994 resultados para NOONAN-LIKE SYNDROME


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Through the influenza virus surveillance from January to October 2002, influenza B/Hong Kong-like strains circulating in the Southeast and Centre East regions of Brazil have been demonstrated. This strain is a variant from B/Victoria/02/88 whose since 1991 and until recently have been isolated relatively infrequently and have been limited to South-Eastern Asia. A total of 510 respiratory secretions were collected from patients 0 to 60 years of age, with acute respiratory illness, living in the Southeast and Centre East regions of Brazil, of which 86 (17.13%) were positive for influenza virus. Among them 12 (13.95%) were characterized as B/Hong Kong/330/2001; 3 (3.49%) as B/Hong Kong/1351/2002 a variant from B/Hong Kong/330/2001; 1 (1.16%) as B/Sichuan/379/99; 1 (1.16%) as B/Shizuoka/5/2001, until now. The percentages of cases notified during the surveillance period were 34.88%, 15.12%, 15.12%, 4.65%, 15.12%, 13.95%, in the age groups of 0-4, 5-10, 11-15, 16-20, 21-30, 31-50, respectively. The highest proportion of isolates was observed among children younger than 4 years but serious morbidity and mortality has not been observed among people older than 65 years, although B influenza virus component for vaccination campaign 2002 was B/Sichuan/379/99 strain. This was probably due to the elderly protection acquired against B/Victoria/02/88. In addition, in influenza A/Panama/2007/99-like (H3N2) strains 22 (25.58%) were also detected, but influenza A(H1N1) has not been detected yet.

