Angular Correlations in the ß Decay of

We present the first measurement of the α-ß-ν angular correlation in the Gamow-Teller ß^{+} decay of ^{8}B. This was accomplished using the Beta-decay Paul Trap, expanding on our previous work on the ß^{-} decay of ^{8}Li. The ^{8}B result is consistent with the V-A electroweak interaction of the standard model and, on its own, provides a limit on the exotic right-handed tensor current relative to the axial-vector current of |C_{T}/C_{A}|^{2}<0.013 at the 95.5% confidence level. This represents the first high-precision angular correlation measurements in mirror decays and was made possible through the use of an ion trap. By combining this ^{8}B result with our previous ^{8}Li results, we demonstrate a new pathway for increased precision in searches for exotic currents.

Natural history of growth and anaemia in children with epidermolysis bullosa: a retrospective cohort study.

BACKGROUND: Impaired growth and anaemia are major extracutaneous complications of epidermolysis bullosa (EB), but data on their development are lacking. OBJECTIVES: To determine the clinical course of growth and anaemia in children with EB and clarify the impact of nutritional compromise, inflammation and genetic factors. METHODS: A retrospective study was conducted of 200 children, 157 with recessive dystrophic EB (RDEB) and 43 with junctional EB (JEB)-generalized intermediate, followed at the main referral centre in Germany. Growth charts were calculated using the modified LMS method and were correlated with parameters of anaemia, nutrition, inflammation and the molecular defect in a linear model. RESULTS: In our cohort of patients with RDEB, weight impairment started at 12-18 months old; by the age of 10 years, 50% showed wasting. The predicted median weight at age 20 years was 35·2 kg for men and 40·1 kg for women. In JEB, growth resembled that of healthy children. Anaemia was present from the second year of life onwards in RDEB and JEB. Low levels of haemoglobin, iron, vitamin D, zinc and albumin, high levels of C-reactive protein, and absence of collagen VII correlated significantly with low weight in RDEB. No correlation was observed in JEB. CONCLUSIONS: The results highlight that nutritional compromise occurs early in children with RDEB and therefore may require interventions as of the first year or two of life. What's already known about this topic? Children with epidermolysis bullosa (EB) suffer from failure to thrive and anaemia as major extracutaneous complications. The course of growth and the development of anaemia in EB are poorly characterized. What does this study add? A molecularly well characterized cohort of 200 children with EB was followed with regard to anthropometrics, anaemia and inflammation. We demonstrate early onset of growth failure and anaemia, most pronounced in the subset of recessive dystrophic EB. Awareness of early growth delay and nutritional deficiencies will improve EB care in daily practice.

[Syndromes with skin fragility]. / Syndrome mit Hautfragilität.

Syndromic disorders with skin fragility belong to different groups of genodermatoses: epidermolysis bullosa (EB), Ehlers-Danlos syndrome and porphyria. The genetic defects mainly concern structural proteins which assure the mechanical stability of the skin and other tissues. Depending on the expression pattern of the affected protein in the skin, cutaneous fragility may manifest as superficial erosions, blisters, wounds, wound healing defects or scars. Extracutaneous manifestations are manifold and involve the heart, skeletal muscles, intestine, kidneys, blood vessels or the skeleton. Syndromic types of EB include in addition to skin blistering: (i) cardiomyopathy in case of desmoplakin, plakoglobin, or kelch-like protein mutations; (ii) muscular dystrophy in case of plektin mutations; (iii) pyloric atresia in case of integrin α6ß4 or plectin mutations; (iv) nephrotic syndrome in case of CD151 or integrin α3 mutations. Lysyl hydroxylase 3 mutations affect posttranslational modifications of collagens and lead to a dystrophic epidermolysis bullosa-like multisystemic disorder. Ehlers-Danlos syndromes are due to defects of dermal collagens or their processing and affect the skin, joints and blood vessels. Finally porphyrias are complex metabolic disorders with photosensitivity and sometimes skin fragility, liver or neurologic problems. Their pathogenesis relies on the accumulation of precursors in the tissues. Although these syndromes are rare in clinical practice, knowledge of the syndromic constellation contributes to early diagnosis and detection of complications.

[Clinical pharmacokinetics of anti-infective drugs in extracorporeal membrane oxygenation]. / Klinische Pharmakokinetik der Antiinfektiva bei extrakorporaler Membranoxygenierung.

