Congenital haemophilia A is a chromosome-linked recessive disorder caused by the deficiency or reduction of factor VIII (FVIII) pro-coagulant activity. During treatment, some patients develop alloantibodies (FVIII inhibitors) that neutralize the action of exogenously administered FVIII. Currently, the presence of these inhibitors is the most serious adverse event found in replacement therapy. Some studies have suggested that genetic factors influence the development of the FVIII coagulation inhibitors. To identify the class I and II alleles that may be influencing the formation of inhibitors in severe haemophilic patients. Genotyping of the class I (HLA-A, -B and -C) and class II (HLA-DRB1, -DQA1 and -DQB1) alleles of 122 patients with severe haemophilia A, including 36 who had developed antibodies to factor VIII, was performed. After the comparison of the group without inhibitors and the group with inhibitors, HLA-C*16 [Odds ratio (OR) = 7.73; P = 0.0092] and HLA-DRB1*14 (OR = 4.52; P = 0.0174) were found to be positively associated with the formation of the inhibitors. These results confirm that HLA alleles are involved in inhibitor production and could be used as a tool for recognition of groups at high risk of possible inhibitor development in Southern Brazilian haemophilic patients.
A diversity of native hops can be found in Portugal, but little is known concerning their volatile and sensory profiles. Nowadays, the exponential growth of the craft beer sector and the preference for more flavoured beers promote the research of unexplored wild hops that have the advantage of being well adapted to the Portuguese edaphoclimatic conditions. Therefore, the goal of this study was to characterize the volatile profile of 75 native Portuguese hops and compare with 34 commercial varieties by means of headspace solid‐phase microextraction gas chromatography/mass spectrometry (HS‐SPME‐GC/MS), in order to select those that present similarities with commercialized hops and confirm by check‐all‐that‐apply (CATA) analysis if they present similar organoleptic characteristics. Due to the complexity of hop volatile profile and the great number of samples analysed, robust chemometric treatment of chromatographic and sensorial data was required to make reliable conclusions. Twelve Portuguese hops present a volatile profile and sensory characteristics quite similar to some commercial varieties, because 11 Portuguese hops were grouped with the European varieties Challenger, Hallertauer Magnum and Perle, both in volatile profile and sensory analysis and one clustered with American registered varieties.
Mandarina Bavaria is a "Special Flavor" hop variety, described as fruity, with pronounced mandarin and citrus, combined with traditional hoppy sensations. The relationship between fruity-citrus intensity and the volatile profile of dry-hopped beers was assessed in order to predict the sensory perception of those dry-hopped beers using the content of selected volatile compounds. For this purpose, two base beers (A and B) that presented statistical differences (p < 0.05) in the composition of volatile compounds and on the sensory perception were dry hopped with 3 g/L Mandarina Bavaria hop. Twenty-four volatiles from hop were quantified during 15 days of dry hopping, while the sensory perception was followed by a certified trained panel. The sensory perception of total hoppy content (in a scale from 0 to 5) can be estimated using a PLS equation (Q 2 = 0.654): total hoppy = 1.8 + [myrcene (μg/L) × 7.5). Successful models were also obtained to predict citrus (Q 2 = 0.745), green fruit (Q 2 = 0.598), and sweet fruit (Q 2 = 0.626) characteristics of dry-hopped beers.
This suggests that polymorphisms in INFG and in TGFB1 genes are related to risk of developing inhibitor, and could contribute to a genetic profile of the individual HA for the risk of inhibitors development to FVIII.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.