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)

A case of non-O:1 Vibrio cholerae bacteremia and prostatic abscess in a patient with idiopathic aplastic anemia was studied, and the data were compared with those from 23 previously reported cases of non-O:1 V. cholerae bacteremia. The case-fatality rate for the 13 cases for which the outcome is known is 61.5%. The majority of known cases have occurred in immunocompromised patients, particularly those with hematologic malignancy or cirrhosis. Host susceptibility is potentially important in this condition. Bacterial products such as a cholera-like toxin and El Tor hemolysin also may play a role in the disease process. The incidence of enteritis due to non-O:1 V. cholerae is unclear because of the methods used for routine stool culture; however, the small number of reported isolates from blood is likely to reflect the infrequency of bacteremia, since Vibrio species are readily identifiable on sheep blood agar. While non-O:1 V. cholerae is sensitive to most antibiotics in vitro, no data are available on antibiotic efficacy.
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PMID:Non-O:1 Vibrio cholerae bacteremia: case report and review. 305 82

A 28-yr-old woman with severe idiopathic aplastic anemia received an HLA-identical mixed lymphocyte culture nonreactive bone marrow transplant from her brother. In the months after successful engraftment, she developed cutaneous and hepatic graft-versus-host disease, associated with marked cholestatic jaundice. Despite a series of therapeutic maneuvers, cholestasis persisted but remained relatively stable over the ensuing 10 yr. However, serial liver biopsies revealed progressive biliary-type fibrosis culminating in cirrhosis. Subsequently, her clinical course deteriorated and she developed signs of hepatic failure, and ultimately died 10.5 yr after bone marrow transplantation. The evolution of chronic graft-versus-host disease to cirrhosis may be a limiting factor in the long-term survival of this group of bone marrow transplant recipients. The lack of correlation between the stable clinical or biochemical indices and the progressive hepatic disease underscores the need for sequential liver biopsies in patients with sustained liver function abnormalities after bone marrow transplantation.
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PMID:Cirrhosis as a consequence of graft-versus-host disease. 353 93

166 aplastic anaemia patients involved in a cooperative study including androgens were followed up for more than 2 years from the beginning of treatment. The mortality rate from aplasia was low (20% of all cases) after 2 years and nil beyond 5 years. The patients who died were either those who failed to improve but survived 2 years or those who late untreated relapse. Five deaths were due to acute leukaemia, but in 3 of these the diagnosis of aplastic anaemia was retrospectively doubtful. Long-term adverse reactions were rare: 2 cases each of benign hepatoma and peliosis; jaundice was frequent, but no cirrhosis was observed. Survivors who had been treated before puberty reached normal weight and height. Six normal pregnancies occurred. Paroxysmal nocturnal haemoglobinuria was noted in 8 patients with uncontrolled disease. After androgen therapy was discontinued 50% of the patients relapsed with equally or less severe symptoms. Relapses were more frequent when androgens were abruptly withdrawn or in cases with incomplete initial improvement. Most relapses responded to androgen treatment. More than 25% of the patients are still androgen-dependent. With or without maintenance androgen therapy, long-term improvement is incomplete in most cases, which suggests residual bone marrow deficiency, as seen in rodents with experimentally induced aplastic anaemia.
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PMID:[Long-term survival of patients with bone marrow aplasia treated with androgen therapy. 166 cases]. 622 31

Hepatic tumors unassociated with cirrhosis were encountered in seven patients aged 10 to 19 years. Four patients had received androgens for aplastic anemia. Two patients had transfusional hemosiderosis. One patient had had a renal transplant 2 1/2 years ago. Two patients are alive at 2 3/4 and 2 1/2 years after surgical resections. Nodules were found at autopsy in the others. The tumors were well differentiated and, in the androgen-related cases, differed from the others in the following features: canalicular bile retention, mild nuclear atypia, and acinar formation. No mitoses, vascular invasion, or metastatic tumor were evident. The clinical setting was variable; different factors, including iron overload and androgen therapy, played a role in the development of tumor. Although the androgen-related cases showed mild cellular atypia, biologic evidence of malignancy was lacking as in most previous reports.
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PMID:Benign hepatocellular tumors in the young. A clinicopathologic spectrum. 632 Jul 60

In 193 cases autopsied between 1945 and 1980, all persons who had been intravascularly injected with Thorotrast in life, the authors found 131 malignant hepatic tumors, 20 liver cirrhoses, 6 myeloid leukemias, 4 erythroleukemias, 5 aplastic anemias, 4 lung cancers, 1 mesothelioma and 1 osteosarcoma. The causes of death in the Thorotrast-administered autopsy group (193 cases) were compared with those of a non-Thorotrast-administered autopsy group (95,000 cases) of the same sex and age at death as recorded in the Annals of Japanese Pathological Autopsy cases from 1958 to 1978. This comparison revealed that the frequencies of malignant hepatic tumors, liver cirrhosis, erythroleukemia, and aplastic anemia were significantly higher in the Thorotrast-administered group than in the non-Thorotrast-administered group.
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PMID:Statistical analysis of Japanese Thorotrast-administered autopsy cases--1980. 657

