• Journal of the Korean Orthopaedic Association
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• v.54 no.2
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• pp.164-171
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• 2019

Purpose: A dedifferentiated chondrosarcoma is a rare lethal tumor characterized by a low grade chondrosarcoma juxtaposed with a high grade dedifferentiated sarcoma, such as osteosarcoma, fibrosarcoma. The aim of our study was to document the clinical manifestation and oncologic outcomes of a dedifferentiated chondrosarcoma. Materials and Methods: This study identified 11 patients who were diagnosed and treated for dedifferentiated chondrosarcoma between January 2007 and December 2016. The identified cohort was then reviewed regarding age, sex, symptom onset, tumor location, magnetic resonance imagings (MRIs), surgical margin, and pathologic diagnosis. The time to local recurrence and/or metastasis, follow-up duration, and the patients' final status were analyzed. Results: The patients were comprised of 7 males and 4 females with a mean age of 54 years (range, 33-80 years). The location of the tumor was in the femur in 6 cases, pelvis in 4 cases, and metatarsal in 1 case. The average tumor diameter was 12.7 cm (range, 6.0-26.1 cm). At the time of diagnosis, 2 patients showed pathologic fracture; 1 patient was Enecking stage IIA, 9 patients were stage IIB, and 1 patient was stage III. Eight patients were classified as a primary dedifferentiated chondrosarcoma and 3 patients were secondary. One of the primary lesions was misinterpreted initially as a low grade chondroid lesion by MRI and underwent curettage. Local recurrence occurred in 8 cases and distant metastasis occurred in 10 cases with a mean duration of 8 months (range, 2-23 months) and 7 months (range, 1-32 months), respectively. The three-year overall survival of patients with dedifferentiated chondrosarcoma was 18%, and 10 patients died due to disease progression. Conclusion: Dedifferentiated chondrosarcoma developed lung metastases in the early period of the clinical courses and the prognosis was dismal.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.1
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• pp.25-30
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• 2023

Leigh syndrome is a rare progressive neurodegenerative mitochondrial disorder with clinical and genetic heterogeneity. Recently, balletic IARS2 variants have been identified in a number of patients presenting broad clinical phenotypes from Leigh and West syndrome to a rare syndrome CAGSSS characterized by cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia syndrome (OMIM#616007). We describe a child with Korean Leigh syndrome with urologic manifestations resulting from a compound heterozygote mutation in IARS2. A 5-year-old girl visited the emergency room with a complaint of abdominal pain accompanied by abdominal distension. Abdominal-pelvic CT showed a markedly distended urinary bladder without definite obstructive lesions. She was diagnosed with neurogenic bladder dysfunction based on a urodynamic study. She had global delayed development due to neurologic regression after 6 months of age and a history of bilateral cataract surgery at the age of 2 years. Her brain magnetic resonance imaging showed symmetrically increased signal intensities in the bilateral putamen and caudate nuclei with diffuse cerebral atrophy. No gene variants were identified through whole-mitochondrial genome analysis. Whole exome sequencing was performed for diagnosis, and compound heterozygous pathogenic variants were identified in IARS2: c.2446C>T (p. Arg816Ter) and c.2450G>A (p. Arg817His). To the best of our knowledge, this is the first case report of bladder dysfunction manifestation in a patient with IARS2-related Leigh syndrome. Thus, it broadens the clinical and genetic spectrum of IARS2-associated diseases.

• Food Science and Preservation
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• v.31 no.1
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• pp.149-160
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• 2024

Dextran is a glucose homo-polysaccharide with a predominantly α-1,6 glycosidic linkage of microbial source and is known to be produced primarily by lactic acid bacteria. However, it can also be obtained through the dextran dextrinase of acetic acid bacteria (Gluconobacter oxydans). The dextrin-based dextran was obtained from rice starch using G. oxydans fermentation of rice hydrolysate, and its properties were studied. Both dextrin- and rice hydrolysate-added media maintained the OD value of 6 after 20 h of incubation with acetic acid bacteria, and the gel permeation chromatography (GPC) analysis of the supernatant after 72 h of incubation confirmed that a polymeric material with DP of 480 and 405, which was different from the composition of the substrate in the medium, was produced. The glucose linkage pattern of the polysaccharide was confirmed using the proton nuclear magnetic resonance (¹H-NMR) and the increased α-1,4:α-1,6 bond ratio from 0.23 and 0.13 to 1:2.37 and 1:4.4, respectively, indicating that the main bonds were converted to α-1,6 bonds. The treatment of dextrin with a rat-derived alpha-glucosidase digestive enzyme resulted in a slow release of glucose, suggesting that rice hydrolysate can be converted to dextran using acetic acid bacteria with glycosyltransferase activity to produce high-value bio-materials with slowly digestible properties.

