Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neuropsychological development was evaluated in 71 infants. Groups: G I, healthy full term infants; G II, premature infants; G III, sick newborns. In these groups, total bilirubin (TB), free bilirubin (FB) and bilirubin binding capacity (BBC) of albumin were determined during the neonatal period. TB and FB concentration were determined by Brodersen and Bartels method. Neurological evaluation was estimated by physical exam, EEG, ophthalmological and audiometric exam. Psychological evaluation was done by Brunet-Lezine test. Infants were subdivided in three groups in relation to the degree of encephalopathy. Premature infants in whom FB was detected in the early neonatal period, the risk of suffering encephalopathy was twice that for simply premature infants. The incidence of encephalopathy in sick full term infants was 68%; 63% of which corresponded to newborns with ABO-Rh iso-immunization, in whom FB was detected in 85% of cases.
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PMID:[Developmental diseases of the central nervous system caused by neonatal hyperbilirubinemia]. 608 68

This paper reports the clinical syndrome of fulminant hepatic failure (FHF) following liver transplantation. FHF was defined as the sudden onset of liver failure [encephalopathy and prolonged International Normalised Ratio (INR)] without arterial thrombosis in the setting of a liver allograft. FHf post-transplant was seen in 8/154 (5.2%) adult patients undergoing transplantation. These eight patients developed a clinical syndrome characterised by: (a) a rapid rise in ALT levels to above 1000 U/l (mean maximum 1600 U/l), (b) a sudden increase in the INR to above 5 (mean maximum 5.6), (c) the development of high fever, (d) the persistence of thrombocytopenia (mean nadir 40 x 10(9)/dl), (e) a progressive rise in the bilirubin (mean maximum 400 mumol/l) and (f) the development of hepatic encephalopathy. In seven cases this syndrome occurred following good initial graft function at day 6 post (mean)-transplant. In one case the above syndrome developed immediately after liver transplantation. Four of the eight patients developed multiorgan failure associated with systemic acidosis (mean pH 6.84). All of these patients died (mean day 11). Four patients developed systemic alkalosis. Two of these four patients underwent successful retransplantation (on days 12 and 13) and remain alive at a mean of 11 months post-transplant. Six of the eight patients received OKT3 therapy without any apparent affect on clinical outcome. Compared to control group of patients (n = 28), 8 versus 2/28 had a positive cross-match with donor lymphocytes (P = NS), 1/8 versus 7/28 were ABO-non-identical (P = NS), 3/8 versus 10/21 had total MHC mismatches (P = NS) and 5/7 versus 6/16 had UW ischemic times above 10 h (P = NS). No patients had main hepatic artery thrombosis on angiography although four patients had evidence of intrahepatic microthrombi or arterial necrosis at autopsy. In all cases the histology showed massive haemorrhagic necrosis. Three cases had evidence of veno-occlusive lesions whilst foam cell arteriopathy was seen in two cases. Immunofluorescence was performed in three cases. In two cases there was evidence of immunoglobulin, complement and fibrin deposition in blood vessels. In conclusion, we describe an uncommon clinical syndrome occurring post liver transplant. This syndrome represents humorally mediated allograft rejection but there seems to be no relationship with tissue matching (antibody, ABO, MHC) or donor ischaemic times. If recognised earlier in the absence of multiorgan failure, urgent retransplantation seems to be the only effective therapy.
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PMID:Fulminant hepatic failure post liver transplantation: clinical syndromes, correlations and outcomes. 788 47

The factors that can influence the outcome of orthotopic liver transplantation (OLT) are numerous. The purpose of this study was to determine the effects of recipient preoperative factors on patient mortality. Between April 1986 and April 1998 a total of 600 OLTs were performed in our institution. We retrospectively reviewed our first 203 consecutive primary adult OLTs with at least 4 years of follow-up. A case-control comparison was performed between survivors and nonsurvivors, and differences in recipient variables were studied for their correlation with patient mortality. A logistic regression analysis was also performed. Mortality was significantly increased among those with fulminant hepatic failure (FHF) (66.6%, p = 0.003), primary cancer (63.1%, p = 0.018), females (46.1%, p = 0. 043), encephalopathy grade IV (72.7%, p = 0.012), recipients under respiratory support (69.2%, p = 0.031), and ABO-incompatible transplants (80%, p = 0.05). FHF, primary cancer, and female gender were the only variables that had a significant association with mortality in the logistic regression analysis. A higher incidence of prolonged respiratory support, bacterial and fungal infections, pneumonia, and chronic rejection contributed to the lower outcome observed in females. These results stress the need for continuous evaluation of the selection criteria of candidates for OLT suffering from primary cancer and FHF. The impact of recipient gender on mortality warrants further analysis but suggests that in the future more attention must be paid to the influence of this factor on the final outcome of OLT.
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PMID:Recipient factors as determinants of mortality after adult liver transplantation. 1055 22

