Improved growth and nutrition status in children with methylmalonic or propionic acidemia fed an elemental medical food.

@article{Yannicelli2003ImprovedGA,
  title={Improved growth and nutrition status in children with methylmalonic or propionic acidemia fed an elemental medical food.},
  author={Steven Yannicelli and Phyllis B. Acosta and Antonio Vel{\'a}zquez and Hans-georg Bock and Barbara J. Marriage and Thaddeus W. Kurczynski and Marvin Miller and Mark S. Korson and Robert D. Steiner and Lane S. Rutledge and Laurie Bernstein and Jeffrey M. Chinsky and Patricia Galvin-Parton and Georgianne Arnold},
  journal={Molecular genetics and metabolism},
  year={2003},
  volume={80 1-2},
  pages={
          181-8
        }
}
The Nutritional Intake of Patients with Organic Acidaemias on Enteral Tube Feeding: Can We Do Better?
TLDR
In MMA/PA, energy intake decreases over time, weight gain accelerates, but linear height is poor, and the quality of long-term diet in these conditions deserves more attention.
Variable dietary management of methylmalonic acidemia: metabolic and energetic correlations.
TLDR
The spectrum of nutritional approaches used to treat patients with MMA, measure REE, and analyze the dependence of REE on body composition, biochemical, and nutritional variables were documented to help optimize nutrition and protect individuals from overfeeding.
Nutrition therapy of organic acidaemias with amino acid-based formulas: Emphasis on methylmalonic and propionic acidaemia
TLDR
Although protein requirements in patients with organic acidaemias have not been elucidated, providing an adequate balance of protein, energy and other nutrients will help promote growth.
A critical reappraisal of dietary practices in methylmalonic acidemia raises concerns about the safety of medical foods. Part 1: Isolated methylmalonic acidemias (MMA)
TLDR
Increased leucine intake in patients with MMA resulted in iatrogenic amino acid deficiencies and was associated with adverse growth outcomes and medical foods for propionate oxidation disorders need to be redesigned and studied prospectively to ensure efficacy and safety.
Dietary Treatment from Birth to Pregnancy in a Woman with Methylmalonic Aciduria
TLDR
This successful long-term follow-up of a patient with MMA from childhood, throughout pregnancy, delivery, and postpartum confirms that careful clinical, biochemical, and dietetic monitoring is crucial to ensure a favourable outcomes in MMA.
Clinical Course and Nutritional Management of Propionic and Methylmalonic Acidemias
TLDR
The findings indicate that implementation of NBS, vigilant and proactive management of decompensation episodes, and pursuing normal ammonia levels during monitoring can help patients achieve a better neurological prognosis.
Dietary practices in methylmalonic acidaemia: a European survey
TLDR
A high percentage of centres used PFAA in MMA patients together with a protein prescription that provided less than the safe levels of natural protein intake, however, there was inconsistent practices across Europe.
Propionic acidemia: clinical course and outcome in 55 pediatric and adolescent patients
TLDR
The data show that the outcome of propionic acidemia is still unfavourable, in spite of improved clinical management, and that Impairment of neurocognitive development is of special concern.
Methylmalonic and propionic acidurias: Management without or with a few supplements of specific amino acid mixture
TLDR
81 patients (51 MMA and 30 PA) diagnosed between 1988 and 2005 are reported in more detail, with severe forms followed for more than 3 years, and all patients but one have normal mental development.
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Evaluation of branched-chain amino acid intake in children with maple syrup urine disease and methylmalonic aciduria
TLDR
In two children with a disorder of branched-chain amino acid metabolism, maple syrup urine disease, and another with methylmalonic aciduria leucine intake, which gave normal growth without toxicity, was always judged to be less than the FAO/WHO recommendations.
Comparison of an elemental and polymeric enteral diet in patients with normal gastrointestinal function.
TLDR
The present findings provide no evidence that chemically defined 'elemental' diets containing free amino acids as their nitrogen source are in any way superior to polymeric diets containing whole protein and fat when administered to patients with normal gastrointestinal function.
Resting energy expenditure in disorders of propionate metabolism.
TLDR
REE in patients with disorders of propionate metabolism is reduced when they are well, representing 80% +/- 18% of that predicted by the Schofield height and weight equation.
Effect of Dosage and Timing of Amino Acid Mixtures on Nitrogen Retention in Patients with Phenylketonuria
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The main source of nitrogen for patients with phenylketonuria is a phenylala-nine-free amino acid mixture, and a complete anabolic metabolization of these large amounts of amino acids is impossible.
Propionic acidaemia: clinical, biochemical and therapeutic aspects
Comprehensive data on 30 patients with propionic acidaemia, diagnosed by selective screening for inborn errors of metabolism, are presented. The most valuable diagnostic metabolites found were
Parenteral nutrition in propionic and methylmalonic acidemia.
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TLDR
The transient depression in plasma Retinol produced by subclinical infection increased the number of at-risk children by 10% and 56% for plasma retinol concentrations <0.7 and < 0.35 micromol/L, respectively, and dietary inadequacy may be responsible for retInol concentrations being approximately 16% lower in Pakistani children than in children in the United Kingdom, where dietary vitamin A is adequate.
Long-term follow-up of 77 patients with isolated methylamalonic acidaemia
TLDR
Evaluated critically the clinical outcome in patients with MMA-aemia in relation to the type of defect, quality of treatment and degree of metabolic control, which includes 77 patients from 7 European countries whose fibroblasts were referred to us for enzyme analysis during the last 20 years.
Increased Urinary Metabolite Excretion during Fasting in Disorders of Propionate Metabolism
TLDR
Increases in metabolite excretion are most easily explained by mobilization and oxidation of odd-chain fatty acids in the fasting state and should be avoided in children with disorders of propionate metabolism.
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