Sotelo-Cruz N, Cordero-Olivares A, Ramírez-Rodríguez C, López-Cervantes G, Hurtado-Valenzuela J, López-Cervantes BM

Language: Spanish
References: 17
Page: 409-414
PDF size: 123.56 Kb.

ABSTRACT

We report two cases of infants who presented seizures and persistent hypoglycemia. In both infants, we established diagnosis of persistent hypoglycemia by hyperinsulinism (PHH); glycemic/insulinic ratio was > 0.3, and we found no pancreatic changes by means of other diagnostic procedures. The medical treatment consisted of hydrocortisone, diazoxide, and octreotide without response. Definitive treatment made was sub-total pancreatectomy; postoperatory evolution was satisfactory, and the histologic report showed changes in Langerhans islet of nesidioblastosis. We conclude that PHH and glucose/insulin ratio > 0.3 indicates that surgical treatment with pancreatectomy should not be delayed.

REFERENCES

1. Cryer PE. Glucose homeostasis and hypoglycemia. In: Wilson JD, Foster DW, editors. Williams’ textbook of endocrinology. 8th ed. Philadelphia, PA, USA: W.B. Saunders Co.; 1992. pp. 1223-1253.

2. Dacou-Voutetakis C, Psychou F, Maniati-Cristidis M. Persistent hyperinsulinemic hypoglycaemia of infancy: long term results. J Pediatr Endocrinol Metab 1998;11:131-141.

3. Lee PJ, Leonard JV. Hypoglycaemia. In: Brook GD, editor. Clinical pediatric endocrinology. 3rd ed. London: Blackwell Science;1995;35: 677-693.

4. Anzoátegui-Espínola RE, Dorantes-Álvarez LM, García-Morales L, Bracho-Blanchet E, Garibay-Nieto GN, Sadowinski-Pine S. Hipoglucemia hiperinsulinémica persistente de la infancia; revisión de casos en un período de 10 años. Bol Med Hosp Infant Mex 2000;57: 383-389.

5. Glasgow AM. Hipoglucemia. En: Hung W, editor. Endocrinología pediátrica clínica. San Louis, MO, USA: Mosby Year Book;1992; 10:332-354.

6. Camberos MC, Abendur J, Cresto JC. Hipoglucemia. En: Pombo M, editor. Endocrinología pediátrica. 3ra ed. Madrid, España: McGraw-Hill Interamericana; 2002. pp. 1103-1121.

7. Tiznado-Ortega G. Recién nacido hijo de madre diabética. Análisis retrospectivo de dos años en el Hospital Infantil del Estado de Sonora. Tesis. México: Facultad de Medicina, UNAM;1999.

8. Pascale L, Fournet JC, Rahier J. Somatic deletion of the imprinted 11 p 15 region in sporadic persistent hyperinsulinemic hypoglycemia of infancy is specific of focal adenomatosus hyperplasia and endorses partial pancreatectomy. J Clin Invest 1997;100:802-807.

9. Stanley C. Hiperinsulismo en lactantes y niños. Clin Pediatr Norteam 1997;2:385-396.

10. Weinsimer SA, Stanley CHA, Berry GT, Yudkoff M, Tuchman M, Thorn Ton PS. A syndrome of congenital hyperinsulinism and hyperammonemia. J Pediatr 1997;130:661-664.

11. Glaser B. Persistent hyperinsulinemic hypoglycemia of infancy: long-term octreotide treatment without pancreatectomy. J Pediatr 1993; 123:644-650.

12. Anil-Apak R. Preoperative use of octreotide in a newborn with persistent hyperinsulinemic hypoglycaemia of infancy. J Pediatr Endocrinol Metab 1998;11:143-145.

13. Bas F, Daremdeliler F, Demirkol D, Bunkak R, Saka N, Gunoz H. Successful therapy with calcium chanel blocker (nifedipine) in persistent neonatal hyperinsulinemic hypoglycemia of infancy. J Pediatr Endocrinol Metab 1999;12:873-878.

14. Rahier J. Partial or near-total pancreatectomy for persistent neonatal hyperinsulinemic hypoglycaemia: the pathologist’s role. Histopathology 1998;32:15-19.

15. Baker L. Management of hyperinsulinism in infants. J Pediatr 1991; 119:755-757.

16. Leibowitz G, Glaser B. Hyperinsulinemic hypoglycemia of infancy (nesidioblastosis) in clinical remission: high incidence of diabetes mellitus and persistent B-cell dysfunction at long-term follow-up. J Clin Endocrinol Metab 1995;80:386-392.

17. Naon H, Thomas DW. Secretory neoplasms of the pancreas. In: Wyllie R, Hyams J, editors. Pediatric gastrointestinal disease. Philadelphia, PA, USA: W.B. Saunders Co.; 1999. pp. 695-701.