Factor XI deficiency acquired by liver transplantation
Article Abstract:
Organ transplantation has been used in the treatment of several types of genetic disorders. However, the possibility exists for an unrecognized genetically defective organ to be transplanted into a recipient. This event has now occurred in the case of a 54-year-old man who received a transplanted liver for chronic hepatitis; the patient was deteriorating rapidly at the time of transplantation. A donor liver was obtained from a 47-year-old man who had died of a ruptured aneurysm in the brain. Over the first four weeks following the surgery the condition of the liver recipient improved, including his brain function, which had been severely affected by the failing liver. However, the activated partial thromboplastin time (aPTT) had started to rise about a week after the surgery, indicating an abnormality in the blood clotting system. Further evaluation a month after surgery revealed a deficiency in clotting factor XI. The liver donor's family was questioned, and it was found that the donor was an Ashkenazi Jew with a history of bleeding after dental work. Apparently, this man was known to have an abnormal aPTT. These observations suggest that the liver donor had suffered from the Rosenthal syndrome, an inherited disorder in which factor XI is deficient and which results in abnormal bleeding. The liver patient apparently received an organ which was genetically incapable of manufacturing the necessary factor XI; this observation confirms incidentally the belief that the liver is the sole source of factor XI within the body. Fortunately, the transplant recipient has not yet experienced any serious bleeding episodes, which may indicate that factors outside the liver may distinguish the bleeding tendency of the donor and recipient. Obviously, it would be preferable to rule out as many metabolic disorders as possible before transplanting a donor organ. However, in cases where rapid transplantation may save a patient's life, the problems associated with a specific genetic defect may be outweighed. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Annals of Internal Medicine
Subject: Health
ISSN: 0003-4819
Year: 1991
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The prevalence of hepatitis C virus antibodies among hemodialysis patients
Article Abstract:
Hepatitis, a potentially life-threatening disease, is an inflammatory disease of the liver and is characterized by enlargement of the liver, fever and yellowing of the skin and eyes. Hepatitis can be caused by bacteria, drugs, alcohol, transfusions of incompatible blood, or by viruses. Hepatitis A, B and C are now known to be caused by viruses. Patients undergoing kidney hemodialysis, which is the removal of toxic substances from the blood, may develop hepatitis due to the hepatitis C virus. A method has recently been developed that allows the levels of antibodies against the hepatitis C virus to be measured. Antibodies are globular proteins produced by the immune system which bind and inactivate foreign substances such as viruses. To test the prevalence of the hepatitis C virus in patients undergoing hemodialysis, blood from 102 hemodialysis patients was analyzed for antibodies against the hepatitis C virus. Only 15.7 percent of the patients had antibodies against the hepatitis C virus. All of these patients had histories of other markers that put them at risk of developing hepatitis C independent of dialysis treatment. Such findings support an earlier study that also reported less than 20 percent of hemodialysis patients have antibodies against the hepatitis C virus. Thus, hemodialysis treatment alone does not seem to place a patient at risk of developing hepatitis C. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Annals of Internal Medicine
Subject: Health
ISSN: 0003-4819
Year: 1990
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Hepatitis C in patients undergoing liver transplantation
Article Abstract:
Until the recent identification of hepatitis C virus (HCV) as its most common causative agent, post-transfusion hepatitis was described as non-A, non-B hepatitis. Non-A, non-B chronic active hepatitis with cirrhosis has been the most common cause of liver failure in patients undergoing liver transplantation. While it is known that patients with chronic hepatitis B infection will virtually all develop hepatitis B in their transplanted livers, the recurrence rate of HCV infection is unknown. To learn more about HCV recurrence after liver transplantation, researchers assessed 128 liver transplant patients, of whom 26 (20 percent) had antibodies to HCV pre-operatively. (The presence of antibodies indicates an immune response to HCV.) Sixty-six patients were available for long-term follow-up. Of these, 53 initially tested negative for HCV antibodies and 13 tested positive. Post-transplant hepatitis developed in 11 of the 66, but the rate of occurrence was unrelated to pre-transplant HCV status. Of the 13 initially HCV-positive patients, 4 (31 percent) developed post-transplant hepatitis, as did 7 (13 percent) of the initially HCV negative-patients. This difference was felt to be statistically insignificant. Thus, while HCV is an important cause of chronic liver disease leading to transplantation, its role in post-transplant hepatitis is less clear. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Annals of Internal Medicine
Subject: Health
ISSN: 0003-4819
Year: 1991
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