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
AU - Kurczynski, Thaddeus W.
AU - Miller, Marvin
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
Y1 - 2003
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±0.9g and 645±10kcal; 18.3±1.1g and 741±92kcal; and 25.1±2. 46g and 1062±100kcal, 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±0.9g and 645±10kcal; 18.3±1.1g and 741±92kcal; and 25.1±2. 46g and 1062±100kcal, 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
KW - α-Tocopherol
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U2 - 10.1016/j.ymgme.2003.08.012
DO - 10.1016/j.ymgme.2003.08.012
M3 - Article
C2 - 14567967
AN - SCOPUS:0142089740
SN - 1096-7192
VL - 80
SP - 181
EP - 188
JO - Biochemical Medicine and Metabolic Biology
JF - Biochemical Medicine and Metabolic Biology
IS - 1-2
ER -