• Journal of The Korean Society of Inherited Metabolic disease
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• v.12 no.1
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• pp.14-22
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• 2012

Objectives: This study was performed to review clinical manifestations of hyperphenylalaninemia patients in Korean. Methods: 178 cases of hyperphenyalaninemia were diagnosed at department of pediatrics, Soonchunhyang University Hospital from January, 1983 to August. We reviewed DNA analysis, MR imaging, EEG, radiography of the left hand and wrist, bone densitometry (BMD), IQ test of hyperphenyalaninemia patients. Results: Out of 178 cases, 161 cases were diagnosed classic phenylketonuria and 17 cases were diagnosed BH4 deficiency. 122 cases performed DNA analysis. R243Q (10.3%), Y204C (9.9%), and IVS4-1G>A (8.1%) mutations were predominant. 22 cases underwent MR imaging. Varying degrees of symmetrical high signal intensity were noted on T2-weighted sequences in the periventricular deep white matter of 15 cases. 23 cases were performed EEG. 12 cases (52.3%) showed abnormal pattern. EEG abnormalities showed in 11 cases. On lumbar BMD four of 11 cases (36%) showed reduced bone density of more than 1 S.D. in four of 11 cases, bone age was less than chronological age by at least one year. 18 cases were performed IQ test. Mean IQ scores was $84{\pm}21.6$. Among older than 15 years (9 cases), Mean IQ scores was $72{\pm}21.2$. PTPS deficiency was 14 cases, DHPR deficiency was 2 cases, and GTPCH deficiency was 1 case. Conclusion: We confirmed there were varieties of DNA mutations. And MR imaging and EEG were nonspecific in PKU patients. Older children showed lower IQ score. Low phenylalanine diet prevents brain damage in PKU patient. Not only first few years of life but also lifetime, Keeping low phenylalanine diet is important.

• Journal of radiological science and technology
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• v.28 no.2
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• pp.91-95
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• 2005

The cervical spine of anteroposterior oblique view is essential to observe the intervertebral foramen(IVF). The morphologic changes of IVFs were proved to be abnormal with nerve roots and peripheral structures. The purpose of this study is to evaluate the effective projection angle for observing the IVFs in the Korean adults. In a prospective clinical study of 100 normal persons, $45^{\circ}$, $50^{\circ}$ and $55^{\circ}$ oblique views were compared by measuring the maximal transverse diameter of all the cervical IVFs. $45^{\circ}$ oblique views provided slightly better visualization of upper cervical level(C2-C3, C3-C4, C4-C5), but the lower cervical level(C5-C6, C6-C7, C7-T1) of IVF transverse diameters were substantially increased on the $55^{\circ}$ AP oblique projection. In the comprasion of mean differences between 8 obese person(BMI > 25) and 58 normal person(18.5 < BMI < 22.9) proved to be statistically not significant. Consequently this study shows that $55^{\circ}$AP oblique(tube angle $15^{\circ}$cephalad) view is optimal for evaluating the lower cervical IVFs.

• Korean Journal of Psychosomatic Medicine
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• v.25 no.2
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• pp.153-165
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• 2017

Objectives : Recent neuroimaging studies focus on dysfunctions in connectivity between cognitive circuits and emotional circuits: anterior cingulate cortex that connects dorsolateral orbitofrontal cortex and prefrontal cortex to limbic system. Previous studies on pediatric depression using DTI have reported decreased neural connectivity in several brain regions, including the amygdala, anterior cingulate cortex, superior longitudinal fasciculus. We compared the neural connectivity of psychotropic drug naïve adolescent patients with a first onset of major depressive episode with healthy controls using DTI. Methods : Adolescent psychotropic drug naïve patients(n=26, 10 men, 16 women; age range, 13-18 years) who visited the Korea University Guro Hospital and were diagnosed with first onset major depressive disorder were registered. Healthy controls(n=27, 5 males, 22 females; age range, 12-17 years) were recruited. Psychiatric interviews, complete psychometrics including IQ and HAM-D, MRI including diffusion weighted image acquisition were conducted prior to antidepressant administration to the patients. Fractional anisotropy(FA), radial, mean, and axial diffusivity were estimated using DTI. FMRIB Software Library-Tract Based Spatial Statistics was used for statistical analysis. Results : We did not observe any significant difference in whole brain analysis. However, ROI analysis on right superior longitudinal fasciculus resulted in 3 clusters with significant decrease of FA in patients group. Conclusions : The patients with adolescent major depressive disorder showed statistically significant FA decrease in the DTI-based structure compared with healthy control. Therefore we suppose DTI can be used as a bio-marker in psychotropic drug-naïve adolescent patients with first onset major depressive disorder.

