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Hyperbilirubinemia in the early phase after allogeneic hematopoietic SCT (HSCT) is due to various causes. One of the most important causes of hyperbilirubinemia is veno-occlusive disease/sinusoidal obstructive syndrome (VOD/SOS). However, the prognosis of patients who are clinically diagnosed as SOS varies. We retrospectively evaluated 82 patients who underwent their first allogeneic HSCT. GVHD prophylaxis was a combination of short-term MTX and CsA (n=77) or tacrolimus (n=5). Thirty-three patients developed hyperbilirubinemia, with a bilirubin level of at least 2 mg/dL, within 20 days after HSCT. Of these patients, 24 were diagnosed as VOD/SOS using the modified Seattle criteria. Twenty-six recovered to a bilirubin level of <2 mg/dL. We focused on the serum alkaline phosphatase/total bilirubin ratio (ALP/TB) at the onset of hyperbilirubinemia and found that it significantly predicted the recovery from hyperbilirubinemia. OS was significantly higher in patients with a lower ALP/TB ratio (P=0.00056). In addition, a lower ALP/TB ratio was associated with better survival even in patients who were clinically diagnosed as SOS (P<0.001). The ALP/TB ratio at the onset of hyperbilirubinemia may be a useful predictor for the prognosis of hyperbilirubinemia and SOS early after HSCT.

Background/aims: A high incidence of hyperbilirubinemia and an increased incidence of gallbladder disorders after esophagectomy have been reported. Moreover, several studies have documented an increased incidence of gallbladder disease in patients receiving long-term total parenteral nutrition. We studied the incidence of cholecystitis and cholestasis and hyperbilirubinemia associated with total parenteral nutrition after esophagectomy., Methodology: We retrospectively studied 42 patients who underwent esophagectomy. These patients were divided into 2 groups: the hyperbilirubinemia group and the non-hyperbilirubinemia group. The incidence of cholecystitis or cholestasis after the surgery was compared between the 2 groups., Results: The mean total serum bilirubin level of the hyperbilirubinemia group (2.40±0.35mg/dL) was significantly higher than that of the non-hyperbilirubinemia group (1.20±0.34mg/dL; p<0.0001). No significant differences were observed between the 2 groups with respect to the mean duration for which total parenteral nutrition was required around the time of the operation (i.e. pre- and postoperatively) and the incidence rate of cholecystitis or cholestasis after esophagectomy., Conclusions: Hyperbilirubinemia after esophagectomy was frequently observed; however, it may not contribute to gallbladder problems. We suggest that parenteral modalities such as tube feeding should be initiated soon after surgery to prevent gallbladder problems after esophagectomy.

Background: To further improve the outcomes of liver resection, it is important to identify and prevent the causes of the hyperbilirubinemia occurring after hepatectomy and postoperative liver failure., Methods: Between 2004 and 2009, 591 consecutive patients underwent a hepatectomy at our center. Twenty-two patients who developed hyperbilirubinemia (postoperative total bilirubin over 5 mg/dL) after hepatectomy were classified as Hi-Bi group and another 569 whose total bilirubin did not increase beyond 5 mg/dL were classified as non-Hi-Bi group., Results: A preoperative prothrombin test of less than 80% and a blood loss of more than 1000 mL were identified as independent risk factors for the Hi-Bi group by multivariate analysis. The hyperbilirubinemia of 16 cases improved, while that of 6 cases was prolonged. One of these patients died of liver failure without responding to treatment. The mortality rate for postoperative liver failure in this study was 0.16% (1/591)., Conclusion: It is important to reduce the length of surgery and intraoperative blood loss to prevent hyperbilirubinemia after hepatectomy. Additionally, decision-making using our algorithm and full examination of the accurate evaluation results, including those for prothrombin time, residual liver function and liver damage, can help reduce the development of hyperbilirubinemia.

