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Sample records for rendu-osler-weber disease patient

  1. [Rendu-Osler-Weber disease

    NARCIS (Netherlands)

    Sys, L.M.; Hoogen, F.J.A. van den

    2005-01-01

    Rendu-Osler-Weber disease or hereditary hemorrhagic telangiectasia (HHT) is a multisystem autosomal dominant hereditary disorder. The disorder is manifested by multiple dysplasia of blood vessels of the skin and mucous membranes. This results in recurrent and sometimes severe bleeding, of which

  2. Rendu-Osler-Weber syndrome presenting with pulmonary arteriovenous fistula

    International Nuclear Information System (INIS)

    Halefoglu, A.M.

    2005-01-01

    A pulmonary arteriovenous fistula is an abnormal connection between pulmonary arteries and veins. Patients with Rendu-Osler-Weber syndrome may present with this vascular malformation, which is a typical finding of the disease. Approximately 5-15% of Rendu-Osler-Weber syndrome patients have pulmonary arteriovenous malformations (AVM) and there is usually a family history of AVM in these patients. The malformations are usually located in the lower lobes. In this paper, I describe a 49-year-old male patient with dyspnoea, cough, haemoptysis and epistaxis. Physical examination showed nasal telangiectasias, cyanosis of the lips and nails, and a systolic bruit over the left lung. Chest X-ray revealed a 5-cm mass in the left lower lobe and after magnetic resonance examination, together with 3-D magnetic resonance angiography, it was demonstrated to be a pulmonary arteriovenous fistula. The history of a niece with a similar history of suspected pulmonary arteriovenous fistula led me to consider the possibility of Rendu-Osler-Weber syndrome presenting with a pulmonary arteriovenous fistula. Copyright (2005) Blackwell Science Pty Ltd

  3. Intrahepatic porto-hepatic venous shunts in Rendu-Osler-Weber disease: imaging demonstration

    International Nuclear Information System (INIS)

    Matsumoto, Shunro; Mori, Hiromu; Yamada, Yasunari; Hayashida, Tomoko; Hori, Yuzo; Kiyosue, Hiro

    2004-01-01

    This study describes the imaging features of the intrahepatic portohepatic venous (PHV) shunt, which is a potential cause of portosystemic encephalopathy in Rendu-Osler-Weber disease. Six patients with Rendu-Osler-Weber disease (two men, four women; age range 42-73 years) were retrospectively studied. There were two from one family and three from another family. Of these patients, one was diagnosed with definitive portosystemic encephalopathy because of a psychiatric disorder. We retrospectively reviewed the radiological examinations, including abdominal angiography (n=6), three-phase dynamic helical computed tomography (CT; n=3), and conventional enhanced CT (n=1). In one patient, CT during angiography and CT angioportography were also performed. Evaluation was placed on the imaging features of intrahepatic PHV shunts. On angiography, intrahepatic PHV shunts showing multiple and small shunts <5 mm in diameter in an apparent network were detected in all patents. In two patients, a large shunt with a size of either 7 or 10 mm was associated. These intrahepatic PHV shunts were predominantly distributed in the peripheral parenchyma. Intrahepatic PHV shunts would be characterized by small and multiple shunts in an apparent network on the periphery with or without a large shunt. (orig.)

  4. The Rendu-Osler-Weber Disease Revealed by a Refractory Hypoxemia and Severe Cerebral Fat Embolism

    Directory of Open Access Journals (Sweden)

    Leonel Barreto

    2013-01-01

    Full Text Available The Rendu-Osler-Weber disease is a genetic disease which may lead to severe hemorrhage and less frequently to severe organ dysfunction. We report the case of a 22-year-old patient with no personal medical history who was involved in a motorcycle accident and exhibited severe complications related to large arteriovenous pulmonary shunts during his ICU stay. The patient developed an unexplained severe hypoxemia which was attributed to several arteriovenous shunts of the pulmonary vasculature by a contrast study during a transesophageal echocardiographic examination. The course was subsequently complicated by a prolonged coma associated with hemiplegia which was attributed to a massive paradoxical fat embolism in the setting of an untreated femoral fracture. In addition to hemorrhagic complications which may lead to intractable shock, arteriovenous malformations associated with the Rendu-Osler-Weber disease may involve the pulmonary vasculature and result in unexpected complications, such as hypoxemia or severe cerebral fat embolism in high-risk patients.

  5. Absceso intramedular en paciente con enfermedad de Rendu-Osler-Weber Spinal abscess in a patient with hereditary hemorrhagic telangiectasia

    Directory of Open Access Journals (Sweden)

    Lorena V Maldonado

    2007-12-01

    Full Text Available La enfermedad de Rendu-Osler-Weber es una enfermedad autosómica dominante que se manifiesta por telangiectasias en piel y mucosas y malformaciones arteriovenosas en diversos órganos. El compromiso neurológico puede ocurrir por la presencia de malformaciones arterio-venosas cerebrales, hemorragia intracraneal, y más habitualmente por accidentes cerebrovasculares isquémicos y abscesos cerebrales secundarios a embolia paradojal, en pacientes con malformaciones arteriovenosas pulmonares. El absceso intramedular es una entidad rara, inusual como complicación de la enfermedad de Rendu-Osler-Weber. Presentamos el caso de una mujer de 56 años de edad, con antecedentes familiares de enfermedad de Rendu-Osler- Weber, que ingresó a Terapia Intensiva con cuadriplejia aguda e hipoxemia, en quien se diagnosticó fístulas arteriovenosas pulmonares y un absceso intramedular cervical.Rendu-Osler-Weber syndrome is an autosomal dominant disorder characterized by multiple skin and mucosal telangiectasis and multiorgan arteriovenous malformations. Neurological manifestations may occur because of cerebral arteriovenous malformations, intracranial hemorrhage, and most commonly by ischemic stroke and brain abscess secondary to paradoxical embolization in patients with pulmonary arteriovenous malformations. Intramedullary abscess is a rare, unusual condition, in Rendu-Osler-Weber syndrome. We report the case of a 56 years old woman, with a familial history of Rendu-Osler-Weber syndrome, admitted to intensive care with acute quadriplegia and hypoxemia. Our diagnosis was pulmonary arteriovenous malformations and intramedullary abscess.

  6. Osler-Weber-Rendu syndrome complicated with pulmonary arteriovenous malformation: A case report and review of literatures

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    Kuan-Yu Wang

    2015-01-01

    Full Text Available Osler-Weber-Rendu syndrome is a hereditary disease which is diagnosed by criterions of clinical symptoms and examinations. Here, we report a definite case of Osler-Weber-Rendu syndrome who had epistaxis, skin telangiectasia, and pulmonary arteriovenous malformation (PAVM. His initial clinical presentations were growing pulmonary nodule with cough and occasional chest pain. PAVM with rupture of aneurysm was diagnosed histologically after wedge resection of the nodule in his right lower lung.

  7. [Cerebral ischemia in Rendu-Osler-Weber disease].

    Science.gov (United States)

    Delgado Reyes, S; García de la Rocha, M L; Fernández-Armayor Ajo, V; Sierra Sierra, I; Martín Araguz, A; Moreno Martínez, J M

    2000-02-01

    Neurologic manifestations occur in 8-12% of the patients with Rendu-Osler-Weber disease or hereditary hemorrhagic telangiectasia (HHT), principally infectious and hemorrhagic and, less frequently, ischemic ones. More than a half of these neurologic complications are associated with pulmonary arterio-venous malformations (PAVM). The diagnosis of HHT is based on the presence of telangiectases, hemorrhagic events and a family history with an autosomal dominant pattern. We report a case of a patient diagnosed as having HHT with transient ischemic attacks and a PAVM, which was occluded by the use of embolotherapy. Cerebral ischemia in HHT is related to the existence of a PAVM and results from three mechanisms: 1) secondary poliglobulia and hyperviscosity because of the hypoxemia due to a right-left shunt; 2) communication between the airway and the pulmonary circulation during cough access, which produces gas embolism and hemoptysis; 3) and, finally, paradoxical embolism trough the PAVM, the same mechanism proposed to the infectious neurologic manifestations of the disease. When the diagnosis of HHT is suspected, early search and treatment of PAVM, with embolotherapy or surgery, are necessary in order to avoid respiratory problems (hemoptysis, exertional dyspnea, cianosis, clubbing) and neurologic complications.

  8. Osler-Weber-Rendu disease presenting with hepatocellular carcinoma: radiologic and genetic findings

    OpenAIRE

    Lee, Joo Ho; Lee, Yung Sang; Kim, Pyo Nyun; Lee, Beom Hee; Kim, Gu-Whan; Yoo, Han-Wook; Heo, Nae-Yun; Lim, Young-Suk; Lee, Han Chu; Chung, Young-Hwa; Suh, Dong Jin

    2011-01-01

    This is a case report of a 68-year-old man with hepatocellular carcinoma (HCC) accompanied by hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, and hepatic vascular malformation. HHT is an autosomal dominant disorder of the fibrovascular tissue that is characterized by recurrent epistaxis, mucocutaneous telangiectasias, and visceral arteriovenous malformations. HHT is caused by mutation of the genes involved in the signaling pathway of transforming growth f...

  9. [Pulmonary arteriovenous fistula with Rendu-Osler-Weber disease].

    Science.gov (United States)

    Segawa, Masataka; Touge, Masayoshi; Seki, Kouji; Kusajima, Yoshinori; Saito, Katsuhiko

    2012-09-01

    A 36-year-old man was admitted to our hospital for examination of a nodular shadow in the left lung. Chest 3-dimensional computed tomography (3D-CT) revealed a pulmonary arteriovenous fistula (PAVF) of 21 mm in diameter composed of the feeding artery (A4) and the draining vein (V4) in the left S4. Abdominal enhanced CT revealed multiple hepatic arteriovenous fistula. Brain CT revealed a cavernous hemangioma in right occipital cerebrum. He had a family history, habitual epistaxis, and oral telangiectasia and was diagnosed as Rendu-Osler-Weber disease (hereditary hemorrhagic telangiectasia:HHT). According to his family history, PAVF was likely to be a risk factor of brain infarction and abscess, and the wedge resection of the lingual lobe was performed to remove PAVF.

  10. Bevacizumab for Refractory Gastrointestinal Bleeding in Rendu-Osler-Weber Disease

    Science.gov (United States)

    Bernardes, Carlos; Santos, Sara; Loureiro, Rafaela; Borges, Verónica; Ramos, Gonçalo

    2018-01-01

    Rendu-Osler-Weber disease, also known as hereditary hemorrhagic telangiectasia, is a rare autosomal dominant disorder which is often characterized by recurrent epistaxis, mucocutaneous and gastrointestinal telangiectasias, and visceral arteriovenous malformations. Patients with gastrointestinal involvement can present with a wide spectrum of severity, which may vary from uncomplicated iron deficiency anemia to continuous and refractory bleeding. We present the case of a 62-year-old female, who was admitted with anemia following several episodes of melena, and whose endoscopic examination revealed multiple angiodysplasias in the stomach and small bowel. Despite endoscopic and medical treatment attempts with hormonal agents and octreotide, she developed persistent hemorrhage and severe anemia, requiring frequent red blood cell transfusions. Immediately after initiating bevacizumab (7.5 mg/kg, every 3 weeks), complete cessation of bleeding episodes was observed. Currently, after 1 year of follow-up, she maintained sustained remission without the occurrence of adverse events. PMID:29662934

  11. Pulmonary arteriovenous fistulae associated with Rendu-Osler-Weber disease: report of two cases and review of the literature

    International Nuclear Information System (INIS)

    Souza, Ricardo Pires de; Setubal, Roger; Soares, Ademir Humberto; Gomes, Marcio Rogerio Alcala; Mayo, Suzete Varela

    1996-01-01

    Pulmonary arteriovenous fistula (PAVF) is an uncommon disease with approximately 500 cases report in the literature and its association with Rendu-Osler-Weber (ROW) disease is well established. This study relates two cases of PAVF associated with ROW disease and proceeds a literature review with particularly emphasis on imaging diagnosis modalities used nowadays. (author). 14 refs., 6 figs

  12. Guidelines of the French Society of Otorhinolaryngology (SFORL) (short version). Specific treatment of epistaxis in Rendu-Osler-Weber disease.

    Science.gov (United States)

    Robard, L; Michel, J; Prulière Escabasse, V; Bequignon, E; Vérillaud, B; Malard, O; Crampette, L

    2017-02-01

    The authors present the guidelines of the French Oto-Rhino-Laryngology - Head and Neck Surgery Society (Société Française d'Oto-Rhino-Laryngologie et de Chirurgie de la Face et du Cou: SFORL) concerning specific treatment of epistaxis in Rendu-Osler-Weber disease. A multidisciplinary work-group was entrusted with a review of the scientific literature on the above topic. Guidelines were drawn up, based on the articles retrieved and the group members' individual experience. They were then read over by an editorial group independent of the work group. The final version was established in a coordination meeting. The guidelines were graded as A, B, C or expert opinion, by decreasing level of evidence. Rendu-Osler-Weber disease is diagnosed from the presence of at least three of Curaçao's four criteria. In acute epistaxis, bidigital compression is recommended. Embolization is reserved for resistant epistaxis. Non-resorbable nasal packing and cauterization are contraindicated. Patient education is essential. Telangiectasia of the nasal mucosa can be treated by various local means. In the event of insufficient control, systemic administration of tranexamic acid is recommended. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  13. Intensity-Modulated Radiotherapy for a Rendu-Osler-Weber Disease Patient with Recurrent Severe Epistaxis: A Case Report

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    Maximilian Niyazi

    2010-01-01

    Full Text Available We present a case of a Rendu-Osler-Weber disease patient with recurrent life threatening epistaxis demanding multiple blood transfusions despite of repetitive endoscopic laser and electrocoagulations, endovascular embolisation, septodermoplasty, and long-term intranasal dressings. As alternative treatment modalities repeatedly failed and the patient became almost permanently dependent on nasal dressing, we performed a highly conformal intensity-modulated radiotherapy of the nasal cavity; a total dose of 50 Gy in 2 Gy single fractions was applied. The therapy was very well tolerated, no acute toxicities occurred. Two weeks after the last radiation dose had been applied, the nasal dressing could be removed without problems. Endoscopical control revealed an almost avascular white mucosa without any trace of bleeding spots; previously existing hemangiomas and crusts had disappeared. After a 1-year-follow up, the patient had no significant recurrent epistaxis.

  14. Osler-Weber-Rendu disease presenting with hepatocellular carcinoma: radiologic and genetic findings.

    Science.gov (United States)

    Lee, Joo Ho; Lee, Yung Sang; Kim, Pyo Nyun; Lee, Beom Hee; Kim, Gu Whan; Yoo, Han Wook; Heo, Nae Yun; Lim, Young Suk; Lee, Han Chu; Chung, Young Hwa; Suh, Dong Jin

    2011-12-01

    This is a case report of a 68-year-old man with hepatocellular carcinoma (HCC) accompanied by hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, and hepatic vascular malformation. HHT is an autosomal dominant disorder of the fibrovascular tissue that is characterized by recurrent epistaxis, mucocutaneous telangiectasias, and visceral arteriovenous malformations. HHT is caused by mutation of the genes involved in the signaling pathway of transforming growth factor-β, which plays an important role in the formation of vascular endothelia. Hepatic involvement has been reported as occurring in 30-73% of patients with HHT. However, symptomatic liver involvement is quite rare, and the representative clinical presentations of HHT in hepatic involvement are high-output heart failure, portal hypertension, nodular regenerative hyperplasia, and symptoms of biliary ischemia. Some cases of HCC in association with HHT have been reported, but are very rare. We present herein the characteristic radiologic and genetic findings of HHT that was diagnosed during the evaluation and treatment of HCC.

  15. Embolotherapy for pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)

    DEFF Research Database (Denmark)

    Andersen, P E; Kjeldsen, A D; Oxhøj, H

    1998-01-01

    experienced an improved functional level. One patient experienced severe pleurisy and another a rise in temperature following treatment, but otherwise no symptomatic complications were observed. CONCLUSION: Embolotherapy is a definitive treatment for PAVMs: it is very effective with a high success rate......PURPOSE: To evaluate the clinical results of embolization of pulmonary arteriovenous malformations (PAVMs) in patients with hereditary hemorrhagic telangiectasia (HHT), the Rendu-Osler-Weber syndrome. MATERIAL AND METHODS: Twelve patients in the county of Fyn, Denmark, were treated...... with transcatheter embolization of 20 PAVMs using 12 detachable silicone balloons and 26 steel coils. RESULTS: All PAVMs were completely occluded and we observed a significant rise in PaO2 after treatment and a significant decrease in right-to-left shunt estimated by contrast echocardiography. All patients...

  16. Rendu-Osler-Weber syndrome: what radiologists should know. Literature review and three cases report

    International Nuclear Information System (INIS)

    Agnollitto, Paulo Moraes; Barreto, Andre Rodrigues Facanha; Barbieri, Raul Fernando Pinsetta; Junior, Jorge Elias; Muglia, Valdair Francisco

    2013-01-01

    Rendu-Osler-Weber syndrome or hereditary hemorrhagic telangiectasia is an autosomal dominant vascular disease involving multiple systems whose main pathological change is the presence of abnormal arteriovenous communications. Most common symptoms include skin and mucosal telangiectasias, epistaxis, gastrointestinal, pulmonary and intracerebral bleeding. The key imaging finding is the presence of visceral arteriovenous malformations. The diagnosis is based on clinical criteria and can be confirmed by molecular biology techniques. Treatment includes measures for management of epistaxis, as well as surgical excision, radiotherapy and embolization of arteriovenous malformations, with emphasis on endovascular treatment. The present pictorial essay includes a report of three typical cases of this entity and a literature review. (author)

  17. Rendu-Osler-Weber syndrome: what radiologists should know. Literature review and three cases report; Sindrome de Rendu-Osler-Weber: o que o radiologista precisa saber: revisao da literatura e apresentacao de tres casos

    Energy Technology Data Exchange (ETDEWEB)

    Agnollitto, Paulo Moraes; Barreto, Andre Rodrigues Facanha; Barbieri, Raul Fernando Pinsetta; Junior, Jorge Elias; Muglia, Valdair Francisco, E-mail: agno53@gmail.com [Universidade de Sao Paulo (HC/FMRP/USP), Ribeirao Preto, SP (Brazil). Fac. de Medicina. Hospital das Clinicas

    2013-05-15

    Rendu-Osler-Weber syndrome or hereditary hemorrhagic telangiectasia is an autosomal dominant vascular disease involving multiple systems whose main pathological change is the presence of abnormal arteriovenous communications. Most common symptoms include skin and mucosal telangiectasias, epistaxis, gastrointestinal, pulmonary and intracerebral bleeding. The key imaging finding is the presence of visceral arteriovenous malformations. The diagnosis is based on clinical criteria and can be confirmed by molecular biology techniques. Treatment includes measures for management of epistaxis, as well as surgical excision, radiotherapy and embolization of arteriovenous malformations, with emphasis on endovascular treatment. The present pictorial essay includes a report of three typical cases of this entity and a literature review. (author)

  18. Rendu-Osler-Weber syndrome: what radiologists should know. Literature review and three cases report

    Directory of Open Access Journals (Sweden)

    Paulo Moraes Agnollitto

    2013-06-01

    Full Text Available Rendu-Osler-Weber syndrome or hereditary hemorrhagic telangiectasia is an autosomal dominant vascular disease involving multiple systems whose main pathological change is the presence of abnormal arteriovenous communications. Most common symptoms include skin and mucosal telangiectasias, epistaxis, gastrointestinal, pulmonary and intracerebral bleeding. The key imaging finding is the presence of visceral arteriovenous malformations. The diagnosis is based on clinical criteria and can be confirmed by molecular biology techniques. Treatment includes measures for management of epistaxis, as well as surgical excision, radiotherapy and embolization of arteriovenous malformations, with emphasis on endovascular treatment. The present pictorial essay includes a report of three typical cases of this entity and a literature review.

  19. Imaging findings of arteriovenous malformations involving lung and liver in hereditary hemorrhagic telangiectasia(Osler-weber-rendu disease): two cases report

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    Yi, Jeong Geun; Lee, Joo Hyuk; Seong, Su Ok [Cheongju St. Mary' s Hospital, Cheongju (Korea, Republic of)

    1999-09-01

    Hereditary hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu disease is an autosomal dominant disorder characterized by repeated episodes of bleeding. Multiple telangiectases consisting of thin-walled, dilated vascular channels with arteriovenous communication may involve, for example, mucocutaneous tissue, the gastrointestinal tract, and the liver, lung, and brain. We report the imaging findings of two cases of HHT involving arteriovenous malformation of both the lungs and liver, a rare condition. Chest radiography revealed a round mass, while helical CT showed a feeding artery and draining vein with arteriovenous malformation in the lung. Color Doppler sonography revealed an enlarged and tortuous hepatic artery with high systolic velocity. CT demonstrated an enlarged hepatic artery, arteriovenous shunt, and early draining hepatic vein in the liver. Celiac angiography showed arteriovenous malformation.

  20. Imaging findings of arteriovenous malformations involving lung and liver in hereditary hemorrhagic telangiectasia(Osler-weber-rendu disease): two cases report

    International Nuclear Information System (INIS)

    Yi, Jeong Geun; Lee, Joo Hyuk; Seong, Su Ok

    1999-01-01

    Hereditary hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu disease is an autosomal dominant disorder characterized by repeated episodes of bleeding. Multiple telangiectases consisting of thin-walled, dilated vascular channels with arteriovenous communication may involve, for example, mucocutaneous tissue, the gastrointestinal tract, and the liver, lung, and brain. We report the imaging findings of two cases of HHT involving arteriovenous malformation of both the lungs and liver, a rare condition. Chest radiography revealed a round mass, while helical CT showed a feeding artery and draining vein with arteriovenous malformation in the lung. Color Doppler sonography revealed an enlarged and tortuous hepatic artery with high systolic velocity. CT demonstrated an enlarged hepatic artery, arteriovenous shunt, and early draining hepatic vein in the liver. Celiac angiography showed arteriovenous malformation

  1. Osler's disease; Morbus Osler

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    Ahlhelm, F.; Mueller, U. [Kantonsspital Baden AG, Institut fuer Radiologie, Baden (Switzerland); Lieb, J. [Universitaetsspital Basel, Klinik fuer Radiologie und Nuklearmedizin, Basel (Switzerland); Schneider, G. [Universitaetskliniken des Saarlandes, Klinik fuer Diagnostische und Interventionelle Radiologie, Homburg/Saar (Germany); Ulmer, S. [Medizinisch-Radiologisches Institut, Zuerich (Switzerland)

    2013-12-15

    Osler's disease, also known as hereditary hemorrhagic telangiectasia (HHT) and Osler-Weber-Rendu syndrome, is an autosomal dominant disorder leading to abnormal blood vessel formation in the skin, mucous membranes and often in organs, such as the lungs, liver and brain (arteriovenous malformations AVM). Various types are known. Patients may present with epistaxis. Teleangiectasia can be identified by visual inspection during physical examination of the skin or oral cavity or by endoscopy. Diagnosis is made after clinical examination and genetic testing based on the Curacao criteria. Modern imaging modalities, such as computed tomography (CT) or magnetic resonance imaging (MRI) have become more important as they can depict the AVMs. Pulmonary AVMs can be depicted in CT imaging even without the use of a contrast agent while other locations including the central nervous system (CNS) usually require administration of contrast agents. Knowledge of possible clinical manifestations in various organs, possible complications and typical radiological presentation is mandatory to enable adequate therapy of these patients. Interventional procedures are becoming increasingly more important in the treatment of HHT patients. (orig.) [German] Der Mobus Osler (Synonyme: hereditaere haemorrhagische Teleangiektasie [HHT], Morbus Rendu-Osler-Weber) ist eine Multisystemerkrankung und gehoert zur Gruppe der vaskulaeren haemorrhagischen Erkrankungen. Bei der autosomal dominanten Erkrankung, die zu den haeufigsten Phakomatosen zaehlt, kann je nach Gendefekt zwischen verschiedenen Formen, die zu einer Stoerung der Blutgefaessbildung fuehren, unterschieden werden. Neben der genetischen Diagnostik und der klinischen Untersuchung sind bildgebende Verfahren entscheidend fuer die Diagnose. Klinisch stehen die Epistaxis, mukokutane Teleangiektasien und viszerale arteriovenoese Malformationen (AVM) v. a. in Lunge, Leber und Hirn sowie die Folgen dieser Gefaesspathologien wie z. B

  2. Osler's disease

    International Nuclear Information System (INIS)

    Ahlhelm, F.; Mueller, U.; Lieb, J.; Schneider, G.; Ulmer, S.

    2013-01-01

    Osler's disease, also known as hereditary hemorrhagic telangiectasia (HHT) and Osler-Weber-Rendu syndrome, is an autosomal dominant disorder leading to abnormal blood vessel formation in the skin, mucous membranes and often in organs, such as the lungs, liver and brain (arteriovenous malformations AVM). Various types are known. Patients may present with epistaxis. Teleangiectasia can be identified by visual inspection during physical examination of the skin or oral cavity or by endoscopy. Diagnosis is made after clinical examination and genetic testing based on the Curacao criteria. Modern imaging modalities, such as computed tomography (CT) or magnetic resonance imaging (MRI) have become more important as they can depict the AVMs. Pulmonary AVMs can be depicted in CT imaging even without the use of a contrast agent while other locations including the central nervous system (CNS) usually require administration of contrast agents. Knowledge of possible clinical manifestations in various organs, possible complications and typical radiological presentation is mandatory to enable adequate therapy of these patients. Interventional procedures are becoming increasingly more important in the treatment of HHT patients. (orig.) [de

  3. Complications from office sclerotherapy for epistaxis due to hereditary hemorrhagic telangiectasia (HHT or Osler-Weber-Rendu).

    Science.gov (United States)

    Hanks, John E; Hunter, David; Goding, George S; Boyer, Holly C

    2014-05-01

    The aim of this study was to identify and evaluate adverse clinical outcomes following office-based sclerotherapy using sodium tetradecyl sulfate (STS) for epistaxis due to hereditary hemorrhagic telangiectasias (HHT or Osler-Weber-Rendu). A retrospective chart review of 36 adult patients treated with STS sclerotherapy for severe and/or recurrent epistaxis due to HHT was performed. A total of 153 separate treatment sessions were analyzed. Each patient underwent an average of 4.3 sessions with an average of 7 intralesional injections per session. Bleeding during the procedure was experienced by 8 patients with a maximum reported blood loss of 200 mL in 1 patient, but less than 50 mL in all others. Seven patients reported some postinjection pain, which included nasal, cheek, and eye pain. Nasal congestion, sneezing, and vasovagal responses were each noted to occur 2 times. No complications of postprocedural visual loss, deep venous thrombosis/pulmonary embolus, transient ischemic attack (TIA)/stroke, or anaphylaxis were encountered. Conventional therapies used in the management of HHT-related epistaxis, such as laser coagulation, septodermoplasty, selective arterial embolization, and Young's occlusion each have specific associated complications, including worsened epistaxis, septal perforation, foul odor, nasal crusting, and compromised nasal breathing. STS is a safe office-based treatment option for HHT-mediated epistaxis that is associated with exceedingly few of the aforementioned serious sequelae. © 2014 ARS-AAOA, LLC.

  4. Specific proof of various stages of Osler's disease of the brain via high field magnetic resonance tomography (1. 5 Tesla)

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    Billet, F; Bluemm, R G; Beyer, H K

    1988-08-01

    MR is a sensitive noninvasive examination method for diagnosing parenchymatous cryptic arteriovenous malformations and sequels of cerebral haemorrhage. In a patient with recurring nosebleed and brain stem syndrome eleven so-called cryptic arteriovenous malformations or their haemorrhage sequels were diagnosed via magnetic resonance tomography. Basing on these specific findings, nosebleeding, and a positive family anamnesis, the findings could be classified as belonging to manifestations of Osler's disease (hereditary haemorrhagic telangiectasia). This rare disease is characterised by a triad of signs: telangiectasias, recurring bleeding, and heredity. In this article the specific MR image is compared with the CT pattern and the results are discussed against the background of literature on Osler's disease which is also known as Rendu-Osler-Weber disease.

  5. Specific proof of various stages of Osler's disease of the brain via high field magnetic resonance tomography (1. 5 Tesla)

    Energy Technology Data Exchange (ETDEWEB)

    Billet, F.; Bluemm, R.G.; Beyer, H.K.

    1988-08-01

    MR is a sensitive noninvasive examination method for diagnosing parenchymatous cryptic arteriovenous malformations and sequels of cerebral haemorrhage. In a patient with recurring nosebleed and brain stem syndrome eleven so-called cryptic arteriovenous malformations or their haemorrhage sequels were diagnosed via magnetic resonance tomography. Basing on these specific findings, nosebleeding, and a positive family anamnesis, the findings could be classified as belonging to manifestations of Osler's disease (hereditary haemorrhagic telangiectasia). This rare disease is characterised by a triad of signs: telangiectasias, recurring bleeding, and heredity. In this article the specific MR image is compared with the CT pattern and the results are discussed against the background of literature on Osler's disease which is also known as Rendu-Osler-Weber disease.

  6. Étude épidémiologique de la maladie de Rendu-Osler en France : répartition géographique et prévalence

    OpenAIRE

    J.m. Robert; Guy Brunet; Henri Plauchu; Alain Bideau

    1989-01-01

    Bideau Alain, Plauchu Henri, Brunet Guy and Robert J.M. ? Epidemiological study of Rendu-Osler's disease in France : geographical distribution and frequency. This study of the geographical distribution of Rendu-Osler's disease is based on a postal questionnaire involving over 23,000 doctors from 52 French départements. The impetus for this survey, which was later extended to other regions, was originally provided by the observation that there was a concentration of patients in the Rhône-Alpes...

  7. Manuseio anestésico de paciente portador de telangiectasia hemorrágica hereditária (síndrome de Rendu-Osler-Weber: relato de caso Manoseo anestésico de paciente portador de telangiectasia hemorrágica hereditaria (síndrome de Rendu - Osler - Weber: relato de caso Anesthetic management of a patient with hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome: case report

    Directory of Open Access Journals (Sweden)

    Alexandre Palmeira Goulart

    2009-02-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: A telangiectasia hemorrágica hereditária (THH, também conhecida como síndrome de Rendu-Osler-Weber, é uma doença autossômica dominante, caracterizada por displasia vascular mucocutânea e visceral associada a episódios freqüentes de epistaxe e sangramentos gastrintestinais. O objetivo do presente relato foi descrever a anestesia em paciente portador dessa síndrome. RELATO DO CASO: Paciente do sexo masculino, 25 anos, submetido à correção cirúrgica de fratura de órbita esquerda. Portador da tríade epistaxe recorrente, histórico familiar e telangectasia, apresentava diagnóstico de THH. Durante a investigação pré-operatória não foram encontradas malformações vasculares pulmonares, encefálicas ou do trato gastrintestinal. O paciente foi submetido à anestesia venosa total, uma hora após a administração profilática de antifibrinolítico. O sangramento foi considerado normal para a operação proposta e não houve instabilidade hemodinâmica ou necessidade de transfusão sanguínea perioperatória. A extubação ocorreu na sala cirúrgica e o paciente foi liberado para o quarto após 60 minutos e a alta hospitalar foi após 24 horas. CONCLUSÕES: A THH é uma doença autossômica dominante que provoca displasia vascular musculocutânea e visceral. Pode haver perda sanguínea perioperatória acima da esperada para pacientes portadores dessa síndrome. Como o sangramento não é resultado de defeito na cascata de coagulação mas da exposição cirúrgica da estrutura vascular malformada, a conduta perioperatória inclui o emprego de antifibrinolíticos, a realização de hemostasia adequada e da hipotensão arterial induzida, quando não houver contra-indicação. A avaliação pré-anestésica deve incluir a pesquisa de malformações vasculares encefálicas, pulmonares ou do trato gastrintestinal.JUSTIFICATIVA Y OBJETIVOS: La telangiectasia hemorrágica hereditaria (THH, también conocida como

  8. Síndrome de Rendu-Osler-Weber: presentación de un caso clínico

    Directory of Open Access Journals (Sweden)

    Freyli Bustamante

    2016-08-01

    Full Text Available El síndrome de Rendu-Osler-Weber (SROW, conocido también como Telangiectasia Hemorrágica Hereditaria (THH, es un desorden vascular infrecuente. Se trata de una alteración vascular displásica multisistémica de carácter autosómico dominante, con diversas complicaciones, siendo las malformaciones arterio-venosas (MAV responsables de la morbi-mortalidad de los pacientes cuando no son diagnosticadas ni tratadas a tiempo. Muchas veces este síndrome presenta síntomas sutiles, por lo que puede pasar desapercibida clínicamente. Presentamos un caso de una paciente femenina de 51 años, con historia de epistaxis frecuentes quien acude a nuestro hospital por presentar cefalea de fuerte intensidad, meningismo y pérdida de la consciencia. La tomografía axial computada (TAC de cráneo identificó hemorragia sub aracnoidea (HSA difusa. La angiografía cerebral permitió identificar aneurisma arteria comunicante anterior (AcoA roto que fue tratado por vía endovascular, sin eventualidades. Además, se observó telangectasias mucosas nasales múltiples y fistula arterio-venosa (FAV dural occipital izquierda. Estudios ulteriores permitieron identificar MAV pulmonares múltiples. Se confirmó el diagnóstico de SROW, con criterios de diagnóstico vigentes (criterios de Curazao. Durante los tres meses posteriores a su egreso el paciente presentó hemoptisis severa y disnea, que no pudo ser tratada a tiempo por lo que falleció.

  9. High-output cardiac failure secondary to multiple vascular malformations in the liver: case report

    International Nuclear Information System (INIS)

    Spaner, S.; Demeter, S.; Lien, D.; Shapiro, J.; McCarthy, M.; Raymond, G.

    2001-01-01

    High-output cardiac failure is associated with several systemic illnesses, including hyperthyroidism, thiamine deficiency, severe anemia, multiple myeloma, Paget's disease of bone and Osler-Weber-Rendu syndrome. We present an unusual case of a woman with high-output cardiac failure as a result of multiple arteriovenous fistulas in the liver, most likely representing an unusual variant of Osler-Weber-Rendu syndrome (i.e., no other telangiectasias or a family history of vascular malformations was demonstrated). (author)

  10. Falla cardíaca de alto débito por fístulas arteriovenosas sistémicas en paciente con síndrome de Osler-Weber-Rendu

    Directory of Open Access Journals (Sweden)

    Juan M Senior

    2013-09-01

    Full Text Available Paciente de 65 años, con historia de epistaxis a repetición, sin otros antecedentes patológicos de importancia, quien presentó síntomas de un año de evolución consistentes en disnea de esfuerzo progresiva hasta el reposo, acompañado de ortopnea y edemas que iniciaron en miembros inferiores y progresaron hasta la anasarca. Al examen clínico llamó la atención ingurgitación yugular, hepatomegalia, ascitis y edemas periféricos. La piel presentó telangiectasias en extremidades y mucosas. Se realizaron estudios que reportaron falla cardiaca con severa dilatación de las cavidades derechas, con función sistólica del ventrículo izquierdo conservada. Se demostraron fístulas arteriovenosas en hígado y pulmón. Se estableció el diagnóstico de síndrome de Osler-Weber-Rendu o telangiectasia hemorrágica hereditaria (THH. Se propuso cierre de fístulas por vía percutánea y se planteó posible trasplante hepático como tratamiento; sin embargo, el paciente tuvo una evolución tórpida, presentó fibrilación auricular y embolismo al sistema nervioso central y falleció por enfermedad cerebrovascular.

  11. Clinical features of Hereditary Haemorrhagic Telangiectasia

    NARCIS (Netherlands)

    Hosman, A.E.

    2017-01-01

    Hereditary Haemorrhagic Telangiectasia (HHT), also known as Rendu-Osler-Weber disease (ROW), is an autosomal dominant disease with multi-systemic vascular dysplasia characterized by mucocutaneous telangiectasia, arteriovenous malformations and recurrent spontaneous epistaxis (nosebleeds). Most cases

  12. Osler-Weber-Rendu syndrome

    Science.gov (United States)

    ... ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 98. McDonald J, Wooderchak-Donahue W, VanSant Webb C, Whitehead ... M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health ...

  13. Hereditary hemorrhagic telangiectasia clinical and molecular genetics

    NARCIS (Netherlands)

    Letteboer, T.G.W.

    2010-01-01

    Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber (ROW) syndrome is an autosomal dominant disease characterized by vascular malformations in multiple organ systems. HHT has an age-related penetrance and variable clinical expression. The clinical symptoms are caused by direct

  14. Contact endoscopy of the nose in patients with Rendu-Osler-Weber syndrome.

    Science.gov (United States)

    Folz, B J; Werner, J A

    2007-03-01

    Teleangiectases are the source of hemorrhage in many HHT patients. Most frequent site of bleeding is the nose and more than 90% of all individuals with HHT suffer from recurrent epistaxis. Despite all efforts, treatment of epistaxis in HHT continues to be a problem for many otorhinolaryngologists, who can alleviate recurrent nosebleeds by Septodermoplasty or laser therapy, but rarely can stop nasal hemorrhages permanently. Recurrence is almost inevitable, but the mechanisms of recurrence are not fully understood. Prior to routine Nd:YAG laser therapy of nasal telangiectases the nasal mucosa of 17 patients with HHT according to the clinical diagnostic criteria of the HHT Foundation International was examined with a 0 degrees contact rhinoscope in areas with clinically visible telangiectases as well as in clinically normal mucosa. The digitally recorded images were compared to findings of a group of five healthy volunteers and the findings of five patients with polypoid sinusitis. Visualization of subepithelial vessels was feasible in all individuals of the study group as well as the control groups. Dilated vascular loops and tortuous vessels could be found in the study groups as well as in the control group, but the overall density of telangiectatic vessels was on an average higher in the HHT group. The process of vessel dilatation and tortuous configuration seemed to progress with age. Contact endoscopy allows the investigation of the angioarchitecture of capillaries of the nasal mucosa in vivo. This observation may be of significance for studies of nasal diseases, which are accompanied by epistaxis. With this regard it seems to be of special interest for studies of HHT.

  15. Pulmonary arteriovenous fistulas

    International Nuclear Information System (INIS)

    Medeiros Sobrinho, J.H. de; Kambara, A.M.

    1987-01-01

    Six cases of pulmonary arteriovenous fistulas, isolated, without hemorrhagic hereditary telangiectasia (Rendu-Osler-Weber Symdrome) are reported emphasizing the radiographic, tomographic and angiographic examinations, (M.A.C.) [pt

  16. Brain abscesses and hereditary hemorrhagic telangiectasia

    International Nuclear Information System (INIS)

    Vives, Daniel A.; Bauni, Carlos E.; Mendoza, Monica E.

    2003-01-01

    Rendu-Osler-Weber disease or Hereditary Hemorrhagic Telangiectasia (HHT) is a generalized familial angiodysplastic disorder. The neurological manifestations of this entity are due to Central Nervous System vascular lesions or to complications of other visceral lesions such as pulmonary arteriovenous fistulae. This report describes two patients (males, 40 and 61 years old), with brain abscesses associated with HHT. The CT, MRI and Angiographic findings as well as the therapeutic approach are analyzed. Patients with brain abscess of unknown origin must be evaluated for the presence of lung vascular malformation in association with HHT. (author)

  17. Neuro image in neuroectodermal disorders. Part III: angiomatous and melanotic syndromes

    International Nuclear Information System (INIS)

    Marti-Bonmati, L.; Menor, F.; Poyatos, C.; Cortina, H.; Esteban, M.J.; Vilar, J.

    1994-01-01

    Twenty-eight consecutive patients affected by these rare angiomatous melanotic neuroectodermal disorders are assessed. The diagnostics value and clinical correlation of neuroimaging methods, both CT and MR, are established. Patients with Sturge-Weber syndrome (15 cases), Klippel-Trenaunay syndrome (1 case), Rendu-Osler disease (3 cases), multiple hemangiomatosis (4 cases), von Hippel-Lindau syndrome (3 cases), neuro cutaneous melanosis (1 case) and hypo melanosis of Ito (1 case) are included. In vascular phacomatosis, neuroimaging methods usually contribute to the positive diagnosis. In melanotic disorders, the neuroradiological findings most often are unspecific and do not contribute to the diagnosis of the disease

  18. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)

    DEFF Research Database (Denmark)

    Shovlin, C L; Guttmacher, A E; Buscarini, E

    2000-01-01

    Hereditary Hemorrhagic Telangiectasia (HHT) is easily recognized in individuals displaying the classical triad of epistaxis, telangiectasia, and a suitable family history, but the disease is more difficult to diagnosis in many patients. Serious consequences may result if visceral arteriovenous ma...... in this disorder. These criteria may be refined as molecular diagnostic tests become available in the next few years....... of the HHT Foundation International, Inc., we present consensus clinical diagnostic criteria. The four criteria (epistaxes, telangiectasia, visceral lesions and an appropriate family history) are carefully delineated. The HHT diagnosis is definite if three criteria are present. A diagnosis of HHT cannot...

  19. Telangiectasia hemorrágica hereditária: ácido tranexâmico no tratamento de úlcera plantar Hereditary hemorrhagic telangiectasia: tranexamic acid for plantar ulcer

    Directory of Open Access Journals (Sweden)

    Gabriella Corrêa de Albuquerque

    2005-12-01

    Full Text Available Relato de um caso de úlcera plantar por fístula arteriovenosa em paciente portador de telangiectasia hemorrágica hereditária ou doença de Rendu-Osler-Weber tratado com ácido tranexâmico. Este fármaco é utilizado para tratamento de epistaxe, referindo-se o principal achado deste artigo ao uso eficaz desse medicamento na terapia de úlceras plantares hemorrágicas. São descritos os aspectos fisiopatológicos e clínicos da doença e as propriedades antifibrinolíticas do ácido tranexâmico. Este foi bem tolerado e apresentou evidências de eficácia na utilização para controle do sangramento e cicatrização da úlcera.Case report of one patient with Hereditary Hemorrhagic Telangiectasia, also known as Rendu-Osler-Weber syndrome, treated with Tranexamic Acid for arteriovenous plantar ulcer. This drug has proved effective in controlling epistaxis, but the main point of this report is to expose the success use of this medication in the therapy of skin bleeding ulcer. The pathophysiologic and clinical features of the disease are reviewed and also the pharmacological aspects of the antifibrinolytic drugs. This drug was well tolerated by the patient and show evidence of good activity in the bleeding and healed the ulcer.

  20. Pulmonary arterio-venous micro fistulae - Diagnostic

    International Nuclear Information System (INIS)

    Ebram, J.C.

    1989-01-01

    Four patients with pulmonary arterio-venous micro-fistulae - of which two were male (50%) - the ages varying from 10 to 43 (X sup(∼) = 22,7), were studied at the Cardiology Centre of the 6th Ward of Santa Casa da Misericordia Hospital in Rio de Janeiro. They were all basically suffering from Manson's Schistosomiasis, the hepato-splenic form in 3 cases (75%) and the Rendu Osler Weber disease with juvenile cirrhosis in 1 case (25%). All four of them had portal hypertension. The individual cases were clinically evaluate with X-rays, scintillographic and hemodynamic tests. (author)

  1. Neurologic Manifestation as Initial Presentation in a Case of Hereditary Haemorrhagic Telangiectasia

    Directory of Open Access Journals (Sweden)

    Yeow Kwan Teo

    2010-01-01

    Full Text Available Hereditary Haemorrhagic Telangiectasia (HHT, or Osler-Weber-Rendu syndrome is an uncommon autosomal dominant multi-organ condition of vascular dysplasias. We describe a 19 year old Indian female who presented with cerebral abscess secondary to paradoxical emboli from pulmonary arteriovenous malformations (PAVMs associated with HHT. Cerebral, pulmonary, hepatic and gastrointestinal involvement can be life-threatening and it is important to have lifelong follow-ups on these patients.

  2. Carefully calibrated microsphere embolization of tumors and AVMs

    International Nuclear Information System (INIS)

    Laurent, A.; Wassef, M.; Beaujeux, R.; Hodes, J.E.; Gobin, P.Y.; Schenker, C.; Bouchez, B.; Brette, M.D.; Aymard, A.; Cophignon, J.; Merland, J.J.

    1990-01-01

    This paper evaluates the efficacy of a new type of uniformly calibrated microsphere for embolization of various pathologies. Fourteen tumors (five intracranial, eight extracranial, one spine), and seven facial arteriovenous malformations (AVMs) (nose, lip, and scalp) were embolized preoperatively. Angiographic, surgical devascularization, and histologic findings were correlated with the spheres' size. In the second part of the study, spinal cord (n = 22) and intracerbral (n = 14) AVM, Weber-Osler-Rendu disease (n = 2), and epistaxis (n = 1) embolization was angiographically evaluated

  3. Osler-Weber-Rendu syndrome: Case report | Akintayo | Nigerian ...

    African Journals Online (AJOL)

    It is an under-recognized disorder that results from multisystem vascular dysplasia. It is characterized by telangiectasias and arteriovenous malformations (AVMs) of skin, mucosa and viscera. Most affected persons present with recurrent epistaxis which increases in frequency and severity with age. We report a 35 year old ...

  4. Lung involvement in Osler's disease and cerebral complications

    Energy Technology Data Exchange (ETDEWEB)

    Piepgras, U.; Sielecki, S.

    1986-01-01

    About 50% of patients with Morbus Osler also have arteriovenous lung malformations. The wall-insufficiency mural inadequacy of the malformated vessels is due to the secondary infectious cerebral and meningeal complications which frequently occur in the disease. If a brain abscess is diagnosed one has first to take into consideration in a.-v. malformation of the lungs as possible source of the infection. (orig.).

  5. Hepatic telangiectasia in Osler's disease treated with arterial embolization

    Energy Technology Data Exchange (ETDEWEB)

    Goethlin, J H; Nordgard, K; Jonsson, K; Nyman, U

    1982-02-01

    Hepatic hereditary telangiectasia in 2 females was treated with hepatic artery embolization. In one patient both peripheral and central arterial occlusion was performed; the patient died of massive gastro-intestinal bleeding 2 months later. Autopsy showed extensive regions with necrosis in the right liver lobe. In the next patient only central occlusion of the right hepatic artery was performed and the arterio-venous shunting in the left liver lobe left remaining. After a long reconvalescence period the patient recovered completely. It is advocated to centrally occlude only that hepatic artery supplying the most affected parts of the liver in Osler's disease. Thus extensive necrosis with possible ensuing death may be avoided.

  6. Abdominal vascular syndromes: characteristic imaging findings

    International Nuclear Information System (INIS)

    Cardarelli-Leite, Leandro; Velloni, Fernanda Garozzo; Salvadori, Priscila Silveira; Lemos, Marcelo Delboni; D'Ippolito, Giuseppe

    2016-01-01

    Abdominal vascular syndromes are rare diseases. Although such syndromes vary widely in terms of symptoms and etiologies, certain imaging findings are characteristic. Depending on their etiology, they can be categorized as congenital - including blue rubber bleb nevus syndrome, Klippel-Trenaunay syndrome, and hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) - compressive - including 'nutcracker' syndrome, median arcuate ligament syndrome, Cockett syndrome (also known as May-Thurner syndrome), and superior mesenteric artery syndrome. In this article, we aimed to illustrate imaging findings that are characteristic of these syndromes, through studies conducted at our institution, as well as to perform a brief review of the literature on this topic. (author)

  7. Abdominal vascular syndromes: characteristic imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Cardarelli-Leite, Leandro; Velloni, Fernanda Garozzo; Salvadori, Priscila Silveira; Lemos, Marcelo Delboni; D' Ippolito, Giuseppe, E-mail: leandrocleite@gmail.com [Universidade Federal de Sao Paulo (EPM/UNIFESP), Sao Paulo, SP (Brazil). Escola Paulista de Mediciana. Departmento de Diagnostico por Imagem

    2016-07-15

    Abdominal vascular syndromes are rare diseases. Although such syndromes vary widely in terms of symptoms and etiologies, certain imaging findings are characteristic. Depending on their etiology, they can be categorized as congenital - including blue rubber bleb nevus syndrome, Klippel-Trenaunay syndrome, and hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) - compressive - including 'nutcracker' syndrome, median arcuate ligament syndrome, Cockett syndrome (also known as May-Thurner syndrome), and superior mesenteric artery syndrome. In this article, we aimed to illustrate imaging findings that are characteristic of these syndromes, through studies conducted at our institution, as well as to perform a brief review of the literature on this topic. (author)

  8. Manganese accumulation in the brain: MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Uchino, A.; Nomiyama, K.; Takase, Y.; Nakazono, T.; Nojiri, J.; Kudo, S. [Saga Medical School, Department of Radiology, Saga (Japan); Noguchi, T. [Kyushu University, Department of Clinical Radiology, Graduate School of Medicine, Fukuoka (Japan)

    2007-09-15

    Manganese (Mn) accumulation in the brain is detected as symmetrical high signal intensity in the globus pallidi on T1-weighted MR images without an abnormal signal on T2-weighted images. In this review, we present several cases of Mn accumulation in the brain due to acquired or congenital diseases of the abdomen including hepatic cirrhosis with a portosystemic shunt, congenital biliary atresia, primary biliary cirrhosis, congenital intrahepatic portosystemic shunt without liver dysfunction, Rendu-Osler-Weber syndrome with a diffuse intrahepatic portosystemic shunt, and patent ductus venosus. Other causes of Mn accumulation in the brain are Mn overload from total parenteral nutrition and welding-related Mn intoxication. (orig.)

  9. Interventional treatment of pulmonary arteriovenous malformations

    DEFF Research Database (Denmark)

    Andersen, Poul Erik; Kjeldsen, Anette Drøhse

    2010-01-01

    Pulmonary arteriovenous malformations (PAVM) are congenital vascular communications in the lungs. They act as right to left shunts so that the blood running through these malformations is not oxygenated or filtered. These patients are typically hypoxaemic with exercise intolerance and are at high...... risk of paradoxical emboli to the brain and other organs. These malformations are most commonly seen in hereditary haemorrhagic telangiectasia (HHT) (Mb. Osler-Weber-Rendu syndrome). Nowadays, the generally accepted treatment strategy of first choice is embolization of the afferent arteries...... the functional level. Embolization is a well-established method of treating PAVM, with a significant effect on oxygenation of the blood. Screening for PAVM in patients at risk is recommended, especially in patients with HHT....

  10. Imaging of Hereditary Hemorrhagic Telangiectasia

    International Nuclear Information System (INIS)

    Carette, Marie-France; Nedelcu, Cosmina; Tassart, Marc; Grange, Jean-Didier; Wislez, Marie; Khalil, Antoine

    2009-01-01

    This pictorial review is based on our experience of the follow-up of 120 patients at our multidisciplinary center for hereditary hemorrhagic telangiectasia (HHT). Rendu-Osler-Weber disease or HHT is a multiorgan autosomal dominant disorder with high penetrance, characterized by epistaxis, mucocutaneous telangiectasis, and visceral arteriovenous malformations (AVMs). The research on gene mutations is fundamental and family screening by clinical examination, chest X-ray, research of pulmonary shunting, and abdominal color Doppler sonography is absolutely necessary. The angioarchitecture of pulmonary AVMs can be studied by unenhanced multidetector computed tomography; however, all other explorations of liver, digestive bowels, or brain require administration of contrast media. Magnetic resonance angiography is helpful for central nervous system screening, in particular for the spinal cord, but also for pulmonary, hepatic, and pelvic AVMs. Knowledge of the multiorgan involvement of HHT, mechanism of complications, and radiologic findings is fundamental for the correct management of these patients.

  11. Osler on migraine.

    Science.gov (United States)

    Boes, Christopher J

    2015-03-01

    William Osler's 1892 textbook The Principles and Practice of Medicine became the dominant medical text in the English-speaking world. Osler was labeled a therapeutic nihilist by some. The topic of migraine, including treatment, was succinctly covered in his text. The objectives of this study were to review Osler's thoughts on migraine, and outline his therapeutic recommendations. Preventively Osler mentioned bromides, iron, arsenic, nitroglycerin, and cannabis. Acutely he recommended coffee, chloroform, cannabis, antipyrin, antifebrin, phenacetin, caffeine citrate, nux vomica, or ergot. He thought cannabis was the most satisfactory remedy. Osler was not a therapeutic nihilist when it came to migraine, and his treatment recommendations were similar to other writers of his time. Osler did not draw upon his personal experience to contribute new knowledge about migraine. Regardless, given the popularity and clarity of his text, Osler's well-summarized migraine chapter had a great influence on practicing physicians.

  12. Transcatheter embolization therapy of the gastrointestinal hemorrhage

    International Nuclear Information System (INIS)

    Sim, Jae In; Park, Auh Whan; Ryeom, Hun Kyu; Kim, Yong Joo

    1994-01-01

    To evaluate the effectiveness of transcatheter embolization for the treatment of massive gastrointestinal arterial bleeding. The study was based on retrospective analysis of twelve cases(8 men, 4 woman) including two patients with hemobilia in which transcatheter embolization was attempted for the control of massive gastrointestinal bleeding from March 1987 to October 1993. Clinical diagnoses of these patients were peptic ulcer(5), pseudoaneurysm formation(3) following percutaneous transhepatic biliary drainage or traffic accident, stomach cancer(1), typhoid fever(1), duodenal leiomyoma(1) and Osler-Weber-Rendu disease (1). Embolized vessels are as follows: gastroduodenal artery(6), left gastric artery(2), ileocolic artery(2), and hepatic artery(2). Embolization was effective in immediate control of bleeding in all patients. Although five of the six patients who had undergone embolization of the gastroduodenal artery developed rebleeding within 24 hour, only 2 required surgery and none showed serious complication. Embolization therapy is safe and effective initial treatment of choice for life-threatening massive gastrointestinal bleeding

  13. Transcatheter embolization therapy of the gastrointestinal hemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    Sim, Jae In; Park, Auh Whan; Ryeom, Hun Kyu; Kim, Yong Joo [Kyungpook National University Hospital, Daegu (Korea, Republic of)

    1994-05-15

    To evaluate the effectiveness of transcatheter embolization for the treatment of massive gastrointestinal arterial bleeding. The study was based on retrospective analysis of twelve cases(8 men, 4 woman) including two patients with hemobilia in which transcatheter embolization was attempted for the control of massive gastrointestinal bleeding from March 1987 to October 1993. Clinical diagnoses of these patients were peptic ulcer(5), pseudoaneurysm formation(3) following percutaneous transhepatic biliary drainage or traffic accident, stomach cancer(1), typhoid fever(1), duodenal leiomyoma(1) and Osler-Weber-Rendu disease (1). Embolized vessels are as follows: gastroduodenal artery(6), left gastric artery(2), ileocolic artery(2), and hepatic artery(2). Embolization was effective in immediate control of bleeding in all patients. Although five of the six patients who had undergone embolization of the gastroduodenal artery developed rebleeding within 24 hour, only 2 required surgery and none showed serious complication. Embolization therapy is safe and effective initial treatment of choice for life-threatening massive gastrointestinal bleeding.

  14. Follow-up of Thalidomide treatment in patients with Hereditary Haemorrhagic Telangiectasia.

    Science.gov (United States)

    Hosman, A; Westermann, C J J; Snijder, R; Disch, F; Mummery, C L; Mager, J J

    2015-12-01

    Patients with a hereditary vascular disorder called Rendu-Osler-Weber syndrome (Hereditary Haemorrhagic Telangiectasia, HHT) haemorrhage easily due to weak-walled vessels. Haemorrhage in lungs or brain can be fatal but patients suffer most from chronic and prolonged nosebleeds (epistaxis), the frequency and intensity of which increases with age. Several years ago, it was discovered serendipitously that the drug Thalidomide had beneficial effects on the disease symptoms in several of a small group of HHT patients: epistaxis and the incidence of anaemia were reduced and patients required fewer blood transfusions. In addition, they reported a better quality of life. However, Thalidomide has significant negative side effects, including neuropathy and fatigue. We followed up all HHT patients in the Netherlands who had been taking Thalidomide at the time the original study was completed to find out (i) how many had continued taking Thalidomide and for how long (ii) the nature and severity of any side-effects and (iii) whether side-effects had influenced their decision to continue taking Thalidomide. Only a minority of patients had continued taking the drug despite its beneficial effects on their symptoms and that the side effects were the primary reason to stop. Despite symptom reduction, alternative treatments are still necessary for epistaxis in HHT patients and a large-scale clinical trial is not justified although incidental use in the most severely affected patients can be considered.

  15. THE USE OF TUMOR NECROSIS FACTOR α INHIBITORS IN PATIENTS WITH WEBER-CHRISTIAN DISEASE

    Directory of Open Access Journals (Sweden)

    Olga Nikolayevna Egorova

    2013-01-01

    Full Text Available Weber-Christian disease (WCD, also known as idiopathic lobular panniculitis, is a rare disease belonging to the group of diffuse connective tissue diseases. No therapy for WCD has been developed; empirical treatment is typically used. The first description of the use of tumor necrosis factor α inhibitors in a female patient with infiltrative WCD is presented. The tactics of managing this patient category are analyzed.

  16. Udredning og behandling af morbus Osler

    DEFF Research Database (Denmark)

    Kjeldsen, Anette Drøhse; Andersen, Poul Erik; Tørring, Pernille Mathiesen

    2011-01-01

    Morbus Osler or hereditary haemorrhagic telangiectasia (HHT) is a genetic disorder resulting in development of arteriovenous malformations in the mucosa and in visceral organs. The most common symptom is epistaxis. The disease may, however, cause a variety of other serious manifestations such as ......Morbus Osler or hereditary haemorrhagic telangiectasia (HHT) is a genetic disorder resulting in development of arteriovenous malformations in the mucosa and in visceral organs. The most common symptom is epistaxis. The disease may, however, cause a variety of other serious manifestations...

  17. Plain radiographic, nuclear medicine and angiographic observations of hepatogenic pulmonary angiodysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Sang Oh, K; Bender, T M; Bowen, A D; Ledesma-Medina, J

    1983-05-01

    Clinical and radiological findings of hepatogenic pulmonary angiodysplasia are reported in two cases. Myriad spidery pulmonary blood vessles are seen on plain radiographs and verified with right to left intrapulmonary shunting on pulmonary angiogram and pulmonary isotopic perfusion scan. Pathophysiology and differential diagnosis are discussed. We propose the medical term ''pulmonary angiodysplasia'' to include: 1) Pulmonary telangiectasia 2) Hereditary hemorrhagic telangiectasia (Osler-Rendu-Weber syndrome) 3) Classical discrete pulmonary arteriovenous fistula 4) Hepatogenic discrete pulmonary angiodysplasia: pulmonary arteriovenous communications with right to left shunting secondary to hepatic cirrhosis.

  18. Transcatheter embolization of hepatic arteriovenous fistulas in Rendu-Osler-Weber disease: a case report and review of the literature

    International Nuclear Information System (INIS)

    Stockx, L.; Raat, H.; Caerts, B.; Wilms, G.; Marchal, G.; Cutsem, E. van

    1999-01-01

    A patient with hereditary hemorrhagic telangiectasia and diffuse intrahepatic arteriovenous fistulas developed secondary high-output ventricular failure and pulmonary hypertension. A serial staged hepatic arterial coil embolization was performed with long-term resultant haemodynamic and clinical improvement. The methods of this procedure and related complications are discussed. (orig.)

  19. Nasal closure for the treatment of epistaxis secondary to hereditary hemorrhagic telangiectasia.

    Science.gov (United States)

    Sena Esteves, Sara; Cardoso, Carla; Silva, Ana; Abrunhosa, José; Almeida E Sousa, Cecília

    Hereditary hemorrhagic telangiectasia (HHT), also known by the eponym Osler-Weber-Rendu syndrome, is an autosomal dominant disorder characterised by the presence of multiple arteriovenous malformations (AVMs) affecting multiple organs. Many procedures have been used for epistaxis control in patients with this disorder. The objective of this study was to report the treatment of severe HHT-related epistaxiswith the modified Young's procedure. We describe the treatment of 4 patients with severe blood-transfusion-dependent epistaxis who underwent a modified Young's procedure in a tertiary hospital. The nasal closure was bilateral and complete in all cases. All patients were followed for 12 months or longer. The procedure was well tolerated and complete cessation of bleeding was achieved in all the patients. Young's technique is a safe surgical procedure, well tolerated by patients with severe epistaxis and HHT. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

  20. Anomalies of Pulmonary Circulation as a Cause of Hemoptysis: A Series of Unusual Cases and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Kamini Gupta

    2015-05-01

    Full Text Available The expectoration of blood originating from the lower respiratory tract, called hemoptysis, is a common clinical condition with many potential etiologies. Massive hemoptysis is life threatening and needs urgent intervention. Multidetector computed tomography (MDCT is a useful non-invasive imaging modality for the initial assessment of hemoptysis. Using MDCT with multiplanar reformatted images has improved the diagnosis and management of hemoptysis by providing a more precise depiction of bronchial and non-bronchial systemic arteries than conventional computed tomography (CT. In 95% of hemoptysis cases, the systemic arterial system is the origin of bleeding and pulmonary vascular anomalies are a rare cause. Among these, pulmonary arteriovenous malformation, hereditary hemorrhagic telangiectasia, and Osler-Weber-Rendu disease are well known entities. However, primary anomalies affecting pulmonary vessels in the mediastinum or diseases secondarily affecting the pulmonary vessels are unusual causes. Here we present three cases where patients had pulmonary vascular anomalies causing hemoptysis. These patients had decreased pulmonary arterial pressures leading to bronchial and systemic arterial hypertrophy and development of bronchopulmonary collaterals. Secondary CT signs in the parenchyma and mediastinum (mosaic attenuation, ground glass haze, subpleural interstitial thickening, and hypertrophied bronchial arteries were similar in all patients. Hence, evaluation of the MDCT images for primary abnormality led to the diagnosis.

  1. From Osler's Library to the Osler Library of the History of Medicine, McGill University, Montreal: an overview.

    Science.gov (United States)

    Lyons, Christopher

    2007-01-01

    The Osler Library of the History of Medicine was opened in 1929 at McGill University, Montreal, Canada. Sir William Osler (1849-1919), arguably McGill's and Canada's most famous doctor at the time, had bequeathed his magnificent library of almost 8,000 historical works in medicine and, to a lesser extent, science and literature to the university. Under the 30-year reign of its first librarian, Dr. W W. Francis, the Osler Library became famous for its rare books and for its connection with Sir William. Since the 1950s, however, the library has pursued an active collection development policy for both primary and secondary material that has taken it far beyond Osler's original gift. The library has grown in both the size and scope of its holdings and the services it offers to scholars and students of the history of medicine. These have made the Osler Library a major resource centre for studies in the history of the health sciences. This article looks at the Osler Library today in the hopes of making the range of its collections and services better known to the Canadian and international communities.

  2. CLINICAL APPROACH TO HEREDITARY HEMORRHAGIC TELANGIECTASIA

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    Mary Hachmeriyan

    2013-11-01

    Full Text Available Background: Hereditary hemorrhagic telangiectasia (HHT or Rendu-Osler-Weber disease is a rare syndrome, inherited as an autosomal dominant trait with incidence of 1/10000. The clinical manifestations are due to vascular malformations and predisposition to hemorrhages in different organs, the leading symptom being recurrent epistaxis. If diagnosed with HHT, the patient and his relatives and especially children have to be screened for occult vascular malformations.Case report: A 30 years old woman was treated for cerebral stroke, epistaxis, anemia, arterio-venous malformations for over 6 months. Only at this point she was diagnosed with HHT, after noticing the typical mucosal changes. Focused family history revealed symptoms of HHT in her only child, her father, aunt and two cousins The child was screened for occult vascular malformations – attainment of the nasal mucosa, lungs, gastrointestinal system, liver and brain. Pulmonary and gastrointestinal arterio-venous malformations were proven.Conclusion: Any case of recurrent epistaxis should be evaluated for HHT. After confirmation of the diagnosis every patient and close relatives have to be screened for attainment of other organs and followed up in order to prevent severe life threatening complications.

  3. A large pulmonary arteriovenous malformation causing cerebrovascular accidents.

    Science.gov (United States)

    Sladden, David; Casha, Aaron; Azzopardi, Conrad; Manche', Alexander

    2015-04-16

    The incidence of pulmonary arteriovenous malformations (PAVMs) is 2.5 in 100,000. 80% are associated with Osler-Weber-Rendu syndrome or hereditary haemorrhagic telangiectasia. We report the case of a 70-year-old man with a 6 cm spherical mass incidentally found on chest X-ray. There was a localised systolic bruit over the right lower zone posteriorly; however, he was asymptomatic. He had suffered a stroke, affecting his right hand and his speech, from which he recovered. He experienced regular transient ischaemic attacks, on an average of every 2 months. He underwent a right lower lobectomy and on ligating the right lower lobe pulmonary artery the saturations of oxygen rose from 92% to 97%, demonstrating a significant right to left extracardiac shunt. Postoperative recovery was excellent and 1 year later the patient reports no further neurological symptoms. 40% of such lesions exhibit symptoms, however, only one-third are neurological. Treatment should be by percutaneous embolisation. 2015 BMJ Publishing Group Ltd.

  4. [Epistaxis: causes and management].

    Science.gov (United States)

    Siwiec, H; Szymański, M; Szymańska, A; Klatka, J

    2001-01-01

    Evaluation of 265 patients treated in hospital due to nasal bleeding revealed that almost half of them suffered from hypertension and in about 30% of cases it was impossible to establish the cause of epistaxis. The most frequent way to stop the bleeding was anterior nasal packing and in case of failure posterior nasal packing together with anterior one. Posterior nasal packing with Foley's catheter is relatively simple and effective procedure. Introduction of superselective embolization of maxillary artery and dermoplasty in Rendu-Osler disease was very helpful.

  5. William Osler's "The Nervousness of American Women".

    Science.gov (United States)

    Golden, Richard L

    2008-02-01

    Almost a century ago, William Osler, the foremost physician of his time, was approached by a leading periodical to write a series of articles on the health of the American woman. Osler, then the Regius Professor of Medicine at Oxford, wrote an essay dealing with the psychological stresses affecting the "new woman" of the early 20th century at varying stages of her development and the "nervousness" that ensued. The article was never published as a result of his belated reservations on the propriety of a professional writing for a lay journal. Osler's thinking frequently reflected the spirit of his Victorian-Edwardian era, although at times he demonstrated advanced and prescient ideas about sexuality, not often the subject of discussion, even in circumspect form, in contemporary nonprofessional literature.

  6. Sturge-Weber syndrome. Early manifestation and visualization of disease course

    International Nuclear Information System (INIS)

    Stranzinger, E.; Huisman, T.A.G.M.

    2007-01-01

    The purpose of this study was to evaluate ultrasound, MRI, and CT investigations in children with Sturge-Weber syndrome. From 1996 to 2005, a total of 15 investigations of the brain performed in 6 children with Sturge-Weber syndrome were reviewed. We evaluated four ultrasound, five CT, and six MRI exams. With ultrasound an increase of the echogenicity of the periventricular white matter was depicted in the first days of life. MRI is the best modality to demonstrate the vascular malformation, the impaired cerebral venous drainage, and the atrophy of one hemisphere. One-sided periventricular hyperechogenicity on ultrasound can be an early sign of Sturge-Weber syndrome in children with a nevus flammeus. MRI is the method of choice to diagnose Sturge-Weber syndrome and to follow up these children if the neurological status of the patients changes. (orig.) [de

  7. Hereditary hemorrhagic telangiectasia with bilateral pulmonary vascular malformations: A case report

    Directory of Open Access Journals (Sweden)

    Lončarević Olivera

    2016-01-01

    Full Text Available Introduction. Hereditary hemorrhagic telangiectasia (HHT also known as Osler-Weber-Rendu syndrome is an autosomal dominant disease that occurs due to vascular dysplasia associated with the disorder in the signaling pathway of transforming growth factor β (TGF-β. The clinical consequence is a disorder of blood vessels in multiple organ systems with the existence of telangiectasia which causes dilation of capillaries and veins, are present from birth and are localized on the skin and mucosa of the mouth, respiratory, gastrointestinal and urinary tract. They can make a rupture with consequent serious bleeding that can end up with fatal outcome. Since there is a disruption of blood vessels of more than one organic system, the diagnosis is very complex and requires a multidisciplinary approach. Case report. We reported a 40-year-old female patient with a long-time evolution of problems, who was diagnosed and treated at the Clinic for Lung Diseases of the Military Medical Academy in Belgrade, Serbia, because of bilaterally pulmonary arteriovenous malformations associated with HHT. Embolization was performed in two acts, followed with normalization of clinical, radiological and functional findings with the cessation of hemoptysis, effort intolerance with a significant improvement of the quality of life. Conclusion. HHT is a rare dominant inherited multisystem disease that requires multidisciplinary approach to diagnosis and treatment. Embolization is the method of choice in the treatment of arteriovenous malformations with minor adverse effects and very satisfying therapeutic effect.

