Von Hippel-Lindau disease: a single gene, several hereditary tumors.

The Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder characterized by the predisposition for multiple tumors caused by germline mutations in the tumor suppressor gene VHL. This disease is associated with a high morbidity and mortality and presents a variable expression, with different phenotypes from family to family, affecting different organs during the lifetime. The main manifestations of VHL are hemangioblastomas of the central nervous system and retina, renal carcinomas and cysts, bilateral pheochromocytomas, cystic and solid tumors of the pancreas, cystadenomas of the epididymis, and endolymphatic sac tumors. The discovery of any of the syndrome components should raise suspicion of this disease and other stigmas must then be investigated. Due to the complexities associated with management of the various VHL manifestation, the diagnosis and the follow-up of this syndrome is a challenge in the clinical practice and a multidisciplinary approach is needed. The particular relevance to endocrinologists is the detection of pheochromocytomas in 35% and islet cell tumors in 17% of VHL patients, which can be associated with hypertension, hypoglycemia, cardiac arrhythmias, and carcinoid syndrome. The purpose of this review is to define the Von Hippel-Lindau syndrome addressing its clinical aspects and classification, the importance of genetic counseling and to propose a protocol for clinical follow-up.

Lewis histo-blood group system phenotyping and genotyping reveal divergence in the association of Le(a-b-) phenotype and type 1 diabetes.

BACKGROUND AND OBJECTIVES: The red blood cell Le(a-b-) phenotype was proposed as risk factor for type 1 diabetes, but contradictory results were published elsewhere. This study re-examined the potential association between Lewis histo-blood group system and type 1 diabetes. MATERIAL AND METHODS: Patients and controls of both sexes, Caucasians and non-Caucasians, matched by sex, geographical origin and ethnicity were evaluated. The red blood cell Lewis phenotypes were identified by gel column agglutination and also inferred from the FUT2 and FUT3 genotyping. RESULTS: The Le(a-b-) phenotype was prevalent in patients with type 1 diabetes, and the Le(a-b+) phenotype was prevalent in controls when both were determined by gel columns agglutination. No differences were observed in the frequencies of the Le(a-b-) phenotype inferred from the FUT2 and FUT3 genotyping between patients and controls. CONCLUSIONS: The Lewis red blood cell phenotyping and genotyping reveal divergence in the association of Le(a-b-) phenotype and type 1 diabetes.

Rendimento de coentro (Coriandrum sativum L. ) em sistema de adubação verde com a planta jitirana (Merremia aegyptia L. ) / Yield of coriander (Coriandrum sativum L. ) in a system of green manure with the plant scarlet starglory (Merremia aegyptia L.)

O coentro é a hortaliça mais utilizada como condimento na região de Mossoró-RN. Um experimento foi conduzido na Fazenda Experimental Rafael Fernandes da Universidade Federal Rural do Semi-Árido - UFERSA, no período de maio a junho de 2010, com o objetivo de avaliar o rendimento de coentro sob diferentes quantidades da planta trepadeira jitirana, incorporada ao solo como adubo verde. O delineamento experimental usado foi o de blocos completos casualizados com sete tratamentos e três repetições. Os tratamentos consistiram da incorporação de sete quantidades de jitirana (3,0; 6,0; 9,0; 12,0; 15,0; 18,0 e 21,0 t ha-1 de matéria seca de jitirana). A cultivar de coentro plantado foi a Verdão. As características avaliadas foram: altura e número de hastes por planta, rendimento e massa seca da parte aérea. O melhor desempenho agronômico do coentro foi observado na quantidade de 21,0 t ha-1 de jitirana. Para cada tonelada de jitirana incorporada ao solo observa-se um rendimento de coentro de 395 kg ha-1.

