• Journal of The Korean Society of Inherited Metabolic disease
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• v.24 no.1
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• pp.10-16
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• 2024

Congenital Disorders of Glycosylation (CDG) represent a complex group of inherited metabolic disorders resulting from defects in multiple pathways of glycosylation, a critical biochemical process for protein functionality and cellular communication. This review provides a comprehensive overview of CDG, including its history, epidemiology, classification, diagnostic complexities, and therapeutic developments. Despite advancements in understanding CDG and identifying over 160 subtypes, challenges remain due to the diverse clinical manifestations and multi-systemic involvement. Targeted therapy is available for only a few CDGs, but promising treatments are being investigated. Ongoing research is vital to developing targeted treatments and improving patient outcomes.

• The Journal of Korean Society for Radiation Therapy
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• v.26 no.1
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• pp.137-147
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• 2014

Purpose : The purpose of this study is to verify the accuracy of dose delivery according to the patient's breathing cycle in Gated Volumetric Modulated Arc Therapy Materials and Methods : TrueBeam STxTM(Varian Medical System, Palo Alto, CA) was used in this experiment. The Computed tomography(CT) images that were acquired with RANDO Phantom(Alderson Research Laboratories Inc. Stamford. CT, USA), using Computerized treatment planning system(Eclipse 10.0, Varian, USA), were used to create VMAT plans using 10MV FFF with 1500 cGy/fx (case 1, 2, 3) and 220 cGy/fx(case 4, 5, 6) of doserate of 1200 MU/min. The regular respiratory period of 1.5, 2.5, 3.5 and 4.5 sec and the patients respiratory period of 2.2 and 3.5 sec were reproduced with the $QUASAR^{TM}$ Respiratory Motion Phantom(Modus Medical Devices Inc), and it was set up to deliver radiation at the phase mode between the ranges of 30 to 70%. The results were measured at respective respiratory conditions by a 2-Dimensional ion chamber array detector(I'mRT Matrixx, IBA Dosimetry, Germany) and a MultiCube Phantom(IBA Dosimetry, Germany), and the Gamma pass rate(3 mm, 3%) were compared by the IMRT analysis program(OmniPro I'mRT system software Version 1.7b, IBA Dosimetry, Germany) Results : The gamma pass rates of Case 1, 2, 3, 4, 5 and 6 were the results of 100.0, 97.6, 98.1, 96.3, 93.0, 94.8% at a regular respiratory period of 1.5 sec and 98.8, 99.5, 97.5, 99.5, 98.3, 99.6% at 2.5 sec, 99.6, 96.6, 97.5, 99.2, 97.8, 99.1% at 3.5 sec and 99.4, 96.3, 97.2, 99.0, 98.0, 99.3% at 4.5 sec, respectively. When a patient's respiration was reproduced, 97.7, 95.4, 96.2, 98.9, 96.2, 98.4% at average respiratory period of 2.2 sec, and 97.3, 97.5, 96.8, 100.0, 99.3, 99.8% at 3.5 sec, respectively. Conclusion : The experiment showed clinically reliable results of a Gamma pass rate of 95% or more when 2.5 sec or more of a regular breathing period and the patient's breathing were reproduced. While it showed the results of 93.0% and 94.8% at a regular breathing period of 1.5 sec of Case 5 and 6, it could be confirmed that the accurate dose delivery could be possible on the most respiratory conditions because based on the results of 100 patients's respiratory period analysis as no one sustained a respiration of 1.5 sec. But, pretreatment dose verification should be precede because we can't exclude the possibility of error occurrence due to extremely short respiratory period, also a training at the simulation and careful monitoring are necessary for a patient to maintain stable breathing. Consequently, more reliable and accurate treatments can be administered.