  8. Ultrasonographic findings of Weber-Christian disease of the breast: a case report

    International Nuclear Information System (INIS)

    Jang, Ji Youn; Chang, Yun Woo; Lee, Dong Wha

    2005-01-01

    Weber-Christian disease is a rare inflammatory disease of subcutaneous fat necrosis that may or may not involve systemic disease. We report the results of a breast sonograph of a case diagnosed as a form of an acute lobular panniculitis affecting the breast and upper limbs

  9. Sir William Osler's perceptions of urolithiasis and the case of the indigo calculus.

    Science.gov (United States)

    Moran, Michael E; Das, Sakti; Rosenberg, Stuart A

    2005-12-01

    Sir William Osler published his textbook, The Principles and Practice of Medicine, in 1892. It became the definitive treatise on a wide variety of diseases. The section on nephrolithiasis clearly presents the etiology, pathology, symptoms, diagnosis, and treatments. What remains a mystery is the mention, under rare forms of human stones, of a type called "indigo." A search of Index Medicus starting from 1909 backward to its inception in 1879 was performed for key words "indigo," "calculus," "renal" or "bladder stones" and "indicanuria." Twelve textbooks of urology published before 1940 were scrutinized for references to indigo calculi. Only two references to indigo were found, both related to its use for treating constipation (1887 and 1891). Of the 12 textbooks, only 4 make passing reference to "indigo stones." They all mention that such calculi are very rare, but direct references to cases are lacking. One textbook references a study of blue stones from Egyptian mummies. It is unlikely that Osler's reference to an indigo calculus was taken lightly during his writing of The Principles and Practice of Medicine. The case of the indigo calculus is fascinating and perhaps enlightening if only for the source of Osler's intrigue.

  10. Disorders of brain development and phakomatosis

    International Nuclear Information System (INIS)

    Merhemis, Z.

    2006-01-01

    Full text: Disorders of brain development and phakomatosis are resulting from disturbed embryonic-foetal development One third of all major embryological anomalies involve CNS, and over 2000 different anomalies have been described. Anomalies of the brain often cause foetal and neonatal death, and mental and physical retardation in pediatric group. The majority of disorders of brain development and phakomatosis are idiopathic, and most of them are not hereditary or familial. Ultrasonography plays the important role in screening foetal and neonatal brain, but after closure of fontanels it is difficult to find the acoustic window. CT has limited contrast resolution, and disadvantage exposing infant to ionizing radiation. It is helpful to demonstrate the presence of calcifications. MR imaging has proved to be a diagnostic tool of major importance in children with disorders of brain development and phakomatosis. The excellent grey/white matter differentiation and multiplanar imaging capabilities of MR allow a systematic analysis of the brain. Disorders occurring in the first 4 weeks of gestation: Disorders of neural tube closure; Chiari malformation; Cephaloceles; Dermoid/Epidermoid. Disorders occurring between 5 and 10 weeks of gestation: Holoprosencephaly; Septo-optic dysplasia; Diencephalic cyst; Dandy Walker complex; Mega cistern magna. Disorders occurring between 2 and 5 months of gestation: Disorders of sulcation and cellular migration; Lissencephaly; Pachigyria; Schizencephaly; Heterotopias; Megaencephaly; Polymicrogyria; Porencephaly; Arachnoid cyst. Corpus callosum anomalies. Phakomatosis: Neurocutaneous Syndromes Neurofibromatosis Type 1 and 2; Tuberous Sclerosis; von Hippel-Lindau disease; Studge-Weber sy; Osler-Weber- Rendu sy

  11. [Max Weber's disease. Research on the disease and therapeutic management at the turn of the century].

    Science.gov (United States)

    Frommer, J; Frommer, S

    1998-05-01

    This article on the German economist and sociologist Max Weber (1864-1920) continues our pathographic and psychohistoric studies on the interrelations between his life, his illness, and his work on "Protestantic Ethics and the Spirit of Capitalism". In a former paper (Frommer u. Frommer, 1993) we focussed on Weber's definition of modern society as an "iron cage" determined by Western rationalism. His theory, which shows that this cultural background demands a great amount of role conformity from the individual, converges with current psychopathological approaches on the personality of depressed patients. In the second article we report on results of our research on further personal documents, and some medical certificates by Weber's physicians. These documents demonstrate the diagnosis of a severe depression in a narcisstic and hypernomic personality.

  12. The Effect of Agave tequilana Weber Inulin on Postprandial Ghrelin Concentration in Obese Patients.

    Science.gov (United States)

    Contreras-Haro, Betsabe; Robles-Cervantes, Jose A; Gonzalez-Ortiz, Manuel; Martinez-Abundis, Esperanza; Espinel-Bermudez, Claudia; Gallegos-Arreola, Martha P; Morgado-Castillo, Karina C

    2017-02-01

    This study was performed to investigate the effect of Agave tequilana Weber inulin on postprandial ghrelin levels in obese patients. A randomized, double-blind, cross-over design was performed. A total of 14 patients were allocated into two groups: one group received a drink that contained 500 mL lemon water, 24 g of A. tequilana Weber inulin, and 75 g glucose and the other group received a placebo drink with 500 mL lemon drink and 75 g of glucose. After a 7-day washout period, the groups were crossed. The primary outcome measure was postprandial ghrelin levels between minute 240 and minute 270. A. tequilana Weber inulin did not change postprandial ghrelin concentration in obese patients.

  13. Teleangiectasia hemorrágica familiar: registro de um caso

    Directory of Open Access Journals (Sweden)

    João Aris Kouyoumdjian

    1983-12-01

    Full Text Available Um caso de teleangiectasia hemorrágica familiar (THF ou doença de Rendu-Osler-Weber com manifestação neurológica é relatado. O paciente apresentou disfunção cortical difusa com crises convulsivas após episódio de hemorragia gástrica. Na ausência de malformações cerebrais ou de fistula arteriovenosa pulmonar (FAVP, uma encefalopatia anóxica inespecífica ou uma disfunção microangiopática é sugerida. As principais complicações neurológicas da THF são discutidas, salientando em primeiro lugar a grande importância da FAVP na gênese de hipoxemia e abscesso cerebral e ocasionalmente trombose cerebral por policitemia e em segundo lugar as malformações vasculares cerebrais e espinhais. A encefalopatia porto-sistêmica também pode ocorrer em menor proporção.

  14. A case report with Weber-Christian disease which recognized interesting abdominal lesions by abdominal ultrasonography and computerized tomography

    International Nuclear Information System (INIS)

    Ishizuka, Jin; Chiba, Junko; Ota, Kei; Mori, Kazuo; Toyota, Takayoshi; Goto, Yoshio

    1984-01-01

    A 59-year-old woman who had Weber-Christian disease associated with typical histological findings such as panniculitis was reported. The patient had painful subcutaneous nodules as the first symptom. She had diabetes mellitus and rheumatoid arthritis, which are reported to be rare complications of this disease. Abdominal ultrasonography and CT scanning revealed various sized cystic lesions and masses in the tail of pancreas. These findings have not yet been reported and seems to be rare in this disease. (Namekawa, K.)

  15. Staphylococcus cohnii as a cause of multiple brain abscesses in Weber-Christian disease.

    Science.gov (United States)

    Yamashita, Satoshi; Yonemura, Kiminobu; Sugimoto, Ryoko; Tokunaga, Makoto; Uchino, Makoto

    2005-11-15

    We report a patient with multiple brain abscesses due to Staphylococcus cohnii. While these brain abscesses markedly responded to the antibiotics, this patient was subsequently suffered from subcutaneous inflammatory nodules in the adipose tissue, which diagnosed him as having Weber-Christian disease (WCD). This is the first report that subcutaneous inflammatory nodules in the adipose tissue, which lead the diagnosis of WCD, followed multiple brain abscesses. To our knowledge, S. cohnii has not yet been reported to cause multiple brain abscesses in humans. Although the etiology of WCD is unknown, an immune mechanism has been implicated in the pathogenesis. Therefore, we should notice that patients with WCD could be immunocompromised hosts with a higher risk to suffer from severe opportunistic infections.

  16. Weber and Durkheim

    DEFF Research Database (Denmark)

    Jensen, Henrik S.

    Weber and Durkheim is a systematic , comparative analysis of the methodologies of Max Weber and Émile Durkheim.......Weber and Durkheim is a systematic , comparative analysis of the methodologies of Max Weber and Émile Durkheim....

  17. Sir William Osler's speech at Troy: a Trojan horse?

    Science.gov (United States)

    Moran, Michael E

    2012-01-01

    Troy, New York, is a city of 55,000 people in upstate New York located along the Hudson River. A city of surprisingly rich cultural heritage, it was the home of New York state's first hospital outside New York City. The 50th anniversary celebration of Troy's hospital brought William Osler to the city as the keynote speaker. This speech, delivered on November 28, 1900, is one of Sir William's less well known addresses. Osler began his comments with Sir Thomas More's Utopia and talked at length about the hospital, its obligations, the influences it has upon the community, and the role of physicians and surgeons. He broached one of his old saws, the salary of attending physicians and their needed role in hospital management. His words were published in the diamond jubilee's records, but the hospital did not outlive its prominent guest professor, and it closed its doors in 1914. Just like the great historical city of Troy, New York's own Troy was on the brink of decline, and its hospital would be the first fatality. Therefore, it is almost prescient that the words of Osler, taken into historical context juxtaposed against the socioeconomic forces at work, are akin to the Greek's offering of a wooden edifice to end the Trojan War.

  18. 'The greatest Brahmin among them': William Osler's (1849-1919) perspective on Oliver Wendell Holmes (1809-94).

    Science.gov (United States)

    Bryan, Charles S

    2010-02-01

    Although North American physicians commonly identify William Osler as their best example of excellence in both medicine and the humanities, Osler himself held Oliver Wendell Holmes as the best example of such an avatar. Holmes made substantial contributions to medicine, including a landmark essay on the 'Contagiousness of Puerperal Fever', and was for a while the best-selling American author on both sides of the Atlantic. Holmes' lesser reputation today when compared with Osler's is best explained by his having fewer devoted protégées, his confining his adult life to Boston and its environs, and his tendency to flit from one thing to another as opposed to consolidating his efforts in a single task as Osler did in writing his Principles and Practice of Medicine.

  19. Weber's alleged emotivism.

    Science.gov (United States)

    Tester, K

    1999-12-01

    This paper seeks to refute Alasdair MacIntyre's contention that the sociology of Max Weber is emotivist. MacIntyre understands emotivism to involve the collapse of all moral judgment into statements of personal preference. It is shown that Weber's sociology analyses this condition and seeks to repudiate it. In no way does Weber embrace emotivism. MacIntyre misses Weber's repudiation because he misreads Weber's sociological project. The paper shows that MacIntyre's reading of Weber can be refuted if attention is paid to the 'Politics as a Vocation' lecture.

  20. Idiopathic pleural panniculitis with recurrent pleural effusion not associated with Weber-Christian disease

    Directory of Open Access Journals (Sweden)

    Laperuta Paolo

    2016-01-01

    Full Text Available A 82-year-old patient with dyspnea and a recurrent history of pleural effusion was admitted into our unit. He performed a Chest computed tomography showing right pleural effusion. Video-assisted thoracoscopy (VATS exploratory showed parietal pleural thickening of adipose tissue. The surgical procedure consisted, therefore, in the execution of multiple biopsies of the parietal pleura which appeared covered, on the whole surface, by islands of adipose tissue, without macroscopic pathological aspects. After the procedure was performed pleurodesis with talc. The definitive histological examination consisted of normal mesothelial cells surrounded by fatty tissue infiltrated by small lymphocytes in a patient without skin lesions or visceral or systemic signs of inflammatory involvement of the adipose tissue. We reported a rare case of idiopathic pleural panniculitis with recurrent pleural effusion not associated with Weber-Christian disease.

  1. Croatian Chemists. II. Karlo Weber

    Directory of Open Access Journals (Sweden)

    Soljačić, I.

    2009-01-01

    Full Text Available An account of Professor Karlo Weber's life is given. He was born January 25th, 1902 in Mramorak (Vojvodina and died September 1st, 1978 in Zagreb. He studied chemistry and physics in Graz and Freiburg and obtained his Ph. D. at the University of Graz in 1926. The first part of his career from 1927 to 1945 Weber spent at the Department of Chemical Technology, Technical Faculty. His advancement from assistantship to full-professorship was not smooth. After May 8th, 1945, Weber barely saved his life and on September 5th, 1945 was formally dismissed from the Technical Faculty. He was out of a job, but soon was employed in the bookstore Juraj Križanić first as a staff-member and then as a salesman. Weber was dismissed from this job on June 30th, 1946. Two weeks later, he joined the Department of Forensic Medicine, Faculty of Medicine in Zagreb. He again started at the bottom of the hierarchy as a low-paid lab-assistant, but being a very able researcher he advanced relatively quickly to the position of full research professor in 1960.Weber was a physical chemist whose main research interest were chemical kinetics, photochemistry and optical phenomena (luminescence, fluorescence, photography. The more important part of his research work was published while he was at the Department of Chemical Technology. In the Department of Forensic Medicine, Weber encountered research different kind of problems – problems from forensic medicine and toxicology. This change in research environment did not prevent Weber in his productivity, he published 159 research papers, more than 70 technical papers, and 4 monographs. Among them especially prominent is Inhibitorwirkungen – Eine Darstellung der Negative Katalyse in Lösungen (Ferdinand Enke-Verlag, Stuttgart, 1938., which was the first monograph on the subject.Under his supervision, 13 M. Sc. theses and 24 Ph. D. theses were produced. Data for the Ph. D. theses are given (although incomplete. Weber is

  2. Effect of Topical Intranasal Therapy on Epistaxis Frequency in Patients With Hereditary Hemorrhagic Telangiectasia: A Randomized Clinical Trial.

    Science.gov (United States)

    Whitehead, Kevin J; Sautter, Nathan B; McWilliams, Justin P; Chakinala, Murali M; Merlo, Christian A; Johnson, Maribeth H; James, Melissa; Everett, Eric M; Clancy, Marianne S; Faughnan, Marie E; Oh, S Paul; Olitsky, Scott E; Pyeritz, Reed E; Gossage, James R

    2016-09-06

    Epistaxis is a major factor negatively affecting quality of life in patients with hereditary hemorrhagic telangiectasia (HHT; also known as Osler-Weber-Rendu disease). Optimal treatment for HHT-related epistaxis is uncertain. To determine whether topical therapy with any of 3 drugs with differing mechanisms of action is effective in reducing HHT-related epistaxis. The North American Study of Epistaxis in HHT was a double-blind, placebo-controlled randomized clinical trial performed at 6 HHT centers of excellence. From August 2011 through March 2014, there were 121 adult patients who met the clinical criteria for HHT and had experienced HHT-related epistaxis with an Epistaxis Severity Score of at least 3.0. Follow-up was completed in September 2014. Patients received twice-daily nose sprays for 12 weeks with either bevacizumab 1% (4 mg/d), estriol 0.1% (0.4 mg/d), tranexamic acid 10% (40 mg/d), or placebo (0.9% saline). The primary outcome was median weekly epistaxis frequency during weeks 5 through 12. Secondary outcomes included median duration of epistaxis during weeks 5 through 12, Epistaxis Severity Score, level of hemoglobin, level of ferritin, need for transfusion, emergency department visits, and treatment failure. Among the 121 patients who were randomized (mean age, 52.8 years [SD, 12.9 years]; 44% women with a median of 7.0 weekly episodes of epistaxis [interquartile range {IQR}, 3.0-14.0]), 106 patients completed the study duration for the primary outcome measure (43 were women [41%]). Drug therapy did not significantly reduce epistaxis frequency (P = .97). After 12 weeks of treatment, the median weekly number of bleeding episodes was 7.0 (IQR, 4.5-10.5) for patients in the bevacizumab group, 8.0 (IQR, 4.0-12.0) for the estriol group, 7.5 (IQR, 3.0-11.0) for the tranexamic acid group, and 8.0 (IQR, 3.0-14.0) for the placebo group. No drug treatment was significantly different from placebo for epistaxis duration. All groups had a significant

  3. [Max Weber's illness--sociologic aspects of the depressive structure].

    Science.gov (United States)

    Frommer, J; Frommer, S

    1993-05-01

    Between 1897 and 1902 the economist and sociologist Max Weber from Heidelberg suffered from a severe depressive crisis with multiple recurrences of its symptomatology in the following years. The biographic background of the disease process is examined. Questions regarding the specific diagnosis are discussed. Furthermore, his work shows that Weber was indirectly deeply concerned with the cultural, historical and social background conditions of depressive experience and behavior in the context of his study on Protestantic Ethics and the Spirit of Capitalism. Weber's definition of modern society as an iron cage, determined by Occidental Rationalism, shows that this cultural background demands a great amount of role conformity from the individual. Weber's theoretical approach should spark interest in the current psychopathological discussion of the characteristic structural features of a depressed personality.

  4. Max Weber

    DEFF Research Database (Denmark)

    Harste, Gorm

    2006-01-01

    Temaet er dels Webers teori om magt, herredømme og autoritet, herunder om en udøvende nagts disciplinære bureaukrati og dets retligt-legale legitimeringsform. Dels hvorledes Weber kunne tænke teorier om magt, og hvordan magt påvirkede tænkning. Hvorledes er det muligt at tænke magtens former, når...

  5. Educational Administration's Weber.

    Science.gov (United States)

    Gronn, Peter

    1994-01-01

    Discusses Max Weber's importance in Greenfield's work, particularly in Greenfield and Ribbins'"Greenfield on Educational Administration" (1993). In concentrating on human actors' subjective understanding, Greenfield was a faithful Weberian. However, he deviated from Weber by disavowing structural explanations of social and organizational…

  6. Treatment of severe neuropatic pain for patients with the syndrome of Klippel-Trenaunay-Weber: case report

    Directory of Open Access Journals (Sweden)

    Danielle Cristina de Oliveira Soares

    2016-12-01

    Full Text Available Introduction: Klippel-Trenaunay-Weber syndrome is a rare congenital anomaly whose etiology has not yet been elucidated. It is characterized by the triad of vascular malformations, cutaneous hemangiomas and overgrowth of bone and soft tissue adjacent. Pain is a common symptom and can be triggered in the following situations: venous disease, scoliosis by the different members and hemangiomas that affect the body surface, the deep planes or viscera. Case report: A female patient, 17, sent from the SARAH Hospital - Fortaleza to the Institute of Clinic Pain José Frota diagnosed with cerebral palsy type triplegia on right eye and anophthalmia featuring an expansive process of vascular origin extending to the paraspinal region, surpassing sciatic foramen and affecting posterior muscles of the left thigh and leg. There are signs of involvement of the sciatic nerve and deep femoral vessels. He was diagnosed with Klippel-Trenaunay-Weber syndrome. She had continuous pain in shock and burning, EVA 10, extending from the lower back, groin and left leg region. The source of hospital treatment consisted of gabapentin 180mg 12/12h, codeine 10mg 12/12h, ibuprofen 300mg 12/12h, codeine/paracetamol 30mg 12/12h and morphine oral 10mg while in pain, but without pain relief. She also used paroxetine 20mg daily, due to depression. The painful condition prevented sleep, caused irritation and worsened the quality of life of the patient. She continued treatment with gabapentin 300mg 12/12h, dipyrone 500mg 12/12h, Methadone 5mg 12/12h and reassessed two weeks showing significant improvement in pain symptoms and sleep, EVA 2. Conclusion: Pain is a symptom commonly described in the clinical picture of patients with this syndrome. The treatment of this disease is still a challenge. The malformations great gifts made it not always possible embolization or painkillers locks. The therapeutic results with strong opioid were satisfactory and have improved the quality of life of

  7. Max Weber and Robert Michels.

    Science.gov (United States)

    Scaff, Lawrence A.

    1981-01-01

    This paper investigates the unique intellectual partnership of Max Weber and Robert Michels, with particular emphasis on Weber's influence on Michel's inquiry into the sociology of parties and organization. Concludes with an evaluation of the import of Weber's critique of Michels' work. (DB)

  8. Clinical Study on 1 Case of Klippel-Trenaunay-Weber Syndrome

    Directory of Open Access Journals (Sweden)

    Chang-Kil, Yoo

    2002-12-01

    Full Text Available Objective : This study was performed to evaluate the treatment of Acupuncture therapy including Herbal Acupuncture theraphy and Korean Bee Venom Theraphy in Klippel-Trenaunay-Weber Syndrome. Methods : We treated 1 case of Klippel-Trenaunay-Weber Syndrome patient with mainly Korean Bee Venom Theraphy, and herbal Acupuncture, acupuncture theraphy from 27th August, 2001 to February, 2003. Results : After 3 weeks of Korean Bee Venom Theraphy, and herbal Acupuncture, acupuncture theraphy, a remarkable improvement was made for Klippel-Trenaunay-Weber Syndrome. Conclusions : Korean Bee Venom Theraphy and other acupuncture therapies were effective in reducing the symptoms. We think that it need the further study and clinical trial for Klippel-Trenaunay-Weber Syndrome.

  9. Brain Perfusion SPECT Imaging in Sturge - Weber Syndrome : Comparison with MR Imaging

    International Nuclear Information System (INIS)

    Ryu, Jin Sook; Choi, Yun Young; Moon, Dae Hyuk; Yang, Seoung Oh; Ko, Tae Sung; Yoo, Shi Joon; Lee, Hee Kyung

    1996-01-01

    The purpose of this study was evaluate the characteristic perfusion changes in patients with Sturge-Weber syndrome by comparison of the findings of brain MR images and perfusion SPECT images. 99m Tc-HMPAO or 99m Tc-ECD interictal brain SPECTs were performed on 5 pediatric patients with Struge-Weber syndrome within 2 weeks after MR imaging. Brain SPECTs of three patients without calcification showed diminished perfusion in the affected area on MR image. A 3 month-old patient without brain atrophy or calcification demonstrated paradoxical hyperperfusion in the affected hemisphere, and follow-up perfusion SPECT revealed decreased perfusion in the same area. The other patient with advanced calcified lesion and atrophy on MR image showed diffusely decreased perfusion in the affected hemisphere, but a focal area of increased perfusion was also noted in the ipsilateral temporal lobe on SPECT. In conclusion, brain perfusion of the affected area of Sturge-Weber syndrome patients was usually diminished, but early or advanced patients may show paradoxical diffuse or focal hyperperfusion in the affected hemisphere. Further studies are needed for better understanding of these perfusion changes and pathophysiology of Struge-Weber syndrome.

  10. The Battle that Weber Lost: Exploring The Fate of Weber's Contribution to the Methodenstreit

    DEFF Research Database (Denmark)

    Jagd, Søren

    This paper explores the outcome of Max Weber's contribution to the Methodenstreit between teh Austrian marginal utility economists and teh German historicist economists in his methodological writings. The main conclusion is the neither economists nor sociologists have found Weber's contribution...... worth a closer look. The result is that Weber's contribution never has found its way int the methodological discussions in economics as well as into economic sociology...

  11. Anticonvulsant Efficacy in Sturge-Weber Syndrome

    Science.gov (United States)

    Kaplan, Emma H.; Kossoff, Eric H.; Bachur, Catherine D.; Gholston, Milton; Hahn, Jihoon; Widlus, Matthew; Comi, Anne M.

    2016-01-01

    OBJECTIVE We analyzed individuals with epilepsy due to Sturge-Weber syndrome to determine which anticonvulsants provided optimal seizure control and which resulted in the fewest side effects. METHODS One-hundred-eight records from a single center were retrospectively analyzed for Sturge-Weber syndrome brain involvement, epilepsy, Sturge-Weber syndrome neuroscores, and currently used anticonvulsants. RESULTS Of the fourteen anticonvulsants that had been employed, the most often used agents were oxcarbazepine or carbamazepine, and levetiracetam. Individuals whose seizures at the most recent visit were fully controlled (seizure-free) for 6 months or longer were more likely to have ever tried, or currently used, oxcarbazepine or carbamazepine than those with uncontrolled seizures. Thirty-nine of 69 individuals (56.5%) were seizure-free with oxcarbazepine or carbamazepine history versus 11 of 35 individuals (31.4%) who had not taken these agents (P anticonvulsants versus 12 of 42 (28.6%) not taking them (P < 0.01). Patients with seizure control for 6 months or longer were less likely to have ever tried, or to currently be taking, levetiracetam than those without control. Sixteen of 56 individuals (28.6%) were seizure-free with levetiracetam history versus 34 of 48 (70.8%) without it (P < 0.001); 14 of 43 individuals (32.6%) were seizure-free and currently taking levetiracetam versus 36 of 61 (59.0%) not taking it (P < 0.01). When topiramate was added as second-line medication, five of nine patients (55.6%) experienced decreased seizure severity, and worsening of glaucoma was not reported. CONCLUSIONS Carbamazepine and oxcarbazepine were associated with better seizure control than levetiracetam in this Sturge-Weber syndrome cohort and so may be preferred as the initial therapy. When used as adjunctive therapy, topiramate was effective in this limited analysis without a clear increased incidence of glaucoma. PMID:26997037

  12. CLINICAL CASE OF PARKES-WEBER-RUBASHOV SYNDROME

    Directory of Open Access Journals (Sweden)

    Zhdonec S. V.

    2018-03-01

    Full Text Available A clinical case of one variant of congenital venous angiodysplasia – Parkes Weber-Rubashov syndrome of the right lower extremity is presented in the article. The features of its clinical presentation and diagnosis difficulties are described. The analysis of the scientific data and own clinical observation showed that Parkes Weber-Rubashov syndrome belongs to the rare congenital disease of the vascular system, in some cases with the absence of typical clinical manifestations and combination with other disorders of the venous system. The best method for diagnosing the syndrome is radiopaque arteriography. The separation of the patent’s arteriovenous fistulas is justified as a radical method of its surgical treatment.

  13. Ascese e prazer: Weber vs. Sombart Ascesis and pleasure: Weber vs. Sombart

    Directory of Open Access Journals (Sweden)

    Glaucia Villas Bôas

    2001-01-01

    Full Text Available De que fontes retira o capitalismo moderno a energia para constituir-se e desenvolver-se? A resposta de Weber é mais lembrada do que a de Sombart, que mergulhou num esquecimento quase total. Mas o exame do contraste entre ambos esses mestres da fase heróica da Sociologia alemã no século XX fornece elementos preciosos para entender melhor não só o seu objeto de estudo como também a potência e os limites da análise sociológica que propunham, por ângulos opostos, o ''hedonista" Sombart e o "ascético" Weber.From which sources does modern capitalism get the energy for constituting and developing itself? Weber's answer is more readily remembered than Sombart's, which plunged into almost total oblivion. But an examination of the contrast between these two masters of the heroic phase of German Sociology in the 20th century gives precious elements for understanding not only the subject of study but also the power and the limits of the sociological analysis which was proposed from opposed angles by the "hedonist" Sombart and the "ascetic" Max Weber.

  14. Detection of acute gastrointestinal bleeding by means of technetium-99m in vivo labelled red blood cells

    International Nuclear Information System (INIS)

    Dolezal, J.; Vizd'a, J.; Bures, J.

    2002-01-01

    Prognosis of gastrointestinal (GI) bleeding depends on the timely and accurate detection of the source of bleeding and sequential surgical or endoscopy therapy. Scintigraphy with red blood cells (RBCs) in vivo labelled by means of technetium-99m hastened detection of source of GI bleeding and improved management of the particular disease. Gastrointestinal endoscopy is the method of choice for the diagnostics of bleeding from upper tract and large bowel. For diagnostics of bleeding from the small bowel we can use scintigraphy with in vivo labelled autological red blood cells if pushenteroscopy, intra-operative enteroscopy or angiography are not available. 31 patients (13 men, 18 women, aged 20-91, mean 56 years) underwent this investigation from 1998 till 2001 at the Department of Nuclear Medicine. All patients had melaena or enterorrhagia associated with acute anaemia. Gastroscopy, colonoscopy, enteroclysis or X-ray angiography did not detect the source of bleeding. Twenty-one patients had positive scintigraphy with in vivo labelled RBCs - 9 patients were already positive on dynamic scintigraphy, and 12 patients were positive on static images. Scintigraphy with in vivo labelled RBCs was negative in 10 patients. GI bleeding stopped spontaneously in these 10 patients with negative scintigraphy. These patients did not undergo intra-operative enteroscopy or surgery. The final diagnosis of the 21 patients with positive scintigraphy was determined in 16 patients by push-enteroscopy (6 patients), intra-operative enteroscopy (6 patients) or by surgery (4 patients). Of these 16 patients the correct place of bleeding was determined by scintigraphy with labelled RBCs in 11 (69%) patients. Final diagnoses of our 16 patients with positive scintigraphy with autological labelled RBCs were: bleeding small bowel arteriovenous malformation (6 patients), uraemic enteritis with bleeding erosions in ileum and jejunum (2 patients), Osler-Rendu- Weber disease (1 patient), pseudocyst of

  15. Emerging roles of BMP9 and BMP10 in hereditary hemorrhagic telangiectasia

    Directory of Open Access Journals (Sweden)

    Emmanuelle eTillet

    2015-01-01

    Full Text Available Rendu-Osler-Weber syndrome, also known as hereditary hemorrhagic telangiectasia (HHT, is an autosomal dominant vascular disorder. Three genes are causally related to HHT: the ENG gene encoding endoglin, a co-receptor of the TGFß family (HHT1, the ACVRL1 gene encoding ALK1 (activin receptor-like kinase 1, a type I receptor of the TGFß family (HHT2, and the SMAD4 gene, encoding a transcription factor critical for this signaling pathway. Bone morphogenetic proteins (BMPs are growth factors of the TGFß family. Among them, BMP9 and BMP10 have been shown to bind directly with high affinity to ALK1 and endoglin, and BMP9 mutations have recently been linked to a vascular-anomaly syndrome that has phenotypic overlap with HHT. BMP9 and BMP10 are both circulating cytokines in blood, and the current working model is that BMP9 and BMP10 maintain a quiescent endothelial state that is dependent on the level of ALK1/endoglin activation on endothelial cells. In accordance with this model, to explain the etiology of HHT we hypothesize that a deficient BMP9/BMP10/ALK1/endoglin pathway may lead to re-activation of angiogenesis or a greater sensitivity to an angiogenic stimulus. Resulting endothelial hyperproliferation and hypermigration may lead to vasodilatation and formation of arteriovenous malformation (AVM. HHT would thus result from a defect in the angiogenic balance. This review will focus on the emerging role played by BMP9 and BMP10 in the development of this disease and the therapeutic approaches that this opens.

  16. Outcome of Hemispherectomy in Sturge-Weber Syndrome

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2002-12-01

    Full Text Available A questionnaire was mailed to the parents of 70 patients identified by the Sturge-Weber Foundation as recipients of hemispherectomy between 1979 and 2001, and responses obtained from 32 (46% were analysed at the Johns Hopkins Hospital, Baltimore, MD.

  17. Max Weber and the Iron Cage of Technology

    Science.gov (United States)

    Maley, Terry

    2004-01-01

    Max Weber is seen by mainstream social scientists as a sociologist, social theorist, and theorist of bureaucracy. In this reassessment of Weber's social science and its methodology, it is suggested that Weber can also be seen as a compelling early 20th-century critic of science and technology. The theme of technology, and Webers ambivalence about…

  18. Max Weber - bedaget og aktuel

    DEFF Research Database (Denmark)

    Eistrup, Jens

    2010-01-01

    undervisningsartiklen introducerer en række af Max Webers centrale begreber om herredømme, bureaukrati, social handlen, rationalitet og profession. dernæst illustreres hvorledes Webers begreber kan tjene til at belyse spændingsfeltet mellem politik og profession i en socialfaglig kontekst....

  19. Genetics Home Reference: Sturge-Weber syndrome

    Science.gov (United States)

    ... Testing Registry: Sturge-Weber syndrome Other Diagnosis and Management Resources (7 links) Boston Children's Hospital: Sturge-Weber Syndrome Clinic Children's Hospital of Philadelphia: Capillary Vascular Malformations: Port ...

  20. Weber, Durkheim, and the comparative method.

    Science.gov (United States)

    Kapsis, R E

    1977-10-01

    This essay compares and contrasts the means by which Durkheim and Weber dealt with methodological issues peculiar to the comparative study of societies, what Smelser has called "the problem of sociocultural variability and complexity." More specifically, it examines how Weber and Durkheim chose appropriate comparative units for their empirical studies. The approaches that Weber and Durkheim brought to theproblem of cross-cultural comparison have critical implications for more current procedures used in the comparative study of contemporary and historical societies.

  1. DHPLC-based mutation analysis of ENG and ALK-1 genes in HHT Italian population.

    Science.gov (United States)

    Lenato, Gennaro M; Lastella, Patrizia; Di Giacomo, Marilena C; Resta, Nicoletta; Suppressa, Patrizia; Pasculli, Giovanna; Sabbà, Carlo; Guanti, Ginevra

    2006-02-01

    Hereditary haemorrhagic telangiectasia (HHT or Rendu-Osler-Weber syndrome) is an autosomal dominant disorder characterized by localized angiodysplasia due to mutations in endoglin, ALK-1 gene, and a still unidentified locus. The lack of highly recurrent mutations, locus heterogeneity, and the presence of mutations in almost all coding exons of the two genes makes the screening for mutations time-consuming and costly. In the present study, we developed a DHPLC-based protocol for mutation detection in ALK1 and ENG genes through retrospective analysis of known sequence variants, 20 causative mutations and 11 polymorphisms, and a prospective analysis on 47 probands with unknown mutation. Overall DHPLC analysis identified the causative mutation in 61 out 66 DNA samples (92.4%). We found 31 different mutations in the ALK1 gene, of which 15 are novel, and 20, of which 12 are novel, in the ENG gene, thus providing for the first time the mutational spectrum in a cohort of Italian HHT patients. In addition, we characterized the splicing pattern of ALK1 gene in lymphoblastoid cells, both in normal controls and in two individuals carrying a mutation in the non-invariant -3 position of the acceptor splice site upstream exon 6 (c.626-3C>G). Functional essay demonstrated the existence, also in normal individuals, of a small proportion of ALK1 alternative splicing, due to exon 5 skipping, and the presence of further aberrant splicing isoforms in the individuals carrying the c.626-3C>G mutation. 2006 Wiley-Liss, Inc.

  2. Long-term results of the Weber operation for chronic ankle instability: 37 patients followed for 20-30 years

    NARCIS (Netherlands)

    de Vries, Jasper; Struijs, Peter A. A.; Raaymakers, Ernst L. F. B.; Marti, René K.

    2005-01-01

    BACKGROUND: The Weber operation is an anatomical reconstruction of the anterior talofibular ligament with the plantaris tendon. Few long-term studies have been published. METHODS: We evaluated 40 ankles in 37 patients (19 women) at an average of 24 years after the procedure. RESULTS: At follow-up,

  3. Homoisoflavanones from Agave tequilana Weber.

    Science.gov (United States)

    Morales-Serna, José Antonio; Jiménez, Armando; Estrada-Reyes, Rosa; Marquez, Carmen; Cárdenas, Jorge; Salmón, Manuel

    2010-05-04

    Three homoisoflavanones were isolated from the "piña" and leaves of Agave tequilana Weber. The compounds were identified as: 5,7-dihydroxy-3-(4-methoxybenzyl)-chroman-4-one (1), 7-hydroxy-3-(4-hydroxybenzyl)-chroman-4-one (2) and 4'-demethyl-3,9-dihydro-punctatin (3). This is the first phytochemical study carried out to Agave tequilana Weber.

  4. Max Weber entre liberalismo y republicanismo

    Directory of Open Access Journals (Sweden)

    Villacañas Berlanga, José Luis

    2005-12-01

    Full Text Available This article attempts to analyze the differences between social democracy and political democracy as modern processes, as these were understood by Max Weber. The archetype of modernity resides, from this point of view, in a convergence of both processes, as occurred in countries like the USA and Great Britain. Weber's diagnosis is that, whereas social democracy can be organized around liberal arguments, political democracy cannot advance without republican concepts like those of virtue, rigour, responsability and representation. For Weber, these values could only become present in Germany thanks to an adequate and democratic reception of Nietzsche, and in some way this reception could make sense of the former's work.

    Este artículo pretende analizar las diferencias entre democracia social y democracia política como procesos modernos, tal y como fueron comprendidos por Max Weber. Lo arquetípico de la modernidad reside, desde este punto de vista, en una convergencia de ambos procesos, tal y como se dio en países como USA y Gran Bretaña. El diagnóstico de Weber es que, mientras la democracia social puede organizarse sobre argumentos liberales, la democracia política no puede avanzar sin conceptos republicanos como el de virtud, rigor, responsabilidad y representación. Para Weber, estos valores sólo podrían hacerse presentes en Alemania a partir de una recepción adecuada y democrática de Nietzsche y en cierto modo esta recepción daría sentido a su obra.

  5. Max Weber e a história cultural da modernidade Max Weber and the cultural history of modernity

    Directory of Open Access Journals (Sweden)

    Gangolf Hübinger

    2012-01-01

    Full Text Available Este artigo oferece um novo olhar sobre Weber como historiador. O fato de que a MWG reconstrói, de maneira bastante detalhada, o contexto histórico científico e intelectual torna mais evidente como os diferentes tópicos sobre os quais Weber trabalhou - a economia de mercado capitalista, as religiões do mundo ou o Estado moderno - foram abordados em última instância "do ponto de vista da história cultural". Diante desse quadro, meu artigo explora quatro aspectos principais: história econômica; ou mais precisamente o sentido cultural da ação econômica como ponto-chave para o pensamento histórico de Weber como um todo (I; a insistência de Weber na primazia de problemas analíticos e da escolha de perspectivas como pressupostos intelectuais do conhecimento histórico (II; a questão fundamental da história cultural em geral: como as ideias se atualizam em conflitos e constelações históricos e como a singularidade do desenvolvimento cultural europeu deve ser descrita em sua significação histórico-universal (III; a relevância da Primeira Guerra Mundial para os problemas e perspectivas de Weber: seu foco em uma teoria histórico-política do poder e da dominação e da gênese e estrutura do Estado moderno e da democracia (IV.This article takes a new look at Weber as a historian. The MWG's detailed reconstruction of the scientific and intellectual historical context provides a clear insight into how all the different topics on which Weber worked - the capitalist market economy, world religions, or the modern state - were ultimately treated "from the view-point of cultural history." In response the article examines four aspects: Economic history; or more precisely, the cultural meaning of economic action as a key to Weber's historical thinking as a whole (I; Weber's insistence on the primacy of analytical problems and of choices of perspectives as intellectual presuppositions of historical knowledge (II; the cardinal question of

  6. Homoisoflavanones from Agave tequilana Weber

    Directory of Open Access Journals (Sweden)

    José Antonio Morales-Serna

    2010-05-01

    Full Text Available Three homoisoflavanones were isolated from the “piña” and leaves of Agave tequilana Weber. The compounds were identified as: 5,7-dihydroxy-3-(4-methoxybenzyl-chroman-4-one (1, 7-hydroxy-3-(4-hydroxybenzyl-chroman-4-one (2 and 4’-demethyl-3,9-dihydro-punctatin (3. This is the first phytochemical study carried out to Agave tequilana Weber.

  7. Cerebellar arteriovenous malformations in children

    International Nuclear Information System (INIS)

    Griffiths, P.D.; Humphreys, R.P.

    1998-01-01

    We review the presentation, imaging findings and outcome in 18 children with cerebellar arteriovenous malformations (AVM). This group is of particular interest because of the reported poor outcome despite modern imaging and neurosurgical techniques. All children had CT and 15 underwent catheter angiography at presentation. Several of the children in the latter part of the study had MRI. Of the 18 children, 17 presented with a ruptured AVM producing intracranial haemorrhage. The remaining child presented with temporal lobe epilepsy and was shown to have temporal, vermian and cerebellar hemisphere AVM. This child had other stigmata of Osler-Weber-Rendu syndrome. Three other children had pre-existing abnormalities of possible relevance. One had a vascular malformation of the cheek and mandible, one a documented chromosomal abnormality and another a midline cleft upper lip and palate. Six of the 17 children with a ruptured cerebellar AVM died within 7 days of the ictus. Vascular pathology other than an AVM was found in 10 of the 14 children with a ruptured cerebellar AVM who had angiography: 4 intranidal aneurysms, 5 venous aneurysms and 2 cases of venous outflow obstruction (one child having both an aneurysm and obstruction). The severity of clinical presentation was directly related to the size of the acute haematoma, which was a reasonable predictor of outcome. (orig.)

  8. Cerebellar arteriovenous malformations in children

    Energy Technology Data Exchange (ETDEWEB)

    Griffiths, P.D. [Sheffield Univ. (United Kingdom). Acad. Dept. of Radiol.; Blaser, S.; Armstrong, D.; Chuang, S.; Harwood-Nash, D. [Division of Neuroradiology, The Hospital for Sick Children and University of Toronto, Toronto (Canada); Humphreys, R.P. [Division of Neurosurgery, The Hospital for Sick Children and University of Toronto, Toronto (Canada)

    1998-05-01

    We review the presentation, imaging findings and outcome in 18 children with cerebellar arteriovenous malformations (AVM). This group is of particular interest because of the reported poor outcome despite modern imaging and neurosurgical techniques. All children had CT and 15 underwent catheter angiography at presentation. Several of the children in the latter part of the study had MRI. Of the 18 children, 17 presented with a ruptured AVM producing intracranial haemorrhage. The remaining child presented with temporal lobe epilepsy and was shown to have temporal, vermian and cerebellar hemisphere AVM. This child had other stigmata of Osler-Weber-Rendu syndrome. Three other children had pre-existing abnormalities of possible relevance. One had a vascular malformation of the cheek and mandible, one a documented chromosomal abnormality and another a midline cleft upper lip and palate. Six of the 17 children with a ruptured cerebellar AVM died within 7 days of the ictus. Vascular pathology other than an AVM was found in 10 of the 14 children with a ruptured cerebellar AVM who had angiography: 4 intranidal aneurysms, 5 venous aneurysms and 2 cases of venous outflow obstruction (one child having both an aneurysm and obstruction). The severity of clinical presentation was directly related to the size of the acute haematoma, which was a reasonable predictor of outcome. (orig.) With 4 figs., 4 tabs., 23 refs.

  9. Growing skull hemangioma: first and unique description in a patient with Klippel-Trénaunay-Weber syndrome.

    Science.gov (United States)

    van der Loo, Lars E; Beckervordersandforth, Jan; Colon, Albert J; Schijns, Olaf E M G

    2017-02-01

    We present the first and unique case of a rapid-growing skull hemangioma in a patient with Klippel-Trénaunay-Weber syndrome. This case report provides evidence that not all rapid-growing, osteolytic skull lesions need to have a malignant character but certainly need a histopathological verification. This material offers insight into the list of rare pathological diagnoses in an infrequent syndrome.

  10. Max Weber Visits America: A Review of the Video

    Directory of Open Access Journals (Sweden)

    Michael Wise

    2006-11-01

    Full Text Available The North Carolina Sociological Society is proud to announce the long-awaited video of Max Weber's trip to North Carolina as retold by two of his cousins. Max Weber made a trip to visit relatives in Mount Airy, North Carolina, in 1904. This 2004 narrative by Larry Keeter and Stephen Hall is the story of locating and interviewing two living eyewitnesses (1976 to Max Weber's trip. The video includes information about Weber's contributions to modern sociology. Dowloadable files are provided using the .mp4 format. The video should appeal to students and professors interested in Max Weber. It can be included in courses ranging from introductory sociology to theory.

  11. Max Weber Visits America: A Review of the Video

    OpenAIRE

    Michael Wise

    2006-01-01

    The North Carolina Sociological Society is proud to announce the long-awaited video of Max Weber's trip to North Carolina as retold by two of his cousins. Max Weber made a trip to visit relatives in Mount Airy, North Carolina, in 1904. This 2004 narrative by Larry Keeter and Stephen Hall is the story of locating and interviewing two living eyewitnesses (1976) to Max Weber's trip. The video includes information about Weber's contributions to modern sociology. Dowloadable files are provided...

  12. Is Bauman's bureau Weber's bureau?: a comment.

    Science.gov (United States)

    du Gay, P

    1999-12-01

    In his highly regarded and influential Modernity and the Holocaust Zygmunt Bauman launched one of the most passionate and sustained critiques of 'bureaucratic rationality' seen within social theory for some time. In so doing he drew heavily upon the work of Max Weber for support. In this brief paper I am interested in exploring the extent to which Weber really is the anti-bureaucratic ally Bauman claims him to be. In the first part of the paper I outline the main elements of Bauman's critique of bureaucratic rationality, drawing particular attention to its reliance upon a self-consciously Weberian theoretical lexicon. In the second part of the paper I seek to indicate that, despite his claims to be following in Weber's tracks, Bauman's conclusions regarding the moral vacuity of bureaucratic conduct are the very antithesis of Weber's own.

  13. Natural history and magnetic resonance imaging follow-up in 9 Sturge-Weber Syndrome patients and clinical correlation.

    Science.gov (United States)

    Udani, Vrajesh; Pujar, Suresh; Munot, Pinki; Maheshwari, Shailendra; Mehta, Nirad

    2007-04-01

    The natural history of Sturge-Weber Syndrome is variable where some patients have refractory epilepsy and persistent neurologic deficits while others do well. Also, evolution of MRI abnormalities is largely unknown. This long-term follow-up study tries to address these two issues. This retrospective and later prospective study followed 9 children with confirmed SWS. Clinical details of seizures, stroke-like episodes, neurologic and developmental deficits were ascertained specifically. Patients were divided into those with onset below or after 6 months of age for analysis. Disease was classified as active or inactive and correlations were made with the use of aspirin. All past, as well as prospectively acquired imaging was reviewed by two independent blinded neuroradiologists and the images were analysed as ictal (temporally related to seizure/stroke-like event) or interictal. Degree and extent of leptomeningeal enhancement was specifically looked for. Four boys and five girls were followed up for a mean of 6.1 years. Disease activity subsided in 8/9. Early-onset patients had a severe early course with significant residual deficits while late-onset patients did uniformly well. In 6 patients where aspirin was used, a stable course ensued. There was a significant increase in degree/extent of leptomeningeal enhancement during an ictus which returned to the baseline in the interictal state in all 7 patients where both images were available. Focal cerebral atrophy worsened in early-onset cases. In conclusion, SWS patients with onset of seizures/stroke-like events before 6 months of age seem to do worse with a severe early course and persistent neurologic deficits. However the course stabilizes after 5 years of age in most. Late-onset SWS patients have a benign course. Aspirin use is associated with a stable course though further studies are needed. The leptomeningeal enhancement appears to increase during acute events before returning to baseline suggesting that extent

  14. Klippel-Trenaunay-Weber Syndrome:A case report

    Energy Technology Data Exchange (ETDEWEB)

    Ryu, Kyung Nam; Lee, Sun Wha; Yoon, Yup; Lim, Jae Hoon [Kyung Hee University Hopital, Seoul (Korea, Republic of)

    1990-10-15

    The Klippel-Trenauna-Weber Syndrome is characterized by a classical triad that includes unilateral cutaneous capillary hemangiomas, varicose veins, and local gigantism with both soft tissue and osseous overgrowth. Authors have experience on case of Klippel-Trenaunay-Weber Syndrome with hemangiomas of ipsilateral scrotum and foot.

  15. Imaging of the sturge-weber syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Choong Gon; Kim, In One; Kim, Woo Sun; Han, Moon Hee; Moon, Woo Kyung; Chang, Kee Hyun; Yeon, Kyung Mo [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1994-02-15

    The purposes of this article are to illustrate the typical imaging features of eight patients with this syndrome and to discuss the advantage of each imaging modality with a concise review of literatures. We retrospectively reviewed plain skull radiographs, computed tomographic (CT) scans, magnetic resonance (MR) images and cerebral angiograms of eight patients with Sturge-Weber syndrome. We analyzed the radiographic findings of Sturge-Weber syndrome and compared the findings of CT, MR and angiography. Plain radiographs showed characteristic gyriform calcification after 2 years of age. CT scans excellently demonstrated cortical calcifications, prominently enhancing choroid plexi and dilated periventricular veins. MR revealed dilated deep cerebral veins as tubular or spot-like signal void structures at periventricular areas and showed stripes of cortical enhancement after gadolinium infection. Angiography showed dilated tortuous medullary and deep cerebral veins as the collateral pathways of blood shunting. MR was superior to CT in the detection of parenchymal atrophy, venous abnormalities and the extent of angiomatous involvement. Angiography showed enlarged deep cerebral or medullary veins better than MR imaging. We think that each imaging modality including CT, MR or angiography has unique advantages in the diagnosis of this syndrome but MR will be used frequently because of its superior ability for the detection of atrophy, vascular abnormalities and direct visualization of leptomeningeal angiomatosis with contrast enhancement.

  16. Imaging of the sturge-weber syndrome

    International Nuclear Information System (INIS)

    Choi, Choong Gon; Kim, In One; Kim, Woo Sun; Han, Moon Hee; Moon, Woo Kyung; Chang, Kee Hyun; Yeon, Kyung Mo

    1994-01-01

    The purposes of this article are to illustrate the typical imaging features of eight patients with this syndrome and to discuss the advantage of each imaging modality with a concise review of literatures. We retrospectively reviewed plain skull radiographs, computed tomographic (CT) scans, magnetic resonance (MR) images and cerebral angiograms of eight patients with Sturge-Weber syndrome. We analyzed the radiographic findings of Sturge-Weber syndrome and compared the findings of CT, MR and angiography. Plain radiographs showed characteristic gyriform calcification after 2 years of age. CT scans excellently demonstrated cortical calcifications, prominently enhancing choroid plexi and dilated periventricular veins. MR revealed dilated deep cerebral veins as tubular or spot-like signal void structures at periventricular areas and showed stripes of cortical enhancement after gadolinium infection. Angiography showed dilated tortuous medullary and deep cerebral veins as the collateral pathways of blood shunting. MR was superior to CT in the detection of parenchymal atrophy, venous abnormalities and the extent of angiomatous involvement. Angiography showed enlarged deep cerebral or medullary veins better than MR imaging. We think that each imaging modality including CT, MR or angiography has unique advantages in the diagnosis of this syndrome but MR will be used frequently because of its superior ability for the detection of atrophy, vascular abnormalities and direct visualization of leptomeningeal angiomatosis with contrast enhancement

  17. Regional cerebral blood flow characteristics of the Sturge-Weber syndrome

    International Nuclear Information System (INIS)

    Riela, A.R.; Stump, D.A.; Roach, E.S.; McLean, W.T. Jr.; Garcia, J.C.

    1985-01-01

    Four patients with the Sturge-Weber syndrome were studied using the non-invasive Xenon-133 inhalation technique. All four patients had decreased regional cerebral blood flow in the area of their lesion, and in two patients who were subsequently tested with 5% carbon dioxide inhalation, impaired vasomotor reactivity was documented. Diminished regional cerebral blood flow is consistent with previously described nuclide flow studies which demonstrated a delay in the initial perfusion blush in the region of the abnormal vasculature. The focal decrease in blood flow was greatest in the most severely affected patient, but was also prominent in the two younger patients, both of whom have excellent neurologic function. These studies suggest that localized decrease in blood flow and vasomotor dysfunction in Sturge-Weber syndrome can precede the occurrence of severe neurologic impairment and extensive cerebral atrophy and possibly be a major contributing factor in progressive dysfunction. A secondary observation was that the blood flow in the unaffected hemisphere was significantly greater in two children compared to the two adults and was similar to the age-related differences reported for normal children and adults

  18. Weber e o advento do novo

    Directory of Open Access Journals (Sweden)

    Renan Springer de Freitas

    2010-01-01

    Full Text Available Établir la distinction entre le vieux et le neuf et montrer comment on passe de l'un à l'autre sont des enjeux primordiaux dans la sociologie de la religion de Max Weber. Dans cet article, on montre comment Weber les a traités ainsi que ses difficultés à les affronter. Pour Weber, la transition du vieux vers le neuf exige une certaine "transformation de l'âme humaine", ou bien une rupture tranchée avec les types de mentalités tenues pour traditionnelles. On propose que cette conception l'a mené à considérer des ruptures inexistantes (par exemple entre les esprits luthériens et calvinistes et à ne pas s'apercevoir de ruptures importantes (par exemple, entre la tolérance particulière à nos jours et celle d'époques précédentes. On conclut qu'il y a une analogie entre la sociologie webérienne de la religion et la physique aristotélicienne et que, à la lumière de cette analogie, on peut mieux comprendre ce que Weber veut signifier lorsqu'il affirme que des transitions historiquement significatives demandent une "transformation de l'âme humaine".

  19. "Weber-tesen"

    DEFF Research Database (Denmark)

    Bruun, Hans Henrik

    2009-01-01

    Der er næppe nogen anden samfundsvidenskabelig tese der er blevet så berømt på et grundlag der som oftest er så misforstået som Max Webers (1864-1920) tese om sammenhængen mellem den protestantiske etik og kapitalismens ånd. Man har set ham som den der ”vender Marx på hovedet” ved at vise, at det...

  20. Max Weber in the American Journal of Sociology: A case of circulating knowledge.

    Science.gov (United States)

    Rijks, Marlise

    2012-01-01

    By the middle of the 1970s, a Max Weber revival commenced in American sociology. Almost 75 percent of the articles on Weber in the American Journal of Sociology published in the past six decades appeared in the 1970s and 1980s. The Weber revival in American sociology is a phenomenon observed in leading literature. New translations and publications are frequently indicated reasons for the renewed interest in Weber. Without dismissing this factor, it is not an entirely satisfactory explanation. This article accounts for the Weber revival in a new way. Taking the American Journal of Sociology as a case study, I argue that the Weber revival was a case of circulating knowledge. Certain historically set issues led to a reorientation of Weber, which meant that knowledge about Weber was moved, extended, and transformed. © 2012 Wiley Periodicals, Inc.

  1. Weberized Mumford-Shah Model with Bose-Einstein Photon Noise

    International Nuclear Information System (INIS)

    Shen Jianhong; Jung, Yoon-Mo

    2006-01-01

    Human vision works equally well in a large dynamic range of light intensities, from only a few photons to typical midday sunlight. Contributing to such remarkable flexibility is a famous law in perceptual (both visual and aural) psychology and psychophysics known as Weber's Law. The current paper develops a new segmentation model based on the integration of Weber's Law and the celebrated Mumford-Shah segmentation model (Comm. Pure Appl. Math., vol. 42, pp. 577-685, 1989). Explained in detail are issues concerning why the classical Mumford-Shah model lacks light adaptivity, and why its 'weberized' version can more faithfully reflect human vision's superior segmentation capability in a variety of illuminance conditions from dawn to dusk. It is also argued that the popular Gaussian noise model is physically inappropriate for the weberization procedure. As a result, the intrinsic thermal noise of photon ensembles is introduced based on Bose and Einstein's distributions in quantum statistics, which turns out to be compatible with weberization both analytically and computationally. The current paper focuses on both the theory and computation of the weberized Mumford-Shah model with Bose-Einstein noise. In particular, Ambrosio-Tortorelli's Γ-convergence approximation theory is adapted (Boll. Un. Mat. Ital. B, vol. 6, pp. 105-123, 1992), and stable numerical algorithms are developed for the associated pair ofnonlinear Euler-Lagrange PDEs

  2. Cancer cachexia and its impact on patient dignity: What nurses need to know

    Directory of Open Access Journals (Sweden)

    Susan McClement

    2016-01-01

    Full Text Available Noted physician, Sr. William Osler, is credited with saying, “Care more particularly for the individual patient than for the special features of the disease”. Osler understood that each patient for whom we care is first and foremost a person, who also happens to be living with a particular illness. In addition to understanding the nature of the patient's illness, therefore, it is also critically important that we come to understand the patient's unique story and set of circumstances. Doing so allows us to engage with patients in a way that affirms their sense of dignity and personhood. Drawing on the exemplar of cancer cachexia, this editorial reminds clinicians of the importance of Osler's sage advice to attend to patient dignity and personhood, and provides nurses with direction about how they can do that in practice.

  3. Pulmonary arteriovenous malformations: overview and transcatheter embolotherapy

    International Nuclear Information System (INIS)

    Pugash, R.A.

    2001-01-01

    The majority of pulmonary arteriovenous malformations (pAVMs) are found in people with hereditary hemorrhagic telangiectasia (HHT), a condition also known as Osler-Weber-Rendu syndrome. HHT is a clinically heterogeneous autosomal dominant disorder in which abnormal blood vessels cause bleeding and arteriovenous shunting. The 2 basic lesions of HHT - telangiectasias and arteriovenous malformations (AVMs) - are closely related. Multisystem involvement leads to a staggering array of clinical manifestations, making HHT one of medicine's less familiar 'great pretenders'. Telangiectasias are dilated blood vessels, typically located in mucocutaneous surfaces (i.e., skin, conjunctiva, respiratory tract, gastrointestinal tract, urinary tract). Small telangiectasias are simply dilated post-capillary venules, whereas larger telangiectasias are made up of dilated arterioles and venules, often with no intervening capillary. They are, in essence, diminutive AVMs. These tiny lesions are visible as punctate bright red spots on skin and mucosal surfaces (Fig. 1). Their fragility and superficial location account for the disabling epistaxis and chronic gastrointestinal bleeding, which are so common with HHT. Hematuria (caused by urothelial telangiectasias) occurs occasionally but is not a prominent feature of the disease. Although tracheobronchial telangiectasias do occur and may cause hemoptysis, severe hemoptysis is typically related to pAVM rupture. AVMs are direct artery-to-vein connections. Though larger and far more impressive radiologically than telangiectasias, AVMs are more likely to be clinically silent until they either declare themselves in a catastrophic fashion or are detected by screening tests. In contrast to telangiectasias, which are generally found in epithelial surfaces, AVMs tend to develop within organs, most commonly the lung and brain. As screening methods evolve, liver involvement with both telangiectasias and complex AVMs is being recognized with increasing

  4. Pulmonary arteriovenous malformations: overview and transcatheter embolotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Pugash, R.A. [Univ. of Toronto, St. Michael' s Hospital, Dept. of Medical Imaging, Toronto, Ontario (Canada)

    2001-04-01

    The majority of pulmonary arteriovenous malformations (pAVMs) are found in people with hereditary hemorrhagic telangiectasia (HHT), a condition also known as Osler-Weber-Rendu syndrome. HHT is a clinically heterogeneous autosomal dominant disorder in which abnormal blood vessels cause bleeding and arteriovenous shunting. The 2 basic lesions of HHT - telangiectasias and arteriovenous malformations (AVMs) - are closely related. Multisystem involvement leads to a staggering array of clinical manifestations, making HHT one of medicine's less familiar 'great pretenders'. Telangiectasias are dilated blood vessels, typically located in mucocutaneous surfaces (i.e., skin, conjunctiva, respiratory tract, gastrointestinal tract, urinary tract). Small telangiectasias are simply dilated post-capillary venules, whereas larger telangiectasias are made up of dilated arterioles and venules, often with no intervening capillary. They are, in essence, diminutive AVMs. These tiny lesions are visible as punctate bright red spots on skin and mucosal surfaces (Fig. 1). Their fragility and superficial location account for the disabling epistaxis and chronic gastrointestinal bleeding, which are so common with HHT. Hematuria (caused by urothelial telangiectasias) occurs occasionally but is not a prominent feature of the disease. Although tracheobronchial telangiectasias do occur and may cause hemoptysis, severe hemoptysis is typically related to pAVM rupture. AVMs are direct artery-to-vein connections. Though larger and far more impressive radiologically than telangiectasias, AVMs are more likely to be clinically silent until they either declare themselves in a catastrophic fashion or are detected by screening tests. In contrast to telangiectasias, which are generally found in epithelial surfaces, AVMs tend to develop within organs, most commonly the lung and brain. As screening methods evolve, liver involvement with both telangiectasias and complex AVMs is being recognized

  5. Sturge-Weber syndrome. The current neuro-imaging data; Syndrome de Sturge-Weber. Donnees actuelles de l'imagerie neuroradiologique

    Energy Technology Data Exchange (ETDEWEB)

    Boukobza, M.; Cambra, M.R.; Merland, J.J.; Enjolras, O. [Hopital Lariboisiere, 75 - Paris (France)

    2000-07-01

    Sturge-Weber syndrome (SWS) is a rare congenital sporadic disease with neuro-ocular and cutaneous vascular findings. Clinically, the full-blown condition consists of a facial port-wine stain (PWS) the V1 facial trigeminal skin area, alone or in combination with V2 and V3 PWS, seizures and ocular abnormalities (glaucoma and choroidal angioma). Radiologically, a leptomeningeal (pial) capillary and venous malformation, mostly located in the parieto-occipital area, cerebral atrophy and calcifications are demonstrated. An ipsilateral enlarged choroid plexus may be an early anatomic symptom. Development neuro-diagnostic technique for the screening of infants with an at-risk V1 PWS, as well as for the follow-up of patients with evidence SWS. Accelerated myelination in the involved hemisphere may be early diagnostic feature before 6 months of age. Later, hyperintensity of white matter on T2 is considered a symptom of gliosis. Clinically, progression of the diseases is associated with anatomic changes and correlates with the extent of the pial vascular anomaly, extent and severity of cerebral atrophy, and white matter abnormalities. A neonatal neuro-imaging work-up, using CT or MRI, may not demonstrate the pial anomaly and should be repeated after 6 to 12 months in an at-risk infant with V1 PWS. (authors)

  6. Angiography, gingival hyperplasia and Sturge-Weber syndrome: report of case.

    Science.gov (United States)

    Wilson, S; Venzel, J M; Miller, R

    1986-01-01

    This syndrome, also known as encephalotrigeminal angiomatosis, is a condition with multiple clinical findings, including vascular anomalies and intraoral involvement. The patient was a nine-year-old black boy with Sturge-Weber syndrome. He had a lesion removed and diagnosed as pyogenic granuloma. There were no complications and the tissue healed normally.

  7. Sturge-Weber syndrome

    Directory of Open Access Journals (Sweden)

    Natarajan Manivannan

    2012-01-01

    Full Text Available Encephalotrigeminal angiomatosis (Sturge-Weber syndrome is a rather uncommon congenital condition characterized by the combination of venous angioma of the leptomeninges over the cerebral cortex with ipsilateral angiomatous lesions of the face, and sometimes the skull, jaws, and oral soft tissues. A case of portwine stain with intraoral gingival hemangioma is presented. There were no other systemic manifestations. Patient reported with a complaint of localized tumor-like swelling in gums. Based on the presence of sharply demarcated vascular lesion unilaterally on the face and with ipsilateral intraoral vascular hyperplasia in the lip and gingiva, a variant of encephalotrigeminal angiomatosis was diagnosed. Ultrasound Doppler flowmetry was used to determine the blood flow. Dental management included plaque control instructions, scaling, root planning, and excision of the lesion done under general anesthesia. Close follow-up and meticulous plaque control have kept the oral condition under fairly good control.

  8. Bureaucracy and Education: an analysis since Max Weber

    Directory of Open Access Journals (Sweden)

    João Alfredo Costa de Campos Melo Júnior

    2010-06-01

    Full Text Available This paper aims at providing a discussion on bureaucracy and education based on the comprehensive sociology developed by Weber. The choice of Max Weber seems to be the most appropriate due the diversity and depth of his intellectual production, what includes the issue of bureaucracy. The use of Weber should serve as a precise instrument of conceptual analysis on the theme addressed here. Certainly, this thinker has been one of the main scholars on bureaucracy, with focus on Germany at the time of the First World War (1914-1918. It is within such a productive theoretical framework that we intend to provide a few reflections about education and bureaucracy from a Weberian epistemological base.

  9. Max Weber's Critique of the Bureaucratisation of Education

    Science.gov (United States)

    Madan, Amman

    2014-01-01

    In this commentary, the author discusses the critique by Max Weber and his views on bureaucratisation of education. The modern school, said Max Weber (1864-1920) over a hundred years ago, has as its educational ideal the bureaucrat and no longer the cultivated elite of older times. The shift to modernity and to its characteristic institution, the…

  10. Síndrome de Sturge weber. Revisión

    Directory of Open Access Journals (Sweden)

    Otman Fernández Concepción

    1999-09-01

    Full Text Available La angiomatosis encefalotrigeminal (síndrome de Sturge-Weber es el más frecuente de los síndromes neurocutáneos con predominio de anomalías vasculares. Es una enfermedad congénita, que aparece de manera esporádica y en su forma completa consiste en la asociación de angiomas cerebral, cutáneo y ocular que se caracterizan clínicamente por una mancha color vino en la cara, epilepsia, retraso mental, déficits neurológicos (hemiparesia y hemianopsia y glaucoma. Para el diagnóstico de la enfermedad resultan de mucha utilidad los estudios de tomografía axial computadorizada y resonancia magnética nuclear craneales, así como para estudiar la relación del angioma leptomeníngeo u atrofia subyacente, importantes para el pronóstico y la conducta médica que se debe seguir. El control de las crisis epilépticas constituye un elemento capital para una evolución favorable de los pacientes. El tratamiento quirúrgico debe ser reservado para enfermos de corta edad, con afectación unilateral exclusiva, con epilepsia refractaria y sin retraso mental profundo; mediante la resección del área afectada, lobectomía o hemisferectomía, siempre que el otro hemisferio esté intacto. Se profundiza en los aspectos clínicos, diagnósticos, patogénicos y terapéuticos de esta enfermedadThe encephalotrigeminal angiomatosis (Sterge-Weber syndrome is the commonest of the neurocutaneous syndromes with predominance of vascular anomalies. Its complete form consists in the association of cerebral, cutaneous and ocular angiomas, which are characterized by a wine-clored stain, epilepsy, mental retardation, neurological deficits (hemiparesis and hemianopsia and glaucoma. CAT and cranial magnetic resonance imaging are very useful for diagnosing the disease and for studying the extension of the leptomeningeal angioma or underlying atrophy. These studies are also important for the prognosis and medical conduct to be followed. The control of the epileptic seizures

  11. Os sentidos de compreensão nas teorias de Weber e Habermas The meanings of understanding in the theories of Weber and Habermas

    Directory of Open Access Journals (Sweden)

    José Geraldo A. B. Poker

    2013-01-01

    Full Text Available Partindo do pressuposto de que a teoria social elaborada por Habermas em muito se assemelha àquela construída por M. Weber, procedeu-se a um estudo comparativo com a intenção de identificar as formas pelas quais Weber e Habermas elaboraram o conceito de compreensão, ao mesmo tempo em que e o elegeram, cada um a seu modo, como instrumento metodológico adequado às dificuldades da produção de conhecimento científico nas Ciências Sociais. Tanto para Weber, como para Habermas, o conhecimento nas Ciências Sociais não consegue escapar das influências diretas da subjetividade do cientista, como também não é capaz de se proteger das contingências histórico-culturais aos quais inevitavelmente toda ação humana está vinculada. Por isso, fundamentados em suas próprias razões, tanto Weber quanto Habermas apontam a compreensão como a forma possível de conhecimento, o que implica a renúncia às pretensões explicativas e à produção de teorias gerais de fundamentação última, que são típicas das ciências convencionais.Assuming that the social theory developed by Habermas is very similar to that constructed by M. Weber, this article performs a comparative study with the intention of identifying the ways in which Weber and Habermas built the concept of understanding. Both authors chose this concept, each in his own way, as a methodological tool appropriate to the difficulties of the production of scientific knowledge in the social sciences. As much for Weber as for Habermas, knowledge in the social sciences cannot escape the direct influences of the subjectivity of the scientist, as it also cannot protect itself from the historical and cultural contingencies to which every human action is inevitably linked. Therefore, based on their own reasons, both Weber and Habermas point to understanding as the possible form of knowledge, which implies the renunciation of the explanatory pretensions and general theories of ultimate

  12. Sturge-Weber syndrome. The current neuro-imaging data

    International Nuclear Information System (INIS)

    Boukobza, M.; Cambra, M.R.; Merland, J.J.; Enjolras, O.

    2000-01-01

    Sturge-Weber syndrome (SWS) is a rare congenital sporadic disease with neuro-ocular and cutaneous vascular findings. Clinically, the full-blown condition consists of a facial port-wine stain (PWS) the V1 facial trigeminal skin area, alone or in combination with V2 and V3 PWS, seizures and ocular abnormalities (glaucoma and choroidal angioma). Radiologically, a leptomeningeal (pial) capillary and venous malformation, mostly located in the parieto-occipital area, cerebral atrophy and calcifications are demonstrated. An ipsilateral enlarged choroid plexus may be an early anatomic symptom. Development neuro-diagnostic technique for the screening of infants with an at-risk V1 PWS, as well as for the follow-up of patients with evidence SWS. Accelerated myelination in the involved hemisphere may be early diagnostic feature before 6 months of age. Later, hyperintensity of white matter on T2 is considered a symptom of gliosis. Clinically, progression of the diseases is associated with anatomic changes and correlates with the extent of the pial vascular anomaly, extent and severity of cerebral atrophy, and white matter abnormalities. A neonatal neuro-imaging work-up, using CT or MRI, may not demonstrate the pial anomaly and should be repeated after 6 to 12 months in an at-risk infant with V1 PWS. (authors)

  13. The theoretical root of Karl Jaspers' General Psychopathology. Part 2: The influence of Max Weber.

    Science.gov (United States)

    Kumazaki, Tsutomu

    2013-09-01

    The present study explores and compares Jaspers' methodology of psychopathology with Weber's methodology of sociology. In his works, Weber incorporated the arguments of many other researchers into his own methodology. Jaspers respected Weber as a mentor and presented arguments that were very similar to Weber's. Both Weber and Jaspers began from empathic understanding, but at the same time aimed for a rational and ideal-typical conceptualization. In addition, their methodologies were similar with respect to their detailed terminology. Such similarities cannot be seen with any other scholars. This suggests that Weber may have played an integral role as a mediator between his contemporary scholars and Jaspers. Thus, Weber may have had the most significant influence on Jaspers.

