Continuous Renal Replacement Therapy for Treatment of Severe Attacks of Inborn Errors of Metabolism

Ayse Filiz Yetimakman; Selman Kesici; Murat Tanyildiz; Benan Bayrakci

doi:10.1055/s-0039-1683991

Journal of Pediatric Intensive Care, Table of Contents

J Pediatr Intensive Care 2019; 08(03): 164-169
DOI: 10.1055/s-0039-1683991

Original Article

Georg Thieme Verlag KG Stuttgart · New York

Continuous Renal Replacement Therapy for Treatment of Severe Attacks of Inborn Errors of Metabolism

Ayse Filiz Yetimakman

¹Division of Pediatric Intensive Care, Department of Pediatrics, Hacettepe University, Ankara, Turkey

,

Selman Kesici

²Department of Pediatrics, Pediatric Intensive Care Unit, Dr. Sami Ulus Maternity and Children's Training and Research Hospital, Ankara, Turkey

,

Murat Tanyildiz

¹Division of Pediatric Intensive Care, Department of Pediatrics, Hacettepe University, Ankara, Turkey

,

Benan Bayrakci

¹Division of Pediatric Intensive Care, Department of Pediatrics, Hacettepe University, Ankara, Turkey

› Author Affiliations

Abstract

Severe metabolic crises in children with inborn errors of metabolism can result in mortality or severe morbidities where continuous renal replacement therapy (CRRT) can be lifesaving. Clinical data, the pediatric risk of mortality (PRISM) scores calculated in the first 24 hours, and pediatric logistic organ dysfunction (PELOD) scores calculated in the last 24 hours before CRRT, were studied. Overall, CRRT was successful in restoring metabolic balance in 72% of patients. PELOD scores before CRRT were lower in survivors (p = 0.02). Despite numerous comorbid factors, CRRT can be used effectively in management of metabolic crises. Early intervention with this therapy before occurrence of complications must be targeted.

Keywords

continuous renal replacement therapy - inborn errors of metabolism - acute metabolic crises

Full Text

References

References
1 Lai YC, Huang HP, Tsai IJ, Tsau YK. High-volume continuous venovenous hemofiltration as an effective therapy for acute management of inborn errors of metabolism in young children. Blood Purif 2007; 25 (04) 303-308
2 Arbeiter AK, Kranz B, Wingen AM. , et al. Continuous venovenous haemodialysis (CVVHD) and continuous peritoneal dialysis (CPD) in the acute management of 21 children with inborn errors of metabolism. Nephrol Dial Transplant 2010; 25 (04) 1257-1265
3 Tsai IJ, Hwu WL, Huang SC. , et al. Efficacy and safety of intermittent hemodialysis in infants and young children with inborn errors of metabolism. Pediatr Nephrol 2014; 29 (01) 111-116
4 Chen CY, Tsai TC, Lee WJ, Chen HC. Continuous hemodiafiltration in the treatment of hyperammonemia due to methylmalonic acidemia. Ren Fail 2007; 29 (06) 751-754
5 Schaefer F, Straube E, Oh J, Mehls O, Mayatepek E. Dialysis in neonates with inborn errors of metabolism. Nephrol Dial Transplant 1999; 14 (04) 910-918
6 Picca S, Dionisi-Vici C, Bartuli A. , et al. Short-term survival of hyperammonemic neonates treated with dialysis. Pediatr Nephrol 2015; 30 (05) 839-847
7 Picca S, Dionisi-Vici C, Abeni D. , et al. Extracorporeal dialysis in neonatal hyperammonemia: modalities and prognostic indicators. Pediatr Nephrol 2001; 16 (11) 862-867
8 McBryde KD, Kershaw DB, Bunchman TE. , et al. Renal replacement therapy in the treatment of confirmed or suspected inborn errors of metabolism. J Pediatr 2006; 148 (06) 770-778
9 Thompson GN, Butt WW, Shann FA. , et al. Continuous venovenous hemofiltration in the management of acute decompensation in inborn errors of metabolism. J Pediatr 1991; 118 (06) 879-884
10 Hanudel M, Avasare S, Tsai E, Yadin O, Zaritsky J. A biphasic dialytic strategy for the treatment of neonatal hyperammonemia. Pediatr Nephrol 2014; 29 (02) 315-320
11 Brismar J, Ozand PTCT. CT and MR of the brain in the diagnosis of organic acidemias. Experiences from 107 patients. Brain Dev 1994; 16 (Suppl): 104-124
12 Radmanesh A, Zaman T, Ghanaati H, Molaei S, Robertson RL, Zamani AA. Methylmalonic acidemia: brain imaging findings in 52 children and a review of the literature. Pediatr Radiol 2008; 38 (10) 1054-1061
13 Ozand PT, Rashed M, Gascon GG. , et al. Unusual presentations of propionic acidemia. Brain Dev 1994; 16 (Suppl): 46-57
14 Thimm E, Hadzik B, Höhn T. Continuous venovenous hemofiltration rapidly lowers toxic metabolites in a patient with MSUD and imminent cerebral herniation. Klin Padiatr 2010; 222 (04) 264-265
15 Duval M, Fenneteau O, Doireau V. , et al. Intermittent hemophagocytic lymphohistiocytosis is a regular feature of lysinuric protein intolerance. J Pediatr 1999; 134 (02) 236-239
16 Stork LC, Ambruso DR, Wallner SF. , et al. Pancytopenia in propionic acidemia: hematologic evaluation and studies of hematopoiesis in vitro. Pediatr Res 1986; 20 (08) 783-788
17 Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Eur J Pediatr 2007; 166 (02) 95-109
18 Dellinger RP, Levy MM, Rhodes A. , et al; Surviving Sepsis Campaign Guidelines Committee including the Pediatric Subgroup. Surviving sepsis campaign: international guidelines for management of severe sepsis and septic shock: 2012. Crit Care Med 2013; 41 (02) 580-637
19 Westrope C, Morris K, Burford D, Morrison G. Continuous hemofiltration in the control of neonatal hyperammonemia: a 10-year experience. Pediatr Nephrol 2010; 25 (09) 1725-1730
20 Foland JA, Fortenberry JD, Warshaw BL. , et al. Fluid overload before continuous hemofiltration and survival in critically ill children: a retrospective analysis. Crit Care Med 2004; 32 (08) 1771-1776
21 Guerra-Moreno J, Barrios N, Santiago-Borrero PJ. Severe neutropenia in an infant with methylmalonic acidemia. Bol Asoc Med P R 2003; 95 (01) 17-20