• 제목/요약/키워드: Hemophilia A

검색결과 55건 처리시간 0.019초

응고인자 투여와 surgical splint를 이용한 혈우병 환자의 발치 치험례 (TOOTH EXTRACTION USING REPLACEMENT THERAPY AND SURGICAL SPLINT IN HEMOPHILIAC PATIENT)

  • 이재문;남순현;김영진
    • 대한소아치과학회지
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    • 제23권3호
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    • pp.575-581
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    • 1996
  • Hemophilia is a hereditary condition in which one of the normal blood clotting factors is absent. This sex-linked, recessive disease, transmitted by the female and expressed in some of her male offspring, is characterized by significantly delayed coagulation times and by the tendency to hemorrhage. On the dental treatment especially, surgical procedure, appropriate methods of hemorrhage control are required. These methods are replacement therapy, antifibrinolytic agents, and local hemostatic methods. In this case, we successfully extract residual roots of hemophiliac patient without eccessive post operative bleeding using replacement therapy and surgical splint.

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자폐증상이 있는 혈우병 A 환아의 전신 마취를 통한 치과적 치험례 (DENTAL TREATMENT OF THE PATIENT WITH AUTISM AND HEMOPHILIA A UNDER GENERAL ANESTHESIA A CASE REPORT)

  • 박경순;이긍호
    • 대한소아치과학회지
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    • 제23권3호
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    • pp.593-600
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    • 1996
  • 본 증례의 혈우병 및 자폐증 환아의 치료를 통해 다음과 같은 결론을 얻었다. 1. 혈우병 환자의 내원시 내과의사나 소아과의사 및 혈액학자에게 의뢰를하여 출혈찬도 및 부족인자 보충 퉁에 관한 자문을 얻어야 한다. 2. 매우심한중증의 혈우병 환자이거나 외과적 수술등으로 인해 많은 출혈이 예상될 경우에는 포괄적인 환자 관리를위해 입원 치료를 해주어야 한다. 3. 자폐중으로인한 행동조절이 어렵거나 그외 다른 요인에 의해 행동조절이 불가능한 혈우병 환자의 경우 입원치료가 필요하다. 4. 치과 시술을 행할때에는 최소의 관혈적인 치과치료 행하도록 노력해야 하며, 응급 상황을 대비해서 국소적인 지혈방법을 습득해야 한다. 5. 치과 시술시 환자의 동통 완화 및 소염작용을 위한 약물 처치시에도 주의를 요한다. Non-steroidal anti-inflammatory drugs는 혈소판응집을 방해하기 때문에 동통완화를 위해서는 aspirin 대신에 acetaminophen을 사용하며, 소염효과를 위해서는 steroid 계통의 약물을 사용하며 ibuprofen, indomethacin, phenylbutazone 등의 약제 사용은 금한다. 6. 혈우병 환자라고해서 두려워하거나 치과 치료의 질을 변경해서는 안된다.

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NONPARAMETRIC MAXIMUM LIKELIHOOD ESTIMATION OF A CONCAVE RECEIVER OPERATING CHARACTERISTIC CURVE VIA GEOMETRIC PROGRAMMING

  • Lee, Kyeong-Eun;Lim, Johan
    • 대한수학회보
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    • 제48권3호
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    • pp.523-537
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    • 2011
  • A receiver operating characteristic (ROC) curve plots the true positive rate of a classier against its false positive rate, both of which are accuracy measures of the classier. The ROC curve has several interesting geometrical properties, including concavity which is a necessary condition for a classier to be optimal. In this paper, we study the nonparametric maximum likelihood estimator (NPMLE) of a concave ROC curve and its modification to reduce bias. We characterize the NPMLE as a solution to a geometric programming, a special type of a mathematical optimization problem. We find that the NPMLE is close to the convex hull of the empirical ROC curve and, thus, has smaller variance but positive bias at a given false positive rate. To reduce the bias, we propose a modification of the NPMLE which minimizes the $L_1$ distance from the empirical ROC curve. We numerically compare the finite sample performance of three estimators, the empirical ROC curve, the NMPLE, and the modified NPMLE. Finally, we apply the estimators to estimating the optimal ROC curve of the variance-threshold classier to segment a low depth of field image and to finding a diagnostic tool with multiple tests for detection of hemophilia A carrier.

혈우환아 어머니의 혈우병 관련 지식, 우울 및 삶의 질과의 관계연구 (A Study of Relationship Knowledge, Depression and Quality of Life of Mothers with Hemophilic Children)

  • 송영아;강현숙;김원옥
    • 여성건강간호학회지
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    • 제12권2호
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    • pp.150-155
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    • 2006
  • Purpose: This study was conducted to survey knowledge, depression, and quality of life of mothers with hemophilic children, and to provide basic data for a health promoting intervention improving their quality of life. Method: The subjects of this study were 140 mothers of hemophilic children by convenience sampling. The collected data were processed using SPSS program and analyzed using descriptive statistics, and Pearson correlation. Result: The knowledge of mothers with hemophilic children was 2.8 points out of 4 points. The depression of the subjects was 2.1 points out of 4 points. The quality of life of the subjects was 3.2 points out of 5 points. Results also showed a positive relationship between quality of life and knowledge (r=.45, p<.001) and a negatives relationship between quality of life and depression (r=-.41, p<.001). Conclusion: Knowledge and depression affect quality of life of mothers with hemophilic children. Therefore, it is necessary to develop a nursing intervention with these variables to increase the quality of life for mothers with hemophilic children.

