Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 55-year-old male underwent orthotopic liver transplantation for sub-fulminant hepatitis B/delta infection superimposed on probable genetic hemochromatosis with early cirrhosis. Pre-operatively, he demonstrated serologic evidence of cytomegalovirus reactivation and developed cytomegalovirus viremia when ganciclovir was discontinued post-operatively. His post-operative course was complicated by chronic ductopenic rejection, biliary anastomotic leak, and persistent confusion and malaise. At the time of laparotomy for repair of the bile leak, nodular peritoneal lesions were noted, with biopsy and culture showing angioinvasive Aspergillus fumigatus. Despite administration of amphotericin B, the patient continued to have culture-confirmed evidence of infection at follow-up peritoneoscopy. Oral itraconazole was begun, but the patient died of liver failure secondary to progressive ductolpenic rejection. At autopsy, Aspergillus organisms were seen in histologic sections taken from the small bowel; there was no evidence of disseminated disease.
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PMID:A case of Aspergillus fumigatus peritonitis complicating liver transplantation. 816 55

The histologic diagnosis of extrahepatic biliary obstruction in the setting of orthotopic liver transplantation (OLT) may be complicated by nonobstructive factors, such as rejection, preservation injury, drug effects, and infection. Indeed, biopsy specimens from OLT patients frequently exhibit early posttransplant cholestasis, which is likely the result of several of these factors acting in concert. We reviewed the clinicopathologic features of 14 OLT patients whose biopsy specimens displayed prominent cholangitis with "obstructive"-type features. Cholangiograms, Doppler ultrasound examinations, and bacterial cultures were obtained in conjunction with all biopsy specimens. Despite histologic evidence of biliary obstruction, cholangiography revealed obstruction in only one case, leaks in four cases, and nine normal studies. Ten patients had severe systemic bacterial or cytomegalovirus infections. Biliary infection was documented in two cases. Hepatic artery occlusion was diagnosed in one case. The cholangitic pattern persisted in patients with unremitting infections, and two patients developed secondary biliary cirrhosis despite having consistently normal cholangiograms. Features of acute rejection or viral hepatitis were not observed on any biopsy specimen. The presence of this clinicopathologic spectrum was associated with a poor prognosis. Nine patients died and only four patients who responded to antibiotics survived. These findings demonstrate a striking cholangitic response to systemic infections in OLT grafts. The diagnosis of biliary obstruction in OLT liver biopsy specimens cannot be rendered without cholangiographic verification.
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PMID:Rejection-independent cholangitis and cirrhosis following orthotopic liver transplantation. 825 64

A retrospective study was carried out in 56 patients to establish the association of cytomegalovirus (CMV) with active or inactive hepatitis B virus (HBV) infection as a possible risk factor in the development of severe liver disease. Patients with positive CMV serology and active or inactive HBV infection had elevated alanine aminotransferase activity and had a relatively high incidence of more severe lesions (chronic hepatitis and active cirrhosis). In the absence of CMV, only one case of cirrhosis was identified compared with seven cases of hepatic fibrosis. By analogy with hepatitis C virus, CMV may bring about activation of the host inflammatory response against hepatocytes following HBV infection, resulting in the development of severe hepatitic disease.
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PMID:Liver pathology in cytomegalovirus infection associated with hepatitis B virus. 829 58

Hepatitis C virus (HCV) is important to the liver transplant recipient for several reasons. First, chronic HCV infection is a frequent cause of end-stage liver disease in North America and Europe, where the majority of liver transplants are performed. Second, recurrence of HCV after liver transplantation is almost universal so that many liver transplant recipients with and without overt hepatitis are viraemic. Third, HCV infection in the organ donor and/or in the blood transfused in the peri-transplant period make acquisition of HCV possible. Finally, liver transplantation for chronic HCV infection represents a major financial burden to the health-care system in the USA and worldwide. Histological hepatitis not due to HBV or cytomegalovirus (CMV) is present in 14-35% of allografts from patients undergoing liver transplantation, and the majority of this is due to HCV infection. HCV infection recurs post-transplant in almost all patients with pre-transplant infection. Proof that HCV does recur has been provided by the sequencing of the hypervariable domain of the E2/NS1 region. The magnitude of HCV infection is underestimated by using serological assays in this immunosuppressed population. Using the b-DNA assay, HCV-RNA levels have been shown to increase in patients with recurrent infection. The long-term consequences remain to be defined but post-transplantation HCV infection is generally much less devastating than post-transplantation HBV infection, and many patients have clinically silent disease. It is likely that a carrier state exists in immunosuppressed transplant recipients since high HCV-RNA levels occur in the absence of liver damage. In one study, 1, 2 and 3 year patient survival rates were shown to be comparable in patients with chronic active HCV infection and with cryptogenic cirrhosis (94, 89 and 87%, and 84, 84 and 73%, respectively). A more recent study from the University of Pittsburgh which compared the outcome of a larger number of HCV-infected patients (n = 237) with a large number of control patients with non-viral disease (n = 801), showed that indeed HCV infection does impact negatively on both patient and graft survival (1, 2 and 3 year patient survival in the study and control groups of 78, 68 and 66% and 84, 82 and 78%, respectively, P = 0.001). Undoubtedly with time, the full impact of recurrent HCV infection will become apparent, although short-term survival is sufficiently good to warrant continued transplantation of this group of patients.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Hepatitis C and liver transplantation. 852 17

