Euglycemic Ketoacidosis in Spinal Muscular Atrophy

Case Reports in Pediatrics
28 Jan, 2019 ,

A 13-year-old-female, was transferred to ICU post-op after spondylodesis procedure. She was a k/c/o SMA type 1 (Werdnig–Hoffmann disease). During her ICU stay on day-3 she developed metabolic acidosis. She already had compromised respiration requiring NIV at home due to respiratory muscle weakness. She also had a history of fasting on the day of surgery and two days post-surgery. Her Kidney and Liver functions were normal and there was no h/o DM. Urine reports showed severe ketonuria and a diagnosis of euglycemic ketoacidosis was made. The patient was managed with fat-free enteral nutrition  and the acidosis resolved within 48-hours.