BACKGROUND Tuberous sclerosis complex (TSC) is a genetic disorder with an incidence of 1:6000 live births and associated with the development of benign tumors in several organs. It is also characterized by high rates of neurological and neuropsychiatric abnormalities, including epilepsy affecting 70-90% of patients and being one of the major risk factors of intellectual disability. The first se...

This paper describes log-linear generation models for Example-based Machine Translation (EBMT). In the generation model, various knowledge sources are described as the feature functions and are incorporated into the log-linear models. Six features are used in this paper: matching score and context similarity, to estimate the similarity between the input sentence and the translation example; wor...

Tuberous sclerosis complex (TSC) is an autosomal dominant disease with a wide spectrum of pathological lesions [1,2]. Almost half of the patients with TSC have an underlying renal pathology, mainly angiomyolipomas, cysts and/or renal cell carcinoma [3,4]. TSC rarely involves medium size and large vessels, including the renal arteries. Arterial aneurysms have been reported for intracranial arter...

t In this paper the most general Hz control problem is considered. We derive necessary and sufficient conditions when the infimum is attained by state feedback. We do the same for the measurement feedback case where we derive necessary and sufficient conditions when the infimum is attained by proper dynamic compensators. We also investigate reduced-order compensators if some states are observab...

Heave compensator is a system that mitigates transmission of heave movement from vessels to the equipment in the vessel. In drilling industry, a heave compensator enables drilling in offshore environments. Heave compensator attenuates movement transmitted from the vessel to the drill string and drill bit ensuring security and efficiency of the offshore drilling process. Common types of heave co...

RATIONALE AND OBJECTIVES Tuberous sclerosis complex (TSC) is a genetic neurocutaneous syndrome in which cognitive and social-behavioral outcomes for patients vary widely in an unpredictable manner. The cause of adverse neurologic outcome remains unclear. The aim of this study was to investigate the hypothesis that disordered white matter and abnormal neural connectivity are associated with adve...

A 23-year-old New Zealand Māori male with tuberous sclerosis (TSC) and associated neurocognitive abnormalities presented with altered behavior and increasing seizure frequency. Endogenous hyperinsulinemia from an underlying insulinoma was confirmed and this was managed surgically. This case represents only the sixth description of insulinoma in TSC to date. The role of the hamartin-tuberin comp...

Tuberous sclerosis complex (TSC) is an autosomal dominant neurogenetic disorder caused by mutations in the TSC1 or TSC2 genes and is frequently associated with hamartoma formation in multiple organ systems. Here, we report two novel mutations in the TSC2 gene, including a splicing mutation (IVS 29 +1G>C) in intron 29 and a deletion/insertion mutation (C.5090-5092delCCA- inAG) in exon 39 in two ...

Tuberous Sclerosis Complex (TSC) was first described in the late 1800s as a relative of neurofibromatosis, but it has since been identified as a discrete disorder. Patients with TSC typically present with facial adenomas, seizure disorder, and a developmental disability. The syndrome is caused by mutations in either chromosomes 9 or 16, both of which code for cell development and maturation. Th...

Classical angiomyolipomas are benign tumours composed of various tissues, including components of fat, abnormal blood vessels and smooth muscle cells. They are often found in association with tuberous sclerosis complex (TSC). The present study reports a male patient affected by TSC with intermittent, massive chylous pleural effusions, who developed recurrent mediastinal angiomyolipomas. The tum...