Liver Transplantation in a Hemophiliac

To the Editor: A cure rather than a treatment has long been the goal of those caring for and those suffering from hemophilia. Encouraging results were obtained some years ago with the transplantation of normal livers into a dog with mild hemophilia1,2 and into four others with severe hemophilia.3 Two dogs given transplants survived more than 100 days and produced coagulation factor VIII in quantities sufficient to maintain normal levels. The first “cure” in a human being appears to have been achieved, at least temporarily, in a 15-year-old boy with hemophilia and severe chronic active hepatitis, who received a liver. © 1985, Massachusetts Medical Society. All rights reserved

A non-invasive monitoring on European wildcat (Felis silvestris silvestris Schreber, 1777) in Sicily using hair trapping and camera trapping: does scented lure work?

An hair trapping protocol, with camera trapping surveillance, was carried out on the south-western side of the Etna, inhabited by an abundant population of the European wildcat. We aimed to collect hair for genetic analysis on the base of a field study conducted in Switzerland, where valerian tincture had been used to attract wildcats to rub again wooden sticks and therefore leaving hairs. We placed 18 hair trapping stations, plus one camera trap per scented wooden stick, 1 km away from each other for 60 days (October 29 2010 to December 28 2010). The rate of "capture" success (1 capture / 24.5 trap-days) by camera trapping was substantially the same as those obtained during previous surveys performed in the same study area without the use of any attractants. No wildcats were photographed while rubbing against the wooden sticks, neither any wildcat was interested in the scent lure. We discuss limitations of the hair trapping, providing possible explanations on the failure of valerian tincture, while suggesting some field advices for future monitorings

Liver transplantation in hemophilia A

Four patients with hemophilia A have undergone liver transplantation in our institution, three successfully. The first was a 21-year-old man with chronic active hepatitis (CAH) in whom the effects of previous abdominal operations prevented the satisfactory technical insertion of the new liver. He died intraoperatively. The second patient was a 15-year-old boy with CAH who began to synthesize factor VIII coagulant activity (F VIII:C) within 18 hours of successful liver transplantation and has continued to do so for almost 2 years (F VIII:C range 0.89 to 3.20 U/mL). The first 2 months of his postoperative course were complicated by infections, but since that time he has done well and has returned to school. The third patient was a 48-year-old man with portal fibrosis and severe ascites. He synthesized F VIII:C (range 0.96 to 1.50 U/mL) within six hours after reestablishment of circulation through the new liver. His postoperative course was complicated by numerous infections, and he died with sepsis and an acquired immunodeficiency-like syndrome 4 months after transplantation. The fourth patient was a 47-year-old mild hemophiliac with CAH who produced adequate factor VIII:C levels following transplantation (range 0.79 to 2.80 U/mL). These patients demonstrate that liver transplantation in hemophiliacs with end-stage liver disease may be lifesaving and results in correction of the F VIII:C deficiency and associated hemorrhagic tendency

Liver transplantation: Intraoperative changes in coagulation factors in 100 first transplants

Six intraoperative blood samples were obtained at intervals from each of 100 individuals undergoing their first liver transplants. The patients fell into the following diagnostic categories: postnecrotic cirrhosis 28, primary biliary cirrhosis 20, sclerosing cholangitis 19, miscellaneous diseases 14, carcinoma/neoplasia 12 and fulminant hepatitis 7. Coagulation factor values in the initial (baseline) blood samples varied by patient diagnosis. In general, all factor levels were reduced except factor VIII:C, which was increased to almost twice normal. The slight intraoperative changes in factors II, VII, IX, X, XI and XII suggested that a steady‐state relationship existed between depletion (consumption/bleeding) and repletion (transfusion, transit from extra‐ to intravascular space), even in the anhepatic state. In contrast, there were rapid and very significant falls in factor VIII and fibrinogen and a less pronounced decrease in factor V, all reaching their nadirs in early to mid‐Stage III. The cause of these coagulation changes appears to be activation of the fibrinolytic system. Copyright © 1989 American Association for the Study of Liver Disease

