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The relation between angiotensin converting enzyme (ACE) gene polymorphism and neonatal hyperbilirubinemia in Korea (한국인 신생아 황달과 안지오텐신 전환효소 유전자의 다형성)

  • Kim, Mi Yeoun;Lee, Jae Myoung;Kim, Ji Sook;Kim, Eun Ryoung;Lee, Hee Jae;Yoon, Seo Hyun;Chung, Joo Ho
    • Clinical and Experimental Pediatrics
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    • v.50 no.1
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    • pp.28-32
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    • 2007
  • Purpose : Human angiotensin converting enzyme (ACE) gene shows an insertion/deletion polymorphism in 16 intron, and three genotypes are determined by whether a 287 bp fragment of the DNA is present or not; II, ID and DD genotype. DD genotype has been suggested as a risk factor of chronic nephrotic disease such as IgA nephropathy and diabetic nephropathy, various cardiovascular diseases and several other diseases. ACE activity increases in acute hepatitis, chronic persistent hepatitis, chronic active hepatitis and cirrhosis. On the other hand, patients with fatty livers have normal ACE activity. This study was designed to find out the relation between polymorphsims of the ACE genes and neonatal hyperbilirubinemia in Koreans. Methods : The genomic DNA was isolated from 110 full-term Korean neonates who had hyperbilirubinemia with no obvious causes (serum bilirubin$${\geq_-}12mg/dL$$) and 164 neonates of a control population (serum bilirubin <12 mg/dL). We performed polymerase chain reaction (PCR) to see the allele of the ACE gene. Electrophoresis was done in the PCR products in 1.5 percent agarose gel, and then DNA patterns were directly visualized under ethidium bromide staining. Results : ACE genotypes in the hyperbilirubinemia group are as follows; 26.36 percent for II, 53.64 percent for ID, 20.00 percent for DD, 0.532 for I allele and 0.468 for D allele. These distributions were not significantly different from those in the control group; 24.39 percent for II, 51.83 percent for DI, 23.78 percent for DD, 0.503 for I allele and 0.497 for D allele. Conclusion : In this study, ACE gene polymorphism was detected in the neonatal hyperbilirubinemia and control group. The most frequent genotype was ID. Our results indicate that the ACE gene polymorphism is not associated with the prevalence of neonatal hyperbilirubinemia in Koreans.

Etiological Studies on the Acute Fatal Disease of Angora Rabbits : The So-Called Rabbit Viral Sudden Death (앙고라 토끼의 급성폐사성질병(急性斃死性疾病)의 병인학적(病因學的) 연구(硏究) : 소위(所謂) 토끼의 바이러스성(性) 급사병(急死病))

