目的 探讨间变性大细胞淋巴瘤激酶蛋白(ALK)在外周神经母细胞性肿瘤(pNT)中的表达及意义.方法 收集46例pNT的临床病理资料,采用免疫组化方法检测ALK蛋白,荧光原位杂交技术检测N-MYC基因.结果 46例患儿临床分期:Ⅰ～Ⅱ期和Ⅲ～Ⅳ期各23例;病理诊断:神经母细胞瘤(NB)40例、节细胞性神经母细胞瘤(GNB)6例,其中预后良好型(FH型)32例、预后不良型(UH型)14例;ALK在NB和GNB中的阳性表达率分别为55％(22/40)和0(0/6),差异具有统计学意义(P=0.038).40例NB中N-MYC基因扩增阳性4例、获得7例,6例GNB均无N-MYC基因扩增和获得.15例新生儿NB中ALK蛋白阳性表达率为40％(6/15),100％N-MYC基因扩增阴性.ALK蛋白在Ⅲ～Ⅳ期、UH型和N-MYC基因异常中表达率高,差异具有统计学意义(P=0.018、0.001、0.010),在不同性别、年龄和部位间无差异表达(P=0.382、0.400、0.617).结论 儿童pNT应常规检测ALK蛋白,其阳性表达与病理分型和临床分期有一定的关系.不同年龄段NB中ALK蛋白的表达意义及机制有待进一步研究.

Objective To investigate the expression and clinical significance of transglutami-nase 2(TGM2)in Henoch-Sch?nlein purpura nephritis(HSPN).Methods The mRNA expression of TGM2 in peripheral blood was detected by real-time PCR in 20 children with Henoch-Sch?nlein purpura(HSP),20 children with HSPN(ISKD grade Ⅱ in 4,grade Ⅲ in 12 and grade Ⅳ in 4), and 20 healthy controls.The protein expression of TGM2 in kidney tissue was measured by im-munohistochemistry in HSPN children and 5 healthy controls.Results The mRNA expression of TGM2 in peripheral blood in HSP control group was lower than that in control group or HSPN group(P <0.05).There was a significant difference between control group and HSPN group in renal TGM2 protein expression(P <0.01),but not in peripheral blood TGM2 mRNA expression (P > 0.05 ).The expression of TGM2 was positively correlated with the pathological HSPN grades(P <0.001).Conclusion The expression of TGM2 plays an important role in HSPN and is associated with renal pathological grades,suggesting that TGM2 may be involved in the pathogen-esis of HSPN.