Extracorporeal membrane oxygenation (ECMO) is becoming more and more clinically important. The extracorporeal circuit for membrane oxygenation consists of a pump, a membrane oxygenator and large volume tubing. The ECMO device forms an additional compartment, which can absorb drugs with high lipophilia and protein binding. Thus, ECMO affects the volume of distribution and the clearance. As a consequence, the pharmacokinetic-pharmacodynamic (pk-pd) target parameters cannot be achieved. The selection of an appropriate substance and the mode of application, combined with therapeutic drug monitoring (TDM), can significantly improve the therapeutic outcome of critically ill patients.

Development of an Age Band on the ManuVis for 3-Year-Old Children with Visual Impairments.

AIM: To compare fine motor performance of 3-year-old children with visual impairment with peers having normal vision, to provide reference scores for 3-year-old children with visual impairment on the ManuVis, and to assess inter-rater reliability. METHOD: 26 children with visual impairment (mean age: 3 years 7 months (SD 3 months); 17 boys) and 28 children with normal vision (mean age: 3 years 7 months (SD 4 months); 14 boys) participated in the study. The ManuVis age band for 3-year-old children comprised two one-handed tasks, two two-handed tasks, and a pre-writing task. RESULTS: Children with visual impairment needed more time on all tasks (p < .01) and performed the pre-writing task less accurately than children with normal vision (p < .001). Children aged 42-47 months performed significantly faster on two tasks and had better total scores than children aged 36-41 months (p < .05). Inter-rater reliability was excellent (Intra-class Correlation Coefficient = 0.96-0.99). CONCLUSIONS: The ManuVis age band for 3-year-old children is appropriate to assess fine motor skills, and is sensitive to differences between children with visual impairment and normal vision and between half-year age groups. Reference scores are provided for 3-year-old children with visual impairment to identify delayed fine motor development.

Usefulness of High-Quality Core Genome Single-Nucleotide Variant Analysis for Subtyping the Highly Clonal and the Most Prevalent Salmonella enterica Serovar Heidelberg Clone in the Context of Outbreak Investigations.

Salmonella enterica serovar Heidelberg is the second most frequently occurring serovar in Quebec and the third-most prevalent in Canada. Given that conventional pulsed-field gel electrophoresis (PFGE) subtyping for common Salmonella serovars, such as S. Heidelberg, yields identical subtypes for the majority of isolates recovered, public health laboratories are desperate for new subtyping tools to resolve highly clonal S. Heidelberg strains involved in outbreak events. As PFGE was unable to discriminate isolates from three epidemiologically distinct outbreaks in Quebec, this study was conducted to evaluate whole-genome sequencing (WGS) and phylogenetic analysis as an alternative to conventional subtyping tools. Genomes of 46 isolates from 3 Quebec outbreaks (2012, 2013, and 2014) supported by strong epidemiological evidence were sequenced and analyzed using a high-quality core genome single-nucleotide variant (hqSNV) bioinformatics approach (SNV phylogenomics [SNVphyl] pipeline). Outbreaks were indistinguishable by conventional PFGE subtyping, exhibiting the same PFGE pattern (SHEXAI.0001/SHEBNI.0001). Phylogenetic analysis based on hqSNVs extracted from WGS separated the outbreak isolates into three distinct groups, 100% concordant with the epidemiological data. The minimum and maximum number of hqSNVs between isolates from the same outbreak was 0 and 4, respectively, while >59 hqSNVs were measured between 2 previously indistinguishable outbreaks having the same PFGE and phage type, thus corroborating their distinction as separate unrelated outbreaks. This study demonstrates that despite the previously reported high clonality of this serovar, the WGS-based hqSNV approach is a superior typing method, capable of resolving events that were previously indistinguishable using classic subtyping tools.

How to assess diabetes distress: comparison of the Problem Areas in Diabetes Scale (PAID) and the Diabetes Distress Scale (DDS).

AIMS: To compare the properties of the two most commonly used assessment tools for diabetes distress, the Problem Areas in Diabetes Scale (PAID) and the Diabetes Distress Scale (DDS), in order to discriminate their psychometric capabilities and functions. METHODS: Six hundred and twenty-eight people with diabetes (67% Type 1, 33% Type 2) were cross-sectionally assessed with the PAID, the DDS and further self-report scales regarding coping, quality of life, depressive symptoms and self-care, and medical data were gained. We analysed the PAID and DDS for areas of contentual/psychometric divergence in assessing diabetes distress and compared their associations with criteria of interest. RESULTS: Content analysis: The PAID covers a greater variety of emotional concerns and shows a stronger focus on food-related problems and complications. The DDS is more reflective of physician-related distress and problems concerning diabetes self-management. Psychometric analysis: Exploratory factor analyses revealed four-factor structures of both scales, explaining 60% (PAID) and 67% (DDS) of variance. Confirmatory factor analyses confirmed that single-factor and four-factor models fit the data. Total scales proved high and subscales mostly satisfactory reliability. Associations with criteria of interest: The PAID was significantly more strongly associated with dysfunctional coping styles, quality of life and depressive symptoms. The DDS showed significantly stronger associations with diabetes self-care and metabolic outcomes. CONCLUSION: Our results support both PAID and DDS as good self-report measures of diabetes distress. The observed contentual/psychometric differences suggest that a justified choice with regard to the intended clinical or scientific purpose can improve the acquisition of the required data.

