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 present the first case report of bilateral renal papillary necrosis developing in a patient on hemodialysis. A 37-year-old hypertensive male with chronic glomerulonephritis had a normal retrograde pyelogram one year prior to initiation of hemodialysis. After two years of maintenance dialysis, he presented with gross hematuria and was found to have extensive bilateral renal papillary necrosis. His only predisposing factor was hepatic cirrhosis. It is postulated that recurrent hypotensive episodes associated with hemodialysis may lead to renal papillary necrosis in predisposed individuals. This diagnosis should be considered in hemodialysis patients who develop hematuria.
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PMID:Renal papillary necrosis in a hemodialysis patient. 851 6

To date, no attempt has been made to study alterations occurring in the amino acid profile in chronic models of thioacetamide-induced liver cirrhosis. In this work, changes in serum amino acids and proteins in rats with thioacetamide-induced liver cirrhosis are reported, together with changes in enzyme activities in the liver and serum. Seventeen female Wistar rats were used. Eight rats were given 300 mg thioacetamide/l in drinking water for 4 months and nine rats were given water ad libitum during the same time-period. Significant increases in glycine, alanine, serine, methionine, glutamate, ornithine, phenylalanine, tyrosine, histidine and proline were observed in rats with the resulting experimental liver cirrhosis. Threonine, taurine, glutamine, lysine and citrulline tended to increase while isoleucine, leucine, aspartate, arginine and tryptophan tended to decrease. Total and nonessential amino acids increased significantly in cirrhotic animals. Total essential and aromatic amino acids tended to increase in the thioacetamide-treated group, whereas branched chain amino acids tended to decrease in the same group. Regarding serum proteins, a decrease in albumin concentration in the thioacetamide-treated animals was the only change detected. The liver enzyme activities under observation (aspartate and alanine aminotransferases, glutamate dehydrogenase and threonine deaminase) were lower in the thioacetamide group. Decreases were significant for both transaminases and threonine deaminase. Results for serum activities showed that transaminases did not change in thioacetamide-treated rats in comparison with controls. In contrast, alkaline phosphatase rose dramatically in cirrhotic rats. We conclude that the serum amino acid pattern in this chronic model of liver cirrhosis resembles in part that of the corresponding human disease.
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PMID:Serum amino acid changes in rats with thioacetamide-induced liver cirrhosis. 857 92

This paper evaluates the role of decreased food intake in protein metabolism in cirrhotic animals by comparing the changes with those observed in pair-fed controls. Rats were injected with [14C]leucine and then divided into 3 groups. Liver cirrhosis was induced in 1 group of rats by repeated intragastric administration of CCl4 in oil over a period of 8 weeks. Control animals were gavaged with oil and either pair-fed or given access to food ad libitum. Three days after the last intragastric dose, rats were injected with [3H]leucine and sacrificed 20 min later. The daily food intake of CCl4 rats declined to 60% of that of the ad libitum controls. Both the pair-fed control group and the cirrhotic group showed decreased body weight gain, and a decline in muscle and intestinal protein degradation. The pair-fed and the cirrhotic groups differed from one another in many metabolic abnormalities. In the cirrhotic group we observed higher levels of serine, asparagine, proline, methionine, tyrosine, phenylalanine, ornithine and histidine, and lower levels of valine, isoleucine and arginine. In these animals higher relative (per kilogram body weight) weights and protein content of the spleen, kidneys and heart were observed. Additionally higher liver weight despite lower protein concentration, as well as lower liver protein degradation and lower skeletal muscle protein synthesis were found.
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PMID:Protein metabolism in cirrhotic rats: effect of dietary restriction. 867 70

A 67-year-old Japanese male, suffering from liver cirrhosis with hepatoma, was admitted to the Yokohama National Hospital because of ascites retention. On physical examination, his abdomen was massively distended with ascites and his lower extremities were edematous. Laboratory findings on admission revealed hypoalbuminemia, moderate icterus, pancytopenia and hepatitis C virus antibody positivity. After admission, abdominal distention and edema were improved with the use of diuretics. On the 15th day of hospitalization, the patient noted diarrhea and bowel movements that occurred 10 times a day. On the following day, his body temperature rose to over 39 degrees C. On the morning of the 17th day, he complained of severe pain in the right lower extremity. Swelling and erythema over his right lower leg were evident. The skin lesion spread rapidly over the knee and became necrotic. His right leg became increasingly swollen with the development of edema and hemorrhagic bullae. About 4 hrs after the emergence of the skin lesion, his blood pressure fell to less than 60 mmHg. Laboratory findings suggested disseminated intravascular coagulation and multiple organ failure due to serious bacterial infection. In spite of vigorous treatment including administration of antibiotics, dopamine, gabexate mesilate and plasma, he did not recover from the state of shock and died about 14 hrs after the appearance of leg pain. Bacterial culture of the blood and contents of the bullae grew a gram negative rod identified as Edwardsiella tarda (E. tarda). Histological findings showed necrotizing fasciitis. E. tarda has recently become recognized as a pathogenic bacteria, particularly in patients with an underlying illness. This is the first reported case of E. tarda septicemia with necrotizing fasciitis.
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PMID:[A fulminating case of Edwardsiella tarda septicemia with necrotizing fasciitis]. 874 15

