• Clinical and Experimental Pediatrics
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• v.46 no.5
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• pp.440-446
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• 2003

Purpose : The genetic disturbance of galactosemia is expressed as a cellular deficiency of either galactose-1-phosphate uridyltransferase(GALT) or galactokinase(GALK) or UDP galactose 4-epimerase(GALE). To find-out the pattern of galactosemia in Korea, we retrospectively analyzed cases of galactosemia detected by neonatal screening program. Methods : We analyzed medical records of patients who visited Soonchunhyang University Hospital at age of 1 month after showing abnormalities in neonatal screening of galactosemia. For accurate diagnosis, galactose was measured by enzyme immunoassay(EIA) and fluorophotometer, also galactose-1-phosphate by fluorophotometer. Enzyme activities of GALK, GALT and GALE in RBC and galactose-1-phosphate were measured by radioisotope assay(RIA). Beutler test were done. Patients went on a lactose-free diet and follow-up tests for galactose, galactose-1-phosphate level and enzyme activity were performed. Results : 10 patients(male : 6, female : 4) were diagnosed as galactosemia. Two patients had GALK deficiency and two had GALT deficiency. Six were GALE deficient showing the largest number. In two patients with GALK deficiency, GALT and GALE activities were normal but GALK activities showed respectively reduced activity. For GALT deficiency, two patients had low GALT activity in RBC and showed genotype of Duarte 2/G(galactosemia) in DNA analysis. In one patient, GALT activity was normal. Three patients seemed to be heterozygote state of GALE deficiency according to GALE activity levels. Four patients showed GALK hyperactivity. Conclusion : GALE deficiency provided the highest number. After lactose-free diet, galactose and galactose-1-phosphate were normaly maintained. Neonatal screening on galactosemia is essential for preventing life-threatening symptoms and an accurate diagnosis is needed for finding out the type of galactosemia which is important for prognosis.

• Clinical and Experimental Pediatrics
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• v.52 no.8
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• pp.898-903
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• 2009

Purpose : This study investigated the predictive factors for identifying infection-prone febrile infants younger than three months. Methods : We conducted a retrospective study of 167 infants younger than three months with an axillary temperature >$38^{\circ}C$ who were hospitalized between 2006 and 2008. If they met any of the following criteria, positive blood culture, CSF WBC ${\geq}11/mm^3$ or positive CSF culture, urinalysis WBC ${\geq}6$/HPF and positive urine culture, WBC ${\geq}6$/HPF on microscopic stool examination or positive stool culture, they were considered at high risk for severe infection. Infants with focal infection, respiratory infection or antibiotic administration prior to admission to the hospital were excluded. We evaluated the symptoms, physical examination findings, laboratory data, and the clinical course between the high risk and low risk groups for severe infection. Results : The high-risk group included 77(46.1%) infants, and the most common diagnosis was urinary tract infection (51.9%). Factors, such as male sex, ESR and CRP were statistically different between the two groups. But, a multilinear regression analysis for severe infection showed that male and ESR factors are significant. Conclusion : We did not find the distinguishing symptoms and laboratory findings for identifying severe infection-prone febrile infants younger than three months. However, the high-risk group was male and ESR-dominated, and these can possibly be used as predictive factors for severe infection.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.13 no.2
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• pp.146-153
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• 2010

Purpose: Recent studies have reported an increase in the incidence of community-acquired Clostridium difficile-associated disease (CA-CDAD) among children. There is an overall lack of information on CA-CDAD in the pediatric population. The aim of our study was to compare the epidemiologic and clinical features between CA-CDAD and hospital-acquired C. difficile-associated disease (HA-CDAD) in children. Methods: We retrospectively reviewed the medical records of all patients who were diagnosed with C. difficile-associated disease (CDAD) at Gil Hospital between April 2008 and March 2009. The diagnosis of CDAD was made when patients with gastrointestinal symptoms had positive results for C. difficile toxins A and B assay or stool culture. Results: Sixty-one (male, 32 and female, 29) patients were included. The mean age was 3.79${\pm}$4.54 years. Of the 61 patients, 22 (36.1%) were <1 year of age. Twenty-three patients (37.7%) had a history of antibiotic exposure in the previous 3 months. Forty-one patients (67.2%) were diagnosed with CA-CDAD. There were no significant differences in age, gender, symptoms, laboratory findings, recovery period, complications, and recurrence between the CA-CDAD and HA-CDAD groups. On the other hand, exposure to antibiotics was significantly more frequent among patients in the HA-CDAD group (p=0.005). Conclusion: This study suggests that the occurrence of CA-CDAD is increasing in the pediatric population, especially in younger children with no history of exposure to antibiotics and in outpatients. Awareness of the increasing incidence of CA-CDAD and prompt investigation of C. difficile in susceptible patients is needed to avoid misdiagnosis and for appropriate therapy.

