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Online registry of genetic and clinical immunodeficiency diagnostic laboratories, IDdiagnostics.

Primary immunodeficiencies (IDs) are caused by inherited genetic defects leading to intrinsic defects in cells of the immune systems. Most IDs are rare diseases and can be difficult to diagnose because similar symptoms characterize several disorders. Mutation detection is the most reliable method in such cases. These tests are not available at most centers and physicians can have difficulties in f

Water Vapor Transport and Moisture Budget over Eastern China: Remote Forcing from the Two Types of El Nino

The water vapor transport and moisture budget over eastern China remotely forced by the cold-tongue (CT) and warm-pool (WP) El Nino show striking differences throughout their lifetime. The water vapor transport response is weak in the developing summer but strong in the remaining phases of CT El Nino, whereas the opposite occurs duringWP El Nino. WP El Nino causes a moisture deficit over the Yangt

PDGF-A signaling is a critical event in lung alveolar myofibroblast development and alveogenesis.

A mouse platelet-derived growth factor A chain (PDGF-A) null allele is shown to be homozygous lethal, with two distinct restriction points, one prenatally before E10 and one postnatally. Postnatally surviving PDGF-A-deficient mice develop lung emphysema secondary to the failure of alveolar septation. This is apparently caused by the loss of alveolar myofibroblasts and associated elastin fiber depo

Significance of a notch in the otoacoustic emission stimulus spectrum.

Objective: To explain a clinical observation: a notch in the stimulus spectrum during transient evoked otoacoustic emission measurement in ears with secretory otitis media. Methods: The effects of tympanic under-pressure were investigated using a pressure chamber. A model of the ear canal was also studied. Results: Tympanic membrane reflectance increased as a consequence of increased stiffn

FVIIa as therapeutic agent in hemophilia and beyond.

Around 20percent of the patients with severe hemophilia develop inhibitory antibodies against the factor they lack. In these patients the administration of FVIII/FIX-concentrates are not hemostatically effective. Since FVIIa is not enzymatically active unless complexed with tissue factor (TF) exposed following an injury to the vessel wall, it was considered an attractive candidate for improved tre

The risk of contralateral breast cancer in daughters of women with and without breast cancer.

We aimed to estimate the 15-year and lifetime risks of contralateral breast cancer in breast cancer patients according to the age of diagnosis of the first cancer and the history of breast cancer in the mother. The risks of contralateral breast cancer were estimated for all 78,775 breast cancer patients in the Swedish Family-Cancer Database (age at diagnosis of first breast cancer

Association of central and peripheral pulse pressure with intermediate cardiovascular phenoytpes.

OBJECTIVE: We assessed the relationship between pulse pressure and intermediate cardiovascular phenotypes in a middle-aged cohort with high prevalence of hypertension. BACKGROUND: It has been suggested that central pulse pressure (cPP) is a better predictor of cardiovascular outcome than peripheral pulse pressure (pPP), particularly in the elderly. Yet, it is unclear if cPP provides additional pro

Distribution of the maximum in air pollution fields

Air quality standards are set to protect public health. The values of the standards are often based on health effect studies, without any statistical considerations. In order to judge if a standard is met measurements of ambient air quality are taken at monitoring stations, and these measured values are used to decide whether or not the standard has been violated. In this paper we examine the stat

The secretory leukocyte protease inhibitor (SLPI) and the secondary granule protein lactoferrin are synthesized in myelocytes, colocalize in subcellular fractions of neutrophils, and are coreleased by activated neutrophils.

The secretory leukocyte protease inhibitor (SLPI) re-establishes homeostasis at sites of infection by virtue of its ability to exert antimicrobial activity, to suppress LPS-induced cellular immune responses, and to reduce tissue damage through inhibition of serine proteases released by polymorphonuclear neutrophil granulocytes (PMNs). Microarray analysis of bone marrow (BM) populations highly enri

Long-term survival in uterine clear cell carcinoma and uterine papillary serous carcinoma.

Uterine clear cell carcinoma (UCC) and uterine papillary serous carcinoma (UPSC) are rare entities that differ in clinical behavior from endometrial adenocarcinoma. Compared with endometrioid adenocarcinoma, they more often metastasize early and more commonly in the upper abdomen including the omentum. Treatment programs of UCC and UPSC at different stages vary and range from no adjuvant therapy i

Rehabilitation Engineering and Design Research - Theory and Method

All scientific research requires problem definition and discipline, as well as concept and method formation. However, from a human point of view the most important problems are probably those found in areas where it may be difficult to provide such ideal research conditions. Rehabilitation engineering and design research is one such field. Nonetheless, we believe that these theory and method forma

The Controversy of Growth. A debate between economists and sociologists about the Swedish public sector

Has the 'Swedish model' forced Sweden into stagnating economic growth? And has this caused Sweden to lag behind other comparable OECD-countries from the 1970s and onwards, i.e. since Sweden chose a welfare path different from many other countries? This has been the subject of a more than twenty year long controversy between Walter Korpi, professor of sociology and social policy, and leading Swedis

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gesture, second language acquisition, reference tracking, French, Dutch, infomation structure, discourse

Grund, uppbyggnad och verktyg - diskurspsykologi som analysredskap i musikpedagogisk forskning

ABSTRACT Fundament, construction and tools – discursive psychology at work in music education This article addresses how discursive psychology can be used in the discipline of music education. Engaging examples from the field are chosen to provide an introduction to the analysing tools in discursive psychology. Empirical data consist, amongst others, of interviews with students and teachers in Swe