• Journal of the Korean Society of Radiology
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• v.12 no.3
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• pp.427-433
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• 2018

Because of the expectation of the non-invasive treatment effect, Various studies on the treatment of varicose veins using focused ultrasound are reported. In this study, the bio-tissue phantom and tissue equivalent phantom that can be applied to estimation of ultrasonic varicose veins treatment effect. Each phantom was evaluated for its usefulness by evaluating the acoustic characteristics and the shrinkage rate according to the ultrasonic irradiation. A multi-layer structure phantom with three layers of skin, fat, and muscle was constructed considering the structure of the tissue where the varicose veins occurred. The materials constituting each layer were made to have characteristics similar to human body. In addition, the multi-layered phantoms with blood vessel mimic tube, with bovine blood vessel, and with animal tissue were fabricated. The degree of shrinkage of blood vessel mimic material and vascular tissue according to ultrasonic irradiation was evaluated using B-mode image. As the results of this study, it was thought that the proposed phantom could be used effectively in the evaluation of ultrasonic varicose veins treatment. In addition, it is thought that these phantoms could be applied to the development of varicose vein treatment device using the focused ultrasound and the verification of the therapeutic effect.

• Proceedings of the Korean Society of Medical Physics Conference
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• 2002.09a
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• pp.129-132
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• 2002

The practical virtual compensator, which uses a dynamic multi-leaf collimator (dMLC) and three-dimensional radiation therapy planning (3D RTP) system, was designed. And the feasibility study of the virtual compensator was done to verify that the virtual compensator acts a role as the replacement of the physical compensator. Design procedure consists of three steps. The first step is to generate the isodose distributions from the 3D RTP system (Render Plan, Elekta). Then isodose line pattern was used as the compensator pattern. Pre-determined compensating ratio was applied to generate the fluence map for the compensator design. The second step is to generate the leaf sequence file with Ma's algorithm in the respect of optimum MU-efficiency. All the procedure was done with home-made software. The last step is the QA procedure which performs the comparison of the dose distributions which are produced from the irradiation with the virtual compensator and from the calculation by 3D RTP. In this study, a phantom was fabricated for the verification of properness of the designed compensator. It is consisted of the styrofoam part which mimics irregular shaped contour or the missing tissues and the mini water phantom. Inhomogeneous dose distribution due to the styrofoam missing tissue could be calculated with the RTP system. The film dosimetry in the phantom with and without the compensator showed significant improvement of the dose distributions. The virtual compensator designed in this study was proved to be a replacement of the physical compensator in the practical point of view.

• The Korean Journal of Gastroenterology
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• v.72 no.6
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• pp.286-294
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• 2018

Background/Aims: The predictive factors of functional dyspepsia (FD) remain controversial. Therefore, we sought to investigate symptom responses in FD patients after Helicobacter pylori (H. pylori) eradication and used predictive factor analysis to identify significant factors of FD resolution at one-year after commencing eradication therapy. Methods: This prospective, multi-center clinical trial was performed on 65 FD patients that met Rome III criteria and had H. pylori infection. Symptom responses and factors that predicted poor response were determined by analysis one year after commencing H. pylori eradication therapy. Results: A total of 63 patients completed the one-year follow-up. When an eradication success group (n=60) and an eradication failure group (n=3) were compared with respect to FD response rate at one year, results were as follows; complete response 73.3% and 0.0%, satisfactory response 1.7% and 0.0%, partial response 10.0% and 33.3%, and refractory response 15.0% and 66.7%, respectively (p=0.013). Univariate analysis showed persistent H. pylori infection (p=0.021), female gender (p=0.025), and medication for FD during the study period (p=0.013) were associated with poor FD response at one year. However, age, smoking, alcohol consumption, and underlying disease were not found to affect response. Finally, multivariate analysis showed that female gender (OR, 4.70; 95% CI, 1.17-18.88) was the sole independent risk factor of poor FD response at one year after commencing H. pylori eradication therapy. Conclusions: Female gender was found to predict poor response in FD patients despite H. pylori eradication. Furthermore, successful H. pylori eradication appears to be associated with FD improvement, but the number of non-eradicated patients was too small to conclude.

