• Toxicological Research
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• 제33권4호
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• pp.299-304
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• 2017

Thallium and its compounds are a class of highly toxic chemicals that cause wide-ranging symptoms such as gastrointestinal disturbances; polyneuritis; encephalopathy; tachycardia; skin eruptions; hepatic, renal, cardiac, and neurological toxicities; and have mutagenic and genotoxic effects. The present research aimed to evaluate the efficacy of the chelating agents deferasirox (DFX) and deferiprone (L1) in reducing serum and tissue thallium levels after the administration of thallium (III), according to two different dosing regimens, to several groups of Wistar rats for 60 days. It was hypothesized that the two chelators might be more efficient as a combined therapy than as monotherapies in removing thallium (III) from the rats' organs. The chelators were administered orally as either single or combined therapies for a period of 14 days. Serum and tissue thallium (III) and iron concentrations were determined by flame atomic absorption spectroscopy. Serum and tissue thallium (III) levels were significantly reduced by combined therapy with DFX and L1. Additionally, iron concentrations returned to normal levels and symptoms of toxicity decreased.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권1호
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• pp.80-85
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• 2019

Purpose: Abernethy malformation is a rare condition, which was first described in 1793 as a congenital extrahepatic porto-systemic shunt (CEPS) directing splanchnic blood flow into the inferior vena cava. Eighty cases have been published so far that reported CEPS, while in Korea, very few cases have been reported. Through this study, we present 6 cases of patients diagnosed with CEPS at Samsung Medical Center and compare these with other such cases published in France and China. Methods: We reviewed clinical, laboratory, and imaging data of 6 children with CEPS in our pediatric clinic between 2004 and 2017. Results: A total of 6 children with CEPS was included in this study, namely, one with type 1a, two with type 1b, and three with type 2 CEPS. The most common presenting symptom was gastrointestinal bleeding (50.0%). Therapeutic interventions included shunting vessel ligation (16.7%) in type 2 CEPS and liver transplantation (16.7%) in type 2 CEPS patient with suddenly developed hepatic encephalopathy. Conclusion: There is no consensus guideline for the optimal management of patients with CEPS. Large-sample studies regarding CEPS are needed to evaluate the characteristics of patients with CEPS and determine the treatment guideline for CEPS.

• 대한유전성대사질환학회지
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• 제16권1호
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• pp.34-41
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• 2016

성인기에 나타난 운동으로 인한 스트레스와 과량의 단백질 투여에 의한 의식 변화, 기억 장애, 행동 장애등의 고암모니아혈증성 뇌병증 소견을 토대로 저자들은 성인형 제 2형 시트룰린혈증을 의심하였다. 검사 결과에서 고암모니아혈증, 혈중 시트룰린 상승, 요중 오로트산 경도 상승을 보였으며, 이를 통해 성인형 제 2형 시트룰린혈증을 진단하였다. SLC25A13 유전자 분석 결과를 통해 환자에게서 복합 이형 접합성 돌연변이(IVS11+1G>A, c.674C>A)를 확인하였다. 환자의 가족에서도 유전자 분석 검사를 진행하였고, 아버지, 어머니, 남동생에게서 성인형 제 2형 시트룰린혈증 보인자를, 언니에게서 성인형 제 2형 시트룰린혈증 환자임을 확인하였다. 그 동안 보고된 성인형 제 2형 시트룰린혈증의 임상 경과를 고려했을 때, 증상이 없던 언니에게서 질환을 발견하고 보존적 치료를 선제적으로 시작함으로써, 신경학적 장애 없이 일상 생활을 영위하고, 추가적인 뇌 손상을 방지하기 위한 간 이식 등 장기적인 치료 계획을 수립했다는 점에서 의의가 있다. 본 환자의 증례를 통해 고암모니아혈증성 뇌병증이 발생한 성인에서, 간질환 및 뇌질환의 증거가 없으며 다른 뚜렷한 원인이 없는 경우에는, 성인형 제 2형 시트룰린혈증을 고려해야 한다는 점을 인지하였다. 빈도가 드문 질환이지만 간성 혼수로 흔히 오인되고 있으며, 조기 진단 및 적절한 치료가 이루어지지 않으면 비가역적인 신경학적 후유증이 발생할 수 있다. 또한 다른 요소 회로 대사 질환 및 간성 혼수와는 달리, 고탄수화물 식이가 질병의 경과를 인위적으로 악화시킬 수 있기에 적절한 대사 이상 검사 및 유전자 검사가 시행되어야 하겠다. 본 증례는 반복적인 고암모니아혈증성 뇌병증 소견을 보인 37세 여성과 뇌병증 소견이 없었던 언니에게서 성인형 제 2형 시트룰린혈증을 진단하고, 즉각적인 치료를 통해 심각한 신경학적 장애 없이 일상 생활을 지속하고 있는 환자들을 문헌 고찰과 함께 보고하는 바이다.

