• Clinical and Experimental Pediatrics
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• v.51 no.6
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• pp.650-654
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• 2008

Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which result from a deficiency in specific enzymatic activities and the accumulation of partially degraded acid mucopolysaccharides. A case of a 16-month-old boy who presented with hepatomegaly is reported. The liver was four finger-breadth-palpable. A laboratory study showed slightly increased serum AST and ALT levels. The liver biopsy showed microscopic features compatible with GSD. The liver glycogen content was 9.3% which was increased in comparison with the reference limit, but the glucose-6-phosphatase activity was within the normal limit. These findings suggested GSD other than type I. Bony abnormalities on skeletal radiographs, including an anterior beak and hook-shaped vertebrae, were seen. The mucopolysaccharide concentration in the urine was increased and the plasma iduronate sulfatase activity was low, which fulfilled the diagnosis criteria for Hunter syndrome (MPS type II). To the best of the authors' knowledge, this is the first case of GSD and Hunter syndrome being identified at the same time.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.159-166
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• 2005

Purpose : Recently the merits of 6 weeks of initial prednisolone treatment for pediatric primary nephrotic syndrome have been reported, and the use of the 6 week regimen is increasing. We compared our experiences with the 6 week treatment versus the 4 week treatment for Korean patients. Methods : We conducted a retrospective analysis of 69 children who had primary nephrotic syndrome and who were followed up for at least 12 months in the 4 major medical centers in Daegu. The remission rate, the relapse rate, the frequency of relapse and complication of steroid treatment were compared between the 4 weeks and 6 weeks treatment group. Results : Of the 69 children, 42 were in the 4 week treatment group and 27 were in the 6 week group. The median age, blood pressure, serum total protein, serum albumin, cholesterol, creatinine, estimated creatinine clearance, 24 hour urine protein and 12 month cumulative dose did not differ between the two groups. Among the children who relapsed after steroid treatment, the relapse time was significantly later for the 6 week treatment group. The relapse rate after 1 year of treatment was 62$\%$ in the 4 week treatment group and 52$\%$ in the 6 week treatment group; however, there was no statistically significant difference between the two groups. The frequency of relapse at 12 months was $1.5{\pm}1.2$ times in the 4 week treatment group and $1.1{\pm}1.2$ times in the 6 week treatment group, and there was not different between the two groups. The most common side effects of steroid treatment were an increase of appetite and a cushingoid appearance, and there was no statistical difference between the two groups. Among the 27 children who had kidney biopsies performed, 21 suffered from minimal change nephrotic syndrome. Conclusion : The first relapse time after steroid treatment was significantly later in the 6 week steroid treatment group. The frequency of relapse and the 12 month cumulative dose of steroid were lower in the 6 week treatment group, but there was no statistical significance between the two groups. The side effects of steroid treatment did not differ between the two groups. We need to study the long term side effects and the advanced regimens of steroid treatment in the future.(J Korea Soc Pediatr Nephrol 2005;9:159-166)

• Clinical and Experimental Pediatrics
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• v.48 no.7
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• pp.760-765
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• 2005

Purpose : We studied the changes in antibiotic sensitivity to the causative organisms of urinary tract infection(UTI), in order to provide useful information on the choice of adequate drugs in the treatment of UTI. Methods : We retrospectively analyzed the major causative organisms and their antibiotic sensitivities in 69 patients diagnosed with UTI in the Department of Pediatrics, Samsung Cheil Hospital from 2002 to 2003. Results : The frequency of UTI was the highest in infants younger than 1 year of age(88.4 percent). The male to female ratio was 3.05 : 1. Escherichia coli was the most frequent organism(78.3 percent), followed by Klebsiella(11.6 percent), Pseudomonas(2.9 percent), Proteus(2.9 percent), Enterobacter, Morganelle, and Enterococcus(1.4 percent) in descending order. Antibiotic sensitivity of gram negative organisms was above 90 percent against imipenem, amikacin, 80 percent against aztreonam, cefepime, ceftriaxone, 50-70 percent against gentamicin, trimethoprime-sulfamethoxazole (TMP/SMX), and 23 percent against ampicillin(23.4 percent). Conclusion : Antibiotict sensitivity of gram negative organisms was high to amikacin and third generation cephalosporins but low to ampicillin, gentamicin and TMP/SMX. The use of ampicillin or TMP/SMX, as the first choice of the empiric and prophylactic treatment for UTI, should be reconsidered and investigated further.

