• 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.

• Tuberculosis and Respiratory Diseases
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• v.42 no.1
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• pp.19-24
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• 1995

Background: Although pulmonary tuberculosis is effectively controlled with 6 months or 9 months short course standard regimens, comparable numbers of treatment failures ensued because of inadequate treatment mainly due to patient's poor compliance. Indequate treatment with standard regimens during initial treatment may cause emergence of drug resistance and prolong the duration of chemotherapy. Also it may make the patient lesser compliant and finally increase the morbidity and the mortality. Methods: A clinical study was done to evaluate clinical and bacteriological characteristics of 94 patients who were retreated for pulmonary tuberculosis. Results: 1) 62 of the 94 patients were male and 32 patients were female. Mean age is 51 years old in male and 45 years old in female. 2) The extent of the disease on the chest radiograph was minimal in 10(11.1%) patients, moderate in 31(33.3%) patients, and far advanced in 52(55.6%) patients. 3) On sputum bacteriologic examination, 73(77.7%) patients were positive in sputum AFB smear and/or culture for Mycobacterium tuberculosis. 4) Results of drug sensitivity test performed in 42 patients showed that the resistance to one drug is in 9(20.5%) patients, two drugs in 18(40.8%) patients, and more than three drugs in 14(31.8%) patients. 5) Poor patient's compliance was the leading cause of the retreatment of pulmonary tuberculosis(43.6%) 6) Only 24(25.5%) patients of the 94 retreatment patients were successfully treated and 39(41.6%) patients were dropped out during follow-up. Conclusion: We concluded that poor patient's compliance was the most important cause of treatment failure not only in primary treatment patients but also in retreatment patients. Primary treatment of pulmonary tuberculosis should be completed under strict monitoring of the patient because significant number of retreatment patients had multiple drug resistance and poor outcome.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.103-108
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• 2017

Kabuki syndrome is a rare congenital disorder that causes multiple birth defects and mental retardation. Mutation of the lysine methyltransferase 2D (KMT2D) gene is the primary cause of Kabuki syndrome. We report a 4-year-old Korean girl diagnosed with Kabuki syndrome based on distinctive facial features (eversion of the lower lateral eyelid, arched eyebrows, depressed nasal tip, prominent ears), skeletal anomalies, short stature, and molecular analysis, which revealed a novel frameshift mutation in the KMT2D gene. A 4-year-old patient had a past history of congenital cardiac malformations (coarctation of the aorta, ventricular septal defect, atrial septal defect, patent ductus arteriosus), subclinical hypothyroidism and dysmorphic features at birth including webbed neck, short fingers, high arched palate, micrognathia and horseshoe kidney. She showed unique facial features such as a long palpebral fissure, long eyelashes, arched eyebrows with sparseness of the lateral third, broad nasal root, anteverted ears, and small mouth. Her facial features suggested Kabuki syndrome, and genetic analysis discovered a novel heterozygous frameshift mutation (c.4379dup, p.Leu1461Thrfs*30) in exon 15 of the KMT2D gene. The diagnosis of our 4-year-old patient was made through thorough physical examination and history taking, and genetic testing. It is challenging to diagnose patients with Kabuki syndrome at birth, since the characteristic facial features are expressed gradually during growth. Clinical suspicion aroused by regular follow-ups may lead to earlier diagnosis and interventions.

• Research in Plant Disease
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• v.16 no.1
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• pp.27-34
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• 2010

This study was performed to develop a formulation using an antagonistic bacterium Bacillus amyloliquefaciens A-2 to control tomato leaf mold caused by Fulvia fulva. B. amyloliquefaciens A-2 was grown in a medium with rice oil and mixed with various carrier and additives. One of the formulations, A2-MP, showed the best disease control value among the tested formulations. The disease control value of A2-MP at 100-fold and 500-fold diluted treatment was not significantly different from that of chemical fungicide triflumizole in a growth chamber. Although disease control effect was decreased by serial diluted treatment of the prepared A2-MP, 1,000-fold diluted treatment of A2-MP still showed high disease control value of 72.0%. For the green house experiments, the disease control values of A2-MP was indicated as 79.4% which is similar to that of chemical fungicide, triflumizole showing 79.6%. When the disease control activity of the formulation A2-MP was compared in tomato production conditions, disease control values of 100-fold diluted A2-MP and 3,000 fold diluted triflumizole exhibited 60%, 81.6%, respectively. The disease control efficiency by A-2MP was 73% of the disease control value of chemical fungicide. The formulation A-2MP maintained the stable bacterial viability and disease control activity when stored at $4^{\circ}C$. This result suggested that A-2MP develped from B. amyloliquefaciens A-2 could be used to control tomato leaf mold.

