biological and preanalytical variation. However, a possible diurnal expression of VWF has not been...... of light and 9 h of darkness); the plasma concentration of melatonin was used as an internal control to confirm the normal 24-h rhythms of the individual participants. RESULTS: The data, analyzed by rhythmometric statistics, revealed a significant variation (P = 0.02) and total amplitude of 22.6% in VWF...... and VWF and (ii) VWF propeptide and VWF was determined. Taken together, the data suggest changes in release and not in clearance. CONCLUSIONS: Diurnal variation in von Willebrand antigen and activity in plasma represents an important aspect of the biological variation. Standardized time-of-day plasma...

  13. Degenerative diseases of the spine. Rare and often unrecognized causes of pain syndromes; Degenerative Erkrankungen der Wirbelsaeule. Seltene und oft verkannte Ursachen von Schmerzsyndromen

    Energy Technology Data Exchange (ETDEWEB)

    Baur-Melnyk, A.; Triantafyllou, M.; Reiser, M. [Klinikum Grosshadern der Ludwig-Maximilians-Universitaet, Institut fuer Klinische Radiologie, Muenchen (Germany); Birkenmaier, C. [Klinikum Grosshadern der Ludwig-Maximilians-Universitaet Muenchen, Orthopaedische Klinik und Poliklinik (Germany)

    2006-06-15

    The aim of this article is to describe rare and often unrecognized causes of spinal pain syndromes. Intervertebral disc degeneration frequently appears in early adulthood and can have a symptomatic or asymptomatic course. This article discusses incidence, pathophysiology, imaging, and pain symptomatology involved in the origin of back pain. Anulus tears are often found in asymptomatic individuals but could be implicated in lumbar pain symptomatology in correlation with the provocative discography. Transient disorders can lead to pseudarthrosis of the iliac bone and to degeneration or to a reactive hypermobility with intervertebral disc degeneration in the level above. Modic type 1 erosive osteochondrosis is characterized by bone marrow edema near the hyaline cartilage end plate, which mostly elicits severe pain and results in serious limitations in everyday activities. The most important differential diagnosis is spondylodiscitis. Schmorl's nodes can exhibit considerable surrounding bone marrow edema that can be mistaken for metastases. A combination of MRI and CT should be employed for the diagnostic work-up of fatigue fracture of the interarticular portion, which is often overlooked due to its location. Synovial cysts of the facet joints can lead to radicular symptoms. Insufficiency fracture of the sacrum is frequently mistaken for metastasis due to intense scintigraphic enhancement and its signal behavior in MRI. CT provides instructive information. Differential diagnosis should include less common causes such as anulus tears, transient disorders, activated Schmorl's nodes, synovial cysts of the facet joints, fatigue fractures of the interarticular portion of the spine and the sacrum and distinguish from metastases in particular. (orig.) [German] Darstellung seltener und oft verkannter Ursachen von Wirbelsaeulenschmerzsyndromen. Eine Bandscheibendegeneration tritt haeufig im fruehen Erwachsenenalter auf und kann symptomatisch oder asymptomatisch

  14. Comparison of HR-SPECT and MR-imaging in the diagnosis of Perthes disease; Vergleich von HR-SPECT und MRT bei der Diagnostik des Morbus Perthes

    Energy Technology Data Exchange (ETDEWEB)

    Mellerowicz, H. [Orthopaedische Klinik und Poliklinik, Oskar-Helene-Heim, Freie Univ. Berlin (Germany); Schulze, C. [Orthopaedische Klinik und Poliklinik, Oskar-Helene-Heim, Freie Univ. Berlin (Germany); Stelling, E. [Orthopaedische Klinik und Poliklinik, Oskar-Helene-Heim, Freie Univ. Berlin (Germany); Stabell, U. [Praxis fuer Nuklearmedizin, Berlin (Germany); Schedel, H. [Strahlenklinik und Poliklinik, Klinikum Rudolf Virchow, Freie Univ. Berlin (Germany)

    1993-12-31

    Children, who are supposed to suffer from M. Legg-Perthes, are diagnosed by clinical-, X-ray examination and today mostly by MRT. MRT gives the most reliable information but high cost, small availability and the demand for resting motionless during the examination is regarded as a problem, especially concerning children. The aim of our study was to evaluate 3D-HR-SPECT as an alternative diagnostic procedure to MRT. 28 children (2-12 years, mean 6,2 years) suspected of suffering from M. Legg-Perthes underwent 3D-HR-SPECT and 16 of them MRT examination. In all 28 children diagnostic assessment of M. Legg-Perthes could be achieved by HR-SPECT (incl. 4 negative cases of transient synovialitis). In 10 cases findings corresponded to MRT evaluation. The statement upon the area of necrosis was similar in both methods, but the reaction of bone to necrosis could be judged in a more differentiated way in HR-SPECT. Besides lower costs further advantages of HR-SPECT were found due to the deficit of movement artifacts (no sedation was required). A disadvantage of this method is X-ray contamination at a low level of 250-400 MBrg. To our experience HR-SPECT is an alternative to MRT for early diagnosis of M. Calve-Legg-Perthes. (orig.) [Deutsch] Bei Kindern mit klinischem Verdacht auf einen M. Perthes ist neben der Anamnese, der klinischen Untersuchung und dem konventionellen Roentgenbild heute haeufig die Durchfuehrung einer kernspintomographischen Untersuchung der naechste Schritt. Der hohen Treffsicherheit dieses Verfahrens stehen jedoch die vergleichsweise geringe Verfuegbarkeit, die hohen Kosten und die Notwendigkeit, waehrend der Untersuchung ruhig zu liegen, was vor allem juengeren Kindern nicht immer gelingt, gegenueber. Ziel unserer Studie war es, die diagnostische Wertigkeit der 3D-HR-SPECT (High Resolution - Single Photonen Emission Computed Tomography) als alternatives Verfahren zur MRT zu ueberpruefen. Hierzu wurden 28 Kinder im Alter von 2-12 Jahren mit klinischem

  15. Die Wertigkeit von Bildinformationskategorien und ihre Rolle bei der Beurteilung von Bildern als Medien

    OpenAIRE

    Wieczorek, Ulrich

    1997-01-01

    Die Wertigkeit von Bildinformationskategorien und ihre Rolle bei der Beurteilung von Bildern als Medien. - In: Die Geographiedidaktik ist tot, es lebe die Geographiedidaktik : Festschr. zur Emeritierung von Josef Birkenhauer / hrsg. von Friedhelm Frank ... - München, 1997. - S. 253-271. - (Münchner Studien zur Didaktik der Geographie ; 8)

  16. Ethnographische Filme und die Darstellung von Frauen

    Directory of Open Access Journals (Sweden)

    Judith Keilbach

    2002-03-01

    Full Text Available In dieser Filmographie werden ethnographische und koloniale Filme aus dem Bestand des Nederlands Filmmuseum kommentiert, die für die Frage nach der Darstellung von Frauen und Geschlechterverhältnissen von Interesse sind.

  17. John von Neumann selected letters

    CERN Document Server

    2005-01-01

    John von Neuman was perhaps the most influential mathematician of the twentieth century, especially if his broad influence outside mathematics is included. Not only did he contribute to almost all branches of mathematics and created new fields, but he also changed post-World War II history with his work on the design of computers and with being a sought-after technical advisor to many figures in the U.S. military-political establishment in the 1940s and 1950s. The present volume is the first substantial collection of (previously mainly unpublished) letters written by von Neumann to colleagues, friends, government officials, and others. The letters give us a glimpse of the thinking of John von Neumann about mathematics, physics, computer science, science management, education, consulting, politics, and war. Readers of quite diverse backgrounds will find much of interest in this fascinating first-hand look at one of the towering figures of twentieth century science.

  18. An Invitation from Lars von Trier

    DEFF Research Database (Denmark)

    Skadhauge, Troels; Tønder, Lars

    2015-01-01

    Translation of Martin Krasnik interview with Lars von Trier broadcast on DR2, January 12, 2015. The following is an interview of Lars von Trier (LvT) by the Danish journalist Martin Krasnik (MK). The interview took place in Lars von Trier’s home in Brede, a small town just outside Copenhagen...

  19. Management von Anforderungsdokumenten mittels semantischer Wikis

    OpenAIRE

    Decker, B.

    2005-01-01

    Im Projekt RISE wird untersucht, wie durch den Einsatz von Wikis das Anforderungsmanagement von Software Projekten agil unterstützt werden kann. Um das oftmals chaotische Wachstum von Inhalten in den Griff zu bekommen, werden maschinelle Lernverfahren genutzt, um Inhalte zu strukturieren und dem Benutzer zugänglich zum machen.

  20. Multivariat-statistische Auswertung von energiedispersiven Röntgenfluoreszenzspektren zur Identifizierung von Substanzen

    OpenAIRE

    Kessler, Thorsten

    2001-01-01

    Es wurde der Einsatz von multivariater Auswertung von energiedispersiven Röntgenfluoreszenzanalyse (EDRFA) -Spektren für die Zuordnung von Substanzen getestet. Hierbei erfolgten u. a. Untersuchungen zur Zuordnungsanalytik von Chemikalien ohne und mit Verpackungen. Zur Untersuchung von Chemikalien mit Verpackung wurden ausschließlich anorganische, feste Chemikalien in z.T. unterschiedlicher Verpackung herangezogen. Zusätzlich sollte ein Vergleich zwischen Messungen durch gleiche Verpackungen u...

  1. Homopteren von Java, gesammelt von Herrn Edw. Jacobson

    NARCIS (Netherlands)

    Melichar, L.

    1914-01-01

    Herr Edw. Jacobson hat auf Java in den Jahren 1908— 1910 Homopteren gesammelt und mir die Bearbeitung des gesammelten Materiales übertragen. Die Jacobson’sche Ausbeute ist insoferne bemerkenswert, als dieselbe viele Mikrohomopteren enthält, welche von nichtfachkundigen Sammlern gewöhnlich nicht

  2. Prevention, screening and therapy of thyroid diseases and their cost-effectiveness; Praevention, Screening und Therapie gutartiger Schilddruesenerkrankungen unter dem Aspekt von Kosten und Nutzen

    Energy Technology Data Exchange (ETDEWEB)

    Dietlein, M.; Moka, D.; Schmidt, M.; Theissen, P.; Schicha, H. [Koeln Univ. (Germany). Klinik und Poliklinik fuer Nuklearmedizin

    2003-10-01

    Cost-effectivness analyses focused on benign thyroid diseases are under-represented in the literature. The calculation of costs per additionally gained life year is difficult: The benefit of prevention is shifted into the distant future. The influence of an untreated subclinical thyroid disease on life expectancy can only be demonstrated by a long-term follow-up and by epidemiological databases. Iodine supplementation and programs for the prevention of tobacco smoking (primary prevention) are very cost-effective. Smoking increases the risk both of multinodular goiter and of Graves' disease. Screening programs (secondary prevention) are discussed for the laboratory parameters thyrotropin (TSH), calcium and calcitonin. TSH testing seems to be very cost-effective for epidemiological considerations in a certain lifespan (newborn, pregnancy, postpartal), older persons, hospitalisation due to acute diseases and in persons with previously elevated TPO-antibodies for TSH-values >2 mU/l, but dedicated cost-effectiveness analyses are lacking. On the other hand, the cost-effectiveness of a routine TSH testing beyond the age of 35 years has been shown by a high-quality decision analysis. Therapeutic strategies (tertiary prevention) aim at the avoidance of complications (atrial fibrillation, myocardial infarction, death for cardiac reasons) and of iatrogenic complications. Examples of a tertiary prevention are: firstly the definitive therapy of Graves' disease in patients who have on increased risk of relapse after antithyroid drugs (ATD), secondly the radioiodine therapy for subclinical hyperthyroidism and the radioiodine therapy of large goiters in older patients or in patients suffering from a relevant comorbidity. Cost-effectiveness analyses for different therapeutic strategies of Graves' disease were published using a lifelong time-horizon. The ablative radioiodine dose-regime is cost-effective as a fist line therapy if the risk of relapse after ATD exceeds

  3. Imaging technique and current status of valvular heart disease using cardiac MRI; Untersuchungstechniken und Stellenwert der MRT bei der Diagnostik von Herzklappenerkrankungen

    Energy Technology Data Exchange (ETDEWEB)

    Lotz, J.; Sohns, J.M. [Universitaetsmedizin Goettingen, Georg-August-Universitaet, Institut fuer Diagnostische und Interventionelle Radiologie, Goettingen (Germany)

    2013-10-15

    The main indications for cardiac magnetic resonance imaging (MRI) in the evaluation of valvular heart disease are pathologies of the aortic and pulmonary valve. For mitral and tricuspid valve pathologies MRI is not the first line modality as these are usually well visualized by echocardiography. The advantages of MRI in valvular heart disease are a high reliability in the evaluation of ventricular volumes and function as well as the assessment of the perivalvular arterial or atrial structures. This reliability and the limitless access to any imaging plane partially compensates for the lower temporal and spatial resolution in comparison to echocardiography. In patients with congenital heart disease, cardiac MRI is established as a valuable diagnostic tool in daily clinical management, especially for the evaluation of pulmonary valve defects. Nevertheless, echocardiography remains the first-line diagnostic imaging tool for the foreseeable future. (orig.) [German] Die Untersuchung der Herzklappen in der MRT umfasst derzeit v. a. die Aorten- und Pulmonalisklappe. Pathologien der atrioventrikulaeren Klappen bilden demgegenueber nur selten die zentrale Fragestellung einer kardialen MRT-Untersuchung, da diese normalerweise einer echokardiographischen Untersuchung gut zugaenglich sind. Die Staerke der MRT ist die hohe Zuverlaessigkeit, mit der neben der Klappenmorphologie und -funktion die Funktionsparameter des jeweiligen Ventrikels und die Morphologie der nachgeschalteten Arterien bestimmt werden koennen. Dadurch kann die MRT die prinzipielle Schwaeche in der Orts- und Zeitaufloesung gegenueber der Echokardiographie teilweise kompensieren. Bei Patienten mit kongenitalen Herzvitien ist die MRT-basierte Klappendiagnostik fester Bestandteil des klinischen Managements. Das gilt besonders fuer die Evaluation der Pulmonalisklappe. Die Echokardiographie bleibt absehbar die Modalitaet der ersten Wahl fuer die Klappendiagnostik am Herzen. (orig.)

  4. Replikation von µ-Peptidarrays

    OpenAIRE

    Striffler, Jakob

    2014-01-01

    In der vorliegen Arbeit wurde ein Verfahren erarbeitet und mit Photometrie, XPS und ToF-SIMS charakterisiert, mit dem durch mikropartikelbasierte Verfahren hergestellte µ-Peptidarrays von Syntheseoberflächen abgespalten und auf beliebige Zieloberflächen transferiert werden können.

  5. Von Krahl ON! / Terje Metsavas

    Index Scriptorium Estoniae

    Metsavas, Terje

    2008-01-01

    Madli Pesti ja Terje Metsavas kohtusid Von Krahli teatri tehnilise meeskonnaga teatri black-box-saalis. Teatri tehniline meeskond on tehnikadirektor Enar Tarmo, video- ja multimeediaga tegelev Lauri Sepp, tehnikud Janno "John" Jaanus, Oliver Kulpsoo ja Allan Räim. Selles, kuidas sünnivad "tehnikaimed" ja kuidas lahendatakse lavastuslikke projekte

  6. Integration von Produktion und Instandhaltung

    OpenAIRE

    Hans-Böckler-Stiftung

    2003-01-01

    Die Faktoren Produktqualität und Kosten-Nutzen-Verhältnis sind entscheidend im Wettbewerb. Um diese Faktoren zu optimieren, suchen die Unternehmen nach innovativen Modellen der Integration von Produktion und Instandhaltung. Über Konzeption und Erprobung solcher Modelle berichtet eine Arbeitsgruppe der Engeren Mitarbeiter der Arbeitsdirektoren Stahl Fachausschuss 1/00.

  7. [Friedrich Ludwig von Maydelli pildid Baltimaade ajaloost = Friedrich Ludwig von Maydells Baltische Geschichte in Bildern = Friedrich Ludwig von Maydell ́s Baltic history in images] / Ulrike Plath

    Index Scriptorium Estoniae

    Plath, Ulrike, 1972-

    2014-01-01

    Arvustus: Friedrich Ludwig von Maydelli pildid Baltimaade ajaloost = Friedrich Ludwig von Maydells Baltische Geschichte in Bildern = Friedrich Ludwig von Maydell ́s Baltic history in images. Eesti kunstimuuseum, Kadrioru kunstimuuseum. Tallinn 2013

  8. Laboratory testing for von Willebrand's disease: an assessment of current diagnostic practice and efficacy by means of a multi-laboratory survey. RCPA Quality Assurance Program (QAP) in Haematology Haemostasis Scientific Advisory Panel.

    Science.gov (United States)

    Favaloro, E J; Smith, J; Petinos, P; Hertzberg, M; Koutts, J

    1999-10-01

    We report an evaluation of current laboratory practice for the diagnosis of von Willebrand's disease (VWD) by means of a multilaboratory survey. This assessment was undertaken with the RCPA Quality Assurance Program (QAP) in Haematology, which covers a wide geographic area encompassing Australia, New Zealand and Asia. A total of 25 laboratories actively involved in testing for VWD were selected to participate in a sample testing assessment exercise. Samples comprised 10 plasmas: (i) a normal plasma pool (in duplicate), (ii) this pool diluted to 50% (in duplicate), (iii) a normal individual (X1), (iv) severe Type 1 VWD (X1), (v) Type 2B VWD (x2 unrelated donors), (vi) Type 3 VWD (x1), (vii) Type 2A VWD (x1). Laboratories were asked to perform all tests available to them in order to establish a laboratory diagnosis of VWD, and then to comment on the possibility or otherwise of VWD. Overall findings indicated a wide variation in test practice, in the effectiveness of various test procedures in detecting VWD, and in the ability of various composite test panels to identify type 2 VWD subtypes. Firstly, while all laboratories (n = 25) performed tests for FVIII:C activity, von Willebrand factor 'antigen' (VWF:Ag) and a functional VWF assay [using the ristocetin cofactor assay (VWF:RCo; n = 23) and/or the collagen binding assay (VWF:CBA; n = 12)], only three laboratories carried out VWF:Multimer analysis. Secondly, for the three quantitative VWF assays, 10/25 (40%) laboratories performed all three, whereas 15/25 (60%) performed only two [VWF:Ag and VWF:RCo (n = 13); VWF:Ag and VWF:CBA (n = 2)]. Thirdly, a variety of assay methodologies were evident for VWF:Ag [ELISA, electro-immuno diffusion (EID), latex immuno-assay (LIA), and VIDAS assay] and VWF:RCo (platelet agglutination/'aggregometry' and a 'functional VWF:RCo-alternative' ELISA assay). Between method analysis for the quantitative VWF assays showed that the VWF:RCo yielded the greatest degree of inter

  9. Von den Veranschaulichungsmethoden in den Rundfunkpredigten von der Heiligkreuz- Kirche

    Directory of Open Access Journals (Sweden)

    Witold Ostafiński

    2004-12-01

    Full Text Available In der gegenwärtigen Verkündigung des Wortes Gottes spielt die Veranschaulichung der Überlieferung eine nicht zu überschätzende Rolle. Vor diesem Hintergrund wird es sehr wichtig, dass man den Predigern nicht nur auf die Funktion des Bildes in der Verkündigung, sondern auch auf die Art und Weise seines Entstehens auf der narrativen Ebene aufmerksam macht. Während die Kenner der Homiletik die Notwendigkeit des Auftretens der Bildeigenschaften in den Predigten betonen, erinnern sie gleichzeitig daran, dass der moderne, von der „Bild-Zivilisation“ gestaltete Mensch, „immer mehr sehen als hören“ möchte. Der vorliegende, die Rundfunkpredigten von der Heiligkreuz-Kirche analysierende Aufsatz, nimmt auf und bringt zugleich nahe die Problematik der Notwendigkeit des Bildes in der Wort-Gottes-Verkündigung. Der Artikel stellt die Erzählungsform als eine wichtige Möglichkeit der Glaubensüberlieferung dar, behandelt das Problem der Aktualisierung evangelischen Werte, bringt das von den Predigten gestaltete synästhetische Weltbild nahe und analysiert die Rolle des Zitats und Beispiels (exemplum bei der Gestaltung der Bildhaftigkeit einer Predigt. Darüber hinaus überprüft der Aufsatz in seinem letzten Teil die in den gegenwärtigen Rundfunkpredigten meist verwendeten Redefiguren

  10. Analyse von Schnittstellenkompatibilität von Steuergeräten auf Basis von MSC-Beschreibungen

    OpenAIRE

    Ma, Zheng

    2008-01-01

    In modernen Fahrzeugen befindet sich eine Vielzahl von Steuergeräten, die verschiedenste Funktionen, wie z.B. das Antiblockiersystem (ABS) realisieren. Die Funktionalitäten von Steuergeräten werden heute mit unterschiedlichen Methoden beschrieben. Eine dieser Methoden sind Message Sequence Charts (MSCs). Aufgrund der Freiheitsgrade von MSCs gibt es verschiedene Möglichkeiten gleiche Funktionalität unterschiedlich zu beschreiben. In dieser Arbeit wird eine Methode definiert, wie...

  11. Männlichkeiten von rechts

    Directory of Open Access Journals (Sweden)

    Marc Gärtner

    2004-11-01

    Full Text Available Oliver Geden, Doktorand am Institut für europäische Ethnologie der Humboldt Universität Berlin untersucht Männlichkeitskonzepte im zeitgenössischen Rechtspopulismus – ein Thema, zu dem überraschend lange keine einschlägigen Studien vorlagen. Anhand von Parteimedien und Interviews rekonstruiert er strategische Positionen zu Männlichkeit und Geschlechterverhältnissen auf unterschiedlichen Organisationsebenen der Österreichischen „Haider-Partei“ FPÖ. Statt eines monolithischen „Neo-Macho-Projektes“ findet er im Kern zwar ähnliche Traditionalisierungsbemühungen auf allen Ebenen, je nach Kontext aber differenzierbare pragmatische, verunsicherte und neo-naturalistische Perspektiven auf die Geschlechterthematik. Durch die Einbeziehung von Gruppeninterviews im Nachwuchsverband der Partei erhält die Publikation eine besondere Brisanz. Geden analysiert die methodischen und forschungsethischen Implikationen ebenso hervorragend wie das Kernthema selbst.

  12. Methode zur Entwicklung von Prozessportalen

    OpenAIRE

    Alt, Rainer; Caesar, Marc; Leser, Florian; Österle, Hubert; Puschmann, Thomas; Reichmayr, Christian; Zurmuehlen, Rudolf

    2004-01-01

    Die in Kapitel 2 dargestellte Architektur zeigt Gestaltungselemente auf den drei Ebenen Strategie, Prozess und System. Zahlreiche Beispiele in den darauf folgenden Kapiteln haben die Anwendung dieser Architektur gezeigt. Um ein intuitives Vorgehen bei Entwurf und der Umsetzung von Echtzeit-Portalen zu vermeiden, sollen die bei den Fallbeispielen erzielten Erkenntnisse und Erfahrungen in ein systematisches Vorgehen einfliessen. Obgleich sich der Entwurf einer Architektur für das Echtzeit-Unter...

  13. Einfluss von Hypoxie auf das Transkriptom und das mitochondriale Proteom von Arabidopsis thaliana

    OpenAIRE

    Willeke, Claudia

    2011-01-01

    Hypoxie beeinflusst Wachstum und Entwicklung von Pflanzen. Da Sauerstoff als finaler Elektronenakzeptor der Atmungskette dient, sind Mitochondrien möglicherweise an der Wahrnehmung von Hypoxie und der Weiterleitung des Signals durch retrograde Regulierung beteiligt. In dieser Arbeit wurden daher die Auswirkungen von Hypoxie auf das Transkriptom und das mitochondriale Proteom von Arabidopsis thaliana untersucht. Zu diesem Zweck wurden Inkubationssysteme für Zellkulturen, Keimlinge und Pflanzen...

  14. Dr. von Braun Briefing Walt Disney

    Science.gov (United States)

    1965-01-01

    Dr. von Braun began his association with Walt Disney in the 1950s when the rocket scientist appeared in three Disney television productions related to the exploration of space. Years later, Dr. von Braun invited Disney and his associates to tour the Marshall Space Flight Center (MSFC) in Huntsville, Alabama. This photograph is dated April 13, 1965. From left are R.J. Schwinghamer from the MSFC, Disney, B.J. Bernight, and Dr. von Braun.

  15. Offenes Baukastensystem zur effizienten Dimensionierung von Materialflusssystemen

    OpenAIRE

    Meinhardt, Ingolf; Marquardt, Hans-Georg

    2007-01-01

    Die optimale Gestaltung logistischer Systeme und Prozesse bekommt eine immer größere Bedeutung für die Wirtschaftlichkeit und Wettbewerbsfähigkeit von Unternehmen. Für Einzelkomponenten von Materi-alflusssystemen sind neben exakten analytischen Verfahren auch Näherungslösungen und Ersatzmodelle in Form von Polynomen, neuronalen Netzen oder zeitdiskreten Verfahren vorhanden, mit denen eine gute Nachbildung des Verhaltens dieser Komponenten möglich ist. Ziel des Baukastensystems ist es, für...

  16. Die filmstilistische Darstellung von Klaras Gehbehinderung im Kinderfilm "Heidi"

    Directory of Open Access Journals (Sweden)

    Maria Ohrfandl

    2016-09-01

    Full Text Available Dieser Beitrag soll die Forschungslücke zur Darstellung von Behinderungen speziell im Kinderfilm im deutschsprachigen Raum füllen. Auf Basis einer neoformalistisch orientierten Filmanalyse nach Bordwell und Thompson (2008, wird anhand von drei Filmsequenzen eine mögliche Lesart des Kinderfilms "Heidi" (Marcus 2005 entwickelt, um die filmstilistische Darstellung der Gehbehinderung des Mädchens Klara zu untersuchen. Die Ergebnisse werden mit theoretischen Überlegungen zur Problematik der Begriffsbestimmung von Körperbehinderung sowie zur Mobilität und Selbstbestimmung von Menschen mit Körperbehinderungen in Bezug gesetzt. Dabei zeigt sich im Wesentlichen, dass im Kinderfilm "Heidi" (Marcus 2005 Klaras Gehbehinderung als 'heilbare Krankheit' und der Rollstuhl als Einschränkung von Mobilität begriffen wird. Außerdem unterliegt Klara aufgrund ihrer Körperbehinderung überwiegend der Fremdbestimmung von Erwachsenen. Zur Klärung wahrscheinlicher filmischer Bildungspotenziale werden die Ergebnisse schließlich anhand der von Jörissen und Marotzki (2009 entwickelten Orientierungsdimensionen "Wissens-, Handlungs-, Grenz- und Biographiebezug" diskutiert. This article aims to bridge a knowledge gap by showing ways of representing disabilities in the German children film genre. Using Bordwell and Thomson's (2008 neoformalistically orientated film analysis approach, the movie "Heidi" (Marcus 2005 is analysed. Bordwell and Thompson's (2008 method is applied to three sequences of "Heidi" in order to present an interpretation and to discuss the representation of Clara's walking impediment. The results are then associated with theoretical considerations regarding the mobility and self-determination of people with disabilities and problematic definitions of the term 'physical disability'. It is shown that characters in the children's film "Heidi" (Marcus 2005 regard Clara's walking impediment as a 'curable disease' and the wheelchair as a limitation

  17. Von Hippel-Lindau Disease (VHL)

    Science.gov (United States)

    ... ALS) Information Page NINDS Anencephaly Information Page NINDS Angelman Syndrome Information Page NINDS Antiphospholipid Syndrome Information Page ... ALS) Information Page NINDS Anencephaly Information Page NINDS Angelman Syndrome Information Page NINDS Antiphospholipid Syndrome Information Page ...

  18. Modellierung von Anwenderverhalten im Social Semantic Web

    Science.gov (United States)

    Ulbrich, Armin; Höfler, Patrick; Lindstaedt, Stefanie

    Ziel dieses Kapitels ist es, gemeinsame Verwendungsszenarien des Semantic Web und des Social Web zu identifizieren und zu benennen. Dabei wird ein Teilaspekt des Themengebiets im Detail betrachtet: die Nutzung von Services, die Beobachtungen des Verhaltens von Anwendern analysieren, um daraus maschinell interpretierbare Informationen zu erhalten und diese als Modelle zu organisieren. Es werden zunächst einige Eigenschaften und Unterscheidungsmerkmale von Anwenderverhalten und organisierten Modellen dargestellt. Anschließend wird der mögliche wechselseitige Nutzen von Anwenderverhalten und Modellen diskutiert. Den Abschluss bildet eine Betrachtung einiger exemplarischer Software-Services, die heute schon verwendet werden, um Anwenderverhalten in Modelle überzuführen.

  19. The Social Science of Carl von Clausewitz

    Science.gov (United States)

    2006-01-01

    The Social Science of Carl von Clausewitz JANEEN KLINGER C arl von Clausewitz’s great, unfinished book On War is well-known as be- ing prone to...COVERED 00-00-2006 to 00-00-2006 4. TITLE AND SUBTITLE The Social Science of Carl von Clausewitz 5a. CONTRACT NUMBER 5b. GRANT NUMBER 5c...Raymond Aron, Clausewitz: Philos- opher of War (New York: Simon and Schuster, 1983), p. 206. 88 Parameters 2. Carl Von Clausewitz , On War, ed. and trans

  20. Die naturräumliche Gliederung von Schwaben

    OpenAIRE

    Fischer, Klaus

    1985-01-01

    Die naturräumliche Gliederung von Schwaben. - In: Historischer Atlas von Bayerisch-Schwaben / hrsg. von Hans Frei ... - 2., neu bearb. u. erg. Aufl. - Augsburg : Schwäb. Forschungsgem. - Lifg. 2. (1985). - Kt. II, 7.

  1. Acquired Von Willebrand’s Syndrome in Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Sara Taveras Alam

    2014-01-01

    Full Text Available Acquired von Willebrand syndrome (AVWS is an uncommon, underdiagnosed, and heterogeneous disease which is increasingly recognized as a cause of bleeding diatheses. Systemic lupus erythematosus (SLE is an infrequent cause of AVWS. Herein, we report a case of AVWS diagnosed during the initial presentation of SLE in a previously healthy young man with no family history of bleeding diathesis who presented with worsening epistaxis, gastrointestinal bleeding, and anasarca. He was found to have severe anemia and prolonged activated partial thromboplastin time (aPTT with severely decreased levels of von Willebrand factor (VWF measurements in addition to markedly decreased factor VIII levels. Further evaluation revealed nephrotic syndrome and interstitial lung disease due to SLE. He initially received combination therapy with intravenous immunoglobulin (IVIG and von Willebrand factor/factor VIII concentrates without significant improvement. Treatment with steroids, cyclophosphamide, and rituximab was followed by clinical improvement evidenced by cessation of bleeding. The short follow-up did not allow us to definitely prove the therapeutic effect of immunosuppressive treatment on AVWS in SLE patients. This case adds to the literature supporting the relationship between AVWS and SLE and highlights the importance of combination therapy in the treatment of severe AVWS as well as the role of IVIG, cyclophosphamide, and rituximab in AVWS associated with SLE.

  2. Planung von CLIL-Unterricht

    Directory of Open Access Journals (Sweden)

    Josef Leisen

    2015-10-01

    Full Text Available Der Beitrag formuliert die Sprachlernbedingungen und die Leitlinien des Sprachlernens im Fach und nennt Merkmale des guten integrierten Fach- und Sprachlehrens. Ausgehend von den Sprachproblemen, die sich im CLIL-Unterricht auftun, wird der sprachsensible CLIL-Unterricht definiert, und es werden Anregungen zur Gestaltung gegeben. Sprachliche Standardsituationen umfassen die kommunikativen Situationen im CLIL-Unterricht, die beim fachlichen Lernen auftreten und von der CLIL-Lehrkraft professionell bewältigt werden müssen. Im Beitrag wird ein Lehr-Lern-Modell ausführlich beschrieben und erläutert. Die Steuerungen von Sprachlernprozessen im CLIL durch Aufgabenstellungen, Methoden-Werkzeuge, Moderation und Diagnose/Rückmeldung werden ausführlich beschrieben. Die Planung einer Lernlinie in sechs Schritten wird dargestellt und erläutert. Planungsraster für CLIL-Lernlinien schließen den Beitrag ab. The article sets out the conditions for language learning in general as well as guiding principles of language learning in the subject and puts forward criteria for successfully integrated content and language learning. Based on typical language problems arising in CLIL-classrooms, the language-sensitive CLIL-classroom is defined and organisational suggestions are given. Linguistic standard situations comprise the communicative situations in the CLIL-classroom which are encountered in subject-related learning and have to be professionally mastered by the CLIL-teacher. The article extensively describes and explains a model of teaching and learning that can be used for the CLIL-classroom. It gives detailed information on managing language learning processes in CLIL by means of tasks, resources and methods as well as discourse facilitation, evaluation and feedback. In addition, a six-step lesson unit is outlined and explained. The article concludes by giving a planning matrix for CLIL lesson units.

  3. Homoepitaxie von ZnO

    OpenAIRE

    Neumann, Christian

    2006-01-01

    Die Arbeit beschäftigt sich mit der homoepitaktischen Darstellung von ZnO-Dünnschichten. Die Arbeit beginnt mit einer theoretischen Betrachtung des Materialsystems und den daraus für eine erfolgreiche Epitaxie zu ziehenden Schlüssen. Es wurde eine Temperbehandlung zur Oberflächenmodifikation kommerziell erhältlicher ZnO-Einkristallsubstrate erarbeitet, die für eine erfolgreiche Epitaxie erforderlich ist. Im Anschluß daran wird die Entwicklung eines CVD-Prozesses mit metallischer Zin...

