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

Steven Yannicelli, Phyllis B. Acosta, Antonio Velazquez, Hans Georg Bock, Barbara Marriage, Thaddeus W. Kurczynski, Marvin Miller, Mark Korson, Robert D. Steiner, Lane Rutledge, Laurie Bernstein, Jeffrey M Chinsky, Patricia Galvin-Parton, Georgianne L. Arnold

Research output: Contribution to journalArticle

Abstract

Background: Failure-to-thrive (FTT) has been described in patients with organic acidemias treated with low protein diets. Objective: To determine if patients with methylmalonic (MMA) or propionic acidemia (PA) can achieve normal growth and nutrition status. Methods: A 6-month multicenter outpatient study was conducted with infants and toddlers treated with Propimex-1 Amino Acid-Modified Medical Food With Iron (Ross Products Division, Abbott Laboratories, Columbus, OH). Main outcome measures were anthropometrics, protein status indices, plasma retinol, and α-tocopherol. Results: Sixteen patients completed the study. Mean baseline age was 0.54±0.02 years (range 0.03-3.00 years). By study end, mean National Center for Health Statistics (NCHS) weight centile increased from 26 to 49%; mean crown-heel length centile from 25 to 33%; and mean head circumference centile from 43 to 54%. Mean (±SE) protein and energy intakes by

Original languageEnglish (US)
Pages (from-to)181-188
Number of pages8
JournalMolecular Genetics and Metabolism
Volume80
Issue number1-2
DOIs
StatePublished - Sep 2003

Fingerprint

Propionic Acidemia
Nutrition
Nutritional Status
Food
Growth
National Center for Health Statistics (U.S.)
Failure to Thrive
Protein-Restricted Diet
Proteins
Tocopherols
Heel
Energy Intake
Crowns
Vitamin A
Multicenter Studies
Outpatients
Iron
Head
Outcome Assessment (Health Care)
Health

Keywords

  • α-Tocopherol
  • Growth
  • Methylmalonic acidemia
  • Nutrition
  • Propionic acidemia
  • Retinol

ASJC Scopus subject areas

  • Biochemistry
  • Genetics
  • Endocrinology, Diabetes and Metabolism

Cite this

Yannicelli, S., Acosta, P. B., Velazquez, A., Bock, H. G., Marriage, B., Kurczynski, T. W., ... Arnold, G. L. (2003). Improved growth and nutrition status in children with methylmalonic or propionic acidemia fed an elemental medical food. Molecular Genetics and Metabolism, 80(1-2), 181-188. https://doi.org/10.1016/j.ymgme.2003.08.012

Improved growth and nutrition status in children with methylmalonic or propionic acidemia fed an elemental medical food. / Yannicelli, Steven; Acosta, Phyllis B.; Velazquez, Antonio; Bock, Hans Georg; Marriage, Barbara; Kurczynski, Thaddeus W.; Miller, Marvin; Korson, Mark; Steiner, Robert D.; Rutledge, Lane; Bernstein, Laurie; Chinsky, Jeffrey M; Galvin-Parton, Patricia; Arnold, Georgianne L.

In: Molecular Genetics and Metabolism, Vol. 80, No. 1-2, 09.2003, p. 181-188.

Research output: Contribution to journalArticle

Yannicelli, S, Acosta, PB, Velazquez, A, Bock, HG, Marriage, B, Kurczynski, TW, Miller, M, Korson, M, Steiner, RD, Rutledge, L, Bernstein, L, Chinsky, JM, Galvin-Parton, P & Arnold, GL 2003, 'Improved growth and nutrition status in children with methylmalonic or propionic acidemia fed an elemental medical food', Molecular Genetics and Metabolism, vol. 80, no. 1-2, pp. 181-188. https://doi.org/10.1016/j.ymgme.2003.08.012
Yannicelli, Steven ; Acosta, Phyllis B. ; Velazquez, Antonio ; Bock, Hans Georg ; Marriage, Barbara ; Kurczynski, Thaddeus W. ; Miller, Marvin ; Korson, Mark ; Steiner, Robert D. ; Rutledge, Lane ; Bernstein, Laurie ; Chinsky, Jeffrey M ; Galvin-Parton, Patricia ; Arnold, Georgianne L. / Improved growth and nutrition status in children with methylmalonic or propionic acidemia fed an elemental medical food. In: Molecular Genetics and Metabolism. 2003 ; Vol. 80, No. 1-2. pp. 181-188.
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