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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Liver transplantation has become a clinical therapeutic modality for end stage liver diseases. The results achieved in children are better than in adults: in T.E. Starzl unique experience in Pittsburgh, USA, the survival rate at one and four years are 75 and 70% respectively. Complete rehabilitation of these children can nowadays be expected. Between March 1984 and June 1985, 8 children received an orthotopic liver transplantation at the University of Louvain Medical School in Brussels, Belgium; one child received two transplantations after acute and irreversible rejection of a first ABO incompatible graft. The indications were biliary atresia in five (polysplenia in one), biliary hypoplasia in one, alpha-1-antitrypsine deficiency in one and Crigler-Najjar syndrome type I in one. The age of the patients at the time of liver replacement was 12 to 18 months in four, 8 to 13 years in four. Six patients are alive after 17, 14, 12, 10, 3 and 3 months; the two youngest children deceased during the first postoperative month. The Kaplan-Meyer one year survival rate is 75%; all surviving children are in excellent clinical condition with a normal liver function. The 9 transplanted livers were harvested from multiorgan cerebral death donors with the exception of one neonate whose liver alone was removed; 4 were retrieved locally, the five others were offered by foreign hospitals through the organ procurement agencies (Eurotransplant, France-Transplant, U.K. Transplant). Due to appropriate logistics with air flight transportation of the harvesting team when indicated, the total ischaemia time was kept below 6 hours in every case. Two small children underwent a left lobe orthotopic transplantation after ex vivo right trisegmentectomy of the liver retrieved from an older donor with one long term survival. The indications for liver transplantation in children are end-stage liver diseases consisting of a) cholestatic diseases among which the most frequent is biliary atresia after unsuccessful Kasai procedure followed by familial cholestasis (Byler syndrome) and the paucity of the intrahepatic bile ducts of the syndromatic (Alagille syndrome) or non syndromatic type. b) the metabolic diseases resulting either in cirrhosis with liver failure (alpha-1-antitrypsin deficiency, Wilson disease, glycogen storage disease type I and IV, protoporphyria) or in extrahepatic complications of enzymatic deficiency of an otherwise normally functioning liver (Crigler-Najjar syndrome type I, familial hypercholesterolemia and perhaps oxalosis). c) the hepatocellular diseases either chronic with cirrhosis of various origin or acute, eg. toxic hepatitis.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Liver transplantation in children]. 391 72

Plasma exchange is an effective technique for removal of antibodies prior to ABO incompatible marrow grafting. However, expense and hepatitis risk make alternative methods desirable. A solid phase immunoadsorbent column using blood group A trisaccharide has been demonstrated to specifically remove anti-A antibody from human plasma in vitro and immunized dogs in vivo with no toxicity. Preliminary results in patients are encouraging.
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PMID:Immune adsorption of anti-A and anti-B antibodies. 704 29

Potential transfusion reactions include hemolysis, disease transmission (particularly hepatitis), allergic and febrile reactions, and symptoms of circulatory overload. Limiting the number of transfusions given to patients for whom the procedure will achieve some clearly defined clinical goal is one way of reducing the number of adverse reactions. When transfusion is to be carried out, great care should be taken in correctly identifying both patient and blood to avoid ABO mix-ups, and thorough pretransfusion laboratory testing should be done. During and after transfusion the patient should be closely observed for complications.
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PMID:Untoward effects of blood transfusion: common problems and simple safeguards. 745 51