• Journal of Radiation Protection and Research
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• v.32 no.3
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• pp.105-110
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• 2007

Purpose : In spite of recent remarkable improvement of diagnostic imaging modalities such as CT, MRI, and PET and radiation therapy planing systems, ICR plan of uterine cervix cancer, based on recommendation of ICRU38(2D film-based) such as Point A, is still used widely. A 3-dimensional ICR plan based on CT image provides dose-volume histogram(DVH) information of the tumor and normal tissue. In this study, we compared tumor-dose, rectal-dose and bladder-dose through an analysis of DVH between CTV plan and ICRU38 plan based on CT image. Method and Material : We analyzed 11 patients with a cervix cancer who received the ICR of Ir-192 HDR. After 40Gy of external beam radiation therapy, ICR plan was established using PLATO(Nucletron) v.14.2 planing system. CT scan was done to all the patients using CT-simulator(Ultra Z, Philips). We contoured CTV, rectum and bladder on the CT image and established CTV plan which delivers the 100% dose to CTV and ICRU plan which delivers the 100% dose to the point A. Result : The volume$(average{\pm}SD)$ of CTV, rectum and bladder in all of 11 patients is $21.8{\pm}6.6cm^3,\;60.9{\pm}25.0cm^3,\;111.6{\pm}40.1cm^3$ respectively. The volume covered by 100% isodose curve is $126.7{\pm}18.9cm^3$ in ICRU plan and $98.2{\pm}74.5cm^3$ in CTV plan(p=0.0001), respectively. In (On) ICRU planning, $22.0cm^3$ of CTV volume was not covered by 100% isodose curve in one patient whose residual tumor size is greater than 4cm, while more than 100% dose was irradiated unnecessarily to the normal organ of $62.2{\pm}4.8cm^3$ other than the tumor in the remaining 10 patients with a residual tumor less than 4cm in size. Bladder dose recommended by ICRU 38 was $90.1{\pm}21.3%$ and $68.7{\pm}26.6%$ in ICRU plan and in CTV plan respectively(p=0.001) while rectal dose recommended by ICRU 38 was $86.4{\pm}18.3%$ and $76.9{\pm}15.6%$ in ICRU plan and in CTV plan, respectively(p=0.08). Bladder and rectum maximum dose was $137.2{\pm}50.1%,\;101.1{\pm}41.8%$ in ICRU plan and $107.6{\pm}47.9%,\;86.9{\pm}30.8%$ in CTV plan, respectively. Therefore, the radiation dose to normal organ was lower in CTV plan than in ICRU plan. But the normal tissue dose was remarkably higher than a recommended dose in CTV plan in one patient whose residual tumor size was greater than 4cm. The volume of rectum receiving more than 80% isodose (V80rec) was $1.8{\pm}2.4cm^3$ in ICRU plan and $0.7{\pm}1.0cm^3$ in CTV plan(p=0.02). The volume of bladder receiving more than 80% isodose(V80bla) was $12.2{\pm}8.9cm^3$ in ICRU plan and $3.5{\pm}4.1cm^3$ in CTV plan(p=0.005). According to these parameters, CTV plan could also save more normal tissue compared to ICRU38 plan. Conclusion : An unnecessary excessive radiation dose is irradiated to normal tissues within 100% isodose area in the traditional ICRU plan in case of a small size of cervix cancer, but if we use CTV plan based on CT image, the normal tissue dose could be reduced remarkably without a compromise of tumor dose. However, in a large tumor case, we need more research on an effective 3D-planing to reduce the normal tissue dose.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.109-114
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• 2016

