Doenças respiratórias associadas à atividade de mineração no município de Parelhas, região do seridó norte-riograndense

This work aims to characterize the workers in mineral activities exposed to lung injuries in Parelhas Municipality, Rio Grande do Norte State, seeking to relate respiratory diseases to the mining activity. The studied area (Parelhas City), with about 19,700 inhabitants, is located in the Serido region, approximately 232 km far from Natal City. The number of people involved in informal mining activity (garimpo) in the Seridó region reaches about 5,000. These workers generally do not use any kind of individual protection equipments and develop, at early ages of greater productivity, severe forms of diseases, which end up disabling them to professional activities, family and social life. Deceases by respiratory problems (e.g. silicosis) have been reported in very young adults. A descriptive observational study was conducted based on information from the records found in Dr. José Augusto Dantas Hospital, between the years 1996- 2006. The occupational and socio-economic features of the population, which was selected by using the hospital records, were achieved through individually answered forms. The purpose was to link the occupational activities with the respiratory diseases. The next stage of the research was an observational case-control study, in the 1:1 proportion. The achieved data allowed confirming the central hypothesis of the research, which states that the pneumoconiosis cases are due to the mineral-based activities in the studied area. The final step of the investigation tried to assess the knowledge of relatives of students in public and private elementary and high schools from Parelhas City, regarding silicosis. About 15.4% of urban schools were analyzed through application of a structured questionnaire. The results show distinct socio-economic levels and a difference in the perception of the relatives of students in public and private schools, concerning silicosis. It was possible to identify the characteristics of the population economically involved with mineral-based activities and to define the group that deserves preferential attention in preventive actions. The work indicates some environmental problems caused by inadequate mining operations in the region

Três alternativas estocásticas para modelar morbimortalidade por doenças respiratórias e cardiovasculares via variáveis atmosféricas

Climate and air pollution, among others, are responsible factors for increase of health vulnerability of the populations that live in urban centers. Climate changes combined with high concentrations of atmospheric pollutants are usually associated with respiratory and cardiovascular diseases. In this sense, the main objective of this research is to model in different ways the climate and health relation, specifically for the children and elderly population which live in São Paulo. Therefore, data of meteorological variables, air pollutants, hospitalizations and deaths from respiratory and cardiovascular diseases a in 11-year period (2000-2010) were used. By using modeling via generalized estimating equations, the relative risk was obtained. By dynamic regression, it was possible to predict the number of deaths through the atmospheric variables and the betabinomial-poisson model was able to estimate the number of deaths and simulate scenarios. The results showed that the risk of hospitalizations due to asthma increases approximately twice for children exposed to high concentrations of particulate matter than children who are not exposed. The risk of death by acute myocardial infarction in elderly increase in 3%, 6%, 4% and 9% due to high concentrations CO, SO2, O3 and PM10, respectively. Regarding the dynamic regression modeling, the results showed that deaths by respiratory diseases can be predicted consistently. The beta-binomial-poisson model was able to reproduce an average number of deaths by heart insufficiency. In the region of Santo Amaro the observed number was 2.462 and the simulated was 2.508, in the Sé region 4.308 were observed and 4.426 simulated, which allowed for the generation of scenarios that may be used as a parameter for decision. Making with these results, it is possible to contribute for methodologies that can improve the understanding of the relation between climate and health and proved support to managers in environmental planning and public health policies.

Equações preditivas para as pressões respiratórias máximas de crianças brasileiras