The ciliandro is the vegetable more used as seasoning in the area at Mossoró-RN. An experiment was conducted at the Experimental Farm Rafael Fernandes of the Universidade Federal Rural do Semi-Árido - UFERSA in the period from may to june 2010, with the objective of evaluating the coriander yield in under different amounts of plant clambering scarlet starglory, incorporated into the soil. The experimental design was randomized complete blocks with eight treatments and three replicates. The treatments were combinations of eight amounts of scarlet starglory (3.0; 6.0; 9.0; 12.0; 15.0; 18.0 and 21.0 t ha-1 dry matter). The coriander cultivar planted was Verdão. The characteristics evaluated in the coriander were: plant height and number of stalks per plant, yield and dry matter mass of shoots. The best agroconomic performance lettuce was observed in the amount of 21,0 t ha-1 scarlet starglory. For each fresh or dry scarlet starglory ton incorporated into the soil, it was observed a mean yield of coriander mass of 395 kg ha-1.

Environmental education in the Upper Paraná River floodplain, municipality of Porto Rico (Paraná State), Brazil / Educação ambiental na planície alagável do Alto Rio Paraná, Porto Rico - PR, Brasil

Since 2003, researchers, faculty, graduate and undergraduate students from the State University of Maringá have been working alongside teachers from the state and local schools in the municipality of Porto Rico (Paraná State), located on the banks of the Paraná River. Their objective is to outline actions and strategies with the purpose of building methodological paths to insert environmental education into the school curriculum. Based on the action-research methodology, the group has developed the following programs: a) the Continuing Education Program in Environmental Education; b) the Development of Interdisciplinary Projects; c) the Insertion of Information and Communication Technologies (ICTs); and d) the Production of Teaching Materials. The evaluations of the programs indicate that teachers have been able to gradually build a theoretical and methodological basis for environmental education while simultaneously growing into the role of teacher-researchers as they create the conditions to investigate their pedagogical practices, reflect upon them, share experiences, innovate, and make the teaching-learning process more significant. Allied to the advances in educational practices and with the aid of ICTs, the activities developed in the classroom, in the field and in the lab - all of which involve natural and cultural aspects of the region - have contributed to teachers' and students' better understanding of the ecological, cultural, social and economic value of the floodplain, and consequently, of the importance of preservation and management in order to maintain local biodiversity.

Desde 2003, pesquisadores, professores, pós-graduandos e graduandos da Universidade Estadual de Maringá vêm trabalhando em conjunto com os professores das escolas, estadual e municipal, do município de Porto Rico - PR, localizado às margens do Rio Paraná, com o objetivo de delinear ações e estratégias, no sentido de construir caminhos metodológicos para a inserção da educação ambiental no currículo escolar. Com base na metodologia da pesquisa- ação, o grupo vem desenvolvendo alguns programas: a) Programa de Educação Continuada em Educação Ambiental; b) Desenvolvimento de Projetos Interdisciplinares; c) Inserção de Tecnologias de Informação e Comunicação (TICs); d) Produção de Material Didático. As avaliações dos programas indicam que, gradativamente, os professores têm construído uma base teórica e metodológica da educação ambiental e, ao mesmo tempo, tem desenvolvido uma postura de professor-pesquisador, ao criar condições de investigação de sua prática pedagógica, de refletir sobre a mesma, de trocar experiências, de inovar, de tornar o processo de ensino-aprendizagem mais significativo. Aliado ao aprimoramento das práticas educacionais, e com o apoio das TICs, as atividades desenvolvidas, em sala de aula, campo e laboratório, abordando aspectos naturais e culturais da região, têm contribuído para que professores e alunos compreendam melhor o valor ecológico, cultural, social e econômico da planície e, consequentemente, da importância da conservação e manejo da mesma para a manutenção da biodiversidade local.

Emissões de queimadas em ecossistemas da América do Sul / Emissões de queimadas em ecossistemas da América do Sul

As queimadas que ocorrem majoritariamente em áreas tropicais do planeta, são fontes importantes de poluentes para a atmosfera. Na América do Sul, durante os meses de inverno, uma área, principalmente de ecossistemas de cerrado e floresta, da ordem de 40 mil km² é queimada anualmente. Estas queimadas ocorrem primariamente nas regiões Amazônica e do Brasil Central, porém, através do transporte atmosférico de suas emissões resulta uma distribuição espacial de fumaça sobre uma extensa área, ao redor de 4-5 milhões de km², em muito superior a área onde estão concentradas as queimadas. Durante a combustão de biomassa são emitidos para a atmosfera gases poluentes e partículas de aerossol que interagem eficientemente com a radiação solar e afetam os processos de microfísica e dinâmica de formação de nuvens e a qualidade do ar. Os efeitos destas emissões excedem, portanto, a escala local e afetam regionalmente a composição e propriedades físicas e químicas da atmosfera na América do Sul e áreas oceânicas vizinhas, com potencial impacto em escala global.