Assessment of diabetes acceptance can help identify patients with ineffective diabetes self-care and poor diabetes control.

AIMS: To estimate the associations between insufficient diabetes acceptance and relevant diabetes outcomes. METHODS: A total of 320 patients completed questionnaires on diabetes non-acceptance (the Acceptance and Action Diabetes Questionnaire), diabetes distress (the Problem Areas in Diabetes Scale), depressive mood (the Center for Epidemiologic Studies Depression Scale), coping with illness (the Freiburg Questionnaire of Coping with Illness), self-care activities (the Summary of Diabetes Self-Care Activities Measure) and quality of life (the Short Form-36 Health Questionnaire). A six-item version of the Acceptance and Action Diabetes Questionnaire showing good reliability and validity was established, and the associations between insufficient acceptance and clinical outcomes were estimated. RESULTS: Higher diabetes non-acceptance correlated significantly with less active coping (-0.37), reduced self-care (-0.43) and higher HbA1c levels (0.31), higher diabetes distress (0.53) and more depressive symptoms (0.36). Correlations of diabetes non-acceptance with diabetes self-care/glycaemic control were significantly higher than were those of depressive mood or diabetes distress with these criteria. CONCLUSIONS: Low diabetes acceptance is associated with impaired self-care and glycaemic control. Assessment of diabetes acceptance may facilitate the detection of patients at high risk and may present an essential target for treatments to improve diabetes control that is more relevant than elevated depressive mood or diabetes distress.

Development and application of MLVA methods as a tool for inter-laboratory surveillance.

Multiple-locus variable-number of tandem-repeats analysis (MLVA) has emerged as a valuable method for subtyping bacterial pathogens and has been adopted in many countries as a critical component of their laboratory-based surveillance. Lack of harmonisation and standardisation of the method, however, has made comparison of results generated in different laboratories difficult, if not impossible, and has therefore hampered its use in international surveillance. This paper proposes an international consensus on the development, validation, nomenclature and quality control for MLVA used for molecular surveillance and outbreak detection based on a review of the current state of knowledge.

Scaling up conservation agriculture: An exploration of challenges and opportunities through a stakeholder engagement process.

Increasing the resilience of agricultural landscapes requires fundamental changes to the dominant commodity production model, including incorporating practices such as reduced tillage, cover cropping, and extended rotations that reduce soil disturbance while increasing biological diversity. Increasing farmer adoption of these conservation systems offers the potential to transform agriculture to a more vibrant, resilient system that protects soil, air, and water quality. Adoption of these resilience practices is not without significant challenges. This paper presents findings from a participatory effort to better understand these challenges and to develop solutions to help producers overcome them. Through repeated, facilitated discussions with farmers and agricultural and conservation professionals across the U.S. state of Michigan, we confronted the policy, economic, and structural barriers that are inhibiting broader adoption of conservation systems, as well as identified policies, programs, and markets that can support their adoption. What emerged was a complex picture and dynamic set of challenges at multiple spatial scales and across multiple domains. The primary themes emerging from these discussions were barriers and opportunities, including markets, social networks, human capital, and conservation programs. Exacerbating the technical, agronomic, and economic challenges farmers face at the farm level, there are a host of community constraints, market access and availability problems, climatic and environmental changes, and policies (governmental and corporate) that cross-pressure farmers when it comes to making conservation decisions. Understanding these constraints is critical to developing programs, policies, and state and national investments that can drive adoption of conservation agriculture.

[Long-term outcome after penetrating keratoplasty for bullous keratopathy - influence of preoperative visual acuity on final outcome]. / Langzeitergebnisse nach perforierender Keratoplastik bei bullöser Keratopathie - Auswirkungen von präoperativem Visus auf die Ergebnisse.