• Asian Pacific Journal of Cancer Prevention
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• v.14 no.12
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• pp.7367-7369
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• 2013

Background: Inoperable and metastatic hepatocellular carcinoma (HCC) is associated with a poor prognosis and low chemotherapeutic efficiency. Sorafenib is an oral multi-kinase inhibitor exerting its effects via the RAF/MEK/ERK pathway, vascular endothelial growth factor receptor (VEGFR) and platelet derived growth factor receptor beta (PDGFR-${\beta}$) tyrosine kinases. Randomized studies have shown a significant contribution of sorafenib to life expectancy and quality of life of cancer patients. The aim of the present study is to evaluate the efficacy and side effects of sorafenib therapy in Turkey. Materials and Methods: Data for 103 patients (82 males, 21 females) receiving sorafenib therapy in 13 centers from February 2008 to December 2012 were evaluated. Median age was 61 years and median ECOG performance status was 1 (range: 0-2). 60 patients (58%) had hepatitis B, 15 patients (15%) had hepatitis C infection and 12 patients (12%) had a history of alcohol consumption. All of the patients had Child scores meeting the utilization permit of the drug in our country (Child A). Results: A total of 571 cycles of sorafenib therapy were administered with a median of four per patient. Among the evaluable cases, there was partial response in 15 (15%), stable disease in 52 (50%), and progressive disease in 36 (35%). Median progression-free survival was 18 weeks and median overall survival was 48 weeks. The dose was reduced only in 6 patients and discontinued in 2 patients due to grade 3-4 toxicity, 18 patients (17%) suffering hand-foot syndrome, 7 (7%) diarrhea, and 2 (2%) vomiting. Conclusions: This retrospective study demonstrated better efficacy of sorafenib therapy in patients with advanced HCC compared to the literature while progression-free survival and overall survival findings were comparable. The side effect rates indicate that the drug was tolerated well. In conclusion, among the available treatment options, sorafenib is an efficient and tolerable agent in patients with inoperable or metastatic HCC.

• Progress in Medical Physics
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• v.16 no.1
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• pp.24-31
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• 2005

The stereotactic radiosurgery (SRS) describes a method of delivering a high dose of radiation to a small tar-get volume in the brain, generally in a single fraction, while the dose delivered to the surrounding normal tissue should be minimized. To perform automatic plan of the SRS, a new method of multi-isocenter/shot linear accelerator (linac) and gamma knife (GK) radiosurgery treatment plan was developed, based on a physical lattice structure in target. The optimal radiosurgical plan had been constructed by many beam parameters in a linear accelerator or gamma knife-based radiation therapy. In this work, an isocenter/shot was modeled as a sphere, which is equal to the circular collimator/helmet hole size because the dimension of the 50% isodose level in the dose profile is similar to its size. In a computer-aided system, it accomplished first an automatic arrangement of multi-isocenter/shot considering two parameters such as positions and collimator/helmet sizes for each isocenter/shot. Simultaneously, an irregularly shaped target was approximated by cubic structures through computation of voxel units. The treatment planning method by the technique was evaluated as a dose distribution by dose volume histograms, dose conformity, and dose homogeneity to targets. For irregularly shaped targets, the new method performed optimal multi-isocenter packing, and it only took a few seconds in a computer-aided system. The targets were included in a more than 50% isodose curve. The dose conformity was ordinarily acceptable levels and the dose homogeneity was always less than 2.0, satisfying for various targets referred to Radiation Therapy Oncology Group (RTOG) SRS criteria. In conclusion, this approach by physical lattice structure could be a useful radiosurgical plan without restrictions in the various tumor shapes and the different modality techniques such as linac and GK for SRS.

• Progress in Medical Physics
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• v.18 no.2
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• pp.87-92
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• 2007

A thermal neutron beam facility utilizing a typical tangential beam port for Neutron Capture Therapy was installed at the HANARO, 30 MW multi-purpose research reactor. Mixed beams with different physical characteristics and relative biological effectiveness would be emitted from the BNCT irradiation facility, so a quantitative analysis of each component of the mixed beams should be performed to determine the accurate delivered dose. Thus, various techniques were applied including the use of activation foils, TLDs and ionization chambers. All the dose measurements were perform ed with the water phantom filled with distilled water. The results of the measurement were compared with MCNP4B calculation. The thermal neutron fluxes were $1.02E9n/cm^2{\cdot}s\;and\;6.07E8n/cm^2{\cdot}s$ at 10 and 20 mm depth respectively, and the fast neutron dose rate was insignificant as 0.11 Gy/hr at 10 mm depth in water The gamma-ray dose rate was 5.10 Gy/hr at 20 mm depth in water Good agreement within 5%, has been obtained between the measured dose and the calculated dose using MCNP for neutron and gamma component and discrepancy with 14% for fast neutron flux Considering the difficulty of neutron detection, the current study support the reliability of these results and confirmed the suitability of the thermal neutron beam as a dosimetric data for BNCT clinical trials.

