Impact assessment of autonomous DRT systems

The market entrance of shared autonomous vehicles (SAV) may have disruptive effects on current transport systems and may lead to their total transformation. For many small and medium-sized cities, a full replacement of public transport services by these systems seems to be possible. For a transport system operator, such a system requires a bigger fleet of vehicles than before, however, vehicles are less expensive and fewer staff is needed for the actual operation. In this paper, we are using a simulation-based approach to evaluate the service quality and operating cost of a demand responsive transit (DRT) system for the city of Cottbus (100 000 inhabitants), Germany. The simulation model used is based on an existing MATSim model of the region that depicts a typical work day. Results suggest, that the current public transport system may be replaced by a system of 300 to 400 DRT vehicles, depending on their operational mode. Compared to previous, schedule based public transport, passengers do not need to transfer, and their overall travel times may be reduced significantly. Results for the cost comparison are preliminary, but results suggest that an autonomous DRT system is not necessarily more expensive than the current public transport system

Allogeneic hematopoietic cell transplantation in Farber disease

BACKGROUND Farber disease (FD) is a rare, lysosomal storage disorder caused by deficient acid ceramidase activity. FD has long been considered a fatal disorder with death in the first three decades of life resulting either from respiratory insufficiency as a consequence of airway involvement or from progressive neurodegeneration because of nervous system involvement. Peripheral symptoms associated with FD, including inflammatory joint disease, have been described to improve relatively rapidly after hematopoietic cell transplantation (HCT). AIMS To evaluate the disease-specific status and limitations in the long-term follow-up after HCT, investigate genotype/phenotype correlations and the benefit of allogeneic HCT in FD patients with nervous system involvement. PATIENTS AND METHODS Transplant- and disease-related information of ten FD patients was obtained by using a questionnaire, physicians' letters and additional telephone surveys. ASAH1 gene mutations were identified to search for genotype/phenotype correlations. RESULTS After mainly busulfan-based preparative regimens, all patients engrafted with one late graft loss. The inflammatory symptoms resolved completely in all patients. Abnormal neurologic findings were present pre-transplant in 4/10 patients, post-transplant in 6/10 patients. Mutational analyses revealed new mutations in the ASAH1 gene and a broad diversity of phenotypes without a genotype/phenotype correlation. With a median follow-up of 10.4 years, overall survival was 80% with two transplant-related deaths. CONCLUSION Allogeneic HCT leads to complete and persistent resolution of the inflammatory aspects in FD patients. It appears to have no beneficial effect on progression of nervous system involvement. New mutations in the acid ceramidase gene were identified. A genotype/phenotype correlation could not be established

Defibrotide for prophylaxis of hepatic veno-occlusive disease in paediatric haemopoietic stem-cell transplantation: an open-label, phase 3, randomised controlled trial

Hepatic veno-occlusive disease is a leading cause of morbidity and mortality after haemopoietic stem-cell transplantation (HSCT). We aimed to assess whether defibrotide can reduce the incidence of veno-occlusive disease in this setting