ХИРУРГИЧЕСКОЕ ЛЕЧЕНИЕ ВРОЖДЕННОГО ГИПЕРИНСУЛИНИЗМА: ПРЕДВАРИТЕЛЬНЫЙ АНАЛИЗ

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Детская хирургия. Журнал им. Ю.Ф. Исакова. 2019; 23: 124-127

ХИРУРГИЧЕСКОЕ ЛЕЧЕНИЕ ВРОЖДЕННОГО ГИПЕРИНСУЛИНИЗМА: ПРЕДВАРИТЕЛЬНЫЙ АНАЛИЗ

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

https://doi.org/10.18821/1560-9510-2019-23-3-124-127

Аннотация

Список литературы

1. McQuarrie I. Idiopathic spontaneously occurring hypoglycemia in infants; clinical significance of problem and treatment. Am. J. Dis. Child. 1954; 87(4): 444-7.

2. Stanley C.A., Baker L. Hyperinsulinism in infants and children: diagnosis and therapy. AdvPediatr. 1976; 23: 315-55.

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5. 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; 47(1): 130-135.

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13. Katherine Lord, 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): 1786-1789.

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

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Russian Journal of Pediatric Surgery. 2019; 23: 124-127

SURGICAL TREATMENT OF THE CONGENITAL HYPERINSULINISM: A PRELIMINARY ANALYSIS

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

https://doi.org/10.18821/1560-9510-2019-23-3-124-127

Abstract

Introduction. Advances in molecular genetics, imaging techniques (PET/CT), medicamentous therapy and surgical treatment in the recent decades have improved hypoglycemia control and , thus, improved treatment outcomes in children with congenital hyperinsulinism. Purpose. To define indications for different techniques of surgical correction in patients with congenital hyperinsulinism depending on the disease form. Material and methods. 23 children were operated on for congenital hyperinsulinism in the department of pediatric surgery in the Almazov Medical Research Center from 2011 till 2018. In 2011-2016, 5 children were operated on by the standard approach which included subtotal pancreas resection (95%). Since 2017, PET tomography with 18-F-DOPA and intraoperative rapid biopsy of the pancreas have been introduced into the curative algorithm. In 2017-2018, 18 children were operated: 6 patients with the diffuse form and 10 - with the focal one. Two more children had controversial outcomes. Results. Fifteen children (83%) had complete hyperinsulinism control and significant improvement in their psycho-motor function. Out of 18 children, 10 (56%) had a complete recovery: among them - 8 (89%) out of 9 children with the focal form of hyperinsulinism and 2 (22%) out of 9 children with the diffuse form. Two children (11%) had a marked positive dynamics. Six patients (33%) needed insulin therapy with minimal dosages - all were children with the diffuse form of hyperinsulinism (6 (67% ) out of 9). Conclusion. Partial pancreatectomy in patients with focal forms or subtotal pancreatectomy in patients with diffuse and atypical forms allow to eliminate hypoglycemia caused by the congenital hyperinsulinism and to prevent damage to the central nervous system in newborns and infants.

References

1. McQuarrie I. Idiopathic spontaneously occurring hypoglycemia in infants; clinical significance of problem and treatment. Am. J. Dis. Child. 1954; 87(4): 444-7.

2. Stanley C.A., Baker L. Hyperinsulinism in infants and children: diagnosis and therapy. AdvPediatr. 1976; 23: 315-55.

3. Roženková K., Güemes M., Shah P., Hussain K.L. The Diagnosis and Management of HyperinsulinaemicHypoglycaemia. Journal Clin. Res. Pediatr. Endocrinol. 2015; 7(2): 86-97.

5. 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; 47(1): 130-135.

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

7. Melikyan M.A., i dr. Vrozhdennyi giperinsulinizm: diagnostika i lechenie. Pediatriya. 2011; 90(1): 59-65. [Melikyan M.A., et al. Congenital giperinsulinizm: diagnostics and treatment. Pediatriya. 2011; 90 (1): 59-65. (in Ryssian)]

8. Graham E.A., Hartmann A.F. Subtotal resection of the pancreas for hypoglycaemia. Surg. Gynecol. Obstet. 1934; 59: 474-9.

9. 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.

12. Lord K.1., et al. High Risk of Diabetes and Neurobehavioral Deficits in Individuals With Surgically Treated Hyperinsulinism. J. Clin. Endocrinol. Metab. 2015; 100(11): 4133-9.

13. Katherine Lord, 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): 1786-1789.

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

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

ХИРУРГИЧЕСКОЕ ЛЕЧЕНИЕ ВРОЖДЕННОГО ГИПЕРИНСУЛИНИЗМА: ПРЕДВАРИТЕЛЬНЫЙ АНАЛИЗ

Аннотация

SURGICAL TREATMENT OF THE CONGENITAL HYPERINSULINISM: A PRELIMINARY ANALYSIS

Abstract

События

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