Primary liposarcoma of the liver: a case report and review of literature.

Liposarcoma is a rare mesenchymal malignant tumor, which usually originates in the retroperitoneum and the extremities. Seven cases of primary liposarcoma of the liver have been previously reported. We present the eighth case, which occurred in an adult female patient. Primary liposarcoma of the liver, although extremely rare, must be considered in the differential diagnosis of a hepatic mass that develops in a noncirrhotic liver, especially in patients who are potential candidates for orthotopic liver transplantation. Liposarcoma is an absolute contraindication for liver transplantation.

Malignant vascular tumors of the liver presenting as liver failure and portal hypertension.

We describe three patients referred for orthotopic liver transplantation with liver failure and portal hypertension who were found to have malignant vascular tumors: two patients with angiosarcoma and one patient with epithelioid hemangioendothelioma. Their clinical presentation mimicked decompensated chronic liver disease. None had tumor masses on computed tomography and ultrasonography. Massive tumor involvement of the liver was identified in the two patients studied by magnetic resonance imaging. Pathological examination of the explanted liver at the time of transplantation in one patient and autopsy in a second patient showed angiosarcoma. Laparoscopic liver biopsies in the third patient showed epithelioid hemangioendothelioma. The vascular origin of the tumor was established by histopathologic examination and confirmed with immunohistochemistry. Malignant vascular tumors of the liver should be included in the differential diagnosis of liver failure of unclear etiology.

Acute hepatitis associated with the Chinese herbal product jin bu huan.

OBJECTIVE: To describe the hepatotoxicity associated with ingestion of the Chinese herbal product Jin Bu Huan Anodyne Tablets (Lycopodium, serratum) and to propose possible mechanisms of injury. DESIGN: Retrospective analysis. SETTING: Academic hepatology units and private practice facilities. PATIENTS: Seven previously healthy patients. MEASUREMENTS: Clinical, laboratory, radiologic, and histologic studies. RESULTS: Acute hepatitis occurred after a mean of 20 weeks (range, 7 to 52 weeks) of Jin Bu Huan ingestion and resolved in six patients within a mean of 8 weeks (range, 2 to 30 weeks); another patient is currently improving. Hepatitis was associated with symptoms of fever, fatigue, nausea, pruritus, and abdominal pain and with signs of jaundice and hepatomegaly. Biopsy specimens showed that one patient had hepatitis with eosinophils (consistent with a drug reaction) and the other had mild hepatitis, moderate fibrosis, and microvesicular steatosis. Decreasing the Jin Bu Huan dose in one patient improved liver test results. Reusing Jin Bu Huan in two other patients caused abrupt recrudescence of hepatitis. CONCLUSION: Jin Bu Huan can cause liver injury. Although the hepatotoxic mechanisms are not defined, they may include hypersensitive or idiosyncratic reactions or direct toxicity to active metabolites. Hepatotoxicity caused by herbal products underscores the toxicity caused by herbal products underscores the importance of national surveillance programs and quality control of the manufacture of these products.

Acute liver failure due to lymphoma. A diagnostic concern when considering liver transplantation.

Lymphomatous involvement of the liver may present as acute liver failure but is an absolute contraindication for liver transplantation. Therefore it is imperative to diagnose such patients since survival in this group is poor and recurrence is high. We describe two patients with acute liver failure referred for liver transplantation whose diagnostic testing revealed hepatic lymphoma. These cases underscore the importance of considering lymphoma in the differential diagnosis of acute liver failure prior to liver transplant.

Control of cerebral oedema by total hepatectomy and extracorporeal liver support in fulminant hepatic failure.

Keeping a patient with fulminant hepatic failure (FHF) alive until a donor liver is available for transplantation can be a problem. We describe an 18-year-old woman with paracetamol-induced FHF, who was treated by total hepatectomy, hypothermia, plasma exchange, and extracorporeal liver support. The patient was anhepatic for 14 h. The liver-support system consisted of plasma separation and perfusion through a charcoal filter and a hollow-fibre module seeded with matrix-attached porcine hepatocytes. With artificial liver treatment there was reversal of severe neurological dysfunction, normalisation of intracranial pressure, and decreased serum ammonia. The patient underwent emergency transplantation with an ABO-incompatible liver, followed by transplantation with a compatible organ eight days later. The patient has fully recovered and is neurologically intact.

Treatment of fulminant hepatic failure with insulin and glucagon. A randomized, controlled trial.

Insulin and glucagon are among the therapeutic modalities that have been investigated in the treatment of fulminant hepatic failure (FHF). We have completed a randomized, controlled trial of insulin and glucagon in 38 patients with FHF from either viral or toxin exposure. The control and treatment groups consisted of 21 and 17 patients, respectively, and did not differ significantly in etiology or admission laboratory values. Mortality was not significantly different between control and treatment groups and was 67% and 82%, respectively. Time from randomization to death or discharge was not significantly different between the two groups. Peak levels of alpha-fetoprotein were statistically higher in survivors than in nonsurvivors (P less than 0.01). We conclude that even though a type-2 error may exist, the combination of insulin and glucagon is not useful in the treatment of FHF.

