R116C mutation of cationic trypsinogen in a Turkish family with recurrent pancreatitis illustrates genetic microheterogeneity of hereditary pancreatitis

Hereditary pancreatitis is due to heterozygosity for gain-of-function mutations in the cationic trypsinogen gene which result in increased levels of active trypsin within pancreatic acinar cells and autodigestion of the pancreas. The number of disease-causing defects is generally considered to be low. To gain further insight into the molecular basis of this disorder, DNA sequence analysis of all five exons was performed in 109 unrelated patients with idiopathic chronic pancreatitis in order to determine the variability of the underlying mutations. Two German females and one German male were carriers of the most common N291 and R122H mutations (trypsinogen numbering system). In a Turkish proband, an arginine (CGT) to cysteine (TGT) substitution at amino acid position 116 was identified. Family screening demonstrated that the patient had inherited the mutation from his asymptomatic father and that he had transmitted it to both of his children, his daughter being symptomatic since the age of 3 years. In addition, a German male was found to be a heterozygote for a D100H (GAC-->CAC) amino acid replacement. Our data provide evidence for genetic heterogeneity of hereditary pancreatitis. The growing number of cationic trypsinogen mutations is expected to change current mutation screening practices for this disease

Parametrised phonological event parsing

This paper describes a phonological event parser for spoken language recognition which has been provided with a parametrisable development environment for examining the extent to which linguistically significant issues such as linguistic competence (structural constraints) and linguistic performance (robustness) can play a role in the spoken language recognition task.Ein phonologischer Ereignisparser zur Erkennung gesprochener Sprache wird zusammen mit einer parametrisierbaren Entwicklungsumgebung vorgestellt. Diese Umgebung dient nicht nur der Entwicklung und Konsistenz- und Vollständigkeitsprüfung des zugrundeliegenden computerphonologischen Modells, sondern ermöglicht auch eine gezielte Evaluierung ausgewählter linguistisch motivierter constraints zur robusten Erkennung gesprochener Sprache

Ein Synkretismusmodell für die deutsche Morphologie

Morphologische Modelle in der Computerlinguistik reflektieren typischerweise sprachspezifische Eigenschaften, indem sie Datenstrukturen und Operationen verwenden, die der Typologie einzelner Sprachen entsprechen. Ausgehend von einer Diskussion der synkretistischen Eigenschaften der deutschen Flexionsmorphologie wird eine generische denotationelle Semantik fur die bekannten sprachübergreifenden morphologischen Strukturen entwickelt. Diese Semantik liegt dem Konzept eines Compilers für ein generatives morphologisches Lexikon zugrunde, das 7000 Stämme aus einem Corpus spontan gesprochener deutscher Dialoge auf 30.000 Vollformen und 120.000 morphologische Kategorienabbildungen (nach Auflösung der Synkretismen) projiziert.Morphology models in computational linguistics have tended to be language-specific, in that the data structures and operations used have reflected the typology of individual languages. Starting with a discussion of the syncretistic properties of German inflectional morphology, a generic denotational semantics for known language-independent inflectional structures is outlined. This semantics underlies the design of a generative morphological lexicon compiler for spoken German, which projects 7000 stems extracted from a corpus of spoken language dialogues to 30,000 fully inflected forms and 120,000 morphological category mappings (after resolution of syncretism)

The treatment of compounds in a morphological component for speech recognition

This paper describes a morphological component in a speech recognition system for German dealing with the construction of complex word form hypotheses out of a lattice of simplex forms.Our example is the recognition of compounds from their individual components. Evaluation results are presented for speech recognition with and without morphologically based word recognition.Dieser Aufsatz beschreibt eine Morphologiekomponente in einem Spracherkennungssystem für das Deutsche, welche die Konstruktion von komplexen Worthypothesen aus einem Wörtergitter von Simplizia am Beispiel der Erkennung von Komposita aus ihren Einzelbestandteilen behandelt. Evaluationsergebnisse fur morphologisch und nicht-morphologisch basierte Worterkennung werden vorgestellt

Morphology and speech technology

This paper describes a morphological component in a speech recognition architecture for German dealing with the recognition of compounds from their individual constituents. The specification of our morphological model allows for variation in functionality, e.g. the reconstruction of split compounds, of lexicalised, and of non-lexicalised (unknown) compounds.An implementation and evaluation results for split compounds are presented

Price Discrimination in Two-Sided Markets

We examine the profitability and the welfare implications of price discrimination in two-sided markets. Platforms have information about the preferences of the agents that allows them to price discriminate within each group. The conventional wisdom from one-sided horizontally differentiated markets is that price discrimination hurts the firms and benefits consumers, prisoners' dilemma. Moreover, it is well-known that the presence of indirect externalities in two-sided markets can intensify the competition. Despite all these, we show that the possibility of price discrimination, in a two-sided market, may actually soften the competition. Therefore, the implications of price discrimination from one-sided markets may not carry over to two-sided markets. This is the case regardless of whether prices are public or private, although private prices boost profits. Our analysis also sheds light on the welfare properties of price discrimination in intermediate goods markets, such as Business-to-Business (B2B) markets

Strongly coupled chameleon fields: possible test with a neutron Lloyd's mirror interferometer

The consideration is presented of possible neutron Lloyd's mirror interferometer experiment to search for strongly coupled chameleon fields. The chameleon scalar fields were proposed to explain the early and late time acceleration of expansion of the Universe. They may produce short-range interaction between particles and matter. This interaction causes phase shift of neutron waves in the interferometer. Estimates of sensitivity are performed.Comment: 11 p, 3 fig; the title is changed, extended feasibility consideration: expected intensity and systematic effect

Location Decisions of Competing Platforms

There are examples of entry in two-sided markets, where first entrants occupy a 'central location' and serve agents with 'intermediate tastes', while later entrants are niche players. Why would the first entrant choose to become a 'general' platform, given that later entrants will not have enough room for differentiation, resulting in an intense price competition? This one-sided market logic may not apply in a two-sided market. A key difference in a two-sided market, stemming from the presence of cross-group network externalities, is stronger demand creation. We develop a model which can deliver the above mentioned empirical observation, when the network externalities are intermediate. On the other hand, when externalities are low, our model predicts that differentiation will be maximum, as it would be in a one-sided market. Finally, for strong externalities only one platform is active and locates at the center

Outcome of children with low-grade cerebellar astrocytoma: long-term complications and quality of life

Objects: To study the long-term outcome of surgically treated low-grade cerebellar astrocytomas in children. Materials and methods: We followed 31 consecutive patients under 16years of age who were diagnosed between 1980 and 2005 in a single institution. In 21 of 31 survivors (median follow-up time 7.9years; range 5.6-27.4years) who agreed to participate, tumor control, neurological and cognitive complications, and their impact on behavioral and emotional adjustment and health-related quality of life (HRQoL) were comprehensively assessed qualitatively and quantitatively. Results: Neurological sequelae were found in 43%. However, age-appropriate ability to perform daily life activities was normal in all patients. Remarkably, cognitive deficits leading to significant school problems occurred in 19% and behavioral and emotional adjustment disturbances in 27%. In comparison with healthy controls, the survivors rated their HRQoL similarly or even higher. Conclusion: Childhood low-grade cerebellar astrocytomas have an excellent cure rate by tumor surgery alone. When compared with other pediatric brain tumors, the risk of neurological, cognitive, emotional, and behavioral complications is relatively small. HRQoL is similar to that of healthy control