Liang Qifeng,Wen Zhe,Liang Jiankun,et al.The surgical treatment of congenital hyperinsulinism[J].Journal of Clinical Pediatric Surgery,,20():612-618.[doi:10.12260/lcxewkzz.2021.07.003]
The surgical treatment of congenital hyperinsulinism
- Keywords:
- Congenital Hyperinsulinism/SU; Treatment Outcome
- CLC:
- R725.76;R726.12
- Abstract:
- Objective The purpose of the study is to discuss the strategy,effectiveness and safety of surgical treatment for CHI patients by using the clinical data in our hospital retrospectively.Methods From December 2012 to December 2017,a total of 9 patients with CHI underwent partial or subtotal pancreatectomy in Guangzhou Women and Chilren’s Medical Center,including 6 males and 3 females.Preoperative ultrasonography,CT and MR examinations were performed on all of them,and the age of operation ranged from 32 days to 163 days.Results 1 patient was considered as focal CHI by preoperative 18F-DOPA PET-CT examination,but was confirmed as atypical CHI by intraoperative frozen pathology.1 case of focal CHI,7 cases of diffuse CHI and 1 case of atypical CHI were confirmed by postoperative paraffin pathology.After partial pancreatectomy for atypical type,the patient only needs regular intake of juice and starch to maintain normal blood glucose except for three meals now.The patient with focal CHI underwent 95% subtotal pancreatectomy,and he needed no drugs at discharge with his blood glucose fluctuating between 4.2 and 7.2mmol/L.At present,the patient has a normal diet and maintains normal blood glucose.All 7 diffuse CHI patients underwent subtotal pancreatectomy.1 patient who underwent 90% pancreas resection still had intermittent hypoglycemia at discharge and needed oral diazoxide to control her blood glucose.She developed type 1 diabetes 2 years after surgery and needs oral drugs to lower her blood glucose now.At discharge,1 patient who underwent 95% pancreas resection had occasional hypoglycemia before milk drinking or at night,requiring additional sugar water feedings to control her blood glucose.She developed type 1 diabetes in the third postoperative year and needs an insulin pump to lower blood glucose until now.The remaining 5 patients underwent 95% pancreatectomy and were fed normally at discharge without episodes of hypoglycemia.Their blood glucose fluctuated between 3.9 and 10mmol/L without drug treatment.At present,all the 5 patients have normal diet and no hypoglycemic episode or diabetes.Conclusion In our study,patients with CHI who have failed to respond to medication such as diazoxide may be treated surgically.The diagnostic value of 18F-DOPA PET-CT in atypical CHI needs to be further explored.Patients with diffuse CHI can effectively control blood glucose by subtotal pancreatectomy,and some of them can obtain normal blood glucose,but some of them will develop into type 1 diabetes,especially those who still have hypoglycemic episodes in the short term after the surgery have a higher possibility of developing diabetes.A 95% subtotal pancreatectomy is appropriate for most patients with diffuse CHI.Preoperative PET-CT is of great significance for identifying focal CHI.Preoperative PET-CT and intraoperative frozen pathology examination can confirm the classification of CHI and guide the surgical procedure.
References:
1 Shah P,Demirbilek h,Hussain k.Persistent hyperinsulinaemic hypoglycaemia in infancy[J].Semin Pediatr Surg,2014,23(2):76-82.DOI:10.1053/j.sempedsurg.2014.03.005.
2 Arnoux JB,Verkarre V,Saint-Martin C.Congenital hyperinsulinism:current trends in diagnosis and therapy[J].Orphanet J Rare Dis,2011,6:63-77.DOI:10.1186/1750-1172-6-63.
3 Yorifuji T,Horikawa R,Hasegawa T,et al.Clinical practice guidelines for congenital hyperinsulinism[J].Clin Pediatr Endocrinol,2017,26(3):127-152.DOI:10.1297/cpe.26.127.
4 Lovvorn HN 3rd,Nance ML,Ferry RJ Jr,et al.Congenital hyperinsulinism and the surgeon lessons learned over 35 years[J].J Pediatr Surg,1999,34(5):786-793.DOI:10.1016/s0022-3468(99)90374-3.
5 Scott Adzick N.Surgical treatment of congenital hyperinsulinism[J].Semin Pediatr Surg,2020,29(3):150924.DOI:10.1016/j.sempedsurg.2020.150924.
6 Laimon W,Aboelenin HM,El Tantawi NT.Clinical characteristics,outcome,and predictors of neurological sequelae of persistent congenital hyperinsulinism:A single tertiary center experience[J].Pediatr Diabetes,2021,22(3):388-399.DOI:10.1111/pedi.13186.
7 Kapoor RR,Flanagan SE,Arya VB,et al.Clinical and molecular characterisation of 300 patients with congenital hyperinsulinism[J].Eur J Endocrinol,2013,168(4):557-564.DOI:10.1530/EJE-12-0673.
8 Snider KE,Becker S,Boyajian L,et al.Genotype and phenotype correlations in 417 CHIldren with congenital hyperinsulinism[J].J Clin Endocrinol Metab,2013,98(2):E355-E363.DOI:10.1210/jc.2012-2169.
9 Flanagan SE,Kapoor RR,Hussain K.Genetics of congenital hyperinsulinemic hypoglycemia[J].Semin Pediatr Surg,2011,20(1):13-17.DOI:10.1053/j.sempedsurg.2010.10.004.
10 Verkarre V,Fournet JC,de Lonlay P,et al.Paternal mutation of the sulfonylurea receptor (SUR1) gene and maternal loss of 11p15 imprinted genes lead to persistent hyperinsulinism in focal adenomatous hyperplasia[J].J Clin Invest,1998,102(7):1286-1291.DOI:10.1172/JCI4495.
