用适当的营养疗法能避免反复发作的低血糖,改善代谢后遗症,使患者存活至成年。然而,并发症依然是个主要问题。[17]Weinstein DA, Wolfsdorf JI. Effect of continuous glucose therapy with uncooked cornstarch on the long-term clinical course of type Ia glycogen storage disease. Eur J Pediatr. 2002;161:S35-S39.http://www.ncbi.nlm.nih.gov/pubmed/12373568?tool=bestpractice.com大多数患者30岁时将发生肝腺瘤。可致出血或演变为肝癌。青少年患者和成年患者中,肾病和骨质疏松症是常见的其他问题。
已有报道GSD Ia的女性患者正常妊娠。[18]Martens DH, Rake JP, Schwarz M, et al. Pregnancies in glycogen storage disease type Ia. Am J Obstet Gynecol. 2008;198:646.e1-e7.http://www.ncbi.nlm.nih.gov/pubmed/18241814?tool=bestpractice.com直到最近,GSD Ib患者也不能存活到能够怀孕那么长。[19]Dagli AI, Lee PJ, Correia CE, et al. Pregnancy in glycogen storage disease type Ib: gestational care and report of first successful deliveries. J Inherit Metab Dis. 2010;33:s151-s157.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3800278/http://www.ncbi.nlm.nih.gov/pubmed/20386986?tool=bestpractice.com然而,随着治疗水平的提高,年轻女性患者将能够达到生育年龄,因此,该亚型患者的首次怀孕得到报道。在整个孕期和分娩过程中母亲和胎儿都需要专业的照顾,以避免发生特殊的代谢风险。