• Journal of the Korean Society of Radiology
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• v.83 no.1
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• pp.102-111
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• 2022

Purpose To evaluate the correlation between Lee's grades and surgical intervention for central lumbar spinal stenosis (CLSS) and to assess whether this grading system can be used as a decision-making tool for the surgical treatment of this condition. Materials and Methods This retrospective study included 290 patients (M:F = 156:134; mean age, 46 ± 16 years). Radiologists assessed the presence and grade of CLSS at the stenosis point according to Lee's grading system, in which CLSS is classified into four grades according to the shape of the cauda equina. Correlation coefficients (r_s) between Lee's grades and the operation were calculated with Spearman rank correlation. Results Among the operated patients, grade 2 was the most commonly assigned grade (50%-58%), grade 3 was less common (35%), and grade 0 was the least common (2%-3%). Among the non-operated patients, grade 1 was the most common (63%-65%), grade 0 was less common (15%-16%), and grade 3 was the least common (8%). The distribution of grades differed between the operated and non-operated groups (p < 0.001). Less than 25% of patients who underwent surgery were assigned grades 0 and 1, and more than 88% were assigned grades 2 and 3. A moderate correlation was found between the grade and surgical intervention (r_s = 0.632 and r_s = 0.583). Conclusion Lee's grade was moderately correlated with surgical intervention. Lee's grading system can be a decision-making tool for the surgical treatment of CLSS.

• Journal of the Korean Society of Radiology
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• v.82 no.3
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• pp.562-574
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• 2021

MRI has the advantages of having excellent soft-tissue contrast and providing functional information without any harmful ionizing radiation. Although previous technical limitations restricted the use of chest MRI, recent technological advances and expansion of insurance coverage are increasing the demand for chest MRI. Recognizing the need for guidelines on appropriate use of chest MRI in Korean clinical settings, the Korean Society of Radiology has composed a development committee, working committee, and advisory committee to develop Korean chest MRI justification guidelines. Five key questions were selected and recommendations have been made with the evidence-based clinical imaging guideline adaptation methodology. Recommendations are as follows. Chest MRI can be considered in the following circumstances: for patients with incidentally found anterior mediastinal masses to exclude non-neoplastic conditions, for pneumoconiosis patients with lung masses to differentiate progressive massive fibrosis from lung cancer, and when invasion of the chest wall, vertebrae, diaphragm, or major vessels by malignant pleural mesothelioma or non-small cell lung cancer is suspected. Chest MRI without contrast enhancement or with minimal dose low-risk contrast media can be considered for pregnant women with suspected pulmonary embolism. Lastly, chest MRI is recommended for patients with pancoast tumors planned for radical surgery.

• Journal of the Korean Society of Radiology
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• v.83 no.5
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• pp.1059-1070
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• 2022

Purpose To compare the sensitivity of tumor detection and inter-observer agreement between acquired diffusion-weighted imaging (aDWI) b2000 and computed DWI (cDWI) b2000 in patients with prostate cancer (PCa). Materials and Methods Eighty-eight patients diagnosed with PCa by radical prostatectomy and having undergone pre-operative 3 Tesla-MRI, including DWI (b, 0, 100, 1000, 2000 s/mm²), were included in the study. cDWI b2000 was obtained from aDWI b0, b100, and b1000. Two independent reviewers performed a review of the aDWI b2000 and cDWI b2000 images in random order at 4-week intervals. A region of interest was drawn for the largest tumor on each dataset, and a Prostate Imaging-Reporting and Data System (PI-RADS) score based on PI-RADS v2.1 was recorded. Histologic topographic maps served as the reference standard. Results The study population's Gleason scores were 6 (n = 16), 7 (n = 53), 8 (n = 9), and 9 (n = 10). According to the reviewers, the sensitivities of cDWI b2000 and aDWI b2000 showed no significant differences (for reviewer 1, both 94% [83/88]; for reviewer 2, both 90% [79/88]; p = 1.000, respectively). The kappa values of cDWI b2000 and aDWI b2000 for the PI-RADS score were 0.422 (95% confidence interval [CI], 0.240-0.603) and 0.495 (95% CI, 0.308-0.683), respectively. Conclusion cDWI b2000 showed comparable sensitivity with aDWI b2000, in addition to sustained moderate inter-observer agreement, in the detection of PCa.

• Journal of the Korean Society of Radiology
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• v.81 no.3
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• pp.676-687
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• 2020

Purpose To analyze the altered brain regions and intrinsic brain activity patterns in trauma-exposed firefighters without posttraumatic stress disorder (PTSD). Materials and Methods Resting-state functional MRI (rsfMRI) was performed for all subjects. Thirty-one firefighters over 40 years of age without PTSD (31 men; mean age, 49.8 ± 4.7 years) were included. Twenty-six non-traumatized healthy controls (HCs) (26 men; mean age, 65.3 ± 7.84 years) were also included. Voxel-based morphometry was performed to investigate focal differences in the brain anatomy. Seed-based functional connectivity analysis was performed to investigate differences in spontaneous brain characteristics. Results The mean z-scores of the Seoul Verbal Learning Test for immediate and delayed recall, Controlled Oral Word Association Test (COWAT) score for animals, and COWAT phonemic fluency were significantly lower in the firefighter group than in the HCs, indicating decreased neurocognitive function. Compared to HCs, firefighters showed reduced gray matter volume in the left superior parietal gyrus and left inferior temporal gyrus. Further, in contrast to HCs, firefighters showed alterations in rsfMRI values in multiple regions, including the fusiform gyrus and cerebellum. Conclusion Structural and resting-state functional abnormalities in the brain may be useful imaging biomarkers for identifying alterations in trauma-exposed firefighters without PTSD.

