• Childhood Kidney Diseases
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• v.2 no.1
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• pp.77-81
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• 1998

The author exprienced a case of glycogen storage disease type Ia(GSD-I) in an 18-year-old male patient who was admitted to our hospital due to proteinuria and hypertension. he was suspected to have GSD when 12 years old because of his family history of short stature and hepatomegaly. On admission, physical examination revealed short stature, heparomegaly, and The diagnosis of GSD-I was confirmed by compatible liver biopsy finding and enzyme assay which erealeddeficiency of glcose-6-phosphatase if hepatocyte. Sympromatic treatment was done using antihypertensive drugs and allopurinol with diet control. The authors report a case of glycogen storage disease type Ia completely confirmed by typical clinical manifestation, pathologic findings of the liver and the kidney, and the result of enzyme assay which revealed deficiency of glucose-6-phosphatase in hepatocytes with brief review fo related literatures.

• Journal of the Korean Society of Food Science and Nutrition
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• v.44 no.2
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• pp.216-225
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• 2015

The purpose of this study was to evaluate night time eating habits, dietary habits, and nutrient intake in university students according to residence type. A survey was conducted by administering questionnaires to 664 students. Questionnaire interview and 24-h dietary recall were conducted. Subjects were divided into three groups according to residence type: dormitory boarding (DB group, N=313), self-boarding (SB group, N=246), and living with parents (LWP group, N=105). Average ages in the DB, SB, and LWP groups were 21.3, 22.2, and 22.1 years, respectively. There were no significant differences in body mass index between the three groups. In total, 77.3% of students regularly ate night time snacks. The proportion of students who reported night time eating was 84.0% in the DB group, 73.6% in the SB group, and 65.7% in the LWP group (P<0.001). In terms of food types consumed during night time eating, the DB group showed a significantly higher rate of consumption of fried chicken and flour-based foods than the SB and LWP groups, whereas the SB group showed a significantly higher rate of consumption of alcohol beverages than the DB and LWP groups. Energy, carbohydrates, protein, fat, vitamins, and mineral intakes were significantly higher in the DB group than in the SB and LWP groups. In addition, intake of cholesterol per 1,000 kcal was significantly higher in the DB group than in the SB and LWP groups. Thus, SB and DB students seemed to have more night time eating problems than LWP students. Accordingly, nutritional education is needed to support the development of healthier eating habits, in particular, night time eating habits, among students living in dormitories and in self-boarding situations.

• Pediatric Infection and Vaccine
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• v.13 no.1
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• pp.71-77
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• 2006

Purpose : Shigellosis is still an important cause of acute food-borne diarrheal diseases throughout the world. We investigated the transmission routes and clinical course through the outbreak of shigellosis in Mapo-Gu, Seoul. Methods : From October 7th to November 19th in 2005, Mapo-Gu public health center had referred 32 patients with confirmed or suspected shigellosis to the National Medical Center. We had investigated source of infection, epidemiology, laboratory findings, and clinical course of the cases occurred during this outbreak. Results : Among 32 patients, 24 patients had been confirmed with shigellosis, 8 patients had been diagnosed with suspected shigellosis. They ranged in age from 5 months to 12 years old and their mean age was 6.5 years. The clinical manifestations were as follows; diarrhea, fever, abdominal pain and asymptomatic condition. Symptoms had sustained for 3.7 days on the average. S. sonnei were cultured by rectal swab and founded to be resistant to ampicillin and TMP/SMX except to 3rd generation cephalosporin. After treatment with antibiotics such as cefixime and ceftriaxone or imipenem and conservative treatment with electrolyte and fluid replacement for 5~7 days, Stool cultures of the rectal swab grew no Shigella in these cases except 3 cases. Conclusion : An outbreak of shigellosis had occurred in a preschool and elementary school children. From the same results of antimicrobial susceptibility and pulsed-field gel electrophoresis patterns in this study, we suggest that the outbreak of shigellosis in this report had been originated from a single strain. According to all negative results about suspected food and water cultures, we couldn't find out source of infection. Through materials offerred by Mapo-Gu Public Health Center, we presumed the trasmission routes probably were person-to-person.