Spontaneous Salmonella infection of high-protein noncirrhotic ascites.

Spontaneous bacterial peritonitis rarely complicates high-protein (greater than 2.5 g/dl) ascites. The relatively high endogenous antimicrobial (opsonic) activity of the ascitic fluid in this setting appears to protect the patient from infection. We report two patients with high-protein, noncirrhotic ascites complicated by spontaneous peritonitis due to Salmonella species. One patient had ascites due to heart failure, whereas the other patient's ascites was due to peritoneal carcinomatosis. The ascitic fluid total protein concentrations were 3.1 and 3.3 g/dl, respectively, and the opsonic activity of the ascitic fluid specimens were 2.03 and 2.00 log kill, respectively, indicating a high degree of bacterial killing. We hypothesize that the virulence of the Salmonella species was able to overcome the high opsonic activity in the ascitic fluid, resulting in infection in these two patients. Fever, abdominal pain, or encephalopathy in a patient with high-protein ascites may suggest the presence of an unusual organism causing spontaneous bacterial peritonitis.

Failure of rifampin to relieve pruritus in chronic liver disease.

We investigated the effect of rifampin on pruritus in 12 patients with chronic liver disease: non-A, non-B hepatitis (n = 3), alcoholic cirrhosis (n = 4), primary biliary cirrhosis (n = 4), and primary sclerosing cholangitis (n = 1). The study was a crossover, randomized, double-blind trial where placebo and drug were given daily in identical capsules (300 mg) for 2 weeks each, with a 1 week washout before and after each cycle. Mean duration of pruritus was 1.6 years (range of 4 months-5 years). Blood tests were done weekly and patients used a visual analogue scale (VAS) from 0 to 100 to mark their level of itchiness daily. Only transaminases were significantly lower while the patients were on rifampin. VAS scores were minimally affected by either rifampin or placebo. At the end of the trial, four patients said they were less itchy on rifampin and three preferred placebo. Of these seven patients, small falls in VAS scores occurred in two patients on rifampin and two on placebo; there was no change in the remaining three. There was little change in serum bile salt levels during the trial. No patient became jaundiced and deepening of jaundice did not occur in the four patients with initially elevated bilirubin. We conclude that a daily 300 mg dose of rifampin was not effective in relieving pruritus in a variety of chronic liver diseases.

A study to examine agreement between endoscopy and histology for the diagnosis of columnar lined (Barrett's) esophagus.

Eleven subjects with a previous diagnosis of columnar lined esophagus and 12 control patients underwent esophagogastroduodenoscopy, including vital staining with Lugol's iodine and biopsy. Histology in 47 of 48 biopsies from controls agreed with endoscopic findings, and no patient had a columnar lined esophagus. Of 60 biopsies taken from study patients, the endoscopic appearance in 8 (13%) was not consistent with the histology. Good agreement was demonstrated between endoscopy and histology (kappa = 0.72). Compared with histology as the reference measure, the sensitivity, specificity, and accuracy of endoscopy were 89%, 93%, and 91%, respectively.

Treatment of primary liver graft nonfunction with prostaglandin E1.

Primary nonfunction following orthotopic liver transplantation is characterized by rapidly rising serum transaminases, minimal bile production, and severe coagulopathy, which can progress to hypoglycemia, hepatic encephalopathy, and acute renal failure. Untreated it has a mortality of over 80% and to date the only treatment has been retransplantation. As a result of the beneficial effect of Prostaglandin E1 infusion in patients with fulminant hepatic failure, this trial was conducted to determine whether PGE1 would be of value in primary nonfunction. We have encountered 16 cases of primary nonfunction in 94 liver transplants, an incidence of 17%. Initially in the program, there were 6 occurrences of nonfunction that did not receive PGE1; 3 underwent retransplantation (2 survivors), 2 died awaiting another liver, and in one recovery of hepatocellular function occurred with supportive care but the patient died of cytomegalovirus infection. Ten patients received PGE1 within 4-34 hr of transplantation. Within 12 hr of treatment, 8 patients responded with a significant fall in the AST (129 U/hr) whereas, in the untreated group, the AST continued to rise (267 +/- 102 U/hr) at the same rate as prediagnosis (337 +/- 95 U/hr). At the conclusion of the infusion (4-7 days) in the 8 responders, there were significant decreases in AST (4386 +/- 546 U/L to 102 +/- 21 U/L), prothrombin time (22 +/- 2 to 12 +/- .4 sec) and partial thromboplastin time (45 +/- 3-29 +/- 4 sec), and significant increases in coagulation factor V (26 +/- 8 to 95 +/- 12%) and factor VII (10 +/- 5 to 61 +/- 4%). No serious side effects occurred, although 2 patients developed diarrhea, and abdominal cramps. Two patients treated with PGE1 were retransplanted at 10-36 hr and were considered nonresponders. Graft survival was 80% in the PGE1-treated group and 17% in the untreated group (P less than 0.05) and patient survival was 90% and 33%, respectively. This study suggests a potential benefit of PGE1 in the treatment of primary nonfunction.

