• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1217-1221
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• 2008

Purpose : An association between idiopathic thrombocytopenic purpura (ITP) and systemic lupus erythematosus (SLE) has been recognized for decades because thrombocytopenia is the first manifestation in some patients with SLE. However, the risk of later development of SLE in childhood ITP is currently unknown. We retrospectively evaluated the incidence and clinical significance of the positive antinuclear antibody (ANA) in children with acute ITP. Methods : This study was retrospectively performed to review the clinical and laboratory characteristics in 77 children diagnosed to have acute ITP and admitted to the Pusan National University Hospital between January 2003 and December 2006. Patients tested positive for ANA were regularly followed-up for at least 12 months for symptoms indicative of SLE. Results : Seventy-seven children were included in the study; 38 males (49.4%) and 39 females (50.5%), the mean age was 4.5 years. Sixteen (20.8%) ITP patients had a positive ANA, with a median titer of 1:320. The mean age of the patients with positive ANA was 9.3 years, which is much older than 3.3 years for patients with negative ANA (P<0.05). The positive ANA group was predominantly female (81.3%) compared to the negative ANA group (P<0.05). There was no statistically significant difference in mean platelet counts between both groups. No statistically significant difference was found in ANA positivity and progression to chronic ITP or SLE. After the median follow-up of 32 months, SLE was diagnosed only in one ITP patient with positive ANA. Conclusion : Our data demonstrated that ANA positivity is often found in children with acute ITP. Large-scale studies should be considered to determine the significance of ANA positivity in childhood ITP for the later development of SLE.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.170-175
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• 1997

Purpose : Hemolytic uremic syndrome(HUS), known as a most common cause of childhood renal failure in western countries, has been a relatively rare disease in Korea. Although the reported cases were not related to any specific cause in Korea, there was an outbreak of HUS with bloody diarrhea in Japan last year. We report here that we experienced the several typical HUS last year. Patients : From Jan.1996 to Dec. 1996, five patients were diagnosed as HUS at Samsung Seoul Medical Center, Dept. Pediatrics. Results : 1) The age of onset was below 3 years in 3 cases and above 9 years in 2 cases. 2) All the cases happened between summer and autumn. Three patients had domestic travel and 4 patients drank well or spring water before the symptoms. 3) The clinical manifestation was generalized edema, oliguria, anuria and hematuria. The bloody diarrhea were present in 5 cases and 1 patient had operation with the impression of appendicitis. 4) There was no bacteria which was isolated from the blood or stool samples. 5) Renal biopsies were performed in 2 cases, because of protracted clinical course. One showed microthrombotic angiopathy and the other cortical necrosis with necrotizing glomeruli. 6) Complete recovery was the outcome in 4 cases and one case progressed to chronic renal failure. Conclusion : In conclusion, typical HUS associated with bloody diarrhea, epidemic and good prognosis can be found in Korea and careful surveillance of the pre-clinical cases will be necessary.

• Journal of the Korean Society of School Health
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• v.6 no.1
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• pp.9-44
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• 1993