• Clinical and Experimental Pediatrics
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• v.48 no.6
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• pp.624-633
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• 2005

Purpose : This study analyzed the expression of HLA-DR and DQ genotypes and anti-thyroid autoantibodies[anti-thyroid peroxidase(TPO) and anti-thyroglobulin(TG) antibodies] in Korean patients with type 1 diabetes(T1DM) to investigate the susceptible HLA alleles to T1DM in Korea and the prevalence of thyroid autoantibodies and their significance for the development of thyroid disorders. Methods : A total of 59 Korean patients with type 1 diabetes[26 males, median age 13.7 years(range 5.7-29.9 years), diabetes duration 7.6 years(-1.7-22.5 years)] were enrolled in this study, and 200 healthy Koreans without a family history of diabetes were selected as a normal control for the comparison of HLA genotypes. Seventeen patients with anti-TPO or anti-TG were followed [median duration 3.96 years(1 day-10.7 years)] with measurement of anti-TPO, anti-TG, $T_3$, $T_4$ or free $T_4$, TSH levels and physical examinations. HLA-DR and DQ genotyping were done by PCR-SSO, PCR-SSCP, PCR-RFLP and PCR-SSP methods. Results : HLA analysis showed higher frequencies of HLA-DRB1*0301, *090102 and DQB1*0201, *030302 alleles, DRB1*0301/*090102, *090102/*090102 and DQB1 *0201/*030302, *030302/*030302, *0201/ *0302 genotypes in T1DM patients compared to controls(Pc<0.05). Fifteen(25.4 percent) had anti-TPO antibody, 12(20.3 percent) had anti-TG, 17(28.8 percent) had either autoantibody and 10(16.9 percent) had both autoantibodies. No clinical or subclinical hypothyroidism developed during follow-up after the first detection of anti-thyroid autoantibody. There was no significant correlation between thyroid autoimmunity and gender, onset age of T1DM, and diabetes duration, respectively(P>0.05). Conclusion : We thought this unique HLA-DR, DQ allele distribution might be an important factor for the low incidence of T1DM in Korea. And a high prevalence of thyroid autoantibodies in these populations suggests examinations of thyroid antibodies should be performed regularly. Optimal age for the initial screening and the frequency of re-screening for associated thyroid autoimmune diseases in T1DM remains to be determined through prospective follow-up.

• Clinical and Experimental Pediatrics
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• v.51 no.3
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• pp.299-306
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• 2008

Purpose : Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disorder characterized by fever, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow and other tissues. In this study, we investigated the clinical manifestations and prognostic factors in patients with HLH. Methods : We retrospectively analyzed the data from 29 patients who were diagnosed whit HLH in the Severance Children's Hospital from Jan. 1996 to Feb. 2007. Results : The median age at diagnosis was 3.8 years (range 0.1-12.2). The ratio of male to female patients was 1.1:1. The 5-year overall survival rate was 55.2% with a median follow-up duration of 32 months. In a multivariate analysis, the duration of fever before admission (survival vs. non-survival, 6.5 days vs. 14 days, P=0.010), the interval from the day of fever onset to the day of initiation of etoposide (survival vs. non-survival, 10 days vs. 35 days, P=0.002) and the presence of neurologic symptoms (survival vs. non-survival, 1 case vs. 7 cases, P=0.010) were independent, poor prognostic factors of HLH. EBV infection, gender, and the level of serum ferritin had no relations to the poor prognosis of the disease. Conclusion : This study showed that the presence of neurologic symptoms and a longer duration of fever were related to a poor prognosis. Therefore, if a patient develops neurologic symptoms and the duration of fever is prolonged, a prompt diagnostic approach and aggressive treatment for HLH are necessary.

