نتایج جستجو برای: hemophilia a
تعداد نتایج: 13433047 فیلتر نتایج به سال:
n engl j med 366;3 nejm.org january 19, 2012 281 not match that of the patient. Tiede et al.2 observed that some patients with acquired hemophilia have been exposed to a factor VIII protein that does not match their own. Specifically, there are polymorphisms of factor VIII at amino acids 1241 (Asp→Glu) and 2004 (Glu→Lys). In patients with certain HLA-DRB1 alleles, exposure to a “foreign” factor...
The incidence of neutralizing isoantibody formation to infused factor VIII in a cohort of 67 hemophilia A patients, born between January 1, 1971 and April 30, 1990, who had been treated exclusively with lyophilized cryoprecipitate, was 6% (5.3 per 1,000 patient years of observation). The age-dependent cumulative risk was 4.6% at 4 years of age and 6.7% at 8 years of age. Recent reports in patie...
During a 4-year multicenter cooperative study of acquired factor VIII inhibitors in persons with hemophilia A. new inhibitors were detected in 31 of 1 .306 patients who entered the study without an inhibitor or the history of an inhibitor. The incidence of new inhibitors was eight per 1 .000 patient-years of observation. The factor Vlll:C level before inhibitor development was 0.03 U/mi in 29 m...
Acquired hemophilia is a rare disease, presenting with severe hemorrhage, we present a case caused by a duodenal tumor, the clinical management, ethical implications, treatment recommendations, and a review of the literature.
23 p23 UPDATE informs you of changes in clinical practice. This week: smoking prevalence p26 PrAcTicAl ProcEDUrEs illustrates procedures. This week: assessing consciousness p28 gUiDED lEArNiNg includes online cPD. This week: surgery and haemophilia p30 DEVEloPmENT shows how nurses are developing practice. This week: delegating consent-taking p32 rEsEArcH presents research papers. This week: pat...
The activity of the factor VIII coagulation protein can be measured by three methods: a one or two-stage clotting assay and a chromogenic assay. The factor VIII activity of most individuals with mild hemophilia A is the same regardless of which method is employed. However, approximately 30% of patients show marked discrepancies in factor VIII activity measured with the different methods. The ob...
Type B hemophilia usually affects patients with a family history of this disease and has a typical clinical picture. However, in the present case it appeared in a patient outside the typical age with no family history of hematologic malignancies and with an unusual clinical picture.
The objective of this study was to examine the association of the intensity of treatment, ranging from high-dose intensive factor VIII (FVIII) treatment to prophylactic treatment, with the inhibitor incidence among previously untreated patients with severe hemophilia A. This cohort study aimed to include consecutive patients with a FVIII activity < 0.01 IU/mL, born between 2000 and 2010, and ob...
An 18-year-old man has severe hemophilia A that has been complicated by a high-titer inhibitory antibody (peak 170 BU/mL). He had previously failed a trial of immune tolerance induction (ITI) using daily high-dose (100 units/kg/d) factor VIII (FVIII) for 20 months and would like to know if immunomodulatory agents, with or without another course of ITI, might eradicate the inhibitor.
1Life and Health Sciences Research Institute (ICVS), School of Medicine, University of Minho, Braga, Portugal 2 ICVS/3B’s – PT Government Associate Laboratory, Braga/Guimarães, Portugal 3School of Psychology, University of Minho, Braga, Portugal 4Faculty of Psychology and Education Sciences, University of Porto, Porto, Portugal 5Portuguese Haemophilia Association, Lisbon, Portugal 6European Hae...
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