• Journal of Life Science
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• v.9 no.6
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• pp.639-645
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• 1999

This research was for investigating the physiological effect caused by genetic disorder and others. Serum protein, serum LDH, and serum CPK were analyzed on Fragile X syndrome patients, carriers, unclassified mental retardees, and Down's syndrome patients by cellulose acetate plate electrophoresis. Also enzyme activity of LDH and CPK were measured. Significant differences were observed between normal group and mental retardees in compositions of serum protein, serum LDH, serum CPK, and enzyme activities. Mean percentages of albumin were 53.70$\pm$7.73% for Fragile X syndrome patients, 57.09$\pm$7.73% for carriers, 47.33$\pm$6.06% for unclassified mental retardees, 50.19$\pm$ 15.72% for Down's syndrome patients. Mean percentages of ${\gamma}$-globulin were 19.64$\pm$6.71% for Fragile X syndrome patients, 19.24$\pm$3.38% for carries, 25.66$\pm$4.74 for unclassified mental retardees, 23.41$\pm$6.08% for Down's syndrome patients. Mean percentages of LDH3 were 27.76$\pm$2.72% for Fragile X syndrome patients, 22.70$\pm$2.76% for carriers, 25.42$\pm$1.26% for unclassified mental retardees, 27.72$\pm$2.58% for Down's syndrome patients. Mean percentages of LDH4 were 2.70$\pm$2.04 for Fragile X syndrome patients, 3.79$\pm$2.74% for carriers, so both of them were significantly lower than normal(P<0.05). Mean percentages of CK-MB were 3.96$\pm$5.56% for Fragile X syndrome patients, 8.80$\pm$7.92%. Mean percentages of CK-MM were 95.81$\pm$5.50% for Fragile X syndrome patients, 91.20$\pm$7.92% for carriers. These results showed that significant abnormal compositions of blood proteins might be caused by genetic disorder. However, further analysis of many patients will be needed for clear conclusion.

• The korean journal of orthodontics
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• v.13 no.1
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• pp.31-43
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• 1983

The following results were abtained based on the research of the occlusal patterns among 1074 handicapped persons (cerebral palsy: 46, mental subnormality: 619, deafmute: 285, blind: 111, childish autism:8, cleft lip and cleft palate:3, polimyelitis:2) of the age between 6 and 23 in Chollanamdo, Korea, in comparison with a normal group of 1048 children of the age between 6 and 15 selected at random in J primary school in Gwang-ju City. 1. According to Angle's malocclusion classification, all the handicapped groups, except the cerebral palsy and the blind, showed a higher prevalence of malocclusion than that of the normal. Especially the prevalence of Class II, devision 1 malocclusion in the cerebral palsy was the highest, and the prevalence of Class III malocclusion in all the handicapped groups was higher than that of the normal group. Among these groups the highest prevalence of Class III malocclusion was in the Down's syndrome group. 2. On the the abnormal pattern of the anterior region, there was no significant difference $(P\leqq0.05)$ between the normal and the cerebral palsy, the deafmute, and e blind. The open bite $(7.27{\pm}1.04\%)$ and the cross-bite $(32.7{\pm}6.33\%)$ of the Down's syndrome wire higher than that of the normal, and the forward position of the mandible could be recognized in the Down's syndrome group. 3. On the midline position of the dentition, all the handicapped showed the same percentage of deviation, but the degree of mandibular shift to the right $(20.00{\pm}5.39\%)$ or left $(10.91{\pm}4.20\%)$ was higher than that of the normal only in the Down's syndrome group. 4. On the abnormal pattern of the posterior region, the cross-bite of the Down's syndrome was higher than that of the normal by $20.00{\pm}5.39\%$, the cross-bite of the cerebral palsy and the cross-bite and the open bite of the mental subnormality were slightly higher than that of the normal. The other handicapped groups showed no significant difference $(P\leq0.05)$ to the normal.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.4
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• pp.717-725
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• 2005

