• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.16 no.2
• /
• pp.79-85
• /
• 2016

Purpose: This study aimed to analyze genetic mutations, clinical manifestations, and treatment of patients with benign HPA in Korea. Methods: This case series study involved ten HPA patients who were referred to our hospital because of high phenylalanine concentration. We investigated their demographic features, clinical manifestations, and mutations of the PAH gene through direct DNA sequencing. Results: Among ten patients with benign HPA, two pairs of patients were related (father-daughter, mother-daughter relationship) cases, and all of them showed no specific clinical manifestations or notable past history. Their plasma phenylalanine levels ranged between 1.2 and 4.2 mg/dL. In the tetrahydrobiopterin (BH4) loading test, all patients were nonresponsive to BH4. In the confirmation test of PAH mutation analysis, we identified eleven different alleles out of twelve. The most common allele was R53H (c.158G> A). In addition, two novel PAH gene mutations, V423A (c.1268T>C) and V51A (c.152T>C), were identified. Although the patients did not receive any pharmacologic treatment or continuous phenylalanine restriction dietary therapy, their neurocognitive development was normal. Moreover, on serial outpatient follow-up tests, all patients maintained phenylalanine levels below 6 mg/dL. Conclusion: This study is the first in Korea to analyze benign HPA patients. All patients with benign HPA could maintain phenylalanine levels below 6 mg/dL with normal neurocognitive development, without continuous therapy. Therefore, performing mutation analysis and distinguishing benign HPA from phenylketonuria (PKU) are important to help improve life quality in patients with benign HPA by avoiding unnecessary lifelong therapy.

• Korean Journal of Psychosomatic Medicine
• /
• v.7 no.1
• /
• pp.116-123
• /
• 1999

Objectives : The authors attempted to assess how much the mechanism of dissociation affects somatization disorder patients psychopathologically, and explore the relationship between sexual or physical abuse and somatic symptoms in somatization disorder patients. Methods : The authors administered the Dissociative Experiences Scales-Korean version(DESK) and Dissociative Disorders Interview Schedule to 25 patients with somatization disorder and 51 normal subjects. Results : There were no significant demographic differences between patient and control groups. The mean score of DES-K for patient group was 18.2, and 10.0 for the control group. The percentage of the individuals with high scores(20 and over) was 36.0 in the patient group and 7.8 in the control group, respectively. The percentage of the individuals with sexual and/or physical abuse was 16.0 in the patient group and zero in the control group. Our results showed that DES-K scores were not influenced by the factor of age or religion in either group, but the scores of the patients with somatization disorder were significantly higher than those in the normal subjects. Conclusion : There was an implication that the mechanism of dissociation affects issues of psychopathogenesis and psychopathology in Korean patients with somatization disorder, even though they have different sociocultural backgrounds in comparison to Western patients. The authors suggest it is useful to focus attention on childhood abuse and dissociation in the evaluation and dynamic psychotherapy of patients with somatization disorder.

• The Korean Journal of Nuclear Medicine Technology
• /
• v.12 no.3
• /
• pp.241-246
• /
• 2008

Purpose: To study of difference among primary aldosteronism patients and normal groups and essential hypertension patients and to confirm aldosterone/plasma renin activity ratio increase in secondary hypertension group which was diagnosed as primary aldosteronism. Materials and method: 1. Period: from April 2006 to March 2008. 2.Targets: 901 patients who visited seoul national university bundang hospital. 3. Groups: we divided by three groups. (normal group (n=147), essential hypertension (n=709), primary aldosteronism (n=45)) 4. Then calculated aldosterone/plasma renin activity ratio. 5. We used ROC curve to measure sensitivity and specificity. Results: 1. normal groups aldosterone/plasma renin activity ratio: $52.8{\pm}52.46$ essential hypertension patients aldosterone/plasma renin activity ratio: $171.04{\pm}291.56$ primary aldosteronism patients aldosterone/plasma renin activity ratio: $2325{\pm}2200$. 2. Aldosterone/renin ratio was significant in comparing each groups (p<0.001). 3. The sensitivity was 91.1% and the specificity was 92.4% when cut off of aldosterone/renin ratio was 485. Conclusion: It was confirmed that aldosterone/plasma renin activity ratio in primary aldosteronism was higher than normal group. According to this result, we can tell that aldosterone/ plasma renin activity ratio is very useful in diagnosis of primary aldosteronism.