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ABSTRACT - The authors’ main purpose is to present ideas on defining Health Law by highlighting the particularities of the field of Health Law as well as of the teaching of this legal branch, hoping to contribute to the maturity and academic recognition of Health Law, not only as a very rich legal field but also as a powerful social instrument in the fulfillment of fundamental human rights. The authors defend that Health Law has several characteristics that distinguish it from traditional branches of law such as its complexity and multidisciplinary nature. The study of Health Law normally covers issues such as access to care, health systems organization, patients’ rights, health professionals’ rights and duties, strict liability, healthcare contracts between institutions and professionals, medical data protection and confidentiality, informed consent and professional secrecy, crossing different legal fields including administrative, antitrust, constitutional, contract, corporate, criminal, environmental, food and drug, intellectual property, insurance, international and supranational, labor/employment, property, taxation, and tort law. This is one of the reasons why teaching Health Law presents a challenge to the teacher, which will have to find the programs, content and methods appropriate to the profile of recipients which are normally non jurists and the needs of a multidisciplinary curricula. By describing academic definitions of Health Law as analogous to Edgewood, a fiction house which has a different architectural style in each of its walls, the authors try to describe which elements should compose a more comprehensive definition. In this article Biolaw, Bioethics and Human Rights are defined as complements to a definition of Health Law: Biolaw because it is the legal field that treats the social consequences that arise from technological advances in health and life sciences; Bioethics which evolutions normally influence the shape of the legal framework of Health; and, finally Human Rights theory and declarations are outlined as having always been historically linked to medicine and health, being the umbrella that must cover all the issues raised in the area of Health Law. To complete this brief incursion on the definition on Health Law the authors end by giving note of the complex relations between this field of Law and Public Health. Dealing more specifically on laws adopted by governments to provide important health services and regulate industries and individual conduct that affect the health of the populations, this aspect of Health Law requires special attention to avoid an imbalance between public powers and individual freedoms. The authors conclude that public trust in any health system is essentially sustained by developing health structures which are consistent with essential fundamental rights, such as the universal right to access health care, and that the study of Health Law can contribute with important insights into both health structures and fundamental rights in order to foster a health system that respects the Rule of Law.-------------------------- RESUMO – O objectivo principal dos autores é apresentar ideias sobre a definição de Direito da Saúde, destacando as particularidades desta área do direito, bem como do ensino deste ramo jurídico, na esperança de contribuir para a maturidade e para o reconhecimento académico do mesmo, não só como um campo juridicamente muito rico, mas, também, como um poderoso instrumento social no cumprimento dos direitos humanos fundamentais. Os autores defendem que o Direito da Saúde tem diversas características que o distinguem dos ramos tradicionais do direito, como a sua complexidade e natureza multidisciplinar. O estudo do Direito da Saúde abrangendo normalmente questões como o acesso aos cuidados, a organização dos sistemas de saúde, os direitos e deveres dos doentes e dos profissionais de saúde, a responsabilidade civil, os contratos entre instituições de saúde e profissionais, a protecção e a confidencialidade de dados clínicos, o consentimento informado e o sigilo profissional, implica uma abordagem transversal de diferentes áreas legais, incluindo os Direitos contratual, administrativo, antitrust, constitucional, empresarial, penal, ambiental, alimentar, farmacêutico, da propriedade intelectual, dos seguros, internacional e supranacional, trabalho, fiscal e penal. Esta é uma das razões pelas quais o ensino do Direito da Saúde representa um desafio para o professor, que terá de encontrar os programas, conteúdos e métodos adequados ao perfil dos destinatários, que são normalmente não juristas e às necessidades de um currículo multidisciplinar. Ao descrever as várias definições académicas de Direito da Saúde como análogas a Edgewood, uma casa de ficção que apresenta um estilo arquitectónico diferente em cada uma de suas paredes, os autores tentam encontrar os elementos que deveriam compor uma definição mais abrangente. No artigo, Biodireito, Bioética e Direitos Humanos são descritos como complementos de uma definição de Direito da Saúde: o Biodireito, dado que é o campo jurídico que trata as consequências sociais que surgem dos avanços tecnológicos na área da saúde e das ciências da vida; a Bioética cujas evoluções influenciam normalmente o quadro jurídico da Saúde; e, por fim, a teoria dos Direitos Humanos e as suas declarações as quais têm estado sempre historicamente ligadas à medicina e à saúde, devendo funcionar como pano de fundo de todas as questões levantadas na área do Direito da Saúde. Para finalizar a sua breve incursão sobre a definição de Direito da Saúde, os autores dão ainda nota das complexas relações entre este último e a Saúde Pública, onde se tratam mais especificamente as leis aprovadas pelos governos para regular os serviços de saúde, as indústrias e as condutas individuais que afectam a saúde das populações, aspecto do Direito da Saúde que requer uma atenção especial para evitar um desequilíbrio entre os poderes públicos e as liberdades individuais. Os autores concluem afirmando que a confiança do público em qualquer sistema de saúde é, essencialmente, sustentada pelo desenvolvimento de estruturas de saúde que sejam consistentes com o direito constitucional da saúde, tais como o direito universal ao acesso a cuidados de saúde, e que o estudo do Direito da Saúde pode contribuir com elementos

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6th Graduate Student Symposium on Molecular Imprinting

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A two year-old female child was admitted at the Pediatric Intensive Care Unit in a septic shock associated with a lymphoproliferative syndrome, with history of fever, adynamia and weight loss during the last two months. On admission, the main clinical and laboratory manifestations were: pallor, jaundice, disseminated enlarged lymph nodes, hepatosplenomegaly, crusted warts on face, anemia, eosinophilia, thrombocytopenia, increased direct and indirect bilirubin, alkaline phosphatase, and gammaglutamyl transpeptidase. A parenteral administration of fluids, dobutamine and mechanical ventilation was started, without improvement of the clinical conditions. A direct examination of exsudate collected from cervical lymph node revealed numerous oval-to-around cells with multiple budding, like a "pilot wheel" cell, suggesting Paracoccidioides brasiliensis. Even though treatment with intravenous sulfamethoxazole-trimethoprine was soon started, the child died 36 hours after hospital admission. Disseminated paracoccidioidomycosis was confirmed in the autopsy. This is the youngest case of paracoccidioidomycosis in children reported in the literature.