It is widely known that individuals with Down's syndrome(DS) often develop early onset severe periodontal diseases. In this study, We examined the prevalence of periodontopathic bacteria in DS patients to compare controls with mental disabilities(MD) The subjects were 27 DS patients (7 to 19 years old) and 27 age-matched controls with MD. Plaque index and gingival index were measured. And 5 pathogens, Actinobacillus actinomycetemcomitans, Porphyromonas gingivalis, Tannerella forsythia, Treponema denticola, Fusobacterium nucleatum, were surveyed in subgingival plaque samples using a polymerase chain reaction. No significant difference in plaque index and gingival index were observed between the DS and control group. The prevalence in DS was 96.3% for F. nucleatum, 74.1% for T. forsythia, 63.0% for P. gingivalis, 55.6% for A. actinomycetemcomitans. 40.7% for T. denticola. No significant differences were observed in the prevalence of periodontopathic bacterias between the DS and control. Prevalence of P.g(16.7%) at age $7{\sim}10$ is lower than other age group in DS, but its prevalence increased with age. Prevalence of A.a(83.3%) is peak at age $7{\sim}10$ in DS. These results suggest that various periodontopathic pathogens can colo nize in the very early childhood of DS and MD patients. But no significant difference was observed in the prevalence of periodontopathic bacterias between the DS and control.

• Journal of Chest Surgery
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• v.27 no.11
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• pp.902-906
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• 1994

Between May 1991 and August 1993, 16 patients underwent repair of complete atrioventricular septal defect without another major anomaly at Cardiovascular Center,Yonsei University College of Medicine. Ages of the patients ranged from 3 months to 38 years with a mean of 42 months. Among 16, 10 patients[63%] are associated with Down`s syndrome. All patients underwent primary repair except and one who received had been repaire of coactation of aorta and patent ductus arteriosus 2 month before. Preoperative mitral valve regurgitation [MR] was evaluated with Doppler echocardiography and angiography which revealed absent or grade I in 1, grade II in 8, grade III in 4, and grade IV in 3. Operative technique was performed under the moderate hypothermic cardiopulmonary bypass with crystalloid cardioplegia. Intraoperative echocardiography was performed epicardial approach [n=7] in the operative table or transthoracic approach [n=9] at intensive care unit. In all patients except 3, MR were improved. But in 3 patients, was not improved or exagerated comparing preoperative one. All of them were died.One patient was showed MR grade IV in intraoperative echocardiography, we re-repaired atriventricular valve with cardiopulmonary bypass. During follow-up period [at a mean of 11 months after repair], doppler echocardiography was performed in all patients. The follow up echocardiography revealed that the degree of MR in immediate postoperative period was not changed except in two patients in whom it was aggravated. Thus it seems that intraoperative and early postoperative echocardiography was employed important role of survival and can be predictable for long term results.

• Journal of Preventive Medicine and Public Health
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• v.22 no.3 s.27
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• pp.368-379
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• 1989

The relationship between mercury level of hair and mental retardation was investigated. The 297 subjects with mental retardation were drawn from two schools providing special educational services, one, consisted of children living in an orphan home, another, children with parents. The 117 centre] subjects were drawn from whom had got average or above average academic achivement in a regular elementary school. Hair sample were taken from the nape of the neck and the mercury analysis was carried out on an atomic absorption spectrophotometer(IL 551). There was no relationship between mercury contents and age, and there was a statistically significant difference in mercury contents between male and female in the mentally retarded children living with parents. Children in the retarded group had significantly higher mercury contents compared with control group except the female group with parents. Also, the mercury levels in the retarded group living in an orphan home were significantly higher than that of the retarded group with parents. The concomitant diseases were Down's syndrome, epilepsy, cerebral palsy and autism. There were statistically significant differences in hair mercury levels in the cases of accompanying Down's syndrome and cerebral palsy in male and Down's syndrome and autism in female compared with the control group of the same sex. The most accompanying handicap was speech disturbance(40.7%) and the others were crippled, emotional disturbance etc. The percentages of double handicap were 66.7% among 6 persons exceeding 6ppm of their hair mercury contents. 10.4% among $3{\sim}6$ ppm and 15.7% among the group of 3ppm or less. The findings of this study suggest that the more opportunities of exposure to mercury in mentally retarded children may have occurred, so it can not be excluded the possibility of mercury as a contributing factor to mental retardation. Therefore, the causal relationship between mercury levels and mental retardation should be established through the examinations about their living environments, dietary pattern, eating habit etc.

