Brucato A, Franceschini F, Gasparini M, De Juli E, Ferraro G, Quinzanini M, Vignati G, Bortolon C, Ghessi A, Pozzoli R
Dipartimento di Cardiologia De Gasperis, Ospedale Niguarda, Milano, Italy.
J Rheumatol. 1995 Mar;22(3):533-40.
To assess the longterm outcome of mothers of children with isolated congenital complete heart block (CCHB), and the maternal specific immunoblot pattern and HLA antigens.
Fifteen mothers of 16 children with isolated CCHB were investigated; their followup extended up to 15.8 years on average after the index delivery. Anti-Ro and La antibodies were detected by counterimmunoelectrophoresis and ELISA; anti-Ro antibodies were studied by immunoblot. HLA typing was done using a microcytotoxicity test.
One mother has systemic lupus erythematosus (SLE) before the index delivery. The other mothers developed only minor symptoms (arthralgia, dry eyes and photosensitivity) resembling primary Sjögren's syndrome more than classic lupus. All 15 mothers were anti-Ro and 9 were also anti-La positive, a mean of 12.5 years after the index delivery. Eight mothers reacted with the 52 kDa SSA(Ro) component, and 2 also with the 60 kDa SSA(Ro) component. The prevalence of the DR3 antigen and of the B44/DR5, DR3/DQ2 and A1/Cw7/B8/DR3/DQ2 haplotypes was significantly increased.
The longterm outcome for the mothers of children with CCHB is more reassuring than generally assumed. All the mothers were anti-Ro positive by sensitive ELISA: Reactivity to the denaturated 52 kDa SSA(Ro) component seems characteristic of these mothers, who presented a particular immunogenetic background.
评估孤立性先天性完全性心脏传导阻滞（CCHB）患儿母亲的长期预后，以及母亲的特异性免疫印迹模式和HLA抗原。
对16例孤立性CCHB患儿的15位母亲进行了调查；自首次分娩后，她们的随访平均长达15.8年。通过对流免疫电泳和酶联免疫吸附测定法检测抗Ro和抗La抗体；通过免疫印迹研究抗Ro抗体。使用微量细胞细胞毒性细胞毒性试验进行HLA分型。
1位母亲在首次分娩前患有系统性红斑狼疮（SLE）。其他母亲仅出现轻微症状（关节痛、干眼症和光敏反应），这些症状更类似于原发性干燥综合征而非典型狼疮。所有15位母亲均为抗Ro阳性，9位母亲也是抗La阳性，这是在首次分娩后平均12.5年时的情况。8位母亲与52 kDa SSA（Ro）成分发生反应，2位母亲还与60 kDa SSA（Ro）成分发生反应。DR3抗原以及B44/DR5、DR3/DQ2和A1/Cw7/B8/DR3/DQ联合单倍型的患病率显著增加。
CCHB患儿母亲的长期预后比一般认为的更令人安心。通过敏感的酶联免疫吸附测定法，所有母亲均为抗Ro阳性：对变性的52 kDa SSA（Ro）成分的反应似乎是这些母亲的特征，她们呈现出特定的免疫遗传背景。
