• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.2
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• pp.102-105
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• 2018

Hemophilia is an X-linked herediatry disorder. Hemophilia A is a defiency of factor VIII and hemophilia B (Christmas disease) is a deficiency of factor IX. The most widely used classification is based on plasma procoagulant levels, with people <1% factor defined as severe; 1 - 5% moderately severe; and >5% as mild. A 7 years old boy visited our clinic with chief complaint of impacted supernumerary tooth. This boy had severe hemophilia A. With the previous $ADVATE^{(R)}$ injection and factor VIII replacement, Extraction of supernumerary tooth was successfully done under General anesthesia. A 9 years old boy referred from local clinic for dental caries treatment. This boy had severe hemophilia B. With the preparation of $BeneFIX^{(R)}$ injection, dental treatment was done successfully under general anesthesia. It is often first detected by dental trauma. Since bleeding after dental treatment may cause severe or even fatal complications, people with hemophilia must be given special dental care. The treatment of the patients with either hemophilia A or hemophilia B involves the intravenous infusion to either control or prevent bleeding.

• Archives of Plastic Surgery
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• v.39 no.2
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• pp.150-153
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• 2012

Hemophilia B is a rare blood coagulation disorder. Complications such as bleeding and hematoma can cause necrosis of flaps, wound disruption, and the disturbance of wound healing. In particular, guidelines for flap operations in hemophilia B patients have still not been defined, and case reports are rare. We reconstructed the heel of a 41-year-old male hemophilia B patient using a reverse sural artery flap operation. The patient presented with mild hemophilia, having 27% of the normal value of coagulation factor IX. Coagulation and the changing value of the coagulation factor were regularly measured, and 70% of the normal value of coagulation factor IX was maintained through the injection of recombinant coagulation factors and antihemorrhagics. Hematoma developed twice (postoperative day [POD] 5 and POD 7) and in each case the hematoma was removed. Injections of recombinant coagulation factors and antihemorrhagics were continuously administered until postoperative week 2. When the coagulation factors were within normal ranges. In this article, a hemophilia B patient underwent reverse sural artery flap surgery and the healing progress was analyzed. We conclude that higher than baseline levels of coagulation factors are needed for successful healing in reverse sural artery flap surgery.

• Clinical and Experimental Pediatrics
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• v.49 no.8
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• pp.821-829
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• 2006

Hemophilia is the most common coagulation disorder. It has a long history. Hemophilia A is caused by FVIII gene mutation, and hemophilia B by FIX gene mutation. Those genes are located on X chromosome long arm. Bleedings in hemophiliacs predominantly occur in joints and muscles. Because those site are insufficient in tissue factor to induce hemostasis. Among joints knee, ankle and elbow are most frequently affected because their synovial structure is vulnerable to injury compared to other joints. Hemophilia is diagnosed with factor assay. Severe hemophilia is below 1% of FVIII : C, moderate between 1% and 5%, mild over 5%. Carrier detection and prenatal diagnosis have been conducted with RFLP-based linkage analysis and DNA sequencing. Mainstay of treatment is factor replacement therapy so far. Bleedings can be controlled by infusion of factor concentrates. Hemophilc arthropathy and muscle contracture are representative sequelae. Complications of facotor replacement therapy are inhibitor development and infections. Hemophiliacs with inhibitor should be managed with large dose factor concentrate, bypassing agent, ITI and immunosuppression. Ultimately, hemophilia could be cured by gene therapy.

• 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.

• The Journal of the Korea Contents Association
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• v.22 no.4
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• pp.700-705
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• 2022

Hemophilia is a congenital hemorrhagic disease in which blood clotting is impaired and hemostatic abnormalities occur when even one of the 13 factors required for blood clotting are deficient. Among the 13 factors, hemophilia A (factor 8), hemophilia B (factor 9), and hemophilia C (factor 10), depending on the deficient coagulation factor. A male patient in his 40s diagnosed with hemophilia A visited the dentist at Eulji University Hospital for problems with alveolar bone absorption and periodontal abscess caused by overall chronic tooth inflammation, and showed overall bone absorption and inflammation at the time of initial diagnosis. No complications or bleeding tendency were observed in the rest of the area except for #35 and #36 due to regular visits for 10 years, overall good oral conditions, and no side effects or complications were observed.