I ntroduction: The assessment of respiratory muscle strength is important in the diagnosis and monitoring of the respiratory muscles weakness of respiratory and neuromuscular diseases. However, there are still no studies that provide predictive equations and reference values for maximal respiratory pressures for children in our population. Aim: The purpose of this study was to propose predictive equations for maximal respiratory pressures in healthy school children. Method: This is an observational cross-sectional study. 144 healthy children were assessed. They were students from public and private schools in the city of Natal /RN (63 boys and 81 girls), subdivided in age groups of 7-8 and 9-11 years. The students presented the BMI, for age and sex, between 5 and 85 percentile. Maximal respiratory pressures were measured with the digital manometer MVD300 (Globalmed ®). The maximal inspiratory pressure (MIP) and maximal expiratory pressures (MEP) were measured from residual volume and total lung capacity, respectively. The data were analyzed using the SPSS Statistics 15.0 software (Statistical Package for Social Science) by assigning the significance level of 5%. Descriptive analysis was expressed as mean and standard deviation. T'Student test was used for unpaired comparison of averages of the variables. The comparison of measurements obtained with the predicted values in previous studies was performed using the paired t'Student test. The Pearson correlation test was used to verify the correlation of MRP's with the independent variables (age, sex, weight and height). For the equations analysis the stepwise linear regression was used. Results: By analyzing the data, we observed that in the age range studied MIP was significantly higher in boys. The MEP did not differ between boys and girls aged 7 to 8 years, the reverse occurred in the age between 9 and 11 years. The boys had a significant increase in respiratory muscle strength with advancing age. Regardless sex and age, MEP was always higher than the MIP. The reference values found in this study are similar to a sample of Spanish and Canadian children. The two models proposed in previous studies with children from other countries were not able to consistently predict the values observed in this studied population. The variables sex, age and weight correlated with MIP, whereas the MEP was also correlated with height. However, in the regression models proposed in this study, only gender and age were kept exerting influence on the variability of maximal inspiratory and expiratory pressures. Conclusion: This study provides reference values, lower limits of normality and proposes two models that allow predicting, through the independent variables, sex and age, the value of maximal static respiratory pressures in healthy children aged between 7 and 11 years old

Estado nutricional e efeito da vitamina A na resposta imune frente à infecção por Leishmania Infantum

Nutritional status is an important determinant to the response against Leishmania infection, although few studies have characterized the molecular basis for the association found between malnutrition and the disease. Vitamin A supplementation has long been used in developing countries to prevent mortality by diarrheal and respiratory diseases, but there are no studies on the role of vitamin A in Leishmania infection, although we and others have found vitamin A deficiency in visceral Leishmaniasis (VL). Regulatory T cells are induced in vitro by vitamin A metabolites and are considered important cells implicated T CD4+ cell suppression in human VL. This work aimed to examine the correlation of nutritional status and the effect of vitamin A in the response against Leishmania infantum infection. A total of 179 children were studied: 31 had active VL, 33 VL history, 44 were DTH+ and 71 were DTH- and had negative antibody to Leishmania (DTH-/Ac-). Peripheral blood monuclear cells were isolated in a subgroup of 10 active VL and 16 DTH-/Ac- children and cultivated for 20h under 5 different conditions: 1) Medium, 2) Soluble promastigote L. infantum antigens (SLA), 3) All-trans retinoic acid (ATRA), 4) SLA + ATRA and 5) Concanavalin A. T CD4+CD25highFoxp3+, T CD4+CD25-Foxp3- and CD14+ monocytes were stained and studied by flow cytometry for IL-10, TGF-β and IL-17 production. Nutritional status was compromised in VL children, which presented lower BMI/Age and retinol concentrations when compared to healthy controls. We found a negative correlation between nutritional status (measured by BMI/Age and serum retinol) and anti-Leishmania antibodies and acute phase proteins. There was no correlation between nutritional status and parasite load. ATRA presented a dual effect in Treg cells and monocytes: In healthy children (DTH-/Ac-), it induced a regulatory response, increasing IL-10 and TGF-β production; in VL children it modulated the immune response, preventing increased IL-10 production after SLA stimulation. Furthermore, we found a positive correlation between BMI/Age and IL-17 production and negative correlation between serum retinol and IL-10 and TGF-β production in T CD4+CD25highFoxp3+ cells after SLA stimulus. Our results show a potential dual role of vitamin A in the immune system: improvement of regulatory profile during homeostasis and down modulation of IL-10 in Treg cells and monocytes during symptomatic VL. Therefore, the use of vitamin A concomitant to VL therapy might improve recovery from disease status in Leishmania infantum infection

Análise cintilográfica da deposição pulmonar de radioaerossol após associação da nebulização através dos dispositivos mesh e jato com a ventilação não invasiva em indivíduos normais e com pneumopatias obstrutivas crônicas

