Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To clarify the pathogenesis of hepatic cytomegalovirus (CMV) infection, we clinicopathologically investigated 18 infants and 10 adults with cytomegalic inclusion bodies (CIB) in the liver among a total of 75 autopsy cases with CIB in any organ of the body. CMV infection was confirmed by immunohistochemistry and in situ hybridization. When CIB were present in the liver, CMV infection also tended to be systemic. All the adults were immunocompromised patients, but diseases inducing immunodeficiency were present in only two of the infants. The severe and systemic CMV infections we found in infants might have been associated with congenital CMV infection. Histologically, hepatocyte necrosis, cholestasis, extramedullary hematopoiesis and fatty degeneration were more frequent and prominent in infants than in adults. However, inflammatory cell infiltration was only slight. In addition, the frequent association with premature birth and hypoplasia of the thymus suggested that insufficient development of immunity may result in hepatic CMV involvement in infants. CIB were most frequently observed in hepatocytes in both infants and adults, but in infants they were also frequently seen in the bile duct epithelium. These histopathological findings and the high incidence of jaundice in infant patients suggest that the bile duct is also an important site of CMV proliferation in infants, and that CMV infection may be one cause of infantile jaundice.
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PMID:Clinicopathological study of liver involvement in cytomegalovirus infection in infant autopsy cases. 166 63

The 9-month-old daughter of human immunodeficiency virus (HIV)-seropositive parents presented with cholestasis and was found on liver biopsy to have giant cell hepatitis. No viral inclusions or particles were seen by light or electron microscopy. Ultrastructural studies of the liver biopsy demonstrated tubuloreticular structures in the endothelium and cylindrical confronting cisternae in inflammatory cells in the portal tracts. Serologic studies for hepatitis B, hepatitis A, and Epstein-Barr viruses were negative. Cytomegalovirus (CMV) was cultured from the urine, but buffy coat, nasopharyngeal, and liver cultures were negative and CMV antibody titer was low. The hepatitis responded dramatically to prednisone therapy. A repeat biopsy several months later revealed similar morphologic findings. AIDS was suspected on clinical and immunologic grounds, and was confirmed by the demonstration of HIV-specific IgG and IgM in serum. Five months after initial presentation, the infant developed Pneumocystis pneumonia, disseminated CMV infection, and died. This appears to be the first reported association of infantile giant cell hepatitis with HIV infection.
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PMID:HIV as a cause of giant cell hepatitis. 283 97

A significant cholestasis constellation (alk. phosphatase and GGT each increased to twice as much as the norm) in 73 consecutive patients with AIDS was found in the course of the disease in 29 patients (= 39.7%). In most of the patients advanced immunodeficiency with an average of 2.5 AIDS-defining diseases already existed. In the predominant number of cases it was intrahepatic cholestasis with only slight icterus. Therapeutic measures such as medicamentous treatment or high-caloric parenteral nutrition were causal in two thirds of the cases; in a quarter of the cases opportunistic infections, above all disseminating mycobacteriosis were found. One third of the patients had already existing liver diseases. Liver biopsy frequently proved diagnostic and should be performed more often in the case of unclear liver findings in AIDS patients.
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PMID:[Incidence and differential diagnosis of cholestasis in AIDS--a retrospective analysis of 73 patients]. 748 95

A total of 437 patients with chronic pancreatitis (CP) were examined. The authors detected interstitial or acute, parenchymal or recurrent, hyperplastic or pseudotumorous, and cystic variants in 91 (20.8%), 218 (49.9%), 78 (17.8%), 22 (5.1%), and 28 (6.4%) patients, respectively, Severe types due to the permanent pain syndrome, substantial weight loss and overall intoxication phenomena were seen in 21.5%. Complications of chronic pancreatitis were revealed in 32.3%. Pyoseptic complications (11.2%), anicteric cholestasis (8.5%), subhepatic portal hypertension (8.0%), cholestatic jaundice (7.8%) were most common. Immunodeficiency states developed with long-term treatment of CP. A small portion (0.9%) of patients with CP developed pancreatic carcinoma. 3% of patients had deaths directly due to the active course of the disease whose causes were pyoseptic processes, pancreatic carcinoma and profuse hemorrhages from exulcerations of the duodenal postbulbar part.
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PMID:[Severe and complicated forms of chronic pancreatitis]. 768 83

Several clinical and experimental studies have demonstrated that the high incidence of septic complications and the high mortality of surgery and invasive diagnostic and therapeutic procedures (ERCP and PTC) in obstructive jaundiced patients are mostly secondary to immune impairment (deficit of Kupffer's cells phagocytic activity and of cell-mediated immunity). The fundamental role of endotoxaemia, that's tightly related to stopped defluxion of biliary salts into the bowel, with bacterial flora increase and secondary passage of germs and toxins into portal and systemic circulation, has recently been demonstrated in the pathogenesis of the main homeostatic alterations in cholestasis (immunodeficiency, disorders of coagulation and renal functionality). This pathogenetic hypothesis explains not only high morbidity and mortality rates, but also the failures of external biliary drainage. The only treatment able to oppose endotoxaemia is internal biliary drainage, endoscopic or percutaneous. These techniques allow the defluxion of biliary salts into the bowel and a relatively quick restore of homeostasis. The authors after having treated high-risk jaundiced patients with internal endoscopic biliary drainage and surgical treatment (after 20-30 days), report excellent results.
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PMID:[The immune changes in the patient with obstructive jaundice: their onset and reversibility after drainage]. 774 38