  4. Riidaja mõis / Elma von Stryk

    Index Scriptorium Estoniae

    Stryk, Elma von

    2006-01-01

    renoveerimise ootel barokne puithäärber (1762), kompleksi ümbritsev kivimüür, maakividest hobusetall, pargivaade, punastest tellistest neogooti stiilis kabel (1864), mis taastati von Strykkide perekonna toel. 11 ill.; fotod: Raivo Tasso; kabelis on Elma von Stryki loodud Kristust kujutav altarimaal

  5. Sorten- und Nachbaueignung von Hafer und Weizen

    OpenAIRE

    Spieß, Hartmut

    2000-01-01

    Der Beitrag gibt anhand alter und neuer Sortenvergleiche von Hafer und Weizen Einblick in die Problematik ökologischer Züchtung und Sortenwahl. Dabei geht es neben spezifischen Sorteneigenschaften um die Ausgewogenheit von Ertrags- und Qualitätsbildung im Verhältnis zur Pflanzengesundheit.

  6. Von Willebrand factor for menorrhagia: a survey and literature review.

    Science.gov (United States)

    Ragni, M V; Machin, N; Malec, L M; James, A H; Kessler, C M; Konkle, B A; Kouides, P A; Neff, A T; Philipp, C S; Brambilla, D J

    2016-05-01

    von Willebrand disease (VWD) is the most common congenital bleeding disorder. In women, menorrhagia is the most common bleeding symptom, and is disabling with iron deficiency anaemia, high health cost and poor quality of life. Current hormonal and non-hormonal therapies are limited by ineffectiveness and intolerance. Few data exist regarding von Willebrand factor (VWF), typically prescribed when other treatments fail. The lack of effective therapy for menorrhagia remains the greatest unmet healthcare need in women with VWD. Better therapies are needed to treat women with menorrhagia. We conducted a survey of US haemophilia treatment centres (HTCs) and a literature review using medical subject heading (MeSH) search terms 'von Willebrand factor,' 'menorrhagia' and 'von Willebrand disease' to assess the use of VWF in menorrhagia. Analysis was by descriptive statistics. Of 83 surveys distributed to HTC MDs, 20 (24.1%) provided sufficient data for analysis. Of 1321 women with VWD seen during 2011-2014, 816 (61.8%) had menorrhagia, for which combined oral contraceptives, tranexamic acid and desmopressin were the most common first-line therapies for menorrhagia, whereas VWF was third-line therapy reported in 13 women (1.6%). Together with data from 88 women from six published studies, VWF safely reduced menorrhagia in 101 women at a dose of 33-100 IU kg(-1) on day 1-6 of menstrual cycle. This represents the largest VWD menorrhagia treatment experience to date. VWF safely and effectively reduces menorrhagia in women with VWD. A prospective clinical trial is planned to confirm these findings. © 2016 John Wiley & Sons Ltd.

  7. Measurement of thyrotropin receptor antibodies (TRAK) with a second generation assay in patients with Graves' disease; Die Bestimmung von Thyreotropin-Rezeptor-Antikoerpern (TRAK) mit einem Assay der zweiten Generation bei Patienten mit Morbus Basedow

    Energy Technology Data Exchange (ETDEWEB)

    Zoephel, K.; Wunderlich, G.; Franke, W.G. [Klinik und Poliklinik fuer Nuklearmedizin, Technische Univ. Dresden (Germany); Koch, R. [Inst. fuer Medizinische Informatik und Biometrie, Technische Univ. Dresden (Germany)

    2000-06-01

    Aim: The detection of TSH-receptor-antibodies (TRAb) in patients (pts) with Graves' disease (GD) is routinely used in nuclear medicine laboratories. It is performed by commercial, porcine radioreceptorassays (RRA) measuring TSH binding inhibitory activity. A second generation assay using the human, recombinant TSH-receptor was developed during the last years. The manufacturer composed this new assay as a coated tube RRA (CT RRA) and claimed a higher sensitivity for GD. Methods: TRAb was measured in 207 pts with various thyroid disorders and 205 healthy controls using the new coated tube RRA (Fa. B.R.A.H.M.S. Diagnostica GmbH, Berlin, Germany) as well as a conventional RRA (Fa. Medipan Diagnostica GmbH, Selchow, Germany): 60 pts suffering from GD showing a relapse after anti-thyroid drug treatment and before radioiodine therapy, 109 pts with disseminated autonomia (DA) and 38 pts suffering from Hashimoto's thyroiditis. A ROC-analysis was performed to find the optimal decision threshold level for positivity. Results: We found 42/60 TRAb-positive pts with GD in the established RRA (threshold 6 U/L) and 52/60 in the CT RRA, respectively. The sensitivity increased from 70% (RRA) to 86,7% (CT RRA). The CT RRA found 2 false positives (one Hashimoto's and one healthy control) and the RRA detected 3 Hashimoto's and 2 healthy controls as false positive. Conclusion: The increased sensitivity of CT RRA for GD provides an advantage compared to conventional RRA, especially in GD-patients relapsing afte antithyroid drug treatment. Functional sensitivity and Interassayvariation of CT RRA are very precisely compared to conventional RRA. Handling of the new assay is also improved. (orig.) [German] Ziel: Die Bestimmung der TSH-Rezeptorantikoerper (TRAK) bei Patienten mit Morbus Basedow ist fester Bestandteil der nuklearmedizinischen In-vitro-Diagnostik. Seit kurzem ist die Bestimmung mit einem TRAK-Assay moeglich, bei dem im Gegensatz zu den herkoemmlichen

  8. Disease-specific questionnaire for quality of life in patients with peripheral arterial occlusive disease in the stage of critical ischemia (FLeQKI): psychometric evaluation of its responsiveness and practicability. Pt. 2; Fragebogen zur gesundheitsbezogenen Lebensqualitaet von Patienten mit peripherer arterieller Verschlusskrankheit (pAVK) im Stadium kritischer Ischaemie (FLeQKI): Psychometrische Bestimmung von Veraenderungssensitivitaet und Praktikabilitaet. T. 2

    Energy Technology Data Exchange (ETDEWEB)

    Wohlgemuth, W.A.; Bohndorf, K.; Kirchhof, K. [Klinikum Augsburg (Germany). Klinikum fuer Diagnostische Radiologie und Neuroradiologie; Olbricht, W. [Bayreuth Univ. (Germany). Lehrstuhl fuer Mathematik VII; Klarmann, S. [Klinikum Augsburg (Germany). Klinik fuer Neurologie und Klinische Neurophysiologie; Engelhardt, M. [Bundeswehrkrankenhaus Ulm (Germany). Klinik fuer Gefaesschirurgie; Freitag, M.H. [Bayreuth Univ. (Germany). Inst. fuer Medizinmanagement und Gesundheitswissenschaften; Woelfle, K. [Klinikum Augsburg (Germany). Klinik fuer Gefaesschirurgie

    2007-12-15

    Purpose: To test the 'Questionnaire for Life Quality in Patients with Peripheral Arterial Occlusive Disease at the Stage of Critical Ischemia' (FLeQKI) in a prospective study with respect to responsiveness and practicability. Patients and methods: The responsiveness of the FLeQKI for therapy-induced changes of health related quality of life was prospectively determined in 65 consecutive patients with peripheral occlusive arterial disease at the stage of critical ischemia prior to percutaneous transluminal angioplasty (PTA) or bypass operation, and 1 month and 6 months after. 40 healthy individuals who were matched for age and questioned with the FLeQKI twice within 6 months served as the control. Additionally, all patients and healthy volunteers were questioned with the 'Medical Outcomes Study Group Short Form' (SF-36). In all patients, the ankle/brachial index (ABI) was measured along with each of the three interviews. To validate practicability, we measured the time needed to fill out the questionnaires and asked the patients to indicate on a visual analog scale (VAS) graduated from 0 - 10 how strenuous they found the questionnaires to be. For statistical analysis, two-sided paired t-tests were used. Results: The treatment group consisted of 35 men and 30 women with an age of 75.1 {+-} 7.0 years, and the control group was comprised of 21 men and 19 women with an age of 73.4 {+-} 7.8 years. In the control group, none of the FLeQKI scales showed significant changes over time (p > 0.05). In the pAVK group, all scales improved between the interviews before and 6 months after therapy. Changes were either significant (comorbidity, p < 0.05) or highly significant (all other scales, p < 0.005 or p < 0.0001). ABI increased above 0.5 (p < 0.05). With an average of 12 minutes, the FLeQKI required less time than the SF-36 with an average of 15.57 minutes (p < 0.05). Both questionnaires were evaluated with a VAS value of less than 1 (p > 0.05) as non

  9. Jacob Stael von Holstein ja palladionism eesti arhitektuuris / Ants Hein

    Index Scriptorium Estoniae

    Hein, Ants, 1952-

    2005-01-01

    Sõjaväeinsenerist ja arhitektist Jacob Stael von Holsteinist (1628, Pärnu-1679), tema kavandatud hoonetest ja rollist 17. sajandi II poole arhitektuuris. Fabian von Ferseni Toompeal Lossiplatsil asunud elamust (lammutati 1894) ja Maardu mõisahoonest, Hans von Ferseni Mäo mõisahoonest Järvamaal, Otto Wilhelm von Ferseni majast Toompeal, Axel von Roseni majast Tallinnas Pikk t. 28 (1670-74), Otto Reinhold von Taube majast Tartus (hävinud), J. S. von Holsteini enda hoonetest: majast Tallinnas Toompea 1, Anija mõisahoonest, elamust Riias jm. Bibliograafia lk. 452

  10. Neues über das Tertiär von Java und die mesozoischen Schichten von West-Borneo

    NARCIS (Netherlands)

    Martin, K.

    1888-01-01

    Die Monographie, welche unter dem Titel Die Fossilien von Java herausgegeben wird ¹), ist so weit fortgeschritten, dass sich aus den in ihr beschriebenen Gastropoden bereits eine Anzahl von Schlussfolgerungen ableiten lässt, welche für die in Bearbeitung begriffene, geologische Karte von Java von

  11. Indikatorenbasierte Bewertung von Entwurfsentscheidungen auf Systemebene

    Science.gov (United States)

    Freier, M.; Wenzler, A.; Mayer, T.; Gerlach, J.; Rosenstiel, W.

    2013-07-01

    Bei dem Entwurf von anwendungsspezifischen integrierten Schaltungen muss ein Entwickler zusätzliche Funktionen integrieren und die zunehmende Komplexität bewältigen. Für die Reduzierung der Kosten bleibt weiterhin die Verkürzung der benötigten Entwicklungszeit ein Ziel. Ein entscheidender Faktor dabei ist die zuverlässige Absicherung von Entwurfsentscheidungen in einer frühen Entwurfsphase. In der vorliegenden Arbeit wird eine Methode vorgestellt, die eine Bewertung von Entwurfsentscheidungen bei Systemmodellen mittels automatisch ermittelter Indikatoren ermöglichen soll. Die Systemmodelle können mit der Entwurfsumgebung MATLAB/Simulink oder in SystemC beschrieben sein.

  12. Disease: H00559 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available description, gene) Maher ER, Neumann HP, Richard S von Hippel-Lindau disease: a clinical and scientific revi...amasaki I, Tamura K, Okuda H, Furihata M, Ashida S Von Hippel-Lindau disease: molecular pathological basis, clinic...al criteria, genetic testing, clinical features of tumors and treatment. Jpn J Clin Oncol 36:337-43 (2

  13. The Digital Von Fahrenheid Pyramid

    Science.gov (United States)

    Bura, M.; Janowski, J.; Wężyk, P.; Zięba, K.

    2017-08-01

    3D Scanners Lab from Digital Humanities Laboratory at the University of Warsaw initiated the scientific project, the purpose of which was to call attention to systematically penetrated and devastated pyramid-shaped tomb from the XVIII/XIX century, of family von Fahrenheid in Rapa in Banie Mazurskie commune (NE Poland). By conducting a series of non-invasive studies, such as 3D inventory using terrestrial laser scanning (TLS), thermal imaging, georadar measurements (around and inside the tomb) and anthropological research of mummified remains as well - the complete dataset was collected. Through the integration of terrestrial (TLS) and airborne laser scanning (ALS) authors managed to analyse the surroundings of Fahrenheid pyriamid and influence of some objects (like trees) on the condition and visibility of the Pyramids in the landscape.

  14. Khazanah: Friedrich Karl von Savigny

    Directory of Open Access Journals (Sweden)

    Atip Latipulhayat

    2015-04-01

    Full Text Available Friedrich Karl von Savigny (1779-1861 adalah ahli hukum Jerman yang juga dianggap sebagai salah satu Bapak hukum Jerman. Savigny adalah tokoh mazhab sejarah (historical school jurisprudence yang dikembangkannya pada paruh pertama abad ke 19. Dia juga dianggap sebagai pelopor kajian mengenai relasi antara perkembangan hukum dan sosial. Sebagai seorang pemikir hukum yang senantiasa kreatif dalam membuat terobosan-terobosan (trail-blazing legal scientist, Savigny memberikan kontribusi penting dalam perkembangan ilmu hukum dan bahkan terhadap ilmu sosial. Dari sekian banyak kontribusinya antara lain teorinya mengenai kontinuitas antara institusi hukum saat ini dengan institusi hukum masa lalu, meletakkan fondasi bagi kajian sosiologi hukum, dan menegaskan mengenai urgensi metode historis dalam kajian hukum.

  15. Evaluierung und Entwicklung von Methoden zur automatisierten Erfassung von Waldstrukturen aus Daten flugzeuggetragener Fernerkundungssensoren

    OpenAIRE

    Heurich, Marco

    2006-01-01

    Auf Grund der rasanten technologischen Entwicklung im Bereich der Sensorik sowie der Computerhard- und -software haben sich in den letzten Jahren neue Perspektiven zur Erforschung von Wäldern ergeben. Im Rahmen dieser durch die High-Tech-Offensive Bayern geförderten Arbeit wurde deshalb untersucht, inwieweit sich der Einsatz neuer Technologien zur automatisierten Erfassung von Waldstrukturen eignet. Als Voraussetzung dafür wurde zunächst ein Referenzgebiet zum Testen von Fernerkundungssensore...

  16. Spezifische Bindung von Aluminium und Eisen an den kationenselektiven Kanal MppA von Microthrix parvicella

    OpenAIRE

    Knaf, Tobias

    2013-01-01

    Schwermetallsalze wie beispielsweise Aluminium- oder Eisensalze werden in der Abwasserbehandlung zur Prävention und Bekämpfung von Blähschlamm, Schwimmschlamm und Schaumbildung verwendet. Dadurch kann eine Verbesserung der Schlammabsetzeigenschaften im Nachklärbecken erreicht werden. Übermäßiges Wachstum des grampositiven Bakteriums Microthrix parvicella gilt dabei als Hauptursache von Schlammabsetzproblemen und kann ebenfalls durch die Dosierung von schwermetallhaltigen Flockungs- und Fällun...

  17. Das Tumorsuppressorprotein p53 - Rolle bei der Induktion von Apoptose und bei der Replikation von Adenoviren

    OpenAIRE

    Koch, Philipp

    2003-01-01

    Das Tumorsuppressorgen p53 gehört zu den zentralen Steuereinheiten von Proliferation und Wachstum in der Zelle. Mutationen und funktioneller Verlust bei diesem Protein ist eng mit der Entstehung maligner Tumoren assoziiert. So kann man in über 50% aller soliden Tumoren Mutationen von p53 nachweisen. Es vermittelt seine Funktion hauptsächlich durch transkriptionelle Aktivierung p53-responsiver Gene. Folge der Aktivierung von p53 is...

  18. Johann Friedrich Wilhelm Adolf von Baeyer

    Indian Academy of Sciences (India)

    Home; Journals; Resonance – Journal of Science Education; Volume 19; Issue 6. Johann Friedrich Wilhelm Adolf von Baeyer: A Pioneer of Synthetic Organic Chemistry. Gopalpur Nagendrappa. General Article Volume 19 Issue 6 June 2014 pp 489-522 ...

  19. Von Onlineplattformen und mittelalterlichen Märkten

    Directory of Open Access Journals (Sweden)

    Claudius Gräbner

    2017-12-01

    Full Text Available Mit der zunehmenden Digitalisierung und Vernetzung ökonomischer Systeme haben plattformbasierte Interaktionsbeziehungen stark an Bedeutung gewonnen. Hier werden zwei oder mehr Nutzungsgruppen durch eine dritte Seite, die Plattform, zusammengebracht. Die Interaktion wird in solchen zweiseitigen Märkten von den Plattformbetreibern kontrolliert. Kreditkartensysteme, Softwaremärkte oder werbefinanzierte Online-Communities stellen typische Beispiele dar. Da die Analyse von zweiseitigen Märkten mit Gleichgewichtsmodellen aufgrund der algebraischen Struktur des Problems in der Regel nicht praktikabel ist, präsentieren wir im vorliegenden Beitrag ein agentenbasiertes Modell, welches in einem kürzlich erschienenen Artikel ausführlicher diskutiert wird. Anhand von illustrativen Beispielen verdeutlichen wir die Implikationen eines agentenbasierten Ansatzes für die Innovationsökonomik im Allgemeinen und das Studium von Technologieentwicklung im Besonderen.

  20. Ist Pop die Volksmusik von heute?

    OpenAIRE

    Arbeitskreis Studium Populärer Musik

    1986-01-01

    Zugegeben, die Frage, ob Pop die Volksmusik von heute sei, ist provokant. Genau das war beabsichtigt. Sehr schnell erhielten wir auf unser Vorhaben, ein Seminar zu diesem Thema zu veranstalten, eine Vielzahl von Rückmeldungen. Pop-, Rock- und Jazzspezialisten, Musikwissenschaftler und Musikotiunologen fühlten sich animiert, Stellung zu beziehen und ein Referat zu halten. Intensive Diskussionen schlossen sich an, beflügelt auch durch die ansprechende Atmosphdre in der stil...

  1. Begehrte Körper jenseits von Geschlecht?

    Directory of Open Access Journals (Sweden)

    Corinna Tomberger

    2001-03-01

    Full Text Available Obwohl die visuelle Konstruktion des männlichen Körpers im Mittelpunkt von Daniel Wildmanns Studie steht, gelingt es ihm paradoxerweise, die Kategorie Geschlecht weitgehend zu ignorieren. In seiner Analyse des Films Olympia von Leni Riefenstahl zeigt der Autor auf, wie der ‚arische‘ Körper als Objekt des Begehrens für das Publikum entworfen wird.

  2. Dr. von Braun With German Rocket Experimenters

    Science.gov (United States)

    1930-01-01

    Dr. von Braun was among a famous group of rocket experimenters in Germany in the 1930s. This photograph is believed to be made on the occasion of Herman Oberth's Kegelduese liquid rocket engine being certified as to performance during firing. From left to right are R. Nebel, Dr. Ritter, Mr. Baermueller, Kurt Heinish, Herman Oberth, Klaus Riedel, Wernher von Braun, and an unidentified person.

  3. 50th birthday of Christian von Ferber

    Directory of Open Access Journals (Sweden)

    2011-06-01

    Full Text Available On May 15, 2011 Christian von Ferber - member of the Editorial Board of "Condensed Matter Physics" and renowned expert in the fields of soft matter physics and complex systems - celebrated his 50th birthday. The Editorial board of CMP, colleagues and friends warmy congratulate Christian von Ferber and wish him and his family continuing good health and to enjoy many adventures and discoveries in his future scientific travels!

  4. Dr. von Braun With Management Team

    Science.gov (United States)

    1961-01-01

    Dr. von Braun is shown in this photograph, which was probably taken in the early 1960s, with members of his management team. Pictured from left to right are, Werner Kuers, Director of the Manufacturing Engineering Division; Dr. Walter Haeussermarn, Director of the Astrionics Division; Dr. William Mrazek, Propulsion and Vehicle Engineering Division; Dr. von Braun; Dieter Grau, Director of the Quality Assurance Division; Dr. Oswald Lange, Director of the Saturn Systems Office; and Erich Neubert , Associate Deputy Director for Research and Development.

  5. Hullud päevad von Krahlis

    Index Scriptorium Estoniae

    2006-01-01

    Von Krahli Teatris mängitakse neil päevi neli korda Peeter Jalaka lavastuses Tõnu Kõrvitsa kammeroopereid "Tuliaed" ja "Mu luiged, mu mõtted", mille aluseks on luuletaja Marie Heibergi saatus. Libreto autor on Maarja Kangro. Kammerooperid tulevad lavale Von Krahli Teatri ja Nargen Opera koostöös. Esitavad Kädy Plaas, Helen Lokuta, Nargen Opera koor ja Tallinna Kammerorkester, dirigent Tõnu Kaljuste

  6. Visualisierung von typisierten Links in Linked Data

    Directory of Open Access Journals (Sweden)

    Georg Neubauer

    2017-09-01

    Full Text Available Das Themengebiet der Arbeit behandelt Visualisierungen von typisierten Links in Linked Data. Die wissenschaftlichen Gebiete, die im Allgemeinen den Inhalt des Beitrags abgrenzen, sind das Semantic Web, das Web of Data und Informationsvisualisierung. Das Semantic Web, das von Tim Berners Lee 2001 erfunden wurde, stellt eine Erweiterung zum World Wide Web (Web 2.0 dar. Aktuelle Forschungen beziehen sich auf die Verknüpfbarkeit von Informationen im World Wide Web. Um es zu ermöglichen, solche Verbindungen wahrnehmen und verarbeiten zu können sind Visualisierungen die wichtigsten Anforderungen als Hauptteil der Datenverarbeitung. Im Zusammenhang mit dem Sematic Web werden Repräsentationen von zuhammenhängenden Informationen anhand von Graphen gehandhabt. Der Grund des Entstehens dieser Arbeit ist in erster Linie die Beschreibung der Gestaltung von Linked Data-Visualisierungskonzepten, deren Prinzipien im Rahmen einer theoretischen Annäherung eingeführt werden. Anhand des Kontexts führt eine schrittweise Erweiterung der Informationen mit dem Ziel, praktische Richtlinien anzubieten, zur Vernetzung dieser ausgearbeiteten Gestaltungsrichtlinien. Indem die Entwürfe zweier alternativer Visualisierungen einer standardisierten Webapplikation beschrieben werden, die Linked Data als Netzwerk visualisiert, konnte ein Test durchgeführt werden, der deren Kompatibilität zum Inhalt hatte. Der praktische Teil behandelt daher die Designphase, die Resultate, und zukünftige Anforderungen des Projektes, die durch die Testung ausgearbeitet wurden.

  7. [Temporal lobe epilepsy due to an intracranial meningocele in a patient with neurofibromatosis type 1].

    Science.gov (United States)

    Boughammoura, A; Daoussi, N; Younes, S; Kilani, M; Chebel, S; Hattab, M; Frih-Ayed, M

    2013-01-01

    Epilepsy has been rarely reported in patients with neurofibromatosis type 1 (formally known as von Recklinghausen disease), which may occur in 3 to 6% of cases. This condition is generally related to neuronal migration anomalies or cortical malformations. We report a case of temporal epilepsy secondary to temporal meningocele due to sphenoidal dysplasia in a patient who presented with neurofibromatosis type 1 and also discuss this association. © 2013 Elsevier Masson SAS. All rights reserved.

  8. Schwannomas in the head and neck: retrospective analysis of 21 patients and review of the literature

    Directory of Open Access Journals (Sweden)

    Erwin Langner

    Full Text Available CONTEXT AND OBJECTIVE: Schwannomas are benign neoplasms of the peripheral nerves originating in the Schwann cells. According to their cellularity, they can be subdivided into Antoni A or Antoni B types. They are rare and usually solitary, with clearly delimited capsules. They occur in the head and neck region in only 25% of the cases, and may be associated with Von Recklinghausen's disease. The present study retrospectively analyzed some data on this disease in the head and neck region and reviewed the literature on the subject. DESIGN AND SETTING: Retrospective study at Head and Neck Service, Universidade Estadual de Campinas. METHODS: Data on 21 patients between 1980 and 2003 were reviewed. The sites of cervical schwannomas and the intraoperative, histopathological and postoperative clinical status of these cases were studied. Diagnostic methods, type of surgery and association with neurofibromatosis were evaluated. RESULTS: The patients' ages ranged from 16 to 72 years. Four patients had a positive past history of type I neurofibromatosis or Von Recklinghausen's disease. The nerves affected included the brachial and cervical plexuses, vagus nerve, sympathetic chain and lingual or recurrent laryngeal nerve. The nerve of origin was not identified in six cases. Tumor enucleation was performed in 16 patients; the other five required more extensive surgery. CONCLUSION: Schwannomas and neurofibromas both derive from Schwann cells, but are different entities. They are solitary lesions, except in Von Recklinghausen's disease. They are generally benign, and rarely recur. The recommended surgical treatment is tumor enucleation.

  9. Effect of heavy metals and benzene on porphyrin synthesis of human epithelial larynx carcinoma cells (HEp-2); Wirkung von Schwermetallen und Benzol auf die Porphyrinsynthese von Humanen epithelialen Larynxkarzinomzellen (HEp-2)

    Energy Technology Data Exchange (ETDEWEB)

    Tumat, C.; Kath, H.G.; Perlewitz, J.; Manuwald, O. [Institut fuer Umweltmedizin, Erfurt (Germany); Herbarth, O. [UFZ - Umweltforschungszentrum Leipzig-Halle GmbH, Leipzig (Germany). Sektion Expositionsforschung und Epidemiologie

    2001-07-01

    Aim of the investigation was to prepare an environmental epidemiological test to detect the effects of pollutants by a pattern of porphyrin excretion in the urine. The task was to test whether lead, mercury, copper, zinc and benzene in environmentally relevant concentrations influence porphyrin synthesis of cells. The model used was the permanent epithelial larynx carcinoma cell line HEp-2, which was stimulated to synthesize porphyrins by adding {delta}-amino-levulinic acid ({delta}-ALA) in excess. 8- to 4-carboxyporphyrins and protoporphyrin IX were measured both in the cells and in the culture medium by means of high performance liqid cromatography (HPLC). The following pollutant effects were found: lead: extracellular increase of hexacarboxyporphyrin and intracellular decrease of heptacarboxy- and copro-porphyrin; mercury: extracellular decrease of heptacarboxyporphyrin and intracellular increase of coproporphyrin; copper: extracellular decrease of uro-, coproporphyrin and protoporphyrin IX, extracellular increase of heptacarboxyporphyrin and intracellular decrease of uro-, heptacarboxy- and coproporphyrin; zinc: extracellular increase of coproporphyrin; benzene: a tendency to extracellular increase of hexacarboxyporphyrin. The main result is the decrease in the concentrations of porphyrins caused by copper. This result is possibly linked to gastro-intestinal diseases among children due to drinking water from copper water pipes. (orig.) [German] Ziel der Untersuchungen war es, Vorarbeit zu leisten fuer die Entwicklung eines umwelt-epidemiologischen Tests, der es ermoeglicht, geringe Schadstoffwirkungen durch ein Porphyrin-Ausscheidungsmuster im Urin nachzuweisen. In diesem Zusammenhang bestand die Aufgabe zu pruefen, ob Blei, Quecksilber, Kupfer, Zink und Benzol in umweltmedizinisch relevanten Konzentrationen die Porphyrinsythese von Zellen beeinflussen. Als Untersuchungsmodell wurde die permanente humane epitheliale Larynxkarzinomzelllinie HEp-2 genutzt, die

  10. Privatiserung von Geschichte. Probleme einer differenzierten Aufarbeitung

    Directory of Open Access Journals (Sweden)

    Birthe Kundrus

    2000-11-01

    Full Text Available Ausgangspunkt der Untersuchung von Vera Neumann ist die These, daß im Krieg Erlebtes und Erlittenes in den Wiederaufbau- und Wirtschaftswunderjahren Westdeutschlands einem Thematisierungstabu unterlegen sei. Anhand von 50 Interviews, die in den 80er Jahren im Rahmen des von Lutz Niethammer geleiteten Projekts „Lebensgeschichte und Sozialgeschichte im Ruhrgebiet 1930–1960“ (LUSIR entstanden sind und die sie jetzt anhand der Tonbandprotokolle und Abschriften ein zweites Mal auswertet, möchte die Historikerin diese Verschüttungen aufspüren. Im Anschluß an Niethammers These der „Privatisierung von Geschichte“ will sie zeigen, daß seelische und körperliche Kriegsfolgen wie Deprivations- und Überlastungsgefühle, der Verlust von Angehörigen und Kriegsbeschädigungen „privatisiert“, d.h. an die Familien übertragen wurden. Dort seien dann in erster Linie die weiblichen Familienmitglieder mit der Versorgungs- und Pflegearbeit konfrontiert worden. An vier Fallbeispiele schließt sich eine detaillierte Auswertung des gesamten Interviewmaterials an. Diesen Teilen folgt ein Abschnitt zur staatlichen Kriegsopferversorgung in der frühen Bundesrepublik.

  11. An MRI Von Economo - Koskinas atlas.

    Science.gov (United States)

    Scholtens, Lianne H; de Reus, Marcel A; de Lange, Siemon C; Schmidt, Ruben; van den Heuvel, Martijn P

    2016-12-28

    The cerebral cortex displays substantial variation in cellular architecture, a regional patterning that has been of great interest to anatomists for centuries. In 1925, Constantin von Economo and George Koskinas published a detailed atlas of the human cerebral cortex, describing a cytoarchitectonic division of the cortical mantle into over 40 distinct areas. Von Economo and Koskinas accompanied their seminal work with large photomicrographic plates of their histological slides, together with tables containing for each described region detailed morphological layer-specific information on neuronal count, neuron size and thickness of the cortical mantle. Here, we aimed to make this legacy data accessible and relatable to in vivo neuroimaging data by constructing a digital Von Economo - Koskinas atlas compatible with the widely used FreeSurfer software suite. In this technical note we describe the procedures used for manual segmentation of the Von Economo - Koskinas atlas onto individual T1 scans and the subsequent construction of the digital atlas. We provide the files needed to run the atlas on new FreeSurfer data, together with some simple code of how to apply the atlas to T1 scans within the FreeSurfer software suite. The digital Von Economo - Koskinas atlas is easily applicable to modern day anatomical MRI data and is made publicly available online. Copyright © 2017 Elsevier Inc. All rights reserved.

  12. Von der Universitätsbibliothek Regensburg zum Bayerischen Bibliotheksverbund

    OpenAIRE

    Frankenberger, Rudolf

    1989-01-01

    Von der Universitätsbibliothek Regensburg zum Bayerischen Bibliotheksverbund : d. Entwicklung d. Bayer. Bibliotheksverbundes. - In: Bibliothekslandschaft Bayern : Festschr. für Max Pauer zum 65. Geburtstag / hrsg. von Paul Niewalda. - Wiesbaden : Harrassowitz, 1989. - S. 84-96

  13. Kerttu Wagner. Die historischen romane von Jaan Kross / Wolfgang Drechsler

    Index Scriptorium Estoniae

    Drechsler, Wolfgang, 1963-

    2001-01-01

    Arvustus: Wagner, Kerttu. Die historischen Romane von Jaan Kross : am Beispiel einer Untersuchung der deutschen und englischen Übersetzungen von "Professor Martensi ärasõit" (1984). Frankfurt am Main [etc.] : P. Lang, 2001.

  14. Gemeinsam hilfreich oder einsam lästig? Beurteilung von praktikumsbegleitenden Weblogs von angehenden Lehrpersonen

    Directory of Open Access Journals (Sweden)

    Nives Egger

    2017-02-01

    Full Text Available Weblogs werden an Hochschulen vermehrt zur Unterstützung von Lern- und Reflexionsprozessen eingesetzt, in der Lehrer/innenbildung auch zunehmend während der berufspraktischen Ausbildung. Die Studierenden beurteilen den Einsatz von Weblogs jedoch unterschiedlich. Dabei ist wenig bekannt, welchen Einfluss die Vergabe und das Erhalten von Peerfeedback auf den Beurteilungsprozess von praktikumsbegleitenden Weblogs sowie auf den Umfang von Blogpostings haben. Im folgenden Beitrag wird dieser Frage mittels einer Befragung von 74 angehenden Lehrpersonen nachgegangen, die praktikumsbegleitende Weblogs mit oder ohne Peerfeedback nutzten, um über herausfordernde Ereignisse im Praktikum zu reflektieren. Die Ergebnisse zeigen, dass Studierende, die Peerfeedback erhielten und andere Blogbeiträge kommentierten, den Weblogeinsatz nützlicher einschätzen sowie eine höhere Motivation und positivere Einstellung gegenüber den Einsatz von Weblogs aufweisen als Studierende, die ohne Peerfeedback bloggen. Keinen Einfluss hat Peerfeedback auf den Umfang der Blogbeiträge und auf die dafür aufgewendete Zeit.

  15. Polylaktidbeschichtung zur antithrombogenen Ausrüstung von Biomaterialien

    OpenAIRE

    Mebus, Siegrun

    2007-01-01

    In der Klinik etablierte Biomaterialien wurden mit einer Polylaktidbeschichtung und eingearbeiteten Antikoagulanzien ausgerüstet. Die mit einem in-vitro-Modell erarbeiteten Daten zeigen, daß der Arzneistoffträger innerhalb von 90 Tagen nicht abgebaut wurde. Die Freisetzung der Arzneistoffe erfolgte abhängig von deren Konzentration und dem Mischungsverhältnis von Iloprost und Hirudin. Iloprost beeinflußt die Freisetzungsrate von PEG-Hirudin bzw. r-Hirudin. Es konnte mit mikrobiologischen Metho...

  16. Nutzung der Eisenkorrosion zur Entfernung von Arsen aus Trinkwasser

    OpenAIRE

    Karschunke, Karsten

    2005-01-01

    In dieser Arbeit werden experimentelle Untersuchungen zur Nutzung von Eisenkorrosionsprozessen zur Entfernung von Arsen aus Trinkwasser vorgestellt. Auch in geringen Konzentrationen stellt diese Verunreinigung auf Grund der krebserregenden Wirkung von Arsen langfristig eine Gefahr für die menschliche Gesundheit dar. Verschiedene Entfernungsmethoden sind bekannt und in der Praxis der Trinkwasseraufbereitung bewährt, beispielsweise Flockungsverfahren unter Einsatz von Eisensalzen oder die Adsor...

