SULFONYLUREA THERAPY IN PATIENTS WITH MUTATIONS IN CF CONDUCTANCE REGULATOR GENE

CF 电导调节基因突变患者的磺酰脲治疗

• 批准号: 6414964
• 负责人: DAVID J KLEIN
• 金额: $ 2.85万
• 依托单位: CINCINNATI CHILDRENS HOSP MED CTR
• 依托单位国家: 美国
• 财政年份: 2000
• 资助国家: 美国
• 起止时间: 2000-12-01 至 2001-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/6414964
• 关键词: clinical research; cystic fibrosis; developmental nutrition; drug screening /evaluation; gene mutation; human subject; human therapy evaluation; inborn carbohydrate metabolism disorder; longitudinal human study; metabolism disorder chemotherapy; nutrition related tag; prediabetic state; respiratory disorder chemotherapy; respiratory function; sulfonylurea

There is a progressive decline in carbohydrate tolerance associated with CF genotypes whose major manifestations include pancreatic insufficiency. During the long silent period prior to the development of overt diabetes mellitus, these alterations may impair the patients' ability to maintain optimal nutrition, growth and pulmonary function. We will test the hypothesis that three years of sulfonylurea therapy will improve the nutritional status, promote growth, and slow the progressive decline in pulmonary function in patients with CF who are not yet insulin-requiring diabetics.

与CF基因型相关的碳水化合物耐受性逐渐下降，其主要表现包括胰腺功能不全。在发展为明显糖尿病之前的漫长沉默期内，这些改变可能会损害患者维持最佳营养、生长和肺功能的能力。 我们将检验这样的假设：三年的磺酰脲类药物治疗将改善尚未需要胰岛素的糖尿病患者的营养状况、促进生长并减缓肺功能的进行性下降。