The Journal of Pediatrics
Volume 150, Issue 2 , Pages 140-145 , February 2007

Diagnosis and Localization of Focal Congenital Hyperinsulinism by 18F-Fluorodopa PET Scan

Received 8 February 2006 ,Revised 5 July 2006 ,Accepted 12 August 2006.

References 

  1. Glaser B, Thornton P, Otonkoski T, Junien C. Genetics of neonatal hyperinsulinism. Arch Dis Child Fetal Neonatal Ed. 2000;82:F79–F86
  2. Stanley CA. Hyperinsulinism in infants and children. Pediatr Clin North Am. 1997;44:363–374
  3. de Lonlay-Debeney P, Poggi-Travert F, Fournet JC, Sempoux C, Vici CD, Brunelle F, et al. Clinical features of 52 neonates with hyperinsulinism. N Engl J Med. 1999;340:1169–1175
  4. Verkarre V, Fournet JC, de Lonlay P, Gross-Morand MS, Devillers M, Rahier J, et al. Paternal mutation of the sulfonylurea receptor (SUR1) gene and maternal loss of 11p15 imprinted genes lead to persistent hyperinsulinism in focal adenomatous hyperplasia. J Clin Invest. 1998;102:1286–1291
  5. Grimberg A, Ferry RJ, Kelly A, Koo-McCoy S, Polonsky K, Glaser B, et al. Dysregulation of insulin secretion in children with congenital hyperinsulinism due to sulfonylurea receptor mutations. Diabetes. 2001;50:322–328
  6. Henwood MJ, Kelly A, Macmullen C, Bhatia P, Ganguly A, Thornton PS, et al. Genotype-phenotype correlations in children with congenital hyperinsulinism due to recessive mutations of the adenosine triphosphate-sensitive potassium channel genes. J Clin Endocrinol Metab. 2005;90:789–794
  7. Stanley CA, Thornton PS, Ganguly A, MacMullen C, Underwood P, Bhatia P, et al. Preoperative evaluation of infants with focal or diffuse congenital hyperinsulinism by intravenous acute insulin response tests and selective pancreatic arterial calcium stimulation. J Clin Endocrinol Metab. 2004;89:288–296
  8. Adzick NS, Thornton PS, Stanley CA, Kaye RD, Ruchelli E. A multidisciplinary approach to the focal form of congenital hyperinsulinism leads to successful treatment by partial pancreatectomy. J Pediatr Surg. 2004;39:270–275
  9. Sandler MP. Diagnostic Nuclear Medicine. 4th edition. Philadelphia: Lippincott Williams & Wilkins; 2003;
  10. Sundin A, Eriksson B, Bergstrom M, Langstrom B, Oberg K, Orlefors H. PET in the diagnosis of neuroendocrine tumors. Ann N Y Acad Sci. 2004;1014:246–257
  11. Aguanno A, Afar R, Albert VR. Tissue-specific expression of the nonneuronal promoter of the aromatic L-amino acid decarboxylase gene is regulated by hepatocyte nuclear factor 1. J Biol Chem. 1996;271:4528–4538
  12. Borelli MI, Villar MJ, Orezzoli A, Gagliardino JJ. Presence of DOPA decarboxylase and its localisation in adult rat pancreatic islet cells. Diabetes Metab. 1997;23:161–163
  13. Lindstrom P, Sehlin J. Aromatic amino acids and pancreatic islet function: a comparison of L-tryptophan and L-5-hydroxytryptophan. Mol Cell Endocrinol. 1986;48:121–126
  14. Hoegerle S, Altehoefer C, Ghanem N, Koehler G, Waller CF, Scheruebl H, et al. Whole-body 18F dopa PET for detection of gastrointestinal carcinoid tumors. Radiology. 2001;220:373–380
  15. Hoegerle S, Nitzsche E, Altehoefer C, Ghanem N, Manz T, Brink I, et al. Pheochromocytomas: detection with 18F DOPA whole body PET: initial results. Radiology. 2002;222:507–512
