Lung function in Birt-Hogg-Dubé syndrome: a retrospective analysis of 96 patients.

Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominant disorder caused by mutations in the FLCN gene coding for folliculin. Its clinical expression includes cutaneous fibrofolliculomas, renal tumors, multiple pulmonary cysts, and recurrent spontaneous pneumothoraces. Data on lung function in BHD are scarce and it is not known whether lung function declines over time. We retrospectively assessed lung function at baseline and during follow-up in 96 patients with BHD. Ninety-five percent of BHD patients had multiple pulmonary cysts on computed tomography and 59% had experienced at least one pneumothorax. Mean values of forced expiratory volume in 1 second (FEV <sub>1</sub> ), forced vital capacity (FVC), FEV <sub>1</sub> /FVC ratio, and total lung capacity were normal at baseline. Mean (standard deviation) residual volume (RV) was moderately increased to 116 (36) %pred at baseline, and RV was elevated > 120%pred in 41% of cases. Mean (standard deviation) carbon monoxide transfer factor (DLco) was moderately decreased to 85 (18) %pred at baseline, and DLco was decreased < 80%pred in 33% of cases. When adjusted for age, gender, smoking and history of pleurodesis, lung function parameters did not significantly decline over a follow-up period of 6 years. Cystic lung disease in BHD does not affect respiratory function at baseline except for slightly increased RV and reduced DLco. No significant deterioration of lung function occurs in BHD over a follow-up period of 6 years

Efeito de dietas com níveis crescentes de caroço de algodão integral sobre a composição química e o perfil de ácidos graxos da carne de cordeiros Santa Inês Effect of diets with increasing levels of whole cotton seed on chemical composition and fatty acid profile of Santa Inez (Santa Inês) lamb meat

Esta pesquisa foi realizada com o objetivo de avaliar o efeito da inclusão (0, 20, 30 e 40%) de caroço de algodão integral (Gossypium hirsutum) na dieta sobre a composição química e o perfil de ácidos graxos da carne de cordeiros Santa Inês. Foram utilizados 24 cordeiros machos não-castrados (peso corporal inicial de 19,0 ± 0,2 kg e 4 meses de idade), todos criados em regime de confinamento em baias individuais. Os níveis de caroço de algodão integral não afetaram a composição centesimal e os percentuais de colesterol e fosfolipídios da carne ovina. Entretanto, houve diferença entre os percentuais dos ácidos graxos mirístico, palmítico e linolênico e entre a relação C18:0 + C18:1 / C16:0. Do ponto de vista nutricional, a utilização de caroço de algodão integral na dieta pode ser recomendada, durante períodos curtos, em níveis de até 40% para ovinos em terminação. Ressalta-se que o caroço de algodão integral é um subproduto economicamente viável por apresentar baixo custo de produção em comparação ao milho e à soja.<br>The objective of this research was to evaluate the effect of inclusion (0, 20, 30 and 40%) of whole cottonseed (Gossypium hirsutum) in the diet on chemical composition and fatty acids profile of Santa Inez sheep meat. Twenty four no castrated male sheep were used, (initial 19.0 ± 0.2 kg BW and 4 month old), all kept in confinement regime in individual stalls. The levels of whole cottonseed did not affect chemical composition and the percentages of cholesterol and phospholipids of the lamb meat. However, there was difference among the percentages of the miristic, palmitic and linolenic fatty acids and also to the relationship C18:0 + C18:1 / C16:0. At nutritional point of view, the utilization of whole cottonseed could be recommended, during short periods, up to the level of 40% for finishing animals. In addition, whole cottonseed is a by-product economically viable for presenting low production cost as compared to corn and soybean

Alzheimer's disease and symbiotic microbiota: an evolutionary medicine perspective

Microorganisms resident in our bodies participate in a variety of regulatory and pathogenic processes. Here, we describe how etiological pathways implicated in Alzheimer’s disease (AD) may be regulated or disturbed by symbiotic microbial activity. Furthermore, the composition of symbiotic microbes has changed dramatically across human history alongside the rise of agriculturalism, industrialization, and globalization. We postulate that each of these lifestyle transitions engendered progressive depletion of microbial diversity and enhancement of virulence, thereby enhancing AD risk pathways. It is likely that the human life span extended into the eighth decade tens of thousands of years ago, yet little is known about premodern geriatric epidemiology. We propose that microbiota of the gut, oral cavity, nasal cavity, and brain may modulate AD pathogenesis, and that changes in the microbial composition of these body regions across history suggest escalation of AD risk. Dysbiosis may promote immunoregulatory dysfunction due to inadequate education of the immune system, chronic inflammation, and epithelial barrier permeability. Subsequently, proinflammatory agents—and occasionally microbes—may infiltrate the brain and promote AD pathogenic processes. APOE genotypes appear to moderate the effect of dysbiosis on AD risk. Elucidating the effect of symbiotic microbiota on AD pathogenesis could contribute to basic and translational research