Zeman L, Zeman J, Kozich V, Matousová M, Velísková J
Centrum dĕdicných metabolických poruch fakultní nemocnice 2 s fakultní poliklinikou, Praha.
Cesk Pediatr. 1991 Jan;46(1):19-22.
The phosphoethanolamine (PEA) concentration in morning urine was assessed by liquid chromatography in 866 patients examined because of suspected impaired aminoacid metabolism. In 763 patients the blood was also examined. A serum concentration of PEA above 10 mumol/l was recorded in 2.1%. The concentration of PEA in urine above 10 mmol/mol creatinine was recorded in 42%. The authors revealed a significant relationship between urinary PEA excretion and the patient's age. The urinary PEA concentrations are higher during the first weeks and months of life, in older children and during adolescence its excretion declines and this trend was recorded also in the author's group of patients. In a group of 111 infants with impaired function of the CNS, in 66 infants with systemic skeletal affections and in 73 infants with hepatopathies a significantly higher mean urinary PEA concentration was found that in the control group of healthy infants. In children aged 3-14 years and in the group of older children the mean PEA concentration was elevated only in patients with systemic skeletal affections, even after elimination of patients with hypophosphatasia. Long-term or intermittently increased PEA excretion is a manifestation of specific metabolic disease only in hypophosphatasia. In other diseases it can be interpreted as a secondary finding conditioned by an impaired phospholipid metabolism at the level of cellular membranes in the CNS, liver or skeleton.
采用液相色谱法对866例因怀疑氨基酸代谢受损而接受检查的患者晨尿中的磷酸乙醇胺(PEA)浓度进行了评估。其中763例患者还进行了血液检查。血清PEA浓度高于10μmol/L的患者占2.1%。尿中PEA浓度高于10mmol/mol肌酐的患者占42%。作者发现尿中PEA排泄与患者年龄之间存在显著相关性。在生命的最初几周和几个月里,尿中PEA浓度较高,在大龄儿童和青少年期其排泄量下降,作者的患者组中也记录到了这种趋势。在111例中枢神经系统功能受损的婴儿、66例全身性骨骼疾病婴儿和73例肝病婴儿中,发现其尿PEA平均浓度显著高于健康婴儿对照组。在3 - 14岁儿童以及大龄儿童组中,仅全身性骨骼疾病患者的PEA平均浓度升高,即使排除了低磷酸酯酶症患者也是如此。长期或间歇性PEA排泄增加仅在低磷酸酯酶症中是特定代谢疾病的表现。在其他疾病中,它可被解释为中枢神经系统、肝脏或骨骼细胞膜水平磷脂代谢受损所导致的继发发现。