A 21-year-old woman with severe aplastic anemia received an allogeneic bone marrow transplant (allo-BMT) from an HLA-matched and ABO-matched sibling donor after conditioning with cyclophosphamide, rabbit ATG (Lymphoglobuline; Aventis-Pharma), and total lymphoid irradiation. She had a long history of cyclosporin A (CsA) therapy before conditioning. She complained of severe headache and convulsions on day 0, and findings on magnetic resonance images suggested CsA-induced encephalopathy. CsA was immediately stopped, and tacrolimus for prevention of graft-versus-host disease (GVHD) was started on day 2. Hematological engraftment was observed on day 14 without serious GVHD. Prompt diagnosis, replacement of immunosuppressive agents, and careful monitoring of serum drug concentrations are thought to have contributed to the patient's good clinical course, since CsA-induced encephalopathy tends to be recurrent but to improve completely without any sequelae.
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PMID:Cyclosporin A-induced encephalopathy after allogeneic bone marrow transplantation with prevention of graft-versus-host disease by tacrolimus. 1170 97

Recently, new calcineurin inhibitors, such as tacrolimus (FK-506) and microemulsion cyclosporin, have been approved for maintenance immunosuppression in renal transplant recipients and short-term outcomes have been accumulating. In the majority of patients, these calcineurin inhibitors have been used in combination with new immunosuppressive drugs, such as mycophenolate mofetil (MMF) or sirolimus. Under these circumstances, a comparison of cyclosporin and tacrolimus provides the answer to a very important controversial issue. Which drug should we choose in individual patients? In an attempt to answer this question, this review compared the use of tacrolimus and cyclosporin in modern immunosuppressive regimens, which have already been published in well designed clinical studies, and discusses how immunosuppression should be individualised in renal transplant patients.Overall, short-term patient and graft survival with cyclosporin microemulsion and tacrolimus is almost identical. The incidence of acute rejection is generally lower in tacrolimus/azathioprine- than in cyclosporin/azathioprine-treated patients. However, in conjunction with MMF, the difference in the incidence of acute rejection between tacrolimus- and cyclosporin-treated patients became smaller. Adverse events, such as hypertension, hyperlipidaemia and cosmetic changes (gum hypertrophy, hirsutism) seem to be less frequent in tacrolimus-treated than in cyclosporin-treated patients. Recent randomised studies showed that the incidence of post-transplant diabetes mellitus was almost identical between low-dose tacrolimus- and cyclosporin-treated patients. According to the data discussed in this review, the recommendation on the choice of calcineurin inhibitors at this moment is that either cyclosporin or tacrolimus can be used safely and effectively for patients without any risk factors. However, at our centre, we prefer tacrolimus to cyclosporin in patients with a high risk for rejection, such as those with ABO-incompatibility, delayed graft function, sensitisation, and African American race and some other risk factors, such as hypertension and hyperlipidaemia. Moreover, tacrolimus may be preferable to cyclosporin for women because of hirsutism and for children because of the steroid-sparing effect. We consider that cyclosporin should be chosen when patients experience tacrolimus-related adverse events, such as severe chest pain, tremor, gastrointestinal symptoms and encephalopathy. In conclusion, well tolerated and effective immunosuppression is feasible with both cyclosporin and tacrolimus. In the current immunosuppressive regimens, a calcineurin inhibitor, either tacrolimus or cyclosporin, is the essential basic standard immunosuppressant. Clinicians need to decide the best means of optimising therapy for individual patients, based on various risk factors, such as risk of rejection, i.e. sensitisation, delayed graft function and ABO-incompatibility, and some adverse events, such as hypertension, hyperlipidaemia and cosmetic changes.
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PMID:Calcineurin inhibitors in renal transplantation: what is the best option? 1288 61

