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, Galvin-Parton Patricia, Arnold Georgianne L
Ross Products Division, Abbott Laboratories, 625 Cleveland Avenue, Columbus, OH 43215-1724, USA.
Mol Genet Metab. 2003 Sep-Oct;80(1-2):181-8. doi: 10.1016/j.ymgme.2003.08.012.
Failure-to-thrive (FTT) has been described in patients with organic acidemias treated with low protein diets.
To determine if patients with methylmalonic (MMA) or propionic acidemia (PA) can achieve normal growth and nutrition status.
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 alpha-tocopherol.
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 alpha-tocopherol concentrations were within reference ranges at study end.
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.
在采用低蛋白饮食治疗的有机酸血症患者中曾有生长发育不良（FTT）的描述。
确定患有甲基丙二酸血症（MMA）或丙酸血症（PA）的患者能否实现正常生长和营养状况。
对使用含铁血氨酸改性医用食品Propimex-1（罗斯产品部，雅培实验室，俄亥俄州哥伦布市）治疗的婴幼儿进行了一项为期6个月的多中心门诊研究。主要结局指标为人体测量学指标、蛋白质状态指标、血浆视黄醇和α-生育酚。
16名患者完成了研究。平均基线年龄为0.54±0.02岁（范围0.03 - 3.00岁）。到研究结束时，美国国家卫生统计中心（NCHS）平均体重百分位从26%增至49%；平均顶跟身长百分位从25%增至33%；平均头围百分位从43%增至54%。年龄＜6个月、6 - 12个月和1 - 4岁患者的平均（±标准误）蛋白质和能量摄入量分别为15.3±0.9 g和645±10 kcal；18.3±1.1 g和741±92 kcal；以及25.1±2.46 g和1062±100 kcal。血浆甘氨酸浓度与能量摄入呈显著负相关（r = -0.77，p＜0.0005）。未发现膳食蛋白质摄入量与血浆氨浓度之间存在相关性。研究结束时蛋白质状态指标、视黄醇和α-生育酚浓度均在参考范围内。
当给予足够量的Propimex-1时，其在6个月内改善了MMA或PA患者的生长和营养状况。按照追赶生长推荐摄入量为FTT患者提供能量和蛋白质可能会带来更好的生长效果。