Carbamoyl phosphate synthetase 1 (CPS1) deficiency is an autosomal recessive urea cycle disorder which causes hyperammonemia. CPS1 is the first enzyme step in the urea cycle and almost patients present their symptoms during neonatal period. We report a case of CPS1 deficiency in a boy who developed symptoms including lethargy and seizure at 3 days of age. The ammonia level was up to $2,325{\mu}mol/L$, sodium benzoate (250 mg/kg/d) and high calories of both dextrose and lipid was promptly administered. Central access by experienced pediatric surgeon and emergent continuous hemodialysis by pediatric nephrologist was performed within 3 hours and ammonia was less than $100{\mu}mol/L$ at 5 days of age. Currently, he has showed excellent response to treatments including scavenging drugs and a low-protein diet. Despite of diffuse increasing signal intensity on cerebral white matters and basal ganglia on brain MRI, his development and weight gain were good at the last follow-up at 11 months of age. Molecular assay of the CPS1 gene demonstrated that patient had compound heterozygous for c.1529del ($p.Gly510Alafs^*5$) in exon 14 and c.3142-1G>C (IVS25(-1)G>C) in intron 25 and exon 26 boundary. The splicing mutation was novel mutation and inherited from patient's mother. Here, we report a neonatal lethal type CPS1 deficiency patient having novel mutation.

• Investigative Magnetic Resonance Imaging
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• v.13 no.2
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• pp.137-145
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• 2009

Purpose : To compare 12 and 32-element surface coil arrays for highly accelerated coronary magnetic resonance angiography (MRA) using parallel imaging. Materials and Methods : Steady state free precession coronary MRA was performed in 5 healthy volunteers at 1.5 T whole body MR scanner using both 12 and 32-element surface coil arrays. Left anterior descending and right coronary artery data sets were acquired for each volunteer. Data sets were sub-sampled for parallel imaging using reduction factors from 1 to 6. Mean geometry factor (g-factor), maximum g-factor, and artifact level were calculated for each of the two coil arrays. Results : Over all reduction factors, the mean and maximum g-factors and artifact level were significantly reduced using the 32-element array compared to the 12element array (P << 0.1). The mean g-factor was sensitive to the imaging orientations of coronary arteries while the maximum g-factor and artifact level were independent of orientation. Conclusion : The 32-element surface coil array significantly improves artifact and noise suppression for highly accelerated coronary MRA using parallel imaging. The increased acceleration factors made feasible with the 32-element array offer the potential to enhance spatial resolution or increase volumetric coverage for 3D coronary MRA.

• Journal of KIISE:Software and Applications
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• v.37 no.2
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• pp.110-119
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• 2010

In this paper, we propose an automatic segmentation of the prostate using intensity distribution and gradient information in MR images. First, active shape model using adaptive intensity profile and multi-resolution technique is used to extract the prostate surface. Second, hole elimination using geometric information is performed to prevent the hole from occurring by converging the surface shape to the local optima. Third, the surface shape with large anatomical variation is corrected by using 2D gradient information. In this case, the corrected surface shape is often represented as rugged shape which is generated by the limited number of vertices. Thus, it is reconstructed by using surface modelling and smoothing. To evaluate our method, we performed the visual inspection, accuracy measures and processing time. For accuracy evaluation, the average distance difference and the overlapping volume ratio between automatic segmentation and manual segmentation by two radiologists are calculated. Experimental results show that the average distance difference was 0.3${\pm}$0.21mm and the overlapping volume ratio was 96.31${\pm}$2.71%. The total processing time of twenty patient data was 16 seconds on average.

