• Journal of Hospice and Palliative Care
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• v.8 no.2
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• pp.143-151
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• 2005

Purpose: Bereavement Memorial Service has been held every year by the hospice team at St. Vincent's Hospital for the purpose of supporting the bereaved family who feel grief and mourning. The purpose of this study is to find out the attitude of the bereaved attending at bereavement memorial service (BMS) and to find out the areas needing improvements to set up better memorial service. Methods: Hospice team sent invitation card to 180 families of patients who admitted and passed away at hospice ward Nov., 2003${\sim}$Oct., 2004. Among them 22 families attended the BMS meeting, which was held on 26th Nov., 2004. The researcher collected data from 22 families with 'Questionnaire' survey. Except identifying data and 2 dichotomy questions, we used open-ended questionnaire. 1 researcher conducted a telephone interview survey in 18 families who couldn't attend at BMS meeting. Results: The median age was 56 (range $16{\sim}19$) and there were 37 females and 3 males. They were patient's wife (22), mother (4), husband (5), daughter (4), mother-in-law (1), siblings (1), brothers wife (1). Duration after bereavement, $1{\sim}3$ months (17) was the highest frequency. 36 families agreed 'the dead experienced the death with dignity'. The reason of agreement to the death with dignity was 'the patient died in preparation' (16). 'the patient died in well-being condition spiritually' (9), 'the patient died in comfort physically (7). 4. persons thought the dead died with indignity. The bereaved defined 'the death with dignity' as follows: 'acceptance of death & death in spiritual well-being' (9), 'death in physical comfort condition' (7), 'the death in psycho-social well-being' (3), non-respondents (10). Most families (21) were still in difficulty to overcome bereavement grief. The answer regarding the method to overcome the difficulty was 'with spiritual sublimation' (13), 'with devotion of oneself in daily life' (10), 'with devotion to mourning as it is' (3). With regard to their attitude to invitation, 'having joy and thanks from hospice team' (21), 'grief' (4), 'suffering' (4). Toward the existence of hesitation about attendance at BMS meeting, the result as follows. Nonexistence of hesitation respondent (34), existence respondent (6), the reason for hesitation was various; 'the meeting reminds me of the suffering times', 'the meeting makes me to recall, and it will be likely to cry', and so on. The needs and feelings to memorial service meeting were various; 'it was meaningful time', 'it was good to recall about the dead', 'more meeting annually' and so on. In respect of the most difficulty after bereavement, in attendant family, 'depression' (10) was the highest frequency, whereas, in non-attendant family, the most difficult thing was 'financial problem/role difficulty (6). Conclusion: This study shows the rate of attendance was high in bereaved whose bereavement duration $1{\sim}3$ month. Most of bereaved were still suffering from bereavement grief within 1 year. Although most families didn't hesitate and felt positive mood to invitation, the rate of attendance was low. Comparing with two groups between attendant family and non-attendant, the latter felt more difficulty in 'financial problem/role difficulty, on the other hand, the former felt difficulty in 'depression'. Hereafter, the additional study about the factor relating to these attitude and needs of the bereaved relating to memorial service will be necessary.

• Korean Journal of Soil Science and Fertilizer
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• v.21 no.4
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• pp.386-408
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• 1988