We describe three men and two women, aged 18-50, with an occasional finding of increased aspartate and alanine aminotransferase and gamma-glutamyl transpeptidase levels in the absence of any drug treatment and past or current alcohol abuse. Two patients were overweight (body mass index 29 and 32, respectively) and physical examination was normal in all but one case. Tests for hepatitis A, B and C, Epstein-Barr virus, cytomegalovirus, toxoplasma and autoimmune hepatitis were negative and metabolic diseases (Wilson's disease, haemochromatosis, alpha-l-antitrypsin deficiency) were excluded by specific tests. Ultrasound liver scan revealed massive steatosis in all patients. Liver histology showed diffuse steatosis and parenchymal inflammation in all cases, with concomitant fibrosis and Mallory bodies in three of them. Findings were consistent with non-alcoholic steatohepatitis, a rare condition with potential progression to cirrhosis in a minority of cases. This disease, for which no treatment is currently available, must be considered in all subjects with elevated aminotransferases, in the absence of known causes of liver damage.
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PMID:Non-alcoholic steatohepatitis. Report of five cases and review of the literature. 878 33

A retrospective analysis was undertaken to determine if the incidence, timing, and severity of acute and chronic rejection were influenced by the primary disease necessitating transplantation. Of the 875 liver transplantations performed between 1984 and 1992, 768 were primary transplantations and 107 were retransplantations. Among the former, 330 patients that were liver transplant recipients for a chronic liver disease without cancer in the native liver received an ABO-compatible and cross-match-negative graft and were given a cyclosporine- or tacrolimus-based immunosuppression. These included primary biliary cirrhosis (PBC, 66 patients), primary sclerosing cholangitis (PSC, 23 patients), alcoholic cirrhosis (ALC, 21 patients), autoimmune cirrhosis (AIC, 17 patients), hepatitis B virus-induced cirrhosis (HBV-C, 116 patients) and hepatitis C virus-induced cirrhosis (HCV-C, 87 patients). The incidence of acute (48% +/- 3% [SE] at 1 year) and chronic rejection (10% +/- 2% at 3 years) was comparable in patients who have undergone transplantation for PBC, PSC, AIC, and HCV-C. However, the incidence of acute (but not chronic) rejection was significantly lower in patients who have undergone transplantation for ALC (29% at 1 year). This reduced incidence of acute rejection was associated with an increased incidence of bacterial infections. In patients who have undergone transplantation for HBV-C (the majority of whom had received long-term anti-hepatitis B surface antigen [HBs] immunoglobulins), the incidence of both acute (21% at 1 year) and chronic rejection (0% at 3 years) was significantly lower, whereas the incidence of septic complications was comparable with that in the other groups. The incidence of acute rejection in patients who have undergone transplantation for nonviral disease receiving polyclonal human anti-cytomegalovirus (CMV) immunoglobulins was also significantly lower than that of patients who did not receive the immunoglobulins (19% vs. 48% at 3 months; P = .01), and this was identical to that of patients who have undergone transplantation for viral disease receiving polyclonal human anti-HBs immunoglobulins (19% at 3 months). These results show that the risk of rejection is unequal among patients, being lower in patients who have undergone transplantation for ALC (probably as a result of a state of nonspecific hyporesponsiveness) and in patients who have undergone transplantation for HBV-C (possibly as a result of long-term administration of polyclonal human immunoglobulins).
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PMID:Incidence of rejection and infection after liver transplantation as a function of the primary disease: possible influence of alcohol and polyclonal immunoglobulins. 1100 37

A boy underwent liver transplantation for postnecrotic cirrhosis secondary to Wilson's disease. The patient had no neurological clinical manifestations prior to the transplantation. The patient developed dysarthria, dysphagia, spasticity, rigidity, and intention and resting tremor of all extremities. Cranial computerized tomography revealed hypodensity of the thalamus, basal ganglia and external capsule. Anti-cytomegalovirus IgM became positive. At autopsy, there were severe pathological changes at the thalamus and basal ganglia.
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PMID:Extrapyramidal disorder secondary to cytomegalovirus infection and toxoplasmosis after liver transplantation. 874 Jan 36

