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Tyr2105Cys mutation in exon 22 of FVIII gene is a risk factor for the development of inhibitors in patients with mild/moderate haemophilia A

We report the case of a patient with mild haemophilia A, due to a Tyr2105Cys mutation in exon 22 of the C1 domain, who developed a high-titre factor VIII inhibitor (maximum titre 1600 BU) with recurrent severe haemorrhages and fatal intracranial bleeding. Based on published data, it appears that although this mutation occurs rarely in patients with mild or moderate haemophilia A, it is frequently

Validation of the Dutch version of the Hip Disability and Osteoarthritis Outcome Score

Introduction: The Hip disability and Osteoarthritis Outcome Score (HOOS) was constructed in Sweden; this questionnaire has proved to be valid for persons with hip disability with or without hip osteoarthritis (OA) and with high demands of physical function. Objective: The objective of this study was to evaluate the internal consistency, reliability, construct validity, and floor and ceiling effect

Automated quantification of myocardial infarction from MR images by accounting for partial volume effects: animal, phantom, and human study.

Ethics committees approved human and animal study components; informed written consent was provided (prospective human study [20 men; mean age, 62 years]) or waived (retrospective human study [16 men, four women; mean age, 59 years]). The purpose of this study was to prospectively evaluate a clinically applicable method, accounting for the partial volume effect, to automatically quantify myocardia

Landscape composition affects habitat use and foraging flight distances in breeding European starlings

The recent decline of the European starling in northern Europe has been associated with changes in agricultural land-use. We investigated how agricultural land-use affected starling habitat use and home-range size in a heterogeneous landscape in southern Sweden. Breeding starlings primarily foraged in pastures, a habitat with a high availability of potential food for nestlings. When the availabili

Numerical study of localization in soil systems

A numerical study of the mechanical behavior of heterogeneous soil systems, consisting of a bulk of sand with embedded stiff gravel inclusions or soft clay inclusions, is performed. A solution scheme using parallel computing is employed when analyzing two different categories of problems. First, a homogenization problem is studied, where use of a single representative volume element subjected to p

Recombinant factor VIIa (rFVIIa): its potential role as a hemostatic agent

Recombinant activated coagulation factor VII (rFVIIa) was developed for the treatment of patients with hemophilia who have developed inhibitors against the factor they are missing. Hemophilia is a serious bleeding disorder and patients with hemophilia develop repeated spontaneous CNS, joint and muscle bleeding. Any trauma, even mild events, may cause life-threatening bleeding, and without treatmen

Studies of protein A and herpes simplex virus-1 induced Fc gamma-binding specificities. Different binding patterns for IgG3 from Caucasian and Oriental subjects

Herpes simplex virus type 1 (HSV-1) expresses a receptor that binds the Fc portion of IgG. This HSV-1 Fc gamma-binding protein is, like protein A of Staphylococcus aureus, known to bind human IgG1, IgG2 and IgG4 but not IgG3 subclasses. However, IgG3 with the allotype Gm(s+)(t+), prominent in the Oriental population, reacts with protein A. This prompted us to investigate the reactivity of Oriental

Isolation of human cationic antimicrobial protein-18 from seminal plasma and its association with prostasomes.

BACKGROUND: Cathelicidins are a group of antibiotic peptides with broad antimicrobial activity. They are considered to be an essential part of the innate immune system. The only known human cathelicidin is the human cationic antimicrobial protein (hCAP-18), from which the antimicrobial peptide LL-37 is released. METHODS AND RESULTS: In the present study, we purified hCAP-18 from seminal plasma and

Phase behavior of the monoolein-water system - The effects of addition of the hydrocarbon 1,8-cineole and supercritical carbon dioxide

The results of a study on the phase behavior of the monoolein/water system with the addition of the hydrocarbon 1,8-cineole (C10H18O) and supercritical carbon dioxide at 15 MPa is presented. To two mixtures consisting of 9 and 18 wt.% water in monoolein, 5, 10, and 20 wt.% cineole were added and heated to between 35 and 80 degreesC at atmospheric pressure and in the presence of supercritical carbo

Selective increase of the contractile response to endothelin-1 in subcutaneous arteries from patients with essential hypertension

Endothelin-1 has been shown to contribute to basal vascular tone in man. Since endothelin-1 is a potent vasoconstrictor putatively involved in hypertension, we have compared the contractile responses of endothelin-1 and noradrenaline in relation to potassium chloride in subcutaneous resistance arteries from subjects with established essential hypertension with matched controls. Furthermore, with R

Modular organisation and spinal somatosensory imprinting

The withdrawal reflex system has been extensively used as a model system for studies of pain related mechanisms, sensorimotor integration, learning and memory. For a long time, this system was assumed to be organised as a flexion reflex system. However, recent studies indicate that this system has a modular organisation, each module performing a detailed and functionally adapted sensorimotor trans

Polysulfone ionomers for proton-conducting fuel cell membranes - 2. Sulfophenylated polysulfones and polyphenylsulfones

Polysulfones and polyphenylsulfones having pendant phenyl groups with sulfonic acid units have been prepared by lithiation of the respective polymer, followed by reaction with 2-sulfobenzoic acid cyclic anhydride. The resulting ionomers were cast into membranes and properties such as thermal stability, ion-exchange capacity, water sorption and proton conductivity were evaluated. These membranes pr

Bronchoscopy and bronchoalveolar lavage findings in cross-country skiers with and without "ski asthma"

Bronchial hyperresponsiveness to methacholine with asthma-like symptoms ("ski asthma") is frequent in elite cross-country skiers. To further the understanding of "ski asthma", 10 nonasthmatic, nonatopic controls and 30 adolescent elite skiers were investigated by bronchoscopy and bronchoalveolar lavage (BAL). Nine skiers were atopic without allergy symptoms. Compared with controls, the macroscopic

Sustained citalopram treatment in experimental hepatic encephalopathy: Effects on entrainment to the light-dark cycle and melatonin

Patients with chronic hepatic encephalopathy often display altered diurnal rhythm as well as other affective disturbances which motivate treatment with antidepressants. We investigated the effects of sustained treatment with citalopram (10 mg/kg daily, 10 days) on 24-hr behavioural open-field activities in portacaval-shunted (PCS) rats and sham-operated control rats. In addition, the daytime and n

Maxima for Gaussian seas

The probability distribution of the height of global maximum for a Gaussian random field evolving in time is studied. In particular, the effect of spreading is studied and the role of the wave kinematics is discussed. It is observed that taking into account time dynamics of spatial characteristics results in distributions different from those obtained for the static case. The results are illustrat

An experimental evaluation of the arctic fox (Alopex lagopus) as a seed disperser

Arctic foxes are evaluated as seed dispersal vectors for Greenlandic plant species through a feeding experiment with subsequent scat analysis and germination test. Seeds of 22 common species with different morphology were tested. Passage time ranged between 4 and 48 hours. No significant differences was detected in passage time for seeds with different morphology. Cerastium alpinum and Stellaria l

On-line signature verification by explicit solution to the point correspondence problem

This paper presents a novel tool to be used for signature verification. The method is based on the vector context matrix concept that enables robust and precise extraction of the point correspondences between a sample and a database model. Using a digitizing tablet as a source for input, information of the pressure, speed and pen inclination is obtained in addition to the coordinates of the sample