  14. Weber and Kafka: The rational and the enigmatic bureaucracy

    DEFF Research Database (Denmark)

    Beck Jørgensen, Torben

    2012-01-01

    Max Weber’s and Franz Kafka’s respective understandings of bureaucracy are as different as night and day. Yet, Kafka’s novel The Castle is best read with Max Weber at hand. In fact, Kafka relates systematically to all the dimensions in Weber’s ideal type of bureaucracy and give us a much......’s painting: Ascending and Descending. Nevertheless, Weber and Kafka can both be right. While Kafka looks at the bureaucratic phenomenon through persons who are marginalized, Weber’s perspective is historic-comparative and top-down. Are the observations of the one more correct than the other? The question...... is meaningless. As two opposite poles, Weber and Kafka ‘magnetize’ each other....

  15. Two-body problem for Weber-like interactions

    International Nuclear Information System (INIS)

    Clemente, R.A.; Assis, A.K.T.

    1991-01-01

    The problem of two moving bodies interacting through a Weber-like force is presented. Trajectories are obtained analytically once relativistic and quantic considerations are neglected. The main results are that in the case of limited trajectories, in general, they are not closed and in the case of open trajectories, the deflection angles are not the same for similar particles with given energies and angular momenta but opposite potentials. This last feature suggests the possibility of a direct verification of the validity of Weber's law of force for electromagnetic interactions

  16. Max Weber o el enigma emocional del origen del capitalismo

    OpenAIRE

    EDUARDO BERICAT ALASTUEY

    2001-01-01

    Se analiza el trabajo clave de Max Weber, La ética protestante y el espíritu del capitalismo, desde la perspectiva de la Sociología de las Emociones. Aunque la inclusión de las emociones de los fenómenos sociales es clave para entender la idea de Weber sobre el dogma de la predestinación, los sociólogos han trivializado el papel de la emoción. Tras detallar la creencia de Weber en que el espíritu del capitalismo parte del conocimiento de que el destino del hombre está predeterminado (la ética...

  17. The ontology of the questionnaire: Max Weber on measurement and mass investigation.

    Science.gov (United States)

    Brain, R M

    2001-12-01

    Although contemporary sociologists of science have sometimes claimed Max Weber as a methodological precursor, they have not examined Weber's own writings about science. Between 1908 and 1912 Weber published a series of critical studies of the extension of scientific authority into public life. The most notable of these concerned attempts to implement the experimental psychology or psycho-physics laboratory in factories and other real-world settings. Weber's critique centered on the problem of social measurement. He emphasized the discontinuities between the space of the laboratory and that of the factory, showing how several qualitative and historically conditioned differences between the two settings rendered the transfer of instruments and methods between them highly problematic. Weber's critical arguments prepared the ground for his greatest foray into empirical sociology, a survey he directed for the Verein für Sozialpolitik investigating the conditions and attitudes affecting the lives and performance of industrial workers. Using a different measuring instrument - the questionnaire - Weber tried to implement a concept of social measurement which implied a different ontology, drawn not from natural sciences but from the historical sciences. Copyright 2001 Published by Elsevier Science Ltd.

  18. Reliability Of Kraus-Weber Exercise Test As An Evaluation Tool In ...

    African Journals Online (AJOL)

    Reliability Of Kraus-Weber Exercise Test As An Evaluation Tool In Low Back ... strength and flexibility of the back, abdominal, psoas and hamstring muscles. ... Keywords: Kraus-Weber test, low back pain, muscle flexibility, muscle strength.

  19. Sturge-Weber syndrome with no leptomeningeal enhancement on MRI

    Energy Technology Data Exchange (ETDEWEB)

    Fischbein, N.J.; Barkovich, A.J. [Department of Radiology, San Francisco, CA (United States); Wu, Y.; Berg, B.O. [Department Pediatric Neurology, University of California, San Francisco, California (United States)

    1998-03-01

    Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome characterized by a facial nevus flammeus associated with seizures, developmental delay, and, often, with hemiparesis and hemianopia. On MRI, the most characteristic finding has been reported to be leptomeningeal enhancement, believed to represent leakage of contrast medium through the anomalous pial vessels that characterize the disease. We present a case of SWS with no evidence of leptomeningeal enhancement. This case illustrates that leptomeningeal enhancement need not be present in SWS, and the absence of this characteristic finding does not preclude the diagnosis. (orig.) With 2 figs.

  20. Anaesthesia for caesarean section in a patient with Sturge-Weber syndrome following acute neurological deterioration.

    LENUS (Irish Health Repository)

    Tadrous, R

    2011-07-01

    Sturge-Weber syndrome consists of facial capillary malformation (port-wine stain) and abnormal blood vessels in the brain or eye. Seizures, developmental delay and intracranial and airway angiomata are principal concerns. We report a 28-year-old primiparous woman at 41 weeks of gestation with Sturge-Weber syndrome who developed unilateral weakness, aphasia, blurred vision and confusion. Preeclampsia was excluded. Neuroimaging showed left sided cerebral oedema and a right parieto-occipital lesion, most likely an angioma. Caesarean section was planned to avoid the risk of angioma rupture during labour. General anesthesia was avoided due to the haemodynamic response to laryngoscopy and reports of seizure-related mortality. Despite the possibility of raised intracranial pressure and precipitation of cerebral herniation, a lumbar epidural block was administered but failed. A subarachnoid block was successfully performed and a healthy infant delivered. The choice of anaesthesia was strongly influenced by detailed radiological investigations and multidisciplinary participation.

  1. Anaesthesia for caesarean section in a patient with Sturge-Weber syndrome following acute neurological deterioration.

    LENUS (Irish Health Repository)

    Tadrous, R

    2012-02-01

    Sturge-Weber syndrome consists of facial capillary malformation (port-wine stain) and abnormal blood vessels in the brain or eye. Seizures, developmental delay and intracranial and airway angiomata are principal concerns. We report a 28-year-old primiparous woman at 41 weeks of gestation with Sturge-Weber syndrome who developed unilateral weakness, aphasia, blurred vision and confusion. Preeclampsia was excluded. Neuroimaging showed left sided cerebral oedema and a right parieto-occipital lesion, most likely an angioma. Caesarean section was planned to avoid the risk of angioma rupture during labour. General anesthesia was avoided due to the haemodynamic response to laryngoscopy and reports of seizure-related mortality. Despite the possibility of raised intracranial pressure and precipitation of cerebral herniation, a lumbar epidural block was administered but failed. A subarachnoid block was successfully performed and a healthy infant delivered. The choice of anaesthesia was strongly influenced by detailed radiological investigations and multidisciplinary participation.

  2. MARX AND WEBER - POSSIBLE AFFINITIES IN LEGAL SOCIOLOGY

    Directory of Open Access Journals (Sweden)

    Daniel Nunes Pereira

    2015-12-01

    Full Text Available This paper aims to draw parallels between the works of Max Weber and Karl Marx, demontrating their acquiescences and objections on Law and State. This study assumes that both authors are, each in their measures, critics of capitalism and the society that forms around its Mode of production. For such, this paper attempts to analyze the similarities, disagreements and parallel discussions between Marx and Weber regarding to outline what would be a so called possible "Weberian Marxism", and how it epistemologically occurs on Legal Sociology.

  3. Maladie de Rendu-Osler: un diagnostic à ne pas méconnaitre ...

    African Journals Online (AJOL)

    Homme, âgé de 78 ans, ayant des antécédents familiaux d'épistaxis chronique non explorée, avait depuis l'âge de 45 ans des épisodes d'épistaxis récidivante, bilatérale, spontanée et de faible abondance isolée. L'examen physique a objectivé des lésions cutanéo-muqueuses à type de télangiectasies au niveau des ...

  4. O pensamento de Max Weber

    Directory of Open Access Journals (Sweden)

    Maurício Tragtenberg

    1966-06-01

    Full Text Available Max Weber inicia sua carreira universitária em 1893, como professor extraordinário de Direito Mercantil e Romano, na Universidade de Berlim. Em 1894 assume a Cadeira de Economia Política na Universidade de Hamburgo e em 1897 leciona na Universidade de Heidelberg.

  5. Genealogie della legittimità. Città e Stato in Max Weber

    Directory of Open Access Journals (Sweden)

    Furio Ferraresi

    2014-05-01

    Full Text Available This essay explores the theme of the legitimacy of power in Weber from the standpoints of social science, historical investigation and political reflection. It investigates how Weber develops this theme in Kategorienaufsatz (1913, Soziologische Grundbegriffe (1920, Die Stadt (1911-14 and Politische Schriften (1917-19. Power, as a relationship of command/obedience, is founded on the representations of legitimacy of the individuals involved and on the Einverständnis (consent which is the condition of possibility of this relationship. It is back to the medieval city that Weber traces the genesis and interweaving of various forms of legitimation, which continue to exist as possibilities even within the modern State. The memory of medieval events re-emerges when Weber, after Germany’s defeat in the First World War and the threat of revolution that followed, tries to lay a foundation for the legitimacy of “leader democracy” by reintroducing the command/obedience relationship between leaders and masses.

  6. Weber's law, the magnitude effect and discrimination of sugar concentrations in nectar-feeding animals.

    Science.gov (United States)

    Nachev, Vladislav; Stich, Kai Petra; Winter, York

    2013-01-01

    Weber's law quantifies the perception of difference between stimuli. For instance, it can explain why we are less likely to detect the removal of three nuts from a bowl if the bowl is full than if it is nearly empty. This is an example of the magnitude effect - the phenomenon that the subjective perception of a linear difference between a pair of stimuli progressively diminishes when the average magnitude of the stimuli increases. Although discrimination performances of both human and animal subjects in various sensory modalities exhibit the magnitude effect, results sometimes systematically deviate from the quantitative predictions based on Weber's law. An attempt to reformulate the law to better fit data from acoustic discrimination tasks has been dubbed the "near-miss to Weber's law". Here, we tested the gustatory discrimination performance of nectar-feeding bats (Glossophaga soricina), in order to investigate whether the original version of Weber's law accurately predicts choice behavior in a two-alternative forced choice task. As expected, bats either preferred the sweeter of the two options or showed no preference. In 4 out of 6 bats the near-miss to Weber's law provided a better fit and Weber's law underestimated the magnitude effect. In order to test the generality of this observation in nectar-feeders, we reviewed previously published data on bats, hummingbirds, honeybees, and bumblebees. In all groups of animals the near-miss to Weber's law provided better fits than Weber's law. Furthermore, whereas the magnitude effect was stronger than predicted by Weber's law in vertebrates, it was weaker than predicted in insects. Thus nectar-feeding vertebrates and insects seem to differ in how their choice behavior changes as sugar concentration is increased. We discuss the ecological and evolutionary implications of the observed patterns of sugar concentration discrimination.

  7. Weber's law, the magnitude effect and discrimination of sugar concentrations in nectar-feeding animals.

    Directory of Open Access Journals (Sweden)

    Vladislav Nachev

    Full Text Available Weber's law quantifies the perception of difference between stimuli. For instance, it can explain why we are less likely to detect the removal of three nuts from a bowl if the bowl is full than if it is nearly empty. This is an example of the magnitude effect - the phenomenon that the subjective perception of a linear difference between a pair of stimuli progressively diminishes when the average magnitude of the stimuli increases. Although discrimination performances of both human and animal subjects in various sensory modalities exhibit the magnitude effect, results sometimes systematically deviate from the quantitative predictions based on Weber's law. An attempt to reformulate the law to better fit data from acoustic discrimination tasks has been dubbed the "near-miss to Weber's law". Here, we tested the gustatory discrimination performance of nectar-feeding bats (Glossophaga soricina, in order to investigate whether the original version of Weber's law accurately predicts choice behavior in a two-alternative forced choice task. As expected, bats either preferred the sweeter of the two options or showed no preference. In 4 out of 6 bats the near-miss to Weber's law provided a better fit and Weber's law underestimated the magnitude effect. In order to test the generality of this observation in nectar-feeders, we reviewed previously published data on bats, hummingbirds, honeybees, and bumblebees. In all groups of animals the near-miss to Weber's law provided better fits than Weber's law. Furthermore, whereas the magnitude effect was stronger than predicted by Weber's law in vertebrates, it was weaker than predicted in insects. Thus nectar-feeding vertebrates and insects seem to differ in how their choice behavior changes as sugar concentration is increased. We discuss the ecological and evolutionary implications of the observed patterns of sugar concentration discrimination.

  8. Bilateral Sturge-Weber Syndrome and glaucoma controlled with Ahmed valve implant

    Directory of Open Access Journals (Sweden)

    Marcelo Jarczun Kac

    2015-02-01

    Full Text Available Sturge-Weber Syndrome is a rare neuro-oculocutaneous disorder. The authors describe the case of a 13 years old boy, presented with bilateral Sturge-Weber Syndrome and glaucoma. Surgical treatment with Ahmed valve implantation in both eyes was carried out achieving lower levels of intraocular pressure.

  9. Centennial ties: Harvey Cushing (1869-1939) and William Osler (1849-1919) on Andreas Vesalius (1514-1564).

    Science.gov (United States)

    Toodayan, Nadeem

    2017-08-01

    Andreas Vesalius is often regarded as the founding father of modern anatomical study. The quincentennial anniversary of his birth - 31 December 2014 - has been very widely commemorated, and it is the purpose of this article to contrast these celebrations with what happened during the Vesalius quatercentenary year of 1914. More specifically, we look at how Vesalius was perceived a century ago by examining his influence on two of western medicine's most iconic gentlemen - Harvey Williams Cushing (1869-1939) and his larger than life mentor, Sir William Osler (1849-1919).

  10. Port-wine vascular malformations and glaucoma risk in Sturge-Weber syndrome.

    Science.gov (United States)

    Sharan, Sapna; Swamy, Brighu; Taranath, Deepa Ajay; Jamieson, Robyn; Yu, Tao; Wargon, Orli; Grigg, John R

    2009-08-01

    Treatment of the capillary vascular malformation (port-wine stain) in Sturge-Weber syndrome with the use of a laser is helpful cosmetically. However, concerns have been raised that laser obliteration of port-wine stains may result in ocular hypertension. The aim of this study was to review clinical features and management of ocular complications of SWS and assess the effects of dermatological laser treatment on the incidence of glaucoma or ocular hypertension. This retrospective cohort study was conducted in an institutional setting. All patients had involvement of the face. Patients who underwent skin laser to the port-wine vascular malformation were analyzed further. Ocular involvement, glaucoma, and skin laser treatment and the relationship to ocular hypertension/glaucoma were observed. Forty-one Sturge-Weber syndrome patients with port-wine vascular malformation were analyzed. Glaucoma was observed in 24 patients (58.5%) at mean age of 2.9 years (range, 0.0-16.5). Of these, 18 (75.0%) were treated with medical therapy, and 10 (41.7%) required trabeculectomy, with 2 of these requiring Seton implant. Of the 41 patients, 28 (68.3%) underwent laser to face/forehead. Mean age of laser commencement was 5 years (range, 0.4-16.5). Thirteen did not undergo laser treatment. Fourteen of the 28 and 10 of the 13 developed ocular hypertension/glaucoma. This retrospective review did not find evidence to suggest that laser treatment of port-wine vascular malformations causes glaucoma or that it can worsen a preexisting ocular hypertension or glaucoma. Statistical analysis was inconclusive.

  11. Hemimegalencephaly in the Klippel-Trenaunay-Weber syndrome

    International Nuclear Information System (INIS)

    Urui, Seishiro; Oi, Shizuo

    1987-01-01

    The Klippel-Trenaunay-Weber syndrome is characterized by skeletal and soft-tissue hypertrophy, segmentally distributed cutaneous nevi, and vascular anomalies. Only a few cases of this syndrome associated with hemimegalencephaly have been reported previously. However, we have experienced such a case of Klippel-Trenaunay-Weber syndrome with hemicranial hypertrophy, and so have analyzed this case from the neuroradiological point of view. The patient was a 6-month-old female with hemihypertrophy and hemimegalencephaly on the same, right side. A skull X-ray showed that the skull and facial bones were all hypertrophic on the right side and that all the sutures were open. A CT scan demonstrated that all the intracranial structures, including the ventricle on the right, were larger than those on the left. On the right side, we noticed a periventricular low-density area and spotted a calcified high-density region adjacent to the anterior horn of the lateral ventricle. Metrizamide CT cisternography exhibited ventricular stasis at 6 hours and a delayed clear-up in the subarachnoid space over the cerebral convexity in 24 hours. When we used the inversion recovery method on magnetic resonance imaging, the white matter on the right, especially in the periventricular area, was shown by a lower signal intensity than was that on the left. This could be thought to indicate the poor progress of the myelination process on the right side of the cerebrum. (author)

  12. Síndrome de Klippel-Trenaunay-Parkes-Weber com angiomatose medular Klippel-Trénaunay-Parkes-Weber syndrome with spinal cord angioma: a case report

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    James Pitagoras de Mattos

    1975-09-01

    Full Text Available É relatado um caso de síndrome de Klippel-Trénaunay-Parkes-Weber associada a angiomatose medular. O autor salienta ter encontrado na literatura somente uma referência com tal associação.A case of Klippel-Trénaunay-Parkes-Weber syndrome associated with spinal cord angioma is reported. The author points out that it was found only one reference in literature with such association.

  13. Comparison between Weber's electrodynamics and classical ...

    Indian Academy of Sciences (India)

    but not how these fields act on another test charge у moving with velocity з relative to an ... the force between two electric particles depends on their relative velocity, .... ment of magnetic quantities in the first rank of methods of precision, Weber.

  14. Temperature-touch interaction: Weber's phenomenon revisited.

    Science.gov (United States)

    Stevens, J C; Green, B G

    1978-09-01

    The six experiments reported here stemmed from Weber's (E. H. Weber, In R. Wagner (Ed.), Handwnörterbuch der Physiologie, 1846. Vol. 3, pp. 481-588) report that cold objects on the forehead feel heavier than warm ones, implying an effect of temperature on the touch modality. The experiments arrived at first-order answers to how temperature, force of stimulation, areal size of stimulation, and body locus might influence the magnitude of touch sensation. Typically, concomitant cooling greatly intensifies touch magnitude as perceived via the forehead and the forearm. Warning has little or no effect via the forehead but gives a significant intensification (less pronounced than that of cooling) on the forearm. When the areal size of the stimulation becomes very small, the intensification effects cannot be reliably demonstrated. The findings are reviewed in the light of what is known about the physiology of the cutaneous nerves.

  15. The Rationalization of Everything? Using Ritzer's McDonaldization Thesis To Teach Weber.

    Science.gov (United States)

    Lippmann, Stephen; Aldrich, Howard

    2003-01-01

    Outlines a plan for helping undergraduate students appreciate Max Weber's theoretical achievements, teaching critical thinking about what constitutes 'the good life' in rationalized societies. Uses the book "The McDonaldization of Society" (George Ritzer) to encourage student interest in Weber's work. Describes field exercises and…

  16. Max Weber in the United States

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    Lawrence A. Scaff

    2014-05-01

    Full Text Available In his contribution L. Scaff outlines the reception history of Max Weber’s work from its beginnings down to the present. It highlights the importance of the first translations, including those by prominent American authors and particularly those who had studied in Germany; and then in the post-war years the role of emigrants familiar with Weber’s work. The establishment of Weber texts as compulsory reading in the curricula of American colleges also played a significant role. The striking readiness, even ease, with which Weber was received in America is something Scaff deduces from three “narratives that captured the imagination of the American audience”. The first is the narrative of voluntarism, i.e. “the way in which Weber developed his conception of the sect and its effects on the individual and society”. Next is the narrative of achievement, in the sense of “mastery of the world”, preceded by “mastery of the self”, which “when put into practice entailed the conquest of the New World’s primordial wilderness”. Finally there is the narrative of redemption as “the most potent founding myth of the American experience”: “The quest for salvation that began as a religiously inspired message became transformed into a secularized cultural theme: the search for the possibility of breaking free from constraints in order to create a better life, to renew the self, to gain a second chance by atoning for moral failures, and to find reconciliation with God, humankind, and the world.”

  17. From Mill via von Kries to Max Weber: Causality, Explanation, and Understanding

    Science.gov (United States)

    Heidelberger, Michael

    In the second part of his "Critical studies in the logic of the cultural sciences" published in 1906, which carries the title "Objective possibility and adequate causation in historical explanation" (Weber 1906, 164-188/266-290)1 Max Weber (1864-1920) wrote that he feels "almost embarrassed in view of the extent to which here again, as in so much of the preceding argument, I am 'plundering' von Kries' ideas" (Weber 1906, 186/288).2 Weber thus admits a very strong influence on his approach by the physiologist, philosopher, and theoretician of probability, von Kries (1853-1928), who was for sometime his colleague in Freiburg in southwest Germany. Von Kries had suggested a legal criterion for attributing a deed to an agent that exerted a strong influence on German civil law and was also taken up by the legal system of other countries. This earned him the title of an honorary doctor of the law faculty of the University of Erlangen in 1897.

  18. Hemodynamics in the Sturge-Weber syndrome utilizing stable Xe-CT

    International Nuclear Information System (INIS)

    Okudaira, Yojiro; Bandoh, Kuniaki; Ito, Masanori; Sato, Kiyoshi; Tsuji, Masahiro

    1993-01-01

    In 4 Sturge-Weber-syndrome child patients with calcified lesions mainly in the occipital lobe, regional cerebral blood flow (rCBF) was determined with stable Xe-CT in the resting state and after iv injection of acetazolamide (AA) and megimide (M), with the purpose of examining factors affecting deterioration of neurologic manifestations and the influence of lobectomy. In the resting state, rCBF of the temporal and occipital areas was significantly lower on the affected than unaffected sides. The side-to-side asymmetry of rCBF in the affected and unaffected sides decreased from the frontal to temporal and occipital areas. It was indistinct after AA injection because cerebral vasoreactivity became higher on the affected side. A low rCBF in association with a high cerebral vasoreactivity on the lesion side suggested that the low rCBF matched the low cerebral metabolism of the brain area affected by leptomeningeal angiomatosis. However, cerebral vasoreactivity to AA depended on clinical presentations. Two patients presented with progressive mental retardation. The other two patients were in clinically stable condition. Cerebral vasoreactivity to AA in the former two cases was poorer on both the affected and unaffected sides than that in the latter two cases. M administration was associated with a significantly decreased rCBF in the area of leptomeningeal angiomatosis and cerebral calcification and with a significantly increased rCBF of the 'pericalcified' area. In one patient undergoing extended occipital lobectomy on the affected side, no decrease in rCBF was noted in the resting state in either affected or unaffected side, and cerebral vasoreactivity to AM on the unaffected side was apparently increased. Both circulatory disturbance and seizure appear to play a role in clinical presentations, as well as their deterioration in Sturge-Weber-Syndrome patients. (N.K.)

  19. Religião, educação e economia em Max Weber = Religion, education and the economy in Max Weber

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    Carvalho Filho, Juarez Lopes de

    2014-01-01

    Full Text Available Este artigo propõe relacionar a religião, a educação e a economia em Max Weber. Apesar de não ter escrito uma obra específica sobre a educação, é possível verificar em escritos como Confucionismo e taoísmo um esboço de tipos ideais da educação encontrados em qualquer sistema de formação, que Weber articulava aos tipos de dominação legitima (racional-legal, tradicional, carismática, seja política seja religiosa. Cada tipo de educação desenvolve nos alunos uma cultura, uma conduta de vida, ou as qualidades que convêm a um tipo de dominação político-religiosa. Esses elementos dos tipos ideais de educação articulados a uma cultura religiosa exercem um papel importante na formação de sistemas econômicos, notadamente de uma cultura capitalista. Como Weber demonstrou na Ética protestante e o “espírito” do capitalismo, existe uma real adaptação, de uma educação religiosa (ascética ao capitalismo e à sua cultura

  20. Weber's Critique of Advocacy in the Classroom: Critical Thinking and Civic Education.

    Science.gov (United States)

    Weaver, Mark

    1998-01-01

    Discusses the four aspects of Max Weber's argument against including advocacy in the political science classroom. Believes that Weber's critique is a useful starting point for considering the issue in relation to contemporary education. Describes two models, critical thinking and civic education, that present advocacy in the political science…

  1. Um mapeamento das aproximações entre Weber e Nietzsche

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    Leandro dos Santos

    2014-06-01

    Full Text Available Este artigo tem por objetivo um levantamento dos principais tópicos de aproximação entre a Sociologia de Max Weber (1864-1920 e a Filosofia de Friedrich W. Nietzsche (1844-1900. Para tanto, sublinharam-se os pontos mais expressivos e correntemente observados na literatura acerca das aproximações entre Weber e Nietzsche. Nesse sentido, os traços de maior afinidade foram encontrados nos seguintes temas: poder, conhecimento, modernidade e ética.

  2. Weber on Education and Its Administration: Prospects for Leadership in a Rationalized World.

    Science.gov (United States)

    Samier, Eugenie

    2002-01-01

    Uses Max Weber's writings to reconstruct his views on education, administration, and leadership; relates these views to some recent significant changes in education, especially higher education. Discusses problems of educational leadership in modern universities from a Weberian perspective. Provides three conclusions from Weber's writing; draws…

  3. Rectal and splenic vascular malformation in klippel trenaunay weber syndrome: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ha Youn; Chang, Yun Woo; Lee, Dong Hwan [Soonchunhyang Univ. Hospital, Seoul (Korea, Republic of)

    2012-10-15

    Klippel Trenaunay Weber syndrome (KTWS) is a rare congenital disorder, characterized by a cutaneous vascular nevus of the involved extremity, vascular malformations, bone and soft tissue hypertrophy of the extremity. We present the case of an 18 year old female patient with KTWS, showing a marked rectosigmoid wall thickening and phlebolith, and also variable sized cystic masses in the spleen, as a result of vascular malformations.

  4. Comparison between Weber's electrodynamics and classical ...

    Indian Academy of Sciences (India)

    We analyse the relation between Weber's law and Maxwell's equations. Finally, .... which is so elegant, so mathematical, and so entirely different from anything in this paper, that I ... In these theories the force acting between the two bodies is treated with refer- .... The formula of Gauss is inconsistent with this principle, and.

  5. [Psychiatry as cultural science: considerations following Max Weber].

    Science.gov (United States)

    Bormuth, M

    2010-11-01

    Psychiatry can be seen as a natural and cultural science. According to this the postulate of freedom is its strong value judgment. Since the times of enlightenment it has been described metaphorically by the myth of the expulsion from Paradise. Following Max Weber and Wilhelm Dilthey, Karl Jaspers has introduced this perspective into psychiatry. His strict dichotomy between explaining and understanding has later been critically revised by Werner Janzarik and Hans Heimann. Their concepts of structure dynamic, of pathography and of anthropology are closer to Max Weber who connected natural and cultural sciences in a much stronger way. Especially the pathographic example of Nietzsche allows to demonstrate the differences between Jaspers and the later psychopathologists of the Heidelberg and Tübingen schools.

  6. Acercamiento a la actualidad de Max Weber

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    Javier E. Ortiz Cárdenas

    2012-01-01

    Full Text Available El acercamiento a Weber, como científico social, nos permite encontrar aportaciones teóricas, de método, enfoques y estudios comparativos para entender el funcionamiento de instituciones actuales, tal es el cometido del presente artículo.

  7. Max Weber e a crise do liberalismo

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    Ana Cleide Chiarotti Cesário

    2004-07-01

    Full Text Available This article tries to show how Weber perceived the crisis of liberalism based on the analysis of the social and political conditions in Germany. Following Weberian thought, it seeks to indicate the shape of a political democracy in a "post-bourgeois society".

  8. Diffuse choroidal haemangioma in Sturge-Weber syndrome treated with photodynamic therapy under general anaesthesia

    NARCIS (Netherlands)

    Huiskamp, EA; Muskens, RPHM; Ballast, A; Hooymans, JMM

    Purpose: To report the treatment outcome of photodynamic therapy with verteporfin (PDT) for exudative retinal detachment associated with diffuse choroidal haemangioma in Sturge-Weber syndrome. Methods: An interventional case report of a 12-year-old girl with Sturge-Weber syndrome who developed an

  9. Multimodal Imaging in Klippel-Trénaunay-Weber Syndrome: Clinical Photography, Computed Tomoangiography, Infrared Thermography, and 99mTc-Phytate Lymphoscintigraphy.

    Science.gov (United States)

    Kim, Su Wan; Song, Heesung

    2017-12-01

    We report the case of a 19-year-old man who presented with a 12-year history of progressive fatigue, feeling hot, excessive sweating, and numbness in the left arm. He had undergone multimodal imaging and was diagnosed as having Klippel-Trénaunay-Weber syndrome (KTWS). This is a rare congenital disease, defined by combinations of nevus flammeus, venous and lymphatic malformation, and hypertrophy of the affected limbs. Lower extremities are affected mostly. Conventional modalities for evaluating KTWS are ultrasonography, CT, MRI, lymphoscintigraphy, and angiography. There are few reports on multimodal imaging of upper extremities of KTWS patients, and this is the first report of an infrared thermography in KTWS.

  10. Max Weber's Contribution to the Sociology of Education: A Critical Appreciation

    Science.gov (United States)

    Rao, S. Srinivasa; Singh, Smriti

    2018-01-01

    Karl Emil Maximilian Weber (1864-1920), more widely known as Max Weber, is credited with numerous contributions to modern sociology and is considered one of the pillars of the discipline along with Karl Marx and Emile Durkheim. Marx (1818-1883) was an established predecessor from Germany whose study of the nineteenth-century European society was…

  11. Os sentidos de compreensão nas teorias de Weber e Habermas

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    José Geraldo A. B. Poker

    2013-01-01

    Full Text Available Partindo do pressuposto de que a teoria social elaborada por Habermas em muito se assemelha àquela construída por M. Weber, procedeu-se a um estudo comparativo com a intenção de identificar as formas pelas quais Weber e Habermas elaboraram o conceito de compreensão, ao mesmo tempo em que e o elegeram, cada um a seu modo, como instrumento metodológico adequado às dificuldades da produção de conhecimento científico nas Ciências Sociais. Tanto para Weber, como para Habermas, o conhecimento nas Ciências Sociais não consegue escapar das influências diretas da subjetividade do cientista, como também não é capaz de se proteger das contingências histórico-culturais aos quais inevitavelmente toda ação humana está vinculada. Por isso, fundamentados em suas próprias razões, tanto Weber quanto Habermas apontam a compreensão como a forma possível de conhecimento, o que implica a renúncia às pretensões explicativas e à produção de teorias gerais de fundamentação última, que são típicas das ciências convencionais.

  12. Max Weber: o processo de racionalização e o desencantamento do trabalho nas organizações contemporâneas Max Weber: rationalization process and the disenchantment of work in contemporary organizations

    Directory of Open Access Journals (Sweden)

    Hermano Roberto Thiry-Cherques

    2009-08-01

    Full Text Available Este artigo indica algumas implicações do pensamento weberiano sobre a compreensão do trabalho e da forma de administrá-lo. Com base nos conceitos weberianos de racionalidade e de racionalização, é feita uma apreciação do trabalho na história ocidental, com ênfase no momento da passagem do capitalismo tradicional para o contemporâneo. A tese central é a de que as teorias de Weber são um modo válido para compreender o trabalho desencantado da atualidade.This article discusses some implications of Weber's thoughts about understanding and managing labor. Based on Weber's concepts of rationality and rationalization, the article develops an account of the transformations of labor in Western history, stressing the shift from early to contemporary capitalism. The central thesis is that Weber's theories are a well grounded way to explain today's disenchanted work.

  13. Weber, Nietzsche and the ethical answers to the critique of modernity

    OpenAIRE

    Nobre, Renarde Freire

    2003-01-01

    O artigo consiste num estudo comparativo entre as concepções de cultura de Friedrich Nietzsche e Max Weber, suas análises críticas da modernidade e as respostas éticas que as acompanham. Apesar das importantes afinidades encontradas entre os autores, o artigo procura mostrar que o caráter principal da comparação entre os seus pensamentos é o do desencontro e da incompatibilidade.The article consistes in a comparative study between Friedrich Nietzsche's and Max Weber's conceptions of culture, ...

  14. Max Weber and the atom of Sociology: a moderate methodological individualism?

    OpenAIRE

    Sell, Carlos Eduardo

    2016-01-01

    Qual a natureza do individualismo metodológico de Max Weber? Para esclarecer essa questão o artigo revisa o debate entre holistas e individualistas, destacando as tentativas de aproximação entre essas posições. Em seguida, com base em Economia e sociedade, busca-se determinar, de maneira imanente, a compreensão weberiana sobre o individualismo como método. Na parte final discutem-se criticamente as tentativas de releitura da Sociologia de Max Weber à luz da controvérsia entre as perspectivas ...

  15. Evaluation of the syndesmotic-only fixation for Weber-C ankle fractures with syndesmotic injury.

    Science.gov (United States)

    Mohammed, R; Syed, S; Metikala, S; Ali, Sa

    2011-09-01

    With the length of the fibula restored and the syndesmosis reduced anatomically, internal fixation using a plating device may not be necessary for supra-syndesmotic fibular fractures combined with diastasis of inferior tibio-fibular joint. A retrospective observational study was performed in patients who had this injury pattern treated with syndesmosis-only fixation. 12 patients who had Weber type-C injury pattern were treated with syndesmosis only fixation. The treatment plan was followed only if the fibular length could be restored and if the syndesmosis could be anatomically reduced. Through a percutaneous or mini-open reduction and clamp stabilization of the syndesmosis, all but one patient had a single tricortical screw fixation across the syndesmosis. Patients were kept non-weight-bearing for 6 weeks, followed by screw removal at an average of 8 weeks. Outcomes were assessed using an objective ankle scoring system (Olerud and Molander scale) and by radiographic assessment of the ankle mortise. At a mean follow-up of 13 months, the functional outcome score was 75. Excellent to good outcomes were noted in 83% of the patients. Ankle mortise was reduced in all cases, and all but one fibular fracture united without loss of fixation. Six patients had more than one malleolar injury, needing either screw or anchor fixations. One patient had late diastasis after removal of the syndesmotic screw and underwent revision surgery with bone grafting of the fibula. This was probably due to early screw removal, before union of the fibular fracture had occurred. We recommend syndesmosis-only fixation as an effective treatment option for a combination of syndesmosis disruption and Weber type-C lateral malleolar fractures.

  16. THE WEBER THESIS OF CALVINISM AND CAPITALISM--ITS VARIOUS VERSIONS AND THEIR "FATE" IN SOCIAL SCIENCE.

    Science.gov (United States)

    Zafirovski, Milan

    2016-01-01

    The paper identifies and examines various multiple renditions of the so-called Weber Thesis of an historical association and convergence between ascetic Protestantism, above all Calvinism, and the emergence and development of modern capitalism as an economic spirit and system. Specifically, it detects at least four different versions and formulations or interpretations, thus casting doubt in the common view of the Weber Thesis as a single and monolithic theory or hypothesis. The paper also considers the status of the multiple versions of the Weber Thesis in post-Weberian and contemporary sociology and related disciplines like economics and history. It concludes that the weaker, relaxed renditions of the Weber Thesis have attained a greater success and more endured in contemporary social science than have its stronger, stricter versions. © 2015 Wiley Periodicals, Inc.

  17. Unusual presentation of Sturge-Weber syndrome: Progressive megalencephaly with bilateral cutaneous and cortical involvement

    Directory of Open Access Journals (Sweden)

    Kundan Mittal

    2014-01-01

    Full Text Available The Sturge Weber syndrome is characterized by developmental delay, seizures in infancy, unilateral cutaneous lesions with ipsilateral leptomeningeal enhancement. We report an unusual presentation of Sturge Weber syndrome with bilateral port wine nevus on the trunk and face along with bilateral cortical involvement in a developmentally normal child with progressive megalencephaly.

  18. Environmental Setting of the Sugar Creek and Leary Weber Ditch Basins, Indiana, 2002-04

    Science.gov (United States)

    Lathrop, Timothy R.

    2006-01-01

    The Leary Weber Ditch Basin is nested within the Sugar Creek Basin in central Indiana. These basins make up one of the five study sites in the Nation selected for the Agricultural Chemicals: Sources, Transport, and Fate topical study, a part of the U.S. Geological Survey’s National Water-Quality Assessment Program. In this topical study, identifying the natural factors and human influences affecting water quality in the Leary Weber Ditch and Sugar Creek Basins are the focus of the assessment. A detailed comparison between the environmental settings of these basins is presented. Specifics of the topical study design as implemented in the Leary Weber Ditch and Sugar Creek Basins are described.

  19. A traumatic case of Weber's syndrome

    International Nuclear Information System (INIS)

    Shirakuni, Takayuki; Tamaki, Norihiko; Matsumoto, Satoshi; Ishida, Kazuhiko; Fujiwara, Masayasu.

    1983-01-01

    The present authors encountered a rare traumatic case of Weber's syndrome in which computerized tomography (CT) revealed a hematoma localized in the tegmentum of the midbrain. This 49-year-old male was admitted because of a disturbed consciousness level caused by a hit on the right frontal area in a traffic accident. A neurological examination on admission disclosed Weber's syndrome: a dilatation of the right pupil, no reaction to direct or indirect light stimuli, ptosis of the right eyelid, lateral deviation of the right eyeball, and left hemiparesis with increased deep tendon reflexes and Babinski's sign. CT revealed a high-density area localized in the tegmentum of the midbrain, but interpeduncular and quadrigeminal cisterns were patent. After admission, his motor function and level of consciousness have showed improvement with medical treatment, but his right oculomotor palsy is unchanged. CT performed 7 days after admission showed a low-density area in place of the absorbed hematoma. In this case, we cannot explain the mechanism of the primary brain-stem injury. However, there are four possible mechanisms: nerve avulsion, neurovascular friction, localized contusion, and shear strain injury. Careful consideration of the anatomical relationship shows that it is likely that the lesion was a contusional hemorrhage caused by impact on the tentorial edge. (author)

  20. An unusual presentation of pediatric osteoblastoma in a patient with Klippel-Trenaunay-Weber syndrome: case report.

    Science.gov (United States)

    Wallace, Scott A; Ignacio, Romeo C; Klugh, Arnett; Gates, Gregory; Henry, Marion C W

    2015-06-01

    Osteoblastoma is an uncommon primary bone tumor that usually presents as a painful lesion in a long bone or in the spine. Osteoblastoma has been reported only twice in the literature in conjunction with systemic fibromatosis. The authors report the case of an 8-year-old girl with suspected Klippel-Trenaunay-Weber syndrome, a rare syndrome of systemic fibromatosis, who presented with a painless thoracic rib lesion that was found to be an osteoblastoma.

  1. Weber, authority and the organisation of health care.

    Science.gov (United States)

    Alaszewski, A; Manthorpe, J

    The third paper in the series on sociology discusses the work of Max Weber. It traces the origins and main themes of his work. The parallels between his work and contemporary issues in the organisation of health care are outlined, in particular, the insights provided into bureaucracy and authority.

  2. Weber, Nietzsche e as respostas éticas à crítica da modernidade Weber, Nietzsche and the ethical answers to the critique of modernity

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    Renarde Freire Nobre

    2003-01-01

    Full Text Available O artigo consiste num estudo comparativo entre as concepções de cultura de Friedrich Nietzsche e Max Weber, suas análises críticas da modernidade e as respostas éticas que as acompanham. Apesar das importantes afinidades encontradas entre os autores, o artigo procura mostrar que o caráter principal da comparação entre os seus pensamentos é o do desencontro e da incompatibilidade.The article consistes in a comparative study between Friedrich Nietzsche's and Max Weber's conceptions of culture, their critical analyses of modernity and the ethical answers that follow from them. In spite of the important affinities between the two authors, the article tries to show that the main character of the comparison is that of the divergence and incompatibility.

  3. Hipoxemia por fístulas artério-venosas pulmonares em criança: relato de caso Hipoxemia por fístulas artério-venosas pulmonares en niño: relato de caso Hypoxemia by pulmonary arteriovenous fistulae in childhood: case report

    Directory of Open Access Journals (Sweden)

    Aleksandra Paula Lima

    2004-08-01

    ístulas pulmonares o en otros órganos podrán surgir, caracterizando la síndrome de Rendu-Osler-Weber.BACKGROUND AND OBJECTIVES: Pulmonary arteriovenous fistulae (PAVF should be investigated in patients with cyanosis of unknown cause. This is a case of cyanosis in a child submitted to pulmonary lobectomy with PAVF. CASE REPORT: Male patient, 3 years old, with history of cyanosis without dyspnea since 8 months of age. He presented significant cyanosis, finger clubbing and normal heart auscultation. Chest X-ray showed condensation in the upper half of the left lung. Normal echocardiography and ECG. PaO2 = 28 mmHg in room air and PaO2 = 31.5 mmHg with nasal O2 catheter. Pulmonary arteriovenous fistula was diagnosed through magnetic resonance imaging, with no possibility of embolization. Patient was submitted to upper left lobectomy under general anesthesia associated to spinal anesthesia with morphine and bupivacaine. Selective tracheal tube for the right lung was inserted for monopulmonary ventilation. SaO2 was 59% in room air; at operating room admission 69% (FiO2 = 1.0; after general anesthesia induction 65% (FiO2 = 1.0; during monopulmonary ventilation 58% (FiO2 = 1.0, after lobectomy 98% (FiO2 = 1.0 and 98% at the end of the surgery (FiO2 = 0.6. Extubation was performed one hour after surgery completion. As from the fifth postoperative day patient started presenting progressive SpO2 decrease down to 83% due to increase of another PAVF, which was embolized under general anesthesia. Patient was discharged on the 15th PO day. CONCLUSIONS: In this case, there was poor preoperative response to oxygen therapy, and patient has immediately improved after surgical treatment. However, after surgical resection of the largest PAVF, another fistula increased, thus leading to decrease in postoperative SpO2, reverted by embolization. As the child grows, other pulmonary fistula or fistulae in other organs may be diagnosed, indicating Rendu-Osler-Weber Syndrome.

  4. Max Weber o el enigma emocional del origen del capitalismo

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    EDUARDO BERICAT ALASTUEY

    2001-01-01

    Full Text Available Se analiza el trabajo clave de Max Weber, La ética protestante y el espíritu del capitalismo, desde la perspectiva de la Sociología de las Emociones. Aunque la inclusión de las emociones de los fenómenos sociales es clave para entender la idea de Weber sobre el dogma de la predestinación, los sociólogos han trivializado el papel de la emoción. Tras detallar la creencia de Weber en que el espíritu del capitalismo parte del conocimiento de que el destino del hombre está predeterminado (la ética protestante, se destaca que la angustia, más específicamente la humillación, invocada por esta creencia, debe figurar dentro de cualquier teoría acerca de la cultura. El sentimiento de humillación es explorado a través de los componentes cognitivos, evaluativos y emotivos de la cultura, proporcionando una mejor comprensión de la omnisciencia divina, la falta de respeto que la humanidad se merece y la ausencia de felicidad. A causa de la humillación y la falta de dignidad sufrida en muchos niveles, el hombre busca el orgullo personal, el éxito mundano y la modernidad, olvidando la humildad, la salvación eterna y la tradición.

  5. Historical Paths and Intellectual Projects: The Case of Max Weber

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    Sven Eliaeson

    2017-06-01

    Full Text Available Max Weber is an elusive classic, and competing traditions claim his legacy. As in other cases, the interpretation of this classic should take into account formative experiences, historical context and links to older traditions. Weber theorized rational modernity, albeit with a certain ambiguity (the “iron cage”. He reworked the legacy of German historicism, but took it in the direction of methodological individualism; at the same time, and through the very affiliation to historicism, he contributed to German nation-building. His emphasis on the state as a necessary unit of analysis now seems obsolete, and so does his inclination to take the imperialistic dominance of the European great powers for granted. He was not immune to the ideology that opposed Germany’s cultural calling to Western civilization and Russian barbarism.

  6. Hemiparesis is a clinical correlate of general adaptive dysfunction in children and adolescents with Sturge-Weber syndrome.

    Science.gov (United States)

    Reesman, Jennifer; Gray, Robert; Suskauer, Stacy J; Ferenc, Lisa M; Kossoff, Eric H; Lin, Doris D M; Turin, Elizabeth; Comi, Anne M; Brice, Patrick J; Zabel, T Andrew

    2009-06-01

    This study sought to identify neurologic correlates of adaptive functioning in individuals with Sturge-Weber syndrome. A total of 18 children, adolescents, and young adults with Sturge-Weber syndrome with brain involvement were recruited from our Sturge-Weber center. All underwent neurologic examination (including review of clinical brain magnetic resonance imaging) and neuropsychological assessment. Neuropsychological assessment included measures of intellectual ability and standardized parent report of adaptive functioning. Overall, Full Scale IQ and ratings of global adaptive functioning were both lower than the population-based norms (P adaptive functioning ratings, clinician ratings of cortical abnormality, and ratings of neurologic status. Hemiparesis (minimal versus prominent) was the only individual component of the rating scales that differentiated between individuals with nonimpaired and impaired adaptive functioning scores. Information obtained during neurological examination of children and adolescents with Sturge-Weber syndrome particularly hemiparetic status is useful for identifying children who may need additional intervention.

  7. Reproducibility of the Lauge-Hansen, Danis-Weber, and AO classifications for ankle fractures

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    Lucas Lopes da Fonseca

    Full Text Available ABSTRACT Objective: This study evaluated the reproducibility of the three main classifications of ankle fractures most commonly used in emergency clinical practice: Lauge-Hansen, Danis-Weber, and AO-OTA. The secondary objective was to assess whether the level of professional experience influenced the interobserver agreement for the classification of this pathology. Methods: The study included 83 digitized preoperative radiographic images of ankle fractures, in anteroposterior and lateral views, of different adults that had occurred between January and December 2013. For sample calculation, the estimated accuracy was approximately 15%, with a sampling error of 5% and a sampling power of 80%. The images were analyzed and classified by six different observers: two foot and ankle surgeons, two general orthopedic surgeons, and two-second-year residents in orthopedics and traumatology. The Kappa statistical method of multiple variances was used to assess the variations. Results: The Danis-Weber classification indicated that 40% of the agreements among all observers were good or excellent, whereas only 20% of good and excellent agreements were obtained using the AO and Lauge Hansen classifications. The Kappa index was 0.49 for the Danis-Weber classification, 0.32 for Lauge Hansen, and 0.38 for AO. Conclusion: The Hansen-Lauge classification presented the poorest interobserver agreement among the three systems. The AO classification demonstrated a moderate agreement and the Danis-Weber classification presented an excellent interobserver agreement index, regardless of professional experience.

  8. Correction of Facial Deformity in Sturge–Weber Syndrome

    Science.gov (United States)

    Yamaguchi, Kazuaki; Lonic, Daniel; Chen, Chit

    2016-01-01

    Background: Although previous studies have reported soft-tissue management in surgical treatment of Sturge–Weber syndrome (SWS), there are few reports describing facial bone surgery in this patient group. The purpose of this study is to examine the validity of our multidisciplinary algorithm for correcting facial deformities associated with SWS. To the best of our knowledge, this is the first study on orthognathic surgery for SWS patients. Methods: A retrospective chart review included 2 SWS patients who completed the surgical treatment algorithm. Radiographic and clinical data were recorded, and a treatment algorithm was derived. Results: According to the Roach classification, the first patient was classified as type I presenting with both facial and leptomeningeal vascular anomalies without glaucoma and the second patient as type II presenting only with a hemifacial capillary malformation. Considering positive findings in seizure history and intracranial vascular anomalies in the first case, the anesthetic management was modified to omit hypotensive anesthesia because of the potential risk of intracranial pressure elevation. Primarily, both patients underwent 2-jaw orthognathic surgery and facial bone contouring including genioplasty, zygomatic reduction, buccal fat pad removal, and masseter reduction without major complications. In the second step, the volume and distribution of facial soft tissues were altered by surgical resection and reposition. Both patients were satisfied with the surgical result. Conclusions: Our multidisciplinary algorithm can systematically detect potential risk factors. Correction of the asymmetric face by successive bone and soft-tissue surgery enables the patients to reduce their psychosocial burden and increase their quality of life. PMID:27622111

  9. A Course between Bureaucracy and Charisma: A Pedagogical Reading of Max Weber's Social Theory

    Science.gov (United States)

    Fantuzzo, John

    2015-01-01

    Philosophers of education tend to mention Max Weber's social theory in passing, assuming its importance and presuming its comprehension, but few have paused to consider how Weber's social theory might consciously inform educational theory and research, and none have done so comprehensively. The aim of this article is to begin this…

  10. A new model for quantum games based on the Marinatto–Weber approach

    International Nuclear Information System (INIS)

    Frąckiewicz, Piotr

    2013-01-01

    The Marinatto–Weber approach to quantum games is a straightforward way to apply the power of quantum mechanics to classical game theory. In the simplest case, the quantum scheme is that players manipulate their own qubits of a two-qubit state either with the identity 1 or the Pauli operator σ x . However, such a simplification of the scheme raises doubt as to whether it could really reflect a quantum game. In this paper we put forward examples which may constitute arguments against the present form of the Marinatto–Weber scheme. Next, we modify the scheme to eliminate the undesirable properties of the protocol by extending the players’ strategy sets. (paper)

  11. Síndrome de Klippel-Trenaunay-Parkes-Weber com angiomatose medular

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    James Pitagoras de Mattos

    1975-09-01

    Full Text Available É relatado um caso de síndrome de Klippel-Trénaunay-Parkes-Weber associada a angiomatose medular. O autor salienta ter encontrado na literatura somente uma referência com tal associação.

  12. Extracardiac manifestations of infective endocarditis and their historical descriptions.

    Science.gov (United States)

    Silverman, Mark E; Upshaw, Charles B

    2007-12-15

    In his landmark "Gulstonian Lectures on Malignant Endocarditis," published in 1885, William Osler commented, "Few diseases present greater difficulties in the way of diagnosis than malignant endocarditis, difficulties which in many cases are practically insurmountable." At that time, the fields of microbiology and blood cultures were in their infancy, and the diagnosis was made premortem in just half the patients with the condition. After Osler's report, extracardiac physical findings became essential clues to earlier diagnosis. Today, infective endocarditis is most commonly suggested from the history and often clinched by an echocardiogram and blood cultures. Although prized physical manifestations are much less frequent now, they still do occur and may be an invaluable clue that leads to earlier, more effective treatment. The investigators review these extracardiac findings along with their historical descriptions: splinter hemorrhages, emboli, Osler's nodes, Janeway and Bowman lesions of the eye, Roth spots, petechiae, and clubbing.

  13. A Contingent Affinity: Max Weber, Carl Schmitt, and the Challenge of Modern Politics.

    Science.gov (United States)

    Magalhães, Pedro T

    2016-01-01

    The thesis that the theory of charismatic-plebiscitary democracy developed by Max Weber in the wake of the Weimar Republic was developed to its ultimate consequences by Carl Schmitt in the final crisis of Weimar has been hotly debated since it was first advanced in the 1950s. This paper proposes a fresh look at the controversy. By comparing both authors' concepts of politics in their relation to the problem of modernity, it argues that the Weber-Schmitt affair is neither a baseless legend nor a case of natural continuity. Instead, it should rather be understood in terms of a contingent affinity.

  14. Prinsip-prinsip Dasar Rasionalisasi Birokrasi Max Weber Pada Organisasi Perangkat Daerah Kota Kendari Provinsi Sulawesi Tenggara

    OpenAIRE

    Kadir, Abdul

    2015-01-01

    : This research entitled Fundamental Principles of Max Weber's Rational Bureaucracy in Kendari City, South- East Sulawesi Province. The purpose of study is to explain and interpret the characteristics of Max Weber's bureaucracy that canastas an imperative control for officials ‘actions of Kendari City, South-East Sulawesi Province. This study utilizes a rational system perspective and applies qualitative research strategy with ethnography. The phenomenon of bureaucracy was explored naturally ...

  15. Prinsip-prinsip Dasar Rasionalisasi Birokrasi Max Weber pada Organisasi Perangkat Daerah Kota Kendari Provinsi Sulawesi Tenggara

    OpenAIRE

    Kadir, Abdul

    2015-01-01

    : This research entitled Fundamental Principles of Max Weber's Rational Bureaucracy in Kendari City, South- East Sulawesi Province. The purpose of study is to explain and interpret the characteristics of Max Weber's bureaucracy that canastas an imperative control for officials ‘actions of Kendari City, South-East Sulawesi Province. This study utilizes a rational system perspective and applies qualitative research strategy with ethnography. The phenomenon of bureaucracy was explored naturally ...

  16. Hindemith: Symphonic Metamorphosis on Themes of Carl Maria von Weber / Jonathan Swain

    Index Scriptorium Estoniae

    Swain, Jonathan

    1990-01-01

    Uuest heliplaadist "Hindemith: Symphonic Metamorphosis on Themes of Carl Maria von Weber, Mathis der Maler - Symphony, Nobilissima visione - suite. Atlanta Symphony Orchestra, Yoel Levi" Telarc/ Conifer CD 80 195

  17. Gabor Weber Local Descriptor for Bovine Iris Recognition

    OpenAIRE

    Sun, Shengnan; Zhao, Lindu; Yang, Shicai

    2013-01-01

    Iris recognition is a robust biometric technology. This paper proposes a novel local descriptor for bovine iris recognition, named Gabor Weber local descriptor (GWLD). We first compute the Gabor magnitude maps for the input bovine iris image, and then calculate the differential excitation and orientation for each pixel over each Gabor magnitude map. After that, we use these differential excitations and orientations to construct the GWLD histogram representation. Finally, histogram intersectio...

  18. Max Weber y Georg Simmel: ¿Dos teorías sociológicas de la modernidad?

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    JOSÉ Mª GONZÁLEZ GARCÍA

    2000-01-01

    Full Text Available A pesar de reconocer las diferencias entre Simmel y Weber en el análisis de la modernidad, el artículo aboga por la complementariedad de los dos enfoques: el análisis fragmentario y ensayístico de la sociedad moderna realizado por Simmel tendría su complemento más sistemático en el análisis del proceso de racionalización occidental hecho por Weber: Después de señalar la matriz intelectual común marcada por la herencia de Goethe y de Kant, así como por la recepción crítica de Marx y de Nietzsche, se estudian las críticas que Weber realizó de la obra de su amigo y colega Simmel, así como la influencia que la concepción Simmeliana de la tragedia de la cultura moderna ejerció sobre el diagnóstico desencantado de Weber acerca de la sociedad moderna como "jaula de hierro" de la razón burocratizada. Finalmente se contraponen la perspectiva de Weber en el análisis de la modernidad desde "el paradigma de la producción" con el análisis de Simmel desde "el paradigma del consumo", señalando las novedades introducidas por éste en el estudio de la mercancía, del consumidor y de la moda o de los cambios en la gran ciudad. Simmel construye una teoría de la modernidad basada en las vivencias del individuo, en lo "nuevo", en la fragmentación y en la estética.

  19. Patients Are Our Teachers

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    Robert E. Becker

    2018-04-01

    Full Text Available In the patient-physician encounter, physicians hone their skills while alleviating the patient’s suffering. Both benefit. Leaning on the work of Hippocrates, Darwin, and William Osler, the authors sketch out the case for honoring patients as indispensable teachers of the art and science of medicine. They argue that this tradition of Hippocratic medicine both anticipates modern precision medicine and reawakens a focus on public health medicine, each a benefit to the patients and communities served by physicians. A community that compromises the learning relationship of physician to patient and population undermines quality of care.

  20. Optimization study of fructans extraction from Agave tequilana Weber azul variety

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    Jesús Aarón SALAZAR-LEYVA

    Full Text Available Abstract Agave tequilana Weber azul variety is a valuable source of fructans. In this study, a response surface methodology was employed to optimize the extraction yield of fructans from agave pines. A Box Behnken factorial design was applied to evaluate the effects of several conditions such as extraction temperature, water raw material ratio and extraction time on extraction yield. Under the optimum extraction conditions (extraction temperature of 79.1 °C, water raw material ratio of 5.13 mL/g, and extraction time of 1.48 h fructans extraction yield was 83.12%. The chromatographic profile of the agave crude extract presented four peaks, out of which fructans were the most preponderant (~87%. The obtained results suggest that the response surface methodology is adequate to optimize fructans extraction from Agave tequilana Weber azul variety.

  1. Max Weber, la borsa e la ‘crisi del credito’ del 2008

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    Carlo Rossetti

    2011-02-01

    Full Text Available A partire da una rilettura di opere, come Die Protestantische Ethik und der Geist der Kapitalismus e Die Boersenequete, l'articolo mostra che – in linea con quanto Max Weber aveva descritto – l'assenza di regole generali condivise e di controlli pubblici mette in crisi le fondamenta del sistema finanziario. La privatizzazione neoliberista degli organi di controllo della finanza e la creazione di oscuri strumenti finanziari hanno contribuito alla regressione dell'organizzazione giuridica dello Stato, mettendo a serio rischio il principio di calcolabilità razionale che per secoli ha costituito il fondamento della teoria economica. In conclusione, Weber contribuisce a cogliere uno dei nodi fondamentali della crisi americana contemporanea: l'erosione dell’autorità indipendente posta a tutela dell’integrità del sistema.

  2. The dynamics of the water droplet impacting onto hot solid surfaces at medium Weber numbers

    Science.gov (United States)

    Mitrakusuma, Windy H.; Kamal, Samsul; Indarto; Dyan Susila, M.; Hermawan; Deendarlianto

    2017-10-01

    The effects of the wettability of a droplet impacting onto a hot solid surface under medium Weber numbers were studied experimentally. The Weber numbers used in the present experiment were 52.1, 57.6, and 63.1. Three kinds of solid surfaces with different wettability were used. These were normal stainless steel (NSS), TiO2 coated NSS, and TiO2 coated NSS radiated with ultraviolet rays. The surface temperatures were varied from 60 to 200 °C. The image of side the view and 30° from horizontal were taken to explain the spreading and the interfacial behavior of a single droplet during impact the hot solid surfaces. It was found that under medium Weber numbers, the surface wettability plays an important role on the droplet spreading and evaporation time during the impact on the hot solid surfaces. The higher the wettability, the larger the droplet spreading on the hot surface, and the lower the evaporation time.

  3. Angiome plan de la face: À propos de deux cas dont l’un révélant un syndrome de Sturge Weber et Krabb

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    Bénilde Marie Ange Tiemtoré-Kambou

    2017-01-01

    Full Text Available Sturge Weber and Krabbe syndrome or Sturge weber Syndrome (SWS is a rare and sporadic disease. It associates with a variety of degrees a flat facial skin angioma in the V1 territory of the trigeminal, a pial angioma and a choroidal angioma all in the one side. The neurological involvement of epileptic seizures in fact all the gravity. The authors report two cases of angiomas plane of the face. The first case presents a complete association of the 3 signs in a 5 year old child. The second case presents an association of angioma and choroidal involvement without cerebral damage to imaging in a 25-year-old adult. They thus highlight the interest of imaging examinations in particular MRI in the diagnosis through the classification and follow-up of this pathology.

  4. "Economia e sociedade": a herança de Max Weber à luz da edição de sua Obra completa (MWG "Economy and society": Max Weber's legacy following the publication of his Complete works (MWG

    Directory of Open Access Journals (Sweden)

    M. Rainer Lepsius

    2012-01-01

    Full Text Available O artigo relata o entendimento atual do conjunto de textos conhecido como Economia e sociedade, procurando esclarecer em que consistia originalmente; como foi organizado nas edições anteriores por Marianne Weber e Johannes Winckelmann; como foi tratado no âmbito da nova edição da MWG. Para tanto, procura indicar como Max Weber situava os diversos textos, as diversas versões e fases de concepção e composição desses escritos. A partir daí, sistematiza os principais aspectos dos textos evidenciados na MWG, assim como os principais campos de discussão que se abrem para a pesquisa, em especial a sociologia econômica.The article reviews the current understanding of the set of texts known as Economy and Society, looking to identify the original content, how these texts were edited in previous editions by Marianne Weber and Johannes Winckelmann, and how they have been dealt with in the new edition of the MWG. These aims in mind, the text looks to show how Max Weber himself situated the various texts, including the different versions and phases of conception and composition of the writings. The article then summarizes the main aspects of the works, as evinced in the MWG, as well as the principal fields of discussion opened up for research, especially in economic sociology.

  5. Some preliminary notes on the Algae collection Weber-van Bosse

    NARCIS (Netherlands)

    Koster, Joséphine Th.

    1936-01-01

    This extensive collection, famous among algologists both of the Old and the New World, forms part of the collections of the National Herbarium (Rijksherbarium) Leiden since 1934. About fifty years ago it was started by Mrs. Dr. A. A. WEBER-VAN BOSSE (1852—hodie), an enthusiastic pupil of HUGO DE

  6. Les Ecrits politiques de Max Weber: esquisse d’une lecture sociologique

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    François Chazel

    2014-05-01

    Full Text Available The author argues that Max Weber in his Politische Schriften (from 1906 to 1919 is using a sociological approach to the prevailing problems of Russia and Germany, grounded on categories of his political sociology. Weber’s in-depth analysis of the Russian Revolution of 1905 and of its outcome brilliantly illustrates this approach. In the first of his memoirs, overviewing Russian society as a whole, he highlights the multiple impediments to a success of bourgeois democracy in Russia; in the second one, he points out the political factors, as symbolized by “pseudo-constitutionalism”, that caused the failure of the liberal Revolution. In his “Parliament and Government in a reorganized Germany”, Weber rests on the analysis of bureaucracy in his first “sociology of domination”, pointing out that bureaucracy is unavoidable in modern society as well as it threatens to be overpowering. It is therefore required, in Weber’s view, to build institutions assuming a counterpoising function: he first conferred this role on a strong parliament, on the model of the English Parliament. Then, after the fall of the Wilhelminian Empire, he emphasized the plebiscitary power of a Reichspräsident. As a whole, Weber’s analyses bring out the dilemma between oligarchy and democracy but also the subtle relations linking them in the context of political modernity. Oligarchy is a threat to democracy because bureaucracy’s power can, if not checked, extend into the political field and because political parties are more and more oligarchic organizations. Large-scale socialism can only worsen the situation and lead to “bureaucracy’s dictatorship”. Therefore, Weber argues for the setting up of counterweights on the societal level. There is a way out of Michels’ dilemma in the national competition between bureaucratized parties. Weber is faced again with an authoritarian danger when he emphasizes in his last political writings the plebiscitary

  7. Le concept de « configuration » et ses implications empiriques : Elias avec et contre Weber

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    André Ducret

    2011-04-01

    Full Text Available Comment se fait-il que Norbert Elias ait autant cherché à prendre ses distances avec la conception de la sociologie que se faisait Max Weber alors que leurs démarches paraissent si proches ? On évaluera d’abord la portée des arguments avancés contre ce « bourgeois libéral » que serait demeuré le second. Puis on en dégagera les implications empiriques à partir d’extraits choisis dans l’œuvre du premier. On s’arrêtera enfin sur la démarche dite « synoptique » que Norbert Elias propose à tout sociologue d’adopter. En fin de compte, il apparaîtra que ce dernier défend une approche relationnelle de la contrainte sociale que n’aurait pas désavouée Max Weber.The notion of « configuration » and its empirical implications: Elias with or against WeberWhy is it that Norbert Elias went to such lengths to distance himself from Max Weber’s conception of sociology whilst their respective approaches seem so close? I will first evaluate the relevance of his arguments against the “liberal bourgeois” that Weber was to the end. I will then bring out the empirical implications from a number of extracts selected from his works. I will conclude by examining his “synoptic” approach, one to be followed by all sociologists. All things considered, it appears that Elias defends a relational approach of social constraint that Max Weber would not have disowned.El concepto de « configuración » y sus consecuencias empíricas: ¿Es Norbert Elias partidario o opuesto a  Weber?¿Como es posible que Norbert Elias haya tomado tanto la distancia con respecto a la visión sociológica de Max Weber a pesar de que su propia concepción se le asemeje mucho? Evaluaremos en primer lugar el alcance de los argumentos dirigidos del primero contra el «burgués liberal». Seguidamente subrayaremos las implicaciones empíricas a partir de citas escogidas de los escritos de Elias. Finalmente haremos hincapié en el encaminamiento

  8. El nuevo ideal del matrimonio en la autobiografía de Marianne Weber

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    Montserrat Bascoy Lamelas

    2012-11-01

    Full Text Available La socióloga Marianne Weber fue una de las teóricas más importantes del feminismo burgués en Alemania. Uno de los temas que le preocuparon especialmente fue la revisión del concepto de matrimonio, ante los cambios sociales en torno a 1900. A pesar de las nuevas necesidades de la mujer en este momento de transformaciones que conduce a los movimientos por la emancipación femenina y al rechazo generalizado del matrimonio, Marianne Weber, impulsada también por sus propias experiencias vitales, afirma la relevancia y la necesidad del mismo como ideal de las relaciones sexuales, y lo define nuevamente.

  9. Dynamic Impacts of Water Droplets onto Icephobic Soft Surfaces at High Weber Numbers

    Science.gov (United States)

    Ma, Liqun; Liu, Yang; Hu, Hui; Wang, Wei; Kota, Arun

    2017-11-01

    An experimental investigation was performed to examine the effects of the stiffness of icephobic soft PDMS materials on the impact dynamics of water drops at high weber numbers pertinent to aircraft icing phenomena. The experimental study was performed in the Icing Research Tunnel available at Iowa State University (ISU-IRT). During the experiments, both the shear modulus of the soft PDMS surface and the Weber numbers of the impinging water droplets are controlled for the comparative study. While the shear modulus of the soft PDMS surface was changed by tuning the recipes to make the PDMS materials, the Weber number of the impinging water droplets was altered by adjusting the airflow speed in the wind tunnel. A suite of advanced flow diagnostic techniques, which include high-speed photographic imaging, digital image projection (DIP), and infrared (IR) imaging thermometry, were used to quantify the transient behavior of water droplet impingement, unsteady heat transfer and dynamic ice accreting process over the icephobic soft airfoil surfaces. The findings derived from the icing physics studies can be used to improve current icing accretion models for more accurate prediction of ice formation and accretion on aircraft wings and to develop effective anti-/deicing strategies for safer and more efficient operation of aircraft in cold weather.

  10. Gravity Reduction View: A Radiographic Technique for the Evaluation and Management of Weber B Fibula Fractures

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    Lauren Ehrlichman

    2017-03-01

    Full Text Available Background: While various radiographic parameters and application of manual/gravity stress have been proposed to elucidate instability for Weber B fibula fractures, the prognostic capability of these modalities remains unclear. Determination of anatomic positioning of the mortise is paramount. We propose a novel view, the Gravity Reduction View (GRV, which helps elucidate non-anatomic positioning and reducibility of the mortise.Methods: The patient is positioned lateral decubitus with the injured leg elevated on a holder with the fibula directed superiorly. The x-ray cassette is placed posterior to the heel, with the beam angled at 15˚ of internal rotation to obtain a mortise view. Our proposed treatment algorithm is based upon the measurement of the medial clear space (MCS on the GRV versus the static mortise view (and in comparison to the superior clear space (SCS and is based on reducibility of the MCS. A retrospective review of patients evaluated utilizing the GRV was performed.Results: 26 patients with Weber B fibula fractures were managed according to this treatment algorithm. Mean age was 50.57 years old (range: range:18-81, SD=19. 17 patients underwent operative treatment and 9 patients were initially treated nonoperatively. 2 patients demonstrated late displacement and were treated surgically. Using this algorithm, at a mean follow-up of 26 weeks, all patients had a final MCS that was less than the SCS (final mean MCS 2.86 mm vs. mean SCS of 3.32 indicating effectiveness of the treatment algorithm.Conclusion: The GRV is a novel radiographic view in which deltoid competency, reducibility and initial positioning of the mortise are assessed by comparing a static mortise view with the appearance of the mortise on the GRV. We have developed a treatment algorithm based on the GRV and have found it to be useful in guiding treatment and successful at achieving anatomic mortise alignment.

  11. En busca de las sociólogas fundadoras: Marianne Weber

    Directory of Open Access Journals (Sweden)

    Luz Gabriela Arango Gaviria

    2006-01-01

    Full Text Available Con miras a difundir el trabajo de las académicas norteamericanas por rescatar la obra de Mlarianne Weber, entre otras sociólogas fundadoras, hemos traducido un fragmento de su ensayo: "Autoridad y autonomía en el matrimonio". En éste, la autora analiza las distintas configuraciones culturales del matrimonio en la historia occidental, desde el "patriarcado primitivo" hasta las formas modernas de matrimonio civil y religioso. La autora destaca la influencia de las doctrinas católicas y protestantes en la transformación de esta institución hacia una mayor equidad entre los sexos y señala críticamente las con tradicciones de las formas modernas de contra to matrimonial que preservan la primacía masculina y contradicen las enseñanzas éticas del idealismo alemán en torno a la libertad. Marianne \\V'cber defiende un ideal matrimonial que respete y reconozca plenamente la individualidad y autonomía de la mujer. Palabras clave: Marianne Weber, matrimonio, mujeres, igualdad.

  12. [Thought Experiments in Historiographic Function: Max Weber on Eduard Meyer and the Question of Counterfactuality].