A study was conducted to develop a model for estimating evapotranspiration and yield of Chinese cabbages from meteorological factors from 1981 to 1986 in Suweon, Korea. Lysimeters with water table maintained at 50cm depth were used to measure the potential evapotranspiration and the maximum evapotranspiration in situ. The actual evapotranspiration and the yield were measured in the field plots irrigated with different soil moisture regimes of -0.2, -0.5, and -1.0 bars, respectively. The soil water content throughout the profile was monitored by a neutron moisture depth gauge and the soil water potentials were measured using gypsum block and tensiometer. The fresh weight of Chinese cabbages at harvest was measured as yield. The data collected in situ were analyzed to obtain parameters related to modeling. The results were summarized as followings: 1. The 5-year mean of potential evapotranspiration (PET) gradually increased from 2.38 mm/day in early April to 3.98 mm/day in mid-June, and thereafter, decreased to 1.06 mm/day in mid-November. The estimated PET by Penman, Radiation or Blanney-Criddle methods were overestimated in comparison with the measured PET, while those by Pan-evaporation method were underestimated. The correlation between the estimated and the measured PET, however, showed high significance except for July and August by Blanney-Criddle method, which implied that the coefficients should be adjusted to the Korean conditions. 2. The meteorological factors which showed hgih correlation with the measured PET were temperature, vapour pressure deficit, sunshine hours, solar radiation and pan-evaporation. Several multiple regression equations using meteorological factors were formulated to estimate PET. The equation with pan-evaporation (Eo) was the simplest but highly accurate. PET = 0.712 + 0.705Eo 3. The crop coefficient of Chinese cabbages (Kc), the ratio of the maximum evapotranspiration (ETm) to PET, ranged from 0.5 to 0.7 at early growth stage and from 0.9 to 1.2 at mid and late growth stages. The regression equation with respect to the growth progress degree (G), ranging from 0.0 at transplanting day to 1.0 at the harvesting day, were: $$Kc=0.598+0.959G-0.501G^2$$ for spring cabbages $$Kc=0.402+1.887G-1.432G^2$$ for autumn cabbages 4. The soil factor (Kf), the ratio of the actual evapotranspiration to the maximum evapotranspiration, showed 1.0 when the available soil water fraction (f) was higher than a threshold value (fp) and decreased linearly with decreasing f below fp. The relationships were: Kf=1.0 for $$f{\geq}fp$$ Kf=a+bf for f$$I{\leq}Esm$$ Es = Esm for I > Esm 6. The model for estimating actual evapotranspiration (ETa) was based on the water balance neglecting capillary rise as: ETa=PET. Kc. Kf+Es 7. The model for estimating relative yield (Y/Ym) was selected among the regression equations with the measured ETa as: Y/Ym=a+bln(ETa) The coefficients and b were 0.07 and 0.73 for spring Chinese cabbages and 0.37 and 0.66 for autumn Chinese cabbages, respectively. 8. The estimated ETa and Y/Ym were compared with the measured values to verify the model established above. The estimated ETa showed disparities within 0.29mm/day for spring Chinese cabbages and 0.19mm/day for autumn Chinese cabbages. The average deviation of the estimated relative yield were 0.14 and 0.09, respectively. 9. The deviations between the estimated values by the model and the actual values obtained from three cropping field experiments after the completion of the model calibration were within reasonable confidence range. Therefore, this model was validated to be used in practical purpose.

• Clinical and Experimental Pediatrics
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• v.50 no.1
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• pp.33-39
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• 2007

Purpose : In spite of medical advances, empyema is a serious complication of pneumonia in children. Vaccination practices and antibiotic prescribing practices promote the change of clinical manifestations of empyema and causative organisms. So we made a nationwide clinical observation of 122 cases of empyema in children from 32 hospitals during the 5 year period from September 1999 to August 2004. Methods : Demographic data, and clinical information on the course and management of empyema patients were collected retrospectively from medical records in secondary and tertiary hospitals in Korea. Results : One hundred twenty two patients were enrolled from 35 hospitals. The most frequent age group was 1-3 years, accounting for 48 percent of all cases. The male to female sex ratio was 1.2:1. The main symptoms were cough, fever, respiratory difficulty, lethargy and chest pain in order of frequency. Hematologic findings on admission revealed decreased hemoglobin levels ($10.4{\pm}1.6g/dL$) and increased leukocyte counts ($16,234.3{\pm}10,601.8/{\mu}L$). Pleural fluid obtained from patients showed high leukocyte counts ($30,365.8{\pm}64,073.0/{\mu}L$), high protein levels ($522.3{\pm}1582.3g/dL$), and low glucose levels ($88.1{\pm}523.5mg/dL$). Findings from pleural fluid cultures were positive in 80 cases(65.6 percent). The most common causative agent was Streptococcus pneumoniae. The majority of patients were treated with antibiotics and closed drainage. Some patients needed open drainage (16.4 percent) or decortication (3.3 percent). The mean duration of hospitalization was $28.6{\pm}15.3days$. Conclusion : We analyzed childhood empyema patients during a period of 5 years in Korean children. The most frequent age group was 1-3 years and the most common causative agent was Streptococcus pneumoniaeiae. The majority of patients were treated with antibiotics and close drainage.