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior

Úlceras de pressão: um estudo com pacientes de unidade de terapia intensiva

To characterize patients according to gender, age category, internment time, diagnostical hypothesis and location of the pressure ulcer; to identify the susceptibility conditions, intrinsical and extrinsical factors present on ICU patients and to verify on the existence of association between the susceptibility conditions and the intrinsecal and extrinsecal factors on the occurrence of PU. Methods: It is a descriptive study, of longitudinal design of the panel type, with quantitative approach, performed on two ICU s of a private hospital located in Natal/RN, with 40 patients interned at these units. The data collection was performed on all three shifts through a structured observation and physical exam of the patients' skin form seeking to identify the presence of PU. Results: The greatest occurence of PU was on individuals of the male gender (70%) when compared to the female gender (30%), that difference being statistically significant (p=0,0267), with the male gender presenting 4,3 times greater chance of developing PU than the female; the predominant age category was from 60 years of age on (85%), 60,0% presented 1 to 2 PU s after 7 (seven) days of permanence in the ICU s, the predominant diagnostical hypothesis on the patients with PU were the respiratory diseases (42,3%) and the most frequent locations of PU were the sacral region (40,0%) and heels (36,0%). 25 PU s of stage I were diagnosed on 50,0% of the followed patients, with general incidence of 50,0% on both ICUs. from the 88 variables researched, 75 were identified on the patients from the study, being the predominant conditions (anemia, hypotension, leukocytosis, other diseases hypertension blood pressure, cardiac insuffience, pneumonia - and ansiolythic), the intrinsecal factors (diminished muscular strenght and/or mass, discrete edema, totally compromised mobile coordination and total inability for movement on the bed) and the extrinsecal factors (inadequate mattress type, permanence on a single position for >2 hours, shearing/friction force, bed clothes with folds that leave marks on the body, pressure force) predominated on patients with PU. The male gender variables (p=0,0267,OR=4,3), sedation (p=0,0006,OR=4,1), psychomotive agitation (p=0,0375,OR=5,8) and leukocytosis (p=0,0285,OR=5,0) presented a significant statistical diference when analyzed independently. We verified an association of 17,3%, statistically significant (p=0,0384), between the susceptibility conditions (anemia, leukocytosis and hypotension), the intrinsecal factors (age equal or above 60 years, diminished/absent pain sensibility and smooth, fine or delicate skin) and the extrinsecal factors (inadequate mattress, pressure forces, shearing/friction force, permanence on a single position for more than 2 hours, elevation between 30 to 45 degrees and inadequate bed clothes' conditions), with a chance ratio of 4,6 times the risk of occurrence of PU on the patients that presented the referred association. Conclusion: The incidence of PU detected on the ICU-interned patients was high and we made evident the existence of association between the susceptibility conditions, the intrinsecal and extrinsecal factors on the occurrence of PU s on the ICUinterned patients, and thus we accept the alternative hypothesis proposed on the study

Estado nutricional e efeito da vitamina A na resposta imune frente à infecção por Leishmania Infantum

