Differential diagnosis of congenital hyperinsulinism histological form using [18F]-DOPA PET/CT

Full Text

Open Access Open Access
Restricted Access Access granted
Restricted Access Subscription Access

Abstract

Backgraund: Congenital hyperinsulinism (CHI) is a complex condition, which is caused by hyperproduction of insulin by pancreatic β-cells. The care management is strongly depends on the histological form of the disease.
Aims: Differential diagnosis of congenital hyperinsulinism histological form using [18F]-DOPA positron emission tomography (PET) with computed tomography (CT) in patients with pharmacoresistant CHI.
Materials and methods: [18F]-DOPA PET/CT was performed in 17 patients with pharmacoresistant CHI. Subsequently, [18F]-DOPA PET/CT results were compared with the morphological study of postoperative pancreatic tissue which is known as the "gold standard" for differential diagnosis of histological forms of CHI.
Results: Diffuse form of CHI was diagnosed in 7 of 17 patients based on the results of [18F]-DOPA PET/CT, the focal form was found in the remaining 10 patients. In patients with focal disease, the median pancreatic ratio 50-60 minutes after [18F]-DOPA injection was 1.64 (from 1.14 to 3.51). Moreover in this group of patients local accumulation of radioactive tracer on PET images was visually observed. In patients with diffuse form of CHI, the median pancreatic ratio 50-60 minutes after [18F]-DOPA injection was 1.15 (from 1.02 to 1.42). The expected morphological form of CHI was confirmed by histological study in 16 children after the surgical treatment. In one patient the surgery and histological study was not performed due to relatively stable euglycemia.
Conclusions: Results of PET/CT with [18F]-DOPA in patients with CHI should be analyzed using both visual and design parameters.

About the authors

Diliara Gubaeva

ФГБУ Национальный медицинский исследовательский центр эндокринологии Минздрава России

Author for correspondence.
Email: gubaevadn@gmail.com
ORCID iD: 0000-0003-3922-2869
SPIN-code: 3431-3323

аспирант

Russian Federation, 117036, г. Москва, ул. Дмитрия Ульянова, д. 11 м. Академическая

Maria Melikian

ФГБУ Национальный медицинский исследовательский центр эндокринологии Минздрава России

Email: melikian.maria@gmail.com
ORCID iD: 0000-0002-1491-2460
SPIN-code: 4184-4383

Ведущий научный сотрудник, к.м.н.

Russian Federation, 117036, г. Москва, ул. Дмитрия Ульянова, д. 11 м. Академическая

Daria Ryjkova

ФГБУ Национальный медицинский исследовательский центр имени В. А. Алмазова Минздрава России
 

Email: d_ryjkova@mail.ru
ORCID iD: 0000-0002-7086-9153
SPIN-code: 7567-6920

Руководитель научно-клинического объединения ядерной медицины, главный научный сотрудник НИЛ ядерной медицины НИО лучевой диагностики, д. м. н., профессор РАН, врач-радиолог высшей категории, заведующая курсом радиологии кафедры лучевой диагностики и медицинской визуализации

Russian Federation, 197341, Санкт-Петербург, ул. Аккуратова, д. 2

Lubov Mitrofanova

ФГБУ Национальный медицинский исследовательский центр имени В. А. Алмазова Минздрава России

Email: lubamitr@yandex.ru
ORCID iD: 0000-0003-0735-7822
SPIN-code: 9552-8248

Заведующая НИЛ патоморфологии, д. м. н.

197341, Санкт-Петербург, ул. Аккуратова, д. 2

Irina Nikitina

ФГБУ Национальный медицинский исследовательский центр имени В. А. Алмазова Минздрава России

Email: nikitina0901@gmail.com
ORCID iD: 0000-0003-4013-0785
SPIN-code: 7707-4939

Заведующая НИЛ детской эндокринологии, зав. кафедрой детских болезней, д. м. н., врач-детский-эндокринолог высшей категории, Заслуженный работник здравоохранения РФ

