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. 2022 Jun;26(3):658-666.
doi: 10.1111/1744-9987.13816. Epub 2022 Feb 24.

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

İbrahim Deger  1 , Muhittin Çelik  2 , İbrahim Taş  3 , Serhat Samancı  4
Affiliations

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

İbrahim Deger et al. Ther Apher Dial. 2022 Jun.

Abstract

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.

Keywords: CVVHDF; MSUD; newborn; peritoneal dialysis.

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References

REFERENCES

    1. Stroek K, Boelen A, Bouva MJ, Sain-van D, der Velden M, Schielen PCJI, et al. Evaluation of 11 years of newborn screening for maple syrup urine disease in The Netherlands and a systematic review of the literature: strategies for optimization. JIMD Rep. 2020;54(1):68-78.
    1. Frazier DM, Allgeier C, Homer C, Marriage BJ, Ogata B, Rohr F, et al. Nutrition management guideline for maple syrup urine disease: an evidence- and consensus-based approach. Mol Genet Metab. 2014;112(3):210-7.
    1. Strauss KA, Puffenberger EG, Carson VJ. Maple syrup urine disease. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, LJH B, Stephens K, et al., editors. GeneReviews® [internet]. Seattle (WA): University of Washington, Seattle; 2006 1993-2020. PMID: 20301495.
    1. Demirkol D, Şık G, Topal N, Çıtak A, Zeybek Ç, Tüten A, et al. Continuous venovenous hemodiafiltration in the treatment of maple syrup urine disease. Blood Purif. 2016;42(1):27-32.
    1. Spector BL, Misurac JM. Renal replacement therapy in neonates. NeoReviews. 2019;20(12):e697-710.

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