• Childhood Kidney Diseases
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• v.5 no.2
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• pp.147-155
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• 2001

Purpose : IgA nephropathy(IgAN) and thin glomerular basement membrane disease(TGBMD) are common glomerular diseases that cause hematuria in childhood. IgAN has characteristics of IgA deposit as the sole or predominantly localized to the mesangium Recently, it has been reported that thinning of glomerular basement membrane(GBM) is commonly accompanied with precipitation of electron dense deposits in IgAN. We performed this study to examine the frequency of thinning of GBM among children with IgAN and to analysis tile correlation between urinary abnormalities and GBM thickness and furthermore to conduct comparative analysis of the clinical and pathological features of IgAN and TGBMD. Methods : This study summarizes data collected from Department of Pediatrics, Busan Paik Hospital, Inje Medical College. Data include 51 cases who were diagnosed as IgAN from 1995 to 2000, and 26 cases who were diagnosed as TGBMD from 1990 to 2000 by percutaneous renal biopsy. Results : Males accounted for 29/51($56.9\%$) patients with IgAN and 8/26($30.8\%$) of those with TGBMD. The clinical and laboratory features between IgAN and TGBMD were significantly different regarding the incidence of proteinuria(IgAN vs TGBMD: $43.1\%\;vs\;3.8\%$, p=0.001), the incidence of co-appearance of proteinuria with hematuria ($41.2\%\;vs\;3.8\%$, p=0.001), total amount of protein in 24 hours collected urine ($808{\pm}\;mg\;vs\;251{\pm}200.7\;mg$, p=0.001) and the incidence of proteinuria more than 1 gm in 24 hours collected urine ($23.5\%\;vs\;3.8\%$, p=0.01). On the contrary, there were no significant differences in the levels of serum albumin, creatinine, BUN, and Ccr between two groups. The mean thickness of GBM in patients with IgAN was $293.0{\pm}79.2\;nm$(139.7-461.9 nm) and $180.9{\pm}35.8\;nm$(110.5-229.5 nm) in patients with TGBMD. The mean GBM thickness revealed significantly thinner in TGBMD compared than those with IgAN (P=0.0001). The frequency of thickness being less than 250 nm was $37.4{\pm}34.4\%$ in IgAN and $93.0{\pm}7.0\%$ in TGBMD (P=0.0001). But there were no correlations between urinary abnormalities and GBM thickness in patients with IgAN. Conclusion : The thinning of GBM would be one of the common pathological findings in IgAN Moreover, there is no significant correlations between urinary abnormalities and GBM thickness in patients with IgAN, However, patients with IgAN tend to have significantly higher possibilities of proteinuria, co-appearance of proteinuria with hematuria and higher total amount of protein in 24 hours collected urine compared those with TGBMD. These differences might be play all important role as progressive prognostic indicators in patients with IgAN. (J Korean Soc Pediatr Nephrol 2001;5 : 136-46)

• Clinical and Experimental Pediatrics
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• v.48 no.9
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• pp.960-968
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• 2005