An explorative and descriptive study in order to determine the effect of family structure and other socio-demographic variables on health behavior, belief, locus, and value and utilization of public health service was carried out. Data were collected from 1,653 subjects randomly sampled in three areas, Seoul, Kyunggi province, and Cheju province. From Seoul 849 subjects were selected, 397 subjects Kyunggi, and 407 subjects from Cheju, respectively. Self-reporting questionaires were administered during the period from March to June, 1992. The major findings were as follows: 1) The subjects visited herb-doctor's at irregular intervals mainly to have tonic medicine prepared. They preferred herb-doctor's rather than with doctors at clinics and hospitals. Statistically significant difference was found among the regions studied (p<0.05). 2) The reason for visiting hospitals was primarily for treatment of diseases. They preferred hospital because they felt that the hospitals offer much highly reliable treatment services as well as medical accessibility. For the purpose of hospital utilization, statistically significant differences existed among sex, educational level, family type and region. However, no significance was found among sex, educational level, and region (p<0.05). 3) The subjects utilized general hospitals mainly for diagnosis and treatment of diseases. They preferred general hospitals because of their much better facilities and reliability. Statistical significance was found among sex, educational level, and region (p<0.05). 4) The subjects visited dentist at irregular intervals basis. They visited once half a year or three to four months. their purpose of visit was mainly for diagnosis and treatment of diseases. Statistical significance differences were found among educational level, region and economic standard (p<0.05). 5) Whenever their illnesses were mild and the pharmacies was located in nearby they visited to pharmacies. They visited once a month and patient medicines. Statistically significant differences were found among sex, educational level and region (p<0.05). 6) The subjects believed that herb medicine was quite efficacious for treatment of some diseases, particularly by information handed down through time-honored tradition and experience. However, they recognized that the efficacy of folk medicine can vary with type and severity of diseases. Statistical significance was among sex, educational level, region and economic standard (p<0.05). 7) The reason why subjects believed that pray and superstition are effective for treatment of certain type of diseases, particularly in neuropathy, was the belief in God's almighty. Statistically significant differences were found among sex, educational level, regions and economic standard (p<0.05). 8) Most of subjects under same condition preferred western medicine because they believed that it is more scientific and prompts in showing therapeutic effect. Statistical significance was not found in the choice of type of public health service among, regions. But significant differences were found among sex, educational level and region (p<0.05). 9) The subjects looked for pharmacy if they thought the symptom was mild. However, they visited hospitals for chronic disease and general hospitals for emergency treatment. Statistical significances were found among educational level, region and economic standard (p<0.05). 10) Although most of students wanted to have a healthy life as for the component of health standard and value, they think that they are not healthy (p<0.05). As for the health behavior, significant difference was found in the proportion of smoking and drinking between educational level and region (p<0.05). The health locus was affected by educational level, and health behavior was influenced by region, sex and educational level. The utilization of type of public health service was influenced by family type and region, and health belief by region and educational level, and the health values by region and economic standard respectively, most of correlation showed statistical significance. Among them, the highest correlation was seen between locus of control and external/internal locus of control, which is quite obvious. The correlation between health belief and behavior was the next highest, but still low (0.343). All the other variables are low but significant except only a few of those. These findings indicate that health education should be incorporated into the curriculum so as to develop desirable health habit, and ability of self-control in accordance with their growth stages. A systematic and scientific understanding on the herb/folk medicine is needed, and greater reliability of the utilization of public health services are is still required. Health policy for equal distribution of health service throughout the country along the hierarchical health service system and complementary mutual assistance and cooperation among various health organizations are also required.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.135-141
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• 2014

Objectives: Fabry disease (FD) is a lysosomal storage disease caused by the inappropriate accumulation of globotriaosylceramide (Gb3) in tissues due to a deficiency in the enzyme ${\alpha}$-galactosidase A. Hypertrophic cardiomyopathy is one of the chronic complications of FD. We tried to evaluate the prevalence of Fabry disease in the Korean patients with left ventricular hypertrophy (LVH). Methods: A total of 257 patients with LVH were recruited and they were 172 males (mean 56 years, range 30-81 years) and 84 females (mean 66 years, range 45-85 years). Urinary Gb3 was used to screen FD by high performance liquid chromatography-tandem mass spectrometry. Confirmatory tests were done by alpha-galactosidaseA activity using fluorometric assay and by GLA mutation analysis using sequencing. Results: Four patients were screening positive by urinary Gb3 analysis (cutoff, 25 ug/mmol creatinine). But, one female patient was diagnosed with FD confirmed by enzyme analysis in leukocytes as well as by genetic analysis (1/257 patients, 0.4%). She showed 54.3 ug/mmoL creatinine of Gb3 and 15.5 nmole/hr/mg protein (reference range, $55.2{\pm}12.7nmole/hr/mg$ protein) of alphagalactosidase A activity. And she had a heterozygous GLA mutation of c.796G>A (p.D266N). Her daughter was found to be a carrier for FD confirmed by GLA mutation analysis. Asymptomatic carrier showed 25.5ug/mmol creatinine of Gb3 and 42.5 nmole/hr/mg protein (reference range, $55.2{\pm}12.7nmole/hr/mg$ protein) of alpha-galactosidase A activity. Conclusions: The prevalence of FD in Koran patients with LVH was detected as 0.4%. Although the prevalence seems to be low, screening studies are of great importance for detecting hidden cases as well as for identifying other effected family members.

• Journal of Nutrition and Health
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• v.41 no.2
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• pp.147-155
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• 2008

