• Title/Summary/Keyword: 경인

Search Result 34,792, Processing Time 0.074 seconds

Studies on the Changes of N-Compounds during the Fermentation Process of the Korean Daenjang (한국된장의 발효과정(醱酵過程)에 따른 N-Compounds의 소장(消長)에 관(關)한 연구(硏究))

  • Yoon, Il-Sup;Kim, Hyun-Oh;Youn, Se-Eok;Lee, Kap-Sang
    • Korean Journal of Food Science and Technology
    • /
    • v.9 no.2
    • /
    • pp.131-137
    • /
    • 1977
  • This study was conducted to examine the changes of pH, proteolytic enzymic activity, and every kind of nitrogen compounds during their fermentation of the three groups of meju for 90days. Among the three groups, the first group was conventional Korean meju which was proved to be good quality (sample J), the second group was prepared with soybean paste using B. subtilis (sample K), and the third group was an improved meju which was fermented with the soybean and wheat (7 : 3) mixtured paste with Asp. sojae (sample L). These groups were analyzed at an interval of 10 day and the results are summarized as follows: 1) The pH of the all three groups was lowered from $6.45{\sim}6.75\;to\;4.85{\sim}5.20$ in just the 30 days and maintained the weak acidity during this fermentation. 2) The proteolytic enzymic activity was increased as soon as the three groups of meju were fermented and marked the maximum value in 30 days. The maximum value of the three groups of meju J.K. and L was 147, 112, and 52 respectively. The proteolytic enzymic activity of sample J and K was decreased to 23.5 and 20.5 in 20 days, while that of sample L was decreased to 18.0 in 40 days, and maintained the volues to the end of fermentation for 90 days. The conventional meju J and the improved meju K showed sparkling activity at the pH 7, while the activity of improved meju L was strong at the pH 10. 3) The PAA-N content of sample J and K was increased and reached to the peak point with 1.55% and 1.49% respectively in 60 days. But the sample L marked the maximum value with 1.28% after 80 days. 4) The amino-N content of sample J was increased and reached to 0.36% after 60 days, and that of sample K and L was increased and reached to 0.29% and 0.21% respectively after 40 days. After reaching to the peak point, the contents were decreased. 5) The content of ammonia-N was most abundant in sample K which was fermented with soybean paste using B. subtilis. 6) The peptide-N content of sample K and L was increased after decreasing in the middle of fermentation period, while that of sample J was increased gradually during fermentation. 7) The changes of nitrogen compounds were seemed to complete in 60 days of fermentation.

  • PDF

A Study on the Extraction Rate of Brain Tissues from a $^{99m}Tc$-HMPAO Cerebral Blood flow SPECT Examination of a Patient ($^{99m}Tc$-HMPAO 뇌혈류 SPECT 검사 시 환자에 따른 뇌조직 추출률에 대한 고찰)

