1/8. Late manifestation of Indian childhood cirrhosis in a 3-year-old German girl.We report on a 3.8-year-old German girl who presented with signs of subacute liver failure based on a 4-month history. Investigations done before admission excluded infectious, metabolic and immunological diseases. Laboratory findings were increased values of aminotransferases, hyperbilirubinaemia, hyperammonaemia and deteriorated plasmatic coagulation. Caeruloplasmin and serum copper concentrations were normal; however, urinary copper excretion was elevated (80 microg/l). Liver biopsy showed a micronodular liver cirrhosis and an extremely high liver copper concentration (1400 microg/g dry weight). Epidemiological investigations revealed an elevated copper concentration (8645 microg/l, normal: <2000) and a low pH value (6.3) of the drinking water supplied by copper pipes. The girl had been exposed to copper-contaminated drinking water since the age of 2 years. CONCLUSION: Laboratory, histopathological findings and a proven chronic copper intoxication lead to the diagnosis of Indian childhood cirrhosis in a German girl. Whereas this disease is mostly described in patients with increased copper intake in infancy, our patient developed toxic liver cirrhosis with relatively late copper exposure. Indian childhood cirrhosis should be considered in the differential diagnosis of early childhood liver cirrhosis.- - - - - - - - - - ranking = 1keywords = intoxication (Clic here for more details about this article) |
2/8. Fulminant liver failure in a young child following repeated acetaminophen overdosing.acetaminophen (paracetamol), a widely used analgetic drug, is well tolerated at therapeutic doses, but may cause severe hepatotoxicity when ingested in large overdose. Self-poisoning is still very popular in adults and accidental ingestion of one single overdose occurs occasionally in children. In contrast, lethal intoxication in children after repeated administration of therapeutic doses is a very rare event. This case report describes an iatrogenic acetaminophen overdosing in a 5-year-old child receiving 8.5 g acetaminophen in 48 h. Fulminant liver failure developed within 60 h. autopsy findings included panlobular liver cell necrosis. acetaminophen serum levels were rather low compared to cases with ingestion of one single overdose. Postmortem diagnosis of chronic acetaminophen intoxication as cause of death should include the clinical history as well as, if available, the calculated drug serum half-life.- - - - - - - - - - ranking = 2keywords = intoxication (Clic here for more details about this article) |
3/8. mars: optimistic therapy method in fulminant hepatic failure secondary to cytotoxic mushroom poisoning--a case report.BACKGROUND: Poisoning by cytotoxic mushrooms (amanita phalloides and related species) is associated with severe morbidity and a high mortality rate (lethality > 20% in adults and > 50% in children). The main causes of this intoxication are the amatoxines, which inhibit dna-dependent rna polymerase ii or B. This interaction leads to a tight complex, and the inhibition is of a non-competitive type (1); in addition to those tight binding inhibitors of adenosine kinase, papain, cathepsin l, cathepsin b, cysteine proteinase and bromelain (2), inhibit the synthesis of messenger RNA in the hepatocytes, decrease the formation of coagulation factors and of immunoglobulins and effect a vasoconstriction. They also have an influence on the transcription and lesions that are seen in cells with rapid protein synthesis, particularly in liver and renal cells, with the cellular changes causing the fragmentation and segregation of all nuclear components, even at low toxin concentrations (3). Phallotoxin, which is the other toxin isolated from death cap, binds with a high affinity to microfilamentous structures - in particular, to F-actin, which stimulates the polymerization of G-actin, stabilizes the F-actin filaments, irreversibly polymerizes actin filaments and causes cholestasis (4). Liver is recognized as the target organ for amanita phalloides toxins; it is presented by fatty degeneration, acute toxic dystrophy and centrilobular necroses (5). Therapeutic options employed to treat mushroom intoxication, such as hemodiaperfusion on activated charcoal, high dosages of penicillin g, oral charcoal, etc., very often failed to act properly and liver transplantation (when a graft is available) appeared to be the only solution. The most polarized debate concerns the value of extracorporeal