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)

Fibrinogen glue of human origin was used in 44 patients. Indications are of two types: as a glue mainly for dermoepidermoid grafts but also to assist mucosal suturing and bone obturation, and for hemostasis after extractions in patients with coagulation disorders (hemophilia, cirrhosis, leukemia). Positive results were obtained, particularly in hemophilic patients, the incidence and severity of secondary hemorrhage appearing to diminish.
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PMID:[Value of Tissucol in dentistry and maxillofacial surgery]. 387 92

Hepatic histologic materials (biopsy or autopsy) and associated clinical data from 155 hemophiliacs were collected by an ad hoc hemophilia study group and analyzed retrospectively in an effort to determine the spectrum of liver disease in this population and to examine the relationship between the severity of liver disease and treatment history. Clinical information on the frequency of complications from 126 biopsies in 115 hemophilic patients provided a unique opportunity to assess the safety of liver biopsy in such patients. The incidence of cirrhosis (15%) and chronic active hepatitis (7%) was lower than previously reported. The frequency of severe liver disease (chronic active hepatitis or cirrhosis) in patients receiving large pooled concentrates was no greater than in patients treated principally with cryoprecipitate or plasma. The risks of liver biopsy in this setting are relatively high: clinically significant hemorrhage followed 12.5% of the procedures.
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PMID:A study of liver biopsies and liver disease among hemophiliacs. 392 25

Delta antigen (delta) is a transmissible agent requiring hepatitis B virus (HBV) for its replication. Antibody to delta (anti-delta) was present in nine of 71 (13%) British HBV carriers: six were intravenous drug abusers and two were haemophiliacs. Anti-delta was negative in 30 HBsAg positive homosexuals. Cirrhosis was common in patients with anti-delta and those with anti-delta positive cirrhosis were significantly younger than those with anti-delta negative cirrhosis. In British HBV carriers delta infection is associated with intravenous drug abuse and haemophilia and perhaps a more rapid progression of chronic liver disease.
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PMID:Significance of delta agent infection in chronic hepatitis B virus infection: a study in British carriers. 662 17

The modern, comprehensive care of patients with hemophilia requires an awareness that complications other than those caused by acute hemorrhage can occur. The use of newer, more potent plasma concentrates has been accompanied by an increased incidence of liver disease in transfusion-requiring hemophiliacs. The progression to chronic active hepatitis and cirrhosis are particularly ominous developments in these patients. There is also a high incidence of urinary tract abnormalities in hemophiliacs, though the long-term consequences of these abnormalities are unknown. Furthermore, it must be remembered that urinary tract disorders unrelated to hemorrhage, such as nephrolithiasis, tumors, and nephritis, can occur in patients with hemophilia and may be mistaken for hemorrhage. Finally, hypertension occurs more frequently in patients with hemophilia than in the general population and may in part contribute to the occurrence of bleeding within the central nervous system. Methods for evaluating and treating these various disorders are discussed. Greater awareness of these potentially treatable medical complications will improve further the quality of care in hemophilia.
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PMID:Medical complications of hemophilia. 676 70

Chronic liver disease has become a significant complication of the therapy of hemophilia disorders. We describe two patients with hemophilia A and hepatitis B virus hepatitis who progressed to cirrhosis with bleeding esophageal varices. Each underwent distal splenorenal shunt under plasma concentrate therapy without difficulty. One patient died 19 months after operation and unsuspected hepatocellular carcinoma was found at autopsy. These cases illustrate the potential severity of liver disease in hemophilia and the ability to safely perform surgery for portal hypertension if required.
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PMID:Cirrhosis, variceal bleeding, and distal splenorenal shunt in hemophilia A. 712 25

Liver biopsies were performed in 5 boys aged between 2 and 9 years with severe classical haemophilia who had persistently abnormal liver function tests. Abnormal histology was present in all; 4 had chronic persistent hepatitis and the fifth chronic aggressive hepatitis with early cirrhosis. Evidence of previous hepatitis B infection was present in one patient, 3 had antibodies to hepatitis, A, and 2 had subnormal levels of alpha-1-antitrypsin. Haemobilia occurred as a late complication of biopsy in one. The significance of these findings in young boys is discussed, as is the role of exposure to factor VIII containing blood products. It is concluded that cryoprecipitate should be used in preference to large pool factor VIII concentrates in children with haemophilia.
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PMID:Liver disease complicating severe haemophilia in childhood. 743 4

