• 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.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.44 no.1
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• pp.124-135
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• 1997

Background : Diffuse panbronchiolitis(DPB) is a disease characterized clinically by chronic cough, expectoration and dyspnea; and histologically by chronic inflammation localized mainly in the region of the respiratory bronchiole. It is prevalent in Japanese, but is known to be rare in Americans and Europians. Only a few cases in Chinese, Italians, North Americans and Koreans have been reported. It is diagnosed by characteristic clinical, radiological and pathologic features. High-resolution CT(HRCT) is known to be valuable in the study of the disease process and response to therapy in DPB. To our knowledge, there has been no correlation of its appearance on HRCT with the severity of the disease process, and radioaerosol scan(RAS) of the lung has not previously been used for the diagnosis of DPB. Method : During recent two years we have found 12 cases of DPB in Kangnam St. Mary's Hospital, Catholic University Medical College. We analysed the clinical characteristics, compared HRCT classifications with clinical stages of DPB, and determined characteristic RAS manifestations of DPB. Results : 1. The ages ranged from 31 to 83 years old(mean 54.5 years old), and male female ratio was 4:8. 75%(9/12) of patients had paranasal sinusitis, and only one patient was a smoker. 2. The patients were assigned to one of three clinical stages of DPB on the basis of clinical findings, sputum bacterology and arterial blood gas analysis. of 12 cases, 5 were in the first stage, 4 were in the second stage, and 3 were in the third stage. In most of the patients, pulmonary function tests showed marked obstructive and slight restrictive impairments. Sputum culture yielded P.aeruginosa in 3 cases of our 12 cases, K.pneumoniae in 2 cases, H.influenzae in 2 cases, and S.aureus in 2 cases. 3. Of 12 patients, none had stage I characteristics as classified on HRCT scans, 4 had slage II findings, 5 had stage III findings, and 3 had stage IV characteristics. 4. We peformed RAS in 7 of 12 patients With DPB. In 71.4% (5/7) of the patients, RAS showed mottled aerosol deposits characteristically in the transitional and intermediary airways with peripheral airspace defects, which contrasted sharply with central aerosol deposition of COPD. 5. There were significant correlations between HRCT stages and clinical stages(r= 0.614, P < 0.05), between HRCT types and Pa02(r= -0.614, P < 0.05), and between HRCT types and ESR(r= 0.618, P < 0.01). Conclusion : The HRCT classifications correspond well to the clinical stage. Therfore in the examination of patients with DPB, HRCT is useful in the evaluation of both the location and severity of the lesions. Also, RAS apears to be a convenient, noninvasive and useful diagnostic method of DPB.

• Progress in Medical Physics
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• v.20 no.4
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• pp.298-307
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• 2009