elimination. plasmapheresis and peritoneal dialysis proved much less useful for this purpose; neither haemodialysis (HD) nor haemoperfusion (HP) contributed to the clearance of amatoxin (6, 7). Recently, Stange et al. (8). introduced a new detoxication method (referred to as mars) for protein-bound substances in patients with liver failure and grade III and IV hepatic encephalopathy. mars was performed with an albumin-containing dialysate, which is recycled in a closed loop that contains a charcoal cartridge, an anion exchanger resin adsorber and a conventional haemodialyser. With dialysis using an albumin-containing dialysate, protein-bound substances, which are usually not sufficiently dialysable, can be eliminated. The treatments increase the rate of toxin elimination to the extent that the toxic exposure of highly susceptible cells, such as hepatocytes, is minimized. This leads to the surprise recovery of the poisoning patient, despite her severe condition, even as late as up to a week after mushroom ingestion.- - - - - - - - - - ranking = 2keywords = intoxication (Clic here for more details about this article) |
4/8. Ecstasy-induced brain death and acute hepatocellular failure: multiorgan donor and liver transplantation.BACKGROUND: Ecstasy is a neurotoxic and hepatotoxic drug. brain edema and fulminant hepatic failure are two of the most serious complications associated with the consumption of ecstasy. Acute ecstasy intoxication can transform a patient into an organ donor or a hepatic graft recipient. MATERIALS AND methods: In the last 5 years in our centers, we have had two multiorgan donors who died from ecstasy-induced brain edema and three patients who required urgent orthotopic liver transplantation for treatment of severe acute hepatocellular failure induced by this drug. We performed eight transplantations using the organs of these two brain-dead donors: one heart, one bipulmonary, three kidneys, one kidney-pancreas, and two livers. RESULTS: Toxicity caused by ecstasy was not observed in any of the eight patients who underwent transplantation. The clinical state and the graft function of the heart, two liver, renopancreatic, and three kidney recipients were normal for a follow-up period that ranged between 7 months and 4.5 years. The lung recipient died from multiorgan failure secondary to bilateral pneumonia 5 days after the transplantation, and one of the kidney transplant patients died as a result of intestinal lymphoma 6 months after transplantation. The three liver transplantations in the three patients with ecstasy-induced fulminant hepatic failure were performed successfully using orthotopic transplantation. These three recipients are asymptomatic and have normal-functioning hepatic grafts after follow-up of 3.5 years, 15 months, and 11 months, respectively. CONCLUSIONS: The thoracic and abdominal organs of people dying from ecstasy intoxication can be viable for transplantation. The short- and medium-term survival of the graft and of the recipient have been similar to that of other organ donors. Urgent liver transplantation is an effective therapeutic option in patients with ecstasy-induced acute hepatocellular failure.- - - - - - - - - - ranking = 2keywords = intoxication (Clic here for more details about this article) |
5/8. Initial experience with urgent adult-to-adult living donor liver transplantation in fulminant hepatic failure.BACKGROUND: The prognosis of patients with fulminant hepatic failure without timely liver transplantation is dismal. Given the limited availability of cadaveric organs for urgent transplantation in israel, adult-to-adult living donor segmental liver transplantation may be the only alternative. OBJECTIVES: To report our initial experience with urgent lifesaving LDLT in this unique scenario. methods: Three adult patients with FHF (two of unknown etiology, one with paracetamol intoxication) were transferred from other institutions and admitted to our intensive care unit. Initial treatment and monitoring included intracranial pressure monitoring and hepatic dialysis using the Molecular Adsorbent Recirculating System. Expeditious potential donor selection included medical, psychosocial and surgical evaluation. Liver volume and vascular anatomic compatibility were assessed with computed tomography angiography. RESULTS: Between July and October 2003 we performed three procedures of urgent adult-to-adult LDLT. The donors (two uncles, one sister) underwent hepatic resection (two right lobes, one left lateral segment) and recovered well. The recipients underwent total hepatectomy with caval preservation, followed by lobar grafting. All recipients recovered and are alive with good liver function and without any neurologic complications. CONCLUSIONS: Urgent adult-to-adult living donor segmental liver transplantation can be performed safely and timely as a lifesaving procedure in the setting of comatose patients with FHF.- - - - - - - - - - ranking = 1keywords = intoxication (Clic here for more details about this article) |
