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)

Indications for liver transplant in acute fulminating hepatitis (AFH) are predominantly affected by the high mortality of this spontaneous evolution (80-100%). At present patients with AFH have priority for transplant since they form part of the 0 emergency group according to the National Transplant Organisation. During the period between 1986 and the end of February 1992, a total of 254 liver transplants were performed in 202 patients (52 retransplants). In 26 patients (12.8%) (16 females and 10 males) the indication was fulminating acute hepatitis. Etiology was unknown in 20 patients, secondary to hepatitis B in 4 and to hepatitis A in 1, and was caused by isonazide ingestion in 1 case. The age limits were 3-60 years (X = 31.5 years). An isogroup graft was performed in 16 patients (61.5%), compatible in 3 (11.6%) and incompatible in 7 (26.9%). Due to anthropometric differences, a partial graft was used in 7 patients (26.9%); in 2 of the latter the graft was taken from the same donor ("split-liver"). Placement was always orthotopic with resection of the retrohepatic vena cava in 25 patients and its preservation in 1 (left lobe of split-liver). Peroperative (30 days) mortality was 23% (6/26); 2 due to cerebral death, 2 due to sepsis, 1 due to multisystemic insufficiency (MSI) and 1 due to acute pancreatitis. Four patients (15.3%) died some time after transplant; 1 after 5 months due to broncho-pulmonary complications, 1 after 7 months due to subacute hepatitis, 1 after 3 months due to respiratory failure and the last after 5 months due to anoxic encephalopathy and lung infection. Ten patients (39.4%) were re-transplanted; 4 following chronic rejection, 4 due to primary graft no function, 1 due to arterial thrombosis and 1 due to recurrent hepatitis (with cirrhosis). Two of the latter patients died intraoperatively due to coagulopathy and hemorrhage, and 3 following surgery (1 due to sepsis, 1 due to respiratory complications and 1 due to respiratory insufficiency). Two patients underwent a second re-transplant (1 due to chronic rejection and 1 due to recurrent hepatitis) and of these 1 died peroperatively due to sepsis and MSF. Overall mortality was therefore 61.5% (16/26) and the actuarial survival rate of 17 patients (10 living + 7 postoperative deaths) was 68% at 12 months and 52.9% at 36 months. Even if peroperative mortality is relatively high, liver transplant is currently the elective treatment for fulminating acute hepatitis.
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PMID:[The treatment of acute liver failure due to fulminating hepatitis by total or partial orthotopic liver transplantation. The clinical results]. 832 33

The clinical pattern and the pathophysiological mechanism of the hereditary disorder alpha 1-antitrypsin deficiency are outlined. It appeared that the patient described suffered not only from emphysema, cirrhosis of the liver and hepatocellular carcinoma, but also from acute haemorrhagic pancreatitis. The combination of acute pancreatitis and alpha 1-antitrypsin deficiency has not been described before. Screening of relatives is important because of possible treatment.
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PMID:[Acute pancreatitis in a patient with alpha 1-antitrypsin deficiency. Is there a causal relationship?]. 841 21

The behavior of plasma von Willebrand factor (vWF) in patients with acute leukemia (n = 5), decompensated cirrhosis (n = 10), and acute pancreatitis (n = 5) was investigated to evaluate whether the systemic proteolytic states associated with these diseases had affected the structure and function of the molecule. vWF antigen and, to a lesser degree, ristocetin cofactor activity in patient plasma were high. Multimeric analysis of plasma vWF revealed loss of high molecular weight multimers. The subunit composition and proteolytic pattern of vWF immunopurified from patient plasmas and reduced were studied by sodium dodecyl sulfate (SDS)-polyacrylamide gel electrophoresis followed by transblotting and probing with monoclonal antibodies that distinguish cleavages caused by plasmin from those caused by other proteases. There was marked reduction of the relative concentration of the native vWF subunit of 225 Kd in all patient groups, indicating heightened cleavage of the protein. The concentrations of 189- and 140-Kd vWF fragments, normally present in plasma, were increased in cirrhosis and pancreatitis but not in leukemia. Novel fragments, ranging in size from less than 225 to approximately 120 Kd were present in leukemia and cirrhosis, including plasmin-generated fragments of 176 and 145 Kd. These data indicate that in clinical conditions in which there is heightened proteolysis vWF is degraded in vivo by plasmin and other proteases. Degraded vWF may be less effective than native vWF in supporting primary hemostasis, thereby being a cofactor in the multifactorial bleeding diathesis accompanying systemic proteolytic states.
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PMID:Degradation of von Willebrand factor in patients with acquired clinical conditions in which there is heightened proteolysis. 842 64

