Детская хирургия. Журнал им. Ю.Ф. Исакова. 2021; 25: 11-18
Зависимость объёма резекции поджелудочной железы при врождённом гиперинсулинизме от данных ПЭТ-томографии и экспресс-биопсии
https://doi.org/10.18821/1560-9510-2021-25-1-11-18Аннотация
Введение. Достижения в области молекулярной генетики, методы визуализации (ПЭТ/КТ), медикаментозная терапия и хирургическое лечение дали возможность лучше контролировать гипогликемии и, таким образом, улучшили результаты лечения детей с врождённым гиперинсулинизмом в последние десятилетия.
Цель – определить показания, объем и результаты хирургического лечения врождённого гиперинсулинизма в зависимости от формы, при использовании для дифференциальной диагностики различных форм заболевания до операции ПЭТ/КТ, интраоперационно – экспресс-биопсии.
Материал и методы. В отделении детской хирургии НМИЦ им. В.А. Алмазова за 2011–09.2020 гг. оперирован 41 ребёнок с врожденным гиперинсулинизмом. В первую группу вошли 6 детей, у которых применялся стандартный подход, заключающийся в субтотальной резекции поджелудочной железы (95%). Во вторую группу вошли 35 пациентов, у которых до операции проводилась ПЭТ-томография с 18-F-ДОФА, и интраоперационно экспресс-биопсия поджелудочной железы. Они были оперированы в 2017–09.2020 гг. По данным ПЭТ-томографии с 18-F-ДОФА,диагностированы 10 диффузных форм и 22 фокальных, еще у 3 детей данные были сомнительными.
Результаты. После операции у 10 (29%) детей подтверждено диффузное поражение железы, у 21 (60%) ребёнка подтверждён фокальный характер поражения, у 4 (11%) интраоперационно диагностирована атипичная форма. Из 35 детей второй группы у 33 (94%) – полное купирование гиперинсулинизма, значительное улучшение психомоторного развития, однако из них 10 (29%) пациентов нуждаются в заместительной инсулинотерапии минимальными дозировками – 8 детей с диффузной формой гиперинсулинизма и 2 детей с атипичными формами. Интра- и послеоперационных осложнений не отмечалось. 20 (95%) из 21 ребёнка с фокальными формами – полное выздоровление. Интраоперационно объём резекции всегда определялся с помощью экспресс-биопсии. Представлены сложности и особенности срочного гистологического исследования.
Заключение. Таким образом, частичная панкреатэктомия при фокальных формах, субтотальная при атипичных и почти тотальная при диффузных формах позволяют справиться с гипогликемиями вследствие врождённого гиперинсулинизма.
Список литературы
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24. Lord K, De León DD. Hyperinsulinism in the Neonate. Clinics in Perinatology 2018;45(1):61–74.
25. SempouxCh, Guiot Y, Jaubert F et al. Focal and diffuse forms of congenital hyperinsulinism: the keys for differential diagnosis. Endocrine Pathology 2004;15(3):241–6.
26. Han B, Newbould M, Batra G et al. Enhanced islet cell nucleomegaly defines diffuse congenital hyperinsulinism in infancy but not other forms of the disease. Am J Clin.Pathol 2016;145:757–68.
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Russian Journal of Pediatric Surgery. 2021; 25: 11-18
Dependence of pancreas resection volume on PET-tomography and express biopsy findings
https://doi.org/10.18821/1560-9510-2021-25-1-11-18Abstract
Introduction. Recently, achievements in molecular genetics, imaging techniques (PET/CT), medicamentous therapy as well as in surgical treatment have promoted a better control of hypoglycemia and, consequently, better outcomes in children with congenital hyperinsulinism.
Purpose. To specify indications, volume and outcomes of surgical treatment in patients with congenital hyperinsulinism depending on pathology form, differential diagnostics with PET / CT and intraoperative express biopsy.
Material and methods. 41 children with congenital hyperinsulinism were operated in the department of pediatric surgery in Almazov National Medical Research Centre (Saint-Petersburg) during 2011 – September 2020. In Group 1, there were 6 children who had standard treatment with subtotal resection of the pancreas (95%). In Group 2, there were 35 patients who had PET tomography with 18-F-DOPA before surgery and intraoperative express biopsy of pancreas tissue. The analyzed patients were operated on in 2017-September 2020. PET tomography with 18-F-DOPA findings revealed that these children had 10 diffuse forms and 22 focal forms; the other 3 children had a disputable picture.
Results. After surgery, diffuse form was confirmed in 10 (29%) children; focal form - in 21 (60%) children; 4 (11%) patients had the atypical form which was diagnosed intraoperatively. Of 35 children from Group 2, 33 (94%) had complete hyperinsulinism reversal, significant improvement in their psycho-motor function; however, 10 (29%) of them require insulin replacement therapy with minimal dosages - 8 children with the diffuse form of hyperinsulinism and 2 children with the atypical one. There were no intra- and postoperative complications. 20 (95%) out of 21 children with focal forms recovered completely. The authors also describe problems and peculiarities of urgent histological examination.