• Journal of the Korean Society of Radiology
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• v.82 no.4
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• pp.862-875
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• 2021

Purpose To investigate the MRI features associated with neural foraminal stenosis (NFS) in patients older than 60 years with lumbar (L) radiculopathy. Materials and Methods This study included 133 retrospectively selected patients older than 60 years with lumbar radiculopathy who had undergone a lumbar spine MRI (from January 2018 to April 2018). For L4/L5 and L5/sacral (S)1 levels, NFS was reviewed blindly by two radiologists. Spondylolisthesis, retrolisthesis, disc height loss, disc bulging/herniation/central canal stenosis, ligamentum flavum thickening, and facet hypertrophy were evaluated separately for the NFS and non-NFS groups, and they were compared using univariate and multivariate analyses. Results The univariate analysis revealed that disc height loss (p = 0.006) was associated with NFS for L4/L5. For L5/S1, both spondylolisthesis (p = 0.005) and facet hypertrophy (p = 0.006) were associated with NFS. The multivariate logistic analysis revealed that disc height loss was associated with NFS for L4/L5 [odds ratio (OR) = 4.272; 95% confidence interval (CI) 1.736-10.514]. For L5/S1, spondylolisthesis (OR = 3.696; 95% CI 1.297-10.530) and facet hypertrophy (OR = 6.468; 95% CI 1.283-32.617) were associated with NFS. Conclusion Disc height loss was associated with NFS for L4/L5 and spondylolisthesis and facet hypertrophy were associated with NFS for L5/S1.

• Journal of the Korean Society of Radiology
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• v.82 no.3
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• pp.654-669
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• 2021

Purpose To evaluate the accuracy of MRI in predicting the pathological complete response (pCR) and the residual tumor size of breast cancer after neoadjucant chemotherapy (NAC), and to determine the factors affecting the accuarcy. Materials and Methods Eighty-eight breast cancer patients who underwent surgery after NAC at our center between 2010 and 2017 were included in this study. pCR was defined as the absence of invasive cancer on pathological evaluation. The maximum diameter of the residual tumor on post-NAC MRI was compared with the tumor size of the surgical specimen measured pathologically. Statistical analysis was performed to elucidate the factors affecting pCR and the residual tumor size-discrepancy between the MRI and the pathological measurements. Results The pCR rate was 10%. The diagnostic accuracy of MRI and the area under the curve for predicting pCR were 90.91% and 0.8017, respectively. The residual tumor sizes obtained using MRI and pathological measurements showed a strong correlation (r = 0.9, p < 0.001), especially in patients with a single mass lesion (p = 0.047). The size discrepancy between MRI and the pathological measurements was significantly greater in patients with the luminal type (p = 0.023) and multifocal tumors/non-mass enhancement on pre-NAC MRI (p = 0.047). Conclusion MRI is an accurate tool for evaluating pCR and residual tumor size in breast cancer patients who receive NAC. Tumor subtype and initial MRI features affect the accuracy of MRI.

• Journal of the Korean Society of Radiology
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• v.81 no.2
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• pp.365-378
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• 2020

Purpose This study was performed to determine whether the T1 relaxation time of gadoxetic acid-enhanced liver MR imaging is useful for detecting and staging liver fibrosis in patients with chronic liver disease. Materials and Methods One hundred and three patients with suspected focal liver lesion underwent MR imaging and Fibroscan. Fibroscan was chosen as the reference standard for classifying liver fibrosis. T1 relaxation times were acquired before (preT1), 20 minutes after (postT1) contrast administration, and reduction rate of T1 relaxation time (rrT1) on transverse 3D VIBE (volumetric interpolated breath-hold examination) sequence using 3T MR imaging. The optimal cut-off values for the fibrosis staging were determined with ROC analysis. Results PreT1 and postT1 increased and rrT1 decreased constantly with increasing severity of liver fibrosis according to the METAVIR score (F0-F4). There were statistically significant differences between F2 and F3 in preT1 (F2, 836.0 ± 74.7 ms; F3, 888.6 ± 77.5 ms, p < 0.05) and between F3 and F4 in postT1 (F3, 309.0 ± 80.2 ms; F4, 406.6 ± 147.7 ms, p < 0.05) and rrT1 (F3, 65.4 ± 7.7%; F4, 57.3 ± 11.4%, p < 0.05). ROC analysis revealed that combination test (preT1 + postT1) was the best test for predicting liver fibrosis. Conclusion PreT1 and postT1 increased constantly with increasing severity of liver fibrosis. T1 mapping in gadoxetic acid-enhanced liver MR imaging could be a helpful complementary sequence to determine the liver fibrosis stage.