Defining and Characterising a Toolkit for the Development of a Successful European Registry for Rare Liver Diseases.

Background: A rare disease is defined by the European Health Commission as a disorder affecting less than 5/10,000 population. There are at least 20 rare liver diseases seen frequently in the adult and paediatric liver clinic, signifying that the hepatology community can be influential in developing such patient databases for registering patients with rare hepatic conditions. The aim of this systematic review was to 1) identify registries for rare liver diseases in Europe and 2) design a universal blueprint for the development of a registry for rare liver diseases by using lessons learnt from the already established European registries. Methods: We searched PubMed, google scholar and clinicaltrials.gov using the MESH terms “registries”, “database management systems”, “database” and the non-MESH terms “database$”, “registry”, “repository” and “repositories”. We only included studies in English from countries/consortia of the European Union (EU). Our literature search was performed in 2020. Results: We identified 37 registries for rare liver disease in Europe. Using information from the design of these registries we developed a blueprint for the development of a patient registry for rare liver diseases consisting of a theoretical, technical and maintenance phase. Interpretation: It is believed that rare diseases may affect as much as 6–8% of the EU population across its 28 member states. Here we have provided a toolkit for designing a registry for rare liver disease. Our article will complement the efforts of loco-regional, national and international groups seeking to establish robust systems for data collection and analysis for orphan liver diseases.

Lessons From Transitional Reforms: What Are The Fundamentals For Successful Transformation?

After more than two decades of enduring global reforms, the current paper makes an attempt to investigate the fundamental prerequisites of a successful reform program. The study is particularly complicated by the ambiguity surrounding the very subject matter. While most scholars are united in the view that in order to facilitate a successful transitional process, transitional economies must execute fundamental grassroots reforms, there is no formally accepted universal blueprint of what is considered to be an acceptable transitional program. In an effort to broaden the scope of the investigation, the author probes into neoclassical economic thinking and examines ‘conventional reform indicators’ commonly associated with successful reforms. But the quest brings the author to the realization that mainstream neoclassical economic thinking by itself is not sufficient; it leaves behind unanswered fundamental questions which—for the sake of methodological and pragmatic necessity—demands resolution. Consequently, the author excavates beneath superficial philosophical thinking, and probes ‘mainstream theories’ for answers to valid problems confronting transitioning economies. The inquiry is not only beneficial for knowledge, but has implications for policy-making. The paper is sectioned into four parts: introduction is an appraisal of conventional thinking; section II examines reform pre-requisites and pragmatic questions; section III is an attempt to suggest answers to questions prompted in section II and rationalize unequal reform outcomes in reforming societies that execute identical reform program; the concluding section synchronously weaves together the different pieces and ideas by drawing attention on the uniqueness of contemporary reforms and lessons learned from past reforms.

Unified field—the universal blueprint?

The current status of the studies of the origin of the fundamental particles and the universe is presented. These studies indicate the unified field to be the source of both the fundamental particles and the universe itself. Furthermore, as a consequence of the unique properties of the quantum vacuum, the unified field is presumed to exist, in a quantum physical sense, everywhere in the very fabric of spacetime. In an analogy to the characteristics of the human genome, unified field appears to have the basic blueprint of at least everything physical in this universe.