• Childhood Kidney Diseases
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• v.2 no.1
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• pp.77-81
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• 1998

The author exprienced a case of glycogen storage disease type Ia(GSD-I) in an 18-year-old male patient who was admitted to our hospital due to proteinuria and hypertension. he was suspected to have GSD when 12 years old because of his family history of short stature and hepatomegaly. On admission, physical examination revealed short stature, heparomegaly, and The diagnosis of GSD-I was confirmed by compatible liver biopsy finding and enzyme assay which erealeddeficiency of glcose-6-phosphatase if hepatocyte. Sympromatic treatment was done using antihypertensive drugs and allopurinol with diet control. The authors report a case of glycogen storage disease type Ia completely confirmed by typical clinical manifestation, pathologic findings of the liver and the kidney, and the result of enzyme assay which revealed deficiency of glucose-6-phosphatase in hepatocytes with brief review fo related literatures.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.229-238
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• 2004

Purpose: This study was perfonned to elucidate the clinical pictures of acute focal bacterial nephritis(nephronia) in children. Methods: We reviewed 9 children with nephronia diagnosed by ultrasonography or computed tomography of kidneys from September 1994 to August 2004. Results: The overall male to female ratio was 2:1, and the age distribution ranged from 0.1 to 6 years(mean $2.8{\pm}2.2$). The cardinal symptoms were fever, chills, abdominal pain and dysuria/frequency. The initial leukocyte count was $21,000{\pm}5,600/uL$, ESR $60{\pm}23mm/hr$, CRP $17{\pm}10\;mg/dl$. Pyuria was noted in every patient and persisted for $10.5{\pm}7.8$ days after antimicrobial treatment. Abdominal sonography demonstrated focal lesion of ill-defined margin and low echogenicity in 5 of 9 patients(55.6%), while computed tomography revealed nonenhancing low density area in all patients(100%). Three of 9 patients(33.3%) had vesicoureteral reflux, greater than grade III. The initial $^{99m}Tc-DMSA$ scan showed one or multiple cortical defects in every patient, and improvements were noted in 2(33.3%) of 6 patients who received follow up scan after 4 months. Intravenous antibiotics was given in every patient under admission. Total febrile period was $11.8{\pm}6.3$ days(pre-admission, $4.0{\pm}3.0;$ post-admission, $7.8{\pm}5.5$ days) and the patients needed hospitalization for $17.2{\pm}8.1$ days. Conclusion: For the early diagnosis of 'acute focal bacterial nephritis' we should perform renal computed tomography first rather than ultrasonography, when the child has toxic symptoms and severe inflammatory responses in blood and urine.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.14 no.2
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• pp.155-160
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• 2011

Purpose: This study was performed to evaluate the role of colonoscopy in children with hematochezia. Methods: We retrospectively reviewed the medical records of 277 children who underwent colonoscopy because of hematochezia between January, 2003 and July, 2010. Results: The mean age of the patients was $6.0{\pm}4.4$ (7 days~17.8 years) years. The male to female ratio was 2.2:1. The duration between the 1st episode of hematochezia and colonoscopy was $4.9{\pm}12.1$ months. Characteristics of hematochezia included red stool (65.1%), blood on wipe (12.8%), bloody toilet (11.9%), and blood dripping (10.2%). The most proximal region of colonoscopic approach was terminal ileum (84.5%), cecum (9.5%), hepatic flexure (2.8%), and splenic flexure (3.2%). Eighty five patients (30.6%) had no specific abnormal findings. Major causes of hematochezia were polyp (26.4%), food protein induced proctocolitis (6.9%), infectious colitis (5.4%), lymphofolliculitis (5.7%), non specific colitis (5.7%), and vascular ectasia (5.1%). The hemorrhagic sites included the rectum (24.0%), rectosigmoid junction (18.1%), sigmoid colon (13.5%), ascending colon (14.2%), transverse colon (11.3%), descending colon (7.8%), cecum (8.1%), and terminal ileum (3.1%). The recurrence rate of hematochezia after colonoscopy was 19.1%. Colonoscopy was performed in 262 patients (94.6%) with conscious sedation. Endoscopic hemostasis was performed in 5 patients. Complications of colonoscopy or sedation were not found. Conclusion: The causes and lesional localization of pediatric hematochezia were diverse. Colonoscopy has an important role in the diagnosis and treatment of hematochezia in children. Total colonoscopy is recommended to detect the cause of hematochezia.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.161-169
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• 2009

