Hardy Olga T, Hernandez-Pampaloni Miguel, Saffer Janet R, Scheuermann Joshua S, Ernst Linda M, Freifelder Richard, Zhuang Hongming, MacMullen Courtney, Becker Susan, Adzick N Scott, Divgi Chaitanya, Alavi Abass, Stanley Charles A
Division of Endocrinology, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania 19104, USA.
J Clin Endocrinol Metab. 2007 Dec;92(12):4706-11. doi: 10.1210/jc.2007-1637. Epub 2007 Sep 25.
Focal lesions in infants with congenital hyperinsulinism (HI) represent areas of adenomatosis that express a paternally derived ATP-sensitive potassium channel mutation due to embryonic loss of heterozygosity for the maternal 11p region. This study evaluated the accuracy of 18F-fluoro-l-dihydroxyphenylalanine ([18F]DOPA) positron emission tomography (PET) scans in diagnosing focal vs. diffuse disease and identifying the location of focal lesions.
A total of 50 infants with HI unresponsive to medical therapy were studied. Patients were injected iv with [18F]DOPA, and PET scans were obtained for 50-60 min. Images were coregistered with abdominal computed tomography scans. PET scan interpretations were compared with histological diagnoses.
The diagnosis of focal or diffuse HI was correct in 44 of the 50 cases (88%). [18F]DOPA PET identified focal areas of high uptake of radiopharmaceutical in 18 of 24 patients with focal disease. The locations of these lesions matched the areas of increased [18F]DOPA uptake on the PET scans in all of the cases. PET scan correctly located five lesions that could not be visualized at surgery. The positive predictive value of [18F]DOPA in diagnosing focal adenomatosis was 100%, and the negative predictive value was 81%.
[18F]DOPA PET scans correctly diagnosed 75% of focal cases and were 100% accurate in identifying the location of the lesion. These results suggest that [18F]DOPA PET imaging provides a useful guide to surgical resection of focal adenomatosis and should be considered as a guide to surgery in all infants with congenital HI who have medically uncontrollable disease.
先天性高胰岛素血症（HI）婴儿的局灶性病变代表腺瘤病区域，由于母源11p区域杂合性的胚胎丢失，这些区域表达父源的ATP敏感性钾通道突变。本研究评估了18F-氟-L-二羟基苯丙氨酸（[18F]DOPA）正电子发射断层扫描（PET）在诊断局灶性与弥漫性疾病以及确定局灶性病变位置方面的准确性。
共研究了50例对药物治疗无反应的HI婴儿。给患者静脉注射[18F]DOPA，并进行50 - 60分钟的PET扫描。图像与腹部计算机断层扫描进行配准。将PET扫描结果与组织学诊断进行比较。
50例病例中有44例（88%）局灶性或弥漫性HI的诊断正确。[18F]DOPA PET在24例局灶性疾病患者中的18例中识别出放射性药物高摄取的局灶区域。在所有病例中，这些病变的位置与PET扫描上[18F]DOPA摄取增加的区域相匹配。PET扫描正确定位了5个在手术中无法看到的病变。[18F]DOPA诊断局灶性腺瘤病的阳性预测值为100%，阴性预测值为81%。
[18F]DOPA PET扫描正确诊断了75%的局灶性病例，在确定病变位置方面的准确率为100%。这些结果表明，[18F]DOPA PET成像为局灶性腺瘤病的手术切除提供了有用的指导，对于所有患有先天性HI且药物治疗无法控制病情的婴儿，应考虑将其作为手术指导。
