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Last update 08 May 2025

Nesidioblastosis

Last update 08 May 2025

Basic Info

Synonyms

Familial hyperinsulinaemia with pancreatic nesidioblastosis, Familial hyperinsulinemia with pancreatic nesidioblastosis, HYPERINSULINISM, FAMILIAL, WITH PANCREATIC NESIDIOBLASTOSIS

+ [37]

Introduction

An inherited autosomal recessive syndrome characterized by the disorganized formation of new islets in the PANCREAS and CONGENITAL HYPERINSULINISM. It is due to focal hyperplasia of pancreatic ISLET CELLS budding off from the ductal structures and forming new islets of Langerhans. Mutations in the islet cells involve the potassium channel gene KCNJ11 or the ATP-binding cassette transporter gene ABCC8, both on CHROMOSOME 11.

Drugs associated with Nesidioblastosis

68Ga-NOTA-exendin-4

Target

GLP-1R

Mechanism

GLP-1R agonists

Active Org.

First Affiliated Hospital Of Fujian Medical University

Originator Org.-

Active Indication

Insulinoma

Inactive Indication

Nesidioblastosis

Drug Highest PhasePhase 1

First Approval Ctry. / Loc.-

First Approval Date20 Jan 1800

Clinical Trials associated with Nesidioblastosis

JPRN-jRCTs041220114

/ Not yet recruitingPhase 2IIT

The single-center phase II study for the efficacy of proton pump inhibitor for postoperative delirium in elderly patients undergoing hepatobiliary and pancreatic surgery

Start Date13 Jan 2023

Sponsor / Collaborator-

ChiCTR2000036342

/ SuspendedPhase 2IIT

A prospective, single center clinical study of denosumab in the treatment of benign osteolytic tumors in adolescents

Start Date01 Oct 2020

Sponsor / Collaborator

Shanghai Changzheng Hospital

NCT02560376

/ Unknown statusEarly Phase 1IIT

68Ga-NOTA-exendin-4 PET/CT for the Localization of Insulinoma and Diagnosis of Nesidioblastosis

This is an open-label positron emission tomography/computed tomography (PET/CT) study to investigate the diagnostic performance and evaluation efficacy of 68Ga-NOTA-exendin-4 in insulinoma and nesidioblastosis patients. A single dose of 55.5-111 Mega-Becquerel (MBq) 68Ga-NOTA-exendin-4 will be injected intravenously. Visual and semiquantitative method will be used to assess the PET/CT images.

Start Date01 Feb 2014

Sponsor / Collaborator

Peking Union Medical College Hospital

[+1]

100 Clinical Results associated with Nesidioblastosis

100 Translational Medicine associated with Nesidioblastosis

0 Patents (Medical) associated with Nesidioblastosis

1,251

Literatures (Medical) associated with Nesidioblastosis

01 Dec 2025·Journal of Gastrointestinal Cancer

Initial Application of Fluorescence Imaging for Intraoperative Localization of Small Neuroendocrine Tumors in the Pancreas: Case Report and Review of the Literature

Review

Author: Fang, Xiaosan ; Qian, Daohai ; Shen, Zhengchao ; Zheng, Xingyuan ; Xi, Shihang ; Muhammad, Danish Irshad ; Wang, Xiaoming ; Wang, Guannan ; Wang, Xu ; Jiang, Bin ; Jiang, Yaqi

BACKGROUNDIndocyanine green is commonly used for laparoscopic hepatectomy but remains uncommon in pancreatic surgery. Given the increasing number of small neuroendocrine tumors found in the pancreas and the heavy reliance on laparoscopic ultrasound for intraoperative localization, we attempted to use indocyanine green for these tumors. Our results show good localization and have the potential to provide a valuable clinical aid.CASE PRESENTATIONThis case report details five patients with preoperative diagnosis of pancreatic neuroendocrine tumors of small endocrine tumors, intraoperative successful localization, and successful completion of laparoscopic partial resection of pancreatic tumors by indocyanine green fluorescence staining; none of the patients experienced serious complications after surgery and were discharged from the hospital, and routine pathology confirmed that four cases were pancreatic neuroendocrine tumors of G1 stage, and one case was pancreatic neuroendocrine cell hyperplasia.CONCLUSIONFluorescence imaging technology safely aids in the intraoperative localization of small pancreatic neuroendocrine tumors.

