Chouraqui J P, Leluyer B
Presse Med. 1986 Sep 20;15(30):1405-8.
Two children with type I glycogen-storage disease were treated at home with continuous nocturnal intragastric feeding, using a high glucose formula. The children were 6.5 years and 32 months old respectively when initiating treatment, and they have now been treated for 24 and 18 months. A high carbohydrate meal was given soon after stopping the nocturnal infusion, and this was followed by frequent daytime feeding. In this way, the children were rapidly discharged from hospital and soon returned to school. This regimen stabilized blood glucose levels, avoiding hypoglycemic complications, and improved tolerance to fasting and exercise. Moreover it decreased serum triglyceride, cholesterol, uric acid and lactate levels as well as liver size. The increase in linear growth rate was remarkable and was associated with an increase in insulin-glucagon ratio. No complications resulted from the gastric tube. The method proved to be effective, simple, practical and acceptable by children and their parents. In addition, it is relatively inexpensive and represents a reliable long-term alternative therapy to portocaval shunting for patients with type I glycogen-storage disease. The nocturnal infusions should be continued until after adolescence.
两名患有I型糖原贮积病的儿童在家中接受持续夜间胃内喂养，使用高糖配方奶。开始治疗时，两名儿童分别为6.5岁和32个月大，目前已接受治疗24个月和18个月。夜间输注停止后不久给予高碳水化合物餐，随后白天频繁喂食。通过这种方式，孩子们很快出院并重返学校。该方案稳定了血糖水平，避免了低血糖并发症，提高了对禁食和运动的耐受性。此外，它降低了血清甘油三酯、胆固醇、尿酸和乳酸水平以及肝脏大小。线性生长速度的增加显著，且与胰岛素-胰高血糖素比值的增加有关。胃管未引发并发症。该方法被证明是有效、简单、实用且为儿童及其父母所接受的。此外，它相对便宜，是I型糖原贮积病患者门腔分流术可靠的长期替代疗法。夜间输注应持续至青春期之后。