• KSBB Journal
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• 제23권5호
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• pp.381-386
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• 2008

본 연구에서는 암모니아에 의해 손상된 사람의 간세포주 Hep G2 cell과 rat의 hepatocyte에 대하여 metronidazole이 간 세포 손상을 억제하는 효과가 있음을 밝혔다. Metronidazole은 암모니아에 의한 세포 생존율 감소, 배지내의 암모니아 수준 및 지질과산화 증가 및 항산화 효소 발현 감소 그리고 세포 내 DNA 손상과 세포사멸을 억제하였다. 따라서 metronidazole은 암모니아로부터 기인하는 세포손상을 감소시켜 간세포 기능을 보호함으로써 간 기능의 저하로 발생한 과암모니아혈증에 효과적인 치료제로서의 가능성을 시사한다.

• 한국임상약학회지
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• 제26권4호
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• pp.341-347
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• 2016

Background: Liver cirrhosis causes substantial socio-economic burden and is one of the major severe liver diseases in Korea. Nonetheless, there is only a few studies that analyzes disease burden of liver cirrhosis in Korea. Such study must be carried out due to its increasing need from the invention of new drugs for chronic hepatitis and demand for cost-effectiveness analyses. Methods: Patient sample data with ensured representativeness was analyzed retrospectively to compare the medical costs and uses for patients with compensated cirrhosis and decompensated cirrhosis. Patient claims data that include K74 and K703 from the year of 2014 were selected. Within the selected data, decompensated cirrhosis patient was identified if complications such as ascites (R18), encephalopathy (B190), hepatic failure (K72), peritonitis (K65), or esophageal varices (I85) were included, and they were compared to compensated cirrhosis patients. Results: 6,565 patients were included in the analysis. The average cost per patient was 6,471,020 (SD 8,848,899) KRW and 2,173,203 (4,220,942) KRW for decompensated cirrhosis and compensated cirrhosis, respectively. For inpatients, the average hospitalized days was 38.0 (56.4) days and 27.2 (57.2) days for decompensated cirrhosis and compensated cirrhosis, respectively. For outpatients, the average number of visits was 8.7 (9.1) days and 5.3 (7.5) days for compensated cirrhosis and decompensated cirrhosis, respectively. Conclusion: Compared to compensated cirrhosis patients, decompensated cirrhosis patients had higher costs, especially for hospitalization, injection, examination, and drugs administrated within medical institutions.

• Clinical and Experimental Pediatrics
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• 제56권5호
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• pp.224-226
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• 2013

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is characterized by a severe idiosyncratic reaction including rash and fever, often with associated hepatitis, arthralgias, lymph node enlargement, or hematologic abnormalities. The mortality rate is approximately 10%, primarily owing to liver failure with massive or multiple disseminated focal necrosis. Here, we report a case of a 14-year-old girl treated with vancomycin because of a wound infection by methicillin-resistant Staphylococcus aureus, who presented with non-specific symptoms, which progressed to acute liver failure, displaying the hallmarks of DRESS syndrome. With the presence of aggravated hepatic encephalopathy and azotemia, the patient was refractory to medical treatments, she received a living-donor liver transplantation, and a cure was achieved without any sign of recurrence. Vancomycin can be a cause of DRESS syndrome. A high index of suspicion and rapid diagnosis are necessary not to miss this potentially lethal disease.

• 생약학회지
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• 제36권1호통권140호
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• pp.56-59
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• 2005

Urease plays an important role in the urea metabolism and the effect of urease activity on human and environment is enormous. For instance, urease acts as a virulence factor of the urinary and gastrointestinal tracts infections in human and animal, being involved in kidney stone formation, catheter encrusatation, pyelonephritis, ammonia encephalopathy, hepatic coma, and urinary tract infections. Widespread urease activity in soil induces a plant damage due to ammonia toxicity and pH increase. Therefore, urease activity regulation through urease inhibitors would lead to an enhanced efficiency of urea nitrogen uptake in plants and to the improved therapeutic strategies for ureolytic bacterial infections. To search for new inhibitory compounds on urease activity from herbs, MeOH extracts of herbs were screened. Among of them, the MeOH extracts of Areca catechu exhibited an excellent inhibitory effect on urease activity. Two compounds were isolated from the ethyl acetate fraction by the activity guided fractionation. Their chemical structures were identified as (+)-catechin(compound I) and allantoin(compound II) by spectroscopic evidence, respectively. Compound I showed a stronger inhibitory effect on urease activity than compound II.