Medical nutrition therapy (MNT) is considered a keystone of medical treatment of chronic diseases. However, only few studies have evaluated medical and economical outcome of MNT. The study was performed on the patient with type 2 diabetes mellitus to evaluate the effect of clinical and cost-effective outcomes of MNT. Subjects from two general hospitals were randomly assigned to two different groups; One receiving basic nutritional education (BE) (n = 35), and the other receiving intensive nutritional education (IE) (n = 32) for a 6-month clinical trial. The group which received BE had a single visit with a dietitian, while the other group which received IE had an initial visit with a dietitian addition to two visits during the first 4 weeks of the study periods. Anthropometric parameters, blood components, and dietary intake were measures at the beginning of study period and after 6 month. Cost-effective analysis included direct labor costs, educational materials and medication cost difference during 6 months. After 6 month, subjects from IE group showed significant reduction of body weight (p <0.05) and systolic blood pressure (p <0.05), whereas BE group did not show any significant changes. Result from biochemical indices showed glycated hemoglobin concentration was significantly reduced by 0.7% (p <0.05) only in the IE group. The ratio of energy intake to prescribed energy intake decreased significantly in both groups (p <0.05). Mean time taken for a dietitian to educate the subject was 67.9 ${\pm}$ 9.3 min/person for BE group, while 96.4 ${\pm}$ 12.2 min/person for IE group. Mean number of educational materials was 1.9 ${\pm}$ 0.7/person for BE group and 2.5 ${\pm}$ 0.7/person for IE group. Change in glycated hemoglobin level along the 6 month period of study can be achieved with an investment of \88,510/% by implementing BE and \53,691/% by implementing IE. Considering the net cost-effect of blood glucose control and HbA Ic, IE which provides MNT by dietitian had a cost efficiency advantage than that of BE. According to this study, MNT provided by dietitian had a significant improvements in medical and clinical outcomes compared to that of BE intervention. Therefore, MNT protocol should be performed by systemic intensive nutrition care by dietitian in clinical setting to achieve good therapeutic results of DM with lower cost.

• Tuberculosis and Respiratory Diseases
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• v.46 no.4
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• pp.489-499
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• 1999

Background: The patient with bronchiectasis may have obstructive ventilatory impairment combined with mild restrictive ventilatory impairment due to fibrosis of surrounding lung parenchyme and pleural adhesions caused by chronic recurrent pulmonary infections. Since hyperinflation or emphysematous change can be occured in bronchiectasis, pulmonary functions such as lung volumes and diffusing capacity may also vary with associated emphysema. Methods: For the evaluation of lung volumes and diffusing capacity in bronchiectasis with respect to the anatomic types and severity of bronchiectasis, a total of 40 cases comprising 24 cases of tubular, and 16 cystic type of bronchiectasis were analyzed retrospectively. Correlation between lung functions and extent of bronchiectasis or associated emphysema detected in HRCT were also evaluated. Results: Vital capacity(VC) tended to decrease in cystic type than in tubular type. As the severity of bronchiectasis became serious, the VC were significantly reduced, whereas the total lung capacity(TLC), residual volume(RV) and its ratio to the total lung capacity(RV/TLC) had no significant difference. Lung clearance index(LCI) was significantly increased in cystic type than in tubular type, whereas the slope of phase III in single breath nitrogen curve($\triangle$N2/L) was not significantly changed regard to the type and severity of bronchiectasis. DLCO and DLCO/VA reflecting diffusing capacity were significantly decreased in cystic type and also as the severity of bronchiectasis became serious. The correlation coefficient of VC, DLCO and LCI with the extent of bronchiectasis were -0.322, -0.339 and 0.487, respectively, whereas other parameters were not significantly correlated with the extent of bronchiectasis. VC and DLCO correlated negatively with the extent of emphysema while RV, RV/TLC, LCI and $\triangle$N2/L correlated positively. Conclusion: These findings suggest that the reduction of VC and diffusing capacity or uneven distribution of inspired gas in bronchiectasis are related to both the extent of bronchiectasis and associated emphysema while increased residual volume be related to the extent of associated emphysema alone.

• Tuberculosis and Respiratory Diseases
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• v.55 no.5
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• pp.516-521
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• 2003

The Aspergillus species produces metabolic products that play a significant role in the destructive processes in the lung. We experienced a case of chronic necrotizing pulmonary aspergillosis caused by an Aspergillus niger infection, which contained numerous calcium oxalate crystals in the necrotic lung tissue. A 46-year-old man, who had a history of pulmonary tuberculosis, presented with high fever, intermittent hemoptysis and pulmonary infiltrations with a cavity indicated by the chest radiograph. Despite being treated with several antibiotics and anti-tuberculosis regimens, the high fever continued. The sputum cultures yielded A. niger repeatedly, and intravenous amphotericin B was then introduced. The pathological specimen obtained by a transbronchial lung biopsy revealed numerous calcium oxalate crystals in a background of acute inflammatory exudates with no identification of the organism. Intravenous amphotericin B was continued at a total dose of 1600 mg, and at that time he was afebrile, although the intermittent hemoptysis continued. On the $63^{rd}$ hospital day, a massive hemoptysis (about 800 mL) developed, which could not be controlled despite embolizing the left bronchial artery. He died of respiratory failure the next day. It is believed that the oxalic acid produced by A. niger was the main cause of the patient's pulmonary injury and the ensuing massive hemoptysis.