  • Kim, Hwa-San;Lee, Dong-Ho;Ahn, Byeong-Pil;Kim, Hyun-Ki;Jung, Jin-Yung;Lee, Hyung-Nam;Kim, Jung-Ho
    • The Korean Journal of Nuclear Medicine Technology
    • /
    • v.16 no.1
    • /
    • pp.17-26
    • /
    • 2012
  • Purpose: This study mainly focuses on the patients treated with chemically stable radiopharmaceutical product $^{99m}Tc$-HMPAO (d,l-hexamethylpropylene amine oxime) which yielded reduced image quality due to a decreased brain extraction rate. $^{99m}Tc$-HMPAO will be examined further to determine whether this product may be accounted as a factor for this cause. Material and Methods: From January 2010 until December 2010, out of 272 patients who were all subjected to $^{99m}Tc$-HMPAO brain blood flow SPECT scans resulting from Cerebral Infarction; 23 patients(ages $55.3{\pm}9$, 21 males, 3 females) with decreased tissue extraction rate were examined in detail. The radiopharmaceutical product $^{99m}Tc$-HMPAO was used on patients with normal brain tissue exchange rate as well as those with reduced rate in order to prove its' chemical stability. The patients' age, sex, blood pressure, existence of diabetes, drug use, current health status, known side effects from CT/MRI, examination of the patients' past SPECT before/after images were accounted to determine the factors and correlations affecting the rate of blood tissue extractions. Result: After multiple linear regression analysis, there were no unusual correlations between the 6 factors excluding sex, and before/after examination images. Male subjects showed reduced brain tissue extraction rate than the females ($p$ > 0.05) 91.3% male, 8.7% female. Wilcoxon Matched-Pairs Signed-Ranks Test was used on the before/after images which yielded a value of 0.06, which did not indicate a significant amount of difference on the 2 tests ($p$ > 0.05). As a result, the before/after images indicated similar brain tissue extraction rates, and there were variations depending on the individual patient. Conclusion: The effects of the chemically stable radiopharmaceutical product $^{99m}Tc$-HMPAO depended on the patient's personal characteristics and status, therefore was considered to be a factor in reducing brain tissue extraction rate. The related articles of $^{99m}Tc$-HMPAO cerebral blood flow SPECT speculates a cerebrovascular disease and factors resulting from portal veins, and it was not possible to pin point the exact cause of decreasing brain tissue extraction rate. However, the $^{99m}Tc$-HMPAO cerebral blood flow SPECT scan proved to be extremely useful in tracking and inspecting brain diseases, as well as offering accurate results from patients suffering from reduced brain tissue extraction rates.

  • PDF

Risk Factor Analysis and Surgical Indications for Pulmonary Artery Banding (폐동맥 밴딩의 위험인자 분석과 수술적응중)

  • Lee Jeong Ryul;Choi Chang Hyu;Min Sun Kyung;Kim Woong Han;Kim Yong Jin;Rho Joon Ryang;Bae Eun Jung;Noh Chung I1;Yun Yong Soo
    • Journal of Chest Surgery
    • /
    • v.38 no.8 s.253
    • /
    • pp.538-544
    • /
    • 2005
  • Background: Pulmonary artery banding (PAB) is an initial palliative procedure for a diverse group of patients with congenital cardiac anomalies and unrestricted pulmonary blood flow. We proved the usefulness of PAB through retrospective investigation of the surgical indication and risk analysis retrospectively. Material and Method: One hundred and fifty four consecutive patients (99 males and 55 females) who underwent PAB between January 1986 and December 2003 were included. We analysed the risk factors for early mortality and actuarial survival rate. Mean age was $2.5\pm12.8\;(0.2\sim92.7)$ months and mean weight was $4.5\pm2.7\;(0.9\sim18.0)\;kg$. Preoperative diagnosis included functional single ventricle $(88,\;57.1\%)$, double outlet right ventricle $(22,\;14.2\%)$, transposition of the great arteries $(26,\;16.8\%)$, and atrioventricular septal defect $(11,\;7.1\%)$. Coarctation of the aorta or interrupted aortic arch $(32,\;20.7\%)$, subaortic stenosis $(13,\;8.4\%)$ and total anomalous pulmonary venous connection $(13,\;8.4\%)$ were associated. Result: The overall early mortality was $22.1\%\;(34\;of\;154)$, The recent series from 1996 include patients with lower age $(3.8\pm15.9\;vs.\;1.5\pm12.7,\;p=0.04)$ and lower body weight $(4.8\pm3.1\;vs.\;4.0\pm2.7,\;p=0.02)$. The early mortality was lower in the recent group $(17.5\%;\;16/75)$ than the earlier group $(28.5\%;\;18/45)$. Aortic arch anomaly (p=0.004), subaortic stenosis (p=0.004), operation for subaortic stenosis (p=0.007), and cardiopulmonary bypass (p=0.007) were proven to be risk factors for early death in univariate analysis, while time of surgery (<1996) (p=0.026) was the only significant risk factor in multivariate analysis. The mean time interval from PAB to the second-stage operation was $12.8\pm10.9$ months. Among 96 patients who survived PAB, 40 patients completed Fontan operation, 21 patients underwent bidirectional cavopulmonary shunt, and 35 patients underwent biventricular repair including 25 arterial switch operations. Median follow-up was $40.1\pm48.9$ months. Overall survival rates at 1 year, 5 years and 10 years were $81.2\%\;65.0\%,\;and\;63.5\%$ respectively. Conclusion: Although it improved in recent series, early mortality was still high despite the advances in perioperative management. As for conventional indications, early primary repair may be more beneficial. However, PA banding still has a role in the initial palliative step in selective groups.

