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)

We report a 73-year-old woman with homozygous ZZ alpha-1-antitrypsin deficiency (AATD), micronodular cirrhosis, cholestatic jaundice, and emphysema. An elevated SGOT/SGPT ratio was noted in the absence of chronic alcoholism. ERCP demonstrated a normal extrahepatic biliary system and suggested obstruction of the intrahepatic ducts. An operative liver biopsy demonstrated periodic acid-Schiff-positive, diastase resistant intracytoplasmic inclusion bodies. This patient reminds us that metabolic causes of cryptogenic liver disease need to be considered, even in the elderly. We review briefly the literature concerning AATD and liver disease.
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PMID:Alpha-1-antitrypsin deficiency--a cause of cryptogenic liver disease in the elderly. 698 Feb 39

A three year old child with cirrhosis of the liver and ascites caused by alpha-1-antitrypsin deficiency, developed severe abdominal pain with diarrhea and fever. Spontaneous bacterial peritonitis was diagnosed by demonstrating a purulent ascitic fluid with gram-positive cocci in the smear which were identified as pneumococci in the bacterial culture. The peritonitis subsided under antibiotic treatment without complications. Spontaneous bacterial peritonitis in children with cirrhosis of the liver is mentioned in the literature, but up to now, however, only three cases were reported in detail. In order to establish the diagnosis, abdominal tap should be tried rather than explorative laparotomy, the demonstration of gram-positive cocci is diagnostic of spontaneous bacterial peritonitis. With early antibiotic therapy, prognosis of the disease is favourable. Newborns and children with nephrotic syndrome, however, are particularly at risk.
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PMID:[Spontaneous bacterial peritonitis in cirrhosis of the liver caused by alpha-1-antitrypsin deficiency (author's transl)]. 698 Oct 63

Percutaneous needle biopsy specimens of the liver from three elderly persons (aged 77, 71, and 66) demonstrated eosinophilic intracytoplasmic globules within hepatocytes, particularly in the periportal and periseptal areas. These globules were periodic acid-Schiff positive and diastase resistant, and were identified as alpha-1-antitrypsin by immunofluorescence technics. Two of the patients had cirrhosis, and identification of protease inhibitor (Pi) type by acid starch electrophoresis and crossed immunoelectrophoresis demonstrated SZ and MZ genotypes. The patient with SZ genotype also had a long history of chronic obstructive pulmonary disease. Pi-typing was not performed for the third patient, who did not have cirrhosis. The morphologic identification of alpha-1-antitrypsin disease in liver biopsies of persons of any age is important because of (1) possible multisystem involvement (hepatic and pulmonary), (2) increased frequency of hepatocellular carcinoma, and (3) implications for genetic counseling for other family members.
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PMID:Alpha-1-antitrypsin globules in hepatocytes of elderly persons with liver disease. 701 69

One hundred and twenty-four infants admitted to hospitals in Norway between 1955 and 1974 during the first 3 months of life with cholestatic jaundice were studied retrospectively. Sixty-four infants had had extrahepatic atresia of the biliary tree and 60 had had intrahepatic cholestasis. This gives an incidence of about 1:9000 live births for cholestasis. In 4 of the 64 infants with extra-hepatic atresia a bile duct-to-bowel anastomosis had been performed but this was successful in only 2. Sixty of these infants had died by their 2nd birthday. Twenty-six of the infants with intrahepatic cholestasis had died by 1978 and the most common causes of death were cholestasis complicated by infection, bleeding, or hepatoma. The survivors aged between 4 and 23 years were followed up in 1978. In about two-thirds of them aetiological factors--such as alpha-1-antitrypsin deficiency, arteriohepatic dysplasia, cholestasis with lymphoedema--and other familial or genetic factors, or infections were found. Four of the 34 survivors are known to have cirrhosis. Twenty patients had biochemical abnormalities, and 12 had normal liver function tests. Two patients could not be examined. Of the 19 patients with familial or genetic aetiological factors, 4 had cirrhosis, 14 had biochemical abnormalities, and only 5 had normal liver function tests. Of 11 survivors with idiopathic disease or septicaemia, none had cirrhosis and only 4 had abnormal liver function tests.
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PMID:Cholestatic jaundice in infancy. The importance of familial and genetic factors in aetiology and prognosis. 727 1

