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)

It is well known that incidence of chronic obstructive lung disease in adult patients with alpha 1-antitrypsin deficiency (ATD) is high. Adult carriers of this genetic trait with cirrhosis of the liver, and also with fibrosis of the liver and hepatoma, have been reported. A causal relationship between ATD and liver lesions has been suspected. In most cases liver disease has been recognized at post morten, - in a few cases, however, intra vitam, when severe symptoms of the liver disease had become apparent. The case of a 59 year-old patient is reported with PIZZ-homozygous ATD, moderate pulmonary emphysema and with marked portal fibrosis and focal transition in cirrhosis of the liver without any sequelae. The clinical course has been rather benign so far.
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PMID:[Alpha 1-antitrypsin deficiency, liver cirrhosis and pulmonary emphysema (author's transl)]. 16 Apr 81

The enzyme gamma-glutamyl transpeptidase is widely distributed throughout the body, notably kidney, seminal vesicles, pancreas, liver, spleen and brain. Being one of the enzymes of the gamma-glutamyl cycle, it is involved in aminoacid transport, catalysing a transpeptidation reaction between gamma-glutamyl peptides and most common amino acids. Methods of assay of the enzyme are based on its ability also to act on synthetic amides of glutamic acid; kinetic methods monitoring the release of p-nitroaniline from the substrate L-gamma-glutamyl p-nitroanilide are the most satisfactory. In diseases of the liver, the highest levels occur in association with cirrhosis, alcoholism, hepatic secondaries and cholestasis. As the enzyme is present in the endoplasmic reticulum of the hepatocyte, its activity is increased in situations leading to microsomal enzyme induction. Raised levels can also occur in pancreatitis, diabetes, myocardial infarction, congestive cardiac failure, chronic renal failure, cerebrovascular accidents, cerebral tumours and chronic obstructive pulmonary disease. Although the lack of specificity must be recognised, the estimation can be useful in the elucidation of some clearly defined problems arising during investigation of patients with suspected hepatic disease, especially where performed as part of a biochemical profile.
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PMID:Role of gamma-glutamyl transpeptidase activity in the diagnosis of hepatobiliary disease. 24 76

Clinical data from 246 adult Swedish individuals with severe alpha1-antitrypsin deficiency, Pi Z, diagnosed in 1963--77, were analyzed. Primary emphysema was present in 109 cases. Of 75 Pi Z patients with other types of chronic obstructive pulmonary disease (COPD), all but 7 showed signs of emphysema. Median age at onset of dyspnoea in Pi Z smokers was 40 years, compared to 53 in non-smokers (p less than 0.001). Of the Pi Z individuals over the age of 50, 19% had a diagnosis of liver cirrhosis and 15% signs of glomerular renal damage. Of 91 deceased patients, 56 died from COPD and 12 from liver disease. A greatly reduced survival was demonstrated in Pi Z individuals, regardless of sex. Smoking Pi Z individuals had a significantly lower life expectancy than Pi Z non-smokers (p less than 0.01).
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PMID:Natural history and life expectancy in severe alpha1-antitrypsin deficiency, Pi Z. 30 8

The respiratory function in 24 patients with liver cirrhosis (21 of them with ascites) was examined. A moderate hypoxemia was established as well as hypocapnia and high alveolar-aterial gradient for O2 pressure. The rest of the indices for gas and blood distribution in lungs do not reveal considerable deviations from the norm. The results obtained provide grounds that the manifested alveolar-arterial gradient for O2 pressure and the light to moderate hypoxemia in advanced liver cirrhosis are due mainly to increased right-left shunt and probably to increased number of alveoli with the ventilation/profusion ration equal to zero. The pathogenetic mechanism of the disturbances of gas metabolism in liver cirrhosis differ from that in chronic obstructive lung disease, characterized by manifested heterogenicity of the ventilation/perfusion raton.
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PMID:[Observations of respiratory function in advanced liver cirrhosis]. 93 91

