• Title/Summary/Keyword: hepatic encephalopathy

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Chelation of Thallium (III) in Rats Using Combined Deferasirox and Deferiprone Therapy

  • Salehi, Samie;Saljooghi, Amir Sh.;Badiee, Somayeh;Moqadam, Mojtaba Mashmool
    • Toxicological Research
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    • v.33 no.4
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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.

The Characteristics and Outcomes of Abernethy Syndrome in Korean Children: A Single Center Study

  • Kim, Eun Sil;Lee, Ki Wuk;Choe, Yon Ho
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.22 no.1
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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.

A Case of Adult-onset Type II Citrullinemia Confirmed by Mutation of SLC25A13 (SLC25A13 유전자 돌연변이로 확진된 성인형 제 2형 시트룰린혈증 1례)

  • Jeung, Min Sub;Yang, Aram;Kim, Jinsup;Park, Hyung-Doo;Lee, Heon Ju;Jin, Dong-Kyu;Cho, Sung Yoon
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.16 no.1
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    • pp.34-41
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    • 2016
  • Adult-onset type II citrullinemia (CTLN2) is characterized by episodes of neurologic symptoms associated with hyperammonemia leading to disorientation, irritability, seizures, and coma. CTLN2 is distinct from classical citrullinemia, which is caused by a mutation of the argininosuccinic acid synthetase (ASS) gene. The serum citrulline level is elevated, while the activity of ASS in liver tissue is decreased. CTLN2 is known to have a poor prognosis if the proper treatment is not taken. We reported a female aged 37 years who developed recurrent attacks of altered consciousness, aberrant behavior, and vomiting. We initially suspected the patient had CTLN2 because of the signs of hyperammonemic encephalopathy, such as altered mentality, memory disturbance, and aberrant behaviors provoked by exercise-induced stress and excessive intravenous amino acid administration. Through her peculiar diet preferences and laboratory findings that included hyperammonemia and citrullinemia, we diagnosed the patient as CTLN2, and SLC25A13 sequencing revealed known compound heterozygous mutations (IVS11+1G>A, c.674C> A). Her parents were heterozygous carriers, and we identified that her older sister had the same mutations. The older sister had not experienced any episodes of hyperammonemia, but she had peculiar diet preferences. The patient and her sister have been well with conservative management. When considering the clinical course of CTLN2, it was meaningful that the older sister could be diagnosed early in an asymptomatic period and that preemptive treatment was employed. Through this case, CTLN2 should be considered in adults who present symptoms of hyperammonemic encephalopathy without a definite etiology. Because of its rare incidence and similar clinical features, CTLN2 is frequently misdiagnosed as hepatic encephalopathy, and it shows a poor prognosis due to the lack of early diagnosis and proper treatment. A high-carbohydrate diet, which is usually used to treat other urea cycle defects, can also exaggerate the clinical course of CTLN2, so proper metabolic screening tests and genetic studies should be performed.

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Metronidazole Reduced Ammonia Toxicity in Human Hep G2 cell and Rat Hepatocytes (Hep G2 세포와 rat 간세포에서 Metronidazole에 의한 암모니아 독성 감소)

  • Kim, Bo-Ae;Kim, Hyun-Jung;Kim, You-Young
    • KSBB Journal
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    • v.23 no.5
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    • pp.381-386
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    • 2008
  • Lipophilic ammonia is toxic gas and can easily diffuse across cell membranes. Excess ammonia is implicated in the pathogenesis of several metabolic disorders including hepatic encephalopathy and may result in the death. The purpose of this study was to clarify the inhibition effect of metronidazole on liver cell damage due to ammonia in human Hep G2 cell and rat hepatocytes. The effects of metronidazole were studied in ammonium chloride treated human Hep G2 cell (75 mM) and rat hepatocyte (100 mM) following $0.1{\mu}M$ metronidazole treatment. In MTZ+AC group, cell viabilities increased prominently and LDH activities decreased over 25% than AC group. Furthermore, ammonia level according to ammonium chloride treatment reduced over 30% and lipid peroxidation as an index of cell membrane damage decreased more than twice. By comparison with control, catalase activity showed more than 30% reduction in AC group while less than 10% reduction in MTZ+AC group, respectively. In addition, MTZ+AC group showed the similar cell structure as control in cell morphology study by using light microscope, and represented fluorescent intensity decrement compared with AC group in fluorescent microscopic study with avidin-TRITC fluorescent dye. And cleaved PARP expression due to ammonia reduced twofold or more in MTZ+AC group. As the results suggest, metronidazole may protect the liver cell by inhibiting cell damages due to ammonia and be used for an effective antagonist of ammonia in hyperammonemia.

