Instead of dietary modification, surgical management is considered for correcting growth retardation, poor metabolic control, and hepatocellular adenoma (HCA) in glycogen storage disease (GSD) type I.The records of 55 GSD type I patients were retrospectively reviewed. Thirty-two patients underwent only dietary management (group D) and 23 underwent surgical management (group S). In group S, 17 underwent portacaval shunting (PCS), 13 underwent liver transplantation (LT; 7 underwent both PCS and LT). Height-for-age and body mass index-for-age Z-scores based on World Health Organization data were used to compare growth patterns before and after surgery. Changes in metabolic abnormalities and HCA after operation were also investigated.Height-for-age Z-scores for group S were higher by an average of 0.377 compared to that for group D. Metabolic abnormalities often disappeared after LT but improved partially after PCS. De novo HCA was detected in 4 patients (13%) from group D, 12 (100%) who underwent PCS, and none who underwent LT. One case of hepatocellular carcinoma and one of hemorrhage from a HCA were noted in group D. Two cases of hepatocellular carcinoma, 2 of hemorrhage, and 1 of necrosis were noted after PCS.Surgery yielded greater growth improvement than dietary management. However, after PCS, metabolic abnormalities remained unresolved, and the de novo HCA rate was high. Portacaval shunting can be used to improve growth in GSD type I patients when LT is not possible, but close observation for metabolic abnormalities and HCA is essential.
The main limitation of liver transplantation is the shortage of organ donors. The scarcity of donor has led to the increased use of marginal grafts. An injured liver, especially lacerated, is generally considered as the marginal graft having high risk and poor outcome because it is susceptible to malfunctioning, bleeding, bile leakage and infection after liver transplantation. However, some authors have reported successful liver transplantation using injured graft under the meticulous care and careful selection of recipient. We report a case of successful liver transplantation using a right liver graft after in situ left hepatectomy with a lacerated graft with hyperbilirubinemia. Recipient was 52 years old male patient with alcoholic liver cirrhosis. He admitted due to esophageal variceal bleeding with a MELD score of 27 and Child Pugh score of 11. The donor was a 23 years old male who had multiple injuries from fall down. Emergency laparotomy was performed for intraabdominal bleeding. But his condition was deteriorated to brain death and liver function test revealed high bilirubin level (6.04mg/dL) and elevated AST/ALT levels (213/224U/L). The graft liver has 5cm vertical linear laceration in the inferior surface of the liver and it was sutured by Nylon 1‐0 in the previous laparotomy. Procurement team decided to perform in situ left hemihepatectomy to discard an injured left liver because bile duct ligation or injury during previous emergency operation was suspected due to hyperbilirubinemia. After liver transplantation using a right graft, liver function was rapidly normalized and he discharged without any major complication.
Donor against recipient one-way Human leukocyte antigen (HLA) mismatch (D → R one-way HLA MM) seemed strongly associated with graft-versus-host disease (GVHD). The aim of this study is to investigate the relevance of D → R one-way HLA MM in outcome of liver transplantation (LT). We retrospectively analyzed 2670 patients in Korean Organ Transplantation Registry database between April 2014 and December 2020. The patients were categorized into two groups whether D → R one-way HLA MM or not and evaluated the outcomes of LT between the two groups. 18 patients were found to be D → R one-way HLA MM. The incidence of GVHD (0.3% vs. 22.2%, p < 0.001) and mortality rate (11.6% vs. 38.9%, p = 0.003) was much higher in D → R one-way HLA MM group. D → R one-way HLA MM at 3 loci was seemed to be strongly associated with the incidence of GVHD (OR 163.3, p < 0.001), and found to be the strongest risk factor for patient death (HR 12.75, p < 0.001). Patients with D → R one-way HLA MM at 3 loci showed significantly lower overall survival (p < 0.001) but there were no significant differences in rejection-free survival and death-censored graft survival. D → R one-way HLA MM at 3 loci not only affects the overall survival of LT patients but also the incidence of GVHD.