Pancreatic phospholipase A2 (PLA2) is secreted into the pancreatic juice by pancreatic acinar cells as a proenzyme (proPLA2), which is activated by trypsin. Radioimmunoassays with monoclonal antibodies to PLA2 and proPLA2 were used to examine the serum PLA2 and proPLA2 levels simultaneously in patients with various pancreatic diseases. In healthy subjects, proPLA2 proved to be the major form of the enzyme. The serum PLA2 level were found to be significantly increased in patients with acute pancreatitis, the active phase of chronic relapsing pancreatitis, and the early stage of pancreatic cancer. In the terminal stage of pancreatic cancer the serum PLA2 level became low. In patients with chronic pancreatitis, significant correlations were observed between the levels of factors evaluated by the secretin test and the serum total PLA2 and proPLA2 level, but not the PLA2 level. The serum PLA2 and proPLA2 concentrations, and the proportion of proPLA2 in the total, were within normal ranges in patients with liver cirrhosis, hepatocellular carcinoma, and chronic renal failure. These results suggest that simultaneous measurements of serum PLA2 and proPLA2 are clinically useful for diagnosis and monitoring of the active phase of pancreatitis.
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PMID:Simultaneous determinations of pancreatic phospholipase A2 and prophospholipase A2 in various pancreatic diseases. 844 83

Serum pancreatic stone protein (PSP) was determined in sera of pancreatic and nonpancreatic diseases using enzyme immunoassay specific to human PSP to study the diagnostic and pathophysiological significance of PSP. Serum PSP in acute pancreatitis (mean +/- SD = 1075.4 +/- 2849.1 ng/mL, n = 33) was significantly higher than that in controls (78.6 +/- 31.8 ng/mL, n = 37, p < 0.01), chronic pancreatitis (156.8 +/- 82.8 ng/mL, n = 32, p < 0.05), and pancreatic cancer (148.468.8 ng/mL, n = 26, p < 0.05). No significant difference was found between noncalcified and calcified chronic pancreatitis. Serum PSP levels were significantly higher in chronic renal failure under hemodialysis (1796.0 +/- 1492.9 ng/mL) than in other diseases such as peptic ulcer, liver cirrhosis, gallstone, and diabetes mellitus. Low but significant correlation was obtained between serum PSP and serum immunoreactive trypsin (r = 0.22, p < 0.05). Increased serum PSP levels in acute pancreatitis and chronic renal failure suggest that serum PSP levels reflect reflex from pancreatic secretion, release from damaged pancreatic acinar cells, or retention in circulation, and can be useful for diagnosis of acute pancreatitis, but not chronic calcified pancreatitis.
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PMID:Serum pancreatic stone protein in pancreatic diseases. 850 56

