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Inherited Bleeding Disorders (inherited + bleeding_disorders)
Selected AbstractsHepatitis A and B immunization for individuals with inherited bleeding disordersHAEMOPHILIA, Issue 2 2009M. STEELE Summary., Hepatitis A and B vaccines are highly effective tools that can greatly reduce infection risk in the bleeding disorder population. Although hepatitis A and B immunization for individuals with bleeding disorders is universally recommended, various advisory bodies often differ with respect to many practical aspects of vaccination. To review the published literature and guidelines and form a practical, comprehensive and consistent approach to hepatitis A and B immunization for individuals with bleeding disorders. We reviewed published immunization guidelines from North American immunization advisory bodies and published statements from North American and international haemophilia advisory bodies. A search of the MEDLINE database was performed to find original published literature pertaining to hepatitis A or B immunization of patients with haemophilia or bleeding disorder patients that provided supporting or refuting evidence for advisory body guidelines. Various advisory bodies' immunization guidelines regarding individuals with bleeding disorders have contradictory statements and often did not clarify issues (e.g. post vaccination surveillance). Published literature addressing immunization in bleeding disorder patients is sparse and mostly examines route of vaccine administration, complications and corresponding antibody response. Although the risk of hepatitis A and B infection is low, the use of simple measures such as vaccination is reasonable and advocated by haemophilia advisory bodies. Following our review of the available literature and North American guidelines, we have developed comprehensive and practical recommendations addressing hepatitis A and B immunization for the bleeding disorder population that may be applicable in Bleeding Disorder clinics. [source] Review of quality of life: menorrhagia in women with or without inherited bleeding disordersHAEMOPHILIA, Issue 1 2008M. SHANKAR Summary., The objectives of this study were to identify the impact of menorrhagia on the health-related quality of life (HRQOL) of women in general and those with inherited bleeding disorders and to identify the commonly used tools in assessing quality of life. A review of studies evaluating quality of life in women suffering from menorrhagia was conducted. Data sources used included electronic databases Medline and Embase. Reference lists and bibliographies of the relevant papers and books were hand-searched for additional studies. Eighteen of the 53 studies identified measured quality of life prior to treatment of menorrhagia. Ten of the studies used a validated measure of quality of life. Five studies involving a total of 1171 women with menorrhagia in general and using SF-36 were considered for further review. The mean SF-36 scores in women with menorrhagia were worse in all the eight scales when compared with normative scores from a general population of women. Three studies, involving 187 women, assessed the quality of life in women with menorrhagia and inherited bleeding disorders. None of these studies used a validated HRQOL score making it difficult for comparison. However, all reported poorer scores in study women compared to the controls. In conclusion, HRQOL is adversely affected in women with menorrhagia in general and in those with inherited bleeding disorders. HRQOL evaluation is useful in the management of women with menorrhagia for assessment of treatment efficacy. [source] The obstetric and gynaecological management of women with inherited bleeding disorders , review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors' OrganizationHAEMOPHILIA, Issue 4 2006C. A. LEE Summary., The gynaecological and obstetric management of women with inherited coagulation disorders requires close collaboration between obstetrician/gynaecologists and haematologists. Ideally these women should be managed in a joint disciplinary clinic where expertise and facilities are available to provide comprehensive assessment of the bleeding disorder and a combined plan of management. The haematologist should arrange and interpret laboratory tests and make provision for appropriate replacement therapy. These guidelines have been provided for healthcare professionals for information and guidance and it is also intended that they are readily available for women with bleeding disorders. [source] Identification and management of women with inherited bleeding disorders: a survey of obstetricians and gynaecologists in the United KingdomHAEMOPHILIA, Issue 4 2006C. CHI Summary., A mail survey of members and fellows of Royal College of Obstetricians and Gynaecologists was carried out to determine current practices of obstetricians and gynaecologists in the United Kingdom in the management of women with inherited bleeding disorders. In total, 3929 questionnaires were sent, 707 returned and analysis was limited to 545 valid questionnaires. In the past 5 years, 91% have managed women with inherited bleeding disorders. The majority (83%) considered inherited bleeding disorders to be under diagnosed in obstetrics and gynaecology. More than 80% considered the prevalence of von Willebrand's disease (VWD) to be <0.2% in the general population and <1% in women with menorrhagia and no gynaecological pathology, although the reported prevalence is 1% and 5,25% respectively. Twelve percent of the respondents would arrange testing for VWD when reviewing an 18-year-old with menorrhagia and no pelvic pathology, while only 2% would do the same for a 35-year-old with the same presentation. Twenty-one percent thought elective caesarean section is indicated in all fetuses known to be at risk of being affected by haemophilia. Eighty-four percent considered vacuum extraction unsafe in these cases, but 76% would consider the use of low forceps. In conclusion, obstetricians and gynaecologists