• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.1
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• pp.108-113
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• 2006

In case of Wilson's disease complicated with hemolytic anemia and fulminant hepatic failure; plasma exchange or liver transplantation should be considered. We report an 11 year-old male with fulminant Wilson's disease who developed hemolytic crisis. He was recovered by exchange transfusion after 6 times of plasma exchange.

• Tuberculosis and Respiratory Diseases
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• v.63 no.5
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• pp.435-439
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• 2007

Standard antituberculous therapy, including isoniazid (INH), rifampin, ethambutol, and pyrazinamide (PZA), is widely used to treat active tuberculosis. The most important side effect is hepatotoxicity. In a standard four-drug regimen, PZA was the most common cause of drug-induced hepatitis and was dose-related. The incidence of drug-induced hepatitis is high at doses of 40~70 mg/kg per day but has fallen significantly since the recommended dose was reduced. Liver toxicity induced by PZA is rare at doses of 25 mg/kg per day or less. PZA-induced fulminant hepatic failure is also rare but fatal. We report a case of fulminant hepatic failure caused by a re-challenge of PZA.

• Journal of The Korean Society of Clinical Toxicology
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• v.2 no.2
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• pp.133-136
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• 2004

Phalloides syndrome has usually occurred after incidental mushroom ingestion. It is sometimes difficult to differentiate toxic mushrooms from edible ones, especially in lay person because of their morphological similarities. In Korea, Amanita virosa and Amanita subjunquillea have been reported as a cause of fulminant hepatic failure in mushroom poisoning (phalloides syndrome). We report a case of phalloides syndrome who came to death with fulminant hepatic failure even though we planned liver transplantation and used T-PLS(R) (Twin Pulse Life Support) for hemodynamic support while waiting for the donor.

• Proceedings of the KSME Conference
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• 2008.11a
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• pp.1419-1426
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• 2008

Fulminant hepatic failure is a clinical syndrome associated with a high mortality rate. Orthotopic liver transplantation is the only clinically proven effective treatment for patients with end-stage liver disease who do not respond to medical management. A major limitation of this treatment modality is the scarcity of donor organs available, resulting in patients dying while waiting for a donor liver. An extracorporeal bioartificial liver (BAL) device containing viable hepatocytes has the potential to provide temporary hepatic support to liver failure patients, serving as a bridge to transplantation while awaiting a suitable donor. In some patients, providing temporary hepatic support may be sufficient to allow adequate regeneration of the host liver, thereby eliminating the need for a liver transplant. Although the BAL device is a promising technology for the treatment of liver failure, there are several technical challenges that must be overcome in order to develop systems with sufficient processing capacity and of manageable size. In this overview, the authors describe the critical issues involved in developing a BAL device. They also discuss their experiences in hepatocyte culture optimization within the context of a microchannel flat-plate BAL device.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.sup2
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• pp.50-58
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• 2008

Acute liver failure is a devastating disease in children. Most cases of acute liver failure in children are indeterminate; however, metabolic liver disease is one of the main causes in the pediatric age group. Though a major symptom of acute liver failure is hepatic encephalopathy, this is very difficult to diagnose, particularly in younger children. Liver transplantation has improved the chances of survival dramatically; however, it is not known which patients are ideal candidates for liver transplantation. Because patients may deteriorate rapidly, arranging care in a center with expertise will secure the best possible outcomes.

• Pediatric Infection and Vaccine
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• v.24 no.3
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• pp.188-192
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• 2017

Respiratory syncytial virus (RSV) typically causes lower respiratory tract infections in children, and most patients recover successfully. However, some infants and young children can have a severe course of disease with respiratory failure, and extrapulmonary manifestations can occur in severe RSV disease. We report one case of severe RSV bronchiolitis complicated with acute myocarditis, fulminant hepatic failure, and disseminated intravascular coagulation.