Russian Federation, 197341, Санкт-Петербург, ул. Аккуратова, д. 2

References

  1. Otonkoski T, Ammälä C, Huopio H, et al. A point mutation inactivating the sulfonylurea receptor causes the severe form of persistent hyperinsulinemic hypoglycemia of infancy in Finland. Diabetes. 1999;48(2):408-415.
  2. De León DD, Stanley CA. Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates. Nat Clin Pract Endocrinol Metab. 2007;3(1):57-68. doi: 10.1038/ncpendmet0368
  3. Dunne MJ, Kane C, Shepherd RM, et al. Familial persistent hyperinsulinemic hypoglycemia of infancy and mutations in the sulfonylurea receptor. N Engl J Med. 1997;336(10):703-706. doi: 10.1056/NEJM199703063361005
  4. de Lonlay P, Fournet J-C, Touati G, et al. Heterogeneity of persistent hyperinsulinaemic hypoglycaemia. A series of 175 cases. Eur J Pediatr. 2002;161(1):37-48.
  5. Verkarre V, Fournet JC, de Lonlay P, 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(7):1286-1291. doi: 10.1172/JCI4495
  6. Yorifuji T. Congenital hyperinsulinism: current status and future perspectives. Ann Pediatr Endocrinol Metab. 2014;19(2):57-68. doi: 10.6065/apem.2014.19.2.57
  7. James C, Kapoor RR, Ismail D, Hussain K. The genetic basis of congenital hyperinsulinism. J Med Genet. 2009;46(5):289-299. doi: 10.1136/jmg.2008.064337
  8. Ismail D, Hussain K. Role of 18F-DOPA PET/CT imaging in congenital hyperinsulinism. Rev Endocr Metab Disord. 2010;11(3):165-169. doi: 10.1007/s11154-010-9145-1
  9. Evaluation of [18F]fluoro-L-DOPA positron emission tomography-computed tomography for surgery in focal congenital hyperinsulinism. - PubMed - NCBI. https://www.ncbi.nlm.nih.gov/pubmed/18073294. Accessed October 22, 2017.
  10. Treglia G, Sadeghi R, Annunziata S, Caldarella C, Bertagna F, Giovanella L. Diagnostic performance of fluorine-18-fluorodeoxyglucose positron emission tomography in the postchemotherapy management of patients with seminoma: systematic review and meta-analysis. BioMed Res Int. 2014;2014:852681. doi: 10.1155/2014/852681
  11. Otonkoski T, Näntö-Salonen K, Seppänen M, et al. Noninvasive diagnosis of focal hyperinsulinism of infancy with [18F]-DOPA positron emission tomography. Diabetes. 2006;55(1):13-18.
  12. Meintjes M, Endozo R, Dickson J, et al. 18F-DOPA PET and enhanced CT imaging for congenital hyperinsulinism: initial UK experience from a technologist’s perspective. Nucl Med Commun. 2013;34(6):601-608. doi: 10.1097/MNM.0b013e32836069d0
  13. Shield JPH. Fluorine-18 L-3,4-dihydroxyphenylalanine positron emission tomography: improving surgery and outcome in focal hyperinsulinism. Commentary to Mohnike et al.: Proposal for a standardized protocol for F-DOPA-PET (PET/CT) in congenital hyperinsulinism (Horm Res 2006;66:40-42). Horm Res. 2006;66(1):43-44. doi: 10.1159/000093472
  14. Kapoor RR, Flanagan SE, Arya VB, Shield JP, Ellard S, Hussain K. Clinical and molecular characterisation of 300 patients with congenital hyperinsulinism. Eur J Endocrinol. 2013;168(4):557-564. doi: 10.1530/EJE-12-0673
  15. Banerjee I, Skae M, Flanagan SE, et al. The contribution of rapid KATP channel gene mutation analysis to the clinical management of children with congenital hyperinsulinism. Eur J Endocrinol. 2011;164(5):733-740. doi: 10.1530/EJE-10-1136
  16. Snider KE, Becker S, Boyajian L, et al. Genotype and phenotype correlations in 417 children with congenital hyperinsulinism. J Clin Endocrinol Metab. 2013;98(2):E355-363. doi: 10.1210/jc.2012-2169
  17. Blomberg BA, Moghbel MC, Saboury B, Stanley CA, Alavi A. The value of radiologic interventions and (18)F-DOPA PET in diagnosing and localizing focal congenital hyperinsulinism: systematic review and meta-analysis. Mol Imaging Biol MIB Off Publ Acad Mol Imaging. 2013;15(1):97-105. doi: 10.1007/s11307-012-0572-0
  18. Yang J, Hao R, Zhu X. Diagnostic role of 18F-dihydroxyphenylalanine positron emission tomography in patients with congenital hyperinsulinism: a meta-analysis. Nucl Med Commun. 2013;34(4):347-353. doi: 10.1097/MNM.0b013e32835e6ac6
  19. Christiansen CD, Petersen H, Nielsen AL, et al. 18F-DOPA PET/CT and 68Ga-DOTANOC PET/CT scans as diagnostic tools in focal congenital hyperinsulinism: a blinded evaluation. Eur J Nucl Med Mol Imaging. November 2017. doi: 10.1007/s00259-017-3867-1

Supplementary files

There are no supplementary files to display.


Copyright (c) 2021 Gubaeva D., Melikian M., Ryjkova D., Mitrofanova L., Nikitina I.

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies