| Objective:To analysis nutritional problem in children with phenylketonuria treated with dietotherapy, so as to provide a basis for intervention.Methods:A historical cohort study was performed in children with phenylketonuria detected by neonatal screening in Qingdao.46 children with phenylketonuria who had been born from December 2003 to June 2009 were studied and compared to 61 matched children born at the same time. Physical and mental development of the children was evaluated on the basis of growth, blood analysis, trace element, blood biochemical parameters, analysis of amino acids in serum respectively. Self-made inventory questionnaire and feeding behavior questionnaire are used to investigate the related factors of the two groups.Results:①After birth, the children with phenylketonuria was significantly higher than the control group in eczema, feeding difficulties and vomiting(p<0.01).②The children with phenylketonuria was significantly higher than the control group in cooking single-handedly for children, dining with adults, Snatching adult food(p<0.01).③Birth height,birth weight, current height and current weight were comparable to that of the controls(p>0.05).④The blood Zn,Cu in children with phenylketonuria were lower than that of the normal controls(p<0.05). Zinc deficiency in children with phenylketonuria was significantly higher than the normal controls(x2=7.017, p=0.008). The blood Ca,Mg,Fe in children with phenylketonuria were comparable to that of the controls(p>0.05).⑤There was no significant differences in heamoglobin(Hb) between children with phenylketonuria and the controls(p>0.05).⑥There were differences in serum total protein, serum albumin(p<0.05) and significant differences in serum globulin(p<0.01).The blood ALT,AST,CHO,TG,HDL,LDL were comparable to that of the controls(p>0.05).⑦There were significant differences in blood aspartic acid, methionine, phenylalanine, argine, citrulline, glycine, ornithine between children with phenylketonuria and the controls(p<0.01).The blood alanine, glutamic acid, tyrosine, leucine, valine, free carnitine, acetylcarnitine, propionylcarnitine and butylcarnitine were no significant differences between the two groups(p>0.05).Conclusion:The present findings strongly suggest that there were nutrient deficiencies and feeding behavior problems in children with phenylketonuria, though having been early-diagnosed and early-treate. Timely identification and correction of the nutrient deficiencies and feeding behavior problems in children with phenylketonuria are important. To seek the best treatment program is the direction of our efforts. |
