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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a case of severe factor V (FV) deficiency (<1%) associated with multiple episodes of intracranial bleeding which presented at birth. The clinical course was further complicated by the development of an inhibitor, episodes of sepsis and cardiac failure. The management using virally inactivated FFP and platelets is discussed.
Haemophilia 2000 Jan
PMID:Severe factor V deficiency and neonatal intracranial haemorrhage: a case report. 1063 41

Joint replacement in HIV-positive patients remains uncommon, with most experience gained in patients with haemophilia. We analysed retrospectively the outcome of 102 replacement arthroplasties in 73 HIV-positive patients from eight specialist haemophilia centres. Of these, 91 were primary procedures. The mean age of the patients at surgery was 39 years, and the median follow-up was for five years. The overall rate of deep sepsis was 18.7% for primary procedures and 36.3% for revisions. This is a much higher rate of infection than that seen in normal populations. A total of 44% of infections resolved fully after medical and/or surgical treatment. The benefits of arthroplasty in haemophilic patients are well established but the rates of complications are high. As this large study has demonstrated, high rates of infection occur, but survivorship analysis strongly suggests that most patients already diagnosed with HIV infection at the time of surgery should derive many years of symptomatic relief after a successful joint replacement. Careful counselling and education of both patients and healthcare workers before operation are therefore essential.
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PMID:Infected joint replacements in HIV-positive patients with haemophilia. 1160 22

We describe the experience of a single medical centre with continuous factor VIII (FVIII) infusion therapy in a cohort of patients undergoing elective surgery. Twenty-eight patients had a total of 45 procedures. Intraoperative haemostasis was considered excellent in all 45 cases. FVIII levels were maintained between 46% and 191% of normal (median, 103%) for 2-7 days. Bleeding occurred after five procedures (11%) at times when factor VIII levels were within haemostatic range. No patient required reoperation to control bleeding. There were no cases of sepsis related to continuous infusion of factor VIII. We conclude that continuous infusion: (1) is a safe and effective means of replacement therapy in patients with haemophilia undergoing surgery; (2) provides easier monitoring and more constant coagulation factor levels; and (3) has the potential to decrease the cost of replacement therapy by reducing overall usage of product.
Haemophilia 2002 Sep
PMID:Continuous factor VIII infusion therapy in patients with haemophilia A undergoing surgical procedures with plasma-derived or recombinant factor VIII concentrates. 1219 70

Historically, the leading cause of death among persons with haemophilia and other congenital coagulation disorders was uncontrolled bleeding. Mortality was associated with severe deficiency of coagulation factors VIII or IX and especially with high-titre antifactor neutralizing antibodies (inhibitors). The catastrophic contamination of plasma donor pools with human immunodeficiency virus (HIV) resulted in acquired immunodeficiency syndrome replacing haemorrhage as the leading cause of death among persons with haemophilia. Rather little has been written, however, about mortality among those not infected with HIV. The objective of this study was to identify conditions associated with all-cause mortality among HIV-uninfected patients who were followed for a mean of 8.8 years in the Multicentre Hemophilia Cohort Study. Among the 364 children (mean age 8 years), there were four deaths; two related to cancer, one to trauma, and the fourth to haemorrhage, end-stage liver disease and sepsis. Among the 387 HIV-uninfected adults (mean age 35 years) there were 29 deaths, with haemorrhage the leading cause of death, followed by hepatic, stroke and cancer deaths. Prognostic factors for all-cause mortality among the adults included haemophilia Type A with neutralizing antibodies [age-adjusted relative rate (RR) 3.1, 95% confidence interval (CI) 1.4-6.9] and serologic evidence of both hepatitis B and C virus (RR 4.1, 95% CI 0.97-17.6). Although hepatitis C viral load was slightly lower in patients with hepatitis B virus surface antigenaemia, it was unrelated to vital status. We conclude that causes of death and prognostic factors for current HIV-uninfected haemophilia patients are similar to those noted before the HIV epidemic. Better understanding, prevention and control of neutralizing antibodies and hepatitis infections may substantially improve longevity for people with haemophilia.
Haemophilia 2002 Sep
PMID:Factors predictive of death among HIV-uninfected persons with haemophilia and other congenital coagulation disorders. 1219 76

