• 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.

• Archives of Plastic Surgery
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• 제34권4호
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• pp.516-519
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• 2007

Purpose: Hemophilia is one of the most common bleeding disorder, and circulating levels of factor VIII or IX are closely related to the severity and frequency of the bleeding. The severity of hemophilia is classified to be severe, moderate, and mild when the factor level is less than 1%, between 1% and 5%, and between 5% and 25%, respectively. Hemophilic pseudotumor is a rare complication occurring in 1 - 2% of patients with factor VIII or IX deficiency. If the treatment is delayed, it would cause disabling and life threatening results. Methods: We experienced hemophilic pseudotumor developed at the fracture site of the proximal phalanx of the hand in a hemophilic B patient. Hemophilic pseudotumor was successfully treated with perioperative factor replacement and surgical intervention included excision and autologous bone graft. Results: Hemophilic pseudotumor was healed with complete regression, and no specific complication was developed. Conclusion: When we accounter hematoma like lesion after surgery unpredictably, we must consider hemophilic pseudotumor and make a accurate diagnosis with preoperative hematologic screening and various imaging study. Subsequently, adequate perioperative supplement of concentrate and surgical intervention brings to the satisfactory result without recurrence.

• 아동학회지
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• 제4권
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• pp.37-51
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• 1983

The purpose of this study was the investigation of theoretical and empirical relationships between Piagetian and psychometric assessments of intelligence. Specifically, the factor structure of Piagetian-type scales, the relationship between Piagetian scales and psychometric intelligence tests, and differences in the factor structure of Piagetian and psychometric assessments of intelligence were studied. The subjects of this stuby were 70 children (35 boys and 35 girls) in the 1st grade of an elementary school in Seoul The Piagetian-type scales and the K-WISC were administered individually, and the General Intelligence Test was administered to groups of children. Statistical analysis of the obtained data consisted of the SPSS Computer program including factor analysis and Pearson's product moment correlation coefficient. The Piagetian-type scales were found to consist of three factors, which accounted for 55 percent of the total common-factor variance. Factor-I was a factor indicating "conservation". Factor-II was a factor indicating "moral judgements". Factor-III was a factor indicating "classification and identity". Correlations between subtests of psychometric tests and Piagetian scales were relatively low or moderate. Relations between IQs assessed by the psychometric tests and Piagetian scales were also relativeyly low or moderate. Eight factors were extracted from the joint factor analysis of psychometric intelligence tests and Piagetian scales, and they accounted for 67 percent of the total common-factor variance. Factors-I, II, III, and V consisted of subtests of psychometric assessments, and Factors-IV, VI, VII and VIII were composed of Piagetian scales. Factor-I was a factor for "reasoning ability based upon language". Factor-II was a factor for "performance ability". Factor-III was a factor for "grouping ability". Factor-IV was a factor for "conservation". Factor-V was a factor indicating "symbol and language usage ability". Factor- VI was a factor indicating "moral judgments". Factor-VII was a factor indicating "length consevation". Factor-VIII was a factor indicating "classification and identity".

• Nuclear Engineering and Technology
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• 제52권12호
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• pp.2725-2732
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• 2020

This paper presents the neutronics benchmark analysis of the first core of the Indonesian multipurpose research reactor RSG-GAS (Reaktor Serba Guna G.A. Siwabessy) calculated by the Serpent Monte Carlo code and the newly released ENDF/B-VIII.0 nuclear data library. RSG-GAS is a 30 MWth pool-type material testing research reactor loaded with plate-type low-enriched uranium fuel using light water as a coolant and moderator and beryllium as a reflector. Two groups of critical benchmark problems are derived on the basis of the criticality and control rod calibration experiments of the first core of RSG-GAS. The calculated results, such as the neutron effective multiplication factor (k) value and the control rod worth are compared with the experimental data. Moreover, additional calculated results, including the neutron spectra in the core, fission rate distribution, burnup calculation, sensitivity coefficients, and kinetics parameters of the first core will be compared with the previous nuclear data libraries (interlibrary comparison) such as ENDF/B-VII.1 and JENDL-4.0. The C/E values of ENDF/B-VIII.0 tend to be slightly higher compared with other nuclear data libraries. Furthermore, the neutron reaction cross-sections of ¹⁶O, ⁹Be, ²³⁵U, ²³⁸U, and S(𝛼,𝛽) of ¹H in H₂O from ENDF/B-VIII.0 have substantial updates; hence, the k sensitivities against these cross-section changes are relatively higher than other isotopes in RSG-GAS. Other important neutronics parameters such as kinetics parameters, control rod worth, and fission rate distribution are similar and consistent among the nuclear data libraries.