Objective. The total relative value unit (TRVU) of surgery reflects surgical complexity. However, its impact on mortality after noncardiac surgery has not been identified. This study aimed to investigate the association of TRVUs for surgery with postoperative 90-day mortality in adult patients who received planned, elective noncardiac surgery. We hypothesized that higher TRVU was associated with an increase in 90-day mortality after noncardiac surgery. Method. This retrospective cohort study analyzed medical records of adult patients admitted to a single tertiary academic hospital between January 2012 and December 2018 for planned elective noncardiac surgery. The primary end point was 90-day mortality. Results. A total of 112 606 patients were included. Among them, 561 patients (.5%) exhibited mortality within 90-days. In the multivariable model, an increase of 10 000 points of TRVUs was not significantly associated with 90-day mortality (odds ratio: .98, 95% confidence interval: .93 to 1.04; P = .536). Additionally, when it was divided into 4 quartile groups (Q1, Q2, Q3, and Q4), Q2, Q3, and Q4 group of TRVUs were not associated with 90-day mortality compared to the Q1 group of TRVUs (P = .058, .984, and .237, respectively). In receiver-operating characteristic analysis, the area under the curve of TRVUs for a 90-day mortality rate was .61. Conclusions. In conclusion, TRVUs were not associated significantly with a 90-day mortality rate after noncardiac surgery and have a low predictive ability for 90-day mortality after noncardiac surgery alone.
This study aimed to classify the anatomical types of biliary strictures, including intrahepatic biliary stricture (IHBS), after living donor liver transplantations (LDLTs) using right liver grafts and evaluate their prognosis. Among 692 adult patients who underwent right liver LDLT, 198 recipients with biliary strictures (28.6%) were retrospectively reviewed. Based on data obtained during the first cholangiography, the patients' biliary strictures were classified into the following three types according to the levels and number of branches involved: Types 1 (anastomosis), 2 (second‐order branch [a, one; b, two or more; c, extended to the third‐order branch]), and 3 (whole graft [a, multifocal strictures; b, diffuse necrosis]). IHBS was defined as a nonanastomotic stricture. Among the 198 recipients with biliary strictures, the IHBS incidence rates were 38.4% ( n = 76). The most common type of IHBS was 2c ( n = 43, 56.6%), whereas Type 3 ( n = 10, 13.2%) was uncommon. The intervention frequency per year significantly differed among the types (Type 1, 2.3; Type 2a, 2.3; Type 2b, 2.8; Type 2c, 4.3; and Type 3, 7.2; p < 0.001). The intervention‐free period for more than 1 year, which was as follows, also differed among the types: Type 1, 84.4%; Type 2a, 87.5%; Type 2b, 86.7%; Type 2c, 72.1%; and Type 3, 50.0% ( p = 0.048). The graft survival rates of Type 3 (80.0%) were significantly lower than those of the other types ( p = 0.001). IHBSs are relatively common in right liver LDLTs. Although Type 3 IHBSs are rare, they require more intensive care and are associated with poorer graft survival rates than anastomosis strictures and Type 2 IHBS.
Abstract Antithrombin‐III (AT‐III) concentrates have been used in the immediate postoperative period after liver transplantation to prevent critical thrombosis. We aimed to investigate a more appropriate method for AT‐III concentrate administration to maintain plasma AT‐III activity level within the target range. In this randomized controlled trial, 130 adult patients undergoing living‐donor liver transplantation were randomized to either the intermittent group or continuous group. In the intermittent group, 500 international units (IU) of AT‐III concentrate were administered after liver transplantation and repeated every 6 h for 72 h. In the continuous group, 3000 IU of AT‐III were continuously infused for 71 h after a loading dose of 2000 IU over 1 h. Plasma AT‐III activity level was measured at 12, 24, 48, 72, and 84 h from the first AT‐III administration. The primary outcome was the target (80%–120%) attainment rate at 72 h. Target attainment rates at other timepoints and associated complications were collected as secondary outcomes. A total of 107 patients were included in the analysis. The target attainment rates at 72 h post‐dose were 30% and 62% in the intermittent group and continuous group, respectively ( p = 0.003). Compared to the intermittent group, patients in the continuous group reached the target level more rapidly (12 vs. 24 h, median time, p < 0.001) and were more likely to remain in the target range until 84 h. For maintaining the target plasma AT‐III activity level after living‐donor liver transplantation, continuous infusion of AT‐III seemed to be more appropriate compared to the conventional intermittent infusion regimen.
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