The relation of preoperative coagulation findings to diagnosis, blood usage, and survival in adult liver transplantation

A group of 70 adults with end-stage liver disease received 87 homologous liver transplants from 7/11/81 and 7/11/83. The recipients fell into the following diagnostic categories: Postnecrotic cirrhosis (PNC) in 22, primary biliary cirrhosis (PBC) in 18, cancer or neoplasia (CA) in 11, sclerosing cholangitis (SC) in 8 and miscellaneous (MISC) in 11. Survival for six months or longer was 46%: Survival by group was PBC=67%, CA=55%, PNC=45%, SC=25%, and MISC=18%. Preoperative coagulation profiles were evaluated on 64 of the 70 first transplant patients by assigning a score derived from one point per abnormality in each of 8 tests. Mean coagulation abnormality scores (CAS) were strikingly elevated in the PNC and MISC groups. Mean intraoperative blood product usage was 43 units of RBCs, 40 units of fresh frozen plasma (FFP), 21 units of platelets, and 9 bags of cryoprecipitate. Direct correlations were found between CAS and RBC usage (+0.454, P=001), CAS, and survival of 6 months or longer (-0.281, P=.02), and RBC usage and survival (-0.408, P=.001). These findings indicate that the degree of coagulation abnormality and the type of liver disease may be predictive of intraoperative blood usage and survival in liver transplantation in adults. © 1985 by The Williams & Wilkins Co

Blood use in liver transplantation

During the first 5 years (1981–1985) of the liver transplantation program in Pittsburgh, a total (preoperative, intraoperative, and postoperative) of 18,668 packed red cell units, 23,627 fresh‐frozen plasma units, 20,590 platelet units, and 4241 cryoprecipitate units was transfused for the procedures. This represents 3 to 9 percent of the total of blood products supplied by the Central Blood Bank to its 32 member hospitals. Six hundred thirty‐six (636) transplants were performed on 485 patients in two hospitals: the Presbyterian University Hospital (564 beds) and Children's Hospital of Pittsburgh (236 beds). All of the blood components used in the operations were procured and released by the Central Blood Bank. This report describes some of these findings. 1987 AAB

Infection with human immunodeficiency virus in the pittsburgh transplant population: A study of 583 donors and 1043 recipients, 1981-1986

We performed a retrospective serologic survey of 583 organ donors and 1043 transplant recipients for antibodies to human immunodeficiency virus type 1 (HIV- 1). Two (0.34%) of the 583 donors and 18 (1.7%) of the 1043 recipients had HIV-1 antibodies by enzyme immunoassay and by Western blot. Two of 5 seropositive recipients tested also had blood cultures positive for HIV-1. Seven (0.7%) of the 1043 transplant recipients had antibodies to HIV-1 before transplantation; 2 of these had hemophilia A, and 5 had previous transfusions. Eleven (1.3%) of 860 recipients followed for 45 days or more seroconverted to HIV-1 a mean of 96 days after transplantation. Likely sources of HIV-1 infection for 3 of these 11 recipients included a seropositive organ donor in 1 patient and high-risk blood donors in 2 patients. A definite source of HIV-1 infection was not found for the other 8 recipients, 3 of whom seroconverted to HIV-1 after institution of blood donor screening for HIV-1 antibodies. Seroconversion to HIV-1 was less common in kidney recipients than in liver, heart, or multiple-organ recipients (P<0.02). Nine (50%) of the 18 HIV-1 seropositive transplant recipients died a mean of 6 months after transplant surgery, and 9 (50%) are still alive a mean of 43 months after transplantation. AIDS-like illnesses occurred in 3 of the dead and 1 of the living patients and included pneumocystis pneumonia (3 cases), miliary tuberculosis (1 case), and recurrent cytomegalovirus infection (1 case). These data suggest that the course of HIV-1 infection is not more severe in transplant recipients receiving cyclosporine than in other hosts and that, despite screening of blood and organ donors, a small number of transplant recipients will become infected with HIV-1. © 1989 by The Williams and Wilkins Co