• Journal of The Korean Society of Inherited Metabolic disease
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• v.22 no.1
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• pp.9-14
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• 2022

Glutaric aciduria type 1 (GA1; OMIM #231670) is a rare autosomal recessive inherited neurometabolic disorder caused by the deficiency of glutaryl-CoA dehydrogenase. Infantile-onset GA1 is the most common form characterized by striatal injury and progressive movement disorder, and it is often triggered by an acute encephalopathic crisis within the first three years of life. Once this crisis occurs, there is a high likelihood for ineffective or limited conventional interventions, neurological disorders, or even death. Therefore, early diagnosis and immediate preventive management, such as dietary therapy, is essential. In the past decades, newborn screening (NBS) by tandem mass spectrometry for GA1 has been largely introduced in many countries including Korea, and it has led to improvements in the neurological outcomes of patients with GA1. In this review, the clinical symptoms, natural histories, and outcomes before and after the introduction of NBS in patients are discussed.

• Child Health Nursing Research
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• v.6 no.1
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• pp.89-102
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• 2000

Asthma is one of the most common chronic disease of childhood. Although an improved understanding of the pathophysiology of asthma has had a positive impact on the treatment and management of the disease, there has been a gradual but significant increase in asthma mortality. Also, mother's caring type is the essential factor in management of the child with asthma. The purpose of this study is to identify and understand the caring patterns of mathers of children with Athma by Q methodology. As a research method, 35 Q-statements were collected through individual interviews and review of related literatures. 37 subjects were interviewed and the data were analyzed by the PC QUANL program with principal component analysis. The were 3 different caring types classified as follows : Type I was the therapeutic compliance obeying type, compliancing medication and preventing cold etc. Type II was the physical strengthening type, taking Chinese medicine and folk remedy etc. Type III was the environment control type, managing house and environment clearly and ventilating room air etc. Therefore, it needs considering above each type in nursing care of the family and children with asthma.

• Journal of Genetic Medicine
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• v.10 no.2
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• pp.81-87
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• 2013

Pseudohypoaldosteronism (PHA), a rare syndrome of systemic or renal mineralocorticoid resistance, is clinically characterized by hyperkalemia, metabolic acidosis, and elevated plasma aldosterone levels with either renal salt wasting or hypertension. PHA is a heterogeneous disorder both clinically and genetically and can be divided into three subgroups; PHA type 1 (PHA1), type 2 (PHA2) and type 3 (PHA3). PHA1 and PHA2 are genetic disorders, and PHA3 is a secondary disease of transient mineralocorticoid resistance mostly associated with urinary tract infections and obstructive uropathies. PHA1 includes two different forms with different severity of the disease and phenotype: a systemic type of disease with autosomal recessive inheritance (caused by mutations of the amiloride-sensitive epithelial sodium channel, ENaC) and a renal form with autosomal dominant inheritance (caused by mutations of the mineralocorticoid receptor, MR). In the kidneys, the distal nephron takes charge of the fine regulation of water absorption and ion handling under the control of aldosterone. Two major intracellular actors necessary for the action of aldosterone are the MR and the ENaC. Impairment of the intracellular aldosterone signal transduction pathway results in resistance to the action of mineralocorticoids, which leads to PHA. Herein, ion handling the distal nephron and the clinico-genetic findings of PHA are reviewed with special emphasis on PHA type 1.

• Journal of Nutrition and Health
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• v.28 no.5
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• pp.451-459
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• 1995

This study was intended to figure out the effects of the amount and the distribution of body fat on the risk fators of adult disease. Sixty-four male college students paticipatied in this study, whose to find out body fat distributions were classified on the basis of Waist/hip ratio(WHR) into three groups-upper body type(UBTM), intermediate body type (IBTM) and lower body type(LBTM). Various risk factors such as adiposity, body fat ammount, serum lipid amount and blood pressure and their intercorrelations were analyzed. The three bodys type groups showed significant differences each other in weight(P<0.001), WHR showed considerable correlations with BMI and the percentage of body fat. The frequency of obesity assessed by BMI$\geq$25 and body fat percentage were the highest in the UBTM of the three groups. Thus, we could conclude that the closer the body fat distribution is to the upper body type, the higher the BMI and body fat percentage. Waist/girth ratio(WTR, P<0.01) and BMI(P<0.05) were positively correlated with serum triglyceride levels, and % of body fat was positively correlated with both serum triglyceride (P<0.01) and serum total cholesterol(P<0.05) levels. WHR (P<0.05), BMI(P<0.01) and % of body fat(P<0.01) also showed positive correlations with systolic blood pressure. From the above results, we could conclude that body fat distribution was a good index reflecting adiposity and body fat amount and that blood and serum amount of triglyceride was highest in the upper body type group showing the highest frequency of obesity.

