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Query: UMLS:C0684275 (haemophilia)
 10,958 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Central venous access devices (CVADs) can facilitate repeated and/or urgent administration of coagulation factors in haemophilic patients. We conducted a systematic review and meta-analysis of complication rates and risk factors for poor outcome. Forty-eight studies with a total of 2704 patients and 2973 CVADs were included. The primary indications for CVADs were immune tolerance therapy (34.9% of patients), difficult venous access (31.8%) and prophylaxis (29.1%). Fully implanted CVADs were employed in 77.4% of cases and external CVADs in 22.6%. A total of 1190 infections were reported, and the pooled incidence of infection was 0.66 per 1000 CVAD days [confidence interval (CI), 0.44-0.97 per 1000 CVAD days]. Among patients developing infection, the pooled time to first infection was 295 days (CI, 181-479 days). Presence of inhibitors was an independent risk factor for infection with an incidence rate ratio (IRR) of 1.67 (CI, 1.15-2.43). Infection was less likely in patients >6 years of age (IRR, 0.46; CI, 0.27-0.79) and recipients of fully implanted CVADs (IRR, 0.31; CI, 0.12-0.86). Available information on thrombosis was limited, with only 55 cases being reported. Eventually, 31.3% of CVADs were removed, and infection was the reason for removal in 69.9% of cases and thrombosis in 4.1%. The pooled time period CVADs remained indwelling prior to removal or the expiration of the study observation period was 578 days per CVAD (CI, 456-733 days per CVAD). CVADs can confer major benefits in patients with haemophilia requiring long-term venous access, and serious complications are rare.
Haemophilia 2004 Mar
PMID:Central venous access devices in haemophilia. 1496 2

Infections, thrombosis and technical problems are the most frequent complications when using implantable central venous access devices in patients with haemophilia. There seem to be two major experiences concerning infections in non-inhibitor patients, one is approx. 0.2 infections per 1000 days and the other approx. 1.0(0.7-1.6)/1000 days. Infections are more frequent in inhibitor patients and one can expect approx. one infection per 6-12 months of use. The figures are low for clinically apparent thrombosis in the larger series on record, but routine venograms were not done in most of these series. In studies where this has been done, a high frequency of abnormalities on venograms have been seen in some but not in others. The final decision to use a central line has to be a compromise between the medical goal, the patient's bleeding tendency, the social situation and the expected risk of complications at the particular haemophilia center. Some of the complications may be reduced by adequate aseptic measures both during implantation and in the subsequent use and clear basic routines for surveillance of the systems and repeated education of the users.
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PMID:Central venous catheters in children with haemophilia. 1501 Jan 48

Venous access is essential for delivery of haemophilia factor concentrate. Wherever possible, peripheral veins remain the route of choice, and the use of central venous access devices (CVADs) should be limited to cases of clear need in patients with caregivers able to exercise diligence in CVAD care and should continue no longer than necessary. CVADs are of recognized value for repeated administration of coagulation factors in haemophilia, particularly for prophylaxis and immune tolerance therapy and in young children. Evidence to guide best practices has been fragmentary, and standardized methods for CVAD usage have yet to be established. We have developed management recommendations based upon available published evidence as well as extensive clinical experience. These recommendations address patient and CVAD selection; CVAD placement, care and removal; caregiver/patient guidance; and complications, including infection and thrombosis. In the absence of inhibitors, ports are recommended, primarily because of fewer associated infections than with external catheters. For patients with inhibitors, ports also appear to be associated with fewer infections. Infection is the most frequent complication, and recommendations to prevent and treat infections are supported by extensive clinical data and experience. Strict adherence to handwashing and aseptic technique are essential elements of catheter care. Evidence-based data regarding the detection and treatment of CVAD-related thrombotic complications are limited. Caregiver education is an integral part of CVAD use and the procedural practices of users should be regularly re-assessed. These recommendations provide a basis for sound current CVAD practice and are expected to undergo further refinements as new evidence is compiled and clinical experience is gained.
Haemophilia 2004 Sep
PMID:Consensus recommendations for use of central venous access devices in haemophilia. 1535 90