Purpose : This multi-center, open-label, clinical study was designed to evaluate the safety and immunogenicity of a trivalent, live, attenuated measles-mumps-rubella(MMR) vaccine, $Priorix^{TM}$ in Korean children. Methods : From July 2002 to February 2003, a total of 252 children, aged 12-15 months or 4-6 years, received $Priorix^{TM}$ at four centers : Han-il General Hospital, Kyunghee University Hospital, St. Paul's Hospital at the Catholic Medical College in Seoul, and Korea University Hospital in Ansan, Korea. Only subjects who fully met protocol requirements were included in the final analysis. The occurrence of local and systemic adverse events after vaccination was evaluated from diary cards and physical examination for 42 days after vaccination. Serum antibody levels were measured prior to and 42 days post-vaccination using IgG ELISA assays at GlaxoSmithKline Biologicals (GSK) in Belgium. Results : Of the 252 enrolled subjects, a total of 199 were included in the safety analysis, including 103 from the 12-15 month age group and 96 from the 4-6 year age group. The occurrence of local reactions related to the study drug was 10.1 percent, and the occurrence of systemic reactions was 6.5 percent. There were no episodes of aseptic meningitis or febrile convulsions, nor any other serious adverse reaction. In immunogenicity analysis, the seroconversion rate of previously seronegative subjects was 99 percent for measles, 93 percent for mumps and 100 percent for rubella. Both age groups showed similar seroconversion rates. The geometric mean titers achieved, 42 days pos-tvaccination, were : For measles, in the age group 12-15 months, 3,838.6 mIU/mL [3,304.47, 4,458.91]; in the age group 4-6 years, 1,886.2 mIU/mL [825.83, 4,308.26]. For mumps, in the age group 12-15 months, 956.3 U/mL [821.81, 1,112.71]; in the age group 4-6 years, 2,473.8 U/mL [1,518.94, 4,028.92]. For rubella, in the age group 12-15 months, 94.5 IU/mL [79.56, 112.28]; in the age group 4-6 years, 168.9 IU/mL [108.96, 261.90]. Conclusion : When Korean children in the age groups of 12-15 months or 4-6 years were vaccinated with GlaxoSmithKline Biologicals' live attenuated MMR vaccine ($Priorix^{TM}$), adverse events were limited to those generally expected with any live vaccine. $Priorix^{TM}$ demonstrated excellent immunogenicity in this population.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.1
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• pp.30-40
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• 2000

Purpose: During the first year of life, cow's milk protein is the major offender causing food allergy. Cow's milk allergy (CMA) affects 2~7% of infants, of which approximately one-half show predominantly gastrointestinal symptoms. We studied the clinical types of cow's milk allergy with predominantly gastrointestinal symptoms (CMA-GI) of childhood. Methods: The retrospective study was performed on 30 (male 22, female 8) patients who had diagnosed as CMA-GI during 2 years and 3 months from March 1995 to June 1997. Results: 1) Children with CMA-GI presented in the three types of clinical manifestation on the basis of time to reaction to milk ingestion: Quick (Q) onset (5 cases), Slow (S) onset (20 cases), Quick & Slow (Q&S) (5 cases). 2) Age on admission of the three groups was significantly different (p<0.05): (Q onset: $81.4{\pm}67.1$ days, S onset: $31.9{\pm}12.7$ days, Q&S: $366.0{\pm}65.0$ days). Although the body weight at birth was 10~95 percentile in all patients, body weight on admission was different: (Q onset: 10~50 percentile, S onset: below 10 percentile, Q&S: 10~25 percentile). S onset group was significantly different compared with other groups (p<0.05) and 90% of this one was failure to thrive below 3 percentile. 3) Peripheral leukocyte counts were as followings: (Q onset: $5,700{\sim}12,300/mm^3$, S onset: $10,000{\sim}33,400/mm^3$, Q&S: $5,200{\sim}14,900/mm^3$). Slow onset group was significantly different compared with other groups (p<0.05). Serum albumin levels on admission were as followings: (Q onset: $4.2{\pm}0.4\;g/dl$, S onset: $3.0{\pm}0.3\;g/dl$, Q&S: $4.0{\pm}0.3\;g/dl$). S onset group was significantly different compared with other groups (p<0.05) and 85% of this one was below 3.5 g/dl. 4) Although morphometrical analysis on small intestinal mucosa did not show enteropathy in Q onset and Q&S groups, all cases of S onset revealed enteropathy: 45% of this one showed subtotal villous atrophy, 55 % showed partial villous atrophy. 5) Allergic reaction test to other foods was not performed in S onset group because of ethical problem and high risk in general condition. In Q onset group, allergic reaction to one or two other foods: soy formula, weaning formula and eggs. Q&S goup revealed allergic reactions to several foods or to most of all foods except protein hydrolysate formula: eggs, potatos, some kinds of sea food, apples, carrots, beef and chicken. 6) Serum IgE level, peripheral eosinophil counts, milk RAST, soy RAST, skin test were not significantly different among groups. Conclusion: CMA-GI may present in three clinical ways on the basis of time to reaction to milk ingestion, typical clinical findings and morphologic changes in the small bowel mucosal biopsy specimens. This clinical subdivision might be helpful in diagnostic and therapeutic approaches in CMA-GI. Early suspicion is mandatory especially in S onset type because of high risks with malnutrition and enteropathy.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1116-1124
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• 2007

