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）：先天性高胰岛素血症引起的低血糖是婴儿和儿童中最常见的低血糖形式。如果不及时诊断和充分治疗，受影响的儿童将面临癫痫发作和永久性脑损伤的极高风险。许多患者需要进行近全胰腺切除术来控制低血糖。最近的进展已经发现，在患有高胰岛素血症的婴儿和患有导致先天性糖尿病的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存在遗传缺陷。 β细胞功能的临床和基础科学的这些进展和其他进展已经导致婴儿胰岛素过多症和相关胰岛功能障碍的诊断和治疗取得重大进展。本提案寻求支持 2006 年 6 月 15 日至 16 日在费城举行的为期两天的高胰岛素血症和新生儿糖尿病国际研讨会。本次研讨会将召集一个杰出的国际专家小组，讨论过去几年在因高胰岛素血症所致婴儿和儿童低血糖的基础科学和临床治疗方面取得的进展。研讨会面向内分泌学、外科、病理学、遗传学和放射学领域的临床研究人员和临床医生，以及涉及胰岛生物学和生物化学的基础科学家。研讨会的目标是促进基础科学家和临床科学家在高胰岛素血症和胰岛失调相关疾病的诊断和治疗方面的转化研究，并促进提高临床护理标准。该提案中要求的资金将有助于为十名实习生和年轻研究人员参加研讨会并展示自己的研究提供支持。这种支持将促进这些年轻研究人员的培训以及他们在与儿童胰岛调节障碍相关的基础和转化研究方面的学术生涯的发展。高胰岛素血症是一种罕见疾病，但却是婴儿和儿童中最常见、也是最危险的低血糖形式。研讨会的目标是促进基础科学家和临床科学家在高胰岛素血症和胰岛失调相关疾病的诊断和治疗方面的转化研究，并促进提高临床护理标准。