Nutritional status is an important determinant to the response against Leishmania infection, although few studies have characterized the molecular basis for the association found between malnutrition and the disease. Vitamin A supplementation has long been used in developing countries to prevent mortality by diarrheal and respiratory diseases, but there are no studies on the role of vitamin A in Leishmania infection, although we and others have found vitamin A deficiency in visceral Leishmaniasis (VL). Regulatory T cells are induced in vitro by vitamin A metabolites and are considered important cells implicated T CD4+ cell suppression in human VL. This work aimed to examine the correlation of nutritional status and the effect of vitamin A in the response against Leishmania infantum infection. A total of 179 children were studied: 31 had active VL, 33 VL history, 44 were DTH+ and 71 were DTH- and had negative antibody to Leishmania (DTH-/Ac-). Peripheral blood monuclear cells were isolated in a subgroup of 10 active VL and 16 DTH-/Ac- children and cultivated for 20h under 5 different conditions: 1) Medium, 2) Soluble promastigote L. infantum antigens (SLA), 3) All-trans retinoic acid (ATRA), 4) SLA + ATRA and 5) Concanavalin A. T CD4+CD25highFoxp3+, T CD4+CD25-Foxp3- and CD14+ monocytes were stained and studied by flow cytometry for IL-10, TGF-β and IL-17 production. Nutritional status was compromised in VL children, which presented lower BMI/Age and retinol concentrations when compared to healthy controls. We found a negative correlation between nutritional status (measured by BMI/Age and serum retinol) and anti-Leishmania antibodies and acute phase proteins. There was no correlation between nutritional status and parasite load. ATRA presented a dual effect in Treg cells and monocytes: In healthy children (DTH-/Ac-), it induced a regulatory response, increasing IL-10 and TGF-β production; in VL children it modulated the immune response, preventing increased IL-10 production after SLA stimulation. Furthermore, we found a positive correlation between BMI/Age and IL-17 production and negative correlation between serum retinol and IL-10 and TGF-β production in T CD4+CD25highFoxp3+ cells after SLA stimulus. Our results show a potential dual role of vitamin A in the immune system: improvement of regulatory profile during homeostasis and down modulation of IL-10 in Treg cells and monocytes during symptomatic VL. Therefore, the use of vitamin A concomitant to VL therapy might improve recovery from disease status in Leishmania infantum infection

Crianças com infecções respiratórias agudas: como são cuidadas em casa?

Objetivou-se, neste estudo exploratório/descritivo, identificar conhecimentos e necessidades dos cuidadores da criança com Infecção Respiratória Aguda (IRA) através de uma amostra de 129 crianças atendidas no serviço de crescimento e desenvolvimento de uma unidade de saúde. Foram aplicados formulários junto aos cuidadores, em seu domicílio, nos meses de fevereiro e março de 2002. Os cuidadores eram do sexo feminino, a maioria jovem, com ensino fundamental incompleto e com renda de 1 a 2 salários. Em relação aos primeiros cuidados com a criança doente, 48,2% dos cuidadores procuram o médico, 36,6% medicam por conta própria e 13,2% utilizam fitoterápicos. Como medidas de prevenção contra pneumonia em crianças com IRA leve, os cuidadores utilizam medidas de senso comum e sabem reconhecer sinais de gravidade das doenças, por experiências anteriores, revelando uma lacuna do conhecimento dos principais sinais e sintomas de doenças graves do trato respiratório

Avaliação das pressões respiratórias máximas em crianças e adolescentes da grande Natal: elaboração de uma equação preditiva

The human respiratory system was so designed that would allow efficient ventilation, regardless of variations in the external environment that may hinder the act of breathing, such an act involves dozens of variables, among them we find the respiratory depression, which is nothing more than respiratory muscle strength. The pressures are widely used in several cases: Neuro-muscular; evolution of pulmonary dysfunction and a predictor for discontinuation of mechanical ventilation. Therefore it was proposed to carry out evaluations of these respiratory pressures for children and adolescents aged 10 to 16 years and propose a predictive equation that involves the anthropometric variables age (A, years), body mass (BM, kilograms) and height (H, meters) with maximal respiratory pressures (maximum inspiratory and expiratory pressure). Evaluations were performed in this age group of students in public and private schools of the Grande Natal , measurements were performed using the analogue manometer, were children and adolescents and their parents gave informed consent. 517 samples were taken, and 250 for males (M), 255 for females (F) and 12 were excluded according to our exclusion criteria. The sample was subdivided into three age groups (10-11, 12-13 and 14 to 16 years old). It was found through the student s t test (p ≤ 0.05) for all variables studied, children and male adolescents had higher means than females, except for the MC. For the correlation between the variables found significant correlation (p <0.05) among all the variables when analyzed as pairs except between MIP and height for females. The development of predictive equations (for p ≤ 0.05) based on three types of strategies adopted were restricted to two association between anthropometric variables isolated, resulting in: for males: MIP = -32.29 + (-2.11*A) + (-0.52*BM), MIP = 9.99 + (-0.36*BM) + (-49.40*H); MEP = 18.54 + 3.53*A + 0, 42*BM, MEP = -33.37 + 2.78*A + 52.18* H, MEP = -17.39 + 0.33*BM + 55.04*H; and, for females we find: MEP = 24.32 + 2.59 * A + 0.24*BM

Crianças com infecções respiratórias agudas: como são cuidadas em casa?

