• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.12 no.1
• /
• pp.49-53
• /
• 2012

X-linked adrenoleukodystrophy (ALD) is a rare inherited metabolic disease which results in impaired peroxisomal ${\beta}$-oxidation and the accumulation of very long chain fatty acids (VLCFA) in the adrenal cortex, the myelin of the central nervous system, and the testes. X-linked ALD is caused by mutations in the ABCD1 gene encoding an ATP-binding cassette transporter superfamily located in the peroxisomal membrane. This disease is characterized by a variety of phenotypes. The classic childhood cerebral ALD is a rapidly progressive demyelinating condition affecting the cerebral white matter before the age of 10 years in boys. We report the case of a 8-year-old with childhood cerebral X-linked ALD who developed inattention, hyperactivity, motor incoordination and hemiparesis. We diagnosed ALD with elevated plasma very long chain fatty acid level and diffuse high signal intensity lesions in both parieto-occipital white matter and cerebellar white matter in brain MRI. We identified a novel c.983delT (p.Met329CysfsX7) mutation of the ABCD1 gene. There is no correlation between X-ALD phenotype and mutations in the ABCD1 gene. Further studies for searching additional non-genetic factor which determine the phenotypic variation will be needed.

• Journal of radiological science and technology
• /
• v.38 no.4
• /
• pp.389-394
• /
• 2015

There is a progressive development in the medical imaging technology, especially of descriptive capability for anatomical structure of human body thanks to advancement of information technology and medical devices. But however maintenance of correct posture is essential for the medical imaging checkup on the shoulder joint requiring rotation of the upper limb due to the complexity of human body. In the cases of MRI examination, long duration and fixed posture are critical, as failure to comply with them leads to minimal possibility of reproducibility only with the efforts of the examiner and will of the patient. Thus, this study aimed to develop an auxiliary device that enables rotation of the upper limb as well as fixing it at quantitative angles for medical imaging examination capable of providing diagnostic values. An auxiliary device has been developed based on the results of precedent studies, by designing a 3D model with the CATIA software, an engineering application, and producing it with the 3D printer. The printer is Objet350 Connex from Stratasys, and acrylonitrile- butadiene-styrene(ABS) is used as the material of the device. Dimensions are $120{\times}150{\times}190mm$, with the inner diameter of the handle being 125.9 mm. The auxiliary device has 4 components including the body (outside), handle (inside), fixture terminal and the connection part. The body and handle have the gap of 2.1 mm for smooth rotation, while the 360 degree of scales have been etched on the handle so that the angle required for observation may be recorded per patient for traceability and dual examination.

• Journal of the Korean Society of Radiology
• /
• v.6 no.1
• /
• pp.19-26
• /
• 2012

It is very important for early diagnosis and therapy with ischamic cerebral infarction patients. This study was to know the ischemic penumbra lesion which compared CT-perfusion and diffusion weighted MRI(DWMRI) with acute cerebral infarction patients. 12 acute cerebral infarction patients had performed perfusion CT and performed DWMRI. Perfusion images including cerebral blood volume(CBV), cerebral blood flow(CBF), time to peak(TTP) and mean transit time(MTT) maps obtained the values with defect lesion and contralateral normal cerebral hemisphere and DWMRI was measured by signal intensity and compared of lesion size between each perfusion map. All perfusion CT maps showed the perfusion defect lesions in all patients. There were remarkable TTP and MTT delay in perfusion defect lesions. The lesions on CBF map was the most closely correlated with the lesions on DWMRI. The size of perfusion defect lesions on TTP and MTT map was larger than that of lesions on DWMRI, suggesting that MTT map can evaluate the ischemic penumbra. Perfusion CT maps make it possible to evaluate not only ischemic core and ischemic penumbra, but also hemodynamic status in the perfusion defect area. These results demonstrate that perfusion CT can be useful to the diagnosis and treatment in the patients with acute cerebral ischemic infarction.

