INSPPIRE to Study Pediatric Chronic Pancreatitis

INSPPIRE 研究小儿慢性胰腺炎

• 批准号: 9045340
• 负责人: Aliye Uc
• 金额: $ 48.01万
• 依托单位: UNIVERSITY OF IOWA
• 依托单位国家: 美国
• 财政年份: 2015
• 资助国家: 美国
• 起止时间: 2015-09-28 至 2020-08-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/9045340
• 关键词: Abdominal Pain; Acute; Address; Adult; Anxiety; Calcium-Sensing Receptors; Carboxypeptidase; Characteristics; Child; Childhood; Clinical; Cognitive Therapy; Control Groups; Cystic Fibrosis; DNA Sequence Alteration; Data; Development; Diabetes Mellitus; Diagnostic; Disease; Disease Progression; Emotional; Endocrine; Enrollment; Environmental Risk Factor; Epidemiology; Exocrine pancreatic insufficiency; Foundations; Frequencies; Functional disorder; Gene Mutation; Genes; Genotype; Goals; Growth; High Prevalence; Hospitalization; Hybrids; Impaired health; Incidence; Interdisciplinary Study; International; Internet; Knowledge; Laboratories; Manuscripts; Measures; Mental Depression; Modality; Natural History; Nutritional; Onset of illness; Outcome; Pain; Pain management; Pancreas; Pancreatitis; Phenotype; Population; Problem behavior; Procedures; Prognostic Factor; Randomized Controlled Trials; Recurrence; Registries; Research Personnel; Role; SPINK1 gene; Serine Protease; Serine Proteinase Inhibitors; Severity of illness; Site; Testing; Trypsinogen; United States National Institutes of Health; Work; acute pancreatitis; base; behavioral health; burden of illness; chronic pain; chronic pancreatitis; chymotrypsin C; clinical practice; clinically relevant; cohort; data registry; early onset; genetic risk factor; health care service utilization; health related quality of life; improved; innovation; pediatric patients; programs; prospective; public health relevance; repository; socioeconomics

 DESCRIPTION (provided by applicant): Acute pancreatitis (AP) is increasingly recognized in children, with an incidence approaching that of adults. Most children with pancreatitis have a single, mild, acute episode that resolves without complications. However, a subset of children with AP develops recurrent episodes (defined as acute recurrent pancreatitis or ARP) and some progress to chronic pancreatitis (CP). Few studies have been performed to characterize ARP and CP in children. We recognized that the lack of a well-phenotyped pediatric cohort presented a major obstacle in understanding the pathophysiology of CP and its sequelae. To address this knowledge gap, we have formed INSPPIRE (INternational Study Group of Pediatric Pancreatitis: In search for a cuRE), composed of 18 participating sites. Results from 301 pediatric patients enrolled into our study revealed a high frequency of pancreatitis-associated gene mutations and substantial disease burden in children with CP. The objective of this application is to determine the natural history of pediatric CP, identify risk factors and genetic modifiers for its onset and sequelae and develop approaches to improve clinical outcomes. The overall hypothesis of this proposal is that pediatric CP has unique characteristics that determine disease onset and sequelae. Our specific aims are: 1) Characterize pediatric CP, determine predictors of disease severity and progression from ARP to CP; 2) Define the role of genetic mutations in early onset CP and identify disease modifiers; 3) Determine the efficacy of early pain management using cognitive-behavioral intervention on pediatric CP. Our long-term goal is to develop diagnostic modalities, prognostic factors and innovative treatment approaches for pediatric ARP and CP.

 描述（由适用提供）：急性胰腺炎（AP）在儿童中越来越认识到成人的事件方法。大多数患有胰腺炎的儿童都有一个单一的，轻度的急性发作，可以解决无关。但是，一部分患有AP发育的儿童复发发作（定义为急性复发性胰腺炎或ARP），以及慢性胰腺炎（CP）的一些进展。很少进行研究以表征儿童的ARP和CP。我们认识到，缺乏良好的小儿队列构成了理解CP及其后遗症病理生理学的主要障碍。为了解决这一知识差距，我们成立了Insppire（小儿胰腺炎国际研究小组：寻找治疗方法），由18个参与地点组成。参加我们研究的301名小儿患者的结果显示，CP儿童中胰腺炎相关的基因突变和实质性疾病伯嫩的频率很高。该应用的目的是确定小儿CP的自然史，确定其发作和后遗症的危险因素和遗传修饰符，并开发改善临床结果的方法。该提议的总体假设是小儿CP具有决定疾病发作和后遗症的独特特征。我们的具体目的是：1）描述儿科CP，确定疾病严重程度的预测因素以及从ARP到CP的进展； 2）定义遗传突变在早期发作中的作用并鉴定疾病修饰剂； 3）使用小儿CP的认知行为干预来确定早期疼痛管理的效率。我们的长期目标是开发小儿ARP和CP的诊断方式，预后因素和创新治疗方法。