• Tuberculosis and Respiratory Diseases
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• v.44 no.6
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• pp.1308-1317
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• 1997

Background : Pulmonary aspergillomas usually arise from colonization and proliferation of Aspergillus in preexisting cavitary lung disease of any cause. About 15% of patients with tuberculous pulmonary cavities were found to have aspergilloma. We analyzed the clinical features and course of 91 patients with pulmonary aspergilloma. Method : During the ten-year period from June 1986 to May 1996, 91 patients whose condition was diagnosed as pulmonary aspergilloma at 4 university hospitals in Taegu city were reviewed. All patients fulfilled one of the following criteria : 1) histologic evidence of aspergilloma within abnormal air space in tissue sections, or 2) a positive Aspergillus serum precipitin test with the radiologic finding of a fungus ball. The histological diagno-sis was established in 81 patients(89.0%) and clinical diagnosis in 10 patients(11.0%). Results : 1) The age range was 22 to 65 years, with an average of 45 years. A male and female ratio was 1.7 : 1 (57 men and 34 women). 2) Hemoptysis was far the most frequent symptom(89%), followed by cough, dyspnea, weakness, weight loss, fever, chest pain. 3) In all but 14 cases(15.4%) there had been associated conditions. Pulmonary tuberculosis was far the most frequent underlying condition found(74.7%), followed by bronchiectasis (6.6%), cavitary neoplasm(2.2%), pulmonary sequestration(1.1%). 4) The involved area was usually in the upper lobes; the right upper lobe was involved in 39(42.9%), the left upper lobe in 31(34.1%), the left lower lobe in 13(14.3%), the right lower lobe in 7(7.7%), and the right middle lobe in 1(1.1%). 5) On standard chest roent geno gram the classic "bell-like" image of a fungus ball was found in 62.6% of the subjects. On CT scan, 88.1% of the subjects in which they were done. 6) The surgical therapy was undertaken in 76 patients, and medical therapy in 15 patients, including 4 patients with intracavitary instillation of amphotericin B. 7) The surgical modality was lobectomy in 55 patients(72.4%), segmentectomy in 16 patients(21.1%), pneumonectomy in 4 patients(5.3%), wedge resection in 1 patient(1.3%). The mortality rate was 3.9% (3 patients) ; 2 patients died of sepsis and 1 died of hemoptysis. The postoperative complications were encountered in 6 patients (7.9%), including each one patient with respiratory failure, bleeding, bronchopleural fistula, empyema, and vocal cord paralysis. 8) In the follow-up cases, each 2 patients of 71 patients with surgical treatment and 10 patients with medical treatment had recurrent hemoptysis. Conclusion : During follow-up of the chronic pulmonary disease with abnormal air space, if the standard chest roentgenograms are insufficient to detect a fungus ball, computed tomographic scan and serum precipitin test are likely to aid the diagnosis of patients with suspected pulmonary aspergilloma. A reasonable recommendation for management of a patient with aspergilloma would be to reserve surgical resection for those patients who have had severe, recurrent hemoptysis. And a well controlled cooperative study to the medical treatment such as intracavitary antifungal therapy is further needed.

• The Korean Journal of Nuclear Medicine Technology
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• v.14 no.2
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• pp.3-8
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• 2010