Smoking rain clouds over the Amazon.

Heavy smoke from forest fires in the Amazon was observed to reduce cloud droplet size and so delay the onset of precipitation from 1.5 kilometers above cloud base in pristine clouds to more than 5 kilometers in polluted clouds and more than 7 kilometers in pyro-clouds. Suppression of low-level rainout and aerosol washout allows transport of water and smoke to upper levels, where the clouds appear "smoking" as they detrain much of the pollution. Elevating the onset of precipitation allows invigoration of the updrafts, causing intense thunderstorms, large hail, and greater likelihood for overshooting cloud tops into the stratosphere. There, detrained pollutants and water vapor would have profound radiative impacts on the climate system. The invigorated storms release the latent heat higher in the atmosphere. This should substantially affect the regional and global circulation systems. Together, these processes affect the water cycle, the pollution burden of the atmosphere, and the dynamics of atmospheric circulation.

Mouse B-1 cell-derived mononuclear phagocyte, a novel cellular component of acute non-specific inflammatory exudate.

At least three B cell subsets, B-1a, B-1b and B-2, or conventional B cells are present in the mouse periphery. Here we demonstrate that B-1 cells spontaneously proliferate in stationary cultures of normal adherent mouse peritoneal cells. B-1 cells were characterized by morphology, immunohistochemistry and flow cytometry. IgM was detected in the supernatants of these cultures. We demonstrated that the major cell population analyzed expresses the B-1b phenotype. When these cells were transferred to a new culture, a large proportion of them adhere to the plastic surface, and spread as bipolar cells endowed with the capacity to phagocytose via Fc and mannose receptors. Flow cytometry analysis of these adherent cells demonstrated that the great majority of them share both B-220 and Mac-1 antigens. Nevertheless, 45% of them were exclusively Mac-1(+). Finally, when they were labeled in vitro with [(3)H]thymidine and transferred to the peritoneal cavity of naive mice, they migrate to a non-specific inflammatory focus induced by a foreign-body implant. These data demonstrate that B-1 cells, mainly B-1b cells, not only proliferate and differentiate into a mononuclear phagocyte in vitro, but also that they exit the peritoneal cavity and migrate to a non-specific inflammatory milieu.

Hypocellular anaplastic large cell lymphoma mimicking inflammatory lesions of lymph nodes.

The subgroup of T/null-cell primary systemic anaplastic large cell lymphoma that expresses anaplastic lymphoma kinase (ALK) constitutes a distinctive clinicopathologic entity that exhibits a broad morphologic spectrum. The examples predominated by small cells or showing a mixed cell population can be difficult to recognize as being neoplastic. We report four such cases with a remarkably hypocellular granulation tissue-like appearance, mimicking an inflammatory or reparative process. All patients were young and presented with lymphadenopathy in multiple sites. The lymph node biopsies showed a hypocellular appearance, with wide separation of the small to medium-sized lymphoid cells by edematous or fibromyxoid stroma. There were interspersed spindly neoplastic cells resembling myofibroblasts, sometimes forming short, sweeping fascicles, as well as histiocytes. Occasional large cells with atypical nuclei were identified. The larger lymphoid cells tended to form cuffs around the venules. In two cases, the capsule and fibrous trabeculae were markedly broadened with increased spindly cells, mimicking inflammatory pseudotumor of lymph node. Immunostaining showed dispersed and clustered CD30+ ALK+ cells, confirming a diagnosis of anaplastic large cell lymphoma. In conclusion, a diagnosis of hypocellular anaplastic large cell lymphoma requires a high index of suspicion. The young age of the patients and the presence of perivascular cuffs of larger lymphoid cells should provide the strongest clues to the correct diagnosis.

