ХИРУРГИЧЕСКОЕ ЛЕЧЕНИЕ ФОКАЛЬНЫХ ФОРМ ВРОЖДЁННОГО ГИПЕРИНСУЛИНИЗМА: ВСЕ ЛИ ОПРЕДЕЛЕНО?

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Детская хирургия. Журнал им. Ю.Ф. Исакова. 2019; 23: 296-302

ХИРУРГИЧЕСКОЕ ЛЕЧЕНИЕ ФОКАЛЬНЫХ ФОРМ ВРОЖДЁННОГО ГИПЕРИНСУЛИНИЗМА: ВСЕ ЛИ ОПРЕДЕЛЕНО?

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

https://doi.org/10.18821/1560-9510-2019-23-6-296-302

Аннотация

Введение. у 30-60% всех пациентов с ВГИ отмечается задержка психомоторного развития, а у 15-25% наблюдается тяжелое органическое поражение головного мозга, включая эпилепсию. Своевременная диагностика и интенсивное лечение позволяют предотвратить развитие тяжелых неврологических осложнений. При неэффективности консервативной терапии необходимо хирургическое лечение. Цель исследования - определить показания к различным способам хирургической коррекции фокальных форм врожденного гиперинсулинизма. Материал и методы. в отделении детской хирургии НМИЦ им. В.А. Алмазова за период с 2017 по 03.2019 г. оперированы 14 детей с фокальными формами ВГИ, по данным ПЭТ-КТ, из них у 2 данные были сомнительными. Результаты. у 13 (93%) детей - полное купирование гиперинсулинизма, значительное улучшение психомоторного развития. У 10 (71%) из 14 детей - полное выздоровление, у1 (7%) - достигнута значительная положительная динамика. 3 (21%) пациентов нуждаются в заместительной инсулинотерапии минимальными дозировками - это все дети с атипичной (смешанной аденоматозно-диффузной) формой гиперинсулинизма. Выводы. При фокальном поражении поджелудочной железы показана частичная панкреатэктомия с максимальным сохранением здоровой, по данным экспресс-биопсии, ткани железы при любой локализации поражения, при атипичных формах объем панкреатэктомии определяется индивидуально.

Список литературы

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Russian Journal of Pediatric Surgery. 2019; 23: 296-302

SURGICAL TREATMENT OF FOCAL FORMS OF CONGENITAL HYPERINSULINISM: IS ALL CLEAR?

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

https://doi.org/10.18821/1560-9510-2019-23-6-296-302

Abstract

Introduction. 30-60% of all patients with congenital hyperinsulinism have a delayed psychomotor development, and 15-25% of them have severe organic brain damage, including epilepsy. Timely diagnostics and intensive treatment can prevent severe neurological complications. With the ineffectiveness of conservative therapy, surgical treatment is a necessary alternative. Objective. To define indications for various options of surgical correction of focal forms of congenital hyperinsulinism. Material and Methods. In the Department of Pediatric Surgery of V.A. Almazova National Medical Research Centre, in 2017-03.2019, 14 children with focal forms of congenital hyperinsulinism were operated after analyzing their PET-CT findings; in 2 patients out of them they were doubtful. Results. 13 (93%) patients had a complete relief of hyperinsulinism and a significant improvement in their psycho-motor development. 10 (71%) of 14 children had a complete recovery; 1 (7%) - a significant positive trend. 3 (21%) patients needed insulin therapy with minimal dosages. They all were children with an atypical (mixed adenomatous-diffuse) form of hyperinsulinism. Conclusions. In case of focal lesions in the pancreas, partial pancreatectomy with maximal preservation of healthy tissue, defined by the express biopsy, and with any lesion localization is indicated. For atypical forms, the volume for pancreatectomy dissection is defined individually.

References

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

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

3. Mathew PM, Young JM, Abu-Osba YK, et al. Persistent neonatal hyperinsulinism. ClinPediatr (Phila). 1988; 27: 148-51. [PubMed]

4. Roženková K, Güemes M, Shah P, Hussain K1. The Diagnosis and Management of HyperinsulinaemicHypoglycaemia. J Clin Res Pediatr Endocrinol. 2015 Jun; 7(2): 86-97. doi: 10.4274/jcrpe.1891.

5. Giurgea I1,et al. Acute insulin responses to calcium and tolbutamide do not differentiate focal from diffuse congenital hyperinsulinism. J. Clin Endocrinol Metab. 2004 Feb; 89(2): 925-9.

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

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

9. Graham EA, Hartmann AF. Subtotal resection of the pancreas for hypoglycaemia. Surg. Gynecol. Obstet. 1934; 59: 474-9.

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

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

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

17. Mazor-Aronovitch K1, Landau H, Gillis D. Surgical versus non-surgical treatment of congenital hyperinsulinism. Pediatr. Endocrinol. Rev. 2009 Mar; 6(3): 424-30.

19. Meissner T1, et al. Long-term follow-up of 114 patients with congenital hyperinsulinism. Eur. J. Endocrinol. 2003 Jul; 149(1): 43-51.

ХИРУРГИЧЕСКОЕ ЛЕЧЕНИЕ ФОКАЛЬНЫХ ФОРМ ВРОЖДЁННОГО ГИПЕРИНСУЛИНИЗМА: ВСЕ ЛИ ОПРЕДЕЛЕНО?

Аннотация

SURGICAL TREATMENT OF FOCAL FORMS OF CONGENITAL HYPERINSULINISM: IS ALL CLEAR?

Abstract

События

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