• Clinical and Experimental Pediatrics
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• 제56권7호
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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.

• 대한기관식도과학회:학술대회논문집
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• 대한기관식도과학회 1972년도 춘계종합 학술대회 초록집
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• pp.17.1-17
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• 1972

편도적출은 우리 이비인후과 임상에서 평소에 다반사로 행해지는 수술의 하나이라 하겠다. 수술술기의 개량, 마취의 발달과 더불어 각종 항생제의 발견등은 편도 적출술에 따르는 여러 가지 합병증을 감소시켰지만, 이중 수술중 또는 수술후에 왕왕 당하는 출혈문제는 가장 흔한 합병증의 하나이며 술자에게는 골치거리의 하나이기도 하여 아직껏 국내외에서 그 보고가 끊어지지 않고 있다. 특히 본증례에서와 같이 본태성 질환으로 인한 출혈의 해결에 있어서는 더욱 복잡곤란한 문제가 많을 것으로 생각된다. 혈우병은 1950년대에 이르러 각 Factor가 발견되어 현재는 혈우병 A.B.C.로 나뉘고 있다. 본증례는 11세의 남아로서 편도 척출술전에는 혈우병의 유무가 인지 못되었던 예로서 수술후 수술창에서 계속되는 출혈로 혈액응고장애를 가진 질환이라고 생각되어 여러 가지 검사결과 P.T.C의 결여로 오는 혈우병 B란 진단을 얻었기에 교훈적이고 귀중한 체험을 얻었다고 생각되어 보고하는 바이다. 환자는 수차의 수혈과 비인강탐폰 등의 방법으로 겨우 지혈되어 수술 20일만에 수술창이 치유되어 퇴원하였다.

• 대한장애인치과학회지
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• 제4권2호
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• pp.73-76
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• 2008

만 1세의 남아가 입에서 피가 계속 난다는 주소로 본과에 내원하였으며 지혈을 위해 봉합술을 시행하였다. 수일 후 다시 출혈 양상이 관찰되어 혈액종양과 협진 의뢰 및 임상 병리검사를 시행하여 경증의 혈우병 A 로 진단하였고 FFP를 투여하여 최종 지혈을 얻었다. 경증의 혈우병은 치과 외상을 통해 처음 발견되는 경우가 종종 있기 때문에 출혈이 지속되거나 수일 후 재발되는 경우에는 혈액 관련 전문가에게 의뢰하고 병리 검사를 시행하는 것이 바람직하다.

• Journal of Genetic Medicine
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• 제13권2호
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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 Korean Biological Nursing Science
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• 제26권1호
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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.

• 대한임상검사과학회지
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• 제54권2호
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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.

• 대한장애인치과학회지
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• 제4권1호
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• pp.7-11
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• 2008

치과의사는 혈우병 환자의 치료 시 주의사항에 대해 잘 알고 있어야 한다. 혈우병 환자의 내원 시 소아과 의사 및 혈우재단 등 전문가에게 의뢰하여 환자의 상태에 대한 자문을 얻어야 하고, 치과 치료 시 적절한 계획 하에 최소의 침습적인 치료가 행해지도록 노력해야 하며, 응급 상황을 대비하여 지혈방법을 습득해야 한다. 또 환자와 보호자에게 평소 구강 관리의 중요성을 일깨워 침습적 치과 치료의 빈도를 줄일 수 있도록 해야 한다.

• Journal of Acupuncture Research
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• 제20권4호
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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.

• 한국동물위생학회지
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• 제34권2호
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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.

• Clinical and Experimental Pediatrics
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• 제53권3호
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• pp.397-407
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• 2010

목 적 : F9 유전자는 혈우병B의 병인 유전자이다. 기존의 RFLP를 이용한 연관분석은 정보제공율이 55.6%에 불과하였다. 직접 염기서열 분석법은 98%의 돌연변이를 진단할 수 있지만, 고가의 비용이 든다. 본 연구는 F9 유전자를 대상으로 돌연변이의 선별검사로써 mPCR-CSGE를 사용하고, 이후 특정 유전자 부위만을 염기서열 분석하여 mPCR-CSGE의 유용성을 확인하기 위해 고안되었다. 방 법 : 연구대상은 비혈연 관계인 27명의 혈우병B 환자였다. 직접염기서열 분석법은 독립된 다른 기관에서 시행하였고, mPCR-CSGE 선별 후 염기서열 분석법은 본 연구자의 기관에서 시행되었다. 직접 염기서열 분석법의 결과가 참고치가 되어 mPCR-CSGE 선별 후 염기서열 분석법을 정확성, 경제성, 신속성, 편이성 측면에서 비교하였다. 두가지 방법으로 진단이 되지 않는 환자에게는 MLPA를 이용하여 돌연변이를 발견하였다. 결 과 : 직접 염기서열 분석법으로 26명(96.3%)의 환자에서 돌연변이를 확인할 수 있었다. mPCR-CSGE 선별 후 염기서열 분석법으로는 23명(85.2%)의 환자에서 돌연변이를 찾아낼 수 있었다. 1명의 환자는 MLPA로써 돌연변이를 확인할 수 있었다. 27명의 환자에게 21개의 독립적인 돌연변이가 있었다. mPCR-CSGE 선별 후 염기서열 분석법은 직접 염기서열 분석법에 비해 비용은 55.7%로 줄일 수 있었으나, 실험 단계는 더욱 복잡하였고, 시간도 하루가 더 걸렸으며, 세심한 실험상의 주의가 필요하였다. 결 론 : mPCR-CSGE 선별 후 염기서열 분석법은 85.2%의 높은 돌연변이 선별력을 보이고, 직접 염기서열 분석법의 57.7%의 비용만 소모하였으나, 실험과정에 세한 주의가 요구되었으며, 노동집약적이고, 실험 시간도 하루가 더 소요되었다.