Departement of Child Health,Faculty of Medicine Universitas of Padjajaran Hasan Sadikin General Hospital, Bandung, Indonesia Background Neonatal diabetes mellitus is a rare metabolic disorder, affecting approximately 1 in 500,000 live births and may be either transient or permanent. Affected neonates frequently present with hyperglycemia, intrauterine growth retardation, and variable degrees of dehydration. Genotyping identifies the exact unique molecular aetiology of very early onset insulin requiring diabetes and has the potential to dramatically alter the management of the patient, who would otherwise be insulin dependent for life. Objective To diagnose and management of diabetic ketoacidosis in a patient with neonatal diabetes mellitus. Case illustration An infant 2 months old with diabetic ketoacidosis. The patient presented with chief complain of dyspnea. Physical examination revealed sign of dehydration, tachycardia and fever. From history taking the patient had polyuria, polydipsi and intrauterine growth retardation. The laboratory analysis showed hyperglycemia, metabolic acidosis, ketonuria, low level of Cpeptide and negative antibody for islet cell. There were fluctuation in blood glucose level during monitoring, the range of glucose level was 20 mg/dl to 1057 mg/dl. Genetic molecular analysis was in progress until this case report released. The patient was treated with regular insulin infusion in acute period and was converted to oral sulphonylureas after the condition was stable. Conclusion Correct identification and appropriate management of diabetic ketoacidosis will gives optimal blood glucose control in patient with neonatal diabetes mellitus. Keywords: diabetic ketoacidosis, neonatal diabetes, sulphonylurea
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