• Journal of Chest Surgery
• /
• v.40 no.8
• /
• pp.574-577
• /
• 2007

Primary malignant neoplasm of the pericardium is very rare, Neoplastic involvement of the pericardium may result in rapidly developing hemorrhagic effusion. A 30-year-old male who occasionally suffered from chest tightness was referred to our hospital under the diagnosis of unstable angina. He presented with acute chest pain and severe dyspnea that had developed one day previously. The diagnostic investigations such as echocardiography, chest CT and magnetic resonance image suggested cardiac tamponade that was caused by rupture of the pericardial teratoma. An operation to remove the tumor and effusion was performed. The pericardial mass was completely excised, and the result of the frozen biopsy favored malignancy. The final pathologic report was malignant fibrosarcoma of the pericardium and no malignant cells were found on the cytology of the pericardial effusion. The patient had a smooth postoperative course and was referred to another hospital for additional radiation therapy. We report here on this case of cardiac tamponade that was caused by primary pericardial fibrosarcoma, and this required urgent diagnosis and surgical management.

• Clinics in Shoulder and Elbow
• /
• v.16 no.2
• /
• pp.135-140
• /
• 2013

We report a case of recurrent shoulder dislocation and combined proximal humerus fracture in neurofibromatosis type I. A twenty-year-old male patient with known neurofibromatosis type I presented with right shoulder dislocation and proximal humerus fracture following a minor trauma. His injured arm also had large plexiform neurofibromas on the elbow, and the dislocation of the shoulder joint was proven to be recurrent. Bony deformation of the humerus and scapula were revealed on X-ray and computed tomography, as well as multiple neurofibromatic changes of the deltoid and periscapular muscles were noted on magnetic resonance imaging. Open reduction and capsular reconstruction were performed and humeral fracture was stabilized with a reconstruction plate. Satisfactory union and functional result were achieved and the dislocation did not recur until the 7-month followup after the procedure. Recurrent shoulder dislocation and combined proximal humerus fracture in neurofibromatosis type I are rare complications. However, it should still be considered in the orthopaedic evaluation process of the diseases.

• Journal of Oral Medicine and Pain
• /
• v.33 no.4
• /
• pp.387-394
• /
• 2008

Purpose : The purpose of this study was to verify the usefulness and feasibility of ultrasonographic imaging for the detection of the disorders of the surrounding supporting structure such as articular capsule, retrodiscal tissue and related ligaments, osteoarthritic evidence and associated disc displacement at the temporomandibular joint(TMJ) Materials and Methods : 20 patients(40 joints) with periodic lock and crepitations were investigated prospectively using 12 MHz array transducer. Ultrasonographic Imagings were assessed for osteoarthritic surface changes of condyle, extent of disc displacement and disorders of surrounding structures. Ultrasonographic images were compared with clinical investigations, conventional radiography and Dental Computed Tomographic scans. Results : In clinical and conventional radiography, osteoarthritic changes were diagnosed in 8 joints. Ultrasonographically 7 of the 8 osteoarthritic changes were diagnosed correctly. Sensitivity, specificity, and accuracy of ultrasonography in the osteoarthritic detections were 87.5%, 62.5%, and 67.5% respectively. About the detection of disc displacement, disc displacement were diagnosed in 21 joints clinically. Ultrasonographically 19 of the 21 disc displacements were diagnosed correctly. Sensitivity, specificity, and accuracy of ultrasonography in the osteoarthritic detections were 95%, 90%, and 92.5% respectively. when the disorders of supportive structure were figured out, the disorders of supportive structure were diagnosed in 18 joints clinically. Ultrasonographically 1 of the 18 the disorders of supportive structure were diagnosed correctly. Sensitivity, specificity, and accuracy of ultrasonography in the osteoarthritic detections were 5.5%, 4.5%, and 55% respectively. Conclusion : Ultrasonography is an relatively reliable diagnostic tool for the detection of disc displacement and some of osteoarthritic changes. But it's not an insufficient imaging technique for the detection of the disorders of the surrounding structure.