To prevent small-for-size syndrome in adult-to-adult living donor liver transplantation (A-LDLT), larger grafts (ie, right lobe grafts) have been selected in many transplant centers. However, some centers are investigating the benefits of portal pressure modulation. Five hundred sixty-six A-LDLT procedures using right or left lobe grafts were performed between 1998 and 2008. In 2006, we introduced intentional portal pressure control, and we changed the graft selection criteria to include a graft/recipient weight ratio >0.7% instead of the original value of >0.8%. All recipients were divided into period I (1998-2006, the era of unintentional portal pressure control; n = 432) and period II (2006-2008, the era of intentional portal pressure control; n = 134). The selection of small-for-size grafts increased from 7.8% to 23.9%, and the selection of left lobe grafts increased from 4.9% to 32.1%. Despite the increase in the number of smaller grafts in period II, 1-year patient survival was significantly improved (87.9% versus 76.2%). In 129 recipients in period II, portal pressure was monitored. Patients with a portal pressure <15 mm Hg demonstrated better 2-year survival (n = 86, 93.0%) than patients with a portal pressure >or=15 mm Hg (n = 43, 66.3%). The recovery from hyperbilirubinemia and coagulopathy after transplantation was significantly better in patients with a portal pressure <15 mm Hg. In conclusion, our strategy for A-LDLT has changed from larger graft-based A-LDLT to controlled portal pressure-based A-LDLT with smaller grafts. A portal pressure <15 mm Hg seems to be a key for successful A-LDLT., ((c) 2010 AASLD.)

Objective: Lipoprotein metabolism in FIC1 deficiency due to ATP8B1 mutations has never been studied sufficiently. This study was performed to investigate the detailed lipoprotein metabolism in benign recurrent intrahepatic cholestasis (BRIC) caused by FIC1 deficiency., Patients and Methods: Lipoprotein profile and major lipoprotein regulators such as lecithin:cholesterol acyltransferase (LCAT), hepatic triglyceride lipase (HTGL), lipoprotein lipase, and cholesteryl ester transfer protein in a Japanese patient with BRIC were serially examined during a bout of cholestasis. Liver expression of farnesoid X receptor (FXR), which suppresses high-density lipoprotein (HDL) generation, was also examined., Results: Hypercholesterolemia and lipoprotein X accumulation were never observed throughout this study. When the cholestasis was severe, triglyceride-rich low-density lipoprotein (LDL) accounted for most of the plasma lipoproteins whereas HDL was hardly detectable. Concurrently, activities of all regulators were decreased, together with decreases of the serum parameter for liver protein synthesis. In particular, suppressions of LCAT and HTGL activities were severe and greatly contributed to the appearance of triglyceride-rich LDL. As the cholestasis improved, this LDL gradually transformed into normal LDL with the recoveries of LCAT and HTGL activities. The activities of all regulators for the last 1 to 2 months were normal but HDL remained depleted. His liver showed low FXR expression compared with control livers., Conclusions: The present study showed an appearance of triglyceride-rich LDL due to suppressions of LCAT and HTGL activities and a depletion of HDL that is not able to be explained by lipoprotein regulators or FXR in our patient.

Background: Hyperbilirubinaemia is a common occurrence in patients who are admitted to intensive care units (ICUs) after major surgery, and it is associated with high mortality. We investigated the incidence of hyperbilirubinaemia after two major types of thoracic surgery: open-heart surgery and oesophagectomy. In order to identify the risk factors associated with hyperbilirubinaemia after major surgery, we compared the incidence after open-heart surgery with that after oesophagectomy., Results: Hyperbilirubinaemia was detected in 51% of the open-heart surgery patients (n = 133) and in 64% in the oesophagectomy group (n = 74). The incidence of hyperbilirubinaemia was significantly related to the duration of surgery (P< 0.05). In the open-heart surgery group, duration of surgery was 465 +/- 24 min for the patients without hyperbilirubinaemia and 571 +/- 26 min for the patients with hyperbilirubinaemia. In the oesophagectomy group, the procedure durations were 415 +/- 17 min and 493 +/- 20 min, respectively. The overall mortality rate was 8% in the open-heart surgery group; the rate was 12% in those with hyperbilirubinaemia, but 5% in those without hyperbilirubinaemia. No members of the oesophagectomy group died, with or without hyperbilirubinaemia. Infection significantly affected both the occurrence of hyperbilirubinaemia and mortality in the open-heart surgery group. In the subgroups from the open-heart surgery group, 5% (three out of 65) of those without hyperbilirubinaemia (or evidence of infection) died; of the patients with hyperbilirubinaemia, 3% (one out of 38) of those without infection died and 23% (seven out of 30) with detected infection died., Conclusion: After open-heart surgery and oesophagectomy, approximately half of the patients studied had higher levels of serum total bilirubin. Time spent in surgery was significantly related to the occurrence of hyperbilirubinaemia. Infection significantly affected mortality and total bilirubin levels after open-heart surgery. Control of infection plays a crucial role in the prevention of hyperbilirubinaemia and in reducing mortality.