Conclusion. Thus, partial pancreatectomy in focal forms, subtotal in atypical ones and almost total in diffuse forms allows to cope with hypoglycemia caused by congenital hyperinsulinism.
References
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2. Menni F., de Lonlay P., Sevin C. et al. Neurologic outcomes of 90 neonates and infants with persistenthyperinsulinemic hypoglycemia. Pediatrics. 2001; (107): 476–479.
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4. Roženková K., Güemes M., Shah P., Hussain K. The Diagnosis and Management of Hyperinsulinaemic Hypoglycaemia. Journal Clin Res Pediatr Endocrinol. 2015; 7(2): 86–97.
5. Mathew PM, Young JM, Abu-Osba YK et al. Persistent neonatal hyperinsulinism. Clinical Pediatrics (Phila) 1988; 27(3):148–51.
6. Galcheva S, Demirbilek H, Al-Khawaga S et al. The genetic and molecular mechanisms of congenital hyperinsulinism. Front Endocrinol. 2019; (10): 111.
7. Giurgea I1,et al. Acute insulin responses to calcium and tolbutamide do not differentiate focal from diffuse congenital hyperinsulinism. J Clin. EndocrinolMetab. 2004;89(2):925–9.
8. Ribeiro MJ1, et al. The added value of [18F]fluoro-L-DOPA PET in the diagnosis of hyperinsulinism of infancy: a retrospective study involving 49 children. Eur J Nucl. Med. Mol Imaging. 2007; 34(12): 2120–8.
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14. Han B, Mohamed Z, Estebanez MS et al. Atypical forms of congenital hyperinsulinism in infancy are associated with mosaic patterns of immature islet cells.J. Clin. Endocrinol.Metab. 2017;102(9):3261–67.
15. Houghton J, Banerjee I, Shaikh G et al. Unravelling the genetic causes of mosaic islet morphology in congenital hyperinsulinism. J. Pathol.Clin. Res 2020;6(1):12–16.
16. Gussinyer M., et al. Glucose intolerance and diabetes are observed in the long-term follow-up of nonpancreatectomized patients with persistent hyperinsulinemic hypoglycemia of infancy due to mutations in the ABCC8 gene. Diabetes Care 2008; 31(6): 1257–59.
17. Lord K1, et al. High Risk of Diabetes and Neurobehavioral Deficits in Individuals With Surgically Treated Hyperinsulinism. J ClinEndocrinol-Metab. 2015; 100(11): 4133–9.
18. Lord K, Dzata E, Snider KE et al. Clinical Presentation and Management of Children With Diffuse and Focal Hyperinsulinism: A Review of 223 Cases. J Clin.Endocrinol.Metab. 2013; 98 (11): 1786–9.
19. Mazor-Aronovitch K1, Landau H, Gillis D. Surgical versus non-surgical treatment of congenital hyperinsulinism. Pediatr.Endocrinol Rev. 2009; 6 (3): 424–30.
20. Ismail D1, Werther G. Persistent hyperinsulinaemichypoglycaemia of infancy: 15 years’ experience at the Royal Children’s Hospital (RCH), Melbourne. J. Pediatr. Endocrinol.Metab. 2005;18(11):1103–9.
21. Meissner T1, et al. Long-term follow-up of 114 patients with congenital hyperinsulinism. Eur J Endocrinol. 2003; 149(1): 43–51.
22. Ni J, Ge J, Zhang M et al. Genotype and phenotype analysis of a cohort of patients with congenital hyperinsulinism based on DOPA-PET CT scanning. European Journal of Pediatrics 2019;178: 1161–9.
23. Sukhotskaya A.A., Bairov V.G.,Nikitina I.L., Ryzhkova D.V., Mitro- fanova L.B., Amidkhonova S.A. Khirurgicheskoe lechenie vrozhdenno- go giperinsulinizma: predvaritel'nyi analiz. Detskaya khirurgiya. 2019; 23 (3): 124–7.
24. Lord K, De León DD. Hyperinsulinism in the Neonate. Clinics in Perinatology 2018;45(1):61–74.
25. SempouxCh, Guiot Y, Jaubert F et al. Focal and diffuse forms of congenital hyperinsulinism: the keys for differential diagnosis. Endocrine Pathology 2004;15(3):241–6.
26. Han B, Newbould M, Batra G et al. Enhanced islet cell nucleomegaly defines diffuse congenital hyperinsulinism in infancy but not other forms of the disease. Am J Clin.Pathol 2016;145:757–68.
27. Bensley RR. Studies on the pancreas of the Guinea pig.Am J Anat 1911; 12(3): 308–11.
28. Bowie DJ. Cytological studies of the islets of Langerhans in a teleost, Neomaenisgriseus. Anat. Rec 1924;29(1):57–73.
29. Lilli R. Patogistologicheskaya tekhnika i prakticheskaya gistokhimiya. M.: MIR, 1969. 274–79.
