• Childhood Kidney Diseases
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• v.10 no.1
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• pp.18-26
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• 2006

Purpose : Empirical antimicrobial treatment is indicated before bacteriological results are available for young children with febrile UTI to minimize renal scarring. To ensure appropriate therapy, knowledge of the prevalence of causative organisms and their susceptibility patterns to antimicrobials is mandatory. We performed a retrospective analysis investigating the local prevalence and resistance patterns of uropathogens, primarily E. coli, isolated from community-acquired UTIs. Methods : A total of 103 positive urine cultures from children with febrile UTI collected at Bundang CHA General Hospital from February 2004 to February 2005 were analyzed. Inclusion criteria were fever higher than $37.5^{\circ}C$, significant bacteriuria with single strain growth of at least 10s colony forming units/mL urine, and leukocyturia >5/HPF. Results : E. coli(89.3%) was the leading uropathogen followed by Enterococcus spp.(3.9%) Klebsiella spp.(2.9%), Citrobctcter spp.(1.9%) and Enterobacter spp.(1.9%). E. coli strains revealed a low proportion of antimicrobial susceptibility to ampicillin(AMP; 27.2%) ampicillinsulbactam(AMS; 34.8%) and trimethoprim-sulfamethoxazole(SXT; 65.2%). Susceptibility patterns to cephalosporins were as follows; cefazolin(1st generation; 91.3%), cefoxitin(2nd; 100%), ceftriaxone(3rd; 97.8%) and cefepime(4th; 97.8%). Three E. coli isolates produced ex tended - spectrum beta-lactamase(ESBL). Conclusion : Empirical treatment with AMP, AMS and SXT, which are commonly used in pediatric clinics, is not recommended for childhood UTI due to high incidence of resistance. The high level of susceptibility to cephalosporins makes these drugs reasonable alternatives. However the emergence of ESBL-producers, even though they are quite few, may have an impact on cephalosporin treatment in the future. (J Korean Soc Pediatr Nephrol 2006;10:18-26)

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.125-132
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• 2003

Purpose : Lipoprotein(a) is a genetically determined risk factor for atherosclerotic vascular disease and is elevated in patients with renal disease. Especially the patients with nephrotic syndrome exhibit excessively high Lp(a) plasma concentrations. Also the patients with end-stage renal disease have elevated Lp(a) levels. But the mechanism underlying this elevation is unclear. Thus, in this study, by measuring the level of serum Lp(a) in common renal diseases in children, we hoped to see whether there would be a change in Lp(a) in renal diseases other than nephrotic syndrome. Then, we figured out its implications, and looked for the factors that affect the Lp(a) concentrations. Methods : A total of 75 patients(34 patients with hematuria of unknown etiology, 10 with hematuria and hypercalciuria, 8 with IgA nephropathy, 8 with poststreptococcal glomerulone phritis, 3 with $Henoch-Sch\"{o}nlein$ nephritis, 7 with urinary tract infection, and 5 with or- thostatic proteinuria) were studied. The control group included 20 patients without renal and liver disease. Serum Lp(a), total protein, and albumin levels, 24-hour urine protein and calcium excretions, creatinine clearance and the number of RBCs and WBCs in the urinary sediment were evaluated. Data analysis was peformed using the Student t-test and a P-value less than 0.05 was considered to be statistically significant. Results : LP(a) was not correlated with 24-hour urine calcium and creatinine. Lp(a) level had a positive correlation with proteinuria and negative correlation with serum albumin and serum protein. Among the common renal diseases in children, Lp(a) was elevated only in orthostatic proteinuria (P<0.05). Conclusion : Lp(a) is correlated with proteinuria, serum protein, and serum albumin, but not with any kind of specific renal disease. Afterward, Lp(a) needs to be assessed in patients with orthostatic proteinuria and its possible role as a prognostic factor could be confirmed.

• 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 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.

• Journal of Genetic Medicine
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• v.4 no.1
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• pp.72-79
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• 2007

Purpose : Glycogen storage disease type III (GSD-III) is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme, amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), is responsible for the debranching of the glycogen molecule during catabolism. The disease shows clinical and biochemical heterogeneity, reflecting genotype-phenotype heterogeneity among different patients. In this study, we aim at analyzing mutations of the AGL gene in three unrelated Korean GSD-III patients, and characterizing their clinical and laboratory findings. Methods : We characterized the clinical features of three unrelated Korean GSD-III patients by biochemical, histological and imaging studies. The 35 exons and part of exon-intron boundaries of AGL were analyzed by direct sequencing using genomic DNA extracted from the peripheral leukocytes of patients. Results : Diverse clinical features were observed in these patients including hepatomegaly (all patients), seizures (patient 2), grow th failure (patients 1 and 2), hyperlipidemia (patients 1 and 3), raised transaminase and creatine kinase concentrations (all patients), and mild cardiomyopathy (patient 2). Liver transplantation w as performed in patient 2 due to progressive hepatic fibrosis. A dministration of uncooked corn starch maintained normoglycemia and improved biochemical and growth profiles. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 had c.1510_1511insT (p.Y 504L fsX 10), and patient 3 had c.3416 T >C (p.L 1139P) and c.1735+1 G>T (p.Y 538_R578delfsX 4) mutations. A part from the p.R428K mutation, the 4 other substitutions identified w ere nov el. Conclusion : GSD-III patients display variable phenotypic characteristics resembling those of GSD-Ia. Molecular defects in the AGL gene of Korean GSD-III patients are genetically heterogeneous.

