CAJ | 학술논문

目的探讨肌张力低下型脑瘫患儿血氨等生化因素及血氨基酸代谢情况与患儿IgA+C3、喂养困难、易疲劳之间的相关性. 方法选择自2009年1月至2012年1月郑州市儿童医院康复中心住院的96例肌张力低下型脑瘫患儿为研究对象,采用问卷调查法了解患儿临床症状,常规血生化检测血氨、肝功能、IgA+C3及血串联质谱检测患儿氨基酸代谢情况,分析血氨基酸与血氨、喂养困难、IgA+C3、易疲劳之间的关系. 结果 96例患儿中检出精氨酸含量降低63例(65.63％),其中精氨酸含量降低且血氨水平升高的有52例,两者间存在负相关关系(r=-0.776,P=0.000);精氨酸含量降低且IgA+C3含量降低的有42例,两者间存在正相关关系(r=0.351,P=0.000);精氨酸含量降低且喂养困难的有47例,两者间存在正相关关系(r=0.372,P=0.000).96例患儿中,肉碱代谢异常30例(31.25％),其中丙酰肉碱/游离肉碱、丙酰肉碱/乙酰肉碱比值降低21例(21.88％),羟棕榈酰肉碱/羟棕榈烯酰肉碱比值升高9例(9.37％),半胱氨酸含量降低3例(3.12％).结论肌张力低下型脑瘫患儿存在氨基酸代谢异常.大量运动会引起体内精氨酸、肉碱及半胱氨酸的消耗增加,加速患儿疲劳.血精氨酸降低的患儿常伴血氨水平增高、IgA+C3含量降低,表现出易呕吐、易感染、喂养困难等症状,影响患儿的精神状态、免疫功能和运动耐受适应能力.
목적 탐토기장력저하형뇌탄환인혈안등생화인소급혈안기산대사정황여환인IgA+C3、위양곤난、역피로지간적상관성. 방법 선택자2009년1월지2012년1월정주시인동의원강복중심주원적96례기장력저하형뇌탄환인위연구대상,채용문권조사법료해환인림상증상,상규혈생화검측혈안、간공능、IgA+C3급혈천련질보검측환인안기산대사정황,분석혈안기산여혈안、위양곤난、IgA+C3、역피로지간적관계. 결과 96례환인중검출정안산함량강저63례(65.63％),기중정안산함량강저차혈안수평승고적유52례,량자간존재부상관관계(r=-0.776,P=0.000);정안산함량강저차IgA+C3함량강저적유42례,량자간존재정상관관계(r=0.351,P=0.000);정안산함량강저차위양곤난적유47례,량자간존재정상관관계(r=0.372,P=0.000).96례환인중,육감대사이상30례(31.25％),기중병선육감/유리육감、병선육감/을선육감비치강저21례(21.88％),간종려선육감/간종려희선육감비치승고9례(9.37％),반광안산함량강저3례(3.12％).결론 기장력저하형뇌탄환인존재안기산대사이상.대량운동회인기체내정안산、육감급반광안산적소모증가,가속환인피로.혈정안산강저적환인상반혈안수평증고、IgA+C3함량강저,표현출역구토、역감염、위양곤난등증상,영향환인적정신상태、면역공능화운동내수괄응능력.
Objective To explore the correlations of biochemical factors as gamma-aminobutyric acid (GABA) level with amino acid metabolism level in the blood, levels of intestinal immunoglobulin A (IgA)+complement factor 3 (C3), feeding intolerance and fatiguability in children with hypotonia cerebral palsy. Methods Ninety-six children with hypotonia cerebral palsy,admitted to our hospital from January 2009 to January 2012,were chosen in our study; questionnaire was used to understand the clinical symptoms of the children; the blood ammonia level,hepatic function and IgA+C3 level were obtained from routine blood chemistry testing, and amino acid metabolism was detected by blood tandem mass spectrometry; the correlations of GABA level with amino acid metabolism level in the blood, levels of IgA+C3, feeding intolerance and fatiguability were statistically analyzed. Results In all the 96 children with hypotonia cerebral palsy,63 (65.63％) had low arginine; 52 had both decreased arginine and elevated blood ammonia levels, enjoying negative correlation (r=-0.776,P=0.000); 42 had decreased arginine and reduced levels of IgA+C3 enjoying positive correlation (r=0.351,P=0.000); both decreased arginine level and feeding intolerance were noted in 47 with positive correlation (r=0.372,P=0.000).In these 96 children,30 (31.25％) had carnitine metabolism abnormality,including decreased propionyl carnitine/free carnitine or propionyl carnitine/acetylcarnitine levels in 21 (21.88％),increased hydroxyl palmitoyl carnitine/hydroxyl Palm enoyl carnitine level in 9 (9.37％),and decreased cysteine content in 3 (3.12％). Conclusion Metabolic abnormalities of arginine,carnitine and cysteine are noted in children with hypotonia cerebral palsy; a lot of exercise will consume arginine,carnitine and cysteine,which causes fatigue; children with low blood arginine content might also have increased blood ammonia level,reduced IgA+C3 level,trends of vomiting,susceptibility to infection and feeding difficulties,and therefore,children's mental state,immune function and exercise tolerance ability are affected.