Cross-sectional study of 168 patients with hepatorenal tyrosinaemia and implications for clinical practice

Background: Hepatorenal tyrosinaemia (Tyr 1) is a rare inborn error of tyrosine metabolism. Without treatment, patients are at high risk of developing acute liver failure, renal dysfunction and in the long run hepatocellular carcinoma. The aim of our study was to collect cross-sectional data. Methods. Via questionnaires we collected retrospective data of 168 patients with Tyr 1 from 21 centres (Europe, Turkey and Israel) about diagnosis, treatment, monitoring and outcome. In a subsequent consensus workshop, we discussed data and clinical implications. Results: Early treatment by NTBC accompanied by diet is essential to prevent serious complications such as liver failure, hepatocellular carcinoma and renal disease. As patients may remain initially asymptomatic or develop uncharacteristic clinical symptoms in the first months of life newborn mass screening using succinylacetone (SA) as a screening parameter in dried blood is mandatory for early diagnosis. NTBC-treatment has to be combined with natural protein restriction supplemented with essential amino acids. NTBC dosage should be reduced to the minimal dose allowing metabolic control, once daily dosing may be an option in older children and adults in order to increase compliance. Metabolic control is judged by SA (below detection limit) in dried blood or urine, plasma tyrosine (<400 μM) and NTBC-levels in the therapeutic range (20-40 μM). Side effects of NTBC are mild and often transient. Indications for liver transplantation are hepatocellular carcinoma or failure to respond to NTBC. Follow-up procedures should include liver and kidney function tests, tumor markers and imaging, ophthalmological examination, blood count, psychomotor and intelligence testing as well as therapeutic monitoring (SA, tyrosine, NTBC in blood). Conclusion: Based on the data from 21 centres treating 168 patients we were able to characterize current practice and clinical experience in Tyr 1. This information could form the basis for clinical practice recommendations, however further prospective data are required to underpin some of the recommendations.

[Reviews] Turkey and the European Union: prospects for a difficult encounter

Book review of 'Turkey and the European Union: Prospects for a Difficult Encounter', edited by Esra Lagro and Knud Erik Jorgensen, Basingstoke and New York: Palgrave Macmillan, 2007 Pp. xii þ 240; 1 table; 1 figure; index; ISBN 9780230019553; £47.00 (hb).

Condition assessment of a 63-year old reinforced concrete structure exposed to the North Sea

This paper describes the condition of a reinforced concrete balustrade consisting of some 1000 individual beam elements all exposed similarly to the hostile marine environment of the North Sea at Arbroath, Scotland since 1943. A comparison is made of the condition of the original construction with the condition of repairs carried out in 1968 and in 1993. It is shown that the 1943 construction shows very little corrosion-induced cracking and little rust staining even though it does not appear to be of high construction quality. Only a very low percentage of the balustrade beams have been replaced. In contrast the beam installed in 1968 and later in 1993 show very considerable and large concrete cracks directly attributable to the corrosion of the longitudinal reinforcement, even though the concrete is of a higher quality and density. A detailed condition survey and statistics of crack sizes are presented in the paper. It is found that the higher corrosion resistance of the 1943 concrete is generally consistent with the concrete electrical resistivity measurements but the degree of corrosion of the reinforcing bars is inconsistent with chloride penetration measurements. The results are compared with the very few observations available in the literature for ageing concrete structures in marine environments. The results cast doubt on the conventional wisdom that chloride content at the reinforcement correlates well with reinforcement corrosion.

A comparison of the degree of implementation of marine biodiversity indicators by European countries in relation to the Marine Strategy Framework Directive (MSFD)

The degree of development and operability of the indicators for the Marine Strategy Framework Directive (MSFD) using Descriptor 1 (D1) Biological Diversity was assessed. To this end, an overview of the relevance and degree of operability of the underlying parameters across 20 European countries was compiled by analysing national directives, legislation, regulations, and publicly available reports. Marked differences were found between countries in the degree of ecological relevance as well as in the degree of implementation and operability of the parameters chosen to indicate biological diversity. The best scoring EU countries were France, Germany, Greece and Spain, while the worst scoring countries were Italy and Slovenia. No country achieved maximum scores for the implementation of MSFD D1. The non-EU countries Norway and Turkey score as highly as the top-scoring EU countries. On the positive side, the chosen parameters for D1 indicators were generally identified as being an ecologically relevant reflection of Biological Diversity. On the negative side however, less than half of the chosen parameters are currently operational. It appears that at a pan-European level, no consistent and harmonized approach currently exists for the description and assessment of marine biological diversity. The implementation of the MSFD Descriptor 1 for Europe as a whole can therefore at best be marked as moderately successful.