    Science.gov (United States)

    Ernst, Florian

    2015-03-01

    Thought Experiments in Historiographic Function: Max Weber on Eduard Meyer and the Question of Counterfactuality. Max Weber's remarks on his colleague Eduard Meyer regarding counterfactual reasoning in history reflects a significant shift during the Methodenstreit around 1900. The question of attributing historical change strictly to either individual causes or abstract general laws has been tackled in a new way: By counterfactual reasoning a historian should be able to detect the most significant (and therefore meaningful) cause, event, or action for a certain historical outcome. Following Fritz Ringer, this paper argues that given the predominating methods of the natural sciences, scholars of the humanities conducted historical research by counterfactual thought experiments. This way, Weber pried open contemporary narratives (e.g. historicism), and by employing a unique historical causal analysis he made way for refined concepts to offer a model of interpretation that gave hope for a more feasible, practice-oriented approach to historical research than the epistemological discussions had hitherto offered. © 2015 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  13. 75 FR 3955 - Environmental Impact Statement: Davis and Weber Counties, UT

    Science.gov (United States)

    2010-01-25

    ... and Weber Counties, UT AGENCY: Federal Highway Administration (FHWA), DOT. ACTION: Notice of Intent... FHWA, in cooperation with UDOT, will prepare an EIS on a proposal to address projected transportation... east of the Great Salt Lake. To provide for local and regional travel demands, the regional...

  14. Pyogenic Granuloma in a Patient of Sturge-Weber Syndrome with Bilateral Port Wine Stain- A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Shantala Arunkumar

    2014-07-01

    Full Text Available Sturge-Weber syndrome (SWS also known as encephalotrigeminal angiomatosis. It is a neurocutaneous syndrome, characterized by a facial vascular birthmark and neurological abnormalities. An ipsilateral or bilateral facial cutaneous vascular malformation Port Wine Stain (PWS usually affects the upper face. Other clinical manifestations are seizures, glaucoma, hemiparesis, mental retardation and delayed developmental milestones. The main objective of this case report is to unravel such a rarest syndrome with bilateral port-wine stain, which has intraoral manifestation of pyogenic granuloma involving gingiva in an 11 year old boy.

  15. Talcott Parsons’ Early Essay on Capitalism. An American Interpretation of Werner Sombart and Max Weber

    Directory of Open Access Journals (Sweden)

    Mariano Longo

    2015-03-01

    Full Text Available The paper analyzes Talcott Parsons’ early essay published in two issues of The Journal of Political Economy in 1928 and 1929. ““Capitalism” in recent German Literature: Sombart and Weber”, which introduces both Sombart and Weber to the American public. It represents a relevant moment in the development of Parsons as a sociologist. After discussing Parsons’ interpretation of both Sombart’s and Weber’s theories about the origin of Capitalism, the paper gives an interpretation of Parsons’ role in the establishment of Weber as a founding father of sociology and the relative irrelevance of Sombart as a sociological classic.

  16. Max Weber, educación para las ciencias y formación democrática

    Directory of Open Access Journals (Sweden)

    Javier Benito Seoane Cobas

    2010-04-01

    Full Text Available El ensayo explora la relación que hay entre la educación para las ciencias y la educación para la democracia en el pensamiento de Max Weber, un problema no tratado por el sociólogo de Erfurt. Ello se realiza a partir del análisis del plano epistemológico y del análisis sobre la ética del científico de Weber. Al final, se elaboran dos interpretaciones razonables sobre el objeto del ensayo y se selecciona una de las mismas sobre la base de una apuesta pluralista.

  17. Interpreted Modernity. Weber and Taylor on Values and Modernity

    OpenAIRE

    Reckling, Falk

    2001-01-01

    The writings of Weber and Taylor have some strong affinities. Both start from the anthropological idea that man evaluates his position in the world and constitutes the social world by values. Their analyses of values aim at an understanding of those intersubjective meanings that have constituted western modernity. But, at the same time, their anthropological starting point leads to different interpretations of modernity. Historically, both argue that rationalization (as instrumental rationali...

  18. Desvendando a religião e as religiões mundiais em Max Weber (Revealing religion and the world religions in Max Weber - DOI: 10.5752/P.2175-5841.2009v7n14p136

    Directory of Open Access Journals (Sweden)

    Arilson Silva Oliveira

    2009-10-01

    Full Text Available Apresentamos Max Weber como um dos sociólogos e historiadores mais importantes dentre aqueles que se dedicaram ao estudo do fenômeno religioso. Na verdade, é possível afirmar que a análise da religião compreende um dos aspectos mais fundamentais de sua obra sócio-histórica. De modo geral, esse tema aparece em seus textos de duas maneiras diferentes, quais sejam: enquanto um objeto analisado em sua singularidade e enquanto uma manifestação social que influencia de maneira significativa os demais aspectos da vida comunitária. Aqui, observamos como ele muniu-se de um método particular e o utilizou como parâmetro para compreender historicamente a religião. Ao se debruçar sobre as religiões mundiais (confucionismo-taoísmo, judaísmo-cristianismo e hinduísmo-budismo, Weber estuda a racionalização cultural de suas cosmovisões. Todavia, para ele, a influência da religião sobre a vida prática varia muito segundo o caminho da salvação/libertação que é prescrito e segundo a qualidade psíquica (ou imaginada da salvação que se pretende alcançar. Palavras-chave: Max Weber; Religião; Religiões Mundiais; Racionalização.   AbstractWe present Max Weber as one of the most important sociologists and historians among those who dedicated themselves to the study of the religious phenomenon. Actually, it is possible to say that the analysis of religion involves one of the most fundamental aspects of his socio-historical work. As a whole, this subject appears in his texts in two different forms, i.e., as an analyzed object in its particularities, and as a social manifestation which influences, in a significant way, the other aspects of communitarian life. Here, we observe how he equipped himself with a particular method, rescued Kantian rationality and applied it as a parameter to historically understand religion. While he dedicated himself to study world religions (Confucianism-Taoism, Judaism-Christianity, and Hinduism

  19. Patterns of structural reorganization of the corticospinal tract in children with Sturge-Weber syndrome.

    Science.gov (United States)

    Kamson, David O; Juhász, Csaba; Shin, Joseph; Behen, Michael E; Guy, William C; Chugani, Harry T; Jeong, Jeong-Won

    2014-04-01

    Reorganization of the corticospinal tract after early damage can limit motor deficit. In this study, we explored patterns of structural corticospinal tract reorganization in children with Sturge-Weber syndrome. Five children (age 1.5-7 years) with motor deficit resulting from unilateral Sturge-Weber syndrome were studied prospectively and longitudinally (1-2 years follow-up). Corticospinal tract segments belonging to hand and leg movements were separated and their volume was measured by diffusion tensor imaging tractography using a recently validated method. Corticospinal tract segmental volumes were normalized and compared between the Sturge-Weber syndrome children and age-matched healthy controls. Volume changes during follow-up were also compared with clinical motor symptoms. In the Sturge-Weber syndrome children, hand-related (but not leg-related) corticospinal tract volumes were consistently decreased in the affected cerebral hemisphere at baseline. At follow-up, two distinct patterns of hand corticospinal tract volume changes emerged. (1) Two children with extensive frontal lobe damage showed a corticospinal tract volume decrease in the lesional hemisphere and a concomitant increase in the nonlesional (contralateral) hemisphere. These children developed good hand grasp but no fine motor skills. (2) The three other children, with relative sparing of the frontal lobe, showed an interval increase of the normalized hand corticospinal tract volume in the affected hemisphere; these children showed no gross motor deficit at follow-up. Diffusion tensor imaging tractography can detect differential abnormalities in the hand corticospinal tract segment both ipsi- and contralateral to the lesion. Interval increase in the corticospinal tract hand segment suggests structural reorganization, whose pattern may determine clinical motor outcome and could guide strategies for early motor intervention. Copyright © 2014 Elsevier Inc. All rights reserved.

  20. A clinical and neurological study on the Sturge-Weber syndrome

    International Nuclear Information System (INIS)

    Ochiai, Takako

    1987-01-01

    The author studied the relationship between facial nevus and intracranial changes seen on cranial computed tomography (CCT) scannings in 12 children with typical Sturge-Weber syndrome. The evaluation of epileptic attacks, repeat EEG and cranial CT examinations with or without enhancement during the follow-up period of 8 years in average were analysed. In 7 cases (58.3 %), the dominant side of facial hemangioma was identical with that of calcification on CCT. Three cases of central facial nevus showed calcification in one hemisphere, on either side. One who had facial nevus on one side showed dominant calcification on the other side on CT. The area and side of the facial nevus did not always coincide with those of the intracranial lesion. In 4 of the 9 patients who were followed up by repeat CCT, we recognized increases in degree of brain atrophy with or without increases in the area of calcification. In the enhancement study, 6 patients (89 %) showed positive choroid plexus images with abnormal enhancement on the same side as the calcification. On EEG 5 cases showed epileptiform activity over the hemisphere with calcification, and 3 showed it on the intact side of the brain. (author)

  1. Democracia com liderança: Max Weber e o conceito de democracia plebiscitária

    OpenAIRE

    Sell, Carlos Eduardo

    2011-01-01

    As complexas relações entre regimes políticos (burocracia) e liderança política (carisma) ocupam o centro da reflexão sociopolítica de Max Weber. Este artigo propõe-se a examinar esta temática a partir do conceito weberiano de democracia plebiscitária. O trabalho está dividido em quatro partes. A primeira examina a trajetória do conceito nos escritos políticos e sociológicos de Weber. Após reconstruir a biografia do conceito, o segundo tópico analisa o tema do ponto de vista sistemático. Dist...

  2. [Max Weber. Poliitika kui elukutse ja kutsumus. Teadus kui elukutse ja kutsumus] / Hermo Kuusk

    Index Scriptorium Estoniae

    Kuusk, Hermo, 1976-

    2011-01-01

    Tutvustus: Weber, Max. Poliitika kui elukutse ja kutsumus ; Teadus kui elukutse ja kutsumus / tõlkijad: Jaan Isotamm, Henn Käärik, Kaia Sisask. Tallinn : TLU Press, 2010. (Bibliotheca controversiarum)

  3. Vision for action and perception elicit dissociable adherence to Weber's law across a range of 'graspable' target objects.

    Science.gov (United States)

    Heath, Matthew; Manzone, Joseph; Khan, Michaela; Davarpanah Jazi, Shirin

    2017-10-01

    A number of studies have reported that grasps and manual estimations of differently sized target objects (e.g., 20 through 70 mm) violate and adhere to Weber's law, respectively (e.g., Ganel et al. 2008a, Curr Biol 18:R599-R601)-a result interpreted as evidence that separate visual codes support actions (i.e., absolute) and perceptions (i.e., relative). More recent work employing a broader range of target objects (i.e., 5 through 120 mm) has laid question to this claim and proposed that grasps for 'larger' target objects (i.e., >20 mm) elicit an inverse relationship to Weber's law and that manual estimations for target objects greater than 40 mm violate the law (Bruno et al. 2016, Neuropsychologia 91:327-334). In accounting for this finding, it was proposed that biomechanical limits in aperture shaping preclude the application of Weber's law for larger target objects. It is, however, important to note that the work supporting a biomechanical account may have employed target objects that approached -or were beyond-some participants' maximal aperture separation. The present investigation examined whether grasps and manual estimations differentially adhere to Weber's law across a continuous range of functionally 'graspable' target objects (i.e., 10,…,80% of participant-specific maximal aperture separation). In addition, we employed a method of adjustment task to examine whether manual estimation provides a valid proxy for a traditional measure of perceptual judgment. Manual estimation and method of adjustment tasks demonstrated adherence to Weber's law across the continuous range of target objects used here, whereas grasps violated the law. Thus, results evince that grasps and manual estimations of graspable target objects are, respectively, mediated via absolute and relative visual information.

  4. Juan Daniel Weber en Aullagas (1790: De minero de segunda en Europa a experto en Potosí

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    María Concepción Gavira Marquez

    2015-02-01

    Full Text Available Este trabajo aborda la expedición de metalúrgicos centro europeos comisionados por la Corona española para modernizar la minería americana a fines del siglo XVIII. En concreto, nos centraremos en un personaje especial, Juan Daniel Weber, miembro de la comisión destinada a la región andina liderada por Nordenflycht y que pasó por Potosí sin ningún éxito en cuanto a sus objetivos prioritarios. Aunque Weber en principio no estaba destinado a Alto Perú, permaneció en Potosí, donde se distinguió por dedicarse a defender sus intereses particulares en Aullagas, Chayanta, concretamente invirtiendo en empresas para el desagüe de la veta de Colquechaca y San Bartolomé.   This paper addresses the issue of Central European metal workers commissioned by the Spanish Crown to modernize the American mining activity in the late 18th century. In particular, we focus on a special character, Juan Daniel Weber, a member of the commission sent to the Andean region led by Nordenflycht and that passed by Potosí without achieving any of its main goals. Although, initially, Weber was not destined to go to Upper Peru, he stayed in Potosí, where he stood out for defending his own interests in Aullagas, Chayanta, investing specifically in companies dedicated to seam drainage in Colquechaca and San Bartolomé.

  5. Wandering ethos and romantic spirit: Reflections following the trail of Max Weber's sociology

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    Jeremić-Molnar Dragana

    2013-01-01

    Full Text Available First part of the article is devoted to the main categories of Max Weber's historicist sociology: ethos and spirit. The authors are examining his concepts of capitalist spirit and protestant spirit and, relaying especially on Weber's unfinished treatise on music, contemplating the missing concept of romantic spirit, which could be tied with wandering ethos. In the second part of article, they are separating the phenomena of wandering (moving without purpose or goal from travelling (moving towards the determined end and analysing the possibilities of re-constructing the wandering ethos in context of emerging early Romanticism. In the end they are dealing with the main change the wandering underwent in 18th Century and early 19th Century: from despised activity practiced by social outcasts only (with few exceptions such as journeymen and merchants to the tool of regeneration.

  6. Max Weber's Types of Rationality: Cornerstones for the Analysis of Rationalization Processes in History.

    Science.gov (United States)

    Kalberg, Stephen

    1980-01-01

    Explores rationality in Max Weber's works and identifies four types of rationality which play major roles in his writing--practical, theoretical, substantive, and formal. Implications for society and education are discussed. (DB)

  7. O tema da morte trágica de Liev Tolstói e seu impacto em Max Weber e György Lukács: sobre a autonomia nas ciências e na arte * Leo Tolstoy´s tragic death and his impacts on Max Weber and György Lukács: on autonomy of arts and science

    Directory of Open Access Journals (Sweden)

    LUIS FELIPE DE SALLES ROSELINO

    2014-04-01

    Full Text Available Resumo: O tema da morte trágica, presente nos escritos de Liev Tolstói, auxiliou tanto a Max Weber como a György Lukács a caracterizarem o pathos moderno de pressentimento da morte como uma contemplação do vazio. Weber e Lukács encontraram, através das leituras de Tolstói, uma interessante maneira de questionar a autonomia da arte e da ciência moderna, considerando pela esfera estética, como se mostra sem sentido a recente realidade imanente. Ambos assumiram o tema central das obras de Tolstói segundo uma mesma imagem, derivada do contraste entre o mundo antigo e o moderno. Max Weber adequou esse tema a sua teoria do desencantamento do mundo e Lukács, de modo muito semelhante, seguindo seu conceito do paradoxo da necessidade religiosa.Palavras-chave: Tolstói – Weber – Lukács – Desencantamento – Necessidade Religiosa – L’Art Pour l’Art. Abstract: The tragic death in Tolstoy's writings has helped both Max Weber and György Lukács in characterizing the modern pathos as a tragic contemplation of the emptiness of life. Through Tolstoy's readings, Weber and Lukács found an interesting source of denying arts and modern sciences autonomy, considering, from the aesthetics sphere, the meaningless of this new immanent reality. Both has assumed Tolstoy main theme from the same perspective, contrasting ancient and modern worldviews. Max Weber presented this theme in his disenchantment of world theory and Lukács, in a very similar way, following the paradox of religious needing as a mainline.Keywords: Tolstoy – Weber – Lukács – Disenchantment – Religious Needing – L’Art Pour l’Art.

  8. Values on Paper, in the Head, and in Action: On Max Weber and Value Freedom Today.

    Science.gov (United States)

    Betta, Michela; Swedberg, Richard

    2017-11-01

    This article starts out with a summary of Weber's views on value freedom, by emphasizing: (1) that value freedom constitutes a special constellation of values; and (2) that value freedom makes it possible for the social scientist to theorize on the basis of new and more extensive knowledge than if she had simply stated her own values and focused the analysis around these. The latter point emerges most clearly in Weber's instructions for how a social scientist should proceed when carrying out an analysis of her own preferred social policy. After the section on Weber's views on value freedom, an attempt is made to update his views. This is done by arguing that the impact of values (and value freedom) differs, depending on where these can be found: on paper, in the head of the social scientist, or in her actions. "Actions," in the context of value freedom, refer to the research process and especially to the element of theorizing. Value freedom helps to guide the research into new and fruitful directions and to steer it clear of propaganda. © 2017 Canadian Sociological Association/La Société canadienne de sociologie.

  9. Vertical parasagittal hemispherotomy for Sturge-Weber syndrome in early infancy: case report and literature review.

    Science.gov (United States)

    Liu, Xiangyu; Otsuki, Taisuke; Takahashi, Akio; Kaido, Takanobu

    2016-01-01

    The authors here present a rare case of a 3-month-old infant with unilateral Sturge-Weber syndrome (SWS) who had excellent seizure control and no aggravation of previous existed neurological deficits after vertical parasagittal hemispherotomy (VPH). To our knowledge, this patient with SWS was the youngest one who received VPH. The use of VPH results in a successful treatment of intractable epilepsy in a patient with seizure onset in early infancy. At follow-up, the patient's neurodevelopmental status has been improved since the surgery. It is generally accepted that early-onset seizures in children with SWS are associated with worse neurological and developmental outcomes. However, when surgical treatment should be considered and how it should be performed remain a longstanding controversy. We promote early surgery in children with SWS and early-onset epilepsy. We suggest that VPH may be a useful adjuvant in the management of SWS with refractory epilepsy in early infancy and this procedure carries low neurological risk.

  10. Max Weber y Georg Simmel: ¿Dos teorías sociológicas de la modernidad?

    OpenAIRE

    JOSÉ Mª GONZÁLEZ GARCÍA

    2000-01-01

    A pesar de reconocer las diferencias entre Simmel y Weber en el análisis de la modernidad, el artículo aboga por la complementariedad de los dos enfoques: el análisis fragmentario y ensayístico de la sociedad moderna realizado por Simmel tendría su complemento más sistemático en el análisis del proceso de racionalización occidental hecho por Weber: Después de señalar la matriz intelectual común marcada por la herencia de Goethe y de Kant, así como por la recepción crítica de Marx y de Nietzsc...

  11. Georg Simmel y Max Weber: ¿Dos teorías sociológicas de la modernidad?

    OpenAIRE

    González García, José M.

    2000-01-01

    A pesar de reconocer las diferencias entre Simmel y Weber en el análisis de la modernidad, el artículo aboga por la complementariedad de los dos enfoques: el análisis fragmentario y ensayístico de la sociedad moderna realizado por Simmel tendría su complemento más sistemático en el análisis del proceso de racionalización occidental hecho por Weber. Después de señalar la matriz intelectual común marcada por la herencia de Goethe y de Kant, así como por la recepción crítica de Ma...

  12. Weber, Simmel e a morte sem sentido

    Directory of Open Access Journals (Sweden)

    Cláudia Maria Guedes Joaquim

    2007-01-01

    uma relação que se apresenta mais problemática e carente de significado. Duas características fundamentais da modernidade apontadas por dois clássicos da teoria social, Weber e Simmel, apresentam pistas para se pensar a nova (in compreensão sobre a morte: os processos de racionalização e de individualização. A obra destes autores não necessariamente circunda o tema da morte; no entanto, seus diagnósticos da modernidade acabam por, conseqüentemente, apontar para a falta de sentido da morte do homem moderno. Por fim, escritos poucos conhecidos de Simmel, do final de sua vida, demonstram sua tentativa de buscar o significado perdido.

  13. Gabor Weber Local Descriptor for Bovine Iris Recognition

    Directory of Open Access Journals (Sweden)

    Shengnan Sun

    2013-01-01

    Full Text Available Iris recognition is a robust biometric technology. This paper proposes a novel local descriptor for bovine iris recognition, named Gabor Weber local descriptor (GWLD. We first compute the Gabor magnitude maps for the input bovine iris image, and then calculate the differential excitation and orientation for each pixel over each Gabor magnitude map. After that, we use these differential excitations and orientations to construct the GWLD histogram representation. Finally, histogram intersection is adopted to measure the similarity between different GWLD histograms. The experimental results on the SEU bovine iris database verify the representation power of our proposed local descriptor.

  14. Liquid-liquid phase transition in Stillinger-Weber silicon

    International Nuclear Information System (INIS)

    Beaucage, Philippe; Mousseau, Normand

    2005-01-01

    It was recently demonstrated that Stillinger-Weber silicon undergoes a liquid-liquid first-order phase transition deep into the supercooled region (Sastry and Angell 2003 Nat. Mater. 2 739). Here we study the effects of perturbations on this phase transition. We show that the order of the liquid-liquid transition changes with negative pressure. We also find that the liquid-liquid transition disappears when the three-body term of the potential is strengthened by as little as 5%. This implies that the details of the potential could affect strongly the nature and even the existence of the liquid-liquid phase

  15. Max Weber Revisited, the Verstehen of Migration through Qualitative Research

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    Jorge A. BUSTAMANTE

    2017-01-01

    Full Text Available Se incluye 1 una reinterpretación de un par de conceptos básicos ( Gemeinten Sinn y Verstehen de Max Weber. Se define a la migración internacional (inmigración como una relación social de carácter laboral. Se hace referencia al libro de Ernesto Galarza. La noción teórica de Verstehen entendida por Weber como un “entendimiento pro - fundo” de un fenómeno social, da lugar a la narración que hace el autor, derivada del uso del 2 método de “observación participante” de una experiencia personal. De ésta se deriva el concepto de vulnerabilidad que implica una innovación a los enfo - ques teóricos de la migración. Lo anterior se encuadra en un enfoque metodológico de investigación cualitativa de la cual 3 no se desprenden “hallazgos” en su sentido empírico, sino explicaciones complementarias del “sentido” de los hechos relatados. 4 El encuentro del autor con un compañero migrante en el centro de detención, se presenta como el origen empírico del concepto de vulnerabilidad.

  16. Polycythemia vera

    Science.gov (United States)

    Primary polycythemia; Polycythemia rubra vera; Myeloproliferative disorder; Erythremia; Splenomegalic polycythemia; Vaquez's disease; Osler's disease; Polycythemia with chronic cyanosis; Erythrocytosis ...

  17. Determinants of outcome in operatively and non-operatively treated Weber-B ankle fractures

    NARCIS (Netherlands)

    van Schie-van der Weert, E. M.; van Lieshout, E. M. M.; de Vries, M. R.; van der Elst, M.; Schepers, T.

    2012-01-01

    Treatment of ankle fractures is often based on fracture type and surgeon's individual judgment. Literature concerning the treatment options and outcome are dated and frequently contradicting. The aim of this study was to determine the clinical and functional outcome after AO-Weber B-type ankle

  18. O historiador Max Weber: indologia e historicismo na obra weberiana

    Directory of Open Access Journals (Sweden)

    Arilson Silva de Oliveira

    2010-06-01

    Full Text Available Max Weber assumed the methodological requirements of German historicism against all natural illuminist philosophy and all the idealistic philosophy of history. He improved them in order to prevent the conclusions of the romanticism and the psychological deviations of the neo-historicism. Here, we observe how he equipped himself with a particular method, rescued rationality and applied it as a parameter to historically understand Indian society in opposition to the disenchanted historicism of his time.

  19. Lateral malleolus hook plate for comminuted Weber A and B fractures: A retrospective study

    Directory of Open Access Journals (Sweden)

    Fang Zhenhua

    2013-01-01

    Full Text Available Background: The goal of managing the comminuted fracture of lateral malleolus is to restore length, rotation and alignment which might be more challenging with extensive comminution around the area of the distal tip. The common osteosynthesis techniques such as the one-third tubular plate, tension band wiring, K-wires, screws, or intramedullary nail may be insufficient in cases with a comminuted lateral malleolus. The anatomical hook plate is an alternative implant in such cases. We present our results of the comminuted lateral malleolar fractures (Weber A, B, managed by open reduction and internal fixation (ORIF with an anatomical hook plate of lateral malleolus (Königsee Implant Company, Germany. Materials and Methods: We retrospectively reviewed 20 patients of comminuted fracture of distal lateral malleolus between 2008 and 2010. There were 12 males and 8 females, right side was involved in 18 patients and left in 2. The mean age was 51.9 years (range 18-75 years. The fractures were categorized by Denis-Weber classification type A (n=1, B1 (n=1, B2 (n=13 and B3 (n=5. Nineteen cases were of closed injury and one of open injury (Gustilo Anderson type II. These patients underwent ORIF with a lateral malleolus anatomical hook plate. Followup including radiographs and clinical examinations were performed. The American Orthopedic Foot and Ankle Society (AOFAS ankle hindfoot scores were documented at followups. Results: The mean followup was 21.4 months (range 16-27 months. The average AOFAS score was 94.3 (range 78-100 points. A stable anatomic reduction and bony union were obtained in all the cases. The average time was 3.1 months (range 2.5-4 months. Four cases had complications like posttraumatic osteoarthritis, hardware impingement and superficial wound infection. Conclusion: A reasonably good stability can be obtained in distal most comminuted fractures of lateral malleolus with a lateral malleolus anatomical hook plate. We believe this

  20. Weber's Illusion and Body Shape: Anisotropy of Tactile Size Perception on the Hand

    Science.gov (United States)

    Longo, Matthew R.; Haggard, Patrick

    2011-01-01

    The perceived distance between touches on a single skin surface is larger on regions of high tactile sensitivity than those with lower acuity, an effect known as "Weber's illusion". This illusion suggests that tactile size perception involves a representation of the perceived size of body parts preserving characteristics of the somatosensory…

  1. RELATIONSHIPS BETWEEN THE SOCIAL ETHICS OF LUTERO AND THE CONCEPTION OF WEBER ON THE PROTESTANT ETHICS IN THE EDUCATIONAL CONTEXT

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    ALVORI AHLERT

    2007-07-01

    Full Text Available This text has as objective to understand the differences between the social ethics of Luther and the conceptions on the protestant ethics developed by Max Weber. The lutheran social ethics do not enroll in the religious ascetism of the pietism and in the ethical vision of the calvinist protestantism of the analysis of Max Weber on the protestant ethics. The thesis of Weber does not allow to credit to the social ethics of Luther bonds with the spirit of the development of the capitalism. The proper Weber, in moment, believes that the spirit of the capitalism if must the influences of the Reformation Lutheran or that the capitalism as system is a product of the Reformation. Therefore, a conception of lutheran ethics, established in the social ethics of Luther, remains as important instrument of critical in a society in which the human being comes losing its ethical referent and its citizencommunitarian base for the absence of the practical learning of these values, for being it delivers to the individualism and he process of coisificação accomplished for the industrial capitalism and its current marketing face that an limited citizenship imposes the majority of the population of the planet, lead for those that they control the economy and the politics (the money and the power and for the ethics of the market.

  2. Places de Max Weber et Gaston Bachelard chez Pierre Bourdieu

    OpenAIRE

    Colas, Dominique

    2005-01-01

    Can we better understand Pierre Bourdieu's sociology by appealing to the intellectual references he made use of in his works? Not really, because confronting Bourdieu with the classical sources he relies on, as Marx, Weber or Bachelard, the reader can note a rather lax exercise of the concepts he is borrowing and he is making his. Or, this insight in Bourdieu's work takes the opposite way, as it tries to analytically read Bourdieu intellectual project on building sociology as science by start...

  3. From Weber to Parsons and Shutz: The Eclipse of History in Modern Social Theory.

    Science.gov (United States)

    Zaret, David

    1980-01-01

    Compares the relationship between theoretical synthesis and historical research in light of research by Max Weber, Talcott Parsons, and Alfred Schutz. Traces theoretical developments within one subfield of sociology (action theory) and relates these developments to research problems confronting contemporary theoretical work in sociology. (DB)

  4. Aberrant meiotic behavior in Agave tequilana Weber var. azul.

    Science.gov (United States)

    Ruvalcaba-Ruiz, Domingo; Rodríguez-Garay, Benjamin

    2002-10-23

    Agave tequilana Weber var. azul, is the only one variety permitted by federal law in México to be used for tequila production which is the most popular contemporary alcoholic beverage made from agave and recognized worldwide. Despite the economic, genetic, and ornamental value of the plant, it has not been subjected to detailed cytogenetic research, which could lead to a better understanding of its reproduction for future genetic improvement. The objective of this work was to study the meiotic behavior in pollen mother cells and its implications on the pollen viability in Agave tequilana Weber var. azul. The analysis of Pollen Mother Cells in anaphase I (A-I) showed 82.56% of cells with a normal anaphase and, 17.44% with an irregular anaphase. In which 5.28% corresponded to cells with side arm bridges (SAB); 3.68% cells with one bridge and one fragment; 2.58% of irregular anaphase showed cells with one or two lagging chromosomes and 2.95% showed one acentric fragment; cells with two bridges and cells with two bridges and one acentric fragment were observed in frequencies of 1.60% and 1.35% respectively. In anaphase II some cells showed bridges and fragments too. Aberrant A-I cells had many shrunken or empty pollen grains (42.00%) and 58.00 % viable pollen. The observed meiotic irregularities suggest that structural chromosome aberrations have occurred, such as heterozygous inversions, sister chromatid exchanges, deletions and duplications which in turn are reflected in a low pollen viability.

  5. Aberrant meiotic behavior in Agave tequilana Weber var. azul

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    Rodríguez-Garay Benjamin

    2002-10-01

    Full Text Available Abstract Background Agave tequilana Weber var. azul, is the only one variety permitted by federal law in México to be used for tequila production which is the most popular contemporary alcoholic beverage made from agave and recognized worldwide. Despite the economic, genetic, and ornamental value of the plant, it has not been subjected to detailed cytogenetic research, which could lead to a better understanding of its reproduction for future genetic improvement. The objective of this work was to study the meiotic behavior in pollen mother cells and its implications on the pollen viability in Agave tequilana Weber var. azul. Results The analysis of Pollen Mother Cells in anaphase I (A-I showed 82.56% of cells with a normal anaphase and, 17.44% with an irregular anaphase. In which 5.28% corresponded to cells with side arm bridges (SAB; 3.68% cells with one bridge and one fragment; 2.58% of irregular anaphase showed cells with one or two lagging chromosomes and 2.95% showed one acentric fragment; cells with two bridges and cells with two bridges and one acentric fragment were observed in frequencies of 1.60% and 1.35% respectively. In anaphase II some cells showed bridges and fragments too. Aberrant A-I cells had many shrunken or empty pollen grains (42.00% and 58.00 % viable pollen. Conclusion The observed meiotic irregularities suggest that structural chromosome aberrations have occurred, such as heterozygous inversions, sister chromatid exchanges, deletions and duplications which in turn are reflected in a low pollen viability.

  6. MAX WEBER AND ERNEST BERNEA UNIVERSAL AND LOCAL VISIONS OF THE SPIRITUAL DIMENSION OF SOCIETY

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    MĂDĂLINA MĂNDIŢĂ

    2015-05-01

    Full Text Available In this study we analyze two ways of understanding the role and importance of the spiritual dimension of social reality: first from a universal point of view with the sociology of Max Weber concerning the research of the religious phenomenon, and secondly from a local point of view, aided by the contributions of Eugeniu Sperantia, Petre Andrei and especially Ernest Bernea relative to the basic elements necessary to understand society. Both in comprehensive sociology and in Romanian interwar sociology we find the necessity of a complex research of social reality, in order to avoid the perils represented by the materialistic or idealistic views of sociology. If Weber rejects these two limited positions by making efforts to understand social actions, and thus coming to be labeled a causalist, Bernea also advocates for a sociology that reaches a synthesis of society, considering himself to be an adherent of integral realism. A common element of these two ways of conceiving and applying sociology (that of Weber – which has demonstrated its universal capability, and that developed by Bernea – which has proved its local capability is the recognition of the importance of the spiritual dimension and the imperative of multiple conditionality in the scientific understanding of social realities. In this article we are concerned with the way and the incentive of these two sociologists for an interpretative knowledge of society and for a whole realistic knowledge of social reality

  7. Glioblastoma multiforme in Klippel-Trenaunay-Weber syndrome: a case report.

    Science.gov (United States)

    Yilmaz, Tevfik; Cikla, Ulas; Kirst, Alice; Baskaya, Mustafa K

    2015-04-17

    Klippel-Trenaunay-Weber syndrome (KTWS) is a rare syndrome in which patients usually present with cutaneous hemangiomas, venous varicosities, and bone and soft tissue hypertrophy of the affected limb. Intracranial lesions in patients with KTWS are extremely rare, and are generally reported as single cases in the literature. We describe a rare case, where a patient with KTWS was found with a hemorrhagic grade IV astrocytoma. Although central nervous system abnormalities such as intracranial aneurysms and cerebral and spinal cord cavernomas have been described in patients with KTWS, to the best of our knowledge, this is the first report of an association between glioblastoma multiforme (grade IV astrocytoma) and KTWS in the English-language medical literature. A 61-year-old white Caucasian man with a history of KTWS presented with seizures. Left upper and lower extremity hypertrophy, left foot, leg and ear gigantism and left-sided abdominal capillary hemangiomas were noted in the physical examination. Cranial computed tomography (CT) and magnetic resonance imaging (MRI) were obtained, showing a heterogeneous lesion in the cingulate gyrus, with peripheral and central areas of T1 hyperintensity and layering T2 hypointensity consistent with a hemorrhage. A right parasagittal frontal craniotomy was performed with an interhemispheric approach. We had difficulty controlling the bleeding with bipolar electrocautery during surgery and finally were able to stop the bleeding using surgicel and gelfoam. Postoperative cranial CT and MRI scans showed intraparenchymal hemorrhage centered within the medial right frontal lobe. There was no increase in hematoma size in consecutive CT scans. Co-occurrence of vascular abnormalities with KWTS should be taken into consideration to avoid perilous preoperative and postoperative complications.

  8. Long-term intraocular pressure after combined trabeculotomy-trabeculectomy in glaucoma associated with Sturge-Weber syndrome.

    Science.gov (United States)

    Sood, Devindra; Rathore, Aanchal; Sood, Ishaana; Kumar, Dinesh; Sood, Narender N

    2018-03-01

    Vision loss in Sturge-Weber syndrome (SWS), a rare congenital disorder, is primarily due to glaucoma. We reviewed the data of all consecutive SWS-associated glaucoma cases in patients who had undergone combined trabeculotomy-trabeculectomy (CTT) at a tertiary glaucoma facility between January 1993 and December 2015. We analyzed the preoperative and postoperative intraocular pressure (IOP), corneal clarity, visual acuity, success rate, need for repeat surgery, and number of topical antiglaucoma medications needed at last follow-up. Twenty-six eyes of 20 patients with SWS (surgical age 0.7-96 months; mean 18.64 ± 29.74 months) had undergone primary CTT. The mean preoperative IOP was 32.76 ± 7.86 mm Hg (range 22-54 mm Hg) with medication (mean 3.11 ± 1.17; range 1-5). At the last follow-up (61-288 months); mean SD 134.73 ± 67.77 months), two eyes had IOP glaucoma in children. Long-term visual and surgical outcomes are encouraging.

  9. Freud, Weber, Durkheim: A Philosophical Foundation for Writing in the Humanities and Social Sciences.

    Science.gov (United States)

    LeFevre, Karen B.; Larkin, T. J.

    1983-01-01

    Proposes a continuum of lines of inquiry applicable to many of the human sciences. Illustrates the continuum by discussing the approaches of Sigmund Freud, Max Weber, and Emile Durkheim. Suggests uses of the continuum as an aid to invention and as a method of analysis. (RAE)

  10. Infraslow status epilepticus: A new form of subclinical status epilepticus recorded in a child with Sturge-Weber syndrome.

    Science.gov (United States)

    Bello-Espinosa, Luis E

    2015-08-01

    Analysis of infraslow EEG activity (ISA) has shown potential in the evaluation of patients with epilepsy and in the differentiation between focal and generalized epilepsies. Infraslow EEG activity analysis may also provide insights into the pathophysiology of refractory clinical and subclinical status epilepticus. The purpose of this report is to describe a girl with Sturge-Weber syndrome (SWS) who presented with a 96-h refractory encephalopathy and nonischemic hemiparesis and who was identified to have infraslow status epilepticus (ISSE), which successfully resolved after midazolam administration. The continuous EEG recording of a 5-year-old girl with known structural epilepsy due to Sturge-Weber syndrome is presented. The patient presented to the ED with acute confusion, eye deviation, and right hemiparesis similar to two previous admissions. Despite administration of lorazepam, fosphenytoin, phenobarbital, and valproic loads, the patient showed no improvement in the clinical condition after 48 h. The continuous video-EEG monitoring (VEM) showed continuous severe diffuse nonrhythmic asymmetric slowing but no apparent ictal activity on continuous conventional EEG recording settings. As brain CT, CTA, CTV, and complete MRI scans including DWI obtained within 72 h of presentation failed to demonstrate any ischemic changes, analysis of the EEG infraslow (ISA) activity was undertaken using LFF: 0.01 Hz and HFF: of 0.1 Hz, respectively. Continuous subclinical unilateral rhythmic ictal ISA was identified. This was only evident on the left hemisphere which correlated with the structural changes due to SWS. A trial of continuous 120 to 240 μg/kg/h of IV midazolam resulted in immediate resolution of the contralateral hemiparesis and encephalopathy. Continuous prolonged rhythmic ictal infraslow activity (ISA) can cause super-refractory subclinical focal status epilepticus. This has not been previously reported, and we propose that this be called infraslow status

  11. TRANSMITTING CHARISMA: Re-reading Weber through the Traditional Islamic Leader in Modern Java

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    Achmad Zainal Arifin

    2016-09-01

    Full Text Available This paper tries to reconsidering the theory of authority, propounded by Max Weber, a pioneer of classical theory of sociology, particularly on his idea of charisma. Weber predicted that modernisation, as represented by the rationalisation process which is unstoppable phenomena for modern world lead the changing process of charismatic authority into other forms of authorities, especially the legal-rational one. By describing the dynamics of Kyai’s charismatic authority within the pesantren milieu, the rationalisation process tends to enhance the existence of charismatic authority of kyai. Based on a case study of a famous traditional pesantren in Yogyakarta, this paper elaborates the process of rationalisation describes how all instruments of traditional pesantren, including those that are resulted from the process, have contribution in preserving and maintaining the charismatic authority o kyai. Most members of traditional pesantren even believe that the quality of charisma, which is laid on the foundation of supernatural power, can be transmitted to the next successor through the institutionalisation of practices to gain that power.

  12. Grasping trajectories in a virtual environment adhere to Weber's law.

    Science.gov (United States)

    Ozana, Aviad; Berman, Sigal; Ganel, Tzvi

    2018-06-01

    Virtual-reality and telerobotic devices simulate local motor control of virtual objects within computerized environments. Here, we explored grasping kinematics within a virtual environment and tested whether, as in normal 3D grasping, trajectories in the virtual environment are performed analytically, violating Weber's law with respect to object's size. Participants were asked to grasp a series of 2D objects using a haptic system, which projected their movements to a virtual space presented on a computer screen. The apparatus also provided object-specific haptic information upon "touching" the edges of the virtual targets. The results showed that grasping movements performed within the virtual environment did not produce the typical analytical trajectory pattern obtained during 3D grasping. Unlike as in 3D grasping, grasping trajectories in the virtual environment adhered to Weber's law, which indicates relative resolution in size processing. In addition, the trajectory patterns differed from typical trajectories obtained during 3D grasping, with longer times to complete the movement, and with maximum grip apertures appearing relatively early in the movement. The results suggest that grasping movements within a virtual environment could differ from those performed in real space, and are subjected to irrelevant effects of perceptual information. Such atypical pattern of visuomotor control may be mediated by the lack of complete transparency between the interface and the virtual environment in terms of the provided visual and haptic feedback. Possible implications of the findings to movement control within robotic and virtual environments are further discussed.

  13. Molecular structures of fructans from Agave tequilana Weber var. azul.

    Science.gov (United States)

    Lopez, Mercedes G; Mancilla-Margalli, Norma A; Mendoza-Diaz, Guillermo

    2003-12-31

    Agave plants utilize crassulacean acid metabolism (CAM) for CO(2) fixation. Fructans are the principal photosynthetic products generated by agave plants. These carbohydrates are fructose-bound polymers frequently with a single glucose moiety. Agave tequilana Weber var. azul is an economically important CAM species not only because it is the sole plant allowed for tequila production but because it is a potential source of prebiotics. Because of the large amounts of carbohydrates in A. tequilana, in this study the molecular structures of its fructans were determined by fructan derivatization for linkage analysis coupled with gas chromatography-mass spectrometry (GC-MS), nuclear magnetic resonance (NMR), and matrix-assisted laser desorption time-of-flight mass spectrometry (MALDI-TOF-MS). Fructans were extracted from 8-year-old A. tequilana plants. The linkage types present in fructans from A. tequilana were determined by permethylation followed by reductive cleavage, acetylation, and finally GC-MS analysis. Analysis of the degree of polymerization (DP) estimated by (1)H NMR integration and (13)C NMR and confirmed by MALDI-TOF-MS showed a wide DP ranging from 3 to 29 units. All of the analyses performed demonstrated that fructans from A. tequilana consist of a complex mixture of fructooligosaccharides containing principally beta(2 --> 1) linkages, but also beta(2 --> 6) and branch moieties were observed. Finally, it can be stated that fructans from A. tequilana Weber var. azul are not an inulin type as previously thought.

  14. Stewart-Bluefarb acroangiodermatitis in a case of Parkes-Weber syndrome

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    Deepti H Ghia

    2014-01-01

    Full Text Available Stewart-Bluefarb acroangiodermatitis is the occurrence of pseudo-kaposiform changes with an underlying arterio-venous malformation. Parkes-Weber syndrome is a triad of arterio-venous malformation with varicose veins with bony and soft-tissue hypertrophy involving the extremity. A 13 year-old-female presented with ulcerated growth with bleeding episodes on right leg since past four years. A reddish discoloration over right leg was noticed at four years of age which remained unchanged until nine years of age, after which it showed rapid increase in size along with disproportionate increase in length and girth of right lower limb. Examination revealed hyperpigmented soft verrucous plaque over the right lower one-third of leg, along with presence of ulcers and dilated tortuous veins in the right lower leg with thrill and bruit over the right popliteal and inguinal region. A differential diagnosis of verrucous hemangioma and arterio-venous malformation with pseudo-kaposiform change was considered. Skin biopsy showed multiple fenestrated capillaries with perivascular lymphocyte infiltrate suggestive of capillary malformations. X-ray showed osteolytic defect in right tibia. Duplex ultrasound and magnetic resonance imaging of right leg showed multiple fast flowing small and medium sized arterio-venous malformations of small to moderate size. Thus, on the basis of clinical and radiological features, we made a diagnosis of Stewart-Bluefarb type of acroangiodermatitis with Parkes-Weber syndrome. She was managed conservatively with compression stockings.

  15. History and sociology – the First Century. From Ranke to Weber

    OpenAIRE

    Tyrell, Hartmann

    2010-01-01

    The contribution deals with the complicated history of the sciences of history and sociology (predominantly in Germany). It shows, firstly, how Ranke formulated one of his essential world-historical insights as definitely closing off from contemporary discourses of the social and the »social movement«. Almost one hundred years later Max Weber integrated this insight of Ranke in his early days into his sociology. Secondly, Tyrell highlights the importance of Dilthey’s »studies of society and h...

  16. Was Weber Wrong? A Human Capital Theory of Protestant Economic History. PEPG/07-04

    Science.gov (United States)

    Becker, Sascha O.; Wohmann, Ludger

    2007-01-01

    Max Weber attributed the higher economic prosperity of Protestant regions to a Protestant work ethic. We provide an alternative theory, where Protestant economies prospered because instruction in reading the Bible generated the human capital crucial to economic prosperity. County-level data from late 19th-century Prussia reveal that Protestantism…

  17. Moving the Weber Fraction: The Perceptual Precision for Moment of Inertia Increases with Exploration Force

    Science.gov (United States)

    Debats, Nienke B.; Kingma, Idsart; Beek, Peter J.; Smeets, Jeroen B. J.

    2012-01-01

    How does the magnitude of the exploration force influence the precision of haptic perceptual estimates? To address this question, we examined the perceptual precision for moment of inertia (i.e., an object's “angular mass”) under different force conditions, using the Weber fraction to quantify perceptual precision. Participants rotated a rod around a fixed axis and judged its moment of inertia in a two-alternative forced-choice task. We instructed different levels of exploration force, thereby manipulating the magnitude of both the exploration force and the angular acceleration. These are the two signals that are needed by the nervous system to estimate moment of inertia. Importantly, one can assume that the absolute noise on both signals increases with an increase in the signals' magnitudes, while the relative noise (i.e., noise/signal) decreases with an increase in signal magnitude. We examined how the perceptual precision for moment of inertia was affected by this neural noise. In a first experiment we found that a low exploration force caused a higher Weber fraction (22%) than a high exploration force (13%), which suggested that the perceptual precision was constrained by the relative noise. This hypothesis was supported by the result of a second experiment, in which we found that the relationship between exploration force and Weber fraction had a similar shape as the theoretical relationship between signal magnitude and relative noise. The present study thus demonstrated that the amount of force used to explore an object can profoundly influence the precision by which its properties are perceived. PMID:23028437

  18. Syndrome de Sturge-Weber : A propos d'un cas a Lome. | Adigo ...

    African Journals Online (AJOL)

    Le syndrome de Sturge-Weber est une rare maladie congénitale, sporadique et non héréditaire. Il implique des malformations vasculaires de la peau (angiome cutané plan), des yeux et du cerveau (angiome leptoméningé). Les auteurs rapportent un cas révélé à l'âge adulte et dont le traitement a été médical ...

  19. Moving the Weber fraction: the perceptual precision for moment of inertia increases with exploration force

    NARCIS (Netherlands)

    Debats, N.B.; Kingma, I.; Beek, P.J.; Smeets, J.B.J.

    2012-01-01

    How does the magnitude of the exploration force influence the precision of haptic perceptual estimates? To address this question, we examined the perceptual precision for moment of inertia (i.e., an object's "angular mass") under different force conditions, using the Weber fraction to quantify

  20. Durkheim's and Weber's notions of the State in Marxist political theory: similarities and differences

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    Graciela Alejandra Inda

    2009-05-01

    Full Text Available This article is a contribution to the comparatively scarce literature on how Durkheim and Weber - those canonical sociological theorists - conceived of the State. I shall examine just what relation each of their conceptions has to Marxist theory, and ask to what degree they coincide or diverge.    

  1. Weber e o desencantamento do mundo: uma interlocução com o pensamento de Nietzsche

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    Renarde Freire Nobre

    2006-01-01

    Full Text Available Dans cet article, on oppose la pensée de Max Weber à celle de Friedrich Nietzsche à propos de l'Occident moderne, en prenant comme point de départ les notions de "mort de Dieu" et de "désenchantement du monde". On y examine leurs affinités et différences, surtout sous l'angle du diagnostic de Weber sur la "culture de la technique" et ses effets inéluctables sur la subjectivité. L'hypothèse centrale, c'est que, malgré les points de contact qu'on peut et qu'on doit établir entre ces deux penseurs, il reste un fossé infranchissable, dû surtout à la reconnaissance des pouvoirs de la raison et à la vue du monde culturel construites sous la direction des orientations conscientes.

  2. On Legal Authority, Crisis of Legitimacy and Schooling in the Writings of Max Weber.

    Science.gov (United States)

    Lenhardt, Gero

    In an attempt to gain a better perspective on the relationship between education and the modern state, this paper reopens the theoretical debate on the key role of formal rationality in Max Weber's interpretation of the capitalist economy and the modern bureaucratic state. Against the background of an extensive review of the development and the…

  3. A Recepção Controversa de Max Weber no Brasil (1940-1980

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    Glaucia Villas Bôas

    2014-03-01

    Full Text Available El artículo analiza cómo se recibieron las ideas de Max Weber en las primeras décadas de la institucionalización de la sociología en Brasil, y argumenta que la apropiación de los postulados del autor alemán se hizo a partir de las cuestiones propias de la sociología brasileña. Este punto de vista permitió observar que proposiciones centrales de la sociología weberiana, como, por ejemplo, la formulación de los tipos ideales y del sentido de la ciencia, se recibieron con mucha parsimonia y se reelaboraron a medida que los textos del autor se fueron leyendo en contraste con otros autores. Inicialmente, se retoman textos de la revista Sociología; en seguida se discute la valorización de las proposiciones de Hans Freyer y Karl Mannheim en detrimento de las posiciones de Max Weber sobre el papel del sociólogo y, finalmente, se trata la controversia sobre los tipos ideales, liderada por Maria Sylvia de Carvalho Franco, que involucra los escritos de Florestan Fernandes y Fernando Henrique Cardoso.

  4. RELAÇÕES DE PODER E ESTRUTURA ORGANIZACIONAL EM WEBER E CHIAVENATO

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    Caio Calmon Vinícius de Souza

    2012-03-01

    Full Text Available O presente artigo tem por objetivo discutir a relação entre poder e estrutura organizacional buscando apontar a importância das relações de poder para a estruturação da organização. Para isso recorreu-se às contribuições de Max Weber (1979 e de Chiavenato (2003, ambos teóricos que trataram de questões envolvendo poder e estrutura organizacional.

  5. Max Weber: la racionalización del mundo como proceso histórico-universal

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    Eduardo Weisz

    2011-01-01

    Full Text Available Este artículo examina la sociología de la religión de Max Weber centrándose en el surgimiento de las religiones de salvación. La tendencia universal a la racionalización se presenta allí como una fuerza que en cada caso histórico debe confrontar con otras variables. El judaísmo antiguo puede verse entonces no solo como antecedente de Occidente, sino también como el producto de una combinación de factores históricos que permitió que la fuerza racionalizadora se haya desplegado con mayor intensidad. Esto permite constituirlo en herramienta heurística para estudiar otras civilizaciones. En China e India, la racionalización se vio impedida o inhibida por distintas variables. La ratio, entonces, es para Weber una fuerza motriz universal, en torno a la que construye su fi losofía de la historia. Así, la mirada weberiana sobre la modernidad queda enmarcada metahistóricamente.

  6. Arte, erotismo y redención, la esfera estética en el pensamiento de Max Weber

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    Ana María Rodríguez Sierra

    2016-07-01

    Full Text Available La prolífica obra de Max Weber ha sido estudiada ampliamente, sin embargo, la concepción que tenía del arte, aunque presente en sus escritos, no ha sido indagada. En Sociología de la religión Weber aborda la esfera estética de un modo que vale la pena conocer y destacar, allí se presenta una concepción filosófica del arte, amplia y ahistórica, en la que el arte y el erotismo se ligan y se muestran como medios de redención en un mundo desencantado. Este artículo expone la filosofía del arte que subyace en los últimos textos del científico social alemán.

  7. Max Weber : uma leitura da sociologia da religião

    OpenAIRE

    Julia Maria de Souza Rodrigues

    2001-01-01

    Resumo: A religião é, para Weber, a chave de interpretação para o entendimento de processos culturais mais amplos, como o desencantamento do mundo e a secularização. Além disso, ele a define como estilo de vida próprio fomentado pelo indivíduo, que, conseqüentemente, interfere na conduta de um grupo ou de uma coletividade historicamente determinados. A análise da religião possibilita também a compreensão interpretativa de "individualidades históricas", ou seja, das seis religiões ou sistemas ...

  8. Feng shui väärtus on vaimujõus / Marika Tomberg ; komment. Lea Eermann, komment. Rein Weber

    Index Scriptorium Estoniae

    Tomberg, Marika

    1999-01-01

    Feng shui järgi korraldatud Lea Eermanni kodust Nõmmel. Feng shui- ja geobioloogia-alase nõustamisega tegelev arhitekt Rein Weber keskkonna mõjust tervisele, magamistoast. Enda ja maja kaitsmisest halbade mõjude eest. 10 illustratsiooni

  9. Weber, Schutz e a busca de "sentido" na ação social: uma análise comparativa

    OpenAIRE

    Eliane Fioravante Garcez

    2014-01-01

    http://dx.doi.org/10.5007/1806-5023.2014v11n1p63   O artigo discorre sobre as teorias de Max Weber e de Alfred Schutz e a aproximação desses mundos teóricos com a fenomenologia do filósofo Edmund Husserl. No texto são explorados os conceitos de sentido, compreensão, ação, ação social, relação social dentre outros. São apresentados também a tipologia da ação e os dois níveis de análise sociológica (microssocial e macrossocial) na concepção de Weber, para quem a ação é fruto da subjetiv...

  10. The possibilities of applying infrared lasers in otolaryngology

    Science.gov (United States)

    Kukwa, Andrzej; Tulibacki, Marek P.; Wojtowicz, Piotr; Oledzka, Iwona; Obarska, Agnieszka

    2003-10-01

    The authors present their many years of experience in the area of laryngological surgery with various types of solid lasers. In our clinical practice we use Nd-YAG, Ho-YAG and Er-YAG surgical lasers. There are many reasons for applying lasers, depending on medical conditions. One of the most frequent cases are the nasal polyps, where surgical treatment is aimed at curing the results. For this type the procedures there was applied Nd-YAG laser, which has shortened the period of treatment and extended the remission. We also use this laser for mucal tissue corrections of the nasal conchs through deep coagulation effect. The above mentioned laser is also useful for the preventative treatment of granulation stenosis by removal of granulation tissue from different sections of the upper way; from the nose to the trachea.Other laser procedures in the nasal area include coagulation of Kisselbach locus, bleeding in Rendu-Osler disease, separating adhesions of the nasal concha from the nasal septum, coagulation of septal bleeding polyps, angiomas of different location, small papillomae of throat and nose, and reduction of hypertrophy nasopharyngeal mucoosa in OSAS patients, and uvolo-palato-pharyngoplasty (UPPP). Neodymium laser is also used for the management of neoplastic diseases in palliative and radical (CA. basocellulare) treatment for the resection of the premalignant changes (papilloma, leukoplakia) and early neoplastic changes of throat and larynx. Keloplasty in the area of larynx and trachea is performed with the use of Ho-YAG laser, with shallow effect, regardless of the density of tissue, enabling coagulation of vascular vessels. It enables non-traumatic resections of bone structures, such as the wall of maxillary sinus, nasal spine, or conchoplasty; the mucal tissue and the frame. Recent clinical tests are examining the possibilities of Er-YAG laser application for crushing calculus in the salivary ducts. Using lasers in laryngology enables their extensive

  11. The emergence of Islam through the theoretical prism of Max Weber: The role of spatial context, social actors and social strata in the process of defining and expansion of the Islamic religion

    Directory of Open Access Journals (Sweden)

    Jovanović Nataša

    2016-01-01

    Full Text Available Weber's theoretical legacy contains only fragments of thinking about Islam, primarily in the texts and monographs that as the main object of study did not have this religion. This fact is a big problem to contemporary researchers who want to reconstruct Weber's perception of Islam and positioning it in its theoretical and methodological coordinates. However, this barrier gives rise to modern researchers to engage in the challenge of reconstruction of the mentioned segment Weber's thoughts. This paper is an attempt of such a reconstruction. The paper analyzes the emergence of Islam, ie. spatial and social context of the founding of the Islamic religious dogma. The author starts from the hypothesis that in Weber's theoretical and analytical equation the importance of urban areas for the development and spread of Islam is absent. Also, in the paper author points to another error that Weber makes. Weber classified Islam in the category of warrior religion, and ignored the role of trade and commercial bed in the formulation of Islamic dogma and the (economic practices. In order to more adequately respond to the request 'placement' of Islam in the theoretical coordinates of Weber's sociology (of religion, author of the paper emphasizes the universality of the rationalization process, proving that Islam is one of the better examples of substantive rationality in the religious domain (because Islamic dogma implies a holistic approach to the believer and the high level of submission to the sacred text in all areas of its life.

  12. A questão universitária em Max Weber: entre escritos metodológicos e Sociologia Política

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    Mariana Toledo Ferreira

    2015-06-01

    Full Text Available O presente artigo busca tratar da concepção de educação em Max Weber e, mais especificamente, em como ele aborda a questão universitária, em uma série de seus escritos. Além de textos mais conhecidos, tal qual Ciência como vocação, o estudo incorpora conferências e artigos de intervenção escritos por Weber e alguns de seus trabalhos identificados com sua Sociologia Política. Assim, se o processo de desencantamento do mundo é vital para que se consiga interpretar os diagnósticos elaborados à época, também ocupam lugar central o papel do docente e o conceito de probidade intelectual. Analogamente, o avanço da burocracia – em alguns casos, de maneira a cercear o trabalho intelectual e acadêmico – é tematizado pelo autor, notadamente pela análise crítica do “caso Bernhard” e do “sistema Althoff”. Este artigo busca, então, retraçar essas discussões de Weber, inserindo-as em um quadro analítico mais amplo, ao relacionar sua discussão sobre a educação a conceitos elaborados tanto no âmbito de sua Sociologia da religião quanto de sua Sociologia política. Por fim, delineia-se brevemente o modo como Weber reflete sobre o sentido da liberdade, sobretudo aquela de cunho acadêmico, para destacar a relevância que assumiriam formas de organização dos professores que pudessem atuar em defesa da autonomia exigida pelo trabalho científico-intelectual sério.

  13. Hemodynamics in the Sturge-Weber syndrome utilizing stable Xe-CT; With special reference to the perfusion reserve and the epileptic focus activated by megimide

    Energy Technology Data Exchange (ETDEWEB)

    Okudaira, Yojiro; Bandoh, Kuniaki; Ito, Masanori; Sato, Kiyoshi; Tsuji, Masahiro (Juntendo Univ., Tokyo (Japan). School of Medicine)

    1993-02-01

    In 4 Sturge-Weber-syndrome child patients with calcified lesions mainly in the occipital lobe, regional cerebral blood flow (rCBF) was determined with stable Xe-CT in the resting state and after iv injection of acetazolamide (AA) and megimide (M), with the purpose of examining factors affecting deterioration of neurologic manifestations and the influence of lobectomy. In the resting state, rCBF of the temporal and occipital areas was significantly lower on the affected than unaffected sides. The side-to-side asymmetry of rCBF in the affected and unaffected sides decreased from the frontal to temporal and occipital areas. It was indistinct after AA injection because cerebral vasoreactivity became higher on the affected side. A low rCBF in association with a high cerebral vasoreactivity on the lesion side suggested that the low rCBF matched the low cerebral metabolism of the brain area affected by leptomeningeal angiomatosis. However, cerebral vasoreactivity to AA depended on clinical presentations. Two patients presented with progressive mental retardation. The other two patients were in clinically stable condition. Cerebral vasoreactivity to AA in the former two cases was poorer on both the affected and unaffected sides than that in the latter two cases. M administration was associated with a significantly decreased rCBF in the area of leptomeningeal angiomatosis and cerebral calcification and with a significantly increased rCBF of the 'pericalcified' area. In one patient undergoing extended occipital lobectomy on the affected side, no decrease in rCBF was noted in the resting state in either affected or unaffected side, and cerebral vasoreactivity to AM on the unaffected side was apparently increased. Both circulatory disturbance and seizure appear to play a role in clinical presentations, as well as their deterioration in Sturge-Weber-Syndrome patients. (N.K.).

  14. La teoría pura del derecho y la secularización incompleta del derecho: Hans Kelsen y Max Weber

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    Isaac de León Beltrán

    2006-01-01

    Full Text Available Este trabajo trata de mostrar algunas similitudes entre los pensamientos teóricos de Hans Kelsen y de "'Max Weber en torno a la noción de derecho-Estado. Se muestra que la discusión en torno a la validez y al valor del derecho se puede plantear en términos de la consolidación del Estado moderno. También se propone una explicación de las tensiones entre el iusnaturalismo al que se enfrenta Kclsen y aquello que he denominado la secularización incompleta del derecho. Palabras clave: Weber, Kelsen, formación del Estado, teoría del derecho.

  15. Results of operative fixation of unstable ankle fractures in geriatric patients.

    Science.gov (United States)

    Pagliaro, A J; Michelson, J D; Mizel, M S

    2001-05-01

    It is widely accepted that operative fixation of unstable ankle fractures yields predictably good outcomes in the general population. The current literature, however reports less acceptable results in the geriatric population age 65 years and older. The current study analyzes the outcome of the surgical treatment of unstable ankle fractures in patients at least 65 years old. Twenty three patient over 65 years old were surgically treated after sustaining 21 (91%) closed and 2 (9%) open grade II unstable ankle fractures. Fractures were classified according to the Danis-Weber and Lauge-Hansen schemes. Fracture type was predominantly Weber B (21/23, 91%), or supination external rotation stage IV (21/23, 91%). Fracture union rate was 100%. There were three significant complications including a lateral wound dehiscence with delayed fibular union in an open fracture dislocation, and two below knee amputations, neither of which was directly related to the fracture treatment. There were three minor complications; one superficial wound infection and two cases of prolonged incision drainage, all of which resolved without further surgical intervention. Complications were associated with open fractures and preexisting systemic disease. These results indicate that open reduction and internal fixation of unstable ankle fractures in geriatric patients is an efficacious treatment regime that with results that are comparable to the general population.

  16. MR imaging of white-matter diseases in children

    International Nuclear Information System (INIS)

    Sato, Y.; Yuh, W.T.C.; Mathews, K.; Wiese, J.; Kao, S.; Schreiber, A.; Farner, R.; Smith, W.

    1987-01-01

    MR imaging has become a valuable tool in the investigation of central nervous system abnormalities in children. This exhibit displays the MR imaging patterns in 30 children with diseases involving the white matter. Clinical, CT, and pathologic findings will be presented for comparison. The white matter disease entities studies include acquired white matter diseases, metabolic diseases, and phycomatoses. Specific examples include acute dissemination encephalomyelitis, anoxic encephalopathy, disseminated necrotizing leukoencephalopathy, demyelinating adrenoleukodystrophy, Krabbe disease, metachromatic leukodystrophy, Tay-Sachs disease, Gaucher disease, neurofibromatosis, and Sturge-Weber syndrome

  17. Visually and memory-guided grasping: aperture shaping exhibits a time-dependent scaling to Weber's law.

    Science.gov (United States)

    Holmes, Scott A; Mulla, Ali; Binsted, Gordon; Heath, Matthew

    2011-09-01

    The 'just noticeable difference' (JND) represents the minimum amount by which a stimulus must change to produce a noticeable variation in one's perceptual experience and is related to initial stimulus magnitude (i.e., Weber's law). The goal of the present study was to determine whether aperture shaping for visually derived and memory-guided grasping elicit a temporally dependent or temporally independent adherence to Weber's law. Participants were instructed to grasp differently sized objects (20, 30, 40, 50 and 60mm) in conditions wherein vision of the grasping environment was available throughout the response (i.e., closed-loop), when occluded at movement onset (i.e., open-loop), and when occluded for a brief (i.e., 0ms) or longer (i.e., 2000ms) delay in advance of movement onset. Within-participant standard deviations of grip aperture (i.e., the JNDs) computed at decile increments of normalized grasping time were used to determine participant's sensitivity to detecting changes in object size. Results showed that JNDs increased linearly with increasing object size from 10% to 40% of grasping time; that is, the trial-to-trial stability (i.e., visuomotor certainty) of grip aperture (i.e., the comparator) decreased with increasing object size (i.e., the initial stimulus). However, a null JND/object size scaling was observed during the middle and late stages of the response (i.e., >50% of grasping time). Most notably, the temporal relationship between JNDs and object size scaling was similar across the different visual conditions used here. Thus, our results provide evidence that aperture shaping elicits a time-dependent early, but not late, adherence to the psychophysical principles of Weber's law. Copyright © 2011 Elsevier Ltd. All rights reserved.

  18. High fidelity simulation and analysis of liquid jet atomization in a gaseous crossflow at intermediate Weber numbers

    Energy Technology Data Exchange (ETDEWEB)

    Li, Xiaoyi, E-mail: lixy2@utrc.utc.com; Soteriou, Marios C. [United Technologies Research Center, East Hartford, Connecticut 06108 (United States)

    2016-08-15

    Recent advances in numerical methods coupled with the substantial enhancements in computing power and the advent of high performance computing have presented first principle, high fidelity simulation as a viable tool in the prediction and analysis of spray atomization processes. The credibility and potential impact of such simulations, however, has been hampered by the relative absence of detailed validation against experimental evidence. The numerical stability and accuracy challenges arising from the need to simulate the high liquid-gas density ratio across the sharp interfaces encountered in these flows are key reasons for this. In this work we challenge this status quo by presenting a numerical model able to deal with these challenges, employing it in simulations of liquid jet in crossflow atomization and performing extensive validation of its results against a carefully executed experiment with detailed measurements in the atomization region. We then proceed to the detailed analysis of the flow physics. The computational model employs the coupled level set and volume of fluid approach to directly capture the spatiotemporal evolution of the liquid-gas interface and the sharp-interface ghost fluid method to stably handle high liquid-air density ratio. Adaptive mesh refinement and Lagrangian droplet models are shown to be viable options for computational cost reduction. Moreover, high performance computing is leveraged to manage the computational cost. The experiment selected for validation eliminates the impact of inlet liquid and gas turbulence and focuses on the impact of the crossflow aerodynamic forces on the atomization physics. Validation is demonstrated by comparing column surface wavelengths, deformation, breakup locations, column trajectories and droplet sizes, velocities, and mass rates for a range of intermediate Weber numbers. Analysis of the physics is performed in terms of the instability and breakup characteristics and the features of downstream

  19. Addressing the Specificity of Social Concepts: Rickert, Weber, and the Dual contrast Theory

    OpenAIRE

    Dewalque, Arnaud

    2016-01-01

    In this chapter, it is argued that Weber's particular combination of Von Kries' naturalistic paradigm and Rickert's antinaturalistic paradigm might become less puzzling if we return to the interpretation that emerged in the middle of the nineteen-twenties within the South-Western School of neo-Kantianism. The basic intuition which underlies this interpretation is that the social sciences are best understood as generalizing cultural sciences. On this understanding, they differ both from the na...

  20. WEBER AND THE LUXURY CONSUMPTION: A DISCUSSION ABOUT “THE PROTESTANT ETHIC AND THE SPIRIT OF CAPITALISM”

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    Priscilla Schramm Portugal

    2007-12-01

    Full Text Available The magnitude of the sociologist Max Weber’s studies is undeniable in the Social Sciences history, but does his book, The Protestant Ethic and the Spirit of Capitalism, explain the luxury consumption nowadays? In this article, it is going to be demonstrated some critiques of this book of Weber, made by authors who believe that the sociologist has disdained some important data that influenced the whole society in the 17th century, as the fashion, the value of romantic love and the European society outlook in general. Campbell, for example, says that because this “historical segmentation” Weber had ignored important social changes. If, when it was written, Weber’s book could not explain the luxury consumption, this fact is more evident nowadays, whereas the asceticism is not predominant in the occidental societies and even developing countries have consumed luxury articles.

  1. Telltale signs of patient-centered diagnosis.

    Science.gov (United States)

    Millenson, Michael L

    2014-01-01

    A best-selling book from the mid-1980s was entitled, All I Really Need to Know I Learned in Kindergarten. Some doctors may similarly feel that well-worn epigrams from Hippocrates, Osler and others have told them all they really need to know about patient-centered care. The problem is that aphorisms and action are not one and the same. The workup for patient-centered diagnosis takes work, and there are telltale signs along the way. Effective patient engagement requires training and practice. It means incorporating patient-generated data into the diagnostic process. And it means being sensitive to new economic constraints on patients. Ensuring that diagnostic processes and decisions meet the test of patient-centeredness poses a challenge. The new criteria do not replace the professional obligation of beneficence; rather they add an additional obligation of power sharing. While that is neither simple nor easy, it promises better care for patients, a more satisfying clinical encounter and a better health care system for all.

  2. Una revisión histórica de los métodos clásicos de resolución del problema de Fermat-Weber.

    Directory of Open Access Journals (Sweden)

    Ma Belén Cobacho Tornel

    2004-01-01

    Full Text Available El problema de Weber consiste en calcular la localizaci´on ´optima de un punto de modo que sea m´ınima la suma ponderada de las distancias desde dicho punto a un conjunto de puntos dados. Este problema, que tiene su aplicacion economica en el ambito de la localizaci´on y que ha sido ampliamente tratado en la literatura cientıfica, tiene como origen una cuestion matematica formulada en el siglo XVII por Fermat. Los primeros algoritmos de resolucion, propuestos por Torricelli y Simpson, estaban basados en razonamientos puramente geometricos, aunque tambien es destacable la existencia la denominada m´aquina de Varignon, un ingenio mecanico que permite determinar el ´optimo del problema de Weber. A pesar de que estos m´etodos son citados en numerosas fuentes bibliograficas, las demostraciones de los teoremas en los que se sustentan no son tan accesibles para investigadores y docentes por tratarse de textos clasicos con varios siglos de antig¨uedad. En este trabajo, ademas de realizar una revisi´on de diversos m´etodos empleados a lo largo de la historia para resolver el problema de Weber, se presentan demostraciones basadas en argumentos geometricos y fısicos que demuestran la veracidad de estos algoritmos, ası como algunas conclusiones y deducciones sobre el problema de Weber que se extraen de los razonamientos presentados.

  3. Operationalizing Max Weber's probability concept of class situation: the concept of social class.

    Science.gov (United States)

    Smith, Ken

    2007-03-01

    In this essay I take seriously Max Weber's astonishingly neglected claim that class situation may be defined, not in categorial terms, but probabilistically. I then apply this idea to another equally neglected claim made by Weber that the boundaries of social classes may be determined by the degree of social mobility within such classes. Taking these two ideas together I develop the idea of a non-categorial boundary 'surface' between classes and of a social class 'corridor' made up of all those people who are still to be found within the boundaries of the social class into which they were born. I call social mobility within a social class 'intra-class social mobility' and social mobility between classes 'inter-class social mobility'. I also claim that this distinction resolves the dispute between those sociologists who claim that late industrial societies are still highly class bound and those who think that this is no longer the case. Both schools are right I think, but one is referring to a high degree of intra-class social mobility and the other to an equally high degree of inter-class mobility. Finally I claim that this essay provides sociology with only one example among many other possible applications of how probability theory might usefully be used to overcome boundary problems generally in sociology.

  4. Síndrome de Sturge-Weber: relato de caso dos achados da avaliação fonoaudiológica Sturge-Weber syndrome: a case report on the results of the phonoaudiological evaluation

    Directory of Open Access Journals (Sweden)

    Sara Virgínia Paiva Santos

    2010-02-01

    Full Text Available TEMA: avaliação fonoaudiológica em síndrome de rara ocorrência. PROCEDIMENTOS: descrever o desempenho apresentado na avaliação fonoaudiológica clínica de uma paciente com diagnóstico genético de Síndrome de Sturge-Weber. Para isso, utilizou-se de avaliação da motricidade e funções orofaciais, das habilidades pragmática e semântica da linguagem oral, processos perceptuais visual e auditivo, funções cognitivas e aplicação do Denver II. RESULTADOS: na avaliação da motricidade orofacial a paciente apresentou anomalias de estruturas ósseas da face, inadequação quanto à morfologia, mobilidade e tônus de todos os órgãos fonoarticulatórios, presença de reflexos de procura e sucção primitivos. Na avaliação das habilidades pragmática e semântica da linguagem oral, processos perceptuais visual e auditivo, funções cognitivas, a paciente apresentou exploração sensoriomotora, com pouca interação e atenção compartilhada e dificuldade de contato de olhos; comunicação predominantemente gestual, não havendo registros de atos comunicativos verbais. Apresentou funções comunicativas de pedido de ação, exclamativa, exploratória e protesto, e grande uso de função não focalizada. A compreensão oral mostrou-se alterada, gestos representativos esporádicos, processos perceptuais funcionais e lateralidade indefinida. No Denver II, falhou nas áreas pessoal-social, linguagem, motor fino e grosseiro. CONCLUSÃO: a paciente apresentou diagnóstico fonoaudiológico de Distúrbio de Linguagem e Disfagia Neurogênica Orofaríngea moderada sendo necessária intervenção fonoaudiológica a fim de maximizar a comunicação, bem como adequar as estruturas e funções motoras orofaciais.BACKGROUND: phonoaudiological evaluation of rare occurrence syndrome. PROCEDURES: describe the performance shown in phonoaudiological clinical evaluation of a patient with genetic diagnosis of Sturge-Weber Syndrome. Evaluation of the drive

  5. Renal disease in patients with celiac disease.

    Science.gov (United States)

    Boonpheng, Boonphiphop; Cheungpasitporn, Wisit; Wijarnpreecha, Karn

    2018-04-01

    Celiac disease, an inflammatory disease of small bowel caused by sensitivity to dietary gluten and related protein, affects approximately 0.5-1% of the population in the Western world. Extra-intestinal symptoms and associated diseases are increasingly recognized including diabetes mellitus type 1, thyroid disease, dermatitis herpetiformis and ataxia. There have also been a number of reports of various types of renal involvement in patients with celiac disease including diabetes nephropathy, IgA nephropathy, membranous nephropathy, membranoproliferative glomerulonephritis, nephrotic syndrome related to malabsorption, oxalate nephropathy, and associations of celiac disease with chronic kidney disease and end-stage kidney disease. This review aims to present the current literature on possible pathologic mechanisms underlying renal disease in patients with celiac disease.

  6. [Osteosynthesis of Weber B ankle fractures using the one-third tubular plate and refixation of the syndesmosis].

    Science.gov (United States)

    Spering, C; Lesche, V; Dresing, K

    2015-08-01

    Anatomical reconstruction and recovery to complete range of function of the upper ankle joint. Therefore, the most stable but least invasive osteosynthesis is required to enable the patient early functional mobilization. Supination and pronation fracture with luxation mechanism of the upper ankle joint with or without rupture of the syndesmosis. Open fracture of the distal fibula including displaced and instable fractures. Severe peripheral arterial occlusive disease; contaminated open fractures (≥ 2nd degree); pediatric fractures with open epiphyseal plate. Supine position with ipsilateral slightly elevated hip and knee. Incision of about 8 cm length along the dorsal edge of the distal fibula. When reaching the lateral malleolus, a slight ventral angulation is necessary. Open reduction through this posterolateral approach. Secure the reposition using an interfragmentary lag screw and anatomically adjusted third tubular plate. Followed by a revision of the syndesmosis and transfixation using a tricortical position screw. Mobilization on day 1 after surgery with reduced weight-bearing when position screw is not applied; when position screw is implanted with ground contact for 6 weeks. Removal of position screw under local anesthesia after 6 weeks and pain-controlled full weight-bearing. Removal of metal after 1.5 years. Open reduction using the third tubular plate and an interfragmentary lag screw through a dorsolateral approach used in 90 % of all Weber B fractures in our clinic. Additional revision of a ruptured syndesmosis performed in 70 % and transfixation through a position screw in 40 %. Persisting instability in the upper ankle joint significantly reduced after surgical treatment compared to a conservative approach. Revisions necessary in 3.7 % of patients and pseudarthrosis diagnosed in 0.9 %. It has been shown that the preoperative x-ray and clinical examination is limited in detecting a ruptured syndesmosis.

  7. Dermatological diseases in patients with chronic kidney disease.

    Science.gov (United States)

    Gagnon1, Amy L; Desai, Tejas

    2013-04-01

    There are a variety of dermatological diseases that are more commonly seen in patients with chronic kidney disease (CKD) and renal transplants than the general population. Directory of Open Access Journals (DOAJ), Google Scholar, Pubmed (NLM), LISTA (EBSCO) and Web of Science has been searched. Some cutaneous diseases are clearly unique to this population. Of them, Lindsay's Nails, xerosis cutis, dryness of the skin, nephrogenic systemic fibrosis and acquired perforating dermatosis have been described in chronic kidney disease patients. The most common malignancy found in all transplant recipients is non-melanoma skin cancer. It is important for patients and physicians to recognize the manifestations of skin disease in patients suffering from chronic kidney disease to mitigate the morbidity associated with these conditions.

  8. Empreendedorismo religioso: quando Weber se associa a Granovetter

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    Victor Silva Corrêa

    2016-12-01

    Full Text Available O fenômeno religioso no Brasil vem atraindo crescente atenção da academia, impulsionado, em grande parte, pela expansão das seitas protestantes no país, sobretudo de vertente pentecostal. Na esteira dessa transformação, um tema, em particular, reveste-se de especial importância. Trata-se do papel dos chamados empreendedores religiosos aqui compreendidos como os novos pastores evangélicos. No entanto, tais atores não vêm sendo muito estudados. O presente trabalho busca preencher essa lacuna. Recorre-se, para isso, às contribuições teóricas clássicas derivadas de Weber, em associação às contribuições contemporâneas originárias de Granovetter, em particular, sobre acoplamento e desacoplamento. Conclui-se o trabalho propondo um novo referencial para a análise de certas dimensões do empreendedorismo religioso.

  9. Noniterative Solution of Some Fermat-Weber Location Problems

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    Reuven Chen

    2011-01-01

    Full Text Available The Fermat-Weber problem of optimally locating a service facility in the Euclidean continuous two-dimensional space is usually solved by the iterative process first suggested by Weiszfeld or by later versions thereof. The methods are usually rather efficient, but exceptional problems are described in the literature in which the iterative solution is exceedingly long. These problems are such that the solution either coincides with one of the demand points or nearly coincides with it. We describe a noniterative direct alternative, based on the insight that the gradient components of the individual demand points can be considered as pooling forces with respect to the solution point. It is demonstrated that symmetrical problems can thus be optimally solved with no iterations, in analogy to finding the equilibrium point in statics. These include a well-known ill-conditioned problem and its variants, which can now be easily solved to optimality using geometrical considerations.

  10. AZÚCARES EN AGAVES (Agave tequilana Weber CULTIVADOS EN EL ESTADO DE GUANAJUATO Azúcares en agaves (Agave tequilana Weber cultivados en el Estado de Guanajuato

    Directory of Open Access Journals (Sweden)

    L. A. Parra-Negrete

    2012-02-01

    Full Text Available The objetive of this work was to determine if the quality of Guanajuato agaves is equal to the agaves cultivated in Jalisco. A comparative study was made of the sugar content of the agave (A. tequilana Weber cultivated in both states. Used were data of total reducing sugars (%TRS and Brix degrees (ºBx of raw juice samples provided by a tequilera of the region between August and December, 1998 and January and May, 2000. The data were statistically processed, applying an ANOVA analysis and a Tukey test to compare the media and the correlation between °Bx and %TRS was made. In the first period, the media %TRS from the supplier of Manuel Doblado, Gto. was 24.43 ±2.29 and did not show a significant statistical difference (p El objetivo de este trabajo fue determinar si la calidad de los agaves guanajuatenses es igual a la de los cultivados en Jalisco. Se realizó un estudio comparativo del contenido de azúcares de los agaves (A. tequilana Weber cultivados en ambos estados. Se emplearon datos de azúcares reductores totales (%ART agrupados en dos períodos y grados Brix (°Bx de muestras de jugo crudo, proporcionados por una tequilera de la región: agosto a diciembre de 1998 y de enero a mayo del 2000. Los datos se procesaron estadísticamente aplicando un análisis de varianza y prueba de Tukey para comparación de medias. Se determinó la correlación entre los °Bx y el %ART. En el primer período, la media del %ART para el proveedor de Manuel Doblado, Gto. fue de 24.43 ±2.29 y no presentó diferencia estadística significativa (p

  11. BUROCRACIA E CRISE DE LEGITIMIDADE: A PROFECIA DE MAX WEBER

    OpenAIRE

    Branco, Pedro H. Villas Bôas Castelo

    2016-01-01

    Resumo A sociologia da dominação e a do direito de Max Weber representam uma mudança de paradigma no campo da teoria política. Ao deslocarem o eixo de análise dos meios de aquisição e instituição do poder dos dominadores para a crença dos dominados, operam uma notável inversão no modo de compreensão da legitimidade do poder político. A exposição da tensão entre a expansão burocrática e a estrutura da dominação democrática conferiram à reflexão weberiana um tom profético, cuja repercussão se f...

  12. Sturge Weber Syndrome: review of literature with case illustration

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    Satyarthee Guru Dutta

    2017-03-01

    Full Text Available Sturge-Weber syndrome (SWS also called as encephalotrigeminal angiomatosis, is a sporadically occurring rare neuro-cutaneous syndrome, characterized by vascular malformation with capillary venous angiomas involving face, choroidal layer of eye globe and leptomeninges responsible for ophthamological as well as neurological signs and symptoms. Authors report an interesting case, a six year old girl, who presented with seizures, facial port wine stain and normal psychomotor development. CT scan showed left cerebral hemiatrophy, left frontal and parieto occipital calcification with cortical calcification in left high frontal convexity. Cranial MRI scan also confirmed finding of left cerebral hemiatrophy and also revealed presence of gyriform cortical calcification, prominent flow voids seen in left basal ganglia. Her seizure is well controlled with antiepileptic medication. The pertinent literature is reviewed and management of such cases is discussed briefly.

  13. Cerebrofacial venous metameric syndrome (CVMS) 3: Sturge-Weber syndrome with bilateral lymphatic/venous malformations of the mandible

    International Nuclear Information System (INIS)

    Ramli, N.; Sachet, M.; Bao, C.; Lasjaunias, P.

    2003-01-01

    We present a case of Sturge-Weber syndrome with a bilateral lymphatic/venous malformation of the mandible. Modern biology suggests an explanation for such a case. The classification of cerebrofacial venous metameric syndromes (CVMS) enables us to recognise this lesion as involving the most caudal of the cranial metamere (CVMS 3). (orig.)

  14. MARCUSE CRÍTICO DE WEBER. A política no capitalismo tardio

    OpenAIRE

    Vasconcellos, Caio Eduardo Teixeira

    2018-01-01

    O objetivo deste artigo é analisar aspectos da leitura de Herbert Marcuse da sociologia de Max Weber, privilegiando suas considerações sobre transformações na esfera política desencadeadas com o fim do período liberal do capitalismo. A partir de uma crítica imanente do pensamento weberiano, Marcuse correlaciona sua interpretação da imbricação entre a forma de dominação racional-burocrática e as lideranças de tipo carismático aos pressupostos materiais das sociedades modernas em seu decurso ta...

  15. [The Concept of Typology in Psychiatry in the Context of Historical Contributions of Max Weber and Karl Jaspers].

    Science.gov (United States)

    Jäger, M; Becker, T; Wigand, M E

    2016-08-01

    Against the background of Max Weber's and Karl Jaspers' outstanding historical contributions to the conceptual development of different typologies, the importance of a psychiatric typology is examined. The term "ideal type" was introduced into social science by Weber as an analytical construct to describe and classify cultural phenomena. This concept was adopted for the psychiatric context by Jaspers who proposed to establish a typological system in the field of psychotic disturbances without an organic correlate. He emphasized the importance of the course of psychopathological symptoms for such a typological system. The concept of typology can be regarded as a promising heuristic approach in psychiatry, providing a classification system for complex psychopathological symptoms. Even though several historic typologies exist in psychopathology, their usefulness in the fields of therapy and prognosis needs to be critically assessed. Also, new typologies will have to be developed, taking into account neurobiological knowledge now available. © Georg Thieme Verlag KG Stuttgart · New York.

  16. A girl of Klippel-Trenaunay Weber syndrome coexistence of recurrent bloody vaginal discharge.

    Science.gov (United States)

    Turkmen, Mehmet; Kavukçu, Salih; Çakmakci, Handan; Soylu, Alper; Aktan, Sebnem; Çağan, Yeliz

    2010-09-01

    Klippel-Trenaunay Weber syndrome (KTWS) is the coexistence of capillary vascular malformations, varicose veins, dilated arteries and arteriovenous fistulas, soft tissue and/or bone hypertrophy. We present a girl of KTWS associated with hypertrophied left kidney, enlargement in venous structures of the left kidney, recurrent bloody vaginal discharge and angiokeratomas. A 6-year-old girl was admitted to our department with complaints of recurrent bloody vaginal discharge and swelling in the left inguinal region. Physical examination revealed hypertrophy of the left lower extremity. Vaginoscopy and cystoscopy revealed normal findings. Abdominal ultrasound revealed an enlarged left kidney. Enlargement in the venous structures of the hypertrophied left kidney was detected by abdominal magnetic resonance imaging, and arteriovenous fistulas were revealed by conventional angiography. The patient was diagnosed KTWS. Ophthalmic examination was normal. Galactosidase A (GLA) level was found to be at the lower limit of the normal range, and mutation was not detected in the GLA gene. In conclusion, we have emphasized that the girls with recurrent vaginal discharge might be KTWS. Angiokeratoma may be considered as a dermatological finding of KTWS. KTWS may also have enlarged kidney and enlargement in venous structures of the kidney in hypertrophied side.

  17. Aportaciones de la mitología nórdica para comprender la modernidad: revisión de Max Weber

    Directory of Open Access Journals (Sweden)

    Maximiliano Emanuel Korstanje

    2012-06-01

    Full Text Available La antropología nos ha enseñado que se pueden alcanzar interesantes conclusiones cuando se llevan a cabo estudios en forma interdisciplinar y cuando se observa las diferencias de los “otros” desde una posición “de nativo”. Además, la diferencia de estos otros puede ser contrastada con las “nuestras” y de esa forma conocer las contradicciones y estructuraciones de la cultura en forma introspectiva. En concordancia con ello y luego del desarrollo expuesto, consideramos oportuno señalar que Max Weber ha omitido, en parte por razones que desconocemos el rol de la mitología germánica antigua en la configuración del Ethos Protestante. Misteriosamente o mejor dicho por la propia influencia familiar, el profesor de Friburgo giró su cabeza hacia el mundo de la piedad, la humildad y la predestinación insertas en la reforma. Si bien nuestro sociólogo no equivocó la identificación de los elementos que han conformado al capitalismo, evitó claramente formular mayores consideraciones sobre el papel de la mitología germánica de la antigüedad clásica en el pensamiento indo-europeo. En ese contexto, nuestro artículo pretende ser una contribución que sea desarrollada en futuros abordajes por otros autores.Palabras Claves: Weber; Capitalismo; Secularización; Modernidad; Mitología Germánica._________________________AbstractAnthropology has taught us that interesting conclusions can be reached when carrying out interdisciplinary studies. Also, when looking at the differences in the "other" from a position "native". Furthermore, unlike these others may be contrasted with "our". In this connection, it should be noted that Max Weber has omitted the role of the ancient germanic mythology in shaping the protestant ethos. By family influence, Professor of Freiburg turned his head toward the world of piety, and predestination own reform. Although no mistake in identifying the elements that have made capitalism, avoided making further

  18. disease patient

    Directory of Open Access Journals (Sweden)

    Setareh Mamishi

    2016-09-01

    Full Text Available Background and Purpose: Chronic granulomatous disease (CGD is an inherited disorder of the nicotinamide adenine dinucleotide phosphate (NADPH oxidase complex. This disorder results in recurrent life-threatening bacterial and fungal infections. Aspergillus species are the most common fungal infections in these patients. Case Report: Herein, we present a case of fungal infection in a girl with CGD. We confirmed aspergillosis through the positive microscopic and macroscopic examinations, as well as radiology results. Invasive aspergillosis in this patient with pneumonia, lung abscess, and osteomyelitis of the ribs was not initially treated with amphotericin B (Am B and recombinant interferon-gamma. Conclusion: Among infectious diseases, fungal infections, in particular aspergillosis, remain a serious problem in CGD patients. Considering poor clinical response and deficient immune system, rapid diagnosis of fungal infection and optimizing the treatment of these patients are recommended.

  19. Sturge-Weber syndrome: Continued vigilance is needed.

    Science.gov (United States)

    Hassan, Saeed; Babiker, Amir; Bashiri, Fahad A; Hassan, Hamdi H; Husseini, Maha El; Salih, Mustafa A

    2015-01-01

    Sturge-Weber syndrome (SWS) is a non-hereditary congenital disorder due to somatic mosaic mutations in the GNAQ gene. The classical presentation relates to the brain lesion (cerebral angiomatous lesion of leptomeninges, which is responsible for epileptic seizures, hemiparesis and mental retardation), skin lesion (unilateral facial nevus), ocular and oral involvement. We present a 12-year-old boy who was referred to the Division of Pediatric Neurology, King Saud University Medical City, Riyadh, Saudi Arabia with left-sided hemiparesis. Physical examination showed a port wine stain involving the right side of the face, extending to the upper thorax, and enlargement of both the right eye globe and cornea (megalocornea), indicating the presence of glaucoma. Following urgent referral to ophthalmology service, his eye condition improved dramatically post surgery. Neuroradiological investigations, including cranial computed tomography (CT) and magnetic resonance angiography (MRI) revealed the classical brain lesions of SWS, as well as right leptomeningeal choroidal angioma. Ten months later, he developed focal-onset seizures which responded to treatment. His cognition is normal with good school performance. Continued vigilance is needed to identify and manage the complications of SWS.

  20. Max Weber or Jesus Christ: in whose image?

    Science.gov (United States)

    Fitzpatrick, A M; Gaylor, C C

    1985-10-01

    The bureaucratic model of organization and the model that emerges from the Gospel and Vatican Council II provide a stark contrast for Catholic health care administrators to ponder. As articulated by sociologist Max Weber, a bureaucracy is based on a hierarchical structure with sharply defined and protected levels of authority, centralized decision making, rules that govern personnel behavior, and an impersonalized view of employees that stresses their roles as functionaries. The Christian, Gospel-based model, on the other hand, places a premium on collegiality, shared authority, the mediation of conscience, the common good, and the work of the individual. Even though only a few will shape management policy in a large hospital, policymaking should be viewed as a service, a ministry that entails stewardship. Catholic health care leaders should reflect the spirit of collegiality by bringing various strata of staff together in a dynamic, united effort. Thus the institution's statement of mission will not be an abstract recitation of pieties but a living reality, a philosophy that is "owned" by the workers.

  1. Epistaxis grading in Osler's disease: comparison of comprehensive scores with detailed bleeding diaries.

    Science.gov (United States)

    Parzefall, Thomas; Wolf, Axel; Frei, Klemens; Kaider, Alexandra; Riss, Dominik

    2017-03-01

    Use of reliable grading scores to measure epistaxis severity in hereditary hemorrhagic telangiectasia (HHT) is essential in clinical routine and for scientific purposes. For practical reasons, visual analog scale (VAS) scoring and the Epistaxis Severity Score (ESS) are widely used. VAS scores are purely subjective, and a potential shortcoming of the ESS is that it is based on self-reported anamnestic bleeding data. The aim of this study was to validate the level of correlation between VAS scores, the ESS, and actual bleeding events, based on detailed epistaxis diaries of patients. Records from daily epistaxis diaries maintained by 16 HHT patients over 112 consecutive days were compared with the monthly ESS and daily VAS scores in the corresponding time period. The Spearman rank correlation coefficient, analysis of variance models, and multiple R 2 measures were used for statistical analysis. Although the ESS and VAS scores generally showed a high degree of correlation with actual bleeding events, mild events were underrepresented in both scores. Our results highlight the usefulness of the ESS as a standard epistaxis score in cohorts with moderate to severe degrees of epistaxis. The use of detailed epistaxis diaries should be considered when monitoring patients and cohorts with mild forms of HHT. © 2016 ARS-AAOA, LLC.

  2. Clinical application of MR susceptibility weighted imaging in cerebrovascular diseases

    International Nuclear Information System (INIS)

    Zhu Wenzhen; Qi Jianpin; Shen Hao; Wang Chengyuan; Xia Liming; Hu Junwu; Feng Dingyi

    2007-01-01

    Objective: To assess clinical application value of susceptibility weighted imaging (SWI) in cerebrovascular diseases. Method: Twenty-three patients with cerebrovascular disease were investigated, including 7 cases of cavernoma, 4 of venous hemangioma, 3 of small AVM, 1 of Sturge-Weber Syndrome, 2 of cerebral venous sinus thrombosis and 6 of chronic cerebral infarction. All patients underwent standard Mill and SWI, and most of them also underwent enhanced T 1 WI and MRA. The corrected phase (CP) values were obtained at the lesions and control areas. Results: The average CP values of the lesions and the control areas were -0.112±0.032 and -0.013±0.004, respectively (t=2.167, P 2 WI. The cavemoma could be differentiated from the hemorrhage within lesions. Moreover, multiple microcavernomas were detected on SWI. In 4 cases of venous hemangioma, SWI detected spider-like lesions with more hair-thin pulp veins adjacent to the dilated draining vein than contrast MRI. In 3 cases of small AVM, SWI was more advantageous than MRA in clearly detecting the small feeding artery. In 1 case of Sturge-Weber Syndrome, SWI demonstrated large areas of calcification and the abnormal vessels on the cerebral surface and the deep part of the cerebrum at the same time. In 2 cases of cerebral venous sinus thrombosis, the deep draining veins and superficial venous rete were generally dilated and winding, and the hemorrhagic lesions could be detected earlier than conventional MR images in one case. In 6 eases of cerebral infarction, old hemorrhage was clearly displayed within the lesions. Conclusion: SWI has more predominant advantages than conventional MRI and MRA in detecting the low-flow cerebral vascular malformations, identifying microbleeds and cerebral infarction accompanying hemorrhage, and the dilation of cerebral deep or superficial veins in patients with cerebral venous sinus thrombosis. Moreover, SWI can show the phase contrast between the lesions and the control areas. (authors)

  3. Bedside teaching with unwell patients: Can it ever be appropriate?

    Science.gov (United States)

    Jayakumar, Nithish

    2017-03-01

    Bedside teaching has seen a decline in its popularity since the heady days of Boerhaave and Osler; a number of hurdles have been noted in the literature including time constraints on senior clinicians. While organizing a bedside teaching course for final-year medical students, it was apparent that the availability of suitable patients was also a limiting factor. Often, we would be turned away from patients with physical signs because they were deemed to be too unwell for an examination. However, the benefits to medical students of examining unwell patients with physical signs cannot be underestimated. Identifying the unwell patient, and the associated physical signs relevant to the underlying diagnosis, should be a priority; although simulation is increasingly being used in teaching, it cannot fully replicate the learning experience gained in bedside teaching. As long as the patient is not under duress, bedside teaching with unwell patients should be encouraged.

  4. Pathophysiology, diagnosis, and management of glaucoma associated with Sturge-Weber syndrome.

    Science.gov (United States)

    Javaid, Usman; Ali, Muhammad Hassaan; Jamal, Samreen; Butt, Nadeem Hafeez

    2018-02-01

    Sturge-Weber syndrome (SWS), also known as encephalotrigeminal angiomatosis, is a condition which includes leptomeningeal hemangioma, facial angiomatosis or nevus flammeus, and ocular changes. SWS can lead to severe complications of anterior segment involving conjunctiva and eyelids, whereas posterior segment of the eye may also be affected by diffuse choroidal hemorrhages. This article was written with the objectives to determine the pathophysiology, diagnosis, and treatment of glaucoma associated with this rare and challenging disorder. A detailed literature search was conducted on PubMed, EMBASE, Cochrane Library, and Google Scholar using the key words. Forty-five articles matched our inclusion criteria that were included in this systematic review. Glaucoma is the one of the commonest ocular manifestations of SWS. It is caused by anterior chamber malformations, increased pressure in the episcleral veins, and changes in ocular hemodynamics. Glaucoma associated with SWS is usually congenital but can develop adults as well. The treatment of glaucoma associated with SWS is quite challenging because of early-onset, severe visual field impairment at the time of diagnosis, and unresponsiveness to standard medical treatment. Several surgical procedures have been devised but the long-term control of the intraocular pressure and visual function remain unsatisfactory. Modifications in the filtration surgery techniques and use of newer anti-fibrotic agents have produced good control of intraocular pressure. Management of glaucoma associated with SWS is multi-dimensional and needs both medical and surgical interventions for better control. The treatment should be devised on case to case basis depending upon the intraocular pressure, stage of the disease, and type of glaucoma.

  5. Prevalence of coeliac disease in Italian patients affected by Addison's disease.

    Science.gov (United States)

    Biagi, Federico; Campanella, Jonia; Soriani, Alessandra; Vailati, Alberto; Corazza, Gino R

    2006-03-01

    It is well known that coeliac disease is associated with autoimmune endocrine diseases, such as autoimmune thyroid disease and insulin-dependent diabetes mellitus. Recently, coeliac disease has been shown in approximately 10% of patients with autoimmune Addison's disease. Addison's disease is the most common cause of primary adrenocortical insufficiency and it shares several clinical features with coeliac disease. Although hyperpigmentation and hypotension are the most specific signs, gastrointestinal symptoms are common and can be the first complaints of the patients. The aim of our study was to investigate the prevalence of coeliac disease in Italian patients with Addison's disease. Seventeen consecutive patients affected by Addison's disease (14 F, mean age 53.9 years, range 26-79 years) were enrolled in the study. Eleven of them were affected by Addison's disease associated with autoimmune thyroid disease and/or insulin-dependent diabetes mellitus; the other 6 patients were suffering from isolated Addison's disease. Diagnosis had been performed at the age of 40.5 years (range 23-55). Steroid treatment had already been started in 16 of the patients. Endomysial antibodies were tested in all of them and a duodenal biopsy was taken in those found to be positive for antiendomysial antibody (EMA). One out of 17 patients was found to be EMA positive. Duodenal biopsy confirmed the diagnosis of coeliac disease by showing subtotal villous atrophy. Although we studied only a small sample, our preliminary results confirmed that Addison's disease is associated with coeliac disease, being present in 5.9% of patients with Addison's disease. Since the symptoms can be similar and treatment of Addison's disease can mask coeliac disease, this association should always be actively investigated.

  6. Generation of Maillard compounds from inulin during the thermal processing of Agave tequilana Weber Var. azul.

    Science.gov (United States)

    Mancilla-Margalli, Norma A; López, Mercedes G

    2002-02-13

    During the cooking process of Agave tequilana Weber var. azul to produce tequila, besides the hydrolysis of inulin to generate fermentable sugars, many volatiles, mainly Maillard compounds, are produced, most of which may have a significant impact on the overall flavor of tequila. Exudates (agave juice) from a tequila company were collected periodically, and color, Brix, fructose concentration, and reducing sugars were determined as inulin breakdown took place. Maillard compounds were obtained by extraction with CH(2)Cl(2), and the extracts were analyzed by GC-MS. Increments in color, Brix, and reducing sugars were observed as a function of time, but a decrease in fructose concentration was found. Many Maillard compounds were identified in the exudates, including furans, pyrans, aldehydes, and nitrogen and sulfur compounds. The most abundant Maillard compounds were methyl-2-furoate, 2,3-dihydroxy-3,5-dihydro-6-methyl-4(H)-pyran-4-one, and 5-(hydroxymethyl)furfural. In addition, a series of short- and long-chain fatty acids was also found. A large number of the volatiles in A. tequilana Weber var. azul were also detected in tequila extracts, and most of these have been reported as a powerful odorants, responsible for the unique tequila flavor.

  7. Coronary artery disease in patients with cerebrovascular disease: a prospective study

    International Nuclear Information System (INIS)

    Rokey, R.; Rolak, L.A.; Harati, Y.; Kutka, N.; Verani, M.S.

    1984-01-01

    Coronary artery disease is the cause of death in most patients who have transient ischemic attacks or stroke. Evaluation for this condition is not routinely performed in such patients, and no prospective studies have been reported. We prospectively examined 50 consecutive patients with transient ischemic attacks or mild stroke to determine the prevalence and importance of coronary artery disease. All patients were examined by a cardiologist and underwent both exercise thallium-201 scintigraphy and exercise radionuclide ventriculography. Sixteen patients were suspected to have coronary artery disease on the basis of clinical evaluation. In 15 of these the was confirmed by the nuclear scans. The remaining 34 patients had no clinical evidence of heart disease, yet 14 had abnormal cardiac scans. Twenty of 22 patients with abnormal scans who underwent cardiac catheterization had significant coronary artery disease or a cardiomyopathy. The discovery of heart disease altered clinical management in 13 patients. Overall, 29 of 50 patients had significant coronary artery disease, compared with a 7% prevalence of the condition in other patients of similar age at the same institution

  8. The Power of the Stage and the Dignity of the Academic Calling in Imperial Germany: The Writings of Max Weber on University Problems

    Science.gov (United States)

    Minerva, 1973

    1973-01-01

    Contains Max Weber's writings on the problems of the German university in the face of political and bureaucratic authority and on the fundamental principles of university autonomy and academic freedom. (PG)

  9. The Intermediate Set and Limiting Superdi erential for Coalition Games: Between the Core and the Weber Set

    Czech Academy of Sciences Publication Activity Database

    Adam, Lukáš; Kroupa, T.

    2017-01-01

    Roč. 46, č. 4 (2017), s. 891-918 ISSN 0020-7276 R&D Projects: GA ČR GA15-00735S Institutional support: RVO:67985556 Keywords : coalition game * limiting superdi erential * intermediate set * core * Weber set Subject RIV: BA - General Mathematics OBOR OECD: Statistics and probability Impact factor: 0.713, year: 2016 http://library.utia.cas.cz/separaty/2016/MTR/adam-0467365.pdf

  10. ROENTGEN-OPTHALMOLOGICAL ASPECTS OF SOME EYE DISEASES

    Directory of Open Access Journals (Sweden)

    Gordana Stanković-Babić

    2002-03-01

    Full Text Available The paper presents the roentgen-ophtalmological image, the diagnostic procedures and the differential diagnostics of the eye diseases that can be recognized by the proper roentgen examination of the cranium, the orbit, the saddle pit, the craniostenosis, the M. Sturge-Weber-Krabbe, the congenital hydrocephalus, the eye melanoma malign, the chiasmal syndrome, the retrobulbar tumors, tie primary meningeom n. opticus, the face tumors.

  11. [Inpatients days in patients with respiratory diseases and periodontal disease].

    Science.gov (United States)

    Fernández-Plata, Rosario; Olmedo-Torres, Daniel; Martínez-Briseño, David; González-Cruz, Herminia; Casa-Medina, Guillermo; García-Sancho, Cecilia

    2017-01-01

    Periodontal disease is a chronic inflammatory gingival process that has been associated with the severity of respiratory diseases. In Mexico a prevalence of 78% was found in population with social security and > 60 years old. The aim of this study is to establish the association between periodontal disease and respiratory diseases according to the inpatient days. A cross-sectional study was conducted from January to December 2011. We included hospitalized patients, ≥ 18 years of age, without sedation or intubated. A dentist classified patients into two groups according to the severity of the periodontal disease: mild-to-moderate and severe. We estimated medians of inpatient days by disease and severity. Negative binomial models were adjusted to estimate incidence rate ratios and predicted inpatient days. 3,059 patients were enrolled. The median of observed and predicted inpatient days was higher in the group of severe periodontal disease (p disease, tuberculosis, and influenza had the highest incidence rates ratios of periodontal disease (p periodontal disease is positively -associated with inpatient days of patients with respiratory diseases.

  12. Celiac Disease in Patients with Cystic Fibrosis-Related Bone Disease

    Directory of Open Access Journals (Sweden)

    Melissa S. Putman

    2017-01-01

    Full Text Available Both cystic fibrosis (CF and celiac disease can cause low bone mineral density (BMD and fractures. Celiac disease may occur at a higher frequency in patients with CF than the general population, and symptoms of these conditions may overlap. We report on two patients presenting with CF-related bone disease in the past year who were subsequently found to have concurrent celiac disease. Because adherence to a gluten-free diet may improve BMD in patients with celiac disease, this could have important implications for treatment. Clinicians should consider screening for celiac disease in patients with CF who have low BMD, worsening BMD in the absence of other risk factors, and/or difficult to treat vitamin D deficiency.

  13. EL Agave tequilana Weber Y LA PRODUCCIÓN DE TEQUILA EL Agave tequilana Weber Y LA PRODUCCIÓN DE TEQUILA

    Directory of Open Access Journals (Sweden)

    J. E. Barboza Corona

    2012-02-01

    Full Text Available Tequila is one of the most famous drinks on a national and worldwide level. It is obtained from the Agave tequilana Weber plant, better known as Agave Azul. The part of the plant used for making tequila is the core. Once the cores are cut, they are taken away to an oven where a cooking operation is made, this with the object of degrading the complex sugars of the core into simple sugars that can be used as yeasts in the fermentation process. The principal complex carbohydrate present in agave is inulin, which is a polymeric fructose with a terminal glucose residue. When the inulin is degraded by action of the juice´s acidity and the heat of cooking, fructose and glucose are obtained. Next, the cores are milled to extract the juice which is placed in vats and inoculated with a yeast culture of Saccharomyces cerevisiae, responsible for the fermentation of the sugars present in the juice. After fermentation, the juice is distilled to obtain ethanol and vinazes (a mixture of dead yeasts, nonfermentable sugars, and minerals. Tequila can be bottled immediately after distillation or it can be aged in the wooden casks wich confer characteristic flavor and color to tequila.El tequila es una de las bebidas más famosas a nivel nacional y mundial. Se obtiene de la planta Agave tequilana Weber, mejor conocido como Agave azul. La parte de la planta que es utilizada para la elaboración del tequila es la piña. Una vez que las piñas son cortadas, se llevan a un horno en donde se realiza la operación del cocimiento; éste tiene la finalidad de degradar los azúcares complejos presentes en la piña en azúcares simples para que puedan ser utilizados por la levadura en la operación de fermentación. El principal carbohidrato complejo presente en el agave es la inulina, el cual es un polímero de fructosa con un residuo terminal de glucosa. Cuando la inulina se degrada por la acción de la acidez del jugo y del calor en el cocimiento, se obtienen

  14. Celiac disease and other autoimmune diseases in patients with collagenous colitis.

    Science.gov (United States)

    Vigren, Lina; Tysk, Curt; Ström, Magnus; Kilander, Anders F; Hjortswang, Henrik; Bohr, Johan; Benoni, Cecilia; Larson, Lasse; Sjöberg, Klas

    2013-08-01

    Collagenous colitis (CC) is associated with autoimmune disorders. The aim of the present study was to investigate the relationship between CC and autoimmune disorders in a Swedish multicenter study. Patients with CC answered questionnaires about demographic data and disease activity. The patient's files were scrutinized for information about autoimmune diseases. A total number of 116 CC patients were included; 92 women, 24 men, median age 62 years (IQR 55-73). In total, 30.2% had one or more autoimmune disorder. Most common were celiac disease (CeD; 12.9%) and autoimmune thyroid disease (ATD, 10.3%), but they also had Sjögren's syndrome (3.4%), diabetes mellitus (1.7%) and conditions in skin and joints (6.0%). Patients with associated autoimmune disease had more often nocturnal stools. The majority of the patients with associated CeD or ATD got these diagnoses before the colitis diagnosis. Autoimmune disorders occurred in one-third of these patients, especially CeD. In classic inflammatory bowel disease (IBD), liver disease is described in contrast to CC where no cases occurred. Instead, CeD was prevalent, a condition not reported in classic IBD. Patients with an associated autoimmune disease had more symptoms. Patients with CC and CeD had an earlier onset of their colitis. The majority of the patients with both CC and CeD were smokers. Associated autoimmune disease should be contemplated in the follow-up of these patients.

  15. El cambio social y la 'Constitución moderna' en los clásicos de la Sociología. Durkheim y Weber en perspectiva comparada

    Directory of Open Access Journals (Sweden)

    Santiago, José

    2011-08-01

    Full Text Available The aim of this article is to analyse the conception of social change of Durkheim and Weber from a comparative perspective. It proposes a reading of their works attending to the place these authors occupy in what, following Bruno Latour, may be named as “Modern Constitution”. From this metatheoretical level, the comparison brings to light the tools and resources of the “Modern Critique” which they utilise to build their theories of social change. From this comparative perspective, the article examines how Durkheim and Weber theorize on the “fourth guarantee” of the “Modern Constitution”, that refers to God’s presence in modernity. Finally, this “guarantee” is problematized in the frame of the contemporary society attending to current theories.

    El objetivo de este artículo es analizar la concepción del cambio social de Durkheim y Weber desde una perspectiva comparada. Se propone una lectura de sus obras prestando atención al lugar que ambos ocupan en lo que, siguiendo a Bruno Latour, llamaremos “Constitución moderna”. Desde este nivel metateórico, la comparación permitirá mostrar las herramientas y recursos de la “Crítica moderna” de los que estos autores se sirven para construir sus teorías del cambio social. Con esta perspectiva comparada, el artículo examina de qué manera Durkheim y Weber teorizan sobre la “cuarta garantía” de la “Constitución moderna”, que hace referencia al lugar que ocupa Dios en la modernidad. Por último, esta “garantía” es problematizada en el marco de la sociedad contemporánea a partir de las propuestas de algunos teóricos actuales.

  16. Role of Ankle Arthroscopy in Management of Acute Ankle Fracture.

    Science.gov (United States)

    Chan, Kwok Bill; Lui, Tun Hing

    2016-11-01

    To report the operative findings of ankle arthroscopy during open reduction and internal fixation of acute ankle fractures. This was a retrospective review of 254 consecutive patients with acute ankle fractures who were treated with open reduction and internal fixation of the fractures, and ankle arthroscopy was performed at the same time. The accuracy of fracture reduction, the presence of syndesmosis disruption and its reduction, and the presence of ligamentous injuries and osteochondral lesions were documented. Second-look ankle arthroscopy was performed during syndesmosis screw removal 6 weeks after the key operation. There were 6 patients with Weber A, 177 patients with Weber B, 51 patients with Weber C, and 20 patients with isolated medial malleolar fractures. Syndesmosis disruption was present in 0% of patients with Weber A fracture, 52% of patients with Weber B fracture, 92% of patients with Weber C fracture, and 20% of the patients with isolated medial malleolar fracture. Three patients with Weber B and one patient with Weber C fracture have occult syndesmosis instability after screw removal. Osteochondral lesion was present in no patient with Weber A fracture, 26% of the Weber B cases, 24% of the Weber C cases, and 20% of isolated medial malleolar fracture cases. The association between the presence of deep deltoid ligament tear and syndesmosis disruption (warranting syndesmosis screw fixation) in Weber B cases was statistically significant but not in Weber C cases. There was no statistically significant association between the presence of posterior malleolar fracture and syndesmosis instability that warrant screw fixation. Ankle arthroscopy is a useful adjuvant tool to understand the severity and complexity of acute ankle fracture. Direct arthroscopic visualization ensures detection and evaluation of intra-articular fractures, syndesmosis disruption, and associated osteochondral lesions and ligamentous injuries. Level IV, case series

  17. Peripheral vascular disease in patients with coronary artery disease

    International Nuclear Information System (INIS)

    Bashir, E. A.; Aslam, N.

    2001-01-01

    Objective: The prevalence of peripheral vascular disease (PVD) in patients with coronary artery disease (CAD) has been investigated in many different ways. It depends on the diagnostic methods used and definition of atherosclerotic manifestations in the different vascular beds. This study was carried out to determine the prevalence of PVD in the lower limbs in group of patients with CAD. Design: This is a prospective observational study. Place and duration of study: The study was conducted at Combined Military Hospital/Armed Forces institute of Cardiology, Rawalpindi, over a period of one year (January 1998 to January 1999). Subjects and methods: A total number of 200 patient (171 male and 29 females) aged 55-77 years with CAD. Diagnosed by coronary angiography were included in the study. In all patients blood pressure was recorded in both arms by sphygmomanometer and ankle systolic pressure by Doppler ultrasound. Ankle branchial index was calculated. Demographic data were obtained from the patient's hospital files. Results: The prevalence of PVD was 22.5% in patients with CAD in agreement with the results of most previous investigation. There was tendency towards increasing prevalence of PVD with more advanced CAD. Thirty patients (27%) showed evidence of triple vessel disease as compared to 13 patient (18%) with double vessel and 2 patients (1%) with single vessel disease. Conclusion: A non-invasive investigation of peripheral arterial circulation should be included early in the clinical consideration of patients with chest pain or similar symptoms suggesting coronary artery disease. Ankle systolic pressure appears to be simple and cheap technique for evaluation of results. (author)

  18. Hodgkin's disease: Analysis of 75 patients

    International Nuclear Information System (INIS)

    Akram, M.; Cheema, M. H.; Sana, S.; Aziz, Z.

    2001-01-01

    Objective: To evaluate patients suffering from Hodgkin's disease to identify its epidemiological characteristics, modalities of treatment and features of survival. Place and Duration of Study: The study was conducted between July 1997 to June 1999 at the Oncology Department of Jinnah Hospital, Lahore. Subjects and Methods: A total 75 patient with Hodgkin's disease underwent Cotswold staging classification, complete hematological, renal and hepatic profile, LDH and uric acid. Chest radiograph abdominal and pelvic ultrasonography and CT scan/MRI bone marrow biopsies. All the patients received combination chemotherapy. Patients with bulky disease received field radiation. Results: Median age was eight ( 18 years). Male to female ratio was 3.5:1, advanced disease accounted for 68%, mixed cellularity was documented in 61% of patients. Eighty-two percent of patients belonged to lower socioeconomic strata. Forty-six patients (65.4%) achieved complete remission (CR) Major toxicities were hematological with febrile neutropenia in 14.6% patients. OS of patients achieving CR with good socioeconomic status was superior compared to patients with lower socioeconomic status (p<0.02). Treatment delays were due to economic constraints, illiteracy, malnutrition and co-morbid conditions. Conclusion: Advanced disease, mixed cellularity and male predominance was common. Socioeconomic status had a significant impact on the presentation of the disease. Poor tolerance to chemotherapy and enhanced toxicities are especially seen in the low socioeconomic group. (author)

  19. [History of the tuning fork. II: Evolution of the classical experiments by Weber, Rinne and Schwabach].

    Science.gov (United States)

    Feldmann, H

    1997-05-01

    Since the 17th centrury it was known that sounds could be perceived via air conduction and bone conduction and that this provided a means of differentiating between hearing disorders located in the middle ear and those located in the acoustic nerve. For a long time to come, however, there was no need for such a differential diagnosis. After the invention of the tuning fork in 1711 this instrument had soon become widely used in music, but it took well over 100 years until it was introduced into physiology and otology. FROM DIRECTIONAL HEARING TO WEBER'S TEST: J. B. Venturi, a physicist in Modena, Italy, in 1802 had shown that the perception of the direction from which a sound is coming is governed by the fact that one ear is hit by the sound more intensely than the other ear. C. T. Tourtual, a physician in Münster, Germany, demonstrated in 1827 that this also holds true for sound conducted via the skull bones. He used a watch as sound source. He found that occlusion of both ear canals would increase the sensation in both ears equally, but that occlusion of only one ear would increase the sensation only in the occluded ear, thus giving the impression that the sound were coming from that side. He was interested in a comparison between vision and audition, and he concluded that with regard to recognizing the direction of a sensory signal vision was superior to audition. In the same year 1827 C. Wheatstone, a physicist in London, investigating the mode of vibration of the tympanic membrane and using a tuning fork found the same phenomena as Tourtual and some more effects. E. H. Weber, an anatomist and physiologist in Leipzig, Germany, described the very same phenomena as Tourtual and Wheatstone once more in 1834. He wanted to prove that airborne sound is perceived by the vestibulum and the semicircular canals, bone conducted sound by the cochlea. None of these investigators was thinking of a clinical use of their findings and made no such suggestion. E. Schmalz, an

  20. Prevalence and overlap of Disease Management Program diseases in older hospitalized patients

    DEFF Research Database (Denmark)

    Juul-Larsen, Helle Gybel; Petersen, Janne; Sivertsen, Ditte Maria

    2017-01-01

    Many countries, like Denmark, have tailored Disease Management Programs (DMPs) based on patients having single chronic diseases [defined institutionally as "program diseases" (PDs)], which can complicate treatment for those with multiple chronic diseases. The aims of this study were (a) to assess...... the prevalence and overlap among acutely hospitalized older medical patients of PDs defined by the DMPs, and (b) to examine transitions between different departments during hospitalization and mortality and readmission within two time intervals among patients with the different PDs. We conducted a registry study...... of 4649 acutely hospitalized medical patients ≥65 years admitted to Copenhagen University Hospital, Hvidovre, Denmark, in 2012, and divided patients into six PD groups (type 2 diabetes, chronic obstructive pulmonary disease, cardiovascular disease, musculoskeletal disease, dementia and cancer), each...

  1. Oscillation and non-oscillation criterion for Riemann–Weber type half-linear differential equations

    Directory of Open Access Journals (Sweden)

    Petr Hasil

    2016-08-01

    Full Text Available By the combination of the modified half-linear Prüfer method and the Riccati technique, we study oscillatory properties of half-linear differential equations. Taking into account the transformation theory of half-linear equations and using some known results, we show that the analysed equations in the Riemann–Weber form with perturbations in both terms are conditionally oscillatory. Within the process, we identify the critical oscillation values of their coefficients and, consequently, we decide when the considered equations are oscillatory and when they are non-oscillatory. As a direct corollary of our main result, we solve the so-called critical case for a certain type of half-linear non-perturbed equations.

  2. Durkheim e Weber: inspiração para uma nova sociabilidade, o neotribalismo

    Directory of Open Access Journals (Sweden)

    Sílvia Jurema Quaresma

    2005-01-01

    Full Text Available Este artigo tem como principal finalidade ampliar um pouco mais o conhecimento sobre o conceito de neotribalismo idealizado pelo sociólogo francês Michel Maffesoli. Este autor considera o neotribalismo como uma das características da pós-modernidade, porém estas características nos fazem retornar as idéias de Durkheim e Weber. Sendo assim, neste artigo pretendemos mostrar como Maffesoli introduz a nova concepção de sociabilidade das tribos, mas apoiada nos clássicos da Sociologia. Também expomos algumas idéias de autores como: Anthony Giddens, Boaventura de Souza Santos, Edgar Morin referentes ao paradigma da modernidade.

  3. Patterns of structural reorganization of the corticospinal tract in children with Sturge-Weber syndrome

    Science.gov (United States)

    Kamson, David O.; Juhász, Csaba; Shin, Joseph; Behen, Michael E.; Guy, William C.; Chugani, Harry T.; Jeong, Jeong-Won

    2014-01-01

    Background Reorganization of the corticospinal tract (CST) after early damage can limit motor deficit. In this study, we explored patterns of structural CST reorganization in children with Sturge-Weber syndrome. Methods Five children (age 1.5-7 years) with motor deficit due to unilateral Sturge-Weber syndrome were studied prospectively and longitudinally (1-2 years follow-up). CST segments belonging to hand and leg movements were separated, and their volume was measured by diffusion tensor imaging (DTI) tractography using a recently validated method. CST segmental volumes were normalized and compared between the SWS children and age-matched healthy controls. Volume changes during follow-up were also compared to clinical motor symptoms. Results In the SWS children, hand-related (but not leg-related) CST volumes were consistently decreased in the affected cerebral hemisphere at baseline. At follow-up, two distinct patterns of hand CST volume changes emerged: (i) Two children with extensive frontal lobe damage showed a CST volume decrease in the lesional hemisphere and a concomitant increase in the non-lesional (contralateral) hemisphere. These children developed good hand grasp but no fine motor skills. (ii) The three other children, with relative sparing of the frontal lobe, showed an interval increase of the normalized hand CST volume in the affected hemisphere; these children showed no gross motor deficit at follow-up. Conclusions DTI tractography can detect differential abnormalities in the hand CST segment both ipsi- and contralateral to the lesion. Interval increase in the CST hand segment suggests structural reorganization, whose pattern may determine clinical motor outcome and could guide strategies for early motor intervention. PMID:24507695

  4. Rheumatic Disease Autoantibodies in Patients with Autoimmune Thyroid Diseases.

    Science.gov (United States)

    Nisihara, Renato; Pigosso, Yasmine; Prado, Nathalia; Utiyama, Shirley R R; Carvalho, Gisah; Skare, Thelma

    2018-06-04

    Patients with autoimmune thyroid diseases (ATD) such as Graves' disease (GD) and Hashimoto thyroiditis (HT) may have non-organ specific autoantibodies such as ANA (antinuclear antibodies) and RF (rheumatoid factor). To study the prevalence of rheumatic autoantibodies in a group of ATD patients without known rheumatic diseases and to evaluate its association with the patients' epidemiological and treatment profile. To follow positive non-organ specific autoantibody-positive ATD individuals to investigate whether they will develop a rheumatic disorder. A sample of 154 ATD patients (70 HT and 84 GD; mean age 45.3 ± 14.2) had determination of ANA by immunofluorescence, using hep-2 cells as substrate, extractable nuclear antigen (ENA) profile by ELISA kits and RF by latex agglutination. Epidemiological and treatment profile were obtained through chart review. These patients were followed for the mean period of five years, between 2010 to 2015. Positive ANA was found in 17.5% (27/154) of the patients: anti-Ro/SS-A in 4/154 (2.5%); anti-RNP in 4/154 (2.5%) and anti-La/SS-B in 3/154 (1.9%). None had anti-Sm antibodies. RF was detected in 12/154 (7.7%) of ATD patients and was more common in older individuals (p = 0.007). There was a positive association between the presence of RF and ANA (p = 0.03; OR = 3.89; 95% CI = 1.1-13.3). None of the patients with positive autoantibodies developed clinical rheumatic diseases during the period of observation. We found rheumatic autoantibodies in 17.5% of ATD patients without rheumatic diseases. None of them were associated with the appearance of clinical rheumatic disorder during the period of five years. ©2018The Author(s). Published by S. Karger AG, Basel.

  5. Identification and characterization of microsporidia from fecal samples of HIV-positive patients from Lagos, Nigeria.

    Directory of Open Access Journals (Sweden)

    Oladele Teslim Ojuromi

    Full Text Available BACKGROUND: Microsporidia are obligate intracellular parasites that infect a broad range of vertebrates and invertebrates. They have been increasingly recognized as human pathogens in AIDS patients, mainly associated with a life-threatening chronic diarrhea and systemic disease. However, to date the global epidemiology of human microsporidiosis is poorly understood, and recent data suggest that the incidence of these pathogens is much higher than previously reported and may represent a neglected etiological agent of more common diseases indeed in immunocompetent individuals. To contribute to the knowledge of microsporidia molecular epidemiology in HIV-positive patients in Nigeria, the authors tested stool samples proceeding from patients with and without diarrhea. METHODOLOGY/PRINCIPAL FINDINGS: Stool samples from 193 HIV-positive patients with and without diarrhea (67 and 126 respectively from Lagos (Nigeria were investigated for the presence of microsporidia and Cryptosporidium using Weber's Chromotrope-based stain, Kinyoun stain, IFAT and PCR. The Weber stain showed 45 fecal samples (23.3% with characteristic microsporidia spores, and a significant association of microsporidia with diarrhea was observed (O.R. = 18.2; CI: 95%. A similar result was obtained using Kinyoun stain, showing 44 (31,8% positive samples with structures morphologically compatible with Cryptosporidium sp, 14 (31.8% of them with infection mixed with microsporidia. The characterization of microsporidia species by IFAT and PCR allowed identification of Enterocytozoon bieneusi, Encephalitozoon intestinalis and E. cuniculi in 5, 2 and 1 samples respectively. The partial sequencing of the ITS region of the rRNA genes showed that the three isolates of E.bieneusi studied are included in Group I, one of which bears the genotype B. CONCLUSIONS/SIGNIFICANCE: To our knowledge, this is the first report of microsporidia characterization in fecal samples from HIV-positive patients from

  6. Wireless Monitoring for Patients with Cardiovascular Diseases and Parkinson's Disease.

    Science.gov (United States)

    Kefaliakos, Antonios; Pliakos, Ioannis; Charalampidou, Martha; Diomidous, Marianna

    2016-01-01

    The use of applications for mobile devices and wireless sensors is common for the sector of telemedicine. Recently various studies and systems were developed in order to help patients suffering from severe diseases such as cardiovascular diseases and Parkinson's disease. They present a challenge for the sector because such systems demand the flow of accurate data in real time and the use of specialized sensors. In this review will be presented some very interesting applications developed for patients with cardiovascular diseases and Parkinson's disease.

  7. Adult Congenital Heart Disease Patients Experience Similar Symptoms of Disease Activity.

    Science.gov (United States)

    Cedars, Ari M; Stefanescu Schmidt, Ada; Broberg, Craig; Zaidi, Ali; Opotowsky, Alexander; Grewal, Jasmine; Kay, Joseph; Bhatt, Ami B; Novak, Eric; Spertus, John

    2016-03-01

    There is a lack of objective data on the symptoms characterizing disease activity among adults with congenital heart disease (ACHD). The purpose of this study was to elicit the most important symptoms from patients across the spectrum of ACHD and to examine whether reported symptoms were similar across the spectrum of ACHD as a foundation for creating a patient-reported outcome measure(s). We constructed a 39-item survey using input from physicians specializing in ACHD to assess the symptoms patients associate with disease activity. Patients (n=124) prospectively completed this survey, and the results were analyzed based on underlying anatomy and disease complexity. A confirmatory cohort of patients (n=40) was then recruited prospectively to confirm the validity of the initial data. When grouped based on underlying anatomy, significant differences in disease-related symptom rankings were found for only 6 of 39 symptoms. Six symptoms were identified which were of particular significance to patients, regardless of underlying anatomy. Patients with anatomy of great complexity experienced greater overall symptom severity than those with anatomy of low or moderate complexity, attributable exclusively to higher ranking of 5 symptoms. The second patient cohort had symptom experiences similar to those of the initial cohort, differing in only 5 of 39 symptoms. This study identified 6 symptoms relevant to patients across the spectrum of ACHD and remarkable homogeneity of patient experience, suggesting that a single disease-specific patient-reported outcome can be created for quality and outcome assessments. © 2016 American Heart Association, Inc.

  8. Historicismo, sujeto y moral (Max Weber y el «mito de la transparencia de la razón»

    Directory of Open Access Journals (Sweden)

    Valdecantos, Antonio

    1990-11-01

    Full Text Available Not available

    Recurso frecuente de lo que hoy reconstruimos como Ilustración fue desarbolar supersticiones y mitos. Es, sin embargo. lugar común redargüir que quien derriba ídolos no está libre de edificar otros nuevos (ni -tópico éste que cunde por doquier- de convertir su gesto iconoclasta en mito fundacional. No menos acostumbrado es apuntar a Max Weber como al primer desvelador de algún que otro episodio sobresaliente de la historia de la razón ilustrada, ya sea para abundar en su diagnóstico, ya para intentar salir de lo que se tiene por su «círculo hechizado ». En lo que sigue, se trata de rastrear algunas de las claves configuradoras de la crítica weberiana de la cultura procurando retrotraer a su desmantelamiento metodológico del ideal historicista, los términos en que elabora su problematízacíón del sujeto rnoderno. La inversión semántica de las categorías centrales de la epistemología y la Weltanschauung del historicísmo alemán decimonónico permite a Max Weber descubrir patologías del paradigma ilustrado allí donde se suele situar un momento de critica del mismo. Siguiendo los pasos de esta estrategia weberiana, nos es posible reconstruir la noción hípostasiada de racionalidad y de sujeto que Weber parece denunciar como tentación dogmática de la razón ilustrada. El programa weberiano constituye así el desenmascaramiento de un relato complaciente de lo moderno -al que llamo el «mito de la transparencia de la razóns-ey sobre ese supuesto cabe releer sus propuestas éticas más controvertidas, en particular la distinción entre «ética de convicción» y "ética de responsabílidad ».

  9. [Hypothyroidism in patients with heart disease].

    Science.gov (United States)

    Jiskra, Jan

    Hypothyroidism is frequently found in patients with heart disease. It is a risk factor for atherosclerosis and ischemic heart disease and has a direct negative effect on both the left and right ventricular functions (hypothyroidism-induced cardiomyopathy). The confirmed manifest hypothyroidism is always a reason for replacement therapy with levothyroxine; regarding patients with heart disease, we always begin treatment with a small dose and increase it gradually. The treatment of subclinical hypothyroidism in patients with heart disease is disputable and its benefits probably depend on age. At a higher age, the therapy-related risks often outweigh its benefits, so we make do with the target levels of the thyroid stimulating hormone being within the upper band of the normal range, or even slightly above it, rather than overdosing the patient. To summarize in a simplified way, the treatment of subclinical hypothyroidism in patients with heart disease is the most effective in younger individuals, mainly those aged below 65, while at a higher age > 80 years the risk usually outweighs the benefit.Key words: cardiovascular risk - hypothyroidism - ischemic heart disease - left ventricular dysfunction - right ventricular dysfunction - subclinical hypothyroidism - thyroid peroxidase antibodies.

  10. RELIGIÓN Y MODERNIDAD EN LOS SISTEMAS TEÓRICOS DE PARSONS Y WEBER

    Directory of Open Access Journals (Sweden)

    Carlos Acevedo

    2016-09-01

    Full Text Available En este artículo se presenta el sistema teórico parsoniano, el lugar de la religión en este y su papel en la modernidad. Debido a que Parsons afirma basarse en Weber en gran parte de esta propuesta,desarrollamos elsistema teórico weberianoy lo ponemos en diálogo con la perspectiva parsoniana. Sobre esta base,se ahonda enlas diferencias de ambas construcciones analíticas. Si bien a primera vista las diferencias de ambos sistemas los convierte en sistemas encontrados, en las conclusiones esbozamos algunas razones por las cuales estos sistemas se pueden complementar, lo cualotorgaríauna perspectiva compleja para aprehender el rol de los “sentidos últimos”en la acción social.

  11. Mortality in patients with pituitary disease.

    LENUS (Irish Health Repository)

    Sherlock, Mark

    2010-06-01

    Pituitary disease is associated with increased mortality predominantly due to vascular disease. Control of cortisol secretion and GH hypersecretion (and cardiovascular risk factor reduction) is key in the reduction of mortality in patients with Cushing\\'s disease and acromegaly, retrospectively. For patients with acromegaly, the role of IGF-I is less clear-cut. Confounding pituitary hormone deficiencies such as gonadotropins and particularly ACTH deficiency (with higher doses of hydrocortisone replacement) may have a detrimental effect on outcome in patients with pituitary disease. Pituitary radiotherapy is a further factor that has been associated with increased mortality (particularly cerebrovascular). Although standardized mortality ratios in pituitary disease are falling due to improved treatment, mortality for many conditions are still elevated above that of the general population, and therefore further measures are needed. Craniopharyngioma patients have a particularly increased risk of mortality as a result of the tumor itself and treatment to control tumor growth; this is a key area for future research in order to optimize the outcome for these patients.

  12. Prevalence of mucocutaneous findings in Celiac disease patients

    Directory of Open Access Journals (Sweden)

    Derya Yayla

    2015-12-01

    Full Text Available Background and Design: Celiac disease is an immune-mediated enteropathy which develops as a result of exposure to gluten in food products in individuals with a genetic predisposition. Gastrointestinal and extra-gastrointestinal clinical findings can be seen in these patients. An increased frequence of autoimmune diseases has been reported in patients with celiac disease. Some dermatological diseases, such as dermatitis herpetiformis, vitiligo, psoriasis, alopecia areata and recurrent aphthous stomatitis have been reported to be more common among patients with celiac disease. However, there are no controlled studies on this subject. The aim of this study was to identify the mucocutaneous symptoms seen in celiac patients and to compare these findings with a control group. Materials and Methods: Forty-nine celiac patients and 54 age-and sex-matched healthy volunteers were included in the study. In the patient group, celiac disease history, height and weight parameters, the medications of the patients, compliance to a gluten-free diet, concomitant skin disorders and additional illnesses were questioned; height and weight parameters, diagnosed illnesses, and medications were questioned in the control group. Dermatological analyses were performed in all participants. Results: Mucocutaneous findings were found to be present in 38 patients (77.6% in the celiac patient group and in 31 (57.4% individuals in the control group. The presence of mucocutaneous findings in celiac patients was significantly more common than in the control group. While immune-mediated mucocutaneous diseases were detected in 8 celiac patients (16.3%, none of the individuals in the control group had immune-mediated mucocutaneous diseases and a statistically significant difference was found between the two groups. Conclusion: In celiac patients, the frequency of immune-mediated mucocutaneous diseases and all mucocutaneous diseases were found to be increased. Therefore, we suggest

  13. Inhibitory actions of the gamma-aminobutyric acid in pediatric Sturge-Weber syndrome.

    Science.gov (United States)

    Tyzio, Roman; Khalilov, Ilgam; Represa, Alfonso; Crepel, Valerie; Zilberter, Yuri; Rheims, Sylvain; Aniksztejn, Laurent; Cossart, Rosa; Nardou, Romain; Mukhtarov, Marat; Minlebaev, Marat; Epsztein, Jérôme; Milh, Mathieu; Becq, Helene; Jorquera, Isabel; Bulteau, Christine; Fohlen, Martine; Oliver, Viviana; Dulac, Olivier; Dorfmüller, Georg; Delalande, Olivier; Ben-Ari, Yehezkel; Khazipov, Roustem

    2009-08-01

    The mechanisms of epileptogenesis in Sturge-Weber syndrome (SWS) are unknown. We explored the properties of neurons from human pediatric SWS cortex in vitro and tested in particular whether gamma-aminobutyric acid (GABA) excites neurons in SWS cortex, as has been suggested for various types of epilepsies. Patch-clamp and field potential recordings and dynamic biphoton imaging were used to analyze cortical tissue samples obtained from four 6- to 14-month-old pediatric SWS patients during surgery. Neurons in SWS cortex were characterized by a relatively depolarized resting membrane potential, as was estimated from cell-attached recordings of N-methyl-D-aspartate channels. Many cells spontaneously fired action potentials at a rate proportional to the level of neuronal depolarization. The reversal potential for GABA-activated currents, assessed by cell-attached single channel recordings, was close to the resting membrane potential. All spontaneously firing neurons recorded in cell-attached mode or imaged with biphoton microscopy were inhibited by GABA. Spontaneous epileptiform activity in the form of recurrent population bursts was suppressed by glutamate receptor antagonists, the GABA(A) receptor agonist isoguvacine, and the positive allosteric GABA(A) modulator diazepam. Blockade of GABA(A) receptors aggravated spontaneous epileptiform activity. The NKCC1 antagonist bumetanide had little effect on epileptiform activity. SWS cortical neurons have a relatively depolarized resting membrane potential and spontaneously fire action potentials that may contribute to increased network excitability. In contrast to previous data depicting excitatory and proconvulsive actions of GABA in certain pediatric and adult epilepsies, GABA plays mainly an inhibitory and anticonvulsive role in SWS pediatric cortex.

  14. SCIENTIFIC METHODOLOGY FOR THE APPLIED SOCIAL SCIENCES: CRITICAL ANALYSES ABOUT RESEARCH METHODS, TYPOLOGIES AND CONTRIBUTIONS FROM MARX, WEBER AND DURKHEIM

    Directory of Open Access Journals (Sweden)

    Mauricio Corrêa da Silva

    2015-06-01

    Full Text Available This study aims to discuss the importance of the scientific method to conduct and advertise research in applied social sciences and research typologies, as well as to highlight contributions from Marx, Weber and Durkheim to the scientific methodology. To reach this objective, we conducted a review of the literature on the term research, the scientific method,the research techniques and the scientific methodologies. The results of the investigation revealed that it is fundamental that the academic investigator uses a scientific method to conduct and advertise his/her academic works in applied social sciences in comparison with the biochemical or computer sciences and in the indicated literature. Regarding the contributions to the scientific methodology, we have Marx, dialogued, the dialectical, striking analysis, explicative of social phenomenon, the need to understand the phenomena as historical and concrete totalities; Weber, the distinction between “facts” and “value judgments” to provide objectivity to the social sciences and Durkheim, the need to conceptualize very well its object of study, reject sensible data and imbue with the spirit of discovery and of being surprised with the results.

  15. ISCHEMIC HEART DISEASE IN PATIENTS OF CHRONIC KIDNEY DISEASE ON MAINTENANCE HEMODIALYSIS

    OpenAIRE

    Dr. Aijaz Ahmed, Dr. Muhammad Nadeem Ahsan, Dr. Pooran Mal*, Dr. Hamid Nawaz Ali Memon, Dr. Samreen and Dr. Sajjad Ali

    2017-01-01

    Objective: To determine the frequency of ischemic heart disease in patients of chronic kidney disease on maintenance hemodialysis Patients and Methods: A total of 160 patients with diagnosis of CKD in department of Nephrology, Liaquat National Hospital Karachi were recruited in this six months cross sectional study. Demographic information was recorded. Then patients were underwent ECG. Reports were assessed and ischemic heart disease was labeled while all the data was collected using the pro...

  16. Two adolescent patients with coexistent Graves' disease and Moyamoya disease in Korea.

    Science.gov (United States)

    Cheon, Chong Kun; Kim, Su Yung; Yoo, Jae-Ho

    2014-06-01

    Moyamoya disease is a cerebrovascular condition that results in the narrowing of the vessels of the circle of Willis and collateral vessel formation at the base of the brain. Although relationships between Graves' disease and cerebrovascular accidents in Moyamoya disease are obscure, the coexistence of the two diseases is noteworthy. Moyamoya disease has been rarely reported in adolescent patients with thyrotoxicosis. Recently, we encountered two adolescent Korean patients with Moyamoya disease associated with Graves' disease who presented with episodic right-sided hemiparesis and syncope. These two girls who had Graves' disease had no history of other diseases or head trauma. A thyroid function test revealed a euthyroid state and a high thyroid-stimulating hormone (TSH) receptor antibody titer at that time. The patients were diagnosed with Moyamoya disease based on brain magnetic resonance angiography and cerebral four-vessel angiography. The patients underwent cranial revascularization by encephalo-duroarterio-synangiosis as soon as a diagnosis was made, which resulted in successful symptom resolution. They fared well and had no additional neurological symptoms as of their last follow-up visits. Here, we report these two cases of confirmed Moyamoya disease complicated by Graves' disease with a review of the literature, and discuss the possible association between the two diseases. To our knowledge, this is the first report in South Korea on Moyamoya disease associated with Graves' disease in adolescents with a euthyroid.

  17. Evaluation of the Stillinger-Weber classical interaction potential for tetragonal semiconductors in nonideal atomic configurations

    International Nuclear Information System (INIS)

    Dodson, B.W.

