Clinical Characteristics and Outcomes of Patients Screened for but Deemed Clinically Not Suitable for Transcatheter Mitral Valve Replacement: DECLINE-TMVR Registry.

BACKGROUND: Transcatheter therapies are a recognized alternative intervention in patients with severe mitral regurgitation who are at high surgical risk. The purpose of this study was to characterize patients screened for transcatheter mitral valve replacement (TMVR), establish the clinical and anatomic reasons for unsuitability, and determine clinical course and early outcomes. METHODS: International multicentre registry was conducted of consecutive patients screened for TMVR at 12 centres in Europe, the United States, and Canada between April 2015 and September 2018. Patient-level retrospective data were collected for all patients screened. RESULTS: From a total of 294 patients, 87 (30%) patients were suitable for and underwent TMVR, whereas 207 (70%) patients were unsuitable for TMVR. There was no difference in Society of Thoracic Surgeons predicted risk of mortality (6.3% ± 4.3% vs 6.7 ± 6.1%, P = 0.52) for mitral valve replacement between the groups. The most common reasons for TMVR unsuitability were mitral annular size outside therapeutic range (28%) and small predicted neo-LVOT (25%). Preprocedural multidetector computed tomographic demonstrated that patients unsuitable for TMVR had smaller predicted neo-left ventricular outflow tract (LVOT) area (318 ±192 mm2 vs 495 ± 202 mm2, P = 0.04). At 30 days, there was no difference in rates of rehospitalization (8% vs 8%, P = 0.21), stroke (1% vs 2%, P = 0.42), or mortality (4% vs 10%, P = 0.10), unadjusted for procedural risk, between unsuitable for TMVR and TMVR groups, respectively. CONCLUSIONS: Two-thirds of patients failed screening as anatomically unsuitable for TMVR. The findings of this study have important clinical implications, highlighting an unmet clinical need and provide a target for design innovation in future iterations of TMVR devices.

Situaciones de violencia por parte de pacientes percibidos por personal de enfermería en un hospital público Corrientes capital 2021 / Situations of violence by patients received by nursing personnel in a public hospital Corrientes capital 2021

Describir las situaciones de violencia por parte de los pacientes percibidas por el personal de enfermería en una institución de salud pública de Corrientes capital durante 2021. Metodología: Estudio observacional, descriptivo, transversal. Muestreo de tipo consecutivo. La población personal de enfermería asistencial de un hospital. Variables: edad, género, instrucción, antigüedad laboral, área de trabajo, turno, identifcación de maltrato físico, verbal y asedio sexual, principal modo de actuar e impacto frente a situaciones de violencia. Recolección de datos mediante cuestionario Google Forms con consentimiento informado anónimo, que fue validado por prueba piloto. Análisis con Microsof Excel y Epidat 4.1. Resultados: Población estudiada 115 trabajadores de enfermería; 69,57% mujeres; rango etario 22-56 años, promedio 35,5 años. Formación: 58,26% enfermeros, 22,61% licenciados en enfermería y 19,13% auxiliares en enfermería. Turno: 37,39% mañana, 30,43% tarde, 17,39% noche y 14,78% rotativo. Área laboral: cuidados intensivos 27,83%, clínica médica 21,74%, clínica quirúrgica 15,65%, emergencias 15,65%, consultorios 10,43%, coronaria 8,70%. De los entrevistados, 89,56% había percibido algún tipo de violencia, de estos 61,17% estaba entre 22 y 38 años. La percepción de violencia mostró que 92,53% eran enfermeros, 88,46% licenciados y 81,81% auxiliares de enfermería. Los tipos de violencia identificados fueron maltrato verbal en el 86,09%, maltrato físico en el 72,17% y asedio sexual en el 24,35%. El principal género afectado por los hechos violentos fue el femenino y el turno de trabajo con mayor identificación de situaciones violentas fue el de la mañana. En cuanto a los modos de proceder para comunicar a la institución las situaciones vividas, el 66,96% lo reportaron a un superior Los hechos de violencia según área de trabajo se reportaron por 80% de los de unidad coronaria, 84,38% de unidad de cuidados intensivos, 88,89% de emergencias, 92% de clínica médica, 94,44% de clínica quirúrgica y 100% de consultorios externos. Conclusión: La violencia hacia enfermería está presente en el hospital y afecta más a las mujeres. La forma más frecuente es la verbal, seguida de la física y del asedio sexual. En general frente a las agresiones optan por no hacer nada[AU]

