open access

Ahead of print
Brief communication
Published online: 2021-07-12
Submitted: 2021-05-24
Accepted: 2021-06-27
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Retrospective analysis of 23 Chinese children with congenital hyperinsulinemia undergoing pancreatectomy

Lin Zhang, Zi-Di Xu, Min Liu, Qiao Zeng, Rong-Min Li, Jie-Ying Wang, Huan-Min Wang, Long Li, Hong Qin, Jie Yan, Yu-Jun Wu, Cheng Zhu, Gui-Chen Ni, Yan-Mei Sang
DOI: 10.5603/EP.a2021.0066
·
Pubmed: 34292572

open access

Ahead of print
Short communication
Published online: 2021-07-12
Submitted: 2021-05-24
Accepted: 2021-06-27

Abstract

Objectives: The clinical data of 23 cases of congenital hyperinsulinemia (CHI) treated by pancreatectomy were retrospectively analyzed, and the therapeutic effect and prognosis of pancreatectomy in the treatment of CHI were discussed. Methods: A total of 23 Chinese children with CHI who had undergone pancreatectomy between February 2002 and March 2020 were selected as the study objects. The clinical data, the results of the 18Fluoro-L-3-4 dihydroxyphenylalanine positron emission tomography/computerized tomography (18F-DOPA PET/CT) scanning, and the diagnosis, treatment, and follow-up were analyzed retrospectively. Results: Among the 23 cases, 14 patients were diagnosed with focal-type CHI via a 18F-DOPA PET/CT scan prior to the operation, with the lesions removed via partial pancreatectomy. After the operation, ten patients (71%) had normal blood glucose levels, while frequent feeding was required in four patients (29%) to control the hypoglycemia. Three cases were diagnosed as diffuse-type CHI via preoperative scanning, two of which were treated by subtotal pancreatectomy, with one returning to normal blood glucose levels, while a subcutaneous injection of octreotide was required in the other to prevent hypoglycemia. The other case was treated by near-total pancreatectomy, and the blood glucose level was normal following the operation. The remaining six cases were not diagnosed via the pancreatic scanning prior to the operation due to the limitation of certain conditions. Here, pancreatectomy was performed directly due to severe hypoglycemia. Conclusions: 18F-DOPA PET/CT scanning was a reliable method for determining the histological type and localizing the lesion before the operation. Partial pancreatectomy for focal-type CHI had a high cure rate. In the children with diffuse-type CHI, the surgical methods were more complex and the therapeutic effect and the prognosis were also different.

Abstract

Objectives: The clinical data of 23 cases of congenital hyperinsulinemia (CHI) treated by pancreatectomy were retrospectively analyzed, and the therapeutic effect and prognosis of pancreatectomy in the treatment of CHI were discussed. Methods: A total of 23 Chinese children with CHI who had undergone pancreatectomy between February 2002 and March 2020 were selected as the study objects. The clinical data, the results of the 18Fluoro-L-3-4 dihydroxyphenylalanine positron emission tomography/computerized tomography (18F-DOPA PET/CT) scanning, and the diagnosis, treatment, and follow-up were analyzed retrospectively. Results: Among the 23 cases, 14 patients were diagnosed with focal-type CHI via a 18F-DOPA PET/CT scan prior to the operation, with the lesions removed via partial pancreatectomy. After the operation, ten patients (71%) had normal blood glucose levels, while frequent feeding was required in four patients (29%) to control the hypoglycemia. Three cases were diagnosed as diffuse-type CHI via preoperative scanning, two of which were treated by subtotal pancreatectomy, with one returning to normal blood glucose levels, while a subcutaneous injection of octreotide was required in the other to prevent hypoglycemia. The other case was treated by near-total pancreatectomy, and the blood glucose level was normal following the operation. The remaining six cases were not diagnosed via the pancreatic scanning prior to the operation due to the limitation of certain conditions. Here, pancreatectomy was performed directly due to severe hypoglycemia. Conclusions: 18F-DOPA PET/CT scanning was a reliable method for determining the histological type and localizing the lesion before the operation. Partial pancreatectomy for focal-type CHI had a high cure rate. In the children with diffuse-type CHI, the surgical methods were more complex and the therapeutic effect and the prognosis were also different.

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Keywords

congenital hyperinsulinemia; hypoglycemia; pancreatectomy; 18F-DOPA PET/CT scan

About this article
Title

Retrospective analysis of 23 Chinese children with congenital hyperinsulinemia undergoing pancreatectomy

Journal

Endokrynologia Polska

Issue

Ahead of print

Article type

Brief communication

Published online

2021-07-12

DOI

10.5603/EP.a2021.0066

Pubmed

34292572

Keywords

congenital hyperinsulinemia
hypoglycemia
pancreatectomy
18F-DOPA PET/CT scan

Authors

Lin Zhang
Zi-Di Xu
Min Liu
Qiao Zeng
Rong-Min Li
Jie-Ying Wang
Huan-Min Wang
Long Li
Hong Qin
Jie Yan
Yu-Jun Wu
Cheng Zhu
Gui-Chen Ni
Yan-Mei Sang

References (5)
  1. Yorifuji T, Horikawa R, Hasegawa T, et al. (on behalf of The Japanese Society for Pediatric Endocrinology and The Japanese Society of Pediatric Surgeons). Clinical practice guidelines for congenital hyperinsulinism. Clin Pediatr Endocrinol. 2017; 26(3): 127–152.
  2. Ferrara C, Patel P, Becker S, et al. Biomarkers of Insulin for the Diagnosis of Hyperinsulinemic Hypoglycemia in Infants and Children. J Pediatr. 2016; 168: 212–219.
  3. Arnoux JB, Verkarre V, Saint-Martin C, et al. Congenital hyperinsulinism: current trends in diagnosis and therapy. Orphanet J Rare Dis. 2011; 6: 63.
  4. Lord K, De León DD. Monogenic hyperinsulinemic hypoglycemia: current insights into the pathogenesis and management. Int J Pediatr Endocrinol. 2013; 2013(1): 3.
  5. Galcheva S, Al-Khawaga S, Hussain K. Diagnosis and management of hyperinsulinaemic hypoglycaemia. Best Pract Res Clin Endocrinol Metab. 2018; 32(4): 551–573.

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