Inhaler training and asthma knowledge are associated with a higher proportion of patients with correct inhaler technique in young but not in elderly asthmatic patients.

Objective: Incorrect inhaler usage is frequent, particularly in elderly asthmatic patients. This study aimed at comparing inhaler technique errors and their determinants, as well inhaler technique self-perception versus real performance, between elderly and non-elderly asthmatics. Methods: Cross-sectional assessment of 92 elderly and 100 non-elderly asthmatics followed at specialty clinics. A standardized questionnaire was applied and inhaler technique demonstration was requested. Errors were assessed using checklists based on manufacturers' instructions and inhaler technique was graded as correct, acceptable or incorrect. Chi-Square Test and Fischer's Exact Test were used for comparative analysis of nominal variables. A p value equal to or less than 0.05 was considered statistically significant. Results: Inhaler technique was correct in a minority of elderly and young patients, without significant differences between study groups. Only 11.1% of the elderly who classified their inhaler as easy and 12.7% who stated their technique was correct had no errors. Previous regular inhaler training was associated with better actual performance in young but not in elderly patients. Conclusion: Our study showed that in spite of regular follow up at specialized outpatient clinics, inhaler devices are associated with a high frequency of errors in elderly and non-elderly asthmatics. In addition, most patients tend to overestimate their technique as correct. Finally, previous, frequent training was associated with a significantly higher percentage of patients showing correct or acceptable technique but only in non-elderly asthmatics, which suggests that elderly asthmatics may need specifically tailored inhaler education programs.

Generation of patient-specific pluripotent induced stem cell line UFRJi007-A from a Brazilian familial amyotrophic lateral sclerosis patient.

Induced pluripotent stem cell (iPSC) line were generated from erythroblasts of a Brazilian patient with familiar form of amyotrophic lateral sclerosis (ALS). NGS analysis demonstrated that patient carried a mutation in SOD1 gene, as well as a deletion in FUS gene. CytoTune™-iPS 2.0 Sendai Reprogramming Kit (containing the reprogramming factors OCT3/4, KLF4, SOX2 and cMYC) was used to generate the cell lines. The iPSCs express pluripotency markers, have normal karyotype and differentiated spontaneously in the three germ layers. The expression of Sendai virus was lost in all iPSC lines after 15 passages.

Generation of four patient-specific pluripotent induced stem cell lines from two Brazilian patients with amyotrophic lateral sclerosis and two healthy subjects.

Induced pluripotent stem cell (iPSC) lines were generated from erythroblasts of two patients with amyotrophic lateral sclerosis (ALS) and two healthy individuals. One familial and one sporadic ALS patients were used, both with genetic alterations in VAPB gene. CytoTune™-iPS 2.0 Sendai Reprogramming Kit (containing the reprogramming factors OCT3/4, KLF4, SOX2 and cMYC) was used to generate the iPSC cell lines. The four iPSCs express pluripotency markers, have normal karyotype and differentiated spontaneously in the three germ layers. The expression of Sendai virus was lost in all iPSC lines after 15 passages.

Reaching 100 in the Countryside: Health Profile and Living Circumstances of Portuguese Centenarians from the Beira Interior Region.

The interest in studying a specific population of centenarians who lives in the country's interior region (PT100-BI) emerged during the first Portuguese systematic study about centenarians (PT100 Oporto Centenarian Study). This region of Portugal is predominantly rural and is one of the regions with the largest number of aged people. The aim of this study is to provide information on the centenarians who live in the Beira Interior region, specifically in terms of their health status and the health services they use. A total of 101 centenarians (mean age: 101.1 years; SD = 1.5 years), 14 males and 87 females, were considered. Most centenarians lived in the community, and 47.6% lived in nursing homes. Nearly half (47.5%) presented cognitive functioning without deficits. A noteworthy percentage presented conditioned mobility and sensory problems. The most common self-reported diseases include urinary incontinence (31.7%), high blood pressure (23.8%), and heart conditions (19.8%). Despite these health and functional characteristics, formal support services and technical assistance were found to be scarcely used. Further research is needed to understand how the role of contextual variables and the countryside environment contribute to the centenarians' adaptation to advanced longevity.

Consumo de aminoácido de cadeia ramificada na esclerose lateral amiotrófica: suplemento proteico ou substância neurotóxica? / Branched-chain amino acid consumption in amyotrophic lateral sclerosis: protein supplement or neurotoxic substance?

