(1426) Kabadi Syndrome: A Diagnostic Dilemma of Ketoacidosis

Introduction: Ketoacidosis has been well-documented in relation to uncontrolled diabetes, acute alcoholism, and prolonged starvation. A less known cause is acute pancreatitis-induced euglycemic ketoacidosis (Kabadi Syndrome), caused by high circulating lipase levels. Given similarities in presentation and biochemical parameters, identifying the differentiating features of various etiologies is imperative

Description: A 63-year-old female with alcohol use disorder presented with acute encephalopathy was noted to have critically high anion gap metabolic acidosis, anion gap 50, pH 6.9, serum bicarbonate 4 mmol/L ketonemia 15 mmol/liter with ketonuria, normoglycemia and elevated lipase 1263 u/l. Serum osmolar gap elevated with acute kidney injury, negative toxic alcohols, serum ethanol, and urine toxicology. Abdominal CT showed diffuse peri pancreatic fat stranding suggestive of pancreatitis. The patient received aggressive fluid resuscitation first with D5W followed by Isotonic bicarbonate infusion. The patient improved clinically with concurrent decline and normalization of serum lipase, anion gap, ketones and serum pH, and was transferred out of ICU

Discussion: Pancreatic ketoacidosis is a diagnosis of exclusion. In diabetics with a state of starvation or on SGLT2i, euglycemic Diabetic ketoacidosis is a possibility. However, our patient was neither. Similarly, contrary to starvation, which rarely causes ketonemia or acidosis and only induces ketonuria which resolves with resuscitation, our patient had severe acidosis and ketones persisted despite resuscitation with intravenous fluids with dextrose. Our patient’s serum ETOH was negative, ketones were significantly and persistently elevated, while in alcoholic ketoacidosis, serum ketones are negative/minimally elevated and persist for short periods. In acute pancreatitis, elevated serum lipase levels cause peripheral adipose stores mobilization, lipolysis, and ketogenesis, inducing ketonemia and ketoacidosis. Therefore, ketoacidosis declines with supportive treatment in concurrence with serum lipase decline and resolution occurs with normalization of lipase levels. Therefore it was attributed to ketoacidosis in our patient to acute pancreatitis as both ketonemia and ketonuria also known as Kabadi syndrome which resolved with recovery from pancreatitis.