Alpha-1-antitrypsin is a glycoprotein in human serum that inhibits several proteases. It is a polymorphic protein. A single autosomal locus (Pi), with multiple codominant alleles is responsible of the synthesis of alpha-1-antitrypsin. Of particular interest are alleles that lead to lower than normal concentrations of alpha-1-antitrypsin in serum, namely, PiS, PiP and PiZ. Some of these subjects carry a high risk of developing chronic obstructive pulmonary disease, especially when they are homozygotes for PiZ. In children, cirrhosis of the liver are also found in association with homozygosity for PiZ. recently, TALAMO discovered a subject whose serum contained no alpha-1-antitrypsin; this was the first case of total deficiency, and the patient carried a double dose of the so-called Pi--allele (Pi nul). We were able to demonstrate that a single dose of this allele exists in three families which we have studied in this paper. In a fourth family, the propositus carries Pi-- in duplicate. We report here the second case of the strange homozygous phenotype, Pi--. Surprisingly, we have found that alpha-1-antitrypsin is not completely absent in this serum; its concentration is 200 times lower than normal (less than 10 microgrammes per ml). At the moment, the existence of the Pi-- allele is obvious, but the significance of this small quantity of alpha-1-antitrypsin in the serum of such a patient remains unknown.
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PMID:[Deficiency of alpha-1-antitrypsin and the allele Pi nul]. 108 63

In order to clarify the role of free fatty acid (FFA) in thyroid hormone abnormalities in patients with nonthyroidal illness, thyroid function, FFA, inhibitor of extrathyroidal conversion of T4 to T3 (IEC) and thyroid hormone binding inhibitor (THBI) were studied in 99 patients with various nonthyroidal illnesses including diabetes mellitus (DM) (n = 35), liver cirrhosis (LC) (n = 33), chronic obstructive pulmonary disease (COPD) (n = 17) and chronic heart failure (CHF) (n = 14). Patients were divided into three groups based on the level of serum T3: Group I (T3 < 50 ng/dl), Group II (50 < or = T3 < 80) and Group III (80 < or = T3). Serum T4, FT3 and the T3/T4 ratio decreased significantly in the order Group III, Group II and Group I (Group III > II > I). The plasma FFA level was 0.91 +/- 0.12 mmol/l in Group I (P < 0.05, vs. Group III), 0.65 +/- 0.06 in Group II and 0.54 +/- 0.04 in Group III, respectively. The incidence of positive IEC was 80.0% in Group I (P < 0.05, vs. Group III), 53.7% in Group II (P < 0.05, vs. Group III) and 34.2% in Group III. However, IEC was not correlated with the serum T3 concentration. The incidence of positive THBI was 80% in Group I (P < 0.05, vs. Group III), 68.3% in Group II and 47.4% in Group III, but THBI was not correlated with the serum T4 level. Positive correlations were observed among FFA, IEC and THBI (P < 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Plasma free fatty acids, inhibitor of extrathyroidal conversion of T4 to T3 and thyroid hormone binding inhibitor in patients with various nonthyroidal illnesses. 147 85

Our aim was to analyze the predictive value of a variety of preoperative risk factors on operative outcomes. We reviewed all colorectal resections performed in a single hospital between January 1985 and May 1990. Nine hundred seventy-two resections were performed on 825 patients. We studied 17 preoperative risk factors generated from various medical risk categories. Using the multivariate discriminant function analysis, we calculated that 11 of the 17 risks were of significance in predicting outcomes (all with P less than or equal to 0.031). These factors included emergent operation, age greater than or equal to 75 years, congestive heart failure (CHF), prior abdominal or pelvic radiation therapy, corticosteroid use, albumin less than 2.7 g/dl, chronic obstructive pulmonary disease (COPD), previous myocardial infarction (MI), diabetes, cirrhosis, and renal insufficiency. The classification function generated by the discriminant analysis was used to categorize patients into one of four risk groups depending on their "risk score." The index used to develop each patient's "risk score" ranged from six points for an emergency operation to one point for diabetes. The mortality rates for the various risk groups were as follows: Group 1, zero to four points, 1 percent; Group 2, five to eight points, 10 percent; Group 3, 9 to 13 points, 19 percent; Group 4, greater than 13 points, 33 percent. In contrast to previous reports, we showed that age greater than or equal to 75 years alone is not a major preoperative risk factor but, rather, acts as a modifier for the other predictors of postoperative complications. We then assessed clinical questions concerning specific preoperative risks, such as steroid use, obesity, inflammatory bowel disease, COPD, and prior laparotomy, and their associated specific postoperative complications and have developed prevention strategies based on these findings. Through the use of the risk index, we also were able to assess an individual patient's operative risk more accurately.
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PMID:Multifactorial index of preoperative risk factors in colon resections. 173 12

