Introduction: Neonatal diabetes is a rare genetic disease characterized by severe, persistent hyperglycemia requiring treatment. It commonly occurs between the neonatal period and infancy, and rarely between 6 months and 1 year. presentation happens before 6 months of age. The mode of presentation can vary widely, ranging from simple incidental hyperglycemia to full on dehydration and diabetic ketoacidosis (DKA). Patients with neonatal diabetes are responding very well to insulin therapy and rarely showed insulin resistance .In our case the patient did not respond to high doses of intravenous insulin reaching 0.5 iu/kg/hr and required peritoneal dialysis.

Case Report: We report the case of a 7 month old Omani male, born to non-consanguineous young parents,who is clinically and biochemically healthy , previously investigated for stress related hyperglycemia and all his investigations was normal , presented to the private clinic with high grade fever, reduced activity and poor oral intake of and found to have Diabetic ketoacidosis and subsequently referred to Royal hospital as an emergency case for further management .on arrival to Emergency room the patient appeared tachypneic, with subcostal and suprasternal recession and grunting in keeping with Kussmaul breathing with SMBG 38 mmol /dl and severe Metabolic acidosis ( pH 6.9, bicarbonate 3.7 mEq/L, lactate 3.2, sodium 152, potassium 5.7.Management of DKA initiated as per protocol in ER , but the patient did not respond and He subsequently developed respiratory and cardiovascular compromise warranting admission to pediatric intensive care unit (PICU). As such, he was intubated and started on adrenaline 0.05 mcg/kg/min. Blood gas continued to show persistent metabolic acidosis PH 6.9 and HCO3 4mEq/L, not responding to fluid and insulin dose increasing from 0.1 unit /kg/hr reaching 0.7 IU /kg/hr (which consider to be a high dose with a dextrose fluid of 20% to avoid rapid drop in Blood glucose), After 12 HRS of resistant to DKA measures, peritoneal dialysis initiated. After starting peritoneal dialysis the acidosis improved and insulin dose weaned to gradually reached 0.1 iu /kg/hr .He was also initially started on Ceftriaxone, later kept on tazocin, vancomycin, and tamiflu for continuous spikes of fever.

Result: After three days we are able to stop peritoneal dialysis and the baby out of DKA with normal fine and motor function. He was then shifted to the high dependency unit for further care and was transferred to the ward under the endocrine team. All cultures, including blood, urine cultures came as no growth and subsequently antibiotics stopped and the baby was able to take orally well and started on subcutaneous insulin. Genetic test confirmed a heterozygous for a likely pathogenic INS missense variant (Monoallelic pathogenic variants in INS cause permanent neonatal diabetes.

Conclusion: Neonatal Diabetes Mellitus is a rare disease and requires urgent medical intervention. Intravenous insulin infusion is the standard of care in infants with hyperglycemia & amp; DKA. Peritoneal dialysis is previously not reported in literature to manage DKA in neonatal diabetes. In this particular case of severe and resistance to IV insulin Diabetic ketoacidosis, was successfully managed with peritoneal dialysis.