BACKGROUND: Gaucher disease is an autosomal recessive lysosomal glycosphingolipid storage disorder resulting from a deficiency of lysosomal enzyme acid â-glucosidase (glucocerebrosidase). This partial enzyme deficiency results in accumulation of glycosphingolipid-laden macrophages (Gaucher cells) throughout the liver, spleen, bone marrow, skeleton, lungs and brain (only in types 2 and 3). OBJECTIVE: These guidelines aim to provide a standard of care for patients with Gaucher disease in keeping with international standards, but also realistic for South Africa, and to provide a shared-care model for treating physicians and funders regarding care for these patients. RECOMMENDATIONS: All healthcare professionals involved in the diagnosis and management of Gaucher disease should take note of and implement these guidelines in clinical practice as far as possible. VALIDATION: These guidelines were developed through consensus by the Lysosomal Storage Disorder Medical Advisory Board. They are largely based on the UK 2005 National Guidelines for Gaucher Disease, but include new treatment recommendations for enzyme replacement therapy based on subsequent publications. The Southern African Society for Human Genetics (SASHG) (who have endorsed the guidelines) and the National Osteoporosis Foundation of South Africa (NOFSA) provided valuable input. GUIDELINES SPONSOR: Genzyme initiated the project and sponsored the meetings of the Advisory Board and all costs generated by these meetings. CONCLUSION: It is intended that these guidelines will enable all patients suffering from Gaucher disease to be diagnosed and offered the best possible care available.

Different axioms underlie efficient market theory and Keynes's liquidity preference theory. Efficient market theory assumes the ergodic axiom. Consequently, today's decision makers can calculate with actuarial precision the future value of all possible outcomes resulting from today's decisions. Since in an efficient market world decision makers "know" their intertemporal budget constraints, decision makers never default on a loan, i.e., systemic defaults, insolvencies, and bankruptcies are impossible. Keynes liquidity preference theory rejects the ergodic axiom. The future is ontologically uncertain. Accordingly systemic defaults and insolvencies can occur but can never be predicted in advance.

Este ensayo de Paul Davidson critica los postulados básicos y la adopción en bloque de las diez reformas del Consenso de Washington, cuya aplicación en los últimos lustros ha sido desastrosa para los países en desarrollo. Muestra que los postulados del libre cambio y algunas de las recomendaciones que se derivan de ellos (disciplina fiscal, liberalización de las tasas de interés, tasa de cambio competitiva, libre inversión extranjera directa y búsqueda de la competitividad internacional mediante la reducción de los salarios) no corresponden al mundo real y son engañosas y perjudiciales. Luego de revisar las consecuencias de la aplicación en bloque de estas medidas, presenta una propuesta de reforma del sistema de pagos internacionales basada en la que Keynes presentó en Bretón Woods. Para terminar, comenta críticamente las propuestas de James Tobin, George Soros y Milton Friedman.

Paul Davidson’s essay criticises the basic postulates and blanket adoption of the Washington Consensus’ ten reforms, whose application has been disastrous for developing countries during the last few years. It shows that the postulates regarding free trade and some of the recommendations deriving from them (fiscal discipline, liberalising interest rates, competitive exchange rate, free direct foreign investment and the search for international competitiveness by reducing wages) do not correspond to the real world and are misleading and prejudicial. After revising the consequences of a blanket application of these measures, he presents a proposal for reforming the international payment system based on that which Keynes presented at Breton Woods. The essay finishes by making some critical comments about proposals put forward by James Tobin, George Soros and Milton Friedman.

Cet essai de Paul Davidson, critique les postulats de bases et l´adoption en bloc des dix réformes du Consensus de Washington, dont l´application ces derniers lustres a été désastreuse pour les pays en voie de développement. Il signale que les postulats du libre échange et quelques une des recommandations qui en dérive (discipline fiscale, libéralisation des taux d´intérêt, taux de change compétitif, investissement étranger direct libre et recherche de la compétitivité internationale par l´intermédiaire de la réduction des salaires) ne correspondent pas au monde réel en plus d´être trompeuses et nuisibles. Après avoir révisé les conséquences de l´application en bloc de ces mesures, il présente une proposition de réforme du système des paiements internationaux basée sur celle que Keynes a présenté à Breton Woods. Pour finir, il commente de façon critique les propositions de James Tobin, George Soros et Milton Friedman.

RESUMO Até 1973, o sistema de pagamentos internacionais do pós-guerra foi, em grande medida, moldado pela tese de Keynes de que as taxas de câmbio flexíveis e a livre mobilidade internacional de capital são incompatíveis com o pleno emprego global e o rápido crescimento econômico em uma era de livre comércio multilateral (Felix, 1977-8) . Isso resultou em um sistema monetário internacional estável que permitiu que a economia global experimentasse um crescimento econômico e prosperidade sem paralelo, apesar dos controles de capital generalizados e das regulamentações do mercado financeiro internacional. Desde 1973, o sistema financeiro tem se tornado cada vez mais frágil, com crises recorrentes e cada vez mais estressantes da dívida internacional e da liquidez da moeda, ameaçando a estabilidade da economia global.

ABSTRACT Until 1973 the postwar international payments system was, in large measure, shaped by Keynes’s thesis that flexible exchange rates and free international capital mobility are incompatible with global full employment and rapid economic growth in an era of multilateral free trade (Felix, 1977-8). This resulted in a stable international monetary system that permitted the global economy to experience unparalleled economic growth and prosperity despite widespread capital controls and international financial market regulations. Since 1973, the financial system has grown progressively more fragile with recurrent and increasingly stressful international debt and currency liquidity crises threatening the stability of the global economy.

ABSTRACT This note discusses A. P. Thirlwall’s economic development model as a “demand-driven” model.

RESUMO esta nota discute o modelo de desenvolvimento econômico de A. P. Thirlwall como um modelo “orientado pela demanda”.