Liver biopsies were performed in 5 boys aged between 2 and 9 years with severe classical haemophilia who had persistently abnormal liver function tests. Abnormal histology was present in all; 4 had chronic persistent hepatitis and the fifth chronic aggressive hepatitis with early cirrhosis. Evidence of previous hepatitis B infection was present in one patient, 3 had antibodies to hepatitis, A, and 2 had subnormal levels of alpha-1-antitrypsin. Haemobilia occurred as a late complication of biopsy in one. The significance of these findings in young boys is discussed, as is the role of exposure to factor VIII containing blood products. It is concluded that cryoprecipitate should be used in preference to large pool factor VIII concentrates in children with haemophilia.
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PMID:Liver disease complicating severe haemophilia in childhood. 743 4

We investigated 37 patients with ascites and liver cirrhosis in order to examine whether on the basis of correlation of cytokines and acute phase proteins of the ascitic fluid, prognosis of spontaneous bacterial peritonitis can be made. Significantly enhanced levels of interleukin-6, as well as acute phase reactants alpha-1-antitrypsin and C-reactive protein were found in the ascitic fluid of patients with spontaneous bacterial peritonitis. The levels of tumour necrosis factor alpha (TNF-alpha), neopterin, interleukin 2-receptor and granulocyte-macrophage colony stimulating factor were higher in patients with spontaneous bacterial peritonitis, but without statistical significance, whereas no differences were found between the interferon gamma, interleukin-2 and interleukin-1 levels. In addition, interleukin-6, TNF-alpha and neopterin levels were found to correlate significantly with the outcome of the disease. These findings show that acute phase reaction occurs in the ascitic compartment in correlation with the development of spontaneous bacterial peritonitis.
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PMID:Spontaneous bacterial peritonitis is associated with high levels of interleukin-6 and its secondary mediators in ascitic fluid. 751 36

In a prospective study, 102 hospital patients with liver disease were evaluated in West Cameroon, Africa. Blood donors, pregnant women and patients without liver disease served as controls. A total of 757 individuals were tested for markers of hepatitis A, B, C and D and for immunological markers (autoantibodies, procollagen III, alpha-foetoprotein, CA50 antigen, alpha-1-antitrypsin and antibodies to human immunodeficiency virus types 1 and 2). One-third of the liver disease patients had focal lesions on ultrasound examination. Histologically, 20 cases of cirrhosis, 14 cases of chronic hepatitis, 15 hepatocellular carcinomas and 17 cases of acute hepatitis were detected. All hepatic patients and virtually all controls had had a previous hepatitis A virus infection. Over 85% of adult patients and controls had at least one marker of hepatitis B virus infection. Over 30% of patients with liver disease had markers of possible hepatitis B virus replication. Antihepatitis C virus antibody was present in 18% of hepatic patients and in 6% of controls. Hepatitis C virus infection seems to play an important role in the development of chronic liver pathology; 40% of cirrhotic patients had a combined hepatitis B and C virus infection. Serum autoantibodies were frequently found and were not correlated with the presence of autoimmune liver disease.
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PMID:Liver pathology in rural south-west Cameroon. 757 Aug 83