  • Lee, Cha-soo;Park, Cheong-kyu
    • Korean Journal of Veterinary Research
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    • v.27 no.2
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    • pp.277-290
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    • 1987
  • This paper dealt with etiological studies on the acute fatal disease of Angora rabbits occurring as a group in Korea. The disease was confirmed as an acute infectious disease caused by virus. The results obtained were summarized as follows: The disease produced a high morbidity in the rearing Angora rabbits and a high mortality in the infected rabbits, and was acute. The infected rabbits died soon without premonitory signs after inappetence. The body temperature of the affected rabbits rose to $40^{\circ}C$ and nearly all deaths occurred within 48 hours after inoculation. In many cases a bloody foam was visible from the nostrils after death. According to the progress of the disease the nervous signs, such as ataxia, paralysis of the legs, and torticollis could be recognized in the some cases. Rabbits that had recovered from the disease were severe emaciation, and bristly and sparse hairs. In macroscopical findings, there were hemorrhage and edema of the lung, hemorrhage or hyperemia of the tracheal and broncheal mucosae, appearance of blood-tinged effusion in the respiratory tract. The principal lesions were found in the liver. Usually the lobular necrosis of the liver cells was progressed, and focal necrosis and hemorrhagic spots of various sizes were often observed in the liver. Liver was as a whole pale. In chronic cases, however, there was a slight liver cirrhosis with the atrophy of the parenchymal cells. The other lesions encountered grossly consisted of swelling and petechiae of the kidney, hyperemia and hemorrhage of the spleen, catarrh of the small intestine, and hyperemia of the brain. The urinary bladder contained a lot of turbid urine or bloody urine and urinary cast, and was distended with the urine. In microscopical findings, the most striking lesions occurred in the liver and may be classified as viral hepatitis. The hepatic lesions were initially characterized by progression from periportal to peripheral necrosis of the lobules with the infiltration of mononuclear cells. Focal necrosis of various sizes, hemorrhage and hyperemia were often observed in the hepatic lobules. In chronic cases, there were intensive infiltration of lymphocytes, proliferation of fibroblasts, appearance of plasmal cells, and atrophy of parenchymal cells in the hepatic tissue. Perivascular lymphocytic infiltration and meningitis were seen in the brain and spinal cord. In the kidney, there were acute glomerulonephritis, hemorrhage, necrosis of the uriniferous tubules, and retention of eosinophilic substance within the renal tubules. Proliferation of fibroblasts and infiltration of mono-nuclear cells were found in the interstitial stroma of the kidney in chronic case. There were also hemorrhage and edema in the lung, hyperemia and hemorrhage in the trachea and bronchus, perivascular lymphocytic infiltration and focal myocardial necrosis in the heart, hyperemia and hemorrhage in the spleen, vacuolization and desquamation of mucous epithelia in the urinary bladder, catarrhal inflammation of the small intestine, hemorrhage in the adrenal cortex and hyperemia in the other organs. In the electron microscopical findings of the hepatic tissue, crystals of viral particles appeared in the cytoplasm of the hepatocytes and the sinusoidal endothelial cells, and the viral particles, were small in size and polygonal. The authors suppose the virus may belong to picornaviridae family of RNA viruses. Also immature virus-like particles, dilated rough endoplasmic reticulum and destruction of nuclear membrane were seen in the hepatocytes. From these results, it is concluded that the sudden death is an acute viral disease characterized by hepatitis and the affected rabbits may be died of viremia.

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Clinical Characteristics, Histology and Prognosis of Autoimmune Hepatitis in Korean Children (소아 자가면역성 간염: 임상적 특성, 조직 소견 및 예후)

  • Chung, Dae-Lim;Seo, Jeong Kee;Yang, Hye Ran;Ko, Jae Sung;Park, Sung Hye
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.7 no.2
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    • pp.186-196
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    • 2004
  • Purpose: Autoimmune hepatitis is a chronic inflammatory liver disease with unknown cause that is characterized by liver histology, circulating autoantibodies and increased levels of immunoglobulin G. Only sporadic reports are available on autoimmune hepatitis in children. The aim of this study was to evaluate the clinical, biochemical, and histological features, and the long-term outcome of autoimmune hepatitis in Korean children. Methods: We reviewed the medical records of 14 children diagnosed as having autoimmune hepatitis at Seoul National University Children's Hospital from 1990 to 2004, and analyzed clinical, biochemical, and histological features, and clinical outcomes. Results: Mean age at diagnosis was 9 years and 11 of the 14 children were female. Six children presented with acute hepatitis-like manifestations. Jaundice and fatigue were the most common symptoms. Other autoimmune diseases accompanied in 6 children. Anti-nuclear antibody was detected in 13 patients and anti-smooth muscle antibody was positive in 8. All 14 patients were type 1 autoimmune hepatitis. The main histologic findings were interface hepatitis, rosette formation, and cirrhosis. Clinical and biochemical features were improved in six patients treated with ursodeoxycholic acid. Eight patients were treated with corticosteroid alone or in combination with azathioprine and five of them are in biochemical remission. Conclusion: Autoimmune hepatitis is an inflammatory liver disease, which has a favorable long-term outcome if it is diagnosed and treated promptly. Therefore, autoimmune hepatitis should be suspected in children with chronic hepatitis of unknown etiology, especially in female patients who show hypergammaglobulinemia or some clinical features of autoimmune disease.