Purpose : This study was performed to report the diagnosis and treatment of nephrotic syndrome manifesting in the first year of life. Methods : We retrospectively reviewed the clinical data with chart review in 7 patients who were diagnosed as nephrotic syndrome manifesting in the first year of life from 1996 to 2007. Results : Three patients had congenital nephrotic syndrome, the other 4 patients had infantile nephrotic syndrome. Their ages ranged from birth to 11 months and male to female ratio was 1 to 6. Renal biopsies were done in 6 patients. One patient had Finnish type congenital nephrotic syndrome, 2 patients had diffuse mesangial sclerosis, 2 patients had focal segmental glomerulosclerosis and 1 patient had minimal change disease. Genetic analyses of NPHS2, PLCE1, and WT1 were done in 4 patients and 2 of them had WT1 mutation. Among 3 patients with congenital nephrotic syndrome, 1 patient was diagnosed as congenital nephrotic syndrome of Finnish type and the other 2 patients were diagnosed as Denys-Drash syndrome. All of the patients with congenital nephrotic syndrome died due to sepsis. Among 4 patients with infantile nephrotic syndrome, 2 patients died and 1 had remission, another patient progressed to end stage renal disease. Conclusion : Most of nephrotic syndrome manifesting in the first year was hereditary renal disease. Patients with nephrotic syndrome manifesting in the 3 month of life had poorer prognosis and needed more aggressive management including early dialysis and renal transplantation might be considered compared with infantile nephrotic syndrome. Further genotype-phenotype correlation studies are needed.

• Journal of Sasang Constitutional Medicine
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• v.11 no.1
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• pp.271-279
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• 1999

1. Purpose : From a viewpoint of Lee Je-ma, We consider the principle of shape(form) and image and try out a share of recognition about shape(form) and image in all its aspects. 2. Method : Especially we compared HeoJun's point of view with Lee Je-ma's point of view. 3. Result & Conclusion 1) The most important thing for an appearance of shape(form) and image is a differential Qi of sorrow- anger-joy-pleasure. 2) Lee Je-Ma chose a point of view from inside to outside in principle. 3) A method of materialism can be used when we give medical treatment. 4) Shape(form) and image is a method of constitutional diagnosis from a viewpoint of Lee Je-ma. 5) Shape(form) and image has a formal(typical) type, but the thing which is fixed do not exist, therefore partial shape(form) and image is possible. 6) We should observe a condition of mind, but objectivity and reappearance always remain.

• Journal of Gastric Cancer
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• v.6 no.2
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• pp.109-113
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• 2006

Purpose: Various minimally invasive surgical techniques, such as an endoscopic mucosal resection and a laparoscopic gastrectomy, are becoming common practice for some cases of early gastric cancer (EGC) defined in terms of the depth of invasion being limited to the mucosa or submucosa. However, there are rare cases of early gastric cancer with massive lymph-node metastasis. Materials and Methods: From 6 university hospitals of Korea, 2,772 EGC cases were resected during the various period of analysis (1,432 cases of mucosal cancer and 1,340 of submucosal cancer). Results: As control data, we used the data from a single institute, CHA University Hospital. There were nine cases of early gastric cancer (9/2,772, 0.32%) with N3 lymph node metastasis defined by more than 15 lymph nodes being metastasized according to the UICC-TNM classification (pT1N3, stage IV). Two cases were mucosal cancer (2/1,432, 0.1 4%), and seven cases were submucosal cancer (7/1,340, 0.52%). Metastasized lymph nodes varied in number from 18 to 52. There were three male and six female patients with a mean age of 57. This is a totally reversed sex ratio compared to the usual gastric cancer or EGC. Among the total of 9 EGC patients, there were 5 who had superficial spreading carcinomas with surface areas larger than $25\;cm^2$. This is a significantly higher proportion compared to the general EGC population. When we compared the tumor size according to the LN status, the N3 group was definitely larger than the other groups. 78% of the pT1N3 cases showed lymphatic invasion, which is very high compared to the 4.7% in general EGC cases. Among the 9 cases, 6 patients had too short a follow-up period to evaluate the correct prognosis, but there was one patient with a non-curative resection and two patients with early recurrence. Although the sample size is small and the follow-up period is short, we can expect a very poor prognosis when we consider the common prognosis of EGC that is widely known and accepted. Conclusion: From these results, we can a conclude that the risk factors for pT1N3 gastric cancer are female patients, submucosal invasion, larger tumor size, and lymphatic invasion. However rare, the existence of pT1N3 gastric cancer needs to be taken into consideration, especially during the diagnosis. Furthermore, minimally invasive treatment for EGC needs to be chosen with great precaution. Since the prognosis of pT1N3 gastric cancer is expected to be poor, aggressive adjuvant chemotherapy may be necessary. (J Korean Gastric Cancer Assoc 2006;6:109-113)