Acute leukaemia is the commonest form of malignancy in childhood. The coincidental development of leukaemia in children or adults with haemophilia is extremely rare, although cases of leukaemia and other malignancies have been reported previously in HIV-positive subjects. Of a total of 440 people with haemophilia registered with our society, two were diagnosed with acute leukaemia last year. The development of leukaemia in a subject with haemophilia has previously been reported from our country in 1985, but the negative HIV status of these recent cases is very interesting. The first case involved a 14-year-old boy with moderate haemophilia A, who developed acute lymphoblastic leukaemia (ALL) [French-American-British (FAB) classification L2]. The second subject was a 16-year-old boy who had moderately severe haemophilia A with no previous family history, and developed acute nonlymphocytic (myelomonocytic) leukaemia (FAB-M4). Both patients received conventional chemotherapy and this report discusses the potential problems in management of such cases, including diagnosis and administration of chemotherapy in subjects with a pre-existing haemorrhagic disorder. Extensive cutaneous and mucosal bleeding, as well as bleeds in joints previously affected by haemarthrosis and alterations of haematological values were all initially suggestive of the development of inhibitors against factor VIII, but the appearance of blasts in the peripheral blood and bone marrow led to the definitive diagnosis. The risk of bleeding, due to the combination of both leukaemia and the consequences of the chemotherapy, was overcome by the administration of coagulation factor concentrates (daily initially followed by prophylactic doses after successful induction of remission in both patients). The young patient with ALL is now receiving the maintenance phase of the Children's Cancer Study Group 1961 protocol and is in the 15th month of follow-up, without any complications. The other case relapsed in the seventh month, developing enterobacter sepsis, and died. An important lesson to be learnt from these cases is that the possible diagnosis of leukaemia should not be overlooked in a patient with haemophilia and severe haemorrhagic problems, if the first-line differential diagnosis of inhibitor development against factor VIII (or IX) has been excluded.
Haemophilia 2002 Sep
PMID:Two patients with haemophilia and acute leukaemia. 1219 82

We conducted a retrospective survey of our experience with central venous access devices (CVADs) implanted in children with haemophilia seen at the Vanderbilt Hemostasis-Thrombosis Clinic from 1986 to 2000. Following discussion with parents on the merits and risks associated with the use of CVADs for immune tolerance induction or factor prophylaxis, catheters were inserted under sterile technique in the operating room. One nurse provided demonstration and teaching about catheter care and access. Thirty central venous catheters were inserted in 22 children. Our survey revealed that the two most common complications associated with central venous catheters were bacteraemia and thrombosis. We found a sepsis rate of 0.30/1000 catheter-days or one episode of bacteraemia for every 3346 days of catheter use. The thrombosis rate of our cohort was 0.13/1000 catheter-days or one episode of thrombosis for every 7529 days of catheter use. Uncomplicated venous access is essential in children with severe haemophilia who require prophylaxis or immune tolerance induction. While infection was the most common complication observed in our series, we experienced a lower overall infection rate than several reported series. Catheter thrombosis and subsequent obstruction may occur as a result of intraluminal fibrin deposits. We conclude that the use of implantable central venous catheters is an effective method for accessing children with haemophilia. We accept that the benefits of CVADs in the treatment of paediatric haemophilia patients outweigh the previously documented risks. Future prospective studies should be designed to define all associated risks and to determine effective strategies to reduce them.
Haemophilia 2003 Jan
PMID:Complications of central venous access devices in paediatric haemophilia patients. 1255 78

