• 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.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.4 no.2
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• pp.73-76
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• 2008

Hemophilia A is an X-linked disorder resulting from a deficiency in factor VIII. Hemophilia A is classified into severe, moderate, and mild forms. A 12-month old boy visited for uncontrolled bleeding on the upper labial frenum area. Spontaneous bleeding occurred about 13 days ago. We sutured the wound. However, the patient returned after 6 days with large hematoma formation. Consultation to the hematologist and laboratory examination were carried out. Activated PTT was elongated and factor VIII was only 6%. He was diagnosed as mild hemophilia A. Fresh frozen plasma (FFP) was provided and hemostasis was achieved. At 5-day check-up, no more bleeding was observed. For the management of prolonged bleeding in hemophilia A patient, not only local hemostatic measures, but factor VIII replacement therapy, antifibrinolytics, and Desmopressin are also available. Mild hemophilia A often occurs without either familial or medical history. It is often first detected by dental trauma.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1972.03a
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• pp.17.1-17
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• 1972

Hemophilia B is hereditary disorder of blood coagulation known as a deficiency of P.T.C. or Christmas factor. Anyway it's indistinguishable clinically with hemophia A and C. Here's one of most interesting problems gave us because we could only find it out as a hemophilia B. when we stood a situation to be met continuous bleeding from the operative wound of tonsillectomy and Adenoidectomy. Wintrobe proposed that easy bruising, difficulty in stopping the bleeding from razor cuts, and prolonged bleeding after tonsillectomy or tooth extraction usually are the major manifestations. For a long time little attention was paid to reports of a milder form of the diseases. In such cases coagulation time, and even prothrombin consumption were normal and the symptoms were correspondinly mild, small wounds as a rule giving little trouble and hemoarthrosis being uncommon. In our cases, there's no specific contributory history except nasal bleeding intermittently and also no specific contributory laboratory data before the operation. After the T. & A., there's continuous bleeding from nasopharynx and the coagulas on the both tonsilar fossas being grown day by day. Therefore we suspected some abnormality of coagulation mechanism and then we could get conclusion of hemophilia B. after various laboratory tests including T.G.T., As I know, this case, hemophilia B. is 3rd one as a report in Korea. We have no data of hemophilia family at present time, so that I propose that we have to make system of hemophilia family in Korea. In a next, we have to make routinely complete laboratory test, ego routine C.B.C., bleeding time, coagulation time, prothrombine time, partial thromboplastine time, before operation of T.& A. If we can solve these problems we can get the T. & A. without any problems of bleeding according to get the operation of T. & A.

• 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.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.4 no.1
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• pp.7-11
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• 2008

Hemophilia is a group of gender-linkage inherited bleeding disorders that impair the body's ability to control blood clotting or coagulation. This sex-linked disorder is transmitted on the X chromosome. These genetic disorders have lower blood plasma clotting factor level of coagulation factor. Most common form is Hemophilia A and B. Restorative dental care and simple surgery for the hemophiliac patient are quite often neglected for fear of bleeding during procedures. Even dental specialist avoid these patients and make them severe problem patients. On the dental treatment especially, invasive procedure, special considerations of bleeding control are required. Inter-consultation with the hematologist will provide orientation on the best approach to dental treatment, such as the need of replacement therapy, the modification of antifibrinolytic therapy, the application of local hemostatic methods. In this case reports, we successfully treat early childhood caries of patient with hemophilia B under the general anesthesia.

• Journal of Acupuncture Research
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• v.20 no.4
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• pp.237-244
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• 2003

Objective : Hemophilia, a genetically determined disorder, is characterized by abnormality of the coagulation mechanism due to functional deficiency of a specific factor, namely VIII or IX. In this study, the effect of Dong-Si acupuncture therapy on the cerebral hemorrhage patient with hemophilia was evaluated. Methods : We treated him with acupuncture by stimulating Dong-Si acupuncture point(Yuk Wan) and assessed the effect by using manual dynamic evaluation of MRC(Medical Research Council) and Modified Barthel Index. Results : After treatment, the bleeding tendency was grossly reduced and bleeding lesion was absorbed gradually. And manual dynamic evaluation of the upper extremity increased from 4 grade to 5 grade. Conclusions : This result suggest that Dong-Si acupuncture therapy on the cerebral hemorrhage patient with hemophilia was effective. And futher studies are required to concretely prove the effectiveness of Dong-Si acupuncture therapy for treating hemorrhagic diseases.