• The Journal of the Korean bone and joint tumor society
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• v.16 no.1
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• pp.21-26
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• 2010

Purpose: To analyze clinical, radiological and pathological features as well as clinical outcome after surgical treatment of patients with secondary chondrosarcoma arising from osteochondroma(tosis). Materials and Methods: We retrospectively reviewed clinical records, radiographs, pathologic slides of 14 patients. Nine patients were male and fi ve were female. The mean age was 34 years. The mean follow-up period was 54 months. Results: All patients had a history of previous mass since childhood or puberty. Preexisted osteochondroma was single in 3 patients and multiple in 10. Remaining 1 patient had multiple osteochondromatosis with enchondromatosis. MRI clearly provided thickness of cartilage cap, which was over 2 cm except in 2 cases. Chondrosarcoma was grade 1 in all except 1 case, which was grade 2. Wide excision was performed in 10 patients, marginal excision in 3 and amputation in 1. Twelve patients were doing very well without evidence of disease. Among 3 patients with marginal excision, 1 patient had local recurrence and 1 patient died of disease. Conclusion: Comprehensive understanding of clinical, radiological and pathological features of secondary chondro sarcoma is warranted for accurate diagnosis. The best result can be expected with early recognition of malignant change of osteohcondroma(tosis) and wide excision.

• The Korean Journal of Nuclear Medicine Technology
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• v.20 no.1
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• pp.37-41
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• 2016

Purpose The importance of quality control by the error to a minimum, which for the purpose of enhancing the reliability of the examination is not be emphasized excess. Currently, most nuclear medicine laboratory are conducting the internal and external quality control, and they are applying the Levey-Jennings or Westgard Multi-Rules by using the commercialized quality control materials. The reliability of the nuclear medicine blood test which affects the diagnosis of patients and the treatment policy is being secured through this quality control activity. Therefore, researchers will evaluate the utility of the statistic quality control using the population distribution of the nuclear medicine blood test conducted targeting the checkup examinees by the additional technique of the reliability improvement. Materials and Methods A statistic analysis was performed about 12 items of the nuclear medicine blood test targeting 41,341 peoples who used the health screening and promotion center in Asan Medical Center from January, 2014 to December, 2014. The results of 12 items of the nuclear medicine blood test was divided into the monthly percentage of three groups: within reference values, over reference, and under reference to analyze the average value of the population distribution, standard deviation, and standard deviation index (SDI). Results The standard deviation of the population distribution mostly showed a result within ${\pm}2SD$ in all groups. However, When the standard deviation of the population distribution represented a result over ${\pm}2SD$, it was confirmed SDI was showing a result of SDI > -2 or SDI > 2. As a result of analyzing the population distribution of 12 items(AFP, CEA, CA19-9, CA125, PSA, TSH, FT4, Anti-Tg-Ab, Anti-TPO-Ab, Calcitonin, 25-OH-VitD3, Insulin) of the nuclear medicine blood part basic test, when SDI of the monthly percentage which deviated from the reference values was over ${\pm}2.0$, CA19-9 September was 2.2, Anti-Tg-Ab may was 2.2, Insulin January was 2.3, Insulin March was 2.4. It was confirmed these cases were attributed to the abnormality of the test reagent (maximum combination rate of isotope reagent declined) and the decline of the test response time. Conclusion The population distribution includes the entire attribute which becomes the study object. It is expected the statistic quality management using the population distribution which was conducted targeting the checkup examinees by dividing into three groups: within reference values, over reference, and under reference by means of this characteristics will be able to play a role of complementing the internal quality control program which is being carried out in the laboratory.

