Background： Late-onset multiple acyl-CoA dehydrogenase deficiency （MADD） is the most common type of lipid storage myopathies in China. Most patients with late-onset MADD are well responsive to riboflavin. Up to now...Background： Late-onset multiple acyl-CoA dehydrogenase deficiency （MADD） is the most common type of lipid storage myopathies in China. Most patients with late-onset MADD are well responsive to riboflavin. Up to now, these patients are often treated with glucocorticoids as the first-line drug because they are misdiagnosed as polymyositis without muscle biopsy or gene analysis. Although glucocorticoids seem to improve the fatty acid metabolism of late-onset MADD, the objective evaluation of their rationalization on this disorder and comparison with riboflavin treatment are unknown. Methods： We performed a historical cohort study on the efficacy of the two drugs among 45 patients with late-onset MADD, who were divided into glucocorticoids group and riboflavin group. Detailed clinical information of baseline and 1-month follow-up were collected. Results： After 1-month treatment, a dramatic improvement of muscle strength was found in riboflavin group （P 〈 0.05）. There was no significant difference in muscle enzymes between the two groups. Significantly, the number of patients with full recovery in glucocorticoids group was less than the number in riboflavin group （P 〈 0.05）. On the other hand, almost half of the patients in riboflavin group still presented high-level muscle enzymes and weak muscle strength after 1-month riboflavin treatment, meaning that l-month treatment duration maybe insufficient and patients should keep on riboflavin supplement for a longer time. Conclusions： Our results provide credible evidences that the overall efficacy of riboflavin is superior to glucocorticoids, and a longer duration of riboflavin treatment is necessary for patients with late-onset MADD.展开更多
Since its revival in the mid-1980s,the study of Chinese social history has enjoyed steady growth and prominence as an emerging field.Its development over the past three decades can be roughly divided into three stages...Since its revival in the mid-1980s,the study of Chinese social history has enjoyed steady growth and prominence as an emerging field.Its development over the past three decades can be roughly divided into three stages.The first is the rejuvena-tion stage,when“social history”started to gain ground as a new branch of study and developed into regional and cultural histories.The second stage started around the 1990s when the study of“new social history”arose to break away from the old paradigms and establish new approaches for a self-sustained branch of study.Due to its narrowly targeted narrative framework,obscure jargon,and exclusive rhetoric,it was later reduced to new historical research that focused on investigating concepts without due emphasis on revisiting the origin of the study.The third stage featured the rise of historical anthropology,which sprang up in the late 1990s and brought forth new ideas that spread across the country into the twentieth century with extensive influence.Hence,social history,4<new social history,and historical anthropology mark the three different yet connected stages in the development of Chinese social history studies.Each played a significant part in history with a lasting impact on the development of the field and issues raised for further inquiry.展开更多
基金grants from the National Natural Science Foundation of China，National Key Clinical Specialty Discipline Construction Program,and Fujian Key Clinical Specialty Discipline Construction Program
文摘Background： Late-onset multiple acyl-CoA dehydrogenase deficiency （MADD） is the most common type of lipid storage myopathies in China. Most patients with late-onset MADD are well responsive to riboflavin. Up to now, these patients are often treated with glucocorticoids as the first-line drug because they are misdiagnosed as polymyositis without muscle biopsy or gene analysis. Although glucocorticoids seem to improve the fatty acid metabolism of late-onset MADD, the objective evaluation of their rationalization on this disorder and comparison with riboflavin treatment are unknown. Methods： We performed a historical cohort study on the efficacy of the two drugs among 45 patients with late-onset MADD, who were divided into glucocorticoids group and riboflavin group. Detailed clinical information of baseline and 1-month follow-up were collected. Results： After 1-month treatment, a dramatic improvement of muscle strength was found in riboflavin group （P 〈 0.05）. There was no significant difference in muscle enzymes between the two groups. Significantly, the number of patients with full recovery in glucocorticoids group was less than the number in riboflavin group （P 〈 0.05）. On the other hand, almost half of the patients in riboflavin group still presented high-level muscle enzymes and weak muscle strength after 1-month riboflavin treatment, meaning that l-month treatment duration maybe insufficient and patients should keep on riboflavin supplement for a longer time. Conclusions： Our results provide credible evidences that the overall efficacy of riboflavin is superior to glucocorticoids, and a longer duration of riboflavin treatment is necessary for patients with late-onset MADD.
文摘Since its revival in the mid-1980s,the study of Chinese social history has enjoyed steady growth and prominence as an emerging field.Its development over the past three decades can be roughly divided into three stages.The first is the rejuvena-tion stage,when“social history”started to gain ground as a new branch of study and developed into regional and cultural histories.The second stage started around the 1990s when the study of“new social history”arose to break away from the old paradigms and establish new approaches for a self-sustained branch of study.Due to its narrowly targeted narrative framework,obscure jargon,and exclusive rhetoric,it was later reduced to new historical research that focused on investigating concepts without due emphasis on revisiting the origin of the study.The third stage featured the rise of historical anthropology,which sprang up in the late 1990s and brought forth new ideas that spread across the country into the twentieth century with extensive influence.Hence,social history,4<new social history,and historical anthropology mark the three different yet connected stages in the development of Chinese social history studies.Each played a significant part in history with a lasting impact on the development of the field and issues raised for further inquiry.