  17. Liouville-von Neumann molecular dynamics.

    Science.gov (United States)

    Jakowski, Jacek; Morokuma, Keiji

    2009-06-14

    We present a novel first principles molecular dynamics scheme, called Liouville-von Neumann molecular dynamics, based on Liouville-von Neumann equation for density matrices propagation and Magnus expansion of the time-evolution operator. The scheme combines formally accurate quantum propagation of electrons represented via density matrices and a classical propagation of nuclei. The method requires a few iterations per each time step where the Fock operator is formed and von Neumann equation is integrated. The algorithm (a) is free of constraint and fictitious parameters, (b) avoids diagonalization of the Fock operator, and (c) can be used in the case of fractional occupation as in metallic systems. The algorithm is very stable, and has a very good conservation of energy even in cases when a good quality conventional Born-Oppenheimer molecular dynamics trajectories is difficult to obtain. Test simulations include initial phase of fullerene formation from gaseous C(2) and retinal system.

  18. Gasification of liquid manure; Vergasung von Guelle

    Energy Technology Data Exchange (ETDEWEB)

    Gudenau, H.W.; Hoberg, H.; Hirsch, U. [Technische Hochschule Aachen (Germany). Inst. fuer Eisenhuettenkunde

    1996-12-31

    The potential of thermal use of liquid manure is investigated. It is recommended to separate the liquid and solid fraction. While the liquid fraction can be used as fertilizer, the solid fraction can be used for generating a fuel gas for combined heat and power generation. (orig) [Deutsch] Die Untersuchungen haben ergeben, dass in der thermischen Verwertung von Guelle ein bisher nur wenig genutztes Potential vorliegt. Die zum Anbau von Getreide notwendige Duengung kann durch Separation von Guelle in Fest- und Duennfraktion effektiver und kostenguenstiger durchgefuehrt werden. Der Naehrstoffgehalt des Guellefeststoffs sollte durch thermische Aufbereitung aufkonzentriert werden, so dass eine Vermarktung auch mit laengeren Transpoertwegen darstellbar ist. Die bei der thermischen Behandlung gewonnene Waerme kann prozessintern genutzt und ueberschuessige Waerme an Abnehmer in der naeheren Umgebung geliefert werden. Genauso besteht die Moeglichkeit, ein Brenngas zu erzeugen und die Energie mit Kraft-Waerme-Kopplung zu nutzen. (orig)

  19. [Anja Wilhelmi. Lebensweltlen von Frauen der deutschen Oberschicht im Baltikum (1800-1939). Eine Untersuchung anhand von Autobiographien] / Alexander Ewig

    Index Scriptorium Estoniae

    Ewig, Alexander, 1980-

    2011-01-01

    Arvustus: Wilhelmi, Anja. Lebenswelten von Frauen der deutschen Oberschicht im Baltikum (1800-1939). Eine Untersuchung anhand von Autobiographien. (Veröffentlichungen des Nordost-Instituts, 10.) (Wiesbaden : HArrassowitz 2008)

  20. Wachstum und Aufbau von Spongomorpha aeruginosa (Chlorophyta, Acrosiphoniales)

    NARCIS (Netherlands)

    Kornmann, Peter

    1967-01-01

    In zahlreichen Arbeiten dienten Algen als Objekte für das Studium von Regenerationsvorgängen und Erscheinungen der Polarität. Dagegen ist — von Chara abgesehen — über das normale Wachstum, den zeitlichen Ablauf von Teilung und Streckung der Zellen und die Entstehungsgeschichte des typischen Thallus

  1. Modellierung von Dienstleistungen mittels Business Service Blueprinting Modeling

    OpenAIRE

    Meis, Jochen; Menschner, Philipp; Leimeister, Jan Marco

    2010-01-01

    Der Beitrag stellt mit Business Service Blueprint Modeling (BSBM) einen konzeptionellen Ansatz zur Modellierung von Dienstleistungen vor, der ein systematisches und standardisiertes Vorgehen zur Modellierung von Dienstleistungen mit Kundeninteraktion anstrebt. BSBM verwendet hierzu die Business Process Modeling Notation (BPMN) in einem Service Blueprint (SB). Somit wird ein Modellierungskonzept geschaffen, das sowohl Kundeninteraktionspunkte berücksichtigt als auch von aktuellen Werkzeugen un...

  2. Spectral theory and quotients in Von Neumann algebras | West ...

    African Journals Online (AJOL)

    In this note we consider to what extent the functional calculus and the spectral theory in von Neumann algebras are preserved by the taking of quotients relative to two-sided ideals of the von Neumann algebra. Keywords:von Neumann algebra, functional calculus, spectral theory, quotient algebras. Quaestiones ...

  3. Market Timing und Finanzierungsentscheidungen: Unterscheidet sich Europa von den USA?

    NARCIS (Netherlands)

    Sautner, Z.; Spranger, J..

    2009-01-01

    Seit der Arbeit von Baker/Wurgler (2002) hat sich die Market-Timing-Theorie als alternative Kapitalstrukturtheorie etabliert. Sie impliziert, dass die Kapitalstruktur von Unternehmen das kumulierte Ergebnis von Versuchen des Equity Market Timing ist. Im Rahmen dieser Studie wird untersucht,

  4. Bewertung von Fahrzeuggeräuschen

    Science.gov (United States)

    Genuit, Klaus; Schulte-Fortkamp, Brigitte; Fiebig, André; Haverkamp, Michael

    Bei der Wahrnehmung und Beurteilung eines Automobils sind unzählige Merkmale und Eigenschaften von Bedeutung. Dabei können Merkmale objektiv-technisch beschrieben werden, wie Angaben zur Motorisierung, Höchstgeschwindigkeit, Drehmoment, zulässige Zuladung, Verbrauch usw. Daneben sind weitere Eigenschaften von Bedeutung, die sich einer einfachen objektiv-technischen Beschreibung entziehen. Hier sind Begriffe zu nennen, wie Sicherheit, allgemeine Qualitätsanmutung, Design, Ergonomie, Komfort, Haptik, Fahrdynamik, Zuverlässigkeit, die deutlich schwieriger objektiv erfassbar und beschreibbar sind (Abb. 4.1).

  5. Zur Aktivierung des Verbundtragverhaltens von Glaslaminaten

    OpenAIRE

    Sastré-Terrasa, Johannes (Hanno)

    2007-01-01

    Für jedes konstruktiv planerische Handeln ist ein fundiertes Wissen über die Baustoffe primäre Voraussetzung. Das trifft für Glas, auf Grund der Komplexität des Materialverhaltens, im besonderen Maße zu. Diese Arbeit beschäftigt sich deshalb zunächst mit dem Werkstoff Glas, den Grundlagen zum schadensfreien Fügen von Glas sowie dem Verhalten seiner Fügepartner. Die wichtigste Methode, das Kleben von Glas, wird im Wesentlichen durch die viskoelastischen und temperaturabhängig...

  6. Erwachsenenbildung im Spannungsfeld von Wissenschaft, Politik und Praxis: Heide von Felden, Christiane Hof und Sabine Schmidt-Lauff (Hrsg.). [Rezension

    OpenAIRE

    Filla, Wilhelm

    2016-01-01

    Rezension von: Felden, Heide von/Hof, Christiane/Schmidt-Lauff, Sabine (Hrsg.) (2013): Erwachsenenbildung im Spannungsfeld von Wissenschaft, Politik und Praxis. Dokumentation der Jahrestagung der Sektion Erwachsenenbildung der Deutschen Gesellschaft für Erziehungswissenschaften vom 27. bis 29. September 2012. Baltmannsweiler: Schneider Verlag Hohengehren.

  7. Die Verteilung und Eigenschaften von Bodenformen in der Deutschen Bucht, eine Rekonstruktion der Karten von Ulrich (1973)

    DEFF Research Database (Denmark)

    Winter, Christian; Lefebvre, Alice; Benninghoff, Markus

    2015-01-01

    Entstehung, Gestalt und Dynamik von Bodenformen in vergleichsweise kleinen Untersuchungsgebieten ist die Arbeit von ULRICH (1973) über die Verteilung von Bodenformen in der Deutschen Bucht bis heute die einzige verfügbare zusammenhängende Darstellung für die deutsche Nordseeküste. Die analogen Karten und die...

  8. Der Briefwechsel zwischen Alexander von Humboldt und Karl Ernst von Baer

    Directory of Open Access Journals (Sweden)

    Thomas Schmuck

    2012-10-01

    Full Text Available Zusammenfassung Der kurze, nicht vollständig erhaltene Briefwechsel zwischen Alexander von Humboldt und Karl Ernst von Baer behandelt sehr verschiedene Themen: Politik, Kriegsgefahr, den Wissenschaftsbetrieb, Auszeichnungen und Tagungen, Embryologie und Cholera. Gemeinsam mit Briefen und Reden ergibt sich dabei ein differenziertes Bild der Beurteilung der beiden Wissenschaftler durch den jeweils anderen. Während Baer sich als Bewunderer Humboldts erweist, erscheint umgekehrt Humboldts Einschätzung Baers als ambivalent. Abstract The short, not completely preserved correspondence between Alexander von Humboldt and Karl Ernst von Baer deals with a wide range of subjects: politics and the danger of war, academic activities, scientific awards and conferences, aspects of embryology and the cholera. Letters to third persons and speeches, together with the correspondence between Humboldt and Baer show a differentiated pattern of mutual appraisal: While Baer always remained an admirer of the elder naturalist, Humboldt’s estimation was characterized by ambivalence.

  9. Genetics Home Reference: glycogen storage disease type I

    Science.gov (United States)

    ... storage disease type I glycogen storage disease type I Printable PDF Open All Close All Enable Javascript ... expand/collapse boxes. Description Glycogen storage disease type I (also known as GSDI or von Gierke disease) ...

  10. Interpenetration bei Parsons und Luhmann : Von der Integration zur Produktion von Unordnung

    OpenAIRE

    Künzler, Jan

    2010-01-01

    Talcott Parsons hatte den Interpenetrationsbegriff eingeführt, um strukturelle Beziehungen zwischen verschiedenen System arten erfassen zu können. Niklas Luhmann übernimmt den Begriff von Parsons und benutzt ihn nach weitreichenden Revisionen v. a. dazu, das Verhältnis von psychischen und sozialen Systemen auf den Begriff zu bringen. In dieser Intersystembeziehung spielt Sprache eine entscheidende Rolle. Ein systemtheoretisches Sprachkonzept, das dieser Vermittlungsleistung Rechnung trägt, st...

  11. Dentinhaftung von Zementen. Der Haftverbund von Zementen mit Dentin in Kombination mit verschiedenen indirekten Restaurationsmaterialien

    OpenAIRE

    Peutzfeldt, Anne; Sahafi, Alireza; Flury, Simon

    2011-01-01

    Einleitung: Die Anzahl zahnärztlicher Zemente sowie Restaurationsmaterialien steigt stetig. Die richtige Zementwahl für einen zuverlässigen Haftverbund zwischen Restaurationsmaterial und Zahnsubstanz ist von Interesse für den Kliniker. Ziel der vorliegenden in vitro-Studie war es daher, den Dentinhaftverbund von verschiedenen Zementen in Kombination mit verschiedenen indirekten Restaurationsmaterialien zu untersuchen. Material und Methoden: Zylindrische Probekörper aus sechs Restaurations...

  12. Identifizierung von Mikrodomän-Proteinen und funktionelle Charakterisierung von Cholesterin im Golgi-Apparat

    OpenAIRE

    Stüven, Ernstpeter

    2002-01-01

    Mittels Detergenzextraktion von Golgimembranen bei 0°C mit anschließender Dichtegradientenzentrifugation wurden sphingolipid- und cholesterinreiche Mikrodomänen isoliert, die zehn Proteine entsprechend der Coomassiefärbung in der SDS-PAGE enthalten. Im Rahmen dieser Arbeit wurden die zwei bisher nicht charakterisierten Proteine mit Molekulargewichten von 72 und 56 kDa als Untereinheiten A und B der vakuolären ATPase identifiziert. Daneben konnte mit NAP-22 eine elfte Komponente identifiziert ...

  13. Der "Bindungsfragebogen" von Grau und der "Bielefelder Fragebogen zu Partnerschaftserwartungen" von Hoger und Buschkamper im Vergleich

    National Research Council Canada - National Science Library

    Ina Grau; Ulrich Clashausen; Diether Hoger

    2003-01-01

    ...". Das Bindungsmuster ergibt sich aus der speziellen anhand von Clusteranalysen ermittelten Konfiguration der Skalen. In Clusteranalysen mit dem BinFB wurden vier Cluster aufgefunden, mit dem BFPE funf Cluster. Wahrend vier der BFPE-Cluster recht gut mit den vier BinFB-Clustern ubereinstimmen, wird eine funfte Personengruppe vom BFPE als "vermeidend-offnungsbereit" und von BinFB als "sicher" klassifiziert. Beide Interprctationsmoglichkeiten werden unter besonderer Berucksichtigung der Skala "Zuwendungsbedurfn...

  14. Authentizität von Milch und Fisch – Erkennung von Bioprodukten im Labor

    OpenAIRE

    Molkentin, Joachim

    2008-01-01

    Die Nachfrage nach Bio-Lebensmitteln ist in Deutschland in den letzten Jahren stetig gestiegen. So erhöhte sich der Absatz von Bio-Trinkmilch in 2007 im Vergleich zum Vorjahr erneut um 34 %. Aufgrund sporadisch resultierender Lieferengpässe bei Bio-Milch aber auch der erheblichen Handelspreisdifferenz – wie z. B. insbesondere bei Bio-Lachs – besteht ein potenzielles Risiko der Falschdeklaration konventioneller Produkte als Bio-Ware. Zum Schutz von Verbrauchern wie auch Erzeugern werden dah...

  15. Die Rezeptionsgeschichte von Ulrich Schmidels Wahrhaftige Beschreibung von 1567 bis heute

    OpenAIRE

    Obermeier, Franz

    2001-01-01

    Schmidels Werk hat bisher nur in Ansätzen die ihm zustehende Würdigung als eines der wichtigsten Quellenwerke der frühen Kolonialzeit Südamerikas erfahren. Es stand lange Zeit im Schatten des bekannteren und auch literarisch ansprechenderen Reisebuchs von Hans Staden (Wahrhaftige Historia, Marburg 1557), dessen persönliche Schilderung seiner Gefangenschaft dem modernen Leser durch den geschickten Einsatz von narrativen Elementen entsprechend der heutigen Genretradition des persönlich gefasste...

  16. Analyse von Arbeitsplätzen auf halbautomatischen Pflanzmaschinen mit Hilfe von MTM (Methods Time Measurement)

    OpenAIRE

    Remmele, Edgar

    2017-01-01

    Mit Hilfe von MTM (Methods Time Measurement), einem System vorbestimmter Zeiten, werden Bewegungsstudien an drei verschiedenen halbautomatischen Pflanzmaschinen durchgeführt. Hauptunterscheidungsmerkmal der untersuchten Pflanzmaschinen ist die Methode der Übergabe der Pflanze von der Pflanzperson an die Pflanzmaschine. Analysiert wird der Arbeitsplatz der Pflanzmaschine ACCORD-Standard mit der Methode Klemmscheibe, PES-Beetpflanzer 1500/1900 mit der Methode Pflanzfinger und Lännen RT-2 mit de...

  17. von Willebrand factor, Jedi knight of the bloodstream.

    Science.gov (United States)

    Springer, Timothy A

    2014-08-28

    When blood vessels are cut, the forces in the bloodstream increase and change character. The dark side of these forces causes hemorrhage and death. However, von Willebrand factor (VWF), with help from our circulatory system and platelets, harnesses the same forces to form a hemostatic plug. Force and VWF function are so closely intertwined that, like members of the Jedi Order in the movie Star Wars who learn to use "the Force" to do good, VWF may be considered the Jedi knight of the bloodstream. The long length of VWF enables responsiveness to flow. The shape of VWF is predicted to alter from irregularly coiled to extended thread-like in the transition from shear to elongational flow at sites of hemostasis and thrombosis. Elongational force propagated through the length of VWF in its thread-like shape exposes its monomers for multimeric binding to platelets and subendothelium and likely also increases affinity of the A1 domain for platelets. Specialized domains concatenate and compact VWF during biosynthesis. A2 domain unfolding by hydrodynamic force enables postsecretion regulation of VWF length. Mutations in VWF in von Willebrand disease contribute to and are illuminated by VWF biology. I attempt to integrate classic studies on the physiology of hemostatic plug formation into modern molecular understanding, and point out what remains to be learned. © 2014 by The American Society of Hematology.

  18. Clinical measurement of von Willebrand factor by fluorescence correlation spectroscopy.

    Science.gov (United States)

    Torres, Richard; Genzen, Jonathan R; Levene, Michael J

    2012-06-01

    Identification of von Willebrand factor (vWF) abnormalities in a variety of conditions is hampered by the limitations of currently available diagnostic tests. Although direct multimer visualization by immunoelectrophoresis is a commonly used method, it is impractical as a routine clinical test. In this study, we used a biophysical analysis tool, fluorescence correlation spectroscopy (FCS), to measure vWF distributions. The goals were to develop a method that is quicker and simpler than vWF gel electrophoresis and to evaluate the potential of FCS as a clinical diagnostic technique. We analyzed plasma from 12 patients with type 1 von Willebrand disease (vWD), 14 patients with type 2 vWD, and 10 healthy controls using a fluctuation-based immunoassay approach. FCS enabled identification and proper classification of type 1 and type 2 vWD, producing quantitative results that correspond to qualitative gel multimer patterns. FCS required minimal sample preparation and only a 5-min analysis time. This study represents the first implementation of FCS for clinical diagnostics directly on human plasma. The technique shows potential for further vWF studies and as a generally applicable laboratory test method.

  19. [Richard von Volkmann, one career of orthopaedic surgeon and poet].

    Science.gov (United States)

    Bumbasirević, M; Lesić, A; Sudjić, V; Zagorac, S

    2010-01-01

    Richard von Volkman was one of the most famous and important surgeons in the 19th century. He pioneered antiseptic procedures and was especially known for his achivements in orthopedic surgery. Von Volkmann was born in Leipzig, Germany and attended medical schools in Giessen, Halle, and Berlin. Starting in 1867, he worked as a professor of surgery at the University of Halle, also leading its surgical clinic. He was active as a surgeon during Seven Weeks' War with Austria in 1866 and the Franco-Prussian war 1870/1871, in the latter as consulting Generalarzt. He was important in the introduction of antiseptic wound treatment in Germany, and through it to the United States of America. Two observations in orthopaedic surgery bear his name to these days: Volkmans contracture and Heuter-Volkmans low. Volkmann also wrote poetry under the name Richard Leander and his book entitled "Dreams by French Firesides" which still has a place in literature. He died of paralysis due to a chronic spinal disease, following a prolonged illness, in the Binswanger institution in Jena in 1889, at the top of his careere.

  20. Zur Entwicklung von Containerschiffsflotten : eine Paneldatenanalyse

    OpenAIRE

    Prinz, Alexander; Schulze, Peter M.

    2004-01-01

    Diese Arbeit untersucht die regional aggregierten Containerschiffsflotten der wichtigsten Wirtschaftsräume entlang der Hauptverkehrsrouten der Seeschifffahrt. Anhand einer Paneldatenanalyse mit festen Effekten werden die positive Abhängigkeit der Containerschifffahrt von Weltwirtschaft und Welthandel quantifiziert und erklärende Variablen für die Entwicklung der Containerschifffahrt identifiziert.

  1. Von Kalifornien nach Taxham / Katri Soe

    Index Scriptorium Estoniae

    Soe, Katri, 1971-

    2005-01-01

    Neljas osa magistritööst "Von Kalifornien nach Taxham: Formen und Strukturen der Kommunikation in Peter Handkes Romanen "Der kurze Brief zum langen Abschied" und "In einer dunklen Nacht ging ich aus meinem stillen Haus"" (Tartu, 2000. Juhendaja: Claus Sommerhage)

  2. Euler-Chelpin, Prof. Hans von

    Indian Academy of Sciences (India)

    Euler-Chelpin, Prof. Hans von. Nobel Laureate (Chemistry) - 1929. Date of birth: 15 February 1873. Date of death: 6 November 1964 ... Posted on 21 December 2017. ASTROPHYSICS: An Observational View of the Universe. Math Art and Design: MAD about Math, Math Education and Outreach. Math and Finance ...

  3. Ueberreste vorweltlicher Proboscidier von Java und Banka

    NARCIS (Netherlands)

    Martin, K.

    1884-01-01

    Junghuhn führte in seinem Werke über Java nur einen einzigen Wirbelthierrest, Carcharias megalodon, an (7, IV, pag. 97); es war ihm nicht gelungen bei seinem ersten Aufenthalte auf der Insel Reste von Säugethieren zu finden, so eifrig er auch darnach in den Höhlen des Tertiaergebirges suchte (7, IV,

  4. Alte und neue Scyphomedusen von Australien

    NARCIS (Netherlands)

    Stiasny, G.

    1926-01-01

    In einer Collection Coelenterata, von verschiedenen australischen Fundorten herrührend, die mir vom Direktor des Australian Museum in Sydney, Dr. Ch. Anderson, zur Untersuchung übersendet wurden, fanden sich auch einige Scyphomedusen, die in den australischen Gewässern bisher noch nicht nachgewiesen

  5. Eigenschaften von Gott. Eine quantitative Analyse

    Directory of Open Access Journals (Sweden)

    Thomas Benesch

    2016-12-01

    Full Text Available ENGLISH: A quantitative research on 32 pupils of an elementary school (third and fourth class was performed. The questions regarded to socio-demographic issues as well as so called opposition pairs for God. These opposition pairs include the semantic differential of the loving God combined with the selection of 27 opposition pairs which were derived from literature. The image of God has been developed due to a factor analysis with respect to human and divine properties. These characteristics are depending on the religious education of children as well as their habits of reading in the Holy Bible. DEUTSCH: Eine quantitative Erhebung von 32 Volksschulkindern einer 3. und 4. Klasse wurde einerseits mit erweiterten sozio-demographischen Fragen und gleichzeitig mit Gegensatzpaaren für Gott beschrieben. Die Gegensatzpaare beinhalteten das semantische Differential des liebenden Gottes zusammen mit einer Auswahl von 27 Gegensatzpaaren, die deduktiv aus der Literatur abgeleitet wurden. Das Gottesbild hat sich anhand einer Faktorenanalyse darin entwickelt, dass Gott einerseits mit menschlichen Attributen versehen wird und andererseits mit Eigenschaften von Gott. Diese Eigenschaften von Gott sind abhängig davon, ob die Kinder religiös erzogen werden und ob sie gerne die Heilige Schrift lesen.

  6. Active Von Willebrand Factor, thrombocytopenia and thrombosis

    NARCIS (Netherlands)

    Hulstein, J.J.J.

    2006-01-01

    Platelets and von Willebrand factor (VWF) are unable to interact in circulation. To induce an interaction, a conversion of VWF to a platelet-binding conformation is required. At higher shear stresses, the first step in thrombus formation is binding of VWF to the subendothelium. This results in

  7. Molecular mechanisms of von Willebrand Factor mechanoregulation

    NARCIS (Netherlands)

    Jakobi, A.J.|info:eu-repo/dai/nl/311489621

    2012-01-01

    Von Willebrand factor (VWF) multimers mediate primary adhesion and aggregation of platelets. The potency to recruit platelets critically depends on the size of VWF multimers, which is regulated by a feedback mechanism involving shear-induced unfolding of the A2 domain in VWF and cleavage by the

  8. Measurements and von Neumann projection/collapse

    Indian Academy of Sciences (India)

    unwanted superpositions of (system + apparatus)-states can be shown to be suppressed, leading eventually to the projection/collapse rule postulated in von Neumann's treatment of measurements [3]. In the next section, the measurement problem in quantum mechanics (QM) is recalled. In §3, some proposed improvements ...

  9. Guido von Pirquet: Austrian pioneer of astronautics

    Science.gov (United States)

    Sykora, F.

    1977-01-01

    The works of Guido von Pirquet, Austrian pioneer of rocketry, were assessed. Major emphasis was given to Pirquet's calculation of the route to Venus which in fact was followed by the first Russian rocket to Venus. Of interest also is Pirquet's valuable construction of a space station and his analysis of interstellar space flight.

  10. Soolopartiid Von Krahli katuse all / Kristi Eberhart

    Index Scriptorium Estoniae

    Eberhart, Kristi

    2007-01-01

    Von Krahli Teatri kolmest lavastusest: "Erki ja Tiina" (lavastaja Mart Kangro, tantsivad Erki Laur ja Tiina Tauraite), "Faust" (J. W. Goethe ainetel tekst ja lavastus Taavi Eelmaa, muusika ja laulud Chalice, osades Jarek Kasar ja Rein Pakk), "Hamletid" (William Shakespeare'i ainetel kontseptsiooni, lavastuse, koreograafia, kujunduse, valguse autor Sasha Pepeljajev, video- ja helikunstnik Taavet Jansen. Esitaja Juhan Ulfsak)

  11. Ueber eine Anomalie von Acromitus flagellatus (Stiasny)

    NARCIS (Netherlands)

    Stiasny, G.

    1934-01-01

    Bei erneuter Durchsicht des reichhaltigen Materiales von Acromitus flagellatus (Stiasny) in der Scyphomedusen-Sammlung des Rijksmuseum van Natuurlijke Historie in Leiden (vergl. meine Mitteilungen daruber, 1920, Uebersichtstabelle III und 1921, p. 131/136) fand ich ein Exemplar, das eine

  12. Laue, Prof. Max Theodor Felix von

    Indian Academy of Sciences (India)

    Home; Fellowship. Fellow Profile. Elected: 1954 Honorary. Laue, Prof. Max Theodor Felix von. Nobel Laureate (Physics) - 1914. Date of birth: 9 October 1897. Date of death: 24 April 1960. YouTube; Twitter; Facebook; Blog. Academy News. IAS Logo. Theory Of Evolution. Posted on 23 January 2018. Joint Statement by the ...

  13. Ueber zwei neue Vogelarten von Java

    NARCIS (Netherlands)

    Finsch, O.

    1902-01-01

    Die beiden nachstehend beschriebenen Vögel wurden mir von Herrn Max Bartels als muthmasslich neu zugesandt. Die genaue Untersuchung hat dies bestätigt. Ich freue mich daher eine der Arten zu Ehren des Entdeckers benennen zu können. Seit einigen Jahren als Leiter der Plantage Pangerango bei Pasir

  14. Das Druckprofil von Sportkompressionsstrümpfen.

    Science.gov (United States)

    Reich-Schupke, Stefanie; Surhoff, Stefan; Stücker, Markus

    2016-05-01

    Zunehmend werden Sportkompressionsstrümpfe (SKS) eingesetzt, doch fehlt eine Norm in Analogie zu medizinischen Kompressionsstrümpfen (MKS). Ziel dieser Pilotstudie war der Vergleich der Druckprofile von fünf SKS ex vivo und in vivo miteinander sowie mit MKS-Normen. CEP Running-Progressive-Socks, Falke Running--Energizing, Sigvaris Performance, X-Socks Speed-Metal-Energizer und 2XU Compression--Race-Socks wurden an zehn Leistungssportlern (standardisiert in vivo, Kikuhime--Sensor) sowie im Hohenstein Institut (ex vivo) hinsichtlich ihres Druckprofils getestet. Die Ex-vivo-Messungen im Fesselbereich von CEP (25,6 mmHg) und 2XU (23,2 mmHg) entsprachen der deutschen MKS-Klasse 2, von Sigvaris-SKS (20,8 mmHg) der deutschen MKS-Klasse 1. Die übrigen SKS lagen darunter. Die ermittelten Druckprofile differierten stark und entsprachen nicht der MKS-Norm. Die In-vivo-Messungen zeigten für drei SKS (2XU, CEP, Sigvaris) durchschnittlich einen höheren Anpressdruck als die SKS von Falke und X-Socks. Alle SKS lagen in vivo jedoch unter der deutschen MKS-Klasse 1. Kein SKS bot den für MKS geforderten Druckabfall vom Messpunkt B nach D. Die in vivo und ex vivo ermittelten SKS-Druckprofile zeigten heterogene Ergebnisse und folgten kaum den MKS-Anforderungen. Entsprechend sind auch die klinisch-praktischen Effekte von SKS nicht vergleichbar. Wünschenswert wäre eine SKS-Klassifikation, die erlaubt, Produkte einzuordnen, zu vergleichen und nach konkreten Vorlieben und Bedürfnissen (hoher vs. geringer Druck, progressiver vs. degressiver Gradient) auszuwählen. © 2016 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.

  15. Disease: H00152 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available to increased intestinal absorption and reduced biliary secretion of neutral sterols. Inherited metabolic disea...dhop T, von Bergmann K ... TITLE ... Sitosterolemia--a rare disease. Are elevated plant sterols an additional

  16. Integrales Lernen in und von Organisationen

    Directory of Open Access Journals (Sweden)

    Wendelin Kupers

    2006-06-01

    Full Text Available Bezogen auf das integrale Models von Ken Wilber untersucht der Beitrag die Bedeutung des Lernens in und von Organisationen. Nach einer Darstellung der Relevanz und des Grundverständnisses des Lernens im Organisationskontext, werden integrale Dimensionen des Lernens dargestellt. Im Einzelnen werden die verschiedenen Sphären eines inneren-subjektiven und äusseren-„objektiven“ Lernens des Einzelnen als auch ein gemeinschaftliches Lernen und Lernen im System auf der kollektiven Ebene dargestellt sowie deren interrelationaler Zusammenhang diskutiert. Schließlich beschreibt der Beitrag noch integrale Lernprozesse sowie integrale Gestaltungsfelder zur Förderung des Lernens in den verschiedenen Bereichen. Abschließend spricht der Artikel noch Schwierigkeiten und Probleme an sowie nimmt im Fazit ein perspektivischen Ausblick vor.

  17. Wir zeigen andere Bilder von Frauen ...

    Directory of Open Access Journals (Sweden)

    Bettina Rulofs

    2010-03-01

    Full Text Available Der vorliegende Beitrag beleuchtet die Bedeutung der Geschlechterordnung im Prozess der sportmedialen Kommunikation. Im Kern geht es um die Frage, inwiefern im Prozess der medialen Vermittlung von Sport traditionelle Geschlechterstereotype aufrechterhalten werden oder Möglichkeiten der Irritation solcher Stereotype bestehen. Dazu werden verschiedene Ebenen des massenmedialen Kommunikationsprozesses in den Blick genommen: die Medienprodukte, die Öffentlichkeitsarbeit und Selbst-Präsentation der Sportler/innen, die Medienrezeption und die Herstellungsprozesse von Medien in den Sportredaktionen. This article illustrates the relevance of gender in processes of sports media communication. The question in focus is in what way traditional gender stereotypes are perpetuated in the process of media communication in sport and how such stereotypes can be irritated. Therefore different levels of mass media communication are considered: the media products, the public relations of athletes and the presentation of themselves as athletes, the media-reception and the production processes in sport departments of media institutions.

  18. Evolution equations of von Karman type

    CERN Document Server

    Cherrier, Pascal

    2015-01-01

    In these notes we consider two kinds of nonlinear evolution problems of von Karman type on Euclidean spaces of arbitrary even dimension. Each of these problems consists of a system that results from the coupling of two highly nonlinear partial differential equations, one hyperbolic or parabolic and the other elliptic. These systems take their name from a formal analogy with the von Karman equations in the theory of elasticity in two dimensional space. We establish local (respectively global) results for strong (resp., weak) solutions of these problems and corresponding well-posedness results in the Hadamard sense. Results are found by obtaining regularity estimates on solutions which are limits of a suitable Galerkin approximation scheme. The book is intended as a pedagogical introduction to a number of meaningful application of classical methods in nonlinear Partial Differential Equations of Evolution. The material is self-contained and most proofs are given in full detail. The interested reader will gain a ...

  19. La Medea de Lars von Trier

    Directory of Open Access Journals (Sweden)

    Iratxe Fresneda Delgado

    2013-04-01

    Full Text Available El presente artículo analiza el modo en el que Lars von Trier recrea para el cine el estereotipo de Medea. Mediante el análisis fílmico de la película y apoyándose en los estudios culturales, el texto se interroga acerca de la importancia y el poder potencial del cine a la hora recuperar el antiguo mito y demostrar su vigencia. El análisis amplía horizontes para la compresión de los mecanismos que articulan el entramado de significados de la película, donde Von Trier aporta una nueva visión del arquetipo de Medea uniéndola, a la tradición pictórica del Romanticismo. Una influencia que habita en las posteriores obras del director danés, donde el paisaje, la naturaleza, se erige en elemento catalizador de las pulsiones humanas, en su cómplice y testigo.This paper explores the way that Lars von Trier’s film recreates the stereotype of Medea. Using film analysis and based on cultural studies the article asks about the importance and potential power of cinema to recover the ancient myth and show their effects. The analysis expands horizons for the understanding of the mechanisms that link the network of meanings of the film, where the author offers a new vision of Medea's archetype attaching it to the pictorial tradition tied to the Romanticism. An influence that can be seen in the later works of Lars von Trier, where the landscape, the nature, stands as a catalyst of human drives, as his accomplice and witness.