• Childhood Kidney Diseases
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• v.6 no.1
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• pp.61-67
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• 2002

Purpose: The urinary mass screening program in school aged population has been performed since 1981, but the consensus on the follow-up schedule and the management of isolated proteinuria has not been reached yet. The aim of this study was to investigate the cause of isolated proteinuria and to propose a guideline for the treatment and follow-up afterwards Methods: The medical records of 114 cases of isolated proteinuria detected through the analysis of urinary mass screening and evaluated at the pediatric outpatient clinic of Asan Medical Center from January 1990 to July 2001 have been reviewed. Results: The classification of isolated proteinuria was as follows. Transient proteinuria $32\%$, orthostatic proteinuria $65\%$, persistent proteinuria $3\%$, In orthostatic proteinuria group, daytime and nighttime proteinuria were $319.2{\pm}189.1\;mg/dL$ and $56.5{\pm}56.1\;mg/dL$. In persistent proteinuria group, daytime and nighttime proteinuria were $1140{\pm}540.5\;mg/dL$ and $289{\pm}58\;mg/dL$. After 30 month follow-up, 2 cases of persistent proteinuria were needed renal biopsy and 1 case revealed focal segmental glomerular sclerosis. In all cases, serum creatinine, albumin and complements levels were normal. In the orthostatic proteinuria group, no significant renal diseases were detected. Conclusion: Since most of the isolated proteinuria detected through the school urinary mass screening were orthostatic proteinuria or transient proteinuria, initially aggressive diagnostic method such as renal biopsy is not needed and regular follow- up with quantitation of proteinuria is warranted.(J Korean Soc Pediatr Nephrol 2002 ; 6 61-7)

• Journal of Chest Surgery
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• v.35 no.10
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• pp.705-711
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• 2002

The advantages of mitral valve reconstruction have been well established and so mitral valve reconstruction is now considered as the procedure of choice to correct mitral valve disease. This is the report of intermediate-term results of 38 cases that performed mitral valve reconstruction for valve insufficiency(the total number of mitral valve reconstruction were 49 cases, but 11 cases that performed mitral valve replacement due to incomplete reconstruction were excluded). Material and Method : From March 1991 to March 2001, 38 patients underwent mitral valve repair due to mitral valve regurgitation with or without stenosis. Mean age was 47.6$\pm$14.7 years(range 15 to 70 years) : 11 were men and 27 were women. The causes of mitral valve regurgitation were degenerative in 14, rheumatic in 21, infective in 2 and the other was congenital. Result : According to the Carpentier's pathologic classification of mitral valve regurgitation, 3 were type 1 , 16 were type II and 19 were type III. Surgical procedures were annuloplasty 15, commissurotomy 19, leaflet resection and annular plication 9, chordae shortening 11, chordae transfer 5, new chordae formation 2, papillary muscle splitting 2 and vegetectomy 2. These procedures were combined in most patients. There were 2 early death and the causes of death were respiratory failure, renal failure and sepsis. There was no late death. Valve replacement was done in 6 patients after repair due to valve insufficiency or stenosis 3 weeks, 1, 3, 51, 69, 84months later respectively. These patients have been followed up from 1 to 116 months(mean 43.0 months). The mean functional class(NYHA) was 2.36 pre-operatively and improved to 1.70. Conclusion : In most cases of mitral valve regurgitation, mitral valve reconstruction when technically feasible is effective operation that can achieve stable functional results and low surgical and late mortality.