The association between deficiency of alpha-1-antitrypsin (A1AT) and glomerulonephritis has been only sporadically reported on, as opposed to the linkage between A1AT-deficiency and lung emphysema or hepatic cirrhosis. We describe the case of a 30-year-old man with A1AT deficiency who developed hepatic cirrhosis in early childhood, and IgA glomerulonephritis and hypertension in adult life. The IgA nephritis followed an unusual course. After three years of slight elevation of serum creatinine levels, the patient rapidly developed renal failure necessitating acute hemodialysis. The deterioration of the renal function was preceded by eruption of skin lesions, believed to represent a vasculitis. After six months of hemodialysis, the patient successfully received a transplanted kidney from his mother. The literature is reviewed with respect to the association between A1AT-deficiency and renal disease. We discuss possible underlying causes for the rapid deterioration of renal function in this patient.
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PMID:[Alpha 1-antitrypsin deficiency--not only pulmonary and hepatic involvement]. 770 89

Seventy-two long-surviving liver transplant recipients were evaluated prospectively, including a baseline allograft biopsy for weaning off of immunosuppression. Thirteen were removed from candidacy because of chronic rejection (n = 4), hepatitis (n = 2), patient anxiety (n = 5), or lack of cooperation by the local physician (n = 2). The other 59, aged 12-68 years, had stepwise drug weaning with weekly or biweekly monitoring of liver function tests. Their original diagnoses were PBC (n = 9), HCC (n = 1), Wilson's disease (n = 4), hepatitides (n = 15), Laennec's cirrhosis (n = 1), biliary atresia (n = 16), cystic fibrosis (n = 1), hemochromatosis (n = 1), hepatic trauma (n = 1), alpha-1-antitrypsin deficiency (n = 9), and secondary biliary cirrhosis (n = 1). Most of the patients had complications of long-term immunosuppression, of which the most significant were renal dysfunction (n = 8), squamous cell carcinoma (n = 2) or verruca vulgaris of skin (n = 9), osteoporosis and/or arthritis (n = 12), obesity (n = 3), hypertension (n = 11), and opportunistic infections (n = 2). When azathioprine was a third drug, it was stopped first. Otherwise, weaning began with prednisone, using the results of corticotropin stimulation testing as a guide. If adrenal insufficiency was diagnosed, patients reduced to < 5 mg/day prednisone were considered off of steroids. The baseline agents (azathioprine, cyclosporine, or FK506) were then gradually reduced in monthly decrements. Complete weaning was accomplished in 16 patients (27.1%) with 3-19 months drug-free follow-up, is progressing in 28 (47.4%), and failed in 15 (25.4%) without graft losses or demonstrable loss of graft function from the rejections. This and our previous experience with self-weaned and other patients off of immunosuppression indicate that a significant percentage of appropriately selected long-surviving liver recipients can unknowingly achieve drug-free graft acceptance. Such attempts should not be contemplated until 5-10 years posttransplantation and then only with careful case selection, close monitoring, and prompt reinstitution of immunosuppression when necessary.
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PMID:Weaning of immunosuppression in long-term liver transplant recipients. 783 42

The authors measured immunoenzymatically circulating intercellular adhesion molecule-1 (cICAM-1) concentration in 135 patients with liver disease of either viral or toxic etiology: 13 had acute hepatitis; 58 had mild chronic liver disease; and 64 had cirrhosis (superimposed in 30 by hepatocellular carcinoma). Forty patients with extrahepatic diseases (19 with malignancies) and 28 healthy blood donors were tested as controls. One-way analysis of variance demonstrated a significant variability of cICAM-1 concentration among groups (F = 76.67, P < .0001), the highest value being recorded in acute hepatitis (Bonferroni's test for pairwise comparisons, P < .01). Total bilirubin showed a strong correlation with cICAM-1 (R = 0.766, P < .001). By stepwise multiple regression analysis the independent predictors of cICAM-1 concentration were chosen in the following order: total bilirubin; aspartate aminotransferase; cholinesterase; alpha-1-antitrypsin; and immunoglobulins. Thus, in addition to inflammation, cholestasis and decline of functioning hepatic mass may influence cICAM-1 concentration.
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PMID:Circulating intercellular adhesion molecule-1 (cICAM-1) concentration in liver disease. Relationship with cholestasis and functioning hepatic mass. 794 24


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