Treatment of cryptosporidiosis with spiramycin in AIDS. An "N of 1" trial.

We describe a randomized, double-blind, placebo-controlled, crossover trial with spiramycin in a single patient with acquired immune deficiency syndrome (AIDS) and a severe secretory diarrhea caused by cryptosporidium. Spiramycin, a potentially harmful antibiotic, had no clinical or microbiological effect in this patient. The application of the single patient (N of 1) trial to common clinical problems is a simple way to analyze the value of different therapeutic approaches. The time-consuming, expensive, multi-patient trial with ultimate extrapolation to the individual patient can be avoided. Single-patient trials can influence management and improve patient care and have potentially wide use in patients with gastrointestinal disease.

Nutritional absorption in short bowel syndrome. Evaluation of fluid, calorie, and divalent cation requirements.

Eight patients with a short bowel resulting from intestinal resection and clinically stable for at least one year were studied for 10 days. The diet chosen was lactose-free with a low fiber content and contained 22% of total calories as protein, 32% as carbohydrate, and 46% as fat. Total fluid volume was kept constant, and all patients were in positive nitrogen balance. During the 10-day period, blood chemical concentrations, stool, and/or ostomy volume, urine volume, electrolyte excretion, and calorie and divalent cation absorption were measured. In addition it was determined that fluid restriction during meals did not affect these parameters. In these patients the absorptions of fat, carbohydrate, protein, and total calories were 54%, 61%, 81%, and 62%, respectively. Similarly the absorption of the divalent cations, calcium, magnesium, and zinc, were 32%, 34%, and 15%, respectively. We suggest that patients with short bowel syndrome, who have been stable for at least one year and who can tolerate oral diets, do not need to restrict fat or to separate fluids from solids during their meals. Furthermore, they should increase their oral intake to 35-40 kcal/kg ideal body weight in order to counteract their increased losses. The diet should contain 80-100 g protein/day in order to maintain a positive nitrogen balance and a large margin of safety. In addition, these patients may take oral supplementation of calcium, magnesium, and zinc to maintain divalent cation balance.

Diet for patients with a short bowel: high fat or high carbohydrate?

Eight patients with a short bowel resulting from intestinal resection and clinically stable for at least 6 mo were studied on two diets. Each diet was given for 5 days at a time and crossed over with the other. Both diets contained 20% of total calories as protein. The high-fat diet had 60% of calories as fat and 20% as carbohydrate. This ratio was reversed in the high-carbohydrate diet. Both diets were lactose free with low fiber. Fluid intake was kept constant. The results showed that there was no difference in the blood chemistry, stool, or ostomy volume, the zinc, calcium, and magnesium balances, urine volume, and electrolyte excretion between patients on the two diets. Bomb calorimetry showed that the total calories absorbed and excreted were comparable between the two diets. It was concluded that low-fat diets had no special benefit in the overall nutrition of the patient who has been in remission in regard to bowel disease for 6 mo or longer. Hence, dietary restriction is not recommended in these patients. However, this study did not resolve the question of the requirements and losses of fat-soluble vitamins in such patients when on a high-fat diet.

Effect of sleep fragmentation on ventilatory and arousal responses of sleeping dogs to respiratory stimuli.

We studied the effects of sleep fragmentation on arousal and ventilatory responses to hyperoxic hypercapnia, isocapnic hypoxia, and chemical stimulation of the larynx during sleep in 5 dogs. Sleep fragmentation was induced by repeatedly arousing the dogs with acoustic stimuli throughout 2 to 3 consecutive nights. Responses to respiratory stimuli were then studied during a subsequent daytime sleep. Arterial O2 saturation was measured with an ear oximeter, and sleep stage was determined by electroencephalographic and behavioral criteria. Hypercapnic and hypoxic ventilatory responses were unimpaired by sleep fragmentation. In contrast, alveolar PCO2 levels at arousal increased after sleep fragmentation, from a mean +/- SEM of 52.2 +/- 1.4 mm Hg to 55.6 +/- 1.5 mm Hg (p < 0.05) during slow-wave sleep, and from 57.9 +/- 1.5 mm Hg to 61.3 +/- 2.2 mm Hg (p < 0.05) during rapid-eye movement sleep. Similarly, arterial O2 saturation at arousal decreased after sleep fragmentation from 80.1 +/- 1.0% to 70.2 +/- 2.7% (p < 0.05) during slow-wave sleep, and from 66.3 +/- 3.6% to < 55% (p < 0.05) during rapid-eye-movement sleep. Arousal responses to laryngeal stimulation were also impaired after sleep fragmentation. We conclude that arousal responses to respiratory stimuli are decreased by sleep fragmentation.