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Optimization of the Flip Angle and Scan Timing in Hepatobiliary Phase Imaging Using T1-Weighted, CAIPIRINHA GRE Imaging

  • Kim, Jeongjae;Kim, Bong Soo;Lee, Jeong Sub;Woo, Seung Tae;Choi, Guk Myung;Kim, Seung Hyoung;Lee, Ho Kyu;Lee, Mu Sook;Lee, Kyung Ryeol;Park, Joon Hyuk
    • Investigative Magnetic Resonance Imaging
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    • v.22 no.1
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    • pp.1-9
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    • 2018
  • Purpose: This study was designed to optimize the flip angle (FA) and scan timing of the hepatobiliary phase (HBP) using the 3D T1-weighted, gradient-echo (GRE) imaging with controlled aliasing in parallel imaging results in higher acceleration (CAIPIRINHA) technique on gadoxetic acid-enhanced 3T liver MR imaging. Materials and Methods: Sixty-two patients who underwent gadoxetic acid-enhanced 3T liver MR imaging were included in this study. Four 3D T1-weighted GRE imaging studies using the CAIPIRINHA technique and FAs of $9^{\circ}$ and $13^{\circ}$ were acquired during HBP at 15 and 20 min after intravenous injection of gadoxetic acid. Two abdominal radiologists, who were blinded to the FA and the timing of image acquisition, assessed the sharpness of liver edge, hepatic vessel clarity, lesion conspicuity, artifact severity, and overall image quality using a five-point scale. Quantitative analysis was performed by another radiologist to estimate the relative liver enhancement (RLE) and the signal-to-noise ratio (SNR). Statistical analyses were performed using the Wilcoxon signed rank test and one-way analysis of variance. Results: The scores of the HBP with an FA of $13^{\circ}$ during the same delayed time were significantly higher than those of the HBP with an FA of $9^{\circ}$ in all the assessment items (P < 0.01). In terms of the delay time, images at the same FA obtained with a 20-min-HBP showed better quality than those obtained with a 15-min-HBP. There was no significant difference in qualitative scores between the 20-min-HBP and the 15-min-HBP images in the non-liver cirrhosis (LC) group except for the hepatic vessel clarity score with $9^{\circ}$ FA. In the quantitative analysis, a statistically significant difference was found in the degree of RLE in the four HBP images (P = 0.012). However, in the subgroup analysis, no significant difference in RLE was found in the four HBP images in either the LC or the non-LC groups. The SNR did not differ significantly in the four HBP images. In the subgroup analysis, 20-min-HBP imaging with a $13^{\circ}$ FA showed the highest SNR value in the LC-group, whereas 15-min-HBP imaging with a $13^{\circ}$ FA showed the best value of SNR in the non-LC group. Conclusion: The use of a moderately high FA improves the image quality and lesion conspicuity on 3D, T1-weighted GRE imaging using the CAIPIRINHA technique on gadoxetic acid, 3T liver MR imaging. In patients with normal liver function, the 15-min-HBP with a $13^{\circ}$ FA represents a feasible option without a significant decrease in image quality.

Beneficial Effects of Water Extracts of Scutellariae Radix on Immune Function in Mice Fed Alcohol (알코올 섭취한 생쥐에서 황금(黃芩) 열수추출물이 생쥐의 면역기능에 미치는 영향)

