<Emphasis Type="Italic">Hunter disease eClinic:</Emphasis>interactive,computer-assisted,problem-based approach to independent learning about a rare genetic disease

Background  

Computer-based teaching (CBT) is a well-known educational device, but it has never been applied systematically to the teaching of a complex, rare, genetic disease, such as Hunter disease (MPS II).     

Histogram analysis of MR imaging-derived cerebral blood volume maps： combined glioma grading and identification of low-grade oligodendroglial subtypes

BACKGROUND AND PURPOSE： Inclusion of oligodendroglial tumors may confound the utility of MR based glioma grading. Our aim was, first, to assess retrospectively whether a histogram-analysis method of MR peifusion images may both grade gliomas and differentiate between low-grade oligodendroglial tumors with or without loss of heterozygosity （LOH） on 1p/19q and, second, to assess retrospectively whether low-grade oligodendroglial subtypes can be identified in a population of patients with high-grade and low-grade astrocytic and oligodendroglial tumors.     

Automated measurement of the elastase-inhibitory capacity of plasma with a centrifugal analyzer.

We describe an automated method for measuring the elastase-inhibitory capacity of plasma. We use a GEMSAEC centrifugal analyzer and succinyltrialanine-p-nitroanilide as an elastase substrate. The method is fast (80--100 samples/h) and accurate (CV = 3.6%). The detection limits are from 20 to 300% of the normal value. The elastase-inhibitory capacity is well correlated with the trypsin-inhibitory capacity and with the immunochemically determined concentration of alpha 1-proteinase inhibitor (alpha 1-antitrypsin). Because elastase is not inhibited by other plasma inhibitors, this method can be used as an enzymatic technique for measuring the concentration of plasma alpha 1-proteinase inhibitor. In an apparently normal population, this concentration was 40 +/- 11 mumol/L.     

Reninaktivität im Nierenvenenblut und ihre Stimulierbarkeit durch Hydralazin und Theophyllin

Zusammenfassung 1. Es wird über die Plasmareninaktivität im Nierenvenenblut bei 50 Patienten mit primärer und renaler Hypertonie nach Gabe von Hydralazin und Theophyllin berichtet. Die nach diesen Pharmaka beobachtete Stimulation der Reninsekretion beim Gesunden und bei Kranken mit labiler Hypertonie bleibt bei der Mehrzahl der Patienten mit fortgeschrittener primärer Hypertonie aus. Die Stimulierbarkeit bzw. Starre der Reninsekretion durch Natriumentzug zeigt ein analoges Verhalten.2. Ursächlich kommt für den Plasmareninanstieg nach Hydralazin in erster Linie der Blutdruckabfall in Betracht, der zu einer Erregung der Baroreceptoren im terminalen Vas afferens führt. Für Theophyllin wird eine Erregung der Chemoreceptoren an der Macula densa angenommen.3. Die fehlende Stimulierbarkeit der Reninsekretion bei Patienten mit fortgeschrittener primärer Hypertonie, die sich sowohl nach Hydralazin wie nach Theophyllin oder Natriumentzug zeigt, führen wir auf eine verminderte Ansprechbarkeit der Receptoren des juxtaglomerulären Zellkomplexes zurück, die als adaptiver Mechanismus aufgefaßt werden kann.Mit Unterstützung der Deutschen Forschungsgemeinschaft     

Visuelle Funktionen bei Legasthenie

BACKGROUND: We aimed to test children with developmental dyslexia for possible alterations of their spatial and visuoperceptual ability. METHODS: A total of 31 children with developmental dyslexia were included in the study. All children underwent a complete ophthalmological and orthoptic examination. Neuropsychological testing was performed using the Visual Object and Space Perception Battery (VOSP) and the subtest "Gestalt Closure" of the Kaufman Assessment Battery for children. Even though the VOSP is validated and standardized for adults, no normal values for children exist thus far. Therefore, a sex- and age-matched control group was tested. In addition, the parents of the dyslectic children completed the Child Behavior Checklist for Ages 4-18 (CBCL/4-18). RESULTS: All children presented normal ophthalmological and orthoptic findings. Performance in the VOSP subtests "progressive silhouettes test'" and "number location" was significantly reduced in dyslectic children when compared to controls ( p<0.0003 and p<0.046, respectively). There was also a tendency towards lower values in the subtest "position discrimination" ( p=0.07). In the other subtests no significant difference between dyslectic children and controls was observed. The results of the subtest "Gestalt Closure" of the K-ABC and the CBCL/4-18 were within normal ranges. CONCLUSION: The study demonstrates that developmental dyslexia is not based on ocular deficits, but indicates possible disturbances of visual-spatial cognition and visual-spatial perception in dyslectic children.