Purpose : Juvenile dermatomyositis (JDM) is the most common of the idiopathic inflammatory myopathies in children. The purpose of this study is to observe demographic, initial presentations, duration of time between disease onset and diagnosis, clinical manifestations and laboratory findings at diagnosis of patients with JDM. Methods : Forty seven patients identified at Seoul National University Children's Hospital from January 1986 to May 2007. Medical records were reviewed retrospectively focusing on initial presentations, clinical manifestations and laboratory findings at the time of diagnosis of patients with JDM. Results : Male and female patients were 25 and 22, respectively and sex ratio was 1.14:1. The average age at the time of diagnosis was 6.51 years. Skin rash (94%) was the most common symptom, followed by the proximal muscle weakness (89%). The disease activity score was 10.8. The duration between the onset of the skin rash and the muscle weakness and diagnosis was 7.18 and 4.70 months, respectively. The serum muscle enzymes, LDH, AST, CK and aldolase, were elevated in the patient with JDM. Autoimmune antibodies, antinuclear antibody, anti SSA antibody and anti SSB antibody, were negative findings. Electromyography findings were consistent with JDM in 88% of the patients, the muscle biopsy was in 91% and all MRI findings were compatible with those of patients with JDM. The most common symptom besides musculocutaneous lesions was the calcinosis (62.5%). The most common site of calcinosis was the pelvic area and buttocks. Conclusion : This study shows that the major symptoms are proximal muscle weakness and cutaneous lesion, and they are important to diagnose JDM.

• Tuberculosis and Respiratory Diseases
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• v.63 no.3
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• pp.242-250
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• 2007

Background: Abnormal angiogenesis can induce hypoxia within a highly proliferating tumor mass, and these hypoxic conditions can in turn create clinical problems, such as resistance to chemotherapy. However, the mechanism by which hypoxia induces these changes has not yet been determined. Therefore, this study was conducted to determine how hypoxia induces changes in cell viability and extracellular microenvironments in an in vitro culture system using non-small cell lung cancer cells. Methods: The non-small cell lung cancer cell line, A549 was cultured in DMEM or RPMI-1640 media that contained fetal bovine serum. A decrease in the oxygen tension of the media that contained the culture was then induced in a hypoxia microchamber using a $CO_2-N_2$ gas mixture. A gas analysis and an MTT assay were then conducted. Results: (1) The decrease in oxygen tension was checked the anaerobic gas mixture for 30 min and then reoxygenation was induced by adding a 5% $CO_2-room$ air gas mixture to the chamber. (2) Purging with the anaerobic gas mixture was found to decrease the further oxygen tension of cell culture media. (3) The low oxygen tension resulted in a low pH, lactic acidosis and a decreased glucose concentration in the media. (4) The decrease in glucose concentration that was observed as a result of hypoxia was markedly different when different types of media were evaluated. (5) The decrease in oxygen tension inhibited proliferation of A549 cells. Conclusion: These data suggests that tumor hypoxia is associated with acidosis and hypoglycemia, which have been implicated in the development of resistance to chemotherapy and radiotherapy.