Purpose: $^{11}C$-Acetate PET/CT is useful in detecting lesions that are related to livers in the human body and leads to a sensitivity of 87.3%. On the other hand, $^{18}F$-FDG PET/CT has a sensitivity of 47.3% and it has been reported that if both $^{18}F$-FDG and $^{11}C$-Acetate PET/CT are carried out together, their cumulative sensitivity is around 100%. However, the normal intake of the pancreas and the spleen in $^{11}C$-Acetate PET/CT can influence the $^{18}F$-FDG PET/CT leading to an inaccurate diagnosis. This research was aimed at the verification of the usefulness of how much influence these two radioactive medical supplies can cause on the medical images through comparative analysis between the one-day and two-day protocol. Materials and Methods: This research was carried out based on 46 patients who were diagnosed with liver cancer and have gone through the PET/CT (35 male, 11 female participants, average age: $54{\pm}10.6$ years, age range: 29-69 years). The equipment used for this test was the Biograph TruePoint40 PET/CT (Siemens Medical Systems, USA) and 21 participants who went through the one-day protocol test were first given the $^{11}C$-Acetate PET/CT and the $^{18}F$-FDG PET/CT, the latter exactly after one hour. The other 25 participants who went through the two-day protocol test were given the $^{11}C$-Acetate PET/CT on the first day and the $^{18}F$-FDG PET/CT on the next day. These two groups were then graded comparatively by assigning identical areas of interest of the pancreas and the spleen in the $^{18}F$-FDG images and by measuring the Standard Uptake Value (SUV). SPSS Ver.17 (SPSS Inc., USA) was used for statistical analysis, where statistical significance was found through the unpaired t-test. Results: After analyzing the participants' medical images from each of the two different protocol types, the average${\pm}$standard deviation of the SUV of the pancreas carried out under the two-day protocol were as follows: head $1.62{\pm}0.32$ g/mL, body $1.57{\pm}0.37$ g/mL, tail $1.49{\pm}0.33$ g/mL and the spleen $1.53{\pm}0.28$ g/mL. Whereas, the results for participants carried out under the one-day protocol were as follows: head $1.65{\pm}0.35$ g/mL, body $1.58{\pm}0.27$ g/mL, tail $1.49{\pm}0.28$ g/mL and the spleen $1.66{\pm}0.29$ g/mL. Conclusion: It was found that no statistical significant difference existed between the one-day and two-day protocol SUV in the pancreas and the spleen (p<0.05), and nothing which could be misconceived as false positive were found from the PET/CT medical image analysis. From this research, it was also found that no overestimation of the SUV occurred from the influence of $^{11}C$-Acetate on the $^{18}F$-FDG medical images where those two tests were carried out for one day. This result was supported by the statistical significance of the SUV of measurement. If $^{11}C$-Acetate becomes commercialized in the future, the diagnostic ability of liver diseases can be improved by $^{18}F$-FDG and one-day protocol. It is from this result where tests can be accomplished in one day without the interference phenomenon of the two radioactive medical supplies and furthermore, could reduce the waiting time improving customer satisfaction.

• The korean journal of orthodontics
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• v.29 no.4 s.75
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• pp.467-481
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• 1999

Cleft lip and/or palate is one of the most common congenital craniofacial anomalies. According to previous epidemiologic studies, incidence of cleft lip and/or palate has been increasing nowadays. However, there is no report about epidemiologic study of cleft lip and/or palate patients who visited dept. of orthodontics in Korea. So the purpose of this study was to provide the epidemiological characteristics and important basic clinical data for the diagnosis and the treatment of the cleft lip and/or palate patients. With the orthodontic and cleft charts, diagnostic models and X-ray films from 250 patients with cleft lip and/or palate who visited Dept. of Orthodontics, Seoul National University Dental Hospital during the last 11 years, the authors investigated patient's visiting yew, types of cleft, patient's gender, and Angle's classification of malocclusion, and surgery timing. The results were as follows ; 1. The number of cleft patients who visited Dept. of Orthodontics, SNUDH increased during 1988-1990 and then it declined until 1992. From 1993 to 1996, it showed a stationary trend. After 1997 it showed an overwhelmingly increasing trend. 2. In the cleft type, the ratio of cleft lip cleft lip and alveolus cleft palate : cleft lip and palate was 7.6:19.2:9.6:63.6. In cleft position, unilateral clefts were more than bilateral ones (cleft lip 79:21, cleft lip and alveolus 77:23, cleft lip and palate 75.5:24.5). In cleft side, left clefts were mote than right clefts (cleft lip 53.3:46.7 cleft lip and alveolus 59.5:40.5, cleft lip and palate 59.2:40.8). 3. In gender ratio, males were more than females in cleft lip (57.9:42.1), cleft lip and alveolus (68.8:31.2) and cleft lip and palate (76.1:23.9). But in cleft Palate females were more than males as 41.7: 58.3. 4. In the age groups, 7-12 year group was the most abundant as $52\%$, and then 0-6 year group ($20.4\%$), 13-18 year group ($17.2\%$), more than 18 yew group ($10.4\%$) were followed as descending order. 5. Most of the cleft lip repair surgeries were operated in 0-3 month ($60.3\%$) and 4-6 month ($17.9\%$). 6. The cleft palate repair surgeries were done in 1-2 year ($31.7\%$), 0-1 year ($25.6\%$), 2-3 year ($12.1\%$), more than 5 year ($11.6\%$) as descending order. 7. The lip scar revision surgeries were done before admission at elementary school in $60\%$. (4-6 you ($27.5\%$), 6-8 year ($19.6\%$), more than 10 year ($19.6\%$), 2-4 year ($13.7\%$) as descending order) 8. The rhinoplasties were done before admission at elementary school in $51.7\%$. (0-2 year ($7.1\%$), 2-4 year ($14.3\%$), 4-6 year ($21.4\%$), 6-8 year ($14.3\%$)). 9. The pharyngeal flap were done at 6 Y (72.5 months) after birth on average and there was even distribution of surgery timing. 10. In relationship between Angle's classification of malocclusion and cleft types, Class I was most abundant and Class III, Class II were followed as descending order in cleft lip group. But Class III was most abundant and Class I, Class II were followed as descending order in cleft lip and alveolus group, cleft palate group, and cleft lip and Palate group. The percentage of frequency in Class III malocclusion was overwhelmingly higher in cleft lip and palate group than any other groups. 11. Because the frequency of class III malocclusion was most prevalent in all age groups, anterior crossbite was the most common chief complaint of cleft patients.