Modified Thoracoscopic Bullectomy (흉강경하 기낭절제술의 변형 술식)

  • Park, Chan-Beom;Cho, Min-Seob;Sa, Young-Jo;Lee, Jong-Ho;Jin, Ung;Kwon, Jong-Bum;Cho, Deog-Gon;Park, Kuhn;Cho, Kyu-Do;Kim, Chi-Kyeong
    • Journal of Chest Surgery
    • /
    • v.36 no.12
    • /
    • pp.937-942
    • /
    • 2003
  • Morbidity, the use of analgesics, the amount of postoperative drainage and the postoperative hospital stay were reduced in VATS for pneumothorax. However, some authors preferred minithoracotomy to VATS because the rate of recurrence after VATS were between 5% and 10%. Therefore, we present a modified thoracoscopic bullectomy (MTB) which we believe has the advantages of conventional VATS and minithoracotomy. Material and Method: Sixty-six patients who received the operation from January 2002 to December 2002 were divided into 3 groups. Twenty-six patients were treated by axillary minithoracotomy and thirteen by conventional VATS and 18 by modified thoracoscopic bullectomy, The mean age was 21.9 years (range, 16∼35 years) for minithoracotomy group, 20.6 years (range, 17∼28 years) for conventional VATS group and 22.6 years (range, 16∼39 years) for MTB group. The mean follow-ups were 11.4 months for minithoracotomy group, 9.5 months for conventional VATS group and 4.7 months for MTB group. Result: The mean duration of operation was 55.79$\pm$23.35 minutes in MTB and 44.23$\pm$19.24 minutes in conventional VATS (p=0.333). The number of staplers being used was 1.63 $\pm$0.76 in MTB, 1.41$\pm$0.64 in minithoracotomy (p=0.663), and 2.92$\pm$1.19 in conventional VATS (p<0.001). The duration of indwelling chest tube was 1.63$\pm$0.76 day in MTB, 4.07$\pm$ 1.41 day in minithoracotomy (p<0.001) and 4.46$\pm$2.33day in conventional VATS (p<0.001). Hospital length of stay was 3.26$\pm$0.81 day in MTB, 6.04$\pm$2.21 day in minithoracotomy (p<0.001) and 6.69$\pm$3.33 day in conventional VATS (p<0.001). The number of postoperative complication and recurrence were 2 in minithoracotomy (7.4%), 5 in conventional VATS (38.5%) and 1 in MTB (5.6%). Conclusion: Modified thoracoscopic bullectomy is an effective procedure in the treatment of spontaneous pneumothorax.

Studies on the Chemical Compositions of Korean Sweet Potatoes ('한국산(韓國産) 고구마의 화학적(化學的) 조성(組成)에 관(關)한 연구(硏究)')

  • Kim, Ho-Sik;Lee, Chun-Yeong;Kim, Jae-Uk
    • Applied Biological Chemistry
    • /
    • v.4
    • /
    • pp.1-9
    • /
    • 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.

