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 the first two indigenously acquired cases of melioidosis in Taiwan, diagnosed by positive culture and biochemically identified using the ID 32 GN system (BioMerieux Vitek Inc, Hazelwood, MO, USA). The first patient was a 75-year-old Chinese woman who had not travelled abroad since her arrival from mainland China (San-Tung province) 47 years ago. She presented with spontaneous bacterial peritonitis and hepatitis C-related liver cirrhosis with septic shock. Burkholderia pseudomallei (formerly Pseudomonas pseudomallei) was isolated from cultures of both blood and ascites fluid. The second patient, a 70-year-old Chinese man, presented with right lower lobar pneumonia complicated with empyema and septic shock. Blood cultures grew B. pseudomallei. Both patients had underlying diabetes mellitus; one also had liver cirrhosis and chronic renal failure, while the other had a renal stone. The first patient died of refractory septic shock prior to diagnosis. The second patient survived with the use of intravenous ceftazidime for 30 days, followed by oral amoxicillin-clavulanic acid for a further 3 months. These cases serve as a reminder to clinical physicians that melioidosis is now no longer exclusive to patients with a history of travel to endemic areas. A high index of clinical suspicion is required for early diagnosis and treatment in order to reduce the mortality and improve clinical outcome.
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PMID:Melioidosis: two indigenous cases in Taiwan. 884 Jul 61

Excessive ethanol consumption has been related with the development of liver cirrhosis, as well as with rapid intestinal transit time and diarrhea. Moreover, heavy drinking is associated with an increased incidence of cancer of the oropharynx, larynx, esophagus, and colorectum. Acetaldehyde of microbial origin has recently been suggested as a possible pathogenic factor behind this alcohol-associated gastrointestinal morbidity. The present in vitro study was aimed to investigate alcohol dehydrogenase activity and acetaldehyde formation capacity of some major aerobic bacteria representing the normal colonic flora in man. Cytosolic alcohol dehydrogenase activity and cytosolic protein concentration were determined spectrophotometrically. Alcohol dehydrogenase activity was then calculated as nmoles of reduced substrate produced by milligrams of protein per minute. The ability of different bacteria to produce acetaldehyde was determined by incubating the intact bacterial suspension in closed vials containing ethanol (final concentration 22 mM) for 1 hr at 37 degrees C. The acetaldehyde formed during the incubation was analyzed by headspace gas chromatography. Marked differences in the alcohol dehydrogenase activity and acetaldehyde forming capacity were found among the strains tested. The alcohol dehydrogenase activity varied from 606 +/- 91 nmol/min/mg protein (Escherichia coli IH 50546) to 1 +/- 0.2 nmol/min/mg protein (E. coli IH 50817), and acetaldehyde formation varied from 1,717 +/- 2 nmol acetaldehyde/10(9) colony-forming units (Klebsiella oxytoca IH 35403) to 5 +/- 2 nmol acetaldehyde/10(9) colony-forming units (Pseudomonas aeruginosa ATCC 27853). There was a statistically significant correlation (r = 0.77; p < 0.001) between alcohol dehydrogenase activity and acetaldehyde production from ethanol, strongly suggesting the catalytic role of bacterial alcohol dehydrogenase in this reaction.
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PMID:In vitro alcohol dehydrogenase-mediated acetaldehyde production by aerobic bacteria representing the normal colonic flora in man. 889 13