The patient was a 59-year-old man who had been in hospital suffering from aplastic anemia with transfusion hemosiderosis. Sudden onset of weakness, shaking chills and headache was observed after his staying out overnight on July 25, 1981. His temperature was 39.3 degrees C and he complained of abdominal pain and abdominal distension. His blood pressure dropped to a dangerous level and tonic convulsions that had begun in the upper body gradually extended to the whole body and he died 23 hours after his return. V. vulnificus was isolated by the blood culture performed before death. During his stay away from the hospital, he had eaten raw cuttlefish, which was considered to be the source of infection. V. vulnificus is one of the halophilic marine vibrios and is isolated frequently in summertime from the sea foods and sea water near Japan. It has been disclosed that the presence of underlying diseases such as liver cirrhosis, hemochromatosis can predispose a person to fatal sepsis by V. vulnificus. In this case, besides leukocytopenia, the presence of hemosiderosis induced by many transfusions was considered to be a major cause leading to the fulminating course of the disease.
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PMID:[Fatal Vibrio vulnificus infection in a patient with aplastic anemia]. 667 24

Our experience at the Ramathibodi Hospital with 20 infants and children who had Aeromonas septicemia is reviewed. Their ages were from 1 day to 14 years. Eighteen patients had underlying diseases: leukemia, 5; aplastic anemia, 4; cirrhosis, 2; thalassemia/hemoglobinopathy, 3; renal failure, 1; ileal perforation, 1; marasmus, 1; and cavernous hemangioma with thrombocytopenia, 1. Blood cultures yielded Aeromonas hydrophila in all patients, and four patients had polymicrobial bacteremia. Fifteen episodes of septicemia were community-acquired and five were hospital-acquired. The clinical manifestations of these patients were similar to septicemia due to other Gram-negative enteric bacilli. Two patients each had ecthyma gangrenosum, necrotizing fasciitis and meningitis. Antibiotic treatment included penicillins, cephalosporins, aminoglycosides and sulfamethoxazole-trimethoprim. The overall case fatality rate was 50%; eight of the nine patients with acute leukemia or aplastic anemia died. With the exception of one child the blood cultures were sterile in all patients before death. Aeromonas septicemia is an uncommon but severe infection which occurs predominantly in compromised hosts.
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PMID:Aeromonas septicemia in infants and children. 672 2

In a prospective group study, 254 cases of aplastic anemia treated with a high dose of androgens were followed a minimum of 4 months and up to 8 years. Damage to the liver was evaluated by the appearance of jaundice or abnormal liver function tests; these parameters were evaluated at least every 3 months. Of these patients, 17.3% had overt jaundice and 18.2% abnormal hepatic function tets (total 35.5%). The anomalies appeared rapidly, before month 4 for 50% of the patients and before month 10 for 80%. They were more frequent in men than in women or children, but not linked with previous biological abnormalities. Nor did frequency depend on whether C17 methyl androgen or C17 ethyl androgen was used. Associated corticosteroids offered no protection. None of the patients showed severe jaundice or cirrhosis. If the treatment was stopped, readministration led to relapse of the jaundice in only one-third of the cases. Only two patients in this series revealed a nonmalignant tumor of the liver. Therefore, this complication does not seem to suggest restriction in the use of androgens at high dosage and for long periods, considering the severity of aplastic anemia.
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PMID:[Liver toxicity of androgen therapy in aplastic anemia]. 678 50

Congestive cardiomyopathy and final stage of a liver cirrhosis due to massive overload of iron are often the cause of death in polytransfused patients with aplastic anaemia. With increasing frequency of transfusion the problem of overload of iron becomes more actual. The toxicity of abundant iron is based on various mechanisms of action, which are compiled with the help of literature. Our experiences hitherto made with the intravenous long-term infusion of desferrioxamine are reported. This regimen of therapy is not to be practiced in the long run.
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PMID:[Transfusion induced hypersiderosis in aplastic anemia--physiopathology and therapeutic aspects]. 684 80

Two hundred thousand infants born in Sweden between November 1972 and September 1974 were screened at birth for alpha 1-antitrypsin (alpha 1 AT) deficiency. At age 4 years 172 of 183 children with alpha 1 AT deficiency were examined and compared with 80 randomly selected control children. The children with alpha 1 AT deficiency had the following Pi types: 118 PiZ, 50 PiSZ, 2 PiZ-, 1 PiS-, 1 PiFZ. Two PiZ children have severe liver cirrhosis and 1 PiZ boy had died of aplastic anemia. Abnormal levels of serum alanine aminotransferase (S-ALAT) were found in one PiSZ and 47 PiZ children. Upper and lower respiratory infections, otitis, eczema, urinary infections or complications of child diseases did not occur more often in children with alpha 1 AT deficiency than in controls. More parents of alpha 1 AT deficient children had stopped smoking and their fathers smoked significantly less. Forty parents of children with alpha AT deficiency PiZ answered a questionnaire concerning their reaction to, knowledge about and attitudes towards neonatal screening for alpha 1 AT deficiency. Many parents reported having reacted with lack of understanding, shock or depression upon learning that the child had alpha 1 AT deficiency. About 4 years later 44% reported still lack of understanding, and 18% depression or feelings of guilt. About two-thirds had not fully understood why alpha 1 AT deficiency had been identified, despite the fact that they had seen their doctor 3--4 times for check-ups and counselling since birth.
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PMID:Four-year-old children with alpha 1-antitrypsin deficiency. Clinical follow-up and parental attitudes towards neonatal screening. 697 48


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