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Biomedical subjects

S Uemoto

Publications and source records attributed to S Uemoto.

At least 73 records · Page 4Linked to original sources

One-way donor-recipient HLA-matching as a risk factor for graft-versus-host disease in living-related liver transplantation.

Although one-way matching between an HLA-homozygous donor and a haploidentical recipient is a recognized risk factor in transfusion-associated graft-versus-host disease (GVHD), its impact in living-related liver transplantation (LRLT) has so far not been investigated. We present a case of fatal acute GVHD in our LRLT program that was attributed to one-way HLA matching between donor and recipient. Although the disappearance of donor cells in peripheral blood was suggested by genetic analysis, severe septicemia led to a fatal outcome. We further reviewed 280 LRLT cases and correlated one-way HLA matching with outcome. A total of 8 out of 280 donors (2.9%) and 11 out of 278 recipients (4.0%) were completely HLA homozygous in our LRLT program. Complete one-way HLA matching linked to GVHD was observed in four cases, including the present case. Although other contributing factors also need to be clarified, one-way HLA matching is a definite risk factor for GVHD in LRLT. We advocate caution before proceeding with one-way HLA donor-recipient combinations.

Fatal Outcome↗

Hemolytic reaction due to graft-versus-host (GVH) antibody production after liver transplantation from living donors: report of two cases.

Among 27 patients who received minor ABO-incompatible partial liver transplantations and 19 who received major ABO-incompatible partial liver transplantations from living donors, 2 developed hemolytic anemia within 2 weeks after transplantation. These 2 patients had received livers from their living fathers whose blood type was ABO-incompatible. B-to-A transplantation was performed in patient 1 and O-to-B transplantation was performed in patient 2. Anti-A IgM and IgG were detected in the serum of patient 1, and anti-B IgM and IgG were detected in the serum of patient 2. These antibodies were eluted from the red blood cells of the patients. The coexistence of donor-specific DNA in the peripheral blood of the patients proved that they had chimerism, and graft-versus-host antibody production due to passenger B lymphocytes in the donor's liver was subsequently confirmed.

ABO Blood-Group System↗

Treatment of ornithine transcarbamylase deficiency in girls by auxiliary liver transplantation: conceptual changes in a living-donor program.

BACKGROUND/PURPOSE: Ornithine transcarbamylase (OTC) deficiency is an X-chromosome-linked genetic disorder resulting in hyperammonemia hepatic dysfunction, coma, and serious neurological sequelae. This report describes an experience in treating this condition with living-related liver transplantation. METHODS: Three children with OTC were treated with a living-related liver transplantation. Potential donors were evaluated with the allopurinol loading test to exclude heterozygotes. FK506 and low-dose steroids were used for immunosuppression. Auxiliary partial orthotopic transplantation (APOLT) was used in two of the cases. RESULTS: All three children survived and are doing well without protein restriction. CONCLUSIONS: These observations suggest that in OTC removal of the native liver is not necessary because (1) a partial liver segment with normal enzyme activity corrects the hyperammonemia, (2) in case of graft failure the native liver is an available backup, and (3) the native liver has the ability to recover function. As long as living-related donors serve as the main source of donor organs, APOLT is a safer approach to liver transplantation in OTC.

Amino Acid Metabolism, Inborn Errors↗

Systemic cytokine response to hepatic resections under total vascular exclusion.

OBJECTIVE: To investigate the systemic cytokine response to major liver surgery as the basis for assessing potential new treatments. DESIGN: Open prospective study. SETTING: University hospital, UK. SUBJECTS: Thirteen patients undergoing elective hepatic resections that involved total vascular exclusion of the liver. INTERVENTIONS: Blood samples were taken preoperatively, during the operation, and during the first four postoperative days. Concentrations of endotoxin, interferon gamma (IFN-gamma), tumour necrosis factor alpha (TNFalpha), interleukin-1 (IL-1), and interleukin-6 (IL-6) were measured. RESULTS: Endotoxin concentrations were raised in 3/13 patients before operation and in 6 patients during the postoperative period. TNFalpha concentrations were undetectable. IFN-gamma and IL-1 responses followed a low and inconclusive pattern. IL-6 was significantly increased from 6 hours after operation to the third postoperative day, peaking at 699 (+/-277) pg/ml at 24 hours (p < 0.01). The two patients who died had the highest postoperative concentrations of IL-6. CONCLUSIONS: There is a pronounced systemic response to hepatic resection under total vascular exclusion that is reflected by the increase in IL-6 concentration and correlates with the operative blood loss and postoperative outcome. This might be used as an indicator of the response to specific treatments in this type of surgery. Treatments that minimise the IL-6 response to major hepatic resection may be of value.

APACHE↗

Portal vein reconstruction in pediatric liver transplantation from living donors.