Objetivou-se, neste estudo exploratório/descritivo, identificar conhecimentos e necessidades dos cuidadores da criança com Infecção Respiratória Aguda (IRA) através de uma amostra de 129 crianças atendidas no serviço de crescimento e desenvolvimento de uma unidade de saúde. Foram aplicados formulários junto aos cuidadores, em seu domicílio, nos meses de fevereiro e março de 2002. Os cuidadores eram do sexo feminino, a maioria jovem, com ensino fundamental incompleto e com renda de 1 a 2 salários. Em relação aos primeiros cuidados com a criança doente, 48,2% dos cuidadores procuram o médico, 36,6% medicam por conta própria e 13,2% utilizam fitoterápicos. Como medidas de prevenção contra pneumonia em crianças com IRA leve, os cuidadores utilizam medidas de senso comum e sabem reconhecer sinais de gravidade das doenças, por experiências anteriores, revelando uma lacuna do conhecimento dos principais sinais e sintomas de doenças graves do trato respiratório

Análise do estado da arte dos aspectos diagnóticos, periciais e jurisprudenciais das LER/DORT no contexto previdenciário das doenças do trabalho no Brasil

Objetivo: analisar o estado da arte dos aspectos diagnósticos, periciais e jurisprudenciais das LER/DORT (Lesões por Esforços Repetitivos / Distúrbios Osteomusculares Relacionados ao Trabalho) no Brasil. Materiais e Método: trata-se de pesquisa descritiva, de natureza qualitativa, com formato documental, utilizando-se a técnica de análise de conteúdo. A avaliação evolutiva da legislação previdenciária relacionada as LER/DORT foi realizada através da pesquisa no banco de dados disponibilizado pelo Governo Federal e mediante a consulta ao DATAPREV/Sislex. A avaliação dos aspectos diagnósticos foi instrumentalizada através, principalmente, de artigos científicos publicados entre 2003 e 2008, nas línguas portuguesa, inglesa, espanhola e francesa, relacionados com os métodos de diagnósticos complementares das LER-DORT (ressonância magnética, tomografia computadorizada, ultrassonografia e eletroneuromiografia). As jurisprudências foram obtidas através da busca dos julgados sobre o tema, entre 2003 e 2008, pertencentes ao Supremo Tribunal Federal, Superior Tribunal de Justiça, Tribunais Regionais Federais, Tribunal Superior do Trabalho e Tribunais Regionais do Trabalho. Resultados: foram identificados 48 artigos abordando os aspectos diagnósticos das LER-DORT, observando-se que os exames por ressonância magnética, ultrassonografia e eletromiografia demonstraram ser mais efetivos, dentro das suas especificidades, para a complementação do exame clínico de patologias relacionadas às LER-DORT. A análise das 123 jurisprudências selecionadas demonstrou, de forma geral, que as LER-DORT equiparam-se ao acidente de trabalho, devendo apresentar nexo de causalidade (atestado através de laudo médico-pericial) e, ainda, ensejam a ação por danos morais, a qual, devido à EC nº 45 passou a ser competência da Justiça do Trabalho. O Estado da arte dos aspectos periciais encontra-se representado pela vigência da Instrução Normativa n. 98/2003, a qual traz como aspecto fundamental a determinação de novos parâmetros a serem considerados na definição de um quadro de LER-DORT, dispondo, ainda, sobre a conduta ética que deve ser adotada pelo médico perito, bem como chama a atenção para a necessidade dessas doenças do trabalho serem comunicadas às autoridadades competentes, através da emissão da Comunicação de Acidente de Trabalho (CAT). Conclusão: as LER-DORT representam, hoje, um problema de importante impacto, não apenas previdenciário, mas também econômico em diversos países, nos quais o Brasil encontra-se inserido. Estudos sobre o estado da arte relacionados às LER-DORT são fundamentais para auxiliar na construção de um modelo crítico e consciente que colabore com a garantia de sustentabilidade do sistema previdenciário no Brasil

