High-dose Continuous Renal Replacement Therapy for Neonatal Hyperammonemia

Overview

Journal Pediatr Nephrol

Specialties Nephrology
Pediatrics

Date 2013 Mar 9

PMID 23471476

Citations 31

Authors

Joann M Spinale

Benjamin L Laskin

Neal Sondheimer

Sarah J Swartz

Stuart L Goldstein

Affiliations

Soon will be listed here.

Abstract

Background: Infants with hyperammonemia can present with nonspecific findings so ordering an ammonia level requires a high index of suspicion. Renal replacement therapy (RRT) should be considered for ammonia concentrations of >400 μmol/L since medical therapy will not rapidly clear ammonia. However, the optimal RRT prescription for neonatal hyperammonemia remains unknown. Hemodialysis and continuous renal replacement therapy (CRRT) are both effective, with differing risks and benefits.

Case-diagnosis/treatment: We present the cases of two neonates with hyperammonemia who were later diagnosed with ornithine transcarbamylase deficiency and received high-dose CRRT. Using dialysis/replacement flow rates of 8,000 mL/h/1.73 m(2) (1,000 mL/h or fourfold higher than the typical rate used for acute kidney injury) the ammonia decreased to <400 μmol/L within 3 h of initiating CRRT and to <100 μmol/L within 10 h.

Conclusions: We propose a CRRT treatment algorithm to rapidly decrease the ammonia level using collaboration between the emergency department and departments of genetics, critical care, surgery/interventional radiology, and nephrology.

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References

Picca S, Bartuli A, Dionisi-Vici C . Medical management and dialysis therapy for the infant with an inborn error of metabolism. Semin Nephrol. 2008; 28(5):477-80. DOI: 10.1016/j.semnephrol.2008.05.007. View

Bunchman T, Brophy P, Goldstein S . Technical considerations for renal replacement therapy in children. Semin Nephrol. 2008; 28(5):488-92. DOI: 10.1016/j.semnephrol.2008.05.009. View

Wong K, Wong S, Lam S, Tam S, Tsoi N . Ammonia clearance by peritoneal dialysis and continuous arteriovenous hemodiafiltration. Pediatr Nephrol. 1998; 12(7):589-91. DOI: 10.1007/s004670050511. View

Puliyanda D, Harmon W, Peterschmitt M, Irons M, Somers M . Utility of hemodialysis in maple syrup urine disease. Pediatr Nephrol. 2002; 17(4):239-42. DOI: 10.1007/s00467-001-0801-2. View

Phan V, Clermont M, Merouani A, Litalien C, Tucci M, Lambert M . Duration of extracorporeal therapy in acute maple syrup urine disease: a kinetic model. Pediatr Nephrol. 2006; 21(5):698-704. DOI: 10.1007/s00467-006-0044-3. View

Fakler C, Kaftan H, Nelin L . Two cases suggesting a role for the L-arginine nitric oxide pathway in neonatal blood pressure regulation. Acta Paediatr. 1995; 84(4):460-2. DOI: 10.1111/j.1651-2227.1995.tb13673.x. View

Chen C, Tsai T, Lee W, Chen H . Aminograms during continuous hemodiafiltration in the treatment of hyperammonemia due to ornithine transcarbamylase deficiency. Ren Fail. 2007; 29(6):661-5. DOI: 10.1080/08860220701459618. View

Msall M, Batshaw M, Suss R, Brusilow S, Mellits E . Neurologic outcome in children with inborn errors of urea synthesis. Outcome of urea-cycle enzymopathies. N Engl J Med. 1984; 310(23):1500-5. DOI: 10.1056/NEJM198406073102304. View

Pela I, Seracini D, Donati M, Lavoratti G, Pasquini E, Materassi M . Peritoneal dialysis in neonates with inborn errors of metabolism: is it really out of date?. Pediatr Nephrol. 2007; 23(1):163-8. DOI: 10.1007/s00467-007-0607-y. View

10.

McBryde K, Kershaw D, Bunchman T, Maxvold N, Mottes T, Kudelka T . Renal replacement therapy in the treatment of confirmed or suspected inborn errors of metabolism. J Pediatr. 2006; 148(6):770-8. DOI: 10.1016/j.jpeds.2006.01.004. View

11.

Schaefer F, Straube E, Oh J, Mehls O, Mayatepek E . Dialysis in neonates with inborn errors of metabolism. Nephrol Dial Transplant. 1999; 14(4):910-8. DOI: 10.1093/ndt/14.4.910. View

12.

Hackbarth R, Bunchman T, Chua A, Somers M, Baum M, Symons J . The effect of vascular access location and size on circuit survival in pediatric continuous renal replacement therapy: a report from the PPCRRT registry. Int J Artif Organs. 2008; 30(12):1116-21. DOI: 10.1177/039139880703001212. View

13.

Enns G, Berry S, Berry G, Rhead W, Brusilow S, Hamosh A . Survival after treatment with phenylacetate and benzoate for urea-cycle disorders. N Engl J Med. 2007; 356(22):2282-92. DOI: 10.1056/NEJMoa066596. View

14.

Bunchman T, Barletta G, Winters J, Gardner J, Crumb T, McBryde K . Phenylacetate and benzoate clearance in a hyperammonemic infant on sequential hemodialysis and hemofiltration. Pediatr Nephrol. 2007; 22(7):1062-5. DOI: 10.1007/s00467-007-0436-z. View

15.

Mathias R, Kostiner D, Packman S . Hyperammonemia in urea cycle disorders: role of the nephrologist. Am J Kidney Dis. 2001; 37(5):1069-80. DOI: 10.1016/s0272-6386(05)80026-5. View