By using the MR T2 map technique, this study intends, first, to measure the change of T2 values of cartilage between healthy people and patients with osteoarthritis and, second, to assess the form and the damage of cartilage in the knee-joint, through which this study would consider the utility of the T2 map technique. Thirty healthy people were selected based on their clinical history and current status and another thirty patients with osteoarthritis of the knee who were screened by simple X-ray from November 2007 to December 2008 were selected. Their T2 Spin Echo (SE hereafter) images for the cartilage of the knee joint were collected by using the T2 SE sequence, one of the multi-echo methods (TR: 1,000 ms; TE values: 6.5, 13, 19.5, 26, 32.5. 40, 45.5, 52). Based on these images, the changes in the signal intensity (SI hereafter) for each section of the cartilage of the knee joint were measured, which yielded average values of T2 through the Origin 7.0 Professional (Northampton, MA 01060 USA). With these T2s, the independent samples T-test was performed by SPSS Window version 12.0 to run the quantitative analysis and to test the statistical significance between the healthy group and the patient group. Closely looking at T2 values for each anterior and lateral articular cartilage of the sagittal plane and the coronal plane, in the sagittal plane, the average T2 of the femoral cartilage in the patient group with arthritis of the knee ($42.22{\pm}2.91$) was higher than the average T2 of the healthy group ($36.26{\pm}5.01$). Also, the average T2 of the tibial cartilage in the patient group ($43.83{\pm}1.43$) was higher than the average T2 in the healthy group ($36.45{\pm}3.15$). In the case of the coronal plane, the average T2 of the medial femoral cartilage in the patient group ($45.65{\pm}7.10$) was higher than the healthy group ($36.49{\pm}8.41$) and so did the average T2 of the anterior tibial cartilage (i.e., $44.46{\pm}3.44$ for the patient group vs. $37.61{\pm}1.97$ for the healthy group). As for the lateral femoral cartilage in the coronal plane, the patient group displayed the higher T2 ($43.41{\pm}4.99$) than the healthy group did ($37.64{\pm}4.02$) and this tendency was similar in the lateral tibial cartilage (i.e., $43.78{\pm}8.08$ for the patient group vs. $36.62{\pm}7.81$ for the healthy group). Along with the morphological MR imaging technique previously used, the T2 map technique seems to help patients with cartilage problems, in particular, those with the arthritis of the knee for early diagnosis by quantitatively analyzing the structural and functional changes of the cartilage.

• The Korean Journal of Nuclear Medicine Technology
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• v.20 no.2
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• pp.3-8
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• 2016

Purpose SUV is one of the parameters that assist diagnosis in origin, metastasis and staging of cancer. Specially, it is important to compare SUV before and after chemo or radiation therapy to find out effectiveness of treatment. Storing PET data which has no quantitative change is needed for SUV comparison. However, there is a possibility to loss the data in external hard drive or MINIpacs that are managed by department of nuclear medicine. The aim of this study is to evaluate SUV and metabolic tumor volume (MTV) among reconstructed data (R-D) in workstation, R-D and re-sliced data (S-D) in PACS. Materials and Methods Data of 20 patients (aged $60.5{\pm}8.3y$) underwent $^{18}F-FDG$ PET (Biograph truepoint 40, mCT 40, mCT 64, mMR, Siemens) study were analysed. $SUV_{max}$, $SUV_{peak}$ and MTV were measured in liver, aorta and tumor after sending R-D in workstation, R-D and S-D in PACS to syngo.via software. Results R-D of workstation and PACS showed the same value as mean $SUV_{max}$ in liver, aorta and tumor were $2.95{\pm}0.59$, $2.35{\pm}0.61$, $10.36{\pm}6.15$ and $SUV_{peak}$ were $2.70{\pm}0.51$, $2.07{\pm}0.43$, $7.67{\pm}3.73$(p>0.05) respectively. Mean $SUV_{max}$ of S-D in PACS were decreased by 5.18%, 7.22%, 12.11% and $SUV_{peak}$ 2.61%, 3.63%, 10.07%(p<0.05). Correlation between R-D and S-D were $SUV_{max}$ 0.99, 0.96, 0.99 and $SUV_{peak}$ 0.99, 0.99, 0.99. And 2SD in balnd-altman analysis were $SUV_{max}$ 0.125, 0.290, 1.864 and $SUV_{peak}$ 0.053, 0.103, 0.826. MTV of R-D in workstation and PACS show the same value as $14.21{\pm}12.72cm^3$(p>0.05). MTV in PACS was decreased by 0.12% compared to R-D(p>0.05). Correlation and 2SD between R-D and S-D were 0.99 and 2.243. Conclusion $SUV_{max}$, $SUV_{peak}$, MTV showed the same value in both of R-D in workstation and PACS. However, there was statistically difference in $SUV_{max}$, $SUV_{peak}$ of S-D compare to R-D despite of high correlation. It is possible to analyse reliable pre and post SUV if storing R-D in main hospital PACS system.