The incidence of postoperative infections, especially due to multi-drug resistant strains such as Pseudomonas sp., Enterococcus sp., and Methicillin resistant Staphylococcus aureus (MRSA), is high in compromised hosts. Among them, respiratory infection, catheter sepsis, and drug-associated enteritis are frequently observed and respiratory infection is liable to fall into serious illness. These infections have characteristics in causative organisms. Pseudomonas aeruginosa or MRSA are frequently isolated in respiratory infections and Candida or coagulase-negative staphylococcus are frequently isolated in catheter sepsis. G-test in addition to blood culture is necessary for early diagnosis of Candida sepsis, vancomycin should be administered in early phase of antibiotic-associated enteritis, since this infection is usually caused by MRSA or Clostridium difficile and frequently falls into serious illness. The patients with protein-calorie malnutrition, liver cirrhosis, renal failure, diabetes melitis, administration of anticancer drugs and/or radiation therapy, serious injury, or severe operative stress are considered to be compromised hosts in surgical field, and the adequate perioperative managements according to these disorders should be carried out against postoperative infections.
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PMID:[Perioperative managements for postoperative severe infections in compromised host]. 903 82

First rate collaboration between clinicians and research scientists in a multiplicity of fields have brought new hope to patients with cystic fibrosis (CF). The gene, mutations of which give rise to the disease, has been exhaustively mapped, and the functional defects are becoming steadily clearer. Diagnosis is continually being improved and simplified. Neonatal screening has been introduced in many countries and has yielded good results. Promising new advances in treatment include inhalatory DNase (deoxyribonuclease), lung and liver transplantation, UDCA (ursodeoxycholic acid) against cirrhosis, and in vitro fertilisation for men with CF. Pseudomonas species are being combatted more and more effectively with new antibiotics, with immunoglobulins (IgY) for prophylaxis, and possibly new vaccines to come. Future treatment strategies, designed to correct anomalies of cellular biology, are already undergoing clinical trials, and gene therapy using a variety of vectors is undergoing phase-1 trials. A definitive cure remains a realistic hope.
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PMID:[Further clarification of functional issues in cystic fibrosis. Current research and future prospects]. 1047 96

We have studied the characteristics of 202 cystic fibrosis adult patients, all with chronic respiratory symptoms, with a median age of 27 yrs (18 to 55 yrs) and a male predominance (56%). At genetic analysis, delta F508 homozygotes were 41%, delta F508 heterozygotes 42% and 17% had no delta F508. The respiratory disease was more severe and complications were more frequent in adults: hemoptysis in 14%, pneumothorax in 15%, lung transplantation in 25 patients. Chronic bronchial colonisation with Pseudomonas aeruginosa, in 76% of patients, contributed to making treatments more severe because of antibiotic i.v. courses and nebulised antibiotics. Respiratory function showed a mean FVC of 62 +/- 22% and a mean FEVI of 48 +/- 94%. External pancreatic insufficiency was found in 83%, diabetes in 14%. Intestinal occlusion syndromes were observed in 11% of patients and hepatic cirrhosis in 8%. In spite of the severity of the respiratory disease, theses patients succeeded in social and occupational insertion; 62% were independent, 18% had children and 77% were working or studying. Analysis of the patients according to age at diagnosis showed that, in 38 patients diagnosed after the age of 18 yrs, the respiratory disease was less severe, pancreatic insufficiency and non-respiratory complications were less frequent (34% had pancreatic insufficiency, 5% had diabetes and none had cirrhosis). This may partly be due to the presence of milder CFTR mutations. In conclusion, cystic fibrosis in adulthood frequently looks like an evolutive form of cystic fibrosis in childhood. Nevertheless, some late diagnosed forms in adults, with better prognosis, have been recently identified.
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PMID:[Characteristics and specificities of cystic fibrosis in adults: evolutive disease of childhood or recently diagnosed disease?]. 1107 85

