Tommaso Adriana Maria Alves de, Hessel Gabriel, Riccetto Adriana Gut, Semenzati Graziela de Oliveira, Gusmão Reinaldo Jordão
Universidade Estadual de Campinas, São Paulo, SP, Brazil.
Rev Paul Pediatr. 2019 Jul 4;37(4):516-519. doi: 10.1590/1984-0462/;2019;37;4;00005. eCollection 2019.
To discuss aspects of pre and post-operative otorhinolaryngology surgery in patients with glycogen storage disease type 1b.
Description of three clinical cases with probable glycogen storage disease type 1b who underwent otorhinolaryngology surgery, showing the importance of multidisciplinary interaction to avoid episodes of hypoglycemia.
Patients with glycogen storage disease type 1b present recurrent infections, including the otorhinolaryngology affections. When there is an indication for surgical treatment, the caloric intake should be carefully followed in order to prevent hypoglycemia. The way to ensure this is to perform the pre and postoperative period in the hospital ward. In the postoperative period, it is important to make a slow transition between the intravenous and oral routes and not suspend the infusion of glucose during the surgical procedure. The cases illustrate the need for the interaction of the otorhinolaryngologic surgeon with the anesthesiologist, the pediatrician and the gastro-pediatrician in the management of these patients, avoiding hypoglycemic episodes.
探讨1b型糖原贮积病患者耳鼻咽喉科手术的术前和术后相关问题。
描述3例可能患有1b型糖原贮积病并接受耳鼻咽喉科手术的临床病例，显示多学科协作对于避免低血糖发作的重要性。
1b型糖原贮积病患者常反复发生感染，包括耳鼻咽喉科相关感染。当有手术治疗指征时，应密切关注热量摄入以预防低血糖。确保这一点的方法是在医院病房进行术前和术后阶段的管理。在术后，重要的是在静脉和口服途径之间缓慢过渡，并且在手术过程中不要停止葡萄糖输注。这些病例说明了耳鼻咽喉科外科医生与麻醉师、儿科医生和胃肠病儿科医生在管理这些患者时相互协作以避免低血糖发作的必要性。
