3,019 research outputs found
Acute neonatal arterial occlusion: is thrombolysis safe and effective?
No full textPurposeWe report our experience of the management of arterial occlusion in the newborn.MethodsA case note review was carried out after ethical approval. Doppler ultrasonography confirmed the occlusion. Thrombolysis was the primary intervention. Surgery was used selectively. A good outcome was one without tissue loss or functional impairment or minimal tissue loss without functional impairment. Data are presented as medians with ranges.ResultsTen patients (9 male; median gestational age, 35.5 weeks [range, 28-39 weeks]) presented on day 1 (range, 1-8 days). Initial management included systemic tissue plasminogen activator (8 patients) and surgery (2 infants in whom thrombolysis was contraindicated). Improvement was noted in 7 of 8 infants treated medically and in both who underwent surgery. Three infants had significant tissue loss. Outcome at 29 months (range, 1.3-95.4 months) was good in the remaining 7.ConclusionsA multidisciplinary approach, thrombolysis and selective surgery achieved tissue preservation and function in the majority while minimizing complications. Early referral to centers with multidisciplinary teams is recommended
Diagnosis and treatment of factor VIII and IX inhibitors in congenital haemophilia: (4th edition). UK Haemophilia Centre Doctors Organization
No full textIncidence of factor VIII inhibitors throughout life in severe hemophilia A in the United Kingdom
No full textAbstract
The age-adjusted incidence of new factor VIII inhibitors was analyzed in all United Kingdom patients with severe hemophilia A between 1990 and 2009. Three hundred fifteen new inhibitors were reported to the National Hemophilia Database in 2528 patients with severe hemophilia who were followed up for a median (interquartile range) of 12 (4-19) years. One hundred sixty (51%) of these arose in patients ≥ 5 years of age after a median (interquartile range) of 6 (4-11) years' follow-up. The incidence of new inhibitors was 64.29 per 1000 treatment-years in patients < 5 years of age and 5.31 per 1000 treatment-years at age 10-49 years, rising significantly (P = .01) to 10.49 per 1000 treatment-years in patients more than 60 years of age. Factor VIII inhibitors arise in patients with hemophilia A throughout life with a bimodal risk, being greatest in early childhood and in old age. HIV was associated with significantly fewer new inhibitors. The inhibitor incidence rate ratio in HIV-seropositive patients was 0.32 times that observed in HIV-seronegative patients (P < .001). Further study is required to explore the natural history of later-onset factor VIII inhibitors and to investigate other potential risk factors for inhibitor development in previously treated patients.</jats:p
A subjetividade jurídica internacional na história da doutrina brasileira: oposições entre Nação e Estado nos séculos XIX e XX
No full textTCC(graduação) - Universidade Federal de Santa Catarina. Centro de Ciências Jurídicas. Direito.Com o objetivo de observar de que maneira a pergunta “quem é o sujeito de direitos na comunidade internacional” foi respondida, ressaltando as oposições entre Nação e Estado, o presente trabalho investigou o pensamento de Pasquale Stanislao Mancini, descrevendo como que, utilizando por fundamento do direito internacional o Princípio da Nacionalidade, o jurista italiano propõe a exclusividade da Nação como sujeito de direito internacional, em oposição ao Estado e de que maneira isto influenciou os autores da escola italiana de direito internacional, em especial Terenzio Mamiami e Pasquale Fiore. A seguir, adentra-se na doutrina brasileira da segunda metade do século XIX para verificar de que forma se deu a oposição entre os conceitos de Nação e Estado. Posteriormente, debruçando-se por sobre o pensamento de Lafayette Rodrigues Pereira e Clóvis Beviláqua, busca-se demonstrar como se tratou a problemática da subjetividade jurídica internacional no Brasil durante o início do século XX. Finalmente, verifica-se qual termo passou a ser adotado majoritariamente pela doutrina brasileira, até se encontrar um autor nacional que não mais defenda o exclusivismo de um ente político em específico, mas, consciente das diversas configurações históricas pelas quais passou a comunidade internacional, aceite uma pluralidade de sujeitos. Busca-se, ainda, ao longo de todo trabalho reforçar as continuidades e rupturas entre os diversos autores analisados, além de se proporem, quando cabíveis, críticas às concepções apresentadas.In order to observe how the question “who is the subject of rights in the international community” was answered, focusing on the contrasts between Nation and State, the present study investigated the thought of Pasquale Stanislao Mancini, describing how, using the Principle of Nationality as the basis of international law, the Italian jurist proposes the exclusivity of the Nation as a subject of international law in opposition to the State and how it influenced the authors of the Italian school of international law, especially Terenzio Mamiami and Pasquale Fiore. Hereafter, enter in the Brazilian doctrine of the second half of the nineteenth century to verify how occurred the opposition between the concepts of Nation and State. Posteriorly, looking at the thought of Lafayette Rodrigues Pereira and Clóvis Beviláqua, we seek to demonstrate how the problem of international juridical subjectivity was treated in Brazil during the early twentieth century. Finally, we verify which term was mostly adopted by Brazilian doctrine, until find a national author who no longer defends the exclusivity of a political entity in specific, but, aware of different historical settings of the international community, accepts a plurality of subjects. We also try throughout the study to highlight the continuities and changes between the various authors analyzed, and to propose, as appropriate, critical to the concepts presented
