• Korean Journal of Clinical Laboratory Science
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• v.47 no.4
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• pp.237-242
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• 2015

The coagulation factor activity compared two groups of the lower 10% (29.1~30.9 sec) and the upper 10% (38.0~41.9 sec) of the normal reference range of aPTT. The aim of this study was to investigate the influence of sex, age, and ABO blood type on coagulation factor activity. There was significant difference in the activity of the coagulation factor assay based on age. The VIII (p<0.0001) and IX (p=0.0050) in the lower group of samples from those over sixty years of age is higher than from those under sixty. In contrast, XII (p=0.0285) for samples over sixty was lower than for samples under sixty. While in the upper group V (p=0.0219), VIII (p=0.0005), and IX (p=0.0014) for samples from the over sixty group was higher than those under sixty. In the case of activity of coagulation factor between O and non-O blood type, VIII (p<0.001) activity of the non-O blood type was higher than that of the O blood type in the both groups. The XII (p=0.016) activity of non-O blood type was lower than that of O blood type in the upper group. According to the multiple logistic regression analysis, when other variables are under the same conditions between lower and upper groups, there is a strong possibility for the lower group when activity of V (p=0.001), VIII (p<0.001), X (p<0.001) and XII (p<0.001) is increased. Furthermore, there is also a strong possibility of upper group when activity of II (p=0.004) and IX (p=0.012) is increased. However, no significant difference in between sex, age and XI was observed.

• Neonatal Medicine
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• v.17 no.1
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• pp.132-135
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• 2010

Although the majority of abnormal bleeding during the neonatal period results from acquired coagulation disorders, inherited coagulation disorders can also manifest at this time. Hemophilia is the most common of inherited coagulation disorder. Although 40-70% of cases with hemophilia are diagnosed in the neonatal period, few cases have been reported in premature infants. We report a case of a premature infant born at 31 weeks of gestation, diagnosed with hemophilia A by blood coagulation test, coagulation factor assay and study of the F8 gene. The baby was treated with recombinant factor VIII (Recombinate$^{(R)}$, USA) because of repeated seizures and intramuscular hematoma.

• Journal of Genetic Medicine
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• v.13 no.2
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• pp.89-94
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• 2016

Purpose: Molecular genetic analysis is the main approach used for prenatal diagnosis of hemophilia A and B. However, in certain cases, such analysis is uninformative. In such situations, direct measurement of fetal coagulation factor levels is still the best option, and it may be the only option in some cases. This study was conducted to determine the normal ranges of mid-trimester cord blood factor VIII (FVIII) and IX (FIX) in a Korean population. Materials and Methods: Twenty-six FVIII samples and 29 FIX samples were assayed in fetal cord blood acquired by ultrasound-guided cordocentesis. Sampling was conducted during gestational ages of 19-24 weeks. Results: The mean and standard deviations for FVIII and FIX activity were $45.5{\pm}30.5%$ and $19.9{\pm}12.2%$, respectively. Ranges for FVIII and FIX were 1.5-125.0% and 6.0-52.0%, respectively. Conclusion: Our study revealed the normal ranges and lowest level of factor VIII and factor IX in non-affected normal fetus by fetal cord blood sampling during the mid-trimester in a Korea population. The factor assay of the fetal cord blood is invasive but feasible and provides important basic data related to hemophilia.

• Journal of Genetic Medicine
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• v.7 no.1
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• pp.1-8
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• 2010

Hemophilia A is a sex-linked recessive coagulation disorder associated with diverse mutations of the factor VIII gene and a variety of phenotypes. The type of mutation involved dictates the activity of factor VIII, and in turn the severity of bleeding episodes and development of alloantibodies against factor VIII (inhibitors). Missense mutations are the most common genetic risk factors for hemophilia A, especially mild to moderate cases, but carry the lowest risk for inhibitor development. On the other hand, intron 22 inversion is the most common mutation associated with severe hemophilia A and is associated with high risk of inhibitor formation. Large deletions and nonsense mutations are also associated with high risk of inhibitor development. Additional mutations associated with hemophilia A include frameshift and splice site mutations. It is therefore valuable to assess the mutational backgrounds of hemophilia A patients in order to to interpret their symptoms and manage their health problems.

