先天性心脏病患儿的免疫功能状态

目的：先天性心脏疾病(CHD)患儿易发生感染,有作者认为CHD可能是DiGeorge综合征(DGS)的一部分,CHD患儿对感染的易感性与其存在不同程度的免疫缺陷有关。本文探讨CHD患儿有无免疫功能缺陷,结合文献讨论CHD与DGS的关系。方法：通过胸部X片回顾性观察因患肺炎而住院的72例单纯性和34例复杂性CHD新生儿胸腺影的大小,50例同日龄肺炎新生儿作为对照。检测28例学龄前期CHD患儿外周血淋巴细胞亚群、淋巴细胞增殖功能及外周血单个核细胞(PBMC)白细胞介素-4(IL-4)和干扰素-γ(IFN-γ)mRNA表达情况及培养上清中IL-4和IFN-γ水平,血浆IgG、IgA、IgM及C3水平。20例同年龄健康儿童作为对照。结果：所有新生儿胸片均可见到胸腺影,单纯性和复杂性CHD新生儿胸腺影大小与肺炎新生儿比较差异均无显著性(P>0.05);学龄前期CHD患儿外周血CD3+,CD4+,CD8+,CD19+及CD16+CD56+T细胞和CD4+/CD8+与对照组差异无显著性(P>0.05);血浆免疫球蛋白及C3水平与对照组比,差异也无显著性(P>0.05);PBMC加植物血凝素(PHA)和脂多糖(LPS)刺激后的每分钟脉冲数、PBMC培养上清中IL-4,IFN-γ水平和mRNA表达与对照组比较差异无显著性(P均>0.05)。结论：并非所有CHD患儿均伴有胸腺发育不全或免疫功能缺陷,CHD患儿易感染不一定是先天性免疫功能低下的表现。

Immunological Function in Children with Congenital Heart Disease

OBJECTIVE: It was thought that congenital heart disease (CHD) may be part of DiGeorge syndrome (DGS) and that the susceptibility to infection in children with CHD was related to immonodeficiency. This study explores whether immunodeficiency is present in children with CHD and aims to clarify the relationship between CHD and DGS by reviewing published papers. METHODS: The sizes of thymus shadow on chest X ray film were measured in 72 neonates with simple CHD, 34 neonates with complex CHD (n= 34) and 50 neonates with pneumonia. The partial immunologic laboratory data, including lymphocyte subsets counts, the peripheral blood monoeuclear cell (PBMC) IL-4 and IFN-7 mRNA expressions and production in culture supernatant, the PBMC proliferative response to phytahematoagglutinin (PHA) or lipopolysaccharide (LPS), and plasma IgG, IgA, IgM and complement 3 (C3) levels' were measured in 28 pre-school children with CHD and 20 age-matched healthy children. RESULTS: The thymus shadows on chest X-ray films were found in both neonates with CHD and neonates with pneumonia. There was no difference in the sizes of the thymus shadow between them. The peripheral lymphocyte subsets counts in children with CHD did not differ from those in the healthy children. There were also no differences in the plasma IgG, IgA, IgM and C3 levels between them. The counts of per minute impulse (cpm) of PBMC induced by PHA and LPS and the PBMC IL-4 and IFN-γ mRNA expressions in children with CHD did not differ from those in the healthy children. CONCLUSIONS: Not all children with CHD have congenital thymus aplasia or immunodeficiency. It is not certain that primary immunodeficiency is the reason why children with CHD may be prone to infection.