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)

Cystic fibrosis (CF) is the commonest, fatal, autosomal recessive disorder and is associated with lung sepsis, pancreatic failure and elevated sweat electrolytes. The CF gene on chromosome 7 encodes a protein identified as CF transmembrane conductance regulator (CFTR) which regulates chloride ion transport in epithelial cell membranes. Almost 100 mutations have been identified in this gene which cause defective chloride-channel control. Recently, this abnormality has been reversed in affected CF cells in vitro by retrovirus-mediated transfer of a normal gene. Fifty years ago, most cases died in childhood, but now up to 80% reach adulthood. Chronic lung sepsis is the principal cause of death, and intensive antibiotic therapy with chest physiotherapy is used to control this. Advanced lung disease can be successfully treated by heart-lung transplantation. Nebulised recombinant DNase and antineutrophil elastase agents such as alpha-1-antitrypsin and secretory leucoprotease inhibitor are potentially promising new therapies. Pancreatic insufficiency is managed by high-calorie diets and enteric coated enzyme supplements. Other prominent gastrointestinal complications include meconium ileus equivalent, biliary cirrhosis and cholelithiasis. Specially dedicated CF centres have led to improved survival rates and allow experienced staff to treat the many complications of CF while promoting research in this multisystem disorder.
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PMID:Cystic fibrosis in adolescents and adults. The coming of age of cystic fibrosis. 155 Dec 44

Galactosemia in newborns and infants is associated with the following symptoms: jaundice, hepatomegaly, failure to thrive, feeding difficulties, hypoglycemia, convulsions, lethargy, amino-aciduria, cataracts, hepatic cirrhosis, ascites, and mental retardation. If the preliminary evaluation indicates galactosemia, there is high risk for E. coli sepsis and death. Strong consideration should therefore be given for early antibiotic therapy in infants with suspected galactosemia in spite of the absence of clinical signs or symptoms of sepsis.
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PMID:Association of Escherichia coli sepsis and galactosemia in neonates. 156 28

We have analyzed the indications and results of shunt operation versus orthotopic liver transplantation (OLT) in 22 patients with Budd-Chiari syndrome (BCS). The underlying cause of the syndrome was similar between the two groups and was related to myeloproliferative disorders or the use of birth control pills in 18 of 22 patients. The results of biopsies of the liver showed centrilobular congestion and necrosis in all candidates who underwent shunting and the presence of fibrosis and cirrhosis in the OLT candidates. The indications for shunts included symptoms related to portal hypertension only and well-preserved synthetic hepatic function. Ten patients were treated with 12 shunt procedures, including mesoatrial (eight patients) and side to side portacaval shunt (four patients). Significant complications after shunt procedure included fulminant (one of ten patients) and progressive (one of ten patients) hepatic failure requiring urgent OLT; one death occurred because of pulmonary sepsis. Indications for OLT were signs of end stage liver expressed by severe portal hypertension and variceal bleeding (four of 14 patients), progressive encephalopathy (seven of 14 patients) and poor synthetic function (bilirubin greater than 3 milligrams per deciliter in eight of 14 patients and albumin less than 3.0 grams per liter, or both, in ten of 14 patients). Fourteen patients were treated with 16 OLT, three patients had retransplantation for primary nonfunction graft (two of 14 patients) or chronic rejection (one of 14 patients). There were two early deaths in the group. With a follow-up period between two months to five years, 12 of 14 patients undergoing OLT are alive, fully functional and have normal liver function tests. Seven of ten patients who had shunts are alive, six are able to maintain normal activity and one has progressive end stage hepatic disease and is not a candidate for OLT. However, the hepatic function continues progressively to be abnormal. Various options are available for the treatment of the syndrome. Portosystemic decompression is effective and should be considered at the early stage of the disease, prior to the development of significant hepatic failure. However, few of the patients will continue to have slow, but progressive hepatic failure and may require OLT. The only effective treatment for end stage hepatic disease secondary to the BCS is OLT.
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PMID:Portosystemic shunt versus orthotopic liver transplantation for the Budd-Chiari syndrome. 159 20

