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

We investigated 110 cases, selected at random out of the total of 1876 autopsies performed in the Institute of Pathological Anatomy and Histology at the University of Ferrara-Arcispedale Santa Anna on patients who had died at the hospital during 1983-87. Clinical data were taken from 'necropsy request forms' filled in by clinicians and from medical records. We then evaluated the extent of agreement and disagreement, expressed as underdiagnosis (false-negative) and overdiagnosis (false-positive), between the clinical and pathological records with regard to primary disease and to cause of death. Agreement between the diagnoses was 81% for primary disease and 58% for cause of death. The diagnoses of neoplastic, cerebrovascular and cardiovascular diseases showed the closest agreement. Among the neoplasms, those of the liver, gall-bladder, pancreas, retroperitoneal space and prostate were most often overlooked in clinical diagnoses. We had conflicting results for cancer of the lung and of the colon-rectum, for which there was a high level of agreement, but also a large number of false-positive cases and cases in which they were found by chance at autopsy. For cerebrovascular diseases, false-negative and false-positive diagnoses were seen most often for cause of death. With regard to cardiovascular diseases, a relatively uniform distribution was found for myocardial infarction among the three categories, and a high rate of agreement was found for pulmonary embolism. Of all diseases, bronchopneumonia was associated with the highest percentage of false-negative diagnoses for cause of death. Our data on digestive diseases show the strongest agreement on diagnosis of primary disease in relation to cirrhosis of the liver; a high rate of agreement on cause of death was confirmed for alimentary bleeding. Active tuberculosis was detected only at autopsy. We conclude that autopsy is a valid tool for investigation, despite the availability of sophisticated diagnostic techniques.
 ...
PMID:Correlation of clinical diagnosis with autopsy findings. 185 55

Congenital biliary tract atresia was found in 0.58% of children dying in the first year of life. Most frequently atresia included the extrahepatic bile ducts, among them the common bile duct. Five cases (35.7%) had atresia associated with other developmental anomalies, which may suggest a genetic determination of the atresia. The most frequent cause of death of the newborns and infants with congenital biliary tract atresia was bronchopneumonia with associated biliary cirrhosis of the liver.
 ...
PMID:[Biliary atresia in children]. 262 25

From January 1978 to August 1987, 21 patients received a peritoneovenous shunt using the Le Veen valve (LVV). The indications criteria were the long-term diuretic therapy failure (mean time = 24.4 months) or resistence to medical therapy during hospital internment. The 21 patients underwent 36 surgeries, being 4 valve position review and 11 changes of LVV. The mean age was 51.6 years. Fifteen patients had alcoholic cirrhosis, 3 postnecrotic cirrhosis, one Budd-Chiari syndrome, one mansoni Schistosomiasis, and one malignant ascites. Ten were Child B and 9 Child C patients. Eight patients with history of previous esophageal varices bleeding (EVB) underwent endoscopic sclerotherapy (EE) before LVV implantation. Seven patients died in the early postoperative period (3 Child B and 4 Child C patients). Three patients died due to EVB and the others as consequence of hepatic failure (one), cardiac insufficiency (one), sepsis (one), and bronchopneumonia (one). The mean follow-up was 19.9 months (1-61). Early LVV occlusion occurred in 4 patients and late valve occlusion in others 4 patients. The LVV changes were done at ambulatorial preceeding. Ten patients (47.6%) died in late follow-up and in these cases death was related to the main disease course. It is concluded that: 1) LVV is a useful therapy in patients with intractable ascites, since it is not the terminal manifestations of disease; 2) early mortality is related to liver function and late mortality to main disease course; 3) ascitic patients with EVB should undergo endoscopic sclerotherapy before LVV implantation.
 ...
PMID:[Use of the Leveen shunt in the treatment of clinically intractable ascites]. 325 81

Despite Scotland's well-recognised alcohol problem, there is scant information of the aetiology of cirrhosis in this country. This study of 222 patients, reviewed 197 cases presenting as cirrhosis and 25 cases presenting as primary liver cell carcinoma (PLCC) in the East Tayside area of Scotland between 1975 and 1979. The survey was based on an analysis of all histologically proven cases of cirrhosis and PLCC encountered during a five-year period. There was a constant rate of presentation of cirrhosis of about 40 new patients per year, with a stable pattern of aetiology. About 55 per cent were due to alcohol, and there was no significant change in this proportion over the study. No evidence was found for an increasing female susceptibility or earlier female morbidity in alcoholic cirrhosis. Cryptogenic cirrhosis, cardiac cirrhosis and secondary biliary cirrhosis were more often diagnosed at post mortem. Ninety one per cent of patients with primary biliary cirrhosis were females, but the expected male preponderance in haemochromatosis was not present. In addition to the 25 patients presenting with PLCC, three of the cirrhotic patients developed the tumour by the end of 1979. Seventy one per cent of PLCC cases arose in already cirrhotic livers, none were HBsAg positive. Bronchopneumonia, hepatic failure, gastrointestinal bleeding and cardiac failure were the most frequent causes of death.
 ...
PMID:Cirrhosis and primary liver cell carcinoma in Tayside: a five year study. 627 84

