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Published in: Pediatric Nephrology 4/2004

01-04-2004 | Original Article

Acute hemodialysis for hyperammonemia in small neonates

Authors: Deepak K. Rajpoot, John J. Gargus

Published in: Pediatric Nephrology | Issue 4/2004

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Abstract

Neonatal metabolic emergencies require a multidisciplinary team approach for supportive management that has increasingly come to feature renal replacement therapies in addition to nutritional support, the use of pharmaceutical agents, and testing to guide management and provide a definitive diagnosis. An increased appreciation for the mechanisms involved in ammonia neurotoxicity has placed greater emphasis on the need for its rapid yet safe resolution to optimize long-term prognosis. We examined our experience of intermittent hemodialysis (HD) and considered (1) the feasibility of HD in low-weight neonates, (2) the rate of decrease in ammonia, (3) complications during HD in small neonates weighing <4 kg presenting at University Children′s Hospital between 1999 and 2002. Additionally, we review the current cellular and molecular mechanism of ammonia-induced brain injury. All patients tolerated intermittent HD and all required pressor agents. We primed all our patients with 20 U/kg of heparin and there was no subsequent need for further heparinization. We also noted that hemodynamic instability persisted during the first 1–2 h of the procedure and improved thereafter, as indicated by a decreased need for pressor agents. All neonates are alive to date. In conclusion, HD remains an effective and practical mode of renal replacement therapy having readily managed complications in preterm neonates weighing <4 kg with metabolic disorders.
Literature
1.
go back to reference Burton BK (1987) Inborn errors of metabolism: the clinical diagnosis in early infancy. Pediatrics 79:359–369PubMed Burton BK (1987) Inborn errors of metabolism: the clinical diagnosis in early infancy. Pediatrics 79:359–369PubMed
2.
go back to reference Saudubray JM, Ogier H, Bonnefont JP, Munnich A, Lombes A, Herve F, Mitchel G, The BP, Specola N, Parvy P (1989) Clinical approach to inherited metabolic diseases in the neonatal period: 20-year survey. J Inherit Med Dis 12:25–41 Saudubray JM, Ogier H, Bonnefont JP, Munnich A, Lombes A, Herve F, Mitchel G, The BP, Specola N, Parvy P (1989) Clinical approach to inherited metabolic diseases in the neonatal period: 20-year survey. J Inherit Med Dis 12:25–41
3.
go back to reference Brusilow SW, Horwich AL (2001) Urea cycle enzymes. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic and molecular basis of inherited disease. McGraw-Hill, New York pp 1909–1963 Brusilow SW, Horwich AL (2001) Urea cycle enzymes. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic and molecular basis of inherited disease. McGraw-Hill, New York pp 1909–1963
4.
go back to reference Schafer F, Straube E, Oh J, Mehls O, Mayatek E (1999) Dialysis in neonates with inborn errors of metabolism. Nephrol Dial Transplant 14:910–918PubMed Schafer F, Straube E, Oh J, Mehls O, Mayatek E (1999) Dialysis in neonates with inborn errors of metabolism. Nephrol Dial Transplant 14:910–918PubMed
5.
go back to reference Sadowski RH, Harmon WE, Jabs K (1994) Acute hemodialysis of infants weighing less than five kilograms. Kidney Int 45:903–906PubMed Sadowski RH, Harmon WE, Jabs K (1994) Acute hemodialysis of infants weighing less than five kilograms. Kidney Int 45:903–906PubMed
6.
go back to reference Gouyon JB, Desgres J, Mousson (1994) Removal of branched chain amino acids by peritoneal dialysis, continuous venovenous hemofiltration, and continuous arterio arteriovenous hemodialysis in rabbits: implications for maple syrup urine disease treatment. Pediatr Res 35:357–361PubMed Gouyon JB, Desgres J, Mousson (1994) Removal of branched chain amino acids by peritoneal dialysis, continuous venovenous hemofiltration, and continuous arterio arteriovenous hemodialysis in rabbits: implications for maple syrup urine disease treatment. Pediatr Res 35:357–361PubMed
7.
go back to reference Brusilow SW, Valle DL, Batshaw M (1979) New pathways of nitrogen excretion in inborn errors urea synthesis. Lancet II:452–454CrossRef Brusilow SW, Valle DL, Batshaw M (1979) New pathways of nitrogen excretion in inborn errors urea synthesis. Lancet II:452–454CrossRef
8.