Infection with Helicobacter pylori is the main aetiological factor for erosive gastritis and duodenal or gastric peptic ulcers often complicated with life-threatening bleeding in patients with coagulation disorders. The aim of this prospective study was to evaluate the prevalence of Helicobacter pylori infection in haemophilia patients, and to assess the risk of gastrointestinal bleeding associated with this infection. From 2000 to 2002, 146 patients with haemophilia (129, haemophilia A; 13, haemophilia B), mean age, 39.9 years (+/-7.3), were investigated for H. pylori infection using IgG and IgA latex serological test. The control group included 100 men with no coagulation disorders, mean age, 40.9 years (+/-9.2). For 72 (49.3%) patients with haemophilia and 39 controls (39.0%) serological tests were positive indicating the presence of H. pylori infection (P =0.1112). A history of gastrointestinal bleeding was reported in 46 patients (31.5%) with haemophilia and in two control group patients (2.0%) (P < 0.0001). Gastrointestinal bleeding was significantly more frequent in patients with haemophilia infected with H. pylori (33/46; 71.7%) than in patients with no H. pylori infection (13/46; 28.3%; P = 0.0002). In conclusion, the prevalence of H. pylori infection in haemophilic patients in Poland is comparable with that in patients with no coagulation disorders. Helicobacter pylori infection is a risk factor for duodenal and gastric ulcer bleeding in haemophilia patients. In view of the high frequency of upper gastrointestinal bleeding associated with H. pylori infection, we believe that screening and eradication therapy are appropriate in haemophilia patients.
Haemophilia 2005 Jul
PMID:Helicobacter pylori infection in patients with haemophilia in Poland: prevalence and risk of upper gastrointestinal bleeding. 1601 91

The aim of our study was to determine prevalence of hepatitis C virus infection (HCV) among patients with haematological and oncological diseases treated in the past with blood or blood products (about 20% among adults and about 50% in children in selected wards in 1992). Infection with HCV was detected in 3 out of 64 examined patients (4.7%) and in 64 of 14 098 blood donors (0.4%), p < 0.05. The two HCV cases in the study group were in persons with haemophilia A treated with cryoprecipitate before 1992. Among 51 persons receiving transfusions only after 1992--1 case of HCV infection was detected (2.0%) Our results indicate improvement in the safety of treatment with blood and/or blood products and suggest that the present risk of HCV infection among patients with haematological and oncological diseases treated with these products after 1992 is rather small.
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PMID:[Prevalence of hepatitis C virus infection among haematological and oncological patients treated with blood products]. 1678 61

Many patients with hemophilia, particularly those with inhibitory antibodies, utilize central venous access devices (CVADs) to facilitate frequent infusions. Infection of these devices is a common complication of factor replacement therapy. This communication reports our center's experience with CVAD infection in three patients with severe hemophilia A undergoing immune tolerance therapy (ITT) in whom intermittent infusions of recombinant tissue plasminogen activator (rTPA, Cathflo Activase) were utilized. In this small experience, patients experienced a decreased frequency of gram-positive infections when receiving routine rTPA treatments. Larger randomized trial should be performed in this patient population at high risk of CVAD infection.
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PMID:Recombinant tissue plasminogen activator may reduce frequency of central venous access device infection in hemophilia patients undergoing immune tolerance therapy. 1714 76

Infections are the most frequent complications associated with the use of central venous lines (CVLs) in children with haemophilia. Several retrospective studies that include data from a substantial number of patients have reported approximately 0.2-0.3 infections per 1000 catheter-days (mainly Port-A-Cath). Some studies have shown a much higher frequency of infections, 1-2/1000 catheter-days. The most plausible explanations, for the difference seen in frequency of infections with Port-A-Caths, are probably related to the protocol used for the device care and the quality of education and the compliance of the users, whether these are parents or health-care professionals. The figures are low for clinically apparent thrombosis in the larger series on record, but routine venograms were not performed in most of these series. In studies, where this has been performed, a high frequency of abnormalities (>50%) on venograms have been seen in some series but not in others. Despite obvious potential risks with CVLs, they are useful in many cases and facilitate the treatment of a serious disorder. With careful guidelines and surveillance protocols, the risk of complications should be reduced in the future.
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PMID:The risk associated with indwelling catheters in children with haemophilia. 1768 52

