Showing papers by "Cindy A. Leissinger published in 2005"
••
TL;DR: This first randomized clinical trial of prophylaxis in young children with FVIII deficiency showed improved joint function by age 6 years in children on early every other day prophYLaxis in comparison to an aggressive program of multiple infusions administered promptly at the time of joint hemorrhage.
41 citations
••
National Institutes of Health1, Thomas Jefferson University2, Case Western Reserve University3, Children's Memorial Hospital4, Palmetto Health5, Cincinnati Children's Hospital Medical Center6, Boston Children's Hospital7, University of California, Irvine8, University of Pennsylvania9, Children's National Medical Center10, University of North Carolina at Chapel Hill11, Emory University12, University of Minnesota13, Georgetown University14, Pennsylvania State University15, University of Western Ontario16, North Shore-LIJ Health System17, Tulane University18, Virginia Commonwealth University19, University of Colorado Denver20, Icahn School of Medicine at Mount Sinai21, Primary Children's Hospital22, Ohio State University23, Puget Sound Blood Center24, Saint Louis University25, University of Arizona26, University of California, San Francisco27, University of Cincinnati28, University of Iowa29, University of Mississippi30, University of New Mexico31, University of Tennessee Health Science Center32, University of Texas Health Science Center at Houston33, Veterans Health Administration34, Wake Forest University35, National and Kapodistrian University of Athens36
TL;DR: The scale of hepatic and haematological disease that is likely to manifest in the adult haemophilic population during the coming years unless most of them are successfully treated for HIV‐1, HCV or both is revealed.
Abstract: Before the mid-1980s, haemophilia often was unknowingly treated with contaminated plasma products, resulting in high rates of human immunodeficiency virus (HIV-1), hepatitis C virus (HCV) and hepatitis B virus (HBV) infections. To estimate the impact of these infections, a new cohort was established. All HCV-seropositive patients, age 13-88 years, at 52 comprehensive haemophilia treatment centres were eligible. Cross-sectional data collected during April 2001 to January 2004 (median June 2002) were analysed. Plasma HIV-1 and HCV RNA were quantified by polymerase chain reaction. Highly active antiretroviral therapy (HAART) was defined as use of at least three recommended medications. Among 2069 participants, 620 (30%) had HIV-1. Of 1955 with known HBV status, 814 (42%) had resolved HBV and 90 (4.6%) were HBV carriers. Although 80% of the HIV-1-positive participants had > or = 200 CD4+ cells microL(-1), only 59% were on HAART. HIV-1 RNA was undetectable in 23% of those not taking antiretroviral medications. Most (72%) participants had received no anti-HCV therapy. HCV RNA was detected less frequently (59%) among participants treated with standard interferon plus ribavirin (P = 0.0001) and more frequently among HIV-1-positive than HIV-1-negative participants (85% vs. 70%, P < 0.0001). HIV-1-positive participants were more likely to have pancytopenia and subclinical hepatic abnormalities, as well as persistent jaundice, hepatomegaly, splenomegaly and ascites. HAART recipients did not differ from HIV-negative participants in the prevalence of ascites. The clinical abnormalities were more prevalent with older age but were not confounded by HBV status or self-reported alcohol consumption. Eleven participants presented with or previously had hepatocellular carcinoma or non-Hodgkin lymphoma. Although prospective analysis is needed, our data reveal the scale of hepatic and haematological disease that is likely to manifest in the adult haemophilic population during the coming years unless most of them are successfully treated for HIV-1, HCV or both.
31 citations
••
TL;DR: The data suggest that some severe hemophiliacs with heavy exposure to infectious HIV contaminated CFC had only transient low-level humoral immune responses reactive with HIV antigens yet remained HIV-negative and apparently uninfected, and supports the possibility of HIV exposure without sustained infection and the existence of HIV-natural resistance in some individuals.
Abstract: Background
Approximately 25% of hemophiliacs that were frequently exposed to blood clotting factor concentrates (CFCs) contaminated with human immunodeficiency virus (HIV) are presently HIV seronegative. In this study, we sought to determine if some of these individuals were at any time transiently HIV seropositive. In the early to mid-1980s the majority of severe hemophilia patients were exposed to CFCs contaminated with HIV. Although many of these hemophiliacs became HIV-positive, a small percentage did not become infected. To determine if some of these individuals successfully resisted viral infection, we attempted to document the presence of transient HIV reactive antibodies in archived plasma samples (1980–1992) from currently HIV-negative severe hemophiliacs who had a high probability of repeated exposure to HIV contaminated CFC. Archived plasma samples were retrospectively tested using an FDA approved HIV-1Ab HIV-1/HIV-2 (rDNA) enzyme immunoassay (EIA) and a HIV-1 Western blot assay (Wb), neither of which were commercially available until the late 1980s, which was after many of these samples had been drawn.