Analysis of Medical Use and Costs Related to the Management of Liver Cirrhosis Using National Patients Sample Data (환자표본자료를 이용한 간경변증 환자의 의료이용 특성 및 의료비용 분석)

  • Kim, Hye-Lin;Park, Jae-A;Sin, JiYoung;Park, Seung-Hoo;Lee, Eui-Kyung
    • Korean Journal of Clinical Pharmacy
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    • v.26 no.4
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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.

Liver transplantation in a child with acute liver failure resulting from drug rash with eosinophilia and systemic symptoms syndrome

  • Song, Seung Min;Cho, Min Sung;Oh, Seak Hee;Kim, Kyung Mo;Park, Young Seo;Kim, Dae Yeon;Lee, Sung Gyu
    • Clinical and Experimental Pediatrics
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    • v.56 no.5
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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.

Isolation of Urease Inhibitory Compounds from Arecae Semen (빈랑자 (Arecae Semen)로부터 Urease 억제 활성 물질의 분리)

  • Ryu, Jei-Man;Jang, Hwan-Bong;Rho, Yang-Kook;Oh, Seong-Jun;Lee, Hyun-Yong;Leem, Moon-Jeong
    • Korean Journal of Pharmacognosy
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    • v.36 no.1 s.140
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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.

A Study on the Metabolite Changes in Brain Diseases: 3 Teslar 1H Magnetic Resonance Spectroscopy (뇌질환 대사물질 변화의 고찰; 3 Teslar 수소 자기공명분광법)

  • Eun, Sungjong;Kim, Jeongjae;Yoo, Seungcheol
    • Journal of the Korean Society of Radiology
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    • v.8 no.1
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    • pp.35-42
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    • 2014
  • The purpose of this study is to know the differences of metabolism in abnormal brain disease using a single-voxel proton MR spectroscopy(1H MRS) Together with five normal volunteers and each five patients with brain diseases, pathologically proved, underwent MRI and 1H MRS. The quantitative results of 1H MRS in adrenoleukodystrophy(ALD), hepatic encephalopathy(HE), and infarction gave unique information on the metabolite changes related with the white matter: the concentration of NAA decreased in all diseases; Cho, mI and Lac increased in ALD; Cho decreased in HE; and ${\beta}{\cdot}{\gamma}$-Glx and Lac increased in infarction. It is concluded that 1H MRS is capable of diagnosing brain diseases by monitoring metabolite changes in vivo that subsequently develope into abnormalities. 1H MRS may be a useful clinical tool for in both diagnosis and prognosis of brain diseases.

Combined Effect of Granulocyte-Colony-Stimulating Factor-Induced Bone Marrow-Derived Stem Cells and Red Ginseng in Patients with Decompensated Liver Cirrhosis (Combined Effect of G-CSF and Red Ginseng in Liver Cirrhosis)

  • Kim, Hyun Hee;Kim, Seung Mo;Kim, Kyung Soon;Kwak, Min A;Kim, Sang Gyung;Kim, Byung Seok;Lee, Chang Hyeong
    • The Journal of Korean Medicine
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    • v.37 no.4
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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.

Hospice and palliative care for the terminal patients with hepatocellular carcinoma (간세포암 환자의 호스피스 완화 의료)

  • Gil, Hyeon-Ja;Mun, Do-Ho
    • Korean Journal of Hospice Care
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    • v.7 no.2
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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.

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