Estudos demonstraram efeito positivo principalmente no ganho ponderalem pacientes com esclerose lateral amiotrófica (ELA), suplementadoscom aminoácidos de cadeia ramificada (AACR). Achados recentes têm mostrado que o consumo excessivo e crônico de AACR pode contribuir para a progressão da doença, provavelmente devido a estes serem precursores do glutamato. O objetivo deste estudo foi avaliar a evidência acerca da utilização dos AACR por pacientes com ELA, a fim de elucidar questões pertinentes a sua ingestão. Foi feita busca em base de dados de artigos científicos relacionados ao consumo de AACR na ELA, no período de 1988 a 2013. Foram encontrados seis artigos relacionados ao consumo de AACR por pacientes com ELA. Desses, um referiu melhora da força, enquanto os outros relataram ganho ponderal ou não mostraram resultados significativosem relação aos desfechos. Além disso, foi possível observar uma estreita relação entre o consumo excessivo e crônico dos AACR com o agravamento da doença. À luz dos conhecimentos ora disponíveis, a suplementação com AACR não é recomendada devido aos possíveis efeitos nocivos. O consumo adequado de alimentos proteicos, fontes desses aminoácidos, deve ser utilizado pelos pacientes, respeitando as recomendações estabelecidas. No entanto, estudos adicionais devem ser desenvolvidos em virtude do escasso número de publicações disponíveis.

Studies have shown positive effect mainly in weight gain inpatients with amyotrophic lateral sclerosis (ALS) supplemented with branched chain amino acids (BCAA). However, recent studies have shown that excessive and chronic intake has contributed to the worsening of the disease progression, probably because the amino acids are glutamate precursors. The objective of this study was to assess the evidence about the use of BCAA by patients with ALS, with the aim to clarify pertinent issues for its intake. A search was conducted in data bases for scientific papers related to the intake of BCAA in ALS, between 1988 and 2013. For these review six articles related to the use of BCAA in ALS were found. Of these, one described strength improvement, while the remaining reported weight gain or no significant effects in relation to the outcome. Additionally, it waspossible to observe a close relationship between the excessive and chronic BCAA intake with the worsening of the disease. Considering the presente day available knowledge BCAA supplementation should not be indicated due to the possible harmful effect. The intake of appropriated protein foods should be consumed by these patients, respecting the suggested recommendation. However, more studies are necessary due to the scarce papers in this area.

Clinical aspects of amyotrophic lateral sclerosis in Rio de Janeiro/Brazil.

OBJECTIVE: The clinical and epidemiological profile of sporadic amyotrophic lateral sclerosis (ALS), a chronic, degenerative, progressive motor neuron disease of unknown etiology, was described and evaluated in the city of Rio de Janeiro. METHOD: Patients with a diagnosis definite of ALS according to the revised criteria of the El Escorial World Federation of Neurology were included in this retrospective, descriptive study (n=227). Demographic data, clinical variables, mortality and survival of these patients were assessed. RESULTS: Of the 227 included cases, 143 (63%) were male and 84 (37%) were female, resulting in a male/female ratio of 1.7:1. Mean age at onset of the disease was 53.6 ± 12.1 years, overall median survival time was 49 months (95%CI: 42.4-55.5) and the majority of patients (71.4%) were white, black patients 15.9% and mulattos 12.8%. The most common forms of the disease were classic and bulbar ALS. CONCLUSION: Taking classic and bulbar ALS together, the disease was more common in white, male patients of 50 to 70 years of age. When analyzed separately, the bulbar form was more common in women and in older patients. Survival of patients with bulbar ALS was shorter compared to that of patients with classic ALS.

Importância das infecçöes respiratórias agudas (IRA) em crianças no município de Säo Pualo / Importance of acute respiratory infections in children in the municipality of Säo Paulo

Através do estudo da demanda anual por IRA, da letalidade global e específica por pneumonias agudas, realizado no Departamento de Pediatria da Faculdade de Ciências Médicas da Santa Casa de Säo Paulo e dos dados estatísticos oficiais sobre mortalidade por doenças respiratórias, procura-se evidenciar a importância destas doenças como problema de saúde pública na cidade de Säo Paulo. Os resultados obtidos permitem concluir pela altíssima incidência das IRA na infância e pela pequena proporçäo de casos graves. A demanda, letalidade e a mortalidade alcançaram níveis muito elevados, principalmente no primeiro ano de vida. Säo necessários estudos populacionais multicêntricos bem com pesquisas sobre características das doenças graves, a fim de aumentar nossos conhecimentos sobre a epidemiologia destas enfermidades em diferentes áreas. Paralelamente, é importante elaborar e viabilizar programas para o controle deste grupo de doenças