Sistemas de informação geográfica e análise espacial de dados como ferramentas para determinação de agregação espacial de doenças: a hanseníase como modelo

This work demonstrates the importance of using tools used in geographic information systems (GIS) and spatial data analysis (SDA) for the study of infectious diseases. Analysis methods were used to describe more fully the spatial distribution of a particular disease by incorporating the geographical element in the analysis. In Chapter 1, we report the historical evolution of these techniques in the field of human health and use Hansen s disease (leprosy) in Rio Grande do Norte as an example. In Chapter 2, we introduced a few basic theoretical concepts on the methodology and classified the types of spatial data commonly treated. Chapters 3 and 4 defined and demonstrated the use of the two most important techniques for analysis of health data, which are data point processes and data area. We modelled the case distribution of Hansen s disease in the city of Mossoró - RN. In the analysis, we used R scripts and made available routines and analitical procedures developed by the author. This approach can be easily used by researchers in several areas. As practical results, major risk areas in Mossoró leprosy were detected, and its association with the socioeconomic profile of the population at risk was found. Moreover, it is clearly shown that his approach could be of great help to be used continuously in data analysis and processing, allowing the development of new strategies to work might increase the use of such techniques in data analysis in health care

Origem física das curvas sigmoidais respiratórias pressão-volume: recrutamento alveolar e elasticidade não-linear

An important unsolved problem in medical science concerns the physical origin of the sigmoidal shape of pressure volume curves of healthy (and some unhealthy) lungs. Such difficulties are expected because the lung, which is the most important structure in the respiratory system, is extremely complex. Its rheological properties are unknown and seem to depend on phenomena occurring from the alveolar scale up to the thoracic scale. Conventional wisdom holds that linear response, i.e., Hooke s law, together with alveolar overdistention, play a dominant role in respiration, but such assumptions cannot explainthe crucial empirical sigmoidal shape of the curves. In this doctorate thesis, we propose an alternative theory to solve this problem, based on the alveolar recruitment together with the nonlinear elasticity of the alveoli. This theory suggests that recruitment may be the predominant factor shaping these curves in the entire range of pressures normally employed in experiments. The proposed model correctly predicts the observed sigmoidal pressure volume curves, allowing us to discuss adequately the importance of this result, as well as its implications for medical practice

