Search results for "philia"

Secondary prophylaxis vs. on-demand treatment to improve quality of life in severe adult haemophilia A patients: a prospective study in a single cent…

2013

Background Retrospective publications show a decrease in the bleeding frequency and an improvement in the quality of life (QoL) in severe adult haemophilia A (SAHA) after switching from the on-demand treatment (DT) to secondary prophylaxis (SP). But there are no prospective studies which demonstrate, using a haemophilia-specific questionnaire, an improvement in the QoL after such treatment change. The main objective of this study is to prospectively compare the QoL and the musculoskeletal assessment after switching from DT to SP in SAHA using the A36 Hemofilia-QoL®. As secondary objective, we compare the haemarthrosis frequency and factor VIII consumption in DT and SP during a similar perio…

Predictive factors of immune tolerance treatment response in severe haemophilia A patients with inhibitors: A real‐world report from a single centre,…

2019

De novo mutation in a male patient with Fabry disease: a case report

2014

Abstract Background Fabry disease is an X-linked inherited metabolic condition where the deficit of the α-galactosidase A enzyme, encoded by the GLA gene, leads to glycosphingolipid storage, mainly globotriaosylceramide. To date, more than 600 mutations have been identified in human GLA gene that are responsible for FD, including missense and nonsense mutations, small and large deletions. Such mutations are usually inherited, and cases of de novo onset occur rarely. Case presentation In this article we report an interesting case of a 44-year-old male patient suffering from a severe form of Fabry disease, with negative family history. The patient showed signs such as cornea verticillata, ang…

Airway neutrophilia in COPD is not associated with increased neutrophil survival

2006

Neutrophilic airway inflammation is a prominent feature of chronic obstructive pulmonary disease (COPD) and correlates with disease severity. The mechanisms that determine the extent of neutrophilia could involve increased influx or prolonged survival of neutrophils. The aim of the study was to assess whether neutrophil pro-survival mechanisms are increased in the airways of subjects with COPD owing to the presence of anti-apoptotic factors in the bronchial lining fluid. Induced sputum samples were collected from 20 subjects with stable COPD, 14 healthy smokers and 14 healthy controls. Quantification of apoptotic neutrophils was based on typical morphological cell changes. Anti-apoptotic, p…

Reslizumab as add-on therapy in patients with refractory asthma

2020

### Key messages #### What is the key question? #### What is the bottom line? #### Why read on? Asthma is a common disease, affecting an estimated 334 million people worldwide, with considerable impact on quality of life and high associated costs.1–3 Asthma severity is assessed retrospectively from the level of treatment required to control symptoms and exacerbations. Approximately 5%–10% of patients with asthma are believed to suffer from severe disease.4 Patients with severe asthma typically require ongoing maintenance therapy with high-dose inhaled corticosteroid (ICS)/long-acting beta-agonist (LABA).2 Furthermore, systemic corticosteroids (SCS) are often required for potentially life-th…

Foot health-related quality of life in hemophiliacs: A case-control study

2020

Background: Haemophilia is considered as a chronic genetic disease related with alteration in coagulation mechanism which affects to health related quality of life (HQoL). Purpose: The goal compared marks of HQoL, in haemophiliacs with respect non haemophilic subjects. Methods: A population of 74 subjects, were recruited from association of haemophilic illness separated in haemophilic subjects (n = 37) and no haemophilic (n = 37). For subjects who suffered haemophilia were enlisted from the association of haemophilic illness after a seminar of 45 minutes to them and to their relatives about foot health. Control subjects, were recruited from their relatives who live with the patient. The mar…

Test–Retest Reliability and Temporal Agreement of Direct and Indirect Sexual Interest Measures

2020

The Explicit and Implicit Sexual Interest Profile (EISIP) is a multimethod measure of sexual interest in children and adults. It combines indirect latency-based measures such as the Implicit Association Test (IAT), Viewing Time (VT), and explicit self-report measures. This study examined test–retest reliability and absolute temporal agreement of the EISIP over a 2-week interval in persons who were convicted of sexual offenses against children ( n = 33) and nonoffending controls ( n = 48). Test–retest reliability of the aggregated EISIP measures was high across the whole sample ( rtt = .90, intraclass correlation coefficient [ICC] = .90) with the IAT yielding the lowest retest correlations …

Correlations Between Clinical and Histological Aspects in Nasal Polyposis

2008

Introduction The histological study of nasal polyps does not reveal any specific lesions but eosinophilic infiltration nasal mucosae seems to be characteristic of nasal polyposis. The aim of this work is to study possible links between certain histological and clinical aspects in nasal polyposis. Furthermore, we attempt to compare the quantification of tissue eosinophilia according to the number of eosinophils per field with the percentage figure obtained with respect to the total of inflammatory cells. Material and method We have studied 40 patients with idiopathic bilateral nasal polyposis, assessing the correlations between various clinical aspects such as their endoscopic and radiologic…

Deferral of assessment of pulmonary embolism

2007

We evaluated a simplified algorithm for safely postponing diagnostic imaging for pulmonary embolism (PE). At the index visit, patients were identified as being at high or low risk of PE; the former received full dosage low molecular weight heparin while the latter were left untreated until performance of diagnostic imaging (max 72 hours). During this period, no thromboembolic events occurred in low-risk patients (0/211, 0.% [upper 95% CI 0.9%]); only one event occurred in those at high-risk (1/125, 0.8% [upper 95% CI, 1.2]). Our study demonstrates that diagnostic imaging for PE can be safely deferred for up to 3 days.

Cancer patients requiring interruption of long-term warfarin because of surgery or chemotherapy induced thrombocytopenia: The use of fixed sub-therap…

2012

No data are available regarding the management of cancer patients requiring interruption of long-term vitamin-K antagonist (VKA) therapy. For this purpose, we tested the efficacy and safety of fixed doses of low-molecular weight heparin (LMWH) in substitution of VKA because of invasive procedures or chemotherapy-induced thrombocytopenia. In cancer patients on VKA, therapy was discontinued 5 ± 1 days before surgery or chemotherapy. Heparin was given at prophylactic dosage in patients at low risk and at fixed subtherapeutic doses (3,800 or 4,000 UI anti-FXa, b.i.d.) in those at high-risk for thrombosis. LMWH was reinitiated 12 hr after surgery and VKA the day after. In patients receiving chem…