1.Three Cases of Congenital Adrenal Hyperplasia Owing to Multiple Mutations of CYP21 Gene.
Jun HEO ; Jae Hong YU ; Dong Ki JEONG
Journal of Korean Society of Pediatric Endocrinology 2001;6(2):176-181
Congenital adrenal hyperplasia(CAH) results from an inherited defect in enzymatic steps required to synthesize cortisol from cholesterol. 21-hydroxylase deficiency accounts for 95% cases of CAH. We have analyzed CYP21 genes of CAH by PCR direct sequencing. Our results shows three cases of CAH owing to multiple mutations of CYP21 gene; first case, IVS2AS, A/G, -13, Ile172Asn; second case, IVS2AS, A/G, -13, Ile236Asn, Val237Glu, Met239Lys; third case, Ile172Asn, C to G at 1590nt, Val281Leu, Arg484Pro, G to A at 2697nt. Mutations such as Ile236Asn, Val237Glu, Met239Lys, and Arg484Pro are first noted in Korea.
Adrenal Hyperplasia, Congenital*
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Cholesterol
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Hydrocortisone
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Korea
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Polymerase Chain Reaction
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Steroid 21-Hydroxylase
2.A case of retroauricular neurothekeoma.
Kil Yang JEONG ; Tong Suk CHUN ; Ki Hwan HONG
Korean Journal of Otolaryngology - Head and Neck Surgery 1992;35(1):139-142
No abstract available.
Neurothekeoma*
3.Cases of the surgical correction of facial asymmetry
Hong Yell HUH ; Sung Ki MIN ; Sang Ki CHO ; In Won JEONG
Journal of the Korean Association of Maxillofacial Plastic and Reconstructive Surgeons 1991;13(2):191-198
No abstract available.
Facial Asymmetry
4.A case of nocardiosis.
Jeong Hee KIM ; Ki Heon YOON ; Jee Hong YOO ; Hong Mo KANG ; Jin Tae SUH
Tuberculosis and Respiratory Diseases 1992;39(4):355-360
No abstract available.
Nocardia Infections*
5.Antiarrhythmic Effect of Artemisinin in an Ex-vivo Model of Brugada Syndrome Induced by NS5806
Hyung Ki JEONG ; Seo Na HONG ; Namsik YOON ; Ki Hong LEE ; Hyung Wook PARK ; Jeong Gwan CHO
Korean Circulation Journal 2023;53(4):239-250
Background and Objectives:
Brugada syndrome (BrS) is an inherited arrhythmia syndrome that presents as sudden cardiac death (SCD) without structural heart disease. One of the mechanisms of SCD has been suggested to be related to the uneven dispersion of transient outward potassium current (Ito ) channels between the epicardium and endocardium, thus inducing ventricular tachyarrhythmia. Artemisinin is widely used as an antimalarial drug. Its antiarrhythmic effect, which includes suppression of Ito channels, has been previously reported. We investigated the effect of artemisinin on the suppression of electrocardiographic manifestations in a canine experimental model of BrS.
Methods:
Transmural pseudo-electrocardiograms and epicardial/endocardial transmembrane action potentials (APs) were recorded from coronary-perfused canine right ventricular wedge preparations (n=8). To mimic the BrS phenotypes, acetylcholine (3 μM), calcium channel blocker verapamil (1 μM), and Ito agonist NS5806 (6–10 μM) were used.Artemisinin (100–150 μM) was then perfused to ameliorate the ventricular tachyarrhythmia in the BrS models.
Results:
The provocation agents induced prominent J waves in all the models on the pseudoelectrocardiograms. The epicardial AP dome was attenuated. Ventricular tachyarrhythmia was induced in six out of 8 preparations. Artemisinin suppressed ventricular tachyarrhythmia in all 6 of these preparations and recovered the AP dome of the right ventricular epicardium in all preparations (n=8). J wave areas and epicardial notch indexes were also significantly decreased after artemisinin perfusion.
Conclusions
Our findings suggest that artemisinin has an antiarrhythmic effect on wedge preparation models of BrS. It might work by inhibition of potassium channels including Ito channels, subsequently suppressing ventricular tachycardia/ventricular fibrillation.
6.A Case of Congenital Hemangiopericytoma of Small Bowel Associated with Intestinal Obstruction.