01 Apr 2025·Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease

Pathogenesis and therapeutic effect of sitagliptin in experimental diabetic model of COVID-19

Article

Author: Lawal, Akinkunmi ; Meng, Qinghe ; Wang, Chunyan ; Ma, Julia ; Suo, Liye ; Cooney, Robert N ; Wang, Guirong ; Jacob, Ikechukwu ; Zhao, Wenlu ; Song, Yuqi

This study evaluates the pathogenesis of COVID-19 and the therapeutic efficacy of sitagliptin in diabetic and obese mice. Using a novel double-transgenic mouse model (db/db and K18-hACE2), the findings demonstrates that SARS-CoV-2 infection (Delta variant) causes severe multi-organ damage, glucose metabolism abnormalities, insulin resistance, and pancreatic islet cell damage in diabetic mice. Infected diabetic mice displayed higher mortality, inflammation (elevated TNF-α, IL-6, IL-1β), and fibrinolytic activity (PAI-1), alongside dysregulated diabetes-related hormones (GLP-1, leptin, ghrelin, resistin) compared to non-diabetic controls. Sitagliptin treatment reduced organ injury, hyperglycemia, inflammation, and fibrinolytic activity while improving insulin resistance and glucose metabolism. This was evidenced by decreased fasting blood glucose levels, improved insulin sensitivity, and elevated insulin and GLP-1 levels. These findings suggest sitagliptin is a promising therapeutic strategy to mitigate the severity of COVID-19 in experimental diabetes by modulating inflammation and improving metabolic syndrome. Further mechanistic investigations revealed that the level of hACE2 expression, along with the activation of NF-κB and IRS-1, play critical roles in the development of SARS-CoV-2-induced diabetes, the exacerbation of pre-existing diabetes, and the therapeutic efficacy of sitagliptin.

01 Mar 2025·Diabetologia

Diet-enhanced LRG1 expression promotes insulin hypersecretion and ER stress in pancreatic beta cells

Article

Author: Smith, Maia R ; Lynch, Jane L ; Wei, Cong ; Bai, Juli ; Hadley, Jason T ; Dong, Lily Q ; Mummidi, Srinivas ; Lopez-Alvarenga, Juan C ; Morales, Desirae D ; Duggirala, Ravindranath ; Ryu, Jiyoon ; Liu, Feng

AIMS/HYPOTHESISUpregulation of serum leucine-rich α-2-glycoprotein 1 (LRG1) has been implicated in diet-induced obesity and metabolic disorders. However, its specific hormonal actions remain unclear. This study aimed to determine whether diet-enhanced serum LRG1 levels promote hyperinsulinaemia by directly stimulating insulin secretion from pancreatic beta cells.METHODSHuman serum samples were obtained from individuals (both male and female) undergoing plastic surgery. Male C57BL/6 wild-type and Lrg1 whole-body knockout (Lrg1^KO) mice were fed a 45% high-fat diet, with serum samples collected every 2 weeks to monitor LRG1 and insulin levels throughout diet-induced obesity. MIN6 beta cells were used to investigate the effects of LRG1 on insulin secretion and intracellular Ca²⁺ release. Antibodies targeting various LRG1 epitopes were used to neutralise LRG1 stimulation in MIN6 cells, and their effectiveness was tested in vivo to assess their ability to prevent LRG1-induced hyperinsulinaemia.RESULTSWe observed a significant positive association between human serum LRG1 levels and both age and BMI, with elevated levels observed in individuals with vs without type 2 diabetes. In mice fed a high-fat diet, LRG1 upregulation in serum was associated with hyperinsulinaemia. Lrg1 knockout protected mice from diet-induced islet hyperplasia and the loss of beta cell mass. Furthermore, neutralising LRG1 activity prevented the onset of diet-induced hyperinsulinaemia and preserved glucose tolerance. Mechanistically, LRG1 induces inositol triphosphate (IP₃) production and intracellular Ca²⁺ release from the endoplasmic reticulum (ER) in a phospholipase C (PLC)-dependent manner, leading to excessive insulin secretion and ER stress in MIN6 beta cells.CONCLUSIONS/INTERPRETATIONIn summary, this study identifies LRG1 as a significant contributor to hyperinsulinaemia and beta cell dysfunction. Targeting LRG1 activity emerges as a promising therapeutic approach for addressing diet-induced beta cell dysfunction and managing type 2 diabetes.

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Nesidioblastosis

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