• The Korean Nurse
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• v.35 no.2
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• pp.71-91
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• 1996

The objectives of thes study were to identify the need for equipment development in nursing, and to determine the priorities for that development. The study was descriptive study done between March 2 and May 30, 1995, in which the subjects, including 421 patients, 223 family members, and 198 nurses from neurosurgery, orthopedic, rehabilitation medicine, internal medicine and intensive care units of nine general hospitals in Seoul, completed a questionnarie developed by the research team. The questionnaire consisted of 35 open and closed questions. Data was analyzed using frequencies and percentages. The results ware summarized as follows: 1) The average age of the nurses was 27.9 years, 48% of the patients were between 20 and 40 years of age, and 17% were over 60. The average lingth of experience for the nurse subjects was four years five months with 36.9%. having over five years experience. The most frequent diagnoses of patients were spinal disc(35.9%), internal medicine disease(26.0%), cerebral vascular accident(16.6%) and spinal cord injury(10%) 2) Many of the nurses(96.4%) reported deficiencies with existing equipment and 96.5% of the nurses, but only 79.8% of the patients, nurses' time. Further, 82.3% of the nurses and 75.8% of the patients felt that the development of new equipment would lead to a decrease in the cost of nursing care. 3) Nurses felt that the greatest areas of inconvenience were patient feeding(71.7%), hygiene(71.2%), caring for a patient confined to bed(70.7%), patient clothing(67.2%), mobility transfers(63.5%) and urinary elimination(52.0%). However, patients and family members listed the following as being the most inconvenient: urinary elimination(58.7%), Hygiene(50.5), feeding(48.4%), mobility transfers(47.1%) and bed care(45.2%). 4) Generally the nurses listed more inconveniences and patients and family members listed more demands for the development of equipment. These included utensils with large handles, and regulators for tube feedings; mattresses that provide for automatic position change and massage, which have patient controlled levers and a place for bed pan insertion; automatic lifts or transfer from bed to wheelchair; equipment to facilitate washing and oral hygiene as well as equipment that will allow patients with spinal cord injuries easy access to showers; a bed pan/urinal for women that is comfortable and effective from which urine can be measured and disposed of easily; disposable dressing sets and tracheostomy care sets and a convenient way of measuring changes in wound size; a safe delivery system for oxygen, a variety of mask sizes and better control of humidity, tracheal material than at present, as well as a communication system for patients with tracheostomies; clothing that will allow access to various parts of the body for treament or assessment without patients having to remove all of their clothing; and finally a system that will allow the patient to control lighting, telephones and pagers. Priority areas for equipment development reported by the nurses were, urinary elimination(58. 7%), hygiene(50.5%), feeding(48.4%), mobility transfers(47..1%), bowel elimination(40.8%). Those reported by the patients family members were feeding(71.7%), hygiene(70.0%), bedcare(70.7%), clothing(67.2%), mobility transfers(63.6%), urinary elimination(52.9%) and bowel elimination(50.5%) Altogether, nurses, patients and family members listed the following as priorities; clothing (178), bed care(144), urinary elimination(92), environment(81), hygiene(70). Further, a health professional forum listed urinary elimination, oxygen delivery, medication delivery, mobility transfers, bed care and hygiene in that order as priority areas. From this study it can be concluded that the first need is to develop equipment that will address the problems of urinary elimination. To do (l)This nurses who are interested in equipment development should organize an equipment development team to provide a forum for discussion and production of equipment for nursing.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.1
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• pp.16-23
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• 2004

Purpose: In this study, we tried to evaluate the clinical characteristics or circumstances that lead to unintentionally the delay in the diagnosis of intussusception or to the wrong direction that prevent the proper management early. Methods: All the patients of intussusception with delayed diagnosis in the department of pediatrics or emergency room at Gyeongsang National University Hospital from 1990 to 2003 were enrolled and reviewed retrospectively. Results: There were 8 boys and 6 girls and their median age was 8 months (range 2 months to 10 years). Their initial symptoms and signs were vomiting, seizure, diarrhea, lethargy, irritability, bloody stool, palpable abdominal mass, foul odor of urine and tachycardia. Clinical diagnosis or impressions at admission consisted of acute gastroenteritis, shigellosis and toxic encephalopathy, convulsive disorders, urinary tract infections, sepsis, abdominal mass and intestinal obstruction. Eight patients were luckily diagnosed due to the delayed manifestations of cyclic irritability or currant jelly stool. Six patients were not paid attentions for the possibilities of intussusception and diagnosed serendipitiously by the abdominal sonography or CT during the evaluation of the abdominal mass or distension. Only five of 14 cases (35.7%) were successfully managed by barium or air reductions. The other 9 cases needed surgical operations. Conclusion: Delayed diagnosis of intussusception arise when doctors initially diagnose the patients incorrectly due to the unusual presentations or when they overlook the newly arising symptoms or signs suggestive intussusception after the admission because they are ardently attached to the first impressions or initial clinical diagnosis.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.24-31
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• 2007