• Korean Journal of Psychosomatic Medicine
• /
• v.28 no.1
• /
• pp.20-28
• /
• 2020

Objectives : The aim of this study was to compare psychosocial characteristics of the functional gastrointestinal disorders FGID group, non-FGID group, and control group and determine factors affecting the QOL of patients with FGID. Methods : 135 patients diagnosed with FGID were selected. 79 adults had no observable symptoms of FGID (control group) and 88 adults showed symptoms of FGID (non-FGID group). Demographic factors were investigated. The Korean-Beck Depression Inventory-II, Korean-Beck Anxiety Inventory, Korean-Childhood Trauma Questionnaire, Multidimensional Scale of Perceived Social Support, Connor-Davidson Resilience Scale, Patient Health Questionnaire-15 and WHO Quality of Life Assessment Instrument Brief Form were used to assess psychosocial factors. A one-way ANOVA was used to compare differences among groups. Pearson correlation test was performed to analyze the correlation of psychosocial factors and QOL of the FGID group. Further, a hierarchical regression analysis was conducted to determine factors affecting the QOL of the FGID group. Results : Between-group differences were not significant in demographic characteristics. Depression (F=48.75, p<0.001), anxiety (F=14.48, p<0.001), somatization (F=24.42, p<0.001) and childhood trauma (F=12.71, p<0.001) were significantly higher in FGID group than in other groups. Social support (F=39.95, p<0.001) and resilience (F=17.51, p<0.001) were significantly lower in FGID group than in other groups. Resilience (β=0.373, p<0.01) was the most important explanatory variable. The explained variance was 47.2%. Conclusions : Significantly more symptoms of depression, anxiety, childhood trauma, and somatization were observed for the FGID group. This group also had less social support, resilience, and quality of life than the non-FGID and control groups. The key factor for quality of life of the FGID group was resilience.

• Biomedical Science Letters
• /
• v.5 no.2
• /
• pp.181-190
• /
• 1999

To evaluate the usefulness of transforming growth factor-$\beta$1 (TGF-$\beta$1) as a new tumor marker, we determined the plasma TGF-$\beta$1 levels using sandwich ELISA assay in cancer patients. Patients with three most common adult cancers in Korea (stomach, liver and breast cancer) and children's cancers (leukemia and two kinds of solid tumor) were enrolled for the study. Furthermore, 39 individuals were subjected to age and sex-stratified plasma TGF-$\beta$1 analysis. No statistical difference was demonstrated with respect to age or sex. The mean plasma TGF-$\beta$1 level (16.0 ng/ ml) of stomach cancer patients was significantly higher than that (8.3 ng/ml) of controls. However, there was no difference among the mean plasma TGF-$\beta$1 levels of liver, breast cancer patients and controls. Seven of 16 patients (43.7%) with stomach cancer, one of 8 (12.5%) with liver cancer, and one of 7 (14.3%) with breast cancer showed higher TGF-$\beta$1 levels compared to controls. Plasma TGF-$\beta$1 concentrations of five leukemic children remained in the normal range regardless of the remission state. In contrast, initial high TGF-$\beta$1 levels from two children with solid tumors returned to normal range on surgical resection of tumors. From the above results, we could conclude that plasma TGF-$\beta$1 levels of apparently healthy individuals seem to be rather constant irrespective of difference in age or sex, and the plasma TGF-$\beta$1 has the limited value as a screening test for the diagnosis of aforementioned adult cancers because of its low sensitivity. Finally, additional studies need to be pursed for the large number of stomach cancer and pediatric solid tumor patients in order to reach a secure conclusion on the usefulness of plasma TGF-$\beta$1 as a tumor marker in these patients.

• Clinical and Experimental Reproductive Medicine
• /
• v.32 no.1
• /
• pp.65-70
• /
• 2005

본 연구는 이식 후 남은 잉여의 포배기 배아를 두 번의 냉동과 융해 과정을 반복적으로 실시한 후 이식한 결과에 관한 보고이다. 사람 포배기 배아의 동결보존에서 높은 생존율과 성공적인 임신율이 보고되고 있으나 미성숙 난자로부터 발달한 포배기 배아에 두 번의 초급속 냉동 방법을 실시한 후 이식한 보고는 되어 있지 않다. 이에 본 연구에서는 다낭성 난소 증후군 환자에게서 얻은 미성숙 난자로부터 발달한 포배기 배아를 artificial shrinkage 후 초급속 냉동함으로써 생존율을 높이는 방법을 이용하여 재냉동 이식하였을 때 임신에 성공한 증례를 보고하고자 한다. 29세의 환자로부터 채취한 55개의 미성숙 난자들(germinal vesicle stage oocytes)을 체외배양 하여 성숙한 37개의 난자들로부터 30개의 수정란을 얻을 수 있었다. 12개의 배아가 포배기 배아까지 발달하였으며 이 중 3개의 양질의 포배기 배아를 선별하여 이식하였고, 이식을 한 후에 남은 9개의 포배기 배아들은 artificial shrinkage의 과정을 마친 후에 초급속 냉동 방법을 이용하여 동결보존 하였다. 그 중, 4개의 포배기 배아들을 융해한 후 이식을 하지 않고 다시 재냉동을 하여 보관하였고 이 후 재냉동 되었던 4개의 포배기 배아들을 다시 융해 하여 이식을 한 결과 임신이 되어 건강한 남아를 분만하였다. 이로써 미성숙 난자로부터 얻은 포배기 배아가 두 번의 냉동과 융해의 과정을 통해 크게 손상을 입지 않고 생존할 수 있다는 것을 알 수 있었다. 그러므로 융해이식 후 남은 잉여의 포배기 배아를 다시 냉동 보관하여 다음 주기에 이용함으로써 축적된 임신율을 증가시킬 수 있을 것으로 사료된다.