• Tuberculosis and Respiratory Diseases
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• v.44 no.1
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• pp.25-43
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• 1997

Introduction : The outcome and incidence of acute respiratory distress syndrome (ARDS) could be variable related to the varied definitions used for ARDS by researchers. The purpose of the national survey was to define the risk factors of ARDS and investigate the prognostic indicies related to mortality of ARDS in Korea according to the definition of ARDS determined by the American-European Concensus Conference on 1992 year. Methods : A Multicenter registry of 48 University or University-affliated hospital and 18 general hospital s equipped with more than 400 patient's beds conducted over 13 months of patients with acute respiratory distress syndrome using the same registry protocol. Results : 1. In the 12 months of the registry, 167 patients were enrolled at the 24 hospitals. 2. The mean age was 56.5 years (${\pm}17.2$ years) and there was a 1.9:1 ratio of males to females. 3. Sepsis was the most common risk factors (78.1%), followed by aspiration (16.6%), trauma (11.6%), and shock (8.5%). 4 The overall mortality rate was 71.9%. The mean duration was 11 days (${\pm}13.1$ days) from the diagnosis of ARDS to the death. Respiratory insufficiency appeared to be a major cause in 43.7% of the deaths followed by sepsis (36.1%), heart failure (7.6%) and hepatic failure (6.7%). 5. There were no significant differences in mortality based on sex or age. No significant difference in mortality in infectious versus noninfectious causes of ARDS was found. 6. There were significant differences in the pulse rate, platelet numbers, serum albumin and glucose levels, the amounts of 24 hour urine, arterial pH, $Pa0_2$, $PaCO_2$, $Sa0_2$, alveolar-arterial oxygen differences, $PaO_2/FIO_2$, and PEEP/$FI0_2$ between the survivors and the deaths on study days 1 through 6 of the first week after enrollment. 7. The survivors had significantly less organ failure and lower APACHE III scores at the time of diagnosis of ARDS (P<0.05). 8. The numbers of organ failure (odd ratio 1.95, 95% confidence intervals:1.05-3.61, P=0.03) and the score of APACHE III (odd ratio 1.59, 95% confidence interval:1.01-2.50, P=0.04) appeared to be independent risk factors of the mortality in the patients with ARDS. Conclusions : The mortality was 71.9% of total 167 patients in this investigation using the definition of American-European Consensus Conference on 1992 year, and the respiratory insufficiency was the leading cause of the death. In addition, the numbers of organ failure and the score of APACHE III at the time of diagnosis of ARDS appeared to be independent risk factors of the mortality in the patients with ARDS.

• Journal of The Korean Society of Grassland and Forage Science
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• v.6 no.2
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• pp.103-110
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• 1986

The experiments were carried out to study the influence of meteorological factors and cutting management on dry matter accumulation and net energy value in orchardgrass (Dactlylis glomerata L.) cv. Potomac and Baraula, perennial ryegrass (Lolium perenne L.) cv. Reveille and Semperweide and meadow fescue (Festuca pratensis Huds.) cv. Cosmos 11 and N.F.G.. The field trials were designed as a split plot design with three cutting regimes of 6-7 cuts at grazing stage, 4-5 cuts at silage stage and 3 cuts at hat stage in Korea and West Germany from 1975 to 1979. The results obtained are summarized as follows: 1. Productivity of orchardgrass, perennial ryegrass and meadow fescue were mainly affected by cutting systems and meteorological factors, especially air temperature, rainfalls, solar radiation and their interactions. In West Germany, cutting frequency was to be found asan most important factor influenced to dry matter yield and net energy value. 2. Orchardgrass, taken as average of all experimental sites in Korea, produced high yield of 875 kg/10 a in dry matter, which was as much as 32% and 27% higher than those of perennial ryegrass and meadow fescue, respectively. The annual dry matter yields of orchardgrass from 1976 to 1977 were shown a little variation. Dry matter yields in Freising and Braunschweig in West Germany were increased in all grass species continuously. 3. Orchardgrass, perennial ryegrass and meadow fescue showed different response to cutting frequency. The highest dry matter yields were found under 3 cuts at hay stage for orchardgrass and 4-5 cuts at silage stage for perennial ryegrass and meadow fescue. In West Germany, dry matter yields, as average of all grass species under different cutting systems, were 1326 kg, 1175 kg and 1098 kg/10a for 3 cuts, 4-5 cuts and 6-7 cuts, respectively. 4. Chemical composition and net energy concentration of temperate grasses were influenced by cutting managements. The highest yields of digestible crude protein were obtained under 6-7 cuts at grazing stage both in Korea and West Germany. In net energy yields, 3 cutting system produced the highest yield with 694 (orchardgrass), 665 (perennial ryegrass) an 623 kStE/10 a (meadow fescue). However, frequent cutting at grazing and silage stage produced higher yields than 3 cuts at hay stage in Cheju, Suweon and Taekwalyong.