Here, we describe the clinical course of an adult patient who had living-related liver transplantation (LRLT) from a donor with Gilbert's syndrome and problems of living-related liver transplantation to adult patients. A 22-year-old woman had been diagnosed as having liver cirrhosis at the age of 5. She had undergone devascularization and transsection of the esophagus, and splenectomy for esophageal varices at the age of 8. Complications such as spontaneous bacterial peritonitis and nonspecific colitis sometimes appeared from 17 years old, and icterus and ascites had appeared on all such occasions. Such complications had frequently occurred from 21 years old. Total bilirubin was 5.5 mg/dl and direct bil. was 4.2 mg/dl. The patient and her family members were informed that LRLT might be possible, and they indicated that the patient's 57-year old father was willing to act as the donor. His liver function was examined. His hepatic function was normal except for hyperbilirubinemia (T. Bil. 2.3 mg/dl, D. Bil. 0.3 mg/dl). He was diagnosed with Gilbert's Syndrome because of an increase of unconjugated bilirubin levels during low caloric intake and nicotinic acid test. The standard liver volume for the patient was calculated to be 900 ml on the basis of body weight. Volumetric analysis with computed tomography revealed that the left lobe volume of the donor's liver was 512 ml, corresponding to 56% of the recipient standard liver volume. This proposal was submitted to the ethical committee of Tokushima University School of Medicine and was accepted. In March 28, 1995, the patient underwent LRLT with the donor's left lobe as the graft. The graft weight was 440 g and the graft corresponded to 49% of the recipient's standard liver volume. Volumetric analysis showed rapid enlargement of graft to 683 ml as early as one week after the operation. The donor has returned to work after discharge from the hospital. The recipient is well 11 months after surgery. Total bilirubin was 1.8 mg/dl and D. Bil 0.5 mg/dl. LRLT may become an option for adult recipients, if graft of more than 30% of the recipient standard liver volume is transplanted even from a donor with Gilbert's syndrome.
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PMID:[Problems of living-related partial liver transplantation to adult patients]. 881 56

Type IV glycogenosis (polyglucosan body disease) is a rare congenital autosomal recessive inherited disorder, caused by lack of the branching enzyme (amylo-1,4-1,6 transglucosidase). This deficiency leads to storage of abnormal glycogen (polyglucosan bodies) in the liver and other tissues. The clinical onset of the disease is insidious with non-specific gastrointestinal symptoms followed by progressive hepatic failure. Usually patients die due to hepatic cirrhosis within 4 years. Sometimes myopathy of the heart and skeletal muscle is also present. In these cases, the clinical onset is often later than in typical cases. We report on two brothers with primarily cardiac manifestation and late onset of the disease. The older one started to suffer from progressive dilated cardiomyopathy at the age of 18 years, presenting with severe heart failure, hepatosplenomegaly, ascites and peripheral oedema. He also demonstrated myopathy and muscular atrophy especially of the shoulder and lower limbs. Initially he improved on medical therapy, but one year later severe heart failure recurred followed shortly afterwards by sudden cardiac death. Right heart and skeletal muscle biopsies were performed while he was alive. These, as well as the autopsy, revealed massive accumulation of polyglucosan bodies. In both heart and skeletal muscle, complete branching enzyme deficiency could be proven. His 14-year-old brother showed similar clinical findings of mild dilated cardiomyopathy. His muscle biopsy also revealed polyglucosan body myopathy. Thus, in young patients presenting with congestive cardiomyopathy, type IV glycogenosis has to be considered in the differential diagnosis.
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PMID:A new variant of type IV glycogenosis with primary cardiac manifestation and complete branching enzyme deficiency. In vivo detection by heart muscle biopsy. 888 67