• Journal of Life Science
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• v.22 no.4
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• pp.524-531
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• 2012

Soybean is one of the most common food materials causing food hypersensitivity reactions in Korea. In this study, we have investigated the effect of roasting and fermentation on the allergenicity of soybean. Three kinds of soybean ($Daepung$, $Daewon$, and $Taegwang$) were prepared as raw, roasted, and fermented by $Bacillus$ $subtilis$ GSK 3580, and then their proteins were extracted. The proteins were separated using SDS-PAGE, and the detection of IgE specific to soybean proteins was performed by immunoblotting using 7 sera of soybean allergy patients and non-allergic control individuals. Serum specific IgE to soybean was measured by ELISA. The SDS-PAGE of raw soybean proteins showed various-sized bands ranging from 9 to 76 kDa, which are known as major allergens. In particular, 9, 21, 34, 52, 72, and 76 kDa proteins are known as LTP, Kunits trypsin inhibitor, $Gly$ m Bd 30K, ${\beta}$-subunit, ${\alpha}$-subunit, and ${\alpha}$'-subunit of ${\beta}$-conglycinin, respectively; these are major allergens in soybean. In contrast, only peptides of less than 35 kDa were found in roasted and fermented soybeans. IgE immunoblot analysis of three roasted species of soybeans commonly detected at 38-40 kDa and 10-15 kDa. The protein bands in fermented soybean showed very weak signals or were not detected. In addition, the reactivity of most patients' sera to soybean was decreased after roasting and fermentation. With these results, it may be concluded that the allergenicity of soybeans is reduced by the roasting and fermentation processes. It is supposed that allergenic proteins in soybean were degraded by heat treatment methods and proteolytic enzymes were secreted from fermenting microorganisms.

• Korean Journal of Poultry Science
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• v.42 no.1
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• pp.33-40
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• 2015

This study was conducted to investigate the effects of dietary quercetin on growth performance, blood biochemical parameters, immunoglobulin, and blood antioxidant activity in broiler chickens. Three hundred twenty one-day old Ross broilers were divided 8 treatments (C(-), basal diet; C(+), basal diet with antibiotics; vitamin E 20 IU; vitamin E 200 IU; quercetin 20 ppm; quercetin 200 ppm; methoxylated quercetin 20 ppm; methoxylated quercetin 200 ppm) with 4 replicates and 10 birds per replicate. Birds were reared for 35 days and their feed intake and weight gain were measured weekly. At 35d, eight birds of average weight from each replicate were selected for blood collection and analysis. Weight gain of birds in the groups fed quercetin was higher when compare to NC but there was no significant difference. In the serum, creatinine, BUN and AST in quercetin groups significantly decreased compared to those of control (NC and PC) (P<0.05). The contents of IgA and IgM were significantly lower in quercetin groups than those of NC (P<0.05). SOD like activity and MDA content tended to decrease in quercetin groups, however, there was no significant difference among treatments. In conclusion, supplemental quercetin to poultry diet could be positive aspect on performance and blood metabolites. Optimum adding levels was more than 20 ppm.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.93-101
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• 2016

Purpose: McArdle disease, glycogen storage disease type V (GSD V), is one of the most common adolescent-onset glycogen storage diseases. It is caused by recessive mutations in PYGM encoding myophosphorylase, which is critical to glycogen metabolism. Since only a few korean patients have been reported, we will observe the clinical and genetic features of three korean patients with McArdle disease. Methods: We retrospectively reviewed the medical records of three patients with genetically confirmed McArdle disease, including the results of forearm ischemic exercise test, electromyogram, nerve conduction velocity, muscle biopsy, and PYGM analysis in peripheral leukocytes. Results: All three cases were males and their age of symptom onset was 12, 5, 14 years old, respectively. A high basal level of serum creatine kinase was noted in all three patients. They experienced the recurrent episodes of rhabdomyolysis, but second wind phenomenon was not definite. In muscle biopsy, subsarcolemmal space vacuoles including periodic acid schiff stained materials were found in two patients, while no evidence of glycogen storage disease was found in the other. A total of five different mutations, $p.Arg50^*$, p.Trp798Arg, $p.Arg50^*$, p.Glu779del, $p.Asp511Thrfs^*28$ and p.Phe710del, were found in three patients. Avoidance of isometric exercise, aerobic exercise and glucose intake before each exercise were recommended for all patients. Conclusion: The three Korean patients with McArdle disease showed the typical manifestations of the condition. The most mutations were private. Therefore, identification of more cases with long-term follow-up will be required to understand the clinical and genetic features of this disease among Korean population.

