Vaccination in patients with primary immune deficiency, secondary immune deficiency and autoimmunity with immune regulatory abnormalities.

Vaccination has been an important healthcare measure in preventing infectious diseases. The response to vaccination is reduced in immunocompromised patients, primary immune deficiency (PID) and secondary immune deficiency (SID), but vaccination studies still demonstrated a protective effect resulting in reducing complications, hospitalization, treatment costs and even mortality. The primary physician and the specialist directing patient care are responsible for vaccination.

Modulation of Glycosaminoglycans Affects PrPSc Metabolism but Does Not Block PrPSc Uptake.

Unlabelled: Mammalian prions are unconventional infectious agents composed primarily of the misfolded aggregated host prion protein PrP, termed PrP(Sc). Prions propagate by the recruitment and conformational conversion of cellular prion protein into abnormal prion aggregates on the cell surface or along the endocytic pathway. Cellular glycosaminoglycans have been implicated as the first attachment sites for prions and cofactors for cellular prion replication.

Leaky RAG Deficiency in Adult Patients with Impaired Antibody Production against Bacterial Polysaccharide Antigens.

Loss of function mutations in the recombination activating genes RAG1 and RAG2 have been reported to cause a T-B-NK+ type of severe combined immunodeficiency. In addition identification of hypomorphic mutations in RAG1 and RAG2 has led to an expansion of the spectrum of disease to include Omenn syndrome, early onset autoimmunity, granuloma, chronic cytomegalovirus- or EBV-infection with expansion of gamma/delta T-cells, idiophatic CD4 lymphopenia and a phenotype resembling common variable immunodeficiency. Herein we describe a novel presentation of leaky RAG1 and RAG2 deficiency in two unrelated adult patients with impaired antibody production against bacterial polysaccharide antigens.

Prolonged Activated Clotting Time after Protamine Administration Does Not Indicate Residual Heparinization after Cardiopulmonary Bypass in Pediatric Open Heart Surgery.

Background: In open heart surgery, heparinization is commonly neutralized using an empirical heparin:protamine ratio ranging between 1:1 and 1:1.5. However, these ratios may result in protamine overdose that should be avoided for its negative side effects on the coagulation system.

Accurate Location and Manipulation of Nanoscaled Objects Buried under Spin-Coated Films.

Detection and precise localization of nanoscale structures buried beneath spin-coated films are highly valuable additions to nanofabrication technology. In principle, the topography of the final film contains information about the location of the buried features. However, it is generally believed that the relation is masked by flow effects, which lead to an upstream shift of the dry film's topography and render precise localization impossible.

Surface Plasmon Resonance Analysis Shows an IgG-Isotype-Specific Defect in ABO Blood Group Antibody Formation in Patients with Common Variable Immunodeficiency.

Background: Common variable immunodeficiency (CVID) is the most common clinically severe primary immunodeficiency and comprises a heterogeneous group of patients with recurrent severe bacterial infections due to the failure to produce IgG antibodies after exposure to infectious agents and immunization. Diagnostic recommendations for antibody failure include assessment of isoagglutinins. We have readdressed this four decades old but still accepted recommendation with up to date methodology.

Insights into mechanisms of capillary assembly.

Capillary assembly in a topographical template is a powerful and flexible method for fabricating complex and programmable particle assemblies. To date, very little attention has been paid to the effects that the trap geometry--in particular the trap depth--has on the outcome of the assembly process. In this paper, we provide insights into the mechanisms behind this directed assembly method by systematically studying the impact of the trap depth and the surface tension of the suspension.

Strigolactam: New potent strigolactone analogues for the germination of Orobanche cumana.

Very recently, strigolactones have been conclusively identified as phytohormones. The progresses achieved in this field are culminating in the identification of the molecular receptors involved in the signal transduction mechanism. The exact mechanism of the mode of action of strigolactones still remains to be fully elucidated and we were interested to gain some insight into the mechanism of action of strigolactones by selectively modifying the reactivity of the lactone C-ring.

Nest and food search behaviour in desert ants, Cataglyphis: a critical comparison.

North African desert ants, Cataglyphis, use path integration to calculate a home vector during their foraging trips, constantly informing them about their position relative to the nest. This home vector is also used to find the way back to a productive feeding site the ant has encountered and thus memorized. When the animal fails to arrive at its goal after having run off the home or food vector, a systematic search is initiated.

2 year neurodevelopmental and intermediate perinatal outcomes in infants with very preterm fetal growth restriction (TRUFFLE): a randomised trial.

Background: No consensus exists for the best way to monitor and when to trigger delivery in mothers of babies with fetal growth restriction. We aimed to assess whether changes in the fetal ductus venosus Doppler waveform (DV) could be used as indications for delivery instead of cardiotocography short-term variation (STV).

Methods: In this prospective, European multicentre, unblinded, randomised study, we included women with singleton fetuses at 26-32 weeks of gestation who had very preterm fetal growth restriction (ie, low abdominal circumference [<10th percentile] and a high umbilical artery Doppler pulsatility index [>95th percentile]).

Detection of impaired IgG antibody formation facilitates the decision on early immunoglobulin replacement in hypogammaglobulinemic patients.