PURPOSE: The aim of this study was to analyse the long-term functional results of penetrating keratoplasty for pseudophacic bullous keratopathy and Fuchs endothelial dystrophy and to analyse the potential influence of the preoperative visual acuity on the final outcome. PATIENTS AND METHODS: This retrospective study describes the postoperative results of 413 patients. 154 of them with bullous keratopathy and 259 patients with Fuchs dystrophy. All received penetrating excimer laser keratoplasty (PK).The results include the examination of the patients 1 / 3 / 5 / 8 / > 9 years postoperatively. For analysis the patients were divided in two groups: group I includes patients with a visual acuity ≤ 0.1 and group II with a visual acuity > 0.1. The trephination was performed using a 193 nm excimer laser. The transplanted cornea was fixed by using a double running cross-stitch suture. The outcome measures included best corrected visual acuity (CCV), keratometric equivalent (KEQ), keratometric astigmatism (AST), spherical equivalent (SEQ), refractive cylinder (CYL), best corrected visual acuity (BCVA), surface regularity index (SRI), surface asymmetry index (SAI), potential visual acuity (PVA), pachymetry (PAC), endothelial cell count (ECC), intraocular tension (TEN), topographic equivalent (T-EQ) and topographic astigmatism (T-AST). RESULTS: 5 / 8 years postoperatively CCV was 0.55 / 0.58; KEQ was 43.75 / 45.64 D; SEQ - 0.88 /- 1.88 D; AST was 3.91 / 4.41 D; CYL was 3.09 / 3.40 D; ECC was 1230 / 1080 cells/mm (2); TEN was 13.61 / 14.36 mmHg; PAC was 593.21 / 579.50 µm; SRI was 1.08 / 0.89; SAI was 1.31 / 1.10; PVA was 0.77 / 0.83; T-EQ was 44.78 / 44.92 D and T-AST was 4.85 / 3.92 D. The outcome of patients with a preoperative visual acuity of ≤ 0.1 was significantly worse than the group with the visual acuity of > 0.1. CONCLUSION: This study shows that the long-term results after penetrating keratoplasty for pseudophacic bullous keratopathy and Fuchs endothelial dystrophy are good. Patients with preoperative visual acuity of ≤ 0.1 had worse final results.

Legionella pneumophila monoclonal antibody subgroups and DNA sequence types isolated in Canada between 1981 and 2009: Laboratory Component of National Surveillance.

Legionella pneumophila (Lp) is a significant cause of nosocomial, community-acquired, and travel-associated pneumonia in industrialized regions. Legionellosis has been a nationally notifiable disease in Canada since 1986, with an average of 75 cases reported annually; however, only the most severe, and often fatal, cases are reported or investigated. Here, epidemiological relationships, types, and distribution of Lp referrals to the Canadian national reference center were studied. Lp strains from different years, sources, and geographic locations were subtyped using a sequence-based typing (SBT) scheme and by the 'Joly' and/or 'Dresden' monoclonal antibody panels. Included were 128 epidemiologically unrelated clinical and 86 unrelated environmental strains. Sixty-four (index of diversity [IOD] = 0.964) and 45 (IOD = 0.888) sequence types (STs) were observed among clinical and environmental sources, respectively. Serogroup (sg) 1 was represented by 60.2% (77/128) and 52.3% (45/86) of clinical and environmental strains, respectively, and 63.6% (49/77) and 15.6% (7/45) of those were mAb2-positive, respectively. Serogroup 1, ST1 accounted for 14.1% (18/128) and 30.2% (26/86) of unrelated clinical and environmental isolates, respectively. This database will serve as a basis for Canadian epidemiological surveillance efforts and is linked to global surveillance initiatives curated by the European Working Group for Legionella Infections (EWGLI) network.

Photosynthesis-induced biofilm calcification and calcium concentrations in Phanerozoic oceans.

Photosynthetic carbon assimilation is commonly invoked as the cause of calcium carbonate precipitation in cyanobacterial biofilms that results in the formation of calcareous stromatolites. However, biofilm calcification patterns in recent lakes and simulation of photosynthetically induced rise in calcium carbonate supersaturation demonstrate that this mechanism applies only in settings low in dissolved inorganic carbon and high in calcium. Taking into account paleo-partial pressure curves for carbon dioxide, we show that Phanerozoic oceans sustaining calcified cyanobacteria must have had considerably higher calcium concentrations than oceans of today. In turn, the enigmatic lack of calcified cyanobacteria in stromatolite-bearing Precambrian sequences can now be explained as a result of high dissolved inorganic carbon concentrations.

[Surgical treatment of esophageal cancer].

Prospective study, included 117 patients with esophageal cancer, all received resection of the esophagus through laparatomy and and right-side thoracotomy without neoadjuvant chemotherapy. 70,0% of patients demonstrated stage higher then IIb UICC. R0 resection was possible in 101 patients (86,3%). Hospital lethality was 5,1%. Overall lethality among the operated patients was 21,4%. Long-term follow-up results were obtained in 96,6%. Surgical treatment alone does not provide satisfactory results for the patients with cancer of esophagus. Further therapy individualization and combination of surgery with modern neoadjuvant chemotherapy can provide better prognosis for these patients.

