Molecular analysis has been performed on a Malaysian patient with a severe bleeding disorder due to factor XIII(A) subunit deficiency. Total mRNA was isolated from the patient's leucocytes and four overlapping segments corresponding to the entire coding region of the A subunit cDNA were amplified by RT-PCR. The cDNA segments amplified efficiently and were of expected size. Direct sequencing of the complete reading frame revealed a single homozygous base change (nt 1327G-T) in exon 10 corresponding to a missense mutation, Val414Phe, in the catalytic core domain of the A subunit monomer. The mutation eliminates a BsaJ1 restriction site and family screening showed that both parents were heterozygous for the defect. The base substitution was absent in 55 normal individuals. Val414 is a highly conserved residue in the calcium-dependent transglutaminase enzyme family. Computer modelling based on 3D crystallographic data predicts that the bulky aromatic side chain of the substituted phenylalanine residue distorts protein folding and destabilizes the molecule. In addition, conformation changes in the adjacent catalytic and calcium binding regions of the A subunit are likely to impair the enzymatic activity of any protein synthesized.
Matched MeSH terms: Factor XIII/genetics*; Factor XIII/chemistry; Factor XIII Deficiency/genetics*
A 26-year-old male presented with claw toes and Factor XIII deficiency. Correction for his deformity was undertaken. Pre, intra and post-operative transfusions of plasma and blood prevented any haemorrhagic complications.
Matched MeSH terms: Factor XIII Deficiency/therapy*
Coagulation factor XIII and other haemostatic markers are known strengthen fibrin clot formation and, hence, may facilitate safer surgery. Currently however, factor XIII activity levels are not routinely screen. Therefore, the purpose of this study was to determine the association of perioperative factor XIII activity levels and other haemostatic markers with postoperative intracranial haematoma formation in neurosurgical patients.
Floods are one of the most devastating types of disasters that cause loss of lives and property worldwide each year. This study aimed to evaluate and compare the prediction capability of the naïve Bayes tree (NBTree), alternating decision tree (ADTree), and random forest (RF) methods for the spatial prediction of flood occurrence in the Quannan area, China. A flood inventory map with 363 flood locations was produced and partitioned into training and validation datasets through random selection with a ratio of 70/30. The spatial flood database was constructed using thirteen flood explanatory factors. The probability certainty factor (PCF) method was used to analyze the correlation between the factors and flood occurrences. Consequently, three flood susceptibility maps were produced using the NBTree, ADTree, and RF methods. Finally, the area under the curve (AUC) and statistical measures were used to validate the flood susceptibility models. The results indicated that the RF method is an efficient and reliable model in flood susceptibility assessment, with the highest AUC values, positive predictive rate, negative predictive rate, sensitivity, specificity, and accuracy for the training (0.951, 0.892, 0.941, 0.945, 0.886, and 0.915, respectively) and validation (0.925, 0.851, 0.938, 0.945, 0.835, and 0.890, respectively) datasets.
The distribution of plasma coagulation factor XXIIB polymorphism was determined by PAG isoelectric focusing and immunoblotting in a group of 670 subjects comprising 375 Chinese, 110 Malays and 185 Indians. The frequencies of FXIIIB*1, FXIIIB*2, and FXIIIB*3 were found to be 0.27, 0.03 and 0.70 in the Chinese; 0.33, 0.05 and 0.64 in the Malays and 0.58, 0.08 and 0.33 in the Indians. The phenotypic distribution of FXIIIB alleles was at Hardy-Weinberg equilibrium in all three populations. A two-dimensional principal-components analysis on the basis of three common alleles at the FXIIIB locus among 19 populations, so far studied, clearly differentiates the Negroid, Mongoloid and Caucasoid populations into three major groups with the exception of Amerindians (Minnesota) and US Blacks showing some Caucasoid influence.
Lignocellulosic biomasses such as wheat straw are widely used as a feedstock for biogas production. However, these biomasses are mainly composed of a compact fibre structure and therefore, it is recommended to treat them prior to its usage for biogas production in order to improve their bioavailability. The aim of this work is to evaluate, in terms of performance stability, methane yield and economic feasibility, two different scenarios: a mesophilic codigestion of wheat straw and animal manure with or without a low-energy demand alkaline pre-treatment (0.08gKOHgTS-1of wheat straw, for 24h and at 25°C). Besides this, said pre-treatment was also analysed based on the improvement of the bioavailable carbohydrate content in the untreated versus the pre-treated wheat straw. The results pointed out that pre-treated wheat straw prompted a more stable performance (in terms of pH and alkalinity) and an improved methane yield (128% increment) of the mesophilic codigestion process, in comparison to the "untreated" scenario. The pre-treatment increased the content of cellulose, hemicellulose and other compounds (waxes, pectin, oil, etc.) in the liquid fraction, from 5% to 60%, from 11.5% to 39.1% TS and from 57% to 79% of the TS in the liquid fraction for the untreated and pre-treated wheat straws, respectively. Finally, the pre-treated scenario gained an energy surplus of a factor 13.5 and achieved a positive net benefit of 90.4€tVS-WS-1d-1, being a favourable case for an eventual scale-up of the combined process.