• Journal of Genetic Medicine
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• v.4 no.1
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• pp.72-79
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• 2007

Purpose : Glycogen storage disease type III (GSD-III) is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme, amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), is responsible for the debranching of the glycogen molecule during catabolism. The disease shows clinical and biochemical heterogeneity, reflecting genotype-phenotype heterogeneity among different patients. In this study, we aim at analyzing mutations of the AGL gene in three unrelated Korean GSD-III patients, and characterizing their clinical and laboratory findings. Methods : We characterized the clinical features of three unrelated Korean GSD-III patients by biochemical, histological and imaging studies. The 35 exons and part of exon-intron boundaries of AGL were analyzed by direct sequencing using genomic DNA extracted from the peripheral leukocytes of patients. Results : Diverse clinical features were observed in these patients including hepatomegaly (all patients), seizures (patient 2), grow th failure (patients 1 and 2), hyperlipidemia (patients 1 and 3), raised transaminase and creatine kinase concentrations (all patients), and mild cardiomyopathy (patient 2). Liver transplantation w as performed in patient 2 due to progressive hepatic fibrosis. A dministration of uncooked corn starch maintained normoglycemia and improved biochemical and growth profiles. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 had c.1510_1511insT (p.Y 504L fsX 10), and patient 3 had c.3416 T >C (p.L 1139P) and c.1735+1 G>T (p.Y 538_R578delfsX 4) mutations. A part from the p.R428K mutation, the 4 other substitutions identified w ere nov el. Conclusion : GSD-III patients display variable phenotypic characteristics resembling those of GSD-Ia. Molecular defects in the AGL gene of Korean GSD-III patients are genetically heterogeneous.

• Asia pacific journal of information systems
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• v.19 no.4
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• pp.77-100
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• 2009

Information Systems (IS) is an essential tool for any organizations. The last decade has seen an increasing body of knowledge on IS usage. Yet, IS often fails because of its misuse or non-use. In general, decisions regarding the selection of a system, which involve the evaluation of many IS vendors and an enormous initial investment, are made not through the consensus of employees but through the top-down decision making by top managers. In situations where the selected system does not satisfy the needs of the employees, the forced use of the selected IS will only result in their resistance to it. Many organizations have been either integrating dispersed legacy systems such as archipelago or adopting a new ERP (Enterprise Resource Planning) system to enhance employee efficiency. This study examines user resistance prior to the adoption of the selected IS or ERP system. As such, this study identifies the importance of managing organizational resistance that may appear in the pre-adoption context of an integrated IS or ERP system, explores key factors influencing user resistance, and investigates how prior experience with other integrated IS or ERP systems may change the relationship between the affecting factors and user resistance. This study focuses on organizational members' resistance and the affecting factors in the pre-adoption context of an integrated IS or ERP system rather than in the context of an ERP adoption itself or ERP post-adoption. Based on prior literature, this study proposes a research model that considers six key variables, including perceived benefit, system complexity, fitness with existing tasks, attitude toward change, the psychological reactance trait, and perceived IT competence. They are considered as independent variables affecting user resistance toward an integrated IS or ERP system. This study also introduces the concept of prior experience (i.e., whether a user has prior experience with an integrated IS or ERP system) as a moderating variable to examine the impact of perceived benefit and attitude toward change in user resistance. As such, we propose eight hypotheses with respect to the model. For the empirical validation of the hypotheses, we developed relevant instruments for each research variable based on prior literature and surveyed 95 professional researchers and the administrative staff of the Korea Photonics Technology Institute (KOPTI). We examined the organizational characteristics of KOPTI, the reasons behind their adoption of an ERP system, process changes caused by the introduction of the system, and employees' resistance/attitude toward the system at the time of the introduction. The results of the multiple regression analysis suggest that, among the six variables, perceived benefit, complexity, attitude toward change, and the psychological reactance trait significantly influence user resistance. These results further suggest that top management should manage the psychological states of their employees in order to minimize their resistance to the forced IS, even in the new system pre-adoption context. In addition, the moderating variable-prior experience was found to change the strength of the relationship between attitude toward change and system resistance. That is, the effect of attitude toward change in user resistance was significantly stronger in those with prior experience than those with no prior experience. This result implies that those with prior experience should be identified and provided with some type of attitude training or change management programs to minimize their resistance to the adoption of a system. This study contributes to the IS field by providing practical implications for IS practitioners. This study identifies system resistance stimuli of users, focusing on the pre-adoption context in a forced ERP system environment. We have empirically validated the proposed research model by examining several significant factors affecting user resistance against the adoption of an ERP system. In particular, we find a clear and significant role of the moderating variable, prior ERP usage experience, in the relationship between the affecting factors and user resistance. The results of the study suggest the importance of appropriately managing the factors that affect user resistance in organizations that plan to introduce a new ERP system or integrate legacy systems. Moreover, this study offers to practitioners several specific strategies (in particular, the categorization of users by their prior usage experience) for alleviating the resistant behaviors of users in the process of the ERP adoption before a system becomes available to them. Despite the valuable contributions of this study, there are also some limitations which will be discussed in this paper to make the study more complete and consistent.

