Background Few families with autosomal dominant forms of chronic idiopathic pseudo-obstruction (CIP) have been identified and reported. Methods We compared two families by clinical, laboratory, histopathologic, and genealogical investigations. Ten patients (pts) (five women) from two families, A and B, both with a family history suggesting autosomal dominant CIP, were investigated. Key Results All

Incoming Photosynthetic Active Radiation (PAR) is an essential variable for modelling aboveground primary production of ecosystems through the light-use efficiency approach. A method is presented where daily classifications of cloud cover (CLAVR) from the NOAA AVHRR satellite sensor is used to estimate surface incident short wave (SW) flux from which PAR can be assessed. The study area is the Inne

There are at least 120 completely characterized human papillomavirus (HPV) types and putative new types are continuously found. Both squamous cell carcinoma of the skin (SCC) and other skin lesions commonly contain multiple cutaneous HPV types. The objective of this study was to achieve an improved resolution of the diversity of HPV types in lesions such as SCCs, actinic keratoses (AKs) and kerato

The importance of tyrosine residues in ligand-mediated endocytosis of the platelet-derived growth factor beta-receptor was analyzed using a series of tyrosine residue-mutated beta-receptors, which together cover all of the tyrosine residues in the juxtamembrane segment, the kinase insert, and the carboxyl-terminal tail; also certain of the tyrosine residues within the first and second parts of the

Multicentre patch test studies (MPTSs) can contribute useful information for diagnostic and preventive measures. The aim of the present paper is to propose how to perform high-quality MPTSs. To this end, factors of significance for the patch test result are discussed with regard to the standardization and calibration of high-quality MPTSs. The 16 factors discussed are scored 0, 1, 2, or 3, dependi

Surgery in infants and young children with haemophilia, when preceded by accurate diagnosis and accompanied by safe and effective factor prophylaxis, is not associated with a significant risk of haemorrhage. Haemophilic newborns undergoing circumcision or major surgery prior to diagnosis and in the absence of appropriate haemostatic prophylaxis remain as a concern. Inhibitor development has replac

The mucosal immune system identifies and fights invading pathogens, while allowing non-pathogenic organisms to persist. Mechanisms of pathogen/non-pathogen discrimination are poorly understood, as is the contribution of human genetic variation in disease susceptibility. We describe here a new, IRF3-dependent signaling pathway that is critical for distinguishing pathogens from normal flora at the m

Many methods for distance estimation, such as the ultrasonic pulse-echo method, involve the estimation of a time-of-flight (TOF). In this paper, a signal model is developed that, apart from the TOF, accounts for an unknown, linear frequency dependent distortion as well as for additive noise. We derive a TOF estimator for this model based on the criteria of maximum likelihood. The resulting receive

A method is described for identifying simultaneous signals from different transmit antennas in multiantenna sounders. Multiple-orthogonal signals are produced from one pseudonoise code by using it to modulate RF carriers at precalculated offset frequencies. Although their frequency spectra are interleaved and they occupy the same band, the spectral lines from different transmitters are separable a

AIMS/HYPOTHESIS: Recently, three groups independently reported that variation in MTNR1B, the gene encoding melatonin receptor 1B, was associated with an increased risk of type 2 diabetes, increased fasting plasma glucose and impaired insulin secretion in populations of European ancestry. In the present study, we investigated whether a single MTNR1B polymorphism was associated with type 2 diabetes

BACKGROUND & AIMS: Corticosteroids alone or in conjunction with azathioprine (AZA) is the standard treatment in autoimmune hepatitis (AiH). Individual variations in thiopurine (TP) metabolism may affect both drug efficacy and toxicity. Our aim was to investigate the utility of thiopurine methyltransferase (TPMT) as well as thioguanine nucleotide (TGN) and methylthioinosine monophosphate (meTIM

The role of the gaseous messengers NO and CO for β-cell function and survival is controversial. We examined this issue in the hyperglycemic-hyperinsulinemic ob/ob mouse, an animal model of type 2 obese diabetes, by studying islets from obese vs lean mice regarding glucose-stimulated insulin release in relation to islet NO and CO production and the influence of modulating peptide hormones. Glucose-

Islet hormone secretion is regulated by a variety of factors, and many of these signal through G protein-coupled receptors (GPCRs). A novel islet GPCR is GPR54, which couples to the Gq isoform of G proteins, which in turn signal through the phospholipase C pathway. Ligands for GPR54 are kisspeptins, which are peptides encoded in the KISS1 gene and also expressed in islet beta-cells. The KISS1 gene

BACKGROUND: Renal glomerular filtration rate (GFR) of pediatric cancer patients at diagnosis has previously been investigated in a limited number of studies. PROCEDURE: GFR, measured by iohexol clearance, was prospectively investigated in 55 children over the age of 1 year with malignancies, (group A). Elevated GFR (>175 ml/min/1.73 m(2)) at diagnosis was found. To investigate if this finding was

OBJECTIVE: To study muscle strength and functional performance in patients with anterior cruciate ligament (ACL) injury with or without surgical reconstruction 2 to 5 years after injury. Good muscle function is important in preventing early-onset osteoarthritis (OA), but the role of reconstructive surgery in restoring muscle function is unclear. METHODS: Of 121 patients with ACL injury included in

OBJECTIVE: To compare ambulatory blood pressure recorded during a work day with a leisure day regarding masked hypertension. DESIGN: A randomized crossover study in middle-aged normotensives. PARTICIPANTS: Thirty-seven untreated middle-aged normotensives (17 women), who had repeated office blood pressure less than 140/90 mmHg. MAIN OUTCOME MEASURE: Individual difference of masked hypertension betw