One hundred and fifty-eight elective hepatectomies carried out between 1976 and 1989 for hepatocellular carcinoma were classified into three groups according to age: group I (n = 20) under 30 years of age; group II (n = 102) 30 to 60; and group III (n = 36) over 60. The number of operations carried out on patients in group III has increased since 1983, and the types of hepatic resection in the three different age groups were slightly but not significantly different. The incidence of small tumours (diameter less than 5 cm) and associated cirrhosis were relatively low in the younger patients. Postoperative complications developed in 4 patients (20%) in group I, 18 (18%) in group II and 8 (22%) in group III, the main ones being hepatic failure and intraabdominal sepsis. Operative mortality was 4%; one patient (5%) died in group I, 4 (4%) in group II, and 2 (6%) in group III, and the principal causes were hepatic failure and massive haemorrhage. Hepatic resection for hepatocellular carcinoma in most patients over 60 years old was associated with slightly higher operative morbidity and mortality, but the risks were such that we recommend that operation should not be denied to selected patients in this age group.
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PMID:Influence of age on results of resection of hepatocellular carcinoma. 168 50

A diagnostic agent for enzyme immunoassay of ceruloplasmin has been developed. The method is highly sensitive and specific. The minimal detectable amount of ceruloplasmin is 25 ng/ml, variance coefficients with 1 lot being 2.6-7.3 percent and 4.2-10.8 percent within 3 lots. Blood serum ceruloplasmin concentration in health has made up 0.9 to 1.3 mg/ml. Ceruloplasmin levels were regularly shifted in Wilson's disease, cerebral lymphomas, sepsis, injuries, liver cirrhosis and other diseases.
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PMID:[A commercial immunoenzyme reagent for the determination of ceruloplasmin]. 171 23

Two fatal sepsis cases in two male patients (58 and 14 years old) due to Vibrio cholerae non 01 are described. Their original diseases were hepatic cirrhosis and acute lymphoblastic leukemia in its third complete remission. In this last case, gastroenteritis due to V. cholerae non 01 was also diagnosed. These sepsis presented a rapid evolution and positive hemoculture after 24 and 48 hours of incubation. Both strains isolated presented similar biochemical characteristics and did not agglutinate in the presence of the specific serum against V. cholerae. Both strains were susceptible to most of the antibiotics available. Sepsis due to V. cholerae non 01 is usually associated to other original diseases and to immunodepression. Management of these sepsis is difficult and mortality rates are very high.
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PMID:[Sepsis due to Vibrio cholerae no 01]. 182 May 1

Serum androgen levels were studied in 100 patients (50 male) with varying degrees of severe illness, determined by Acute Physiological and Chronic Health Evaluation (APACHE). Comparison with normal subjects revealed the following changes: (1) Basal dehydroepiandrosterone sulphate (DHEAS) values were decreased in the ill female patients (P less than 0.001) as well as in the ill males (two groups, P less than 0.01; P less than 0.05). Androstenedione values did not differ from the controls in patients of either sex. Basal testosterone levels were decreased in ill male patients (P less than 0.001), but not in females. (2) The low testosterone concentrations in the severely ill male patients correlated inversely with the APACHE score; additionally, a dependence on diagnostic categories could be demonstrated in men, since the lowest values were found in patients suffering from sepsis or liver cirrhosis. Acutely ill males had a moderately decreased testosterone, whereas chronically ill males showed a marked reduction of testosterone compared to the controls. Lowered DHEAS and androstenedione levels could be measured in chronically ill males but not in ill females. (3) 17 alpha-OH-progesterone and 17 alpha-OH-pregnenolone levels in subgroups of the patients suggested a probable enzymatic block in the delta 5-pathway of androgen biosynthesis in severe illness. The ratio of 17 alpha-OH-pregnenolone to DHEAS was significantly higher in male patients and tended to be high in ill females, whereas the ratio of 17 alpha-OH-progesterone to androstenedione showed no difference between healthy and ill subjects.
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PMID:Serum androgens in intensive-care patients: correlations with clinical findings. 183 39