• The Korean Journal of Nuclear Medicine
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• v.39 no.1
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• pp.44-48
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• 2005

Purpose: Ethylenediamine-tetramethylenephosphonic acid (EDTMP) has widely used chelator for the labeling of bone seeking radiopharmaceuticals complexed with radiometals. $^{153}Sm$ can be produced by the HANARO reactor at the Korea Atomic Energy Research Institute, Taejon, Korea. $^{153}Sm$ has favourable radiation characteristics $T1/2=46.7\;h,\;{\beta}_{max}=0.81\;MeV\;(20%),\;0.71\;MeV\;(49%),\;0.64\;MeV\;(30%)\;and\;{\gamma}=103\;keV\;(30%)$ emission which is suitable for imaging purposes during therapy. We investigated the labeling condition of $^{153}Sm$-EDTMP and imaging of $^{153}Sm$-EDTMP in normal rats. Materials and methods: EDTMP 20 mg was solved in 0.1 mL 2 M NaOH. $^{153}SmCl^3$ was added to EDTMP solution and pH of the reaction mixtures was adjusted to 3 and 12, respectively. Radiochemical purity was determined with paper chromatography. After 30 min. reaction, reaction mixtures were neutralized to pH 7.4, and the stability was estimated upto 120 hrs. Imaging studies of each reaction were perfomed in normal rats (37 MBq/0.1 mL). Results: The labeling yield of $^{153}Sm$-EDTMP was 99%. The stability of pH 8 reaction at 60, 96 and 120 hr was 99%, 95%, 89% and that of pH 12 at 36, 60, 96 and 120 hr was 99%, 95%, 88%, 66%, respectively. The $^{153}Sm$-EDTMP showed constantly higher bone uptake from 2 to 48 hr after injection. Conclusion: $^{153}Sm$-EDTMP, labeled at pH 8 reaction condition, has been stably maintained. Image of $^{153}Sm$-EDTMP at 2, 24, 48 hr after injection, demonstrate that $^{153}Sm$-EDTMP is a good bone seeking radiopharmaceuticals.

• The Korean Journal of Nuclear Medicine
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• v.39 no.1
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• pp.49-56
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• 2005