• 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.8 no.2
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• pp.186-194
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• 2004

Purpose: Membranoproliferative glomeulonephritis(MPGN) has been diagnosed in an increasing number of asymptomatic cases. These cases have been detected by school urinary screening test even though the total cases of MPGN show a decreasing trend. We have analyzed the clinical and pathological characteristics of children with MPGN according to the clinical manifestations at the time of disease presentation. Methods: A total of 18 patients who had been diagnosed with idiopathic MPGN by percutaneous renal biopsy from January 1990 to February 2004 were involved in our study. The patients were divided into 2 groups as the school urinary screening(A) group and the symptomatic(S) group according to the clinical manifestations at the time of disease presentation. Results: Out of the total 18 patients, 8(44.4%) were in the S group and 10(55.6%) were in the A group. The mean serum total protein, albumin and $C_3$ levels in the S group were significantly lower than those levels of the A group, respectively($4.9{\pm}1.2\;g/dL,\;vs\;7.0{\pm}0.5\;g/dL\;P=0.002,\;2.8{\pm}0.9\;g/dL\;vs.\;4.1{\pm}0.3\;g/dL\;P=0.002,\;63.9{\pm}36.4\;mg/dL\;vs.\;100.8{\pm}39.5\;g/dL\;P=0.041$). The mean total protein amount of 24 hour collected urine in the S group were significantly higher than that of the A group($3684.0{\pm}2601.3\;mg/m^2\;vs.\;559.4{\pm}4.6.9\;mg/m^2$, respectively, P=0.001). Hypocomplementemia was observed in 11(61.1%) out of 18 patients at the time of disease onset, 7(87.5%) in the S group and 4(40%) in the A group. However the hypocomplementemia was decreased in 6(33.3%) out of 18 patients at the time of final follow-up, 3(37.5%) in the S group and 3(30%) in the A group. According to the pathologic type, hypocomplementemia was observed 8 patients(61.5%) with type I disease, 1 patients (100%) with type II disease, 2 patients(50%) in type III disease at the disease onset, but 4 patients(30.8%) in type I disease, 1 patient(100%) in type II disease, 1 patient(33.3%) with type III disease at the time of last follow-up. The incidence of cellular crescent formation and tubular atropy. as observed on light microscopy, were higher in the S group compared to the A group. Mean grade of capillary wall thickening and, mesangial proliferation were significantly higher in the S group. Conclusion: MPGN, as diagnosed in patients with only asymptomatic urinary abnormalities, has been increasing, it is more frequent in asymptomatic patients than in patients with presenting symptoms. Our result suggests that MPGN should be considered in the renal biopsy diagnosis regardless of serum $C_3$ level when urinary abnormalities are found by school urinary screening test.

• Tuberculosis and Respiratory Diseases
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• v.39 no.5
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• pp.417-424
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• 1992

