Dambska M, Labrador E B, Kuo C L, Weinstein D A
Glycogen Storage Disease Program, Connecticut Children's Medical Center, Hartford, Connecticut.
Department of Community Medicine and Health Care, University of Connecticut School of Medicine, Farmington, Connecticut.
Pediatr Diabetes. 2017 Aug;18(5):327-331. doi: 10.1111/pedi.12540. Epub 2017 Jun 1.
Prior to 1971, type Ia glycogen storage disease was marked by life-threatening hypoglycemia, lactic acidosis, severe failure to thrive, and developmental delay. With the introduction of continuous feeds in the 1970s and cornstarch in the 1980s, the prognosis improved, but complications almost universally developed. Changes in the management of type Ia glycogen storage disease have resulted in improved metabolic control, and this manuscript reviews the increasing evidence that complications can be delayed or prevented with optimal metabolic control as previously was seen in diabetes.
1971年以前，I型糖原贮积病的特征是危及生命的低血糖、乳酸酸中毒、严重发育不良和发育迟缓。随着20世纪70年代持续喂养法的引入以及80年代玉米淀粉的应用，预后有所改善，但几乎普遍出现了并发症。I型糖原贮积病管理方式的改变带来了更好的代谢控制，本文综述了越来越多的证据，即通过优化代谢控制可以延缓或预防并发症，就像之前在糖尿病中看到的那样。
