Buyon J, Roubey R, Swersky S, Pompeo L, Parke A, Baxi L, Winchester R
Department of Medicine, Hospital for Joint Diseases, NYU School of Medicine, NY 10003.
J Rheumatol. 1988 Jul;15(7):1104-8.
A retrospective literature review revealed that in 41% of cases of complete congenital heart block (CCHB) there was at least one other affected sibling, emphasizing the considerable risk of carrying a second affected fetus with CCHB. Therefore an aggressive approach was taken to prevent CCHB in a fetus with a high risk for the condition as defined by (1) presence of DR3 and high titers of antibodies to SSA(Ro) and SSB(La) in the mother (2) previous history of CCHB in a sibling. In a feasibility study, thrice weekly plasmapheresis was initiated in the 19th week of gestation to remove antibodies from the maternal circulation in advance of major placental transport to the developing fetus. Prednisone was also administered to decrease antibody synthesis. The concentration of total maternal IgG and antibodies to SSA(Ro) and SSB(La) were decreased by greater than 60% during the course of therapy. Planned delivery of a healthy baby was done at 36 weeks.
一项回顾性文献综述显示，在41%的完全性先天性心脏传导阻滞（CCHB）病例中，至少有一个其他受影响的兄弟姐妹，这凸显了怀有第二个患有CCHB胎儿的巨大风险。因此，对于符合以下条件定义为该病高风险胎儿采取了积极措施来预防CCHB：（1）母亲存在DR3以及高滴度的抗SSA（Ro）和抗SSB（La）抗体；（2）兄弟姐妹有CCHB既往史。在一项可行性研究中，妊娠第19周开始每周进行三次血浆置换，以便在主要胎盘转运至发育中的胎儿之前，从母体循环中清除抗体。还给予泼尼松以减少抗体合成。治疗过程中，母体总IgG以及抗SSA（Ro）和抗SSB（La）抗体的浓度降低了60%以上。计划在36周时分娩出健康婴儿。
