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.
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