An enzyme-linked immunosorbent assay, based on two monoclonal antibodies (Hreg1-1 and Hreg101-1) specific for pancreatic stone protein (PSP)/reg-protein, was developed to determine the concentration of this protein in serum from individuals with various diseases. The serum concentration of PSP/reg-protein was significantly higher in patients with various pancreatic diseases than in normal controls, and was also significantly higher in patients with acute pancreatitis or chronic relapsing pancreatitis than in patients with chronic pancreatitis. Furthermore, the serum PSP/reg-protein concentration was also significantly increased in liver cirrhosis, choledocholithiasis, and various cancers of the digestive system, and was extremely high in all patients tested with chronic renal failure. A significant correlation was apparent between the serum concentration of PSP/reg-protein and elastase-I in 68 patients with chronic pancreatitis or pancreatic cancer. Whereas only 7 of these patients showed a normal serum PSP/reg-protein concentration and a significantly increased elastase-I concentration, 15 of these patients showed a significantly increased serum PSP/reg-protein concentration and a normal serum elastase-I concentration. These results indicate that the serum PSP/reg-protein concentration may reflect pancreatic damage, especially in acute pancreatitis, and may be a sensitive a marker for such damage as elastase-1, although false positivity was apparent in renal failure and in some patients with hepatic dysfunction or digestive system malignancies.
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PMID:Measurement of serum PSP/reg-protein concentration in various diseases with a newly developed enzyme-linked immunosorbent assay. 857 38

Pancreatitis-associated protein I (PAP I) is a secretory protein first described as an acute phase reactant during acute pancreatitis. Recently, induction of the PAP I gene was also described in liver during hepatocarcinogenesis. To investigate the molecular mechanisms of this induction, we used constructs carrying progressive deletions of the PAP I promoter fused to the CAT gene. We showed that the silencer conferring tissue specificity on the PAP I gene was inactive in hepatoma cells. Then, in an vitro transcription system, we compared the transcription capacity of nuclear extracts from normal liver and HepG2 cells on constructs containing the silencer. The results confirmed that a trans-acting factor interacting with the PAP I silencer was present in liver cells and absent from hepatoma cells. On the other hand, immunohistochemistry showed that PAP I was expressed in a limited number of transformed hepatocytes. It was concluded that expression of PAP I in hepatocarcinoma occurred through inactivation of its silencer element and was not concomitant in all malignant cells. On that basis, we assayed PAP I in serum from patients with chronic hepatitis, liver cirrhosis or hepatocarcinoma. PAP I levels were normal in chronic active or persistent hepatitis, significantly higher in cirrhosis and strongly elevated in hepatocarcinoma. Because those clinical entities often develop in that sequence, serum PAP I appeared as a potential marker of hepatocarcinoma development.
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PMID:Mechanism of PAP I gene induction during hepatocarcinogenesis: clinical implications. 895 91

It is still not clear why some alcoholic patients acquire certain organ-specific complications of alcoholism whereas other alcoholic patients acquire different ones. As we know the liver alcohol dehydrogenase (ADH), aldehyde dehydrogenase (ALDH), and cytochrome P4502E1 (P4502E1) are polymorphic at the ADH2, ADH3, and ALDH2 loci and the 5'-flanking region of the P4502E1. The aim of this study was to investigate the differences between Chinese alcoholic patients with cirrhosis and acute pancreatitis by studying the genetic polymorphisms of ADH2, ADH3, ALDH2, and P4502E1. Genotyping of ADH2, ADH3, ALDH2, and P4502E1 was performed using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) methods on peripheral white blood cell DNA from 75 alcoholic cirrhotic patients, 48 acute alcoholic pancreatitis patients, 19 heavy drinkers without liver disease or pancreatitis, and 235 controls. The results showed that the frequencies of the alleles ADH2*1 and ALDH2*1 in the alcoholic cirrhotic patients were significantly higher than those in the nonalcoholic controls. In acute alcoholic pancreatitis patients, only the frequency of allele ALDH2*1, not ADH2*1 was significantly higher than in the nonalcoholic controls. The allele frequency of ADH2*1 in acute pancreatitis patients was significantly lower (P < .01) than in alcoholic cirrhotic patients. The daily amount of alcohol consumption was significantly lower in patients with acute pancreatitis than in patients with cirrhosis (P < .0005). The genotype distributions of P4502E1, detected by RsaI and PstI, were not different among alcoholic cirrhotic patients, alcoholic pancreatitis patients, heavy drinker, and nonalcoholic controls. In conclusion, ALDH2*1 is the most important alcohol metabolizing gene affecting predisposition to alcoholism whereas the ADH2*2 gene may influence susceptibility to acute alcoholic pancreatitis. The patients with alcohol-induced cirrhosis and with alcohol-induced acute pancreatitis are of two different subpopulations.
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PMID:Alcoholism and alcoholic organ damage and genetic polymorphisms of alcohol metabolizing enzymes in Chinese patients. 898 75