• The KIPS Transactions:PartA
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• v.13A no.5 s.102
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• pp.465-472
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• 2006

Recently, many suggestions have been made in image segmentation methods for extracting human organs or disease affected area from huge amounts of medical image datasets. However, images from some areas, such as brain, which have multiple structures with ambiruous structural borders, have limitations in their structural segmentation. To address this problem, clustering technique which classifies voxels into finite number of clusters is often employed. This, however, has its drawback, the influence from noise, which is caused from voxel by voxel operations. Therefore, applying image enhancing method to minimize the influence from noise and to make clearer image borders would allow more robust structural segmentation. This research proposes an efficient structural segmentation method by filtering based clustering to extract detail structures such as white matter, gray matter and cerebrospinal fluid from brain MR. First, coherence enhancing diffusion filtering is adopted to make clearer borders between structures and to reduce the noises in them. To the enhanced images from this process, fuzzy c-means clustering method was applied, conducting structural segmentation by assigning corresponding cluster index to the structure containing each voxel. The suggested structural segmentation method, in comparison with existing ones with clustering using Gaussian or general anisotropic diffusion filtering, showed enhanced accuracy which was determined by how much it agreed with the manual segmentation results. Moreover, by suggesting fine segmentation method on the border area with reproducible results and minimized manual task, it provides efficient diagnostic support for morphological abnormalities in brain.

• Journal of Biomedical Engineering Research
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• v.25 no.5
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• pp.323-328
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• 2004

The development of group-specific tissue probability maps (TPM) provides a priori knowledge for better result of cerebral tissue classification with regard to the inter-ethnic differences of inter-subject variability. We present sequential procedures of group-specific TPM and evaluate the age effects in the structural differences of TPM. We investigated 100 healthy volunteers with high resolution MRI scalming. The subjects were classified into young (60, 25.92+4.58) and old groups (40, 58.83${\pm}$8.10) according to the age. To avoid any bias from random selected single subject and improve registration robustness, average atlas as target for TPM was constructed from skull-stripped whole data using linear and nonlinear registration of AIR. Each subject was segmented into binary images of gray matter, white matter, and cerebrospinal fluid using fuzzy clustering and normalized into the space of average atlas. The probability images were the means of these binary images, and contained values in the range of zero to one. A TPM of a given tissue is a spatial probability distribution representing a certain subject population. In the spatial distribution of tissue probability according to the threshold of probability, the old group exhibited enlarged ventricles and overall GM atrophy as age-specific changes, compared to the young group. Our results are generally consistent with the few published studies on age differences in the brain morphology. The more similar the morphology of the subject is to the average of the population represented by the TPM, the better the entire classification procedure should work. Therefore, we suggest that group-specific TPM should be used as a priori information for the cerebral tissue classification.

• The Korean Journal of Nuclear Medicine
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• v.39 no.6
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• pp.413-420
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• 2005

Purpose: Neuroreceptor PET studies require 60-120 minutes to complete and head motion of the subject during the PET scan increases the uncertainty in measured activity. In this study, we investigated the effects of the data-driven head mutton correction on the evaluation of endogenous dopamine release (DAR) in the striatum during the motor task which might have caused significant head motion artifact. Materials and Methods: $[^{11}C]raclopride$ PET scans on 4 normal volunteers acquired with bolus plus constant infusion protocol were retrospectively analyzed. Following the 50 min resting period, the participants played a video game with a monetary reward for 40 min. Dynamic frames acquired during the equilibrium condition (pre-task: 30-50 min, task: 70-90 min, post-task: 110-120 min) were realigned to the first frame in pre-task condition. Intra-condition registrations between the frames were performed, and average image for each condition was created and registered to the pre-task image (inter-condition registration). Pre-task PET image was then co-registered to own MRI of each participant and transformation parameters were reapplied to the others. Volumes of interest (VOI) for dorsal putamen (PU) and caudate (CA), ventral striatum (VS), and cerebellum were defined on the MRI. Binding potential (BP) was measured and DAR was calculated as the percent change of BP during and after the task. SPM analyses on the BP parametric images were also performed to explore the regional difference in the effects of head motion on BP and DAR estimation. Results: Changes in position and orientation of the striatum during the PET scans were observed before the head motion correction. BP values at pre-task condition were not changed significantly after the intra-condition registration. However, the BP values during and after the task and DAR were significantly changed after the correction. SPM analysis also showed that the extent and significance of the BP differences were significantly changed by the head motion correction and such changes were prominent in periphery of the striatum. Conclusion: The results suggest that misalignment of MRI-based VOI and the striatum in PET images and incorrect DAR estimation due to the head motion during the PET activation study were significant, but could be remedied by the data-driven head motion correction.