underestimate inherited bleeding disorders as an underlying cause for menorrhagia. Increased awareness and management guidelines are essential in minimizing haemorrhagic complications and improving quality of care of these women. [source] Dental procedures in adult patients with hereditary bleeding disorders: 10 years experience in three Italian Hemophilia CentersHAEMOPHILIA, Issue 5 2005M. Franchini Summary., Excessive bleeding after dental procedures are one of the most frequent complications occurring in patients with hereditary bleeding disorders. In this retrospective study we collected data from 10 years of experience in the oral care of patients with congenital haemorrhagic disorders in three Italian Hemophilia Centers. Between 1993 and 2003, 247 patients with inherited bleeding disorders underwent 534 dental procedures including 133 periodontal treatments, 41 conservative dentistry procedures, 72 endodontic treatments and 288 oral surgery procedures. We recorded 10 bleeding complications (1.9%), most of which occurred in patients with severe/moderate haemophilia A undergoing multiple dental extractions. Thus, our protocol of management of patients with hereditary bleeding tendency undergoing oral treatment or surgery has been shown to be effective in preventing haemorrhagic complications. [source] Clinical, laboratory and therapeutic aspects of platelet-type von Willebrand diseaseINTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 2 2008M. FRANCHINI Summary Platelet-type von Willebrand disease (PT-VWD), or pseudo-VWD, is a rare inherited platelet disorder characterized by an increased affinity of the platelet membrane glycoprotein Ib, receptor for normal von Willebrand factor leading to characteristic platelet hyperaggregability. As PT-VWD shares most of the clinical and laboratory features of subtype 2B VWD, the differential diagnosis between these two inherited bleeding disorders requires either platelet-mixing or molecular genetic studies. In this review, the main clinical, laboratory and therapeutic characteristics of PT-VWD are concisely reported. [source] The effect of highly active antiretroviral therapy for HIV on the anti-HCV specific humoral immune responseJOURNAL OF MEDICAL VIROLOGY, Issue 2 2004Esteban Herrero-Martínez Abstract The effect of highly active antiretroviral therapy (HAART) on HCV replication is controversial, with some studies reporting no effect and others increases, reductions and even clearances of HCV RNA after treatment. In this study, the effect of HAART was investigated on the titre of anti-HCV specific antibodies and on the relationship between these antibodies and HCV RNA level in a cohort of 24 patients with inherited bleeding disorders. A significant inverse correlation between antibodies to both total HCV proteins and HCV RNA (R,=,,0.42, P,=,0.05) and between antibodies to HCV envelope glycoproteins and HCV RNA (R,=,,0.54, P,=,0.01) was observed pre-HAART. The relationship disappeared or was obscured after therapy (R,=,0.24, P,=,0.30 and R,=,0.16, P,=,0.50, respectively). Thus, we show that HAART affects the HCV specific humoral immune responses without affecting the HCV RNA level. J. Med. Virol. 72:187,193, 2004. © 2004 Wiley-Liss, Inc. [source] ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disordersJOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 9 2010F. RODEGHIERO No abstract is available for this article. [source] Management of third molar removal with a single dose of recombinant Factor IX (BeneFIX) and local measures in severe haemophilia BAUSTRALIAN DENTAL JOURNAL, Issue 3 2010ID Hewson Abstract Background:, Patients with inherited bleeding disorders have historically had factor cover for oral surgery. Factor support is expensive, time consuming and places the patient at a potential risk of blood-borne diseases. This case describes the use of a significant reduction in factor support for a severe haemophilia B patient having third molars surgically removed. Methods:, Local measures were used after a single preoperative dose of Factor IX to obtain good postoperative haemostasis. Results:, Excellent haemostasis was achieved using local measures of 5% tranexamic acid solution, Surgicel® and Monocryl® sutures after a single preoperative dose of Factor IX. Conclusions:, Oral surgery may be performed on patients with inherited bleeding disorders using minimal factors and local haemostatic measures. A study of this patient population has commenced at The Alfred Hospital. [source] The use of levonorgestrel-releasing intrauterine system for treatment of menorrhagia in women with inherited bleeding disordersBJOG : AN INTERNATIONAL JOURNAL OF OBSTETRICS & GYNAECOLOGY, Issue 10 2005May Wahab No abstract is available for this article. [source] The use of levonorgestrel-releasing intrauterine system for treatment of menorrhagia in women with inherited bleeding disordersBJOG : AN INTERNATIONAL JOURNAL OF OBSTETRICS & GYNAECOLOGY, Issue 12 2004C.E.C. Kingman Background The levonorgestrel-releasing intrauterine system (LNG-IUS) is used commonly by gynaecologists as a contraceptive and to treat menorrhagia. However, its efficacy has not been examined in women with inherited bleeding disorders. Design A prospective pilot study. Setting A teaching hospital in north London with a designated haemophilia centre. Population Female patients with a known inherited bleeding disorder. Methods Sixteen women with subjective and objective menorrhagia caused by inherited bleeding disorders (13 von Willebrand's Disease, two factor XI deficiency and one Hermansky,Pudlak syndrome), who had previously undergone unsuccessful medical treatment were followed up for nine months after LNG-IUS insertion. Bleeding was measured by pictorial chart and haemoglobin concentration. Main outcome measure Results All women reported that their periods were improved, pictorial chart scores were lower and 56% became amenorrhoeic. None reported side effects. Conclusion The LNG-IUS is well tolerated and effective and improves quality of life. [source] | ||||||||||