Bücherschau

Ohne Zusammenfassung VDI     

Familial giant hemangiomas of the liver. Study of a family and review of the literature

In this report we present for the first time strong evidence for autosomal-dominant inheritance of grant liver hemangiomas. Furthermore, we review the current literature about incidence, pathogenesis, clinics, as well as diagnostic and therapeutic aspects of LH. A large family of Italian origin is described where three female patients in three successive generations suffered from large symptomatic LH. In addition, two other female relatives exhibited asymptomatic LH on sonographic scans. The restriction of the disease to the female gender could be explained by a sex-dependent difference in penetrance or expressivity of a presumable "liver-hemangioma" gene, or by known proliferative factors such as female sex hormones. We also observed an increased incidence of adenomas of the thyroidea among members with or without LH of the presented family. This uncommon familial association has also not yet been described and its genetic aspects are discussed.     

Solutions to a Radical Problem: Overview of Current and Future Treatment Strategies in Leber’s Hereditary Opic Neuropathy

Leber’s Hereditary Optic Neuropathy (LHON) is the most common primary mitochondrial DNA disorder. It is characterized by bilateral severe central subacute vision loss due to specific loss of Retinal Ganglion Cells and their axons. Historically, treatment options have been quite limited, but ongoing clinical trials show promise, with significant advances being made in the testing of free radical scavengers and gene therapy. In this review, we summarize management strategies and rational of treatment based on current insights from molecular research. This includes preventative recommendations for unaffected genetic carriers, current medical and supportive treatments for those affected, and emerging evidence for future potential therapeutics.     

Nonparallel training for voice conversion based on a parameter adaptation approach

The objective of voice conversion algorithms is to modify the speech by a particular source speaker so that it sounds as if spoken by a different target speaker. Current conversion algorithms employ a training procedure, during which the same utterances spoken by both the source and target speakers are needed for deriving the desired conversion parameters. Such a (parallel) corpus, is often difficult or impossible to collect. Here, we propose an algorithm that relaxes this constraint, i.e., the training corpus does not necessarily contain the same utterances from both speakers. The proposed algorithm is based on speaker adaptation techniques, adapting the conversion parameters derived for a particular pair of speakers to a different pair, for which only a nonparallel corpus is available. We show that adaptation reduces the error obtained when simply applying the conversion parameters of one pair of speakers to another by a factor that can reach 30%. A speaker identification measure is also employed that more insightfully portrays the importance of adaptation, while listening tests confirm the success of our method. Both the objective and subjective tests employed, demonstrate that the proposed algorithm achieves comparable results with the ideal case when a parallel corpus is available.     

The processing element design for a large-scale spatio-temporal pattern clustering system

The clustering of spatio-temporal patterns are essential for many applications. Established from the biological analogy of the cortex, the parametrically coupled logistic map network (PCLMN) provides a viable solution to the clustering problem. To engineer for a single-chip spatio-temporal pattern clustering system, the highly modular PCLMN is designed in analog circuit. In this paper, the 0.6 μm 5 V CMOS design of the processing element is presented. The analog design employs self-calibration techniques to improve the accuracy and robustness of the nonlinear circuits. A fabricated element covers a die area of 0.55 mm², and consumes 240 mW power at 5 V supply. After calibration, simulation and testing results show that the element fulfills the system-level requirement of the Cort-X model for driving signals up to 1 MHz.