Biomonitoring of air pollutants by using lichens (Evernia prunastri) in areas between Kenitra and Mohammedia cities in Morocco.

In this study, Evernia prunastri, a lichen growing in its natural habitat in Morocco was analysed for the concentration of five heavy metals (Fe, Pb, Zn, Cu and Cr) from eleven sites between Kenitra and Mohammedia cities. The control site was Dar Essalam, an isolated area with low traffic density and dense vegetation. In the investigated areas, the concentration of heavy metals was correlated with vehicular traffic, industrial activity and urbanization. The total metal concentration was highest in Sidi Yahya, followed by Mohammedia and Bouznika. The coefficient of variation was higher for Pb and lower for Cu, Zn and Fe. The concentrations of most heavy metals in the thalli differed significantly between sites (p<0.01). Principal component analysis (PCA) revealed a significant correlation between heavy metal accumulation and atmospheric purity index. This study demonstrated also that the factors most strongly affecting the lichen flora were traffic density, the petroleum industry and paper factories in these areas. Overall, these results suggest that the index of atmospheric purity and assessment of heavy metals in lichen thalli are good indicators of the air quality at the studied sites.

Short reflections on the history of African communication

This article aims to propose a chronological subdivision in the history of African communication. African communication today is one of the most important axes for implementing development strategies, sustaining education, health, and schooling programmes, and so on. However, many of these programmes fail due to a lack of or ineffective communication between international organisations, local elite and lay people. The reasons for this situation must be found in Africa’s history of communication, which has undergone radical transformations in its different phases. Using the functionalist analysis drawn up by Jakobson, this article proposes a new chronological subdivision of Africa’s history of communication, reflecting on the current contradictions in contemporary communication in Africa.

'The Aftermath of the Annan Plan Referendums' Cross-Voting Moderation for Cyprus

This article examines recent developments in the Cyprus negotiations and suggests a number of changes to the proposed electoral system. Specifically, cross-voting and other electoral methods that encourage coalition-building across ethnic communities might add significantly to the functionality of the Annan Plan. Combined with other innovative mechanisms already in the plan, cross-voting could force political parties to seriously take into account the interests and concerns of the two Cypriot communities, an element that is currently missing from both the Turkish Cypriot (TC) and Greek Cypriot (GC) political systems. Special conditions on the island, as well as the way most political parties operated in the critical pre-April 2004 referendum period, suggest the need for this amendment. Although this study respects the consociational logic of the Annan Plan, it supplements consociationalism with elements that foster integration and inter-dependence between the two communities and their voters. The article also reviews the postreferendum developments in Cyprus which might have worrisome future implications, not only for its two communities, but also for EU enlargement in general. Cyprus both holds one of the keys to Turkey's entrance into the EU and is a litmus test for the Euro-Atlantic nexus and its capacity to pacify and integrate ethnically divided societies in Europe and elsewhere.

Optical colors of 56 near-Earth objects: trends with size and orbit

We present the results of BVRIZ photometry of 56 near-Earth objects (NEOs) obtained with the 1-m Jacobus Kapteyn telescope on La Palma during 2000 and 2001. Our sample includes many NEOs with particularly deep 1 - mum pyroxene/olivine absorption bands, similar to Q-type asteroids. We also classify three NEOs with particularly blue colors. No D-type asteroids were found, placing an upper limit of similar to2% on the fraction of the NEO population originating in the outer main belt or the Trojan clouds. The ratio of dark to bright objects in our sample was found to be 0.40, significantly higher than current theoretical predictions. As well as classifying the NEOs, we have investigated color trends with size and orbit. We see a general trend for larger silicate objects to have shallower absorption bands but find no significant difference in the distribution of taxonomic classes at small and large sizes. Our data clearly show that different taxonomic classes tend to occupy different regions of (a, e) space. By comparing our data with current model predictions for NEO dynamical evolution we see that Q- R-, and V-type NEOs tend to have orbits associated with "fast track" delivery from the main belt, whereas S-type NEOs tend to have orbits associated with "slow track" delivery. This outcome would be expected if space weathering occurs on time scales of >10(6) years. (C) 2003 Elsevier Science (USA). All rights reserved.