  16. Otonkoski T, Veijola R, Huopio H, Nanto-Salonen K, Tapanainen P, deLonlay P, et al Diagnosis of focal persistent hyperinsulinism of infancy with 18F-fluoro-L-DOPA PET. In:  Krzisnik C editors. 42nd Annual Meeting of the European Society for Paediatric Endocrinology (ESPE); 2003. Ljubljana (Slovenia): Hormone Research; 2003;p. 2
  17. Otonkoski T, Nanto-Salonen K, Seppanen M, Veijola R, Huopio H, Hussain K, et al. Noninvasive diagnosis of focal hyperinsulinism of infancy with [18F]-DOPA positron emission tomography. Diabetes. 2006;55:13–18
  18. de Lonlay P, Simon-Carre A, Ribeiro MJ, Boddaert N, Giurgea I, Laborde K, et al. Congenital hyperinsulinism: pancreatic [18F]fluoro-L-dihydroxyphenylalanine (DOPA) positron emission tomography and immunohistochemistry study of DOPA decarboxylase and insulin secretion. J Clin Endocrinol Metab. 2006;91:933–940
  19. Ribeiro MJ, De Lonlay P, Delzescaux T, Boddaert N, Jaubert F, Bourgeois S, et al. Characterization of hyperinsulinism in infancy assessed with PET and 18F-fluoro-L-DOPA. J Nucl Med. 2005;46:560–566
  20. Finegold DN, Stanley CA, Baker L. Glycemic response to glucagon during fasting hypoglycemia: an aid in the diagnosis of hyperinsulinism. J Pediatr. 1980;96:257–259
  21. Stanley CA, Baker L. Hyperinsulinism in infancy: diagnosis by demonstration of abnormal response to fasting hypoglycemia. Pediatrics. 1976;57:702–711
  22. Glaser B, Ryan F, Donath M, Landau H, Stanley CA, Baker L, et al. Hyperinsulinism caused by paternal-specific inheritance of a recessive mutation in the sulfonylurea-receptor gene. Diabetes. 1999;48:1652–1657
  23. Kassem SA, Ariel I, Thornton PS, Hussain K, Smith V, Lindley KJ, et al. p57(KIP2) expression in normal islet cells and in hyperinsulinism of infancy. Diabetes. 2001;50:2763–2769
  24. Rahier J, Guiot Y, Sempoux C. Persistent hyperinsulinaemic hypoglycaemia of infancy: a heterogeneous syndrome unrelated to nesidioblastosis. Arch Dis Child Fetal Neonatal Ed. 2000;82:F108–F112
  25. Suchi M, MacMullen C, Thornton PS, Ganguly A, Stanley CA, Ruchelli ED. Histopathology of congenital hyperinsulinism: retrospective study with genotype correlations. Pediatr Dev Pathol. 2003;6:322–333
  26. Namavari M, Bishop A, Satyamurthy N, Bida G, Barrio JR. Regioselective radiofluorodestannylation with [18F]F2 and [18F]CH3COOF: a high yield synthesis of 6-[18F]Fluoro-L-dopa. Int J Rad Appl Instrum [A]. 1992;43:989–996
  27. Karp JS, Surti S, Daube-Witherspoon ME, Freifelder R, Cardi CA, Adam LE, et al. Performance of a brain PET camera based on anger-logic gadolinium oxyorthosilicate detectors. J Nucl Med. 2003;44:1340–1349
  28. Newcombe RG. Two-sided confidence intervals for the single proportion: comparison of seven methods. Stat Med. 1998;17:857–872

 Supported in part by grants from the National Institutes of Health: RO1-DK-56268 (C.A.S.) and MO1-RR-00240. O.T.H. was supported by National Institutes of Health training grant T32-DK63688 (C.A.S.).

PII: S0022-3476(06)00790-6

doi: 10.1016/j.jpeds.2006.08.028

The Journal of Pediatrics
Volume 150, Issue 2 , Pages 140-145 , February 2007