Altered metabolism has been shown to exist in the settings of surgical stress, cancer, cirrhosis, sepsis, and trauma. Each condition is characterized by varying degrees of alteration in metabolic processes, and within a given patient, these metabolic alterations will change as the patient's status changes. Nutrition support is an integral part of the metabolic management of critically ill patients. Metabolic changes impact nutritional substrate requirements and utilization. As the patient's clinical condition deteriorates, clinical signs and symptoms become less reliable in predicting or assessing the existing physiologic state. Objective measurements are needed to define the metabolic status during these physiologic changes. The purpose of this article is to review selected indices that have been used to identify abnormalities in nutritional substrate metabolism. Although some of these tests are readily available and inexpensive, many have not been used outside of the research setting and, therefore, their clinical utility has yet to be determined. However, their use as research tools for defining metabolism warrants their inclusion in order to assist the clinician in interpreting research studies. The biochemical markers discussed include glucose, lactate, pyruvate, triglycerides, beta-hydroxybutyrate, acetoacetate, urinary nitrogen, acute phase proteins, visceral proteins, 3-methylhistidine, plasma amino acids, oxygen consumption, and resting energy expenditure. Each marker is defined in terms of its biochemical significance, and the literature describing changes that occur in various stress states is cited.
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PMID:Overview of biochemical markers used for nutrition support. 190 7

Sixteen patients with massive bowel resection receiving long-term home total parenteral nutrition (HTPN) for 31 to 145 months were reviewed for evidence of liver disease. Patients were divided into three groups: group 1 with duodenocolostomy (n = 3), group 2 with an estimated 15-43 cm residual small bowel (n = 7), and group 3 with an estimated 55-120 cm residual small bowel (n = 6). Two patients in group 1 developed liver cirrhosis; one was diabetic and died of sepsis and liver failure at the 88th month on HTPN; the other died of lung cancer at the 46th month on HTPN. The third patient, followed for 33 months, had transient severe liver function abnormalities associated with a blood transfusion. In groups 2 and 3, only one patient (with a history of probable liver disease before HTPN) developed biopsy-proven cirrhosis at the 60th month of HTPN. All four patients with clinically apparent liver disease developed persistent elevation of serum aspartate aminotransferase (AST) early in HTPN. Four other patients (all in group 3) with abnormal AST values in the early phase of HTPN normalized them later; they did not develop clinical liver disease over a mean follow-up time of 110 months (range, 39-152). None of the remaining eight patients (seven in group 2 and one in group 3) had significant liver function test abnormalities and none developed clinical liver disease over a mean follow-up period of 72 months (range, 39-120).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Liver dysfunction associated with long-term total parenteral nutrition in patients with massive bowel resection. 190 76

Among patients examined at the Central Laboratory of Yokohama City University Hospital over the 25 years from 1965 to 1989, those whose clinical samples showed Cryptococcus were studied in greater detail. The following findings were obtained. Of 16 patients who were found to have cryptococcosis, 14 (87.5%) were treated at the department of internal medicine, and one each at the departments of neurosurgery and dermatology. A study of these patients in terms of clinical type revealed 10 patients (62.5%) with meningitis, two with pneumonia and one with sepsis. The remaining three patients had complicated diseases: meningitis with sepsis, pneumonia with cutaneous cryptococcosis, or pleuritis with sepsis. Underlying disease, including liver cirrhosis, leukemia, multiple myeloma, malignant lymphoma and collagen disease, was found in 6 patients (37.5%), who were all from the department of internal medicine. All patients but one were given antimycotic agents. They were treated by a combination therapy except for three patients who received single amphotericin B (AMPH) therapy. The most frequent combination was AMPH + 5-flucytosine (5-FC), which was found in 7 cases. Seven patients (43.4%) died, three males and four females. Analysis of these cases in terms of clinical type revealed meningitis in four, and pneumonia, sepsis, or pleuritis complicated with sepsis in the remaining three patients. Four patients (57.1%) had underlying diseases. In addition, eleven strains isolated from the specimens were examined for serotypes and minimum inhibitory concentration (MIC) using three types of antimycotic agents. Serotypes of Cryptococcus neoformans were all A and the MIC was 0.1-0.39 micrograms/ml for AMPH, 0.05-0.2 micrograms/ml for 5-FC and 0.2-0.78 micrograms/ml for miconazole (MCZ).
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PMID:[Mycological and clinical study of cryptococcosis in Yokohama City University Hospital during the period from 1965 to 1989]. 207 57


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