• Radiation Oncology Journal
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• v.26 no.4
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• pp.247-256
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• 2008

Purpose: The purpose of this study is to evaluate anal sphincter preservation rates, survival rates, and prognostic factors in patients with rectal cancer treated with preoperative chemoradiotherapy. Materials and Methods: One hundred fifty patients with pathologic confirmed rectal cancer and treated by preoperative chemoradiotherapy between January 1999 and June 2007. Of the 150 patients, the 82 who completed the scheduled chemoradiotherapy, received definitive surgery at our hospital, and did not have distant metastasis upon initial diagnosis were enrolled in this study. The radiation dose delivered to the whole pelvis ranged from 41.4 to 46.0 Gy (median 44.0 Gy) using daily fractions of $1.8{\sim}2.0\;Gy$ at 5 days per week and a boost dose to the primary tumor and high risk area up to a total of $43.2{\sim}54\;Gy$ (median 50.4 Gy). Sixty patients (80.5%) received 5-fluorouracil, leucovorin, and cisplatin, while 16 patients (19.5%) were administered 5-fluorouracil and leucovorin every 4 weeks concurrently during radiotherapy. Surgery was performed for 3 to 45 weeks (median 7 weeks) after completion of chemoradiotherapy. Results: The sphincter preservation rates for all patients were 73.2% (60/82). Of the 48 patients whose tumor was located at less than 5 cm away from the anal verge, 31 (64.6%) underwent sphincter-saving surgery. Moreover, of the 34 patients whose tumor was located at greater than or equal to 5 cm away from the anal verge, 29 (85.3%) were able to preserve their anal sphincter. A pathologic complete response was achieved in 14.6% (12/82) of all patients. The downstaging rates were 42.7% (35/82) for the T stage, 75.5% (37/49) for the N stage, and 67.1% (55/82) for the overall stages. The median follow-up period was 38 months (range $11{\sim}107$ months). The overall 5-year survival, disease-free survival, and locoregional control rates were 67.4%, 58.9% and 84.4%, respectively. The 5-year overall survival rates based on the pathologic stage were 100% for stage 0 (n=12), 59.1% for stage I (n=16), 78.6% for stage II (n=30), 36.9% for stage III (n=23), and one patient with pathologic stage IV was alive for 43 months (p=0.02). The 5-year disease-free survival rates were 77.8% for stage 0, 63.6% for stage I, 58.9% for stage II, 51.1% for stage III, and 0% for stage IV (p<0.001). The 5-year locoregional control rates were 88.9% for stage 0, 93.8% for stage I, 91.1% for stage II, 68.2% for stage III, and one patient with pathologic stage IV was alive without local recurrence (p=0.01). The results of a multivariate analysis with age (${\leq}55$ vs. >55), clinical stage (I+II vs. III), radiotherapy to surgery interval (${\leq}6$ weeks vs. >6 weeks), operation type (sphincter preservation vs. no preservation), pathologic T stage, pathologic N stage, pathologic overall stage (0 vs. I+II vs. III+IV), and pathologic response (complete vs. non-CR), only age and pathologic N stage were significant predictors of overall survival, pathologic overall stage for disease-free survival, and pathologic N stage for locoregional control rates, respectively. Recurrence was observed in 25 patients (local recurrence in 10 patients, distant metastasis in 13 patients, and both in 2 patients). Acute hematologic toxicity ($\geq$grade 3) during chemoradiotherapy was observed in 2 patients, while skin toxicity was observed in 1 patient. Complications developing within 60 days after surgery and required admission or surgical intervention, were observed in 11 patients: anastomotic leakage in 5 patients, pelvic abscess in 2 patients, and others in 4 patients. Conclusion: Preoperative chemoradiotherapy was an effective modality to achieve downstaging and sphincter preservation in rectal cancer cases with a relatively low toxicity. Pathologic N stage was a statistically significant prognostic factor for survival and locoregional control and so, more intensified postoperative adjuvant chemotherapy should be considered in these patients.

