Search results for "AEM"
showing 10 items of 1006 documents
Immune tolerance induction with moroctocog-alpha (Refacto/Refacto AF) in a population of Italian haemophilia A patients with high-titre inhibitors: D…
2019
Background: The appearance of inhibitors is the most serious complication in haemophilia A (HA) patients. The primary objective is their eradication. Up to date, immune tolerance induction (ITI) was the only therapeutic option to achieve this. Aim: To assess the efficacy of moroctocog-alpha as an ITI regimen in a population of HA patients with high-titre inhibitors. Methods: The REF.IT Registry is a retrospective-prospective study that collected data on all patients with HA and high-titre inhibitors treated with moroctocog-alpha as an ITI regimen at twelve Italian Haemophilia Centres. Results: We enrolled 27 patients, 85.2% were children. All patients were high responders, 88.9% had severe …
Long-term sequential deferiprone-deferoxamine versus deferiprone alone for thalassemia major patients: a randomised clinical trial
2009
A multicentre randomized open-label trial was designed to assess the effectiveness of long-term sequential deferiprone–deferoxamine (DFO–DFP) versus DFP alone to treat thalassaemia major (TM). DFP at 75 mg/kg, divided into three oral daily doses, for 4 d/week and DFO by subcutaneous infusion (8–12 h) at 50 mg/kg per day for the remaining 3 d/week was compared with DFP alone at 75 mg/kg, administered 7 d/week during a 5-year follow-up. The main outcome measures were differences between multiple observations of serum ferritin concentrations. Secondary outcomes were survival analysis, adverse events, and costs. Consecutive thalassaemia patients (275) were assessed for eligibility; 213 of these…
Percutaneous liver biopsy: a safe outpatient procedure?
2001
Following the discovery of hepatitis C virus, more liver biopsies (LB) than before are being performed to assess the severity of liver disease. In this study, following the recommendations for outpatient LB made by the Patient Care Committee of the American Gastroenterological Association, we assessed the feasibility and benefits of LB performed as an outpatient versus inpatient procedure over the last 7 years in our centre. The study included 1,581 patients consecutively examined in our institute; all LBs were performed by a single operator with a 16-gauge needle using the Menghini technique, and in all cases the puncture site was determined using prebiopsy ultrasound. Liver lesions were c…
Aneurysm surgery of patients in poor grade condition. Indications and experience
1994
Out of a total of 196 patients admitted with aneurysmal subarachnoid haemorrhage (SAH) to the neurological department in Mainz over a 42 month period, 48 patients (24.5%) were considered as grade IV or V on admission. Aneurysm surgery within 48 hours after SAH was performed in 56.3% of these patients, 2% were operated between day 3 and 7 and 16.6% were operated after day 7. 25% did not undergo operation because of severe neurological deficit and brain damage. The overall outcome according to the Glasgow outcome scale in the surgically treated group was full recovery in 11.1%, moderate disability in 16.7%, severe disability in 47.2%, vegetative state in 2.8% and death in 22.2%. All patients …
The PFO anatomy evaluation as possible tool to stratify the associated risks and the benefits arising from the closure
2010
Aims According to the current guidelines, the patent foramen ovale (PFO) is still considered a qualitative factor and, as a consequence, its closure is recommended just on the basis of its ‘presence’. Methods and results In the year 2008, we evaluated 25 patients (mean age 62.7) with acute cerebrovascular event and 92 patients (mean age 27.3) suffering from migraine with aura. No PFO was reported in 79 patients. A venous-to-arterial circulation shunt had been shown in 38 patients (29 subjects with migraine and 9 subjects with prior stroke). According to the number of microbubbles arrived during the Valsava manoeuvre, we found: 25 small PFO, 6 moderate PFO, and 6 severe PFO. In the baseline …
Assessment of Kinect V2 for elbow range of motion estimation in people with haemophilia using an angle correction model
2018
Introduction The joint range of motion (ROM) is an important clinical parameter used to assess the loss of functionality resulting from joint bleedings in people with haemophilia. These episodes require a close follow-up and, to decrease patients' hospital dependence, telemedicine tools are needed. Therefore, this study is aimed to analyse the validity of the Microsoft Kinect V2 sensor with corrected angle measurement to be used in the monitoring of elbow ROM in people with haemophilia. Methods A convenience sample of 10 healthy controls (CG) and 10 patients with haemophilia with elbow arthropathy (HG) participated in this study. Full ROM of elbow joints was measured in the frontal view wit…
Angina bullosa haemorrhagica:a 14-year multi-institutional retrospective study from Brazil and literature review
2021
Background Angina bullosa haemorrhagica (ABH) is characterized by the recurrent appearance of blood blisters on the oral mucosa, mainly in adults' soft palate. In general, the blisters rupture spontaneously, lacking the necessity for biopsy. We report the clinical features of 23 ABH cases, emphasizing the clinical behavior and the management of these conditions. Material and Methods A retrospective descriptive cross-sectional study was performed. A total of 12,727 clinical records of oral and maxillofacial lesions from four dental services in Brazil were analyzed. Clinical data were collected from the clinical records and evaluated. Results The series comprised 12 males (52.2%) and 11 femal…
Cilioretinal artery occlusion.
2015
Background: The aim of this study was to evaluate characteristic findings and functional outcome of this rare type of retinal vascular occlusion in a large patient series. Patients and Methods: A retrospective chart review of all patients with a cilioretinal artery obstruction (CAO) seen at the University Eye Clinic Mainz (Germany) over a ten-year period between 1/1999 and 12/2008 was undertaken. The mean follow-up amounted to 42 months. Results: Thirty-three eyes were diagnosed with CAO. Three distinct groups were differentiated: (i) isolated CAO in 20/33 eyes; visual outcome achieving 20/50 in 17/20 eyes; (ii) CAO combined with central retinal vein occlusion in 9/33 eyes; visual outcome a…
Neuromuscular control during gait in people with haemophilic arthropathy
2019
Introduction: Effects of haemophilic arthropathy on neuromuscular control during gait are currently unknown. Aims: (a) To assess how haemophilic arthropathy affects the complexity of neuromuscular control during gait; (b) To investigate the relationship between complexity of neuromuscular control and joint impairment. Methods: Thirteen control subjects (CG) walked overground at their preferred and a slow velocity and thirteen people with haemophilic arthropathy (PWHA) walking at their preferred velocity. Surface electromyography (EMG) was collected from eleven leg muscles. Electromyography variance explained by muscle synergies (sets of co-activated muscles that can be recruited by a single…
Prognostic value of N-terminal pro-brain natriuretic peptide in elderly people with acute myocardial infarction: prospective observational study
2009
Objective To examine the influence of age on the predictive value of N-terminal pro-brain natriuretic (NT-proBNP) peptide assay in acute myocardial infarction. Design Prospective observational study. Setting All intensive care units in one French region. Participants 3291 consecutive patients admitted for an acute myocardial infarction, from the RICO survey (a French regional survey for acute myocardial infarction). Main outcome measure Cardiovascular death at 1 year. Results Among the 3291 participants, mean age was 68 (SD 14) years and 2356 (72%) were men. In the study population, the median NT-proBNP concentration was 1053 (interquartile range 300-3472) pg/ml. Median values for age quart…