摘要
目的:观察婴儿肝炎综合征与半乳糖血症的患儿血浆氨基酸变化特点及与疾病的关系方法:对30例婴儿肝炎综合征和10例半乳糖血症的患儿于治疗前进行血浆17种游离氨基酸测定,并与52例年龄和性别相匹配的健康婴儿比较。结果:与正常对照组相比较,发现婴儿肝炎综合征的患儿血浆苏氨酸、胍氨酸、蛋氨酸、甘氨酸明显增高,分别为(175.4±58.1)、(29.7±14.4)、(49.7±28.2)、(257.6±63.5)nmol/mL(P<0.05),而丝氨酸、牛磺酸、异亮氨酸、亮氨酸、缬氨酸、色氨酸明显降低,分别为(119.9±36.8)、(74.6±36.6)、(48.1±16.9)、(88.5±25.9)、(139.8±40.5)nmol/mL、(44.7±17.3)nmol/mL(P<0.05);半乳糖血症的患儿血浆苏氨酸、胍氨酸、蛋氨酸、组氨酸、精氨酸和酪氨酸均高于正常对照组,分别为(428.7±276.7)、(172.9±153.8)、(139.5±82.3)、(89.6±25.3)、(106.1±56.4)、(154.5±94.1)nmol/mL(P<0.05),异亮氨酸、亮氨酸、缬氨酸、色氨酸、谷氨酰胺和丙氨酸则低于正常对照组,分别为(37.4±7.9)、(63.9±10.9)nmol/mL、(111.9±21.8)、(17.6±8.4)、(355.6±151.8)、(265.2±62.9)nmol/mL(P<0.05);婴儿肝炎综合征、半乳糖血症患儿所测定的血浆氨基酸谱中,除苯丙氨酸和赖氨酸外,其余氨基酸在两组间均存在不同程度的差异(P<0.05)。结论:婴儿肝炎综合征和半乳糖血症患儿的血浆氨基酸谱之间有明显改变,血浆游离氨基酸分析有助于把半乳糖血症从婴儿肝炎综合征中鉴别出来。
Objective： To investigate the plasma amino acid patterns in infantile hepatitis syndrome and galactosemia. Methods： Plasma free amino acid concentrations were measure in 30 patients with infantile hepatitis syndrome and 10 with galactosemia before therapy, with 52 age- and gender-matcbed cases of healthy infants selected as controls. Results： Compared with control group, plasma concentrations of threonine （175.4±58. 1 nmol/mL）, citrnlline （29.7±14.4 nmol/mL）, methionine （49.7±28.2 nmol/mL） and glycin （257.6 ±63.5 nmol/mL） were significantly increased （ all P 〈 0.05 ） , while those of serine （ 119.9± 36.8 nmol/mL） , taurine （ 74.6 ±36.6 nmol/ mL） , isoleucine （48.1 ± 16.9 nmol/mL） , leucine （ 88.5 ± 25.9 nmol/mL） , valine （ 139.8 ±40.5 nmol/mL） and tryptophane （44.7± 17.3 nmol/mL） were significantly reduced in infantile hepatitis syndrome group （all P 〈 0.05 ） ; in galactosemia group, plasma levels of threonine （428.7 ±276.7 nmol/mL） , citrulline （ 172.9 ± 153.8 nmol/mL） , methionine （139.5 ± 82.3 nmol/mL） , histidine （89.6 ±25.3 nmol/mL） , arginine （106. 1 ±56.4 nmol/mL） and tyrosine （ 154.5± 94.1 nmol/mL） were higher, while isoleucine （37.4±7.9 nmol/mL）, leucine （ 63.9± 10.9 nmol/ mL）, valine （ 111.9 ±21.8 nmol/mL）, tryptophane （ 17.6± 8.4 nmol/mL）, glutamic acid （355.6 ± 151.8 nmol/mL） and alanine （265.2± 62.9 nmol/mL） were lower than those in control group （all P 〈 0.05 ）. Differences in plasma amino acid patterns, except for phenylalanine and lysine, were found between pediatric patients with infantile hepatitis syndrome and galactosemia （ P 〈 0. 05 ）, Conclusion： Plasma amino acid patterns differ significantly in patients with galactosemia from those with infantile hepatitis syndrome, Analysis of plasma free amino acids may help screening of liver damage and differential diagnosis between infantile hepatitis syndrome and inherited metabolic disorders.
出处
《广州医学院学报》
2007年第2期26-28,共3页
Academic Journal of Guangzhou Medical College
基金
广州市卫生局一般引导项目(2005-YB-176)
