Kidney Week

Abstract: TH-PO505

A Systematic Review of Efficacy and Safety of Dialysis Modalities in Neonates/Children with Inborn Errors of Metabolism

Session Information

• Pediatric Nephrology - I
  November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

• 1900 Pediatric Nephrology

Authors

• Raina, Manan, Hawken High School, Cleveland, Ohio, United States

Manan Raina,

• Shah, Raghav, Northeast Ohio Medical University, Rootstown, Ohio, United States

Raghav Shah,

• Doshi, Kush, Cleveland Clinic Akron General, Akron, Ohio, United States

Kush Doshi,

• Sharma, Pranjal, Northeast Ohio Medical University, Rootstown, Ohio, United States

Pranjal Sharma,

• Sethi, Sidharth Kumar, Medanta The Medicity Medanta Institute of Kidney and Urology, Gurugram, Haryana, India

Sidharth Kumar Sethi,

Background

Inborn errors of Metabolism (IEM) are due to mutations that involve aberrations in the metabolism of macromolecules and synthesis of essential compounds, especially hyperammonia. Treatment of IEM typically involves RRT initiation. Our systematic review in neonates and children investigates survival rates and ammonia level reduction in varying RRT modalities (CRRT, HD, PD).

Methods

The literature search was conducted through PubMed, Web of Science, and Embase. The search terms included ‘neonates’ and ‘pediatric’ linked with ‘IEM’ and the varying RRT modalities. The studies included entailed survival rate and ammonia level data in patients with IEM with an intervention of RRT. Analysis variables included efficacy outcomes [% reduction in ammonia (RIA) from pre-dialysis to post-dialysis and time to 50% RIA] and mortality among neonates with IEM on different dialysis modalities. The data was analyzed using R version 3.1.0.

Results

A total of 38 studies were included, with a total sample size of 697. The mortality was observed to be lower among neonates with IEM receiving CRRT vs. PD or HD. The pooled proportion (95% CI) of mortality of those who got CRRT was 25.4% (21.26% - 29.91%) [I²: 44.6%; p=0.0117. Those who received PD was 36.64% (30% - 43.6%) [I²: 39.5%; p=0.0579; And those who received HD was 35.9 % (26.5% - 46.2%) [I²: 18.0%; p=0.2876. The mean (SD) time to 50% RIA level was seen to be higher with CAVHD or CVVHD vs. HD [3.4 (4.1) or 4.3 (3.5) vs. 1.6 (0.4)] hrs. The time to 50% ammonia reduction was significantly lower with CVVHDF vs. PD [median (IQR): 6 (6–9.5) vs. 14 (6–30) hrs; p=0.01, CVVH vs. PD [mean (SD): 4.4 (1.5) vs. 16.3 (10.9) hrs; p=0.042] and CVVHD vs. PD [4.7 (2.5) vs. 13.5 (6.2) hrs; p<0.0001. Likewise, the mean (SD) time to 50% decline in plasma ammonia or leucine levels with CVVHD (n=7) vs. PD (n=5) [7.1 (4.1) vs. 17.9 (12.4) hrs; p<0.02.

Conclusion

Results indicate that the efficacy of the mean % RIA is superior in CRRT. Comparing time to 50% RIA levels, CRRT is superior to PD, while HD appears slightly superior to CRRT. Findings show lower mortality among patients with IEM who received CRRT vs. PD or HD. In conclusion, PD has been shown to be less effective compared to HD and CRRT. The ability to reduce ammonia quicker by HD is also associated with a greater risk of mortality than CRRT.