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Papillon-Lefevre syndrome (PLS) results from mutations that inactivate cysteine protease cathepsin C (CTSC), which processes a variety of serine proteases considered essential for antimicrobial defense. Despite serine protease-deficient immune cell populations, PLS patients do not exhibit marked immunodeficiency. Here, we characterized a 24-year-old woman who had suffered from severe juvenile peri

Climate change poses a serious threat to sustainable urban development, placing many cities at risk. As a consequence, city authorities are increasingly facing the challenge of finding ways to include adaptation strategies into their work, although related knowledge and competence is still scarce and fragmented. With the aim to contribute to knowledge development and organizational learning, the o

Popular Abstract in English During recent decades, significant changes in fertility pattern are seen in the Western World. Declining birth rates and smaller family sizes affect the population size, but also cause unwanted effects for the desired number of children to be achieved by the individual couple. Also involuntary childlessness is a consequence of postponing childbearing to an age where theDeclining birthrates and infertility are now a common problem in the Western World. Due to the tendency to postpone childbearing an increasing age of women who consider motherhood is generally seen. A factor closely related to female fertility is the ovarian reserve, a term used to designate both quantitative as well as qualitative aspects of the remaining gametes in the female gonads. Despite a n

Alpha1-Antitrypsin (AAT), an acute-phase glycoprotein produced predominantly in the liver by hepatocytes, but also by macrophages, pulmonary alveolar cells and intestinal epithelial cells, is a member of the SERPIN superfamily (SERine Protease INhibitors), and is one of the major inhibitors of neutrophil elastase in human plasma. Although AAT plays a critical role in regulating inflammation by inh

Popular Abstract in Swedish Sköldkörtelhormoner och bröstcancer Insjuknandet i sköldkörtelsjukdomar är liksom bröstcancer vanligast hos kvinnor efter klimakteriet och ett eventuellt samband har diskuterats i över ett sekel. Experimentella studier har visat att sköldkörtelhormoner påverkar både utveckling av normala bröstceller och tillväxt av bröstcancerceller. Det finns också belägg för att sköThyroid hormones and breast cancer-prospective studies on incidence, mortality and prognostic factors The aim of this thesis was to investigate in a prospective design, thyroid hormones in relation to breast cancer risk, mortality and prognostic factors. The association between total triiodothyronine (T3) levels and breast cancer risk was studied in 2185 women with146 incident breast cancer case

Phosphor thermometry is a semi-invasive measurement technique which is commonly used for temperature determination in combustion applications. Surface temperature measurements using thermographic phosphors rely on the assumption that the phosphor layer is thin enough in order to adopt the surface temperature non-intrusively. This work compares the temperature information, recorded from two opposin

The purpose of this study was to investigate the effect of moisture on the heat transfer through clothing in relation to the water vapour resistance, type of underwear, location of the moisture and climate. This forms part of the work performed for work package 2 of the European Union THERMPROTECT project. Thermal manikin results of dry and wet heat loss are presented from different laboratories f

Background: Asthma guidelines emphasize both maintaining current control and reducing future risk, but the relationship between these 2 targets is not well understood. Objective: This retrospective analysis of 5 budesonide/formoterol maintenance and reliever therapy (Symbicort SMART Turbuhaler*) studies assessed the relationship between asthma control questionnaire (ACQ-5) and Global Initiative fo

In this paper, the flint depositions at Tuve 46 are analysed. Here around 30 depositions containing several thousands of waste flakes were recovered together with flint tools such as large bifaces, thick-butted axes, spoon-shaped scrapers, bifacial points. It is suggested that specialized flint knappers from flint-rich areas in southern Scandinavia came to the site during the early Late Neolithic

We investigate the second-order von Neumann approach from a diagrammatic point of view and demonstrate its equivalence with the resonant tunneling approximation. The investigation of higher order diagrams shows that the method correctly reproduces the equation of motion for the single-particle reduced density matrix of an arbitrary non-interacting many-body system. This explains why the method rep

The intention of the present study was to evaluate possible cardioprotective properties of inhalation anesthesia with sevoflurane.

OBJECTIVE - Low-grade chronic inflammation has been hypothesized to underlie the constellation of cardiometabolic risk factors, possibly by inducing insulin resistance. In the present study, we investigated associations between inflammation markers, insulin sensitivity (expressed as the ratio of the M value to the mean plasma insulin concentrations measured during the Final 40 min of the clamp [M/

Chronic obstructive pulmonary disease is a major cause of increased morbidity and mortality. The aim of this study was to investigate hospital admission rates among individuals with symptoms of chronic bronchitis and among those with airflow limitation corresponding to GOLD stages 1-4. Method: Between 1974 and 1992, 22 044 middle-aged individuals participated in a health screening, which included

Background and purpose The role of synovitis and high fluid pressure in the loosening process after total hip arthroplasty has gained increasing attention. We investigated the correlation between head size, polyethylene wear, and capsular distention. Patients and methods We analyzed 39 unrevised, radiographically stable hips that had been operated with 28 or 32 mm femoral heads 10 years earlier be

Detailed echolocation behavior studies on free-swimming dolphins require a measurement system that incorporates multiple hydrophones (often >16). However, the high data flow rate of previous systems has limited their usefulness since only minute long recordings have been manageable. To address this problem, this report describes a 47-channel burst-mode recording hydrophone system that enables high

Transport through nanosystems is treated within the second-order von Neumann approach. This approach bridges the gap between rate equations which neglect level broadening and cotunneling, and the transmission formalism, which is essentially based on the single-particle picture thereby treating many-particle interactions oil an approximate level. Here we provide an alternative presentation of the m

The observed almost complete dominance of prolate over oblate deformations in the ground states of deformed even-even nuclei is related to the splitting of high center dot "surface" orbits in the Nilsson diagram: on the oblate side the occurrence of numerous strongly avoided crossings which reduce the fanning out of the low Lambda orbits, while on the prolate side the same interactions increase th

Objectives The aims of the study were to temporally and externally validate the diagnostic performance of two logistic regression models containing clinical and ultrasound variables in order to estimate the risk of malignancy in adnexal masses, and to compare the results with the subjective interpretation of ultrasound findings carried out by an experienced ultrasound examiner ('subjective assessm