Abstract: SA-PO0357
Metabolically Symptomatic Asymptomatic Bacteriuria: Recurrent AKI Triggered by Escherichia coli Colonization in Methylmalonic Acidemia
Session Information
- AKI: Case Reports - Drug/Toxin Injury, Crystals, Obstruction, and Unusual Presentations
October 24, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 102 AKI: Clinical, Outcomes, and Trials
Authors
- Hariharan, Nithesh, New York City Health and Hospitals Jacobi, New York, New York, United States
- Shastri, Rujul Piyush, New York City Health and Hospitals Jacobi, New York, New York, United States
- Tekalign, Rediet, New York City Health and Hospitals Jacobi, New York, New York, United States
- Suresh, Priyadarshini, New York City Health and Hospitals Jacobi, New York, New York, United States
Introduction
Methylmalonic acidemia (MMA) results from a deficiency of methylmalonyl-CoA mutase (MMUT), impairing the conversion of methylmalonyl-CoA to succinyl-CoA. The mut0 subtype lacks all MMUT activity and carries the worst prognosis. Infections trigger catabolism, overwhelming the impaired propionate pathway and leading to methylmalonic acid accumulation, which causes mitochondrial damage. While treating asymptomatic bacteriuria (ASB) is discouraged, its potential to trigger metabolic crises in MMA is undescribed.
Case Description
A 31-year-old female with mut0 MMA had three admissions in three months for metabolic crisis. Each admission presented with nausea, vomiting, and poor intake. Labs showed metabolic acidosis with elevated anion gap, increased methylmalonic acid, hyperammonemia, anemia requiring transfusion, and AKI on CKD. Urine cultures grew >100,000 CFU E. coli all three times despite absent urinary symptoms. Management included fluids, IV L-carnitine, protein restriction, and IV antibiotics. Creatinine returned to baseline, but eGFR declined from 20 to 16 mL/min/1.73 m2 over 3 months. To our knowledge, this is the first report of recurrent metabolic crises triggered exclusively by ASB in MMA.
Discussion
This illustrates a self-amplifying cycle (Figure 1). ASB triggers catabolism, releasing propiogenic amino acids that generate excess methylmalonic acid. This depletes ATP in proximal tubular cells and increases ROS levels, leading to AKI. AKI impairs renal excretion of methylmalonic acid, worsening the crisis. Each episode causes irreversible nephron loss. MMA patients may require surveillance cultures, treatment of bacteriuria regardless of symptoms, and CKD-safe prophylaxis. For progressive CKD despite management, early liver-kidney transplantation should be considered.
Figure 1:Metabolic Renal Injury Cycle