  • PDF

Steroid Effect on the Brain Protection During OPen Heart Surgery Using Hypothermic Circulatory Arrest in the Rabbit Cardiopulmonary bypass Model (저체온순환정지법을 이용한 개심술시 스테로이드의 뇌보호 효과 - 토끼를 이용한 심폐바이패스 실험모델에서 -)

  • Kim, Won-Gon;Lim, Cheong;Moon, Hyun-Jong;Chun, Eui-Kyung;Chi, Je-Geun;Won, Tae-Hee;Lee, Young-Tak;Chee, Hyun-Keun;Kim, Jun-Woo
    • Journal of Chest Surgery
    • /
    • v.30 no.5
    • /
    • pp.471-478
    • /
    • 1997
  • Introduction: The use of rabbits as a cardiopulmonary bypass(CPB) animal model is extremely dif%cult mainly due to technical problems. On the other hand, deep hypothermic circulatory arrest(CA) is used to facilitate surgical repair in a variety of cardiac diseases. Although steroids are generally known to be effective in the treatment of cerebral edema, the protective effects of steroids on the brain during CA are not conclusively established. Objectives of this study are twofold: the establishment of CPB technique in rabbits and the evaluation of preventive effect of steroid on the development of brain edema during CA. Material '||'&'||' Methods: Fifteen New Zealan white rabbits(average body weight 3.5kg) were divided into three experimental groups; control CA group(n=5), CA with Trendelenberg position group(n=5), and CA with Trendelenberg position + steroid(methylprednisolone 30 mglkg) administration group(n=5). After anesthetic induction and tracheostomy, a median sternotomy was performed. An aortic cannula(3.3mm) and a venous ncannula(14 Fr) were inserted, respectively in the ascending aorta and the right atrium. The CPB circuit consisted of a roller pump and a bubble oxygenator. Priming volume of the circuit was approximately 450m1 with 120" 150ml of blood. CPB was initiated at a flow rate of 80~85ml/kg/min, Ten min after the start of CPB, CA was established with duration of 40min at $20^{\circ}C$ of rectal temperature. After CA, CPB was restarted with 20min period of rewarming. Ten min after weaning, the animal was sacrif;cod. One-to-2g portions of the following tissues were rapidly d:ssected and water contents were examined and compared among gr ups: brain, cervical spinal cord, kidney, duodenum, lung, heart, liver, spleen, pancreas. stomach. Statistical significances were analyzed by Kruskal-Wallis nonparametric test. Results: CPB with CA was successfully performed in all cases. Flow rate of 60-100 mlfkgfmin was able to be maintained throughout CPB. During CPB, no significant metabolic acidosis was detected and aortic pressure ranged between 35-55 mmHg. After weaning from CPB, all hearts resumed normal beating spontaneously. There were no statistically significant differences in the water contents of tissues including brain among the three experimental groups. Conclusion: These results indicate (1) CPB can be reliably administered in rabbits if proper technique is used, (2) the effect of steroid on the protection of brain edema related to Trendelenburg position during CA is not established within the scope of this experiment.

  • PDF

The Cox-Maze Procedure for Atrial Fibrillation Concomitant with Mitral Valve Disease (승모판막질환에 동반된 심방세동에서 Cox-Maze 술식)

