• Childhood Kidney Diseases
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• v.9 no.1
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• pp.21-30
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• 2005

Purpose : Metabolic bone diseases have been mai or problems in children with renal diseases and steroid treatment is the main precipitating factor reducing bone mineral density(BMD). This study was performed to assess the prevalence of osteoporosis and to evaluate the clinical factors associated with decreased BMD in children with renal diseases. Methods : Forty-four children with renal diseases who were diagnosed at the Pediatric no phrology division of Ajou University hospital since Oct. 1994 were included. Using a new quantitative ultrasound device, BMD and the prevalence of osteoporosis were evaluated. The clinical and serological data were analyzed in association with decreased BMD. Results : A total of 44 patients were evaluated. The age at initial diagnosis was 6.7$\pm$4.2 years. At the time of evaluation, the chronological and bone age was 9.3$\pm$4.2 years and 8.2 $\pm$ 4.6 years, respectively. The renal diseases included nephrotic syndrome 24(54.5%), Henoch Schonlein purpura nephritis 7(15.9%), IgA nephropathy 6(13.9%), reflux nephropathy(RN) 2 (4.5%), and other renal disease 5(%). The prevalence of osteoporosis was 11%. There was no difference in the clinical factors between the long-term and the short-term treated steroid groups. Conclusion : The prevalence of osteoporosis was 12% in 44 children with renal diseases No significant factor was found in association with decreased BMD and there was no relationship between osteoporosis and steroid usage duration or cumulative dose. A new quantitative ultrasound, which is relatively easy to perform, especially in children, is expected to be in common use and will enable clinicians to evaluate metabolic bone disorders with ease.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.38-45
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• 2005

Purpose : The purpose of this study was to analyze the therapeutic effect of plasmapheresis in various pediatric diseases. Methods : Therapeutic plasmapheresis was performed by COBE Spectra centrifugation. Nine cases were included in this study. The number an[;. method of plasmapheresis, together with the progress and prognosis of each case were retrospectively reviewed. Results : The patients' ages ranged from 26 mont]Is to 16 years of age, and the mean age was 9.9 years. There were S males and 4 females. The underlying diseases requiring plasmapheresis included 2 cases of hemolytic uremic svndrome(HUS), 1 case of lupus nephritis, 2 cases of rapidly Progressive glomerulonephritis(RPGN), 1 case of focal segmental glomorulosclerosis(FSGS), 1 case of systemic vasculitis after pulmonary hemorrhage, 1 case of acute renal failure associated with pulmonary hemoIThage, and 1 case of acute rejection after renal transplantation. The average number of plasmapheresis performed was 6.2 times with a range of 3 to 13 times. The patients with HUS, lupus nephritis, ANCA positive systemic vasculitis induced by pulmonary hemorrhage and ARF-associated pulmonary hemorrhage showed a good response to therapeutic plasmapheresis, but the patients with RPGN, refractory FSGS, and acute rejection after renal transplantation were not responsive to treatment. The most common side effect was hypocalcemia which was rarely symptomatic. Vital signs were not compromised. Conclusion : Although it is presumptuous to generalize the therapeutic effects of plasma pheresis in different diseases due to the small number of study subjects, this study shows that plasmapheresis may be an effective therapeutic modality in various pediatrics diseases and should be considered as a therapeutic option.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.157-165
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• 2003

Purpose : $Henoch-Sch\"{o}nlein$ purpura(HSP) is a systemic vasculitis that involves multiple organs, especially the kidney, which is the most important organ in determining the prognosis of the disease. The morbidity of HSP nephritis in adults is low and there have been little research done on its clinical course so far. Therefore, we have compared the clinical course of HSP nephritis in children and adults in Korea. Methods : We retrospectively analyzed 81 cases of HSP nephritis in children younger than 15 years of age, and 25 cases of adults older than 15 years of age who were admitted to Yonsei University Medical College Severance Hospital from Jan. 1986 to May 2003. Results : The male to female ratio was 1.5 : 1 in children and 1.3 : 1 in adults. The incidence of HSP nephritis for both age groups was found to be increased during the autumn and winter. Infection was the predisposing factor in 39 cases(48.1%) of children, 16 cases(64.0%) of adults, and drugs were the predisposing factor in 8 cases(9.9%) of children and 4 cases (16.0%) of adults. All patients initially presented with microscopic hematuria. Thirteen cases (16.0%) of children and 7 cases(28.0%) of adults initially showed proteinuria of nephrotic range. Thirty four cases(42.0%) of children and 4 cases(16.0%) of adults showed normal urinalysis after treatment. Asymptomatic urinary abnormalities were found in 41 cases(50.6%) of children and 18 cases(72.0%) of adults. Complications such as nephrotic syndrome and hypertension were found in 3 cases(3.7%) of children and 2 cases(8.0%) of adults. Three children(3.7%) and 1(4.0%) adult required dialysis or renal transplantation. Follow-up renal biopsies were performed on 21 children, of whom 10 cases(47.6%) did not show any histologic change, 9 cases(42.9%) showed low grade changes, and 2 cases(9.5%) showed high grade changes. Prognosis was gloomy when proteinuria of nephrotic range and high grade of abnormal histology were present at diagnosis, and there was no significant difference between the two groups(P<0.05) Conclusion : This study showed that there was no difference in terms of the clinical features and courses between the children and adults with HSP nephritis. Proteinuria of nephrotic range and the severity of abnormal histologic changes at diagnosis were found to be associated with a bad prognosis, therefore we recommend that patients with these features require long term follow-up and management.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.174-180
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• 2003

