Portal vein thrombosis in a patient with severe hemophilia B: A challenging balanced management.

Key Clinical Message: The increased life expectancy in patients with hemophilia (PwH) over the last years has raised the incidence of comorbidities, including thromboembolic events. Thromboembolic events are rare in PwH and most of them occur in the presence of exogenous risk factors. There is still scarce scientific evidence on the optimal antithrombotic treatment and management approach in this population. Abstract: In the hemophilic population thromboembolic events are rare. Most of them are often multifactorial and occur in the presence of both exogenous (orthopedic surgery, intensive replacement therapy, use of central venous catheters…) and endogenous (cardiovascular diseases) risk factors. We describe the case of a 43-year-old patient with severe hemophilia B (sHB) receiving prophylaxis with eftrenonacog alfa (rFIXFc) and antithrombotic treatment due to portal vein thrombosis. The patient was treated with extended half- life factor IX (EHL-FIX) prophylaxis maintaining higher trough levels to avoid new bleeding episodes associated to the underlying disease and the use of antithrombotic therapy with low molecular weight heparin. EHL-FIX concentrates allow prolonged intervals between intravenous infusions and higher hemostatic protection thanks to increased factor trough levels. This current case report provides clinical evidence in antithrombotic management in a patient with severe hemophilia B.

Benefits of rIX-FP prophylaxis in patients with Haemophilia B: real-world evidence from a Spanish reference centre.

Standard FIX prophylaxis for PWHB require frequent injections, which has led to the development of extended half-life products like rIX-FP (albutrepenonacog alfa) that has shown good efficacy in clinical studies. This ambispective study aims to report a real-world experience with rIX-FP in a Spanish centre with PWHB who switched from SHL-FIX or began prophylaxis with rIX-FP. Five PWHB were included in this study, Four PTP switched to rIX-FP with prophylaxis every 7 days whilst one PUP started with an every-14-days regimen. 3 PTPs extended their dosing intervals to every 14 days or every 21 days. In all PTPs, median annualized spontaneous and joint bleeding rates were maintained at 0.00 and median (range) of ABR was 0.92 (0.00-2.77) after switch to rIX-FP. Mean trough level with previous product was 3.68% (SD = 2.06), while it was 7.08% (SD = 3) with all rIX-FP dosing intervals. After switching to rIX-FP, all PTP reduced their annual infusion rate between 50 and 84% and their annual FIX consumption by 61% (59-67%). This is the first reported real-world experience with albutrepenonacog alfa in a small cohort in Spain and demonstrates good bleeding control together with a reduction of the infusion rate, factor consumption and higher through factor level than previous treatment.

VHrare study: Prevalence, clinical features and management of severe rare bleeding disorders in a large cohort.

INTRODUCTION: Rare bleeding disorders (RBD) constitute 5% of total hereditary bleeding disorders, although the number could be higher, due to the presence of undiagnosed asymptomatic patients. The objective of this study was to analyze the prevalence and characteristics of patients with severe RBDs in our area. MATERIAL AND METHODS: We analyzed the patients with RBD followed at a tertiary-level hospital between January 2014 and December 2021. RESULTS: A total of 101 patients were analyzed, with a median age at diagnosis of 27.67 years (range 0-89), of which 52.47% were male. The most frequent RBD in our population was FVII deficiency. Regarding the diagnostic reason, the most frequent cause was a preoperative test and only 14.8% reported bleeding symptoms at the time of diagnosis. A genetic study was carried out in 63.36% of patients and the most frequent mutation type found was finding a missense mutation. CONCLUSIONS: The distribution of RBDs in our centre is similar to the one reported in the literature. The majority of RBDs were diagnosed from a preoperative test and this allowed preventive treatment prior to invasive procedures to avoid bleeding complications. 83% of patients did not have a pathological bleeding phenotype according to ISTH-BAT.

Neurosurgery in a patient with severe hemophilia B: an experience using eftrenonacog alfa as perioperative management.

Extended half-life FIX (EHL-FIX) concentrates have been developed with the purpose of reducing the frequency of infusions in patients with severe or moderate hemophilia B. We describe the case of a 63-year-old patient with severe hemophilia B (sHB) treated with FIX-Fc fusion protein (rFIXFc) who underwent neurosurgery.

[Pocket computers. Applications for personal digital assistants, PDAs]. / Ordenadores de bolsillo. Aplicativos informáticos para asistentes personales digitales.

In the sanitary environment there is a constant flow of all types of information; this fact obliges professionals to have at this disposition the tools which permit them to store, update, and have easy consultation access to this information. Personal Digital Assistants (PDAs) form part of these technologies which can improve both access to and storage of this information. In this article, the authors review some general techniques these PDAs have, as well as data bases which can be useful to professional practices in nursing. The authors carried out a bibliographical search over the years 1999-2006 and an Internet search for sites which describe the uses of PDAs. The authors found 94 useful applications which include: planning and management of nursing practices; nursing techniques and procedures; filing clinical data or clinical histories; surgical nursing care, pediatric nursing and geriatric nursing; pharmacology calculating and administration of drugs and fluid therapy; reference values for diagnostic tests; medical guides and treatment (diagnostic and treatment) and for surgical nursing; medical dictionaries; medical specialties; miscellaneous. For each reference, the authors provide a description of the content, bibliographical sources, operating system, memory requirements, cost, website, and the possibility to download a test version or a demo. The authors conclude that PDAs make available a wide range of useful applications in the distinct phases where nurses perform their duties, offering many possibilities to the user.

[Positive and negative aspects of pharmaceutical services in Spain: about antidepressants and antipsychotics]. / Luces y sombras de la prestación farmacéutica en España: a propósito de los antidepresivos y antipsicóticos.

Mental disorders mortality rates are low but they are extremely disabling so that the diagnosis and treatment of mental illness is an important task for public health and it is central in current therapy strategies and sanitary policy. The aim of this study is to analyse conditions influencing antidepressants and antipsychotics use in Spain from 1997 to 2004. Tryciclic antidepressants have been of first-choice in depression treatment for many years but their side effects profile are related to a lack of treatment adherence. Trying to increase tolerance, new antidepressants have been developed and research into new therapeutic uses is being done. These are some of the reasons causing an increase in medical utilization and costs. Medical boxes sold have increased from 14,14 million in 1997 to 26,76 million in 2004, meaning 168,61 and 447,11 million euros respectively. Antipsychotic use has been maintained, 11,74 million boxes in 1997 and 12,65 in 2004; however, there has been a rise in price from 61,84 to 317,46 million euros due to second generation antipsychotics prescription (mainly risperidone and olanzapine) Some conditions have contributed to current situation: increased number of diagnosed patients with depression and other mental disorders, new approved therapeutic uses and a rise in elderly people receiving new antipsychotic agents. Moreover, promotional marketing is causing an increase in new recently commercialised drugs use.