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A Follow up Case Study of Neonatal Diabetes due to Mutation of ABCC 8 Gene: Transition from Insulin to Oral Glibenclamide was effective and safe

Yong-Ju Kong, Kang Yu, Gwang-Ryong Pak, Yong-Il Kim, Ju-Ryong Sim, Jong-Hui Jo


Context: Neonatal diabetes is a rare condition occurring in approximately 1 in 500, 000 live births. It is occasionally due to mutations of genes which encode potassium ATP channel (KATP).

Aim: The aim of this study is to identify the long term efficacy and safety of switch from insulin to oral sulfonylurea in a case of neonatal diabetes due to a mutation of ABCC 8 gene.

Subject and Method: 6 years old female was diagnosed as a life threatening DKA in the 4th month of life and after genetic analysis identified a missense mutation (p. F577L) in her ABCC8 gene, we switched her insulin therapy to oral glibenclamide administration. Since then, we have observed her blood glucose control and general status for 6 years.

Results: Her metabolic control has been good and the glibenclamide dose has been reduced from the initial dose of 1mg/kg/day to the current dose of 0.25mg/kg/day.

Conclusion: Switch from insulin to oral glibenclamide in a case of neonatal diabetes due to an ABCC 8 gene mutation is effective and safe.


neonatal diabetes, sulfonylurea, switch, ABCC 8, mutation

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