    1986-01-01

    A classical potential incorporating two- and three-body interaction terms has recently been introduced by Stillinger and Weber (SW) for simulation of the liquefaction transition of silicon. The equilibrium mechanical properties of this potential are determined and found to agree well with experimental values. The potential also seems to be adequate for problems involving computation of defect energies, such as the stability of strained-layer superlattice interfaces. However, inadequate treatment of configurations with low coordination number makes modeling of the epitaxial growth of (111) silicon impossible. Simple modifications of the SW potential form do allow for (111) epitaxial growth, but the earliest stages of growth then become unphysical

  18. Radiotherapy in patients with connective tissue diseases.

    Science.gov (United States)

    Giaj-Levra, Niccolò; Sciascia, Savino; Fiorentino, Alba; Fersino, Sergio; Mazzola, Rosario; Ricchetti, Francesco; Roccatello, Dario; Alongi, Filippo

    2016-03-01

    The decision to offer radiotherapy in patients with connective tissue diseases continues to be challenging. Radiotherapy might trigger the onset of connective tissue diseases by increasing the expression of self-antigens, diminishing regulatory T-cell activity, and activating effectors of innate immunity (dendritic cells) through Toll-like receptor-dependent mechanisms, all of which could potentially lead to breaks of immune tolerance. This potential risk has raised some debate among radiation oncologists about whether patients with connective tissue diseases can tolerate radiation as well as people without connective tissue diseases. Because the number of patients with cancer and connective tissue diseases needing radiotherapy will probably increase due to improvements in medical treatment and longer life expectancy, the issue of interactions between radiotherapy and connective tissue diseases needs to be clearer. In this Review, we discuss available data and evidence for patients with connective tissue diseases treated with radiotherapy. Copyright © 2016 Elsevier Ltd. All rights reserved.

  19. An Analysis of Emergency Department Overcrowding at The Johns Hopkins Hospital

    Science.gov (United States)

    2001-06-20

    Jefferson 3rd floor (JEF3), Medical Care Progressive Unit ( MPC4 ), Nelson 4th floor (NEL4), Osler 4th floor (OSL4), Osler 5th floor (OSL5), and Osler...ranged from a low of 73.78% for OSL5 to a high of 94.60% for JEF3. The LOS for the DOM units ranged from 2.89 days for HAL5 to 10.39 days for MPC4

  20. Misdiagnosis of Addison's disease in a patient with end-stage renal disease.

    Science.gov (United States)

    Kocyigit, Ismail; Unal, Aydin; Tanriverdi, Fatih; Hayri Sipahioglu, Murat; Tokgoz, Bulent; Oymak, Oktay; Utas, Cengiz

    2011-01-01

    Addison's disease is a rare disorder in patients with end-stage renal disease (ESRD). In patients, the diagnosis of Addison's disease is difficult in clinical practice because most of the clinical findings of this disease are similar to those of the renal failure. We present a 51-year-old male patient, who underwent hemodialysis therapy for 8 years, diagnosed with Addison's disease after having myalgia, skin hyperpigmentation, weight loss, sweating, and nausea for the past few weeks. The physical examination was completely normal except for muscle weakness, hyperpigmentation on labial mucosa and skin in a patient. The laboratory tests revealed anemia and hypoglycemia. Serum cortisol, adrenocorticotropic hormone (ACTH) levels, and ACTH stimulation test results were consistent with Addison's disease. Adrenal computerized tomography revealed bilateral atrophic glands. Additionally, it was found that elevated serum thyroid stimulating hormone levels and antithyroid peroxidase antibody titer were positive. Our purpose is to emphasize that physicians should be alert to the potential for additional different conditions particularly in terms of adrenal failure in patients with ESRD.

  1. [Serum glycosaminoglycans in Graves' disease patients].

    Science.gov (United States)

    Winsz-Szczotka, Katarzyna B; Olczyk, Krystyna Z; Koźma, Ewa M; Komosińska-Vassev, Katarzyna B; Wisowski, Grzegorz R; Marcisz, Czesław

    2006-01-01

    The aim of the study was to determine the blood serum sulfated glycosaminoglycans (GAGs) and hyaluronic acid (HA) concentration of Graves' disease patients before treatment and after attainment of the euthyroid state. The study was carried out on the blood serum obtained from 17 patients with newly recognised Graves' disease and from the same patients after attainment of the euthyroid state. Graves' patients had not any clinical symptoms neither of ophthalmopathy nor pretibial myxedema. GAGs were isolated from the blood serum by the multistage extraction and purification using papaine hydrolysis, alkali elimination, as well as cetylpyridium chloride binding. Total amount of GAGs was quantified by the hexuronic acids assay. HA content in obtained GAGs sample was evaluated by the ELISA method. Increased serum concentration of sulfated GAGs in non-treated Graves' disease patients was found. Similarly, serum HA level in untreated patients was significantly elevated. The attainment of euthyroid state was accompanied by the decreased serum sulfated GAGs level and by normalization of serum HA concentration. In conclusion, the results obtained demonstrate that the alterations of GAGs metabolism connected with Graves' disease can lead to systemic changes of the extracellular matrix properties.

  2. Colorectal cancer in patients with inflammatory bowel disease

    DEFF Research Database (Denmark)

    Andersen, Vibeke; Halfvarson, Jonas; Vogel, Ulla Birgitte

    2012-01-01

    The inflammatory bowel diseases (IBD), Crohn's disease (CD) and ulcerative colitis (UC), may be complicated by colorectal cancer (CRC). In a recent population-based cohort study of 47 347 Danish patients with IBD by Tine Jess and colleagues 268 patients with UC and 70 patients with CD developed C...... preventive strategies in order to avoid CRC in IBD patients. The achieved knowledge may also be relevant for other inflammation-associated cancers.......The inflammatory bowel diseases (IBD), Crohn's disease (CD) and ulcerative colitis (UC), may be complicated by colorectal cancer (CRC). In a recent population-based cohort study of 47 347 Danish patients with IBD by Tine Jess and colleagues 268 patients with UC and 70 patients with CD developed CRC...... during 30 years of observation. The overall risk of CRC among patients with UC and CD was comparable with that of the general population. However, patients diagnosed with UC during childhood or as adolescents, patients with long duration of disease and those with concomitant primary sclerosing...

  3. [Thyroid cancer in patients with Grave's Disease].

    Science.gov (United States)

    Mssrouri, R; Benamr, S; Essadel, A; Mdaghri, J; Mohammadine, El H; Lahlou, M-K; Taghy, A; Belmahi, A; Chad, B

    2008-01-01

    To evaluate the incidence of thyroid carcinoma in patients operated on for Graves' disease, to identify criteria which may predict malignancy, and to develop a practical approach to determine the extensiveness of thyroidectomy. Retrospective study of all patients who underwent thyroidectomy for Graves' disease between 1995 and 2005. 547 patients underwent subtotal thyroidectomy for Graves' disease during this period. Post-operative pathology examination revealed six cases of thyroid cancer (1.1%). All six cases had differentiated thyroid carcinoma (papillary carcinoma in 3 cases, follicular carcinoma in 2 cases and papillo-follicular carcinoma in 1 case). The indication for initial thyroidectomy was a palpable thyroid nodule in 3 cases (50%), failure of medical treatment for Grave's disease in 2 cases (33%), and signs of goiter compression in 1 case (17%). Five patients underwent re-operative total thyroidectomy. This study shows that while malignancy in Grave's disease is uncommon, the presence of thyroid nodule(s) in patients with Grave's disease may be considered as an indication for radical surgery. The most adequate radical surgery in this situation is to perform a total thyroidectomy.

  4. Lumbar Spine Surgery in Patients with Parkinson Disease.

    Science.gov (United States)

    Schroeder, Joshua E; Hughes, Alexander; Sama, Andrew; Weinstein, Joseph; Kaplan, Leon; Cammisa, Frank P; Girardi, Federico P

    2015-10-21

    Parkinson disease is the second most common neurodegenerative condition. The literature on patients with Parkinson disease and spine surgery is limited, but increased complications have been reported. All patients with Parkinson disease undergoing lumbar spine surgery between 2002 and 2012 were identified. Patients' charts, radiographs, and outcome questionnaires were reviewed. Parkinson disease severity was assessed with use of the modified Hoehn and Yahr staging scale. Complications and subsequent surgeries were analyzed. Risk for reoperation was assessed. Ninety-six patients underwent lumbar spine surgery. The mean patient age was 63.0 years. The mean follow-up duration was 30.1 months. The Parkinson disease severity stage was Parkinson disease severity stage of ≥3 (p Parkinson disease is good, with improvement of spine-related pain. A larger prospective study is warranted. Copyright © 2015 by The Journal of Bone and Joint Surgery, Incorporated.

  5. Frequency of undiagnosed chronic obstructive pulmonary disease in patients with coronary artery disease

    International Nuclear Information System (INIS)

    Ullah, R.; Ghaffar, T.; Khan, I.; Muhammad, R.; Salman, S.

    2017-01-01

    Chronic obstructive airway disease (COPD) is considered as risk factor for coronary artery disease (CAD) along with other risk factors. This study was conducted to determine the frequency of undiagnosed chronic obstructive pulmonary disease in patients with coronary artery disease. Methods: This cross-sectional study was conducted in the Pulmonology and Cardiology wards/OPD's of Khyber Teaching Hospital Peshawar. Patients more than 35 years of age, diagnosed with CAD of either gender were included. Patients already diagnosed with COPD, recent myocardial infarction (within 7 days), left ventricular impairment, pneumothorax, bronchiectasis, comatose patient, asthmatic and those with chest trauma were excluded. All the patients underwent spirometry examination before and after administration of salbutamol (5 mg for 5 minutes) via nebulizer. FEV1/FVC less than 70% confirmed the presence of COPD. Results: Out of 151 patients, 57 (37.7%) were found to have COPD. Among them, 39 (68.42%) were male and 18 (31.57%) were female. Among male patients with COPD, 82.05% (n=32) were smokers and 17.94% (n=7) were nonsmokers while in females with COPD no one was smoker. Conclusion: COPD is an under-diagnosed progressive disease in patients with high risk patients with coronary artery disease. (author)

  6. DIAGNÓSTICO TARDÍO DE INTOXICACIÓN POR FENITOÍNA EN UN PACIENTE CON SÍNDROME DE STURGE-WEBER

    Directory of Open Access Journals (Sweden)

    Yesenia M Huerta-Collado

    2013-07-01

    Full Text Available Introducción: La Fenitoína es un fármaco antiepiléptico utilizado comúnmente en crisis focales y tónico-clónicas generalizadas. Este fármaco tiene una cinética no lineal compleja, alta unión a proteínas y una ventana terapéutica estrecha, por lo cual la intoxicación se convierte en un riesgo latente. El cuadro clínico de intoxicación por fenitoína está conformado por alteración de la función cerebelosa y vestibular, somnolencia, debilidad generalizada, visión bo­rrosa, movimientos coreo-atetósicos y coma. Caso clínico: Paciente masculino de 20 años de edad con diagnóstico de síndrome de Sturge-Weber asociado a crisis epilépticas parciales complejas tratado con 300 mg de fenitoína diaria durante tres meses, tiempo en el que presenta síntomas progresivos caracterizados por somnolencia, visión borrosa, imposibilidad para la marcha, disartria y movimientos involuntarios coreo-atetósicos que fueron interpretados como parte de su enfermedad hasta antes de su ingreso al Instituto Nacional de Ciencias Neurológicas, Lima, Perú. El paciente ingresa con un título de 75,6 ug/ml de fenitoína en plasma, por lo que se decide el retiro del fármaco y su reemplazo por fenobarbital. El cuadro clínico fue remitiendo conforme disminuían los títulos de fenitoína en el paciente. Interpretación: La evaluación de factores asociados al metabolismo y monitorización periódica de los niveles séricos de fenitoína en los pacientes que reciben este fármaco es crucial, asimismo es importante el conocimiento de síntomas que indiquen toxicidad para que no sean confundidos con manifestaciones presentes en enfermedades subyacentes como el síndrome de Sturge-Weber en este caso. Palabras Clave: Atetosis, Fenitoína, síndrome cerebeloso, síndrome Sturge-Weber, toxicidad

  7. Diagnosis and treatment of invasive fungal diseases in patients with severe liver diseases

    Directory of Open Access Journals (Sweden)

    ZANG Hong

    2016-09-01

    Full Text Available Invasive fungal diseases (IFDs are an important factor affecting the prognosis of patients with severe liver diseases, and their early diagnosis remains a challenge for clinicians. The four most commonly seen IFDs are candidiasis, aspergillosis, cryptococcosis, and pneumocystis pneumonia. We should pay attention to the risk of developing IFDs in patients with severe liver diseases during clinical management. Particularly, early diagnosis and proper treatment of IFDs are important in high-risk patients. These are vital to improving the prognosis of patients with severe liver diseases.

  8. A prospective study of risk for Sturge-Weber syndrome in children with upper facial port-wine stain.

    Science.gov (United States)

    Dutkiewicz, Anne-Sophie; Ezzedine, Khaled; Mazereeuw-Hautier, Juliette; Lacour, Jean-Philippe; Barbarot, Sébastien; Vabres, Pierre; Miquel, Juliette; Balguerie, Xavier; Martin, Ludovic; Boralevi, Franck; Bessou, Pierre; Chateil, Jean-François; Léauté-Labrèze, Christine

    2015-03-01

    Upper facial port-wine stain (PWS) is a feature of Sturge-Weber syndrome (SWS). Recent studies suggest that the distribution of the PWS corresponds to genetic mosaicism rather than to trigeminal nerve impairment. We sought to refine the cutaneous distribution of upper facial PWS at risk for SWS. This was a prospective multicenter study of consecutive cases of upper facial PWS larger than 1 cm² located in the ophthalmic division of trigeminal nerve distribution in infants aged less than 1 year, seen in 8 French pediatric dermatology departments between 2006 and 2012. Clinical data, magnetic resonance imaging, and photographs were systematically collected and studied. PWS were classified into 6 distinct patterns. In all, 66 patients were included. Eleven presented with SWS (magnetic resonance imaging signs and seizure). Four additional infants had suspected SWS without neurologic manifestations. Hemifacial (odds ratio 7.7, P = .003) and median (odds ratio 17.08, P = .008) PWS patterns were found to be at high risk for SWS. A nonmedian linear pattern was not associated with SWS. Small number of patients translated to limited power of the study. Specific PWS distribution patterns are associated with an increased risk of SWS. These PWS patterns conform to areas of somatic mosaicism. Terminology stipulating ophthalmic division of trigeminal nerve territory involvement in SWS should be abandoned. Copyright © 2014 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  9. Psychotropic medication use among patients with celiac disease.

    Science.gov (United States)

    Zylberberg, Haley M; Ludvigsson, Jonas F; Green, Peter H R; Lebwohl, Benjamin

    2018-03-27

    Celiac disease is a multi-system disorder with manifestations that may result in psychiatric disorders. We assessed the prevalence of medication use to treat psychiatric disorders in celiac disease patients. We conducted a cross-sectional study of patients undergoing esophagogastroduodenoscopy over 9-years at a celiac disease referral center. We compared the prevalence of psychotropic medication use among celiac disease patients (n = 1293) to a control group (n = 1401) with abdominal pain or reflux. Among all patients the mean age was 48.4 years, most were female (69.5%), and 22.7% used any psychotropic medication. There was no difference between overall psychotropic medication use among celiac disease patients and controls (23.9% vs 21.8%, OR 1.16; 95% CI 0.96-1.39, p = 0.12). However, those with celiac disease were more likely to use antidepressants on univariate (16.4% vs 13.4%, p = 0.03) and multivariate analysis (OR 1.28; 95% CI 1.03-1.59; p = 0.03). Use of psychotropic medications was not associated with disease duration or mode of presentation of celiac disease. Celiac disease patients use psychotropic medications at similar rates as those with other gastrointestinal diseases, though subgroup analysis suggests they may use more antidepressants. Future studies should investigate whether celiac disease is associated with mood disorders that are not treated with medications.

  10. Gallstones in Patients with Chronic Liver Diseases

    Directory of Open Access Journals (Sweden)

    Xu Li

    2017-01-01

    Full Text Available With prevalence of 10–20% in adults in developed countries, gallstone disease (GSD is one of the most prevalent and costly gastrointestinal tract disorders in the world. In addition to gallstone disease, chronic liver disease (CLD is also an important global public health problem. The reported frequency of gallstone in chronic liver disease tends to be higher. The prevalence of gallstone disease might be related to age, gender, etiology, and severity of liver disease in patients with chronic liver disease. In this review, the aim was to identify the epidemiology, mechanisms, and treatment strategies of gallstone disease in chronic liver disease patients.

  11. Karl Marx vis-à-vis Max Weber: perspectiva comparada das visões acerca do Estado

    Directory of Open Access Journals (Sweden)

    Felippe Ramos

    2010-12-01

    Full Text Available Este trabalho aborda a distinção analítica entre Marx e Weber, em relação ao tema do Estado. A hipótese é de que ambos os autores, a despeito do que pode parecer à primeira vista, estavam imbuídos da impossibilidade de um Estado impessoal, o que remete, em última instância, à politização do Estado. O que parece claro na visão de Marx é, contudo, obscurecido pela construção metodológica (e epistemológica de Weber, com base na busca constante da objetividade do conhecimento, a partir de uma ciência que se afasta do engajamento político e da importância das categorias analíticas da burocracia e racionalização. Essa dificuldade de construir uma análise crítica da proposta weberiana pode ser esclarecida, contudo, constituindo uma análise do Estado, em oposição à esfera de legitimação do Estado moderno que se encontra claramente na burocracia, cuja característica maior é a impessoalidade, a partir da descrição de suas instâncias de decisão – um elemento político sobre a burocracia. A análise marxiana do Estado classista, por sua vez, também precisa ter suas nuances analisadas de acordo com o próprio amadurecimento teórico do autor, que, em suas obras de análise de eventos históricos escritas tardiamente, torna complexa a classificação do Estado, demonstrando suas aporias e disputas internas, para além de sua visão inicialmente instrumental do Estado.

  12. Polycythemia Vera

    Science.gov (United States)

    ... Polycythemia Vera Also known as What Is Polycythemia vera (POL-e-si-THEE-me-ah VAY-rah or VE- ... MY-e-lo-PATH-ik) polycythemia Myeloproliferative (MY-e-lo-pro-LIF-er-ah-tiv) disorder Osler disease Polycythemia rubra vera Polycythemia with chronic cyanosis (SI-ah-NO-sis) ...

  13. Max Weber: posición política, posición teórica y relación con el marxismo en la primera etapa de su producción

    Directory of Open Access Journals (Sweden)

    María Celia Duek

    2009-01-01

    Full Text Available Max Weber redactó sus primeros trabajos en tre 1889 y 1898. Si bien las obras más conocidas del sociólogo alemán fueron escritas en tre 1903 y 1920, es en la producción de la primera etapa en donde ponemos la atención en este artículo. Particularmente nos interesa responder a la siguiente interrogante: ¿cuál fue la relación teórica de Weber con Marx o con el materialismo histórico en dicha fase de su carrera y cómo se vincula esto con las posiciones políticas de ambos clásicos?.

  14. The relationship between different information sources and disease-related patient knowledge and anxiety in patients with inflammatory bowel disease.

    Science.gov (United States)

    Selinger, C P; Carbery, I; Warren, V; Rehman, A F; Williams, C J; Mumtaz, S; Bholah, H; Sood, R; Gracie, D J; Hamlin, P J; Ford, A C

    2017-01-01

    Patient education forms a cornerstone of management of inflammatory bowel disease (IBD). The Internet has opened new avenues for information gathering. To determine the relationship between different information sources and patient knowledge and anxiety in patients with IBD. The use of information sources in patients with IBD was examined via questionnaire. Anxiety was assessed with the hospital anxiety and depression scale and disease-related patient knowledge with the Crohn's and colitis knowledge score questionnaires. Associations between these outcomes and demographics, disease-related factors, and use of different information sources were analysed using linear regression analysis. Of 307 patients (165 Crohn's disease, 142 ulcerative colitis) 60.6% were female. Participants used the hospital IBD team (82.3%), official leaflets (59.5%), and official websites (53.5%) most frequently in contrast to alternative health websites (9%). University education (P sex (P = 0.004), clinically active disease (P sources are associated with better knowledge or worse anxiety levels. Face-to-face education and written information materials remain the first line of patient education. Patients should be guided towards official information websites and warned about the association between the use of alternative health websites or random links and anxiety. © 2016 John Wiley & Sons Ltd.

  15. Reflexión teórica sobre la validez de los preceptos de acción social de Max Weber para el análisis del campesinado actual Theoretical reflection on the validity of the rules of social action of Max Weber for the analysis of the present peasantry

    Directory of Open Access Journals (Sweden)

    Diosey Ramón Lugo Morin

    2005-12-01

    Full Text Available En un fin de siglo marcado por un nuevo orden mundial, los campesinos no pueden quedarse al margen; la nueva socialidad rural ya no sólo tiene sus nudos en barrios y comunidades, también en proyectos y aparatos económicos gestionados por las organizaciones. Las empresas medianas o grandes de carácter asociativo son parte del nuevo perfil campesino y los conocimientos y las habilidades necesarias para operarlos se están incorporando a su cultura. El campesino no es una persona o familia, el campesino es un fenómeno social, él se ubica en el contexto histórico, ético, folklórico y religioso y difícilmente puede ser definible, ni siquiera indefinible, debido a la multiplicidad de roles que cumple en la sociedad actual. El campesino actual es una mezcla de pasado, presente y futuro, esta afirmación en definitiva es la clave de su reproducción. En este sentido, tratar de entender y comprender las organizaciones campesinas a través de teorías sociológicas propuestas en el pasado, en especial el concepto de acción social de Max Weber sería poco prudente, ya que las estructuras de valores de estos individuos ha cambiado sustancialmente o de lo contrario ha desaparecido. Aunque el concepto de los valores impera, el mismo está inmerso dentro un momento histórico distinto al de Weber. La finalidad del presente ensayo es hacer una reflexión teóricaacerca de los preceptos de acción social de Weber y mostrar que los mismos no son una herramienta de análisis válida ni adecuada para entender al individuo actual, en especial al campesino. La acción en este sentido se origina en una interacción de individuos o grupos, los cuales a su vez de manera individual o grupal están caracterizados por su sistema psíquico (no solamente se considera la estructura de valores delindividuo, también existen otros elementos tales como las necesidades e impulsos. Este micro-entorno que se genera debe estar en contacto con la complejidad del mundo o

  16. Chronic kidney disease in congenital heart disease patients: a narrative review of evidence.

    Science.gov (United States)

    Morgan, Catherine; Al-Aklabi, Mohammed; Garcia Guerra, Gonzalo

    2015-01-01

    Patients with congenital heart disease have a number of risk factors for the development of chronic kidney disease (CKD). It is well known that CKD has a large negative impact on health outcomes. It is important therefore to consider that patients with congenital heart disease represent a population in whom long-term primary and secondary prevention strategies to reduce CKD occurrence and progression could be instituted and significantly change outcomes. There are currently no clear guidelines for clinicians in terms of renal assessment in the long-term follow up of patients with congenital heart disease. Consolidation of knowledge is critical for generating such guidelines, and hence is the purpose of this view. This review will summarize current knowledge related to CKD in patients with congenital heart disease, to highlight important work that has been done to date and set the stage for further investigation, development of prevention strategies, and re-evaluation of appropriate renal follow-up in patients with congenital heart disease. The literature search was conducted using PubMed and Google Scholar. Current epidemiological evidence suggests that CKD occurs in patients with congenital heart disease at a higher frequency than the general population and is detectable early in follow-up (i.e. during childhood). Best evidence suggests that approximately 30 to 50 % of adult patients with congenital heart disease have significantly impaired renal function. The risk of CKD is higher with cyanotic congenital heart disease but it is also present with non-cyanotic congenital heart disease. Although significant knowledge gaps exist, the sum of the data suggests that patients with congenital heart disease should be followed from an early age for the development of CKD. There is an opportunity to mitigate CKD progression and negative renal outcomes by instituting interventions such as stringent blood pressure control and reduction of proteinuria. There is a need to

  17. Procedural learning changes in patients with Wilson's disease

    Institute of Scientific and Technical Information of China (English)

    Yumei Jiang; Xiang Shen; Xiaoping Wang; Wenjie Li

    2011-01-01

    In the present study, we compared explicit memory performance, using the Wechsler Memory Scale, and implicit memory performance, using the Nissen software version of the serial reaction time task, in patients with Wilson's disease to normal controls. The Wilson's disease patients exhibited deficits in explicit memory tasks, such as figure recall and understanding memory. Moreover, the Wilson's disease patients exhibited deficits in implicit memory tasks, including significantly prolonged response times. These findings indicate that Wilson's disease patients have explicit and implicit partial memory impairments.

  18. Facial expression recognition based on weber local descriptor and sparse representation

    Science.gov (United States)

    Ouyang, Yan

    2018-03-01

    Automatic facial expression recognition has been one of the research hotspots in the area of computer vision for nearly ten years. During the decade, many state-of-the-art methods have been proposed which perform very high accurate rate based on the face images without any interference. Nowadays, many researchers begin to challenge the task of classifying the facial expression images with corruptions and occlusions and the Sparse Representation based Classification framework has been wildly used because it can robust to the corruptions and occlusions. Therefore, this paper proposed a novel facial expression recognition method based on Weber local descriptor (WLD) and Sparse representation. The method includes three parts: firstly the face images are divided into many local patches, and then the WLD histograms of each patch are extracted, finally all the WLD histograms features are composed into a vector and combined with SRC to classify the facial expressions. The experiment results on the Cohn-Kanade database show that the proposed method is robust to occlusions and corruptions.

  19. Dental profile of patients with Gaucher disease

    Science.gov (United States)

    Fischman, Stuart L; Elstein, Deborah; Sgan-Cohen, Harold; Mann, Jonathan; Zimran, Ari

    2003-01-01

    Background This study was conducted to determine whether patients with Gaucher disease had significant dental pathology because of abnormal bone structure, pancytopenia, and coagulation abnormalities. Methods Each patient received a complete oral and periodontal examination in addition to a routine hematological evaluation. Results Gaucher patients had significantly fewer carious lesions than otherwise healthy carriers. Despite prevalence of anemia, there was no increase in gingival disease; despite the high incidence of thrombocytopenia, gingival bleeding was not noted; and despite radiological evidence of bone involvement, there was no greater incidence loss of teeth or clinical tooth mobility. Conclusions These data represent the first survey of the oral health of a large cohort of patients with Gaucher disease. It is a pilot study of a unique population and the results of the investigation are indications for further research. Based on our findings, we recommend regular oral examinations with appropriate dental treatment for patients with Gaucher disease as for other individuals. Consultation between the dentist and physician, preferably one with experience with Gaucher disease, should be considered when surgical procedures are planned. PMID:12875661

  20. Empowering Patients with Chronic Diseases

    DEFF Research Database (Denmark)

    Bestek, Mate; Meglič, Matic; Kurent, Blaž

    2012-01-01

    Background: Chronic diseases require most of the resources in todays healthcare systems. Healthcare systems, as such, are thus not sustainable in the long term. Solutions to this problem are needed and a lot of research is focused on finding new approaches to more sustainable healthcare systems...... himself to become empowered. The patient needs to see data about his health in order to start thinking about new decisions in life that can lead to change in his behaviour. Objective: We have approached the problem of empowering patients with chronic diseases from a biological, psychological, sociological....... We want to develop extensible technology to support even more new interventions for different chronic diseases. We want the technology to enable semantic interoperability with other systems. Methods: We have collaborated with doctors in order to model the care plans for different chronic diseases...

  1. Role of CT in patients with prostatic disease

    International Nuclear Information System (INIS)

    Yoshizako, Takeshi; Sugimura, Kazuro; Kaji, Yasushi; Moriyama, Masahiro; Ishida, Tetsuya

    1994-01-01

    The purpose of this study was to evaluate the role of CT in patients with and without prostatic disease. CT and MR findings were reviewed in 25 patients without known prostatic disease, 11 patients with benign prostatic hyperplasia and 11 patients with prostatic cancer. Differential attenuation allowed for distinction of the peripheral zone and inner gland of the prostate by CT in 72% of normal patients. The distinction rate of prostatic zonal anatomy by CT decreased to 30% in the diseased group. When zonal anatomy of the prostate is not visualized on pelvic enhanced CT, the presence of prostatic disease might be considered. (author)

  2. Clinically Unsuspected Prion Disease Among Patients With Dementia Diagnoses in an Alzheimer's Disease Database.

    Science.gov (United States)

    Maddox, Ryan A; Blase, J L; Mercaldo, N D; Harvey, A R; Schonberger, L B; Kukull, W A; Belay, E D

    2015-12-01

    Brain tissue analysis is necessary to confirm prion diseases. Clinically unsuspected cases may be identified through neuropathologic testing. National Alzheimer's Coordinating Center (NACC) Minimum and Neuropathologic Data Set for 1984 to 2005 were reviewed. Eligible patients had dementia, underwent autopsy, had available neuropathologic data, belonged to a currently funded Alzheimer's Disease Center (ADC), and were coded as having an Alzheimer's disease clinical diagnosis or a nonprion disease etiology. For the eligible patients with neuropathology indicating prion disease, further clinical information, collected from the reporting ADC, determined whether prion disease was considered before autopsy. Of 6000 eligible patients in the NACC database, 7 (0.12%) were clinically unsuspected but autopsy-confirmed prion disease cases. The proportion of patients with dementia with clinically unrecognized but autopsy-confirmed prion disease was small. Besides confirming clinically suspected cases, neuropathology is useful to identify unsuspected clinically atypical cases of prion disease. © The Author(s) 2015.

  3. Celiac disease in patients with Williams-Beuren syndrome.

    Science.gov (United States)

    Mıhçı, Ercan; Nur, Banu Güzel; Berker-Karaüzüm, Sibel; Yılmaz, Aygen; Artan, Reha

    2015-01-01

    Celiac disease is an autoimmune, gastrointestinal disorder characterized by intolerance to the dietary grain protein gluten. An increased prevalence of celiac disease has been reported in Down syndrome and Turner syndrome, but there has been only few previous reports with respect to the association of celiac disease in Williams-Beuren syndrome. The aim of this study was to evaluate the frequency of celiac disease in our 24 Williams-Beuren syndrome patients. Gastrointestinal problems and celiac disease symptoms of patients were noted. All patients were analyzed by the titer of tissue transglutaminases IgA and IgG. HLA genotyping and intestinal biopsy was performed to the patients with positive serology. We also performed gluten free diet in the presence of compatible symptoms, serology, HLA genotyping and intestinal biopsy. In our study, two patients had positive tTG antibodies, but only one had positive biopsy finding for celiac disease. The frequency of celiac disease in patients with Williams-Beuren syndrome was estimated as 1/24 (4.1%). Though the number of participants in this study was limited, the results show that the frequency of celiac disease is higher in Williams-Beuren syndrome compared to the general population. We suggest that a high suspicion and testing for celiac disease should be recommended at certain intervals in all cases with Williams-Beuren syndrome to detect the cause of growth retardation and gastrointestinal problems.

  4. Early Sociology of the Business Enterprise: Max Weber's Theory of the Modern Business Enterprise in Economy and Society

    DEFF Research Database (Denmark)

    Jagd, Søren

    be explained as the unfolding of a sociological theory of the modern business enterprise. Some of the most important features of Weber’s theory of the modern business enterprise are presented. Weber points to the multidimensional institutional embeddedness of the modern business enterprise and to the crucial...... importance of ongoing tensions between formal and substantive rationality. Weber’s theory of the modern business enterprise in chapter 2 of Economy and Society may then be seen as an important but still unexplored early contribution to a sociological theory of the modern business enterprise....

  5. The spectrum of bone disease in Jordanian hemodialysis patients

    International Nuclear Information System (INIS)

    Younes, Nidal A.; Al-Mansour, M.; Sroujieh, Ahmad S.; Wahbeh, A.; Ailabouni, W.; Hamzah, Y.; Mahafzah, W.

    2006-01-01

    To evaluate the spectrum of mineral abnormalities and bone disease (BD) in hemodialysis patients at Jordan University Hospital (JUH), Amman, Jordan. A cross-sectional study was conducted among 63 patients (38 males and 25 females), mean age 44.19 years (range 17-76 years), with chronic kidney disease (CKD) on regular hemodialysis at JUH between November 2004 and April 2005. All patients have undergone complete blood count, chemistry profile, alkaline phosphatase, serum albumin, intact parathyroid hormone (iPTH) and plain x-rays. Bone disorders were identified in 45 patients on x-rays (70%). Osteopenia was found in 43 patients (68.3%), subperiosteal resorption in 24 patients (38.3%) and metastatic calcification in 22 patients (35%). Hypocalcemia was found in 28.6% and hypercalcemia in 7.9%. All patients were taking calcium carbonate, and 55.5% of patients were on vitamin D supplements. The calcium levels in 63.5% and the phosphorus levels in 50.8% of patients were within the recommended guidelines of the National Kidney Foundation Kidney Disease Outcomes Quality Initiative (K/DOQI). Serum i-PTH level was above 300 pg/ml high turnover bone disease in 24.6% of patients, 21.3% had iPTH of 150-300 pg/ml target, and 44.3% had i-PTH levels below 100 pg/mL suggesting a dynamic bone disease. Patients with severe bone disease had a statistically significant higher iPTH levels (p<0.005). Bone disease and mineral abnormalities are common in hemodialysis patients at JUH. Earlier detection of bone disease and better overall management strategy may reduce the frequency and severity of bone disease in CKD patients in Jordan. (author)

  6. Screening Fabry's disease in chronic kidney disease patients not on dialysis: a multicenter study.

    Science.gov (United States)

    Yeniçerioğlu, Yavuz; Akdam, Hakan; Dursun, Belda; Alp, Alper; Sağlam Eyiler, Funda; Akın, Davut; Gün, Yelda; Hüddam, Bülent; Batmazoğlu, Mehmet; Gibyeli Genek, Dilek; Pirinççi, Serhat; Ersoy, İsmail Rıfkı; Üzüm, Atilla; Soypaçacı, Zeki; Tanrısev, Mehmet; Çolak, Hülya; Demiral Sezer, Sibel; Bozkurt, Gökay; Akyıldız, Utku Oğan; Akyüz Ünsal, Ayşe İpek; Ünübol, Mustafa; Uslu, Meltem; Eryılmaz, Ufuk; Günel, Ceren; Meteoğlu, İbrahim; Yavaşoğlu, İrfan; Ünsal, Alparslan; Akar, Harun; Okyay, Pınar

    2017-11-01

    Fabry's disease is an X-linked inherited, rare, progressive, lysosomal storage disorder, affecting multiple organs due to the deficient activity of α-galactosidase A (α-Gal A) enzyme. The prevalence has been reported to be 0.15-1% in hemodialysis patients; however, the information on the prevalence in chronic kidney disease not on dialysis is lacking. This study aimed to determine the prevalence of Fabry's disease in chronic kidney disease. The patients older than 18 years, enclosing KDIGO 2012 chronic kidney disease definitions, not on dialysis, were enrolled. Dried blood spots on Guthrie papers were used to analyze α-Gal A enzyme and genetic analysis was performed in individuals with enzyme activity ≤1.2 μmol/L/h. A total of 1453 chronic kidney disease patients not on dialysis from seven clinics in Turkey were screened. The mean age of the study population was 59.3 ± 15.9 years. 45.6% of patients were female. The creatinine clearance of 77.3% of patients was below 60 mL/min/1.73 m 2 , 8.4% had proteinuria, and 2.5% had isolated microscopic hematuria. The mean value of patients' α-Gal A enzyme was detected as 2.93 ± 1.92 μmol/L/h. 152 patients had low levels of α-Gal A enzyme activity (≤1.2 μmol/L/h). In mutation analysis, A143T and D313Y variants were disclosed in three male patients. The prevalence of Fabry's disease in chronic kidney disease not on dialysis was found to be 0.2% (0.4% in male, 0.0% in female). Fabry's disease should be considered in the differential diagnosis of chronic kidney disease with unknown etiology even in the absence of symptoms and signs suggestive of Fabry's disease.

  7. Prevalence of celiac disease among first-degree relatives of Indian celiac disease patients.

    Science.gov (United States)

    Mishra, Asha; Prakash, Shyam; Kaur, Gurvinder; Sreenivas, Vishnubhatla; Ahuja, Vineet; Gupta, Siddhartha Datta; Makharia, Govind K

    2016-03-01

    Celiac disease, once thought to be uncommon in Asia, is now recognized in Asian nations as well. We investigated the prevalence of celiac disease in first-degree relatives of celiac disease patients followed in our centre. First-degree relatives were screened prospectively for celiac disease using questionnaire-based interview and anti-tissue transglutaminase antibody. Serology positive first-degree relatives underwent duodenal biopsies. Diagnosis of celiac disease was made based on positive serology and villous abnormality Marsh grade 2 or higher. Human leucocyte antigen DQ2/-DQ8 was also assessed in 127 first-degree relatives. 434 first-degree relatives of 176 celiac disease patients were prospectively recruited; 282 were symptomatic (64.9%), 58 were positive for serology (13.3%). Seroprevalence was higher in female than in males (19% vs 8.5%; p=0.001) and highest in siblings (16.9%) than parents (13.6%) and children (5.9%) of celiac patients (p=0.055); 87.4% first-degree relatives were human leucocyte antigen-DQ2/-DQ8 positive. Overall prevalence of celiac disease was 10.9% amongst first-degree relatives. The prevalence of celiac disease in first-degree relatives of celiac disease patients was 10.9% in our cohort, and 87% had human leucocyte antigen-DQ2 or -DQ8 haplotype. All first-degree relatives of celiac disease patients should be screen for celiac disease even if asymptomatic or with atypical manifestations. Copyright © 2015 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

  8. Small bowel ultrasound in patients with celiac disease

    Energy Technology Data Exchange (ETDEWEB)

    Bartusek, D. [Department of Radiology, Masaryk University hospital Brno (Czech Republic)], E-mail: dbartusek@fnbrno.cz; Valek, V. [Department of Radiology, Masaryk University hospital Brno (Czech Republic)], E-mail: v.valek@fnbrno.cz; Husty, J. [Department of Radiology, Masaryk University hospital Brno (Czech Republic)], E-mail: jhusty@fnbrno.cz; Uteseny, J. [Department of Pediatric Internal Medicine, Masaryk University hospital Brno (Czech Republic)], E-mail: juteseny@fnbrno.cz

    2007-08-15

    Objective: Celiac disease (CD) is a common, lifelong disease with small bowel malabsorption based on genetically conditioned gluten intolerance. The clinical manifestation could be very heterogeneous. The proof of celiac disease is now based mainly on clinical and laboratory (antibodies and enterobiopsy) signs, which are in some cases problematic and inconvenient. Materials and methods: In our study we have examined 250 patients with suspection or with proven celiac disease and we evaluated specific ultrasound small bowel changes in this group. In the next step, we chose 59 patients with laboratory proved celiac disease and we statistically compared ultrasound, other laboratory and clinical findings in different forms and stages of the disease. Results: Specific small bowel pathologies in patients with celiac disease (like changes of intestinal villi in different parts of small bowel, abnormal peristalsis and mesenterial lymphadenopathy) can be well visualized by ultrasound and in combination with clinical and laboratory signs ultrasound examination could have an important role in screening, determination of diagnosis and monitoring of patients with different forms of celiac disease.

  9. Small bowel ultrasound in patients with celiac disease

    International Nuclear Information System (INIS)

    Bartusek, D.; Valek, V.; Husty, J.; Uteseny, J.

    2007-01-01

    Objective: Celiac disease (CD) is a common, lifelong disease with small bowel malabsorption based on genetically conditioned gluten intolerance. The clinical manifestation could be very heterogeneous. The proof of celiac disease is now based mainly on clinical and laboratory (antibodies and enterobiopsy) signs, which are in some cases problematic and inconvenient. Materials and methods: In our study we have examined 250 patients with suspection or with proven celiac disease and we evaluated specific ultrasound small bowel changes in this group. In the next step, we chose 59 patients with laboratory proved celiac disease and we statistically compared ultrasound, other laboratory and clinical findings in different forms and stages of the disease. Results: Specific small bowel pathologies in patients with celiac disease (like changes of intestinal villi in different parts of small bowel, abnormal peristalsis and mesenterial lymphadenopathy) can be well visualized by ultrasound and in combination with clinical and laboratory signs ultrasound examination could have an important role in screening, determination of diagnosis and monitoring of patients with different forms of celiac disease

  10. Patients with inflammatory bowel disease have increased risk of autoimmune and inflammatory diseases

    DEFF Research Database (Denmark)

    Halling, Morten L; Kjeldsen, Jens; Knudsen, Torben

    2017-01-01

    were significantly increased (P celiac disease, type 1 diabetes (T1D), sarcoidosis, asthma, iridocyclitis, psoriasis, pyoderma gangrenosum, rheumatoid arthritis, and ankylosing spondylitis. Restricted to UC (P ...AIM: To investigate whether immune mediated diseases (IMD) are more frequent in patients with inflammatory bowel disease (IBD). METHODS: In this population based registry study, a total of 47325 patients with IBD were alive and registered in the Danish National Patient Registry on December 16, 2013....... Controls were randomly selected from the Danish Civil Registration System (CRS) and matched for sex, age, and municipality. We used ICD 10 codes to identify the diagnoses of the included patients. The IBD population was divided into three subgroups: Ulcerative colitis (UC), Crohn's disease (CD) and Both...

  11. Oral Anticoagulation in Patients With Liver Disease.

    Science.gov (United States)

    Qamar, Arman; Vaduganathan, Muthiah; Greenberger, Norton J; Giugliano, Robert P

    2018-05-15

    Patients with liver disease are at increased risks of both thrombotic and bleeding complications. Many have atrial fibrillation (AF) or venous thromboembolism (VTE) necessitating oral anticoagulant agents (OACs). Recent evidence has contradicted the assumption that patients with liver disease are "auto-anticoagulated" and thus protected from thrombotic events. Warfarin and non-vitamin K-antagonist OACs have been shown to reduce thrombotic events safely in patients with either AF or VTE. However, patients with liver disease have largely been excluded from trials of OACs. Because all currently approved OACs undergo metabolism in the liver, hepatic dysfunction may cause increased bleeding. Thus, the optimal anticoagulation strategy for patients with AF or VTE who have liver disease remains unclear. This review discusses pharmacokinetic and clinical studies evaluating the efficacy and safety of OACs in patients with liver disease and provides a practical, clinically oriented approach to the management of OAC therapy in this population. Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  12. Weber, Schutz e a busca de "sentido" na ação social: uma análise comparativa

    Directory of Open Access Journals (Sweden)

    Eliane Fioravante Garcez

    2014-07-01

    Full Text Available http://dx.doi.org/10.5007/1806-5023.2014v11n1p63   O artigo discorre sobre as teorias de Max Weber e de Alfred Schutz e a aproximação desses mundos teóricos com a fenomenologia do filósofo Edmund Husserl. No texto são explorados os conceitos de sentido, compreensão, ação, ação social, relação social dentre outros. São apresentados também a tipologia da ação e os dois níveis de análise sociológica (microssocial e macrossocial na concepção de Weber, para quem a ação é fruto da subjetividade. Já para Schutz, a ação é intersubjetiva, socializada e a compreensão está por detrás da ação, quer para o sentido que o ator dá a sua própria ação (subjetivo, quer para a ação de seu semelhante (intersubjetivo. Tendo em vista essas teorias, este trabalho apresenta os pontos divergentes e convergentes entre elas, mas acima de tudo, registra o quanto são complementares na busca de compreensão do sentido da ação social.  Além dos preceitos conceituais, o texto discute a construção e a aplicação do modelo “tipo ideal” (TI amparados numa concepção schutziana.

  13. Color Discrimination in Patients with Gaucher Disease and Parkinson Disease.

    Science.gov (United States)

    Simon-Tov, Shlomi; Dinur, Tama; Giladi, Nir; Bar-Shira, Anat; Zelis, Mayaan; Zimran, Ari; Elstein, Deborah

    2015-01-01

    Poor color discrimination among patients with Parkinson disease (PD) has long been recognized. It has been shown that carrying one or two mutations in the β-glucocerebrosidase gene (GBA) for the autosomal disease Gaucher disease (GD), as based initially on clinical evidence, is a genetic risk factor for early-onset PD. The purpose of this study was to assess color discrimination in patients with one or two GBA mutations relative to healthy controls to ascertain whether this function is affected when persons with GD or even one GBA mutation develop PD. The Farnsworth-Munsell 100 hue test (FMHT) was evaluated among patients with GD+PD compared to patients with GD only, obligate GBA carriers with and without PD, patients with PD only, and healthy controls. FMHT outcome include computer-generated TES (Total Error Score) and values recommended by Vingrys & King-Smith. Six groups of 10 persons were tested. Significant differences were seen for male GD+PD and for age in PD. The highest mean TES was in the PD only group, the lowest in the GD only group. There was a significant difference because of PD in groups with GD and GBA carriers. GD+PD means were between GD only and PD only mean scores. These findings confirm that PD impacts color discrimination, more in males with GD+PD but nonetheless, GD+PD patients (but not GBA carriers) had better scores than PD only patients.

  14. The Nun Study and Alzheimer's disease: Quality of vocation as a potential protective factor?

    Science.gov (United States)

    Keohane, Kieran; Balfe, Myles

    2017-01-01

    Data from the Nun Study, the most famous longitudinal epidemiology of Alzheimer's disease to date, are analyzed and reformulated in terms of Max Weber's discussion of the centrality of beruf - "calling" - fulfilling a life task in pursuit of a vocation. Qualitative differences in the Nuns' vocations are uncovered, which are correlated to their resilience to dementia. Extrapolating, we argue that Alzheimer's disease and the dementia reputed to it should be understood as a social pathology of contemporary civilization related to the loss of moral foundations from which coherent, meaningful life-projects can be conducted.

  15. Medications Used for Cognitive Enhancement in Patients With Schizophrenia, Bipolar Disorder, Alzheimer's Disease, and Parkinson's Disease.

    Science.gov (United States)

    Hsu, Wen-Yu; Lane, Hsien-Yuan; Lin, Chieh-Hsin

    2018-01-01

    Cognitive impairment, which frequently occurs in patients with schizophrenia, bipolar disorder, Alzheimer's disease, and Parkinson's disease, has a significant impact on the daily lives of both patients and their family. Furthermore, since the medications used for cognitive enhancement have limited efficacy, the issue of cognitive enhancement still remains a clinically unsolved challenge. We reviewed the clinical studies (published between 2007 and 2017) that focused on the efficacy of medications used for enhancing cognition in patients with schizophrenia, bipolar disorder, Alzheimer's disease, and Parkinson's disease. Acetylcholinesterase inhibitors and memantine are the standard treatments for Alzheimer's disease and Parkinson's disease. Some studies have reported selective cognitive improvement in patients with schizophrenia following galantamine treatment. Newer antipsychotics, including paliperidone, lurasidone, aripiprazole, ziprasidone, and BL-1020, have also been reported to exert cognitive benefits in patients with schizophrenia. Dopaminergic medications were found to improve language function in patients with Parkinson's disease. However, no beneficial effects on cognitive function were observed with dopamine agonists in patients with schizophrenia. The efficacies of nicotine and its receptor modulators in cognitive improvement remain controversial, with the majority of studies showing that varenicline significantly improved the cognitive function in schizophrenic patients. Several studies have reported that N -methyl-d-aspartate glutamate receptor (NMDAR) enhancers improved the cognitive function in patients with chronic schizophrenia. NMDAR enhancers might also have cognitive benefits in patients with Alzheimer's disease or Parkinson's disease. Raloxifene, a selective estrogen receptor modulator, has also been demonstrated to have beneficial effects on attention, processing speed, and memory in female patients with schizophrenia. Clinical trials with

  16. Somatostatin receptor scintigraphy in patients with cat-scratch disease

    International Nuclear Information System (INIS)

    Krause, R.; Schnedl, W.J.; Hoier, S.; Piswanger-Soelkner, C.; Lipp, R.W.; Daxboeck, F.; Reisinger, E.C.

    2006-01-01

    Aim: somatostatin receptor scintigraphy images various neoplastic, granulomatous, and auto-immun diseases. Cat-scratch disease in an infectious granulomatous disease usually affecting the lymphnodes. It is not known whether cat-scratch disease provides positive somatostatin receptor scintigrams. Patients, methods: twelve patients with lymphadenitis and suspected cat-scratch disease were investigated by immunofluorescence antibody testing and somatostatin receptor scintigraphy. Suppurated lymphnodes were extracted or drained and Bartonella henselae specific PCR was then performed. Results: eleven of 12 patients showed IgG antibodies against B. henselea. SRS showed positive scintigraphic results in 6 of 11 patients with CSD. B. henselae DNA was detected in tissue of lymphnodes from 4 of 5 patients with lymphnode extraction or lymphnode drainage. SRS demonstrated positive scintigrams in all patients with a positive PCR. In one patient with suspected CSD SRS was negative as well as antibody testing. Conclusion: somatostatin receptor scintigraphy correlated with positive Bartonella henselae specific PCR tests and positive Bartonella henselae specific antibody tests in patients with CSD. (orig.)

  17. Somatostatin receptor scintigraphy in patients with cat-scratch disease

    Energy Technology Data Exchange (ETDEWEB)

    Krause, R.; Schnedl, W.J.; Hoier, S. [Div. of Infectious Diseases, Dept. of Internal Medicine, Univ. Graz (Austria); Piswanger-Soelkner, C.; Lipp, R.W. [Div. of Nuclear Medicine, Dept. of Internal Medicine, Univ. Graz (Austria); Daxboeck, F. [Clinical Inst. for Hygiene and Medical Microbiology, Div. of Hospital Hygiene, Univ. of Vienna (Austria); Reisinger, E.C. [Div. of Infectious Diseases and Tropical Medicine, Dept. of Internal Medicine, Univ. Rostock (Germany)

    2006-07-01

    Aim: somatostatin receptor scintigraphy images various neoplastic, granulomatous, and auto-immun diseases. Cat-scratch disease in an infectious granulomatous disease usually affecting the lymphnodes. It is not known whether cat-scratch disease provides positive somatostatin receptor scintigrams. Patients, methods: twelve patients with lymphadenitis and suspected cat-scratch disease were investigated by immunofluorescence antibody testing and somatostatin receptor scintigraphy. Suppurated lymphnodes were extracted or drained and Bartonella henselae specific PCR was then performed. Results: eleven of 12 patients showed IgG antibodies against B. henselea. SRS showed positive scintigraphic results in 6 of 11 patients with CSD. B. henselae DNA was detected in tissue of lymphnodes from 4 of 5 patients with lymphnode extraction or lymphnode drainage. SRS demonstrated positive scintigrams in all patients with a positive PCR. In one patient with suspected CSD SRS was negative as well as antibody testing. Conclusion: somatostatin receptor scintigraphy correlated with positive Bartonella henselae specific PCR tests and positive Bartonella henselae specific antibody tests in patients with CSD. (orig.)

  18. Thrombophilic Risk Factors in Patients With Inflammatory Bowel Disease.

    Science.gov (United States)

    Yazici, Ayten; Senturk, Omer; Aygun, Cem; Celebi, Altay; Caglayan, Cigdem; Hulagu, Sadettin

    2010-06-01

    Inflammatory bowel disease (IBD) patients have an increased risk for thromboembolism. The aim of this study was to assess the presence of thrombophilic risk factors in IBD patients and to assess the associations of these factors with disease activity. Forty-eight patients with IBD (24 ulcerative colitis, 24 Crohn's disease) and 40 matched healthy control individuals were enrolled. In addition to routine biochemical analysis, fasting blood samples were studied for prothrombin time (PT), activated partial thromboplastin time (aPTT), fibrinogen, protein-C, protein-S, antithrombin III, factor VII, factor VIII, D-dimer, vitamin B 12 , folic acid and homocysteine. Levels of erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), fibrinogen, D-dimer and the number of platelets were significantly higher in patients with IBD. When compared to control group, in patients with Crohn's disease serum homocystein levels were significantly higher (p = 0.025) while serum folic acid levels were significantly lower (p homocystein and the number of platelets were found to be significantly higher in Crohn's disease patients who were in active period of the disease. Thrombophilic defects are multifactorial and might be frequently seen in IBD patients. They might contribute to thrombotic complications of this disease.

  19. Helping Patients Cope with Inflammatory Bowel Disease.

    Science.gov (United States)

    1984-01-01

    these strategies can be effective as long as the strategy leads to 1) containment of guilt, fear, anxiety, and grief, 2) generation of hope , 3...patients with a sense of hope and a feeling that the disease can be coped with. The most difficult aspect of living with inflammatory bowel disease is...Recovery (mastectomy patients) and the Ostomy Association. They consist of people with Inflammatory Bowel Disease. Members support one another by sharing

  20. [Disease management for chronic heart failure patient].

    Science.gov (United States)

    Bläuer, Cornelia; Pfister, Otmar; Bächtold, Christa; Junker, Therese; Spirig, Rebecca

    2011-02-01

    Patients with chronic heart failure (HF) are limited in their quality of life, have a poor prognosis and face frequent hospitalisations. Patient self-management was shown to improve quality of life, reduce rehospitalisations and costs in patients with chronic HF. Comprehensive disease management programmes are critical to foster patient self-management. The chronic care model developed by the WHO serves as the basis of such programmes. In order to develop self-management skills a needs orientated training concept is mandatory, as patients need both knowledge of the illness and the ability to use the information to make appropriate decisions according to their individual situation. Switzerland has no established system for the care of patients with chronic diseases in particular those with HF. For this reason a group of Swiss experts for HF designed a model for disease management for HF patients in Switzerland. Since 2009 the Swiss Heart Foundation offers an education programme based on this model. The aim of this programme is to offer education and support for practitioners, patients and families. An initial pilot evaluation of the program showed mixed acceptance by practitioners, whereas patient assessed the program as supportive and in line with their requirements.

  1. Heart disease in patients with pulmonary embolism.

    Science.gov (United States)

    Pesavento, Raffaele; Piovella, Chiara; Prandoni, Paolo

    2010-09-01

    Several heart diseases are promoters of left-side cardiac thrombosis and could lead to arterial embolism. The same mechanism may be responsible for right-side cardiac thrombosis and therefore be a direct source of pulmonary embolism. Yasuoka et al. showed a higher incidence of perfusion defects in lung scan in patients with spontaneous echocontrast in the right atrium than in those without it (40% and 7% respectively; P=0.006). We recently assessed the prevalence of heart diseases in 11.236 consecutive patients older than 60 years discharged from Venetian hospitals with a diagnosis of pulmonary embolism. We observed a higher prevalence of all-cause heart diseases (odds ratio 1.26; 95% confidence interval, 1.13-1.40) in patients with a diagnosis of pulmonary embolism alone (secondary or unprovoked) compared with those discharged with a diagnosis of pulmonary embolism associated with deep vein thrombosis, generating the hypothesis that some specific heart diseases in older patients could themselves be a possible source of pulmonary emboli. Further prospective studies are required to confirm these findings, which have the potential to open new horizons for the interpretation and management of venous thromboembolic disease.

  2. Summer sudden Na number density enhancements measured with the ALOMAR Weber Na Lidar

    Directory of Open Access Journals (Sweden)

    D. Heinrich

    2008-05-01

    Full Text Available We present summer Na-densities and atmospheric temperatures measured 80 to 110 km above the Arctic Lidar Observatory for Middle Atmosphere Research (ALOMAR. The Weber Na Lidar is part of ALOMAR, located at 69° N in Norway, 150 km north of the Arctic Circle. The sun does not set here during the summer months, and measurements require a narrowband Faraday Anomalous Dispersion Optical Filter (FADOF.

    We discuss an observed sudden enhancement in the Na number density around 22:00 UT on 1 to 2 June 2006. We compare this observation with previous summer measurements and find a frequent appearance of Na number density enhancements near local midnight. We describe the time of appearance, the altitude distribution, the duration and the strength of these enhancements and compare them to winter observations. We investigate possible formation mechanisms and, as others before, we find a strong link between these Na number density enhancements and sporadic E layers.

  3. Summer sudden Na number density enhancements measured with the ALOMAR Weber Na Lidar

    Directory of Open Access Journals (Sweden)

    D. Heinrich

    2008-05-01

    Full Text Available We present summer Na-densities and atmospheric temperatures measured 80 to 110 km above the Arctic Lidar Observatory for Middle Atmosphere Research (ALOMAR. The Weber Na Lidar is part of ALOMAR, located at 69° N in Norway, 150 km north of the Arctic Circle. The sun does not set here during the summer months, and measurements require a narrowband Faraday Anomalous Dispersion Optical Filter (FADOF. We discuss an observed sudden enhancement in the Na number density around 22:00 UT on 1 to 2 June 2006. We compare this observation with previous summer measurements and find a frequent appearance of Na number density enhancements near local midnight. We describe the time of appearance, the altitude distribution, the duration and the strength of these enhancements and compare them to winter observations. We investigate possible formation mechanisms and, as others before, we find a strong link between these Na number density enhancements and sporadic E layers.

  4. Celiac Disease Autoimmunity in Patients with Autoimmune Diabetes and Thyroid Disease among Chinese Population.

    Directory of Open Access Journals (Sweden)

    Zhiyuan Zhao

    Full Text Available The prevalence of celiac disease autoimmunity or tissue transglutaminase autoantibodies (TGA amongst patients with type 1 diabetes (T1D and autoimmune thyroid disease (AITD in the Chinese population remains unknown. This study examined the rate of celiac disease autoimmunity amongst patients with T1D and AITD in the Chinese population. The study included 178 patients with type 1 diabetes and 119 with AITD where 36 had both T1D and AITD, classified as autoimmune polyglandular syndrome type 3 variant (APS3v. The study also included 145 patients with type 2 diabetes (T2D, 97 patients with non-autoimmune thyroid disease (NAITD, and 102 healthy controls. Serum islet autoantibodies, thyroid autoantibodies and TGA were measured by radioimmunoassay. TGA positivity was found in 22% of patients with either type 1 diabetes or AITD, much higher than that in patients with T2D (3.4%; p< 0.0001 or NAITD (3.1%; P < 0.0001 or healthy controls (1%; p<0.0001. The patients with APS3v having both T1D and AITD were 36% positive for TGA, significantly higher than patients with T1D alone (p = 0.040 or with AITD alone (p = 0.017. T1D and AITD were found to have a 20% and 30% frequency of overlap respectively at diagnosis. In conclusion, TGA positivity was high in the Chinese population having existing T1D and/or AITD, and even higher when both diseases were present. Routine TGA screening in patients with T1D or AITD will be important to early identify celiac disease autoimmunity for better clinical care of patients.

  5. Perianal disease, small bowel disease, smoking, prior steroid or early azathioprine/biological therapy are predictors of disease behavior change in patients with Crohn's disease.

    Science.gov (United States)

    Lakatos, Peter Laszlo; Czegledi, Zsofia; Szamosi, Tamas; Banai, Janos; David, Gyula; Zsigmond, Ferenc; Pandur, Tunde; Erdelyi, Zsuzsanna; Gemela, Orsolya; Papp, Janos; Lakatos, Laszlo

    2009-07-28

    To assess the combined effect of disease phenotype, smoking and medical therapy [steroid, azathioprine (AZA), AZA/biological therapy] on the probability of disease behavior change in a Caucasian cohort of patients with Crohn's disease (CD). Three hundred and forty well-characterized, unrelated, consecutive CD patients were analyzed (M/F: 155/185, duration: 9.4 +/- 7.5 years) with a complete clinical follow-up. Medical records including disease phenotype according to the Montreal classification, extraintestinal manifestations, use of medications and surgical events were analyzed retrospectively. Patients were interviewed on their smoking habits at the time of diagnosis and during the regular follow-up visits. A change in disease behavior was observed in 30.8% of patients with an initially non-stricturing, non-penetrating disease behavior after a mean disease duration of 9.0 +/- 7.2 years. In a logistic regression analysis corrected for disease duration, perianal disease, smoking, steroid use, early AZA or AZA/biological therapy use were independent predictors of disease behavior change. In a subsequent Kaplan-Meier survival analysis and a proportional Cox regression analysis, disease location (P = 0.001), presence of perianal disease (P < 0.001), prior steroid use (P = 0.006), early AZA (P = 0.005) or AZA/biological therapy (P = 0.002), or smoking (P = 0.032) were independent predictors of disease behavior change. Our data suggest that perianal disease, small bowel disease, smoking, prior steroid use, early AZA or AZA/biological therapy are all predictors of disease behavior change in CD patients.

  6. Novel combined management approaches to patients with diabetes, chronic kidney disease and cardiovascular disease.

    Science.gov (United States)

    Spaak, J

    2017-03-01

    Most patients we care for today suffer from more than one chronic disease, and multimorbidity is a rapidly growing challenge. Concomitant cardiovascular disease, renal dysfunction and diabetes represent a large proportion of all patients in cardiology, nephrology and diabetology. These entities commonly overlap due to their negative effects on vascular function and an accelerated atherosclerosis progression. At the same time, a progressive subspecialisation has caused the cardiologist to treat 'only' the heart, nephrologists 'only' the kidneys and endocrinologists' 'only' diabetes. Studies and guidelines follow the same pattern. This often requires patients to visit specialists for each field, with a risk of both under-diagnosis and under-treatment. From the patient's perspective, there is a great need for coordination and facilitation of the care, not only to reduce disease progression but also to improve quality of life. Person-centred integrated clinics for patients with cardiovascular disease, renal dysfunction and diabetes are a promising approach for complex chronic disease management.

  7. On new applications of fractional calculus

    Directory of Open Access Journals (Sweden)

    Shilpi Jain

    2019-07-01

    Full Text Available In the present paper author derive a number of integrals concerning various special functions which are applications of the one of Osler result. Osler provided extensions to the familiar Leibniz rule for the nth derivative of product of two functions.

  8. Patient and Disease Characteristics Associated with Activation for Self-Management in Patients with Diabetes, Chronic Obstructive Pulmonary Disease, Chronic Heart Failure and Chronic Renal Disease: A Cross-Sectional Survey Study

    Science.gov (United States)

    Bos-Touwen, Irene; Schuurmans, Marieke; Monninkhof, Evelyn M.; Korpershoek, Yvonne; Spruit-Bentvelzen, Lotte; Ertugrul-van der Graaf, Inge; de Wit, Niek; Trappenburg, Jaap

    2015-01-01

    A substantial proportion of chronic disease patients do not respond to self-management interventions, which suggests that one size interventions do not fit all, demanding more tailored interventions. To compose more individualized strategies, we aim to increase our understanding of characteristics associated with patient activation for self-management and to evaluate whether these are disease-transcending. A cross-sectional survey study was conducted in primary and secondary care in patients with type-2 Diabetes Mellitus (DM-II), Chronic Obstructive Pulmonary Disease (COPD), Chronic Heart Failure (CHF) and Chronic Renal Disease (CRD). Using multiple linear regression analysis, we analyzed associations between self-management activation (13-item Patient Activation Measure; PAM-13) and a wide range of socio-demographic, clinical, and psychosocial determinants. Furthermore, we assessed whether the associations between the determinants and the PAM were disease-transcending by testing whether disease was an effect modifier. In addition, we identified determinants associated with low activation for self-management using logistic regression analysis. We included 1154 patients (53% response rate); 422 DM-II patients, 290 COPD patients, 223 HF patients and 219 CRD patients. Mean age was 69.6±10.9. Multiple linear regression analysis revealed 9 explanatory determinants of activation for self-management: age, BMI, educational level, financial distress, physical health status, depression, illness perception, social support and underlying disease, explaining a variance of 16.3%. All associations, except for social support, were disease transcending. This study explored factors associated with varying levels of activation for self-management. These results are a first step in supporting clinicians and researchers to identify subpopulations of chronic disease patients less likely to be engaged in self-management. Increased scientific efforts are needed to explain the greater

  9. Patient and disease characteristics associated with activation for self-management in patients with diabetes, chronic obstructive pulmonary disease, chronic heart failure and chronic renal disease: a cross-sectional survey study.

    Science.gov (United States)

    Bos-Touwen, Irene; Schuurmans, Marieke; Monninkhof, Evelyn M; Korpershoek, Yvonne; Spruit-Bentvelzen, Lotte; Ertugrul-van der Graaf, Inge; de Wit, Niek; Trappenburg, Jaap

    2015-01-01

    A substantial proportion of chronic disease patients do not respond to self-management interventions, which suggests that one size interventions do not fit all, demanding more tailored interventions. To compose more individualized strategies, we aim to increase our understanding of characteristics associated with patient activation for self-management and to evaluate whether these are disease-transcending. A cross-sectional survey study was conducted in primary and secondary care in patients with type-2 Diabetes Mellitus (DM-II), Chronic Obstructive Pulmonary Disease (COPD), Chronic Heart Failure (CHF) and Chronic Renal Disease (CRD). Using multiple linear regression analysis, we analyzed associations between self-management activation (13-item Patient Activation Measure; PAM-13) and a wide range of socio-demographic, clinical, and psychosocial determinants. Furthermore, we assessed whether the associations between the determinants and the PAM were disease-transcending by testing whether disease was an effect modifier. In addition, we identified determinants associated with low activation for self-management using logistic regression analysis. We included 1154 patients (53% response rate); 422 DM-II patients, 290 COPD patients, 223 HF patients and 219 CRD patients. Mean age was 69.6±10.9. Multiple linear regression analysis revealed 9 explanatory determinants of activation for self-management: age, BMI, educational level, financial distress, physical health status, depression, illness perception, social support and underlying disease, explaining a variance of 16.3%. All associations, except for social support, were disease transcending. This study explored factors associated with varying levels of activation for self-management. These results are a first step in supporting clinicians and researchers to identify subpopulations of chronic disease patients less likely to be engaged in self-management. Increased scientific efforts are needed to explain the greater

  10. Patient and disease characteristics associated with activation for self-management in patients with diabetes, chronic obstructive pulmonary disease, chronic heart failure and chronic renal disease: a cross-sectional survey study.

    Directory of Open Access Journals (Sweden)

    Irene Bos-Touwen

    Full Text Available A substantial proportion of chronic disease patients do not respond to self-management interventions, which suggests that one size interventions do not fit all, demanding more tailored interventions. To compose more individualized strategies, we aim to increase our understanding of characteristics associated with patient activation for self-management and to evaluate whether these are disease-transcending. A cross-sectional survey study was conducted in primary and secondary care in patients with type-2 Diabetes Mellitus (DM-II, Chronic Obstructive Pulmonary Disease (COPD, Chronic Heart Failure (CHF and Chronic Renal Disease (CRD. Using multiple linear regression analysis, we analyzed associations between self-management activation (13-item Patient Activation Measure; PAM-13 and a wide range of socio-demographic, clinical, and psychosocial determinants. Furthermore, we assessed whether the associations between the determinants and the PAM were disease-transcending by testing whether disease was an effect modifier. In addition, we identified determinants associated with low activation for self-management using logistic regression analysis. We included 1154 patients (53% response rate; 422 DM-II patients, 290 COPD patients, 223 HF patients and 219 CRD patients. Mean age was 69.6±10.9. Multiple linear regression analysis revealed 9 explanatory determinants of activation for self-management: age, BMI, educational level, financial distress, physical health status, depression, illness perception, social support and underlying disease, explaining a variance of 16.3%. All associations, except for social support, were disease transcending. This study explored factors associated with varying levels of activation for self-management. These results are a first step in supporting clinicians and researchers to identify subpopulations of chronic disease patients less likely to be engaged in self-management. Increased scientific efforts are needed to explain

  11. [Anesthesia for patients with neurological diseases].

    Science.gov (United States)

    Kimura, Masafumi; Saito, Shigeru

    2010-09-01

    Several surgical treatments can be employed for the patients with neurological disorders, such as multiple sclerosis, Guillain-Barré syndrome, Parkinson's disease, amyotrophic lateral sclerosis, Alzheimer disease and spinal cord injury. It is possible that anesthesia related complications are induced in these neurologically complicated patients in the perioperative period. Respiratory dysfunction and autonomic nervous system dysfunction are most common in this population. Respiratory muscle weakness and bulbar palsy may cause aspiration pneumonia. Sometimes, postoperative ventilatory support is mandatory in these patients. Autonomic nervous system dysfunction may cause hypotension secondary to postural changes, blood loss, or positive airway pressure. Some therapeutic agents prescribed for neurological symptoms have drug interaction with anesthetic agents. Patients with motor neuron disease should be considered to be vulnerable to hyperkalemia in response to a depolarizing muscle relaxant. Although perioperative treatment guideline for most neurologic disorders has not been reported to lessen perioperative morbidity, knowledge of the clinical features and the interaction of common anesthetics with the drug therapy is important in planning intraoperative and postoperative management.

  12. Prognosis of patients with rheumatic diseases admitted to intensive care.

    Science.gov (United States)

    Beil, M; Sviri, S; de la Guardia, V; Stav, I; Ben-Chetrit, E; van Heerden, P V

    2017-01-01

    Variable mortality rates have been reported for patients with rheumatic diseases admitted to an intensive care unit (ICU). Due to the absence of appropriate control groups in previous studies, it is not known whether the presence of a rheumatic disease constitutes a risk factor. Moreover, the accuracy of the Acute Physiology and Chronic Health Evaluation II (APACHE II) score for predicting outcome in this group of patients has been questioned. The primary goal of this study was to compare outcome of patients with rheumatic diseases admitted to a medical ICU to those of controls. The records of all patients admitted between 1 April 2003 and 30 June 2014 (n=4020) were screened for the presence of a rheumatic disease during admission (n=138). The diagnosis of a rheumatic disease was by standard criteria for these conditions. An age- and gender-matched control group of patients without a rheumatic disease was extracted from the patient population in the database during the same period (n=831). Mortality in ICU, in hospital and after 180 days did not differ significantly between patients with and without rheumatic diseases. There was no difference in the performance of the APACHE II score for predicting outcome in patients with rheumatic diseases and controls. This score, as well as a requirement for the use of inotropes or vasopressors, accurately predicted hospital mortality in the group of patients with rheumatic diseases. In conclusion, patients with a rheumatic condition admitted to intensive care do not do significantly worse than patients without such a disease.

