Role of 18F-DOPA PET/CT imaging in congenital hyperinsulinism

被引:0
作者
Dunia Ismail
Khalid Hussain
机构
[1] University College London and Great Ormond Street Hospital for Children NHS Trust,Clinical and Molecular Genetics Unit, The Developmental Endocrinology Research Group, Institute of Child Health
来源
Reviews in Endocrine and Metabolic Disorders | 2010年 / 11卷
关键词
Glucose; Hypoglycaemia; Dopamine; PET scan;
D O I
暂无
中图分类号
学科分类号
摘要
Congenital hyperinsulinism is a leading cause of severe hypoglycaemia in the newborn period. There are two (diffuse and focal) histological subtypes of congenital hyperinsulinism. The diffuse form affects the entire pancreas and if medically unresponsive will require a near total (95%–98%) pancreatectomy. The focal form affects only a small region of the pancreas (with the rest of the pancreas being normal in endocrine and exocrine function) and only requires a limited pancreatectomy. This limited section of the focal lesion has the potential for curing the patient. Thus the pre-operative differentiation of these two subgroups is extremely important. Recent advances in Fluorine-18-L-dihydroxyphenylalanine positron emission tomography (18F-DOPA PET/CT) have radically changed the clinical approach to patient with congenital hyperinsulinism. In most patients this novel imaging technique is able to offer precise pre-operative localisation of the focal lesion, thus guiding the extent of surgical resection.
引用
收藏
页码:165 / 169
页数:4
相关论文
共 227 条
[1]  
Aynsley-Green A(2000)The Practical Management of Hyperinsulinism in Infancy Arch Dis Child Fetal Neonatal Ed 82 F98-F107
[2]  
Hussain K(2009)Hyperinsulinaemic hypoglycaemia Arch Dis Child 94 450-457
[3]  
Hall J(2004)Medications used in the treatment of hypoglycemia due to congenital hyperinsulinism of infancy (HI) Pediatr Endocrinol Rev 2 163-167
[4]  
Saudubray JM(2006)Proposal for a standardized protocol for 18F-DOPA-PET (PET/CT) in congenital hyperinsulinism Horm Res 66 40-42
[5]  
Nihoul-Fékété C(2002)Persistent hyperinsulinaemic hypoglycaemia of infancy: a heterogeneous syndrome unrelated to nesidioblastosis Arch Dis Child Fetal Neonatal Ed 82 F108-F112
[6]  
De Lonlay-Debeney P(1996)Mutation of the pancreatic islet inward rectifier Kir6.2 also leads to familial persistent hyperinsulinemic hypoglycemia of infancy Hum Mol Genet 5 1809-1812
[7]  
Kapoor RR(1995)Mutations in the sulfonylurea receptor gene in familial persistent hyperinsulinemic hypoglycemia of infancy Science 268 426-429
[8]  
Flanagan SE(1997)A nonsense mutation in the inward rectifier potassium channel gene, Kir6.2, is associated with familial hyperinsulinism Diabetes 46 1743-1748
[9]  
James C(2000)Dominantly inherited hyperinsulinism caused by a mutation in the sulfonylurea receptor type 1 J Clin Invest 106 897-906
[10]  
Shield J(2008)Clinical characteristics and biochemical mechanisms of congenital hyperinsulinism associated with dominant KATP channel mutations J Clin Invest 118 2877-2886