• Childhood Kidney Diseases
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• v.9 no.2
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• pp.213-221
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• 2005

Purpose : Urachal anomalies are rare but are known to develop several complications, especially infection. Moreover, uniform guidelines for management have not been presented because of the variable clinical characteristics of these anomalies. The purpose of this report is to review our experience with urachal anomalies and attempt to determine the optimal management. Methods : We retrospectively reviewed the records of fourteen children with a variety of urachal anomalies who had been treated from January 1996 to June 2005 at Dong Kang General Hospital. Results : The age distribution of the patients(mean age; 3.8 years) was six neonates, one infant, five preschool-age and two school-age children. The male to female ratio was 1:1. Six cases of urachal cyst, four cases of patent urachus, two cases of urachal sinus and two cases of urachal diverticulum were found. Three patients with patent urachus and one with urachal cyst had hydronephrosis. Other associated anomalies included an inguinal hernia in one patient with urachal sinus and a vesicoureteral reflux in one patient with urachal diver ticulum. As a first-line diagnostic tool, high-resolution ultrasound examination was performed in thirteen cases and computed tomography in one case. Surgical excision was performed in nine patients with urachal anomaly. Five cases out of six neonatal cases experienced spontaneous improvement during a three-month follow up period. Due to frequent infection of the umbilicus, surgical excision was performed on one neonate with urachal sinus. Conclusion : All patients with urachal anomalies should undergo investigation for associated anomalies. The neonate with urachal anomalies, especially patent urachus, do not require surgical excision unless the patient has multiple episodes of recurrent infection. (J Korean Soc Pediatr Nephrol 2005;9:213-221)

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.368-374
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• 2006

Purpose : The prevalence of obesity in children is increasing rapidly. Epidemiologic studies suggest that obesity induced atherosclerosis may start in childhood. We investigated whether obese children show early abnormalities of the arterial wall and endothelial dysfunction. Methods : Thirty-eight obese children(14-16 years old of age, male, body mass index $29.40{\pm}3.18kg/m^2$) and forty-five age and sex-matched healthy control children(body mass index $18.43{\pm}1.01kg/m^2$) were enrolled. Their carotid artery intima-media thickness(IMT) and brachial artery flowmediated dilation(FMD) response were measured by high-quality ultrasound system, and compliance, distensibility, stiffness index, incremental elastic modulus and wall stress were calculated by equation. In addition, we looked at the relations between these arterial features and metabolic cardiovascular risk factors. Results : The obese children had significantly increased IMT($0.52{\pm}0.09mm$ vs $0.40{\pm}0.07mm$, P< 0.001) and markedly impaired FMD($7.35{\pm}7.78$ percent vs $20.34{\pm}16.81$ percent, P<0.001) than the healthy controls. But the compliance and distensibility were lower, and the stiffness index, incremental elastic modules and wall stress were higher in the obese group than the control group, but not statistically significantly. Body mass index was highly associated with increased IMT(r=0.612, P<0.001) and reduced FMD(r=-0.414, P<0.001). Conclusion : We showed the deleterious effect of child obesity on both early functional and structural atherosclerotic markers. The ultrasonic findings will be used for screening and follow up markers to identify high-risk patients among obese children.

• Radiation Oncology Journal
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• v.17 no.1
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• pp.30-35
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• 1999

