• Clinical and Experimental Pediatrics
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• v.53 no.4
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• pp.592-597
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• 2010

Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency disease which is characterized by high serum IgE levels, eczema, and recurrent infections. Herein we present the case of a patient with HIES associated with STAT3 gene ($stat3$) mutation. A 16 year-old girl was admitted to our hospital due to hemoptysis caused by pneumonia with bronchiectasis. She had a history of recurrent skin and respiratory tract infections, such as pneumonia caused by MRSA (methicillin-resistant $Staphylococcus$ $aureus$) and $Pseudomonas$ $aeruginosa$. On physical examination, a broad round shaped nose, oral thrush, and chronic eczematous skin rash over her whole body were found. Laboratory data showed an elevated eosinophil count ($750/{\mu}L$) and total IgE level (5,001 U/mL). The patient's National Institutes of Health (NIH) score for HIES was 44. Direct sequencing of the STAT3 gene revealed that the patient was heterozygous for a missense mutation in the DNA binding domain of the STAT3 protein (c.1144C>T, p. Arg382Trp). HIES should be suspected in patients with recurrent infections and can be confirmed by clinical scoring and genetic analysis.

• Journal of the Korean Society of Food Science and Nutrition
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• v.35 no.3
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• pp.344-352
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• 2006

The purpose of this study was to evaluate whether a daily regimen of green vegetable drink supplementation to male subjects improves the blood pressure and enhances other antioxidant enzyme status, lipid profiles and liver functionalities. Twenty nonsmokers and 19 smokers aged $23{\sim}60$ were given 240 mL of green vegetable drink every day for 8 weeks in addition to their normal diet, and blood samples were drawn before and after the intervention. The 8 weeks of green vegetable drink consumption resulted in a significant decrease of diastolic blood pressure in smokers (p<0.05) as well as in nonsmokers (p<0.01), and systolic blood pressure in smokers (p<0.05). Erythrocyte superoxide dismutase (SOD) activity was significantly increased in both nonsmokers and smokers after the trials. Plasma total cholesterol (TC) level was decreased in smokers and plasma TC and LDL-cholesterol (LDL-C) levels were decreased in nonsmokers while triglyceride (TG) and HDL-cholesterol (HDL-C) was not affected in both nonsmokers and smokers by green vegetable drink consumption. Plasma glutamic pyruvic transaminase (GPT), a marker of liver function was significantly reduced in both nonsmokers and smokers and plasma glutamic oxaloacetic transaminase (GOT) level was significantly reduced in nonsmokers after the supplementation. These results support the hypothesis that green vegetable drink exerts a blood pressure reducing effect and affects not only the activity of antioxidant enzymes, lipid profiles but also plasma GOT and/or GPT levels.

• Pediatric Infection and Vaccine
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• v.12 no.1
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• pp.61-66
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• 2005

Purpose : Since hepatitis A virus almost is transmitted by fecal-to-oral route, individual and public sanitation affects the prevalence and ages of hepatitis A infection. We researched the positivity rate of hepatitis A antibody at Pohang to make the basic data for subclinical infection and vaccination schedule. Methods : From January 2004 to February 2005, a total of 603 patients who were admitted at Hangdong University Sunlin Hospital, Dongguk University Hospital, and Christianity Hospital without any hepatic disease and vaccination of hepatitis A were enrolled. IgG antibody to hepatitis A virus were measured by electrochemiluminescence immunoassay(ECLIA). Results : Among 603 patients, 523 patients were less than 15 year of age and 80 patients were in above 15 years. The prevalence rate was 19.3%(101/523) in pediatric group and 70.0%(56/80) in above 15 years. In detail, the prevalence rate was 73.2%(52/71) in 0~5 months, 14.9%(11/74) in 6~11 month, 8.9%(7/79) in 12~17 month, 7.3%(6/82) in 18~23 month, 5.5%(4/72) in 2~3 years, 12.1%(9/74) in 4~8 years, 16.9%(12/71) in 9~14 years, 52.5%(19/40) in 15~29 years, and 92.5%(37/40) in group aged over 30 years. The prevalence rates in male and female showed no significant differences. Conclusion : The prevalence rate of hepatitis A in the group of 4~8 years and 9~14 years at Pohang was lower comparing with previous reports of other cities in Korea. We can postulate that the sanitation of children living at Pohang is at least not bad than other cities. And for the prevalence rate of hepatitis A is increased after 3 years, we should recommend that the vaccination of hepatitis A may be finished until 3 years.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.203-211
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• 2007

