WordNet

be a contributing factor; "make things factor into a companys profitability"
any of the numbers (or symbols) that form a product when multiplied together
an independent variable in statistics
anything that contributes causally to a result; "a number of factors determined the outcome"
consider as relevant when making a decision; "You must factor in the recent developments" (同)factor in, factor out
resolve into factors; "a quantum computer can factor the number 15" (同)factor in, factor out
an event known to have happened or something known to have existed; "your fears have no basis in fact"; "how much of the story is fact and how much fiction is hard to tell"
a concept whose truth can be proved; "scientific hypotheses are not facts"
a piece of information about circumstances that exist or events that have occurred; "first you must collect all the facts of the case"
a statement or assertion of verified information about something that is the case or has happened; "he supported his argument with an impressive array of facts"

PrepTutorEJDIC

(…の)『要因』,(…を生み出す)要素《+『in』+『名』(do『ing』)》 / 囲数,約数 / 代理人,《おもに英》仲買人 / =factorize
〈C〉『事実』,実際にある(あった)事 / 〈U〉真相,真実(truth) / 《the~》(法律用語で)犯行
〈U〉〈C〉(…の)(量・額などの)不足,欠乏《+『of』(『in』)+『名』》 / 〈C〉不足分,不足量,不足額 / 〈C〉(精神・肉体などの)欠陥

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/11/06 11:52:23」(JST)

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Factor XIII deficiency may occur very rarely, and can cause a severe bleeding tendency. Incidence is 1 in a million to 1 in 5 million people. Most are due to mutations in the A subunit. Administration of recombinant A subunit improves clot stability and is becoming a therapeutic option for patients with this condition.^[1]^[2] This deficiency leads to defective cross-linking of fibrin and vulnerability to late re-bleeds when the primary hemostatic plug is overwhelmed. Bleeding tendencies similar to hemophiliacs develop such as hemarthroses and deep tissue bleeding.

Therapy options

Fresh frozen plasma and cryoprecipitate

Fresh frozen plasma and cryoprecipitate are the mainstay of therapy for Factor XIII deficiency, but carries risk related to transfusion.

Factor XIII concentrates

Two commercially-produced factor XIII concentrates are currently available in Europe, one is manufactured by Bio Products Laboratory (BPL) and is only available in the United Kingdom, and the other called Fibrogammin-P and is produced by Beringwerke of Germany. FXIII concentrate is only available under IND or through clinical trial in the United States.^[3]

Recombinant Factor XIII

Recombinant Factor XIII (rFXIII) is the only prospective drug alternative to receiving blood transfusions, the traditional treatment for Factor XIII deficiency. Novo Nordisk’s rFXIII is not currently a marketed drug but just recently finished phase III clinical trials in Spring 2010.^[2] Although it is a recombinant protein, rFXIII subunit A is identical in structure and function to the A subunit of factor XIII naturally produced in the body by healthy individuals.^[4] These patients need exogenous subunit A of factor XIII since they have a mutation which prevents production of the A subunit. However, since the B-subunit is located on a separate chromosome, factor XIII deficient patients actually produce the B-subunit normally. When these two subunits interact in the plasma, the enzyme is activated and can act within the clotting cascade.^[1] rFXIII acts by inhibiting fibrinolysis factors which enzymatically cleave the fibrin matrix, leading to the ultimate formation of clots.

rFXIII is synthetically bio-engineered through a yeast expression system and administered intravenously. In clinical trials, the drug was administered once every four weeks or administered on-demand in order to treat bleeding episodes.^[5] The introduction of rFXIII as a treatment for factor XIII deficiency eliminates the risk of pathogenic infection present in plasma-based treatments. rFXIII treatment would also not be dependent on blood donations consequently increasing availability and product quality. One of the biggest fears in developing rFXIII was that the body would mount an immune-response to the protein; however, several safety and pharmokinetics studies have reported no immunogenic response to rFXIII or associated yeast products.^[1]