Liver transplantation has become the standard treatment for a variety of inherited metabolic disorders. We report on two patients who underwent successful transplantation for posthepatitis viral cirrhosis, which developed following blood factor replacement for haemophilia A. The second patient was transplanted before the occurrence of major complications of either his liver or haemophilic disease. We propose early liver transplantation to achieve metabolic cure of haemophilia.
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PMID:Liver transplantation and haemophilia A. 765 Mar 39

Over the past decade, with the use of plasma-derived factor VIII and factor IX, treated with virucidal methods, as well as with recombinant factor VIII, the replacement therapy of hemophilia has been intensified. In developed countries, a majority of patients are being treated at home, and large groups of children benefit from primary prophylaxis. A serious task in these countries for the coming years is the management of patients infected with HIV. In Poland and less-developed countries, the supply of antihemophilic factor concentrates is inadequate. Patients with inhibitor antibodies should be included in programmes of immune tolerance inducement. Many patients who had been multitransfused with cryoprecipate or received lyophilized concentrates before 1985, have developed chronic hepatitis associated with viral infections. About 15-30% show evidence of cirrhosis. Recombinant technologies should be improved and become more accessible in order to provide patients with safe and cheap antihemophilic factor concentrates. A true break-through in the hemophilia treatment would be a repair of the inherited clotting defect with gene therapy.
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PMID:[Current status and future prospects of hemophilia treatment]. 765 34

Over the past several years, the use of 1-deamino-8-D-arginine vasopressin (DDAVP), a synthetic analogue of vasopressin, has been found to be useful in the treatment of patients with abnormal bleeding tendency. This article is a review of inherited and acquired disorders with prolonged bleeding time in which DDAVP is supposed to shorten the bleeding time. DDAVP is established as effective therapy of the abnormal haemostasis in mild or moderate haemophilia A and von Willebrand's disease. Frequently, DDAVP infusions are used to control bleeding in patients with uraemia. Bleeding time is also significantly shortened in patients with liver cirrhosis, although randomized trials of DDAVP therapy of gastrointestinal bleeding in this group of patients are still needed. Shortening or normalization of the bleeding time with DDAVP has also been observed in patients with inherited platelet dysfunctions, acquired disorders of haemostasis and abnormal haemostasis in chronic myeloproliferative diseases. In addition, preoperative treatment with DDAVP seems to reduce blood loss during surgery.
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PMID:[Desmopressin in the treatment of hemorrhagic diathesis]. 854 Jan 36

Liver biopsy (n = 35) and autopsy (n = 71) specimens from HIV infected HCV-positive and HCV-negative haemophiliacs and non-haemophiliacs and liver biopsies (n = 33) from HIV-negative HCV-infected haemophiliacs and non-haemophiliacs were studied by histo- and immunohistochemistry to investigate the influence of HIV-coinfection on chronic C hepatitis (> 10 years duration). Almost all HIV-infected patients had a CD4 cell counts < 200/microns3. In biopsies and autopsies HCV-infection lead to stronger portal, periportal and lobular inflammatory changes independent from HIV-infection and haemophilia. However, HIV-infected patients with HCV-coinfection showed much more granulocytic infiltrates, particularly in the small bile ducts. In biopsies and autopsies HCV infection was associated with a stronger (centrilobular) fibrosis, particularly in HIV-positive haemophiliacs, and significantly stronger compared to HCV-negative patients. In the autopsy group half of the HIV-infected and HCV-positive haemophiliacs (n = 20) had developed posthepatitic liver cirrhosis due to C hepatitis, contrasted by two liver cirrhosis in HCV-infected non-haemophiliacs (n = 6) due to chronic B and C hepatitis and chronic alcohol abuse; no liver cirrhosis was observed in HIV-positive HCV-negative non-haemophiliacs (n = 45). Cholestasis and mild granulocytic cholangiolitis was a predominant feature in HIV/HCV-coinfection and similar distributed in haemophiliacs and non-haemophiliacs. The findings are suggestive that HIV-coinfection aggravates the course of a preceding hepatitis C virus infection, by a more granulocytic inflammatory infiltrate, stronger (centrilobular) fibrosis followed by a high incidence of posthepatitic cirrhosis--particularly in multitransfused haemophiliacs--and by cholestatic hepatopathy.
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PMID:[Hepatitis and posthepatic cirrhosis in AIDS]. 860 Jun 88


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