We reviewed 37 living related liver transplantations (LRLT) performed by our department during the last 27 months on children with end-stage liver disease. The patients were 15 boys and 22 girls aged 7 months to 15 years with biliary atresia (27), cryptogenic cirrhosis (3), Budd-Chiari syndrome (2), progressive intrahepatic cholestasis (2), protoporphyria (1), Wilson's disease (1), and fulminant hepatitis (1). The donors were 14 fathers and 23 mothers. Grafts were made from the left lateral segment (19), left lateral segment with partial S4 (11), left lobe (6), and right lobe (1). After graft harvesting all donors resumed normal liver function and normal life. The recipient underwent total hepatectomy with preservation of the inferior vena cava. FK506 and low-dose steroids were used for immunosuppression. The survival rate was 90% (27/30) in elective cases and 57% (4/7) in emergency cases. Six recipients had functioning grafts but died of extrahepatic complications. Hepatic vein stenosis occurred in 3 cases at 3 months after LRLT and was successfully treated by balloon dilatation. Portal vein stenosis occurred in 1 case at 8 months after LRLT and was also safely dilated. We incurred no hepatic artery thrombosis after introducing microsurgery techniques. Among 12 viral, 5 bacterial, and 3 fungal postoperative infections, 1 Candida pneumonia and 1 EBV-associated lymphoma were lethal. Three patients with ABO-blood group compatible grafts and one with an incompatible graft developed acute rejection, which was controlled in evey case by steroid bolus and/or increasing the dose of FK506. There were no definite episodes of rejection in ABO-identical cases. Children with moderate growth retardation (> or = -1.5 SD of normal growth) caught up in growth soon after LRLT, but those with severe retardation (<-1.5 SD) were slow to attain age-normal height. Appropriate timing, meticulous surgical procedures, and comprehensive management of complications are crucial for successful outcome with LRLT. LRLT is a promising option for alleviating the shortage of livers for pediatric transplantation and may be regarded as an independent modality to supplement cadaver donation.
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PMID:Living related liver transplantation in children. 751 49

The growth in liver transplantations recorded by the Pitt-UNOS Liver Transplant Registry since October 1987 continues, and in 1993 the rate of increase was greater than it had been in recent years. This is in spite of the fact that the net growth of new centers was smaller in 1993 than in any previous year examined. Pediatric recipients in 1993 were compared with those in previous years, and no significant differences were found for sex, race, or age. In contrast to prior years, the majority of pediatric recipients in 1993 awaited transplantation at home. The most common indication for liver transplantation in children was biliary atresia, although the proportion of recipients with this primary liver disease decreased slightly in 1993. Significant increases were noted in the proportions of pediatric recipients with fulminant liver failure, and hepatoblastoma. Significantly fewer children received ABO-incompatible livers in 1993 compared with prior years, part of which may be a function of the increasing use of living-related donors. Many of the characteristics examined for adult recipients had different distributions in 1993 than in prior years. The proportion of White recipients declined in 1993, due to increases among Black and Hispanic recipients. The mean and median ages of adult recipients continued to increase because of the increasing proportion of recipients aged 60 and over. The proportion of adult recipients awaiting transplantation outside of the hospital continued to increase in 1993. The increase in the proportion of recipients with positive CMV serology is likely due to the increasing age of the recipients in 1993. A smaller proportion of multiorgan transplantations was performed in 1993, due to the elimination of procedures involving only the liver and pancreas. Alcoholic cirrhosis was replaced by hepatitis non-A, non-B, or C as the most common reason for LTX. The proportions of recipients with fulminant liver failure and malignancies, indications for poorest survival, declined significantly in 1993. The cumulative probability of surviving for 6 years after initial transplantation was 0.70 (without retransplantation = 0.58) for pediatric recipients.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Liver transplantation in the United States: results from the National Pitt-UNOS Liver Transplant Registry. United Network for Organ Sharing. 754 39

