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)

In 100 adult patients with severe haemophilia A (78 patients) and B (22 patients) sera were screened for the presence of serological markers of hepatitis B virus (HBV) and of cytomegalovirus (CMV) and liver function tests were performed which included measurement of serum aminotransferase AST and ALT activities, total bilirubin concentration and plasma levels of factor VII and X. In all the patients at least one out of five determined HBV markers (HBsAg. HBeAg, anti-HBs, anti-HBc and anti-HBe) was detected. HBsAg was found in 10% of the patients, and its prevalence in haemophiliacs B was higher than than observed in haemophiliacs A (22.7% and 6.4%, respectively). HBsAg appeared more frequently in patients receiving factor VIII concentrates (16.7%) than in those treated with cryoprecipitate (4.5%). Anti-CMV antibody was detected in sera of 98% of the patients. In 1/3 samples of cryoprecipitate anti-HBc or anti-HBs were present, and in the half of samples anti-CMV occurred. Abnormal liver function tests indicating chronic hepatitis or liver cirrhosis were obtained in 8 patients. Raised ALT activity which could suggest chronic infection with non-A, non-B virus occurred in 6 cases. The present study indicates that haemophiliacs frequently transfused with plasma products are at high risk for viral infections leading to liver dysfunction.
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PMID:[Serological markers of hepatitis B virus and cytomegalovirus in patients with hemophilia]. 217 33

We analyzed the postoperative complications excluding graft rejection in 52 consecutive orthotopic liver transplantations performed from March 1986 to November 1988 in 48 patients. Thirteen patients died: one intraoperatively, seven during the first 2 months, and five between 5 and 28 months. Complications were predominant during the first 3 months; infection was the most common complication. The main cause was viral agents. Cytomegalovirus was responsible for infection in 62 percent of cases, but was symptomatic in only 37 percent of patients and always had a favorable outcome. Six cases of disseminated candidiasis were observed with fatal outcome in 3 cases. Ten patients had septicemia due to Gram positive germs with a favorable course in all cases. Two patients required retransplantation on the 2nd postoperative day because of primary graft failure. Three patients had hepatic infarction which was fatal in one case. Technical complications were represented by intra-abdominal bleeding in 3 cases, perihepatic hematoma in 10 cases and stenosis of the biliary anastomosis in 8 cases; in one patient, partial portal vein thrombosis occurred; no hepatic arterial thrombosis occurred during the first postoperative days but this complication was diagnosed later in 3 instances by arteriography. Five out of 7 patients transplanted for malignant liver disease experienced recurrence which cause death in 4 cases. In 3 out of the 5 patients transplanted for postviral B cirrhosis, chronic active hepatitis occurred 6 months after transplantation and one of these patients had to be retransplanted at 13 months for recurrence of cirrhosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Complications in 52 liver transplantations excluding graft rejection]. 236 77

Severe neonatal cholestasis is a clinical problem requiring a differential diagnosis of intra- (hepatitis) and extrahepatic (biliary atresia) causes, prognosis and therapy being different in the two cases. Eighteen patients of pediatric age underwent US and hepatobiliary scintigraphy. US findings were aspecific in both hepatitis and biliary atresia. In the 11 patients with hepatitis, hepatobiliary scintigraphy after phenobarbital revealed labeled bile in the bowel. Only in 1 patient with cytomegalovirus hepatitis was a scintigraphic pattern similar to that of biliary atresia. On the contrary, no intestinal radioactivity within 24 hours was seen in 6 patients with biliary atresia. A portoenterostomy (Kasai's operation) was performed on 4/6 cases with biliary atresia. These patients were followed with hepatobiliary scintigraphy in order to evaluate anastomotic functionality. In a case of biliary cirrhosis secondary to occlusion, orthotopic liver transplantation was performed whose success was scintigraphically monitored. Our results point to hepatobiliary scintigraphy after phenobarbital as the best noninvasive procedure for both diagnosis and postoperative follow-up of biliary atresia. Labeled bile excretion within 24 hours was rarely found in both atresia and neonatal hepatitis.
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PMID:[Hepatobiliary scintigraphy in the study of neonatal hepatic cholestasis]. 251 35