  20. Von Medien, Übertragungen und Automaten

    Directory of Open Access Journals (Sweden)

    Alessandro Barberi

    2013-12-01

    Full Text Available Im Zuge der Debatten zum Medialen Habitus wurde vielfach betont, dass die >Theorie der Praxispraxeologischen Medientheorie< des Medialen Habitus avant la lettre gesprochen werden kann. Dieser Artikel untersucht – ausgehend von den Debatten zur "Medienkompetenz" – wie Bourdieu Sprache, Sprechen und Diskurs, sowie Akteure, Felder und Habitus als Medien begreift und betont dabei die Nützlichkeit der Bourdieuschen Bildungssoziologie im Rahmen einer sozialwissenschaftlichen Grundlegung der Medienpädagogik.

  1. Untersuchungen von Nanostrukturen magnetischer Kolloide mit Kleinwinkelstreuung

    OpenAIRE

    Kammel, Martin

    2005-01-01

    Mit Hilfe der Neutronenkleinwinkelstreuung mit polarisieren Neutronen wird eine Analyse so genannter Ferrofluide durchgeführt. Ferrofluide sind Dispersionen magnetisierbarer Teilchen, welche durch in den meisten Fällen organische Hüllen gegen irreversibler Aggregation geschützt sind. Anhand von verdünnten Magnetit-Kolloidlösungen wurden als Stabilisierung Dextran, Tenside (Laurinsäure/Marlipal) und elektrostatisch stabilisierte Magnetitteilchen untersucht. Dabei fanden sich in jedem dieser Fl...

  2. Maschinelle Erkennung von Verkehrsteilnehmern mittels heterogener Sensorik

    OpenAIRE

    Walchshäusl, Leonhard

    2009-01-01

    In modernen Fahrzeugen halten verstärkt Fahrerassistenzsysteme zum Insassenschutz und zum Schutz anderer Verkehrsteilnehmer Einzug. Typische, bereits seit längerem verfügbare Systeme, wie Airbag und Antiblockiersystem, erreichen schon heute eine hohe Marktdurchdringung. Künftige aktive Sicherheitssysteme, die von der Kollisionswarnung bis hin zur autonomen Gefahrenbremsung reichen, sind Gegenstand aktueller Forschung. Insbesondere Systeme, die aktiv in das Fahrgeschehen eingreifen, erfordern ...

  3. Mikrobieller Befall von Elektrotauchlack in der Automobilindustrie

    OpenAIRE

    Gühring, Ina Katrin

    2000-01-01

    Nach Umstellung auf umweltverträglichere Lackmaterialien (Reduzierung biozid wirkender Lösemittel, Schwermetalle) traten in Elektrotauchanlagen verschieder Werke der DaimlerChrysler AG vermehrt Beschichtungsstörungen bei Karossen auf. Dabei war ein pH-Anstieg des Lackmaterials, Schichtdickenanstieg, Abblättern des Lacks („Striptease“ Effekt), Oberflächenstörungen (Pusteln, Blasen, Krater), schlechter Umgriff, eine Bildung von Lackschlamm und geringerer Schichtwiderstand zu beobachten. Gleichz...

  4. Die Usability von Rich Internet Applications

    Science.gov (United States)

    Linder, Jörg

    Interaktiven Services, die dem Themenkreis Web 2.0 zugeordnet werden, haftet unter anderem das Attribut an, besonders leicht bedienbar zu sein. Flickr, Youtube und Wikipedia gelten als Erfolgsprojekte dieser neuen Art von interaktiven Websites. Wodurch zeichnet sich nun eine Usability 2.0 (so es sie überhaupt geben sollte) aus? Was ist zu beachten, wenn so genannte Rich Internet Applications gestaltet werden?

  5. Therapie von Vorhofflimmern: Rolle der Katheterablation

    OpenAIRE

    Hindricks G; Kircher S; Gaspar T.; Arya A

    2009-01-01

    Vorhofflimmern ist die häufigste Herzrhythmusstörung in der Gesamtbevölkerung und geht einher mit erhöhter Morbidität und Mortalität. Als prinzipielle Behandlungskonzepte stehen die "Frequenzkontrolle" und die "Rhythmuskontrolle" zur Verfügung. Während sich die medikamentöse Rhythmuskontrolle als insgesamt unzureichend erwiesen hat, stellt die Katheterablation von Vorhofflimmern einen etablierten, potenziell kurativen Therapieansatz mit beachtlichen Erfolgsraten dar. Obwohl hinsichtlich d...

  6. Modellierung von Preiserwartungen durch neuronale Netze

    OpenAIRE

    Heinemann, Maik; Lange, Carsten

    1997-01-01

    Das Papier untersucht, wie Erwartungsbildung mit Hilfe neuronaler Netze modelliert werden kann. Die Grundlage bildet ein Cobweb-Modell, in dem Firmen Preiserwartungen auf Basis eines Feedforward-Netzes bilden.Zunächst wird anhand von Simulationen gezeigt, daß Firmen durch neuronale Erwartungsbildung approximativ rationale Erwartungen bilden können. Im Gegensatz zur Hypothese rationaler Erwartungen ist dafür die Kenntnis des relevanten Modells nicht mehr erforderlich, lediglich Beobachtungen d...

  7. Expression von Blutgruppenantigenen durch humane mesenchymale Stammzellen

    OpenAIRE

    Schüle, Michael Christian

    2013-01-01

    Die vorliegende experimentelle Arbeit befasst sich mit der Untersuchung der Expression von Blutgruppenantigenen durch humane mesenchymale Stammzellen (MSC) aus dem Knochenmark. Der Fokus lag hierbei auf den klinisch bedeutsamen Antigenen A, B, H, Rhesus-D, -C, -c, -E, -e, RhAG, K, k, Jka, Jkb und DARC. Die Expression wurde systematisch auf Genom-, Transkriptom- und Proteinebene evaluiert. Alle untersuchten MSC Populationen exprimierten FUT1, RHCE, KEL und Kidd (HUT11) mRNA. mRNA der AB0-Gl...

  8. Weitere Vorkommen von Impatiens capensis in Hessen

    OpenAIRE

    Nawrath, Stefan

    1996-01-01

    Neue hessische Fundorte von Impatiens capensis aus dem Taunus und dessen Vorland sowie aus der Rheinebene werden berichtet. Die insbesondere im Schwarzbach-System zahlreichen Fundorte werden beschrieben und in einer Verbreitungskarte dargestellt. New Hessian stations of Impatiens capensis in the Taunus mountains as well as their foothills and in the Rhine valley are reported on. The stations which are particularly numerous in the Schwarzbach system, are described and demonstrated on a dist...

  9. ["Because the mole sometimes shows itself" argumentative structures in Hildegard von Bingen 'causeae et curae'].

    Science.gov (United States)

    Riha, Ortrun

    2011-01-01

    The paper examines the didactic strategies in Hildegard von Bingen's 'Causae et curae'. Based on her self-developed, complicated concept of primary qualities, elements, and body fluids, she connects cosmic processes with physical activities and disease patterns. Hildegard combines an allopathic and a homoeopathic approach and, as an attentive observer of nature, she looks upon everyday situations as signatures of healing. Hildegard's imagination in analogy and comparison constitutes a specific quality of her medical advice.

  10. Gestalt und Funktion von Animal Design : Versuch einer semiotischen Analyse

    OpenAIRE

    Schlüter, Wolfgang

    2009-01-01

    Dissertation zur Gestalt und Funktion von Animal Design (tieranaloges Design). Untersucht wird eine Auswahl an Produkten, die in ihrem Äußeren an Tiere erinnern. Hierbei stehen Aspekte wie die Emotionalisierung von Produkten, demonstrativer Konsum und die soziale Funktion im Fokus der Analyse. Anhand von Abbildungen und Zeichnungen wird u.a. dargestellt, welche Bedeutung die Tiergestalt für das jeweilige Produktdesign hat. Die Objekte werden jeweils unter phänomenologischen, hermeneutischen u...

  11. Bioethik in der Ukraine: Von Medizinischer zur Integrativen Bioethik

    OpenAIRE

    Gubenko, Anna

    2014-01-01

    Im Artikel wird die Chronologie der Entstehung und Entwicklung der Bioethik in der Ukraine analysiert. Dabei werden drei Hauptetappen der Herausbildung von Bioethik als a) einer gesellschaftlichen Organisation, b) einer Lerndisziplin und c) einer integrativen Wissenschaft unterschieden. Der Autor hat den Vektor von medizinischer zur integrativen Bioethik durch Ausbildung und soziale sowie pädagogische Praktiken bezeichnet. In diesem Sinne wird der Begriff von “pädagogischer Bioethik“ einge...

  12. Portrait of Dr. Von Braun with Walt Disney, 1954.

    Science.gov (United States)

    1954-01-01

    Marshall Center Director Dr. Wernher Von Braun is pictured with Walt Disney during a visit to the Marshall Space Flight Center in 1954. In the 1950s, Dr. Von Braun while working in California on the Saturn project, also worked with Disney studios as a technical director in making three films about Space Exploration for television. Disney's tour of Marshall in 1965 was Von Braun's hope for a renewed public interest in the future of the Space Program at NASA.

  13. Risiko und Akzeptanz von Industrieansiedlungen: Eine empirische Studie

    OpenAIRE

    Burgemeister, Jörg; Weber, Martin

    1992-01-01

    In der Bundesrepublik Deutschland stoßen Industrieansiedlungen oft auf den Widerstand der Bevölkerung. Bei der Akzeptanz einer Ansiedlung sind von den Bürgern subjektiv wahrgenommene Risiken und Nutzen der Ansiedlung von erheblicher Bedeutung. In einer Fragebogenstudie haben wir untersucht, wie Personen die Nutzen und Risiken von Industrieansiedlungen einschätzen, inwieweit sie Absiedlungen akzeptieren, welche Bestimmungsfaktoren ihren Risiko- und Akzeptanzurteilen zugrundeliegen, und wie sic...

  14. Erfolgsfaktoren von Internet-Auktionen: Eine empirische Analyse in PLS

    OpenAIRE

    Fritz, Wolfgang; Möllenberg, Antje; Dees, Heiko

    2004-01-01

    Internet-Auktionen haben sich zu einem der erfolgreichsten Geschäftsmodelle im E-Commerce entwickelt. Die stetig steigende Anzahl von Auktionen beim Marktführer eBay zeugen von einer zunehmenden Beliebtheit solcher Auktionen. Doch welche Determinanten beeinflussen den Erfolg einer Internet-Auktion? Im vorliegenden Beitrag wird dieses Thema aus dem Blickwinkel des Verkäufers am Beispiel von Ticket-Auktionen empirisch untersucht. Im Mittelpunkt steht dabei eine Kausalanalyse, die den Einfluss v...

  15. Saage tuttavaks : Elisabeth ja Karl von Hoerschelmann / Mai Levin

    Index Scriptorium Estoniae

    Levin, Mai, 1942-

    2004-01-01

    Kuraator M. Levin kuni 10. X 2004 Adamson-Ericu muuseumis avatud näitusest "Tuntud ja tundmatud Elisabeth von Rosendorff-Hoerschelmann ja Karl von Hoerschelmann". Enamik töid on kunstnike laste Konstantin Hoerschelmanni ja Anna Röder-Hoerschelmanni omand. Elisabeth Rosendorff (1898-1984) sündis Virumaal Maidlas eesti perekonnas, Karl von Hoerschelmann (1899-1951) Sevastoopolis saksa perekonnas

  16. Abbaukinetiken - und mechanismen von Lignin und Vanillin unter hydrothermalen Bedingungen

    OpenAIRE

    Curtze, Jan Henning

    2017-01-01

    Eine Methode zur energetischen Aufkonzentrierung von feuchten Abfallbiomassen ist die Hydrothermale Carbonisierung (HTC). Um technische HTC-Reaktoren rational auslegen zu können, sollte die Formalkinetik der vier Hauptkomponenten von Biomasse - Kohlenhydrate, Lignin, Fette/Öle und Proteine – bekannt sein. Des Weiteren spielt die Änderungskinetik des Feststoffes während der HTC eine wichtige Rolle. Der Fokus dieser Arbeit liegt auf der Untersuchung der HTC von Lignin, was ein hochkomplexes, ch...

  17. Studien zur Kinetik der Fehlfaltung un Aggregation von Proteinen

    OpenAIRE

    Modler, Andreas Johannes

    2003-01-01

    Diese Arbeit befasst sich mit der Kinetik der Fehlfaltung und Aggregation von Proteinen. Anhand dreier Beispiele, der Phosphoglyceratkinase (PGK) aus Hefe, einer Variante von Barstar und des Prion-Proteins des Syrischen Hamsters (SHaPrP(90-232)) wurde insbesondere die Kinetik der Bildung von Amyloidfibrillen und deren kinetischer Vorläuferstrukturen mittels dynamischer und statischer Lichtstreuung, Circulardichroismus, Infrarotspektroskopie, Elektronenmikroskopie und teilweise analytischer Ch...

  18. Giant aneurysm of the vertebral artery in neurofibromatosis type 1: report of a case and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Hoffmann, K.T.; Hosten, N.; Liebig, T.; Felix, R. [Strahlenklinik und Poliklinik, Klinikum Rudolf Virchow, Humboldt-Universitaet zu Berlin, Augustenburger Platz 1, D-13353 Berlin (Germany); Schwarz, K. [Neurochirurgische Klinik, Virchow-Klinikum Humboldt-Universitaet zu Berlin, Berlin (Germany)

    1998-04-01

    Cranial and cervical vessels are rarely involved in neurofibromatosis type 1. Stenoses and occlusion due to intimal or medial dysplasia are most often seen, followed by aneurysms. We report a rare asymptomatic giant aneurysm of the vertebral artery in a 59-year old man with von Recklinghausen`s neurofibromatosis and review the literature. (orig.) With 3 figs., 1 tab., 22 refs.

  19. Dr. von Braun and Army Ballistics Missile Agency (ABMA) Group

    Science.gov (United States)

    1959-01-01

    This photograph of Dr. von Braun, shown here to the left of General Bruce Medaris, was taken in the fall of 1959, immediately prior to Medaris' retirement from the Army. At the time, von Braun and his associates worked for the Army Ballistics Missile Agency in Huntsville, Alabama. Those in the photograph have been identified as Ernst Stuhlinger, Frederick von Saurma, Fritz Mueller, Hermarn Weidner, E.W. Neubert (partially hidden), W.A. Mrazek, Karl Heimburg, Arthur Rudolph, Otto Hoberg, von Braun, Oswald Lange, Medaris, Helmut Hoelzer, Hans Maus, E.D. Geissler, Hans Heuter, and George Constan.

  20. Medical radiation exposure and justification at a large teaching hospital. Comparison of radiation-related and disease-related risks; Medizinische Strahlenexposition und ihre Rechtfertigung an einem Grossklinikum. Vergleich von strahlungs- und krankheitsbedingtem Risiko

    Energy Technology Data Exchange (ETDEWEB)

    Loose, R.W.; Popp, U.; Adamus, R. [Klinikum Nuernberg-Nord (Germany). Inst. fuer Diagnostische und Interventionelle Radiologie; Wucherer, M. [Klinikum Nuernberg (Germany). Inst. fuer Medizinische Physik

    2010-01-15

    The medical X-ray exposure was determined in a 2400-bed hospital. The radiation-related risk was compared with the severity of disease (ICD) to verify the justification for X-ray procedures. A model to estimate radiation and disease-related ''loss of lifetime'' was applied. X-ray exposure from radiography, fluoroscopy and CT was determined for diagnostic and interventional procedures during one hospital stay of 403 patients (0.5 % of all 80 000 patients/year). CTDI and DLP in CT, DAP in fluoroscopy or SED in radiography were used to calculate the effective dose (ED). The disease and radiation-related risk were compared with a simple ''loss of lifetime'' model. The mean age of all patients was 60. Only a subgroup of 170 patients (42 %) with a mean age of 67.6 had one or more X-ray procedures. The average ED of these exposed patients was 5.12 mSv. 14.4 % CT examinations had a dose contribution of 52.5 % followed by 5.3 % radiology and cardiology procedures at 37.2 %. 90 % of ED was applied to only 44 patients (11 %) and 50 % of ED was applied to only 10 patients (2.5 %) with a mean age of 71.2. 4 of these 10 patients had malignant tumors, the other 6 suffered from pulmonary embolism, pneumonia, stentgraft infection, CAD, Crohn's disease and severe hypertension. The ratio of disease vs. radiation-related risk was between 2.1:1 and 1800:1. In this study referral for justified X-ray procedures in a large hospital with high level medical care offers a significant patient benefit. (orig.)

  1. Einfluss von rekombinantem Adiponektin auf die monozytäre Expression von Annexin A6

    OpenAIRE

    Stögbauer, Fabian

    2009-01-01

    In der vorliegenden Arbeit wurden zunächst die CD14 Micro Beads titriert und so die Menge an CD14 Micro Beads bestimmt, die nötig ist, um eine große Anzahl Monozyten mit einem hohen Reinheitsgrad, verbunden mit geringen Kosten, aus Vollblut isolieren zu können. In den folgenden in vitro Untersuchungen wurden dann periphere Monozyten von humanen Spendern isoliert, um die dosis- und zeitabhängige Veränderung der Annexin A6-Proteinexpression unter dem Einfluss von Adiponektin feststellen zu k...

  2. Einfluss von Eugenol und Silikonöl auf die Dentinhaftung von adhäsiven Befestigungsmaterialien

    OpenAIRE

    Bigdali, Puria

    2015-01-01

    Zielsetzung Eugenolhaltige Zemente und Silikone stehen in Verdacht den Verbund von Kompositzementen zu beeinflussen. Ziel dieser Arbeit war es den Einfluss eugenolhaltiger und eugenolfreier Zemente und von Additions- und Kondensationssilikonen auf konventionell adhäsive und selbstadhäsive Kompositzemente zu untersuchen. Material und Methode Aus 200 extrahierten Rinderzähnen wurden 200 Dentinscheiben mit einer Dicke von 1 mm hergestellt, in welche eine definierte Kavität mit einem ...

  3. Automatisierte Generierung von Postleitzahlgebieten aus OpenStreetMap-Daten unter Verwendung von Open Source GIS Software

    OpenAIRE

    Hauck, Christian

    2011-01-01

    Das Projekt OpenStreetMap als freie Wiki-Weltkarte gewinnt als Quelle von Geodaten für unter-schiedlichste Bedürfnisse innerhalb der Geowissenschaften, des Geomarketings und auch im Alltag immer mehr an Bedeutung. Die kostenlosen, von Freiwilligen einer Community gesammelten geo-graphischen Daten, sogenannte nutzergenerierte Daten, dienen heute vielen Anwendern als Daten-grundlage und stehen in der Konkurrenz zu proprietären Geodaten von kommerziellen Anbietern. Neben Straßendaten sind zahlre...

  4. Feasibility study for an epidemiological investigation of the relation between radiation exposure and a cataract disease; Machbarkeitsstudie fuer eine epidemiologische Studie zur Untersuchung des Zusammenhangs von Strahlenbelastung und einer Katarakterkrankung

    Energy Technology Data Exchange (ETDEWEB)

    Hammer, Gael; Scheidemann-Wesp, Ulrike; Wicke, Henryk

    2012-03-15

    The feasibility study for en epidemiological investigation of the relation between radiation exposure and a cataract disease consisted of three working packages. The first package included the definition of the relevant status of science and the development of criteria for evaluation of available studies. The second package concerned the determination of relevant radiation exposed collectives and a preliminary evaluation with respect to the potential adequacy for the study. The third package included a comprehensive study of the assigned appropriate collectives: interventional active physicians and the aviation personnel.

  5. Ööklubi Club von Überlingen = Club von Überlingen

    Index Scriptorium Estoniae

    2008-01-01

    Ööklubi Club von Überlingen (Madara 22A, Tallinn) sisekujundusest. Sisearhitekt: Taavi Aunre (Boom.ee OÜ). Taavi Aunrest, tema tähtsamad tööd. I-II korruse plaan, 11 värv. vaadet, foto T. Aunrest

  6. Von den Liven von Oesel / August Ludwig Schlözer

    Index Scriptorium Estoniae

    Schlözer, August Ludwig

    2008-01-01

    Avaldatud Eberhard Winkleri initsiatiivil A. L. Schlözeri teose põhjal: Gesammelte Nachrichten von den Ueberresten der Liven, in Livland und Kurland. (1770), mis moodustab osa teosest: Schlözer, August Ludwig. M. Johann Joseph Haigold's Beylagen zum Neuveränderten Russland. Zweiter Theil. Riga und Leipzig, 1770

  7. Aino Lepik von Wiren / Aino Lepik von Wiren ; interv. Kadi Alatalu

    Index Scriptorium Estoniae

    Lepik von Wirén, Aino, 1961-

    2007-01-01

    Heinrich Marga eksiilvalitsuse (20.06.1990-7.10.1992) kohtuminister Aino Lepik von Wiren pagulaslapse elust Rootsis, haridusest ja elukutse valikust, tööst eksiilvalitsuses, tööleasumisest Eestisse 1992. aastal, seadusandlikust tegevusest ning aluse panekust välismaalaste- ja kodakondsuspoliitikale, naiste võimalustest poliitilisse tippu tõusmiseks, kodu- ja väliseestlaste vastandamisest

  8. Rezension: Träume von Räumen von Georges Perec

    Directory of Open Access Journals (Sweden)

    Thomas Ballhausen

    2015-12-01

    Full Text Available Mit "Träume von Räumen" liegt ein zentraler Text aus dem umfangreichen Œuvre Geroges Perecs vor, der zuletzt 1990 als Übersetzung greifbar war. Der ursprünglich 1974 erschienene Band ist in mehrfacher Hinsicht wesentlich für das Verständnis Perecs und seiner literarisch-philosophische Wunderwelt ...

  9. Heritability of plasma von Willebrand factor antigen concentration in German Wirehaired pointers.

    Science.gov (United States)

    Brooks, M B; Castillo-Juarez, H; Oltenacu, P

    2001-07-01

    We applied quantitative genetic analyses to a population of German Wirehaired pointer dogs affected with type 2 von Willebrand disease. Plasma von Willebrand factor (vWF) protein concentration measured as vWF antigen (vWF:Ag), clinical history, and pedigree data were compiled for 331 dogs over a 5-year test period. Eight dogs had histories of abnormal bleeding and had markedly decreased plasma vWF:Ag concentrations (dogs were inbred, with an average inbreeding of 2.52%. The estimated heritability of plasma vWF concentration was 0.52. We found a major gene effect on vWF concentration. Using a single gene locus model and two different prediction methods, the upper threshold value for the aa genotype was less than 1% vWF:Ag, and the optimal threshold value for discrimination between the AA and Aa genotypes was between 68% and 72% vWF:Ag. Our analyses indicate that phenotype, assigned on the basis of a single vWF:Ag determination, is heritable and can be applied for selective breeding in a von Willebrand disease test programme.

  10. Wege des Exports von Glutathion aus Astrocyten

    OpenAIRE

    Minich, Tobias Florian

    2008-01-01

    Vorliegender Arbeit lag die Aufgabe zugrunde herauszufinden, welche(s) Transportsystem(e) außer dem Multidrug-Transporter 1 (multidrug resistance protein 1, MRP1) zum Austritt des Tripeptides Glutathion aus Primärkulturen von Astrogliazellen in das extrazelluläre Milieu beitragen. Als Grundlage für die Untersuchungen wurde zunächst an zwei Wildtyp Mausstämmen (NMRI und FVB/N) sichergestellt, dass astrogliareiche Primärkulturen aus Mäusen sowohl reduziertes als auch oxidiertes Glutathion expor...

  11. Die Behandlung von Intersexuellen in der Diskussion

    Directory of Open Access Journals (Sweden)

    Heinz-Jürgen Voß

    2008-11-01

    Full Text Available Wissenschaftler/-innen aus unterschiedlichen Fachdisziplinen und mit unterschiedlichen Ansichten zum Umgang mit intersexuellen Menschen kommen zu Wort – in diesem Sinne ist der Sammelband sehr heterogen. Es werden Erfahrungen mit Geschlechtsanpassung dargestellt, Begrifflichkeiten analysiert und Probleme bei der Therapie und in der Forschung erörtert. Vorangestellt, aber erfreulicherweise nicht abgetrennt, äußern sich Betroffene. Bereits wegen der sich im Abschluss des Bandes befindlichen Handlungsempfehlungen zur Behandlung von Säuglingen und Kindern mit ‚abweichenden‘ bzw. ‚uneindeutigen‘ Geschlechtsmerkmalen, die sich insbesondere an Eltern, Mediziner/-innen und Sozialpädagog/-innen wenden, ist das Buch unbedingt empfehlenswert.

  12. Electronic Resource Management System. Vernetzung von Lizenzinformationen

    OpenAIRE

    Michaela Selbach; Daniel Armin Rupp

    2014-01-01

    In den letzten zehn Jahren spielen elektronische Ressourcen im Bereich der Erwerbung eine zunehmend wichtige Rolle: Eindeutig lässt sich hier ein Wandel in den Bibliotheken (fort) vom reinen Printbestand zu immer größeren E-Only-Beständen feststellen. Die stetig wachsende Menge an E-Ressourcen und deren Heterogenität stellt Bibliotheken vor die Herausforderung, die E-Ressourcen effizient zu verwalten. Nicht nur Bibliotheken, sondern auch verhandlungsführende Institutionen von Konsortial- und ...

  13. Authentifizierung von Bio-Milch im Labor

    OpenAIRE

    Molkentin, Joachim

    2008-01-01

    In Deutschland ist die Nachfrage nach Bio-Lebensmitteln in den letzten Jahren stetig gestiegen. So erhöhte sich der Absatz von Bio-Trinkmilch in 2007 im Vergleich zum Vorjahr erneut kräftig um 34 Prozent (ZMP, Bonn) und der Bio-Anteil beträgt inzwischen bei Frischmilch knapp elf Prozent. Aufgrund sporadisch resultierender Lieferengpässe bei Bio-Milch sowie der vorhandenen Handelspreisdifferenz besteht zunehmend ein potenzielles Risiko der Falschdeklaration konventionell erzeugter Milch als Bi...

  14. Der Denkmalwert von Illegalität

    OpenAIRE

    Beyer, Dennis

    2012-01-01

    Kunst oder Schmiererei - Graffiti spalten die Gesellschaft. Für die einen sind sie illegale Schmierereien und Ausdruck von Vandalismus, für die anderen gelten sie als Kunst am Bau einer verkannten Avantgarde. Das Spektrum an Motivationen und Produkten innerhalb der Sprayer-Gemeinschaft ist tatsächlich derart vielfältig, dass eine sachliche Debatte ohne Polarisation kaum möglich scheint. Dennoch stellt sich die Frage, welche Bedeutung diese flüchtigen und illegalen Zeugnisse einer Minderheit i...

  15. Illustrierte Bestimmungshilfe zur Unterscheidung von Candelaria concolor und Candelaria pacifica

    OpenAIRE

    Stapper, Norbert J.

    2012-01-01

    Unterscheidungsmerkmale von Candelaria concolor und C. pacifica werden anhand von Makro- und Mikrofotos dargestellt. Im Gelände kann C. pacifica anhand der fehlenden Unterrinde erkannt werden. Characteristic features separating Candelaria concolor from C. pacifica are presented on macro and micro photographs. The lack of a lower cortex can be used to identify C. pacifica in the field.

  16. On two matrix derivatives by Kollo and von Rosen

    OpenAIRE

    Neudecker, Heinz

    2003-01-01

    The article establishes relationships between the matrix derivatives of Fwith respect to Xas introduced by von Rosen (1988), Kollo and von Rosen (2000) and the Magnus-Neudecker (1999) matrix derivative. The usual transformations apply and the Moore-Penrose inverse of the duplication matrix is used. Both Xand F have the same dimension. Peer Reviewed

  17. A New Generalization of von Neumann Relative Entropy

    Science.gov (United States)

    Li, Jing; Cao, Huaixin

    2017-11-01

    In quantum information, von Neumann relative entropy has a great applications and operational interpretations in diverse fields, and von Neumann entropy is an important tool for describing the uncertainty of a quantum state. In this paper, we generalize the classical von Neumann relative entropy S( ρ|| σ) and von Neumann entropy S( ρ) to f-von Neumann relative entropy \\widetilde {S}f(ρ ||σ ) and f-von Neumann entropy \\widetilde {S}f(ρ ) induced by a logarithm-like function f, respectively, and explore their properties. We prove that \\widetilde {S}f(ρ ||σ ) is nonnegative and then prove that \\widetilde {S}f(ρ ) has nonnegativity, boundedness, concavity, subadditivity and so on. Later, we show the stability and continuity of the \\widetilde {S}f(ρ ) with respect to the trace distance. In the case that f( x) = -log x, the resulted entropies reduce the classical von Neumann relative entropy and von Neumann entropy, respectively. This means that our results extend the usual results to a more general setting and then have some potential applications in quantum information.

  18. An accurate von Neumann's law for three-dimensional foams

    NARCIS (Netherlands)

    Hilgenfeldt, Sascha; Kraynik, Andrew M.; Koehler, Stephan A.; Stone, Howard A.

    2001-01-01

    The diffusive coarsening of 2D soap froths is governed by von Neumann's law. A statistical version of this law for dry 3D foams has long been conjectured. A new derivation, based on a theorem by Minkowski, yields an explicit analytical von Neumann's law in 3D which is in very good agreement with

  19. Minimum Moduli in Von Neumann Algebras | Gopalraj | Quaestiones ...

    African Journals Online (AJOL)

    Minimum Moduli in Von Neumann Algebras. Perumal Gopalraj, Anton Ströh. Abstract. In this paper we answer a question raised in [12] in the affirmative, namely that the essential minimum modulus of an element in a von. Neumann algebra, relative to any norm closed two-sided ideal, is equal to the minimum modulus of the ...

  20. Spin–momenta entanglement in moving frames: Properties of von ...

    Indian Academy of Sciences (India)

    well as locality, of information [9–11]. Expressed differently, the von Neumann entropy measures the stored qubits of information per states of the combined system while the reduced one quantifies the qubits per states that may be stored in one of the subsystems. [1]. It is then obvious that a deeper understanding of von ...

  1. Victor or Villain? Wernher von Braun and the Space Race

    Science.gov (United States)

    O'Brien, Jason L.; Sears, Christine E.

    2011-01-01

    Set during the Cold War and space race, this historical role-play focuses on Wernher von Braun's involvement in and culpability for the use of slave laborers to produce V-2 rockets for Nazi Germany. Students will grapple with two central questions. Should von Braun have been allowed to emigrate to the United States given his affiliation with the…

  2. Von Krahli teatris etenduvad Tõnu Kõrvitsa kammerooperid / Esme Kassak

    Index Scriptorium Estoniae

    Kassak, Esme

    2006-01-01

    20. apr. esietenduvad Von Krahli Teatris Peeter Jalaka lavastuses Tõnu Kõrvitsa uued kammerooperid "Tuleaed" ja "Mu luiged, mu mõtted", mille aluseks on luuletaja Marie Heibergi saatus. Libretode autor on Maarja Kangro. Kammerooperid tulevad lavale Von Krahli Teatri ja Nargen Opera koostöös. Esitavad Kädy Plaas, Helen Lokuta, Nargen Opera koor ja Tallinna Kammerorkester, dirigent Tõnu Kaljuste

  3. Early diagnosis of Legg-Calve-Perthes disease: Value of bone scintigraphy and magnetic resonance imaging in comparison to radiography. Fruehdiagnostik des Morbus Perthes: Wertigkeit von Skelettszintigraphie und Kernspintomographie im Vergleich zu Roentgenbefunden

    Energy Technology Data Exchange (ETDEWEB)

    Theissen, P.; Linden, A.; Smolarz, K.; Voth, E.; Schicha, H. (Koeln Univ. (Germany). Klinik und Poliklinik fuer Nuklearmedizin); Ruett, J. (Koeln Univ. (Germany). Klinik und Poliklinik fuer Orthopaedie)

    1991-12-01

    The value of conventional radiology, bone scintigraphy and magnetic resonance imaging (MRI) in the early diagnosis of Legg-Calve-Perthes disease (LCPD) was assessed. The initial results were compared with the clinical and radiological findings of long-term follow-up in 43 children. Radiological and scintigraphic examination resulted in a relatively high number of equivocal findings (16% and 10%, respectively). MRI findings were less equivocal (3%). Depending on whether such findings were classified as normal or as pathological, the diagnostic accuracy ranged as follows: Radiography 88-93%, bone scintigraphy 88-91%, and MRI 97-99%. Therefore, MRI by itself seems to be sufficient to detect or exclude LCPD. The results of this study and the radiation exposure associated with radiography and scintigraphy raise the question whether MRI should be the diagnostic method of choice in patients with suspected LCPD. (orig.).

  4. [Heinz von zur Mühlen. Das Bürgertum Fordert Blut. Ein Bolschewistiches Flugblatt von 1918] / Paul Kaegbein

    Index Scriptorium Estoniae

    Kaegbein, Paul

    2007-01-01

    Arvustus: Heinz von zur Mühlen. Das Bürgertum Fordert Blut. Ein Bolschewistiches Flugblatt von 1918. In : Ostseeprovinzen, baltische Staaten und das Nationale. Münster : LIT, 2005, lk. 403-414. 1918. aastast pärit lendlehest, mille käekirja järgi otsustades on kirjutanud Viktor Kingissepp

  5. Kontextualisierung von Queer Theory Contextualizing Queer Theory

    Directory of Open Access Journals (Sweden)

    Anna Voigt

    2008-03-01

    Full Text Available Christine M. Klapeer legt in diesem Einführungsband dar, aus welchen politischen und theoretischen Kontexten heraus sich ‚queer‘ zu einem Begriff mit besonderem politischem und theoretischem Gehalt entwickelt hat. Wesentlich zielt sie dabei auf eine kritische Kontextualisierung von „queer theory”. Die Autorin geht zunächst auf das Gay Liberation Movement ein, grenzt die Queer Theory vom Poststrukturalismus, von feministischen Theorien und den Lesbian and Gay Studies ab, beleuchtet Eckpunkte queeren Denkens und zeichnet schließlich die Entwicklungen in Österreich sowohl politisch-rechtlich als auch bewegungsgeschichtlich und in der Wissenschaftslandschaft nach.Christine M. Klapeer’s introductory volume demonstrates the manner in which ‘queer’ grew out of various political and theoretical contexts to become a term with special political and theoretical content. She focuses primarily on a critical contextualization of “queer theory.” The author begins by approaching the Gay Liberation Movement and then distinguishes Queer Theory from poststructuralism, from feminist theories, and from Lesbian and Gay Studies. She continues on to illuminate the key aspects of queer thought and concludes by sketching the development in Austria in terms of politics and the law, the history of movements, and within the landscape of knowledge.