• The korean journal of orthodontics
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• v.31 no.5 s.88
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• pp.525-534
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• 2001

Bone remodeling in response to force requires coordinated actions of osteoblasts, osteoclasts, osteocytes, and periodontal ligament cells. Coordination among these cells may be mediated, in part, by cell-to-cell communication via gap junctions. This study was designed to evaluate the expression of gap junction, connection 43 In periodontal tissue during the experimental movement of rat's incisors, by LSAB(labelled streptavidine biotin) immunohistochemical staining for connexin 43. Twenty seven Sprague-Dawley rats were divided into a control group(3 rats), and 6 experimental groups(24 rats) where 75g of force was applied from helical springs across the maxillary incisors. Rats of experimental groups were sacrificed at 12 hours, 1, 4, 7, 14 and 28 days after force application, respectively. And the tissues of a control group and experimental groups were studied immunohistochemically. The results were as follows : 1. In control group, the expression of connexin 43 was rare in gingiva, dentin, cementum, periodontal ligament, and bone cells. 2. In experimental group, the expression of connexin 43 was increased in pulp, periodontal ligament, osteoblasts, and osteoclasts, comparing to that in control. And it was rare in gingiva, dentin, and odontoblasts regardless of the duration of force application, which was not different from that of control group. 3. The expression of connexin 43 in pulp of experimental group began to increase in 4-day after force application and got to the highest degree at 7-day. And it decreased after 14-day to be similar to that of control group at 28-day. 4. The expression of connexin 43 in periodontal ligament was noted in small capillaries adjacent to alveolar bone, showing higher intensity of immunolabelling after 4-day And it was stronger in the pressure side than in tension side of periodontal ligament. After 7-day, decrease in connexin 43 expression was observed. 5. The expression of connexin 43 in alveolar bone began to increase 1-day, reached to the highest degree at 4-day, and decreased at 7-day. And the expression in osteoclasts was more than that in osteoblasts or osteocyte at 7-day.

• KOREAN JOURNAL OF CROP SCIENCE
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• v.27 no.4
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• pp.371-384
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• 1982

Highly cold tolerant varieties are requested not only at high latitute cool area but also tropical high elevated areas, and the required tolerance is different from location to location. IRRI identified 6 different types of cold tolerance required in the world for breeding purpose; a) Hokkaido type, b) Suweon type, c) Taipei 1st season type, d) Taipei 2nd season type, e) Tropical alpine type and, f) Bangladesh type. The cold tolerance requested in Korea is more eargent in Tongil group cultivars and their required tolerance is the one such as the physiological activities at low temperature are as active as in Japonica group cultivars at least during young seedling stage and reproduction stage. With conventional Japonica cultivars, such cold tolerant characters are requested as short growth duration but stable basic vegetative growth, less sensitive to high temperature and less prolonged growth duration at low temperature. The methods screening for cold tolerance were developed rapidly after the Tongil cultivar was reliesed. The facilities of screening for cold tolerance, such as, low temperature incubator, cold water tank, growth cabinet, phytotron, cold water nursery in Chuncheon, breeding nursery located in Jinbu, Unbong and Youngduk, are well established. Foreign facilities such as, cold water tank with the rapid generation advancement facilities, cold nurseries located in Banaue, Kathmandu and Kashimir may be available for the screening of some limitted breeding materials. For the reference, screening methods applied at different growth stages in Japan are introduced. The component characters of cold tolerance are not well identified, but the varietal differences in a) germinability, b) young seedling growth, c) rooting, d) tillering, e) discolation, f) nutrition uptake, g) photosynthesis rate, h) delay in heading, i) pollen sterility, and j) grain fertility at low temperature are reported to be distinguishable. Relationships among those traits are not consistent. Reported studies on the inheritance of cold tolerance are summarized. Four or more genes are controlling low temperature germinability, one or several genes are controlling seedling tolerance, and four or more genes are responsible for the pollen fertility of the rice treated with cold air or grown in the cold water nursery. But most of those data indicate that the results may come out in different way if those were tested at different temperature. Many cold tolerant parents among Japonicas, Indicas and Javanicas were identified as the results of the improvement of cold tolerance screening techniques and IRTP efforts and they are ready to be utilized. Considering a) diversification of germ plasm, b) integration of resistances to diseases and insects, c) identification of adaptability of recommending cultivars and, d) systematic control of recommending cultivars, breeding strategies for short term and long term are suggested. For short term, efforts will be concentrated mainly to the conventional cultivar group. Domestic cultivars will be used as foundation stock and ecologically different foreign introductions such as from Hokkaido, China or from Taiwan, will be used as cross parents for the adjustment of growth durations and synthsize the prototype of tolerances. While at the other side, extreme early waxy Japonicas will be crossed with the Indica parents which are identified for their resistances to the diseases and insects. Through the back corsses to waxy Japonicas, those Indica resistances will be transfered to the Japonicas and these will be utilized to the crosses for the improvement of resistances of prototype. For the long term, efforts will be payed to synthsize all the available tolerances identified any from Japonicas, Indicas and Javanicas to diversify the germ plasm. The tolerant cultivars newly synthsized, should be stable and affected minimum. to the low temperature at all the growing stages. The resistances to the diseases and insects should be integrated also. The rapid generation advancement, pollen culture and international cooperations were emphasized to maximize the breeding efficiency.