Purpose : We attempted to compare the independent factors such as age, sex, C-reactive protein(CRP), and white blood cell count(WBC) in children with radiologic studies and assess the necessity of performing voiding cystourethrography(VCUG). Method : 98 children who have been diagnosed their first time febrile urinary tract infection from Janurary 2002 to Januray 2005 were enrolled. In all patient, the duration of fever which occurred before and after treatment was recorded, and CRP, WBC, $^{99m}Tc$-2,3-dimercaptosuccinic acid($^{99m}Tc$-DMSA) renal scans, renal ultrasound and VCUG were analyzed. Results : Of the 98 children diagnosed with urinary tract infection(UTI), 52 were male and 46 were female. 18 had abnormalities in VCUG, 17 had abnormalities in kidney ultrasound, and 20 had partial defects or diffuse uptake decrease in $^{99m}Tc$-DMSA renal scans. There were no significant relationship between incidence of radiologic abnormalities and age. The risk of renal scar was significantly higher in children who had a longer febrile period before treatment than in those with shorter period. Both CRP and WBC were significantly elevated in children with the radiological abnormalities. A positive of $^{99m}Tc$-DMSA renal scans and renal ultrasound were highly associated with vesicoureteral reflux(VUR). Conclusion : If there are abnormalities in the kidney ultrasound and $^{99m}Tc$-DMSA renal scan of a child with initial UTI, a VCUG is recommended. Even in cases without abnormal findings in $^{99m}Tc$-DMSA renal scan and renal ultrasound, clinical data such as CRP and WBC should be assessed, and VCUG should be Performed for the undetected VUR.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.62-69
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• 2008

Purpose: The most concerning issue in children with urinary tract infection(UTI) is the probability of underlying genitourinary anomalies and vesicoureteral reflux (VUR), which is frequently associated with renal scarring and eventually end-stage renal disease. Therefore, voiding cystourethrography(VCUG) is usually recommended at the earliest convenient time for children with UTI. However, VCUG is an invasive procedure that requires catheterization and exposure to X-ray. In this study, we aimed to determine the predictability of clinical, laboratory and imaging parameters for VUR in children with UTI. Methods: Data of children with bacteriologically proven UTI who underwent VCUG were evaluated retrospectively for clinical(age, gender, fever), laboratory(leukocytosis, ESR, CRP, pyuria, blood urea nitrogen, serum creatinine) and imaging(renal ultrasound and DMSA renal scan) findings. First, children with UTI were divided into two groups according to the presence of VUR as non-VUR group and with VUR group, and clinical, laboratory variables were compared between these groups. Second, patients who had VUR were reclassified as low-grade VUR(grade I-II) group and high-grade(grade III-V) VUR group according to grading of VUR, and clinical, laboratory and imaging variables were compared between these groups. Results: Among 410 children with UTI, 137 had VUR and 78 high-grade VUR. Fever, leukocytosis, ESR, CRP, pyuria were associated with VUR. In addition, abnormal findings of ultrasonography and DMSA renal scan were closely related to VUR. However, these clinical and laboratory variable in patients with high grade VUR were not different significantly, compared to those with low-grade VUR group. Conclusion: Fever, leukocytosis, ESR, CRP seems to be potentially useful predictors of VUR in pediatric patients with UTI. In addition, renal ultrasonography and DMSA renal scan findings supported the presence of VUR. Further study of these findings could limit unnecessary VCUG in patients with UTI.

• Journal of Chest Surgery
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• v.37 no.1
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• pp.35-42
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• 2004

Background: For the treatment of isolated left main coronary artery disease, twelve arterial revascularizations with Y-composite grafts using left internal thoracic artery and radial artery or right gastroepiploic artery were peformed. This study was performed to investigate whether V-composite graft can satisfy the blood flow required to make myocardium act properly or not. Borderline stenotic lesions on the left main coronary artery, which are very prone to remodel the bypassed vessels due to competitive flows, were also considered. Material and Method: Among 247 patients who underwent coronary artery bypass grafting from March 2000 to April 2003, 12 patients (4.7%) who had received total arterial revascularizations for the isolated left main coronary artery disease were studied retrospectively. Result: left anterior descending arteries were bypassed with left internal thoracic artery by off-pump technique in all patients, however, 2 cases of left obtuse marginal branches were bypassed under on-pump beating heart. Except for one patient, who did not have an obtuse marginal branch more than 1 mm in diameter, 11 patients had gone through complete arterial revascularizations by use of the Y shape arterial graft. Among five patients who had less than 75% stenosis, one patient showed string sign on left internal thoracic artery grafted to left anterior descending artery. However, two grafts to obtuse marginal blanches were completely obstructed and one showed slender sign. There were no graft-dominant flow in patients with stenotic lesion less than 75%. On the contrary to the result of patients with stenotic lesions less than 75%, all the patients with stenotic lesions more than 90% showed graft-dominant blood flow. Conclusion: In conclusion, it is assumed that, when stenotic lesions are over 90%, coronary artery bypass grafting with an Y shape arterial graft could possibly give enough help to the obstructed coronary arteries in blood supplying to myocardium, which needs massive quantity of blood to act well. However, when patients have borderline stenoses, through scrupulous examinations, more prudent and flexible decisions are required in choosing the treatment methods, such as, direct anastomosis of vein or artery to aorta, or adding supplementary treatment methods like percutaneous coronary intervention, rather than choosing a fixed treatment methods.