  13. Epiretinal membrane removal in patients with Stargardt disease

    Directory of Open Access Journals (Sweden)

    Muna Bhende

    2015-01-01

    Full Text Available Epiretinal membranes (ERMs in Stargardt disease have been known to undergo spontaneous separation in children. Results of surgical intervention in adult patients with Stargardt disease have rarely been reported. A retrospective review of results of surgical intervention for ERM causing visual impairment in two adult patients of Stargardt disease was carried out. Both patients developed ERM in one eye during their follow-up period with the resultant drop in their preexisting visual acuity. Postsurgery, restoration of foveal contour with some improvement in visual acuity was observed in both patients. No adverse effect of surgery was noted.

  14. Distal tibiofibular synostosis after ankle fracture. A 14-year follow-up study

    NARCIS (Netherlands)

    Albers, G. H.; de Kort, A. F.; Middendorf, P. R.; van Dijk, C. N.

    1996-01-01

    Over an eight-year period up to 1983, a total of 322 consecutive patients had operations for ankle fractures; 176 were Weber type B and 128 type C. We were able to review 230 of these patients after a mean follow-up of six years (1 to 11) including 128 with Weber B and 102 with Weber C fractures. We

  15. Suicide and patients with neurologic diseases. Methodologic problems

    DEFF Research Database (Denmark)

    Stenager, E N; Stenager, Egon

    1992-01-01

    OBJECTIVE: The suicide risk in patients with many neurologic diseases has been reported to be greater than that in the general population. Studies on the subject are, however, often encumbered with methodologic problems. We appraised these problems and, based on an evaluation, reappraised knowledge...... of the suicide risk in patients with specific neurologic diseases. DATA SOURCE: Using the computerized database MEDLINE, we identified all published reports with the key words suicide, attempted suicide, and neurologic diseases. STUDY SELECTION: We assessed and reviewed studies concerning the most common...... of the studies, the methods used gave rise to uncertainty about the conclusion presented. CONCLUSION: An increased suicide risk was found in patients suffering from multiple sclerosis and spinal cord lesions as well as in selected groups of patients with epilepsy. In other neurologic diseases, the suicide risk...

  16. The Prevalence of Fabry Disease in Patients with Chronic Kidney Disease in Turkey: The TURKFAB Study

    Directory of Open Access Journals (Sweden)

    Kultigin Turkmen

    2016-12-01

    Full Text Available Background/Aims: Fabry disease is a treatable cause of chronic kidney disease (CKD characterized by a genetic deficiency of α-galactosidase A. European Renal Best Practice (ERBP recommends screening for Fabry disease in CKD patients. However, this is based on expert opinion and there are no reports of the prevalence of Fabry disease in stage 1-5 CKD. Hence, we investigated the prevalence of Fabry disease in CKD patients not receiving renal replacement therapy. Methods: This prospective study assessed α-galactosidase activity in dried blood spots in 313 stage 1-5 CKD patients, 167 males, between ages of 18-70 years whose etiology of CKD was unknown and were not receiving renal replacement therapy. The diagnosis was confirmed by GLA gene mutation analysis. Results: Three (all males of 313 CKD patients (0.95% were diagnosed of Fabry disease, for a prevalence in males of 1.80%. Family screening identified 8 aditional Fabry patients with CKD. Of a total of 11 Fabry patients, 7 were male and started enzyme replacement therapy and 4 were female. The most frequent manifestations in male patients were fatigue (100%, tinnitus, vertigo, acroparesthesia, hypohidrosis, cornea verticillata and angiokeratoma (all 85%, heat intolerance (71%, and abdominal pain (57%. The most frequent manifestations in female patients were fatigue and cornea verticillata (50%, and tinnitus, vertigo and angiokeratoma (25%. Three patients had severe episodic abdominal pain attacks and proteinuria, and were misdiagnosed as familial Mediterranean fever. Conclusions: The prevalence of Fabry disease in selected CKD patients is in the range found among renal replacement therapy patients, but the disease is diagnosed at an earlier, treatable stage. These data support the ERBP recommendation to screen for Fabry disease in patients with CKD of unknown origin.

  17. Learning disabilities in Darier's disease patients.

    Science.gov (United States)

    Dodiuk-Gad, R; Lerner, M; Breznitz, Z; Cohen-Barak, E; Ziv, M; Shani-Adir, A; Amichai, B; Zlotogorski, A; Shalev, S; Rozenman, D

    2014-03-01

    Neuropsychiatric features and intellectual difficulties have been reported in studies of Darier's disease. Learning disabilities have never been reported or evaluated systematically in these patients. To assess the prevalence of learning disabilities in 76 patients with Darier's disease, and cognitive functioning in 19 of them. The data were collected by two methods: a questionnaire, as part of a larger study on the clinical characteristics of 76 patients; and neuropsychological measures for the assessment of learning disabilities in 19 of them. Thirty-one of the 76 patients reported learning disabilities (41%) and 56 (74%) reported a family history of learning disabilities. Significant differences were found between the 19 patients evaluated on cognitive tasks and a control group of 42 skilled learners on subtraction and multiplication tasks. Six (32%) of the 19 were identified as having reading difficulties and five (26%) exhibited low performance on the Concentration Performance Test. All patients had general cognitive ability in the average range. Findings suggest an association between Darier's disease and learning disabilities, a heretofore unreported association, pointing to the need to obtain personal and family history of such disabilities in order to refer cases of clinical concern for further study. © 2013 The Authors Journal of the European Academy of Dermatology and Venereology © 2013 European Academy of Dermatology and Venereology.

  18. Fibromyalgia in patients with other rheumatic diseases: prevalence and relationship with disease activity.

    Science.gov (United States)

    Haliloglu, Sema; Carlioglu, Ayse; Akdeniz, Derya; Karaaslan, Yasar; Kosar, Ali

    2014-09-01

    Fibromyalgia (FM) is a syndrome characterized by chronic widespread pain and the presence of specific tender points. The prevalence of FM has been estimated at 2-7 % of the general global population. The presence of FM in several rheumatic diseases with a structural pathology has been reported as 11-30 %. The objectives of this study were to determine the prevalence of FM and to evaluate the possible relationship between FM existence and disease activity among rheumatic diseases. The study group included 835 patients--197 rheumatoid arthritis (RA), 67 systemic lupus erythematosus (SLE), 119 ankylosing spondylitis (AS), 238 osteoarthritis (OA), 14 familial Mediterranean fever (FMF), 53 Behçet's disease (BD), 71 gout, 25 Sjögren's syndrome (SS), 20 vasculitis, 29 polymyalgia rheumatica (PMR), and two polymyositis (PM)--with or without FM. Recorded information included age, gender, laboratory parameters, presence of fatigue, and disease activity indexes. The prevalence of FM in patients with rheumatologic diseases was found to be 6.6 % for RA, 13.4 % for SLE, 12.6 % for AS, 10.1 % for OA, 5.7 % for BD, 7.1 % for FMF, 12 % for SS, 25 % for vasculitis, 1.4 % for gout, and 6.9 % for PMR. One out of two patients with PM was diagnosed with FM. Some rheumatologic cases (AS, OA) with FM were observed mostly in female patients (p = 0.000). Also, there were significant correlations between disease activity indexes and Fibromyalgia Impact Questionnaire scores for most rheumatologic patients (RA, AS, OA, and BD) (p diseases, and its recognition is important for the optimal management of these diseases. Increased pain, physical limitations, and fatigue may be interpreted as increased activity of these diseases, and a common treatment option is the prescription of higher doses of biologic agents or corticosteroids. Considerations of the FM component in the management of rheumatologic diseases increase the likelihood of the success of the treatment.

  19. Nonmotor symptoms in patients suffering from motor neuron diseases

    Directory of Open Access Journals (Sweden)

    Rene Günther

    2016-07-01

    Full Text Available Background: The recently postulated disease spreading hypothesis has gained much attention, especially for Parkinson’s disease (PD. The various nonmotor symptoms (NMS in neurodegenerative diseases would be much better explained by this hypothesis than by the degeneration of disease-specific cell populations. Motor neuron disease (MND is primarily known as a group of diseases with a selective loss of motor function. Recent evidence, however, suggests disease spreading into nonmotor brain regions also in MND. The aim of this study was to comprehensively detect NMS in patients suffering from MND.Methods: We used a self-rating questionnaire including 30 different items of gastrointestinal, autonomic, neuropsychiatric and sleep complaints (NMSQuest which is an established tool in PD patients. 90 MND patients were included and compared to 96 controls.Results: In total, MND patients reported significantly higher NMS scores (median: 7 points in comparison to controls (median: 4 points. Dribbling, impaired taste/smelling, impaired swallowing, weight loss, loss of interest, sad/blues, falling and insomnia were significantly more prevalent in MND patients compared to controls. Interestingly excessive sweating was more reported in the MND group. Correlation analysis revealed an increase of total NMS score with disease progression.Conclusions: NMS in MND patients seemed to increase with disease progression which would fit with the recently postulated disease spreading hypothesis. The total NMS score in the MND group significantly exceeded the score for the control group, but only 8 of the 30 single complaints of the NMSQuest were significantly more often reported by MND patients. Dribbling, impaired swallowing, weight loss and falling could primarily be connected to motor neuron degeneration and declared as motor symptoms in MND.

  20. Roland Weber first part

    DEFF Research Database (Denmark)

    2016-01-01

    The Sooty bloch complex - species identity, infection biology and control options. Rubbery rot and Black summer rot - two new diseases of apple......The Sooty bloch complex - species identity, infection biology and control options. Rubbery rot and Black summer rot - two new diseases of apple...

  1. Prevalence of celiac disease in siblings of Iranian patients with celiac disease.

    Science.gov (United States)

    Chomeili, Bashir; Aminzadeh, Majid; Hardani, Amir Kamal; Fathizadeh, Payam; Chomeili, Pooya; Azaran, Azarakhsh

    2011-01-01

    Celiac disease, one of the best-known autoimmune human leukocyte antigen-dependent disorders, has a relatively increased prevalence in first-degree relatives. To determine the prevalence of celiac disease in siblings of patients with confirmed celiac disease. Siblings of confirmed celiac disease patients in our center were identified and enrolled in this study. Their serum immunoglobulin A and tissue transglutaminase antibody-enzyme-linked immunosorbent assay (anti-tissue transglutaminase, immunoglobulin A, and immunoglobulin G) were measured and multiple endoscopic duodenal biopsy specimens were obtained with parental consensus. Celiac disease was confirmed by observation of characteristic histological changes. A total of 49 children (male, 29; female, 20; age, 2-16 years) with confirmed celiac disease in a pediatric gastroenterology ward were studied from 1999 to 2006. We found 30 siblings (female, 16) all shared in both parents. The only measurement available was for immunoglobulin A tissue transglutaminase antibody. A duodenal biopsy was performed in all 30 siblings. Clinical findings such as abdominal pain, fatigue, growth retardation and diarrhea were found in 53.3% of the completely studied siblings, and positive serology without histological changes was identified in four cases. Both serology and biopsy (confirmed new cases) were positive in 2 of the 30 siblings. High prevalence of celiac disease among siblings of patients with confirmed celiac disease necessitates serologic screening (and confirmatory biopsy if indicated) in families having celiac disease. It is advantageous to diagnose the disease as soon as possible because early diagnosis and diet intervention may prevent serious complications such as growth retardation, short stature, chronic diarrhea, and malignancy.

  2. La presencia de Max Weber en el pensamiento español. Historia de una doble recepción

    Directory of Open Access Journals (Sweden)

    Ruano de la Fuente, María Yolanda

    2007-08-01

    Full Text Available This paper examines two diverse and confronted receptions of Max Weber’s thought in the Hispanic world which we have described as “conservative” and “liberal” lines of interpretation. This analytical scheme allows us to group and highlight some of the most distinguished Spanish intellectuals whose work was influenced by Max Weber’s thought. We will review the context and the particular mode in which these intellectuals assimilated the German author’s work, the meaning of their interpretations and their labour of translation. In short, we will focus on the works of some renowned Hispanic philosophers, jurists and sociologists belonging to the “first interpretative generation” at work from the 1920’s to the 1940’s.Este artículo examina una doble y contrapuesta línea de recepción del pensamiento de Max Weber en el mundo hispanoparlante, que hemos denominado línea “conservadora” y línea “liberal”. Este esquema interpretativo permite agrupar y reseñar a algunos de los más destacados intelectuales españoles, cuyas obras estuvieron marcadas por la influencia del pensamiento de Max Weber. Revisamos el contexto, el sentido de la recepción, la labor de traducción y el modo peculiar en que asimilaron el trabajo del autor alemán. Nos centramos, en suma, en la obra de algunos renombrados filósofos, juristas y sociólogos de habla hispana, si bien limitamos nuestro análisis a la “primera generación receptora”, que fechamos en las décadas de los años 20 a los 40 del siglo XX.

  3. Enhanced thyroid iodine metabolism in patients with triiodothyronine-predominant Graves' disease

    International Nuclear Information System (INIS)

    Takamatsu, J.; Hosoya, T.; Naito, N.

    1988-01-01

    Some patients with hyperthyroid Graves' disease have increased serum T3 and normal or even low serum T4 levels during treatment with antithyroid drugs. These patients with elevated serum T3 to T4 ratios rarely have a remission of their hyperthyroidism. The aim of this study was to investigate thyroid iodine metabolism in such patients, whom we termed T3-predominant Graves' disease. Mean thyroid radioactive iodine uptake was 51.0 +/- 18.1% ( +/- SD) at 3 h, and it decreased to 38.9 +/- 20.1% at 24 h in 31 patients with T3-predominant Graves' disease during treatment. It was 20.0 +/- 11.4% at 3 h and increased to 31.9 +/- 16.0% at 24 h in 17 other patients with hyperthyroid Graves' disease who had normal serum T3 and T4 levels and a normal serum T3 to T4 ratio during treatment (control Graves' disease). The activity of serum TSH receptor antibodies was significantly higher in the patients with T3-predominant Graves' disease than in control Graves' disease patients. From in vitro studies of thyroid tissue obtained at surgery, both thyroglobulin content and iodine content in thyroglobulin were significantly lower in patients with T3-predominant Graves' disease than in the control Graves' disease patients. Thyroid peroxidase (TPO) activity determined by a guaiacol assay was 0.411 +/- 0.212 g.u./mg protein in the T3-predominant Graves' disease patients, significantly higher than that in the control Graves' disease patients. Serum TPO autoantibody levels determined by immunoprecipitation also were greater in T3-predominant Graves' disease patients than in control Graves' disease patients. Binding of this antibody to TPO slightly inhibited the enzyme activity of TPO, but this effect of the antibody was similar in the two groups of patients

  4. Using electronic patient records to discover disease correlations and stratify patient cohorts.

    Directory of Open Access Journals (Sweden)

    Francisco S Roque

    2011-08-01

    Full Text Available Electronic patient records remain a rather unexplored, but potentially rich data source for discovering correlations between diseases. We describe a general approach for gathering phenotypic descriptions of patients from medical records in a systematic and non-cohort dependent manner. By extracting phenotype information from the free-text in such records we demonstrate that we can extend the information contained in the structured record data, and use it for producing fine-grained patient stratification and disease co-occurrence statistics. The approach uses a dictionary based on the International Classification of Disease ontology and is therefore in principle language independent. As a use case we show how records from a Danish psychiatric hospital lead to the identification of disease correlations, which subsequently can be mapped to systems biology frameworks.

  5. Kleptomania in Patients with Neuro-Behçet's Disease

    OpenAIRE

    Shugaiv, Erkingül; Kıyat-Atamer, Aslı; Tüzün, Erdem; Kürtüncü, Murat; Baral-Kulaksızoğlu, Işın; Akman Demir, Gülşen

    2013-01-01

    Objective This study was conducted to characterize the prevalence and clinical features of kleptomania, an impulse control disorder, in patients with Behçet's disease involving the central nervous system. Subjects and Methods Medical records of 350 patients with neuro-Behçet's disease were evaluated, and clinical and neuropsychological features of patients with kleptomania were noted. Results Of the 350 neuro-Behçet's disease patients 6 (1.7%) had presented with symptoms that fulfilled the cr...

  6. Sexual Health Concerns in Patients with Cardiovascular Disease

    Science.gov (United States)

    ... Patient Page Sexual Health Concerns in Patients With Cardiovascular Disease Lindsey Rosman , John M. Cahill , Susan L. McCammon , ... and difficulty achieving and maintaining an erection. 2 Cardiovascular disease and its treatment may also affect a man’s ...

  7. Electrocardiographic Characteristics of Patients with Chronic Obstructive Pulmonary Disease

    NARCIS (Netherlands)

    Warnier, M.J.; Rutten, F.H.; Numans, M.E.; Kors, J.A.; Tan, H.L.; de Boer, A.; Hoes, A.W.; de Bruin, M.L.

    2013-01-01

    Patients with chronic obstructive pulmonary disease (COPD) are at increased risk of cardiovascular disease. Electrocardiography (ECG) carries information about cardiac disease and prognosis, but studies comparing ECG characteristics between patients with and without COPD are lacking. We related ECG

  8. [DIET CHARACTERISTICS IN PATIENTS WITH CHRONIC KIDNEY DISEASE].

    Science.gov (United States)

    Bašić-Marković, N; Šutić, I; Popović, B; Marković, R; Vučak, J

    2016-12-01

    Because of the increasing number of patients, chronic kidney disease (CKD) has become a significant public health problem. As kidney function decreases, it is necessary to introduce certain dietary modifications. The aim was to investigate what is the appropriate approach to diet of CKD patients, which could contribute to slowing down progression of the disease. Dietary recommendations are individual for each patient, but also vary in the same patient depending on the stage of disease progression because special attention must be paid to appropriate intake of macronutrients (protein, carbohydrates and fats), micronutrients (sodium, potassium, calcium, phosphorus, zinc, selenium, various vitamins), and water. In newly diagnosed patients, it is necessary to assess their nutritional status and energy requirements. It has been shown that protein-energy malnutrition, muscle loss and cachexia are strong predictors of mortality in CKD. Comparing different dietary approaches in everyday life of patients suffering from CKD, it was found that the most effective diet is Mediterranean food style. Studies confirm that Mediterranean diet has a preventive effect on renal function and reduces progression of the disease. Preventive measures, correct identification and early intervention can increase survival of patients and improve their quality of life. Mediterranean diet tailored to individual stages of CKD has been confirmed as the best choice in CKD patients.

  9. Sexually transmitted diseases among psychiatric patients in Brazil.

    Science.gov (United States)

    Dutra, Maria Rita Teixeira; Campos, Lorenza Nogueira; Guimarães, Mark Drew Crosland

    2014-01-01

    Sexually transmitted diseases are still highly prevalent worldwide and represent an important public health problem. Psychiatric patients are at increased risk of sexually transmitted diseases but there are scarce published studies with representative data of this population. We sought to estimate the prevalence and correlates of self-reported sexually transmitted diseases among patients with mental illnesses under care in a national representative sample in Brazil (n=2145). More than one quarter of the sample (25.8%) reported a lifetime history of sexually transmitted disease. Multivariate analyses showed that patients with a lifetime sexually transmitted disease history were older, had history of homelessness, used more alcohol and illicit drugs, suffered violence, perceived themselves to be at greater risk for HIV and had high risk sexual behavioral: practised unprotected sex, started sexual life earlier, had more than ten sexual partners, exchanged money and/or drugs for sex and had a partner that refused to use condom. Our findings indicate a high prevalence of self-reported sexually transmitted diseases among psychiatric patients in Brazil, and emphasize the need for implementing sexually transmitted diseases prevention programs in psychiatric settings, including screening, treatment, and behavioral modification interventions. Copyright © 2013 Elsevier Editora Ltda. All rights reserved.

  10. [Consumer surveys among hospitalized patients with lung disease].

    Science.gov (United States)

    Humborstad, O T; Omenaas, E; Gulsvik, A

    2001-03-30

    The aim of our survey was to record the experiences of hospitalised patients with respiratory diseases in order to create a more patient-friendly department. Our study included 609 patients (response rate 70%) with a median age of 64 years (range 13-91) who were discharged from the Department of Thoracic Medicine, Haukeland University Hospital in October 1991, 1992, 1994, 1995 and 1996. 268 patients had obstructive lung disease, 82 had lung cancer. They answered a questionnaire with 24 questions. Patient reception to the ward and staff knowledge of the patients' illnesses, were for the physicians rated as good or better by 92% and 79% and for the nurses by 94% and 70% respectively. 16% of the patients experienced insecurity, 17% anxiety, 12% helplessness, 9% loneliness and 12% little say in the decision making process. Trend factors for these emotional experiences were female sex, old age, obstructive lung disease and long stay in hospital. Patients aged 50 to 69 years and patients with lung cancer had the lowest rate of negative emotional experiences. Despite staff awareness of the prevalence and of the patients' emotional experiences and the risk factors involved, there was no clear reduction of negative experiences in the later surveys compared to the first survey. Patients in a university hospital with respiratory diseases showed unchanged experiences of health care and personal emotions in repeated surveys over a period of five years.

  11. Assessment of jeopardized myocardium in patients with one-vessel disease

    International Nuclear Information System (INIS)

    Iskandrian, A.S.; Lichtenberg, R.; Segal, B.L.; Mintz, G.S.; Mundth, E.D.; Hakki, A.-H.; Kimbiris, D.; Bemis, C.E.; Croll, M.N.; Kane, S.A.

    1982-01-01

    The size of the perfusion defect was assessed from a quantitative analysis of exercise thallium-201 images. Quantitative analysis was determined by measuring the area and the perimeter of the perfusion defect and expressing it as a percentage of the total left ventricular area or perimeter in three projections. Using this technique, we studied 50 patients with one-vessel disease of 50% or greater diameter narrowing. The planimetric and the perimetric methods correlated well (p<0.001, r=0.97). Of the 11 patients with less than 70% diameter narrowing, only one patient had abnormal exercise thallium-201 images. Of the remaining 39 patients with 70% or greater diameter narrowing, 35 had abnormal exercise images. The defects were larger in patients with left anterior descending coronary artery disease (33 +/- 10%; mean +/- SD by perimetry) than in patients with left circumflex coronary artery disease (19 +/- 14%, p<0.01) or right coronary artery disease (17 +/- 11%, p<0.01). There was also significant variation in the sizes of the defects in the three projections in patients with left circumflex and right coronary artery disease, but not in patients with left anterior descending coronary artery disease. Patients with left anterior descending coronary artery disease with collaterals had smaller defects than their counterparts without collaterals

  12. Desencantamento do mundo e ética na ação pedagógica: reflexões a partir de Max Weber Disenchantment of the world and ethics in pedagogical action: reflections upon Max Weber

    Directory of Open Access Journals (Sweden)

    Alonso Bezerra de Carvalho

    2010-08-01

    Full Text Available Este artigo, de caráter teórico, apresenta ideias do pensamento weberiano, destacando aquelas que fornecem elementos para se pensar a educação na atualidade, sobretudo a partir de sua compreensão da modernidade. Desencantamento do mundo é o conceito-chave de sua sociologia, tomado como referência para refletir sobre o significado que a ciência e o professor podem ter hoje. Autor clássico, a interpretação que Max Weber faz do mundo moderno traz às nossas reflexões temas os mais diversos, influenciando sobremaneira o pensamento e a prática da cultura contemporânea, ao se preocupar em compreender as ações e os valores humanos. Ao estudar o processo de racionalização no Ocidente, provoca discussões na área da ciência, da ética e, correlativamente, na educação, possibilitando ampliarmos nosso entendimento sobre qual o sentido, o significado e o papel que devemos cumprir em relação ao conhecimento e ao ensino. O objetivo principal da educação é proporcionar aos alunos um conteúdo que incentive a liberdade de reflexão. Para isso, é preciso que o professor adote uma ética não partidária na sala de aula, apresentando um conteúdo que não exponha a sua opinião política e, se o fizer, que tenha a honestidade de dizer que o está fazendo. Assim, é de extrema importância que o professor mantenha uma posição "neutra" para que, não só na sala de aula, mas também fora dela, o aluno possa refletir e questionar o que observa, experimenta e decide. Somente assim a conduta do professor estaria condizente com o processo de racionalização de nossa cultura.This article has a theoretical character and presents some of Weber's ideas, with emphasis on those that can supply elements to reflect on the current state of education, more particularly based on his understanding of modernity. Disenchantment of the world is the key concept in his sociology, and is taken as the basis for a reflection about the meaning that science

  13. Transvascular lipoprotein transport in patients with chronic renal disease

    DEFF Research Database (Denmark)

    Jensen, Trine Krogsgaard; Nordestgaard, Børge Grønne; Feldt-Rasmussen, Bo

    2004-01-01

    BACKGROUND: While increased plasma cholesterol is a well-established cardiovascular risk factor in the general population, this is not so among patients with chronic renal disease. We hypothesized that the transvascular lipoprotein transport, in addition to the lipoprotein concentration in plasma......, determines the degree of atherosclerosis among patients with chronic renal disease. METHODS: We used an in vivo method for measurement of transvascular transport of low-density lipoprotein (LDL) in 21 patients with chronic renal disease and in 42 healthy control patients. Autologous 131-iodinated LDL...... was reinjected intravenously, and the 1-hour fractional escape rate was taken as index of transvascular transport. RESULTS: Transvascular LDL transport tended to be lower in patients with chronic renal disease than in healthy control patients [3.3 (95% CI 2.4-4.2) vs. 4.2 (3.7-4.2)%/hour; NS]. However...

  14. Heart valve disease among patients with hyperprolactinemia

    DEFF Research Database (Denmark)

    Steffensen, Maria Charlotte; Maegbaek, Merete Lund; Laurberg, Peter

    2012-01-01

    Increased risk of heart valve disease during treatment with certain dopamine agonists, such as cabergoline, has been observed in patients with Parkinson's disease. The same compound is used to treat hyperprolactinemia, but it is unknown whether this also associates with heart valve disease....

  15. Pre-operative evaluation of patients with chronic liver disease

    International Nuclear Information System (INIS)

    Tapias M Monica; Idrovo Cubides, Victor

    2006-01-01

    Patients with advanced liver disease have an increased risk of complications, compared to healthy patients when they undergo a surgical procedure. This risk is related to the type of surgery, to the type of anesthetic used, and to the severity of the underlying liver disease. Several risk factors for liver disease should be identified prior to a procedure. Those with advanced disease should undergo specific pre-surgical diagnostic tests. The Child Pugh score, and the MELD score, are very useful to establish the surgical risk in individuals with liver disease. The Child-Pugh score is a very useful tool that correlates closely to morbidity and mortality in patients with liver disease. Mortality rates in these patients undergoing major surgery is 10, 30 and 82% for Child-Pugh scores A, B and C respectively. In order to optimize the patient's condition before surgery, a complete evaluation and management of conditions such as jaundice, coagulopathy, ascites, electrolyte abnormalities, renal insufficiency and encephalopathy must be performed. This approach helps to reduce the complication rate in these individuals

  16. Search for Pompe disease among patients with undetermined myopathies.

    Science.gov (United States)

    Lindberg, C; Anderson, B; Engvall, M; Hult, M; Oldfors, A

    2015-07-20

    Pompe disease is a rare treatable glycogen storage disease with in adults - a limb-girdle muscle weakness. Muscle biopsy may fail to show the typical vacuolar myopathy. We asked if we had un-diagnosed patients with Pompe disease in western Sweden. We searched the muscle biopsy registry during the time period 1986 until 2006 including 3665 biopsies and included patients at our Neuromuscular Center with unspecified myopathy or limb-girdle muscular dystrophy. The dry blood spot test was used to identify patients with Pompe disease. A total of 82 patients (46 from the biopsy register and 36 from our center) were seen and dry blood spot test was obtained. No patient with Pompe disease was found. The dry blood spot test was low in three cases (11, 16, and 18% of normal) but a second blood sample showed a normal result based on GAA enzyme activity in lymphocytes in all three patients. In one patient with low normal result of the analysis in lymphocytes a genetic test showed no pathogenic mutations. Further investigation gave a definite diagnose of another myopathy in 12 patients. The prevalence of Pompe disease in western Sweden (3 in 1.27 million or 0.24 per 100.000 inhabitants) is lower than in the Netherlands and New York. Re-evaluation of patients with myopathies but without definite diagnosis is rewarding since 12 of 82 patients in our study had a definite molecular diagnosis after workup. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  17. Value of electrocardiogram in predialytic chronic kidney disease patient without known coronary artery disease

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    Dutta PK, Das S

    2014-11-01

    Full Text Available Chronic Kidney disease (CKD is a pressing public health burden occurring in about 10% of the population. The majority of them die before reaching End Stage Renal Disease (ESRD due to cardiovascular disease (CVD. Hypertension (HTN and anaemia are two reversible factors for progression of CKD. Besides asymptomatic coronary artery disease, the electrolyte abnormalities such as hyperkalaemia and hypocalcaemia also subject these patients to sudden cardiac death. This study is aimed at to see the changes in electrocardiogram (ECG in hospitalized predialytic CKD patients due to these abnormalities. Methods: This is a 6 months cross-sectional study carried out at Chittagong Medical College Hospital in Chittagong, Bangladesh. 50 patients with stages 3, 4 and 5 CKD were recruited from the Nephrology and Medicine wards. Patients with prior history of coronary artery disease, cardiomyopathy, valvular heart disease and dialysis were excluded. All had their standard 12–lead electrocardiogram (ECG recorded and various findings were critically studied and interpreted independently by two consultant physicians including a cardiologist. Data analysis was done using SPSS version 19. Results: LVH (left ventricular hypertrophy (66%, LAE (left atrial enlargement (30% and unrecognized myocardial infarction (28% were very common ECG abnormalities in our predialytic CKD patients. HTN, anaemia, late presentation, and male gender appear to be associated with ECG abnormalities. Though 28 patients (56% were hyperkalaemic only 9 patients (38% of them had tall tented T wave in ECG. Conclusion: Detection of HTN and anaemia in male predialytic CKD patients will arouse suspicion which will help in early detection of cardiac outcome by ECG abnormality which will help in taking treatment strategy in resource limited country.

  18. Cardiovascular disease in patients with end-stage renal disease on hemodialysis in a developing country

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    Leila S. V. Silva

    2012-01-01

    Full Text Available Cardiovascular disease is the main cause of death among patients with end-stage renal disease (ESRD. The present study was undertaken to identify the main cardiovascular diseases and their risk factors in 160 patients with ESRD on hemodialysis (HD in Brazil. Their mean age was 47 ± 39 years. The main risk factors for cardiovascular diseases were arterial hypertension (89.4%, dyslipidemia (78.3%, low high-density lipoprotein levels (84.2% and low physical activity (64.1%. Family history of coronary insufficiency and high low-density lipoprotein levels were significantly associated with coronary artery disease (P = 0.005 and P = 0.029, respectively. Sedentary life style, diabetes mellitus, secondary hyperparathyroidism and hyperglycemia also showed a significant association with the underlying vascular disease (P = 0.017, P = 0.039, P = 0.037 and P = 0.030, respectively. Hypercalcemia, hypertension and black race were factors significantly associated with left ventricular systolic dysfunction (P = 0.01, P = 0.0013 and P = 0.024, respectively. Our study shows that the most prevalent cardiovascular diseases in patients with ESRD were left ventricular hypertrophy, atherosclerotic disease, valvular disease and coronary artery disease. Hypertension and dyslipidemia were the common risk factors associated with cardiovascular diseases. The present study was undertaken to identify the main cardiovascular diseases and their risk factors in 160 patients with ESRD on HD in a single center in Brazil.

  19. Burn mortality in patients with preexisting cardiovascular disease.

    Science.gov (United States)

    Knowlin, Laquanda; Reid, Trista; Williams, Felicia; Cairns, Bruce; Charles, Anthony

    2017-08-01

    Burn shock, a complex process, which develops following burn leads to severe and unique derangement of cardiovascular function. Patients with preexisting comorbidities such as cardiovascular diseases may be more susceptible. We therefore sought to examine the impact of preexisting cardiovascular disease on burn outcomes. A retrospective analysis of patients admitted to a regional burn center from 2002 to 2012. Independent variables analyzed included basic demographics, burn mechanism, presence of inhalation injury, TBSA, pre-existing comorbidities, and length of ICU/hospital stay. Bivariate analysis was performed and Poisson regression modeling was utilized to estimate the incidence of being in the ICU and mortality. There were a total of 5332 adult patients admitted over the study period. 6% (n=428) had a preexisting cardiovascular disease. Cardiovascular disease patients had a higher mortality rate (16%) compared to those without cardiovascular disease (3%, pwill likely be a greater number of individuals at risk for worse outcomes following burn. This knowledge can help with burn prognostication. Copyright © 2017 Elsevier Ltd and ISBI. All rights reserved.

  20. Perianal disease, small bowel disease, smoking, prior steroid or early azathioprine/biological therapy are predictors of disease behavior change in patients with Crohn’s disease

    Science.gov (United States)

    Lakatos, Peter Laszlo; Czegledi, Zsofia; Szamosi, Tamas; Banai, Janos; David, Gyula; Zsigmond, Ferenc; Pandur, Tunde; Erdelyi, Zsuzsanna; Gemela, Orsolya; Papp, Janos; Lakatos, Laszlo

    2009-01-01

    AIM: To assess the combined effect of disease phenotype, smoking and medical therapy [steroid, azathioprine (AZA), AZA/biological therapy] on the probability of disease behavior change in a Caucasian cohort of patients with Crohn’s disease (CD). METHODS: Three hundred and forty well-characterized, unrelated, consecutive CD patients were analyzed (M/F: 155/185, duration: 9.4 ± 7.5 years) with a complete clinical follow-up. Medical records including disease phenotype according to the Montreal classification, extraintestinal manifestations, use of medications and surgical events were analyzed retrospectively. Patients were interviewed on their smoking habits at the time of diagnosis and during the regular follow-up visits. RESULTS: A change in disease behavior was observed in 30.8% of patients with an initially non-stricturing, non-penetrating disease behavior after a mean disease duration of 9.0 ± 7.2 years. In a logistic regression analysis corrected for disease duration, perianal disease, smoking, steroid use, early AZA or AZA/biological therapy use were independent predictors of disease behavior change. In a subsequent Kaplan-Meier survival analysis and a proportional Cox regression analysis, disease location (P = 0.001), presence of perianal disease (P < 0.001), prior steroid use (P = 0.006), early AZA (P = 0.005) or AZA/biological therapy (P = 0.002), or smoking (P = 0.032) were independent predictors of disease behavior change. CONCLUSION: Our data suggest that perianal disease, small bowel disease, smoking, prior steroid use, early AZA or AZA/biological therapy are all predictors of disease behavior change in CD patients. PMID:19630105

  1. Perioperative Management of Patients with Rheumatic Diseases

    Science.gov (United States)

    Bissar, Lina; Almoallim, Hani; Albazli, Khaled; Alotaibi, Manal; Alwafi, Samar

    2013-01-01

    This paper aims to explore the assessment of patients with rheumatologic diseases, especially rheumatoid arthritis (RA), before undergoing orthopedic surgery. Perioperative assessment ensures an early diagnosis of the patient's medical condition, overall health, medical co-morbidities, and the assessment of the risk factors associated with the proposed procedures. Perioperative assessment allows for proper postoperative management of complications and of the management of drugs such as disease-modifying anti-rheumatic drugs (DMARD) and anti-platelets, and corticosteroids. The assessment also supports follow up plans, and patient education. Perioperative assessment enables the discussion of the proposed treatment plans and the factors associated with them in each case among the different specialists involved to facilitate an appropriate early decision-making about the assessment and treatment of patients with rheumatologic diseases. It also enables the discussion of both condition and procedure with the patient to ensure a good postoperative care. The article identifies the components of perioperative medical evaluation, discusses perioperative management of co-morbidities and the management of specific clinical problems related to RA, systemic lupus erythematosus, the management of DMARDs, like methotrexate (MTX) and biologic therapies, prophylactic antibiotics, and postoperative follow up, including patient education and rehabilitation PMID:24062860

  2. Prevalence of celiac disease in siblings of Iranian patients with celiac disease

    Directory of Open Access Journals (Sweden)

    Bashir Chomeili

    2011-06-01

    Full Text Available CONTEXT: Celiac disease, one of the best-known autoimmune human leukocyte antigen-dependent disorders, has a relatively increased prevalence in first-degree relatives. OBJECTIVE: To determine the prevalence of celiac disease in siblings of patients with confirmed celiac disease. METHODS: Siblings of confirmed celiac disease patients in our center were identified and enrolled in this study. Their serum immunoglobulin A and tissue transglutaminase antibody-enzyme-linked immunosorbent assay (anti-tissue transglutaminase, immunoglobulin A, and immunoglobulin G were measured and multiple endoscopic duodenal biopsy specimens were obtained with parental consensus. Celiac disease was confirmed by observation of characteristic histological changes. RESULTS: A total of 49 children (male, 29; female, 20; age, 2-16 years with confirmed celiac disease in a pediatric gastroenterology ward were studied from 1999 to 2006. We found 30 siblings (female, 16 all shared in both parents. The only measurement available was for immunoglobulin A tissue transglutaminase antibody. A duodenal biopsy was performed in all 30 siblings. Clinical findings such as abdominal pain, fatigue, growth retardation and diarrhea were found in 53.3% of the completely studied siblings, and positive serology without histological changes was identified in four cases. Both serology and biopsy (confirmed new cases were positive in 2 of the 30 siblings. CONCLUSION: High prevalence of celiac disease among siblings of patients with confirmed celiac disease necessitates serologic screening (and confirmatory biopsy if indicated in families having celiac disease. It is advantageous to diagnose the disease as soon as possible because early diagnosis and diet intervention may prevent serious complications such as growth retardation, short stature, chronic diarrhea, and malignancy.

  3. Analysis of electrocardiogram in chronic obstructive pulmonary disease patients.

    Science.gov (United States)

    Lazović, Biljana; Svenda, Mirjana Zlatković; Mazić, Sanja; Stajić, Zoran; Delić, Marina

    2013-01-01

    Chronic obstructive pulmonary disease is the fourth leading cause of mortality worldwide. It is defined as a persistent airflow limitation usually progressive and not fully reversible to treatment. The diagnosis of chronic obstructive pulmonary disease and severity of disease is confirmed by spirometry. Chronic obstructive pulmonary disease produces electrical changes in the heart which shows characteristic electrocardiogram pattern. The aim of this study was to observe and evaluate diagnostic values of electrocardiogram changes in chronic obstructive pulmonary disease patients with no other comorbidity. We analyzed 110 electrocardiogram findings in clinically stable chronic obstructive pulmonary disease patients and evaluated the forced expiratory volume in the first second, ratio of forces expiratory volume in the first second to the fixed vital capacity, chest radiographs and electrocardiogram changes such as p wave height, QRS axis and voltage, right bundle branch block, left bundle branch block, right ventricular hypertrophy, T wave inversion in leads V1-V3, S1S2S3 syndrome, transition zone in praecordial lead and QT interval. We found electrocardiogram changes in 64% patients, while 36% had normal electrocardiogram. The most frequent electrocardiogram changes observed were transition zone (76.36%) low QRS (50%) and p pulmonale (14.54%). Left axis deviation was observed in 27.27% patients. Diagnostic values of electrocardiogram in patients with chronic obstructive pulmonary disease suggest that chronic obstructive pulmonary disease patients should be screened electrocardiographically in addition to other clinical investigations.

  4. Glaucoma in Patients with Eyes Close to Areas Affected by Port-wine Stain has Lateral and Gender Predilection

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    Yue Wu

    2017-01-01

    Conclusions: High glaucoma incidence was observed in patients with eyes close to PWS. More attention should be paid to glaucoma screening for right lateral and bilateral PWS patients. The predominance of males in Sturge–Weber syndrome (SWS early-onset glaucoma patients might be due to the limitation of the case number; however, it might also provide us a new clue of potential relationship between SWS and PCG.

  5. Factors contributing to malnutrition in patients with Parkinson's disease.

    Science.gov (United States)

    Kim, Sung R; Chung, Sun J; Yoo, Sung-Hee

    2016-04-01

    Our objective in this study was to evaluate the nutritional status and to identify clinical, psychosocial, and nutritional factors contributing to malnutrition in Korean patients with Parkinson's disease. We used a descriptive, cross-sectional study design. Of 102 enrolled patients, 26 (25.5%) were malnourished and 27 (26.5%) were at risk of malnutrition based on Mini-Nutritional Assessment scores. Malnutrition was related to activity of daily living score, Hoehn and Yahr stage, duration of levodopa therapy, Beck Depression Inventory and Spielberger's Anxiety Inventory scores, body weight, body weight at onset of Parkinson's disease, and body mass index. On multiple logistic regression analysis, anxiety score, duration of levodopa therapy, body weight at onset of Parkinson's disease, and loss of body weight were significant factors predicting malnutrition in Parkinson's disease patients. Therefore, nutritional assessment, including psychological evaluation, is required for Parkinson's disease patients to facilitate interdisciplinary nutritional intervention for malnourished patients. © 2014 John Wiley & Sons Australia, Ltd.

  6. Pooled analysis of the CONFIRM Registries: outcomes in renal disease patients treated for peripheral arterial disease using orbital atherectomy.

    Science.gov (United States)

    Lee, Michael S; Yang, Tae; Adams, George L; Mustapha, Jihad; Das, Tony

    2014-08-01

    Patients with renal disease typically have severely calcified peripheral arterial disease. As a result, this population may have worse clinical outcomes following endovascular intervention compared to patients without renal insufficiency. Clinical trials typically exclude this patient population. Analysis of the CONFIRM I-III registries revealed 1105 patients with renal disease (1777 lesions) and 1969 patients without renal disease (2907 lesions) who underwent orbital atherectomy. This subanalysis compared the composite procedural complication rate including dissection, perforation, slow flow, vessel closure, spasm, embolism, and thrombus formation in patients with and without renal disease. Patients with renal disease had a higher prevalence of diabetes (Patherectomy resulted in similar low rates of procedural complications in the renal disease group compared with the non-renal disease group despite more unfavorable baseline clinical and lesion characteristics in the renal disease group.

  7. Patients' information-seeking activity is associated with treatment compliance in inflammatory bowel disease patients.

    Science.gov (United States)

    Pittet, Valérie; Rogler, Gerhard; Mottet, Christian; Froehlich, Florian; Michetti, Pierre; de Saussure, Philippe; Burnand, Bernard; Vader, John-Paul

    2014-06-01

    Despite the chronic and relapsing nature of inflammatory bowel diseases (IBD), at least 30% to 45% of the patients are noncompliant to treatment. IBD patients often seek information about their disease. To examine the association between information-seeking activity and treatment compliance among IBD patients. To compare information sources and concerns between compliant and noncompliant patients. We used data from the Swiss IBD cohort study, and from a qualitative survey conducted to assess information sources and concerns. Crude and adjusted odds ratios (OR) for noncompliance were calculated. Differences in the proportions of information sources and concerns were compared between compliant and noncompliant patients. A total of 512 patients were included. About 18% (n = 99) of patients were reported to be noncompliant to drug treatment and two-thirds (n = 353) were information seekers. The OR for noncompliance among information seekers was 2.44 (95%CI: 1.34-4.41) after adjustment for confounders and major risk factors. General practitioners were 15.2% more often consulted (p = 0.019) among compliant patients, as were books and television (+13.1%; p = 0.048), whereas no difference in proportions was observed for sources such as internet or gastroenterologists. Information on tips for disease management were 14.2% more often sought among noncompliant patients (p = 0.028). No difference was observed for concerns on research and development on IBD or therapies. In Switzerland, IBD patients noncompliant to treatment were more often seeking disease-related information than compliant patients. Daily management of symptoms and disease seemed to be an important concern of those patients.

  8. Current treatments for patients with Alzheimer disease.

    Science.gov (United States)

    Osborn, Gerald G; Saunders, Amanda Vaughn

    2010-09-01

    There is neither proven effective prevention for Alzheimer disease nor a cure for patients with this disorder. Nevertheless, a spectrum of biopsychosocial therapeutic measures is available for slowing progression of the illness and enhancing quality of life for patients. These measures include a range of educational, psychological, social, and behavioral interventions that remain fundamental to effective care. Also available are a number of pharmacologic treatments, including prescription medications approved by the US Food and Drug Administration for Alzheimer disease, "off-label" uses of medications to manage target symptoms, and controversial complementary therapies. Physicians must make the earliest possible diagnosis to use these treatments most effectively. Physicians' goals should be to educate patients and their caregivers, to plan long-term care options, to maximally manage concurrent illnesses, to slow and ameliorate the most disabling symptoms, and to preserve effective functioning for as long as possible. The authors review the various current treatments for patients with Alzheimer disease.

  9. Optimization of Agave tequilana Weber var. Azul Juice Spray Drying Process

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    Alejandra Chávez-Rodríguez

    2014-01-01

    Full Text Available In this work, the response surface methodology was employed to optimize the microencapsulation of Agave tequilana Weber var. azul juice with whey protein isolated using a spray drying technique. A Box-Behnken design was used to establish optimum spray drying conditions for Agave tequilana juice. The process was optimized to obtain maximum powder yield with the best solubility time, hygroscopicity, bulk density, water activity, and reducing sugars. The independent parameters for the spray drying process were outlet temperature of 70–80°C, atomizer speed of 20000–30000 rpm, and airflow of 0.20–0.23 m3 s−1. The best spray drying condition was at outlet temperature of 80°C, atomizer speed of 20000 rpm, and air flow rate of 0.23 m3 s−1 to obtain maximum powder yield (14.65%bm, minimum solubility time (352.8 s, maximum bulk density (560 kg m−3, minimum hygroscopicity (1.9×10-7 kgwater s−1, and minimum aw (0.39. The Agave tequilana powder may be considered as an interesting source of dietary fiber used as food additive in food and nutraceutical industries.

  10. Rare disease patients in China anticipate the sunlight of legislation.

    Science.gov (United States)

    Gao, J J; Song, P P; Tang, W

    2013-06-01

    It is estimated that there are over ten million rare disease patients in China currently. Due to a lack of effective drugs and reimbursement regulations for medical expenses the diseases bring most patients enormous physical suffering and psychological despair. Past experience in other countries such as the United States, Japan, and the European Union have shown that legislation is the critical step to improve the miserable situation of rare disease patients. Laws and regulations for rare diseases in these countries prescribe a series of incentives for research and development of orphan drugs which turn out to obviously allow these drugs to flourish. Legislation has also established a drug reimbursement system to reduce the medical burden of the patients. These measures effectively protect the rights and interests of patients with rare diseases. In China, legislation for rare diseases has begun to attract the attention of authorities. It is anticipated that relevant laws and regulations will be established as early as possible to provide safeguards for rare disease patients in China.

  11. Immune defects in active mycobacterial diseases in patients with primary immunodeficiency diseases (PIDs

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    Wen-I Lee

    2011-12-01

    Full Text Available Natural human immunity to the mycobacteria group, including Mycobacterium tuberculosis, Bacille Calmette-Guérin (BCG or nontuberculous mycobacteria (NTM, and/or Salmonella species, relies on the functional IL-12/23-IFN-γ integrity of macrophages (monocyte/dendritic cell connecting to T lymphocyte/NK cells. Patients with severe forms of primary immunodeficiency diseases (PIDs have more profound immune defects involving this impaired circuit in patients with severe combined immunodeficiencies (SCID including complete DiGeorge syndrome, X-linked hyper IgM syndrome (HIGM (CD40L mutation, CD40 deficiency, immunodeficiency with or without anhidrotic ectodermal dysplasia (NEMO and IKBA mutations, chronic granulomatous disease (CGD and hyper IgE recurrent infection syndromes (HIES. The patients with severe PIDs have broader diverse infections rather than mycobacterial infections. In contrast, patients with an isolated inborn error of the IL-12/23-IFN-γ pathway are exclusively prone to low-virulence mycobacterial infections and nontyphoid salmonella infections, known as Mendelian susceptibility to the mycobacterial disease (MSMD phenotype. Restricted defective molecules in the circuit, including IFN-γR1, IFN-γR2, IL-12p40, IL-12R-β1, STAT-1, NEMO, IKBA and the recently discovered CYBB responsible for autophagocytic vacuole and proteolysis, and interferon regulatory factor 8 (IRF8 for dendritic cell immunodeficiency, have been identified in around 60% of patients with the MSMD phenotype. Among all of the patients with PIDs referred for investigation since 1985, we have identified four cases with the specific defect (IFNRG1 for three and IL12RB for one, presenting as both BCG-induced diseases and NTM infections, in addition to some patients with SCID, HIGM, CGD and HIES. Furthermore, manifestations in patients with autoantibodies to IFN-γ (autoAbs-IFN-γ, which is categorized as an anticytokine autoantibody syndrome, can resemble the relatively

  12. Voluntary imitation in Alzheimer’s disease patients

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    Ambra eBisio

    2016-03-01

    Full Text Available Although Alzheimer's disease (AD primarily manifests as cognitive deficits, the implicit sensorimotor processes that underlie social interactions, such as automatic imitation, seem to be preserved in mild and moderate stages of the disease, as is the ability to communicate with other persons. Nevertheless, when AD patients face more challenging tasks, which do not rely on automatic processes but on explicit voluntary mechanisms and require the patient to pay attention to external events, the cognitive deficits resulting from the disease might negatively affect patients’ behaviour. The aim of the present study was to investigate whether voluntary motor imitation, i.e. a volitional mechanism that involves observing another person’s action and translating this perception into one’s own action, was affected in patients with Alzheimer’s disease. Further, we tested whether this ability was modulated by the nature of the observed stimulus by comparing the ability to reproduce the kinematic features of a human demonstrator with that of a computerized-stimulus. AD patients showed an intact ability to reproduce the velocity of the observed movements, particularly when the stimulus was a human agent. This result suggests that high-level cognitive processes involved in voluntary imitation might be preserved in mild and moderate stages of AD and that voluntary imitation abilities might benefit from the implicit interpersonal communication established between the patient and the human demonstrator.

  13. Treatment of patients with minimal stage IIIA Hodgkin's disease

    International Nuclear Information System (INIS)

    Powlis, W.D.; Mauch, P.; Goffman, T.; Goodman, R.L.

    1987-01-01

    Treatment recommendations for patients with upper abdominal Stage IIIA Hodgkin's (III1A) disease have varied widely. The current study reports on a combined institutional retrospective review of 85 patients with surgically staged III1A Hodgkin's disease. Twenty-two patients received combined modality therapy (CMT), 36 patients were treated initially with total nodal irradiation (TNI), and 27 with mantle and para-aortic radiotherapy (MPA). Patients treated with CMT had an actuarial 8-year freedom from relapse (FFR) of 96% as compared to a FFR of 51% in TNI treated patients (p = 0.002), and a FFR of 54% in MPA treated patients (p = 0.004). Of the 11 relapses in MPA treated patients, 7 had a component of their failure in the untreated pelvic or inguinal nodes. The patients treated with CMT had an 8-year actuarial survival of 100% as compared to 79% in TNI treated patients (p = 0.055) and 78% in patients treated with MPA (p = 0.025). Histology and the number of splenic nodules were the most important prognostic variables. Patients with MC/LD histology and greater than or equal to 5 splenic nodules have a high risk of relapse (10/13) when treated with radiation alone (TNI or MPA). We recommend CMT for this group of patients. Patients with NS/LP histology and 1-4 splenic nodules represent a favorable subset of Stage III1A patients. Only 4/21 patients have relapsed and all 21 patients are currently alive without disease regardless of treatment. We currently feel that patients with Stage III1A Hodgkin's disease with NS/LP histology and splenic disease limited to 1-4 nodules are good candidates for MPA as an alternative to TNI or CMT

  14. Neoplastic pericardial disease. Analysis of 26 patients

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    Helena Nogueira Soufen

    1999-01-01

    Full Text Available PURPOSE: To characterize patients with neoplastic pericardial disease diagnosed by clinical presentation, complementary test findings, and the histological type of tumor. METHODS: Twenty-six patients with neoplastic pericardial disease were retrospectively analyzed. RESULTS: Clinical manifestations and abnormalities in chest roentgenograms and electrocardiograms were frequent, but were not specific. Most patients underwent surgery. There was a high positivity of the pericardial biopsy when associated with the cytological analysis of the pericardial liquid used to determine the histological type of the tumor, particularly when the procedure was performed with the aid of pericardioscopy. CONCLUSION: The correct diagnosis of neoplastic pericardial disease involves suspicious but nonspecific findings during clinical examination and in screen tests. The suspicious findings must be confirmed through more invasive diagnostic approaches, in particular pericardioscopy with biopsy and cytological study.

  15. Increased self-transcendence in patients with intractable diseases.

    Science.gov (United States)

    Iwamoto, Rie; Yamawaki, Niwako; Sato, Takeshi

    2011-12-01

    Patients with intractable disease require long-term treatment and experience repeated bouts of progressive symptoms and resolutions, which cause them severe suffering. The aim of this study was to elucidate the concepts of self-transcendence and subjective well-being in patients with intractable disease. Forty-four patients with intractable disease (men/women: 22/22) participated. The diseases of the participants were classified into five systems: (i) neural/muscle system; (ii) digestive system; (iii) immunity/blood system; (iv) visual system; and (v) bone/joint system. The controls were 1854 healthy individuals (men/women: 935/869). Participants completed the Self-Transcendence Scale (STS) and the Japanese version of the World Health Organization-Subjective Inventory. The Japanese version of the Mini-International Neuropsychiatric Interview was also used for the intractable disease group. Analysis of covariance found a significant increase in STS score among the intractable disease group (P self-transcendence. The results also showed that there was a strong correlation between self-transcendence and respondents' subjective well-being. Our results suggest that patients with life-changing intractable disease can have a high level of self-transcendence, which may lead them to regain mental well-being, and increase their psychological health even in situations that cause physical and mental suffering. © 2011 The Authors. Psychiatry and Clinical Neurosciences © 2011 Japanese Society of Psychiatry and Neurology.

  16. Health care resource use and costs among patients with cushing disease.

    Science.gov (United States)

    Swearingen, Brooke; Wu, Ning; Chen, Shih-Yin; Pulgar, Sonia; Biller, Beverly M K

    2011-01-01

    To assess health care costs associated with Cushing disease and to determine changes in overall and comorbidity-related costs after surgical treatment. In this retrospective cohort study, patients with Cushing disease were identified from insurance claims databases by International Classification of Diseases, 9th Revision, Clinical Modification (ICD-9-CM) codes for Cushing syndrome (255.0) and either benign pituitary adenomas (227.3) or hypophysectomy (07.6×) between 2004 and 2008. Each patient with Cushing disease was age- and sex-matched with 4 patients with nonfunctioning pituitary adenomas and 10 population control subjects. Comorbid conditions and annual direct health care costs were assessed within each calendar year. Postoperative changes in health care costs and comorbidity-related costs were compared between patients presumed to be in remission and those with presumed persistent disease. Of 877 identified patients with Cushing disease, 79% were female and the average age was 43.4 years. Hypertension, diabetes mellitus, and hyperlipidemia were more common among patients with Cushing disease than in patients with nonfunctioning pituitary adenomas or in control patients (PCushing disease had significantly higher total health care costs (2008: $26 440 [Cushing disease] vs $13 708 [nonfunctioning pituitary adenomas] vs $5954 [population control], Pdisease-related costs with remission. A significant increase in postoperative health care costs was observed in those patients not in remission. Patients with Cushing disease had more comorbidities than patients with nonfunctioning pituitary adenomas or control patients and incurred significantly higher annual health care costs; these costs decreased after successful surgery and increased after unsuccessful surgery.

  17. Health behavior of patients with ischemic heart disease

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    Paweł Węgorowski

    2017-06-01

    Full Text Available Admission By analyzing the available scientific literature, it is possible to define ischemic heart disease as a set of disease symptoms that are a consequence of a chronic state of imbalance between the ability to supply nutrients and oxygen and the real need of myocardial cells for these substances. Adapting life-style behaviors to healthy living is a priority to prevent the onset and development of cardiovascular disease, especially ischemic heart disease, Purpose of research The aim of the study is to determine the health behavior of patients with ischemic heart disease. Materials and methods The study was conducted from 01.08.2015 to 28.12.2015 in a group of 35 people (15 women and 20 men. The research method used in the work is a diagnostic survey, the research technique used was a survey of its own author. Conclusions By analyzing the data collected, it is important to note that patients with coronary heart disease are often associated with health problems such as hypertension, diabetes and abnormal weight. The nutritional habits of the subjects studied can be described as abnormal, particularly the excessive intake of oily meat and too little fish intake. It has also been observed that most of the patients studied have familial predisposition to ischemic heart disease. Discussion Heart attacks occur mostly in people with obesity, diabetes and atherosclerosis. It is also closely related to ischemic heart disease. The health behaviors of patients suffering from Ischemic Heart Disease are moderately satisfactory and therefore the role of a nurse practitioner as a health educator is very difficult but essential in the prevention of ischemic heart disease.

  18. Changes in Disease Behaviour and Location in Patients With Crohn's Disease After Seven Years of Follow-Up

    DEFF Research Database (Denmark)

    Lo, B; Vester-Andersen, M K; Vind, I

    2018-01-01

    Background and Aim: Crohn's disease [CD] is a progressive inflammatory bowel disease that can lead to complications such as strictures or penetrating disease, and ultimately surgery. Few population-based studies have investigated the predictors for disease progression and surgery in CD according....... Disease progression was defined as the development of bowel stricture [B2] or penetrating disease [B3] in patients initially diagnosed with non-stricturing/non-penetrating disease [B1]. Associations between disease progression and/or resection, and multiple covariates, were investigated by Cox regression...... analyses. Results: In total, 213 CD patients were followed. A total of 177 [83%] patients had B1 at diagnosis. Patients who changed location had increased risk of disease progression (hazard ratio [HR] = 3.1, 95% CI: 1.12,8.52). Biologic treatment was associated with lower risk of change in location [HR...

  19. ECG abnormalities in patients with chronic kidney disease

    International Nuclear Information System (INIS)

    Shafi, S.; Saleem, M.; Anjum, R.; Abdullah, W.; Shafi, T.

    2017-01-01

    Chronic kidney disease (CKD) is associated with increased risk of cardiovascular disease. Electrocardiographic (ECG) abnormalities are common in CKD patients. However, there is variation in literature regarding frequency of ECG abnormalities in CKD patients and limited information in local population. Methods: The study design was cross-sectional in nature. All patients between ages of 20-80 years with CKD not previously on renal replacement therapy who were admitted to nephrology ward at a tertiary care facility over a 6-month period were included. All patients underwent 12 lead electrocardiograms (ECG). ECG abnormalities were defined based on accepted standard criteria. Results: Total number of patients included in the study was 124. Mean age of all patients was 49.9+-13.8 years, 106 (84.8%) had hypertension, 84 (70%) had diabetes mellitus, and 35 (29.9%) had known cardiovascular disease. Mean serum creatinine was 7.2+-3.4 mg/dl, mean eGFR was 10.6+-9.2 ml/min/1.73 m/sup 2/. Overall 78.4% of all CKD patients have one or more ECG abnormality. Left ventricular hypertrophy (40%), Q waves (27.2%), ST segment elevation or depression (23.4%), prolonged QRS duration (19.2%), tachycardia (17.6%) and left and right atrial enlargement (17.6%) were the most common abnormalities. Conclusion: ECG abnormalities are common in hospitalized CKD patients in local population. All hospitalized CKD patients should undergo ECG to screen for cardiovascular disease. (author)

  20. Predictive factors of thyroid cancer in patients with Graves' disease.

    Science.gov (United States)

    Ren, Meng; Wu, Mu Chao; Shang, Chang Zhen; Wang, Xiao Yi; Zhang, Jing Lu; Cheng, Hua; Xu, Ming Tong; Yan, Li

    2014-01-01

    The best preoperative examination in Graves' disease with thyroid cancer still remains uncertain. The objectives of the present study were to investigate the prevalence of thyroid cancer in Graves' disease patients, and to identify the predictive factors and ultrasonographic features of thyroid cancer that may aid the preoperative diagnosis in Graves' disease. This retrospective study included 423 patients with Graves' disease who underwent surgical treatment from 2002 to 2012 at our institution. The clinical features and ultrasonographic findings of thyroid nodules were recorded. The diagnosis of thyroid cancer was determined according to the pathological results. Thyroid cancer was discovered in 58 of the 423 (13.7 %) surgically treated Graves' disease patients; 46 of those 58 patients had thyroid nodules, and the other 12 patients were diagnosed with incidentally discovered thyroid carcinomas without thyroid nodules. Among the 58 patients with thyroid cancer, papillary microcarcinomas were discovered in 50 patients, and multifocality and lymph node involvement were detected in the other 8 patients. Multivariate regression analysis showed younger age was the only significant factor predictive of metastatic thyroid cancer. Ultrasonographic findings of calcification and intranodular blood flow in thyroid nodules indicate that they are more likely to harbor thyroid cancers. Because the influencing factor of metastatic thyroid cancers in Graves' disease is young age, every suspicious nodule in Graves' disease patients should be evaluated and treated carefully, especially in younger patients because of the potential for metastasis.

  1. Cross-cultural variation in disease-related concerns among patients with inflammatory bowel disease.

    Science.gov (United States)

    Levenstein, S; Li, Z; Almer, S; Barbosa, A; Marquis, P; Moser, G; Sperber, A; Toner, B; Drossman, D A

    2001-06-01

    The aim of this work was to study cross-cultural variations in the impact of inflammatory bowel disease (IBD) on health-related quality of life by an international comparison of disease-related concerns. Item and factor scores on the Rating Form of Inflammatory Bowel Disease Patient Concerns and overall mean concern levels were compared by analysis of variance among 2002 IBD patients in eight countries. The overall level of concern varied from 51 out of 100 in Portugal to 19 in Sweden, with intermediate scores for Italy (43), Canada (40), United States (39), France (39), Austria (33), and Israel (25). Having surgery, an ostomy, the uncertain nature of the disease, and medication side effects were each rated among the first five in importance in six countries. Other items varied considerably. For example, concern regarding pain and suffering was high in Israel and low in Portugal, whereas concern over developing cancer was low in Italy. Concern over financial issues and access to high-quality health care were inversely associated with measures of national economic prosperity. 1) Cross-cultural comparisons of patient concerns related to IBD are feasible using translated scales. 2) Reporting tendencies vary greatly; within Europe, patients from southern countries report greater overall concern. 3) The complications and the variable evolution of disease elicit general concern, but the importance of specific issues varies among countries. 4) The reasons for national differences may have social, cultural, and/or economic determinants with relevance to the patient-physician relationship, patient education, and therapeutic decision making.

  2. Brain MRI in 17 patients with ocular Behcet's disease

    International Nuclear Information System (INIS)

    Accorinti, Massimo; Pivetti Pezzi, Paola; Di Biasi, Claudio; Trasimeni, Guido; Melone, Antonio; Gualdi, Gianfranco

    1997-01-01

    Behcet's disease is a chronic relapsing disorder of unknown etiology characterized by oral aphthous ulcerations, uveitis, genital ulcerations and bone lesions. A variety of other signs including polyarthritis, vascular conditions (blood vessel occlusions and aneurysms), epididymitis, gastrointestinal, pulmonary and heart lesions may also occur. Central nervous system (CNS) involvement is reported in 10-49 % of cases and it is the first symptom of the disease in 5 % of subjects. The neuro-Behcet's syndrome may appear as a brainstem syndrome, and an organic confusional syndrome or dementia. cranial hypertension, mostly related to cerebral venus thrombosis, is also present in neuro-Behcet's disease and its incidence is reported in up to 10 % of Behcet's patients. MRI is reportedly the most sensitive neuroradiological approach to detect the focal lesions related to neuro-Behcet disease and several single cases or series of Behcet's patients with neurologic sings have been examined with MRI. They used MRI to investigate CNS involvement in Behcet's disease patients with and without previous neurologic sings. MRI was carried out on 17 patients with ocular Behcet's disease without neurological symptoms to assess the possible subclinical involvement of the CNS. PD and T2-weighted hypersignal foci were demonstrated in parietal, frontal, subcortical and periventricular white matter in 6 subjects. Neuroradiological abnormalities were found only in patients with complete disease and with the disease diagnosed more than 10 years earlier. Even though the pathogenesis of these neuroradiological abnormalities and their correlation with Behcet's disease remain to be clarified, their study suggests the possibility of subclinical CNS involvement in these patients, which may affect the therapeutic approach and their prognosis

  3. Mortality in patients with Parkinson's disease

    DEFF Research Database (Denmark)

    Wermuth, L; Stenager, E; Stenager, E

    1995-01-01

    INTRODUCTION: After the introduction of L-dopa the mortality rate in Parkinson's disease (PD) patients has changed, but is still higher than in the background population. MATERIAL & METHODS: Mortality, age at death and cause of death in a group of PD patients compared with the background population....... In the background population the median age at death was 80.69 years for men and 84.37 years for women. The SMR for men was 1.92 and for women 2.47. Infections, in particular lung infections, and heart diseases were the most common causes of death. Seventy percent of the death certificates had PD as a diagnosis....... CONCLUSION: It is likely that several factors can influence the changed mortality of PD: more effective treatment, changing diagnostic practice, and inter-disease competition....

  4. Cerebrovascular disease in pediatric patients

    Directory of Open Access Journals (Sweden)

    Rotta Newra Tellechea

    2002-01-01

    Full Text Available Although rare in childhood, stroke may have a serious impact when it happens in this stage of life. Also, it may be the first sign of a systemic disease. We report 12 cases of patients with stroke treated in the Neuropediatrics Unit of Hospital de Clínicas de Porto Alegre (HCPA from March 1997 to March 2000. All patients, from term infants to 12-year-old children hospitalized in the Pediatrics Unit of HCPA, had clinical suspicion of stroke, which was later confirmed by radiological studies. Patient follow up ranged from 1 to 6 years (mean = 3.4 years. Presenting symptoms were hemiparesis in 9 patients, seizures in 7, deviation of labial commissure in 3, and loss of consciousness in 1. The increase in the number of cases of childhood stroke identified and later confirmed by noninvasive methods had helped in the determination of different ethiologies of stroke: the most frequent being hematologic, cardiac and genetic diseases. However, our study included 6 newborns with stroke whose ethiology was not identified. Seven children with seizures received phenobarbital. Six term infants had neonatal seizures secondary to stroke and restricted to the first 72 hours of life.

  5. Periodontal disease in diabetic patients - clinical and histopathological aspects.

    Science.gov (United States)

    Corlan Puşcu, Dorina; Ciuluvică, Radu Constantin; Anghel, Andreea; Mălăescu, Gheorghe Dan; Ciursaş, Adina Nicoleta; Popa, Gabriel Valeriu; Agop Forna, Doriana; Busuioc, Cristina Jana; Siloşi, Izabela

    2016-01-01

    Periodontal disease is one of the most frequent diseases affecting people all over the world. The relation between periodontal disease and diabetes mellitus raised the interest both of dentists and doctors treating metabolic diseases, as the two conditions influence one another. In our study, we analyzed a number of 75 patients with diabetes mellitus and periodontal disease that presented to the medical consultory for conditions of the dental maxillary system. The clinical study showed that periodontal disease and diabetes may affect young adults as well, still this pathological association more frequently appears after the age of 50. The disease was identified especially in the women living in urban area. The clinical examination of the dental maxillary system identified the presence of gingival ulcerations, dental calculus, gingival bleeding, radicular leftovers with anfractuous margins, fixed prostheses with an inappropriate cervical adjustment. Of the systemic diseases associated to periodontal disease and diabetes mellitus, there was observed that 66.66% of the patients also suffered from cardiovascular diseases (high blood pressure, ischemic cardiopathy, heart failure), and 37.33% suffered from obesity. The histopathological and immunohistochemical tests highlighted the presence of an inflammatory chronic, intense reaction, mainly formed of lymphocytes, plasmocytes, macrophages and granulocytes, heterogeneously disseminated and alteration of the structure of marginal and superficial periodontium. The inflammatory reaction in the patients with periodontal disease and diabetes was more intense than in the patients with periodontal disease without diabetes.

  6. Cardiometabolic factors and disease duration in patients with Parkinson's disease.

    Science.gov (United States)

    Cassani, Erica; Cereda, Emanuele; Barichella, Michela; Madio, Carmen; Cancello, Raffaella; Caccialanza, Riccardo; Zini, Michela; Cilia, Roberto; Pezzoli, Gianni

    2013-01-01

    Previous studies have reported that patients with Parkinson's disease (PD) have a favorable cardiometabolic risk profile. The aim of this study was to investigate the relationship between cardiometabolic risk factors and the duration of disease. One hundred and fifty patients with PD (56.7% men) were studied, measuring body mass index (BMI), waist circumference (WC), body fat percentage (BF%) by impedance, fasting glucose, serum lipids, and transaminases. In sex- and age-adjusted correlation models, duration of PD was inversely related to BMI (r = -0.20; P HDL) levels were positively correlated with disease duration (P HDL-cholesterol ratio was also inversely associated with duration of PD (P HDL levels and total HDL-cholesterol ratio were favorably associated with duration of PD. This factor may contribute to cardiometabolic protection in PD. The mechanisms underlying this association deserve further investigation. Copyright © 2013 Elsevier Inc. All rights reserved.