  6. Guy von Dardel 1919-2009

    CERN Multimedia

    2009-01-01

    Guy von Dardel, a well-known figure at CERN and in the international particle physics community, passed away on 28 August. Guy von Dardel came to CERN when it was founded in 1954 and was a full-time staff member until 1964, performing several experiments and working on technical developments. These included the first measurement of the neutral pion’s life-time. Called to Lund University in 1964, he became professor there in 1965 and director of the 1.2 GeV electron accelerator. In the late 1960s, he performed an experiment at CERN’s PS that measured the decays of the Λ. Then, in the early 1970s, he involved the Lund group in a series of experiments at the Intersecting Storage Rings (ISR), where he measured the production of various types of particles. In particular, he participated in a series of experiments that observed the production of a high abundance of particles with large transverse momenta. This required an explanation...

  7. Suitability of tracers; Eignung von Tracern

    Energy Technology Data Exchange (ETDEWEB)

    Klotz, D. [GSF - Forschungszentrum fuer Umwelt und Gesundheit GmbH, Neuherberg (Germany). Inst. fuer Hydrologie

    1999-02-01

    Hydrological tracer techniques are a means of making statements on the direction and speed of underground water. One of the simpler tasks is to find out whether there is hydrological communication between two given points. This requires a determination of the direction of flow, which places less exacting demands on the properties of the tracer than does the task of determining the flow velocity of underground water. Tracer methods can serve to infer from flow velocity the distance (flow) velocity, which is defined as the ratio between the distance between two points located in flow direction and the actual time it takes water to flow from one to the other. [Deutsch] Mit Hilfe der hydrologischen Markierungstechniken koennen Aussagen ueber die Richtung und die Geschwindigkeit von Bewegungen des unterirdischen Wassers gemacht werden. Der einfachere Fall liegt vor, wenn festgestellt werden soll, ob zwischen zwei Punkten eine hydrologische Verbindung besteht. Bei dieser Fliessrichtungsbestimmung sind die Forderungen an die Eigenschaften der einzusetzenden Tracer geringer als bei der Bestimmung der Geschwindigkeit des unterirdischen Wassers. Von den Geschwindigkeiten des unterirdischen Wassers ist die Abstands-(Fliess)geschwindigkeit, die definiert ist durch das Verhaeltnis aus dem Abstand und der wahren Fliesszeit zwischen zwei in Bewegungsrichtung gelegenen Punkten, durch Tracermethoden zu bestimmen. (orig.)

  8. Die Krakauer Kirche von Karmeliten und Karmelitaninnen

    Directory of Open Access Journals (Sweden)

    Józef Szymon Wronski

    2004-12-01

    Full Text Available 1. Die Klosterkirche der unbeschuhten Karmelitinnen Zum Heiligen Josef. Ein glücklicher Auftakt und somit die charakteristische Einführung in das 20. Jh. für den Kirchenbau von Krakau war die Errichtung der Karmeliterinnen St. Josephs-Kirche an der Lobzowska-Straße 40, in der die Architekten (Tadeusz Stryjeński (1849-1943 - Projektant der Kirche und Franciszek Mączyński (1874-1947 - Ausführer des Bauprojektes mit eigenen Verbesserungen die historisch- eklektizistischen Formen d.h. die mittelalterlichen Formen vor allem des sog. Übergangsstils vereinfachten. Die Kirche, die in den Jahren 1903-1905 errichtet wurde, steht inmitten vom Baukomplex des Klosters, dessen Grundriss das Quadrat ist49. Der Kirchenbau ist ein dem Gesamtbau des Klosters eingefügter Längsraum. Hinter dem Chor liegt das Oratorium des Konvents. Das Material Backstein (mit Verwendung von Stein erinnert an die Baukunst eines der besten Architekten jener Zeit, nämlich Teodor Talowskis (1857-1910, der viele Kirchen in Südpolen, aber auch viele Bürgerhäuser nicht nur in Krakau im ausgehenden 19. Jh. baute. Die Architektur der Karmeliterinnen St. Josephs-Kirche ist immer noch im Malerischen begriffen. Aber die Vereinfachung der historischen Formen führt zu ihrer „Beruhigung” und dadurch zur Wuchtigkeit des Ausdrucks. Bemerkenswert ist die in dieser Zeit (in der Anlage noch seltene ausgewogene Proportionierung des Baukörpers. Das methodische Prinzip jener Zeit ist der Kontrast. Im Kontrast zu den gedrungenen und wuchtigen Formen des Baukörpers stehen z.B. die sich verjüngenden obeliskenförmigen und pylonenartigen Türmchen, die nicht nur die Eingangsfassade der Kirche flankieren, sondern auch an den Ecken des Querhauses der Kirche stehen. Die ingravierten weißen Steine an der Fassade in der Zahl von Zehn erinnern an das Gebot des Karmelterinnenordens: Bete täglich den Rosenkranz und sind zugleich ein Vanitativmotiv. 2. Die Klosterkirche der unbeschuhten

  9. Von Neumann's quantization of general relativity

    Science.gov (United States)

    Arbuzov, A. B.; Cherny, A. Yu.; Cirilo-Lombardo, D. J.; Nazmitdinov, R. G.; Han, Nguyen Suan; Pavlov, A. E.; Pervushin, V. N.; Zakharov, A. F.

    2017-05-01

    Von Neumann's procedure is applied to quantizing general relativity. Initial data for dynamical variables in the Planck epoch, where the Hubble parameter value coincided with the Planck mass are quantized. These initial data are defined in terms of the Fock orthogonal simplex in the tangent Minkowski spacetime and the Dirac conformal interval. The Einstein cosmological principle is used to average the logarithm of the determinant of the spatial metric over the spatial volume of the visible Universe. The splitting of general coordinate transformations into diffeomorphisms and transformations of the initial data is introduced. In accordance with von Neumann's procedure, the vacuum state is treated is a quantum ensemble that is degenerate in quantum numbers of nonvacuum states. The distribution of the vacuum state leads to the Casimir effect in gravidynamics in just the same way as in electrodynamics. The generating functional for perturbation theory in gravidynamics is found by solving the quantum energy constraint. The applicability range of gravidynamics is discussed along with the possibility of employing this theory to interpret modern observational data.

  10. Entwicklung von Nickel-Trägerkatalysatoren für die Methanisierung von Kohlenstoffdioxid unter Anwendung von Parallelpräparation und statistischer Versuchsplanung

    OpenAIRE

    Thomys, Oliver Dieter

    2017-01-01

    Die Methanisierung von Kohlenstoffdioxid (Sabatier-Reaktion) gilt als aussichtsreiche Technologie für die chemische Speicherung überschüssigen elektrischen Stroms aus erneuerbaren Energien. Diese Arbeit befasst sich mit der Präparation von Nickel-Trägerkatalysatoren mit hoher Niedrigtemperatur-Aktivität und Langzeitstabilität. Durch Parallelsynthese-Konzepte und Methoden der statistischen Versuchsplanung konnten entsprechende Katalysatoren mit niedriger Metallbeladung präpariert werden, deren...

  11. Techniques for quantification of liver fat in risk stratification of diabetics; Techniken zur Leberfettquantifizierung bei der Risikostratifikation von Diabetikern

    Energy Technology Data Exchange (ETDEWEB)

    Kuehn, J.P.; Spoerl, M.C.; Mahlke, C.; Hegenscheid, K. [Universitaetsmedizin Greifswald, Abteilung Experimentelle Radiologie, Institut fuer Diagnostische Radiologie und Neuroradiologie, Greifswald (Germany)

    2015-04-01

    Fatty liver disease plays an important role in the development of type 2 diabetes. Accurate techniques for detection and quantification of liver fat are essential for clinical diagnostics. Chemical shift-encoded magnetic resonance imaging (MRI) is a simple approach to quantify liver fat content. Liver fat quantification using chemical shift-encoded MRI is influenced by several bias factors, such as T2* decay, T1 recovery and the multispectral complexity of fat. The confounder corrected proton density fat fraction is a simple approach to quantify liver fat with comparable results independent of the software and hardware used. The proton density fat fraction is an accurate biomarker for assessment of liver fat. An accurate and reproducible quantification of liver fat using chemical shift-encoded MRI requires a calculation of the proton density fat fraction. (orig.) [German] Die Fettleber scheint einen unmittelbaren Einfluss auf die Pathophysiologie des Diabetes mellitus Typ 2 zu besitzen. Zur Detektion und Quantifizierung des Leberfetts werden in der klinischen Diagnostik akkurate Verfahren gebraucht. Ein einfaches Verfahren ist die Chemical-shift-kodierte Magnetresonanztomographie (MRT). Eine suffiziente Quantifizierung von Leberfett mithilfe der Chemical-shift-kodierten MRT erfordert eine Beruecksichtigung von Stoervariablen, wie den T2*-Zerfall, den T1-Wiederaufbau und die multispektrale Komplexitaet von Fett. Eine Korrektur aller Stoervariablen wird als Proton-density-Fettfraktion bezeichnet. Diese liefert unabhaengig von der verwendeten Einstellung und Hardware reproduzierbare Ergebnisse. Die korrigierte Proton-density-Fettfraktion ist ein akkurater Biomarker zur Quantifizierung von Leberfett. Die akkurate und reproduzierbare Quantifizierung von Leberfett in der MRT erfordert eine Berechnung der Proton-density-Fettfraktion. (orig.)

  12. Alexander von Humboldt in Daniel Kehlmanns Welt

    Directory of Open Access Journals (Sweden)

    Ottmar Ette

    2012-12-01

    Full Text Available Zusammenfassung Wie stark sich im Verlauf des zurückliegenden Vierteljahrhunderts der Bekanntheitsgrad Alexander von Humboldts in der deutschsprachigen Öffentlichkeit verändert hat, zeigen nicht nur Fernsehumfragen zu den berühmtesten Deutschen, in denen Alexander von Humboldt mittlerweile figuriert, oder Fernsehserien, die über aktuelle Expeditionen berichten und auf Humboldts Namen zurückgreifen. Am deutlichsten vielleicht belegt dies der enorme Erfolg von Daniel Kehlmanns Roman Die Vermessung der Welt, der ohne die zuvor skizzierte Entwicklung nicht denkbar gewesen wäre. Es ist vor diesem Hintergrund nicht nur reizvoll, sondern aufschlußreich, sich mit dem großen Erfolg dieses kleinen Romans zu beschäftigen. Worum geht es in Die Vermessung der Welt? Und wie läßt sich das „Phänomen Kehlmann“ aus etwas größerer Distanz erklären? Abstract In the last 25 years, Alexander von Humboldt‘s popularity has radically changed in the german-speaking public opinion. Proof of this are not only television surveys about the most famous germans - in which Alexander von Humboldt now regularly figures - or television series about contemporary expeditions, which constantly refer to Humboldt‘s name; perhaps what most clearly verifies this change is the great success of Daniel Kehlmann‘s novel Die Vermessung der Welt. Without the recent developments outlined above, this novel‘s degree of impact would have been unimaginable. To study the great success of this text against this backdrop is not only attractive, but also revealing. What is Die Vermessung der Welt really about? And how can we explain the „Kehlmann phenomenon“ from a greater distance? Resumen La popularidad de Alejandro de Humboldt ha cambiado profundamente dentro del último cuarto de siglo en la opinión pública de habla alemana. Prueba de esto son no sólo las encuestas televisivas sobre los alemanes más famosos, dentro de las cuales figura en estos momentos Alejandro

  13. Deep-seated segmental neurofibromatosis without café au lait spots.

    Science.gov (United States)

    Ogose, A; Hotta, T; Imaizumi, S; Saito, H; Homma, T; Takahashi, H E

    2000-09-01

    Segmental neurofibromatosis is a rare disease characterized by neurofibromas with or without café au lait spots localized to one segment of the body. The majority of reported cases have had cutaneous neurofibromas, and patients with deep involvement have rarely been described. We report on two patients with deep-seated segmental plexiform neurofibromatosis and review the literature. All reviewed cases including the present two had no café au lait spots, axillary freckling, Lisch nodules, family history or malignant progression of disease. Differential diagnoses from neuro-fibromatosis 1 (von Recklinghausen disease) and malignant peripheral nerve sheath tumor are important for genetic counseling and avoiding overtreatment.

  14. Radiofrequency ablation of liver metastases; Radiofrequenzablation von Lebermetastasen

    Energy Technology Data Exchange (ETDEWEB)

    Pereira, P.L.; Clasen, S.; Schmidt, D.; Wiskirchen, J.; Tepe, G.; Claussen, C.D. [Abt. fuer Radiologische Diagnostik, Eberhard-Karls-Univ. Tuebingen (Germany); Boss, A. [Abt. fuer Radiologische Diagnostik, Eberhard-Karls-Univ. Tuebingen (Germany); Sektion fuer Experimentelle Radiologie der Abt. fuer Radiologische Diagnostik, Eberhard-Karls-Univ. Tuebingen (Germany); Gouttefangeas, C. [Abt. Immunologie des Inst. fuer Zellbiologie, Eberhard-Karls-Univ. Tuebingen (Germany); Burkart, C. [Zentrum fuer gastroenterologische Onkologie der Medizinischen Klinik, Eberhard-Karls-Univ. Tuebingen (Germany)

    2004-04-01

    The liver is the second only to lymph nodes as the most common site of metastatic disease irrespective of the primary tumor. Up to 50% of all patients with malignant diseases will develop liver metastases with a significant morbidity and mortality. Although the surgical resection leads to an improvement of the survival time, only approximately 20% of the patients are eligible for surgical intervention. Radiofrequency (RF) ablation represents one of the most important alternatives as well as complementary methods for the therapy of liver metastases. RF ablation can lead in a selected patient group to a palliation or to an increased life expectancy. RF ablation appears either safer (vs. cryotherapy) or easier (vs. laser) or more effective (percutaneous ethanol instillation [PEI]), transarterial chemoembolisation [TACE] in comparison with other minimal invasive procedures. RF ablation can be performed percutaneously, laparoscopically or intraoperatively and may be combined with chemotherapy as well as with surgical resection. Permanent technical improvements of RF systems, a better understanding of the underlying electrophysiological principles and an interdisciplinary approach will lead to a prognosis improvement in patients with liver metastases. (orig.) [German] Die Leber ist unabhaengig vom Primaertumor nach den Lymphknoten die zweithaeufigste Lokalisation von Metastasen. Bis zu 50% aller Patienten mit malignen Erkrankungen werden im Verlauf ihrer Erkrankung Lebermetastasen entwickeln, die mit einer signifikanten Morbiditaet und Mortalitaet verbunden sind. Obwohl die chirurgische Resektion zu einer verlaengerten Ueberlebenszeit fuehrt, sind nur ca. 20% der Patienten fuer einen chirurgischen Eingriff geeignet. Die Radiofrequenz-(RF-)Ablation stellt derzeit eine der effektivsten Alternativen und komplementaeren Methoden bei der Therapie von Lebermetastasen dar. In einem selektierten Patientengut fuehrt die RF-Ablation ueber den palliativen Einsatz hinaus zu einer

  15. Die Rolle von RANK-Ligand und Osteoprotegerin bei Osteoporose

    Directory of Open Access Journals (Sweden)

    Hofbauer LC

    2004-01-01

    Full Text Available Receptor activator of nuclear factor (NF- κB ligand (RANKL, sein zellulärer Rezeptor RANK und der Decoy-Rezeptor Osteoprotegerin (OPG stellen ein essentielles Zytokinsystem für die Zellbiologie von Osteoklasten dar. Verschiedene Untersuchungen belegen die Bedeutung von Störungen des OPG/RANKL/RANK-Systems bei der Pathogenese metabolischer Knochenerkrankungen. In dieser Arbeit werden die wichtigsten Störungen des OPG/RANKL/RANK-Systems bei verschiedenen Osteoporoseformen dargestellt. Östrogenrezeptor- (ER- Agonisten wie 17 β-Östradiol, Raloxifen und Genistein stimulieren die osteoblastäre Produktion von OPG durch Aktivierung von ER- α in vitro, während Lymphozyten von Patientinnen mit Östrogenmangel RANKL überexprimieren. Die parenterale Gabe von OPG vermag den mit Östrogenmangel assoziierten Knochenverlust im Tiermodell und in einer kleineren klinischen Studie zu verhindern. Glukokortikoide und Immunsuppressiva steigern gleichzeitig die RANKL-Expression und hemmen die OPG-Produktion in osteoblastären Zellen in vitro. Glukokortikoide sind auch in vivo imstande, die OPG-Serumspiegel deutlich zu reduzieren. Dagegen hemmen biomechanische Reize in vitro die RANKL-Produktion und steigern die OPG-Produktion. Ein Fehlen dieser biomechanischen Reize bei längerer Immobilisierung kann daher den RANKL/OPG-Quotienten steigern, während die tierexperimentelle Immobilisierungs-Osteoporose durch die parenterale Gabe von OPG gemildert werden kann.

  16. Drei Dimensionen von filmischem Archivmaterial. Die Untersuchung von Archivfilmen aus der Zeit des Holocaust

    Directory of Open Access Journals (Sweden)

    Tobias Ebbrecht-Hartmann

    2017-04-01

    Full Text Available Filmisches Archivmaterial aus der Zeit des Holocaust ist eine ambivalente Quelle. Daher sind spezifische Ansätze notwendig, um dieses Material zu ‘lesen’. Es geht darum, den besonderen Inhalt der Bilder zu analysieren, den Kontext ihrer Entstehung zu bestimmen, aber auch, ihre spätere Verwendung und Zirkulation in der visuellen Kultur und damit auch ihren wechselnden Status mit einzubeziehen. Am Beispiel ausgewählter Filmfragmente aus dem Warschauer Ghetto und dem Ghetto Theresienstadt skizziert der Artikel einen Ansatz, wie man diese drei Dimensionen von filmischem Archivmaterial analysieren kann und schlägt damit eine entsprechende Historiographie von Archivfilmen aus der Zeit des Holocaust vor.

  17. Polyphosphate binds to human von Willebrand factor in vivo and modulates its interaction with glycoprotein Ib.

    Science.gov (United States)

    Montilla, M; Hernández-Ruiz, L; García-Cozar, F J; Alvarez-Laderas, I; Rodríguez-Martorell, J; Ruiz, F A

    2012-11-01

    Polyphosphate, a phosphate polymer released by activated platelets, has recently been described as a potent modulator of blood coagulation and fibrinolysis. In blood plasma, polyphosphate binds to and alters the biological functions of factor XII, fibrin(ogen), thrombin and factor VII activating protease. The aim of the present study is to investigate whether polyphosphate also binds to von Willebrand factor (VWF) and alters some of its activities. When studying patients with type 1 von Willebrand disease (VWD) and their healthy relatives, we discovered a significant correlation between von Willebrand factor (VWF) and platelet polyphosphate levels. We have also found polyphosphate in preparations of VWF isolated from normal platelets and plasma. Surface plasmon resonance and electrophoretic mobility assays indicated that polyphosphate interacts with VWF in a dose- and time-dependent manner. Treatment of normal plasma with active exopolyphosphatase decreased the VWF ristocetin cofactor (VWF:RCo) activity, a functional measure of VWF binding to platelet glycoprotein receptor Ib. VWF collagen binding and multimerization were unaltered after polyphosphate depletion. Moreover, addition of polyphosphate increased the deficient VWF:RCo activity presented by plasma from patients with type 1 VWD. Our results reveal that a new role is played by polyphosphate in hemostasis by its interaction with VWF, and suggest that this polymer may be effective in the treatment of some types of VWD. © 2012 International Society on Thrombosis and Haemostasis.

  18. Neurological surgery in the nineteenth century: the principles and techniques of Ernst von Bergmann.

    Science.gov (United States)

    Hanigan, W C; Ragen, W; Ludgera, M

    1992-05-01

    Born in Latvia in 1836, Ernst von Bergmann received his medical education and first academic position at the University of Dorpat in Russia. In 1866, he served as a military surgeon in the Prusso-Austrian War, followed by duty in the Franco-Prussian War of 1870. He was appointed to the faculty of the University of Würzburg in 1878 and 4 years later moved to the University Clinic in Berlin. As a professor and chairman of surgery, he taught until his death in 1907. Von Bergmann practiced general surgery but devoted a large part of his career to the treatment of neurological diseases. Known for his development of aseptic technique, his early military experiences directed his attention to cranial trauma and, ultimately, neurosurgery. In 1880, he authored his first textbook, which described missile ballistics and animal experiments first demonstrating the physiological response later known as "the Cushing reflex" and advocated meticulous intracranial debridement with thorough closure after trauma. Twenty years later, as senior editor of the massive System of Practical Surgery, his contributions included pediatric neurosurgery, successful treatment of abscesses and tumors, diagnostic radiography, and cerebral localization using external landmarks and the neurological examination. Revered by his students and honored by his colleagues, von Bergmann became a proponent for aggressive neurosurgical treatment. His skilled techniques, developed in parallel with accurate experimental physiology, advanced 19th century surgical progression and formed a solid framework for the advances of neurosurgical specialists.

  19. Modellierung von Angebots- und Nachfrageverhalten zur Analyse von Agrarpolitiken: Theorie, Methoden und empirische Anwendungen

    OpenAIRE

    Henningsen, Arne

    2006-01-01

    Die Analyse der Auswirkungen von Agrarpolitiken ist in der Regel sehr komplex und erfordert eine detaillierte Modellierung des Angebots- und Nachfrageverhaltens im Agrarsektor. Eine empirische Anwendung setzt daher eine angemessene ökonomische Theorie sowie fortgeschrittene quantitative Methoden voraus. Alle neun Aufsätze dieser Dissertation lassen sich in diese Themengebiete einordnen. Zunächst werden die Nachfrage nach Bananen und die Folgen der EU Bananenmarktordnung für deutsche Konsument...

  20. Charakterisierung von Mutanten im Lösungsmittelstoffwechsel von Clostridium acetobutylicum

    OpenAIRE

    Hönicke, Daniel

    2015-01-01

    Im Rahmen dieser Arbeit wurden insgesamt neun durch ClosTron-basierte Mutagenese erhaltene Insertionsmutanten von C. acetobutylicum ATCC 824 einer umfangreichen Charakterisierung unterzogen. Für alle im Batch-Fermentationsmaßstab und/oder in kontinuierlicher Kultur fermentierten Stämme erfolgte eine globale Analyse des Transkriptoms unter Verwendung der DNA-Microarray-Technologie. Für eine Analyse des Acetat- und Aceton-Stoffwechsels wurden Stämme mit einer inaktivierten Phosphotransacetyl...

  1. Informationssuchverhalten als Grundlage für die Gestaltung von Veranstaltungen zum Erwerb von Informationskompetenz

    Directory of Open Access Journals (Sweden)

    Martin Wollschläger-Tigges

    2015-09-01

    Full Text Available Für die Gestaltung bibliothekarischer Veranstaltungen zum Erwerb von Informationskompetenz stellen die bisherigen Ergebnisse und Befunde der Forschung zum Informationssuchverhalten eine wertvolle Grundlage dar, indem bestimmte und v.a. typische Verhaltensmuster, Routinen und Präferenzen im Umgang mit Informationsressourcen und Informationen in konzeptionelle und inhaltliche Anpassungen von IK- Veranstaltungen eingebracht und eingesetzt werden. Das Informationssuchverhalten untersucht sowohl individuelle als auch kollektive Informationsprozesse und Faktoren, die diese maßgeblich beeinflussen. Hierzu werden der gesamte Informationsprozess oder einzelne Abschnitte untersucht, die den Information Need, Seeking und Using (INSU-Prozess bilden. Die Befunde der einzelnen INSU-Prozesses-Einheiten zeigen, dass Faktoren wie Informationsbedarf, Informationsart und -form sowie die Einbindung in übergeordnete Arbeitsprozesse wesentlichen Einfluss auf das Informationssuchverhalten haben. Des Weiteren konnte die ISB-Forschung eine Klassifizierung von Benutzergruppen entwickeln und z.B. Fast Surfers, Broad Scanners und Deep Divers charakterisieren bzw. auch das Bouncing oder Flicking Behavior beschreiben. Hinzu kommen mehrere Modelle, die Informationssuchverhalten und Informationsverhalten in bestimmten Situationen nachbilden und darstellen können.

  2. Tragverhalten von Verbunddübeln unter Zugbelastung

    OpenAIRE

    Appl, Jörg-Jochen

    2009-01-01

    Im Bauwesen werden in zunehmendem Maße Dübel zur Einleitung von Lasten in den erhärteten Beton eingesetzt. Vor allem Befestigungssysteme auf der Grundlage chemischer Mörtel werden vermehrt verwendet. Allerdings fehlte bisher ein genügend genaues Berechnungsmodell zur Bemessung von beliebigen Befestigungen mit Verbunddübeln für unterschiedliche Verankerungstiefen und für verschiedene Verbundfestigkeiten. In der vorliegenden Arbeit wurde das Tragverhalten von Verbundübeln unter Zugbelastung...

  3. A von Bertalanffy growth model with a seasonally varying coefficient

    Science.gov (United States)

    Cloern, James E.; Nichols, Frederic H.

    1978-01-01

    The von Bertalanffy model of body growth is inappropriate for organisms whose growth is restricted to a seasonal period because it assumes that growth rate is invariant with time. Incorporation of a time-varying coefficient significantly improves the capability of the von Bertalanffy equation to describe changing body size of both the bivalve mollusc Macoma balthicain San Francisco Bay and the flathead sole, Hippoglossoides elassodon, in Washington state. This simple modification of the von Bertalanffy model should offer improved predictions of body growth for a variety of other aquatic animals.

  4. Abenteuer Alexander-von-Humboldt-Bibliographie

    Directory of Open Access Journals (Sweden)

    Christian Suckow

    2002-04-01

    Full Text Available Article in German; Abstracts in English and German.The two attempts (1860 and 1872 to compile a bibliography of all works of Alexander von Humboldt have to be regarded as failures. Due to Humboldt’s scientific methods, the contemporary popularity of his writings, and the conditions of book publishing and selling, it has been difficult to achieve an overview of his œuvre. Thus each attempt to write a reliable or at least partially complete bibliography of Humboldt’s works constituted an adventure. Horst Fiedler and Ulrike Leitner have mastered this undertaking. As a result of their efforts over more than two decades, a bibliography of those works by Humboldt which were published in book form appeared in the year 2000.

  5. Johann von Lamont: A Pioneer in Geomagnetism

    Science.gov (United States)

    Soffel, Heinrich

    2006-06-01

    The 200th birthday of John Lamont (1805-1879, Figure 1), a pioneer in the study of geomagnetism, was marked on 13 December 2005. Lamont founded the Munich Geomagnetic Observatory in 1840 and was a member of the group of scientists including Carl Friedrich Gauss, Alexander von Humboldt, Eduard Sabine, Jonas Angstrøm, Humphret Lloyd, Adolf Kupffer, Karl Kreil, and Adolphe Quetelet who composed the Göttingen Magnetic Union. They organized an international network of geomagnetic observatories [Barraclough et al., 1992]. The present knowledge of the geomagnetic field and its secular variation is largely based on the data collected by the global network of geomagnetic observatories during the last 170 years. Lamont's talents and his dedication and enthusiasm for discovery are reflected in the depth and scope of his contributions to a broad variety of natural sciences such as astronomy, meteorology, geomagnetism, and geodesy. However, this article just touches on his merits in geomagnetism.

  6. Bertha von Suttner 1843-1914

    OpenAIRE

    Hoock-Demarle, Marie-Claire; Hessel, Stéphane

    2016-01-01

    Née dans « le monde d'hier » cher à son compatriote Stefan Zweig, morte le 21 juin 1914 à la veille du déclenchement d’une guerre qu’elle appelle Weltkrieg, Bertha von Suttner témoigne d’un parcours hors du commun dans un moment crucial où s’amorcent les conflits générés par les militarismes et les nationalismes menant inexorablement à la catastrophe. Arpenteuse d’Europe, voyageuse du Nouveau Monde, elle voit dans « l’américanisation du monde » une raison de construire dans la paix et la coop...

  7. Modernisation of ventilation systems; Modernisierung von Lueftungsanlagen

    Energy Technology Data Exchange (ETDEWEB)

    Richter, W. [Technische Univ. Dresden (Germany). Inst. fuer Thermodynamik und Technische Gebaeudeausruestung

    1997-12-31

    When redeveloping buildings it is common to use window constructions with air-tight rabbets which obviate the inflow of fresh air almost entirely. This leads to the well-known structural-physics and hygienic consequences. As an added effect, the distinctly enhanced thermal insulation results in changes in dynamic heat loss. The paper focuses on the issues of how to safeguard the inflow of fresh air, and the heating capacity of radiators. (MSK) [Deutsch] Da die bei Gebaeudesanierungen eingesetzten fugendichten Fensterkonstruktionen die Nachstroemmoeglichkeiten fuer die Zuluft fast ganz unterbinden, fuehrt das zu den bekannten bauphysikalischen und hygienischen Konsequenzen. Dazu kommen veraenderte dynamische Waermverlustverhaeltnisse aufgrund der deutlich verbesserten Waermedaemmung. Im Folgenden wird schwerpunktmaessig auf die Probleme Zuluftsicherung und Waermeleistung von Heizkoerpern eingegangen.

  8. Hepatische Effekte von Wachstumshormon auf den Glukosestoffwechsel

    Directory of Open Access Journals (Sweden)

    Rufinatscha K

    2015-01-01

    Full Text Available Untherapierter Wachstumshormonmangel im Erwachsenenalter (AGHD ist mit viszeraler Adipositas, Dyslipidämie, Insulinresistenz und Fettleber verbunden. Interessanterweise finden sich viele der AGHD-Merkmale auch bei Patienten mit metabolischem Syndrom. Die nichtalkoholische Fettlebererkrankung (NAFLD gilt als hepatische Manifestation des metabolischen Syndroms. In einigen Studien wurden bei Patienten mit NAFLD verminderte Konzentrationen von zirkulierendem IGF-1, dessen Synthese zu einem großen Teil durch Wachstumshormon (GH reguliert wird, beschrieben. Mäuse, welche eine hepatische Wachstumshormondefizienz aufweisen, zeigen zahlreiche phänotypische Charakteristika des metabolischen Syndroms, unter anderen auch eine Fettlebererkrankung. Dies legt einen Zusammenhang zwischen dem Wachstumshormon und der Entstehung einer NAFLD nahe. Ziel unserer Studie ist es, in einem Zellkulturmodell die Effekte des Wachstumshormons auf den intrazellulären Glukosestoffwechsel näher zu untersuchen. Im Detail sollen Auswirkungen einer verminderten Wachstumshormonrezeptorexpression auf den Insulinsignaltransduktionsweg, den Glykogengehalt und auf Schlüsselenzyme der Glukoneogenese untersucht werden. Präliminäre Daten zeigen, dass eine verminderte Wachstumshormonrezeptorexpression mit intrazellulären Veränderungen des hepatischen Glukosestoffwechsels verbunden ist. Die verminderte Insulinsensitivität könnte auf Alterationen im Insulinsignaltransduktionsweg und Änderungen der Glukoneogenese zurückzuführen sein. Diese präliminären Daten weisen darauf hin, dass Wachstumshormon einen direkten Einfluss auf den Glukosestoffwechsel in der Leber hat. Zudem legen sie nahe, dass Veränderungen im Wachstumshormonstoffwechsel einen wichtigen pathophysiologischen Mechanismus in der Entstehung der Fettlebererkrankung bei Patienten mit metabolischem Syndrom darstellen könnten. Der folgende Artikel soll einen kurzen Überblick über die Effekte von Wachstumshormon

  9. Spontaneous hemorrhage simulating rapid growth of a benign subperiosteal plexiform neurofibroma

    Energy Technology Data Exchange (ETDEWEB)

    Blitman, Netta M. [Albert Einstein College of Medicine, Department of Radiology, Children' s Hospital at Montefiore, Montefiore Medical Center, Bronx, NY (United States); Albert Einstein College of Medicine, Children' s Hospital at Montefiore, Department of Radiology, Bronx, NY (United States); Levsky, Jeffrey M.; Thornhill, Beverly A. [Albert Einstein College of Medicine, Department of Radiology, Children' s Hospital at Montefiore, Montefiore Medical Center, Bronx, NY (United States); Villanueva-Siles, Esperanza [Albert Einstein College of Medicine, Department of Surgical Pathology, Children' s Hospital at Montefiore, Montefiore Medical Center, Bronx, NY (United States)

    2007-09-15

    Spontaneous subperiosteal hemorrhage is a rare complication of von Recklinghausen's disease. There are few reports describing the MR imaging characteristics of this entity. Our case is unique among these as an underlying plexiform neurofibroma was visualized by MR imaging. We present a 12-year-old child with neurofibromatosis 1 who presented with a rapidly enlarging mass of the fibula. Surgery and pathology revealed subperiosteal hemorrhage into a benign, plexiform neurofibroma. The MR imaging features, pathogenesis and clinical implications of this entity are discussed. Recognition of this disease process and differentiating it from malignant transformation can prevent unnecessary surgery. (orig.)

  10. Von Willebrand factor in patients on mechanical circulatory support - a double-edged sword between bleeding and thrombosis.