Ki Min KIM ; Seong Hee PARK ; Eun Jeong KIM ; Ki Hong PARK ; Byung Heon KIM ; Seong Ryul RYU
Journal of the Korean Society of Neonatology 1997;4(2):276-279
Hemangiopericytoma was first described by Stout & Murray in 1942 and was an uncommon soft tissue tumor thought to be derived from vascular pericytes. Approximately 10-15% of the cases occur in children have a propensity to develop in the region of head, neck and lower extremities. We experienced a case of congenital hemangiopericytoma of small bowel in a male neonate who showed vomiting and abdorninal distension. We reported a case of congenital hemangiopericytoma of small bowel with brief review of related literature.
Child
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Head
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Hemangiopericytoma*
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Humans
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Infant, Newborn
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Intestinal Obstruction*
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Intestine, Small
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Lower Extremity
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Male
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Neck
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Pericytes
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Vomiting
7.Value of the Left Portal Vein Angle (LPVA) on CT for the Diagnosis of Liver Cirrhosis: Comparison with the Caudateto Right Lobe (C/RL) Ratio.
Byung Ihn CHOI ; Joon Koo HAN ; Kyung Mo YEON ; Yoong Ki JEONG ; Hong Dae KIM
Journal of the Korean Radiological Society 1995;32(5):737-742
PURPOSE: To verify the usefulness of left portal vein angle (LPVA) on CT scan in the diagnosis of liver cirrhosis and to compare its diagnostic value with that of caudate to right lobe ratio (C/RL ratio). MATERIALS AND METHODS: LPVA, an angle formed by a vertical line and a line connecting the center of the vertebral body to the umbilical point of the left portal vein, and C/RL ratio were measured on CT scans of 100 cirrhotic and 100 normal livers. Diagnostic values of LPVA and C/RL ratio were compared statistically. RESULTS: The mean of LPVA was 18.9 degrees(SD; 7.6) for normal livers and 25.8 degreesSD; 8.4) for cirrhotic livers (P<0.001). The mean of C/RL ratio was 0.47(SD; 1.10) for normal livers and 0.58(SD;0.14) for cirrhotic livers (P<0.001). When LPVA was greater than 30 degreesliver cirrhosis was diagnosed with 36% sensitivity and 92% specificity. When C/RL ratio was greater than 0.60, the diagnose of liver cirrhosis was with 41% sensitivity and 90% specificity. There was no significant difference of the diagnostic accuracy between LPVA and C/R L ratio in ROC analysis. CONCLUSION: Both LPVA and C/RL ratio are useful diagnostic indices of liver cirrhosis on CTscan. LPVA is more convenient to measure than C/RL ratio.
Diagnosis*
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Fibrosis
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Liver Cirrhosis*
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Liver*
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Portal Vein*
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ROC Curve
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Sensitivity and Specificity
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Tomography, X-Ray Computed
8.Effect of Sulfhydryl Compounds on Melanosomal Morphology of Epidermal Melanocytes in UV - Irradiated Black Mice.
Kyung Won HAN ; Ki Bum MYUNG ; Jeong Hee HAHM ; Hong Il KOOK
Korean Journal of Dermatology 1987;25(5):553-561
The present study was performed to evaluate the effect of sulfhydryl compounds, cysteine and glutathione, on the size of melanosomes and the ratio of melanosormai stages of epidermal melanocytes in UV-irradiated black mice. The results were as follows; 1. Both of cysteine and glutathione showed significant diminution in the short axis of melanosomes and the percentage of stage 4 melanosomes of epidermal melanocytes in C57BL black mice skin. 2. The length of short axis of melanosomes in glutathione-treated group is smaller than those in cysteine-treated group at the end of 3rd week of intraperitoneal injection. The percentage of stage IV melanosomes significantly decreased in glutathione-treated group and cysteine-treated group at the end of 3rd week and 5th week respectively. 3. In glutathione-treated group, the short axis of melanosomes and the percentage of stage 4 melanosomes both decreased in proportional to the period of intraperitoneal in]ection.
Animals
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Axis, Cervical Vertebra
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Cysteine
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Glutathione
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Injections, Intraperitoneal
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Melanocytes*
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Melanosomes
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Mice*
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Skin
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Sulfhydryl Compounds*
9.Screening for Attention Deficit/Hyperactivity Disorder in Community Mental Health Services for Children.