Neonatal hepatitis is a syndrome of unknown etiology occurring in children with viral liver disease, as well as children with unidentified disorders of bile salt synthesis and other poorly understood metabolic diseases. It is characterized by jaundice, giant cell hepatitis and rare liver failure necessitating liver transplantation. In the present investigation, the outcome of liver transplantation performed in 16 children with neonatal hepatitis at the investigators' institution was determined from 1 January 1989 to 31 December 1991. The results were compared to those obtained in 288 children transplanted for biliary atresia and 66 children transplanted for recognized metabolic liver disease. The children transplanted for neonatal hepatitis (4.1 +/- 1.3 years) and metabolic liver disease (5.8 +/- 0.6 years) were older than those transplanted for biliary atresia (3.3 +/- 0.2 years) (p < 0.01), but did not differ in terms of sex, ABO type, UNOS status or year in which the transplant procedure was performed. Interestingly, first allograft survival was equal in the children with neonatal hepatitis (74%) and those with metabolic liver disease (74%), but was greater than that for children transplanted for biliary atresia (68%) (p < 0.01). Despite this significant difference in first graft survival, no differences in 5-year survival were seen for the three groups (81% for neonatal hepatitis, 68% for biliary atresia and 79% for metabolic liver disease).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Liver transplantation for neonatal hepatitis as compared to the other two leading indications for liver transplantation in children. 769 24

During the last 31 months, 50 children between 3 months and 15 years of age have undergone living related liver transplantation (LRLT) for end-stage liver diseases (39 biliary atresia, 2 Budd-Chiari syndrome, 2 progressive intrahepatic cholestasis, 3 liver cirrhosis, 1 Wilson disease, 1 protoporphyria, 1 tyrosinemia, and 1 fulminant hepatitis). Combined FK-506 and low-dose steroids were routinely used for immunosuppression. There were seven deaths, two of which were related to infection (Candida pneumonia and Epstein-Barr virus [EBV]-associated lymphoproliferative syndrome [LPS]). Five patients had a bacterial infection, all of which were associated with surgical complications. Three patients had Candida infection, all of which were malnourished, had biliary atresia, and had been managed with prolonged antibiotics against obstinate ascending cholangitis. There were 14 symptomatic viral infections (1 herpes simplex virus, 1 herpes zoster virus, 5 cytomegalovirus [CMV], 6 EBV, and 1 EBV-associated LPS). Three of the five CMV infections appeared in patients whose graft was ABO-incompatible, who were managed with prophylactic OKT-3. Most of the viral infections (except 1 EBV-associated LPS) were minor and were treated successfully. The low incidence and successful treatment of CMV infection are related to the high compatibility and low incidence of allograft rejection in LRLT. Bacterial and fungal infections can be decreased by greater refinement of surgical technique and more aggressive preoperative management. Treatment of EBV infection is still an unsolved problem.
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PMID:Infectious complications in living related liver transplantation. 801 5

The safety of steroid withdrawal in orthotopic liver transplant (OLT) recipients has been studied in a prospective trial with a comparison control group. Sixty-four recipients of ABO-compatible grafts (42 adults, 22 children) were randomized into a steroid withdrawal (SW) group and a control group. Inclusion criteria included survival > one year post-OLT and no rejection > six months after OLT. Exclusion criteria included previous graft loss secondary to rejection, > two episodes of documented rejection, patients transplanted for autoimmune hepatitis, and patients unable to receive azathioprine. Target HPLC cyclosporine levels in both groups were 100-200 ng/ml. Thirty-three patients entered the SW group and 31 the control group at a mean of 3.5 years after OLT; follow-ups were 592 and 527 days, respectively. Two patients in each group developed biopsy-proven rejection. In the SW group one patient rejected at three months, the other at nine months. Both rejection episodes resolved with only reinstitution of oral prednisone. Of the two patients who rejected in the control group (one at 7 months, one at 11 months) one required conversion to tacrolimus and the other intravenous steroids. There were no significant differences between the two groups for prednisone, azathioprine, cyclosporine doses, cyclosporine levels, liver function tests, and white blood cell counts at base line compared with 12 months. Fasting serum cholesterol in the SW group decreased from 194 +/- 44 mg/dl at baseline to 175 +/- 37 mg/dl at one year, whereas in the control group cholesterol rose from 180 +/- 48 mg/dl to 193 +/- 44 mg/dl. In pediatric patients no significant difference in age-adjusted height velocities over one year was seen between the two groups. We concluded that dual therapy with cyclosporine and azathioprine in stable long-term liver allograft recipients is not associated with an increase in rejection incidence. Prednisone withdrawal may be associated with an improvement in lipid profiles.
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PMID:A randomized prospective trial of steroid withdrawal after liver transplantation. 854 72