Purpose : Unstable bladder has been known to be one of the reasons for the genesis and persistance of primary vesicoureteral reflux(VUR) in children. And treatment of unstable bladder by anticholinergic agent may contribute to the resolution of primary VUR. We evaluated the effect of an anticholinergic agent(oxybutynin) on the resolution of primary VUR in children with different toilet training and voiding functions. Methods : 152 children with persistant primary VUR after one year of follow up were randomly assigned to the oxybutynin group(n=59, oxybutynin 0.2 mg/kg twice daily) and the control group(n=93, no oxybutynin) at Ewha Womans University Mok-Dong Hospital from October 1996 to April 2002. The resolution rate of the VUR and the difference according to the status of toilet training and voiding dysfunction were analyzed. Statistical analysis was done by the Chi-square test and a P-value of less than 0.05 was considered as significant. Results : VUR was resolved in 49.2%, improved in 20.3% and not changed in 30.5% in the oxybutynin group(n=59) which was not significantly different to 45.2%, 16.1%, 38.7% in the control group(n=93), respectively. In the non-toilet trained young children, VUR was resolved in 50.0%, improved in 23.5% and not changed in 26.5% in the oxybutynin group(n=34) which was not significantly different to 44.2%, 19.2%, 36.6% in the control group(n=52), respectively. In the toilet trained older children, VUR was resolved in 48.0%, improved in 16.0% and not changed in 36.0% in the oxybutynin group(n=25) which was not significantly different to 46.3%, 12.2%, 41.5% in the control group(n=41), respectively. In the toilet trained older children with no voiding dysfunction, VUR was resolved in 33.3%, improved in 11.1% and not changed in 55.5% in the of oxybutynin group(n=9) which was not significantly different to 53.6 %, 10.7%, 35.7% in the control group(n=28), respectively. In the toilet trained older children with voiding dysfunction, VUR was resolved in 56.3%, improved in 18.7% and not changed in 25.0% in the oxybutynin group(n=16), which looked higher than 30.7%, 15.4%, 53.9% in the control group(n=13), respectively, but these were not significantly different either. Conclusion : Oxybutynin was not effective in the resolution of primary VUR in non-toilet trained young children and toilet trained older children. Oxybutynin showed slightly higher tendency of reflux resolution in toilet-trained older children with voiding dysfunction but the difference was not statistically significant. Judicious use of oxybutynin is required in selected older children with VUR and voiding dysfunction.

• Journal of Marine Bioscience and Biotechnology
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• v.1 no.2
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• pp.91-97
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• 2006

For efficient removal of nitrogenous compounds produced in recirculating aquaculture system, the N removal characteristics of immobilized mixed microorganisms were investigated at various mixing ratios of photosynthetic bacteria (PSB) immobilized in PVA beads or CTA cubes and ammonium utilizing bacteria (AUB) immobilized in PVA beads. On the optimal medium of AUB, the maxium gas production rate was obtained at the mixing ratio of 10:40 (PSB:AUB), and the gas production rate increased as the portion of AUB beads in the mixed beads increased. When the mixing ratios of PSB:AUB beads were 50:0, 40:10, 25:25 and 10:40, the final pHs were measured to be 6.29, 6.01, 5.69 and 5.13, respectively. On the optimal medium of PSB, however, the volume and the rate of gas production decreased remarkably as the portion of AUB beads in the mixed beads increased. The final pH was measured to be approximately 6.5, regardless of the mixing ratio. In the reactions by the mixed culture of PSB cubes and AUB beads, all results showed the same tendency of those by the mixed culture of PSB and AUB beads, but the volume and the rate of gas production decreased remarkably, even with 0.2ml of gas production in control. From all the results, the use of mixed PSB and AUB beads at the ratio of 10:40 seems to be efficient to remove nitrogenous compounds in wastewater from recirculating aquaculture system.