  7. Abdominal fat and risk of coronary heart disease in patients with peripheral arterial disease

    NARCIS (Netherlands)

    Brouwer, Beate G.; Visseren, Frank L. J.; Stolk, Ronald P.; van der Graaf, Yolanda

    Objective: We investigated whether the presence of concomitant coronary heart disease (CHD) in patients with peripheral arterial disease (PAD) can be explained by intra-abdominal fat accumulation and compared different measures of adiposity as predictors of CHD in patients with PAD. Research Methods

  8. Stillinger-Weber potential for elastic and fracture properties in graphene and carbon nanotubes

    Science.gov (United States)

    Hossain, M. Z.; Hao, T.; Silverman, B.

    2018-02-01

    This paper presents a new framework for determining the Stillinger-Weber (SW) potential parameters for modeling fracture in graphene and carbon nanotubes. In addition to fitting the equilibrium material properties, the approach allows fitting the potential to the forcing behavior as well as the mechanical strength of the solid, without requiring ad hoc modification of the nearest-neighbor interactions for avoiding artificial stiffening of the lattice at larger deformation. Consistent with the first-principles results, the potential shows the Young’s modulus of graphene to be isotropic under symmetry-preserving and symmetry-breaking deformation conditions. It also shows the Young’s modulus of carbon nanotubes to be diameter-dependent under symmetry-breaking loading conditions. The potential addresses the key deficiency of existing empirical potentials in reproducing experimentally observed glass-like brittle fracture in graphene and carbon nanotubes. In simulating the entire deformation process leading to fracture, the SW-potential costs several factors less computational time compared to the state-of-the-art interatomic potentials that enables exploration of the fracture processes in large atomistic systems which are inaccessible otherwise.

  9. Chronic Inflammatory Periodontal Disease in Patients with Human Immunodeficiency Virus.

    OpenAIRE

    Vania López Rodríguez; Emilio Carpio Muñoz; Vicente Fardales Macías; Iralys Benítez Guzmán

    2009-01-01

    Background: The Chronic Inflammatory Periodontal Disease is related with multiple risk factors. Those patients with human immunodeficiency virus have higher risk of presenting this disease and it is usually more serious in these cases. Objective: To describe the prevalence of Chronic Inflammatory Periodontal Disease in patients with HIV. Methods: Descriptive, observational, cross-sectional study including patients with HIV in Sancti Spiritus province. The occurrence of the disease was determi...

  10. Medications Used for Cognitive Enhancement in Patients With Schizophrenia, Bipolar Disorder, Alzheimer’s Disease, and Parkinson’s Disease

    Directory of Open Access Journals (Sweden)

    Wen-Yu Hsu

    2018-04-01

    Full Text Available Background/aimsCognitive impairment, which frequently occurs in patients with schizophrenia, bipolar disorder, Alzheimer’s disease, and Parkinson’s disease, has a significant impact on the daily lives of both patients and their family. Furthermore, since the medications used for cognitive enhancement have limited efficacy, the issue of cognitive enhancement still remains a clinically unsolved challenge.Sampling and methodsWe reviewed the clinical studies (published between 2007 and 2017 that focused on the efficacy of medications used for enhancing cognition in patients with schizophrenia, bipolar disorder, Alzheimer’s disease, and Parkinson’s disease.ResultsAcetylcholinesterase inhibitors and memantine are the standard treatments for Alzheimer’s disease and Parkinson’s disease. Some studies have reported selective cognitive improvement in patients with schizophrenia following galantamine treatment. Newer antipsychotics, including paliperidone, lurasidone, aripiprazole, ziprasidone, and BL-1020, have also been reported to exert cognitive benefits in patients with schizophrenia. Dopaminergic medications were found to improve language function in patients with Parkinson’s disease. However, no beneficial effects on cognitive function were observed with dopamine agonists in patients with schizophrenia. The efficacies of nicotine and its receptor modulators in cognitive improvement remain controversial, with the majority of studies showing that varenicline significantly improved the cognitive function in schizophrenic patients. Several studies have reported that N-methyl-d-aspartate glutamate receptor (NMDAR enhancers improved the cognitive function in patients with chronic schizophrenia. NMDAR enhancers might also have cognitive benefits in patients with Alzheimer’s disease or Parkinson’s disease. Raloxifene, a selective estrogen receptor modulator, has also been demonstrated to have beneficial effects on attention, processing

  11. Surgical care of the pediatric Crohn's disease patient.

    Science.gov (United States)

    Stewart, Dylan

    2017-12-01

    Despite the significant advances in the medical management of inflammatory bowel disease over the last decade, surgery continues to play a major role in the management of pediatric Crohn's disease (CD). While adult and pediatric Crohn's disease may share many clinical characteristics, pediatric Crohn's patients often have a more aggressive phenotype, and the operative care given by the pediatric surgeon to the newly diagnosed Crohn's patient is very different in nature to the surgical needs of adult patients after decades of disease progression. Children also have the unique surgical indication of growth failure to consider in the overall clinical decision making. While surgery is never curative in CD, it has the ability to transform the disease process in children, and appropriately timed operations may have tremendous impact on a child's physical and mental maturation. This monograph aims to address the surgical care of Crohn's disease in general, with a specific emphasis on the surgical treatment of small intestinal and ileocecal involvement. Copyright © 2017 Elsevier Inc. All rights reserved.

  12. Towards safer surgery in patients with sickle cell disease

    International Nuclear Information System (INIS)

    Meshikhes, Abdul-Wahed N.

    2007-01-01

    Surgery in patients with sickle cell disease (SCD) has been associated with high morbidity and mortality. In recent years, a marked improvement in the safety of surgery and anesthesia in this high-risk group of patients has been witnessed; owing to the improvements in surgical and anesthetic care, greater awareness of pathophysiology of disease, proper perioperative preparation and attention to factors predisposing to vasoocclusive crises. However, this is not paralleled by similar improvement in countries where the disease is not prevalent. Greater population mobility in recent years makes recognition of surgical manifestations of the disease and awareness of perioperative management of sickle cell patients undergoing surgical interventions of paramount importance. This article aims to summarize steps towards safer surgery in patients with SCD. (author)

  13. Significant variations in Weber fraction for changes in inter-onset interval of a click train over the range of intervals between 5 and 300 ms.

    Science.gov (United States)

    Ungan, Pekcan; Yagcioglu, Suha

    2014-01-01

    It is a common psychophysical experience that a train of clicks faster than ca. 30/s is heard as one steady sound, whereas temporal patterns occurring on a slower time scale are perceptually resolved as individual auditory events. This phenomenon suggests the existence of two different neural mechanisms for processing of auditory sequences with fast and slow repetition rates. To test this hypothesis we used Weber's law, which is known to be valid for perception of time intervals. Discrimination thresholds and Weber fractions (WFs) for 12 base inter-click intervals (ICIs) between 5 and 300 ms were measured from 10 normal hearing subjects by using an "up-down staircase" algorithm. The mean WF, which is supposed to be constant for any perceptual mechanism according to Weber's law, displayed significant variation with click rate. WFs decreased sharply from an average value of around 5% at repetition rates below 20 Hz to about 0.5% at rates above 67 Hz. Parallel to this steep transition, subjects reported that at rates below 20 Hz they perceived periodicity as a fast tapping rhythm, whereas at rates above 50 Hz the perceived quality was a pitch. Such a dramatic change in WF indicated the existence of two separate mechanisms for processing the click rate for long and short ICIs, based on temporal and spectral features, respectively. A range of rates between 20 and 33 Hz, in which the rate discrimination threshold was maximum, appears to be a region where both of the presumed time and pitch mechanisms are relatively insensitive to rate alterations. Based on this finding, we speculate that the interval-based perception mechanism ceases to function at around 20 Hz and the spectrum-based mechanism takes over at around 33 Hz; leaving a transitional gap in between, where neither of the two mechanisms is as sensitive. Another notable finding was a significant drop in WF for ICI = 100 ms, suggesting a connection of time perception to the electroencephalography alpha rhythm.

  14. Ultrasonographic Features of Papillary Thyroid Carcinoma in Patients with Graves' Disease

    Science.gov (United States)

    Chung, Jin Ook; Cho, Dong Hyeok; Chung, Dong Jin

    2010-01-01

    Background/Aims To characterize ultrasonographic findings in papillary thyroid carcinoma (PTC) combined with Graves' disease. Methods Medical records and ultrasonographic findings of 1,013 patients with Graves' disease and 3,380 patients without Graves' disease were analyzed retrospectively. A diagnosis of PTC was based on a pathologic examination. Results The frequency of hypoechogenicity was lower in patients with PTC and Graves' disease than in patients with PTC alone (p Graves' disease was significantly higher than in those with PTC alone (p Graves' disease was characterized by more ill-defined borders and less frequency of overall calcification, punctate calcification, and heterogeneous echogenicity, although the difference was not statistically significant. Conclusions Our results suggest that patients with Graves' disease more frequently have atypical PTC findings on ultrasonography. PMID:20195406

  15. LOCAL ANESTHETICS IN PATIENTS WITH CARDIOVASCULAR DISEASES.

    Directory of Open Access Journals (Sweden)

    risto Daskalov

    2015-03-01

    Full Text Available A significant problem in the dental medicine is pain alleviation. Many studies in the dental anesthesiology result in the production of new agents for locoregional anesthesia. Objective: This article aim to present the results of the last studies on the effect of the local anesthetics used in the oral surgery on patients with cardiovascular diseases. Material: A general review of the existing literature on the effect of the adrenaline, included as vasoconstrictor in the local anesthetics, used in patients with cardiovascular diseases is made. The benefits of vasoconstrictors for the quality of the anesthetic effect are proven. Conclusion: A small amount of adrenaline in the anesthetic solution does not result in complications development in patients with controlled cardiovascular diseases. Articaine is recommended agent of first choice for local anesthesia in the oral surgery.

  16. The debate between Weber and Sombart on the Protestant ethic and the development of capitalism: Author Antikritiken as footnote

    Directory of Open Access Journals (Sweden)

    Mariachiara Spagnolo

    2015-03-01

    Full Text Available In 1904/05 the first edition of Weber’s Protestant Ethics was issued, where the thematic framing is ‘uncertain’ and not yet completed: religion is linked to a certain kind of ethos (of the economic system and of ethic (rational, the ethic of «ascetic Protestantism». Around the same years, Sombart outlines the expository path contained in Modern Capitalism, a detailed «picture of the general economic development», which seems to be a ‘more serious’ work – for its content and set of problems – when compared to the research conducted by his friend and colleague Max Weber. Both scholars view the question of the rise and development of capitalism as a rhythmic research: from the historic origins of the phenomenon, to the elaboration, synthesis and change of the economic process that becomes an autonomous and corrosive force in the modern stage. In the Sombartian discourse, however, the particular ‘attitude’ of the Protestant ethic as analysed by Weber can be regarded at most as one of the possible forms of heresy that justifies a certain approach (moral/punitive to money – and not as the only movement which has roused and regulated the economic or capitalistic processes in the strict meaning of economic surplus, profitable and to be reinvested following entrepreneurial logics. Whereas, what the two sociologists have in common is not only the use of sources (almost identical, but the very concept of Geist and individual predisposition of the subjects who accept the modes of action of this ordering spirit that gives an ethical/psychological boost to men endowed with charisma or with particular energy.

  17. Neurological symptoms in patients with biopsy proven celiac disease.

    Science.gov (United States)

    Bürk, Katrin; Farecki, Marie-Louise; Lamprecht, Georg; Roth, Guenter; Decker, Patrice; Weller, Michael; Rammensee, Hans-Georg; Oertel, Wolfang

    2009-12-15

    In celiac disease (CD), the gut is the typical manifestation site but atypical neurological presentations are thought to occur in 6 to 10% with cerebellar ataxia being the most frequent symptom. Most studies in this field are focused on patients under primary neurological care. To exclude such an observation bias, patients with biopsy proven celiac disease were screened for neurological disease. A total of 72 patients with biopsy proven celiac disease (CD) (mean age 51 +/- 15 years, mean disease duration 8 +/- 11 years) were recruited through advertisements. All participants adhered to a gluten-free diet. Patients were interviewed following a standard questionnaire and examined clinically for neurological symptoms. Medical history revealed neurological disorders such as migraine (28%), carpal tunnel syndrome (20%), vestibular dysfunction (8%), seizures (6%), and myelitis (3%). Interestingly, 35% of patients with CD reported of a history of psychiatric disease including depression, personality changes, or even psychosis. Physical examination yielded stance and gait problems in about one third of patients that could be attributed to afferent ataxia in 26%, vestibular dysfunction in 6%, and cerebellar ataxia in 6%. Other motor features such as basal ganglia symptoms, pyramidal tract signs, tics, and myoclonus were infrequent. 35% of patients with CD showed deep sensory loss and reduced ankle reflexes in 14%. Gait disturbances in CD do not only result from cerebellar ataxia but also from proprioceptive or vestibular impairment. Neurological problems may even develop despite strict adherence to a gluten-free diet. (c) 2009 Movement Disorder Society.

  18. Using Electronic Patient Records to Discover Disease Correlations and Stratify Patient Cohorts

    DEFF Research Database (Denmark)

    Roque, Francisco S.; Jensen, Peter B.; Schmock, Henriette

    2011-01-01

    Electronic patient records remain a rather unexplored, but potentially rich data source for discovering correlations between diseases. We describe a general approach for gathering phenotypic descriptions of patients from medical records in a systematic and non-cohort dependent manner. By extracting...... phenotype information from the free-text in such records we demonstrate that we can extend the information contained in the structured record data, and use it for producing fine-grained patient stratification and disease co-occurrence statistics. The approach uses a dictionary based on the International...

  19. Socioeconomic status in HCV infected patients – risk and prognosis

    Directory of Open Access Journals (Sweden)

    Oml

    2013-05-01

    Full Text Available Lars Haukali Omland,1 Merete Osler,2 Peter Jepsen,3,4 Henrik Krarup,5 Nina Weis,6 Peer Brehm Christensen,7 Casper Roed,1 Henrik Toft Sørensen,3 Niels Obel1 On behalf of the DANVIR Cohort Study1Department of Infectious Diseases, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark; 2Research Center for Prevention and Health, Copenhagen University Hospital, Glostrup Hospital, Glostrup, Denmark; 3Department of Clinical Epidemiology, Aarhus University Hospital, Aarhus, Denmark; 4Department of Medicine V (Hepatology and Gastroenterology, Aarhus University Hospital, Aarhus, Denmark; 5Department of Clinical Biochemistry, Aalborg Hospital, Aalborg, Denmark; 6Department of Infectious Diseases, Copenhagen University Hospital, Hvidovre Hospital, Hvidovre, Denmark; 7Department of Infectious Diseases, Odense University Hospital, Odense, DenmarkBackground and aims: It is unknown whether socioeconomic status (SES is a risk factor for hepatitis C virus (HCV infection or a prognostic factor following infection.Methods: From Danish nationwide registries, we obtained information on three markers of SES: employment, income, and education. In a case control design, we examined HCV infected patients and controls; conditional logistic regression was employed to obtain odds ratios (ORs for HCV infection for each of the three SES markers, adjusting for the other two SES markers, comorbidity, and substance abuse. In a cohort design, we used Cox regression analysis to compute mortality rate ratios (MRRs for each of the three SES markers, adjusting for the other two SES markers, comorbidity level, age, substance abuse, and gender.Results: When compared to employed persons, ORs for HCV infection were 2.71 (95% confidence interval [CI]: 2.24–3.26 for disability pensioners and 2.24 (95% CI: 1.83–2.72 for the unemployed. When compared to persons with a high income, ORs were 1.64 (95% CI: 1.34–2.01 for low income persons and 1.19 (95% CI: 1.02–1.40 for

  20. Dental considerations in patients with heart disease

    OpenAIRE

    Cruz Pamplona, Marta; Jiménez Soriano, Yolanda; Sarrión Pérez, María Gracia

    2011-01-01

    Summary: Cardiovascular diseases are one of the main causes of death in the developed world, and represent the first cause of mortality in Spain. In addition to their associated morbidity, such disorders are important due to the number of affected individuals and the many patients subjected to treatment because of them. Objective: An update is provided on the oral manifestations seen in patients with arterial hypertension, ischemic heart disease, arrhythmias and heart failure, and...

  1. Celiac disease, iron deficiency anaemia, grave's disease, osteopenia and short stature in single patient

    International Nuclear Information System (INIS)

    Radaideh, A.M.

    2015-01-01

    Celiac disease is an intestinal immune mediated disorder, triggered by ingestion of gluten-containing diet in genetically susceptible individuals. The genetic pre-disposition is related to human leukocyte antigen (HLA) class II genes, especially HLA-DQ2 positive patients. The prevalence of celiac disease in high worldwide and it has been estimated to be 1-26% in Western countries. Many auto-immune diseases can be associated with celiac disease including auto-immune thyroid disease; hashimoto thyroiditis and grave's disease. The opposite also appears to be true, celiac disease is found on persons with auto-immune thyroid disorders at high rates than the general population. Celiac disease is also associated with other extraintestinal diseases other the auto-immune diseases like anemia, short stature, metabolic bone disease and others. Screening for celiac disease should be considered in patients with auto-immune thyroid disease, anemia, short stature and metabolic bone disease. The life-long adherence to gluten-free diet is the only cure in celiac disease and can improve the quality of patients life and prevent future complications. This report describes a case of Grave's disease, Iron deficiency anemia, Short stature, Osteopenia, diagnosed to have Celiac disease. (author)

  2. Evaluation of Asymptomatic Peripheral Arterial Disease by Ankle-brachial Index in Patients with Concomitant Coronary Arterial Disease

    Directory of Open Access Journals (Sweden)

    Hosein Vakili

    2012-12-01

    Full Text Available Background: Peripheral arterial disease is associated with adverse cardiovascular outcomes. As such, it is found that screening for peripheral arterial disease (PAD improves risk assessment. Thus, intensive risk factor modification and medical treatment in these patients are necessary. Objectives: The purpose of this study was to determine the prevalence of asymptomatic peripheral arterial disease in patients with concomitant coronary arterial disease. Methods: Asymptomatic peripheral arterial disease was investigated in 400 patients (60% males, 40% females, aged 59.7± 11.3 with a documented coronary arterial disease. Results: Among patients with documented CAD, 12% had asymptomatic PAD with the ABI ratio of less than 0.9. Conclusions: It is advisable to screen for PAD not only as a disease but also as a risk assessment method for atherosclerosis.

  3. Mortality in patients with Parkinson's disease

    DEFF Research Database (Denmark)

    Wermuth, L; Stenager, E; Stenager, E

    1995-01-01

    INTRODUCTION: After the introduction of L-dopa the mortality rate in Parkinson's disease (PD) patients has changed, but is still higher than in the background population. MATERIAL & METHODS: Mortality, age at death and cause of death in a group of PD patients compared with the background population...

  4. Cannabis use amongst patients with inflammatory bowel disease.

    Science.gov (United States)

    Lal, Simon; Prasad, Neeraj; Ryan, Manijeh; Tangri, Sabrena; Silverberg, Mark S; Gordon, Allan; Steinhart, Hillary

    2011-10-01

    Experimental evidence suggests the endogenous cannabinoid system may protect against colonic inflammation, leading to the possibility that activation of this system may have a therapeutic role in inflammatory bowel disease (IBD). Medicinal use of cannabis for chronic pain and other symptoms has been reported in a number of medical conditions. We aimed to evaluate cannabis use in patients with IBD. One hundred patients with ulcerative colitis (UC) and 191 patients with Crohn's disease (CD) attending a tertiary-care outpatient clinic completed a questionnaire regarding current and previous cannabis use, socioeconomic factors, disease history and medication use, including complimentary alternative medicines. Quality of life was assessed using the short-inflammatory bowel disease questionnaire. A comparable proportion of UC and CD patients reported lifetime [48/95 (51%) UC vs. 91/189 (48%) CD] or current [11/95 (12%) UC vs. 30/189 (16%) CD] cannabis use. Of lifetime users, 14/43 (33%) UC and 40/80 (50%) CD patients have used it to relieve IBD-related symptoms, including abdominal pain, diarrhoea and reduced appetite. Patients were more likely to use cannabis for symptom relief if they had a history of abdominal surgery [29/48 (60%) vs. 24/74 (32%); P=0.002], chronic analgesic use [29/41 (71%) vs. 25/81 (31%); Pmedicine use [36/66 (55%) vs. 18/56 (32%); P=0.01] and a lower short inflammatory bowel disease questionnaire score (45.1±2.1 vs. 50.3±1.5; P=0.03). Patients who had used cannabis [60/139 (43%)] were more likely than nonusers [13/133 (10%); Pcannabis for IBD. Cannabis use is common amongst patients with IBD for symptom relief, particularly amongst those with a history of abdominal surgery, chronic abdominal pain and/or a low quality of life index. The therapeutic benefits of cannabinoid derivatives in IBD may warrant further exploration.

  5. Non-Motor Symptoms in Patients Suffering from Motor Neuron Diseases.

    Science.gov (United States)

    Günther, René; Richter, Nicole; Sauerbier, Anna; Chaudhuri, Kallol Ray; Martinez-Martin, Pablo; Storch, Alexander; Hermann, Andreas

    2016-01-01

    The recently postulated "disease spreading hypothesis" has gained much attention, especially for Parkinson's disease (PD). The various non-motor symptoms (NMS) in neurodegenerative diseases would be much better explained by this hypothesis than by the degeneration of disease-specific cell populations. Motor neuron disease (MND) is primarily known as a group of diseases with a selective loss of motor function. However, recent evidence suggests disease spreading into non-motor brain regions also in MND. The aim of this study was to comprehensively detect NMS in patients suffering from MND. We used a self-rating questionnaire including 30 different items of gastrointestinal, autonomic, neuropsychiatric, and sleep complaints [NMS questionnaire (NMSQuest)], which is an established tool in PD patients. 90 MND patients were included and compared to 96 controls. In total, MND patients reported significantly higher NMS scores (median: 7 points) in comparison to controls (median: 4 points). Dribbling, impaired taste/smelling, impaired swallowing, weight loss, loss of interest, sad/blues, falling, and insomnia were significantly more prevalent in MND patients compared to controls. Interestingly, excessive sweating was more reported in the MND group. Correlation analysis revealed an increase of total NMS score with disease progression. NMS in MND patients seemed to increase with disease progression, which would fit with the recently postulated "disease spreading hypothesis." The total NMS score in the MND group significantly exceeded the score for the control group, but only 8 of the 30 single complaints of the NMSQuest were significantly more often reported by MND patients. Dribbling, impaired swallowing, weight loss, and falling could primarily be connected to motor neuron degeneration and declared as motor symptoms in MND.

  6. Vitamin D deficiency in patients with either rheumatic diseases or inflammatory bowel diseases on biologic therapy.

    Science.gov (United States)

    Bruzzese, Vincenzo; Zullo, Angelo; Picchianti Diamanti, Andrea; Ridola, Lorenzo; Lorenzetti, Roberto; Marrese, Cinzia; Scolieri, Palma; De Francesco, Vincenzo; Hassan, Cesare; Migliore, Alberto; Laganà, Bruno

    2016-09-01

    Vitamin D deficiency has been reported in patients with chronic inflammatory conditions, such as rheumatic and inflammatory bowel diseases (IBD). We evaluated the role of biologic therapy on vitamin D, calcium and parathormone (PTH) levels. This cross-sectional study enrolled consecutive patients with either rheumatic diseases or IBD who underwent an ambulatory visit. Patients receiving vitamin D/calcium supplementation were excluded. Vitamin D deficiency or insufficiency was diagnosed when values were rheumatic disease (M/F 37/99; mean age 60.7 ± 12.9 years) and 64 with IBD (M/F 41/23; Mean age 49.6 ± 13.1 years) were enrolled. Vitamin D deficiency/insufficiency was detected in as many as 63.5 % patients, being 61.8 and 67.2 % in patients with either rheumatic diseases or IBD, respectively. The prevalence of vitamin D deficiency/insufficiency was higher in those receiving biologics than other therapies (78.3 vs 43.2 %; p rheumatic diseases (78.7 vs 41 %; p rheumatic diseases or IBD receiving a biologic therapy.

  7. Patient-Centered Medical Home in chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    Ortiz G

    2011-10-01

    Full Text Available Gabriel Ortiz1, Len Fromer21Pediatric Pulmonary Services, El Paso, TX; 2Department of Family Medicine, David Geffen School of Medicine, UCLA, Los Angeles, CA, USAAbstract: Chronic obstructive pulmonary disease (COPD is a progressive and debilitating but preventable and treatable disease characterized by cough, phlegm, dyspnea, and fixed or incompletely reversible airway obstruction. Most patients with COPD rely on primary care practices for COPD management. Unfortunately, only about 55% of US outpatients with COPD receive all guideline-recommended care. Proactive and consistent primary care for COPD, as for many other chronic diseases, can reduce hospitalizations. Optimal chronic disease management requires focusing on maintenance rather than merely acute rescue. The Patient-Centered Medical Home (PCMH, which implements the chronic care model, is a promising framework for primary care transformation. This review presents core PCMH concepts and proposes multidisciplinary team-based PCMH care strategies for COPD.Keywords: Patient-Centered Medical Home, chronic care model, chronic obstructive pulmonary disease, patient education, physician assistants, nurse practitioners

  8. Survival Analysis of Patients with End Stage Renal Disease

    Science.gov (United States)

    Urrutia, J. D.; Gayo, W. S.; Bautista, L. A.; Baccay, E. B.

    2015-06-01

    This paper provides a survival analysis of End Stage Renal Disease (ESRD) under Kaplan-Meier Estimates and Weibull Distribution. The data were obtained from the records of V. L. MakabaliMemorial Hospital with respect to time t (patient's age), covariates such as developed secondary disease (Pulmonary Congestion and Cardiovascular Disease), gender, and the event of interest: the death of ESRD patients. Survival and hazard rates were estimated using NCSS for Weibull Distribution and SPSS for Kaplan-Meier Estimates. These lead to the same conclusion that hazard rate increases and survival rate decreases of ESRD patient diagnosed with Pulmonary Congestion, Cardiovascular Disease and both diseases with respect to time. It also shows that female patients have a greater risk of death compared to males. The probability risk was given the equation R = 1 — e-H(t) where e-H(t) is the survival function, H(t) the cumulative hazard function which was created using Cox-Regression.

  9. Pain Correlates with Sleep Disturbances in Parkinson's Disease Patients.

    Science.gov (United States)

    Fu, Yun-Ting; Mao, Cheng-Jie; Ma, Li-Jing; Zhang, Hui-Jun; Wang, Yi; Li, Jie; Huang, Jun-Ying; Liu, Jun-Yi; Liu, Chun-Feng

    2018-01-01

    Both sleep disorders and pain decrease quality of life in patients with Parkinson's disease (PD). However, little is known about the relationship between objective sleep disturbances and pain in patients with PD. This study aimed to (1) examine the clinical characteristics of pain in PD patients and (2) explore the correlation between pain and sleep disturbances in PD patients. Parkinson's disease patients (N = 144) underwent extensive clinical evaluations of motor and nonmotor symptoms and characteristics of pain. Overnight video-polysomnography was also conducted. Clinical characteristics and sleep parameters were compared between PD patients with or without pain. Pain was reported by 75 patients (52.1%), with 49 (65.3%) reporting pain of at least moderate severity. PD patients with pain were older and had longer disease duration, more severe PD symptoms as assessed by Hoehn and Yahr stage and the Unified Parkinson's Disease Rating Scale, and higher L-dopa equivalent daily dose compared with PD patients without pain. PD patients with pain also showed significantly decreased sleep efficiency (57.06% ± 15.84% vs. 73.80% ± 12.00%, P daily living, depressed mood, higher percentage of N1 sleep, and lower sleep efficiency were independent predictors of pain in patients with PD. Musculoskeletal pain is the most common type of pain in patients with PD. Disrupted sleep continuity, altered sleep architecture, depressed mood, and compromised activities of daily living may be associated with pain in patients with PD. © 2017 World Institute of Pain.

  10. Disease management programs for CKD patients: the potential and pitfalls.

    Science.gov (United States)

    Rocco, Michael V

    2009-03-01

    Disease management describes the use of a number of approaches to identify and treat patients with chronic health conditions, especially those that are expensive to treat. Disease management programs have grown rapidly in the United States in the past several years. These programs have been established for patients with chronic kidney disease (CKD), but some have been discontinued because of the high cost of the program. Disease management programs for CKD face unique challenges. Identification of patients with CKD is hampered by incomplete use of the International Classification of Diseases, Ninth Revision (ICD-9) codes for CKD by physicians and the less than universal use of estimated glomerular filtration rate from serum creatinine measurements to identify patients with an estimated glomerular filtration rate less than 60 mL/min/1.73 m(2). CKD affects multiple organ systems. Thus, a comprehensive disease management program will need to manage each of these aspects of CKD. These multiple interventions likely will make a CKD disease management program more costly than similar disease management programs designed for patients with diabetes mellitus, congestive heart failure, or other chronic diseases. The lack of data that can be used to develop effective disease management programs in CKD makes it difficult to determine goals for the management of each organ system affected by CKD. Finally, long periods of observation will be needed to determine whether a particular disease management program is effective in not only improving patient outcomes, but also decreasing both resource use and health care dollars. This long-term observation period is contrary to how most disease management contracts are written, which usually are based on meeting goals during a 1- to 3-year period. Until these challenges are resolved, it likely will be difficult to maintain effective disease management programs for CKD.

  11. Sindrome de Sturge – Weber - Os síndromes raros também existem

    Directory of Open Access Journals (Sweden)

    Cátia Canelas

    2015-12-01

    Recorreu ao serviço de urgência por crises convulsivas tónico-clónicas generalizadas quase diárias desde há 1 mês. Ao exame físico o doente apresentava-se pouco colaborante mas hemodinamicamente estável. Ao nível da cabeça eram evidentes diversas alterações: macrocefalia, hipertrofia dos tecidos moles da face com manchas em vinho do porto exuberantes bilaterais com distribuição oftálmica, maxilar e mandibular do nervo trigémio, e hemangiomas conjuntivais bilaterais (Fig. 1. Ao exame neurológico apresentava hemiparesia esquerda grau 3/5 em ambos os membros. Realizou tomografia computorizada crânio-encefálica que mostrou atrofia do hemisfério encefálico direito com calcificação cortical de configuração giriforme de predomínio frontal e occipital e maior volume do plexo coroide do ventrículo lateral direito (Fig.2, aspetos descritos na literatura como característicos de SSW4. Analiticamente sem alterações nomeadamente da função tiroideia. O doente foi observado por oftalmologia tendo sido diagnosticado glaucoma bilateral com perda visual acentuada. Trata-se então de um caso de síndrome de Sturge-Weber tipo I (angiomas da face e das leptomeninges e glaucoma segundo a The Roach Scale, que por falta de seguimento médico apresentava as diversas complicações da síndrome não controladas, convulsões frequentes, perda visual acentuada, história de episódios semelhantes a AVC com hemiparésia sequelar, cefaleias, défice cognitivo e isolamento social. Apesar da forma de apresentação grave com sintomas desde a infância o diagnóstico foi estabelecido numa idade muito pouco comum. A síndrome de Sturge-Weber é uma entidade a ter presente no diagnóstico diferencial das manchas vinho do porto e das crises convulsivas. O diagnóstico precoce e o seguimento/vigilância médica apertada são as únicas formas de melhorar o prognóstico sombrio das manifestações e complicações desta síndrome que são verdadeiramente limitadoras de

  12. Disease-specific self-efficacy in spasmodic dysphonia patients.

    Science.gov (United States)

    Hu, Amanda; Isetti, Derek; Hillel, Allen D; Waugh, Patricia; Comstock, Bryan; Meyer, Tanya K

    2013-03-01

    Self-efficacy (SE) is an optimistic self-belief that one can perform a novel task. This concept involves empowerment, self-esteem, and adaptation to a stressful situation. SE is a strong predictor of health behaviors. Our objectives were to study SE in spasmodic dysphonia (SD) and to develop a disease-specific SE-SD scale. Prospective study. Academic hospital. Disease-specific SE-SD items were developed with laryngologists, speech pathologists, and SD patients. These items, General SE Scale, Voice Handicap Index-10 (VHI-10), Consensus Auditory Perceptual Evaluation of Voice (CAPE-V), and Hospital Anxiety and Depression Scale (HADS), were administered to SD patients who presented for botulinum toxin injections. One hundred forty-five SD patients (mean age 59.5 ± 13.6 years) had a general SE score (Cronbach's α = 0.894) of 33.4 ± 5.2 out of 40. This was negatively correlated with HADS-A (r = -0.42, P < 0.001) and HADS-D (r = -0.42, P < .001), but not correlated with VHI-10 (r = -0.098, P = .243) and CAPE-V (r = -0.047, P = .57). Factor analysis selected 8 items from the general SE scale and 5 disease-specific SE-SD items to generate a 13-item disease-specific SE-SD scale (Cronbach's α = 0.907). Disease-specific SE-SD score was 42.1 ± 6.9 out of 52 and was negatively correlated with VHI-10 (r = -0.19, P = .005), HADS-A (r = -0.43, P < .001), and HADS-D (r = -0.57, P < .001), but not correlated with CAPE-V (r = -0.024, P = .60). SD patients established on botulinum toxin injections have high degrees of general and disease-specific SE. Patients with higher SE-SD demonstrate lower vocal handicap and lower levels of anxiety and depression. A 13-item disease-specific SE-SD scale has been developed.

  13. Endocrine Abnormalities in Patients with Chronic Kidney Disease.

    Science.gov (United States)

    Kuczera, Piotr; Adamczak, Marcin; Wiecek, Andrzej

    2015-01-01

    In patients with chronic kidney disease the alterations of the endocrine system may arise from several causes. The kidney is the site of degradation as well as synthesis of many different hormones. Moreover, a number of concomitant pathological conditions such as inflammation, metabolic acidosis and malnutrition may participate in the pathogenesis of endocrine abnormalities in this group of patients. The most pronounced endocrine abnormalities in patients with chronic kidney disease are the deficiencies of: calcitriol, testosterone, insulin-like growth factor and, erythropoietin (EPO). Additionally accumulation of several hormones, such as: prolactin, growth hormone and insulin frequently also occur. The clinical consequences of the abovementioned endocrine abnormalities are among others: anemia, infertility and bone diseases.

  14. Quality of patients life with celiac disease

    OpenAIRE

    Bičíková, Michaela

    2016-01-01

    This thesis is dealing with the quality of life of patients with celiac disease. In the theoretical part, I am presenting the characteristics of celiac disease and I am describing its history briefly. For better orientation in the issue, I also joined a description of the anatomy of the small intestine and pathophysiology of celiac disease. In this thesis I am also dealing with incidence of disease, etiology and risk factors participating in the development of celiac disease, symptoms and the...

  15. [Subclinical and established kidney disease in recently diagnosed hypertensive patients].

    Science.gov (United States)

    Gómez-Marcos, Manuel Angel; Martínez-Salgado, Carlos; Grandes, Gonzalo; Recio-Rodríguez, José Ignacio; Castaño Sánchez, Yolanda; Rodríguez Sánchez, Emiliano; García-Ortiz, Luis

    2010-03-06

    To estimate renal disease in recently diagnosed hypertensive patients, and to identify factors related to renal disease. Cross-sectional study, with 425 hypertensive patients recently diagnosed in primary health care; renal disease was estimated with serum creatinine, albumin/creatinine index and glomerular filtration rate (GFR). We analyzed cardiovascular risk factors (CRF), subclinical organ injury and cardiovascular disease following the criteria of the 2007 European Guide of Hypertension. Average age: 58,96 +/- 12,73 years old, 63,3% male. We found dyslipemia in 80%, abdominal obesity in 49% and metabolic syndrome in 36% patients. These patients showed increased serum creatinine 3,3%, a reduction in GFR 9,6%, hidden renal disease 6,4%, microalbuminuria 7,5% and nephropathy 2,4%. Hypertensive patients with renal disease (17,88%) were older, with higher systolic pressure, higher incidence of metabolic syndrome, abnormal carotid intima-media thickness and ankle-arm index, and presence of cardiovascular disease. Variables associated with renal disease were metabolic syndrome (odds ratio = 11,12) and ankle-arm index (odds ratio = 17,55). Variables related to creatinina were sex, ankle-arm index and metabolic syndrome; variables related to GFR were sex, age, ankle-arm index, metabolic syndrome and body mass index (BMI); variables related with albumin/creatinine index included diabetes mellitus. Renal disease is detected in about 2 out of 10 hypertensive patients, when, besides serum creatinina, we analyze albumin/creatinine index and GFR. Metabolic syndrome and ankle-arm index are the main variables associated with renal disease. Copyright 2009 Elsevier España, S.L. All rights reserved.

  16. Role of hepatic resection for patients with carcinoid heart disease

    DEFF Research Database (Denmark)

    Bernheim, A.M.; Connolly, H.M.; Rubin, J.

    2008-01-01

    OBJECTIVE: To evaluate the effects of resection of hepatic carcinoid metastases on progression and prognosis of carcinoid heart disease. PATIENTS AND METHODS: From our database of 265 consecutive patients diagnosed as having carcinoid heart disease from January 1, 1980, through December 31, 2005...... nonrandomized study, our data suggest that patients with carcinoid heart disease who undergo hepatic resection have decreased cardiac progression and improved prognosis. Eligible patients should be considered for hepatic surgery Udgivelsesdato: 2008/2...

  17. INTESTINAL PERMEABILITY IN PATIENTS WITH CELIAC-DISEASE AND RELATIVES OF PATIENTS WITH CELIAC-DISEASE

    NARCIS (Netherlands)

    van Elburg, R. M.; Uil, J. J.; Mulder, C. J.; Heymans, H. S.

    1993-01-01

    The functional integrity of the small bowel is impaired in coeliac disease. Intestinal permeability, as measured by the sugar absorption test probably reflects this phenomenon. In the sugar absorption test a solution of lactulose and mannitol was given to the fasting patient and the

  18. INTESTINAL PERMEABILITY IN PATIENTS WITH CELIAC-DISEASE AND RELATIVES OF PATIENTS WITH CELIAC-DISEASE

    NARCIS (Netherlands)

    VANELBURG, RM; UIL, JJ; MULDER, CJJ; HEYMANS, HSA

    The functional integrity of the small bowel is impaired in coeliac disease. Intestinal permeability, as measured by the sugar absorption test probably reflects this phenomenon. In the sugar absorption test a solution of lactulose and mannitol was given to the fasting patient and the

  19. rCBF SPECT in Parkinson's disease patients with mental dysfunction

    International Nuclear Information System (INIS)

    Bissessur, S.; Tissingh, G.; Wolters, E.C.; Scheltens, P.

    1997-01-01

    Functional imaging of the brain using SPECT provides information correlative to the alterations of regional blood flow. In this paper we review the literature pertaining to SPECT in Parkinson's disease with and without dementia and depression. Parkinson's disease itself is not associated with a consistent pattern of cerebral blood flow alterations in the basal ganglia, but reduced parietal blood flow is more often reported. The heterogeneity of blood flow changes possibly reflects the multifactorial pathophysiology of the disease. In demented Parkinson's disease patients frontal hypoperfusion is often found or bilateral temporoparietal deficits, probably indicative of concomitant Alzheimer's disease. The SPECT studies undertaken in depressed patients with and without Parkinson's disease show highly conflicting and inconsistent results, probably due to methodological and diagnostic flaws (especially the inclusion of demented Parkinson patients). Several lines of reasoning point to a prefrontal dysfunction and future SPECT studies are planned to study this region in non-demented Parkinson's disease patients with and without major depression. (author)

  20. Kleptomania in patients with neuro-Behçet's disease.

    Science.gov (United States)

    Shugaiv, Erkingül; Kıyat-Atamer, Aslı; Tüzün, Erdem; Kürtüncü, Murat; Baral-Kulaksızoğlu, Işın; Akman Demir, Gülşen

    2013-01-01

    This study was conducted to characterize the prevalence and clinical features of kleptomania, an impulse control disorder, in patients with Behçet's disease involving the central nervous system. Medical records of 350 patients with neuro-Behçet's disease were evaluated, and clinical and neuropsychological features of patients with kleptomania were noted. Of the 350 neuro-Behçet's disease patients 6 (1.7%) had presented with symptoms that fulfilled the criteria of kleptomania according to the revised 4th version of the Diagnostic and Statistical Manual of Mental Disorders. The 6 patients (5 men, 1 woman) had parenchymal lesions and had developed kleptomania during remission. Magnetic resonance imaging done on the 6 patients before the onset of kleptomania mostly revealed brainstem lesions. Psychiatric assessment did not show any comorbid psychiatric disorders and neuropsychological evaluation showed executive dysfunction in all patients. The 6 patients with kleptomania had developed a frontal lobe syndrome. Copyright © 2013 S. Karger AG, Basel.

  1. Hematologic Abnormalities in Cyanotic Congenital Heart Disease Patients

    Directory of Open Access Journals (Sweden)

    Soheila Chamanian

    2015-01-01

    Full Text Available Introduction: Patients with cyanotic heart disease may have an acceptable quality of life. However, they are invariably prone to several complications. The aim of this study is search about hematologic abnormalities in cyanotic congenital heart disease patients. Materials and Methods:  In this cross sectional study every cyanotic congenital heart disease patients who was referred to the adult congenital heart disease clinic was selected and asked of any possible hyperviscosity symptoms, gingival bleeding, Epistaxis, hemoptysis, hypermenorrhagia and gouty arthritis irrespective of their age, gender and primary diagnosis in a six-month period. In this regard, 02 saturation was obtained via pulse oximetry, an abdominal ultrasound was done in order to discover any gallstones and lab tests including CBC, coagulation parameters (bleeding time(BT,clotting time(CT, prothrombin time(PT,international ratio( INR, Ferritin, blood urea nitrogen (BUN and creatinine (Cr were provided as well. Results:  A total of 69 patients were enrolled in the present study. The mean age of the patients was 22.44±5.72 with a minimum of 15 and the maximum of 46 years old. Twenty two (34.4% of them were female and 45(65.6% were male. Conclusion: Our patients had less hyperuricemia, there is no correlation between hyperviscosity symptoms and haematocrit level and an inverse correlation between the Ferritin level and hyperviscosity symptoms were seen.  

  2. Asymmetrical Pedaling Patterns in Parkinson's Disease Patients

    Science.gov (United States)

    Penko, Amanda L.; Hirsch, Joshua R.; Voelcker-Rehage, Claudia; Martin, Philip E.; Blackburn, Gordon; Alberts, Jay L.

    2015-01-01

    Background Approximately 1.5 million Americans are affected by Parkinson's disease [1] which includes the symptoms of postural instability and gait dysfunction. Currently, clinical evaluations of postural instability and gait dysfunction consist of a subjective rater assessment of gait patterns using items from the Unified Parkinson's Disease Rating Scale, and assessments can be insensitive to the effectiveness of medical interventions. Current research suggests the importance of cycling for Parkinson's disease patients, and while Parkinson's gait has been evaluated in previous studies, little is known about lower extremity control during cycling. The purpose of this study is to examine the lower extremity coordination patterns of Parkinson's patients during cycling. Methods Twenty five participants, ages 44-72, with a clinical diagnosis of idiopathic Parkinson's disease participated in an exercise test on a cycle ergometer that was equipped with pedal force measurements. Crank torque, crank angle and power produced by right and left leg were measured throughout the test to calculate Symmetry Index at three stages of exercise (20 Watt, 60 Watt, maximum performance). Findings Decreases in Symmetry Index were observed for average power output in Parkinson's patients as workload increased. Maximum power Symmetry Index showed a significant difference in symmetry between performance at both the 20 Watt and 60 Watt stage and the maximal resistance stage. Minimum power Symmetry Index did not show significant differences across the stages of the test. While lower extremity asymmetries were present in Parkinson's patients during pedaling, these asymmetries did not correlate to postural instability and gait dysfunction Unified Parkinson's Disease Rating Scale scores. Interpretation This pedaling analysis allows for a more sensitive measure of lower extremity function than the Unified Parkinson's Disease Rating Scale and may help to provide unique insight into current and

  3. Suicidal behavior among Turkish patients with Parkinson’s disease

    Directory of Open Access Journals (Sweden)

    Ozdilek B

    2014-03-01

    Full Text Available Betul Ozdilek,1 Bulent Kadri Gultekin21Department of Neurology, 2Department of Psychiatry, Erenkoy Mental Health and Neurology Training and Research Hospital, Istanbul, TurkeyObjective: To investigate the predictors of suicidal ideation and attempts among Turkish Parkinson’s disease (PD patients.Materials and methods: The study comprised 120 patients with PD. Clinical findings were obtained by using the Unified Parkinson’s Disease Rating Scale. Disease severity was measured by the Hoehn and Yahr staging scale, and the Schwab and England Activities of Daily Living scale was used for patient disability. Psychiatric evaluation was performed by the same psychiatrist using the Structured Clinical Interview for Diagnostic and Statistical Manual of Mental Disorders (DSM-IV Axis I Disorders. Severity of depression was measured with the Hamilton Depression Rating Scale. Suicidal ideation and attempts were considered positive if experienced during the patient’s lifetime. The Suicide Probability Scale was used to assess the risk of suicide. Data were analyzed by logistic regression models to identify variables associated with suicidal ideation and attempts.Results: Based on logistic regression analysis, education level, age of disease onset, disease duration, depression, and history of impulse-control disorder (ICD behaviors were significant predictors of suicidal ideation. The risk rate in the presence of depression and history of ICD behaviors was increased by 5.92 and 4.97, respectively. Additionally, lifetime prevalence of suicidal ideation was found in 11.6% (14 of 120 of PD patients, although no patient had ever attempted suicide.Conclusion: Turkish patients with PD who exhibit a high risk for suicidal ideation also experience disease starting at an earlier age, longer disease duration, presence of depression, and ICD behaviors, and should be monitored carefully.Keywords: Parkinson’s disease, suicidal behavior, risk factors

  4. Preprohypocretin polymorphisms in Parkinson disease patients reporting "sleep attacks".

    Science.gov (United States)

    Rissling, Ida; Körner, Yvonne; Geller, Frank; Stiasny-Kolster, Karin; Oertel, Wolfgang H; Möller, J Carsten

    2005-07-01

    Previously, we found a significant association between the dopamine D2 receptor gene polymorphism Taq IA and sudden onset of sleep in patients with Parkinson disease. Here we evaluated the association between the preprohypocretin (-909T/C), (-22C/T), and (-20C/A) polymorphisms and sudden onset of sleep in the same population of patients with Parkinson disease. We conducted an association study analyzing the distribution of preprohypocretin polymorphisms in Germanic, caucasian Parkinson disease patients with and without sudden onset of sleep, matched according to drug therapy, disease duration, sex, and age. Movement disorders section at a university hospital. 132 Parkinson disease patients with sudden onset of sleep and 132 Parkinson disease patients without sudden onset of sleep. Blood samples were taken from each participant and used for DNA extraction. Polymorphisms were analyzed by established polymerase chain reaction protocols or direct sequencing. The variant allele T of the (-909T/C) preprohypocretin polymorphism was more commonly found in Parkinson disease patients with sudden onset of sleep. Statistical analysis showed that there were significant differences in the genotype (P = .024) and allele (P = .018) distribution between both groups. For heterozygous and homozygous carriers of allele T, the genotype relative-risk estimates for the presence of sudden onset of sleep were 2.01 (95% confidence interval: 0.76-5.34) and 2.81 (95% confidence interval: 1.09-7.25), respectively. Our results show a significant association between the (-909T/C) preprohypocretin polymorphism and sudden onset of sleep in Parkinson disease. However, we could not demonstrate any interaction between the Taq IA and (-909T/C) polymorphisms with respect to the occurrence of sudden onset of sleep, suggesting that multiple genetic factors may contribute to the pathogenesis of this phenomenon.

  5. Celiac disease and pulmonary hemosiderosis in a patient with chronic granulomatous disease

    NARCIS (Netherlands)

    Hartl, Dominik; Belohradsky, Bernd H.; Griese, Matthias; Nicolai, Thomas; Krauss-Etschmann, Susanne; Roos, Dirk; Wintergerst, Uwe

    2004-01-01

    We report on a patient with the hitherto undescribed combination of chronic granulomatous disease, pulmonary hemosiderosis, and celiac disease. The hemosiderosis resolved with a gluten-free diet and glucocorticosteroid pulse therapy, but the restrictive lung function pattern remained unchanged. Lung

  6. Prevalence of chronic kidney disease among patients undergoing transradial percutaneous coronary interventions.

    Science.gov (United States)

    Hossain, Mohammad A; Quinlan, Amy; Heck-Kanellidis, Jennifer; Calderon, Dawn; Patel, Tejas; Gandhi, Bhavika; Patel, Shrinil; Hetavi, Mahida; Costanzo, Eric J; Cosentino, James; Patel, Chirag; Dewan, Asa; Kuo, Yen-Hong; Salman, Loay; Vachharajani, Tushar J

    2018-03-01

    While transradial approach to conduct percutaneous coronary interventions offers multiple advantages, the procedure can cause radial artery damage and occlusion. Because radial artery is the preferred site for the creation of an arteriovenous fistula to provide dialysis, patients with chronic kidney disease are particularly dependent on radial artery for their long-term survival. In this retrospective study, we investigated the prevalence of chronic kidney disease in patients undergoing coronary interventions via radial artery. Stage of chronic kidney disease was based on estimated glomerular filtration rate and National Kidney Foundation - Kidney Disease Outcomes Quality Initiative guidelines. A total of 497 patients undergoing transradial percutaneous coronary interventions were included. Over 70.4% (350/497) of the patients had chronic kidney disease. Stage II chronic kidney disease was observed in 243 (69%) patients (estimated glomerular filtration rate = 76.0 ± 8.4 mL/min). Stage III was observed in 93 (27%) patients (estimated glomerular filtration rate = 49 ± 7.5 mL/min). Stage IV chronic kidney disease was observed in 5 (1%) patients (estimated glomerular filtration rate = 25.6 ± 4.3 mL/min) and Stage V chronic kidney disease was observed in 9 (3%) patients (estimated glomerular filtration rate = 9.3 ± 3.5 mL/min). Overall, 107 of 350 patients (30%) had advanced chronic kidney disease, that is, stage III-V chronic kidney disease. Importantly, 14 of the 107 (13%) patients had either stage IV or V chronic kidney disease. This study finds that nearly one-third of the patients undergoing transradial percutaneous coronary interventions have advanced chronic kidney disease. Because many of these patients may require dialysis, the use of radial artery to conduct percutaneous coronary interventions must be carefully considered in chronic kidney disease population.

  7. Periodontal disease and inflammatory blood cytokines in patients with stable coronary artery disease

    Directory of Open Access Journals (Sweden)

    Cassio KAMPITS

    Full Text Available ABSTRACT Periodontal disease has been associated with elevations of blood cytokines involved in atherosclerosis in systemically healthy individuals, but little is known about this association in stable cardiovascular patients. The aim of this study was to assess the association between periodontal disease (exposure and blood cytokine levels (outcomes in a target population of patients with stable coronary artery disease (CAD. Material and Methods This cross-sectional study included 91 patients with stable CAD who had been under optimized cardiovascular care. Blood levels of IL-1β, IL-6, IL-8, IL-10, IFN-γ, and TNF-α were measured by Luminex technology. A full-mouth periodontal examination was conducted to record probing depth (PD and clinical attachment (CA loss. Multiple linear regression models, adjusting for gender, body mass index, oral hypoglycemic drugs, smoking, and occurre:nce of acute myocardial infarction were applied. Results CAD patients that experienced major events had higher concentrations of IFN-γ (median: 5.05 pg/mL vs. 3.01 pg/mL; p=0.01, IL-10 (median: 2.33 pg/mL vs. 1.01 pg/mL; p=0.03, and TNF-α (median: 9.17 pg/mL vs. 7.47 pg/mL; p=0.02. Higher numbers of teeth with at least 6 mm of CA loss (R2=0.07 and PD (R2=0.06 were significantly associated with higher IFN-γ log concentrations. Mean CA loss (R2=0.05 and PD (R2=0.06 were significantly related to IL-10 concentrations. Elevated concentrations of TNF-α were associated with higher mean CA loss (R2=0.07. Conclusion Periodontal disease is associated with increased systemic inflammation in stable cardiovascular patients. These findings provide additional evidence supporting the idea that periodontal disease can be a prognostic factor in cardiovascular patients.

  8. Rationale for Using Social Media to Collect Patient-Reported Outcomes in Patients with Celiac Disease.

    Science.gov (United States)

    Park, Kt; Harris, Merissa; Khavari, Nasim; Khosla, Chaitan

    2014-02-01

    Patients with celiac disease (CD) are increasingly interconnected through social media, exchanging patient experiences and health-tracking information between individuals through various web-based platforms. Social media represents potentially unique communication interface between gastroenterologists and active social media users - especially young adults and adolescents with celiac disease-regarding adherence to the strict gluten-free diet, gastrointestinal symptoms, and meaningful discussion about disease management. Yet, various social media platforms may be underutilized for research purposes to collect patient-reported outcomes data. In this commentary, we summarize the scientific rationale and potential for future growth of social media in patient-reported outcomes research, focusing on college freshmen with celiac disease as a case study and provide overview of the methodological approach. Finally, we discuss how social media may impact patient care in the future through increasing mobile technology use.

  9. Clinical impact of exercise in patients with peripheral arterial disease.

    Science.gov (United States)

    Novakovic, Marko; Jug, Borut; Lenasi, Helena

    2017-08-01

    Increasing prevalence, high morbidity and mortality, and decreased health-related quality of life are hallmarks of peripheral arterial disease. About one-third of peripheral arterial disease patients have intermittent claudication with deleterious effects on everyday activities, such as walking. Exercise training improves peripheral arterial disease symptoms and is recommended as first line therapy for peripheral arterial disease. This review examines the effects of exercise training beyond improvements in walking distance, namely on vascular function, parameters of inflammation, activated hemostasis and oxidative stress, and quality of life. Exercise training not only increases walking distance and physiologic parameters in patients with peripheral arterial disease, but also improves the cardiovascular risk profile by helping patients achieve better control of hypertension, hyperglycemia, obesity and dyslipidemia, thus further reducing cardiovascular risk and the prevalence of coexistent atherosclerotic diseases. American guidelines suggest supervised exercise training, performed for a minimum of 30-45 min, at least three times per week, for at least 12 weeks. Walking is the most studied exercise modality and its efficacy in improving cardiovascular parameters in patients with peripheral arterial disease has been extensively proven. As studies have shown that supervised exercise training improves walking performance, cardiovascular parameters and quality of life in patients with peripheral arterial disease, it should be encouraged and more often prescribed.

  10. [Metronome therapy in patients with Parkinson disease].

    Science.gov (United States)

    Enzensberger, W; Oberländer, U; Stecker, K

    1997-12-01

    We studied 10 patients with Parkinson's disease and 12 patients with Parkinson-plus-syndrome, trying to improve patients' gait by application of various external rhythmic stimuli, including metronome stimulation (96 beats per minute = middle andante). The test course of the patients was 4 x 10 meters and 3 U-turns. The patients' gait quality under stimulation was compared with their free walk (velocity, number of steps, number of freezing episodes). Metronome stimulation significantly reduced the time and number of steps needed for the test course and also diminished the number of freezing episodes. March music stimulation was less effective and tactile stimulation (rhythmically tapping on the patient's shoulder) even produced negative results. The positive effect of metronome stimulation was also found, when the tests were not performed inside the hospital building, but outside in the hospital parc. Metronome stimulation was comparably effective in both patient sub-groups examined in this study (M. Parkinson, Parkinson-plus-syndrome) and seems to be an important additional help in the treatment of these patients. Electronical metronomes are not expensive, easy in handling, and portable. A theoretical explanation of metronome stimulation effectivity in patients with Parkinson's disease still needs to be elucidated.

  11. Bone marrow transplantation in patients with storage diseases: a developing country experience

    Directory of Open Access Journals (Sweden)

    Lange Marcos C.

    2006-01-01

    Full Text Available Bone marrow transplantation (BMT is a therapeutic option for patients with genetic storage diseases. Between 1979 and 2002, eight patients, four females and four males (1 to 13 years old were submitted to this procedure in our center. Six patients had mucopolysaccharidosis (MPS I in 3; MPS III in one and MPS VI in 2, one had adrenoleukodystrophy (ALD and one had Gaucher disease. Five patients had related and three unrelated BMT donor. Three patients developed graft versus host disease (two MPS I and one MPS VI and died between 37 and 151 days after transplantation. Five patients survived 4 to 16 years after transplantation. Three patients improved (one MPS I; one MPS VI and the Gaucher disease patient, one patient had no disease progression (ALD and in one patient this procedure did not change the natural course of the disease (MPS III.

  12. Fructan active enzymes (FAZY) activities and biosynthesis of fructooligosaccharides in the vacuoles of Agave tequilana Weber Blue variety plants of different age.

    Science.gov (United States)

    Mellado-Mojica, Erika; González de la Vara, Luis E; López, Mercedes G

    2017-02-01

    Biosynthesis of agave fructans occurs in mesontle vacuoles which showed fluctuations in FAZY activities and synthesized a diverse spectrum of fructooligosaccharide isomers. Agave tequilana Weber Blue variety is an important agronomic crop in Mexico. Fructan metabolism in A. tequilana exhibits changes in fructan content, type, degree of polymerization (DP), and molecular structure. Specific activities of vacuolar fructan active enzymes (FAZY) in A. tequilana plants of different age and the biosynthesis of fructooligosaccharides (FOSs) were analyzed in this work. Vacuoles from mesontle (stem) protoplasts were isolated and collected from 2- to 7-year-old plants. For the first time, agave fructans were identified in the vacuolar content by HPAEC-PAD. Several FAZY activities (1-SST, 6-SFT, 6G-FFT, 1-FFT, and FEH) with fluctuations according to the plant age were found in protein vacuolar extracts. Among vacuolar FAZY, 1-SST activities appeared in all plant developmental stages, as well as 1-FFT and FEH activities. The enzymes 6G-FFT and 6-SST showed only minimal activities. Lowest and highest FAZY activities were found in 2- and 6-year-old plants, respectively. Synthesized products (FOS) were analyzed by TLC and HPAEC-PAD. Vacuolar FAZYs yielded large FOS isomers diversity, being 7-year-old plants the ones that synthesized a greater variety of fructans with different DP, linkages, and molecular structures. Based on the above, we are proposing a model for the FAZY activities constituting the FOS biosynthetic pathways in Agave tequilana Weber Blue variety.

  13. Disease awareness and management behavior of patients with atopic dermatitis: a questionnaire survey of 313 patients.

    Science.gov (United States)

    Kim, Jung Eun; Lee, Young Bok; Lee, Ji Hyun; Kim, Hye Sung; Lee, Kyung Ho; Park, Young Min; Cho, Sang Hyun; Lee, Jun Young

    2015-02-01

    Patients with atopic dermatitis (AD) should be relatively well informed about the disorder to control their condition and prevent flare-ups. Thus far, there is no accurate information about the disease awareness levels and therapeutic behavior of AD patients. To collect data on patients' knowledge about AD and their behavior in relation to seeking information about the disease and its treatment. We performed a questionnaire survey on the disease awareness and self-management behavior of AD patients. A total of 313 patients and parents of patients with AD who had visited the The Catholic University of Korea, Catholic Medical Center between November 2011 and October 2012 were recruited. We compared the percentage of correct answers from all collected questionnaires according to the demographic and disease characteristics of the patients. Although dermatologists were the most frequent disease information sources and treatment providers for the AD patients, a significant proportion of participants obtained information from the Internet, which carries a huge amount of false medical information. A considerable number of participants perceived false online information as genuine, especially concerning complementary and alternative medicine treatments of AD, and the adverse effects of steroids. Some questions on AD knowledge had significantly different answers according to sex, marriage status, educational level, type of residence and living area, disease duration, disease severity, and treatment history with dermatologists. Dermatologists should pay more attention to correcting the common misunderstandings about AD to reduce unnecessary social/economic losses and improve treatment compliance.

  14. Enhanced thyroid iodine metabolism in patients with triiodothyronine-predominant Graves' disease

    Energy Technology Data Exchange (ETDEWEB)

    Takamatsu, J.; Hosoya, T.; Naito, N.; Yoshimura, H.; Kohno, Y.; Tarutani, O.; Kuma, K.; Sakane, S.; Takeda, K.; Mozai, T.

    1988-01-01

    Some patients with hyperthyroid Graves' disease have increased serum T3 and normal or even low serum T4 levels during treatment with antithyroid drugs. These patients with elevated serum T3 to T4 ratios rarely have a remission of their hyperthyroidism. The aim of this study was to investigate thyroid iodine metabolism in such patients, whom we termed T3-predominant Graves' disease. Mean thyroid radioactive iodine uptake was 51.0 +/- 18.1% ( +/- SD) at 3 h, and it decreased to 38.9 +/- 20.1% at 24 h in 31 patients with T3-predominant Graves' disease during treatment. It was 20.0 +/- 11.4% at 3 h and increased to 31.9 +/- 16.0% at 24 h in 17 other patients with hyperthyroid Graves' disease who had normal serum T3 and T4 levels and a normal serum T3 to T4 ratio during treatment (control Graves' disease). The activity of serum TSH receptor antibodies was significantly higher in the patients with T3-predominant Graves' disease than in control Graves' disease patients. From in vitro studies of thyroid tissue obtained at surgery, both thyroglobulin content and iodine content in thyroglobulin were significantly lower in patients with T3-predominant Graves' disease than in the control Graves' disease patients. Thyroid peroxidase (TPO) activity determined by a guaiacol assay was 0.411 +/- 0.212 g.u./mg protein in the T3-predominant Graves' disease patients, significantly higher than that in the control Graves' disease patients. Serum TPO autoantibody levels determined by immunoprecipitation also were greater in T3-predominant Graves' disease patients than in control Graves' disease patients. Binding of this antibody to TPO slightly inhibited the enzyme activity of TPO, but this effect of the antibody was similar in the two groups of patients.

  15. Vagus nerve stimulation in patients with Alzheimer's disease

    DEFF Research Database (Denmark)

    Merrill, Charley A; Jonsson, Michael A G; Minthon, Lennart

    2006-01-01

    BACKGROUND: Cognitive-enhancing effects of vagus nerve stimulation (VNS) have been reported during 6 months of treatment in a pilot study of patients with Alzheimer's disease (AD). Data through 1 year of VNS (collected from June 2000 to September 2003) are now reported. METHOD: All patients (N = 17......) met the National Institute of Neurological and Communicative Disorders and Stroke and the Alzheimer's Disease and Related Disorders Association (NINCDS-ADRDA) criteria for probable AD. Responder rates for the Alzheimer's Disease Assessment Scale-cognitive subscale (ADAS-cog) and Mini-Mental State...

  16. Ecos da contribuição de Guerreiro Ramos para a divulgação da obra de Weber no campo de estudos organizacionais (EOs

    Directory of Open Access Journals (Sweden)

    Leonor Moreira Camara

    2012-02-01

    Full Text Available Em 1946, Guerreiro Ramos publica a resenha "A sociologia de Max Weber - sua importância para a teoria e a prática da administração", na Revista do Serviço Público - RSP. Nela, mais do que apresentar ao leitor a obra de Weber Economia e sociedade, com foco em sua metodologia, também procura demonstrar sua aplicabilidade no trato das questões da administração pública e sua relevância num país "culturalmente colonial" como o Brasil. A considerar as especificidades da produção de conhecimento no campo de estudos organizacionais e o fato de Guerreiro Ramos expressar, no ensaio, uma visão que não é a que prevaleceu no campo, mas que é fiel à sociologia compreensiva e à metodologia ideal-típica, cabe aqui o esforço de reiterar o referido trabalho de Guerreiro Ramos a partir da proposição de uma discussão de caráter teórico conceitual com base no conteúdo resenhado relacionado à construção metodológica da crítica ideal-típica.

  17. Dynamic contrast-enhanced MRI in patients with luminal Crohn's disease

    International Nuclear Information System (INIS)

    Ziech, M.L.W.; Lavini, C.; Caan, M.W.A.; Nio, C.Y.; Stokkers, P.C.F.; Bipat, S.; Ponsioen, C.Y.; Nederveen, A.J.; Stoker, J.

    2012-01-01

    Objectives: To prospectively assess dynamic contrast-enhanced (DCE-)MRI as compared to conventional sequences in patients with luminal Crohn's disease. Methods: Patients with Crohn's disease undergoing MRI and ileocolonoscopy within 1 month had DCE-MRI (3T) during intravenous contrast injection of gadobutrol, single shot fast spin echo sequence and 3D T1-weighted spoiled gradient echo sequence, a dynamic coronal 3D T1-weighted fast spoiled gradient were performed before and after gadobutrol. Maximum enhancement (ME) and initial slope of increase (ISI) were calculated for four colon segments (ascending colon + coecum, transverse colon, descending colon + sigmoid, rectum) and (neo)terminal ileum. C-reactive protein (CRP), Crohn's disease activity index (CDAI), per patient and per segment Crohn's disease endoscopic index of severity (CDEIS) and disease duration were determined. Mean values of the (DCE-)MRI parameters in each segment from each patient were compared between four disease activity groups (normal mucosa, non-ulcerative lesions, mild ulcerative and severe ulcerative disease) with Mann–Whitney test with Bonferroni adjustment. Spearman correlation coefficients were calculated for continuous variables. Results: Thirty-three patients were included (mean age 37 years; 23 females, median CDEIS 4.4). ME and ISI correlated weakly with segmental CDEIS (r = 0.485 and r = 0.206) and ME per patient correlated moderately with CDEIS (r = 0.551). ME was significantly higher in segments with mild (0.378) or severe (0.388) ulcerative disease compared to normal mucosa (0.304) (p < 0.001). No ulcerations were identified at conventional sequences. ME correlated with disease duration in diseased segments (r = 0.492), not with CDAI and CRP. Conclusions: DCE-MRI can be used as a method for detecting Crohn's disease ulcerative lesions.

  18. Osteoprotegerin and mortality in hemodialysis patients with cardiovascular disease

    DEFF Research Database (Denmark)

    Winther, Simon; Christensen, Jeppe Hagstrup; Flyvbjerg, Allan

    2013-01-01

    Abstract BACKGROUND: Patients treated with hemodialysis (HD) have an increased mortality, mainly caused by cardiovascular disease (CVD). Osteoprotegerin (OPG) is a glycoprotein involved in the regulation of the vascular calcification process. Previous studies have demonstrated that OPG.......08; in the adjusted analyses, the p-value for trend was 0.03. CONCLUSIONS: In a high-risk population of hemodialysis patients with previously documented cardiovascular disease, a high level of OPG was an independent risk marker of all-cause mortality....... is a prognostic marker of mortality. The aim of this study was to investigate if OPG was a prognostic marker of all-cause mortality in high-risk patients with end-stage renal disease and CVD. METHODS: We prospectively followed 206 HD patients with CVD. OPG was measured at baseline and the patients were followed...

  19. Gastroparesis in patients with inactive Crohn's disease: a case series

    Directory of Open Access Journals (Sweden)

    Oyen Wim JG

    2007-03-01

    Full Text Available Abstract Background Few studies have described patients with foregut dysmotility in inflammatory bowel disease. The aim of this case series was to evaluate clinical characteristics of 5 patients with inflammatory bowel disease and symptoms and signs of upper gut dysmotility. Case presentations We describe a series of four patients with Crohn's disease and one with indeterminate colitis who presented with severe symptoms and signs of gastroparesis. We reviewed medical records of all cases. Gastric emptying of a solid meal was assessed by scintigraphy. Small bowel enteroclysis, gastroduodenoscopy and colonoscopy with biopsies were performed to estimate the activity of the disease and to exclude organic obstruction. None of the patients had any signs of active inflammation or stricture. All of the patients had markedly delayed gastric emptying with a mean t 1/2 of 234 minutes (range 110–380 minutes; normal values 54–94 minutes. Conclusion Clinicians should consider impaired gastric emptying when evaluating patients with Crohn's disease and severe symptoms of upper gut dysmotility, which cannot be attributed to active inflammation or organic obstruction of the digestive tract. Symptoms in these patients are refractory to various therapeutic interventions including tube feeding and gastric surgery.

  20. Improving the quality of care of patients with rheumatic disease using patient-centric electronic redesign software.

    Science.gov (United States)

    Newman, Eric D; Lerch, Virginia; Billet, Jon; Berger, Andrea; Kirchner, H Lester

    2015-04-01

    Electronic health records (EHRs) are not optimized for chronic disease management. To improve the quality of care for patients with rheumatic disease, we developed electronic data capture, aggregation, display, and documentation software. The software integrated and reassembled information from the patient (via a touchscreen questionnaire), nurse, physician, and EHR into a series of actionable views. Core functions included trends over time, rheumatology-related demographics, and documentation for patient and provider. Quality measures collected included patient-reported outcomes, disease activity, and function. The software was tested and implemented in 3 rheumatology departments, and integrated into routine care delivery. Post-implementation evaluation measured adoption, efficiency, productivity, and patient perception. Over 2 years, 6,725 patients completed 19,786 touchscreen questionnaires. The software was adopted for use by 86% of patients and rheumatologists. Chart review and documentation time trended downward, and productivity increased by 26%. Patient satisfaction, activation, and adherence remained unchanged, although pre-implementation values were high. A strong correlation was seen between use of the software and disease control (weighted Pearson's correlation coefficient 0.5927, P = 0.0095), and a relative increase in patients with low disease activity of 3% per quarter was noted. We describe innovative software that aggregates, stores, and displays information vital to improving the quality of care for patients with chronic rheumatic disease. The software was well-adopted by patients and providers. Post-implementation, significant improvements in quality of care, efficiency of care, and productivity were demonstrated. Copyright © 2015 by the American College of Rheumatology.