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Research progress of 18F-L-DOPA PET/CT in the diagnosis of congenital hyperinsulinism

  • Department of Nuclear Medicine, Beijing Friendship Hospital Affiliated to Capital Medical University, Beijing 100050, China

  • Congenital hyperinsulinism (CHI) is a condition leading to recurrent hypoglycemia due to an inappropriate insulin secretion by the pancreatic islet β cells. There are two main histologic subtypes: diffuse and focal, which may require different treatment strategies. Patients with diffuse CHI disease require a neartotal pancreatectomy. However patients with focal CHI disease only require a limited pancreatectomy to remove only the focal lesion thus providing complete cure to the patient. Hence the preoperative diagnosis of the histological subtypes of CHI becomes paramountin during the management of CHI. There was no advantages of morphological imaging methods, such as CT and MRI, in the differential diagnosis of focal and diffuse CHI. The CHI patient should perform 18Fluoride-L-dihydroxyphenylalanine(18F-L-DOPA) PET scan to differentiate focal from diffuse lesion, which can provide guidance for operation.This paper reviewed the research progress of 18F-L-DOPA PET scan in the diagnosis of CHI in recent years.
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  • [1] Arnoux JB,Verkarre V,Samt-Martm C,et al.Congenital hyperin-sulinism:current trends in diagnosis and therapy[J].Orphanet J Rare Dis,2011,6:63.
    [2] Arnoux JB,de Lonlay P,Ribeiro MJ,et al.Congenital hyperin-sulinism[J].Early Hum Dev,2010,86(5):287-294.
    [3] Bellanné-Chantelot C,Saint-Martin C,Ribeiro M J,et al.ABCC8 and KCNJ11 molecular spectrum of 109 patients with diazoxide-unresponsive congenital hyperinsulinism[J].J Med Genet,2010,47(11):752-759.
    [4] Ismail D,Hussain K.Role of 18F-DOPA PET/CT imaging in con-genital hyperinsulinism[J].Rev Endocr Metab Disord,2010,11(3):165-169.
    [5] Hussain K,Seppänen M,Nänto-Salönen K,et al.The diagnosis ofectopic focal hyperinsulinism of infancy with[18F]-dopa positronemission tomography[J].J Clin Endocrinol Metab,2006,91(8):2839-2842.
    [6] Peranteau W H,Bathaii SM,Pawel B,et al.Multiple ectopic lesions of focal islet adenomatosis identified by positron emission tomogra-hy scan in an infant with congenital hyperinsulinism[J].J Pediatr Surg,2007,42(1): 188-192.
    [7] Otonkoski T,Näntö-Salonen K,Seppänen M,et al.Noninvasive di-gnosis of focal hyperinsulinism of infancy with[18F]-DOPA positron emission tomography[J].Diabetes,2006,55(1): 13-18.
    [8] de Lonlay P,Simon-Carre A,Ribeiro M J,et al.Congenital hyper-nsulinism: pancreatic[18F]fluoro-L-dihydroxyphenylalanine(DOPA) positron emission tomography and immunohistochemistry study of DOPA decarboxylase and insulin secretion[J].J Clin Endocrinol Metab,2006,91(3): 933-940.
    [9] Meintjes M,Endozo R,Dickson J,et al.18F-DOPA PET and en-anced CT imaging for congenital hyperinsulinism: initial UK ex-erience from a technologist's perspective[J].Nucl Med Commun,2013,34(6):601-608.
    [10] Koopmans KP,Neels ON,Kema IP,et al.Molecular imaging in neuroendocrine tumors: molecular uptake mechanisms and clinical results[J].Crit Rev Oncol Hematol,2009,71(3): 199-213.
    [11] Jager PL,Chirakal R,Marriott CJ,et al.6-L-18F-fluorodihydrox-yphenylalanine PET in neuroendocrine tumors: basic aspects and emerging clinical applications[J].J Nucl Med,2008,49(4): 573-586.
    [12] Ribeiro MJ,De Lonlay P,Delzescaux T,et al.Characterization of hyperinsulinism in infancy assessed with PET and 18F-fluoro-L-DOPA[J].J Nucl Med,2005,46(4): 560-566.
    [13] Mohnike K,Blankenstein O,Minn H,et al.[18F]-DOPA positron emission tomography for preoperative localization in congenital hyperinsulinism[J].Horm Res,2008,70(2): 65-72.
    [14] 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 Pedi-atr Surg,2013,48(2): 388-393.
    [15] Capito C,Khen-Dunlop N,Ribeiro MJ,et al.Value of 18F-fluoro-L-dopa PET in the preoperative localization of focal lesions in con-enital hyperinsulinism[J].Radiology,2009,253(1): 216-222.
    [16] Zani A,Nah SA,Ron O,et al.The predictive value of preoperative fluorine-18-L-3,4-dihydroxyphenylalanine positron emission to-ography-computed tomography scans in children with congenital hyperinsulinism of infancy[J].J Pediatr Surg,2011,46(1): 204-208.
    [17] Masue M,Nishibori H,Fukuyama S,et al.Diagnostic accuracy of [18F]-fluoro-L-dihydroxyphenylalanine positron emission tomogra-hy scan for persistent congenital hyperinsulinism in Japan[J].Clin Endocrinol(Oxf),2011,75(3): 342-346.
    [18] Yang J,Hao R,Zhu X.Diagnostic role of 18F-dihydroxyphenylala-nine positron emission tomography in patients with congenital hy-perinsulinism: a meta-analysis[J].Nucl Med Commun,2013,34(4): 347-353.
    [19] Yang J,Yuan L,Meeks J K,et al.18F-DOPA positron emission to mography/computed tomography application in congenital hyperin-sulinism[J].J Pediatr Endocrinol Metab,2012,25(7-8): 619-622.
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Research progress of 18F-L-DOPA PET/CT in the diagnosis of congenital hyperinsulinism

    Corresponding author: Yang Jigang, email:nmyangjigang@gmail.com
  • Department of Nuclear Medicine, Beijing Friendship Hospital Affiliated to Capital Medical University, Beijing 100050, China
  • Received Date: 2014-11-12

Abstract: Congenital hyperinsulinism (CHI) is a condition leading to recurrent hypoglycemia due to an inappropriate insulin secretion by the pancreatic islet β cells. There are two main histologic subtypes: diffuse and focal, which may require different treatment strategies. Patients with diffuse CHI disease require a neartotal pancreatectomy. However patients with focal CHI disease only require a limited pancreatectomy to remove only the focal lesion thus providing complete cure to the patient. Hence the preoperative diagnosis of the histological subtypes of CHI becomes paramountin during the management of CHI. There was no advantages of morphological imaging methods, such as CT and MRI, in the differential diagnosis of focal and diffuse CHI. The CHI patient should perform 18Fluoride-L-dihydroxyphenylalanine(18F-L-DOPA) PET scan to differentiate focal from diffuse lesion, which can provide guidance for operation.This paper reviewed the research progress of 18F-L-DOPA PET scan in the diagnosis of CHI in recent years.

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