Walton and Nattrass first proposed limb-girdle muscular dystrophy (LGMD) as a nosological entity in 1954.Their definition included the following characteristics:, 20. Schematic of the sarcomere with labeled molecular components that are known to cause limb-girdle muscular dystrophy or myofibrillar myopathy. Mutations in actin and nebulin cause the congenital myopathy nemaline rod myopathy, and the mutations in myosin cause familial hypertrophic cardiomyopathy.
In Lean, Standard Work (often called standardized work) is the cornerstone of any continuous improvement effort. It locks in gains and provides a foundation for future advances. Standard Work helps companies reach their improvement targets, but also provides a stable, reasonable working environment for frontline employees.
Performance Management & Compensation. Defining Performance Standards. Performance standards provide the employee with specific performance expectations for each major duty. They are the observable behaviors and actions which explain how the job is to be done, plus the results that are expected for satisfactory job performance. The purpose of performance standards is to communicate expectations. Keep in mind that good performance typically involves more than technical expertise.