• Title/Summary/Keyword: Factor IX

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Experience of Hemophilic Pseudotumor Developed at the Fracture Site of the Proximal Phalanx of the Hand (수부 근위지골 골절 수술 후 발생한 혈우병성 가성종양의 치험례)

  • Park, Ji Ung;Kim, Chang Yeon;Shin, Jong In;Cho, Sang Hun
    • Archives of Plastic Surgery
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    • v.34 no.4
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    • pp.516-519
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    • 2007
  • Purpose: Hemophilia is one of the most common bleeding disorder, and circulating levels of factor VIII or IX are closely related to the severity and frequency of the bleeding. The severity of hemophilia is classified to be severe, moderate, and mild when the factor level is less than 1%, between 1% and 5%, and between 5% and 25%, respectively. Hemophilic pseudotumor is a rare complication occurring in 1 - 2% of patients with factor VIII or IX deficiency. If the treatment is delayed, it would cause disabling and life threatening results. Methods: We experienced hemophilic pseudotumor developed at the fracture site of the proximal phalanx of the hand in a hemophilic B patient. Hemophilic pseudotumor was successfully treated with perioperative factor replacement and surgical intervention included excision and autologous bone graft. Results: Hemophilic pseudotumor was healed with complete regression, and no specific complication was developed. Conclusion: When we accounter hematoma like lesion after surgery unpredictably, we must consider hemophilic pseudotumor and make a accurate diagnosis with preoperative hematologic screening and various imaging study. Subsequently, adequate perioperative supplement of concentrate and surgical intervention brings to the satisfactory result without recurrence.

A Plausible Method for the Diagnosis of Genetic Disorders Using Full Length cDNA

  • Hur, Hyang-Suk;Lee, Young-Won;Park, Hyoung-Woo;Kim, Myoung-Hee
    • Biomedical Science Letters
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    • v.7 no.1
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    • pp.1-5
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    • 2001
  • A cDNA of coagulation Factor IX gene has been screened from the $\lambda$gt11 human fetal liver cDNA library, and used to construct a 2.8-kb full length cDNA after recombining with the N-terminal fragment from pTZ-FIX. Human genomic DNA was isolated, digested with the restriction endonucleases, TaqI, EcoRI, and HindIII, and Southern hybridization was performed using the full length factor IX cDNA as a probe. The hybridized bands generated by the restriction endonucleases were the followings: TaqI, 0.3, 1.0, 1.6, 1.8, 2.7, 3.7, and 5.3 kb bands; EcoRI, 1.8, 4.8, 4.9, 5.5, 6.8, and 12.6 kb bands; HindIII, 4.1, 4.4, 5.2, 5.8, 7.6, and 12.5 kb bands. When the Southern bands were physically mapped along the genome, about 50-kb continuous region harboring almost all of the genomic region of Factor Ⅸ gene was covered. These results suggest a possibility of using an exonal cDNA probe to diagnose abnormalities including large deletions, insertions, and rearrangements along the genome, if there is any.

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Polycythemia vera combined with coagulation disorder -A case report- (혈액응고 장애가 동반된 진성다혈증 1례)

  • Ahn, Jae-Hee;Lee, Du-Ha;Chun, Hyo-Jin;Hyun, Myung-Soo;Lee, Hyun-Woo;Kim, Chung-Sook
    • Journal of Yeungnam Medical Science
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    • v.6 no.2
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    • pp.247-255
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    • 1989
  • We report a case of polycythemia vera combined with coagulation disorder. The patient was 54 years old man who complained of continuous bleeding after incision of skin abscess 20days ago. Laboratory tests were revealed prolonged aPTT and slightly prolonged PT. Coagulation factor, I, VIII, IX, XI and fibrinogen decreased, however FDP did not increased. It appears that patient with polycythemia vera have chronic activation of coagulation system, probably initiated by activation of factor XII. Platelet aggregation test to ADP, collagen, epinephrine was also revealed poor response.