Subcutaneous infection of mice with Paracoccidiodes brasiliensis induces a peculiar pattern of inflammatory and immune responses.

It is well established that resistance or susceptibility to Paracoccidioidis brasiliensis infection in mice is under strict host's genetic control. Mice from A/Sn strain inoculated by the ip route are resistant to fungal infection while infection induced in mice from B10.A strain results in a fatal disease. The early cellular events of infection in both strains are characterized by a marked neutrophilic infiltration that is more prominent in B10.A mice. A peculiar characteristic of the Paracoccidioides brasiliensis-mouse model is that the subcutaneous (sc) inoculations of the fungus either in resistant (A/Sn) or susceptible (B10.A) mice is self-curing and tums mice from the B10.A strain able to express typical DTH reaction to fungal antigens, as observed in A/Sn mice. Here we report the investigation on the early events of the inflammatory response induced by the inoculation of live fungus into the hind footpad of A/Sn (resistant) and B10.A (susceptible) mice. The influence of neutrophils on the inflammatory response and antibody titers or DTH response to gp43, the major fungal antigen, was also evaluated. Results showed a different course of the inflammatory response induced by fungal inoculation in A/Sn and B10.A mice. Neutrophil depletion before infection differently influenced the kinetics of the inflammatory process in both mice strains but did not modify the fungal load in the lesions. In neutrophil depleted mice from both strains, a decrease in DTH response and an increase in total antibody titers to gp43 were observed. The significant increase in the fungal load in lesions seen in nude mice indicates that the self-limited infection evoked by fungal inoculation into the subcutaneous tissue is a T-cell dependent phenomenon. The implications of these observations in the pathogenesis of paracoccidioidomycosis are discussed.

Laparoscopic diagnosis and treatment of splenogonadal fusion associated with intra-abdominal cryptorchidism in a child.

The authors describe the surgical findings and laparoscopic treatment in a child with splenogonadal fusion associated with intra-abdominal cryptorchidism. Laparoscopy was shown to be an excellent method for the diagnosis and treatment of this condition. No reports of similar cases using the technique were found in the literature.

Characterization of the mechanism involved in the relaxant response of dopexamine in the guinea pig pulmonary artery in vitro.

Dopexamine is a synthetic catecholamine used for the management of low-cardiac-output states. The purpose of this study was to characterize some of the mechanisms underlying dopexamine-mediated relaxation in the guinea pig pulmonary artery (PA) in vitro. Dopexamine (EC50, 1.2 microM; Rmax, 100%), like dobutamine (EC50, 1.4 microM, Rmax, 93.3%), prostacyclin (PGI2; EC50, 37 nM; Rmax, 96.2%), sodium nitroprusside (EC50, 370 pM; Rmax, 96.9%), forskolin (EC50, 47 pM: Rmax, 98.6%), and SKF 38393 (EC50, 120 nM; Rmax, 100%), caused graded relaxation in rings of PA precontracted by phenylephrine. The dopexamine vasorelaxation was antagonized by propranolol (1 microM), SCH 23390 (100 nM, a D1-dopamine antagonist), sulpiride (1 microM), glibenclamide (30 microM), tetraethylammonium (3 mM), apamin (100 nM), charybdotoxin (100 nM), SQ 22536 (10 microM, an adenylyl cyclase inhibitor), KT 5720 (10 microM, a protein kinase A inhibitor) and by calcitonin gene-related peptide (CGRP) or vasoactive intestinal peptide (VIP)-receptor antagonists (both 100 nM), as well as by chymotrypsin (1 U/ml). Neither the prior incubation of N(G)-nitro-L-arginine (100 pM), indomethacin (1 microM), nor removal of the vascular endothelium interfered with dopexamine vasorelaxation response in PA. Thus dopexamine relaxation in PA is mediated by activation of beta-adrenoceptors and dopamine receptors, and by the opening of both low- and high-conductance Ca2+-activated K+ channels, partially through adenosine triphosphate (ATP)-sensitive K+ channels. In addition, dopexamine-induced relaxation in PA seems to involve the release of peptides such as VIP and CGRP, an effect mediated by a cyclic adenosine monophosphate (cAMP)-dependent mechanism.