Estudo genético-clínico de mucopolissariodoses no estado do Ceará

As mucopolissacaridoses (MPS) são doenças genéticas raras decorrente da deficiência de enzimas lisossomais envolvidas no catabolismo de glicosaminoglicanos, resultando em um amplo espectro de manifestações clínicas, progressivas e multissistêmicas, exigindo tratamento por uma equipe multidisciplinar. Embora o Nordeste brasileiro seja uma região com grande taxa de consangüinidade e um efeito fundador envolvendo MPS, não há estudos caracterizando os pacientes dessa região. Nosso objetivo foi determinar o perfil epidemiológico, clínico e genético de casos não publicados com MPS provenientes do Ceará, identificando as diferenças entre outros estudos com MPS e possíveis problemas a serem enfrentados para a realização do diagnóstico precoce. O estudo foi seccional, descritivo, com amostra de pacientes com MPS em acompanhamento no Hospital Infantil Albert Sabin e Hospital Geral Cesar Cals no período de 2006-2013. Os dados foram obtidos a partir da avaliação clínica, revisão de prontuários médicos e entrevista com os pacientes e/ou familiares realizadas pelo investigador principal. Cinquenta e três pacientes foram incluídos no estudo (36 do sexo masculino), sendo 6 MPS I, 17 MPS II, 7 MPS III (3 MPSIII-A, 3 MPS III-B, 1 MPS III-C), 7 MPS IV-A, 16 de MPS VI. O óbito ocorreu em 16 casos (3 MPS I, MPS II 6, 1 MPS IIIA , IIIB 1MPS , 1 MPS IV , 4 MPS VI). A amostra foi composta principalmente por crianças. Houve elevada taxa de consangüinidade e recorrência familiar. Os tipos mais comuns foram MPS II e MPS VI. Exceto para macrossomia em MPS II, os dados de nascimento indicam que não houve risco para desenvolvimento de viii complicações perinatais. Os sintomas iniciaram em crianças com menos de 2 anos. As manifestações clínicas foram heterogêneas exceto para atraso no desenvolvimento neurológico em MPS III e manifestações esqueléticas em MPS IV. As principais características clínicas foram macrocefalia, baixa estatura, alterações odontológicas, respiratórias, cardíacas, hepatoesplenomegalia, hérnia umbilical, rigidez articular e anormalidades esqueléticas. A terapia de reposição enzimática foi instituída em 26 casos (4 MPS I, 10 MPS II, 12 MPS VI). Os problemas sócio-econômicos das famílias, o amplo espectro de sintomas e a gravidade da doença foram causas das dificuldades em realizar a avaliação periódica pela equipe multidisciplinar, além de exames complementares de maior custo para determinar as complicações da doença. Este foi o maior estudo transversal sobre MPS no Nordeste do Brasil. Em contraste com a maior incidência de MPS I na maioria das populações ocidentais, houve maior incidência de MPS II e VI. As alterações respiratórias foram um dos principais contribuintes para a mortalidade precoce, exceto nos casos de MPS I, em que a cardiomiopatia foi prevalente. A menor expectativa de vida ocorreu em MPS I. O envolvimento cognitivo foi comum em casos graves e o maior número de órgãos envolvidos representou maior risco de morrer. Para o diagnóstico precoce, deve-se buscar indivíduos afetados em famílias em que há parentes com MPS, além do maior reconhecimento de sinais e sintomas de MPS por profissionais de saúde

Avaliação dos parâmetros hematológicos e imunofenotípicos de pacientes com doenças linfoproliferativas crônicas no Rio Grande do Norte

Chronic lymphoproliferative disorders (DLPC) are lymphoid system diseases characterized by the abnormal proliferation of mature lymphocytes that affect B cells, T lymphocytes and NK cells. The aim of the study was to demonstrate the relevance of immunophenotyping by flow cytometry in patients with prolonged lymphocytosis and / or cytomorphological changes compatible with lymphoproliferative diseases. In this study 460 patients (244 men and 216 women) with DLPC were evaluated. Were analyzed by flow cytometry with a panel of monoclonal antibodies consisting of CD3, CD4, CD5, CD8, CD10, CD19, CD22, CD23, CD25, CD38, CD45, CD16/CD56, and HLADR heavy and light chains of immunoglobulins. It also examines information regarding age, gender of patients and laboratory data as leucocytes, cytomorphological analysis, platelet count and hemoglobin determination. The results showed 398 cases of chronic lymphoproliferative disorders and 62 of DLPC B cell lymphoproliferative diseases T. B showed the following distribution : 253 cases of chronic lymphocytic leukemia (CLL), 42 cases of multiple myeloma ( MM ), 37 cases of lymphoma non - Hodgkin lymphoma in leukemic phase (NHL) , 17 cases of pro- B lymphocytic leukemia ( B -PLL), 15 cases of mantle cell lymphoma (MCL ), 12 cases of plasma cell leukemia ( PCL), 9 cases of lymphoma Burkitt (Linf B), 8 cases of leukemia villous cells ( LCV), 3 cases of splenic lymphoma with villous cells (LECV), a case of follicular lymphoma (LF) and a Waldenströn macroglobulinemia ( MW). The diseases source NK / T were 23 cases of peripheral T cell lymphoma (LCTP), 14 cases of T prolymphocytic leukemia (T -PLL), 10 cases of leukemia T of large granular lymphocytes (LGL -T) 9 cases of leukemia cells of adult T (LCTA), 5 cases of Sezary syndrome (SS) and a case of large granular NK leukemia (LGL -NK) lymphocytes. In conclusion, the combined use of the monoclonal antibody panel careful cytomorphological analysis was shown to be essential in immune diagnosis and classification of chronic lymphoproliferative disorders. This study was approved by the IRB - HUOL under number 356 / 09