The Hospital for Sick Children's initial 2-year experience with pediatric liver transplantation is reviewed. Patients are divided into high- and low-risk groups according to certain criteria. The high-risk group includes patients under 10 kg in weight, those with extrahepatic biliary atresia (EHBA), those with portal vein atresia or thrombosis, and those in hepatic coma. All others were considered low risk. Twenty-nine patients were assessed for transplantation: 18 were transplanted and 6 (21% of total referred) died while on the waiting list. Eighteen patients received 23 transplants. Of the 18 recipients, nine had EHBA, four had fulminant hepatic failure, two had tyrosinemia, one had glycogen storage disease, one had Indian childhood cirrhosis, and one had idiopathic cirrhosis. Seven of the 13 patients in the high-risk group survived (55% survival) with 1 to 23 month follow-up. Survival was significantly higher (80%) in the low-risk group (P less than 0.05). Four patients were retransplanted and two survived. Early deaths occurred from prolonged warm ischemia, recurrent portal vein thrombosis, and brain death in a patient who had been transplanted in hepatic coma. Late deaths occurred from cytomegalovirus (CMV) disease (2 patients), acute rejection (1 patient), and myocardial infarction (1 patient). The incidence of primary nonfunction was 4.3% (1 of 23) and of arterial thrombosis was 13% (3 of 23). Survival in patients transplanted for EHBA (67%) was slightly higher than it was for the rest of the group, although not as good as it was in the low-risk group.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Liver transplantation in children: the initial Toronto experience. 255 8

We report the case of a 44-year-old man who was transplanted in 1986 for hepatocellular carcinoma in a HBsAG-positive liver cirrhosis. The patient had no severe complications postoperatively. He received passive immunization for the prevention of hepatitis B reinfection during the first 6 months after liver grafting. Twelve months after the transplantation the new liver was reinfected with hepatitis B virus. Without any clinical or laboratory signs of severe hepatitis, the patient developed a histologically proven complete liver cirrhosis within 8 months after reinfection of the graft. The reasons for this might have been, first, a deleterious course of the infection under immunosuppressive therapy, and, second, the additional influence of a postoperatively acquired CMV infection or the combined toxic influence of cyclosporin A and its metabolites on the acute inflammation in the liver.
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PMID:Unusually rapid development of a HBsAG-positive liver cirrhosis after liver transplantation. 255 27

Hepatitis non-A, non-B (HNANB) is due to one or more transmissible agents, probably viruses. Epidemiologically, HNANB is transmitted predominantly by transfusion of blood or plasma derivatives, and percutaneous inoculation, but a non-percutaneous transmission by the fecal-oral route is also established. However, despite 10 years of intense world-wide research, the transmissible agent, or agents, have not been identified and there are no serological assays for either an antigen or an antibody that can be used to detect this infection. The clinical diagnosis of HNANB remains, therefore, a diagnosis of exclusion mainly of hepatitis A and B, Epstein-Barr virus, cytomegalovirus and drug-induced liver disease. In contrast to hepatitis A and B, the clinical and biochemical course of HNANB tends to be less severe and the proportion of asymptomatic and anicteric cases is higher, but fulminant hepatitis and fatalities also occur. Typically, there is a fluctuating waxing and waning pattern of the serum aminotransferase activities in HNANB. HNANB has a relative high tendency to progress to a chronic stage. The exact frequency of HNANB-induced liver cirrhosis and convincing evidence for an association with hepatocellular carcinoma cannot be assessed, although the persistence of the infectious agent in chronic HNANB and the existence of a chronic asymptomatic carrier state have been proved. By light microscopy there is a broad morphologic spectrum of acute and chronic viral hepatitis, but no single pathognomonic lesion exists that allows a reliable distinction to be made of HNANB from hepatitis A and B. Electron microscopy of liver biopsy specimens of chimpanzees, experimentally infected with HNANB agents, permits the visualisation of cytoplasmic changes, which appear to be specific for infection with HNANB viruses. In human liver biopsy specimens from patients with HNANB, identical ultrastructural cytoplasmic changes could not consistently be demonstrated. In contrast, intranuclear aggregates of spherical and tubular particles measuring 20-29 nm, first described in experimental HNANB in chimpanzees, have been repeatedly demonstrated in acute and chronic HNANB in man. These nuclear particles have been considered as compelling evidence of human HNANB infection. The specificity has been challenged, however, by the demonstration of identical particles in other viral and non-viral hepatopathies and in liver biopsies of healthy volunteers. By immune electron microscopy, a multiplicity of virus-like particles are described in association with HNANB.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Hepatitis non-A, non-B: epidemiologic, clinical, serologic and morphologic aspects]. 258 79