• Korean Journal of Psychosomatic Medicine
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• v.25 no.1
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• pp.73-85
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• 2017

Objectives : In this study I made investigations how 'strange hunger' and 'compulsive overeating' threatening the ego could be resolved and healed. And I aim to present a healing model of psychotherapy and analysis as one of methods of treatment for 'eating disorder'. Methods : The analysands of this study were outpatients who visited the department of psychiatry of Yong-In Mental Hospital from March 2008 to February 2017 with 'hunger' and 'compulsive overeating' as their chief complaints. This study is based on the detailed records of the process of analysis including dreams and visions. Results : 1) Throughout the process of analysis that explore both consciousness and unconsciousness(dream, vision), hunger and compulsive overeating is improved and healed in all analysands. 2) The Imago of 'Snake' appeared in dreams and visions of all analysands. 3) By suffering impulse rather than acting it out, impulse transformes itself into 'Imago'. As impulse transforms into 'Imago' and reveals the 'meaning' of it, ego-threatening power of impulse weakens and mood is calmed. And as a result, synthesis of consciousness and unconsciousness and creative transformation of personality can be possible. Conclusions : In some people, 'hunger' and 'compulsive overeating' are 'creative impulses' that aim 'Self-realization' which can be fruited as creative transformation of personality and as creative transformation in the relation with the world. 'Creative impulses', which often can be experienced as instinctive impulse or emotional suffering unless ego realizes the meaning, reveal the meaning in dreams or visions through 'Imago' and 'Symbol'.

• Korean Journal of Psychosomatic Medicine
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• v.26 no.1
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• pp.1-8
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• 2018

Objectives : This study aimed to investigate the psychosocial characteristics of functional gastrointestinal disorder (FGID) in patients with psychiatric disorders. Methods : This study was conducted with 144 outpatients visiting the psychiatric clinic at a university hospital. FGIDs were screened according to the Rome III questionnaire-Korean version. Demographic factors were investigated, and psychosocial factors were evaluated using the Hospital Anxiety Depression Scale, Patient Health Questionnaire-15, Childhood Trauma Questionnaire-Korean, and State-Trait Anger Expression Inventory. Chisquared test and student's t-test were used as statistical analysis methods. Results : There were differences in education level between two groups divided according to FGID status (${\chi}^2=10.139$, p=0.017). Comparing the psychiatric disorder by FGID group, irritable bowel syndrome (IBS) group showed significant differences (${\chi}^2=11.408$, p=0.022). According to FGID status, IBS group showed significant differences for anxiety (t=-3.106, p=0.002), depressive symptom (t=-2.105, p=0.037), somatic symptom (t=-3.565, p<0.001), trait anger (t=-3.683, p<0.001), anger-in (t=-2.463, p=0.015), and anger-out (t=-2.355, p=0.020). Functional dyspepsia group showed significant differences for anxiety (t=-4.893, p<0.001), depressive symptom (t=-3.459, p<0.001), somatic symptom (t=-7.906, p<0.001), trait-anger (t=-4.148, p<0.001), state-anger (t=-2.181, p=0.031), anger-in (t=-2.684, p=0.008), and anger-out (t=-3.005, p=0.003). Nonerosive reflux disease group showed significant differences for anxiety (t=-4.286, p<0.001), depressive symptom (t=-3.402, p<0.001), somatic symptom (t=-7.162, p<0.001), trait anger (t=-2.994, p=0.003), state anger (t=-2.259, p=0.025), anger-in (t=-2.772, p=0.006), and anger-out (t=-2.958, p=0.004). Conclusions : Patients with psychiatric disorders had a high prevalence of FGID, and various psychosocial factors contributed to such differences. Therefore, the psychiatric approach can offer better understandings and treatments to patients with FGID.

• Journal of Hospice and Palliative Care
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• v.11 no.1
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• pp.42-50
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• 2008