Jae Won KIM ; Ki Hong PARK ; Min Jeong CHOI
Journal of Korean Neuropsychiatric Association 2004;43(2):200-208
OBJECTIVES: Developing mental health services and systems to identify children with Attention Deficit/Hyperactivity Disorder (ADHD) and providing early therapeutic interventions for them are important to prevent further impairments or disturbances associated with the disorder. The purpose of this study is to examine the clinical validities and efficiencies of Korean Child Behavior Checklist (K-CBCL) and Korean ADHD Rating Scale (K-ARS) for screening and identifying children with ADHD in community. METHODS: Randomly selected 1st to 3rd graders of the two elementary schools (n=1668) in the City of Gunsan participated in the study. K-CBCL and K-ARS were used as screening instruments. Diagnoses were determined by clinical psychiatric interviews and confirmed by Kiddie-Schedule for Affective Disorders and Schizophrenia-Present and Lifetime Version-Korean Version (K-SADS-PL-K) using DSM-IV criteria. RESULTS: The rate of inclusion above the T score of 60 with regard to the attention problems profile of K-CBCL was 4.5%. There was a significant correlation (p<.01) between parent and teacher reports of K-ARS, and the rate of inclusion was 2.5% when 90th percentile cut-off points were applied for both of the reports. Of the 46 subjects who underwent clinical psychiatric interviews, 33 (71.7%) were diagnosed as ADHD. The T score of 60 with regard to the attention problems profile of K-CBCL resulted in a reasonable level of sensitivity or positive predictive value in the diagnosis of ADHD. 90th percentile cut-off scores in both parent and teacher reports of K-ARS resulted in a high level of predictive value. The highest level of specificity and positive predictive value were computed when K-CBCL (T> or =60 in attention problems) and K-ARS (parent/teacher total> or =90th percentile) reports were combined. CONCLUSION: These findings suggest that the K-CBCL and K-ARS together could serve as a rapid and useful screening instrument to identify children with ADHD in epidemiologic case definitions.
Checklist
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Child Behavior
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Child*
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Community Mental Health Services*
;
Diagnosis
;
Diagnostic and Statistical Manual of Mental Disorders
;
Humans
;
Jeollabuk-do
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Mass Screening*
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Mental Health
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Mental Health Services
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Mood Disorders
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Parents
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Sensitivity and Specificity
10.Development of a Rapid Detection Method for Yersinia pestis by Polymerase Chain Reaction.
Ho Jung OH ; Hong Ki MIN ; Yeo Won SOHN ; Jeong Hoon CHUN ; Han Oh PARK
Journal of the Korean Society for Microbiology 1999;34(4):373-383
A polymerase chain reaction (PCR) method for detection of the pathogenic Yersinia pestis from other Yersinia spp. was developed. Five Y. pestis strains, ninety-two other Yersinia species and twenty-four Enterobacteriaceae strains were collected in Korea and from other countries. Oligonucleotide primers were designed from pathogenic gene of antiphagocytic protein capsule gene (fra 1) and plasminogen activator gene (pla). The 428 bp DNA fragment was amplified from five Y. pestis which contained the fra I gene. No product was amplified from other Yersinia species and other strains of the Enterobacteriaceae. The 439 bp DNA fragment was amplified from three K pestis which contained the pla gene. No product was amplified from two Y. pestis, other Yersinia species and other strains of the Enterobacteriaceae. These showed that the designed primers were specific for detection of Y. pestis among other Yersinia species and Enterobacteriaceae strains. Amplification was successful whether the template was derived from purified DNA or from aliquots of boiled bacterial suspension. The detection limits were 100 pg of DNA and 100 colony forming units (CFU) for fra I and 100 pg DNA and 10 CFU for pla, respectively. Our results prove that the PCR method using specific primers for Y. pestis is a rapid and convenient procedure for routine clinical detection and identification of Y. pestis.
DNA
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DNA Primers
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Enterobacteriaceae
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Korea
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Limit of Detection
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Plasminogen Activators
;
Polymerase Chain Reaction*
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Stem Cells
;
Yersinia pestis*
;
Yersinia*