CAJ | 학술논문

【目的】为提高苯丙酮尿症(PKU)患儿的诊治水平，加强和改善患儿的治疗效果。【方法】对北京市1993～1999年经过新生儿筛查确诊的43例PKU患儿，低苯丙氨酸饮食的监测结果进行分析。【结果】43例 PKU患儿，除4例放弃治疗、1例死亡之外，治疗的38例患儿，有28例智能发育达正常同龄儿水平(73.7％)，有10例智能发育落后(26.3％)。生后40天内即开始治疗的PKU患儿，智能发育均达正常。而家长主动配合治疗的19例患儿中，18例智能发育正常，1例出现智能发育落后。【结论】患儿开始治疗时间的早晚及家长配合治疗程度直接影响患儿治疗效果。若患儿能在生后一个月内治疗，同时家长给予配合，患儿智力低下的发生是可以避免的。
【목적】위제고분병동뇨증(PKU)환인적진치수평，가강화개선환인적치료효과。【방법】대북경 시1993～1999년경과신생인사사학진적43례PKU환인，저분병안산음식적감측결과진행분석。【결과】43례 PKU환인，제4례방기치료、1례사망지외，치료적38례환인，유28례지능발육체정상동령인수평(73.7％)，유10례지 능발육락후(26.3％)。생후40천내즉개시치료적PKU환인，지능발육균체정상。이가장주동배합치료적19례환인 중，18례지능발육정상，1례출현지능발육락후。【결론】환인개시치료시간적조만급가장배합치료정도직접영향 환인치료효과。약환인능재생후일개월내치료，동시가장급여배합，환인지력저하적발생시가이피면적。
【Objective】 To improve the diagnosis and curative effect for PKU children 【Methods】 To analyse result treated by low phenylalanine diet therapy in 43 cases of PKU children chosen with neonetal screening from 1993 to 1999 in Beijing. Results As for the 43 cases,except that 4 cases had given up treatment and one child died ,among the other 38 cases treated, 28 children have reached the normal development quotient (DQ)(73.7%); 10 children fell behind in DQ(26.3% ). Those children started to treat before 40 days after birth were normal DQ. In 19 children whose parents co-operated with doctors actively and among them 18 chilchen got normal DQ, only one child had low DQ. 【Conclusions】 The early beging of the treatment and the co-opera tion from the parents have direct influence on the curative result of the PKU children. If the children could get the treatment within a month after birth and could get their parents active co-operation,low DQ could be prevented.