• Advances in pediatric surgery
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• v.8 no.2
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• pp.143-155
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• 2002

A survey on biliary atresia was made among 26 members of the Korean Association of Pediatric Surgeons. The members were required to complete a questionnaire and a case registration form for each patient during the twentyone-year period of 1980-2000. Three hundred and eighty patients were registered from 18 institutions. The average number of patients per surgeon was one to two every year. The male to female ratio was 1:1.3. The age of patients on diagnosis with biliary atresia was on average $65.4{\pm} 36.2$ days old. The national distribution was 32.8% in Seoul, 25.3% in Gyoungki-Do, 21.6% in Gyoungsang-Do, 9.27% in Choongchung-Do, etc. in order. The most common clinical presentation was jaundice (98.4%) and change of stool color (86.2%) was second. Two hundred eighty (74.7%) of 375 patients were operated by 80 days of age. Three hundred thirty six (9 1.9%) of 366 patients were operated on by the original Kasai procedure, and 305 (84.3%) of 362 patients were observed by bile-drainage postoperatively. The overall postoperative complication rate was 18.5% and the overall postoperative mortality rate was 6.8%. The associated anomalies were observed in 72 cases (22.5%). One hundred ninty five (64.7%) of 302 patients have been alive in follow-up and 49 (25.1%) have survived over 5 years without problem after operation. Ascending cholangitis, varices and ascites affected survival significantly, and the important long-term prognostic factor was the occurrence of complications.

• Radiation Oncology Journal
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• v.22 no.4
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• pp.237-246
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• 2004

Purpose: To investigate the effects of radiation dose-escalation on the treatment outcome, complications and the other prognostic variables for glioblastoma patients treated with 3D-conformal radiotherapy (3D-CRT). Materials and Methods: Between Jan 1997 and July 2002, a total of 75 patients with histologically proven diagnosis of glioblastoma were analyzed. The patients who had a Karnofsky Performance Score (KPS) of 60 or higher, and received at least 50 Gy of radiation to the tumor bed were eligible. All the patients were divided into two arms; Arm 1, the high-dose group was enrolled prospectively, and Arm 2, the low-dose group served as a retrospective control. Arm 1 patients received $63\~70$ Gy (Median 66 Gy, fraction size $1.8\~2$ Gy) with 3D-conformal radiotherapy, and Arm 2 received 59.4 Gy or less (Median 59.4 Gy, fraction size 1.8 Gy) with 2D-conventional radiotherapy. The Gross Tumor Volume (GTV) was defined by the surgical margin and the residual gross tumor on a contrast enhanced MRI. Surrounding edema was not included in the Clinical Target Volume (CTV) in Arm 1, so as to reduce the risk of late radiation associated complications; whereas as in Arm 2 it was included. The overall survival and progression free survival times were calculated from the date of surgery using the Kaplan-Meier method. The time to progression was measured with serial neurologic examinations and MRI or CT scans after RT completion. Acute and late toxicities were evaluated using the Radiation Therapy Oncology Group neurotoxicity scores. Results: During the relatively short follow up period of 14 months, the median overall survival and progression free survival times were $15{\pm}1.65$ and $11{\pm}0.95$ months, respectively. The was a significantly longer survival time for the Arm 1 patients compared to those in Arm 2 (p=0.028). For Arm 1 patients, the median survival and progression free survival times were $21{\pm}5.03$ and $12{\pm}1.59$ months, respectively, while for Arm 2 patients they were $14{\pm}0.94$ and $10{\pm}1.63$ months, respectively. Especially in terms of the 2-year survival rate, the high-dose group showed a much better survival time than the low-dose group; $44.7\%$ versus $19.2\%$. Upon univariate analyses, age, performance status, location of tumor, extent of surgery, tumor volume and radiation dose group were significant factors for survival. Multivariate analyses confirmed that the impact of radiation dose on survival was independent of age, performance status, extent of surgery and target volume. During the follow-up period, complications related directly with radiation, such as radionecrosis, has not been identified. Conclusion: Using 3D-conformal radiotherapy, which is able to reduce the radiation dose to normal tissues compared to 2D-conventional treatment, up to 70 Gy of radiation could be delivered to the GTV without significant toxicity. As an approach to intensify local treatment, the radiation dose escalation through 3D-CRT can be expected to increase the overall and progression free survival times for patients with glioblastomas.

