Evaluation Of Neonatal Hyperinsulinemic Hypoglycemia According To Pharmacological Treatment Approach: Single Center Experience

Öz

Aim: Hyperinsulinism is the most common cause of persistent and recurrent hypoglycaemia in newborn. The aim of this study was to evaluate patients who were diagnosed with hyperinsulinemic hypoglycemia retrospectively.Material and Methods: Patients’ records that were admitted to Zekai Tahir Burak Women’s Health Education and Research Hospital Neonatal Intensive Care Unit to between January 2012 and January 2015 were overviewed.Results: Levels of insulin and glucose, insulin/glucose ratio, the highest glucose infusion rates, time of recovery from hypoglycemia and duration of hospitalization were similar between diazoxide monotherapy group and combined multi-therapy group p>0.05 .Conclusion: Hyperinsulinemic hypoglycemia in the newborn period should be immediately and effectively treated to prevent further neurological damage. Maintaining normoglycemia with appropriate glucose infusion rates and early pharmacologic treatment is essential. Diazoxide is an effective and well-tolerated option as first line therapy in newborn hypoglycemia.

Anahtar Kelimeler

• Hyperinsulinism,hypoglycemia,diazoxide,newborn

Yenidoğan Döneminde Hiperinsülinemik Hipoglisemi Tanısı Alan Hastaların Farmokolojik Tedavi Yaklaşımına Göre Değerlendirilmesi: Tek Merkez Deneyimi

Öz

Amaç: Hiperinsülinizmin infantlarda persistan ve tekrarlayan hipogliseminin en sık nedenidir. Bu çalışmamızın amacı hiperinsülinemik hipoglisemi tanısı alan hastaları tedavi ihtiyaçlarına göre gruplandırmak ve kısa dönem prognozlarını araştırmaktır.Gereç ve Yöntemler: Ocak 2012-Ocak 2015 tarihleri arasında Zekai Tahir Burak Kadın Sağlığı Eğitim ve Araştırma Hastanesi Yenidoğan Yoğun Bakım ünitesinde yatan ve hiperinsülinemik hipoglisemi tanısı alan hastalar çalışmaya dahil edildi.Bulgular: Diazoksit ile kombine tedavi grubu karşılaştırıldığında insülin, glukoz, insülin/glukoz oranı, en yüksek dekstroz ihtiyacı, hipoglisemi kontrol zamanı ve hastanede yatış süreleri açısından istatistiksel bir farklılık görülmedi p>0.05 .Sonuç: Yenidoğanda hiperinsülinemik hipoglisemi, nörolojik hasarı önlemek için acil ve etkin tedavi edilmesi gereken bir durumdur. Hiperinsülinemik hipoglisemi yenidoğanlarda normoglisemiyi sağlamak için yüksek konsantrasyonda glukoz infüzyonu yapılması ve erken dönemde ilaç tedavisi başlanması gerekmektedir. İlk seçenek tedavi olan diazoksit etkindir ve iyi tolere edilmektedir.

Anahtar Kelimeler

• Hiperinsulinizm,hipoglisemi,diazoksit,yenidoğan

Kaynakça

1. Aynsley-Green A, Polak JM, Bloom SR, Gough MH, Keeling J, Ashcroft SJ et al. Nesidioblastosis of the pancreas: definition of the syndrome and the management of the severe neonatal hyperinsulinaemic hypoglycae- mia. Arch Dis Child. 1981; 56 : 496-508.
2. Glaser B, Thornton P, Otonkoski T, Junien C.. Genetics of neonatal hype- rinsulinism. Arch Dis Child. 2000; 82 : 79-86.
3. Bellanné-Chantelot C1, Saint-Martin C, Ribeiro MJ, Vaury C, Verkarre V, Arnoux JB et al. ABCC8 and KCNJ11 molecular spectrum of 109 patients with diazoxide-unresponsive congenital hyperinsulinism. J Med Genet. 2010; 47 : 752-759.
4. James C, Kapoor RR, Ismail D, Hussain K. The genetic basis of congeni- tal hyperinsulinism. J Med Genet. 2009; 46 : 289-299.
5. De Lonlay P1, Cormier-Daire V, Amiel J, Touati G, Goldenberg A, Fournet JC et al. Facial appearance in persistent hyperinsulinemic hypoglycemia. Am J med genet .2002; 111 : 130-133.
6. Touati G1, Poggi-Travert F, Ogier de Baulny H, Rahier J, Brunelle F, Nihoul-Fekete C et al. Long-term treatment of persistent hyperinsulina- emic hypoglycaemia of infancy with diazoxide: a retrospective review of 77 cases and analysis of efficacy-predicting criteria. Eur J Pediatr. 1998; 157: 628-633.
7. Hussain Khalid. Diagnosis and management of hyperinsulinaemic hy- poglycaemia of infancy. Horm Res. 2008; 69: 2-13.
8. Committee on Fetus and Newborn, Adamkin DH. Postnatal glucose ho- meostasis in late-preterm and term infants. Pediatrics 2011; 127: 575– 579.

9. Hussain K, Blankenstein O, De Lonlay P, Christesen HT., et al. Hype- rinsulinaemic hypoglycaemia: biochemical basis and the importance of maintaining normoglycaemia during management. Arch Dis Child. 2007; 92: 568-570.
10. Panten U1, Burgfeld J, Goerke F, Rennicke M, Schwanstecher M, Wal- lasch A et al. Control of insulin secretion by sulfonylureas, meglitinide and diazoxide in relation to their binding to the sulfonylurea receptor in pancreatic islets. Biochem Pharmacol. 1989; 38: 1217-1229.
11. Shilyansky J1, Fisher S, Cutz E, Perlman K, Filler RM et al. Is 95% pancre- atectomy the procedure of choice for treatment of persistent hyperinsuli- nemic hypoglycemia of the neonate? J Pediatr Surg. 1997; 32: 342-346.
12. Degirmencioglu H, Oncel MY, Yurttutan S, Ekmen S, Suna Oguz S, Uras N, et al. Intractable Feeding Intolerance and Abdominal Distention in a Preterm Infant: An Unusual Side Effect of Diazoxide. Pediat Therapeut. 2013; 3:149.
13. Wuthrich C, Schubiger G, Zuppinger K. Persistent neonatal hyperinsuli- nemic hypoglycemia in two siblings successfully treated with diazoxide. Helv Paediatr Acta. 1986; 41: 455-459.
14. Stanley CA, Baker L. The causes of neonatal hypoglycemia. N Engl J Med. 1999; 340:1200-1201.
15. Meissner T, Wendel U, Burgard P, Schaetzle S, Mayatepek E. Long-term follow-up of 114 patients with congenital hyperinsulinism. Eur J Endoc- rinol. 2003; 149: 43-51.