Starvation ketoacidosis in patient with muscular dystrophy
Srour K, Khan BSA, and Novak J. Starvation ketoacidosis in patient with muscular dystrophy. Am J Kidney Dis 2020; 75(4):640.
Am J Kidney Dis
Patients with Muscular Dystrophy have small muscle mass; thus, they have less glycogen stores, and therefore they are more prone to develop ketoacidosis with minimal stress or decreased oral intake. In our case we are presenting a rare presentation of ketoacidosis in a patient with muscular dystrophy, who was treated successfully with Lactated Ringer and Dextrose solution due to her concurrent hyperchloremic non-anion gap metabolic acidosis. Our Patient is forty-eight-year-old female with history of Muscular Dystrophy and Chronic Respiratory Failure dependent on Ventilator, she was referred to our hospital for evaluation of a granulation tissue in her Trachea. She was admitted and found to have non-AG metabolic Acidosis, VBG showed Ph of 7.23 Bicarbonate of 12, BMP showed Cr <0.1, Chloride 114 (high), BMI 18, in addition to leukocytosis with WBC>16000. Next day her labs showed Bicarbonate of 8, Ph of 7.29 and AG of 16 (Na 137, Cl 113), Albumin 4.2. We checked her Beta Hydroxybutyrate (BHB) which came back 6.6 (High). Due to her underlying Muscular Dystrophy and decrease oral intake, she was diagnosed with Starvation Ketoacidosis. Since she had a non-anion gap metabolic acidosis at the time of admission which was most likely secondary to Renal Tubular Acidosis, (Patient labs showed Hypokalemia, Urine AG of 30), we decided to start her on ringer lactate and dextrose solution. After 24 hours, her labs showed significant improvement and her BHB trended down and after 48 hours her labs have been normalized. Few cases have been reported regarding ketoacidosis in patients with muscular dystrophy, all reported cases were treated directly with dextrose and normal saline. In our case we used LR and D5% due to concurrent non anion gap metabolic acidosis which we believe it was due to RTA, in which giving normal saline (hgih Chloride content) will worsen the acidosis. HAGMA in patient with no diabetes and Muscular dystrophy should raise suspicion for Ketoacidosis. Treatment is with Dextrose and normal saline ususally. Using LR solurion sometime is better option in cases with concurrent hyperchloremic acidosis.