Children transplanted for ALF urgently require an optimal graft and have lower post-transplant survival compared with children transplanted for chronic liver disease. Over 10 yr, 33 consecutive children transplanted for ALF were followed. Demographics, encephalopathy, intubation, dialysis, laboratory values, graft type ABOI, XL (GRWR > 5%), DDSLT, LDLT and WLT were evaluated. Complications and survival were determined. ALF accounted for 33/201 (16.4%) of transplants during this period. Twelve of 33 received ABOI, five XL grafts, 18 DDSLT, and three LDLT. Waiting time pretransplant was 2.1 days. One- and three-yr patient survival in the ALF group was 93.4% and 88.9%, and graft survivals were 86.4% and 77.7%. Median follow-up was 1452 days. ABOI one- and three yr patient and graft survival in the ALF was 91.6% and 78.6%. No difference in graft or patient survival was noted in the ALF and chronic liver disease group or the ABOI and the ABO compatible group. A combination of ABO incompatible donor livers, XL grafts, DDSLT, LDLT and WLT led to a short wait time and subsequent graft and patient survival comparable to patients with non-acute disease.
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PMID:Pediatric liver transplantation for acute liver failure at a single center: a 10-yr experience. 1951 99

Acute liver failure (ALF) is associated with significant morbidity and mortality. The outcome is highly unpredictable and recovery depends on several factors. Patients can deteriorate with increasing encephalopathy, coagulopathy and progress to multiorgan failure (MOF). In such patients, liver transplantation (LT) is the only current potential cure. Orthotopic liver transplantation remains the standard procedure for LT in ALF, however, other surgical options have been explored. This review summarises the use of a variety of alternative transplant procures for the treatment of acute liver failure including: Two stage OLT, Auxiliary liver transplant, Living donor liver transplantation (LDLT), and ABO incompatible liver transplant.
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PMID:Alternative transplant procedures for acute liver failure. 2108 42

ABO allo-immunization is the most frequent hemolytic disease of the newborn and ABO incompatibility is present in 15-25 % of pregnancies. True ABO alloimmunization occurs in approximately one out of 150 births. Intensity is generally lower than in RhD allo-immunization. We report on three cases showing that ABO allo-immunization can lead to severe hemolytic disease of the newborn with potentially threatening hyperbilirubinemia and complications. Early diagnosis and adequate care are necessary to prevent complications in ABO incompatibility. A direct antiglobulin test is the cornerstone of diagnosis and should be performed at birth on cord blood sampling in all group infants born to O mothers, especially if of African origin. Risk factor analysis and attentive clinical monitoring during the first days of life are essential. Vigilance is even more important for infants discharged before the age of 72 h. Every newborn should be assessed for the risk of developing severe hyperbilirubinemia and should be examined by a qualified healthcare professional in the first days of life. Treatment depends on the total serum bilirubin level, which may increase very rapidly in the first 48 h of life in cases of hemolytic disease of the newborn. Phototherapy and, in severe cases, exchange transfusion are used to prevent hyperbilirubinemia encephalopathy. Intravenous immunoglobulins are used to reduce exchange transfusion. Treatments of severe hemolytic disease of the newborn should be provided and performed by trained personnel in neonatal intensive care units.
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PMID:[Neonatal ABO incompatibility underlies a potentially severe hemolytic disease of the newborn and requires adequate care]. 2125 89

Because of a shortage of organs, non-heart-beating donors have been proposed to be a possible source of grafts for orthotopic liver transplantation. Herein, we have presented a blood group A+ patient with primary biliary cirrhosis, who underwent orthotopic liver transplantation from a non-heart-beating blood group A- donor. On day 5 after transplantation the patient displayed a low hemoglobin levels as well as an increased total bilirubin with progressive encephalopathy, hypotension, and oligoanuria on day 11. The patient responded to steroid treatment. We assume the main cause of organ dysfunction was a passenger lymphocyte syndrome (ABO-Rh incompatibility). Biliary complications were detected at a 6-month follow-up visit by increased hepatic enzymes. We thus concluded that it is useful to take Rh group into account.
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PMID:Early detection of biliary complications and graft rejection in a non-RH Identitical liver transplant recipient from a non-heart-beating donor: a case report. 2297 31

ABO iso-immunization is the most frequent haemolytic disease of the newborn. Treatment depends on the total serum bilirubin level, which may increase very rapidly in the first 48 hours of life in cases of haemolytic disease of the newborn. Phototherapy and, in severe cases, exchange transfusion are used to prevent hyperbilirubinaemic encephalopathy. Intravenous immunoglobulins (IVIG) are used to reduce exchange transfusion. Herein, we present a female newborn who was admitted to the NICU because of ABO immune haemolytic disease. After two courses of 1 g/kg of IVIG infusion, she developed necrotizing enterocolitis (NEC). Administration of IVIG to newborns with significant hyperbilirubinaemia due to ABO haemolytic disease should be cautiously administered and followed for complications.
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PMID:Necrotizing enterocolitis in a newborn following intravenous immunoglobulin treatment for haemolytic disease. 2393 Aug 83


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