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This work presents the development of a low cost sensor device for the diagnosis of breast cancer in point-of-care, made with new synthetic biomimetic materials inside plasticized poly(vinyl chloride), PVC, membranes, for subsequent potentiometric detection. This concept was applied to target a conventional biomarker in breast cancer: Breast Cancer Antigen (CA15-3). The new biomimetic material was obtained by molecularly-imprinted technology. In this, a plastic antibody was obtained by polymerizing around the biomarker that acted as an obstacle to the growth of the polymeric matrix. The imprinted polymer was specifically synthetized by electropolymerization on an FTO conductive glass, by using cyclic voltammetry, including 40 cycles within -0.2 and 1.0 V. The reaction used for the polymerization included monomer (pyrrol, 5.0×10-3 mol/L) and protein (CA15-3, 100U/mL), all prepared in phosphate buffer saline (PBS), with a pH of 7.2 and 1% of ethylene glycol. The biomarker was removed from the imprinted sites by proteolytic action of proteinase K. The biomimetic material was employed in the construction of potentiometric sensors and tested with regard to its affinity and selectivity for binding CA15-3, by checking the analytical performance of the obtained electrodes. For this purpose, the biomimetic material was dispersed in plasticized PVC membranes, including or not a lipophilic ionic additive, and applied on a solid conductive support of graphite. The analytical behaviour was evaluated in buffer and in synthetic serum, with regard to linear range, limit of detection, repeatability, and reproducibility. This antibody-like material was tested in synthetic serum, and good results were obtained. The best devices were able to detect 5 times less CA15-3 than that required in clinical use. Selectivity assays were also performed, showing that the various serum components did not interfere with this biomarker. Overall, the potentiometric-based methods showed several advantages compared to other methods reported in the literature. The analytical process was simple, providing fast responses for a reduced amount of analyte, with low cost and feasible miniaturization. It also allowed the detection of a wide range of concentrations, diminishing the required efforts in previous sample pre-treating stages.

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Ollier Disease and Maffucci Syndrome are two rare diseases that can cause tumors in several organs, having a special predilection for the hand. However, there have been very few reports in the literature focusing on hand manifestations of these diseases. We report the cases of three female patients: one with Ollier Disease, and two other with Maffucci Syndrome. All patients had hand involvement as their initial primary complaint. The Ollier Disease patient developed chondrosarcomas of two digits and had to have these fingers amputated. One of the Maffucci patients died one year after presentation from a brain glioblastoma. These cases emphasize the importance of early diagnosis of Ollier Disease and Maffucci Syndrome, as these two conditions are associated not only to crippling hand deformity, but also to a significant risk of chondrosarcoma, and other malignant tumors.

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Posterior interosseous nerve entrapment syndrome and spontaneous rupture of the extensor pollicis longus tendon are rare conditions. The authors describe the bizarre combination of a spontaneous rupture of the extensor pollicis longus tendon in a 82-year-old lady with a posterior interosseous nerve syndrome. As far as the authors know, this is the first description of such an association in the literature. Surgical exploration revealed compression of the posterior interosseous nerve at the proximal portion of the supinator muscle and at Henry's leash. The nerve was freed, and the tendon of the extensor index proprius was transferred to the extensor pollicis longus. Six months after the procedure, the patient had resumed her daily activities, showing a good functional result.

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Since October 2001, the Adolfo Lutz Institute has been receiving vesicular fluids and scab specimens of patients from Paraíba Valley region in the São Paulo and Minas Gerais States and from São Patricio Valley, in the Goiás State. Epidemiological data suggested that the outbreaks were caused by Cowpox virus or Vaccinia virus. Most of the patients are dairy milkers that had vesiculo-pustular lesions on the hands, arms, forearms, and some of them, on the face. Virus particles with orthopoxvirus morphology were detected by direct electron microscopy (DEM) in samples of 49 (66.21%) patients of a total of 74 analyzed. Viruses were isolated in Vero cell culture and on chorioallantoic membrane (CAM) of embryonated chicken eggs. Among 21 samples submitted to PCR using primers for hemagglutinin (HA) gene, 19 were positive. Restriction digestion with TaqI resulted in four characteristic Vaccinia virus fragments. HA nucleotide sequences showed 99.9% similarity with Cantagalo virus, described as a strain of Vaccinia virus. The only difference observed was the substitution of one nucleotide in the position 616 leading to change in one amino acid of the protein in the position 206. The phylogenetic analysis showed that the isolates clustered together with Cantagalo virus, other Vaccinia strains and Rabbitpox virus.