• 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 The Korean Dental Society of Anesthesiology
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• v.12 no.4
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• pp.229-233
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• 2012

Hemophilia, the most common of the inherited bleeding disorder, is the result of a deficiency of clotting factor. Since bleeding after dental treatment may cause severe or even fatal complications, people with hemophilia must be given special dental care. We report on the diagnosis and treatment of a 9-year-old boy having severe hemophilia visited our department with the chief complaints of pus discharge on the left lower molar region. In the clinical and radiographic examination, periapical abscess and dental caries were diagnosed. Considering complexity of the treatment and complication in the coagulation, it was decided to carry on the treatment under general anesthesia. Clotting factor IX concentrates were intended to provide 50-70% plasma level. Pulpectomy, resin restoration and Stainless steel crown were given under general anesthesia. Several teeth were extracted and the sockets were packed with Surgicel$^{(R)}$ (Oxidized Regenerated Cellulose, Johnson and Johnson Co. Neuchatel, Switzerland) under general anesthesia. Transpalatal arch and lingual arch were given for maintaining the extracted space before discharged. For people with severe hemophilia, factor replacement is necessary before scaling, surgery or regional block injections. Therefore, if several extractions are needed, dental care under general anesthesia would be effective and efficient management.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.4
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• pp.589-594
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• 2005

With the progress of medical treatment techniques of bleeding control, dental care of the patient with hemophilia has become more convenient. So many surgical treatments can be performed with out-patient. 2 cases of intraoral surgical treatment of children, one with hemophilia 3, sever, the other with hemophilia A, severe. While the former was treated under general anesthesia, the latter was treated under local anaesthesia. There are principles : 1. When a patient with hemophilia need dental treatment, the dentist must consult to his physician, pediatrician, or hematologist before dental treatment. 2. When the dentist make a treatment plan, there should be consideration of the general condition, cooperation of the patient and risk of the treatment needed. 3. Minimize the number of times of coming for dental treatment so that reduce the times that need replacement therapy of coagulation factor. And during the treatment, dentist should care for infection and bleeding.

• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.397-407
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• 2010

Purpose : The F9 gene is known to be the causative gene for hemophilia B, but unfortunately the detection rate for restriction fragment length polymorphism-based linkage analysis is only 55.6%. Direct DNA sequencing can detect 98% of mutations, but this alternative procedure is very costly. Here, we conducted multiplex polymerase chain reactions (PCRs) and conformation sensitive gel electrophoresis (CSGE) to perform a screened DNA sequencing for the F9 gene, and we compared the results with direct sequencing in terms of accuracy, cost, simplicity, and time consumption. Methods : A total of 27 unrelated hemophilia B patients were enrolled. Direct DNA sequencing was performed for 27 patients by a separate institute, and multiplex PCR-CSGE screened sequencing was done in our laboratory. Results of the direct DNA sequencing were used as a reference, to which the results of the multiplex PCR-CSGE screened sequencing were compared. For the patients whose mutation was not detected by the 2 methods, multiplex ligation-dependent probe amplification (MLPA) was conducted. Results : With direct sequencing, the mutations could be identified from 26 patients (96.3%), whereas for multiplex PCRCSGE screened sequencing, the mutations could be detected in 23 (85.2%). One patient's mutation was identified by MLPA. A total of 21 different mutations were found among the 27 patients. Conclusion : Multiplex PCR-CSGE screened DNA sequencing detected 88.9% of mutations and reduced costs by 55.7% compared with direct DNA sequencing. However, it was more labor-intensive and time-consuming.

• 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.