    Science.gov (United States)

    Hudzik, Bartosz; Kaczmarski, Jacek; Pacholewicz, Jerzy; Zakliczynski, Michal; Gasior, Mariusz; Zembala, Marian

    2015-09-01

    Mechanical circulatory support (MCS) is an umbrella term describing the various technologies used in both short- and long-term management of patients with either end-stage chronic heart failure (HF) or acute HF. Most often, MCS has emerged as a bridge to transplantation, but more recently it is also used as a destination therapy. Mechanical circulatory support includes left ventricular assist device (LVAD) or bi-ventricular assist device (Bi-VAD). Currently, 2- to 3-year survival in carefully selected patients is much better than with medical therapy. However, MCS therapy is hampered by sometimes life-threatening complications including bleeding and device thrombosis. Von Willebrand factor (vWF) has two major functions in haemostasis. First, it plays a crucial role in platelet-subendothelium adhesion and platelet-platelet interactions (aggregation). Second, it is the carrier of factor VIII (FVIII) in plasma. Von Willebrand factor prolongs FVIII half-time by protecting it from proteolytic degradation. It delivers FVIII to the site of vascular injury thus enhancing haemostatic process. On one hand, high plasma levels of vWF have been associated with an increased risk of thrombosis. On the other, defects or deficiencies of vWF underlie the inherited von Willebrand disease or acquired von Willebrand syndrome. Here we review the pathophysiology of thrombosis and bleeding associated with vWF.

  11. Patient with von Willebrand Disease for Gynaecologic Surgery - Perianaesthetic Concerns

    Directory of Open Access Journals (Sweden)

    Rakesh Garg

    2008-01-01

    Patients with vWD do not carry an increased operative risk during elective procedures if appropriate prophylac-tic and corrective therapy is administered. Although the administration of cryoprecipitate and other blood products has traditionally been the cornerstone of treatment for vWD, the recent development of desmopressin(DDAVP for clinical use may provide an effective alternative to replacement therapy with blood products. Further laparaoscopic procedures, taking care during ryle′s tube and foley′s catheter insertion, in such patients are the safer alternative for all kind of gynecologic surgeries.

  12. Surveillance in von Hippel-Lindau disease (vHL)

    DEFF Research Database (Denmark)

    Poulsen, Marie Louise Mølgaard; Budtz-Jørgensen, E; Bisgaard, M L

    2010-01-01

    54 living vHL-mutation carriers, risks of intercurrent manifestations in-between surveillance examinations were determined and clinical consequences of surveillance findings evaluated. Current recommendations of annual ophthalmic and abdominal examinations corresponded to acceptably low intercurrent...... for the patient. Also, pre-symptomatic surveillance increased cumulative incidence of clinical vHL diagnosis from 46% to 72% and from 89% to 94% by age 30 and 50 years, respectively. The present results promote optimization of surveillance, expectantly improving clinical vHL outcomes....

  13. Von Hippel-Lindau disease (vHL)

    DEFF Research Database (Denmark)

    Binderup, Marie Louise Mølgaard; Bisgaard, Søs Marie Luise; Harbud, Vibeke

    2013-01-01

    in the VHL gene. vHL is inherited in an autosomal dominant manner. Predisposed individuals are advised to undergo prophylactic examinations, as they are at lifelong risk of developing multiple cysts and tumours, especially in the cerebellum, the spinal cord, the retina (hemangioblastomas), the kidneys (renal...... are recommended to start in infancy with annual paediatric examinations and ophthalmoscopy until the age of five years. From five to 14 years, annual plasma-metanephrine and plasma-normetanephrine tests, as well as annual hearing examinations are added. Also, an MRI (Magnetic Resonance Imaging) examination....../MRI of the abdomen, e) annual plasma-metanephrine, plasma-normetanephrine, and plasma-chromogranin A tests, and f) annual hearing examination at a department of audiology. It is advised that one doctor takes on the responsibility of coordination of and referral to the many examinations, and the communication...

  14. Von Willebrand's disease: case report and review of literature ...

    African Journals Online (AJOL)

    His birth histories, his familie's social history and developmental milestones were unremarkable. He was born at full term with no antenatal or perinatal complications. Prior to the symptoms, the child was on a normal diet and was thriving appropriately. The child presented one days before his admission trauma to the inner ...

  15. Von Hippel-Lindau disease (vHL)

    DEFF Research Database (Denmark)

    Binderup, Marie Louise Mølgaard; Bisgaard, Søs Marie Luise; Harbud, Vibeke

    2013-01-01

    cell carcinoma), the adrenal glands (pheochromocytoma), the pancreas, as well as in other organs. As many different organs can be affected, several medical specialities often take part in both diagnosis and treatment of manifestations. vHL should be suspected in individuals with a family history...

  16. Von Krahli Teatri lavastus pääses tippfestivalile

    Index Scriptorium Estoniae

    1999-01-01

    Von Krahli Teatri uuendatud lavastus "Eesti mängud. Pulm" esietendub festivalil Theater Der Welt Berliinis. Peeter Jalaka multimeediaetendus Eesti ajaloost ja traditsioonidest osaleb ka suveteatrite festivalil Hamburgis, seejärel antakse kuus etendust Amsterdamis

  17. La actualidad del pensamiento de Carl von Clausewitz

    National Research Council Canada - National Science Library

    Borrero Mansilla, Armando

    2003-01-01

    The article presents the validity of the thought of Carl von Clausewitz, which application, beyond the wars among national States, can be extended to the analysis of the confrontation among political...

  18. Lexikon lesbischer Frauen im Film von Daniela Sobek

    Directory of Open Access Journals (Sweden)

    Karin Windt

    2002-03-01

    Full Text Available Das Lexikon stellt ein neues Basiswerk über lesbische Figuren im Film dar. Neben zahlreichen Artikeln von Anbeginn der Filmgeschichte bis heute gibt es Features über bekannte Schauspielerinnen und lesbische role models.

  19. Magnus Georg von Paucker (1787-1855) / Eckhard Spring

    Index Scriptorium Estoniae

    Spring, Eckhard

    2013-01-01

    Eestis sündinud ja Tartu Ülikoolis õppinud silmapaistvast baltisaksa teadlasest ja tema lapselapsest Alexandrine Pauckerist. 23. novembril 2012 Jelgavas/Mitaus toimunud Magnus Georg von Pauckerile pühendatud teaduskonverentsist

  20. Von Krahli Teater toob Eestisse erakordse auhinna / Tiit Tuumalu

    Index Scriptorium Estoniae

    Tuumalu, Tiit, 1971-

    2004-01-01

    Bessie-auhinna puhul, millega New Yorgis pärjati Von Krahli Teatri lavastust "Luikede järv." Mõjukuselt on seda võrreldud Broadway teatriauhinna Tonyga. Lisatud lühiandmed P. Jalaka ja S. Pepeljajevi kohta

  1. Politische Bildung im Kontext von Regierung, Unterricht und Zucht

    National Research Council Canada - National Science Library

    Thomas Rucker

    2014-01-01

    .... In diesem Beitrag wird zu zeigen versucht, dass Herbart die Unterstützung politischer Bildung als eine Aufgabe von Regierung, Unterricht und Zucht bestimmt und in seinem Werk wichtige „Bausteine...

  2. Die zentrale Wirkung von Candesartan in der Schlaganfalltherapie

    OpenAIRE

    Maydowski, Katja

    2011-01-01

    Ziel der vorliegenden Arbeit war es, die Auswirkungen des AT1-Blockers Candesartan auf einen ischämischen Insult durch eine rein zentrale AT1-Blockade am Tiermodell mit direkter intracerebroventrikulärer Applikation von Candesartan zu untersuchen.

  3. Zur sportlichen Laufbahn von Marathonläufern

    OpenAIRE

    Rümmele, Edgar

    1984-01-01

    Zur sportlichen Laufbahn von Marathonläufern : e. Beitr. zur Psychologie d. Marathonläufers. - Thun u.a. : Deutsch, 1984. - 253, 14 S. - Zugl.: Tübingen, Univ., Diss. - (Beiträge zur Sportwissenschaft ; 1)

  4. Warum das Controlling den systematischen Einsatz von Simulationen vorantreiben sollte

    DEFF Research Database (Denmark)

    Spitzner, Jan; Schneider, Melanie L.

    2017-01-01

    Simulationen werden in der Unternehmenssteuerung heutzutage noch nicht systematisch eingesetzt. Dass sich jedoch die existierenden Hemmnisse durchaus überwinden lassen, legen die Antworten von Studienteilnehmern nahe, die Simulationen häufig einsetzen. Dabei kann Controlling eine Vorreiterrolle e...

  5. Von Krahli Teater viis Helsingisse kaks menulavastust / Andres Laasik

    Index Scriptorium Estoniae

    Laasik, Andres, 1960-2016

    2003-01-01

    Von Krahli Teater esineb 29. nov. Helsingis "Baltic Circle" festivalil lavastustega "Luikede järv" ja "Taksojuhid". Tutvustatakse lühidalt ka paari olulisemat soome lavastust ("Puksiirabi" ja "Hamlet"), mis tulevad festivalil esitamisele

  6. Disease: H00069 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H00069 Glycogen storage diseases (GSD), including: von Gierke disease (GSD type Ia); Pompe disea...se (GSD type II); Cori disease, Forbe disease (GSD type III); Andersen disease (GSD type IV); McArdle disea...se (GSD type V); Hers disease (GSD type VI); Tarui disease (GSD type VII); Phosphorylase kinase ...e deficiency (GSD type 0) Glycogen storage disease (GSD) is an autosomal recessive (all types except IXa and...gen, and type 0 for glycogen synthesis. Pompe disease (type II) is a lysosomal storage disease. Inherited metabolic disea

  7. Zur Dialektik von Soft Skills und fachlicher Kompetenz

    OpenAIRE

    Jendrowiak, Hans-Werner

    2010-01-01

    [Der Autor stellt folgende Thesen zur Dialektik von Soft Skills und fachlicher Bildung auf:] 1. Soft Skills sind normale Bildungskategorien und Teil einer Allgemeinen Bildung. […] 2. Soft Skills sind als personalgebundene Kriterien auch immer schon Gegenstand bildungstheoretischer Debatten. […] 3. Soft Skills ist eine trendorientierte Bezeichnung für Bildung. […] 4. Soft Skills sind Ausdruck von Vorstellungen, Ideen und Theorien (Schulkultur, Unternehmenskultur, Unternehmensphilosophie). 5. S...

  8. Behandlung von Cybermobbing durch die Soziale Arbeit in der Schule

    OpenAIRE

    Dill, Irina; Wahl, Lisa

    2015-01-01

    Gegenstand dieser Arbeit ist die Behandlung von Cybermobbing durch die Soziale Arbeit in der Schule. Cybermobbing ist eine neuere Form von schulischer Gewalt und verlangt nach neuen Formen der Beratung und Intervention der Sozialen Arbeit in der Schule. Anhand einer Literaturrecherche gehen die Autorinnen Lisa Wahl und Irina Dill der Frage nach, inwiefern die Soziale Arbeit in der Schule Cybermobbing behandeln kann. Die Konzipierung der Sozialen Arbeit in der Schule fundiert primär auf dem Bu...

  9. Zusammenhang von Prozess, Mikrostruktur, Eigenspannungen und Eigenschaften bei Aluminiumverbundwerkstoffen

    OpenAIRE

    Kachold, Franziska

    2016-01-01

    Wo die mechanische Leistungsfähigkeit von monolithischen Materialien an ihre Grenzen stößt, bieten Verbundwerkstoffe neue Perspektiven. Besonders wenn hohe Festigkeit oder Steifigkeit bei gleichzeitig niedrigem spezifischen Gewicht gefordert ist, kommen Faserverbundwerkstoffe zum Einsatz. Faserverstärkte Polymere existieren bereits seit ca. 1900 und werden heute für ein breites Spektrum von Bauteilen und Produkten im Automobil-, Flugzeug- und auch Sportgerätebau verwendet. Die geringe T...

  10. Von Neumann's impossibility proof: Mathematics in the service of rhetorics

    Science.gov (United States)

    Dieks, Dennis

    2017-11-01

    According to what has become a standard history of quantum mechanics, in 1932 von Neumann persuaded the physics community that hidden variables are impossible as a matter of principle, after which leading proponents of the Copenhagen interpretation put the situation to good use by arguing that the completeness of quantum mechanics was undeniable. This state of affairs lasted, so the story continues, until Bell in 1966 exposed von Neumann's proof as obviously wrong. The realization that von Neumann's proof was fallacious then rehabilitated hidden variables and made serious foundational research possible again. It is often added in recent accounts that von Neumann's error had been spotted almost immediately by Grete Hermann, but that her discovery was of no effect due to the dominant Copenhagen Zeitgeist. We shall attempt to tell a story that is more historically accurate and less ideologically charged. Most importantly, von Neumann never claimed to have shown the impossibility of hidden variables tout court, but argued that hidden-variable theories must possess a structure that deviates fundamentally from that of quantum mechanics. Both Hermann and Bell appear to have missed this point; moreover, both raised unjustified technical objections to the proof. Von Neumann's argument was basically that hidden-variables schemes must violate the ;quantum principle; that physical quantities are to be represented by operators in a Hilbert space. As a consequence, hidden-variables schemes, though possible in principle, necessarily exhibit a certain kind of contextuality. As we shall illustrate, early reactions to Bohm's theory are in agreement with this account. Leading physicists pointed out that Bohm's theory has the strange feature that pre-existing particle properties do not generally reveal themselves in measurements, in accordance with von Neumann's result. They did not conclude that the ;impossible was done; and that von Neumann had been shown wrong.

  11. Möglichkeiten und Grenzen von Social Media Marketing

    OpenAIRE

    Kreutzer, Ralf T.; Hinz, Jule

    2010-01-01

    In diesem Arbeitspapier wird das Konzept des Social Media Marketing theoretisch dargestellt und seine Möglichkeiten und Grenzen analysiert. Dabei werden die einzelnen Instrumente des Social Media Marketings sowie die verschiedenen Nutzertypen erläutert und auf die Erfolgsmessung von Methoden des Social Media Marketings eingegangen. Schließlich wird an den Beispielen HUGO BOSS, Starbucks und Nestlé auf die Umsetzung von Maßnahmen des Social Media Marketings eingegangen und aufgezeigt, welche E...

  12. Rekonstruktion von Gletscherschwankungen mit Hilfe fossiler Hölzer

    OpenAIRE

    Holzhauser, H.

    1984-01-01

    Im vorliegenden Artikel wird gezeigt, wie mit Hilfe von fossilen Hölzern aus dem Gletschervorfeld minimale Gletscherausdehnungen und Gletschervorstöße erfaßt werden können. Einige zeitgenössische Berichte und Zeichnungen aus der Zeit um 1850, als die Alpengletscher ihren letzten neuzeitlichen Hochstand erreichten, sollen veranschaulichen, wie Gletscher in bewaldetes Gebiet vordrangen und Bäume umdrückten. Es wird im weiteren kurz auf die Lage von fossilem Holz im Gletschervo...

  13. Nationale Kapitalanlagengarantien als Instrumente zur Förderung von Direktinvestitionen

    OpenAIRE

    Stern, Thomas

    1988-01-01

    Wegen ihres hohen entwicklungspolitischen Stellenwertes ist die Steigerung von Direktinvestitionen als langfristiges Instrument zur Verbesserung der Verschuldenssituation der Länder der Dritten Welt anerkannt. Trotz dieser Erkenntnis blieben die Direktinvestitionen in Richtung der Entwicklungsländer in den letzten fünf Jahren hinter den Erwartungen zurück und erreichten lediglich einen Anteil von ca. 12 % an den Gesamtkapitalströmen. Wichtige Motive für die Zurückhaltung der Investoren in die...

  14. Kommunale Versorgung, Verortung und Steuerung von Risiken psychischer Gesundheit

    OpenAIRE

    Warner, Joanne

    2006-01-01

    Das Risikokonzept ist heute zentral für alle Bereiche von gesundheitlicher und sozialer Wohlfahrt in Großbritannien. Im Bereich psychischer Gesundheit und insbesondere seit den Maßnahmen zur kommunalen Versorgung in den 1990er Jahren kommt dem Konzept jedoch besondere Aufmerksamkeit zu. Dabei kamen bisher vor allem die Risiken in den Blick, die von Personen mit psychischen Erkrankungen oder in psychischer Not ausgehen, aber nicht die Risiken, mit denen diese selbst konfrontiert sind. Der vorl...

  15. Untersuchung der verfahrenstechnischen Potentiale von CO2-Minderungsoptionen

    OpenAIRE

    Geng, Jin

    2017-01-01

    Die vorliegende Arbeit beschäftigt sich mit der Untersuchung der verfahrenstechnischen Potentiale von CO2-Minderungsoptionen. CO2 ist ein Treibhausgas und führt zu Klimaproblemen. Wie erfolgreich der Kampf gegen den Klimawandel ist, hängt stark von den politischen Entscheidungen ab, welche auf internationalen Vereinbarungen und der wirtschaftlichen Situation basieren. Allerdings ist die technische Machbarkeit die Voraussetzung aller Entscheidungen. In der Arbeit wird diskutiert, welches P...

  16. CyLaw-Report V : "Sicherheit von ec-Karten"

    OpenAIRE

    Schmid, Viola

    2008-01-01

    Entscheidung des BGH vom 05.10.2004 - XI ZR 210/03 Die vorliegende Entscheidung des Bundesgerichtshof (BGH) bringt - nach jahrelanger juristischer Diskussion und divergierender Rechtsprechung verschiedener Gerichte - weiteren Aufschluss bei beweis- und haftungsrechtlichen Fragen von ec-Karten. Die besondere Bedeutung bargeldloser Zahlungsverfahren im Bereich von eCommerce, eGovernment etc. machen das Urteil des BGH zu einer für das Cyberlaw interessanten Entscheidung.

  17. Hochtemperaturverhalten von Stahlbetonplatten mit Textilbetonverstärkung

    OpenAIRE

    Hothan, Sascha; Ehlig, Daniel

    2011-01-01

    Die Verwendung von Endlosfilamenten aus Carbon als Bewehrungsmaterial für Beton, sogenannter Textilbeton, bietet die Möglichkeit der Sanierung und der Verstärkung bestehender Stahlbetonkonstruktionen. Dabei muss die Frage nach dem Feuerwiderstand von derart verstärkten Tragwerken beantwortet werden. Aufschluss darüber liefern Brandversuche. Mit Textilbeton verstärkte Stahlbetonplatten haben in Brandversuchen nach der Einheits- Temperaturzeitkurve bei 33 % der Traglast mehr als 60 Minuten stan...

  18. Expression von Wachstumsfaktoren unter Vakuumversiegelungstherapie in chronischen Wunden

    OpenAIRE

    Rosenberg, Birgit

    2012-01-01

    Hintergründe und Zielsetzung Die Vakuumversiegelungsmethode (V.A.C.® = Vac.) ist ein von M. Morykwas und C. Argenta 1988 entwickeltes Verfahren zur Konditionierung von akuten und chronischen Wunden, das sich in den letzten Jahren klinisch etabliert und dessen Anwendungsspektrum kontinuierlich zugenommen hat. In vielen klinischen Studien konnte die Wirksamkeit dieses Therapieverfahrens in der Wundbehandlung nachgewiesen werden, unklar ist jedoch bislang welche physiologischen Veränderungen auf...

  19. Nash y von Neumann: mundos posibles y juegos de lenguaje

    Directory of Open Access Journals (Sweden)

    Salazar , Boris

    2004-06-01

    Full Text Available Este ensayo emplea las nociones de juego de lenguaje y de equivalencia entre juegos para examinar la decisión de John Nash de no jugar el juego coalicional que propuso John von Neumann. El argumento central es que Nash concibió una clase de mundos posibles incompatible con la de von Neumann, y que en el origen de esa divergencia estarían sus distintas nociones de racionalidad.

  20. Entwicklung von Cellulosefaser-Leichtbeton und Untersuchung des bruchmechanischen Verhaltens

    OpenAIRE

    Thiel, Thomas

    2016-01-01

    Im Zentrum der vorliegenden Arbeit steht ein zementgebundener Leichtbeton auf der Basis von aus Altpapier herausgelösten Cellulosefasern; hierfür wird im Folgenden die Bezeichnung CFLC (Cellulose-Fibre Lightweight Concrete) verwendet. Die Intention zur Untersuchung dieses mitunter auch als Papercrete bezeichneten und bislang nur verhältnismäßig wenig erforschten Materials beruht insbesondere darauf, dass von einer Kombination eines leicht verfügbaren pflanzlichen Faserstoffs mit einem mineral...

  1. Ludwig von Bertalanffy Forerunner of Evolutionary Systems Theory

    OpenAIRE

    Wolfgang, Hofkirchner

    2005-01-01

    Ludwig von Bertalanffy is known as founding father of the General System Theory (GST). When Ludwig von Bertalanffy created his GST amidst the last century, he was able to overcome the deep cleft between the controversial theoretical approaches to biology - mechanicism and vitalism. He did so by formulating laws of organisation ruling biota and after generalising them he successfully applied them to different domains such as medicine, psychology, psychotherapy. Methodologically, Bertalanffy re...

  2. Optimized sampling of hydroperoxides and investigations of the water vapour dependence of hydroperoxide formation during ozonolysis of alkenes; Optimierung der Probenahme von Hydroperoxiden und Untersuchungen zur Wasserdampfabhaengigkeit der Bildung von Hydroperoxiden bei der Ozonolyse von Alkenen

    Energy Technology Data Exchange (ETDEWEB)

    Becker, K.H.; Plagens, H.

    1997-06-01

    There are several sampling methods for hydroperoxides none of which is particularly reliable. The authors therefore tested three new methods in order to optimize hydroperoxide sampling and, using the optimized sampling procedure, to investigate the water vapour dependence of hydroperoxide formation during ozonolysis of alkenes. (orig.) [Deutsch] Fuer die Probenahme von Hydroperoxiden existieren verschiedene Verfahren, von denen bisher keines als besonders zuverlaessig angesehen werden konnte. Daher wurden in dieser Arbeit drei Verfahren getestet, um die Probenahme von Hydroperoxiden zu optimieren und mit dem entsprechenden Verfahren die Wasserdampfabhaengigkeit der Bildung von Hydroperoxiden bei der Ozonolyse von Alkenen zu untersuchen. (orig.)

  3. Entwicklung einer kontaktfreien nichtdestruktiven Methode zur Messung von mechanischen und elastischen Eigenschaften von mikromechanischen Mehrschichtsystemen mit akustischen Oberflächenwellen

    OpenAIRE

    Bennis, Abdelali

    2009-01-01

    Mit dieser Arbeit wird ein Beitrag zur Weiterentwicklung der akustischen lasermesstechnischen Verfahren zur Ermittlung von mechanischen und elastischen Eigenschaften von mikromechanischen Mehrschichtsystemen geleistet. Zu diesen Eigenschaften zählen das E-Modul, die Dichte, die Dicke sowie die Poissonzahl. Die meisten akustischen lasermesstechnischen Verfahren basieren auf der optischen Erzeugung von breitbandigen akustischen Wellen in einem Schichtsystem und der Ermittlung der Geschwindig...

  4. Die graphic des ersten buches von Prekmurje

    Directory of Open Access Journals (Sweden)

    Vilko Novak

    1978-12-01

    Full Text Available Das erste Buch, geschrieben im Dialekt von Prekmurje (Übermurgebiet in Slowenien, war die Übersetzung des Kleinen Katechismus von M.Luther, angefertigt von Ferenc Temlin: Mali katechismus … erschienen in Halle, 1715. Über den Übersetzer schrieben bisher ausführlicher M. Rupel und V. Novak, s. Anmerkung 3. Der Verfasser untersucht, wie sich Temlin in seiner Schreibweise an die ungarische Graphik anlehnte, wie vor ihm schon die handschriftlichen Gesangbücher in Prekmurje und die kaj-kroatischen Schriftsteller. Vokalen.- Temlin benutzt konsequent die akutierten a und e fur lange und enge Vokale, indem ihm das nichtakutierte ein nichtakzentuiertes oder kurzakzentuiertes, niedriger aber einen breeiinten, nichtakzentuier­ten oder kurzakzentuierten Vokal bedeutet. Den Reflex von jat  schreibt er konsequent als ei, die dem Dialekt bekannten ö aus dem engen, kurzen postlabialen oder postdentalen e oder ,ü, und ü aus dem ethymologischen u (nur einige Ausnahmen in Lehnwörtern schreibt er mit diesen Buchstaben. (Beispiele für diese und die nächsten Buchstaben und Laute in der Abhandlung. Das lange o, das in seiner Sprache als Diphtong ou  reflektiert, bezeichnet er auch als solches. Konsonanten.- Für einige davon verwendet Temlin ungarische Doppelzeichen, deren Alter und Entwicklung der Verfasser der Abhandlung angibt. Das c bezeichnet T.ständig als cz, das s als /z, nur in einigen Fällen als z. Den letzt angeführten Buchstaben benutzt er konsequent für z.- Komplizierter ist die Bezeichnung der Zischlaute: für č schreibt T. sehr haufig cf, aber noch häufiger mit cs, sehr selten dagegen ch - Dies scheint aus kirchlichen Ausdrücken, aus kaj-kroatischen Büchern entnommen bzw. Durch die Kirche erlernt.- Das š bezeichnet T. mit doch nicht selten auch mit Dieselben zwei Buchstaben dienen ihm auch für die Bezeichnung des Lautes ž. Bei den Labiallauten ist zu bemerken, dass T. für die Präposition vu manch­ mal schreibt, sonst

  5. Politische Bildung im Kontext von Regierung, Unterricht und Zucht

    Directory of Open Access Journals (Sweden)

    Thomas Rucker

    2014-02-01

    Full Text Available Johann Friedrich Herbart (1776-1841 gilt als der Begründer der wissenschaftlichen Pädagogik. In diesem Beitrag wird zu zeigen versucht, dass Herbart die Unterstützung politischer Bildung als eine Aufgabe von Regierung, Unterricht und Zucht bestimmt und in seinem Werk wichtige „Bausteine“ für eine Theorie der politischen Bildung unter den Bedingungen von Erziehung vorlegt hat. Nach Herbart ist das politische Selbst- und Weltverhältnis auf Sachverhalte bezogen, die nicht nur die Lebensführung einzelner Menschen, sondern die Lebensführung einer Mehrzahl von Menschen betreffen. Nicht das Zusammenleben von Menschen generell ist jedoch Orientierungsgesichtspunkt des politischen Selbst- und Weltverhältnisses, sondern nur das problematisch gewordene Zusammenleben. Politik ergibt sich nach Herbart aus einem Konflikt hinsichtlich der Frage, wie das Zusammenleben von Menschen geregelt sein sollte. Während die Regierung lediglich mittelbar einen Beitrag zur politischen Bildung leistet, indem sie die Voraussetzungen für Unterricht und Zucht bereitstellt, fungieren die beiden zuletzt genannten Formen von Erziehung als die eigentlichen Medien, in denen die politische Bildung im Sinne Herbarts ihren Ort hat.

  6. Design of recuperators; Nachrechnung von Rekuperatoren. Moeglichkeiten zur Loesung der inverse Aufgabe bei waermetechnischen Berechnungen von Rekuperatoren mit Metallrohren

    Energy Technology Data Exchange (ETDEWEB)

    Stanev, R. [Univ. fuer Chemische Technologie und Metallurgie, Sofia (Bulgaria). Lehrstuhl fuer Physikalische Metalurgie und Waermeanlagen

    2004-02-01

    Possibilities for solution of the inverse task by heat-technical calculation of recuperators with metal pipes. The utilization of the waste heat in recuperators permits a decrease the fuel consumption of industrial and especially of metallurgical furnaces up to 30%. In the paper is represented a possibility for calculation of recuperators, which is useable not only for this application, than also for the design of air-coolers and other heat-exchangers. (orig.) [German] Die Abgaswaermenutzung mittels Rekuperator erlaubt eine Senkung des Brennstoffverbrauchs von Industrieoefen und insbesondere von den metallurgischen Oefen um bis zu 30%. Im Beitrag wird eine Moeglichkeit der Berechnung von Rekuperatoren vorgestellt, die nicht nur fuer diese Anwendung, sondern auch fuer die Auslegung von Luftkuehlern und anderen Waermeuebertragern verwendet werden kann. (orig.)

  7. Bitcoins. Eine Analyse von Kryptowaehrungen und deren Anwendung von kontroversiellen Angeboten im Darknet bis hin zu traditionellem Onlinehandel.

    OpenAIRE

    Linzner, Manfred

    2016-01-01

    von Manfred Linzner Arbeit an der Bibliothek noch nicht eingelangt - Daten nicht geprueft - Link noch nicht aktiv Abweichender Titel nach Übersetzung der Verfasserin/des Verfassers Technische Universität Wien, Univ., Diplomarbeit, 2016

  8. Electronic Resource Management System. Vernetzung von Lizenzinformationen

    Directory of Open Access Journals (Sweden)

    Michaela Selbach

    2014-12-01

    Full Text Available In den letzten zehn Jahren spielen elektronische Ressourcen im Bereich der Erwerbung eine zunehmend wichtige Rolle: Eindeutig lässt sich hier ein Wandel in den Bibliotheken (fort vom reinen Printbestand zu immer größeren E-Only-Beständen feststellen. Die stetig wachsende Menge an E-Ressourcen und deren Heterogenität stellt Bibliotheken vor die Herausforderung, die E-Ressourcen effizient zu verwalten. Nicht nur Bibliotheken, sondern auch verhandlungsführende Institutionen von Konsortial- und Allianzlizenzen benötigen ein geeignetes Instrument zur Verwaltung von Lizenzinformationen, welches den komplexen Anforderungen moderner E-Ressourcen gerecht wird. Die Deutsche Forschungsgemeinschaft (DFG unterstützt ein Projekt des Hochschulbibliothekszentrums des Landes Nordrhein-Westfalen (hbz, der Universitätsbibliothek Freiburg, der Verbundzentrale des Gemeinsamen Bibliotheksverbundes (GBV und der Universitätsbibliothek Frankfurt, in dem ein bundesweit verfügbares Electronic Ressource Managementsystem (ERMS aufgebaut werden soll. Ein solches ERMS soll auf Basis einer zentralen Knowledge Base eine einheitliche Nutzung von Daten zur Lizenzverwaltung elektronischer Ressourcen auf lokaler, regionaler und nationaler Ebene ermöglichen. Statistische Auswertungen, Rechteverwaltung für alle angeschlossenen Bibliotheken, kooperative Datenpflege sowie ein über standardisierte Schnittstellen geführter Datenaustausch stehen bei der Erarbeitung der Anforderungen ebenso im Fokus wie die Entwicklung eines Daten- und Funktionsmodells. In the last few years the importance of electronic resources in library acquisitions has increased significantly. There has been a shift from mere print holdings to both e- and print combinations and even e-only subscriptions. This shift poses a double challenge for libraries: On the one hand they have to provide their e-resource collections to library users in an appealing way, on the other hand they have to manage these

  9. Jade aus Amerika und weitere Grünsteinobjekte von Alexander von Humboldt aus dem Berliner Mineralienkabinett

    OpenAIRE

    Renate Nöller

    2009-01-01

    Alexander von Humboldt schickte aus Amerika Mineralproben, die nicht nur von naturwissenschaftlichem oder wirtschaftlichem Interesse waren. Vor allem grünes Steinmaterial, sogenannte Jade, war in Mesoamerika kulturell sehr bedeutsam und weit verbreitet, so daß sich aus diesem Rohstoff in der Sammlung auch bearbeitete Stücke vorfinden. Sie erlangten in Europa unterschiedliche Aufmerksamkeit. Die Jadeaxt und die Sonnenscheibe - beide im zweiten Weltkrieg verloren gegangen, hatten als “Humboldt-...

  10. Entwicklung von Dünnschicht-Reaktionsröhrchen zur Bestimmung von Schwefelwasserstoff, Chlor und Tetrachlorethen in Luft

    OpenAIRE

    Schwierz, Torge

    2000-01-01

    Dünnschicht-Reaktionsröhrchen zeichnen sich gegenüber konventionellen Prüfröhrchen durch höhere analytische Leistungen (Nachweisvermögen, Reproduzierbarkeit, Empfindlichkeit, objektive Auswertung) aus, ohne die Vorteile der einfachen praktischen Handhabung bei gasspurenanalytischen Messungen aufzugeben. In der vorliegenden Arbeit wird die Entwicklung von Dünnschicht-Reaktionsröhrchen für die Analyten Schwefelwasserstoff, Chlor und Tetrachlorethen beschrieben. Zum Nachweis von Schwefelwasserst...