13 citations
••
TL;DR: A 39-year-old man with severe congenital haemophilia A without an inhibitor with stable asymptomatic HIV infection with an undetectable viral load and normal CD4 counts is admitted to the hospital for an ultrasound guided transjugular liver biopsy in preparation for a hepatitis C treatment protocol.
Abstract: Most haemophilia patients who received exposure to clotting factor concentrates prior to the introduction of effective viral inactivation techniques were exposed to blood-borne hepatitis viruses. For older patients the prevalence of chronic hepatitis C infection approaches 85%, while persistent hepatitis B affects as many as 5%. Liver disease has become a serious problem for many older haemophilia patients, especially those concomitantly affected by HIV disease. With the advent of effective treatment strategies for hepatitis C and HIV, liver biopsies have played a larger role in the evaluation and planning of therapeutic strategies for these patients. The recent introduction of the transjugular approach to liver biopsies in the USA has transformed the practice of many haemophilia treaters who feel that the risk of major haemorrhage and the potential cost in clotting factor concentrates are less than with percutaneous biopsy. Several recently published case series have reported experience with transjugular biopsies in over 150 patients with congenital bleeding disorders with good results [1–4]. We too have conducted liver biopsies by the transjugular approach. While most of these patients have performed very well following the procedure, one patient experienced a serious, unexpected and potentially life-threatening bleeding complication following transjugular biopsy. The patient is a 39-year-old man with severe congenital haemophilia A without an inhibitor. He has stable asymptomatic HIV infection with an undetectable viral load and normal CD4 counts despite having never been on anti-retroviral therapy. He also was known to have asymptomatic hepatitis C infection with measurable hepatitis C viraemia, but normal liver functions and no thrombocytopenia. At 37 years, he was admitted to the hospital for an ultrasound guided transjugular liver biopsy in preparation for a hepatitis C treatment protocol. Prior to the procedure, he was given a bolus infusion of his usual factor VIII (FVIII, 50 U kg). The procedure was preformed without complications and following the procedure the patient was asymptomatic. He then received additional FVIII bolus doses (25 U kg) 12 h later and again prior to his discharge the next morning. He was discharged in stable condition with a haemoglobin of 13.9 (reference 13.0–18.0 g dL) and, as instructed, took additional doses of factor (25 U kg) daily for the next 2 days. Four days after the biopsy, he developed sharp epigastric pain, and took an additional dose of FVIII of 25 U kg; the pain was not completely reduced but changed to a dull ache. The following day he returned to the emergency room complaining of upper abdominal pain. He denied fever, nausea, vomiting or bowel changes. On examination, he was afebrile with normal vital signs. His abdomen was soft, non-tender to direct and indirect palpitation with no palpable masses, and no signs of an acute abdomen. He had a normal WBC count and differential, normal platelet count and haemoglobin of 15.4 g dL. His liver enzymes which had been within normal limits 3 months earlier, were now elevated with an AST of 398 (reference <39 U L), ALT of 275 (reference 30–65 U L), alkaline phosphatase of 219 (reference 40–120 U L) and total bilirubin of 1.6 (reference <1.1 mg dL). His amylase was elevated at 346 (reference 19–76 U L) and lipase at 116 (reference 114–286 U L). An ultrasound of the abdomen showed well defined, non-mobile echogenic foci in the gallbladder which appeared to be adherent to the wall without intrahepatic biliary duct or common bile duct dilation. The diagnosis of cholecystitis vs. haemobilia was entertained. Correspondence: Rebecca Kruse-Jarres, MD, Tulane University School of Medicine, 1430 Tulane Avenue SL-78, New Orleans, LA 70112. Tel.: 504 988 6352; fax: 504 988 5483; e-mail: rkrusej@cox.net
6 citations
••
TL;DR: This study was a rigorous effort to quantify adherence and then compare it to QofL and found that body pain in children was higher with OD compared with HI, however this was not demonstrated in adults.
2 citations
••
TL;DR: The PK results are comparable to PK data from other clinical studies with Humate-P®/Haemate P® and given the interindividual variability underline the importance of pharmacokinetic studies prior to surgical interventions.
1 citations
••
TL;DR: This analysis supports VWF/FVIII concentrate safety and efficacy to prevent excessive bleeding during and after a range of surgical procedures in patients with VWD.
1 citations