Antinociceptive effect of the calcium-binding protein MRP-14 and the role played by neutrophils on the control of inflammatory pain.

Macrophages secrete a variety of chemical mediators that play a central role in the pathophysiology of inflammatory pain. Therefore, the activation or deactivation of these cells in an inflammatory focus could modulate the intensity of the algogenic response. Based on these premises and on our previous demonstration that the calcium-binding protein MRP-14, highly expressed in neutrophils, deactivates activated macrophages in vitro, we decided to investigate the role of MRP-14 and of neutrophils in the control of inflammatory pain in mice. Our results show that this protein is endowed with antinociceptive activity. When tested in the writhing model it was able to inhibit pain response but did not change the behavior of the animals in the hot plate test. This observation indicates that MRP-14 down-regulates inflammatory but not central pain. Using a model of acute neutrophilic peritonitis induced by glycogen, a close correlation between neutrophil migration and antinociception was detected. Surgical adrenalectomy demonstrated that the antinociceptive response induced by glycogen was not due to endogenous liberation of glucocorticoids. The treatment of animals either with a monoclonal antibody anti-MRP-14 or a monoclonal antibody that depletes the animals of neutrophils reverts the antinociceptive response observed in the glycogen-induced peritonitis. These data define the calcium-binding protein MRP-14 as a novel mediator for the control of inflammatory pain and consequently discloses an anti-inflammatory role for the neutrophil.

[Gastric liposarcoma: report of a case]. / Lipossarcoma gástrico: relato de um caso.

The authors present a case of a well differentiated gastric liposarcoma. The tumor was situated in the submucosa of the antrum, had a diameter of 9 cm, and showed well defined borders. A subtotal gastrectomy with BI reconstruction was performed. No abdominal metastases were observed. The patient is in good condition, without recurrence of the tumor 19 months after its removal.

Poliglactina (vicryl) em cirurgia urologica. / Polyglactina (vicryl) in urological surgery

Os autores assinalam sua experiencia com relacao ao fio de sutura poliglactina 910 utilizado nas vias urinarias. Apresentam 40 (quarenta) casos de cirurgias urologicas em que foram realizados pontos separados ou suturas continuas interessando o urotelio, com este novo fio de sutura sintetica absorvivel, constatando o seu facil manejo, boa elasticidade, suficiente resistencia e bom contraste com o campo operatorio

Cytosine arabinoside induced gastrointestinal toxic alterations in sequential chemotherapeutic protocols: a clinical-pathologic study of 33 patients.

Sequential chemotherapeutic regimens, primarily used in the treatment of hematopoietic malignancies, and employing ara-C as a basic antineoplastic agent induce mucosal alterations in the entire gastrointestinal tract. These are characterized by surface and glandular epithelial atypia, immaturity, and necrosis. Glandular regeneration is characteristically delayed leading to a state of intestinal aproliferative cytopenia. Other toxic intestinal changes include telangiectasia of blood vessels and the formation of intramural hematomas. Intestinal infections develop frequently and are complicated by peritonitis, liver abscesses, pneumatosis cystoides in testinalis and sepsis. These intestinal lesions are accompanied by a predictable clinical syndrome which begins concomitantly with ara-C infusions and is characterized by diarrhea, ileus, abdominal pain, hematemesis and melena, severe hypokalemia, hypocalcemia and a protein-losing enteropathy. Additional toxic manifestations induced by ara-C include transient weight gains, fever elevations and severe bone marrow depression. The genesis of the intestinal lesions is linked to the three day dose schedule of ara-C infusions which insures both arrest of the cycling intestinal cells in the S-phase and a high cytotoxic index. The severity of these lesions is markedly augmented by prior treatment with ara-C and cyclophosphamide which causes synchronization and probable recruitment of intestinal stem cells, respectively.