Cholelithiasis is a disease of high prevalence in the adult population. Prevalence increases with age; the incidence of complications, such as choledocholithiasis, acute pancreatitis, and cancer of gallbladder, also increase with age. Cholecystectomy has been considered as the gold standard in the treatment of symptomatic or complicated cholelithiasis. Laparoscopic cholecystectomy has become the new gold standard. Our Department of Surgery has adopted a policy of advising laparoscopic cholecystectomy in all patients with symptomatic cholelithiasis, but also subpopulation of high risk asymptomatic patients. This subgroup is made up by patients with long life expectancy, radioopaque stones, small calculus with patent cystic duct, nonfunctioning or calcified gall bladder, and patients with concomitant diabetes, cirrhosis, chronic hemolytic anemia, those that are candidates for kidney or heart transplantation, and those with underling degenerative diseases that are more likely to develop severe complication of cholelithiasis. Csendes of Chile has reported very high incidence of gallbladder cancer in Chile and Bolivia. He considers that cholecystectomy is indicated in asymptomatic patients as a "prophylactic" measure. Our group agrees that this is a valid indication in areas or populations groups where gallbladder cancer is of high prevalence.
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PMID:[Suitability of laparoscopic cholecystectomy in the asymptomatic cholelithiasis patient]. 918 Sep 56

Surgical literature around 1980 has emphasized the technical challenge and the risks of cholecystectomy in cirrhotic patients reporting discouraging results. The aim of this study is the retrospective analysis of laparoscopic cholecystectomy in cirrhotics. The collected laparoscopic experience of 3 surgical groups for the last 5 years is reported. Cirrhotics were classified according to Child-Pugh criteria. Postoperative complications were classified using Clavien's rules. Forty patients were recruited; 31 received successful laparoscopic cholecystectomy. Liver cirrhosis was preoperatively diagnosed in all Child-Pugh B (n = 11) and in 11/20 Child-Pugh A patients. Compared with 989 noncirrhotics undergoing laparoscopic cholecystectomy, cirrhotics were similar in terms of age (59.9+/-10.3 vs. 58.1+/-10.9) and sex (male: 51.6% vs. 50.1%). Acute cholecystitis has a similar frequence in cirrhotics and noncirrhotics (3.2% vs. 4.1%, respectively). Bile duct stones and acute pancreatitis were significantly more frequent in cirrhotic patients (6.4% vs. 3.7%, p < 0.001; and 6.4% vs. 0.3%, p < 0.001, respectively). Endoscopic papillotomy and stone extraction combined with laparoscopic cholecystectomy was performed in 2 patients. Intraoperatively, technical problems occurred in 5 (16.1%) patients: liver bed bleeding (n = 4) was significatively more frequent in cirrhotics vs. noncirrhotics (p < 0.001). Mean operative time was 90 min, range 50-180, and it was not significantly longer than in noncirrhotics (85 min, range 30-200). Conversion rate was also similar (3%). Seven patients presented 8 postoperative complications (Class II): right side lung effusion (n = 2), ascites (n = 2), temporary worsening of Child-Pugh status (n = 2), hyperosmotic coma (n = 1), and umbilical hernia (n = 1). Mean hospital stay in noncomplicated cases was the same for noncirrhotics (3+/-1). The authors suggest a more liberal use of laparoscopic cholecystectomy for symptomatic gallstones in selected Child-Pugh A and B patients.
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PMID:Gallstones in cirrhotics revisited by a laparoscopic view. 944 15


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