References

^ ^a ^b ^c Lovejoy A, Reynolds T, Visich J, Butine M, Young G, Belvedere M, Blain R, Pederson S, Ishak L, Nugent D (2006). "Safety and pharmacokinetics of recombinant factor XIII-A2 administration in patients with congenital factor XIII deficiency.". Blood 108 (1): 57–62. doi:10.1182/blood-2005-02-0788. PMID 16556896.
^ ^a ^b "Recombinant Factor XIII.". 2010. http://clinicaltrials.gov/ct2/results?intr=%22Recombinant+factor+XIII%22.
^ http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=71&contentid=58
^ Muszbek, Laszlo et al. (1999). "Blood coagulation factor XIII: structure and function.". Thrombosis Research 94 94 (5): 271–305. doi:10.1016/S0049-3848(99)00023-7.
^ Clinical Trials at Novo Nordisk. (2010). "Evaluation of Recombinant Factor XIII for Prevention of Bleeding in Patients with FXIII Inherited Deficiency.". http://novonordisk-trials.com/website/search/trial-registry-details.aspx?id=1678&p=1&search=t.

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1. 稀な（劣性遺伝性）凝固異常 rare recessively inherited coagulation disorders
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
3. 出血性素因を有する成人患者へのアプローチ approach to the adult patient with a bleeding diathesis
4. 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
5. 凝固検査の臨床使用 clinical use of coagulation tests

English Journal

Perioperative course of FXIII in children undergoing major surgery.

Haas T, Korte W, Spielmann N, Mauch J, Madjdpour C, Schmugge M, Weiss M.SourceDepartment of Anaesthesia, University Children's Hospital Zurich, Zurich, Switzerland Institute for Clinical Chemistry and Haematology, Kantonsspital, St Gallen, Switzerland Department of Haematology, University Children's Hospital Zurich, Zurich, Switzerland.
Paediatric anaesthesia.Paediatr Anaesth.2012 Jul;22(7):641-6. doi: 10.1111/j.1460-9592.2011.03709.x. Epub 2011 Sep 20.
Background: Acquired deficiency of FXIII because of perioperative hemodilution has been described several times in adults; however, data in children are scarce. We performed a prospective observational trial to evaluate the intraoperative course of FXIII in children undergoing elective major surg
PMID 21933302

[Bleeding and coagulation disorders in tonsillectomies].

Papaspyrou K, von Creytz H, Kolonko K, Mewes T, Mann W, Scharrer I.SourceKlinik für Hals-Nasen-Ohren-Heilkunde und Kopf-Hals-Chirurgie, Universitätsmedizin Mainz, Langenbeckstr. 1, 55130, Mainz, Deutschland, konstantinos.papaspyrou@unimedizin-mainz.de.
HNO.HNO.2012 Jun;60(6):511-9.
BACKGROUND: The aim of our prospective analysis was to show the incidence of bleeding disorders among a tonsillectomy patient population and in case of bleeding disorders.PATIENTS AND METHODS: This study comprised 92 consecutive patients who underwent tonsillectomies from 1 January 2007 to 31 Decem
PMID 22398952

Japanese Journal

ITPに合併した抗VWF抗体によるvon Willebrand症候群

井原章裕,鈴木伸明,松下正 [他],一瀬白帝
臨床血液 56(7), 901-904, 2015
… 第VIII因子活性22%, VWF: RCo<6%, VWF: Ag276%, 第XIII因子活性42%, VWF large multimer (±)。 … 本症例は，原因不明の第XIII因子欠乏症も合併した稀な例である。 …
NAID 130005093126

後天性血友病XIIIを伴う末期腎不全患者に対し透析療法を導入した経験

神田壮平,齋藤満,福田歴視 [他],山本竜平,小泉淳,五十嵐龍馬,千葉修治,沼倉一幸,秋濱晋,井上高光,成田伸太郎,土谷順彦,佐藤滋,羽渕友則
日本透析医学会雑誌 48(1), 57-60, 2015
… 凝固第XIII因子 (F13) 異常を疑いF13活性を測定. … 17% (基準値: 70-140%) と著明な低下を認め, 後天性血友病XIIIと診断した. …
NAID 130004880122

遺伝子組換えトロンボモジュリン製剤が奏効した維持透析中の解離性大動脈瘤に合併した慢性播種性血管内凝固

早川佳奈,田村志宣,義間大也,早川隆洋,栗原稔男,大浦真紀,中野好夫,惣宇利正善,一瀬白帝,藤本特三
臨床血液 55(11), 2300-2305, 2014
… さらに，第XIII因子活性の低下も認めたが，そのインヒビターは検出されなかった。 … 以上より，解離性大動脈瘤に合併した慢性播種性血管内凝固ならびに二次性出血性第XIII因子欠乏症と診断した。 …
NAID 130004705979