• Title, Summary, Keyword: hepatic encephalopathy

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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
    • Korean Journal of 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.

Novel Mutations in the CPT1A Gene Identified in the Patient Presenting Jaundice as the First Manifestation of Carnitine Palmitoyltransferase 1A Deficiency

  • Choi, Jong Sub;Yoo, Hyeoh Won;Lee, Kyung Jae;Ko, Jung Min;Moon, Jin Soo;Ko, Jae Sung
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.19 no.1
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    • pp.76-81
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    • 2016
  • Carnitine palmitoyltransferase 1A (CPT1A) is an enzyme functioning in mitochondrial fatty acid oxidation (FAO) of the liver. Patients with CPT1A deficiency have impaired mitochondrial FAO and display hypoketotic hypoglycemia and hepatic encephalopathy as typical manifestations. In this report, we present a case of CPT1A deficiency presenting jaundice as the first manifestation. A 1.9 years old boy showed jaundice and elevated levels of free and total carnitine were observed. From direct sequencing analysis of CPT1A, two novel mutations, c.1163+1G>A and c.1393G>A (p.Gly465Arg), were identified. At the age of 2.2 years, hypoglycemia, tachycardia, and altered mental status developed just after cranioplasty for craniosynostosis. High glucose infusion rate was required for recovery of his vital signs and mentality. Diet rich in high carbohydrate, low fat and inclusion of medium chain triglyceride oil resulted in improvement in cholestatic hepatitis and since then the boy has shown normal growth velocity and developmental milestones to date.

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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Longitudinal Intrinsic Brain Activity Changes in Cirrhotic Patients before and One Month after Liver Transplantation

  • Cheng, Yue;Huang, Li-Xiang;Zhang, Li;Ma, Ming;Xie, Shuang-Shuang;Ji, Qian;Zhang, Xiao-Dong;Zhang, Gao-Yan;Zhang, Xue-Ning;Ni, Hong-Yan;Shen, Wen
    • Korean Journal of Radiology
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    • v.18 no.2
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    • pp.370-377
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    • 2017
  • Objective: To evaluate the spontaneous brain activity alterations in liver transplantation (LT) recipients using resting-state functional MRI. Materials and Methods: Twenty cirrhotic patients as transplant candidates and 25 healthy controls (HCs) were included in this study. All patients repeated the MRI study one month after LT. Amplitude of low-frequency fluctuation (ALFF) values were compared between cirrhotic patients (both pre- and post-LT) and HCs as well as between the pre- and post-LT groups. The relationship between ALFF changes and venous blood ammonia levels and neuropsychological tests were investigated using Pearson's correlation analysis. Results: In the cirrhotic patients, decreased ALFF in the vision-related regions (left lingual gyrus and calcarine), sensorimotor-related regions (left postcentral gyrus and middle cingulate cortex), and the default-mode network (bilateral precuneus and left inferior parietal lobule) were restored, and the increased ALFF in the temporal and frontal lobe improved in the early period after LT. The ALFF decreases persisted in the right supplementary motor area, inferior parietal lobule, and calcarine. The ALFF changes in the right precuneus were negatively correlated with changes in number connection test-A scores (r = 0.507, p < 0.05). Conclusion: LT improved spontaneous brain activity and the results for associated cognition tests. However, decreased ALFF in some areas persisted, and new-onset abnormal ALFF were possible, indicating that complete cognitive function recovery may need more time.

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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Intrahepatic Portosystemic Shunt Fixed By Transvenous Coil Embolization in a Samoyed Dog (샤모예드종 개에서 발생한 간내성 문맥-정맥 문합을 경정맥 코일장착으로 치료한 증례)

  • Lee, Moo-Hyun;Lee, Seoung-Jin;Lee, Seung-Gon;Moon, Hyeong-Sun;Lee, Joon-Seok;Choi, Ran;Park, In-Chul;Hyun, Chang-Baig
    • Journal of Veterinary Clinics
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    • v.24 no.3
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    • pp.426-431
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    • 2007
  • A 5-month-old female Samoyed dog was presented with primary complaints including exercise tolerance and neurological sign associated with hepatic encephalopathy. The major findings in clinical examination included an intermittent seizure, anemia, elevated pre- and post-prandial serum bile acid, hypoproteinemia and bilirubinuria. Diagnostic imaging studies revealed an intrahepatic portosystemic shunt (IPSS). The shunted vessel was successfully occluded by transvenous coil embolization. Clinical signs were gradually improved after shunt occlusion. This case is a rare case of IPSS in a large breed dog fixed by transvenous coil embolization.

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.

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
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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.

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.