• Toxicological Research
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• 제18권1호
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• pp.87-98
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• 2002

This study was conducted to evaluate the safety of a recombinant human Factor VIII(GC-$\gamma$ AHF) manufactured by Korea Green Cross Company with different technology according to the Regulation of Korean Food and Drug Administration (l 998. 12. 3). In acute toxicity test, both genders of Sprague-Dawley rats and Beagle dogs were administered intravenously with GC-$\gamma$ AHF of three doses (3,125, 625 and 125 IU/kg), and single dose of 3,125 IU/kg, respectively. No dead animal and abnormal autopsy findings were found in Control and GC-$\gamma$ AHF treated group. Therefore, the 50% lethal dose ($LD_{50}$) of GC-$\gamma$ AHF was conidered to be higher than 3,125 IU/kg in rats and dogs. In the four weeks repeated intravenous toxicity study, GC-$\gamma$ AHF was administrated intravenosly to both genders of rats and dogs with 3 doses (500, 150, 50 IU/kg). There were neither dead animals nor significant changes of body weights during the experimental Period. In addition, no significant GC-$\gamma$ AHF related changes were found in clinical sign, urinalysis and other finding. Statistically changes were observed in hematological, biochemical and organ weight parameters of treated groups: however these changes were not dose dependent. No histopathological lesion were observed in both control and treated animals. Above data suggest that no observed adverse effect level of test materials in rats and dogs might be over 500 IU/kg/day in this study. In ocular irritation test, any injury on iris, conjunctiva and cornea in rabbits were not observed. The acute ocular irritation index (A.O.I.), mean ocular irritation index (M.O.I.) and Day-7 individual ocular irritation Index (I.O.I.) of GC-$\gamma$ AHF were 0. In the primary skin Irritation test, the primary irritation index (P.I.I.) oj GC-$\gamma$ AHF were 0. Therefore, the GC-$\gamma$ AHF is considered not to have the primary skin and eye toxicity in rabbits. In active systemic anaphylaxis (ASA) test, GC-$\gamma$ AHF and GC-$\gamma$ AHF emulsified with Freund's complete adjuvant (FCA) did not induce any symptom of anaphylactic shock in guinea pigs. In passive cutaneous anaphylxis (PCA) test, after sensitization with antisera of GC-$\gamma$ AHF sensitized mice, blue spots were observed on the hypodermis of back of rats, but diameter of each spot was smaller than 5 mm in each test groups except the positive control group. Based on the results of this study, GC-$\gamma$ AHF is not conidered to have any antigenic potential. In conclusion, at levels of up to 500 IU/kg, GC-$\gamma$ AHF did not produce treatment-related toxicity under the conditions of these acute-, four week repeated-toxicity, primary skin and eye toxicity, and antigenicity test.

• 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 Interdisciplinary Genomics
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• 제4권1호
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• pp.15-18
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• 2022

Hemophilia A is a rare X-linked congenital deficiency of clotting factor VIII (FVIII) that is traditionally diagnosed by measuring FVIII activity. Various mutations of the FVIII gene have been reported and they influence on the FVIII protein structure. A deficiency of or reduction in FVIII protein manifests as spontaneous or induced bleeding depending on the disease severity. Mutations of the FVIII gene provide important information on the severity of disease and inhibitor development. FVIII mutations also affect the discrepant activities found using different FVIII assays. FVIII activity is affected differently depending on the mutation site. Long-range PCR is commonly used to detect intron 22 inversion, the most common mutation in severe hemophilia. However, point mutations are also common in patients with hemophilia, and direct Sanger sequencing and copy number variant analysis are being used to screen for full mutations in the FVIII gene. Advances in molecular genetic methods, such as next-generation sequencing, may enable accurate analysis of mutations in the factor VIII gene, which may be useful in the diagnosis of mild to moderate hemophilia. Genetic analysis is also useful in diagnosing carriers and managing bleeding control. This review discusses the current knowledge about mutations in hemophilia and focuses on the clinical aspects associated with these mutations and the importance of genetic analysis.

• 생약학회지
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• 제45권4호
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• pp.294-299
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• 2014

The simultaneous determination of platyphylloside, aceroside and betulin was established for the quality control of Betula platyphylla bark using a high performance liquid chromatography and diode-array UV/Vis detector (HPLC-DAD). Separation and quantification were successfully achieved with a INNO C18 column ($5{\mu}m$, 4.6 mm $I.D.{\times}150mm$) by gradient elution of a mixture of methanol and water at a flow rate of 1.0 ml/min. Validation of the developed method was performed by various factor such as linearity, specificity, precision, accuracy, system suitability and stability. This method was successfully applied to the determination of contents of platyphylloside, aceroside VIII and betulin in three batches of Betula platyphylla bark extract. These results suggest that the developed HPLC method is simple, effective and could be utilized as a quality control method for Betula platyphylla bark products.

• 한국정보통신학회논문지
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• 제9권8호
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• pp.1722-1729
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• 2005

본 연구에서는 OpenGL 프로그램으로 4족 보행로봇인 TITAN-VIII의 가상로봇을 설계한 후, 실제로봇의 관절각도, 본체 자세각을 가상로봇에 입력하고 벡터 회전과 평행이동을 이용하여 보행 중 본체를 수평으로 유지하는 제어를 10[ms]마다 행하였다. 디딤율 $\beta$를 0,5로 일정하게 하고, 주기가 1.5, 2.0, 3.0[sec]일 때 한주기당 이동거리를 0.2, 0.3[m]로 변경하여 좌우요동보행을 시키면서 가상로봇의 ZMP, 실제로봇의 ZMP 무게중심의 이동경로를 구하고 발바닥 좌표 변화와의 관계를 비교 분석하였다.

• Journal of Yeungnam Medical Science
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• 제6권2호
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• pp.247-255
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• 1989

피부 농양절개술 후 계속되는 출혈을 주소로 입원한 진성다혈증 환자에서 혈액응고인자, I, VIII, IX, XI등의 감소와 혈소판의 기능 장애를 동반한 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.