• The Korean Journal of Nuclear Medicine Technology
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• v.14 no.1
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• pp.61-66
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• 2010

Purpose: Recently, Department of Nuclear Medicine have an interest in Video Display Terminals (VDT) syndrome including musculoskeletal disorders, ophthalmologic disorders, trouble of electromagnetic waves and stress disorders occur to VDT workers as the growing number of users and rapid pace of service period supply in large amount. This study research on the actual condition for VDT syndrome in Nuclear Medicine, Seoul National University Hospital (SNUH), discover the problem and draw a plan of upcoming improvement. The aim of this study establish awareness about VDT syndrome and is to prevent for it in the long run. Materials and Methods: Department of Nuclear Medicine, SNUH is composed Principle part, Pediatric part and PET center. We estimated risk factors visit in each part directly. Estimation method use "Check list for VDT work" of Wonjin working environment health laboratory and check list is condition of VDT work, condition of work tables, condition of chairs, condition of keyboards, condition of monitors, working position, character of health management and other working environment. Analysis result is verified in Department of Occupational and Environment, Hallym University Sacred Heard Hospital. Results: As a result of analysis, VDT condition of Department of Nuclear Medicine, SNUH is rule good. In case of work tables, recent of things are suitable to users upon the ergonomical planning, but 15% of existing work tables are below the standard value. In case of chairs are suitable, but 5% of theirs lost optimum capacity become superannuated. The keyboards are suitable for 98% of standard value. In case of monitors, angle control of screen is possible of all, but positioning control is impossible for 38%. In case of working position, 10% is fixed positioning for long time and some of the items researched unsuitable things for standard. At health management point, needed capable of improvement. Also, other working condition as lighting, temperature, noise and ventilation, discovered the problem, but is sufficient to advice value. Conclusion: VDT syndrome is occurrences of possibility continuously, come economical expensive about improvement, is inherent in various causes and originate without your knowledge. So, there is need systematic management system. In Nuclear Medicine, VDT syndrome make it better that constant interest and effort as improvement of ergonomical working environment, improvement of working procedure, regular exercise and steady stretching, and can be prevented fairly. This promote physical and mental condition of worker in top form in comfortable working environment, so this is judged by enlargement of operations efficiency and rising of satisfaction ratings of the inside client.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.213-221
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• 2005

Purpose: Hemophagocytic syndrome (HPS) is characterized by persistent high fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, and/or hypofibrinogenemia. Hepatic manifestations including overt hepatic failure and fulminant hepatitis are common in HPS. Liver transplantation (LT) should be considered in a case of fulminant hepatitis by other than HPS, but LT is contraindicated and complete cure is possible by chemotherapy in HPS. Therefore, we conducted this study to define the characteristics of HPS presenting as severe acute hepatitis. Methods: Among the total of 23 patients diagnosed as HPS by bone marrow examination between 1994 and 2005 in Asan Medical Center, 11 cases presented as severe acute hepatitis were enrolled in this study. We analyzed the clinical features, laboratory findings and outcome retrospectively. Results: Seven (64%) of the 11 children with HPS and hepatitis were referred to pediatric gastroenterologist at first. The mean age of onset was 50 months. There was no case with family history of primary HPS. Epstein-Barr virus was positive in 4, and herpes Simplex virus was positive simultaneously in 1 case. As the presenting symptoms and signs, fever was present in 10, hepatosplenomagaly was noted in all and jaundice in 10. Anemia was observed in 10, thrombocytopenia in 10, leukopenia in 8, hypertriglyceridemia in 9, hypofibrinogenemia in 8 and hyperferritinemia in 7 cases, respectively. Nine children received chemotherapy including etopside. The overall mortality rate was 72% (8/11). Conclusion: HPS, which needs chemotherapy, should be considered as a cause of severe acute hepatitis especially when accompanied with prolonged high fever and cytopenias.

