• 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.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.

• 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.

• Journal of the korean academy of Pediatric Dentistry
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• v.23 no.3
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• pp.593-600
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• 1996

This case report is a treatment of patient with hemophilia A and autism. The patient's chief complaint was treatment of dental caries on entire dentition and he has an impacted mesiodens located on the apex of the upper right primary central incisor. The patient was consulted with pediatrician and anesthetist about the detailed discussion of the complexities of hemorrhagic disorder. Because he had some problems of behavior management and bleeding, the treatment was done under the gerneral anesthesia. The following results were obtained. 1. Consult with the patient's physician and hematologist about the replacement therapy and bleeding tendency. 2. For the severe hemophiliac child who requires extensive or surgical treatment, general anesthesia may be indicated for the comprehensive care. 3. For the hemophiliac child who has a behavior management problem due to autism and other defects, general anesthesia may be considerable. 4. Care must be taken during dental procedures not to causing a bleeding. 5. Local hemostatic methods must be acquired for the emergency state. 6. Neve prescribe aspirin or non-steroidal anti-inflammatory drugs such as ibuprofen, indomethacin, and phenylbutazone. These drugs affect platelet aggregation and exaggerate the bleeding defect. 7. Do not be afraid of hemophiliac patient, and never compromise quality of dental care.

• Journal of the Korean Arthroscopy Society
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• v.6 no.2
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• pp.177-182
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• 2002

Purpose : To validate the arthroscopic synovectomy for the treatment of hemophilic knee. Materials and Methods: From January 1996 to January 2001, 28 arthroscopic synovectolny were performed in 26 patients with hemophilic arthropathy of the knee. The mean age was 17.8 years. The mean follow-up period was 3 years 11 months. We used six portals (two anterior, two suprapatellar and two posterior) and posterior trans-septal portal in all cases. Result : The mean frequency of hemarthrosis was 4 times per month preoperatively and 2 postoperatively. The mean amount of factor replacement was 4,633 units preoperatively and 1,505 postoperatively. The mean range of motion was $112^{\circ}$ preoperatively and $107^{\circ}$ postoperatively. On radiographic evaluation, three cases were progressed at the latest follow-up. On the subjective evaluation, significant or moderate improvement were in 19 cases $(68\%)$, and no improvement or deterioration in 9 cases $(32\%)$. Conclusion : With complete synovectomy through the appropriate arthroscopic portals, arthroscopic synovectomy of the knee in hemophilic patients is the successful method in decreasing bleeding episodes, amount of factor replacement, knee pain and preventing or delaying onset of end-stage hemophilic arthropathy.

• 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.

• 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.

• Radiation Oncology Journal
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• v.23 no.2
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• pp.106-110
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• 2005

Purpose : Repetitive bleeding into the joint space is the cause of debilitative hemophilic arthropathy. To interrupt this process, we treated the hemophilic patients suffering from repetitive joint bleeding with radiation therapy. Materials and Methods : From 1997 to 2001, a total of 41 joints from 37 hemophilic arthropathy patients were treated with radiation therapy at Kyung Hee University Hospital. The treated joints were 35 ankles, 3 knees and 3 elbows, respectively. The age of the patients ranged from 4 to 27 years (median age: 11 years). The radiation dose ranged from 900 cGy to 2360 cGy (median dose: 900cGy). The fraction size was 150 cGy, 180 cGy or 200 cGy. The number of bleeding in one year before and after radiotherapy was compared. Results : There was a tendency of frequent bleeding for the Patients younger than 11 (p=0.051) but there was also a tendency for more improvement in this group (p=0.057). The number of joint bleedings was related with joint pain (p=0.012) and joint swelling (p=0.033) but not with the Arbold-Hilgartner stage (p=0.739), cartilage destruction (p=0.718) and synovial hypertrophy (p=0.079). The number of bleeding was reduced in thirty-three cases, and eight cases showed no improvement after radiation therapy. The average number of bleeding in a month was 2.52 before radiotherapy, but this was reduced to 1.4 after radiotherapy (p=0.017). Conclusion : Radiation therapy was effective for the hemophilia patients with repetitive joint bleeding to decrease the bleeding frequency and to prevent hemophilic arthropathy.

• 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.

• 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.