Cell-mediated immunity is impaired during cholestasis, and there is evidence for the involvement of endogenous bile acids. The aim of this study was to evaluate the effects of individual bile acids on immunity and to determine whether monocytes are a target. The effects of bile acids on the procoagulant activity of human monocytes, a lymphocyte-dependent model of monocyte activation, were assessed. Chenodeoxycholic acid, one of the main human primary bile acids, had a concentration-dependent inhibitory effect on procoagulant activity expressed by endotoxin-stimulated mononuclear cells, with half-maximal and maximal inhibitions at 100 and 250 microM, respectively. The inhibitory concentrations were similar for the procoagulant activity of unstimulated mononuclear cells and for endotoxin-stimulated isolated monocytes. In contrast, ursodeoxycholic acid, a bile acid which has beneficial effects in cholestatic diseases, had no significant inhibitory effects at concentrations up to 250 microM. These results indicate that endogenous bile acids tend to inhibit monocyte activation, suggesting a potential role for primary endogenous bile acids in the immune defect associated with cholestasis; ursodeoxycholic acid, which is devoid of effects on the immune system, may potentially reverse cholestasis-induced immunodeficiency.
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PMID:Differential effects of chenodeoxycholic and ursodeoxycholic acids on expression of procoagulant activity by human monocytes. 805 83

A distinct clinical syndrome of cholestasis and hepatitis occurred during early infancy in seven infants with perinatally acquired human immunodeficiency virus 1 infection. In five infants hepatitis was the first manifestation of human immunodeficiency virus 1 infection. The median age of onset of hepatitis was 7 months (range, 5 to 10 months). The mean total bilirubin concentration at presentation was 7.4 mg/dl (range, 3.9 to 11 mg/dl), the mean aspartate aminotransferase was 1512 IU/liter (range, 782 to 2960 IU/liter) and the mean alanine amino-transferase 512 IU/liter (range, 92 to 1247 IU/liter). The absolute CD4 count at the time of onset of hepatitis ranged from 191 to 2298 cells/mm3 (mean, 766 cells/mm3). Six of the seven children died within 12 weeks of onset of hepatitis, three as a result of complications of Pneumocystis carinii pneumonia, and two died of complications secondary to cytomegalovirus. In only one infant was the cause of death the direct consequence of liver failure. The seventh infant died 17 months after the onset of hepatitis of dilated cardiomyopathy. No specific etiologic agent has been identified as the cause of cholestatic hepatitis in these infants. In situ hybridization studies to detect human immunodeficiency virus 1 messenger RNA was negative in the liver tissue obtained at biopsy and autopsy in five of the samples tested.
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PMID:Cholestatic hepatitis in children infected with the human immunodeficiency virus. 810 98

About 60 cases of cholangitis in patients with the acquired immune deficiency syndrome (AIDS) have been described. We report our experience concerning 15 patients infected with the human immunodeficiency virus (HIV) and who had cholangitis as defined by radiological findings. Cholangitis was the revealing event of AIDS or HIV infection in 4 patients (27%). Twelve (80%) of the patients were homosexual men. The main diagnostic features were abdominal pain (73%), cholestasis without jaundice (100%), intestinal cryptosporidiosis (80%) and abnormal findings on abdominal ultrasonography (87%). Endoscopic retrograde cholangiopancreatography appears to be essential for diagnostic and therapeutic purposes, particularly for patients with papillary stenosis who represented 64% of our population. Biological and morphological pancreatic abnormalities were associated in 2 of the 8 patients who underwent retrograde opacification of the Wirsung duct. The microbiological yield was highest in patients who underwent multiple biopsies (duodenal and papillary) and bile sampling. The organisms found included Cryptosporidium (57%) of cases), CMV (28%) and Microsporidia (7%). Twelve-month survival after the diagnosis of cholangitis was only 14% and all deaths were related to AIDS progression. Endoscopic sphincterotomy relieved abdominal pain in 86% of the patients who underwent the procedure. Evaluation of medical treatment, particularly ursodeoxycholic acid, is necessary.
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PMID:AIDS-related cholangitis: diagnostic features and course in 15 patients. 844 17

The case of a man with the acquired immunodeficiency syndrome who had been intravenous drug abuser is reported. He was investigated because of the presence of both severe upper abdominal pain and raised levels of serum alkaline phosphatase and gamma-glutamyltransferase. The endoscopic retrograde cholangiopancreatography show stenosis of the major duodenal papilla associated with thickening and dilatation of intrahepatic bile ducts. The sclerosing cholangitis (SC) diagnostic was made with histological confirmation. Cryptosporidium oocysts were identified in sputum and bronchial lavage. We discuss his SC implication and we review the literature. We highlight the need to suspect this pathology in both positive serology human immunodeficiency patients or AIDS with abdominal pain and biochemical cholestasis.
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PMID:[Sclerosing cholangitis in a patient with acquired immunodeficiency syndrome]. 848 17

Fibrosing cholestatic hepatitis is a well-described syndrome in patients with immunodeficiency and chronic hepatitis B. It is clinically, biologically, and histologically characterized by rapidly progressive hepatic failure, a mildly elevated serum aminotransferase level, an extensive periportal fibrosis associated with intense cholestasis, mild inflammatory cellular infiltrate, no cirrhosis, and a high hepatocellular level expression of B viral antigens. This syndrome reflected a direct hepatocytopathic injury linked to high intrahepatic viral antigen expression. Because the syndrome of fibrosing cholestatic hepatitis has not been described in chronic hepatitis C, we report the first well-characterized case in a renal transplant patient with chronic hepatitis C and discuss the clinical and pathogenic implications of such a syndrome in this setting.
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PMID:Hepatitis C virus-related fibrosing cholestatic hepatitis after renal transplantation. 900 Jun 79


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