• Journal of Nutrition and Health
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• v.41 no.1
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• pp.41-53
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• 2008

Gestational diabetes mellitus (GDM) is defined as glucose intolerance discovered or onset during pregnancy and attention is needed because of increased risk of perinatal morbidties and higher incidence of diabetes afterward. This study was performed to identify dietary factors associated with the development of gestational diabetes mellitus (GDM). Developed food frequency questionnaire containing 192 food items were used to assess nutritional status of 246 control subjects and 104 GDM subjects. Food habits of subjects were examined in the questionnaire. The more irregularity and less variety of meal were found in GDM group compared to control group and GDM group tended to eat rapidly and do not like vegetables and greasy foods. Total score of food habits in GDM was lower than control group, which suggests that GDM group have undesirable food habits. Mean daily energy and carbohydrate intakes of GDM group were higher than those of control group, and percent energy from protein was significantly higher in control. Nutrient density of protein, calcium, phosphorus and vitamin BI of GDM group was significantly lower than those of control group. Therefore dietary quality of GDM group was lower than that of control group. Odds ratio for GDM was high when energy and carbohydrate intakes were high. And when the intakes of rice, noodle, shiruduk, hamburger, boiled egg, steamed pork shank were high, the odds ratio for GDM was high. These results indicate that the amount and frequencies of several foods and dish items were related with the occurrence of GDM subjects. On the whole, GDM subjects consumed more cereals and less vegetables and less legumes. From these results, pregnant women with GDM tended to have unhealthy food habits, and carbohydrate intake was important dietary factors on the onset of GDM.

• Journal of Korean Orthopaedic Sports Medicine
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• v.6 no.2
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• pp.110-114
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• 2007

Purpose: This study evaluate clinical findings & management of rhabdomyolysis after strenuous activities in military police recruit. Materials and Methods: This study was carried out from June $1^{st}$, 2004 and May $23^{nd}$, 2005. The study subjects were 13 military police recruit patients who were admitted to our hospital with intractable muscle pain and swelling, and had suspicions of Rhabdomyolysis. The patients were given various blood tests (CPK, CK-MB, AST, BUN/Cr, and Electrolyte) and clinically observed. The patients were all males, and their average age was 20 $(19\sim21)$ years. Seven cases were due to push-up exercises, 5 was due to a soccer game, and 1 was due to riot control activities. The patients complained of swelling and tenderness in various parts of the extremities. Four complained of swelling and tenderness in forearm, 3 in upper arm, 1 in shoulder, and 5 in lower extremity. The diagnosis of rhabdomyolysis was made if the patient complained clinical symptom and had a blood CPK level of above 1,000 IU/L at the time of admission. Patients who took medication or had medical problem were excluded from this study. Bone scans were taken of all patients 4 hours after giving 99mTc-MDP 20mCi intravenously. Treatment was bed rest and fluid therapy. Patients who complained of excessive pain were given splint immobilization. Results: The average hospitalization day for the 13 patients was 20 days ($14\sim42$ days). Excluding one patient who exhibited ARF at time of admission, all patients showed a decrease of blood CPK below 1000 IU/L at an average hospitalization time of 8 days ($2\sim11$ days). The patient with ARF recovered after hemodialysis and fluid therapy. Conclusion: Patients complaining of swelling and severe muscle pain after excessive exercise or training should be suspicious of exercise induced rhabdomyolysis, and should be given blood tests and fluid therapy immediately.