• Archives of Pharmacal Research
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• v.28 no.1
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• pp.81-86
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• 2005

The hepatoprotective effects of chalcone derivatives were evaluated in D-galactosamine/lipopolysaccharide (D-GaIN/LPS)-induced fulminant hepatic failure in mouse. Thirteen chalcone derivatives were synthesized for study and their hepatoprotective effects were evaluated by assessing aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels in serum. Chalcone preparations were injected into mice at 12 hand 1 h before intraperitoneal injection of D-GaIN/LPS. After abdominal administration, changes in AST and ALT between the control and treated groups were observed. Ten of the synthesized chalcone derivatives exhibited inhibitory effects on D-GaIN/LPS-induced levels of AST and ALT in mice. Compounds 2, 3, 8, 9, and 12 markedly reduced serum AST and ALT at 8 h, inhibited hepatocyte necrosis and showed significant hepatoprotective activities. The activity of compound 3 was compared with the bifendate (DDB) through oral administration. Compound 3 showed much higher inhibitory effects than bifendate for decreasing AST and ALT activity. The results indicate that compound 3 has strong hepatoprotective activity through suppression of tumor necrosis factor­alpha (TNF-alpha) preduction, reduction of the histological change in the liver, and attenuated of hepatocyte apoptosis confirmed by DNA fragmentation assay.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.18 no.2
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• pp.121-127
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• 2015

Purpose: To evaluate clinical and laboratory profile of Wilson's disease (WD) in children. Methods: This cross sectional study was conducted at Bangabandhu Sheikh Mujib Medical University Hospital. Bangladesh, over a period of 3 years. One hundred consecutive children of WD between 3 to 18 years of age were evaluated. Results: Mean age was $8.5{\pm}1.5years$. Male female ratio was 2:1. Ninety-one percent of patients were Muslim and 9% Hindu. A total of 53% cases of hepatic WD presented between 5 to 10 years of age and most of the neurologic WD manifested in 10-15 years age group. Sixty-nine children presented only with hepatic manifestations, 6 only with neurological manifestations, 14 with both hepatic and neurological manifestation, 10 children was asymptomatic and 1 patient presented with psychiatric features. WD presented as chronic liver disease (CLD) in 42%, CLD with portal hypertension in 34%, acute hepatitis in 20% and fulminant hepatic failure in 4% cases. Stigmata of CLD were found in 18% patients. Keiser-Fleischser ring was found in 76% total patients. Elevated serum transaminase was found in 85% cases, prolonged prothrombin time in 59% cases and hypoalbuminaemia in 53% cases. A total of 73% patients had low serum ceruloplasmin, basal urinary copper of >$100{\mu}g/day$ was found in 81% cases and urinary copper following penicillamine challenge of >$1,200{\mu}g/day$ was found in 92% cases. Conclusion: Majority of studied WD children presented with hepatic manifestation of which 76% presented with CLD. Any child presented with jaundice after the age of 3 years should be investigated for WD.

• 한국생물공학회:학술대회논문집
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• 2003.10a
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• pp.305-306
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• 2003

To treat fulminant hepatic failure (FHF) patients, various extracorporeal bioartificial liver (BAL) systems have been developed. Several requirements should be met for the development of BAL systems: hepatocytes should be cultured in a sufficiently high density; their metabolic functions should be of a sufficiently high level and duration; and the BAL systems module should permit scaling-up and aseptic handling. Several investigators have found that freshly isolated primary hepatocytes can be cultured into three dimensional, tightly packed, freely suspended, multicellular aggregates, or spheroids. These specialized cell structures exhibited enhanced liver specific functions and a prolonged differentiated state compared to cells maintained in a monolayer culture. Cells in spheroids appear to mimic the morphology and ultrastructure of the in vivo liver lobule. The ability of hepatocytes to organize into three-dimensional structures was hypothesized to contribute to their enhanced liver-specific activities. In this study, the ammonia removal rate and urea secretion rate of pig hepatocytes spheroids encapsulated in Ca-alginate bead were determined. A packed-bed bioreactor with encapsulated pig hepatocytes was devised as BAL support system. The efficacy of the system was evaluated in vitro.

• Journal of Biomedical Engineering Research
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• v.19 no.3
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• pp.215-224
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• 1998

This paper is fundamental study to develope the extracorporeal liver support system for patient with fulminant hepatic failure(FHF) or being expected for orthotopic liver transplantation. The polyurethane foam, which is composed of the density of 33kg/m3, the average pore diameter of 500${\mu}{\textrm}{m}$, the closed window of 60-70%, was manufactured with the prepolymer of 15% NCO-, Hepatocytes were inoculated to form spheroids in polyurethane foam. The time of spheroid formation in BSA(Bovine Serum Albumin) coated polyurethane foam was shorter than that in raw polyurethane foam. To verify the function of hepatocyte spheroids, we measured ammonia removal rate, urea and albumin secretion rate. Polyurethane foam was suitable for culture of hepatocyte spheroids. And culture of hepatocyte spheroids in polyurethane foam has high possibility in using as an extracorporeal liver support system.