• Journal of Nutrition and Health
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• v.42 no.6
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• pp.523-535
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• 2009

The aim of this study was to examine and compare the eating habits and dietary intake patterns of people with or without allergy by the survey during August-September, 2008. The 131 subjects aged 10'-50' (AG = allergy group, n = 62; NG = non-allergy group, n = 69) participated in this study. The questionnaire included general characteristics, dietary habits, and food frequency. Income level tended to be higher in AG than in NG, and AG had more pets and flower pots than NG (p < 0.01). In AG, most affected area of allergy was the skin and the subjects in AG experienced the mixed symptoms and more than 2 types of allergy. Family history of allergy was highly related with allergy of the subjects (AG: 66.1%, NG: 33.9%). Both groups did not exercise regularly, but frequency of alcohol drinking in AG was significantly higher than in NG (p < 0.01). AG skipped meals and had snacking more often than NG (p < 0.05). Most favorite snacks in AG were instant foods, fast foods, cookies, and ice cream (p < 0.05). Therefore, AG tended to consume more allergy-related foods than NG. Highly-consumed allergy-related foods were egg, pork, walnut, onion, tuna, shellfish, and kiwi (p < 0.05). Therefore, nutrition education and guidance is needed to establish good eating habits, food intakes, and life style in people having allergy.

• Korean Security Journal
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• no.9
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• pp.99-131
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• 2005

Terrorism which became today's common phenomena over the world is one of the most serious threats the world confront. Although International society make and operate outstanding anti-terrorism system, terror would never end without solving fundamental problems. The main body of terrorism converts from nation to organization and from organization to cell, which makes it difficult for us to recognize the main body. Since the target of today's new terrorism is many and unspecified persons, terrorists will never hesitate to use mass destruction weapons such as nuclear, biological, chemical weapons, and also use cyber-technique or cyber-terrorism. So, effective counter-terrorism measures should be performed as follows. First, it must be better for international society should make long-time plan of solving fundamental problems of terrorism other than to operate directly on terror organization and its means. Second, preventive method should be made. The most effective method of eradicating terrorism is prevention. For this, it is necessary to remove environmental elements of terrorism and terrorist bases, and to stop inflow of money and mass destruction weapons to terrorists. Third, integrated anti-terror organization should be organized and operated for continuous counter-terrorism operations. Also international alliance for anti-terrorism should be maintained to share informations and measures. Fourth, concerned department in the government should prepare counter-terrorism plans in their own parts as follows and make efforts to integrate the plans. - Ministry of Government Administration and Home Affairs : conventional terror - Ministry of Health and Welfare : bio-terror - Ministry of Science and Technology : nuclear-terror Especially, they should convert their policy and operation from post-terror actions to pre-terror actions, designate terror as national disaster and organize integrated emergency response organization including civil, government, and military elements. In conclusion, pre-terror activities and remedy of fundamental causes is the best way to prevent terror. Also, strengthening of intelligence activities, international cooperations, and preventive and comprehensive counter-measures must not ignored.

• Journal of Genetic Medicine
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• v.5 no.1
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• pp.26-33
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• 2008

Purpose : Phenylketonuria(PKU) is an inborn error of metabolism and a genetic disorder resulting from a deficiency of phenylalanine hydroxylase(PAH) and decreased activity of tetrahydrobiopterin(BH4).In this study the correlation between the DNA mutation and clinical manifestations was investigated and PAH DNA mutations were compared bewteen Asian and Caucasian populations. Methods : DNA was isolated from peripheral leukocytes. The PAH gene was amplified by Polymerase Chain Reaction(PCR) and the sequence was analyzed with Multiplex Ligation-dependent Probe Amplification(MLPA). Results : We characterized the PAH gene of 102 independent Korean patients with PKU. PAH nucleotide sequence analysis revealed 44 different mutations, including 10 novel mutations comprising 9 missense mutations(N207D, K95del, A447P, G344D, P69S, S391I, A202T, G103S, and I306L) and 1 novel splice-site variant mutation(IVS10-3C>G). R243Q was the most prevalent mutation in this study. A259T has not previously been reported in Asian populations, but we found that this mutation had a frequency of 10.1% in our study. Furthermore, the genotypes of $BH_4$ responsive patients were analyzed and were divided into two groups: $BH_4$ medication-only group and $BH_4$ medication with diet therapy group. In the $BH_4$ medication-only group and $BH_4$ medication with diet therapy group, R241C was the most common mutation. Conclusion : Novel mutations in the PAH gene of PKU patients are still being discovered. Additional information as to the frequency of mutations in the tetrahydrobiopterine responsive gene is also accumulating. We anticipate that knowledge of these PKU gene mutations will assist the diagnosis, genetic counseling, and therapeutic treatment of PKU patients in future.