Purpose: Dedicated animal PET is useful equipment for the study of new PET tracer. recently, microPET R4 was installed in the Korea institute of radiology and medical science. In this study, we measured the characteristics of scanner. Materials and methods: Resolution was measured using a line source (F-18:65 ${\mu}Ci$, inner diameter: 0.5 mm). The line source was put in the axial direction and was moved from the center of field of view to outside with 1 mm interval. PET images were reconstructed using a filtered back-protection and ordered subset expectation maximization. line source (16.5 ${\mu}Ci$, 78 mm) was put on the tenter of axial direction to measure the sensitivity when the deadtime was under 1%. Images were acquired during 4 minutes respectively from center to 39 mm outward. Delayed count was subtracted from total count and then decay was corrected for the calculation of sensitivity. Noise equivalent count ratio and scatter fraction were calculated using cylindrical phantom. Results: Spatial resolution of reconstructed image using filtered back-projection was 1.86 mm(radial), 1.95 mm(tangential), 1.95 mm(axial) in the tenter of field of view, and 2.54 mm, 2.8 mm, 1.61 mm in 2 cm away from the center respectively. Sensitivity was 2.36% at the center of transaxial field of view. Scatter fraction was 20%. Maximal noise equivalent count ratio was 66.4 kcps at 242 kBq/mL. Small animal images were acquired for confirmation of performance. Conclusion: Performance characteristics of microPET R4 were similar with reported value. So this will be a useful tool for small animal imaging.

• The Korean Journal of Nuclear Medicine
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• v.39 no.3
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• pp.191-199
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• 2005

Purpose: In order to obtain better quantitation of kidney uptake, this study is to evaluate a conjugate view method (CVM) using a geometric mean attenuation correction for kidney uptake and to compare it to Gate's method. Materials & Methods: We used a Monte Carlo code, SIMIND and a Zubal phantom, to simulate kidney uptake. SIMIND was both simulated with or without scatter for the Zubal phantom. Also, a real phantom test was carried out using a dual-head gamma camera. The activity of 0.5 mCi was infused into two small cylinder phantoms of 5 cm diameter, and then, they were inserted into a cylinder phantom of 20 cm diameter. The results by the CVM method were compared with ideal data without both of attenuation and scatter and with Gate's method. The CVM was performed with or without scatter correction. The Gate's method was performed without scatter correction and it was evaluated with regards to $0.12cm^{-1}\;and\;0.15cm^{-1}$ attenuation coefficients. Data were analyzed with comparisons of mean counts in the legions of interest (ROI), profiles drawn over kidney images and linear regression. Correlation coefficients were calculated with ideal data, as well. Results: In the case of the computer simulation, mean counts measured from ideal data, the CVM and the Gate's method were (right $998{\pm}209$, left: $896{\pm}249$), (right: $911{\pm}207$, left: $815{\pm}265$), and (right: $1065{\pm}267$, left: $1546{\pm}267$), respectively. The ideal data showed good correlation with the CVM and the correlation coefficients of the CVM, Gate's method were (right: 0.91, left: 0.93) and (right: 0.85, left: 0.90), respectively. Conclusion: The conjugate view method using geometric mean attenuation correction resulted in better accuracy than the Gate's method. In conclusion, the conjugate view method independent of renal depths may provide more accurate kidney uptake.

• The Korean Journal of Nuclear Medicine
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• v.36 no.4
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• pp.261-270
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• 2002

Purpose: Nicotinic acetylcholine receptors (nAChRs), which mediate excitatory neurotransmission, are known to participate in various neurophysiological functions. Severe losses of nAChRs have been noted in Alzheimer's and Parkinson's diseases. Therefore, noninvasive and quantitative imaging of nAChRs would offer a better understanding on the function of these receptors. In this study, $2-[^{18}F]fluoro-A85380\;([^{18}F]1)$, an ${\alpha}_4{\beta}_2$ nAChRs radioligand, was prepared using one HPLC purification and evaluated in mouse brain, and the results were compared with those in the literature. Materials and Methods: $[^{18}F]1$ was prepared by $[^{18}F]$fluorination of the iodo precursor followed by acidic deprotection and then purified by HPLC. Tissue distribution studies were performed in mouse brain at the indicated time points and the result was expressed as %ID/g. Inhibition studies were also carried out with pretreatment of various ligands. Results: One HPLC purification method gave the desired product in 15-20% radiochemical yield and with high specific activity ($38-55GBq/{\mu}mol$). Tissue distribution studies showed that $[^{18}F]1$ specifically labeled nAChRs in mouse brain with a high thalamus to cerebellum uptake ratio (13.8 at 90 min). Inhibition studios demonstrated selective binding of $[^{18}F]1$ to nAChRs, blocking the uptake of the $[^{18}F]1$ in nAChR-rich legions by selective ligands such as cytisine and nicotine which are well-known nAChRs agonists. Conclusion: This study demonstrated that the $[^{18}F]1$ produced by the method using one HPLC purification gave the results similar to those reported in the literature. Therefore, this synthetic method can be readily applied to the routine preparation of $[^{18}F]1$, a PET radioligand for ${\alpha}_4{\beta}_2$ nAChRs imaging.

