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Noninvasive Diagnosis of Focal Hyperinsulinism of Infancy With [¹⁸F]-DOPA Positron Emission Tomography

¹ Hospital for Children and Adolescents and the Program of Developmental and Reproductive Biology, Biomedicum, University of Helsinki, Helsinki, Finland
² Departments of Pediatrics and Radiology, Turku University Hospital, Turku, Finland
³ Turku PET Centre, University of Turku, Turku, Finland
⁴ Department of Pediatrics and Adolescence, Oulu University Hospital, Oulu, Finland
⁵ Departments of Pediatrics and Medicine, Kuopio University Hospital, Kuopio, Finland
⁶ London Centre for Paediatric Endocrinology and Metabolism, Great Ormond Street Hospital for Children National Health Service Trust, Institute of Child Health, University College London, London, U.K
⁷ Astrid Lindgren’s Children Hospital, Karolinska University Hospital, Stockholm, Sweden
⁸ Department of Pathology, Université Catholique de Louvain, Brussels, Belgium

Address correspondence and reprint requests to Timo Otonkoski, MD, PhD, Biomedicum Helsinki, Room C503b, PO Box 63, FIN-00014 University of Helsinki, Helsinki, Finland. E-mail: timo.otonkoski{at}helsinki.fi

Abbreviations: [¹⁸F]-DOPA, fluorine-18 L-3,4-dihydroxyphenylalanine; CHI, congenital hyperinsulinism of infancy; L-DOPA, L-3,4-dihydroxyphenylalanine; MRI, magnetic resonance imaging; NET, neuroendocrine tumor; PACS, pancreatic arterial calcium stimulation; PET, positron emission tomography; PVS, pancreatic venous sampling; SUV, standardized uptake value

Congenital hyperinsulinism of infancy (CHI) is characterized by severe hypoglycemia due to dysregulated insulin secretion, associated with either focal or diffuse pathology of the endocrine pancreas. The focal condition is caused by a paternally inherited mutation in one of the genes encoding the subunits of the ß-cell ATP-sensitive potassium channel (SUR1/ABCC8 or Kir6.2/KCNJ11) and somatic loss of maternal 11p15 alleles within the affected area. Until now, preoperative diagnostics have relied on technically demanding and invasive catheterization techniques. We evaluated the utility of fluorine-18 L-3,4-dihydroxyphenylalanine ([¹⁸F]-DOPA) positron emission tomography (PET) to identify focal pancreatic lesions in 14 CHI patients, 11 of which carried mutations in the ABCC8 gene (age 1–42 months). To reduce bias in PET image interpretation, quantitative means for evaluation of pancreatic [¹⁸F]-DOPA uptake were established. Five patients had a visually apparent focal accumulation of [¹⁸F]-DOPA and standardized uptake value (SUV) >50% higher (mean 1.8-fold) than the maximum SUV of the unaffected part of the pancreas. When these patients were operated on, a focus of 4–5 x 5–8 mm matching with the PET scan was found, and all were normoglycemic after resection of the focus. The remaining nine patients had diffuse accumulation of [¹⁸F]-DOPA in the pancreas (SUV ratio <1.5). Diffuse histology was verified in four of these, and pancreatic catheterization was consistent with diffuse pathology in four cases. In conclusion, [¹⁸F]-DOPA PET is a promising noninvasive method for the identification and localization of the focal form of CHI.

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