A 60-year-old man with a chronic hepatitis C virus (HCV) infection and histological features of chronic active hepatitis was treated with interferon-alpha (IFN). He successfully responded to IFN with normalization of serum ALT and disappearance of serum HCV-RNA. His liver biochemistry profile remained normal and serum HCV-RNA was not detected throughout the entire follow-up period. One year later, a small hepatocellular carcinoma (HCC) was detected by routine ultrasonographic screening. Laparotomy revealed a small tumour with no metastasis and the non-tumorous liver demonstrated macronodular cirrhosis. Although no space-occupying lesions were detected by frequent radiological examinations prior to IFN therapy, the small size of the tumour suggested de novo development of HCC. Patients with chronic HCV infection, including those who have complete responses to IFN and lack clinical and histological evidence of cirrhosis, should be followed up for the potential development of HCC.
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PMID:Case report: development of hepatocellular carcinoma in a patient with chronic hepatitis C infection after a complete and sustained response to interferon-alpha. 891 34

A case of a 41-year-old man with diarrhea, hypoalbuminemia, and cryptogenic cirrhosis with features of portal hypertension is described. Protein-losing enteropathy was confirmed by analysis of whole-gut lavage fluid, and intestinal inflammation and infection were excluded. Distal duodenal biopsy specimens showed evidence of edematous villi with prominent submucosal vascular and lymphatic vessels. A transjugular intrahepatic portosystemic stent-shunt was inserted, resulting in resolution of both his diarrhea and elevated whole gut lavage fluid protein concentrations. His symptoms recurred and then again improved after shunt thrombosis and parallel shunt placement, respectively. Histological improvement of the villous edema was also noted. This is the first recorded case of protein-losing enteropathy caused by portal hypertension confirmed by successful treatment with transjugular intrahepatic portosystemic stent-shunt.
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PMID:Transjugular intrahepatic portosystemic shunt as a treatment for protein-losing enteropathy caused by portal hypertension. 894 50

We report a 6-week-old boy with meperidine neurotoxicity. What distinguished our patient from those previously reported was his minimal exposure to therapeutic doses of meperidine in the setting of normal renal function, and no history of sickle cell anemia, cancer, hepatitis, or cirrhosis. In addition, our patient had no abnormal changes in the electroencephalogram during the event. After only 2 doses of meperidine, he exhibited acute orofacial dyskinesias consisting of tongue thrusting, lip pursing, and facial grimacing combined with prominent flexion of the arms and stiffening of his legs. However, a normal sucking response remained. His symptoms resolved over the next 36 hours and did not respond to naloxone. We believe that this unique presentation of meperidine-induced neurotoxicity may be due to changes in the basal ganglia resulting from perinatal hypoxemia.
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PMID:Acute neurotoxicity of meperidine in an infant. 896 93

Since plasma is generally employed for amino acid analysis, we compared amino acid levels in plasma with those in serum for healthy individuals and examined the influence of separation and storage conditions on the stability of the samples. Then, we determined the amino acid levels of frozen serum samples obtained from sarin poisoned patients. A. Comparison of Amino Acid Levels in Plasma and Those in Serum Blood was collected from 5 healthy individuals. Then, heparinated plasma and serum were separated by centrifugation immediately after blood collection. Serum was also separated by centrifugation after standing whole blood at room temperature for 1 hour. Frozen plasma and serum were store at -40 degrees C for 5 months. All were subjected to analysis in an amino acid analyzer. It was found that the cystine (Cys) and 3-methyl-histidine (3-M-His) levels in serum and plasma were affected when stored in a frozen state, that the aspartate (Asp) level was changed according to the method of collecting serum, and that the taurine (Tau) and ornithine (Orn) levels were affected by standing blood. B. Analysis of Blood Taken from Sarin Poisoned Patients Twelve sarin poisoned patients were selected as subjects, and serum cholinesterase (Ch-E) and serum albumin (Alb) levels were determined. Amino acid analysis was conducted using an amino acid analyzer. Serum samples which had been obtained from the 6 patients and frozen and stored at -40 degrees C from 5 months were used for amino acid analysis. As a result, the serum Ch-E level decreased and the Alb level tended to rise. Since the Ch-E/Alb ratio was reduced in the sarin poisoned patients, it is considered useful for discrimination from liver cirrhosis in which both Ch-E and Alb levels decreased. Amino acid levels in the serum obtained from the sarin poisoned patients were compared with those of healthy individuals, both of which had been stored under the same conditions. There were significant differences in Asp, glutamate (Glu), phenylalanine (Phe), 3-M-His, glutamine (Gln), and Cys levels. The Glu, Phe, and Gln levels were not affected by storage of serum in a frozen state, while the Glu and Phe levels were elevated and the Gln level was reduced. Although Cys exhibited lower values in frozen serum samples, the Cys level was elevated with a rise in the serum Ch-E levels. Therefore, we deduced that Cys metabolism disorders also occur in sarin poisoning. As stated above, the Glu and Phe levels were elevated and the Gln and Cys levels were reduced, suggesting the presence of abnormal amino acid metabolism, in patients with sarin-poisoning.
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PMID:[Blood amino acid levels in sarin poisoning patients]. 928 31


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