  • Kim, Ki-Bong;Cho, Kwang-Ree;Ahn, Hyuk
    • Journal of Chest Surgery
    • /
    • v.31 no.10
    • /
    • pp.939-944
    • /
    • 1998
  • Background: The sugical results of the Cox-Maze procedure (CMP) for lone atrial fibrillation(AF) have proven to be exellent. However, those for AF associated with mitral valve(MV) disease have been reported to be a little inferior. Materials and methods: To assess the efficacy and safety of the CMP as a combined procedure with MV operation, we studied retrospectively our experiences. Between April 1994 and October 1997, we experienced 70 (23 males, 47 females) cases of CMP concomitantly with MV operation. Results: The etiologies of MV disease were rheumatic in 67 and degenerative in 3 cases. The mean duration of AF before sugery was 66$\pm$70 months. Fifteen patients had the past medical history of thromboembolic complications, and left atrial thrombi were identified at operation in 24 patients. Twelve cases were reoperations. Aortic cross clamp (ACC) time was mean 151$\pm$44 minutes, and cardiopulmonary bypass (CPB) time was mean 246$\pm$65 minutes. Concomitant procedures were mitral valve replacement (MVR) in 19, MVR and aortic valve replacement (AVR) in 14, MVR and tricupid annuloplasty (TAP) in 8, MVR with AV repair in 3, MV repair in 11, MVR and coronary artery bypass grafting (CABG) in 2, MVR and AVR and CABG in 1, redo-MVR in 10, redo-MVR and redo-AVR in 2 patients. The rate of hospital mortality was 1.4%(1/70). Perioperative recurrence of AF was seen in 44(62.9%), and atrial tachyarrhythmias in 10(14.3%), low cardiac output syndrome in 4(5.7%), postoperative bleeding that required mediastinal exploration in 4(5.7%) patients. Other complications were acute renal failure in 2, aggravation of preoperative hemiplegia in 1, and transient delirium in 1 patient. We followed up all the survivors for 16.4 months(3-44months) on an average. Sinus rhythm has been restored in 65(94.2%) patients. AF has been controlled by operation alone in 73.9% and operation plus medication in 20.3%. Two patients needed permanent pacemaker implantation; one with sick sinus syndrome, and the other with tachycardia- bradycardia syndrome. Only two patients remained in AF. We followed up our patients with transthoracic echocardiography to assess the atrial contractilities and other cardiac functions. Right atrial contractility could be demonstrated in 92% and left atrial contractility in 53%.We compared our non-redo cases with redo cases. Although the duration of AF was significantly longer in redo cases, there was no differences in ACC time, CPB time, postoperative bleeding amount and sinus conversion rate. Conclusions: In conclusion, the CMP concomitant with MV operation demonstrated a high sinus conversion rate under the acceptable operative risk even in case of reoperation.

  • PDF

6-Month Short-Course Chemotherapy for Tuberculous Pleural Effusion (결핵성 흉막염에서의 6개월 단기 치료)

  • Kim, Tae-Hyung;Kim, Kyoung-A;Lim, Chae-Man;Lee, Sang-Do;Koh, Youn-Suck;Kim, Woo-Sung;Kim, Dong-Soon;Kim, Won-Dong
    • Tuberculosis and Respiratory Diseases
    • /
    • v.45 no.3
    • /
    • pp.509-518
    • /
    • 1998
  • Background: Short-course chemotherapy for 6 months is well established for pulmonary tuberculosis. However, little is known about the efficacy of the short-course chemotherapy for tuberculous pleural effusion. Tuberculous pleural effusion itself may be self-limiting without any treatment, but about two thirds of the patients with tuberculous pleural effusion may subsequently develop pulmonary tuberculosis within 5 years. After completing treatment for tuberculous pleural effusion. prolonged follow-up is necessary for evaluating the efficacy of the treatment There is still no report on the efficacy of 6-month regimens for tuberculous pleural effusion in Korea, where the incidence of tuberculous disease and drug resistance is high. We studied the efficacy of 6 month short-course chemotherapy comparing with 9 month chemotherapy. Method : Retrospective study was done through medical record review in 238 patients with tuberculous pleural effusion who admitted to Asan Medical Center during May 1989-May 1993. The diagnosis of tuberculous pleural effusion was made by bacteriologic or histopathologic study. Results: Among 238 patients, 38 patients were dropped out during follow-up period. In 2 patients, second line drugs were prescribed according to known drug resistance results. And, in 23 patients, treatment longer than 9 months was done due to accompanying extrapulmonary tuberculosis or durg resistance. In 8 patients, treatment regimen was changed due to hepatotoxicity. Remaining 167 cases (70.2%) completed the treatment as scheduled ; 6 month chemotherapy in 88 cases and 9 month chemotherapy in 79 cases. In 60 patients (35.9%) with pleural effusion only in chest X-ray finding, sputum smear or culture for M.tuberculosis was positive in 6 cases (10.0%), and in 63 patients (37.7%) with radiologically inactive pulmonary tuberculosis, sputum smear or culture was positive in 18 cases (28.6%). In 44 patients (26.3%) with radiologically active pulmonary tuberculosis, the sputum smear or culture was positive in 24 cases (54.5%). In 6-month chemotherapy group (n=88), during mean 23 months (range; 1~61months) follow-up period, pulmonary tuberculosis developed in 1 case (1.4%). In 9-month chemotherapy group(n=79), during mean 23 months (range; 3~70months) follow-up period, pulmonary tuberculosis developed in 2 cases (2.5%). All the cases who developed pulmonary tuberculosis also showed active pulmonary tuberculosis on initial chest X-ray before treatment Conclusion: In patients with tuberculous pleural effusion, the incidence of pulmonary tuberculosis after 6 month chemotherapy showed no difference from that after 9 month chemotherapy. Thus, 6 month short-course chemotherapy seems to be an effective treatment for tuberculous pleural effusion.