Hypogammaglobulinemia (serum IgG lower than 2 SD below the age-matched mean) and clinical symptoms such as increased susceptibility to infection, autoimmune manifestations, granulomatous disease, and unexplained polyclonal lymphoproliferation are considered to be diagnostic hallmarks in patients with common variable immunodeficiency (CVID), the most frequent clinically severe primary immunodeficiency syndrome. In the present study, we investigated patients with hypogammaglobulinemia and no clinical or immunological signs of defective cell-mediated immunity and differentiated two groups on the basis of their IgG antibody formation capacity against a variety of different antigens (bacterial toxins, polysaccharide antigens, viral antigens). Patients with hypogammaglobulinemia and intact antibody production (HIAP) displayed no or only mild susceptibility to infections, while CVID patients showed marked susceptibility to bacterial infections that normalized following initiation of IVIG or subcutaneous immunoglobulin replacement therapy.

Direct spectroscopic evidence of the mechanism behind the phase transition of [2,2]-paracyclophane.

[2,2]-Paracyclophane undergoes phase transitions at 45 and 60 K. Based on simultaneous Raman spectroscopy and inelastic neutron scattering experiments (12-70 K), it was shown that a twisting motion of the ethylene bridge perpendicular to the plane of the aromatic rings drives the phase transition. The low-temperature (<45 K) and high-temperature (>60 K) conformers only differ by this twisting motion, which freezes out below 45 K and is thermally averaged above 60 K.

Food searches and guiding structures in North African desert ants, Cataglyphis.

North African desert ants, Cataglyphis fortis, use path integration as their primary means of navigation. The ants also use landmarks when these are available to improve navigation accuracy. Extended landmarks, such as walls and channels, may serve further functions, for example, local guidance or triggering of local vectors.

Characterization of monoclonal gammopathy in patients with amyotrophic lateral sclerosis.

Background: Recently, new guidelines for the monitoring and the risk evaluation of monoclonal gammopathy with undetermined significance (MGUS) became available and the light chain MGUS subtype was defined.

Aims Of The Study: To characterize the type, risk and diagnostic implications of MGUS in patients with amyotrophic lateral sclerosis.

Methods: We screened 97 consecutive patients with ALS and 97 age- and gender-matched controls for MGUS by serum electrophoresis and immunofixation and compared the characteristics of MGUS with population-based data.

Charge density investigations on [2,2]-paracyclophane – in data we trust.

Four datasets on [2,2]-paracyclophane were collected in-house and at the Advanced Photon Source at two different temperatures for charge density investigation. Global data quality indicators such as high resolution, high I/σ(I) values, low merging R values and high multiplicity were matched for all four datasets. The structural parameters did not show significant differences, but the synchrotron data depicted deficiencies in the topological analysis.

Peripheral facial palsy as an initial symptom of Lyme neuroborreliosis in an Austrian endemic area.

Background: The objective of this study was to analyze the percentage as well as clinical and laboratory characteristics of Lyme neuroborreliosis (LNB) in patients admitted with peripheral facial palsy. Additionally, we looked for diagnostic criteria to distinguish Bell's palsy from facial palsy due to LNB.

Methods: Data collection was done retrospectively from 2007 until 2012.

[Physicians in the northern part of Schleswig at the time of reunification with Denmark].

Marie Hollensen (1884-1942) and Hans Storm (1886-1945) were Danish-minded inhabitants of Schleswig, a part of Denmark conquered in 1864 by Prussia. Both wanted to become general medical practitioners in their homeland. As a Danish medical degree would not allow them to practise in Germany they were forced to take their medical degrees in Germany.

Tolerability of the SQ-standardised grass sublingual immunotherapy tablet in patients treated with concomitant allergy immunotherapy: a non-interventional observational study.

Background: The majority of allergic patients are poly-sensitized. For causal treatment by allergy immunotherapy (AIT) a single or few allergen products containing the clinically most relevant allergens are applied, but few data on tolerability of multiple application of AIT is available. The aim of our study was to investigate safety and tolerability in patients who started treatment by sublingual immunotherapy (SLIT) with the standardised SQ(®) grass SLIT-tablet and were treated with concomitant AIT products.

Colon cancer screening for Colorado's underserved: a community clinic/academic partnership.

Background: Colorectal cancer (CRC) is largely preventable by finding and removing adenomas, but many people have not been screened, especially the uninsured with low income.

Purpose: To establish a statewide infrastructure to ensure that low-income Coloradans receive colonoscopy for CRC screening and diagnostic evaluation.

Design: In 2006, a statewide program to provide free colonoscopy to uninsured Coloradans was developed as a partnership between the University of Colorado Cancer Center and Colorado safety-net clinics.

[Quality Assurance in Sociomedical Evaluation by Peer Review: A Pilot Project of the German Statutory Pension Insurance].

Background: The sociomedical evaluation by the German Pension Insurance serves the purpose of determining entitlement to disability pensions. A quality assurance concept for the sociomedical evaluation was developed, which is based on a peer Review process. Peer review is an established process of external quality assurance in health care.

"It is all about the fear of being discriminated [against]…the person suffering from HIV will not be accepted": a qualitative study exploring the reasons for loss to follow-up among HIV-positive youth in Kisumu, Kenya.

Background: Youth represent 40% of all new HIV infections in the world, 80% of which live in sub-Saharan Africa. Youth living with HIV (YLWH) are more likely to become lost to follow-up (LTFU) from care compared to all other age groups. This study explored the reasons for LTFU among YLWH in Kenya.

The EADC-ADNI Harmonized Protocol for manual hippocampal segmentation on magnetic resonance: evidence of validity.

Background: An international Delphi panel has defined a harmonized protocol (HarP) for the manual segmentation of the hippocampus on MR. The aim of this study is to study the concurrent validity of the HarP toward local protocols, and its major sources of variance.

Methods: Fourteen tracers segmented 10 Alzheimer's Disease Neuroimaging Initiative (ADNI) cases scanned at 1.