Darby C1, Sullivan G2, Rennison A3, Marr R3 Brazil N1, Breen E1, Crowley VEF4, Gannon P4

Porphyria Lab1, IMS Dept2 Dept of Clinical Biochemistry4, St James's Hospital, Dublin 8 Ireland, Wren Computing Ltd3 PO Box 180 Bury England BL8 4FQ UK

At present the Biochemistry Department, St James’s Hospital, Dublin is the only laboratory providing a comprehensive porphyria diagnostic service for the Republic of Ireland (ROI). The laboratory, with over 30 years of biochemical and clinical diagnostic experience, retains the most comprehensive record relating to the nature and extent of porphyria in ROI. Consequently, to coordinate this archive, a bespoke database was commissioned with very specific functional requirements. These included (1) a standard patient module with unique family identification (2) family tree generation facility (3) porphyrin result module (4) capability to integrate with the current hospital and laboratory information systems, thus allowing biochemical result and clinical data download (5) capability to interface with current analytical systems (6) word processing feature and (7) access to current acute porphyria safe drug list. Wren Computing developed a system based on these criteria. Our database contains a full audit trail and is stored on a Microsoft SQL 2000 server with inbuilt FTP capability for data import. Analytical traces can be directly captured from laboratory screens and historical data can be scanned into the system. The family tree facility is highly visual in a rich windows environment. The presentation will elaborate on the practical usefulness of this system.