SURGICAL TREATMENT OF ATIPICAL FORMS OF CONGENITAL HYPERINSULINISM



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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.

作者简介

A. Sukhotskaya

V.A. Almazov National Medical Research Centre

编辑信件的主要联系方式.
Email: sukhotskaya_aa@almazovcentre.ru
ORCID iD: 0000-0002-8734-2227
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V. Bairov

V.A. Almazov National Medical Research Centre

Email: noemail@neicon.ru
ORCID iD: 0000-0002-8446-830X
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L. Mitrofanova

V.A. Almazov National Medical Research Centre

Email: noemail@neicon.ru
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D. Ryzhkova

V.A. Almazov National Medical Research Centre

Email: noemail@neicon.ru
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I. Nikitina

V.A. Almazov National Medical Research Centre

Email: noemail@neicon.ru
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S. Amidhonova

V.A. Almazov National Medical Research Centre

Email: noemail@neicon.ru
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参考

  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.
  2. Arnoux J.B., Verkarre V., Saint-Martin C. et al. Congenital hyperinsulinism; current trends in diagnosis and therapy. Orphanet J Rare Dis. 2011; 6(63): doi: 10.1186/1750-1172-6-63. [PMC free article] [PubMed] [CrossRef]
  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.
  7. Ribeiro M.J. 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.
  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. Меликян М.А., и соавт. Врожденный гиперинсулинизм: диагностика и лечение. Педиатрия. 2011; 90 (1): 59-65
  10. 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: 1257-9.
  11. 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. doi: 10.1210/jc.2015-2539. Epub 2015 Sep 1.
  12. Rahier J., Wallon J., Henquin J.C. Cell populations in the endocrine pancreas of human neonates and infants. Diabetologia. 1981; 20: 540-6.
  13. Kassem S.A., Ariel I., Thornton P.S., Scheimberg I., Glaser B. Beta-cell proliferation and apoptosis in the developing normal human pancreas and in hyperinsulinism of infancy. Diabetes. 2000; 49: 1325-33.
  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.
  16. 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): E1786-9. Published online 2013 Sep 20. doi: 10.1210/jc.2013-2094 PMCID: MC3816257 PMID: 24057290
  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.
  18. Ismail D.1., Werther G. Persistent hyperinsulinaemi chypoglycaemia of infancy: 15 years’ experience at the Royal Children’s Hospital (RCH), Melbourne. J Pediatr. Endocrinol Metab. 2005; 18(11): 1103-9.
  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.

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