• Nuclear Medicine and Molecular Imaging
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• v.42 no.1
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• pp.29-38
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• 2008

Purpose: The aim of this study was to investigate the feasibility of 3 ' -[F-18]fluoro-3 ' -deoxythymidine positron emission tomography(FLT-PET) for the detection of locally advanced breast cancer and to compare the degree of FLT and 2' -deoxy-2 ' -[F-18]fluoro-d-glucose(FDG) uptake in primary tumor, lymph nodes and other normal organs. Material & Methods: The study subjects consisted of 22 female patients (mean age; $42{\pm}6$ years) with biopsy-confirmed infiltrating ductal carcinoma between Aug 2005 and Nov 2006. We performed conventional imaging workup, FDG-PET and FLT PET/CT. Average tumor size measured by MRI was $7.2{\pm}3.4$ cm. With visual analysis, Tumor and Lymph node uptakes of FLT and FDG were determined by calculation of standardized uptake value (SUV) and tumor to background (TB) ratio. We compared FLT tumor uptake with FDG tumor uptake. We also investigated the correlation between FLT tumor uptake and FDG tumor uptake and the concordant rate with lymph node uptakes of FLT and FDG. FLT and FDG uptakes of bone marrow and liver were measured to compare the biodistribution of each other. Results: All tumor lesions were visually detected in both FLT-PET and FDG-PET. There was no significant correlation between maximal tumor size by MRI and SUVmax of FLT-PET or FDG-PET (p>0.05). SUVmax and $$SUV_{75} (average SUV within volume of interest using 75% isocontour) of FLT-PET were significantly lower than those of FDG-PET in primary tumor (SUVmax; $6.3{\pm}5.2\;vs\;8.3{\pm}4.9$, p=0.02 /$SUV_{75};\;5.3{\pm}4.3\;vs\;6.9{\pm}4.2$, p=0.02). There is significant moderate correlation between uptake of FLT and FDG in primary tumor (SUVmax; rho=0.450, p=0.04 / SUV75; rho=0.472, p=0.03). But, TB ratio of FLT-PET was higher than that of FDG-PET($11.7{\pm}7.7\;vs\;6.3{\pm}3.8$, p=0.001). The concordant rate between FLT and FDG uptake of lymph node was reasonably good (33/34). The FLT SUVs of liver and bone marrow were $4.2{\pm}1.2\;and\;8.3{\pm}4.9$. The FDG SUVs of liver and bone marrow were $1.8{\pm}0.4\;and\;1.6{\pm}0.4$. Conclusion: The uptakes of FLT were lower than those of FDG, but all patients of this study revealed good FLT uptakes of tumor and lymph node. Because FLT-PET revealed high TB ratio and concordant rate with lymph node uptakes of FDG-PET, FLT-PET could be a useful diagnostic tool in locally advanced breast cancer. But, physiological uptake and individual variation of FLT in bone marrow and liver will limit the diagnosis of bone and liver metastases.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.17-24
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• 2000