We identified 54 patients with AIDS and ascites seen over a 4.5-year period at a university hospital. This retrospective study is the largest reported series of patients with AIDS and ascites. Patients with AIDS who are evaluated for ascites should be stratified by the CD4 + cell count and the presence or absence of portal hypertension based upon the serum-ascites albumin gradient and clinical presentation. Awareness of possible surgery-related causes of ascites is crucial, as these patients may not manifest the usual signs and symptoms of peritonitis or abdominal catastrophes seen in immunocompetent hosts. Patients with evidence of portal hypertension due to hepatic cirrhosis and an elevated ascitic neutrophil count should be suspected to be infected with common bacterial pathogens associated with peritonitis unless the CD4 + cell count is below 50 cells/mm3. When the CD4 + cell count declines below this threshold, infections due to Mycobacterium avium complex, cytomegalovirus, and other opportunistic infections should be considered.
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PMID:Ascites and the acquired immunodeficiency syndrome. Report of 54 cases. 896 82

We report an association between X-linked immunodeficiency with hyper-IgM (XHIM) and carcinomas affecting the liver, pancreas, biliary tree, and associated neuroectodermal endocrine cells. The tumors were fatal in eight of nine cases and in most instances were preceded by chronic cholangiopathy and/or cirrhosis. An additional group of subjects with XHIM had chronic inflammation of the liver or bile ducts but no malignancy. Many patients with XHIM were infected with cryptosporidia. CD40 is normally expressed on regenerating or inflammed bile duct epithelium. A CD40+ hepatocellular carcinoma cell line, HepG2, susceptible to cryptosporidia and CMV infection became resistant when cell surface CD40 was cross-linked by a CD40 ligand fusion protein. Apoptosis was triggered in HepG2 cells if protein synthesis was blocked by cycloheximide or if the cells were infected by cryptosporidia. Ligation of CD40 on biliary epithelium may contribute to defense against infection by intracellular pathogens. We propose that the CD40 ligand mutations that cause XHIM deprive the biliary epithelium of one line of defense against intracellular pathogens and that malignant transformation in the biliary tree follows chronic infection or inflammation. The resulting tumors may then progress without check by an effective immune response. Patients with XHIM who have abnormal liver function tests should be considered at increased risk for cholangiopathy or malignancy.
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PMID:Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immunodeficiency with hyper-IgM. 899 19

In summary, the following principles are worth reiterating: 1. In the treatment of acute liver failure, protection of the native liver in anticipation that it will recover, but positioning of the allograft in a manner that optimizes its function for both the short and long term (in the event that the native liver does not recover) are important goals. Therefore, orthotopic positioning offers advantages over the heterotopic position in most cases. Development of better techniques for predicting native liver recovery might remove any of these advantages of the orthotopic position. 2. Other than the presence of fibrosis, the performance of a native liver biopsy does not appear to predict native liver recovery. The decision of whether to attempt auxiliary grafting must be based on an understanding of the natural history of the disease causing the acute liver failure. 3. The heterotopic position has the advantage of not requiring partial native hepatectomy in order to accommodate the allograft. However, except for the recent experience of Terpstra et al, this technique has carried a higher risk of venous outflow obstruction. It also requires additional space within the abdomen, usually mandating the use of prosthetic abdominal wall closures and the construction of venous conduits for portal venous inflow to the liver. There is the additional theoretical concern about competition for portal venous flow leading to eventual atrophy of the allograft liver. 4. Common events that follow liver transplantation result in changes in portal venous resistance within the liver, events that therefore alter the relative distribution of portal venous inflow between native and auxiliary livers. These events include reperfusion injury, allograft rejection, allograft viral infection (e.g., cytomegalovirus, Epstein-Barr virus, recurrent viral hepatitis), and native liver regeneration. Attempts to control portal venous flow to favor one liver over the other must account for the effect of these factors. 5. In general terms, auxiliary transplantation is not indicated for diseases in which the residual native liver either represents an ongoing threat to the recipient or is incapable of supporting life alone. This may be the case in both metabolic disorders and in cirrhosis. Most of the alleged difficulties of native hepatectomy are no longer relevant. Therefore, auxiliary transplantation is rarely if ever indicated for chronic liver disease and may not be of any additional benefit over total transplantation in the treatment of many metabolic disorders. 6. In the treatment of acute liver failure, the value of an auxiliary transplant over total transplant is obtained when the native liver recovers and the patient is withdrawn from immunosuppression. If further experience shows the effectiveness of this option, total liver transplantation with the requirement for life-long immunosuppression will no longer be appropriate for the treatment of patients with acute liver disease.
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PMID:Auxiliary liver transplantation for acute liver failure. 934 65


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