Prenatal diagnosis of cerebral lesions in Tuberous sclerosis complex (TSC). Case report and review of the literature.

Tuberous sclerosis complex (TSC) is an autosomal-dominant neurocutaneous disorder with multi-organ involvement. The diagnosis is suspected at fetal ultrasound on the discovery of multiple cardiac rhabdomyomas (CRs). They typically develop in utero and undergo spontaneous regression during the first years of live. With developing neuroradiological methods more light is shed on antenatal cerebral lesions like cortical tubers or giant cell astrocytomas. Unfortunately these do not regress, but instead are in principle progressive in size and number, correlated with epilepsy, mental retardation and behavioral problems. It is unknown whether fetal cerebral lesions, are always correlated with a poor neurological outcome or a progressive course of disease. This makes prenatal counseling extremely difficult. We report one case of de novo TSC with first detection of cortical tubers on fetal ultrasound, later developing multiple CRs. The pregnancy was continued and the child is developing well during 16 months of follow-up. Minor motor seizures from the 10th month onwards are successfully treated with Valproate. The published cases with antenatal diagnosis of TSC are revised, trying to get more insight into the postnatal course of prenatally diagnosed TSC. This is crucial, either when termination of pregnancy (TOP) is considered, but even more for proper postnatal care and follow-up.

[QT interval prolongation and psychotropic drugs in children and adolescents: proposed guideline]. / QT-tijdverlenging en psychofarmaca bij kinderen en jeugdigen: voorstel voor een richtlijn.

--Several psychotropic drugs that are prescribed to children and adolescents can prolong the QT interval on ECG. QT prolongation can cause a potentially fatal type ofventricular tachycardia known as 'torsade de pointes'. --In 1999, the American Heart Association published a guideline on cardiovascular monitoring of children and adolescents receiving psychotropic drugs. --A similar guideline does not exist in the Netherlands. A survey of Dutch child and adolescent psychiatrists revealed a need for guidelines on QT monitoring in children and adolescents taking psychotropic drugs associated with QT prolongation. --A multidisciplinary panel of professionals drafted a proposed guideline for QT monitoring in patients treated with QT-prolonging psychotropic drugs. --The proposed guideline is summarised in two flowcharts, one for the first consultation and one for follow-up visits.

Canadian Public Health Laboratory Network position statement: Non-culture based diagnostics for gastroenteritis and implications for public health investigations.

As clinical laboratories transition to using culture-independent detection test (CIDT) panels for cases of acute gastroenteritis, culture of clinical specimens is becoming less common. The reduction in bacterial cultures available for public health activities is expected to hinder surveillance and outbreak response by public health laboratories at the local, provincial, national and international levels. These recommendations are intended to serve as guidelines for the implementation of CIDT panels in frontline laboratories in Canada. The United States of America has already seen a significant reduction in culture of stool specimens despite the Association of Public Health Laboratories recommendation to perform reflex culture on positive CIDT specimens. Priority public health organisms addressed in these Canadian guidelines include Shiga toxin-producing Escherichia coli, Shigella and Salmonella and, under regional circumstances, other organisms such as Campylobacter jejuni/coli and Yersinia enterocolitica. These recommendations suggest active engagement between primary diagnostic laboratories and provincial public health laboratories to determine the workflow and protocols for reflex or parallel culture. Consequently, notifiable disease definitions will also need modification, with consultation of all stakeholders. Stakeholders need to work together to enhance recovery of bacterial isolates with best practices used for stool transport and storage.

Use of Whole Genome Sequencing and Patient Interviews To Link a Case of Sporadic Listeriosis to Consumption of Prepackaged Lettuce.

We report on a case of listeriosis in a patient who probably consumed a prepackaged romaine lettuce-containing product recalled for Listeria monocytogenes contamination. Although definitive epidemiological information demonstrating exposure to the specific recalled product was lacking, the patient reported consumption of a prepackaged romaine lettuce-containing product of either the recalled brand or a different brand. A multinational investigation found that patient and food isolates from the recalled product were indistinguishable by pulsed-field gel electrophoresis and were highly related by whole genome sequencing, differing by four alleles by whole genome multilocus sequence typing and by five high-quality single nucleotide polymorphisms, suggesting a common source. To our knowledge, this is the first time prepackaged lettuce has been identified as a likely source for listeriosis. This investigation highlights the power of whole genome sequencing, as well as the continued need for timely and thorough epidemiological exposure data to identify sources of foodborne infections.