Surgery in infants and young children with haemophilia, when preceded by accurate diagnosis and accompanied by safe and effective factor prophylaxis, is not associated with a significant risk of haemorrhage. Haemophilic newborns undergoing circumcision or major surgery prior to diagnosis and in the absence of appropriate haemostatic prophylaxis remain as a concern. Inhibitor development has replaced haemorrhage as the major surgical complication in the developed world, largely because of the intensity of treatment used to secure haemostasis. For that reason only, essential surgery should be performed. Intracranial haemorrhage (ICH) during the neonatal period affects 3.5-4.0% of all haemophilia boys in countries with a good standard of health care, which is considerably (40-80 times) higher than expected in the normal population. Because of the high frequency of sporadic cases, ICH in the neonatal period can only be partially prevented by improved carrier diagnosis and counselling. Infections and thrombosis are the major serious complications of central venous lines. Large differences are seen in the frequency of these complications, the most plausible explanations are probably related to the protocol used for device care, the quality of education and the compliance of the users, an issue addressed in an on-going study.
Haemophilia 2008 Jul
PMID:Haemophilia in the first years of life. 1851 May 41

Inherited coagulopathies are bleeding disorders, which require treatment for life. Keeping an updated registry on these diseases is crucial for planning care, documenting prevalence of diseases and evaluating effectiveness of resources. We have analysed data from 26 treatment centres on coagulopathies in Brazil. Information included socio-demographic data, diagnosis of coagulopathies, severity of haemophilias A and B, presence and quantification of inhibitors in haemophilia, type of von Willebrand disease (VWD) and infection status for viral diseases. On 1 July 2007, there were 10 982 patients with inherited coagulopathies in Brazil, of which 6881 (62.7%) corresponded to haemophilia A, 1291 (11.7%) to haemophilia B, 2333 (21.2%) to VWD, 258 (2.4%) to other coagulopathies and 219 (2.0%) to undiagnosed bleeding disorders. Haemophilia A and B inhibitors were present in 9.9% and 1.9% of the patients, respectively. Human immunodeficiency virus infection was present is 6.5%, 4.8% and 1% of patients with haemophilia A, B and VWD, respectively. Hepatitis C virus infection was present in 34.9%, 29.7% and 12% of patients with haemophilia A, B and VWD, respectively. Infection by hepatitis B and human T-cell leukemia-lymphoma virus was also reported. This is the first report on the registry of patients with inherited coagulopathies in Brazil, supposed to be the third largest population of patients with haemophilia.
Haemophilia 2009 Jan
PMID:Registry of inherited coagulopathies in Brazil: first report. 1897 55

Use of a central venous access device (CVAD) can facilitate early introduction of home-based infusion of factor concentrate for long-term prophylaxis or immune tolerance therapy in children with bleeding disorders. The aim was to review outcomes associated with use of CVAD. Retrospective review of paediatric patients with bleeding disorders was observed at the Mayo Clinic Comprehensive Hemophilia Center. Thirty-seven CVAD were placed in 18 patients (haemophilia A [n = 15], type 3 von Willebrand disease [n = 2] and haemophilia B [n = 1]). Follow-up was for 45 952 CVAD days, and median time that CVAD remained in place was 1361 days per device. Factor VIII (FVIII) inhibitors were present in 4 of the 15 patients. Ten CVAD-related infections occurred (median, 672 days; range, 72-1941 days), of which six were in one patient with FVIII inhibitors. Overall infection rate was 0.22 (95% confidence interval [CI], 0.10-0.40) per 1000 CVAD days, with 0.11 infections in patients without FVIII inhibitors compared with a pooled incidence of 0.66 (95% CI, 0.44-0.97) reported in the literature. Indications for removal of 27 CVAD were blockage, change to peripheral venous access, catheter displacement, infection, leak in the port septum, short catheter and skin erosion. No clinically apparent thrombosis or sequelae of thrombosis were observed. Infection is the most common complication associated with CVAD use and is increased in patients who have inhibitors. The low rate of clinically apparent thrombosis reflects our practice of not screening for thrombosis. The low infection rate reflects our practice of using and reinforcing the aseptic technique.
Haemophilia 2009 Jan
PMID:Central venous access devices for paediatric patients with haemophilia: a single-institution experience. 1914 49


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