Purpose : Nitric oxide(NO) is a very potent vasodilator synthesized from L-arginine by endothelial cells. We investigated whether urinary NO excretion was altered in various renal diseases in children and whether urinary NO excretion could be used in predicting pathologic causes and fibrosis in renal diseases in children. Methods : We recruited 48 patients(32 minimal change nephrotic syndrome[MCNS] and 16 vesicoureteral reflux[VUR] patients from the pediatric renal clinic in Korea University Guro Hospital. We measured the concentration of nitrite$(NO_2)$ and nitrate$(NO_3)$ by Griess reaction and that of creatinine(Cr) by Jaffe method in randomized spot urines. We then analyzed the urinary$(NO_2+NO_3)/Cr$ ratios and compared the values between each patient group. Urinary $(NO_2+NO_3)/Cr$ ratios were also evaluated according to the recurrence and the degree of proteinuria at sampling in the MCNS group and compared according to the presence of renal scarring and the grade of reflux in the VUR group. Results : The ratios of urinary$(NO_2+NO_3)/Cr$ were significantly increased in the VUR and MCNS groups, as compared to the control group. In the MCNS group, a higher level of urine $(NO_2+NO_3)/Cr$ was observed In frequent relapse patients(relapse over four times within one year after first diagnosis) and the patients with severe proteinuria at sampling, respectively. The VUR group with renal scars also showed a higher level of urinary$(NO_2+NO_3)/Cr$ compared to that without scars. Conclusions : In summary, VUR may play a role in the pathogenesis of VUR and MCNS. NO also seems to affect proteinuria and renal scar formation. (J Korean Soc Pediatr Nephrol 2007;11:24-31)

• Pediatric Infection and Vaccine
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• v.17 no.1
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• pp.23-29
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• 2010

Purpose : Urinary tract infection (UTI) in children is the most common disease during the infantile period, therefore early diagnosis and treatment are important. Pyuria is a useful clinical parameter for the initial diagnosis of a UTI. In this study we aimed to compare the clinical, laboratory, and imaging findings in relation to the duration of pyuria in infants with UTIs. Methods : Three hundred seventy-four infants <12 months of age who were admitted between January 1995 and December 2005 for the first episode of a febrile UTI were retrospectively reviewed. Patients were divided into two groups according to the duration of pyuria as follows: group 1, pyuria resolved <3 days after initial treatment; and group 2, pyuria lasted at least 3 days after initial treatment. Results : There were no significant differences between the two groups in relation to gender, age, total duration of fever, and organisms in the urine. Group 2 had a significantly higher peripheral blood leukocyte count ($14,360.86{\pm}5,526.16cells/mm^3$ vs. $11,822.55{\pm}5,687.26cells/mm^3$, P <0.001), erythrocyte sedimentation rate ($32.81{\pm}19.34mm/hr$ vs. $23.74{\pm}20.43mm/hr$, P <0.001), and C-reactive protein ($6.84{\pm}5.68mg/dL$ vs. $3.78{\pm}3.99mg/dL$, P <0.001) than group 1. There was a significantly higher incidence of hydronephrosis and a higher grade of vesicoureteral reflux (VUR) in group 2 compared to group 1. Conclusion : In infants with UTI, pyuria of longer duration is related to severe UTI and higher grade VUR, therefore aggressive radiologic studies may be necessary.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.247-254
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• 2007

Purpose : Continuous renal replacement therapy(CRRT) has been the first choice for the treatment of acute renal failure in critically ill children not only in western countries but also in Korea. However, there are very few studies that have analyzed the outcome and prognosis of this modality in Korean children. We performed this study to evaluate the factors associated with the outcome and prognosis of patients treated with CRRT. Methods : We retrospectively reviewed the medical records of 32 children who had received CRRT at Severance hospital from 2003 to 2006. The mean age was 7.5 years(range 4 days-16 years) and the mean body weight was 25.8 kg (range 3.2-63 kg). Results : Eleven(34.4%) of the 32 patients survived. Bone marrow transplantation and malignancy were the most common causes of death and underlying disease leading to the need for CRRT Mean patient weight, age, duration of CRRT, number of organ failures, urine output, estimated glomerular filtration rate(eGFR), C-reactive protein, and blood urea level did not differ significantly between survivors and nonsurvivors. (1) Pediatric risk of mortality(PRISM) III score at CRRT initiation($9.8{\pm}5.3$ vs. $26.7{\pm}7.6$, P<0.0001), (2) maximum pressor number ($2.1{\pm}1.2$ vs. $3.0{\pm}1.0$, P=0.038), and (3) the degree of fluid overload($5.2{\pm}6.0$ vs. $15.0{\pm}8.9$, P=0.002) were significantly lower in survivers than in nonsurvivors. Multivariate analysis revealed that fluid overload was the only independent factor reducing survival rate. Conclusion : CRRT was successfully applied to the treatment of acute renal failure in a wide range of critically ill children. To improve survival, we suggest the early initiation of CRRT to prevent the systemic worsening and progression of fluid overload in critically ill children with acute renal failure. (J Korean Soc Pediatr Nephrol 2007;11:247-254)

