Neonatal hemodialysis: Effective therapy for the encephalopathy of inborn errors of metabolism

Rutledge, S. Lane; Havens, Peter L.; Haymond, Morey W.; McLean, Robert H.; Kan, Jean S.; Brusilow, Saul W.

doi:10.1016/s0022-3476(05)81661-0

Cited by 93 publications

(42 citation statements)

References 12 publications

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“…The blood flow was usually set at around 6-9 ml/kg/min in the small infant. In 1990, Rutledge et al [8] used a Trave- nol extracorporeal single-patient dialysate delivery system (SPS) to set the blood flow rate at 20 ml/min and the dialysate flow at 500 ml/min in a 3-kg infant with maple syrup urine disease. The leucine 50% reduction rate was around 3.1 h. Compared to another report [9] , in which the blood flow rate was 5 ml/min and dialysate flow was 100 ml/min, the leucine 50% reduction rate was 16-36 h in 3 small infants.…”

Section: Discussionmentioning

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: Discussionmentioning

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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“…PD was the method of choice for BCAA clearance during 1980s [6] . In early 1990s, the authors demonstrated that CVVH could decrease leucine level from 3,000-4,000 to 500-1,000 μmol/l in neonates within 12 h [7] . After Gouyon et al [8] showed that with CRRT 100-150% higher BCAA removal rates was possible than PD, CRRT was implemented as the standard care for life threatening conditions in MSUD.…”

Section: Discussionmentioning

confidence: 99%

“…Because of avid proximal tubular reabsorption of filtered BCAAs, endogenous renal clearance of BCAAs is limited and accounts for <0.3% of the total turnover, extracorporeal removal therapy is indicated in severe metabolic crisis [5] . However, there is no consensus about the choice of extracorporeal removal modalities; it is influenced by several factors including the efficacy of extracorporeal removal, advantages and disadvantages of each modality and institutional expertise [6][7][8][9][10][11][12][13] . In our 2 institu-tions, MSUD metabolic crisis resistant to nutritional support is treated with continuous venovenous hemodiafiltration (CVVHDF).…”

Section: Introductionmentioning

confidence: 99%

Continuous Venovenous Hemodiafiltration in the Treatment of Maple Syrup Urine Disease

Demirkol

Şık²,

Topal³

et al. 2016

Blood Purif

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Background: The study aims to define the efficacy of continuous renal replacement therapy in acute metabolic decompensation treatment of maple syrup urine disease (MSUD). Methods: All the neonates, infants and children who have had life threatening conditions due to MSUD and were treated with continuous venovenous hemodiafiltration (CVVHDF) were analyzed retrospectively. Results: Fourteen patients underwent 15 sessions of CVVHDF (age range 15 days to 87 months, mean 40.8 ± 31.4 months). One patient required additional CVVHDF 1 week after cessation of CVVHDF. Twenty seven percent (n = 4) of the patients were intubated and mechanically ventilated. Twelve patients responded to treatment and dramatic neurological improvement was observed within 24 h. Two of the 14 patients required 36 h of CVVHDF for neurological improvement. The mean duration of CVVHDF was 20.2 ± 8.6 (9-36) h. The mean leucine level was 1,648 ± 623.8 (714-2,768) μmol/l before and was 256.5 ± 150.6 (117-646) μmol/l at the end of treatment. No mortality was observed. Conclusion: Continuous hemodiafiltration is an effective and safe method in correcting metabolic disturbances in MSUD.

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“…Megavitamin therapy with biotin and cyanocobalamin, and carnitine supplementation are adjunctive therapies whilst patients with mitochondrial disorders may bene®t from dichloroacetate, thiamine, ascorbic acid, and/or ubidecarenone (coenzyme Q10). Haemodialysis is an option for patients who continue to deteriorate biochemically and neurologically [42].…”

Section: Additional Measures For Metabolic Disordersmentioning

confidence: 99%

Chronic cardiac failure in children due to dilated cardiomyopathy: diagnostic approach, pathophysiology and management

Venugopalan¹,

Agarwal²,

Worthing

2000

European Journal of Pediatrics

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Current understanding of the pathophysiology of chronic congestive cardiac failure resulting from dilated cardiomyopathy has shifted management strategy from steps that directly improve myocardial function to those that modulate the neuroendocrine profile and peripheral vascular reactivity. Similar advances in therapeutic applications would be assisted by controlled studies and full licensing of drugs for use in children. Medical intervention will remain the cornerstone of management until advances in surgical techniques become more widely available.

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Neonatal hemodialysis: Effective therapy for the encephalopathy of inborn errors of metabolism

Cited by 93 publications

References 12 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

Continuous Venovenous Hemodiafiltration in the Treatment of Maple Syrup Urine Disease

Chronic cardiac failure in children due to dilated cardiomyopathy: diagnostic approach, pathophysiology and management

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