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Comparison of The Peritoneal Dialysis and Continuous Veno-Venous Hemodiafiltration in 16 Neonates With Maple Syrup Urine Disease


 Introduction: Continuous Renal Replacement Therapy (CRRT) is a well-known treatment modality for patients with acute renal failure and has been increasingly used for the treatment of metabolic disorders such as Maple Syrup Urine Disease (MSUD) in recent years. Herein, we aimed to discuss our experience in 16 newborn patients with MSUD who were treated with urgent renal replacement therapy (RRT).Materials and Method: The data of patients who presented with an acute metabolic crisis due to Maple syrup urine disease and who were treated with RRT at Neonatal intensive care unit(NICU) between November 2016 and March 2020 were retrospectively evaluated. The patients underwent continuous veno-venous hemodiafiltration (CVVHDF) or peritoneal dialysis (PD) as renal replacement therapy.Results: The study enrolled a total of 16 patients, of which 8 were male and 8 were female. Eleven (68.75%) patients underwent CVVHDF and five (31.25%) underwent peritoneal dialysis. The median post-treatment leucine level was 198(20-721) μmol/L in the CVVHDF group and 1050(303-1653) μmol/L in the PD group; the median leucine reduction rate per hour was 2.56% (1.75-7.6) in the CVVHDF group and 0.78% (0.54-1.83) in the PD group. There was a significant difference between both groups regarding both parameters (p= 0.08, p=0.001, respectively). Complications such as hypotension, electrolyte imbalance, and filter obstruction occurred in the CVVHDF group while catheter revision was needed due to catheter obstruction in one patient in the PD group.Conclusion: This study showed that CVVHDF is more effective than PD for rapidly eliminating elevated Leucine levels caused by MSUD in the newborn and it is not associated with increased complication rates.