• Journal of Life Science
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• v.17 no.3 s.83
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• pp.435-443
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• 2007

Antimicrobial susceptibility test of the 116 strains of Mycoplasma pneumoniae isolates were performed by a broth micro-dilution method against to moxifloxacin, levofloxacin, sparfloxacin, ofloxacin, ciprofloxacin, clarithromycin minocycline, erythromycin, josamycin, and tetracycline. The initial-minimum inhibitory concentration (I-MIC) was evaluated as the lowest concentration of antimicrobial agents that prevented a color change in the medium at that time when the drug-free growth control, about 7 days after incubation, and the final-minimum inhibitory concentration (F-MIC) was defined a color change about 14 days after incubation. The evaluation to the drug-resistant M. pneumoniae isolates were determined the $MIC{\pm}1.0$ ${\mu}g/ml$ of each antimicrobial agent. According to the I-MIC, single drug-resistant M. pneumoniae strains to ciprofloxacin, ofloxacin, clarithromycin and erythromycin were 79.3, 53.5, 10.3, and 7.8%, respectively. Two kinds of drug-resistant M. pneumoniae strains to ofloxacin and ciprofloxacin, or ciprofloxacin and clarithromycin were 42.2 and 9.5%. Three kinds of drug-resistant M. pneumoniae strains to erythromycin, ofloxacin, and ciprofloxacin, or ofloxacin, ciprofloxacin and clarithromycin were 6.9 and 6.0% . According to the F-MIC, single drug-resistant M. pneumoniae strains to tetracycline, ciprofloxacin, ofloxacin, minocycline,erythromycin, josamycin, clarithromycin and sparfloxacin were 91.4, 91.4, 91.4, 89.7, 68.1, 52.6, 28.5, and 11.2%, respectively. The incidence of two kinds of drug-resistant M. pneumoniae strains were from 20.7% to 91.4%, three kinds of drug-resistant M. pneumoniae strains were from 28.5% to 89.7%, four kinds of drug-resistant M. pneumoniae strains were 2.6%, five kinds of drug-resistant M. pneumoniae were from 2.6% to 21.6%, six kinds of drug-resistant M. pneumoniae strains were from 0.9% to 24.1%, seven kinds of drug-resistant M. pneumoniae strains were from 0.9% to 2.6%, and eight kinds of drug-resistant M. pneumoniae strains were 1.7%. These results suggest that sparfloxacin, moxifloxacin and levofloxacin might be promising antimicrobial agents for the treatment of M. pneumoniae infection in Korea. However, most strains of M. pneumoniae isolates were single or multi-resistance pattern to the other tested antimicrobial agents. Therefore, tetracycline, minocycline, erythromycin, clarithromycin, and second-generation quinolones are more carefully used to patients with M. pneumoniae infection in Korea.

• Journal of Life Science
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• v.21 no.2
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• pp.183-193
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• 2011

Migration and differentiation of mesenchymal stem cells are crucial for tissue regeneration in response to injury. Sphingosine-1-phosphate (S1P) is a bioactive lipid that regulates a variety of biological processes, including proliferation, survival, differentiation and motility. In the present study, we determined the role of S1P in migration and differentiation of human bone marrow-derived mesenchymal stem cells (BMSCs). S1P stimulated migration of BMSCs in a dose- and time-dependent manner, and pre-incubation of the cells with pertussis toxin completely abrogated S1P-induced migration, suggesting involvement of Gi-coupled receptors in S1P-induced cell migration. S1P elicited elevation of intracellular concentration of $Ca^{2+}$ ($[Ca^{2+}]_i$) and pretreatment with VPC23019, an antagonist of $S1P_1/S1P_3$, blocked S1P-induced migration and increase of $[Ca^{2+}]_i$. Small interfering RNA-mediated knockdown of endogenous $S1P_1$ attenuated S1P-induced migration of BMSCs. Furthermore, S1P treatment induced expression of $\alpha$-smooth muscle actin ($\alpha$-SMA), a smooth muscle marker, and pretreatment with VPC23019 abrogated S1P-induced $\alpha$-SMA expression. S1P induced phosphorylation of p38 mitogen-activated protein kinase (MAPK), and pretreatment of cells with SB202190, an inhibitor of p38 MAPK, or adenoviral overexpression of a dominant-negative mutant of the p38 MAPK blocked S1P-induced cell migration and $\alpha$-SMA expression. Taken together, these results suggest that S1P stimulates migration and smooth muscle differentiation of BMSCs through an $S1P_1$-p38 MAPK-dependent mechanism.

