TY - JOUR
T1 - Improved Growth and Nutrition Status in Children with Methylmalonic or Propionic Acidemia Fed an Elemental Medical Food
AU - Yannicelli, Steven
AU - Acosta, Phyllis B.
AU - Velazquez, Antonio
AU - Bock, Hans-Georg
AU - Marriage, Barbara J.
AU - Kurczynski, Thaddeus W.
AU - Miller, Marvin E.
AU - Korson, Mark
AU - Steiner, Robert D.
AU - Rutledge, Lane
AU - Bernstein, Laurie
AU - Chinsky, Jeffrey
AU - Galvin-Parton, Patricia
AU - Arnold, Georgianne L.
PY - 2003/1/1
Y1 - 2003/1/1
N2 - 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 <6-month-old, 6 < 12-month-old, and 1< 4-year-old patients were 15.3 ± 0.9 g and 645 ± 10 kcal; 18.3 ± 1.1 g and 741 ± 92 kcal; and 25.1 ± 2.46 g and 1062 ± 100 kcal, respectively. Plasma glycine concentrations were significantly and negatively correlated with energy intake ( r =−0.77, p <0.0005). No correlation was found between dietary protein intakes and plasma ammonia concentrations. Protein status indices, retinol and α-tocopherol concentrations were within reference ranges at study end. Conclusions: Propimex-1 improved growth and nutrition status in patients with MMA or PA in just 6 months when fed in sufficient amounts. Providing energy and protein for patients with FTT at intakes recommended for catch-up growth may have resulted in even better growth.
AB - 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 <6-month-old, 6 < 12-month-old, and 1< 4-year-old patients were 15.3 ± 0.9 g and 645 ± 10 kcal; 18.3 ± 1.1 g and 741 ± 92 kcal; and 25.1 ± 2.46 g and 1062 ± 100 kcal, respectively. Plasma glycine concentrations were significantly and negatively correlated with energy intake ( r =−0.77, p <0.0005). No correlation was found between dietary protein intakes and plasma ammonia concentrations. Protein status indices, retinol and α-tocopherol concentrations were within reference ranges at study end. Conclusions: Propimex-1 improved growth and nutrition status in patients with MMA or PA in just 6 months when fed in sufficient amounts. Providing energy and protein for patients with FTT at intakes recommended for catch-up growth may have resulted in even better growth.
KW - Growth
KW - Methylmalonic Acidemia
KW - Nutrition
KW - Propionic Acidemia
KW - Retinol
UR - https://corescholar.libraries.wright.edu/pediatrics/7
U2 - 10.1016/j.ymgme.2003.08.012
DO - 10.1016/j.ymgme.2003.08.012
M3 - Article
VL - 80
JO - Molecular Genetics and Metabolism
JF - Molecular Genetics and Metabolism
ER -