  • PDF

Microbiological Evaluation of Foods and Kitchen Environments in Childcare Center and Kindergarten Foodservice Operations (보육시설과 유치원 급식의 식품 및 환경 미생물의 오염도 평가)

  • Seol, Hye-Rin;Park, Hyoung-Su;Park, Ki-Hwan;Park, Ae-Kyung;Ryu, Kyung
    • Journal of the Korean Society of Food Science and Nutrition
    • /
    • v.38 no.2
    • /
    • pp.252-260
    • /
    • 2009
  • Whereas the numbers of childcare centers and kindergartens are increasing rapidly, systematic management to control the food safety of foodservice operation is not yet well established. Samples from 12 centers in Seoul and Gyeonggi Province were collected to assess the microbiological quality of 32 raw materials, 24 cooked foods, 76 food-contact surfaces (knives, cutting boards, dish towels and gloves), 17 employees' hands and 12 air-borne bacteria. The microbiological analyses were performed for aerobic plate counts (APC), Enterobacteriaceae, E. coli and 7 pathogens (B. cereus, C. jejuni, C. perfringens, L. monocytogenes, Salmonella spp., S. aureus, and V. parahaemolyticus). Among raw materials, E. coli ($1.39{\sim}2.08\;\log\;CFU/g$) were detected in 4 out of 6 meats and 7.46 log CFU/g of APC in tofu. High enterobacteriaceae levels of 4.23, 5.14 and 4.19 log CFU/g were found in cucumber salad, steamed spinach with seasonings and steamed bean sprout with seasonings, respectively. No pathogens were found in all samples except for C. perfringens detected from raw spinach and raw lotus root. Only APC and enterobacteriaceae were found in food-contact surfaces. Two of the 23 knives and three of the 24 kitchen boards showed over 500 CFU/$100\;cm^2$ of APC; also, APC levels (5.03 to 5.44 log CFU/g) were detected in 4 of the 12 dish towels. Only one glove showed Enterobacteriaceae (2.44 log CFU/glove) contamination. Enterobacteriaceae were found in 2 employees' hands ($2.37{\sim}4.44\;\log\;CFU$/hand) among the 16 employees. The contamination levels of air-borne bacteria were shown unacceptable in two (2.25 and 2.30 log CFU/petri-film/15 min) out of the 12 kitchen areas. These results suggest that the microbiological hazards in some foods and environments are not well controlled and thus a guideline should be provided to ensure the food safety in childcare center and kindergarten foodservice operations.

Clinical Characteristics of Arthrogryposis, Renal Tubular Dysfunction, Cholestasis(ARC) Syndrome in Korea (ARC(Arthrogryposis, Renal Tubular Dysfunction, Cholestasis) 증후군의 발병양상에 관한 연구)

  • Lee Soon Min;Kim Ji Hong;Lee Jae Seung;Han Suk Joo
    • Childhood Kidney Diseases
    • /
    • v.9 no.2
    • /
    • pp.222-230
    • /
    • 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)

  • PDF