A 19-year-old man was admitted with haematemesis and over the next 24 hours developed a bronchopneumonia and septicaemia. Three blood cultures yielded a growth of Pasteurella ureae. An autopsy revealed liver cirrhosis and bleeding oesophageal varices. Past. ureae has been isolated from cases of sinusitis and chronic lung disease but is an extremely rare cause of meningitis and septicaemia. This report describes the second documented case of Past. ureae septicaemia in an adult and the literature is briefly reviewed.
 ...
PMID:Pasteurella ureae septicaemia. A case report. 745 32

The records of 52 children with Niemann-Pick disease type C were reviewed to establish whether the disease process and outcome varied with the initial clinical pattern; 34 children (65%) had cholestatic liver disease and hepatosplenomegaly in infancy; 18 were seen at a mean age of 4 years with splenomegaly or neurologic disease or both. Of the 34 children with early cholestatic liver disease, three died in the neonatal period; cholestasis and hepatomegaly subsided in the remaining 31 children, although splenomegaly persisted. Of these 31 children, 15 had persistent liver disease with elevated aminotransferase values. Serial liver biopsy specimens showed that 3 of the 15 children had normal architecture and 12 had hepatic fibrosis, with progression to cirrhosis in 5. No other significant morbidity or additional deaths were associated with the liver disease. The clinical importance of persistent liver disease was overshadowed by the subsequent development of severe neurologic disease. There was no difference in the age at onset of the disease (mean, 4.5 years) or in the pattern of neurologic disease, including supranuclear ophthalmoplegia, whether or not the child had early liver disease. Overt neurologic disease has not yet developed in seven surviving children with liver disease at onset. Sixty-seven percent of children died during the study; the main cause of death was bronchopneumonia. We conclude that the diagnosis of Niemann-Pick disease type C should be considered in patients with unexplained neonatal hepatitis, especially if splenomegaly is a persistent feature. Because liver biopsy specimens may not demonstrate storage cells, bone marrow aspiration to detect the characteristic storage cells is recommended in such patients.
 ...
PMID:Niemann-Pick disease type C: diagnosis and outcome in children, with particular reference to liver disease. 815 88

1. We report the first demonstration of the pathophysiological importance and clinical applications of the relatively recently discovered circulating enzyme, phosphoinositol-specific phospholipase D. This enzyme is known to cleave the large variety of important cell-surface molecules linked to the cell membrane by glycan-phosphatidylinositol linkages (glycan-phosphatidylinositol anchors). 2. When measured in the sera of healthy individuals, phosphoinositol-specific phospholipase D activity was found to show a strong negative correlation with age, the degree of depreciation being greater than that measured for most other analytes. 3. Serum phosphoinositol-specific phospholipase D activity was considerably depressed in patients presenting with conditions leading to reduced liver synthetic reserve, such as hepatocellular carcinoma or liver cirrhosis caused by chronic viral hepatitis, and correlated with reduced albumin levels in these conditions, indicating that the liver is the site of phosphoinositol-specific phospholipase D synthesis and that phosphoinositol-specific phospholipase D may be used as an additional marker of liver synthetic reserve. 4. When measured in patients with acute liver disease, such as acute viral hepatitis, or in patients with bronchopneumonia, phosphoinositol-specific phospholipase D activity was found to be significantly raised, demonstrating features characteristic of an acute-phase reactant. 5. These findings indicate that, besides its pathophysiological importance, phosphoinositol-specific phospholipase D and the measurement of its activity in serum may have a useful place in the investigation of a range of clinical conditions, including tissue injury and inflammation.
 ...
PMID:Inositol-specific phospholipase D activity in health and disease. 816 40

The autopsy findings of 80 human immunodeficiency virus (HIV)-infected adults, who died between 1982-1995, are presented with special emphasis on the risk factor of hemophilia. The study included 23 blood product recipients (hemophiliacs n = 21; non-hemophiliacs n = 2), 34 homosexuals, four intravenous drug abusers, and 19 patients with no known risk factor. Nearly all individuals (93%) showed the late stage of acquired immunodeficiency syndrome (AIDS). Blood product recipients had a significantly lower overall frequency of opportunistic infections (p < 0.05). Homosexuality was associated with the highest overall frequency of opportunistic infections and HIV-associated malignancies, such as Kaposi's sarcoma and malignant non-Hodgkin's lymphoma. Exclusive visceral involvement of Kaposi's sarcoma was frequent, and no decrease of Kaposi's sarcoma was observed during the study period. Pneumocystis infections, atypical mycobacteriosis, and non-Hodgkin's lymphoma showed a significant increase during the last five years (1991-1995) of the observation interval. Opportunistic infections and malignancies were the cause of death in approximately one-half of the patients. In blood product recipients, hepatic failure due to posthepatitic cirrhosis and hemorrhage due to hepatic failure with subsequent coagulopathy and in non-blood product recipients, bacterial bronchopneumonia, and diffuse alveolar damage were additional major causes of death. The data suggest a lower risk for HIV-infected blood product recipients, particularly hemophiliacs, to acquire opportunistic infections and malignant neoplasms.
 ...
PMID:Autopsy findings in patients with human immunodeficiency virus infection with emphasis on the risk factor of hemophilia. 878 Sep 28

