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Clearance of branched chain amino acids by peritoneal dialysis in maple syrup urine disease.
Adv Perit Dial. 1990; 6:31-4.AP

Abstract

Thirty-eight hours of peritoneal dialysis (PD) was used in an 8-day-old, 3.35 kg boy with maple syrup urine disease (MSUD) to enhance the removal of branched chain amino acids (BCAA). PD clearances for leucine, isoleucine, and valine were 3 ml/min/1.73 m2, compared to urinary clearances of 0.16 ml/min/1.73 m2 (0.4 ml/min/1.73 m2 for isoleucine). The augmented clearance was reflected by a faster rate of fall in the plasma leucine level during dialysis (38 microM/hr) than before or immediately after dialysis (10 microM/hr). The biochemical improvement was accompanied by neurologic improvement in the baby. Once the leucine level fell to below 1500 mu + M, the unaugmented rate of fall for leucine (21 microM/hr) was similar to that augmented by PD. Our experience demonstrates that PD is useful for enhancing clearance of BCAA at leucine levels greater than 1500 microM and that it speeds neurologic recovery.

Links

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Authors+Show Affiliations

McMahon Y
Division of Pediatric Nephrology, Vanderbilt University Medical Center, Nashville, Tennessee.
MacDonnell RC
No affiliation info available

MeSH

Amino Acids, Branched-ChainHumansInfant, NewbornMaleMaple Syrup Urine DiseasePeritoneal Dialysis

Pub Type(s)

Case Reports
Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

1982835

Citation

McMahon, Y, and R C. MacDonnell. "Clearance of Branched Chain Amino Acids By Peritoneal Dialysis in Maple Syrup Urine Disease." Advances in Peritoneal Dialysis. Conference On Peritoneal Dialysis, vol. 6, 1990, pp. 31-4.
McMahon Y, MacDonnell RC. Clearance of branched chain amino acids by peritoneal dialysis in maple syrup urine disease. Adv Perit Dial. 1990;6:31-4.
McMahon, Y., & MacDonnell, R. C. (1990). Clearance of branched chain amino acids by peritoneal dialysis in maple syrup urine disease. Advances in Peritoneal Dialysis. Conference On Peritoneal Dialysis, 6, 31-4.
McMahon Y, MacDonnell RC. Clearance of Branched Chain Amino Acids By Peritoneal Dialysis in Maple Syrup Urine Disease. Adv Perit Dial. 1990;6:31-4. PubMed PMID: 1982835.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Clearance of branched chain amino acids by peritoneal dialysis in maple syrup urine disease. AU - McMahon,Y, AU - MacDonnell,R C,Jr PY - 1990/1/11/pubmed PY - 2001/3/28/medline PY - 1990/1/11/entrez SP - 31 EP - 4 JF - Advances in peritoneal dialysis. Conference on Peritoneal Dialysis JO - Adv Perit Dial VL - 6 N2 - Thirty-eight hours of peritoneal dialysis (PD) was used in an 8-day-old, 3.35 kg boy with maple syrup urine disease (MSUD) to enhance the removal of branched chain amino acids (BCAA). PD clearances for leucine, isoleucine, and valine were 3 ml/min/1.73 m2, compared to urinary clearances of 0.16 ml/min/1.73 m2 (0.4 ml/min/1.73 m2 for isoleucine). The augmented clearance was reflected by a faster rate of fall in the plasma leucine level during dialysis (38 microM/hr) than before or immediately after dialysis (10 microM/hr). The biochemical improvement was accompanied by neurologic improvement in the baby. Once the leucine level fell to below 1500 mu + M, the unaugmented rate of fall for leucine (21 microM/hr) was similar to that augmented by PD. Our experience demonstrates that PD is useful for enhancing clearance of BCAA at leucine levels greater than 1500 microM and that it speeds neurologic recovery. SN - 1197-8554 UR - https://www.unboundmedicine.com/medline/citation/1982835/Clearance_of_branched_chain_amino_acids_by_peritoneal_dialysis_in_maple_syrup_urine_disease_ L2 - https://www.diseaseinfosearch.org/result/4453 DB - PRIME DP - Unbound Medicine ER -