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A 12 y old girl was admitted 24 days after start a WHO multidrug therapy scheme for multibacillary leprosy (dapsone, clofazimine and rifampicin) with intense jaundice, generalized lymphadenopathy, hepatoesplenomegaly, oral erosions, conjunctivitis, morbiliform rash and edema of face, ankles and hands. The main laboratory data on admission included: hemoglobin, 8.4 g/dL; WBC, 15,710 cells/mm³; platelet count, 100,000 cells/mm³; INR = 1.49; increased serum levels of aspartate and alanine aminotransferases, gamma-glutamyl transpeptidase, alkaline phosphatase, direct and indirect bilirubin. Following, the clinical conditions had deteriorated, developing exfoliative dermatitis, shock, generalized edema, acute renal and hepatic failure, pancytopenia, intestinal bleeding, pneumonia, urinary tract infection and bacteremia, needing adrenergic drugs, replacement of fluids and blood product components, and antibiotics. Ten days after admission she started to improve, and was discharged to home at day 39th, after start new supervised treatment for leprosy with clofazimine and rifampicin, without adverse effects. This presentation fulfils the criteria for the diagnosis of dapsone hypersensitivity syndrome (fever, generalized lymphadenopathy, exfoliative rash, anemia and liver involvement with mixed hepatocellular and cholestatic features). Physicians, mainly in geographical areas with high prevalence rates of leprosy, should be aware to this severe, and probably not so rare, hypersensitivity reaction to dapsone.

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Dragonflies demonstrate unique and superior flight performances than most of the other insect species and birds. They are equipped with two pairs of independently controlled wings granting an unmatchable flying performance and robustness. In this paper it is studied the dynamics of a dragonfly-inspired robot. The system performance is analyzed in terms of time response and robustness. The development of computational simulation based on the dynamics of the robotic dragonfly allows the test of different control algorithms. We study different movement, the dynamics and the level of dexterity in wing motion of the dragonfly. The results are positive for the construction of flying platforms that effectively mimic the kinematics and dynamics of dragonflies and potentially exhibit superior flight performance than existing flying platforms.

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The trend to have more cooperative play and the increase of game dynamics in Robocup MSL League motivates the improvement of skills for ball passing and reception. Currently the majority of the MSL teams uses ball handling devices with rollers to have more precise kicks but limiting the capability to kick a moving ball without stopping it and grabbing it. This paper addresses the problem to receive and kick a fast moving ball without having to grab it with a roller based ball handling device. Here, the main difficulty is the high latency and low rate of the measurements of the ball sensing systems, based in vision or laser scanner sensors.Our robots use a geared leg coupled to a motor that acts simultaneously as the kicking device and low level ball sensor. This paper proposes a new method to improve the capability for ball sensing in the kicker, by combining high rate measurements from the torque and energy in the motor and angular position of the kicker leg. The developed method endows the kicker device with an effective ball detection ability, validated in several game situations like in an interception to a fast pass or when chasing the ball where the relative speed from robot to ball is low. This can be used to optimize the kick instant or by the embedded kicker control system to absorb the ball energy.

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Progressive disseminated histoplasmosis (PDH) is an increasingly common cause of infection in patients with acquired immune deficiency syndrome (AIDS). We report 21 cases of PDH associated with AIDS diagnosed by lysis-centrifugation blood culture method. The most prevalent clinical findings were fever, weight loss, respiratory symptoms, and mucocutaneous lesions. Chest roentgenogram showed diffuse pulmonary infiltrates in 13 of 21 patients (62%). Brochoalveolar fluid has yelded positive culture in four patients only in medium with cycloheximide.