Establishment of New National Rare Disease (Nambyo) Registry and Registry Guidelines in Japan.

A New legal structure for rare disease (nambyo) has been established in Japan this year, after 42 years of measures of nambyo. We have been accumulating registry for nambyo from 2003, however, as it was based on paper registration, quality was not enough. Our new registry system will be based under ISO13606 which is a new medical international standard. Authorized doctors can put in data On Line by the new system, which has data cleaning filter for accurate data entry. Patients will be supported their medical expense by authorization by this system, so the registry will be efficient.

[Patient database and orphan drug development].

There is no concept of rare disease (RD) but Nambyo (intractable disease) since 1972. In 1995 the definition of Nambyo included the concept of rareness and the frequency for a Nambyo is less than 50000 in Japanese population. Currently Nambyo are selected for special research support, and special treatment of medical expenses. The budget for research is 10 billion yen. The approximate number of medical recipients is estimated to be 700000. The measures already developed for Nambyo are not comprehensive, therefore currently several additional measures are being envisaged. We are now planning to join the Orphanet. The expectations for Orphanet Japan are to: Enhance international collaboration of RD, providing international up-to-date information of RD in Japanese, inform historical and up-to-date research of Nambyo, and promote information exchange, joint research and network establishment. It is necessary to make a Patient Registry for rare disease, and hopefully have a structure to integrate worldwide registry with same concept. Recently "International Rare Disease Research Consortium (IRDiRC)" was formed. The purpose of this consortium is to make an international coordination of the rare disease research, and to integrate the knowledge of the rare disease research. We will also talk about the Patient Registry by Patient Advocacy group, including Patient Reported Outcome (PRO).

Effects of rehydration and food consumption on salivary flow, pH and buffering capacity in young adult volunteers during ergometer exercise.

BACKGROUND: The aim of this study was to investigate the influences of rehydration and food consumption on salivary flow, pH, and buffering capacity during bicycle ergometer exercise in participants. METHODS: Ten healthy volunteers exercised on a bicycle ergometer at 80% of their maximal heart rate. These sessions lasted for two periods of 20 min separated by 5-min rest intervals. Volunteers were subjected to one of the following conditions: (1) no water (mineral water) or food consumption, (2) only water for rehydration, (3) water and food consumption, (4) a sports drink only for rehydration, and (5) rehydration with a sports drink and food. Statistical significance was assessed using one-way analysis of variance and Dunnett's test (p < 0.05). RESULTS: The salivary pH decreased significantly during and after exercise in conditions 4 and 5. The salivary buffering capacity decreased significantly during exercise and/or after the exercise in conditions 1, 3, 4, and 5. CONCLUSIONS: The results showed that salivary pH and buffering capacity decreased greatly depending on the combination of a sports drink and food.