To describe the situations of violence by patients perceived by the nursing staff in a public health institution in Corrientes capital during 2021. Methodology: Observational, descriptive, cross-sectional study. Consecutive type sampling. Te nursing staff population of a hospital. Variables: age, gender, education, job seniority, work area, shif, identifcation of physical, verbal and sexual abuse, main mode of action and impact in situations of violence. Data collection through Google Forms questionnaire with anonymous informed consent, which was validated by pilot test. Analysis with Microsof Excel and Epidat 4.1.Results: Population studied 115 nursing workers; 69.57% women; age range 22-56 years, average 35.5 years. Training: 58.26% nurses, 22.61% nursing graduates and 19.13% nursing assistants. Shif: 37.39% morning, 30.43% afernoon, 17.39% night and 14.78% rotating. Work area: intensive care 27.83%, medical clinic 21.74%, surgical clinic 15.65%, emergencies 15.65%, doctor's ofces 10.43%, coronary 8.70%. Of those interviewed, 89.56% had perceived some type of violence, of these 61.17% were between 22 and 38 years old. Te perception of violence showed that 92.53% were nurses, 88.46% licensed and 81.81% nursing assistants. Te types of violence identifed were verbal abuse in 86.09%, physical abuse in 72.17% and sexual harassment in 24.35%. Te main gender affected by violent acts was female, and the work shif with the highest identifcation of violent situations was the morning shif. Regarding the ways of proceeding to communicate the situations experienced to the institution, 66.96% reported it to a superior Te acts of violence according to work area were reported by 80% of those in the coronary unit, 84.38% of intensive care unit, 88.89% emergencies, 92% medical clinic, 94.44% surgical clinic and 100% outpatient clinics. Conclusion: Violence towards nursing is present in the hospital and affects women more. Te most frequent form is verbal, followed by physical and sexual harassment. In general, when faced with aggression, they choose to do nothing[AU]

Descrever as situações de violência por pacientes percebidas pela equipe de enfermagem em uma instituição pública de saúde da capital Corrientes durante o ano de 2021. Metodologia: Estudo observacional, descritivo, transversal. Amostragem do tipo consecutivo. A população da equipe de enfermagem de um hospital. Variáveis: idade, sexo, escolaridade, tempo de serviço, área de atuação, turno, identifcação de abuso físico, verbal e sexual, principal forma de atuação e impacto em situações de violência. Coleta de dados por meio de questionário Google Forms com consentimento informado anônimo, que foi validado por teste piloto. Análise com Microsof Excel e Epidat 4.1. Resultados: População estudada 115 trabalhadores de enfermagem; 69,57% mulheres; faixa etária de 22 a 56 anos, média de 35,5 anos. Formação: 58,26% enfermeiros, 22,61% graduados em enfermagem e 19,13% auxiliares de enfermagem. Turno: 37,39% matutino, 30,43% tarde, 17,39% noturno e 14,78% rotativo. Área de atuação: terapia intensiva 27,83%, clínica médica 21,74%, clínica cirúrgica 15,65%, emergências 15,65%, clínicas 10,43%, coronariana 8,70%. Dos entrevistados, 89,56% já perceberam algum tipo de violência, destes 61,17% tinham entre 22 e 38 anos. A percepção da violência mostrou que 92,53% eram enfermeiros, 88,46% licenciados e 81,81% auxiliares de enfermagem. Os tipos de violência identifcados foram abuso verbal em 86,09%, abuso físico em 72,17% e assédio sexual em 24,35%. O principal gênero acometido por atos violentos foi o feminino, e o turno de trabalho com maior identifcação de situações de violência foi o turno da manhã. Em relação às formas de proceder para comunicar as situações vivenciadas à instituição, 66,96% relataram a um superior Os atos de violência por área de trabalho foram relatados por 80% daqueles na unidade coronariana, 84,38% na unidade de terapia intensiva, 88,89% emergências, 92% clínica médica, 94,44% clínica cirúrgica e 100% ambulatório. Conclusão: A violência contra a enfermagem está presente no hospital e atinge mais as mulheres. A forma mais frequente é a verbal, seguida do assédio físico e sexual. Em geral, diante da agressão, optam por não fazer nada[AU]

Long-term follow-up in patients with rheumatic mitral stenosis and severe pulmonary artery hypertension submitted to percutaneous mitral balloon commissurotomy