• Journal of Genetic Medicine
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• v.7 no.2
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• pp.160-164
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• 2010

Structural abnormalities of the Y chromosome affect normal testicular differentiation and spermatogenesis. The present case showed a rare monocentric derivative Y chromosome with partial duplication of Yp including the SRY gene and deletion of Yq12 heterochromatin. The karyotype was 46,X,der(Y)(pter${\rightarrow}$q11.23::p11.2${\rightarrow}$pter).ish der(Y)(DYZ3+,DYZ1-,SRY++), confirmed through a FISH study. Even though the patient possessed an abnormal Y chromosome, testicular biopsy showed normal testicular volumes in the proband, with gonadal hormonal levels in the normal range but bilateral varicocele and hypospermatogenesis. We speculate that the abnormal Y chromosome arose from sister chromatids during Y chromosome recombination or intra chromosomal NAHR (non-allelic homologous recombination) during meiosis in the patient's father or in the very early stages of embryogenesis. The derivative Y chromosome might interfere in the meiotic stage of spermatogenesis, leading to the developmental arrest of germ cells. The present case illustrates that the infertility phenotype can have various causes. Also, it emphasizes the importance of accurate and various genetic analyses and could aid in male infertility treatment.

• Journal of Genetic Medicine
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• v.4 no.1
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• pp.45-52
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• 2007

Purpose : A simple, rapid, and highly sensitive analytical method for Gb3 in plasma was developed without labor-ex tensive pre-treatment by electrospray ionization MS/ MS (ESI-MS/MS). Measurement of globotriaosy lceramide (Gb3, ceramide trihex oside) in plasma has clinical importance for monitoring after enzyme replacement therapy in Fabry disease patients. The disease is an X-linked lipid storage disorder that results from a deficiency of the enzyme ${\alpha}$-galactosidase A (${\alpha}$-Gal A). The lack of ${\alpha}$-Gal A causes an intracellular accumulation of glycosphingolipids, mainly Gb3. Methods : Only simple 50-fold dilution of plasma is necessary for the extraction and isolation of Gb3 in plasma. Gb3 in diluted plasma was dissolved in dioxane containing C17:0 Gb3 as an internal standard. After centrifugation it was directly injected and analyzed through guard column by in combination with multiple reaction monitoring mode of ESI-MS/MS. Results : Eight isoforms of Gb3 were completely resolved from plasma matrix. C16:0 Gb3 occupied 50% of total Gb3 as a major component in plasma. Linear relationship for Gb3 isoforms w as found in the range of 0.001-1.0 ${\mu}g$/mL. The limit of detection (S/N=3) was 0.001 ${\mu}g$/mL and limit of quantification was 0.01 ${\mu}g$/mL for C16:0 Gb3 with acceptable precision and accuracy. Correlation coefficient of calibration curves for 8 Gb3 isoforms ranged from 0.9678 to 0.9982. Conclusion : This quantitative method developed could be useful for rapid and sensitive 1st line Fabry disease screening, monitoring and/or diagnostic tool for Fabry disease.

• Neonatal Medicine
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• v.15 no.1
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• pp.67-74
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• 2008

Purpose : The purpose of this study was to assess the natural history and perinatal outcomes of twin gestations according to chorionicity. Methods : We retrospectively reviewed the medical records of 99 monochorionic (MC) and 206 dichorionic (DC) twin gestations delivered at Il Sin Christian Hospital in Busan between January 2002 and December 2007. The incidences of twin-to-twin transfusion syndrome (TTS) and selective intrauterine growth restriction (sIUGR), as well as perinatal morbidity and mortality, were evaluated. Results : MC twins had a lower gestational age (35.7 vs. 36.6 weeks, P=0.03) at birth and a higher incidence of intrauterine fetal loss (10% vs. 1.5%, P<0.001) than DC twins. The incidence of intrauterine fetal loss was higher in MC sIUGR than in DC sIUGR (19% vs. 2.5%, P=0.025) twins. The number of admissions to the neonatal intensive care unit (NICU; 31% vs. 16%, P=0.042), and the incidence of periventricular leukomalacia (7% vs. 0%, P=0.031), and respiratory distress syndrome with surfactant treatment (27% vs. 11%, P=0.049) were higher in MC than DC twins. The incidences of sIUGR and TTS were 21 and 9% among the MC twins. The incidences of intrauterine fetal loss were higher in MC twins with TTS [6 of 9 (67%)] or sIUGR [4 of 21 (19%)] than uncomplicated MC twins (P<0.001). The frequency of admission to the NICU (P=0.001), the length of hospital stay (P=0.033), the prevalence of periventricular leukomalacia (P=0.011), and intraventricular hemorrhage (P=0.007) were also higher in MC with TTS or sIUGR than in uncomplicated MC twins. Conclusion : The incidence of neonatal complications was higher in MC twins, especially those gestations complicated by TTS or sIUGR.