  • Lee, In-Seok;Kang, Kyoung-Lan;Choue, Ryo-Won
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.35 no.5
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    • pp.536-542
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    • 2006
  • High fat diet and chronic alcohol consumption cause hyperlipidemia, steatohepatitis and in some cases, cirrhosis. But the detailed mechanisms are not known. Scutellariae Radix (SR) has been known to have hepatoprotective effects. The aim of this study was, therefore, to determine whether SR water extract (100 mg/kg) could affect immune function in mice abused by long-term alcohol consumption (feed 25% ethanol in water for 1 month, ad libitum) with high fat diet (40% fat of total calorie). Mice received either a regular diet (RD, AIN 93) or a high fat diet (HD); high fat diet group were divided into ethanol group (HED) or ethanol with SR water extract group (HEDS). Food consumption was measured daily and body weights recorded weekly throughout the experiment. Immunological parameters (Ig A, Ig E, TNF-$\alpha$, IFN-$\gamma$, IL-$1{\beta}$) were measured from the serum and the supernatant of spleen lymphocytes from the all groups. The concentration of serum Ig A, Ig E and cytokines were significantly higher in the alcohol consumed groups. Also the concentration of supernatant of spleen lymphocytes, Ig A, Ig E, cytokines were significantly higher in the ethanol consumption groups. Otherwise, HEDS group were significantly lower than HED group. These results suggest that SR water extract may improve the haptic immune function in mice fed high fat diet with alcohol.

Dynamic Study on the Hepatobiliary Diseases with Combination of $^{131}I$-Rose bengal and $^{198}Au$-Colloid Scintiphotography (간담도질환(肝膽道疾患)의 Scintiphotography 상(像)에 있어서 $^{131}I$-Rose bengal 및 $^{198}Au$-colloid의 섭취(攝取)와 배설(排泄)에 관(關)한 역동학적(力動學的) 연구(硏究))

  • Rhee, Yong-Kook
    • The Korean Journal of Nuclear Medicine
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    • v.5 no.1
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    • pp.49-64
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    • 1971
  • The radioactive $^{131}I$-rose bengal serial scintiphotography was performed in 62 patients with the hepatobiliary diseases and in 20 normal subjects. This approach permitted visualization of the hepatic uptake of $^{131}I$-rose bengal from the circulation and its excretion into the biliary trees and the intestines. In some of these patients, gallbladder function was examined, using eggs as a gallbladder constrictor. The time of maximum hepatic uptake was well correlated to the conventional biochemical liver function tests. In addition to $^{131}I$-rose bengal scintiphotography, $^{198}Au$-colloid scintiphotography was also performed to make comparison of these two tests. The results obtained were as follows: 1. In normal subjects, the maximum hepatic uptake of $^{131}I$-rose bengal occurred at $23{\pm}2.9$ minutes, the initial hepatic excretion at $34{\pm}5.1$ minutes, the visualization of the gallbladder at $29{\pm}5.7$ minutes and the intestinal visualization at $54{\pm}25.8$ minutes. The radioactivity in the gallbladder decreased to $10.7{\pm}5.0%$ one hour after the ingestion of eggs. 2. In the patients with cirrhosis of the liver, there was a delayed and decreased hepatic uptake. The maximun hepatic upake occurred at $43{\pm}12.9$ minutes. The differences in the results of uptake between the cirrhotic and the normal group were statistically significant. The initial hepatic excretion occurred at $60{\pm}18.5$ minutes and had tendency of delaying compared with the normal controls. The gallbladder was visualized in 13 of 16 cases (81%) and its visualization occurred at $49{\pm}14.6$ minutes with a tendency to be delayed compared with the normal controls. The intestinal visualization occurred at $63{\pm}15.8$ minutes and its delaying tendency was somewhat more prominent. 3. In patients with hepatitis, the maximum hepatic uptake occurred at $59{\pm}21.4$ minutes and was significantly delayed. The initial hepatic excretion occurred at $82{\pm}34.3$ minutes and the results revealed a delaying tendency. The gallbladder was visualized in 15 of 20 cases (75%) at $57{\pm}18.7$ minutes, which was significantly delayed. The Intestinal visualization was noted in all cases with marked delay. 4. In patients with obstructive jaundice, the maximum hepatic uptake was noted at $83{\pm}14.7$ minutes, showing the most significant delay. The hepatic excretion into biliary trees and intestines was not entirely noted in all cases except the only one case with gallbladder visualization. 5. In patients with cholelithiasis, the maximum hepatic upake and the initial hepatic excretion were slightly delayed with mean times of $39{\pm}11.2\;and\;48{\pm}17.1$ minutes respectively. The visualization of the gallbladder was demonstrated in 10 of 17 cases (59%) and occurred at $52{\pm}25.6$ minutes with a slight delay. The intestinal visualization occurred at $67{\pm}47.7$ minutes and was slightly delayed. $^{131}I$-rose bengal in the gallbladder remained high, $49.3{\pm}21.3%$, which suggested quantitatively decreased power of gallbladder constriction. 6. The time of the maximum hepetic uptake was correlated well to BSP retention and serum alkaline phosphatase ativity. However, the maximum hepatic uptake had no definite correlation with serum albumin, serum globulin, TTT, serum cholesterol, SGPT or SGOT. 7. In the diagnosis of the hepatobiliary diseases with jaundice, $^{131}I$-rose bengel serial scintiphotography has proved to be more useful than $^{198}Au$-colloid scintiphotography. With these results, it could be justified that $^{131}I$-rose bengal scintiphotography is an excellent diagnostic aid for dynamic hepatobiliary function studies in the clinical practice.