Background: There were many reports about the clinical aspect and outcomes of pulmonary tuberculosis in health center but few in a medium sized general hospital. The purposes of this study were to find any characteristic differencies in the patients and the general outcomes of the treatments and also to give some suggestive points for the insurance policy making. Methods: We made a retrograde analysis of the medical records of 1981 patients (male 992 female 1,059) who attended our clinics of the 4th internal department, Daedong general hospital during two years from January 1989 to December 1990. Result: 1) Of 1981 patients, 96 were diagnosed as pulmonary tuberculosis taking relatively large proportion in the prevalence. The ratio of prevalence between male and female was 7.81% to 2.27%. The 61.46% were the first diagnosis & initial treatment cases and the remaining 38.54% were the retreatment cases with no statistical significance between sex. 2) The most prevalent age group was between 21~40 years old and the prevalence rate was 45.45% of male and 76.76% of female. The lowest age group in male patient was above 61 showed 3.03%, and there was no female patients above age 60 years old. This phenomena could be thought as the negligence for the treatment of pulmonary tuberculosis in the old age groups rather than true tuberculosis prevalence and it could be proved by the higher rates towards old age groups in the national tuberculosis prevalence survey. 3) There were 57.07% of the minimal case, 48.96% of the moderate, 18.75% of the far advanced. The sputum examination showed 37.07% were culture positive, 46.88% were the negative, and 15.63% of the patients had no stutum examination. Moreover, uncoperatives among the far advanced cases were notable showing 22.22% of the stutum examination, where 16.13% in the minimal cases. The stutum positive rate among the initial treatment cases were 41.07% and 55.00% for the retreatment cases. The sputum no examination rates were 17.86% and 12.50% respectively. 4) The classfication of the mode of disease onset showed 68.75% with gradual onset, 9.38% hemoptic, 3.13% acute pneumonic and 18.75% was found through the radiologic examination in various occasions. 5) The percentages of patients who continued their treatment for more than 8 month were 35.71% (for initial treatment), 25.00% (for retrement), 16.13% (for the minimal), 27.78% (for the far advanced). 6) The group of patients who were treated more than 8 months showed the negative conversion rate of 80% on sputum and marked improvement on chest x-ray in 56.67%. However, in far advanced or retreatment cases, the rate of negative conversion on sputum and the rate of improvement on chest x-ray were low being 60% and 20% for the former and 60% and 10% for the latter, each respectively. Conclusion: It would be strongly emphasized that the improvement of National medical insurance system and social welfare system in Korea must be definite to improve overall treatment and control of tuberculosis diseases as well as physician's devotious National tuberculosis control policy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.1 no.1
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• pp.45-55
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• 1998

Purpose: To make objective standards of small intestinal mucosal changes in cow's milk-sensitive enteropathy (CMSE) we analyzed histological changes of endoscopic duodenal mucosa biopsy specimens from normal children and patients of CMSE. Methods: We review the medical records of patients who had been admitted and diagnosed as CMSE by means of gastrofiberscopic duodenal mucosal biopsy following cow's milk challenge and withdrawal. Thirteen babies with CMSE, ranging from 14 days to 56 days of age, were studied. Five non-CMSE patients were used as control, ranging from 22 days to 72 days of age. The morphometric parameters under study were villous height, crypt zone depth, ratio of villous height to crypt zone depth, total mucosal thickness and length of surface epithelium by using H & E stained specimens under the drawing apparatus attached microscope. In addition, the numbers of lymphocytes in the epithelium and eosinophil cells in the lamina propria and epithelium were measured. Results: In the duodenal mucosal biopsy specimens in CMSE we found partial and subtotal villous atrophy with an increased number of interepithelial lymphocytes. The mean villous height($135{\pm}59\;{\mu}m$), ratio of villous height to crypt zone depth ($0.46{\pm}0.28$), total mucosal thickness ($499{\pm}56\;{\mu}m$), length of surface epithelium of small intestinal mucosa ($889{\pm}231\;{\mu}m$) in CMSE was significantly decreased compared with the control (p<0.05). The mean crypt zone depth ($311{\pm}65\;{\mu}m$) was significantly greater than the control ($188{\pm}24\;{\mu}m$)(p<0.05). Infiltration of interepithelial lymphocytes ($34.1{\pm}10.5$) were significantly greater than the control ($13.6{\pm}3.6$)(p<0.05). The number of eosinophil cells in both lamina propria and epithelium was no significant differences between groups (p>0.05). The small intestinal mucosa in treated CMSE showed much improved enteropathy of villous height, crypt zone depth, interepithelial lymphocytes compared with the control as well as untreated CMSE. Conclusion: Quantitation of mucosal dimensions confirmed the presence of CMSE. It seems to be a limitation in the capacity of crypt cells to compensate for the loss of villous epithelium in CMSE. Specimens obtained by gastrofiberscopic duodenal mucosal biopsy were suitable for morphometric diagnosis of CMSE. Improvement of CMSE also can be confirmed histologically after the therapy of protein hydrolysate.

• 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)