Abstract
Electrical status epilepticus during sleep (ESES) is an age-related, self-limited epileptic encephalopathy characterized by heterogeneous clinical manifestations and a specific ...electroencephalographic pattern of continuous spikes and waves during slow sleep. The etiology of ESES is not completely clear, although structural brain lesions, abnormal immunological markers, and genetic mutations have been associated with the syndrome. ESES was first described in 1971 and since then, the diagnostic criteria have changed multiple times. Additionally, inconsistency between authors in how to record and evaluate the electroencephalogram also leads to variability between studies. These inconsistencies hamper objectivity, comparison, and generalization. Because of this, one of the first priorities of physicians treating this condition should be defining the parameters of this disease so that cooperative building can occur.
ALG6-CDG is a rare, but second most common, type 1 congenital disorder of glycosylation (CDG) caused by a defect in the α-1-3-glucosyltransferase (ALG6) enzyme in the N-glycan assembly pathway. Many ...mutations have been identified and inherited in an autosomal recessive pattern. There are less than 100 ALG6-CDG cases reported, all sharing the phenotype of hypotonia and developmental delay. The majority (perhaps >70%) have seizures, but a minority have intractable epilepsy or epileptic encephalopathy. We report the clinical course, EEG findings, and neuroimaging of a child found to have compound heterozygous alleles c.257 + 5G > A and c.680G > A (p.G227E) who developed explosive onset of intractable epilepsy and epileptic encephalopathy. Initially, CDG was not suspected due to its rarity and lack of multi-organ system involvement, but rapid whole exam sequence (8-day turnaround) revealed the specific diagnosis quickly.
The epidemy of metabolic syndrome (MetS) is typically preceded by adoption of a "risky" lifestyle (e.g., dietary habit) among populations. Evidence shows that those with low socioeconomic status ...(SES) are at an increased risk for MetS. To investigate this, we recruited 123 obese subjects (body mass index BMI ≥ 30) from Chicago. Multi-omic data were collected to interrogate fecal microbiota, systemic markers of inflammation and immune activation, plasma metabolites, and plasma glycans. Intestinal permeability was measured using the sugar permeability testing. Our results suggest a heterogenous metabolic dysregulation among obese populations who are at risk of MetS. Systemic inflammation, linked to poor diet, intestinal microbiome dysbiosis, and gut barrier dysfunction may explain the development of MetS in these individuals. Our analysis revealed 37 key features associated with increased numbers of MetS features. These features were used to construct a composite metabolic-inflammatory (MI) score that was able to predict progression of MetS among at-risk individuals. The MI score was correlated with several markers of poor diet quality as well as lower levels of gut microbial diversity and abnormalities in several species of bacteria. This study reveals novel targets to reduce the burden of MetS and suggests access to healthy food options as a practical intervention.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
Die Pfriemenmücke Sylvicola zetterstedti sowie die Fliegen Mycetaulus latipennis und Scathophaga decipiens wurden im 21. Jahrhundert neu für Nordrhein-Westfalen nachgewiesen.
Neuere westfälische Funddaten der Zikaden Centrotus cornutus, Goniagnathus brevis und Platymetopius major, der Blattflöhe Livia junci, Camarotoscena speciosa und Arytaina genistae, der ...Kamelhalsfliege Xanthostigma xanthostigma und des Winterhaftes Boreus hyemalis werden mitgeteilt.
Abstract Background Prognostication of congestive heart failure post-myocardial infarction (MI) is important for decision making. We sought of a head-to-head comparison between the prognostic ...implication of clinical, cardiopulmonary, and left ventricular (LV) function assessment. Methods Retrospectively, 100 consecutive post-MI patients (MI history 1418 ± 1668 days ago) were stratified by NYHA functional classification system, cardiopulmonary exercise testing (CPX) oxygen consumption at maximal exercise (VO2max ) and at the anaerobic threshold (VO2AT ) resulting in the Weber classification, and LV function analysis by M-mode and two-dimensional echocardiography LV end-diastolic and -systolic diameter index (LVDDI and LVSDI), shortening fraction (%D), and LV end-diastolic and -systolic volume index (EDVI and ESVI), LV ejection fraction (EF). Patients were controlled by phone call 1470 ± 607 days later. Results There was only a modest correlation between NYHA and Weber classes ( r = 0.402) and no correlation between VO2max and ESVI ( r = 0.080) nor between NYHA and ESVI ( r = 0.174). Several parameters (ESVI, LVDDI, LVSDI, %D) could discriminate NYHA classes to a higher significance ( p = 0.05; 0.0008; 0.0002; 0.04) than the Weber classes (n.s.; p = 0.03; n.s.; n.s.). The following parameters could significantly differentiate quartiles in a log-rank analysis (Kaplan–Meier survival curves): NYHA classes ( p = 0.0001), Weber classes ( p = 0.069), EDVI ( p = 0.004), ESVI ( p = 0.0001), EF ( p = 0.002), LVDDI ( p = 0.002), LVSDI ( p < 0.001) and %D ( p < 0.001). Multivariate analysis isolated the following three parameters implying decreasing, independent prognostic information: NYHA classes ( p = 0.001), ESVI ( p = 0.003), and Weber classes ( p = 0.040). Conclusions In post-MI patients the thorough clinical assessment according the NYHA functional classification system implies higher prognostic information than more objective measures. This should be considered especially in primary care and should lessen the dependence on costly and expertise-dependent technical investigations.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
Die Echten Blattwespen Aglaostigma nebulosum, Macrophya rufipes, M. recognata und Nematus caeruleocarpus, die Gespinstblattwespe Pamphilius betulae sowie die Holzwespe Xeris spectrum wurden 2002 bzw. ...2003 in Westfalen erstmals bzw. wieder nachgewiesen.
Die acalyptraten Fliegen Phyllomyza longipalpis Schmitz und Gymnochiromyia inermis Collin wurden bei Hagen in Mehrzahl aus Eichenmulm gezogen und damit für die deutsche und die westfälische Fauna ...nachgewiesen.
Der in Westfalen verschollene Laufkäfer Diachromus germanus wurde im Herbst 2002 auf dem Boden der abgelassenen Glör-Talsperre in mehreren frischen Exemplaren gefunden.
