Mohnike K, Blankenstein O, Minn H, Mohnike W, Fuchtner F, Otonkoski T
Department of Pediatrics, Otto-von-Guericke University Magdeburg, Magdeburg, Germany.
Horm Res. 2008;70(2):65-72. doi: 10.1159/000137655. Epub 2008 Jun 12.
In recent years, considerable progress has been made in the biochemical, morphological and molecular genetic differentiation of congenital hyperinsulinism (CHI). Fluorine-18 L-3,4-dihydroxyphenylalanine positron emission tomography ((18)F-DOPA-PET) has been introduced for differentiation between focal and diffuse CHI. The ability to take up L-DOPA and convert it into dopamine is correlated with the activity of the aromatic amino acid decarboxylase and increased in the hyperfunctional affected pancreatic area in comparison to normally functioning pancreas. The high sensitivity of this method allows the surgeon to perform a curative limited resection of a focus without the risk of long-term diabetes. The exact preoperative planning by (18)F-DOPA-PET/CT computer tomography allows laparoscopic operation in selected cases with the focus in the tail and limits necessity to open the pancreatic duct in cases with focus in the head. Patients with persistent CHI should be managed within a strong network of diagnostic, treatment, and research institutions.
近年来，先天性高胰岛素血症（CHI）在生化、形态学及分子遗传学鉴别方面取得了显著进展。氟-18 L-3,4-二羟基苯丙氨酸正电子发射断层扫描（(18)F-DOPA-PET）已被用于鉴别局灶性和弥漫性CHI。摄取L-多巴并将其转化为多巴胺的能力与芳香族氨基酸脱羧酶的活性相关，与正常功能的胰腺相比，功能亢进的受累胰腺区域该能力增强。该方法的高灵敏度使外科医生能够对病灶进行根治性局限性切除，而无长期糖尿病风险。通过(18)F-DOPA-PET/CT计算机断层扫描进行精确的术前规划，对于病灶位于胰尾的部分病例可进行腹腔镜手术，并减少了病灶位于胰头时打开胰管的必要性。持续性CHI患者应在强大的诊断、治疗和研究机构网络中接受管理。