• The Korean Journal of Nuclear Medicine
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• v.33 no.4
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• pp.398-404
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• 1999

Purpose: Diabetic nephropathy is the most common cause of end stage renal disease and the incidence is progressively increasing. The aim of this study was to investigate the differences of $^{99m}Tc$-DMSA renal uptake among diabetic patients with normoalbuminuria, microalbuminuria and overt proteinuria, and then to determine the clinical usefulness of $^{99m}Tc$-DMSA in predicting early diabetic nephropathy Materials and Methods: $^{99m}Tc$-DMSA scan was performed and a total renal uptake of $^{99m}Tc$-DMSA was measured in 145 diabetic patients. Patients were divided into 3 groups according to the amount of 24 hour urinary albumin excretion as Group I (normoalbuminuria, 74 cases), Group II (microalbuminuria, 39 cases), and Group III (overt proteinuria, 32 cases). The differences of $^{99m}Tc$-DMSA renal uptake among the 3 groups and the correlation between the renal uptake of $^{99m}Tc$-DMSA and other clinical parameters were analyzed. Results: The total renal uptake of $^{99m}Tc$-DMSA of Group II ($40.8{\pm}11.0%$) was significantly lower than that of Group I ($54.4{\pm}6.3%$, p<0.001). The uptake of Group III ($27.7{\pm}12.0%$) was significantly lower than those of both Group I and Group II (p<0.001). $^{99m}Tc$-DMSA total renal uptakes correlated negatively with serum creatinine level (r=-0.629, p<0.001) and positively correlated with creatinine clearance rate (r=0.102, p<0.001). Conclusion: $^{99m}Tc$-DMSA total renal uptake of diabetic patients with microalbuminuria was significantly decreased compared with that of patients of normoalbuminuria. Therefore, $^{99m}Tc$-DMSA scan can be used as a diagnostic study for early detection of the diabetic nephropathy.

• The Korean Journal of Nuclear Medicine
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• v.33 no.4
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• pp.405-412
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• 1999

Purpose: It is important to evaluate the healing process of avascular necrosis (AVN) involving femoral head after treatment. The purpose of this study was to assess the usefulness of pinhole bone scintigraphy in the AVN of femoral head after surgery. Materials and Methods: We analyzed the changing pattern of pinhole bone scintigram in 21 femoral heads of 16 patients (14 lesions/11 male, 7 lesions/5 female, mean age: 39.4 yrs) before and after multiple drilling or vascularized bone grafting for AVN of the femoral head. In all patients, pre-operative scintigrams were obtained at 1 to 3 months before treatment and the first post-operative scintigrams were obtained at 1 to 3 months after treatment. All patients were followed for 2 to 4 years after operation. Results: The findings of the pinhole scintigrams were divided into three patterns: 1) curvilinear, 2) scattered spotty and 3) undetermined. The 10 of 11 lesions with curvilinear pattern had good postoperative clinical and radiological follow-up findings. However, all 6 lesions with scattered spotty pattern showed poor postoperative findings, which necessitated total hip joint replacement. Of the 4 lesions with undetermined pattern, 2 required total hip joint replacement. There was significant difference in postoperative prognosis between the curvilinear and scattered spotty patterns (p<0.05). Conclusion: We conclude that the pattern of pinhole bone scintigram obtained within 1 to 3 months after multiple drilling or vascularized bone graft operation is a useful prognostic indicator in the AVN of femoral head.