BACKGROUND: Percutaneous balloon mitral commissurotomy (PMBC) is an attractive therapeutic approach in patients with mitral stenosis. The aim of this study was to assess the immediate and long-term clinical, echocardiographic and haemodynamic outcomes of PMBC in patients with severe pulmonary hypertension (PH). METHODS: Among all procedures (in more than two decades of experience), PMBC was performed from 1987 until 2011 at a single-center in 147 patients who had significant PH defined as baseline pulmonary artery mean pressure (PAMP) (systolic pulmonary pressure > 75 mmHg). All-cause mortality, need for mitral valve replacement (MVR) or new PMBC, and valve restenosis were evaluated during follow-up yearly. RESULTS: Mean age was 33.8 ± 12.8 years and 83.6% (123 patients) were women. Primary success was achieved in 89.8% of the patients (132 patients). Mitral valve area (MVA) increased from 0.83 ± 0.17 cm2 to 2.03± 0.35 cm2 (p<0.001), and at 20-years, mitral valve area was 1.46± 0.34 cm2 (p=0.235). Systolic pulmonary artery pressure decreased from 87.0 ± 6.0 mmHg to 60.0 ± 0.9 mmHg (p<0.001) The rates of all-cause mortality, need for MVR, new PMV, and valve restenosis were 0.67%, 20.0%, 8.78% and 30.4%, respectively, in long-term follow- up (mean 15.6 ± 4.9 years). CONCLUSIONS: PMBC is a safe and effective technique for the treatment of patients with mitral stenosis and PH. A significant decrease in pulmonary pressure was observed after commissurotomy. Although there was a gradual decrease of MVA at long-term follow-up, most patients remained asymptomatic and without major adverse events.

Mosquito (Diptera: Culicidae) larval ecology in natural habitats in the cold temperate Patagonia region of Argentina.

BACKGROUND: Knowledge of immature habitats is an important focus for investigations of mosquito community ecology, and may improve our understanding of how environmental variables increase risk of mosquito-borne diseases by influencing the distributions and abundances of species. In Patagonia region, where climatic and ecological factors could be only borderline suitable for mosquito development, relatively little is known about larval ecology. The present study focuses on associations of environmental conditions in natural aquatic habitats with abundances of mosquito species that have colonized such habitats in Patagonia. METHODS: We described the mosquito community composition within 26 natural temporary pools, and assessed the general relationships between environmental variables (pH, water temperature, conductivity, salinity, dissolved oxygen, aquatic plant cover and main nutrients) and larval abundances using redundancy analysis (RDA). Additionally, we compiled monthly climate data and vegetation indices for each larval habitat, and estimated the probability of presence for two of the most abundant species, describing through generalized linear models (GLM) the environmental, climatic and landscape variables-probability of occurrence relationships. RESULTS: Seven species belonging to the genera Culex and Aedes were identified, with Culex apicinus, Cx. acharistus and Aedes albifasciatus being the most abundant. Mean larval densities were low (6.8 ± 2.8 larvae/dip), and the highest species richness and larval densities were recorded in northern and central areas. Aedes albifasciatus, a species of sanitary importance, was widely distributed, being the only one collected south of the 45th parallel of S latitude. RDA indicated that aquatic conductivity, pH, water depth, dissolved oxygen, ammonia and soluble reactive phosphorous accounted for the main part of the variation in the species composition. According to GLMs, wind speed was the variable that best described the presence of Ae. albifasciatus, and the probability of finding this species was positively associated with high wind speed values. On the other hand, the EVI vegetation index was the only variable included in the Cx. apicinus model, whereby there was a great probability of presence in arid areas with lower EVI values. CONCLUSIONS: Our results enhance our knowledge of larval habitat ecology under the extreme environmental conditions of Patagonia and will guide future efforts to understand how multiple effects can affect mosquito ecology and public health at higher latitudes.

Effect of Aortic Regurgitation by Cardiovascular Magnetic Resonance After Transcatheter Aortic Valve Implantation.

