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We report on ciliary aplasia of the respiratory tract, a rare disorder of the mucociliary apparatus, that is insufficiently recognized as a distinct entity. A culture method for ciliogenesis was developed by our laboratory and offers the advantage of studying cilia free of secondary changes associated with infection. Three cases of primary ciliary aplasia were documented histologically in direct biopsy specimens and also in biopsy specimens cultured specifically for ciliogenesis. Primary ciliary aplasia should be differentiated from secondary ciliary aplasia in which basal bodies are present and ciliogenesis takes place in specific culture. Only hereditary ciliary abnormalities are expressed in cell cultures. We critically review the cases of ciliary aplasia reported to date.  相似文献   
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Introduction

Thiopental is a cornerstone in the treatment of refractory status epilepticus and intractable intracranial hypertension. In our center we observed that thiopental might cause falsely elevated serum sodium levels.

Methods

Triggered by a recent case experience of extremely elevated serum sodium levels during thiopental treatment, we retrospectively identified 53 patients treated with thiopental in our intensive care unit between 2007 and 2011 and evaluated electrolyte changes. We differentiated the analysis before and after introduction of a new device for sodium assays (Dimension Vista, Siemens) in the central laboratory in April 2010. Standardized in vitro laboratory tests were performed to study the effect of thiopental on sodium analysis.

Results

Before April 2010, serum sodium levels determined in the central laboratory showed a good agreement with the bedside point-of-care (POC) device during thiopental therapy with [sodium]laboratory ? [sodium]POC of only 1.08 mmol/L (P = .0517). After April 2010, a strong discrepancy between laboratory values and POC values was observed with [sodium]laboratory ? [sodium]POC = 11.57 mmol/L (P < .0001). Standardized in vitro testing confirmed that thiopental induced a dose-dependent false hypernatremia (P = .002).

Conclusions

Thiopental treatment can result in falsely elevated serum sodium. This is a critical finding since high sodium levels preclude administrating mannitol or hypertonic saline for the treatment of elevated intracranial pressure. Moreover, a false high sodium level might lead to the inappropriate administration of hypotonic fluids potentially resulting in increased brain edema and even higher intracranial pressure. To our knowledge, this is the first paper describing this clinically relevant phenomenon.  相似文献   
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Background:

In several European countries, centralization of oesophagogastric cancer surgery has been realized and clinical audits initiated. The present study was designed to evaluate differences in resection rates, outcomes and annual hospital volumes between these countries, and to analyse the relationship between hospital volume and outcomes.

Methods:

National data were obtained from cancer registries or clinical audits in the Netherlands, Sweden, Denmark and England. Differences in outcomes were analysed between countries and between hospital volume categories, adjusting for available case‐mix factors.

Results:

Between 2004 and 2009, 10 854 oesophagectomies and 9010 gastrectomies were registered. Resection rates in England were 18·2 and 21·6 per cent for oesophageal and gastric cancer respectively, compared with 28·5–29·9 and 41·4–41·9 per cent in the Netherlands and Denmark (P < 0·001). The adjusted 30‐day mortality rate after oesophagectomy was lowest in Sweden (1·9 per cent). After gastrectomy, the adjusted 30‐day mortality rate was significantly higher in the Netherlands (6·9 per cent) than in Sweden (3·5 per cent; P = 0·017) and Denmark (4·3 per cent; P = 0·029). Increasing hospital volume was associated with a lower 30‐day mortality rate after oesophagectomy (odds ratio 0·55 (95 per cent confidence interval 0·42 to 0·72) for at least 41 versus 1–10 procedures per year) and gastrectomy (odds ratio 0·64 (0·41 to 0·99) for at least 21 versus 1–10 procedures per year).

Conclusion:

Hospitals performing larger numbers of oesophagogastric cancer resections had a lower 30‐day mortality rate. Differences in outcomes between several European countries could not be explained by differences in hospital volumes. To understand these differences in outcomes and resection rates, with reliable case‐mix adjustments, a uniform European upper gastrointestinal cancer audit with recording of standardized data is warranted. Copyright © 2012 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd.  相似文献   
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