Modern management of severe platelet function disorders
No full textSevere platelet function defects are rare disorders that require expertise in diagnosis and management. Therefore patients with such disorders should be referred to and managed in centres with the full laboratory repertoire of tests and clinical support necessary to optimise their quality of care. The aim of this review is to discuss the management of these patients in various clinical situations including surgical intervention.</p
Factor VIII brand and the incidence of factor VIII inhibitors in previously untreated UK children with severe hemophilia A, 2000-2011
No full textThe effect of recombinant factor VIII (rFVIII) brand on inhibitor development was investigated in all 407 severe hemophilia A previously untreated patients born in the United Kingdom (UK) between 1 January 2000 and 31 December 2011. Eighty-eight (22%) had been in the RODIN study. Information was extracted from the National Haemophilia Database. Because exposure days (EDs) were not known for some patients, time from first treatment was used as a surrogate for rFVIII exposure. An inhibitor developed in 118 (29%) patients, 60 high and 58 low titer, after a median (interquartile range) of 7.8 (3.3-13.5) months from first exposure and 16 (9-30) EDs. Of 128 patients treated with Kogenate Bayer/Helixate NexGen, 45 (35.2%, 95% confidence interval [CI] 27.4-43.8) developed an inhibitor compared with 42/172 (24.4%, 95% CI 18.6% to 31.4%) with Advate (P = .04). The adjusted hazard ratio (HR) (95% CI) for Kogenate Bayer/Helixate NexGen compared with Advate was 2.14 (1.12-4.10) (P = .02) for high titer and 1.75 (1.11-2.76) (P = .02) for all inhibitors. When excluding UK-RODIN patients, the adjusted HR (95% CI) for high-titer inhibitors was 2.00 (0.93-4.34) (P = .08). ReFacto AF was associated with a higher incidence of all, but not high-titer, inhibitors than Advate. These results will help inform debate around the relative immunogenicity and use of rFVIII brands.</p
Supervision of DPD RI in The Implementation of Regional Autonomy
No full textIn a bicameral system, Regional Representative Council of Indonesia (Dewan Perwakilan Daerah Republik Indonesia/DPD RI) acts as a regional representative institution that fights for regional problems. The authority of DPD RI is to supervise implementation of the law. The supervisory functions of the DPD RI are to monitor and supervise the government and public institutions, including the implementation of laws, regulations and policies. In this study the authors used a qualitative approach. The purpose of this study is to see the extent of supervision carried out by DPD RI on the implementation of regional autonomy carried out by Committee I. The author used strategies, analysis and relevant and important sources related to the supervisory function of the DPD RI on the implementation of regional autonomy. The author found that the authority of supervision and follow-up of the results of DPD RI's supervision on the implementation of regional autonomy is still very limited, there are supervisory problems starting from the relationship between DPD RI and the central government and local governments, human resources and supporting resources are inadequate, and the level of public confidence in DPD RI decreases
PARALLEL RI-MP2 ENERGIES AND GRADIENTS: IMPLEMENTATION AND APPLICATIONS
No full textAuthor Institution: Northeast Parallel Architectures Center, Syracuse UniversityThe so-called ``resolution of the identity'' integral approximation applied to second-order many-body perturbation theory (RI-MP2) is a method well-suited to the treatment of large chemical systems at a much lower computational cost than conventional MP2 calculations. In addition, features of the RI-MP2 method make it more efficient to implement on parallel computers than the conventional MP2 approach, an important advantage in pushing in limits of large-scale correlated calculations. I will describe the RI-MP2 method itself and the implementation of both energies and gradients, emphasizing the important differences from the conventional MP2 as well as the parallelism. I will discuss fitting basis sets, introduced by the RI approximation, and present results from several RI-MP2 applications, including energy calculations on systems of more than 2400 AO basis functions, which run an estimated 30 times faster than the equivalent conventional MP2 calculation
Assessing thrombogenesis and treatment response in congenital thrombotic thrombocytopenic purpura
No full textDespite clinical remission and normal platelet counts, congenital TTP (cTTP) is associated with non-overt symptoms. Prophylactic ADAMTS13 replacement therapy such as plasma infusion (PI) prevents acute episodes and improves symptomatology. There is no current method to investigate disease severity or monitor the impact of treatment. We utilize a dynamic high shear flow assay to further understand disease pathophysiology and determine the impact of cTTP on symptomatology and therapy, despite normal platelet counts. Whole blood, under high shear, was run over collagen-coated channels, causing platelet adhesion to von Willebrand factor (VWF) multimers. The resulting surface coverage by platelet-VWF thrombus was assessed. The normal range was 6-39% in 50 controls. Twenty-two cTTP patients with normal platelet counts were evaluated. Median pre-treatment surface coverage was 89%, and PI reduced coverage to a median of 44% (p = 0.0005). Patients taking antiplatelets had further reduced coverage when combined with PI and improved non-overt symptoms such as headache, lethargy, and abdominal pain in 100% of patients compared to 74% with PI alone (p = 0.046). We use a dynamic assay to report increased in vitro platelet adhesion and aggregation and additionally demonstrate significantly decreased thrombi following PI, with levels in the normal range levels achieved in patients taking additional antiplatelet therapy
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