OBJECTIVE: The authors analyze the surgical pattern and the underlying rationale for the use of different types of portal vein reconstruction in 110 pediatric patients who underwent partial liver transplantation from living parental donors. SUMMARY BACKGROUND DATA: In partial liver transplantation, standard end-to-end portal vein anastomosis is often difficult because of either size mismatch between the graft and the recipient portal vein or impaired vein quality of the recipient. Alternative surgical anastomosis techniques are necessary. METHODS: In 110 patients age 3 months to 17 years, four different types of portal vein reconstruction were performed. The portal vein of the liver graft was anastomosed end to end (type I); to the branch patch of the left and right portal vein of the recipient (type II); to the confluence of the recipient superior mesenteric vein and the splenic vein (type III); and to a vein graft interposed between the confluence and the liver graft (type IV). Reconstruction patterns were evaluated by their frequency of use among different age groups of recipients, postoperative portal vein blood flow, and postoperative complication rate. RESULTS: The portal vein of the liver graft was anastomosed by reconstruction type I in 32%, II in 24%, III in 14%, and IV 29% of the cases. In children <1 year of age, type I could be performed in only 17% of the cases, whereas 37% received type IV reconstruction. Postoperative Doppler ultrasound (mL/min/100 g liver) showed significantly (p < 0.05) lower portal blood flow after type II (76.6 +/- 8.4) versus type I (110 +/- 14.3), type III (88 +/- 18), and type IV (105 +/- 19.5). Portal vein thrombosis occurred in two cases after type II and in one case after type IV anastomosis. Portal stenosis was encountered in one case after type I reconstruction. Pathologic changes of the recipient native portal vein were found in 27 of 35 investigated cases. CONCLUSION: In living related partial liver transplantation, portal vein anastomosis to the confluence with or without the use of vein grafts is the optimal alternative to end-to-end reconstruction, especially in small children.

Adolescent↗

Liver transplantation in a case of hypoproteinemia and coagulopathy.

A female infant with hypoproteinemia and coagulopathy associated with hypertyrosinemia was successfully treated with living-related liver transplantation (LRLT). On the 12th day of life plasma amino acid analysis revealed a marked elevation of tyrosine, so the patient was fed on a low-tyrosine and low-phenylalanine diet. However, hepatosplenomegaly, hypotonia, alopecia, eczema and psychomotor delay did not improve and recurrent episodes of disseminated intravascular coagulation (DIC) caused her condition to deteriorate. Liver biopsy on the 230th day revealed marked fatty change accompanied by mild to moderate cholestasis. Therefore, LRLT from her father was performed on the 286th day resulting in improvement of all the aforementioned signs and symptoms. Despite a thorough examination, no diagnosis of a known disorder could be established. However, her elder brother had also been born with severe hypoproteinemia and coagulopathy, and died of DIC on the second day of life. Thus, the disorder is designated as a new entity, namely 'congenital hypoproteinemia and coagulopathy associated with hypertyrosinemia'.

Disseminated Intravascular Coagulation↗

Living-related small bowel transplantation: the first case in Japan.

Intestinal failure has been managed with total parenteral nutrition (TPN), but occasionally complications such as obliteration of venous access or liver dysfunction occur. To overcome such complications, small bowel transplantation (SBT) was introduced. Since the introduction of tacrolimus in 1990, successful SBT cases have been reported. We performed SBT by using a living donor for a child with short bowel syndrome. The recipient (2.5 years old, male) was born with intestinal necrosis secondary to midgut volvulus. The length of the remaining small bowel was 30 cm. While being managed with TPN, his venous access gradually obliterated. Long-term survival could not be expected because of the difficulty in securing TPN access. The donor was his mother, whose distal ileum (100 cm) was used as a graft. The immunosuppression regimen consisted of tacrolimus, steroids and azathioprine. Three episodes of severe rejection and subsequent episodes of viral (EBV, CMV) infection were managed with steroid pulse therapy and antiviral drugs, respectively. The recipient suffered from anastomotic stenosis, and an operation was performed 13 months after transplantation to resect the stenotic segment. However, the patient died of Pneumocystis carinii pneumonia 16 months after transplantation. We conclude that organ retrieval from a living donor can be performed safely for SBT, but further study of the management of rejection as well as of viral infection is necessary, as it is for non-living-related SBT.

Adult↗

Growth and quality of life after living-related liver transplantation in children.

Fifty-six consecutive pediatric recipients surviving more than 3 yr after living-related liver transplantation (LRLT) were evaluated in terms of growth, quality of life (QOL) and need for maintenance immunosuppression. Significant improvement in Z-score for height and weight were observed at last follow-up, ranging from 3 to 6 yr after transplantation, although catchup height gain lagged behind recovery in weight (height: -1.77 pre-transplant to -0.77 post-transplant, p<0.001; weight: -1.12 pre-transplant to -0.18 post-transplant, p<0.0001). 82% (46) recipients have remained in good health and have an excellent QOL as assessed in the most recent 6 months; these children lead similar daily lives to normal healthy children, with daily school attendance and full participation in activities including gymnastics and hiking. 3.6% (2) recipients attended school regularly but were unable to participate in sporting activities. 14% (8) recipients remain home or hospital-bound due to persistent complications in the past 6 months, with only minimal school attendance. Less than 10% of recipients were taking steroids by 2 yr post-transplantation, although approximately half of the children were receiving low-dose maintenance steroids at 1 yr. The mainstay immunosuppressant was tacrolimus, with 68% (38) recipients receiving daily therapy, 8.9% (5) alternate-day, 8.9% (5) twice a week, and 5.4% (3) a single dose weekly or alternate weeks. 7.1% (4) recipients were withdrawn completely from all immunosuppressants, including tacrolimus, for various reasons. 8.9% (5) patients have needed multiple immunosuppressive agents over the last 6 months. In conclusion, LRLT restores growth and offers excellent quality of life in pediatric recipients. The majority of recipients require minimal, steroid-free, immunosuppression by 2 yr post-transplant, but the occasional recipient still needs intensive longterm immunosuppression.