• 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.

• Applied Biological Chemistry
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• v.4
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• pp.1-9
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• 1963

For the studies on the Chemical compositions of Sweet potatoes grown in Korean soil, Suwon No. 118 and Suwon No. 147 were applied as the samples during growing period, and 15 varieties of swee tpotatoes as the samples or comparisons among them. As the results of the studies followings were obtained. Changes of the chemical compositions of root tuber during growing period. 1. Total weights of root tubers and mean weight per root tuber were increased gradually as grew with the values of Suwon No. 147 is higher than that of Suwon No. 18 except the weight of Suwon No. 147 on September 9. 2. The moisture content of the roots were fairy uniform. 3. While the starch contents and crude starch yield in the root tuber were gradually increased with almost parallel as grew except the values of Suwon No. 147 on September 7. were markedly higher. 4. The total weight of the Sweet potatoes per Dan-Bo (about 0.25 acre) showed increased values with Suwon No. 147 is higher than Suwon No. 118 except the unexpected lower weight of Suwon No. 147 on September 9. and the crude starch yield of Suwon No. 147 per Dan-Bo also showed higher values than that of Suwon No. 18 with almost parallel increase of them as they grew. 5. Reducing sugar contents of them showed gradual decreases at earlier stages then increases at latter stages as grew, and total sugar and sucrose of them also showed gradual increases except extremly higher contents of Suwon No. 147 and lower values of Suwon No. 118 on September 9. 6. Total protein and soluble protein contents of them showed that initial and last stages of the growth are in higher values but middle stages are fairy low values with a little changes of difference between total protein, and soluble protein. The comparisons among those varieties. 7. The moisture contents of root tuber varies from 63% to 72% among them. 8. The starch contents of Suwon No. 18 (23.9%) is highest value among them, Ko-Ke No. 14 and Won-Ki successively lower and Dae-Nong No. 45 is the lowest one. Crude starch yield (%) of Ko-Ke No. 14 and Won-Ki successively lower and Dae-Nong No.45 is the lowest one. Crude starch yield (%) of Ko-Ke No. 147 is highest value, Suwon No. 118, Won-Ki are successively lower and Do-Ip is the lowest one. 9. Won-Ki is highest value in reducing sugar content, and Do-Ip No. 2 is lowest one in it. The sucrose content of Chil-Bok is highest and Won-Ki is lowest among them. Soluble total sugar content of Yu-Sim is in highest and Chun-Mi is in lowest value. 10. Total protein content showed that Suwon No. 147 is in highest value and Yu-Sim is in lowest one. On the other hand, soluble protein contents showed that Chil-Bok is in highest value and Yu-Sim is in lowest one.

• 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)