• The Journal of Internal Korean Medicine
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• v.28 no.1
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• pp.34-46
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• 2007

Objectives: This study was to assess characteristics in acute stroke patients according to Sasangconstitution (SC). Methods : We assessed the type of SC of acute stroke patients in $2^{nd}$ Department of Internal Medicine at KyungHee University Oriental Hospital from October 2005 to September 2006. Each subject's SC type was confirmed when the result of QSCC II+ was in accordance with the opinion of the Sasangconstitutional medicine specialist. We investigated general characteristics, stroke type, blood test results, alcohol drinking, smoking and dietary preferences according to SC. Results: 108 subjects were included in the final analysis. This study showed that out of the total patients, the proportion of So-yang to Tae-eum to So-eum was equal to 5.5 to 3.5 to 1. Especially, men were higher proportion So-yang while women recorded a more significant ratio of Tae-eum. Also, the BMI, waist circumference, hip circumference, and WC/HC ratio recorded significantly high results. The result indicated that even though the Tae-eum let on the highest numerical value in the aspect of their past history and the final diagnosis, no significant difference was revealed according to SC. The ratio of cerebral infarction to cerebral hemorrhage was 9 to 1. SVO, LAA, SUE are the 3 types of cerebral infarction classified by TOAST; SVO ranked the highest while SUE ranked the lowest in all constitutions. The blood test result displayed that averages of both TL and the T-chol were the highest in Tae-eum while that of hematocrit in So-yangand that of HDL-chol in So-eum. However, there was no significant difference. There were no significant differences classified by gender or by SC in the aspect of the alcohol drinking and smoking history. The majority of So-yang and Tae-eum demonstrated a preference for meat while So-eum and So-yang displayed high preferences for sea food. Conclusions : With respect to these results. we could observe the general disposition of various characteristic distributions according to SC of acute stroke patients. For further research in this field. it seems necessary to construct fundamental databases for the prevention and treatment of stroke by increasing the number of patients observed and by analyzing delicate characteristics of each constitution type.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.194-201
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• 2005

Purpose: Gall bladder (GB) stones in children are rarely observed, and so reports of them have been quite limited in Korea. Therefore, we tried to assess the epidemiology and clinical features of children with GB stone diagnosed at Severance Hospital. Methods: A clinical study was performed on 18 patients below 15 years of age diagnosed with GB stone by abdominal ultrasonography or CT scan at Severance hospital from January 1999 to May 2005. Results: Concerning patients' profile, their sex ratio of male to female in total 18 cases was 2 : 1 with the mean age of $6{\pm}4.3years$. 15 patients (83.3%) were asymptomatic. Inducing factors were found in 14 children (77.8%) including ceftriaxone therapy in 6 cases (33.3%), infection in 4 cases (22.2%), spherocytosis in 3 cases (16.7%), Down syndrome and abdominal operation in 1 case (5.6%) respectively. Single stone was found in 13 cases (76.5%) and multiple in 4 cases (23.5%). 15 cases (88.2%) had the stones less than 5 mm in size, and 2 cases (11.8%) between 5 and 10 mm. 17 patients received conservative treatment, and one patient had cholecystectomy due to Meckel diverticulum. Conclusion: In this report, Pediatric GB stones are predominant in male children, without typical symptoms, having inducing factors, single and small. Recently the diagnosis of them has been increased because of the development of imaging study. But the reports for them were still rare in Korea. Therefore the study for them is more necessary to find it's clinical characteristics.