6/8. Auxiliary partial orthotopic liver transplantation for acute liver failure: the Hannover experience.APOLT has been developed both to enable the native liver to regenerate in acute liver failure and to avoid the risks of long-term immunosuppressive therapy. We present our experience with APOLT in 4 patients with acute liver failure. The patients were 33, 18, 5, and 34 years of age, respectively. The causes of hepatic failure were: one hellp syndrome, one paracetamol intoxication, and 2 causes which remained undetermined. All patients were in coma before transplantation. First symptoms had occurred 13, 2, 30, and 20 days prior to APOLT, respectively. In all cases, segments II and III of the recipient's own liver were resected before implanting the auxiliary liver orthotopically. The auxiliary graft consisted of segments II and III in 2 cases and of II, III, and IV in the other 2 patients. The auxiliary graft showed good initial function in all cases. All 4 patients are alive 5 years, 15 months, 6 months, and one month after transplantation. In the last patient, an arterial thrombosis of the graft on the 5th postoperative day required retransplantation. The immunosuppressive therapy could be stopped in 3 cases and the graft was removed in 2 patients 15 and 40 days after APOLT, respectively. This shows that APOLT represents an effective treatment for patients with acute liver failure, which enables restoration of native liver function. Thus, APOLT should be considered in every patient with acute and potentially reversible liver failure in whom a transplantation is indicated.- - - - - - - - - - ranking = 1keywords = intoxication (Clic here for more details about this article) |
7/8. manganese intoxication and chronic liver failure.manganese intoxication and chronic liver failure are associated with strikingly similar clinical, imaging, and pathological abnormalities. As manganese is primarily cleared by the liver, inadequate elimination of manganese absorbed from the normal diet may lead to manganese overload in patients with liver disease. We report a significant elevation of blood manganese concentration in 3 patients with biopsy-proved hepatic cirrhosis who exhibited neurological dysfunction and characteristic abnormal signal hyperintensity in the globi pallidi and substantia nigra on T1-weighted magnetic resonance imaging. We speculate that manganese accumulation in the brain accounts for the magnetic resonance imaging abnormalities and contributes to neurological dysfunction in patients with liver disease.- - - - - - - - - - ranking = 5keywords = intoxication (Clic here for more details about this article) |
8/8. acetaminophen toxicity in children: diagnostic confirmation using a specific antigenic biomarker.Chronic acetaminophen (APAP) toxicity poses a difficult diagnostic challenge to the clinician. signs and symptoms are nonspecific and no currently available laboratory study can confirm APAP as the causative agent of hepatic injury. In this study an antigenic biomarker for APAP toxicity was used to confirm the diagnosis of APAP-induced hepatic failure in two children with chronic APAP toxicity. APAP that has been metabolized to N-acetyl-benzoquinone imine (NAPQI) reacts with cellular proteins to form 3-(cystein-S-yl)-APAP protein adducts (3-Cys-A). serum from both patients was submitted for quantitation of 3-Cys-A by a competitive inhibition enzyme-linked immunosorbent assay (ELISA). Concentrations of 3-Cys-A in the two patients were 1.97 and 2.77 nmol/mg protein, which are similar to concentrations found in adults with hepatic injury secondary to an overdose of APAP. Individuals with no exposure to APAP have no detectable 3-Cys-A in serum. It was concluded that 3-Cys-A is a useful marker of APAP intoxication after long-term ingestion of APAP when total dose and time course of ingestion are uncertain, and may prove to be a useful clinical and investigative tool in the study of APAP intoxication.- - - - - - - - - - ranking = 2keywords = intoxication (Clic here for more details about this article) |