Continuous veno-venous hemodiafiltration in neonates with maple syrup urine disease.

INTRODUCTION

Herein, we aimed to discuss our experience in 16 newborn patients with Maple syrup urine disease (MSUD) who were treated with urgent renal replacement therapy (RRT).

METHODS

The patients underwent continuous veno-venous hemodiafiltration (CVVHDF) or peritoneal dialysis (PD) as renal replacement therapy.

RESULTS

Eleven (68.75%) patients underwent CVVHDF and five (31.25%) underwent peritoneal dialysis. The median leucine reduction rate per hour was 2.56% (1.75-7.6) in the CVVHDF group, 0.78% (0.54-1.83) in the PD group, and was significantly higher in the CVVHDF group (p = 0.001). Posttreatment plasma leucine levels were found to be 198 (20-721) μmol/L in the CVVHDF group and 600 (250-967) μmol/L in the PD group, and CVVHDF was found to be significantly lower (p = 0.08). Complications such as hypotension, electrolyte imbalance, and filter obstruction occurred in the CVVHDF 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.