Gout, allopurinol intake and clinical outcomes in the hospitalized multimorbid elderly.

Background: Increased serum uric acid has been considered a cardiovascular risk factor but no study has assessed its relation with hospital mortality or length of stay. On the basis of data obtained from a prospective registry, the prevalence of gout/hyperuricemia and its association with these and other clinical parameters was evaluated in an Italian cohort of elderly patients acutely admitted to internal medicine or geriatric wards.

Methods: While the prevalence of gout was calculated by counting patients with this diagnosis hyperuricemia was inferred in patients taking allopurinol at hospital admission or discharge, on the assumption that this drug was only prescribed owing to the finding of high serum levels of uric acid.

The natural history of occult or angiodysplastic gastrointestinal bleeding in von Willebrand disease.

Recurrent gastrointestinal bleeding is one of the most challenging complications encountered in the management of patients with von Willebrand disease (VWD). The commonest cause is angiodysplasia, but often no cause is identified due to the difficulty in making the diagnosis. The optimal treatment to prevent recurrences remains unknown.

The real value of thrombophilia markers in identifying patients at high risk of venous thromboembolism.

Thrombophilia is defined as a condition predisposing to the development of venous thromboembolic complications. Over the past decades, there have been great advances in the understanding of the pathogenesis of venous thromboembolism (VTE) through the identification of several inherited and acquired risk factors. However, in spite of such progress, a number of questions remain unanswered.

New oral anticoagulants in thrombotic antiphospholipid syndrome.

A main goal in clinical management of patients with antiphospholipid syndrome (APS) is to prevent thrombotic recurrences and/or miscarriages. For many decades, the only available oral anticoagulant drugs have been vitamin K antagonists (VKA), which are still the cornerstone of long-term treatment of thromboembolism. However, the limits of VKA treatment are well known: narrow therapeutic window and high patient-to-patient variability of response.

Adjunct agents for bleeding.

Purpose Of Review: This review provides a summary of the most recent clinical trials evaluating the hemostatic agents for the treatment of coagulopathy associated with critical bleeding. In particular, antifibrinolytic agents (i.e.

Gender-differences in disease distribution and outcome in hospitalized elderly: data from the REPOSI study.

Background And Purpose: Women live longer and outnumber men. On the other hand, older women develop more chronic diseases and conditions such as arthritis, osteoporosis and depression, leading to a greater number of years of living with disabilities. The aim of this study was to describe whether or not there are gender differences in the demographic profile, disease distribution and outcome in a population of hospitalized elderly people.

The European standards of Haemophilia Centres.

Introduction: The European haemophilia community of professionals and patients has agreed on the principles of haemophilia care to address comprehensive optimal delivery of care which is nowadays scattered throughout Europe. Many of the health facilities call themselves Haemophilia Centres despite their variation in size, expertise and services provided. Only a small number of countries have Haemophilia Centre accreditation systems in place.

The methodology for defining the European standards for the certification of Haemophilia Centres in Europe.

Introduction: Work Package 4 Development of the standardisation criteria of the European Haemophilia Network project has the main objective of implementing a common and shared European strategy for a certification system for two levels of Haemophilia Centres: European Haemophilia Treatment Centres and European Haemophilia Comprehensive Care Centres in the Member States of the European Union.

Materials And Methods: An inclusive and participatory process for developing shared standards and criteria for the management of patients with inherited bleeding disorders has been carried out. The process has been implemented through four different consultation events involving the entire European community of stakeholders that significantly contributed in the drafting of the European Guidelines for the certification of Haemophilia Centres.

A certification/accreditation model for Haemophilia Centres in Italy.

Background: The Italian Association of Haemophilia Centres has developed a voluntary programme of professional accreditation of Haemophilia Centres, run by its members. Participation in the programme, which aims to foster staff involvement in clinical governance, includes both medical personnel and nurses.

Materials And Methods: Accreditation is awarded provided the candidate Haemophilia Centre is able to adhere to a pre-established set of quality standards and meet a number of clinical and organisational requirements, previously defined on the basis of evidence-based medicine.

MicroRNA as potential biomarker in HCV-associated diffuse large B-cell lymphoma.

Aims: To identify molecular characteristics to hepatitis C virus (HCV)-associated diffuse large B-cell lymphoma (DLBCL) through a comprehensive miRNAs expression profiling.

