1/16. Inappropriate liver transplantation in a child with Alpers-Huttenlocher syndrome misdiagnosed as valproate-induced acute liver failure.A 3-yr-old boy received valproic acid (VPA) for recurrent seizures. He developed coma and acute liver failure that were attributed to VPA toxicity, and underwent emergency orthotopic liver transplantation (OLTx). Despite good graft function, his neurological state worsened and led to death a few months later. The diagnosis of Alpers-Huttenlocher syndrome (AHS) was suspected, subsequently to liver Tx, in view of ongoing neurologic deterioration and magnetic resonance imaging (MRI) findings. The syndrome, recessively inherited, associates brain degeneration with liver failure, and is now considered a mitochondrial disease. Enzyme activity deficiencies of the respiratory chain were identified in muscle mitochondria, as well as morphologic abnormalities of mitochondria in the explanted liver. Guidelines for diagnosis are presented, in order to differentiate the liver failure in AHS from that induced by genuine VPA toxicity. It is recommended to avoid liver Tx in patients with AHS given the fatal neurological course of the disease.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
2/16. Early experiences with a porcine hepatocyte-based bioartificial liver in acute hepatic failure patients.Orthotopic liver transplantation (OLT) is the only effective therapeutic modality in severe acute hepatic failure (AHF). The scarcity of organs for transplantation leads to an urgent necessity for temporary liver support treatments in AHF patients. A hepatocyte-based bioartificial liver (BAL) is under investigation with the main purpose to serve as bridging treatment until a liver becomes available for OLT, or to promote spontaneous liver regeneration. We developed a novel radial-flow bioreactor (RFB) for three-dimensional, high-density hepatocyte culture and an integrated pumping apparatus in which, after plasmapheresis, the patient's plasma is recirculated through the hepatocyte-filled RFB. Two hundred thirty grams of freshly isolated porcine hepatocytes were loaded into the RFB for clinical liver support treatment. The BAL system was used 8 times in supporting 7 AHF patients in grade III-IV coma, all waiting for an urgent OLT Three patients with no history of previous liver diseases were affected by fulminant hepatic failure (FHF) due to hepatitis b virus, 3 by primary non-function (PNF) of the transplanted liver, and one by AHF due to previous abdominal trauma and liver surgery. Six out of 7 patients underwent OLT following BAL treatment(s), which lasted 6-24 hours. All patients tolerated the procedures well, as shown by an improvement in the level of encephalopathy, a decrease in serum ammonia, transaminases and an amelioration of the prothrombin time, with full neurological recovery after OLT Our initial clinical experience confirms the safety of this BAL configuration and suggests its clinical efficacy as a temporary liver support system in AHF patients.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
3/16. A case of acute hepatic insufficiency treated with novel plasmapheresis plasma diafiltration for bridge use until liver transplantation.A patient with acute hepatic insufficiency induced by a drug presented to our institution, and we performed a novel plasmapheresis that we call plasma dia-filtration (PDF). The patient was a 36 year old woman. She underwent 11 sessions of PDF for a duration of about 9 h for each procedure using the Evacure EC-2A filter together with 20 units of fresh frozen plasma and dialysate simultaneously. serum levels of total bilirubin and prothrombin time were significantly improved after she underwent each procedure. However, after the third procedure the levels returned to the same level as on the previous day. Encephalopathy improved after the first procedure, and this improvement was maintained until the ninth procedure. The patient prepared to undergo liver transplantation after the tenth procedure because of the development of hepatic coma, but she died of respiratory insufficiency before undergoing the procedure. Accordingly in this case, PDF worked to maintain liver function in acute liver failure and may act as bridge therapy until the patient can undergo liver transplantation.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
4/16. rhabdomyolysis accompanying thyroid crisis: an autopsy case report.rhabdomyolysis is occasionally associated with metabolic disorders such as diabetic coma, severe electrolyte disturbances and myxedema coma. We describe rhabdomyolysis accompanying thyroid crisis. A 50-year-old man with Graves' disease developed rhabdomyolysis, congestive heart failure and hepatic failure during the course of thyroid crisis and then died of acute renal failure. Postmortem examination revealed rhabdomyolysis in the cardiac and psoas muscles, old myocardial infarction, hepatic centrilobular necrosis, renal cortical necrosis, and follicular hyperplasia in the thyroid. Circulatory collapse and dehydration under excessive hypermetabolic state presumably suppressed the source of energy and oxygen for muscle cells, leading to cellular damage.- - - - - - - - - - ranking = 2keywords = coma (Clic here for more details about this article) |
5/16. A case of acute liver failure caused by herpes simplex type 2.Unlike mucocutaneous infections, disseminated herpes simplex virus infections are rare and often fatal owing to acute liver failure (ALF). Typically, the course of the disease is rapid and the lack of specific symptoms may result in delay in diagnosis. This study reports a case of genital herpes caused by herpes simplex-type 2 that resulted in ALF. The patient was a 24-y-old woman with a 1 y history of Crohn's disease, treated with oral prednisolone. She was hospitalized with fatigue, anorexia and abdominal pain. Blood tests showed pancytopenia, renal failure and coagulopathy. Pelvic examination revealed signs of severe colpitis and prompt therapy with parenteral acyclovir was initiated. Despite the early institution of antiviral therapy, progressive hepatic coma, gastrointestinal bleeding, oliguria and severe intracranial hypertension characterized the clinical course. The patient received intensive supportive care and recovered without liver grafting. A subsequent screening for immunodeficiency diseases revealed an immeasurable blood mannose-binding lectin (MBL) concentration. 10 weeks after admission, she was discharged for further rehabilitation. This case stresses the importance of suspecting disseminated herpes virus infection in patients with ALF without known aetiology as it may secure prompt initiation of antiviral therapy and reduce the risk that transplantation is needed for survival.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
