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Maple syrup urine disease: treatment of the acutely ill newborn.
Eur J Pediatr. 1978 Oct 12; 129(3):157-65.EJ

Abstract

Three patients with maple syrup urine disease were treated during the acute neonatal stage. Multiple exchange transfusions proved to be a satisfactory means of achieving rapid clinical and biochemical improvement during this phase. On the other hand, evidence is provided suggesting that in addition to exchange transfusions, a high calorie intake above 150 Cal/kg body weight/day is necessary to lower the plasma concentration of the branched chain amino acids to near-normal levels. As long as this calorie intake was not provided, further exchange transfusions failed to lower the plasma leucine concentration to below 17 mg/100 ml in one patient. It is assumed that this high calorie intake is necessary to prevent the breakdown of endogenous protein. Treatment of acute episodes in maple syrup urine disease should therefore not only eliminate the elevated alpha-keto acids and branched chain amino acids quickly (i.e. by multiple exchange transfusions or peritoneal dialysis), but in addition should provide a high calorie intake.

Authors

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Pub Type(s)

Journal Article

Language

eng

PubMed ID

699921

Citation

Hammersen, G, et al. "Maple Syrup Urine Disease: Treatment of the Acutely Ill Newborn." European Journal of Pediatrics, vol. 129, no. 3, 1978, pp. 157-65.
Hammersen G, Wille L, Schmidt H, et al. Maple syrup urine disease: treatment of the acutely ill newborn. Eur J Pediatr. 1978;129(3):157-65.
Hammersen, G., Wille, L., Schmidt, H., Lutz, P., & Bickel, H. (1978). Maple syrup urine disease: treatment of the acutely ill newborn. European Journal of Pediatrics, 129(3), 157-65.
Hammersen G, et al. Maple Syrup Urine Disease: Treatment of the Acutely Ill Newborn. Eur J Pediatr. 1978 Oct 12;129(3):157-65. PubMed PMID: 699921.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Maple syrup urine disease: treatment of the acutely ill newborn. AU - Hammersen,G, AU - Wille,L, AU - Schmidt,H, AU - Lutz,P, AU - Bickel,H, PY - 1978/10/12/pubmed PY - 1978/10/12/medline PY - 1978/10/12/entrez SP - 157 EP - 65 JF - European journal of pediatrics JO - Eur J Pediatr VL - 129 IS - 3 N2 - Three patients with maple syrup urine disease were treated during the acute neonatal stage. Multiple exchange transfusions proved to be a satisfactory means of achieving rapid clinical and biochemical improvement during this phase. On the other hand, evidence is provided suggesting that in addition to exchange transfusions, a high calorie intake above 150 Cal/kg body weight/day is necessary to lower the plasma concentration of the branched chain amino acids to near-normal levels. As long as this calorie intake was not provided, further exchange transfusions failed to lower the plasma leucine concentration to below 17 mg/100 ml in one patient. It is assumed that this high calorie intake is necessary to prevent the breakdown of endogenous protein. Treatment of acute episodes in maple syrup urine disease should therefore not only eliminate the elevated alpha-keto acids and branched chain amino acids quickly (i.e. by multiple exchange transfusions or peritoneal dialysis), but in addition should provide a high calorie intake. SN - 0340-6199 UR - https://www.unboundmedicine.com/medline/citation/699921/Maple_syrup_urine_disease:_treatment_of_the_acutely_ill_newborn_ L2 - https://www.diseaseinfosearch.org/result/4453 DB - PRIME DP - Unbound Medicine ER -