Manz F, Schmidt H, Schärer K, Bickel H
Pediatr Res. 1977 Oct;11(10 Pt 2):1084-7.
Blood acid-base status, serum electrolytes, and urine pH were examined in 64 infants and children with phenylketonuria (PKU) treated with three different low phenylalanine protein hydrolyzates (Aponti, Cymogran, AlbumaidXP) and two synthetic amino acid mixtures (Aminogran, PAM). The formulas caused significant differences in acid-base status, serum potassium, and chloride, and in urine pH. In acid-base balance studies in two patients with PKU, Aponti, PAM, and two modifications of PAM (P2 + P3) were given. We observed a change from mild alkalosis to increasing metabolic acidosis from Aponti (serum bicarbonate 25,8 mval/liter) to P3 (24,0Y, P2 (19, 3) and PAM (17,0). Urine pH decreased and renal net acid excretion increased. In the formulas PAM, P2 and P3 differences in renal net acid excretion correlated with differences in chloride and sulfur contents of the diets and of the urines. New modifications of AlbumaidXP and of PAM, prepared according to our recommendations, showed normal renal net acid excretion (1 mEq/kg/24 hr) in a balance study performed in one patient with PKU and normal acid base status in 20 further patients.
对64例接受三种不同的低苯丙氨酸蛋白水解物（阿庞蒂、西莫格兰、白蛋白XP）和两种合成氨基酸混合物（氨基格兰、PAM）治疗的苯丙酮尿症（PKU）婴幼儿和儿童进行了血液酸碱状态、血清电解质和尿液pH值检查。这些配方在酸碱状态、血清钾和氯以及尿液pH值方面造成了显著差异。在对两名PKU患者进行的酸碱平衡研究中，给予了阿庞蒂、PAM以及PAM的两种改良配方（P2 + P3）。我们观察到，从阿庞蒂（血清碳酸氢盐25.8毫当量/升）到P3（24.0）、P2（19.3）和PAM（17.0），出现了从轻度碱中毒到代谢性酸中毒加重的变化。尿液pH值降低，肾脏净酸排泄增加。在PAM、P2和P3配方中，肾脏净酸排泄的差异与饮食和尿液中氯和硫含量的差异相关。根据我们的建议制备的白蛋白XP和PAM的新改良配方，在对一名PKU患者进行的平衡研究中显示肾脏净酸排泄正常（1毫当量/千克/24小时），在另外20名患者中酸碱状态正常。