• The Journal of the Korea institute of electronic communication sciences
• /
• v.9 no.1
• /
• pp.123-130
• /
• 2014

The aim of this study was to determine the effect of action observational physical training on upper extremity function and activities of daily living in stroke patient. 19 hemiparetic patients participated in this study and were randomly selected into an experimental group and a control group. An experimental group observed performance actions of purposeful activity task through a video and imitated actions with the traditional occupational therapy, and a control group only observed actions with the traditional occupational therapy. Traing was performed 3 times a week and 30 min for each round for 4 weeks. WMFT were performed for an upper extremity function and MBI were performed for activities of daily living. As a result, WMFT and MBI showed significant difference between before and after in two groups but didn't show significant difference between two groups.

• The Journal of Korea Assosiation for Disability and Oral Health
• /
• v.13 no.2
• /
• pp.114-118
• /
• 2017

Sedation or general anesthesia is often required to offer dental treatment to patients with special needs. However, patients with ASA classification of III or higher are not indicated for moderate sedation and might be exposed to danger when treated at outpatient dental clinic. For this reason, it is recommended to treat those patients under general anesthesia. The dental team can supervise and monitor the whole procedures during treatment with an appropriately equipped facility. This case report describes the dental treatment of a 7-year-old girl with multiple disabilities. Preoperative evaluation including medical consultation was carried out thoroughly and dental treatment was performed under general anesthesia safely.

• Journal of Chest Surgery
• /
• v.36 no.12
• /
• pp.928-928
• /
• 2003

승모판성형술 직후에 시행한 재수술에 대한 연구보고는 많지 않다. 저자들은 승모판성형술 직후 성형술 실패로 판단되는 환자에서 즉시 시행한 재수술 결과에 대해 알아보았다. 대상 및 방법: 1995년 4월부터 2001년 7월까지 세종병원에서 승모판성형술을 받은 환자 중 체외순환 이탈 직후 시행한 경식도초음파 검사에서 승모판막폐쇄부전 혹은 협착이 의미있게 잔존하거나 다른 이유로 재수술이 즉시 필요하였던 18명을 대상으로 후향적으로 조사하였다. 남녀비는 5 : 13이었고 평균 연령은 44세였다. 승모판막 질환은 폐쇄부전 12명, 협착 3명, 그리고 혼합형이 3명이었다. 원인은 류머치스성 9명, 퇴행성 8명, 그리고 심내막염이 1명이었다. 재수술의 원인은 잔존 승모판폐쇄부전 13명, 협착 4명, 그리고 좌심실천공이 1명이었다. 14명(77.8%)에서 재성형술을, 4명에서는 인공기계판막치환술이 시행되었다. 결과: 조기사망은 없었다. 조기결과는 승모판막치환을 한 4명을 제외한 14명 중 13명(92.9%)에서 0-I도의 폐쇄부전을 보였고 협착은 14명 모두 경도 이하 상태였다. 평균 33개월을 추적조사 한 결과 1명이 술 후 4개월 후 심기능부전으로 사망하였다. 승모판폐쇄부전은 9명(64.3%)에서 0-I도를, 승모판협착은 11명(78.6%)에서 경도 이하였고 재수술은 1명에서 시행되었다. 6년 생존율과 재수술로부터의 자유도는 각각 94%와 90%였다. 4년 후 승모판폐쇄부전 및 협착 재발로부터의 자유도는 각각 56%와44%였다. 결론: 승모판막성형술 직후 재수술은 양호한 조기 및 중기 생존율을 보이며 일차성형술 실패 후에도 높은 빈도에서 재성형술이 가능하다. 하지만 재성형술 시 특히 류머치스성 판막질환에서는 판막 기능부전 발생률이 높기 때문에 성형술 후 판막부전의 재발을 줄이기 위해서는 성형술의 적절한 적용 및 적응증 선별이 중요할 것으로 생각된다.

• Journal of the Korea Academia-Industrial cooperation Society
• /
• v.20 no.11
• /
• pp.189-196
• /
• 2019

This study identifies the moderating effects of cognition on the relationship between self-care confidence and self-care behavior in the diabetic elderly. The participants were 205 elderly subjects with diabetes, who visited a citizen health center located in D city, Korea. Data were collected from 20th April to 31st August 2015, and were analyzed by t-test and multiple regression. Our results reveal no significant differences between the depressive and non-depressive groups, when considering diabetes self-management confidence, knowledge, behavior and cognitive function. Cognitive function had a moderating effect in the relationship between self-care confidence and self-care behavior only in the depressed group. Our results indicate that it is therefore necessary to apply individual nursing intervention based on cognitive function and depression level, and to develop various programs for improving the cognitive function and depression amongst the diabetic elderly.