Purpose: Undertreatment of canter pain, especially due to the differences in the perception of pain between the patients and caregivers, is a well recognized problem. The purpose of this study were to determine if there exist differences in communication about pain intensity scores between patients and their family caregivers in Korea. Methods: A total of 127 patient-family caregiver dyads who have experienced canter pain participated in this study at a hospital in Seoul for six months. The data were obtained by fare to face interview with a structured questionnaire based on Brief Pain Inventory-Korean version and other previous researches. The clinical information for all patients was compiled by reviewing their medical records. Results: Patients' 'worst-pain for 24-hour' and 'right-now-pain' scores estimated by family caregivers were significantly higher than those by patient themselves. The degree of agreement between patients and family caregivers in the estimate of patients' 'worst-pain for 24-hour' intensity categories was 78.7% for 'severe pain', 40% for 'no pain', 27.5% for 'mild pain' and 22.9% for 'moderate pain'. In case of 'right-now-pain' intensity categories, the agreement was 50% for 'severe pain', 47.2% for mild pain, 46.3% for 'no pain', and 26.3% for 'moderate pain'. Conclusion: This study demonstrates that the degree of agreement between patients and family caregivers in the estimate of patients 'pain intensity categories was less than 50% except for 'severe pain'. The results indicate that Korean family caregivers tend to overestimate the canter pain intensity of their caring patients, especially, when a lancer patient has 'moderate' or 'mild pain'. Health Providers are advised to educate patient-family caregiver dyads to use a pain measurement scale to promote their agreement in pain Intensity stores. Further analyses and studies are needed to identify the factors and differences that influence their communication about pain intensity scores between patients and their family caregivers.

• Clinical and Experimental Pediatrics
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• v.46 no.8
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• pp.811-816
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• 2003

Purpose : Kawasaki disease is an acute systemic vasculitis of unknown etiology with a predilection for the coronary arteries. Vascular endothelial growth factor(VEGF) is a cytokine which promotes vascular permeability and angiogenesis. We investigated serum VEGF(sVEGF) levels in Kawasaki disease to determine whether sVEGF level can be used as a risk factor to predict the occurrence of coronary artery lesions(CAL) in Kawasaki disease. Methods : We measured sVEGF levels in 11 patients with Kawasaki disease in acute phase(patient group)and 11 normal children(control group) by enzyme-linked immunosorbent assay(ELISA) method. We investigated the relationship between sVEGF levels and the lumen diameters of coronary artery and other potential CAL risk factors; duration of fever, hemoglobin, WBC counts, platelet counts, ESR, CRP and LDH levels. Results : SVEGF levels of patients in the acute phase of Kawasaki disease(mean $847.9{\pm}495.7pg/mL$) were significantly higher than that of normal controls(mean $279.9{\pm}150.6pg/mL$; P<0.05). SVEGF levels showed significant positive correlation with the lumen diameters of the coronary artery(P<0.05, $r_s=0.75$) in the patient group. There was no significant correlation between sVEGF levels and duration of fever or other laboratory measurements. Conclusion : Our results support the notion that sVEGF level may be considered as a predictive indicator for the occurrence of coronary artery lesions in Kawasaki disease.

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.410-416
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• 2006

Purpose : Ciliary abnormalities of the respiratory system usually accompany recurrent or persistent respiratory diseases such as paranasal sinusitis, bronchiectasis, rhinitis, and/or otitis media, since they cause certain derangements in ciliary cleaning activities. This disease is usually inherited by autosomal recessive trait, but may also be found to be acquired or transient in rare cases after heavy exposure to pollutants, cigarette smoking or severe infection. We performed this study in children with frequently recurrent or persistent respiratory diseases to clarify if the ciliary abnormalities are preceding factors. Methods : We enrolled 17 children with suspected respiratory ciliary abnormalities. The indications for evaluation of ciliary ultrastructure were recurrent or persistent respiratory infections. Children with immunologic abnormalities were excluded. From August 2000 to July 2003, we performed a biopsy on nasal mucosa and examined the structure of ciliary status by using an electron microscope. Results : Of the subjects, there were seven males and 10 females, aged 2 to 10 years. Out of the 17 subjects, 12 cases of chronic paranasal sinusitis, nine chronic coughs, nine frequent upper respiratory infections, seven cases of recurrent otitis media, four cases of recurrent pneumonia, and four cases of bronchial asthma were found. Out of the 17 cases on which histologic examinations were conducted, four cases showed pathologic findings, including one case of inner dynein arm defect, one of microtubular transposition, one of supernumerous tubules, and one singlet, respectively. Conclusion : It is essential for differential diagnosis and effective treatment to identify the abnormalities of ultrastructure of nasal cilia in children with symptoms of frequently recurrent or persistent respiratory diseases, if immunodeficiency or respiratory allergy could be excluded.