Primary spontaneous pneumothorax (PSP) is a common manifestation of Birt-Hogg-Dubé syndrome caused by folliculin gene (FLCN) mutation, which is also found in isolated familial PSP cases. A complete genetic analysis of FLCN was performed in 102 unrelated Chinese patients with isolated PSP and 21 of their family members. Three novel mutations (c.924_926del, c.1611_1631del and c.1740C>T) and a previously reported mutation (c.1733insC) were identified in five familial and five sporadic PSP patients. Of the 21 family members of patients with PSP including 3 previous considered as sporadic, 4 (19%) had history of at least one episode of PSP and 9 (43%) were FLCN mutant carriers without PSP. Seven of the nine (78%) mutant carriers had pulmonary cysts detected by high-resolution computed tomography (HRCT). Although c.924_926del and c.1611_1631del were found in eight patients from the same geographic district, haplotype analysis demonstrated that they did not share the same affected haplotype, thus excluding common ancestry. This study first demonstrates that FLCN mutation contributes to not only familial but also 'apparently sporadic' patients with isolated PSP. It suggests that mutation analysis and HRCT scan may be recommended for first-degree family members of PSP patients with FLCN mutations, irrespective of their family history status of PSP.
OBJECTIVES: To evaluate the association between anthropometric parameters and lipid levels among Taiwanese school children. DESIGN AND METHODS: Using a probability-proportional-to size sampling and multi-stages sampling procedure, we sampled 1500 school children from 10 schools in Taipei city. Anthropometric parameters including body weight, body height, waist circumference, hip circumference and skinfolds were measured. Serum total cholesterol (CHOL), triglycerides (TG), high density lipoprotein-cholesterol (HDL-C), apolipoprotein A1 and B (ApoA1 and ApoB) were measured by standard methods, low density lipoprotein-cholesterol (LDL-C) and CHOLaHDL-C ratio were calculated by formula. RESULTS: We included in our analyses 1366 children (681 boys and 685 girls) with a mean age of 13.3 y (from 12 to 16 y) and with valid anthropometric and biochemical parameters. The boys had higher body height (P`0.001) and larger body weight (P`0.05), waist circumference (P`0.01) and waistahip ratio (WHR, P`0.001) than the girls. However, the girls had larger skinfolds than the boys. After adjusting for age, girls had higher total CHOL, TG, HDL-C, LDL-C, ApoA1 and ApoB concentrations than boys. In general, TG was positively associated with most anthropometric parameters (except body height); a similar negative association between HDL-C and anthropometric variables was noted. After controlling, for age, cigarette smoking, alcohol drinking and puberty development, shorter body height was the strongest predictor of total CHOL, LDL-C and ApoB concentrations among boys. Although body mass index (BMI) was a signi®cant positive predictor (P`0.01) of the CHOLaHDL-C ratio; skinfold measurements were the strongest anthropometric predictors of most lipid concentrations among boys. Among girls, we found WHR and BMI to be the strongest positive predictors of TG and ApoB level respectively (both P`0.001), but skinfold measurements were best for predicting HDL-C, LDL-C, ApoA1 and the CHOLaHDL-C ratio. CONCLUSIONS: From this large study of school-age children from Taiwan, we found anthropometric parameters, such as body height, BMI or WHR, are adequate predictors of blood lipid levels; however, skinfold measurements are generally more strongly associated with lipid levels in both genders.
The direct injection by thoracoscope of mesenchymal stem cells (MSCs) that had been genetically modified to express angiopoietin-1 was investigated in a porcine model to determine their effect on arteriogenesis and the effectiveness of this technique. Chronic myocardial ischaemia was established using a thoracoscope to insert an ameroid constrictor around the left circumflex coronary artery. Six weeks after establishing the ischaemia, 20 pigs were randomly divided into three groups to receive injections by thoracoscope of either genetically-modified MSCs, unmodified MSCs or phosphate-buffered saline into the ischaemic border area. The injections were repeated 1 month later. The genetically modified MSCs were found to restore blood flow significantly more than the other observed treatments and immunohistochemical evaluation of arteriogenesis supported this finding. In conclusion, the injection of MSCs that had been genetically modified to express angiopoietin-1 improved arteriogenesis and increased collateral blood flow in the myocardial ischaemic area. Thoracoscope delivery of the injection was safe and minimally invasive.
In this paper, the isothermal crystallization of nylon 6 in the presence of Kevlar 129 fiber was investigated by polarized optical microscopy (POM). The formation of a transcrystalline domain was found to be mainly controlled by crystallization conditions, such as the temperature of the isothermal crystallization, residual time at melting temperature and the cooling rate of the melt. The nucleation rate of nylon 6 on the fibers was mainly affected by the crystallization temperature. The interfacial transcrystallinity of nylon 6 occurred on the surface of Kevlar 129 fiber in the temperature range 130-190 • C. The reason for the formation of interfacial transcrystalline morphology is discussed from the molecular level, based on the understanding of the packing mode of nylon 6 chains around fibers and the interaction between matrix and fibers. It was found that the lattice matching and hydrogen-bonding between nylon 6 and poly(p-phenylene terephthalamide) (PPTA) crystals play an important role in the epitaxial crystallization.
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