• Clinical and Experimental Pediatrics
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• v.51 no.12
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• pp.1359-1362
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• 2008

The most effective treatment strategy for patients with hemophilia is replacement therapy with FVIII or FIX concentrates, which usually requires long-term, uncomplicated venous access. However, central venous access device (CVADs, ports) insertion requires inpatient admission and general anesthesia, and presents some problems regarding health insurance coverage. Peripherally inserted central catheters (PICCs) were inserted in two severe hemophilia patients aged 7 and 11 years with high titers of inhibitors. They experienced frequent bleeding episodes and required replacement therapy, which eventually resulted in difficulty in acquiring venous line access. Factor VIII activity was below 1%, and inhibitor titers were 160 and 26.3 BU/ml. In an outpatient setting, PICC lines are easily placed by radiological guidance and require local anesthesia alone. PICC has been feasible, in particular, for hemophilia patients with frequent bleeding episodes.

• Journal of Korean Biological Nursing Science
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• v.26 no.1
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• pp.1-15
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• 2024

Purpose: This study aimed to evaluate the effectiveness of exercise in patients with hemophilia. Methods: We carried out a systematic review and meta-analysis in accordance with the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) guidelines. A literature search was conducted of published randomized controlled trials for exercise interventions from January 1, 2014 to March 15, 2023. To estimate the size of the effects of exercise, a meta-analysis was performed using the R package "meta." Results: Five databases were searched to obtain articles published in Korean or English. Of 1,150 articles reviewed, 13 were included in the systematic review and 9 in the meta-analysis. The risk of bias was assessed using RoB 2.0. The overall effect sizes of exercise interventions, calculated as the standardized mean difference, were -0.11 (95% confidence interval [CI] = -1.41 to -1.20) for pain, -2.13 (95% CI = -3.33 to -0.93) for joint health, 9.96 (95% CI = 7.51 to 12.28) for physical activity, and 0.59 (95% CI = -0.39 to -1.56) for quality of life. Conclusion: These findings suggest that exercise is useful for improving the joint health and physical activity of patients with hemophilia. Thus, it is necessary to develop and apply exercise interventions for patients with hemophilia to reduce their pain and improve their quality of life.

• Korean Journal of Clinical Laboratory Science
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• v.54 no.2
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• pp.163-166
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• 2022

Acquired hemophilia A (AHA) is an uncommon autoimmune bleeding disorder in which autoantibodies that affect the functions of factor VIII (FVIII) are present in the blood. The initial diagnosis of AHA is difficult as the presentations of AHA differ from those of congenital hemophilia A. Moreover, the treatment of AHA is more complex due to the presence of autoantibodies against FVIII. Here, we present a case report of postpartum AHA, to increase the perception and knowledge regarding the recognition and management of such cases. We present a young female with the chief complaint of vaginal bleeding and upper arm ecchymosis. Laboratory results exhibited isolated prolonged activated partial thromboplastin time (APTT) and FVIII inhibitors. The patient was treated with corticosteroids, FVIII concentrates, and a bypassing agent. In conclusion, unexplained postpartum bleeding, unmanageable with basic hemostatic measures, should lead to clinical suspicion of an acquired bleeding disease.

• Korean Journal of Veterinary Service
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• v.34 no.2
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• pp.191-193
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• 2011

A 3-month-old intact male, Labrador retriever was presented with the history of coagulopathy and anemia. The results of initial screening tests of the hemostatic system yielded a tentative diagnosis of hemophilia. Activated partial thromboplastin time (APTT) was distinctly prolonged (106 seconds) and prothrombin time (PT) was not detected due to markedly prolonged test time. Whole blood transfusions (20 me l/kg body weight) were carried out prior to assays of coagulation factor. After transfusion, the patient recovered well and hemorrhage ceased. Blood samples were assessed for coagulation factor activity. The patient showed markedly low factor IX coagulation activity (5%, reference range: 7~140%) and was diagnosed with hemophilia B. After recovery, the patient was discharged from the hospital. However, 4 months later the patient was re-hospitalized for recurrence of the initial symptoms. The owner did not want to pursue further treatment and the patient died of respiratory distress two days later.