University College London

About: University College London is a education organization based out in London, United Kingdom. It is known for research contribution in the topics: Population & Context (language use). The organization has 81105 authors who have published 210603 publications receiving 9868552 citations. The organization is also known as: UCL & University College, London.

Myotonic dystrophy: time for evidence-based therapy.

Abstract: This editorial refers to ‘Cardiac involvement in myotonic dystrophy: a nationwide cohort study’[†][1], by M. Lund et al. , on page 2158 . Lund and colleagues report on the association between cardiac disease and myotonic dystrophy using data derived from Danish patient registries.1 The major finding that myotonic dystrophy is strongly associated with cardiac disease throughout life emphasizes the importance of continuous cardiac follow-up and the urgent need for prevention and treatment of cardiac complications in this disease. The study also illustrates the value of research based on routinely collected healthcare data. Myotonic dystrophy is the most common muscular dystrophy in adults (incidence 1 in 8000 live births).2 There are two genetically distinct forms; myotonic dystrophy type 1 (DM1 or Steinert's disease) and the rarer myotonic dystrophy type 2. DM1 is caused by expansion of a repetitive trinucleotide sequence (CTG) in the 3'-untranslated region of the myotonic dystrophy protein kinase (DMPK) gene which, when transcribed into CUG-containing RNA, forms aggregates of mutant transcripts that sequester RNA-binding proteins and cause abnormal splicing of downstream effector genes. In addition to this ‘RNA toxicity’ mechanism, other effects on protein translation and turnover and activation of cellular stress pathways have been observed2−7 ( Figure 1 ). Figure 1 Summary of pathophysiological pathways that may be involved in the cardiac manifestations of myotonic dystrophy type 1.2–7 The current disease paradigm for myotonic dystrophy type 1 is based on a toxic RNA gain-of-function hypothesis that results in gene missplicing, dysregulation of … [1]: #fn-2

Voriconazole versus itraconazole for antifungal prophylaxis following allogeneic haematopoietic stem-cell transplantation

Abstract: Antifungal prophylaxis for allogeneic haematopoietic stem-cell transplant (alloHCT) recipients should prevent invasive mould and yeast infections (IFIs) and be well tolerated. This prospective, randomized, open-label, multicentre study compared the efficacy and safety of voriconazole (234 patients) versus itraconazole (255 patients) in alloHCT recipients. The primary composite endpoint, success of prophylaxis, incorporated ability to tolerate study drug for ≥ 100 d (with ≤ 14 d interruption) with survival to day 180 without proven/probable IFI. Success of prophylaxis was significantly higher with voriconazole than itraconazole (48·7% vs. 33·2%, P 10%) treatment-related adverse events were vomiting (16·6%), nausea (15·8%) and diarrhoea (10·4%) for itraconazole, and hepatotoxicity/liver function abnormality (12·9%) for voriconazole. More itraconazole patients received other systemic antifungals (41·9% vs. 29·9%, P < 0·01). There was no difference in incidence of proven/probable IFI (1·3% vs. 2·1%) or survival to day 180 (81·9% vs. 80·9%) for voriconazole and itraconazole respectively. Voriconazole was superior to itraconazole as antifungal prophylaxis after alloHCT, based on differences in the primary composite endpoint. Voriconazole could be given for significantly longer durations, with less need for other systemic antifungals.

Objects look different sizes in the right and left eyes.

Abstract: Coren and Porac (1976) reported that objects looked larger in the right eye of right-eye dominant subjects and in the left eye of left-eye dominant subjects. This paper attempts to repeat that finding. Two circles of same or different size were presented haploscopically in a binocular three-field tachistoscope, to right or left visual half-field and to the upper or lower visual field, one to the right eye and one to the left. A total of 43 subjects reported which of the two circles was the larger, each subject carrying out 120 trials of the experiment. Overall subjects reported that the stimulus to the left eye was significantly larger than that presented to the right eye. There was no association with eye dominance, and therefore the Coren and Porac finding could not be repeated. There was however a very significant association with handedness, left-handed subjects tending to report that the stimulus in the right eye looked larger, and right-handed subjects reporting that the stimulus in the left eye looked larger.