Klupa Tomasz, Małecki Maciej, Skupień Jan, Szalecki Mieczysław, Jałowiec Irena, Surdej Barbara, Myśliwiec Małgorzata, Sieradzki Jacek
Katedra i Klinika Chorób Metabolicznych, Collegium Medicum, Uniwersytet Jagielloński, Kraków.
Przegl Lek. 2007;64(6):398-400.
Activating mutations in the KCNJ11 gene encoding the ATP-sensitive potassium-channel subunit of Kir6.2 result in the phenotype of permanent neonatal diabetes (PNDM). Patients with PNDM can be successfully transferred from insulin to sulphonylurea. It is not clear, however, whether the type of diet may play a role in the metabolic control in PNDM patients. This report describes two cases of patients with PNDM due to the R201H mutation coming from the Polish Nationwide Registry of PNDM treated with the same sulphonylurea (glipizide GITS). In one of them, diet was practically free (Pol1), the other one (Pol2) avoided high glycemic-index products. Both mutation carriers were submitted to a 72 h continuous glucose monitoring system (CGMS) (Medtronic, CA). Before the CGMS record, families were encouraged not to alter their usual pattern of food intake during recording periods and to use food diaries. The postprandial glycemia in Poll reached the maximal level of 9.5 mmo/l, 5 episodes of glycemia above 8.0 mmol/l lasting overall for about 6 hours followed the ingestion of high-glycemic-index (>70) meals. Patient Pol2 did not use high-glycaemic-index-products and his postprandial blood glucose did not exceed 7.0 mmol/l. Following the CGMS record, an additional diet-oriented educational session with patient Poll and his parents was performed, Poll declared to avoid the intake of high-glycemic-index products. He remained on the same dose of Glipizide GITS. Results of home blood glucose monitoring performed 2 months later showed normoglycemia. We conclude that to achieve normoglycemia, patients with PNDM who are on sulphonylurea should refrain from eating high glycemic-index products.
编码Kir6.2的ATP敏感性钾通道亚基的KCNJ11基因激活突变会导致永久性新生儿糖尿病（PNDM）的表型。PNDM患者能够成功地从胰岛素治疗转换为磺脲类药物治疗。然而，饮食类型是否会在PNDM患者的代谢控制中发挥作用尚不清楚。本报告描述了两例因R201H突变导致的PNDM患者，他们来自波兰全国性PNDM登记处，均接受相同的磺脲类药物（格列吡嗪控释片）治疗。其中一例患者的饮食几乎不受限制（Pol1），另一例（Pol2）则避免食用高血糖指数产品。两名突变携带者均接受了72小时连续血糖监测系统（CGMS）（美敦力公司，加利福尼亚州）监测。在进行CGMS记录之前，鼓励家属在记录期间不要改变他们通常的食物摄入模式，并使用食物日记。Pol1患者餐后血糖最高达到9.5 mmol/L，在摄入高血糖指数（>70）餐食后，有5次血糖超过8.0 mmol/L，持续时间总计约6小时。患者Pol2未食用高血糖指数产品，其餐后血糖未超过7.0 mmol/L。在CGMS记录之后，对Pol1患者及其父母进行了一次额外的以饮食为导向的教育课程，Pol1宣称会避免摄入高血糖指数产品。他继续服用相同剂量的格列吡嗪控释片。2个月后进行的家庭血糖监测结果显示血糖正常。我们得出结论，为实现血糖正常，接受磺脲类药物治疗的PNDM患者应避免食用高血糖指数产品。