• Clinical and Experimental Pediatrics
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• v.45 no.7
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• pp.875-883
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• 2002

Purpose : Airways eosinophilia and increased IgE, characteristic features of asthma, result from a predominant Th2 response. In this study, we investigated the effect of CpG oligodeoxynucleotides (ODNs) on the inhibition of airways eosinophilia in mice with established airway inflammation. We also investigated the immunological mechanisms involved. Methods : Groups of BALB/c mice were sensitized intradermally with ovalbumin(OVA). At week 10, airway inflammation was induced by intranasal challenge of the mice with OVA. At week 14, the mice were challenged intranasally again with OVA in the presence and without the presence of CpG ODNs. Mice with saline administration served as negative controls. Bronchoalveolar lavage fluids(BALF) were obtained and eosinophils were counted. Th1 and Th2 cytokines in the spleen cell cultures were measured by ELISA. Serum OVA-specific IgE and IgG2a antibodies were also measured by ELISA. Results : BALF eosinophils were significantly inhibited in the CpG ODNs-treated mice(P<0.01). IgE and IgG2a levels increased significantly in both CpG ODNs-treated and untreated groups as compared to the negative control group; there was, however, no significant difference between the two groups four days after intranasal administration of CpG ODNs. Cytokine analysis revealed decreased production of IL-4, IL-5, and IL-13 and increased production of IL-12 in the CpG ODNs-treated group as compared to the untreated group. Interestingly, $IFN-{\gamma}$ levels were not upregulated in the CpG ODNs-treated group. Conclusion : CpG ODNs vaccination is a potentially useful approach for reversing airways eosinophilia in mice with established airways inflammation.

• Journal of the Korean Society of Food Science and Nutrition
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• v.39 no.5
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• pp.684-691
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• 2010

The present study was conducted to evaluate the effect of resistant starch (RS) on the large bowel function and plasma lipids in rats with constipation induced by Loperamide. Animals were divided into six groups: normal control-5% cellulose, constipation-5% cellulose, constipation-5% pectin, constipation-5% RS-type 2 (RS2), constipation-8% RS2 and constipation-5% RS type 3 (RS3) groups, and fed experimental diets for five weeks. The results from RS groups were compared with those from other dietary fiber groups. The groups supplemented with RS3 or high level of RS2 showed significantly increased counts of bifidobacteria in the cecum than the other groups. The production of total short chain fatty acids in the cecal contents was significantly high in pectin, RS3 and high RS2 groups. The pH in the cecal contents of the RS supplemented groups was significantly decreased compared with the cellulose supplemented groups. The production of prostaglandin E2 in the colon mucus of the RS groups was higher than the normal group; however, it was significantly decreased compared to the cellulose or pectin supplemented constipated groups. The thickness of the mucus layer and the production of mucus from epithelial cells were significantly increased in RS3 group compared to the constipated cellulose group. Supplementation of resistant starch significantly elevated the ratio of HDL-cholesterol to total cholesterol and significantly lowered plasma atherogenic index compared with cellulose or pectin supplementation in constipated rats. The results of the present study demonstrated that resistant starch supplementation may help in improving the large bowel environment by stimulation of bifidobacterial proliferation, reduction of pH and inflammation factor and by increased production of mucus. It has also been found that an additional health benefit is improvement in lipid levels of serum.