Retrospective analysis of 23 Chinese children with congenital hyperinsulinism undergoing pancreatectomy
Abstract
Introduction: The aim of the study was to discuss therapeutic effect and prognosis of pancreatectomy in the treatment of congenital hyperinsulinism (CHI).
Material and methods: A total of 23 Chinese children with CHI, who had undergone pancreatectomy, 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 analysed 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 hypoglycaemia. Three cases were diagnosed as diffuse-type CHI via preoperative scanning, two of which were treated by subtotal pancreatectomy. 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 hypoglycaemia.
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.
Keywords: congenital hyperinsulinismhypoglycaemiapancreatectomy18F-DOPA PET/CT scan
References
- 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.
- 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.
- Lord K, De León DD. Monogenic hyperinsulinemic hypoglycemia: current insights into the pathogenesis and management. Int J Pediatr Endocrinol. 2013; 2013(1): 3.
- Galcheva S, Al-Khawaga S, Hussain K. Diagnosis and management of hyperinsulinaemic hypoglycaemia. Best Pract Res Clin Endocrinol Metab. 2018; 32(4): 551–573.