Cardiovascular magnetic resonance (CMR) has demonstrated a high accuracy for evaluating the severity of aortic regurgitation (AR). However, scarce data exist on the impact of AR as evaluated by CMR on clinical outcomes following transcatheter aortic valve implantation (TAVI). The objective of this study was to evaluate the impact of AR as determined by CMR on clinical outcomes (mortality, heart failure [HF] hospitalization) post-TAVI. A total of 448 TAVI recipients from 2 centers (mean age: 80 ± 7 years, mean STS: 5.8 ± 5.4%) who survived the periprocedural period with no pacemaker implantation were included. A newer generation transcatheter valve system was used in 213 patients (48%). The CMR examination was performed at a median of 12 (IQR: 7 to 21) days post-TAVI. After a mean follow-up of 24 ± 19 months, a total of 94 patients (21%) had died and 72 patients (16%) had at least 1 hospitalization because of decompensated HF. The aortic regurgitation fraction (RF) as determined by CMR was an independent predictor of mortality (hazard ratio[HR]:1.06 for each increase of 10%, 95% confidence interval [CI]: 1.01 to 1.12, p = 0.03) and HF hospitalization (HR:1.15 for each increase of 10%, 95% CI:1.02 to 1.30, p = 0.02). The rate of moderate-severe CMR-AR defined as a RF ≥30% was 3%, and this was associated with an increased risk of mortality (HR: 2.63, 95% CI: 2.30 to 2.99, p <0.001) and HF hospitalization (HR: 2.96, 95% CI: 1.62 to 5.42, p ˂0.001). A stepwise increase in the risk of mortality and HF hospitalization was observed with an increase in AR severity, with a peak increase among patients with RF ≥30%. In conclusion, our results showed the clinical usefulness of evaluating AR severity by CMR post-TAVI. CMR would be particularly helpful in doubtful cases or those with discordances between echocardiography and clinical data.

Very Long Term Follow-Up After Percutaneous Balloon Mitral Valvuloplasty

OBJECTIVES: The aim of this study was to assess very long term outcomes after successful percutaneous balloon mitral valvuloplasty (PBMV).BACKGROUND: PBMV remains the preferred treatment for patients with severe symptomatic rheumatic mitral stenosis and suitable anatomy.METHODS: All consecutive patients who underwent successful PBMV between 1987 and 2010 were included. The primary endpoint was the composite of all-cause mortality, need for mitral surgery, or repeat PBMV up to 23 years.RESULTS: Among all 1,582 consecutive patients undergoing PBMV, acute success was achieved in 90.9% (n»1,438).Independent predictors of acute success included left atrial size (odds ratio: 0.96; 95% confidence interval [CI]: 0.93 to0.99; p»0.045), Wilkins score#8 (odds ratio: 1.66; 95% CI: 0.48 to 0.93; p»0.02) and age (odds ratio: 0.97; 95% CI:0.96 to 0.99; p»0.006). Very long-term follow-up (median 8.3 years, mean 15.6 years) was obtained in 79.1% of successful cases. The incidence of the primary endpoint was 19.1% (95% CI: 17.0% to 21.1%). The rates of overall lmortality, need for mitral valve surgery, or repeat PBMV were 0.6% (95% CI: 0.3% to 1.2%), 8.3% (95% CI: 7.0% to9.9%), and 10.0% (95% CI: 8.5% to 11.7%), respectively. On multivariate analysis, New York Heart Association functional class III or IV (hazard ratio: 1.62; 95% CI: 1.26 to 2.09; p<0.001), higher age (hazard ratio: 0.97; 95% CI: 0.96 to0.98; p»0.028), and mitral valve area#1.75 cm2after the procedure (hazard ratio: 1.67; 95% CI: 1.28 to 2.11;p»0.028) were independent predictors of the primary endpoint. CONCLUSIONS: In very long term follow-up, more than 75% of patients exhibited sustained results. Prediction of late favorable results is multifactorial and strongly determined by age, previous symptoms and post-procedural mitral valve area.(J Am Coll Cardiol Intv 2018;11:1945­52) © 2018 by the American College of Cardiology Foundation.

Very Long Term Follow-Up After Percutaneous Balloon Mitral Valvuloplasty.