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Ascitic Fluid Analysis for the Differentiation of Malignancy-Related and Nonmalignant Ascites (악성 복수와 비악성 복수의 감별을 위한 복수액 분석)

  • Lee, Eun-Young;Kim, Byeoung-Deok;Choi, Jae-Hyuk;Lee, Sang-Yeop;Ryu, Hun-Mo;Lee, Kyung-Hee;Hyun, Myung-Soo
    • Journal of Yeungnam Medical Science
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    • v.16 no.1
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    • pp.76-84
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    • 1999
  • The differentiation between malignancy-related ascites(MRA) and non-malignant ascites (NMA) is important for further diagnostic and therapeutic purposes. Although many parameters were investigated, none has provided a complete distinction between MRA and NMA. We investigated several ascitic fluid parameters to determine the differential power, and to differentiate malignant-related from nonmalignant-related ascites with a sequence of sensitive parameters followed by specific parameters. For the present study, 80 patients with ascites were divided into two groups: MRA and NMA, The MRA group was consisted of 27 patients with proven malignancy by image study, biopsy, and follow up: 21 of these patients had peritoneal carcinomatosis, but the remaining 6 showed no evidence of peritoneal carcinomatosis. The NMA group was consisted of 53 patients with no evidence of malignancy: among these patients, one had SLE, and others had liver cirrhosis, The samples of blood and ascites were obtained simultaneously, and then the levels of ascites cholesterol, CEA. protein and LDH, cytology, albumin gradient, ascites/serum concen-tration ratios of LDH(LDH A/S), and ascites/serum concentration ratios of protein(protein A/S) were measured. Applying cut-off limits for determined parameters, we estimated the diagnostic efficacy of each parameter, Among the eight parameters investigated, ascites fluid cholesterol yielded the best sensitive value of 93%(cut-off value 30mg/dl), and cytologic examination and the protein A/S(cut-off value 0.5) showed the most specific value of 100% and 96%, respectively. Based on the above results, the diagnostic sequence with cholesterol as a sensitive parameter followed by the combination of cytologic examination and protein A/S as specific parameters, was tested in 80 patients. This diagnostic sequence identified 81.5% of patients with malignancy, and all patients with peritoneal carcinomatosis were classified as malignancy-related ascites. In spite of many limitations, this proposed diagnostic sequence may permit a cost-effective and simple differentiation of malignancy-related ascites from nonmalignant ascites.

