The present article focuses on the indemnities from public administrations to the Galician fishing sector damaged by the Prestige oil slick. Despite the fact that the fishing sector is compounded of ...quite different activities direct allowances were standardized. We analyze the distributional effects of the uniform indemnities among the four main fishing activities on the area: coastal fishing, aquaculture, seafood harvesting on-foot and seafood harvesting on-boat. We also estimate the total cost of indemnities. The paper shows how a simple re-allocation of public funds could rectifty the redistributive effects of theuniform allowances. We specify the non standard indemnities to the main fishing activities damaged by the oil slick.
Forkhead box protein N1 (FOXN1) transcription factor plays an essential role in the development of thymic epithelial cells, required for T-cell differentiation, maturation, and function. Biallelic ...pathogenic variants in FOXN1 cause severe combined immunodeficiency (SCID). More recently, heterozygous variants in FOXN1, identified by restricted gene panels, were also implicated with causing a less severe and variable immunodeficiency.
We undertook longitudinal follow-up and advanced genetic investigations, including whole exome sequencing and whole genome sequencing, of newborns with a heterozygous variant in FOXN1.
Five patients (3 female, 2 male) have been followed since they were first detected with low T-cell receptor excision circles during newborn screening for SCID. Patients underwent immune evaluation as well as genetic testing, including a primary immunodeficiency panel, whole exome sequencing, and whole genome sequencing in some cases.
Median follow-up time was 6.5 years. Initial investigations revealed low CD3+ T lymphocytes in all patients. One patient presented with extremely low lymphocyte counts and depressed phytohemagglutinin responses leading to a tentative diagnosis of SCID. Over a period of 2 years, CD3+ T-cell counts rose, although in some patients it remained borderline low. One of 5 children continues to experience recurrent upper respiratory infections and asthma episodes. The remaining are asymptomatic except for eczema in 2 of 5 cases. Lymphocyte proliferation responses to phytohemagglutinin were initially low in 3 patients but normalized by age 10 months. In 3 of 5 cases, T lymphocyte counts remain low/borderline low.
In cases of monoallelic FOXN1 variants, using whole exome sequencing and whole genome sequencing to rule out possible other significant pathogenic variants allowed us to proceed with confidence in a conservative manner, even in extreme cases consistent with newborn screen–positive early presentation of SCID.
Our aim was to investigate whether different human leukocyte antigen (HLA) genes might be associated with hepatitis C virus (HCV) infection. DNA obtained from 141 Spanish patients with HCV infection ...(48 with alanine aminotransferase levels in the range considered to be normal, 47 with liver cirrhosis, and 46 with hepatocellular carcinomas HCCs) and from 116 control subjects were typed for HLA-B, HLA-DRB1, and HLA-DQB1 alleles, as well as for major histocompatibility complex class I chain-related gene A (MICA) transmembrane polymorphism. The frequency of HLA-DR11 was increased in HCV carriers, compared with patients with end-stage liver disease (ESLD) (corrected P value Pc, .0002) and, especially, with patients who c had HCC (Pc=.003). The frequency of the HLA-B18 allele was increased in patients with HCC, and the allele c was absent in HCV carriers (Pc=.003). The MICA-A4 allele was overrepresented in patients with HCC, c compared with HCV carriers (Pc=.0002). The DR3/MICA-A4/B18 haplotype was associated with HCC c (Pc=.01). In conclusion, HLA-DR11 seems to be protective against the development of severe forms of c infection, and the DR3/MICA-A4/B18 haplotype may be an important factor in the progression to the most severe HCV-infection status.
Background Psoriasis and atopic dermatitis (AD) are common, complex inflammatory skin diseases. Both diseases display immune infiltrates in lesions and epidermal growth/differentiation alterations ...associated with a defective skin barrier. An incomplete understanding of differences between these diseases makes it difficult to compare human disease pathology to animal disease models. Objective To characterize differences between these diseases in expression of genes related to epidermal growth/differentiation and inflammatory circuits. Methods We performed genomic profiling of mRNA in chronic psoriasis (n = 15) and AD (n = 18) skin lesions compared with normal human skin (n = 15). Results As expected, clear disease classifications could be constructed on the basis of expected immune polarity (TH 1, TH 2, TH 17) differences. However, even more striking differences were identified in epidermal differentiation programs that could be used for precise disease classifications. Although both psoriasis and AD skin lesions displayed regenerative epidermal hyperplasia, which is a general alteration in epidermal growth, keratinocyte terminal differentiation was differentially polarized. In AD, we found selective defects in expression of multiple genes encoding the cornified envelope, with the largest alteration in loricrin (expressed at 2% of the level of normal skin). At the ultrastructural level, the cornified envelope in AD was broadly defective with highly decreased compaction of corneocytes and reduced intercellular lipids. Hence, the entire keratinocyte terminal differentiation program (cytoplasmic compaction, cornification, and lipid release) is defective in AD, potentially underlying the immune differences. Conclusion Our study shows that although alterations in barrier responses exist in both diseases, epidermal differentiation is differentially polarized, with major implications for primary disease pathogenesis.
Objectives The objective of this prospective, single-site, 2-year dietary intervention study was to evaluate the effects of moderate weight reduction and subsequent partial weight regain on ...cardiovascular structure and function. Background Obesity is associated with adverse cardiac and vascular structural and functional alterations. Methods Sixty obese subjects (age 46 ± 10 years, body mass index 37 ± 3 kg/m2 ) were evaluated during their participation in a weight loss study. Cardiac and vascular ultrasound studies were performed at baseline and at 3, 6, 12, and 24 months after start of intervention. Results Forty-seven subjects (78%) completed the entire 2-year follow-up. Average weight loss was 7.3 ± 4.0%, 9.2 ± 5.6%, 7.8 ± 6.6%, and 3.8 ± 7.9% at 3, 6, 12, and 24 months, respectively. Age- and sex-adjusted mixed linear models revealed that the follow-up time was significantly associated with decreases in weight (p < 0.0001), left ventricular (LV) mass (p = 0.001), and carotid intima-media thickness (p < 0.0001); there was also significant improvement in LV diastolic (p ≤ 0.0001) and systolic (p = 0.001) function. Partial weight regain diminished the maximal observed beneficial effects of weight loss, however cardiovascular parameters measured at 2 years still showed a net benefit compared with baseline. Conclusions Diet-induced moderate weight loss in obese subjects is associated with beneficial changes in cardiovascular structure and function. Subsequent weight regain is associated with partial loss of these beneficial effects. (The Safety and Effectiveness of Low and High Carbohydrate Diets; NCT00079547 )
•Characterisation of virgin olive oils produced in Extremadura.•Analysis of fatty acids, triglycerides, sterols and triterpenic dialcohols.•Relation between chemistry composition and ripeness ...maturation.
The aim of this study was the classification of olive oil according to the stage of ripeness of the olives used for its manufacture. A discriminant analysis was applied taking as dependent variables the fatty acids, triglycerides and sterols profile. The evolution of parameters of quality of the oils during ripening of the fruit has also been studied in three successive crops. The results of the classification shows that 88.5% of selected original grouped cases are correctly classified (85.7% green, 80% spotted and 78.9% ripe) and furthermore the validation classified the 69.6% of the unselected original cases. The most discriminating variables have been: avenasterol, linolenic acid, β-sitosterol and gadoleico. Fig. 1 shows graphically the values of the canonical discriminant functions and the centroids of the intervals of three or the maturity index.
PDF