Fifty-six patients with moderate to severe neonatal hepatitis were followed for 12 to 78 months. Two died from causes other than hepatitis itself and were free from liver disease at the time of death. Of the remaining 54 patients, seven died of hepatitis, two are living with chronic liver disease and psychomotor retardation, and 45 are living without liver disease. High peak bilirubin levels and liver histologic findings of periportal fibrosis, moderate to severe portal inflammation, and/or diffuse giant cell transformation appear to be major factors predictive for poor outcome. Cytomegalovirus (CMV) infection was a common associated infection. Evidence of CMV infection was found in 22 (49%) of the 45 patients studied. Three of them died, and one is still living with cirrhosis of the liver. Metabolic disorders such as alpha-1-antitrypsin deficiency, galactosemia, and aminoaciduria and/or aminoacidemia were carefully screened but were not found in these cases. A fatal case had a sibling who had died of a similar disease course. Chinese infants may have metabolic and familial cholestasis diseases requiring further investigation.
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PMID:Neonatal hepatitis: a follow-up study. 282 43

A 39-year-old woman who had cryptogenic cirrhosis and who had received two liver transplants developed necrotic skin lesions over the chest, upper arms, and thighs. Biopsy showed enlarged endothelial cells with intranuclear and intracytoplasmic inclusions characteristic of cytomegalovirus (CMV) infection. However, a few multinucleated gaint cells were observed beneath a necrotic epidermis. This, combined with the clinical presentation, suggested to infectious disease consultants an infection with herpes simplex virus. Using the immunoperoxidase technique, inclusions stained positively with antibody to CMV and showed a negative reaction for herpes simplex antigen. In situ hybridization using biotinylated DNA probes on formalin-fixed, paraffin-embedded tissue sections confirmed the diagnosis of CMV infection and failed to substantiate infection with herpes simplex virus. Subsequently, blood cultures became positive for CMV. The early recognition of CMV infection in the skin permitted institution of antiviral therapy with gancyclovir. Specific skin lesions of CMV infection are likely to be encountered with increasing frequency among immunosuppressed patients. Lesions may be vesicular, and epidermal multinucleated giant cells can occasionally be identified. In situ hybridization is a technique that is readily adaptable to surgical pathology laboratories and permits both a rapid, specific diagnosis and the early institution of appropriate therapy.
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PMID:Cutaneous cytomegalovirus infection in a liver transplant patient. Diagnosis by in situ DNA hybridization. 285 Dec 72

The safety of blood and blood products in Scandinavia today is high. An absolutely safe blood supply is, however, an unattainable goal. The dominating risk is transmission of non-A, non-B virus (NANBV). The calculated per blood unit risk is 1:200. The incidence of cirrhosis due to post-transfusion hepatitis NANB is calculated to at most 0.1% among recipients of blood components from about 5 donors. Other risk factors are transmission of hepatitis B virus (HBV), human immunodeficiency virus (HIV-1) and cytomegalovirus (CMV). The prevalence of HBsAg among first time donors is about 0.05% (Sweden). In Scandinavia, anti-HIV-1 has been found in 0.001% of donations from start of screening in 1985 to December 1987. The prevalence was higher in Denmark, lower in Finland (and perhaps Iceland). The prevalence has declined during the last years. As of June 1988, 117 patients in the Scandinavian countries have been infected by blood components, all but 2 before screening was introduced. Besides these, 226 haemophiliacs have been infected by, in almost all cases, imported clotting factor concentrates before heat treatment was introduced. Most of the infected patients are still asymptomatic. About 70% of blood donors have anti-CMV, a few percent of which will transmit CMV-infection, with severe symptoms, to immunosuppressed patients without anti-CMV.
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PMID:Safety of blood and blood products in Scandinavia today. 306 87

Eleven patients of Chinese origin experienced spontaneous reactivation of chronic active hepatitis B. Eight HBsAg-positive patients were followed for an average of 15 months prior to, while three others presented during reactivation. Fatigue, hepatomegaly and jaundice were frequent findings. Elevation of both serum ALT (average = 1,212 units per liter) and hepatitis B virus DNA levels were noted in all patients, and reactivation lasted an average of 4.4 months. During resolution, clinical symptoms abated, serum ALT levels reverted toward normal, and in nine patients, the hepatitis B virus DNA values became undetectable. All patients lacked evidence for acute hepatitis A, Epstein-Barr Virus, cytomegalovirus or hepatitis delta virus infection. Histologic findings of liver tissue from eight patients showed piecemeal necrosis and fibrosis. Within the parenchyma, varying degrees of hepatocytolysis with cuffing, perivenular necrosis and acidophilic bodies were noted. Ground-glass cells and regenerative changes also were observed. Cirrhosis was not present in any of the liver biopsies. These findings suggest that spontaneous reactivation of hepatitis B occurs in heterosexual patients with chronic active hepatitis B and contributes to chronic inflammation and to the progression of their liver disease.
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PMID:Spontaneous reactivation of hepatitis B in Chinese patients with HBsAg-positive chronic active hepatitis. 361 49


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