  11. Die Rolle von Aldosteron im Knochenstoffwechsel

    Directory of Open Access Journals (Sweden)

    Tomaschitz A

    2012-01-01

    Full Text Available Das Steroidhormon Aldosteron wird in der Zona glomerulosa (ZG der Nebennierenrinde produziert. Kalzium spielt als gemeinsamer „Second Messenger“ eine zentrale Rolle in der durch Angiotensin II, ACTH und Kalium regulierten Aldosteronsynthese. Zahlreiche Studien der vergangenen Jahre dokumentierten die bedeutende Rolle des Aldosterons in der Pathogenese kardiovaskulärer Erkrankungen. Effekte von Aldosteron auf den Knochenstoffwechsel wurden bisher kaum untersucht. In humanen fetalen und adulten Knochenzellen wurde jedoch eine ausgeprägte Expression des Mineralokortikoidrezeptors (MR detektiert. In experimentellen Tierstudien wurden sowohl eine spezifische Aldosteron-MR-Interaktion im Knochengewebe als auch die osteoprotektive Wirkung der MR-Blockade mit Spironolakton beobachtet. Aldosteron kann durch die Hemmung der alkalischen Phosphatase indirekt eine gesteigerte Osteoblastenproliferation induzieren. Die wechselseitige Beeinflussung von Aldosteron und Parathormon (PTH könnte eine bedeutende Rolle in der Pathophysiologie kardiovaskulärer Erkrankungen und des Knochenstoffwechsels spielen. PTH kann sowohl über die Aktivierung spannungsabhängiger Kalziumkanäle als auch durch Bindung an den PTH/PTH-rP-Rezeptor der Zona-glomerulosa- Zellen eine Stimulation der Aldosteronsynthese induzieren. Dies bestätigt sich mit einem signifikanten Rückgang der Plasmaaldosteronkonzentration nach einer Parathyreoidektomie bei Patienten mit einem primären Hyperparathyreoidismus. In Tiermodellen der Herzinsuffizienz führt der relative Aldosteronüberschuss über eine verstärkte renale Kalziumexkretion zu einem sekundären Hyperparathyreoidismus, der durch die gesteigerte Kalziummobilisation aus dem Knochengewebe zu einem erhöhten Frakturrisiko in dieser Patientengruppe beiträgt. Der im Rahmen des primären Hyperaldosteronismus beobachtete sekundäre Hyperparathyreoidismus kann durch eine Hemmung der Aldosteronwirkung/Aldosteronsynthese supprimiert

  12. Von Roll RCP method - first experiences; Von Roll RCP - Verfahren. Erste Erfahrungen

    Energy Technology Data Exchange (ETDEWEB)

    Capitaine, P.; Engweiler, J. [Roll Umwelttechnik AG, Zuerich (Switzerland)

    1998-09-01

    The RCP method was designed as a residue-optimised alternative to the thermally optimised grate firing of residual wastes. Its technical realisation and development to market maturity took no more than 5 years. In the first process stage the waste is converted to high-carbon pyrolysis charcoal and high-rank gas in the absence of oxygen. In the second stage these substances are oxidised by addition of oxygen. The resulting temperature causes the non-combustible constituents of the slag to melt. In a third, optional, stage this molten slag can be liberated of (heavy) metals to such an extent that it can subsequently be used directly as additive for grinding. Further exhaust gas treatment is facilitated by the use of a circulatory fluidised-bed secondary combustion chamber. Despite the reduced flue gas volume and resultant higher pollutant concentrations in the crude gas, overall emissions are lower than in conventional plants. [Deutsch] Das reststoffoptimierte RCP Verfahren ist als Alternative zur thermisch optimierten Rostverbrennung von Restabfaellen konzipiert. In nur fuenf Jahren wurde das Verfahren technisch umgesetzt und zur Marktreife entwickelt. In einer ersten Stufe des Verfahrens wird der Abfall unter Luftabschluss in einen kohlenstoffreichen Pyrolysekoks und eine heizwertreiches Gas umgesetzt. Im zweiten Schritt werden diese Stoffe unter Zugabe von Sauerstoff oxidiert. Dabei treten Temperaturen auf, bei denen die nichtbrennbaren Bestandteile der Schlacke schmelzen. Optional wird diese Schmelzschlacke in einem dritten Schritt derart von (Schwer-) Metallen befreit, dass sie anschliessend direkt als Zement-Zumahlstoff eingesetzt werden kann. Die Nutzung der zirkulierenden Wirbelschicht - Nachbrennkammer zur Abgasbehandlung vereinfacht die weitergehende Abgasbehandlung. Trotz verringertem Rauchgasvolumen und damit hoeheren Schadstoffkonzentrationen im Rohgas werden die Gesamtemissionen gegenueber konventionellen Anlagen verringert. (orig./SR)

  13. GFRP life in wind rotor blades; Lebensdauer von GFK im Holmgurt und im Schubsteg von Rotorblaettern

    Energy Technology Data Exchange (ETDEWEB)

    Kensche, C.W. [Deutsche Forschungsanstalt fuer Luft- und Raumfahrt e.V. (DLR), Stuttgart (Germany). Inst. fuer Bauweisen- und Konstruktionsforschung

    1999-07-01

    Lives of GFRP rotor blade components were calculated on the basis of Woehler curves and the wind power specific standard WISPERX using the linear Palmgren-Miner rule. The Calculations were based on experiments with belts made of the L20/SL epoxy resin system at pressure ratios R=0.1, and -1 and with torsionally stressed GFRP tubes of linen tissue with the epoxy resing GE162/C260 at R=0.1 and -1. The results were incorporated into Haigh diagrams, from which the damage from the collective load was calculated by linear interpolation of the averaged stresses and amplitudes resp. the lines of constant life. The life curves calculated for different maximum stress horizons are in good agreement with experimental collective loads. A comparison of life curves showed that the linen tissue material was more fatigue-sensitive than the belt material. [German] Fuer GFK-Gurte und Schubstege von Rotorblaettern wurden Lebensdauerberechnungen auf der Basis von Woehlerkurven und dem windenergiespezifischen Standard WISPERX unter Anwendung der linearen Palmgren-Miner Regel durchgefuehrt. Bei der Betrachtung der Gurte wurde auf Versuche an UD-Proben mit dem Epoxidharzsystem L20/SL zurueckgegriffen, die bei Spannungsverhaeltnissen von R=0.1, -1 und 10 durchgefuehrt worden waren. Grundlage fuer die Lebensdauerbestimmung der Schubstege waren Ermuedungsversuche an torsionsbelasteten GFK-Rohren aus Leinengewebe mit dem Epoxidharz GE162/C260 bei R=0.1 und -1. In den daraus konstruierten Haighdiagrammen wurden die Schaedigungen durch das Lastkollektiv mittels linearer Interpolation der Mittelspannungen und Amplituden bzw. der Linien konstanter Lebensdauer ermittelt. Die Lebensdauerkurven, die fuer verschiedene Maximalspannungshorizonte ermittelt wurden, stimmen gut mit experimentellen Lastkollektivergebnissen ueberein. Beim Vergleich der Lebensdauerkurven zeigt sich, dass ein Leinengewebesteg erheblich ermuedungsgefaehrdeter ist als ein Gurt. (orig.)

  14. Entstehungsbedingungen von Übergängen von der Schule in den Beruf aus qualitativer und quantitativer Perspektive

    Directory of Open Access Journals (Sweden)

    Nora Gaupp

    2013-03-01

    Full Text Available Methodenintegration wird in den Sozialwissenschaften mit dem Argument sich ergänzender Erkenntnismöglichkeiten häufig gefordert. Die vorliegende Analyse zu Übergangswegen von der Schule in Ausbildung und Arbeit von Jugendlichen mit Hauptschulbildung beruht auf Daten einer quantitativen Panelstudie und einer qualitativen Ergänzungsstudie. Aus quantitativer und qualitativer Perspektive wird jeweils ein Einzelfall betrachtet sowie im Rahmen einer Modellbildung eine Verallgemeinerung auf Aggregatebene vorgenommen. Über ein logistisches Regressionsmodell wird die Bedeutung von zentralen individuellen, sozialen und strukturellen Merkmalen wie Schulleistungen, beruflichen Plänen, individuellen Problembelastungen, Migrationshintergrund und Arbeitslosigkeit der Eltern sowie regionaler Arbeitsmarktsituation für den Verlauf von Übergangswegen (Direkteinstieg in Ausbildung, Umweg in Ausbildung über schulische oder berufsvorbereitende Zwischenschritte, fortgesetzter mehrjähriger Schulbesuch und prekäre Wege in Ausbildungslosigkeit untersucht. Über das qualitative Paradigma konnten vier für den Verlauf von Übergängen zentrale Dimensionen identifiziert werden: Agency, Motivation, kritische biografische Ereignisse und soziale Interaktionen. Der Schritt der Modellbildung integriert die vier Erfahrungsebenen und zeigt ihre Bedeutung für Übergänge von der Schule in den Beruf. Die Diskussion fragt nach Möglichkeiten und Grenzen der Erkenntnisentwicklung der beiden Forschungszugänge. URN: http://nbn-resolving.de/urn:nbn:de:0114-fqs1302126

  15. Noninfectious differential diagnoses of pneumonia; Nichtinfektioese Differenzialdiagnosen von Pneumonien

    Energy Technology Data Exchange (ETDEWEB)

    Wielandner, A.; Toelly, A. [Medizinische Universitaet Wien, Universitaetsklinik fuer Radiologie und Nuklearmedizin, Wien (Austria); Agarwal, P. [Medizinische Universitaet Freiburg, Universitaetsklinik fuer Radiologie, Freiburg (Germany); Bardach, C. [Krankenhaus Hietzing, Abteilung fuer Radiologie, Wien (Austria)

    2017-01-15

    In patients with a clinical suspicion of pneumonia, typical clinical and laboratory features along with the detection of infiltrates on chest X-ray are as a rule considered diagnostic and therapy is immediately initiated; however, studies have shown that in up to 5% of patients with an initial suspicion of pneumonia, another noninfectious pulmonary disease was the underlying cause. Early recognition and differentiation of diseases mimicking pneumonia are prerequisites for an adequate therapy. The aim of this review is to present the important noninfectious differential diagnoses of pneumonia and to provide the reader with tools for a systematic diagnostic approach. A literature search was carried out. As alterations in the lungs often result in similar imaging appearances and a differentiation between transudates, exsudates, blood and cells is not feasible by chest X-ray or CT, a systematic approach is essential to make an appropriate diagnosis. Hence, consideration of the temporal course, predominant pattern, distribution of findings, additional findings and clinical presentation are indispensable. (orig.) [German] Bei der Verdachtsdiagnose Pneumonie sind die Kombination aus typischer Klinik, Labor und der Nachweis einer Verdichtung in der Roentgenthoraxaufnahme in der Regel diagnostisch und es wird umgehend mit der Therapie begonnen. Studien haben jedoch gezeigt, dass bei bis zu 5 % der Patienten mit Erstverdacht auf Pneumonie eine andere (pulmonale) Erkrankung zugrunde liegt. Ein fruehzeitiges Erkennen und eine Differenzierung von Erkrankungen, die eine Pneumonie vortaeuschen, sind fuer die weitere Behandlung essenziell. Uebersicht ueber wesentliche nichtinfektioese Differenzialdiagnosen der Pneumonie. Es wurde eine Literaturrecherche durchgefuehrt. Da krankhafte Lungenveraenderungen oft aehnliche Bilder hervorrufen und anhand der Roentgenthoraxaufnahme oder der CT-Untersuchung nicht zwischen Blut, Transsudat, Exsudat und Zellen differenziert werden kann, ist

  16. Einfluss von verschiedenen Erodierstrukturen auf den Reibwert von Kunststoff-Kunststoff Paarungen

    OpenAIRE

    Bergmann, André

    2016-01-01

    Im Beitrag werden bei verschiedenen Lastkollektiven und zwei Reibgeschwindigkeiten die Erodierstrukturen K27, K36, K42 und die Ätzstruktur K36 mit glatten Probekörpern hinsichtlich des Reibwertes und des Verschleißverhaltens untersucht. Als strukturierte Probekörper kommen die Kunststoffe POM und PA und als Gegenkörper zwei verschiedene PE-UHMW´s zum Einsatz. Die Ergebnisse zeigen eine deutliche Abhängigkeit des Reibwertes von der Oberflächenstruktur der Probekörper, jedoch ist die Senkun...

  17. "Die Toten reiten schnelle." Literarische Zitate in Briefen von und an Alexander von Humboldt

    Directory of Open Access Journals (Sweden)

    Ingo Schwarz

    2007-04-01

    Full Text Available Article in GermanAbstract in English and GermanAlexander von Humboldt and his correspondents liked to enrich or embellish their letters with quotations from classic works of ancient literature as well as from recent poems, plays, or novels. This paper tries to show that references to literature were very often used in order to express thoughts and feelings more clearly not so much for impressing the recipients of letters with high education. In educated circles a good knowledge of ancient and modern works of literature was a standard requirement, which often allowed the letter writers to use quotations in a playful manner.

  18. Entwurf und Simulation von Makromodellen zur transienten Simulation von thermo-elektrischen Kopplungen in einem Netzwerksimulator

    OpenAIRE

    Schacht, Ralph Karl Benjamin

    2002-01-01

    In der vorliegenden Arbeit wird eine Methode vorgestellt, welche die Beschreibung und Si-mulation von thermo-elektrischen Kopplungen während des Design-Prozesses mittels Mak-romodellierung unterstützt. Das Makromodell ist für den Einsatz in dem Schaltungssimulator PSpice zugeschnitten. Es ermöglicht die rechenzeiteffiziente transiente Simulation zwischen thermo-elektrisch gekoppelten Komponenten eines komplexen Mikrosystems. Im Rahmen dieser Arbeit wurde zunächst eine für die Problemstellung ...

  19. POLYCYSTIC KIDNEY DISEASE IN A PATIENT WITH ...

    African Journals Online (AJOL)

    hi-tech

    2003-01-01

    Jan 1, 2003 ... Autosomal dominant polycystic kidney disease is a multisystem disease involving many organs. An association with other diseases such as tuberous sclerosis, von Hippel-Lindau disease and Marfan syndrome have been previously described. We describe a 35 year old female with achondroplasia who ...

  20. Zur Struktur und Funktion von Synonymen

    Directory of Open Access Journals (Sweden)

    Marina Zorman

    1998-12-01

    Full Text Available Der Artikel behandelt die Synonyme im Licht der funktionell-strukturellen Sprachtheorie Coserius. 1 Die Wahl der theoretischen Grundsätze wurde durch die Festellung diktiert, daß die bisherigen Erkenntnisse über die Synonymie widersprüchlich sind und daß auch schon über alle Feststellungen berechtigte Einsprüche erhoben wurden. Deshalb wird in dieser Arbeit auf der genannten theoretischen Grundlage, die verschiedene Gesichtspunkte des Problems verbindet, anstatt sie zu trennen (s. Punkt 2, ein Modell für die Analyse von Synonymen vorgestellt (3, das die klassischen Schwächen der Semantikforschung (zu geringe Menge tatsächlicher Daten, Subjektivität und Introspektion durch eine empirische Untersuchung in einem Textkorpus zu ersetzen versucht. In die Analyse sind drei Inhaltsebenen einbezogen (Bezeichnung, Bedeutung und Sinn und ihnen sind jeweils entsprechende Untersuchungsmethoden zugeordnet. Im Sinne des Modells wurden in der Arbeit Zorman 1997 zwei slowenische Synonympaare (kuverta - ovojnica und borba - boj analysiert, die als Beispiele reiner (und totaler Synonymie gelten können. Die Analyse wird aus Platzgründen an dieser Stelle nicht detailliert dargestellt,2 veranschaulicht werden nur ihre Tragweite und die Ergebnisse (4. Im letzten Teil (5 werden die Leistungen der vorgeschlagenen Methode bewertet und einige Annahmen dargestellt, die der weiteren Erforschung der Synonyme dienen könnten. Die objektive Behandlung der Synonymie unter unterschiedlichen Gesichtspunkten scheint einige Dogmen über Synonyme in Frage zu  stellen.

  1. Disease: H00219 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available and disease (VWD); von Willebrand disease, platelet-type Hemophilia A and B are X-linked recessive disorders... which are the most common hereditary hemorrhagic disorders caused by a deficiency or dysfunction of blood c... Spreafico M, Tagliabue L, Asselta R, Duga S, Mannucci PM Genetic diagnosis of haemophilia and other inherited bleeding disorders

  2. Small bowel gastrointestinal stromal tumours and ampullary cancer in Type 1 neurofibromatosis

    Directory of Open Access Journals (Sweden)

    Fisher Cyril

    2004-01-01

    Full Text Available Abstract Background Type 1 neurofibromatosis (NF-1 is an autosomal dominant disorder with variable penetrance; approximately 50% of cases present as new mutations Case report We report a case of a 56 year-old man with Von Recklinghausen's disease, carcinoma of the ampulla of Vater and incidental benign gastrointestinal stromal tumours of the jejunum. Conclusions Coexistence between ampullary carcinoid, ectopic pancreatic tissue in the jejunum and neurofibroma of the jejunum in NF-1 has been previously described however; the association of synchronous carcinoma of the ampulla of Vater and gastrointestinal stromal tumour of the jejunum in NF-1 has not been previously reported.

  3. Solitary submucous neurofibroma of the mandible: review of the literature and report of a rare case

    Directory of Open Access Journals (Sweden)

    Kübler Norbert R

    2009-11-01

    Full Text Available Abstract Solitary neurofibroma is a rare benign non-odontogenic tumor. Particularly in the oral cavity, neurogenic tumors are rare, especially if they are malignant. Neurofibromas may present either as solitary lesions or as part of the generalised syndrome of neurofibromatosis or von Recklinghausen's disease of the skin. Clinically, oral neurofibromas usually appear as pediculated or sessile nodules, with slow growth and mostly without pain. The diagnosis can be confirmed by histological examination. Neurofibromas are immunopositive for the S-100 protein, indicating its neural origin. Treatment is surgical and the prognosis is excellent. For illustration a rare case of a solitary neurofibroma in the mandible is presented.

  4. Glomus-like bodies within a neurofibroma: a novel neoplasm arising in neurofibromatosis type 1 or a coincidence?

    Science.gov (United States)

    Thareja, Sumeet; Honigbaum, Alexis; Jukic, Drazen

    2015-04-01

    Neurofibromatosis type 1 is a relatively common genetic disorder with variable phenotypes. Tumors with features of both glomus tumors and neurofibromas are exceedingly rare in literature. Herein, we report a not yet described neoplasm with features of both a glomangioma/glomus tumor and a neurofibroma arising in a patient with segmental neurofibromatosis. Our case report supports the theory of a common lineage/ancestor cell between neurofibromas and glomus tumors and adds it to the spectrum of neoplasms that may arise in the setting of Von Recklinghausen's disease. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  5. Entwicklungsperspektiven von Social Software und dem Web 2.0

    Science.gov (United States)

    Raabe, Alexander

    Der Artikel beschäftigt sich zunächst mit dem derzeitigen und zukünftigen Einsatz von Social Software in Unternehmen. Nach dem großen Erfolg von Social Software im Web beginnen viele Unternehmen eigene Social Software-Initiativen zu entwickeln. Der Artikel zeigt die derzeit wahrgenommenen Einsatzmöglichkeiten von Social Software im Unternehmen auf, erörtert Erfolgsfaktoren für die Einführung und präsentiert mögliche Wege für die Zukunft. Nach der Diskussion des Spezialfalles Social Software in Unternehmen werden anschließend die globalen Trends und Zukunftsperspektiven des Web 2.0 in ihren technischen, wirtschaftlichen und sozialen Dimensionen dargestellt. Wie aus den besprochenen Haupttrends hervorgeht, wird die Masse an digital im Web verfügbaren Informationen stetig weiterwachsen. So stellt sich die Frage, wie es in Zukunft möglich sein wird, die Qualität der Informationssuche und der Wissensgenerierung zu verbessern. Mit dem Einsatz von semantischen Technologien im Web wird hier eine revolutionäre Möglichkeit geboten, Informationen zu filtern und intelligente, gewissermaßen verstehende" Anwendungen zu entwerfen. Auf dem Weg zu einem intelligenten Web werden sich das Semantic Web und Social Software annähern: Anwendungen wie Semantic Wikis, Semantic Weblogs, lightweight Semantic Web-Sprachen wie Microformats oder auch kommerzielle Angebote wie Freebase von Metaweb werden die ersten Vorzeichen einer dritten Generation des Webs sein.

  6. Biographical sketch: Georg Hermann von Meyer (1815-1892).

    Science.gov (United States)

    Skedros, John G; Brand, Richard A

    2011-11-01

    This biographical sketch on Georg Hermann von Meyer highlights the interactions in the 1860s that von Meyer, a famous anatomist, had with Karl Culmann, a famous structural engineer and mathematician. The published papers from this interaction caught the attention of Julius Wolff and stimulated his development of the trajectorial hypothesis of bone adaptation--now called "Wolff's Law." The corresponding translations are provided: (1) von Meyer's 1867 paper that highlights the regularity of arched trabecular patterns in various human bones, and his discussions with Culmann about their possible mechanical relevance; and (2) Wolff's 1869 paper that first mentions the correspondence of stress trajectories in a solid, crane-like structure to the arched trabecular patterns in the proximal human femur. This biographical sketch on Georg Hermann von Meyer corresponds to the historic texts, The Classic: The Architecture of the Trabecular bone (by von Meyer), and The Classic: On the Significance of the Architecture of the Spongy Substance for the Question of Bone Growth. A preliminary publication (by Wolff) available at DOIs 10.1007/s11999-011-2041-5 , 10.1007/s11999-011-2042-4 .

  7. Magnetic resonance imaging in the evaluation of pneumonia; Nachweis von pneumonischen Infiltraten mit der MRT

    Energy Technology Data Exchange (ETDEWEB)

    Eibel, R.; Herzog, P.; Dietrich, O.; Reiser, M.; Schoenberg, S.O. [Ludwig-Maximilians-Universitaet Muenchen (Germany). Institut fuer Klinische Radiologie; Rieger, C.; Ostermann, H. [Ludwig-Maximilians-Universitaet Muenchen (Germany). Abteilung fuer Haematologie/Onkologie, Klinikum Grosshadern

    2006-04-15

    Magnetic resonance imaging (MRI) of the lung is challenging because of substantial drawbacks. However, lung pathologies that are associated with increased attenuation values in CT enhance visualization in MRI: proton density is increased and tissue-air interfaces, resulting in susceptibility artifacts, are reduced in pneumonia, pneumonitis, edema, and carcinoma. On the other hand, many lung diseases result in shortness of breath, so that patients cannot hold their breath for long periods. Therefore, fast imaging techniques are required which should also allow for high spatial resolution so that small lesions can be detected. Calcifications and air pockets within lesions are not readily recognized with MRI. Thin section CT is standard for the diagnosis of pneumonia. With parallel imaging techniques, MRI examination of the lungs can be performed with short periods of breath holding, which allow for sub-centimeter resolution in the z-axis. Especially for follow-up examinations in immunocompromised patients and, in some instances, for the staging of malignant diseases (malignant pleural mesothelioma, lung cancer, respectively), MRI is very promising and may contribute to a decrease in the radiation exposure of the patients. (orig.) [German] Die MRT des Lungenparenchyms ist wegen des extrem niedrigen Protonengehalts des Lungengewebes und den starken Grenzflaechen zwischen Luft und Parenchym prinzipiell sehr problematisch. Allerdings liegt bei pathologischen Lungengewebeveraenderungen eine andere Situation vor, da infolge von Oedem, Entzuendung bzw. Tumorwachstum der Protonengehalt erhoeht und die Grenzflaechen reduziert sind. Viele Erkrankungen der Lunge fuehren dazu, dass der Patient den Atem nicht lange anzuhalten vermag. Daher sind MRT-Pulssequenzen erforderlich, die eine Datenakquisition in wenigen Sekunden gestatten. Aus technischer Sicht werden die vom CT bekannten Submillimeterschichten vielleicht nie realisierbar sein, sodass sicher auch in Zukunft kleine

  8. Groves-Schemata zur Lösung von Anreizproblemen bei der Budgetierung

    OpenAIRE

    Locarek, Hermann

    1992-01-01

    Groves-Schemata zur Lösung von Anreizproblemen bei der Budgetierung / von Günter Bamberg u. Hermann Locarek. - In: Controlling / Klaus Spremann ... (Hrsg.). - Wiesbaden : Gabler, 1992. - S. 657-670. - Auch ersch. als: Arbeitspapiere zur mathematischen Wirtschaftsforschung ; 110

  9. Proof of pipeline strength based on measurements of inspection pigs; Festigkeitsnachweis von Pipelines aufgrund der Messergebnisse von Pruefmolchen

    Energy Technology Data Exchange (ETDEWEB)

    De la Camp, H.J.; Feser, G.; Hofmann, A.; Wolf, B.; Schmidt, H. [TUeV Sueddeutschland Bau und Betrieb GmbH, Muenchen (Germany); Herforth, H.E.; Juengling, K.H.; Schmidt, W. [TUeV Anlagentechnik GmbH, Berlin-Schoeneberg (Germany). Unternehmensgruppe TUeV Rheinland/Berlin-Brandenburg

    2002-01-01

    The report is aimed at collecting and documenting the state of the art and the extensive know how of experts and pipeline operators with regard to judging the structural integrity of pipelines. In order to assess the actual mechanical strength of pipelines based on measurement results obtained by inspection pigs, guidance is given for future processing, which eventually can be used as a basis for an industry standard. A literature study of the commercially available types of inspection pigs describes and synoptically lists the respective pros and cons. In essence the report comprises besides check lists of operating data for the pipeline and the pig runs mainly the evaluation of defects and respective calculating procedures. Included are recommendations regarding maintenance planning, verification of defects as well as repetition of pig runs. (orig.) [German] Ziel des Berichtes ist die Erfassung und Dokumentation zum derzeitigen Stand der Technik und des vorhandenen umfangreichen Know-how von Sachverstaendigen und Pipelinebetreibern auf dem Gebiet der sicherheitstechnischen Beurteilung von Pipelines. Fuer den Festigkeitsnachweis von Pipelines aufgrund der Messergebnisse von Pruefmolchen wurde ein Leitfaden als Basis fuer die zukuenftige Vorgehensweise erstellt, der eventuell die Grundlage eines normativen Regelwerkes bilden kann. In einer Literaturstudie wurden die auf dem Markt befindlichen Pruefmolchtypen zusammenfassend beschrieben und ihre Vor- und Nachteile tabellarisch gegenuebergestellt und bewertet. Neben der Erstellung von Checklisten fuer notwendige Daten zum Betrieb der Pipeline und der Molchlaeufe bildet die Fehlerbewertung mit entsprechenden Berechnungsverfahren den Hauptteil dieses Berichtes. Hinweise zur Instandhaltungsplanung (Fehlerverifikation und Molchlaufwiederholung) werden gegeben. (orig.)

  10. Applikation von fermentierter Soja bei Krebspatienten zur Verminderung von Kachexie und Erhöhung der Apoptose - eine prospektive Pilotstudie

    Directory of Open Access Journals (Sweden)

    Jacob U

    2009-01-01

    Full Text Available Kachexie ist eine Begleiterkrankung von Krebs, Aids oder rheumatoider Arthritis. Andere Symptome dieser Erkrankungen sind Depression sowie chronische Erschöpfung. Für alle diese Begleiterkrankungen, die die Lebensqualität der schwererkrankten Patienten negativ beeinflussen, wird eine Erhöhung von Entzündungsmediatoren (z.B. IL-6 und TNF-α verantwortlich gemacht. Für Krebs wird eine Erniedrigung der Apoptose, ausgelöst durch eine Erhöhung von NFκB verantwortlich gemacht. Eine Gabe eines fermentierten Soja-Produktes, das in den USA seit 10 Jahren zur Verbesserung der Lebensqualität von Krebspatienten eingesetzt wird, wurde in einer prospektiven Studie untersucht: Für je 5 Patienten, die unter Prostata-, Ovar- oder Brustkrebs litten, konnte eine signifikante Verminderung von Kachexie, Stress und Depression festgestellt werden. Gleichzeitig konnte man für Prostata- und Ovarkrebs-Patienten eine Erhöhung der Genexpression apoptotischer Marker disseminierter Tumorzellen feststellen. Zusätzlich wurde eine starke Erhöhung der Genexpression des Tumorsuppressorgens p21 festgestellt. Auch wenn die prospektive Studie präliminär ist und die Anzahl der untersuchten Patienten zu klein, so widerspricht sie nicht unserer Annahme, dass es einen direkten Zusammenhang zwischen Krebs und Begleiterkrankungen, besonders der Kachexie, gibt.

  11. "Face to Interface": Zum Problem der Vertrauenskonstitution im Internet am Beispiel von elektronischen Auktionen

    OpenAIRE

    Brinkmann, Ulrich; Seifert, Matthias

    2001-01-01

    Am Beispiel von Internetauktionen geht der Beitrag der Frage nach, welche Rolle Vertrauen bei e-Commerce-Transaktionen spielt und über welche Institutionalisierungsprozesse Vertrauen befördert werden kann. Dazu wird zunächst mit Rückgriff auf den Stand der Forschung ein multidimensionales Verständnis von Vertrauen entwickelt. Anschließend wird die Spezifik von Internetauktionen als Sonderfall von e-Commerce aus der Perspektive der Vertrauensproblematik beleuchtet: beiden Transaktionsparteien ...

  12. Analyse von Videodokumentationen in der kunstpädagogischen Forschung

    Directory of Open Access Journals (Sweden)

    Anja Mohr

    2001-03-01

    Full Text Available Am Institut für Kunstpädagogik der Justus-Liebig-Universität werden seit 1996 unter der Leitung von Prof. Dr. A. von Criegern Untersuchungen zum kindlichen Bildverhalten am Computer durchgeführt. Aus ersten explorativen Studien ist inzwischen ein Forschungsprojekt entstanden, das auf breiterer theoretisch und empirischer Basis das bildnerische Verhalten von Vor- und frühen Grundschulkindern am Computer untersucht. Entgegen der bisher ergebnisorientierten, eher quantitativ arbeitenden Kinderzeichnungsforschung, stellte das Giessener Team sehr schnell fest, dass der gesamte Malprozess und mehr noch das gesamte ästhetische Verhalten am Computer mit qualitativen Methoden erforscht werden muss. Um diesem Anspruch gerecht zu werden, wurden die Beobachtungs-, Aufzeichnungs- und Analyseverfahren schrittweise optimiert und angepasst. Der Beitrag stellt die Bedeutung der im Projekt angewendeten Form der Videodokumentation für die Erforschung der digitalen Kinderzeichnung dar.

  13. Die Welt der Metadaten im Universum von Repositorien

    Directory of Open Access Journals (Sweden)

    Susanne Blumesberger

    2016-02-01

    Full Text Available Im Rahmen des österreichweiten Projekts „e-Infrastructures Austria“ wird derzeit im Cluster I „Metadatenkomplex aus technischer und nichttechnischer Sicht“ ein Wissensnetzwerk und eine Wissensplattform über Metadaten aufgebaut. Ziel ist die Vorbereitung von Guidelines wie man sowohl im IT-Bereich als auch in Bibliotheken im Bereich von Aufbau und Betrieb eines Repositoriums mit unterschiedlichen Fragen bzgl. Metadaten umgehen kann und soll. Neben Übersichten über die Umsetzungsmöglichkeiten von Linked (Open Data, Metadatenstandards, Best-Practicemodellen, neuen technischen Entwicklungen z. B. im Bereich Controlled Vocabularies, einer Bedarfserhebung von ForscherInnen zum Thema Metadaten für Forschungsdaten und einer Reihe an Use Cases wird auch eine Studie über den derzeitigen Stand der Dinge beim Umgang mit Metadaten an österreichischen Institutionen vorbereitet. Auch die Frage, wie Metadaten möglichst barrierefrei gestaltet sein können, bzw. wie Metadaten dazu beitragen können, dass Objekte für möglichst alle Menschen frei zugänglich sind, wird zentral behandelt. All diese Expertisen, die zu einer Umsetzung von Open Science führen sollen, fließen in die tägliche Praxis ein, zum Beispiel in die Gestaltung von Datamanagementplänen. 2016 wird an der Universität Wien eine Veranstaltung stattfinden, die zur Bewusstseinsbildung für die Wichtigkeit qualitätsvoller Metadaten beitragen soll. ExpertInnen sind dabei eingeladen, miteinander zu diskutieren und Erfahrungen auszutauschen. Es ist geplant, die Ergebnisse, die im Rahmen dieses Clusters entstehen, Open Access zu publizieren.

  14. Contribution to increase coach safety; Die Sicherheit von Omnibussen

    Energy Technology Data Exchange (ETDEWEB)

    Grandel, J.; Mueller, C.F.

    1996-09-01

    The risk of injuries for coach passengers is comparatively low. Nevertheless some periodical occurring serious accidents cause discussions about the coach safety. To objectivize this discussion and to find measures to increase coach safety the Dekra accident research team has started to analyse the real accident events. The basic instrument for this work is the Dekra accident database. The analysis of about 200 real coach accidents shows that single accidents and rollover accidents cause a high risk of serious injuries for the coach occupants. The ejection of unbelted passengers and the collapse of the roof structure are often cause of death and severe injury. To get more detailed information about the kinematic processes during the impact it was necessary to design crash tests based on the results of the real accident investigations. To realize these crash tests Dekra used its own crash centre in Neumuenster. The present study shoes that an integral passenger safety-system consisting of seat, restraint system and body structure is able to increase coach safety. The effectiveness of this system assumes an optimised interacting between every part of the system. (orig.) [Deutsch] Einzelne schwere Busunfaelle sorgen immer wieder fuer negative Schlagzeilen und fuehren zu Diskussionen ueber die Sicherheit von Omnibussen. Fehlendes Grundlagenwissen fuehrt dabei haeufig zu falschen Schlussfolgerungen. Wichtigste Basis fuer eine sachliche Diskussion und die Ableitung von Verbesserungsmassnahmen bildet eine sorgfaeltige und tiefgehende Analyse der realen Omnibusunfaelle. Das Defizit in der grundlegenden Erforschung von Busunfaellen wurde durch eine umfassende Unfallanalyse der Dekra-Unfallforschung beseitigt. Aus dem Realunfallgeschehen abgeleitete und im Dekra-Crash-Zentrum durchgefuehrte Versuche vervollstaendigen das Gesamtbild und geben detaillierte Erkenntnisse bezueglich der Kinematik von Omnibus und Businsassen. Ausgehend von einem bereits hohen Sicherheitsniveau

  15. Der Beitrag von Schreib Peer Tutor_innen zur nachhaltigen Entwicklung von Schreibzentren. Eine empirische Untersuchung auf der Grundlage von Expert_inneninterviews mit Peer Tutor_innen aus deutschen Schreibzentren.

    OpenAIRE

    Poloubotko, Anja

    2014-01-01

    Die vorliegende Arbeit trägt zur Erweiterung der Schreibzentrumstheorie bei, indem die Perspektiven von Peer Tutor_innen auf ihre Rolle im Schreibzentrum und deren Beitrag zur Entwicklung von Schreibzentren beleuchtet werden. Inspiriert durch das Peer Writing Tutor Alumni Research Project (Hughes et al. 2010) und die Erkenntnis von Katrin Girgensohn, dass Peer Tutor_innen eine Schlüsselrolle innerhalb der Schreibzentrumsentwicklung wahrnehmen, sollen im Rahmen der Arbeit folgende Fragen beant...