• The Korean Journal of Nuclear Medicine
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• v.34 no.4
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• pp.353-359
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• 2000

Purpose: Although ${\alpha}$-fetoprotein is one of the most commonly used tumor markers in Korea, most of the radioimmunoassay kits for ${\alpha}$-fetoprotein have been imported from foreign countries. The purpose of this study was to evaluate the performance of a recently developed domestic immunoradiometric kit for ${\alpha}$-fetoprotein (Riakey AFP IRMA $CT^R$, Sin-Jin Medics, Seoul, Korea). Materials and Methods: We evaluated intra- and inter-assay precision, recovery rate, parallelism, and sensitivity of serum ${\alpha}$-fetoprotein measurement using Riakey AFP IRMA $CT^R$ kit. The values of ${\alpha}$-fetoprotein measured by Riakey AFP IRMA $CT^R$ kit were compared with those measured by two foreign commercial kits (${\alpha}$-fetoproteina of Radim and ${\alpha}$-feto.riabead of Abbott). Results: Intra-assay coefficients of variation on three different levels were 5.3% for 18.9 ng/ml, 3.4% for 133 ng/ml and 1.6% for 330 ng/ml. Inter-assay coefficients of variation were 9.7% for 20.9 ng/ml, 3.2% for 137 ng/ml and 4.1% for 330 ng/ml respectively. Recovery rate tests on all three different levels showed within $100{\pm}10%$. Parallelism was also good and the sensitivity was 0.63 ng/ml. There was strong correlation between the measurement of ${\alpha}$-fetoprotein by Riakey AFP IRMA $CT^R$ and that by two foreign commercial kits(r=0.98). Conclusion: The first Korean domestic immunoradiometric kit for ${\alpha}$-fetoprotein, Riakey AFP IRMA $CT^R$, performed well for clinical use.

• Nuclear Medicine and Molecular Imaging
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• v.42 no.4
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• pp.267-274
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• 2008

Purpose: Transient neurological deterioration (TND) is one of the complications after extracranial-intracranial bypass surgery, and it has been assumed to be caused by postoperative transient hyperperfusion. This study was performed to evaluate the relationship between TND and preoperative and postoperative cerebral perfusion status on brain perfusion SPECT following superficial temporal artery - middle cerebral artery (STA-MCA) anastomosis surgery. Materials and Methods: A total of 60 STA-MCA anastomosis surgeries of 56 patients (mean age: $50{\pm}16$ yrs; M:F=29:27; atherosclerotic disease: 33, moyamoya disease: 27) which were done between September 2003 and July 2006 were enrolled. The resting cerebral perfusion and cerebral vascular reserve (CVR) after acetazolamide challenge were measured before and 10 days after surgery using 99mTc-ethylcysteinate dimer (ECD) SPECT. Moreover, the cerebral perfusion was measured on the third postoperative day. With the use of the statistical parametric mapping and probabilistic brain atlas, the counts for the middle cerebral artery (MCA) territory were calculated for each image, and statistical analyses were performed. Results: In 6 of 60 cases (10%), TND occurred after surgery. In all patients, the preoperative cerebral perfusion of affected MCA territory was significantly lower than that of contralateral side (p=0.002). The cerebral perfusion on the third and tenth day after surgery was significantly higher than preoperative cerebral perfusion (p=0.001, p=0.02). In TND patients, basal cerebral perfusion and CVR on preoperative SPECT were significantly lower than those of non-TND patients (p=0.01, p=0.05). Further, the increases in cerebral perfusion on the third day after surgery were significant higher than those in other patients (p=0.008). In patients with TND, the cerebral perfusion ratio of affected side to contralateral side on third postoperative day was significantly higher than that of other patients (p=0.002). However, there was no significant difference of the cerebral perfusion ratio on preoperative and tenth postoperative day between patients with TND and other patients. Conclusion: In patients with TND, relative and moderate hyperperfusion was observed in affected side after bypass surgery. These finding may help to understand the pathophysiology of TND.