In the first part of the medical education article on haemostatic disorders in ENT patients the basic physiology of haemostasis and main diagnostic tools were presented and discussed. The second part presents disorders of the coagulation system,thrombocyte function and blood vessels with special emphasis on clinical practice in ENT surgery. In this context, haemophilia A and B, von Willebrand disease and different forms of thrombocytopenia are of main clinical importance. Some underlying diseases such as malignomas, renal and hepatic insufficiency in combination with drug therapy (e.g.anticoagulants and thrombocyte function inhibitors) play an important role in clinical practice as well. Sepsis and haemorrhage may lead to disseminated intravascular coagulation (DIC). Beside a systematic review, important haemostatic disorders are illustrated with case reports.
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PMID:[Haemostatic disorders in ENT patients. Part 2: Pathophysiology, diagnostics, clinical feature and therapy]. 1262 55

The aim of this retrospective review was to assess the overall effectiveness of prophylaxis when compared with on-demand treatment of haemophilic patients. Twenty-five children (22 with severe haemophilia A and three with severe haemophilia B) were evaluated. Five haemophilia A patients received primary prophylaxis (instituted before the onset of any joint bleed) while the other 17 haemophilia A and all three haemophilia B patients were on secondary prophylaxis. We compared factor usage, number of bleeding episodes, emergency room (ER) visits and hospitalizations while on prophylaxis to those while on demand therapy. All subjects were male, the median age at time of review was 11.4 years and at start of prophylaxis was 4.5 years. Thirteen of the 25 patients (52%) required indwelling venous catheters for access, seven of these had one or more (one-six) episodes of line sepsis. Haemophilia A patients received an average of 23.8 U kg(-1) (20-30 U kg(-1)) of recombinant factor VIII three times a week while haemophilia B patients received 50 U kg(-1) recombinant FIX twice weekly. There was a significant reduction in the mean number of major bleeds on prophylaxis from 15.5 to 1.9 per year and a significant decrease in target joints, ER visits and hospitalizations. Although factor usage per year was higher on prophylaxis, there was an overall reduction in number of bleeds and resultant decrease in hospitalizations and ER visits. By preventing new target joints, prophylaxis can lead to reduction in long-term morbidity and a better quality of life despite increased central lines and higher factor usage.
Haemophilia 2003 May
PMID:The overall effectiveness of prophylaxis in severe haemophilia. 1269 17

Recombinant factor VIIa (rFVIIa; NovoSeven(R), Novo Nordisk, Bagsvaerd, Denmark) has been used for many years in the successful management of bleeding episodes in patients with hemophilia and inhibitors. More recently, rFVIIa has also shown considerable success as a hemostatic agent in trauma and surgery patients without pre-existing coagulopathy. Despite extensive and varied usage of rFVIIa, the incidence of serious adverse events associated with its use is less than 1%; however, there remain concerns regarding the agent's potential to induce thrombosis. This paper will review the safety profile of rFVIIa by examining existing clinical evidence, and will demonstrate that the isolated thrombotic events reported following rFVIIa treatment are due primarily to an improvement in the coagulation mechanism rather than rFVIIa treatment per se. The demonstrated safety of rFVIIa is probably due to its localization to injured areas of the vascular tree by binding to tissue factor (TF) and activated platelets at the bleeding site, thus avoiding systemic activation of coagulation. Finally, those situations in which rFVIIa therapy may not be safe, such as disseminated intravascular coagulation (DIC) and sepsis, will also be discussed.
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PMID:Safety profile of recombinant factor VIIa. 1487 30

Central venous catheters (CVC) are frequently used in children with haemophilia to deliver factor infusions for the treatment or prophylaxis of bleeding. Complications of CVCs in patients with haemophilia include thrombosis and infection. We report a young boy with severe haemophilia A and an inhibitor who developed disseminated Staphylococcus aureus infection most likely related to a CVC. To our knowledge, this is the first reported case of fatal sepsis secondary to a CVC in a patient with haemophilia.
Haemophilia 2006 Mar
PMID:Fatal central venous catheter-related infection in haemophilia. 1647 95


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