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Factor XI deficiency and orthognathic surgery: a case report on anesthesia management

  • Lee, Soo Eon;Choi, Yoon Ji;Chi, Seong-In;Kim, Hyun-Jeong;Seo, Kwang-Suk
    • Journal of Dental Anesthesia and Pain Medicine
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    • 제15권1호
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    • pp.25-29
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    • 2015
  • Factor XI deficiency (Hemophilia C) is a very rare autosomal recessive bleeding disorder. Patients with factor XI deficiency do not typically show any spontaneous bleeding or specific symptoms. Sometimes those who have this disorder are identified during special situations such as trauma or surgery. Orthognathic surgery is particularly associated with a high bleeding risk. Therefore, great care must be taken when treating patients with bleeding disorders such as factor XI deficiency. There are a few reports that address the management of patients with bleeding disorders during orthognathic surgery. The current report describes a patient with factor XI deficiency who underwent Le Fort I osteotomy together with bilateral sagittal split osteotomy. The patient's condition was assessed using both rotation thromboelastometry ($ROTEM^{TM}$) and noninvasive measurements of total hemoglobin (SpHb) using Masimo Radical 7 (Masimo Co. CA, USA).

혈우병성 관절증 7세 환아의 한방적 처치에 대한 증례보고 (A Case Report of a 7-year-old Hemophilic Arthropathy Patient Treated by Oriental Medical Treatment)

  • 홍효신;이진용
    • 대한한방소아과학회지
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    • 제25권2호
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    • pp.27-38
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    • 2011
  • Objectives: The purpose of this study is to demonstrate the therapeutic effect of oriental medical treatment on Hemophilic arthropathy. Methods: The patient was treated by Herb medicine, Acupuncture, Moxibustion, and Cupping. As a result, improvement in the ROM of joints was shown. ROM(Range of motion), Lequesne's functional index(LFI), WOMAC index and Visual analogue scale(VAS) were used to measure the progression. Results The ROM, LFI, WOMAC index and VAS were evaluated during admission. All scales improved; ROM of knee joint : $60^{\circ}$($105^{\circ}$ to $165^{\circ}$), ROM of ankle joint : $40^{\circ}$($20^{\circ}$ to $60^{\circ}$), LFI : 15 to 4, WOMAC index : 55 to 12 and VAS : 5.0 to 1.0. We also improved the patient's symptoms with oriental medical treatment. Conclusions: According to the results, oriental medical treatment is considered to be effective on Hemophilic arthropathy and further study is needed.

항응고제 투여중인 다발성 전신질환자에서 과도한 발치창 출혈부의 진정요법과 국소마취 시행하에 창상주위 봉합과 배농술 통한 출혈과 감염조절 (Bleeding & Infection Control by the Circumferential Suture & Drainage on Active Bleeding Extraction Socket under Sedation And Local Anesthesia in a Multiple Medically Compromised Patient with Anticoagulation Drug)

  • 유재하;김종배
    • 대한치과마취과학회지
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    • 제11권2호
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    • pp.177-182
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    • 2011
  • There are five principal causes for excessive bleeding in the immediate postextraction phase ; (1) Vascular wall alteration (wound infection, scurvy, chemicals, allergy) (2) Disorders of platelet function (genetic defect, drug-aspirin, autoimmune disease) (3) Thrombocytopenic purpuras (radiation, leukemia), (4) Inherited disorders of coagulation (hemophilia, Christmas disease, vitamin deficiency, anticoagulation drug-heparin, coumarin, aspirin, plavix). If the hemorrhage from postextraction wound is unusually aggressive, and then dehydration and airway problem are occurred, the socket must be packed with gelatine sponge(Gelfoam) that was moistened with thrombin and wound closure & pressure dressing are applied. The thrombin clots fibrinogen to produce rapid hemostasis. Gelatine sponges moistened with thrombin provide effective coagulation of hemorrhage from small veins and capillaries. But, in dental alveoli, gelatine sponges may absorb oral microorganisms and cause alveolar osteitis (infection). This is a case report of bleeding and infection control by the circumferential suture and iodoform gauze drainage on infected active bleeding extraction socket under sedation and local anesthesia in a 71-years-old male patient with anticoagulation drug.

방사성 활막절제술 (Radiosynovectomy)

  • 김덕윤
    • Nuclear Medicine and Molecular Imaging
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    • 제40권2호
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    • pp.127-131
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    • 2006
  • Radiosynovectomy has been used as an effective treatment in patients with resistant synovitis after failure of long-term medication and intraarticular steroid injection. Although $^{90}Y$ silicate/citrate, $^{186}Re$ sulfide, and $^{169}Er$ citrate were approved in Europe for the appropriate radiopharmaceuticals for radiosynovectomy other radionuclides such as $^{32}P$-chromic phosphate, $^{165}Dy$-ferric hydroxide macroaggregate, $^{188}Rh$-microspheres, $^{153}Sm-particulate, and $^{166}Ho$-ferric hydroxide macroaggregate have been used in many countries. Reported success rates range from 40% to 90% for the different joints and underlying disease. In Korea, $^{188}Re$-tin-colloid and $^{166}Ho$-chitosan complex are now using as the major radionuclides in radiosynovectomy with good clinical results. A study on radiation synovectomy using $^{188}Re$-tin-colloid for patients with Korean rheumatoid arthritis shows the treatment resulted in the improvement of arthritis and well tolerated in our study, the radiosynovectomy with $^{166}Ho$-chitosan complex in 53 hemophilic patients markedly decreased intra-articular bleeding frequency and need for coagulation factor. This review inculdes general priciples in the application of radiosynovectomy and the clinical experience in Korea.

Long-Term Expression of von Willebrand Factor by a VSV-G Pseudotyped Lentivirus Enhances the Functional Activity of Secreted B-Domain-deleted Coagulation Factor VIII

  • Park, Sang Won;Choi, Sang-Yun
    • Molecules and Cells
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    • 제24권1호
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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.