The clinical course of patients with cystic fibrosis (CF) with functionally similar mutations in the CF transmembrane conductance regulator gene is variable and must therefore relate to secondary genetic and environmental factors. We examined the hypothesis that polymorphisms of certain inflammatory mediator and regulatory genes affect clinical outcome by influencing the degree of end-organ damage. By studying the possible association between clinical outcome and angiotensin I-converting enzyme (ACE) and cytokine genotypes by amplification refractory mutation system-polymerase chain reaction, using stored DNA from 261 white patients with CF, we found that ultrasound features of cirrhosis occurred more frequently in patients with the high-producer (DD) rather than the low-producer (II) ACE genotype (odds ratio [95% confidence interval], 3.7 [1.2 to 12]). Moreover, significant pulmonary dysfunction (age at which FEV1 < 50%) was associated with the high-producer ACE genotype (2.3 [1.2 to 4.5]) and transforming growth factor-beta1 genotype (2.6 [1.0 to 6.8]) as well as with age at first colonization with Pseudomonas aeruginosa (9.1 [1.1 to 72]). We conclude that the high-producer ACE genotype predicts patients with CF who have an increased chance of developing portal hypertension; and high-producer ACE and TGF-beta1 genotypes are secondary genetic factors contributing to pulmonary dysfunction in these patients.
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PMID:End-organ dysfunction in cystic fibrosis: association with angiotensin I converting enzyme and cytokine gene polymorphisms. 1255 16

The most common hepatic complications of cystic fibrosis (CF) are steatosis, fibrosis, biliary cirrhosis, atretic gallbladder, cholelithiasis, and sclerosing cholangitis. Cholestatic liver disease is a slow progressive disorder, but will stabilize for many patients. CF patients may suffer from the consequences of their liver disease and without liver transplantation, variceal hemorrhage, malnutrition, or end-stage liver disease can lead to death. Prospective data were collected and reviewed on 311 liver transplants performed in 283 patients at the Children's Medical Center of Dallas between October 1984 and November 2000. Ten children received an orthotopic liver transplant (OTLX) for end-stage liver disease associated with cystic fibrosis. Pulmonary function tests were obtained preoperatively in all cases. There were nine boys and one girl. Six are currently alive, and four are dead. Both patient and graft survival was 5.75 yr. Among those currently alive, mean patient and graft survival is 7.71 yr (range 0.10-12.62 yr). Mean patient and graft survival of those who died was 2.35 yr (range 0.78-5.33 yr). No survivor required re-transplantation and currently, all have normal serum aminotransferase values. Chronic sinusitis was not a significant pre- or post-transplant morbidity, although systematic radiographic evaluation of the sinuses did not occur. Pulmonary deaths occurred in three patients from pulmonary hemorrhage, pulmonary infection with Aspergillus and Candida glabrata, and acute bronchopneumonia associated with polymicrobial sepsis because of Pseudomonas, Klebsiella, and Candida albicans 1.44, 0.78, and 1.83 yr, respectively, after transplantation. The fourth death was associated with chronic rejection, and occurred 5.33 yr after transplantation. All non-survivors were below the 5th percentile for height and weight at the time of liver transplantation. Mean age at transplantation was 9.72 yr (range 1.23-19.09, median 9.61). Survivors were transplanted at a younger age than non-survivors (mean of 9.21 yr vs. 10.66 yr), and had shorter waiting times from diagnosis of end-stage liver disease to transplantation (6.87 months vs. 13.83 months). Eighty percentage (n = 8) of patients had pretransplant variceal bleeds (83% of survivors, 75% of non-survivors). While all non-survivors had a history of meconium ileus and preoperative need of pancreatic enzymes, only 67% of those alive experienced these complications. Preoperative forced vital capacity FVC was 103% for survivors and 95% for non-survivors. The corresponding numbers for forced expiratory flow (FEF) 25-75 were 74-84% respectively. Preoperative Aspergillus was identified in 30% of patients (n = 3). Two of these patients are alive. Cystic fibrosis constitutes an indication for 3.5% of pediatric liver transplants. Evaluation and transplantation for end-stage liver disease associated with cystic fibrosis should be undertaken at an early age. Most deaths were associated with pulmonary/septic events, and occurred less than 2 yr after OLTX. Those children who did not survive had poor growth and nutrition, prolonged waiting times prior to transplantation, were transplanted at an older age, and had a higher incidence of pancreatic insufficiency and meconium ileus. The presence of Aspergillus in the sputum does not constitute a contraindication for OLTX.
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PMID:Liver transplantation for cholestasis associated with cystic fibrosis in the pediatric population. 1265 48