• Pediatric Infection and Vaccine
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• v.11 no.2
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• pp.183-191
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• 2004

Purpose : The purpose of this study was to compare the clinical features of staphylococcal scalded skin syndrome(SSSS) isolated methicillin-resistant Staphylococcal aureus(MRSA) with those of SSSS isolated methicillin-sensitive Staphylococcal aureus(MSSA) in children. Methods : We retrospectively reviewed the medical records and microbiological results of 36 cases of SSSS who were admitted to the Pediatric Department of Ulsan Dong-Kang General Hospital from Jan. 1999 to Dec. 2003. Results : Among 36 cases, there were 7 generalized types, 1 abortive type in MRSA group and 5 generalized types, 6 abortive types in MSSA group. The peak incidence of age was between 2 and 5 years in both groups. SSSS were diagnosed with increasing frequency. Most of MRSA group were diagnosed in 2003(6/8). The peak seasonal incidence was fall and winter in both groups. Facial lesion was observed in all cases followed by flexural lesion in both groups. Staphylococci were isolated from the skin and throat most frequently in both groups. Upper respiratory illness and conjunctivitis were most common preceding infections in both groups. Among 8 cases of MRSA group, 4 cases were treated completely with ampicillin-sulbactam. There was no significant difference of mean duration of admission between MRSA group and MSSA group. Conclusion : There was no significant difference of clinical features between MRSA group and MSSA group although generalized types of SSSS were more common in MRSA group than in MSSA group.

• Pediatric Infection and Vaccine
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• v.16 no.2
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• pp.162-166
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• 2009

Purpose : Urinary tract infection (UTI) is one of the most common bacterial infectious disease in childhood. Renal scarring is an important complication of UTIs. Known risk factors for renal scarring are younger age, anatomic defects, delayed treatment, and causative pathogens other than Escherichia coli. The aim of this study was to compare the characteristics of clinical and laboratory features of UTI with E. coli to those with non-E. coli in infants. Methods : We reviewed the medical records of 1,120 infants under 12 months of age who had been admitted for UTIs between January 1998 and December 2007. All patients who were diagnosed with UTIs were divided into two groups (E. coli and non-E. coli UTIs). Results : Three hundred twenty-four of 1,120 cases met the inclusion criteria. The number of E. coli and non-E. coli UTIs was 273 (84.3%) and 51 (15.7%), respectively. As compared to the non-E. coli UTI group, the E. coli UTI group was younger (3.59 vs. 4.47 months, P =0.008), a longer duration of pyuria (3.96 vs. 3.06 days, P =0.01), higher peripheral white blood cell counts (13.89 vs. $12.13{\times}10^3/mm^3$, P =0.043), and lower rates of high degree (III-V) vesico-ureteral reflux (P =0.005). Conclusion : UTIs with E. coli might have more severe clinical features and a lower prevalence of high grade vesicoureteral reflux than UTIs with non-E. coli. However, no difference was noted in the clinical response to antibiotic therapy between the two groups.

• 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.2
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• pp.183-192
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• 2006

Purpose: This study was undertaken to evaluate the factors correlated with the clinical course and outcome in patients of Henoch-Sch${\ddot{o}}$nlein Purpura. Methods: The medical records of 104 children diagnosed with Henoch-Sch${\ddot{o}}$nlein Purpura (HSP) from January 1996 to April 2006 were reviewed retrospectively. The patients were divided into two groups: patients with Gastrointestinal (GI) symptoms and those without GI symptoms. When there were joint, scrotum, and renal symptoms except for skin lesion in whole HSP, those patients were excluded. The history of acute infection, duration of admission, treatment requirement, recurrence of HSP, CBC, stool occult blood test, abdominal ultrasonographic findings and GI endoscopic findings were reviewed. Results: Among 104 patients, patients with GI symptoms included 66 cases (63.5%), those without GI symptoms accounted for 38 cases (36.5%). GI symptoms included: abdominal pain in 57 cases (54.8%), vomiting 21 cases (20.2%), GI bleeding 5 cases (4.8%), nausea 3 cases (2.9%) and diarrhea 3 case (2.9%). Positive GI symptoms and GI mucosal lesions on GI endoscopy had a statistically significant correlation with increased admission duration, treatment requirement, recurrence of HSP, and positive stool occult blood. Six cases with small intestinal wall thickening were noted on abdominal ultrasonography. Six cases of hemorrhagic gastritis and hemorrhagic duodenitis, 3 cases of duodenal ulcer, 3 cases of hemorrhagic gastritis and duodenal ulcer, 2 cases of hemorrhagic duodenitis and colitis, and 1 case of colitis were noted on GI endoscopy. Conclusion: These results suggest that GI endoscopic examination may be helpful for the diagnosis and treatment of children with HSP.