The most common hepatic complications of cystic fibrosis (CF) are steatosis, fibrosis, biliary cirrhosis, atretic gallbladder, cholelithiasis, and sclerosing cholangitis. Cholestatic liver disease is a slow progressive disorder, but will stabilize for many patients. CF patients may suffer from the consequences of their liver disease and without liver transplantation, variceal hemorrhage, malnutrition, or end-stage liver disease can lead to death. Prospective data were collected and reviewed on 311 liver transplants performed in 283 patients at the Children's Medical Center of Dallas between October 1984 and November 2000. Ten children received an orthotopic liver transplant (OTLX) for end-stage liver disease associated with cystic fibrosis. Pulmonary function tests were obtained preoperatively in all cases. There were nine boys and one girl. Six are currently alive, and four are dead. Both patient and graft survival was 5.75 yr. Among those currently alive, mean patient and graft survival is 7.71 yr (range 0.10-12.62 yr). Mean patient and graft survival of those who died was 2.35 yr (range 0.78-5.33 yr). No survivor required re-transplantation and currently, all have normal serum aminotransferase values. Chronic sinusitis was not a significant pre- or post-transplant morbidity, although systematic radiographic evaluation of the sinuses did not occur. Pulmonary deaths occurred in three patients from pulmonary hemorrhage, pulmonary infection with Aspergillus and Candida glabrata, and acute bronchopneumonia associated with polymicrobial sepsis because of Pseudomonas, Klebsiella, and Candida albicans 1.44, 0.78, and 1.83 yr, respectively, after transplantation. The fourth death was associated with chronic rejection, and occurred 5.33 yr after transplantation. All non-survivors were below the 5th percentile for height and weight at the time of liver transplantation. Mean age at transplantation was 9.72 yr (range 1.23-19.09, median 9.61). Survivors were transplanted at a younger age than non-survivors (mean of 9.21 yr vs. 10.66 yr), and had shorter waiting times from diagnosis of end-stage liver disease to transplantation (6.87 months vs. 13.83 months). Eighty percentage (n = 8) of patients had pretransplant variceal bleeds (83% of survivors, 75% of non-survivors). While all non-survivors had a history of meconium ileus and preoperative need of pancreatic enzymes, only 67% of those alive experienced these complications. Preoperative forced vital capacity FVC was 103% for survivors and 95% for non-survivors. The corresponding numbers for forced expiratory flow (FEF) 25-75 were 74-84% respectively. Preoperative Aspergillus was identified in 30% of patients (n = 3). Two of these patients are alive. Cystic fibrosis constitutes an indication for 3.5% of pediatric liver transplants. Evaluation and transplantation for end-stage liver disease associated with cystic fibrosis should be undertaken at an early age. Most deaths were associated with pulmonary/septic events, and occurred less than 2 yr after OLTX. Those children who did not survive had poor growth and nutrition, prolonged waiting times prior to transplantation, were transplanted at an older age, and had a higher incidence of pancreatic insufficiency and meconium ileus. The presence of Aspergillus in the sputum does not constitute a contraindication for OLTX.
 ...
PMID:Liver transplantation for cholestasis associated with cystic fibrosis in the pediatric population. 1265 48

Data on pathologic changes of the 2019 novel coronavirus disease (COVID-19) are scarce. To gain knowledge about the pathology that may contribute to disease progression and fatality, we performed postmortem needle core biopsies of lung, liver, and heart in four patients who died of COVID-19 pneumonia. The patients' ages ranged from 59 to 81, including three males and one female. Each patient had at least one underlying disease, including immunocompromised status (chronic lymphocytic leukemia and renal transplantation) or other conditions (cirrhosis, hypertension, and diabetes). Time from disease onset to death ranged from 15 to 52 days. All patients had elevated white blood cell counts, with significant rise toward the end, and all had lymphocytopenia except for the patient with leukemia. Histologically, the main findings are in the lungs, including injury to the alveolar epithelial cells, hyaline membrane formation, and hyperplasia of type II pneumocytes, all components of diffuse alveolar damage. Consolidation by fibroblastic proliferation with extracellular matrix and fibrin forming clusters in airspaces is evident. In one patient, the consolidation consists of abundant intra-alveolar neutrophilic infiltration, consistent with superimposed bacterial bronchopneumonia. The liver exhibits mild lobular infiltration by small lymphocytes, and centrilobular sinusoidal dilation. Patchy necrosis is also seen. The heart shows only focal mild fibrosis and mild myocardial hypertrophy, changes likely related to the underlying conditions. In conclusion, the postmortem examinations show advanced diffuse alveolar damage, as well as superimposed bacterial pneumonia in some patients. Changes in the liver and heart are likely secondary or related to the underlying diseases.
 ...
PMID:Pathological study of the 2019 novel coronavirus disease (COVID-19) through postmortem core biopsies. 3229 99


1