OBJECTIVES: The aim of this study was to assess very long term outcomes after successful percutaneous balloon mitral valvuloplasty (PBMV). BACKGROUND: PBMV remains the preferred treatment for patients with severe symptomatic rheumatic mitral stenosis and suitable anatomy. METHODS: All consecutive patients who underwent successful PBMV between 1987 and 2010 were included. The primary endpoint was the composite of all-cause mortality, need for mitral surgery, or repeat PBMV up to 23 years. RESULTS: Among all 1,582 consecutive patients undergoing PBMV, acute success was achieved in 90.9% (n = 1,438). Independent predictors of acute success included left atrial size (odds ratio: 0.96; 95% confidence interval [CI]: 0.93 to 0.99; p = 0.045), Wilkins score ≤8 (odds ratio: 1.66; 95% CI: 0.48 to 0.93; p = 0.02) and age (odds ratio: 0.97; 95% CI: 0.96 to 0.99; p = 0.006). Very long term follow-up (median 8.3 years, mean 15.6 years) was obtained in 79.1% of successful cases. The incidence of the primary endpoint was 19.1% (95% CI: 17.0% to 21.1%). The rates of overall mortality, need for mitral valve surgery, or repeat PBMV were 0.6% (95% CI: 0.3% to 1.2%), 8.3% (95% CI: 7.0% to 9.9%), and 10.0% (95% CI: 8.5% to 11.7%), respectively. On multivariate analysis, New York Heart Association functional class III or IV (hazard ratio: 1.62; 95% CI: 1.26 to 2.09; p < 0.001), higher age (hazard ratio: 0.97; 95% CI: 0.96 to 0.98; p = 0.028), and mitral valve area ≤1.75 cm2 after the procedure (hazard ratio: 1.67; 95% CI: 1.28 to 2.11; p = 0.028) were independent predictors of the primary endpoint. CONCLUSIONS: In very long term follow-up, more than 75% of patients exhibited sustained results. Prediction of late favorable results is multifactorial and strongly determined by age, previous symptoms and post-procedural mitral valve area.

Disentangling natural and anthropogenic influences on Patagonian pond water quality.

The water quality of wetlands is governed not only by natural variability in hydrology and other factors, but also by anthropogenic activities. Patagonia is a vast sparsely-populated in which ponds are a key component of rural and urban landscapes because they provide several ecosystem services such as habitat for wildlife and watering for livestock. Integrating field-based and geospatial data of 109 ponds sampled across the region, we identified spatial trends and assessed the effects of anthropogenic and natural factors in pond water quality. The studied ponds were generally shallow, well oxygenated, with maximum nutrient values reported in sites used for livestock breeding. TN:TP ratio values were lower than 14 in >90% of the ponds, indicating nitrogen limitation. Water conductivity decreased from de east to the west, meanwhile pH and dissolved oxygen varied associated with the latitude. To assess Patagonian ponds water status we recommend the measure of total suspended solids and total nitrogen in the water, and evaluate the mallín (wetland vegetation) coverage in a 100m radius from the pond, since those features were significantly influenced by livestock land use. To evaluate the relative importance of natural variability and anthropogenic influences as driving factors of water quality we performed three generalized linear models (GLM) that encompassed the hydrology, hydroperiod and biome (to represent natural influences), and land use (to represent anthropogenic influences) as fixed effects. Our results revealed that at the Patagonian scale, ponds water quality would be strongly dependent on natural gradients. We synthetized spatial patterns of Patagonian pond water quality, and disentangled natural and anthropic factors finding that the dominant environmental influence is rainfall gradient.

Avaliação do Conhecimento em Cuidados Paliativos em Estudantes durante o Curso de Medicina / Evaluation of Medical Students' Knowledge of Palliative Care

RESUMO Introdução É crescente a necessidade de uso de cuidados paliativos (e de suporte em cuidados) nas áreas de saúde, em especial na Medicina. Isto acontece devido ao envelhecimento populacional e ao aumento da prevalência das doenças crônicas não transmissíveis, especialmente câncer metastático e demência avançada. Assim, é muito importante iniciar o processo de ensino-aprendizagem em cuidados paliativos já no curso de graduação em Medicina. Objetivo Avaliar os conhecimentos em cuidados paliativos (CCP)em alunos do curso de Medicina da Escola Superior de Ciências da Saúde (Brasília, DF), bem como averiguar se há ganho de CCP entre os alunos da primeira, quarta e sexta séries. Método Realizou-se um estudo transversal com aplicação de questionário anônimo, com perguntas sobre idade, gênero e mais 19 perguntas sobre CCP. Tais questionários foram aplicados separadamente aos alunos da primeira, quarta e sexta séries do curso de Medicina. Foi usado o teste de Kolmogorov-Smirnov (análise de normalidade). Já o teste de Kruskal-Wallis comparou os três grupos (primeira, quarta e sexta séries), e, caso houvesse diferença com significância estatística, a análise post hoc seria realizada com o teste de Mann-Whitney com correção de Bonferroni (valor de p significativo se <0,016).Resultados: Participaram do estudo 193 estudantes (taxa de inclusão:76,8%;IC95%:71,0%-81,8%), com idades de 23,6±4,3 anos, sendo 100 mulheres (51,8%). Os CCP baseados no número de respostas consideradas corretas por aluno na primeira, quarta e sexta séries foram, em mediana (interquartil), 4,00(2,00-5,00), 10,00(9,00-12,75) e 12,00(10,25-14,00), respectivamente (p< 0,001). Quando comparadas diretamente a primeira com a quarta série, e a primeira com a sexta série, a diferença estatística persiste (p<0,001 e p<0,001, respectivamente); já a comparação entre quarta e sexta séries não demonstra diferença estatística significativa (p:0,041). Conclusão O CCP entre os alunos não é bom, e o ganho de CCP entre a quarta e a sexta série não apresentou significância estatística. Isto denota a necessidade de melhorar o processo de ensino-aprendizagem em cuidados paliativos, principalmente nos cenários do internato.