Purpose : To evaluate the significance of squamous cell carcinoma antigen (SCC) and carcinoembryonic antigen (CEA) as tumor markers in uterine cervix carcinoma. Materials and Methods : In 22 patients with histologically proven primary squamous cell carcinoma of uterine cervix, tumor volume was checked either by using MRI (in 20 patients) or ultrasound (in 2 patients). Pre-treatment serum SCC levels were checked in 22 patients and CEA levels in 21 patients. After curative radiotherapy, post-treatment SCC and CEA were checked regularly. Results : SCC was raised In 68.2$\%$ and CEA was raised in 19.0$\%$ before treatment. The coefficient of correlation between tumor volume and pre-reatment SCC was 0.59382 when one extremely deviated case was excluded. And there was no correlation between tumor volume and CEA. After the treatment, SCC was raised En 9.1$\%$ and CEA was raised in 4.8$\%$. In further follow up measurement, raise of SCC was associated with clinical relapse or persistence of disease. The specificity of raised SCC level in association with recurrent or persistent disease was 93.8$\%$ . The sensitivity in association with recurrent or persistent disease was 100$\%$. The positive predictive values was 85.7$\%$. The median lead time for recurrence was 1.2 months. Conclusions: Both SCC and CEA were good tumor markers for monitoring treatment effect in patients with raised pre-treatment levels. But the sensitivity of pretreatment CEA was low, while that of pretreatment SCC was high. And there was no additional gain by adding CEA measurements to SCC measurements.

• Tuberculosis and Respiratory Diseases
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• v.48 no.3
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• pp.357-364
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• 2000

Background : Complicated exudative pleural fluid collections have traditionally been treated by either closed tube thoracostomy drainage or by open surgical drainage. Complete drainage is important in order to control pleural sepsis, restore pulmonary function, and entrapment. Recently intracavitary fibrinolytic therapy has been advocated as a method to facillitate drainage of complicated exudative pleural effusion and to allow enzymatic debridemant of the restrictive fibrinous sheets covering the pleural surface. The purpose of this study is to prospectively evaluate the effects of image-guided catheter drainage with high dose urokinase(UK) instillation in the treatment of complicated pleural effusions. Patients : Twenty complicated pleural effusion patients that poorly respond to image-guided drainage were allocated to receive UK. There were 8 pneumonia and 12 tuberculosis. Methods : Drugs were diluted in 250 mL normal saline and were infused intrapleurally through the chest tube or pig-tail catheter in a daily dose of 250,000 IU of UK. Response was assessed by clinical outcome, fluid drainage, chest radiography, pleural ultrasound and/or computed tomography. Results : The mean UK instillation time was $1.63{\pm}0.10$. The mean volume drained UK instillation was $381.3{\pm}314.4\;mL$, and post-UK was $321.6{\pm}489.5\;mL$. The follow up duration after UK therapy was mean $212.9{\pm}194.5$ days. We had successful results in 19 cases (95.0%). There were 12 pleural thickenings (60.0%), 2 markedly decreased effusions (10.0%) and 5 cases of no thickening or effusion. There was recurrence after treatment in only one patient(5%) with complicated pleural effusiondue to tuberculosis. Conclusions : Image-guided drainage with high dose UK instillation (250,000 U/day) in complicated pleural effusion is a safe and more effective method than closed thoracostomy drainage. And this management, in turn, can obviate surgery in most cases.

• Journal of Gastric Cancer
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• v.5 no.1
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• pp.40-46
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• 2005

Purpose: Gastrointestinal stromal tumors (GISTs) are mesenchymal neoplasms of the gastrointestinal tract. GISTs are positive for the expression of c-Kit protein at immunohistochemistry, and their clinical presentations vary. This retrospective study was performed to evaluate the clincopathologic characteristics of GISTs and to define the prognostic factors. Materials and Methods: 40 patients who underwent a complete resection of a GIST during the period $1996\~2003$ at the Department of Surgery, Korea University College of Medicine, were studied. We divided them into low- and high-risk. groups by using tumor size and mitotic count: 23 cases were low risk, and 17 were high risk. Clinicopathologic features, immunohistochemical findings, and prognoses were compared between the low- and the high-risk groups. Results: The mean age of the 40 patients was $61.3\pm11.1$years, and the male-to-female ratio was 1：1.1. There was no significant difference in age and sex between the groups. A comparative analysis revealed tumor size, mitotic count, clinical symptoms, preoperative pathologic diagnosis, ulceration, and necrosis to be variables that had statistically significant differences between the high- and the low-risk groups. In the univariate analysis, tumor size, mitotic count, ulceration, necrosis, and abnormal endoscopic ultrasound findings were associated with disease-free survival, but in the multivariate analysis, mitotic activity was the only independent factor associated with disease-free survival. 8 patients had recurrences during the follow-up period, and four of them were treated with STI-571 (imatinib mesylate, $Gleevec^{(R)}$). The treated patients have survived until now; however, two of non-treated patients died from disease progression. Conclusion: Based on this study, tumor size, ulceration, and necrosis are significant factors affecting survival, and mitotic activity may be a useful prognostic marker. STI-571 may be used in an adjuvant setting because the drug has shown anticancer activity in patients with recurrence or metastasis.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.20 no.2
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• pp.55-62
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• 2020