One third of patients with cystic fibrosis are adults. The respiratory disease, which conditions the vital prognosis, consists of bronchiectasis with chronic bronchial infection and bursts of acute infection, leading to respiratory insufficiency. Pseudomonas aeruginosa is the most frequent bacteria in adulthood. Exocrine pancreatic insufficiency is associated in 80% of patients. Some complications, such as diabetes, hepatic cirrhosis or osteoporosis, are more frequent in adults. Treatment is based on chest physiotherapy and antibiotics with intravenous courses when there is a colonisation with P. aeruginosa. Pregnancy can be considered in women when respiratory and nutritional status is acceptable. Men are sterile because of congenital bilateral atresia of vas deferens, but can be proposed medical assistance for procreation.
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PMID:[Cystic fibrosis in adults]. 1266 47

Prostatic abscess, though rarely encountered since the introduction of broad-spectrum antibiotics, can cause significant morbidity and mortality. We retrospectively reviewed 17 cases of prostatic abscess treated during an 11-year period at 2 medical centers in southern Taiwan. Most of these patients were elderly (mean age, 59 years) with diabetes mellitus (10 cases, 59%) or hepatic cirrhosis (5 cases, 29%). Fourteen (82%) of the 17 patients were febrile, with chills occurring in about a half of these. Of the symptoms and signs referable to the lower urinary tract, dysuria (71%) was the most common complaint. Pain was usually localized in the suprapubic (35%) or perineal (18%) area. The common findings of digital rectal examination were prostatic enlargement (77%) and fluctuation (23%). Prostatic abscess was impressed from the findings of hypoechoic area with thick walls on transrectal ultrasound or an enlarged gland with fluid-density collections on computed tomography. All causative pathogens were gram-negative bacilli, including Klebsiella pneumoniae (10 cases), Escherichia coli (2), and Pseudomonas aeruginosa (1). Various measures were undertaken to allow drainage, including transurethral incision or resection of the prostate, open perineal incision, laparotomy, and transrectal ultrasound- or computed tomography-guided needle aspiration. In conclusion, K. pneumoniae was the predominant pathogen of prostatic abscess, and was frequently identified as the causative pathogen in patients with diabetes mellitus. Diagnosis of prostatic abscess based merely on symptomatology is implausible, and image studies, such as transrectal ultrasound or computed tomography scan, are warranted. Optimal management includes adequate drainage of abscess and antimicrobial therapy.
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PMID:Prostatic abscess in southern Taiwan: another invasive infection caused predominantly by Klebsiella pneumoniae. 1274 30

In this European study, the phenotype in 68 patients, homozygous or compound heterozygous for the G85E mutation, was investigated. Each index case was compared with two cystic fibrosis (CF) patients from the same clinic, matched for age and sex: one with pancreatic sufficiency (PS) and one with pancreatic insufficiency (PI). When comparing 31 G85E/F508del and F508del/F508del patients, there were no differences in median age at diagnosis, mean sweat chloride value, most recent weight for height, most recent forced expiratory volume in one second % predicted, prevalence of chronic Pseudomonas aeruginosa colonisation and typical CF complications. However, PI was less frequent in the G85E/F508del group. Comparison of 55 G85E patients (with second mutation known and not classified as mild) with PS controls (n=44) showed that the G85E patients had a significantly higher sweat chloride, more often failure to thrive at diagnosis, higher prevalence of PI, worse current weight for height, higher prevalence of chronic P. aeruginosa colonisation and liver cirrhosis. Pulse-chase experiments revealed that G85E cystic fibrosis transmembrane conductance regulator failed to mature on a M470 as well as on a V470 background. Therefore, G85E is a class II mutation. Although there is variability in its clinical presentation, G85E mutation results in a severe phenotype.
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PMID:Genotype/phenotype correlation of the G85E mutation in a large cohort of cystic fibrosis patients. 1517 79


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