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SURGICAL TREATMENT OF THE CONGENITAL HYPERINSULINISM: A PRELIMINARY ANALYSIS
- Authors: Sukhotskaya A.A.1, Bairov V.G.1, Nikitina I.L.1, Ryzhkova D.V.1, Mitrofanova L.B.1, Amidkhonova S.A.1
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Affiliations:
- V.A. Almazov National Medical Research Centre
- Issue: Vol 23, No 3 (2019)
- Pages: 124-127
- Section: ORIGINAL ARTICLES
- Submitted: 05.03.2021
- Published: 05.06.2019
- URL: https://jps-nmp.ru/jour/article/view/137
- DOI: https://doi.org/10.18821/1560-9510-2019-23-3-124-127
- ID: 137
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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.
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About the authors
A. A. Sukhotskaya
V.A. Almazov National Medical Research Centre
Author for correspondence.
Email: sukhotskaya_aa@almazovcentre.ru
ORCID iD: 0000-0002-8734-2227
Россия
V. G. Bairov
V.A. Almazov National Medical Research Centre
Email: noemail@neicon.ru
Россия
I. L. Nikitina
V.A. Almazov National Medical Research Centre
Email: noemail@neicon.ru
Россия
D. V. Ryzhkova
V.A. Almazov National Medical Research Centre
Email: noemail@neicon.ru
Россия
L. B. Mitrofanova
V.A. Almazov National Medical Research Centre
Email: noemail@neicon.ru
Россия
S. A. Amidkhonova
V.A. Almazov National Medical Research Centre
Email: noemail@neicon.ru
Россия
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