go back to reference Feillet F, Leonard JV (1998) Alternative pathway therapy for urea cycle disorders. J Inherit Med Dis 21:101–111CrossRef Feillet F, Leonard JV (1998) Alternative pathway therapy for urea cycle disorders. J Inherit Med Dis 21:101–111CrossRef
9.
go back to reference Yoshino M, Sakaguchi Y, Kuriya N, Ohtani Y, Yamashita F, Hashimoto T, Oyanagi K, Tada K, Narisawa K, Kitagawa T (1991) A nationwide survey on transient hyperammonemia in newborn infants in Japan: prognosis of life and neurological outcome. Neuropediatrics 22:198–202PubMed Yoshino M, Sakaguchi Y, Kuriya N, Ohtani Y, Yamashita F, Hashimoto T, Oyanagi K, Tada K, Narisawa K, Kitagawa T (1991) A nationwide survey on transient hyperammonemia in newborn infants in Japan: prognosis of life and neurological outcome. Neuropediatrics 22:198–202PubMed
10.
go back to reference Leonard JV, Morris AA (2002) Urea cycle disorders. Semin Neonatol 1:27–35CrossRef Leonard JV, Morris AA (2002) Urea cycle disorders. Semin Neonatol 1:27–35CrossRef
11.
go back to reference Picca S, Dionisi-Vici C, Abeni D, Pastore A, Rizzo C, Orzalesi M, Sabetta G, Rizzoni G, Bartuli A (2001) Extracorporeal dialysis in neonatal hyperammonemia: modalities and prognostic indicators. Pediatr Nephrol 11:862–867CrossRef Picca S, Dionisi-Vici C, Abeni D, Pastore A, Rizzo C, Orzalesi M, Sabetta G, Rizzoni G, Bartuli A (2001) Extracorporeal dialysis in neonatal hyperammonemia: modalities and prognostic indicators. Pediatr Nephrol 11:862–867CrossRef
12.
go back to reference Cooper AJ (2001) Role of glutamine in cerebral nitrogen metabolism and ammonia neurotoxicity. Ment Retard Dev Disabil Res Rev 7:280–286CrossRefPubMed Cooper AJ (2001) Role of glutamine in cerebral nitrogen metabolism and ammonia neurotoxicity. Ment Retard Dev Disabil Res Rev 7:280–286CrossRefPubMed
13.
go back to reference Brusilow SW (1985) Inborn errors of urea cycle synthesis. In: Llyod JK, Scriver CR (eds) Genetic and metabolic disease in pediatrics. Butterworth, London, p 140 Brusilow SW (1985) Inborn errors of urea cycle synthesis. In: Llyod JK, Scriver CR (eds) Genetic and metabolic disease in pediatrics. Butterworth, London, p 140
14.
go back to reference Yu SP, Canzoniero LM, Choi DW (2001) Ion homeostasis and apoptosis. Curr Opin Cell Biol 13:405–411CrossRefPubMed Yu SP, Canzoniero LM, Choi DW (2001) Ion homeostasis and apoptosis. Curr Opin Cell Biol 13:405–411CrossRefPubMed
15.
go back to reference Monfort P, Kosenko E, Erceg S, Canales JJ, Felipo V (2002) Molecular mechanism of acute ammonia toxicity: role of NMDA receptors. Neurochem Int 41:95–102CrossRefPubMed Monfort P, Kosenko E, Erceg S, Canales JJ, Felipo V (2002) Molecular mechanism of acute ammonia toxicity: role of NMDA receptors. Neurochem Int 41:95–102CrossRefPubMed
16.
go back to reference Ogier H (2002) Management and emergency treatments of neonates with a suspicion of inborn errors of metabolism. Semin Neonatal 7:17–20CrossRef Ogier H (2002) Management and emergency treatments of neonates with a suspicion of inborn errors of metabolism. Semin Neonatal 7:17–20CrossRef
17.
go back to reference Brusilow SW (1984) Arginine, an indispensible aminoacid for patients with inborn errors of urea synthesis. J Clin Invest 74:2144–2148PubMed Brusilow SW (1984) Arginine, an indispensible aminoacid for patients with inborn errors of urea synthesis. J Clin Invest 74:2144–2148PubMed
18.
go back to reference Ohtani Y, Ohyanagi K, Yamamoto S (1988) Secondary carnitine deficiency in hyperammonemic attacks of ornithine transcarbamylase deficiency. J Pediatr 112:409–414PubMed Ohtani Y, Ohyanagi K, Yamamoto S (1988) Secondary carnitine deficiency in hyperammonemic attacks of ornithine transcarbamylase deficiency. J Pediatr 112:409–414PubMed
19.
go back to reference Prietsch V, Lindner M, Zschocke J, Nyhan WL, Hoffmann GF (2002) Emergency management of inherited metabolic diseases. J Inherit Metab Dis 25:531–546CrossRefPubMed Prietsch V, Lindner M, Zschocke J, Nyhan WL, Hoffmann GF (2002) Emergency management of inherited metabolic diseases. J Inherit Metab Dis 25:531–546CrossRefPubMed
20.
go back to reference Wendel U, Langenbeck U, Lombeck I, Bremer HJ (1982) Exchange transfusion in acute episodes of maple syrup urine disease: studies on branched-chain amino acids and keto-acids. Eur J Pediatr 138:293–296PubMed Wendel U, Langenbeck U, Lombeck I, Bremer HJ (1982) Exchange transfusion in acute episodes of maple syrup urine disease: studies on branched-chain amino acids and keto-acids. Eur J Pediatr 138:293–296PubMed