ABSTRACT Background There is a growing need to use palliative care (and support care) in health areas, particularly in Medicine. This is due to an aging population and the increased prevalence of chronic non-communicable diseases, especially metastatic cancer and advanced dementia. Thus, it is very important to start the teaching-learning process in palliative care during undergraduate medical training. Purpose To evaluate: (1) knowledge in palliative care (KPC) among medical students at the Escola Superior de Ciências da Saúde medical school (Brasília, DF), and (2) the gain in KPC among first, fourth and sixth-year students. Method A cross-sectional study involved the application of an anonymous questionnaire with questions about age, gender, and 19 questions about KPC. These questionnaires were applied separately to the students. The Kolmogorov-Smirnov test was applied. Thereafter, the Kruskal-Wallis test compared the 3 groups (first, fourth and sixth year students), and when any statistically significant difference was verified, post hoc analysis was performed using the Mann-Whitney test with Bonferroni correction (p < 0.016).Results: The study included 193 students (inclusion rate: 76.8%; 95%CI: 71.0%-81.8%), 23.6±4.3 years-old, 100 women (51.8%). The median KPC(interquartile) scores based on the number of correct responses per student of the first, fourth and sixth year of the course were: 4.00(2.00-5.00), 10.00(9.00-12.75), 12.00(10.25-14.00), respectively (p <0.001). When directly comparing the first and fourth year students, and the first and sixth year students, the statistical difference persists (p <0.001 and p <0.001, respectively); but the analysis between fourth and sixth year students shows no statistical difference (p=0.041). Conclusion KPC among our students is limited, and KPC gain between the fourth and sixth years of study was not statistically significant. This shows the need to improve the process of teaching and learning in palliative care, especially in internship scenarios.

Aspectos funcionais das doenças pulmonares fibrosantes / Functional features of fibrotic lung diseases

O objetivo do presente estudo foi avaliar as alterações encontradas na prova de função pulmonar completa, bem como no teste de caminhada de seis minutos, relacionando também a importância dos aspectos funcionais no diagnóstico, na avaliação de resposta ao tratamento e no prognóstico das doenças pulmonares fibrosantes. Assim, nas doenças pulmonares fibrosantes com padrão obstrutivo na prova de função pulmonar completa, deve-se pensar em sarcoidose, linfangioleiomiomatose, pneumonite de hipersensibilidade, histiocitose de células de Langerhans, bronquiolite constritiva e pneumonia intersticial idiopática combinada com enfisema pulmonar. Já na avaliação de resposta ao tratamento, considera-se como má resposta ao tratamento a queda da CVF ≥ 10% e da DLCO ≥ 20%. Em relação ao prognóstico, os pacientes com critérios de má resposta ao tratamento e/ou SpO2≤ 88% no teste de caminhada de seis minutos apresentam uma pior sobrevida.

Lung cyst: an unusual manifestation of Niemann-Pick disease.

Niemann-Pick disease is a rare inherited autosomal recessive disorder, currently classified into six subtypes and characterized by the intracellular accumulation of sphingomyelin in the liver, spleen, lungs, bone marrow or brain. The main pulmonary abnormalities described in high-resolution computed tomography (HRCT) of the chest consist of thickening of the interlobular septa and ground-glass opacities. This case report describes a patient with subtype B Niemann-Pick disease characterized by cysts and ground-glass opacities that were detected on HRCT of the chest.