6/16. Living related liver transplantation for acute fulminant hepatitis B: experience from two possible hyper-acute cases.Fulminant hepatic failure, which is represented by fulminant hepatitis, is fatal in most cases unless prompt liver transplantation is performed. Even if liver transplantation is performed, irreversible neurological damage is often complicated. In this case report, we describe two cases of fulminant hepatitis induced by acute hepatitis b virus infection, both of which were successfully rescued by living related liver transplantation without significant complications. The case 1 was a 45-year-old Japanese male. He complained general malaise and anorexia. His local physician diagnosed him as acute hepatitis B, and referred to our hospital. Due to severe coagulopathy, plasma exchange was performed 3 times. However, his hepatic coma progressed rapidly along with rapid decrease of both his direct/indirect bilirubin (D/T) ratio and serum blood urea nitrogen (BUN) levels. Living related liver transplantation was performed under the diagnosis of acute fulminant hepatitis B. The case 2 was a 34-year-old Japanese male. His complaints were fever and skin rush. He was referred to our hospital under the diagnosis of acute hepatitis B. On the second day after admission, he developed grade II hepatic coma, which deteriorated into grade III in spite of intensive therapy including plasma exchange. He also demonstrated rapid decrease of both D/T ratio and serum BUN level. Living related liver transplantation was performed on the next day. Both cases recovered without any evidence of neurological sequelae. In general, it is extremely difficult to rescue fulminant hepatitis by conservative treatments, particularly in cases with rapid progression. Although emergency liver transplantation may be an only option to rescue in such a case, living related liver transplantation has an advantage in view of urgent organ donation over cadeveric transplantation.- - - - - - - - - - ranking = 2keywords = coma (Clic here for more details about this article) |
7/16. Acute liver failure: a message found under the skin.Acute liver failure is a rare syndrome with rapid progression and high mortality. It is characterised by the onset of coma and coagulopathy usually within six weeks but can occur up to six months after the onset of illness. Viral hepatitis, idiosyncratic drug induced liver injury, and acetaminophen ingestion are common causes. This report describes the case of a 35 year old man who presented with acute liver failure shortly after binge drinking. Repeated history taking disclosed a gluteal disulfiram implant that the patient had received to treat his alcohol dependence. The patient recovered with maximum supportive care after surgical removal but without liver transplantation. This case illustrates that only meticulous history taking will disclose the sometimes bewildering causes of acute liver failure.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
8/16. Case of fulminant hepatic failure due to unrecognized peripartum cardiomyopathy.OBJECTIVE: To describe a postpartum patient who presented with fulminant hepatic failure and hepatic coma as a result of unrecognized peripartum cardiomyopathy. DESIGN: Case report. SETTING: Medical intensive care unit of a tertiary care academic medical center. PATIENT: A 35-yr-old woman 5 wks postpartum from an uneventful spontaneous vaginal delivery who was transferred to our institution with fulminant hepatic failure and worsening hepatic encephalopathy of unknown etiology for consideration of liver transplantation. INTERVENTIONS: An echocardiogram was obtained as part of an evaluation for refractory shock and the patient was found to have severe global hypokinesis with an ejection fraction of approximately 15%. She was diagnosed with peripartum cardiomyopathy and treatment with digoxin and afterload reduction was initiated. MEASUREMENTS AND MAIN RESULTS: After initiation of appropriate treatment for dilated cardiomyopathy, the patient's hepatic failure resolved and she made a full recovery. CONCLUSIONS: Congestive heart failure is one of the few treatable causes of fulminant hepatic failure. Congestive heart failure must always be included in the differential diagnosis of fulminant hepatic failure of unknown pathogenesis.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
9/16. Living donor liver transplantation with noninvasive ventilation for exertional heat stroke and severe rhabdomyolysis.A 16-year-old male with exertional heat stroke (EHS) had extensive hepatocellular damage, severe rhabdomyolysis, renal failure, and coma. hemodiafiltration was started on day 2 and living donor liver transplantation was performed on day 3. He received continuous mechanical ventilation with intubation before and after the surgery. As his mental status improved, he could not tolerate intubation, and he was extubated on postoperative day (POD) 26. He received facial noninvasive positive pressure ventilation until POD 50. hemodiafiltration was discontinued on POD 52. He was discharged on POD 67 and is currently well more than a year after transplantation. A literature search indicates that this patient is the first long-term survivor (>1 year) after liver transplantation for exertional heat stroke.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
10/16. Acute fulminant hepatitis B in a patient with diabetic nephropathy treated successfully with concomitant lamivudine and molecular adsorbents recirculating system.A 36-year-old man with type 2 diabetes and diabetic nephropathy treated with hemodialysis developed hepatitis b virus (HBV)-induced acute fulminant hepatic failure (FHF). Despite supportive treatment, the condition rapidly progressed as manifested by severe jaundice, coagulopathy and hepatic coma. He was placed on the waiting list for liver transplantation and was treated with lamivudine and extracoporeal liver support with the molecular adsorbent recirculating system (mars). After three 8-h sessions of mars treatment in 1 week, he had remarkable improvement in clinical symptoms and serum biochemistry. On the 14th hospital day, surface antigen seroconversion was noted with undetectable hepatitis b virus surface antigen (HBs Ag) and low titre of anti-HBs antibody, indicating a complete recovery from acute fulminant hepatitis B. mars treatment has been reported to benefit patients with liver failure from different causes including acute exacerbation of chronic hepatitis B, poisoning, post transplantation and Wilson's disease. The present case suggests its potential benefit when combined with lamivudine in treating uremic patients with acute fulminant hepatitis B.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
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