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Inhibitory Mechanism of Blood Coagulation by the Anticoagulant Polysaccharide from Coriolus versicolor (구름버섯 기원 항응고성 다당류의 혈액응고 저해기작)

  • Lee, Hyun-Sun;Kweon, Mee-Hyang;Lim, Wang-Jin;Sung, Ha-Chin;Yang, Han-Chul
    • Korean Journal of Food Science and Technology
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    • v.29 no.4
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    • pp.817-822
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    • 1997
  • Inhibitory mechanism of the anticoagulant polysaccharide purified from the fruit body of Coriolus versicolor was investigated in this paper. The anticoagulant polysaccharide (CV-40-Va-1) was proposed to have functions of the inhibition of intrinsic pathway in the blood coagulation pathway together with the interuption of a human platelet aggregation induced by von Willebrand factor (vWF). CV-40-Va-I inhibited other factors of the coagulation cascade such as factor VIII, IX, and as well as thrombin. Especially, CV-40-Va-I inhibited the fibrin formation mediated by thromin, however the polysaccharide did not affect the fibrin formation directly but affected the anticoagulant activity through the activation of antithrombin III. The sulfation of the anticoagulant polysaccharide increased the anticoagulant activity, showing that the sulfate concentration of anticoagulant polysaccharide was important factor in the blood coagulation cascade. Low molecular weight subfraction (MW 1,000) obtained by partial hydrolysis of the CV-40-Va-1 generated potent antiplatelet activity, but showed decreased anticoagulant activity.

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Evaluation of Trapezoidal PWM Inverter using a new Harmonic Torque Factor (새로운 고조파 토오크 평가함수에 의한 Trapezoidal PWM 인버터의 평가)

  • Lee, Chi-Hwan;Koo, Bon-Ho;Kwon, Wu-Hyen
    • Proceedings of the KIEE Conference
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    • 1987.07a
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    • pp.754-756
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    • 1987
  • In this paper, a new Harmonic Torque Factor(HTF) that represents torque ripple of induction motor is proposed. Trapezoidal PWM is analyzed to torque ripple by HTF and than we obtain optimum triangular factor at some carrier ratio. Six-step, sinusoidal PWM and Trapezoidal PWM are compared and evaluated for choosing a good control strategy of PWM inverter. It turns out that six-step inverter is better than sinusoidal PWM and Trapezoidal PWM when their carrier ratio is 9, and Trapezoidal PWM is superior to sinusoidal PWM. At induction motor is driven by PWM inverter, torque ripples are investigated by computer simulation using d-q two axis model. It is proved that a new Harmonic Torque Factor ix valid.

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Control of Platelet Rolling and Adhesion

  • Moskowitz, Samuel E.
    • 제어로봇시스템학회:학술대회논문집
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    • 2002.10a
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    • pp.77.1-77
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    • 2002
  • Platelets arrest bleeding and repair damaged blood vessels. The purpose of this paper is to formulate a mathematical model for the control of platelet adhesion within the vasculature consistent with experimental findings, particularly those of Frenette, Ruggeri , Savage, Yuan, Lawrence and Springer. In addition to providing some, albeit rudimentary, insight into the behavior of platelets, a numerical simulation of this theoretical model may be useful in a systematic study of pathological cases. Glycoprotein receptor complex (GPIb/V/IX), found on the platelet surface membrane, binds to the adhesive protein and ligand von Willebrand factor (vWf), located within the sub-endothelium. The binding...

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Branched N-glycans and their implications for cell adhesion, signaling and clinical applications for cancer biomarkers and in therapeutics

  • Taniguchi, Naoyuki;Korekane, Hiroaki
    • BMB Reports
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    • v.44 no.12
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    • pp.772-781
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    • 2011
  • Branched N-glycans are produced by a series of glycosyltransferases including N-acetylglucosaminyltransferases and fucosyltransferases and their corresponding genes. Glycans on specific glycoproteins, which are attached via the action of glycosyltransferases, play key roles in cell adhesion and signaling. Examples of this are adhesion molecules or signaling molecules such as integrin and E-cadherin, as well as membrane receptors such as the EGF and TGF-${\beta}$ receptors. These molecules also play pivotal roles in the underlying mechanism of a variety of disease such as cancer metastasis, diabetes, and chronic obstructive pulmonary disease (COPD). Alterations in the structures of branched N-glycans are also hall marks and are useful for cancer biomarkers and therapeutics against cancer. This mini-review describes some of our recent studies on a functional glycomics approach to the study of branched N-glycans produced by N-acetylglucosaminyltransferases III, IV, V and IX (Vb) (GnT-III, GnT-IV, V and IX (Vb)) and fucosyltransferase 8 (Fut8) and their pathophysiological significance, with emphasis on the importance of a systems glycobiology approach as a future perspective for glycobiology.