• Journal of Sasang Constitutional Medicine
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• v.23 no.3
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• pp.275-284
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• 2011

1. Objective: This literary review investigates Lee Jema's clinical experiences with Soyang-type patients and their influence on his conceptualization of the Soyang physiology and pathology. 2. Methods: 1) The case reports in "Soyang constitutional type: Spleen Cold-based Exterior-Cold disease" were compared before and after the Sinchuk revision to explore the temporal change in the Sasang medical concepts. 2) The texts in Donguisusebowon (Gabo edition) and Donguisusebowon (Sasangchobongwon) were analyzed to investigate the pathological concepts appearing before the completion of the Donguisusebowon (Gabo edition). 3) The texts in Donguisusebowon (Sinchuk edition) was analyzed to investigate the pathological concepts formed between the Gabo and Sinchuk editions of Donguisusebowon. 3. Results and Conclusions: 1) Gabo edition : The Gabo edition divided the External-origin Exterior disease into Cold-dominant (Heat-moderate) and Heat-dominant (Cold-moderate) patterns and differentiated the severity of Exterior-based Exterior disease and Interior-based Interior disease into mild, moderate, severe, and critical conditions. Cold-damage Delirium disease pattern was categorized as an Interior-based Interior disease, and the treatment protocol using Baekho-tang (Baihu-tang) was established. The stool condition and bowel movement reflecting the patient's defecation habits, the prime indicator of health in the Soyang constitutional type, were emphasized on their importance. 2) Sinchuk edition: The Delirium disease pattern was moved into the Exterior-cold disease, and the treatment protocol applying Jihwangbaekho-tang (Dihuangbaihu-tang) and Hyeongbangsabaek-san (Jingfangxiebai-san), with variational usage of Gypsum, was newly established. The Seong-Jeong and basal disease patterns were suggested as important factors in treating the patients. Also, it was proposed that the symptoms and signs reflecting the condition of the life-preserving energy be assessed to understand the patient's current condition. The importance of post-acute rehabilitation and aftercare as well as the most appropriate acute-stage treatment were emphasized.

• Child Health Nursing Research
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• v.12 no.3
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• pp.322-332
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• 2006

Purpose: This study was done to identify types of subjective perception of their disease by adolescents who have insulin dependent diabetes mellitus (IDDM) in order to better understand these adolescents and to determine effective nursing interventions. Method: Q-methodology was used in this study. The 36 Q-statements were selected through a literature review and in-depth interviews with participants. Data were collected from a 33 P-sample by sorting the 36 Q-statements into a 9 point standard. The data were analyzed using the PC QUANL program. Results: Five types of perception were identified: Type 1 being a recipient and having a will to cope with the disease, Type 2, seeking support from others, Type 3, emotional and negative coping, Type 4, pursuing own beliefs but with conflict at the same time, and Type 5 feeling under heavy pressure as their condition made them feel insecure, and they felt sorry to their families. Conclusion: The findings of the study are expected to contribute to improving clinical practices by helping nurses have a broader understanding of adolescents with IDDM, and exploring ideal nursing interventions.