11 Suchi M,MacMullen CM,Thornton PS,et al.Molecular and immunohistochemical analyses of the focal form of congenital hyperinsulinism[J].Mod Pathol,2006,19(1):122-129.DOI:10.1038/modpathol.3800497.
12 Rahier J,Guiot Y,Sempoux C.Morphologic analysis of focal and diffuse forms of congenital hyperinsulinism[J].Semin Pediatr Surg,2011,20(1):3-12.DOI:10.1053/j.sempedsurg.2010.10.010.
13 Adzick NS,De Leon DD,States LJ,et al.Surgical treatment of congenital hyperinsulinism:Results from 500 pancreatectomies in neonates and CHIldren[J].J Pediatr Surg,2019,54(1):27-32.DOI:10.1016/j.jpedsurg.2018.10.030.
14 Laje P,Palladino AA,Bhatti TR,et al.Pancreatic surgery in infants with Beckwith-Wiedemann syndrome and hyperinsulinism[J].J Pediatr Surg,2013,48(12):2511-2516.DOI:10.1016/j.jpedsurg.2013.05.016.
15 Kalish JM,Boodhansingh KE,Bhatti TR,et al.Congenital hyperinsulinism in CHIldren with paternal 11p uniparental isodisomy and Beckwith-Wiedemann syndrome[J].J Med Genet,2016,53(1):53-61.DOI:10.1136/jmedgenet-2015-103394.
16 Arya VB,Guemes M,Nessa A,et al.Clinical and histological heterogeneity of congenital hyperinsulinism due to paternally inherited heterozygous ABCC8/KCNJ11 mutations[J].Eur J Endocrinol,2014,171(6):685-695.DOI:10.1530/EJE-14-0353.
17 Zhang W,Liu L,Wen Z,et al.A compound heterozygous mutation of ABCC8 gene causing a diazoxide-unresponsive congenital hyperinsulinism with an atypical form:Not a focal lesion in the pancreas reported by (1)(8) F-DOPA-PET/CT scan[J].Gene,2015,572(2):222-226.DOI:10.1016/j.gene.2015.07.012.
18 Lord K,Dzata E,Snider KE,et al.Clinical presentation and management of CHIldren with diffuse and focal hyperinsulinism:a review of 223 cases[J].J Clin Endocrinol Metab,2013,98(11):E1786-E1789.DOI:10.1210/jc.2013-2094.
19 Brunelle F,Negre V,Barth MO,et al.Pancreatic venous samplings in infants and CHIldren with primary hyperinsulinism[J].Pediatr Radiol,1989,19(2):100-103.DOI:10.1007/bf02387895.
20 Ferry RJ Jr,Kelly A,Grimberg A,et al.Calcium-stimulated insulin secretion in diffuse and focal forms of congenital hyperinsulinism[J].J Pediatr,2000,137(2):239-246.DOI:10.1067/mpd.2000.107386.
21 任静芸,杨吉刚.18F-L-DOPA PET/CT显像诊断先天性高胰岛素血症的研究进展[J].国际放射医学核医学杂志,2015,39(1):67-70.DOI:10.3760/cma.j.issn.1673-4114.2015.01.014. Ren JY,Yang JG.Research progress of 18F-L-DOPA PET/CT in the diagnosis of congenital hyperinsulinism[J].J Radiat Med Nucl Med,2015,39(1):67-70.DOI:10.3760/cma.j.issn.1673-4114.2015.01.014.
22 Laje P,States LJ,Zhuang H,et al.Accuracy of PET/CT Scan in the diagnosis of the focal form of congenital hyperinsulinism[J].J Pediatr Surg,2013,48(2):388-393.DOI:10.1016/j.jpedsurg.2012.11.025.
23 Christiansen CD,Petersen H,Nielsen AL,et al.18F-DOPA PET/CT and 68Ga-DOTANOC PET/CT scans as diagnostic tools in focal congenital hyperinsulinism:a blinded evaluation[J].Eur J Nucl Med Mol Imaging,2018,45(2):250-261.DOI:10.1007/s00259-017-3867-1.
24 王乐,沈丽萍.运用达芬奇手术机器人辅助系统治疗小儿胰腺肿瘤[J].临床小儿外科杂志,2018,17(5):363-366.DOI:10.3969/j.issn.1671-6353.2018.05.011. Wang L,Shen LP.Treatment of pediatric pancreatic tumors by robotic surgical system[J].J Clin Ped Sur,2018,17(5):363-366.DOI:10.3969/j.issn.1671-6353.2018.05.011.
25 Pierro A,Nah SA.Surgical management of congenital hyperinsulinism of infancy[J].Semin Pediatr Surg,2011,20(1):50-53.DOI:10.1053/j.sempedsurg.2010.10.009.
26 Beltrand J,Caquard M,Arnoux JB,et al.Glucose metabolism in 105 CHIldren and adolescents after pancreatectomy for congenital hyperinsulinism[J].Diabetes Care,2012,35(2):198-203.DOI:10.2337/dc11-1296.
27 Lord K,Radcliffe J,Gallagher PR,et al.High Risk of Diabetes and Neurobehavioral Deficits in Individuals With Surgically Treated Hyperinsulinism[J].J Clin Endocrinol Metab,2015,100(11):4133-4139.DOI:10.1210/jc.2015-2539.
28 Arya VB,Senniappan S,Demirbilek H,et al.Pancreatic endocrine and exocrine function in children following near-total pancreatectomy for diffuse congenital hyperinsulinism[J].PLoS One,2014,9(5):e98054.DOI:10.1371/journal.pone.0098054.
Memo
收稿日期:2021-04-24。
基金项目:广州市科技局基础与应用基础研究项目（编号：202002030465）
通讯作者:温哲,Email:wenzhe2005@163.con