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Wolf-Hirschhorn syndrome is characterized by severe growth and mental retardation, microcephaly, seizures and 'Greek helmet' facies, caused by partial deletion of the short arm of chromosome 4. Growth charts are given from 0-4 years of age, based on the study of 101 individuals. Use of these specific growth charts is recommended, because standard growth charts are inapplicable for patients with WHS.

Bone disease might occur early in the course in autosomal recessive cutis laxa syndrome. We report on a significant clinical improvement and stabilization in our patients following bisphosphonate therapy. We suggest early, systemic evaluation and follow up of bone density in all children presenting with inherited cutis laxa.

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A 3 4 7 -A 7 6 6 A705 Detection System dominated manual counting alone by preventing 2 additional RSS while resulting in $620,789 fewer costs. Cost reductions were due to the efficiencies gained by using the device, lower utilization of radiographs, and fewer medical/legal costs resulting from RSS. Model results were robust to the sensitivity analyses. Even with legal costs removed from the model, which was by far the greatest cost, to conservatively reflect an ex-U.S. medical malpractice landscape, SituateTM Detection System still reduced costs by $65,448 and continued to dominate manual counting. ConClusions: The implications of RSS can be both clinically and economically significant for patients and healthcare facilities, and the literature suggests that standard manual counting is not optimal in preventing RSS. Our model suggests that, even using conservative estimates, SituateTM Detection System can avert medical and legal costs, decrease unproductive OR time, and most importantly, more effectively prevent RSS.objeCtives: Popular media and the scientific literature frequently point to Big Data analytics as an excellent way to improve health outcomes and reduce healthcare costs. The question is, can it really deliver? This study used early health technology assessment (HTA) methodologies to assess Big Data (BD) analytics in healthcare. Methods: Interviews were conducted with clinicians in three different areas (chronic lymphocytic leukaemia, diabetes and intensive care) to identify potential applications of BD analytics. The potential health and economic impact of each application was then estimated using existing HTA methodologies (including decision tree modelling). Results: The generic nature of BD analytics complicates development and clarification of the scope. Therefore, several critical requirements should be considered besides existing HTA methodologies. These requirements are: the clinical value of the BD analytics application, the technical feasibility, and the availability of data. Case studies revealed that the most common obstacle hampering further development of BD analytics for use in a particular application was insufficient data. Estimates of the potential impact of BD analytics on health gains or cost-savings requires a combination of 'Big Data' (e.g., genetics data) with other data (e.g., long-term survival). ConClusions: BD analytics is unlikely to change clinical decision-making on its own. Certain requirements are essential to ensure that it is not viewed as a big hype that does not deliver on its promises. First, clinicians must identify shortcomings in care. Second, it is essential to consider whether the available data is sufficient to enable BD analytics to achieve its potential. Early HTA can also help BD analytics to meet its potential since it can estimate the potential health and economic benefits before all the work is done.

In addition, the authors would like to clarify the citing sentence, the third sentence of the second paragraph of the right column on page 134, to read:In one study of four physically healthy volunteers with a previous history of amphetamine use, repeated hourly administration of 10 mg of oral dextroamphetamine produced initial euphoria, followed by depressive symptoms at cumulative dosages exceeding 50 mg, and full paranoid psychosis in all subjects at cumulative dosages between 120 and 750 mg.

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