A 22-month-old girl who had taken iron supplements due to iron deficiency anemia, presented bloody mucoid stool for one month. She had a bruise at the right periorbital area due to minor trauma and hepatosplenomegaly. Laboratory studies showed anemia, thrombocytopenia, elevated alkaline phosphatase (ALP), hypophosphatemia, decreased haptoglobin, hypocomplementemia, negative direct/indirect Coomb's test, normal vitamin D3 level and high PTHi. Wrist x-ray showed no signs of rickets. The abdominal ultrasound showed only accessory spleen. Tandem mass spectrometry was normal. During follow up, bloody stool regressed after seven days of withdrawal of iron supplement and cow milk, and the total CO2 level had been within 15-20 mEq/L with normal anion gap. NGS (next generation sequencing) panel test for evaluation of renal tubular acidosis showed negative results. After low dose steroid and vitamin D supplements under the impression of hypocomplementemic vasculitis, thrombocytopenia, C3/C4, decreased haptoglobin, and elevated ALP level became normal. At 57 months of age, laboratory findings showed elevated liver enzyme, ALP and gamma-glutamyl transferase again. And liver cirrhosis with splenomegaly and diffuse renal disease were reported with abdomen CT scan. Liver biopsy reported macro- and micronodular cirrhosis. Urine organic acid profile showed elevated succinylacetone level. Whole exome sequencing revealed novel compound heterozygous mutations (NM_00137.2:c.107T>C, NM_00137, 2:c.614T>C) in FAH gene and confirmed by Sanger sequencing. Consequently, the patient was diagnosed as chronic hereditary tyrosinemia type I. She started low phenylalanine/tyrosine diet and nitisinone treatment. Our case had presented symptoms very slowly, which is the first case of chronic tyrosinemia type I in South Korea.

• Journal of the Korean Society of Radiology
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• v.81 no.1
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• pp.147-156
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• 2020

Purpose The objective of this study was to evaluate the diagnostic value and threshold levels of cytokeratin fragment 21-1 (CYFRA 21-1) in fine-needle aspiration (FNA) washouts for detection of lymph node (LN) recurrence in postoperative breast cancer patients. Materials and Methods FNA cytological assessments and CYFRA 21-1 measurement in FNA washouts were performed for 64 axillary LNs suspicious for recurrence in 64 post-operative breast cancer patients. Final diagnosis was made on the basis of FNA cytology and follow-up data over at least 2 years. The concentration of CYFRA 21-1 was compared between recurrent LNs and benign LNs. Diagnostic performance and cut-off value were evaluated using a receiver operating characteristic curve. Results Regardless of the non-diagnostic results, the median concentration of CYFRA 21-1 in recurrent LNs was significantly higher than that in benign LNs (p < 0.001). The optimal diagnostic cut-off value was 1.6 ng/mL. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of CYFRA 21-1 for LN recurrence were 90.9%, 100%, 100%, 98.1%, and 98.4%, respectively. Conclusion Measurement of CYFRA 21-1 concentration from ultrasound-guided FNA biopsy aspirates showed excellent diagnostic performance with a cut-off value of 1.6 ng/mL. These results indicate that measurement of CYFRA 21-1 concentration in FNA washouts is useful for the diagnosis of axillary LN recurrence in post-operative breast cancer patients.