• Journal of dental hygiene science
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• v.13 no.4
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• pp.493-500
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• 2013

The study aims to determine the status of oral health of mental illness patients and establish the preliminary data. The examinations and questionnaire survey were done 92 psychiatric patients to measure sociodemographic characteristics, decayed, missing and filled teeth (DMFT) index, patient hygiene performance (PHP) index, community periodontal index of treatment need (CPITN). Result of the missing teeth index in the state of oral health was higher in the hospital group (6.42) while the filled teeth index was higher in the center group (4.78). In the DMFT index, mental illness patients were higher than the national sample. The oral health status of medical aid recipients was poorer as the subjects were older and less educated (p<0.05). The PHP index was 3.41, close to the bad oral hygiene state. The hospital group (81.7%) required higher need for periodontal treatment. The periodontal health state was much poorer especially when the subject was in the age of 40's and 60's, received less education, and had no family (p<0.05). $CPITN_3$ was higher in the hospital group (13.3%) than the national sample (5.7%). The mental illness patients were socially vulnerable, therefore oral health care program should be needed and age, education level, health insurance type, presence of family and other factors needs to be considered in this approach.

• Journal of Oral Medicine and Pain
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• v.30 no.1
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• pp.57-67
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• 2005

The aim of this study was to evaluate bone scan(SUR) and SPECT(hot spot) in patients with TMJ osteoarthritis. 99mTc-MDP bone scan and SPECT were done in 27 patients, total 54 temporomandibular joints were examined with clinical examination, plain radiography. 42 TMJs were osteoarthritis and 12 TMJs were normal case, clinically diagnosed. We compared osteoarthritis group and control group of bone scan(SUR) and SPECT(hot spot). thus we compared before and after treatment of bone scan(SUR) and bone SPECT(hot spot) according to history, contributing factor, symptom degree, treatment, treatment period. The obtained results were as follows. 1. Bone scan(SUR) and SPECT(hot spot) activity of osteoarthritis group were higher than control group(P<0.05, P<0.01). 2. Bone scan(SUR) and SPECT(hot spot) activity were decreased after treatment at TMJ with osteoarthritis(P<0.01). 3. Bone scan(SUR) and SPECT(hot spot) activity were decreased at treatment group with splint(P<0.01, P<0.05). 4. Bone scan(SUR) and SPECT(hot spot) activity were decreased at Chronic group(P<0.01, P<0.05). 5. Bone scan(SUR) and SPECT(hot spot) activity were decreased at low noise NAS(<6) group(P<0.01,P<0.05). 6. Bone scan(SUR) and SPECT(hot spot) activity were decreased at TMD patient with parafunctional habit and without trauma history, psychosocial factor. 7. Bone scan(SUR) and SPECT(hot spot) activity were higher at before treatment required more treatment period.

• Journal of Oral Medicine and Pain
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• v.37 no.3
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• pp.161-167
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• 2012

Burning mouth syndrome(BMS) is defined as chronic, painful burning sensation in the oral mucosa. Treatments for BMS include medication and psychiatric interventions. Capsaicin, alpha-lipoic acid, and topical and systemic clonazepam showed more effective in reducing the symptoms of BMS in the previous studies. The purpose of this study is to evaluate of the therapeutic efficacy of systemic clonazepam in BMS and to elucidate the relationships between such a efficacy and various clinical features, including age, pain intensity, pain duration, previous dental history and condition of oral mucosa. A retrospective clinical records audit was performed of patients diagnosed with BMS between January 2011 and August 2012. Patients were prescribed 0.5 mg clonazepam two times daily. Pain was assessed by patients on an 11-point numeric rating scale (NRS; 0 to 10) before and 1-2 weeks after systemic administration of clonazepam. The efficacy of clonazepam was evaluated in terms of patient's age, initial pain intensity, pain duration, presence or absence of precipitating event, condition of the tongue, presence or absence of denture. A total of 50 patients (46 women, 4 men) were included in this study. The patients were divided into two or three groups according to above clinical features. The amount of mean NRS reduction in patients with severe initial pain was $3.33{\pm}2.74$, whereas that in patients with mild initial pain was $1.64{\pm}1.54$. The amount of mean NRS reduction in oldest patients was $3.53{\pm}1.94$ (${\geq}$70yrs), and those in another younger patients were $2.88{\pm}1.80$(< 60yrs) and $1.54{\pm}2.86$(60yrs ${\leq}$ age < 70yrs), respectively. It was concluded that the older patients and the patients with higher intensity of initial pain tend to show better efficacy of clonazepam. However, There were no statistically significant differences according to pain duration, presence or absence of precipitating events, tongue fissuring, and wearing dentures.