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1. 稀な遺伝性凝固異常rare inherited coagulation disorders [show details]
…Factor XIII deficiency (F13D; OMIM 613225 or OMIM 613235) – The factor XIII enzyme is a heterotetramer consisting of two catalytic A subunits and two B subunits . Most reported cases of factor XIII deficiency …
2. 出血症状を有する小児に対するアプローチapproach to the child with bleeding symptoms [show details]
…of factor XIII . However, the clot solubility assay is sensitive only at very low levels (factor XIII 1 to 3 percent) and may miss the diagnosis in less severe cases. Therefore, if factor XIII deficiency …
3. 血漿分画製剤および組換えDNA産生凝固因子plasma derivatives and recombinant dna produced coagulation factors [show details]
… Factor XIII or factor X products can be used to treat bleeding or prevent surgical bleeding in individuals with inherited deficiencies of factor XIII or factor X, respectively. Factor XIII (and occasionally …
4. 後天性の凝固因子阻害物質acquired inhibitors of coagulation [show details]
…factor XIII stabilizes and crosslinks overlapping fibrin strands. Factor XIII inhibitors, which are exceedingly rare, can act by one of several mechanisms: inhibition of activation of factor XIII; interference …
5. 心因性紫斑病（ガードナーダイヤモンド症候群）psychogenic purpura gardner diamond syndrome [show details]
…mild VWD may be difficult to make, and may require serial testing. Congenital (or acquired) factor XIII deficiency is associated with normal PT and aPTT. The most commonly reported clinical symptoms include…

English Journal

Development and Validation of a New Risk Prediction Score for Life-Threatening Ventricular Tachyarrhythmias in Laminopathies.

Wahbi K, Ben Yaou R, Gandjbakhch E, Anselme F, Gossios T, Lakdawala NK, Stalens C, Sacher F, Babuty D, Trochu JN, Moubarak G, Savvatis K, Porcher R, Laforêt P, Fayssoil A, Marijon E, Stojkovic T, Béhin A, Leonard-Louis S, Sole G, Labombarda F, Richard P, Metay C, Quijano-Roy S, Dabaj I, Klug D, Vantyghem MC, Chevalier P, Ambrosi P, Salort E, Sadoul N, Waintraub X, Chikhaoui K, Mabo P, Combes N, Maury P, Sellal JM, Tedrow UB, Kalman JM, Vohra J, Androulakis AFA, Zeppenfeld K, Thompson T, Barnerias C, Bécane HM, Bieth E, Boccara F, Bonnet D, Bouhour F, Boulé S, Brehin AC, Chapon F, Cintas P, Cuisset JM, Davy JM, De Sandre-Giovannoli A, Demurger F, Desguerre I, Dieterich K, Durigneux J, Echaniz-Laguna A, Eschalier R, Ferreiro A, Ferrer X, Francannet C, Fradin M, Gaborit B, Gay A, Hagège A, Isapof A, Jeru I, Morales RJ, Lagrue E, Lamblin N, Lascols O, Laugel V, Lazarus A, Leturcq F, Levy N, Magot A, Manel V, Martins R, Mayer M, Mercier S, Meune C, Michaud M, Minot-Myhié MC, Muchir A, Nadaj-Pakleza A, Péréon Y, Petiot P, Petit F, Praline J, Rollin A, Sabouraud P, Sarret C, Schaeffer S, Taithe F, Tard C, Tiffreau V, Toutain A, Vatier C, Walther-Louvier U, Eymard B, Charron P, Vigouroux C, Bonne G, Kumar S, Elliott P, Duboc D.
Circulation. 2019 Jun;().
An accurate estimation of the risk of life-threatening (LT) ventricular tachyarrhythmia (VTA) in patients with LMNA mutations is crucial to select candidates for implantable cardioverter defibrillator (ICD) implantation. We included 839 adult patients with LMNA mutations, including 660 from a French
PMID 31155932

Factor XIII Prevents Pulmonary Emboli in Mice by Stabilizing Deep Vein Thrombi.

Shaya SA, Gani DM, Weitz JI, Kim PY, Gross PL.
Thrombosis and haemostasis. 2019 Jun;119(6)992-999.
Deep vein thrombosis (DVT) can lead to pulmonary embolism (PE), but the mechanisms responsible for this progression are unknown. Previously, we showed that inhibition of thrombin-mediated activation of factor (F) XIII promotes venous thrombus stability in a murine model. In this study, we inve
PMID 31005064

Convergence of Bigelow and Arrhenius models over a wide range of heating temperatures.

André S, Leguerinel I, Palop A, Desriac N, Planchon S, Mafart P.
International journal of food microbiology. 2019 Feb;291()173-180.
The heat resistance of the bacterial spores of Moorella thermoacetica, Clostridium sporogenes, Geobacillus stearothermophilus and Bacillus coagulans was determined over a wide range of temperatures using the capillary method and thermoresistometer Mastia. The results showed that the two experimental
PMID 30508773

Japanese Journal

書評 Christopher W. Miller, Planning and Profits : British Naval Armaments Manufacture and the Military-Industrial Complex, 1918-1941 (Liverpool, Liverpool University Press, 2018), ⅹⅲ+246pp.