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A basic research for evaluation of a Home Care Nursing Delivery System (가정간호 서비스 질 평가를 위한 도구개발연구)

  • Kim, Mo-Im;Cho, Won-Jung;Kim, Eui-Sook;Kim, Sung-Kyu;Chang, Soon-Bok;Ryu, Ho-Sihn
    • Journal of Home Health Care Nursing
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    • v.6
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    • pp.33-45
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    • 1999
  • The purpose of this study was to develop a basic framework and criteria for evaluation of quality care provided to patients with the attributes of disease in the home care nursing field, and to provide measurement tools for home health care in the future. The study design was a developmental study for evaluation of hospital-based HCN(home care nursing) in Korea. The study process was as follows: a home care nursing study team of College of Nursing. Yonsei University reviewed the nursing records of 47 patients who were enrolled at Yonsei University Medical Center Home Care Center in March, 1995. Twenty-five patients were insured at that time, were selected from 47 patients receiving home care service for study feasibility with six disease groups; Caesarean Section (C/S), simple nephrectomy, Liver cirrhosis(LC), chronic obstructive pulmonary disease(COPD), Lung cancer or cerebrovascular accident(CVA). In this study, the following items were selected : First step : Preliminary study 1. Criteria and items were selected on the basis of related literature on each disease area. 2. Items were identified by home care nurses. 3. A physician in charge reviewed the criteria and content of selected items. 4. Items were revised through preliminary study offered to both HCN patients and discharged patients from the home care center. Second step : Pretest 1. To verify the content of the items, a pretest was conducted with 18 patients of which there were three patients in each of the six selected disease groups. Third step : Test of reliability and validity of tools 1. Using the collected data from 25 patients with either cis, Simple nephrectomy, LC, COPD, Lung cancer, or CVA. the final items were revised through a panel discussion among experts in medical care who were researchers, doctors, or nurses. 2. Reliability and validity of the completed tool were verified with both inpatients and HCN patients in each of field for researches. The study results are as follows: 1. Standard for discharge with HCN referral The referral standard for home care, which included criteria for discharge with HCN referral and criteria leaving the hospital were established. These were developed through content analysis from the results of an open-ended questionnaire to related doctors concerning characteristic for discharge with HCN referral for each of the disease groups. The final criteria was decided by discussion among the researchers. 2. Instrument for measurement of health statusPatient health status was measured pre and post home care by direct observation and interview with an open-ended questionnaire which consisted of 61 items based on Gorden's nursing diagnosis classification. These included seven items on health knowledge and health management, eight items on nutrition and metabolism, three items on elimination, five items on activity and exercise, seven items on perception and cognition, three items on sleep and rest, three items on self-perception, three items on role and interpersonal relations, five items on sexuality and reproduction, five items on coping and stress, four items on value and religion, three items on family. and three items on facilities and environment. 3. Instrument for measurement of self-care The instrument for self-care measurement was classified with scales according to the attributes of the disease. Each scale measured understanding level and practice level by a Yes or No scale. Understanding level was measured by interview but practice level was measured by both observation and interview. Items for self-care measurement included 14 for patients with a CVA, five for women who had a cis, ten for patients with lung cancer, 12 for patients with COPD, five for patients with a simple nephrectomy, and 11 for patients with LC. 4. Record for follow-up management This included (1) OPD visit sheet, (2) ER visit form, (3) complications problem form, (4) readmission sheet. and (5) visit note for others medical centers which included visit date, reason for visit, patient name, caregivers, sex, age, time and cost required for visit, and traffic expenses, that is, there were open-end items that investigated OPD visits, emergency room visits, the problem and solution of complications, readmissions and visits to other medical institution to measure health problems and expenditures during the follow up period. 5. Instrument to measure patients satisfaction The satisfaction measurement instrument by Reisseer(1975) was referred to for the development of a tool to measure patient home care satisfaction. The instrument was an open-ended questionnaire which consisted of 11 domains; treatment, nursing care, information, time consumption, accessibility, rapidity, treatment skill, service relevance, attitude, satisfaction factors, dissatisfaction factors, overall satisfaction about nursing care, and others. In conclusion, Five evaluation instruments were developed for home care nursing. These were (1)standard for discharge with HCN referral. (2)instrument for measurement of health status, (3)instrument for measurement of self-care. (4)record for follow-up management, and (5)instrument to measure patient satisfaction. Also, the five instruments can be used to evaluate the effectiveness of the service to assure quality. Further research is needed to increase the reliability and validity of instrument through a community-based HCN evaluation.