• The Korean Journal of Nuclear Medicine
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• v.39 no.1
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• pp.26-33
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• 2005

Purpose: The purpose of this study is to evaluate migration of technetium-99m hexamethylpropylene amine oxime ($^{99m}Tc$-HMPAO) labeled immature and mature dendritic cells (DC) in the mouse. Methods: DC were collected from bone marrow (BM) of tibiae and femurs of mice. Immature and mature DC from BM cells were radiolabeled with $^{99m}Tc$-HMPAO. To evaluate the functional and phenotypic changes of DC from radiolabeling, the allogeneic mixed lymphocyte reaction (MLR) and fluorescence-activated cell sorting (FACS) analysis were performed before and after labeling with $^{99m}Tc$-HMPAO. Migration of intravenously injected DC (iv-DC) was assessed by serial gamma camera images of mice with or without subcutaneous tumor. Percent injected dose per gram (%ID/g) was calculated in lungs, liver, spleen, kidneys, and tumor through dissection of each mice after 24 hours of injection. Results: Labeling efficiency of immature and mature DC were $60.4{\pm}5.4%\;and\;61.8{\pm}6.7%$, respectively. Iv-DC initially appeared in the lungs, then redistributed mainly to liver and spleen. Migration of mature DC to spleen was significantly higher than that of immature DC ($38.3{\pm}4.0%\;vs.\;32.2{\pm}4.1%$ in control group, $40.4{\pm}4.1%\;vs.\;35.9{\pm}3.8%$ in tumor group; p<0.05). Migration to tumor was also significantly higher in mature DC than in immature DC ($2.4{\pm}0.3%\;vs\;1.7{\pm}0.2%$; p=0.034). Conclusion: Assessment of migration pattern of DC in mice was possible using $^{99m}Tc$-HMPAO labeled immature and mature DC. Migration of mature DC to spleen and tumor was higher than that of immature DC when they were i.v. injected.

• The Korean Journal of Nuclear Medicine
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• v.39 no.3
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• pp.174-181
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• 2005

Purpose: Reduction of respiratory motion artifacts in PET images was studied using respiratory-gated PET (RGPET) with moving phantom. Especially a method of generating simulated helical CT images from 4D-CT datasets was developed and applied to a respiratory specific RGPET images for more accurate attenuation correction. Materials and Methods: Using a motion phantom with periodicity of 6 seconds and linear motion amplitude of 26 mm, PET/CT (Discovery ST: GEMS) scans with and without respiratory gating were obtained for one syringe and two vials with each volume of 3, 10, and 30 ml respectively. RPM (Real-Time Position Management, Varian) was used for tracking motion during PET/CT scanning. Ten datasets of RGPET and 4D-CT corresponding to every 10% phase intervals were acquired. from the positions, sizes, and uptake values of each subject on the resultant phase specific PET and CT datasets, the correlations between motion artifacts in PET and CT images and the size of motion relative to the size of subject were analyzed. Results: The center positions of three vials in RGPET and 4D-CT agree well with the actual position within the estimated error. However, volumes of subjects in non-gated PET images increase proportional to relative motion size and were overestimated as much as 250% when the motion amplitude was increased two times larger than the size of the subject. On the contrary, the corresponding maximal uptake value was reduced to about 50%. Conclusion: RGPET is demonstrated to remove respiratory motion artifacts in PET imaging, and moreover, more precise image fusion and more accurate attenuation correction is possible by combining with 4D-CT.