• Clinical and Experimental Pediatrics
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• v.50 no.5
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• pp.436-442
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• 2007

Purpose : To promote awareness and efforts by pediatricians to identity and prevent child abuse by investigation of characteristics of victim and types of injury caused by abuse. Methods : A retrospective study was performed with 20 patients who had been diagnosed or suspected as child abuse at Hallym University Hospital from January 1999 to December 2005. The medical records, radiologic documents, and social worker's notes were reviewed to investigate age, sex, visiting time, form of abuse, perpetrator, risk factor, and type of injury. Results : The mean age of the subjects was 2.8 years. Fourteen patients were between 0-1 years old, 2 patients between 1-6 years old, 3 patients between 7-12 years old, and 1 case over 13 years old. The ratio of male to female was 1:1. The majority of these patients (70%) visited via emergency department. Eight five percent of these patients reported with physical abuse, 5% psychological abuse, 5% sexual abuse, and 5% neglect respectively. The suspected perpetrator was the biological father in six cases, the biological mother in three cases, the stepmother in two cases, caregiver in one case, relatives in one case and "unknown" in six cases. Bruise and hematoma (80%) were the most common physical findings. Skull fractures were diagnosed in six cases, long bone fractures in two cases, hemoperitoneum in two cases, subdural hemorrhage in 10 cases, epidural hemorrhages in two cases, subarachnoidal hemorrhages in two cases, and retinal hemorrhages in five cases respectively. Seventeen cases required hospitalization and surgical operations performed were in nine cases. Four patients died and three patients had sequalae such as developmental delay and quadriplegia. Conclusion : Child abuse results in high mortality and morbidity in victims. Therefore early recognition and prevention is very important. Pediatricians should always suspect the possibilities of abuse in cases of fracture, intracranial hemorrhage, abdominal injury, or even any injury to the body. We recommend that the clinical investigation of suspicious children should include a full multidisciplinary social assessment, a skeletal survey and CT or MRI.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.74-82
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• 2007

Purpose : To analyze the clinical characteristics, spontaneous resolution rate and predictive factors of resolution in children with primary vesicoureteral reflux(VUR). Methods : Between October 1991 and July 2003, 149 children diagnosed with primary VUR at Chonbuk National University Hospital were reviewed retrospectively. All of the patients were maintained on low-dose antibiotic prophylaxis and underwent radionuclide cystograms at 1 year intervals over 3 years after the initial diagnosis of VUR by voiding cystourethrogram was made. Results : The median time to resolution of VUR was 24 months and the total 3 year-cumulative resolution rate of VUR was 61.7%. The following variables were associated with resolution of VUR according to univariate analysis-; age<1 year, male gender, mild grade of reflux, unilateral reflux, congenital hydronephrosis as clinical presentation at time of diagnosis of VUR, absence of focal defects in the renal scan at diagnosis, absence of recurrent UTI, renal scars and small kidney during follow-up. After adjustment by Cox regression model, five variables remained as independent predictors of VUR resolution; age<1 yew, relative risk 1.77(P<0.05), VUR grade I+II 2.98(P<0.05), absence of renal scars 2.23(P<0.05), and absence of small kidney 5.20(P<0.01) during follow-up. Conclusion : In this study, spontaneous resolution rate of VUR, even high grade reflux, is high in infants during medical management, and it was related to age, reflux grade at diagnosis, absence of renal scars and small kidney during follow-up. Therefore early surgical intervention should be avoided and reserved for the selected groups.