  16. Acquired von Willebrand syndrome in children with aortic and pulmonary stenosis.

    Science.gov (United States)

    Binnetoğlu, Fatih Köksal; Babaoğlu, Kadir; Filiz, Şayegan Güven; Zengin, Emine; Altun, Gürkan; Kılıç, Suar Çakı; Sarper, Nazan

    This prospective study was planned to investigate the frequency and relationship of acquired von Willebrand syndrome (AVWS) with aortic and pulmonary stenosis in patients. A total of 84 children, ranging from two to 18 years of age, were enrolled in this study. Of these, 28 had isolated aortic stenosis, 32 had isolated pulmonary stenosis and 24 were healthy. Children with aortic and pulmonary stenosis associated with other congenital heart diseases were excluded. Children with hypothyroidism, renal or liver disease, malignancy or autoimmune disease were also excluded. Wholeblood count, blood group, factor VIII level, prothrombin time (PT), activated partial thromboplastin time (aPTT), von Willebrand factor antigen (VWF:Ag), ristocetin co-factor (VWF:RCo), and bleeding time using a platelet-function analyser (PFA-100) were performed in all patients. All of the children in the study underwent a detailed physical examination and echocardiographic evaluation. A history of bleeding was positive in 18% of the aortic stenosis group, 9% of the pulmonary stenosis group, and 4% of the control group. Seven of 60 (12%) patients had laboratory findings that implied a diagnosis of AVWS, and two of these (28%) had a history of bleeding. The frequency of AVWS was 14% in patients with aortic stenosis and 9% in those with pulmonary stenosis. AVWS is not rare in stenotic obstructive cardiac diseases. A detailed history of bleeding should be taken from patients with valvular disease. Even if the history is negative, whole blood count, PT and aPTT should be performed. If necessary, PFA-100 closure time and further tests should be planned for the diagnosis of AVWS.

  17. Treatment of Exudative and Vasogenic Chorioretinal Diseases Including Variants of AMD and Other CNV Related Maculopathy

    Science.gov (United States)

    2012-10-24

    Coats' Disease; Idiopathic Retinal Telangiectasia; Retinal Angiomatous Proliferation; Polypoidal Choroidal Vasculopathy; Pseudoxanthoma Elasticum; Pathological Myopia; Multi-focal Choroiditis; Rubeosis Iridis; Von Hippel Lindau Disease; BEST VITELLIFORM MACULAR DYSTROPHY, MULTIFOCAL (Disorder)

  18. Die grossen Physiker und ihre Entdeckungen von den fallenden Körpern zu den Quarks

    CERN Document Server

    Segrè, Emilio

    1998-01-01

    Von Galileo Galilei bis zu Richard Feynman und Murray Gell-Mann - von den fallenden Körpern zu den Quarks: Der Physiknobelpreisträger Emilio Segre hat seine ganz persönliche Geschichte der Physik geschrieben. Er erzählt von den großen Gestalten und deren wichtigen Entdeckungen mit großer Anschaulichkeit und Lebendigkeit.

  19. Muuga / Mary von Grünewaldt ; tõlk. Mati Sirkel

    Index Scriptorium Estoniae

    Grünewaldt, Mary von

    2004-01-01

    Leo Zoegelt ostetud Muuga mõisahoone korrastamisest ja uue hoone ehitamisest C. T. von Neffi kunstikogu paigutamise tarbeks. Tütre mälestused. Tõlgitud raamatust: Skizzen und Bilder aus dem Leben Carl Timoleon von Neff / Mary von Grünewaldt. Darmstadt, 1887

  20. „Der Einsatz von handlungs-, erfahrungs- und erlebnisorientierten Methoden in der Lehrerinnen- und Lehrerfortbildung von pädagogischen Führungskräften zur Initiierung von Lernen – Studien zur Verknüpfung von Erfahrung, Reflexion und Transfer“

    OpenAIRE

    Post, Eva - Maria

    2010-01-01

    Bei der Dissertation handelt es sich um eine Studie zum Einsatz von handlungs-, erfahrungs- und erlebnisorientierten Methoden. Diese wurden in der Lehrerinnen- und Lehrerfortbildung von pädagogischen Führungskräften untersucht und hinsichtlich ihrer Initiierung von Lernen überprüft. Dazu wurden der Transfer, die Wirksamkeit und die Nachhaltigkeit in Bezug auf die angebotenen Verfahren beschrieben und ausgewertet.

  1. Dr. Space the life of Wernher von Braun

    CERN Document Server

    Ward, Bob

    2013-01-01

    Written by veteran aerospace journalist Bob Ward, who spent years investigating his subject, this biography presents a revealing but even-handed portrait of the father of modern rocketry. As he chronicles Wernher von Braun's life, Ward explodes many myths and misconceptions about the controversial genius who was a hero to some, a villain to others. The picture of von Braun that emerges is of a brilliant scientist with limitless curiosity and a drive to achieve his goals at almost any price from, developing the world's first ballistic missile used against the Allies in World War II to help

  2. The smooth entropy formalism for von Neumann algebras

    Energy Technology Data Exchange (ETDEWEB)

    Berta, Mario, E-mail: berta@caltech.edu [Institute for Quantum Information and Matter, California Institute of Technology, Pasadena, California 91125 (United States); Furrer, Fabian, E-mail: furrer@eve.phys.s.u-tokyo.ac.jp [Department of Physics, Graduate School of Science, University of Tokyo, Tokyo, Japan and Institute for Theoretical Physics, Leibniz University Hanover, Hanover (Germany); Scholz, Volkher B., E-mail: scholz@phys.ethz.ch [Institute for Theoretical Physics, ETH Zurich, Zurich (Switzerland)

    2016-01-15

    We discuss information-theoretic concepts on infinite-dimensional quantum systems. In particular, we lift the smooth entropy formalism as introduced by Renner and collaborators for finite-dimensional systems to von Neumann algebras. For the smooth conditional min- and max-entropy, we recover similar characterizing properties and information-theoretic operational interpretations as in the finite-dimensional case. We generalize the entropic uncertainty relation with quantum side information of Tomamichel and Renner and discuss applications to quantum cryptography. In particular, we prove the possibility to perform privacy amplification and classical data compression with quantum side information modeled by a von Neumann algebra.

  3. Von-Neumann and Beyond: Memristor Architectures

    KAUST Repository

    Naous, Rawan

    2017-05-01

    An extensive reliance on technology, an abundance of data, and increasing processing requirements have imposed severe challenges on computing and data processing. Moreover, the roadmap for scaling electronic components faces physical and reliability limits that hinder the utilization of the transistors in conventional systems and promotes the need for faster, energy-efficient, and compact nano-devices. This work thus capitalizes on emerging non-volatile memory technologies, particularly the memristor for steering novel design directives. Moreover, aside from the conventional deterministic operation, a temporal variability is encountered in the devices functioning. This inherent stochasticity is addressed as an enabler for endorsing the stochastic electronics field of study. We tackle this approach of design by proposing and verifying a statistical approach to modelling the stochastic memristors behaviour. This mode of operation allows for innovative computing designs within the approximate computing and beyond Von-Neumann domains. In the context of approximate computing, sacrificing functional accuracy for the sake of energy savings is proposed based on inherently stochastic electronic components. We introduce mathematical formulation and probabilistic analysis for Boolean logic operators and correspondingly incorporate them into arithmetic blocks. Gate- and system-level accuracy of operation is presented to convey configurability and the different effects that the unreliability of the underlying memristive components has on the intermediary and overall output. An image compression application is presented to reflect the efficiency attained along with the impact on the output caused by the relative precision quantification. In contrast, in neuromorphic structures the memristors variability is mapped onto abstract models of the noisy and unreliable brain components. In one approach, we propose using the stochastic memristor as an inherent source of variability in

  4. Regulation of plasma von Willebrand factor [version 1; referees: 3 approved

    Directory of Open Access Journals (Sweden)

    Karl C Desch

    2018-01-01

    Full Text Available Von Willebrand factor (VWF is a multimeric plasma glycoprotein that plays a central role in the initiation of blood coagulation. Through interactions between its specific functional domains, the vascular wall, coagulation factor VIII, and platelet receptors, VWF maintains hemostasis by binding to platelets and delivering factor VIII to the sites of vascular injury. In the healthy human population, plasma VWF levels vary widely. The important role of VWF is illustrated by individuals at the extremes of the normal distribution of plasma VWF concentrations where individuals with low VWF levels are more likely to present with mucocutaneous bleeding. Conversely, people with high VWF levels are at higher risk for venous thromboembolic disease, stroke, and coronary artery disease. This report will summarize recent advances in our understanding of environmental influences and the genetic control of VWF plasma variation in healthy and symptomatic populations and will also highlight the unanswered questions that are currently driving this field of study.

  5. Einfluss von Umweltbedingungen und Entwicklungsstadium auf Ertragsparameter und sekundäre Pflanzeninhaltsstoffe von Zitronenmelisse (Melissa officinalis L.

    Directory of Open Access Journals (Sweden)

    Russo, Marco

    2014-09-01

    Full Text Available Um die Qualität von Arznei- und Gewürzpflanzen langfristig sichern zu können, ist eine genauere Kenntnis der sie beeinflussenden Faktoren von Interesse. Am Beispiel der Zitronenmelisse (Melissa officinalis L. wurden in zwei Feldversuchen an unterschiedlichen Standorten die Einflüsse der Faktoren Lichtintensität, Sorte und Erntezeitpunkt auf Ertragsparameter sowie sekundäre Pflanzeninhaltsstoffe untersucht. Es konnten teilweise deutliche Effekte der unterschiedlichen Lichtbedingungen auf die Gesamtphenole und die antioxidative Kapazität beobachtet werden, die zum Teil signifikanten Wechselwirkungen zwischen den Prüffaktoren unterlagen.

  6. Proteomics in der modernen Toxikologie : Identifizierung, Charakterisierung und Prävalidierung von Protein-Biomarkern zur verbesserten Vorhersage von Leberkanzerogenese

    OpenAIRE

    Fella, Kerstin

    2006-01-01

    Die heutige moderne Toxikologie ist dem 3-R-Prinzip (Russel und Burch, 1959), einem Konzept zur Verminderung und Verkürzung von Tierversuchen, verpflichtet. Allerdings stellt insbesondere die für die Zulassung und Registrierung neuer Arzneistoffe oder Chemikalien behördlich geforderte Prüfung von Substanzen auf kanzerogene Eigenschaften immer noch einen langwierigen Prozess mit einem hohen Bedarf an Versuchstieren dar. Daher sollte unter dem Einsatz von Methoden der Proteomforschung eine Iden...

  7. Einfluss von Futtermitteln und der Fütterungsstrategie auf die Fischproduktion und die Produktqualität von ökologischen Fischereierzeugnissen

    OpenAIRE

    Reiter, Reinhard; Benedikt, Frenzl; Schmidt, Gregor; Karl, Horst; Manthey-Karl, Monika

    2011-01-01

    Im Rahmen eines vom Bundesministerium für Ernährung, Landwirtschaft und Verbraucherschutz (BMELV) finanzierten und von der Bundesanstalt für Landwirtschaft (BLE) betreuten Projektes wurde der Einfluss von Futtermitteln und der Fütterungsstrategie auf die Fischproduktion und die Produktqualität von ökologischen Fischereierzeugnissen untersucht. Für die Untersuchungen wurden Bachforellen (Salmo trutta fario L.) und Seesaiblinge (Salvelinus alpinus L.) unter den Produktionsbedingungen eines ...

  8. Geschlechteridentitäten von Jugendlichen im Kontext von Social Media : Jahrestagung der Fachstelle Gender NRW (FUMA) am 26. Juni 2014 in der Volkshochschule Düsseldorf

    OpenAIRE

    Richter, Susanne

    2015-01-01

    "Auf der Tagung der Fachstelle Gender NRW (FUMA) 'Geschlechteridentitäten von Jugendlichen im Kontext von Social Media', die am 26.06.2014 in Düsseldorf stattfand, stand der Umgang mit neuen Medien im Mittelpunkt. Diskutiert wurden die Praktiken von Jugendlichen in sozialen Netzwerken und die Herausforderungen, die sich hieraus für Mitarbeitende der Kinder- und Jugendhilfe ergeben. Dabei wurde auch der Frage nach Auswirkungen auf Geschlecht und Geschlechtsidentitäten nachgegangen." (Autorenre...

  9. Untersuchungen zur Gentoxizität von Acrylamid in Abhängigkeit von der Cytochrom P450 2E1 Aktivität in humanen Hepatomzellen

    OpenAIRE

    Völkel, Yvonne

    2012-01-01

    Acrylamid ist eine von der IARC als möglicherweise karzinogen eingestufte Substanz, welche vor allem über Zigarettenrauch und kohlenhydratreiche Nahrungsmittel vom Menschen aufgenommen wird. In der vorliegenden Arbeit wurde die Gentoxizität von Acrylamid in HepG2-Zellen mit Hilfe der Einzelzellgelelektrophorese (Cometassay) untersucht. Weiterhin wurde der Einfluss von Ethanol, welches Cytochrom P 450 2E1 induziert, auf die durch Acrylamid hervorgerufene DNA-Migration betrachtet. Acryla...

  10. Die Mediatisierung professionellen Handelns. Zur Notwendigkeit von Handlungskompetenzen im Kontext digitaler Medien in der Sozialen Arbeit

    Directory of Open Access Journals (Sweden)

    Christian Helbig

    2017-04-01

    Full Text Available Digitale Medien durchdringen sowohl den Alltag von Adressat/innen als auch die Institutionen und Organisationen Sozialer Arbeit und in der Folge das professionelle Handeln von Fachkräften. Diese Entwicklungen sind vor dem Hintergrund gesellschaftlicher Teilhabe und somit im Kontext individueller Herausforderungen zu betrachten. Um weder den Anschluss an die Lebenswelten ihrer Adressat/innen zu verlieren, noch institutionelle Ausgrenzungsprozesse zu fördern, ist eine Professionalisierung der Sozialen Arbeit im Kontext von digital vernetzten Medien notwendig. Dies erfordert sowohl die Herausbildung von kritischer Medienkompetenz als auch von medienpädagogischen Handlungskompetenzen.

  11. Thermal processing of animal meal and animal bodies. Conference; Thermische Verwertung von Tiermehl und Tierkoerpern. Tagung

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    2001-07-01

    The problems of BSE and foot-and-mouth disease has shown how important it is to develop economically and ecologically acceptable processes for disposal of animal meal and animal body residues. The focus of the discussion was on co-combustion in power stations and incinerators, mono-combustion and concepts for thermal disposal. VDI-Berichte 1622 addresses planners, producers and operators of power plants, incinerators and plants for disposal of animal residues, licensing authorities, consultants and scientists. [German] Die BSE- und MKS-Problematik in den letzten Monaten hat gezeigt, wie notwendig es ist, wirtschaftliche und oekologisch vertraegliche Verfahren zur Entsorgung von Tiermehl und Tierresten zu entwickeln und umzusetzen. Im Mittelpunkt der Diskussion standen daher Hintergrundinformationen und erste Erfahrungen mit dem UBA-Leitfaden zur Tiermehlbeseitigung, die Technik der Mitverbrennung in Kraftwerken und Muellverbrennungsanlagen, die Monoverbrennung sowie Konzepte zur thermischen Verwertung in Tierkoerperbeseitigungsanlagen. Die VDI-Berichte 1622 wenden sich an Planer, Hersteller und Betreiber von Kraftwerken, Muellverbrennungs- und Tierkoerperbeseitigungsanlagen, an Genehmigungsbehoerden sowie an auf diesem Gebiet taetige Beratungsunternehmen und Wissenschaftler. (orig.)

  12. Molecular characterization of exon 28 of von Willebrand's factor gene in Nigerian population.

    Science.gov (United States)

    Ezigbo, E D; Ukaejiofo, E O; Nwagha, T U

    2017-02-01

    Polymorphisms in von Willebrand factor (VWF) gene are an important contributor to the expression of VWF gene and differences in ethnic distribution of these single nucleotide polymorphisms (SNPs) exists. Our objective was to molecularly characterize the exon 28 of the VWF gene in the three major ethnic groups of Nigeria. We recruited 90 subjects, 45 had a history of bleeding. Questions included those used in the Zimmerman Program for the Molecular and Clinical Biology of von Willebrand disease (VWD), and the bleeding scores were calculated using the Molecular and Clinical Markers for the Diagnosis and Management of type 1 VWD scoring system. Full blood count, coagulation profile, VWF:antigen level and VWF:collagen-binding activities were carried out. Data were analyzed using GraphPad Prism (5.03). GraphPad Software, Inc USA. The BigDye terminator chemistry was used to determine the nucleotide sequences of VWF gene (exon 28). Eight SNPs were identified, rs 216310 (T1547), rs 1800385 (V1565L), rs1800384 (A1515), rs1800383 (D1472H), rs 1800386 (Y1584C), rs 216311 (T1381A), rs 216312 (intronic) and rs 1800381 (P1337). The SNPs rs 216311, rs 1800383 and rs 1800386 associated significantly with bleeding in study subjects. rs1800386 occurred in all with bleeding history, no ethnic variations were noted.

  13. Characterization of Zebrafish von Willebrand Factor Reveals Conservation of Domain Structure, Multimerization, and Intracellular Storage

    Directory of Open Access Journals (Sweden)

    Arunima Ghosh

    2012-01-01

    Full Text Available von Willebrand disease (VWD is the most common inherited human bleeding disorder and is caused by quantitative or qualitative defects in von Willebrand factor (VWF. VWF is a secreted glycoprotein that circulates as large multimers. While reduced VWF is associated with bleeding, elevations in overall level or multimer size are implicated in thrombosis. The zebrafish is a powerful genetic model in which the hemostatic system is well conserved with mammals. The ability of this organism to generate thousands of offspring and its optical transparency make it unique and complementary to mammalian models of hemostasis. Previously, partial clones of zebrafish vwf have been identified, and some functional conservation has been demonstrated. In this paper we clone the complete zebrafish vwf cDNA and show that there is conservation of domain structure. Recombinant zebrafish Vwf forms large multimers and pseudo-Weibel-Palade bodies (WPBs in cell culture. Larval expression is in the pharyngeal arches, yolk sac, and intestinal epithelium. These results provide a foundation for continued study of zebrafish Vwf that may further our understanding of the mechanisms of VWD.

  14. The Influence of Field Marshal Colmar Von Der Goltz on Ottoman Military Effectiveness in Mesopotamia: December 1915 to April 1916

    Science.gov (United States)

    2012-02-23

    the Ottoman Fifth Army was activated to defend the Dardanelles, and the Ottomans gave Limon von Sanders command. With Limon von Sanders’ move to...Fifth Army, von der Goltz took command of the First Army and the German military mission which were both previously commanded by Limon von Sanders.51

  15. Molecular characterization of exon 28 of von Willebrand's factor ...

    African Journals Online (AJOL)

    Background: Polymorphisms in von Willebrand factor (VWF) gene are an important contributor to the expression of VWF gene and differences in ethnic distribution of these single nucleotide polymorphisms (SNPs) exists. Aims: Our objective was to molecularly characterize the exon 28 of the VWF gene in the three major ...

  16. Lars von Trier ja kombekuse riismed / Kadri Kõusaar

    Index Scriptorium Estoniae

    Kõusaar, Kadri, 1980-

    2011-01-01

    Taani filmilavastaja Lars von Trieri Cannes'is persona non grata'ks kuulutamisest tema filmi "Melanhoolia" esilinastusele järgnenud pressikonverentsil öeldu pärast. Autor ja tema teos tuleb hoida lahus. Trieri filmid jäävad sama raputavateks meistriteosteks, kui nad olid enne

  17. Playing the Waves : Lars von Trier's Game Cinema

    NARCIS (Netherlands)

    Simons, Jan

    2007-01-01

    Dogma95 werd toegejuicht als een Europees alternatief voor de Hollywoodblockbuster. Voor veel critici en filmliefhebbers is Dogma95 gaan staan voor de begrippen waarmee de kunstzinnige film gebruikelijk in verband wordt gebracht: low budget en realisme. Von Triers benadering van filmen voert film

  18. Ueber die von Prof. Dr. A. Gerstaecker beschriebenen Ascalaphidae (Neuroptera)

    NARCIS (Netherlands)

    Weele, van der H.W.

    1906-01-01

    In den Mitth. des naturw. Ver. für Neuvorpomm. und Rügen, Band XVI, XIX, XX. und XXV, hat Gerstaecker eine Reihe neuer Arten Planipennia beschrieben, von welchen ich durch die Freundlichkeit seines Nachfolgers Prof. G. W. Müller in Greifswald die Ascalaphiden zur Untersuchung bekommen habe. Obschon

  19. Mollusken aus posttertiären Schichten von Celebes

    NARCIS (Netherlands)

    Schepman, M.M.

    1912-01-01

    Die im Folgenden behandelten Mollusken stammen aus der Nähe von Kajoe ragi auf Celebes, woselbst sie durch R. FENNEMA aus denselben Schichten ausgelesen wurden, aus denen früher durch J. G. DE MAN einige brachyure Krebse beschrieben sind. Es darf deswegen betreffs näherer Angaben über die Herkunft

  20. Sakslased tulevad! Ärevad ajad Tallinnas Von Krahli Teatris

    Index Scriptorium Estoniae

    2001-01-01

    Von Krahli Teater ja Showcase Beat Le Mot valmistuvad oma esimeseks koostööprojektiks - lavastuseks "Pirates", kus osalevad mõlema teatri näitlejad ja tehnikud. Ühisprojekti toetavad Goethe Instituut, Hamburgi Linnavalitsus ja Eesti Kultuurkapital

  1. Von Economo neurons: A review of the anatomy and functions ...

    African Journals Online (AJOL)

    Von Economo neurons (VENs) are large bipolar neurons found in the anterior cingulate, fronto‑insular, and dorsolateral prefrontal cortices of great apes and the humans. VENs are defined by their thin, elongated cell body, and long dendrites projecting from the apical and basal ends. These neurons are mostly present in ...

  2. Alexander von Humboldt's invention of the natural landscape

    NARCIS (Netherlands)

    Kwa, C.

    2005-01-01

    Landscape took on a new meaning through the new science of plant geography of Alexander von Humboldt (1769-1857). In the seventeenth and eighteenth centuries, "landscape" was foremost a painterly genre. Slowly, painted landscapes came to bear on natural surroundings, but by 1800 it was still not

  3. Spin–momenta entanglement in moving frames: Properties of von ...

    Indian Academy of Sciences (India)

    disentangled) as seen by moving observers, is used to investigate the properties of von Neumann entropy, as a measure of spin–momentum entanglement. To do so, we partition the total Hilbert space into momentum and spin subspaces so that the ...

  4. Seminario para el estudio de carl von clausewitz

    OpenAIRE

    Mesa, Darío

    2008-01-01

    Este es el programa del seminario que a propuesta y bajo la dirección del profesor Darío Mesa se realizó en el Departamento de Sociología, entre marzo y septiembre de 1981 sobre el libro de La Guerra del Militar y Teórico Prusiano Cari Von Clausewitz (178

  5. Seminario para el estudio de Carl Von Clausewitz

    Directory of Open Access Journals (Sweden)

    Darío Mesa

    2000-01-01

    Full Text Available Este es el programa del seminario que a propuesta y bajo la dirección del profesor Darío Mesa se realizó en el Departamento de Sociología, entre marzo y septiembre de 1981 sobre el libro de La Guerra del Militar y Teórico Prusiano Cari Von Clausewitz (178

  6. Molecular characterization of exon 28 of von Willebrand's factor ...

    African Journals Online (AJOL)

    2016-05-12

    May 12, 2016 ... two probable cases among 95 patients with hemophilia A and 11 with hemophilia B between 1980 and 1986, but full investigation and family studies were not performed. In. Nigeria, we have been unable to find documented cases of. Molecular characterization of exon 28 of von Willebrand's factor gene in ...

  7. Systematisierung und Evaluierung von Clustering-Verfahren im Information Retrieval

    OpenAIRE

    Kürsten, Jens

    2006-01-01

    Im Rahmen der vorliegenden Diplomarbeit werden Verfahren zur Clusteranalyse sowie deren Anwendungsmöglichkeiten zur Optimierung der Rechercheergebnisse von Information Retrievalsystemen untersucht. Die Grundlage der vergleichenden Evaluation erfolgversprechender Ansätze zur Clusteranalyse anhand der Domain Specific Monolingual Tasks des Cross-Language Evaluation Forums 2006 bildet die systematische Analyse der in der Forschung etablierten Verfahren zur Clusteranalyse. Die Implementierung ...

  8. Update zum klinischen Einsatz von Inhibitoren mutierter Phosphokinasen beim Melanom.

    Science.gov (United States)

    Cosgarea, Ioana; Ritter, Cathrin; Becker, Jürgen C; Schadendorf, Dirk; Ugurel, Selma

    2017-09-01

    Die Behandlungsstrategie beim metastasierten Melanom hat sich mit der Identifizierung therapeutisch angreifbarer molekularer Zielstrukturen innerhalb zellulärer Signalwege radikal geändert. Durch die Zulassung von Substanzen, die gezielt an den zentralen Schaltmolekülen, den Phosphokinasen, angreifen, können diese Signalwege selektiv abgeschaltet werden. Dies ist insbesondere bei denjenigen Tumoren von Interesse, deren Signalwege durch aktivierende Mutationen der für die Schaltmoleküle kodierenden Gene konstitutiv aktiviert sind. Aktuell ist diese therapeutische Strategie insbesondere für Patienten bedeutsam, deren Melanome eine Mutation im BRAF-Gen aufweisen. Diese Patienten können durch eine Kombinationstherapie aus Inhibitoren der Phosphokinasen BRAF und MEK langfristig mit sehr guter Krankheitskontrolle behandelt werden. Unter dieser Kombinationstherapie wird aktuell ein progressionsfreies Überleben von über zehn Monaten und ein Gesamtüberleben von mehr als zwei Jahren bei guter Lebensqualität erzielt. Da unter längerfristiger Therapie mit Kinaseinhibitoren jedoch bei einem Großteil der Patienten eine Resistenzbildung auftritt, sind aktuelle klinische Therapiestudien auf die Suche nach geeigneten Kombinationspartnern unter Blockierung anderer Signalwege oder unter Aktivierung der T-Zell-vermittelten Immunantwort ausgerichtet. Der vorliegende Übersichtsartikel stellt sowohl die aktuell verfügbaren als auch die in der klinischen Testung befindlichen zukünftigen Optionen der zielgerichteten Therapie des Melanoms dar. © 2017 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.

  9. Theodore von Kármán–Rocket Scientist

    Indian Academy of Sciences (India)

    Home; Journals; Resonance – Journal of Science Education; Volume 10; Issue 8. Theodore von Kármán – Rocket Scientist. Jaywant H Arakeri. Article-in-a-Box Volume 10 Issue 8 August 2005 pp 2-3. Fulltext. Click here to view fulltext PDF. Permanent link: http://www.ias.ac.in/article/fulltext/reso/010/08/0002-0003 ...

  10. Lars von Triers fornyelse af filmen 1984-2014

    DEFF Research Database (Denmark)

    Thomsen, Bodil Marie Stavning

    Bogen undersøger Lars von Triers affektskabende og etisk involverende stil med udgangspunkt i hans værker over en 30-årig periode (1984-2014). Centralt i bogens læsninger står væsentlige analytiske begreber som det haptiske, det affektive, det diagrammatiske og det signaletiske materiale, der med...

  11. C. J. Ebhardi ja Balthasar von Campenhauseni projektid / Toivo Meikar

    Index Scriptorium Estoniae

    Meikar, Toivo, 1947-

    2005-01-01

    Lisatud: C. J. Ebhardi Eesti- ja Liivimaa metsa- ja jahiseaduse projekt (valmis hiljemalt aastaks 1770), lk. 199-222 ; Härra maanõuniku parun von Campenhauseni ettepanekud kõikide metsade nõuetekohaseks korralduseks (4. juuli 1778), lk. 223-233

  12. Myriopoden von Ambon und anderen Inseln Der Banda-See

    NARCIS (Netherlands)

    Attems, C.

    1927-01-01

    Dr. Kopstein auf Ambon hat dem Leidener Museum eine Anzahl von ihm gesammelter Myriopoden gesandt, deren Bestimmung ich übernommen habe. Das Material ist nicht gerade reichhaltig, zumeist nicht gut conserviert und umfasst lange nicht die gesammte dortige Fauna; trotzdem war auch etwas neues darin,

  13. Krahv Alexander von Keyserling / Sirje Kivimäe

    Index Scriptorium Estoniae

    Kivimäe, Sirje, 1947-

    2002-01-01

    Alexander Friedrich Michael Lebrecht Arthur Nicolaus James von Keyserlingi (1815-1891) elust ja teaduslikest ekspeditsioonidest Venemaal ning tegevusest Eestis Raikküla mõisnikuna, loodusteadlasena, Eestimaa rüütelkonna peamehena, eesti talupoegade olukorra parandajana, Tartu õpperingkonna kuraatorina, maanõunikuna, tema pereelust ja lastest

  14. Goethe und Alexander von Humboldt - Bau und Geschichte der Erde

    Directory of Open Access Journals (Sweden)

    Wolf von Engelhardt

    2001-10-01

    Full Text Available Article in German, Abstracts in English and German. An account is given on Goethe's criticism of Alexander von Humboldt's writings: "Ueber den Bau und die Wirkungsart der Vulkane in den verschiedenen Erdstrichen" (Berlin 1823 und "Fragmens de géologie et de climatologie asiatique" (Paris 1831.

  15. Die Eintheilung der versteinerungsführenden Sedimente von Java

    NARCIS (Netherlands)

    Martin, K.

    1899-01-01

    Die als Jungtertiär (m 1, m 2 und m 3) kartirten Sedimente von Java und Madura nehmen ± 38% der ganzen Oberfläche dieser Eilande ein, während sich die posttertiären Ablagerungen mit reichlich 33%, die vulkanischen ¹) Bildungen mit fast 28%, alle vorneogenen Gesteine aber nur mit reichlich 1%

  16. Von Kármán between Aachen and Pasadena

    Science.gov (United States)

    Krause, Egon; Kalkmann, Ulrich

    2013-05-01

    In the Introduction the reader is referred back to the academic ceremonials held after Theodore von Kármán's death in Aachen in May 1963. His work as the first director of the Aerodynamisches Institut (Institute of Aerodynamics) of the RWTH Aachen University of Technology from 1913 on and his initiative to re-establish international cooperation after World War I, resulting in the International Union of Theoretical and Applied Mechanics (IUTAM), are commented on. The following chapter describes von Kármán's relation to his former teacher Ludwig Prandtl. Some of von Kármán's scientific contributions during his time in Aachen are briefly reviewed. Thereafter, his first contacts to the California Institute of Technology are covered. Finally, the scientific and political circumstances, which led to von Kármán's decision to leave Germany in the early thirties, are elucidated in some detail. The English translation of the titles of the Aachen papers is given in Appendix I.

  17. Bemerkungen zu Alexander von Humboldts Russland-Tagebuch

    Directory of Open Access Journals (Sweden)

    Petra Werner

    2008-04-01

    Full Text Available Article in German, Abstracts in English and GermanIn this study the fragment of Alexander von Humboldt’s Siberian personal diary of 1829 is introduced. The evaluation is focussed on Humboldt’s remarks about mineral resources, especially gold and diamonds. Also mentioned are results which Humboldt recorded about geographical, botanical, meteorological, zoological and ethnological questions.

  18. Über die Gyriniden (Col.) von Guiana

    NARCIS (Netherlands)

    Ochs, Georg

    1962-01-01

    Von 14 Taumelkäfer-Arten, die bislang für Guiana nachgewiesen waren, kannte man aus Suriname lediglich 2. Dank der Sammeltätigkeit der Naturwissenschaftlichen Suriname Expedition 1949 in den Nassau Gebergte erhöhen sich diese Zahlen um 3 neue Arten und 2 Unterarten, die hierunter beschrieben werden,

  19. Die Rolle von Osteoklasten bei der arthritischen Knochenerosion

    Directory of Open Access Journals (Sweden)

    Schett G

    2003-01-01

    Full Text Available Knöcherne Erosionen stellen eine wesentliche Ursache für die funktionelle Einschränkung von Patienten mit rheumatoider Arthritis dar. Bis vor kurzem war über den Pathomechanismus dieser Veränderungen nur wenig bekannt. Dieser Review faßt die letzten Erkenntnisse über die Ausbildung einer Knochenschädigung bei entzündlichen Gelenkerkrankungen zusammen und fokussiert insbesondere die Rolle von Osteoklasten. Es wird ein Überblickzu den Hinweisen für eine Präsenz von Osteoklasten im entzündlich veränderten Gelenk gegeben. Weiters werden die Faktoren diskutiert, die die Osteoklastogenese im Rahmen einer Gelenkentzündung aktivieren können. Hier wird im besonderen auf die Rolle von Receptor-Activator of Nuclear Factor Kappa B Ligand (RANKL eingegangen. Im weiteren werden die Auswirkungen einer Osteoklastenhemmung in den verschiedenen Arthritismodellen zusammengefaßt und Möglichkeiten einer therapeutischen Nutzung bei rheumatoider Arthritis diskutiert.

  20. Linckia rosenbergi Von Martens, a Synonym of L. laevigata (L.)

    NARCIS (Netherlands)

    Engel, H.

    1942-01-01

    When Clark (1921) cleared the genus Linckia from superfluous synonyms, he could give no opinion on Linckia rosenbergi Von Martens 1866, p. 63 from Amboina. The unique holotype was in the Berlin Museum and hence not easily accessible to him, the species had not been described again. In his key Clark