Search results for "philia"
showing 10 items of 299 documents
ABO Blood Group and Inhibitor Risk in Severe Hemophilia A Patients: A Study from the Italian Association of Hemophilia Centers
2021
AbstractConsidering the profound influence exerted by the ABO blood group system on hemostasis, mainly through the von Willebrand factor and factor VIII (FVIII) complex, we have conducted a study evaluating the possible role of blood type on the risk of inhibitor development in hemophilia A. A total of 287 consecutive Caucasian patients with severe hemophilia A (202 without FVIII inhibitors and 85 with FVIII inhibitors) followed at seven Italian Hemophilia Treatment Centers belonging to the Italian Association of Hemophilia Centers (AICE) were included in the study. A higher prevalence of O blood group was detected in patients without inhibitors as compared in inhibitor patients (55 vs. 30.…
Effects of Conjugated Linoleic Acid and Endurance Training on Peripheral Blood and Bone Marrow of Trained Mice
2007
Fat supplements, especially conjugated linoleic acid (CLA), are increasingly popular ergogenic aids among endurance athletes. To evaluate the importance of fat supplementation in the practice of endurance sports, we investigated the effects of CLA supplementation on body weight, muscle hypertrophy, peripheral blood composition, and bone marrow composition in healthy, young, endurance-trained mice. Young, healthy mice were subdivided into control, trained, and treated groups, according to their running attitudes. Training was performed over a period of 6 weeks on a treadmill, at a gradually increasing duration and speed. CLA-treated groups were gavaged with 0.425 mg x d(-1) CLA supplement fo…
Factor VIII products and inhibitor development in severe hemophilia A
2013
For previously untreated children with severe hemophilia A, it is unclear whether the type of factor VIII product administered and switching among products are associated with the development of clinically relevant inhibitory antibodies (inhibitor development). We evaluated 574 consecutive patients with severe hemophilia A (factor VIII activity, <0.01 IU per milliliter) who were born between 2000 and 2010 and collected data on all clotting-factor administration for up to 75 exposure days. The primary outcome was inhibitor development, which was defined as at least two positive inhibitor tests with decreased in vivo recovery of factor VIII levels. Inhibitory antibodies developed in 177 of th…
Low dose of aPCC after the initial treatment in acquired haemophilia A is useful to reduce bleeding relapses: Data from the FAIR registry
2019
Background: Bypassing agents are the first line therapy in patients with acquired haemophilia A (AHA). Activated prothrombin complex concentrate (aPCC) proved to be effective as initial treatment, but 20% of patients (pts) had relapses. aPCC as short-term prophylaxis to reduce subsequent bleeds is still not clear. Aim: To evaluate whether a short-term prophylaxis with low dose of aPCC can reduce bleeding relapses after initial AHA treatment, maintaining safety. Methods: The FAIR Registry is a retrospective-prospective study started on December 2012, that collected data on all pts with AHA treated with aPCC in 12 Italian Haemophilia Centers. All statistical analyses were carried out in the 5…
Impairment of nasal mucociliary clearance after radiotherapy for childhood head cancer.
2001
Background Radiotherapy of the head region in children is known to cause long-term sequelae, such as facial, dental, and ocular abnormalities. We investigated whether a decreased nasal mucociliary function occurs after radiotherapy of the head in children. Methods A saccharin/charcoal test was performed in 20 children treated with radiotherapy of the head and in 20 controls, age-matched and gender-matched. Results We found a decreased nasal mucociliary clearance (lower percentage of responses (p = 0083) and longer mucociliary transport times (p = .0001) in the patients compared with the controls. The radiotherapy dosage influenced the response to the test (p = .0046). Conclusions Irradiatio…
Balance evaluation in haemophilic preadolescent patients using Nintendo Wii Balance Board®
2016
Introduction: Alterations in the musculoskeletal system, especially in the lower limbs, limit physical activity and affect balance and walking. Postural impairments in haemophilic preteens could increase the risk of bleeding events and deteriorate the physical condition, promoting the progression of haemophilic arthropathy. Aim: This study aims to evaluate static postural balance in haemophilic children, assessed by means of the Wii Balance Board® (WBB). Methods: Nineteen children with haemophilia and 19 without haemophilia aged 9-10 years, have participated in this study. Postural balance was assessed by performing four tests, each one lasting 15 s: bipodal eyes open (BEO), bipodal eyes cl…
Correlation between FIX genotype and pharmacokinetics of Nonacog alpha according to a multicentre Italian study
2016
Introduction Pharmacokinetic (PK) studies on recombinant FIX concentrate, Nonacog alpha, were conducted with different sampling time designs which gave rise to not complete and homogenous outcomes. In addition, patient's FIX genotype/PK relationship has never been investigated. Aim Investigate how different sampling times may affect PK parameters and try to find a FIX genotype/PK relationship. Patients and Methods A cohort pharmacokinetic, Nonacog Alpha single-dose, open-label, non-comparative study was conducted in eight Comprehensive Care Haemophilia Centres in Italy. Seventeen previously treated moderate or severe haemophilia B patients were enrolled. Factors IX:C one-stage clotting assa…
Positional brain ischemia with MCA occlusion successfully treated with extra-intracranial bypass.
2010
408 Positional cerebral ischemia (PCI) is an extremely rare condition in which dramatic central nervous system deficits are triggered by standing in patients with severe stenosis or occlusion of the major brain-supplying blood vessels [1] . Because PCI is generally associated with orthostatic hypotension [2, 3] , a hemodynamic mechanism has been proposed [4] . However, PCI has been reported even in patients without orthostatic hypotension [1] . The treatment is medical, but surgery can be considered in refractory cases (endarterectomy for carotid artery stenosis or either highor low-flow extra-intracranial bypass – for carotid occlusion) [3] . We report a case of PCI associated with middle …
Acute painful paraplegia in a 49-year-old man with allergic asthma.
2014
We present a case of a 49-year-old man, with a 10-year history of bronchial asthma and nasal polyposis, who developed acutely painful paraplegia and paresthesias. Laboratory data showed elevated blood creatine kinase levels and myoglobinuria, which were diagnostic for rhabdomyolysis but only partially explained the neurological deficit. Electrophysiological studies revealed a sensorimotor neuropathy of multiple mononeuritis type. The patient also had leucocytosis with marked eosinophilia and antineutrophil cytoplasmic autoantibodies. Bronchial biopsies showed inflammatory infiltrates with a prevalence of eosinophils. All these findings led us to diagnose eosinophilic granulomatosis with pol…
[Contraception in chronically ill adolescents].
2003
Contraception in chronically ill adolescents has to consider side effects with regard to metabolic or hematological disease. Additionally the compliance of the patient has to be taken in account when consulting about contraceptives. In juvenile diabetics with good glycemic control and metabolic diseases of the amino acids oral contraceptives are justifiable. Adolescents with galactose-mia mostly suffer from premature ovarian failure and therefore have to be controlled wether they need contraception at all. Young women with sickle-cell anemia are at risk of thrombophilia. They should not be given hormonal contraceptives. In v. Willebrand-Jurgens-Syndrome oral contraceptives are favourable be…