Methods: In this study, miRNA profiles were obtained from 37 patients with DLBCLs and 60 patients with reactive lymph nodes, equally distributed according to HCV presence. Germinal centres, from reactive lymph nodes were used as controls.

Prescription drug use among older adults in Italy: a country-wide perspective.

In Italy, prescription drug costs represent approximately 17% of total public health expenditures. Older adults commonly use multiple drugs and, for this reason, this population is responsible for a large portion of drug-related costs. In 2012, public expenditure for pharmaceuticals in primary care exceeded 11 billion Euros (approximately 15.

Cooperation between pathologists and clinicians allows a better diagnosis of Philadelphia chromosome-negative myeloproliferative neoplasms.

As no specific genetic lesions have yet been identified, the diagnosis of Philadelphia chromosome-negative myeloproliferative neoplasms is based on a simultaneous evaluation of the clinical, morphological and molecular features defined by the updated WHO classification, which allow most cases of full-blown disease to be classified. Nevertheless, about 10-15% of the patients have unclassifiable myeloproliferative neoplasms, most of whom are in the prodromal (early) phase of disease and identified by the presence of the JAK2 mutation, but lack the complete phenotype required by the WHO classification. The detection of these prodromal phases is extremely important in order to prevent dramatic thrombo-hemorrhagic complications and improve prognosis.

Orthopaedic surgery in patients with von Willebrand disease.

Patients with von Willebrand disease (VWD) may need orthopaedic surgery because of disabling chronic arthropathy due to recurrent joint bleeding. They may also require this surgery independently of their haemostasis disorder. Knowledge regarding the management of orthopaedic surgery in VWD is limited.

Pharmacokinetics and safety of a novel recombinant human von Willebrand factor manufactured with a plasma-free method: a prospective clinical trial.

Safety and pharmacokinetics (PK) of recombinant von Willebrand factor (rVWF) combined at a fixed ratio with recombinant factor VIII (rFVIII) were investigated in 32 subjects with type 3 or severe type 1 von Willebrand disease (VWD) in a prospective phase 1, multicenter, randomized clinical trial. rVWF was well tolerated and no thrombotic events, inhibitors, or serious adverse events were observed. The PK of rVWF ristocetin cofactor activity, VWF antigen, and collagen-binding activity were similar to those of the comparator plasma-derived (pd) VWF-pdFVIII.

Investigational drugs for coagulation disorders.

Introduction: The current standard treatment in persons with hemophilia (PWH) is prophylaxis, given intravenously twice or thrice weekly, which is associated with a non negligible burden on patients' quality of life. Therefore the main attempts aiming to improve the management of PWH are targeted towards the development of a new generation of coagulation factors endowed with properties facilitating prophylaxis and/or a better control of bleeding.

Areas Covered: This article summarizes the main results obtained so far in the development of new antihemophilic products, and emphasizes the formidable requirements imposed upon by regulatory agencies to get marketing authorization for new drugs, which make progress in this field difficult.

Is haemophilia B less severe than haemophilia A?

A number of observations suggest that severe factor IX deficiency (<1%) may be less clinically severe than the corresponding factor VIII deficiency: (i) Less factor consumption. There is evidence that patients with haemophilia B (HB) consume yearly less FIX for replacement therapy than patients with haemophilia (HA). Patient registries and data from various sources indicate that regular prophylaxis is implemented less frequently in HB.

Hemostatic defects in liver and renal dysfunction.

Multiple and complex abnormalities of hemostasis are revealed by laboratory tests in such common diseases as cirrhosis and end-stage renal insufficiency. Because these abnormalities are associated with a bleeding tendency, a causal relationship is plausible. Accordingly, an array of transfusional and nontransfusional medications that improve or correct these abnormalities is used to prevent or stop hemorrhage.

Evolution of the European guidelines for the clinical development of factor VIII products: little progress towards improved patient management.

In the process of clinical development and licensing of factor VIII (FVIII) products for treatment of haemophilia A, the safety concerns generated in the 1980s by the risk of pathogen transmission were tremendously reduced by the implementation of an array of methods for inactivation/removal of blood borne pathogens. The current focus on the risk of FVIII inhibitors does not stem from a new awareness, because this multifactorial complication has long been recognized. With this background, I believe that the current European regulatory guidelines for the clinical development and licensing of FVIII products fail to reflect the tremendous progress made in terms of clinical efficacy and safety, because they are witnessing a continuous increase in the demands from health agencies to the point that clinical studies have become more and more difficult to carry out.