• Molecules and Cells
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• v.24 no.1
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• pp.125-131
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• 2007

von Willebrand factor (vWF) is a multimeric glycoprotein which functions within the coagulation system. It colocalizes with factor VIII (FVIII) by non-covalent interaction and alters its intracellular trafficking. vWF is also instrumental in maintaining the stability of secreted FVIII. The principal objective of this study was to generate a lentivirus-based vWF expression vector for gene therapy of hemophilia A. We inserted a vWF of 8.8 Kb into a lentiviral vector thereby producing VSV-G-pseudotyped vEx52. However, its titer was quite low, presumably because the length of vWF gene exceeds the size limit of the lentiviral vector. In order to overcome the low-titer, we concentrated the vEx52 and thus increased the efficiency of transduction approximately 6-fold with $1/100^{th}$ of the volume. However, as concentration requires an additional laborious step, we attempted to enhance the transduction efficiency by deleting exons 24-46 and 29-46 in pRex52 to construct pRex23 and pRex28, and in pvEx52, yielding pvEx23 and pvEx28, respectively. The transfected pRex52 had a profound effect on the activity of secreted FVIII, and this activity declined as domains of vWF were deleted. However, when the domain-deleted vWF-lentiviruses were transduced into K562 cells, the vEx28 increased the activity of the secreted FVIII compared to what was observed with vEx52. This result is probably due to higher efficiencies of transduction and expression while retaining the essential domains required for proper interaction with FVIII.

• 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.

• Journal of Dental Anesthesia and Pain Medicine
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• v.21 no.3
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• pp.261-268
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• 2021

Hemophilia A is a hemorrhagic disease caused by coagulation factor VIII deficiency. In head and neck cancer surgery, especially during a reconstructive one, complications can occur. These include hematomas due to bleeding which can then lead to flap ischemia, necrosis, and impaired wound healing. There are fewer cases of reconstructive surgery in patients with hemophilia A. Here in we report, a reconstructive surgery that involved mass resection, partial glossectomy (right), selective neck dissection (right, Levels I, II, III, IV), and reconstruction at the lateral arm free flap (left) in a 25-year-old man with hemophilia A. The surgery was successfully performed without any complications after pretreatment with Factor VIII concentrate, which has not been reported earlier.

• 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.

• Clinical and Experimental Pediatrics
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• v.56 no.7
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• pp.291-297
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• 2013

Purpose: The aim of this study was to investigate prophylactic treatment effects in Korean patients with severe hemophilia A. Methods: A prospective study of 32 severe hemophilia A patients was conducted with the approval of the Institutional Review Board at the Eulji University Hospital. Two patients received primary prophylaxis; whereas, the other 30 patients were divided into 2 groups-secondary prophylaxis (n=15) and on-demand (n=15)-on the basis of their consent for secondary prophylaxis. A 20-25 IU/kg dose of factor VIII concentrate was administered to the primary and secondary prophylaxis group patients every 3 days for 1 year. The prophylactic effect was evaluated by observing changes in the Pettersson scores, annual number of total and joint bleeds, and factor VIII consumption for 1 year. Results: No moderate or severe bleeding was observed, and the Pettersson scores remained unchanged during the prophylaxis period in the patients who received primary prophylactic treatment. After the treatment was changed from on-demand to secondary prophylaxis, the annual number of total and joint bleeds in the secondary prophylaxis group decreased by $64.4%{\pm}13.0%$ and $70.0%{\pm}15.2%$, respectively. The average increase in Pettersson scores within 1 year was $0.5{\pm}0.8$ and $1.3{\pm}1.1$ in the secondary prophylaxis and on-demand groups, respectively. Prophylactic effects were also observed in patients >17 years who had nearly the same initial Pettersson scores. Conclusion: Intermediate-dose prophylactic treatment may delay hemarthropathy progression and prevent its occurrence in Korean severe hemophilia A patients.

• Toxicological Research
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• v.33 no.3
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• pp.225-231
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• 2017

National reference standards (NRSs) for biologics are established through potency estimation by a multi-center joint study of standard materials used in the approval process for national lot release and quality control of vaccines, blood products, and other biologics. In this study, a stability evaluation was conducted to determine whether the potency of NRSs for six blood products was being maintained at a consistent level in Korea. The present study conducted real-time stability tests via in-vivo/in-vitro bioassay on NRSs for blood coagulation factor VIII concentrate (2nd standard), antithrombin concentrate, prekallikrein activator, anti-hepatitis B immunoglobulin, blood coagulation factor IX concentrate, and anti-tetanus human immunoglobulin, as well as a trend analysis using cumulative annual results. The real-time stability test results showed that the mean potency of six NRSs was all within the control limit. In the trend analysis, the potency of NRS for blood coagulation factor VIII concentrate (2nd standard) showed a decreasing trend, while the potency of all other products had been stably maintained. The present study confirmed that the mean potency of NRSs for six blood products had been stably maintained in Korea. The findings of the present study establish a foundation that can ensure the quality of NRSs for biologics in Korea, and it is expected to make a major contribution to the supply of high-quality biologics.