• Journal of the Korean Chemical Society
• /
• v.33 no.5
• /
• pp.484-495
• /
• 1989

Reactions of $(Et_4N)_2[MoOCl_5]$ with multidentate ligands containing nitrogen or/and oxygen donor atom (EDTA, DTPA, IDA, CyDTA, OX) produce a series of binuclear molybdate (V) complexes. The prepared Mo (V) complexes has been identified by Elemental Analysis, Infrared Spectra, Proton Magnetic Resonance Spectra, and Electronic Spectra. The electrochemical reduction mechanism has been studied by Cyclic voltammetry, Controlled Potential Coulometry, and Spectrophotometry in pH 3.571-10.375 acetate, borate, phosphate/sodium hydroxide, phosphate, ammonium/ammonia buffers. The cyclic voltammogram of the Mo-EDTA, DTPA, IDA, CyDTA complexes at pH < ca. 6.00 have shown two reduction waves. The first reduction wave shows two electron process and the second reduction wave shows two electron process. The cyclic voltammogram of the Mo-EDTA, DTPA, IDA, CyDTA complexes at pH < ca. 8.00 has shown one reduction wave. This reduction wave show four electron process. The cyclic voltammogram of the Mo-OX complex at pH < ca. 7.2 has shown one reduction wave. This reduction wave show four electron process.

• Journal of Veterinary Clinics
• /
• v.26 no.3
• /
• pp.273-275
• /
• 2009

A 6-year-old castrated male Shih-tzu was presented because of a 1-month history of progressive tetraplegia. On the basis of the findings of neurological examination, radiographs and magnetic resonance imaging, herniated intervertebral disc of C4-C5, C5-C6 and C6-C7 compressed spinal cord. The oxygen-ozone was intra-operatively injected into the C4-C5 and C6-C7 intervertebral disc and a ventral decompression performed at C5-C6 intervertebral space. Immediately after the surgery, neck pain was disappeared and three weeks after surgery, there was complete resolution of the neurological deficits with normal gaits. On magnetic resonance images at 2-month after surgery, herniated disc materials of C5-C6 and C6-C7 were completely removed and that of C4-C5 remarkably reduced. Consequently decompression which was performed at all of the three lesions resulted in good clinical outcome without additional internal fixation for minimizing postoperative instability.

• Radiation Oncology Journal
• /
• v.18 no.4
• /
• pp.221-225
• /
• 2000

Purpose : This study was designed to examine the reliability of the new version of the AJCC staging system (1997) of nasopharyngeal carcinoma in comparison with the AJCC staging system of 1992. Materials and Methods :Between 1983 and 1996, 185 patients with histologically proven nasopnaryngeal carcinoma were treated with radiation therapy at the Department of Therapeutic Radiology Seoul National University Hospital. For these patients, AJCC staging system of 1992 was compared with the 1997 version by reviewing hospital records, computed tomography (CT) and/or magnetic resonance imaging (MRI). Results :5-year overall suwival rates according to the 1992 and 1997 AJCC staging systems were 100$\%$, and 100$\%$ at stage 1: 100$\%$, and 68.8$\%$ at stage 11; 61.4$\%$, and 63.8$\%$ at stage 111; 61.1$\%$, and 63.2$\%$ at stage IV. S-year overall survival rates of each classification showed significant differences between stages (p=0.0049 for the old version, p=0.01 for the new), but no significant difference was found between the staging systems except at stage 11. Conclusion : The new AJCC staging system allows staging as reliably as the 1992 version, but the adequacy of the newly modified staging classification should be confirmed by further clinical examination.

• Journal of Gifted/Talented Education
• /
• v.20 no.2
• /
• pp.503-526
• /
• 2010

We investigated the brain activity in perceiving the emotional stimuli between a science invention group(n=13) and a general group(n=13). The science invention group, which mostly consisted of recipients of creativity prizes, scored 96% on creative personality tests(WKOPAY, SAM), whereas the general group performed at an average level on these tests. Analyzing the brain activity in perceiving the emotional stimuli(IAPS pictures), the science invention group than the general group showed higher activity in certain areas, such as MTG, STG, and so on. When correlation analysis was performed on the creative personality score and brain activity, MTG, STG, and so on areas showed significant correlations. There were more correlation areas in valence than in arousal. These results show that scientific creativity is related to emotional susceptibility. Thus, we insist that emotion be considered in the assessment and education programs for the gifted in science.