• Journal of KIISE:Software and Applications
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• v.27 no.8
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• pp.815-826
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• 2000

This study is for the segmentation and volume calculation of the white matter and gray matter from brain MRI. In general, the volume of white and gray matter is reduced by contraction of each components in the case of mental retardation which are Alzheimer's disease and Down's syndrome. As results, it is useful for diagnostic and early detection for various mental retardation through the tracing of variation for its volume from the brain MRI. But, until now, it was very difficult to calculate the partial volume of each components existing in some thickness, because MR image was represented by single gray value after scanning by MR scanner. Accordingly, new segmentation algorithm proposed in this paper is to calculate the partial volume of the white and gray matter existing in some thickness through the analysis of the blurred gray value, and is to determine the threshold for segmentation of white and gray matter, and is to calculate the volume of each segmented component. And finally, proposed algorithm was applied the models which was created manually, and then acquired results was compared with that of original model.

• Journal of Chest Surgery
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• v.24 no.2
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• pp.123-134
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• 1991

Twenty eight patients had undergone repair of an isolated complete atrioventricular septal defect between April 1986 and September 1990 in Seoul National University Children`s Hospital. The group comprised 13 male and 15 female patients. They ranged in age from 2 months to 8 years[mean 18.6months] and in weight from 3. 4kg to 23kg[mean 9.0$\pm$4.6kg]. They were analysed as Rastelli type A in 17 patients, Rastelli type B in 2 patients, and Rastelli type C in 9 patients. Seven patients had concomitant Down`s syndrome. All patients had large left-to-right shunt[mean pulmonary to systemic flow ratio 3.5 $\pm$2.2 ranging from 0.68 to 10.0] and high pulmonary systolic pressure[mean 74$\pm$18.8mmHg, ranging from 35 to 110]. In 11 patients, one patch technique was used to close the atrial and ventricular septal defect and 16 patients were undergone by two patch technique. We urgently managed only one patient by pulmonary artery banding whose anatomy was Rastelli type C and severe mitral regurgitation was identified. Postoperative complete A - V block was noted in 3 patients, two of whom were dead in operating room due to combined LVOTO and myocardial failure, and one patient with Rastelli type C was undergone by VVI type permanent pacemaker insertion 1wk later after two patch technique, but we had to manage him by modified Konno operation and total correction due to LVOTO and VSD leakage and severe mitral regurgitation 3 years later. Another two reoperation cases due to severe mitral regurgitation after two patch technique were undergone, one of whom we managed by mitral annuloplasty 3 months later but aggravated mitral regurgitation made us to control him by MVR 3 months later. Another one case of VSD leakage and tricuspid regurgitation was managed by total correction but she died of respiratory insufficiency 14 days later. We experienced pulmonary hypertensive crisis in 3 patients, who were dead in two cases comparing with one control case. So operative mortality is 9/27[33.6%], in one patch group of 3/11[29.2%] comparing with two patch group of 6/16[37.5%]. In summary, causes of death were pump weaning failure, myocardial failure and low cardiac output syndrome and pulmonary hypertensive crisis, resp. failure, complete AV block. Mean follow up period is 15.8$\pm$10.7 months[ranging from 3months to 37 months]

• Journal of Chest Surgery
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• v.29 no.3
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• pp.263-270
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• 1996

Twenty two patients underwent total anatomic correction of complete atrioventricular septal defect associated with other cardiac anomalies between July 1986 and December 1994. Age ranged from 6 months to 11 years(mean 49.6 $\pm$ 35.8 months), and they were composed of 7 males and 15 females. Combined major cardiac anomalies were tetralogy of Fallot(TOF) in 11 cases, double outlet of right ventricle (DORV) in 6 ca es, and transposition of great arteries (TGA) in 5 cases. Down's syndrome was associated in 5 patients with TOF and 1 patient with DORV. They were classified as Rastelli type A in 3 patients, B in 2 patients, and C in 17 patients. Modified Blalock-Taussig shunt was performed.in 5 patients and Waterston shunt in 1 patient as a palliative procedure. There were 7 perioperative deaths(31.8%) and the causes were pump weaning failure, low cardiac output, acute renal failure, persistant pulmonary hypertension and hypertensive crisis, and sepsis. Reoperations were performed in 4 cases to repair atrioventricular valvular regurgitation or to relieve the right ventricular outflow tract (RVOT) or pulmonary arterial stenosis. One late death was due to aspiration pneumonia. Second reoperation was necessary for progressive worsening of left atrioventricular regurgitation and RVOT stenosis in one patient. Fourteen survived patients were followed up for a mean of 66.0 $\pm$ 26.7months and all of them w re NYHA functional class I or II.