• 한국방사선학회논문지
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• 제8권1호
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• pp.35-42
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• 2014

본 연구의 목적은 단일 복셀 자기공명 분광법을 이용하여 비정상적인 뇌질환을 가진 환자의 뇌대사물질이 어떻게 다른지를 알아보는데 있다. 수소 자기공명분광법의 정량적 분석의 결과 부신백질이영양증, 간선뇌병증, 뇌경색은 백질에 관련된 대사물질에 변화를 보였다. 세질환 모두 NAA가 감소하였고, ALD에서는 Cho, mI 과 Lac가 증가하였고, 간성뇌병변에서는 Cho가 감소, 그리고 뇌경색에서는 ${\beta}{\cdot}{\gamma}$-Glx와 Lac가 증가되었다. 결론적으로 수소 자기공명분광법은 비정상적으로 발전하는 뇌질환의 대사물질의 변화를 관찰하여 병을 진단할 수 있고 이는 임상에서 뇌질환의 진단과 예후를 평가하는데 유용할 것이다.

• 대한한의학회지
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• 제37권4호
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• pp.36-44
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• 2016

Objectives: Granulocyte-colony-stimulating factor (G-CSF) mobilized bone marrow (BM)-derived hematopoietic stem cells could contribute to improvement of liver function. In addition, liver fibrosis can reportedly be prevented by the Rg 1 component of red ginseng. This study investigated the combined effect of G-CSF and red ginseng on decompensated liver cirrhosis. Methods: Four patients with decompensated liver cirrhosis were injected with G-CSF to proliferate BM stem cells for 4 days ($5{\mu}g/kg$ bid subcutaneously) and followed-up for 3 months. The patients also received red ginseng for 4 days (2 tablets tid per os). We analyzed Child-Pugh scores, Model for End-Stage Liver Disease (MELD) scores and cirrhotic complications. Results: All patients showed marked increases in White blood cell (WBC) and CD34+ cells in the peripheral blood, with a peak time of 4 days after G-CSF injection. Spleen size also increased after G-CSF injection, but not severely. At end of the study, 2 patients showed improvement in Child-Pugh scores, hepatic encephalopathy, and refractory ascites. During the clinical trial period, none of the 4 patients showed any other adverse events or deterioration of liver function. Conclusions: We conclude that G-CSF/red ginseng combination therapy is relatively effective in improving liver function and major complications of decompensated liver cirrhosis without adverse effects. Further clinical trials are warranted to assess the clinical effects of G-CSF for decompensated liver cirrhosis.

• 호스피스학술지
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• 제7권2호
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• pp.6-14
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• 2007

Purpose: Hepatocellular carcinoma is the 3rd leading cause of cancer death in Korea and its prognosis is very poor. We aimed to investigate the clinical characteristics of terminal patients with hepatocellular carcinoma on admission into a hospice unit, and to know if they had received appropriate hospice and palliative care. Methods: We retrospectively reviewed the medical records in 62 patients with hepatocellular carcinoma who had admitted, received palliative care, and died in a hospice unit between January 2003 and December 2005. Results: The median age of patients was 56.5 years with 50 men(80.65%) and 12 women(19.35%) and gender ratio(male to female) was 417. Child-Pugh class A, B, and C were 6(9.68%), 22(35.38%), and 34(58.84%) respectively. We divided the patients into two groups and compared, the terminal HCC patients with class C as group I and those with class A & B as group 2. The median time from hospice referral to death was significantly short in group 1 with 15.5 days compared to group 2 with 53 days. Statistically more prevalent symptoms in group I were ascites, dyspnea, peripheral edema, and hepatic encephalopathy with abnormal laboratory findings (jaundice, hypoalbuminemia, or renal insufficiency). There, however, was no significant difference in complications and managements during admission between group 1 and 2. Conclusion: Most terminal HCC patients were often accompanied with chronic liver disease. The length of hospice and palliative care for above patients was not enough to attend them. Therefore, we suggest that proper education and information should be provided to physicians, patients, and their family members for effective hospice and palliative care.