• The Journal of Korean Physical Therapy
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• v.10 no.1
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• pp.75-80
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• 1998

To survey the recognition of Community Based Rehabilitation (CBR) by Public Health Center(PHC) Physical Therapists in Kyoungki Province. This study analysed general characteristic of Physical Therapists and congnition, participation, education, problems and requireanents for CBR. The objects of this study were 37 therapists who work at 27 of 39 PHC in Kyoungki province and data was collected for 20days from Jan. 12 to 31. 1998 and the results computed from 32 responses. First, Female therapists outnumber males. The age category 30 to 35 old represneted $56.2\%$ of the total number studied. $59.4\%$ of therapists had worked more than 7 years and therapists usually$(46.9\%)$ worked from 6 month to 2 years at the PHC. Junior college graduates were $84.4\%$, and respondents with family responsibilirt were $65.6\%$. Second, Recently pain control has become the major requirment of patiants at PH$(53.1\%)$. If they offered expaneded services - a rehabilitation program $(53.1\%)$, home vistation $(31.3\%)$. when asked about a working guide book, nobody had one. Third, $68.7\%$ of therapists were aware of the present level of CBR, $40.9\%$ of them had gotten an information from educated colleagues, and $68.7\%$ of therapists were aware of the present level of CBR, $86.4\%$ of therapists felt responsible to provide rehabilitation Fourth, For CBR, $93.7\%$ had positive ideas in which they could participate. $73.3\%$ wished to composed a team of various rehabilitational specialists. $73.3\%$ wished for the meeting day to be flexible and $86.6\%$ desired to meet once or twice a week. Fifth, Almost all$(96.9\%)$ desired more educational opportunity about CBR. Example, once or twice a year$(56.2\%)$ and training period of 4-7 days $(43.7\%)$. They wanted to learn theory and practice together$(74.2\%)$ and $74.2\%$ wanted the mational multi-rehabilitation center as a educational managing organization. Sixth, The most important requirment for accomplishing CBR by physical therapists was job security and an adequate number of therapists$(84.4\%)$ Required number of physical therapists in the PHC was 3 $(43.7\%)$. The cooperative agency should be the district goverment organization $(56.2\%)$. Factors inhibiting the execution of CBR were lack of administrative support, physical therapists, and equipments for pain control and for therapeutic exercise.

• Journal of Korean Neurosurgical Society
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• v.54 no.6
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• pp.489-495
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• 2013

Objective : To evaluate the efficacy of temozolomide (TMZ) chemotherapy for recurrent anaplastic oligodendroglioma (AO) and anaplastic oligoastrocytoma (AOA). Methods : A multi-center retrospective trial enrolled seventy-two patients with histologically proven AO/AOA who underwent TMZ chemotherapy for their recurrent tumors from 2006 to 2010. TMZ was administered orally (150 to 200 $mg/m^2/day$) for 5 days per 28 days until unacceptable toxicity occurred or tumor progression was observed. Results : TMZ chemotherapy cycles administered was median 5.3 (range, 1-41). The objective response rate was 24% including 8 cases (11%) of complete response and another 23 patients (32%) were remained as stable disease. Severe side effects (${\geq}$grade 3) occurred only in 9 patients (13%). Progression-free survival (PFS) of all patients was a median 8.0 months (95% confidence interval, 6.0-10.0). The time to recurrence of a year or after was a favorable prognostic factor for PFS (p<0.05). Overall survival (OS) was apparently differed by the patient's histology, as AOA patients survived a median OS of 18.0 months while AO patients did not reach median OS at median follow-up of 11.5 months (range 2.7-65 months). Good performance status of Eastern Cooperative Oncology Group 0 and 1 showed prolonged OS (p<0.01). Conclusion : For recurrent AO/AOA after surgery followed by radiation therapy, TMZ could be recommended as a salvage therapy at the estimated efficacy equal to procarbazine, lomustine, and vincristine (PCV) chemotherapy at first relapse. For patients previously treated with PCV, TMZ is a favorable therapeutic option as 2nd line salvage chemotherapy with an acceptable toxicity rate.