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Dietary therapy in two patients with vitamin B12-unresponsive methylmalonic acidemia.

Authors
  • Satoh, T
  • Narisawa, K
  • Igarashi, Y
  • Saitoh, T
  • Hayasaka, K
  • Ichinohazama, Y
  • Onodera, H
  • Tada, K
  • Oohara, K
Type
Published Article
Journal
European journal of pediatrics
Publication Date
Feb 01, 1981
Volume
135
Issue
3
Pages
305–312
Identifiers
PMID: 7227387
Source
Medline
License
Unknown

Abstract

The biochemical and therapeutic responses to dietary therapy were studied in a 25-month-old girl and a 1-month-old girl with methylmalonic acidemia (MMA-emia), which was unresponsive to vitamin B12. The minimum daily intake of protein which patients could tolerate and display a good development was between 1.0 and 1.2 g per kg body weight. Supplementation with amino acid mixture devoid of toxic amino acids was required to prevent protein malnutrition when daily protein intake was restricted to 0.6 g per kg body weight. Caloric intake should be sufficient, not only to promote growth but also to prevent a rise in MMA level, especially when a patient has ketoacidosis. It was found that MMA excretion per mg creatinine in random urine specimens correlated significantly with serum MMA and twenty four-hour output of MMA per kg body weight. Therefore measurement of MMA in a single urine specimen is useful for evaluating the in vivo accumulation of MMA.

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