Willlink Unit, Genetic Medicine, Central Manchester University Hospitals NHS Foundation Trust, St Mary's Hospital, Oxford Road, Manchester M13 9WL, UK.
J Inherit Metab Dis. 2011 Jun;34(3):631-42. doi: 10.1007/s10545-011-9335-3. Epub 2011 May 10.
Continuous glucose monitoring systems (CGMS) are now in widespread use in diabetes management with an increasing evidence base. There are few reports of their use in GSD. Liver glycogen storage disorders (GSDs) are most often managed by intensive dietary regimens. Risks of over and under-treatment remain. We describe our use of CGMS in a cohort of GSD patients, the results obtained and the frequency of complications. Our experience is that CGM is a reliable, well accepted and valid tool in the monitoring of GSD patients and allows for assessment of blood sugar control in the 'real-life' setting, unlike hospital admissions. Combining CGM with urine ketone and / or blood lactate measurements, again at home, improves the investigation yet further. It is possible to perform CGM for periods including both schooldays and weekends, and also to change the dietary regimen during the period of monitoring to reduce the frequency of assessments. Risks of decreased reliability in the low range of blood sugars may be outweighed by the increased validity of the patient being in the home environment, with a normal diet and activity schedule.
连续血糖监测系统（CGMS）在糖尿病管理中得到了广泛应用，其证据基础也在不断增加。在 GSD 中使用 CGMS 的报道很少。肝糖原贮积症（GSD）的治疗主要依赖于强化饮食方案。过度和治疗不足的风险仍然存在。我们描述了 CGMS 在一组 GSD 患者中的使用情况、获得的结果以及并发症的发生频率。我们的经验是，CGMS 是监测 GSD 患者的可靠、可接受和有效的工具，它可以评估患者在“真实生活”环境中的血糖控制情况，而不需要住院。将 CGMS 与尿酮和/或血乳酸测量结合起来，同样在家里进行，可进一步提高检查的效果。可以进行包括上学日和周末在内的 CGMS 监测，并且可以在监测期间改变饮食方案以减少评估的频率。低血糖范围的可靠性降低的风险可能会被患者在家中环境、正常饮食和活动计划下的更高的有效性所抵消。
