Mutations in the RNA component of RNase MRP cause a pleiotropic human disease, cartilage-hair hypoplasia.

The recessively inherited developmental disorder, cartilage-hair hypoplasia (CHH) is highly pleiotropic with manifestations including short stature, defective cellular immunity, and predisposition to several cancers. The endoribonuclease RNase MRP consists of an RNA molecule bound to several proteins. It has at least two functions, namely, cleavage of RNA in mitochondrial DNA synthesis and nucleolar cleaving of pre-rRNA. We describe numerous mutations in the untranslated RMRP gene that cosegregate with the CHH phenotype. Insertion mutations immediately upstream of the coding sequence silence transcription while mutations in the transcribed region do not. The association of protein subunits with RNA appears unaltered. We conclude that mutations in RMRP cause CHH by disrupting a function of RNase MRP RNA that affects multiple organ systems.

Regional inequalities in health and health care in Finland and Norway.

One of the main health policy goals in Finland and Norway is to decrease regional differences in health status and in accessibility of health services. The purpose of this paper is to analyze how this policy has been implemented, how its goals have been achieved, and what the obstacles are to it. It is of special interest to assess whether the centralized Finnish planning system has been more successful in the implementation of this policy, than the more decentralized system in Norway. Of these two countries, only Norway has applied any objective computing criteria for assessing the relative need of health care resources in each province or municipality. Neither of these two countries is using any relevant statistical indicators to describe the present situation, or time-related trends in regional inequities. According to available data the regional inequalities in health status and in the provision of health services have in both countries remained more or less unchanged over the last two decades. In future a more exact definition of the concept of equity, better methods for assessing the need for services, and an improved system of health statistics and indicators for monitoring progress in equity is needed. Also the potential of the health care policy in decreasing inequities in health should be questioned at a more fundamental level.

Health policies and Health For All strategies in the Nordic countries.

In the 1980s all five Nordic countries expressed, at the highest political level, their commitment to WHO's program Health for All (HFA) by the Year 2000. This program aims at improving the prerequisites for a healthier and longer life by changing lifestyles, decreasing environmental hazards and by shifting the emphasis in health care from specialized services towards primary health care. Following their policy statements all the Nordic countries formulated national HFA strategies during the latter half of the 1980s. This paper looks at the background and the formulation and implementation of these strategies, covering the period up until the end of the 1980s. The implementation of national strategies seems to be a slow and difficult process. The monitoring of progress toward accepted healthy policy goals is not easy, because measurable objectives or targets are mostly missing.

Is primary health care progressing in Europe?

Primary health care (PHC) is the key to attaining the goal of health for all (HFA) by the year 2000. Also the European countries have accepted the declarations and WHO resolutions related to global and European HFA strategies. However, the implementation of regional and national strategies has met with many obstacles, caused by reluctant attitudes and poor planning and management systems. In this review the objective of PHC in industrialized countries, the evaluation process of HFA strategy, and progress in PHC in Europe in the 1980s are discussed. Lack of defined national objectives, and health information systems which are not adopted to purposes of monitoring progress in PHC are causing most of the problems in evaluation. There is a clear positive development in PHC resources and organization in the Nordic countries. Generally speaking PHC is progressing very slowly in Europe, if there is any progress at all. This can be said both about organization of health care, planning and management systems and about application of PHC principles like community participation and intersectoral collaboration. The national health information systems should be quickly revised to allow more exact monitoring of progress towards the 38 European targets and additional national targets.