Continuous venovenous hemofiltration in the management of acute decompensation in inborn errors of metabolism

Thompson, Greg; Butt, Warwick; Shann, Frank; Kirby, Denise M.; Henning, Robert; Howells, David W.; Osborne, A. D.

doi:10.1016/s0022-3476(05)82198-5

Cited by 73 publications

(35 citation statements)

References 8 publications

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“…In the National Taiwan University Hospital from February 2001 to April 2005, 8 patients were included by entering extracorporeal RRTs with medical failure in maintaining their consciousness and stable hemodynamics including sodium phenylbutyrate and arginine for urea cycle disorders, and carnitine and vitamin B 12 for organic acidemia. Different renal replacement modalities were performed to rescue neurological function and rapidly reduce the toxic metabolites (TMs) including ammonia or organic acid.…”

Section: Patientsmentioning

confidence: 99%

High-Volume Continuous Venovenous Hemofiltration as an Effective Therapy for Acute Management of Inborn Errors of Metabolism in Young Children

et al. 2007

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Background/Aim: Renal replacement therapies (RRTs) have been used for the acute management of inborn errors of metabolism. Hemodialysis is the most effective modality. The aim of this article is to demonstrate that high-volume hemofiltration can offer an alternative way to effectively remove small molecules. Methods: Eight patients presented with acute neurological deterioration due to ammonia or organic acid accumulation. Different RRTs were applied, including continuous venovenous hemofiltration (CVVH, n = 7), continuous arteriovenous hemofiltration (CAVH, n = 2), continuous venovenous hemodialysis (CVVHD, n = 1), intermittent hemodialysis (HD, n = 1), and peritoneal dialysis (PD, n = 2). Results: Ammonia 50% reduction time in HD was 1.7 h while in CVVH it was 2–14.5 h. The greater the ultrafiltration flow was, the sooner patients regained consciousness. CAVH, CVVHD or PD was not sufficient enough.Conclusion:CVVH also has a good clearance for organic acid and ammonia if applying high-volume hemofiltration (>35 ml/kg/h). It can be therefore be considered as an alternative therapy if infant HD is not available.

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Section: Patientsmentioning

confidence: 99%

High-Volume Continuous Venovenous Hemofiltration as an Effective Therapy for Acute Management of Inborn Errors of Metabolism in Young Children

et al. 2007

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“…Because of this impaired enzyme activity, leucine accumulates in cells and body fluids when the load exceeds leucine metabolism capacity and/or when a increased protein catabolism occurs. Given that renal clearance of BCAA and its derivatives is poor and due to the fact that their acute elevation causes neurologic deterioration, CECRT is indicated in severe cases and/or when nutritional support free of BCAA is not possible (2).…”

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confidence: 99%

Kinetic Modeling of Plasma Leucine Levels during Continuous Venovenous Extracorporeal Removal Therapy in Neonates with Maple Syrup Urine Disease

et al. 2005

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A kinetic modeling of leucine plasma concentration changes is proposed to describe the plasma leucine reduction rate during continuous extracorporeal removal therapy (CECRT) in neonates with maple syrup urine disease. Data were obtained from seven neonates using a bicompartmental model for the best fitted curve of plasma leucine decrease during CECRT. During the first 3 h, leucine plasma levels decreased according to an exponential curve: [Leu] -0.05t . Plasma leucine levels obtained from three other neonates were similar to those predicted by the model. The apparent distribution volumes for leucine that correspond to the two exponential equations obtained were calculated from the leucine mass removal collected in the spent dialysate and ultrafiltrate. The distribution volume was 34 Ϯ 3% of body weight during the first 3 h of CECRT and 72 Ϯ 7% from h 4 to the end of CECRT. These figures are similar to known values for the extracellular water compartment and for total body water in the newborn. The findings suggest that leucine handling during CECRT is similar to that of nonprotein-bound small-molecularweight solutes such as urea. MSUD is an inherited metabolic disease due to a deficiency in branched chain ketoacid dehydrogenase that leads to the accumulation of BCAA (leucine, isoleucine, and valine) and their keto-acid derivatives (1). Because of this impaired enzyme activity, leucine accumulates in cells and body fluids when the load exceeds leucine metabolism capacity and/or when a increased protein catabolism occurs. Given that renal clearance of BCAA and its derivatives is poor and due to the fact that their acute elevation causes neurologic deterioration, CECRT is indicated in severe cases and/or when nutritional support free of BCAA is not possible (2).Because assessment of leucine plasma levels by amino acid chromatography is not easily available and is time consuming, the aim of this study was to design a kinetic model that predicts leucine plasma level changes over time during the acute phase of the illness in neonates on CECRT. METHODS Patients.Between January 1991 and April 2000, seven neonates with MSUD presented with a severe acute deterioration of their status. After a 6-h period of conservative management that included correction of dehydration and increased caloric intake, all seven patients presented two of the three following criteria that are associated with a high risk of severe brain damage: comatose state, gastrointestinal intolerance, and leucine plasma levels Ն1700 M. The patients were referred to pediatric and neonatal intensive care for alternative therapies, in particular CECRT (3). Blood and spent dialysate samples were collected to monitor treatment efficiency. We retrospectively used these biologic analyses to elaborate the parameters involved in leucine kinetic modeling during CECRT (Table 1). Subsequently, between May 2000 and December 2001, three other neonates were treated with the same therapeutic strategy and were retrospectively studied to validate the leucine kinetic model...

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“…[14] However, ammonium clearance was lower than with CVVHD, as blood flow was hampered by hypotension. [15] This complication has been reported in 64% of neonates treated with hemodialysis. In CVVHD, the dialytic mass removal of ammonium was almost five times that of diuresis.…”

Section: Discussionmentioning

confidence: 99%

Continuous Hemodiafiltration in the Treatment of Hyperammonemia Due to Methylmalonic Acidemia

Chen¹,

Tsai²,

Lee³

et al. 2007

Renal Failure

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Methylmalonic academia (MMA) is a rare inborn error of branched-chain amino acid metabolism. Therapy consists of a special formulated protein diet, carnitine supplementation, and emergent detoxification during acute decompensation. Continuous hemodiafiltration is a modality choice to treat acute metabolic decompensation in inborn error of metabolism. We report the successful use of continuous hemodiafiltration in the management of acute decompensation in patients with methylmalonic academia. Three male patients were diagnosed with methylmalonic academia with the initial presentation of hyperammonemia. Continuous hemodiafiltration was utilized to treat acute decompensation of metabolic crisis. This approach results in a rapid reduction of systemic toxin levels. Continuous hemodiafiltration should be considered as a treatment modality for symptomatic neonates with MMA where hemodialysis is not feasible.

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Continuous venovenous hemofiltration in the management of acute decompensation in inborn errors of metabolism

Cited by 73 publications

References 8 publications

High-Volume Continuous Venovenous Hemofiltration as an Effective Therapy for Acute Management of Inborn Errors of Metabolism in Young Children

High-Volume Continuous Venovenous Hemofiltration as an Effective Therapy for Acute Management of Inborn Errors of Metabolism in Young Children

Kinetic Modeling of Plasma Leucine Levels during Continuous Venovenous Extracorporeal Removal Therapy in Neonates with Maple Syrup Urine Disease

Continuous Hemodiafiltration in the Treatment of Hyperammonemia Due to Methylmalonic Acidemia

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