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- Weinreb, Neal J, et al.
(författare)
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Gaucher disease type 1 : revised recommendations on evaluations and monitoring for adult patients.
- 2004
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Ingår i: Semin Hematol. - 0037-1963. ; 41:4 Suppl 5, s. 15-22
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)abstract
- For patients with type 1 Gaucher disease, challenges to patient care posed by clinical heterogeneity, variable progression rates, and potential permanent disability that can result from untreated or suboptimally treated hematologic, skeletal, and visceral organ involvement dictate a need for comprehensive, serial monitoring. An updated consensus on minimum recommendations for effective monitoring of all adult patients with type 1 Gaucher disease has been developed by the International Collaborative Gaucher Group (ICGG) Registry coordinators. These recommendations provide a schedule for comprehensive and reproducible evaluation and monitoring of all clinically relevant aspects of this disease. The initial assessment should include confirmation of deficiency of beta-glucocerebrosidase, genotyping, and a complete family medical history. Other assessments to be performed initially and at regular intervals include a complete physical examination, patient-reported quality of life using the SF-36 survey, and assessment of hematologic (hemoglobin and platelet count), visceral, and skeletal involvement, and biomarkers. Specific radiologic imaging techniques are recommended for evaluating visceral and skeletal pathology. All patients should undergo comprehensive regular assessment, the frequency of which depends on treatment status and whether therapeutic goals have been achieved. Additionally, reassessment should be performed whenever enzyme therapy dose is altered, or in case of significant clinical complication.
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- Maier, Lena, 1964-
(författare)
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EU, arbetsrätten och normgivningsmakten : en rättslig studie av gemenskapsrättens inverkan på medlemsstaternas arbetsrättsliga normgivningsutrymmen
- 2000
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Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- When Sweden joined the Community its freedom of action became limited. The national legislator's and social partners' lawmaking discretion in the area of labour law has been circumscribed partly through the transfers of competence, partly through the Community obligations that the Member States are bound to fulfil. These restrictions have been more and more visible during the last decade, as a result of the European integration process, which has resulted in an explicit and more independent social dimension whithin the Community.The dissertation deals with the limitations of the national lawmaking discretion in the labour law area that follow from Community law. It is the strategies and courses of action that the institutions of the Community, including the Court of Justice, have taken, that form the central starting point of the study. The division of powers between the Community and the Member States is usually discussed whithin the framework of the constitutional law, and the study begins in this area of law. With a view to adapt this discussion to a labour law environment a model of analysis has been developed, which is adjusted to this specific area. The model is finally applied through an analysis of the lawmaking whithin the area of pay.The analysis of the area of pay concludes that even if wage formation usually is regarded as a matter solely for the Member States and their social partners, such an assumption can be nothing but a point of departure. It is not possible to categorically exclude any pay related matter from the Community's competence from a legal point of view. In reality, however, there has been an almost unanimous wish on both national and Community level to let pay, with the exemption of pay discrimination, remain a matter for the Member States. This position has nevertheless been weakened to some extent as a result of the continuous integration process and its tendency to create an increased number of links between various material areas and matters.In general the analysis distinguishes a development towards a strategy of regulation at Community level which aims at creating a balance between national and Community considerations in the labour law area. It is also possible to identify the principle of diversity as a fundamental element in the Community's lawmaking regarding labour law, particularly with a view to protect both the Member States' legal orders and the autonomy of the social partners. The analysis also reveals that the social objectives are now given a greater importance than earlier in relation to the economic rationale on which the free movement and the internal market are founded
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