Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids

Wendel, U.; Becker, Kathrin; Przyrembel, Hildegard; Bulla, M; Manegold, Christian; Mench-Hoinowski, A.; Langenbeck, U.

doi:10.1007/bf00442404

Cited by 33 publications

(17 citation statements)

References 17 publications

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“…The BCAA and a-ketoisocaproate blood levels obtained in our experimental model were close to those observed in neonates with MSUD when extracorporeal blood purification was required (Wendel et al 1980;Gortner et al 1989;Thompson et al 1991;Ring and Zobel 1992;Falk et al 1994). The epurative procedures were performed with a haemo(dia)filter and operational parameters already used in newborn infants.…”

Section: Discussionsupporting

confidence: 76%

“…The a-ketoisocaproate renal clearance is very low (Langenbeck et al 1979) and data about removal by peritoneal dialysis are scarce. Peritoneal clearance of a-ketoisocaproate in MSUD has been reported to be 0.62 ml/min in one neonate (Gortner et al 1989) and 1.1-1.9ml/min in an 18-month-old infant (Wendel et al 1980). The present study demonstrated that VVHDF gave higher a-ketoisocaproate clearances as compared with VVHE The a-ketoisocaproate clearances obtained with VVHDF at a 2L/h dialysate flow rate were 3.1 +0.7 and 5.0 + 1.2ml/min when Qb was 8.3 and 16.6 ml/min, respectively.…”

Section: Discussionmentioning

confidence: 93%

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Removal of branched‐chain amino acids andα‐ketoisocaproate by haemofiltration and haemodiafiltration

Semama

Prevot

et al. 1995

J of Inher Metab Disea

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Venovenous haemofiltration (VVHF) and haemodiafiltration (VVHDF) were performed with a neonatal haemo(dia)filter (Miniflow 10, Hospal) on 8 anaesthetized rabbits infused with branched-chain amino acids (leucine, isoleucine and valine) and alpha-ketoisocaproate. The branched-chain amino acids (BCAA) and alpha-ketoisocaproate blood levels were close to those previously observed in neonates with maple syrup urine disease when extracorporeal blood purification was required. VVHF and VVHDF performances were assessed with two different blood flows (Qb = 8.3 and 16.6 ml/min). VVHDF was performed with four dialysate flow rates (Qd = 0.5, 1.0, 2.0 and 3.0 L/h). Within each period, clearances of the three BCAA were strictly similar. BCAA clearances obtained by VVHF were similar to ultrafiltration rates (respectively, 0.78 +/- 0.14 and 1.79 +/- 0.28 ml/min at high and low Qb; p < 0.05). The alpha-ketoisocaproate clearances obtained by VVHF were 0.39 +/- 0.17 and 0.92 +/- 0.43 ml/min at low and high Qb (not significantly different). Whatever the Qd value, the VVHDF procedures always allowed higher BCAA and alpha-ketoisocaproate clearances as compared with the corresponding VVHF period with similar Qb. BCAA clearances obtained by VVHDF with a 0.5 L/h dialysate flow were 4.1 +/- 0.5 and 5.4 +/- 0.5 mL/min at low and high Qb, respectively. The concurrent alpha-ketoisocaproate clearances were 2.5 +/- 0.8 and 2.9 +/- 1.0 ml/min.

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Section: Discussionsupporting

confidence: 76%

Section: Discussionmentioning

confidence: 93%

Removal of branched‐chain amino acids andα‐ketoisocaproate by haemofiltration and haemodiafiltration

Semama

Prevot

et al. 1995

J of Inher Metab Disea

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“…The currently available renal replacement therapy modalities are peritoneal dialysis (PD), intermittent hemodialysis (HDi) and several forms of continuous extracorporeal removal therapy including continuous venovenous hemofiltration (CVVH), continuous venovenous hemodialysis (CVVHD) and CVVHDF. 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] .…”

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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“…Neurological symptoms arise when a-ketoisocaproate and leucine blood levels exceed 1 mM. Extremely high blood and tissue levels of branched-chain amino and keto acids require urgent treatment by peritoneal dialysis or exchange transfusion [6,10,20,21].…”

Section: Introductionmentioning

confidence: 99%

Maple syrup urine disease-therapeutic use of insulin in catabolic states

et al. 1982

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High and neurotoxic blood levels of leucine and its ketoanalogue develop in catabolic patients with maple syrup urine disease. The use of relatively high doses of insulin and additional glucose had a more pronounced effect on lowering leucine (and alpha-ketoisocaproate) blood levels than dietary elimination of leucine alone. This is demonstrated in 2 neonates after blood exchange transfusion and in one 4-months old patient suffering from febrile diarrhea.

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Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids

Abstract: Abstract. We report biochemical data on a child with MSUD who underwent peritoneal ,dialysis for severe metabolic imbalance.

Cited by 33 publications

References 17 publications

Removal of branched‐chain amino acids andα‐ketoisocaproate by haemofiltration and haemodiafiltration

Removal of branched‐chain amino acids andα‐ketoisocaproate by haemofiltration and haemodiafiltration

Continuous Venovenous Hemodiafiltration in the Treatment of Maple Syrup Urine Disease

Maple syrup urine disease-therapeutic use of insulin in catabolic states

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