АТИПИЧНЫЕ ФОРМЫ ВРОЖДЁННОГО ГИПЕРИНСУЛИНИЗМА: ХИРУРГИЧЕСКОЕ ЛЕЧЕНИЕ

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Детская хирургия. Журнал им. Ю.Ф. Исакова. 2020; 24: 83-88

АТИПИЧНЫЕ ФОРМЫ ВРОЖДЁННОГО ГИПЕРИНСУЛИНИЗМА: ХИРУРГИЧЕСКОЕ ЛЕЧЕНИЕ

Сухоцкая А. А., Баиров В. Г., Митрофанова Л. Б., Рыжкова Д. В., Никитина И. Л., Амидхонова С. А.

https://doi.org/10.18821/1560-9510-2020-24-2-83-88

Аннотация

Введение. У 30-60% всех пациентов с врождённым гиперинсулинизмом (ВГИ) отмечается задержка психомоторного развития, а у 15-25% - тяжёлое органическое поражение головного мозга, включая эпилепсию. Своевременная диагностика и интенсивное лечение позволяют предотвратить развитие тяжёлых неврологических осложнений. При неэффективности консервативной терапии необходимо хирургическое лечение. Цель исследования - показать особенности хирургической коррекции атипичных форм врождённого гиперинсулинизма. Материал и методы. В отделении детской хирургии НМИЦ им. В.А. Алмазова с 2017 по март 2019 г. оперированы 3 детей с атипичными формами ВГИ и с поражением различных участков поджелудочной железы. Результаты. У 2 детей - полное купирование гиперинсулинизма при наблюдении в течение 9 мес (1,5 года после операции), у 1 достигнута значительная положительная динамика, значительное улучшение психомоторного развития. Выводы. У части детей с предположительно фокальными формами гиперинсулинизма, по данным ПЭТ-КТ, можно столкнуться с гистологически атипичными формами. При атипичном поражении поджелудочной железы интраоперационная биопсия кардинально меняет планируемый объём панкреатэктомии вплоть до практически тотального удаления железы.

Список литературы

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Russian Journal of Pediatric Surgery. 2020; 24: 83-88

SURGICAL TREATMENT OF ATIPICAL FORMS OF CONGENITAL HYPERINSULINISM

Sukhotskaya A. A., Bairov V. G., Mitrofanova L. B., Ryzhkova D. V., Nikitina I. L., Amidhonova S. A.

https://doi.org/10.18821/1560-9510-2020-24-2-83-88

Abstract

Introduction. 30-60% of all patients with congenital hyperinsulinism have a delayed psychomotor development, and 15-25% of them have a severe organic brain damage, including epilepsy. The timely diagnostics and intensive care can prevent severe neurological complications. If the conservative therapy is not effective, then the surgical treatment is a need. Objective: To demonstrate features of surgical correction of atypical forms of congenital hyperinsulinism. Material and methods. 11 children with atypical forms of congenital hyperinsulinism were operated on in the department of pediatric surgery in the V.A. Almazov National Medical Center in 2017-03.2019. Results. 2 children demonstrated a complete relief of hyperinsulinism in 9 months (1.5 years) of follow-up; one patient had a significant improvement of his psycho-motor development. Conclusion. Some children with presumably focal forms of hyperinsulinism, by PET-CT findings, may have histologically atypical forms. In the atypical lesion of the pancreas, an intraoperative biopsy dramatically changes the planned volume of pancreatectomy up to almost total removal of the gland.

References

1. Mc Quarrie I. Idiopathic spontaneously occurring hypoglycemia in infants; clinical significance of problem and treatment. Am J Dis Child. 1954; 87(4): 399-428.

3. Bruining G.J. Recent advances in hyperinsulinism and the pathogenesis of diabetes mellitus. Curr Opin Pediatr. 1990; 2: 758-65.

4. Mathew P.M., Young J.M., Abu-Osba Y.K. et al. Persistent neonatal hyperinsulinism. Clin Pediatr (Phila). 1988; 27: 148-51. [PubMed]

5. Roženková K., Güemes M., Shah P., Hussain K.I. The Diagnosis and Management of Hyperinsulinaemic Hypoglycaemia. J.Clin Res Pediatr Endocrinol. 2015; 7(2): 86-97. doi: 10.4274/jcrpe.1891.

6. Giurgea I.1. et al. Acute insulin responses to calcium and tolbutamide do not differentiate focal from diffuse congenital hyperinsulinism. J.Clin Endocrinol Metab. 2004; 89(2): 925-9.

8. Lovvorn H.N. 3rd1 et al. Congenital hyperinsulinism and the surgeon: lessons learned over 35 years. J Pediatr Surg. 1999; 34(5): 786-92; discussion 792-3.

9. Melikyan M.A., i soavt. Vrozhdennyi giperinsulinizm: diagnostika i lechenie. Pediatriya. 2011; 90 (1): 59-65

12. Rahier J., Wallon J., Henquin J.C. Cell populations in the endocrine pancreas of human neonates and infants. Diabetologia. 1981; 20: 540-6.

14. Pablo Laje et al. Pancreatic head resection and Roux-en-Y pancreatico jejunostomy for the treatment of the focal form of congenital hyperinsulinism. J Pediatr.Surg. 2012; 47(1): 130-5.

15. Palladino A.A., Stanley C.A. Nesidioblastosis no longer! It’s all about genetics. J. Clin Endocrinol Metab. 2011; 96: 617-9.

17. Mazor-Aronovitch K.1., Landau H., Gillis D. Surgical versus non-surgical treatment of congenital hyperinsulinism. Pediatr. Endocrinol Rev. 2009; 6(3): 424-30.

19. Meissner T.1. et al. Long-term follow-up of 114 patients with congenital hyperinsulinism. Eur J. Endocrinol. 2003; 149(1): 43-51.

20. Jack M.M. et al. The outcome in Australian children with hyperinsulinism of infancy: early extensive surgery in severe cases lowers risk of diabetes. Clin. Endocrinol (Oxf). 2003; 58(3): 355-64.

АТИПИЧНЫЕ ФОРМЫ ВРОЖДЁННОГО ГИПЕРИНСУЛИНИЗМА: ХИРУРГИЧЕСКОЕ ЛЕЧЕНИЕ

Аннотация

SURGICAL TREATMENT OF ATIPICAL FORMS OF CONGENITAL HYPERINSULINISM

Abstract

События

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