• Journal of the Korea Academia-Industrial cooperation Society
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• v.17 no.4
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• pp.188-198
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• 2016

This study examined the effects of a smoking cessation coaching program based on the motivation stage applying the Transtheoretical Model to stop the smoking of patients in terms of the amount of smoking, nicotine dependence, CO level, and urine cotinine. The study design was a multi-repeat multiple repeat intermittent time series study with one-group, a pre-post design. The participants were 47 smoking patients (44 males and 3 females), who were treated at a public hospital in N city. The participants were 4 (8.51%) subjects in the action stage and 43 subjects (91.49%) in the preparation stage of the motivation stage. The coaching program intervention was conducted at the first day, second week, and 6th week. The smoking cessation maintenance of the subjects was checked at the 12th week. A chi-square test and t-test were used to analyze the data. The subjects in the action stage were kept under the condition of no smoking and nicotine dependence. After the program of subjects in the preparation stage, the amount of smoking, nicotine dependence, and CO level were significantly lower compared to the pre-test (p<.001). The findings suggest that the coaching program based on the motivation stage was effective in improving the smoking cessation for patients who smoke. On the other hand, the patients in the smoking cessation program may require much more financial assistance than those of healthy people. A greater workforce and budget will be needed for patients to stop smoking.

• Proceedings of the Korean Society of Food Hygiene and Safety Conference
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• 2004.11a
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• pp.59-70
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• 2004

A randomized double-blind placebo-controlled study was conducted on 63 subjects to determine the antihypertensive effect of a vegetable drink in which sardine protein hydrolysates containing a dipeptide, Valyl-Tyrosine (VY), were incorporated. The subjects, consisting of people with mild hypertension, high-normal blood pressure and normal blood pressure, were randomly divided into test (male/female=25.6, average age 50.1${\pm}$10.4 years old) and control groups (26/6, 49.0${\pm}$5.0). Each subjects in the test group was given 195g of the vegetable drink containing 0.5g of sardine peptides (sardine protein hydrolysates) with 0.4 mg of VY (test drink) once a day for 13 weeks in a row, and subjects in the control group were given the same amount of the vegetable drink without sardine peptides (control drink) in the same manner. In the test group, 40 subjects with mild hypertension of high-normal blood pressure (130 mmHg${\leq}$systolic blood pressure (SBP)<160 mmHg and/or 80 mmHg${\leq}$diastolic blood the start of the test to 134.4${\pm}$11.1 mmHg during the first week of the test period, after which similar values were seen throughout the test period (13 weeks). Compared to the control group, the difference in SBP from vaseline was statistically significant in the test group throughout the intake period. DBP also decreased significantly from 88.0${\pm}$7.9 mmHg at baseline to 83.5${\pm}$8.6 mmHg after 13 weeks. In the control group, SBP and DBP were 140.8${\pm}$8.4 mmHg and 90.5${\pm}$6.6 mmHg respectively at the start of the test, and neither decreased during the test period. In subjects with normal blood pressure, neither those in the test group nor those in the control group showed a significant change in SBP and DBP during the test period. An excessive ingestion test was performed on 25 subjects with hypertension, mild hypertension, high-normal blood pressure, and normal blood pressure by giving 585g (3 times the recommended amount of intake) of the test drink for 14 days in a row. As a result, a significant decrease of blood pressure was observed in the hypertension, mild hypertension and high-normal blood pressure groups, but no excessive decline in blood pressure or any side-effects were associated with any subjects during the test period. In the groups with normal blood pressure, the excessive ingestion of the test drink did not affect blood pressure. In these two studies, physical check-ups and biochemical analyses of blood and urine were also conducted in all subjects, and no abnormalities were observed. These results suggest that the test drink containing sardine protein hydrolysates exhibited the antihypertensive effect in only the subjects with mild hypertension or high-normal blood pressure. No adverse effects were observed in either hypertensive of normotensive subjects.