In 1901, 20% of autopsied subjects in Trieste were under the age of 30 and 28.8% were over 70. By 1985, only 0.2% were under 30 years of age and 74.5% over 70. An analysis of autopsy reports for 1901 reveals that the primary causes of death at that time were tuberculosis (22.4%), acute pulmonary infections (13.7%) and malignant neoplasms (10.6%). Other pathological conditions found at autopsy were infectious lesions (10.4%), chronic obstructive pulmonary disease (10.2%), arteriosclerosis (only 6.4%), syphilis (4.7%), nutritional deficiency (4.7%), cirrhosis of the liver (4.6%) and acute infections (1.1%). Overall, infectious diseases accounted for 55% of deaths in 1901. In 1985, the cause of death was infection in only 3.7% of cases. During the period analysed, the percentage of deaths from cancer tripled and mean length of survival increased by more than 20 years. In 1901, the neoplasms found most frequently were gastric cancer in males (17.9%) and cancers of the uterus and ovary in females (both 13%). Lung cancer accounted for 7.7% of all deaths from malignant neoplasms in males, and breast cancer for 10.8% of such deaths among females. By 1985, lung cancer accounted for 32.4% of deaths from malignant neoplasms among males and breast cancer for 18% among females. Between 1901 and 1985, there were highly significant increases in the numbers of deaths due to arteriosclerosis and to malignant neoplasms in people of each sex.
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PMID:Changes in underlying causes of death during 85 years of autopsy practice in Trieste. 185 46

A case-control design was used to investigate the effects of preexisting chronic conditions on in-hospital mortality in adult trauma patients. Cases consisted of all trauma deaths (n = 3074) that occurred in 1983 in any of the 331 acute care hospitals in California. Three to four control patients (injured survivors) were matched to each case patient on the basis of injury severity, age, and individual hospital (n = 9869). The data source consisted of hospital discharge abstract data uniformly collected on all admissions to acute care hospitals in the state. Conditional logistic regression techniques were used to estimate the relative odds of dying for patients with and without one or more of 11 preexisting chronic conditions identified as potentially detrimental to outcome. The presence of cirrhosis (relative odds = 4.5), congenital coagulopathy (relative odds = 3.2), ischemic heart disease (relative odds = 1.8), chronic obstructive pulmonary disease (relative odds = 1.8), and diabetes (relative odds = 1.2) all significantly increased the risk of dying. These data provide statistical evidence to support the recommendation of the American College of Surgeons that the presence of underlying disease be considered in decisions to triage and transfer patients to trauma centers. They also underscore the importance of underlying disease in the case-mix adjustment of case-fatality rates and the identification of unexpected deaths for quality assurance review.
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PMID:The effect of preexisting conditions on mortality in trauma patients. 231 71

Red lunulae are associated with rheumatoid arthritis, systemic lupus erythematosus, alopecia areata, cardiac failure, hepatic cirrhosis, lymphogranuloma venereum, psoriasis, carbon monoxide poisoning, twenty-nail dystrophy, and reticulosarcoma. We examined four patients with red lunulae. Three had chronic obstructive pulmonary disease. Two of these three were alcohol abusers and were without any of the conditions previously associated with red lunulae. Two of the four also had palmar erythema. Histopathologic examination of the red lunula in one of the four cases did not show signs of neovascularization. We report our findings in these patients, which suggest that red lunulae result from increased arteriolar blood flow, a vasodilatory capacitance phenomenon, or changes in the optical properties of the overlying nail so that normal blood vessels become more apparent.
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PMID:Red lunulae revisited: a clinical and histopathologic examination. 264 22


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