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The Role of Radiotherapy in Treatment of Hepatocellular Carcinoma (간세포암에서 방사선 치료의 역할)

  • Eun, Jong Ryul;Choi, Kyo Won;Lee, Heon Ju;Kim, Mung Se
    • Journal of Yeungnam Medical Science
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    • v.17 no.2
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    • pp.137-145
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    • 2000
  • Background: Hepatocellular carcinomal(HCC) has been considered to be relatively radioresistant. The role of radiotherapy(RT) in the treatment of HCC is controversial. But RT has a role in the treatment of hepatocellular carcinoma as a single or combination modalities. The effect of radiotherapy on HCC was evaluated. Patients and Methods: From January 1984 through January 2000, a total of 18 patients with unresectable HCC underwent radiotherapy alone or in conjunction with transarterial embolization(TAE). We reviewed the medical ecords of patients treated with RT and measured the tumor size using measured the tumor size using planimetry method. The Kaplan-Meier method was used to calculate the survival rate. Results: The RT patients were 15 men and 3 women. The mean age was 51 years. four(22.2%) of them were accompanied with ascites. Eleven(61.1%) of them were accompanied with liver cirrhosis and their functions were 6, 3, 2 in each Child-Pugh A, B, C, respectively. A partial response(PR) was observed in 2 patients(11.1%), minimal response(MR) in 4 patients (22.2%) and no change(NC), in 11 patients(61.1%), whereas progressive disease(PD) was seen in 1 patients(6%), respectively. Conclusions: Although the radiotherapy in HCC did not improve the survival rate. it decreased the tmor size. Radiotherapy strengthens the therapeutic efficacy when combined with TAE, but more studies are needed.

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Living-Related Liver Transplantation with Heterozygote Carrier Graft in Children with Wilson Disease (윌슨병 환아에서 이형 접합 보인자 공여간을 이용한 혈연간 생체 부분 간이식)

  • Kim, Jin-Taek;Chang, Soo-Hee;Choi, Bo-Hwa;Kim, Kyung-Mo;Yoo, Han-Wook;Lee, Young-Joo;Lee, Sung-Gyu
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.6 no.2
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    • pp.161-166
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    • 2003
  • Purpose: The purpose of this study was to evaluate the efficiency of treatment of living-related liver transplantation (LRLT) with the parental heterozygote carrier graft in children with Wilson disease. Methods: We retrospectively evaluated 7 children with Wilson disease who had received liver transplantation from 1994 to 2002 at Asan Medical Center. All the donors were parental. Liver functions, Kayser-Fleischer ring, and other factors regarding to copper metabolism were analyzed. Results: Of the 7 children, 5 had fulminant hepatitis and 2 had decompensated liver cirrhosis irresponsive to medical therapy. All donors being parental, all grafts came to be heterozygote carrier grafts. Survival rate was 100% in those 7 children, 87% in all children with liver transplantation in the same period, and 84% in children with non-metabolic liver disease. After liver transplantation, all 7 children could stop low copper diet and penicillamine therapy and their AST, total bilirubin and prothrombin time were recovered to normal. After liver transplantation, ceruloplasmin and serum copper levels were also recovered to normal. A marked reduction in 24 hr-urinary copper excretion was observed in all recipients after transplantation. During follow-up, Kayser-Fleischer rings resolved completely after LRLT in 5 children and partially in 1 child. Conclusion: We concluded that living-related liver tranplantation in children with Wilson disease with parental heterozygote carrier graft is an effective treatment modality.

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