Purpose: MCNS is found in approximately $85\%$ of the idiopathic nephrotic syndrome in children and shows good prognosis with initial steroid therapy. However in FSGS, there is poor prognosis with initial therapy and shows higher rate of progression to chronic renal failure and relapse after kindney transplantation. We have experienced 8 patients who were diagnosed as MCNS on initial renal biopsy and then progressed to FSGS on follow-up biopsy. So we have investigated their clinical course and risk factors for transition of MCNS to FSGS. Methods: We conducted a retrospective study with a review of histopathologic findings and clinical manifestations of 296 cases of MCNS and FSGS that were diagnosed from January 1988 to May 1999. We classified them into 3 groups according to the histopathologic finding; MCNS, FSGS, MCNS progressed to FSGS in follow-up biopsy. Results: The number of children was 296 cases comprising 241 cases($81.4\%$) showing MCNS, 8 cases($2.7\%$) transition group, 47 cases($15.9\%$) FSGS. The mean onset age was $6.0{\pm}2.6$years in MCNS, transition group $8.3{\pm}2.3$years, FSGS $7.2{\pm4.3$years, and the gender (M:F) ratio was 3.7:1 in MCNS, 3:1 in transition group, 1.8:1 in FSGS. Comparing the presence of initial hematuria, hypertension,24 hour urine protein, serum albumin, serum creatinine, there were significant difference between the transition group and the FSGS group in the following points; 24hour urine protein $684:342mg/m^2/hr$(P<0.05), serum albumin 1.92: 2.47g/dL(P<0.05), serum cholesterol 494:343mg/dL(P<0.05). Refractoriness to steroid therapy was 13.3$\%$ in MCNS. $12.5\%$ in transition group, $29.6\%$ in FSGS; significantly higher in FSGS(P<0.05). Immunosuppressant therapy was performed in $58.5\%$ of MCNS, $100\%$ in transition group, $80.8\%$ in FSGS; transition group showed significantly higher .ate(P<0.05) comparing with MCNS. Mean number of relapse and duration from onset to first relapse showed no significance difference between these groups. Conclusion: 249 patients with MCNS have been followed and $3.2\%$ (8 patients) of them has shown change in pathologic diagnosis from MCNS to FSCS. The risk factor for transition could not be found. Our results point to the need for a follow-up biopsy to certify the possibility of transition to FSCS in some MCNS cases with refractory cases to steroid therepy, frequent relapsing cases, or in case of no remission in spite of vigorous immunosuppressant therapy.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.25-32
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• 2000

Purpose: This retrospective study of 126 children with symptomless primary hematuria was undertaken to determine the distribution of various histologic types by renal biopsy, clinical outcome according to the biopsy findings and also to find out feasibility of performing renal biopsy in these children. Patients and Methods : Study population consisted of 126 children with symptom-less primary hematuria who have been admitted to the pediatric department of Kyung-poot National University Hospital for the past 11 years from 1987 to 1998 and renal biopsy was performed percutaneously. Hematuric children with duration of less than 6 months, evidences of systemic illness such as SLE or Henoch-Schonlein purpura, urinary tract infection, and idiopathic hypercalciuria were excluded from the study. Results : Mean age of presentation was 9.2${\pm}$3.3 years (range ; 1.5-15.3 years) and male preponderance was noted with male to female ratio of 2:1. IgA nephropathy was the most common biopsy finding occuring in 60 children ($47.6\%$), followed by MsPGN in 13 ($10.3\%$), MPGN in 5 ($3.9\%$), TGBM in 6 ($4.7\%$), Alport syndrome in 2 ($1.6\%$), FSGS in 1 ($0.8\%$), and in 39 children ($30.9\%$), 'normal' glomeruli were noted. Recurrent gross hematuria was more common than persistent microscopic hematuria (84 versus 42), and especially in IgA nephropathy, recurrent gross hematuria was the most prevalent pattern of hematuria. In 58 out of 126 cases ($46.0\%$), hematuria was isolated without accompa-nying proteinuria and this was especially true In cases of MsPGN and 'normal' glomer-uli by biopsy finding. Normalization of urinalysis (disappearance of hematuria) in IgA nephropathy, MsPGN and 'normal' glomuli group were similar and it was $14\%,\;27\%\;and\;21\%$ respectively during 1-2 years of follow-up period, and $37.1\%,\;40\%\;and\;35\%$ respectively during 3-4 years of follow-up periods. However, abnormal urinalysis persi-sted in the majority of children with MPGN, TGBM. Alport syndrome and FSGS. Renal function deteriorated progressively in 6 cases (3 with IgA nephropathy, 2 with Alport syndrome and 1 with TGBM). Conclusion : In summary, present study demonstrates that in 126 children with symptomless primary hematuria, IgA nephropathy was the most common biopsy findings followed by MsPGN, MPGN, TGBM, Alport syndrome and FSGS, and 'normal glomeruli' was also seen in 39 cases ($30.9\%$). Renal histology could not be predictable on the clinical findings, so that to establish appropriate long-term planning for these children, we would recommend to obtain precise histologic diagnosis by renal biopsy.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.222-230
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• 2005

Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)