• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.57-61
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• 2016

Isovaleric acidemia (IVA) is an autosomal recessively inherited organic acid disorder due to a defect of the enzyme isovaleryl-CoA dehydrogenase in the leucine metabolic pathway. Deficiency of this enzyme results in the accumulation of derivatives of isovaleryl-CoA. In acute illness in IVA, isovaleric acid and its derivatives accumulate and profound metabolic acidosis with ketosis, characteristic pungent body odor, hypoglycemia, and hyperammonemia can be developed. Additionally, recurrent vomiting, failure to thrive, developmental delay, epilepsy and mental retardation are chronic presenting symptoms and signs for IVA. On the result of newborn screening for inherited metabolic disorders, increased levels of isovalerylcarnitine (C5) are shown. However, C5 elevation can be accompanied with short/branched-chain acyl-CoA dehydrogenase (SBCAD) and therapy with certain antibiotics containing pivalic acid. Quantitative measurement of organic acids in urine and acylcarnitine profiles in plasma are necessary to differential diagnosis. Molecular genetic analysis of the IVD gene for IVA and ACADSB is also helpful to confirm IVA and SBCAD deficiency, respectively. Considering that IVA can be associated with significant morbidity and mortality at acute presentation of metabolic crisis, early diagnosis prior to the onset of symptoms by newborn screening enable to introduction of early treatment and prevention of acute and chronic complications.

• Korean Journal of Biological Psychiatry
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• v.3 no.1
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• pp.66-74
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• 1996

There has been an increase in head trauma due to rapid industralization and improvement in transportation. This poses difficulties in differentiating between neuropsychiatric disabilities resulting from real organic changes and those arising from compensation issues. It is the purpose of this study to seek out the differences between normal and abnormal finding group in the structural brain imaging studies via the results of the functional brain imaging studies and psychological tests. Out of 132 subjects, 62 comprised normal and 70 the abnormal finding group. EEG and SPECT were chosen for inspection of functional brain imaging. MMPI and K-WAIS were chosen for psychological test. The subjects were further divided into right hemispheric damage, left hemispheric damage, both hemispheric damage, diffuse damage group and negative group in order to find out whether any differences in the psychological lest results could be localized. The results are as follows : 1) The abnormal finding group, the EEG and SPECT were proven to be a good predictor of brain lesion. This implies that even in the functional brain studies, abnormalities are more easily detected if there are visible brain lesions. 2) The FSIQ of the abnormal finding group is lower than that of normal finding group. this difference is mainly due to low V1Q. The left hemispheric damage group lend to shaw low V1Q. This lowered in was the difference between left hemispheric damage group and negative group. Furthermore, there were no group differences in the PIQ. It is concluded that K-WAIS is effective as evaluator of VIQ mainly of those patients with left hemispheric damage and it is ineffective as a evaluator of PIQ. 3) In the MMPI profile, the both groups displayed high neurotic profiles. There was no difference in the psychotic profiles. The scores of the Depression and Hystery were high in abnormal finding group. This can be seen as one of the lypical findings of chronic head trauma patients. 4) The abnormal finding group tend to be diagnosed as organic mental disorder in the psychological tests more frequently.

• Anxiety and mood
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• v.7 no.1
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• pp.48-53
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• 2011

Objective : Korean Version of Beck-II Depression Inventory to verify the reliability and validity of the proposed standards are practical and standardized, cut-off score by establishing a baseline indicating the presence of depression and depression On in the evaluation was to evaluate the clinical usefulness. Methods : 739 patients with major depression using the SCID and normal controls were 302 study subjects. Of patients with clinically significant medical condition, or psychotic disorders, organic mental disorder, epilepsy or seizure disorder, eating disorders are associated with patients taking anti-convulsants experienced in the past, patients were excluded from the study. Results : The main findings of this study were as follows. First, with respect to the KBDI-II items, the correlation between them ranged from 0.51 to 0.74, and was 0.60 over all questions. Further, the overall correlation of the KBDI-II plates showing confidence 'normal' than it was verified that. Second, the BDIII was used in each group to examine internal consistency and thus, whether Cronbach's alpha values were greater than 0.94. Third, the principal component analysis sought to extract factors in a way consistent with the results inspected last 3 factors were extracted and the total variance explained was 47.3%. Fourth, the Cutting calculated the score on the KBDI-II for ROC (Receiver operator characteristic) analysis yielding 18 dot, with the highest sensitivity and specificity was seen. Conclusion : Based on the results of this Study, the KBDI-II cut-off point should be valid as prescribed in 18 is considered.