• The Korean Journal of Nuclear Medicine
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• v.36 no.6
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• pp.357-367
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• 2002

Purpose: While cerebral blood flow and cerebrovascular reserve could be evaluated with basal/acetazolamide Tc-99m-HMPAO SPECT in cerebrovascular disease, objective quantification is necessary to assess the efficacy of the revascularization. In this study we adopted the SPM method to quantify basal cerebral blood flow and cerebrovascular reserve on basal/acetazolamide SPECT in assessment of the patients who underwent bypass surgery for linternal carotid artery (ICA) stenosis. Materials and Methods: Twelve patients ($51{\pm}15$ years) with ICA stenosis were enrolled. Tc-99m-HMPAO basal/acetazolamide perfusion SPECT was peformed before and after bypass surgery. After spatia1 and count normalization to cerebellum, basal cerebral blood flow and cerebrovascular reserve were compared with 21 age-matched normal controls and postoperative changes of regional blood flow and reserve were assessed by Statistical Parametric Mapping method. Mean pixel values of each brain region were calculated using probabilistic anatomical map of lobes. Perfusion reserve was defined as the % changes after acetazolamide over basal counts. Results: Preoperative cerebral blood flow and cerebrovascular reserve were significantly decreased in involved ICA territory, comparing with normal control (p<0.05). Postoperative improvement of cerebral blood flow and cerebrovascular reserve was observed in grafted ICA territories, but cerebrovasculr reserve remained with significant difference with normal control. Improvement of the cerebrovascular reserve was most prominent in the superior temporal and the angular gyrus, nearest to the anastomosis sites. Conclusion: Using SPM quantification method on hasal/acetazolamide Tc-99m-HMPAO SPECT, the cerebral blood flow and cerebrovascular reserve could be assessed before revascularization and so could the efficacy of the bypass surgery.

• The Korean Journal of Nuclear Medicine
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• v.34 no.4
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• pp.336-343
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• 2000

Purpose: Thallous-201 chloride produced at Korea Cancer Center Hospital(KCCH) is used in detecting cardiovascular disease and cancer. Thallium impurity can cause emesis, catharsis and nausea, so the presence of thallium and other metal impurities should be determined. According to USP and KP, their amounts must be less than 2 ppm in thallium and 5 ppm in total. In this study, the detection method of trace amounts of metal impurities in $[^{201}Tl]$TICI injection with polarography was optimized without environmental contamination. Materials and Methods: For the detection of metal impurities, Osteryoung Square Wave Stripping Voltammetry method was used in Bio-Analytical System (BAS) 50W polarograph. The voltammetry was composed of Dropping Mercury Electrode (DME) as a working electrode, Ag/AgCl as a reference electrode and Pt wire as a counter electrode. Square wave stripping method, which makes use of formation and deformation of amalgam, was adopted to determine the metal impurities, and pH 7 phosphate buffer was used as supporting electrolyte. Results: Tl, Cu and Pb in thallous-201 chloride solution were detected by scanning from 300 mV to -800 mV Calibration curves were made by using $TINO_3,\;CuSO_4\;and\;Pb(NO_3){_2}$ as standard solutions. Tl was confirmed at -450 mV peak potential and Cu at -50 mV Less than 2 ppm of Tl and Cu was detected and Pb was not detected in KCCH-produced thallous-201 chloride injection. Conclusion: Detection limit of thallium and copper is approximately 50 ppb with this method. As a result of this experiment, thallium and other metal impurities in thallous-201 chloride injection, produced at Korea Cancer Center Hospital, are in the regulation of USP and KP Polarograph could be applied for the determination of metal impurities in the quality control of radiopharmaceuticals conveniently without environmental contamination.

• The Korean Journal of Nuclear Medicine
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• v.36 no.4
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• pp.271-276
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• 2002

Purpose: To measure reliable glomerular filtration rate by using the representative values of transplanted renal depths, which are measured with ultrasonography. Materials and Methods: We included 54 patients (26 men, 28 women), with having both renal scintigraphy and ultrasonography after renal transplantation. We measured GFR with Gates' method using the renal depth measured by ultrasonography, and median and mean ones in each patient. We compared GFR derived from ultrasonography-measured renal depth with GFR derived from median and mean renal depths. The correlation coefficients were obtained among GFR derived from ultrasonography-measured renal depths, median and mean renal depths under linear regression analysis. We determined whether GFR derived from median or mean renal depth could substitute GFR derived from ultrasonography-measured renal depth with Bland-Altman method. We analyze the expected errors of the GFR using representative renal depth in terms of age, sex, weight, height, creatinine value, and body surface. Results: The transplanted renal depths range from 3.20 cm to 5.96 cm. The mean value and standard deviation of renal depths measured by ultrasonography are $4.09{\pm}0.65cm$ in men, and $4.24{\pm}0.78cm$ in women. The median value of renal depths measured by ultrasonography is 4.36 cm in men and 4.14 cm in women. The GFR derived from median renal depth is more consistent with GFR derived from ultrasonography-measured renal depth than GFR derived from mean renal depth. Differences of GFR derived from median and ultrasonography-measured renal depth are not significantly different in the groups classified with creatinine value, age, sex, height, weight and body surface. Conclusion: When median value is adapted as a representative renal depth, we could obtain reliable GFR in transplanted kidney simply.