The growth in liver transplantations recorded by the Pitt-UNOS Liver Transplant Registry since October 1987 continues as does the net growth of new centers. Characteristics of pediatric recipients in 1994 were compared to those of previous years and no significant differences were found for gender, race or age. The majority of pediatric recipients in 1994 awaited transplantation at home. The most common indication for liver transplantation in children was bilary atresia, though the proportion of recipients with this primary liver disease decreased significantly. Significant increases were noted in the proportions of pediatric recipients with autoimmune disease (though this remains a relatively uncommon indication) and fulminant liver failure. There was a significant decrease in the proportion of children who received ABO-incompatible livers. Many of the characteristics examined for adult recipients changed over time. The proportion of male recipients continued to increase. The mean age of adult recipients continued to increase, likely contributing to the increased prevalence of positive CMV-serology. The proportion of adult recipients awaiting transplantation outside the hospital increased over time. The increase in the proportion of multiorgan transplantations was in large part due to the increased reporting of bone marrow/liver transplants in 1994. Hepatitis non-A, non-B, or C and alcoholic liver disease were the most common reasons for LTX. The proportions of recipients with hepatitis B, fulminant liver failure and malignancies, indications with the poorest survival, all declined significantly. The cumulative probability of surviving (without retransplantation) for 7 years after initial transplantation was 0.70 (0.57) for pediatric recipients. Despite changes in recipient characteristics, the one-year survival for pediatric recipients did not change significantly over time. Significant differences in survival, unadjusted for other factors, were found by age (the youngest recipients had the worst survival), location awaiting transplantation (greater medical intervention just prior to transplantation led to poorer survival), multiorgan transplantation, primary liver disease (survival was worst for recipients transplanted due to malignancies, and best for patients with metabolic diseases), and donor/recipient ABO matching (survival was best for recipients of livers from donors with the same blood type). These results are similar to those previously reported for 4- and 5-year survivals. The cumulative probability of adults surviving (without retransplantation) for 7 years following LTX was 0.59 (0.52). Significant differences in survival, unadjusted for other factors, were found for year of transplantation (recipients in 1994 had better one-year survival than those transplanted in previous years), sex (males had worse survival than females), race (Blacks and Asians had the poorest survivals), age (recipients 50 years of age and older had the poorest survival), location awaiting transplantation (greater medical intervention just prior to transplantation led to poorer survival), multiorgan transplantation (recipients of organs in addition to the liver had worse patient survival than recipients of liver only), and primary liver disease (the best survival was for cirrhosis due to cryptogenic or cholestatic cirrhosis, the poorest survival was for malignancies and hepatitis B). Similar results were also reported previously for 4- and 5-year survivals.
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PMID:An update on liver transplantation in the United States: recipient characteristics and outcome. 879 52

Living related liver transplantation (LPLT) as well as reduced sized graft or split liver transplantation has come from the situation with donor shortage for conventional whole liver transplantation from brain dead donors (cadaveric liver transplantation; CLT). To date, about fifty thousands liver transplants have been undergone all over the world. CLT has much diversity of the original diseases, but mainly liver cirrhosis in adults and biliary atresia in children. Original diseases for LRLT have been deviated to cholestatic diseases in childhood, but now LRLT for adults and other diseases in children, i.e. fulminant hepatitis and metabolic disorders, are increasing. The survival rate of LRLT in Kyoto University for first 230 consecutive cases was 79.5%. The main cause of death was infection-related events. There are several problems in LRLT to be resolved; perioperative management of infectious complications, how to deal with the unavoidable ABO incompatible matching transplants, how to expand the national capacity of LRLT to respond the urgent transplantations. Earlier establishment of CLT program in Japan will be necessary to make the liver transplantation the beneficial procedure for more people with end stage liver diseases.
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PMID:[Liver transplantation--present status]. 901 Aug 53


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