Clinical and Molecular Genetics Unit, The Developmental Endocrinology Research Group, Institute of Child Health, University College London, Great Ormond Street Hospital for Children NHS Trust, 30 Guilford Street, London, WC1N 1EH, UK.
Rev Endocr Metab Disord. 2010 Sep;11(3):165-9. doi: 10.1007/s11154-010-9145-1.
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 ((18)F-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.
先天性高胰岛素血症是新生儿期严重低血糖的主要原因。先天性高胰岛素血症有两种（弥漫性和局灶性）组织学亚型。弥漫性病变累及整个胰腺，如果药物治疗无效，需要进行近全（95%-98%）胰腺切除术。局灶性病变仅影响胰腺的一小部分区域（其余胰腺在内分泌和外分泌功能方面正常），仅需要进行有限的胰腺切除术。局灶性病变的这一有限部分有治愈患者的潜力。因此，这两种亚组的术前区分极为重要。氟代-18-左旋-3,4-二羟基苯丙氨酸正电子发射断层扫描（(18)F-DOPA PET/CT）的最新进展彻底改变了先天性高胰岛素血症患者的临床处理方式。在大多数患者中，这种新型成像技术能够精确地对局灶性病变进行术前定位，从而指导手术切除的范围。
