Hawaii's Neuromuscular Disease Biopsy Registry. A quarter-century compilation of muscle biopsy diagnoses in Hawaii.

Abstract

INTRODUCTION: The Department of Pathology at Straub Clinic & Hospital began using enzyme histochemical techniques to examine muscle biopsies in 1976 and has processed most of the muscle biopsies performed in Hawaii for the past 25 years. A Muscle Biopsy Registry of biopsy findings was established in 1992 and information concerning 1,230 biopsies during the period December 1976 to June, 2000 entered into the Registry's data base. The goal was to document data derived from biopsy-based diagnoses of neuromuscular diseases since none currently exists. METHODS: Muscle biopsies received in a fresh state in the Straub Pathology Department were processed utilizing a modern approach incorporating routine histological and enzyme histochemical staining techniques of frozen sections. One pathologist [LJL] documented findings and established diagnoses for all cases. Patient information, demographics and diagnoses were recorded utilizing a Microsoft Excel spreadsheet program. RESULTS: Data sorts conducted from the Registry's data base of 1,230 biopsies produced graphs and tables illustrating demographic statistics regarding diagnoses and findings in 1,044 different patients some of whom had multiple biopsies. CONCLUSIONS: Hawaii's Neuromuscular Disease Biopsy Registry constitutes a compilation of a quarter-century of muscle biopsy-based diagnoses utilizing enzyme histochemical diagnostic techniques in pediatric and adult age groups, including specific diagnoses encountered in different ethnic groups. This information constitutes a benchmark for future epidemiologic studies, and a basis for comparison with data derived from clinical diagnoses and data derived from Death Certificates.