[Recovery after serious mushroom poisoning (grade IV encephalopathy) with intensive care support and without liver transplantation. Clinical case]. / Guarigione dopo intossicazione grave da funghi selvatici (encefalopatia di IV grado) con sola terapia medica intensiva e senza trapianto di fegato. Caso clinico.

Despite consistent improvement in its treatment, amatoxin poisoning still extolls an elevated overall mortality, ranging between 10 and 15%, which approaches 100% when severe (grade 3-4 encephalopathy) hepatic failure supervened. Therefore, the proper treatment of intoxication by amatoxin containing mushrooms, and particularly of its complications, remains a challenge in emergency medicine. Klein and coworkers reviewed the role of liver transplantation in amatoxin poisoning as a useful therapeutic tool for patients with severe impairment of liver function. Their indication for intervention is the presence of any of the following signs: grade 2 encephalopathy or higher; prothrombin time twice than normal, despite fresh frozen plasma infusion; hypoglycemia requiring hypertonic glucose infusion; hyperbilirubinemia (greater than 25 mg/dl). During the past autumn two patients with fulminant hepatic failure due to amatoxin poisoning were referred to our institutions as candidates for liver transplantation, since both satisfied Klein's criteria. However, due to shortage of organ donors it was impossible to transplant them over the following days. Despite they did not receive liver transplantation, both patients wakened from coma, their liver function improved, and they recovered from terminal amatoxin poisoning. After one year, both patients are long-term survivors, in good health and without any sequelae either in brain or liver function.

Effect of taurine supplementation on fat and bile acid absorption in patients with cystic fibrosis.

Eleven children with cystic fibrosis (CF) and pancreatic insufficiency were given supplementation with taurine (30-40 mg/kg/day) for 2 months, while taking their usual dosage of enzymatic therapy. One patient dropped out of the study because she developed severe constipation. In the other 10 patients, urinary taurine excretion (88 +/- 30.1 mg/m2s.a./24 h) was similar to that of controls (86.2 +/- 6 mg/m2s.a./24 h) before taurine and increased markedly after supplementation (618.2 +/- 79.97 mg/m2s.a./24 h), indicating efficient intestinal absorption. Their coefficient of fat absorption was 81.2 +/- 2.3% and increased significantly after taurine (91.3 +/- 1.13%; p less than 0.01); the area under the curve of plasma triglyceride postprandial levels (1 +/- 0.1 mg X min/ml) also increased significantly after taurine (1.4 +/- 0.3 mg X min/ml; p less than 0.05), showing values very similar to those of controls. Conversely, no change was observed in the serum postprandial levels of glycocholic acid: the maximum postprandial peak before (1.2 +/- 0.3 mumol/l) and after taurine (1 +/- 0.1 mumol/l) remained significantly lower than in controls (2.4 +/- 0.3 mumol/l); p less than 0.01 and p less than 0.001, respectively. Mean total fecal bile acid (BA) excretion was 10.24 +/- 2.15 mg/kg/day before taurine and 12.8 +/- 4.27 mg/kg/day after taurine (normal pediatric values, 2.91 +/- 1.1 mg/kg/day); however, in the individual patients we found a variable trend, four of them showing a net increase in fecal BA excretion.(ABSTRACT TRUNCATED AT 250 WORDS)