Adolescent↗

Direct measurement of graft and recipient liver fossa size by computed tomography for avoiding problems due [correction of clue] to large graft size in living-related liver transplantation.

We investigated the incidence and manifestation of problems associated with large graft size in living-related liver transplantations and assessed the usefulness of determining volume and dimensions of the graft and recipient's liver fossa by computed tomography to indicate the risk. Five of 150 living related liver transplantations had grafts that were too large, resulting in difficulty in primary abdominal closure or in sudden worsening of hemodynamics during primary closure. No significant difference existed in recipient age, sex, body weight, selection of the graft segment, hepatic vein reconstruction, recipient resected liver weight, graft volumetry value, the ratio of body weight of donor relative to recipient, and the percentage of graft weight relative to recipient body weight, between the groups with and without these problems. Mean +/- SEM of maximal dimensional ratio, defined as the maximum of the ratios of 3 dimensions of the graft relative to recipient liver fossa, were 2.36 +/- 0.64 for patients with grafts that were too large and 1.00 +/- 0.02 for the cases without size problems. The mean +/- SEM of liver fossa index, defined as the product of 3 dimensions of recipient liver fossa, were (25.03 +/- 7.18) x 10(4) mm3 and (127.54 +/- 5.07) x 10(4) mm3, respectively. These two indices clearly indicated the risk of problems due to large graft size, and will help to protect recipients and provide a basis for evaluating graft size in reductions.

Child, Preschool↗

Application of in situ hybridization technique for quantitative assessment of ongoing symptomatic Epstein-Barr virus infection after living related liver transplantation.

For quantitative assessment of ongoing symptomatic Epstein-Barr virus (EBV) infection in pediatric recipients of liver transplantation, we determined the number of peripheral blood mononuclear cells (PBMC) infected by EBV by in situ hybridization (ISH) and related the results with clinical courses of those patients. Twenty-four patients had symptomatic EBV infection between February 1995 and March 1996. Blood samples were obtained from these 24 patients at the time of acute phase, from 13 of them during convalescence, and 37 pediatric patients before transplantation. ISH was performed on the PBMC and polymerase chain reaction (PCR) on DNA from whole blood. Oligonucleotide probes for ISH were chosen from coding sequences of EBV-encoded small nuclear RNA 1 (EBER1). Results of ISH were reported in a number of cells expressing EBER1/5 x 104 PBMC (#EBER1). Fever, diarrhea, upper respiratory symptoms, pleural effusion, ascites, lymphadenopathy, and lymphoproliferative disease (LPD) accompanied with EBV infection proven by serology, viral-specific stain or PCR were regarded as EBV related diseases (EBVD). All samples with positive #EBER1 were accompanied by positive EBV PCR. #EBERI was 68.2 +/- 144.9 (mean +/- SD) ranging from 0 to 621 in the acute phase, 0.20 +/- 0.41 ranging from 0 to 2 in the convalescence phase, 0.27 +/- 0.77 in 23 preoperative patients with positive serology, and 0 in all 14 preoperative patients with negative serology. The #EBER1 in ongoing EBVD was significantly greater than that of patients in convalescence or before transplantation. Patients with #EBERI greater than 10 had a significantly lower chance of convalescence and a higher mortality than patients with #EBER 1 less than 10. We conclude that #EBER1 could be a specific and quantitative marker of EBVD and might predict progression to LPD.

Child↗

Biliary complications in pediatric living related liver transplantation.

BACKGROUND: The goal of this study was to evaluate cause and outcome of biliary complications occurring after pediatric living related liver transplantation (LRLT). METHODS: A database of 205 pediatric patients (71 male and 134 female) undergoing 208 LRLT from June 1990 to April 1996 was reviewed. RESULTS: The overall incidence of bile duct complications was 13.9% (29 patients). There were 19 bile leaks, 7 anastomotic strictures, 8 intrahepatic biliary complications, and the bile duct was ligated inadvertently in 2 cases. Logistic regression analysis revealed hepatic artery thrombosis, ABO incompatible transplantation, intrapulmonary shunting in recipients, mode of artery reconstruction, and cytomegalovirus infection were all significant risk factors for biliary complications. CONCLUSIONS: Avoidance of ABO incompatible transplantation where possible, routine use of microvascular techniques for hepatic artery reconstruction to minimize the risk of artery thrombosis, earlier transplantation for patients with intrapulmonary shunt, and prophylaxis against cytomegalovirus infection should all reduce the rate of biliary complications after LRLT in pediatric recipients.

ABO Blood-Group System↗