• Clinics in Shoulder and Elbow
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• v.2 no.2
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• pp.110-114
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• 1999

We reviewed the results of arthroscopic surgery in patients with rotator cuff disease. Arthroscopic subacromial decompression(ASD) was performed on 22 patients with rotator cuff disease who had not responded to nonoperative measures. In the patients who had a complete tear of the rotator cuff(four of ten Neer's stage III patients), mini-open repair also was performed. Results were determined by questioning patients about their satisfaction with the outcome of surgery and by functional assessment of the shoulder with the parameters of pain, function, and range of motion according to the UCLA shoulder rating scale. The average follow-up was 21 months. The results were sixteen(72%) excellent, three(14%) good, two(9%) fair, and one(5%) poor. The following variables were analyzed to assess their influence on final outcome; duration of preoperative symptoms, Neer's stage(extent of damage to the cuff), type of acromion. Satisfactory results were achieved in thirteen of fourteen patients(93%) who had duration of preoperative symptoms below one year, and in four of six(67%) above 2 years. And satisfactory results were achieved in eleven of twelve patients(92%) who had Neer's stage II and in eight of ten(80%) stage III and achieved in six of seven patients(86%) who had Bigliani's acromion type I, in nine of eleven(82%) type II, and in four of four(100%) type III. There are multiple factors that may influence the recovery after ASD or the eventual outcome. However, although there is still a controversy about the pathogenesis of rotator cuff disease, We found that arthroscopic subacromial decompression and mini-open repair in patients with rotator cuff disease were well enough documented to be considered a standard treatment.

• Journal of Korean society of Dental Hygiene
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• v.10 no.2
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• pp.335-344
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• 2010

Objectives : Targeting Korean adults, the food pattern are grasped. And, its correlation with oral disease is analyzed. In order to offer basic data to developing the nutritional policy and nutritional program for the future prevention from oral disease, a research was conducted by utilizing the Korean National Health and Nutrition Examination Survey 2005(the 3rd term). Methods : The subjects in this study were 6,526 adults in more than fully 19 years among 9,047 persons who participated in the food intake survey out of those who completed the health interview survey. The statistical analysis was analyzed by using SPSS 12.0 program. Results : 1. As a result of Group Analyzing was indicated to dangerous-type food pattern and protection-type food pattern. 2. As a result of analyzing the answers for having dental caries in the annually personal recognition was indicated to be high in the dangerous-type food pattern, and had not the statistically significant difference. 3. As a result of analyzing the answers for having periodontal disease in the annually personal recognition was indicated to be high in the dangerous-type food pattern, and had the statistically significant difference(p<0.05). 4. As a result of analyzing the food pattern factors that have influence upon both major oral illnesses in the annually personal recognition, the person, who has the dangerous-type food pattern, had high risk level of the periodontal disease in the annually personal recognition. Conclusions : In the above results, as a result of surveying and analyzing importance of the food pattern in the incidence of both major oral illnesses, it is considered that there will be necessity of continuing to research into developing the nutritional policy and nutritional program in order to prevent oral illness in the future.

• Journal of Nutrition and Health
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• v.41 no.4
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• pp.327-340
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• 2008

This study was designed to investigate the correlation between nutrition status and cardiovascular disease in type 2 diabetes patients with metabolic syndrome. The subjects were 66 patients and divided into Non MS (a group without metabolic syndrome, n = 37) and MS (a group with metabolic syndrome, n = 29). The percentage of patients accompanying metabolic syndrome was 43.9% and family history such as DM, skipping meal and eating speed were higher in MS (P < 0.05) The average values of BMI, body fat (%), waist circumference were significantly higher in patients of MS than that of Non MS. For hamatological values, MS showed higher FRS, HOMA-IR, LDL-Cholesterol, CRP. Percentage of FRS was 21.63% in MS that is relatively higher in comparison with 16.81% in Non MS. Moreover, the incidence of cardiovascular disease appeared 13.8% in MS that is higher than 2.7% in Non MS. The intake of sodium and vitamin E were higher, but the intake of fat, vitamin A and zinc were lower in MS than in Non MS. Close correlations were elucidated among FRS, occurrence of cardiovascular disease, weight, waist circumference, Total-Cholesterol, LDLCholesterol, sodium in both groups. In conclusion, cardiovascular disease risk factors would be higher in type 2 diabetes patients with metabolic syndrome and there were distinctive patterns that were associated with hamatological values, nutrition intake risk factors. This result should be considered when designing nutrition study and intervetion programs.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.3 no.1
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• pp.63-63
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• 2003