Budd-Chiari syndrome: portacaval shunt and subsequent liver transplantation

The Budd-Chiari syndrome (BCS) is caused by hepatic venous outflow obstruction, which often leads to death as a result of portal hypertension and liver failure. Therapeutic approaches vary widely from conventional medical therapy to liver transplantation. If and when a patient suffering with BCS needs surgery remains a matter of contention. However, it is well accepted that portacaval shunt surgery and orthotopic liver transplantation represent efficient surgical treatments of this condition. We report on a patient with an eventful course after BCS was diagnosed. After portacaval shunt surgery the patient had acute liver failure and had a successful orthotopic liver transplantation.     

Long-term experience after ex situ liver surgery

BACKGROUND: Ex situ liver surgery allows liver resection and vascular reconstruction in patients who have liver tumors located at critical sites. Only a small series of studies about ex situ liver surgery is available in the literature. No long-term results have been published. METHODS: Twenty-four patients were considered for ex situ liver surgery because conventional liver surgery was considered impossible or too hazardous. The patient's ages were 51.3 +/- 7.5 years. Indications were various primary and secondary liver malignancies and benign liver tumors in 2 patients. RESULTS: In 22 of 24 patients, the ex situ liver resection and subsequent autotransplantation were performed. The anhepatic periods in these patients lasted for 5.6 +/- 1.1 hours. In the remaining 2 patients, autotransplantation was not possible and allogenic liver transplantation was performed 17 and 19 hours after hepatectomy. In 4 patients, liver failure occurred after autotransplantation and required transplantation. The confluens between hepatic veins and the inferior vena cava was reconstructed in 5 patients. Fifteen patients survived the postoperative period and were discharged after 36.5 +/- 16 days. The median survival time of 6 patients who had metastases of colonic carcinoma was 21 months. The 2 patients with benign liver disease are alive 9 and 5 years after ex situ surgery. CONCLUSIONS: Extended liver resections with difficult reconstructions of the hepatic venous confluens are feasible by ex situ liver surgery and subsequent autotransplantation. However, the early postoperative mortality rate is high, especially in patients with cholestatic livers. Early tumor recurrence remained the problem in these patients with extended local tumor spread. Ex situ liver surgery should only be performed in selected patients.     

Isolated hyperthermic liver perfusion with high dose tumor necrosis factor alpha in pigs: an experimental study in preparation of clinical Use

Isolated hyperthermic perfusion of the liver was performed for 45 min in 27 pigs via hepatic artery and portal vein at mean inflow temperatures between 40.7 and 41.2 degrees C. In two study groups B and C (n = 9 pigs each) 50 microg recombinant human tumor necrosis factor-alpha (rhTNFalpha) per kg body weight were added to the perfusate, whereas in a control group A liver perfusion was done without rhTNFalpha. Before reperfusion the livers were washed out with Ringer's solution in all groups followed by a protein solution in group C. At 30 and 60 min after reperfusion the maximum systemic rhTNFalpha concentrations were significantly higher in group B with 68 and 61 ng/ml compared to 14.5 and 14.9 ng/ml in group C (p < 0. 05). Mean systemic porcine TNFalpha concentration was significantly higher in group B (217 pg/ml) compared to group C (50 pg/ml) 30 min after reperfusion (p = 0.012). Survival was 7/9 in group A and C and only 2/9 in group B with 6/7 pigs dying due to severe cardiopulmonary failure within 12 h after operation. In surviving pigs of group A and C only mild and transient hepatotoxicity was registered. The presented study underlines the feasibility of high dose rhTNFalpha application in an isolated hyperthermic liver perfusion system. Washout of the liver with a protein solution before reperfusion reduces systemic TNFalpha levels as well as associated lethal cardiocirculatory and hepatotoxic side effects.     

A Hodgkin cell-specific antigen is expressed on a subset of auto- and alloactivated T (helper) lymphoblasts

A Hodgkin cell-specific antigen detected by the monoclonal antibody Ki- 1 was found on T helper lymphocytes after activation by autologous and allogeneic stimulator cells. About 50% of lymphoblasts generated by auto- and alloactivation reacted with the antibody. In contrast, only less than 6% of lymphoblasts stimulated with Con-A, phytohemagglutinin (PHA), or protein A, and none of lymphoblasts activated by oxidative mitogenesis, expressed this antigen. Among several permanent cell lines tested, the K562, MOLT-4, HL-60, and EBV transformed B lymphoblastoid cells reacted with the Ki-1 antibody. The results may indicate possible relationships between the autoreactive subset of T lymphocytes and the pathogenesis of Hodgkin's disease.     

To tell or not to tell – what to do about p.C282Y heterozygotes identified by HFE screening

Hereditary hemochromatosis (HH) is a common preventable disorder of iron overload that can result in liver cirrhosis and reduced lifespan. Most HH is due to homozygosity for the HFE p.C282Y substitution. We conducted a study of screening for p.C282Y in high schools where p.C282Y heterozygotes (CY) individuals were informed of their genotype by letter. We studied whether these individuals understood the implications of their genotype, whether this resulted in anxiety or reduced health perception and whether cascade testing was higher in families of CY than wild‐type homozygous (CC) individuals. We found 586 of 5757 (1 in 10) screened individuals were CY. One month after receiving their result, 83% correctly answered that they have one copy of p.C282Y. There was no adverse change in anxiety or health perception from prior to screening to 1 month after receiving results. Significantly more family members of CY individuals than CC individuals were informed about HH and had testing for HH. In conclusion, we found that informing CY individuals of their genotype does not increase anxiety and the implications are generally well understood. This leads to cascade testing in a minority of families. CY individuals should be informed of their genetic status when identified by population screening.