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Exchange transfusion in acute episodes of maple syrup urine disease. Studies on branched-chain amino and keto acids.
Eur J Pediatr. 1982 Jul; 138(4):293-6.EJ

Abstract

Two neonates with maple syrup urine disease were treated by exchange transfusion. Within 15 h blood leucine and KICA concentrations were lowered from 2.6 mM to 1.1 mM using 570 to 620 ml blood per kg body weight. The other branched-chain amino acid/keto acid pairs fell to normal. During exchange transfusion the patient's nitrogen balance seems to be negative. Further exchange transfusion was useless. More importantly the patient should be forced into an anabolic state by high caloric supply or insulin plus glucose treatment. More KICA than leucine was eliminated, however, KICA blood levels remained slightly higher than that of leucine indicating different leucine/KICA equilibria in extravascular compartments than in blood. In a given time interval exchange transfusion was more effective than peritoneal dialysis, probably due to a lack of an additional (peritoneal) membrane. Renal excretion of branched-chain amino and keto acids was very inefficient. The allegedly most toxic metabolite, KICA, had the lowest renal clearance of the branched-chain keto acids.

Authors

No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

7128634

Citation

Wendel, U, et al. "Exchange Transfusion in Acute Episodes of Maple Syrup Urine Disease. Studies On Branched-chain Amino and Keto Acids." European Journal of Pediatrics, vol. 138, no. 4, 1982, pp. 293-6.
Wendel U, Langenbeck U, Lombeck I, et al. Exchange transfusion in acute episodes of maple syrup urine disease. Studies on branched-chain amino and keto acids. Eur J Pediatr. 1982;138(4):293-6.
Wendel, U., Langenbeck, U., Lombeck, I., & Bremer, H. J. (1982). Exchange transfusion in acute episodes of maple syrup urine disease. Studies on branched-chain amino and keto acids. European Journal of Pediatrics, 138(4), 293-6.
Wendel U, et al. Exchange Transfusion in Acute Episodes of Maple Syrup Urine Disease. Studies On Branched-chain Amino and Keto Acids. Eur J Pediatr. 1982;138(4):293-6. PubMed PMID: 7128634.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Exchange transfusion in acute episodes of maple syrup urine disease. Studies on branched-chain amino and keto acids. AU - Wendel,U, AU - Langenbeck,U, AU - Lombeck,I, AU - Bremer,H J, PY - 1982/7/1/pubmed PY - 1982/7/1/medline PY - 1982/7/1/entrez SP - 293 EP - 6 JF - European journal of pediatrics JO - Eur J Pediatr VL - 138 IS - 4 N2 - Two neonates with maple syrup urine disease were treated by exchange transfusion. Within 15 h blood leucine and KICA concentrations were lowered from 2.6 mM to 1.1 mM using 570 to 620 ml blood per kg body weight. The other branched-chain amino acid/keto acid pairs fell to normal. During exchange transfusion the patient's nitrogen balance seems to be negative. Further exchange transfusion was useless. More importantly the patient should be forced into an anabolic state by high caloric supply or insulin plus glucose treatment. More KICA than leucine was eliminated, however, KICA blood levels remained slightly higher than that of leucine indicating different leucine/KICA equilibria in extravascular compartments than in blood. In a given time interval exchange transfusion was more effective than peritoneal dialysis, probably due to a lack of an additional (peritoneal) membrane. Renal excretion of branched-chain amino and keto acids was very inefficient. The allegedly most toxic metabolite, KICA, had the lowest renal clearance of the branched-chain keto acids. SN - 0340-6199 UR - https://www.unboundmedicine.com/medline/citation/7128634/Exchange_transfusion_in_acute_episodes_of_maple_syrup_urine_disease__Studies_on_branched_chain_amino_and_keto_acids_ L2 - http://babysfirsttest.org/newborn-screening/conditions/maple-syrup-urine-disease-msud DB - PRIME DP - Unbound Medicine ER -