Peritoneal Dialysis for End-Stage Kidney Disease due to Mitochondrial Encephalopathy, Lactic Acidosis, and...

We report a 23-year-old male with kidney failure secondary to childhood Focal Segmental Glomerulosclerosis who presented with sepsis, severe lactic acidosis, and encephalopathy. Following stabilization of the acute condition with Continuous Veno-Venous Hemofiltration, a diagnosis of Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like episodes (MELAS) was confirmed by muscle biopsy and the identification of the m.3243A>G mutation. This case highlights the diagnostic difficulties of this rare disease and the challenges in selecting a long-term renal replacement therapy. Specifically, the use of lactate-buffered peritoneal dialysis fluids in these patients remains controversial. In our case, the patient demonstrated poor tolerance to intermittent haemodialysis due to MELAS-associated cardiomyopathy. However, despite theoretical concerns regarding lactate load, steady-state serum lactate remained stable on Continuous Ambulatory Peritoneal Dialysis (CAPD), and the patient achieved good functional recovery. We conclude that while acute decompensation requires aggressive management, CAPD is a feasible long-term dialysis modality for patients with kidney failure secondary to MELAS.