International Medical Conference of Congenital Hyperinsulinism

先天性高胰岛素血症国际医学会议

• 批准号: 7162041
• 负责人: CHARLES ALFRED STANLEY
• 金额: $ 0.6万
• 依托单位: CHILDREN'S HOSP OF PHILADELPHIA
• 依托单位国家: 美国
• 财政年份: 2006
• 资助国家: 美国
• 起止时间: 2006-06-01 至 2007-05-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7162041
• 关键词: children; congenital disorders; diabetes mellitus; hyperinsulinism; hypoglycemia; international cooperation; meeting /conference /symposium

DESCRIPTION (provided by applicant): Hypoglycemia due to congenital hyperinsulinism is the most common form of hypoglycemia in infants and children. Affected children are at extreme risk of seizures and permanent brain damage if not promptly diagnosed and adequately treated. Many require surgical treatment with near-total pancreatectomy to control hypoglycemia. Recent advances have identified genetic defects in several different steps in the pathways regulating beta-cell insulin secretion in infants with hyperinsulinism and in neonates with closely related disorders causing congenital diabetes mellitus. These and other advances in clinical and basic science of beta-cell function have already led to major improvements in the diagnosis and treatment of infants with hyperinsulinism and related disorders of islet function. The present proposal seeks support for a two-day international symposium on hyperinsulinism and neonatal diabetes on June 15 & 16, 2006 in Philadelphia. This symposium will assemble a distinguished panel of international experts to discuss the advances that have occurred during the past few years in both the basic science and clinical management of infants and children with hypoglycemia due to hyperinsulinism. The symposium is targeted at clinical investigators and clinicians in endocrinology, surgery, pathology, genetics, and radiology, as well as basic scientists involved with islet biology and biochemistry. The goals of the symposium are to stimulate translational research between basic and clinical scientists in the diagnosis and treatment of hyperinsulinism and related disorders of islet dysregulation and to facilitate improved standards of clinical care. The funds requested in this proposal will help provide support for ten trainees and young investigators to attend the symposium and present their own research. This support will foster the training of these young investigators and their development of academic careers in basic and translational research related to the disorders of islet regulation in children. Hyperinsulinism is a rare condition, but represents the most common, as well as the most dangerous form of hypoglycemia in infants and children. The goals of the symposium are to stimulate translational research between basic and clinical scientists in the diagnosis and treatment of hyperinsulinism and related disorders of islet dysregulation and to facilitate improved standards of clinical care.

描述（由申请人提供）：由于先天性高胰岛素而引起的低血糖是婴儿和儿童中最常见的低血糖形式。如果未及时诊断并接受充分治疗，受影响的儿童面临着癫痫发作和永久性脑损伤的极端风险。许多人需要接受近距离胰腺切除术的手术治疗以控制低血糖症。最近的进步已经确定了在高胰岛素高度胰岛素主义的婴儿以及引起先天性糖尿病密切相关疾病的新生儿中调节β细胞胰岛素分泌的途径的几个不同步骤中的遗传缺陷。 β细胞功能的临床和基础科学方面的这些进展和其他进步已经导致了胰岛素过度胰岛素和胰岛功能相关疾病的诊断和治疗的重大改善。本提案在2006年6月15日至16日在费城举行了为期两天的国际高胰岛素和新生儿糖尿病的国际研讨会。该研讨会将组装一个杰出的国际专家小组，讨论过去几年中在婴儿和低血糖儿童过度胰岛素的基础科学和临床管理中所取得的进步。该研讨会针对内分泌，手术，病理学，遗传学和放射学以及与胰岛生物学和生物化学有关的基础科学家的临床研究者和临床医生。研讨会的目标是刺激基本科学家和临床科学家之间的转化研究，以诊断和治疗胰岛失调的高胰岛素和相关疾病，并促进改善临床护理标准。本提案中要求的资金将有助于为十名受训者和年轻调查员参加研讨会并介绍自己的研究。这种支持将促进对这些年轻研究者的培训及其在与儿童胰岛调节疾病有关的基础和转化研究中的学术职业发展。超胰岛素是一种罕见的疾病，但代表了婴儿和儿童中最常见的低血糖形式。研讨会的目标是刺激基本科学家和临床科学家之间的转化研究，以诊断和治疗胰岛失调的高胰岛素和相关疾病，并促进改善临床护理标准。