Visser Gepke, Rake Jan Peter, Labrune Philippe, Leonard James V, Moses Shimon, Ullrich Kurt, Wendel Udo, Smit G Peter A
Beatrix Children's Hospital, Groningen, The Netherlands.
Eur J Pediatr. 2002 Oct;161 Suppl 1:S120-3. doi: 10.1007/s00431-002-1017-6. Epub 2002 Sep 13.
Life expectancy in glycogen storage disease type 1 (GSD-1) has improved considerably. Its relative rarity implies that no metabolic centre has experience of large series of patients and therefore experience with long-term management and follow-up at each centre is limited. There is wide variation in methods of dietary and pharmacological treatment. Based on data from the European Study on Glycogen Storage Disease Type 1, discussions within this study group together with those at the International SHS Symposium 'Glycogen Storage Disease Type I and II: Recent Developments, Management and Outcome', Fulda, Germany (2000) and on data from the literature, a series of guidelines were drawn up.
the following guidelines for the management of patients with glycogen storage disease type 1b are in addition to those general guidelines for glycogen storage disease type 1 and address specific problems related to neutropenia and neutrophil dysfunction.
1型糖原贮积病（GSD - 1）患者的预期寿命有了显著提高。该病相对罕见，这意味着没有哪个代谢中心有大量患者的治疗经验，因此每个中心在长期管理和随访方面的经验有限。饮食和药物治疗方法存在很大差异。基于欧洲1型糖原贮积病研究的数据，该研究小组内部的讨论以及在德国富尔达举行的国际SHS研讨会“1型和2型糖原贮积病：最新进展、管理与结果”（2000年）上的讨论，再结合文献数据，制定了一系列指南。
以下关于1b型糖原贮积病患者管理的指南是对1型糖原贮积病通用指南的补充，针对与中性粒细胞减少和中性粒细胞功能障碍相关的具体问题。
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