21.
go back to reference Batshaw Ml, Bruilow SW (1980) Treatment of hyperammonemic coma caused by inborn errors of urea synthesis. J Pediatr 97:893–900PubMed Batshaw Ml, Bruilow SW (1980) Treatment of hyperammonemic coma caused by inborn errors of urea synthesis. J Pediatr 97:893–900PubMed
22.
go back to reference Donn SM, Swartz RD, Theone JG (1979) Comparison of exchange transfusion, peritoneal dialysis, and hemodialysis for the treatment of hyperammonemia in an anuric infant. J Pediatr 95:67–70PubMed Donn SM, Swartz RD, Theone JG (1979) Comparison of exchange transfusion, peritoneal dialysis, and hemodialysis for the treatment of hyperammonemia in an anuric infant. J Pediatr 95:67–70PubMed
23.
go back to reference Gortner L, Leupold D, Pohlandt F, Bartmann P (1989) Peritoneal dialysis in the treatment of metabolic crises caused by inherited disorders of organic and amino acid metabolism. Acta Pediatr Scand 78:706–711 Gortner L, Leupold D, Pohlandt F, Bartmann P (1989) Peritoneal dialysis in the treatment of metabolic crises caused by inherited disorders of organic and amino acid metabolism. Acta Pediatr Scand 78:706–711
24.
go back to reference Sefton G, Farrell M, Noyes J (2001) The perceived learning needs of pediatric intensive care nurses caring for children requiring hemofiltration. Intensive Crit Care Nurs 17:40–50CrossRefPubMed Sefton G, Farrell M, Noyes J (2001) The perceived learning needs of pediatric intensive care nurses caring for children requiring hemofiltration. Intensive Crit Care Nurs 17:40–50CrossRefPubMed
25.
go back to reference Harvey B, Watson AR, Jepson S (2002) A renal critical educator: the interface between pediatric intensive care and nephrology. Intensive Crit Care Nurs 18:250–254CrossRefPubMed Harvey B, Watson AR, Jepson S (2002) A renal critical educator: the interface between pediatric intensive care and nephrology. Intensive Crit Care Nurs 18:250–254CrossRefPubMed
26.
go back to reference Symons JM, Brophy PD, Gregory MJ, McFee N, Somers MJ, Bunchman TE, Goldstein SL (2003) Continuous renal replacement therapy in children up to 10 kg. Am J Kidney Dis 41:984–989CrossRefPubMed Symons JM, Brophy PD, Gregory MJ, McFee N, Somers MJ, Bunchman TE, Goldstein SL (2003) Continuous renal replacement therapy in children up to 10 kg. Am J Kidney Dis 41:984–989CrossRefPubMed
27.
go back to reference Brophy PD, Maxvold V, Buchman ET (2002) CAVH/CVVH in pediatric patients. In: Nissenson AR, Fine RN (eds) Dialysis therapy. Hanely and Belfus, Philadelphia, pp 501–506 Brophy PD, Maxvold V, Buchman ET (2002) CAVH/CVVH in pediatric patients. In: Nissenson AR, Fine RN (eds) Dialysis therapy. Hanely and Belfus, Philadelphia, pp 501–506
28.
go back to reference Rutledge SL, Havens PL, Haymond MW, McLean RH, Kan JS, Brusilow SW (1990) Neonatal hemodialysis: effective therapy for the encephalopathy of inborn errors of metabolism. J Pediatr 116:125–128PubMed Rutledge SL, Havens PL, Haymond MW, McLean RH, Kan JS, Brusilow SW (1990) Neonatal hemodialysis: effective therapy for the encephalopathy of inborn errors of metabolism. J Pediatr 116:125–128PubMed
29.
go back to reference Neu AM, Christensen MJ, Brusilow SW (1992) Hemodialysis for inborn errors of metabolism. In: Nissenson AR, Fine RN (eds) Dialysis therapy. Hanely and Belfus, Philadelphia, pp 371–372 Neu AM, Christensen MJ, Brusilow SW (1992) Hemodialysis for inborn errors of metabolism. In: Nissenson AR, Fine RN (eds) Dialysis therapy. Hanely and Belfus, Philadelphia, pp 371–372
30.
go back to reference Bredt DS, Hwang PM, Snyder SH (1990) Localization of nitric oxide synthase indicating a neural role of nitric oxide. Nature 347:768–770PubMed Bredt DS, Hwang PM, Snyder SH (1990) Localization of nitric oxide synthase indicating a neural role of nitric oxide. Nature 347:768–770PubMed
31.
go back to reference Toda N, Kitamura Y, Okamura T (1993) Neural mechanism of hypertension by nitric oxide synthase inhibitor in dogs. Hypertension 21:3-8PubMed Toda N, Kitamura Y, Okamura T (1993) Neural mechanism of hypertension by nitric oxide synthase inhibitor in dogs. Hypertension 21:3-8PubMed
Metadata
Title
Acute hemodialysis for hyperammonemia in small neonates
Authors
Deepak K. Rajpoot
John J. Gargus
Publication date
01-04-2004
Publisher
Springer-Verlag
Published in
Pediatric Nephrology / Issue 4/2004
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-003-1389-5

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