• Pediatric Infection and Vaccine
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• v.5 no.1
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• pp.69-78
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• 1998

Purpose : The risk of severe tuberculous disease such as meningitis or miliary tuberculosis increases as younger is the child at the time of infection. Therefore, the early diagnosis and prompt treatment is mandatory for infants with tuberculosis. This study was undertaken to describe the epidemiology, clinical and radiographic manifestations, and response to therapy in infants with tuberculous disease. Methods : Medical records of 29 infants with tuberculosis diagnosed at the Seoul National University Children's Hospital from July, 1985, to April, 1997, were reviewed, retrospectively. A case of tuberculosis was confirmed if M. tuberculosis was isolated from any body site or if there was histologic proof of tuberculosis. Otherwise, the diagnoses were individualized considering history of contact with contagious adult case, clinical manifestations, chest X-ray findings, result of a Mantoux test reaction with 5 tuberculin unit of PPD, and the response to therapy. Results : The mean age at diagnosis was $7.00{\pm}2.65$ months (range, 3 to 12 months). Twelve cases had isolated pulmonary diseases, and the rest had pulmonary disease and meningitis, 5 cases; pulmonary disease and cervical lymphadenitis, 3; isolated meningitis, 3; and miliary tuberculosis, 6. Source case was identified in 19 cases, 7 of which were detected with retrograde manner. Twenty seven of 29 were symptomatic at their initial visit. The presenting symptoms were mainly respiratory or neurologic, and respiratory difficulty was accompanied in 7 cases. Physical examination revealed wheezing in 7 cases and decreased breath sounds in 9. Hepatomegaly or hepatosplenomegaly were frequent. Chest radiographs showed lung parenchymal disease with hilar lymphadenopathy in 18 cases, and focal or generalized emphysematous change in 7 cases. Conclusion : Most of the infants with tuberculosis are symptomatic at diagnosis, and many of infants with intrathoracic tuberculosis presented with symptoms of bronchial obstruction. When tuberculosis is suspected in an infant, the adult source case should be vigorously investigated to aid in diagnosis and for the prevention of further transmission of tuberculous disease. Almost half of infant tuberculosis are preventable if prophylaxis were given when adult cases were diagnosed.

• Research in Plant Disease
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• v.21 no.3
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• pp.201-207
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• 2015

This study was conducted to establish a simple mass-screening method for resistant melon to Fusarium wilt caused by Fusarium oxysporum f. sp. melonis (FOM). Root-dipping inoculation method has been used to investigate resistance of melon plants to Fusarium wilt. However, the inoculation method requires a lot of labor and time because of complicate procedure. To develop a simple screening method on melon Fusarium wilt, occurrence of Fusarium wilt on susceptible and resistant cultivars of melon according to inoculation method including root-dipping, soil-drenching, tip, and scalpel methods was investigated. Scalpel and tip methods showed more clear resistant and susceptible responses in the melon cultivars than root-dipping inoculation method, but tip method represented slightly variable disease severity. In contrast, in the case of soil-drenching inoculation method, disease severity of the susceptible cultivars was very low. Thus we selected scalpel method as inoculation method of a simple screening method for melon Fusarium wilt. By using the scalpel inoculation method, resistance degrees of the cultivars according to incubation temperature after inoculation (25 and $30^{\circ}C$) and inoculum concentration ($1{\times}10^6$ and $1{\times}10^7conidia/ml$) were measured. The resistance or susceptibility of the cultivars was hardly affected by all the tested conditions. To look into the effectiveness of scalpel inoculation methods, resistance of 22 commercial melon cultivars to FOM was compare with root-dipping inoculation method. When the melon cultivars were inoculated by scalpel method, resistance responses of all the tested cultivars were clearly distinguished as by root-dipping method. Taken together, we suggest that an efficient simple mass-screening method for resistant melon plant to Fusarium wilt is to sow the seeds of melon in a pot (70 ml of soil) and to grow the seedlings in a greenhouse ($25{\pm}5^{\circ}C$) for 7 days, to cut the root of seedlings with a scalpel and then pour a 10 ml-aliquot of the spore suspension of $1{\times}10^6conidia/ml$ on soil. The infected plants were cultivated in a growth room at 25 to $30^{\circ}C$ for about 3 weeks with 12-hr light a day.