Long-term disease progression in pediatric acute recurrent and chronic pancreatitis: A report from INSPPIRE

IF 3.9 2区医学 Q2 GASTROENTEROLOGY & HEPATOLOGY

Pancreatology Pub Date : 2026-06-01 Epub Date: 2026-02-02 DOI:10.1016/j.pan.2026.01.076

Elissa M. Downs , Emily R. Perito , Fuchenchu Wang , Gretchen A. Cress , Maisam Abu-El-Haija , Ankur Chugh , Reuven Zev Cohen , Douglas S. Fishman , A. Jay Freeman , Cheryl E. Gariepy , Matthew J. Giefer , Tanja Y. Gonska , Amit S. Grover , Sohail Z. Husain , Douglas Lindblad , Quin Y. Liu , Asim Maqbool , Jacob A. Mark , Brian A. McFerron , Megha S. Mehta , Aliye Uc

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引用次数: 0

Abstract

Background

Acute recurrent pancreatitis (ARP) in childhood can rapidly progress to chronic pancreatitis (CP). Prospectively-collected data from the INternational Study group of Pediatric Pancreatitis: In search for a cuRE (INSPPIRE) provides novel insight into disease progression.

Methods

INSPPIRE subjects were categorized as persistent ARP (pARP = remained ARP through last follow-up), incident CP (iCP = ARP at enrollment, developed CP), or prevalent CP (pCP = CP at enrollment). Time-to-sequelae and risk factors were analyzed.

Results

Of 626 total children, 384 (61%) were ARP at baseline; of these, 81 (21.1%) were iCP at follow-up.

iCP were more likely to have PRSS1 mutations, obstructive risk factors, and more acute pancreatitis episodes (AP) vs. pARP, but didn't differ in age at first AP.

Exocrine pancreatic insufficiency (EPI) developed in 24% during follow-up, 10% of pARP, 32% of iCP. In all CP, 50% had EPI by 17.7yrs, median 10yrs after first AP.

Diabetes mellitus (DM) developed in 8% during follow-up, 6% of pARP, 5% of iCP. In all CP, median event-free survival from birth and after first AP was not reached.

Conclusions

Prospective follow-up of children with ARP revealed nearly 1 in 5 progressed to CP, with a subset developing irreversible sequelae, and highlighted risk factors associated with progression including genetics, obstructive disease, and episode frequency.

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儿童急性复发性和慢性胰腺炎的长期疾病进展：一份来自inspire的报告。

背景：儿童急性复发性胰腺炎（ARP）可迅速发展为慢性胰腺炎（CP）。来自儿科胰腺炎国际研究小组的前瞻性收集数据：在寻找治疗（inspire）提供了对疾病进展的新见解。方法：将受试者分为持续性ARP （pARP =最后一次随访时仍然存在的ARP）、偶发性CP （iCP =入组时ARP，发展性CP）和流行性CP （pCP =入组时CP）。分析了发病时间和危险因素。结果：626例患儿中，基线时有384例（61%）出现ARP；其中81例（21.1%）随访时为iCP。与pARP相比，iCP更有可能发生PRSS1突变、阻塞性危险因素和更多的急性胰腺炎发作（AP），但首次发生AP的年龄没有差异。在随访期间，外分泌性胰腺功能不全（EPI）的发生率为24%，pARP为10%，iCP为32%。在所有CP中，50%的患者在首次AP后的17.7年出现EPI，中位时间为10年。在随访期间，糖尿病（DM）发生率为8%，pARP为6%，iCP为5%。在所有CP中，从出生到首次AP后的中位无事件生存期均未达到。结论：ARP患儿的前瞻性随访显示，近1 / 5进展为CP，其中一个亚群发展为不可逆的后遗症，并强调了与进展相关的危险因素，包括遗传、阻塞性疾病和发作频率。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pancreatology 医学-胃肠肝病学

CiteScore

7.20

自引率

5.60%

发文量

194

审稿时长

44 days

期刊介绍： Pancreatology is the official journal of the International Association of Pancreatology (IAP), the European Pancreatic Club (EPC) and several national societies and study groups around the world. Dedicated to the understanding and treatment of exocrine as well as endocrine pancreatic disease, this multidisciplinary periodical publishes original basic, translational and clinical pancreatic research from a range of fields including gastroenterology, oncology, surgery, pharmacology, cellular and molecular biology as well as endocrinology, immunology and epidemiology. Readers can expect to gain new insights into pancreatic physiology and into the pathogenesis, diagnosis, therapeutic approaches and prognosis of pancreatic diseases. The journal features original articles, case reports, consensus guidelines and topical, cutting edge reviews, thus representing a source of valuable, novel information for clinical and basic researchers alike.